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pmc-6086026-1
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In September 2015, a middle age woman (44 years old) resident from Recife, northeast Brazil, gave birth to a male in a local public hospital. At birth, the neonate had 2580 g, 45.5 cm in length and head circumference of 29.5 cm (suspected case of microcephaly). The child was born after a 38 weeks single-gestation period (full-term), during the first trimester of pregnancy the mother reported a febrile episode followed by headache, joint pain, and rash, the symptoms described did not last more than 3 days and no other symptoms were reported, dengue virus (DENV) IgM serology was negative. At 20 weeks of the gestational age, a prenatal intrauterine ultrasound was performed and the diagnosis was consistent with congenital microcephaly. After birth, during the first month of life, a complete brain imaging examination of the infant evidenced the findings consistent with severe microcephaly, following the protocol of the Brazilian Ministry of Health for Microcephaly Investigation. Brain imaging (magnetic resonance) demonstrated the presence of lissencephaly, decreased brain parenchymal volume, decreased cortical mantle and white matter together with hypoplasia of the corpus callosum. Computed tomography and transfontanellar cranial ultrasound evidenced the presence of multiple brain calcifications, colpocephaly and gliosis in the left cerebellar hemisphere were also documented (Fig. ). Serological tests were performed on the mother (18 days after she gave birth), the results for STORCH laboratory screen (syphilis, toxoplasmosis, rubella, cytomegalovirus and herpes simplex virus), Parvovirus B19 IgM and chikungunya virus (CHIKV) IgM were all negative (Fig. ), no ZIKV serological (ELISA or PRNT50) or molecular tests (rRT-PCR) were performed during pregnancy since these tests were not available at that time, being implemented in Brazil only later in 2016.
Laboratory STORCH tests were also performed in the child with 1 month of life, the results were all negative, including chikungunya IgM and anti-Epstein-Barr virus (EBV) IgM (Fig. ). No Zika virus serology or molecular tests were performed on the child and no samples were stored for later investigations. After 1 month of life, a detailed neurological examination in the infant evidenced upper and lower limb spasticity (mainly on upper limbs), extreme irritability, continuous cry and head circumference measurement of 31 cm was consistent with severe microcephaly (< 3 SD on the Fenton growth chart).
With approximately 6 months of life, the child was admitted to a local hospital after presenting several recurrent seizure episodes. Electroencephalographic (EEG) examination evidenced the presence of focal frontal epileptiform discharges. At the hospital, a blood and a cerebrospinal fluid (CSF) samples were collected. Laboratory results of serum and CSF confirmed the presence of ZIKV viral RNA on both samples, analyzed by rRT-PCR following a well-established laboratory protocol []. Additionally, rRT-PCR for chikungunya (CHIKV) and ELISA tests were all negative in serum and CSF as follows anti-ZIKV IgM, anti-DENV I, M and IgG, anti-CHIKV IgM and IgG (Fig. ). To confirm ZIKV infection a plaque reduction neutralization test (PRNT50) was performed from the CSF sample, a ZIKV positive neutralization titer of 99 confirmed the previous infection, no neutralizing DENV antibody titers were observed (Table and Fig. ).
The interesting presence of ZIKV RNA previously identified at 6 months of life prompted us to investigate ZIKV persistence in a second sample collected with the age of 1 year and 5 months (here denominated 17 months for a better understanding). Given the risk of an invasive lumbar puncture, only a blood sample was collected from the child following a regular visit to the pediatrician, on the same occasion, a blood sample from the mother was also requested. On child serum, after 17 months of life, ZIKV rRT-PCR was still positive. Serology was negative for the tested arboviruses as follows anti-ZIKV IgM, anti-DENV IgM and IgG, anti-CHIKV IgM and IgG (Fig. ). A plaque reduction neutralization test (PRNT50) was again performed on child serum, ZIKV positive neutralization dilution titer of 527.9 reinforced our previous result of ZIKV infection, and again no neutralizing DENV antibody titers were observed. Moreover, the presence of a high titer of ZIKV neutralization antibodies (titer of 972) on mother’s serum confirms Zika virus infection (Table and Fig. ). On mother’s serum the presence of positive DENV neutralization titer is not surprisingly since dengue virus is endemic in Brazil, especially in the northeast region.
It’s intriguing the presence of ZIKV RNA for such a long time and since samples were stored with RNA preservative solution viral isolation was not attempted. Thus, to further confirm the presence of ZIKV on child CSF and serum we performed genome sequencing direct from blood and CSF positive samples, employing a previously published protocol []. We obtained two partial ZIKV genomes from samples CSF-six-months and serum-17-months of 6653 and 5292 base pairs, respectively, corresponding to 60.6 and 48.9% of genomic coverage (Fig. ). Phylogenetic analysis showed that these two draft genomes belong to the Asian genotype and clustered closely with a ZIKV isolate from Paraíba/Brazil (Fig. ), located around 120 km from where the mother gave birth. Since six-months-CSF sample differs from Paraiba_01 ZIKV strain by two single nucleotide polymorphisms (SNPs) in the envelope gene (E) and serum-17-months sample differs from Paraíba_01 by three SNPs, these results are consistent with a single ZIKV infecting strain. The child is being followed in a local hospital. Clinical evolution consists mainly in delayed neuropsychomotor development, dysphagia (difficulty swallowing), visual impairment, and double spastic hemiplegia. Treatment consists of clobazam (antiepileptic drug). After almost 2 years of life, the child presents controlled structural epilepsy.
CSF and blood were collected through standard procedures. Anti-dengue and anti-chikungunya virus IgM and IgG antibodies were detected by commercially available capture ELISA kits (Anti-Dengue Virus ELISA IgM/IgG and Anti-CHIKV IgM/IgG from EuroImmun AG, Luebeck - Germany), following manufacturers instructions. Serotype-specific anti-DENV and anti-Zika antibodies were assessed by plaque reduction neutralization test (PRNT). The antibody titer was determined as the serum dilution that inhibited 50% of the tested virus inoculum (PRNT50). Anti-Zika IgM antibodies were detected by Capture Enzyme-Linked Immunosorbent Assay (MAC-ELISA). Viral RNA was extracted by the use of a QIAamp Viral RNA kit (Qiagen, Hilden - Germany), following manufacturers instructions and rRT-qPCR reactions were performed from purified RNA serum samples accordingly to Lanciotti et al.[], with modifications. Briefly, reactions were performed with the kit GoTaq® Probe 1-Step RT-qPCR System (Promega, Fitchburg, USA) in a 20 μl final volume, primers and probes employed were as follows: Zika 1087 5’-CCGCTGCCCAACACAAG-3′, Zika1163c 5’-CCACTAACGTTCTTTTGCAGACAT-3′ and Zika 1087 VIC (probe) 5’-AGCCTACCTTGACAAGCAGTCAGACACTCAA-3′. Samples with a Ct value < 38 in duplicate wells were considered to be positive for ZIKV. For sequencing, total RNA extracted as above was converted to cDNA and amplified, PCR products were then quantified and libraries were prepared with Nextera XT Library Prep Kit (Illumina, San Diego - USA), MiSeq Reagent Kit V3 of 150 cycles was used to sequence employing a paired-end strategy. Mapped reads were visualized and majority rule consensus genomes were extracted with Integrated Genome Viewer (IGV) and carefully checked on the mapping results. Phylogenetic reconstruction was performed on an alignment of the coding region of all ZIKV genomes available by maximum likelihood. All sequences were aligned with Mafft v 7.
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pmc-6086047-1
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This is a 59- year-old -male patient without pathological history, followed in the nephrology department of the Mohammed V Military Teaching Hospital for renal insufficiency and anemia syndrome. The history and physical examination revealed stigmata of hemorrhagic syndrome including hemothorax and hemoptysis. The patients was not treat with anticoagulants.
The hemostasis assessment showed an isolated prolonged activated partial thromboplastin time (APTT) with APTT ratio of 2.0 (normal < 1.2). The prothrombin time (PT) (87%), the bleeding time (2 min and 30 s) and the fibrinogen level (2.88 g/l) were in the range of their physiologic values.
The exploration of prolonged APTT included: the confirmation of the prolongation of the APTT on two successive samples by using two different reagents: STA®-Cephascreen® (Diagnostica Stago) and STA®-PTT® automaton (Diagnostica Stago).The correction of the APTT in the mixing study performed by mixing equal parts of the patient’s plasma with normal pooled plasma, demonstrated the presence of circulating anticoagulants,the index of circulating anticoagulants was 10.7% and 37.2%, respectively, before and after 2 h incubation at 37 °C (normal < 15%), the dilute Russell viper venom time (dRVVT) showed the absence of lupus anticoagulants (LA) antibodies with normalized ratio of 0.99 (normal< 1.20) and the intrinsic pathway factors assay objectified the decrease of the factor XI activity corrected by the addition of the control plasma confirming the presence of anti-factor XI autoantibodies (Table ).
Furthermore, the blood count showed bicytopenia with non-regenerative normocytic normochromic anemia (Hemoglobin = 7.1 g/dL, mean corpuscular volume = 83 fl, mean corpuscular hemoglobin = 28.9 pg, mean corpuscular hemoglobin concentration = 34.9%, absolute reticulocyte count: 60800 / μl) and thrombocytopenia at 22000/μl. The blood smear demonstrated a plasma cell count of 49%, or 2.842 Giga / l evoking PCL. The bone marrow examination showed marrow invaded up to 90% by dystrophic plasma cells.
The biochemical assessment suggested downstream renal (hypercreatininaemia at 27.7 mg/dl and kidney failure with glomerular filtration rate estimated according to Modification of Diet in Renal Disease (MDRD) = 2 ml/min/1.73 m2) and electrolyte disturbances from exuberant light chain production with abnormalities including hyperuricemia (15 mg/dl), hypercalcemia (110 mg/l), elevated lactate dehydrogenase (LDH) (943UI/l), non nephrotic-range proteinuria (1.26 g/24 h) and high level of C reactive protein (CRP) (13.2 mg/l) .
The serum proteins electrophoresis revealed the presence of monoclonal peak migrating in the beta-1 globulins region amounted to 9.92 g/l with significant hypogammaglobulinemia. The immunofixation showed the presence of monoclonal free lambda light chains.
The radiological assessment did not show bone damage.
The patient was dialyzed, transfused and then treated with velcade, thalidomide and dexamethasone. He died after 2 weeks due to respiratory distress secondary to alveolar hemorrhage.
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pmc-6086052-1
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A 41-year-old man, a native of Cangnan County in the Zhejiang province of southeast China, was admitted to our hospital because of a 3-week history of daily hyperpyrexia and sputum-coughing in April 2017. The first time that multiple pulmonary nodules and bilateral hilar lymphadenopathy were found in chest CT (Fig. ) was 7 years ago. The patient was diagnosed with pulmonary sarcoidosis according to the results of a transbronchial needle aspiration (TBNA) and transbronchial lung biopsy (TBLB), which revealed lymphocytes, columnar epithelial cells and a cloud of epithelial-like cells. In the following years, he received follow-up chest CT examination and corticosteroid treatment irregularly. The patient met the ATS/WASOG diagnostic criteria for sarcoidosis because there was no progression of the lesions in recent years. With the pre-existing pulmonary sarcoidosis, he had been diagnosed with the progression of pulmonary sarcoidosis in a certain hospital in Shanghai 12 days prior. At that time, he was examined with chest CT and central ultrasound bronchoscopy. The chest CT showed space-occupying lesions of the right superior lobe, probably a malignant tumour, mediastinal and right hilum lymphadenopathy, and plaques and nodules disseminated throughout the bilateral lung, probably pneumoconiosis and metastasis (MT) (Fig. ). Compared to the initial chest CT performed in 2015 (Fig. ), Fig. shows increased miliary pulmonary nodules and a new pulmonary consolidation. Central ultrasound bronchoscopy revealed that a nodular projection was on the surface of both superior lobar bronchus and that stenosis appeared in the right superior lobar bronchus, especially the right apical segment (Fig. ). The patient received transbronchial needle aspiration (TBNA) 6 times when the ultrasound probed a tumour outside of the right primary bronchus and lymphadenectasis in 11R and 10 L. The pathology exam found fibrous tissue hyperplasia accompanied by apparent infiltration of monocytes and lymphocytes. There was no evidence of non-caseating epithelioid granuloma. Moreover, eosinophils were infiltrated in some areas. After 3 days of prednisone and levofloxacin, the fever and cough persisted, and there was no clinical improvement; even worse, skin lesions (Fig. ) erupted on his back.
The patient was pale and had intermittent high fever on the day of admission to our hospital. Born and raised in Cangnan, he denied a residential history in any epidemic regions. After his admission, imipenem cilastatin was promptly used against the infection. Immediate HRCT revealed bilateral lung diffuse miliary nodules, multiple patchy exudative shadows in the bilateral superior lobes and right inferior lobes, air bronchogram in the consolidation of the right superior lobe, multiple hilar and mediastinal lymphadenopathies and local pleural thickening (Fig. ). The image did not change much compared to the previous one. The results of the following laboratory routine examinations are shown in Table . Microbiology analysis showed that repeated sputum smears, sputum culture, and blood cultures were negative. Combining these clinical manifestations and biochemical analyses, the chance of tuberculosis diagnosis could not be excluded. However, the T-spot was negative, and acid-fast bacilli were not present. It was difficult to explain the extremely elevated IgE and eosinophils in the blood as well as the eosinophil infiltration in the bronchus on the monism of tuberculosis. After providing informed consent, the patient underwent bronchoscopy again, which revealed an unevenness of the trachea in the subglottic region as well as protrusions on the tracheal wall, especially in the right superior lobar bronchus (Fig. ). A subsequent (1/3) -b-D-glucan (G) assay was positive, and a smear was positive for fungus in the BAL. All the evidence above was associated with a higher likelihood of fungal infection. Along with the evidence that there was no clinical improvement after 4 days of antibacterial therapy, the patient was suspected of pulmonary aspergillosis because it was endemic in the region of Cangnan. He was immediately treated with voriconazole at a dosage of 200 mg/dL every 12 h via intravenous administration starting on April 14, 2017. Ultimately, the fungal culture of bronchoalveolar confirmed the diagnosis of T. marneffei infection on April 20 (Fig. ). His fever returned to normal, and his respiratory signs disappeared gradually after an 8-day treatment, as well as his skin lesions. The results of the chest CT re-examination showed that the lung lesions were markedly absorbed after 3 months (Fig. ). He continued to receive follow-up antifungal treatment.
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pmc-6086067-1
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The index patient was a 27-year-old Chinese Han female with non consanguineous parents. She was accidentally noted to have a low SUA level on two occasions (SUA = 0.52 mg/dl and 0.45 mg/dl) in her routine check-up in the local hospital. She was asymptomatic and had no other discomfort, such as joint pain, loin pain, hematuria, urine foam, nausea, vomiting, anorexia, or diarrhea. No special treatment was recommended. She was recently admitted into the metabolism division, the Affiliated Hospital of Qingdao University due to the repeated detection of hypouricemia; thorough laboratory and imaging examinations were carried out. Biochemical tests confirmed the diagnosis of hypouricemia (SUA = 0.33 mg/dl) with hyperuricosuria (FEUA = 50%). The current reference ranges for SUA used in our laboratory department are 3.5–7.0 mg/dL in males and 2.5–6.0 mg/dL in females. Meanwhile, the reference range for FEUA is 4–10%, irrespective of gender. Other biomedical findings were within normal range or negative. No kidney stones were found by local ultrasound. Acquired hypouricemia resulting from Fanconi syndrome and other clinical disorders were excluded. Both parents of the patient are alive and healthy, with no evident medical history, such as urolithiasis and AKI. The SUA concentration in the father was 3.68 mg/dl, which was within the lower-normal range, and the mother was 4.35 mg/dl, which was within the fully normal range. A FEUA test was not performed in both parents because they refused to have it done. The patient does not have any siblings. There is no history of hereditary diseases and there are no specific physical abnormalities. In addition, the patient stated she was a vegetarian who did not favor meat. Based on the overall information, we concluded that the patient had idiopathic RHUC. Clinical data are shown in Table . Pharmacologic therapy was not administered due to the absence of significant clinical manifestations and the lack of guidelines for RHUC management. However, several medical orders were required in order to inhibit the urate overproduction and prevent the incidence of adverse events: the patient was instructed to drink plenty of water and consume a low purine diet, avoid engaging in strenuous exercise, alkalize urine with sodium bicarbonate continuously and moderately, and monitor renal function regularly via laboratory and imaging examinations during subsequent outpatient follow-up. The case reports (CARE) guidelines were followed.
The patient and both of her parents were recruited for this study. Peripheral blood samples were collected upon the patient signing the relevant informed consent. Genomic DNA was subsequently extracted using LifeFeng Genomic DNA Purification Kit (Lifefeng Biotech Co., Ltd., Shanghai, China) and then quantified for concentration and purity evaluation by a NanoDrop 1000 Spectrophotometer (Thermo Scientific, USA). All of the samples were within the reference range (double-stranded DNA concentration: > 20 ng/ul, 260/280: > 1.8, 260/230: > 2.0) that satisfied the requirements of the genomic analysis.
The study was approved by the Ethics Committee of Shanghai Jiao Tong University and the Affiliated Hospital of Qingdao University, and it was conducted in accordance with the principles of the Declaration of Helsinki.
An initiation of 0.2 μg genomic DNA was subjected to WES. Genomic DNA was sheared into fragments of ~ 150 bp in size by a Covaris™ S2 Ultrasonicator System (Covaris, Woburn, MA, USA) and the product was ligated to paired-end adapters containing Illumina-specific indexes using a SureSelect XT Library Prep Kit ILM (Agilent Technologies, Santa Clara, CA). The resulting product was amplified by ligation-mediated polymerase chain reaction (LM-PCR). The adapter-ligated DNA library was enriched and captured with a SureSelect Target Enrichment Kit Box 1 (Agilent Technologies, Santa Clara, CA). Each well-constructed library was then sequenced as 150 bp paired-end reads on an Illumina HiSeq 2500 platform (Illumina, San Diego, California, USA), at the coverage depth of 50×. Briefly, crude sequencing data were checked for quality control by FastQC v 0.11.2 () and then trimmed by Trimmomatic v 0.30 to exclude unqualified reads. The resulting clean reads were aligned to NCBI build GRCh37/hg19 by Burrows-Wheeler Aligner (BWA) v 0.7.7. The aligned reads were processed to remove the duplications by Picard v 1.110. Then, local realignment and annotation with RefSeq genes, dbSNP 138, and 1000 Genomes Project were performed by Genome Analysis Toolkit (GATK) v 2.8–1 and Annovar software [], respectively.
Candidate mutations of interest were further validated by PCR amplification and direct Sanger sequencing. PCR primers were designed using a primer designing tool () and they were ordered from Shanghai Generay Biotech Co., Ltd., Shanghai, China. Primer sequences are listed in Table . PCR amplification was performed on a GeneAmp PCR System 9700 (Applied Biosystems, Foster City, CA) with 2X Taq PCR Master Mix (Lifefeng Biotech Co., Ltd., Shanghai, China), and the product was loaded on ABI 3100 instruments (Applied Biosystems, Foster City, CA) at Shanghai Jieli Biotechnology Co., Ltd. for Sanger sequencing.
Annotations with SIFT, Polyphen-2, LRT, and MutationTaster by Annovar, were performed to evaluate the mutation pathogenicity. Since the crystal structure has not been available, TMpred online software and Pymol 2.0, a free and user-friendly molecular visualization tool, were used to predict the secondary functional domains of the URAT1 protein and its 3-D image, respectively.
To screen out the genetic mutations leading to RHUC, WES was applied in genomic DNA from the RHUC patient and both of her parents. On average, the total number of bases in the reads was 4520 Mb and the ratio of bases having a phred score of greater than 30 (Q30) reached up to 94%. The detailed outputs are shown in Table .
According to an assumed inheritance of the autosomal recessive mode for this disease, we first analyzed the putative genetic mutations homozygous in the patient and heterozygous in both of the parents. Seeking functional variants, we filtered out those referred to as either unknown or synonymous. Focusing on rare variants, we sought for those having frequencies < 0.01 or unknown in 6500 Exome Sequencing Project database. Then, we excluded those present in the in-house exome sequence data obtained from 16 normal unrelated individuals, and finally, 29 variants were retained (see Sheet 1 in Additional file ). Unfortunately, none of the variants in the genes were functionally candidates for purine/uric acid metabolism and excretion. Next, we analyzed the putative causal mutations present in the patient but not present in the parents based on the prioritization strategy mentioned above. A total of 521 suspicious mutations were screened out (see Sheet 2 in Additional file ). In terms of the known candidate genes related to purine/uric acid metabolism and excretion, we finally chose the mutations located in the SLC22A12 gene for further Sanger sequencing.
Sanger sequencing revealed that the patient possessed two heterozygous mutations (see Table and Fig. ). One was a G to A substitution at nucleotide 269 in exon 1 (c.269G > A) leading to an alteration of arginine by histidine at codon 90 (p.R90H), which had been confirmed many times [, , , –]. Another was an insertion of GG at nucleotide 1289_1290 in exon 8 (c.1289_1290insGG), resulting in a frameshift and a truncated protein of 466 amino acids with a premature stop codon (p.M430fsX466), which was first reported in our study. The heterozygous c.269G > A was verified in the patient’s mother and the heterozygous c.1289_1290insGG in her father, suggesting that each heterozygous mutation of the patient derived from her mother and father, respectively (see Table and Fig. ).
The c.269G > A mutation is considered to be “damaging” by Polyphen-2 (= 0.992), LRT (= 0) and MutationTaster prediction (= 1), while it is “tolerated” by SIFT (= 0.061). Protein domain analysis showed that URAT1 has 12 transmembrane (TM) helices, and we made a discovery that amino acid substitution p.R90H, which is located in the intracellular region close to the second TM (TM2) (Fig. ), is highly conserved among different species (Fig. ). Further, we conducted molecular modeling to predict the effect of this mutation on protein structure (Fig. ). In the wild model, Arg90 formed hydrogen bonds with Cys88 and Gln93 effectively, building the conformational stability of this intracellular domain. In the mutation model, the hydrogen bond between His90 and Gln93 were weakly formed, which may decrease the stability of this domain and further affect urate transport activity. The insertion mutation c.1289_1290insGG produced a truncated protein of 466 amino acids from the position 430, with the amino acids sequence altered compared to the wild mature protein of 553 amino acids. Protein domain analysis showed that this truncated protein lacks the last three transmembrane domains, including the tripeptide motif (S/T)XΦ (X = any amino acid and Φ = hydrophobic residue) at the C-terminal, which interacts with scaffolding protein PDZK1, and together they strengthen urate transport [].
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pmc-6086074-1
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A 53-year-old African-American female with a history of uterine fibroids presented with a two-month history of nausea, vomiting, nervousness, weight gain and left lower quadrant abdominal pain. Weeks prior, she also went to the emergency department for similar symptoms where an abdominal computerized tomography (CT) scan showed an enlarged uterus along with fibroids, a hemorrhagic cyst of the right ovary and a teratoma of the left ovary (Fig. ) (Fig. ) (Fig. ). Regions of calcification were present measuring a maximum of 1.8 cm.
Physical exam revealed a soft, non-distended abdomen with pain upon deep palpation localized to the left lower quadrant. The thyroid gland was palpable, with an estimated weight of thirty to forty grams. It was nodular and nontender. The Pemberton sign checking for venous obstruction due to a goiter was negative. Tachycardia was present with a normal rhythm. S1 and S2 were normal with no S3, S4, gallops or murmurs detected. Initial labs revealed a mild normocytic anemia, a low TSH level and slightly elevated transaminases (Table ).
An endocrinology consultation was placed due to the abnormal TSH levels indicating hyperthyroidism. Baseline thyroid testing showed a TSH 0.04mIU/L (ref. range: 0.340–5.600mIU/L), free T4 level of 4.03 ng/dL (ref. range: 0.61–1.12 ng/dL) and total T3 of 451 ng/dL (ref. range: 60-181 ng/dL). A baseline thyroid ultrasound was also performed that showed a bilaterally enlarged thyroid gland, consistent with a goiter. Both lobes were heterogeneous. Multiple cystic and complex nodules were seen bilaterally. The largest of these measured 1.5 × 1.1 × 1.6 cm on the left lobe. There were small encapsulated nodules seen on each lobe. These findings were consistent with a multinodular goiter. The hyperthyroid symptoms improved after the administration of antithyroid medications: propranolol and methimazole.
Beta-hCG intact was measured twice, showing a value greater than 450,000mIU/mL. Because of the combination of ovarian and uterine pathology present, the patient was recommended for a total abdominal hysterectomy with bilateral salpingo-oophorectomy. Following surgery, b-hCG levels dropped from greater than 450,000mIU/mL to 200,000mIU/mL and continued to decrease over the course of the hospital stay (Fig. ). The methimazole and propranolol doses were also titrated as the hyperthyroidism became less symptomatic. Tissue diagnosis by the pathology laboratory confirmed the ovarian teratoma (Fig. ). Sections of the myometrial wall containing massive hydropic chorionic villi with the presence of intravillous vascular cistern were noted along with excess trophoblastic proliferation (Fig. ). This, along with the extremely high b-hCG levels, was consistent with the presentation of a complete hydatidiform mole.
After discharge, the patient underwent serial b-hCG measurements until the levels were undetectable to monitor the potential development of GTN. Post-surgical management also included methotrexate for chemotherapy. During the course of chemotherapy, lung nodules were found on a subsequent CT scan along with a slight upswing in b-hCG. However, as more time passed, the levels of b-hCG were eventually undetectable, and there was no subsequent choriocarcinoma post complete molar pregnancy.
Thyroid hormone levels were also monitored after discharge from the hospital and low-dose methimazole was continued. There was a significant improvement in the thyroid hormone levels 1 month following the surgery: TSH 0.5mIU/L, free T4 0.77 ng/dL and free T3 2.26 pg/mL (ref. range: 2.3–4.2 pg/mL). TSH levels were continuously monitored for 12 months following the surgery and remained normal. A year after the surgery, the patient also sought treatment at the Mayo Clinic, who performed a fine needle aspiration biopsy (FNAB) of a thyroid nodule. The patient reported the FNAB results as negative and the methimazole was then discontinued.
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pmc-6086494-1
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A 33-year-old woman was admitted to the intensive care unit after emergency cesarean section for respiratory failure due to suspected pulmonary embolism. The patient had clinical history of heterologous artificial insemination, without any evidence of deep vein thrombosis, or history of coagulopathy.
A CVC was placed in the right IJV for intravenous infusion of inotropes and sodium heparin under real time ultrasound guidance. During the procedure, there was no sign of accidental arterial puncture and no swelling was observed at the end of CVC placement.
A thoraco-abdominal Computed Tomography (CT) angiography was then performed, showing a subtotal atelectasis of the left lung with the presence of a pulmonary arterio-venous malformation. The patient underwent the embolization of pulmonary arterio-venous malformation and uterine arteries. However, due to massive hemothorax, the patient underwent an urgent left thoracotomy. In the early postoperative period, 3 days after the CVC placement, the patient complained dysphonia, swallowing disorder with progressive breathing insufficiency. A physical examination revealed the presence of a pulsatile mass in right lateral cervical region. A CT angiography of the neck demonstrated the presence of a 45-mm pseudoaneurysm in the right cervical region, compressing the IJV and adjacent neck structures (Fig. ). The patient was considered at risk for open repair due to high risk of intraoperative bleeding and for concomitant comorbidities, and a 2 steps procedure was planned. The first procedure was performed through a retrograde right common femoral artery access under sonography guidance. After 6-Fr, 55-cm-long sheath introduction (Flexor RAABE, Cook Medical Inc., Bloomington, IN), 5000 units of heparin were administered, and a BER II diagnostic catheter (4-Fr, 100-cm, Cordis Corporation, Bridgewater, NJ) was advanced into the brachiocephalic trunk with the support of a 0,035” hydrophilic guidewire (Cordis Aquatrack; Baar, Switzerland). In coaxial the sheath introducer was placed in right subclavian artery and the BER II was advanced till the origin of thyro-cervical trunk. A preliminary angiography demonstrated the presence of a giant false aneurysm of the right thyro-cervical trunk with its entry tear located below the right ascending cervical artery (Fig. ). Due to the large size of the sac, coil embolization was not preferred, and a balloon-expandable covered stent (Advanta V12, Atrium Medical Corporation, Merrimack, NH) was introduced over a 0,014” guidewire (Hi-Torque Command, Abbott Vascular, Santa Clara, CA) and deployed in the right thyro-cervical trunk achieving the complete coverage of communication between artery and pseudo aneurysm sac (Fig. ).
Two days after the endovascular repair, a color-doppler ultrasound of the neck confirmed the absence of flow and the complete thrombosis of the pseudoaneurysm sac, which was finally surgically drained in local anesthesia to resolve the compressive symptoms.
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pmc-6086502-1
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A 57-year-old female, with a history of tuberculosis and diabetes, visited the department of rheumatology in March 2016. She reported 19 years of swelling, pain, and numbness of multiple joints. The pain started on her ankles and feet, with both hands and feet numbness 19 years ago. Joints swelling and pain gradually developed in the metacarpophalangeal joints, proximal interphalangeal joints, wrists, shoulders, and knees, with morning stiffness for 1 hour. She was diagnosed with RA, and the drug treatment was unknown. Ten years after the diagnosis, she developed low back pain, which was more obvious at night than in daytime, and both the low back pain and joint pain were relieved after movement. Three years ago, a diagnosis of “binocular uveitis” was made due to the binocular vision blurring.
On physical examination, the patient presented heel tenderness, right 2 to 5 proximal interphalangeal joints swelling, left 2 to 5 proximal interphalangeal joints flexion and deformities. The skin on the hands showed rashes, thickening, desquamation and chapping, with both thenar muscles atrophy (Fig. ). She also had severe hypoalgesia of the whole body, and loss of the external third of the eyebrow. Lumbar spinous process and sacroiliac joints were slightly tender. Bilateral Patrick sign was negative. Bilateral Lasegue test was positive. Modified Schober test was 3 cm. Thoracic expansion was 3 cm. Floor-finger tip distance was 5 cm. Wall-tragus distance was 11 cm.
Initial laboratory test results were normal, including complete blood count, ESR (3 mm/h), CRP (1.7 mg/L), creatinine (44 μmol/L), transaminase (AST 18.9 IU/L, ALT 7.5 IU/L), anti-CCP (<25 RU/mL), anti-MCV (<40 IU/mL), and HLA-B27. RF was 33.90 IU/mL, but RF IgG was<25 IU/mL, RF IgA<25 IU/mL, RF IgM<15 IU/mL. ANA was positive, with speckled pattern, and weakly positive tuberculosis antibody. Electromyogram indicated extensive peripheral neuropathy. Chest computed tomography (CT): small nodules were seen in the lateral segment of the middle lobe of the right lung. X-ray of the hands (Fig. ): no bone destruction, the bone density of the metacarpophalangeal joints was reduced, the joint space was normal. X-ray of the spine: the curvature of the cervical spine was slightly straight, with lumbar degenerative changes. No abnormality was found in the MRI of the sacroiliac joint or the hips (Fig. ). Joint ultrasound: synovitis was found in all the metacarpophalangeal joints, proximal interphalangeal joints and bilateral wrists; the right finger flexor tendon and right ulnar tendon showed tenosynovitis.
The initial diagnosis was RA according to the 2010 ACR/EULAR RA classification criteria [ with 4 small joints involvement, low-positive RF, duration of symptoms >6 weeks, a total score of 6. However, this could not explain the systemic extensive peripheral neuropathy and thenar atrophy, and the joint deformities showed no destruction of bone or joint space narrowing in the radiological examination after a course of 19 years, which were inconsistent with RA. A detailed enquiry of the patient's medical history revealed that she had developed diffuse skin nodules on the whole body ten years ago, along with a loss of the external third of the eyebrow. She was diagnosed as lepromatous leprosy after skin biopsy. The biopsy was characterized by infiltration zone under the epidermis, which had mild atrophy and thinning. There were a few lymphocytes in the dermis and many different sizes of histiocyte masses with lightly stained cytoplasm. The appendages were reduced. Numerous bacilli were found by acid-fast stain (Fig. ). After three years of standardized treatment at the local epidemic prevention station, the skin masses disappeared. One year after drug withdrawal, the symptoms of joint swelling, pain, and numbness reappeared. Given the extensive peripheral neuropathy, chronic specific skin lesions, and previous skin biopsy diagnosis of lepromatous leprosy, the patient was diagnosed as joint damage and peripheral neuropathy due to Hansen disease.
After the diagnosis, the patient underwent two months neurotrophic treatment consisting of mecobalamin 500 μg orally tid and vitamin B1 5 mg orally tid, instead of anti-rheumatic treatment. At the 1-year follow-up, the arthritic symptoms did not disappear, but no further aggravation of joint swelling and pain was detected.
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pmc-6086511-1
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A 65-year-old male patient with a history of hypertension and hyperlipidemia was admitted with symptoms of angina pectoris in the preceding 9 months. An electrocardiogram revealed ST-segment depression in anteroseptal leads and pathological Q waves in inferior wall leads. An echocardiography determined a normal left ventricle cavity size with good systolic function and no any regional wall motion abnormalities (left ventricular ejection fraction 57%). Coronary angiography showed a severe stenosis in the proximal left anterior descending artery (LAD) (Fig. A) and a CTO in the proximal right coronary artery (RCA) (Fig. C). The complexity of the CTO was stratified using the J-CTO score (3; re-try lesion, blunt stump type, presence of calcification, and occlusion length ≥20 mm) and the PROGRESS CTO score (2; proximal cap ambiguity and absence of “interventional” collaterals).[
Firstly, the LAD artery was treated with a 3.5 mm × 18 mm biodegradable polymer drug-eluting stent (Fig. B). The RCA revascularization was then attempted employing a wire escalation strategy with the Fielder XT, Miracle 3 and 6 guidewires (Asahi Intecc., Nagoya, Japan), however without success. Hence a retrograde approach was initiated. The collateral connection from the left circumflex artery to RCA was avoided due to its severely corkscrew type tortuosity and the small collateral size (collateral connections 1, CC1). Consequently the septal 1 (S1) collateral channel was considered a viable option despite its diminutive size (CC class 0).
A Sion guidewire (Asahi Intecc., Nagoya, Japan) was advanced into the S1 branch supported by a Finecross MG coronary microguide catheter (Terumo Medical Corporation, NJ). A tip injection failed to identify collateral flow from the S1 branch to the distal RCA (Fig. A). Hereafter sodium nitroprusside (100 μg) was selectively injected twice through the Finecross microcatheter. Repeated tip injections ensued that the collateral size significantly improved from CC 0 to CC 1-2, and enabled adequate visualization of the distal RCA (Fig. A′, B, and B′). Quantitative angiography analysis was performed using the QAngio XA analysis software (Medis Medical Imaging Systems Inc., Leiden, The Netherlands). The mean reference vessel diameter of the first S1 branch increased from 0.71 to 1.20 mm (Fig. C and C′). Accordingly, the Sion guidewire was advanced with no difficulty to the distal segment of the occluded RCA with the assistance of the Finecross microcatheter (Fig. D). Hereafter, a retrograde wire crossing technique with the Sion, Gaia First, Gaia Second, and Conquest Pro guidewires (Asahi Intecc., Nagoya, Japan) was attempted but failed. Thus, a kissing wire technique was used for the recanalization of the occluded RCA with a Conquest Pro 8-20 guidewire supported by a Finecross 130 mm microcatheter (Fig. E). Retrograde angiography confirmed that the antegrade wire was in the distal true lumen of the RCA. Finally, 2 biodegradable polymer sirolimus-eluting stents (2.5 × 33 mm and 3.0×36 mm) were implanted from the distal to the proximal RCA after predilation (Fig. F and G). After post-dilation, final angiography and intravascular ultrasound demonstrated that stents were well expanded with excellent stent strut apposition (Fig. H).
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pmc-6086521-1
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A 24-year-old male with HA was admitted to our department with pain in multiple joints on May 23, 2011. The patient had a medical history of hemophilia A since the age of 3 and was intermittently treated with factor VIII. During these years, he sequentially developed left knee, left elbow, left hip, and right knee joint pain and swelling with limited activity and was soon diagnosed as HA. Initially, the joint manifestations could be largely relieved by factor VIII replacement therapy. Factor VIII inhibitor screening remained negative. Later, factor replacement therapy failed to achieve satisfactory effects, so in 2002 and 2006, he received left elbow synovectomy and left total hip arthroplasty, separately. In the subsequent years, the patient still suffered from the recurrent episodes of left elbow and bilateral knee joints hemorrhage, pain, and swelling. In recent 2 years, the frequency of joint hemorrhage had increased to approximately 2 times a week and only slightly relieved after factor VIII replacement therapy. Currently, the activity of those joints was limited to various degrees. Other medical history involved 2 cerebral hemorrhages 18 and 15 years ago, separately.
On physical examination, significant tenderness was noted in the left elbow joint with limited pronation and decreased grip strength. The preoperative Mayo elbow performance score (MEPS)[ was 55 for the left elbow. Moreover, knee valgus (left 20° and right 15°) was noted, and hyperextension, hyperflexion, and positive grinding test results were noted in both knee joints with a swollen and warm right knee. The preoperative Hospital for Special Surgery (HSS) knee scores[ were 58 for the left knee and 65 for the right knee.
Bilateral knee joints and left elbow joint exhibit advanced arthropathy on radiographs (Figs. A and 2A). These joints present narrowing of joint space, erosions of the articular facets, and bone deformation to various degrees.
Our diagnosis was hemophilia A and HA of the left elbow joint, both knee joints, and left hip joint. The patient received left elbow synovectomy and left total hip arthroplasty, but the condition continued to deteriorate over time with worsening of the left elbow and both knee joints. Taking all of these factors into account, surgical methods were our top priority, and simultaneous total multi-joint replacement was indicated. Due to the complicated joint lesions and medical conditions, our preparations for this arthroplasty were far more sufficient than usual. Given that arthroplasty for patients with hemophilia A, particularly the simultaneous replacement of multiple joints, is challenging, the patient and his family were informed in detail of the possible benefits and risks of the surgery. We performed a full musculoskeletal assessment and thorough medical evaluation beforehand. Blood products were prepared for possible bleeding events. Then, our team performed bilateral total knee arthroplasty (Zimmer NexGen) and left total elbow arthroplasty (Zimmer) under tightly regulated factor VIII replacement therapy. Antibiotic prophylaxis was administered 30 minutes prior to surgery, and an additional dose was administered once during the operation. Local hemorrhage was carefully controlled to prevent secondary joint damage. Approximately 1800 mL blood was lost during the entire surgery. The patient received 900 mL blood by autotransfusion and 4 units of red blood cells plus 800 mL fresh frozen plasma by intraoperative infusion. During surgery, we observed hemarthrosis and villous synovial hypertrophy at the joints, and severe erosion of the articular surface and various degrees of bone deformation were noted. These findings confirmed the preoperative diagnosis and preoperative assessments. After surgery, hemostasis management, such as compressive bandage, factor VIII infusion, and rigorous monitoring of coagulation indicators, was performed. An early rehabilitation program was applied to achieve improved regain of function.
We managed factor VIII replacement therapy during perioperative period under the guidance of hematologists. On the day of surgery, 3000 U/12 h (the body weight of this patient is 63 kg) factor VIII (ADVATE) was administered intravenously followed by 2000 U/12 h on postoperative days 1 to 3 (POD 1–3). Then, on POD 4 to 6, a dose of 1500 U/12 h was administered followed by 1000 U/12 h over the following 6 weeks. Factor VIII inhibitor remained negative in perioperative tests.
At the follow-up, the patient's joints functioned well. The MEPS of the left elbow was 85, and the HSS score of knee joints were 71 (left) and 81 (right). On radiographs (3 months and 5 years after operation), the arthropathy of bilateral knee joints and left elbow joint was significantly relieved (Figs. B,C and 2B,C).
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pmc-6086531-1
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A 62-year-old Chinese male was referred to our center with complaints of painless jaundice. He had no family history of cancer. His medical history was significant for pulmonary tuberculosis, which had been treated with medications. On admission, a physical examination showed moderately icteric sclera and jaundice. The circulatory, respiratory, and abdominal examinations were unremarkable. The initial laboratory tests revealed the following: elevated total bilirubin, 403 μmol/L (normal, 0–21 μmol/L); aspartate aminotransaminase, 153 U/L (normal, 5–40 U/L); alanine aminophosphatase, 93 U/L (normal, 8–40 U/L); carcinoembryonic antigen (CEA), 10.2 ng/mL (normal, 0–5 ng/mL), and carbohydrate antigen 19-9 (CA19-9), 1073.6 U/mL (normal 0–35 U/mL). A pulmonary computed tomography (CT) scan was negative except for a few areas of fibrosis resulting from the previous tuberculosis infection. Magnetic resonance cholangiopancreatography showed severe stenosis at the junction of the left and right common hepatic ducts and marked dilation of the intrahepatic bile ducts (Fig. ). An abdominal enhanced CT scan revealed a 2-cm, moderately enhanced mass in the hepatic hilum and regional lymph node enlargement (Fig. ). Percutaneous transhepatic biliary drainage was performed to relieve cholestasis and improve liver function. With a tentative diagnosis of pCCA (Bismuth IV type), the patient underwent surgical resection including a left hemihepatectomy, cholecystectomy, and lymphadenectomy. The reconstruction was achieved by Roux-en-Y hepaticojejunostomy. This procedure was considered curative since intraoperative frozen examination showed that the resection margin was free of atypical cells.
Within the resected specimen, a yellowish tumor measuring 2 cm × 0.5 cm × 1 cm was found in the hilar bile duct. Microscopically, the tumor showed a nested organoid growth pattern. The tumor cells were small in size and had round hyperchromatic nuclei and scant cytoplasm (Fig. A, B). Metastasis was detected in 1 of 3 resected hepatoduodenal ligament lymph nodes and 2 of 2 cystic lymph nodes. A detailed immunohistochemical (IHC) analysis confirmed a highly proliferative NEC that was chromogranin A (+), CD56 (+), synaptophysin (+), and Ki-67 (+, >80%) (Fig. C–F).
The postoperative course was complicated. The patient developed prolonged bile leakage, and adjuvant chemotherapy was postponed. Repeat abdominal imaging 2 months after the initial diagnosis showed tumor recurrence in the right liver lobe, and the patient died 6 months after the operation.
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pmc-6086556-1
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A 54-year-old male who had a medical history of membranous nephropathy II with nephrotic syndrome (Fig. A and B) was administered with long-term oral glucocorticoids and immunosuppressants. The patient had a 20 pack-year history of smoking, and denied a family history of hereditary diseases. Chest x-ray demonstrated normal findings at one month before admission (Fig. C–E). On August 8, 2016, the patient was hospitalized for fever accompanied by progressive dyspnea, cough, and expectoration for 5 days. On admission, the BMI of the patient was 24.5 kg/m2, and his body temperature was 39.0°C. Furthermore, the patient had symptoms of tachypnea (35 bpm) and severe hypoxemia (SaO2 86%). On auscultation, the patient had good air entrance bilaterally with scattered diffuse crackles and rhonchi. Furthermore, the chest CT scan revealed multiple ground-glass opacities (Fig. F–H), and laboratory tests (Table ) revealed normal white blood cell (WBC) count, but with elevated neutrophil count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and (1→3)-β-D-glucan (Table ). The patient was diagnosed as RSV infection on the fourth day of hospitalization when positive RSV-Ab was detected.
On admission, the patient was immediately given respiratory monitoring and supplemental oxygen to improve the low oxygen saturation, as well as antibiotics (moxifloxacin for 4 days, followed by cefminoxine for 8 days), and antifungal therapy (voriconazole for 10 days). The dose of the glucocorticoids and immunosuppressants remained largely unchanged (Table ). After 10 days of treatment, the patient's condition became worse. Chest CT revealed the progression of the disease (Fig. I–K), and oxygen partial pressure was further decreased (Table ). The patient was transferred to the Emergency Intensive Care Unit, where the patient was intensively treated, including noninvasive mechanical ventilation, broad-spectrum antibiotics (i.v. meropenem, oral moxifloxacin, and cotrimoxazole), antifungal therapy (micafungin), corticosteroids (methylprednisolone 40 mg bid iv) to relieve the inflammation, and other supportive treatment. Ganciclovir was also prescribed due to a possibility of viral infection, such as cytomegalovirus. Five days later, the patient's condition was further aggravated based on the chest x-ray evaluation (Fig. L). Despite receiving another round of treatments, including invasive ventilator-assisted ventilation therapy, methylprednisolone (80 mg bid), antibacterial agents (cefoperazone sulbactam, tigecycline, and cotrimoxazole) and antifungal (micafungin) therapy, the patient eventually died after 2 days.
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pmc-6086918-1
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A 61-year-old woman was seen in a clinic complaining of fever and dyspnea lasting 2 weeks. She was diagnosed with left pneumonia by computed tomography (CT) and referred to our institution. At the time of presentation, she had dyspnea and a fever over 38 °C. Her Eastern Cooperative Oncology Group (ECOG) performance status score was 2, and her Hugh-Jones classification was IV. Rhonchi were evident in the anterior chest. Laboratory studies showed an increased inflammatory response. All tumor markers were within normal limits. The chest X-ray revealed an infiltrative shadow in the lower left lung field. CT imaging showed a solid left main bronchial tumor with carinal involvement. Cartilage destruction was apparent, and the boundaries between the tumor and the esophagus and descending aorta were unclear (Fig. ). Therefore, tumor infiltration into the esophagus and descending aorta was suspected. We diagnosed her with obstructive pneumonia due to a tracheobronchial tumor. For the purposes of securing the airway, performing a tissue diagnosis and evaluating the extent of tumor progression, rigid bronchoscopy was initially performed. The tumor almost completely occluded the left main bronchus, and tumor hemorrhage was evident. By coring out the tumor, the left main bronchus was reopened, and detail of the involved area was revealed. The tumor originated from the left main stem bronchus and occupied almost the entire left main stem bronchus. Two tracheal cartilage rings above the carina and one right main stem bronchial ring distal from the carina were invaded by the tumor (Figs. and ). The pathological examination showed three typical types of histology (cribriform, tubular, solid pattern), and she was diagnosed with tracheobronchial adenoid cystic carcinoma. The rigid bronchoscopic treatment resulted in a significant improvement in the patient’s general condition and cardiopulmonary function (PS 2 → 0, H-J IV → I). Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed abnormal enhancement in the tumor with a maximum standard uptake value of 5.3. At the other sites, abnormal FDG uptake was not observed. We determined that if the tumor did not infiltrate the surrounding organs, complete resection would be possible via LSP. Even if a microscopic lesion remains in the resected stump, we can expect improvement in prognosis by administering additional postoperative irradiation. Therefore, we decided to perform the surgery.
To evaluate the presence of out-of-wall invasion, we proceeded with the left-side operation in the right lateral decubitus position by complete VATS with three ports in a look-up setting (Fig. ). A right-sided double lumen endotracheal tube was used. Initially, the pleura was opened along the subaortic window. We observed no tumor infiltration into the left recurrent laryngeal nerve, esophagus, or descending aorta. Next, we performed a left pneumonectomy by complete VATS. The left pulmonary artery and vein were dissected into their extrapericardial sections by staplers. Subsequently, the left main stem bronchus was cut near the second carina (where the tumor had not progressed) using the stapler. A protective bag provided easy removal of the left lung from the pleural cavity without enlarging the skin incision. After that, the tracheal carina was exposed circumferentially, and the lower trachea and right main stem bronchus were identified by forceps manipulation. The left chest was closed. After exchanging for a 7.5-Fr single-lumen endotracheal tube, the surgical position and approach were switched to the left lateral decubitus position with a posterior lateral incision and a fourth intercostal thoracotomy. Low volume ventilation with small expansion of the right lung simplifies the operation. Prior identification via left thoracotomy around the carina provided easy circumferential exposure of the trachea, carina, and right main stem bronchus after dissecting the azygous vein. Then, the right main stem bronchus was dissected at the two rings distal from the carina followed by surgical field intubation using a 6.5-Fr spiral tube with a short cuff. The tracheal carina was removed after dissection of the three rings above the carina (Figs. and ). Under surgical intubation, the trachea and right main stem bronchus were anastomosed with a telescope technique using interrupted sutures with full-thickness bites and 4-0 PDS. After completing a left-side semicircle anastomosis by surgical intubation, a right-side semicircle anastomosis was performed under intermittent removal of the tube after sufficient oxygenation during one or two stiches. There was no requirement for prepared jet ventilation. The anastomotic site was wrapped with the intercostal muscle pedicles, and the operation was terminated. To avoid tension on the anatomic site, the chin was tagged to the anterior chest wall by two sutures for 2 weeks. Pathological analysis revealed no tumor component in the resected stump, and we achieved complete resection. After the operation, the patient experienced a panic attack, and hospitalization was prolonged. She improved with psychiatric intervention. She was discharged and walking independently on postoperative day 79. Unfortunately, recurrence via bone metastasis to the left humerus was observed 6 months after surgery, and palliative irradiation is underway. We are currently monitoring her progress.
LSP is one of the most challenging operations in thoracic surgery, and surgical approaches need to be individualized. Bilateral thoracotomy and median sternotomy are often favored; [] however, as thoracoscopic surgery becomes mainstream, newer and less invasive approaches for extended surgery, such as LSP, are employed.
Cases for which LSP is indicated are generally locally advanced malignant tumors that often involve surrounding organs, and proper assessment is critical. If tumor invasion to the surrounding organs is suspected and preservation of the left lung cannot be expected upon initial diagnosis, this minimally invasive combined thoracoscopic approach has several advantages. With initial left-sided VATS, resectability can be evaluated in advance and in a less invasive manner than with thoracotomy. Confirmation of no invasion to the surrounding tissue makes left pneumonectomy beneficial. Right thoracotomy provides safety and precise anastomosis at the time of carinal reconstruction.
An initial right thoracotomy could be considered, but it is difficult to evaluate tumor involvement in the left thorax []. Initial right thoracotomy requires tube intubation through the narrowed left main stem bronchus with tumor invasion. It is difficult to insert a large caliber tube enough to maintain ventilation and oxygenation. In recent years, the usefulness of the clamshell approach for carinal reconstruction has been reported, [] but it has several disadvantages, such as poor visibility of the esophagus and descending aorta and the requirement of extensive detachment of respiratory muscles. The disadvantage of poor visibility is similar in anterior approaches such as the transsternal and hemi-clamshell approaches. However, our approach requires a position change, and there are also disadvantages relative to providing ventilation during airway anastomosis, which is complicated and difficult to address in an emergency. In our case, small volume ventilation from the surgical field provided precise anastomotic maneuvering. Because of the difficulty of laryngeal release, this approach is not suitable when the resection length of the trachea is relatively long.
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pmc-6087000-1
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A 3-year-old girl was admitted for fever and cough. She was diagnosed as having pleuresia and pleural drainage along with broad spectrum antibiotics was prescribed. Lack of improvement after a few days led to complete the work-up with a CT scan (Fig. a, b). The scan showed a tissular lesion of the left lower pulmonary lobe associated with a tumor of the right kidney. Lung biopsy showed blastema, without being able to distinguish whether its origin was WT or PPB despite multiple analyses by various pathological experts. Following the recommendations of the national panel of expert of both tumors, we decided to treat the patient as a metastatic WT following the International Society of Paediatric Oncology protocol (SIOP WT2001) []. A nephrectomy was thus performed after 6 weeks of chemotherapy (vincristine and actinomycin), with a good response in both sites (Fig. ).
Histologic analysis confirmed the diagnosis of WT with an intermediate risk (epithelial type) which was classified as a stage IV. Lung surgery was scheduled several weeks later. During this period, using high-throughput sequencing of a panel of genes involved in endocrine tumor development, we identified a heterozygous pathogenic variant in exon 23 of the DICER1 gene (LRG_492). This variant c.4407_4410del, p.Ser1470Leufs*19, leading to loss of the RNase III active site, has been previously associated with pleuropulmonary blastoma [].The mutation was confirmed by Sanger sequencing (Fig. ). This led to a high suspicion of associated PPB. Pre-operative workup showed increase in tumor size, and chemotherapy was modified (by adding ifosfamide and doxorubicin) to try to reduce tumor volume and vascularization. After the first course of chemotherapy, the patient developed respiratory distress due to a massive increase in tumor size, leading to mediastinal compression. It was then decided to remove the left lung (Fig. e, f).
A left pleuro-pneumonectomy with intra-pericardic ligature of the vessels and without cardiac assistance was performed. The early postoperative course was uneventful. The pathologist confirmed the diagnosis of type 3 PPB with R0 resection. Postoperative chemotherapy consisted of eight courses (27 weeks) of vincristine, actinomycin, and doxorubicin. After 1-year follow-up, the patient remains asymptomatic and is considered to be in remission.
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pmc-6087008-1
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A 67-year-old female patient presented with a complaint of per rectal bleeding. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a tumor 4.5 cm in diameter in right posterior wall of the middle rectum with no adjacent infiltration or lymph node metastasis (Fig. , ). Colonoscopy revealed a submucosal mass in the right posterior wall of the middle rectum 7 cm from anal verge (Fig. ). Histologically, a biopsy showed spindle-shaped cells arranged in bundles, positive for CD34 and negative for C-Kit, Desmin, smooth muscle actin, and S-100 (Fig. ). These findings suggested a rectal GIST, and TAMIS was scheduled.
The patient was kept in the modified lithotomy position, and the anus dilated with a self-retaining anal retractor (Lone Star Retractor; Cooper Surgical, Trumbull, CT, USA). A transanal access device (GelPOINT path; Applied Medical, Rancho Santa Margarita, CA, USA) was introduced. Wet gauze was inserted above the lesion, and pneumorectum was maintained at 15 mmHg with carbon dioxide by an AirSeal platform (AirSeal system; CONMED, Utica, NY, USA). Conventional laparoscopic instruments were used. The tumor was located at the right posterior wall in the middle rectum; the incision site 1 cm away from the tumor margin was tattooed circumferentially. Mucosal dissection was performed along the tattoo (Fig. ), and subsequent full-thickness excision was carried out (Fig. , ). The tumor was peeled off and extracted using an Endo Catch specimen pouch (Medtronic, Minneapolis, MN, USA) to avoid dissemination (Fig. ). Intraluminal lavage with saline was performed, and hemostasis was secured (Fig. ). The defect was closed with 3-0 V-Loc (Medtronic) under 8 mmHg pressure by the AirSeal system (Fig. , ). The specimen measuring 4.5 cm × 4.5 cm × 3.5 cm (Fig. ) was sent for histopathology, which confirmed a pT2 rectal GIST positive for KIT (CD117) and CD34. The resection margin was negative, and the mitosis count was 10 per 50 high-power fields. The postoperative period was uneventful with normal anal and urinary functions. The patient was discharged on IM (Gleevec), 400 mg once daily for 3 years under regular follow-up.
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pmc-6087528-1
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The first patient was a 66-year-old female with history of seizure and bedridden for the last three months presenting with hypotension, chest pain and dyspnea. The symptoms begun in the last seven days. Primary echocardiography showed severe RV dysfunction with systolic pulmonary artery pressure (SPAP) of 75 mmHg. The patient received reteplase and during 7 days of admission had no major bleeding or any complications. SPAP had reduced to normal (25 mmHg) in a follow-up echocardiography.
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pmc-6087528-2
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The second case was a 47-year-old male presenting with hypotension, chest pain and dyspnea that had lasted for three days. Doppler sonography showed acute deep vein thrombosis (DVT). Echocardiography showed severe RV dysfunction and RV enlargement with SPAP of 70 mmHg which decreased following reteplase treatment (SPAP=20 mmHg). No complications were noted with reteplase treatment.
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pmc-6087528-3
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The third case was a 64-year-old female who admitted with chest pain and non-ST elevation myocardial infarction; during admission she underwent percutaneous coronary intervention (PCI). Two days following PCI, the patient had persistent chest pain with hypotension and loss of consciousness, and was intubated. Imaging confirmed massive PTE and reteplase was administered. The patient had rectorrhagia in the first 24 hours and cerebellar hemorrhage 36 hours after reteplase administration. Patient was treated conservatively and was extubate in the next three days. Control brain imaging showed no further hemorrhage. Patient was discharged after 10 days with no further complications.
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pmc-6087528-4
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The fourth patient was a 49-year-old male, a known case of COPD and ischemic heart disease with no predisposing factor, presenting with chest pain and vertigo in the last two days. RV enlargement and dysfunction and SPAP of 50 mmHg were reported in the primary echocardiography, which improved in the post treatment follow-up echocardiography with normal RV function. Patient had no complications and discharged after 6 days.
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pmc-6087528-5
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The fifth patient was a 49-year-old male presenting with chest pain during the last two days with highest intensity at the time of visit. The patient had RV enlargement and dysfunction and SPAP of 60 mmHg in echocardiographic study, which improved to 30 mmHg following treatment. Three hours after reteplase administration, the patient had hemoptysis which was managed conservatively. The patient was discharged after 2 days with no complications.
After reteplase treatment, patients received heparin infusion and were discharged with warfarin. Patients were followed for three months and none had any complications during the follow-up period. All patients had follow-up echocardiography a week after discharge that indicated improved PAP and normal RV function. None of the patients died during the hospital stay and follow-up period.
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pmc-6087538-1
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A 30-year-old female presented to the emergency with progressive abdominal pain for about one month, which had increased in severity for the past 2 days. The pain was initially localized to the right iliac fossa, and was described as colicky, lasting for about 4–5 min with 2–3 episodes/day, partially relieved by analgesics. Over the past 2 days, the severity of the pain had increased and had become generalized. She also had multiple episodes of severe vomiting accompanied by obstipation for the same amount of time.
The patient had undergone laparoscopic sterilization 7 years ago and then underwent re-canalization one year back. The patient was initially managed at a primary health centre and was then referred to our hospital with a tentative diagnosis of small bowel obstruction due to adhesions with worsening of symptoms. On examination, the patient had tachycardia, abdominal distension with guarding and rigidity. No abdominal mass was palpable. A per rectal examination revealed hard fecal matter with rectal ballooning. A plain erect abdominal radiograph revealed multiple air-fluid levels suggestive of small bowel obstruction (Fig. ). Sonology was non-contributory. An abdominal computed tomography scan was suggestive of a mass lesion in the small intestine with mottled appearance (Fig. ). On surgical exploration, the small bowel was distended till about 30 cm from the ileocecal junction. An enterotomy was made at the site of the palpable sponge and the sponge was retrieved (Fig. ). Post operatively, the patient developed surgical site infection and was eventually discharged on day 7 of admission.
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pmc-6087565-1
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We present a case of a 39-year-old G8P6M1 Pacific Islander woman who at 15+5 weeks gestation was brought in by ambulance following an out-of-hospital cardiac arrest. The arrest was witnessed by her family at home who contacted the ambulance service and commenced cardiopulmonary resuscitation (CPR). She was resuscitated at the scene involving CPR with approximately 40 minutes downtime, cold intubation, and multiple direct current cardioversions for stabilisation. On arrival to the emergency department she had fixed dilated pupils and was found to be significantly acidotic (pH 6.7, lactate 26mmol/L) with associated hypokalaemia of 2.1mmol/L (range 3.5-5.2mmol/L) (see Tables and ). Her initial resuscitation and stabilisation involved a potassium infusion up to 40 mmol/hr and an adrenaline and noradrenaline infusion, 4 units of packed red blood cells, and 4 units of albumin. Following stabilisation and electrolyte repletion, she had a second 5-minute ventricular fibrillation (VF) arrest 4 hours later in the ICU, where her potassium on her preceding venous blood gas was 1.8mmol/L. She was commenced on 300mg of IV Thiamine daily from day one of her ICU admission. On day one of admission, the pregnancy was still viable with a FHR of 150beats per minute detected. Unfortunately, on day two of her admission, there was fetal demise and she spontaneously miscarried in the ICU and required a dilatation and curettage for retained products of conception. Her inpatient stay was complicated by multiorgan dysfunction including ischaemic hepatitis, mild encephalopathy requiring rehabilitation, and anuric renal failure requiring short-term dialysis.
The patient's pregnancy history was unremarkable preceding the out-of-hospital cardiac arrest except for an early positive oral glucose tolerance test (OGTT) (in the absence of evidence of type 2 diabetes mellitus with a normal HbA1C) performed at 13 weeks' gestation (see ). On the day of her arrest her husband did not notice any additional symptoms and her nausea and vomiting did not particularly worsen, but she did manage to tolerate a small lunch meal prior to arresting. She had a background history of 5 previous pregnancies to the same partner, complicated by some nausea and vomiting in those pregnancies, with no definitive evidence of HG. In this pregnancy, from an early gestation, the patient confirmed the presence of significant nausea and vomiting, with emesis occurring after every meal on most days. She had limited oral intake as a result and ensuing weight loss occured with approximately 10kg's lost in total (9% of total body weight).
The patient's background medical history was otherwise unremarkable with no symptoms or biochemical evidence of a disorder of potassium homeostasis preceding the pregnancy. Specifically, she denied symptoms to suggest hypokalaemic periodic paralysis and serial serum electrolyte testing revealed normal potassium levels before and after pregnancy (see ). Urine electrolyte testing was also unremarkable postpartum, with no evidence of renal potassium wasting (see ). Furthermore, she had no history to suggest an arrhythmogenic disorder with no palpitations, presyncope, or syncope reported. She had no significant family history of cardiomyopathy or sudden cardiac death and no personal history of valvular heart disease or rheumatic fever. She denied symptoms to suggest thyroid disease and pre-pregnancy had normal Thyroid Stimulating Hormone (TSH) levels on serial testing, including a normal TSH level at 7-weeks gestation. She was not hypertensive, and there was no evidence of primary hyperaldosteronism on testing. There was no clinical evidence of cortisol excess, and screening 24-hour urinary free cortisol was normal. She was not on any regular medications and had no known drug allergies.
The patient was thoroughly investigated for causes and contributors to her cardiac arrest. Results of cardiac investigations included a normal coronary angiogram, normal left ventricle ventriculogram, and a transoesophageal echocardiogram which demonstrated preserved biventricular systolic function and structurally normal valves with moderate mitral regurgitation. She had a normal computed tomography pulmonary angiogram (CTPA) with no evidence of pulmonary embolism. She had a largely normal CT head with a 10mm filling defect in her left transverse sinus but nil other acute pathology. She had a normal baseline electrocardiogram (ECG) post-arrest with no evidence of long QT syndrome.
Following stabilisation and correction of her potassium (see ) and acute renal failure, she was discharged home after a total 33-day admission. She transitioned well to home where she is continuing to care for her children, the youngest of which is 3 years old. She is independent with her activities of daily living and mobility and only suffered from mild short-term memory impairment and mild impairment in her concrete problem solving. Importantly, her serum potassium level remains normal.
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pmc-6087570-1
|
The patient was a 36-year-old gravida 0 woman. At the age of 7, she underwent ventricular septal defect closure for the right ventricular outflow tract. At the age of 11, she received a mechanical aortic valve replacement. Since after the replacement, she has been receiving warfarin orally at a dosage of 4.5 mg/day. She conceived naturally and she was referred to our hospital for perinatal management. Oral administration of warfarin was discontinued at 5 weeks of gestation and she began self-injection of heparin. At 21 weeks and 5 days of gestation, she was admitted to our hospital with a high risk of spontaneous abortion and was put on intravenous ritodrine. This successfully prevented a miscarriage. At 21 weeks and 6 days of gestation, we started a continuous infusion of 25,000 units of heparin daily. On the 22nd week, transesophageal echocardiography showed a movable thrombus in the aortic valve. The size of the biggest thrombus was 26 × 8 mm (). We increased the dosage of heparin to 28,000 units daily and restarted the administration of warfarin. Following this, the thrombus reduced in size, and at 23 weeks and 5 days transesophageal echocardiography showed no signs of thrombosis in the patient. At 32 weeks and 2 days of gestation, a routine cardiotocography showed a decreased fetal heart rate; thus, an emergency Cesarean section was performed under general anesthesia because of the presence of warfarin in the blood. The baby was delivered, weighing 1,702 g, with an Apgar Score of l at l minute, and 4 at 5 minutes. The total amount of blood loss during the surgery was 1,410 ml. During the surgery, 16 units of fresh frozen plasma (FFP) was transfused; and after surgery, we continued to infuse 20,000 units of heparin daily. On the 11th day after surgery, owing to continuous genital bleeding, heparin administration was discontinued and uterine artery embolization was performed. This treatment stopped the bleeding and on the 21st postsurgical day; we started warfarin administration at 5 mg/day. She was discharged on the 34th postoperative day due to the stable PT-INR levels ().
The newly born infant was intubated and admitted to the newborn intensive care unit. At the time of admission, activated partial thromboplastin time was 180 seconds or more and bilateral intracerebral ventricular hemorrhage was detected using ultrasonography. On the first day of life, anemia was observed in the infant and red cell concentrate and FFP were transfused (). We attempted to reduce the infant's dependence on the ventilator and at 8 days of age the infant was extubated. On the postnatal 10th day, a cranial CT scan showed bilateral intraventricular hemorrhage with ventricular dilation and midline shift (). Although convulsions accompanying the intracranial hemorrhage were observed, the infant's general condition was stable and oral feeding was started on postnatal day 10. The newborn was discharged on postnatal day 54. However, the infant later developed cerebral palsy and is currently receiving treatment at our hospital.
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pmc-6087572-1
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A 29-year-old female presented for evaluation of progressive right hip pain over the course of several months without a known injury event. She endorsed a constant pain, feelings of tightness in the hip, and a sensation of hip stiffening with ambulation. Physical exam demonstrated significant pain and guarding with passive hip motion, particularly hip flexion past 90 degrees, and provocative maneuvers such as both flexion/adduction/internal rotation (FADIR) and flexion/abduction/external rotation (FABER), suggesting an intra-articular source of her discomfort. There was no palpable mass or neurologic deficits.
The radiographic workup included an AP of the pelvis and special views of the right hip including Dunn lateral and a false profile. Radiographs revealed evidence of a mild mixed-type femoroacetabular impingement (FAI; acetabular crossover sign, 55 degree alpha angle, 25 degree lateral center-edge angle). There was no evidence of degenerative changes (Tönnis grade 0). Advanced imaging with a magnetic resonance arthrogram was available for review at the initial visit and demonstrated a pedunculated intra-articular mass in the superolateral aspect of the joint, near the femoral head-neck junction ().
Hip arthroscopy was performed in a supine position for resection of this lesion. The patient was placed in manual traction and standard midanterior and anterolateral portals were established. An extended intraportal capsulotomy was performed to allow for better access to the anterolateral femoral neck. On initial diagnostic arthroscopy, she was noted to have a concomitant anterior-superior labral tear. Minimal acetabular bone was resected and a three-anchor labral repair was performed. The traction was then released and evaluation of the peripheral compartment commenced. A nodular mass was encountered at the anterior and anterolateral femoral head-neck junction () and excised (). Fluoroscopic guidance was then utilized to perform a femoroplasty, and the hip capsule was closed in standard fashion.
Pathological examination of the mass was remarkable for a multinodular proliferation of benign fibroblasts with variable cellularity including hyalinized hypocellular areas (Figures and ). The fibroblastic cells were spindle to stellate shaped with amphophilic cytoplasm and uniform oval nuclei with small nucleoli. The vascular consisted of small capillaries along with elongated slit-like vessels (). Attenuated synovium was present on the outer surface of some of the nodules. Overall, the histopathological features were characteristic of FTS; however, this case was unusual in that it had an intra-articular multinodular growth pattern akin to that seen with diffuse tenosynovial giant cell tumor involving a large joint.
Postoperatively, the patient was made 50% weight-bearing on the right lower extremity with crutches for one month. No brace was utilized. She began physical therapy the second week. At her first follow-up appointment 10 days postoperatively, she noted resolution of her hip pain and prior symptoms of “tightness.”
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pmc-6087580-1
|
A 45-year-old male sustained a traumatic work-related patellar tendon rupture from the inferior pole of the patella while exiting a vehicle. The patient had a past medical history of diabetes mellitus type II. The patient was evaluated within 22 days of his injury and initially treated with primary repair 81 days after the injury. The tendon was repaired with two number 2 nonabsorbable sutures in a Krackow suture configuration throughout the length of the patellar tendon and anchored through bone tunnels in the patella. This patellar height was corrected to an Insall-Salvati Index (ISI) and Caton-Deschamps Index (CDI) of 1.23 and 1.14 () from 1.4 and 1.34, respectively (). His knee was immobilized in a locking brace for two weeks, and then physical therapy was initiated for range of motion at two weeks postoperatively. The patient progressed slowly through physical therapy gaining 100 degrees of active leg flexion but developed significant quadriceps atrophy, patella alta, and 10 degrees of an extensor lag at 7 months following the procedure. The patient was compliant with the standard rehabilitation protocol and had no history of traumatic reinjury. Eleven months after the primary procedure, the patient was referred to our clinic for persistent pain, pain with squatting and kneeling, instability, and stagnation in functional recovery which prevented him from returning to work up to this point. Subjectively, he reported a 4/10 pain level at rest. Clinical examination revealed proximal migration of the patella, 2+ coarse patellar crepitus, full active range of motion, 3+/5 quadriceps strength, and a 10-degree lag with single leg raise. T2-weighted MRI and lateral knee radiograph at 11-month follow-up confirmed patella alta deformity (CDI = 1.51, ISI = 1.55), an intact albeit lax patellar tendon, and cartilage fissuring near the inferior patellar apex (). There was no additional ligamentous injury noted on MRI. His preoperative patient-reported outcome scores can be found in . A collective decision was made with the patient at this time to proceed with revision patellar tendon repair with the goal of returning to work at some capacity and resuming his normal activities of daily living.
The previous midline incision was dissected to visualize and confirm obvious redundancy and thinning of the patellar tendon. A 2 × 4 × 1 cm rectangular block of redundant patellar tendon tissue was outlined and resected to correct the degree of patella alta. The patella was mobilized using blunt dissection. The suture material from the index repair was removed, and the distal patellar footprint was prepared. Two 2.5 PEEK corkscrew anchors (Arthrex, Naples, FL) were anchored 2 cm apart on the distal patellar footprint. Krackow sutures were passed through the midsubstance of the patellar tendon, and with the opposite limb of each stitch, a half hitch was made such that a pulley mechanism was created (). The tendon was reapproximated, and final fixation was secured with four mattress anchor knots with five alternating half hitches. The knee was brought to 30 degrees of flexion and the construct was stable. The wound was closed with a standard layered closure. Postoperative lateral knee radiograph displayed a CDI of 1.09 and an ISI of 1.16 (), which confirmed that patella alta had been corrected. At 18-month follow-up, the patient had a repeat MRI performed which demonstrated a CDI of 1.35 ().
Following the operation, a locked extension brace was applied for full-time use, and he began physical therapy two weeks postoperatively. The patient was compliant with his rehabilitation protocol and did not suffer any setbacks in the postoperative period. At his 1-year follow-up examination, the patient did not appear in acute distress, had no joint effusion, did not have patellar apprehension, demonstrated a nonantalgic gait, showed full active range of motion with no lag (), displayed a negative Clarke exam, and had 4/5 quadriceps strength. In addition, the patient had 5 mm of anterior translation bilaterally on KT-1000 arthrometry testing and 20.6 pounds of force on maximal muscle testing of leg extension on the right compared to 21.3 pounds on the left as measured by a handheld dynamometer. The patient reported a pain level of 2/10 with activity, which was a decrease from his preoperative pain level (4/10). He reported occasional use of Tylenol for pain. His PRO scores at 1-year follow-up can be found in . Despite a relatively benign physical exam () and subjective reporting of satisfaction with the revision procedure and his outcome, the patient reported moderate functional limitations. Permanent work-duty restrictions were subsequently outlined as a functional capacity examination, and patient reported outcomes did not permit return to his full occupational capacity.
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pmc-6087584-1
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We present the case of an 89-year-old male with a history of end stage renal disease on hemodialysis and localized melanoma of the chest status after excision 15 years ago who presented to the hospital complaining of fatigue, rigors, and fever one day after his first ever hemodialysis session. Complete blood count revealed hemoglobin of 7.7 g/dL, white blood cell count of 16.2 bil/L, and platelet count of 195 bil/L. In the emergency department, the patient was febrile measuring 38.2 degrees Celsius, with a blood pressure of 146/85 mmHg, heart rate of 85 beats/minute, and respiratory rate of 19. Chest X-ray showed a 5-centimeter mass in the right upper lobe of the lung. Blood cultures grew Methicillin-resistant Staphylococcus aureus (MRSA), attributed to the recent tunneled central venous catheter as the source of infection. Five days since the date of hospital admission, the patient's hemoglobin acutely decreased to 5.1 g/dL. A fecal occult blood was positive from digital rectal exam. An esophagogastroduodenoscopy showed a 50-millimeter noncircumferential bleeding mass in the gastric cardia, with raised borders and a central, protruding, ulcerated center from which biopsies were taken ().
Computed Tomography (CT) scan of the abdomen and pelvis (with oral contrast only) showed a heterogeneous density involving the dome of the liver concerning metastatic disease. The biopsy report revealed a high grade malignant neoplasm with immunohistochemistry positive for cytokeratin CAM 5.2, polytypic cytokeratin, and 4 different melanoma markers (SOX-10, S-100, MART-1, and HMB-45) (Figures and ). Considering the patient's history of melanoma and biopsy findings, the diagnosis of metastatic malignant melanoma to the stomach was made. Due to his functional status and suspicion for diffuse metastatic disease to the liver and lung, he was not considered a candidate for surgical resection. The patient was started on treatment with nivolumab. Unfortunately, the patient decompensated 2 months after his diagnosis was made and was enrolled in hospice care.
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pmc-6087602-1
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A 50-year-old Japanese man visited a nearby orthopedic clinic complaining of persistent pain during ambulation and solid mass in his lateral retromalleolar portion, which had gradually grown since 5 years prior to visiting our hospital. Conservative treatment, including immobilization using an ankle brace and administration of NSAIDs, failed to reduce his persistent pain, and the patient was then referred to our hospital for surgical treatment. He had a medical history of severe left ankle sprain 35 years prior, which was treated with only bandage application. He was also diagnosed with rheumatoid arthritis 5 years prior at a nearby hospital, which was not treated with antirheumatic drugs. On the first visit to our hospital, his blood test showed the following results: CRP, 0.67 mg/L; RF, 394 IU/mL; MMP-3, 138 ng/mL; and anti-CCP, 363 U/mL. Physical examination revealed a solid mass sized 1 × 5 cm over the retromalleolar portion of the left ankle along the course of the peroneal tendons (). He had tenderness and slight swelling on the left retromalleolar space, but no local heat or redness. He had no joint swelling and pain other than the swelling on the left lateral retromalleolar area. Pain was elicited by active plantar flexion of the ankle and eversion of the foot. The range of motion of his left ankle was 5° of dorsiflexion and 35° of plantar flexion, which was limited compared with 10° of dorsiflexion and 45° of plantar flexion of his right ankle with his knees flexed. He had no instability in his ankle joint on the manual anterior drawer test.
X-ray and CT showed a 1 × 5 cm elliptical opacification along the course of the peroneal tendon from the level of the ankle joint at its distal end (). Sagittal T1- and T2-weighted MR images showed an elliptical mass of a low intensity partially with high intensity with no contrast effect. Axial T1-weighted MR images showed a low-intensity mass in the peroneal tendon sheath, which seemed to compress both the peroneal brevis and longus tendons (). Ultrasonographic image showed an elliptical mass with an echoic shadow on the affected side of the peroneal tendon sheath (). We assumed that the mechanism of the present symptom was due to HO or calcinosis in the peroneal tendon sheath. Because of intractable pain and inability to walk, he hoped for a surgical treatment.
Surgery was performed through the retromalleolar curvilinear approach with a longitudinal 9 cm skin incision. The superior peroneal retinaculum and peroneal tendon sheath were incised. Longitudinal incision of the peroneus longus tendon revealed that the mass existed inside the peroneus longus tendon and its distal end was connected to the peroneus longus tendon (). We excised the solid mass and a part of the peroneus longus tendon because no viable tendon remained in this part. The peroneus brevis tendon was intact, without any pathologic features. Because of the large defect after retraction of the peroneus longus tendon, an end-to-side transfer of the peroneus longus tendon to the peroneus brevis tendon was performed distally and proximally using monocryl sutures. We found a peroneus quartus muscle running along the peroneus longus tendon and subsequently excised it (). The wound was irrigated and closed, and a sterile dressing was applied.
Microscopic examination revealed a lamellar bone formation with mixed tissue of fat and necrotic muscle. Calcification and cartilage metaplasia existed in the transitional zone between the ossification and the remaining tendon, that is, endochondral ossification (). The pathological findings did not indicate intramembranous ossification. The histological diagnosis was HO of the peroneus longus tendon. A below-knee cast was applied on the patient in the operating room; instructions to maintain nonweight bearing for 3 weeks were also provided. After 3 weeks, full-weight bearing was allowed. The cast was removed 6 weeks postoperatively. The postoperative course was uneventful, and the patient returned to normal activities without any kind of functional disability. He started taking antirheumatic drugs at a nearby hospital 2 months after the surgery. We compared the outcomes of the surgery, using an objective standard rating system, the Japanese Society for Surgery of the Foot (JSSF) scale [, ], and the Self-Administered Foot Evaluation Questionnaire (SAFE-Q) []. The SAFE-Q is an ankle-specific subjective evaluation method consisting of 6 subcategories (i.e., pain and pain-related, physical functioning and daily living, social functioning, shoe-related, general health and well-being, and sports activity [optional]). The preoperative JSSF scale of 54 points (maximum score, 100 points) significantly improved to 100 points after 1 year. Compared to the preoperative condition, all subscale scores in the SAFE-Q improved after 1 year: pain and pain-related, 21 to 100 points; physical functioning and daily living, 50 to 86 points; social functioning, 21 to 88 points; shoe-related 100 to 100 points; and general health and well-being, 25 to 85 points.
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pmc-6088193-1
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A 62 year old right-handed female suffered right middle cerebral artery ischemic stroke 6 years ago with a residual left spastic hemiplegia. She was able to ambulate without any assistive device at a moderate walking speed. She presented with a mild circumductory gait. Lateral trunk flexion to the left side and her left hip hiking were prominent and constant during walking. According to its spread origin of latissimus dorsi muscle from inferior 3–4 ribs, low thoracic spine, lumbar spine and iliac crest, and its insertion to the intertubercular groove of the humerus, a spastic latissimus dorsi muscle was viewed to be responsible for this patient's abnormal posture during walking, including pelvic vertical elevation in the coronal plane, trunk lateral flexion, shoulder adduction, and internal rotation (Figure ). A total of 150 units of onabotulinumtoxin A were injected into this muscle under ultrasound imaging guidance. Trunk lateral flexion and pelvic elevation were much improved at 6 weeks after injection. As shown on Figure , pelvic vertical elevation was decreased from 19 to 9° after injection.
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pmc-6088193-2
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A 27 year old right handed female had a history of stroke after a traumatic brain injury 20 years ago which resulted in right spastic hemiplegia. She received botulinum toxin injections several times in the first 3 years after the accident. At a seated or supine position, she only had very mild muscle weakness in the right upper and lower extremities with minimum to negligible spasticity. The chief complaint was that her right toes were hitting the left toes during the mid-swing phase, i.e., problematic right hip internal rotation and adduction secondary to dynamic tone (Figure ). According to possible pathomechanics, dynamic spasticity in right anterior gluteus medius and TFL muscles could cause excessive anterior rotation of the pelvis in the transverse plane and hip internal rotation, while hip adductor spasticity contributes further to hip adduction. A total of 200 units of incobotulinumtoxin A were injected to these muscles under ultrasound imaging guidance (75 units to gluteus medius, 50 units to TFL, and 75 units to hip adductors). Improved walking posture in the follow up visit at 6 weeks after injection validated the pathomechanics analysis (Figure ).
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pmc-6088412-1
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A 33-year-old Thai woman presented with progressive back and arm pain for 2 weeks. Her body weight (BW) had increased by 10 kg over the preceding 2-year period, and she had also noticed dark striae on her abdominal wall. She complained of excessive acne on her face, but she still had normal menstrual cycles and no hirsutism. She had not visited a hospital about the symptoms before. Then, 2 weeks prior to admission, she had a non-severe falling accident, but she still had worsening back and right arm pain. She also had a history of occasional use of Chinese herbs and weight loss pills. On examination, a rounded face, truncal obesity, and wide purplish striae on her abdominal wall and right thigh were observed. Her blood pressure was 160/90 mmHg. CS was therefore suspected.
The provisional diagnosis of ECS was confirmed by a 24-hour urinary free cortisol (UFC) level of 529.4 μg/day, serum cortisol levels after 1 and 4 mg dexamethasone suppression of 26 and 25.7 mcg/dL, respectively, and a loss of physiologic diurnal variation. ACTH-independent CS was determined by an ACTH level of 3.21 pg/mL (Table ), and computed tomography of her upper abdomen showed a lipid-poor left adrenal adenoma (size, 2.8 cm) and a lipid-poor right adrenal adenoma (size, 1.1 cm; Fig. ). Due to the bilateral adrenal nodules, an atypical finding in adrenal CS, adrenal venous sampling (AVS) was performed to determine the potential side of the excess cortisol production. Using the reference method of William F. Young Jr et al., the AVS revealed a predominantly left-sided ratio of adrenal venous to peripheral plasma cortisol (ratio, 3.21), which was compatible with a left cortisol-producing adrenal adenoma and a right, nonfunctioning adrenal adenoma []. In addition to the investigations for CS, plain films of the lumbosacral spine were obtained to evaluate her back pain, and they revealed a compression fracture of the T5–T10 vertebrae. Moreover, we found several osteolytic lesions in her ribs and pelvic bone that are not normally discovered in CS (Fig. ). Metastatic cancers and MM were suspected.
Further diagnostic investigations were performed to identify the cause of the bone lesions and to determine if abnormal levels of ACTH were the source of the CS. A complete blood count showed hemoglobin (Hb) 10.2 g/dL, hematocrit 31.2%, white blood cells (WBCs) 10.95 × 109/L with neutrophil predominating, and a normal platelet count. A peripheral blood smear displayed normochromic normocytic red cells without Rouleaux formation. There was mildly elevated serum creatinine (1.01 mg/dL), and a normal level of serum calcium (9.8 mg/dL). Serum albumin was 4.3 g/L, while serum globulin was 2.5 g/L. A bone survey revealed generalized osteolytic lesions involving her skull, mandible, both clavicles, her right scapular, both sides of her ribs, her right humerus, and her pelvic bones. A serum protein electrophoresis found hypogammaglobulinemia without detecting monoclonal gammopathy by immunofixation. The serum free kappa light chain level was 3540 mg/L, while the serum free lambda light chain level was 6.7 mg/L, with a Kappa:Lambda ratio of 531:1. A bone marrow aspiration showed plasma cells and plasmablasts over 80% and a bone marrow biopsy demonstrated numerous CD138+ plasma cells with kappa light chain restriction (Fig. ). The serum β2-microglobulin was 2.98 mg/L. Kappa light chain MM, International Staging System stage I, was diagnosed.
Because our patient had hypercortisolism, the endogenous corticosteroid might have been suppressing plasma cell proliferation in the MM. She also had no critical conditions for emergency surgical treatment of the CS. MM was therefore set as the priority treatment to eliminate the clonal plasma cells. We decided to treat her with a bortezomib, cyclophosphamide, and dexamethasone (VCD) regimen consisting of: bortezomib 2 mg intravenously on days 1, 8, 15, and 22; cyclophosphamide 400 mg orally on days 1, 8, 15, and 22; and dexamethasone 40 mg orally on days 1, 8, 15, and 22. The treatment was recycled every 28 days, with a total of six cycles. While she was receiving this chemotherapy regimen, she did not develop hyperglycemia or dyslipidemia. After six cycles, she finally achieved a very good partial response, according to the International Myeloma Working Group (IMWG) response criteria, and a stem cell collection was made after this cycle. The protocol for stem cell mobilization consisted of 4000 mg of cyclophosphamide in normal saline (250 mL) administered intravenously for 4 hours on day 1, and filgrastim 600 mcg in 5% dextrose water (100 mL) administered intravenously for 15 minutes on each of days 2–10. The stem cells were collected on days 10–11 without any negative CS effect on the stem cell collection process; the CD34+ cell count was 4.4 × 106 cells/kg BW. An autologous stem cell transplant was performed in September 2014. There were no serious complications, and gained a deeper response in the form of a complete response after this intervention. Subsequently, 5 months after the stem cell transplant, a left laparoscopic adrenalectomy was successfully undertaken. The pathological report showed an adrenal cortical adenoma of size 2.0 × 2.4 × 0.6 cm (Fig. ). Her serum cortisol level returned to 1 mcg/dL after the operation; she received a steroid replacement, which was gradually discontinued over 9 months. Her blood pressure decreased slowly, and we successfully tapered off the anti-hypertensive drugs over 2 months. Her Cushingoid appearance gradually subsided. Eventually, she was in remission of both diseases after more than 30 months of treatment. Timeline of her symptoms, treatments, and outcome was provided in Additional file .
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pmc-6088461-1
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A 79-year-old female with past medical history of chronic lymphocytic leukemia (in remission) presented to New York-Presbyterian Queens with complaint of chest pain radiating to the back with associated nausea.
On examination, she was afebrile with blood pressure 161/78 mm Hg. She had mild epigastric tenderness to palpation with no guarding or rigidity. Initial laboratory values revealed a white blood cell count of 7.33 K/µL, hemoglobin of 13.3 g/dL, and normal electrolytes. Cardiac enzymes were negative, and liver function tests revealed aspartate transaminase 492 U/L, alanine transaminase 493 U/L, and alkaline phosphatase 353 U/L. Electrocardiography showed no acute ischemic changes.
Abdominal ultrasound revealed a dilated common bile duct (CBD; 1 cm) with slightly dilated gallbladder without stones. Magnetic resonance cholangiopancreatography confirmed CBD dilation without stone or definite stricture. However, subsequent endoscopic retrograde cholangiopancreatography (ERCP) showed a biliary stricture at the hepatic duct bifurcation. A sphincterotomy was performed, biopsies were obtained, and 2 Advanix™ biliary stents (Boston Scientific Corporation, Natick, MA) were placed. One 7-Fr, 12-cm plastic stent was placed into the right system, and a second 10-Fr, 15-cm plastic stent went into the left system, past the stricture into the right and left hepatic ducts and extending distally into the CBD with adequate bile flow. The patient tolerated the procedure well without complications.
After discharge, she immediately began to experience nausea, vomiting, and abdominal pain. On examination, she was markedly tender to palpation in the left upper quadrant. Abdominal X-ray was negative for free air. Computed tomography scan revealed migration of the 7-Fr, 12-cm stent through the duodenal wall. An upper endoscopy was performed, confirming penetration of the stent through the lateral portion of the second part of the duodenum (). The stent was then extracted using a Raptor™ grasping device (US Endoscopy, Mentor, OH) revealing a 0.5 × 0.5 cm transmural defect (). This was closed using four 11-mm TTS endoclips (DuraClip, ConMed Corporation, Utica, NY; ).
She tolerated the procedure well, was started on levofloxacin and metronidazole, and was monitored in the hospital. She remained clinically stable and was started on clear liquid diet on post-procedure day 2. She was discharged on post-procedure day 4 and had no complications in follow-up 2 weeks after discharge.
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pmc-6088758-1
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A 43-years old woman with Marfan’s syndrome was diagnosed with progressive aortic root enlargement to 5.4 cm, aortic valve regurgitation and tricuspid valve regurgitation. Elective repair was highly recommended. Her clinical examination along with the preoperative RX and CT scan revealed a severe pectus excavatum. Her sternum was angled and was only 1.5 cm close to the column vertebrae at the point of apex. This resulted in a total cardiac dislocation to the left ().
Due to the severe sternal deformity, the affinity of the right atrium and the vena cava to the sternum, we enacted the operation with the exposure of the right femoral artery and vein to assure a quick access to cardiopulmonary bypass (CBP) if it was required. After the sternotomy it was very difficult to continue with sternal retractors, even if we tried to use a couple of them in upper and lower sternum. Alternatively, we used an Osler retractor at the left hemisternum to expose ascending aorta and right atrium. A partial CBP was then introduced with the ascending aorta and right atrium cannulation that gradually resulted in a total one with the cannulation of superior and inferior vena cava. Afterwards, a standard valve and aortic root replacement with a re-implantation technique was performed with the use of a 21 cm composite graft and Tricuspid valve annuloplasty with a 32mm physio tricuspid annular ring.
Once the operation was performed and the hemostatic procedures achieved adequate bleeding control, a post-repair transesophageal echocardiography demonstrated normal ventricular function with overall good composite graft function and no tricuspid insufficiency. Then a second Osler retractor was applied to the right hemi-sternum to minimize the compression of the cardiac structure and to achieve a safer disconnection from the CPB (). This technique achieved to minimize the danger of excessive chest compression intraoperatively and revealed as an additional aid for Ravitch procedure. After the CPB termination and protamine administration, we carefully checked all surgical sites to ensure that there was no bleeding. Our next step was to perform a Ravitch II procedure. In brief, bilateral pectoralis muscle flaps were raised and the costal cartilages from rid 3 to 8 were resected, taking care to retain the perichondrial sheaths.
Finally, the sternum closure was performed with steel wires and reduction was maintained with two substernal bars. No significant bleeding occurred postoperatively. The patient remained heamodynamic stable during acute ICU care and was extubated 10 hours later. Sub-pectoral drains, as also sub-sternal and hemi-thorax drains were removed on postoperative day three. Her postoperative course was complicated by a trifascicular block that required the placement of a permanent pacemaker on day 9, with a total hospitalization of 11 days. A follow up CT scan demonstrated satisfactory reduction in the pectus deformity with a stable aortic and tricuspid repair. One year later she is in excellent condition and postoperative tests are normal ().
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pmc-6088764-1
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A 49-year-old female presented to the emergency room with headache, fatigue and decreased stamina. Her past medical history was significant for diabetes, hypertension and prior stroke. Her work-up included a trans-thoracic and trans-esophageal echocardiogram, magnetic resonance imaging (MRI), laboratory evaluation, and computed tomography of her aorta (CTA). A mass was found in the ascending aorta, consistent with an aortic mural thrombus, measuring 1.6 × 1.0 × 1.9 cm with some degree of mobility ().
The patient did not have a history of rheumatic disease or underlying bleeding or clotting disorders. Hypercoagulability testing, including antiphospholipid antibody, beta 2 glycoprotein, factor V Leiden, lupus anticoagulant, and prothrombin gene mutation were all negative. After 48 hours of inpatient observation, with resolution of symptoms, the patient requested to be discharged. Following a thorough discussion of the indications, risks and benefits of surgical intervention, the patient opted for trial of medical therapy. She was discharged on anticoagulation with apixaban, chosen for its immediate therapeutic effect given the patient’s wishes to be discharged, with plans for repeat radiographical surveillance and close clinical monitoring, including surgical intervention for symptoms, increase in size of the thrombus, any embolic phenomenon, aortic valvular involvement, or coronary arterial compromise.
The patient was followed for 15 months after her initial presentation with no further symptoms. Serial surveillance CTAs showing resolution of her aortic thrombus are shown (). There was no discernable associated pathology leading to this thrombus on multiple phases of contrast and the patient was taken off anticoagulation after her latest visit.
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pmc-6089125-1
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A 53-year-old man, who had non-exertional chest pain with positive exercise stress
test, was hospitalized by the cardiology department. Total occlusion of the right
coronary artery (RCA) was detected in the cath-lab. After performing percutaneous
transluminal coronary angioplasty (PTCA), long segment drug-eluting stents (DES) and
bare-metal stent (BMS) were placed. After the procedure he became hypotensive. In
order to exclude pericardial effusion, transthoracic echocardiography was performed.
In the left atrium (LA), a 6,5x4 cm mass was detected with LA dilatation and
estimated systolic pulmonary artery pressure over tricuspid regurgitant jet was 43
mmHg. Then, he was sent to our clinic for surgery with possible diagnosis of LA
myxoma. His left ventricular ejection fraction (LVEF) was 60%. We decided to perform
surgical excision of the mass. Following median sternotomy, we reached the LA via
transseptal approach. We saw a giant mass in the LA, nearby the mitral valve. The
mitral valve was intact without any deformation. We excised the mass, which invaded
the myocardium towards the posterior wall of the LA. The LA was hypertrophic in
nature, so all the mass with LA was resected. The mass consisted of central necrotic
parts which did not resemble to atrial myxoma. Intraoperative view of the mass is
shown in . The defective posterior
atrial wall was then repaired by autologous pericardium, fixed by 0.625%
glutaraldehyde solution ().
Intraoperative frozen pathology specimen was reported as benign tissue, rich in
fibrosis, but not myxoma. After closing the septum and the right atriotomy, RCA
bypass over PD segment was performed using saphenous vein graft. The aortic
occlusion time was 141 minutes. Since the patient had first-degree heart blockage, a
temporary pacemaker lead was inserted and the operation was completed in routine
manner. Postoperative period was uneventful. He had sinus rhythm after 24 hours,
stayed in intensive care unit (ICU) for 2 days, and discharged from the hospital at
postoperative 8th day.
Histopathologic examination of the specimen revealed necrotic areas, lymphocytic and
histiocytic infiltration and sporadic eosinophilia in the striated muscle fibers.
There were also multinuclear giant cells. These giant cells showed positive
expression with CD45 and CD68, with the final diagnosis of GCM (-). After the
accurate diagnosis of GCM, he was followed-up by echocardiography in the
10th day, 1st month, and every 3 months thereafter. He
clinically improved after the operation, and still had LVEF of 55% at the end of 24
months follow-ups. He took only acetylsalicylic acid (100 mg/day), ramipril (5
mg/day) and bisoprolol (5 mg/day) treatment after the operation without immune
suppression therapy.
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pmc-6089126-1
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A 53-year-old male patient was referred to our emergency department due to a
sudden-onset chest pain. In his medical history he had coronary artery disease for
five years and a CABG surgery was recommended for him in another healthcare centre
one year before. The stenosis in the right internal carotid artery was observed in
the preoperative carotid Doppler ultrasonography. The patient was then diagnosed
with Moyamoya disease based on subsequent cranial computed tomography and cerebral
digital subtraction angiographic (DSA) images (). In the DSA scans obtained from the previous institution, the stenosis
of the right internal carotid artery extended to the distal ophthalmic branch was
observed. Due to the low blood flow in the right middle cerebral artery (MCA), CABG
was considered risky for causing cerebral hypoperfusion so their treatment plan was
revised to the placement of a coronary stent into the main circumflex artery.
During his evaluation in our center, the ejection fraction was calculated as 48% and
the basal, mid-basal posterior and anterior segments of the interventricular septum
were hypokinetic. Emergent coronary angiography showed 100% stenosis in the left
anterior descending artery and the stent previously placed was patent in the main
circumflex artery, while 50% stenosis was observed in the second obtuse marginal
artery, followed by 100% stenosis in the main circumflex artery (). We decided to perform an elective CABG
surgery as a treatment option for the patient, according to the results of the
previous examinations.Preoperative written informed consent was obtained from the
patient for open heart surgery.
Under general anesthesia, the patient received continuous esmolol infusion (50
µg/kg/min). The surgery was performed with off-pump beating heart technique
and the heart was stabilized using Octopus(r) (Medtronic Inc, Minneapolis, USA)
tissue stabilizers. The left internal mammary graft was anastomosed to the left
anterior descending artery and a saphenous vein graft was anastomosed sequentially
to the second obtuse marginal artery without any intraoperative complication. The
patient was transferred to the cardiovascular intensive care unit under positive
inotropic support (5 µg/kg/min Dopamine). The patient was, then, successfully
extubated and discharged on the sixth day after the surgery.
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pmc-6089133-1
|
LSA, a 34-year-old male police officer, came to the office to report that he had been
discharged 16 days after being treated for chest injury by firearm. He reported that
the projectiles (two) were still in his body. He reported having been treated in the
public hospital emergency room with two projectiles orifices entries in the lateral
side (subaxillary), on the average height of the right hemithorax, provoked during
the approach of robbers in an attempt of assault. According to a copy of the
admission record, he was slightly discolored, peripheral perfusion maintained,
tachycardic, and with decreased vesicular murmur on the right chest. Initial
stabilization support measures were established. Chest X-rays showed one projectile
in anterior cardiac topography, near the apex of the heart and another in the right
rectus abdominis, near the thoracoabdominal transition. In addition, it showed
moderate right pleural effusion. Computed tomography of the chest ratified the
radiographic findings and showed discrete pericardial effusion. The right hemithorax
was drained, and then the patient was transferred to a private hospital, where the
radiographs and tomography of the chest were repeated, besides transthoracic and
transesophageal echocardiograms. None of them report the presence of a projectile
within the heart. All reports referred to the presence of a projectile in topography
around the heart - nearby. During the hospitalization, the patient had a right
pulmonary embolism, and was anticoagulated with rivaroxaban, being discharged after
two weeks. Upon analyzing the case in detail, we could not rule out the possibility
that one of the projectiles was lodged inside the heart. New imaging exams were
requested from the outpatient in another institution. However, the reports remained
imprecise as to the exact location of the projectile in cardiac topography. After a
clinical meeting for discussion of all the exams, a new echocardiogram was elected,
which, this time, showed the projectile inside the right ventricle (). The patient was promptly hospitalized
and underwent surgical intervention. Access to the thoracic cavity was made by
median sternotomy. No hematoma was observed in the pericardial fat, and after the
pericardiotomy, the orifice of entry of the projectile in the pericardial cavity was
not found. Only discrete sero-sanguineous effusion was found. Extracorporeal
circulation was established by drainage of both vena cava and infusion by the
ascending aorta. A tactile inspection of the diaphragmatic wall of the right
ventricle was performed under total aortic clamping and cardioplegic arrest with
Custodiol(r). This maneuver allowed us to feel the presence of the projectile near
the apex of this ventricle. Unbelievably, the diaphragmatic wall of the right
ventricle did not show the projectile's inlet orifice. A ventriculotomy of
approximately 3 cm was performed on the diaphragmatic face of the right ventricle
followed by removal of the projectile (). Right ventricle closure was performed with separate "U" points (3.0
polypropylene) anchored in two Teflon(r) bars. Extracorporeal circulation was
discontinued as soon as hemodynamic conditions allowed. Through the median incision
the other projectile, which had been lodged in the right rectus abdominis, was
located and removed. Surgery was completed with the revision of hemostasis,
mediastinal drainage, and closure of the thorax. Trans and postoperative
echocardiograms did not show the presence of interventricular communication. The
patient presented a good evolution when he was discharged, thus returning to his
normal healthy life.
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pmc-6089475-1
|
A 36-year-old female presented to the oral diagnosis clinic at Ain Shams University's dental school for the evaluation of a swelling in the lower right quadrant. The patient reported the swelling to be of 10 years' duration with a progressive course during the last year. The patient reported some pain and difficulty during mastication. There was a history of surgical biopsy several years ago, but a biopsy report was not available.
An extraoral examination revealed right facial asymmetry with normal overlying skin (Figure ). Submandibular lymph nodes were palpable on both sides but were not tender. An intraoral examination showed a hard bony swelling related to the right mandibular premolar-molar region. On clinical examination, the mucosa appeared normal, with no evidence of ulceration or bleeding.
A massive buccolingual expansion of the lesion was noted, possibly crossing the midline. The lesion was tender. There was no associated tooth mobility. However, drifting and displacement were noticed (Figure ). The related teeth were also vital. Oral hygiene was poor. Medical history was unremarkable, with vital signs within normal range.
The panoramic radiograph (OP100, Instrumentarium Imaging, France, at kVp 66, 13 mA) showed an expansile multilocular radiolucent lesion involving the mandible. The lesion extended from the lower-right second molar to the contralateral second premolar crossing the midline. The borders were well demarcated and sclerotic. Superiorly, the lesion extended to the alveolar crest, causing expansion. Inferiorly, it spread to the inferior border, causing displacement. Extreme thinning and bowing of the inferior cortex of the mandible was evident but contained an intact cortex. The inferior alveolar canal on the right side appeared to be displaced inferiorly. The divergence of the roots of the related teeth showed a loss of the lamina dura, as well as evidence of slight root resorption in the lower right canine. Multiple radiopaque masses with density compared to that of bone were seen within the lesion (Figure ).
Advanced imaging cone beam computed tomography (CBCT), using Scanora 3D (Soredex, Wisconsin, USA; at 85 kVp, 15 mA, and 337mGy) with 3.5 mm slice thickness, revealed a lesion measuring 45.9 mm x71 mm. The CBCT images showed massive buccolingual expansion with extreme thinning of the cortical plates. The internal structure showed a multilocular appearance with fine septa. Some slices showed fine granular radiopaque foci, while others showed dense radiopaque masses. Although evidence of expansion was noted all over the lesion, the cortical plates appeared to be intact with no perforation (Figure ).
The differential diagnosis includes other lesions, such as fibrous dysplasia, calcifying cystic odontogenic tumor (Gorlin cyst), calcifying epithelial odontogenic tumor, central giant cell granuloma, and adenomatoid odontogenic tumor. The well-defined border of the central cemento-ossifying fibroma helps to differentiate it from the aggressive mixed lesions as chondrosarcoma, osteosarcoma and metastatic malignant lesions [,].
Aspiration biopsy was negative. An open bone biopsy was performed via the intraoral approach, and multiple soft tissue and bone fragments were removed and submitted in 10% formalin for histological evaluation.
The microscopic examination showed a highly cellular fibrovascular lesion with spindle fibroblasts forming a whorled pattern. Irregular bone trabeculae with osteoblastic rimming, dystrophic calcifications, and areas of cementum-like tissue were also noted (Figure ). No signs of malignancy were observed. The histopathological features suggest ossifying fibroma. The patient was referred to the maxillofacial surgery department for lesion removal and jaw reconstruction.
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pmc-6089477-1
|
A 72-year-old left-handed man with past medical history of atrial fibrillation, congestive heart failure and mitral valve repair, but no history of malignancy, presented to the Emergency Department in 2016 for evaluation of right arm pain. The patient heard a crack in his arm while dressing and subsequently his arm pain worsened. His pain, when he was evaluated at the Emergency Department, was subjectively rated as 5/10, worse with activity and palpation and relieved with rest. No edema or erythema was noted. There was no axillary or cervical adenopathy. His pulses were normal and his sensation to light touch was intact. Radiographs obtained in the Emergency Department revealed a minimally angulated proximal right humeral fracture at the superior aspect of a linear sclerotic lesion in the proximal humeral diaphysis. The linear sclerotic lesion was thought to be likely a bone infarct (Figure and Figure ). No definite soft tissue lesion was noted. His fracture was treated conservatively with splinting.
However, his pain progressively worsened, so repeat radiographs were obtained a couple of weeks later to assess healing of the fracture at his fracture follow-up clinic visit (Figure ). These subsequent radiographs demonstrated the development of a lytic lesion with surrounding periosteal reaction at the fracture site. No osteoid production/mineralization was appreciated. Magnetic resonance imaging (MRI) showed a T1-isointense (Figure ), T2 heterogeneously hyperintense (Figure ), heterogeneously enhancing lesion (Figure ) originating from the intramedullary cavity with osseous destruction of the humerus and a soft tissue component that measured up to 15 cm in superior-inferior dimension. A serpiginous, linear area of low T1 and low T2 signal consistent with a bone infarct was noted at the lesion, and this area of infarct extended more distally in the humeral diaphysis. This bone infarct corresponded to the linear area of sclerosis seen in the humeral diaphysis on prior radiographs.
Ultrasound-guided core needle biopsy of the soft tissue component of the lesion was performed. Histological analysis of the biopsy showed neoplastic spindle and epithelioid cells with focal osteoid formation (Figure ). A few giant cells were also noted. There were frequent mitotic figures (4 per high powered field). Areas of hemorrhagic necrosis were present. No chondroid material was noted. Immunohistochemical stains with adequate controls were performed. The epithelioid cells were negative for TTF-1, S100, AE1/3, PSA, HMB45, ERG, CD31, CAIX, HepPar, PAX8, and pancytokeratin. INI1 was retained. This immunoprofile combined with the histological findings supported a diagnosis of a tumor of bone origin and were most consistent with an osteogenic sarcoma (Figure ). Staging 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) showed localized disease (Figure ).
He was treated with reduced dose cisplatin/adriamycin given his age. He had a poor response to chemotherapy with enlargement of the mass through chemotherapy with the development of circumferential disease around the humerus. He underwent a right forequarter amputation. He developed lung metastases and an L4 spinal metastasis with pathological fracture two months following surgery. He received palliative radiation therapy to the L4 vertebra and died seven months after initial presentation.
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pmc-6089479-1
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A 72-year-old African American male presented to the emergency department from the outpatient oncology office with a week of mild abdominal pain, which was not life-threatening. His past medical history was notable for metastatic pancreatic adenocarcinoma, which had been diagnosed one year earlier, from endoscopic retrograde cholangiopancreatography (ERCP) brushings when he presented with obstructive jaundice and required biliary and duodenal stent placement at the time. He had undergone a routine CT scan of his abdomen and pelvis for disease progression two days prior to admission, which revealed a massive pneumoperitoneum (Figures , ). The ominous imaging prompted his admission to the hospital. On presentation to the emergency department, he was in no distress with a blood pressure of 126/85 mm Hg, pulse rate of 93 beats per minute, respiration rate of 18 breaths per minute, and a temperature of 36.9° C. Physical examination was significant for marked abdominal distension with a benign non-peritonitic exam and mild tenderness on palpation. Laboratory tests were unremarkable, except for a white blood cell count of 10.5 x 103/uL with neutrophil predominance (83%). A repeat CT scan showed patent biliary and duodenal stents without a definite source of perforation; however, three suspicious locations were noted by the radiologist containing small foci of free air adjacent to the bowel.
Given the initial concern for perforated viscus, the patient was started on broad-spectrum antibiotics and antifungals. He was managed conservatively with intravenous hydration and strict bowel rest. He remained hemodynamically stable and non-septic throughout his hospitalization and continued to have flatus and intact gastrointestinal function. General surgery recommended no acute surgical intervention. In a few days, he was started on a clear liquid diet which he tolerated well. His abdominal distension gradually improved without any surgical intervention, and he was discharged home a week after admission. Two months later, he underwent a repeat CT scan that showed resolution of the pneumoperitoneum (Figure ).
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pmc-6089482-1
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An ill-appearing 19-year-old male with the one-year history of asthma presented to the emergency room with non-specific symptoms including fatigue, dyspnea, numbness in the right leg, nausea, vomiting, and dizziness. Two months prior to presentation, he had a sinus surgery and within few days after this surgery, he developed cough and dyspnea, so he was admitted to outside hospital for possible pneumonia. He was treated with cefuroxime, Tamiflu, and oral prednisone. He improved momentarily with steroids. Two weeks later, he returned to the outside hospital complaining of right foot plantar numbness and dyspnea, he was discharged home on Levaquin as they thought he may have some residual sinus disease left. One week later he was seen by a pulmonologist at outside hospital and they noticed that one of the cultures grew staph, hence started on Bactrim. He took Bactrim for three days and his mother noticed that he developed some mental status changes, hence Bactrim was stopped. After this, no more symptom of mental status change was noticed. Over the next few weeks, the patient noticed tachypalpitations, continued to have fatigue, shortness of air, and fatigue so the family decided to come to our hospital's emergency department for further workup. While in the emergency room, he was found to be in atrial fibrillation with the rapid ventricular response and elevated troponins. The patient spontaneously converted into sinus rhythm within 10 minutes. His vital signs were stable except for tachycardia with a heart rate of around 100 beats per minute. Physical examination was unremarkable with a normal sensation on right leg and foot. He was admitted to cardiac intensive care unit for further workup due to elevated troponin.
Salient laboratory values and electrocardiogram
The patient’s initial complete blood count was remarkable for white blood cell of 28,800/ul with eosinophil count of 12,960/ul (45%) in spite of the use of low-dose oral corticosteroids for a few days prior to admission. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were both elevated at 35 and 4.14, respectively. His admission troponin was 16.28. His initial electrocardiogram (ECG) showed atrial fibrillation with a heart rate of 161 beats per minute, non-diagnostic Q waves in the inferior leads, T-wave inversions in the inferior leads and no significant ST segment changes noted (Figure ). His repeat ECG 10 minutes later when he converted to sinus rhythm showed sinus tachycardia with a heart rate of 100 beats per minute, Q and T changes as noted earlier, as well and no significant ST segment changes noted (Figure ). Other labs, including TSH, UDS, BNP, lactate, and renal function, were unremarkable. Rheumatological workup including anti-nuclear antibody (ANA), perinuclear antineutrophil cytoplasmic antibody (p-ANCA), cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA), rheumatoid factor, myeloperoxidase (MPO) antibody, serine protease antibody 3, and anti-cyclic citrullinated peptide (anti-CCP) IgG was inconclusive. However, the patient’s IgE and IgG were both markedly elevated. Several infectious causes, such as histoplasma, coccidioides, strongyloides, cytomegalovirus (CMV), human immunodeficiency virus (HIV), tuberculosis (TB), Epstein-Barr virus (EBV), hepatitis B, and hepatitis C, were explored and all were negative.
Imaging
Transthoracic echocardiogram revealed an ejection of 55% with some apical hypokinesis. The transesophageal echocardiogram showed no evidence of endocarditis, thrombus, shunt, or atherosclerosis. Computed tomography angiography (CTA) of the chest with and without contrast showed moderate mediastinal and bilateral hilar adenopathy in addition to bilateral axillary lymphadenopathy, bilateral peribronchial thickening, and patchy ground-glass opacities most predominantly in the posterior lower lobes. There was no evidence of pulmonary embolism (Figure ). Cardiac magnetic resonance (CMR) showed several areas of delayed enhancement within the left ventricular myocardium and decreased perfusion in the mid to apical septal and inferior segments throughout the apex. It also revealed a small pericardial effusion and minimal hypokinesis of the lateral apical wall (Figures -). Due to the abnormal myocardial enhancement, a CT of the heart with coronary CTA was ordered which showed normal coronary artery anatomy with no evidence of stenosis, calcified plaque, or soft plaque (Videos -). Due to his reported neurologic symptoms, CT of the head without contrast was ordered and showed two areas of low-attenuation within right frontal white matter. MRI of the head was subsequently performed which showed many small bilateral punctate infarcts throughout cerebrum and a few additional ones in the cerebellum.
Biopsies
A bone marrow biopsy showed normocellular bone marrow for age and no concern for dysplasia; however, both the bone marrow biopsy and peripheral blood smear showed marked eosinophilia with leukocytosis. Several transbronchial cryobiopsies were taken from the left lower, upper lobes and lingula of the lung which showed patchy areas of eosinophilic venulitis with dense eosinophilic infiltrates involving many of the small venules. This process was happening in the background of chronic bronchiolitis with abundant eosinophils within small airways, smooth muscle hypertrophy, and goblet cell metaplasia (which suggests asthma). All these findings taken into consideration together suggested EGPA (Figure ).
The patient was initially started on 1000 mg of intravenous methylprednisolone for three days and then 1 mg/kg/day of oral prednisone for several months with a gradual taper. He was also started on cyclophosphamide for three to six months. Additionally, due to the patient’s young age, arrangements for sperm preservation were made prior to starting cyclophosphamide. The patient responded well to the treatment and at his one-month rheumatology follow-up, the patient continued to improve. His troponin-I reduced to 0.08 at one month visit.
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pmc-6089485-1
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A 40-year-old male of Caucasian race presented with chest pain propagating to the left arm and the back and shortness of breath. These symptoms started on the day prior to the admission and were preceded by high-grade fever. The vitals were normal. Cardiac examination was normal. The electrocardiogram showed diffuse repolarisation changes. Transthoracic echocardiography revealed impaired systolic function of the left ventricle with ejection fraction 48%. Cardiac enzymes - creatine phosphokinase (CPK), creatine phosphokinase myocardial band fraction (CPK-MB), and troponin T were all markedly elevated. The blood lipid tests were within reference ranges. The initial differential diagnosis included acute myocardial infarction, aortic dissection, and myocarditis. CT of the chest with contrast matter showed no signs of aortic dissection. A peculiar finding was noted in the right lung and was recognized as an azygos vein passing through the upper lobe and separating an azygos lobe with its mesoazygos (Figures -).
The patient was evaluated through coronary angiography which did not show any evidence of coronary artery disease or myocardial infarction. Finally, microbiological tests were conducted and revealed Epstein-Barr virus (EBV) infection, which was discussed as the cause of acute myocarditis. The patient was treated accordingly and returned to normal activity within a few days.
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pmc-6089488-1
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The first case was a 58-year-old male with poorly controlled type 2 diabetes mellitus and hyperlipidemia who experienced a six-fold increase in his serum alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels from baseline. The patient had been taking pravastatin and ezetimibe for a few years for hyperlipidemia. Sitagliptin was recently added to the patient's medical regimen for better control of his diabetes. After initiating sitagliptin, the patient's ALT and AST increased gradually over a period of six months. Work up was done to find the possible cause of the abnormal liver function tests (LFTs). Hepatitis serologies were negative. An abdominal sonogram was also negative for gallstones. In the absence of any pathological cause, a diagnosis of drug-induced liver injury was made. All medications were discontinued and LFTs were done on regular follow-up visits. After discontinuing sitagliptin, pravastatin, and ezetimibe, the patient's ALT and AST returned to baseline levels. Resumption of pravastatin and ezetimibe was not associated with the elevation in ALT and AST levels. When we rechallenged the patient with sitagliptin, we observed a five-fold increase in the levels of serum ALT and AST from the baseline that became normal after discontinuation of sitagliptin. The second case was a 44-year-old female with type 2 diabetes who experienced a more than ten-fold elevation in ALT and AST levels after six months of sitagliptin therapy. Further workup revealed a negative hepatitis B-surface antigen with a normal liver sonogram. The patient's ALT and AST levels returned to normal after discontinuing sitagliptin, pioglitazone, and rosuvastatin. Resumption of pioglitazone and rosuvastatin was not associated with elevation in ALT and AST levels. The patient refused the rechallenge with sitagliptin.
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pmc-6089489-1
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A 50-year-old female with no significant past medical history presented with a one-week history of fevers, chills, generalized body aches, and dark brown urine. She also complained of fatigue, vomiting, and diarrhea for two days prior to admission. She had no known history of liver, kidney, or muscle disorders. She denied any recent history of trauma, immobility (travel, surgery), seizures, new medications, or drug abuse. On presentation, she appeared in moderate distress. Her vital signs were as follows: BP 127/58 mmHg, heart rate (HR) 126/min, respiratory rate (RR) 28/min, maximum temperature (Tmax) 39.3 C, and oxygen saturation (O2 sat) 93% on room air (RA).
The physical examination demonstrated signs of dehydration, clear breath sounds, and mild generalized muscle tenderness on palpation.
The initial laboratory evaluation shows a hemoglobin of 11.3 g/dl. Elevated creatine kinase (CK) had a peak of 28,216 u/L. Rapid influenza antigen testing was positive for influenza A virus and negative for influenza B. Urinalysis showed dark brown urine, specific gravity (sp.gr.) 1.005, large occult blood, elevated protein - 30, red blood cells (RBC) < 1, no casts. Urine toxicology was negative for salicylates, acetaminophen, cocaine, marijuana, benzodiazepines, and alcohol. The rest of the labs were unremarkable (Table ).
The differential diagnosis of rhabdomyolysis can be divided into two categories: traumatic or nontraumatic. The traumatic causes of rhabdomyolysis can be due to motor vehicle accidents, prolonged immobilization, seizures, and compartment syndrome. Nontraumatic causes can be due to hyperthermia, infections, drugs, toxins, medications, metabolic myopathy, or electrolyte abnormalities. In the case of our patient, she didn’t have any trauma before her coming to the hospital and she tested negative for drugs, toxins, and electrolyte derangements. She was not on any medications that could cause rhabdomyolysis. The only possible explanation for her rhabdomyolysis was due to infections, and she tested positive for influenza A. Unfortunately, a muscle biopsy was not performed to rule out metabolic myopathy or polymyositis. However, the clinical presentation and the temporal relationship of her CK levels with influenza resolution strongly suggests a diagnosis of influenza A-induced rhabdomyolysis.
She was started on aggressive fluid resuscitation with normal saline in order to prevent heme pigment-induced acute kidney injury. She was also given oseltamivir 75 mg twice daily. Alkaline diuresis with the administration of sodium bicarbonate should be considered in the treatment of rhabdomyolysis. In our case, the patient's urine pH was 7.0 and alkaline diuresis was not indicated. After three days of hospital stay, her serum creatinine kinase downtrended to 6160, and the patient was discharged home in a stable condition.
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pmc-6089490-1
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A 32-year-old primigravida was admitted at 15 weeks of gestation due to severe vomiting for two months. She had episodes of severe vomiting that required two admissions previously. She also complained of progressive blurred vision, vertigo and unsteady gait for a duration of two weeks. There was no headache, fever, pain on eye movement, hearing loss or confusion.
The patient was on a peculiar diet which consists of mainly fruits and honey. However, her condition deteriorated. She has no other medical illness before her pregnancy state.
She was clinically dehydrated and walked with an ataxic gait. There were reduced reflexes over the lower limbs. Her blood pressure was normal and she was afebrile. There was no fever or signs suggestive of meningism. All other cranial nerves examinations were intact.
Visual acuity in the right eye was 6/24 pinhole 6/18 and 6/18 pinhole 6/12 in the left eye. Bilateral horizontal nystagmus was present. There was no relative afferent pupillary defect or ophthalmoplegia observed. The anterior segment examinations of both eyes were unremarkable. The intraocular pressure was within normal range for both eyes. Fundus examination revealed bilateral swollen and hyperemic optic disc which was more marked on its temporal aspect. There were hemorrhages observed at the peripapillary retinal nerve fiber layer. There was no sign of vitritis, retinitis or choroiditis (Figure ).
She had difficulty interpreting numbers on the pseudoisochromatic ishihara chart. There was no red desaturation or reduced light brightness in both eyes. Bedside confrontation test was grossly normal, with no obvious visual field defect detected. The optical coherence tomography of both eyes showed increased retinal nerve fiber layer (RNFL) thickness with no detectable macular edema. The RNFL thickness of the right eye was 130 μm and the left eye RNFL was 191 μm.
Blood investigations revealed low serum potassium (2.6 mmol/L), and low serum sodium (130 mmol/L). Other electrolytes, creatinine and full blood count were within normal limits. There was deranged liver function test with increased total bilirubin of 46.8 umol/L, alkaline phosphatase 74 u/L and transminitis with alanine transaminase 168 u/L. Urinary analysis showed 2+ ketonuria with no proteinuria.
Magnetic resonance imaging of the brain showed T2 and fluid-attenuated inversion recovery (FLAIR) hyperintensity at the periaqueductal area, bilateral symmetrical medial thalamus and pulvinar of the thalamus suggestive of thiamine deficiency. There was no evidence of restricted diffusion, mass effect or midline shift. Magnetic resonance angiography and venography were within normal limits (Figures -).
The patient was clinically diagnosed as Wernicke encephalopathy based on clinical and radiological evidences. She was treated empirically with high dose intravenous thiamine replacement therapy (500 mg every eight hours for three days and then 500 mg daily for five days) with intravenous fluids.
There was significant clinical improvement within the first day after initiation of thiamine therapy. She became more alert and able to respond quickly to questions. Her visual acuity improved to 6/6 for bilateral eyes. She was able to read all plates from the pseudoisochromatic ishihara chart and other optic nerve function test remained within normal limits. There was prompt resolution of nystagmus and other neurological signs. She completed the treatment for eight days as an inpatient. However, she defaulted subsequent follow-ups.
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pmc-6089491-1
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An 80-year-old man residing in a nursing home, with a past medical history of a prior stroke, hypertension, hyperlipidemia, diabetes mellitus, dementia, and gastrostomy tube placement (inserted endoscopically two years before) due to dysphagia secondary to the stroke, presented to the emergency department (ED) with hematemesis and bleeding around the gastrostomy site. A review of his medication list revealed that he was taking aspirin 81 mg daily but no additional, nonsteroidal anti-inflammatory drugs (NSAIDs), antiplatelet drugs, or anticoagulants. On physical exam, he appeared exhausted. He was tachycardic (pulse rate 116 BPM), hypotensive (blood pressure 98/65 mm Hg), and febrile (temperature 100.6°F). The abdomen was soft and not distended, with no tenderness and normoactive bowel sounds. There was no guarding or rigidity. A gastrostomy tube was seen, and the scale indicated that the internal bumper had dislodged (Figure ). Dried blood was seen at and around the gastrostomy site. On rectal exam, melena was discovered.
Aggressive intravenous hydration with normal saline was given. Laboratory testing was performed and the complete blood count (CBC) showed hemoglobin of 6.8 g/dL (12.0 - 15.5 g/dL) with an unknown baseline, hematocrit of 21% (34 - 48%), white blood cell count of 12.4 × 109/L (4.0 - 11.0 × 109/L), and platelet count of 382 × 109/L (150 - 450 × 109/L). The comprehensive metabolic panel (CMP) was remarkable for an elevated blood urea nitrogen of 42 mg/dL (7 - 22 mg/dL) and creatinine of 1.2 mg/dL (0.6 - 1.3 mg/dL). The liver function tests were normal. Two units of packed red blood cells were transfused.
Repeat laboratory testing performed the following day showed a hemoglobin of 9.0 g/dL (12.0 - 15.5 g/dL) and a hematocrit of 27% (34%-48%). Esophagogastroduodenoscopy (EGD) was performed and revealed a large gastric ulcer, with signs of recent bleeding, at the site of contact with the internal gastrostomy tube bumper, which had compressed and ulcerated the gastric wall (Figure ). The dislodged gastrostomy tube was removed, and a new balloon gastrostomy tube was inserted, via the gastrostomy, into the stomach. The position of the balloon in the stomach was confirmed by endoscopy. The internal balloon was inflated with 15 mL of water.
Enteral feeding was successfully resumed the following day. Over the next few days, the patient’s clinical condition improved, and he returned to his nursing home.
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pmc-6089697-1
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A 77-year-old man with diabetes mellitus and hypertension presented with right lower quadrant (RLQ) abdominal pain that started a few weeks prior to his presentation. Physical examination revealed a palpable RLQ mass. An abdominal computed tomography (CT) scan revealed a 5.5 cm irregular soft tissue mass abutting the ascending colon medially (Figures , ).
Colonoscopy revealed diverticulosis in the sigmoid and ascending colon. On positron emission tomography (PET) scan, the mass exhibited increased metabolic activity suspicious for biological tumor activity (Figure ).
A CT-guided biopsy revealed inflammatory cells. The patient underwent a diagnostic laparoscopy, and a fresh frozen section was inconclusive for malignancy. This was followed by a robotic-assisted right hemicolectomy en bloc with the mass. Pathology showed diverticulitis with localized suppurative granulomatous inflammation in pericolic fat (Figure ).
The non-caseating granulomas consisted of epithelioid histiocytes with abundant eosinophilic cytoplasm and eccentric nuclei (Figure ). Immunostains were positive for CD68 (Figure ) and vimentin (Figure ) and negative for pancytokeratin (Figure ), eliminating the possibility of a carcinoma or sarcoma.
The patient improved clinically and was successfully discharged after few days of his surgery.
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pmc-6089698-1
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This 48-year-old female patient was brought by her mother to the emergency department with complaints of being unarousable two hours after her usual waking time. She was diagnosed to have hypothyroidism two years ago but without compliance with treatment. She had attained menopause at 40 years of age. At 22 years, she had separated from her husband within a year of marriage but she refused to elaborate the reasons for her separation. She did not conceive during her marriage or thereafter. She had been living with her parents ever since and there had been frequent altercations regarding her laziness and the need for them to support her. Her brother had diabetes that was controlled with oral hypoglycemic agents. There was no history of other comorbid illnesses. On examination, she was drowsy, with a bite-mark on the tongue laterally and did not have any focal neurological deficits. Periorbital puffiness, ichthyotic skin, and hoarse voice were observed. Her capillary blood sugar was 24 mg/dl. She was immediately administered 100 mL of an intravenous bolus of 25% dextrose, followed by continuous infusion of 10% dextrose (50g in 2 hours) without significant improvement in either sensorium or capillary glucose levels, which remained <50 mg/dL for two hours. Moreover, she developed hypotension (80 mmHg) within an hour of admission. After obtaining samples for thyroid function and cortisol, intravenous hydrocortisone 100mg was administered. Considering her family history and accessibility to drugs, sulfonylurea overdose was also suspected and hence subcutaneous octreotide 50µg was given. Thereafter, her sugars stabilized between 90 and 140 mg/dL.
Her initial investigations were as follows: thyroid stimulating hormone (TSH) 1.35 μIU/mL (0.34–4.25), free T4 0.40 ng/dL (0.7–1.24), free T3 1.00 pg/mL (2.4–4.2) and random serum cortisol 12.40 μg/dL (5–25). In view of secondary hypothyroidism, computed tomography brain and other hormones analyses were also performed. An empty sella on computed tomography (CT) and skull radiograph, serum prolactin 1.04 ng/ml (1.9–25), luteinizing hormone (LH) 0.97 mIU/mL (16.0–64.0), follicle stimulating hormone (FSH) 0.37 mIU/mL (18.0–153.0) were seen (Figure and Figure ; Figure is a control image taken from another 45-year-old female). Electrocardiogram showed T wave inversion in all leads (Figure ). Her renal and liver function tests were normal. Fasting C peptide 1.41 ng/mL (0.81–3.85 with corresponding blood glucose being 84 mg/dL) and CT abdomen were performed to rule out pancreatic causes of hypoglycemia. Viral serologies (HIV, hepatitis B, and C) were negative. MRI brain and testing for growth hormone, adrenocorticotrophic hormone (ACTH), and antidiuretic hormone was unavailable in our institution. Following a diagnosis of ESS with hypopituitarism, oral prednisolone 7.5 mg/day and levothyroxine 100 mcg were continued. On a follow-up visit two months later, she was cheerful and did not have a recurrence of symptoms.
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pmc-6089699-1
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A 67-year-old male patient of Europid origin presented to the Clinic of Cardiology with symptoms of chest pain on exertion which lasted for approximately 10 minutes and resolved after rest. The patient reported that such events had been occurring for several years, but became more frequent over the previous three months. There was no history of syncope, but the patient reported cases of orthopnea with nocturnal dyspnea. Physical examination revealed a person with normosthenic build, arterial blood pressure 145/95 mm Hg, and pulse rate 65/min. Auscultation of the heart sounds was normal, without any pathological murmurs. The electrocardiogram showed a sinus rhythm and normal axis of the heart and revealed no data of ischemic damage or arrhythmias. Transthoracic echocardiography revealed normal ventricular systolic function with an ejection fraction (EF) of 60% and insignificant mitral regurgitation. Cardiac enzymes were within the reference ranges. Blood lipid tests revealed increased levels of triglycerides and low density lipoprotein (LDL)-cholesterol together with borderline low high-density lipoprotein (HDL)-cholesterol levels. The patient reported no history of tobacco use and claimed not to be exercising regularly. The suspected preliminary diagnosis was coronary artery disease, and so the patient was further evaluated through coronary angiography. It revealed the origin of the LMCA from the RSV, which then gave off the LCX and LAD (Figure -). The LCX was found markedly hypoplastic. The RCA originated in the usual way from the RSV (Figure ), but was larger in size and was recognized as the so-called ‘superdominant’ RCA, which gave off branches for the territory normally supplied by the LCX (Figure ).
The left sinus of Valsalva (LSV) gave off no arteries (Figure ). Atherosclerotic lesions were not observed, and the symptoms were discussed to have been caused by the anomalous pattern of the coronary arteries.
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pmc-6089700-1
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An 83-year-old female with bilateral primary TKA performed 17 years prior presented to the clinic. The patient was referred with worsening left knee pain, reported gait instability, and swelling for three months duration. Until this point, she had been completely asymptomatic. She was initially seen and treated by an orthopaedic surgeon from an outside facility with physical therapy, followed by a left knee arthrocentesis to rule out infection. The aspirate demonstrated proteinaceous fluid with few benign inflammatory and epithelial cells and cultures were found to be negative. Due to the increasing pain, gait instability, and discomfort, coupled with lack of relief by the current measures, she was referred to the orthopaedic surgery clinic at our institution for further evaluation.
At her initial visit, the patient reported steadily increasing, sharp pain localized to her left knee joint with associated swelling that worsened with ambulation and prolonged standing and lacked improvement with conservative management. Her day-to-day activities were becoming restricted secondary to the pain and she reported occasional falls due to the perceived instability of her knee joint. Physical exam revealed a mild antalgic gait and tenderness over her proximal tibia. An in-house X-ray was notable for an increase in size and number of osteochondral bodies in the left suprapatellar recess with a left joint effusion and “lysis and subsidence of the tibial component and decreased thickness, suggestive of loosening and wear” (Figures -). The patient then underwent a bilateral knee bone scan which confirmed the tentative diagnosis of implant loosening with polyethylene wear and instability. The patient was counseled on her treatment options, including surgical and non-surgical management, and elected to undergo revision surgery of her left knee arthroplasty.
A classical anterior approach to the knee was made through the patient’s previous scar. A medial parapatellar arthrotomy was performed, after which the knee joint was exposed, revealing extensive osteophytes around the patella (Figure ). The osteophytes were removed and a medial release was performed, allowing for removal of the previous components (Figure ). The femoral component was found to have bone ingrowth, which had encased the patella (Figure ). Visualization of the bone-cement interface intra-operatively proved difficult. Upon gross visual inspection, it appeared that local long-term reaction at bone-cement interface had engulfed the cement and resulted in direct ingrowth of bone to implant. Bone-implant interface tissue was taken for histology examination. Microscopically, the sections examined showed papillary synovial proliferation which is consistent with the patient’s history of long-standing osteoarthritis (Figure ). Multiple foreign body giant cells, which are formed by fused macrophages, are seen in response to polarizable foreign material (Figure ). Orthopaedic implants can cause chronic inflammation and giant cell foreign body response as seen in this case (Figure ).
After component removal, joint preparation was done in standard fashion by membranous tissue removal and minimal freshening of previous bone cuts. Then revision of knee components was performed as per standard technique.
Postoperative X-rays confirmed excellent placement of a left knee arthroplasty (Figures -). The patient experienced no postoperative complications and was discharged from the hospital on postoperative day 2. She then followed up in the clinic two weeks postoperatively. The patient stated her pain was well controlled and had been working well with physical therapy. X-rays performed at this time reported that the left knee arthroplasty was in expected position with no evidence of hardware failure or loosening (Figures -). She reported that she was pleased with her new prosthesis.
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pmc-6089702-1
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A 35-year-old female with type-2 diabetes mellitus diagnosed five years ago and being managed with metformin was given liraglutide six months ago to improve her glycemic control. However, for a better dosing schedule, liraglutide was subsequently discontinued and a recently improved medication, albiglutide, was added. The following day, post the administration of her first dose, the patient reported a swelling of her lower extremities, which progressed over the next two days to involve the face and upper extremities. She also had a weight gain of five pounds. There was no other possible explanation of generalized edema and the patient did not have cardiac, liver, or renal disease. There were also no recent changes in her medications. Her physical examination was remarkable for generalized edema and periorbital puffiness. There was no stridor and her lungs were clear to auscultation. The further physical examination was unremarkable. Important laboratory investigations are given in Table .
All the blood tests listed in the above table are normal. Chest X-ray, electrocardiogram (EKG), and ultrasonography of her abdomen were all unremarkable. Due to a cause-and-effect relationship, it was presumed that her generalized edema could be due to a possible side effect of albiglutide. Consequently, albiglutide was discontinued, which led to the resolution of her generalized edema, thereby confirming albiglutide as the causative agent. Liraglutide was then resumed and our patient remained symptom-free.
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pmc-6089704-1
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A 77-year-old female presented to the orthopedic hand clinic with a three-year history of an extremely sensitive small mass on her right wrist. The mass had subjectively grown over this period of time. The pain had progressively worsened over time, and she had developed significant hypersensitivity to light contact. There was no complaint of cold sensitivity to the mass. The pain occasionally radiated down the ulnar aspect of her wrist. She had no known history of previous trauma to this area; however, she did have a history of squamous cell carcinoma to the dorsal-radial aspect of that hand. This had been treated previously for which she subsequently developed a reflex sympathetic dystrophy (RSD), resulting in a delayed recovery in the range of motion. A previous stellate ganglion block did not provide relief for her RSD, and her range of motion had been slowly progressing with home exercises.
On physical exam, a small round nodule approximately 5 mm x 5 mm was palpable dorsal to the extensor carpi ulnaris and 1 cm proximal to the ulnar styloid. There was significant point tenderness that did not radiate or display a Tinel’s sign. Her imaging included plain films of the affected extremity that showed no abnormality outside of diffuse osteopenia.
The location and exam were consistent with a neuroma that had evolved from a cutaneous nerve or possibly from the dorsal sensory branch of the ulnar nerve. The patient was taken to the operative theatre and deep dissection revealed a maroon-colored mass approximately 5 mm x 5 mm, connected to a cutaneous nerve branch. The nerve and mass were excised and sent for histopathological review. The ulnar nerve and dorsal sensory branch were visualized and confirmed to have no involvement with the mass. At her first postoperative visit, she reported no pain and that she was very satisfied with the results of her surgery. Diagnostic pathological stains were consistent with a glomus tumor. This was confirmed with strong reactivity to immunostaining of type IV collagen and smooth muscle actin (Figures , ).
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pmc-6089705-1
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A 21-year-old Caucasian female gravida two parity one at 28 weeks presented to the dermatology clinic for evaluation of a large mass on her left lateral thigh (Figure ). The patient had an existing diagnosis of NF One, but no other medical conditions. Denied any use of regular medication besides prenatal vitamins. The mass had been present since early childhood and had been stable in size since 11 to 12 years of age.
Per the patient, the mass started to grow early in her first trimester and was noted to be more painful than in the past. The patient reported pain with minor trauma that lasted for days. No change in consistency of the mass was noted. No reported weakness or altered sensation in the leg, night sweats, fevers, chills, or weight loss by the patient. In her past pregnancy, the patient denied any change to the size of the mass or increase in pain like she was currently experiencing.
The physical exam was notable for multiple 2-3 mm hyperpigmented macules in the bilateral axilla. The upper extremities and back had large hyperpigmented tan macules and patches in various sizes consistent with Cafe au lait spots. The left thigh had a large 18 x 9 cm boggy hyperpigmented mass on the lateral side. It was pendulous and had multinodular consistency. The mass was moderately tender on palpation. The patient was noted to be able to ambulate without difficulty and had equal sensation and strength in both lower extremities.
After discussion with the patient, she was prepped for punch biopsy of the mass. Multiple biopsies were taken from different areas of the mass to ensure adequate sampling. The results of the biopsy revealed plexiform neurofibroma without any indications of malignant changes in any of the sites. The patient returned to the clinic a week later for suture removal and was informed that the mass was a plexiform neurofibroma, but did not appear to be malignant at the time of biopsy.
The patient was educated about the risk of malignant transformation of the plexiform tumor and told to return if the mass changed in size again or became more painful. There were no indications of weakness in the extremity that would need to be evaluated further. The patient was advised to follow up with her obstetrician for routine pregnancy care. The patient was counseled to seek follow-up with an ophthalmologist for a full eye exam and to maintain regular follow-ups with her primary care physician.
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pmc-6089854-1
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A 79-year-old man was admitted to our hospital with symptoms of dysphagia and body weight loss of 10 kg for the past 3 months. His height was 158.0 cm and weight was 53.6 kg. His body mass index (BMI) was 21.5 upon admission. He was bent over due to osteoporosis and had undergone distal gastrectomy due to submucosal tumor (SMT) 40 years ago. According to his operative note, distal gastrectomy by open surgery was performed without lymph node dissection. After the retroperitoneal attachments behind the duodenum were dissected (Kocher maneuver), Billroth I reconstruction was performed. The resected mass was microscopically determined to be aberrant pancreas.
He was admitted to our hospital for dysphagia, and upper gastrointestinal examination revealed the presence of postoperative stomach in the thoracic cavity via delayed barium passage (Fig. ). Endoscopic examination showed that esophagitis and tumor in the upper digestive tract were absent (Fig. ). Enhanced computed tomography (eCT) revealed a large hiatal hernia involving the entire stomach, sliding through the hiatal orifice into the mediastinum, and that the stomach was expanded with food remaining inside (Fig. and ). The stomach in the posterior mediastinum compressed the heart; however, the patient had no symptoms of cardiac failure, and the heart function was normal on echocardiography. Preoperative eCT also showed that the right and left gastric arteries and the gastroepiploic arteries were preserved, indicating that the distal gastrectomy had been performed without lymph node dissection. He was diagnosed with hiatal hernia clinically, and the symptom of dysphagia was relatively severe; therefore, elective surgical repair of the hiatal hernia was performed. A total of five ports were placed in the abdomen, and a Nathanson liver retractor to retract the left liver lobe was inserted by blunt force (Fig. a). A laparoscopic approach revealed a slight adhesion of the greater omentum just under the postoperative scar. Moreover, there was a little adhesion with the liver, spleen, and diaphragm in the abdominal cavity despite gastrectomy. The esophageal hiatus was dilated to 7 cm in diameter. The hiatal hernia was found to contain the entire postoperative stomach (Fig. ). The adhesion in the hernial sac of the mediastinum was stronger than that in the abdominal cavity, and safely exposing the stomach from the hernial sac was difficult. Therefore, we converted the laparoscopic surgery to open surgery, and an upper midline abdominal incision was added (Fig. ). After the dissection of the peritoneal hernial sac from the posterior mediastinum and manual repositioning of the herniated content (Fig. ), cruroplasty through interrupted sutures using non-absorbable 2-0 suture and Toupet fundoplication without short gastric artery division were performed. The operation time was 238 min, and intraoperative blood loss was 380 mL. Upper gastrointestinal examination on day 2 after the repair of the hiatal hernia showed that the stomach was located in the abdomen, with no obstruction or barium reflux through the gastroesophageal junction (Fig. b). Oral intake was started on the ninth postoperative day due to paralytic ileus after surgery. On day 15, after the repair of the hiatal hernia, he was discharged from the hospital without any symptoms of dysphagia. No dysphagia or recurrence of the hernia has been observed for 12 months after the surgery.
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pmc-6089855-1
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A 68-year-old Japanese man with a history of distal partial gastrectomy for gastric cancer 10 years earlier was admitted for surgical treatment of intrathoracic esophageal cancer (T3, N2, M0, stage III). He underwent subtotal esophagectomy with three-field lymph node dissection and removal of the remnant stomach with the abdominal lymph nodes. The alimentary continuity was reconstructed with a pedicled jejunal limb through the antethoracic route. When we separated the diaphragm from the esophagus and removed xiphoid surgically to prevent a pedicled jejunal limb injury, the pericardium was opened. Anastomosis of the esophagus and jejunum was carried out instrumentally with a circular stapler. A postoperative enteral contrast examination showed smooth passage and no deformity of the reconstructed jejunum. The patient was discharged about 4 weeks postoperatively. About 6 months after the esophagectomy, the patient was readmitted to our hospital because of abdominal discomfort and vomiting. His vital signs were stable and unremarkable. He was thin, with a body mass index of 18.6 kg/m2. Physical examination revealed that the pedicled jejunum through the antethoracic space was markedly dilated and the abdomen was soft and flat (Fig. ). Laboratory data showed only leukocytosis with no other remarkable findings. A chest roentgenogram revealed an increased cardiothoracic ratio of 70%, and an enteral contrast study showed a bird’s beak deformity. The swallowed barium remained static in the reconstructed jejunum. Computed tomography of the thoracoabdominal region showed the reconstructed jejunum within the pericardium anterior to the heart (Fig. ). We diagnosed the patient with IPDH after esophagectomy and performed an emergency laparotomy. Upon opening the abdominal cavity, we found that the reconstructed jejunum had herniated into the pericardium through an approximately 4-cm-diameter diaphragmatic defect without a hernia sac (Fig. ). The herniation was easily reduced, and the congestion of the incarcerated jejunum resolved. There was no evidence of bowel ischemia. Primary closure of the diaphragmatic defect was accomplished using vertical mattress sutures with 1–0 silk (Fig. ). Because the diaphragm adhered to the pericardium around the diaphragmatic defect, we closed these together. Moreover, to reinforce the closure of the diaphragmatic defect, we used a graft harvested from the rectus abdominis posterior sheath. Interrupted sutures with 3–0 nylon were placed to fix the 8 × 6 cm graft of the rectus abdominis posterior sheath to the diaphragmatic defect, preventing recurrence of the hernia (Fig. ). Postoperatively, an upper gastrointestinal study confirmed free flow of contrast medium from the cervical esophagus into the intra-abdominal jejunum through the pedicled jejunum. The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day.
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pmc-6089858-1
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A 17-year-old woman was admitted to our hospital complaining of abdominal pain that had persisted for 3 days. She seemed alert and was not pale, with blood pressure of 112/70 mmHg and a regular pulse of 78 bpm. Laboratory data showed a white blood cell count of 7530/μL, hemoglobin concentration of 11.0 g/dL, a platelet count of 249,000/μL, glutamic oxaloacetic transaminase concentration of 22 IU/L, glutamic pyruvic transaminase concentration of 9 IU/L, and lactic dehydrogenase concentration of 259 IU/L.
Computed tomography (CT) revealed a 10 × 10 × 10-cm low-density area in the patient’s mid-abdomen (Fig. ), and magnetic resonance imaging (MRI) showed a large abdominal cystic lesion (Fig. ). However, the tumor position differed notably between CT and MRI, and an unfixed, mesenteric cystic lesion was suspected. Single-port laparoscopic-assisted resection was therefore performed instead of conventional laparotomy.
A single-incision access platform and wound protector were introduced through a 1.5-cm transumbilical skin incision. Laparoscopy showed a large cyst derived from the greater omentum (Fig. ), which was moved to a position under the umbilical wound. The cyst fluid (which was serous in nature) was aspirated using a tissue adhesive (Dermabond™, Ethicon Inc., Somerville, NJ), a suction tube with negative pressure, and a 16-gage over-the-needle catheter and syringe; this reduced the size of the tumor and none of the cyst fluid was released into the abdominal cavity (Fig. ). Then, the tumor was successfully removed via the small incision (Fig. ) and was diagnosed histopathologically as a cystic lymphangioma (Fig. ).
The surgery was uneventful, and the postoperative recovery was normal.
Intraperitoneal cystic tumors are rare. A large cystic tumor usually requires a large skin incision for its removal. However, if the size of the cyst can be reduced by extracting its contents, it is possible to operate via a small wound []. In the present case, an abdominal cystic tumor was successfully removed via a small incision after aspirating the cyst fluid and thereby reducing the size of the tumor. The cystic fluid was successfully aspirated without spillage using a tissue adhesive, suction tube, and 16-gage over-the-needle catheter. The abdominal laparoscopic procedure used a smaller incision than is required for conventional laparotomy, and the laparoscope allowed more detailed observation, which was useful for the operative diagnosis.
Tumors close to the body surface may be resected via a small incision without a laparoscope, but movable tumors that shift to a site distant from the body surface cannot be resected in this way. However, even in such cases, a laparoscope can confirm the lesion and be used to guide it to an appropriate position. Even tumors fixed to a site distant from the body surface can be brought to the surface using a laparoscope after removing the surrounding tumor tissue. Laparoscopy allows for the manipulation of various types of tumor.
When cystic lesions can be reduced by aspirating the cyst contents, it is possible to perform a minimally invasive procedure with a small incision. However, it is crucial that none of the cyst contents leak into the peritoneal cavity. Preoperatively, our patient’s cystic tumor was predicted to be benign; therefore, surgery without lymph node dissection or extended resection was planned. However, the cyst contents may have been infected. In addition, a malignant cystic tumor could not be ruled out. Thus, care was taken to avoid any leakage of the cyst fluid into the surrounding areas. Trocar site recurrences due to bile leakage into the site of incision have been reported during cholecystectomy [].
In our case, surgery was performed without any leakage of cyst fluid using medical equipment that is readily available at all surgical facilities. Laparoscopic-assisted surgery should be used to resect intraperitoneal cystic lesions wherever possible. This method can be applied to various types of cases, such as gynecological abdominal cystic diseases [, ]. The procedure used for the present case was easy to perform and required no special materials.
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pmc-6090027-1
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The patient was a 10-year-old right-handed boy. The duration of the illness was 3 years. The first episode occurred at the age of 7 years, without obvious inducement. Symptomatic manifestations included sudden shouting, right-side strabismus, salivation, and generalized tonic-clonic seizures (GTCS), which lasted for about 4–5 min. The second episode occurred 10 days later, with similar symptomatic manifestations. There was no seizure for 1 year after oral oxcarbazepine medication. Seizures then occurred at the age of 8 years, with symptomatic manifestations such as panic, shouting, and tachycardia and lasted about 1–2 min without loss of consciousness. Headache, left eye pain, abdominal pain, and nausea would occasionally appear in the post-ictal period. Gradually, the patient's seizure frequency ranged from once a month to 4 times/day. Oral administration of oxcarbazepine was ineffective. History of perinatal hypoxia, febrile convulsions, brain injury, and family history of epilepsy were negative. Physical examination showed stable vital signs, and the results of neurological examination were normal. Blood routine, biochemical tests, infection immunoassay, blood coagulation tests, and urinalysis were all normal. Electrocardiogram and chest radiography showed normal results.
To evaluate the ictal fear, both video observation and neurologic interview were conducted according to the diagnosis criteria of ictal fear (). First of all, fearful facial expression and screaming had been observed before the clinical seizure (Figure ). The content of screaming includes “Aha! Aha!” or “Mama!” or “Mama! Find the doctor!” Secondly, the patient could remember and describe feelings of fear, without any specific content or scene. Visual aura or other associated aura was denied. The last but not the least, the patient described this fear feeling start abruptly, concomitant with the seizure.
Scalp video EEG (vEEG) were recorded using a Nicolet video-EEG monitoring system (Thermo Nicolet Corporation, USA), digitized at the rate of 1,024 Hz with the international standard 10–10 electrodes montage. The on-line band-pass filter was 1.6–150 Hz. The patient was monitored for a total of 6 days. During the monitoring, oxcarbazepine was gradually reduced and one typical epileptic seizure was captured (Figure ).
Figure shows the vEEG waveforms during the interictal period. Interictal epileptiform discharges (IEDs) could be observed (denoted by red arrows), which were mainly distributed within the left occipital electrodes (P7 and O1). Figure shows the vEEG waveforms before the epileptic seizure. The EEG onset occurred 1.5 s earlier than the ictal fear. Fearful facial expression and screaming could be observed in the video (Figure ).
Brain imaging results showed that while the T2 signals within the left posterior temporal, occipital, and parietal lobes were high, the T2 signals in the left hippocampus was higher (Figure ). Further positron emission tomography/computed tomography (PET-CT) examination showed that low metabolic areas were mainly distributed in the left posterior region (Figure ).
Stereotactic electrodes were intracranially implanted for further epileptic zone evaluation. A robot-assisted stereotactic operation system (ROSA) was used to place 13 electrodes. The sEEG electrodes were manufactured by Huake Hengsheng Medical Technology Co Ltd., Beijing, China. The diameter of a depth electrode was 0.8 mm. The length of each node was 2 mm, which were spaced 1.5-mm apart from each other. The reference electrode was placed on the forehead. On recording days, the impedance of all the recording electrode nodes was kept below 50 kΩ, and the nodes whose impedances were higher than this value were excluded from analyses. Video EEG was monitored for 6 days after implantation, and the oxcarbazepine dosage was gradually reduced during the monitoring; during this period, a total of 4 seizures were captured. Three-dimensional brain images were reconstructed by pre-implantation of T1 images using BrainSuite (Version 18a, ). Pre-implantation PET-CT and post-implantation CT (included electrode localizations) were co-registered to pre-implantation T1 images using BioImage software (). The localization of sEEG electrodes were then presented using the Brainstorm toolbox [(); ] in the MATLAB environment (Figure ).
Figure shows the sEEG results before a representative seizure. A bipolar lead view showed that the EEG onset was 1 s before ictal fear and 12 s before amygdala (A1-A2) and posterior hippocampus (C2-C3) (Figure ). At the moment of the start of EEG, simultaneous direct current (DC) drifts were observed in J5-J6, J6-J7, J8-J9, K5-K6, and K6-K7, with high frequency components in spectra (Figure ). Direct electrical stimulation (frequency = 50 Hz; pulse wide = 0.3 ms; duration = 5 s; intensity = 1–6 mA) confirmed that only stimulation on J8-J9 could evoked a clinical seizure.
According to the sEEG results, the epileptic zone was mainly distributed in the right posterior parietal, posterior temporal, and occipital areas (Figure ), and then propagated to the posterior part of the middle and inferior temporal gyrus, the posterior part of the hippocampus, the anterior part of the lingual gyrus occipital tongue, and the anterior part of the precuneus. Combined with the evidences of brain imaging (T2 flair and PET-CT), the resection range was planned as shown in Figures ,B shows the actual lesion area a year after surgery. The pathological examinations showed focal cortical dysplasia (FCD) type 2A in the right parietal-occipital area.
According to the follow-up, the neuropsychological performance before and after the surgery were all in the normal range (Table ). No seizure occurred after surgery.
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pmc-6090038-1
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We hereby describe the case of a 4 year old girl admitted to the pediatric emergency department for continuous abdominal pain, more intense in the orthostatic position, associated with abdominal distension, nausea, and vomiting. These symptoms raised the clinical suspicion of acute abdominal syndrome. The patient had no previous clinically significant events.
Based on the findings from initial clinical evaluation, the pediatrician recommended an ultrasound. The radiologist described an intraabdominal, multilocular cystic mass with liquid inside (Figure ), and subsequently recommended an MRI to certify the diagnosis. The MRI confirmed the presence of the liquid filled multicystic structure with hypersignal in ponderate sequences T2 and T2 with fat suppression. The lesion had a multilocular aspect and a moderate contrast snuff at the level of the walls following contrast substance administration (Figures ). These radiologic findings strongly support the diagnosis of intestinal lymphangioma, and tumor removal by surgery was recommended.
The surgically removed tumor was sent for histopathologic evaluation. Macroscopically, the pathologists identified a 8.9 cm length intestinal fragment, with a 0.5–1.2 cm luminal diameter, that was incorporated in a soft, yellow-translucent mass. When sectioned, there were multiple cysts with a wall thickness that was less that 0.1 cm and a smooth inner and outer surface, filled with a white-milky content (Figures ).
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pmc-6090605-1
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The first patient was a 20-year-old man keeping cows and sheep in house, living in a rural area of southern part of Afghanistan. The patient was complaining from shortness of breath and cough for last one and half year and was referred to undergo chest CT examination. A Contrast enhanced chest CT (with intravenous administration of 80 ml of non-ionic water soluble contrast material mnipaque-350-) revealed a well-defined, thin walled, low attenuating, cystic lesion with lobulated outlines in the anterior segment of the left lung upper lobe, measuring approximately 4.2 × 5.5 × 4.5 cm in size (Fig. , arrow). Another small (1.5 × 1.5 cm) cystic lesion with same characteristics was seen in the anterior segment of right lung upper lobe (Fig. - arrow). A lesion of same characteristics was seen in the lateral wall of left ventricle which measured 3.6 × 3.9 × 3.5 cm (Fig. - curved arrow and Fig. ).
Imaged sections through the abdominal cavity revealed at least seven cystic lesions in the liver. The largest lesion in segment seven of liver measured 7 × 8 cm. Some of the lesions demonstrated internal detached membrane, the so called water lily sign (Fig. and , curved arrows). No solid enhancing components, wall calcification or adjacent infiltrative/inflammatory changes were noted with these lesions.
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pmc-6090605-2
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A 28-year-old shepherd man living in rural area of northern part of Afghanistan with chief complaint of cough and shortness of breath for last four years was referred for a chest CT examination. CT images revealed a large, well defined, fluid attenuating, non-enhancing cystic mass lesion in the left lung mostly occupying the left upper lobe measuring approximately 17 × 11 × 15 cm (Fig. ,). Some internal septations were seen in the superior aspect of this cystic lesion (Fig. - arrow). A multiseptated, fluid attenuating cystic lesion was seen in the cardiac apex measuring approximately 6.5 × 5.7 × 5 (Fig. ). The lesion demonstrated some peripheral wall calcification in pre- contrast images (Fig. -curved arrows).
Keeping in mind multiplicity of the lesions, typical CT features, contact with animals and geographic location of the patients, the diagnosis of hydatid cysts was made for both of the patients.
Unfortunately, as the patients were sent to the authors department only for CT examinations, therefore further clinical and laboratory information (including blood tests and ECG findings) as well as follow up of treatment is unavailable.
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pmc-6090622-1
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A 54-year-old man with cutaneous plasmacytosis of the face, chest, and back consulted the neurology service at our hospital with complaints of tingling pain in his face, tenderness in the bilateral lower legs, and easy fatigability. He had developed red-brown plaques on his face and trunk at age 45 years, and consulted his local hospital at age 47. Skin biopsy was performed at that time and histological examination revealed characteristic findings of cutaneous plasmacytosis. At about the same time as he was diagnosed with skin plasmacytosis, the patient also complained of myalgia with easy fatigability and was diagnosed with fibromyalgia. His other medical history was remarkable for hypertension, angina pectoris, left mastoiditis, lumbar stenosis, and osteoarthritis of bilateral knees. Nine years after the onset of myalgia, the patient consulted our neurological service and was admitted to our department to identify the cause of facial and lower leg pain.
On admission, the patient had red-brown plaques on his face and trunk (Fig. ). He complained of myalgia in his bilateral vastus lateralis muscles, hip adductors, and gastrocnemius, which worsened with pressure. Muscle strength tests revealed bilateral trivial weakness of the gastrocnemius with normal muscular tone. The results of general and neurological examinations and routine blood analysis, including creatine kinase 95 U/L (normal range 57–240) and thyroid function, were all normal. Serum antibodies specific to syphilis, neurotrophic viruses, human immunodeficiency virus, and thyroid and autoimmune diseases, including interleukin-6 (IL-6), IgG4, antinuclear, anti-Sm, anti-mitochondria M2, anti-SS-A/SS-B, anti-Jo-1, anti-Scl-70, anti-acetylcholine receptor, myeloperoxidase-, and proteinase 3-anti-neutrophil cytoplasmic antibodies were all normal. Rheumatoid factor and anti-cardiolipin antibodies were positive, but anti-citrullinated protein antibody and lupus anticoagulant were negative. Serum levels of soluble IL-2 receptor 873 U/mL (normal range 145–519), IgG 1914 mg/dL (normal range 870–1700) and IgA 619 mg/dL (normal range 110–410) were all elevated. However, both Bence Jones protein and M protein were negative. We performed a repeat skin biopsy to confirm the diagnosis of plasmacytosis. The biopsy specimen (Fig. , ) showed prominent dermal perivascular proliferation of mature plasma cells, with a normal gamma chain kappa/lambda ratio, and a MIB-1-labeling index of less than 5%. In the specimen, CD79a expression was positive but CD56, cyclin D1, and Epstein Barr virus-encoded RNA were negative. There was no evidence of immunoglobulin heavy-chain gene rearrangements. Therefore, these findings were compatible with cutaneous plasmacytosis, not multiple myeloma. The findings of a nerve conduction study were all normal, but gastrocnemius needle electromyography revealed positive sharp waves, repetitive discharge, and polyphasic motor unit potentials. However, there was no evidence of early recruitment. These findings suggested denervation, but myogenic changes were inconclusive. T1- and T2-weighted MRI of the lower legs revealed abnormal high intensity in several muscles, including bilateral adductors, quadriceps, semimembranosus, and gastrocnemius muscles. Short tau inversion recovery imaging also showed abnormal intensity of the bilateral quadriceps and gastrocnemius muscles (Fig. –). These findings indicated myositis with chronic inflammation, and we therefore performed a biopsy of the right gastrocnemius muscle. Morphological analysis of the muscle specimen showed fiber size variability, scattered necrotic and regenerated fibers, and lymphocyte infiltration predominantly in the endomysium rather than the perimysium or the vessels (Fig. ), but no perifascicular atrophy or rimmed vacuoles. CD8+ toxic T cells surrounded the non-necrotic fibers and major histocompatibility complex (MHC) class 1 antigen expression was ubiquitous (Fig. , ). There were almost no B cells, CD4+ cells, macrophages, or membrane attack complexes around the small vessels. A few plasmacytes were visible in the endomysium (Fig. ), and they stained negative for IgG4. These pathological features were compatible with European Neuromuscular Center classification criteria of polymyositis [].
The patient’s muscle pain was unaffected by acetaminophen and pregabalin, but his myalgia and cutaneous plasmacytosis on his face and back were ameliorated by prednisolone (20 mg/day) (Fig. ).
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pmc-6090635-1
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This is a 48-year-old female patient who was referred after a biopsy of her right axillary mass revealed synovial sarcoma.
The patient incidentally noticed a painless mobile lump in her right axilla about 6 months prior to her biopsy. The mass did not change in size over this period. It was associated with increasing numbness involving her right ring and little fingers that was bothering her during her daily activities especially during the last 2 months. She reported no other masses in her breasts or elsewhere.
Her referring surgeon evaluated her initially for the possibility of breast cancer by ultrasound and mammogram; both of which were negative. So, he performed FNA initially which was inadequate. Then, core-needle biopsy was performed which revealed monophasic synovial sarcoma.
Physical examination of the right axilla showed: 5 × 5 cm ill-defined mobile mass with smooth surface located in the apex of the axilla with no overlying skin changes. No adjacent masses or regional lymph nodes including axillary and cervical lymph nodes were felt. Her peripheral neurovascular examination was unremarkable except for mild decrease in superficial touch sensation in her right little and ring fingers.
Staging studies were performed including MRI of the axilla and CT angiogram for local vascular assessment. CT of the chest, abdomen, and pelvis with bone scan showed no evidence of metastasis.
MRI showed well-defined oval-shaped heterogeneous soft tissue mass in close proximity to the axillary artery with no definite encasement. The mass measured 3 × 3.3 cm in axial diameter and 4 × 4 cm craniocaudally (Figs. and ). The mass was isointense on T1 and slightly hyper intense on T2 with vivid enhancement post-gadolinium administration.
CT angiogram showed mild mass compression at axillary/brachial arteries transition with patent peripheral vessels (Fig. ).
Based on the radiological and the histopathological findings, the plan was to proceed with mass excision after exploration of the axillary artery and the surrounding brachial plexus. However, forequarter amputation and the need for adjuvant radiation were to be considered subsequent to the surgery.
The patient provided consent for the plan, and she was made aware that some neurological deficit is to be expected after surgery among other complications.
The tumor was exposed through extensile deltopectoral approach after developing intermuscular planes and reflecting pectoralis muscles. The tumor was located anteromedial to the third part of the axillary artery pushing the medial root of the median nerve anterolaterally and the origin of the ulnar nerve medially (Fig. ). We were able to dissect out the axillary artery and the median nerve easily away from the tumor. However, the ulnar nerve was intimately adherent to the tumor capsule, so we decided to sacrifice it to achieve tumor free margin (Fig. ). Grossly, the tumor capsule was dissected out completely. No other masses were detected in the surgical bed.
The patient had unremarkable postoperative course. However, she lost her ulnar nerve function with loss of sensation involving her right little and ring fingers.
Her final histopathology revealed monophasic synovial sarcoma with microscopically free margins. The patient did not receive postoperative radiation. Upon follow-up, 9 months after surgery, no clinical or radiological recurrence was observed.
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pmc-6090641-1
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An 86-year-old Sinhalese Sri Lankan woman who had been previously diagnosed as having hypertension, grade 2 MR, and ischemic heart disease with congestive cardiac failure, presented to our preliminary care unit with sudden onset shortness of breath at night while sleeping. She had eaten her dinner and taken her usual medications before sleeping. She had a New York Heart Association (NYHA) heart failure grade of class 2, and could manage her day-to-day activities without support. She could walk 25 meters and could climb 3–4 steps without becoming dyspneic. Apart from her usual symptoms she did not have fever, cough, or chest pain before admission. She is a housewife and mother of five children. She does not smoke tobacco or drink alcohol. At presentation she was on captopril 12.5 mg twice a day, atorvastatin 20 mg at night, soluble aspirin 75 mg at night, bisoprolol 2.5 mg once a day, and furosemide 40 mg in the morning.
On examination, she was found to be dyspneic, drowsy, pale, diaphoretic, and restless. Her body temperature was 37.0 °C. Her blood pressure (BP) was 90/60 mmHg, with a regular, low volume pulse rate of 102 beats per minute. Her heart sounds were unremarkable. Cardiac apex was not palpable. There was a pansystolic murmur at cardiac apex. Her respiratory rate was 26/minute. Her trachea was central and right-sided chest expansion was reduced. Bilateral crepitations and rhonchi were present more significantly on the right side. Her initial oxygen saturation checked by pulse-oximetry was 56% in room air. Her abdomen was not distended and there was mild right hypochondrial tenderness. There was no hepatosplenomegaly. Her cranial nerve examination was normal. Her limbs examination was normal with normal tone, power, and reflexes.
An electrocardiogram showed ST depression in leads V5–V6 and poor R wave progression in leads V1–V4. Her chest X-ray revealed alveolar-interstitial infiltrates and a fluid collection around horizontal fissure in her right lung (Fig. ). Laboratory tests showed a white blood cell count of 12,000/μL with 91.8% neutrophils, hemoglobin of 9.5 g/dL, packed cell volume of 30.3, mean corpuscular volume of 75 fl, mean corpuscular hemoglobin of 23.8 pg, mean corpuscular hemoglobin concentration of 31.4 g/dl, creatinine of 221.5 μmol/l, sodium level of 139 mEq/L, potassium level of 4.4 mEq/L, B-natriuretic peptide (BNP) of 2437.2 pg/ml (normal 450 for NYHA class 2), C-reactive protein (CRP) 7.56 mg/dL (< 10), and troponin I 59.2 ng/mL (< 0.01).
Although our patient’s temperature was normal, pneumonia could not be initially excluded in this older patient in the presence of a unilateral pulmonary infiltrate with effusion along the horizontal fissure, in combination with leukocytosis and awaiting CRP level (which took 4 hours to get the report), treatment with intravenously administered broad spectrum antibiotics (ceftriaxone 1 g twice a day and clarithromycin 500 mg twice a day) was initiated to cover severe community acquired pneumonia, and oseltamivir was started since there was an epidemic of influenza H1N1 at the time.
An emergency two-dimensional echocardiogram facility is not available in the preliminary care unit in our set up and our patient was not in a condition to be transferred to a place where a good quality echocardiogram machine was available to assess the severity of MR accurately. Echocardiography was done on third day of admission which disclosed: an ejection fraction of 25–30% with severe left ventricular (LV) dysfunction; and hypokinesia of anterior wall, LV apex, and lower 2/3 of interventricular septum, with an apical aneurysm. A two-dimensional echocardiogram showed grade 3 MR (Fig. ). Although her BNP level was found to be high it took 4 days to get the report due to delays in laboratory processing. Therefore it helped us to support the diagnosis retrospectively.
With all these challenges our patient was treated for severe acute on chronic heart failure although radiological evidence was unfavorable. Interestingly, she showed a remarkable improvement with preload reduction with loop diuretics and nitrates. After availability of troponin I levels she was treated for a NSTEMI on top of heart failure with intravenous heparin 500 units/hour infusion (her weight was 40 kg). Her condition was stabilized with adjustment of medical therapy for heart failure including diuretics, nitrates, and opioids. She had persistently low BP for which she needed inotropic support with dopamine and dobutamine which were tailed off subsequently. Repeat chest radiography taken 12 hours later showed complete resolution of the UPE (Fig. ). Subsequently, her CRP was normal and antibiotics were de-escalated after 24 hours, but oseltamivir was continued. She had a fast and remarkable recovery to her preadmission state on day 5 of admission after which she was discharged. She refused any further cardiac intervention. Two weeks after discharge she was reviewed at a medical clinic and found to have NYHA class 2 heart failure. Her BP was120/80 mmHg and pulse rate was 70/minute. Her medications were uptitrated and she was followed up in a medical clinic. After 6 month she had a two-dimensional echocardiogram and revealed ejection fraction of 40% with grade 2–3 MR.
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pmc-6090673-1
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We describe a 58-year-old white woman who was living alone at home with a known history of SLE-associated secondary APS. The diagnosis of SLE had been made 27 years previously when she developed recurrent episodes of pneumonitis, malar rash, and renal failure complicated by recurrent deep vein thrombosis. Blood tests at that time revealed positive antinuclear antibody (ANA), elevated anti-double-stranded deoxyribonucleic acid (anti-dsDNA) titers, and low complement (C3 and C4) levels and urine analysis revealed proteinuria, hematuria, and cellular casts suggestive of lupus nephritis. A diagnosis of secondary APS was made on the basis of positive LA in two determinations with a 3-month interval and a history of recurrent deep vein thrombosis. Additional thrombophilia screening (factor V Leiden mutation, prothrombin gene mutation, factor 8 levels, protein C, protein S, and antithrombin 3) was negative.
At the initial diagnosis, she received pulse cyclophosphamide and prednisolone for lupus nephritis and, after resolution of the initial thrombotic event, she was started on hydroxychloroquine and lifelong warfarin anticoagulation with a target INR between 2 and 3.
Her other medical issues included obesity, obstructive sleep apnea, diet-controlled type 2 diabetes mellitus, hypertension, stage 3B moderate chronic kidney disease, fatty liver, endometrial cancer (treated 14 years ago with progesterone, ongoing surveillance showed no recurrence), left total shoulder joint replacement for severe osteoarthritis, and Sydenham’s chorea.
Her regular medications included warfarin, atenolol, hydroxychloroquine, oxycodone, naloxone, paracetamol, and multivitamins.
Two months prior to the current admission, she developed ulcerative lesions on her anterior abdominal wall which she related to an abdominal ultrasound performed for suspected kidney stones. She reported that, at sites of ultrasound probe pressure, she initially noticed small bruises with subsequent skin breakdown associated with oozing of clear fluid and pain. She consulted her general practitioner who prescribed wound dressings and referred her to our hospital after local wound swabs grew multidrug resistant Enterobacter cloacae. At the time of hospital admission she was found to be afebrile and hemodynamically stable and had five ulcers on her anterior abdominal wall. The largest ulcer was located over her right flank and had irregular margins with surrounding erythema with a clean base. She was seen by a surgeon who did not think that the ulcers needed debridement.
Her investigations revealed mild normocytic anemia with stable chronic renal failure, that is, creatinine 134 micromole/L and glomerular filtration rate (GFR) 38 ml/minute; her INR was therapeutic at 2.5. Her INR in the preceding 8 months had been in the therapeutic range of between 2.3 and 3.6 (Fig. ). Her inflammatory markers were elevated with a C-reactive protein (CRP) of 250 mg/L. A wound swab grew Enterobacter cloacae, sensitive to ciprofloxacin, and this was prescribed orally along with local wound dressings. She was discharged home with an instruction to continue warfarin and antibiotics for a period of 2 weeks.
She re-presented 11 days later with worsening of her symptoms: specifically non-healing ulcers and increasing pain. An examination revealed progression of the abdominal ulcers (Figs. and ) with the largest ulcer located over her right flank showing increased size and depth, hemorrhagic scab, black eschar, and yellow exudate (Fig. ). There were new ulcers located on her left breast and left interscapular area. Wound swabs from the abdominal ulcers grew methicillin-resistant Staphylococci but repeated blood cultures remained sterile. Her repeat investigations revealed elevation of her white cell count and a further increase in CRP with a mild worsening of anemia but stable renal function. She was found to be positive for LA, but negative for cardiolipin IgG, anti-β2-glycoprotein 1, cryoglobulin, cryofibrinogen, ANA as determined by indirect immunofluorescence (IIF) assay, extractable nuclear antigen (ENA) determined using enzyme-linked immunosorbent assay (ELISA), and anti-neutrophil cytoplasmic antibody (ANCA) but her complement levels were mildly elevated: complement C3 1.93 g/L (normal range 0.85–1.60 g/L) and complement C4 0.44 g/L (normal range 0.12–0.36 g/L). She had a negative hepatitis C screen and urine analysis revealed mild albuminuria with no active sediment on microscopic examination: albumin/creatinine ratio 3.7 (normal < 3.5 mg/mmol).
A skin biopsy of one abdominal ulcer showed ischemia and necrosis of the subcutaneous fat, with granulation tissue reaction within the dermis and cutaneous necrosis. There was evidence of thrombo-occlusive material (Fig. ) but there was no evidence of calciphylaxis or medium-sized vessel vasculitis (Fig. ).
A second skin biopsy of a right flank ulcer showed numerous microthrombi in small vessels of the subcutaneous fat, including a recanalizing thrombus within a medium-sized vein but no vasculitis (Fig. ). A note was made of focal fat necrosis with mild inflammation of fat with rare Gram-positive cocci.
She was commenced on vancomycin and her warfarin anticoagulation was intensified to maintain the INR between 3 and 4 and wound dressings were continued as per consultation with the dermatologists and surgeons. She reported minimal improvement in her symptoms with ongoing pain and non-healing ulcers despite being on high-intensity warfarin anticoagulation and antibiotics.
After a multi-specialty case conference involving the rheumatology, hematology, and dermatology teams, it was thought that her thrombosis was warfarin-resistant and anticoagulation with low molecular weight heparin was suggested but she refused any form of long-term injections. Her clinical course was further complicated by an episode of per rectal bleeding while on high-intensity warfarin anticoagulation and this was managed by blood transfusions. A malignancy screen, including a computed tomography (CT) scan of her chest, abdomen, and pelvis, and a mammogram and pelvic ultrasound excluded underlying occult malignancy as a cause for her hypercoagulable state; a gynecologist excluded recurrence of endometrial cancer through transvaginal ultrasound, hysteroscopy with dilatation and curettage (D & C), and endometrial biopsy.
She was informed that the most likely cause for her symptoms was APS-related skin necrosis with superadded bacterial infection. She remained resistant to the idea of long-term injectable heparin although she was happy to try one of the newer oral direct inhibitors (ODIs) of coagulation despite a lack of firm evidence of efficacy. She was commenced on apixaban with continuation of wound care and an increase in analgesia.
Changing her anticoagulation from warfarin to apixaban, however, made little difference to her symptoms and she had multiple subsequent admissions with sepsis and bleeding from her ulcers with worsening of renal function. In the subsequent admissions, she was managed with blood transfusions and antibiotics. Plasmapheresis and intravenously administered immunoglobulins were tried along with ulcer debridement with little response to treatment. Due to declining renal function and lack of response to apixaban, she was changed back to warfarin anticoagulation. During her last admission (6 months from the first presentation), she was found to have an infection by Staphylococcus epidermidis of her left shoulder joint prosthesis. The prosthesis was removed and she was commenced on vancomycin. This admission was further complicated by hemarthrosis of her left shoulder and rectal bleeding needing blood transfusions. A colonoscopy performed during this admission revealed bleeding hemorrhoids, which were managed conservatively. Due to recurrent bleeding and the need for repeated blood transfusions, a decision was made, after consultation with our patient, to stop anticoagulation.
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pmc-6090687-1
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A 68-year-old male, resident of Crete island, with history of type 2 DM (diagnosed 2 years before, with satisfactory glycaemic control, HbA1c = 6.5%) and intermittent claudication (Fontaine class IIb) was referred to our diabetic foot clinic due to a 1-month history of erythema, swelling, tenderness and local warmth of the left lower limb along with paronychia and the presence of a diabetic foot ulcer (DFU) in distal phalanx of 1st toe of the left foot probed to bone. The patient had been hospitalized for 10 days in a peripheral hospital, where he underwent surgical nail removal and surgical debridement, and was treated with double antibiotic therapy. He was prescribed antibiotics for 1 week after discharge. Upon admission to our hospital, he had typical “sausage toe” (1st toe of the left foot), Fig. a. Inflammation markers were significantly elevated (erythrocyte sedimentation rate (ESR) = 101 mm/h, C-reactive protein (CRP) = 16.3 mg/L (normal values: < 3.3 mg/L) and white blood cell count (WBC) = 13.310/μL. The rest of biochemical analysis was normal. X-ray of the left foot revealed destruction of proximal and distal phalanx of the 1st toe (image compatible to osteomyelitis), Fig. a. He was initially treated with intravenous daptomycin, aztreonam, plus low-molecular weight heparin and pentoxifylline. Swab culture after debridement showed methicillin-resistant Staphylococcus aureus (MRSA) and Stenotrophomonas maltophilia, both sensitive to ciprofloxacin, so antibiotic therapy was switched to ciprofloxacin and clindamycin. Nasal swab culture was also positive for MRSA, and nasal mupirocin was given.
Additionally, he underwent colour Doppler ultrasound and CT angiography of the lower extremity arteries. The patient presented 95% stenosis of the left popliteal artery and total occlusion of the posterior tibial artery of the right lower limb. Two weeks after admission, patient was discharged and continued his antibiotic treatment with ciprofloxacin and clindamycin per os as outpatient.
After discharge, the patient was seen on weekly basis. A gradual clinical improvement and a significant reduction of the inflammation marker levels were observed. Five weeks after discharge, the patient underwent a successful angioplasty, in order to perform revascularization and restore an adequate blood flow to his left lower limb. Blood tests at this time point were almost within normal ranges (ESR = 17 mm/h, CRP = 4.01 mg/L and WBC = 8.160/μL. He continued antibiotic treatment for 5 more weeks (a total of 3 months). After the end of treatment, inflammation markers had returned to normal (ESR = 19 mm/h, CRP < 3,3 mg/L and WBC = 7.800/μL). Plain X-ray showed focal osteogenesis at the damaged proximal and distal phalanx of the 1st toe. Clinically, the patient presented minimal edema of his toe without erythema and his DFU was almost healed (Fig. b, c).
Eventually 4 months after the end of the treatment, the patient presented with complete healing of his DFU, reconstruction of his osteomyelitis defects and complete restoration of his toe nail and his foot functionality. He was able to walk 3 km without claudication. One and a half year after his initial visit, the patient remains in good shape, and plain X-ray is almost normal (Figs. b, d).
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pmc-6090700-1
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A 14-month-old female child was brought to the hospital because of abnormal appearance and growth retardation. Antenatal history revealed full-term birth at 41 weeks of gestation. She was the second child (birth weight 1.9 kg) of a gravida 2, para 2 (G2P2) mother. There was no evidence of intrauterine fetal distress or hypoxia. The placenta was small and calcified, with oligohydramnios. Immediately after birth, the child was found to have a weak cry, cyanosis, and abnormal appearance, and had an Apgar score of 7 at 1 min and 9 at 5 min. The height (66 cm) and weight (6.5 kg) of the child were three percentage points below the reference height and weight of children of the same age. Her respiratory rate was 26 cycles/min and heart rate was 110 beats/min. The general condition appeared good; breath sounds were clear bilaterally, cardiac sounds were strongly audible, and a continuous murmur was heard at the second intercostal space towards the left margin of the sternum. Abdominal examination was unremarkable and did not reveal any palpable organomegaly. Neurological examination showed a soft neck, negative Babinski’s sign bilaterally, and normal muscle tension. Gesell developmental scale test showed a development quotient of 36, which corresponds to severe mental disability. Unusual findings pertaining to appearance included (Fig. ): fair skin, small palpebral fissures, low-set ears and a preauricular skin tag on the left side, retrognathia, and uneven color distribution of the skin together with patchy pigmentation. Oral examination showed tooth fusion.
Additional investigations revealed a standard chromosomal pattern; fluorescein in situ hybridization (FISH) analysis of blood revealed a female mosaicism karyotype 47, XX, + 10/ 46, XX with trisomy 10 in 42% of metaphases in the blood (Fig. ). Cranial MRI showed (Fig. ) bilateral widening of the frontal subarachnoid space, characteristic of the Dandy–Walker syndrome and enlarged ventricles. Echocardiography was conducted to investigate the abnormalities detected on cardiac auscultation (Fig. ) and revealed the following: the descending aorta formed a multicolored blood-flow signal to the main pulmonary artery, with a flow width of 2.2 mm. The shunt persisted throughout the cardiac cycle. Doppler detected a maximal shunt velocity of 465 cm/s, with a mean pressure gradient of 86 mmHg. A 2-mm wide oblique separation was seen in the middle of the septum, which was indicative of patent ductus arteriosus (PDA) and a patent foramen ovale. No abnormalities were detected on amino acid and carnitine spectrum analysis for inherited metabolic diseases. Routine investigations of blood, urine, stool, liver function, renal function, myocardial enzymes, fasting blood sugar, and thyroid function were normal. The child’s mother and father were 34 and 36 years of age, respectively, at the time of her birth, whereas her elder sibling (brother) was 8 years old. The phenotype and chromosomal patterns in these close family members were normal. At the time of writing, the child has been followed up for 3 months after the diagnosis. At the most recent follow-up, her height has increased by 2 cm and weight has increased by 0.6 kg.
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pmc-6090727-1
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A 44-year-old Chinese female presented with a left renal mass that had been incidentally discovered on ultrasonography during a health check-up. She had no history of flank pain, gross hematuria, foamy urine, pyuria, dysuria, frequent urination, painful urination, urgent urination, or weight loss. Her past medical history and family history were unremarkable. A physical examination produced negative results for the lumbar zones. Routine laboratory test data were within normal limits. Abdominal ultrasonography revealed a 4.5 cm × 4.0 cm nodular solid mass with calcifications of heterogeneous density in the lower portion of the left kidney. The tumor was hypervascular and exhibited a massive internal hyperechoic area. An abdominal CT scan also confirmed a well-circumscribed calcified renal mass. No lymphadenopathy or ascites was discovered. The patient underwent a right radical nephrectomy and partial ureterectomy. At laparotomy, no gross evidence of metastatic spread or the involvement of other intra-abdominal organs was observed. The patient’s postoperative course was uneventful. She refused additional treatment, including radiotherapy or chemotherapy, except for postoperative surveillance with CT. At present, 113 months after surgery, the patient remains well, with no evidence of recurrence or metastasis.
On macroscopic examination, the non-encapsulated nodular mass, sized 4.5 cm × 3.5 cm × 3.0 cm, was located in the inferior pole of the kidney. It was well defined and black in color with moderately firm consistency (Fig. ). The lesion extended to but not through the renal capsule.
With the exception of abundant intracytoplasmic pigmentation, the lesion’s histological features were consistent with those of a clear cell renal cell carcinoma. Low-power observations indicated that the tumor was well demarcated from the renal parenchyma; lacked a fibrous capsule; and was composed of nests and cords of polygonal tumor cells, predominantly nests, and intervening delicate thin-walled fibrovascular septa (Fig. ). Cells in certain nests were focally discohesive, resulting in an alveolar structure. On high-power examination, approximately 55% of tumor cells contained abundant, clear and finely granular eosinophilic cytoplasm and distinct cell borders. Characteristically, the remaining 45% of tumor cells presented with variable quantities of intracytoplasmic brown pigment, ranging from finely dispersed small cytoplasmic granules to massive agglomerations (Fig. ). Tumor cells’ central round to oval nuclei had evenly distributed chromatin with occasional small, non-prominent nucleoli. Intranuclear eosinophilic cytoplasmic pseudoinclusions, which are exceedingly rare, were also present (Fig. ). There was inconspicuous nuclear pleomorphism, and the tumor was assigned a low nuclear grade. Mitotic figures were extremely uncommon. Intriguingly, scattered thick-walled blood vessels with the normal elastic content of arteries, as demonstrated via Gomori’s aldehyde-fuchsin staining, and unusual focal eccentric hyalinization were present throughout the tumor (Figs. and ). In addition, calcifications were readily observed and had frequently formed on hyaline nodules. Neither necrosis nor hemorrhage was observed. Histochemical staining analyses indicated that the brown pigment was negative in Prussian blue staining but was highlighted by Fontana-Masson staining and was completely bleached by potassium permanganate; these findings suggested that this pigment was melanin (Fig. and ). Immunohistochemical staining revealed strong and diffuse nuclear staining for TFE3 in the tumor cells (Dako, Carpinteria, CA, USA, 1:800) (Fig. ), which was performed as previously described []. Patchy and weak cytoplasmic staining for HMB45 (Dako, 1:500) was also observed (Fig. ). Ki-67 (Dako, 1:800) stained only approximately 3% of tumor cells (based on 1000 cells counted using Image-Pro Plus Version 5.1 C (Media Cybernetics, Silver Spring, MD, USA)). In contrast, staining for all other assessed immunomarkers, including AE1/AE3 (Dako, 1:1000), epithelial membrane antigen (EMA; Dako, 1:50), CK7 (Dako, 1:400), CK20 (Dako, 1:100), vimentin (Neomarkers, Fremont, CA, USA, 1:500), smooth muscle actin (SMA; Neomarkers, 1:800), desmin (Dako, 1:400), CD10 (Neomarkers, 1:200), PAX8 (ProteinTech Group, Chicago, IL, USA, 1:200), renal cell carcinoma (RCC) marker (Vector, Burlingame, CA, USA, 1:200), Cathepsin K (Abcam, Cambridge, MA, USA, 1:800), Melan A (Dako, 1:400), MiTF (Dako, 1:150), S100 protein (Dako, 1:1000), CD117 (Dako, 1:600), CD34 (Dako, 1:200), and DOG-1 (Santa Cruz Biotechnology, Santa Cruz, CA, USA, 1:400), was negative.
Break-apart FISH analysis was performed to detect TFE3 gene fusion in paraffin-embedded tissues with a probe consisting of 2 contigs that flank the TFE3 gene on Xp11.2 as previously described []. The distal contig consists of 3 bacterial artificial chromosomes (BAC) clones (RP11-344 N17, CTD-3028 J4, and CTD2522M13) labeled with Spectrum Orange, and the proximal contig consists of 2 BAC clones (RP11-57A11 and RP11211H10) labeled with Spectrum Green; from BacPac Resources at the Children’s Hospital Oakland Research Institute and Invitrogen. FISH analysis revealed the presence of a TFE3 rearrangement (Fig. ). The tumor was diagnosed as Xp11 TRC and staged as T1a.
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pmc-6090787-1
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A 10-year-old spayed female Alaskan malamute (body weight 38 kg) was admitted to the Konkuk University Veterinary Medical Teaching Hospital with persistent intermittent bilateral epistaxis of 5 days’ duration. Per the owner’s report, once initiated, the epistaxis did not stop for 2 h despite nasal plugging. During the initial physical examination, the dog was bright and alert, with no nasal bleeding. No purpuric spots were observed throughout the body, and an oral examination revealed no remarkable findings. Thoracic auscultation revealed no abnormal sounds in the lung and cardiac fields, and an automated oscillometric blood pressure measurement revealed mild hypertension (systolic blood pressure 148 mmHg). However, abdominal palpation revealed a large, round, firm, painful mass on the upper-middle abdomen.
Complete blood count, serum biochemistry profile, prothrombin time, and activated partial thromboplastin time analyses were performed to rule out coagulapathies, polycythemia, and thrombocytopenia as causes of epistaxis (Table ). The complete blood count revealed neutrophilic leukocytosis (white blood cells 42.97 × 109 cells/L; reference range 6–17 × 109 cells/L) and anemia (hematocrit 30%; reference range 37–55%, hemoglobin 8.7 g/dL; RI 12–18 g/dL). Serum chemistry revealed mildly elevated alkaline phosphatase activity (378 U/L; reference range 15–127 U/L) and hypoalbuminemia (2.6 g/dL; reference range 2.9–4.2 g/dL). The results of coagulation tests were within reference limits (activated partial thromboplastin time 9.6 s; reference range 14–18 s, prothrombin time 8.2 s; reference range 6.2–8.2 s).
Thoracic radiography revealed a mild broncho-interstitial pattern in the overall lung field and a normal cardiac size. An abdominal ultrasound examination revealed a splenic mass with a heterogeneous appearance and irregular but encapsulated borders.
The dog’s owner elected to pursue a rhinoscopy and computed tomography (CT) of entire body. Following anesthetization of the dog, the rhinoscopy was performed using flexible endoscopes (Fig. and ). A retroflexed view of the bilateral nasal choana revealed no remarkable findings except an engorged vessel (Fig. and ), and CT images revealed a normal nasal passage and intact cribriform plate. However, an abdominal CT scan detected a massive, continuous splenic mass measuring 13.2 cm × 13.5 cm × 8.4 cm in the mid-abdomen, as well as diffuse contrast-enhancement of the heterogeneous splenic parenchyma and an irregular margin (Fig. ). A thoracic CT scan indicated well-circumscribed, contrast-enhanced miliary nodules on the left cranial lung lobe, consistent with lung metastasis (Fig. ). The patient was hospitalized, during which time an episode of intractable and pulsatile epistaxis occurred without cessation for more than 1 h, despite nasal plugging. This episode caused the dog to become slightly agitated, with a temporary severe increase in blood pressure (systolic blood pressure 180–250 mmHg).
The owner consented to a surgical excision of the splenic tumor, and the dog underwent a surgical exploration of the abdominal cavity while in the dorsal recumbent position. A large (16 cm × 14 cm × 10 cm) mass at the tail of the spleen was found to attach to the greater omentum. The involved section of the greater omentum was resected, and a total splenectomy was performed. The abdomen was lavaged with warmed sterile saline and closed routinely. The dog recovered uneventfully with routine postoperative antibiotics. Histopathologically, the resected spleen comprised pleomorphic neoplastic cells; these included both round histiocyte-like cells and spindle cells with occasional multinucleate cells and mitotic activities ranging from one to four mitoses per high-power field (Fig. ). An immunohistochemistry stain for vimentin yielded a positive result (Fig. ), consistent with the mesenchymal origin, and the main differential diagnosis of spindle cell squamous carcinoma was excluded. A diagnosis of MFH was made based on the histologic and immunohistochemical findings.
Butorphanol (0.1 mg/kg intravenously every 8 h) was provided as postoperative analgesia for the first 24 h. Although intermittent minor episodes of epistaxis and transient hypertension occurred during the 7 days after splenectomy, the significant epistaxis had disappeared by the 5-month follow-up. Based on the histopathological findings, we recommended that the dog undergo adjuvant chemotherapy, but the owners refused. Five months after the first visit, however, the patient presented with acute anorexia and abdominal pain. Although her blood pressure was within normal limits (systolic blood pressure 125 mmHg), a complete blood count revealed neutrophilic leukocytosis (66.7 × 109 cells/L), anemia (hematocrit 26%, hemoglobin 9.5 g/dL), and thrombocytopenia (platelets 136× 109 cells/L; reference range 200–500 × 109 cells/L). A serum chemistry analysis detected elevated hepatic enzyme levels (aspartate aminotransferase 84 U/L, RI 0–50 U/L, alkaline phosphatase activity 1260 U/L, gamma glutamyl transferase 14 U/L; reference range 0–7 U/L) and mild hypertriglyceridemia (173 mg/dL; reference range 10–100 mg/dL). An abdominal ultrasound revealed multiple nodules and masses of various sizes, shapes, and echogenecities that were spread diffusely throughout all liver lobes, leading to the diagnosis of metastatic liver masses and nodules. However, the owner declined further examinations and therapy because of the poor prognosis, and the patient was discharged at the owner’s request. The dog died 3 days later at home; however, the owner unfortunately refused a necropsy.
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pmc-6090825-1
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A 43-year-old right handed woman presented in 2006 with difficulty writing, impaired dexterity of the right hand, and mild unsteadiness with walking. Her sole initial symptom was difficulty writing with the right hand and with time, other dexterous movements with the right hand became affected. Her left hand was asymptomatic. On examination, she scored 30 on the MMSE. There was a subtle dysarthria. She walked well except for a slight tendency to veer to either side. There was mild bilateral upper extremity dysmetria and mild ataxia with heel-to-shin. With the hands outstretched, as well as when performing movements with the right hand (especially writing), the proximal and distal thumb flexed involuntarily into the palm. When attempting to write, there was flexion of the right thumb, extension of the wrist, and excessive flexion at the PIP joint of all fingers. She did not notice any sensory tricks for dystonia and did not have mirror dystonia. Her reflexes were 2+ in the upper extremities and 3+ in the lower extremities. Brain MRI demonstrated cerebellar and brainstem atrophy.
Her mother was reported to have a progressive gait disorder, presenting with unsteadiness, hand clumsiness and dysarthria. We later learned that her mother had been diagnosed with SCA2 with 37 CAG repeats. Prior to death, her mother had a resting tremor, nystagmus, and cervical dystonia. Our patient’s maternal grandmother, two of three siblings and several maternal uncles and cousins were all similarly affected; a total of ten individuals were considered symptomatic in a three generation pedigree (Fig. ).
The patient was not seen again until eight years later, at age 51. During that time, there had been only mild progression of cerebellar symptoms but the dystonia of the right hand continued to worsen to the extent that she was barely able to write and the dystonia also impaired many other tasks with the right hand. On examination, dysarthria remained mild. There was severe writer’s dystonia (Additional file 1: Video 1) with excessive flexion of the thumb and all digits, poor control of the pen, and extension of the wrist. With the hands outstretched there was flexion of the right thumb into the palm and mild extension of the first finger at the MCP joint. There was moderate dysmetria with finger-to-nose on the right. There was slight dysdiadokokinesia of the left hand and mild dysmetria with finger-to-nose on the left. There was mild gait ataxia. Although the saccades were not overtly slow, when making saccades between horizontal targets, they were almost always performed with a simultaneous blink, which can be sign of subtle slowing or difficulty in initiation []. There was no significant improvement with 600 mg per day of levodopa, the maximally tolerated dose of trihexyphenidyl, as well as botulinum toxin. As such, in 2015, at age 52, she underwent deep brain stimulation (DBS) of the left globus pallidus internus (GPi), resulting in significant improvement in the dystonia. The monopolar Medtronic device achieved optimal settings at: Lead C + 0-, frequency of 165 Hz, voltage of 2.4 V and PW of 450 μs.
When seen 2 years after DBS (Additional file 2: Video 2), there was much less spontaneous flexion of the right thumb into the palm with the arms outstretched. Although not shown on the video, there was marked improvement in her ability to write within the first several months following DBS. Compared to pre-operatively, she performed finger-to-nose better on the right suggesting that dystonia rather than ataxia was the main cause of impairment prior to DBS. The dysmetria of the left upper extremity had progressed but there was still only mild dysdiadokokinesia bilaterally. The gait ataxia had progressed but she was still ambulatory without an assist device and reported no falls. She was unable to tandem walk.
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pmc-6090844-1
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A 70-year old man presented at his hematologist’s outpatient clinic on July 12th 2016 with 3 weeks of intermittent fever, myalgia, headaches, tinnitus and an exanthema, which had started under his left eye. His history revealed a mantle cell lymphoma (a B-cell non-Hodgkin’s lymphoma) in 2010, which was initially treated with R-CHOP (rituximab, cyclofosfamide, doxorubicine, vincristine and prednisolone), high dose cytarabine, and autologous stem cell transplantation. After achieving a complete response, he developed progressive disease in 2015, which was treated with R-CHOP. A second complete response was achieved, and he continued to receive rituximab maintenance every other month until July 2016. For his current symptoms, he was treated with azitromycin. A consulting dermatologist observed generalized nummular to palm-sized non-pruritic erythematous macules, sparing the foot soles, which was considered either a drug eruption or a para-infectious skin reaction. An abdominal skin biopsy revealed a non-specific chronic perivascular dermatitis, deemed consistent with a hypersensitivity response. A PCR on blood was negative for EBV and CMV, and a nasopharyngeal swab was negative for respiratory viruses. His next dose of rituximab was delayed until July 18th, at which point his erythema had resolved with only minor myalgias remaining. From July 22nd the patient started to experience a right-sided headache with right-sided rhinorrhea and a tearing eye, tinnitus of both ears and a different sensation for taste, as well as relapsing (sub)febrile episodes. From August 1st, he noted a left facial palsy, and on August 23rd the patient was admitted to the neurology ward with progressive symptoms. By this time, he had developed an unsteady broad-based gait, a mild action tremor, mild apathy, dysphagia and hearing loss. He had an erythema on his wrists and left elbow and had lost 6 kg. An MRI of his brain revealed periventricular white matter lesions, and pathological enhancement of multiple cranial nerves (bilateral trigeminal and vestibulocochlear nerves and right hypoglossal nerve, Fig. ). These findings raised suspicion of a relapse of mantle cell lymphoma in the central nervous system, for which a diagnostic lumbar puncture was performed, and intrathecal prednisone combined with methotrexate was administered. A peripheral leukocyte differentiation was normal besides a lymphopenia (0,30 × 109/ml), while total IgA (0.44 g/l), IgM (< 0.181 g/l) and IgG (2.92 g/l) were reduced (Table ). The CSF showed 279 leukocytes/μl containing 60% non-clonal T-cells and only 0.001% non-clonal B-cells, and elevated protein (2.55 g/L) (Additional file : Table S1). Five days after the lumbar puncture and intrathecal chemotherapy the patient deteriorated with nausea, a nystagmus and progression of the unstable gait. An MRI was repeated and showed a small increase of basal and cerebellar leptomeningeal enhancement, as well as recent focal ischemia in the right medulla oblongata. Additional body CT and a PET-CT did not reveal lymphoma or pathological FDG absorption. Repeated cerebrospinal fluid (CSF) analysis including immunophenotyping did not reveal malignant cells, prompting a search for an alternative diagnosis.
A skin biopsy excluded sarcoidosis. Furthermore, the CSF was negative for all performed microbiological assays (Additional file : Table S2) and PCRs were negative for Borrelia burgdorferi sensu lato (s.l.), the tick-borne relapsing fever genus, as well as a species-specific PCR for Borrelia miyamotoi (Table , Additional file : Supplemental methods). Serology was negative for HIV and Treponema pallidum (Additional file : Table S2), and Borrelia IgM and IgG in serum were negative (Liaison, Diasorin S.p.A., Saluggia, Italy) (Table ). The patient mentioned regular walks with his dog in the woods, but did not remember any tick bites.
During his admission, spontaneous resolution of symptoms made the patient refuse a brain biopsy and he was discharged on September 22nd without definite diagnosis. Due to a relapse of dysarthria, dysphagia, cognitive decline, ataxia and a novel paresis of the right facial nerve he was re-admitted October 5th. While a brain biopsy was already planned, a lumbar puncture was repeated and a PCR on B. burgdorferi s.l. turned out weakly positive (Ct value 37), confirmed by sequencing and PCR in a second laboratory. The patient was diagnosed with neuroborreliosis and was treated with ceftriaxone 2 g/d intravenously for one month. After an initial worsening of symptoms within the first two days, he experienced a recovery and was able to continue the final two weeks of treatment in a home care setting. Finally, minimal dysarthria and dysphagia were the only remaining symptoms after treatment.
After establishing the diagnosis of neuroborreliosis in this patient, we were interested whether other serologic tests would have been able to establish the diagnosis, or that this patient was truly seronegative (which is extremely rare). We also compared serology during (4–11-2016) and after treatment (19–01-2017). Serology was negative in the Enzygnost IgM and IgG ELISA (Siemens Healthcare Diagnostics GmbH, Marburg, Germany), C6 peptide ELISA (Immunetics Inc., Boston, MA, USA), and Liaison IgM in all sera, while the Liaison IgG became equivocal in intra- and post-treatment sera. Interestingly, both IgM and IgG blots (Mikrogen GmbH, Neuried, Germany) were negative, with only an IgG p41 band positive in all tested serum samples. A retrospective PCR on the skin biopsy failed to detect Borrelia DNA.
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pmc-6090845-1
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A 44-year-old Japanese woman with a 6-year history of insulin-dependent diabetes mellitus and an 11-year history of central diabetes insipidus presented with a pain in the genital area worsening over 2 weeks, general fatigue, and loss of appetite. Two months earlier, patient underwent a urinary catheter insertion as a management for urinary frequency, but it was removed during the previous hospital stay, a month before her recent hospitalization, for possible urinary infection. She has had frequent hospital admissions (6 times/year) and was hospitalized 3 months before her recent admission because of edema of the pelvic area and lower limbs. The patient developed diabetes mellitus after undergoing total pancreatectomy for nesidioblastosis, a surgical procedure which involved the removal of patient’s pancreas including the spleen and gallbladder. Her sister was also diagnosed with idiopathic central diabetes insipidus; thus, a family etiology was suspected. The patient had a surgery for suspected tongue cancer 2 years ago and was also suspected of non-alcoholic steatohepatitis with episodes of hepatic encephalopathy. Although she was on multiple medications including subcutaneous insulin injections and desmopressin tablets, her glycemic and hydration status were poorly controlled.
Four days prior to the present admission, she visited a gynecologist for her inguinal pain. No uterine tenderness or exudate was observed, and she was prescribed gentamicin and lidocaine ointments for possible local infection. She developed edema in the pelvic area with loss of appetite, and her home doctor consulted the university department 1 day before the present admission.
Upon admission, the patient appeared weak but was alert and had low-grade fever (37.4 °C) under a regular use of acetaminophen (1500 mg/day) and diclofenac (75 mg/day). Her blood pressure was not significantly different from previous measurements (88/42 mmHg) but a sinus tachycardia (heart rate 125/min) was noted. She complained of continuous abdominal pain and tenderness in all four quadrants. No abdominal guarding or rigidity was observed, but she had severe edema in the pelvic and bilateral femoral areas without necrotic skin discoloration. Laboratory investigations revealed a white cell count of 16,310/μL with neutrophilia (90.8%), elevated C-reactive protein of 22.18 mg/dL, and no serum sodium or potassium abnormalities. Serum aspartate aminotransferase and alanine aminotransferase were elevated at 466 U/L and 148 U/L, respectively. The patient’s international normalized ratio was high (2.26), but disseminated intravascular coagulation score did not meet the criteria. The patient’s HbA1c level was 8.8%, and blood sugar at admission was 316 mg/dL. She had low serum albumin concentration (1.7 g/dL), elevated serum ammonia concentration (154 μg/dL), and elevated lactate level (10.3 mmol/L). No ketonuria was noted, but significant pyuria was observed.
The abdominal ultrasound was unrevealing; thus, an intravenous treatment with ceftriaxone (1 g every 8 h) was initiated empirically after obtaining the blood and urine culture samples. A CT scan performed the following morning revealed the presence of air in the soft tissue of the inguinal and pelvic areas, such as pectineal and psoas major muscles (Fig. ). Immediate infectious and surgical consultations were made, and the antibiotics were changed to meropenem (1 g every 8 h), vancomycin (1 g every 12 h), and clindamycin (600 mg every 8 h). Gram-positive cocci and gram-positive rods were found in the initial blood cultures. In the evening of hospital day 2, a surgical debridement of the extraperitoneal pelvic tissue with colonostomy was performed, and the CT image after the operation suggested a complete resection of the affected tissue. However, hypernatremia (a serum Na concentration of 160 to 170 mEq/L) ensued as the use of nasal desmopressin could not effectively control the patient’s central diabetes insipidus after the operation.
No bacteria could be cultured from the debridement tissues (Table ). The blood culture isolates were finally identified as Streptococcus constellatus using superoxide dismutase A sequencing and C. ramosum by 16S ribosomal DNA sequencing []. The minimum inhibitory concentrations (MICs) of various antibiotics were tested using Etest for C. ramosum [] and the broth microdilution method [] except imipenem and meropenem (Etest) for S. constellatus (Table ). These results were interpreted using the Clinical & Laboratory Standards Institute M11-A8 document [] for C. ramosum and M100-S24 document for S. constellatus. Both strains were susceptible to penicillin, meropenem, and clindamycin. Despite the continued use of susceptible antibiotics and intensive care, disseminated intravascular coagulation and pancytopenia developed, and the patient died on hospital day 8.
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pmc-6090905-1
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A 55-year-old woman with no specific medico-surgical history presented at the emergency room with a 1-day history of squeezing epigastric abdominal pain. Patient also complained of a thick turbid yellowish discharge in the left inguinal area that was intermittently drained for some years.
Vital signs were normal range except for tachycardia (pulse rate, 110/min). Palpation of the abdomen revealed a wood-like hard mass in the left lower quadrant with minimal tenderness and no acute peritoneal signs warranting emergent surgery. A visible scar was noted in the left inguinal area without any discharge.
Initial laboratory testing revealed marked leukocytosis (white blood cells, 24,730 cells/mm3), anemia (hemoglobin concentration of 6.9 g/dL), elevated C-reactive protein (CRP) 32.05 mg/dL (reference range, 0–0.5 mg/dL), hypoalbuminemia (albumin, 2.5 g/dL); and normal AST/ALT and BUN/creatinine. CT scan of the abdomen-pelvis revealed a microperforation of the sigmoid colon, abscess in the left lower quadrant, a hepatic lesion and bilateral hydronephrosis. Furthermore, there was a large infiltrating heterogenous hyperattenuating conglomerated mass invading the urinary bladder, left adnexa, sigmoid, left inguinal canal and left pelvic wall area (Fig. ). Ultrasound revealed an intra-uterine device (IUD) (Fig. ). All these findings initially raised a suspicion of malignancy such as advanced cancer of the colon or ovary with liver metastasis. Despite the rarity of the disease, infectious diseases such as actinomycosis were not excluded because of the IUD found on ultrasound. Colonoscopy or percutaneous needle biopsy was not performed for accurate diagnosis due to suspected colon perforation and the small bowel enclosed mass.
Since the patient showed minimal peritoneal irritation and stable vital signs, and extensive organ resection was expected due to invasion of bladder and ureters, treatment was initially conservative rather than primary debulking surgery. The antibiotic regimen was always determined based on the infectious disease diagnosis after hospitalization. Parenteral antibiotics (ceftriaxone+metronidazole+azithromycin) and fasting with total parental nutrition were prescribed for sigmoid perforation. Because there was a huge left abdominal abscess (11X8X3cm) that could spread to other spaces and cause generalized peritonitis, the imaging-guided percutaneous abscess drainage was performed.
After 10 days of conservative treatment, a repeat CT scan of the abdomen-pelvis showed improvement in abdominal abscess and liver lesion. However, no remarkable change was detected in conglomerated mass invading pelvis. Furthermore, the finding of newly developed mechanical small bowel obstruction warranted surgery.
Exploratory laparotomy was performed for the removal of perforated colon, obstructive small bowel and organs involved. Abscess of the sigmoid colon involved the uterus, adnexa, loop of small bowel and distal colon with severe adhesion between the mass and pelvic organs including the uterus, small and large bowels, and bladder. The abscess compressed the left ureter and caused ureteral dilatation. En-bloc excision of the mass was performed using Hartmann’s procedure, bilateral salpingo-oophorectomy, small bowel resection and appendectomy. The gynecologist decided not to resect uterus because of severe fibrotic adhesion in the pelvis and transvaginal IUD removal failed repeatedly due to severe adhesion.
Since the frozen section excluded malignancy, a double J catheter was inserted into both the ureters without resection. Although Actinomyces spp. failed to grow in preoperative cultures, postoperative permanent histology confirmed a definitive diagnosis of colonic actinomycosis, which showed the granular colonies of bacteria, commonly termed sulfur granule, with aggregates of filamentous bacteria and neutrophils (Fig. ) and abscess with invasion into the uterus and ovaries.
After surgery, the parenteral antibiotic regimen was changed to tigecycline, amikacin, metronidazole and Penicillin G. Three days after surgery, bowel movement was restored and vital signs were stabilized, which decreased the abdominal pain. The patient made an uneventful recovery and was started on a 6-month course of penicillin. At 1-year follow-up, the patient was well and free from disease.
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pmc-6090917-1
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We present the unique case of a 28-year-old male patient displaying a complex clinical picture with mental retardation and various behavioural problems since birth. Symptoms of the autism spectrum comprising difficulties in social interaction and communication are reported since childhood. Additionally, he suffers from auto-aggressive tics in terms of beating himself with objects against his head and lower jaw, head movement tics and simple vocal tics. Striking dysmorphic features are not evident.
Except a one-time bleeding in week 20 of gestation, pregnancy had been without any complications. No infections, medication, smoking, or intake of alcohol or drugs during pregnancy was reported. The patient was delivered in week 40 of gestation with the help of a ventouse due to irregular cardiac activity. During delivery there were minor signs of birth asphyxia. Birth weight was 2.900 g (25th percentile), birth length 51 cm (50th percentile) and head circumference 33 cm (<3rd percentile). During infancy a prominent frontal fissure was conspicuous. A premature ossification of the sagittal fissure could not be detected.
The patient showed psychomotor retardation: he walked alone only by 26 months and was not able to sit alone falling over to one side without shoring up even at the age of 2. Furthermore, tics in terms of eye blinking as well as a muscular hypotonia were described. The patient’s parents reported early autistic features such as difficulties in social communication and interaction with avoiding eye contact and poor interest in social interaction. Development of speech was delayed (first words with 18 months). He refused body contact and demonstrated stereotypic patterns of behaviour such as filling bowls without showing any variations.
When examined at the age of 27 months, the patient presented some special facial features such as synophrys, epicanthus, modelled ears, a deep joined thumb and microcephaly. His weight was 10 kg (3rd percentile), his length 88 cm (25th percentile) and his head circumference 46 cm (2 cm above the 3rd percentile). The patient only spoke a few words and never a whole sentence. Mostly, he only repeated the words he had heard before in terms of an echolalia. A considerable general delay of development with severe perceptual disturbance and autistic traits was diagnosed.
Since the age of two years, the patient shows relevant aggressive symptoms such as throwing his head on the floor or biting into items. He needs extensive support concerning all activities of daily living and requires constant daily routines. During nursery school, auto-aggressive symptoms exacerbated in terms of head banging behaviour injuring his jaw and ears. At the age of 3 years, he started grinding his teeth. Later on, he presented head throwing movements against his left shoulder and banging of one row of his teeth against the other. There was a pattern of aggravation of the tic symptoms during stressful situations. He repeatedly showed refusal of meals and sleeping disturbances. At the age of 3 years, once there was a query febrile convulsion associated with an infection. Apart from that, there was no evidence for further seizures and clearly no epilepsy.
According to ICD-10 and DSM-5 classification, the clinical features of the patient are consistent with early infantile autism as well as with Tourette syndrome.
In the clinical examination, the patient presented with cauliflower-ears as a result of his head banging behaviour and subsequent repetitive ear injuries as well as various injuries of all kinds and healing stages. Dysmorphic features or additional external malformations were not noted, there was no evidence of internal abnormalities (heart, eye, inner ear) either. The patient showed a preserved ability of speech comprehension with rare speech production.
Our patient is the first son of non-consanguineous healthy German parents. His younger brother (24 years old) was diagnosed with an autism spectrum disorder (Asperger syndrome). There was no evidence of syndromal-secondary autism in the brother. He did not show intellectual impairment, dysmorphic signs or organic conditions such as a heart malformation or epilepsy. The mother’s paternal grandparents were cousins. They had five healthy children. In their further progeny three mentally retarded persons were reported, one granddaughter (died at the age of approximately 27 years) and two great-grandchildren (a boy and a girl). The exact diagnosis was unknown to our patient’s parents. Consanguinity of the retarded relatives’ parents was denied. One of the patient’s cousins (the son of his father’s brother) was said to suffer from clinically apparent dyslexia and perhaps an autism spectrum disorder. A disablement of a cousin of our patient’s father (son of his father’s sister) could not be specified further. An assessment of the father revealed no clinical characteristics, neither with respect to striking dysmorphic features, the occurrence of tics nor with respect to autistic symptoms. Further relatives with neurodevelopmental disorders were not reported on the father’s side of the family.
Conventional R-banded karyotyping of the patient was performed according to standard protocols with a resolution of approximately 500 bphs and revealed a structurally and numerically normal male karyotype (46,XY) in all 21 metaphases examined.
Furthermore, the genomic DNA of the patient was examined by microarray-analysis (CytoSureTM Constitutional v3 Array 180 k, OGT (Oxford Gene Technology)) according to the manufacturer’s instructions. After hybridization, the array was scanned with the SureScan Microarray scanner (Agilent), the results were analyzed using CytoSure interpret software v.4.9 (OGT) against the Genome Reference Consortium human genome GRCh37 (hg19). Molecular karyotyping revealed a heterozygous deletion of approximately 719 kb (267 contiguous oligonucleotides) out of the chromosomal region 2q24.3 (karyotype according to ISCN (International System for Human Cytogenetic Nomenclature) (2016): arr[GRCh37] 2q24.3(165471418_166190427)× 1). The deletion encompasses the five genes GRB14 (exon 1 to intron 2–3), COBLL1, SLC38A11, SCN3A and SCN2A (exon 1 to intron 14–15) listed in OMIM (Online Mendelian Inheritance in Man) (Fig. ). With FISH (fluorescence in situ hybridization) analysis using probe RP11-150F4 (Empire Genomics) located at 2q24.3 the deletion could be confirmed. There is no evidence in literature that the microdeletion we detected is a common variant in the Caucasian population [, ]. “Orphanet” states a prevalence rate of a 2q24 microdeletion < 1/1000000 (worldwide) ().
The subsequently performed chromosome analysis of the parents, FISH with RP11-150F4 included, showed a normal female karyotype in the mother and a normal male karyotype in the father with no evidence of a deletion or rearrangement at 2q24.3. Therefore, it can be asserted that the deletion in the patient is a de novo mutation. The patient’s younger brother (suffering from Asperger syndrome) was shown to have a normal male karyotype without a deletion or rearrangement at 2q24.3.
Magnetic resonance imaging conducted in 2014 showed no abnormalities of the patient’s brain. In 1991, a detailed investigation was performed with a metabolic screening of serum, urine and cerebrospinal fluid revealing normal results. In the EEG, there was a slow baseline activity without any epilepsy suspicious potentials. A proton-spectroscopy showed no abnormalities of N-acetyl-aspartate, choline and phosphor-creatinine levels. An ophthalmologic investigation exhibited no deviations. In 1997, gastrointestinal passage was unsuspicious, and in an X-ray of the brain no stenosis of the sagittal fissure was confirmed.
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pmc-6090942-1
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A 60-year-old male was referred to our center with cecal inflammation found during a screening colonoscopy. He did not complain of any abdominal discomfort, such as pain, nausea, vomiting, and diarrhea. He had no past medical history except surgery for an inguinal hernia. He was afebrile with stable vital signs. On a physical examination, there was no tenderness in the abdomen.
Colonoscopy performed at the local clinic revealed a hyperemic inflammatory lesion in the cecum around the appendiceal orifice. Because the lesion felt very hard during the colonoscopic biopsy, it was likely associated with a long period of inflammation. An abdominal CT performed after the colonoscopy in the clinic revealed a 5 × 2.5 × 4 cm mass-like lesion in the cecum around the ileocolic (IC) valve and appendiceal orifice; it had heterogenous enhancement and an ovoid calcification (8 mm) at its center, which was suspected to be an appendicolith (Fig. ). The lesion was accompanied by appendicitis, which was identified based on appendiceal dilation (9 mm) and haziness of the periappendiceal fat. The main lesion seemed to be an inflammatory mass rather than a malignancy because it appeared to be an extraluminal or extramucosal lesion. Ultrasonography revealed a diffuse area of wall thickening (4.9 × 2 × 2.5 cm) in the cecum around the appendiceal orifice that was suspicious for an inflammatory mass or benign mass. The 8-mm calcification identified on the previous CT was probably in the mass-like wall thickening rather than in the appendix. The appendiceal lesion seemed to be a mucocele rather than acute appendicitis because there was no periappendiceal inflammation, a thin wall, and no direct tenderness. Colonoscopic biopsy of the cecum showed mild chronic nonspecific colitis with mucosal lymphoid follicles.
The patient underwent laparoscopic partial cecectomy. In the surgical field, there was a large mass in the appendiceal orifice (Fig. ). There did not appear to be an intussusception involving the appendix or other intestine including the ileum. The cecum was partially resected, with care taken to preserve the IC valve. After the resection, it was thought that the ileocolic valve had enough remaining lumen to allow the passage of bowel contents. Final histopathological analysis of the surgical specimen revealed an appendiceal intussusception without any mucosal lesion of the appendix (Fig. ). Three days postoperatively, the patient had abdominal distension without gas passage. An X-ray revealed a small bowel obstruction pattern. An L-tube was inserted, and the patient remained NPO. Despite conservative management, the symptoms were ongoing, and an abdominal CT was performed 6 days after surgery. This CT showed abrupt narrowing of the terminal ileum with a small bowel obstruction and stenosis of the IC valve. Emergency laparoscopic exploration was performed. In the surgical field, small bowel dilatation was present just proximal to the IC valve and raised concerns for IC valve stenosis. Therefore, a laparoscopic ileocecectomy was performed. The patient was discharged 11 days after the second surgery without a significant postoperative complication. We carried out the follow-ups regularly with CT, and he remained asymptomatic for 2 years postoperatively.
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pmc-6090945-1
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A 67-year-old man was found to have a dilatation of the common bile duct (CBD) (19 mm) during a medical examination at 62 years of age. The dilatation of the CBD subsequently progressed (26 mm), and he was admitted to our hospital for surgical treatment. Abdominal computed tomography revealed a dilatation of the CBD with no tumor or stone. Magnetic resonance cholangiopancreatography revealed a dilatation from the common hepatic duct (CHD) to the middle bile duct (Fig. ) with PBM (Fig. ). Endoscopic retrograde cholangiopancreatography (ERCP) from the papilla of Vater revealed the pancreatic main duct via the pancreatic branch duct (Fig. ). PBM with dilatation of the CBD (26 mm) and incomplete PD were revealed (Fig. ). Figure shows a schema of this case with dilatation of the CBD and PBM, and incomplete PD in which the ventral pancreatic duct joined the dorsal pancreatic branch duct was observed.
We planned an extrahepatic bile duct resection and hepaticojejunostomy because of high risk of malignant transformation. Laparotomy was performed by a right hypochondrium incision. Taping and transection of the bile duct without dilatation on the pancreatic side were performed, and thereafter, two orifices of the common channel and ventral pancreatic duct were ligated (Fig. ). The transection line of the CHD without dilatation was identified using cholangiography (Fig. ), and then, the CBD was resected. The level of amylase in the bile was 7217 IU/L, and a histological examination of the CBD showed an inflammatory change of CBD, not a malignant transformation.
The postoperative course was good and uneventful, and the patient was discharged from the hospital on postoperative day 9. The patient is doing well at 1.5 years after surgery.
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pmc-6090953-1
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An 80-year-old Arabian female presented to our hospital with a 2–month history of swelling over the right eyebrow, pain, proptosis of the right eye and diplopia (Fig. ). Physical examination revealed a 2 cm ill-defined painful mass over the right eyebrow. The patient complains of double vision looking to the left. Computed tomography (CT) of the right orbit demonstrated an ill-defined, homogeneous, contrast-enhancing mass attached to the medial rectus. As a space-occupying orbital lesion, a lymphoma or a sarcoma was suspected. As a result, a biopsy was performed. On microscopic examination, the tumour was composed of interlacing bundles of spindle cells with anisokaryosis and hyperchromatic nuclei (Fig. ). Some mitotic figures were observed. Immunohistochemical study was not possible because neoplastic material has been exhausted. The conclusion was malignant spindle cells tumour, most consistently to sarcoma or sarcomatoid carcinoma. No lymph node or distant metastases were found. Subsequently, total exenteration of the right orbit was performed under general anaesthesia. Dilute adrenaline was injected to lessen bleeding generally abundant in this type of excision. Periosteum was incised right around the orbital rim and separated from the bone passing back towards the orbital apex. The eyeball, eyelids, appendages of the eye and periosteum were removed. Surgical specimen was addressed for pathological examination. At gross examination, the tumour appeared ill-defined, whitish and firm. It measured 4/2.5/1.5 cm. It was attached to the sclera without infiltration into eyeball. It infiltrated the upper eyelid (Fig. ). Microscopic examination revealed spindle cells forming disorganized fascicles. They have an irregular nucleus with vesicular chromatin and an eosinophilic cytoplasm. The mitotic index was 18 per 10 high-power fields. Adipose tissue and striated muscle infiltration was observed (Fig. ). Immunohistochemical panel used for initial work up of this high grade spindle cell neoplasm was desmin, smooth muscle actin (SMA), pancytokeratin AE1/AE3, epithelial membrane antigen (EMA), S100 protein and CD34. This panel served to rule out first leiomyosarcoma, rhabdomyosarcoma, sarcomatoid carcinoma and spindle cell melanoma. It revealed diffuse positive staining for pancytokeratin AE1/AE3, EMA and SMA and focal positivity for S100 protein (Fig. ). The lesion was immunonegative for desmin and CD34 (Fig. ). As pancytokeratin AE1/AE3, EMA, SMA and S100 protein staining was positive, we completed by second panel. It showed h-cladesmon, Melan-A and HMB-45 negative staining. These pathological and immunohistochemical findings suggested the diagnoses of myoepithelioma, epithelial myoepithelial carcinoma and myoepithelial carcinoma. Myoepithelioma was excluded because the tumour borders were infiltrative. Epithelial myoepithelial carcinoma is by definition composed of a biphasic arrangement of inner luminal ductal cells and outer myoepitheliaI cells. However, the tumour did not show bi-layered duct-like structures. The most appropriate diagnosis was myoepithelial carcinoma. The tumour was extending to upper and posterior surgical margins. Radiotherapy was then indicated. The patient was lost to follow-up until she developed local tumour progression 3 months after removal. The patient was again lost to follow-up and therefore did not receive any other treatment in our hospital.
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pmc-6090959-1
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A 40 year old Malay male was seen at the emergency department with 1 week history of left hypochondriac pain with concurrent abdominal distention. He also complained of loss of appetite and feeling lethargic for 1 month duration. He had no fever, nausea, vomiting, changes in bowel habits or any history of bleeding diathesis. There was no history of trauma. Neither there were any significant past medical history nor family history of malignancy. He was an active smoker for 20 years but denied any alcohol consumption or substance abuse.
On clinical examination, he was afebrile, with an elevated heart rate of 110 beats per minute and a blood pressure measurement of 121/79 mmHg. Patient appeared pale. Abdominal examination revealed enlarged, non-tender liver and spleen. There was no ascites or peripheral lymphadenopathy. Cardiovascular and respiratory examinations were otherwise unremarkable.
Haematological investigation revealed a low haemoglobin level at 6.4 g/dl. The patient had a white cell count (WCC) of 33.3 × 10^3 /uL and a platelet count of 568 × 10^3/uL. Differential WCC showed a predominant neutrophil count of 79.9%, lymphocyte count 8.9%, monocytes 9.6%, eosinophils 0.8%, basophils 0.8%, absolute neutrophil count of 25.63 × 10^3 /uL and absolute lymphocyte count of 2.95 × 10^3 /uL. There was an increase in lactate dehydrogenase levels (LDH) from 534 to 666 u/L. Peripheral blood film revealed leucocytosis with neutrophilia with no evidence of blast cells or atypical lymphocytes. Patient was reluctant to undergo a bone marrow aspiration and trephine biopsy. Abdominal ultrasonography demonstrated a large splenic collection. A contrast enhanced computerized tomography of the abdomen further revealed a large heterogenous splenic collection measuring 18 cm × 15 cm × 16.9 cm which was suggestive of a splenic haematoma [Fig. , and ]. There were no intra abdominal or pelvic lymph nodes enlargement. Based on computed tomography findings, a preliminary diagnosis of spontaneous splenic rupture was made. A surgical consult was obtained and an explorative laparotomy was performed on the patient. Intra operative findings showed a ruptured spleen with extensive adhesions to the omentum. No intra peritoneal lymph nodes enlargement were found. Splenectomy was then performed and subsequently, the patient was transferred to intensive care unit for close observation.
From a histological perspective, the gross appearance of the obtained specimen revealed an enlarged spleen with irregular outer surfaces. A cut section of the spleen showed a firm, cream coloured layer occupying almost entire spleen with large area of necrosis with splenic infarcts. There as minimal amount of normal looking parenchyma tissues at the peripheral aspect of the specimen. Further histological examination revealed a diffuse infiltration of malignant lymphoid cells, which exhibited irregular nuclear membrane with vesicular nuclear chromatin and prominent nucleoli. The adjacent splenic parenchyma showed a congested and expanded red pulp with infiltration by atypical lymphoid cells [Fig. ].
The histological report confirmed the presence of diffuse large B-cell non-Hodgkin’s lymphoma (NHL) via immunohistochemical testing. Immunohistochemical staining showed the cells to be positive to CD20 (diffuse), BCL-2, BCL-6 (> 30%), and MUM-1 (> 30%) and negative to CD3, CD10, Cyclin-D1, Tdt, CD30 and ALK. Ki67 proliferative index was > 80%. In accordance with the WHO classification of Lymphoid Neoplasm [, ], these findings were consistent with the diagnosis of a diffuse large B-cell non-Hodgkin’s lymphoma, non-germinal center B-cell (non-GCB) type. Further molecular studies to assess MYC / BCL-2 / BCL-6 translocation or rearrangement was not done as the fluorescence in situ hybridization (FISH) analysis was not available in our local laboratory settings. Thirteen days later, the patient was discharged with prophylactic meningococcal, pneumococcal and influenza vaccinations. He was referred to the haemato-oncologist outpatient clinic at a tertiary care centre for post - operative chemotherapy. Unfortunately, the patient did not turn up for subsequent follow ups, rendering it difficult to further document any information with regards to treatment response in this report.
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pmc-6090968-1
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A 58-year-old Caucasian man presented to the emergency department for acute abdominal pain. The abdominal pain was mainly in the epigastric area, was sharp in nature, with severity of 8/10, non-radiating, worsens with movement, and mildly improves with rest. The pain was associated with nausea and heaves. On review of systems he denied any constitutional symptoms (weight loss, fever, chills, weakness or fatigue), no cardiovascular, respiratory, neurological, musculoskeletal, hematological or endocrinological problems. Past medical history is only significant for hypothyroidism for which he takes levothyroxine. No previous surgeries done. Patient was not taking any medications except for Levothyroxine for hypothyroidism for the past 10 years. The only medication he received prior to presentation was amoxicillin/clavulanic acid as prophylaxis for a dental procedure (even though not indicated at that time) with dosage of 875 mg twice daily for a total of 10 days with his symptoms starting on day 9th of therapy and amoxicillin/clavulanic acid was discontinued on admission to hospital. On further questioning, patient recalled that several years ago he had similar abdominal pain that developed after taking amoxicillin/clavulanic acid but did not seek medical attention at that time and the pain resolved within few days while abstaining from food intake. He is a non-smoker, has never used recreational drugs, drinks only socially on certain occasions not exceeding twice a month and not exceeding 2 beers, 5% alcohol based, in one sitting (a total of 24 oz), and denies binge drinking. On admission, he was hemodynamically stable. His physical examination was noticeable for epigastric tenderness only. Laboratory studies revealed mild leukocytosis (white blood count (WBC): 13.5 × 109/L), increased levels of serum lipase > 600 U/L, amylase: 1220 U/L, and CRP: 19.6 mg/dL. Abdominal CT was notable for acute pancreatitis with no pseudocyst formation (Fig. ).
Based on clinical presentation and CT findings, patient was diagnosed with mild acute pancreatitis with Bedside Index of Severity in Acute Pancreatitis (BISAP) score of 0 (< 1% risk of mortality), which is characterized by the absence of organ failure and local or systemic complications. During his hospital stay, patient was managed with aggressive IV hydration and pain management with bowel rest of 2 days duration and significant improvement being noticed within 72 h after which patient was discharged home.
In order to identify the cause of his acute pancreatitis, extensive history and workup was done with the help of the gastroenterology team to eliminate the most common causes of pancreatitis. Magnetic resonance cholangiopancreatography (MRCP) and abdominal ultrasound and eliminated out the possibility of gallstones, biliary sludge, biliary ductal dilatation, or choledocholithiasis (Figs. and ).
Endoscopic ultrasonography was done as outpatient by the gastroenterologist on the case and ruled out biliary microlithiasis. Patient had no hypertriglyceridemia (his triglyceride (TG): 142 mg/dL), never had endoscopic retrograde cholangiopancreatography (ERCP), no hypercalcemia (his corrected calcium (Ca): 9.3 mg/dL), no steroids taken, no known malignancy, no infection, no trauma, no exposure to scorpions. The most plausible link for his pancreatitis was his use of amoxicillin/clavulanic acid prior to presentation given that he had a similar presentation when he took the same antibiotic several years ago but was not diagnosed with pancreatitis since he did not seek medical attention at that time. Additionally, patient denied intake of any other penicillin agents. Table summarizes our case’s timeline.
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pmc-6091004-1
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In October 2016, a 20-year-old female patient was referred to our outpatient clinic for persistent dry cough.
She reported that at the age of 16 months, because of the inhalation of food bolus, a bronchoscopy was unsuccessfully attempted and the episode resolved with a spontaneous expulsion of the foreign body. Thereafter, her medical history was characterized by recurrent bronchitis. At 11 y.o. she was diagnosed with allergic bronchial asthma (positive methacholine test and positive skin tests for both perennial and seasonal inhalation allergens such as dermatophagoides, cat, horse and pollens of grasses). The patient also reported a history of hypothyroidism and anorexia for which she had been admitted for a few months between 2013 and spring 2015 and fed by a naso-gastric tube.
At her first visit at our outpatients clinic, she complained of persistent irritating cough, which was accompanied by dysphonia in the last month. No dyspnea was reported. The pulmonary function tests showed: FEV1 of 2.88 L (85% of predicted), FVC 3.71 L (96% of predicted) with a FEV1/FVC ratio of 77% and an FEV1/PEF ratio > 8, as in cases of intrathoracic obstruction; furthermore, analysis of the flow/volume curve (Fig. ) showed a flow plateau of the expiratory curve with an armpit at low pulmonary volumes. Chest x-ray showed no pathological signs. A cycle of inhalation therapy with LABA and inhaled steroid was started. After 1 month of therapy, the patient was still complaining dry cough. A new spirometry showed findings similar to the previous one (Fig. ), thus confirming an intrathoracic obstruction.
The diagnosis was made after performing a chest CT with contrast medium. The CT demonstrated the presence of a complete double aortic arch (DAA) imprinting both the esophagus and the trachea, causing a greater narrowing in the expiratory phase (Fig. ).
Then, the patient was referred to the cardiothoracic surgery unit for evaluation for a surgical DAA correction. During the hospitalization were performed: a contrast transthoracic echocardiography (showing a possible minimum pulmonary arterial-venous shunt), a fiberbronchoscopy (confirming ab extrinseco tracheal compression) and an esophageal radiography with contrast medium (showing a dilated upper esophagus followed by 30 mm of narrowed caliber). Given the important anatomical impairment and the poor quality of life due to the symptoms, a surgical correction of the DAA was performed, obtaining improvement of the symptoms.
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pmc-6091012-1
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Amy is a 12.5 year old girl in grade 7 with a history of mild phobias but no previous social anxiety, who lives with two working parents in a middle class home. At 11 years of age she became terrified after witnessing her dog choke on a piece of rawhide bone. Thereafter, she started to worry obsessively about choking. She started to prefer soft foods, to take tiny bites and to chew her food many times before she would swallow. She took a very long time to eat, wouldn’t eat lunch or snacks at school, and became increasingly slow and restrictive in her eating. Amy was admitted to hospital at a body mass index (BMI) of 13.5 kg/m2 (83.0% of TGW) and diagnosed with ARFID. Her case history was most in keeping with the ARFID-aversive subtype. She spent 38 days in hospital, receiving family therapy and psychoeducation and support for parents, who were very resistant to, and anxious during, passes out of the hospital environment. With inpatient family therapy sessions, parents were empowered to take control of nutrition on passes and to ensure that Amy finished everything. Her treatment was initially augmented in hospital with olanzapine 2.5 mg at bedtime, and as she approached her TGW she was started on fluoxetine 10 mg/day to help manage her severe anxiety.
Amy was discharged home at 100% of her TGW, but with an ongoing fear of choking that continued to impact her eating (e.g. still taking very small bites and a long time to eat, and avoiding foods that she feared she might choke on). Outpatient family therapy was augmented with cognitive behavioural therapy (with parents present). Amy developed and worked on a hierarchical ladder of feared foods, and was encouraged to practice eating using a timer, to take bigger bites, and to eat with peers at school. Although Amy did not describe social anxiety at school or worrying about schoolwork, she did develop a fear of going out with parents. Her fluoxetine was increased to 20 mg/day as a consequence of Amy’s increased anxiety. The family described the increased dose of fluoxetine as helping significantly with Amy’s fear of going out, and opted not to increase the dose further. Over the next 5 months (and nine therapy sessions), Amy recovered completely from her ED and fear of choking and was able to reach a healthy weight and to continue to grow, at which point she was discharged from care from the ED team.
Weight at start of family therapy: 30.3 kg.
%TGW at start of family therapy: 83%.
Weight at end of family therapy: 43.2 kg.
%TGW at end of family therapy: > 100% (Fig. ).
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pmc-6091012-2
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Susan is a 10.9 year old girl who lives at home with 2 professional parents and a younger sister. She was described as a very anxious child; past medical history was notable for a history of frequent stomach pains of no known medial cause, and school refusal, though no eating or growth problems. Susan developed repeat episodes of viral gastroenteritis over a two week period which left her convinced that resuming eating had caused her gastro-intestinal symptoms. As a result, over the next few months she progressively ate less and lost weight. She underwent a full medical work-up but no pathology was identified. Her parents progressively eliminated foods that could potentially exacerbate her symptoms of abdominal pain and nausea, but with limited effect. She was admitted to the pediatric ward weighing 75.8% of TGW (BMI 11.8 kg/m2). Given her refusal to eat, she was initially re-nourished with liquid nutrition (Ensure) via nasogastric (NG) tube, but weight gain was very slow and difficult. She refused to eat or drink, would kick and scream and become hysterical whenever food was presented, and screamed throughout the duration of her NG feeds. One month after admission there had been minimal progress, so the ED team was consulted and family therapy was initiated. Her case history was felt to be in keeping with ARFID-aversive subtype.
In addition to family therapy, Susan was treated with olanzapine over the course of her admission to help with her severe agitation and anxiety; she was started on 2.5 mg at night and the dose was gradually increased to a maximum of 2.5 mg in the morning and 5 mg at night.
Both parents were convinced that this must be a medical problem. The therapist worked to empower and educate parents, lift guilt and blame, and also to raise anxiety about the need for parents to take control of the nutrition and help their daughter to eat. The therapist explained the ‘vicious cycle’ of how weight loss leads to more anxiety, a ‘smaller’ stomach, more stomach pain, and the need for more nutrition to reach IBW. Thus, the only ‘way out’ is with renourishment and weight gain. Parents would try to be firm and help Susan to take the nutrition, but Susan would kick and scream hysterically and refuse, crying that her stomach hurt too much and she couldn’t eat. Parents and Susan were helped to see that “not eating is not an option;” it is a mandatory part of treatment and the only way to recover. Susan was taught to use relaxation techniques and her mother was taught to support her, e.g. by providing soothing massage, empathy, and whatever helped Susan to tolerate taking the nutrition. Susan insisted that all that helped was walking. Thus, she would take her (mandatory) nutrition, crying and sobbing loudly, and then walk up and down the hospital halls. Gradually Susan’s intake increased, along with her weight, and the pain and hysterical sobbing lessened. Parents were encouraged to take Susan on as many passes as possible, which she initially resisted, saying that it hurt too much to eat at home. She and her parents were helped to see that change is stressful and that unless Susan was going to live in the hospital forever, she would need help with learning to eat at home. Gradually, passes became increasingly more successful, and weight increased. Susan was discharged home two months after admission, at 100% of her initial TGW.
Following discharge, Susan continued weekly family therapy sessions. Although her weight gain continued as she grew in height, she resisted numerous foods that she was fearful of eating for fear of abdominal pain. She also exhibited separation anxiety and school avoidance. Family therapy continued, cognitive behavioural therapy was also utilized, and gradually, food variety increased. Parents opted for home schooling. Fluoxetine 10 mg in the morning was started for severe anxiety, and the dose was titrated over the following months up to 40 mg/day. Concurrently, her olanzapine was tapered and discontinued. At the end of treatment (after 6 therapy sessions but with follow up by the AM physician over a total of 3.5 months), she was able to eat all foods, she could socialize and participate in sports, and she could eat without issue at restaurants (which previously caused anxiety as well).
Weight at start of family therapy: 26.7 kg.
%TGW at start of family therapy: 79%.
Weight at end of family therapy: 41.6 kg.
%TGW at end of family therapy: > 100% (Fig. ).
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pmc-6091012-3
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Ethan is a 13.1 year old boy with mild-moderate autism spectrum disorder (ASD) who lives on a farm with his father and grandparents. He enjoys school, where he attends a special class because of his intellectual disability. He had a history of “picky” eating but no history of low weight or growth problems. One day while riding, Ethan fell off his horse and injured his ribs. He experienced severe rib pain with swallowing. As a result, he limited his food intake to minimize the pain. As weight decreased, his restriction intensified and he became increasingly anxious about eating. Ethan was eventually admitted to hospital where he spent one month on the pediatric ward, had a full medical work-up, and was discharged home at a slightly lower weight than at admission. His history was in keeping with a mixed ARFID presentation: ARFID-limited variety subtype plus ARFID-aversive subtype. He was readmitted to a specialized ED inpatient unit in the weeks that followed (at 72% TGW). Father needed to work on the farm, so Ethan’s grandmother stayed in hospital with Ethan and worked with the family therapist.
Initially, Ethan would take very small amounts orally and then say he was too “full”. He would have temper tantrums when pushed to eat more, and would often gag or vomit if made to eat. His treatment was augmented with olanzapine and titrated from 2.5 mg up to 7.5 mg/day to help with his anxiety around meals, and to facilitate weight gain. Given his longstanding food selectivity and sensory issues, the ED dietician also allowed accommodations with respect to the meals that he received in hospital. Grandmother was empowered to ensure that Ethan finished everything on his tray, and was asked by staff to tell Ethan that if he didn’t finish his meal he could not have a pass off the ward. However, grandmother said she did not want to be the “bad guy” and instead chose (and was empowered to choose) to find other ways to help Ethan to finish his meals. For example, she chose to take him in a wheelchair for a walk around the hospital after he had finished half his meal, and they would then return and he would finish the rest of his meal. This worked well. Father would visit on weekends to provide meal support, and despite his anxiety about doctors and hospitals, started attending family sessions. Ethan was thrilled to be granted passes to eat at fast-food restaurants, but his weight started to drop as passes increased. He was ordering high calorie foods, but would get up and leave the restaurant before he had finished his portion. The therapist worked with both father and grandmother to help them to support Ethan to stay and finish all of his nutrition before they would leave.
Ethan’s pace, intake and weight gradually improved and he was discharged after a two month stay at 88% of his TGW so that he could begin the new school year. He was discharged on olanzapine 5 mg at bedtime and fluoxetine 10 mg/day. The family continued outpatient family therapy over the next month, but weight dropped and the family was resistant to driving the long distance to the hospital for therapy sessions. The therapist followed up with grandmother by phone and empowered her to track Ethan’s weight at home and to increase nutrition in keeping with his increased activity on the farm. His fluoxetine was gradually titrated up to 40 mg/day over the next three months. The fluoxetine was noted to significantly decrease his anxiety at school, and weight gain improved thereafter. At a follow-up appointment 4 months after discharge, Ethan had reached his TGW. His olanzapine was tapered and discontinued very slowly over the following months and he continued on fluoxetine with excellent effect.
Weight at start of family therapy: 31.8 kg;
%TGW at start of family therapy: 72%;
Weight at end of family therapy: 37.6 kg;
%TGW at end of family therapy: 85% (though some telephone conversations continued).
Weight at last follow up with AM physician: 44.2 kg (100% TGW) (Fig. ).
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pmc-6091012-4
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Jacqueline is a 14.4 year old girl with a history of social and generalized anxiety disorder, obsessive compulsive disorder (OCD) and ASD traits. She presented with a 1–2 year history of increasingly restrictive intake. Parents noted: “She has always been picky, but if you gave her the foods she liked she would eat them; now she won’t eat them.” Parents described getting every bite into her as “a struggle.” They also described her as very rigid, controlling, and more recently having huge temper tantrums, sleep problems and problems concentrating. She also looked and acted like a much younger child, and had symptoms of what appeared to be severe attention-deficit hyperactivity disorder (ADHD), as she frantically ran around the office and refused to sit still during the consultation, although she was described as a good student until this year, with no prior history of ADHD or of school or behavior problems. At initial assessment, she was on the 3rd percentile for weight; this put her at 90% of her TGW based on her growth curve, but with a history of having been underweight for a prolonged period and having always been a picky eater with very low appetite. She was in grade 9 at the time of assessment with a history of social isolation and increased anxiety at school for the past two years. There was a past history of bullying and social isolation in elementary school but no recent acute stressors or trauma. Her history was most consistent with a mixed ARFID presentation: ARFID-limited intake and ARFID-limited variety subtypes. A comprehensive medical work-up was negative.
Family therapy (with both divorced parents attending with Jacqueline) began within weeks of her initial assessment, and her treatment was augmented with olanzapine 2.5 mg at bedtime, which was later increased to 5 mg. The parents opted not to use a weight graph in sessions, as they were worried that Jacqueline would become “obsessed” with the numbers, but parents were informed weekly whether weight was up or down. Jacqueline was kept home from school and her mother stayed home from work to focus on renourishing her. After a few weeks Jacqueline’s weight had improved and she became bored and expressed a desire to return to school, so attended school in the mornings in the “resource room” (where she ate a ‘mandatory’ morning snack) and then came home for lunch. Jacquie gained weight well as her treatment continued over the course of 6 months (13 family therapy sessions). Of note, as weight increased, she became less “picky” about her eating and able to eat more variety. She also became significantly less hyperactive and regressed, and began to speak more in therapy. As a result, her treatment was augmented with weekly individual therapy sessions in addition to her family therapy. She expressed very high anxiety, both about her schoolwork and about socializing with peers. She was given information about ‘Asperger syndrome,’ which she found helpful. She continued to gain weight and reached her TGW, ending up on the 25th percentile for weight, with a height near the 25th percentile and an overall BMI of 50% for girls her age. Once she reached her TGW, the olanzapine was tapered and stopped, and an SSRI (fluvoxamine 25 mg/day) was started to target her anxiety. The fluvoxamine was increased gradually from 50 mg to 100 mg/day, but this dose was associated with increased agitation and restlessness, including one weekend when Jacqueline spent the entire weekend in her pajamas pacing back and forth in her bedroom. The fluvoxamine was decreased back down to 50 mg/day and the agitation disappeared, although the family chose to continue the medication as they found the 50 mg dose continued to have a positive effect on her anxiety. By the end of treatment, Jacqueline could sit well throughout a one-hour therapy session with no symptoms of her initial hyperactivity or restlessness; she was also less irritable, more cheerful and affectionate with her family, and more mature and articulate.
Weight at start of family therapy: 37.4 kg.
%TGW at start of family therapy: 93%.
Weight at end of family therapy: 46.5 kg;
%TGW at end of family therapy: > 100% (new TGW: 46 kg) (Fig. ).
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pmc-6091016-1
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A previously well, 64-year-old South Asian woman who was not on any medication presented to the Teaching Hospital, Anuradhapura, Sri Lanka, with a history of snake bite, five hours before admission while gardening. The offending snake specimen was brought to the hospital and was identified as a Merrem’s hump-nosed viper (Hypnale hypnale - the only species of Hump-nosed pit viper that exists in the area), by the doctor at the hospital emergency department. The patient complained of mild swelling and pain at the bite site and epigastric pain. She was fully conscious, alert and oriented and was not under influence of any substance or alcohol. The only first-aid the patient had received was washing of the bite site. There was mild swelling and tenderness of the right foot with two fang marks below the right lateral malleolus. Her heart rate was 106 beats per minute, blood pressure was 95/60 mmHg on supine position. The patient had mild postural dizziness and, also complained of increased thirst and appeared dehydrated. The twenty-minute whole blood clotting test (WBCT20) was < 20 min and the International Normalized Ratio (INR) was 1.05. She was kept under observation and, was not given antivenom as usual because the only available antivenom (Indian Polyvalent antivenom) is not raised against Hump-nosed pit vipers. The rest of her physical examination was unremarkable. Oral fluids intake of 0.6 L and intravenous infusion of 0.9% saline in the rate of 100 ml/hour (total of 3 L over 24 hours) was commenced. The patient received tetanus toxoid and oral cloaxacillin 500 mg 6 hourly.
Next day, 23 hours after the bite, she developed an episode of generalized tonic-clonic seizure which lasted 30 minutes, followed by two episodes of similar seizures two and four hours after the initial episode. Her serum electrolytes following the first seizure showed profound hyponatremia (Na+ 118 mEq/L; normal: 135–146 mEq/L) and normal serum potassium (3.8 meq/L; normal 3.5–5 mmol/L). The plasma glucose at the time of the seizure was 152 mg/dl. WBCT20 and INR were normal. Her blood cell count and hemoglobin concentration were normal. The serum creatinine was 0.81 mg/L and the blood urea nitrogen level was 2.3 g/L. The serum corrected calcium was normal (2.46 mmol/L; normal: 2.1–2.6 mmol/l). Serum osmolality was low (257 mosm/kg water; normal: 280–300 mosm/kg water). Urine biochemistry showed high urinary sodium of 164 meq/L (normal: 20 meq/L), increased fractional sodium excretion 12.76% (normal < 1%), low urinary potassium 20.4 meq/L (normal: 25–100 meq/24 hour) and high fractional potassium excretion 52.14% (normal < 10%). She had no past history of any co-morbidities. She had no history of seizures and was not on any medications. The ultrasound scan showed normal kidneys. Computer Tomography, Magnetic Resonance Imaging of the brain, cerebral venogram and electroencephalogram were all normal. She was treated with oxygen (6 L/min) via a face mask, intravenous diazepam, oral sodium valproate and the hydration was maintained with 0.9% saline infusion and additional oral fluids to a total 3 L per day after first 24 hours with close monitoring of vital signs and fluid balance. The renal salt loss and the clinical and biochemical parameters improved after careful volume replacement with 0.9% saline for five days. She was discharged from the ward seven days after the snake bite. Her serum sodium level (140 mEq/L) and the urinary osmolality on discharge was normal. Although it was planned to review her after four weeks, the patient defaulted from follow up.
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pmc-6091145-1
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A 67-year-old man was admitted to the hospital with symptoms of loss of appetite and weight. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a pancreatic mass extending into the entire pancreas, splenic vein, and inferior mesenteric vein (Fig. ). The patient underwent total pancreatectomy. Macroscopically, a whitish tumor measuring 10 cm was found in the pancreatic tail and body. Microscopically, eosinophilic tumor cells were found in a trabecular acinar pattern. Immunohistochemical analysis was negative for synaptophysin, chromogranin A, CD56, and trypsin. Finally, we diagnosed it as PACC, T3N0M0 (TNM classification according to the Union for International Cancer Control). According to the protocol for advanced PDAC, adjuvant chemotherapy with S-1 (Taiho Pharmaceutical, Tokyo, Japan) was administered for 11 months after pancreatectomy, and, subsequently, it was stopped due to the side effects (diarrhea, oral mucositis, fatigue, and hand-foot syndrome). Twenty-four months after the pancreatectomy, a solitary mass measuring 1.5 cm was found in segment 7 of the liver on CT (Fig. ). The patient underwent posterior liver segmentectomy with a histopathological diagnosis of liver metastasis of PACC. Twenty-eight months after the pancreatectomy, the patient developed melena. Colonoscopy revealed a type-2 tumor at the lower rectum (1 cm above the dentate line, Fig. ), and biopsy revealed it to be rectal metastasis of PACC. CT and positron-emission tomography (PET) demonstrated the rectal tumor and an enlarged lymph node near the inferior mesenteric artery (Fig. , ). The patient underwent laparoscopic abdominoperineal resection. Peritoneal dissemination was not found intraoperatively. Macroscopically, the tumor was 4 cm long, created polypoid elevation of its surface, contained nodular components and ulceration, and penetrated the rectal mucosa into the submucosa and muscularis propria (Fig. ). Histopathology showed severe nuclear atypia of the tumor cells, and immunohistochemical analysis using CDX2, cytokeratin (CK)7, CK19, and CK20 confirmed the same profile as that of the specimen from pancreatectomy (Fig. ). One lymph node out of 32 contained metastases. Thus, the pathological diagnosis was rectal and lymph node metastasis of PACC. All three surgical operations resulted in no severe postoperative complications. Periodic radiological examinations showed no tumor recurrence at 40 months after the pancreatectomy without additional chemotherapy. Our clinical decision was approved by the cancer board that included surgeons, oncologists, radiologists, and pathologists at the University Hospital of Tsukuba. Informed consent was obtained from the patient.
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pmc-6091168-1
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A previously healthy, premenarchal 12 year-old female presented to a local emergency department with altered mental status in the morning. She was back to baseline upon arrival except for recall deficit. Her initial point-of-care plasma glucose was 44 mg/dL. Serum glucose was confirmed low at 49 mg/dL. Family denied history of any hypoglycemic symptoms, except for occasional sluggishness in the morning. She had no signs/symptoms of infection and there were no medications in the home that could cause hypoglycemia. Family history was also negative for hypoglycemia or seizures. We recommended 24-h observation with frequent plasma glucose monitoring and additional laboratory evaluation. She continued to have hypoglycemia by point-of-care testing, requiring dextrose containing IV fluids overnight. Despite the fluids, AM plasma glucose was 58 mg/dL. Her 8 am cortisol was 2.2 mcg/dL, ACTH 30 pg/mL. TSH was normal at 2.169 mIU/mL, no free T4 resulted. Infectious workup and toxicology screen were negative, including oral hypoglycemic agents. Given the persistent hypoglycemia after 24 h and non-reassuring morning cortisol, we recommended transfer for additional evaluation.
Her initial physical exam was normal (height 144.5 cm, 17th percentile for age; weight 39.5 kg, 38th percentile for age), visual fields intact, no signs of hyperpigmentation, Tanner II breasts (B2) with Tanner I pubic hair (PH1). Review of growth charts from her pediatrician did not demonstrate much change in height percentiles, growing around the 25th percentile for the last few years. ACTH stimulation test revealed cortisol of 1.3, 12, and 14.9 mcg/dL pre-, 30- and 60-min post-cosyntropin, respectively. Other pituitary hormones demonstrated a low free T4 of 0.5 ng/dL, normal FSH and LH and elevated prolactin level of 842.2 ng/mL (Table for SI units). Brain and pituitary MRI showed a 2.0 × 1.5 × 1.9 cm enhancing mass expanding the sella turcica (Fig. ). She was initiated on hydrocortisone 6 mg/m2/day, increased to 12 mg/m2/day secondary to persistent hypoglycemia, levothyroxine 50 mcg daily, and cabergoline 0.25 mg twice weekly. Family was instructed on glucometer usage and advised to monitor blood sugars every morning and for concerning symptoms.
After discharge, she continued to have fasting hypoglycemia. Review of additional critical labs from the referral institution demonstrated an insulin level of 13.6 uIU/mL when serum glucose was 52 mg/dL. Two days after discharge, a second set of fasting critical labs were repeated at a local laboratory and were remarkable for an inappropriately elevated insulin concentration in the setting of hypoglycemia. When serum glucose was 48 mg/dL, insulin level was 11.1 uIU/mL, c-peptide was 2.7 ng/mL (normal range 1.1–4.4), proinsulin was 18.1 pmol/L (normal range 0–10), beta-hydroxybutyrate was 0.6 mg/dL (normal range 0.2–2.8), and urine was negative for ketones. Other pertinent labs included normal calcium levels, ranging from 9.2–9.3 mg/dL. Additional clinical interventions were recommended, including continuous glucose monitor, small frequent meals, cornstarch, and glucagon for emergency use. Imaging studies were pursued to localize the suspected insulinoma. Magnetic resonance imaging (MRI) of abdomen and pelvis with contrast was negative for any lesion or lymph node enlargement (Fig. ). Endoscopic ultrasound (EUS) identified a circumscribed 12 × 11 mm hypoechoic mass in the pancreatic body/tail (Fig. ). Immunostaining of a fine needle aspirate of the suspicious lesion was positive for chromogranin, synaptophysin, and insulinoma-associated-1 gene (INSM1) with 2–3% Ki-67 proliferation, confirming low-grade insulinoma (Fig. ). Computed tomography (CT) of the abdomen was pursued for additional surgical planning, confirming presence of a 1.2 × 0.9 cm hyperenhancing mass in the distal pancreatic body with no evidence of metastatic disease (Fig. ). She underwent successful laparoscopic enucleation of the pancreatic lesion and has had no further hypoglycemia. Prolonged fasting revealed normoglycemia in the setting of ketonuria, confirming resolution of hyperinsulinemic hypoglycemia. She underwent genetic testing of MEN1 to confirm the diagnosis. She was found to have a novel mutation, heterozygous for a single nucleotide duplication, c.1247dupT (Tyr417LeuX32). The duplication causes a frameshift with a premature stop codon at position 32 of the new reading frame. The truncation is expected to result in loss of nuclear localization signals and several modified residues and thus is considered to be pathogenic.
On follow-up, she recovered adrenal function as demonstrated by morning cortisol of 19.1 mcg/dL (527 nmol/L) 4 months after presentation. By 10 months after treatment of the prolactinoma, puberty had progressed slowly to Tanner B2PH2. Height initially declined to the 11th percentile at 4-month follow-up, but was back to the 19th percentile by 10 months, with a growth velocity of 11 cm/year. Repeat MRI 10 months after initiation of cabergoline demonstrated near-normalization of pituitary size (1.3 × 1.4 × 0.9 cm).
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pmc-6091179-1
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A 20-year-old white woman arrived at the Emergency Room (ER) complaining of sudden onset severe left flank and lower left quadrant (LLQ) abdominal pain, nausea, and vomiting. Her height and body weight were 180 cm and 63.5 kg (BMI of 19.5). Her history revealed that at the onset of pain, she believed she was suffering from severe menstrual cramps. The pain was not relieved by non-steroidal anti-inflammatory drugs (NSAIDs) even at higher doses. Eventually she became nauseated and started vomiting. She admitted to having felt increasingly more nauseated for several months prior, but had not vomited until the day she arrived at the ER. Her past clinical history included type IV (Graf classification) congenital bilateral developmental dysplasia of the hip diagnosed at birth (now resolved), adenoidectomy (3 years of age), and severe menstrual pain starting at 15 years of age, which had increased in severity over the course of the subsequent 4 years. There was no other remarkable clinical history, injury, or accident.
She was afebrile, and laboratory results were unremarkable with the exception of a white blood cell (WBC) count of 13 and gross hematuria with significant WBC in her urine. On physical examination, her abdomen was very tender in her left flank, LLQ, and pelvic area. She denied burning during urination and frequency. A pelvic ultrasound (US) was read as unremarkable. No other tests were ordered. She was released with the diagnosis of cystitis/UTI and prescribed ciprofloxacin, ibuprofen, oxycodone, and ondansetron.
Four days later she returned to the ER complaining once again of severe abdominal pain, but now also vomiting violently. The pain was no longer localized to just her left flank and LLQ, but had generalized to her right upper quadrant (RUQ) and periumbilical region. Repeat bloodwork revealed that WBC was now 10, but serum amylase was 220 and lipase was 120. Urine analysis still showed some red blood cells (RBC) and WBC. A CT with intravenously administered contrast was ordered and was read as unremarkable. An abdominal US showed a left-sided kidney stone. A pelvic US showed a right-sided ovarian cyst. A US of her gall bladder revealed no calculi or pathology.
Three days after passing the kidney stone, she had an increased feeling of swelling, pressure, and pain in her left flank. A ureteroscopy was performed to confirm the absence of any pathology. Her left ureter had minimal reflux, but otherwise no abnormalities were noted. A second CT scan was performed, but again no obvious pathology was noted.
With no improvement 2 weeks later, the decision was made to perform an exploratory laparoscopy, which revealed severe pelvic congestion as well as small amounts of endometriosis. Her appendix was thickened and had an appendicolith and was removed. Repeat bloodwork showed that her amylase and lipase levels had returned to normal. She was discharged the same day.
Two weeks after the laparoscopy when there was still no improvement in the pain, nausea, and anorexia and because she started experiencing post-prandial satiety, an endoscopy was performed. It revealed a moderate dilation of her duodenum as well as fluid in her stomach despite the fact that she had not eaten or drunk for more than 24 hours. The findings from the endoscopy prompted a review of the images from the initial CT, and this time it was determined that her duodenum was being compressed by an external structure. An enterography and a barium swallow confirmed the constriction of the duodenum by the SMA, and she was finally given the diagnosis of SMAS. Despite the fact that she had already suffered a net weight loss of 8 kg, the option to treat the disorder conservatively by enteral or parenteral feeding was not considered since she was of normal weight when the symptoms started and there was little expectation that weight gain would resolve the issue. She underwent a Roux-en-Y duodenojejunostomy. An iodine swallow 3 days post-surgery showed that fluid moved freely through the anastomosed areas. She was discharged 4 days later, able to tolerate soft foods.
Four weeks after the duodenojejunostomy, the pain in her left flank became even more severe, and she also started experiencing urinary hesitancy as well as pain in both flanks and her pelvic region during urination and bowel movements. The severity of the pain in her left flank increased even more during the week of her menstrual cycle. Another careful review of the very first CT scan performed at the beginning of the onset of symptoms led to the conclusion that her SMA was also compressing her LRV, her left ovarian vein was engorged, and the severe pelvic congestion, initially discovered during the laparoscopy, was also visible. A selective venography of her LRV showed significant stenosis of the LRV, a markedly enlarged ovarian vein with retrograde flow into her pelvis, and multiple enlarged pelvic veins. The pressure gradient between the renal side of the vein and the IVC side of the vein was 5 mm Hg. These findings established the diagnosis of NCS.
Eleven weeks after the duodenojejunostomy, she underwent an LRV transposition in which her adrenal vein and the ovarian vein were also tied off. Following this second surgery she began to improve considerably. One month after surgery her left flank and RUQ pain were significantly decreased, and though the nausea persisted, she was able to eat without vomiting and started to regain the weight she lost.
Eight months after the Roux-en-Y duodenojejunostomy and 4 months after the LRV transposition, a CT showed patent anastomoses, reduction in the number of pelvic varices, and reduced diameter of her ovarian vein. She was able to eat with minimal GI disturbance and had regained 5 kg.
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pmc-6091280-1
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A 19-year-old African American man was brought to the emergency department (ED) by emergency medical services (EMS) after an episode of syncope earlier on the day of admission while playing basketball at his college. This occurred suddenly and was associated with a transient episode of lightheadedness, diaphoresis, and blurred vision. This was followed by lost consciousness for less than 10 seconds, with spontaneous recovery as witnessed by his friends on the sidelines. There was not any involuntary movement of the body, urinary or bowel incontinence, or postictal confusion as per the witnesses. After regaining consciousness, there was a complaint of nonradiating, substernal, burning chest pain with a “6 out of 10” intensity, which lasted approximately 20–30 minutes and was relieved with a nitroglycerin sublingual pill given by EMS. On arrival to the ED, the patient was asymptomatic. Blood pressure was 103/67 mmHg, heart rate 85 bpm and regular, afebrile oxygen saturation > 95% on room air, and respiratory rate 12 per minute. Physical examination findings revealed a supple neck with no jugular venous distention; no carotid bruits were audible. Cardiovascular examination revealed a regular heart rhythm with normal S1 and S2 and no significant audible murmurs, parasternal heave, or thrill. The lungs were clear to auscultation bilaterally. There was no pitting pedal edema, and all peripheral pulses were palpable. The abdomen was soft and nondistended; no focal neurological deficits were evident.
History was positive for two similar events in the past. The first episode occurred approximately 10 years ago in Nigeria while playing soccer, and another event occurred a year ago year while running a block to catch a bus. Medical attention was not sought on both occasions. Family history was negative for similar syncopal attacks, sudden cardiac arrest, or arrhythmias. Social history was positive for drinking heavy amounts of Vodka with friends occasionally on the weekends along with marijuana but negative for cigarette smoking or other illicit drug use.
Initial laboratory values in the ED showed hemoglobin of 12.3 gm %, serum creatinine of 1.4 mg/dl (normal reference range 0.50 to 1.20 mg/dl) and BUN of 24 mg/dl, elevated initial troponin I of 0.23 ng/ml (normal reference range 0.00 to 0.07 ng/ml), total creatinine kinase level of 585 units/liter (normal reference range: 49 to 397 U/l), and urine with 11–20 hyaline casts. Thyroid stimulating hormone, serum potassium, and serum magnesium were within normal limits. His chest X-ray did not reveal any acute cardiopulmonary process (). His initial electrocardiogram (EKG) revealed significant 4 mm to 5 mm ST depression in the anterolateral leads but otherwise unremarkable (). A repeat EKG done within 2 hours of the initial presentation showed reversal of ST depression (). A repeat troponin done in 6 hours revealed staggering troponin I of 53.30 ng/ml. Immediate bedside echocardiogram showed a nondilated left ventricular cavity with normal LV wall thickness and a mildly diminished LV ejection fraction at 45–50% (). In the emergency room, immediate treatment with 325 mg of Aspirin and therapeutic Lovenox (1 mg/kg) was given. Overnight, periodic 5–7-beat runs of nonsustained ventricular tachycardia on the cardiac monitor occurred. Cardiac tomography (CT) angiography of the heart revealed a common trunk for the right and left coronary arteries arising from the right coronary cusp. The right coronary artery had a normal course. The LMCA traveled posteriorly between the ascending aorta and pulmonary outflow tract before resuming its normal course.
The LMCA narrowed in its midportion as it passed between these two structures (Figures and ). These findings were confirmed by cardiac catheterization which showed an anomalous origin of the LMCA, originating from the right sinus of Valsalva (Figures and ). The LMCA narrowed in its midportion with over 60–70% luminal stenosis due to a stricture present between the ascending aorta and the left ventricular outflow track. Afterwards, medical stabilization was achieved and a transfer to a tertiary care center was carried out.
Upon arrival at the tertiary care center, a procedure was performed involving the unroofing of the anomalous aortic origin of the left main coronary artery, including retro pulmonary unroofing by a congenital cardiothoracic surgeon. Recovery was satisfactory after the surgery, and postoperative complications did not occur.
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pmc-6091281-1
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An 89-year-old fit female with a history of chronic back pain and an appendectomy during her youth completed using a McBurney incision presented with a one-day history of spontaneous pain in her right flank without any fever, chills, or other symptoms. At the time of her admission, she was not in distress, she was not febrile, and her vital signs were within normal values. On clinical examination, there was swelling with a red area measuring 12 cm × 4 cm and tenderness of the right flank around her appendectomy scar. Crepitus could be felt diffusely on her right and left flanks and the periumbilical and epigastric regions upon palpation. Blood test showed the presence of mild inflammation, with a CRP value of 7 mg/l (within normal values) and an elevated white blood cell count of 18 G/l. The rest of the laboratory results were normal. Emergency ultrasonography was unhelpful because of air interference. An abdominal CT scan () showed diffuse subcutaneous abdominal emphysema extending to the pelvis on the left side that was more pronounced on the right inguinal fossa with a bowel loop in contact with the abdominal wall. An emergency laparotomy centered on the McBurney incision showed feces and pus within the subcutaneous compartment. Furthermore, at the level of the aponeurosis of the external oblique muscle, an inflammatory diverticulum could be seen fistulizing between the lumen of the sigmoid colon loop and the necrotic subcutaneous tissue. We subsequently diagnosed intraoperatively a subcutaneous abscess and emphysema with an enteroparietal fistula caused by a ruptured sigmoid diverticulum in an incisional hernia. The necrotic tissues were excised, and the punctiform sigmoid colon fistula was closed. Revision of the rest of the sigmoid showed important adhesions between the sigmoid colon and the parietal peritoneum of the right flank and between the caecum and the sigmoid colon, respectively. The sigmoid colon also showed diffused diverticulosis with no inflammation. The cutaneous and subcutaneous tissues were left open and dressed with a negative pressure-assisted closure device on postoperative day 1. The patient received intravenous antibiotherapy for two weeks with quinolones and a third-generation cephalosporin at first which was then switched to aztreonam due to an allergic reaction. Bacteriological studies showed polymicrobial digestive bacteria (i.e., Escherichia coli, Streptococcus equinus, and Enterococcus). Subsequently, there was good clinical and biological evolution. At two weeks postoperation, she was reoperated on for closure of the wound. She was discharged from the hospital three weeks after her initial surgical intervention with the indication to continue antibiotics for a total of four weeks.
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pmc-6091283-1
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A 61-year-old man referred from the emergency department to the ear, nose, and throat (ENT) clinic in Al Wakra Hospital because of vertigo and left ear discharge. The vertigo is rotatory in nature and is associated with hearing impairment and tinnitus as well as nausea and vomiting. Ear discharge was purulent, odorless, and intermittent for the last few years, but it became profuse and continuous for the last few days. The described symptoms were associated with severe left-sided headache and diplopia, and there were associated medical comorbidities (diabetic and hypertensive patient). On examination, the patient was conscious, oriented, and not feverish. Left ear examination showed pulsating purulent discharge with granulation tissue filling the middle ear cavity, the tympanic membrane was perforated, and the fistula test was negative. There was left beating nystagmus with left sixth cranial nerve palsy. Other ENT and neurological examinations were not remarkable. Pure tone audiometry showed left-sided severe mixed deafness, and left ear swab for microbiological study for culture and sensitivity was negative. Urgent CT scan was done to rule out intracranial complications, and it showed features of tympanomastoiditis and soft tissue shadow involving the middle ear and attic areas (Figures and ).
MRI with contrast showed asymmetrical signal changes in the bilateral petrous bone with reduced enhancement on the left with high suspicion of petrositis, in the context of chronic tympanomastoiditis ().
In addition to the mentioned pathology, there was a 10 × 4 mm enhancing lesion in the internal auditory meatus involving the 7th-8th nerve complex most likely acoustic neuroma, and there was no extension to the cerebellopontine angle (). Conservative treatment started with local and parenteral antimicrobial agents with labyrinthine sedative drugs. After ten-day treatment with good monitoring of blood sugar, the patient had satisfactory response and improvement regarding symptoms of ear discharge, vertigo, and diplopia, but there is no remarkable response regarding hearing loss and tinnitus. The patient continued on conservative treatment for the coming two months, he developed symptomatic response regarding vertigo, diplopia, and ear discharge, and further appointment given for exploration of mastoid and middle ear. Exploration of the mastoid and middle ear showed tympanomastoiditis with a lot of granulation tissue in the middle ear, mastoid, and attic; canal wall down procedure was done with successful result after postoperative follow-up.
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pmc-6091324-1
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27-year-old woman who had been on PD because of chronic glomerulonephritis confirmed by renal biopsy (histopathological evaluation revealed focal segmental glomerulosclerosis (FSGS)) and end-stage kidney disease (ESKD) for 2 years was admitted to our center with clinical symptoms of peritonitis. She was complaining of diffuse abdominal pain, fever, and cloudy dialysate. There were no signs of exit site infection (ESI). She was on automated peritoneal dialysis (APD) using Home Choice Pro device delivered by Baxter (USA). Her dialysis regimen was 12.0 liters of 1.36% Dianeal fluid. Her ultrafiltration was approximately 1000ml. Residual diuresis was approximately 1.0 liter daily. She had not had any PD-associated infections in the past. Moreover, she was suffering from chronic hepatitis C, hypertension, reflux esophagitis, and chronic gastritis. She had renal anemia treated with darbepoetin alfa s.c. and chronic kidney disease—mineral and bone disorder (CKD-MBD) were treated according to latest updated KDIGO Guidelines 2017 (calcium carbonicum in a dose of 3 x 3,0g daily, cinacalcet in a dose of 30mg daily, paricalcitol 2,0mcg 3 times a week). In 1990 she had episode of hemolytic-uremic syndrome (HUS) and acute kidney injury (AKI stage 3) with need for dialysis (PD was used for a month that time). Her vital signs on admission were as follows: she presented generalized abdominal tenderness, her blood pressure was 130/90mmHg, her temperature was 38°C, heart rate was 100 beats per minute, and respiration rate was 20 per minute. Laboratory test included white blood count (WBC) of 15.000/mm3, hemoglobin (Hgb) of 12,1g/dl, platelets count of 254.000/mm3, C-reactive protein (CRP) of 84,5mg/L, blood urea nitrogen (BUN) of 65mg/dl, serum creatinine of 4,15mg/dl, serum albumin of 3,8g/dl, total protein of 6,58g/dl, total cholesterol of 220mg/dl, dialysate leukocyte count (DLC) of 7217/mm3 (neutrophils/lymphocytes=86% vs 11%), PTH (parathormone) of 984pg/ml, serum calcium of 2,10mmo/l, and phosphorus of 1,86mmo/l. Blood culture and urine culture were both negative. We decided to start initial empiric antibiotics treatment with ceftazidime 1,0g daily and cefazolin 1,0g daily infused intraperitoneally (ip) into Extraneal 2,0-liter bag. Dwell time was 8 hours. Positive culture of A. xylosoxidans colonies was isolated from peritoneal fluid (using BD Bactec™ FX Becton Dickinson System, USA). Isolation of bacteria was performed using routine method at the clinical microbiology laboratory. The identification of isolated strain was further confirmed by applying mass spectrometric method (Matrix-Assisted Laser Desorption/Ionization-Time-Of-Flight, MALDI-TOF) using MALDI Biotyper (Bruker, Germany). Antimicrobial susceptibility testing of examined A. xylosoxidans strain was performed by the agar dilution method and carried out according to the European Committee on Antimicrobial Susceptibility testing recommendation (version 7.0). According to the antibiogram, the pathogen was sensitive to piperacillin/tazobactam, ceftazidime, and imipenem and was resistant to ciprofloxacin and cefepime. The treatment regimen was changed according to antibiogram results into imipenem+cilastatin intravenously (iv) in a dose of 2 x 0,25g daily. She was given the antibiotic for 2 weeks. Laboratory investigation and antibiotics regimen in the first episode of A. xylosoxidans peritonitis are given in .
We observed improvement in her clinical condition. There was no abdominal pain. Dialysate leukocyte count gradually decreased and control dialysate culture was negative. 3 weeks after the treatment, she was discharged from hospital. 7 days later she was admitted again because of another episode of peritonitis. Dialysate culture was positive and A. xylosoxidans colonies were identified again. Pathogen was sensitive to imipenem and ceftazidime and we started these antibiotics in a dose of 2 x 0,25g iv and 1 x 1,0 g intraperitoneally (ip), respectively. This time we decided to perform computed tomography (CT). There were no signs of gut perforation or abdominal abscess. After antibiotics implementation her symptoms disappeared quickly. We continued the treatment in the hospital for another 2 weeks. 2 weeks after being discharged from the hospital, there was another third episode of peritonitis with the same pathogen, A. xylosoxidans isolated from peritoneal fluid. In the case of relapsing A. xylosoxidans peritonitis, we decided to remove peritoneal catheter and transfer her to hemodialysis. After permanent catheter insertion into right carotid vein and control chest X-ray, she started temporary hemodialysis treatment. She was on 3 hemodialysis sessions per week. The tip of removed peritoneal catheter was sent for microbiological evaluation. Colonization of A. xylosoxidans was stated. After 2 months on hemodialysis, we decided to continue PD in her case and she was admitted to Department of Surgery for peritoneal catheter insertion. Then, 3 weeks later she successfully started PD treatment again.
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pmc-6091324-2
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The patient had been a 54-year-old male with CKD stage 5 secondary to multiple myeloma (MM). He was on PD since November 2015. After 2 months on CAPD he started APD using Fresenius Sleep-Safe Cycler. His dialysis regimen was 12,0 liters of 1,5% glucose solution. He had well preserved residual renal function (RRF) with residual diuresis approximately 1,5 liters daily. His ultrafiltration rate ranges from 600 to 800ml daily. Moreover, his medical history included diabetes type 2, hypertension, psoriasis, hernia esophagi, peptic ulcer, and spinal column rupture (compression rupture Th5-Th8) related to MM. On admission to the hospital he presented mild abdominal pain and turbid dialysate. Physical examination revealed the following: his temperature was 37,5°C; his pulse rate was 78 beats/min; blood pressure was 120/70mmHg; respiration rate was 18 per minute; his abdomen was tender to palpation with positive Blumberg sign. Laboratory tests were as follows: WBC 8,280/mm3, Hgb 7,8g/dl; platelets count 307.000; CRP 71,38mg/L; BUN 66,2mg/dl; serum creatinine 7,02mg/dl; serum albumin 3,1g/dl; total protein 5,3g/dl; total cholesterol 228mg/dl; dialysate leukocyte count 530/mm3 (neutrophils/lymphocytes 78% vs 10%), serum calcium 1,77mmo/l; phosphorus 2,1mmo/l; sodium 139,1mmo/l; potassium 3,9mmo/l. Samples of peritoneal fluid, blood, and urine were inoculated. Growth of S. suis from peritoneal dialysis fluid was confirmed by our clinical microbiology laboratory. Blood culture and urine culture were negative. Current methods for serotyping a strain of S. suis are serology, PCR using specific primers (for cps genes) or whole-genome sequencing, and analysis of the cps genes, which were not available in the laboratory. The strain was identified using MALDI-TOF MS technique on MALDI Biotyper apparatus (Bruker) as described above. S. suis colonies were sensitive to penicillin G, ampicillin, cefotaxime, ceftriaxone, imipenem, and clindamycin, as described above. After 2 days of empiric intraperitoneal antibiotics administration (cefazoline+ceftazidime ip), we changed the treatment according to antibiogram results into ceftriaxone iv. We continued ceftriaxone iv 2,0g per day for 2 weeks. His clinical condition improved. There was no abdominal pain; peritoneal fluid was transparent. Control peritoneal fluid culture was negative. After 2 weeks of hospital treatment the patient was discharged. In his case we did not observe relapse of peritonitis.
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pmc-6091331-1
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A 64-year-old male presented to the emergency room with a 2-week history of a severe, persistent headache. The patient's pertinent past medical and surgical history included obesity and an anterior cervical discectomy and fusion five years prior. A CT scan of the head followed by an MRI of the brain and spine revealed extensive pneumocephalus and concerns for meningitis. The MRI of the spine showed the ACDF hardware but did not reveal surrounding defects. The neurosurgery team was consulted, and the patient was admitted.
CT cisternogram/myelogram and high-resolution CT sinus were obtained. The cisternogram/myelogram was negative for leak at the skull base and cervical spine. CT imaging showed an air-fluid level within a left posterior ethmoid air cell with an apparent 2 mm adjacent osseous dehiscence along the fovea ethmoidalis, suspicious for the source of the CSF leak in this patient (). The radiology report also commented that hardware from the anterior and posterior fusion between C3-C6 appeared intact without evidence of fistula or pseudomeningocele.
The otolaryngology/rhinology team was consulted due to the radiographic findings on the sinus CT. On further history and exam, the patient reported intermittent clear rhinorrhea and occasional salty tasting drainage. He denied any significant history of rhinosinusitis. Physical exam demonstrated an obese man who was uncomfortable. Holding the neck in flexion demonstrated clear fluid from the left nostril. The nasal endoscopy was normal. Based on clinical presentation and imaging, there was concern for a left skull defect.
The patient was taken to the operating room for identification and repair of the CSF leak. The neurosurgery team placed a lumbar drain and dilute fluorescein dye injected intrathecally. On nasal endoscopy, fluorescein dye was noted to be pooling in the nasopharynx throughout the case. A total sphenoethmoidectomy was performed, and the area of the potential defect was located and confirmed with intraoperative surgical navigation. Inspissated mucus was found at the area of mucosal thickening on CT () without evidence of fluorescein-dyed cerebrospinal fluid or bony dehiscence at the fovea ethmoidalis. The location was reconfirmed with surgical navigation. The right sinonasal cavity was evaluated without identification of any leak.
Subsequent endoscopic evaluation of the nasopharynx and oropharynx revealed significant pooling of fluorescein, but both openings to the eustachian tubes were normal. Close inspection of the mid-oropharynx, however, revealed a bulge in the posterior oropharynx, and a small, pinpoint fistula with actively leaking fluorescein-dyed CSF was found ().
The CT scan of the neck region was reviewed to evaluate the cervical hardware (), and the head and neck surgery team was asked to assist neurosurgery with repair of the fistula. An anterior neck approach to the cervical hardware was performed. The anterior cervical hardware was removed and revealed a profuse CSF leak in association with the hardware. The defect was repaired with DuraSeal followed by rotation of the right omohyoid muscle into the site of the defect between the prevertebral fascia and the oropharyngeal mucosa. The neck incision was closed in a layered fashion. The patient was transported to the neurosurgery intensive care unit with the lumbar drain in place. He remained in the ICU on bed rest with the lumbar drain open at 10 ml/hr for 5 days. The patient was then allowed to mobilize, and the lumbar drain was removed. His headaches resolved, and repeat imaging showed resolution of the pneumocephalus. He was discharged and has done well with no evidence of CSF leak. A repeat CT myelogram of the spine at 8 months showed no signs of persistent leak.
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pmc-6091365-1
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The patient was a 73-year-old man. He was examined at our hospital for a sore throat that had persisted for 6 months. He had a drinking habit of one 500 ml bottle of beer daily and no history of smoking. Diabetes, hypertension, and hyperlipidemia were noted in his previous medical history.
Pharyngolaryngoscopy revealed a superficial, smooth tumorous lesion with a red hue in the oropharynx at the base of the tongue. In addition, a protruding tumor with atypical blood vessel formation was observed on the mucosal surface in the right piriform recess of the hypopharynx (). On contrast-enhanced CT, thickening of the pharyngeal wall showing irregular contrast enhancement was observed at the right tongue base and in the right piriform recess of the hypopharynx (). No swelling of neck lymph nodes was observed. On fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT), accumulation was observed for maximum standardized uptake values (SUVmax) of 2.0 and 4.2 in the lesions of the oropharynx and hypopharynx, respectively (). No accumulation was observed in neck lymph nodes or other parts.
Based on tissue biopsies, the histopathological diagnosis for the lesion in the right piriform recess of the hypopharynx was squamous cell carcinoma (). While the lesion at the base of the tongue was suspected to represent malignant lymphoma on histopathological examination, no definitive diagnosis could be reached. To achieve a definitive diagnosis, the entire tongue-base tumor was resected by transoral surgery under endoscopy. Subsequent histopathological examination revealed proliferation of plasma cells in the tumor, and immunostaining findings were as follows: κ(−), λ(+), CD3(−), CD20(−), CD138(−), CD79a(+), and MUM-1(+) (). A bone marrow puncture test ruled out multiple myeloma. Based on the above, a definitive diagnosis of Stage I (cT1N0M0) squamous cell carcinoma in the right piriform recess of the hypopharynx and primary extramedullary plasmacytoma in the oropharynx was made. In the treatment plan, radiotherapy was selected for curative treatment. After irradiating the whole neck with 40 Gy in 20 fractions, the irradiation field was reduced to target the tumor in the hypopharynx only, and additional radiotherapy comprising 30 Gy in 15 fractions was conducted (). Treatment outcome was complete response for both cancers, and no recurrences have been observed as of 12 months postoperatively.
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pmc-6091410-1
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A 16-year-old girl with no significant past medical history presented with bloody diarrhea, abdominal cramps, tenesmus, failure to thrive, and 6-Kg weight-loss during the prior 3 months. Physical examination was unremarkable except for age-adjusted BMI at the eleventh percentile. Abdominal examination revealed a soft, nontender abdomen and normoactive bowel sounds. Laboratory analysis revealed leukocyte count=8.1 bil/L, hemoglobin=11.4 g/dL, and platelets=207 bil/L. The alkaline phosphatase is 125 U/L, with other parameters of liver function and parameters of renal function within normal limits. Colonoscopy with terminal ileal intubation revealed severely erythematous and granular mucosa with focal exudation from rectum to ascending colon, findings consistent with UC (), and revealed endoscopically normal appearing cecum and terminal ileum. Histopathologic analysis of colonic biopsies revealed chronic colitis, with a moderate neutrophilic and lymphocytic mucosal infiltrate, crypt distortion, and scattered crypt abscesses. The cecum and terminal ileum appeared histologically normal (). She was treated with infliximab 5 mg/Kg, with initial symptomatic relief, but re-presented 1 year later with recurrent bloody diarrhea and failure to thrive, despite compliance with infliximab therapy. She developed infliximab antibodies necessitating escalating the infliximab dose, and adding extended-release budesonide 9 mg/day and azathioprine 2 mg/kg/day (after determining that her TPMT (thiopurine methyltransferase) activity was within normal limits). Her symptoms, however, progressed despite therapeutic infliximab levels. She underwent RPC and IPAA for refractory UC, which successfully controlled her symptoms but re-presented one year postoperatively with abdominal pain, 10 loose and bloody bowel movements/day, and involuntary 5-Kg-weight-loss. Fecal lactoferrin and calprotectin levels were elevated. Stool for ova and parasites, bacterial cultures, and Clostridium difficile toxin A and B by polymerase chain reaction (PCR) were unremarkable. C-reactive protein (CRP) level was elevated. Computerized tomographic enterography (CTE) showed focal narrowing and enhancement of mucosa within the J-pouch and abnormal mucosal enhancement, mural thickening, and narrowing of afferent ileal limb (Figures ). Pouchoscopy showed moderate exudation and ulcerations in J-pouch () and in afferent ileal limb up to 50 cm (. Histopathologic analysis of J-pouch and afferent ileal limb biopsies revealed chronic active inflammation, highly consistent with pouchitis and PI (. Immunohistochemistry of ileal biopsies for cytomegalovirus was negative. Capsule endoscopy revealed no small intestinal lesions more proximal than 50 cm in the afferent limb. Ciprofloxacin 500 mg twice daily and metronidazole 500 mg thrice daily were administered for the pouchitis and PI, but this treatment was subsequently escalated to include extended-release budesonide 9 mg/day and daily hydrocortisone enemas. As symptoms persisted, azathioprine 2 mg/kg/day was added, which successfully controlled her symptoms 3 months after initiating the azathioprine therapy. Six months after initiating the azathioprine therapy her fecal lactoferrin and calprotectin levels were within the normal range. At four years of follow-up, the patient has continued to be asymptomatic while chronically taking azathioprine, with normal CRP and erythrocyte sedimentation rate (ESR) levels.
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pmc-6091421-1
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Patient was a 54-year-old right-handed male, former professional football player. He first developed memory problems at the age of 46. Initially, he seemed more forgetful. The onset and the progression of the short-term memory problem were gradual over about eight years. He always did his own finances in the past. However at the age of 46, he started spending money more irrationally and was not paying the bills on-time. He repeated questions, sometimes even just a few minutes later. He had trouble learning new information. He could not manage his own calendar. He has become dependent on the GPS to get around. Patient had become less social. He did not have depressed mood; however, he had become more irritable and more easily angered. He had no behavioral issues. His activities of daily living (ADLs) were intact.
Patient started playing football when he was age 7 or 8. He played football in high school and college and then professionally. He played football for total of 23 years. Although he never lost consciousness, he experienced brief moment of flashes. This type of head injury averaged 3-4 times per game. There is no family history of dementia.
His mini-mental status exam (MMSE) was 24/30, and the clinical dementia rating (CDR) was 1. On neuropsychological testing, he had significant impaired verbal and nonverbal learning, recall, and recognition with rapid forgetting (more than two standard deviations). Patient's MRI showed cortical and subcortical atrophy, enlarged ventricles, and cavum septum pellucidum (). The hippocampal volume was below 5 percentile and the inferior lateral ventricle volume was greater than 95 percentile. His diagnosis was major neurocognitive disorder, likely Alzheimer's disease due to CTE.
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pmc-6091430-1
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A 56-year-old female was referred to the nephrologist due to apparently chronic kidney disease (CKD), diagnosed on a routine laboratory check-up. The patient was asymptomatic, Past medical record was contributory for three normal pregnancies. There was no background of alcohol intake, tobacco consumption, drug abuse, or medication exposure. There was a family history of CKD (). Physical examination was unremarkable. Abnormal blood tests were as follows: Haematocrit 37%; haemoglobin 11.9 g/dL; bicarbonate 21 mEq/L; urea 78 mg/dL (normal value 20-50 mg/dL); serum creatinine 2 mg /dL; uric acid 6.4 mg/dL; creatinine clearance 42 ml/min; proteinuria 0.2 g/day; urinary sodium excretion 188 mEq/day; urine pH: 6, urinary density 1015. Urinary sediment was unremarkable. HIV, HCV, and HBV were negative; C3, C4, and CH50 were within normal limits. ANA, p-ANCA, c-ANCA, antiglomerular basement membrane antibody, and antiphospholipid antibodies were reported as negative. Renal sonogram disclosed two kidneys, normal in shape and size. A kidney biopsy was performed. Light microscopy disclosed 30 glomeruli: 6 completely obliterated, 8 presented peripheral sclerosis of the glomerular tuft with adhesions between parietal and visceral epithelial cells of Bowman's capsule, and 6 depicted mild mesangial expansion (). Tubular atrophy and interstitial fibrosis were 30%. Blood vessels showed mild intimal sclerosis in arterioles. Immunofluorescence was negative. Electron microscopy: diffuse effacement of podocyte foot processes existed with microvillous transformation. Basal membrane was normal. Tubules were normal. Pathology report was as follows: focal and segmental glomerulosclerosis with moderate interstitial fibrosis and tubular atrophy. Patient was started on enalapril 5 mg twice a day and simvastatin 10 mg/day and on appropriate diet.
She was lost to follow-up. Sixteen months later the patient returned to the nephrologist due to asthenia, fatigue, and cramps. Blood pressure was 110/70 mmHg. Significant blood test results were as follows: Haematocrit 32%; haemoglobin 9.2 g/dL; potassium 5.5 mEq/L; bicarbonate 19 mEq/L; serum calcium 9.5 mg/dL; serum phosphate 6.2 mg/dL; serum magnesium 2.2 mg/dL; urea 111 mg/dL; serum creatinine 3.78 mg/dL; uric acid 8.1 mg/dL; albumin 4.3 g/dL; creatinine clearance 21 ml/min; proteinuria 0.29 g/day; urinalysis was unremarkable. Urine pH was 7 and urinary density 1010. A renal magnetic resonance imaging was noncontributory. The patient was prescribed erythropoietin 2000 U every other day, enalapril 5 mg bid, calcium carbonate 2 g/day, sodium bicarbonate two tea spoons daily, and polystyrene calcium sulfonate. Six months later the patient was started on hemodialysis (creatinine clearance 12 mL/min). A genetic study disclosed the insertion of a cytosine nucleotide in the VNTR (Variable Number Tandem Repeats) region of the MUC-1 gene, consistent with a mutation of the mucin-1 gene previously reported []: cDNA NM_001204286.1, protein NP_001191215.1, SNaPshot. The diagnosis of ADTKD-MUC1 (Autosomal Dominant Tubulointerstial Kidney Disease-Mucin-1) was finally made. The laboratory results of the patient's daughter revealed mild proteinuria and normal kidney function (). A kidney biopsy revealed mild tubulointerstitial disease and focal and segmental glomerulosclerosis in 2 out of 16 glomeruli. She was started on enalapril and nephroprotection and genetic counseling was given to her.
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pmc-6091454-1
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The patient was a 55-year-old woman with end-stage renal disease of unknown cause who had been undergoing hemodialysis for the past 4 years before receiving a living-related renal transplant from her husband in September 2014. Before the transplantation, the recipient's computed tomography (CT) scan revealed moderate to severe atherosclerosis of the bilateral iliac arteries. Also, the donor's CT revealed two left renal arteries. For blood type-compatible and donor-specific antibody-positive recipients, desensitization was performed before transplantation with three sessions of double filtration plasmapheresis or plasma exchange and two doses of rituximab (100 mg). Immunosuppressive agents included triple immunosuppressive therapy with extended-release tacrolimus, mycophenolate mofetil, and methylprednisolone. Briefly, the transplant bed was made in the right iliac fossa. At the back table, there were two donor renal arteries, namely, the main artery and one narrow artery. Because the length of the narrow artery was short, it had to be extended using the donor's gonadal vein graft with end-to-end anastomosis using a 6-0 monofilament at the back table. Usually when there are two donor renal arteries, we connect the two arteries conjointly or side-to-end at the back table. However, as the length of one artery was short and the distance between the two arteries was large to connect, we thought that connecting the two arteries will enable anastomotic stenosis. Therefore, we decided to intracorporeally anastomose each renal artery to the iliac artery. The transplanted renal vein was anastomosed to the right external iliac vein side-to-end using a 5-0 monofilament with continuous sutures. First, considering the position of the two arteries, we decided to anastomose the extended artery to the right external iliac artery side-to-end using a 6-0 monofilament with continuous sutures. Second, before anastomosing the main artery to the iliac artery, we punched out the external iliac artery using an aorta punch; however, the cavity in the artery was narrow. Firstly, we performed endarterectomy to create sufficient space for anastomosis with the renal artery. However, we could not make a sufficient cavity to ensure adequate blood flow. Therefore, we decided to reconstruct the external artery using a polytetrafluoroethylene (PTFE) vascular graft to achieve adequate blood flow. We were unable to undertake the standard renal artery anastomosis to the right external iliac artery owing to severe atherosclerosis, which would result in complete occlusion. After reconstructing the external artery with the PTFE vascular graft (length, 3 cm; diameter, 6 mm), the transplanted main renal artery was anastomosed directly to the PTFE graft side-to-end using a 5-0 monofilament with continuous sutures (). The surgical procedure lasted for 9 h and 14 min; the total ischemic time was 3 h and 20 min, and we could not confirm the first urine during the operation. The total bleeding volume was 450 mL; the same volume was transfused during the transplantation.
After transplantation, delayed graft function occurred, for which the patient had to undergo hemodialysis session on the third day after transplantation. Seven days after the transplantation, her serum creatinine level started to decrease (). The patient was discharged from the hospital on the 14th day after transplantation. Her serum creatinine level had reduced to 3.01 mg/dL on the day of discharge. Furthermore, after 1 month of discharge, her serum creatinine level reduced even further to 1.12 mg/dL. Presently, after 3 years of transplantation, her serum creatinine level is stable (1.2 mg/dL). She showed no sign of arterial graft infection after the renal transplantation. As she received an internalized antiplatelet agent before the transplantation because of coronary disease, internal resumption was resumed from seven days after transplantation to date.
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pmc-6092529-1
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We present the case of a 23-year old male with the chief complaint of mechanical low back pain of eight months duration. He gave a history of having fallen in the sitting position on two different occasions during his martial arts practice. Initially, his symptoms were localised, infrequent and aggravated by prolonged sitting. Subsequently, after six months, he developed sciatica over his left lower limb radiating distally to the dorsum of his foot and associated with numbness. There was no weakness or any other ominous signs. Systemic review was unremarkable and he had no constitutional symptoms. The left straight leg raising test was positive at 60 degrees. Neurological examinations of both lower limbs were unremarkable. Lumbar radiographs were normal. MRI () revealed a cystic lesion in the anterior epidural space with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.
The patient underwent endoscopic interlaminar surgery under general anaesthesia, in prone position on Jackson table. The knee and hip were flexed at 90 and 45 degrees, respectively, to increase the space of interlaminar window. The abdomen was left free to avoid increase in intra-abdominal pressure, to reduce venous pooling during the operation. All body prominences were protected and supported with soft silicon gel. Under image intensification, the level to be operated was localised. A 23mm cranio-caudal incision was made over the skin at a junctional point 2/3 lateral to midline and 1/3 medial to medial pedicular line (medial facet). The dorso-lumbar fascia was incised along the plane and widened. The underlying paraspinal muscle was then detached from the spinous process using a periosteal elevator and retracted laterally. The endoscopic portal was then opened followed by the placement of the camera into its respective channel. The working portal was kept flush with the lamina as medial as possible. With a 45-degrees Kerrison rongeur and curette, the ligamentum flavum was incised and detached from the lamina to enable entry into the spinal canal. After the incision of ligamentum flavum and opening of the lamina, the traversing nerve root, thecal sac, and the discal cyst were clearly visualised. The discal cyst was found to be communicating with the L4/L5 intervertebral disc. It () appeared faintly white in colour, containing serous fluid and was seen to be partially indenting the L5 nerve root. The cyst was excised at the base of the connection by performing an annulotomy. Histopathological examination showed fragment of cyst wall composed of fibrocartilaginous tissue devoid of epithelial lining.
The recovery was uneventful and patient was ambulating well two days after surgery and was discharged home. He had immediate relief of his sciatica symptoms. A week later he was reviewed, and there were neither any nerve root tension signs nor any post-operative complications. At ten months post-surgery, there were no signs indicating any recurrence.
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pmc-6092532-1
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A 19-year old male presented to us in the outpatient department with complaints pain on lifting weight with the right arm, deformity and limited range of motion of the right elbow for five months. The patient had fallen down and sustained the injury to his right elbow while hanging from the rootlets of a Banyan tree, following which, he had pain, swelling, and deformity of the right elbow. He had sought treatment from a local bone setter for four weeks following which pain and swelling decreased, but the deformity and elbow stiffness had persisted, for which he attended our hospital.
On examination, the Beighton hyperlaxity score of the patient was 5/9. There was flexion deformity of the elbow joint and wasting of muscles of the arm and forearm. The olecranon process was displaced from the olecranon fossa of the right humerus and an abnormal bone mass was palpable on the anterior aspect of the distal humerus. There was a flexion deformity of 40 degrees of the elbow joint with further flexion of 70 degrees. Pronation and supination were normal. There was a valgus laxity of the right elbow joint. The differential diagnoses were neglected dislocation of the elbow joint (posterior/anterior) and mal-united supracondylar fracture.
Antero-posterior and lateral radiographs of right elbow demonstrated an anterior dislocation of the elbow joint with an anterior bone mass at the distal humerus. The bony anatomy of the elbow appeared unclear on radiography, and a Computed Tomogram (CT) with 3D reconstruction () confirmed an anterior dislocation of the right elbow joint with a bony projection from the anterior border of the distal humerus. We hypothesised that because of hyperlaxity the patient had sustained anterior dislocation of the elbow joint without associated fracture. Massage and attempts to reduce the elbow joint by the bone setter had led to the formation of a heterotopic bone mass on the volar aspect of the humerus.
We performed an open reduction of the elbow by combined medial and lateral approach based on findings of the CT scan. We were successful in excising the bone mass but failed to reduce the elbow joint. There was some early degeneration of the articular cartilage of the distal humerus and olecranon. It was impossible to reduce the olecranon posteriorly. We extended the approach through the subcutaneous plane to the posterior aspect and performed an olecranon osteotomy. The humerus was reduced into the osteotomy, and it was fixed with tension-band wiring. Indomethacin was started at 25mg eight hourly after surgery for three weeks after the operation. We did not immobilise the elbow and started active assisted mobilisation of the elbow joint after surgery as tolerated by the patient. The patient was discharged after wound inspection on the 5th post-operative day and advised to attend the rehabilitation department for physiotherapy for six weeks.
At review one year postoperative he had a painless range of motion of 30 degrees to 120 degrees at the elbow joint. He has excellent pronation and supination and could perform light activities. The olecranon osteotomy healed well () though there was a reduction in the joint space of the elbow.
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pmc-6092533-1
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A 60-year old female underwent a phase III Oxford UKA [Biomet UK Ltd, Bridgend, United Kingdom] in 2006 for antero-medial osteoarthritis. A minimally invasive medial para-patellar approach was used and medium sized femur, 44 X 28mm tibia and 3mm meniscal bearing insert were implanted. She had an uneventful post-operative recovery. She was completely asymptomatic and was discharged from the follow-up at two years following the surgery with no symptoms and a range of motion of 0 to 130 degrees.
She presented to the Accident and Emergency (A&E) department in April, 2013 with history of a sudden onset of pain and swelling in the same knee. She heard a ‘pop’ in the knee while standing and did not report any obvious injury to the knee. On clinical examination, she was haemodynamically stable and afebrile and there was moderate effusion in the knee. The range of motion was from 30 to 60 degrees and she was unable to weight bear through the knee due to pain. The radiographs of her knee in the A&E department raised a suspicion of posterior dislocation of the polyethylene insert (). There was no evidence of loosening of femoral or tibial components.
The white cell count and CRP were normal, excluding an acute infection. She was admitted to the ward and surgical exploration was planned for the following day, with the view of changing the polyethylene insert or to revise the components if they were loose or damaged. The knee was opened through the previous scar of medial para-patellar approach. Intraoperatively, both the femoral and the tibial components were found to be well fixed with no scratches and rest of the knee did not show any evidence of osteoarthritis. The polyethylene insert was found to be fractured through the middle (). The anterior half was sitting on the tibial component and the posterior half was dislodged into the posterior compartment of the knee, stuck to the posterior capsule. It was not possible to retrieve that fragment from the front.
The operating surgeon had two options; either to remove both the components and then retrieve the fragment from the front or to retrieve it through a posterior incision. Eventually, rather than making a separate skin incision, the previous skin incision was extended and medial skin flap was raised to expose the postero-medial aspect of the knee. Deep fascia was incised to expose the pes anserinus. An interval was created between the pes anserinus and the medial collateral ligament anteriorly and medial head of Gastronemius posterioly (). Capsulotomy was performed through this
interval and the dislodged part of the polyethylene insert was retrieved through the opening. The polyethylene insert showed some pitting and whitening and it seemed to have fractured cleanly through the thinnest part (). A new polyethylene of the same size insert was implanted. The patient made uneventful recovery following the surgery. At two year follow-up she was completely asymptomatic with the range of knee movement from 0 to 125 degrees.
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pmc-6092534-1
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An 18-year old male patient was referred to our emergency clinic with injuries sustained in a paragliding accident. During takeoff from the slope with a parachute, and after achieving an altitude of 10-15 meters, the patient stated that he had dropped on his feet due to loosening of the security ties.
Physical examination revealed limitation of movement, ecchymosis, and edema over both feet and ankles. Pain and tenderness were especially localised anteriorly on the right ankle and dorsally over the calcaneocuboid joint on the right foot, and anteromedially on the left foot and around the left medial malleolus. There was no open wound and neurovascular status was intact in both feet. Antero-posterior (AP) and lateral (LAT) radiographs of the foot and ankle revealed coronal shear fracture of the body of the talus, an anterior process fracture of the calcaneus extending to the calcaneocuboid joint and a nondisplaced navicular body fracture of the right foot () and a displaced fracture of the navicular body accompanied with posteromedial process fracture of the talus on the left foot ().
To determine the exact localisation of the fragments and the degree of fracture displacement more accurately, a computerised tomography (CT) scan was performed. This revealed the coronal shear fracture of the talar body and the navicular fracture displaced 3mm and 1mm respectively, while the anterior process fracture of the calcaneus was minimally displaced on the right foot (). On the left foot, the posteromedial process fracture of the talus was displaced approximately 3mm, and revealed fragmentation, and that the navicular fracture consisted of three fragments and was dorsally displaced ().
The patient was operated on eight hours from the time of the trauma. Under general anaesthesia and pneumatic tourniquet, an anteromedial incision was made for the talar body fracture at the right side. Neurovascular structures were identified and protected, and an oblique osteotomy was performed on the medial malleolus. The talar body fracture was visualised. The vertical fracture line in the coronal plane was close to the posterior and displaced causing stepping-off on the talar dome but had no fragmentation. Following curettage and irrigation of the fracture site, it was anatomically reduced, and fixation was performed with two 4.5mm self-tapping screws from anterior to posterior to obtain compression. The medial malleolus osteotomy was fixed with one 4.5mm self-tapping screw and figure-eight wire looping. Fractures of the anterior process of the calcaneus and navicular body were managed non-surgically. The foot and leg were immobilised in plaster cast postoperatively ().
An anteromedial incision was also used for the left foot. Following the identification and retraction of the neurovascular structures, an oblique medial malleolus osteotomy was carried out. Posteromedial process of the talus was explored. After anatomic reduction, a small lateral incision was made and a 4.5mm self-tapping screw was placed through the lateral aspect for fixation of the large posteromedial fragment. Extending the incision towards the navicular bone, the fracture line and talonavicular joint were explored. The small bone fragments at the fracture site were removed. After anatomic reduction, fixation was achieved utilising a 4.5mm self-tapping screw from the dorsal to the plantar aspect. The talonavicular joint was stabilised with a Kirschner wire from anterior to posterior, and it was left extruding on the skin. Medial malleolus osteotomy was fixed with a 4.5mm self-tapping screw. A short leg cast was applied postoperatively ().
Parenteral cefazolin sodium (3x1 gr/day) was administered for three days postoperatively. After six weeks of immobilisation, plaster casts were removed and the Kirschner wire stabilising the talonavicular joint was pulled out. Radiographic evaluation following a rehabilitation period of six weeks showed consolidation and union of all fractures without any loss in reduction in both feet, and full weight bearing was encouraged. At 36 months follow-up there was no evidence of avascular necrosis. All fractures had completely healed. There were minimal arthritic changes at the right subtalar and calcaneocuboid and left talonavicular joints. There was no complaint of pain and the range of motion of both feet and ankles were near full. The patient regained his full activity level at the 5th month postoperatively. Maryland scores of the right and left feet were 85 (good) and 90 (excellent), respectively ().
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