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pmc-6358372-1
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A 71-year-old Japanese man experienced dry cough for 2 weeks and visited the Department of Respiratory Medicine at our hospital in August 2007. Enhanced chest-abdomen computed tomography revealed a tumor with a 3-cm diameter in the left lower lobe and left pleural effusion (Fig. ). A 5-mm nodule, considered to be lung metastasis, was detected in the left upper lobe. Cytological analysis of the left pleural effusion by thoracic puncture led to the diagnosis of lung adenocarcinoma. Gadolinium-enhanced brain magnetic resonance imaging and bone scintigraphy did not reveal any other metastases. The tumor was classified as clinical T4N0M1, stage IV according to the TNM classification of the Union of International Cancer Control (UICC), 6th edition. According to the UICC 8th edition, it was classified as clinical T4N0M1a, stage IV A. The patient had a history of hypertension and was a past smoker (60 pack-years) and a company employee. The Eastern Cooperative Oncology Group performance status (ECOG-PS) at the time of admission was 1. The carcinoembryonic antigen (CEA) level was 97.4 ng/mL (normal, 0–5 ng/ml).
Beginning in August 2007, the patient received carboplatin (CBDCA) and docetaxel (DTX). After 4 cycles, the tumor was reduced to 1 cm in diameter. The 5-mm nodule and pleural effusion had also decreased. According to the Response Evaluation Criteria in Solid Tumors version 1.1, partial response was achieved, but he experienced progressive disease (PD) after 8 months. Six cycles of re-challenge chemotherapy (RC) using the same regimen were started in August 2008 and were effective. Thereafter, at each recurrence of PD, 4 to 6 cycles of RC were administered, and by 2013, 38 cycles had been completed over 6 years of treatment (Fig. A). However, we could no longer control disease activity using the same chemotherapy regimen. Moreover, primary tumor size evaluation became difficult owing to massive pleural effusion; although not standard, we estimated the effect of treatment using the increase and decrease of CEA as an index. CEA increased from a minimum of 4.6 ng/ml to 33.3 ng/ml in October 2013 during repeated cytotoxic chemotherapy. Although his EGFR mutation status was unknown, we initiated erlotinib administration and the CEA level decreased. After 8 weeks, the patient developed grade 3 acneiform rash, assessed using the Common Terminology Criteria for Adverse Events version 5.0, and erlotinib administration was discontinued for 6 weeks. Cycles of medication and treatment holiday were repeated, and the patient was carefully observed for skin rash. Dose reduction was attempted once, but it was not effective, because we noted an elevated CEA level and intolerable skin rash. For 3 years, 4-week erlotinib administration was repeated with 4–6-week treatment holiday intervals (Fig. B). CEA increased from a minimum of 3.1 ng/ml to 30.4 ng/ml in January 2017 during treatment with erlotinib. We performed EGFR mutation analysis using adenocarcinoma cells from the pleural effusion and detected exon 19 deletion and exon 20 T790 M mutation; therefore, osimertinib was substituted for erlotinib.
We continued monthly CEA measurements after beginning osimertinib administration and noted that the level continued to decrease. In August 2018, the CEA level was 12.1 ng/ml and the ECOG-PS was 1. As of the last follow-up, the patient has survived for >11 years since the diagnosis of lung cancer.
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pmc-6358390-1
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A 36-year-old woman with an obstetric history of gravida 6, para 3 (all normal spontaneous deliveries), and 2 induced abortions, presented to our emergency department with an acute and persistent dull pain in the right lower quadrant of her abdomen, that had started a few hours before her arrival. This was accompanied by additional signs and symptoms including rebound pain, cold sweating, and nausea without vomiting. There were no relieving or aggravating factors. Her last menstrual cycle was 7 weeks and 4 days ago, and she had no history of pelvic inflammatory disease.
Her past medical history included human papilloma virus infection, administration of 3 doses of Gardasil vaccination (7 years ago), intrauterine device insertion (4 years ago), diagnosis of cervical intraepithelial neoplasia 3, and a cervical conization procedure (3.5 years ago), and laparoscopic tubal sterilization (1-year-4-months ago). Figure presents the result of her laparoscopic tubal sterilization surgery. According to our image records, there was an estimated 2 cm gap between the proximal and distal blunt ends.
Initial physical examination showed stable vitals. Abdominal palpation revealed generalized abdominal stiffness and tenderness. Urine pregnancy test was positive. Transvaginal ultrasound showed no evidence of intrauterine pregnancy but revealed a right adnexal mass and presence of minimal ascites. Laboratory data revealed serum human chorionic gonadotropin (β-hCG) level of 15795 million IU/mL, white blood cells count of 12300/μL, and hemoglobin level of 11.9 g/dL.
Based on the clinical signs, imaging, and high level of serum β-hCG, an ectopic pregnancy was highly suspected, and she was admitted for a next day surgical intervention. However, 14 hours after admission to the gynecology ward, she experienced vomiting, sudden syncope, and hypotension (79/42 mm Hg). Laboratory test revealed that her hemoglobin had dropped to 8.9 g/dL. Based on a suspicion that the ruptured ectopic pregnancy might have caused hypovolemic shock, an emergency laparoscopic operation was performed.
Laparoscopy revealed a 3 cm × 4 cm bulging mass at the right distal end of the remnant stump in the ampulla region, and 1000 mL of internal bleeding. The gap at the site of previous right tubal sterilization was estimated to be 3.5 cm (Fig. ). Both ovaries were normal in shape and size. No endometriotic foci or endometrioma were found. To mitigate the risk of a second ectopic pregnancy and in accordance with the patient's lack of desire for continued fertility, we performed a complete bilateral residual tubal stump excision. Histopathological assessment confirmed the diagnosis of right distal tubal stump ectopic pregnancy. One day post-operation, the B-hCG level reduced to 3968 million IU/mL. The patient recovered well after surgery and was discharged after 3 days.
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pmc-6358403-1
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A 30-year-old male presented with unproductive cough and multiple cervical lymphadenopathy in December 2016. The pathology of the cervical lymph node biopsy revealed T-LBL. Positron emission tomography-computed tomography (PET-CT) revealed multiple lymph node involvements in the neck, mediastinum, bilateral parasternum, and abdomen, the Ann-Arbor stage of the lymphoma reached stage III. The following treatment plan was implemented: 1 cycle of VDLP (vincristine + daunorubicin + L-asparaginase + prednisone) induction chemotherapy, followed by 2 cycles of CAM (cyclophosphamide + A-cytarabine + methotrexate) chemotherapy. After the induction chemotherapy and total body irradiation (TBI)/cyclophosphamide protocol, the patient underwent allo-HSCT with the patient's brothers as donors in July 2017. On the 12th day after transplantation, the CD34+ cell count was 8.33 × 106/kg, the mononuclear cell (MNC) count was 10.15 × 108/kg; the process was uneventful. Forty days after transplantation, the patient developed abdominal pain, diarrhea, and rash across his body. Acute grade three GVHD was considered. The symptoms were relieved after the intravenous administration of methylprednisolone, cyclosporine, and mesenchymal cells. The patient experienced fever during this period, and then the temperature returned to normal after treatment with broad-spectrum antimicrobials, including meropenem and voriconazole. Patients who showed transient BKV, CMV, and hepatitis B virus (HBV) expression levels after transplantation demonstrated improvements after antiviral treatment. The BK DNA level was checked on August 7, 2017, and the result was 4.07 × 107 (copy/ml). The BK DNA level was 1.05 × 105 (copy/ml). A chest CT scan showed diffuse ground-glass opacities (Figs. and ). An abdominal CT scan revealed enlargement of the spleen (Fig. ).
The patient underwent tracheoscopy and CMV-DNA was 3 × 106 in BALF and 6 × 103 in blood. The patient also carried out enteroscopy at the same time and his immunohistochemical CMV antigen was positive Then, the patient was diagnosed with CMV pneumonia and enteritis and treated with intravenous ganciclovir (500 mg/d) and high-dose glucocorticoids (80 mg/d). However, the patient continued to have high fever, progressive shortness of breath, and gastrointestinal bleeding. Intubation and invasive mechanical ventilation support were performed. Unfortunately, the condition continued to deteriorate. After 3 weeks, the patient died because of severe hypoxemia and hemorrhagic shock.
Informed consent for the collection of his medical history and blood samples was obtained in compliance with the Declaration of Helsinki and approved by the local ethical committee, and the patient provided informed consent for publication of the case.
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pmc-6358405-1
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A 52-year-old male who was eventually diagnosed with a Wagner grade 4 diabetic foot ulcer combined with Budd-Chiari syndrome presented at the outpatient clinic of our hospital due to uncontrolled high body temperature, continued expansion of the right lower leg and a foot ulcer with increasing malodor. Random blood glucose test results, ultrasound imaging and magnetic resonance angiogram (MRA) confirmed this diagnosis. The man was diagnosed with type 2 diabetes in a rural community hospital four years previously. Four months ago, he developed a 1 cm × 2 cm ulcer on the lateral side of his right lower leg with a small amount of fluid exudate.
At the time of admission, the patient was in an acceptable condition, with a temperature of 38°C, a pulse of 88 beats / min, a respiration rate of 22 breaths / min, a blood pressure of 130 / 70 mmHg, a height of 1.75 m, a body weight of 60 kg, and a BMI of 19.5 kg / m2. Abdominal shifting dullness was positive, both lower extremities were swollen, and multiple fractures were visible on the wound surface on the medial and lateral sides of the right calf and right ankle. The dorsal right foot ulcer was visible and measured approximately 7 × 8 cm. All ulcers had a significant amount of yellow liquid exudate and odor (Fig. ). The random blood glucose test revealed that his glucose level was 20 mmol/L. The results of the computed tomography (CT) examination showed the followed: cirrhosis, splenomegaly, ascites, portal hypertension and collateral circulation. The MRA results showed the following:
inferior vena cava stenosis, indicating the possible need for embolization, and abdominal imaging changes in line with Budd-Chiari syndrome;
portal hypertension, ascites, splenomegaly, and intrahepatic and extrahepatic collateral circulation.
The ultrasound results showed the following:
the right and left hepatic veins were closed at the proximal end of the heart, and the middle hepatic vein was not apparent;
the intrahepatic traffic branch was formed;
there was diffuse damage to the liver parenchyma;
the portal vein was widened;
the umbilical vein was open;
the abdominal wall veins and gastric coronary veins were widened;
there was splenomegaly and splenic hilum varicose veins;
there was a tubular echo between the spleen and kidney, indicating the formation of traffic branches;
there was gallbladder edema;
there was diffuse injury to the kidneys;
there was abdominal effusion.
These findings conformed to the sonographic presentation of Budd-Chiari syndrome (intrahepatic type).
After admission, we established a treatment team consisting of multidisciplinary experts to develop treatment strategies to stabilize the patient's blood sugar, control the infection, and improve his microcirculation; we decided to apply this strategy throughout the treatment process. Figures and show the trends in the changes in C-reactive protein and 2-hour postprandial blood glucose levels throughout the treatment period. The treatment team then developed the following treatment plan:
interventional treatment of Budd-Chiari syndrome after the patient's general condition improved;
surgical debridement of large areas on the right lower extremity; and
amputation or skin grafting according to the wound condition after surgical debridement.
On the 6th day after admission, the patient underwent inferior vena cava angiography, hepatic venography, superior vena cava angiography, and inferior vena cava balloon dilatation under local anesthesia. During the operation, the hepatic vein was patent, the superior vena cava was unobstructed, the inferior vena cava below the hepatic vein plane was patent, the inferior vena cava above the hepatic vein plane was occluded, and the inferior vena cava was not developed below the opening, showing more collateral circulation. The thicker side branch had a diameter of approximately 10 mm. During the operation, it was difficult to pass the balloon through the occluded inferior vena cava segment. During this procedure, the surgeon used a 2.6-m Boston Amplatz hardened guide wire and delivered 10 × 40 mm and 16 × 40 mm balloons to the occluded inferior vena cava segment for balloon dilation. The procedure went well without complications. Postoperative angiography showed that the inferior vena cava was mostly unobstructed, with a stenosis of approximately 30%, and the collateral circulation had disappeared.
On the 9th day after admission, debridement was performed on the right lower leg and right foot of the patient under general anesthesia. During the operation, a large area of ulcerated skin was found on the right lower leg, and there was a substantial amount of exudate. On the dorsum of the right foot, a large area of necrotic skin was found on the leading edge of the right lower leg. The necrotic tissue spread to the proximal end along the tendon. Subcutaneous edema in the lower right limb was combined with an elevated skin temperature. The surgeon removed the previously confirmed necrotic tissue and preserved any tissue the necrotic status of which had not been verified (Fig. ). The wound was rinsed with saline and hydrogen peroxide after the surgery, sprayed with JEKSUNG (Changyi Pharmaceutical, China) and beifuxin (Zhuhai Yisheng Biological Pharmaceutical, China) on the surface, covered with Mepilex (Mölnlycke Health Care AB, Sweden), and then wrapped with sterile gauze.
On the 21st day after admission, debridement was again performed on the patient's right lower extremity under nerve block anesthesia. Then, a vacuum sealing drainage device was installed over the wound. During the surgery, we could see that there was some fresh granulation tissue covering the wound. The surgeon removed the remaining necrotic tissue, washed the wound with a normal saline and hydrogen peroxide, and then installed the vacuum sealing drainage device over the wound, using pressure dressings of sterile cotton pads and elastic bandages to cover the wound (Fig. ).
On the 32nd day after admission, under combined spinal and epidural anesthesia, the patient received skin grafts. We could see a substantial area covered with fresh granulation tissue above the wound (Fig. ). We saw skin defects in the foot dorsum and the anterior lateral of region the right lower leg; the extensor tendon of the foot was exposed. During the operation, we completely removed the clearly necrotic tissue, washed the wound with normal saline and hydrogen peroxide, excised free skin from the left thigh, and fixed it to the right lower extremity wound with interrupted sutures. Then, the wound surface was sprayed with JEKSUNG and beifuxin. The wound was covered with Mepilex and wrapped with sterile gauze.
On the 52nd day after admission, the attending physician confirmed that the skin had completely covered the wound, the postoperative recovery was excellent and the operation was successful. On the 56th day after admission, the wound had completely healed (Fig. ). The patient was discharged from the hospital after being examined by the attending physician.
During the entire treatment process from admission to discharge, we insisted on assessing the wound every day and changing the dressing based on that assessment. The specific steps involved in changing the dressing were as follows: the old dressing was removed, the wound was rinsed with saline and hydrogen peroxide, and the wound surface was sprayed with JEKSUNG and beifuxin. Then, the wound was covered with Mepilex and wrapped in sterile gauze.
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pmc-6358413-1
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A 12-year-old girl with a mass in her nose was admitted to the Department of Otorhinolaryngology of Shenzhen Children's Hospital in July 2015. She had a 4-month history of progressive, unilateral right nasal obstruction, unilateral mucopurulent rhinorrhea, foul nasal odor, snoring, hyposmia, and occasional epistaxis; there was no itching, sneezing, headache, facial numbness, eye swelling, vision loss, earache, or hearing loss. She first noted the presence of the painless mass in March 2015, and the mass gradually grew in size. A clinical examination revealed a painless mass in the right nasal cavity that was not sensitive to xylometazoline contraction. An anterior rhinoscopy showed a white polypoid neoplasm in the right nose. The anterior segment of the tumor was not smooth and filled the nasal cavity and nasopharynx. There was no swelling on the right side of the patient's face, no changes in the soft and hard palate, and eye movement was normal. The bilateral neck did not reach the enlarged lymph nodes. The patient's lungs had normal respiratory sounds. The liver and spleen were not enlarged or lumped. A computed tomography (CT) scan (Fig. ) of the paranasal sinuses showed a mass (right inflammatory polyp and calcification) involving the nasal cavity, the right maxillary sinusitis, and ethmoid sinusitis. There was no nasal septum, orbital, or skull base involvement. A chest X-ray showed no abnormality in the lungs. A preoperative biopsy of the nasal cavity under topical anesthesia showed an inflammatory change. The initial diagnosis was a right nasal-nasopharyngeal space-occupying lesion. Hemorrhagic necrotizing polyps and ectopic teeth were suspected.
A right nasal cavity-nasopharynx neoplasm resection was performed under general anesthesia on the fourth day after admission. During the operation, a polypoid tumor of the right nasal cavity was seen, including erosion and necrosis of the surface of the anterior segment of the tumor, completely blocking the right nasal cavity (Fig. ). The tumor was removed with a microdebrider; there was less bleeding when the microdebrider was used to cut the nasal cavity and nasopharyngeal mass (Fig. ). The tumor extended from the olfactory cleft to the entire nasal cavity and nasopharynx. No tumor was found after opening the anterior ethmoid sinus and maxillary sinus. Obstructive inflammation of the paranasal sinus was apparent. After resecting the neoplasms in the nasal cavity and nasopharynx, a grayish white bone (2 cm × 1.5 cm × 1 cm) was seen under the mass in the nasopharynx. The bone was loose (Fig. ), and its surface was uneven; further, there was a small amount of bleeding in the bone bed wound. Microscopically, the tumor exhibited hypercellularity and increased mitotic activity. A large number of spindle-shaped cells with a high nuclear to cytoplasmic ratio were arranged in short and long fascicles (Fig. ) with indistinct cytoplasmic border and varying degree of nuclear pleomorphism. Epithelial, cartilaginous, and bony heterologous elements were observed. The bone in the nasopharynx was an immature reticular trabecular bone-like material with increased and irregular adhesion lines and visible bone lacunae. Further immunohistochemical analysis revealed that the mesenchymal marker vimentin and the neuroectodermal marker soluble protein-100 (S-100) and protein gene product (PGP) 9.5 were positive (Figs. –). The clinical diagnosis was nasal and nasopharyngeal sarcoma (T2N0M0). On the basis of these pathological and immunohistological findings, the tumor was diagnosed as an MPNST with heterogeneous components (cartilage and bone) mesenchymal differentiation. Pathological HE staining revealed spindle cells arranged in bundles or fascicles with obvious atypia and high mitotic rates; hence, a malignant tumor was suggested. Vimentin, S-100, and PGP 9.5 were positive.
Two weeks after the operation, the patient received intravenous chemotherapy at another hospital. Chemotherapy was administered using pirarubicin (THP), high-dose cisplatin (DDP), and high-dose methotrexate (HD-MTX). The patient received 8 courses of chemotherapy and was followed-up for more than 3 years postoperatively. To date, she remains healthy with no evidence of recurrence or metastasis. Her physical development is normal. She has good nasal ventilation and no runny nose, nosebleeds, headaches, facial numbness, impaired vision, cough, or asthma. An endoscopic examination showed no recurrence of nasal mass. The cavity was clean, there was no discharge in the nasal passage, and the nasopharyngeal mucosa was smooth. Recurrence of the tumor was not found on CT examinations performed at 9 months after the operation (Fig. ). There was no sign of peripheral metastasis on PET/CT examination.
The patient's parents consented to the publication of this case report.
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pmc-6358610-1
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A 67-year-old gentleman with essential tremor underwent implantation of a Boston Scientific DBS system into the left VIM nucleus of the thalamus. At 3 months postoperatively he returned for modification of his programming due to increased tremor in his right hand. At this visit, there was an error in establishing communication between his IPG and the programming device. This patient had already been programmed at a different location and previously there had been no difficulties in connecting the programmer with his IPG. Troubleshooting of this issue began by replacing each component including the programming device, the connectors, and the computer one at a time, however, the patient’s device still did not connect. The computer would initiate data download from the device but an error message would appear at varying points during this process. The error message: “action unsuccessful: communication link (25035)” would be prompted (Fig. ). This error message was noted to be a sign of radiofrequency interference. The following steps were then performed in an attempt to eliminate any interference: (1) ensure the IPG is fully charged (Fig. ), (2) remove any power sources near the patient. RF readings were taken next to the patient and at every corner on the room. The patient location had an RF reading on 176 (Fig. ) compared to 87, 48, 67, and 78 for each of the corners. Within the patient remote control (RC) there is an option that measures RF. The RF meter is a standard “RSSI” (Received Signal Strength Indicator) indicator. Also, there is an ADC (analog-to-digital converter) that samples the signal as received by the receiver. The value of the ADC reading is then presented on the RC. The RC is held in the spot of interest for the measurement to be taken. The patient was then moved to a new location (RF reading at this new location was 45 (Fig. ), subsequently, the computer connected to the IPG and programming proceed normally. After completion, the patient was moved back to the original location and error message 25035 reappeared. The patient provided written informed consent.
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pmc-6359131-1
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The patient was a 59-year-old male who presented to our emergency department with a complaint of a 5-h duration of lower abdominal pain. He had a previous history of atrial fibrillation and had taken warfarin, digoxin, and bisoprolol. A physical examination revealed tenderness to palpation of the right lower abdomen without signs of peritoneal irritation. Laboratory data showed slight leukocytosis (WBC 8700/mm3) with slightly elevated C-reactive protein (CRP 0.43 mg/dL).
A computed tomography (CT) image revealed a dilated cecum surrounded by free air, while the appendix was intact (). The preoperative diagnosis was perforation of the cecum. We planned to perform an urgent laparoscopic surgery.
During the laparoscopy, the cecum was found to be dilated and discoloured ().
About 10 h after symptoms onset, we performed a laparoscopic-assisted ileocecal resection. After the resection of the damaged intestine, extracorporeal end to end anastomosis was performed. The specimen revealed localized ischemic change on the anti-mesenteric side of the cecum (). Microscopically, the transluminal ischemic change was confirmed.
The lesion was diagnosed as isolated cecal necrosis. On day three postoperative, the patient developed a small intestinal perforation caused by sticking on the drainage tube, and he underwent ileostomy. After that, the patient progressed uneventfully, and the patient underwent closure of the ileostomy on the 51st postoperative day.
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pmc-6359648-1
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A 40-year-old male with a medical history significant for diabetes mellitus and hypertension presented to the emergency department (ED) with complaints of progressively worsening proximal muscle weakness of bilateral lower and upper extremities of 4 days duration. The pain and weakness was reported to be worse in lower extremities. He denied fever, chills, recent trauma, or strenuous activities. He denied rash, photosensitivity, back pain, abdominal pain, dysphagia, diarrhea, dysuria, or incontinence. History was negative for sick contacts or recent travel. He denied recent illnesses, new medications (such as statins or exogenous steroids), alcohol intake, or recreational drug use. He denied prior rheumatologic or thyroid disorders and a personal or family history of musculoskeletal disorders.
At presentation, he was noted to have extreme difficulty standing from the wheelchair. He was initially afebrile but subsequently developed a low-grade fever (maximum temperature of 101.3 °F). Exquisite tenderness on palpation of the proximal muscle groups of upper and lower extremities was present without atrophy. Muscle strength at initial examination was noted as 4/5 on bilateral shoulder abductors, elbow flexors, and extensors; 5/5 on bilateral wrist flexors and extensors; 2/5 on bilateral hip flexors; 3/5 on bilateral quadriceps; and 5/5 on bilateral plantar flexors and extensors. No heliotrope rashes were noted. Neurological examination was non-focal.
Lab tests were notable for slight leukocytosis (white cell count (WBC) of 11 × 103/μL (ref, 4.8–10.8 × 103/μL)) with increased bands. Blood glucose was elevated at 394 (ref, 70–99) mg/dL with hemoglobin A1c of 11%. Urinalysis was consistent with urinary tract infection (UTI) with many bacteria, 26 WBCs, and positive nitrite, with a normal renal ultrasound. UTI at admission and was initially empirically treated and later changed to Cefazolin after urine culture grew methicillin-sensitive Staphylococcus aureus (MSSA). Blood culture was not obtained at the time of presentation; however, was sent after positive urine culture, after about 2 days of antibiotic therapy, and was negative. Alongside, further workups were done to determine the etiology of myopathy, including multiple metabolic, infectious, and rheumatologic workups.
Electrolytes (Na, K, Ca, P, and Mg) within normal limits. Elevated inflammatory markers- C-reactive protein (CRP) of 24.33, ref: <1 mg/dL; Erythrocyte sedimentation rate (ESR) of 40, ref: 0–15 mm/h. Mildly elevated creatinine kinase (CK) of 273 (ref, 30–223) IU/L; normal transaminases; aldolase 6.1 (ref, 1.5–8.1) U/L; and lactate dehydrogenase of 223 (ref, 140–271) IU/L. Unremarkable vitamin B12, folate, and thyroid stimulating hormone levels. Negative urinary drug screen. Negative HIV screen. Negative serology for Lyme disease IgM and IgG; Parvovirus B19 antibody IgM; and Respiratory syncitial virus and Influenza A and B. No monoclonal proteins on protein electrophoresis. Negative serological tests for inflammatory myopathy or associated connective tissue diseases.
Anti-nuclear antibodies (ANA)-negative 1:40; Anti-ds DNA-negative. Antibodies against extractable nuclear antigens-negative anti-Ro and anti-La. “Myositis specific” autoantibodies-e.g., Jo−1 IgG antibody of <0.3 (ref ≤ 6.9) U/mL; signal recognition particle (SRP) and Mi-2 autoantibodies not detected; and cytoplasmic 5′-nucleotidase 1A (cN1A) Ab < 20 (ref < 20) units. Negative serological tests for vasculitis.
Anti-neutrophil cytoplasmic antibody (ANCA) titers-MPO IgG Ab < 0.3 (ref ≤ 3.4 U/mL) and PR3 IgG Ab < 0.7 (ref ≤ 1.9 U/mL). Negative Hepatitis B and C serology.
After all the initial workups and serology were negative, imaging was considered.
On further evaluation with MRI of the extremities and lumbar spine,
MRI of the bilateral thighs showed diffuse muscle edema with innumerable foci of nodular and ring enhancement-concerning for diffuse infectious myositis (). MRI lumbar spine with no evidence of diskitis or osteomyelitis but showed multiple small foci of intramuscular enhancement, including the iliopsoas, gluteal muscles, and posterior paravertebral muscles and possible left perinephric inflammation. Similarly, MRI of the upper extremities showed muscle edema within supraspinatus, infraspinatus, subscapularis, deltoid, and triceps and to a lesser extent, the biceps muscles with multiple small foci of nodular and ring-like enhancement in the visualized muscles of the upper arm and proximal forearm. CT-guided aspiration of the left thigh was attempted only after about 4 days of treatment with antibiotics (no focal fluid collection was noted on CT and random muscle biopsy was obtained). No growth on cultures, including acid fast bacilli, anaerobic and fungal cultures, and no significant inflammatory infiltrate was reported. During the hospital stay, his CK had improved to 81 IU/L on repeat lab tests at 4 days.
After almost about 10 days of antibiotics, the patient started to have a gradual improvement in pain and muscle weakness. He was discharged in a stable condition at day 14 to continue 2 more weeks of oral antibiotics and with recommendation for outpatient follow up. After 2 months of initial presentation, patient was seen at his family physician’s office at which time he had complete resolution of the weakness and diffuse muscle pain. However, he complained of one tender lump at the distal and medial aspect of left thigh and there was no sign of infection. Outpatient MRI of the left thigh showed resolution of previous foci of abnormal signal within the thigh musculature (); however, a new 2.5 cm peripherally enhancing lesion within the vastus medialis muscle was seen, with possible differentials of hematoma, abscess, and myonecrosis. An ultrasound guided aspiration of the mass was attempted; the ultrasound, however, did not show a loculated fluid collection, and core biopsies were obtained from the distal medial left thigh from a location with altered architecture as noted in MRI. The tissue specimen was reported as skeletal muscle with endomysial and perimysial chronic inflammation and atrophy. Microscopic evaluation showed no polymorphonuclear leukocytes and no growth on cultures. An immune-histochemical stain was positive for leukocyte common antigen (LCA) confirming a marked lymphocytic component, with plans for further special histochemical studies.
Informed consent was obtained from the patient for submission of the case.
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pmc-6359751-1
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A 34-year-old male of mixed Japanese and European descent presented with a several month history of lymphadenopathy, arising as a left sided cervical mass. In addition, he had an IgM kappa paraprotein of 30 g/L. He underwent a fine needle aspirate then excision of the left cervical node and a bone marrow biopsy. Examination of the lymph node showed partial effacement of normal nodal architecture by a lymphoma with a marginal zone pattern. There were no proliferation centres. Flow cytometry (on the FNA and the excision specimen) demonstrated a B-cell clone expressing CD19, CD20 (see Fig. ), CD5, CD38, partial CD23, partial FMC7 and moderate kappa light chain. The cells were negative for CD10 and CD200.
Immunohistochemical staining showed the neoplastic B-lymphocytes in the widened marginal zone regions were positive for CD20, CD79a, CD5 (weak) and bcl-2. The cells were negative for CD10, bcl-6, cyclin D1, SOX-11 and CD23. Around the periphery of the expanded neoplastic marginal zone B-cells there was an associated population of neoplastic plasma cells which demonstrated immunohistochemical evidence of kappa light chain restriction. CD21 and CD23 highlighted expanded follicular dendritic cell networks. The Ki67 proliferation rate was around 10%. Molecular testing showed no evidence of a MYD88 L265P mutation.
On the basis of the clinicoradiologic presentation, the morphological appearance and the immunophenotypic and molecular findings the final diagnosis was determined to be nodal MZL with aberrant CD5 positivity.
Conventional GTG-band karyotype analysis was performed from both the lymph node and bone marrow biopsy using standard protocols.
FISH studies were performed using the Vysis CLL probe set which consists of the following locus specific probes: ATM (11q22.3), TP53 (17p13.1), D12Z3 (12p11.1-q11.1), D13S319 (13q14.3) and LAMP1 (13q34). The Vysis break apart IGH (14q32) probe and the Vysis dual-fusion CCND1 (11q13)/IGH probe (14q32) were also used. In addition, an Empire Genomics break-apart probe CCND2 (12p13) was set up. Subsequent to this analysis and to determine if MYCN was involved in this rearrangement, a break-apart probe was created by combining the Vysis MYCN (2p24) locus specific probe combined with a custom made Empire Genomics probe RP11-542H15 (also at 2p24). Processing was performed according to the probe manufacturer’s instructions.
The karyotype reports were written in accordance with the International System for Human Cytogenetic Nomenclature [].
Chromosome analysis of the patient’s lymph node showed an abnormal cell line in 7/10 cells. There was an apparently balanced translocation between the short arm of one chromosome 2 at band p24 and the long arm of one chromosome 14 at band q32 (see Fig. ). In addition to this, there was gain of one additional copy of chromosomes 3, 7 and 18.
Interphase FISH showed no imbalance or rearrangement of ATM, TP53, D12Z3, D13S319, LAMP1, CCND1 or CCND2 loci. Due to the cytogenetic findings of a rearrangement involving chromosome 14, metaphase FISH using the IGH probe was performed. The IGH probe showed a break-apart signal with the 5’ IGH signal on the derivative chromosome 2, the 3’ IGH signal remained on the derivative chromosome 14 (see Fig. ).
Metaphase and interphase FISH using both the custom made Empire Genomics RP11-542H15 and the Vysis MYCN probe in a single hybridisation to form a break-apart probe showed that the MYCN probe had been translocated to the derivative chromosome 14 (see Fig. ).
The same abnormalities were detected in 6/20 cells of the bone marrow aspirate from this patient confirming that infiltration into the bone marrow had occurred.
The karyotype from the LN was reported as: 49,XY,t(2;14)(p24;q32),+3,+7,+18[7]/46,XY[3].ish t(2;14)(MYCN-,RP11-542H15+;MYCN+,3'RP11-542H15-)[4],(3'IGH-,5'IGH+,3'IGH+,5'IGH-)[7].nuc ish (MYCN, RP11-542H15)x2(MYCN sep RP11-542H15x1)[169/200],(5'CCND2,3'CCND2)x2(5'CCND2 con 3'CCND2x2)[200]
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pmc-6359758-1
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A 61-year-old Chinese woman was admitted to the Department of Respiratory Medicine, Peking Union Medical College (PUMC) Hospital with recurrent chest pain and transient syncope. Nine months before her admission, she had developed sudden burning chest and back pain on the right side, along with transient syncope, sweating, fatigue, and dizziness. She was admitted to a local hospital, where hypotension (85/50 mmHg) was detected. Laboratory studies showed a significant decrease in her hemoglobin level (93 g/L to 50 g/L). Thoracic ultrasound revealed right-sided pleural effusion with septa while chest computed tomography (CT) also showed right-sided encapsulated pleural effusion. Her symptoms improved after supportive treatment, and the effusion was seen to be gradually absorbed on serial follow-up CT scans. However, the cause of the chest pain, syncope, and anemia remained unclear.
Furthermore, in the 12 years previous to that visit, the patient had developed recurrent periorbital nodules and a submaxillary mass, and had undergone multiple surgeries in other hospitals. Biopsies of these lesions revealed inflammatory pseudotumor, dacryoadenitis (periobital), and reactive lymph node (submaxillary) hyperplasia. She exhibited an improvement with steroid treatment, but we were unable to obtain detailed records of her treatment regime. The patient had no history of hypertension, diabetes, hepatitis, or tuberculosis. She took no medication on a regular basis and had never smoked or used illicit drugs.
Earlier on the day of her admission to our hospital, the patient experienced another similar episode of chest pain along with sweating and transient syncope. She was sent to the ER of a local hospital and given fluid resuscitation, then transferred to our hospital. On examination, her blood pressure was 110/61 mmHg, and her electrocardiogram was normal. Her hemoglobin level was found to have decreased from 121 g/L to 71 g/L. Physical examination revealed anemia, scars and yellowish nodules on the eyelids, one hemorrhagic vesicle on the buccal mucosa, and skin bruises. Breath sounds over the right lower lung were diminished. Thoracentesis revealed thick bloody fluid with clots, and pleural fluid analysis showed an increase in the total cell number (1.763 × 1012/L), white blood cell count (5.74 × 109/L), total protein (TP) level (154 g/L), and lactate dehydrogenase (LDH) level (13,995 U/L). Other laboratory tests showed a significant increase in the C-reactive protein (CRP) level (31.99 mg/L), erythrocyte sedimentation rate (ESR; 118 mm/h), and bleeding time (BT; 21 min). Chest CT showed encapsulated pleural effusion with atelectasis on the right side, and multiple enlarged lymph nodes in the right intercostal, right hilar, and mediastinal area (Fig. ). CT angiography (CTA) showed that the right intercostal arteries were slightly dilated, but did not reveal a potential cause of bleeding such as arteriovenous malformation (AVM) or aneurysm. PET-CT showed thickened right-sided pleura with diffuse SUV elevation. Exploratory surgery was performed, and the specimens that were obtained from the right pleura and intercostal lymph node showed no sign of neoplasia.
Based on the patient’s past medical history, IgG4-RD was suspected, and the serum IgG4 test showed a significant increase in IgG4 level (17,400 mg/L). The histopathological analysis, conducted by a specialized pathologist, of the right parietal pleura found dense fibrosis of the pleura, lymphoplasmacytic infiltrates, and proliferated small blood vessels with lumen dilation and congestion (Fig. ). Immunohistochemical staining showed that all the sampled tissues (right pleura and intercostal lymph node specimens, together with previously biopsied periorbital mass specimens) were infiltrated by massive lymphocytes and plasma cells, with more than 50 IgG4-positive cells per HPF (high-power field), and an IgG4-positive cells to IgG-positive cells ratio of more than 40% (Fig. ).
This patient was diagnosed with IgG4-RD after discussions with multidisciplinary experts. She was treated with prednisone 60 mg per day for 4 weeks, which was then tapered by 5 mg every week to 30 mg per day, and tapered more slowly thereafter. The patient adhered well to the therapy and no severe side effects occurred. At follow-up visits, regression of the periorbital nodules, and mediastinal and intercostal lymph nodes was confirmed. CRP level, ESR, BT, and the IgG4 level returned to normal. More than 1 year later, there had been no recurrent bleeding event, and chest CT performed at the last examination revealed no obvious abnormalities such as pleural effusion, nodules, solid infiltration or lymphadenopathy.
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pmc-6359825-1
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A 68-year-old Japanese postmenopausal woman developed brown vaginal discharge after radical cystectomy for bladder cancer, which was histologically diagnosed as high-grade UC invading up to the superficial muscularis propria without metastasis (pT2a pN0 cM0 [Union for International Cancer Control, 8th edition]) concomitant with focal urothelial carcinoma in situ in the urethra. She had a history of left renal pelvis UC, which was surgically removed after repeated 80-mg/fraction Bacillus Calmette-Guérin injections via a single J stent, resulting in no residual carcinoma 9 months before the radical cystectomy. She was also treated for breast cancer at 47 years of age without postoperative recurrence. Gynecologic examination of the lower genital tract, including the periurethral region, was unremarkable; however, cervical screening cytology demonstrated severely atypical cells suspicious for adenocarcinoma (Fig. a-c). Cervical colposcopy and diagnostic conization revealed no cervical neoplasm. Eleven months post-cystectomy, punch biopsy of the vulva and vagina confirmed intraepithelial UC in the juxtaposed squamous epithelium with pagetoid spread (Fig. a-c). Concurrent invasive malignancy was ruled out, and CO2 laser vaporization of the vulvar and vaginal lesion was performed. The patient remained alive without evidence of invasive malignancy for 14 months after the radical cystectomy for bladder cancer.
The CINtec® PLUS cytology test (Roche Diagnostics, Basel, Switzerland), an immunocytochemical p16/Ki-67 dual staining kit for screening of cervical disease, was negative for p16 labeling (Fig. d). Immunocytochemistry revealed neoplastic cells positive for cytokeratin (CK) 7, CK20, p63, and GATA3 (Fig. e-h). Immunohistochemical examination of the biopsy sample of the vulva and vagina revealed neoplastic cells positive for uroplakin III, thrombomodulin, and uroplakin II (Fig. d-f) but negative for carcinoembryonic antigen (CEA), gross cystic disease fluid protein 15 (GCDFP15), and S100. No high-risk human papillomavirus (HPV) genotype was identified by an automated DNA chip system (Clinichip™ HPV test; Sekisui Medical, Tokyo, Japan) using LBC samples.
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pmc-6359864-1
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A 67-year-old female multiple myeloma (MM) patient presented with an aggressive MM relapse after recent autologous stem cell transplantation (ASCT). She had been diagnosed with MM 6 years previously and had been first treated with cyclophosphamide (Cy), thalidomide and dexamethasone, followed by high-dose melphalan and ASCT. Her disease returned 5 years later, and after re-induction treatment had a second ASCT but unfortunately relapsed again 3 months later, as indicated by pancytopenia, circulating plasma cells in the peripheral blood, an infiltrate of 90% plasma cells in the bone marrow and serum kappa light chains > 1800 mg/dL. Given the aggressive nature of disease at relapse, she commenced treatment with bortezomib 1.3 mg/m2 subcutaneously, (days 1, 4, 8, 11) and 40 mg dexamethasone (D) orally once daily (days 1–4, 9–12) in a 21-day cycle, with a plan to add lenalidomide in later cycles when the pancytopenia had improved.
Although hyponatraemia occurred during the first cycle of treatment on day 8 (Fig. ), there were no significant symptoms and the full cycle of treatment was completed with plasma sodium concentration returning to 135 mmol/L prior to commencement of cycle 2. However, on day 4 of cycle 2, the patient presented with nausea and abdominal pain. Clinical examination was unremarkable, however laboratory investigations revealed severe hyponatremia of 120 mmol/L (normal range 133–146 mmol/L). Urea was 4.2 mmol/L; urinary sodium was 70 mmol/L and urine osmolality was503 mOsm/kg. Thyroid function tests and serum cortisol levels were within normal ranges (TSH 1.13 mIU/L, normal range 0.38–5.33 mIU/L, free T4 9.2, normal range 7.0–13.0 pmol, morning cortisol 430 (normal range 185–624 nmol/L). Regular medications had been unchanged. Owing to her euvolemic volume status, hyponatremia, hypoosmolality, a urine osmolality > 100 mosmol/kg, urine sodium > 40 mmol/L and the timing of onset of hyponatremia, a diagnosis of bortezomib-induced SIADH was made. [] Following endocrinology consultation a diagnosis of SIADH was made.
Fluid intake was initially restricted to 1500 mls daily and subsequently to 800 mL/24 h. Plasma sodium increased only marginally (118 to 121 mmol/L) over 2 days. Plasma sodium also responded poorly to two single intravenous boluses of 100 mL 3% saline over the following 24 h (122 to 126 mmol/L). The urgent need for chemotherapeutic treatment for disease relapse along with the requirement for adjunctive intravenous fluids, prompted escalation of therapy. Therefore, a trial of tolvaptan 7.5 mg orally once daily was commenced. There was a steady rise in plasma sodium concentration of 8 mmol over 24 h with resolution of the patient’s nausea (Table ).
When normonatremia was established, bortezomib was given on cycle 2 day 8, concurrent with tolvaptan when the serum sodium was then 134 mmol/L. Daily tolvaptan was continued, and the rest of the cycle proceeded without further episodes of hyponatremia (Fig. ). The pancytopenia recovered fully and lenalidomide was commenced at a dose of 25 mg once daily as planned with cycle 3. The patient proceeded with bortezomib, lenalidomide, dexamethasone (RVD) along with tolvaptan, without hyponatremia. Tolvaptan was gradually tapered in the following months, owing to the distressing symptom of excessive thirst and stable normonatremia. Initially tolvaptan was tapered to alternate dosing of 7.5 mg once daily, 3 weeks after first being commenced. Seven weeks later, it was tapered to 3.75 mg, every 4 days. Five months after the initial episode, tolvaptan was discontinued entirely. The patient remains in remission nearly 24 months after starting RVD, which she still receives, without hyponatremia.
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pmc-6359868-1
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A 77-year-old female was referred to our hospital with a 1-month history of right wrist pain after housework. She had a medical history of hypertension, dyslipidemia, and no particular notable family history. During physical examination, she reported a slight pain and tenderness in the ulnar side of her right wrist. The swelling or mass were not palpable. Range of motion of the right wrist was slightly disturbed. Plain radiography revealed a comparatively well-outlined osteolytic lesion in the distal end of the ulna (Fig. ). Magnetic resonance imaging (MRI) also demonstrated a bone tumor in the distal end of the ulna. The mass showed iso-intensity on T1-weighted images (T1-WI), high intensity on T2-weighted images (T2-WI), and was heterogeneously enhanced by gadolinium-diethylenetriaminepentaacetic acid (Gd.-DTPA) (Fig. ). No extraosseous masses were observed. Positron emission tomography-computed tomography (PET-CT) showed no abnormal fluorodeoxyglucose (FDG) uptake in the lesion (Fig. ). No distant lesions, including lung lesions were noted. Clinical and imaging findings suggested a benign bone tumor that was enhanced by Gd.-DTPA. It was thought that the tumor was possibly an enchondroma. Initially, we planned to evaluate the benignancy of the tumor with intraoperative frozen section, followed by curettage and bone graft at one stage. However, when considering carefully, characteristics of the tumor did not perfectly match those of any diagnostic categories including enchondroma. In the case of enchondroma, it usually shows no significant enhancement or only marginal enhancement by Gd.-DTPA, however, the whole lesion was heterogeneously enhanced in this case. Therefore, an incisional biopsy was performed.
Incisional biopsy revealed that the tumor comprised atypical spindle cells with hyper-cellularity (Fig. ). The tumor cells were partially positive for epithelial membrane antigen and positive for B-cell leukemia/lymphoma 2 (Bcl-2) protein. Thus, synovial sarcoma was diagnosed based on histologic features and immunohistochemical results, though fluorescence in situ hybridization (FISH) examination filed to detect a rearrangement of SYT from the biopsy specimen.
Following an elaborate plan, the patient underwent a wide resection of the tumor at the distal part of the right ulna with biopsy tract. Any reconstructive soft tissue procedure was not performed. Histopathologically, the tumor occupied the distal end of the ulna, and demonstrated similar characteristics as the specimen obtained from the biopsy (Fig. a). Reverse transcription-polymerase chain reaction (RT-PCR) from the resected specimen and sequencing of RT-PCR products demonstrated a chimeric SYT-SSX1 transcript (Fig. b), confirming the diagnosis of synovial sarcoma.
At the 2-year follow-up, the patient is progressing favorably with 25 points on the Disability and Symptom section of Disabilities of the Arm, Shoulder, and Hand outcome measure and 93 points on the Toronto Extremity Salvage Score, with no evidence of local recurrence, distant metastasis or lung metastasis.
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pmc-6360029-1
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A 64-year-old woman was referred to our hospital for the treatment of chest wall necrosis. She had undergone Halsted surgery and postoperative radiation therapy with cobalt-60 (30 Gray) and megavoltage X-rays (30 Gray) 25 years earlier. Following the treatment, her left upper limb became completely paralyzed.
Six years earlier, she had sustained a left clavicle fracture due to osteonecrosis, and she had subsequently developed a chronic cutaneous fistula measuring 1 cm in diameter. The fracture and cutaneous fistula failed to heal, and she experienced bleeding from the fistula since 1 year. Two months before visiting our hospital, she was hospitalized in the Department of Breast Surgery at another hospital for massive bleeding and local infection. She was treated with antibiotics and cleansing of the fistula and the infection subsided 2 months thereafter. She visited our hospital after discharge.
At initial examination, her height was 153 cm and weight was 34 kg. Necrosed clavicle was exposed through the 6-cm-wide skin defect in the left clavicular region, and air entered and exited the opening from deep within the chest while breathing (Fig. ). Computed tomography (CT) revealed the part of the first to third left ribs, part of the left clavicle, the subclavian artery, and the brachial plexus to be missing. Her upper left limb was nourished by retrograde blood flow from the thoracodorsal artery; however, blood flow was weak to the skin of the arm, the surface of which was cold, resulting in complete paralysis of the upper extremity. Several rounds of debridement eliminated the necrotic tissue, and the local infection was completely resolved. Debridement revealed a tissue defect measuring 4 cm × 8 cm and 4 cm deep at the base and approximately 10 bronchial stumps on the surface of the collapsed lung covered in granulation tissue, from which exhaled air and sputum were effusing (Fig. ). She presented with severe emaciation and malnutrition, but no other systemic anomalies were noted.
After treatment, she had almost no problem with daily life but frequently coughed while taking baths or when exposed to cold air. Therefore, she consented to undergo the surgical closure of the pulmonary fistula. A left latissimus myocutaneous flap could not be used as the reconstructive material due to the absence of vascular pedicle (Fig. ). A free rectus abdominis flap was considered, but an omental flap was ultimately selected for its perceived ability to create the best air seal. Owing to her severe emaciation, the volume of the omentum available for harvest was considered inadequate; therefore, she was administered a diet by a nutritionist for 9 months to improve her systemic nutritional state and thereby increase her resistance. Subsequently, her weight increased from 34 kg to 38 kg, and her serum albumin level improved from 3.0 g/dL to 3.7 g/dL. Reconstructive surgery was performed. Preoperative blood gas analysis revealed pH of 7.400, carbon dioxide partial pressure of 45.3 mmHg, and oxygen partial pressure of 84.3 mmHg. Lung function test performed while holding down the pulmonary fistula revealed forced vital capacity of 1.16 L and forced expiratory volume as 1% of 100%.
Surgery was performed under general anesthesia with selective one lung ventilation.
The damaged skin, clavicle, and ribs surrounding the defect were debrided. The base of the defect was covered with hemorrhagic granulation tissue and was only rubbed with gauze for debridement to avoid over expansion of the bronchial stumps. A free omental flap (approximately 155 cc) with the right gastroepiploic blood vessel as its pedicle was harvested under laparoscopy. The right transverse cervical artery and right anterior jugular vein were anastomosed with the right gastroepiploic blood vessel; the tissue defect was filled with the omentum. A 20/1000-in.-thick split-thickness skin graft was placed over the omentum, and the graft was immobilized with tie-over dressing (Fig. ).
The tie-over dressing was removed on the 5th postoperative day, and the engraftment of the omentum and skin graft was observed to ensure that there was no air leakage (Fig. ). However, a bulge that moved with breathing was noted on the skin graft over the omentum 2 weeks after surgery. CT confirmed this bulge to be the buildup of air between the transplanted omental tissues (omental emphysema). Pressure was applied to the bulge with scrunched up gauze and adhesive tape for 2 months; however, omental emphysema persisted even after 2 months; therefore, the dressing was removed, and she instructed to apply pressure with the hand when coughing. She is currently undergoing the follow-up and has not developed any subsequent emphysematous or respiratory infection (e.g., pneumonia). Omental emphysema persisted almost entirely without change for at least 2 years after surgery, but it spontaneously resolved by the 3rd postoperative year (Fig. ). Changes over time, as observed in CT, at 7 years after the surgery are presented in Fig. .
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pmc-6360035-1
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A 44-year-old white man, with a previous history of gouty arthritis and type 2 diabetes diagnosed two years earlier, was admitted to the emergency department for a reported episode of generalized tonic-clonic seizure at home, lasting 2 minutes. The patient had no recent history of fever or flu-like symptoms but reported a moderate occipital headache in the previous four days. On the initial clinical examination, the patient was lucid and oriented, hemodynamically stable, and with fever (auricular temperature of 38°C). A thorough neurological examination revealed neither meningism signs nor any focal neurological deficit. Fundoscopic examination was normal. Apart from evidence of tongue biting, he had no visible oral or genital vesicular lesions or any skin rash. There were no palpable lymph nodes.
During the observation period in the emergency room, several convulsive episodes were observed, with postcritical agitation and disorientation, requiring sedation with propofol and intubation for airway protection.
A brain computed tomography (CT) scan was performed, revealing cortical and subcortical edema of the left anterior frontal region and a local linear hyperdensity suggestive of a discrete subarachnoid haemorrhage. A cerebral CT venography revealed venous thrombosis in the anterior two-thirds of the superior longitudinal sinus ().
The patient had no prior personal or family history of epilepsy or thrombotic events. There was no history of cancer. His long-term medication was metformin 700 mg and allopurinol 300 mg once a day. He had good metabolic control of type 2 diabetes with a hemoglobin A1c count of 6.2% and no evidence of end-organ damage. Uric acid was in the normal range.
Complete blood count and renal and hepatic function were normal. He had a normal leukocyte count and a red cell distribution width of 13.9%. Inflammatory markers were slightly elevated, the erythrocyte sedimentation rate was 43 mm/h, and the C-reactive protein was 233.3 nmol/L (normal <4.76 nmol/L).
Two sets of blood cultures were collected but had no bacterial growth after 5 days of incubation. An anteroposterior view of a chest X-ray was obtained and showed no evidence of opacities or consolidations. His electrocardiogram had a normal sinus rhythm.
The cytochemical study of cerebrospinal fluid (CSF) revealed 2 leucocytes/mm3, a protein level of 230 mg/L, and a glucose level of 10 mmol/L (serum level of 14 mmol/L). The CSF multiplex polymerase chain reaction was positive for HSV-1 and negative for all the other microorganisms tested (HSV-2, enterovirus, varicella zoster virus, human herpes virus 6, cytomegalovirus, N. meningitidis, H. influenzae, E. coli K1, S. agalactiae, L. monocytogenes, and C. neoformans). Serology for HSV was IgG positive and IgM negative and negative for both HIV and syphilis.
The autoimmune study (antinuclear antibodies, anticardiolipin antibodies, and lupus anticoagulant) was negative or normal. Complement C3 and C4 test and homocysteine were normal. Protein C, protein S, and antithrombin III levels were normal. The molecular study of genetic thrombophilias was negative for prothrombin 20210G>A mutation but revealed heterozygosity for factor V Leiden (mutation 1691G>A).
The diagnosis of CVT secondary to HSV-1 infection in a patient with heterozygosity for factor V Leiden was made. The treatment was started with enoxaparin 1 mg/kg twice daily, levetiracetam 1500 mg bid, and acyclovir 800 mg tid for 14 days. The patient had a positive clinical evolution during hospitalization, with no more seizures and no neurological deficits at discharge. The selected oral anticoagulant was rivaroxaban 20 mg id, maintained for 12 months, without any new convulsive or thromboembolic episodes registered.
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pmc-6360038-1
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A 29-year-old female was admitted to the emergency department (ED) after sudden cardiac arrest at home followed by effective resuscitation. The first recorded heart rhythm was ventricular fibrillation. After effective resuscitation, due to respiratory insufficiency, the patient was intubated and mechanical ventilation was set. Electrocardiography revealed ST-segment depressions up to 1 mm in I, aVL, II, III, and V1-4 leads. The patient was then transported to the ED. At admission to the ED, the patient was hemodynamically stable and preserved systolic blood pressure without inotropes. Considering blood tests, including elevated level of serum D-dimers, at first, computed tomography of the head and chest in pulmonary embolism algorithm was performed. There were signs of cerebral stroke and evident pulmonary embolism. Chest radiograph depicted features of pulmonary edema. At that time, a cardiological consultation was made on the basis of which the cardiac echocardiography was ordered at patient's bedside. Cardiologist consultation and echocardiography revealed impaired left ventricle ejection fraction (LVEF) (approx. 25-30%) with regional contractility impairment (recent akinesis of the lateral, inferior, and posterior walls and hypokinesis of other walls) and moderate mitral regurgitation. The patient was immediately qualified for coronary artery angiography and transferred from ED to the catheterization laboratory (CathLab). During transportation to the CathLab, the first symptoms of CS had developed, and the patient was given first inotrope—noradrenaline. Coronary artery angiography revealed multivessel disease including recessive right coronary artery with 60% stenosis; the left main coronary artery was without significant stenoses, ostial occlusion of the dominant circumflex branch (Cx), left anterior descendent artery (LAD) with multiple significant stenoses: ostial: 80-90%, proximal segment: 70%, medial segment: 80%, and distal segment: 60%, the intermediate branch with 80-90% ostial stenosis. This is presented in Figures and . Percutaneous coronary intervention (PCI) within Cx with thrombectomy and drug-eluting stent (DES) implantation, Onyx 3.5 × 12 mm, 18 atm., was performed and followed by sequential inflation in the intermediate branch (c.b. 1.5 × 12 mm, 20 atm.) and Cx (4.0 × 8 mm, 24 atm.). Due to the progression of CS and no improvement after adding other inotropes (dopamine, dobutamine) at high doses, the decision was made to implant IABP and perform PCI of LAD. Predilation with b.c. 3.0 × 15 mm 20 atm. was performed. Implantation of two DES in the proximal and medial segment was performed: DES Ultimaster (4.0 × 18 mm, 18 atm.) and DES Create (3.0 × 31 mm, 16 atm.). Postdilatation, with b.c. 4.0 × 15 mm, up to 20 atm., was executed using the proximal optimization technique. Then, the patient was then sent to the intensive care cardiology unit in a critical state, with the blood pressure of 40-50 mmHg. Control echocardiography revealed decreased LVEF (around 20%). A fourth inotrope was added (adrenaline) without significant improvement of systolic blood pressure. After consultation at the cardiac surgery department, the patient was qualified for ECMO treatment. The patient was successfully surgically connected to ECMO after transportation to the cardiac surgery unit and surgeons were able to wean from the ECMO, due to the improvement of LVEF after five days. During the long stay at the cardiac surgery unit, several complications developed, including severe bleeding demanding transfusion of more than 16 units of blood products, acute ischemia of the left lower limb, left femoral artery thrombectomy and surgical angioplasty, respiratory tract infections with negative culture (bronchoscopy), pneumonia, pulmonary edema, and recurrent mechanical ventilation necessity. The patient finally survived, was weaned from mechanical ventilation, and was discharged for further rehabilitation after almost 70 days without any neurological deficiencies and able to walk using a walker.
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pmc-6360061-1
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A 2-year-and-8-month-old Swiss girl presented to a pediatric office with fever of 38.8°C, vomiting, and refusal to eat for 3 days. Prior to admission, according to the parents, the patient had drunk ca. 500 ml of fluids. This was a response to a reviewers remark concerning signs of thirst. Blood analysis demonstrated severe hypernatremia (196 mmol/l), prompting urgent hospital admission.
The patient was born at term (40 + 1 weeks of gestation) and had a birth weight of 3390 g. She was delivered through a C-Section due to pathological cardiotocography (CTG) and green amniotic fluid. The APGAR score was 6/8/10. On the second day of life, she developed bilateral parenchymal and intraventricular grade III brain hemorrhage diagnosed by ultrasound. Additionally, she had recurring seizures, which were successfully treated with phenobarbital (3 mg/kg/d). cMRI at two weeks of age showed hydrocephalus with intraventricular hemorrhage in the caudothalamic groove displacing, but not including the thalamus, as well as a small intraparenchymal hemorrhage of the right parietal side and subarachnoid hemorrhage of the left occipital side, along the tentorium and the cisterna cerebellomedullaris, with signs for slight hypoxia.
Postnatally, the patient also had hypernatremia of 180 mmol/l, which was treated with infusion therapy (glucose 5%). We are not aware of any further urine or serum measurements (e.g. osmolality). Neonatal ultrasound showed, slight hyperplasia and no adrenal hemorrhage and tumor. She was discharged with a sodium level of 160 mmol/l. A “central dysregulation” etiology was hypothesized. Sodium levels in the first year were normal (or slightly elevated) and ranged from 136 to 154 mmol/l.
After birth, fT3 and fT4 serum levels were decreased; thus, thyroxine substitution was initiated. The therapy was ended after 1 month because of a hyperthyroid metabolic state (differential diagnosis at that time was euthyroid sick syndrome). Neonatal screening was unremarkable. Two weeks later, at the age of 6 weeks, fT4 was decreased again to 15.6 pmol/l (normal values 17–32), and thyroxine substitution was restarted.
At the age of 1 year, growth arrest (<3rd percentile, before 25–50%) as well as reduced oral intake were observed. IGF1 was reduced to 1.8 nmol/l (normal values 3.67–20.4), IGFBP3 was normal (1.07 mg/l), with no adrenal insufficiency (ACTH, cortisol, aldosterone, renin, FSH, LH, and prolactin normal), sodium was 136 mmol/l, no celiac disease, and bone age was normal. After re-evaluation of cMRI at 2 weeks of age, retrospective diagnosis of pituitary hypoplasia was carried out (ca 60 mm3, normal values 148 ± 37). Growth hormone deficiency was postulated followed by substitution with Norditropin. At the age of 12 months, cMRI was performed again, which showed partial pituitary dysgenesis and hydrocephalus malresorptivus.
The patient was regularly seen in endocrinological and neurological offices. She showed motor development delay which improved partially until the age of 2 years. At this age, a general development delay of 3–4 months and strabismus divergens/alternans on the left side were observed. She had one seizure at 18 months of age lasting 30–40 minutes with postictal paresis of the right arm and facial nerve paresis on the right side; cMRI at that time was unremarkable.
The patient was referred to our hospital in a reduced general condition. On presentation, the skin color was pale and turgor slightly reduced. She had symmetric limb movements with good muscle tonus but appeared tired. The pupils were equal and reacted promptly to light. The percentiles for weight (10.4 kg), length (93 cm), and head circumference (46 cm) were all below the 3rd percentile. Body temperature was 37.6°C, blood pressure was 97/65 mmHg, and heart rate was 102/min. The remainder of the physical examination was normal.
The patient was admitted to the pediatric intensive care unit. Blood gas analysis showed excessive hypernatremia (187 mmol/l) and hyperchloremia (148 mmol/l) with normal pH and base excess (). Osmolality was 362 mmol/kg (normal values 280–300 mmol/kg).
Infusion with isotonic glucose-electrolyte solution (sodium 140 mmol/l and glucose 5%) was initiated. Potassium chloride (7.46%) was added due to mild hypokalemia of 3.02 mmol/l. Blood gas analysis was performed hourly, revealing a slow decrease of sodium to a minimum of 143 mmol/l (), decreasing by an average of 0.5 mmol/l per hour.
Blood glucose on admission was 11.1 mmol/l and normalized with rehydration, suggesting the high blood glucose on admission was due to stress hyperglycemia. Supplementation of thyroid and growth hormones was continued. On the day of admission, urinary excretion was slightly reduced (ca. 2.4 ml/kg/h), and on the second day, the excretion increased (ca. 4 ml/kg/h). There was no fever or edema, vital parameters were stable, and other laboratory tests showed no abnormalities.
The following values were measured on admission: Urine osmolality was 876 mosmol/kg (normal values 50–1200 mosmol/kg), urine antidiuretic hormone (ADH) level was 23.70 ng/l (normal values 1.3–42.4 ng/l), plasma aldosterone level was 7.0 ng/dl (normal values < 9.0 ng/dl), and copeptin pro-arginine-vasopressin (AVP) was 4.4 pmol/l (normal values 1.70–11.25 pmol/l []) The cortisol level was slightly elevated to 32.72 µg/dl (normal values 5–25 µg/dl).
After normalization of serum electrolytes on day 4 of hospitalization, the child developed tremors, particularly while standing, and a general reduction of movement was observed. A slight bilateral rigor of both arms and ataxia were observed. The rest of the neurological evaluation was normal.
A 10/20-electroencephalography (EEG) was normal. MRI of the head showed vague, nonischemic diffusion impairment in the basal ganglia, corpus callosum, and subcortical regions on both sides (), with only discrete signal modulation in the T2 fluid-attenuated inversion recovery (FLAIR) sequence (). No signs for hemorrhage were observed. Slightly dilated lateral ventricles with no active hydrocephalus or indication of increase in pressure were observed. The pons was unremarkable (). The findings were interpreted as meningoencephalitis, so lumbar puncture was performed.
The results of cell count, protein, and glucose in the cerebrospinal fluid (CSF) were normal. Cefotaxime and aciclovir intravenous were initiated. Multiplex polymerase chain reaction (PCR) of the CSF was negative for cytomegalovirus (CMV), Cryptococcus neoformans, Escherichia coli, Enterovirus, Haemophilus influenzae B, human herpesvirus 6 (HHV-6), human parechovirus, herpes simplex virus (HSV) 1/2, Listeria monocytogenes, Neisseria meningitidis, Streptococcus agalactiae (group B streptococcus (GBS)), Streptococcus pneumoniae, and varicella zoster virus (VZV). Additional PCR for Mycoplasma and Rotavirus was negative. The antibody specificity index (ASI) for mumps, measles, rubella, varicella, CMV, Epstein–Barr virus (EBV), herpes simplex and Borrelia, as well as tick-borne encephalitis (TBE) antibodies was normal. CSF examination showed an absence of oligoclonal bands, and only a slight increase of immunoglobulin M (IgM) of 2.4 mg/l (normal up to 1.3 mg/l) and a slight increase in albumin quotient of 8.5 (normal up to 4.2) were noted.
Creatine kinase (CK) was extremely elevated (12794 U/l), with normal values for other muscle enzymes (aspartate transaminase (AST), alanine transaminase (ALT)). Although CK-isoenzyme analysis was not performed, the elevated CK levels were assumed to be mostly brain creatine kinase (CK-BB). Rhabdomyolysis due to excessive hypernatremia remains a possible explanation. No seizures, as a possible explanation, were observed throughout the hospitalization.
After 5 days, the child was transferred by plane to Switzerland, where the family resides.
At the accepting hospital, the neurological symptoms remained. Rehydration was continued and slowly tapered over time. Brain computed tomography (CT) scan was performed to rule out sinus vein thrombosis. Antibacterial and antiviral therapy were stopped. Central dysregulation was discussed again concerning the origin of the hypernatremia. A retrospective review of the MRI was interpreted as extrapontine myelinolysis following extreme hypernatremia. After hospitalization for nearly 2 weeks, the child recovered completely.
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pmc-6360069-1
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The patient is a 49-year-old obese Hispanic male with a past medical history of granulomatosis with polyangiitis (GPA) complicated by ESRD on hemodialysis who presented with dyspnea and chest pain. He was diagnosed with GPA in 2010 via a renal biopsy showing crescentic glomerulonephritis and treated with corticosteroids, plasmapheresis, and cyclophosphamide but had inconsistent follow-up thereafter. He repeatedly visited the ED for epistaxis and ear infections. Pathology from a 2013 arteriovenous fistula (AVF) repair showed granulomatous inflammation.
On admission, the patient was tachycardic, tachypneic, and hypoxic. Exam was notable for saddle nose deformity, distant heart sounds, jugular venous distention, and an AV fistula bruit. Lab examinations revealed normocytic anemia, elevated BUN (56 mg/dL) and Cr (12.3 mg/dL), hyperkalemia, hypophosphatemia, elevated acute phase reactants (ESR 91 and CRP 46.98), elevated α1 and α2 globulins, and increased κ and λ free light chains. Further workup showed negative c-ANCA, positive p-ANCA, elevated myeloperoxidase antibodies (>8 U), normal serine protease 3 antibodies (<0.2 U), and normal complement levels. EKG showed sinus tachycardia with S wave in lead I, Q wave in lead III, and electrical alternans (). CTA was negative for pulmonary embolism but revealed a moderate pericardial effusion and bilateral pulmonary opacities (). Subsequent echocardiogram was consistent with tamponade with a solid component in the effusion ().
The patient received emergent dialysis and a pericardial window. Pericardial fluid was bloody, and pericardial tissue pathology showed acute inflammation, granulation tissue, and fibrinopurulent exudate. He was prescribed pulse dose steroids with a taper and plan for outpatient follow-up for cyclophosphamide initiation. Unfortunately, our patient was rehospitalized within a month of discharge for occlusion of his AVF and sepsis from CMV colitis and E coli bacteremia. He sadly passed away secondary to cardiogenic shock and hypoxic respiratory failure during this hospitalization.
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pmc-6360158-1
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The index patient was a 24-year-old Chinese woman from non-consanguineous parents (). The index patient was healthy on birth. Since the age of 16 years, the index patient has been suffering from mild proteinuria with normal level of serum creatinine (the normal range of creatinine is 44–106 μmol/L for female). No special treatment was recommended and only periodic review was performed. At the age of 20 years, the index patient gradually developed proteinuria which was occasionally accompanied with binocular edema and blurred vision. Angiotensin converting enzyme inhibitors (ACEI) and some traditional Chinese medicine were recommended for the patient, but the result was not satisfactory. Traditional Chinese medications (for example, Shenyan Kangfu tablet, Huangkui capsule) were used to reduce the proteinuria. Gradually, proteinuria and edema became more serious, so the patient was admitted to our hospital to perform further examination at the age of 24 years.
Pathological tests and routine blood tests of the index patient showed the following results: albumin 38.1 g/L (35–55 g/L), triglyceride 2.31 mmol/L (<1.7 mmol/L), HDL-C 2.18 mmol/L (1.29–1.55 mmol/L), LDL-C 2.26 mmol/L (2.7–3.1 mmol/L) and creatinine 54.2 μmol/L.
There was no abnormality in antinuclear antibody (ANA), antineutrophil cytoplasmic antibodies (ANCA), hepatitis B and free light chain. Urine routine test found proteinuria and erythrocyturia, without leukocyturia. Erythrocyturia manifested with dysmorphic erythrocytes, and 24-h quantitative urine protein was 5.067 g. In the index patient, urine protein screening found that the patient has been suffering from non-selective proteinuria. Albumin creatinine ratio (ACR) was 3200 mg/g. High frequency hearing loss was found by further examination of the index patient, without ocular lesion. FSGS like lesion were identified by renal biopsy and renal pathology. In addition, electron microscopic study revealed uneven thickening of GBM with splitting of the lamina densa. Hence, we suspected that the index patient may be suffering from Alport syndrome. Parents of the index patient and other members of this family were completely normal.
Light microscopy study found FSGS () and diffuse vacuolar degeneration of the GBM in the index patient (). Tubular epithelial cells were swollen or granulated, and scattered foam cells were found in the interstitium.
Immunofluorescence study revealed that the α3 and α5 collagen IV chains were well distributed in the GBM () and IgG, IgM, IgA, C3, C1q, and C4 staining were negative. No immunoglobulin and complement components were deposited outside the glomerulus.
Electron microscopic examination found that the thickness of GBM was not regular and the density was also uneven. The dense layer of GBM was tearing and stratified. Mesangial insertion could be seen in few segments (). There was diffuse effacement of foot processes.
Based on the clinical symptoms, the index patient and her parents were recommended for performing molecular genetic diagnosis by targeted next generation sequencing and confirmatory Sanger sequencing.
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pmc-6360269-1
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A 19-year-old Saudi male was referred to the Department of ENT at our institution with the complaint of facial pain over the upper jaw area along with post-nasal discharge. This complaint has developed over a period of 6 months prior to his presentation. The patient gave a history of recurrent sinusitis but had no other systemic illness, no past surgical history and no history of trauma. No known drug history, no family history of any genetic disorder. The patient and both parents are non-smokers.
Endoscopic examination was unremarkable except for a septal spur to the left side. Paranasal sinuses computed tomography (CT) scan showed bilateral cystic lesions and ectopic teeth in both maxillary sinuses ().
The patient was booked for endonasal endoscopic enucleation of the cysts and extraction of the ectopic impacted teeth.
Intra-operative, bilateral big cystic masses completely filling both maxillary sinuses were visualized along with a tooth impacted in the floor of the left maxillary sinus and another tooth identified within the right osteomeatal complex obstructing the right maxillary ostium.
Bilateral endoscopic wide middle meatal antrostomies were performed under general anesthesia. The cystic masses were dissected from the wall of both maxillary sinuses and removed by using different angel forceps and endoscopes. The right tooth was obstructing the maxillary sinus drainage () removed with the cyst while the left was impacted in the left inferiolateral walls of left maxillary sinus () removed completely with angled giraffe forceps (). Homeostasis was achieved in both sinuses and no nasal packing was needed.
The specimen was sent for histopathologic examination which confirmed the diagnosis of dentigerous cysts.
The patient’s symptoms were resolved completely post-operatively and remained free of symptoms for 5 years follow up.
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pmc-6360270-1
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A 65-year old male patient with early gastric cancer was transferred from Aruba to our institution. He had a 3-year history of black stools and anemia. His past medical history included multiple comorbidities: diabetes, chronic renal failure, alcoholic cirrhosis Child A, complete heart blockade and thrombocytopenia of unknown etiology. An upper endoscopy and biopsy revealed a well-differentiated intestinal type adenocarcinoma in the antrum. Endoscopic ultrasonography showed a hypoechoic, 3.2 cm neoplasm, without muscularis externa infiltration and reactive ganglia (). Endoscopic mucosal resection was chosen due to tumor size, stage and comorbidities of the patient. The tumor was fully resected without complications. At the end of the procedure the anesthesiologist had difficulty with ventilation and abdominal distention was observed (). He had a 128/91 mmHg blood pressure and 70 bpm heart rate. An endoscopic revision was done before finishing the procedure, without identification of any macroscopic perforation. A nasogastric tube was placed and therapeutic strategies to improve abdominal-wall compliance were instituted (changes in ventilation parameters, nasogastric suction, change to a supine position and removal of any strap over the abdomen). A plain abdominal radiography in the operating room showed a massive pneumoperitoneum (). Decision of a nonsurgical management was conducted and the patient was taken to the intensive care unit (ICU) for monitoring. The IAP measured by a trans-bladder catheter was 33 mmHg. Six hours after ending the procedure the patient developed dyspnea and anuria. The diagnosis of an abdominal compartment syndrome was established. Given the worsening status, interventional radiology evaluated the patient. A CT scan confirmed the massive pneumoperitoneum without intraperitoneal extravasation of contrast (a & b). A percutaneous decompression guided by CT scan was performed with a pigtail catheter G14 (c & d). Air was immediately released under pressure. Immediately after the procedure, the patient's symptoms and hemodynamic status improved. Diuresis returned after a few hours. The pigtail catheter was closed the first day after placement and taken out at the third postoperative day. Control CT scan revealed no evidence of pneumoperitoneum. Pathology report confirmed the resected specimen had free malignant cell margins and areas of high-grade and low-grade dysplasia. The patient was discharged from ICU at postoperative day 2 and discharged from hospital at postoperative day 5 without further complications.
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pmc-6360319-1
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A 30-year-old male presented to our clinic with inability to extend all fingers at the metacarpophalangeal joints of the left hand. One year prior to his presentation, he was involved in a car accident and was treated at a local hospital. The medical report indicated that the injury involved the dorsal aspect of the hand, wrist and forearm. There was degloving of the skin without skin loss. However, there was extensor tendon loss of all fingers extending from the proximal one third of zone 6 to zone 8 (including the musculotendinous junctions). Other than debridement, nothing was done to the extensor tendons; and the skin was closed primarily. On examination, there was no active extension at the metacarpophalangeal joints. There was no stiffness with full passive extension at the metacarpophalangeal joints. There were no deficits in active wrist extension/ finger flexion. The overlying skin was scarred but was thought to be adequate for soft tissue coverage. Two-staged extensor tendon reconstruction was planned. However, clinical examination showed the absence of palmaris longus tendon bilaterally. Ultrasound examination also showed the absence of plantaris tendon bilaterally. The patient was counselled regarding choices of the other sources of tendon grafts including: multiple toe extensors, “split” tensor fascia lata, and “split” flexor carpi radialis. The latter option was chosen and surgery was planned. In the first stage, exploration confirmed the presence of extensor tendon defects from the proximal one third of zone 6 to zone 8; including the musculotendinous junctions (). Four silicone rods were inserted. The rods were sutured distally to the remnants of the extensor tendons at the dorsum of the hand. All 4 rods were left free (un-sutured) in the distal forearm (). the patient resumed passive exercises of the metacarpophalangeal joints post-operatively to prevent stiffness. The second stage was done five months later (). The flexor carpi ulnaris tendon was cut near its insertion and was then transferred to the dorso-ulnar aspect of the forearm. The tendon of flexor carpi radialis was harvested as a tendon graft. The tendon graft was split distally in 4 slips on the operating table. The proximal tendon repair was done first between the transferred flexor carpi ulnaris tendon and the tendon graft using multiple figure-of-eight 3/0 polypropylene sutures. The splits of the flexor carpi radialis tendon graft were sutured to the proximal ends of the silicone rods. Each rod was then pulled out from the distal end; thereby introducing each slip into its corresponding pseudo-sheath induced by the rod. Each slip was then weaved through the corresponding remnant of the extensor tendon; and suturing was done using 4/0 polypropylene sutures (). In order to adjust tension of the repair, the wrist was positioned in 30° of extension. Tightening of the distal tendon repair was then done so that the metacarpophalangeal joints were 20° short of the full extension. A plaster cast was applied to the hand and the wrist to protect the repair. The cast was removed at 5 weeks and physiotherapy exercises were started. There were no post-operative complications. At final follow up (1 year later), full active wrist extension/ finger flexion was maintained (). Full active extension of the fingers at the metacarpophalangeal joints was demonstrated with slight wrist flexion (). As expected, active wrist flexion was limited to 20° because of the harvesting of the both wrist flexors (). Power grip was 83% of the contra-lateral hand. The patient was satisfied with the result and resumed his original job as a manual worker without any reported difficulties.
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pmc-6360322-1
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A 36-year-old male patient with no other medical conditions presented to our outpatient clinic with a history of weak urine stream, dysuria, frequency and urgency for the preceding 3 years. The patient was diagnosed in another hospital with prostatitis and given a full course of ciprofloxacin resulting in no improvement. His medical history was not significant in terms of previous urinary tract infections, urethral catheterization, perineal trauma or ejaculatory issues.
His International Prostate Symptom Score (IPSS) was 22, while his score for quality of life due to urinary symptoms was 5. A digital rectal examination revealed a firm, nontender prostate without palpable nodules. Urine analysis results were normal, and culture was sterile. Urine cytology was not suggestive of malignancy. His serum prostatic-specific antigen (PSA) level was 0.875 mcg/l. Other biochemical laboratory examinations were within normal ranges. The maximum flow rate was 6 ml/s with a flat curve.
Pelvic ultrasound revealed a cyst measuring 1.5*1.2 cm that was most likely associated with the proximal part of the prostate gland. The full volume of the urinary bladder was 476 ml, and the postvoiding residual volume was 127 ml. The prostate gland was 38 g ().
CT urography was performed to exclude an ectopic ureterocele. A prostatic cyst measuring 1.5*1.4 cm in size was present at the midline of the upper part of the bladder neck region ().
MRI revealed a prostatic cyst measuring 1.6*1.3 cm with no clear communication with the urethra ().
The patient was scheduled to undergo transurethral resection of the cyst. Under general anesthesia, the patient underwent cystourethroscopy with a 17 French flexible cystoscope. The cyst was obstructing and located at the bladder neck. Due to the age of the patient and the potential risk of retrograde ejaculation, a decision was made to incise the cyst. Retroflexion of the cystoscope clearly revealed the cyst (-B). Therefore, using retroflexion of the cystoscope, a transverse incision was performed in the superior part of the cyst using Holmium Laser. No intraoperative complications were observed (-C).
Postoperatively, the patient complained of minor hematuria and dysuria, which resolved a few days later. The patient’s lower urinary tract symptoms resolved immediately. His last follow-up examination at six months postoperatively showed no recurrence of lower urinary tract symptoms.
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pmc-6360324-1
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A 79-year-old man, who underwent subtotal esophagectomy and reconstruction using a gastric tube 11 years ago, visited a primary care doctor with abdominal pain and no previous signs of disease recurrence over a period of 5 years. Ultrasonography revealed dilatation of the biliary tree and he was referred to our hospital.
Laboratory test showed no hepatorenal or hematological abnormalities. It was observed that the titer of carbohydrate antigen 19-9 increased slightly to 54.1 U/ml, though carcinoembryonic antigen, DUPAN-2, and Span-1 were within normal range.
Contrast-enhanced computerized tomography (CT) showed a low-density area of 20 mm in the pancreatic head at the convergence of the dilated common bile duct and the main pancreatic duct. The tumor did not reach the surface of the pancreas and did not invade the GDA (). The patency of the RGEA, right gastroepiploic vein (RGEV), right gastric artery (RGA), and right gastric vein (RGV) were confirmed (, ). There was no distant metastasis.
Magnetic resonance imaging showed a tumor with irregularly low-intensity on T2 weighted image, mild low-intensity on T1 weighted image, and gradual enhancement with contrast medium from the margin.
A combination of positron emission tomography with fluoro-2-deoxyglucose and CT confirmed the tumor as a thin uptake area with a maximum standard uptake value of 2.6.
Based on the above findings, the patient was diagnosed with resectable pancreatic head cancer. However, as electrocardiogram revealed a complete left bundle branch block and coronary angiogram showed a 99% stenosis of a coronary artery branch, a coronary artery stent was placed. Obstructive jaundice occurred a few days following the stenting and an endoscopic biliary stent was placed. The operation for the pancreatic cancer was performed a month after biliary drainage.
Following laparotomy by upper midline incision, peritoneal exploration was conducted but revealed no peritoneal metastases. Although palpable as a hard mass of about 3 cm in diameter, the tumor was not observable on the anterior surface of the pancreatic head, so the RGEA and RGEV could be preserved.
Dissection of the paraaortic lymph nodes (#16b1-inter, -pre, and -lat) was performed using Kocher’s maneuver, and the nodes were perioperatively confirmed by pathology to be negative for cancer cells. Henle’s trunk was exposed and the inferior pancreaticoduodenal vein and accessory right colic vein, which are tributaries of the trunk, were resected. However, the RGEV, another tributary of Henle’s trunk, was preserved.
The GDA and proper hepatic artery were confirmed after resection of the supraduodenal arteries following the dissection of the lymph nodes around the common hepatic artery (#8a and 8p). After the gallbladder was detached from its hepatic bed, the common hepatic duct was skeletonized and disconnected. The lymph nodes of the hepatoduodenal ligament (#12a, 12p, and 12b) were dissected. After adequately separating the GDA from the posterior surface of the duodenum, the duodenal bulb was cut 2 cm distal to the pyloric ring using GIA 60.
The jejunum was cut about 15 cm distal to the Treitz ligament and the mesentery was resected with the first jejunal artery (J1A) and vein. After skeletonizing the common root of the J1A and the inferior pancreaticoduodenal artery from the superior mesenteric artery (SMA), the root was resected. The proximal cut end of the jejunum was then drawn out to the right and passed posterior to the SMA and the superior mesenteric vein (SMV), and some small SMV branches from the pancreatic head were resected.
The GDA-RGEA was completely separated from the pancreatic head by resecting 3 small branches and the posterior superior pancreaticoduodenal artery. After confirming the inflow site of the RGV, the pancreatic parenchyma was cut with a scalpel along the left margin of the portal vein (PV)-SMV following the tunneling method. Pylorus-preserving pancreatoduodenectomy (PPPD) was then completed after dissecting the lymph nodes along the SMA (#14p, 14d), and the plexus of the pancreatic head and the right half of the SMA ().
Although the reconstruction was performed based on the modified Child procedure, Roux-en-Y procedure was adopted because, in aiming to preserve the RGA, RGV, RGEA and RGEV, the degree of freedom was limited. Stents were not used in duct-to-mucosa pancreaticojejunostomy and hepaticojejunostomy. Prophylactic closed drains with continuous aspiration were placed around hepaticojejunostomy and pancreaticojejunostomy.
The histopathological examination of the resected specimen showed that, although the moderately differentiated invasive ductal carcinoma reached the bile duct and duodenum and metastasized to 1 of the 18 resected regional lymph nodes, the tumor did not extend to the surface of the pancreas. The tumor was stage II according to the TNM classification of the Union for International Cancer Control (7th edition).
Although postoperative physical rehabilitation took a relatively long time because the patient had a poor preoperative physical condition (American Society of Anesthesiologists performance status 3), he did not experience complications like pancreatic fistula or surgical site infection. He was discharged on postoperative day 36 and remains alive with no disease recurrence 5 years and 3 months after the surgery.
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pmc-6360347-1
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A 65-year-old female who suffered from progressive dysphagia for six months was admitted to our hospital with the diagnosis of mid-oesophageal carcinoma. Subsequently, she underwent left thoracotomy, oesophagectomy and intrathoracic oesophagogastrostomy (anastomotic stoma located in the cupula pleurae above the top of the aortic arch; pathological examination: moderately differentiated squamous cell carcinoma, invading the tunica adventitia of the oesophagus, with negative upper and lower incisal margins; staging: pT3N1M0). On the 11th postoperative day (subsequent days refer to the first operation), she began to vomit foul-smelling gastric juice; gastroscopy found a thoracic anastomosis fistula, and the size of orificium fistulae accounted for 1/3–1/2 of the anastomotic circumference (A). Therefore, a second surgery was performed on the 18th postoperative day to reanastomose the oesophagus and stomach in the neck. After this surgery, the patient presented with a cervical anastomotic fistula. One week later, thoracodorsal orificium fistulae, with a diameter of 2.5 cm, appeared in the first thoracic surgical incision, and approximately 400 ml of black gastric juice outflowed every day. On the 30th postoperative day, barium oesophagogram revealed that the contrast agent outflowed from the thoracodorsal sinus tract (B), and gastroscopy confirmed a 10-cm long longitudinal gastric fissure (approximately 20–30 cm away from the patient’s incisor), which appeared on the greater curvature side (C). After effective drainage, dressing changes and positive anti-infection measures, the cervical anastomotic fistula healed, while the patient gradually developed respiratory dyspnoea. On the 80th postoperative day, CT and fibreoptic bronchoscopy found a bound tracheostenosis located in the midtrachea (D, E). When thoracic cavity infection was limited and respiratory dyspnoea was relieved, a third surgery was performed, on the 90th postoperative day, to correct the tracheostenosis, repair the thoracic gastric fissure by filling it with a pedicled muscle flap, partly resect ribs 4–7, and close the thoracodorsal orificium fistula and vomicae (F). Afterwards, adequate nutrition support, thoracic washing and effective chest drainage, dressing changes, improved anti-infection control and other comprehensive treatments were carried out. The patient completely recovered and was discharged six months after admission.
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pmc-6360367-1
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A 58-year-old man was admitted to our hospital with a two-month history of facial erythema and dry cough. He had no remarkable medical history except for essential hypertension five years ago. He was a current smoker with a smoking history of 37 pack-years. Although he initially seemed well and his vital signs were normal, a thorough physical examination revealed characteristic cutaneous manifestations of DM. He had a macular rash along with swelling on his forehead and upper eyelids (Fig. A), suggestive of a heliotrope rash. He also displayed a shawl sign characterized by a widespread, flat, reddened area on his upper back, shoulders, and posterior neck (Fig.B). Additionally, he had a flat, red rash on the back of the fingers and hands, indicating a Gottron’s sign (Fig. C). We observed a marked elevation of muscle enzymes in his serum, including aspartate transaminase (294 IU/L), creatine kinase (7833 IU/L), aldolase (50.3 U/L), and lactase dehydrogenase (606 IU/L) (Table ). Soon after admission, he felt muscle weakness, but not muscle pain, in his extremities. Manual muscle test detected reduced strength in his bilateral deltoid and hamstring muscles (grade 4/4), as well as iliopsoas muscles (grade 2/3), suggesting proximal muscle impairment. Based on the suspicion of idiopathic inflammatory myopathies, further analysis of auto-antibodies in patient’s serum revealed the presence of TIF1-γ auto-antibodies, but not that of anti-aminoacyl-tRNA synthetases (ARS), including anti-Jo-1, anti-PL7, anti-PL12, anti-EJ, anti-OJ, anti-KS, melanoma differentiation-associated gene 5, and anti-Mi-2 (Table ). Thus, the patient was tentatively diagnosed with possible DM and TIF1-γ positive myopathy. Fat suppressed T2-weighted magnetic resonance imaging coronal image demonstrated a high-intensity lesion in the bilateral rectus femoris, right vastus lateralis, vastus medialis, and bilateral obturator muscle. Needle electroneuromyography showed a myopathic pattern, with motor unit potentials diminished in amplitude as well as duration. Based on the DM diagnostic criteria recommended by Bohan & Peter and The Research Committee of the Japanese Ministry of Health and Welfare in 2015, we diagnosed the patient with TIF1-γ positive DM.
In addition, a routine chest X-ray (Fig. D) performed during admission showed a mass in the right middle lung field. Thoracic and abdominal contrast-enhanced computed tomography (CT) identified an inhomogeneously enhanced solitary mass (4 cm in size) in the right upper lobe (Fig. E), with ipsilateral hilar lymphadenopathy (Fig. F) as well as liver and left adrenal metastasis. Subsequent bronchoscopy and tumour biopsy confirmed lung adenocarcinoma. Thereby, the patient was diagnosed with cT2bN1M1b (stage IV) lung adenocarcinoma combined with TIF1-γ positive DM.
Following diagnosis, he was treated with 75 mg/day oral prednisolone for myopathy, which alleviated his muscle weakness, and improved his serum muscle enzymes and skin lesions within two weeks. At day 12 from admission, the patient was administered intravenous chemotherapy with cisplatin, pemetrexed sodium hydrate, and bevacizumab for lung adenocarcinoma. However, on day 19, he developed dysphagia, which was confirmed by a videofluoroscopic swallow study. He displayed hypopharyngeal muscle weakness, dysfunction of laryngeal closure, and ineffective oesophageal motility. Over the following one month, his posterior wall of the oesophagus at the level of the entrance considered to be ruptured due to emergence of oesophagus diverticulum on a repeated videofluoroscopic swallow study along with advent of both cervical subcutaneous emphysema on chest X-ray or the air in the cervical oesophageal wall on cervical CT. Thereafter, his general condition gradually deteriorated. Although the weakness in his extremities and his skin lesions were controlled, his oropharyngeal dysphagia persisted with conservative therapy, and his lung tumour was resistant to chemotherapy, resulting in his death due to respiratory failure six months later.
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pmc-6360458-1
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A 67-year-old man with no medical history consulted a nearby doctor for the main complaints of fever and lower abdominal pain. Laboratory analysis revealed hemoglobin, 13.1 g/dL; white blood cell count, 13.76 × 103/μL; platelets, 12.7 × 104/μL; and C-reactive protein, 1.41 mg/dL. He was diagnosed with acute appendicitis, and oral antibiotic treatment was initiated. On the following day, he was referred to our hospital for suspected DIC, as laboratory analysis revealed hemoglobin, 13.3 g/dL; white blood cell count, 3.55 × 103/μL; platelets, 7.4 × 104/μL; and C-reactive protein, 12.2 mg/dL. At the time of hospital consultation, physical examination revealed stable cardiorespiratory dynamics and a fever of 38.3 °C, no abdominal distension, and only slight spontaneous abdominal pain without tenderness and peritoneal irritation. Laboratory analysis revealed hemoglobin, 14.0 g/dL; white blood cell count, 9.41 × 103/μL; platelets, 6.9 × 104/μL; serum total protein, 5.2 g/dL; serum albumin, 3.3 g/dL; total bilirubin, 1.6 mg/dL; aspartate aminotransferase, 218 IU/L; alanine aminotransferase, 198 IU/L; lactic acid dehydrogenase, 315 IU/L; blood urea nitrogen, 20 mg/dL; creatinine, 0.96 mg/dL; C-reactive protein, 13.47 mg/dL; prothrombin activation, 54%; international normalized ratio of prothrombin time, 1.36; fibrinogen/fibrin degradation products, 116.4 μg/mL; and antithrombin III activity, 70%. The sequential organ failure assessment score was 2 points. The Japanese Association for Acute Medicine DIC diagnostic criteria score [] was 7 points (platelet counts; 3 points, prothrombin time; 1 point, and fibrin/fibrinogen degradation products; 3 points). Contrast-enhanced computed tomography (CT) demonstrated an enlarged appendix (10 mm in diameter) without fecalith, ascites, intraperitoneal free air, and abscess (). There was no evidence of perforating appendicitis. Laboratory analysis revealed septic DIC. The patient was diagnosed with non-perforating acute appendicitis with septic DIC. The patient was distressed regarding whether he should be treated conservatively with an antibiotics-first strategy or undergo an appendectomy, because he had few symptoms, no perforation, and no abscess. Ultimately, laparoscopic appendectomy was performed due to anxiety about exacerbation of septic DIC. The resected specimen revealed a necrotized appendiceal mucous membrane. There was no evidence of appendiceal wall perforation (). Histopathological examination showed non-perforating gangrenous appendicitis. He required DIC therapy (thrombomodulin administration, antithrombin administration, and nafamostat mesilate) for 2 days postoperatively. Preoperative blood culture detected Bacteroides thetaiotaomicron. He was discharged on postoperative day 9, and remained in good health 1 month after surgery.
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pmc-6360460-1
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A fifteen year old Caucasian female (BMI 25 kg/m2) was sent to the surgical office by her primary care physician for a one-month history of increasingly intermittent, right upper quadrant, colicky abdominal pain that radiated to the back. She experienced the onset of pain within 15–20 min following a meal and it spontaneously resolved in thirty minutes. She reported nausea when pain is most severe, but otherwise denied further symptoms. Further history was noncontributory with exception of her mother and sister requiring cholecystectomy at a similar age. The abdominal ultrasound obtained from an outpatient imaging center reported no cholelithiasis, wall thickening, murphy’s sign, and a common bile duct measuring at 3.6 mm. Physical exam in office was unremarkable, noting no jaundice or pallor. Abdominal exam revealed scaphoid, soft abdomen, without mass or explanation/etiology for postprandial abdominal pain. After further discussion with the patient and her mother, she was sent for a HIDA -CCK to evaluate for biliary dyskinesia.
The patient returned to the office the following week with HIDA revealing a patent cystic and common bile ducts without evidence of acute cholecystitis. The patient’s ejection fraction was measured to be 96.5% following CCK administration. Ultrasonographer report stated the patient exhibited no reproduction in symptoms during infusion of CCK. We discussed findings with the patient and the decision was made to perform esophagogastroduodenoscopy (EGD) with biliary crystal analysis to exclude microlithiasis, gastritis, or peptic ulcer disease as the etiology of her symptoms. EGD was performed with gastric antral biopsies and bile collection. Pathology revealed no significant inflammation, intestinal metaplasia, dysplasia, or malignancy. Biliary crystal analysis was negative for monosodium urate or calcium pyrophosphate crystals. One-month trial of a proton pump inhibitor with a gastroesophageal reflux (GERD) minimizing diet performed without symptomatic relief. The patient returned to the office where ROME criteria for Irritable Bowel Syndrome (IBS) were ruled out and ROME IV criteria for biliary dyskinesia reviewed () []. Patient was then taken for an elective cholecystectomy. The procedure was without complication and grossly the gallbladder appeared non- inflamed, dilated, or hydropic. Pathological exam as seen in , , returned a normal gallbladder wall without thickening or inflammation.
Postoperatively the patient reports she is now pain-free, tolerating all foods without reproduction of symptoms. Three month telephone follow up finds that the patient continues to be pain free without symptom recurrence.
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pmc-6360536-1
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The patient is a 48-year-old male with past medical history of human immunodeficiency virus (HIV) on highly active antiretroviral therapy (HAART) and diverticulitis who presented to the ED with fevers and chills for 1 week associated with diarrhea, head and neck pain, and photophobia. He denied any abdominal pain and stated that his diarrhea had resolved by the time he was seen. His highest temperature at home was 104°F. He has had 2 episodes of acute diverticulitis in the past 2 years, in which he complained of left lower quadrant pain associated with nausea and vomiting. He stated that this current episode was unlike the past.
On physical examination, the patient had a temperature of 99.3°F, heart rate of 110, blood pressure of 148/84 mm Hg, and breathing at a rate of 16 with 95% oxygen saturation on room air. He was completely alert and oriented, with no neck stiffness. He had tenderness to palpation in the left upper quadrant and in the periumbilical area. Rest of his exam was essentially normal. His laboratory data revealed a white blood count of 11.4 k/cu·mm (reference range: 4.0–11.0 k/cu·mm), hemoglobin of 15.7 g/dl (reference range: 13.0–17.0 g/dl), glucose of 102 mg/dl(reference range: 70–99 mg/dl), sodium of 131 mmol/L(reference range: 133–144 mmol/L), chloride of 97 mmol/L (reference range: 98–107 mmol/L), and ALT of 56 IU/L (7.0–52.0 IU/L). The rest of the laboratory data was within normal limits.
The patient underwent a computed tomography (CT) head, and lumbar punctures which were negative for meningitis. He then underwent a CT of his abdomen and pelvis in the emergency department which showed wall thickening of the sigmoid colon with pericolonic induration suggestive of acute sigmoid diverticulitis (). On further review, CT abdomen also showed haziness/induration of the mesentery along the entire course of the inferior mesenteric vein with partial filling defects throughout its course suggestive of inferior mesenteric vein thrombophlebitis (). The patient was admitted and infectious disease, general surgery, and gastroenterology followed up the patient while in the hospital and was on intravenous cefoxitin and metronidazole. His blood cultures were positive for E. coli resistant to ciprofloxacin. His condition improved and was transitioned to oral cephalexin. He was started on oral anticoagulation with rivaroxaban and discharged with outpatient follow-up with infectious disease and general surgery.
On follow-up, the patient did not report any abdominal symptoms with no reported episodes of bleeding. Given multiple episodes of diverticulitis, the patient opted for surgical intervention and underwent laparoscopic sigmoid colon resection 3 months later. The surgical specimen reported multiple diverticula without any gross abnormalities. The patient continued to take anticoagulation medication for a duration of 6 months and did not report any abdominal symptoms or adverse events. A repeat CT abdomen was not done at the end of his course as the patient did not report any symptoms, and there is no current evidence to support repeat imaging to document recanalization. The patient underwent right upper quadrant ultrasound 1 year after the surgery for elevated liver enzymes which showed increased echogenicity within the liver but no focal abnormalities, a patent portal vein, no duct dilation, and no evidence of extension of thrombus.
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pmc-6360539-1
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A 17-year-old boy living in a care home due to his cerebral palsy was referred to “Imam Khomeini Hospital Complex”—a tertiary care educational hospital in Tehran, Iran—with a two-month history of fever, abdominal pain, and constipation. A VP shunt had been inserted for him at age four for treating hydrocephalus and subsequent refractory seizures. His recent symptoms had an intermittent pattern subsiding transiently with symptomatic therapies such as antipyretics and laxatives. Empiric antibiotics too, including parenteral ceftriaxone, were prescribed for the patient several times, but no response was achieved.
At presentation, the physical exam revealed a blood pressure of 90/60 mmHg, a pulse rate of 100/min, a temperature of 38.5°C, and generalized tenderness in the abdominal palpation. The initial laboratory findings revealed increased erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) and a mild leukocytosis ().
Although physical examination was not compatible with peritonitis, we asked for a confirmatory abdominal X-ray which was unrevealing. The abdominal ultrasound done for more evaluation of pain showed a pseudocyst exactly at the distal end of the VP shunt. To evaluate the shunt infection, cerebrospinal fluid samples were taken from lumbar and shunt punctures and sent for analysis and culture simultaneously (). To rule out any possible contraindication for lumbar puncture (LP), we ordered a brain computed tomography (CT) scan without contrast which showed normal results for the patient and his history.
The analysis of CSF obtained from VP shunt reservoir and spinal canal was suggestive of a bacterial shunt infection (). Considering the previous antibiotic administration, and for the coverage of both Gram-positive and Gram-negative microorganisms, we treated the patient empirically with meropenem and vancomycin.
Five days later, the result of CSF and blood cultures (Bact/Alert 3D Microbial Identification System) was reported: Brucella spp. We then tested the blood for Wright, Coombs Wright, 2 Mercapto Ethanol agglutination test, and anti-Brucella IgG (ELISA), all of which were compatible with diagnosis of brucellosis (). Since this was an unusual result, we decided to take a more detailed history from the patient's caretakers. The notable finding was consumption of unpasteurized cheese before the symptoms had started.
According to abovementioned findings, anti-Brucella treatment with oral rifampicin (15 mg/kg), oral trimethoprim-sulfamethoxazol (5 mg/kg trimethoprim component twice a day) and parenteral ceftriaxone (1000 mg twice a day for covering CNS infection) was started for the patient with diagnosis of VP shunt infection with Brucella spp. Considering our patient's condition that suffered from cerebral palsy and could not take enough care against esophagitis as an adverse effect of doxycycline (one of the first line drugs in treating brucellosis), we avoided including this medication in our regimen.
As our hospital is one of the busiest centers in the country and our patient needed intensive care before and after any surgery, it took three weeks to replace his shunt with extraventricular drain (EVD). His EVD was removed after 5 days when CSF culture was negative, and our neurosurgeons inserted a new VP shunt for the patient. Following six weeks of triple therapy, the patient was discharged on oral antibiotic therapy with trimethoprim-sulfamethoxazol and rifampicin while he was fine and symptom-free. We planned to continue antibiotic therapy for six months and to have three follow-up visits during this period, after which a lumbar puncture will be done to confirm the disease has eradicated.
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pmc-6360552-1
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A 6-year-old boy, with a history of recurrent throat infections presented to Children's Memorial Hermann Hospital (CMHH) following 5 days of fever, sore throat, nasal congestion, and cough. Prior to presentation to CMHH, on day 1 of illness, he was diagnosed with influenza infection (clinical diagnosis) by his primary care physician (PCP) and prescribed oseltamivir which was discontinued on day 3 due to nausea and vomiting. Subsequently, on the 5th day of illness, he started to have abdominal and joint pain (left knee, right ankle, and right elbow). He was noted to be lethargic and had decreased oral intake with dark urine and, thus, was brought to CMHH emergency center (EC). In the CMHH EC, he was febrile (T 39.4°C), hypotensive (blood pressure 78/47 mm Hg), tachypneic (respiratory rate 33 per minute), and tachycardic (heart rate 160 per minute). He was admitted to a pediatric intensive care unit and was started on intravenous cefepime and vancomycin empirically. He lives with his mother and 3 siblings (aged 2, 9, and 10 years), all of whom were healthy with no current or prior symptoms.
Physical examination revealed an acutely ill but responsive boy with crusted lip lesions, cervical lymphadenopathy, nasal congestion, nonpurulent pharyngeal erythema, systolic murmur, and hepatosplenomegaly and no sign of arthritis. Initial lab studies showed a normal white blood cell (WBC) count (11,600/mm3) with an unremarkable differential, thrombocytopenia (36,000/mm3), anemia (hemoglobin 8.9 g/dL), hypoalbuminemia (1.9 g/dL), proteinuria (100 mg/dL), sterile pyuria (WBC 21/high powered field), and markedly elevated inflammatory markers (C-reactive protein 182 mg/L; erythrocyte sedimentation rate >100 mm/hr). Blood cultures drawn on admission along with pharyngeal culture were positive for GAS within 24 hours.
An echocardiogram was obtained due to the severity of presentation, presence of a murmur, and concern for infective endocarditis (IE). The transthoracic echocardiogram revealed echogenic tissues on the septal leaflet of the tricuspid valve (both on septal and anterior leaflets), tricuspid valve leaflet perforation with possible chordal disruption indicative of IE (). Chest radiographs were normal throughout the admission. Magnetic resonance imaging (MRI) of the body was obtained to evaluate for foci of dissemination and revealed bilateral nephromegaly with bilateral wedge-shaped areas of abnormal signal (possible pyelonephritis and/or thromboembolic disease), osteomyelitis involving the right inferior pubic rami and right obturator externus muscle abscess, periportal edema with hepatosplenomegaly, and bilateral pleural effusions.
Meeting the diagnostic criteria for STSS (hypotension with coagulopathy (thrombocytopenia), pleural/peritoneal effusions with hypoalbuminemia, myositis, and isolation of GAS), his antimicrobial therapy was changed to intravenous penicillin G and clindamycin. Subsequent blood cultures were negative. He did not receive intravenous immunoglobulin. Consultations with cardiology and orthopedic surgery were obtained, but no interventions were needed during hospitalization. He progressively improved and was treated with clindamycin for 1 week and penicillin G IV for 6 weeks to treat GAS IE with close cardiology follow-up for possible tricuspid valve repair.
This is particularly unusual presentation in an otherwise healthy child. GAS invasive disease may be associated with specific GAS clones harboring genetic elements or mutations that predispose to severe disease. Given the severity of diseases (STSS) and unusual multifocal presentation with endocarditis, we performed bacterial WGS, as we have previously described [], to define the bacterial strain characteristics associated with the pharyngeal and blood strains. The pharyngeal and blood GAS isolates from the patient were identified as emm89 GAS and were identical at the nucleotide level apart from a pentanucleotide repeat (10 bp deletion) in the gene encoding streptococcal collagen-like protein B (SclB) of the pharyngeal isolate compared to the blood. However, the deletion is not predicted to alter SclB expression as both isolates maintain a frame-shift mutation in SclB and thus likely do not express the protein. We did not identify mutations in virulence gene regulators such as covRS known to lead to hypervirulence. Comparing the case isolates to recent GAS WGS performed by the Centers for Disease Control and Prevention (CDC) [] revealed a similar clonal relationship to nationally circulating emm89 iGAS (). The case of pharyngeal and blood isolates did not have a unique streptococcal pyrogenic exotoxin (spe) gene content compared to non-STSS iGAS in the CDC collection and was similar to emm89 STSS strains in Houston, TX ().
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pmc-6360555-1
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A 70-year-old woman affected by Waldenström's Macroglobulinemia, under immunosuppressive therapy with melphalan, was admitted to the Emergency Department of Policlinico Universitario A. Gemelli for severe infection of the facial skin in the periorbital region of left eye. The patient had a medical history of recurrent episodes of herpetic keratitis in the left eye associated with periocular vesicles and erythema due to HZ. Consequently, the patient underwent a deep anterior lamellar keratoplasty, on February 2014, and a penetrating keratoplasty, on June 2016. Since the first surgery, the patient had been under prophylactic antiviral therapy with acyclovir. Furthermore, on January 2010, the patient underwent a right dacryocystorhinostomy.
The patient presented to the emergency room having developed periocular blistering, swelling, pain in the same left dermatome of the trigeminal nerve interested in the previous HZ episodes, and also fever in the past 2 days. A diagnosis of shingles was made, and the patient was subsequently prescribed topical and intravenous acyclovir and then discharged.
After 24 hours, the patient represented with worsening of the clinical picture. There were tense periorbital oedema, pain, and erythema spreading to the surrounding areas. The patient was persistently febrile (T≥38.7°C), tachycardic (HR≥105 bpm), and hypotensive (BP≤100/60 mmHg) requiring fluid resuscitation and inotropic support.
A provisional diagnosis of HZ ophthalmicus with secondary bacterial periorbital cellulitis was made. Intravenous piperacillin-tazobactam, clindamycin, linezolid, and acyclovir were initiated.
Non-contrast-enhanced and Iopromide-enhanced cranial computed tomography was urgently performed, showing soft tissue swelling in left periorbital, frontal, temporal, and zygomatic region and at parietal level bilaterally, up to the vertex. The swelling continued caudally to the subcutaneous tissue of the left cheek, reaching the submental and neck region. No evidence of sinus involvement was found ().
Despite the adequate fluid administration and the antibiotic and antiviral therapy, in 2 hours the status of the patient evolved into severe hemodynamic instability (HR of 125 bpm, sinus rhythm, BP< 90/40 mmHg) with visible increase in the soft tissue oedema, persistent metabolic acidosis, high blood lactate levels, malaise, and confusion.
The clinical picture of the patient was consistent with the diagnosis of septic shock secondary to periorbital necrotizing fasciitis.
The patient was immediately transferred to the intensive care unit for cardiovascular monitoring. Orotracheal intubation was performed, high-dose adrenaline infusion started, piperacillin/tazobactam discontinued, and imipenem/cilastatin 1 g intravenously every 6 hours added.
The patient was then referred to the general surgery department and was taken for prompt debridement and fasciotomy for necrotising fasciitis. Two surgical incisions were performed at left frontotemporal and supraclavicular region proceeding with the fasciotomy of temporal and platysma muscle. At the time of the surgery, no purulent discharge was noticed at any levels. All tissue biopsies were reviewed by a consultant pathologist. The patient underwent further surgical debridement after 18 hours. Left upper eyelid showed substantial necrosis of the skin, pretarsal orbicularis muscle, orbital septa, and fat pads. The temporal muscle fascia was also involved by the necrosis and a purulent discharge from the subcutaneous soft tissue at the surgical incisions was observed. Diffuse induration and erythema persisted at left face, neck, and supraclavicular region. Drainage and debridement of the surgical sites were completed and a Negative Pressure Wound Therapy (NPWT) started (Figures and ). The supraclavicular wound was treated with NPWT for 3 days and then substituted by conventional dressings. The frontotemporal wound was treated with NPWT for 10 days (with wound dressing change every 48-72 hrs) and then conventional dressings.
Samples taken from the infected tissues showed group A haemolytic Streptococcus pyogenes infection with histopathological features suggestive of necrotising fasciitis, in keeping with the clinical picture.
On day 7 after surgery, the oedema and erythema of left frontotemporal and supraclavicular region and neck were healed up. Throughout her admission, she received regular ophthalmology review. Ocular bulb integrity and corneal graft remained preserved at all times; an eschar formed on the upper left lid with clear reduction of the periorbital swelling. She was prescribed a tetracycline unguent.
On day 13 after surgery, the patient was diagnosed with postsepsis critical illness myopathy and neuropathy [], confirmed by electromyography of the deltoid and biceps brachii muscles.
On day 28 after surgery, the limbs resulted in severe hypoperfusion and ischemia, thus into wet gangrene, due to the high-dose adrenaline therapy and, possibly, the underlying Waldenström's Macroglobulinemia, responsible for vasculitis and hyperviscosity syndrome. Contrast-enhanced MRI of the limbs showed a gangrene demarcation line more proximal than clinically expected. Necrosis and ischemic damage extended up to all the limbs muscles, predicting a dismal prognosis in the short term. Given the extent of gangrenous area, the only radical intervention seemed to be the hindquarter and forequarter amputation. However, the team of orthopaedic and general surgeons judged this demolitive procedure disproportioned and contraindicated it. The relatives were informed about the critical conditions of the patient. A bioethics consultant was called in to assess the case. In consideration of the irreversible evolution of the clinical picture, the consultant confirmed the unfavourable risk-benefits ratio of the aforementioned procedure.
After 31 days spent in the intensive care unit, the patient was assigned to palliative domiciliary care and died after a total of 61 days from surgery.
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pmc-6360559-1
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The patient was a 25-year-old male presenting with altered mental status and generalized tonic clonic seizures following a 2-week history of an upper respiratory tract infection. MRI of brain was negative for any acute pathology and nonlesional for seizures. Results of a paraneoplastic antibody panel were negative but he was found to have neutrophilic pleocytosis on lumbar puncture. Cerebrospinal fluid cultures were negative but he was empirically treated for bacterial and viral meningoencephalitis with ceftriaxone, vancomycin, and acyclovir. Seizures began increasing in frequency despite frequent treatment with benzodiazepines and, on day three, the patient worsened to have status epilepticus. Treatment with maximal doses of valproate, levetiracetam, and propofol was started but, by day four, the patient's seizures remained refractory. As a result, the patient was placed under a pentobarbital-induced coma with burst suppression pattern on electroencephalography (EEG). On day 8, five days after status and due to continued seizure activity as evidenced by EEG, a VNS was implanted. VNS was turned on with the settings: Output output current 1.5 milliamperes, Pulse Width 500 microseconds, Frequency 30, On time 30 seconds, and Time interval 3 minutes.
Magnet Output current 2 milliamperes, On time 60 seconds. Three days after implantation of VNS, there was generalized suppression of EEG activity with continued use of pentobarbital; however, the patient continued to have electrographic seizures which were successfully aborted by the VNS magnet swiping (see . No other changes were made to the medical regimen. For the next 72 hours, no status epilepticus or electrographic seizures were reported, although few occasional discharges were seen. Unfortunately, seizures recurred on day 14 and the patient succumbed to his multiple comorbidities on day 17. However, VNS was successful in acutely terminating status epilepticus for 72 hours in this critically ill patient when the standard therapies failed.
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pmc-6360570-1
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A 33-year-old primigravida, who was 38 weeks pregnant, presented with spontaneous severe left-sided epistaxis. Her first episode had started the previous week, with about seven-eight episodes a day. Her medical history was unremarkable. She had no personal or family history of bleeding tendencies, and she was not taking any regular medications. Her blood pressure was into normal ranges. She reported no previous episodes of epistaxis in her life. Routine blood tests were normal during the pregnancy.
We tried to control the bleeding first by administrating intravenous (IV) tranexamic acid, without resolution. So, we contacted the otolaryngologist, who performed an endoscopy, showing a left nasal floor bleeding varix. He decided for an anterior nasal packing: he inserted a tampon carefully along the floor of the left nostril, where it expanded on contact with blood. After the nasal tampon was been inserted, the otolaryngologist wetted it with a small amount of topical vasoconstrictor in order to hasten effectiveness. This procedure was repeated three times, inserting a total of six tampons (four in left nostril and two in the right one). Nevertheless, this conservative management of epistaxis failed. Within 4 hours of admission, patient haemoglobin had dropped from 12.5 to 7 mg/dl and she had a further bleed from the left nostril. The otolaryngologist did not consider a posterior nasal packing because the endoscopy showed an anterior bleeding site. A new endoscopy to locate the exact site of bleeding for direct cauterization was not indicated in acute setting due to vascular congestion and mucosal oedema. Patient clotting studies were within the normal range. A blood transfusion was required, using two packed red blood cells (PRBCs). The patient also started antibiotic therapy with IV Cephazolin 2 g every 8 hours. Cardiotocography (CTG), biophysical profile, and fetal Doppler demonstrated fetal well-being.
During her second day of admission, repeated blood tests showed that her haemoglobin remained persistently low at 7.5 g/dl, despite the recent blood transfusion. The patient became tachycardic (rate 157 bpm), tachypnoeic (22 breaths per minute), and asthenic.
After accurate counselling with the patient and considering the failure of conservative treatment, we thereby decided for a surgical management of pregnancy. The patient delivered a healthy baby girl weighing 3.9 kg. The execution of caesarean section was followed by an immediate resolution of the nasal bleeding.
We discharged the patient from the hospital with nasal packing, in order to ensure the formation of an adequate clot. Five days later, the otolaryngologist performed an endoscopy to locate the exact site of bleeding for direct cauterization. The patient experienced no other episodes of epistaxis.
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pmc-6360572-1
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A previously healthy 7-month-old African-American male presented to the ER in late winter after a generalized tonic-clonic seizure. There was no history of trauma or febrile illness. He was exclusively breastfed but had only received a multivitamin supplement for the first three months of life. Physical exam revealed a postictal infant with short stature, length = 64 cm (<5th percentile) and weight of 9.2 kg (75th percentile). Neurologic exam demonstrated brisk patellar reflexes, and the remainder of his physical exam was unremarkable. Initial labs showed severe hypocalcemia with total serum calcium = 5.9 mg/dl (normal range 8.5-10.9 mg/dl) and ionized calcium = .67 mmol/L (normal range 1.18-1.29 mmol/L). He was treated with IV calcium, after which serum calcium levels rose slightly but remained significantly below the normal range. Subsequent evaluation showed an undetectable 25-hydroxy vitamin D level of less than 4 ng/ml, with elevated parathyroid hormone and alkaline phosphatase levels (). Skeletal survey showed rachitic changes ().
The patient was diagnosed with nutritional rickets and was administered vitamin D stosstherapy treatment with ergocalciferol (Virtus Pharmaceuticals, ergocalciferol oral solution, USP, 8,000 international units per mL) 100,000 international units given orally every 2 hours for a total of 600,000 international units over 12 hours. By the last dose, the patient had become lethargic and urine output was decreased from 2.1 cc/kg/hr before treatment to .7 cc/kg/hr after treatment. Labs revealed acute renal failure, metabolic acidosis, and hyperkalemia. Creatinine level rose from .41 mg/dl pretreatment to 3.0 mg/dl following stosstherapy over a 36-hour timespan. He was treated with fluid resuscitation, diuretics, sodium polystyrene sulfonate, bicarbonate, and continued IV calcium. Renal function and calcium levels normalized, and serum creatinine returned to baseline of .41 mg/dl and total calcium = 9.8 mg/dl. The patient was discharged home after 4 days on oral vitamin D and calcium supplementation.
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pmc-6360578-1
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A 68-year-old Albanian man with history of chronic hepatitis B initially presented with progressive dysphagia to solids. Workup included a barium swallow showing a possible esophageal dysmotility problem and an abdominal ultrasound with no acute findings. The subsequent esophagogastroduodenoscopy demonstrated a nodule in the distal esophagus as well as a large, friable, and ulcerated mass along the gastric cardia extending into the gastroesophageal junction, both of which were biopsied. Pathology of the mass demonstrated adenocarcinoma, intestinal type, with neoplastic glands infiltrating the muscularis mucosae. Biopsy of the nodule showed high-grade dysplasia, and antral biopsies showed reactive mucosae. He was staged using positron emission tomography/computed tomography scans which confirmed the gastric mass and showed two fluorodeoxyglucose avid lymph nodes.
He underwent neoadjuvant chemotherapy with epirubicin, oxaliplatin, and capecitabine prior to total gastrectomy with esophagojejunal anastomosis. Following surgical resection, he was treated with paclitaxel, capecitabine, and pegfilgrastim kit.
One year later, he presented with a 3–6-week history of holocranial headaches and falls with dizziness, nausea, and photophobia. Magnetic resonance imaging of his head with and without contrast revealed three peripherally enhancing lesions with surrounding edema in his right cerebral hemisphere (Figures and ). The largest lesion was in the right temporoparietal lobes, resulting in mass effect with midline shift. He underwent right temporoparietal craniotomy for resection of the largest tumor. The tumor stained positive for cytokeratin 7 (CK7) (cytoplasmic) and home box protein CDX-2 (nuclear) and was negative for keratin 20 (CK20), thyroid transcription factor 1 (TTF-1), human epidermal growth factor receptor 2 (HER-2) (+1/+3), and glial fibrillary acidic protein (GFAP), consistent with a gastrointestinal origin. He subsequently underwent gamma knife radiosurgery for the resection cavity and remaining two metastases, which were enlarged on MRI postcraniotomy. He was offered systemic chemotherapy including capecitabine with temazolamide but he declined and agreed to close monitoring. The patient remains to be stable both clinically and radiologically, with no new brain metastases and no evidence of metastases at any other site as of July 2018.
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pmc-6360589-1
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A 37-year-old female presented with a several year history of dysphagia to solid foods. She denied any symptoms of reflux or abdominal pain. She had been taking dexlansoprazole with no improvement in her symptoms. She also reported increased intake of nonsteroidal anti-inflammatory drug (NSAID) medications for a month prior to presentation. On physical examination, she appeared well. Her abdominal exam was unremarkable
Esophagogastroduodenoscopy (EGD) was performed and showed abnormal esophageal mucosa with two esophageal webs. There was also gastritis with vague nodularity in the gastric body (). Esophageal biopsies showed no significant pathological abnormality. The stomach biopsies, taken from gastric body, showed mild chronic active gastritis with mild focal gastric atrophy and significant subepithelial collagen plate thickening. There were also entrapped inflammatory cells, red blood cells, and small capillaries compatible with CG (). The thickened collagen plate was further highlighted by trichrome stain (). The lamina propria was expanded, predominantly by plasma cells with admixed eosinophils and lymphocytes. The surface epithelium was atrophic with intraepithelial neutrophils. A Giemsa stain for Helicobacter pylori was negative and Congo red stain did not reveal any amyloid deposition. Serum protein electrophoresis did not show any evidence of a monoclonal protein and urine protein electrophoresis only showed minor albuminuria. Additional blood work, including celiac screen (anti-transglutaminase IgA: <1.0 U/ml, IgA: 2.36 g/L) and IgG4 titre (0.27 g/L), was all within normal limits. A complete blood count (CBC) performed one year prior to her presentation was normal (hemoglobin: 145 g/L, platelet: 216 × 109/L, WBC: 6.4 × 109/L). A diagnosis of CG was made. No new treatment was initiated and it was elected that she remains on the same dose of dexlansoprazole.
Subsequent upper and lower endoscopy were performed to rule out collagenous disease elsewhere. Colonoscopy was normal. EGD showed presence of friable tissue and multiple rings in the esophagus. There was mucosal cobblestoning and friable tissue in the gastric body. Biopsies of the duodenum, ileum, and colon were unremarkable. Biopsies of the gastric body showed minimal chronic inflammatory cell infiltrates and prominent mucosal lymphoid aggregates. Subsequent barium swallow demonstrated gastroesophageal junction outlet obstruction, for which the patient received esophageal dilation. Repeat EGD on follow-up 6 months later showed persistent esophageal webs and gastric mucosal cobblestoning with histological evidence of CG. In this instance, the gastric body mucosa had moderate chronic active gastritis with increased fibrosis beneath the surface epithelium and within the lamina propria, and the gastric antral mucosa showed a mild increase in fibrosis of the lamina propria. H. pylori was not detected in any of the biopsies taken.
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pmc-6360613-1
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A 39-year-old male was referred to The Fertility Clinic, Skive Regional Hospital, Denmark in 2013 along with his spouse due to primary infertility. They had attempted pregnancy for two years and throughout the treatment period the male delivered normal semen specimens according to 2010 World Health Organization (WHO) criteria [] (). Furthermore, gonadotropin and sex hormone levels were normal () [–]. A test for sperm DNA integrity (SDI-test) showed only a small proportion of damaged sperm cell DNA as DFI was 9.1% (normal range below 15%).
Regarding the clinical examination of the genitals, no abnormality was found. Both testes were of normal size (20 and 15 mL, respectively); the male had normal virilization and normal development of the penis, and ultrasound examination of the testes showed no abnormalities. Furthermore, the medical history of the male was normal with no recorded events affecting spermatogenesis and no familiar disposition to fertility disorders or other conditions. His height was 181 cm, weight was 71.9 kg, and BMI was 21.9 kg/cm2. In addition, an echocardiography showed a normally structured heart without coarctation of the aorta. A standard chromosome analysis based on 10 metaphases from cultivated peripheral lymphocytes in Q-band yielded a low-grade 45,X/46,XY mosaicism. Here, 1 out 10 metaphases contained a 45,X cell line while the remaining 9 contained 46,XY. This result was confirmed by a second karyotype, using fluorescence in situ hybridization (FISH) analysis, in peripheral lymphocytes screening 100 metaphase lymphocytes at 400–450 band resolution with specific probes for chromosome X. Out of 100 metaphases, 6 presented 45,X karyotype, while the remaining 94 presented regular 46,XY karyotype. In order to test for confined tissue mosaicism, FISH analysis, with probes for chromosomes 18, X and Y in mucosal cells from a buccal swab, was performed. The analysis of 162 interphase nuclei yielded one cell with only one X-signal (0.6%), thus showing no gonosomal mosaicism above the cutoff value of 3.0% [] (see discussion).
In addition, a test for sperm aneuploidy was performed using FISH with probes for chromosomes 13, 18, 21, X and Y. The frequency of gonosomal nullisomy was markedly elevated with 2.1% of the sperm containing neither a X nor a Y chromosome. Other values for this sample were within the normal range [, ].
The couple underwent several ART treatments from the inception of their referral to obtaining pregnancy with donor sperm intrauterine insemination (IUI-D). Initially, the couple underwent three IUI attempts using the sperm from the male. Thereafter, they underwent two in vitro fertilization (IVF) attempts and two attempts of combined IVF and intracytoplasmic sperm injection (ICSI). During these treatments, the spouse had a total of 45 metaphase II (MII) oocytes retrieved and 14 good quality embryos were transferred during fresh and frozen thawed transfers. None of the treatments resulted in neither biochemical nor ultrasound verified viable pregnancies. The couple decided to continue treatment using donor semen and subsequently obtained an ongoing pregnancy after their third IUI-D. A statement of consent was obtained from the patient.
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pmc-6360617-1
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Case reports are exempt from institutional review board approval at our institution.
A 75-year-old woman was evaluated for long standing right-sided nasal obstruction, dependent mouth breathing, clear rhinorrhea, congestion, and hyposmia not relieved by intranasal steroids or nasal irrigations. She initially presented with a history of previous endoscopic sinus surgery in Russia more than 20 years ago followed by nasal polyposis treated with ambulatory cauterization. She was also noted to have a history of nonmelanotic skin cancers of the nose treated with radiation in Russia.
Physical exam findings included a fleshy intranasal lesion that, in the setting of previous nasal skin cancer treated with radiation, raised a concern for possible secondary carcinoma. Computed tomography showed complete opacification of the right maxillary sinus, obstruction of the right ostiomeatal complex, and soft tissue density in the right nasal passage ().
Initial biopsy revealed a nasal mass that originated in the right inferior meatus. Pathology showed multiple polypoid fragments lined by a respiratory type epithelium with underlying edematous stroma with mild chronic inflammation. There was invagination of the surface epithelium into the underlying stroma resulting in nested aggregates of bland glandular and mucinous cells and focally benign squamous epithelium (). These features were found to be consistent with a benign inverted papilloma.
The patient presented with continued nasal obstruction and was evaluated for definitive treatment. Given her diagnosis of inverted papilloma and chronic rhinosinusitis, complete excision and revision endoscopic sinus surgery was recommended. During endoscopic sinus surgery, an exophytic mass with abnormal maxillary mucosa was seen emanating from the left inferior meatus that was thought to originate from the right maxillary sinus, given that it was protruding through a bony dehiscence into the inferior meatus and nasal cavity. Right partial inferior turbinectomy was performed, along with right extended maxillary antrostomy and stripping of maxillary mucosa to remove the entirety of the presumed inverted papilloma base within the maxillary sinus. Intraoperative and postoperative pathology again showed inverted papilloma.
Given her suspicious history, a second opinion was requested. This noted stromal hyalinization characterized by thickened, eosinophilic basement membrane-like material. The polypoid lesion had an inverted growth pattern concerning for inverted papilloma, but it had a prominent glandular lining (), leading to a revised diagnosis of REAH.
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pmc-6360626-1
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An 84-year-old man accidentally fell at home and was admitted to our hospital. The patient was hospitalized with several problems such as multiple metastases of prostate cancer, chronic heart failure, emphysema, impaired renal function, and biliary stent placement due to idiopathic biliary stenosis. While no fracture was identified, the patient complained of lower back pain and was unable to move. As a consequence, he was hospitalized for the purpose of pain management. On the third day of hospitalization, the patient developed a fever of 38.2°C, and his laboratory data showed high levels of WBC count and CRP. While the source of infection was not identified, a urinary tract infection was suspected because he had purulent urine from previous examination and no symptom of respiratory tract infection. The patient underwent treatment with CMZ 1 g every 12 hrs. Three days after therapy initiation, the fever declined and the laboratory data of the inflammatory response normalized. Although blood culture was negative, we decided to treat according to sepsis because he was frail. We, therefore, planned to administer CMZ for 14 days. During the treatment course, the patient did not develop fever and had a healthy appetite.
On the morning of the 14th day of hospitalization, the patient complained of a sudden difficulty in breathing. His peripheral artery oxygen saturation decreased to 74%. No fever, coughing, or sputum was identified. A chest computed tomography (CT) scan was performed, showing the presence of ground glass shadows bilaterally (). While the blood work demonstrated the absence of an inflammatory response, Hb decreased by 1.5 g/dL from the previous day. The BNP value was 103 pg/dL, similar to that at initial hospitalization. Because hemostasis of the blood sampling site was difficult, additional laboratory tests were performed. These showed a marked prolongation of PT-INR (). In the evening, his value of Hb dropped from 6.8 to 5.5 g/dl in six hours. We doubted gastrointestinal bleeding, but there was no black stool. Additionally, he began to spit bloody sputum. We considered bronchoscopy but could not carry out because of his poor respiration. Since the patient had an acute respiratory failure accompanied by blood sputum and progressive anemia without exacerbation of heart failure, he was diagnosed with pulmonary alveolar hemorrhage due to coagulation abnormality. Two units of red blood cell concentrates stored in mannitol adenine phosphate and six units of fresh frozen plasma were immediately administered to the patient.
We believe that the pulmonary alveolar hemorrhage was caused by disseminated intravascular coagulation (DIC) due to the prostate cancer. However, the patient did not meet the DIC's diagnostic criteria because each level was fibrinogen 393.8 mg/dL, fibrinogen degradation products (FDPs) 23.0 ug/dl, and platelet count 15.3 × 104/uL, even though PT-INR indicated extremely abnormal value (). We took into consideration the possibility of Vit K deficiency. To overcome this issue, we administered 10 mg menatetrenone per day. Three days later, all coagulation systems had recovered to their normal values (). Protein induced by vitamin K absence-II (PIVKAII) reached 8,884 mAU/mL (normal range below 40 mAU/mL) by the 23rd day of hospitalization.
Based on these observations, we investigated the cause behind the Vit K deficiency. The prescription drugs had not been changed before and after hospitalization, and antiplatelet and anticoagulant agents had not been used. The only additional drug used during hospitalization was CMZ. Furthermore, the patient's food intake remained unchanged in the course of the hospitalization as well as his hepatobiliary system's laboratory tests. No diarrhea developed during the patient's illness. Based on these observations, the patient was diagnosed with hypoprothrombinemia due to CMZ inhibition of Vit K epoxide reductase. Despite discontinuation of Vit K (menatetrenone) administration, the coagulation activity did not decrease. The pulmonary alveolar hemorrhage gradually improved, and the patient was discharged after one month.
One month after discharge, the patient was rehospitalized with pneumonia. He was treated with sulbactam/ampicillin 3 g quaque 12 hrs for 7 days, but hypoprothrombinemia was not observed.
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pmc-6360627-1
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A 19-year-old male patient complained of shoulder pain with no trauma history. He reported that the pain started about 6 months before, with progressive worsening. Pain was present in elevation and abduction, especially above 90°. The pain caused him to interrupt his physical activities, although he did not notice worsening during bodybuilding.
A winged scapula was identified in physical exam, with scapulothoracic grade III dyskinesia, according to Kibler et al. []. He presented infraspinatus atrophy, complete range of motion, preserved upper limb strength, and discrete paraesthesia at the region of the medial border of the right scapula, with no other signs. There were no clinical signs suggestive of rotator cuff injury or glenohumeral instability ().
The imaging exams did not show significant changes. Shoulder MRI showed no rotator cuff lesion, labral lesions, cysts, or other soft tissue involvement. The cervical spine MRI did not show cervical discopathy or syringomyelia. Electroneuromyography with evoked potential of the scapular girdle evidenced diffuse axonal involvement of the long thoracic nerve, without other alterations.
The patient was then referred to the physiotherapy service, where he initiated a program of shoulder girdle rehabilitation focused on analgesia and passive mobilization. During ten weeks, he remained under the care of physiotherapists twice a week, but he did not notice an improvement in the pain. When he returned to the orthopedic clinic, he was informed about the possibility of surgical treatment.
The patient insisted on conservative treatment. We recommended strengthening of the shoulder girdle and swimming. During three months, he practiced swimming three times a week under the guidance of a physical education professional with experience in athlete training. In order to strengthen the periscapular muscles, he tried to practice the four classic styles of swimming, using floats in the lower limbs and increasing the demand on the upper limbs. Progressively, he noticed an improvement in his pain.
The swimming program consists of a 60-minute pool training three times a week, with increasing distances. The front crawl, breaststroke, and backstroke were alternated during training. The main set was a target mile, split in 200 m lengths with 1-minute rest (in a 25 m pool) and alternating strokes.
He returned after 90 days, free of pain. He presented dynamic stabilization of the scapula during elevation, and dyskinesia was no longer perceived. The force remained unchanged, but atrophy was no longer identified. The patient was satisfied with the progress made and was encouraged to stay in the muscle strengthening program in aquatic activities ().
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pmc-6360657-1
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A 19-year-old man presented to our outpatient department with mild pain and swelling at the right shoulder for 3 weeks. There was no preceding history of trauma. The pain, mild in intensity, increased during shoulder movement. There was no history of fevers, night sweats and weight loss. And no previous history of tuberculosis or contact with persons suffering tuberculosis was recorded. The lesion started as a papule and ulcerated with copious purulent discharge for 10 days when the patient presented to our department. Dressing change and anti-infective drug (cephalosporin) were proved ineffective in other hospitals.
On physical examination, there was mild tenderness present over the anterior aspect of the right shoulder. A sinus was seen at the distal of the clavicle, sized 1.5 cm × 2.0 cm, 2.0 cm in-depth, with a purulent base, surrounding erythema and induration. There was a lot of canary yellow purulent discharge in the sinus. Full active range of motion was observed at the shoulder. There were no palpable lymph nodes and systemic examination did not reveal any abnormalities.
On X-ray Radiograph and Computed Tomography (CT) of the right shoulder, obvious lytic destructions were observed at the acromion process of scapula without periosteal reaction (Figs. and ). Radiological examinations (X-ray and CT) of the chest revealed no abnormal finding. Magnetic Resonance Imaging (MRI) of the right shoulder showed marrow edema in the acromion appearing hyperintense on T2 weighted and spectral adiabatic inversion recovery (SPAIR) sequences, and ill-defined area of mixture of hyperintense and hypointense in the acromioclavicular joint suggested an inflammatory process. MRI of the area showed destruction of the acromioclavicular joint, and it was considered to be suppurative arthritis or tuberculous arthritis (Fig. ).
Routine blood investigations were normal, except for a raised erythrocyte sedimentation rate (ESR) of 43 mm/h and C-reactive protein (CRP) of 39.1 mg/L. Human immunodeficiency virus (HIV) serological examination was negative. Canary yellow pus, found in the cavity, was sent for culture and Ziehl-Neelsen (ZN) stain to detect tubercle bacillus. However, no growth in culture and none acid fast bacilli (AFB) were seen. The sputum smear for AFB examined was negative, but detection of tuberculosis infection by T cell and purified protein derivative (PPD) skin test were positive, so presumptive therapy for tuberculosis disease was initiated: the patient was commenced on Rifampicin (RIF) and Isoniazid (INH) daily.
Debridement and vacuum sealing drainage (VSD) were applied on the right shoulder in one-stage operation. During the operation, a large amount of yellowish pus was found in the sinus, accompanied with caseous necrotic substances. The cavity in the sinus was through to the acromioclavicular joint, and part of the articular capsule was hyperemia and edema. Meanwhile, the acromion process of scapula was destroyed, and the bone debris could be found within the joint, but the lateral end of the clavicle remained intact. The bone debris was thoroughly debrided and the synovial membrane was excised during the operation. VSD was applied on the wound to continuous drainage after debridement, and the wound was irrigated with saline solution through the VSD tube after the operation. The necrotic tissues removed during the operation were sent to pathological examination, some specimens were sent for bacterial culture and ZN stain. The pathological examination showed granulomatous inflammation accompanied by caseous necrosis (Fig. ), but the culture of specimens was negative. Multidrug anti-tubercular therapy was applied after the operation: Rifampicin (RIF), Isoniazid (INH), Pyrazinamide (PZA), Ethambutol (EMB). The granulation tissue in the wound was fresh with little exudate after removing the VSD 1 week later. Then the wound was sutured directly after debridement. Two weeks following surgery, the wound healed well and the patient gained full range of movement of the shoulder. Before discharge, ESR was descended at 15 mm/h and CRP at 4.39 mg/L, which were normal. The patient continued treatment with four anti-tubercular drugs (RIF, INH, PZA and EMB) after discharge. At final 1 year follow up, the patient showed painless movement and normal range of motion, without evidence of disease recurrence.
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pmc-6360730-1
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In February 2007, a 30-year-old woman at 16 weeks’ gestation was referred to our department with a one-month history of tongue pain. The patient was an ex-smoker, but had no history of alcohol consumption. A hard, endophytic tumor was present in the midsection of the tongue on the right. The lesion measured 2.6 × 2.2 × 0.8 cm and extended to the floor of the mouth (cT2N0M0).
Neither the patient nor her family wished to continue this pregnancy, preferring to concentrate on treating the SCC. Two weeks after terminating the pregnancy, the patient underwent a supraomohyoid neck dissection and hemi-glossectomy with reconstruction using a free forearm flap. Her post-operative course was uneventful. Pathologic examination of the resected specimen confirmed a well-differentiated SCC with clear margins and no cervical lymph node metastases; it was classified as a pT2 N0 tumor. In the 11.5 years since undergoing surgical treatment for this tumor, the patient has remained healthy, with no recurrence.
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pmc-6360730-2
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In November 2010, a 33-year-old woman who was 8 weeks’ pregnant was referred to our department with a two-month history of tongue pain. The patient was an ex-smoker but had no history of alcohol consumption. Physical examination revealed a painful, ulcerated lesion measuring 2.3 × 1.6 × 4.1 cm on the right side of the tongue (cT2N0M0) (Fig. ). After consulting with an obstetrician, it was decided to avoid performing contrast-enhanced computed tomography (CT) and to use β-lactam antibiotics and acetaminophen in the patient’s perioperative management.
At 13 weeks’ gestation, the patient underwent a trans-oral partial glossectomy under general anesthesia. Fentanyl citrate, thiamylal sodium, and sevoflurane were used for anesthetic induction, and sevoflurane and oxygen in air were used for maintenance of anesthesia. Lidocaine 1% with adrenaline (epinephrine) 1:300,000 was used for local anesthesia. Cefazolin was used to prevent postoperative infection, and acetaminophen was used for analgesia. During surgery, the fetus was monitored by ultrasonic examination. The operation time was 1 h 7 min and the anesthesia time was 2 h 1 min. The patient’s postoperative course was uneventful. The tumor margin was positive, therefore, the patient underwent trans-oral wide excision of the lesion under local anesthesia at 17 weeks’ gestation. The margins of the resected specimen were clear. The child was delivered at term and developed normally. In the 7 years since the operation, the patient has remained free of the disease.
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pmc-6360730-3
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In March 2012, a 32-year-old woman developed SCC recurrence of the right side of her tongue. At that time, the lesion measured 0.7 × 0.4 cm (rT1N0M0). She was then 22 weeks’ pregnant. She was a non-smoker and had no history of alcohol consumption. In consultation with the doctor in charge of obstetrics and gynecology, it was decided that the following drugs be used in the patient’s perioperative management: cefazolin or cefcapene pivoxil hydrochloride (antibiotics) and flurbiprofen axetil and diclofenac sodium (analgesics).
At 25 weeks of pregnancy, a trans-oral partial glossectomy was performed under general anesthesia. Pathologic examination confirmed a well-differentiated SCC with clear margins. Remifentanil hydrochloride, thiamylal sodium, and sevoflurane were used for anesthetic induction, and remifentanil hydrochloride and oxygen in air were used for maintenance of general anesthesia. Lidocaine 1% with adrenaline (epinephrine) 1:300,000 was used for local anesthesia. Cefazolin and cefcapene pivoxil hydrochloride were used to prevent postoperative infection, and acetaminophen was used for analgesia. Ultrasonic examination was used to monitor the fetus intraoperatively. The operation time was 1 h 13 min and the anesthesia time was 2 h 22 min. The patient’s postoperative course was uneventful. Four months after the operation, she delivered a healthy baby. Six years after the final operation, the patient remains free of the disease.
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pmc-6360730-4
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In December 2011, a 38-year old woman was diagnosed with T2 N0 SCC of the left side of the tongue; the diagnosis was confirmed by pathologic examination of a biopsy specimen. Brachytherapy was delivered via a Cs needle, up to a dose of 70 Gy. In May 2012, the patient developed cervical lymph node metastasis and underwent a left modified radical neck dissection. Cervical lymph node metastases were found in two (out of 48) level II lymph nodes, with extracapsular spread. In December 2012, CT revealed multiple pulmonary metastases, as shown in Fig. . However, the patient was at this point five-weeks’ pregnant. She and her family wished to continue the pregnancy, and therefore rejected the option of chemotherapy. Radiotherapy was thus administered for the pulmonary metastases. The patient underwent a caesarean section at 29 weeks’ gestation. Thereafter, she received 6 cycles of chemotherapy with cisplatin and 5-fluorouracil. The SCC relapsed in the patient’s neck and lungs, and she died 10 months after undergoing chemoradiotherapy.
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pmc-6360731-1
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A 23-year-old man was flown in to our level I trauma center following an MVA collision about 2–3 h prior. En route, he became bradycardic and required intubation after losing consciousness. On presentation to our hospital, his vitals were as follows: heart rate 116-bpm; blood pressure 252/183-mmHg; respiratory rate of 19; and SpO2 of 98% on mechanical ventilation.
On examination, the patient was unconscious with dilated and unreactive eyes/pupils. He had several superficial abrasions and an open left femur fracture. There was a 9 × 7 cm contusion along the right chest wall, but chest was clear to auscultation without muffled heart sounds. Carotid, femoral and distal extremity pulses were all 1+ bilaterally without jugular venous distension. He also had mild abdominal distension, left flank ecchymosis, and absent rectal sphincter tone.
Focused assessment with sonography for trauma (FAST) only showed scant abdominal fluid and was negative for tamponade. Bilateral chest tubes were inserted due to suspicion of hemothorax, but produced minimal drainage. The patient deteriorated rapidly despite further resuscitative efforts, which were eventually discontinued at time of death.
Subsequent autopsy revealed an isolated tear in the right cardiac atrium at the junction of the inferior vena cava (IVC). Other findings included bilateral pulmonary contusions, blood in the pleural and peritoneal cavities, and small lacerations of the spleen and liver.
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pmc-6360734-1
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A 52-year-old male presented with paralysis of the left upper extremity; in another hospital 1 year before the current admission, the patient had received a computed tomography (CT) scan, which indicated cerebral infarction. A mass regarded as a myxoma that compressed left atrium was detected by transthoracic echocardiography (TTE), and this was considered to be the cause of cerebral infarction. Blood analysis showed severe thrombocytopenia, whereas erythrocyte and leucocyte counts were at a normal range. Gradually, he developed bilateral lower extremity oedema. For further diagnosis and treatment, the patient was admitted to our hospital. He had no significant past medical history.
His height was 165.0 cm, body weight was 58.1 kg, body temperature was 37 °C, pulse was 110 beats/min, blood pressure was 110/ 60 mmHg, and SpO2 was 100% (room air). Pulmonary sounds were clear with no crackles, but a III/IV systolic murmur could be heard at the junction between the left clavicle midline and the fifth intercostal space. Leg oedema was present. A chest X-ray demonstrated a cardiothoracic ratio of 60% with slight cardiac left dilation. Electrocardiography showed a sinus rhythm with a heart rate of 108 beats/min with slight ST-T segment changes. Abdominal ultrasound showed uniform congestive hepatomegaly with a normal sized spleen. Colour Doppler ruled out deep vein thrombus in the abdomen or lower limbs. A 50 × 35-mm solid mass severely adherent to the posterior part of the mitral valve was found by TTE, with systo-diastolic fluttering. The mass moved through the mitral orifice, which led to increased mitral inflow velocity but not a significant regurgitation. (Fig. a-b). Blood analysis revealed the following: leukocyte count of 4.3 × 109/L, haemoglobin (Hb) 13.2 g/dL, platelet (Plt) count of 20 × 109/L. Blood coagulation analysis revealed: Prothrombin time (14.5 s), Prothrombin activity (66%), Fibrinogen(91 mg/dL), Fibrin degradation products (30.5 μg/ml), and D-dimmer (1877 ng/ml). Blood film was performed and showed no abnormalities of platelets, leukocytes and erythrocytes. Bone marrow study revealed that the number of megakaryocytes increased; G-band and biopsy results had no abnormalities. Antinuclear antibody, Anti-ENA Antibody-Sm, Anti-ENA Antibody-RNP, Anti-ENA Antibody-SSA, Anti-ENA Antibody-SSB, Ro-52, Mitochondrial antibody IgG M2, Anti-myeloperoxidase antibody, Anti-protease 3 antibody, Anti-endothelial cell antibody and Anticardiolipin antibody were all negative. Anti-systemic lupus erythaematosus (SLE) antibodies and antiplatelet factor 4 (PF4) antibodies were also negative. Because severe thrombocytopenia was found at the same time as cerebral infarction, neither anticoagulants nor antiplatelet drugs were used during treatment. The patient received platelet transfusion, but platelet counts decreased quickly. Although operation risk was high, the tumour resection was performed through median sternal incision. Intraoperative transesophageal echocardiography (TEE) showed that the mass was adherent to the posterior mitral annulus, obstructing the mitral orifice, which caused a severe increase of pulmonary artery pressure. Intraoperative exploration revealed that the diameter of the pulmonary artery was widened, and the ratio of diameter of the aorta to the pulmonary artery was approximately 1:2. Cardiopulmonary bypass was initiated, with ascending aortic and bicaval cannulation. Following arrest with antegrade hypothermic crystalloid cardioplegia, the left atrium was revealed by blocking the superior and inferior vena cava and opening the right atrium and atrial septum. The tumour, which was rubbery to the touch, was divided into lobes with poly-papillary protrusions on the surface, and thrombus formation was observed between lobes. The pedicle was located in the area of P2 of the posterior leaflet, completely fused with the mitral annulus and lobes (Fig. a). Extensive resection of the tissue around the pedicle, including the annulus tissue caused mitral valve insufficiency, mitral valve replacement was performed. After cardiac resuscitation, TEE showed that the prosthetic mitral valve works regularly, and there was no residual tumor in the left atrium. The size of the tumour was approximately about 4x6cm, and the surface was lobulated, with white, sea anemone-like protrusions. Sallow fish-like tissue with cystic necrosis and haemorrhage could be seen when the tumour was cut open (Fig. b). Microscopically, the tumour consisted of two obviously different components, which are spindle or ovoid cells with significant marked atypia and epithelioid cells forming gland-like structures (Fig. a). Mitoses and focal necrosis are were present. Immunohistochemical staining showed positivity for CK, EMA, CD99, CK5/6 and CK7, focal positivity for calretinin and WT-1, and negativity for Desmin, S-100 protein, myogenin, SMA, CD31, CD34, D2–40, Sox-10, ERG, CDX-2, CK20, TTF-1, and HBME1. The Ki-67 index was approximately 10%. The result of double- colour fragmentation detection of SS18 gene probe was positive (Fig. b). These findings suggested that the tumour was a biphasic synovial sarcoma. The platelet count returned rapidly to normal early after tumour excision without other treatment (Fig. b). The results of blood coagulation analysis of the third day after surgery was significantly improved over preoperative results: prothrombin time (12.8 s), prothrombin activity (78%), fibrinogen (400 mg/dL), D-dimmer (835 ng/ml). Extubation was performed 10 h after surgery, and the patient was transferred to a general ward 2 days after surgery. The disappearance of the tumour from the annular region was confirmed on TTE 6 days after surgery, and an FDG-PET scan performed 8 days after surgery showed no abnormal accumulation. Our centre has no experience in radiotherapy and chemotherapy for cardiac synovial sarcoma. Then, we read the relevant literatures and consulted the oncologists about treatment and prognosis of synovial sarcoma. When the patient and family members were informed that even with chemotherapy and radiotherapy, the prognosis was poor, they finally decided to stop treatment. The patient was discharged when he was able to independently walk 10 days after surgery. Unfortunately, the patient died suddenly for unknown reasons 6 months later.
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pmc-6360744-1
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An 86-year-old female consulted in our hospital as a result of aggravated lower abdominal pain for 6 days, accompanying with nausea, vomiting and diarrhea. The patient had a past history of hypertension, which was treated with amlodipine besylate tablets. Abdominal examination revealed rebound tenderness at the McBurney’s point, accompanying with normal bowel sounds. Laboratory examination of the patient on admission suggested white blood cell (WBC) count of 20.31*10^9/L and neutrophilic granulocyte percentage (N%) of 94.3%. Abdominal computed tomography (CT) revealed gas in intra- and extra-hepatic portal and mesenteric veins in addition to appendicitis with peripheral peritonitis (Fig. ). Thus, an emergency laparoscopic appendectomy was planned, which was rejected by this patient. Therefore, conservative treatment with antibiotics (Piperacillin Sodium and Tazobactam Sodium for Injection) was applied. The abdominal pain was generalized for the following 4 days; moreover, the signs of diffused peritonitis and borborygmus had gradually disappeared. Nonetheless, no decreases could be seen in inflammatory markers after conservative treatment with antibiotics (Fig. ). Subsequently, repeated abdominal CT scan was conducted, which revealed the absence of gas in intra- and extra-hepatic portal and superior mesenteric veins. However, abdominal CT scan revealed enhanced pneumatosis cystoides intestinalis and dilated small intestine, which had become more and more severe. In addition, the appendicitis with peripheral peritonitis had been always present (Figs. and ).
Intestinal necrosis was suspected based on the abdominal signs, as well as changes of laboratory and frequent imaging examination results. An emergency laparotomy was performed on the fifth day of admission. Intra-operative findings had confirmed the diagnosis of suppurated appendix, with about 20 ml purulent secretion around it. Remarkable necrosis, which was about 100 cm, could be observed in the small intestinal wall and mesentery. Besides, an intestinal perforation could be seen in the necrotic bowel. The intestinal perforation was covered by the omentum majus. No obvious intestinal content overflow was seen. The patient had received appendectomy and part of the small intestine was removed. Afterwards, the patient’s postoperative pathology had confirmed the diagnosis of acute gangrenous appendicitis accompanying with periappendicitis and ileum necrosis (Fig. ). Based on the use of antibiotics (Imipenem and Cilastatin Sodium for Injection), the inflammatory markers decreased significantly after surgery (Fig. ). In addition, abdominal CT was performed on the fifth day after the operation, showing that the intra-abdominal condition was improved (Fig. ). Her symptoms were resolved and she was discharged 11 days postoperatively.
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pmc-6360910-1
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The case is of a 35-year-old right-hand-dominant man who worked in an office. He was a nonsmoker and had no significant medical comorbidities. He was referred with a lump on his left index finger several months following a simple laceration to the area from broken glass. The lump had been progressively enlarging, associated with tenderness and reduced finger flexion secondary to its size. The radiograph () showed soft-tissue prominence with a mostly radiolucent lesion with peripheral calcification volar to the base of the middle phalanx. On ultrasound scan, a relatively well-circumscribed mass was demonstrated with peripheral calcification just lateral to the flexor tendons measuring 14 × 7 × 13 mm. He subsequently underwent exploration in theater, where an encapsulated calcium-filled mass adherent to the middle phalanx and the A3 pulley was encountered. Histology revealed a benign cartilaginous proliferation in keeping with a soft-tissue chondroma. A diagnosis of Nora's lesion was considered but dismissed as the lesion lacked the typical zonal pattern consisting of central or basal new bone and a peripheral cap of cartilage.
At follow-up 6 months later, a recurrent mass was noted. The radiograph () showed recurrence of a calcified mass that appeared separate from the underlying cortex. He subsequently underwent reexploration and removal of a suspected recurrent enchondroma. Histology on this occasion was again consistent with a soft-tissue chondroma.
He then presented with a second recurrence approximately 1 year later. On this occasion, magnetic resonance imaging (MRI) was performed in an attempt to delineate the lesion. The MRI revealed a nonspecific appearance of the lesion () but favored it to be a periosteal chondroma. Clinically, there was a mildly tender lump at the previous operative site with restriction in proximal interphalangeal joint (PIPJ) flexion. Repeat radiography () showed a more well-defined calcified lesion along the volar and radial border of the PIPJ, the larger focus distally measuring up to 8 mm. He underwent reexploration and marginal excision of the bony lesion. Intraoperative findings showed a bony mass arising from the proximal radial-volar base of the middle phalanx (). Histology at this time reported the lesion to be Nora's lesion progressing to acquired exostosis. No clinical recurrence has been seen in the subsequent 6 months.
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pmc-6361077-1
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A 73-year-old woman was admitted to our hospital for close examination of gradually decreasing renal function. She was first referred to our hospital with obstructive jaundice due to a pancreatic head mass 6 years earlier (Fig. ). CE-CT showed focal enlargement of the pancreas. Endoscopic retrograde pancreatography showed irregular narrowing of the main pancreatic duct. After closer examinations, type 1 autoimmune pancreatitis (AIP) was highly suspected because she had an elevated serum IgG4 level (378 mg/dL), which exceeded by more than twofold the upper limit of the normal range. She was treated with prednisolone (PSL) 30 mg/day, after which her symptoms promptly improved with serum IgG4 level decreased (165 mg/dL). Finally, a definite diagnosis of type 1 AIP was made based on the Clinical Diagnostic Criteria for Autoimmune Pancreatitis 2011 (level 1 serology and diagnostic steroid trial) []. The PSL dose was gradually tapered to 5 mg/day. Before starting treatment, no other characteristic lesions of IgG4-RD commonly found in the kidney or lacrimal and salivary gland were present (Fig. a, c). She had a history of hypertension, hyperlipidemia, paroxysmal atrial fibrillation, and old cerebral infarction. Her renal function had remained normal (serum creatinine 0.7 mg/dL) until 1 year before the current admission without any imaging abnormalities in the kidney. However, during this past year her renal function gradually declined (serum creatinine 1.1 mg/dL) and follow-up CT revealed right dominant renal atrophy (Fig. ). On admission, she was afebrile and her consciousness was clear. On physical examination, blood pressure was 99/54 mmHg and pulse 67 beats per minute. There were no remarkable findings except for slightly swollen bilateral lacrimal glands. Pitting edema on the lower extremities was seen. Laboratory findings (Table ) included CRP 0.1 mg/dL, creatinine (Cr) 0.88 mg/dL (eGFR 48.1 mL/min/1.73 m2), BUN 17 mg/dL, IgG 1261 mg/dL, IgG4 201 mg/dL (IgG4/IgG: 16%), IgA 276 mg/dL, IgM 160 mg/dL, IgE 1025 IU/mL, C3 108 mg/dL, C4 23 mg/dL, and CH50 60 U/mL. Rheumatoid factor, anti-nuclear antibodies, anti-SSA antibodies, anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA), and anti-proteinase 3 ANCA were all negative. Neither proteinuria nor hematuria was present. The level of urinary N-acetyl-β-d-glucosaminidase (NAG) was 1.9 IU/L and that of urinary β2-microglobulin (β2-MG) was < 75 µg/L. Ga-scintigraphy showed no uptake on kidneys, pancreas, salivary glands, or lymph node. CE-CT on admission demonstrated multiple low-density lesions in the bilateral kidneys that led us consider the possibility of IgG4-RKD. Renal atrophy was seen predominantly in the right kidney (Fig. b, d). Renal Technetium-99m diethylene triamine pentaacetic acid (Tc-99m DTPA) scintigraphy revealed marked right renal dysfunction (GFR: left 40.6 mL/min, right 10.6 mL/min; Fig. ).
We decided not to perform a renal biopsy because of the right renal atrophy and malformation of the left renal vein in the inferior pole of the left kidney. We performed renal artery ultrasound. Right and left peak systolic velocity (PSV) was 92 and 64 cm/s, respectively, and renal aortic ratio (RAR) was 1.0 and 0.7, respectively. These data supported the absence of renal artery stenosis. d-Dimer levels were not elevated (0.9 µg/mL: normal < 1.0) on admission. Moreover, d-dimer levels were constantly within the normal range throughout the clinical course. Therefore, thrombotic event of the right kidney was also ruled out.
Based on the results of extensive examinations such as chest X-ray, echocardiograph, ultrasound, and gallium scintigraphy, we differentiated IgG4-RKD from other vascular diseases such as renal arterial stenosis, thromboembolism, and aneurysm. Finally, a diagnosis of IgG4-RKD probably due to TIN was made, and we increased the dose of prednisolone to 30 mg/day. 1 month after increasing the dose of corticosteroid, the left kidney lesions showing multiple low-density lesions and mild partial atrophy demonstrated almost no change (Fig. ).
3 months after increasing the dose of corticosteroid, the patient was treated with a maintenance dose of 14 mg/day of prednisolone, and renal function was stable (Fig. ).
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pmc-6361084-1
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Microscopic hematuria and proteinuria were detected by school urinary screening when she was 10 years old. Edema and hypertension were not noted. Hypocomplementemia was detected and the serum levels of complement hemolytic activity (CH50), C3 and C4 were 24 U/mL (normal range 28–48 U/mL), 21 mg/dL (normal range 64–166 mg/dL) and 19 mg/dL (normal range 15–38 mg/dL), respectively. Microscopic hematuria, proteinuria and hypocomplementemia continued and she developed nephrotic syndrome (serum albumin level 2.5 g/dL and urinary protein excretion 3.4 g/day) with normal blood pressure and renal function. The first renal biopsy was performed at 11 years of age and 30 glomeruli were obtained. Increase of mesangial cells and matrix with a lobular pattern of glomeruli and thick glomerular capillary walls with double contours were observed on light microscopic examination (Fig. ). Subendothelial and mesangial deposits were observed on masson trichrome stain. There was no tubulointerstitial change. Lumpy C3 deposits along glomerular capillaries were demonstrated by immune-enzyme method (PAP method). There was weak staining of IgA, IgG and IgM along glomerular capillaries. Glomeruli were not included in the specimen for electron microscopic studies. There were no clinical signs or symptoms of systemic lupus erythematosus, thrombotic microangiopathy and malignancies. Anti-DNA antibody, hepatitis B virus antigen, hepatitis C virus antibody and cryoglobulins were negative. MPGN type I was diagnosed. Prednisolone (PSL) was started with 60 mg (2 mg/kg/day) for 4 weeks and gradually reduced to 15 mg on alternate days over 1 year period. Urinary protein excretion decreased to 2+ by dipstick and hypoalbuminemia and hypercholesterolemia improved after the treatment with PSL and dipyridamole []. Microscopic hematuria, proteinuria and hypocomplementemia continued at 14 years of age and the follow-up renal biopsy was performed; 18 glomeruli were obtained (Fig. ). Mesangial proliferation and capillary wall thickness were decreased. Lobulation of glomeruli was not noted. Fibro-cellular crescents in 3 glomeruli and a sclerosed glomerulus were noted. There was no tubulointerstitial change. Immunofluorescent micrograph showed dominant C3 deposition along the capillary walls, weak IgG staining and traces of IgA and IgM in the mesangium and along the capillary walls. Findings on electron microscopy were not available. Pathological findings were thought to be improved and PSL was reduced to 10 mg on alternate days. At 16 years of age proteinuria increased to 3.4 g/day and she became nephrotic again. Serum creatinine level was normal (0.45 mg/dL). Intravenous methylprednisolone 500 mg per day for 3 days was administrated followed by oral PSL 10 mg on alternate days. Angiotensin-converting enzyme inhibitor for renoprotection was added. Hypocomplementemia improved (C3 > 64 mg/dL) at 19 years of age. At 25 years microscopic hematuria disappeared and hypoalbuminemia improved (serum albumin level > 2.5 g/dL). Serum creatinine was 0.82 mg/dL, estimated glomerular filtration rate (eGFR) was 71.6 mL/min/1.73 m2, and proteinuria was 3.7 g/day. During 26 and 32 years, her serum creatinine was between 0.85 and 1.06 mg/dL, eGFR was between 51.7 and 67.2 mL/min/1.73 m2, and morning spot urine protein/creatinine ratio was between 0.2 and 0.7 g/gCr. Blood pressure was normal and treatment with PSL 10 mg on alternate days was continued.
She wished to have a child even after the risks of maternal and neonatal complications associated with pregnancy, including preeclampsia, CKD development and preterm delivery, were explained to the patient and her husband. She had two gestations at 32 and 33 years of age and both resulted in spontaneous abortion at 9 weeks of gestation. She received ovulation induction with human menopausal gonadotropin and human chorionic gonadotropin at another Obstetrics and Gynecology Clinic and had quintuplet gestation. At 10 weeks of gestation transvaginal selective embryo reduction procedure was performed and five embryos were reduced to twins. After the procedure, she was referred to the Department of Obstetrics and Gynecology of Omori Hospital. Her body height, weight and body mass index before pregnancy were 154 cm, 55 kg and 23.2 kg/m2, respectively. After angiotensin-converting enzyme was discontinued at each gestation, her blood pressure was normal. At 20 weeks of the third gestation, hypertension occurred and administration of methyldopa was started. From 24 weeks she was admitted to the maternal-fetal-intensive-care-unit and anti-hypertensive drug was changed to nifedipine. Hypertension continued. Urinary protein excretion increased to 6.7 g/day and nephrotic syndrome developed. Serum creatinine level increased to 1.29 mg/dL and eGFR decreased to 39.1 mL/min/1.73 m2. Elective cesarean section was performed at 28 weeks of gestation. Twin babies were born. Male baby was 1235 g of weight, 34.5 cm of height and 29.4 cm of head circumstance at birth and was appropriate for date. His Apgar score was 6 at 5 min after birth. Female baby was 883 g of weight (below the 10th percentile for the Japanese population), 33.2 cm of height (above the 10th percentile) and 24.8 cm of head circumstance (above the 10th percentile) at birth. The asymmetrical type intrauterine growth restriction due to placental insufficiency and superimposed preeclampsia was diagnosed. Her Apgar score was 5 at 5 min after birth. The twins were dichorionic diamniotic and discordant. Pathology of placenta showed infarct in some focal regions without signs of chorioamnionitis. Both babies were treated with surfactant administration and mechanical ventilation for respiratory distress syndrome. By the neonatal screening tests, the female baby was diagnosed as having hypothyroidism at 1 month of age (free triiodothyronine 1.91 pg/mL; normal range 2.26–4.15 pg/mL, free thyroxine 0.52 ng/dL; normal range 1.01–1.67 ng/dL, thyroid stimulating hormone 150 µIU/mL; normal range 0.32–4.12 µIU/mL) and was treated with levothyroxine. Urinary iodine concentration of the baby was high (760 µg/L; normal range 121–271 µg/L). The mother had undergone hysterosalpingography after a second abortion at 34 years of age. Excessive maternal iodine intake due to iodinated contrast medium administration may cause hypothyroidism of the baby []. At 3 months of age, she received laser photocoagulation for retinopathy due to prematurity. Any other congenital anomaly was not noted in both babies. They were discharged from neonatal-intensive-care-unit on day 74 and 114, respectively. A month after delivery, blood pressure, serum creatinine, eGFR and serum albumin of the mother returned to baseline levels. Nifedipine was changed to angiotensin-converting enzyme. Six months later, proteinuria decreased to 2+ (1.1 g/gCr). Clinical findings of the patient are shown in Table .
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pmc-6361191-1
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A 34-year-old woman presented to a physician with chief complaints of abdominal pain and fever. The physician suspected acute exacerbation of chronic pancreatitis and referred the patient to the Department of Gastroenterology, Fukushima Medical University Hospital. The patient had a medical history of duodenal atresia treated by emergent surgery 2 days after birth, and annular pancreas and malrotation of the intestine, for which duodenoduodenal anastomosis with Ladd’s procedure was performed. Operative findings revealed no dilatation of the common bile duct. At 9, 23, and 25 years of age, she suffered from repetitive acute pancreatitis and required hospital treatments. Since she had recurrent epigastralgia and back pain, she was diagnosed as having chronic pancreatitis and was prescribed with oral drugs. She was a non-smoker and reported occasional alcohol consumption with no relevant family history.
Laboratory data from blood samples taken at the patient’s first visit to our department exhibited slight elevation of hepatic and biliary tract enzymes (glutamic oxaloacetic transaminase 53 IU/L, glutamic pyruvic transaminase 94 IU/L, alkaline phosphatase 446 IU/L, gamma-glutamyl transpeptidase 259 IU/L). Abdominal computed tomography (CT) showed a small round stone, approximately 9.3 mm in diameter, in the common bile duct, and a pancreatic calculus, approximately 14 mm in diameter, in the pancreatic head duct (Fig. ), causing slight dilatation of the distal pancreatic duct. Abdominal ultrasonography showed no dilatation of the intrahepatic bile duct and no thickness of the gallbladder wall. Magnetic resonance cholangiopancreatography (MRCP) revealed annular pancreas around the second portion of the duodenum (Fig. ). Endoscopic retrograde cholangiography (ERCP) was performed to determine the cause of the pancreatitis. ERCP showed a round filling defect caused by the above-mentioned stone and pancreatic calculus (Fig. a). The distal portion of the common bile duct was bended due to the previous surgical procedures for annular pancreas in the neonatal period. Pancreaticobiliary maljunction was suspected because the contrast medium refluxed to the pancreatic duct during cholangiography (Fig. a). The common channel could not be measured exactly, because of the bended distal portion of the common bile duct. The amylase level in bile juice obtained during ERCP was elevated to 2513 U/L. The 3D-cholangiography reconstructed from the CT data (Fig. b) revealed that the pancreatic duct joined the common bile duct at the bend. Preoperative endoscopic ultrasonography was not performed in the present case. As endoscopic sphincterotomy was difficult to perform because of the deformed duodenum due to the previous surgery, only a balloon dilatation of Vater’s papilla was performed. Furthermore, removal of the common bile duct stone could not succeed. Then, we decided to perform surgical treatment. Although intraoperative cholangiography via the cystic duct showed a filling defect in the common bile duct, cholangioscopy via a small incision on the common bile duct showed no stones. Thus, we concluded that that the stone had passed spontaneously. On the basis of the preoperative findings, high levels of amylase in the gallbladder (22,300 U/L), and the presence of a relatively long common channel detected by cholangiography, our patient was diagnosed as having a pancreaticobiliary maljunction without bile duct dilatation. We performed a cholecystectomy in the laparotomy and placed a C-tube in the common bile duct. In histopathological findings, reactive lymph follicle and lymphocytic infiltration were observed around Rokitansky-Aschoff sinus in the gallbladder with no neoplastic lesion in the mucosa.
The postoperative course was uneventful. The bile juice drained from the C-tube showed elevated levels of amylase (32,572 U/L). On postoperative day 13, the C-tube was removed, and the patient was discharged on postoperative day 17. At time of writing, 17 months after surgery, there has been no relapse of pancreatitis. However, medical treatment has been maintained for chronic pancreatitis in this patient.
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pmc-6361750-1
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A 56-year-old man was admitted to a local hospital for transient (10 min) left limb numbness when he rested on the sofa. Magnetic resonance angiography revealed that the right ICA was occluded from its origin to the intracranial segment. The patient reported taking aspirin, clopidogrel and atorvastatin, and was referred to the authors' center 2 weeks later. Physical examination revealed no obvious positive findings. Although a 2-month history of diabetes was recorded, there was no history of hypertension, hyperlipidemia, or smoking.
The initial CDFI was performed on day 1 of hospitalization, which revealed a patent right ICA with normal blood flow velocity (). However, CTA on day 2 indicated that the right ICA was occluded (). A repeat CDFI scan on day 4 of hospitalization revealed a hypoechoic mass [thickness 2.8 mm (suspected thrombus)] was attached to the anterior wall of the initial segment of the right ICA (). Magnetic resonance imaging (MRI) was performed on day 6; T1-weighted imaging showed that the intracranial segment of the right ICA was invisible, indicating that the right ICA was occluded (). However, digital subtraction angiography (DSA) performed on the same day revealed that the right ICA was normal, with no significant stenosis in any segment ().
CDFI findings were consistent with those of DSA, but nevertheless inconsistent with CTA and MRI. It was not advisable for clinicians to make a definitive diagnosis pertaining to the vascular lesions of the patient. Given the generally good condition of the patient and no significant discomfort, he continued taking aspirin 100 mg/day, clopidogrel 75 mg/day, and atorvastatin 20 mg/day after discharge. Follow-up CDFI 1 and 3 months after discharge did not detect the hypoechoic mass that was attached to the anterior wall of the initial segment of the right ICA, and complete patency and normal blood flow velocity were apparently restored.
Five months later, however, the patient was re-admitted to the authors' hospital again due to transient (~10 min) left limb weakness without obvious cause. Repeat CTA revealed severe stenosis of the right ICA, and the sagittal image revealed the right styloid process compressing the right ICA horizontally at the C2-3 intervertebral disc (). Subsequent 3D computed tomography reconstruction of the cervical spine revealed a bilateral overgrown styloid processes, which was ~6.3 cm in length on the right side and 6.1 cm on the left side ().
Subsequently, CDFI depicted a long hyperechoic bony structure on the right side of the neck (), located between the base of skull and the ICA compressing the ipsilateral ICA causing visible artery stenosis (). Blood flow velocity in the ICA dramatically increased when the patient slowly turned his head to the right (), which was significantly altered compared with the normal position ().
TCD was then used to monitor hemodynamic changes of the bilateral middle cerebral artery (MCA) in real time. Even when the patient was in a normal position, blood flow velocity in the right MCA was lower than in the left, but, nevertheless, remained in the normal range (). When the patient turned his head to the right, blood flow velocity in right MCA was significantly decreased (), but increased significantly when he turned his head back to the starting position (). Blood flow velocity in the left MCA did not change markedly during these maneuvers (). When the patient turned his head slowly and continuously, blood flow velocity in the right MCA changed dramatically, with no significant change on the left side ().
The patient underwent right styloidectomy. During the operation, the elongated styloid process could be visualized on the front of the right ICA. The middle and lower part of the overgrown styloid process was removed; the length of the specimen was approximately 4.2 cm (). Postoperatively, the right styloid process stump was detected and patency in the right ICA was restored (). TCD was repeated to monitor bilateral MCA in real time; there was no significant change on either side when the patient's head was rotated.
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pmc-6362309-1
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This 34 year old left-handed man was referred to our service 10 years ago following an episode of intermittent disorientation, altered awareness, patchy global amnesia, transient slurred speech, and tremulousness lasting <24 h, attributed to likely focal seizures with impaired awareness. He had experienced intermittent, stereotyped left monocular visual blurring with definite left monocular vertical oscillopsia over the preceding 5 years; episodes occurred 1–3 times/month, lasted 20–30 min and resolved over 10 min with eye closure, followed by severe left temporal aching headaches and left monocular photophobia within 5 s of initial symptom onset which then lasted for the same duration as the visual symptoms. There were no other features of migraine or trigeminal autonomic cephalgia. He also experienced independent, identical, left monocular blurring without oscillopsia or headache on exposure to sunlight, resolving within 5 s when he stopped looking at the light and closed his eyes, consistent with left monocular TIAs due to low-flow retinopathy. He initially had untreated hypertension for 2 years, a 4 pack-year smoking history and consumed 35 units of alcohol/week, with no other relevant history. Paternal grandfather died suddenly at age 41 years (unknown cause). Mother has Parkinson disease since age 55. His father, who died from suspected oropharyngeal cancer, was an obligate carrier of the AGS mutation by his position in the pedigree (see below). A paternal aunt has AGS and required pacemaker insertion for “cardiac reasons”; another paternal aunt had a stroke at age 40, but “AGS status” has not been clarified. One brother has genetically-confirmed AGS without apparent clinical manifestations. Three paternal first cousins have confirmed AGS with clinically supportive findings.
General examination revealed hypertension (168/98 mmHg), livedo reticularis, a slightly pointed chin, aortic regurgitation, and diffuse abdominal tenderness without guarding, rigidity or audible abdominal bruits. Cognitive testing revealed decreased verbal fluency (seven “F” words in 1 min), but was otherwise normal. Neurological examination of the cranial nerves, fundi, and limbs was normal.
Fasting total cholesterol was 4.2 mmol/L, with LDL cholesterol 2.7 mmol/L and normal triglycerides before commencing statins. Otherwise, routine hematological and biochemical screening was normal. ANA, ANCA, ENA, rheumatoid factor, anti-cardiolipin and anti-beta 2 glycoprotein I antibodies were negative. Lupus anticoagulant was persistently positive consistent with primary anti-phospholipid syndrome. Electrocardiogram revealed left ventricular hypertrophy. Transthoracic echocardiogram revealed a bicuspid aortic valve, moderate aortic incompetence and symmetrical left ventricular hypertrophy. EEG did not show any epileptiform changes. Color Doppler ultrasound demonstrated low flow in a narrowed left internal carotid artery (LICA), with a LICA peak systolic velocity of 25 cm/sec, but was otherwise normal. Serial brain MRIs revealed normal brain parenchyma over a 10-year period, but features suspicious of moyamoya phenomenon.
Axial T1 fat-saturated MRI neck vessels revealed a diffusely thickened wall of the left CCA (not shown). Extracranial MRA (not shown) and CTA () revealed a diffusely-narrowed left ICA. Formal cerebral angiography confirmed severe left ICA narrowing suspicious of either prior left ICA dissection or vasculopathy (), and confirmed moyamoya phenomenon in the anterior and posterior circulations (). The proximal right middle cerebral artery also showed features of moyamoya phenomenon (not shown). Pre- and post-acetazolamide SPECT imaging revealed exhausted haemodynamic reserve in the left ICA territory.
Molecular genetic analysis revealed heterozygosity for a missense G>C mutation in exon 4 of the JAG1 gene on chromosome 20 resulting in substitution of proline for alanine at codon 211 (p.Ala211Pro). Pathogenicity was confirmed by familial segregation studies.
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pmc-6362377-1
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A previously healthy 6-months-old female infant was referred to the Pediatric Intensive Care Unit of the Clinics Hospital of University of Campinas (CH-UNICAMP) because of a coma. Fifteen days prior to the coma, the patient had presented respiratory symptoms and had received antipyretics and short-acting B2-agonists. Four days prior to the coma, the patient presented respiratory distress and a fever, and was admitted to a secondary hospital in the metropolitan region of Campinas, São Paulo, Brazil. On the first day of hospitalization, the patient had a persistent fever and sinus tachycardia. A chest X-ray revealed an increased cardiac area with no radiographic changes in the lungs (). The following day, an echocardiography revealed pericardial thickening with bulky effusion and signs of cardiac tamponade. The patient was reported to be in good general condition. On the third day of hospitalization, the patient suffered from seizures. Diazepam, phenobarbital, and phenytoin were administered, and an urgent transfer to the intensive care unit was requested. During transport, the patient was intubated and was administered dobutamine. Upon arrival to the CH-UNICAMP, the patient went into hypotensive shock, with a Glasgow coma score of 3.
After hemodynamic stabilization, a computed tomography (CT), a lumbar puncture, radiography, blood cultures, and laboratory tests were performed. The initial hemogram was: Hb 6.1 g/dL; Ht 19.8%; red blood cell count 3,320,000/mm3; white blood cell count 4290/mm3 (15.3% neutrophils; 12.2% band cells; 70.4% lymphocytes; 2.1% monocytes); platelet count 166000/mm3. A cerebrospinal fluid (CSF) analysis revealed: Proteins 1291 mg/dL; Blood cells 730/mm3; Glucose 0 mg/dL; Leukocytes 51 cells/mm3 (86% neutrophils). Numerous gram-positive cocci were visualized in the CSF bacterioscopy. Ceftriaxone was administered to the patient at a dose of 100 mg/kg. For hemodynamic stabilization, epinephrine was required at an infusion rate of 0.3 mcg/kg/ min. The CT revealed diffuse cerebral edema and chest radiography showed a discrete peribronchial heterogeneous opacity in the right hemithorax and cardiomegaly. A bedside ultrasound showed a septate pericardial effusion () without pulmonary consolidation. The neurological examination showed that the patient was brain dead, and a pericardiocentesis was not performed. Streptococcus pneumoniae was identified in the blood and CSF cultures. The bacteria were susceptible to the following antibiotics: sulfamethoxazole-trimethoprim; benzylpenicillin; erythromycin; ceftriaxone; vancomycin; and levofloxacin. Brain death was confirmed according to protocol.
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pmc-6362419-1
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A 67-year-old caucasian male patient presented first to the Clinic for Dermatology in August 2017 suffering since March 2017 from sore throat, intraoral bullae, odynophagia, dysphonia, exertional dyspnea, and erosions of the glans penis. He was first treated by his general practitioner for a suspected oral herpes infection with antiviral medication without improvement. At the onset of the symptoms the patient had been retired.
The medical history of the patient revealed a history of prostate cancer diagnosed and treated by radical prostatectomy ~1 year before the onset of symptoms, epilepsy treated with levetiracetam since 2002, asthma and a chronic rhinosinusitis since 1988 treated with surgery.
The clinical examination revealed dry mucuous membranes in the oral cavity with erosions and swellings of the buccal mucosa and the hard palate. Inspection of the pharynx showed a distinct laryngo-pharyngitis with involvement of the epiglottis. To exclude an involvement of trachea a bronchoscopy was done revealing multiple ulcers of the pharynx, highly vulnerable mucous membranes and granulomatous changes of the vocal cords ().
A biopsy, taken shortly before the first presentation to our clinic in an external hospital showed a subepithelial split together with an inflammatory cell infiltration comprising monocytes and granulocytes. The DIF analysis was negative. In our clinic an additional biopsy of the oral mucous membrane stained with haematoxylin and eosin staining was done. The result was negative for MMP showing an increase of collagen fibers with lymphohistiocytic infiltrate and an increased amount of plasma cells in the corium. The DIF analysis revealed unspecific perivascular C3 deposits. Consistent with the first biopsy, a third biopsy with haematoxylin and eosin staining, showed a subepithelial split (). Indirect immunofluorescence using both monkey esophagus and human salt-split skin did not detect circulating IgG- or IgA-autoantibodies. In addition, serum analysis using ELISA with recombinant BP180 NC16A, BP180, BP230, and desmoglein 1 and 3 was negative (). As serology testings were negative, immunoblotting of extracellular matrix was performed, which was positive for circulating IgG4-autoantibodies to γ2-chain of laminin-332 (). The differential diagnosis of Behçet's disease presenting orogenital ulceration was unlikely as the patient only fulfilled one minor criteria, did not show characteristic histological changes for Behçet's disease or any other major or minor criteria for Behçet's disease. Accordingly, clinical criteria such as uveitis or retinal vasculitis, characteristic skin lesions, HLA-typing for B51 and pathergy test were negative. The differential diagnosis of a cytotoxic-mediated disease like Stevens-Johnson-Syndrome was rather unlikely, given the course of the disease, the affected sites, the lack of a possible trigger and the histological findings without signs of a CD8+-mediated reaction like an interface dermatitis or necrotic keratinocytes. An oral candida infection was excluded by a swap. Given the positive history for prostate cancer we performed a tumor staging. The chest-x-ray, ultrasound of the abdomen and PSA-value (0.1 μg/l) were within normal limits. Based on the clinical course, the histological finding and the immunoblot positive for laminin-332-autoantibodies, we suspected a paraneoplastic MMP.
Due to an acute exacerbation with progressive exertional dyspnea, anxiety choking, dry cough, hoarseness and ocular irritation a chest-x-ray, and body plethysmography were performed to exclude an acute exacerbation of asthma. Because of exertional dyspnea a laryngoscopy was performed which revealed progressive oral ulcers as well as a synechia of the first third of the vocal cords.
Even though the diagnosis could not be confirmed by immunohistological criteria at the time of the first symptoms, a paraneoplastic MMP was suspected based on the clinical manifestation with the positive cancer history. Given both, the critical laryngal involvement causing dyspnoea and the ocular bilateral stage 4 symblepharon according to Tauber und Foster classification () (), systemic treatment was initiated. Intravenous methylprednisolone was applied (250 mg/day) at 3 consecutive days. The pulse therapy was repeated for three times every 4 weeks. Oral therapy with dapsone (100 mg/day), which had been initiated after the first pulse therapy was discontinued by the general practitioner due to methemoglobinemia, cyanosis of the lips, and dyspnoea. Instead a combined oral therapy comprising azathioprine (100 mg/day) and prednisolone (50 mg/day) was given. Prednisolone was consecutively reduced to 10 mg per day. Topical treatment included Hylogel due to ocular involvement, inhalation of Tacholiquin 1% and a prednisolone-dexpanthenol solution. Hereafter disease control was achieved with reduction of hoarsness and dyspnoea. Azathioprine was discontinued after 4 month due to elevated values of gamma-glutamyltransferase.
Due to an acute laryngotracheitis with acute dyspnea as well as inspiratory and expiratory stridor, a microlaryngoscopy with division of the synechia of the anterior commissure was performed in the clinic for ear, nose, and throat followed by a fixation of a silicone sheet.
Given both systemic treatments with azathioprine and dapsone had to be discontinued due to adverse effects, therapy with rituximab 1,000 mg was initiated twice in a 14-days interval. The follow-up examination 8 weeks later revealed a stable disease with no new oral lesions (). According to the patient dyspnea did not appear since the start of rituximab treatment. The ocular manifestation of the MMP was assessed stable by the ophthalmologists. During the latest check-up for cancer no signs of relapse were detected. Differential white blood cell count was taken during and after the treatment with rituximab. Initially, total leukocytes and lymphocytes were within normal limits (Leukocytes: 6.75/nl, lymphocytes: 1.13/nl). 7 weeks after the second treatment with rituximab a lymphocytopenia was detected (0.60/nl). Leukocytes and lymphocytes before and after radical prostatectomy were normal (leukocytes: before 6.44/nl, after 9.90/nl, lymphocytes: before 1.12/nl, after 1.24/nl)
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pmc-6362422-1
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A 7-years-old male born to non-consanguineous Caucasian parents presented to our center at the age of 8 months with cutaneous lesions on his trunk. Skin biopsy revealed Langerhans cell histiocytosis (LCH) (). As the disease progressed (cutaneous and mucosal disease), systemic steroids were added achieving partial remission 3 months later. Shortly thereafter he developed worsening anemia, fever, marked hepatosplenomegaly, and oral ulcers. Radiographic skeletal survey imaging revealed lytic lesions in skull and tibia indicating disease progression. Bone marrow aspirate and trephine biopsy did not show infiltration. At this time, he was 18-months-old and was treated according to protocol LCH-IV. During the continuation phase he received clofarabine due to refractory disease ().
At the age of 3 years LCH was in remission and methotrexate and mercaptopurine were started as maintenance therapy. One month after starting treatment, he developed febrile neutropenia, abdominal pain and night sweats. Biopsies were obtained from bone marrow and gut detecting acid-alcohol resistant bacilli identified as Mycobacterium genavense by PCR techniques (). The patient required four intravenous antimycobacterial drugs (rifampin, ethambutol, clarithromycin, and levofloxacin) at standard doses and improved clinically. Follow-up biopsies taken from both gut and bone marrow 1 year after starting specific therapy demonstrated clearing of non-tuberculous mycobacterial bacilli. The patient continued complaining of chronic abdominal pain which was attributed to post-chemotherapy enteritis. Due to the persistence of the pain an MRI was obtained () revealing sclerosing mesenteritis. Systemic corticosteroids were then added. The patient developed severe protein-losing enteropathy with malabsorption, becoming steroid-dependent and requiring long-term parenteral nutrition. A new gut biopsy revealed chronic lymphocytic plexitis (). He had prolonged shedding after viral infections (RSV and norovirus, both requiring specific treatment with ribavirin in both cases).
During the last 2 years the patient has been asymptomatic and free of infections. Anti-mycobacterial treatment was withdrawn 1 year ago without relapse. Currently, he is receiving oral clarithromycin as secondary prophylaxis.
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pmc-6362471-1
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A 48-year-old male patient visited dental hospital with pain and swelling, along with pus discharge in the left posterior back tooth region of the upper jaw since one week. The patient presented the complaints of nasal regurgitation, cough, and intermittent fever since one week. The patient underwent extraction of 26, twenty days back. Pain was mild, continuous, and localised which aggrevated on talking and relieved on medication. Intraoral examination showed opening along the alveolar ridge extending deep into the cortex in relation to 26 (). The OPG revealed radiolucency extending from the alveolar ridge to maxillary sinus, breaking the floor of the sinus in relation to 26. The patient is diabetic, and he is under medication for the last five years.
The patient was advised for excisional biopsy, and the tissue specimen was sent for microscopic examination (). The oroantral opening was closed surgically (). The biopsy specimen showed consists of soft tissues, bone bits, and extracted teeth. The soft tissue is whitish grey in colour, firm in consistency, and irregular in shape.
Microscopic examination showed parakeratinised stratified squamous epithelium in association with loosely arranged collagen fibrous connective tissue. Numerous hyphae were seen which were broad, septate, branched, and scattered all over the connective tissue and admixed with chronic inflammatory cells. Figures and show the decalcified section of bony trabeculae with empty lacunae without osteoblastic rimming interspersed with little fibrous connective tissue and the fungal hyphae. For confirming the fungal hyphae, the PAS staining was done, which also showed the magenta-coloured hyphae in the PAS staining (). With respect to the microscopic features seen, the disease is diagnosed as mucormycotic osteomyelitis.
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pmc-6362473-1
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A 51-year-old woman was presented with complaints of diarrhoea for 3 years. She had no medical history. Colonoscopy revealed slight extrinsic compression of the hepatic angle. CT examination showed a right retroperitoneal mass of 65 × 60 × 90 mm. The well-defined solid lesion was located in the right retroperitoneum, posterior and inferior to the duodenum, on the right side of the cava, and anterior to and on the left side of the right kidney. Based on these findings, the differential diagnosis was between a neurogenic tumour and a mesodermal tumour.
Blood tests for tumoural markers, chromogranin A and urine metanephrines, were negative.
At a sarcoma multidisciplinary meeting, surgery was favoured over biopsy. Due to the inconclusive diagnosis, a conservative laparoscopic approach treatment was decided.
With the patient placed in left lateral decubitus position, four trocars were placed in a semicircular line in the right hemiabdomen. The right angle of the colon was mobilized to locate the tumour in the right side of the duodenum and the cava. The tumour was then dissected by ultrasonic shears and by blunt dissection from the cava; the right gonadal vein and the ureter were found to be in contact with the tumour but without infiltration.
Dissection indicated the tumour arose from the gonadal vein, and this was therefore clipped and divided. The tumour was maintained and completely removed by an accessory incision in the right flank.
En bloc resection was ruled out in view of the uncertain diagnosis. Postsurgical recovery was uneventful. Oral intake was started on the fourth day because she had nausea and vomiting in the immediate postoperative period. She returns slowly to a regular diet and was discharged on the ninth postoperative day.
The histological report confirmed a high-grade leiomyosarcoma (grade 2), with areas of focal necrosis, dystrophic calcification, and positive resection margins (R1). The tumour was described as a fusocelular sarcoma with crossed bundles and high pleomorphism. The mitotic index was less than 9 on 10 high-power fields. In some sections, the morphology seemed to arise from a vessel wall. Immunohistochemistry was positive for alpha actin, desmin, and caldesmon and negative for CD117 and S-100 protein.
The case was again evaluated at a sarcoma multidisciplinary meeting. After the diagnosis was confirmed, it was decided to administer three cycles of adjuvant chemotherapy (epirubicin-ifosfamide) and 50 Gy radiotherapy. The only medical complication was the raised levels of hepatic transaminases. The follow-up was done by blood tests every 6 months and computed tomography (CT) with endovenous contrast every year.
Three years after surgery, although the recommended treatment with en bloc excision with wide nonaffected margins was not performed, the patient is still alive, free of disease, and has a good quality of life. She has not had a recurrence of the disease.
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pmc-6362479-1
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A 45-year-old Caucasian obese woman presented with small painful ulcers on the back of her hands and fingers that had started three weeks prior to her visit. She first noticed small red flat discolored areas which gradually worsened by developing pain, swelling, and ulcers within two weeks. She did not recall a prior trauma. She had a history of previous laparoscopic gastric sleeve surgery for morbid obesity and a vague diagnosis of mild diabetes for which she was not on any medication. She denied taking a new drug. With the clinical diagnosis of an infectious process, bacterial culture and sensitivity were performed and she was given oral and topical antibiotics (Bactrim and mupirocin, respectively) along with wound care instructions. The patient started developing fever with malaise and was admitted to the emergency room, where she was placed on intravenous antibiotic (vancomycin) due to suspicion of sepsis, originating from her “hand infection”. After a few days, she returned to our clinic. Compared to the original visit, the condition appeared worse with development of erythematous ulcerated nodules and plaques, violaceous borders, and marked surrounding edema, present on the dorsal aspects of right index and left ring fingers along with proximal metacarpophalangeal joint of third digit. The fingers in the nonulcerated areas displayed a fusiform swelling (Figures and ). She also developed tender indurated erythematous plaques on the dorsum of the right wrist. Examination of the rest of the body, including the mucosal surfaces, failed to show any involvement. Based on the clinical progression and lack of response to antibiotics, biopsy was obtained to rule out atypical pyoderma gangrenosum (PG), deep fungal or mycobacterial infection, or other possibilities. Histopathologic examination revealed marked subepidermal edema associated with a superficial and deep perivascular, interstitial, and diffuse infiltrate of neutrophils, many of which were present within the vessel walls, associated with leukocytoclasia and extravasation of erythrocytes. Despite vasculopathic changes, there was no evidence of true vasculitis (Figures , , and ). Although the histopathologic differential diagnosis was most consistent with Sweet's syndrome, based on the clinical presentation, NDDH was the rendered diagnosis. She was immediately started on oral prednisone 80mg per day. In the meantime, a systemic workup was carried out. After a week, the ulcers were already healing and the swelling was subsiding. Tissue cultures yielded negative results and systemic workup was normal. We started to taper down the prednisone at this point. One week later, there was continued flattening of the lesions. After one month, there was mild residual erythema at the previous sites (Figures and ). We continued to taper down her prednisone and prescribed a potent topical corticosteroid in case of local recurrence. She reported complete clearing of the lesions. Seven months later, she presented with a mildly erythematous patch on the dorsum of right third digit. An intralesional steroid injection was performed followed by topical steroids for two weeks. She has not had any recurrence, after one year from the original incidence.
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pmc-6362484-1
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A 17-year-old male patient presented with a left patellar fracture that resulted from a fall from a standing height 8 years ago. He did not undergo any type of surgical treatment during that time, but the fracture was immobilized with crural-crustal plaster, albeit for only 2 weeks. At the present consultation, the patient presented with an active range of motion (ROM) of 70° to 120° and passive ROM of -5° to 120° (Figures and ).
In the first phase, transskeletal patellar traction was performed, and a Steinmann pin with a 3.5 mm thick central thread (Figures –) was inserted transversely into the proximal pole. Transskeletal patellar pin assembly is very easy to perform with the patient under sedation and local anesthesia.
The traction device placed on the patella had an initial weight of 3 kg, which was increased daily by 0.5 kg. Serial radiological images were obtained to quantify the decrease in the distance between the two poles of the area of pseudoarthrosis (). On day 11, diastasis between the fragments, which was 9 cm preoperatively, was reduced to 1.2 cm with the knee in full extension ().
Then osteosynthesis was performed with a tension band. We removed the traction device and the traction pin from the proximal pole of the patella, with the patient under spinal anesthesia with femoral nerve block. We performed median longitudinal surgical access and plane dissection and identified bone fragments of the patella. We passed two 2.0 mm thick Kirschner wires, longitudinally and parallelly, into the two fragments. We attempted to reduce the fragments with two Backhaus clamps (), but the contact between the fragments was not possible.
We performed cerclage wiring with a 1.2 mm thick cerclage wire followed by a figure-of-eight tension band. This technique considerably reduced the distance between the pseudoarthrosis foci, but the contact was insufficient ().
Next, we proceeded with an insertion of a 1.2 mm circular cerclage bonding wire, and finally, we achieved good contact between the bone fragments of the pseudoarthrosis. After thoroughly cleaning the site with 0.9% physiological saline, we focused on the pseudoarthrosis, an autologous spongy osseous graft from the proximal tibia, and removed it through a small osseous hole made in the anteromedial tibia (Figures –).
The patient was reassessed during the postoperative period for bone consolidation (assessed by radiography), pain (visual analog scale (VAS), 0-10), quadriceps strength (motor force rating) [], and ROM of the limb (using a goniometer).
The patient was not immobilized and allowed full weight bearing with crutches, but knee flexion was restricted for 8 weeks. During week 8, physiotherapy focused on increasing the patient's ROM. During this period, the patient had a passive arch of 0° × 10°, grade III quadriceps strength, and a VAS score of 4, and radiography showed signs of bone consolidation. During postoperative month 3, a passive ROM of 0° × 25°, active ROM of 10° × 25°, grade IV quadriceps strength, VAS score of 4, and consolidated patella on radiography () were observed. During postoperative month 18, the patient had an active and passive ROM of 0° × 90° (), grade V quadriceps strength, and VAS score of 1.
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pmc-6362489-1
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A male in his early 30s had a witnessed cardiac arrest shortly after an emotionally stressful event. Bystander CPR was initiated immediately. When emergency medical services arrived, the presenting rhythm was ventricular fibrillation and 1 shock was delivered. He was intubated at the scene. Standard advanced cardiac life support was continued en route to the nearest emergency department. In total, four cycles of CPR, epinephrine, and defibrillation were given prior to the return of spontaneous circulation, obtained upon the patient's arrival at a community hospital. We were urgently contacted by the community hospital because his initial ECG was consistent with left bundle branch block morphology but then progressed on serial ECGs with significant ST elevations noted in leads I, AVL, and V4-V6 and ST depressions in leads III and aVF. The patient was transferred emergently to our institution for emergency coronary angiography in the setting of ST elevation myocardial infarction (STEMI). The patient's past medical history is significant for a stroke at age 7 without any residual deficits and hypertension. He was recently diagnosed with polycythemia vera (Janus kinase 2 (JAK2) positive) and was prescribed enteric-coated ASA 81 mg daily and hydroxyurea. The patient had previously been undergoing regular phlebotomy at our institution but had not attended these appointments over the last two months. It was unclear whether he was taking any medications at the time of presentation. Initial bloodwork showed the following: hemoglobin 184 g/L, hemotocrit 0.59, platelet count 1072 × 109/L, leukocytes 38.8 × 109/L, creatinine 142 μmol/L, hsTroponin 506 ng/L (peak 67322 ng/L) and CK 980 U/L (peak 9013 U/L), pH 7.12, and lactate was 9 mmol/L.
Upon arrival at our institution, he was in cardiogenic shock with a blood pressure (BP) of 95/80 mmHg and a heart rate (HR) of 110 bpm on 2.5 mcg/kg/min of intravenous (IV) dopamine, 1 mg/min IV amiodarone, 20 mcg/kg/min IV propofol, and intermittent IV boluses of 50 mg rocuronium. He also received 120 mg of IV furosemide. IV norepinephrine was initiated at 10 mcg/min, and dopamine was discontinued. His ECG showed an anterolateral STEMI (). ECASA 160 mg and ticagrelor 180 mg were given to the patient via the NG tube, and 4000 units of IV heparin was administered. He was brought urgently to the cardiac catheterization laboratory. Selective coronary angiography demonstrated a normal right coronary artery with collaterals to the left circumflex artery (LCX), complete occlusion of his left anterior descending artery (LAD), and a long 90% lesion of the proximal LCX (Figures and , Supplementary Videos and ). The left ventricular end diastolic pressure was 30 mmHg. As he was on a significant amount of vasoactive medications for hemodynamic support, we did not believe it was appropriate to administer intra-arterial nitroglycerin at this time, nor did we feel this would lead to any change in his clinical management, as there was clear thrombus in the LM, LAD, and LCX. His oxygen saturation was 95% on 100% FiO2, but his BP was 84/68 and HR 118 bpm, despite being on 15 mcg/min of IV norepinephrine. Based on the patient's clinical status and angiographic findings, an 8 French 40 cc intra-aortic balloon pump (IABP, Arrow® International, Teleflex Medical, Athlone, Ireland) was inserted into the right femoral artery followed by the administration of additional 7,000 IU (patient's weight was 110 kg and had already received 4000 units preprocedure) of intra-arterial heparin. We then proceeded with emergency percutaneous coronary intervention (PCI) on the LAD and LCX. There was a significant amount of thrombus in the LAD and LCX (, Supplementary ); and thus, 6 passes with a 6 French Export Advance™ Aspiration Catheter (Medtronic Inc., Minneapolis, MN) were performed. Soon after thrombectomy was completed, the patient experienced recurrent thrombosis of the left coronary system. Repeat thrombectomy was performed; however, the thrombectomy catheter itself became clotted.
To combat the extensive thrombus formation in the setting of PV, we performed intraprocedural phlebotomy. A total of 550 mL of blood was phlebotomized, and two boluses of intracoronary eptifibatide were given. Despite this fact, there was a significant amount of recurrent thrombus formation and a second dose of intra-arterial heparin at 100 IU/kg was given for a total of 21,000 IU of IV unfractionated heparin which was administered for the procedure. Although an ACT was not drawn during the case, at the end of the case, it was 250 seconds. Eventually, the de novo coronary thrombosis stopped, and complex bifurcation stenting of the LAD, left main artery, and LCX could be performed using 2 drug-eluting stents and a bifurcation minicrush technique (, Supplementary Videos and ). Despite these efforts, he remained in cardiogenic shock (BP 101/57 mmHg, HR 107 bpm) while on significant hemodynamic support (IABP at 1 : 1 cycle, IV norepinephrine 20 mcg/min, IV vasopressin 0.04 units/min, and IV epinephrine 20 mcg/min). The advanced heart failure and cardiac surgical teams were consulted, and the decision was made to bring the patient directly to the operating room for an emergency left ventricular assist device (LVAD-CentriMag, Thoratec Corporation, Pleasanton, CA), which the teams felt to be more appropriate than an extracorporeal membrane oxygenation circuit. Additionally, 1 g of hydroxyurea was given at the end of the procedure via an oral gastric tube following consultation with hematology for the acute treatment of PV.
His postoperative course was complicated by left-sided weakness and radiologic evidence of a new stroke (right occipital parietal subacute infarct with hemorrhagic transformation along with multiple small subacute infarcts in the right frontal, left frontal, and left parietal occipital regions), pneumonia, and a slow wean from the LVAD-CentriMag. Postprocedure imaging demonstrated evidence of significant mitral regurgitation secondary to complete flail of the anterior mitral valve leaflet with chordal rupture, which may have occurred following the emergency insertion of the CentriMag device. The patient underwent an Alfieri stitch procedure on LVAD removal, to reduce the degree of mitral regurgitation from severe to moderate. The patient eventually made significant neurological recovery and was discharged from the hospital to an inpatient cardiac rehabilitation facility, 38 days after admission to our institution. His ejection fraction at discharge was 25-35% with moderate mitral regurgitation.
After discharge, the patient had a single chamber ICD inserted due to the persistence of severe LV dysfunction with a left ventricular ejection fraction of 20-25% as well as moderate-to-severe RV dysfunction. The distal half of his LV, as well as the septum, was thinned and akinetic. Within 3 months after discharge, he was readmitted to the hospital with right-sided heart failure symptoms and weight gain. Investigations revealed portal vein thrombosis and right-sided heart failure as the causes. He has required multiple dose adjustments of his cardiac medications due to development of cardiorenal syndrome. At 1 year post MI, his ejection fraction remained <30% with anterior wall akinesis, aneurysmal apex, and moderate to severe mitral regurgitation. He continues to be followed by cardiology, a dedicated heart failure clinic, respirology, neurology, haematology, and nephrology.
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pmc-6362495-1
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A 32-year-old female patient was referred to the Department of Conservative Dentistry and Endodontics with chief complaint of discolouration of the upper right front tooth (tooth 11) for the past 6 months. Past dental history revealed a traumatic fall 5 years ago which involved the upper front teeth for which no dental treatment was sought for. Recently, as the discolouration gradually worsened, the patient sought dental treatment to restore esthetics.
Intraoral clinical examination revealed a slightly discoloured 11 and chipping of the incisal edge with no presenting symptoms (). Both 11 and 12 responded negatively to the electric pulp tester (Gentle Pulse™ Pulp Vitality Tester, Parkell, USA) and elicited mild tenderness on percussion.
On radiographic examination (VistaScan Mini, UK), extensive periapical radiolucency involving the roots of 11 and 12 and an open apex of 11 was revealed (). CBCT for this case (Dentsply Sirona, Orthophos XG 3D) was taken at standardized settings (90 kV, 6 mA, 5 × 5.5 cm, 160 μm, and 14 s) to assess the exact location, size, and extent and proximity of the lesion to anatomical structures.
The preoperative measurements of the lesion as seen in CBCT in different planes can be seen in Figures , , . According to the CBCT-PAI scores, it was graded as a 5D lesion []. The score 5 indicates that the diameter of the periapical lesion is greater than 8 mm, and D represents destruction of the periapical cortical bone in the palatal region.
A tentative diagnosis of an Ellis Class IV fracture and open apex in 11 with apical periodontitis in 11 and 12 was made. The differential diagnosis could be a chronic periapical abscess, periapical cyst, and periapical granuloma. The treatment plan was root canal treatment for 11 and 12. The patient was informed about the risks and benefits of the procedure and a written consent was taken.
Under local anaesthesia (2% lignocaine in 1 : 200,000 dilution adrenaline, Neon Laboratories Ltd.) and rubber dam isolation, an access cavity was prepared in 11 and 12 with an Endo access bur (Dentsply Maillefer, Switzerland). The working length was determined with an apex locator (Propex Pixi, Dentsply Maillefer, Switzerland) and confirmed with radiograph. Cleaning and shaping was initiated with 45 K file (Mani, Inc. Japan), and apical preparation was performed till size 80 K file in 11. In 12, apical preparation was done till size 45 K file to full working length, after which step-back preparation was done till 80 K file. Routine root canal-shaping procedure was done along with copious irrigation using 3% sodium hypochlorite (VIP, Vensons, India) and final flush with 0.9% physiologic saline (acuLIFE, India). Calcium hydroxide medicament (RC Cal, Prime Dental Products, India) was placed thrice for a period of 1 week each. As the root canals exhibited persistent discharge of exudates and due to two prognostic factors (size of the lesion which was more than 10 mm and the thinning or destruction of the palatal bone) which were unfavourable for this case [], periapical surgery was planned following the recommendation given by the Spanish Society of Oral Surgery (point 3: a radiotransparent lesion measuring over 8 to 10 mm in diameter).
The root canals were obturated a day before the surgery using custom-made roll cone technique for 11 and conventional cold lateral compaction technique for 12.
During surgery, after achieving adequate anaesthesia, a crevicular incision was placed from 22 to 14 with a vertical releasing incision on the mesial aspect of 14 to reflect a full thickness mucoperiosteal triangular flap. Cortical softening of the periapical bone was noted from regions 11 to 13. A bony window was created and thorough curettage was done ().
Apicoectomy was performed extending 3 mm into the canal space using no.702 tapered fissure burs (SS White burs) in 12. Apicoectomy was not done in 11 because of the presence of an open apex. Root end cavity preparation was done using zirconium nitride ultrasonic retro-tips (Dentsply Maillefer, Switzerland) in 11 and 12. Subsequently, retrograde filling was done with Mineral Trioxide Aggregate (MTA Angelus® Brazil) (). Later, the surgical site was prepared for placement of CGF fibrin gel and CGF membrane.
A standard, disposable, two 10 mL nonanticoagulant glass tubes and a matching centrifuge device (MEDIFUGE, Silfradent s.r.l., S. Sofia, Italy) were used. 20 mL of intravenous blood sample from the patient was placed in centrifuge tubes without anticoagulants and accelerated for 30 s, centrifuged at 2700 rpm for 2 min, 2400 rpm for 4 min, 2700 rpm for 4 min, and 3000 rpm for 3 min, and decelerated for 36 s to stop. All of these processes are adjusted automatically by “preprogramming” in the machine.
From the three layers formed, the uppermost platelet-deprived fraction was removed with a sterile syringe. The layer in the form of a fibrin gel containing the CGF was separated from the red blood cell layer. The prepared CGF fibrin gel was placed inside the surgical site and covered with CGF membrane. The layer in the form of a membrane containing the concentrated growth membrane was held with a hemostatic clamp and separated from the RBC layer by using microsurgical scissors. The CGF layer is then placed in a condensing disc and compressed to convert to CGF membrane. (Figures , ).
Subsequent to CGF placement, the flap was closed with 3-0 vicryl sutures (Ethicon Inc. Piscataway, USA). Postoperative instructions were given and systemic antibiotics, analgesics, and supplemental 0.2% chlorhexidine mouthwash were prescribed.
The postoperative CBCT at 1-year follow-up showed satisfactory healing with evident reduction in lesion size as shown in Figures , , , and the patient was asymptomatic at all the recall periods, suggesting a successful treatment outcome. The patient is kept under review, to be followed up after 18 months, 24 months, and 36 months.
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pmc-6362495-2
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A 35-year-old female patient reported mild swelling and pus discharge in the lower front region of the mouth for the past 2 months. Past dental history revealed trauma to lower anterior teeth 4 years ago, following which she underwent endodontic treatment. History suggested that there were multiple retreatments in the past for the current chief complaint.
On clinical examination, 31 was discoloured with mild swelling on the labial aspect (). Radiographic examination revealed a well-obturated 31 and 41 with large periapical radiolucency (). The CBCT-PAI score was 5D (Figures , , ). Based on the above findings, a diagnosis of previously root canal-treated 31 and 41 with a chronic apical abscess was made. History suggested that there were multiple retreatments in the past for the current chief complaint, with presenting complaint of recurring, intermittent swelling with pus discharge in the lower front region. And radiographic examination revealed adequate obturation and apical seal, with nonhealing chronic periapical radiolucency. The treatment plan of periapical surgery was decided in accordance with the indications given by the European Society of Endodontology, 2006 (point 3: presence of persisting periapical disease after root canal retreatment).
Under local anaesthesia, the full thickness trapezoidal mucoperiosteal flap was reflected with vertical releasing incisions taken from the mesial aspect of 43 and 33. The surgical site was flushed with sterile saline after thorough curettage of the lesion (). Following which, apicectomy and retrograde MTA filling were done (). The CGF preparation was similar as described in Case Report 1, and the prepared CGF was placed inside the surgical site and covered with CGF membrane (Figures , ). The flap was approximated with interrupted sutures. Follow-up CBCT at 1 year revealed complete healing with complete bone repair, evidently seen in coronal and sagittal views (Figures , , ).
At 1-year follow-up with CBCT, livewire segmentation using OSIRIX Version 9.5 (PIXMEO, Geneva, Switzerland) was done to delineate the lesion from the healthy bone. In case 1, the preoperative and postoperative volume calculations were 0.7862 cm3 and 0.08 cm3, respectively. The lesion size reduction was found to be 89.2%. In case 2, the preoperative and postoperative volume calculations were 0.1358 cm3 and 0.0101 cm3, respectively. The lesion size reduction was found to be 92.5%.
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pmc-6362506-1
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An otherwise healthy 78-year-old man experienced subacute-onset back pain that radiated to both lower extremities and was worse with ambulation. Over the course of a month, the patient then experienced progressive neurological deterioration with bilateral leg weakness leading to an eventual inability to walk. Magnetic resonance imaging (MRI) study, performed one month after onset of symptoms, showed a lesion with extensive enhancement of the lesion periphery () and extension of enhancement to the distal nerve roots on sagittal fat-suppressed T1-weighted MRI (). Axial T1-weighted MRI with contrast demonstrated hyperintense lesion periphery (), and axial T2-weighted MRI demonstrated homogenous hyperintensity of the lesion (). Lastly, sagittal T2-weighted MRI demonstrated evidence of degenerative changes but no involvement of the vertebral bodies or disc spaces (). Since the patient did not have any indication of infection, our working diagnosis was that of a malignant neoplastic process with probable spread to the distal nerve roots.
Based on the symptomatology and extensive involvement of the nerve roots, the plan was to perform a biopsy of the lesion to obtain a histological diagnosis. The patient was positioned prone on a Jackson table, and neuromonitoring for somatosensory evoked potentials (SSEP) and motor evoked potentials (MEP) was established (Supplementary ). A vertical incision was made over L2 through L4, and intraoperative X-ray was used for localization of the lesion. Bilateral laminectomy of these levels was performed, and the dura was fully exposed and opened over the midline. At this point, thickened, reactive arachnoid was encountered. Opening the dura, we found that the nerve roots were plastered and very adherent to this arachnoid. In certain cases where the pathology is not immediately evident, intraoperative ultrasound is a useful adjunct to provide real-time confirmation of the location of the lesion. We have found this to be especially valuable in cases of intrinsic intramedullary lesions. With sharp dissection, an incision was made in this tissue, and the dissection was continued toward the surface of the mass. After incision of the capsule, thick puss was encountered. At this point, the true nature of the lesion became apparent, so pus was carefully drained, cultures were obtained, and the walls of the abscess were removed and biopsied. The dura was closed primarily with no observed changes in SSEPs or MEPs throughout the procedure. Cultures from the intraoperative specimen grew S. aureus. The patient experienced immediate improvement of pain after surgery and progressive, but incomplete, improvement of his weakness. He was discharged on postoperative day two with long-course intravenous antibiotics.
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pmc-6362591-1
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A 54-year-old Japanese woman visited our hospital due to palpitations and wide QRS tachycardia with persistent tiredness for several months. She underwent repair of TOF when she was 2-years old. After the repair, no follow-up check was performed. During the period, she gave birth to three children. She underwent atrial flutter ablation (cavo-tricuspid isthmus block line) when she was 50-years old and 51-years old.
On admission, she had wide QRS tachycardia of 180 beats/minute, but it spontaneously converted to sinus rhythm. This paroxysmal wide QRS tachyarrhythmia of a few minutes’ duration was easily observed several times.
An electrocardiogram showed prolonged QRS duration (199 msec) with a complete right bundle branch block and an echocardiograph demonstrated that her right ventricle was highly enlarged and had poor contraction, and severe pulmonary valve regurgitation with one leaflet flail (Fig. ). Four-dimensional flow MRI demonstrated that regurgitant volumes (RVols) and regurgitant fractions (RFs) of PR (Fig. and Additional file : Movie S1) were calculated as 63.12 ml and 54.0%, respectively. RV end-diastolic/end-systolic volume index (RVEDVI)/(ESVI) was 169.54/99.76 mL/m2, and the cardiac index (CI) was 1.78 L/minute per m2. Flow energy loss (FEL) calculated from four-dimensional flow MRI was 2.93 mW, which is estimated to be three times higher than normal controls (Additional file : Movie S2). An electrophysiological study showed an intact anterior internodal pathway and a slow pathway just through the outside of the right atriotomy line scar, which is supposed to cause a re-entry circuit (Fig. ).
We decided to perform a PVR and the right maze procedure because the energy loss of the right side of her heart system was high and, additionally, arrhythmic therapy was needed, even if the extremely dilated RV size did not get restored. Cardiopulmonary bypass (CPB) was instituted with the ascending aorta and bicaval cannulation. Anterior and posterior pulmonary valve leaflets were prolapsed and the left leaflet was highly degenerated. The pulmonary annulus was intact with sufficient size; thus, a bioprosthetic valve was implanted in the native pulmonary annulus with a supra-annular position. After all the leaflets were resected, a Mosaic Ultra® 27 mm (Medtronic, Minneapolis, MN, USA) was placed on the supra-annular position. Concomitantly, the right maze procedure was performed. The right atrium was plicated with the removal of the scar, adding the incision to the lateral side of the atrium, and cryoablation on the posterior and anterior walls of the atrium from the incision line to the tricuspid isthmus and to the inferior vena cava.
Her postoperative course was uneventful and she discharged on day 21. A four-dimensional flow MRI done 3 months later showed that RVEDVI/ESVI had significantly reduced to 85.24/55.41 mL/m2 and her CI had increased from 1.78 to 2.58 L/minute per m2. Energy loss had greatly improved from 2.93 to 1.48 mW (Fig. ).
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pmc-6363236-1
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A 13-year-old boy presented to pediatric surgery unit due to abdominal pain in the left abdomen without fever, nausea, vomiting, or genitourinary symptoms. He had no history of trauma or injury or operative treatment. His family history revealed no significant disease. On admission, the patient presented blood pressure 120/80 mmHg and 80 bpm, and all laboratory data were within normal limits. Examination of the abdomen showed pain in the left abdomen but negative McBurney or Bloomberg signs. Plain Rx of the abdomen did not show signs of intestinal obstruction or perforation. Abdominal US showed a mass 7 cm in diameter, located anteriorly and inferiorly to the spleen with similar structure. Abdominal MRI with contrast confirmed previous finding and, in addition, showed an area of intracapsular hemorrhage in the mass (Figures –). Diagnosis was intracapsular hemorrhage in a giant accessory spleen. Then, the child was brought to OR. Removal of the accessory spleen was made by left subcostal incision without difficulties. The child resumed liquid diet the day after and was discharged on the 3rd postoperative day. The histological examination confirmed the diagnosis.
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pmc-6363242-1
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A 72-year-old male with a long standing history of chronic lymphocytic leukemia (CLL) presented with upper respiratory symptoms including mild productive cough and dyspnea. An outpatient chest CT showed innumerable bilateral ill-defined solid pulmonary nodules in a peribronchovascular distribution, which were new from a prior scan 6 months earlier (). Many of the lesions had a peripheral ground-glass halo. Nonenlarged mediastinal and bilateral axillary lymph nodes were suspected to be related to the patient's history of CLL. The pulmonary nodules were not a typical manifestation of CLL and other etiologies were considered such as atypical pulmonary infection, sarcoidosis, Kaposi sarcoma, and metastasis, even though patient had no other known malignancy. He was treated with antibiotics and steroids for his symptoms; however there was progressive clinical decline over several weeks and thus the patient was admitted for further work-up.
At the time of admission, vital signs showed exertional hypoxia, mild tachycardia in the low 100's, and a fever up to 102.4, which raised the concern for an infection and septic emboli. However, there were no significant pulmonary findings on exam. Skin examination revealed erythematous/purple skin papules on both lower extremities which broadened the differential to also include autoimmune and vascular etiologies.
Aside from normocytic anemia (Hb 11.6 mg/dL), initial laboratory evaluation with CBC and BMP showed no significant abnormalities. IgG levels were low. Blood and sputum cultures were negative. QuantiFERON was negative for tuberculosis. Serologies for aspergillus, blastomycosis, coccidioides, cryptococcus, histoplasma, HIV, and toxoplasma were negative. Bronchoalveolar lavage was negative for acid-fast bacilli, fungal organisms, and pneumocystis.
Immunologic evaluation was negative for ANCA, proteinase 3, and myeloperoxidase antibodies. This excluded granulomatosis with polyangiitis as a differential diagnosis. Bronchoalveolar lavage was negative for cytology.
Punch biopsy of one of the skin lesions demonstrated poorly circumscribed granulomas surrounding blood vessels and skin appendages, mild lymphocytic infiltration with no features to suggest cutaneous lymphoma, and no evidence of leukocytoclastic vasculitis. Stains for fungal and acid-fast bacilli were negative.
Left upper and lower lobe wedge biopsies of the nodules were taken through video-assisted thoracoscopic surgery (VATS) as the diagnosis was still unclear. The biopsies revealed EBV-positive DLBCL with features of LG grade 3 (). The features that favor LG over DLBCL include a background that consisted predominantly of inflammatory cells with a minority of large B-cells and vascular invasion. In addition, lung involvement and EBV positivity are not exclusive to LG, but are almost always present in LG []. Flow cytometry did not detect the large B-cells which are sometimes too fragile to survive flow cytometric processing, but did detect rare, small monoclonal B-cells with a CLL/small lymphocytic lymphoma (SLL) phenotype. The flow cytometric findings are compatible with the morphology as no significant CLL/SLL population could be identified with CD5 and CD23 immunostaining (). In this case, the source of rare CLL cells is likely peripheral blood, either physiologically through the inflammatory response or contamination during resection. Features of secondary organizing pneumonia were also present.
The patient underwent PET-CT 4 weeks following the initial chest CT (). This showed significant progression and confluence of bilateral peribronchovascular lung opacities. The lung opacities had diffuse FDG uptake with a maximum SUV of 22.3. The PET/CT also demonstrated intense uptake in the cutaneous lesions of the lower extremities.
Bone marrow biopsy was consistent with hypercellular marrow with 20-30% marrow involvement by CLL/SLL cells. The patient was started on R-CHOP chemotherapy with Neupogen support. IVIG was also given for hypogammaglobulinemia.
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pmc-6363593-1
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A 70-year-old Caucasian male presented to emergency with 10 days of dry cough, dyspnoea, and fatigue. His respiratory rate was 18 per minute with saturations of 93% on 2 L of nasal prong oxygen, heart rate 75 beats per minute and in sinus rhythm, blood pressure 104/60 and was afebrile. Examination demonstrated globally reduced air entry with bibasal crackles with no other pertinent findings.
His medical history included HIV diagnosed in 1997 and well controlled with combination anti-retroviral therapy (sequential viral loads undetectable and CD4 >500/mm3) and Barrett's oesophagus. His surgical history included a trans-urethral prostate resection for hypertrophy as well as a left total knee replacement and a decompression for spinal stenosis. Medications were allopurinol, rosuvastatin, atazanavir, ritonavir, lamivudine, zidovudine, and pantoprazole. He had a 40 pack-year smoking history (quit 15 years ago), minimal alcohol, and no illicit substance history. He worked in an office, was an active swimmer, and not in a relationship. Vaccines were up to date.
Initial investigations revealed a haemoglobin of 115 g/L, neutrophils 8.19 × 109/L, lymphocyte 0.49 × 109/L, eosinophils 0.17 × 109/L, and platelets 390 × 109/L. Creatinine was 441 micromol/L (86 six months prior), urea 25.4 mg/dL, and C-reactive protein 148 mg/L. Chest film demonstrated diffuse bilateral infiltrates predominantly in the lower zones. Urinalysis demonstrated >500 erythrocytes, 10 leucocytes, and no bacteria.
With a provisional diagnosis of pneumonia he was commenced on ceftriaxone, azithromycin, and oxygen. Intravenous fluids continued.
The following day his creatinine increased to 512 despite 3 L of intravenous fluid. An arterial blood gas showed type 1 respiratory failure with a haemoglobin 98 g/L. A broad screen for infectious and non-infectious aetiologies was performed, and a routine HIV viral load taken 4 days prior to presentation was 146 copies/mL. Anti-retrovirals were changed to dolutegravir, efavirenz, and lamivudine due to renal failure.
By day 3 his fatigue and dyspnoea had worsened without new symptoms. Repeat chest film demonstrated worsening infiltrates (Fig. ). His oxygen requirement had increased to 50 L/min, 50% FiO2 via hi-flow nasal cannulae. Dialysis was commenced with red cell transfusions as his haemoglobin was now 82 g/L. A repeat urine had >500 erythrocytes, protein 1100 mg/L, creatinine 188 g/mol, myoglobin 14 μg/L, and normal haemosiderin.
Day 4 returned a Perinuclear-ANCA (P-ANCA) titre >1:640 with all other diagnostic investigations unremarkable, and a provisional diagnosis of MPA was made. Treatment with intravenous cyclophosphamide (800 mg second weekly, three doses), plasma exchange (second daily, seven sessions) and methylprednisolone (500 mg daily for 3 days, then long-course prednisolone) was started. He also received prophylactic trimethoprim/sulphamethoxazole, intravenous iron, and darbepoetin alfa.
With a substantial clinical and radiological improvement (Fig. ) he was discharged after a 29-day stay with ongoing thrice weekly haemodialysis. Renal biopsy five days after discharge demonstrated crescentic-class pauci-immune necrotizing glomerulonephritis without granulomata, consistent with MPA.
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pmc-6363659-1
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A 38-year-old Caucasian female presented to the emergency department with 3 days of left lower quadrant abdominal pain rated 6/10 with radiation to the left lower back. She also reported a high-grade fever (103°F) with associated nausea and vomiting. She denied dysuria or hematuria. Medical history was significant for well-controlled type II diabetes mellitus, hypertension, and hyperlipidemia. She was taking canagliflozin 100 mg daily, lisinopril 20 mg daily, and atorvastatin 80 mg daily. She has been on canagliflozin (SGLT-2 inhibitor) for type 2 diabetes mellitus for 14 months prior to the current presentation. She denied any history of urinary tract infections or renal stones. On presentation, her blood pressure was 172/86 mmHg, heart rate 94 beats per minute and temperature 102°F. BMI was 46.61 kg/m2. Physical examination revealed tenderness to palpation in the left lower quadrant and left flank. The rest of the examination was unremarkable. Urinalysis () revealed pyuria, bacteriuria, and nitrites. Hemoglobin A1C was 7.5% (59 mmol/mol). CT abdomen and pelvis without contrast showed an obstructive 4–5 mm left distal ureteral stone associated with mild hydroureteronephrosis. She was diagnosed with obstructing nephrolithiasis complicated by pyelonephritis and was empirically treated with intravenous ceftriaxone 1 gram every 24 h. Cystoscopy with retrograde pyelography was done and a left 6-French × 24 cm double-J ureteral stent was placed. Placement was confirmed with fluoroscopy and cystoscopy. Intraoperative urine cultures obtained from the left renal pelvis and bladder showed no growth. She was discharged home on cefdinir 300 mg twice a day for 14 days and tamsulosin 0.4 mg daily for 30 days with a urology follow-up appointment in 2 weeks.
Ten days later, she presented again with intermittent low-grade fever (100.5°F) and left flank pain. She was hypotensive (96/58 mmHg), tachycardic (104 bpm) and febrile with temperature of 102.8°F. Physical examination revealed severe tenderness to palpation in the left lower quadrant extending to the suprapubic area. Laboratory workup () showed worsening leukocytosis, pyuria, bacteriuria, positive urinary nitrites, and small leukocyte esterase. A repeat CT scan of the abdomen and pelvis with renal stone protocol confirmed the position of the left ureteral stent and absence of previously seen stone. She was diagnosed with sepsis secondary to acute pyelonephritis in the setting of recent stent placement and started on aggressive intravenous hydration and Piperacillin-Tazobactam. Urine culture from the day of admission grew Klebsiella pneumoniae with >100 k CFU/ml however blood cultures remained negative. Despite appropriate antibiotics, she remained febrile even after 48 h. Two days later, the anaerobic blood cultures became positive for C. glabrata. She was started on intravenous micafungin 100 mg every 24 h and antibiotic was narrowed down based on culture sensitivities. Repeat cystoureteroscopy with retrograde pyelogram revealed partial contrast uptake in the left kidney due to an impacted ureteral stone in the mid ureter. The presence of fungal elements and cloudy urine were also found in the left kidney. The previously placed left ureteral stent was removed and a new double-J ureteral stent was placed. Urine from the left renal pelvis and bladder were cultured for a second time which grew C. glabrata susceptible to Fluconazole. On the 4th day of admission, blood cultures from both anaerobic and aerobic samples grew C. glabrata. Dilated fundus examination was negative for fungal ocular involvement and transthoracic echocardiogram was unremarkable for valvular vegetations. Blood cultures after antifungal initiation remained negative. She was eventually discharged on oral fluconazole 800 mg daily for 2 weeks and cefazolin 2 g every 8 h for 7 days.
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pmc-6363666-1
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A 13-years-old mentally impaired girl (since birth due to perinatal asphyxia) presented to the emergency department (ED) with a severe abdominal pain and signs of acute abdomen, fever, and hypovolemic shock. She was unconscious, febrile, with a blood pressure of 80/40 mmHg, pulse rate of 160/min, and a respiratory rate of 34/min. Physical examination revealed a diffuse tenderness and a muscular rigidity. The abdomen was distended and bowel sounds were absent. The abdominal X-ray obtained in a supine position showed a massive free air within the peritoneal cavity and undigested remains of food along the alimentary tract imitating the contrasting liquid (). At hospital admission, a medical audit accompanying the girl described a 4-days history of food rejection, frequent vomiting and progressive deterioration of her general condition. Her anamnesis was negative for traumatic events but was suggestive for PA due to a characteristic appearance of air swelling and abdominal distension that rapidly progressed during the day and caused the flatus during sleep.
Laboratory investigations revealed the following results: White blood cell count: 7.100/uL; mean platelet volume: 11.8 fL; platelet count: 220 × 103/uL; hemoglobin: 11.1 g/dL; hematocrit: 35.2%; serum proteins: 5.7 g/dL; serum albumin: 2.9 g/dL; serum globulin: 2.8 g/dL; aspartate aminotransferase: 107 IU/L; alanine aminotransferase: 30 IU/l; creatinine: 0.9 mg/dl; serum sodium: 135 mEq/L; serum potassium: 5.4 mEq/L; serum chloride: 101 mEq/L; C-reactive protein: 367.4 mg/L; serum lactate dehydrogenase: 401 IU/L; serum creatine kinase: 4,086 IU/L; serum glucose: 107 mg/dL; arterial blood pH: 7.07 nmol/L; PaCO2: 4.93 kPa; pO2: 5.59 kPa; HCO3: 10.5 mmol/L; base excess: 18.8 mEq/L.
Following an aggressive resuscitation with intravenous hydration, decompression of the stomach, a correction of metabolic abnormalities, and administration of empiric antibiotic therapy (amikacin, metronidazole, and meropenem), an emergency laparotomy was performed. It revealed a massively distended and partially necrotic stomach. Huge amounts of free peritoneal fluid (about four liters) with food particles due to perforation of the necrotic gastric wall were removed. The size of the perforation measured 7 × 3 cm affecting the greater curvature on the posterior wall of the stomach ().
A free peritoneal fluid with food particles was washed out from the abdominal cavity. The gastric perforation was treated by debridement of necrotic tissue and a primary closure with additional using an omental patch. The debrided necrotic gastric tissue was submitted to the pathologist for the examination. The pathology assessment of the gastric wall showed a multiple areas of massive, transmural necrosis ().
Post-operatively, the patient was transferred to the pediatric intensive care unit (PICU) on mechanical ventilation and treated with inotropes, cefotaxime, metronidazole, gentamicin, and fluconazole. A fourth day of admission, abdominal cultures taken at the laparotomy as well as a blood culture showed Enterococcus faecalis and Candida glabrata infections.
In the following days, despite an extensive medical support, her clinical condition rapidly deteriorated and she eventually died on day 26 after admission due to overwhelming infections and progressive multisystem failure.
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pmc-6363674-1
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An 8-year-old boy was referred to our hospital for further evaluation of right-sided conductive hearing loss identified at a health check-up at school (). He did not show any other symptoms related to third window syndrome, such as sound-induced dizziness, nausea, autophony or headache (). Serial computed tomography (CT) imaging showed a small soft-tissue density lesion close to the oval window (). A small bone dehiscence within the otic capsule was also indicated in the CT images (). A small cyst (anterior to the oval window) and fixation of the stapes footplate were found during an exploratory tympanotomy (). To investigate the nature of the content fluid of the cyst, we fenestrated the cyst wall. Middle ear lavage fluid (MEL) was taken before and after the opening procedure. CTP concentration in the MEL before fenestration was 0.26 ng/ml (negative), and after fenestration was 2.98 ng/ml (positive), which confirmed the presence of perilymph in the cyst. A small bone dehiscence, considered to be a FAF, was found anterior to the stapes footplate after removal of the cyst (). The small bone dehiscence was sealed with connective tissue and fibrin glue. In the postoperative audiogram, conductive hearing loss improved by 15–20 dB at a low frequency but was still present due to fixation of the footplate (). The conductive hearing loss in this case was caused not only by the cyst but also by another middle ear anomaly: stapes footplate fixation. We plan to perform stapes surgery as the second-stage surgery.
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pmc-6364106-1
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A 38-year-old Saudi female presented in July 2014 complaining of bilateral nasal obstruction for 10 years, for which she sought medical attention and underwent septoplasty and functional endoscopic sinus surgery 7 years ago at a different institute. Postoperatively, the patient noticed minimal improvement in her symptoms with persistence of right nasal obstruction. She also noticed right nasolabial fold fullness for a year, which increased in size over time associated with right facial pain. Clinical examination revealed a swelling in the right nasolabial fold measuring 2 × 1 cm. It was tender to palpation, hard in consistency, but with normal overlying skin. Anterior rhinoscopic examination of the right nasal cavity revealed lateral nasal wall swelling obliterating 90% of the nasal vestibule with normal overlying mucosa and skin colour externally. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a well-defined subcutaneous soft tissue density measuring 1.6 × 1.6 cm located in the right nasolabial fold. The lesion demonstrated isointensity with no drop in fat saturation T1 but showed hypointensity on T2 and homogeneous enhancement postcontrast ().
In August 2014, the patient underwent surgical excision of the mass through a sublabial approach, and the mass was found to be encapsulated with no infiltration to surrounding tissues and was excised completely with its capsule with uncomplicated postoperative period. Histopathological examination of the mass revealed spindle cells proliferation, forming fascicles and whorls on a background of collagen fibres. The fascicles were associated with foci of mixed inflammatory cells infiltrate composed mainly of lymphocytes and plasma cells, along with scattered eosinophils and neutrophils. The lesion infiltrates the surrounding striated muscles and fatty tissue with no infiltration to cutaneous and subcutaneous tissue. Moreover, immunohistochemical studies on the spindle cells revealed they are diffusely positive for vimentin and smooth muscle actin (SMA) () and focally positive for anaplastic lymphoma kinase (ALK). In contrast, they were negative for S100, CD34, P-catenin, CD99, and epithelial membrane antigen (EMA). The patient has been on regular follow-up visits at our clinic, and is now four years with no complaints or tumor recurrence.
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pmc-6364109-1
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A 62-year-old female with no significant past medical history presented to the emergency department in November of 2017 with complaints of arthralgias, most notably in her right knee, left shoulder, and bilateral thighs that made it difficult for her to ambulate. She was also admitted due to a headache that was triggered primarily by coughing. Vital signs on admission were as follows: a blood pressure of 202/90 mmHg, a heart rate of 137 bpm, a respiratory rate of 20, and a temperature of 36.6 Celsius. Physical exam revealed Janeway lesions. She was found to have a neutrophilic leukocytosis, with white blood cell count at 20.4 cells/mm3 and neutrophils at 17.4 bil/L. Troponin was elevated at 1.85; this was deemed to be noncardiac in nature as the patient's pain was relieved with ibuprofen and her EKG showed no acute findings. ESR and CRP were elevated at 95 mm/hr and 24.8 mg/dL, respectively. A computed tomography of the brain showed a high-density mass in the right occipital lobe, with surrounding vasogenic edema. The patient continued to deny any visual changes or symptoms other than what was discussed above. An ophthalmologist was consulted to perform a dilated fundus exam, which was positive for small intraretinal hemorrhages that were deemed to be secondary to the patient's hypertension and less likely positive for Roth's spots. There was no evidence of disc edema. A brain MRI with and without gadolinium showed multiple small punctate bilateral areas of acute or subacute infarctions indicative of embolic phenomenon. The hemorrhagic area in the right occipital lobe was again identified, with subtle surrounding enhancement; the differential diagnosis consisted of neoplasm, vascular malformation, or embolic infarction with hemorrhagic conversion. A transthoracic 2D echo was without vegetation, so a transesophageal echo was ordered, and vegetation was shown on the posterior leaflet of the mitral valve. Two blood cultures from admission then came back positive for Rothia dentocariosa. Infectious disease was confirmed, and the patient's current antibiotics, which consisted of vancomycin and ceftriaxone, were switched to penicillin G on a continuous pump. The patient remained largely asymptomatic during her admission and was deemed to be stable for discharge from the hospital after a nine-day stay with penicillin G via a continuous pump for a total of six weeks and was planned for a follow-up MRI in three weeks. The repeat MRI came back showing new subacute strokes. The patient was reported, again, to be asymptomatic but was directed to come straight to the emergency department. A repeat transesophageal echo was done and showed the known vegetation on the mitral valve with new vegetation seen on the PICC line and an abscess between the mitral and aortic valves extending into the ascending aorta. The patient then requested transfer to another institution for further evaluation. A repeat transesophageal echo was completed at this outside institution which showed small anterior and posterior mitral leaflet vegetation with no significant destruction and no abscess. A cardiac MRI was then performed which showed a focal delayed enhancement in the apical inferior and lateral wall, likely secondary to coronary arterial embolization. The patient went on to complete the full six weeks of penicillin therapy, remained asymptomatic, and refused a mitral valve replacement. Her follow-up was continued in the cardiology clinic.
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pmc-6364110-1
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A 15-year-old boy presented with sudden onset painless diplopia and hyperdeviation of left eye for almost a year, which was stable, painless, and nonprogressive. The binocular diplopia persisted both for near and for distance and was greatest in right gaze and inferior field of view.
There was no history of any associated vision loss, pain, trauma, febrile illness, or any other systemic illness. There was neither any history of weakness after prolonged work or in the evenings nor any past history of strabismus or squinting for far.
There was no history of any weakness, of decreased sensations in body part, of tremors or jerks, of sudden severe pain, of neck stiffness, or of loss of consciousness at the onset of the symptoms. There were no associated neurological symptoms including no other cranial nerve abnormalities. The patient's past medical history and birth history were uneventful. Also there was no history of similar complains among family members.
General physical examination and systemic review were unremarkable.
On ophthalmic examination, the Best Corrected Visual Acuity (BCVA) in both the eyes was 6/6 Snellen's for distance and J6 Jaeger's for near. The patient had a compensatory head posture with the chin at level, face turned towards the right side, and a head tilt to right. Extra ocular movements were full and free in both the eyes with inferior oblique over action in left eye (). On diplopia charting, patient had uncrossed diplopia with tilt and separation maximum in dextrodepression. Park-Bielschowski's three step test suggested a left superior oblique (SO) palsy. On prism bar cover test (PBCT) with prism over the left eye, the deviation was more than 25 PD base-down for both distance and near in all cardinal gazes. Hess charting corroborated the clinical findings. Forced duction test (FDT) and force generation testing (exaggerated FDT) were carried out in an ICU setting and revealed neither any restriction of movement nor laxity of muscles in any gaze.
Revisiting old photographs did not reveal any plagiocephaly or facial asymmetry, thus negating a congenital deviation. Hematological investigations also did not reveal any anaemia, thyroid dysfunction, or any other abnormality.
Ultrasound (USG)-B scan of both the eyes was unremarkable. Gadolinium enhanced MRI of head and orbit was normal, neither any inflammatory lesions in the orbit or the cavernous sinus nor any demyelinating plaques in the periventricular areas (Figures and ). Anti-AchR and anti-MUSK antibodies were negative and single fiber electromyography (SF-EMG) was within normal limits, thus ruling out the possibility of ocular myasthenia. Blood count and thyroid functions were normal. An inflammatory work-up revealed normal ESR and CRP and negative RF, ANA, cANCA, and pCNCA. A provisional diagnosis of “Idiopathic Acquired Superior Oblique Palsy” was made.
The patient was given a trial of oral steroids, i.e., Prednisolone 1 mg/kg body weight. After 2 weeks, the patient was symptomatically better, now with only intermittent diplopia in inferior gaze and decrease in the upward deviation of left eye according to patient's father. PBCT for both near and distance (with prism over the left eye) neutralised the hypertropia with 18 PD base-down over left eye. 4 weeks after starting the oral steroids, the patient's diplopia resolved the left hyperdeviation measured 6PD. After 6 weeks of treatment, the patient remained free of diplopia-hypertropia. Oral steroids were gradually tapered off by 10 mg per week and weekly follow-up was done (). Upon decreasing the dose of prednisolone to 5mg per day, the intermittent diplopia and 18PD left hypertropia reappeared. Oral steroids were restarted at 1 mg/kg body weight and in 2 weeks the diplopia had resolved again and the left hypertropia decreased to 6PD. When the steroid dose was again gradually tapered, intermittent diplopia and 16PD hypertropia reappeared upon reducing the dose to 5 mg per day.
The oral steroid dose was increased back to 10 mg per day and after a week the diplopia disappeared and PBCT neutralised at 6PD base-down in front of left eye.
This unique sequence of events, i.e., disappearance of diplopia-hypertropia at 10 mg OD prednisolone and reappearance at 5 mg OD dosage, led to the final diagnosis of a “Steroid Dependent Isolated Acquired Superior Oblique Palsy”. Presently the patient is maintained on a daily dose of 10 mg oral prednisolone.
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pmc-6364115-1
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A 61-year-old man presented with 2 days of progressively worsening chest pain. Blood pressure was 90/60 mmHg. The 12-lead ECG revealed sinus tachycardia with a rate of 110 bpm and new left bundle branch block. An echocardiogram revealed a left ventricular ejection fraction of 10%, without evidence of mechanical complications. Troponin was 11 ng/mL. In the emergency department, he developed worsening shock and pulmonary edema necessitating mechanical ventilation. He was urgently triaged to the catheterization laboratory.
Femoral angiography revealed no evidence of atherosclerosis and femoral artery diameters of 9 mm. An Impella CP was inserted via the left femoral artery, and coronary angiography/intervention was performed via the right femoral artery. Coronary angiography revealed 70% stenosis of the distal left main coronary artery, chronic total occlusion of the left anterior descending artery, 80% calcific stenosis of the left circumflex, and chronic total occlusion of the right coronary artery (). Invasive hemodynamics revealed refractory cardiogenic shock and biventricular failure (). Right ventricular failure was presumed to be due to collateral insufficiency to the chronically occluded right coronary artery.
Given marginal hemodynamics and the presence of right ventricular failure, an RP Impella was inserted via the right femoral vein. Despite adequate flow from the RP (4.7 L/m) and CP (3.5 L/m), hemodynamics only modestly improved (). Percutaneous revascularization of the culprit severe stenosis in the distal left main and proximal circumflex arteries was challenging but ultimately successful using rotational atherectomy and implantation of a 4.0 × 38 Promus Premiere (Boston Scientific, Marlborough, MA) drug-eluting stent, guided by intravascular ultrasound.
Despite biventricular Impella support using CP and RP catheters and successful revascularization, the patient had persistent cardiogenic shock. This manifested as a markedly reduced cardiac power output (CPO) (). It was elected to escalate left ventricular support using Impella 5.0. Given the large caliber of the femoral arteries and lack of calcification, percutaneous femoral insertion was performed.
Anticipating limb ischemia with large bore sheath insertion, an ex vivo bypass circuit was deemed necessary. With ultrasound guidance, an antegrade 5-French sheath was inserted in the right superficial femoral artery. Next, the existing 6-French sheath in the right femoral artery was replaced with progressively larger sheaths to dilate the arteriotomy. Finally, a 23-French sheath (Abiomed, Danvers, MA) was inserted. Via the 23-French sheath, an Impella 5.0 was inserted into the left ventricle and the existing Impella CP was removed through the left femoral artery. Hemodynamics immediately improved (). Antegrade perfusion of the 14 F left femoral arterial sheath was not undertaken as the caliber of the femoral artery was adequate to accommodate a 4.6 mm sheath. The ex vivo bypass circuit was created by connecting the 14-French left femoral sheath (donor which originally housed the Impella CP) to the 5-French right femoral antegrade sheath (recipient) (Figures and ). Both legs and feet were warm to touch with intact distal pulses.
In the cardiac ICU, Impella 5.0 and RP support was maintained (). Vasopressors were not required. On hospital day 2, the patient developed profound intravascular hemolysis, transient complete heart block, and pulseless electrical activity requiring cardiopulmonary resuscitation. A repeat echocardiogram revealed small LV and RV cavity sizes. Taken together, the findings suggested that the high flow rate from the Impella 5.0 had caused suction of endocardial tissue, leading to hemolysis and increased vagotonia. This improved with volume resuscitation and blood transfusions with reduction in Impella flow rate. On hospital day 3, the Impella RP was explanted uneventfully. The Impella 5.0 was explanted in the OR. Surgical repair of the right common femoral artery was uneventful. Despite confirmation of antegrade flow beyond the femoral artery, fasciotomy of the anterior compartment of the right leg was performed because of elevated compartment pressure and clinical evidence of limb ischemia. There was evidence of hematoma and myonecrosis in the right thigh, which was treated with resection of the affected muscle. The patient slowly recovered and was discharged to inpatient rehabilitation on hospital day 30. Left ventricular ejection fraction improved to 40%. He was discharged home from rehabilitation on hospital day 60.
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pmc-6364118-1
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In a 19-year-old, mentally disabled male, chest radiography was done because of a sudden episode of cough. Metallic, hook-shaped foreign bodies were identified in both the main bronchi.
The right-sided FB was removed by fiber-optic bronchoscopy in the regional hospital, whilst the left-sided extraction failed with the left-sided FB persisting in the left main bronchus ().
Upon urgent admission in a tertiary institution, extraction was attempted by rigid bronchoscope under general anesthesia. Bronchoscopic extraction failed, associated with some bleeding and subcutaneous emphysema immediately after the intervention. The increasing mediastinal and subcutaneous emphysema raised suspicion about an iatrogenic airway lesion, so surgery was indicated. Esophageal injury was previously ruled out by esophagoscopy, revealing many metallic FBs in the stomach. At thoracotomy, a significant mediastinal emphysema (), together with diffuse adhesions, was noticed. After the lung liberation, a proximal 0.5 cm of the noncurved part of the metallic hook was found to protrude through the perforated membranous wall of the left main bronchus, 1 cm away from the descending aorta (, arrow). The part of the FB protruding outside the bronchus was grasped by the clamp and, by following the curved shape of the FB, gentle maneuvers were applied by pulling the sharp end (hook) of the FB in the direction outside the bronchus. The FB was removed from the bronchus (insert on ) without the need for additional bronchotomy. The remaining 10 × 1 mm defect in the bronchial wall, caused both by manipulations during a bronchoscopic extraction attempt and subsequent surgical extraction, was sutured by interrupted PDS 3-0 stitches, and the lung fully inflated. No air leaks appeared during the water test. Having in mind the dimensions of the defect and tensionless suture, no suture-line protection was performed.
After the thoracotomy closure, laparotomy was done and several sharp metallic pieces of different shapes were removed from the stomach (Figures and ). This was followed by an uneventful postoperative course and the discharge after 14 days.
After three years, the patient was urgently readmitted for the new episode of the metallic foreign body aspiration (Figures and ). The abdominal radiography revealed metallic pieces in the digestive tract as well (). With the surgical team on site, rigid bronchoscopy under general anesthesia was done. As the tip of the FB was not impacted in the mucosa, it was possible to grasp it with the rigid biopsy forceps and to withdraw it up to the tip of the bronchoscope. Because of the curved shape of the FB and the impossibility to remove it through the instrument, the FB and bronchoscope were pulled out from the patient together, with the FB firmly grasped, followed by reintubation with the same bronchoscope (Figures and ). After a careful check-up for bleeding and mucosal damage, the patient was extubated.
Metallic pieces left the digestive tract spontaneously after a couple of days.
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pmc-6364119-1
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An 18-month-old female child was brought to the Surgical Outpatient Department with complaints of swelling in right leg. The mass had been present since birth and was slowly progressing in size.
On physical examination, a lump of size 7x3 cm was identified in the right leg shin. The lump was soft, mobile, and nontender. Rest of the systemic examination was normal.
Ultrasonography examination revealed a heterogenous soft tissue mass along the anterior aspect of proximal right leg, suspicious of fibrosarcoma.
Fine needle aspiration cytology was done from the mass and was diagnosed as spindle cell lesion.
The lump was excised and was sent for histopathological examination.
Gross examination showed a partly skin covered, globular capsulated grey-white to grey-brown, firm to hard tissue measuring 6x4x3 cm. On serial sectioning, cut surface was solid, grey-white to grey-brown along with myxoid areas, and interspersed fibrous tissue forming bands.
Microscopic examination revealed a poorly circumscribed lesion comprising three distinct components with vague, irregular, and organoid pattern formed by intersecting trabeculae of fibrous tissue of varying size and shape () along with loosely textured areas consisting of immature small round or stellate cells in a myxoid matrix (). Variable amount of interspersed mature adipose tissue was present at the periphery of the lesion ().
Ancillary test (Immunohistochemistry) was not available. However, special stain showed that mesenchymal matrix was positive for alcian blue stain ().
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pmc-6364120-1
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An 83-year-old man was referred to for assessment of prolonged prothrombin time and cough. His medical history included atrial fibrillation, hypertrophic cardiomyopathy, vasospastic angina, osteoarthritis of the hip, and total hip arthroplasty followed by infection. He presented with mild respiratory distress. His vital signs were as follows: temperature of 36.5°C, irregular pulse of 107 bpm, respiratory rate of 12 per minute, blood pressure of 119/63 mmHg, and oxygen saturation of 89% on room air. He had normal first and second heart sounds, diffuse rhonchi over both lung fields, and purpura on the lateral surface of the left thigh and the medial surface of the right knee. Evidence of other bleeds including petechial, ecchymosis, epistaxis, and gastrointestinal bleeding was not found.
The patient's medications on the day of admission were warfarin (6 mg/day), bisoprolol (2.5 mg/day), ubidecarenone (30 mg/day), benidipine (8 mg/day), nicorandil (10 mg/day), and imidapril (5 mg/day). He had a long-term MRSA infection that had been treated with rifampicin for four years, but this had been discontinued about two months before admission to our hospital. His most recent INR was 3.2 at six weeks before admission. He had no dementia and good compliance with medication.
Laboratory findings on admission were as follows: WBC 5,280/μL, hemoglobin 9.0 g/dL, hematocrit 27.4%, platelets 145,000/μL, INR 11.89, PT 146.6 s, APTT 99.6 s, D-dimer 1.1 μg/mL, random glucose 102 mg/dL, serum sodium 139 mEq/L, serum potassium 4.0 mEq/L, and serum creatinine 0.96 mg/dL. Urinalysis showed macroscopic hematuria. All other laboratory findings including liver function were unremarkable. Serum C-ANCA and P-ANCA were negative. Arterial blood gas analysis indicated a pH of 7.426, partial pressure of carbon dioxide in arterial blood (PaCO2) 37.9 mmHg, partial pressure of arterial oxygen (PaO2) 76.7 mmHg, and bicarbonate (HCO3−) 24.5 mmol/L with 28% oxygen inhalation via a nasal cannula.
Chest radiography revealed alveolar opacity in both lungs, especially in the upper and middle lobes (). High-resolution computed tomography of the chest confirmed reticular opacity with bronchial wall thickening and interlobular septal thickening in both lungs (). Bronchoalveolar lavage samples indicated alveolar hemorrhage. He had no history or recurrence of urothelial infection and no evidence of urothelial malignancy.
The high INR (11.89) indicated that the main cause of the diffuse alveolar hemorrhage and macroscopic hematuria was excessive warfarin anticoagulant activity. Therefore, warfarin was discontinued on the day of admission, and vitamin K 10 mg, carbazochrome sodium sulfonate 50 mg, tranexamic acid 1 g, and four units of fresh frozen plasma (FFP) were administered. The INR decreased to 1.46 on the following day, and oxygen saturation in room air gradually improved from 89% to 93% after admission. Chest radiography on days 6 and 12 revealed decreased ground glass opacity, and high-resolution computed tomography showed improvements in the lung fields. The patient was discharged from hospital on day 13.
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pmc-6364124-1
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A 54-year-old male with a history of renal cell carcinoma, pancreatic adenocarcinoma stage II (T2 N1 3/5 lymph nodes positive), having previously received chemotherapy followed by stereotactic body radiation therapy (SBRT) presented 5 months after the Whipple's surgery with failure to thrive, fatigue, and nausea. Exam was unremarkable and laboratory investigations revealed albumin of 1.3 mg /dl, bilirubin of 2.8 mg/dl, mainly conjugated, serum alkaline phosphatase of 825 U/L, and CA 19-9 of 81.4 (normal <37 U/ml). Computed tomography scan (CT) of abdomen showed a perigastric abscess adjacent to the fundus (). Endoscopic ultrasound (EUS) was suggestive of 35 mm anechoic, heterogeneous, well-circumscribed fluid collection in the immediate perigastric area surrounding the fundus (). Under endosonographic, fluoroscopic, and Doppler guidance, a 10 x 10 mm LAMS was placed from the stomach into the fluid collection with drainage of pus.
The patient improved clinically along with significant improvement in his bilirubin and alkaline phosphatase after the procedure. Repeat CT abdomen after one week of stent placement showed a near-complete resolution of the abscess (), although he had developed ascites by this time, likely due to presence of severe hypoalbuminemia. Removal of the stent was planned after 3 weeks of placement. However, the patient was readmitted 3 weeks later with respiratory failure and altered mental status. His family elected to provide supportive care only and he died shortly thereafter.
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pmc-6364124-2
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54-year-old male underwent distal pancreatectomy with splenectomy for treatment of pancreatic neuroendocrine tumor. In the immediate postoperative period, patient developed a pancreatic fluid leak from the tail of pancreas and an intra-abdominal drain was placed that was removed after it stopped draining. CT scan showed interval increase in the size of the rim enhancing fluid collection around tail of pancreas 1 month after drain removal (). ERCP was performed for suspected pancreatic duct (PD) leak and confirmed a leak from the tail of the pancreas. Endoscopic pancreatic sphincterotomy was done with placement of a 5 Fr x 13 cm pancreatic duct stent with internal barbs. Subsequently, EUS showed a well demarcated, hypoechoic, heterogeneous collection, adjacent to the tail of the pancreas about 6.5 cm in the largest dimension (). Under endosonographic guidance, a 15 mm x 10 mm LAMS was placed from the stomach into the fluid collection with drainage of a large amount of pus. CT scan of abdomen after 1 month showed decrease in the size of the previously demonstrated LUQ rim-enhancing fluid collection (). Unfortunately, patient later had a neurological event that led to his demise, prior to stent removal.
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pmc-6364124-3
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A 34-year-old male presented to hospital after a motor vehicle accident. Patient was hypotensive on arrival and underwent exploratory laparotomy, splenectomy, embolization of hepatic vessels, and right-sided partial hepatectomy due to grade V liver laceration. Postoperatively, the patient developed bilious drainage from an intra-abdominal drain and underwent ERCP for suspected bile leak. ERCP revealed a leak from the right biliary system; therefore a 10 Fr x 5 cm plastic biliary stent was placed. A week later, the patient continued to have high output of amylase-rich fluid from a separate intra-abdominal drain which was suspicious for a pancreatic duct leak. Repeat ERCP with pancreatogram revealed a leak from the pancreatic tail for which a 5 Fr x 13 cm pancreatic duct stent was placed and the biliary stent was upsized to a 10 mm x 4 cm covered self-expanding metallic stent (SEMS) due to a persistent biliary leak.
After 1 month, the patient became septic and was found to have a peri-pancreatic abscess on CT abdomen. IR performed a percutaneous drainage of the peripancreatic abscess with minimal drainage through the drain, without any clinical improvement. EUS was then performed by one of the authors (JN) that revealed a 55 mm, oval, heterogeneous peri-pancreatic fluid collection which had hyperechoic material consistent with the solid debris. A 10mm x 10mm LAMS was then placed from the stomach into the fluid collection with subsequent drainage of pus and debris. The patient clinically improved and was discharged home with improvement of the fluid collection. A follow-up CT Abdomen 4 weeks later showed interval decrease in the size of the previous small fluid collection. The LAMS was uneventfully removed endoscopically at 8 weeks after the initial placement.
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pmc-6364128-1
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A hypertensive and diabetic 78-year-old male presented with decreased left visual acuity. There was a history of intravitreal Ozurdex implantation approximately 85 days ago for upper temporal RVO and CME. The best-corrected visual acuity (BCVA) was 0.16 on the Snellen chart and the IOP was 16 mmHg. Slit lamp examination revealed a Grade II nuclear cataract without anterior chamber inflammation and there was an intralenticular dexamethasone implant in the upper part of the lens (). Fundus examination revealed findings secondary to upper temporal RVO. Scheimpflug photograph of the left eye showed the intralenticular Ozurdex implant with an intact posterior capsule (). Comparison of OCT images of the macular edema prior to Ozurdex injection (central macular thickness (CMT): 565 μm) (Figures and ) and 85 days afterwards (CMT: 290 μm) demonstrated resolution of the edema (Figures and ).
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pmc-6364276-1
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A 12-year-old female patient was referred to the Genetic Counseling Service of the
University of São Paulo on account of short stature (136.5 cm, 3rd
percentile), delayed bone age and skeletal anomalies including hypoplastic scapulae,
thoracolumbar scoliosis, 11 pairs of ribs with hypoplasia of the first four pairs.
Her intellectual development was normal. Chromosome analysis after G-banding
revealed a balanced reciprocal translocation between the short arm of chromosome 7
and the long arm of chromosome 17, 46,XX,t(7;17) (p13;q24). At 31 years of age, her
height (159 cm) and weight (54 kg) were around the 25th centile and she
returned for genetic counseling to assess the risk of having affected offspring
().
At 37 years of age, the patient was referred to our clinic, the Monteleone Center
for Human Reproduction, São Paulo - Brazil, wishing to undergo in
vitro fertilization with PGT to avoid the risk of having affected
children.
The patient underwent IVF + PGT-SR after signing an informed consent term. She
underwent the first IVF cycle in May 2016. The patient was given recombinant FSH for
ovarian stimulation; GnRH antagonist for pituitary blockage; and final oocyte
maturation was triggered with recombinant hCG. Fourteen oocytes were harvested, 12
of which were mature (MII) and two at the germinal vesicle stage (GV). All MII
oocytes were fertilized by ICSI using ejaculated sperm from her partner and cultured
in standard conditions. Two embryos reached the blastocyst stage and were biopsied
on day 5 of development for PGT-SR analysis. PGT was carried out at a reference
laboratory by comparative genomic hybridization array (CGHa) for 24-chromosome
analysis (Igenomix, Brazil) using standardized procedures. The results of blastocyst
genetic analysis revealed anomalies: blastocyst 1 was a male presenting an
unbalanced translocation and whole chromosome aneuploidies (-7p, +9, -17, XY) and
blastocyst 2 was a female presenting only whole chromosome aneuploidies (+7, -11,
XX) ( and ).
The patient underwent a second ovarian stimulation cycle in July 2016, using the same
protocol as before and 13 oocytes were collected: 11 MII, 1 MI and 1 degenerated
oocyte. Twelve oocytes were fertilized by ICSI and six blastocysts were biopsied on
day 5 of development. PGT-SR analysis was carried out in the same reference
laboratory by Next Generation Sequencing (NGS) for 24-chromosome analysis (Igenomix,
Brazil). All embryos presented abnormalities ( and ) and none was
transferred. The patient was advised to start a new IVF cycle using donated
oocytes.
In March 2017, the couple decided to undergo a cycle using donor oocytes on a shared
oocyte donation protocol. The patient received eight donor oocytes after endometrial
preparation with estradiol and progesterone. Seven oocytes fertilized by ICSI with
partner sperm developed until day 5. Two blastocysts were transferred and five were
cryopreserved. bHCG levels measured 9 and 11 days after transfer were 76 IU and 204
IU, respectively. Ultrasound examination four weeks after embryo transfer showed a
gestational sac (). Ultrasound
examination eight weeks after embryo transfer showed a gestational sac without fetal
heartbeat.
In October 2017, the patient underwent a frozen-thawed embryo transfer with embryos
cryopreserved in the previous oocyte donation cycle. After endometrial preparation,
she had one top-quality blastocyst (grade 6) transferred. Eight days after the
transfer the patient had a bHCG level of 250 IU. Ultrasound examination six weeks
after the transfer procedure revealed a gestational sac with fetal heartbeat (). Ultrasound examination eight weeks
after embryo transfer showed a gestational sac without fetal heartbeat.
|
pmc-6364283-1
|
A 32 year-old woman with a ten-year history of primary infertility came to our unit
for IVF/ICSI with the diagnosis of bilateral tubal block and uncorrectable tubal
damage, without hydrosalpinges, and a normal semen profile for her husband. She had
a past history of open myomectomy and two laparoscopies for endometriosis treatment
(one of them involved Laparoscopic ovarian drilling). She had a previous IVF attempt
at another IVF/ICSI clinic, which ended up as an empty follicle syndrome (EFS) and
cycle cancelation. In that trial she was submitted to a standard long agonist
protocol with highly purified urinary FSH and triggered with 10.000 IU of hCG. After
failure to retrieve any oocytes from one ovary she received an additional dose of
10.000 hCF IU and egg collection was rescheduled 24 hours later. Unfortunately, the
second trial ended with no eggs being retrieved.
In the second trial (first at our unit), the basal hormonal profile showed: FSH = 6.5
miu/ml, LH = 4.4 miu/ml and AMH = 4.05 ng/ml. We used a fixed antagonist protocol,
using Cetrorelix (Cetrotide, Merck Serono, London, UK) and HMG (Menogon, Ferring,
Kiel, Germany) 300 IU for 12 days. Dual trigger was done using 10000 IU HCG
(Choriomon, IBSA, Lugano, Suisse) and 0.2 mg triptoreline (Decapetyl, Ferring, Kiel,
Germany) and OPU was scheduled 36 days thereafter. On triggering day, her
transvaginal ultrasound scan showed seven follicles between 17-20 mm. HCG and
Decapeptyl (for triggering) were given by a qualified nurse at the correct time.
Before OPU, a blood sample was withdrawn which showed E2 to be 3510 pg/ml and B-HCG
= 166.3 miu/ml. It ended with the retrieval of one immature oocyte (Germinal vesicle
GV) after thorough repeated flushing and aspiration of all follicles. In
vitro maturation (IVM) was tried for this immature oocyte. However, it
degenerated on the following day.
In the third trial (second at our unit), she was thoroughly counseled before starting
treatment, when we explained that there was a great possibility of the same thing
happening again. She and her husband consented for the third trial. Again, we used a
fixed antagonist protocol with Cetrorelix (Cetrotide, Merck Serono, London, UK) and
another highly purified HMG (Merional, IBSA, Lugano, Suisse) at a dose of 300 IU per
day for 11 days. On the day of triggering, her transvaginal ultrasound scan showed
eight follicles between 17-20 mm. Her E2 level before triggering was 1594 pg/ml.
Dual trigger was used again, with recombinant hCG (Ovitrelle, Merck, London, UK ),
0.2 mg triptoreline (Decapeptyl, Ferring, Kiel, Germany) and her OPU was carried out
36 hours after triggering. Again, HCG and Decapeptyl (for triggering) were given by
a qualified nurse at the correct time. The ß-HCG level was 122.1miu/ml before
egg collection and her E2 was 1049 pg/ml. Her egg collection resulted in the
retrieval of 2 poor quality immature germinal vesicles despite thorough, repeated
flushing and aspiration (). Because of
her past history of EFS, both her oocyte retrievals at our unit were performed by
our most experienced physician. IVM was tried, again, for her two retrieved immature
oocytes. Unfortunately, none of them got mature until day five. The patient and her
husband gave their informed consent to use their information and photos of their
immature eggs for publication as they are quite eager to find a solution for their
problem
|
pmc-6364323-1
|
A 60-year-old man with a past history of RCC (clear cell type, G2, T1b N0 M0 Stage I) treated by a right nephrectomy in June 2015 was required to have a follow-up examination at 6-month intervals after surgery, without the use of an anticancer agent. In January 2018, a routine gastrointestinal endoscopy found an ulcerative lesion of approximately 10 mm diameter in the greater curvature of the gastric body (Fig. ). An endoscopic ultrasonography (EUS) of this lesion showed the first three sonographic layers were blurred, which suggested submucosal invasion. An endoscopic biopsy of the lesion exhibited clear cytoplasm with prominent nucleoli, which was histologically compatible with metastasis to the stomach of the patient’s known RCC. On the other hand, computed tomography (CT) incidentally detected a well contrast-enhancing round-shaped mass in the fundus of the gallbladder (Fig. ). Additional ultrasonography revealed a sessile polypoid lesion, and gallbladder stone and wall thickening were not observed. Although these findings were lacking conclusive evidence of diagnosis whether the gallbladder tumor was primary or metastatic, the circumstantial evidence potentially pointed to the tumor as a metastasis from the patient’s known RCC. 18F-Fluoro-deoxyglucose positron emission tomography combined with CT (FDG-PET/CT) was performed as a preoperative workup to detect other possible remote metastasis. However, specific FDG uptake was not shown, even in the gastric and gallbladder tumors. The blood examination was unremarkable.
In February 2018, a gastric wedge resection via laparoscopic and endoscopic cooperative surgery (LECS) technique was applied to the gastric tumor, and laparoscopic cholecystectomy to the gallbladder tumor was simultaneously performed (Fig. ). The operation lasted 190 min with little intraoperative blood loss. Intraoperative pathologic diagnosis was not performed in this case. The hospitalization period after surgery was not eventful, and the patient was discharged on postoperative day 7. Histological examination confirmed that the tumors of the stomach and gallbladder were both metastatic RCC. Immunohistochemical staining was strongly positive for CAM 5.2 and vimentin, supporting the diagnosis. Macro- and microscopic findings are shown in Fig. . Thereafter, the patient required examination every 3 months without the use of anticancer agents and has survived without relapse to 12 months after the surgery.
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pmc-6364325-1
|
A 46-year-old man complained of heartburn, abdominal distention, and anorexia which persisted for over 2 weeks. He had no significant personal and family history. Body temperature on admission was 39.2 °C. Abdominal physical examination revealed large abdominal masses without tenderness or rebound. Blood examinations showed increased inflammation reaction (white blood cell count, 29,700/μL; C-reactive protein, 33.30 mg/dL). Renal dysfunction was also recognized (Table ). Abdominal computed tomography revealed two large inhomogeneous masses with a diameter of 230 and 146 mm, respectively. The upper part of the intra-abdominal tumor contained liquid and air, which indicated abscess formation (Fig. a–c). As the continuity to the gastrointestinal tract was inexplicit, a mesenchymal tumor was mostly suspected. The tumor compressed the right ureter, which caused right hydronephrosis (Fig. b). On the contrary, the left-sided tumor was solid, and it was thought to be derived from the left kidney without left hydronephrosis. Plane abdominal magnetic resonance imaging, T2-weighed imaging (T2WI), and fat saturation T2WI showed mainly high intensity with some low-intensity area (Fig. ). There is also high intensity in diffusion-weighted imaging and low apparent diffusion coefficient values at solid areas of both masses.
We thought that the intra-abdominal tumor had formed abscess and caused bacterial infection. We decided to resect the right intra-abdominal tumor first to stabilize the general condition from a systemic inflammatory state. After the recovery of renal function, the left retroperitoneal tumor resection was scheduled.
In the first surgery, we inserted a ureteral stent to the right ureter and successfully performed resection of the right tumor without invasion to the right ureter. However, the tumor appeared to invade the small intestine, and combined resection was performed (Fig. a). The operation time was 274 min, and the total blood loss was 3500 ml. We infused 14 units of red blood cells and 16 units of fresh frozen plasma transfusion. The tumor had smooth surface, 26 × 23 cm in size, and weighed 3.9 kg (Fig. b, c). After the first operation, the inflammation reaction values and renal function returned to normal, and he was discharged on postoperative day (POD) 17. On POD 41, the second surgery was performed to remove the retroperitoneal tumor under the left kidney (Fig. ). Via the same incision as the first surgery, we reached the retroperitoneal tumor transperitoneally. The retroperitoneal tumor was in contact with but not derived from the left kidney, and complete tumor resection was performed without left nephrectomy. The operation time was 224 min, and the total blood loss was 640 ml without any transfusion. Macroscopic findings of the retroperitoneal tumor were similar to those of the right intra-abdominal tumor, and it weighed 1.4 kg (Fig. ). Histopathological examination of the intra-abdominal tumor on the right side revealed various sizes of spindle-shaped or round-shaped cells in the myxoid parenchyma which were surrounded by fibrous capsule, with hyperplasia of the capillary or microvascular vessel, collagen fibers, and adipocyte cells (Fig. a–c). Alcian blue staining for mucinous component was positive in most areas (Fig. d). The tumor cells were immunohistochemically positive for S100, MDM2, Rb1, and bcl2, but devoid of CD34, smooth muscle actin, and desmin (Fig. e–g). No invasion to the small intestine was seen, and surgical margin was free (Fig. a). The characteristics of the retroperitoneal tumor were almost similar to those of the intra-abdominal tumor which suggested that both tumors had the same differentiation status. (Fig. a–f). Finally, according to these findings, he was diagnosed with multicentric myxoid and round cell liposarcoma. After the second operation, he was discharged without any complications on POD 14 and received adjuvant triweekly chemotherapy with doxorubicin (30 mg/m2) and ifosfamide (2 g/m2) for eight courses for 6 months. He has been closely monitored and recurrence-free for 9 months after the first surgery.
Liposarcoma is the most frequent soft-tissue sarcomas. It is usually seen in retroperitoneal (45%), extremities (24%), inguinal, gluteal, and popliteal regions, but it is rarely seen in the intra-abdominal space []. Patients with liposarcoma are usually asymptomatic when the tumor is still small; therefore, the tumor is often found after it becomes bigger []. Liposarcoma is histologically classified into five categories, namely, well-differentiated (45%), differentiated (20%), pleomorphic (10%), round cell (5%), and myxoid type (rare). Most giant liposarcomas are usually seen as differentiated tumors [, ]. Myxoid and round cell tumors share the same characteristic cytogenic abnormalities: the translocation of t(12;16)(q13;p11) leading to the genes’ fusion DDIT3 and FUS with generation of a hybrid protein FUS/DDIT3 []. Essentially, round cells are frequently found in myxoid liposarcoma, which is considered to be the marker of poor prognosis when presenting 5% or more of the mass in localized myxoid liposarcoma []. The prognosis differs depending on these subtypes as follows: the 5-year survival rate is 95% in well-differentiated type, 92% in mucinous type, 74% in round-cell type, and 59% in pleomorphic type [].
Liposarcoma is usually a single tumor. When multiple tumors are seen, one of the main problems is to differentiate multicentric lesions from metastases of a single tumor. Multicentric liposarcoma accounts for only 1–2% of all liposarcomas in the USA and Europe []. There are no diagnostic criteria, but some features of multicentric liposarcoma are indicated []: Primary liposarcomas commonly occur in areas such as the thigh, popliteal fossa, retroperitoneum, peritoneal cavity (including the mesentery and greater omentum), upper extremities, and thoracic serosa []. There is no occurrence in sites where metastases are usually found, such as the lungs, liver, and skeletal system []. Histopathological type of the tumors is well differentiated or myxoid patterns, which rarely metastasize []. There is a long time interval between the occurrence of the first and subsequent tumors []. In most cases, it is difficult to differentiate the multicentric liposarcoma from metastatic liposarcoma and there are no criteria. In our case, both liposarcomas had myxoid/round cell types in the common sites and were considered multicentric.
The treatment for liposarcoma has not been established, but en bloc resection is recommended not only for treatment but for pathological diagnosis []. Our case developed renal dysfunction due to compression of the right ureter by the intra-abdominal tumor. If we tried to remove both tumors simultaneously, and if the retroperitoneal tumor was derived from the left kidney, composite resection with the kidney was unsafe, as it might affect kidney function. Thus, we decided to first resect the intra-abdominal tumor which caused abdominal abscess.
In this case, multicentric tumors were composed of multiple compartments. Therefore, we thought that his prognosis would be poor and he should undergo chemotherapy in the adjuvant settings. The effectiveness of adjuvant chemotherapy remains unproven, and proper regimen has not been established.
Tumor size (> 20 cm), histological subtypes, and tumor dissemination are considered important prognostic factors []. In this case, both tumors were myxoid/round cell types and found in two different compartments. The patient was thought to be at risk for recurrence and received adjuvant chemotherapy, but radiation therapy was not initiated because of the wide range of tumor beds. Myxoid/round cell liposarcoma is a chemosensitive soft tissue tumor, and adjuvant chemotherapy with doxorubicin is recommended []. A meta-analysis showed that the effect of the doxorubicin-based chemotherapy improved relapse-free survival, but not overall survival []. In this case, he underwent triweekly chemotherapy with doxorubicin (30 mg/m2) and ifosfamide (2 g/m2) for five cycles, and there has been no recurrence for 9 months. In our case of multicentric myxoid/round cell liposarcoma, en bloc surgical resection and adjuvant chemotherapy were effective.
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pmc-6364428-1
|
A 56-year-old man complained of passing proglottids in his stool intermittently over the last two months. No abdominal symptoms, such as nausea, vomiting, abdominal pain, diarrhea, or constipation were present. He reported frequent consumption of raw beef and fish (both marine and freshwater fish), and had no history of traveling abroad. He had previously obtained 400 mg of albendazole from the pharmacy and taken it once orally without clinical improvement. After that, he was prescribed 600 mg of praziquantel at a local clinic and had taken it once orally as well. He brought his stool sample, which included the passed segments to our hospital (Fig. a). The segments were pressed between two microscope slides and examined macroscopically without staining. We could not observe gravid proglottids, which contain fully developed uteri filled with ova, or branched uterine structures. To identify the tapeworm species, we conducted molecular analysis using the proglottid segments. Genomic DNA was extracted using the QIAamp DNA Mini Kit (Qiagen, Hilden, Germany) and subsequently used as a template for polymerase chain reaction (PCR). The mitochondrial cytochrome c oxidase subunit I (cox1) gene and partial sequences of elongation factor-1 alpha (ef1a) were targeted for PCR amplification. The sequences of the PCR primers used were: T1F (5’-ATATTTACT TTAGATCATAAGCGG-3′) and T1R (5’-ACGAGAAAATATATTAGTCATAAA-3′) for cox1, and Tae_ef1/F4 (5’-TGTGGTGGAATCGATAAAAGG-3′) and Tae_ef1/R4 (5’-TCGATCTCATGTCACGAACG -3′) for ef1a [, ]. PCR was carried out using a 30 μL reaction mixture containing 15 μL Smart 2 × PCR Pre-Mix (SolGent Co., Ltd., Daejeon, Korea), 2 μL template DNA, 10 μM of each primer, and 11 μL distilled water, as described in a previous study []. The amplification process comprised 35 cycles of denaturation (94 °C for 30 s), annealing (60 °C for 30 s), and extension (72 °C for 80–90 s). The PCR products were sent to Macrogen (Korea) for direct sequencing using the same PCR primers. The 480-bp cox1 sequence had a 100% match with the T. saginata sequence and a 94.8% match with the T. asiatica sequence. The 1078-bp ef1a sequence also had a 100% match with the sequence of T. saginata, a 99.3% match with the sequence of T. asiatica and a 95.3% match with the sequence of T. solium. The three Taenia species that infect humans had nucleotide differences at 73 and 57 polymorphic sites for the cox1 and ef1a sequences, respectively (Additional file ). Of these polymorphic sites, three sites (nucleotides 294, 336, 405) in cox1 differentiated the three species. DNA sequences were aligned using the CLUSTAL W computer program []. Phylogenetic trees were constructed using the neighbor joining method [] and genetic distances were computed using the Tamura-Nei method using the Geneious computer program (Fig. b and c). The neighbor joining tree used GenBank sequences derived from samples collected in Asia, and indicated that our specimen was in the same phylogenetic group as T. saginata, but not in the same group as T. asiatica or T. solium. In addition to molecular analysis, we also examined the patient’s stool specimen using the formalin-ether concentration method. However, we could not observe any Taenia species ova on the 10 slides examined. Ova were not observed inside the proglottids either, indicating that these proglottids were immature. Instead, C. sinensis ova were observed on one slide (Fig. d). Conversely, the level of serum IgG specific to C. sinensis measured using Enzyme-Linked Immunosorbent Assay (ELISA) was within the normal range. He was treated with praziquantel at the recommended dose of 25 mg/kg three times daily for 3 days []. After treatment, no proglottids or ova of C. sinensis were found in his stool.
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pmc-6364459-1
|
A white Caucasian 76-year-old woman visited our tertiary referral center with the complaint of decreased vision in both eyes. Clinically bilateral corneal guttae were evident with corneal bullae on her right eye (OD). She was diagnosed as having bilateral FECD subjectively worse on her OD and a DMEK was advised. Her preoperative visual acuity was 20/40 OD and left eye (OS).
After staining the donor endothelium with trypan blue 0.06% for 30 seconds, an 8.0-mm graft was dissected using the forceps’ technique according to Melles immediately prior to surgery.
After standard cataract extraction with a 2.75-mm limbal tunnel incision and two 1-mm incisions at 10 and 2 o’clock, viscoelastic was removed by extensive irrigation/aspiration. The descemetorhexis was performed under air using a price hook (Moria S.A. plc, 92160 Antony, France) and the diseased tissue removed with a stromal scraper.
A standard no-touch technique was applied to keep iatrogenic endothelial trauma to a minimum. The stained DMEK graft was inserted into the anterior chamber using a custom-made glass injector, oriented and adhered onto the recipient’s stroma using air pressurization.
Postoperatively the graft was attached, no further intervention was needed, and no immunological reactions were noted. A standard postoperative regimen was followed (moxifloxacin eye drops four times a day for 2 weeks and prednisolone eye drops four times a day with slow tapering). Her postoperative visual acuity was 20/50 with significant subjective improvement (uncorrected with persistent stromal haze).
After 18 months she returned with decreased vision and an allograft rejection. During the acute episode a pronounced, conjunctival injection, corneal edema, and neovascularizations were prominent. Superficial and deep neovascularizations beyond the 8.0-mm-descemetothexis were observed. The cornea itself had signs of a non-functioning graft with increased corneal thickness, extensive edema, and endothelial cell attenuation on specular microscopy. In addition, a stromal haze and retrocorneal membranous structures were visualized on slit-lamp microscopy (Fig. a–d).
Although local steroids were intensified, the retrocorneal membranes persisted and the graft eventually failed completely. The retrocorneal structures were thin, mesh-like, and whitish in color. A penetrating keratoplasty was advised and the removed tissue sent for histopathological evaluation. On morphological examination, the retrocorneal membranes had an undulating character with an adjacent bare DM with no to very scarce endothelial remnants.
The histopathological report stated an endothelial insufficiency secondary to a retrocorneal fibrous membrane and deep neovascularizations secondary to an allograft rejection (Fig. ).
From the posterior stroma a thin membrane of connective tissue/corneal stroma had grown on the back of the lamellar graft. This membrane continued to the right so that more than 50% of the graft was covered, eventually leading to endothelial decompensation. On histological examination, it was an “ordinary retrocorneal membrane,” as is often observed after penetrating keratoplasty. After DMEK, however, such a membrane has not previously been described histologically (Fig. ).
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pmc-6364949-1
|
A 73-year-old female presented with a new 3 cm mass in the pancreatic head that was found on an annual surveillance computerized tomography (CT) scan eight years after a radical left nephrectomy for renal cell carcinoma (RCC). Her initial nephrectomy was for an 8 cm clear cell RCC that had one positive periaortic lymph node (LN). The patient had no further treatment and denied abdominal pain but reported progressive weight loss (>60 lbs) over the last several months. She was not jaundiced and there was no evidence of duodenal or biliary duct obstruction on the CT scan. Other diagnostic modalities (endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance imaging (MRI)) confirmed the pancreatic head mass. A bone scan and a chest X-ray were negative for metastatic disease. The patient underwent a diagnostic laparoscopy with pelvic washing and biopsies from peripancreatic tissue, celiac, splenic and periportal lymph nodes. There was no evidence of peritoneal, omental or hepatic spread and all biopsies were negative for malignancy. A pancreaticoduodenectomy (Whipple procedure) was performed for presumptive pancreatic cancer. Section through the specimen showed multiple solid yellowish necrotic and hemorrhagic areas (0.5 - 2.2 cm). The histologic exam was consistent with metastatic clear RCC (Figure ), two of nine peripancreatic LN were positive for metastases. There was no neoplastic thrombus in the pancreatic duct and the margins were free from disease. Immunohistochemical stain showed tumor focally positive for cytokeratin 7 and keratin AE1/AE3 but negative for cytokeratin 20 and carcinoembryonic antigen. The tumor stained strongly positive with vimentin; all consistent with RCC. The pathology was identical to the slides from initial nephrectomy. Four months later, a gradually enlarging right lobe thyroid nodule was noticed associated with dysphagia. The nodule was cold on radionuclide scanning and solid on ultrasound, measuring 2.9 x 2.6 x 2 cm. Doppler showed marked increase in vascular flow. A CT scan of the neck, chest, and abdomen failed to demonstrate other lesions. Fine needle aspiration cytology (FNAC) was suggestive of metastatic RCC. A right hemithyroidectomy was performed (the left lobe was removed years ago for benign disease). Section through the tumor revealed a solid, homogenous, bulky yellowish nodular neoplasm. The histologic exam was consistent with metastatic RCC (Figure ). The patient tolerated both procedures and has done well.
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pmc-6364951-1
|
An 84-year-old woman, known to have atrial fibrillation and hypertension, presented with impaired memory and altered mental status. On physical examination, no lymphadenopathy or organomegaly was detected. A neurological examination revealed mild dysmetria in the left upper extremity. Her white cell count was 25,100 × 1012/l, with 61% lymphocytes. Magnetic resonance imaging (MRI) of the brain revealed a homogeneously enhancing cerebellar mass causing mass effect on the tectum and obstruction at the level of the aqueduct associated with the hydrocephalus (Figures -).
The patient underwent a bilateral posterior fossa craniotomy and tumor resection, followed by the insertion of an external ventricular drain. Intraoperatively, the mass was thought to be intra-parenchymal. After surgery, she recovered well, with no new neurological deficits. Histopathology revealed an infiltrating and highly mitotic neoplasm composed of malignant lymphoid cells (Figures -). In situ hybridization (ISH) for Epstein–Barr virus (EBV) was negative. Fluorescence in situ hybridization (FISH) demonstrated no gene rearrangements in B-cell lymphoma 2 (BCL2), (BCL6), and MYC. Polymerase chain reaction (PCR) amplification and capillary gel electrophoresis per the BIOMED-2 protocol were performed on paraffin-embedded tissue, revealing a peak in the immunoglobulin heavy chain (IGH) consistent with a clonal process. The findings were diagnostic of an EBV negative, diffuse large B cell lymphoma (DLBCL).
A bone marrow core biopsy was performed due to low white blood cell (WBC) count, which revealed the involvement of a low-grade lymphoid process (Figures -). Flow cytometric immunophenotyping revealed a Lambda-monotypic, CD5 negative B cell population expressing CD19, CD23, CD22 (dim), and partial CD20 while lacking CD10 and FMC7. Aside from the lack of CD5 expression, the morphologic and immunophenotypic findings were consistent with chronic lymphocytic leukemia/small cell lymphoma. A diagnosis of small B cell lymphoma was rendered, with an offered differential diagnosis including CLL, marginal zone lymphoma, mantle cell lymphoma (CD5 negative), follicular lymphoma (CD10 negative), and lymphoplasmacytic lymphoma. Although the lack of CD5 expression makes mantle cell lymphoma a more reasonable differential, multiple studies have reported cases of CD5 negative CLL [-], with an incidence ranging from 7% to 20% among all CLL cases []. Hence no further investigations to rule out mantle cell lymphoma were required.
As mentioned above, molecular studies performed on the paraffin-embedded tissue from the brain biopsy revealed an immunoglobulin heavy chain (IGH) rearrangement consistent with a clonal process. We endeavored to perform a similar analysis on a sample from the patient's bone marrow biopsy, as the presence of an identical gene rearrangement would have provided support for a relationship between the two neoplasms. However, per standard protocols, the bone marrow core biopsy was acid decalcified for next day processing, compromising the integrity of the DNA necessary for the PCR. Therefore, performing an IGH rearrangement studies on the bone marrow biopsy was not a viable option, and a definitive genetic link between the two neoplastic processes could not be established.
The positron emission tomography/computed tomography (PET/CT) scan showed no other areas of hypermetabolic involvement. Given her advanced age, she might not have tolerated the toxic effect of methotrexate. Therefore, she received one cycle of temozolomide and whole-brain radiation therapy (WBRT). A follow-up CT scan at six months showed no residual tumor. Approximately 10 months following her initial diagnosis, she had a recurrence in the posterior fossa, which was confirmed by MRI. She passed away within one month of recurrence.
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pmc-6365385-1
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This case involves a 66-year-old gentleman with hypertension. He presented with elevated Cancer Antigen 19-9 levels of 128 μ/m incidentally detected on routine screening. Liver function test and other tumor markers (carcinoembryonic antigen, alpha-fetoprotein, cancer antigen 125) were within normal limits.
Magnetic resonance cholangiopancreatogram (MRCP) revealed a lobulated, ill-defined endoluminal soft tissue mass measuring 1.7 × 2.0 cm abutting the lateral wall of the junction between the first (D1) and second (D2) part of the duodenum, but not invading into mucosa. Anatomical pancreatic tissue had no ductal dilatation, and was similar in appearance and consistency to the identified mass (). Differentials at this point included heterotopic pancreas, and other mucosal/submucosal malignancies such as gastrointestinal stromal tumor (GIST). He was further investigated with endoscopic ultrasound (EUS). The D1/D2 junction intramural (submucosal) lesion was identified and measured 1.8 cm × 0.9 cm. It had lobulated margins, acinar cells and an anechoic 0.2 cm central duct-like structure, all suggestive of heterotopic pancreas (). Fine needle aspiration (FNA) revealed streaks of acinar cells of pancreatic morphology and ducts with intervening connective tissue, diagnostic of heterotopic pancreas ().
Given that this patient was asymptomatic with no malignant features seen on investigations, he was managed conservatively. He has since been followed-up in clinic once at an 8-month interval with a repeat CTAP showing a stable mass and no enlargement or invasion.
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pmc-6365392-1
|
A 65-year-old man was referred in April 2013 with an echogenic segment IVa lesion found incidentally on ultrasound scan (USS). He was being investigated for fatty liver disease due to an elevated ALT (45 U/L). The patient consumed one bottle of wine daily with a background of hypertension, dyslipidaemia and gastroesophageal reflux disease. The patient was asymptomatic with a normal abdominal examination. His bloods and tumour markers including carcinoembryonic antigen (CEA), cancer antigen (CA) 19-9 and alpha-fetoprotein (AFP) were normal. A colonoscopy and gastroscopy was performed revealing only non-specific duodenitis with no malignancy.
A triple phase computed tomography (CT) scan identified a bilobed exophytic 70 × 56 x 78 mm segment IV liver lesion adjacent to the gallbladder and displacing the cystic artery. There was heterogenous hyperenhancement during arterial phase with washout in portal venous (PV) phase and no delayed enhancement. A magnetic resonance cholangiopancreatography (MRCP) showed the lesion had a multi-cystic or necrotic component posteriorly measuring 18 mm and was hyperintense in both T1 and T2-weighted imaging (WI) without fat or calcification. There was arterial enhancement that persisted into the PV phase without delayed phase enhancement. The right portal vein and its segmental branches were draped around the lesion causing mild compression, while the segmental biliary tree distal to the mass was dilated. The patient did not undergo a pre-operative biopsy. The differential diagnosis was hepatocellular carcinoma (HCC) in a non-cirrhotic liver, lymphoma or primary gallbladder tumour.
The patient underwent a segment IVb/V liver resection with enbloc cholecystectomy by a specialist hepato-pancreato-biliary (HPB) surgeon. The small bowel was normal. The mass is illustrated in . Macroscopically, there was a well-defined solid mass measuring 64 × 40 × 59 mm (). Microscopically, the tumour demonstrated an insular, acinar and trabecular architecture with variably fibrotic stroma. There was nuclear pleomorphism, readily identifiably mitoses and a Ki67 proliferation index of 3.4%. Immunohistochemical (IHC) stains were positive for synaptophysin, CD56 and weakly for CDX2 consistent for a grade 2 PHNET. IHC stains for chromogranin, gastrin and TTF-1 were negative.
The patient underwent a Dotatate positron emission tomography (PET)-CT scan post-operatively which revealed mild uptake in the head and uncinate process of the pancreas. This was considered to be a normal physiological variant given a normal CT scan pre-operatively. The Dotatate PET-CT scan was repeated six months later revealing a similar but stable appearance in the pancreas. Annual follow-up with CT scans for the last five years have been reassuring with no signs of recurrence or metastatic disease.
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pmc-6365392-2
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A 73-year-old male was referred with mildly deranged liver enzymes. The patient was a heavy drinker, consuming 100 g of alcohol per day for four decades. He had a background of heterozygous haemochromatosis, pacemaker and a transurethral resection of the prostate. The patient was asymptomatic with a normal examination. His hepatocellular liver enzymes were minimally elevated initially but had normalised on repeat testing a month later. Tumour markers including AFP, CA 19-9 and CEA were all normal. The patient had a normal colonoscopy two years prior.
An USS revealed hepatomegaly with a solid 25 mm hypoechoic area in segment III with a cystic component. The CT scan showed a 20 mm segment III liver lesion with enhancement in arterial and PV phase and washout on the delayed phase with an enhancing capsule. Due to the alcohol history the possibility of HCC in a cirrhotic liver was suspected. After a satisfactory indocyanine green clearance test, the patient underwent an uncomplicated left lateral liver resection by the same specialist HPB consultant surgeon. The patient was not macroscopically cirrhotic and there were no lesions in the small bowel. The tumour measured 18 × 17 mm. Microscopically, there was mild hepatic steatosis with no fibrosis. IHC staining for synaptophysin and chromogranin were positive. There were no mitoses but a Ki67% proliferation index of 0.5% was consistent with a well-differentiated grade 1 NET. IHC stains for HepPar 1, CD10, TTF-1, CDX2 and PAX8 were negative.
The patient underwent a Dotatate PET-CT scan two months post operatively to search for an undiagnosed primary and this was normal. Repeat Dotatate PET-CT 6 months later was again normal. Given the patient had a colonoscopy only two years prior and there were no other CT or PET-CT signs of extra-hepatic disease, a final diagnosis of HPNET was made.
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pmc-6365396-1
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A 70-year-old man visited a local clinic with abdominal pain. Blood biochemistry showed marginally high levels on a liver function test, and computed tomography (CT) imaging revealed dilatation of the peripheral left bile ducts (a). Subsequently, he was referred to our department. On admission, the patient’s body temperature was 35.9 °C, and the patient had no abnormal findings in the neck or thoraco-abdominal region. Blood tests showed no abnormalities, including prothrombin time percentage (PT%) and activated partial thromboplastin time (APTT), but blood biochemistry revealed that there was a slight increase in the level of alkaline phosphatase (ALP): 440 U/L. The total bilirubin level was 0.6 mg/dL. Examination of tumour markers revealed a carcinoembryonic antigen (CEA) level of 0.9 ng/mL and a level of cancer antigen 19-9 (CA19-9) was within normal range (6 U/mL) ().
Endoscopic retrograde cholangiopancreatography (ERCP) revealed disruption of contrast medium flow at the hilar part, and enhanced CT showed there was dilatation of left bile duct (). Although brush cytology at the site of the distal bile duct stricture was not scored as class V (adenocarcinoma), we diagnosed hilar cholangiocarcinoma, which is T1N0M0 according to the Union for International Cancer Control (UICC) classification. Extended left lobectomy with hepaticojejunostomy was performed. The tumour was pathologically diagnosed with biliary intraepithelial neoplasia at the hilar left part of the bile duct ().
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pmc-6365425-1
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A 50-year-old man received medical attention due to subacute onset of relapsing intestinal subocclusion episodes characterized by vomiting, diarrhea and marked abdominal distension, which gradually developed within approximately 40 days.
His past medical history included dyslipidemia and previous lipoma removal; additionally, his relatives reported apathy, loss of interest in work and hobbies and progressive social isolation occurring over the last two years. Two months before his presentation, he was hospitalized for acute onset of blurred vision and phosphenes in the left visual field that were associated with a frontal headache and confusion. The neurological evaluation revealed left hemianopia, temporal, and spatial disorientation and moderate psychic and motion slowness. Brain computed tomography (CT) and MRI showed a right temporo-occipital lesion with a high signal in the diffusion-weighted imaging (DWI) sequences, which was interpreted as an ischemic stroke (). The intracranial vessels were normal at the CT angiogram (CTA). The clinical course was complicated by a focal epileptic seizure with subsequent generalization; therefore, an antiepileptic therapy with carbamazepine was started.
To investigate the causes of intestinal obstruction, several diagnostic assessments were conducted. He underwent an abdominal CT and MRI and a colonoscopy to rule out expansive and infiltrative lesions, and total body positron emission tomography (PET) and a periumbilical fat biopsy were performed to exclude systemic vasculitis and amyloidosis, respectively. Therefore, a diagnosis of chronic intestinal pseudo-obstruction (IPO) was formulated. The patient was treated with pro-kinetic drugs and supported with parenteral nutrition, with progressive clinical improvement and restoration of intestinal transit. Lab tests also showed high serum lactate (1.7 mmol/l, normal range 0.0–1.3 mmol/l), hyponatremia and hypokalemia, probably due to inappropriate secretion of antidiuretic hormone syndrome (SIADH) caused by the carbamazepine therapy. The electrolytic disturbance was corrected, and carbamazepine was replaced with levetiracetam without neurological clinical improvement.
He was admitted to our Neurology Department for persistence of confusion and development of left arm clumsiness and stiffness. The brain MRI was repeated and showed evolution of the right hemispheric lesion, which extended to the parietal lobe and the anterior and medial parts of the temporal lobe and involved the subcortical white matter and cortex (, ). The electroencephalogram (EEG) showed slow persistent activity and periodic lateralized epileptiform discharges (PLEDs) in the right hemisphere, whereas the brain PET revealed a severe reduction in cortical glucose metabolism in the posterior right hemisphere (). Therefore, a metabolic etiology of the disturbance was suspected. To confirm this hypothesis, the patient underwent MR spectroscopy (MRS) and demonstrated elevation of the lactate peak within the abnormal lesion (), a muscle biopsy that was consistent with mitochondrial myopathy () and genetic testing, which revealed the presence of a mitochondrial DNA mutation (m.3243A>G) (heteroplasmy 13.1%) in the MT-TL1 gene encoding the leucine transfer RNA. A diagnosis of MELAS was formulated, and therapy with oral arginine, ubidecarenone and riboflavin was administered to the patient.
The genetic analysis was extended to his sister, nephews and two first grade cousins; the family tree is shown in . Four of these family members were positive for heteroplasmy and were asymptomatic. The audiological examination revealed bilateral sensorineural hearing loss.
Three months after hospital discharge, the patient presented with a new onset of an acute confusional state, visual illusion in the right visual field and severe frontal headache. At the neurological examination, the patient appeared confused, slowed down and disoriented. The confrontation visual field test showed right superior quadrantopsia together with the previous left hemianopsia. He also had face-blindness, visual agnosia, left upper arm apraxia and mild anomic aphasia. The lab tests showed increased serum lactate (2.9 mmol/l; normal range 0.0–1.3 mmol/l). A partial resolution of the previous right cortical lesion and the presence of a new cortical DWI abnormality in the left medial temporal and occipital lobes was observed at the brain MRI (, lower part), revealing a new stroke-like episode. Additionally, the Fluid Attenuated Recovery (FLAIR) sequences identified a marked and greater cortical atrophy with increased ventricular sizes (, upper part). To counter the vasogenic edema resulting from blood-brain barrier dysfunction due to mitochondrial microangiopathy, the patient received intramuscular corticosteroids (dexamethasone 8 mg), but the treatment was prematurely stopped due to onset of drug-induced diabetes mellitus, and insulin therapy was started.
In the following month, the patient developed a rapidly progressive ideomotor decline; the patient had spatial and temporal disorientation, psychomotor agitation, speech disturbance with confabulation and cortical-blindness. A new left lateral temporal and occipital lesion was identified on brain MRI (not available); oral arginine therapy was increased, and intravenous L-arginine was administered. During hospitalization, the patient suffered again from acute IPO and was treated conservatively. He also manifested a non-convulsive epileptic status. To achieve seizure control, lacosamide, phenytoin, and clobazam were progressively added to the levetiracetam. Despite therapeutic implementation, the patient did not recover, and he died one month later ().
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pmc-6366039-1
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A 56-year-old man presented with fever for three weeks and memory decline for two weeks, especially deficits in anterograde amnesia. Initial neurological examination revealed rapidly progressive cognitive impairment. The scores of Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) were 19/30 and 15/30, respectively. No epileptic seizures occurred during the disease course.
The cerebrospinal fluid (CSF) showed mildly elevated leukocyte (19/uL, normal range 0–8/uL) and glucose (5.39 mmol/L, normal range 2.5–4.5 mmol/L), lowered chloride (113.5 mmol/L, normal range 120-130 mmol/L), and a normal protein level (44 mg/dL, normal range 20–40 mg/dL). At the same time, the serum tests of sodium, chloride and blood glucose were 126.1 mmol/L, 94.2 mmol/L and 7.26 mmol/L, respectively. The LGI1-Ab was positive (+++) both in the serum and CSF (Fig. ), however, the other biomarkers of AE (NMDAR-Ab, AMPAR2-Ab, GABABR-Ab, Caspr2-Ab), tumor markers (CEA, AFP, CA125, CA19–9, CA15–3, CA724, SCCAg, NSE, T-PSA, CYFRA21-1) and paraneoplastic neuronal antibodies (anti-Hu, −Ri, -Yo, −Ma/Ta, -Amphiphysin, -CV2, -SOX1, −Tr) were all unremarkable. The other laboratory tests revealed within normal limits. Electroencephalogram was normal. Cranial magnetic resonance images (MRI) indicated hyperintensities in bilateral hippocampus on T2-weighted fluid-attenuated inversion recovery (Fig.a) and diffusion weighted imaging (Fig.b) sequences. Twelve days later, the repeat MRI showed some abnormal hyperintensities particularly in the left hippocampus (Fig.c, d). Chest computed tomography and 18F-FDG PET showed no signs of tumor (Fig.). One month after onset of cognitive decline, the findings of ASL and 18F-FDG PET showed no abnormal perfusion/metabolism in the bilateral hippocampus (Fig.).
He was diagnosed with anti-LGI1 AE, with the treatment of methylprednisolone and IVIG, later with oral prednisone for six months. Fifteen days after his admission, he recovered obviously and discharged from our department with mild memory impairment. During 30 days’ follow-up, his symptoms were in complete remission with immunomodulation. The cognitive function became normal with MMSE 30/30.
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pmc-6366044-1
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A 47-year-old male patient, who has no specific past medical history, suffered severe thoracic trauma in a forklift accident 14 h before he was transferred to our hospital. After having his chest crushed by a forklift, the patient instantly had hemoptysis and showed serious signs of respiratory distress. At the local hospital, the physical examination revealed pulse oxygen was at approximately 80%; there was subcutaneous emphysema in the neck and chest; breathing was inaudible by auscultation in the left lung; and, there were moist rales in the right lung. The patient immediately received single-lumen intubation and mechanical ventilation (MV). The CT scan showed left-side pneumothorax, right-side pneumo-hemothorax, bilateral traumatic wet lung, and multiple rib fractures. The bronchoscopy also indicated a left main bronchial rupture. Therefore, the patient was treated immediately with bilateral closed thoracic drainage, fluid infusion, and immobilization of the chest wall.
Treatment notwithstanding, there was no alleviation of the patient’s symptoms, and his pulse oxygen remained consistently low (approximately 80%). Consequently, he was transferred directly to our department. The minute ventilation volume was only 2 to 3 L/min by single-lumen mechanical ventilation. Therefore, the single-lumen tube was replaced with a double-lumen tube, with ventilation only to the right lung to prevent leakage. Nevertheless, the patient’s pulse oxygen remained low, with no remediation of his respiratory distress. On admission, after running the necessary checks and analyses, with his APACHE II score at 25, the predicted odds of mortality was 51%. His blood gas revealed both respiratory acidosis and metabolic acidosis, with both exacerbating gradually. Figure exhibited the chest x-rays at different times, before pneumonectomy (Fig. a) and after the withdrawal of ECMO (Fig. b).
At that critical moment, ECMO was initiated without delay. Upon selection of the veno-venous (V-V) ECMO model, catheters were inserted into the right jugular vein (arterial catheter, the tip nearly reached right atrium) and right femoral vein (venous catheter, the tip located at inferior vena cava). Specifically, blood was drawn out from the right atrium to the ECMO device (Maquet, ROTAFLOW Console), after oxygenation it was infused into the right femoral vein, with the gas flow at 4-6 L/min, fraction of inspiration O2(FiO2) at 100% and the pump operating at 3480—3610 rpm. Upon receipt of ECMO and MV, the patient’s oxygenation stabilized; his pulse oxygen rose to 97%—100%; and his respiratory distress was alleviated significantly, thus permitting urgently needed surgery. With the consent of his family members, the patient had an emergency, video-assisted thoracoscopic exploratory thoracotomy. The edema and consolidation of the entire left lung were severe. 1 cm from the tracheal carina, the postero-lateral wall of the left main bronchus experienced an 8 cm long and irregular rupture, which spread to the distal end of the secondary bronchus of the upper and inferior lobes. The rupture was unable to be ordinarily repaired and anastomosed, so the patient required a total left lung resection. The thoracoscopic pictures during the surgery are exhibited in Fig. .
After left lung resection, with the support of ECMO, the parameters of ventilator (PURITAN BENNETT 840) were set as follows, mode: Synchronized Intermittent Mandatory Ventilation (SIMV), Frequency(F): 12 times/min, VT:200 ml, FiO2: 40%, and positive end expiratory pressure (PEEP): 8cmH2O. Gradually, with ECMO and low tidal volume (VT) MV (VT 200 ml) as the main therapy, assisted by anti-inflammatories, antibiotics, sedatives, and analgesics, the patient made a recovery. ECMO was sustained up to the 10th day, and MV until the 20th day, post-operation. After the initiation of ECMO, heparin was micro-pump injected (125u-750u/hour), and activated clotting time (ACT) was monitored every 2 h. ACT was expected to remain between 160 s to 180 s, which was fluctuating between 130 s to 210 s without severe bleeding complication occurring. After the initiation of ECMO, arterial and venous blood gas were tested every 6 h; 24 h before the patient’s ECMO weaning, the gas flow was reduced to 2 L/min; 6 h before weaning to 0 L/min; FiO2 was reduced to 80%, the O2 and CO2 partial pressure of blood gas were dynamically stable, then ECMO was weaned and the related catheters were removed. During the ECMO treatment, infections such as catheter-related bloodstream infection or ventilator associated pneumonia (VAP) should be anticipated, and antibiotics for most gram-negative and some of the sensitive gram-positive bacteria should be applied. In this case, the culture of sputum samples and broncho-alveolar lavage fluid or blood samples were all negative. In the first week after the operation, piperacillin-sulbactam was used to prevent possible lung infections, later to be replaced by imipenem and levofloxacin when the fever and white blood cell count climbed. Ulinastatin, a glycoprotein found in human urine and blood, proved to be a multivalent, Kunitz-type serine protease inhibitor and exhibited moderate anti-inflammatory effects without any immunosuppression side-effects []; it was used for immuno-modulation and anti-inflammation in our case. Because the invasive double-lumen intubation and right-side multiple rib fractures caused considerable pain, appropriate analgesics and sedatives were essential for the post-op compliance of the patient. The combination of dexmedetomidine and fentanyl or midazolam and morphine were used alternatively for sedation and analgesia. The alteration reduced the risk of drug accumulation while keeping a satisfying effectiveness. Finally, considering the subcutaneous emphysema in the neck and the edema of bronchial local tissue, a tracheotomy was not performed in the early phase, but a double-lumen tube was retained until the 10th day to cope with possible leakage in the bronchial stump.
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pmc-6366080-1
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A 12 year-old boy Caucasian boy was admitted during his summer holydays to a pediatric emergency department with repeated vomiting, malaise, excessive thirst, dizziness on standing and one episode of syncope after drinking 1 liter of water.
His past history was remarkable for a similar episode 3 weeks before: after repeated vomiting he had been admitted to the local hospital and a marked hyponatraemia (124 mEq/L) was found. He was diagnosed with gastroenteritis, hyponatraemia was corrected with normal saline infusion and the boy was discharged. He eventually remained symptom-free until the actual episode.
At admission no fever, diarrhea, change in urinary output or change in weight were reported. Physical examination showed an apyretic, eupnoic, asthenic boy, with a tanned skin color. Heart rate was 63 bpm, blood pressure was 98/62 mmHg and SaO2 was 100%.
Laboratory tests showed hyponatraemia (121 mEq/L), hypochloraemia (86 mEq/L) and mild hyperkalaemia (5,91 mEq/L) with low plasmatic osmolarity (248 mOsm/Kg). Urinary sodium was 163 mEq/L, potassium 48 mEq/L, chlorine 119 mEq/L, with high urinary osmolarity (896 mOsm/L; 300–900). Blood and urinary glucose, white cell count, blood gas analysis and renal function were normal.
A syndrome of inappropriate secretion of antidiuretic hormone (SIADH) was suspected and fluid restriction with two-thirds of the standard maintenance rate of normal saline was carried out.
After 4 hours hyponatraemia worsened (119 mEq/L) despite fluid restriction, while asthenia and inability to stand upright persisted, thus posing the suspect of adrenal insufficiency.
Low levels of cortisol (7.95 mcg/dl; 6.2–19.4 reference range), marked ACTH increase (> 1250 pg/mL), high levels of renin (> 500 mUI/ml) and low levels of aldosterone (3.1 ng/dL; 3–30 reference range) confirmed the diagnosis of adrenal insufficiency. Intravenous hydrocortisone treatment was started at the dose of 100 mg/day, then decreased to 50 mg each 6 h/day; oral daily fludrocortisone, 0.15 mg, was associated. Maintenance therapy with oral hydrocortisone was introduced after 3 days, while electrolyte and ACTH values returned within normal limits in 48 h. The patient presented two further episodes of symptomatic orthostatic hypotension in the first 72 h after diagnosis and recovered in the following days. Anti-adrenal antibodies tested positive, confirming autoimmune adrenalitis (Addison disease).
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