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pmc-6318083-1	Patient One was a 30-year-old woman, American Society of Anesthesiologists (ASA) Classification I (weight, 64 kg; height, 168 cm) with no coexisting diseases. She was scheduled for arthroscopic shoulder surgery. Her preoperative shoulder mobility over the shoulder joint was limited to 120° with severe pain. The patient described acute relief of the pain in her shoulder and increased mobility after ESPB performed before the surgery started. She was treated surgically for arthroscopic acromioplasty and bursectomy. Her pain was recorded as 1/10 on NRS and needed no additional analgesia in the PACU. Her maximum dynamic pain score was 4/10 on NRS. Her 24-hour PCA tramadol consumption was 40 mg total.
pmc-6318083-2	Patient Two was a 65-year-old woman, ASA Class I (weight, 55 kg; height 165 cm) registered for a Neer Type 3 humeral fracture and surgical repair. She reported sudden pain relief after the application of ESPB. An open reduction and internal fixation via the anterolateral method was performed under general anaesthesia. In the PACU, she reported her pain as 2/10 on NRS with mobilization. At the 24-hour follow-up evaluation, she reported no pain score higher than 3/10 on NRS, and no rescue analgesic treatment was administered. Her total PCA consumption was 240 mg in 24 hours. Although her PCA consumption was high, she needed no fentanyl in the PACU nor additional diclofenac sodium and meperidine as rescue analgesia.
pmc-6318083-3	Patient Three was a 39-year-old woman, ASA Class I (weight, 55 kg; height, 165 cm) who presented for arthroscopic shoulder surgery. She had severe pain (8/10) on NRS with abduction-adduction at the shoulder and was not able to move over 30°. After ESPB, the patient was able to move her arm at the shoulder to approximately to 70° abduction without pain. She had acromioplasty and fibrous tissue resection in the shoulder joint including shoulder cap. In the PACU, her pain score was 8/10 on NRS, so she was given 50 µg fentanyl. Her PCA tramadol consumption was 240 mg in 24 hours. Despite the rescue analgesia treatment (IM diclofenac sodium, 75 mg and IV meperidine, 50 mg), her level of pain remained constant between 4/10 to 6/10 on NRS.
pmc-6318085-1	A 67-year-old woman presented to the emergency department with difficulty in breathing. She had a past medical history of hypertension, hyperlipidemia, and hemangiopericytoma of the neck treated by surgical resection and radiation therapy 20 years ago. At the hospital, her vital signs were stable with a heart rate of 96 beats per minute, respiratory rate of 20 per minute, blood pressure of 138/78 mmHg, and percentage oxygen saturation of 96% on room air. Review of systems was otherwise unremarkable. The cardiovascular, respiratory, and abdominal examination did not reveal any pathological findings. Complete blood count showed mild anemia with a hemoglobin level of 11 g/dL. The basic metabolic panel, venous blood gas, and liver profile were unremarkable. Chest X-ray showed a round mass-like opacity in the left suprahilar region (Figure ). Computerized tomography (CT) scan of the chest with intravenous contrast enhancement revealed a well-circumscribed heterogeneous mass arising from the pleura adjacent to the superior and medial left pulmonary artery (Figure ). At the inferior extent of the tumor, there was an invasion of the left inferior pulmonary vein with extension into the left atrium (Figure ). The lesion was biopsied confirming a mesenchymal neoplasm compatible with SFT (Figure ). The patient underwent pneumonectomy and resection of left atrial mass with pericardial patch repair of the left atrium. Postoperatively, her hospital course was complicated with left-sided pneumothorax requiring chest tube placement, septic shock requiring vasopressors, acute respiratory distress syndrome (ARDS) requiring mechanical ventilation and acute renal failure requiring hemodialysis. She underwent tracheostomy and percutaneous gastrostomy tube placement. Despite efforts of the multidisciplinary team, the patient did not show clinical improvement. The family then decided to do not resuscitate (DNR) in case of cardiac arrest. Unfortunately, due to above-stated complications, the patient died in the hospital on day 107.
pmc-6318087-1	An 82-year-old male with a history of type 2 diabetes mellitus, hypertension, benign prostatic hyperplasia (BPH) with a size of 40 gram presented with asthenia, suprapubic pain, and distension of the lower abdomen. He underwent transurethral resection of prostate (TURP) for his BPH one week prior to admission and had an indwelling Foley’s catheter (due to urinary incontinence) at presentation. He also complained of non-bloody, watery diarrhea with four to five episodes per day for five days. He denied fever, cloudy urine, purulent urethral secretion, or any back pain. He also denied any prior history of radiotherapy or other gastrointestinal (GI) surgery related to the colon. At presentation, the patient was alert, awake, and cooperative and his vital signs included a temperature of 98.6° F, respiratory rate of 14 per minute, pulse of 86 beats per minute, BP of 113/56 mm Hg, and saturating 99% in room air. The physical examination revealed an indwelling Foley catheter with an attached right thigh bag showing clear urine. Laboratory investigations were notable for a slight elevation of the creatinine from the baseline. The urinalysis was positive for leukocyte esterase and nitrates, five to 15 red blood cell/high power field (HPF), and 30-50 white blood cell/HPF. The stool culture revealed no growth of microorganisms. The patient was started on intravenous (IV) normal saline and IV ceftriaxone 1 gram daily for a suspected urinary tract infection. Computed tomography (CT) of the abdomen and pelvis with IV contrast demonstrated findings consistent with a RUF. Cystourethrogram under fluoroscopy showed the extravasation of contrast into the rectum, which is also consistent with a rectourethral fistula (RUF) (Figure ). A colonoscopy was performed and revealed a Foley's catheter in the rectum (Figures -). A rectal biopsy was not obtained, as, grossly, there was no evidence of malignancy, infectious process, or inflammatory process. The abdominal and pelvic CT scan also showed RUF (Figures -).
pmc-6318088-1	An 11-year-old female was admitted to the emergency department with complaints of intermittent fever for over one month, periorbital puffiness for four days, and abdominal distension with swelling on hands and feet for one day. In the hospital, she developed periorbital bruising and rashes on the extensor surfaces followed by the trunk over a period of four days. The patient experienced difficulty in walking due to a history of joint pain which was profound at the knee joint. However, there was no history of morning stiffness. She denied photosensitivity, chest pain, shortness of breath, hair loss, oral ulcers, weight loss, fatigue, and any medication use. There was no known family history of autoimmune diseases. The patient reported having suffered from measles seven months back. On examination, she was a tall, lean-built child, lying on bed, oriented to time, person, and place. Her vital signs on arrival were as follows: blood pressure, 142/110 mmHg (reference, 120/80 mmHg); pulse, 154 bpm (reference range, 75 to 118 bpm); and temperature, 37.5oC (reference range, 36.5 to 37.5oC). Her height was measured as 130 cm (at third centile) and weight, 25 kg below the third centile. The patient appeared sick-looking, with signs of pallor, conjunctival hemorrhage, and bruises around the eyes. Palpable purpura and petechiae over the extensor surfaces were seen whereas edema of the hands and feet was noted. Central nervous system (CNS) examination revealed 15/15 on the Glasgow Coma Scale (GCS). Cardiovascular examination revealed muffled heart sounds and good volume pulse. Chest examination revealed decreased breathing sounds on the left side of the chest with dull notes on percussion. Abdominal examination revealed positive shifting dullness with a distended abdomen. Diagnostic criteria Suspicion of SLE was raised on clinical findings, therefore, work-up for SLE was ordered. The updated diagnostic criteria of SLE was used which the patient fulfilled. Diagnosis of SLE is made if four out of 17 (including at least one of the eleven clinical and one of the six immunological) “Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for systemic lupus erythematosus” are present. Biopsy-proven lupus nephritis with a positive antinuclear antibody (ANA) or anti-double-stranded deoxyribonucleic acid antibody (anti-dsDNA) also satisfies the diagnosis of SLE []. From the clinical criteria of SLICC, the following four were present in our patient: (i) serositis (muffled heart sounds and chest X-ray showing pericardial effusion); (ii) acute cutaneous lupus (conjunctival hemorrhage, bruises around eyes, palpable purpura, and petechiae over the extensor surfaces); (iii) renal (proteinuria, red blood cell casts); and (iv) raised spot urine protein/creatinine ratio. From the six immunological criteria of SLICC, the following four were present: (i) ANA positive; (ii) C3 & C4 levels decreased; (iii) anti-dsDNA positive; and (iv) direct Coombs test positive. Chest radiograph showed an enlarged cardiac silhouette indicative of pericardial effusion (Figure ). Echocardiography revealed a large collection of fluid around the posterior aspect of heart with atrial collapse strongly suggestive of cardiac tamponade. Investigative analysis revealed that the patient was anemic and thrombocytopenic but with a normal leukocyte count. Erythrocyte sedimentation rate (ESR) was 92. Urine protein creatinine ratio was raised at 11.98 (reference, <0.3). Urine detailed report showed proteinuria and hematuria. Blood urea trend was recorded for six days which revealed an increasing pattern starting at 149 mg/dL on the first day to 168 mg/dL on the last day (reference range, 10 to 40 mg/dL). Creatinine levels showed a decreasing six-day pattern ranging from 1.49 mg/dL at first day to 1.03 mg/dL on the sixth day (reference range, 0.2 to 1.1 mg/dL). These lab values were suggestive of the autoimmune root of the disease and the renal dysfunction secondary to it (Table ). Apart from ANA and anti-dsDNA positivity, the patient's serum came out positive for anti-Ro/SSA (anti-Sjogren's-syndrome-related antigen A, also called anti-Ro, or the combination anti-SSA/Ro or anti-Ro/SSA autoantibodies) and anti-La/SSB antibodies. Direct Coombs test was also positive. Serum complement levels were low with a C3 of 45 mg/dL (reference range, 101 to 186 mg/dL) and C4 of 8 mg/dL (reference range, 16 to 47 mg/dL). Thus, confirming the diagnosis of cSLE with cardiac tamponade. As a management and definitive diagnostic tool, a fluoroscopy-guided pericardiocentesis was performed without any delay. A total of 430 ml of fluid was drained which was hemorrhagic; cytological analysis of the fluid was negative for malignancy with 60% neutrophils and 40% lymphocytes. Infectious workup was negative for acid-fast bacilli, any growth, or vegetation. Renal biopsy was performed to confirm the underlying cause of renal malfunction, as hypertension and proteinuria was noticed. Post-pericardiocentesis echocardiogram showed trace pericardial effusion and an ejection fraction of 35% with overall improvement. The patient was managed for lupus carditis and nephritis and started on pulse therapy of intravenous methylprednisolone (500 to 1000 mg). This was followed by a low dose regime and treatment with mycophenolate mofetil starting on the seventh day which was aimed at reducing renal disease progression. The patient was continuously provided with organ support care such as positive pressure ventilation, hemodialysis, and invasive cardiovascular monitoring along with the instillation of intravenous fluid supplements. The patient improved significantly, was discharged from our hospital, and asked to follow up.
pmc-6318090-1	A one-year-old girl presented with progressively increased forehead swelling of seven months duration. The swelling progressively increased in size and extended up to the medial edge of both her upper eyelids (Figure ). This large swelling caused the patient to have slight difficulties in opening her eyes widely. On the other hand, there was no eye redness, discharge, or reduced vision. The patient also had constitutional symptoms like loss of appetite and weight loss over this period of several months. On examination, there was a massive, round, multiple lobulated and erythematous swelling extending from the forehead until the medial part of bilateral upper lids with a central crusted lesion surrounded by scaly skin. It was nontender, warm, and firm in consistency. It measured about 7 cm x 7 cm in diameter. Her best corrected vision was 6/6. There was no refractive error noted. Both eyes were orthophoric. The remainder of the eye examination revealed normal anterior segment. The fundus examinations were normal with pink and healthy optic disc. There was no Roth's spot or retinal hemorrhage. The intraocular pressure was normal. The extraocular movements were also normal. A systemic examination revealed multiple leukemic cutis, small red-to-brown lesions on the scalp, and presence of hepatosplenomegaly. Bilateral multiple cervical lymph nodes were palpable. Otherwise, her neurological examination was normal. Her hemoglobin was 9.7 g/dL and the total white cell count (TWBC) was 68.97 x 109/l. Her differential count showed 91.7% lymphocytes, 0.9% neutrophils, and 7.2% monocytes. There was leucoerythroblastic blood film with 62% blast cells on the full blood picture (FBP) (Figure ). Bone marrow aspiration (BMA) showed blast cells, which exhibited high nuclear-cytoplasmic ratio, and scanty cytoplasm with some blast cells showing prominent nucleoli (Figures , ). The flow cytometry result confirmed the diagnosis of B-acute lymphoblastic leukemia by showing positivity for CD79a, HLA-DR, CD 10, CD 19, heterogenous cCD 22, cytoplasmic IgM and negative for CD34 and terminal deoxynucleotidyl transferase (TdT). A cerebral spinal fluid (CSF) analysis was sent for and it showed no intracranial extension. Computed tomographic (CT) scan images of the brain revealed multiple, well-defined enhanced lesions at the frontal scalp with the largest lesion at the anterior glabella. The lesion extended into the bilateral orbital cavities and caused minimal lateral displacement of the bilateral orbits. Otherwise, the globes were normal (Figure ). A repeated computed tomography (CT) scan of the brain showed resolved frontal swelling. Chemotherapy was commenced based on the United Kingdom (UK) Acute Lymphoblastic Lymphoma Protocols 97/99 (regime B: high risk). She attained remission of the disease with significantly reduced frontal swelling after the induction phase of chemotherapy (Figure ). Currently, the patient is still under pediatric oncology follow-up and on the maintenance phase of chemotherapy.
pmc-6318094-1	A 70-year-old female with a history of UC presented with a fever of 101℉ in the setting of progressive weakness over the last six months. Her UC was being treated with adalimumab, with her first dose at 15 months prior to presentation and the most recent dose one week prior to presentation. Physical exam was significant for predominant left submandibular lymphadenopathy without any rashes, bruising, or joint swelling. Labs showed pancytopenia with white blood cell (WBC) count 2.0 K/μL, hemoglobin 11.5 g/dL, platelets 10 K/μL, and absolute neutrophil count (ANC) 1.1 K/μL. A computed tomography (CT) scan of the chest, abdomen, and pelvis illustrated bilateral axillary lymphadenopathy (Figure ) and enlarged retroperitoneal lymph nodes from the level of the celiac axis to the groin. Adalimumab was discontinued, infectious workup was initiated, and antibiotics were started for neutropenic fever. Shortly after, with the resolution of fever and negative infectious workup, antibiotics were discontinued. Tbo-filgrastim was administered with an increase in the WBC count to 8.2 K/μL and ANC to 6.2 K/μL. A lymph node biopsy revealed an atypical lymphoid proliferation with necrotizing granulomas. The patient remained clinically stable with a concordant increase in all cell lines in the following days and was discharged. A repeat CT scan performed two weeks later (Figure ) illustrated a complete resolution of lymphadenopathy.
pmc-6318103-1	A 60-year-old, right-handed male presented to the emergency room (ER) for acute behavioral changes and abnormal movements for two weeks. He is a truck driver with a remote history of encephalitis and stroke with no baseline deficits. On examination, the patient had stereotypic, “tic-like,” bilateral facial twitches more prominent on the left side, constant winking of the left eye, grimacing facial expressions, seeming to indicate pain along with some tongue thrusting (like a gecko) movements. A video shot by his wife documented these movements. In addition, the patient also exhibited pressured speech and tangential thoughts. On the Montreal Cognitive Assessment (MoCA) scale, the patient scored 20/30, scoring poorly on language, word recall, and calculation. Given his acute behavioral changes and abnormal movements, a computed tomography (CT) scan of the head, an electroencephalogram (EEG), a lumbar puncture with cerebrospinal fluid (CSF) analysis, complete metabolic profile (CMP), complete blood counts (CBC), urine drug screen, serum, and CSF paraneoplastic panel, iron/ferritin levels, and serum copper were ordered to rule out limbic encephalitis, seizures, and infection. All the tests were unremarkable. Magnetic resonance imagining (MRI) of the brain on axial sequence showed a lacune at the level of the midbrain on T2 fluid attenuated inversion recovery (FLAIR) suggestive of an old infarct (Figure ). He was seen five days later in the outpatient neurology clinic and was noted to be much calmer and interacted well. He was started on ziprasidone by his primary care physician for suspected schizophrenia, which helped with the behavioral changes and abnormal movements. Upon further questioning, it was noted that the patient had behavioral changes many months prior to the acute onset of abnormal movements. On his second follow-up visit one month later, it was noted that he was started on clopidogrel for deep vein thrombosis (DVT) of the right calf that happened in the interim. On examination, the patient had dyskinetic movements involving the mouth and the tongue. Choreiform and occasional high-amplitude ballistic movements are noted in the left upper and lower extremities. Further workup, consisting of a paraneoplastic panel, vasculitis panel, myasthenia panel, CSF oligoclonal bands, comprehensive drug screen, and CT of chest, abdomen, and pelvis, revealed no abnormalities. CT angiogram of the head did not reveal irregular narrowing or dilatation. MRI of the brain revealed a tiny stroke at the level of the centrum semiovale in the right frontal region (Figures -). A coagulation panel showed an elevated phospholipid IgM antibody and anti-beta 2 glycoprotein 1 (B2GP1) antibody. The diagnosis of APS presenting with chorea was made, and the patient was started on aripiprazole, which improved the symptoms.
pmc-6318104-1	A 34-year-old female, Gravida 4 Parity 3+0, was seen in the emergency room of a tertiary care hospital, in Karachi, with labor pains. She had all previous uneventful vaginal deliveries with healthy and alive babies. The baby was the fourth outcome of a consanguineous marriage, the couple being maternal first cousins. The parents did not report any history of repeated miscarriages/stillbirths, genetic anomalies in the elder children, or any inherited skin disorder. It was an unbooked case. She had no antenatal record available. Abdominal examination revealed the height of fundus roughly equivalent to almost 36 weeks of gestation and the fetal head engaged. The labor progressed spontaneously. A female baby of weight 1.8 kg was delivered with Apgar score 7 and 8 at 1 and 5 minutes, respectively. At the time of birth, the body of the neonate was covered with armour-plate-like, thick and widespread yellowish scales; between these keratin layers of scales were erythematous fissures splitting the scales and extending deep into the dermis. The scales and fissures covered the body in a diamond-like pattern and were marked in the flexures. All limbs and digits were rigidly semi-flexed. Other evident features included ectropion, eclabium with a fixed and wide-open mouth, flat nasal bridge, and rudimentary external ears. The eyebrows and eyelashes were absent; however, scalp hair was evident. All natural orifices were patent (Figure ). The baby was evaluated by the pediatrician and the clinical diagnosis of harlequin ichthyosis was made. The family was counseled and the baby was shifted to the neonatal intensive care unit (NICU) where she was kept in a humidified incubator. Although the baby had adequate sucking reflex, in order to prevent any incidence of aspiration, she was started with parenteral nutrition, intravenous antibiotics and continuous positive airway pressure (CPAP) was delivered via Bubble-CPAP. Skin was cleaned with normal saline. Liquid paraffin and emollients were gently applied to the whole cutaneous surface every two hours. Topical antibiotics were also added to the skincare. Antibacterial eyedrops and sterilized cotton eye pads were applied. The baby developed respiratory distress within a few hours of birth, despite the supportive management. The baby died on the second day of life due to respiratory distress. The parents were psychologically supported, the disease spectrum was explained to them, and genetic counseling was recommended for future pregnancies.
pmc-6318106-1	A 21-year-old Saudi female, previously healthy, presented to the emergency department with a nine-day history of hallucinations, delusions, insomnia, cognitive decline, recurrent episodes of loss of body tone, and inability to walk. These symptoms were preceded by a two-day history of a non-specific headache and prodromal flu-like illness. The patient reported similar symptoms six weeks previously, followed by severe respiratory distress requiring admission to an intensive care unit (ICU) for intubation and mechanical ventilation. Her past surgical and family history were unremarkable. On general physical examination, her vital signs were unremarkable and the patient was drowsy and lethargic. Central nervous examination (CNS) was remarkable for confusion, disorientation (to person, place, and time), delayed responses, speaking in small sentences, inability to move her lower limbs, and being uncooperative. Abdominal examination was remarkable for mild right lower quadrant tenderness without organomegaly or palpable masses. Initial laboratory findings were remarkable for an elevated serum level CA-125 of 205 units/ml (normal: 0 - 35 units/ml) and CA 19-9 of 121 units/ml (normal: 0 - 37 units/ml). Magnetic resonance imaging (MRI) of the brain showed no evidence of acute of intracranial abnormality. An electroencephalogram (EEG) was unremarkable. Cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis and oligoclonal bands. Electromyogram (EMG) and nerve conduction study (NCS) of the lower limbs showed mild non-specific myopathic changes. Computed tomography (CT) scan of the abdomen and pelvis showed a 7.2 x 6.3 x 5.5 cm mass of the right ovary that was highly suspicious for a mature teratoma with fat densities and calcified foci (Figure ). Psychiatric consultation was recommended for the purpose of (a) pulse methylprednisolone therapy to treat the psychotic symptoms of delusions and hallucinations and (b) melatonin therapy to aid in sleep. However, the patient showed no improvement. In view of a possible paraneoplastic teratoma-associated anti-NMDA receptor encephalitis, samples were sent to the Mayo Clinic Hospital, Rochester, Minnesota, USA to test for the presence of anti-NMDA receptor antibodies. The results came back positive for anti-NMDA receptor antibodies in the serum and CSF. Subsequently, the patient was started on a five-day course of intravenous methylprednisolone, 1 gm per day, and intravenous immunoglobulins (IVIG), 400 mg/kg/day. The neuropsychiatric symptoms showed some improvement. Afterward, the patient was subjected to a right oophorectomy. Macroscopically, the resected mass had multi-loculated cystic cavities filled with fatty, sebaceous, tooth, and hair elements. Microscopically, the ovarian cyst was lined by ectodermal derivatives containing skin adnexa, such as hair follicles, arrector pili muscles, and sebaceous glands, as well as the identification of fat cells (Figure ). The final histopathological diagnosis was mature teratoma associated with paraneoplastic anti-NMDA receptor encephalitis. Postoperatively, the patient had an uneventful postoperative course. One day following the right oophorectomy procedure, her neuropsychiatric symptoms improved dramatically. The patient did not receive any secondary immunotherapies. At a six-month follow-up at the outpatient clinic, the patient was symptom-free.
pmc-6318107-1	A never-smoking 64-year-old male of Asian ethnicity presented with stage I disease. He was treated with a left lower lobectomy (stage T1bN0M0). Pathology showed adenocarcinoma histology. Genetic analysis reported no mutation in EGFR exon 19 or 21 using polymerase chain reaction and no anaplastic lymphoma kinase (ALK) re-arrangement using fluorescence in-situ hybridization. He recurred 12 months later with extensive mediastinal lymphadenopathy, a bone metastasis, and a left-sided malignant pleural effusion. Cytology from his pleural fluid showed adenocarcinoma. Genetic analysis showed an EGFR exon 20 variant (c.2313_2314insACG), which results in the insertion of one amino acid residue (p.Asn771_Pro772insThr), and has been associated with reduced sensitivity to EGFR-TKIs []. None of the following variants were observed: EGFR exon 19 or 21, v-raf murine sarcoma viral oncogene homolog B (BRAF), kirsten rat sarcoma viral oncogene (KRAS), ALK, isocitrate dehydrogenase (IDH) 1/2, phosphatase and tensin homolog (PTEN), or tumor protein p53 (TP53). The patient was started on cisplatin and pemetrexed followed by maintenance pemetrexed. After 10 months, he developed tiny (2 mm in size), innumerable, diffusely located, bilateral lung nodules on computed tomography (CT) imaging (Figure ). There was no predominance in any particular lobe, and there was no dominant pulmonary mass. He received docetaxel (but had an infusion reaction) and then nivolumab for four months with no response and further progression of nodules, confirming that the nodules were disease-related rather than a specific drug-related interstitial disease. He then received single-agent vinorelbine, which had a near-complete effect on the tiny diffuse lung metastases. Another 12 months later, he developed numerous (>15 in number), small (3-6 mm in size), diffusely located intracranial metastases, as identified on a head CT (Figure ). There was no significant peritumoral edema and no dominant brain mass. His performance status was deteriorating at that time and he declined whole brain radiotherapy (WBRT). The patient died 25.5 months from the date of metastatic diagnosis from cancer progression.
pmc-6318107-2	A 47-year-old male of Asian ethnicity and a 17 pack-year history of smoking presented with metastatic disease. Investigations showed a left upper lobe primary tumor (4.7 cm in diameter) with extensive thoracic lymphadenopathy and multiple ring-enhancing brain metastases which measured up to 4.5 cm in diameter predominantly in the right cerebral hemisphere. Pathology obtained from the lung mass showed adenocarcinoma histology. Genetic analysis showed an EGFR exon 20 variant (c.2312_2314dup), which had resulted in the insertion of one amino acid residual (p.Asn771_Pro772insHis), and was associated with reduced sensitivity to EGFR-TKI []. None of the following variants were observed: EGFR exon 19 or 21, BRAF, KRAS, ALK, IDH 1/2, PTEN, or TP53. He received WBRT which resulted in a near-complete response in all brain lesions. At the completion of WBRT, re-evaluation with chest CT imaging showed small (8 mm in size), innumerable, diffusely located, bilateral lung nodular metastases (Figure ). He received cisplatin and pemetrexed with a partial response of the left lung primary and near-complete resolution of the miliary lung metastases. At progression, he received docetaxel as second-line and nivolumab as third-line and erlotinib as fourth-line therapy. At 15 months, he developed headaches. Magnetic resonance imaging (MRI) of the head showed new tiny (3-8 mm in size), numerous (>25 in number), diffusely located, nodular brain metastases with no significant peritumoral edema or dominant mass lesion (Figure ). He was treated with repeat WBRT. The patient died 24 months from the time of initial diagnosis from cancer progression.
pmc-6318108-1	A 30-year-old African American male with past medical history of insulin dependent diabetes mellitus, hypertension, sleep apnea and a recent myocardial infarction seven months prior presents with acute onset dysarthria and angioedema secondary to lisinopril use. His angioedema was treated appropriately, and stroke team was consulted for concerns due to dysarthria. The patient also commented that he was having intermittent right-sided upper extremity paresthesias. A non-contrast computer tomography (CT) of head was obtained, which demonstrated a subacute right parietal cortical hypodensity (Figure ). Since his previous myocardial infarction, the patient had complaint with his aspirin and Plavix. He noted that approximately one month ago, he suffered a severe headache, but did not remember which side it was on, what precipitated this headache or how long it lasted. Review of systems was negative except for the angioedema and dysarthria. His labs on presentation were low-density lipoprotein (LDL) 123, HbA1c 7.2, hemoglobin and hematocrit of 20.9 and 65.7 with P2Y12 88. A magnetic resonance imaging (MRI) was performed, which showed no evidence of diffusion restriction to suggest recent infarction. Encephalomalacia was demonstrated involving the right parietal lobe secondary to sequela from remote infarction. Computer tomography angiography (CTA) was negative for any pathology including any significant stenosis. Transthoracic echocardiogram (TTE) showed a left ventricle (LV) with normal size and normal systolic function. LV ejection fraction was 55-60% with normal regional wall motion, normal diastolic function, and no LV thrombus. There was no evidence of atrial septal defect, ventricular septal defect or PFO. At this time the recommendations were cardiac event monitor and outpatient follow-up since symptoms were consistent with a transient ischemic attack (TIA) versus dysarthria secondary to the angioedema, both of which required no intervention. Approximate 36 hours after stroke team visit, the patient was found to have a right-sided facial droop and right-sided arm weakness with last known well reportedly overnight. NIHSS was 4 (right facial droop, dysarthria, right upper extremity drift) and stroke team was re-consulted. MRI showed interval development of diffusion restriction involving the left parietal lobe and there was associated signal hypo-intensity on the apparent diffusion coefficient map most characteristic of recent infarction (Figures , ). This acute left parietal small ischemic stroke was out of window for tissue plasminogen activator administration. Transesophageal echocardiogram (TEE) with saline contrast injection was performed at rest with good effort Valsalva which demonstrated at least 20 early appearing left heart bubbles, consistent with present of a large size PFO. Magnetic resonance angiography showed no evidence for pelvic or common femoral venous thrombosis. The stroke was attributed to a large inter-atrial communication defect with etiology being paradoxical embolus through defect. The patient was considered for transcatheter PFO closure, however, he opted for outpatient closure of defect. He was sent home with close follow-up and event monitor together with atorvastatin and dual antiplatelet therapy.
pmc-6318108-2	A 66-year-old Caucasian male with past medical history significant for hypertension and hyperlipidemia presented with altered mental status. Symptoms at presentation included slurred speech. He was found walking around his yard and his neighbor noted his slurred speech. His last know well state was the day before in the evening hours. Upon arrival, the blood pressure was found to be at 150/70 and his laboratory workup including complete blood count, complete metabolic profile and urinalysis were normal. Urine drug screen was positive for cannabinoids and serum alcohol level was not detectable. CT of the head showed age-related atrophy and mild small vessel ischemic changes without acute intracranial bleeding (Figure ). Questionable loss of gray-white junction and edema was found in the left frontoparietal region. On physical exam, the patient was confused but had normal movements and strength. He denied use of any tobacco products. TTE showed a normal left ventricle size, thickness, and function. The ejection fraction was estimated at 60% with normal right ventricular structure and function. There was a small PFO with predominant left-to-right shunting visualized. MRI showed an acute left middle cerebral artery territory infarction without midline shift or hemorrhagic conversion (Figure ). CTA showed the right internal carotid artery with estimated stenosis of 30-50% and the left internal carotid artery with stenosis estimated at 30-50%. TEE showed the PFO previously demonstrated and an ejection fraction of approximately 60%. Ultrasound of lower extremities failed to show deep vein thrombosis. The patient continued receiving neuro checks during the length of the stay and was started on antiplatelet therapy and atorvastatin with an outpatient follow-up and discharged with an event recorder and possible percutaneous closure of PFO after conduction abnormalities were ruled out.
pmc-6318108-3	An 18-year-old Caucasian female with past medical history significant for Raynaud’s phenomena and vascular headaches, which have never been associated with any neurologic abnormalities, presented after an episode of not being able to articulate and not being able to feel her right arm and the right side of her face. During this episode, the patient had a unilateral frontal headache and a brief episode of lightheadedness. The episode lasted two minutes after which she returned to baseline. The patient was on oral contraceptive pills until this episode. On examination, the patient did not have any significant physical findings. TTE showed a normal ejection fraction of 65% with normal diastolic function and the presence of a PFO. CTA brain with and without contrast did not show any stenosis, dissection, or aneurysm. MRI of the brain with and without contrast showed no acute intracranial findings. The patient underwent the placement of an Amplatzer closure device and was followed up with repeat TEE which showed an ejection fraction greater than 55% and no evidence of spontaneous echo contrast in the left atrium. Repeat echocardiography showed that the Amplatzer device was in good position. On follow-up visits, the patient did not have any new episodes of a cryptogenic stroke. The patient had the last echocardiography done in February 2018, which showed that the ejection fraction was greater than 65% and that the atrial septal occluder device was in a stable position with no residual shunting. There was no evidence of device migration. The remaining valves were grossly normal. Since the placement of the closing device, the patient has been doing well without any residual neurologic defects or repeated episodes of TIA or stroke. RoPE score measured for this patient was 9 with an 88% chance of stroke being due to a PFO.
pmc-6318109-1	A 57-year-old man, who was diabetic, hypertensive and had a family history of ischemic heart disease, presented to the cardiology clinic at the Aga Khan University Hospital, Karachi, Pakistan. He had a history of retrosternal chest burning after meals and occasional chest heaviness at rest with no relationship to exertion. He had been complaining of a dry cough for the past four to five days. He was functional class I. On clinical examination, he had a blood pressure of 138/84 mmHg and a heart rate of 76 beats per minute, with an oxygen saturation of 98% on room air. Cardiac auscultation revealed S1 and S2 with no added heart sounds. Chest examination revealed normal vesicular breathing. Rest of the systemic examination was also within normal limits. Baseline ECG showed normal sinus rhythm with LBBB. Blood workup included complete blood count, creatinine, and electrolytes which were within normal ranges. Two sets of troponin I conducted from the clinic were negative. He was then referred for a myocardial perfusion scan with dipyradimole for ischemia assessment. Baseline ECG is shown in Figure -. As per our institutional protocol, same day rest single photon emission computed tomography (SPECT) imaging was obtained with 760 MBq of intravenous (IV) Tc-99m tetrofosmin. At the start of the stress part of the test, the patient had a bout of dry cough. The ECG monitor documented a conversion of the LBBB to narrow complex after the patient coughed as shown in Figure -. He was then asked to cough again which showed conversion of narrow complex rhythm back to LBBB as shown in Figure -. Then 0.56 mg/kg of IV dipyradimole was injected followed by IV 270 MBq of Tc-99m tetrofosmin for stress SPECT imaging. IV aminophylline was given as per the protocol of myocardial perfusion imaging followed at our center. Neither the IV medications nor the injection of the isotope changed the LBBB to narrow complex rhythm. The patient was then sent for myocardial perfusion imaging. The myocardial perfusion scan was found to have a slightly reduced count in the anteroseptal region likely due to the LBBB as shown in Figure . The gated images revealed a calculated ejection fraction of 60% in stress with anteroseptal hypokinesis (not shown). He was started on oral antacids and oral proton pump inhibitors. It was decided to follow the patient for any recurrent symptoms or development of high-risk features which would necessitate a coronary angiogram. On two months follow-up, the patient remains asymptomatic and can easily walk more than four flights of stairs with no complaints.
pmc-6318110-1	A 26-year-old-female, with no significant medical history and who had recently emigrated from Kosovo, Serbia, presented to the emergency department (ED) with complaints of a cough, chest pain, and shortness of breath for two weeks. She initially presented to urgent care, where she was diagnosed with pneumonia, prescribed azithromycin and sent home. Her symptoms did not resolve and she became more dyspneic despite oral antibiotics, which prompted her to come to the ED. Upon presentation, her vital signs were notable for a fever to 102 degrees Fahrenheit and a blood pressure of 92/60 mm Hg. The physical examination showed decreased breath sounds on the right on auscultation and dullness on percussion. The chest X-ray was notable for significant right-sided pleural effusion (Figure ). Due to the size of the effusion and worsening respiratory status, emergent tube thoracostomy was performed, which drained the effusion significantly (Figure ). The patient was started on ceftriaxone and doxycycline for presumed pneumonia. Pleural fluid showed lactate dehydrogenase (LDH) 314 IU/L (compared with serum LDH 129 IU/L), and protein 5.2 g/L (compared with serum protein 5.5 g/L), indicative of exudative pleural effusion. Adenosine deaminase level was 1.7 units/L (N: 0-9.4 units/L) and a white blood cell (WBC) count of 1673 with 61% lymphocytes. Due to suspicion for TB, three acid-fast bacilli (AFB) sputum smear and cultures, collected eight hours apart, with one early morning sample, were sent. All the three sputum specimens came back negative for AFB. Even the first sputum cultures after six weeks were negative. Three samples were sent for an MTB/RIF assay (real-time PCR), which were also negative. Repeat chest X-ray showed decreased opacity in the right mid to upper chest with an appearance of increased effusion at the right lateral base. The computed tomographic (CT) scan of the chest was positive for pleural thickening and multiloculated pleural effusion (Figure ). Due to high suspicion for TB, the patient underwent right video-assisted thoracoscopic surgery (VATS) with decortication and biopsy. The histology from the pleural rind and a pleural nodule showed necrotizing caseating granuloma. At this point, the data were consistent with a diagnosis of pulmonary TB and the patient was placed on airborne isolation and started on standard anti-tubercular therapy with isoniazid, rifampin, pyrazinamide, ethambutol, and pyridoxine. Liver function tests were normal before initiating the therapy. After a week of therapy, the patient showed significant improvement in her respiratory symptoms. The Department of Health (DOH) was notified. As per their recommendations, three additional sets of sputum were sent for AFB, all of which came back negative, including a negative second set of sputum cultures six weeks later. The patient was discharged per DOH guidance, as she was not contagious. She continued anti-tubercular therapy as per standard protocol with close monitoring by DOH and outpatient infectious disease follow-up. The pleural biopsy from VATS was initially negative for gram stain and later came back positive for Mycobacterium tuberculosis, confirming the diagnosis of tuberculosis.
pmc-6318115-1	A 44-year-old Caucasian male with a past medical history of resistant hypertension and aortic dissection (Type B) was brought to the emergency department after he was found unresponsive in his apartment next to a suicide note. Medication history included lisinopril, clonidine, chlorthalidone, labetalol, and nifedipine. His vital signs on admission were: blood pressure (BP) 132/68 mmHg, heart rate (HR) 54 beats per minute (bpm), respiratory rate (RR) 12 breaths per minute, saturation O2 (SatO2) 98% on a non-rebreather mask. His level of consciousness was severely depressed, with Glasgow Coma Scale (GSC) 6. He was intubated for airway protection and given Narcan intravenously without significant changes in his vital signs or mental status. The chest X-ray was unremarkable, except for a marked widening of the mediastinum (a known history of Type B aortic dissection). A computed tomography (CT) scan of the head without contrast was also unremarkable. Repeat vital signs 30 minutes later were: BP 67/30 mmHg, HR 54 bpm, RR 16 breaths per minute, and SatO2 99% on mechanical ventilation. The patient received 3 liters of normal saline intravenously along with 5 mg intravenous (IV) atropine followed by glucagon IV push and calcium gluconate IV push. After initial therapeutic interventions, his BP dropped to 45/25 mmHg and HR to 48 bpm. He was started on dopamine IV for hypotension and bradycardia and admitted to the medical intensive care unit (MICU). Central and arterial lines were placed for the administration of the vasopressors and continuous BP monitoring, respectively. Despite the initiation of dopamine infusion, the patient continued to remain hypotensive (Figure ). Subsequently, four other vasopressors were added at their maximum infusion rates (Figure ). In light of a suspected severe overdose with hypotension refractory to the infusion of vasopressors, the decision was made to initiate lipid emulsification therapy (LET) along with high-dose insulin infusion (HDI) (Figure ). To prevent hypoglycemia, the patient was started on 5% and then 10% dextrose infusions. No hypoglycemia was recorded. On Day 2, the patient developed an acute kidney injury with non-anion gap metabolic acidosis. He was started on an IV bicarbonate drip. On Day 3, his hemodynamic parameters significantly improved: four out of five vasopressors were weaned off (remained only on dopamine). On the same day, IV insulin (with dextrose) and bicarb infusions were discontinued as well. On Day 4, the patient was off all vasopressors, and he was extubated on Day 5. He was transferred to inpatient psychiatric rehabilitation on Day 7.
pmc-6318116-1	An 81-year-old Caucasian woman presented to the hospital with chief concerns of shortness of breath and productive cough associated with generalized weakness, fatigue, and decreased appetite for a few weeks before presentation. Her medical history was significant for smoking one pack per day for 50 years and chronic obstructive pulmonary disease on two liters of home oxygen via nasal cannula. On physical examination, the patient was afebrile, tachypneic, tachycardic, had bilateral lung crackles more pronounced on the right, and was hypoxic at 86% on two liters of supplemental oxygen. Laboratory testing revealed an elevated leukocyte count of 15.82 k/µL with 91% neutrophils. Serum electrolytes, kidney function, and liver function tests were within reference ranges. The initial chest x-ray showed a right upper lobe opacity with pleural thickening, scarring, right lung volume loss, and bronchiectasis. The patient was started on azithromycin and cefepime for community-acquired pneumonia and sepsis, she was started on non-invasive ventilation via bilevel positive airway pressure for acute hypoxic respiratory failure and admitted to the medical floor. Further imaging with computed tomography (CT) scan of the chest showed a right upper-lobe thick-walled cavitary lesion measuring 2.1 cm x 4.3 cm x 3.1 cm with associated bronchiectasis and pleural parenchymal scarring (Figure , Figure ). One year ago, the patient underwent bronchoscopy for right upper lobe cavitary lesion that incidentally revealed M. xenopi on bronchoalveolar lavage (BAL) culture. BAL was negative for Mycobacterium tuberculosis (TB) and fungal infection. BAL cytology was also negative for the presence of malignant cells. At that time, her condition was stable and M. xenopi was considered a commensal. Her QuantiFERON-TB Gold test was indeterminate, and tests for serum galactomannan antigen and serum Aspergillus antibody were negative. The legionella urine antigen and HIV test results were also negative. During current admission, the patient was started on rifampin, isoniazid, ethambutol, and clarithromycin because M. xenopi was clinically significant, causing symptomatic respiratory failure. The patient’s condition improved over the course of the hospital stay, and, subsequently, she was discharged on oral rifampin, isoniazid, ethambutol, and clarithromycin for at least six months of therapy and a possibility of prolongation of treatment based on clinical and radiological improvement. One month after discharge, while on treatment, the patient was readmitted for shortness of breath and hypotension, tachypnea, and tachycardia. On a repeat CT scan of her chest, we noted right upper lobe consolidation with slight interval enlargement of the right upper lobe cavitary lesion (Figure ). The patient was given broad-spectrum antibiotics for possible bacterial superinfection. The patient improved clinically and was discharged. The patient was advised to have close outpatient follow up with the infectious disease and pulmonary teams.
pmc-6318117-1	A 37-year-old female from Saudi Arabia, non-smoker and non-alcoholic, with no significant history of any previous medical problems presented to the emergency department (ED) at King Fahd Medical City, Riyadh, Saudi Arabia, with a history of fluctuation in her consciousness level for two days. She had a generalized vesicular skin rash for the past one month before this presentation, which required admission to another hospital for two weeks. There, she was diagnosed with chicken pox and received local creams and oral antibiotics (Augmentin). A few days following discharge, she started complaining of a headache, blurring of vision, generalized weakness, a deteriorating level of consciousness, and a worsening skin rash. Upon presentation to our ED, her Glasgow coma scale (GCS) was less than 8; she was intubated and mechanically ventilated in the emergency room. She was hemodynamically stable with a generalized maculopapular, vesicular rash all over her body with some desquamation areas (Figure ). The patient was admitted to the intensive care unit (ICU), intubated, ventilated, and an external ventricular drain (EVD) was inserted because of high intracranial pressure on imaging with the clinical diagnosis of bilateral posterior cerebral artery (PCA) strokes and cortical blindness. Multiple skin biopsies were negative for vasculitis as was the hypercoagulability screen. A skin biopsy showed the presence of subepidermal vesicles, with a scanty intravesicular inflammatory cells infiltrate and linear C3 staining at the basement membrane zone, suggestive of a drug eruption with no evidence of vasculitis. The patient did not have any history of seizures, abnormal movement, behavioral changes, or any flu-like symptoms. She had multiple abortions, the last one being five months before the present illness. There was no history of a similar condition, strokes, or thrombotic events in the family or contact with a sick patient. Upon admission, MRI brain showed diffuse abnormal T2/fluid-attenuated inversion recovery (FLAIR), hyperintensity involving both cerebellar hemispheres, the inferior vermis, the posterior aspect of the left temporal lobe, both occipital lobes, the left thalamus, and the splenium of the corpus callosum with restricted diffusion (Figures -). A similar abnormal signal intensity involved the dorsal aspect of the pons, right middle cerebellar peduncle, and posterior aspect of the bilateral parietal lobes. These findings were consistent with acute infarction. Blooming foci in the right cerebellum hemisphere and inferior vermis denoted a micro-bleed and foci of a high T1 signal intensity within the bilateral occipital and posterior left temporal lobe, denoting microhemorrhages. There was a mass effect evident by cerebellar tonsils herniation through the foramen magnum about 28 mm, with an effacement of the basal cisterns and fourth ventricle, which resulted in supratentorial hydrocephalus and trans-ependymal permeation. A magnetic resonance angiogram (MRA) of the brain showed a marked attenuation of the V4 segment of vertebral arteries as well as the anterior inferior cerebellar artery (AICA), superior cerebellar, and posterior cerebellar artery bilaterally with an attenuation of the supraclinoid segment of the middle cerebral artery (MCA) bilaterally. She was extubated after a few days and shifted to the high dependency unit as her conscious level improved. The patient was evaluated by the ophthalmology team and labeled to have cortical blindness related to a bilateral PCA stroke. The patient was on prednisolone oral dose of 60 mg on admission, which was gradually tapered. A meningeal biopsy was offered to the patient but refused by the family. The patient started to walk with minimal assistance; however, bilateral cortical blindness limited her mobility. She was later discharged on a tapering dose of prednisolone and aspirin 81 mg daily. On discharge, the patient showed significant improvement in the eruptive skin rash, which dried out with some areas of crusting. Upon follow-up in the outpatient clinic after three months, a repeat MRI of the brain was done (Figures -), which revealed a loss of volume and encephalomalacia within the left occipital lobe, cerebellum, and thalamus as well as the corpus callosum with the exo-vacuo lateral ventricle representing sequelae of the previous infarction. It also showed foci of high signal intensity in T2 FLAIR within the white matter and resolution of the left thalamic lesion. Clinically, the patient improved significantly, was completely independent in her activities of daily living (ADLs), and her vision improved significantly to the point that she was planning to return to her job as a teacher.
pmc-6318128-1	A 54-year-old male with previously diagnosed human immunodeficiency virus (HIV) had recurrent presentations for colitis since initiating emtricitabine/tenofovir. In the past, he had self-discontinued this medication, resulting in the resolution of gastrointestinal (GI) complaints, but subsequent re-initiation of the medication led to a recurrence of symptoms and hospitalization. The abdominal computed tomography (CT) scan reported focal colitis in the descending colon, as seen in Figure . The patient was started on empiric antibiotics with a progressive worsening of symptoms. He was taken to the operating room for exploratory laparoscopy, resulting in colonic resection and diverting colostomy. The pathological specimen, as seen in Figure , demonstrated eruptive pseudomembranes, edema, and mixed inflammation, including numerous eosinophils within the colon wall consistent with eosinophilic colitis. Upon reviewing his laboratory and microbiological data, it was noted that the patient did not have peripheral eosinophilia and that stool cultures, fecal leukocytes, and stool ova and parasites were all negative. However, an elevated C-reactive protein (CRP) of 37.6 mg/L was noted on admission that trended up to 61.0 mg/L. Outpatient screening for HLA-B*57:01 was negative and his previous HIV medication regimen was switched to the combination of abacavir, dolutegravir, and lamivudine. No symptom recurrence has been noted since the treatment regimen was adjusted.
pmc-6318130-1	A 60-year-old female patient, status-post maxillectomy for a case of mucormycosis, reported to the Department of Prosthodontics with the chief complaint of missing teeth in the maxillary and mandibular arch that affected her aesthetics and masticatory function. The patient also complained of nasal regurgitation of food and hypernasality of voice. At the time of presentation, the patient was demoralized and psychologically unstable. The maxillectomy adversely affected the patient’s psychological state as she failed to communicate and follow commands, along with unintelligible speech. On extraoral examination, facial asymmetry was found. Her past medical history revealed that the patient had uncontrolled type II diabetes mellitus for which she had been on medication for the past 15 years. The patient was malnourished and was taking nutritional supplements for the same. The patient was diagnosed with mucormycosis of the left maxillary sinus a year earlier for which she underwent surgical debridement one month prior to presentation. Intraoral examination revealed a completely edentulous maxilla and mandible and an oronasal fistula on the left side of the maxilla. The oral side of the defect extended into the buccal vestibule and lateral to the left hard palate with an adequate amount of alveolar ridge overlying the defect (Figure ). Prosthodontic intervention After discussing the possible treatment options with the patient, considering her age and medical condition, fabrication of a conventional complete denture prosthesis that would restore aesthetics and function, as well as obliteration of the fistula, was planned. A maxillary and mandibular preliminary impression was taken with irreversible hydrocolloid using a stock tray (after packing the defect with gauze to prevent impression material from entering the nasal cavity). An impression was poured with dental stone Type IV and a custom tray was fabricated using auto polymerizing acrylic resin. Border molding was done with green stick compound, and a second impression was made with elastomeric impression material after blocking the defect with gauze. In the master cast, the vestibular and palatal defect was blocked with wax to relieve undercuts before fabrication of the denture base and occlusal rim. Tentative jaw relation was done and transferred to a mean value articulator for artificial teeth arrangement. During the wax try-in appointment, the patient's centric relation, esthetics, and phonetics were assessed. A functional impression was made using the trial denture maintaining the correct vertical dimension at occlusion. It was then processed with heat-cured acrylic denture base material (Figure ). During the denture insertion appointment, the patient's occlusion, phonetics, and esthetics were checked, and the patient was asked to drink water to check for nasal regurgitation of fluids (Figure ). Post-insertion instructions on proper use, care, and maintenance of the prosthesis were given to the patient. The patient was reviewed after 24 hours and once every two weeks for the next three months (Figure ). During the follow-up visits, the patient was reevaluated for minor adjustments. The OHIP-14 questionnaire was evaluated pre- and post-complete denture treatment. The details of the treatment were explained, and informed consent was taken from both the patient and the patient’s attender, as the patient was depressed and psychologically unstable. At the end of three months, a second OHIP-14 questionnaire was recorded. The OHIP-14 questionnaire was used to assess the impact of prosthodontic treatment on the OHR-QoL of the patient. The pre- and post-treatment responses of the OHIP-14 questionnaire are tabulated (Table ) [, ].
pmc-6318132-1	In our initial case report [], we described the case of a 10-year-old boy with a history of chronic hepatitis B, whose parents also had chronic hepatitis B, who was diagnosed with hepatocellular carcinoma (HCC) with a central hepatic mass 43.5 cc in volume. At diagnosis, he had metastatic disease in a celiac lymph node. His tumor was deemed unresectable due to the location and lack of clear margins, while he was also not a candidate for liver transplantation, doxorubicin-containing systemic chemotherapy, radiofrequency ablation, radioembolization, or transarterial chemoembolization. Given the paucity of therapeutic options, he was treated with stereotactic body radiotherapy (SBRT), with his primary site receiving 45 Gy in five fractions and the celiac nodal region receiving 35 Gy in five fractions (Figure ). After completing radiotherapy, his total bilirubin level fell below 1.0 mg/dL, allowing him to begin systemic therapy following the SIOPEL4 Block A2 regimen with cisplatin 70 mg/m2 and doxorubicin 30 mg/m2 [] which he received for one cycle. Our previous report concluded at three months follow-up, when his bilirubin was 0.1 mg/dL, alpha-fetoprotein (AFP) was 88 ng/mL, and imaging demonstrated a decrease in tumor size (total volume 28.7 cc), with no evidence of local or distant disease progression. Repeat magnetic resonance imaging (MRI) of the abdomen performed four months after completion of SBRT showed a further decrease in the size of the central hepatic mass, now measuring 2.8 x 2.4 cm compared to the previous measurement of 3.8 x 3.5 cm. Repeat imaging performed six and seven months after completion of radiation continued to show mild decrease in the size of the mass to a size of 2.2 x 1.8 cm. However, 8.5 months after completion of SBRT, an abdominal MRI demonstrated the central hepatic mass stable in size but also showed an enlarging secondary hepatic lesion (1.3 x 1.2 cm, previously 0.7 x 1.0 cm) as well as new additional scattered lesions in the right peripheral lobe of the liver, which measured 1.0 cm, consistent with disease progression. Due to the prior SBRT, he was not a candidate for radioembolization. The liver transplant committee reviewed his case but deferred listing him for liver transplantation because he had presented with extrahepatic disease and thus failed to meet the United Network for Organ Sharing (UNOS) criteria for transplantation and also due to concern that the original tumor may have contained an element of cholangiocarcinoma, thus portending a poorer prognosis []. The committee agreed to reconsider the option of transplantation if repeat biopsy failed to show cholangiocarcinoma and positron emission tomography (PET) imaging did not detect metastatic disease. The patient underwent interventional radiologic (IR)-guided biopsy of the hepatic lesions, but pathology was inconclusive as to whether the process represented HCC or cholangiocarcinoma. Therefore, he was not considered a candidate for hepatic transplantation. However, the PET imaging demonstrated non-avidity, favoring a diagnosis of HCC. He subsequently began systemic chemotherapy with an individualized protocol of gemcitabine 1000 mg/m2 and oxaliplatin 85 mg/m2. Repeat MRI abdominal imaging following two cycles of chemotherapy demonstrated interval growth of the previously noted smaller hepatic lesions, as well as appearance of a new lesion within the left hepatic lobe, though notably the original SBRT-treated lesion did not grow. Given imaging findings suggestive of disease progression, gemcitabine/oxaliplatin was discontinued and the patient was switched to systemic therapy with vincristine 2 mg, irinotecan 50 mg/m2, and temsirolimus 35 mg/m2 (VIT), as per AHEP0731, Regimen H []. Repeat abdominal imaging after cycles two, four, and six of VIT demonstrated stable disease. AFP levels also declined throughout treatment (51 ng/ml post-cycle two, 37 ng/ml post-cycle four, 38 ng/ml post-cycle six). The liver transplant committee was consulted again after the patient completed cycle eight of VIT and decided to move forward with a laparoscopic biopsy of a suspicious portocaval lymph node to determine if there was any active nodal disease present. The biopsy showed no sign of carcinoma and the patient was thus listed for consideration of hepatic transplantation with a Pediatric End-Stage Liver Disease (PELD) score of 40. Two weeks later, the patient underwent orthotopic liver transplant (OLT) and had an uneventful operative course. However, his transaminase levels rose acutely during the first several postoperative days (POD); thus, he underwent liver biopsy on POD nine, which failed to confirm rejection and was more consistent with preservation injury, defined as hepatic dysfunction caused by cold or warm ischemia. His postoperative course was further complicated by influenza B infection and significant acute kidney injury (AKI) secondary to nephrotoxic medications of vancomycin and tacrolimus. Over the following two months, he completed cycles nine and ten of VIT. Follow-up imaging performed two months after OLT showed evidence of complete remission. He remains with no evidence of disease seven months after OLT. He continues on tenofovir with hepatitis B DNA undetectable by polymerase chain reaction (PCR).
pmc-6318133-1	A 23-year-old male with no previous history of disease was admitted to the hospital with complaints of swelling, pain, and loss of sensation on the right side of the face. Physical examination and magnetic resonance imaging (MRI) revealed a mass extending from the inferior orbital rim to the gingivobuccal sulcus. A biopsy was performed, and the pathological examination was reported as poorly differentiated carcinoma with squamous differentiation (compatible with NUT midline carcinoma). Positron emission computed tomography (PET-CT) showed fluorodeoxyglucose (FDG) uptake in the bilateral level II and III lymph nodes and right anterior maxillary sinus. He underwent total maxillectomy and right radical neck dissection. Pathology revealed poorly differentiated carcinoma with squamous differentiation (compatible with NUT midline carcinoma). The tumor was 4 cm in the largest dimension, with perineural invasion. The resection margins were tumor positive, and the dissected 46 lymph nodes were negative for metastases. Postoperatively, he underwent adjuvant concomitant chemoradiotherapy. The prescribed doses were 66 Gy to the primary site and 57 Gy to the right neck levels in 33 fractions, with weekly cisplatin. The treatment was delivered using intensity modulated radiotherapy (IMRT) and it was well-tolerated with moderate acute side effects like grade 1 mucositis and dermatitis. A follow-up MRI scan three months after radiotherapy revealed a recurrent lesion in the right pterion and right orbit, 16 mm in the largest dimension. The PET-CT showed increased metabolic activity in the lateral side of the zygomatic bone, in the right spina scapula and right acetabulum, which were evaluated as bone metastases (Figure ). He received two cycles of docetaxel/cisplatin and six cycles of gemcitabine in combination with oxaliplatin (GEMOX); however, the PET-CT showed progressive disease with increased FDG uptake in the right medial orbit, right zygomatic bone, right acetabulum, right pubic ramus, right lung, and cervical lymph nodes as revealed in Figure and in data not shown. As a second-line treatment option, he was offered pembrolizumab. After four cycles of pembrolizumab, he had both a clinical and a radiological partial response (Figure ). The FDG uptake in the right zygomatic region and right maxillary region decreased. However, the response was not durable and the soft tissue mass progressed clinically after the sixth cycle. The PET-CT performed after the eight cycles showed progression in the soft tissue lesions in the right orbital and zygoma and the right scapula (Figure ). We performed HSRT with a total dose of 24 Gy, in three fractions to the orbital mass (Figure ). The fractions were delivered every other day. He continued pembrolizumab treatment after radiotherapy. Clinically, the tumor responded to radiation. Repeat imaging performed three months following the completion of radiation therapy showed a complete response in the orbital mass and in the metastatic lesion in the left sixth rib (Figure ). There was a significant reduction in the FDG uptake of the right acetabulum and right ischium. However, the right scapular lesion did not respond to treatment. We performed the same radiotherapy dose (24 Gy in three fractions) to this lesion and the follow-up imaging studies showed a complete response in all FDG avid areas.
pmc-6318133-2	A 29-year-old male patient presented with complaints of exophthalmos and swelling in his right eye, which started one month ago and increased gradually. After a detailed history and physical examination, a paranasal sinus CT and nasopharynx MRI was obtained. The images revealed a solid mass, approximately 4x3.4x4.1 cm in size. The right maxillary sinus was obliterated with the lesion, which also invaded the sphenoid bone. It extended into the middle crust in the nasal cavity and to the orbital cavity, eroding the bony structures of the apex and the medial wall of the orbit but not infiltrating the orbital muscle tissues. There was also an inferior temporal fossa extension. A biopsy was performed and the pathology was reported as a "basaloid type SCC." He refused surgery and received three cycles of docetaxel, cisplatin, and 5-fluorouracil (DCF) induction chemotherapy. The imaging studies showed minimal response to chemotherapy. He received HSRT to a total dose of 36 Gy with 7.2 Gy/fraction to the primary site. Follow-up images revealed minimal regression. He was followed without any medication with MRI performed every three months, and the disease was stable. Eight years after radiotherapy, the MRI showed progression in the intracranial and infraorbital compartments and the patient had a complaint of progressive exophthalmos. The MRI revealed a lesion extending into the orbit and the optic nerve swirled all the way, the temporal lobe compartment infiltrated the large wing of the sphenoid sinus, the cavernous sinus, and the Meckel cavity (Figures -). There was also dural infiltration in the temporal region. He refused surgery or chemotherapy and was offered nivolumab. After two cycles, we performed HSRT to the locally recurrent lesion with a fraction dose of 8 Gy to a total dose of 24 Gy, every other day. He is still receiving nivolumab and the follow-up images performed four months after radiotherapy revealed a significant response, and his complaint of exophthalmos resolved completely (Figures -).
pmc-6318134-1	A 44-year-old Hispanic woman presented with acute confusion. The family reported two months of progressive headaches and two weeks of fever, blurred central vision, and restricted visual fields. The review of systems was positive for recent nausea and vomiting. There was a remote history of treatment for latent tuberculosis and a recent history of a treated urinary tract infection. On examination, the patient appeared ill, with a low-grade fever (38.5°C) and stiff neck. She was somnolent and oriented only to self when aroused. Neurological testing was limited but grossly non-focal. A computerized tomography (CT) scan of the head and magnetic resonance imaging (MRI) of the brain showed a large cystic mass arising in the sella, where it displaced the normal pituitary gland. It extended over 1 cm above the tuberculum sella, compressing the optic chiasm (Figure ). There was considerable contrast enhancement of the cyst wall and of the overlying chiasm and adjacent hypothalamic region. The cerebrospinal fluid (CSF) analysis showed mildly elevated opening pressure (30 cm H2O), with high protein (104) and low glucose (29). There were 835 white blood cells (WBCs, 56% neutrophils). Extensive serum and CSF evaluation were negative for infectious agents. Hormonal assays revealed evidence of pan-hypopituitarism. CT scanning of chest, abdomen, and pelvis was unremarkable for metastatic etiology. The patient was initially started on vancomycin, ceftriaxone, acyclovir, and dexamethasone for presumed infectious meningoencephalitis. As tests for bacterial and viral pathogens were normal, she was switched to fluconazole. The mental status returned to normal and she wished to be discharged home on fluconazole and pituitary hormone replacements. She missed her follow-up and returned one month later with a recurrent headache, nausea, and stiff neck. The examination showed meningismus but was otherwise non-focal. MRI brain showed no change in the parasellar mass. Repeat CSF showed an even higher WBC count (2195 leukocytes, 69% neutrophils) and protein (238) with continued hypo-glycorrhachia (26). She underwent an uncomplicated transnasal transsphenoidal surgery for the removal of the sellar lesion (Figure ). Microscopic examination showed a keratin-filled cyst lined with squamous epithelium, typical of epidermoid origin (Figure ). Symptoms improved and she was discharged on hormone supplement alone. She was asymptomatic at her last follow-up appointment.
pmc-6318136-1	A 56-year-old female with a history of uncontrolled biopsy-proven bullous pemphigoid began treatment with cyclosporine 100 mg three times a day for a total daily dose of 300 mg. After five weeks of therapy, the patient complained of nervousness and shakiness, and the cyclosporine dose was decreased to 100 mg twice daily for a total daily dose of 200 mg. After an additional five weeks of cyclosporine therapy at 200 mg daily, the patient returned with complaints of the gradual onset of redness, swelling, and pain of her left hand over the past six weeks (Figure ). On examination, erythema and violaceous swelling were present on the patient’s left hand, most predominant over the dorsal and palmar aspects of the left second and third metacarpals, and left proximal and mid phalanges. There was tenderness to palpation and pain with movement of the left hand and phalanges. The patient described the associated pain as a burning sensation. Although erythromelalgia was considered, this was believed to be a local reaction, and the cyclosporine was continued as treatment for the patient’s bullous pemphigoid. Seven weeks later, the patient returned complaining of significantly worsened redness, swelling, and pain of the left hand with progression of involvement of the right hand. On examination, the erythema and edema involved the dorsal and palmar aspects of the left hand and phalanges, most severely affecting the left proximal phalanges. The right hand was also visibly erythematous with edema most prominently involving the right second proximal phalanx. The patient complained of worsened pain, described as “burning similar to a sunburn” that significantly worsened upon exposure to the sun or heat. The pain also worsened with passive and active movement of the hand or digits, as well as palpation of the affected areas. The patient endorsed improvement with exposure of the hands to cold temperatures, particularly describing relief of the pain while running the hands under cold water. At this time, the cyclosporine dose was decreased to 100 mg once daily with a diagnosis of erythromelalgia secondary to cyclosporine. It was hoped that a lower dose of cyclosporine could be used to treat the bullous pemphigoid, with resolution of the symptoms of erythromelalgia. Six weeks later, the patient returned for a follow-up to evaluate the erythromelalgia. The erythema and edema had significantly improved, with only minor swelling of the second and third phalanges of the left hand and the second phalanx of the right hand. The patient's symptoms improved following the transition to low-dose cyclosporine therapy (100 mg once daily). At an additional follow-up eight weeks later, the patient’s symptoms of erythromelalgia had completely resolved.
pmc-6318137-1	A previously healthy two-year-old male presented with two days of lethargy, progressive decrease in appetite, and emesis. On the day of admission, he developed altered mental status, loss of muscle tone, and perioral cyanosis. There was a concern for an unwitnessed seizure as the child appeared to be post-ictal on presentation to the emergency room where he was subsequently diagnosed with a large midline multi-cystic tumor producing obstructive hydrocephalus. Shortly after being transferred to the pediatric critical care unit (PICU), the child was noted to have unequal pupils with impending herniation. He was emergently endotracheally intubated and an external ventricular drain was placed with subsequent intraoperative cyst drainage. Magnetic resonance imaging (MRI) obtained when the child was stabilized revealed a large multi-cystic hypothalamic optic chiasm tumor. He underwent endoscopic cyst fenestration and subtotal resection of the mass. Pathology revealed low-grade juvenile pilocytic astrocytoma (WHO grade I). Pituitary function was evaluated pre-operatively and was relatively unremarkable, with normal thyroid function tests and a low random serum cortisol of 3.2 mcg/dL (2.9-17 mcg/dL). But the child was receiving high dose dexamethasone at the time. The peak cortisol level after stimulation with adrenocorticotropic hormone (ACTH) was normal at 21 mcg/dL. On post-operative day two, the child was noted to develop polyuria and hyponatremia with urinary output as high as 13 mL/kg/hour and a steep decline in the serum sodium from 135 mEq/L to 128 mEq/L over six hours. Based on a very elevated urinary sodium excretion (229 mEq/L), with increased urine output (>4mL/kg/hr), elevated urine osmolality (523 mOsm/Kg) with a low serum osmolality (270 mOsm/Kg), and low uric acid (0.7 mg/dL), a diagnosis of CSW was made. The child required urine output replacement with isotonic fluids (Na 154 mEq/L) as well as the addition of 3% sodium chloride infusion as high as 3 mL/kg/hour to maintain a serum sodium of at least 130 mEq/L. On post-operative day six, enteral sodium replacement as high as 13 mEq/kg/day was initiated to facilitate weaning off the hypertonic saline. The hypertonic saline was discontinued but serum sodium values on post-operative day nine decreased to 128 mEq/L, demonstrating that the child could not maintain serum sodium levels off intravenous supplementation. The child was also unable to consistently tolerate the high enteral doses of sodium. Fludrocortisone 0.05 mg twice daily was initiated on post-operative day nine. Within 30 hours (after three doses) of treatment, polyuria declined, and serum sodium was maintained above 135 mEq/L (Figures , ). Enteral sodium supplementation was successfully lowered to 3 mEq/kg/day. The child had no hypertension or hypokalemia associated with fludrocortisone administration. Once CSW was controlled, he underwent chemotherapy after subtotal resection of the tumor. Two months post-operatively; the child continues on fludrocortisone 0.05mg twice a day and oral sodium chloride at 3 mEq/kg/day with normal serum sodium and potassium levels.
pmc-6318138-1	This case report will focus on a 39-year-old male with a smoking history of one pack per day and regular alcohol intake of beer for over 20 years. He has had ongoing respiratory and abdominal symptoms which had been attributed to his smoking and drinking history. Starting in his early twenties, he began to have multiple episodes of mild upper respiratory tract infections and bronchitis on a yearly basis. He also experienced mild abdominal discomfort and nausea which would come and go sporadically. High-resolution computed tomography of the chest confirmed emphysematous changes of the lung as shown in Figures - below. His liver enzymes had been slowly trending up throughout the years. Over the span of a few months, his aspartate transaminase (AST) level increased from 52 units per liter (U/L) to 58 U/L. His alanine transaminase (ALT) level increased from 81 U/L to 86 U/L. The upper limit of normal for both AST and ALT levels fall in the low 40s U/L. Other markers of liver damage, including serum prothrombin concentrations and serum albumin, were not affected. His primary care physician instructed him to quit smoking and drinking, eat healthily, and exercise regularly. He was successful in making these lifestyle changes, but over the next few years, his liver enzymes remained elevated and his lungs continued to show persistent emphysema. There was little to no improvement in his overall symptoms. Due to these abnormal findings, he was tested for alpha-1 antitrypsin deficiency (AATD) and was found to be homozygous for the Z allele (PI*ZZ). His alpha-1 antitrypsin (AAT) level was 18, which is well below the protective threshold of 57. Once the diagnosis was confirmed, the patient was immediately started on weekly Prolastin infusions. Since his diagnosis, our patient has completed eight infusions of Prolastin and will continue to receive weekly infusions for as long as he is able to tolerate them in order to maintain normal concentrations of AAT. His AAT level continues to increase and is now closer to the protective threshold; his liver function tests have improved as well.
pmc-6318140-1	A 25-year-old male was brought to our emergency department following a road traffic accident in July 2018. The patient was intubated in view of poor score on Glasgow Coma Scale (GCS). He was hemodynamically stable. On clinical examination, the patient had bilateral decreased air entry and positive chest compression. There were no abdominal signs. On focused assessment with sonography in trauma (FAST) there was minimal free fluid in the abdomen. Contrast-enhanced computed tomography (CECT) of thorax and abdomen showed bilateral hemopneumothorax and a grade III liver laceration. Initial non-contrast computed tomography (NCCT) of brain showed no intracranial injury but later the patient was declared to have a diffuse axonal injury and was shifted to critical care unit for monitoring. The liver laceration was managed conservatively since the patient was hemodynamically stable. Hemopneumothorax was managed with bilateral intercostal drains. The patient’s GCS was persistently poor. Initially, the patient was started on enteral feeds through nasogastric tube and later a PEG tube placement was planned for the purpose of continuing enteral feeds. The procedure was performed while the patient was on endotracheal tube, and under intravenous (IV) sedation. A 20 Fr PEG tube was placed by the standard ‘Pull’ technique. Second look endoscopy confirmed the position of the internal bumper against the anterior wall of the stomach. Externally the tube was fixed and free flow of saline through the PEG was confirmed. There were no complications during the procedure and the patient was started on enteral feeds through the PEG tube on the same day. The patient was extubated after few days but he was continued on PEG feeds as his GCS was persistently poor. At four weeks after PEG there was peritubal leakage noted during feeds with resistance to the flow of feeds initially, which later progressed to complete the blockage. On examination, there was a slight bulge at the site of PEG tube insertion. There was granulation tissue visible sprouting through the tract externally (Figure ). The patient had no signs of peritonitis and the abdomen was soft. On flushing the tube with saline, peritubal leakage was noted and there was resistance to flow. Endoscopic examination was performed to visualize the position of the internal bumper. On endoscopy, the internal bumper was not visualized. Only a small dimple was seen in the mucosa of the anterior wall of the stomach (Figure ). The internal bumper appeared to have migrated through the tract and was entirely covered by the gastric mucosa with only a small dimple seen at the site of the tract. An ultrasound of the abdomen was performed which showed that the internal bumper was in the intramuscular plane of the rectus abdominis muscle (Figure ). As the patient’s GCS was still poor with no mature swallowing reflex, we planned to remove the old PEG tube and replace it with a new one for continued enteral feeding. The procedure was performed under general anaesthesia. Under fluoroscopic guidance, a guide wire was passed through the previous PEG tube from outside piercing the gastric wall. Then the old PEG was removed by gentle firm traction and the tract was dilated using dilator passed over the guide wire. The position of the dilator inside the stomach was confirmed by injecting a contrast dye under fluoroscopic visualization. Since the tract was well formed, a 20 Fr balloon replacement gastrostomy tube was inserted from outside and secured (Figure ). Once again the position was confirmed by fluoroscopy and free flow of saline. The patient was started on PEG feeds later on the same day and had no complications. The patient was followed up for a month and was on continuous PEG feeds without any complications.
pmc-6318142-1	A 32-year-old homeless man with a past medical history of ethanol use disorder (sober for 40 days prior to presentation) was brought in by emergency medical personnel after being found unresponsive in a Walmart parking-lot. After regaining consciousness, the patient stated that the last thing he remembered was “huffing” six to ten cans of the computer cleaning product, Dust-Off. He endorsed sharp, substernal chest and anterior neck discomfort made worse by deep inspiration. Initially, he was given two amps of bicarbonate, as well as empiric antibiotics. Physical exam was unremarkable with the exception of what appeared to be frost-bite on his fingertips of the right hand. Laboratory work was notable for leukocytosis of 17.7 thousand per microliter (K/uL), sodium of 127 millimoles per liter (mmol/L), potassium of 3.5 mmol/L, chloride of 90 mmol/L, bicarbonate of 20 mmol/L, creatinine of 1.63 milligram per deciliter (mg/dl), normal liver enzymes and a negative toxicology screen. Troponin was elevated to 4.34 nanograms per milliliter (ng/mL), creatinine kinase was 1163 units per liters (U/L) and creatinine kinase-muscle/brain (CK-MB) was 21.3 ng/mL. His initial electrocardiogram revealed sinus rhythm with a partial right bundle branch block and a prolonged corrected QT segment (QTc) of 518 milliseconds. On chest X-ray, the patient was found to have pneumomediastinum and pneumopericardium (Figure ). Computed tomography of the chest demonstrated extensive air around the tracheal and laryngeal structures, extending down the cervical tissue planes and into the mediastinum and pericardium (Figure ). Cardiothoracic surgery was consulted and the decision was made to observe the patient as he remained hemodynamically stable and he was protecting his airway. The patient did well, and prior to discharge, his kidney function improved, he had a normal echocardiogram, normalization of his electrocardiogram, and minimal residual mediastinal and pericardial air on repeat chest X-ray. He was provided extensive education and support for cessation of inhalant and alcohol use.
pmc-6318143-1	A 73-year-old male with a past history of antineutrophil cytoplasmic antibody (ANCA) vasculitis, and end-stage renal disease on hemodialysis presented with acute onset hemorrhagic lesions for a day. He had no prior allergies. Two days before the current presentation, he had undergone a computed tomography (CT) scan of the abdomen with intravenous radioiodine contrast for evaluation of an acute episode of abdominal pain. Soon after administration of the radioiodine contrast, he developed generalized hives which resolved with anti-histamines. However, over the next 24-hour period he developed bullous hemorrhagic rash which initially began at the nape of his neck and later centrifugally spread to his face, chest, and back (Figures -). Rashes were associated with fatigue, photophobia, and fever. On examination, he had an oral temperature of 101.1°F (normal = 97°F-99°F) with multiple well-demarcated tender hemorrhagic bullae and plaques. Laboratory workup was significant for leukocytosis of 12,000 per microliter of blood (normal = 4,000 and 11,000 per microliters of blood), chronic stable thrombocytopenia of 88,000 microliters of blood (normal = 150,000-450,000 platelets per microliter of blood), elevated sedimentation rate of 33 mm/hour (normal = 0-22 mm/hour for men), elevated C-reactive protein of 18 mg/dL (normal </= 3 mg/dL), and low complement C3. Due to a history of ANCA vasculitis, he was re-evaluated and found to have a positive perinuclear ANCA and >100 U myeloperoxidase antibody. Dermatology was involved and a shave biopsy of the skin lesion measuring 0.7 cm x 0.7 cm x 0.1 cm was obtained. On hematoxylin and eosin (H&E) stain, pathology was significant for neutrophils admixed with nuclear debris and collagen degeneration spanning throughout the dermis with focal degeneration and separation of epidermis from underlying papillary dermis (Figures -). Infectious workup including blood cultures for bacteria, fungi, special stains for skin biopsy with Grocott’s methenamine silver stain, mucicarmine and immunochemistry for cryptococcus, herpes simplex, and bacteria were all negative. Thus, SS was established on clinical and histopathological basis. The patient was managed with high-dose prednisone course of 40 mg/day for a week with complete resolution of his skin manifestations. He had an uneventful recovery and was discharged home safely.
pmc-6318145-1	A 49-year-old man with a history of spindle cell sarcoma status post left arm below-elbow amputation developed recurrence of the sarcoma three years post amputation. He was found to have metastasis of cancer to the lungs on staging (Figure ) and received three cycles of Doxorubicin and Olaratumab followed by wide excision of the soft tissue tumor. He underwent a transthoracic echocardiogram (TTE) six months after completion of Doxorubicin therapy, and his LVEF was found to be normal at that time. Unfortunately, he continued to have progression of pulmonary metastasis and hence was initiated on Pazopanib, which is a second line therapy for advanced soft tissue sarcomas. Shortly after initiation of therapy, the patient started developing palpitations, shortness of breath on exertion and chest tightness. His symptoms progressively worsened, until he suffered a syncopal episode 10 days after initiation of Pazopanib. On admission to the hospital, the patient’s physical examination was unremarkable. The laboratory data was remarkable for an elevated troponin I to 0.09 ng/dl on admission. His electrocardiogram (ECG) revealed nonspecific ST segment changes (Figure ). As part of the workup for his syncope, he underwent a TTE, which revealed an LVEF of 10% to 15% with severe diffuse hypokinesis, right ventricular systolic dysfunction with normal biventricular chamber sizes. He subsequently underwent cardiac catheterization which revealed normal coronary anatomy (Figure ). He was initiated on guideline directed medical therapy with a beta blocker and angiotensin converting enzyme inhibitor and discharged with cardiology and oncology follow-up. His Pazopanib was stopped on discharge. The patient subsequently underwent repeat TTE six weeks from discharge, which revealed recovery of LVEF to 40 to 45% with normal cavity size and mild diffuse hypokinesis. A repeat TTE three months afterwards revealed an LVEF 45 to 50%. He was subsequently started on immunotherapy with Ipilimumab and Nivolumab combination therapy.
pmc-6318159-1	A 75-year-old Japanese man presented with a 1-month history of epigastric discomfort. He had a medical history of pulmonary tuberculosis treated with antitubercular agents, but his respiratory function tests were normal. On close examination, he was diagnosed with squamous cell carcinoma with a basaloid carcinoma-like component of the esophagus. The preoperative diagnosis was clinical T3N0M0 stage IIA lower esophageal cancer, according to the Union for International Cancer Control, seventh edition. The patient underwent preoperative chemotherapy (5-fluorouracil and cisplatin), but a second preoperative course was canceled because of the deterioration of his renal function to creatinine 1.34 mg/dL after the first course. He underwent video-assisted thoracoscopic esophagectomy in the left lateral position with three-field lymph node dissection and hand-assisted laparoscopic surgery. His thoracic duct was preserved without apparent injury. The surgical time was 394 min, and the intraoperative blood loss was 430 ml. Tube feeding was started from the second postoperative day. Thoracic drain fluid increased to 600 ml on postoperative day 5, and its appearance became milky. Pleural effusion triglyceride levels were 111 mg/dl. The patient was diagnosed with chylothorax, and enteral nutrition was discontinued. However, despite total parenteral nutrition (TPN), the chylothorax continued, and pleural effusion increased to > 1500 ml/day. He underwent lymphography for diagnostic and therapeutic purposes on the ninth postoperative day. We injected patent blue subcutaneously into the left acrotarsium, under local anesthesia. The lymphatic vessels were visualized, and we inserted a 27-gauge needle into one of the lymphatic vessels and injected Lipiodol at 0.1 ml/min. Videofluoroscopy showed lymphatic vessel enhancement up to the pelvis after 5 ml of Lipiodol, and a further 3.5 ml was injected at 0.14 ml/min. Computed tomography (CT) showed enhanced supraclavicular lymphatic vessels (Fig. ), but no lymphatic leakage was detected. No sign of aspiration was observed during lymphography. The patient developed chills, a fever (39 °C), and hypoxia 2 h after lymphography and was treated with oxygen administration and piperacillin-tazobactam for Lipiodol pulmonary embolism and prevention of secondary pneumonia. His hypoxia improved but intermittent fever continued after 4 days. Though his pleural effusion decreased to 200 ml/day on the 11th postoperative day, his hypoxia deteriorated suddenly on the 14th postoperative day, requiring artificial respiration. Blood gas analysis showed PaO2 85.3 mmHg with FiO2 0.6 and positive end-expiratory pressure 12 cmH2O. CT revealed high-density substance in the lung (Fig. a) and bilateral ground-glass opacity (Fig. b). We diagnosed severe ARDS, according to the Berlin definition, and started sivelestat sodium hydrate and lung-protective ventilation with low tidal volumes and prone position. Prednisolone 20 mg was added on the 21st postoperative day to prevent pulmonary fibrosis, without success, and he underwent a tracheotomy on the 28th postoperative day. He was weaned from mechanical ventilation on the 50th day but still required oxygen. He was finally discharged on the 112th day, with domiciliary oxygen therapy for hypoxemia due to pulmonary fibrosis (Fig. ).
pmc-6318841-1	A 67-year-old man complained of edema in both lower extremities. His medical history revealed that he had been diagnosed with stage I clear cell renal cell carcinoma (RCC) and underwent radical nephrectomy 8 years ago. Three years after the surgery, the cancer recurred and metastasized to the lungs and pancreas. The metastatic cancer was removed by performing pylorus-preserving pancreaticoduodenectomy and right upper lobectomy. The resected lung and pancreas indicated clear cell RCC. After metastasectomy, the cancer was classified as stage IV; he was administered with sunitinib (50 mg orally once daily), but 14 months later, the disease progressed. His medication was then changed from sunitinib to everolimus (10 mg orally once daily), but the disease continued to progress; therefore, pazopanib (800 mg orally once daily) was prescribed for mRCC for 17 months. When edema developed, immediate spot urine protein, albumin, and creatinine tests were performed. The albumin/creatinine (alb/cr) and protein/creatinine (prot/cr) ratios were 4300.64 mg/g and 5772.35 mg/g, respectively. A 24-h urine protein excretion test was performed and revealed 7484.58 mg/day of proteinuria, which was within the nephrotic range. Total cholesterol and serum albumin levels were 434 mg/dL and 2.9 g/dL, respectively. He was diagnosed with NS, and pazopanib treatment was discontinued. Other drugs to treat NS, such as angiotensin-converting enzyme inhibitor (ACEi) or glucocorticosteroids, were not administered, and only pazopanib treatment was discontinued. He had been taking calcium channel blocker (lacidipine 4 mg once daily) for hypertension since he had undergone nephrectomy, and his systolic blood pressure was less than 120 mmHg and diastolic blood pressure was less than 80 mmHg when he started taking pazopanib. But 2 months before the diagnosis of NS, systolic blood pressure increased to 140 mmHg, lacidipine was changed to amlodipine (5 mg twice daily), and blood pressure was regulated to normal range. The baseline serum creatinine levels were between 0.99 and 1.43 mg/dL after nephrectomy, with an average value of approximately 1.2 mg/dL. Serum creatinine level was 1.14 mg/dL when NS was diagnosed. In order to determine the cause of NS, a kidney biopsy should be performed. However, in consultation with a nephrologist, we decided not to undergo renal biopsy. Because the patient had a single kidney due to nephrectomy and grade 3 chronic kidney disease with estimated glomerular filtration rate between 50 and 60 ml/min/1.73m2, so there was a risk of aggravation renal failure after renal biopsy. Pazopanib was discontinued for 3 months without further cancer treatment and changed to temsirolimus (25 mg intravenously, weekly) after disease progression, 1 month later. Proteinuria improved 3 months after pazopanib discontinuation, spot urine prot/cr ratio decreased to 1776.84 mg/g at 3 months and 948.31 mg/g at 7 months after discontinuation, and serum cholesterol levels normalized to 186 mg/dL at 4 months (Fig. ). The patient had diabetes mellitus and exhibited trace proteinuria before pazopanib treatment; urine protein levels were completely restored to the previous levels. One month after discontinuation of pazopanib, blood pressure was lowered, and amlodipine was changed to lacidipine (4 mg once daily) again. Temsirolimus was administered for 15 months, but as the disease progressed, nivolumab (100 mg intravenously, every other week) was administered for 11 months; however, the disease continued to progress. No anticancer agents were available that would be eligible for insurance coverage; thus, pazopanib was carefully re-administered for 7 months (800 mg/day as recommended dose), and the dose was not reduced. During this period, trace proteinuria was still present, and pazopanib was successfully administered without any NS recurrence. The spot urine prot/cr ratios upon resuming and discontinuing of pazopanib treatment were 69.05 mg/g and 318.88 mg/g, respectively. Serum creatinine levels were not significantly changed, and blood pressure was maintained in the normal range without any change in antihypertensive medication. Seven months after pazopanib retreatment, the disease progressed, and the patient participated in another clinical trial.
pmc-6318972-1	A 27 year old African lady, from Bungoma County in Western Kenya, was admitted with generalized tonic clonic seizures lasting 5 to 10 min. This was associated with loss of consciousness for 10–20 min and left sided hemiparesis that lasted nearly 30 min after the convulsion. These symptoms had lasted one day but had been preceded by severe frontal headache radiating to the occiput and neck, chills, and photophobia for three days. Her past medical history was significant for peptic ulcer disease and allergic rhino-sinusitis for which she uses omeprazole, antacid and celestamine intermittently. Social history is notable for a diabetic twin sister who had been on insulin. The twin sister had died of pneumonia three days to the patient’s admission. The patient’s mother died ten years ago due to complications associated with diabetes. She is separated from her husband but she stays with her six year old daughter. The patient runs a small pharmacy business. She had travelled with her twin sister to a funeral before they fell sick. The patient had been sickly for a month before this admission. She had had severe frontal headaches associated with chills and photophobia. She had also complained of frequency of micturition and increased intake of water. There was no weight loss. She was treated with artemether-lumefantrine (AL) at a health center for malaria (blood slide was positive for P. falciparum). There was improvement for two weeks. However, the headache and photophobia recurred while polyuria and polydypsia persisted. She sought medical attention in a different health center where she was diagnosed with severe malaria and diabetes. Metformin was instituted in addition to quinine and AL. She developed the convulsions on the third day of treatment and was brought to our facility. Her vital signs was notable for tachycardia (pulse was 112 beats/min). Her blood pressure (BP) was 114/75 mmHg, saturations 93%, respiratory rate was 15 cycles/min, and temperature was 36.7 °C. She had bites on the lateral aspects of her tongue, an equivocal neck and positive Kernig’s and Brudzinski’s signs. Reflexes and remainder of her physical exam was normal. Her initial lab results showed 20 malaria parasites/200WBCs on a blood slide, a random blood sugar of 19.1 mmol/l, HIV non-reactive, ESR 87 mm/hr., cerebro-spinal fluid (CSF): turbid, 60 cells/mm3, pandy test negative, Indian ink positive, gram stain: no organism isolated, Ziehl–Neelsen stain (ZN) negative, venereal disease research laboratory (VDRL): negative; full hemogram (FHG), urea, electrolytes and creatinine (UEC) & CT scan: patient did not do these because of lack of funds; urinalysis: ph = 7.0, glucose +++, blood ++, SG 1.010, ketones nil, deposit: nothing seen; abdominal ultrasound: normal abdominal scan, no renal or splenic abscess. Fundoscopy neither revealed malarial retinopathy nor papilledema. We could not get a CT scan, urea/electrolytes/creatinine and a full hemogram/complete blood count done due to the patient’s financial status. The patient was started on diazepam 10 mg PRN, artesunate: 180 mg at 0, 12 and 24 h, ceftriaxone 2 g BD for 10 days, paracetamol 1 g tid and metformin 500 mg bd. Fluconazole 1200 mg od for 2 weeks was initiated due to the unavailability of amphotericin B and flucytosine. Convulsions ceased on the third day. There was improvement over the next few days but on the eighth day there was severe headache, neck stiffness and positive Kernigs sign. Serial tapping was done to relieve the headaches. After 2 weeks and with marked improvement, the patient was allowed home on metformin 750 mg bd, fluconazole 800 mg od for ten weeks. A repeat CSF analysis was negative for Indian ink after two weeks of treatment. She has been free of symptoms since she was allowed home. Metformin dose was reduced to 500 mg bd because her blood sugar was controlled and ranging between 5 and 8 mmol/l. She was counseled on lifestyle modification, particularly on diet and exercise to enhance control of diabetes. Other findings during her follow up are as outlined in the ‘Timeline’ table.
pmc-6318997-1	A 59-year-old Korean male with complaints of sudden metamorphopsia and reduced visual acuity for three days in the left eye was referred to our clinic. His past ophthalmological and other medical history was unremarkable except for hypertension. On examination, the best-corrected distance visual acuity (BCVA) was 20/20 in the right eye and 20/200 in the left eye. On slit-lamp examination, the cornea and conjunctiva were unremarkable, and there was no evidence of active inflammation in the anterior chamber or neovascularization in the iris. Fundus photography and fluorescein angiography showed BRVO in the left eye (Fig. ). Optical coherence tomography showed ME in the left eye (Fig. ). We performed intravitreal dexamethasone implantation and scatter laser photocoagulation in the left eye. The intravitreal dexamethasone implant injection was performed inferotemporally, 3.5 mm from the limbus. The implant was properly positioned in the vitreous chamber after the injection. One month after the intravitreal dexamethasone implantation, a decrease in the ME and an improvement of the BCVA to 20/40 was observed on left eye examination. Three months after the intravitreal dexamethasone implantation, recurrence of the ME and deterioration of the BCVA to 20/200 was observed on left eye examination. Therefore, we performed the second intravitreal dexamethasone implantation in the left eye in the same manner. One month after the second intravitreal dexamethasone implantation, the ME improved and the BCVA was 20/60 in the left eye. The ME recurred and the BCVA was 20/200 about four months after the second intravitreal injection. Therefore, we performed the third intravitreal dexamethasone implantation in the left eye in the same manner. Every intravitreal injections and the consequent follow-up examinations were performed by an experienced, single vitreoretinal specialist. On every follow-up examinations performed a day after the three dexamethasone implantations, the implant was positioned properly in the vitreous chamber, away from the crystalline lens. On slit-lamp examination performed one week after the third injection, grade 1 posterior subcapsular opacity was observed and the IOP was 42 mmHg by Goldmann applanation tonometer; however, there was improvement in the ME and the BCVA was 20/100. He was treated with oral acetazolamide, topical dorzolamide/timolol, and topical bimatoprost in the left eye. His IOP decreased to 18 mmHg in the left eye. He was discharged and prescribed topical dorzolamide/timolol and topical bimatoprost in the left eye and oral acetazolamide 10 mg/kg three times a day. Three weeks after the treatment, on slit-lamp examination, we observed that the posterior subcapsular cataract had progressed to mature stage (Fig. ); anterior chamber was shallower than that observed in the previous examination. The IOP was 18 mmHg and the BCVA was reduced to hand motion in the left eye. Phacoemulsification and the consequent posterior chamber intraocular lens implantation was performed to treat the mature cataract, and intravitreal ranibizumab was performed in order to decrease the remnant ME in the left eye. The procedure was uneventful. His BCVA in the left eye was 20/60 one week after the procedure.
pmc-6319221-1	Case 1: Mrs. Helen Lupon is 83 years old and homebound from her multiple chronic conditions and functional impairments. Her 84-year-old husband, who is also her primary caregiver, is struggling with her care. In the year before enrolling in HBPC, she had 17 emergency department visits and 13 hospitalizations along with multiple inpatient stays for rehabilitation. Most hospitalizations were for heart failure with reduced ejection fraction, some for exacerbation of COPD, and one for a fall. The patient’s goals of care included not going back to the hospital if at all possible; after HBPC began, the patient required only one hospitalization over the next eight months. Home health was initiated along with telehealth. Medications were aggressively managed, and several were lowered or discontinued. The patient stabilized, home health was discontinued, and hospice services were brought in to assist the HBPC team and her family with her care. She died peacefully at home. Her quality of life during the last eight months was dramatically improved, and her husband voiced immense appreciation for all the help he received.
pmc-6319221-2	Case 2: Mr. Charles Turner is a fully ambulatory 65-year-old diabetic with concomitant mental illness and alcoholism. HBPC was medically necessary because he was poorly adherent to his diabetic regime, and over a 21-month period he had 44 emergency department visits and 27 hospitalizations (over half in the Intensive Care Unit for diabetic ketoacidosis). Frequent HBPC visits were made to engage the patient in his care, and behavioral health clinicians were consulted. New glasses were purchased which enabled patient to read his insulin syringes. After house calls were initiated, the patient’s adherence and quality of life dramatically improved, and he required only one hospitalization over the next two years. HBPC programs are increasing in prevalence across the United States (U.S.). This growth is partly due to an interest among health systems in promoting value-based care. Though some solo practitioners provide house calls much as they were conducted 50 years ago, many new practices are based on an interdisciplinary team approach; this article will focus on this newer type of house call practice. Moreover, many practices ally with an entity that takes on financial risk for patient care and is rewarded with shared cost-savings resulting from better care. While HBPC practices take many forms based on their particular goals and business plans, these models are united by their focus on providing high quality, compassionate care in the home for those with the highest level of medical need and achieving cost savings. HBPC programs are increasingly recognized as adaptable and scalable, and value-based contracting with payors is becoming more common. This article describes the forces behind the resurgence of HBPC in the U.S. and then details different models including hospital based, government run, and free-standing programs.
pmc-6319224-1	JG, an 81 year old, non-smoking, independently active, Caucasian male presented with a vague, two to three week history of generalised malaise, myalgia and decreased physical function which were preceded by coryzal symptoms associated with episodic pyrexia, a sore throat and cough. His past medical history included a graft repair of an abdominal aortic aneurysm 13 years prior to his current admission and hypertension. His regular medication on admission included Losartan 100 mg once daily and Pravastatin 10 mg once daily. There was no history of recent travel although his previous occupation had included staying for long spells in Asia and Africa several decades previously. There was no history of visual disturbances, headaches, jaw ache, nasal congestion or history of skin rashes on systems examination. On admission, he had a haemoglobin of 138 g/L, a raised white blood count of 26.2 × 109/L that was predominantly neutrophilic with toxic degranulation on blood film, an erythrocyte sedimentation rate (ESR) of 22 mm/h, eosinophilia at 1.7 × 109/L, and a c-reactive protein (CRP) of 245 mg/L. His urea and creatinine were elevated at 24.2 mmol/L and 154 μmol/L from a baseline of 8.2 mmol/L and 126 μmol/L, respectively. His alkaline phosphatase (ALP) was 185 U/L, from a baseline of 82. His creatine kinase (CK) was 83 U/L and he had a low albumin of 19 g/L (). A sepsis of unknown origin was suspected however, there were no significant findings on a screen that comprised a urine analysis, chest radiography, and serial blood cultures. He was initially managed with empirical broad spectrum antibiotics and intravenous fluids for the kidney injury. After one week with little clinical improvement and several episodes of fever, a computerised tomography (CT) scan of the abdomen and pelvis was performed to determine a source for his systemic inflammatory response. This revealed ill-defined soft tissue surrounding the abdominal aorta at the level of the aortic repair suspicious for an inflammatory process/infection of the aortic graft. Simultaneously, and especially due to his marked inflammatory response that included a neutrophilia and eosinophilia that had peaked at a level of 6.5 × 109/L, a transthoracic echocardiogram, an autoantibody screen including anti-neutrophil cytoplasm antibody (ANCA) and investigations for haemolytic anaemia were requested and were subsequently unremarkable. Similarly, tests for HIV, Lyme disease, Syphilis, Epstein Barr virus (EBV), cytomegalovirus (CMV), Hepatitis, Legionella, Mycoplasma, Strongyloides, and thyroid dysfunction were all negative. An 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET/CT) was requested in view of the findings at the aortic graft site. It was not intended as an a priori investigation for a diagnosis of vasculitis. However, it revealed non-specific low grade increased tracer uptake in the arterial tree but no clear evidence of aortic graft infection (). Given the diagnostic uncertainties, absence of a causative organism and normal serology, a MDT decision was made to proceed to a temporal artery biopsy (TAB) (a stab in the dark) without prior colour Doppler sonography (CDS) investigation. The 18 mm × 2 mm × 2 mm specimen of the temporal artery revealed an inflammatory cell infiltrate and presence of occasional multinucleated giant cells consistent with temporal (giant cell) arteritis (GCA) (). JG was immediately commenced on 40 mg prednisolone, once a day, along with calcium, vitamin D, and a bisphosphonate for bone protection. He was discharged home with follow up by the rheumatology team. His renal, and liver function had stabilised prior to discharge (). He adhered to the treatment with corticosteroids and was guided by the rheumatology team as to how to taper the steroid dose over time. The timeline of the patient’s investigations, management, and treatment is shown in .
pmc-6319327-1	A 48-year-old Japanese man was admitted to our hospital for evaluation of pain in the distal right tibia. Nephrotic syndrome occurred at the age of 4 years. Subsequently, nail-patella syndrome was diagnosed due to the presence of nail dysplasia, patellar aplasia, and bilateral iliac horns on a pelvic radiograph. Hemodialysis (HD) was initiated at the age of 25 years. A liver tumor was detected by ultrasonography at the age of 43 years and hepatectomy was done. Histological examination showed a moderately differentiated hepatocellular carcinoma with normal background liver architecture. Markers of hepatitis B virus and hepatitis C virus were negative. At the age of 45 years, PTX was performed because hyperparathyroidism became resistant to medical management, including maxacalcitol and cinacalcet, and osteoporosis progressed (). Before PTX, intact parathyroid hormone (iPTH) was 568 pg/mL,bone alkaline phosphatase (BAP) was 43 μg/L, and tartrate-resistant acid phosphatase (TRAP)-5b was 1370 mU/dL. A total of 4 parathyroid glands were removed, and part of the smallest gland was re-implanted in the right forearm muscle. iPTH decreased to 24 pg/ml on postoperative day 1, but returned to 229 pg/ml after 1 year. At the age of 48 years, pain and swelling suddenly developed at the distal right tibia with no precipitating cause when the patient stood up. A plain radiograph showed a radiolucent lesion with a fracture line in the distal right tibia (a). Magnetic resonance imaging revealed a bone tumor-like mass lesion associated with a fracture line (b). On admission, his blood pressure was 156/106 mm Hg. Laboratory tests gave the following results (): BAP, 7.0 μg/L, TRAP-5b, 86 mU/dL; corrected Ca, 10.0 mg/dL; P, 4.0 mg/dL; and iPTH, 162 pg/mL. The lumbar bone mineral density (BMD) measured by dual X-ray absorptiometry was 0.671 g/cm3 (Z score: −0.6 SD), the distal radial BMD was 0.400 g/cm3 (Z score: −6.4 SD), and the proximal femoral BMD was 0.453 g/cm3 (Z score: −3.6 SD). He was taking calcium carbonate (3 g/day) and lanthanum carbonate hydrate (1500mg/day). Genetic testing revealed mutation of the LMX1β gene, which is the typical cause of nail-patella syndrome. The surgical open resection and internal fixation of the distal right tibia brown tumor was performed under general anesthesia.
pmc-6319502-1	A 55 year old male presented to our hospital with critical left lower limb ischemia with associated great toe ulceration. Computed tomography (CT) angiogram revealed a Trans-Atlantic Inter-Society Consensus (TASC) A lesion in the left superficial femoral artery (SFA) with patent run-off vessels. The patient underwent left retrograde SFA angioplasty via an ultrasound guided right common femoral artery puncture. Drug eluting balloon angioplasty was performed for the SFA lesion and a confirmatory angiogram revealed satisfactory results with patent run-off vessels. Access site control was attempted utilizing the Celt ACD® device. Unfortunately, continuous bleeding from the access point was noticed. Therefore, manual compression was applied for 10 min. Post procedure, the patient had palpable pedal pulses and satisfactory access site appearance. His discharge medications included dual anti-platelet therapy and statins. One week following discharge, the patient developed sudden pain and numbness in his right foot with short distance calf claudication. On clinical evaluation, the patient denied any symptoms of critical limb ischemia. Physical examination confirmed an unremarkable access site, palpable femoral and popliteal pulses, but absent pedal pulses. Consequently, a lower limb CT angiogram was conducted and revealed a metallic artifact in the distal popliteal artery consistent with VCD embolisation (Fig. and ). Endovascular retrieval of the closure device was attempted through the right antegrade common femoral artery using a snare (Indy OTW™ Vascular Retriever, 8Fr, .35 mm, 100 cm; Fig. ). Unfortunately, the attempt was unsuccessful and the patient developed severe spasm of the popliteal artery. The following day, the patient’s symptoms worsened and he developed rest pain. Therefore, we opted for surgical exploration of the distal popliteal artery and tibioperoneal trunk (TPT), which revealed a thrombus in the explored arteries with the closure device occluding the TPT bifurcation. Selective embolectomy of the anterior tibial artery and TPT with device retrieval was successfully accomplished (Fig. ). Dacron patch was used for arteriotomy closure. On table doppler ultrasound was done and demonstrated good signals in the patch and crural vessels. The patient’s post-operative course was unremarkable; his pedal pulses were restored and he was discharged 3 days following the procedure on dual anti-platelet therapy. At 3 months follow up, the patient was doing well with completely healed wounds and palpable pedal pulses in both lower limbs.
pmc-6319503-1	An 80-year-old Caucasian man who initially presented with back pain was found to have a 9.8 cm infrarenal abdominal aortic aneurysm on computed tomography (CT). This was suitable for endovascular aneurysm repair (EVAR) but the left common iliac artery (CIA) was short, necessitating the extension of the left iliac limb into the external iliac artery (EIA). The decision was made to proceed with an on table coiling of the left internal iliac artery (IIA) and EVAR. However, a tortuous right CIA made coiling of the left IIA extremely difficult and following a prolonged attempt, the decision was made to proceed with EVAR without coiling the IIA (Fig. ). On a 1-month follow up CT the left limb extension had disengaged from the main body, resulting in a large Type 3 endoleak. The disengagement was presumed to be due to the tortuosity of a heavily calcified external iliac artery returning to its anatomical position on removal of the Meir wire (Boston Scientific). The left IIA remained patent and acted as an outflow for the type 3 endoleak. The left limb was then realigned successfully with termination of the type 3 endoleak. On a follow up CT 1 month post repair, the left IIA remained patent with a large type 2 endoleak demonstrated but the sac size remained static (Fig. ). The failure of the IIA to occlude was presumed due to the cavity created within the aneurysm sac from the type 3 endoleak. Due to the large size of the aneurysm and the relatively large size of the type 2 endoleak the decision was made at multi-disciplinary team meeting to embolise the type 2 endoleak. As antegrade access to the left IIA was not possible due to the presence of the stent graft, an attempt was made to access the internal iliac artery via the buttock. Direct sac puncture was not possible as the endoleak was located within the pelvis and surrounded by bony structures and pelvic viscera. The intention was to puncture the posterior division of the IIA but visualisation with ultrasound was limited and a vessel was punctured, which on subsequent angiography was shown to be the anterior division of the left IIA (Fig. ). The left IIA was embolised using Spirali (Pyramed) coils in a retrograde manner. Due to the absence of a solid structure which to compress the access vessel against, especially as the anterior division of the IIA had been punctured, the arteriotomy needed to be closed using an Angio-Seal (Terumo). Correct intraluminal placement of the Angio-Seal (Terumo) is dependent on backflow of blood into the Angio-Seal (Terumo) sheath from the artery. However, as the left IIA had been embolised, backflow from the Angio-Seal (Terumo) was non-existent. The angioseal was not visualised on ultrasound and therefore a 4 French dilator was inserted within the Angio-Seal (Terumo) sheath through which contrast was injected to directly visualise the tip to the sheath within the artery (Fig. ). The sheath was then withdrawn until just inside the vessel lumen and the plug deployed with immediate haemostasis (Fig. ). This was an off-label use of the Angio-Seal (Terumo). A follow up CT 1 month post procedure revealed resolution of the Type 2 endoleak.
pmc-6319504-1	A 42-year-old man initially presented to hospital with painless jaundice and weight loss of 9 kg over a period of four to six weeks. A CT scan of the abdomen and pelvis in the portal-venous phase revealed a 5.3 × 4.7 cm periportal lymph node adjacent to the pancreatic head (Fig. a). A Positron Emission Tomography –CT (PET-CT) showed avid tracer uptake (Fig. ). There was associated biliary obstruction at the level of the porta hepatis. The patient underwent percutaneous biopsy followed by ERCP and deployment of a 10 mm × 80 mm covered metal stent (WallFlex Biliary RX Stent, Boston Scientific, Marlborough, MA, United States). The core biopsy of the nodal mass revealed diffuse large B-cell lymphoma (DLBCL). Three weeks following the first cycle of Rituximab, Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone (R-CHOP) chemotherapy, the patient presented to hospital complaining of fever and malaise. The patient had pyrexia of unknown origin and was prescribed a course of Ceftazidime and Metronidazole intravenous antibiotics. The following day the patient developed sudden onset haematemesis, passing 500 ml of fresh red blood. An urgent portal venous phase CT of the abdomen showed extensive necrosis of the mass with erosion of the metal biliary stent through the bile duct wall causing a large amount of intralesional gas (Fig. ). This was followed promptly by an urgent duodenoscopy that revealed acute blood actively oozing from within the metal biliary stent and fresh haematoma in the second part of the duodenum. The patient was transferred immediately back to radiology to have a triple phase (pre-contrast, arterial and portal venous phase) CT of the abdomen to assess for a bleeding point. The multiphase CT revealed a 13 mm pseudoaneurysm of the main portal vein. The pseudoaneursym was bulging towards the proximal end of the indwelling biliary stent (Fig. ). There was also hyperdense material within the stent lumen, which was presumed to be acute blood. The portal vein pseudo aneurysm was thought to be due to iatrogenic trauma of the portal vein wall at the time of stent insertion with further erosion of the vessel wall in the weeks following insertion. The other possible causes considered were vascular injury at the time of percutaneous biopsy and infection/inflammation within the surrounding lymphomatous mass causing direct vascular erosion. After multidisciplinary team discussion between Interventional Radiology, Hepatobiliary Surgery and Gastroenterology, the decision was made to perform percutaneous transhepatic covered stenting of the main portal vein. A right anterior portal vein puncture was performed using a 21G micropuncture needle under ultrasound guidance. A 10 Fr sheath was inserted. A C-2 Cobra catheter was positioned in the superior mesenteric vein. Digital subtraction venograms performed via the catheter confirmed a broad-based saccular psudoaneurysm of the main portal vein measuring approximately 13 mm in diameter. The pseudoaneurysm was positioned at the anteroinferior edge of the proximal portal vein and was bulging into the proximal end of the biliary stent (Figs. and ). There was no active bleeding at the time of the procedure. An Amplatz SuperStiff guidewire was passed to the SMV. A covered 14 mm × 60 mm FLUENCY plus Vascular Stent Graft (Bard Peripheral Vascular, Tempe, AZ, United States) was deployed in the main portal vein across the pseudoaneurysm. There was immediate exclusion of the pseudoaneurysm on post deployment venograms (Fig. ). The main portal vein, right portal vein, left portal vein, superior mesenteric vein and splenic vein remained patent. The sheath was removed and the tract plugged with Avitene Microfibrillar Collagen Hemostat (Bard Davol, Warwick, RI, United States). The patient’s haematemesis ceased promptly following the procedure. Post-procedure anticoagulation and antiplatelet treatment was not initiated given the patients risk of further bleeding. There have been no procedure related complications in over 1 year since the stent placement. The patient continued R-CHOP chemotherapy for DLBCL and was clinically well at recent outpatient follow up.
pmc-6319509-1	An 80-year-old woman presented with a history of intermittent, severe epigastric pain. Over the preceding 5 months, she had less severe and self-resolving epigastric pain 15–30 min after every meal. The full blood count, serum biochemistry values, and C-reactive protein level were within normal ranges. Additionally, arterial gas analysis was normal. Abdominal plain radiography showed free air between the liver and diaphragm, and PI (Fig. ). Abdominal computed tomography showed severe calcification of the SMA origin; SMA peripheral flow was reserved (Fig. ). Computed tomography (CT) revealed bubble-like intramural gas of the small bowel with the contrasted wall pneumoperitoneum (Fig. ). Since the patient did not have peritonitis, a conservative approach was performed. She was managed in the condition of intensive care unit, due to fears of the potential for acute mesenteric ischemia due to mesenteric artery occlusion or non-occlusive mesenteric ischemia. She was managed for bowel obstruction, which included fasting and intravenous fluid administration. She received heparin infusion to prevent SMA occlusion and maintain collateral flow. During admission, she reported abdominal pain relief. Seven days after admission, abdominal plain radiography showed improvement in PI and pneumoperitoneum; therefore, she was permitted to begin drinking fluids. There was no evidence of recurrent abdominal pain after the fluid consumption, so she was allowed to eat solid food. Angiography was planned to relieve the postprandial abdominal pain. The findings showed severe stenosis of the SMA origin with calcification, and the SMA had replaced the common hepatic artery (Fig. , ). ET, namely stenting to the SMA and common hepatic artery, was performed. The SMA trunk was engaged with a 6F Parent Plus 60 guiding catheter (Medikit, Tokyo, Japan) from the left brachial artery. Initially, 8000 units of heparin was infused, and additional heparin was added to keep the activated whole blood clotting time over 200 s. The SMA occlusion was traversed using a 0.014” NEO EVT Guide Wire Cruise (ASAHI INTECC J-sales, Tokyo, Japan). The SMA trunk to the hepatic artery was traversed using a 0.014” NEO EVT Guide Wire Cruise, which was engaged with a 6F SheathLess PV (Cardian Health, Ohio, USA) from the right brachial artery. Intravascular ultrasonography (IVUS) (Navifocus WR, TERUMO, Tokyo, Japan) revealed severe stenosis of the SMA trunk with calcification. Balloon dilation was performed with the kissing ballon technique using a 4-mm Coyote ES (Boston Scientific, Marlborough, MA, USA) to the SMA and 5-mm SHIDEN RX (KANEKA MEDICAL, Osaka, Japan) to the common hepatic artery. After dilation, two balloon-expandable stents (5-mm PALMAZ Genesis (Cardian Health, Ohio, USA) to the SMA and 6-mm PALMAZ Genesis to the common hepatic artery) were implanted with the kissing stent technique from the common ostium (Fig. ). The sizes of the stents were based on the IVUS measurements. IVUS showed good orifice expansion. Final angiography showed satisfactory circulation to the terminal organ from the orifice (Fig. ). Endovascular therapy relieved the patient’s symptoms. She required dual anti-platelet therapy (DAPT) with aspirin and clopidogrel post-intervention. Follow-up computed tomography showed patency of the orifice of the common hepatic artery and SMA, and there was no evidence of PI or pneumoperitoneum. No recurrent postprandial abdominal pain occurred for two years after the ET.
pmc-6319511-1	A 73-year-old man with past medical history of stage IV bladder and prostate cancer, status post cystoprostatectomy with ileal conduit, left orchiectomy, and subsequent left radical nephrectomy (for recurrent left hydronephrosis and pyelonephritis), presented with worsening right leg edema and pain for 2–3 weeks which confined him to bedrest. On examination, there was severe pitting edema of the right leg associated with erythema and warmth. Duplex ultrasound examination of the lower extremities revealed a nonocclusive thrombus extending from the right external iliac to the central (cranial) segment of the femoral vein and an occlusive thrombus in the central segment of the deep femoral (profunda femoris) vein. A non-contrast CT scan of the abdomen and pelvis revealed an increase in the size of a pelvic retroperitoneal nodal mass inseparable from the right common iliac artery and right ureter, with no clear visualization of the right iliac venous system (Fig. ). The patient was placed on therapeutic low molecular weight heparin in preparation for right lower extremity catheter-directed thrombolysis and stenting. Pre-procedural ultrasound examination of the access site confirmed findings seen on lower extremity Doppler examination and revealed extension of the venous thrombosis into the femoral and deep femoral vein precluding femoral vein access. The patient was then placed in prone position under general anesthesia and the right popliteal vein was accessed. Venography showed extensive thrombosis and strictures of the right common iliac, right external iliac, and right femoral veins with collateralization (Fig. ). Pharmacomechanical thrombolysis using the Trellis Thrombectomy System (Formerly Covidien, Boston, MA, now discontinued) was performed in the right femoral vein through the popliteal vein access to clear the acute thrombus. Repeat venography showed resolution of the acute DVT in the right femoral and external iliac veins with minimal residual stenosis. There was however no identifiable connection between the right common iliac vein and the inferior vena cava (IVC). Unsuccessful attempts were made using multiple wire/catheter combinations to recanalize the right common iliac vein. Initial attempts to cross the obstruction using an angled catheter in combination with soft and stiff hydrophilic wires were unsuccessful. Furthermore, attempts were made using a crossing Rubicon Support Catheter (Boston Scientific, Marlborough MA) in combination with hydrophilic wires and the stiff (back) end of an Amplatz wire. However, there was inadequate support with this combination. The left popliteal vein was then accessed using a micro puncture set and venography demonstrated a narrow, but patent left common iliac vein and patent IVC without identifiable inflow from the right common iliac vein. The patient was subsequently turned supine. A wire was advanced through a right common femoral artery access into the lower aorta to provide a visual safeguard for preventing arterial injury during sharp venous recanalization. An 18 mm Atlas balloon (Bard, Murray Hill, NJ) placed through a right internal jugular access was inflated across the IVC/left common iliac vein confluence to provide a central target. A 10 French right femoral venous sheath was inserted and the metallic stiffening cannula/catheter combination from a Rösch-Uchida Transjugular Liver Access Set was advanced into the peripheral (caudal) segment of the right common iliac vein stump. The metal cannula was then progressively advanced towards the target balloon in the lower IVC. A 21-gauge Chiba needle was advanced through the cannula (Fig. ). The needle and a 0.018-in. guidewire were used to create a channel through the encasing tumor into the caudal segment of the IVC with one pass (Fig. ). After gaining access into the patent IVC, balloon angioplasty was performed and self-expanding kissing [16 mm diameter × 90 mm length] Wallstents (Boston Scientific, Marlborough MA) were placed in both common iliac veins. Three additional overlapping stents [one 16 mm × 90 mm Wallstent; two 14 mm × 60 mm S.M.A.R.T stents (Cordes, Fremont, CA)] were placed from the right common iliac to the right common femoral vein followed by balloon angioplasty (Fig. ). Initial post-stenting venography demonstrated thrombosis of the central stents, likely due to inadequate inflow. Pharmacomechanical thrombolysis using the Trellis Thrombectomy System was performed to successfully remove the acute thrombus from the central stents. The stents were extended below the femoral head to ensure adequate inflow using an additional 10 mm × 40 mm EV3 Protégé (Medtronic, Minneapolis MN) stent. Completion venography demonstrated widely patent stents with contrast flowing into the IVC (Fig. ). The catheters were removed, and hemostasis achieved. Over the next several days, there was significant improvement of the pain and swelling and the patient was discharged to hospice care. The patient passed away after 2 months due to progression of the metastatic disease.
pmc-6319512-1	We present a case of a 70-year old Caucasian gentleman who underwent a living donor renal transplant 10 years ago following a diagnosis of glomerulonephritis aged 34. More recently, he had developed acute heart failure manifested by bilateral leg oedema, facial oedema and difficulty in breathing together with severe and difficult to control hypertension with wide pulse pressures. Biochemistry at the time of presentation revealed Cr 190 mmol/L and eGFR 27–32. The patient had past history of several transplant biopsies performed to investigate repeated rise in serum creatinine level over the last 10 years. Clinically, there was audible bruit over the right side of the lower abdomen. Ultrasound, CT angiography scan and subsequently catheter angiography demonstrated very high flow arterial venous fistula within the transplanted organ (Fig. ), likely to be the cause of the patients symptoms of decompensated heart failure. The main transplant artery and veins, as well as all intra renal branches were aneurysmal with extreme tortuosity, especially in the intra renal vessels. The maximum diameter of the main transplant artery was 12 mm with relative narrowing at the origin. Other salient findings were ectatic and tortuous iliac vessels (Fig. ). Given the decompensated heart failure being caused as a result of the high flow AVF, patient planned for super-selective embolisation of the arterio-venous communication under interventional radiology. Informed consent was obtained and patient readmitted for elective super selective renal transplant embolization, to be performed under general anaesthesia in order to control the patient’s haemodynamic status. Given the knowledge of high flow through the AVM, plans were made to make both arterial and venous punctures. The contralateral left common femoral artery was punctured under ultrasound guidance and 6F sheath was inserted and positioned in the right common iliac artery. Through the sheath, the transplant artery was selected and 6mmx40mm angioplasty balloon was positioned and inflated at the origin of the transplant artery to reduce inflow and hence pressure into the AVM. The site of the AVM was approached via the venous side. Since the arterial side has a tight, almost 360°, backward bend to AV communication site and the venous channel has shorter and straighter path, a 6F sheath was inserted into the right common femoral vein, aiming to deliver the embolic device. Multiple attempts at cannulating the fistula from the venous side failed due to the predicted factors previously described. The tortuosity of the vessels led to difficult in reaching the exact site of the AVM, whilst the high flow from the arterial side continually forced the guide wires and catheters out of the transplant vein and back up into the common iliac vein as the inflated balloon at the origin of the artery failed to reduce the flow. Therefore, an additional 12F sheath and balloon (Medtronic ReliantM stent graft balloon catheter) were inserted into the venous ipsilateral side, coming from an insertion site just above the initial puncture. This balloon allowed transient occlusion of the common iliac vein (Fig. ), to ensure the guide wire remained in the transplant vein and not forced back into the host common iliac vein by the arterial pressure coming through the AVM. Once the wire was secured in situ, an occlusion plug (10mmx7mm) (AMPLATZER ™ Vascular Plug II, Abbott) was advanced through a 6F sheath and deployed across the fistula (Fig. ). This led to good embolic occlusive result with subsequent angiography showing no flow across the previous fistula (Fig. – (a) pre occlusion plug and (b&c) post occlusion plug). Following successful embolisation of the fistula, patient’s haemodynamic status remained stable. There was a transient rise in serum creatinine immediately after the procedure due to contrast nephrotoxicity from the procedure itself. In the proceeding weeks, the blood pressure decreased to around 130/85 systolic and serum creatinine improved to 138 on latest bloods. Of most importance, the patient is clinically significantly improved and has reported complete resolution of breathlessness and oedema – suggesting his symptoms were a direct result of the AVF causing high flow cardiac failure. USS of the transplanted kidney performed 1 week after intervention showed good perfusion and no evidence of a residual or recurrent AV fistula. Follow up CT Angiogram performed 2 months after procedure confirmed good perfusion of transplanted kidney, stable position of the occlusion device at AVF site and resolution of the previous AVF (Fig. ).
pmc-6319520-1	A 76-year-old male had a history of surgical replacement of the whole aortic arch due to a TAA (total arch replacement with elephant trunk). Three years after the surgery, severe hemoptysis occurred, which resulted in the patient’s emergency hospitalization at our hospital. On arrival, massive hemoptysis (400 mL) and hypotension were seen. The patient’s initial blood pressure was 79/42 mmHg. His hypotension resolved after the intravenous administration of crystalloid fluids. Laboratory tests showed a hemoglobin level of 7.3 g/dL. The patient received blood transfusions, and a computed tomography (CT) scan was performed. The CT images showed two aortic pseudoaneurysms, related to the aortic anastomoses. They were attached to the trachea and the left bronchus, which was suggestive of AB fistula formation (Fig. ). The patient was diagnosed with ruptured pseudoaneurysms of the aortic arch. Emergency TEVAR was performed under general anethesia. Two conformable TAG thoracic devices (W.L Gore and Associates, Flagstaff, AZ, USA; diameter: 34 mm x length: 200 mm, diameter: 37 mm x length: 200 mm) were inserted into the region extending from the aortic arch (elephant trunk) to the descending aorta. After that, the hemoptysis stopped, and the patient was discharged. Two months later, the hemoptysis reccurred so te patient was re-admitted to our hospital. CT showed a type 2 endoleak from the bronchial artery. The pseudoaneurysms remained (Figs. and ). At that time, blood test results were as follows: hemoglobin level of 9.3 g/dL; peripheral white blood cells 5.4 × 109/L; C-reactive protein 0.4 mg/L; and erythrocyte sedimentation rate 12 mm/h. As re-rupturing of the pseudoaneurysms due to a type 2 endoleak was suspected, transcatheter arterial embolization (TAE) was performed to treat the type 2 endoleak. First, the femoral artery was punctured, and aortography was conducted. Aortography did not show any type 1 or 3 endoleaks. However, a type 2 endoleak from the brachiocephalic artery was confirmed. Next, the right brachial artery was punctured, and brachiocephalic arteriography was carried out, which showed a type 2 endoleak into the aortic pseudoaneurysms from the supreme intercostal artery via the bronchial artery (Fig. ). We used the triple coaxial technique to treat it. A 4F diagnostic catheter was advanced through a 2.7-F non-tapered microcatheter (Sniper 2 Selective Revolution; Terumo-Clinical Supply). Next, a 1.9-F non-tapered microcatheter (Marvel; Tokai, Kasugai, Japan) was advanced through a 2.7-F microcatheter, which was coaxially introduced through a catheter. The 1.9-F non-tapered microcatheter was advanced into the aortic sac via the bronchial artery. TAE was performed by injecting 5 ml of an n-butyl cyanoacrylate (NBCA) (B. Braun, Melsungen, Germany): Lipiodol Ultra-Fluide (Guerbet, Roissy, France) (ratio: 1: 3) mixture into the aortic sac and pseudoaneurysms via the inflowing artery. After the TAE, the type 2 endoleak disappeared (Fig. ). The patient’s hemoptysis stopped, and he was discharged. There was no further recurrence of the hemoptysis. One year later, CT showed that the pseudoaneurysms had disappeared, and the diameter of the aorta had also reduced (Fig. ). Gallium scintigraphy showed no abnormal accumulation. The patient’s progress has been good. Hemoptysis is a rare complication of TAA. It usually occurs after the formation of an AB fistula. In such cases, the hemoptysis can be massive if it is not treated surgically (Ahmadi et al. ). A high degree of suspicion regarding this entity is the key to its appropriate management. It is estimated that 5% of patients who present with hemoptysis suffer massive hemoptysis, a life-threatening condition that can cause airway obstruction and exsanguination. It exhibits a mortality rate of 30–70% (Phang et al. ). Nowadays, such hemoptysis can be treated using endovascular techniques involving stent-grafts (Kokotsakis et al. ). In this case, we first treated the AB fistula formed by the rupturing of the anastomotic pseudoaneurysms using TEVAR. The hemoptysis initially stopped because the TEVAR blocked the blood flow to the pseudoaneurysms and reduced the pressure within them. However, 2 months later the hemoptysis relapsed because a type 2 endoleak increased the pressure within the aortic sac and pseudoaneurysms. Moreover, the type 2 endoleak supplied blood flow into the aortic sac and pseudoaneurysms. Previously, Synowiec T, et al. reported that hemoptysis was a unique symptom of type 1A endoleaks that occur after TEVAR and led to the rupturing of thoracic aneurysms (Synowiec et al. ). However, to the best of our knowledge, this is the first report about hemoptysis caused by a type 2 endoleak after TEVAR. The incidence of endoleak after TEVAR ranges from 5% to 20%, which is similar to that after endovascular abdominal aortic aneurysm repair (EVAR) (Parmer et al. ). Type 2 endoleaks are most commonly seen after EVAR. Type 1 and 2 endoleaks occur at similar rates after TEVAR (Stavropoulos et al. ). An accepted management method is aggressive endovascular repair of type 1 and 3 endoleaks along with observation for type 2endoleaks (Alsac et al. ). Collateral circulation in the chest involving the thoracic aorta is not so well developed compared to collateral vessels in the abdomen, making transarterial embolization of thoracic endoleaks difficult (Stavropoulos et al. ). To our knowledge, there is no consensus treatment option for type 2 endoleaks. In the present case, rupturing and hemoptysis occurred due to a type 2 endoleak, which was considered to be an absolute indication for embolization. It is still unclear if embolization of the endoleak nidus alone achieves the same outcomes as embolization of the endoleak nidus and the associated branch vessels. In the current case, using the triple coaxial technique, a microcatheter was inserted into the aortic sac via the bronchial artery. TAE was performed by injecting 5 ml of an A: Lipiodol Ultra-Fluide (ratio: 1: 3) mixture into the aortic sac/pseudoaneurysms via the inflowing artery. After the TAE, the type 2 endoleak disappeared. In this case, TAE was very effective against the patient’s hemoptysis, and the pseudoaneurysms disappeared. However, further investigation is necessary to identify more effective and appropriate embolization methods.
pmc-6319521-1	This 74-year-old male with recurrent peptic ulcer disease presented with acute onset weakness, hypotension, and melena. Following initiation of a rapid transfusion protocol, the patient was taken to the GI lab where arterial hemorrhage from an ulcer in the anterior duodenal bulb was identified. Upon upper endoscopy, epinephrine injection therapy and multiple clip application could not fully control bleeding. These endoscopic clips (in this patient with no prior abdominal surgery) later helped identify the site of bleeding on fluoroscopy. The patient was then brought to the IR suite for mesenteric angiography. Since the patient was borderline unstable, contrast enhanced CT was forgone. Based on the anatomic location of the visualized bleeding peptic ulcer, it was thought the arterial source of bleeding would be the GDA. However, upon catheterization of the celiac axis and subselection of the presumed CHA, angiography demonstrated no native GDA (Fig. ). Proper right and left hepatic arteries were identified (Fig. ). Superior mesenteric artery (SMA) angiogram demonstrated opacification of a replaced GDA (Fig. ), which then supplied a large portion of the left lobe of the liver, with no opacification of the right hepatic artery (Fig. ). Active extravasation of contrast into the duodenal lumen in the region of the endoscopic clips was seen (Fig. ). The replaced GDA was successfully coil embolized, with post-coil embolization angiography demonstrating occlusion of the replaced GDA and patency of the gastroepiploic artery, which also arose from the GDA (seen in Fig. ). The patient’s symptoms of melena and hypotension resolved quickly after intervention. A liver function panel drawn the following day was within normal limits, however the liver panel drawn the following day on post-procedure day 2 demonstrated a mild transaminitis.
pmc-6319522-1	A 50-year-old man on long term haemodialysis through a left-sided brachiocephalic AVF presented with worsening pain and cyanosis in the left hand. The AVF had also become progressively aneurysmal over the preceeding 2 months. Duplex ultrasound demonstrated non-occlusive laminated thrombus in the aneurysmal outflow vein. The distal radial and ulnar arterial pulses were weak with monophasic flow demonstrated on duplex assessment. A diagnostic fistulogram showed very rapid flow across the AVF with an aneurysmal outflow limb but no distal outflow or central venous stenosis (Fig. ). The juxtaanastomotic venous limb measured 13 mm in maximum diameter (Fig. ). Percutaneous AVF banding was performed following ultrasound-guided retrograde access into the cephalic venous outflow limb with placement of a 6 French introducer sheath (Prelude; Merit Medical, South Jordan, UT). The brachial artery inflow was retrogradely catheterised using a 5 French angled KMP catheter (Cook, Bloomington, IN) and 0.035″ hydrophilic guidewire (Glidewire; Terumo, Somerset, NJ) combination. Following this, a 5 × 40 mm angioplasty balloon (EverCross; Covidien, Plymouth, MN) was placed across the venous outflow limb in the juxta-anastomotic region and inflated to nominal pressure using a mechanical inflation device (Fig. ). Two small incisions were made on either side of the inflated balloon and blunt dissection was carefully performed superficial and deep to the outflow vein (Fig. ). A 2–0 braided absorbable suture (Vicryl; Ethicon, Somerville, NJ) was double looped, pulled through the incision, below and above the waist of the inflated angioplasty balloon and secured (Fig. ) to create a stenosis in the outflow vein (Fig. ). Immediate post-banding duplex ultrasound showed reduced velocities in the outflow vein. If there was no change on the immediate post-banding duplex study, the balloon can be exchanged for a smaller diameter balloon for creation of a smaller diameter band. In this case, there was complete resolution of DASS clinical symptoms and no issues related to dialysis or AVF thrombosis 6 months following the procedure.
pmc-6319522-2	An 80-year-old female patient on long term haemodialysis due to diabetic nephropathy presented with a two-week history of left hand paraesthesia, weakness, ulceration and necrosis of her fingertips on the side of a left brachiocephalic AVF (Fig. ). The radial and ulnar arterial pulses were undetectable on clinical and duplex assessment. A diagnostic fistulogram was performed which demonstrated very brisk flow across the AVF with poor opacification of the radial and ulnar arteries in the forearm (Figs. ). She proceeded to undergo percutaneous AVF banding using the same technique described in the case above (Figs. ). However, despite successful flow reduction through the AVF and some improvement of arterial flow to the level of the forearm, the distal perfusion to the hand remained poor due to underlying atherosclerosis with occlusion of the distal radial and ulnar arteries and absence of the palmar arch in the hand (Fig. ). The tissue loss was managed conservatively and eventually healed with associated soft tissue atrophy over a period of 8 weeks.
pmc-6319523-1	Patient A was a 73-year-old male who presented to the emergency department (ED) with abdominal pain and low-grade fevers. He presented 6 months after the last of his five intravesical BCG instillations for his known non-muscle invasive urinary bladder papillary TCC. His medical profile included COPD, Type II diabetes, hypertension, dyslipidemia, and macular degeneration. There was history of remote TB exposure in childhood with no treatment or related hospital admissions. In ED, his complete blood cell count was within normal limits. Serum C-reactive protein was elevated at 58.6 mg/L (normal: 0–8.0 mg/L). Initial cross sectional imaging at the time of presentation demonstrated a new, multi-septated peripherally enhancing 6.3 cm × 1.9 cm × 5.6 cm, low-density collection within the retrocrural/posterior mediastinal region abutting the descending thoracic aorta along 180 degrees of circumference of the vessel (Fig. ). Along the right posterolateral wall, an enhancing focal outpouching arising from the descending thoracic aorta was also identified. On positron emission tomography/computed tomography (PET/CT), this lesion demonstrated peripheral intense hypermetabolism with central photopenia. PET/CT did not demonstrate any additional hypermetabolic lesions and was negative for tumor recurrence or metastatic disease elsewhere. A follow-up MRI of the thoracic spine was negative for discitis or osteomyelitis. CT-guided aspiration of the retrocrural abscess yielded mycobacterium bovis consistent with BCG on pathology. Given the constellation of findings and pathology results, the patient was treated with Isoniazid, rifampin, pyrazinamide, ethambutol, and Vitamin B6. Given the patient’s medical comorbidities, the mycotic aneurysm identified on CT was treated with endovascular stent graft placement as opposed to open surgical repair. No post-procedural complications were identified on CT. Adjunctive percutaneous drainage of the periaortic collection was also performed. Subsequently, over a course of 6 months the patient has remained asymptomatic, although the lesion has shown no signs of regression.
pmc-6319523-2	Patient B was a 67-year-old male who presented to ED with weight loss and night sweats. His complete blood cell count was within normal limits. Serum C-reactive protein was elevated at 41.1 mg/L. He had received fifteen intravesical BCG instillations for his low-grade papillary urothelial carcinoma and his last BCG instillation was 4 months prior to his ED presentation. His medical profile was otherwise negative apart from a remote uncomplicated appendectomy. Outpatient abdominal ultrasound was suspicious for periaortic lymphadenopathy. Follow-up CT scan of the abdomen and pelvis showed a centrally low density and peripherally enhancing periaortic collection measuring up to 5 cm × 1 cm × 4.6 cm in the infrarenal region. At this level, a focal outpouching arising from the aorta posteriorly measuring up to 0.8 cm × 2.2 cm × 0.9 cm (APxTRVxCC) was suspicious for a mycotic aneurysm (Fig. ). A percutaneous CT-guided biopsy of the periaortic collection demonstrated necrotizing granulomatous inflammation, highly suspicious for BCGosis. This patient’s mycotic aneurysm was treated with a surgical resection of the infected infrarenal aortic segment and repaired using an autologous graft harvested from the patient’s left femoral vein. He had an uneventful postoperative course in the hospital and was discharged on a standard antituberculosis medication regime including isoniazid, rifampin, pyrazinamide, ethambutol, and Vitamin B6. Patient has remained asymptomatic awaiting six-month follow-up imaging studies.
pmc-6319526-1	A-52-year-old female with HHT. Saturation at rest was 96% and during exercise decreased to 92%. CE confirmed a shunt with grade I-II. CT without contrast confirmed a simple PAVM in the left lower lobe with a feeding artery of 3 mm. The left pulmonary artery was catheterised, and angiography depicted the PAVM (Fig. ). The PAVM was first embolised with a detachable coil of 4 mm diameter and 10 cm in length (Interlock, Boston Scientific Marlborough, MA, USA). After ten minutes waiting there was still flow thought the PAVM. It was decided to deploy an MVP-3Q (Reverse Medical Corporation, Irvine CA, USA) through a 2.4 Fr microcatheter (Renegade microcatheter, Boston ScientificMarlborough, MA, USA) with following immediate occlusion (Fig. ). No complications occurred during the intervention. The patient was discharged the following day, and follow-up consists of clinical and CE control which showed improved oxygenation and no shunt at CE control six months after embolization.
pmc-6319526-2	A-19-year-old female with HHT and haemoptysis underwent three PAVM embolizations previously. The last embolization was performed in 2009, and the patient was without complaints. Recently she complained about shortness of breath and pain during mild exercise. Saturation at rest was 92% and during the exercise decreased to 89%. CE confirmed a shunt grade I-II. CT without contrast confirmed two new PAVMs. The right pulmonary artery was catheterized, and angiography depicted two PAVMs, one in a right upper lobe and one in the right lower lobe (Fig. ). Both PAVMs had feeding arteries with a diameter of 3.2 mm. The PAVM in the lower lobe was embolized with a detachable coil (Interlock, Boston ScientificMarlborough, MA, USA), 6 mm in diameter and 10 cm long. The PAVM located in the upper lobe was engaged coaxially with microcatheter 2.8 Fr (Renegade Hi-Flo; Boston ScientificMarlborough, MA, USA) and primarily embolized with MVP-5Q (Reverse Medical Corporation, Irvine CA, USA) with following immediate occlusion (Fig. a, b). No complications occurred during the intervention. The patient was discharged the next day, and follow-up consisted of clinical and CE control showed improved oxygenation up to 96% and no shunt at six months CE control.
pmc-6319526-3	A-32-year-old female with nasal bleeding during two years. A diagnosis of HHT was established. Oxygen saturation was 98% without significant changes during exercise. CE confirmed shunt grade II-III and non-contrast CT showed two PAVMs, one located in the right lower lobe with feeding artery of 5 mm and the other with feeding artery of 3.3 mm in diameter located in the right upper lobe (Fig. ). The PAVM in the lower lobe was embolized with Amplatzer plug IV (St Jude Medical, Minnesota, USA), 8 mm in diameter. The PAVM in the upper lobe was embolized with MPV-5Q (Reverse Medical Corporation, Irvine CA, USA) delivered through a microcatheter 2.8 Fr (Renegade Hi-Flo; Boston ScientificMarlborough, MA, USA) with following immediate occlusion of both PAVMs (Fig. a, b). No complications occurred during the deployment. The patient was discharged the next day, and clinical control and CE showed no shunt six months after embolization.
pmc-6319526-4	20-year-old male patient with diagnosed HHT and multiple PAVMs in both lungs. Embolization of PAVMs in the left lung was performed in 2010 and 2011 with a good outcome. In 2017 CE control showed shunt grade II-III and CT confirmed two PAVMs in the right lung. The biggest had a feeding artery of 6 mm and was embolized with Amplatz plug IV (St Jude Medical, Minnesota, USA), 8 mm in diameter a few months before the actual intervention (Fig. ). The smaller PAVM in the right lower lobe had two feeding arteries. One feeding artery with a diameter of 3 mm was embolized with a detachable coil 4 mm and 8 cm in length (Interlock, Boston ScientificMarlborough, MA, USA). The other feeding artery with a diameter of 3.4 mm was embolized with MVP-5Q delivered through a microcatheter 2.8 Fr (Renegade Hi-Flo; Boston ScientificMarlborough, MA, USA), but due to highly angulated feeding artery the MVP was displaced a little proximally and occluded the feeding artery a longer distance to the PAVM than intended (Fig. ). No complications occurred, and the patient was discharged the following day without symptoms. The patient was asymptomatic at clinical control 12 months after the embolization, and no CE was performed during the follow-up. The patient did not experience any symptoms during the 12 months follow-up period.
pmc-6319527-1	A 38-year-old gravida 1, para 0 was transferred to our unit at 26 weeks of gestation with severe right iliac fossa pain and a suspicion of threatened preterm labour. She presented with a 3 day history of right iliac fossa pain, nausea, vomiting and diarrhoea. Vaginal examination and her fetal fibronectin test was negative. However, transabdominal and transvaginal ultrasound examination revealed a 6.0 × 5.5 × 5.9 cm vascular mass within the right pelvis with a clear arterial feeder and turbulent swirling intraluminal flow, leading to the diagnosis of a pseudoaneurysm (Fig. ). The diagnosis was subsequently confirmed by Magnetic resonance imaging (MRI) (Fig. ). The patient was discussed in our multidisciplinary team meeting with interventional radiologists, vascular surgeons, and anaesthetists. The risk of imminent rupture was assessed as high due to the severity of her symptoms and a 5–10 mm overall increase in the size of the pseudoaneurysm documented by sequential MRI 14 days apart. Surgery was considered high risk and so selective embolization of the pseudoaneurysm was performed. This was performed under local anaesthetic in the Interventional Radiology suite with full preparation for emergency delivery by the obstetric and anaesthetic team in case of fetal distress. The right uterine artery was identified angiographically from a contralateral femoral arterial puncture and selectively catheterised using a microcatheter. The artery was small but extravasation into the presumed pseudoaneurysm was identified near its proximal portion. The artery was embolised with a series of microcoils across the neck of the pseudoaneurysm to block flow. The procedure was uneventful and the fetus showed a continuous reactive heart rate pattern. Follow up ultrasound showed a completely thrombosed pseudoaneurysm with no flow. She had regular follow ups in the antenatal clinic with no sign of recurrence. A planned caesarean section was performed at 38 weeks gestation. A healthy baby boy was born weighing 2696 g with a blood loss of 1100mls. During the caesarean section, the thrombosed pseudoaneurysm was seen below the right broad ligament and all other pelvic organs looked completely normal (Fig. ). An ultrasound scan 3 months postpartum showed a small completely thrombosed pseudoaneurysm (Fig. ).
pmc-6319532-1	A 31-year-old female patient with symptomatic postpartum deep venous thrombosis of the right leg up to the distal part of the inferior vena cava prophylactically received a suprarenal IVC filter (Recovery G2; Bard Peripheral Vascular) before surgical thrombectomy. Suprarenal position had to be chosen due to short distance between thrombus in the distal IVC and inflow of renal veins. Surgical access was established via venotomy of the common femoral vein and thrombectomy was performed using an occlusion balloon. In a follow-up CT it was noted that the filter had dislodged and tilted towards the right IVC wall with its legs piercing the contralateral wall and extending into the left renal vein, likely during surgical thrombectomy. Because of re-thrombosis despite continued oral anticoagulation with Warfarin, catheter-directed thrombolysis including stenting of the common iliac veins and the IVC was performed. No filter retrieval was attempted because of large amount of clot within the filter. After 5 weeks of Warfarin therapy the patient was scheduled for retrieval which was considered mandatory due to the displaced and tilted filter with penetrating legs. After introduction of a 14-F sheath (Cook Medical, Bloomington, Indiana, USA), a SOS catheter (Omni 2, 5F, 80 cm, Angiodynamics, New York, USA) together with a Bentson wire (260 cm, Cook Medical, Bloomington, Indiana, USA) and EN Snare (7F, 18–30 mm, Merit Medical, Utah, USA) were used to create a loop. The single loop-snare technique was attempted several times without success because the loop kept slipping around the filter legs. In order to stabilize the loop a second loop using the same technique was formed around the filter tip. Withtwo loops around the filter tip, the filter was successfully pulled away from the wall and into the 14F sheath. A follow-up venography was unremarkable. Follow-up ultrasound after 6 months showed patent IVC with regular flow. Genetic tests revealed a heterozygous Factor V Leiden mutation and Warfarin therapy was continued for at least 2 years, after which the patient was lost to follow-up.
pmc-6319532-2	A 50 year old male received an infrarenal IVC filter (Celect, Cook Medical, Bloomington, Indiana, USA) before orthopedic surgery of the lower extremities due to prior history of Factor V Leiden mutation with several previous episodes of deep vein thromboses. After successful surgery and resumption of Warfarin a filter retrieval was attempted 74 days later. Venography revealed a tilted filter with the tip towards the right IVC wall. Standard technique didn’t seem feasible, therefore a loop-snare technique was attempted using the same equipment and technique as mentioned in Case 1. Despite a successful loop around the filter tip, filter retrieval was unsuccessful. At that time the filter was left in place because of an only moderate tilt and only one leg protruding outside the IVC (Fig. a). Warfarin was continued for at least a year and then stopped due to repeated anorectal bleeding episodes. During a CT for macrohematuria more than 8 years later a severe tilt of the filter was seen with two legs around the aorta and one leg eroding the bone of a vertebral body. In a multidisciplinary board the decision for another retrieval attempt was made. 3146 days after implantation the patient was scheduled for a second attempt. Expecting a difficult retrieval an 18-F sheath (Cook Medical, Bloomington, Indiana, USA) was inserted into the right jugular vein. Venography confirmed a tilted filter with the tip deeply embedded into the IVC wall and two legs protruding outside the IVC (Fig. b). Two loops were formed around the filter tip using the same instruments previously described: two reversed shape SOS-catheters (Omni 2, 5F, 80 cm, Angiodynamics, New York, USA) were placed below the filter and two exchange length Bentson wires (260 cm, Cook Medical, Bloomington, Indiana, USA) were navigated on both sides of the filter tip. Above the tip the wires were snared. A second view confirmed one loop on each side of the filter tip (Fig. c). During traction both loops started to slip away from the tip, therefore a third loop was created (Fig. d) again using a Bentson wire. With 3 loops around the filter tip, the filter could be removed from the wall and finally pulled into the 18F sheath. Configuration of the 3 loops around different filter struts was documented after retrieval (Fig. e). During final traction the patient expressed stinging pain in the back. Post-interventional venography showed a large contrast pocket was visible at the location of the embedded filter tip (Fig. f) without a true extravasation. An immediate CT did not show extravasation or retroperitoneal hematoma. Because the patient was hemodynamically stable no further treatment was undertaken. A venography 6 weeks later showed a regular shape of the IVC with only minimal narrowing (Fig. g).
pmc-6319534-1	We present a 57 year old gentleman with CKD 5 who had an autogenous brachiocephalic fistula 4 months prior to presentation to us. During their last surgical clinical visit, the fistula was noted to be poorly maturing and then referred to interventional radiology for fistulogram and possible endovascular intervention to assist with fistula maturation. The patient had a fistulogram which demonstrated a high grade juxta-anastomotic stenosis which was successfully balloon dilated. After a 6 week follow up clinic visit the fistula was still immature and a duplex scan, a second fistulogram with possible intervention were requested. Fistulogram was performed via an antegrade approach from an access just proximal to the swing point. There was an “apparent” stenosis (Fig. ) which was angioplastied then followed by severe spasm (Fig. ). which was perceived by the operator to be recalcitrant stenosis. In the light of this perceived recalcitrant stenosis, a decision to stent the area was taken. After measuring the vessel diameter based on the immediate post-plasty images a 6 mm diameter × 5 cm length Viabahn stent (Gore & Associates, Flagstaff, AZ) was selected and deployed in the standard fashion. Following stent deployment, the stent migrated and stopped at the confluence of the cephalic vein and the subclavian vein (Fig. ). At the time the operator thought the stent was stable and unlikely to cause harm to the patient in this position. However, after reviewing the images with colleagues including vascular surgeons a decision was made to attempt to retract the stent into the arm which would be easier for the surgeon to retrieve the stent surgically, if required. The patient was subsequently brought back 24 h later to the interventional radiology suite. Initial fluoroscopic image of the left shoulder region demonstrated the stent was absent from the final position documented the previous day indicating the stent had migrated further (Fig. ). Fluoroscopic scanning of the chest identified the stent to overlie the left lower lobe (Fig. ). Subsequent Pulmonary angiogram confirmed the stent to lie within a segmental pulmonary artery of the left lower lobe (Fig. ). After discussion of the options, risks and benefits with the patient and a multidisciplinary team, a decision to attempt stent retrieval was made versus leaving the stent in situ. After appropriate informed written consent, the right groin was prepped and in the standard fashion. Right common femoral vein access was then upsized to accept a 16 F sheath (Cook, Bloomington. IN USA). Main pulmonary access was then performed with an APC pulmonary catheter (Cook, Bloomington. IN USA). The APC catheter was then removed over a Storq wire (Cook, Bloomington. IN USA) wire and subsequently a 12 F 70 cm braided sheath was advanced into the main pulmonary artery and then left lower lobe pulmonary artery. Pulmonary angiograms performed identified the optimal projection to identify the vessel to access. After accessing the appropriate vessel the 12F sheath was advanced just to the origin of the branch above the stent. Subsequently a 15 mm Amplatz Gooseneck snare (ev3, Plymouth MN, USA) was manipulated until the stent was lassoed at about half way along the stent. Given the flexibility and potential collapsibility of the Viabahn stent it was over-sheathed carefully collapsing and gently retracting the captured stent to minimize potential vessel injury (Fig. , Additional file ). Once the stent had been totally ensheathed, the 12F sheath was retracted through the outer 16F sheath coaxially. The stent was retrieved intact (Fig. ). The procedure was performed under moderate sedation using Fentanyl and midazolam with continuous monitoring of the patient’s vitals by a dedicated nurse. Throughout the procedure the patient remained hemodynamically stable with normal respiratory function. Post procedure the patient was observed for 6 h post procedure before being discharged home in a stable condition. The patient has so far been followed up for 3.5 years and has not developed any adverse pulmonary or cardiac condition. Interval CTPA done at an outside facility showed normal pulmonary vasculature with no evidence of pulmonary vessel injury.
pmc-6319538-1	A 58-year-old male arrived at policlinic. He complained on dysuria, ischuria for several months and macrohaematuria. He did not have physical symptoms of acute blood loss. Serum Creatinine level was normal (68 mg/dL). Ultrasound examination of kidneys and urinary tract revealed solid bladder mass of about 55 mm in diameter without signs of metastatic spread. Patient had no significant anemia (HGB 117 g/l), but HGB level decreased from 143 g/l in two days. Before hospitalization additional imaging was performed:MRI: bladder tumor 63 mm with invasion of the right ureter with right ureterohydronephrosis. On nephroscintigraphy: nonfunctioning right kidney. Irrigation through the three-way catheter was ineffective. Conservative treatment of haematuria (Транексам® (Tranexam), Obninsk, Russian Federation, tablets 500 mg 3 times per day) was ineffective. Cystoscopy was non-informative due to profuse haematuria, with multiple blood clots in the bladder. After following discussion with referring physician it was decided to perform selective embolization of bladder tumor due to potential life-threatening blood loss. Pre-operative CT-Angiography showed significant neovasculaturization of the tumor without obvious source of active bleeding. Patient underwent an embolization procedure: Right Common Femoral artery access (Vascular sheath Prelude 5F, Merit Medical, USA., catheter Cobra C2 5F, Merit Medical, U.S.A.), bilateral superselective embolization of anterior division branches of Internal Iliac Arteries using microcatheter 2,4F (Progreat-α micro catheter, Terumo Corporation, Japan) and Avigo hydrophilic guidewire, Medtronic, USA). Superior and inferiorvesical arteries on both sides (Figs. and ), left prostatic artery and left obturator arteries were embolized using 500 μm Embozene particles (Boston Scientific Corporation, USA) 1 vial per procedure (diluted with contrast media and saline in amount to stable suspension) to achieve subtotal stasis (near absence of contrast enhancement of distal parts of above-mentioned arteries), subtotal embolization of both anterior portions of Internal Iliac Arteries (notable ceasing of contrast flow from arising point) upon exit using gelfoam (Pfizer, USA) and Embozene suspension (self-made combination of Gelfoam particles about 20% of 10 ml syringe volume, mixed with Embozene spheres about 10% of syringe volume with contrast media and saline) (Figs. and ). Such comprehensive embolization was performed as our dedicated medical facility with determined equipment is exclusive in terms of treating oncology patients and this particular patient can’t be brought here in time if bleeding reoccure at home. The procedures were performed under local anesthesia using Lidocain 1%. Intravenous Fentanyl 0,2 mg (Fentanyl, Moscow endocrine factory, Moscow, Russia) was injected before embolization for analgesia during the procedure and programmed IV morphine pump connected following the procedure. Patient suffered from severe pelvic pain for 4 h after the procedure and mild pain in pelvic area for 2 days. Haematuria ceased on day 3 after the procedure. For planning further treatment transurethral biopsy of the mass was performed, necrotic villous papillary tumor in lower semicircle of the bladder was visualized. Two days later urologist diagnosed peritonitis. On Ultrasound examination massive amount of free hyperechoic liquid, without gas was reported. Urgent exploratory laparotomy shown 3 to 4 l of free muddy smelly fluid in the abdominal cavity and total necrosis of the bladder wall. Sanation of abdominal cavity, necrectomy, cystostomy, and bilateral nephrostomy were performed. Patient deceased from multiple organ failure on day eight despite symptomatic therapy in intensive care unit. An autopsy report described total necrosis of tumor with necrosis of underlying bladder wall and multiple organ failure due to sepsis (Figs. and ).
pmc-6319539-1	A 37-year-old woman was referred to our institute (Gynaecology Department) due to persistent metrorrhagia and raised serum β-HCG levels (126031 mU/ml). Patient gynaecological and obstetrical history was characterized by one prior term birth in 2012 and a spontaneous miscarriage at seven gestational weeks in 2016. The suspicion of gestational choriocarcinoma was raised as a highly vascularized uterine mass was detected at computed tomography. Gestational Choriocarcinoma is a highly malignant neoplasm of trophoblastic origin, characterized by rapid growth and high tendency to develop hematogenous metastases. Diagnosis is more commonly based on β-HCG serum levels and clinical presentation rather than on histopatological analysis (due to the high risk of bleeding following bioptical procedures). Thanks to its high chemosensitivity, gestational choriocarcinoma is usually associated with a good prognosis and high cure rates. The patient was submitted to a total body triphasic contrast-enhanced Multi Detector Computed Tomography (MDCT) confirming the presence of choriocarcinoma, but also showing a giant pelvic aneurysm suspecious for AVF (Fig. ), lung metastases and pulmonary thrombo-embolisms. The diagnosis of AVF was confirmed by a Color Doppler Ultrasound examination showing a typical arterialized, low-resistance blood flow of the pelvic veins (Fig. ). The case was discussed within a multidisciplinary gynaecological and radiological meeting. Following this, an angiography was planned in order to confirm the AVF diagnosis and to perform an embolization to stop the bleeding trying to occlude the fistula despite its large size. The decision on the opportunity to place a filter to prevent further episodes of pulmonary embolism was postponed until diagnostic angiograpy and embolization were completed. In an emergency setting, the patient was submitted to a diagnostic angiography initially using a right femoral transarterial and right femoral transvenous approach. Diagnostic arteriography confirmed the presence of a giant AVF sustained by branches of both hypogastric arteries with early opacification of the right gonadal vein and the inferior vena cava (Fig. ). After selective catheterization of right (Fig. ) and left (Fig. ) hypogastric arteries, using a coaxial microcatheter (Carnelian 2.2, Tokai, Medical Products, Sarayashiki Taraga Kasugay-city, Japan), the afferent branches to AVF were subsequently embolized using first detachable coils (Interlock, Boston Scientific, Natick, MA, USA) of variable diameter (6–14 mm) and length (10–40 cm), after poly vinyl alcohol (PVA) particles (Contour Embolization particles 500–710 μ, Boston Scientific, Natick, MA, USA) and finally also an ethylene-vinyl alcohol copolymer (EVOH)-based liquid embolic agent (Squid-peri 12, Emboflu, Gland, Switzerland) in order to reduce AVF in-flow (Figs. and ). A transfemoral phlebography with selective catheterization of the right gonadal vein showed multiple thrombi (Fig. ), leading to the pulmonary embolism previously detected at the contrast-enhanced MDCT. Using a right transjugular approach, an Amplatzer plug was finally placed at the confluence of the right gonadal vein in the vena cava (Fig. ), not only to reduce AVF out-flow but also to occlude the right gonadal vein, preventing further episodes of pulmonary embolism. Metrorrhagia almost disappeared after the procedure. A contrast-enhanced MDCT examination performed 24 h after the embolization confirmed the correct placement of the plug (Fig. ) and the significant reduction in volume and enhancement of the AVF. No further pulmonary embolism was demonstrated at MDCT performed during follow-up. A second transarterial embolization using the same embolic agents (PVA particles, coils and Squid) was performed six months later. The second embolization, combined with a complete response to systemic chemotherapy confirmed by β-HCG levels normalization with disappearance of pulmonary metastases, determined the complete AVF resolution (Fig. ). Currently the patient is aymptomatic and enjoys full well-being of health.
pmc-6319599-1	A 40-year-old man had noticed a painless protruding mass under the midline parietal-occipital scalp for approximately one year. On plain radiograph (Figure ), there was erosion of the skull bone underneath. The gigantic mass was better depicted by a magnetic resonance imaging (MRI), which demonstrated a large parasagittal tumor with homogenous contrast enhancement (Figure , Gadolinium-enhanced T1-weighted MRI) that was considered meningioma. Hypervascularity of the lesion was suspected based on the MRI with strong enhancement. Angiography revealed dense stains with distorted vessels over the same location (Figure ). Embolization prior to craniotomy was performed. During surgery, the tumor was noted to be dark-reddish in color and rubbery in texture; it had invaded through the skull but could be easily separated from the dura. The tumor was completely removed along with the invaded skull and bone cement was used for cranioplasty. Histopathology demonstrated plasmacytic type plasmacytoma with positive lambda stain (Figures , ). Systemic oncological evaluations detected no evidence of residual tumor or other skeletal involvement, no tumor cells by bone marrow biopsy, no anemia, and no hypercalcaemia or renal impairment due to plasma cell dyscrasia. Radiotherapy was not performed because of total tumor resection. The postsurgical period was smooth and there was no laboratory or radiologic evidence of recurrence or systemic progression after the patient was regularly followed for one year (Figure ).
pmc-6319615-1	The patient is a 66-year-old woman who initially presented with an incidental finding of a body of pancreas mass on magnetic resonance imaging for follow-up of a stable ovarian cyst. Computed tomography redemonstrated a hypodense mass in the body of the pancreas (). Fine-needle aspiration biopsies returned positive for pancreatic adenocarcinoma. There was no evidence of dissemination, and baseline tumor markers were within normal range. Her medical history was notable for T2DM on canagliflozin and sitagliptin. Her preoperative hemoglobin A1c (HbA1c) was 8.2%. The patient was offered surgical resection and was instructed to hold all oral hypoglycemic agents 24 h before surgery. She underwent an uncomplicated distal pancreatectomy with en bloc splenectomy. Over the first 12 h after surgery, it was noted that the patient had polyuria (urine output range: 100–325 mL/h). On routinely obtained serial laboratory analyses, the serum bicarbonate level was noted to be consistently low in the setting of anion gap and absence of lactic acidosis (). There was suspicion that the patient may be developing euDKA because of her use of canagliflozin. A serum β-hydroxybutyrate was obtained and it was markedly elevated at 48.1 mg/dL (reference range: 0.2–2.8 mg/dL). A urinalysis was performed that demonstrated glucosuria and ketonuria. Up to this point, the patient's serum glucose level was only modestly elevated (range: 155–224 mg/dL). After the recognition of euDKA, an intravenous insulin infusion was initiated and the patient was fluid resuscitated. Within 10 h after such treatment, there was improvement in the β-hydroxybutyrate levels, the anion gap normalized, and the urinalysis only demonstrated trace ketone bodies (). The remainder of the patient's postoperative course was uncomplicated and she was discharged on postoperative day 5 (POD 5). Her final pathology revealed poorly differentiated invasive ductal carcinoma, with 2 of 13 specimen lymph nodes containing metastatic cancer. The patient was educated regarding the benefit of postoperative adjuvant chemotherapy.
pmc-6319615-2	The patient is a 75-year-old man who initially presented with obstructive jaundice and elevated liver function tests. He underwent an endoscopic ultrasound, which demonstrated a mass in the head of the pancreas with an associated bile duct stricture. Endoscopic retrograde cholangiography was performed, with biliary endoprosthesis placement. He had no evidence of metastatic disease on axial imaging, and although there was no identifiable pancreatic mass, the patient did have a double duct sign (). His medical history was notable for T2DM on dapagliflozin, glipizide, metformin, and liraglutide. His preoperative HbA1c was 7.3%. The patient was offered surgical resection and was instructed to hold all oral hypoglycemic agents 24 h before surgery. He underwent an uncomplicated pylorus preserving pancreaticoduodenectomy. Over the first 12 h after surgery, it was noted that the patient had polyuria (urine output range: 150–300 mL/h). On routinely obtained serial laboratory analysis, the serum bicarbonate level was noted to be consistently low in the setting of a normal anion gap and absence of a lactic acidosis (). There was suspicion that the patient may be developing euDKA because of his use of dapagliflozin. A serum β-hydroxybutyrate was obtained and it was markedly elevated at 50.8 mg/dL (reference range: 0.2–2.8 mg/dL). It was only at that time that the patient had an abnormal anion gap of 19 mmol/L. A urinalysis was performed that demonstrated glucosuria and ketonuria. Up to this point, the patient's serum glucose level was only modestly elevated (range: 158–225 mg/dL). β-Hydroxybutyrate levels were retrospectively obtained on POD 1 by analyzing samples that were routinely collected on POD 0, which demonstrated that they were consistently elevated postoperatively in the setting of a normal anion gap. After the recognition of euDKA, an intravenous insulin infusion was initiated and the patient was fluid resuscitated. Within 12 h after such treatment, there was improvement in the β-hydroxybutyrate levels, the anion gap normalized, and the urinalysis was negative for ketone bodies. The remainder of the patient's postoperative course was uncomplicated and he was discharged on POD 6. His final pathology revealed moderately differentiated pancreatic ductal adenocarcinoma, with metastatic carcinoma identified in 2 of 14 specimen lymph nodes. He was also educated regarding the benefits of postoperative adjuvant chemotherapy.
pmc-6319673-1	A 65-year-old female presented with fatigue, 20 lb weight loss, and intermittent painless jaundice. Initial laboratory investigations revealed an elevated bilirubin of 9.8 mg/dL (normal range 0.3–1.7), alkaline phosphatase of 977 U/L (normal 44–127), and carbohydrate antigen (CA) 19-9 of 267 U/mL (normal 0–37). Abdominal ultrasound revealed cholelithiasis with possible central biliary tree dilation. Endoscopic retrograde cholangiography (ERC) with sphincterotomy showed a malignant-appearing stricture in the common hepatic duct but yielded equivocal brushings and biopsies (). A laparoscopic cholecystectomy had been attempted at an outside institution but was aborted secondary to extensive inflammation in the porta hepatis, preventing visualization of the gallbladder. After referral to our institution, the patient underwent a repeat ERC and magnetic resonance cholangiopancreatography (MRCP) () with placement of bilateral 7F biliary stents. Again, duct brushings revealed only benign cells with fibrosis and inflammation. The differential diagnosis included primary gallbladder adenocarcinoma, hilar cholangiocarcinoma, and cholecystitis with extrinsic compression of the biliary tree (Mirizzi syndrome), and the decision was made to proceed with open exploration. During open surgical exploration, the gallbladder was markedly inflamed with dense adhesions to the duodenum, without signs of disease dissemination. On dissection of the gallbladder, an obvious fistula was identified between the infundibulum of the gallbladder and the biliary bifurcation. Intraoperative frozen section analysis was negative for malignancy. After resection of the damaged extrahepatic bile ducts, separate right and left hepatic ducts remained. Reconstruction was performed with bilateral hepaticojejunostomies to a Roux-en-Y segment of the jejunum. Pathological analysis () of the surgical resection specimen revealed xanthogranulomatous cholecystitis with extensive fibrosis and inflammation (without dysplasia or malignancy), consistent with the Mirizzi syndrome. The patient recovered well and returned to her practice as a clinical psychologist.
pmc-6319673-2	A 71-year-old previously healthy female presented with 2 weeks of painless jaundice. Ultrasound evaluation showed dilation of the intrahepatic and extrahepatic bile ducts as well as an impacted calculus in the gallbladder neck. Computed tomography (CT) and MRCP () confirmed the dilation and revealed the common bile duct narrowing at the level of the impacted stone. Before referral to our institution, endoscopic retrograde cholangiopancreatography (ERCP) was performed with successful placement of a biliary endoprosthesis and resolution of her jaundice. In our clinic, the patient was without complaints and laboratory evaluation revealed a bilirubin of 1.7 mg/dL, an alkaline phosphatase of 226 U/L, and aspartate transaminase and alanine transaminase of 49 and 41 U/L, respectively, and both CA 19-9 and carcinoembryonic antigen (CEA) were within normal limits (). The differential diagnosis for this patient was identical to that noted in Case 1. The patient was scheduled for an open exploration—cholecystectomy, extrahepatic biliary resection, and Roux-en-Y hepaticojejunostomy. On operative exploration, a gallstone was found impacted in the neck of the gallbladder. However, a dense mass was also found at the junction of the gallbladder and the bile duct, encasing the right hepatic artery and portal vein—intraoperative frozen section revealed adenocarcinoma. Given the presence of vascular encasement, the most effective palliative measure to drain the biliary tree was determined to be resection of the damaged and partially obstructed bile ducts and performance of a palliative hepaticojejunostomy bypass. Pathological analysis of the resected specimen revealed a moderately differentiated adenocarcinoma, involving the cystic and common hepatic ducts, perineural and angiolymphatic invasion, and metastatic carcinoma in one station 12 lymph node (). After recovery from surgery, the patient was treated with palliative chemoradiotherapy and she survived for an additional 12 months postoperatively.
pmc-6319673-3	A 70-year-old male with a history of smoking and long-standing diabetes presented with a 60 lb weight loss, fatigue, constipation, and painless jaundice. Initial MRCP showed significant intrahepatic and extrahepatic biliary ductal dilation. In addition, it also showed a dilated pancreatic duct and ill-defined hypoechoic mass within the head of the pancreas (). The patient underwent biliary stenting at the time of ERCP and a biopsy was performed through endoscopic ultrasound, which was concerning for malignancy. The patient was referred to our institution for further management. On arrival at our institution, the serum bilirubin and alkaline phosphatase had normalized to 1.1 mg/dL and 80 U/L, respectively. His CEA was within normal limits, but the serum CA 19-9 remained elevated at 184 from an initial value of 328 U/mL before his biliary stenting (). The differential diagnosis for this man was broad, similar to the previous two cases, but due to the distal bile duct obstruction and mass within the head of the pancreas, a primary pancreatic malignancy was strongly suspected. The patient underwent operative exploration and was found not to have any evidence of disseminated disease. A firm mass was noted involving the head and uncinate process of the pancreas. He underwent a cholecystectomy and pylorus-preserving pancreaticoduodenectomy with standard reconstruction with an invagination pancreaticojejunostomy, hepaticojejunostomy, and end-to-side duodenojejunostomy. Pathological analysis revealed a successful R0 resection of a T3N1M0 moderately differentiated invasive pancreatic ductal adenocarcinoma, invading into the peripancreatic soft tissue, ampulla, duodenal wall, and bile duct (). The patient recovered well after the surgery and was discharged home on postoperative day 5. He is currently 6 weeks from his resection and has fully recovered.
pmc-6319675-1	A 24-year-old woman from a Middle Eastern country presented to the Jefferson Pancreas, Biliary and Related Cancer Center for evaluation of a recurrent pancreatic mass. She complained of right upper quadrant fullness, and physical examination revealed a remote right subcostal incision. At the age of 12 years, she had first developed decreased appetite, weight loss, fatigue, pruritus, and subsequently became jaundiced. Medical records from that episode revealed that an endoscopic biliary stent was placed with surgical exploration through a right subcostal incision and partial resection/enucleation of a pancreatic mass. In the intervening 12 years, the mass had persisted and enlarged, although the patient was asymptomatic, having neither anorexia, pruritus, nor jaundice. Routine hematology and basic chemistry panels were normal. The tumor marker cancer antigen 19-9 was mildly elevated at 89 U/mL (<35 U/mL). An abdominal computed tomography (CT) scan with contrast revealed an 8.2 × 7.6 cm heterogeneous-enhancing lesion, prominently involving the uncinate process of the pancreas (). The pancreatic head and neck were displaced and splayed around the anterior aspect of the tumor. The mass abutted the superior mesenteric vein (SMV) as well as the superior mesenteric artery (SMA). There was no evidence of main pancreatic ductal dilatation and the pancreatic neck, body, and tail were normal. Imaging showed no evidence of metastatic disease to the liver or regional lymph nodes. The mass was believed to be an SPT, based on the previous partial resection and the accompanying pathology report. The patient underwent an open cholecystectomy and a difficult classic pancreaticoduodenectomy. The operative time was 12 h and the estimated intraoperative blood loss was 1500 mL. There was no evidence of metastasis, but the tumor had adhered extensively to the SMV and portal vein and surrounded the SMA. We were able to accomplish the separation of the tumor from the venous structures without incident; however, separating the tumor from the SMA proved challenging. At one point, the SMA was transected due to adherence of the tumor. The SMA was subsequently reapproximated in an end-to-end manner with good arterial Doppler signals distal to the anastomosis. Pathological analysis of the surgical specimen revealed the tumor to be a solid pseudopapillary neoplasm (). All surgical margins were free of neoplasia and all harvested regional lymph nodes showed only follicular lymphoid hyperplasia, with no evidence of granulomas or neoplasia. Immunohistochemical stains of the specimen were positive for CD56, CD10, and vimentin, with the neoplastic cells showing strong diffuse nuclear and cytoplasmic staining for β-catenin and weak diffuse staining for synaptophysin. The neoplastic cells were negative for chromogranin A, trypsin, AE1/AE3, and E-cadherin. Molecular genetic analysis was negative for the MYB gene deletion. On the first postoperative day, the patient had a small amount of bile visible in the operatively placed drains, she was fluid seeking, and her abdomen was somewhat distended. Due to the suspicion of a vascular insult related to the SMA reconstruction, an abdominal CT with intravenous contrast was obtained and revealed an intraluminal thrombus in the proximal SMA, ∼1.5 cm from its origin off the aorta, causing near complete occlusion of the SMA (). She was therefore returned to the operating room where the proximal jejunum appeared ischemic. We performed an SMA embolectomy and repaired a leak at her hepaticojejunostomy through reconstruction of the biliary-enteric anastomosis. She tolerated the reoperation well and improved nicely. On the fourth postoperative day, an upper gastrointestinal series with water-soluble contrast instilled into the stomach through a nasogastric tube revealed no contrast extravasation, and both the afferent and efferent limbs of the duodenojejunostomy were grossly patent. The patient and her family were instructed on the home management of the large abdominal incision and superficial wound infection. Healing occurred over the next 4 months. Telehealth monitoring was used by our nursing experts to communicate with the patient on a regular basis, with mobile phone images documenting the status of the wound. She returned to Philadelphia for a follow-up visit after 6 months. At that time, the patient appeared well, her wound was completely healed, and an abdominal CT scan with contrast showed normal after pancreaticoduodenectomy anatomy, without any evidence of recurrent or persistent tumor.
pmc-6319677-1	A 60-year-old male presented to the Thomas Jefferson University Hospital in August 2015 with a syncopal episode, fatigue, abdominal pain, and dyspnea on exertion. A review of the patient's records revealed two previous hospitalizations to an outside institution over the prior 9 months for melena and near syncope. A computed tomography (CT) with enterography performed at the outside hospital in November 2014 revealed a 4 cm intraluminal mass-like density. An esophagogastroduodenoscopy (EGD) with endoscopic ultrasound was performed at that time, which revealed an extrinsic thickened fold of the second portion of the duodenum (D2), concerning for a duodenal duplication cyst. Fine-needle aspiration of the suspected mass revealed normal villous morphology. At the patient's second presentation to the outside hospital in July 2015, a second EGD evaluation revealed a bleeding ulcer in the stomach and a repeat CT showed that the duodenal mass had increased to 5 cm. At the time of transfer to our institution, the patient reported a recent episode of melena without gross blood. He was found to have a hemoglobin of 6.8 g/dL and received two units of packed red blood cells. A CT scan to evaluate the abdominal mass revealed focal dilation and thickening of the third portion of the duodenum (D3) with intussusception of D2 into D3 (). There was also mild intrahepatic biliary dilatation and common bile duct dilatation to 1.2 cm, likely resulting from intussusception of the ampulla of Vater and the distal common bile duct. EGD was performed again, revealing a submucosal lesion along the lateral aspect of D2, causing 80% narrowing of the lumen and erythematous mucosa overlying the lesion. Colonoscopy showed nonbleeding diverticula and a nonbleeding, benign sessile polyp. Capsule endoscopy was nonrevealing. Due to the concern for a malignant mass involving the ampullary complex, a pylorus-preserving pancreaticoduodenectomy was performed. The specimen was removed and gross pathology revealed a 7 × 6 × 3.5-cm duodenal submucosal leiomyoma (). The mass stained positive for actin and desmin and negative for DOG-1, S100, SD34, and ALK-1 (). Negative margins were obtained and none of the 10 harvested lymph nodes were involved with disease. The patient recovered well from surgery, with no further episodes of melena or syncope.
pmc-6319678-1	A previously healthy 50-year-old man was admitted to Henan Provincial People's Hospital with a month's history of unspecific epigastric abdominal discomfort. This patient had no significant history of medical or alcohol abuse and hospitalization. Physical examination and laboratory tests were unremarkable. Abdominal ultrasonography (USG) and CT scan showed an enlarged pancreatic head containing an uneven mixed solid and cystic composition about 3.7 × 4.0 cm (). No surrounding lymphadenopathy was noted. An underlying neoplasm was suspected and these findings suggested a solid pancreatic head neoplasm. The patient underwent enucleation of the neoplasm in the pancreatic head and was sent to the pathology department. On gross examination of the surgical specimen, a soft, round, well-circumscribed mass with pseudocapsule about 3.6 cm in diameter was identified (). It had pale brown or grayish white solid portion, papillary projections, and cystic portions resulted from hemorrhagic necrosis. On histological analysis, the tumor was composed of monotonous uniform polygonal cells with moderate to abundant amphophilic cytoplasm and arranged in solid nests with areas of cystic degeneration, characterized by separation of the cells into pseudopapillary aggregates with intervening accumulation of mucopolysaccharide-rich ground substance (). No vascular space or peripheral invasion was identified. The tumor was encapsulated and although the tumor–pancreatic parenchyma interface was irregular on histological analysis, the tumor did not invade the pancreas. The tumor cell nuclei were oval or coffee bean shaped (). No mitotic figures were identified in 20 HPF (4.75 mm2) from various areas of the tumor. On immunohistochemical analysis, the tumor cells were positive for vimentin, CD10, α-1-antichymotrypsin (AACT), α-1-antitrypsin (AAT), β-catenin, neuron-specific enolase (NSE), progesterone receptor (PR), and synaptophysin (Syn) in approximately 30% of the cells, whereas cells were positive for Ki-67 antigen (a proliferation marker) less than 1% of the neoplastic cells (). These findings helped to establish a diagnosis of SPT of the pancreas. Six months after surgery, a repeat CT scan of the pancreas revealed no evidence of the tumor. The patient is still alive at the time of publication.
pmc-6319679-1	A 56-year-old Caucasian male presented in September 2015 with severe, cramping, epigastric abdominal pain that radiated to his back and was associated with nausea, emesis, decreased appetite, and a 10 pound weight loss over the prior several months. Computed tomography (CT) of the abdomen () revealed a 6 cm mass in the body of the pancreas, closely adjacent to the celiac trunk, with main pancreatic duct dilation and parenchymal atrophy of the pancreatic tail. Although no signs of hepatic or distant metastatic disease were present, prominent regional celiac nodes were found. Fine needle aspiration pancreatic biopsy 2 days later detected cells compatible with acinar cell carcinoma. Per the AJCC TNM staging system, the patient was diagnosed with T4N1M0 (stage III) acinar cell carcinoma. The patient proceeded to receive neoadjuvant chemotherapy with FOLFIRINOX (folinic acid, 5-fluorouracil, irinotecan, and oxaliplatin), beginning in October 2015. He received three cycles with dose reduction secondary to side effects such as diarrhea. After the third cycle, he refused additional chemotherapy and requested evaluation at Thomas Jefferson University Hospital (TJUH) for possible surgical resection. A new CT scan was obtained, and although it showed a slight decrement in the size of the tumor, he was still deemed unresectable, and it was recommended that he continue chemotherapy. The patient resumed chemotherapy in late December with 5-fluorouracil, leucovorin, and oxaliplatin (FOLFOX). The patient tolerated this regimen remarkably well and completed six such cycles by March 2016, for a total of nine cycles of neoadjuvant chemotherapy. A repeat CT scan of the abdomen () showed continued partial response, with the pancreatic body mass now measuring only 2.0 × 1.3 cm (vs. 3.1 × 2.0 cm in December) and the dominant celiac lymph node now measuring only 1.7 × 1.6 cm (vs. 2.5 × 2.3 cm in December). He was reevaluated at TJUH, and this time he was deemed suitable for attempted surgical resection. Notably, his serum levels of CA19-9 (11 U/mL), carcinoembryonic antigen (1.6 ng/mL), and lipase (15 U/L) were all within normal limits immediately before resection. The operation was performed at TJUH on April 2016. Due to the involvement of the celiac axis, the patient underwent distal pancreatectomy with en bloc splenectomy and en bloc celiac artery resection (Appleby procedure). The patient tolerated the procedure well, the postoperative course was uncomplicated, and he was discharged on postoperative day 7. Final pathology analysis showed negative resection margins and extensive chemotherapy effect, with only a single 0.3 cm focus of residual grade 1 carcinoma in a large peripancreatic lymph node. No carcinoma was identified in the pancreatic body proper, indicating a complete response in the pancreas. The residual tumor cells in the lymph node were analyzed by immunohistochemistry (IHC). These cells showed positive staining for trypsin and negative staining for synaptophysin, chromogranin, and beta-catenin, strongly supporting the previous diagnosis of acinar cell carcinoma ().
pmc-6319680-1	A 74-year-old female patient presented with a locally advanced tumor of the proximal pancreatic body. The patient showed no signs of obstructive jaundice and did not require biliary stenting. Initially, her tumor was noted to involve the major visceral vessels, including the celiac artery, portal vein, and splenic artery and vein (). The tumor was deemed unresectable and the patient underwent extensive chemotherapy with multiple cycles of gemcitabine, Abraxane, 5-fluorouracil, Alloxantin, Avastin, and Xeloda. After showing a favorable response, she was referred for surgical re-evaluation. The post neoadjuvant chemotherapy CT scan () showed a poorly defined infiltrative pancreatic neck and body mass measuring ∼25 × 15 mm with ill-defined soft tissue encasing the proximal splenic artery, common hepatic artery (CHA), distal celiac axis, and superior mesenteric artery (SMA). The mass also partially encased the portal vein and superior mesenteric vein (SMV). There was no radiologic evidence of hepatic metastasis and there was mild pancreatic duct dilatation. Pre-treatment and post-treatment serum CA 19–9 levels were 46 and 9, respectively. The patient did not receive any additional studies to assess GDA flow or the need for preoperative coiling/embolization. The absolute need for resection of the celiac axis was not determined until the time of the operation. The patient's functional status, perioperative risk, and likelihood of response were weighed and she was determined to be a candidate for an attempt at a modified Appleby procedure. After being properly informed of her various treatment options, she elected to undergo the operation. Exploration of the duodenum and pancreas revealed a firm mass in the body of the pancreas with a soft pancreatic neck. No gross evidence of metastatic disease was present. There was a benign appearing lesion in the superior aspect of liver segment II, which was excised in its entirety and confirmed to be benign. Cholecystectomy was performed, followed by Kocherization of the duodenum and dissection of the pancreas and major vessels. We were fastidious in preserving the GDA. The splenic artery was controlled distal to the tumor, leaving a normal CHA pulse. The spleen and pancreatic body and tail were then serially elevated out of the retroperitoneum. The aorta was exposed, taking down the diaphragmatic crura. The celiac artery was identified at its origin, tied, divided, and oversewn with 5–0 polypropylene suture. Abnormal soft tissue was palpated along the proximal CHA. In light of this vessel involvement, we divided the distal CHA near the GDA. We then dissected the specimen off the SMA successfully and divided the pancreatic neck. The inferior mesenteric vein was then clamped and tied. The inflamed pancreatic neck and proximal body were then dissected free from the right lateral aspect of the SMV and portal vein. The splenic vein was taken flush with the SMV, and its stump was oversewn, leaving good forward flow of the SMV to the portal vein. The proper hepatic artery (PHA) and the GDA were preserved throughout the resection. A Doppler ultrasound probe was used to test the GDA and PHA. Both arteries had adequate signal and in fact, the PHA had a faint palpable pulse. The liver parenchyma was also found to have a strong arterial signal. The specimen had two short stitches and purple dye placed at the neck margin and the rest of the specimen was inked per our Jefferson protocol. Pathology of the resected specimen showed ductal adenocarcinoma with marked treatment effects with invasion of tumor into the peripancreatic soft tissue. The excision margins were free of neoplasia and the specimen had no regional lymph node metastasis (0/26). The tumor was within 2.0 cm from the pancreatic resection margin and within 0.1 cm of the circumferential margin in the posterior peripancreatic tissue. The maximum diameter of the tumor was 3.0 cm in size. In the initial postoperative period, the patient had a transient transaminitis. A postoperative hepatic vascular ultrasound showed good hepatic arterial flow through the PHA and the transaminases normalized. The patient was discharged on postoperative day 9. She remains well without evidence of disease 7 months postoperatively, and has elected to receive no further chemotherapy.
pmc-6319684-1	Patient A.N. is an 83-year-old woman who was living in Iran in January of 2015 when she presented to a local hospital with painless jaundice and dark urine. A CT scan showed a pancreatic head mass compressing the common bile duct. For palliation of hyperbilirubinemia, a palliative stent was placed in the common bile duct. A fine needle aspirate obtained through an endoscopic ultrasound (EUS) revealed a poorly differentiated adenocarcinoma. A follow-up CT scan performed on February 13, 2015 revealed a 3.6 × 2.7 cm pancreatic head mass that abutted the superior mesenteric artery and one of its proximal branches. There were suspicious peripancreatic and retroperitoneal nodes and no evidence of distant disease. At the beginning of March, she began taking 50,000 U vitamin D3 (cholecalciferol) daily, ordered directly from the Internet and not under the direction of a healthcare practitioner. Repeat EUS and biopsy on March 3, 2015 again confirmed adenocarcinoma with papillary features. She was referred to an oncologist and surgeon for evaluation. A triple phase CT was done on March 16, 2015 that showed a stable mass without any evidence of involvement of the celiac axis or superior mesenteric artery. Upfront chemotherapy was recommended, with consideration of surgery in the future. She received gemcitabine/protein nanoparticle-bound paclitaxel in March. On day 10 of cycle 1, she developed neutropenic fever complicated by atrial fibrillation with rapid ventricular response. She was intubated for a brief time who then recovered to her baseline functional status. She was discharged on April 19. Based on her frailty, she was deemed to be a poor candidate for surgery and chemotherapy was not resumed. She was referred to radiation oncology for consideration of stereotactic body radiotherapy, but she decided to first pursue alternative therapies. She visited an alternative care clinic in April and began taking chelodium, curcumin, community mushroom blend, viscosin, and naltrexone. She also continued to take her daily dose of 50,000 U vitamin D3. Although she was somewhat inconsistent in taking her regimen prescribed by the alternative therapy clinic, she consistently took the same vitamin D3 dose from April until the time of writing. She was seen first at the Inova Schar Cancer Institute in September 2015. On September 4, 2015, CT scan revealed the pancreatic head mass to be 3.1 × 3.0 cm, slightly smaller than previously with a mild increase in pancreatic duct dilatation. On our first visit on September 5, 2015, we did not obtain a full supplement history, but checked a vitamin D, 25 OH level, which surprisingly was elevated above normal value of >150 ng/mL. Her calcium level was 9.7, which had been 7.7 in March 2015, before starting supplementation. In October, 8 months after her only dose of chemotherapy, she had a CT scan that showed continued disease stability (). As of December 4, 2015, she was stable with no evidence of disease progression by both CT and CA 19-9 level. She was admitted to Fairfax hospital in October of 2015 for diverticulitis with abscess formation that was treated with resection. Currently she describes as feeling quite well with no difficulty accomplishing her activities of daily living.
pmc-6319686-1	The first patient is a 58-year-old man who was a previous smoker who presented to an outside hospital with abdominal pain and was diagnosed with gallstone pancreatitis. However, despite having a laparoscopic cholecystectomy, he continued to have episodes of pancreatitis yearly for the next 3 years. Computed tomography (CT) scan demonstrated a pancreatic duct dilated throughout its course to a maximum size of 8 mm without a discrete pancreatic mass in the head of the pancreas. Endoscopic retrograde cholangiopancreatography confirmed a dilated pancreatic duct with a suspected filling defect of the common bile duct, after which the patient underwent sphincterotomy and stent placement. He then presented to our institution for consultation. Further workup with endoscopic ultrasound (EUS) was performed, which also confirmed the dilated pancreatic duct, but noted irregular contour of the duct. In addition, free mucin was seen exiting the main papilla, and there was a papillary growth noted in the pancreatic duct in the head of the pancreas, all of which was consistent with a main duct IPMN (). Preoperative laboratory values, including CA19-9, were within normal limits. Of note, it was unclear if the dilatation of the pancreatic duct was secondary to obstruction from the mass in the pancreatic head or due to main duct IPMN involving the entirety of the duct. He was scheduled for surgical resection after the stated workup. He was planned for a Whipple procedure (pancreaticoduodenectomy) with possible total pancreatectomy if intraoperatively the main duct was found to be involved by IPMN with high-grade dysplasia. Transection of the pancreatic neck identified cells of high-grade dysplasia free floating near the margin (although exclusive of the margin). In addition, upon probing the pancreatic duct in the tail of the pancreas, the probe did not slide smoothly, suggestive of additional growths in the duct. Due to his young age and high risk of developing pancreatic cancer, a completion pancreatectomy and splenectomy were performed. He was reconstructed in a Roux-en-Y manner. He recovered well from surgery without any postoperative complications. His final pathology demonstrated a 1.4 cm moderately differentiated colloid carcinoma arising within main duct IPMN (intestinal type) with high-grade dysplasia in addition to multiple foci of pancreatic intraepithelial neoplasia. Staining of the tumor was strongly positive for MUC2 and weakly positive for MUC1 and MUC5. The tumor involved the peripancreatic soft tissue, but all margins were negative. 0 of 39 lymph nodes was involved with tumor. His final pathologic staging was pT3N0Mx. He was, therefore, referred for medical oncology consultation. He was recommended for adjuvant chemoradiation with gemcitabine before and after fluorouracil-based chemoradiation, which he is currently receiving and tolerating well.
pmc-6319686-2	The second patient is a 72-year-old woman with a past medical history including hypertension, type II diabetes, and obesity, who presented with weight loss and steatorrhea. As her symptoms were suggestive of pancreatic exocrine insufficiency, and with the associated weight loss, she underwent a CT scan for additional evaluation. This demonstrated a suspicious 2.5-cm complex cystic pancreatic head mass with associated pancreatic gland atrophy and a dilated pancreatic duct. She was also noted to have fatty infiltration of the liver (although not overt cirrhosis) and ascites. She was then referred for further evaluation. Magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography demonstrated diffuse dilation of the pancreatic duct up to 7.5 mm with an associated cystic mass in the head of the pancreas, suggestive of main duct IPMN (). EUS was attempted, but was unable to be completed due to severe tortuosity of her esophagus. Positron emission tomography (PET) demonstrated heterogeneous metabolic activity within the pancreatic uncinate process and to a lesser degree within the body and tail. Additional workup included a paracentesis to evaluate the ascites, which was unremarkable and ultimately deemed secondary to poor nutritional status from her exocrine insufficiency. Laboratory results were within normal limits with the exception of tumor markers and a mildly low albumin (3.4 gm/dL). Carcinoembryonic antigen was elevated at 6.0 ng/mL, and CA 19-9 was elevated at 46.7 U/mL. She was placed on pancrelipase, which resolved her symptoms of exocrine insufficiency and helped improve her nutrition. Due to the concern for main duct IPMN, she was referred for surgical resection. Again, it was unclear if the diffuse dilation of the pancreatic duct was secondary to involvement by main duct IPMN or due to proximal pancreatic duct obstruction. The patient was planned for a Whipple procedure with possible total pancreatectomy if high-grade dysplasia was noted intraoperatively at the resection margin. Upon transection of the pancreatic neck, IPMN with focal high-grade dysplasia was indeed noted at the margin. Due to the abnormal preoperative PET scan, in addition to the high-grade dysplasia at the margin, a completion pancreatectomy was performed. Due to her poor nutritional status preoperatively, a gastrojejunal feeding tube was placed at the time of the operation. She had an unremarkable postoperative course and was discharged with feeding tube supplementation. Her final pathology demonstrated a 1.1 cm well-differentiated mucinous colloid carcinoma arising in a background of IPMN (intestinal type) with foci of high-grade dysplasia and intermediate grade dysplasia throughout the remaining pancreatic duct (). Staining of the tumor was positive for MUC1, MUC2, and MUC5. All margins were negative. Zero of 24 lymph nodes was involved with tumor. Her final pathologic staging was pT2N0Mx. She was referred to medical oncology for discussion of adjuvant therapy and will be initiating gemcitabine adjuvant chemotherapy in the near future.
pmc-6319689-1	A 48-year-old man initially presented with lightheadedness, palpitations, and tarry stools. He was found to be anemic with a hemoglobin of 5.5 g/dL. Upper endoscopy revealed nonbleeding gastric varices. Further workup through abdominal CT and MRI demonstrated a large arterial-enhancing pancreatic mass, thought to be a PNET. No metastases were seen. The mass appeared to abut the splenic vein and portosplenic confluence with evidence of thrombus within the lumen of the portal vein (). Endoscopic ultrasound was performed with FNA of the pancreatic mass. Histopathological review of the FNA sample revealed neoplastic cells, positive for Anti-Pan Cytokeratin Antibody, synaptophysin, and CD56, suggesting PNET. An octreotide scan showed focally increased signal at the site of the pancreatic mass, without dissemination. The patient underwent resection of the tumor through distal pancreatectomy and splenectomy with en bloc portal vein resection and reconstruction as the tumor was directly invading the splenic vein with thrombus extending into the portal vein. The patient tolerated this procedure well and his postoperative course was uncomplicated. Pathology from this initial procedure revealed a grade II PNET with 2/15 adjacent lymph nodes positive for metastatic carcinoma and negative margins with a Ki-67 proliferative index of 15% (grade II). Seventeen months after resection, the patient began to have complaints of fatigue, exercise intolerance, and palpitation. He was again found to be anemic with a hemoglobin of 6.6 g/dL. Before the aforementioned initial resection, serum pancreatic polypeptide and chromogranin A levels had been elevated to 547 pg/mL and 15.8 ng/mL, respectively; with the onset of these new symptoms, the pancreatic polypeptide level was found to be only 226 pg/mL, but the chromogranin A level was elevated to 33 ng/mL (). CA 19–9 levels remained normal throughout the patient's full treatment course. Diagnostic endoscopy to localize the source of bleeding revealed a 6 cm malignant-appearing friable mass with central ulceration in the gastric cardia. CT scan findings confirmed the presence of this gastric soft tissue mass and also revealed a posterior nodular extension toward the upper pole of the left kidney (). This lesion demonstrated uptake on octreotide scan, suggesting a PNET origin; a focus of uptake was also noted in the periportal region, suggesting the possibility of a metastatic lymph node. Upon histopathological review of the material retrieved at the endoscopy, the gastric lesion was identified to be morphologically similar to his previous PNET, with positivity for CAM5.2, chromogranin A, and synaptophysin. After extensive discussion with the patient regarding his treatment options, he opted for reresection through an open total gastrectomy. After exploratory laparotomy and extensive lysis of adhesions, the tumor was noted to invade into the capsule of the left adrenal gland. Both the stomach and the left adrenal gland were removed en bloc. No hepatic lesions were noted. Alimentary tract reconstruction was performed through a Roux-En-Y esophagojejunostomy. The periportal region was skeletonized with removal of all nodal tissue. The patient's postoperative course was uncomplicated. He was ultimately discharged to home on postoperative day 6 on a low-volume clear liquid diet and total parenteral nutrition supplementation. Surgical pathology revealed a pancreatic neuroendocrine carcinoma in the gastric wall and adrenal gland with negative resection margins. The sampled periportal lymph nodes were free of disease. The lesion was again confirmed to be morphologically similar to the patient's previous PNET (). Shortly thereafter, pancreatic polypeptide and chromogranin A normalized to 96 pg/mL and 5 ng/mL, respectively.
pmc-6319690-1	A 63-year-old man presented to the emergency department with a 1-month history of intermittent nausea, chills, and jaundice. Preoperative laboratories revealed an elevated total bilirubin and alkaline phosphatase. Serum tumor marker analysis revealed a normal carcinoembryonic antigen (CEA) but an elevated CA 19-9. The patient underwent a magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography that revealed thickening of the proximal common bile duct with proximal biliary dilatation and an absence of distant disease. Incidentally noted on the MRI was that the third portion of the duodenum did not cross to the left of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV), and the majority of the small intestine lies in the patient's right abdomen, whereas the large intestine lies in the left. Also, the SMA lies to the right of the SMV consistent with gastrointestinal malrotation (). The patient underwent endoscopic retrograde cholangiography with biliary endoprosthesis placement for decompression. Upon surgical exploration, a mass was identified in the proximal common bile duct (with no evidence of disseminated disease) and gastrointestinal malrotation was confirmed. Oncological resection of the extrahepatic biliary tree was performed from the level of the bifurcation of the right and left hepatic ducts to the intrapancreatic portion of the bile duct, along with a portal lymphadenectomy. A biliary enteric reconstruction was created with a Roux-en-Y hepaticojejunostomy. To compensate for the malrotation, the proximal jejunum was divided 50 cm distal to where the duodenum exited the retroperitoneum and the 50 cm Roux limb was brought up in a right paracolic position rather easily, as the hepatic flexure of the colon was absent, such that it simply rested over the top of the duodenum. An end-to-side hepaticojejunostomy was formed in a single layer, and the Roux limb was tacked down to the retroperitoneum to prevent intestinal herniation. Specimen pathology revealed a poorly differentiated adenocarcinoma of the common bile duct with negative surgical margins with negative nodal disease. The patient's postoperative course was complicated by a prolonged ileus, and he was discharged to home on postoperative day 16. The patient underwent 5-fluorouracil-based adjuvant chemotherapy and radiation therapy. He unfortunately developed recurrent malignant disease and died 23 months postresection.
pmc-6319690-2	A 47-year-old man presented to his primary care physician with a 3-month history of intermittent epigastric abdominal pain, nausea, and vomiting. Routine laboratory analysis revealed normal liver function. The patient underwent an abdominal computed tomography (CT) scan, which revealed a 4.2 × 4.1 × 4 cm mass in the head and uncinate process of the pancreas. The scan also showed that the patient's entire colon was on the left side of the abdomen and the small bowel to the right (). Tumor marker analysis showed a normal CEA level and an elevated CA 19-9 level. At operative exploration, the fourth portion of the duodenum was in its normal position; however, the proximal jejunum immediately traveled to the patient's right side and most of the small bowel was to the right of the ascending colon and the cecum located in the pelvis. The SMA was rotated closer to the uncinate process and was encased by tumor making the lesion unresectable. A palliative antiperistaltic gastrojejunostomy and Roux-en-Y hepaticojejunostomy bypass were performed. The jejunal limb was not brought up in the usual retrocolic manner but in a right paracolic position due to the abnormal colonic location. Intraoperative biopsy of the palpable mass revealed a moderately differentiated PDA. The patient underwent gemcitabine-based chemotherapy and radiation postoperatively with successful downstaging of the tumor. He underwent operative re-exploration with successful resection of his tumor through a classic pancreaticoduodenectomy ∼12 months after his initial exploration. Pathology of the resected specimen revealed a PDA with perineural tumor involvement at the uncinate process margin. Three out of the 13 lymph nodes examined were positive for metastatic disease. The patient was discharged from the hospital on postoperative day 8. He developed recurrent malignant disease and died 10 months postresection, that is 22 months following the initial exploration.
pmc-6319690-3	A 67-year-old Caucasian woman presented to her primary care physician with a 1-year history of epigastric abdominal pain, occasional nausea, and emesis, and an unintentional 10 pound weight loss. An abdominal CT scan showed a 4.5 × 4.1 cm periampullary mass arising from the pancreas without evidence of metastatic disease. Interestingly, the CT scan also showed the colon to be in a dorsal position in relation to the SMA (). Laboratory analysis revealed normal liver function tests and serum CEA level but an elevated CA 19-9 level. At operative exploration, the entire transverse colon was found to be retroperitoneal and lies dorsal to the SMA and SMV. The duodenum coursed anterior rather than posterior to the mesenteric vessels. The SMA lies further to the right than normal and the SMV–portal vein confluence lies further to the left than usual. Inspection of the duodenum and head of the pancreas revealed a firm mass in the head of the pancreas. A classic pancreaticoduodenectomy was performed. The anterior placement of the duodenum made division of the jejunum beyond the ligament of Treitz relatively easy. The tumor dissected nicely away from the SMA with no uncinate process of the pancreas present. Our standard procedure is to bring the jejunal limb for reconstruction up in a retrocolic position, through a defect made in the transverse mesocolon to the right of the middle colic vessels. This limb is used to create the pancreaticojejunostomy, hepaticojejunostomy, and gastrojejunostomy. Due to the retroperitoneal position of the transverse colon, we brought the jejunal limb up in an antecolic position. Pathology revealed a moderately differentiated PDA with all surgical margins being free of tumor and 1 out of 11 specimen lymph nodes was positive for metastatic disease. The patient tolerated the procedure well and was discharged to home on postoperative day 5. The patient eventually developed recurrent disease and died 34 months postresection.
pmc-6319691-1	A previously healthy 14-year-old male presented to an emergency room with 3–4 days of diarrhea and abdominal pain in November 2014. Abdominal X-ray demonstrated dilated loops of bowel consistent with gastroenteritis. His serum aspartate transaminase was slightly elevated at 65 U/L (ULN 37); however, his remaining hepatic function panel, complete blood count, lipase, and amylase were normal. An abdominal ultrasound suggested a cystic structure medial to the left kidney with thickened internal septations. Despite repeating the abdominal ultrasound in multiple planes, it remained unclear whether the structure represented a distended fluid-filled loop of colon or a unique cystic lesion. Subsequent computerized axial tomography () revealed a multiseptated cystic structure in the pancreatic tail measuring 3.5 × 4 × 5 cm with peripheral hyperdensities compatible with extrapancreatic calcifications. Several of the cystic components demonstrated thickened walls with mild enhancement. The pancreatic parenchyma and pancreatic duct appeared normal. No other pancreatic lesions were identified. Multiple mildly dilated fluid-filled loops of large and small bowel with air-fluid levels compatible with gastroenteritis were also noted, which were felt to be the likely etiology of his symptoms. The patient's symptoms resolved shortly following his emergency room visit, with normalization of his stools and resolution of his abdominal pain. After careful review of his images, his pancreatic lesion was felt to represent a cystic pancreatic mass and less likely a pseudocyst or a solid pseudopapillary tumor with cystic components. He was referred for an endoscopic ultrasound (EUS) to further characterize his lesion. An EUS () performed in December 2014 noted a 2.2 × 2.2 cm thick-walled pancreatic tail lesion with a hypoechoic center and several adjacent anechoic lesions, the largest of which was 2.3 × 1.3 cm. The pancreas otherwise appeared normal. Fine needle aspiration of the largest anechoic lesion was performed using a 22G Expect™ BSCI needle. One milliliter of pink tinged nonviscous fluid was aspirated. Cyst fluid analysis revealed carcinoembryonic antigen 7.1 ng/mL and amylase 633 U/L. Aspirate cytology was nondiagnostic with rare small groups of mildly atypical epithelial cells, benign acinar cells, ductal cells, and fragments of fibrous tissue and debris. RedPath PathFinderTG® integrated diagnosis was also indeterminate, due to lack of polymerase chain reaction amplifiability from low DNA quantity and poor DNA quality. The patient felt generally well and remained active in several sports, but continued to have episodes of recurrent abdominal pain. Repeat contrast-enhanced abdominal computed tomography in July 2015 demonstrated a persistent pancreatic cystic lesion measuring 2.6 × 3.1 × 1.8 cm, with prior imaging not available for comparison at that time. Although a definitive diagnosis had not been established, due to his recurrent pain and persistent lesion, the patient underwent laparoscopic excision in July 2015. The patient tolerated the procedure well. The lesion was easily dissected from the pancreas. Macroscopically, it was noted to have four cystic components, ranging in size from 0.8 × 0.7 × 0.4 to 2.6 × 1.6 × 1 cm. Each cyst contained a white smooth wall filled with white cloudy watery fluid. Microscopically, the cysts were lined by a single layer of cuboidal to columnar epithelium with focal acini, with one cyst demonstrating internal concretions (). No cytological atypia or mitoses were present. These features were consistent with a multilocular acinar cell cystadenoma.
pmc-6319692-1	A 66-year-old Caucasian male presented with obstructive jaundice in January 2015. He was found to have a hypodense mass in the head of pancreas and underwent biliary stent placement. Endoscopic ultrasound and fine needle aspiration revealed adenocarcinoma of the pancreas. The tumor was staged as IB, cT2cN0cM0. The patient was scheduled for a Whipple procedure at the end of February. At surgical exploration, empyema of the gall bladder, pyobilia, and locally advanced tumor were discovered. Surgical resection was aborted and Roux-en-Y hepaticojejunostomy was performed for optimal biliary drainage. No excessive bleeding was encountered during surgery. Broad spectrum antibiotics were started intraoperatively and continued perioperatively. Preoperative hematological evaluation was within normal limits. Postoperatively, the patient developed bleeding from drains and intravenous line sites. Prothrombin time was mildly increased at 13.9 sec and activated partial thromboplastin time (aPTT) was elevated at 85 sec. A 1:1 aPTT mixing study did not correct, suggesting the presence of an inhibitor. Further analysis showed a factor VIII level of 1% and factor VIII inhibitor titer of 14.3 Bethesda units (BU). Specific tests such as lupus anticoagulant, dilute Russel viper venom test, thrombin time, and other coagulation factors were within normal limits ( and ). The patient developed abdominal wall ecchymoses, and multiple intra-abdominal hematomas were detected on axial CT imaging. His hemoglobin dropped from 13.7 to 7.4 g/dL and was supported with packed red blood cell (PRBC) transfusions. He was immediately started on recombinant activated factor VII (rFVIIa) and intravenous immunoglobulin (IVIG) therapies, which controlled bleeding but inhibitor titer increased to 37 BU. Cyclophosphamide was initiated at a dose of 2 mg/kg body weight (200 mg) daily. The inhibitor titer peaked at 243.9 BU and factor VIII at nadir was <1% (). He required 6 units of PRBCs. The patient was discharged on a regimen of prednisone and cyclophosphamide. He was readmitted for gastrointestinal bleeding and symptomatic anemia. Factor VIII was still <1% and the inhibitor titer was 142.9 BU. A descending colonic source of presumed diverticular bleeding was identified and managed with PRBC transfusions and restarting rfVIIa. No interventional procedure was performed due to the risk of catastrophic bleeding. Rituximab was initiated at 375 mg/m2 weekly for four doses. Factor VIII inhibitor bypassing activity (FEIBA) was then started due to the frequent need of transfusions. He required 13 units of PRBCs. He was discharged on a regimen of rfVIIa, cyclophosphamide, prednisone, and completed four doses of rituximab. The factor VIII levels improved to 13% and inhibitor titer decremented to <0.6 BU (). Moderate thrombocytopenia was detected on follow-up with platelet count of 35,000/μL. The cyclophosphamide dose was decreased to 150 mg daily. The platelet count dropped further to 4000/μL and the patient developed wet purpura in the oral cavity. As RBC and WBC counts were stable, he was initiated on IVIG for ITP, resulting in normalization of platelet count (). He was discharged on a regimen of cyclophosphamide and slow steroid taper. Repeat laboratories showed a decline in WBC count to 200/μL, with a normal platelet count and hemoglobin. This was diagnosed as autoimmune neutropenia. The steroid dose was increased to 60 mg daily, cyclophosphamide stopped, and granulocyte-colony stimulating factor (G-CSF) initiated (daily for four doses), with prompt recovery of WBC counts (). He was evaluated by a multidisciplinary team of hematology, and medical, radiation, and surgical oncology. After blood count recovery, a short course of radiation therapy was started for pancreatic cancer followed by chemotherapy.
pmc-6319693-1	A 71-year-old woman initially presented to an outside hospital in December 2015 with vague epigastric discomfort. A chest computed tomography (CT) was obtained to evaluate the possibility of a pulmonary embolism, and a calcified cyst was identified in the tail of the pancreas. Of note, laboratory tests were notable for a serum amylase level of 402 U/L and a lipase level of 553 U/L. The patient underwent additional imaging before surgical consultation to further characterize the pancreatic lesion, including an abdominal ultrasound, CT, and magnetic resonance imaging (MRI). The patient was then referred for a surgical evaluation. At the time of her visit, she was asymptomatic, without abdominal pain, back pain, steatorrhea, jaundice, weight loss, or nausea. Her physical examination did not reveal any abnormalities. Pertinent laboratory data included a serum CA 19-9 level of 35 U/mL, HbA1c level of 6.1%, repeat amylase level of 378 U/L, and repeat lipase level of 439 U/L. A review of her abdominal imaging revealed a 3 cm complex cystic mass in the pancreatic tail, with thickened septa, and an associated solid nodule that appeared calcified, and was associated with central scarring (). These findings were suggestive of a benign serous cystadenoma. Peripheral enhancement was observed on an MRI (), raising the possibility of a mucinous cystic neoplasm as well. Peripancreatic edema on CT () was interpreted as evidence of acute pancreatitis, further confounding the clinical picture. Endoscopic ultrasound and aspiration of the cyst were not performed because of anatomic inaccessibility related to the cyst's location near the splenic hilum, and away from the posterior wall of the stomach. The patient was offered resectional therapy based on the fact that her symptoms and serum tests reflected pancreatitis, attributable to the pancreatic cyst. The possibility of a mucinous neoplasm and a serum CA 19-9 level at the upper range of normal also factored into the decision. A laparoscopic distal pancreatectomy with en bloc splenectomy was performed without incident, although the dissection was challenged by the peripancreatic inflammation observed on imaging, which obscured the natural plane between the splenic vessels and the pancreas. On gross inspection of the resected specimen, the lesion had the appearance of a solid mass with infiltrating tentacles radiating toward the distal resection margin. A cystic component was not immediately apparent. Microscopic examination revealed desmoplastic stroma, which distorted the glands, and atypical epithelium () infiltrating toward the edge of the resection margin. These findings were interpreted to be consistent with invasive ductal adenocarcinoma, and background chronic pancreatitis. The proximal pancreatic neck margin was believed to be microscopically negative for invasive cancer. However, on gross inspection, abnormal tissue extended up to the transected parenchyma, leaving a possibility for a revised diagnosis of a positive resection margin on final pathological review. In light of these unexpected results, the surgical team contemplated converting to an open operation to resect additional parenchyma, and ensure a safe and complete resection in the context of pancreatitis, and a difficult laparoscopic dissection. However, after discussing with the family that the frozen section review was discordant with preoperative imaging, the decision was made to terminate the procedure, close the laparoscopic port site incisions, and defer any further management decisions regarding a more extensive resection until the final pathological review was finalized. Parenthetically, the intraoperative pathological review and family discussion added roughly 30 min to the operation. The postoperative course was uneventful, and the patient was discharged on the third postoperative day. At 6 months follow-up, the patient is well. In the final analysis, the lesion was determined to be a calcified microcystic serous cystadenoma with background chronic fibrosing pancreatitis. There was no evidence of invasive adenocarcinoma ().
pmc-6319694-1	A 53-year-old woman presented with progression of a known cystic lesion within the head and uncinate process of the pancreas. After a diagnosis of type 2 diabetes in 2006, an MRI of the abdomen revealed a 4.4 × 4.1 × 4.7 cm unilocular nonenhancing cystic mass. In 2015, the patient underwent a second MRI of the abdomen as part of a workup for weight-reduction surgery, which showed a centrally enhancing, multilobulated, and septate appearing cystic mass measuring 5.0 × 4.4 × 5.8 cm. Additional findings included several nonenhancing subcentimeter cystic lesions in the body of the pancreas, atrophy of the tail, and periportal adenopathy with nodes measuring up to 3.5 × 1.9 × 4.8 cm (). Endoscopic ultrasonography confirmed the presence of mural nodularity within the cystic tumor. Preoperative laboratories were notable for a slightly elevated cancer antigen 19.9 of 39 U/mL (normal range: <35 U/mL). Given the size of the cyst, mural nodularity, and associated periportal adenopathy, there was a suspicion of potential malignant degeneration of the tumor. The patient was advised to undergo surgical resection and this was successfully accomplished with a pylorus-preserving pancreaticoduodenectomy with standard reconstruction as previously described. The patient tolerated the procedure well, had an uneventful hospitalization, and was discharged to home on postoperative day 7. Postoperative histological sections showed cystic dilation of the main pancreatic duct and side branch ducts. The majority of these ducts were lined by papillary mucinous epithelium consistent with IPMN and showed focal areas of high-grade dysplasia, characterized by cribriform architecture, complex papillary fronds, nuclear stratification, nuclear hyperchromasia, and loss of nuclear polarity. There was also a moderate degree of periductal parenchymal fibrosis and atrophy, indicative of chronic pancreatitis. All ducts retained a lobular configuration, precluding the diagnosis of invasive carcinoma. Admixed within the ducts lined by mucinous epithelium were cysts lined by cuboidal epithelium with prominent eosinophilic cytoplasmic zymogen granules and prominent nucleoli, consistent with an ACA. These cysts showed strong immunohistochemical labeling for trypsin, further supporting an acinar origin; the cysts lined by mucinous epithelium, however, were negative for trypsin. There was no evidence of dysplasia within the acinar epithelium (). The striking morphological differences between the two epithelial cyst linings supported the diagnosis of synchronous lesions, with distinct components of both an IPMN and ACA. Both processes seemed to contribute to pancreatic duct dilation.
pmc-6319695-1	A 62-year-old woman undergoing presurgical evaluation for an olfactory groove meningioma resection was incidentally found to have a pancreatic mass on an abdominal CT in April 2009. The 2.9 × 2.5 cm mass was centered in the pancreatic body with a round shape, well-defined margins, and avid homogenous enhancement (). There was mild upstream pancreatic duct dilation. There was no lymphadenopathy in the abdomen and pelvis or other signs of another primary tumor or metastatic disease. Differential considerations offered included either a neuroendocrine tumor or a vascular abnormality such as an aneurysm or pseudoaneurysm. An endoscopic ultrasound performed at the time revealed a 2.2 cm well-defined solid hypoechoic mass in the pancreatic body, suspicious for a pancreatic neuroendocrine tumor. Fine needle aspiration of this mass showed no evidence of a malignancy, but only changes of chronic pancreatitis. The patient's abdominal physical examination was normal, and she denied any abdominal pain, weight loss, diarrhea, or constipation. Repeat CT in October 2013 demonstrated mild interval increase in size of the mass to 3.0 × 3.0 cm. An endoscopic ultrasound was repeated in December 2013, confirming an increase in size of the solid mass (). Repeat fine needle aspiration of the mass yielded only atypical epithelial cells. The patient's complete blood count, complete metabolic panel, and plasma carcinoembryonic antigen levels were all within normal limits. Urine catecholamine metabolite levels were never checked presurgically as the patient exhibited no symptoms or signs of excess catecholamine production. Hence, metaiodobenzylguanidine (MIBG) scintigraphy was also not performed. The patient underwent a laparoscopic distal pancreatectomy and splenectomy in March 2014 for excision of the mass. A well-circumscribed tan mass measuring 2.8 × 2.8 × 2.7 cm was found on the posterior aspect of the pancreas and was diagnosed as a paraganglioma through pathology. The rest of the pancreas and spleen were unremarkable. The patient tolerated the procedure well, but developed a pancreatic duct leak after the surgery. This was treated with a biliary sphincterotomy and placement of a pancreatic duct stent, which led to complete resolution of the leak. Plasma metanephrine and normetanephrine levels when checked 2 weeks status postsurgery were within normal limits. Multiple repeat CT scans since the surgery, with the most recent one in September 2015, showed no evidence of recurrent disease or lymphadenopathy, and the patient continues to do well.
pmc-6319699-1	The patient is a 55-year-old female with a body–mass index (BMI) of 23 kg/m2, a 20 pack-year smoking history (quit 9 months before presentation), no history of alcohol abuse, and an unremarkable past medical history, who presented for evaluation of newly developed nausea. Her laboratory tests and computed tomography (CT) imaging were all suggestive of biliary obstruction, and she was ultimately diagnosed with an invasive ampullary adenocarcinoma. Staging workup revealed no evidence of metastatic disease, so she was taken to the operating room for a classic PD. A retrocolic hepaticojejunostomy (HJ) was constructed. Similarly, a retrocolic pancreaticojejunostomy (PJ) was created using a duct-to-mucosa technique over a pediatric feeding tube, which was brought out through the lumen of the jejunum and the abdominal wall for external drainage. The pancreatic parenchyma was noted to be very soft, and the pancreatic duct was 2.5 mm in diameter. Estimated blood loss (EBL) was 250 mL. Two 19F Blake drains were left in place above and below the HJ and PJ. Postoperatively, her diet was advanced to soft solids by postoperative day (POD) 4, at which point daily amylase levels of her Blake drainage were checked. One Blake drain was removed on POD5 given an amylase level of 513 U/L. Her remaining Blake drain's amylase level the following day was 6160 U/L, so she was discharged home with plans to remove it in clinic. She was seen on POD13, at which time the drain had an output of <20 mL per day for the past 3 days, so it was removed. She re-presented to our institution on POD23 in transfer from an outside hospital. She endorsed 4 − 5 days of increasing epigastric pain, and a CT of her abdomen and pelvis (CT A/P) demonstrated subphrenic collections, for which she underwent CT-guided placement of two drains. Fluid amylase was 28 U/L and cultures grew Escherichia coli and Klebsiella, so she was started on IV antibiotics. Her serum amylase and lipase were 51 and 141 U/L, respectively. One drain was removed by the Interventional Radiology (IR) service 3 days after placement and the second one on follow-up 1 week after discharge, 13 days after placement. She proceeded to adjuvant chemotherapy 7 weeks after her operation and received five cycles of gemcitabine, which she completed successfully. She did not receive any adjuvant radiation. Approximately 8 months after her original operation, she again presented to our Emergency Department with right upper quadrant and epigastric abdominal pain. Her laboratory tests were notable for a white blood cell (WBC) count of 13K/μL and lipase 522 U/L. A CT A/P demonstrated diffuse peripancreatic inflammation. Her pancreatic enzymes rapidly decreased with bowel rest, IV fluid hydration, and supportive care, so endoscopic retrograde cholangiopancreatography (ERCP) was deferred at that time. She was discharged home on hospital day 6 feeling better. Unfortunately, she was again admitted 2 weeks later with similar symptoms. Laboratory tests were notable for a WBC of 17K/μL, amylase 444 U/L, and lipase 677 U/L, and a CT A/P noted a prominent proximal pancreatic duct to 4 mm as well as a 4.6 × 3 cm collection near the PJ (), for which she underwent CT-guided drain placement. The fluid drained had an amylase and lipase of 21,860 and 86,650 U/L, respectively, and cultures were sterile, findings consistent with a pancreatic fistula. She underwent an ERCP and attempt at pancreatic duct cannulation, which was unsuccessful due to the inability to identify the latter from within the jejunum. Four days later, she underwent a repeat endoscopy and a complex rendezvous procedure was performed. The pancreatic duct was identified from within the stomach using endoscopic ultrasound (EUS), with subsequent transgastric transpancreatic passage of a wire into the duct and out to the jejunum. This was followed by ERCP and rendezvous identification of the wire, dilation of the PJ using a 4-mm Titan balloon, and placement of a 7F 9-cm Hobbs stent with an internal flange at the PJ with good drainage (). Her symptoms rapidly improved, and she was discharged home on hospital day 8.
pmc-6319784-1	A 41-year-old man presented to a local hospital with upper and lower extremity motor disturbances in 2014. Based on a mild reduction in nerve conduction velocity, he was diagnosed with polyneuropathy. In 2016, he experienced 3 episodes of pleurisy with fever (body temperature >38°C) at 2-month intervals. Because he was both myeloperoxidase (MPO)-ANCA and proteinase-3 (PR3)-ANCA-positive, he was referred to our hospital in June 2017. His family history was unremarkable and he had no history of drinking. However, the patient had smoked 20 cigarettes per day for 25 years. His medication included 50 mg/day losartan and 5 mg/day amlodipine for hypertension. Since 2006, he had also been treated with 100 mg/day PTU for hyperthyroidism. His laboratory test results during the first visit were as follows: white blood cell count, 3980/μL; C-reactive protein (CRP), 1.39 mg/dL; MPO-ANCA, 10.20 EU/L; and PR3-ANCA, 6.76 EU/L. PTU-associated AAV was suspected at that time, and PTU was discontinued. After discontinuation of PTU, CRP levels normalized, but the numbness did not improve. In November 2017, he was admitted to our hospital with chest pain and high fever. His body temperature was 37.8°C and blood pressure was 158/109 mm Hg. His consciousness was clear, and he had no signs of neurological, respiratory, or abdominal involvement. Pleural friction rubs were not heard. Manual muscle strength testing was normal. Laboratory findings on admission were as follows: white blood cell count, 6760/μL; CRP, 8.65 mg/dL; creatine kinase, 512 U/L; PR3-ANCA, 6.88 IU/mL; and MPO-ANCA, 12.7 IU/mL. Computed tomography showed longitudinal enlargement of the mediastinal lymph nodes and pleural effusion, indicating pleurisy. After admission, his fever and chest pain improved, and CRP levels decreased spontaneously. As his clinical course was consistent with a major Livneh criterion (typical attack with unilateral pleuritis), he was diagnosed with FMF.[ Genetic analysis also revealed compound heterozygous mutations in exon 2 of the MEFV gene (L110P/E148Q). After initiation of 0.5 mg/day colchicine, his numbness improved, and CRP levels declined to a normal range in March (Fig. ). Fever did not recurrent after the administration of colchicine.
pmc-6319785-1	A 28-year-old man presented to the emergency department of our hospital complaining of sudden painful loss of vision in the left eye. He gave a history of hit by a sharp metallic object on the left eye while hammering an iron plate. His left eyelids were mildly swollen, but the orbital rim was intact with no crepitation. Visual acuity in the left eye was limited to the perception of hand motion, while the visual acuity in the right eye was 20/20, and the left intraocular pressure (IOP) was not measurable. Slitlamp examination revealed a full thickness scleral laceration of 4.0 mm length with prolapsed uveal tissue, a shallow anterior chamber, and a traumatic cataract. The details of the posterior segment could not be visualized. Computed tomography (CT) demonstrated an intraorbital foreign body with intensity of iron that had passed through the left eyeball and was located in the intraorbital space close to the optic nerve (Fig. ). The right eye was normal. This study was conducted at the Shandong University Qilu Hospital and the procedures used were approved by the Ethics Committee of the Shandong University Qilu Hospital. The procedures conformed to the tenets of the Declaration of Helsinki. The primary repair of the scleral perforation with abscission of the prolapsed and necrosed uveal tissue was done on the emergency basis. The conjunctiva was incised along the limbus cornea, the sclera was exposed, and the scleral laceration was confirmed and sutured. We actually attempted to maneuver the foreign body behind the eyeball with the use of a magnet, but this was not successful. We therefore severed medial rectus muscle. An iron foreign body was found and was removed in a single piece (Fig. ). From outside of the eye, the exit laceration could not be confirmed. The operation was completed without a scleral suture of the exit laceration. Postoperatively, the intravenous antibiotics were administered, topical antibiotics and steroids with cycloplegics. Visual acuity in the left eye was perception of hand motion, the wound was healthy with intact sutures, the anterior chamber was formed, and the lens was cataractous with no view of the retina. The IOP in the left eye was 13 mm Hg. Removal of the foreign body was confirmed by a postoperative CT scan. Twelve days later, the patient underwent pars plana lensectomy and 3 ports (23-gauge) pars plana vitrectomy. We observed a scleral exit laceration near the optic disc, but did not see any objects. The full-thickness posterior pole defect was closed. With endolaser photocoagulation and silicone oil injection, the patient had his retina reattached. On follow-up after 4 weeks, the visual acuity was always limited to hand motion perception, scleral sutures were intact. The anterior chamber was formed. The IOP was 15 mm Hg. At 3 months after the operation, visual acuity in the left eye was the perception of hand motion and the left IOP was 15 mm Hg (noncontact tonometer). There were no postoperative complications (including retinal detachment, proliferative vitreoretinopathy, infection, sympathetic ophthalmia, and hemorrhage).
pmc-6319865-1	A 52-year-old man was admitted to our center in late September 2017 presenting with shortness of breath as well as facial and lower limb edema. The patient was diabetic (10-year diagnosis), and was found to have had a stroke 3 months prior to his admission, resulting in slight dullness of speech. His electrocardiogram showed persistent AF (CHA2DS2-VASc score: 4). Renal function serum album levels were within normal. However, his echocardiography showed an enlarged left atrium (55 mm × 70 mm), reduced ejection fraction (44.9%), and a suspected mass (8.0 mm × 8.4 mm) in the left atrium (Fig. A). Upon further evaluation, a normal coronary artery angiography (CAG) made the diagnosis of coronary heart disease unlikely. Subsequently, a cardiac magnetic resonance (CMR) evaluation confirmed an enlarged left atrium, with evidence of diffuse late gadolinium enhancement (LGE) in the left ventricle (Fig. ) and a left atrial thrombus. Based on the clinical presentation, we prescribed diuretics (furosemide 20 mg bid), ACEI (Ramipril 2.5 mg Qd), a β-blocker (metorol 49.5 mg Qd) as well as a NOAC, rivaroxaban (20 mg daily). In response to treatment, the patient's shortness of breath and edema quickly improved. After approximately 1 month (early November 2017), we performed a follow-up transthoracic echocardiography (TTE) examination that showed that the mass within the left atrium had increased in size (8.09 mm × 8.36 mm), which was further confirmed by transesophageal echocardiography (TEE). In response, we started treatment of the thrombus with a different NOAC, dabigatran (150 mg bid). During a follow-up examination in December 2017, both TTE and TEE examinations confirmed that the thrombus had decreased in size (4.36 mm × 6.02 mm) and had completely vanished in January 2018 (as shown in Fig. ), after which AF ablation was performed to safely restore sinus rhythm. In response to its successful treatment of the thrombolytic mass, dabigatran (110 mg bid) was prescribed to this patient as a long-term strategy for stroke prevention. Following up with the patient over the next 4 months, he was free of ischemia or bleeding events.
pmc-6319876-1	A 71-year-old elderly woman was admitted to Shandong Sunshine Union Hospital, Shandong, China, on November 10, 2017, due to chest tightness and breathing shortness after activity for 1 year and coughing for 2 months. The patient began to have chest tightness and breathing shortness after general physical activity 1 year ago and coughing (dry coughs mainly) 2 months ago. A small amount of white sputum was noted after a severe cough, which was heavy at night and light in the daytime. The patient was diagnosed with “severe aortic stenosis” using color Doppler echocardiography at several hospitals, and was admitted to the aforementioned hospital with “severe aortic stenosis (bicuspid deformity) and left ventricular outflow tract stenosis,” which was confirmed using transthoracic echocardiography. The patient had a history of hypertension for 10 years and usually took antihypertensive drugs to control blood pressure. The physical examination revealed a clear respiratory sound of bilateral lungs without rhonchus and moist rales and a systolic 3/6 grade murmur in the third intercostals of the left border of sternum. The liver was not enlarged and had no swelling in both lower extremities. The laboratory examination of brain natriuretic peptide was normal. Transthoracic echocardiography showed asymmetric hypertrophy of the left ventricle with a maximum thickness of 20 mm, the diameter of the left ventricular outflow tract of 13 mm, and the anterior flow velocity of the aortic valve of 4.3 m/s. Cardiac MRI revealed myocardial hypertrophy, left ventricular outflow tract stenosis, aortic stenosis, and bicuspid deformity (Fig. ), indicating the requirement of aortic valve replacement and left ventricular outflow tract dredging.[ Intraoperative transesophageal echocardiography (Fig. ) showed aortic stenosis and left ventricular outflow tract stenosis. Surprisingly, the aortic valve was normal without stenosis or insufficiency, and the asymmetrically hypertrophic myocardium under the aortic valve caused left ventricular outflow tract stenosis. Therefore, the diagnosis was changed to hypertrophic obstructive cardiomyopathy. The Morrow procedure was performed to remove the hypertrophic myocardium from the left side of the middle point of the right coronary leaflet to the left coronary leaflet. After the operation, the transesophageal echocardiography examination revealed that the velocity of left ventricular outflow tract decreased to 2.6 m/s and the opening restriction of aortic valve had been corrected (Figure unreserved). Hypertrophic cardiomyocytes were found in the pathology. The conditions of left ventricular outflow tract and aortic valve were observed using echocardiography in the follow-ups 6 months and 1 year after the operation.
pmc-6319971-1	A 30-year-old Chinese woman was referred to the renal division because of frequent urination and dysuria for almost 2 weeks. She had been healthy without any diagnosed diseases, she was not taking any medications, and her clinical examination revealed no significant findings. The laboratory results showed pyuria and bacteriuria, which suggested a clinical diagnosis of urinary tract infection (UTI). The oral antibiotic levofloxacin was used to treat the UTI. After the treatment, the urinalysis was normal except for persistent glucosuria (urine glucose +-2+). Other test results were as follows: fasting plasma glucose (4.74 mmol/L), hemoglobin A1C 5.1%, oral glucose tolerance test (5.4 mmol/L), urine microalbumin/creatinine (9.89 mg/g), serum potassium (4.6 mmol/L), serum sodium (139 mmol/L), chloride (97 mmol/L), and bicarbonate (23.7 mmol/L). Diabetes mellitus, renal tubular acidosis, and Fanconi syndrome were excluded. Then, FRG was taken into consideration. With the consent of the patient, genomic DNA was extracted from peripheral leukocytes. The entire coding region and adjacent intronic segments of SLC5A2 were screened for mutations by genome sequencing (Jinyu, Shanghai). The results showed that the patient had a heterozygous C to A base pair substitution at 1540 in exon 12. This mutation causes a Pro to Ser missense mutation at position 514 (Fig. ). The heterozygous mutation has not been reported before and may not cause mild to moderate glucosuria. To rule out other possible diseases, immunological tests were performed. Surprisingly, the tests showed a high antinuclear antibody (ANA) titer (1:160), an elevated anti-Rho/SSA antibody level, a decrease in C3 and C4, and normal IgG, IgA, IgM, and RF levels. Schirmer test, tear breakup time, and lip biopsy results were all negative (Table ). The patient did not meet the criteria for any known connective diseases. Therefore, she was diagnosed with UCTD. The patient was then treated with Plaquenil (hydroxychloroquine) 100 mg twice a day orally for 6 months. The patient's monthly follow-up urinalysis showed weakly positive to negative glucosuria during 6 months of treatment (Table ).
pmc-6319990-1	An 18-year-old Chinese female patient complained of faciotruncal obesity of 6 months duration, accompanied with irregular menstruation, easy bruising, facial acne and purple striae on her legs. Before she was admitted to our hospital on June 14, 2017, she had amenorrhea for 2 months. Her medical history was unremarkable. During hospitalization, her blood pressure (BP) and blood glucose remained normal (BP: 134/88mmHg, fasting blood glucose 4.8mmol/L). Her body mass index was 26.6 kg/m2. Plasma cortisol concentrations were 20.67 μg/dL in the morning [8am, 571.3nmol/L, normal: 4.4-19.9 μg/dL (121.6-550.0 nmol/L)], 17.67 μg/dL in the afternoon (4pm, 488.7nmol/L) and 18.95 μg/dL in the midnight (0am, 523.8nmol/L), indicated that the normal circadian rhythm was lost. The morning, afternoon and midnight plasma adrenocorticotropic hormone (ACTH) concentration was 1.32 pg/mL (normal: 7.2–63.3pg/mL), 1.37pg/mL, and 1.01pg/mL, respectively. Urinary free cortisol (UFC) concentration was 1824 μg/24 h (normal: 100-379 μg/24 h). During the low dose dexamethasone depression test (LDDST), the morning plasma cortisol slightly increased from 20.67 μg/dL to 25.66 μg/dL, and the UFC after oral dexamethasone was 1388.8 μg/24 h. The patient underwent a high dose dexamethasone suppression test (HDDST), the morning plasma cortisol was 27.52 μg/dL and UFC was 1726.1 μg/24 h after oral dexamethasone intervention. Both morning plasma cortisol and UFC in LDDST and HDDST were not suppressed, supporting the diagnosis of ACTH-independent Cushing's syndrome. Adrenal computed tomography (CT) scan revealed a well-circumscribed round mass with a maximum diameter of 3.0 cm in the left renal hilum, and bilateral adrenal glands were atrophic (Fig. A, Fig. B). The tumor was clinically suspected as ectopic adrenal cortical adenoma. Ultrasound of obstetrics and gynecology was performed to rule out other possible ectopic adrenal rests, and the ultrasound indicated polycystic ovaries (over 12 cysts for each ovary). The patient underwent laparoscopic resection of the tumor. During the operation, the mass in the left renal hilum was completely resected. Postoperative pathology results confirmed the diagnosis of ectopic adrenocortical adenoma. The patient was followed up for 12 months after the operation. She was under glucocorticoid replacement therapy for 8 months, and hydrocortisone 100 mg was intravenously given in the operating day and 2 days after the operation. Oral prednisone (10 mg bid) was given 7 days after the operation for a week and gradually tapered for 8 months duration according to clinical symptoms and morning plasma cortisol. No tumor recurrence or metastasis was found after 12 months of follow-up. The tumor was in size of 3.0cm × 2.5cm × 1.5 cm. Grossly, the mass was round, solid and well circumscribed. Its cut surface was brown (Fig. C). Immunohistochemistry stain showed the tissue was positive for inhibition, Melan-A, synaptophysin, vimentin and AE1/AE3, partially positive for HMB45, angiographic positive for CD34 and negative for NSE and CgA (Fig. ).
pmc-6320009-1	A 25-year-old man was charged with robbery and incarcerated in a prison. One night, his cellmates found the man snoring loudly, cyanotic and unresponsive in bed. They called the prison staff and sent him to the infirmary. He presented with pulseless and cardiopulmonary arrest. Cardiopulmonary resuscitation was performed, but he died. His medical records indicated that he had a history of recurrent episodes of palpitation. The symptom occasionally occurred in the past year without obvious cause. It lasted for a few minutes to half an hour and alleviated by itself. The last episode of the symptom was two months ago. The physical examination revealed a normal blood pressure (120/90 mm Hg) and a pulse rate of 160 beats/minute when the symptoms appeared. The 12-lead resting electrocardiogram (ECG) showed paroxysmal tachycardia and pre-excitation syndrome type B (Fig. ). He was given symptomatic treatment. The man was 172.0 cm in height and 59.0 kg in weight. No significant injuries were observed on external examination except for a few old scars on the knee. The heart weighed 310 g on examination. The left anterior descending artery was located into the myocardial wall for a distance of 1.5 cm and depth was 0.1 cm. The coronary artery revealed no atherosclerotic changes. Focal hemorrhage was observed in the right ventricular myocardium. Other organs did not show any remarkable changes. The toxicology analysis revealed no positive findings. Genomic DNA was isolated from paraffin embedded tissue of the patient and whole exome sequencing was performed to analyze potential genetic variation related to WPW syndrome and SCD. The results showed no exonic mutations in the PRKAG2 gene. Seven potentially pathogenic mutations for SCD, KCNE1, CACNA1C, CASQ2, ANK2, AKAP9, KCNJ5, and TRDN were identified in the patient. The results of the genetic analysis are shown in Table .
pmc-6320063-1	A 47-year-old man presented to the emergency department with abdominal pain and fever. He complained of abdominal pain and weight loss for the past 2 months. His medical history was negative. On physical examination, the abdomen was tender to palpation in the right upper quadrant. Laboratory data showed elevated level of white blood cell (11.23×109/L), alanine aminotransferase (427 IU/L), aspartate aminotransferase (410 IU/L), total bilirubin (TB, 34.57 μmol/L), direct bilirubin (DB, 25.01 μmol/L), glutamyl transpeptidase (1159 IU/L), alkaline phosphatase (416 IU/L) and carbohydrate antigen-199 (84.68 IU/ml). The level of hemoglobin (Hb, 147 g/L), other biochemistry, blood tumor markers, and coagulation studies were all within the normal range. Contrast-enhanced computed tomography (CT) of the abdomen showed a heterogenous lesion of pancreatic head and multiple, high-density, dot-like enhancing vascular structures (Fig. ). Contrast-enhanced magnetic resonance imaging (MRI) of the abdomen and cholangiopancreatography (MRCP) showed early portal venous filling during the arterial phase, a heterogenous lesion located in pancreatic head, but no dilation or filling defect of CBD (Fig. ). Since pancreatic tumor was suspected, lesion biopsy with endoscopic ultrasonography was attempted. However, the procedure failed due to the intolerance of the patient and the abdominal pain got worse. Another CT scan was performed and high-density masses were detected in gallbladder and CDB, which were not showed on previous CT scan (Fig. A). Then, hemobilia was suspected and repeated blood test showed mild anemia (Hb 117 g/L) and further elevation of bilirubin (TB 110.02 μmol/L, DB 56.68 μmol/L).The patient continued to have severe abdominal pain which was speculated to be due to obstruction of bile duct. Hence endoscopic retrograde cholangiopancreatography (ERCP) was performed. The result showed multiple filling defects in the CBD, which were not showed on precious MRCP (Fig. B). Bleeding from papilla of Vater could be directly seen during ERCP (Fig. C). Nasobiliary tube was placed to alleviate the pain and jaundice. After the procedure, pain was alleviated and the serum bilirubin was decreased. Both AVM and pancreatic tumor were considered as the probable diagnosis, and surgical resection was planned. However, two days later after ERCP, the abdominal pain got severe again and the level of Hb fell to 61 g/L. Laparotomy was performed immediately. There were no abnormal findings of the liver, gallbladder, stomach, spleen, or bowel. The head of pancreas was soft but enlarged. Pancreatoduodenectomy was then performed and the unfixed specimen showed a huge ulceration of distal CBD (Fig. A). Macroscopic examination showed the ulceration connected with the honey-comb like collected vessels which were located in pancreatic head and microscopic examination confirmed the presence of arteriovenous malformation (Fig. B and C). The patient recovered uneventfully and was discharged 10 days after the surgery. He is asymptomatic on 4-month follow up. Informed consent was obtained from the patient regarding the publication of his clinical details and images. The study was approved by the Institutional Ethical Review Board of China–Japan Friendship Hospital.
pmc-6320102-1	In May 2017, a 40-year-old Japanese woman presented arthralgia at the left wrist and visited a local orthopedic department. Laboratory investigations showed that her levels of C-reactive protein (CRP) and rheumatoid factor (RF) were within the normal ranges at 0.14 mg/dL and 9.0 IU/mL, respectively, but her level of anticitrullinated protein antibody (ACPA) was high at 101 U/mL. She was then referred to our Immunology and Rheumatology Department for the evaluation of RA. At the patient's first visit to our department, her arthralgia had already disappeared and she had no clinical symptoms. Imaging findings such as X-ray at both hands and feet, the MSUS assessment of peripheral upper limbs, and magnetic resonance imaging (MRI) at both hands showed no findings of synovitis, tenosynovitis, or damage of the joints. After that visit, the patient suffered from recurrent arthralgia lasting for 1 to 2 weeks at the joints of both wrists, elbows, or hips, and the level of RF became positive. However, she showed no findings of sustained arthritis. We thus finally diagnosed palindromic rheumatism. In January 2018, the patient presented sustained arthralgia at toes of both feet and visited our department again. On physical examination, swelling and tenderness in the 2nd to 4th MTP joints of both feet were observed, but MSUS of both feet showed no findings of synovitis or tenosynovitis. However, during the follow-up, the patient had difficulty walking due to the forefoot pain, and she thus came to our department again in March 2018. On physical examination, opening at both the 2nd to 3rd and 3rdto 4th toes with swelling and tenderness in the 2nd to 4th MTP joints of both feet were newly observed (Fig. ). Laboratory investigations showed the following results: white blood cell count 8300/μL (neutrophils 69.2%), hemoglobin 12.4 g/dL, platelet 28.9 × 104/μL, CRP 0.18 mg/dL. antinuclear antibody 80 times (homogenous: 80 times, speckled: 80 times), erythrocyte sedimentation rate (ESR) 12 mm/h, RF 123.5 IU/mL, ACPA 461.3 U/mL. The serum complement level was normal. X-ray examinations of both the hands and feet showed no findings of bone erosion or joint space narrowing. We suspected the presence of intermetatarsal bursitis based on the findings of opening between toes. Accordingly, we performed another MSUS assessment, which revealed showing intermetatarsal bursitis with remarkable PD signals in both the 2nd to 3rd and 3rd to 4th toes with mild synovitis in the right 1st and left 3th MTP joints (Fig. ). The patient did not have a history of mechanical stress to her forefoot that would trigger intermetatarsal bursitis. The patient's findings fulfilled the 2010 American College of Rheumatology and European League Against Rheumatism classification criteria for RA[ due to one small joint involvement, high-positive RF and ACPA, and duration of her symptoms for more than 6 weeks. Although the findings and numbers of her synovitis were mild and few respectively, we diagnosed RA by comprehensive judgment considering intermetatarsal bursitis with remarkable PD signals and administered 6.0 mg/wk of methotrexate (MTX). Immediately after the initiation of the treatment, the patient presented swelling and tenderness in the 2nd MCP joint. We added on 2.0 mg/d of prednisolone (PSL) and increased MTX to 10 mg/wk. After those treatments, the patient's symptoms showed improvement. As of this writing, the patient's remission has been maintained for >2 months.
pmc-6320108-1	A 67-year-old man visited the emergency department of our hospital due to sudden paraplegia of both lower extremities caused immediately after slip down. On presentation to the department, both his lower limbs were observed to be almost paralyzed, with a motor grade of 2 or lower in both limbs. This paralysis showed a gradual aggravation. The patient had a history of renal cell carcinoma diagnosed 7 years prior, and achieved remission without any recurrence after total nephrectomy and adjuvant chemotherapy. He was also diagnosed with papillary thyroid carcinoma 5 years ago, and yet again achieved remission after surgical treatment. The patient signed an informed consent statement, and the study was approved by the Institutional Review Board of the Yeungnam University Medical Center. The plain radiograph revealed the collapse of the T12 vertebral body. CT showed an acute burst fracture at the T12 vertebral body with an osteolytic mass-like lesion within the vertebral body and pedicle, causing severe encroachment of the spinal canal (Fig. ). Magnetic resonance imaging (MRI) revealed a bulging posterior cortex, with an acute fracture of the vertebral body and severe compression of the spinal cord (Fig. ). Considering his medical history and the MRI findings, a pathologic fracture with a tumorous condition was suspected. Therefore, the evaluation of the malignant potential and metastasis in the remote area was necessary. Hence, 18FDG PET/CT was performed, and 18FDG uptake at the T12 level was measured. A maximum standardized uptake value (maxSUV) of 1.7 was noted, with a central FDG defect on the vertebral body (Fig. ); this was interpreted as a benign fracture by a senior radiologist. Additionally, there was no positive uptake in the other body parts on PET/CT. Although there was a discrepancy between the interpretation of the spine fracture lesion on MRI and PET/CT, the patient required emergent surgical treatment owing to acute paraplegia caused by spinal cord compression, and we thus planned an initial surgery for the main purpose of cord decompression, temporary stabilization, and tissue biopsy. The surgical intervention was as follows. First, we performed T11-12 posterior laminectomy for the spinal cord decompression, followed by T9-L2 posterior instrumentation without a bone graft. Finally, tissue biopsy was obtained at the T12 pedicle and vertebral body. Histological examination confirmed the presence of a malignant solitary bone plasmacytoma (SBP) (Fig. ). Seven days later, a definite surgery with pedicle screw fixation and posterolateral bone graft from T8 to L2 without vertebral body corpectomy was performed. Immediately after the initial surgery, the patient's lower extremity motor function showed gradual improvement. After the surgery, the patient wore a thoracolumbar rigid brace for 2 months, and underwent continuous gait rehabilitation. Three weeks postoperatively, the motor function in his lower extremities had recovered sufficiently, and ambulation was possible with the assistance of a cane. Four weeks after the definite surgery, the patient underwent radiation therapy for 2 months. One year postoperatively, spine radiographs showed that the surgical segment had healed fully, and spine MRI revealed complete decompression of the spinal cord with no evidence of local recurrence (Fig. ).
pmc-6320115-1	A 15-year-old boy sought care at the emergency department with a 1-month history of retrosternal chest pain with no obvious cause. The chest pain was accompanied by shortness of breath and was exaggerated during inspiration. The patient had no fever, cough, dysphagia, choking, dizziness, anorexia, fatigue, abdominal pain, or diarrhea. Physical examination was almost negative except for subcutaneous emphysema over the neck region. Thoracic high-resolution computed tomography (HRCT) of the patient (Fig. A, B) showed gas accumulation in the thoracocervical region and mediastinum. No abnormality was found in the trachea, bronchi, or lung parenchyma. Bedside arterial blood gas analysis showed mild metabolic acidosis with normal oxygen saturation at 99.6% on normal air (Fig. ). Routine blood analysis did not reveal any abnormality in complete blood count, liver and kidney function, coagulation status, cardiac enzymes, tumor markers (alpha-fetoprotein, CEA, CA125, Cyfra 21-1), lactate, or procalcitonin. Hepatitis, syphilis, and human immunodeficiency virus (HIV) screening tests were all negative. Esophagus iodine oil radiography, gastroscopy, and fiber bronchoscopy were performed to help identify the source of air in the mediastinum, but nothing positive was found. Repeated medical histories failed to reveal any cause of the patient's pneumomediastinum. All likely causes of pneumomediastinum were eliminated. On the third day of admission, the serum paraquat concentration was measured, despite the patient's denial of ingestion or contact. The serum paraquat concentration was found to be 467.40 ng/mL, and thus, occult paraquat poisoning was diagnosed. Standard treatment against paraquat poisoning was immediately initiated with the application of hemoperfusion, intravenous glucocorticoid (methylprednisolone 80 mg, 2×/day), and ulinastatin (100,000 IU, 3×/day). The serum paraquat concentration was carefully monitored daily and fell to nil on the sixth day after admission (Fig. ). The symptoms of chest pain and shortness of breath were significantly relieved. Thoracic HRCT on the eighth day after admission (Fig. C, D) showed that gas accumulation in the thoracocervical region and mediastinum was significantly absorbed. On the tenth day, the patient was discharged from the hospital. When followed up at 1 and 3 months after discharge, the patient experienced no more chest pain, and HRCT of the lungs was normal. His serum paraquat concentration remained nil and liver and kidney functions were stable and normal.
pmc-6320138-1	A 73-year old man presented with abdominal pain and vomiting for 1 week. He had a history of early satiety and weight loss of 5 kg in the previous month. His medical history included subtotal gastrectomy with Billroth II anastomosis for benign gastric ulcer perforation 40 years prior. Physical examination showed the following: blood pressure, 120/80 mm Hg; heart rate, 105 beats/minute; respiratory rate, 20 respirations/minute; temperature, 39.0°C. On physical examination of the abdomen, he had marked tenderness, particularly of the epigastric area. The results of laboratory test were as follows: white blood cell count, 10,870 /mm3; hemoglobin, 9.0 g/dL; platelet count, 247,000 /mm3; albumin, 3.3 g/dL; total/direct bilirubin, 2.06/1.38 mg/dL; alanine aminotransferase, 45 U/l; aspartate aminotransferase, 133 U/l; alkaline phosphatase, 172 U/I; CA 19-9, 165.95 U/Ml. Abdominal computed tomography showed prominent dilatation of the duodenal loop, common bile duct, and bilateral intrahepatic ducts, and a large amount of ascites (Fig. ). Based on these findings, the patient was diagnosed with afferent loop syndrome complicated by biliary tree dilatation; he was suspected to have ascending cholangitis. As a result of his poor general condition and the presence of ascites suggesting peritoneal seeding, surgical treatment was not an option. Therefore, a sonography-guided PTBD (percutaneous transhepatic biliary drainage) procedure was performed by inserting an 8.5 French, multiside hole pigtail catheter tip via the dilated left intrahepatic duct, with the catheter tip placed in the common bile duct, under fluoroscopy (Fig. ). For confirmation of the diagnosis and further management, esophagogastroduodenoscopy was performed. Endoscopy showed previous subtotal gastrectomy with Billroth II anastomosis, but the entrance of the afferent loop was not visible due to a fully obstructing mass at the anastomosis site of the remnant stomach (Fig. ). The patient was suspected to have remnant gastric cancer, and biopsies were performed. Pathological examination of the endoscopic biopsy showed poorly differentiated adenocarcinoma with a signet ring cell component. Three days later, cholangiography was performed via PTBD tube as an alternative for continuous opacification of the biliary tree, to guide the insertion of a 10.2 French, multiside hole pig tail catheter via the left intrahepatic duct, with its tip being advanced into the afferent loop (Fig. ). After PTBD reposition, he was able to tolerate a liquid diet without epigastric pain and discomfort. Amylase and lipase levels returned to the normal range. The patient's general condition and symptoms gradually improved during his hospitalization. Ten days after the PTBD reposition, a guide wire was inserted across the dilated loop and a catheter was then passed along the guide-wire to the gastric stricture caused by the tumor. A self-expanding metal stent (Taewoong Medical, Seoul, Korea), 12 mm in diameter and 80 mm in length, was successfully inserted through the stricture site (Fig. ). After stent insertion, follow-up esophagogastroduodenoscopy was performed. We confirmed the stent location at the stricture site that was previously completely obstructed due to remnant gastric cancer. The endoscopy scope was passed through the afferent loop and bile drainage was confirmed (Fig. ). After removing the PTBD tube, the patient was able to live comfortably without the need for a drainage catheter any longer. He died of disease progression 2 months later, without further recurrence of afferent loop syndrome.

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