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A 48-year-old Chinese man was admitted to the Emergency Department at the University Hospitals of Geneva with a sudden onset of severe epigastric pain and an episode of bilious vomiting that subsided completely within a few hours. He had no relevant medical history except hypercholesterolemia. He also denied any history of arterial hypertension, diabetes mellitus or any recent trauma. On physical examination, our patient was pale and sweaty with a temperature of 36.8°C, a regular heart rate of 84 beats per minute and a blood pressure of 130/70 mmHg. An examination of his abdomen revealed epigastric tenderness without signs of peritonism. His white blood cell count was 8.3 × 109 cells/L and his C-reactive protein was 3 mg/L. His liver function tests, serum lipase and amylase levels were normal. His cardiac troponins were 0.012 μg/L and his electrocardiogram result was normal.\nA contrast-enhanced computed tomography (CT) scan revealed an enlarged and irregular diameter of the SMA with a mural thrombus and without signs of bowel ischemia or ascites. A curved multi-planar reconstruction of the SMA showed a dissection of the proximal SMA with extension into the jejunal and ileal arteries (Figure ). Selective angiography of the SMA was performed using a 5F transfemoral Cobra catheter (Cordis, Roden, The Netherlands) which confirmed the diagnosis of dissection with compression of the proximal SMA (Figure ). Interestingly, an accessory right hepatic artery arose from its false lumen, while distal arterial branches of the SMA were patent.\nSince our patient was asymptomatic and did not show any abdominal complications, we considered percutaneous stent placement instead of surgery in order to treat the dissection and to prevent its progression. Oral informed consent was obtained from our patient before the procedure. Using the same transfemoral route, an 8F 55 cm RDC guiding catheter (Cordis, Roden, The Netherlands) was introduced into his abdominal aorta. A 0.035-inch guide wire (Terumo, Leuven, Belgium) was eased into the true lumen of his SMA through the stenosis after an intra-arterial infusion of 5000 IU of heparin. After measuring the diameter of the SMA using a selective arteriogram, a self-expandable metallic endoprosthesis (Wallstent; Boston Scientific, Galway, Ireland), 10 mm in diameter and 20 mm in length was placed over the entrance of the false lumen and the obstructing intimal flap (Figure ). A control angiogram showed a patent true lumen with good flow in all the branches of the SMA, including the accessory right hepatic artery.\nAfter the procedure, our patient commenced treatment with long-term 100 mg aspirin, which was combined with an oral loading dose of 300 mg clopidogrel, followed by 75 mg clopidogrel daily for 28 days. A Doppler ultrasonography was performed at 24 hours then seven days after the stent placement. This showed patency of the endoprosthesis with normal spectral Doppler waveforms in the main distal branches of the SMA. Our patient was discharged eight days after the procedure and has remained completely asymptomatic during the following three months. |
A 76-year-old male with a past medical history of hypertension, hypercholesterolemia, hypothyroidism, chronic obstructive pulmonary disease, diabetes mellitus type two, obstructive sleep apnea, benign prostatic hyperplasia, cerebral vascular accident with residual right-sided weakness, and dextrocardia with situs inversus presented with an abnormal nuclear stress test. Additionally, he had a family history notable for colon cancer and myocardial infarction in his mother and a thrombotic event in his father. He had no history of significant alcohol use, tobacco use, or illicit drug use, and his past surgical interventions were not cardiovascular in nature. He originally presented at an outside hospital following a syncopal event with a 45-second loss of consciousness postmicturition. At the time, he was thought to have orthostatic hypotension, and his tamsulosin was discontinued. He also underwent a nuclear stress test for the syncopal event due to additional complaints of worsening dyspnea on exertion. Nuclear stress testing demonstrated a moderate-sized reversible anterior wall myocardial perfusion defect and a moderate-sized reversible inferolateral wall myocardial perfusion defect. He was transferred to our hospital for further evaluation of symptoms and possible left heart catheterization. Cardiac catheterization revealed the already diagnosed dextrocardia along with a new diagnosis of severe ostial left anterior descending (LAD), mid-LAD, and ostial diagonal coronary artery disease. Transthoracic echocardiography (TTE) showed trace mitral valve regurgitation, mild aortic valve regurgitation, and an ejection fraction of 60%. His underlying dextrocardia with situs inversus caused diagnostic challenge with these tests as his angiographic, radiologic, and echocardiographic images were mirror images of normal cardiac anatomy. Aggressive risk factor modification was initiated, and a consult was placed to cardiothoracic surgery for evaluation for potential coronary artery bypass grafting (CABG). He underwent three-vessel CABG on day 7 of his hospitalization with alterations in management to account for his known dextrocardia with situs inversus. The electrocardiography leads and defibrillator pads were placed in reverse orientation. A left internal jugular vein central venous catheter was placed to provide direct access to the right atrium rather than standard right-sided central venous access. The arterial line was placed in the right radial artery as opposed to the institutional standard left radial artery to accommodate for the position of the surgeon during the operation. Transesophageal echocardiography (TEE) evaluation again confirmed dextrocardia (). To obtain standardly recognized TEE images, the omniplane was adjusted to 180° for the midesophageal four-chamber view. By subtracting 60°, 90°, and 120°, midesophageal midcommissural, two-chamber, and long-axis views were then obtained. Additionally, TEE confirmation of aortic and venous cannulation required turning the probe right for the right-sided aorta and left for liver visualization, respectively. Aside from these alterations in approach, induction, intubation, and anesthetic management were conducted in normal fashion and were uneventful. He remained intubated and was taken to a cardiac intensive care unit postoperatively. A thorough sign-out was given to the intensive care unit staff to ensure understanding of his anatomy and intraoperative course. He was weaned from the ventilator and extubated on postoperative day 1 and transferred to the step-down unit on postoperative day 2. His postoperative course was complicated by urinary retention and an elevation in creatinine that trended down prior to discharge. He was discharged home in stable condition with a urinary catheter in place on postoperative day 6 that was to be removed as an outpatient. Both the patient and the responsible physicians were satisfied with the successful outcome. |
A previously healthy, 52-year-old Caucasian man presented to his family physician a week after having a tonic-clonic seizure. A magnetic resonance imaging (MRI) scan showed a 10 cm left frontal tumor, which was confirmed as an atypical meningioma following craniotomy and resection (Figure ).\nPostoperatively, he took 400 mg of phenytoin PO once a day. He had no seizures postoperatively or afterward. The patient uneventfully received 60 Gy of adjuvant radiation therapy to the postoperative bed in 30 fractions. Three months after the resection of the tumor, the patient began a trial of phenytoin but nine days later, he developed symptoms consistent with a generalized seizure. He resumed his daily phenytoin prophylaxis with good effect.\nTwo months later, he complained of blood in the stool and after an evaluation was diagnosed with a locally advanced nonmetastatic adenocarcinoma of the low rectum (Figure ). A curative-intent dose of 50.4 Gy in 28 fractions of neoadjuvant radiation therapy was prescribed, with 2000 mg PO BID of concurrent radiosensitizing capecitabine []. After 20 of the planned 28 fractions, he began to feel unwell and experienced new, right-sided upper and lower limb dysfunction and an unsteady gait. A contrast-enhanced computed tomography (CT) scan of the brain showed no suspicious findings but his phenytoin level was dramatically elevated at 138 µmol/L, compared to 49 µmol/L just prior to neoadjuvant therapy (normal range: 40-80 µmol/L). His albumin level from a few weeks prior to these symptoms had also been normal at 39 g/L (normal range: 34-46 g/L), and he was taking no other medications other than an occasional stimulant laxative. Capecitabine was discontinued, and the patient was treated with charcoal and admitted for observation. Phenytoin was temporarily discontinued and then reintroduced at the previous dose of 400 mg PO per day once levels began to normalize. His symptoms quickly resolved and he showed no further toxicity.\nHe resumed radiation therapy a few days later without concurrent capecitabine. It was believed that he had developed phenytoin toxicity secondary to impaired clearance as a result of his capecitabine. His phenytoin levels were monitored during the following weeks and his phenytoin dose was bridged with lacosamide and titrated down gradually and then discontinued, with no further symptoms of toxicity. The patient remained on 200 mg PO per day of lacosamide. He underwent a surgical resection with clear margins followed by adjuvant capecitabine and showed no signs of a recurrence of rectal adenocarcinoma thereafter. Three years later, the patient passed away from recurrent meningioma. |
A 68-year-old female presented with a history of painless haematuria associated with passage of clots for one month. She was catheterised following an episode of acute urinary retention 3 weeks back. She had a history of diabetes and hypertension. There was no abdominal mass on palpation. The haemoglobin level was 11.2g/dL. Other haematological and biochemical parameters were within the normal limits. Urine cytology had negative results regarding malignant cells. Spiral computed tomography scan of abdomen and pelvis with urogram revealed a large heterogeneously enhancing infiltrative lesion with specks of calcification in the pelvicalyceal system of the left kidney infiltrating into the left renal cortex and upper half of the ureter with hydronephrosis and hydroureter ().\nLeft nephroureterectomy was performed with a preoperative diagnosis of transitional cell carcinoma.\nGross pathology; resected nephrectomy specimen measuring 12 cm × 5 cm × 4 cm with attached ureter measuring 10 cm. External surface of the kidney was covered with perinephric pad of fat and appeared smooth and nonadherent. Cut surface of the kidney showed a polypoidal whitish solid homogeneous growth in the pelvic region measuring 10 cm × 4.5 cm × 3 cm extending to the renal parenchyma. The corticomedullary differentiation was retained in the adjacent renal parenchyma. The tumor also involved the ureter with marked dilatation, irregular nodularities on the surface of upper ureter, wall thickening and intraluminal extension ().\nThe resected end of the ureter, renal capsule and vessels were free of tumor.\nMicroscopy showed a neoplasm composed predominantly of sheets and poorly formed fascicles of pleomorphic spindle cells with vesicular nuclei and nucleoli.\nExtensive foci of osteosarcomatous differentiation was noted with abundant osteoid matrix production and areas of mineralised osteoid ().\n6-8 mitotic figures/high power field with atypical mitosis, tumour giant cells, foci of necrosis, calcification and hyalinisation were also noted. The tumour involved renal parenchyma and ureter with osteosarcomatous differentiation at both sites. The focal epithelial component was formed by islands of urothelial carcinoma with carcinoma in situ in the urothelium of renal pelvis (, ).\nImmunohistochemistry was performed and epithelial elements were found to have strongly positive results for cytokeratin and negative for vimentin. The sarcomatous component was found to have positive results for vimentin (, ).\nA pathological diagnosis of sarcomatoid carcinoma of renal pelvis with osteosarcomatous differentiation with involvement of the ureter and renal parenchyma was given. The resected end of the ureter, renal capsule and vessels were free of tumor. The postoperative period was uneventful and the patient was discharged 8 days after the operation. Ten months later the patient was receiving regular clinical follow-up and there was no evidence of local or metastatic disease. |
A 53-year-old male presented to the ED with his wife and followed shortly by their daughter for evaluation of dyspnea which began approximately six hours prior to arrival. He had an associated nonproductive cough which had been worsening over that time period. He reported a subjective fever, chills, and myalgias. He had taken Robitussin and Aleve prior to arrival without relief. He stated that the symptoms began 1-2 hours following the cleaning of an exotic coral which he identified as a species of Zoantharia from his home aquarium. He had cleaned the coral using hot tap water in his basement sink. He identified the coral as a Zoantharia species of coral. He had not previously cleaned that particular coral and did not use any protective equipment while cleaning it. Both his wife and daughter were present in the home while he was cleaning the coral. His wife was in an adjoining basement room and his daughter was upstairs on the first floor of the home. Both presented to the ED with similar but less severe symptoms with the degree of severity commensurate with the distance from the location where the coral was being cleaned.\nThe patient's past medical history was significant only for hypothyroidism, hyperlipidemia, and psoriasis for which he was taking levothyroxine, rosuvastatin, and glucosamine chondroitin. Social history was significant for prior tobacco use, with a 20 pack-year history, with cessation one year prior to this encounter.\nVital signs included tachycardia at a rate of 112, a blood pressure of 155/83, respiratory rate of 18 with an oxygen saturation of 96% at room air, and a temperature of 102.6°F taken orally. On physical examination, bilateral expiratory wheezes with normal chest excursion were noted, with normal chest excursion. The patient had no evidence of respiratory distress. Cardiac examination revealed a regular tachycardia without murmurs, rubs, or gallops. The patient's wife's and daughter's examination revealed similar findings.\nAcetaminophen was given for the fever with improvement of temperature to 100.8°F. Nebulized albuterol was given for the dyspnea and wheezing, with minimal improvement of symptoms. A portable chest X-ray was obtained and was normal. An EKG showed sinus tachycardia. Lab findings were significant for leukocytosis of 14,000, and an influenza swab was negative for influenza A and influenza B, and cardiac troponin I and brain natriuretic peptide (BNP) were normal.\nThe patient's condition continued to deteriorate requiring increasing supplemental oxygen to maintain his saturation above 90%. He received repeated doses of nebulized albuterol and oral acetaminophen with no change in condition. An arterial blood gas (ABG) was obtained while the patient was receiving four liters of oxygen by nasal cannula that was significant for a partial oxygen pressure (pO2) of 65 mmHg. The New York City Poison Control Center was consulted and reported that certain toxic coral species have been found to cause pulmonary edema in cases of inhalational exposure when being handled out of water. As a result of the worsening clinical manifestations the patient was admitted to the intensive care unit with a diagnosis of respiratory distress from inhalational palytoxin exposure. During the course of the hospitalization the patient experienced a worsening cough, increased generalized weakness, and malaise. On the second inpatient day he developed hemoptysis. His pO2 on ABG decreased further to 76 mmHg while on 85% FiO2 via face mask. Serial chest X-rays demonstrated worsening bibasilar opacities (). Lab work was significant for continued leukocytosis peaking at 22,000 on the third inpatient day. An echocardiogram was performed with no significant cardiac dysfunction identified. By day 4, the patient began to demonstrate mild clinical improvement with decreasing hemoptysis and improvement of generalized weakness. He continued to require supplemental oxygen by face mask throughout his hospitalization, with O2 saturations dropping to the mid-80s with trials at room air.\nAfter seven days of inpatient treatment the patient was discharged home with the diagnosis of acute toxic lung injury. At the time of discharge he required portable oxygen. He was also discharged with an albuterol metered dose inhaler and prednisone taper. The patient's wife and daughter were also hospitalized at the initial ED encounter but clinically improved after 24 hours and were discharged home in stable condition after symptomatic treatment and observation. The patient continued to require portable O2 by nasal cannula and nebulized albuterol for 1 month following discharge, following which he made a complete recovery. |
A 69-year-old man developed a sudden epigastric pain. He was presented at this hospital as an emergency outpatient. Six years earlier, he underwent laryngoesophagopharyngectomy, bilateral lymph node dissection for hypopharyngeal cancer, and esophageal reconstruction with a free jejunum flap. On physical examination, the abdomen was flat and soft with tenderness in the epigastric region, but no sign of peritoneal irritation. Blood biochemistry findings revealed elevated values: creatinine, 1.16 mg/dl; lactate dehydrogenase, 364 U/l; and creatine phosphokinase, 622 U/l.\nAbdominal contrast computed tomography (CT) revealed twisted mesentery with the small intestine around the point of torsion (whirl sign) and the superior mesenteric artery as the axis. Contrast enhancement was weakened in the same area of the small bowel (Fig. ). Given this information, we suspected small bowel volvulus and performed emergency surgery on the same day.\nA 5-mm camera port was placed in the umbilicus and 5-mm ports in the lower and right lower abdomen. During laparoscopic examination, the upper jejunum adhered to the small bowel close to the terminal ileum with overlapping of the small bowel. The entire part from the upper jejunum to the terminal ileum was twisted clockwise with the superior mesenteric artery and vein as the axes and the adhesion site as the starting point. There were areas of poor color enhancement throughout the twisted section of the small bowel (Fig. ). We laparoscopically separated the adhesion between different sections of the intestinal tract and traced the bowel from the small bowel in the region of the ligament of Treitz toward the anus to confirm the absence of adhesions or torsion up to the terminal ileum. The color of the small bowel improved; hence, the surgery was completed without resecting any part of the intestine.\nPostoperatively, the patient made good postoperative recovery, resumed oral intake on day 2, and was discharged on day 5 after surgery. No recurrence has been reported 1 year postoperatively. |
An 83-year-old female with a past medical history of rheumatoid arthritis (on DMARD's), asthma, depression, gastroesophageal reflux disease (GERD), and lumbar spondylosis, as well as a past surgical history of right posterior total hip arthroplasty (1999), bilateral total knee arthroplasties (2003, 2012), and right shoulder hemiarthroplasty (2010), presented with five days of right hip pain and inability to ambulate after bending down. In the emergency department, initial radiographs revealed a right posterior hip dislocation, as well as chronic appearing fractures of the right greater trochanter and left inferior public rami (). Her right lower extremity was shortened, internally rotated, and adducted. A propofol-induced conscious sedation was performed by the emergency physician and closed reduction was attempted by an experienced orthopaedic resident. The reduction maneuver involved hip flexion, traction, adduction, and internal rotation followed by external rotation and abduction. After three attempts, post reduction radiographs were significant for a right inferior obturator hip dislocation (). The patient tolerated the procedure and was neurovascularly intact distal to her hip. Computed tomography (CT) was performed, which confirmed a persistently dislocated femoral head with intrapelvic migration through the right obturator foramen (Figures and ). Having failed three attempts at closed reduction, the patient was taken to the operating room for open reduction and revision arthroplasty.\nUsing a posterolateral approach, the femoral head was found to be locked inferior and posterior to the acetabulum. Manual traction was utilized to successfully extricate the femoral component from within the obturator ring. Both the femoral and acetabular components were stable; however, a large amount of posterior wear was noted on the liner, which was exchanged for a constrained component. A greater trochanteric hook plate with cerclage cables was then utilized for the fixation of the greater trochanteric fragment (). Excellent stability with a full range of motion was noted.\nPostoperatively, the patient was weight bearing as tolerated, with standard posterior hip precautions including an abduction pillow. Aspirin 325 mg BID was used for deep vein thrombosis (DVT) prophylaxis. Although the patient initially did very well, she developed urosepsis six months after the index procedure, leading to an acute right periprosthetic septic hip with Proteus mirabilis. Radiographs showed greater trochanteric escape from the hook plate (). She then underwent irrigation and debridement with greater trochanter excision and hook plate removal (). The patient was discharged with 6 weeks of ceftriaxone antibiotics via a peripherally inserted central catheter and has since been doing well with no further dislocations. |
A 27-year-old Emirati male presented to Cleveland Clinic Abu Dhabi (CCAD) emergency department (ED) for the first time in Sept 2015 complaining of severe abdominal pain. The pain has been episodic for the last 4 years and had significantly affected his work and family life. He was seen and admitted to multiple hospitals across Abu Dhabi, including our own, attended different specialists, and underwent a wide range of investigations including blood tests (CBC and differential count, liver and renal profiles and CRP), gastroscopies, colonoscopies, and CT scans and a laparoscopy. The results of all his clinical assessments and investigations did not show any sign.\nOn one of his acute admissions to CCAD, the immunologist was asked to review the patient. Detailed examination of the patient's medical history starting from the onset of symptoms reveled that he used to get 2 swelling episodes every week affecting his face, hands, feet or scrotum and severe abdominal pain twice a week. These swelling episodes and abdominal pain appeared suddenly without any obvious triggering factor, developed over 36 hours and resolved spontaneously in 5-7 days without any medication including analgesics. He reported no laryngeal swellings or respiratory compromise. He denied any fevers, night sweats, weight loss, change in bowel habits or blood in his stools. His swelling episodes were occasionally associated with non-pruritic red skin rash that was mistaken for chronic urticaria for which he was treated with Omalizumab (300mg every 4 weeks) for 9 months without any benefit. Moreover, his response to different types of analgesia, high dose antihistamines, antibiotics and corticosteroids was unsatisfactory. He is a thalassemia carrier; otherwise he is fit and healthy and has no past medical history of note. He has no family history of immunodeficiency, inflammatory bowel disease, autoimmunity or FMF. On examination, he was in pain and his abdomen was soft, tender with guarding and decreased bowel sounds. There was no rebound, rigidity, distension or ascites. He had no peripheral swellings.\nDuring his acute admission to CCAD, a review of his blood tests was performed that showed reduced C4 and absent C1 inhibitor function (Table). These tests were performed a month prior to his admission but were not followed up. His abdominal CT scan showed diffuse swelling and long segment of enhancing mucosal thickening involving the proximal jejunum and gastric mucosa with minimal free abdominal fluid (). In addition, he had a colonoscopy which showed severe mucosal edema in the transverse colon with occlusion of the lumen (). A provisional diagnosis of HAE was made based on his limited complement studies. As he had severe abdominal pain for 24 hours prior to his hospital admission, C1 inhibitor concentrate (2000 units IV over 10 minutes) was administered and within 2 hours his pain had almost resolved.\nAfter his recovery, he underwent detailed immunological investigations that revealed markedly reduced C4 level and absent C1 inhibitor function (performed manually, read on Shimadzu UV-1700 equipment) with normal C3 and C1q levels and raised C1 inhibitor serum levels (). His ENA, total immunoglobulin, CBC and differential count, serum protein electrophoresis, liver function tests, hepatitis serology, lipase, amylase, tissue transglutaminase, stools tests and urinalysis did not show any significant abnormality.\nHe was diagnosed with type 2 HAE based on his abnormal complement studies () and was commenced on tranexamic acid for 3 months. He did not want to start with attenuated androgens because he was concerned about their adverse effects. However, he continued to get abdominal pain even when the dose of tranexamic acid was increased to 3 grams daily. He was then switched to danazol 100 mg twice daily with complete resolution of his abdominal symptoms. |
The patient is a 19-year-old man, a seasonal agricultural worker daily in contact with sheep, living in Burgundy and with no history of travel neither abroad nor in the south of France during the previous months. At the end of September 2016, the patient performed farm work in contact with sheep when he had an ocular traumatism caused by a fly. Three hours after the ocular traumatism, the patient complained of a painful right eye discomfort, with sensation of moving foreign. Upon arrival at the department of ophthalmic emergency of the University Hospital Center of Dijon within hours of the onset of the first symptoms, the clinical examination showed a red and irritated conjunctiva in the right eye with the observation of mobile and translucent larvae in the conjunctival fornix. The rest of the ophthalmologic examination was normal. Eight larvae were extracted using Bonn hook forceps under local anesthesia. All larvae were sent to the Parasitology-Mycology Laboratory of the University Hospital Center of Dijon for identification.\nThe parasitological diagnosis allowed the identification of stage 1 Oestrus ovis larvae (L1). Indeed, the macroscopic examination revealed larvae of white color and about 1 mm length. Microscopically, these larvae were composed of eleven metameres, each of these displaying 4 rows of spines (Fig. a). The cephalic segment had two large black buccal hooks (Fig. b), while the posterior segment consisted of two tubercles, each containing about ten curved spines (Fig. c) which is concordant with the morphological description of L1 Oestrus ovis larvae in the literature [].\nAt the first visit, the patient received a local treatment based on the administration of oxybuprocaine and antiseptics (Biocidan®) as eye drops. The curative treatment consisted of the mechanical removal of all of the eight larvae present at the level of the conjunctiva using a forceps, as mentioned before. Subsequently, the treatment was supplemented by the administration of antiseptic eye drops (i.e. desomedine) and antibiotics (i.e. ofloxacin). Removal of the larvae resulted in rapid relief and no complication was further reported. |
A 40-year-old female patient reported to the dental OPD with chief complaint of pain in the lower right and left side of jaw since 2 months. Patient reported that she noticed multiple ulcerations in the attached and marginal gingiva on the right and left mandibular region since 14 months. Initially, ulcers healed by themselves, but with time the exacerbation of ulcers increased and presently the ulcerations did not heal for months. History of pain with the similar kind of ulcers was evident which was dull, aching, nonradiating in nature with no aggravating or relieving factors. Noted a history of bleeding while brushing. No history of any sensory loss, exfoliation of tooth, intraoral burn, or pus discharge from the affected areas. Medical history revealed that the patient was HIV positive and is under antiretroviral treatment (ART) for the last 10 years since 2009. Presently, the CD4 count for the patient was 250. History of fatty liver associated with splenomegaly since 2 years and was under treatment. Patient was married and her husband was also HIV positive patient since 2001 before marriage and was under HAART therapy. Patient had 2 sons who were not affected. No history of any smoking or smokeless tobacco. On general examination, patient was well oriented to time place and person. Temperature was afebrile, pulse 78/min, blood pressure 110/85 mmHg, and respiratory rate 15/min. Built was lean and gait was normal. On extraoral examination, no gross facial asymmetry present. Noted hyperpigmentation on the left malar region []. Swelling present on the lower right chin region, on inspection size 2 cm × 1 cm approximately, shape roughly oval, surface covered with crustrations, reddish in color, margins well defined. On palpation, the inspectory findings were confirmed. Consistency was soft, tenderness was present, and no bleeding or pus discharge on manipulation; similar swelling of smaller size was present 1 cm anterior to it []. Lips were competent and TMJ movements bilaterally were smooth and synchronous. A single right submandibular lymph node was palpable of size 1 cm × 0.5 cm approximately, shape roughly oval, firm in nature, fixed to the underlying structure, and tender on palpation. On intraoral examination, there were multiple ulcerations present with the lower anterior region and right side of jaw extending anteroposteriorly from 35 to 46 region. Consistency was soft to firm in nature; surface was covered with yellowish slough surrounded by erythematous halo. Ulcerations were seen both buccal [] and lingual side [] of the alveolus in the same region. Similar ulceration was seen in the maxillary jaw extending anteroposteriorly from 21 to 24 region on the lingual side, and erythema with a bluish hue with swelling was seen on the buccal side of maxillar []. Gingiva was normal with no bleeding on probing and no clinical pockets. With the clinical examination, the provisional diagnosis was KS affecting the maxillary and mandibular jaw.\nPatient was advised to get the CD4 count and routine blood report. The report revealed CD4 count was 220 cells/mm3, hemoglobin was 10.2 g%, and total white blood cell count was 8400/mm3. The other blood investigations were within normal range.\nHistological examination from the ulcerative area from the right side of gingiva revealed angiomatoid slit like vascular spaces containing red blood cells surrounded by spindle cells []. The spindle cells were arranged in fascicles and their nuclei did not show any atypical features or mitotic activity. In between tumor cells, deposition of hemosiderin pigment and infiltration by mononuclear cells were identified which were suggestive of KS.\nThe treatment advised was oral prophylaxis and antiretroviral therapy (lamivudine 150 mg BD, stavudine 30 mg BD and efavirenz 600 mg HS) with chemotherapy. There was no improvement in patient's condition and she died within 9 months |
A 58-year-old woman without a history of chronic headache suddenly experienced back pain during stretching exercises. Shortly afterwards, she began to complain of a headache which was aggravated by standing up and relieved by lying down. A local physician thought that she had a migraine and prescribed triptans. However, her headache became constant regardless of her posture, and within a month after the initial back pain, she became stuporous and was brought to a local emergency department. She was E2V3M5 on the Glasgow Coma Scale, and mild left-sided hemiparesis was noted on neurologic examination. Her pupils were reactive and isocoric. A brain CT revealed a right chronic subdural hematoma with a marked right-to-left midline shift (fig. ). A neurosurgeon at that hospital thought that she was suffering from an impending transtentorial herniation and performed a small craniotomy to evacuate the hematoma. No brain swelling was observed after dural opening and hematoma evacuation, and no chronological improvement in the degree of the midline shift was seen on her postoperative CT scan (fig. ). After a transient improvement, her consciousness level further deteriorated to E1V1M3 4 days after surgery. She was then referred to our institution; a third CT scan obtained immediately before her transfer showed a worsening of the midline shift and an increase in her subdural hematoma (fig. ).\nA lack of neurologic and radiographic improvement after hematoma evacuation pointed to the presence of underlying intracranial hypotension, and subsequently, an MRI with gadolinium was performed. Diffuse pachymeningeal enhancement on the axial T1-weighted image (fig. ) together with the downward displacement of the cerebellar tonsil on the sagittal T2-weighted image (fig. ), verified the diagnosis of SIH. The brainstem was severely squeezed between the retro-odontoid mass and the posterior edge of the foramen magnum (fig. ). An MRI of the cervical spine demonstrated a mild fluid accumulation in the posterior cervical epidural space, which had been considered a nonspecific finding. A lumbar puncture was performed mainly for the purpose of infusing saline intrathecally, which has a therapeutic potential to temporarily ameliorate neurologic deficits []. The CSF pressure was 2 cm H2O, and her consciousness level improved to E2V1M6 after the intrathecal saline infusion. A CT myelography revealed a massive CSF leakage over the entire thoracic epidural space (fig. ). A blood patch was urgently performed to seal the CSF leakage: 20 ml autologous blood was administered at the T3–4 and T9–10 levels (40 ml of blood volume as a total) via an 18-gauge Tuohy needle. The postprocedural course was uneventful and she became fully conscious, with a complete resolution of the left-sided hemiparesis 2 days after blood patch therapy. A repeat CT myelography obtained 14 days after the blood patch therapy revealed no CSF leakage in the thoracic epidural space (fig. ), and she was discharged free of symptoms 20 days after the admission to our institution. A brain MRI obtained 30 days after the blood patch therapy revealed the disappearance of contrast enhancement, subdural hematoma (fig. ) and the return of the brainstem to the normal position (fig. ). She has not sustained a recurrence of SIH for more than 6 months. A written permission for this publication was granted by the patient. |
A female patient who was 18-year-old and underwent a nasal reconstruction for congenitally missing nostril, lobule-columella, and the alar complex of the nose reported to the department of Prosthodontics, government dental college and hospital for a nasal prosthesis. Patient was treated at ENT hospital for nasal reconstruction with forehead flap and was referred to the department of prosthodontics after 2 days for immediate nasal stents . Nasal stents were required for the patient to prevent adhesions and to prevent collapsing of nostrils, to allow proper breathing. Upon through clinical examination, it was seen that the surgical site was in healing phase and making of impression was difficult to construct customized stents.\nHence, nasal stents were fabricated by taking age into consideration, as age is one of the important criteria which determines nasal anatomy to an extent and impressions of same-aged female patients nostril were made with impression compound and heat processed solid acrylic stents were fabricated which were hallowed out to allow air passage and highly polished and inserted to the patient and instructed to wear continuously, removing it only for short period of time for removal and maintenance.\nNasal cavities of donor nostrils is lubricated with petroleum jelly before making an impression, impression was made with modeling plastic impression compound extending up to inferior nasal concha. Plastic impression compound which is rigid, compliant, and did not dangle due to gravity and it can be re-used if impression can’t be made in the single shot was chosen as impression material. However, the modeling plastic impression compound had to be meticulously inserted, taking care not to damage the nasal mucosa.\nThe compound was softened, and added onto handle made up by bunch of 3 matchsticks joined with cyanoacrylate glue, impression compound molded into cylindrical core of approximate length was added onto the prepared handle, and inserted into each nasal vestibule of donor-patient , after it is set impression was retrieved and checked for accuracy. Impressions were flasked using split mould technique in dental flask, kept in hot water for 5 min at 80° to soften the compound later flask was opened and compound impressions were retrieved and discarded and mold was created into which heat cured clear acrylic in dough stage was packed and acrylzed in conventional processing manner, stents were retrieved, hallowed out, and highly polished and inserted into the patient nostrils .\nThe external prosthesis junction was established at mucocutaneous junction from cosmetic standpoint and the prosthesis was self-retentive as it was slightly wider than the reconstructed nostrils of the patient and patient was allowed to practice removal and insertion before dispersing the patient from the department. |
In a 19-year-old, mentally disabled male, chest radiography was done because of a sudden episode of cough. Metallic, hook-shaped foreign bodies were identified in both the main bronchi.\nThe right-sided FB was removed by fiber-optic bronchoscopy in the regional hospital, whilst the left-sided extraction failed with the left-sided FB persisting in the left main bronchus ().\nUpon urgent admission in a tertiary institution, extraction was attempted by rigid bronchoscope under general anesthesia. Bronchoscopic extraction failed, associated with some bleeding and subcutaneous emphysema immediately after the intervention. The increasing mediastinal and subcutaneous emphysema raised suspicion about an iatrogenic airway lesion, so surgery was indicated. Esophageal injury was previously ruled out by esophagoscopy, revealing many metallic FBs in the stomach. At thoracotomy, a significant mediastinal emphysema (), together with diffuse adhesions, was noticed. After the lung liberation, a proximal 0.5 cm of the noncurved part of the metallic hook was found to protrude through the perforated membranous wall of the left main bronchus, 1 cm away from the descending aorta (, arrow). The part of the FB protruding outside the bronchus was grasped by the clamp and, by following the curved shape of the FB, gentle maneuvers were applied by pulling the sharp end (hook) of the FB in the direction outside the bronchus. The FB was removed from the bronchus (insert on ) without the need for additional bronchotomy. The remaining 10 × 1 mm defect in the bronchial wall, caused both by manipulations during a bronchoscopic extraction attempt and subsequent surgical extraction, was sutured by interrupted PDS 3-0 stitches, and the lung fully inflated. No air leaks appeared during the water test. Having in mind the dimensions of the defect and tensionless suture, no suture-line protection was performed.\nAfter the thoracotomy closure, laparotomy was done and several sharp metallic pieces of different shapes were removed from the stomach (Figures and ). This was followed by an uneventful postoperative course and the discharge after 14 days.\nAfter three years, the patient was urgently readmitted for the new episode of the metallic foreign body aspiration (Figures and ). The abdominal radiography revealed metallic pieces in the digestive tract as well (). With the surgical team on site, rigid bronchoscopy under general anesthesia was done. As the tip of the FB was not impacted in the mucosa, it was possible to grasp it with the rigid biopsy forceps and to withdraw it up to the tip of the bronchoscope. Because of the curved shape of the FB and the impossibility to remove it through the instrument, the FB and bronchoscope were pulled out from the patient together, with the FB firmly grasped, followed by reintubation with the same bronchoscope (Figures and ). After a careful check-up for bleeding and mucosal damage, the patient was extubated.\nMetallic pieces left the digestive tract spontaneously after a couple of days. |
A 7-year-old female experienced abdominal pain without an obvious cause in May 2011, showing continuous dull pains, most of which could be self-relieved. Subsequently, the patient underwent imaging examination at our hospital, which indicated multiple intrahepatic and extrahepatic cystic dilatation of bile duct (Figure ). It was clearly diagnosed as type IVa CCs. After the preoperative examinations, we decided to carry out robotic-assisted laparoscopic choledochocystectomy and biliary-intestinal anastomosis.\nWith the patient in the supine position, the operation bed was in the reverse Trendelenburg position. After the anesthesia was successful, the anesthesiologists conducted internal jugular vein intubation and radial artery catheterization and placed a gastric tube and urinary catheter. Direct trocar puncture was done on the abdominal wall between the umbilicus and pubic symphysis to establish pneumoperitoneum. The pressure was 12 cmH2O, and a 12-mm trocar and robotic lens were inserted.\nBeing monitored under the lens, at about the level of umbilicus, the 8-mm-diameter trocars of arm 1 and arm 2 of the robotic instrument were inserted into the left and right abdomen, respectively, and the arm 3 trocar was inserted into the right upper abdomen at the level of the axillary line (Figure ). A 12-mm auxiliary hole was created between arm 1 and the lens. The robotic arms were placed from the end of the head and were connected.\nNo significant ascites was detected in the abdominal cavity. The gallbladder volume was approximately 7.0 cm × 3.0 cm. The common bile duct was dilated with a diameter of 5.5 cm. Its surface showed hyperemia and edema. An electric hook was used to separate the adhesion between the gallbladder and liver. The gallbladder arteries were ligated, and the gallbladder neck was dissected all the way to the common hepatic duct. The adhesions between the outer serosal membrane of the choledochal cyst and the duodenum and the portal vein were separated. The common hepatic duct was cut transversely. The diameter of the common hepatic duct was approximately 3.2 cm. The left and right hepatic duct openings were visible at the proximal end, and the flow of bile was unobstructed. There was no obvious stenosis. The gallbladder and the choledochal cyst to be removed were turned upside down, the common bile duct was dissected till near the head of the pancreas, and the end of the cyst was significantly narrowed with a diameter of approximately 1 cm and was sutured twice.\nA stapler (EC-60) was used to cut the jejunum transversely at 15 cm from the duodenojejunal flexure, and 35 cm of the proximal end towards the hilus was saved as ascending arm. After joining the jejunum and the ascending branch by three stitches, we used a stapler to perform end-to-side anastomosis between the ascending arm and the proximal jejunum. The 3-0 absorbable suture was used for full-thickness interrupted suture of the intestinal wall, which was reinforced by interrupted suture. The ascending arm of the distal jejunum was lifted from the front of the colon to the common hepatic duct location to perform anastomosis. A 4-0 absorbable suture was used for full-thickness continuous suture of the posterior wall. Then the anterior wall was sutured, and interrupted suturing was done for seromuscular layer for reinforcement.\nPhysiological saline was used for rinsing. One abdominal drainage tube was placed on the lateral posterior side of the gallbladder-intestine anastomosis, which came out of the body through the puncture hole of the arm 2 trocar and was connected to a negative-pressure drainage ball. The abdominal cavity was confirmed to have no active bleeding, and the devices and gauzes were all counted. No. 3 absorbable suture was used to suture the muscular layer at each trocar location, 4-0 absorbable suture was used for interrupted suturing of subcutaneous tissue. Biological fibrin glue was used to bond the edges of the skin, and the drainage tube was properly fixed. Tissue aid was applied to the wound.\nThe surgery went smoothly. The operation time was 420 min, of which the robot operation time was 370 min. The bleeding was approximately 100 mL. There was no perioperative blood transfusion. Two days after the operation, the gastric tube was removed, and fluid and semi-fluid food were given gradually. Two antibiotics were administered for 3 d. Seven days after operation the abdominal drainage tube was removed. The patient was discharged 9 d after the operation. No serious complications occurred. Routine pathological examination further confirmed the diagnosis of cystic dilatation of the common bile duct. |
A 32 year-old woman with a history of hypothyroidism and pre-eclampsia initially presented to an outside hospital with acute onset dense left hemiplegia, right gaze preference, and left-sided neglect. Her initial National Institute of Health Stroke Scale (NIHSS) was 14 and she had an admission Glasgow Coma Scale (GCS) of 10. A computed tomography (CT) angiogram of her neck revealed complete occlusion of the right cervical internal carotid artery (ICA). She was outside the time window for intravenous thrombolysis; however, she underwent mechanical thrombectomy using a stent retreiver device and aspiration (Penumbra System®, Alameda, CA). Immediately after the procedure, there was a successful restoration of the blood flow to the distal ICA, proximal middle cerebral artery (MCA), and to the anterior cerebral artery (ACA), with residual distal M2 occlusion. She was intubated for the procedure and was extubated in the following days. Her left-sided weakness persisted and a repeat CTA showed re-occlusion of the right cervical ICA. No further intervention was done and she was treated with aspirin and statin for secondary stroke prophylaxis. The stroke was deemed cryptogenic after work-up for a potential source was negative including an echocardiogram which demonstrated a normal ejection fraction, normal left atrial size, and negative bubble study. A workup for prothrombotic and hypercoagulable states were negative as well. Magnetic resonance imaging (MRI) of the brain was done which showed a large area of diffusion restriction with corresponding decreased apparent diffusion coefficient (ADC) and T2 hyperintensity in the right frontal, parietal, temporal lobes and in the basal ganglia with areas of hypointensities on gradient echo sequencing, which suggested infarction in these areas with some areas of hemorrhagic conversion (Figure ).\nSubsequently, she was discharged to an inpatient rehabilitation center. While at the rehabilitation center, about four weeks after her stroke, she developed moderate to severe insidious onset headache. A repeat MRI, done four days after the onset of headache, showed diffusion restriction (with corresponding decreased ADC) and a ring-enhancing lesion in the right basal ganglia which involved part of the previous ischemic stroke. An extensive area of T2 hyperintensity was seen around this lesion consistent with vasogenic edema (Figure ).\nWith a recent invasive procedure along with the MRI findings, the possibility of an abscess was entertained, even though she did not have systemic signs of an infection (afebrile, WBC count 7800/mm3, negative blood cultures). She was empirically started on broad-spectrum antibiotics (vancomycin, cefepime, and metronidazole) and admitted to our institute for further management. On day three of admission to our hospital, she developed a high-grade fever and had an acute deterioration in her mentation that progressed to coma. An MRI was repeated to evaluate for any progression of the disease and to obtain stereotactic images for drainage. In addition to the previously mentioned ring-enhancing lesion, the post-contrast sequences now demonstrated enhancement of the right lateral ventricular wall which was suggestive of ventriculitis (Figure ).\nShe underwent a stereotactic drainage of the lesion, which aspirated purulent material. The patient was continued on broad-spectrum antibiotics. Vancomycin was discontinued after 10 days. Cefepime was switched to ceftriaxone, which along with metronidazole, was continued for a total of six weeks. An extensive laboratory workup was done which did not reveal a potential source of infection or immunocompromised state. Due to the high suspicion for an abscess and the purulent aspirate, a bacterial DNA probe was carried out on the aspirate, which revealed the presence of Fusobacterium necrophorum. Since Fusobacterium necrophorum is the implicated organism in Lemierre's syndrome, a surveillance of signs were carried out on the patient but failed to reveal neck pain or thrombosis of the internal jugular vein (imaged with an ultrasound of the neck). On post-drainage day one, her mental status improved and she progressed to her baseline prior to her discharge from the hospital. |
A 67-year-old female presented in July 2012 with defective vision in the right eye for six months. She was a known hypertensive and diabetic on treatment for both conditions for the last 15 years. There was no history of trauma, blood dyscrasias or use of antiplatelet or anticoagulant therapy. On examination, her best-corrected visual acuity (BCVA) was counting fingers at face in the right eye and 6/6 in the left eye. Intraocular pressure (IOP) by non contact tonometry (NCT) was within normal limits in both eyes. Anterior segment examination was unremarkable except for bilateral pseudophakia. Fundus examination of the right eye showed three hemorrhagic pigment epithelial detachments (PED) surrounded by serosanguinous fluid, while the fundus in the left eye was within normal limits. OCT of the right eye showed notched hemorrhagic PEDs with underlying polyps and surrounding subretinal fluid (SRF) suggestive of PCV (Figure 1 ). Based on the clinical picture and OCT findings, a diagnosis of PCV was made and the patient was treated with three loading doses of intravitreal bevacizumab at monthly intervals. After three injections, OCT showed evidence of scarring and resolution of SRF. Best corrected visual acuity improved to 2/60. The patient was kept under observation at monthly intervals for any signs of activity such as drop in vision, SRF with or without intraretinal fluid, PED and subretinal hemorrhage []. She followed up with us on a monthly basis till February 2013, thereafter she was lost to follow-up. After a gap of four months, she presented again to us in June 2013 with complaints of swelling, pain, and redness of the right eye since nine days. At this visit, vision in the right eye was no perception of light (NPL) and IOP was 50 mm Hg by applanation tonometry. Anterior segment examination showed lid edema, conjunctival chemosis, corneal epithelial edema, blood staining of cornea, a 3 mm hyphema, and a shallow anterior chamber. Corneal edema precluded a view of fundus and gonioscopy revealed closed angles in the superior, nasal and temporal angles that were unobscured by hyphema. B Scan ultrasonography showed annular appositional hemorrhagic choroidal detachment (Figure 2a ). The left eye was within normal limits. In order to reduce the pain and IOP, palliative 360° diode cyclophotocoagulation (CPC) was performed. Within four months of diode CPC, the eye became phthisical and the patient has been on regular follow-up till date without any discomfort. |
A 67-year-old female with history of chronic tobacco use, chronic obstructive pulmonary disease, hypertension, and hyperlipidemia, presented to the ED with symptoms of TIA. The patient described the acute onset of left-sided facial weakness that waxed and waned, recurring several times throughout the day, and lasting 2–3 minutes at a time. The left facial weakness was also associated with mild, left-arm weakness and “clumsiness” involving fine motor function of her left hand. She noted lightheadedness but denied leg weakness, headache, visual changes, chest pain or shortness of breath. She also noted that symptoms were brought on by use of her upper extremities and when she changed her body position from lying to sitting. She denied any similar symptoms previously or stroke history. Of note, she noticed a rapid improvement in her symptoms to resolution just prior to ED presentation.\nOn examination, her blood pressure (BP) was 183/86 millimeters of mercury (mmHg). She was awake, alert, oriented, and able to describe a detailed history. Her cranial nerves were intact, motor strength was 5/5 bilaterally, and fine motor movements in both her hands were normal. There was no ataxia, extraocular muscle dysfunction, or indication of posterior circulation involvement.\nJust after her initial asymptomatic presentation to the ED, her symptoms recurred when her systolic BP dropped by 20 mmHg upon standing from a supine position. Emergent computed tomography angiogram (CTA) of the head and neck demonstrated a severe flow-limiting lesion of the innominate artery (). Further investigation with magnetic resonance imaging demonstrated decreased signal intensity within the right internal carotid artery at the cavernous sinus and petrous segments, a finding that potentially represented slow flow ().\nThe patient subsequently underwent emergent cerebral angiogram, which demonstrated occlusion of the proximal innominate artery () at the aortic arch with resultant left to right vertebral artery steal phenomenon supplying the right subclavian artery (). The distal brachiocephalic artery flow was reconstituted via the subclavian artery and secondary steal phenomenon occurred into the right common carotid artery, causing delayed flow to the right cerebral hemisphere ().\nThe patient was maintained on a norepinephrine bitartrate infusion to increase BP, and her symptoms subsequently resolved. The symptoms recurred when she was positioned supine, but upon being placed in the Trendelenburg position her symptoms again resolved. The patient was therefore maintained with systolic BP goals between 160 and 210 mmHg. She remained asymptomatic during this period of elevated BP management. For definitive care, she underwent elective left carotid to right carotid “necklace” bypass surgery with complete and permanent resolution of her symptoms. |
A 21-year-old man presented for evaluation of a pathological femur fracture. The patient had been otherwise healthy until age 18, when he noted onset of lower back pain after soccer practice, which was exacerbated by exercise and improved when being resting. This pain was progressive and he started noticing a loss of height. Over the next several months he experienced sudden, intense lower back pain and was diagnosed with a femur neck fracture. He had surgery with internal fixation, and stayed mainly in bed for the next 6 months. During this time, he recognised a growing tumour (around 2 cm) in the inner thigh of his right leg. This tumour progressively grew until it was around 4 x 10 cm. During this time he also lost height (going from 1.75 m to 1.65 m) and weight (losing 15–20 kg in total), and noticed kyphosis in the thoracic spine and sternum protrusion. While he was walking, he fell and suffered a pathologic right femoral fracture. On physical examination, his vital signs at presentation were normal and he was noted to have kyphosis, sternum protrusion, and no Harrison’s sulcus or pain at rib palpation (see ). Interestingly, a 4 x 10 cm tumour was noticed in the inner thigh of his right leg (rigid consistency, with rough edges, partially mobile and not painful). The neurologic evaluation and strength were unremarkable.\nA biochemical evaluation () was noteworthy for severe hypophosphatemia associated with hyperphosphaturia and reduced tubular reabsorption of phosphorus (0.5 g in 24-hour collection), low serum 1, 25(OH) D, increased serum alkaline phosphatase (ALP). The serum 25(OH) D levels were low and the FGF-23 was 389 RU/m. The remainder of the chemistry profile was within normal limits, including the serum calcium and intact parathyroid hormone (PTH). A diagnosis of hypophosphatemic osteomalacia due to a tumour was made. The magnetic resonance scanning demonstrated a 9 cm mass in the medial right femur, which involved bone and soft tissue (see ).\nResection of the lesion revealed a spindle cell sarcoma with areas of necrosis and nuclear atypia that showed tendency to collagenation, and although it did not form malignant osteoid, cells were positive in immunohistochemistry for osteonectin, which suggests osteoblastic and osteogenic origin with high-grade fibroblastic osteosarcoma being the best fit option, and whose association with hyperphosphaturia is very rare. The neoplasm was in touch with resection borders.\nAfter surgery the patient persisted with low levels of phosphorus, and adjuvant chemotherapy was started with six cycles of adriamycin and cisplatin, with a consequent normalisation of phosphorus levels (2.7 mg/dl). |
A 54-year-old Caucasian woman with open angle glaucoma and a history of suboptimal medical therapeutic compliance owing to an intolerance to drops was referred to our Ophthalmology Department. She was severely obese (body mass index 37 kg/m2) and had type 2 diabetes but was using insulin with good metabolic control. Her best corrected visual acuity (BCVA) in both eyes was 1.0. Her IOP was 35 mmHg without the use of medication, though she achieved values of 18 mmHg in her right eye and 16 mmHg in her left eye with the use of tafluprost once daily. No changes were identified in an examination of her anterior segment. The papillary cup was 0.3 in her right eye with a temporal notch and her left eye had a normal appearance.\nOur patient’s compliance to medical therapy continued to be poor, because she blamed her drops for coughing and dyspnea attacks. Without the use of drops, her IOP values remained consistently in the 30s. After continuous non-compliance with several other ocular medications, surgery was discussed as an alternative treatment option. The risks and potential complications of surgery were explained to our patient. No signs of cornea or ocular media opacity, retinal hemorrhage, macular or peripheral detachments, or other contraindications were observed in either eye. Our patient decided to opt for surgery. Her right eye was operated on first with an uneventful mitomycin C trabeculectomy (0.3 mg/ml, 3 minutes).\nOn the first postoperative day, our patient presented with a diffuse, functioning, and non-leaking filtration bleb, associated with a well-formed anterior chamber, and an IOP of 8 mmHg, with a normal appearance on fundoscopy. Two weeks postoperatively she complained of decreased visual acuity; her BCVA was 20/32 and her IOP was 10 mmHg without medication. Fundoscopy exhibited multiple superficial, flame-shaped retinal hemorrhages located centrifugally from the optic disc associated with optic disc edema (Fig. ). There was no evidence of choroidal effusion. A fundus examination of her left eye was unremarkable.\nOptical coherence tomography (OCT) revealed folding of the macular retina associated with a small detachment of the neurosensory retina (Fig. ). The angiographic pattern showed macular microaneurysms associated with fluorescein diffusion, peripapillary hemorrhages, and late optic disc leakage, without ischemic areas or neovascularization (Fig. ). One month later the overall fundoscopic changes resolved spontaneously (Fig. ). Given this situation, a suspected diagnosis of DR was proposed. Other possible diagnoses were retinal venous occlusion or Valsalva retinopathy, but these were considered unlikely given the diagnostic results pattern. Subsequent follow-up visits were satisfactory, with our patient maintaining a steady IOP of 8–14 mmHg without medication. The peripapillary hemorrhages and optic disc edema spontaneously recovered during the second postoperative month, and our patient’s BCVA reached 20/25.\nThree months later, she underwent an uncomplicated left eye trabeculectomy with mitomycin C (0.3 mg/ml, 3 minutes). No postoperative hypotony was registered. On the first postoperative day, the anterior chamber was formed, the bleb was diffuse, her IOP was 8 mmHg, and results from fundoscopy were normal, without choroidals. The pattern of clinical evolution of this eye was similar, with decreased vision complaints 10 days after surgery (her BCVA was 20/32). Her IOP was 9 mmHg, with no medication. A fundus examination revealed identifiable multiple peripapillary retinal hemorrhages, optic disc swelling, and macular edema (Fig. ). OCT revealed macular folding and neurosensory retinal detachment (Fig. ). Choroidal striation, optic disc leakage, and signs of macular microangiopathy and epitheliopathy were visible on angiography (Fig. ). The localized hemorrhages and sectorial optic disc edema reduced progressively and her macular edema recovered spontaneously during the first postoperative month (Fig. ). Her left eye IOP on the last follow-up visit was 16 mmHg without hypotensive medication and her BCVA was 20/25.\nGiven the occurrence of the same pattern in the contralateral eye, our patient’s complete medical history was reviewed again, with inquiries about possible hypertensive peaks, usual medication, and frequent Valsalva maneuvers. A summary infectious test was performed to rule out any possible systemic causes of bilateral papillitis. Results for the infectious study were negative. |
A 72-year-old male visited a local physician with a 20-day history of progressive abdominal distension and bloody stool. The following day the patient was referred to Ibaraki Medical Center (Ami, Japan) with a diagnosis of rectal cancer. Patient medical history was otherwise unremarkable. On physical examination, all findings were unremarkable with the exception of slight pallor in the palpebral conjunctiva. Hematological investigations revealed anemia (hemoglobin levels, 9.6 g/dl; hematocrit, 28.4%). Other laboratory tests and serum levels of carcinoembryonic antigen and carbohydrate antigen 19-9 were all within normal limits. Colonoscopy revealed the entire circumference of an elevated tumor with central depression and erosion at the lower rectum. A biopsy specimen from the tumor was indicative of a moderately differentiated adenocarcinoma. Abdominal computed tomography (CT) revealed thickening of the rectal wall with regional lymph node swelling but no liver metastasis. Chest CT revealed two metastatic lung tumors measuring 25 mm (in the middle lobe of the right lung) and 10 mm in diameter (in the lower lobe of the left lung) (). A diagnosis of rectal cancer with multiple lung metastases was made, and abdominoperineal resection with lymph node dissection was performed in October 2009. Light microscopy revealed that the tumor had infiltrated the deep tissue layer through the muscularis propria layer of the rectum and that there were cancer metastases in 12 of the 14 lymph nodes. The tumor was diagnosed as stage IVA (T3, N2b, M1a) according to the International Union Against Cancer Tumor Node Metastasis classification (7th edition) (). The patient refused resection of the lung metastases and placement of a peripherally inserted central venous (CV) port, and was hesitant to be treated with oxaliplatin and capecitabine due to potential peripheral neuropathy and hand-foot syndrome as side effects. On providing informed consent, the patient was administered IRIS plus bevacizumab combination therapy against the lung metastases. S-1 (100 mg/body) was administered orally on days 1–14 of a 28-day cycle, and irinotecan (125 mg/m2) and bevacizumab (7.5 mg/kg) were administered by intravenous infusion on days one and 15. Following three courses of therapy, the metastatic right lung tumor decreased in size to ~10 mm in diameter, and the left lung tumor had decreased in size to ~3 mm in diameter (). Following six courses of therapy, the metastatic right lung tumor had become scar tissue and no metastases could be detected in the left lung (). Following nine courses of therapy, no metastatic lung tumors could be identified (). The response was declared clinically complete. The patient refused additional treatment following nine courses of therapy, and there was no recurrence 36 months after the final course of therapy. |
This case describes a 26 year-old G1P0 who presented to a large Manhattan hospital at 37 weeks 6 days for a scheduled external cephalic version. Her past medical history was notable for well-controlled chronic Hepatitis B with an undetectable viral load (< 10 IU/mL) and normal liver function tests. In triage, she was found to have oligohydramnios and a confirmed frank breech presentation. Accordingly, delivery by cesarean section was recommended. As per routine practice in this hospital, the patient was tested for SARS-CoV-2 in triage. She denied symptoms of the disease at the time, was clinically perceived to be asymptomatic, and had normal vitals and a benign physical exam. While in triage, she began to have regular contractions on tocometer, so the decision was made to proceed with the cesarean section prior to obtaining results of the COVID testing. She was treated as a “person under investigation” (PUI) and healthcare personnel took the recommended precautions, including donning the appropriate personal protective equipment, having a more senior resident with an attending perform the cesarean section, and proceeding with spinal anesthesia to avoid aerosolizing procedures. Laboratory values prior to surgery inclusive of platelets (257 K/µL), prothrombin time (13.4 s), and international normalized ratio (1.0) were reported as normal (Table ). Per routine protocol, she was type and crossed upon admission, with whole blood available upon request from the blood bank.\nFrom the start of the case, there was more than an expected amount of bleeding noted, beginning from the insertion of the spinal needle and continuing through the initial incisions through the subcuticular and subcutaneous layers. The cesarean section proceeded in routine fashion. Following delivery of the fetus, uterine atony was noted with moderate bleeding, which persisted after administration of oxytocin and closure of the hysterotomy. Given that the patient had no history of elevated blood pressures and perioperatively had blood pressures of 90's/40's, the decision was made to administer 0.2 mg IV methylergonovine. A second dose was later administered for intermittent uterine atony after 30 min, with significant improvement in uterine tone. Hemostasis at the hysterotomy was confirmed and the muscle and fascia were closed. Just prior to closure of the subcuticular layer, it was necessary to cauterize multiple bleeding capillaries within the subcutaneous layer to achieve hemostasis. The subcuticular layer was closed with 4 − 0 vicryl. At this time, significant and persistent oozing was noted from the skin incision. Pressure was applied for several minutes with an improvement and then a tight pressure dressing was placed. During the case, administration of tranexamic acid was considered given significant intraoperative oozing; however, the decision was made to expectantly monitor the patient, given her unknown COVID status and the possible risk of exacerbating an existing hypercoagulable state. After several hours of monitoring, the patient had no further active bleeding from the abdominal incision and her vaginal bleeding was minimal. The total estimated blood loss was 1000 mL and her postoperative hematocrit was 27.6%.\nApproximately 8 h after her surgery, nasopharyngeal testing by PCR resulted positive for SARS-CoV-2. Upon further questioning, the patient endorsed that she had been experiencing a very mild cough for several days prior to presentation that she did not find significant nor bothersome; she was not experiencing symptoms during her admission. Labs were drawn in response to the positive COVID testing postoperatively and reflected abnormal COVID labs in the setting of relatively normal coagulation factors. Specifically, postoperative laboratory values revealed an elevated D-Dimer, elevated LDH, elevated CRP, normal PT/INR, and normal fibrinogen values (Table ). While D-Dimer, CRP, and LDH elevations can be expected in the setting of inflammation and tissue damage, as one may expect post-operatively, the increase in D-Dimer was significantly higher than what has been noted from patients postoperatively after cesarean section at this hospital. D-Dimer levels have generally been observed to range from approximately 2–6 µg/mL among the population referenced at this time of this publication, while this patient’s post-operative D-Dimer was 19.1 µg/mL – a notable difference.\nThe patient remained stable and afebrile, and she was ultimately discharged on postoperative day two in excellent condition, asymptomatic, with pain well-controlled, and meeting all postoperative milestones. Upon postpartum evaluation by telehealth several days after discharge, the patient reported that she was recovering well without concerns, and she was asymptomatic without any further symptoms of COVID-19. |
A 74-year-old male presented in February 2016 with increasing dysphagia. There was no history of prior abdominal infection or surgery. On esophagogastroscopy, a necrotic and circumferential friable tumor was seen at 33 to 40 cm from the incisors, with an endoscopic appearance of involvement of gastroesophageal (GE) junction and the proximal 2 cm of the stomach. Biopsies of the distal esophageal tumor confirmed poorly differentiated adenocarcinoma. The patient was anemic with a hemoglobin of 89 g/L. Staging endoscopic ultrasound suggested a breach of muscularis propria and four enlarged paraesophageal nodes. Neoadjuvant chemoradiotherapy followed by esophagectomy was initially considered; however, a staging positron emission tomography (PET) scan demonstrated 18-fluorodeoxyglocose (FDG) uptake not only in the primary tumor, but also in the paraesophageal region near the GE junction and upper abdominal lymph nodes extending as far inferiorly as the right renal vessels, in a retrocaval location (Figure ).\nA radiation oncology consultation was sought regarding treatment options of such extensive lymphadenopathy. Palliative radiation therapy (RT) was recommended. The patient was also evaluated by a medical oncologist who advised that chemotherapy may be considered after assessing the response to palliative radiotherapy.\nFrom March 21, 2016 to April 5, 2016, the patient received palliative RT to the symptomatic primary tumor and closest adjacent nodes using a pair of anterior and posterior fields. A total dose of 30 Gray (Gy) was prescribed over 10 daily fractions. As the lymphadenopathy in the lower abdomen was not symptomatic, and would have contributed to increased toxicity, this region was deliberately excluded from the high dose RT volume (Figure ). Other than very mild odynophagia, the patient had no other RT-related side effects. On the first follow-up visit, one month following treatment completion, he had improved swallowing function and a weight gain of six pounds.\nFollow-up computed tomography (CT) scan was obtained on May 24, 2016 to evaluate for the suitability of chemotherapy and to serve as a baseline during systemic therapy. This demonstrated persistent thickening of the lower esophagus, with lymphadenopathy reported to have decreased in size and no significant retroperitoneal adenopathy. When given the option of receiving palliative chemotherapy, the patient declined and chose to continue on observation only. Further CT scans in August and October 2016 showed a complete response in the irradiated primary tumor and nodes, with a stable 10 mm lymph node at the right renal vein.\nIn January 2017, due to symptoms of increasing dysphagia, the patient was assessed by a thoracic surgeon for consideration of esophageal stent placement. Endoscopy on January 12, 2017 noted that there was a possible small amount of residual tumor at the GE junction, but there was no significant narrowing or stricture, and no biopsies were taken. A further CT scan on April 10, 2017 showed minor circumferential thickening of the distal esophagus, but unchanged from previous. Paraesophageal lymphadenopathy was reported to be unchanged. The PET-positive lymph node at the renal vein decreased from 10 mm to 5 mm.\nThe patient’s symptom of dysphagia resolved spontaneously, and an evaluation was made with a further PET scan on May 19, 2017 (Figure ). This demonstrated mild residual FDG activity within the distal esophagus, more likely inflammatory change rather than malignancy. The FDG activity within all the lymph nodes, both treated and untreated, had unexpectedly resolved. |
An 80 years old woman was admitted to female medical ward at Shalamar Hospital Lahore with history of yellow discoloration of sclera for 15 days and altered state of consciousness for 10 days and history of melena for one day. She had no history of other comorbidities like diabetes, hypertension or chronic liver disease. Clinically she was vitally stable having deep jaundice and flapping tremors. Liver was palpable one finger below the costal margin tender having soft consistency. At the time of admission her lab investigations including complete blood urea, creatinine and serum electrolytes were normal LFTs showed total billurubin 6.9mg/dl, ALT 1560 U/L, AST 1398U/L, alkaline phosphatase 540 U/L, albumin 2.5 g/dl, PT prolonged 3 sec, USG shows hepatomegaly with total liver span of 13.5cm with fatty change. Her viral hepatitis screen including Anti HAV, Anti HEV and HBsAg were negative, these tests were performed by using electrochemiluminescence technology. Her Anti HCV was positive with cut off value of 1.00 and patient value of 10.17. Her HCV RNA by PCR was detected with viral load of 6.1 x 105 IU/ml. She developed progressive encephalopathy and coagulopathy. Her peak serum bilirubin was 15mg/dl. Due to deranged coagulation profile liver biopsy was relatively contraindicated. She was treated conservatively, and patient gradually improved and discharged from hospital.\nThe diagnosis of fulminant hepatitis due to acute hepatitis C was based on the assessment of clinical, virologic measures. None of the medications that were administered to the patient were known to be hepatotoxic. There was least possibility of drug induced hepatitis as patient first presented in our clinic with no previous visits to any doctor or hospital stay in the previous six months. She was not taking any homeopathic, Unani medicines and also not taking regular allopathic medications. None of the serum samples had detectable levels of hepatitis B surface antigen, IgM antibodies against hepatitis A or hepatitis E. LFTs done in this patient strongly supported acute hepatic insult. The autoimmune hepatitis is very rare in this age group and no other signs of autoimmune disease were found. We should have excluded the possibility of co-infection with hepatitis G virus (HGV) but we had no facility to perform Anti HGV in Pakistan besides the prevalence of acute hepatitis G in still unknown in Pakistan. Based on these observations diagnosis of FHF due to hepatitis C was made. |
Our patient was a 31-year-old Yemenite man, with no significant past medical or surgical history, who was not on anticoagulants. He presented with progressively worsening dyspnea on exertion and limited functional capacity over the last few years. His physical examination was significant for an opening snap, a grade 2-3/6 diastolic murmur and a 1/6 holosystolic murmur. Laboratory parameters were significant for a normal coagulation profile. Our patient’s electrocardiogram showed left atrial enlargement and no evidence of ventricular hypertrophy. A transesophageal echocardiogram revealed normal left ventricular systolic function with an ejection fraction of 60% and left atrial dilation. The mitral valve was minimally calcified with mild thickening limited to the leaflet margin without chordal involvement. Characteristic echocardiographic features of rheumatic mitral stenosis were present with a Wilkins score of three []. The mean mitral gradient was 11mmHg and the valve area was 1.1cm2. Given our patient’s age, New York Heart Association Class III symptoms and mitral valve morphology, he was deemed to be a good candidate for MBV. Heparin was administered with an activated clotting time measured as 272 seconds. Valvuloplasty was performed utilizing a transeptal approach with a 26mm Inoue-balloon catheter.\nAt the end of the procedure, our patient was noted to have an effusion on the echocardiogram, with features of cardiac tamponade. He was otherwise hemodynamically stable. The effusion was treated with emergent pericardiocentesis and a pericardial catheter was left for continuous drainage. Pericardial catheter output ceased after the drainage of approximately one liter of blood. An hour after arrival to the intensive care unit, a pericardial effusion was noted again on surveillance transthoracic echocardiography. Our patient drained 350ml/h of bright red blood for the next three hours despite a normal coagulation profile. During this time, his presumed coagulopathy was corrected with protamine (25mg intravenously), two units of fresh frozen plasma, two single donor platelets and six units of cryoprecipitate, and his coagulation profile then rechecked. Complete evacuation of his pericardial sac was verified hourly with surveillance transthoracic echocardiography. Despite a normal coagulation profile the bleeding persisted, with its exact site of origin unknown. Our patient was warm, had normal acid-base status, and remained hemodynamically stable throughout, yet an additional 350cc of blood was drained within the next hour. At that time, the decision was made to administer 4.8mg (70μg/kg) of aFVII intravenously over two minutes. Drainage from the pericardial tube almost immediately ceased. Transesophageal echocardiography revealed resolution of the hemopericardium.\nOur patient is doing well two years after the MBV. He has a mitral valve gradient of 4mmHg and he has functionally improved to New York Heart Association Class I. His coagulopathy work-up was negative. |
The patient was an 11-year-old Caucasian boy without any relevant familiar history or comorbidity. He referred to our clinic complaining disability in walking and running with frequent falls (due to the lack of power in knee extensor mechanism). His parents said that despite the absence of pain, the functional limitation of both his knees was at the base of a serious psychological discomfort, conditioning his relationship with peers.\nOn objective examination, his lower limbs revealed mild genu valgum and tibial proximal lateral torsion. Both patellae were on the lateral aspect of the femoral condyle displacing even further during knee flexion. Femoral quadriceps function was present but this muscle was working as a flexor instead of extensor of the knee. It was observed that he was unable to actively extend both knees from the flexed position; passive range of motion (ROM) was complete and was not painful. At the upper limbs, there was a mild deficit in ROM of both elbows. There were no pathological deviation of the spine; normal clinical finding of both feet. The anterior-posterior lateral and axial knee X-rays showed bilateral, superolaterally displaced and hypoplastic patellae and reduction of the trochlear groove (, , ).\nThe extensor mechanism was studied by magnetic resonance imaging (MRI): Both quadriceps were hypotrophic with the vastus medialis enveloped over the trochlear groove which appeared flattened and dysmorphic. The patellar tendons were bilaterally lateralized and stretched. The anterior cruciate ligament could not be identified. On both sides, MRI confirmed the lateral dislocation of the patellae ().\nElbows X-ray showed a slight posterior dislocation of the radial head and absence of radioulnar synostosis. The diagnosis of CDP was confirmed. Surgical correction of the deformity was then proposed to regain walking and running function, with the aim of reduce the grade of the deformity improving the patient quality of life and his psychological well-being. |
A 43-year-old man with a normal Body Mass Index (BMI) presented to our hospital complaining of chronic right chest pain located in the dorsal area and external mammary region. She was submitted to radiation therapy, right mastectomy, full axillary and mammary lymph node dissection and 7 years before our evaluation and now she is still doing lipofilling injections and taking tamoxifen. We used a DN4 questionnaire to detect a neuropathic component of pain, this is a 10 items “yes or no” questions and 4 positive answers mean that the pain is likely to have a neuropathic component.[] DN4 score was 7, while his numerical rating scale (NRS) was 10. The physical examination revealed a tout band above the right Rhomboid major muscle with some tender points and the stimulation of these increased the pain. With these scores and clinical presentation, we suspected a mixed nociceptive and neuropathic mechanism of pain and a coexisting MPS. Paresthesia, dysesthesia, and pain were reported from the mid-clavicular right line to the dorsal area, located at T3 to T7 level. This problem increased during the night, thus the night-sleep quality was poor. The scar was not painful.\nShe had hypoesthesia to pinprick and temperature.\nTreatment after this from his general practitioner was tramadol (maximal daily dose of 300 mg continued for 30 days), duloxetine 60 mg and pregabalin (maximal daily dose was 300 mg continued for 30 days). This treatment was discontinued because it did not relieve his symptoms.\nWe performed an ultrasound-guided ESP block at the right T5 transverse process with the patients placed in a prone position and we injected levobupivacaine 45 mg and triamcinolone 40 mg within 15 ml of normal saline. The linear transducer (13-6 MHz, Mylab One, Esaote) was placed perpendicular to the transverse T5 processes and we used an in-plane technique with a caudal to cranial approach in order to place the needle tip below the ESP muscle by using the already described “deep needle approach.”[]\n7 days later the NRS decreased to 8, and the DN4 was still 6, thus we decided to repeat the same injection, and after 7 days NRS decreased to 5 and DN4 decreased to 1.\nThe neuropathic component of the pain disappeared as the paresthesia, the night-sleep was markedly improved while the hypoesthesia was still there as the MPS.\nWe performed a Rhomboid intercostal block (RIB) and hydro dissection to reduce MPS. The patient was placed in the lateral position with the affected side uppermost and the scapula was moved laterally, the ultrasound probe was placed in the sagittal plane at the T4-5 level, just 2 cm medial to the scapula, to identify the trapezius muscles, the rhomboid major muscles, and the intercostal muscles. A 22-gauge needle was inserted into the plane between the rhomboid major and the intercostal muscles in a caudal to the cephalic direction. We injected levobupivacaine 45 mg and triamcinolone within 15 mL of normal saline and we confirmed the appropriate spread with the US.\n14 days later NRS was 2 and DN4 was 0, 3 months later the result was the same and the patient did not assume any analgesic drugs. An informed consent was obtained from the patiens for the treatment and the pubblication of the report. |
A 50 year-old male, with a history of coronary arterial bypass grafting 14 years back, presented with shortness of breath and dry cough. An X-ray revealed a large mass in the left hemithorax adjacent to the heart silhouette. A chest CT demonstrated the presence of a mass with smooth edges, in middle mediastinum next to the heart and partially intrapericardial (Fig. ). The mass was of heterogeneous density and of 11 cm size. Presence of atelectasis at the left lower lobe abating the mass was clearly seen. Based on clinical and radiologic evidence, we did proceed with CT guided FNA of the mass. The cytology findings revealed inflammatory lesion. Laboratory tests were normal. Based on patient symptoms, history and the presence of a mass potentially compressing the cardiopulmonary structures in vicinity, we decided to offer exploratory surgery for diagnosis and treatment.\nStandard hemodynamic monitoring and general anesthesia were followed by positioning, prepping and draping patient in left lateral decubitus position. An anterolateral left thoracotomy was carried out and entrance in the hemithorax was made without any challenge. The mass was assessed and found to be leaning medially on the surface of the lateral wall of the left ventricle, including the pericardial layer and had smooth edges which didn’t infiltrate the lung (Fig. ). We started dissecting the mass from its smooth capsule, making it through all its layers. An old and degraded piece of surgical swap was visualized (Fig. ). The surgical swap was removed along with the capsular layer of this mass. Patient tolerated the procedure very well and blood loss was minimal. A chest tube was inserted in the left hemithorax and chest wall was closed following standard procedures.\nIn the immediate post-operative phase, patient improved steadily and on day four was discharged home symptom-free. In the long-term follow -up, patient was found to remain without symptoms. |
History and exam
A previously healthy eight-month-old male presented with fever, vomiting, irritability, and decreased oral intake. On exam, he was found to have signs of meningeal irritation. Of note, he did not demonstrate any focal neurologic deficit at the time of initial presentation. A lumbar puncture was attempted, though thick purulence was encountered prior to obtaining a cerebrospinal fluid sample; cultures demonstrated methicillin-resistant Staphylococcus aureus (MRSA). He was admitted to the pediatric intensive care unit and antibiotic therapy was initiated. Two days later, however, he was noted to be acutely paraplegic and diffusely hypotonic, prompting neurosurgical consultation. MRI of the spine demonstrated an extensive epidural fluid collection extending from upper cervical spine to lumbar spine and focal epidural thickening at the L5-S1 level that communicated with a peripherally enhancing 2.4 cm abscess in the paraspinal and psoas musculature. There was upper thoracic spinal cord compression and T2 cord signal abnormality noted in the cervical and thoracic spine (Figure ).
The patient was taken to surgery for an emergent T3 laminotomy for decompression. He tolerated this procedure well. He initially did well postoperatively, with the return of normal muscle tone and motor function, and appropriate pathogen-directed antibiotic therapy was continued. Repeat MRI of the spine was obtained three days later due to fluctuating neurologic exam, persistent fevers, and redemonstration of MRSA bacteremia after initial clearance (Figures ). This demonstrated worsening of the extraspinal abscess and recurrence of the epidural fluid collection. Percutaneous drains were placed by the interventional radiology team for attempted source control of the presacral and psoas abscesses. However, given limited response to medical management and continued intermittent myelopathy on examination, the patient returned to surgery on hospital day 12 for apical laminotomies with catheter-directed irrigation and drainage.
Operation
Laminotomies were made at T3 and L4 to expose the epidural space, with the utilization of the prior |
Male patient, 54-year-old, feoderm attended the university hospital Oral and Maxillofacial Surgery and Traumatology Department of the Federal University of Mato Grosso do Sul, Mato Grosso do Sul, Brazil, on referral by a public health professional. Ovoid swelling was found >5 cm in diameter on the right submandibular region [Figures and ]. Clinical examination showed a mobile lesion, well defined and soft on palpation. The patient denied having suffered any trauma or having undergone any surgical procedure in that region. He reported that the lesion began to develop 10 years ago and decided to seek expert help mainly for esthetic reasons, as this 10-year period had no symptoms. The patient had no significant medical history.\nComputed tomography (CT), ultrasound and fine needle aspiration (FNA) were performed. A CT scan showed large encapsulated homogeneous mass, near the right mandibular angle. The lesion was >5.0 cm in diameter, with a clear boundary, [].\nUltrasound image showed hypoechoic cystic lesion, well outlined, without the Doppler vascularity without calcifications and measuring 5.3 cm × 4.6 cm × 5.1 cm [].\nFNA produced a very characteristic odor when the contents of the cyst was aspirated. The material collected with FNA had a thick consistency. Report of FNA cytology revealed the presence of rare vacuoles of fat and no malignancy criteria, which was very nonspecific. Therefore, based on the clinical information and laboratory test, it was decided to perform an excisional biopsy of the lesion. The enucleation of the lesion was performed under general anesthesia, and surgical specimen was sent for histopathological examination to confirm the diagnosis []. Microscopic examination showed cystic lesion lined by stratified flat epithelium. Granular layer well developed and it showed the presence of ortoceratina in light of the cyst [].\nA skin track was removed to compensate for the excessive presence of expanded skin and prevent its sagging []. The patient returned after 7 days, for review. The surgical wound was in the repair process within the normal range. The patient is in outpatient follow-up []. |
A 55-year-old male patient complained of dysphasia for 4 weeks and continuous deterioration for 5 days. The patient had a history of ischemic stroke (Fig. , ), which manifested as right limb numbness and amaurosis 1 year ago. He had a history of hypertension for 10 years, a myocardial infarction 3 years ago, and cigarette smoking for 40 years, but no history of diabetes, hyperlipidemia, and alcohol consumption. The patient exhibited a disorder of linguistic expression and long-term memory impairment without a limb motor disorder. Cranial MRI confirmed an ischemic stroke at a local hospital (Fig. , ). The symptoms were relieved after medical treatment. The dysphasia relapsed 5 days before admission. Cranial computed tomography (CT) showed a hypodense lesion in the left temporal and occipital lobes (Fig. ). Cerebral infarction was considered based on serial cranial MRI, which showed hyperintensity at the internal border zone and the border zone between the left temporal and occipital lobes, and Doppler ultrasound revealed severe left carotid artery stenosis. The patient was transferred to our stroke center for further treatment. At the time of admission, the vital signs were normal. A neurologic examination showed mild dysphasia, normal cranial nerves, normal limb motor function, and negative Babinski signs bilaterally. CT perfusion imaging showed mild ischemic in the territory of the left middle cerebral artery (Fig. ). Cervical computed tomography angiography (CTA) confirmed the first segment of the carotid artery with severe stenosis (Fig. ). An electrocardiogram revealed an abnormal Q-wave involving the inferior wall, which was consistent with an inferior myocardial infarction. Homocysteine (35.3 μmol/L) and the following laboratory investigations were normal: triglycerides, total cholesterol, low-density lipoprotein cholesterol, antinuclear antibodies, anti-neutrophil cytoplasmic antibodies, prothrombin time, and tumor markers. The patient was given medical treatment consisting of aspirin (100 mg per day), clopidogrel (75 mg), and atorvastatin (20 mg). The patient received combined anti-platelet treatment with 100 mg of aspirin and 75 mg of clopidogrel daily for a week prior to the intervention. Under local anesthesia, the patient underwent placement of a left carotid artery stent (Fig. , ). The patient was discharged 3 days post-operatively. Two months after CAS, the patient experienced right limb numbness and mild dysphasia with a severe headache. The recurrent symptoms and each episode lasted approximately 2 min. An emergency cranial MRI showed massive hyperintense lesions in the left temporal and occipital lobes with ventricular compression (Fig. , ). The cranial CT performed on the same day indicated no cerebral hemorrhage (Fig. ). A neurologic examination was also negative. On the basis of a detailed medical history, a diagnosis of hyperfusion was proposed; an anti-hypertensive and mannitol were introduced, and aspirin was discontinued. Despite the medical treatment, the headache worsened. A repeat cranial CT revealed that the cerebral edema had worsened (Fig. ). Based on the failed medical treatment and imaging features, a brain tumor was considered. Repeat MRI showed multiple ring-enhanced lesions in the left temporal, parietal, and occipital lobes complicated by massive brain edema (Fig. , ). The patient underwent whole-body positron emission tomography–computed tomography (PET/CT), which showed that the increased FDG metabolism in the left parietal and temporal lobes was consistent with malignant lesions. Revised medical treatment included albumin, furosemide, and dexamethasone. Then, the patient was transferred to the neurosurgery ward pre-operatively. The final pathologic diagnosis was glioblastoma multiforme (WHO grade IV). A post-operative intracranial CT showed regression of the cerebral edema (Fig. , ).\nGBM is a malignant tumor with a dismal prognosis that is often associated with extensive angiogenesis due to tumor secretion and local effects exerted by vascular endothelial growth factor, a major regulator of angiogenesis and other cytokines []. The patient described herein initially presented with ischemic stroke and severe ipsilateral carotid artery stenosis and an intracranial massive lesion worsened after CAS. Cerebral hyperfusion syndrome was suspected due to headaches, cerebral edema, focal neurologic deficits, serial images, and a pathologic diagnosis of a GBM. Hyperperfusion syndrome is a relatively rare complication of carotid artery revascularization procedures and may have adverse clinical consequences in 1.1–25.0 % of patients after CAS []. Delayed hyperperfusion syndrome has been reported [], which may be related to prolonged impairment of cerebrovascular autoregulation. Some questions remain which should be addressed. The course of the disease was approximately 2 years, and the patient had numerous atherosclerotic risks, including hypertension, smoking, and coronary artery disease. The patient had right limb weakness and numbness, amaurosis, and mild dysphasia. MRI showed a left internal border zone cerebral infarction and severe left carotid artery stenosis. Thus, an ischemic stroke was not only misdiagnosed, but CAS was reasonable. Imaging features of GBM have no special characteristics. Multiple model MRI images are useful in the diagnosis of GBM and its tumor grade [, –]. The characteristic MRI findings of GBM include enhanced heterogeneous ring mass lesions with significant peritumoral cerebral edema, necrosis, or hemorrhage [, ]. These features also occur in patients with ischemic strokes [, ]. GBMs often pose a diagnostic dilemma on anatomic MRI and may require a surgical biopsy for a definitive diagnosis. In the current patient, whole-body PET/CT revealed no systemic tumor and brain metastasis was ruled out.\nDid CAS prompt the growth of the GBM? The left cerebral hemisphere was ischemic secondary to severe carotid artery stenosis, while the GBM was dependent on the blood supply. Brain ischemia and the GBM were improved when the left carotid artery stenosis was recanalized, which may induce tumor growth. Relative cerebral blood volume (rCBV) maps and measurements have been shown to correlate reliably with tumor grade and histologic findings of increased tumor vascularity [, , ]. In addition, a study has proved that if the rCBV is >1.75, there is a high probability that the tumor will be a high-grade glioma []. GBM complicated with carotid artery stenosis is rarely reported, and there is insufficient evidence to prove a relationship between a worsening tumor and CAS. |
A 63-year-old man presented with low back pain and was found to have a vertebral soft tissue mass in the second lumbar vertebra (L2), a right kidney 7 cm contrast-enhancing tumor, multiple subcentimeter lung lesions, and bilateral adrenal nodules. Needle biopsy of the kidney tumor was consistent with RCC. He underwent palliative radiotherapy to the L2 vertebral lesion and debulking right nephrectomy, with pathology showing Fuhrman grade 3, clear cell RCC. He was started on front line axitinib through a randomized, double-blind phase II study of axitinib with or without dose titration in patients with metastatic renal cell carcinoma. At that time, he did not have symptoms to suggest active CAD, and risk factors for CAD included mixed type hyperlipidemia (normal cholesterol level on simvastatin 20 mg), a former 15 pack-year smoking history (quitted 30 years earlier), and hypertension. His blood pressure readings at baseline were 119/71 mm Hg on metoprolol tartrate 50 mg and hydrochlorothiazide 25 mg. His creatinine level was 1.68 mg/dL with a calculated GFR of 41 mL/min/1.73 m2, which remained relatively stable throughout his disease course. Amlodipine 10 mg was added for grade 3 hypertension that developed on axitinib, and his blood pressure improved to 120/70 mm Hg. Two years and 5 months later, he experienced worsening recurrent chest pain 3 days after holding axitinib for grade 2 anorexia. EKG showed borderline T wave inversion in the anterior leads, and cardiac enzymes were within normal limits. A thallium stress test showed mild reversible changes involving the inferior wall. Further cardiac catheterization showed stenosis involving multiple vessels, including 95% of ostial RCA, 90% of mid and inferior segment of the posterolateral branch, 50% of distal left main, 50–60% of proximal and mid LAD, 90% of distal LAD, and 80% mid circumflex, and diffuse stenosis in distal LAD and mid circumflex. His calculated LVEF was 80% on stress test, and left ventricular systolic function was normally evaluated by ventriculogram. He was evaluated by cardiovascular surgery and deemed not a candidate for surgical intervention. Subsequently, he underwent staged PCI: stage I intervention with 4 DES was placed in ostial RCA, superior branch posterior left ventricular branch, inferior portion posterior left ventricular branch, and posterior descending artery. Stage II intervention with 2 DES was placed in mid-LAD and the circumflex 10 days later. Axitinib was held until 12 days after the stage II PCI. Metoprolol tartrate 50 mg and fenofibrate micronized 48 mg were added; his hypertriglyceridemia was improved from 408 mg/dL to 241 mg/dL. However, he subsequently experienced two more episodes of angina 6 months and 1 year and 10 months after the first angina and received 1 DES for a proximal 95%–99% LAD stenosis and 1 DES for a mid-LAD 95% stenosis, respectively. Axitinib was interrupted for 12 days and 24 days for these events, respectively. Both repeated echocardiograms after these two PCI interventions showed normal sized right and left ventricles with normal systolic function. He had disease progression after the third episode of angina, and the dose of axitinib was increased to 5 mg twice daily from 3 mg twice daily, which resulted in regression of his RCC. He is currently on axitinib 5 mg for an additional year without recurrent cardiac events. summarizes the major clinical features of the three cases. |
A 66-year-old Caucasian man with a past medical history of gastroesophageal reflux presented with two days of right-sided abdominal pain, chills, nausea, and emesis. He was tachycardic but afebrile and had significant right upper quadrant (RUQ) tenderness. Blood work revealed leukocytosis, an elevated lactate of 3.7, and elevated bilirubin of 2.0. CT-scan revealed gallbladder wall thickening and pericholecystic fluid, wall thickening and fat stranding of the duodenum and ascending colon, and a mesenteric soft tissue mass. He was resuscitated, placed on bowel rest, and started on intravenous ertapenem at a dose of 1 g daily. Over the next 36 hours, clinical improvement was observed and WBC and CRP started to normalize. Repeat CT-scan with oral and intravenous contrast showed improvement of the RUQ inflammatory process and revealed a diverticulum of the second portion of the duodenum-associated inflammatory changes and a large pseudoaneurysm of the inferior pancreaticoduodenal artery. The scan also delineated the previously noted soft tissue mass as a stable hematoma (Figures –). Gastroenterology was consulted with a plan to perform endoscopic evaluation after clinical improvement and resolution of the hemorrhage. The patient further improved clinically and was discharged on a clear liquid diet and oral antibiotics. Repeat scan three days later demonstrated an interval increase in size of the pseudoaneurysm (). He was asymptomatic, but due to the growth of the lesion, interventional radiology was consulted. During angiography, the celiac access was cannulated and the gastroduodenal artery was reached. Selective angiography demonstrated predominant inflow to the pseudoaneurysm from the inferior pancreaticoduodenal artery. Therefore, the superior mesenteric artery was cannulated showing a replaced right hepatic artery. This artery hindered access to the branch feeding the pseudoaneurysm, and on multiple attempts, the guidewire could not be advanced. There was no active hemorrhage, and the patient remained hemodynamically stable. He was transferred to a specialized interventional radiology facility. On a first attempt again, the inflow to the pseudoaneurysm could not be accessed; however, during a second attempt two days later, occlusion of the pseudoaneurysm inflow was obtained. The patient recovered without any complication and was well at his six months of follow-up. He had no further episodes of duodenal diverticulitis. Interval endoscopy confirmed the presence of a large duodenal diverticulum without inflammation. |
A 40-year-old man suffering from dilated cardiomyopathy had been prescribed amiodarone for 2.5 years. Seven weeks before the consultation at our department, his serum-free T4 levels increased above the upper limit and thyrotoxicosis developed. His thyroid status was as shown in . An attending cardiologist consulted at our thyroid clinic about the patient's thyrotoxicosis, but he had no complaints. He did not show any tachycardia or finger tremor, despite the thyrotoxicosis. His thyroid gland was not swollen and ultrasonic study revealed a slightly enlarged thyroid gland with almost monotonous echogenicity (). The Doppler flow rate inside the thyroid gland was not increased (). To differentiate the diagnosis of thyrotoxicosis, we planned to investigate thyroid iodine uptake. Ten days after the first visit, he showed symptoms of acute heart failure and was admitted to the intensive care unit of our hospital. His thyrotoxicosis had worsened by the time of admission, with increased levels of thyroglobulin, suggesting destructive thyroiditis (). Amiodarone administration was stopped and inorganic iodine administration (189 mg/day) was started upon admission; however, his thyrotoxicosis was prolonged and worsened. His cardiac function also worsened, with the thyrotoxicosis being exacerbated (). On admission, his heart rate was over 180 bpm and systolic blood pressure was 220 mmHg. Oxygen saturation rate was 70% under 10 L/min of oxygen administration with a venturi mask. Intra-arterial balloon pumping was performed to maintain the circulation. On the day after admission, administration of 200 mg of hydrocortisone was started, in addition to inorganic iodine. After the hydrocortisone administration, free T3 levels were somewhat improved, but free T4 levels remained high. To control and suppress the destruction of the thyroid, 40 mg of PSL was administered instead of hydrocortisone. Subsequently, 60 mg of PSL improved the serum-free T4 levels, so we tapered the dose of PSL gradually. However, at a dose of 20 mg of PSL, the thyrotoxicosis relapsed. At this point, TSH receptor antibody (TRAb) became positive (), so we decided to prescribe 15 mg of methimazole (MMI) together with 40 mg of PSL. Two days after these prescriptions, his free T4 levels increased to above the normal range. Thirty milligrams of MMI, 40 mg of PSL, and inorganic iodine (189 mg/day) did not suppress the destructive thyroiditis. On the 17th day of admission, thyroid 99mTc uptake was investigated, but none was observed (). At this point, we made a final diagnosis of type 2 amiodarone-induced thyrotoxicosis (AIT). On the 23rd day of admission, MMI was discontinued and the administration of 80 mg of PSL was maintained. Subsequently, we attempted to taper the dose of PSL, but under a dose of 80 mg of PSL, overt thyrotoxicosis was not controlled (). Since over 2.5 months had passed since a high dose of PSL had been administered, we decided to perform total thyroidectomy. The administration of 80 mg of PSL was continued until the operation. With informed consent from the patient and his wife, total thyroidectomy was performed on the 78th day of admission. Intravenous administration of 40 mg of PSL and 200 mg of hydrocortisone was performed during the operation. The operation was safely performed and 25.6 g of thyroid was resected. After the operation, PSL was discontinued and the dose of hydrocortisone was carefully tapered. Two days after the thyroidectomy, hydrocortisone was tapered to 100 mg and administered orally. Then, hydrocortisone was again gradually tapered to 15 mg eleven days after the surgery. Twenty-five days after the operation, hydrocortisone was tapered to 5 mg, and it was discontinued on the forty-sixth day after the thyroidectomy. During the tapering of hydrocortisone and after its discontinuation, the patient demonstrated no symptoms of adrenal insufficiency. Pathological findings of the excised thyroid gland are as shown in . Grossly, the lobes became firm in consistency but maintained their normal shape (). On microscopy, several sizes of follicles were regularly lined with flattened follicular epithelium. The lumen was filled with colloid. Scattered disrupted follicles with enlarged epithelium and cytoplasmic vacuoles were observed (). It is of note that macrophages had infiltrated and multinucleated giant cells were also found in the follicular lumen (). Immunostaining with anti-KP1 (CD68) and antithyroglobulin antibodies confirmed that the infiltrated cells were macrophages but not follicular cells (Figures and ). These findings characterized by scattered follicle disruption, vacuoles in epithelial cells, and macrophage infiltration are compatible with amiodarone toxicity [].\nAfter the operation, the patient's thyrotoxicosis rapidly disappeared and the thyroid function was normalized with 100 μg of levothyroxine (L-T4). Thirty-six days after the thyroidectomy, implantation of a left ventricular epicardial lead was performed under the administration of 5 mg of hydrocortisone. We administered 200 mg of hydrocortisone intravenously during the procedure and the implantation was performed safely. After the implantation, his cardiac function was dramatically improved. On the 130th day of admission, the administration of hydrocortisone was discontinued and he was discharged from the hospital on foot. |
In late 2016, a 61 year old non-smoking female presented with exertional shortness of breath, mild pedal edema, distended neck veins, and a recent weight gain of 15 lbs over the prior 2 weeks. It was initially suspected that the patient was experiencing pulmonary embolism based on her clinical presentation and elevated D-dimer, but further testing revealed that she was experiencing cardiac tamponade with mild pulmonary hypertension and heart failure. Pericardiocentesis was performed without definitive diagnosis. In February 2017, the patient exhibited worsening respiratory symptoms without fever, hemoptysis, sputum production, B symptoms, or extremity edema. Baseline chest PET-CT images were reviewed by radiologists at both UCLA Oncology and Texas Tech University Health Sciences Center, demonstrating a large middle mediastinal mass encasing the main pulmonary artery, with pericardial and left pleural effusion (Figure & Figure ). Scattered pulmonary nodules and hypodense lesions were identified in the right lobe of the liver, consistent with a diagnosis of metastatic disease (Figure ). Biopsy of the mediastinal mass revealed a high grade undifferentiated malignant neoplasm composed of highly proliferative (ki67 staining ~50%) pleomorphic anaplastic epithelioid malignant cells with large areas of necrosis and fibrosis. Immunohistochemistry revealed strong antigenicity for CD31 and CD34, and weak antigenicity for D2-40 and Factor VIII indicating a diagnosis of angiosarcoma that was corroborated at both UC San Diego Health and MD Anderson.\nConventional treatment options were recommended, however the patient declined these based on low reported survival rates, and instead, requested the non-selective beta blocker propranolol as a single agent therapy. In May 2017, 40 mg/kg propranolol was administered daily and PET-CT scans were performed at regular intervals to assess the response of the tumor to propranolol. Assessment of tumor response was based on 18F-fluorodeoxyglucose (FDG) tracer uptake and measurements/assessments of the primary tumor and distant metastases. After 12 months of propranolol as a single agent therapy, significant debulking and decreased size of the residual mediastinal mass was observed on PET-CT scans, with resolution of pericardial effusion (Figure & Figure ). Pulmonary nodules were stable to regressed, and the nodules in the right lobe of the liver had completely resolved (Figure ). There was no evidence of residual hyper-metabolic activity based on FDG measurements in the primary lesion or in metastatic sites in the chest, abdomen, or pelvis on PET-CT. |
A 55-year-old male who presented with new onset of abdominal pain to a peripheral hospital. He had a past history of ulcerative colitis, alcoholic pancreatitis, non-alcoholic steatohepatitis, COPD and GERD. His blood work demonstrated pancreatitis (lipase 1440), and jaundice (total bilirubin 98, direct bilirubin 73.9). A CT abdomen/pelvis was performed which showed a new duodenal mass with obstruction (A). In retrospect, the CT additionally demonstrated severe celiac artery stenosis likely from median arcuate ligament (B), which resulted in enlargement of the pancreaticodudenal arteries near the hematoma as a collateral pathway to fill the celiac artery in retrograde from the SMA (C). An esophagogastroduodenoscopy was completed which demonstrated a submucosal mass with yellow to pink hew. He became febrile on day 4 of admission and was placed on piperacillin-tazobactam. The patient was then transferred to a tertiary care center.\nOn arrival, the patient was afebrile with ongoing jaundice (Total bilirubin 80, direct bilirubin 51). An MR pancreas was performed which suggested that the mass in the duodenum was likely a hematoma.\nHe required drainage of his biliary tree and an ERCP was attempted for biliary decompression which was unsuccessful. The following morning, he was peritonitic with a rigid abdomen. A repeat CT abdomen and pelvis was performed which demonstrated free air and mild free fluid in keeping with bowel perforation presumably from the duodenum (D). The duodenal hematoma had enlarged into the 3rd part duodenum and duodenal cap. The common bile duct was enlarged and there was mild intrahepatic biliary dilatation-increased since previous exam.\nThe patient was taken to the operating room urgently as he was becoming increasingly hemodynamically unwell. He was found to have a completely necrotic duodenum from D1-D4 (A) with the lateral wall widely open and extruding bile (B). The difficult decision to perform an emergent damage-control pancreaticoduodenectomy was made given the extent of the necrosis and size of the defect in the lateral wall. The defect size was a critical factor in this decision as we felt there would be significant difficulty in successfully diverting gastrointestinal contents (ex pyloric exclusion) or making a controlled fistula. The pancreas had signs of chronic pancreatitis during dissection, which was later confirmed on pathology. The resection was performed and the patient was taken the intensive care unit with an open abdomen with planned reconstruction after resuscitation. This 24–48 h between ORs was intended to resuscitate and clinically optimize the patient given his hemodynamic instability in the OR and there was no concern for the perfusion of the residual bowel that was left at the end of the first case and we did not feel a second look laparotomy would be required solely for contamination.\nCareful examination of the excised specimen revealed multiple defects within the serosa overlying the duodenum (A, arrowhead). These defects measured between 3 to 5 cm in size, with extensive blood clot extruding from the suspected hematoma cavity (A, arrows). Once the specimen was opened, there were areas of dusky duodenal mucosa and focal necrosis, and there was a clear plane of serosal-mucosal dissection along the majority of the duodenum. This plane of dissection appeared to generate a large 16 cm hematoma cavity (B, h), anatomically distinct from the lumen of the duodenum (B, d). Microscopic examination of the affected areas demonstrated that the hematoma cavity originated from within the muscularis propria layer (A, mp), with areas of abrupt transition to acute ischemic enteritis (B and C, arrows). These were areas with ischemic epithelium and epithelial necrosis, with marked hemorrhage, congestion, and hyalinization of the lamina propria.\nMicroscopic examination of the proximal pancreatic head revealed several prominent cystically dilated pancreatic ducts (A, c) containing eosinophilic concretions commonly seen in chronic pancreatitis (B, arrowhead). Closer examination of these dilated ducts revealed an eroded and ulcerated wall, with a surrounding acute on chronic inflammatory process (B, arrows). The majority of the submitted pancreatic sections appeared healthy and intact, but there were patchy areas of acute pancreatitis demonstrating an intense neutrophilic infiltrate (C, arrow), fat necrosis and saponification (C, arrowheads). This histologic evidence was consistent with the clinical presentation of both acute and chronic pancreatitis.\nReconstruction was performed on postoperative day 2 in a standard reconstruction with a two-layer pancreatico-jejunostomy (PJ), a single-layer hepatico-jejunostomy and a handsewn double-layer gastrojejunostomy. A total pancreatectomy was not considered as the cut edge of the pancreas was healthy and, despite the higher risk of initial complications by performing a PJ, the benefit of residual pancreas in the long-term out weighted these risks in our opinion. On postoperative day 12 he experienced a fascial dehiscence and required a third operation to washout the abdomen and required placement of retention sutures. Subsequent to this last procedure, he developed an infection which grew E. coli, Raoultella, and mixed flora from the drain in his abdomen. He required IV imipenem and fluconazole. He proceeded to have a type II pancreatojejunostomy leak, which was managed medically with octreotide and TPN. He was repatriated to his home hospital on post-operative day 36 once weaned off of TPN. |
An 81-year-old man with Siewert type I esophagogastric junction cancer underwent resection of the distal esophagus and proximal stomach. After shaping the remnant stomach, esophagogastrostomy was performed in the mediastinum under right thoracotomy. The anastomosis was performed using a circular stapler and was wrapped around the stomach to prevent gastroesophageal reflux disease. On POD 11, he had high fever; a computed tomography scan revealed a mediastinal abscess. The mediastinal drain was already removed on POD 9. A contrast medium injected through the NGT showed the anastomotic leakage, which caused the abscess (Fig. a). We inserted the NGT into the abscess cavity through the anastomotic leakage site (Fig. b) and ensured that the inside of the drainage tube was under negative pressure for withdrawing abscess contents by using a low-pressure aspirator. Since the tube caused pain, the patient repeatedly tried to remove it by himself. We had to restrain his limbs, so that the main drainage tube would not be removed. On POD 25, we performed PTEG and inserted a drainage tube instead of an NGT. He was soon released, after PTEG. Even after the abscess cavity disappeared, we confirmed that the anastomotic leakage persisted as a fistula, by injecting a contrast medium under fluoroscopic guidance on POD32 (Fig. c). As enteral feeding was required to start as soon as possible, we exchanged the PTEG tube with a double elementary diet (W-ED) tube with jejunal extension with the side hole located near the anastomosis, on POD43 (Fig. d). We used the 16-Fr W-ED tube, supplied by Japan Covidien Corp., Tokyo, Japan. It measures 150 cm and includes connectors both for drainage and nutrition; one lumen has its openings alongside the tube, 60 cm above the leading edge. The other lumen has its openings at the end of the tube for feeding. On POD 50, the anastomotic fistula disappeared, as seen in the fluoroscopic examination. The patient’s dysphagia persisted, due to disuse atrophy of the swallowing musculature; PTEG was useful for enteral feeding, even after the leakage occurred. |
The present case report is about a 34-year-old gentleman who presented with a painful swelling behind the angle of left mandible of 5 months duration. He also complained of change in character of voice, dysphagia and weakness of left upper limb for 3 months prior to admission.\nOn neurologic examination, he had IX, X and XI cranial nerve palsy on the left side. There was a 3.5 cm × 3.0 cm bony hard swelling underneath the angle of left mandible leading to obliteration of post auricular groove. It was extremely tender. On examination of the oral cavity, the swelling was bulging into the tonsillar fossa and compressing the palatal arches.\nSince, the mass was palpable externally, fine-needle aspiration was attempted pre-operatively (twice) and on both occasions, but it was found to be inconclusive.\nThe axial and reformatted sagittal computed tomography showed a large expansile and destructive bony lesion involving the foramen magnum on the left side from lower clivus up to the axis. There were calcifications seen within the tumor. Thin sclerotic rim was visible around most of the periphery of the lesion [Figure –]. The lesion had involved the lateral masses and part of anterior and posterior elements of upper 2 cervical vertebrae.\nMagnetic resonance imaging showed a heterogenous mass (size approximately 5.3 cm × 6.2 cm × 5.5 cm) with strong post contrast enhancement. The lesion was displacing left internal carotid artery peripherally causing mild compression and had involved the left vertebral artery, the oblique part of V3 segment [Figure –].\nWith pre-operative impression of a high grade bony lesion, the patient was planned for surgical decompression of the mass through left sided far lateral approach. At surgery, near total excision of the tumor was achieved, deliberately leaving a thin rim of tumor tissue attached to posterior pharyngeal wall to prevent opening the oral cavity [Figure and ]. The tumor was very vascular and bled profusely. Occipito-cervical fusion was also done. The patient recovered uneventfully after surgery. Histopathological evaluation of the resected tissue showed epithelioid osteoblasts lining the bony trabeculae separated from the thin walled vessels by the osteoclastic giant cells. Mitotic figures were also seen. Hence, a pathological diagnosis of AO was made [Figure and ]. The patient was referred to radiotherapy for residual lesion. At 5 months follow-up, the patient was doing well. |
A thirty-three-year-old man noticed extension disturbance of the left fingers, 7 months prior to the initial evaluation in our institute. He began to feel tension and pain in the forearm when he extended his fingers. He visited a local hospital 1 month after onset. A swelling in his left forearm appeared and this worsened gradually. The symptoms did not resolve, and the patient was then referred to our institute with a diagnosis of Volkmann's contracture (Figure ). Plain radiographs showed irregularity of the surface of the ulna, which was compatible with periostitis (Figure ). Magnetic resonance imaging (MRI) demonstrated a lesion surrounding the flexor tendons in the flexor compartments of the forearm with iso-signal intensity to the surrounding muscle tissue on T1-weighted images and heterogeneous high-signal intensity on T2-weighted images. The lesion was enhanced by gadolinium on T1-weighted images (Figure ). Based on these clinical symptoms and images, the cause of the Volkmann's contracture was explained as being due to chronic inflammation caused by repeated stress in the forearm, based on the facts that he was a carpenter and he used his upper extremities often. This was despite the fact that he was right-handed.\nAt our institute, surgery was undertaken not only to release the contracture, but also to obtain a biopsy specimen to diagnose the cause of the contracture. The surgery findings showed that the tendons had adhered to each other with cicatricial-like tissue without any obvious mass lesion. Then, release of the adhered flexor tendons was undertaken. The cicatricial-like tissue was sampled for analysis, and histologically, it was found to be composed of rounded or polygonal epithelioid cells, arranged in sheets or a solid trabeculae pattern. Degeneration and necrosis were also observed (Figure ). The neoplastic cells had vesicular nuclei and prominent nucleoli, with characteristic eosinophilic glassy cytoplasm (Figure ). Immunohistochemically, the tumor cells were positive for an epithelial marker of EMA (epithelial membrane antigen) and cytokeratins (AE1/AE3, CAM5.2), but negative for S-100 protein, which is a Schwann-cell marker. These histological findings were typical of epithelioid sarcoma. However, epithelioid sarcoma needs to be differentiated from malignant soft-tissue tumors of epithelioid malignant peripheral nerve sheath tumor (MPNST) and malignant melanoma. Unlike epithelioid sarcoma, epithelioid MPNST tends to stain strongly for S-100 protein and virtually never expresses cytokeratins, whereas malignant melanoma virtually always expresses S-100 protein []. Some epithelioid sarcomas are also difficult to distinguish from epithelial tumor of ulcerating squamous cell carcinoma. However, epithelioid sarcoma lacks keratin pearls, as was true for the current case []. Taken together, a diagnosis of epithelioid sarcoma was made in the current case.\nCT showed no evidence of pulmonary metastasis, but it showed mild swelling of the axillary lymph nodes, which could have been possible metastasis, or simply non-specific swelling due to the biopsy procedure. Amputation above the elbow was undertaken. Three months after the amputation, bone scintigraphy showed no evidence of abnormal findings suggestive of metastasis to the bone (data not shown). However, CT showed increased size of the axillary lymph nodes, suggesting that these lymph nodes were actually metastasized (Figure ). For further examination, FDG-PET was undertaken, and it detected multiple lesions with an increased uptake in the right neck (SUVmax; 4.6 g/ml), right upper arm (SUVmax; 4.1 g/ml), left upper arm (SUVmax; 4.2 g/ml), right thigh (SUVmax; 5.5 g/ml) left thigh (SUVmax; 2.0 g/ml), back (SUVmax; 3.6 g/ml), and lower back (SUVmax; 4.6 g/ml), as well as the left axilla (SUVmax; 3.9 g/ml) (Figure ). As for the right neck lesion, ultrasonography and CT failed to detect swelling of the lymph node just after FDG-PET examination, although the swollen lymph lesion was confirmed physically 3 months later (data not shown). The right thigh lesion with an increased uptake on FDG-PET was not palpable, and had no tenderness on physical examination. However, MRI demonstrated a nodular metastatic lesion measuring 2 × 2.5 cm which was located within the thigh muscle with iso-signal intensity to the muscle tissue on T1 images and heterogeneous high-signal intensity on T2-weighted images. Gadolinium enhancement on T1-weighted images was seen in the lesion. The surrounding reactive lesions were seen mainly longitudinally (Figure ). The thigh lesion was still not palpable 3 months after the FDG-PET examination. |
A 56-year-old woman received a diagnosis of cancer of the right breast 6 years prior. At that time, she underwent right mastectomy with placement of a tissue expander. This was followed by exchange to 650 cc silicone implant for right breast reconstruction and concurrent left breast reduction using inverted-T incision with inferior pole technique to achieve symmetry. Unfortunately, she developed ductal carcinoma in situ with microinvasion in her left breast 4 years later. After left-sided vertical mastectomy, her first tissue expander failed secondary to infection and was removed. After debridement and resolution of the symptoms, a second tissue expander was placed and later exchanged with a 650 cc implant. Within 2 weeks, there was again evidence of infection and the implant was removed. Now, 6 years after being diagnosed with breast cancer, she had a successful right breast reconstruction and a failed left breast reconstruction (a).\nAt this point, we had essentially exhausted implant-based reconstruction techniques and offered alternative autologous tissue options for further repair. She elected to pursue several rounds of fat grafting instead of a pedicled flap or free tissue transfer. Next, the left breast was fit with a Brava dome external tissue expander device (Brava, LLC, Miami, Fla). This was worn only in the preoperative period for 6 weeks, 8 hours per day, in an effort to best prepare the recipient site to receive and support large volume of fat grafts. Over a 2-year period, she received 5 rounds of AFT using the TLL system. Fat was harvested from various sites including abdomen, flanks, posterior hips, thighs, saddlebags, bra line, and lateral chest wall. After 4 AFT procedures, 250, 300, 250, and 270 mL were transferred to the left breast, respectively. In the fifth and final round of AFT, 180 mL of fat was grafted to her left breast and 120 mL to her right breast for symmetry. In total, she received 1250 mL of fat to her left breast over 2 years, with 5 procedures to adequately reconstruct the defect. She experienced minimal fat necrosis in the recipient site and no complications in any of the donor sites. b shows 1 year after her final round of AFT. Now, 2 years after her final round of AFT, the volume of her left breast is equivalent if not slightly greater than her right breast, which was reconstructed with a 650 cc implant (c). |
A 32 year old female Cameroonian gravida 4 para 3 at 34 weeks of gestation presented to the labour and delivery unit of Mbalmayo district hospital with 8 h history of severe generalized headache, expressive aphasia and right sided paralysis in an afebrile context. This was associated with blurred vision but no convulsions. There was no epigastric pain and no difficulty breathing and no history of trauma or fall. For this current pregnancy, antenatal care (ANC) was started at 18 weeks with a booking blood pressure of 100/70 mmHg. She did four ANCs and all were uneventful. During her routine four ANCs here blood pressure was always less than 140/90 mmHg and her urine dipsticks done during the four ANCs were all negative for proteinuria. She refused neurological symptoms such as headache during pregnancy. She has a history of gestational hypertension in her third pregnancy. There was no family history of chronic hypertension, diabetes and chronic kidney diseases. On examination she was afebrile with a blood pressure of 182/126 mmHg and pulse of 112beats/minute. Neurological examination revealed Glasgow coma score of 13/15, right sided hemiparesis and expressive Broca’s aphasia, no signs of meningeal irritation. The abdomen was distended by a gravid uterus with a fundal height of 35 cm, foetus in a longitudinal lie and cephalic presentation. The cervix was long, posterior, soft and closed with a station of − 1. We had a working diagnosis of severe pre-eclampsia complicated by stroke. Shown on Table are laboratory investigations done and their results.\nAn emergency obstetric ultrasound showed a life foetus with an estimated foetal weight of 2300 g at 33 weeks of gestation. Emergency cerebral non contrast-CT scan showed a 3.2 cm hyperdense region in the left parietal lobe with surrounding hypodensity due to clot retraction as shown on Fig. . Emergency management by the obstetrician consisted of MgSO4 using the Pritchard protocol [], which consisted of 14 g loading dose then 5 g maintenance every 6 h until 24 h after caeserean section; bethamethasone 12 mg intramuscular and reduction of blood pressure with nicardipine 5 mg/h. Four hours later an emergency caesarean section was done by the obstetrician under spinal anaesthesia and it let to the extraction of a life female with APGAR 8 and 10 at the 1st and 5th minute respectively and weight 2200 g. The management after caesarean section consisted of hospitalization in the intensive care unit with nicardipine titrated in an electric syringe at 2.5 mg/hour, ceftriaxone 2 g intravenous, Paractamol 1 g 8 hourly, and ringers lactate 6 hourly for 24 h. Post-operative management was done by a multidisciplinary team including a neurologist, cardiologist, intensive care physician, obstetrician, neonatologist and physiotherapist. On postoperative day 2 she was transferred from the intensive care unit to the maternity where she spends five additional days on nicardipine slow release 50 mg 12 hourly and paracetamol 1 g 8hourly and was later release after the ten days on nicardipine 50 mg daily and daily physiotherapy. Six weeks during routine postpartum visit the blood pressure was normal and patient was no longer aphasic and shet has regained the muscle strength partially. The baby was hospitalised in the neonatal unit for 10 days and discharged alongside the mother. |
A 73-year-old Japanese man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. He had high blood pressure, and he started continuous ambulatory peritoneal dialysis (CAPD) because of hypertensive nephrosclerosis 8 years previously. A PD catheter was primarily inserted at the right abdomen, but it was removed and inserted at the left abdomen because of exit site and tunnel infection 5 years previously. He had no past medical history of diabetes mellitus and major abdominal surgery. In the peritoneal equilibration test, his result was high. Bloody ascites was not evident. One year previously, he had been hospitalized for PD-associated peritonitis caused by touch contamination that was treated with intraperitoneal cephazoline and cephtazidime. Bowel adhesion was not noted 5 years previously; however, local bowel adhesions and agglomeration of the intestine were detected by computed tomography (CT) after the identification of PD-associated peritonitis (Fig. , ). The major findings of EPS, such as peritoneal thickening and calcification, were not noted on CT.\nOn physical examination, his blood pressure was 134/74 mmHg, pulse rate was 76 beats/min, and temperature was 99.7 ° F. He complained of severe pain in the right upper quadrant of the abdomen, and this area was tender on palpation. The exit site was clear. Laboratory tests revealed mild inflammation, with a white blood cell count of 10,100 /μL and C-reactive protein level of 0.9 mg/dL. The peritoneal fluid cell count was increased at 980 /mL. Based on these findings, PD-associated peritonitis was diagnosed. CT showed localized dilation of the intestine, which suggested adhesive small bowel obstruction (Fig. ). As we suspected that the peritonitis might be associated with bacterial translocation from the dilated intestine, he was advised to stop eating and was switched from CAPD to hemodialysis. Additionally, he was treated with intravenous vancomycin and cephtazidime. The PD catheter was flushed once a day to prevent catheter obstruction with fibrin, and the characteristics of the peritoneal fluid were monitored. His abdominal pain was resolved and peritoneal fluid cell count decreased to < 30/mL, and thus, he resumed oral intake on day 8.\nAfter resumption of oral intake, his abdominal pain worsened and his peritoneal fluid cell count dramatically increased to 9600/mL on day 15. The peritoneal fluid became cloudy with a high amount of fibrin and white blood cells (Fig. ). Although he stopped eating again, his abdominal pain did not improve, and fecal material with foul smell was identified from the PD catheter on day 23 (Fig. ). Culture of peritoneal dialysate on admission was negative; however, culture of peritoneal dialysate on hospital day 23 was positive for Enterococcus faecalis and Bacteroides caccae. On CT, the intestinal contents disappeared and the dilated intestine collapsed, indicating that the intestinal contents had leaked into the abdominal cavity (Fig. ). Considering these facts, intestinal perforation was diagnosed, and he underwent ileocecal resection with colostomy creation. Although intra-abdominal adhesion was severe, fibrinous encapsulation of the bowel, which would suggest EPS, was not detected macroscopically during surgery (Fig. ). As indicators of EPS were not evident, the PD catheter was removed. The perforation site was located at the adhesive intestine. The tip of the peritoneal catheter was located in Douglas’ pouch, and it did not injure the adhesive intestine. Pathological examination of the resected specimen revealed inflammatory cells associatet with the peritonitis in the intestinal wall. Intestinal fibrosis, arterial alteration, and tissue calcification were not evident pathologically (Fig. , ). Although his serum beta-2 microglobulin (B2M) level was high (41.05 mg/L), amyloidosis and deposition of B2M were not observed (Fig. -). The postoperative course was uneventful and left arteriovenous fistula surgery was performed on day 42. Since then, he has been on maintenance hemodialysis with no recurrence of peritonitis. |
The 8-month-old boy was born at term without any unusual birth history (38 weeks, 3,150 g, by Cesarean delivery) to a 45-year-old father and 36-year-old mother. He had one brother (12-year-old) and sister (8-year-old). None of the family members had any medical history during the growth period.\nHe was admitted to the pediatric department due to an initial seizure event following aspiration pneumonia and was referred to our clinic for the evaluation of unexplained neuroregression. Although he was hypotonic from birth, he achieved a social smile at 3 months and started head control during the first 4 months. He rolled over, and nearly grasped his toys with prone position at 6 months. Generalized tonic–clonic type seizures at 6 months were his first clinical symptom, a detailed history revealed delays in developmental milestones after that. Electroencephalogram (EEG) findings showed abnormal awake and sleep recordings due to slow background activity, suggestive of diffuse cerebral dysfunction with symptomatic or cryptogenic seizures. Magnetic resonance imaging showed cerebral hypoplasia especially in the frontal and temporal lobes at approximately 4 years of age. He was observed at the outpatient clinic for developmental delays associated with encephalopathy and seizure events, which occurred hundreds of times for 2 years and were fairly well-controlled with valproic acid, phenobarbital, and clonazepam.\nAt 26 months after surgery for bilateral cryptorchidism, progressive respiratory difficulty persisted and weaning from the ventilator was not possible; repetitive aspiration pneumonia occurred as he was unable to proceed with sputum expectoration. Therefore, tracheostomy was performed and night-time breathing using a ventilator was maintained subsequently. At the time of admission, repetitive hand flipping without purpose and lip smacking was observed during examination, although epileptiform discharges were not observed during EEG, we decided to proceed with additional evaluation other than that previously considered at this point. The various clinical features of the patient are described in Table .\nThere were no abnormal findings based on laboratory investigation, and genetic analysis of mutations including Prader-Willi gene, spinal muscular atrophy gene, and other chromosomal aberrations. Chromosome analysis revealed a 46, XY karyotype. A muscle biopsy also demonstrated no abnormal findings. |
A 25-year-old woman with a palpable lump in her right breast was referred to our department. She had been diagnosed with NF1 at the age of 17 years. Two of her aunts had cancer; one had breast cancer and the other had ovarian cancer. However, there was no family history of neurofibromatosis.\nOn US, an ill-defined hypoechoic mass with microcalcifications and irregular duct changes, extending to the subareolar area, was noted in her right breast. Additionally, several lesions believed to be metastatic lymph nodes were observed in the ipsilateral axilla (Figures and ). On mammography (MMG), fine pleomorphic microcalcifications with segmental distribution were noted in the lower outer portion of her right breast (). On MRI, the lesion showed about 6.5 cm sized, nonmass enhancement lesion with heterogeneous internal enhancement pattern and occupied most of the right breast, except the lower inner portion ().\nShe underwent US-guided core needle biopsy in the lower outer portion of her right breast and of the pathological lymph nodes in the right axilla. She was diagnosed with ductal carcinoma in situ in the breast and metastatic lymphadenopathy in the right axilla. She underwent modified radical mastectomy and axillary lymph node dissection, and the final diagnosis was invasive ductal carcinoma with axillary metastasis (T2N3M0; estrogen receptor positive; progesterone receptor positive; human epidermal growth factor receptor 2 negative; Ki-67 10–20%). We analyzed DNA from peripheral blood in order to evaluate the presence of mutations in the BRCA1 and BRCA2 genes. Specific coding regions and exon-intron boundaries of the BRCA1 and BRCA2 genes were amplified using polymerase chain reaction (PCR). Sequence alterations were confirmed at the genomic level with PCR amplification, and no mutation was noted in the BRCA1 or BRCA2 gene. She received postoperative chemotherapy and radiation therapy. Presently, she is being regularly followed up, and she has not shown any signs of disease recurrence. |
A 62-year-old male patient came to our observation with an initial diagnosis of clinically silent long-term inguinal hernia. His medical history showed intermittent fever and anorexia had been present for one year. In detail, the patient reported that over the previous year he had had alternating intermittent fever up to 39-40°C of about 2 weeks with quiescence periods of about 20–30 days, every few months together with an overall weight loss of about 12 Kg. These symptoms were not associated with any other abdominal or thoracic signs. Physical examination of the abdomen was negative. The fever disappeared after an empiric antibiotic therapy with third generation cephalosporins (1 gr. for 12 days) was started. Laboratory blood samples obtained when the patient was febrile, showed 12,000 WBC count; liver function tests were in the normal range and serologic screening for Salmonella and Brucella antibodies were negative. Blood cultures, antibiograms and tumor markers were also negative (CEA, CA 19.9).\nAn ultrasound scan of the liver and an abdominal CT scan (Figure A) detected a disomogeneous hypoechoic-hypodense mass with many internal hyperechoic/hyperdense stones in the 5th segment of the liver. In addition, a cholelithiasis with two small stones in the common bile duct was present. Differential diagnosis between hepatic abscess or gallbladder cancer remained open. An abdominal MRI scan confirmed the above mentioned findings with the evidence of a few bubbles of free air suggesting spontaneous perforation of the gallbladder (B). An ERCP was then performed and the choledocolithiasis was resolved through a papillotomy. Afterwards a surgical exploration was planned. The operation started with a laparoscopic approach to confirm the suspected diagnosis and exclude tumor diagnosis and even more important peritoneal tumor spread that would be a contraindication to radical R0-surgical resection. Laparoscopy showed a bulging lesion involving the gallbladder, liver, great omentum and duodenum, (Figure A) while no signs of peritoneal carcinomatosis were detected. We then decided to convert to open procedure due to uncertain anatomical limits of the lesion in front of the duodenum and the liver hilar structures. After resection of the omental adhesions and the spearing of the duodenum, the bulging was resected en-bloc with the gallbladder (Figures B-) and gallbladder bed performing an atypical resection of the 5th hepatic segment. Opening the large abscess we found more than 20 biliary stones that had migrated intra-hepatically. The phlogistic nature of the tumor was histologically confirmed by three intra-operative sections (later frozen) and on the definitive histologic examination (Figure B, C). The post–operative course was uneventful and the patient was discharged on the 4th post-operative day completely afebrile and in good general condition.\nIntra-hepatic gallbladder perforation is generally considered to be a very rare evolution of cholelithiasis, in fact, to date, performing a Pubmed search using as key words: “Intrahepatic abscess, intrahepatic gallbladder perforations, Neimeir’s type I perforation, chronic gallbladder perforation” and then using “related articles” and “see reviews” functions of the database, 20 articles were selected but after reading them and avoiding the well-known confusion [] and mistakes of reporting Neimeir’s classification [,], only 18 cases of chronic gallbladder perforation with formation of intrahepatic abscess were found to be reported in the literature [,]; therefore following the original classification they should be considered as Niemeier type I perforations (chronic perforation with fistula) []. Despite chronic processes with fistula formation, its clinical presentation, even in its rarity, is more often acute (nausea, vomiting, upper quadrant pain, fever, altered mental status, and septic shock) [,], or the consequence of an acute perforation []. The diagnosis is usually made by US or CT scan. In our case CT scan was not able to exclude a tumor in the 5th hepatic segment. The lack of acute abdomen symptoms characterized our case and this clinical presentation is to be considered exceptional, while usually such a perforation leads to an emergency operation []. This subtle clinical picture can be misleading for the physician causing a delay in obtaining prompt imaging tests. Some reports have highlighted the difficult differential diagnosis between gallbladder cancer versus liver abscess [,,]. Because of the very few reports to date, there is no consensus on the standard treatment of such a rare condition [,]. Simple puncture and drainage in these cases seems to not be an effective option []. An endoscopic examination could be an option, even if in this case laparoscopic cholecystectomy could not be performed due to technical reasons (many adhesions, not clear anatomy, high risk of damage to hilar structures). Some Authors question whether this rare condition is more common in patients with an intrahepatic gallbladder []. Therefore chronic liver abscess due to gallbladder perforation is a rare evolution of cholelithiasis. |
Case 1: Ms. K, a 70-year-old woman who immigrated to the US at the age of 53, began complaining of watery eyes, chest pain, lower back and joint pain, leg cramps, and weakness. She harbored delusions of being afflicted with high blood pressure, uterine cancer, blood cancer with bone metastasis, brain cancer with extensive metastasis, and believed that her brain was "shrinking."\nShe first visited a cardiologist in 2013, complaining of intermittent episodes of chest pain over six months. An electrocardiogram (EKG) at the time showed bradycardia, a first-degree atrioventricular (AV) block and a left bundle branch block. At her sixth visit with the cardiologist, she mentioned non-specific somatic complaints, which she said were because of a "hematological problem." Five months later, she was evaluated for “renal hypertension” and imaging studies showed a renal cyst. While she did not follow up with the nephrologist, she continued to make hospital visits for persistent chest pain. A full medical workup was completed and found to be normal at every ER visit. Medical records from a prior ER visit revealed that she had made claims that the Russian military entered her residence and stole her urine, resulting in the disappearance of her kidneys.\nMs. K was brought to the ER by the police after she showed up with a can of gasoline and matches at her primary doctor's office and threatened to burn it down. She was irate and claimed that all of her doctors, in the US and in her home country, were concealing the fact that she had oncological issues. She vehemently denied any psychiatric illness, stating that these diagnoses appeared on her records as a result of a rumor started by an envious former colleague. She explained that because she had been a former practicing neurologist in her home country, she was confident that she had cancer. Upon repeated questioning, she admitted that in a final bid to receive the medical attention that she was rightfully due, she had devised the plan to burn down the doctor’s office.\nWhile in the psychiatric inpatient unit, she remained somatically preoccupied and reported abdominal pain, lower back pain, and weakness, which she attributed to the metastatic spread of uterine cancer to her spine. Radiological imaging confirmed no evidence of uterine cancer, though a thickened endometrium was reported with recommendations for further testing by tissue sampling. Because Ms. K’s ability to make rational and reasonable decisions about her psychiatric and medical treatment was compromised by her delusions, the team sought and was granted a court order allowing them to treat her over her objection. |
A 34-year-old female with no significant past medical history presented to our clinic after experiencing a left second metatarsal stress fracture (Figure ). One year prior, while running errands around town, she suddenly felt a sharp pain in her left midfoot and promptly consulted an orthopedic surgeon who placed her in a boot. Six months later, after experiencing minimal improvement in her pain, a different orthopedic surgeon performed an open reduction and internal fixation by injecting 1 mL of bone cement into the diaphysis of the second metatarsal.\nOver the next six months, she noticed no meaningful improvement in her pain. At this point, she presented to our clinic for a third opinion. During our initial visit with her, she stated that her left foot felt different than her right at baseline.\nOn physical exam, there was no gross deformity of her left lower extremity. The skin was intact with a healed incision over the dorsal midfoot, and there was point tenderness to palpation over the second metatarsal. Active and passive range of motion of the ankle and transverse tarsal joint was full and painless. Strength was 5/5 in dorsiflexion, plantarflexion, inversion, and eversion. Sensation to light touch was intact, Achilles reflex was present, and dorsalis pedis and posterior tibialis pulses were palpable.\nLaboratory work revealed an elevated erythrocyte sedimentation rate of 36 (reference range: 0–20) and C-reactive protein of 34.74 (reference range: 0–10.9). Plain radiographs and a computed tomography (CT) scan of the left foot showed diffuse sclerotic changes and cement within the left second metatarsal (Figures , ). Magnetic resonance imaging (MRI) showed diffuse edema of the left second metatarsal with a non-displaced fracture line (Figure ).\nAll treatment options were discussed with the patient and she agreed with undergoing operative fixation. In the operating room, cultures and a bone biopsy of the left second metatarsal were taken. After performing an osteotomy, curettage was performed to remove the injected cement. Open reduction and internal fixation was performed utilizing a plate and calcaneal bone graft (Figure ). The patient was discharged home on the same day with adequate pain control and a bone stimulator. X-rays taken at two weeks post-revision surgery are shown in Figure .\nAt one-month follow-up, her incision was healing well without signs of infection and she had no complaints of pain. At her most recent appointment—three months post-revision surgery—she again reported no pain and good functional recovery with physical therapy. CT scan at three months post-revision surgery showed appropriate alignment of the healing second metatarsal with intact hardware (Figure ). |
A 69-year-old man was admitted in August 2005, following a six hour history of suprapubic pain, fever and rigors. He had a long term catheter for a prostate problem and his past history included a myocardial infarction in 2001, followed by a series of small strokes. As a result of these, he had a mild residual left hemiparesis and had received a stent to his right carotid artery. On examination he was pyrexial (39.2°C) with otherwise normal vital signs and no new findings. Investigations showed a low white cell count (2.5 × 109/litre) and raised inflammatory markers, with an ESR of 84 mm/hr and C-reactive protein of 176 mg/l. Chest X- ray revealed no abnormalities of clinical significance.\nCultures of blood and urine grew E. coli resistant to all cephalosporins and fluoroquinolones but susceptible to meropenem. A diagnosis of septicaemia originating from a urinary tract infection was made. Intravenous meropenem 1 gm three times a day was commenced. However the patient continued to have raised inflammatory markers with intermittent pyrexia and meropenem was stopped after 14 days. Three sets of blood cultures taken 7 days later all grew E. coli with the same antibiogram as found previously. Meropenem was recommenced at the same dose.\nA computed tomography (CT) scan was requested to explore the possibility of lymphoma or a deep abscess. This showed a small abdominal aortic aneurysm and a mild dilatation of the left subclavian artery (Figure ). A further scan was performed 2 weeks later to exclude leakage from the abdominal aneurysm. Whilst this possibility was discounted, two new intra-thoracic pseudo-aneurysms and associated haematomas were found; one was arising at the anterior aspect of the origin of the left subclavian artery and the second in the descending thoracic aorta (Figure ).\nThe patient was transferred to a cardiothoracic surgery centre but intervention was not advised due to his vascular co-morbidities. Instead he was returned to our care for conservative management and continuation of his antibiotics. His fever and inflammatory markers gradually settled to normal. A third scan (Figure ) was performed after 4 weeks of consistent control over the infection. The rationale was to seek any resolution of the mycotic aneurysm as a prelude to withdrawing antibiotics. As can be seen from these final images, considerable resolution of the pseudo-aneurysms and haematomas had occurred. After a further check that blood cultures and inflammatory markers had normalised, intravenous meropenem was stopped after a total of 16 weeks. No further pyrexia occurred, and the patient remained well 3 months later. |
A 61-year-old Caucasian man with a past medical history of hyperlipidemia, obesity (BMI 37 kg/m2), and osteoarthritis underwent a left knee arthroplasty (LKA) without complication. After undergoing surgery, he received DVT prophylaxis with two days of low molecular weight heparin while hospitalized and was then discharged home on 81 mg of aspirin daily. Prior to surgery, the patient led an active lifestyle, including regular elliptical use and golfing. Following his LKA he attended all physical therapy sessions. One month following surgery, he abruptly became lightheaded and dyspneic, with no relief of symptoms upon rest. He presented to his local emergency department where he was noted to have sinus tachycardia and hypoxia. CT pulmonary angiogram revealed a large saddle pulmonary embolism with an extension of the clot into multiple lobar and segmental branches (Figures -).\nCardiac enzymes were normal and EKG showed no ischemic changes. NT-pro-BNP was elevated at 154 pg/mL. Transthoracic echocardiogram showed moderately increased right ventricular size with moderately depressed right ventricular systolic function, and mild pulmonary hypertension, consistent with the diagnosis of submassive pulmonary embolism. He received high-flow nasal cannula and heparin and was admitted to the ICU.\nDuring his hospital stay, he also had a Doppler ultrasound of the lower extremities revealing a non-occlusive deep venous thrombosis (DVT) in the right popliteal and peroneal veins and a head CT due to dizziness which showed no acute process. He remained hemodynamically stable and supplemental oxygen was weaned. Intravenous anticoagulation therapy with heparin was transitioned to apixaban. He was discharged home on day 6.\nThe patient presented to our center’s chronic thromboembolic pulmonary hypertension (CTEPH) clinic approximately three and a half months after his submassive pulmonary embolism because of persistent symptoms of postural dizziness and chest pain. He also reported paraesthesias along the back of his head, legs, and feet. His ongoing symptoms were limiting his participation in these activities, and he was unable to return to work as a hospital food service director.\nHe had no history of prior pulmonary emboli or deep venous thrombi and no known risk factors for VTE, other than recent surgery. Although knee arthroplasty is a known risk factor for VTE, the patient received anticoagulant prophylaxis and remained active post-operatively engaging in frequent physical therapy sessions. Furthermore, the patient developed a DVT in the contralateral leg, making it unlikely that recent orthopedic surgery alone accounted for his significant VTE burden. His risk factors for pulmonary hypertension (all types) included obesity (a risk factor for heart failure with preserved ejection fraction), recreational methamphetamine use 40 years prior to presentation, and anorexigen use for six months 20 years prior to presentation. Surgical history was notable for left knee and hip replacements. His family history was notable for one brother who had a pulmonary embolism. He denied current tobacco or drug use and drank one glass of wine per day. The physical exam at this time was normal.\nHis chronic thromboembolic pulmonary disease evaluation included blood work (largely unremarkable - including negative anticardiolipin and beta-2-glycoprotein antibodies), imaging and pulmonary and hemodynamic testing. Ventilation-perfusion scan demonstrated no defects, though CT pulmonary angiogram showed chronic thromboemboli in multiple lobar arteries. Transthoracic echo was unremarkable, including normal right ventricular size and function. Pulmonary function tests and a six-minute walk test were also normal.\nHe underwent an invasive cardiopulmonary exercise test (iCPET) to understand the hemodynamic significance of his chronic thromboemboli. Baseline/resting right heart catheterization revealed mild post-capillary pulmonary hypertension (mean pulmonary artery pressure of 29 mmHg, pulmonary capillary wedge pressure of 18 mmHg) with preserved cardiac index (2.99 L/min/m2). With exercise, he developed a pattern consistent with preload failure caused by inadequate cardiac venous return that limits the normal stroke volume increase during activities []. To investigate the preload failure noted on iCPET, he was tested for adrenal insufficiency and small fiber neuropathy. ACTH stimulation testing was normal. Skin biopsy showed evidence of distal small fiber sensory neuropathy.\nHe was then referred for evaluation by our neuro-autonomic dysfunction center. The patient reported no change in symptoms. He remained active and continued exercising using a recumbent bike several times weekly. Neurological examination showed intact sensation to light touch, pinprick, and temperature, normal position sense, normal finger vibratory sense, but markedly reduced toe vibratory sense. Deep tendon reflexes were 2/4 biceps, 1/4 brachioradialis, 1/4 patellar, and 0/4 achilles bilaterally. Toes were silent bilaterally. No coordination deficits were seen. The patient had a sensory ataxic gait, and could not tandem. Romberg sign was present. He had normal muscular strength throughout and no cranial nerve deficits. History, exam, and skin biopsy results were concerning for large and small-fiber sensory neuropathy with autonomic dysfunction. The patient underwent further laboratory screening including erythrocyte sedimentation rate, an autoimmune panel, vitamin B12 level, serum and urine protein electrophoresis, serum-free light chain assay, ganglionic acetylcholine receptor antibodies, and onconeuronal antibodies; which were all unrevealing. QSART and tilt table tests were normal. However, autonomic reflex battery revealed reduced heart rate variability and a low expiratory to inspiratory (E:I) ratio consistent with cardiovagal dysfunction.\nElectromyography and lumbar puncture could not be safely performed due to the patient’s need for continuous anticoagulation. A right sural nerve biopsy was performed to further investigate the etiology of the sensory neuropathy. It showed perivascular chronic inflammation with non-necrotizing vasculitis (Figure ) and mild loss of myelinated axons (Figure ), consistent with CIDP. The patient received intravenous immunoglobulin (IVIG) with resulting in improvement in his sensory ataxic gait and a reduction in his autonomic symptoms. |
A 74-year-old male patient presented to the First Affiliated Hospital of Yangtze University (Jingzhou, China) with a painless mass in the anterior region of the upper neck that had been growing slowly for more than five years; the patient noted that the expansive mass had exhibited a progressive volume increase within the last six months. The patient also complained that swallowing food and pronunciation had been affected by the mass. The patient had no history of trauma, fever or weight loss. Clinical examination revealed a firm and tender mass (diameter, ~8 cm) in the root of the tongue.\nComputed tomography (CT) of the neck revealed a hypodense lesion located in the anterior cervical region of the neck, on the hyoid bone within the hyoglossus muscle. The CT value of the tumor was ~20.3 HU (Hounsfield unit). The oropharyngeal cavity became narrow as a result of tumor pressure () and the diameter of the mass was ~80 mm (). The imaging diagnosis characterized the mass as a cystic space-occupying lesion or lipoma.\nSurgical resection was accomplished through a horizontal incision on the hyoid bone over the tumor site, under general anesthetic. The tumor was easily separated from the normal muscle margins of the root of the tongue and the mucous membrane of the root of the tongue, which was adhered to the tumor was resected.\nThe tumor was encapsulated intact and macroscopically, it exhibited a gray-white appearance and the cut surface of the specimen was ovoid, yellow and gelatinous (). Histopathological analysis revealed a hypocellular neoplasm of low vascularity, composed of small spindle-shaped cells, stellate cells and fibers in abundant myxoid stroma. Mitotic activity, necrosis and nuclear atipias were absent. The fibrous pseudo-encapsulation of the tumor was identified and there was no infiltration of the fascicles of the adjacent skeletal muscle. The neoplastic cells were negative for S-100 protein expression. The pathological diagnosis of the mass was an IM (). The patient experienced an excellent recovery following surgery and was able to swallow food and speak without pronunciation issues. The follow-up at three years demonstrated no evidence of local recurrence. |
A 77-year-old man attended our services with exertional dyspnoea secondary to aortic valve stenosis. He received an orthotopic heart transplantation (HTx) in 1994 for idiopathic dilated cardiomyopathy (DCM). Unfortunately, we have no records of the patient's transplant operative data given the fact that his procedure was done 23 years ago. He remained asymptomatic during follow-up except for paroxysmal atrial flutter for which he received a single chamber pacemaker in 2008 and later, atrial flutter ablation in 2010. Patient was adherent to his medication regimen and did not show any signs of transplant rejection on several cardiac biopsies. His post-transplant cardiovascular risk factors included systemic hypertension, dyslipidaemia, and stable stage 4 chronic renal dysfunction (eGFR 23 mL/min/1.73 m2). Serial transthoracic echocardiography (TTE) performed in our institution showed progressive degenerative aortic valve disease.\nAt presentation, his TTE showed degenerative bicuspid aortic valve with fusion of the right and left coronary cusps by an incomplete raphe. The appearance of the valve was consistent with severe aortic stenosis which was confirmed by hemodynamic Doppler assessment that revealed a peak gradient of 65 mm Hg, aortic valve area of 0.9 cm2 derived from the continuity equation and a dimensionless velocity index (DVI) of 0.24. Left ventricular function was normal with an ejection fraction (EF) of 59% by Simpson's method. Further evaluation of the aortic valve and aorto-iliac anatomy was pursued by a Multi-detector computed tomography (MDCT). It confirmed the morphology of a heavily calcific BAV, the absence of associated aortopathy, and suitability for transfemoral approach. The maximal aortic annulus dimension was measured as 25 mm with an aortic root diameter of 32 mm at the level of the sinuses of Valsalva. Coronary angiography was performed to screen for cardiac allograft vasculopathy (CAV) which did not show any evidence of obstructive coronary disease.\nIn addition, he was noted on admission to be bradycardic with episodes of second-degree mobitz type 2 atrio-ventricular (AV) heart block. Electrophysiology service was consulted and decided the need to upgrade his pacemaker to a dual-chamber system following the TAVI procedure.\nHis case was discussed at the Heart Valve Team meeting with a consensus that TAVI would be the optimal intervention strategy being a high-risk surgical candidate with a Society of Thoracic Surgery (STS) predicted risk of 30 days mortality of 7.035%.\nThe TAVI procedure was performed according to the standard local TAVI protocol. Vascular access was obtained with ultrasound guidance under local anesthesia and conscious sedation. Heparin (6000 units) was given intraoperatively to achieve an activated clotting time (ACT) greater than 250 seconds. A balloon expandable 29 mm Edwards Sapien 3 transcatheter heart valve (Edwards Lifesciences, Irvine, CA, USA) was advanced via the right femoral artery through the calcified, transplanted native aortic valve without prior balloon aortic valvuloplasty. Final positioning was confirmed by fluoroscopic guidance. Under rapid ventricular pacing, by temporary pacing wire via the left femoral vein, expansion of the prosthesis over the stenotic valve was accomplished with excellent results and no immediate complications. The total amount of contrast used was 60 mL and subsequent renal function tests were stable. His pacemaker was electively upgraded to a dual-chamber system the following day as planned earlier due to pre-existing high degree heart block. Pre-discharge TTE revealed a well-positioned aortic valve prosthesis with a peak and mean trans-prosthesis gradients of 14 mm Hg and 12 mm Hg respectively. There was no evidence of valvular or paravalvular regurgitation on color flow Doppler and the LV systolic function remained normal.\nPatient showed immediate symptomatic and hemodynamic improvement and was discharged from hospital 48 hours post index procedure. He was maintained on his regular medication including the immunosuppressive therapy. At the routine 1-month clinic follow-up the patient was doing well and did not report any symptoms with no limitation of his physical activity (NYHA 1). |
A young married female aged 30 years presented to the Medical Emergency Department of our institute with chief complaints of decreased appetite, gradually progressive shortness of breath on exertion, and swelling all over body for 6 months. For the past 15 days, she had been complaining of dyspnea even on mild exertion and progressive abdominal distension. There was no history of fever, exertional chest pain, diabetes, hypertension, or jaundice. The family history was against any inherited causes of DCM. There was no history of any drug intake or alcohol or toxic substance addiction. Her obstetric history was noncontributory with three uneventful pregnancies and normal vaginal deliveries. The last delivery was 2.5 years back.\nOn examination, the patient was tachypneic, normotensive, and afebrile. The body mass index was 24.8 kg/m2. She had a hoarse voice which had changed over the past 2 years and had bilateral pitting pedal edema. The neck veins were engorged, and there were bilateral basal crepitations in the chest. The cardiovascular system examination revealed that apex beat was hyperdynamic in character and was localized in the 6th intercostal space outside the midclavicular line. The first and second heart sounds were normal; left ventricular (LV) third heart sound (S3) was present. There was no murmur or pericardial rub. There was no sign of nutritional deficiencies such as glossitis, cheilitis, limitation of ocular movements, memory impairment, or muscle weakness.\nDuring the course of investigations, hemogram, fasting and postprandial blood glucose, kidney function tests, electrolytes, and liver function tests including serum proteins were found to be within normal limits. Chest X-ray revealed gross cardiomegaly and electrocardiogram showed left bundle branch block. Ultrasound abdomen revealed mild ascites and minimal bilateral pleural effusion. Echocardiography was done and it revealed DCM with severe global LV hypokinesia and ejection fraction 15% along with a minimal pericardial effusion and Grade II LV diastolic dysfunction. Thus, a diagnosis of DCM with heart failure was made, standard treatment for the same was started, and further investigations were carried out to focus on the etiology.\nConsidering the possibility of autoimmune pathology, antinuclear antibody testing by immunofluorescence was done which was not significant. Negative human immunodeficiency virus and hepatitis C virus serologies eliminated potential viral causes. Thyroid profile revealed a grossly elevated thyroid-stimulating hormone (TSH) value of 313 μIU/ml; free thyroxine (fT4) was 0.220 ng/dl. The patient was promptly initiated on levothyroxine 50 μg/day which was gradually increased to 100 μg daily replacement therapy. Within 3 weeks of starting the thyroxine therapy, the patient started showing significant improvement in her symptoms of dyspnea and effort intolerance. A repeat echocardiographic examination after 5 months of replacement therapy revealed remarkable improvement in her LV chamber dimensions and ejection fraction to 45% []. There was a concurrent improvement in thyroid profile with the therapy (TSH 12.30 μIU/ml, fT4 1.13 ng/dl). |
A 51-year-old male with a permanent IVC filter that had been inserted approximately 20 years ago when the patient developed a DVT during a hospitalization for severe non-ischaemic cardiomyopathy, was transferred to our medical intensive care unit for shock and acute renal failure. Following the IVC insertion, he had been treated with warfarin for one year and had been on anti-platelet therapy since.\nHe had been admitted to the hospital three days prior to transfer after presenting with progressive bilateral lower extremity pain and decreased sensation in his gluteal region. Acute bilateral DVTs involving the common femoral and popliteal veins were diagnosed. Over 48 h, despite receiving unfractionated heparin, he developed anuric renal failure and shock. Placement of a right internal jugular dialysis catheter was complicated by airway compromise due to a retropharyngeal haematoma necessitating endotracheal intubation. The heparin infusion was discontinued and the patient was transferred to our hospital.\nOn arrival, his mean arterial pressure was 71 (104/53) mmHg while on norepinephrine, vasopressin, and phenylephrine. Arterial blood gas analysis showed a pH of 7.06, partial pressure of carbon dioxide (PaCO2) of 28 mmHg, partial pressure of oxygen (PaO2) of 312 mmHg, and a lactate of 16 mmol/L. The platelet count was 31 K/μL. Examination was notable for tense bilateral lower extremity oedema. Dorsalis pedis pulses were detectable with Doppler ultrasound. An abdominal computed tomography (CT) showed dilation of the distal IVC suggesting thrombosis (Fig. A). Transthoracic echocardiography showed a 25% ejection fraction with no right ventricular dilation or strain. The IVC was collapsible proximal to the hepatic veins. Lower extremity ultrasound confirmed acute bilateral DVTs involving the external iliac and femoral veins. Laboratory evaluations excluded thrombophilia, heparin-induced thrombocytopenia and thrombotic thrombocytopenic purpura. Infusion of 5 L of isotonic fluid and continuous renal replacement therapy led to a reduction in the vasopressor requirement, a reduction in lactate to 2.0 mmol/L, and pH/PaCO2 normalization. However, the lower extremity oedema progressed with development of bullae and purple skin discolouration (Fig. B). Dorsalis pedis pulses became undetectable, consistent with compartment syndrome due to PCD.\nCatheter-directed thrombolysis, surgical thrombectomy, and fasciotomy were deemed to be contraindicated due to ongoing shock, severe cardiomyopathy, the retropharyngeal haematoma, and persistent thrombocytopenia thought to be the consequence of platelet consumption. Unfractionated heparin was restarted and, within 24 h, lower extremity pulses were again palpable. However, there was a progressive rise in creatinine phosphokinase to 44,000 IU/L and an increase in lactate to 5.8 mmol/L despite continued vasopressor support and continuous dialysis. His family decided to pursue palliation and withdrawal of life-supportive measures. Post-mortem examination confirmed an occluding thrombus at the level of the IVC filter with extension to the internal and external iliac veins (Fig A, B). The autopsy did not identify an underlying malignancy. |
A 4.4 kg male was born to a 25-year-old G2P0 at 39 and 5/7 weeks, complicated by shoulder dystocia, requiring forceps-assisted vaginal delivery. Of note, the amniotic fluid was stained with thick meconium at delivery. He was born with low tone, heart rate less than 50 BPM, and required positive pressure ventilation with APGARS of 1, 6, and 8 at one, five, and ten minutes, respectively. Within fifteen minutes of birth, he required endotracheal intubation for poor respiratory effort. He was extubated on day one of life but continued to be hypoxic and tachypneic, which were attributed to presumed meconium aspiration. He was also noted at this time to hold his left upper extremity in adduction and internal rotation with minimal movement. His left upper extremity exam was significant for persistent weakness in wrist extension/finger extension and shoulder adduction, consistent with an Erb's palsy. There were no obvious deformities of his clavicle or humerus, and his pulses were intact. His right arm was completely unremarkable with normal strength and tone.\nSubsequently, with assistance from occupational therapists, the infant began to recover strength and spontaneous movement of the left arm. However, he continued to require cardiorespiratory monitoring, critical care management, and oxygen via high flow nasal cannula for the next 21 days. During his critical care stay, radiographs demonstrated a progressive clearing of his lung fields as well as persistent elevation of his right hemidiaphragm (). It eventually became clear that his need for oxygen supplementation was exceeding the expected course of meconium aspiration. Ultrasonography of the diaphragm was performed, demonstrating decreased motion of the right hemidiaphragm and normal movement of the left hemidiaphragm. Due to the discordance in laterality between the brachial plexus injury and diaphragm dysfunction, presumed to be from an occult contralateral phrenic nerve injury, a thoracic MRI was obtained to rule out other unexpected pathology, such as soft tissue injury or any intrathoracic pathology like a soft tissue mass causing compression on the cervical nerve roots or phrenic nerve, but was only notable for findings associated with the known left brachial plexopathy.\nFollowing a multidisciplinary discussion, thoracoscopic right diaphragm plication was chosen as the management strategy. This decision was influenced by the fact that our patient was requiring high-flow nasal cannula and was unable to be weaned over multiple weeks. Originally, we thought the respiratory distress may have been due to meconium aspiration, but after there was no improvement over the expected time period, this moved lower on our differential. The continued need for hospital care in an otherwise well baby ultimately indicated surgical plication. He was taken to the operating room and positioned in left lateral decubitus position after induction of general anesthesia and endotracheal intubation. Using a 4 mm thoracoscope and two 3 mm instrument trocars, a flaccid right hemidiaphragm with bulging central tendon was noted, then linearly plicated, and flattened. A self-locking suture was used in a running fashion to imbricate the central tendon down into the abdomen, bringing the muscular edges together and beginning the plication. This method also served to reduce the tension on our final suture line. After this was completed, with care to keep the neurovascular bundle out of harm's way, a second row of vertical mattress sutures was placed to further flatten the hemidiaphragm. At the completion of this step, the diaphragm was noted to be reasonably flat with only the slightest dome shape, though not under any remarkable tension at rest (). He tolerated the procedure without complication and was extubated before returning to the neonatal intensive care unit (NICU), where his respiratory rate was immediately noted to be lower than preoperatively. His initial postoperative radiograph showed a flattened right hemidiaphragm, increased aeration of the right lung, and a small basilar right pneumothorax that did not require chest tube placement. By the second postoperative day, he was off supplemental oxygen and tolerating an oral feeding regimen. He was transitioned off-tube feeds over the next 10 days and was discharged home on postoperative day 12 (). |
A 62-year-old woman presented with a 4-week history of dyspnea on exertion and facial edema in November 1999. Her past medical history was unremarkable. Electrocardiography showed T wave inversion at leads V1-5, III, and aVF, right axis deviation, and right ventricular hypertrophy. Initial echocardiography revealed an echogenic mass in the main pulmonary artery with significant flow obstruction and right ventricular dysfunction suggesting pulmonary embolism. Chest computed tomography (CT) revealed an embolism-like mass in the pulmonary trunk and in both sides of the main pulmonary arteries (). Pulmonary artery endarterectomy with pulmonary valve replacement and pulmonary artery reconstruction were performed. The surgical specimen showed an ill-defined, protruding polypoid mass that was 4.8 cm in the greatest dimension. The pathology revealed pulmonary artery intimal sarcoma, which was supported by positive smooth muscle actin and negative desmin in immunohistochemical staining (). Involvement of the resection margin by the tumor was noted. She received radiation therapy (4,500 cGy fractionated) and chemotherapy (cyclophosphamide, doxorubicin, and dacarbazine) as adjuvant treatments. During follow-up, she was regularly checked by echocardiography and chest CT. There was no evidence of local recurrence on echocardiography. On initial follow-up chest CT (November 2001, 2 years after initial surgery), mild diffuse enlargement of the thyroid gland with multiple small low density lesions was discovered. It is hard to know if these findings existed before the initial operation, because the preoperative chest CT did not include the neck. She showed normal thyroid function tests. This lesion was just followed up with chest CT yearly and showed no change for 3 years.\nIn August 2004 (4.7 years after initial surgery), she was referred to an endocrinologist for abnormal thyroid function tests. There was a palpable nodule in the left thyroid gland. Cervical lymphadenopathy was not present. The serum thyroid stimulating hormone level was 0.12 mIU/L (normal, 0.4 to 5.0), and total T4 was 166 nmol/L (normal, 76 to 178). CT of the neck showed two low density nodules of 2.0×2.5 cm and 0.9×0.9 cm in both lobes of the thyroid glands, which was no different from previous studies (). Fine needle aspiration cytology was done and revealed atypical spindle cells, suggestive of metastatic intimal sarcoma (). She had no other metastatic sites in the chest and abdomen-pelvic CT. She underwent total thyroidectomy, and histology confirmed metastatic intimal sarcoma with clear resection margins. She was followed by neck and chest CT (covering the adrenal gland), and there was no evidence of local recurrence or distant metastasis until August 2005.\nA 10.0×8.5 cm sized round, heterogeneous mass replacing the whole adrenal gland superior to the right kidney was newly detected by chest CT in March 2006 (6.3 years after initial surgery), suggesting a metastatic lesion in the right adrenal gland (). An 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET)/CT scan showed a large hypermetabolic mass (max standardized uptake value, 6.8) with internal necrosis (). The lesion was suspected as a recurrence of intimal sarcoma. Right adrenalectomy was done without preoperative biopsy. The histopathologic diagnosis was metastatic intimal sarcoma. The tumor cells were positive for smooth muscle actin and were negative for inhibin, cytokeratin, chromogranin, and desmin, supporting the above diagnosis.\nDuring follow-up after right adrenalectomy, abdomen-pelvic CT and 18F-FDG PET/CT showed no evidence of recurrences or metastases, including the anastomosis site of the main pulmonary trunk, thyroid bed, and adrenalectomy site. She has been alive and well without any evidence of disease as of May 2012 (12.5 years after initial surgery). |
A 71-year-old man diagnosed with esophageal cancer was referred for evaluation at the oncology outpatient clinic of our institution to determine a treatment plan. He had no significant medical history. He had been smoking one pack a day for 50 years. He had used to consume alcohol daily, but had been unable to drink for 3 months. He lived with his wife.\nHe had had epigastric pain for half a year and had experienced swallowing difficulty as well as vomiting for a month before admission. He was diagnosed with esophageal cancer at another hospital he had previously visited.\nAt the first visit to our institution, he showed no mental status changes or neurological abnormalities, with a blood pressure of 126/78 mmHg and pulse of 77/min. He advised staff that his appetite had been reduced to 1/3 that of normal from 2 months previously, and his weight had decreased from 58 to 50 kg over the previous 2 months.\nAlthough there were no mental status changes, cerebellar symptoms, or abnormal eye movements, we suspected TD based on the reduced food consumption for 2 months and the rapid growth of tumors has been reported to result in excessive thiamine consumption [].\nHigh-performance liquid chromatography was employed to assess his serum thiamine concentration, which was found to be abnormally low (24 ng/mL; reference range: 30–70 ng/mL). The patient was, therefore, diagnosed with subclinical TD. After identification of his TD, thiamine (100 mg) was administered intravenously for 3 days. Thereafter, vitamin B1 was included in any infusions deemed necessary.\nOne week after his visit to the Oncology Clinic, he underwent two cycles of neoadjuvant chemotherapy using cisplatin and fluorouracil, followed 2 months by esophagectomy without complication.\nFour months later, he underwent two cycles of adjuvant chemotherapy. The serum thiamine levels just before and after the adjuvant chemotherapy were 32 and 32 ng/mL, respectively. |
A 26-year-old woman of Cypriot origin presented with an acute onset of palpitations and chest tightness. She reported several weeks’ history of similar palpitations, however, this was the first occasion that the sensation had not self-terminated within minutes and had been associated with discomfort.\nThere was no further medical history of note, no relevant family history, and no regular medications. Our patient admitted to a moderate alcohol intake although no recent intoxication and no recreational drug use.\nOn assessment in the Emergency Department, her electrocardiogram (ECG) demonstrated a narrow-complex tachycardia with a rate of 194 beats per minute (bpm) (Fig. ). Her heart rate varied between 180 and 210 bpm and her blood pressure was 187/101 mmHg on first recording. Suspecting SVT, the assessing emergency physicians proceeded with vagal maneuvers, to no clinical effect. Next, an intravenous bolus dose of 6 mg adenosine was administered. This was followed by immediate development of coarse VF (Fig. ) and circulatory collapse. Rapid deterioration to a fine VF ensued. She was successfully defibrillated with a single 150 J direct current shock. There was immediate return of spontaneous circulation and hemodynamic stability. A 900-mg loading dose of intravenous amiodarone was commenced and our patient was admitted to the cardiac high dependency unit. No further arrhythmias were identified.\nInvestigations revealed normal serum electrolytes, negative serial troponin measurements, normal thyroid function, and negative blood ethanol level. A urine drug screen was negative. A chest radiograph demonstrated clear lung fields. The presenting ECG was reviewed, and was likely to be that of AF with a rapid ventricular response, but with no clear ventricular pre-excitation. Similarly, her ECG in sinus rhythm had no features of ventricular pre-excitation (Fig. ). The Q-Tc interval was within normal range and there were no features of Brugada syndrome. In view of the development of VF with adenosine, an electrophysiology study was performed. Following administration of adenosine, there was progressive PR lengthening and eventual atrioventricular (AV) block with non-conducted P waves, excluding a typical anterograde conducting accessory pathway (Fig. ). There was also a negative programmed ventricular stimulation up to three extrastimuli. On exercise stress testing, there was an appropriate heart rate and blood pressure response with no ST/T wave changes and no arrhythmias identified. Cardiac magnetic resonance imaging (MRI) confirmed normal biventricular volumes and systolic function, no myocardial edema, fibrosis or infarction, and normal origins of the right and left coronary arteries.\nOur patient was discharged, without any prescribed medications, following a short inpatient stay during which no further episodes of arrhythmia were identified on continuous cardiac monitoring. At routine follow-up 3 months later, our patient remained well, with no further symptoms and no recurrence of palpitations. She was satisfied by the care she had received and was discharged from routine outpatient review. |
A 68-year-old male with stage III IgA kappa multiple myeloma who had completed two cycles of chemotherapy using bortezomib and dexamethasone presented to the hospital for shortness of breath and respiratory distress. His diagnosis of multiple myeloma was made four months prior after he was found to have lytic lesions in the right humerus, left tenth thoracic vertebra, left first lumbar vertebra and right sacrum on imaging performed after a right humeral pathological fracture. The imaging also revealed a large left chest wall mass, thought to be likely from extraosseous involvement by myeloma. Staging workup was completed including skeletal survey and bone marrow biopsy. Bone marrow biopsy showed hypercellularity with 90% involvement by atypical plasma cells which appear as large-sized, binucleated cells with dense chromatin and prominent nucleoli (Figure ). Syndecan-1 (CD138) and kappa light chain IHC staining showed extensive plasma cell involvement belonging to kappa subtype (Figures ). The cytogenetic analysis also revealed multiple chromosomal abnormalities compatible with a complex cytogenetic profile of male karyotype with derivative chromosomes 17, 14 and 10. Serum protein electrophoresis detected a monoclonal spike, with immunofixation detecting IgA kappa type. Serum IgA levels were 2519 mg/dL (normal reference range: 66-436 mg/dL); immunoglobulin G (IgG) and immunoglobulin M (IgM) levels were decreased. The patient was determined to have stage III using the revised international staging system (R-ISS). The treatment plan was to start the patient on nine to 12 weeks of bortezomib, lenalidomide and dexamethasone velcade, revlimid, low dose (VRd) regimen followed by re-staging. However, the patient did not receive lenalidomide due to poor performance status and underlying comorbidities. He then completed two cycles of bortezomib and dexamethasone before presenting to the hospital. At the time of presentation to the emergency room (ER), the patient was in respiratory distress and required intubation. On computerized axial tomography (CAT), on the scan of chest with contrast, the patient was noted to have large bilateral pleural effusions with severe compressive atelectasis and in addition to previously seen chest wall mass, there were new widespread pleural and paraspinal metastases (Figure ). Thoracentesis was performed and upon analysis, fluid was determined to be exudative containing 17,240 white blood cells with 84% being atypical plasmacytoid cells (Figure ); Thoracentesis fluid lactate dehydrogenase (LDH) was 665 U/L, total protein was 4.5 g/dL, albumin was 1.5 g/dL and potential of hydrogen (pH) was 7.371. Patient’s serum LDH was 475 U/L and total protein was 7.6 g/dL. IHC staining of thoracentesis fluid further revealed CD138, CD31, and kappa light chain positive plasma cells (Figure ) which was consistent with MPE. Due to patient’s respiratory status and progression of his disease, patient’s family decided to terminally extubate the patient. After extubation patient was placed on comfort measures only (CMO) and he passed away within the next twelve hours. |
A 15-year-old boy from rural West Bengal, India, presented with a history of a jellyfish sting on his lower limbs incurred while bathing in the sea in the Bay of Bengal about four weeks earlier. Initially he had an intense burning sensation and swelling of the affected parts along with a skin rash, comprising blisters, redness and superficial ulcerations. There were no systemic symptoms. The initial symptoms subsided with conservative management, including a systemic antibiotic, an analgesic and an antihistamine, but some peculiar asymptomatic skin lesions persisted, compelling him to seek a dermatological consultation. There was no history of any local application on the sites of the jellyfish sting and he reported no past history of any skin disease. In addition, there was no history of a similar illness in the family.\nAn examination revealed multiple small papules over the lower part of his left thigh on its anterior aspect and also over the patellar region (Figure ). The lesions had a distinct violaceous hue and were discrete as well as confluent in a linear fashion in parallel rows. The lesions on his right calf were small papules, grouped in clusters in a linear fashion. Some of these lesions showed crusted erosions. Other areas of his skin were uninvolved and his nails were normal. There was no regional lymphadenopathy or mucosal involvement.\nHistopathology with hematoxylin and eosin stain revealed a dense perivascular accumulation of mononuclear cells immediately beneath the dermoepidermal junction underneath an acanthotic epidermis with tapering rete ridges (Figure ). Focally, a few mononuclear cells were seen infiltrating the basal layer, and vacuolar change of the basal layer was not seen. Perivascular sparse infiltrate was also seen in the deeper portion of the dermis and the subcutis was normal.\nThe skin lesions significantly subsided with a three-week course of a twice daily topical application of betamethasone dipropionate (0.05%) cream. |
A 49-year-old partially edentulous male was referred to the prosthodontic clinic for the management of his complex dental status. His chief complaint was dissatisfaction of his existing dental condition that affected quality of his life. He requested a predictable treatment to improve his dental function and restore the missing teeth. His medical status was unremarkable. Intraorally (), the prominent features were as followed: all the remaining teeth were unopposed and almost contacting the residual ridges; absence of posterior or anterior teeth support, and no occlusal guidance. The excessive thickness of the attached gingival band indicated over-eruption of the remaining teeth which might be the cause of the significant disfigurement of the occlusal plane. The maxilla and the mandible were partially edentulous and classified as Kennedy Class II modification 1. None of the teeth were mobile. The remaining maxillary incisors and the left mandibular premolar were extensively carious and deemed non-restorable. The mandibular left first molar was carious on the mesial surface and required root canal treatment. Measuring the vertical dimensions revealed excessive loss of vertical dimension of occlusion (VDO) which was manifested as excessive freeway space (FWS) (6 mm) (). There was no sign of temporomandibular disorder. The phonetic assessment revealed difficulties in pronouncing the /s/ and /f/ sounds. However, the patient adapted to the missing incisal edges and was able to produce the sound with relative acuity. Radiographic assessment indicated limited vertical alveolar bone height in the second quadrant.\nThe patient was stabilized by extracting the non-restorable teeth and initiating root canal treatment for the mandibular left molar. Oral hygiene measures were demonstrated and emphasized before considering any rehabilitative treatment.\nAfter obtaining study models, occlusal rims were fabricated to record the centric relation at the restored VDO (FWS = 2 mm). With the aid of facebow transfer record, the study models were mounted on a semi-adjustable articulator (Artex Articulator, Jensen Dental, North Haven, CT, USA) (). Subsequently, it was possible to assess the potential treatment options closely and any adjunctive procedure required correction of the occlusal plane. The diagnostic wax-up was completed with simulated crown lengthening surgery (). The planned occlusal scheme was unilateral group function with long centric following the principles of biological occlusion described by Becker and Kaiser. The bilateral group function was opted because of the tendency for Class II incisal relationship hindering efficient canine guidance. Due to the excessive FWS, there was no need to consider further increase in the VDO. On the basis of the diagnostic wax-up, it was decided to extract the severely over-erupted mandibular left second molar and crown lengthening of all the remaining teeth. Further, the diagnostic wax-up was utilized to determine the location of the implants in the second and fourth quadrant. The next treatment options were presented:\nMaxillary rehabilitation with crowns and precision implant-supported removable partial denture (RPD). Mandibular rehabilitation with crowns, fixed partial denture and implant fixed partial denture. Extraction of the maxillary teeth and rehabilitation with implant fixed prosthesis. Mandibular rehabilitation with crowns, fixed partial denture and implant fixed partial denture. Maxillary and mandibular RPDs\nFor the maxilla, despite the patient's preference for the fixed option, he was reluctant to undergo through extensive bone grafting procedure. Therefore, the first option was selected. As recommended by several authors, strategic freestanding implant placement was considered to modify the Kennedy classification.- Additional advantages included improving of the retention and stability, enhancing of patient comfort, and simplifying of prosthesis design.,,\nSurgical templates were fabricated to guide the implant placement and the crown lengthening surgery. For the mandible, thee regular platform implants (Biomet 3i, Palm Beach Gardens, FL, USA) were inserted in the region of lower right canine, first premolar and first molar. For the maxilla, to overcome the limitation of compromised bone quantity, single wide platform implant (Southern Implants Ltd, Irene, SA) was placed posterior to the maxillary sinus with angular orientation (). The angular implant placement allowed engaging of maximal amount of available bone. To minimize the impact of poor bone quality, conventional healing period was followed as advised by Friberg et al..\nThe first phase of the rehabilitative treatment involved restoring the VDO, stabilizing the occlusion and improving the esthetics by constructing provisional maxillary cobalt-chromium RPD and mandibular screw-retained metal reinforced composite resin FPD (). The patient was monitored closely for 3 months. Through this period, mastication, comfort, phonetics, and esthetics were closely assessed.\nThe second phase of the rehabilitation comprised of preparing the maxillary and mandibular teeth for porcelain fused to metal prostheses. Due to the need to rectify the occlusal plane, it was necessary to prepare all the teeth simultaneously. Chair-side provisional prostheses were fabricated (Luxatemp, DMG, Hamburg, Germany) and cemented with temporary cement (TempBond, Kerr Corporation, Orange, CA, USA). The maxillary RPD was readapted to fit the provisional prosthesis.\nThe technician was instructed to follow the diagnostic wax-up closely to correct the occlusal plane. In this phase, the definitive mandibular prostheses were completed at the correct occlusal plane. The maxilla was provisionally restored with metal-reinforced cross arch provisional prosthesis. As described by Emtiaz and Tarnow, a mesh of cobalt-chromium was constructed and veneered with acrylic resin (SR Ivocron, Ivoclar Vivadent, Schaan, Liechtenstein). This allowed the major portion of the fitting surface along with all margins to be covered with acrylic resin () facilitating future removal and possible adjustments.\nThe mandibular definitive prostheses were cemented with glass ionomer cement (Fuji I, GC Corporation, Tokyo, Japan). The maxillary prosthesis was tried in and cemented temporarily (TempBond, Kerr Corporation, Orange, CA, USA). Any necessary occlusal adjustments were performed on the maxillary provisional prosthesis. From the diagnostic perspective, the maxillary provisional prosthesis allowed more precise assessment of the function, esthetics and phonetics (). The patient was reviewed weekly for a period of one month and demonstrated high level of oral hygiene.\nThe final phase of the rehabilitation involved the fabrication of definitive maxillary fixed prosthesis and precision RPD. Since the maxillary provisional prosthesis provided stable occlusion, it was utilized to obtain precise interocclusal record. To enhance the predictability of the laboratory articulation, medium body silicone impression material (Exahiflex, GC Corporation, Tokyo, Japan) was applied on the occlusal surface and the patient was asked to occlude on the previously achieved occlusion. For the first quadrant, porcelain fused to metal crowns were fabricated with milled surfaces incorporated on the palatal aspect (). As a future contingency planning, extra-coronal precision attachment (Bredent LTD, Chesterfield, UK) was incorporated mesial to the maxillary right canine. For the second quadrant, telescopic retention mechanism was used. Common path of insertion and an occlusal convergence angle of 4 degrees were obtained for palatal milled surfaces and the primary telescopic copings with the aid of a milling system attached to a surveyor table (AmannGirrbach AG, Bregenz, Austria).\nThe merit of applying telescopic attachment is omitting the palatal major connector without compromising the rigidity of the final RPD framework. For the angulated implant, the telescopic attachment was suitable to compensate for angulated orientation without compromising the retention. Another advantage of palatal milled surfaces and telescopic attachments is enhancing the stability and retention by restricting the RPD path of withdrawal and creating friction between the intimately fitting parallel surfaces., In addition, with well distributed abutments, the occlusal forces are directed axially.\nThe maxillary fixed prostheses were tried in and pick-up impression was taken with polyether impression material (Impregum Penta Soft, 3M ESPE, St. Paul, MN, USA). Secondary copings were fabricated by electroforming process on the primary telescopic copings. Precision cobalt-chromium framework was constructed on the milled surfaces and secondary copings (). The framework try-in step revealed passive fit of all the components. The definitive RPD was designed to mimic the morphology and occlusion of the provisional prosthesis ().\nThe fixed prostheses and the primary coping were cemented permanently with glass ionomer cement (Fuji I, GC Corporation, Tokyo, Japan). The implant primary coping was fitted and the retaining screw was tightened to 35 Ncm. The telescope fitting surface of the framework and the external surface of the electroformed copings were sandblasted and treated with metal primer (Panavia F Alloy Primer, Kuraray Dental, Osaka, Japan). Individually, the electroformed copings were cemented with resin based cement (Panavia F, Kuraray Dental, Osaka, Japan) to the RPD and the excess cement was removed. The patient was review weekly for the first month (). |
A 41-year-old gentleman presented to the eye clinic complaining of seeing floaters before both eyes since 2 weeks. On questioning, he admitted getting intermittent dull headache of mild to moderate intensity and dizziness on and off while walking since 2 months. There was no associated nausea or vomiting or other neurological symptoms. He was detected to be a hypertensive few months ago and was on treatment with lisinopril 5-mg daily.\nOcular examination revealed a best-corrected Snellen's visual acuity of 6/6 p in both eyes. Anterior segment examination showed exotropia of about 45° in left eye that was alternating. Rest of anterior segment examination including intraocular pressure was normal. Fundoscopy of the right eye revealed early vitreous hemorrhage. The disc showed slight blurring of margins associated with peripapillary retinal hemorrhages []. Fundus examination of the left eye showed early vitreous hemorrhage and blurring of disc margin. There was a small area of preretinal hemorrhage inferior to the disc []. B scan ultrasound also confirmed early vitreous hemorrhage.\nA diagnosis of bilateral early disc edema with mild vitreous hemorrhage was made, and he was investigated. Contrast-enhanced CT scan of brain showed a well-defined oval nonenhancing hyperdense lesion measuring 2 × 1.9 × 2.3 cm at the anterosuperior aspect of third ventricle bulging through the right foramen of Monro and compressing the left foramen of Monro causing dilatation of both lateral ventricles with cerebrospinal fluid (CSF) seepage []. Fourth ventricle appeared normal. Moderate 0.8-cm midline shift was also seen to the left side.\nA presumptive diagnosis of colloid cyst causing moderate obstructive hydrocephalus was made, and he was referred urgently to the department of neurosurgery. He underwent emergency craniotomy and cyst excision. Histopathological examination of the excised tissue showed cyst contents lined by cyst wall lined by ciliated cuboidal epithelium resting on fibrocollagenous tissue consistent with colloid cyst. Postoperative CT scan showed pneumocephalus and subdural collection in the right frontotemporal region that resolved spontaneously after few days.\nThe patient improved symptomatically following surgery. On follow-up after 1 month, he reported complete resolution of floaters and headache.\nFundus evaluation showed resolving vitreous hemorrhage and disc edema. Follow-up after 3 months showed complete resolution of disc edema and vitreous hemorrhage [Figure and ]. He is being followed up closely for recurrence of symptoms. He has remained asymptomatic for the last 18 months after the intervention. |
A 56-year-old man with no significant past medical history presented to the hand clinic with a 3-year history of right-hand pain and swelling. He works as an electrician and described a chemical spill on his right hand in 2013. He described the substance as consisting of hydraulic fluid, glycol, and penetrant. He was noted to have abnormal skin changes and intermittent swelling in his wrist and hand. At that time, he had been prescribed topical steroids without relief. He reported worsening of his right hand and wrist swelling over the past 2 months, shortly after helping his wife move a fish tank. He does not believe that his hand was in the water tank. He is frequently outdoors and works with the soil in his yard. He also frequently swims in a freshwater lake. His physical exam was abnormal for profound swelling of the right thumb, the volar aspect of the right wrist, and the right 5th finger (). Initial laboratory results showed a slightly elevated CRP of 1.4 mg/dL and normal ESR at 13 mm/h. An MRI demonstrated enhancement of the flexor tendon sheaths in the wrist and hand (). He underwent a radical synovectomy of the hand and wrist (). He was initially started on intravenous piperacillin/tazobactam.\nPathology was suggestive of necrotizing granulomatous tenosynovitis. The initial piperacillin/tazobactam was discontinued. Given the patient's history of exposure to a fish tank, the biopsy tissue was sent for an acid-fast bacilli (AFB) culture. The AFB smear was positive. Mycobacterium tuberculosis was thought to be less likely, given the lack of tuberculosis exposure history and environmental exposures concerning for an atypical mycobacterial infection. He was initially started on rifampin and clarithromycin for possible Mycobacterium marinum. After the culture grew Mycobacterium kansasii, isoniazid (INH) was added. He improved on the above regimen. However, 4 months after starting the regimen, his susceptibilities via the agar proportion method suggested resistance to the INH. INH was discontinued, and he was started on ethambutol, in addition to the rifampin and clarithromycin. His isolate was then sent to an alternative reference lab, which tested resistance via the broth dilution method. This also demonstrated INH resistance. He completed 2 more months of this regimen for a total of 6 months of therapy. Shortly before completing the regimen, the second lab reported resistance to ethambutol. As he had clinically improved, the decision was made not to alter or extend therapy.\nHe has done well since discontinuing therapy, with resolution of the swelling and a return to his previous function. |
A 3-year-old boy under pediatric pulmonary care for CF presented to the Bascom Palmer Eye Institute, University of Miami, with a 2-year history of chronic bilateral conjunctival inflammation, mild epiphora, and irritation. Despite bilateral disease, the symptoms were significantly worse in the right eye than the left. The patient was evaluated in multiple subspecialty ophthalmology clinics both in the United States and internationally and in each, he was diagnosed with allergic conjunctivitis. His parents denied symptoms of nasal obstruction, other than snoring, which had been present prior to the onset of the eye symptoms. No cultures had been obtained from the conjunctiva. Treatment regimens, including emergency room treatment, involved the use of topical anti-allergy drops such as olopatadine (Patanol) which improved the swelling only temporarily. He was referred to us for further evaluation. Clinical examination disclosed erythema in the medial aspect of the orbit, lower lid greater than upper eyelid, a palpable prominence under the medial canthal tendon and in the region of the nasolacrimal sac, and telecanthus on the right ().\nOcular examination was notable for significant papillary conjunctivitis of the right eye. Extraocular motility was full and the pupils were reactive OU without afferent pupillary defect. He had a normal tear lake and no mucopurulent reflex with compression of the nasolacrimal sac. The rest of his examination was within normal limits.\nPast medical history was notable for CF; pancreatic insufficiency had been appropriately managed with pancreatic enzymes. Additionally, he was noted to have chronic airway (deep throat culture based) colonization with E. coli, an organism seen infrequently in young children with CF. Despite multiple attempts to eradicate it with nebulized tobramycin and oral antibiotics, the organism persisted. A decision was made against long-term antibiotics, as E. coli is not considered to be a Gram-negative organism that predicts an adverse pulmonary outcome in patients with CF. The patient was continuously followed by pulmonology to monitor his respiratory status.\nMagnetic resonance imaging (MRI) of the brain was obtained to further evaluate the medial canthal prominence. In the orbits, an area of mass effect was seen in the canthal region of the right orbit. This area extended inferiorly to the level of the inferior turbinate and involved a large area of the ethmoid sinus on the right side. Mass effect also caused bowing of both the lamina papyracea and nasal septum. The mass had high signal intensity on T1 and low signal on T2 weighted images, and there was ring-like enhancement (), most consistent with an ethmoidal mucocele.\nThe patient underwent functional endoscopic sinus surgery at the University of Miami with marsupialization of the right ethmoidal mucocele (), which was later confirmed by culture to be infected with E. coli. After post-operative follow-up and continuous pulmonary care, the patient showed no symptoms of allergic conjunctivitis, the telecanthus and medial canthal mass resolved, and the upper airway has remained free of E. coli. |
A 46-year-old Bahraini female diagnosed as premature ovarian failure at the age of 29 years treated with hormonal replacement therapy presented with a history of epigastric abdominal pain and vomiting at the age of 37 years. Biochemical and radiological assessment showed features of acute pancreatitis in terms of elevated pancreatic enzyme level, and CT abdomen finding showed edematous pancreas with normal ductal system. It was attributed to hormonal replacement therapy after thorough investigation. Although the patient had stopped the implicated medications, she still had recurrent attacks of acute pancreatitis.\nSince there was no obvious cause found for her recurrent episodes of pancreatitis, autoimmune pancreatitis was suspected.\nThen, she underwent endoscopic ultrasound in 2015 which revealed mass swelling at the duodenal ampulla, and biopsy was taken. The biopsy showed ampullary adenoma with high-grade dysplasia (Figures and ).\nThen, the patient decided to go abroad for further assessment where she underwent Whipple's procedure and histopathology confirmed the presence of ampullary adenoma with high-grade dysplasia.\nUnfortunately, she continued to have recurrent episodes of pancreatitis despite the removal of the ampullary adenoma.\nIn 2016, while she was admitted under care of a surgical team for another episode of pancreatitis, she was reviewed by the rheumatology team to rule out autoimmune condition. Therefore, IgG4 level was tested (1.49 g/L (149 mg/dl)). The biopsy was reassessed and found to have increased IgG4-positive plasma cells around 30–40 per high-power field with the background of adenoma with high-grade dysplasia. Accordingly, she was diagnosed to have both IgG4-related disease and ampullary adenoma.\nShe was started on oral prednisolone 0.5 mg/kg and rituximab therapy with significant improvement over 1 year of follow-up as the pancreatitis attacks have reduced from around once in every month to around once in every 3 to 4 months after 3 months of rituximab therapy, and currently she remained attack free for around one year. |
A 43-year-old homosexual man who was HIV positive for 18 years presented with a one-year history of a slowly enlarging mass in the proximal left anterior thigh. He described stabbing pain, often experiencing sharp shooting pains down the left thigh. He had been on and off antiretroviral medication, which he had stopped three years prior to this presentation. He had bilateral total hip replacements for avascular necrosis and osteoarthritis approximately three years prior to this visit. He reported no specific trauma or previous injection to his left thigh.\nOn physical examination, he appeared to be in good health. His gait was antalgic. He had no visible mucocutaneous KS lesions and he did not exhibit features of fat maldistribution. There was a firm 3 cm mass present deep in his left thigh that was tender to palpation. The mass was well away from the groin and inguinal region. In particular, there were no overlying skin changes or associated lymphedema. He had enlarged axillary lymph nodes. His complete blood count was unremarkable and his CD4 T-cell count was 249 cells/mm3 and HIV viral load 72 copies/mL while off all antiretroviral medications.\nAn ultrasound test showed a 2.6 × 1.8 × 1.2 cm solid, vascular, heterogeneous lesion within the deep thigh soft tissue. A magnetic resonance image (MRI) showed a solid, vascular enhancing mass with spiculated margins (Figure ) located within the subcutaneous fat, superficial to muscle, in the left anterior thigh. The mass measured 2.2 cm in greatest diameter, and was associated with a second inferior satellite 1.4 cm subcutaneous tumor. Tumor was isointense to muscle on T1W1 and heterogeneous, but mostly hyperintense on T2WI. After gadolinium administration, both lesions enhanced. The larger index lesion enhanced heterogeneously and vessels were identified entering the proximal and distal aspects (Figure ). No nodal disease was reported. Fecal occult blood test performed for evidence of gastrointestinal KS was negative and a chest x-ray showed no evidence of pulmonary KS.\nFine needle aspiration with a 22-gauge needle yielded only few atypical spindle cells. Therefore, an ultrasound-guided core biopsy was performed which showed KS with spindled tumor cells (Figure ). KS tumor cells were immunoreactive for the vascular markers CD34 and CD31, for the lymphatic endothelial marker D2-40, positive for the HHV8 marker LNA-1, and demonstrated no staining with actin, desmin, cytokeratin cocktail, epithelial membrane antigen and S-100. The patient received pegylated liposomal doxorubicin with subsequent shrinkage of tumor and amelioration of his symptoms. |
A 24-year-old Haitian female with no known past medical history or smoking history was in her usual state of health until three weeks prior to presentation when she started to experience right arm pain, numbness, and tingling sensation. She did not report a history of cancer in her family. She initially presented to an urgent care facility where she was prescribed nonsteroidal anti-inflammatory drugs (NSAIDs) and muscle relaxants without any improvement of her symptoms. She also had two episodes of pre-syncope over the past two months which she attributed to poor diet; she did not seek any medical attention. On the day of presentation to the emergency department (ED), the patient was returning from the Bahamas via airplane when she felt progressively worsening right-sided chest discomfort radiating to the right flank and back. She reported right upper extremity pain and numbness. She did not report cough, fever, shortness of breath, cold symptoms or recent changes in the weight and appetite. Her vitals in ED were a heart rate of 120 beats per minute, oxygen saturation of 98% on room air, blood pressure of 110/78 mm Hg, and temperature of 98.2 F. Her physical examination was unremarkable. Initial laboratory investigation was normal including complete blood count (CBC) and comprehensive metabolic panel (CMP). A preliminary chest X-ray was done (Figure ).\nDue to tachycardia and positive travel history, a computed tomography angiogram (CTA) was performed which was negative for a pulmonary embolism. However, CTA revealed a heterogeneous superior right mediastinal mass, measuring about 10 cm. The trachea was noted to be severely deviated to the left and markedly stenosed. Multiple pleural-based lung masses and nodules were also present on imaging, which are classical imaging signs of adenocarcinoma (Figure ).\nThe patient underwent a mediastinal biopsy and was diagnosed with metastatic poorly differentiated non-small cell adenocarcinoma (Figure ). She later underwent a bronchoscopy with tracheal stent placement. Her right arm pain, numbness, and tingling eventually resolved after treatment with systemic steroids. She is now undergoing systemic chemoimmunotherapy with carboplatin/pemetrexed although she has recurrent admissions to the hospital for chronic malignant pleural effusions and chest pain, requiring tunneled pleural catheter placement. |
A 47-year-old woman was admitted to the hospital with a diagnosis of severe COVID-19-associated pneumonia. Her medical history was positive for hypothyroidism, hypoparathyroidism, and anxiety disorder. She was hospitalized in the ICU for 15 d and kept on mechanical ventilation for 5 d. The patient was subjected to neurological evaluation because of her report of mental confusion, visual and auditory hallucinations, insomnia, and psychomotor agitation soon after sedation removal. After discharge to the infirmary, she showed spontaneous improvement of agitation, though with persistent complaint of short-term memory problems and executive dysfunction. The patient also complained of a burning pain in inferior members and imbalance when walking, being able to walk only with assistance. Upon neurological exam, the patient was alert, conscious, and temporally disoriented. The oculomotor exam revealed paresis of the conjugated downward gaze without nystagmus. She also had discrete cerebellar dysmetria of the upper extremities, ataxic gait needing assistance to walk, compromised static balance, and a positive Romberg test. MRI () and EEG exams showed no abnormalities. Cerebrospinal fluid analysis was also within the normal range, including the absence of oligoclonal bands and normal IgG index. The patient underwent intravenous thiamine therapy (500 mg, 8/8 h) for five d. After two d of treatment, there was partial improvement of memory, executive dysfunction, ophthalmoparesis, cerebellar dysmetria in the upper extremities, and gait ataxia. Thiamine treatment also promoted complete improvement of static balance, insomnia, and neuropathic pain in the lower extremities, though maintaining positive Romberg test. After 20 days from discharge, the patient was reassessed on an outpatient basis and showed discrete memory and executive function complaints, without impairment of the performance of daily life activity and maintenance of functional independence. The patient displayed complete improvement of gait ataxia, a negative Romberg test, oculomotor skills with discrete paresis of the right trochlear nerve, and reported discrete allodynia in the lower extremities. |
A-17-year-old previously healthy female presented to THP with a history of fever for 2 days associated with body aches and nausea. She didn’t have any abdominal pain, bleeding manifestations or postural symptoms. On examination, she was flushed and febrile but was not pale or icteric. She was mildly dehydrated. Blood pressure was 100/70 mmHg, pulse rate 100 beats/min and capillary refilling time (CRFT) was less than 2 s. On abdominal examination, there was no free fluid. Lung fields were clear on respiratory system examination. Other systems examination was normal.\nHer NS1 antigen was positive and serotype was identified as DEN1. She was managed as dengue fever with continuous monitoring. On the 3rd day of fever, she complained of retrosternal chest pain and undue tiredness. At that time her cardiovascular system examination was normal and electrocardiogram (ECG) showed acute T wave inversion in V2-V5 leads. Troponin I was negative and 2D echo showed global left ventricular hypokinesia and mild impairment of LV function. Ejection fraction was 40–45%. She was treated as having dengue fever complicated by myocarditis. Intravenous hydrocortisone 200 mg 8 hourly was administered for 2 days to reduce myocardial inflammation. On the 4rd day following admission, she complained of abdominal pain and ultrasound scanning revealed free fluid in hepato-renal pouch. Blood pressure was 100/70 mmHg, pulse rate 70 bpm, and CRFT was less than 2 s. She was taken to High Dependency Unit (HDU) and was managed as having DHF complicated with myocarditis with continuous monitoring and with careful administration of fluid to avoid fluid overload. She was discharged on day 7 of illness after recovering from critical phase of dengue fever. She was advised on limiting physical activities. During the follow up on day 14 of the illness, ECG showed reversal of T inversions. Echocardiogram showed improvement of left ventricular function with an ejection fraction of 55%. |
A 47-year-old female patient visited hospital for a national health screening program. Esophagogastroduodenoscopy revealed a 0.8-cm-sized depressed lesion at the posterior wall of the first part of the duodenum (, ). Histologic analysis of the biopsy specimen revealed atypical glands forming cell clusters beneath the intact mucosal layer. The atypical clusters were arranged in tubular structures of columnar epithelium consisting of mucin-producing cells. Dystrophic calcification was scattered within the lesion, and foreign body reaction with giant cell formation was noted around the calcified materials (, ). Immunohistochemistry (IHC) for CD56, chromogranin A and synaptophysin showed negative results, excluding the possibility of a neuroendocrine tumor, which is prevalent in this region. The histologic diagnosis was well-differentiated adenocarcinoma, and the patient underwent routine preoperative work-up. Physical examination, blood tests and abdominal computed tomography (CT) scan showed no abnormal findings. A minute duodenal lesion with submucosal invasion was suspected on endoscopic ultrasonography. Since the calcification was minute, it was not recognized by simple X-ray or by CT scan even upon retrospective review.\nEndoscopic resection was not an option because it is usually difficult to remove a lesion by endoscopy at the posterior wall of the duodenum. The possibility of submucosal invasion was also considered; hence, the patient received distal gastrectomy. Since the lesion was located in the duodenal bulb, the duodenal stump was made near the pancreas and the distal resection margin was close to the lesion. D1+ lymph node dissection was performed.\nOn gross examination, the specimen consisted of 2.7 cm long duodenum and 6.5 cm long stomach. On duodenal mucosa, a 0.8 × 0.8-cm-sized superficial depressed lesion was found (). The lesion was located at the posterior wall of the duodenum, 1.9 cm distal to the pyloric ring and 0.3 cm proximal to the distal resection margin. Pathologic examination revealed well-differentiated adenocarcinoma involving mucosal and submucosal layers. Around the carcinoma, dysplastic Brunner gland epithelium was noted. ()\nIHC was done for further evaluation. Gastric foveolar type mucin (MUC5AC) was focally positive and Brunner gland type mucin (MUC6) was diffusely positive (). Carcinoembryonic antigen (CEA) was negative and p53 was positive in an increasing order of intensity from normal to adenoma-adenocarcinoma spectrum (–). A microsatellite instability (MSI) test was performed to investigate the involvement of mismatch repair failure during carcinogenesis. None of the five markers, which include BAT25, BAT26, D2S123, D5S346, and D17S250, showed instability.\nThis study was approved by the Institutional Review Board of Seoul National University Hospital (IRB No. H-1706-098-860) and performed in accordance with the principles of the Declaration of Helsinki. Patient informed consent was waived. |
A 34-year-old female with no significant past medical history presented to our clinic after experiencing a left second metatarsal stress fracture (Figure ). One year prior, while running errands around town, she suddenly felt a sharp pain in her left midfoot and promptly consulted an orthopedic surgeon who placed her in a boot. Six months later, after experiencing minimal improvement in her pain, a different orthopedic surgeon performed an open reduction and internal fixation by injecting 1 mL of bone cement into the diaphysis of the second metatarsal.\nOver the next six months, she noticed no meaningful improvement in her pain. At this point, she presented to our clinic for a third opinion. During our initial visit with her, she stated that her left foot felt different than her right at baseline.\nOn physical exam, there was no gross deformity of her left lower extremity. The skin was intact with a healed incision over the dorsal midfoot, and there was point tenderness to palpation over the second metatarsal. Active and passive range of motion of the ankle and transverse tarsal joint was full and painless. Strength was 5/5 in dorsiflexion, plantarflexion, inversion, and eversion. Sensation to light touch was intact, Achilles reflex was present, and dorsalis pedis and posterior tibialis pulses were palpable.\nLaboratory work revealed an elevated erythrocyte sedimentation rate of 36 (reference range: 0–20) and C-reactive protein of 34.74 (reference range: 0–10.9). Plain radiographs and a computed tomography (CT) scan of the left foot showed diffuse sclerotic changes and cement within the left second metatarsal (Figures , ). Magnetic resonance imaging (MRI) showed diffuse edema of the left second metatarsal with a non-displaced fracture line (Figure ).\nAll treatment options were discussed with the patient and she agreed with undergoing operative fixation. In the operating room, cultures and a bone biopsy of the left second metatarsal were taken. After performing an osteotomy, curettage was performed to remove the injected cement. Open reduction and internal fixation was performed utilizing a plate and calcaneal bone graft (Figure ). The patient was discharged home on the same day with adequate pain control and a bone stimulator. X-rays taken at two weeks post-revision surgery are shown in Figure .\nAt one-month follow-up, her incision was healing well without signs of infection and she had no complaints of pain. At her most recent appointment—three months post-revision surgery—she again reported no pain and good functional recovery with physical therapy. CT scan at three months post-revision surgery showed appropriate alignment of the healing second metatarsal with intact hardware (Figure ). |
An 18 year old woman was referred to our outpatient clinic of Endocrinology, University-Hospital of Naples Federico II because of hypothyroidism due to Hashimoto's thyroiditis. Blood samples showed high levels of thyroperoxidase and thyroglobulin antibodies and normal calcitonin serum levels. The patient was euthyroid with normal serum levels of thyroid-stimulating hormone (TSH), free triiodothyronine (FT3), and free thyroxine (FT4) during replacement therapy with L-T4. At physical examination, a palpable nodule of ~2 cm in size was detected in the isthmus of the thyroid. There were no palpable cervical lymph-nodes. An US evaluation confirmed an isolated lesion located in the isthmus, showing an isoechoic solid nodule with smooth margins; its size was 18 × 13 × 6 mm with intra and perilesional vascularity (Figure ). Therefore, a FNA was performed and cytological results revealed a TIR3A lesion. The cytological specimen showed an increased cellularity with some microfollicular structures in the background of scant colloid (Figure ). Thus, we assessed the risk factors associated with the isolated TIR 3A nodule of our patient. According to the ATA guidelines we repeated the FNA which confirmed the same result (TIR3A). The second US (after 6 months) showed that there were no clear signs suggesting malignancy such as microcalcifications or taller than wide-shaped nodules. However, we found a small hypoechoic cranial component in the nodule with blurred margins and elastography revealed an increased stiffness in this cranial component. No nodules were detected in the contralateral lobes by US; cervical lymph nodes were normal. Among the possible risk factors, our patient referred a familial history of thyroid cancer. Her mother was submitted to total thyroidectomy for a follicular variant of PTC twenty years ago; our subsequent evaluation showed that she was disease free at the moment.\nOn this basis, we decided that a surgical treatment was indicated for our patient and assessed the risk/benefit of total thyroidectomy vs. isthmusectomy. |
We present a 22-month-old boy with transposition of the great arteries (TGA) who underwent the arterial switch operation (ASO) in our paediatric cardiology department at the age of 3 days with excellent surgical result. Cardiac catheterization performed at the age of 13 months showed normal coronary arteries and good biventricular function.\nThe patient presented to our partner hospital with persistent high fever in association with skin rash, conjunctivitis, red and cracked lips, and inflammation of hands and feet. An episode of vomiting and diarrhoea was also reported. The patient appeared weary, but no focal neurological deficit was detected. He and his first-degree relatives had an episode of high fever together with a cough three months before admission. A SARS-CoV2 reverse transcription polymerase chain reaction (RT-PCR) assay in nasopharyngeal secretions of the patient at the time of admission showed negative results, whereas a serological assay detected specific SARS-CoV-2 immunoglobulin G (IgG) antibodies. Brain natriuretic peptide and troponin levels were elevated (N-terminal pro-B-type natriuretic peptide: 9450 ng/L, normal range <320 ng/L; troponin T: 6252 ng/L, normal range <14 ng/L).\nThe overall initial clinical presentation was consistent with the diagnosis of KD. According to the statement from the American Heart Association, intravenous immunoglobulins, acetylsalicylic acid in therapeutic doses and prednisolone were initially administered.\nEchocardiography was performed shortly after admission and did not demonstrate coronary artery changes. A follow-up echocardiography performed 11 days after the onset of symptoms showed dilatation of both coronary arteries. Ventricular function was preserved. Due to clinical deterioration and coronary dilatation, the patient was transferred to the intensive care unit and was treated with infliximab and steroids. This was followed by a clinical improvement. The patient was scheduled for further investigations in our paediatric cardiology department.\nAn electrocardiogram at the time of admission in our department was normal. Detailed assessment of the coronary arteries by echocardiography was difficult () and therefore cardiovascular magnetic resonance imaging (MRI) was performed. This showed ectasia of both coronary arteries after its origin (). The right coronary artery (RCA) had a maximum diameter of 8 × 9 mm but its origin could not be adequately displayed. All branches of the left coronary artery (LCA) were significantly dilated with a maximum diameter of the left anterior descending artery (LAD) of 4 × 7 mm. Perfusion MRI at rest showed a perfusion defect in the RCA territory (). Stress perfusion was not performed due to safety reasons because rest perfusion already suggested significant ischaemia. Biventricular systolic function was normal (left ventricular ejection fraction 63% and right ventricular ejection fraction 61%). Late enhancement imaging did not demonstrate any myocardial fibrosis () and there was no evidence of myocardial inflammation on T2-weighted images.\nAfter multidisciplinary team discussion, the indication for cardiac catheterization was made, considering both, the difficulty to visualize the RCA origin and the perfusion defect. Cardiac catheterization confirmed the likely diagnosis of a proximal RCA stenosis ().\nAnticoagulation therapy with dicoumarol in addition to acetylsalicylic acid was started and a betablocker therapy was initiated. The patient was discharged in stable conditions. In retrospect MIS-C associated with COVID-19 was thought to be the most likely diagnosis for the overall clinical presentation.\nDue to the difficulty to assess the coronary arteries on echocardiography, follow-up MRI scans were performed 7 and 13 weeks after disease onset. These investigations demonstrated a further dilatation of the coronary arteries compared to the first cardiac MRI (maximal RCA diameter 11 mm and LAD diameter 8 mm) with a stable perfusion defect, preserved biventricular systolic function and no evidence of myocardial fibrosis. No further dilatation of the coronary arteries between the two follow-up MRI scans was seen. Two brain MRIs performed in conjunction with cardiac MRI demonstrated stable mild periventricular leukoencephalopathy, enlarged perivascular spaces and some cerebral microbleeds compatible with residuals from heart surgery with heart–lung machine (). No acute or recent inflammatory changes were detected.\nThe follow-up plan includes frequent cardiac examinations every 3 months either in our outpatient department or by a local paediatric cardiologist. Furthermore, the patient continues with long-term thromboprophylaxis as suggested in the AHA Scientific statement including both low-dose aspirin and oral anticoagulation with dicoumarol. In addition, assessment for inducible myocardial ischaemia will be performed every 6–12 months or if the patient has symptoms. |
A 41-year-old Caucasian man presented at an emergency department with acute chest pain. The chest pain began 2 h before presentation. There was no history of trauma or excessive physical exercise. He had no other complaints and used no medication. Medical history recorded a subdural haematoma, as a result of trauma. According to the family history, his mother and aunt were both diagnosed with ADPKD. Our patient was a non-smoker, with no history of hypertension, diabetes mellitus or hypercholesterolaemia.\nOn physical examination, the blood pressure was 155/102 mmHg and the pulse 72 beats/minute; other vital parameters were normal. Electrocardiography showed a sinus rhythm of 65 beats/minute and ST elevation in the precordial leads (). Creatine kinase was 249 U/L, creatine kinase MB was 17.4 U/L and troponin T was 0.21 ng/mL. The other laboratory test results were normal. Twelve hours later, the cardiac enzymes increased to higher levels. Echocardiography revealed a hypokinetic septum and a slightly impaired left ventricular function with an ejection fraction of 45–60%.\nBased on these results, a presumptive diagnosis of acute septal myocardial infarction was made. The patient was immediately transported to the University Medical Centre for cardiac intervention. Coronary artery angiography (CAG) revealed a transient occlusion of the left anterior descending (LAD) coronary artery, most probably as a result of myocardial bridging (). Other coronary arteries appeared normal. Percutaneous coronary intervention was not performed due to small vessel size. The myocardial infarction was treated with anti-platelet therapy, statin and metoprolol.\nHowever, the chest pain returned 3 days after the presentation. A second electrocardiography showed persistent inverted T waves in the precordial leads without ST elevation. The cardiac enzyme levels were again increasing. A second CAG was performed because of suspicion of recurrent myocardial infarction. It disclosed an open LAD with a dissection in the distal part and a double lumen, which was not observed during the first angiography (). Myocardial scintigraphy showed a restricted infarction of the septum.\nThe definitive diagnosis of non-Q-wave anterior infarct as a result of a spontaneous LAD dissection was made. The suspicion of myocardial bridging was rejected.\nUnexpectedly, the echocardiography revealed multiple liver cysts. The following abdominal echography also showed multiple renal cysts. Based on the cysts in both kidneys combined with a family history of ADPKD, the diagnosis of ADPKD was made (Ravine’s criteria) [].\nSince cerebral aneurysms are one of the extrarenal manifestations of ADPKD [], a computerized tomography angiography of the brain was performed, with a negative result for vascular anomalies. An exercise stress test, performed 16 days after the onset of chest pain, was normal. An elective CAG was performed at 6-month follow-up. The LAD was patent without a significant infarction, but still with a double lumen appearance. At 2-year follow-up by a nephrologist, his kidney function is still in the normal range. His blood pressure is also normal with use of perindopril and amlodipine. |
Patient CS, a single 60-year-old male presenting with a history of generalized anxiety with panic, major depressive disorder, and excessive guilt, was referred from a county hospital to a tertiary psychiatric facility for clarification of diagnosis and a more comprehensive assessment. His sister, and the family physician that had been following the patient for the past 4 years, helped provide collateral history. His family noted that he was born with a large head. He had a history of meningitis at the age of 9 or 10 after which it is thought that he developed a non-communicating hydrocephalus. His past psychiatric diagnoses included major depressive disorder, generalized anxiety disorder with panic, personality disorder, and “borderline intelligence.” He had several admissions to a psychiatric ward over the past 3 years for low mood and had been trialed on numerous psychotropic medications (citalopram, lithium carbonate, risperidone, olanzapine, quetiapine, paliperidone, clomipramine, clonazepam, lorazepam) with little effect or benefit. At the time of admission, he did not smoke, drink alcohol, or take illicit drugs. His past medical history was significant for hypothyroidism corrected with the use of thyroxine, bowel resections secondary to possible malignant changes, fatty liver with lobar resection secondary to liver cancer and nephrolithiasis.\nHe was born and raised in Europe until the age of 5, when he immigrated to Canada, and is bilingual. His family reported that he had always had a large head, micropenis, central obesity and short stature. He had a history of being bullied for “looking like a girl” and being different. At school his peers were physically aggressive, hitting him on his head. Born the youngest of seven siblings, he was raised by his parents and lived under their care into adulthood, until both parents passed away—his father had Diabetes Miletus and his mother had a brain tumor. Thereafter, he was taken care of by his sister. He had an older brother who also passed away secondary to a brain malignancy. One brother has dyslipidemia, and two sisters and one brother are healthy. He had no employment history and as a child had always struggled in school, completing a vocational stream of education until grade 10. Socially, he was active in a band for a few years (plays guitar well) and sang in a church choir. However, he never lived independently, and had no romantic relationships.\nInitial assessment revealed that he was a poor historian unable to give an accurate timeline of events. He often expressed fears that he was going to die. He suffered from delusions of guilt that he had caused the deaths of family members. His conversation was repetitive, he repeatedly asked the same questions and restated his fear of dying despite several reassurances. He had no history of self-harm or suicide attempts. On physical examination, he had a wide stance waddling gait, slow movements, limited arm swing and masked facies. He was noted to have enlarged head circumference (62.5 cm) and limited insight into his illness and the need for treatment. His clinical presentation prompted examination with magnetic resonance imagining (MRI) of the brain and formal neuropsychological testing.\nA sagittal T1, axial T2, axial T2 FLAIR and diffusion-weighted images were acquired throughout the brain. Findings indicated a long-standing overt ventriculomegaly, likely due to aqueductal stenosis, with bilateral gross dilation of the lateral and third ventricles, with a small aqueduct and fourth ventricle, with significant thinning of the corpus callosum and overlying cerebral cortex. Vascular flow-voids at the base of the brain were normal and there were no mass lesions, significant sulcal effacement, downward tonsillar herniation or restricted diffusion observed.\nManual segmentation of gray and white matter and cerebrospinal fluid (CSF; Figure ) of high-resolution T1 weighted MRI images was completed with Freeviewer in FSL (Jenkinson et al., ). Automatic segmentation of a comparison group of sex and age matched healthy controls (HCs; one aged 60, three aged 55 years, Table ) was completed with the FreeSurfer () recon tool. The participant’s volumes were converted to Z scores for comparison. Compared to similarly aged control participants, the patient had extremely large ventricular volume (821,452 mm3, Z = 161), reduced white (333,606 mm3, Z = −2.655) and gray (432,184 mm3, Z = −3.07) matter volume, and within normal range total intracranial volume (1,587,242 mm3, Z = 0.57) see Table and Figure .\nThe patient’s neurological exam was unremarkable.\nThe Wechsler Adult Intelligence Scale (WAIS-III; Wechsler, ) revealed a borderline IQ of 79, with a verbal IQ of 88, non-verbal performance IQ of 74, poor working memory IQ of 71, verbal comprehension IQ of 93, and visual-spatial IQ of 80. The patient had difficulty completing tasks requiring working memory, which was in the 3rd percentile, and processing speed was extremely slow (in the 1st percentile). Hopkins Auditory Verbal Learning Test (Brandt, ) indicated severe memory impairment, with initial memory for only a few items, no significant recall between administrations, and inability to recall any information after a brief delay. Rey-Osterrieth Complex Figure Task (Osterrieth, ; Rey et al., ) performance indicated impaired visual spatial and working memory abilities with more attention to small details, missing elements and less attention to the overall image. The Stroop test (Stroop, ) indicated impaired executive function, scoring below the 1st percentile, with a severe inability to suppress automatic responses. |
A 44-year-old woman with a history of metastatic triple negative breast cancer and lung metastases presented with a six-month history of recurrent haemoptysis. She had no other significant medical history. She was initially managed for her right breast cancer with a wide local incision and adjuvant chemoradiotherapy in 2014; however, her malignancy recurred two years later. She had positive margins on subsequent right mastectomy and proceeded to excision of the right pectoralis major and overlying dermis. Six months later she was found to have bilateral pulmonary metastases and underwent initial diagnostic bronchoscopy identifying a bleeding mass in the medial segment of the right middle lobe (RB5), which was subsequently treated with topical adrenaline and biopsied – confirming metastatic disease. Her malignancy progressed despite palliative chemotherapy with epirubicin and cyclophosphamide, during which time she developed worsening haemoptysis of ~1/2 cup (~120 mL) daily. A multidisciplinary decision was then made to perform therapeutic bronchoscopy due to excessive distress caused to the patient because of haemoptysis. She underwent bronchoscopy using a therapeutic video bronchoscope (Olympus BF-TH190, Olympus Corporation, Tokyo, Japan) introduced via a rigid bronchoscope, which provided secure airway access. Endobronchial survey revealed the source of bleeding in the distal right middle lobe, although the actual bleeding source was not directly visible. A volume of 2 mL of TISSEEL was injected into the right middle lobe bronchus via a catheter followed by deployment of a size 6 Spiration (Redmond, WA, USA) IBV to add stability and prevent expectoration (Fig. ). A further 1 mL of TISSEEL was then applied over the valve (Fig. ). The procedure abolished the patient's haemoptysis instantly.\nTwo weeks later the patient developed recurrent haemoptysis; however, repeat bronchoscopy showed a different source of bleeding in the right lower lobe, with the existing combination TISSEEL and IBV still in place in right middle lobe and maintaining haemostasis. To control the new bleeding TISSEEL was injected in the right lower lobe bronchus distal to the opening of RB6, followed by deployment of a size 9 IBV. Further injection of TISSEEL was then applied and haemostasis was achieved. Unfortunately the patient was found to have brain metastases and died of her malignancy 10 weeks later, without recurrence of haemoptysis. |
A 12-year-old girl who had recently immigrated to New York City from Ecuador presented with an 8-month history of a recurring, intensely pruritic rash on her left thigh. She and her family reported that the rash had first developed within a week of their arrival in the United States and that it had appeared three times over the course of the last 8 months, with the most recent occurrence lasting for the 3 months prior to presentation. The rash always appeared the same and occurred in the same place. Neither the patient nor the family were aware of any obvious inciting factors. Both patient and family denied any use of medications, supplements or vitamins in the previous year. Review of systems was negative for accompanying fevers, chills, night sweats or weight loss.\nOn physical examination, there was a 6-cm well-circumscribed annular plaque with a dusky center, a central collapsed bulla and an erythematous border on her left lateral thigh a 6 cm (fig. ). A 3-mm punch biopsy was performed at the periphery of the lesion. Histopathology demonstrated numerous keratinocyte necroses along with a lymphocytic perivascular infiltrate (fig. ), consistent with the clinical diagnosis of a FDE.\nThe patient was instructed to apply alclometasone 0.05% cream to the lesion twice a day for 1 week. Given the lack of an obvious causative agent, the family was recommended to thoroughly review new dietary changes since emigration, particularly ingredients in processed foods and foods containing artificial coloring. At the follow-up visit 1 week later, the plaque was no longer pruritic and appeared xerotic with pink re-epithelialization at the site of the former bulla, suggesting that the lesion was beginning to resolve. After a diligent exploration, the family reported one significant dietary alteration since arriving in the United States: whereas they had previously made yellow rice using natural achiote, or annatto dye, they had switched to a commercially available achiote dye and spice mixture. On review of the ingredient label of this product, the mixture was found to contain the synthetic coloring agent Yellow 5 (tartrazine). The patient was advised to discontinue use of this product. She reported no recurrence at 4-month follow-up. |
In a 19-year-old, mentally disabled male, chest radiography was done because of a sudden episode of cough. Metallic, hook-shaped foreign bodies were identified in both the main bronchi.\nThe right-sided FB was removed by fiber-optic bronchoscopy in the regional hospital, whilst the left-sided extraction failed with the left-sided FB persisting in the left main bronchus ().\nUpon urgent admission in a tertiary institution, extraction was attempted by rigid bronchoscope under general anesthesia. Bronchoscopic extraction failed, associated with some bleeding and subcutaneous emphysema immediately after the intervention. The increasing mediastinal and subcutaneous emphysema raised suspicion about an iatrogenic airway lesion, so surgery was indicated. Esophageal injury was previously ruled out by esophagoscopy, revealing many metallic FBs in the stomach. At thoracotomy, a significant mediastinal emphysema (), together with diffuse adhesions, was noticed. After the lung liberation, a proximal 0.5 cm of the noncurved part of the metallic hook was found to protrude through the perforated membranous wall of the left main bronchus, 1 cm away from the descending aorta (, arrow). The part of the FB protruding outside the bronchus was grasped by the clamp and, by following the curved shape of the FB, gentle maneuvers were applied by pulling the sharp end (hook) of the FB in the direction outside the bronchus. The FB was removed from the bronchus (insert on ) without the need for additional bronchotomy. The remaining 10 × 1 mm defect in the bronchial wall, caused both by manipulations during a bronchoscopic extraction attempt and subsequent surgical extraction, was sutured by interrupted PDS 3-0 stitches, and the lung fully inflated. No air leaks appeared during the water test. Having in mind the dimensions of the defect and tensionless suture, no suture-line protection was performed.\nAfter the thoracotomy closure, laparotomy was done and several sharp metallic pieces of different shapes were removed from the stomach (Figures and ). This was followed by an uneventful postoperative course and the discharge after 14 days.\nAfter three years, the patient was urgently readmitted for the new episode of the metallic foreign body aspiration (Figures and ). The abdominal radiography revealed metallic pieces in the digestive tract as well (). With the surgical team on site, rigid bronchoscopy under general anesthesia was done. As the tip of the FB was not impacted in the mucosa, it was possible to grasp it with the rigid biopsy forceps and to withdraw it up to the tip of the bronchoscope. Because of the curved shape of the FB and the impossibility to remove it through the instrument, the FB and bronchoscope were pulled out from the patient together, with the FB firmly grasped, followed by reintubation with the same bronchoscope (Figures and ). After a careful check-up for bleeding and mucosal damage, the patient was extubated.\nMetallic pieces left the digestive tract spontaneously after a couple of days. |
A 23-year-old woman presented to our hospital with a mass in her left axilla. The mass had
increased in size over the preceding 14 months. The patient was counselled for the first
time by her family doctor to undergo investigation, such as ultrasound or magnetic resonance
imaging (MRI), to obtain a differential diagnosis, but she refused because she thought it
was not necessary. Six months later, the mass was slightly larger compared with the initial
size. She still believed that it was a mammae erraticae and did not undergo any
examinations. Two months before visiting our hospital, she realized that the situation was
worse than what she thought because the contour of the mass could be seen on the skin
surface. She was then referred to the galactophore department and underwent MRI
examination.
There was no pain or paraesthesia in the involved area. Moreover, no associated family
history or clinical features were recorded. Physical findings on the initial examination
indicated a solid ovoid movable mass in the left axilla, measuring 12 cm in length and 8 cm
in width. The mass was partly covered by the pectoralis major muscle. Regular preoperative
blood tests showed normal results. MRI revealed that the mass was subcutaneous and
homogeneous (). With a
clinical diagnosis of a nerve tumor and a preoperative anaesthetist assessment, the patient
underwent surgical excision of the tumor. We explained to the patient that nerve grafting
might be performed if the involved nerve could not be preserved. The tumor arose from a
nerve in the left axilla. It was well encapsulated and located eccentrically to the nerve
trunk (). The involved nerve
was identified as the long thoracic nerve based on the electrical stimulation response.
Because it was not possible to use a tourniquet, enucleation was performed under loupe
magnification to protect the nerve during the dissection procedure. A longitudinal |
A 63-year-old non-hypertensive diabetic male, farmer by profession, presented with complaints of blackish discoloration of distal part of the left foot for 15 days predominantly involving four small toes. He had similar blackish discoloration of left great toe for last one month, which was amputated 15 days back in a local hospital. He also complained of ulcerative wound over the distal part of left foot and amputated great toe (Figure ). He was diagnosed as a case of uncontrolled type-2 diabetes mellitus with poor glycemic control on irregular oral hypoglycemics for five years. He had a history of claudication pain in lower limbs for the last one year, which gradually progresses to pain even at rest and loss of sensation of digits for the last three months. He is a chronic smoker and alcoholic for the past 20 years. There was no history of fever, pus discharge from the distal lower limb, chest pain, blurring of vision, blackouts, or abdominal pain.\nOn clinical examination, there were no signs of pallor, icterus, pedal edema, lymphadenopathy, clubbing, or cyanosis with random blood glucose of 344 mg/dl. Extensive ulceration and necrosis were present over the ankle, heel, and great toe region of left lower limb along with blackish discoloration of second to fifth toes. The peripheral arterial pulses were not palpable in all major peripheral arteries of both lower limbs. Computed tomograghy (CT) angiogram of lower limbs showed high-grade occlusion and luminal narrowing in almost all major arteries of both lower limbs. The provisional diagnosis was made as bilateral lower limb peripheral vascular disease due to uncontrolled diabetes mellitus. The patient was planned for above-knee amputation of left lower limb.\nThe patient had developed few episodes of mild grade fever, for which automated blood culture was collected and sent to the microbiology laboratory. The bottle was loaded in BacT/Alert Virtuo system (BioMérieux, Marcy-l'Étoile, France), which flagged positive signal after 26 hours of aerobic incubation at 37oC. Gram staining was performed directly from flagged broth, which showed short gram-positive bacilli arranged in pairs with acute angular fashion (cuneiform arrangement) as shown in Figure .\nSubculture from bottle was performed on 5% sheep blood agar (SBA), chocolate agar (CA), and MacConkey agar (MAC) and incubated aerobically at 37oC. After 10 to 12 hours of incubation, minute grayish circular low-convex hemolytic colonies were seen on SBA (Figure ). Gram staining from the colonies grown on SBA showed short gram-positive bacilli in cuneiform arrangement with swelling on one or both ends (club-shaped). This is followed by Albert staining from the colony, which showed green-colored bacilli with bluish-purple metachromatic granules in cuneiform arrangement. Identification of the organism from the colony was obtained using matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) (VITEK MS version 3.0). It was identified as C. diphtheriae with a confidence interval of 99%. Later, the identification was also confirmed by conventional method.\nUrea was not hydrolyzed; it produced black-colored colonies on potassium tellurite blood agar (KTBA) and fermented glucose, maltose, and starch with production of acid but no gas on Hiss’s serum sugar fermentation media (Figure ). The isolate was identified as “gravis” biotype. Antimicrobial susceptibility testing (AST) was performed by broth microdilution method using VITEK-2-automated AST system (AST-P628 card for gram-positive cocci). The result was interpreted using clinical breakpoints given in Clinical and Laboratory Standards Institute (CLSI) M-45 document []. The isolate was susceptible for benzylpenicillin, erythromycin, gentamicin, vancomycin, clindamycin, linezolid, and rifampicin, intermediate to ciprofloxacin and resistant to cotrimoxazole and tetracycline. In-house conventional PCR for tox gene (tox A and tox B) and dtxR gene was performed from the culture growth. The isolate was negative for tox genes but positive for dtxR gene, which confirms that the isolate belongs to a non-toxigenic strain of C. diphtheriae, biotype gravis.\nFollowing amputation, intra-operative necrotic tissue bits were sent for bacterial culture, which grew Providencia rettgeri and Enterococcus faecalis. The laboratory failed to show any growth of C. diphtheriae from tissue bit, possibly due to heavy gram-negative bacilli growth. Subsequently, a culture of throat swab specimen was also performed to find out any colonization, which failed to have any growth of C. diphtheriae. Therefore, the source of C. diphtheriae bacteremia was not properly established. The patient was empirically started on amikacin as pre-surgical prophylaxis since admission and subsequently changed to amoxycillin-clavulanate and clindamycin based on the AST report. The patient improved clinically and was discharged on the same regimen. |
A 35-year-old male with a past medical history only significant for hypertension and a family history significant for inflammatory bowel disease (IBD) and colon cancer in a maternal grandmother presented to the emergency department with complaints of chest pain, shortness of breath, and unintentional 30 pound weight loss for 3 months. Workup identified a 7.6 × 2.6 cm anterior mediastinal mass with associated lymphadenopathy seen on CT imaging with no lesions noted elsewhere (). Differential diagnosis for the anterior mediastinal mass consisted of both benign etiologies such as thymoma or thymic cyst and malignant etiologies such as lymphoma, primary germ cell tumor, or a metastatic lesion.\nThe patient underwent a robotically assisted thymectomy via a left thoracoscopic approach by Cardiothoracic Surgery. The mediastinal mass was noted to involve the thymus under thoracoscopy and was partially resected due to extension into the right thorax. Pathology from the lesion revealed a poorly differentiated adenocarcinoma with enteric histomorphology and >5 positive lymph nodes demonstrating extranodal extension with prominent lymphovascular invasion. No residual thymic tissue was identified. The pathologists subjected the specimen to various immunophenotypic stains (). The adenocarcinoma of the anterior mediastinum was diffusely positive for CDX2, CK20, and β-catenin in addition to CK7 positivity. The tumor was negative for TTF-1, PAX8, cKIT, and SALL4, markers for lung adenocarcinoma, thyroid carcinoma, thymic carcinoma, and germ cell tumor, as would be expected from thymic tissue. We were unable to definitively identify whether the mass represented a rare primary thymic adenocarcinoma arising within a thymic cyst or a metastatic lesion giving this clinical presentation and the young age of the patient. The anterior mediastinal tumor was microsatellite stable (MSS) on microsatellite instability testing. Genetic mutation analysis by next generation (Ion Torrent) sequencing on formalin-fixed paraffin-embedded tumor tissue (Department of Anatomical Pathology, University of Florida, Gainesville) revealed that the adenocarcinoma in the anterior mediastinum harbored mutations in KRAS (c.38G>A), SMAD4 (c.1081C>T), and MET (c.2962C>T). A consensus was achieved that this was probably a metastatic lesion, likely colonic in origin, given the aberrant nuclear expression of β-catenin frequently seen in colonic-derived adenocarcinomas.\nA search for a potential occult primary malignancy was conducted with a positron emission tomography scan (PET/CT) along with upper and lower endoscopy. PET scan revealed a residual fluorodeoxyglucose (FDG) avid lesion in the anterior mediastinum and a lesion in the right colon near the hepatic flexure. Endoscopy identified a small, nonbleeding, ulcerated lesion in the duodenum as well as a large, nonobstructing ascending colon mass (). Biopsies confirmed a primary colonic malignancy and a focus of metastatic adenocarcinoma limited to the lymphovascular channels in the mucosal biopsy taken from the duodenum. No precursor lesion (i.e., adenoma) was identified in the duodenal biopsy and the consensus was that this was likely a lymphovascular metastatic deposit from the colonic primary tumor. Carcinoembryonic antigen (CEA) was within normal limits at 1.3 ng/mL.\nThe case was presented at a multidisciplinary tumor board and a consensus was developed to proceed with resection of the primary colonic malignancy followed by systemic chemotherapy. The patient underwent an uncomplicated laparoscopic right hemicolectomy () by Colorectal Surgery with an uneventful recovery. Final pathology of the colonic specimen revealed an invasive, poorly differentiated, adenocarcinoma arising from a tubular adenoma with high grade dysplasia and thirteen of twenty-eight lymph nodes positive for metastatic adenocarcinoma. Despite aggressive en bloc resection of the pericolonic tissue the pathology showed a positive radial margin giving a final pathologic stage of pT4apN2bpM1. Morphologically and genetically, the colonic adenocarcinoma was identical to the resected adenocarcinoma in the anterior mediastinum (). The overall prognosis for the patient is poor and given the final pathologic and genetic test results, the patient is going to continue on palliative chemotherapy with FOLFOX-bevacizumab. |
A 68-year-old Asian woman, 145 cm in height and 45 kg in weight, with a compression fracture of the third lumbar vertebra was admitted for vertebroplasty. Her previous medical history included osteoporosis, and her previous surgical history included appendectomy, removal of a thyroid nodule, and recent repair of the right rotator cuff under general anesthesia. All of the preoperative evaluations were unremarkable except diastolic dysfunction grade I in preoperative echocardiography.\nAfter applying the standard patient monitoring system, including electrocardiography, noninvasive measurement of blood pressure, and pulse oximetry, vertebroplasty was performed with the patient in a prone position. To relieve anxiety, sedation was induced with sevoflurane 8 vol % and oxygen 3L/min via mask ventilation for 2 minutes. Sedation was maintained with sevoflurane of 2~3 vol % with the patient’s head turned sideways. End tidal carbon dioxide (ETCO2) was monitored while maintaining spontaneous ventilation.\nDuring vertebroplasty, 6 ml of bone cement (V-STEADY, Sungwon Medical, Korea) was injected through a hollow needle into the fractured bone. There were no noticeable events during the procedure.\nAfter the procedure, the patient was transferred to the postanesthesia care unit, at which sudden hemoptysis occurred. The amount of hemoptysis could not be measured in exact scales, but the amount of frank bleeding was enough to have drenched 4 to 5 sheets of (4 x 4) gauze. As the patient began to show complaints of dyspnea, oxygen saturation was as low as 80 %. After application of 5 liters of oxygen via facial mask, the oxygen saturation showed recovery up to 93%. The vital signs at that time were stable, and no symptoms other than dyspnea complained. Coarse breathing sounds were heard in both lung field. In the postoperative chest x-ray taken immediately afterwards, newly formed perihilar consolidation and air bronchograms which were not present in the preoperative radiograph images were observed in both lung fields (Fig. ).\nWhile the patient insisted that her symptoms were tolerable, she was transferred to a tertiary medical institution for further evaluation. The vital signs at the time of transfer were stable; body temperature of 36.8 °C, pulse rate 82 beats/min, respiration 20/min, and blood pressure 120 / 75 mmHg. The oxygen saturation was 97%. The computed tomography (CT) findings were as stated: diffuse ground glass opacity and interlobular septal thickening in both lungs central portion (Fig. ). Bronchoscopy was not performed because the symptoms improved and the patient did not want it.\nIn comparison to the preoperative hemoglobin level of 10.4 g/dL, hemoglobin level decreased to 8.5 mg 2 days after admission. The patient received supportive care and discharged without further events. The patient requested an outpatient clinic follow up, but did not visit until 6 months of discharge. |
Informed consent was obtained from the patient. A 22-year-old para 2 at 15 weeks gestation with dichorionic diamniotic twins was transferred to our hospital after several days of intractable vomiting and 30 lbs weight loss over the past few months despite the pregnancy. Her past medical history was significant for two years of frequent vomiting. Chest X-ray revealed pneumomediastinum without any respiratory distress. A barium esophagogram was negative for esophageal perforation but was consistent with esophageal achalasia (). A manometry study confirmed the diagnosis of esophageal achalasia, and esophagogastroduodenoscopy showed Candida esophagitis. This was treated with intravenous Fluconazole for 2 weeks which significantly improved her oral intake and weight, allowing for conservative management.\nShe was readmitted during the 20th week with further weight loss and malnutrition. Total parenteral nutrition (TPN) was started, and laparoscopic lower esophageal myotomy was scheduled. During the attempted induction of anesthesia with cricoid pressure, the patient aspirated esophageal contents and required intensive unit care for aspiration pneumonia. Insertion of a Dophoff tube was attempted for continued gastrointestinal nutrition. Two attempts failed because the tube could not be passed through the LES but coiled in the large dilated esophagus (). CT scan confirmed the presence of pneumomediastinum (), and so pneumatic dilation or Botulinium injections were not considered. The patient declined the passage of the tube under endoscopic guidance, or any further surgical treatment. She was started on oral Nifedipine before meals which helped her gain weight.\nAlthough she gained 15 lbs at home, she was readmitted during the 29th week for intrauterine growth restriction of the twins. Intravenous hyperalimentation was reinitiated, and a Cesarean section was scheduled for the 34th week of her pregnancy. On the morning of the scheduled delivery, intrauterine fetal demise of 1 twin was diagnosed. The other fetus was delivered via Cesarean section later that day. The male baby weighed 1529 grams (<10th percentile) and required basic neonatal care for 2 weeks in the hospital. Robotic thorascopic-assisted Heller esophagomyotomy with a flexible esophagoscopy was performed successfully 5 months postpartum. She did well postoperatively and gained significant weight thereafter.\nSix months later she conceived again and presented at 25 weeks gestation with recurrent intractable vomiting for 2 weeks and 12 lbs weight loss. Esophagogastroduodenoscopy revealed a duodenal ulcer and small hiatal hernia. The hernia was suspected to be a complication of the myotomy surgery <1 year ago. Conservative management including proton-pump inhibitors was initiated. Ten days later, the patient started to experience severe LUQ, left shoulder pain, and mild respiratory distress. A chest CT revealed a large hiatal diaphragmatic hernia with loops of bowel that were compressing the base of the left lung (). Nasogastric tube was inserted to decompress the bowel and possibly decrease her respiratory symptoms, allowing her pregnancy to advance further. Surgeons did not feel the diaphragmatic repair could safely be performed in the gravid state due to the large amount of intestines herniated into the chest. Due to worsening respiratory symptoms and compression atelectasis over the next 10 days, she was given betamethasone for fetal lung maturity, and a repeat Cesarean section with bilateral tubal ligation was performed at 28-week gestation. A male baby weighing 1372 grams (24th percentile) was delivered. Two days postpartum, the mother underwent uneventful repair for the diaphragmatic hernia. The infant required about 8 weeks in neonatal intensive unit care and developed mild bronchopulmonary dysplasia and patent ductus arteriosus that was later ligated. The mother and the baby were doing well 4 years later. |
This case report presents a five-year-old, otherwise healthy female who was seen in the pediatric ophthalmology clinic for the chief complaint of right eye drifting outward. This occurred especially at distance fixation and was first noticed when the patient was 10 months old. Other significant ocular history included a high refractive error and eyeglasses wear. There was a family history of strabismus in the patient’s maternal uncle. On examination, the patient’s visual acuity was 20/200 in her right eye and 20/40 in her left eye. She was found to have intermittent monocular exotropia of the right eye. She was also found to have a high refractive error in her right eye (-6.50 diopters). The slit lamp examination was normal bilaterally. The dilated fundus examination of the right eye revealed flat, white patches in the retina (superotemporal and inferotemporal regions) in a distribution consistent with a MRNF layer (Figure ). The left eye, on the other hand, had a normal dilated fundus examination (Figure ).\nThe patient was therefore diagnosed with strabismus (intermittent exotropia), high myopia, and amblyopia, as well as MRNF layer of the right eye.\nIn the patient, the exotropia was a large-sized deviation that was poorly controlled at distance fixation. One year after the initial presentation, she required eye muscle surgery to realign the right eye. The patient was also found to have high myopia in the affected eye. This refractive error caused blurry vision and contributed to her initial visual acuity of 20/200 and the subsequent onset of amblyopia and strabismus. The refractive error was corrected with eyeglasses. Amblyopia treatment was continued with patching of the left eye four hours daily to force the brain to develop vision in the affected right eye.\nWhile patching her left eye, the child was noticed to adopt a head tilt to use her temporal field of vision in the right eye. There was no anomalous head posture noted when not patched and permitted to use both eyes. Because the myelinated nerve fibers cause a blind spot in the affected area, this observed posture was consistent with a nasal visual field defect in the right eye, corresponding to the temporal retinal lesions.\nThe patient was followed in the pediatric ophthalmology clinic for three years. With the above treatment of strabismus surgery, eyeglasses wear, and patching, the right eye visual acuity improved from 20/200 to 20/50. Her left eye also improved from 20/40 to 20/25. She will continue close follow-up with maintenance patching until at least she is 10 years of age to ensure best potential vision for the affected right eye. |
A 71-year old woman was admitted to the hospital in November 2018. The clinical examination showed no abnormalities. Biochemical parameters in blood showed normal values apart from slightly elevated gamma-glutamyl-transferase (GGT) (160 U/l, normal up to 40U/l). She never smoked and had no family history of lung or gastrointestinal cancers. She developed dry cough over the last 6 weeks, which was resistant to treat. Therefore a chest X-ray was done, which showed an irregular left border of the heart. A subsequent chest CT-scan showed a paracardiac nodule with 2 × 1.8 cm in diameter (Fig. ), no mediastinal lymph nodes enlargement and no pleural effusions were detected.\nA bronchoscopic examination with bronchial lavage was done. The lavage revealed acid proof rods, which were immediately tested for M. tuberculosis by quantiferon screening. Since the medical report was negative for tuberculosis, surgery was performed for histological diagnosis.\nThe exploration of the entire hemithorax left showed massive dorso-basal adhesions between the lower lobe and the thoracic wall, as well as the diaphragm. After adhesiolysis, the tumor within the lingual segment was exposed, biopsied and a histopathological frozen section examination was performed, which showed malignancy. The tumor was then anatomically resected using video assisted thoracic surgery (VATS) to remove both segments of the lingual. Complete mediastinal lymph node dissection was done. Histopathological analysis of the removed tissue indicated a neuroendocrine neoplasm, which was confirmed by immunohistochemistry. In particular, cancerous tissue was positive for synaptophysin (Fig. ), chromogranin A as well as high and low molecular weight cytokeratins detected by the antibody combination of AE1/AE3. Based on the absence of the epithelial marker TTF1 as well as the neuroendocrine markers CDX2 and cytokeratin 20 of the gastrointestinal tract, LCNEC, SCLC or a metastasis of the gastrointestinal tract could be excluded. Neither an apparent necrosis within the tissue, nor pathologic lymph node structures were observed. Further analysis revealed only 1% to 2% Ki67-positive mitotic cells within the tumor, resulting in the final classification of a well-differentiated, low-grade typical carcinoid (TC) in stage IA. Therefore, no adjuvant therapy was suggested. The patient was discharged on the fifth day after surgery in a good general condition. 6 months follow up showed no abnormalities. The somatostatin receptor imaging with 68G DOTATATE PET/CT- showed no abnormal findings.\nNext to a histopathological analysis, a part of the resected typical carcinoid was used for the attempt to cultivate and characterize cancer stem cells. Informed consent according to local and international guidelines was signed and all further experimental procedures were ethically approved (Ethics committee Münster, Germany, 2017–522-f-S). For the isolation of the CSCs the specimen was washed twice with ice-cold phosphate buffered saline (PBS), mechanically disintegrated in 2 to 5 mm pieces followed by an enzymatically digestion with collagenase for 2 hours at 37°C. One half of the minced tissue was used to cultivate spheres in Dulbecco modified Eagle's medium/Ham's F-12 with addition of 200 mM L-Glutamin, epidermal growth factor (EGF; 20 ng/mL), basic fibroblast growth factor (bFGF/FGF-2; 40 ng/mL) and B27 supplement in low adhesion T25 tissue culture flasks (Fig. B). The other half of the tissue was used to grow adherent CSCs, where the cells were cultivated on gelatin coated culture dishes in the medium described above supplemented with 10% fetal calf serum (Fig. A).\nAfter successful cultivation, cells were analyzed according to their expression profile of cancer stem cell and neuroendocrine specific markers, as well as their morphology. Immunocytochemical double staining of the lung cancer stem cell markers CD133 and CD44 confirmed the isolation of cancer stem cells (Fig. C). Additionally, cultivated cells were positive for the neuroendocrine marker synaptophysin underscoring the establishment of the relevant cancer stem cells. Synaptophysin was especially localized within the nucleus of the cells, although some cells also revealed synaptophysin within their cytoplasm (Fig. E). Next to the expression of synaptophysin in the isolated neuroendocrine cancer stem cells (Fig. E, 4C), we detected synaptophysin in neural crest-derived stem cells from the nasal cavity of a female donor[ (Fig. A) and female adipose tissue-derived mesenchymal stem cells (Fig. B), suggesting a new role of synaptophysin as a stem cell marker. Quantification of the nuclear fluorescence intensity of synaptophysin within the different stem cells revealed a significant higher expression within the isolated BKZ1 cell line in comparison to non-pathogenic stem cells (Fig. D). Furthermore, cells expressed the primitive neuroectoderm and stem cell marker nestin, underlining the stem cell characteristics and suggesting a neural crest origin of the cultivated cells (Fig. D). Due to the strong association of NF-κB with chronic inflammation and different cancer types, TC-derived BKZ1 cells were analyzed according to their NF-κB expression. Immunocytochemical staining of the subunit RELA (p65) displayed a high perinuclear expression of the cultured cells (Fig. F). |
A 72-year-old male presented to the emergency department with complaints of four-day history of epigastric abdominal pain, nausea, vomiting, constipation, and inability to tolerate oral diet. He reported early satiety and 24 pounds of unintentional weight loss in the last four months. He was hemodynamical stable on presentation. Abdominal examination revealed mild epigastric tenderness with moderate abdominal distention. The basic laboratory workup was within normal limits. However, computed tomography (CT) abdomen and pelvis with intravenous and oral contrast showed eccentric thickening of the third and fourth part of the duodenum (). A magnetic resonance imaging showed a heterogeneous mass in the duodenum measuring 13.1 × 5.9 × 7.5 cm (). An esophagogastroduodenoscopy (EGD) was performed to obtain the biopsy of the mass which turned out to be adenocarcinoma of the small bowel (Figures –). He underwent open distal duodenectomy with extensive small bowel resection. His postoperative course was complicated by fever, hypotension, tachycardia, and persistent abdominal pain. A follow-up CT scan of the abdomen revealed an anastomotic leak at the surgical site (). Although the patient was started on complete bowel rest, TPN, and broad-spectrum antibiotics, his clinical condition continued to deteriorate. Two 8 French pigtail drainage catheters were placed around the anastomosis leak which continued to have high output over the next few days (). A follow-up CT revealed a persistent anastomotic leak with the formation of an enterocutaneous fistula involving the duodenum. A repeat EGD was performed, and the tip of one of the catheter was found in the lumen of the duodenum forming a fistulous tract (Figures –). The catheter was removed, and we applied argon plasma coagulation (APC) through the fistulous tract and around the edges to allow de-epithelialization and granulation tissue formation. The closure of the tract was achieved by placing over the scope hemoclips (). A follow-up CT scan of the abdomen and pelvis showed resolution of the anastomotic leak (). The patient's clinical condition markedly improved and was transferred out of the ICU and later discharged home. |
A 43-year-old Caucasian female initially presented to her primary care physician (PCP) with the chief complaint of progressive weakness of symmetric upper and lower proximal limbs for 4-5 weeks. Symptoms started suddenly and progressively worsened, and she was also experiencing diffuse muscle aches. The patient was having difficulties climbing stairs, standing from a sitting position, and combing her hair. The patient had type 2 diabetes mellitus, hyperlipidemia, and migraine headaches. She had been on atorvastatin, 40 mg daily, for almost five years. Initial outpatient lab work showed an elevated CPK level of 8000 IU/L and the patient was referred to the ED by her PCP for further evaluation. Atorvastatin was discontinued. The patient was started on intravenous hydration for possible rhabdomyolysis. The patient’s symptoms did not improve after aggressive hydration in the hospital, and the CPK level remained persistently elevated above 7000 IU/L. An MRI of proximal lower extremities showed diffuse, symmetric proximal lower extremity muscle edema. MRI also did not show significant fatty atrophy of posterior thigh muscles and this was indicative of acute myopathy (Figures and 2).\nClinical symptoms of significant muscle weakness, very high CPK level, and abnormal MRI findings initially raised suspicion for inflammatory myositis. To rule out inflammatory myositis, a muscle biopsy was performed. The patient was empirically started on 60 mg of prednisone daily for suspected inflammatory myositis pending biopsy result. The muscle biopsy report was suggestive of statin-induced myopathy. Moreover, the subsequent workup showed positive antinuclear antibodies (ANA), negative extractable nuclear antigens (ENAs), and very high HMGCR antibodies (titer > 200). The patient was diagnosed with statin-associated IMNM. She reported resolution of muscle achiness, but no improvement of weakness with corticosteroid treatment. Due to a lack of adequate response to high-dose corticosteroids for eight weeks, she was started on IVIG 2 gram per kilogram of body weight per month for three months. In outpatient follow-up, the patient reported resolution of all muscle symptoms, CPK had been normalized and the patient did not require any further immunosuppressive drugs. |
A previously healthy, 52-year-old Caucasian man presented to his family physician a week after having a tonic-clonic seizure. A magnetic resonance imaging (MRI) scan showed a 10 cm left frontal tumor, which was confirmed as an atypical meningioma following craniotomy and resection (Figure ).\nPostoperatively, he took 400 mg of phenytoin PO once a day. He had no seizures postoperatively or afterward. The patient uneventfully received 60 Gy of adjuvant radiation therapy to the postoperative bed in 30 fractions. Three months after the resection of the tumor, the patient began a trial of phenytoin but nine days later, he developed symptoms consistent with a generalized seizure. He resumed his daily phenytoin prophylaxis with good effect.\nTwo months later, he complained of blood in the stool and after an evaluation was diagnosed with a locally advanced nonmetastatic adenocarcinoma of the low rectum (Figure ). A curative-intent dose of 50.4 Gy in 28 fractions of neoadjuvant radiation therapy was prescribed, with 2000 mg PO BID of concurrent radiosensitizing capecitabine []. After 20 of the planned 28 fractions, he began to feel unwell and experienced new, right-sided upper and lower limb dysfunction and an unsteady gait. A contrast-enhanced computed tomography (CT) scan of the brain showed no suspicious findings but his phenytoin level was dramatically elevated at 138 µmol/L, compared to 49 µmol/L just prior to neoadjuvant therapy (normal range: 40-80 µmol/L). His albumin level from a few weeks prior to these symptoms had also been normal at 39 g/L (normal range: 34-46 g/L), and he was taking no other medications other than an occasional stimulant laxative. Capecitabine was discontinued, and the patient was treated with charcoal and admitted for observation. Phenytoin was temporarily discontinued and then reintroduced at the previous dose of 400 mg PO per day once levels began to normalize. His symptoms quickly resolved and he showed no further toxicity.\nHe resumed radiation therapy a few days later without concurrent capecitabine. It was believed that he had developed phenytoin toxicity secondary to impaired clearance as a result of his capecitabine. His phenytoin levels were monitored during the following weeks and his phenytoin dose was bridged with lacosamide and titrated down gradually and then discontinued, with no further symptoms of toxicity. The patient remained on 200 mg PO per day of lacosamide. He underwent a surgical resection with clear margins followed by adjuvant capecitabine and showed no signs of a recurrence of rectal adenocarcinoma thereafter. Three years later, the patient passed away from recurrent meningioma. |
A 60 year old woman presented with a twelve month history of fatigue, anorexia, weight loss and abdominal distension. She had a history of well controlled hypertension and type II diabetes mellitus.\nLiver function tests were slightly abnormal (ALP: 383IU/l; GGT: 216IU/l). CT of the abdomen demonstrated a large pancreatic mass (13 × 9 × 5 cm) compressing the confluence of the portal and superior mesenteric veins (Figure ). The right colon and antrum of the stomach also appeared to be intimately involved with the tumour. Additionally, a 12 cm diameter mixed cystic/solid mass was noted to occupy the majority of the right hemi-liver (Figure ). Her serum CgA level was elevated at 507IU/l (range: 0-17.2) and an octreotide scan showed avid uptake within the pancreatic mass and within the periphery of the liver lesion.\nLaparoscopy was performed to exclude additional peritoneal disease and biopsies of the right liver tumour were taken. Biopsy specimens confirmed the diagnosis of a neuroendocrine tumour with a Ki-67 index of 4%.\nAt a multidisciplinary oncology team meeting, consensus of opinion was that the patient should be offered resection. Volumetric analysis demonstrated a 24% future remnant liver volume. A right portal vein embolisation was performed with a view to inducing left lobe hypertrophy. Four weeks later, reassessment of the liver volume confirmed that the future left lateral section remnant volume had increased to 32%.\nA midline laparotomy was performed. Exploration confirmed that the pancreatic mass had invaded into the greater curvature of the stomach and adjacent transverse colon. Initially, an extended right hepatectomy (segments 4a, 5, 6, 7 and 8) was performed including excision of the terminal part of the middle hepatic vein flush with the IVC. The left hepatic duct was divided, and the right hepatic artery was divided 1 cm distal to its confluence with the left hepatic artery. An extended Kocher's manoeuvre was performed and the posterior relations of the mass were assessed. It was evident that while the IVC and aorta were free from disease, the portal vein (PV) and coeliac axis were involved by tumour and would require resection, en-bloc with the mass. The superior mesenteric vein (SMV) and artery (SMA) were identified in the infra-colic compartment and the dissection plane was maintained along the SMA to its aortic origin. The right colon and small bowel were mobilised using the Cattell-Braasch manoeuvre [].\nThe involved PV and SMV were then transected above and below the mass, respectively. Continuity was restored by direct end to end anastomosis; facilitated by the extra mobility gained from the preceding hepatic resection and small bowel mesenteric mobilisation. Following this, an interposition saphenous vein graft was placed from the aorta to the junction of the right and left hepatic artery. The common hepatic artery was divided and the coeliac axis was divided and ligated flush with the aorta. The dissection plane was now continued to the left of the aorta along Gerota's fascia. The left adrenal gland was adherent to the tumour and was included in the en-bloc specimen. The terminal ileum, descending colon and gastro-oesophageal junction were all divided, thus completing the resection which consisted of the stomach, spleen, pancreas, duodenum, left adrenal, right colon and transverse colon (Figure ). Reconstruction consisted of a oesophago-jejunostomy and hepatico-jejunostomy (Figure ). Finally an end ileostomy and colonic mucous fistula were fashioned on the left abdominal wall. The total operative time was 16 hours and the intraoperative blood loss was 1850 mls.\nHistopathological examination revealed a well differentiated pancreatic neuroendocrine carcinoma 95 mm in diameter with a mitotic rate of one mitosis per 10 hpf and a Ki-67 proliferative index of 2% (Figure ). The tumour demonstrated local invasion into the retroperitoneum, colon, stomach and left adrenal gland, but all microscopic margins were clear. A completely excised single liver metastasis, 90 mm in diameter, was found in the hepatectomy specimen, and two out of 24 lymph nodes were involved by metastatic carcinoma.\nThe post-operative course was complicated by refractory chylous ascites, which was successfully managed with a peritoneo-venous shunt on the twenty fourth post-operative day. She was discharged from hospital without any further complications.\nFollow up showed a good functional recovery from surgery with independent resumption of activities of daily living by one month. CT at three and six months showed post-operative changes only. Nine months after surgery, the patient began to complain of left subscapular chest wall pain. A gallium 68 scan confirmed recurrence of tumour in the ribs bilaterally, mediastinum and in the remnant left liver. Slow release octreotide therapy was commenced and transarterial chemoembolisation (TACE) therapy was pursued for local control of hepatic disease. Bony disease was treated with radiotherapy.\nDisease appeared static until 12 months. Systemic chemotherapy was commenced upon medical oncology advice with everolimus. Unfortunately, she developed severe haematological and renal complications as a consequence and died 15 months after her initial operation. |
A 9-year-old boy was admitted to the Shenzhen People's hospital with kidney failure. The laboratory results are shown in Table . Samples and medical history were collected with the full informed consent of the patient and his parents in accordance with the declaration on the human genome prepared by the United Nations Educational Scientific and Cultural Organization. This study was approved by.\nUrine analysis revealed the sample was occult blood + and protein ++. Serological analysis revealed elevated urea nitrogen, creatinine, and cystatin C (26.55 mmol/L, 719.0 μmol/L, and 3.56 mg/L, respectively). In addition, the CT examination of the urinary system revealed that the patient's left and right kidneys were 53 × 22 mm and 61 × 27 mm, respectively, indicating bilateral renal atrophy (Figure ). In addition, except for continuous nocturia, there were no obvious abnormalities on physical examination. In terms of treatment, the patient began continuous peritoneal dialysis after admission to relieve renal deterioration.\nThe patient had occasional chest tightness, palpitations (heart rate 113 bpm), no dyspnea, chest pain or discomfort, and exercise was not restricted under normal conditions. Hypertension, hyperlipidemia, pulmonary hypertension, and no atrial fibrillation. The results of echocardiography indicated a slight increase in left ventricular diameter (left ventricular end-diastolic diameter 56 mm and end-systolic diameter 34 mm), with a left ventricular ejection fraction of 60%. Mitral thickening and prolapse with severe regurgitation and tricuspid thickening and prolapse with mild regurgitation were also observed. The shape of the aortic sinus was acceptable, with an internal diameter of 34 mm. The calculated Z-value of the internal diameter of the aortic sinus was 3.1. The echocardiographic manifestations of the patient are shown in Figure .\nThe parents of the patient were not consanguineous and were in good health. The parents had a negative family history of heart and kidney disease. The mother had no special pregnancy history, and the patient was delivered naturally at term, with a birth weight of 2.9 kg, and no history of neonatal asphyxia or infection. No obvious abnormalities were detected in the outer court genetic metabolic screening and microarray comparative genomic hybridization analysis (Affymetrix Cytogenetics whole genome––2.7 M array). |
A 19-year-old male patient was brought by his parents to our ocular emergency department with a history of sudden onset painful loss of vision along with conjunctival redness, bleeding, and proptosis in both the eyes for about 12 h in the absence of any preceding history of trauma []. Previous records revealed an isolated episode of bleeding over the left elbow joint at the age of 5 years, which subsided without any medical intervention. Following this, the patient remained symptom-free for about 14 years. However, the patient was not compliant with further investigations and follow-up.\nOn presentation to our ocular emergency, examination revealed bilateral proptosis with severe conjunctival hemorrhage, being more severe in the left eye. The extraocular motility was severely restricted in all gazes in both the eyes, but the pupils remained briskly reactive. Visual acuity was recorded as finger counting close to face with accurate projection of rays in both the eyes, and both the eyes had raised intraocular pressures (noted digitally). Bilateral inferior corneal exposure was noted; rest of the anterior segment details were within normal limits. Fundus examination using direct ophthalmoscopy was within normal limits in both eyes.\nThe patient was initially managed with immediate patching of both the eyes along with frequent lubrication, and subsequently, the investigations including a hematological workup and computed tomography (CT) scan were carried out. No immediate surgical intervention was performed due to the obvious risks involved with an invasive procedure in a patient with a suspected coagulation disorder. CT scan of head and orbit demonstrated a diffuse intraorbital hemorrhage with a dense collection noted around the ocular coats and the preseptal compartment but without any intracranial bleed [Fig. and ].\nMeanwhile, the blood investigations revealed the following findings: hemoglobin – 13.4%, total leukocyte count – 5700/mm3, platelets – 2 07,000/mm3, prothrombin time (PT) – 12.6 s, and activated partial thromboplastin time (APTT)- >1 min. Thus, based on the observations of prolonged APTT along with a previous history of bleeding, the diagnosis was pointing toward a possible bleeding disorder. The patient was further evaluated for factor VIII and factor IX levels. The results revealed normal factor VIII levels along with significantly decreased factor IX levels (9% of the normal). A diagnosis of hemophilia B was made and the patient was started on injection mannitol 5 ml/kg body weight once daily for 3 days, injection methylprednisolone 40 mg once daily for 3 days, injection Factor IX 3000 units IV for 7 days, oral amoxicillin and clavulanic acid 625 mg thrice a day for 7 days, oral acetazolamide 250 mg thrice a day for 3 days, topical brimonidine + timolol maleate eye drop twice a day for 5 days, and frequent lubricants both as drops and gel formulation for about 6 weeks. At the end of 6 weeks, patient's visual acuity improved to 20/20 in both the eyes [] and he remained asymptomatic for 24 months. |
A 2-year old girl with a past medical history of microcephalic osteodysplastic primordial dwarfism type 1 and an ill-defined immunodeficiency, manifested by selective hypogammaglobulinemia with inadequate response to the pneumococcal vaccine but no previous serious infections presented to the Emergency Department with a chief complaint of fever, dehydration, and malaise. Three weeks prior to presentation, the patient had completed a course of treatment with azithromycin for otitis media, with subsequent perforation of her tympanic membrane. The patient was prescribed a seven-day course of amoxicillin/clavulanate which was completed five days prior to presentation. Since completion, the patient had some improvement but continued to have daily fevers and ear pain. Three days prior to presentation, the patient began to have 3-5 episodes of non-bilious non-bloody emesis a day. On the day of her presentation the patient was unwilling to drink and had decreased urine output. In the emergency department the patient was initially diagnosed with severe dehydration and constipation but was subsequently admitted after interventions did not improve her condition. On admission the examination revealed a 13% loss of body weight in the previous three weeks. The exam was also significant for fever (39.1°C), tachycardia (170), severe dehydration, profound irritability and purulent drainage from the right ear. Initial laboratory evaluation was remarkable for leukocytosis with a WBC of 17.0 (93% PMNs, no bands) and a microcytic anemia with hemoglobin of 7.8 and an MCV of 71. CMP was unremarkable. Venous blood gas was notable for a respiratory alkalosis with a mildly elevated lactate (2.4).\nGiven the toxic appearance and a seemingly refractory otitis media with persistent fevers, a contrast computerized tomography (CT) scan of the brain was obtained prior to lumbar puncture and the patient was initiated on vancomycin and ceftriaxone. The CT scan revealed a coalescent right mastoiditis with a small underlying dural venous sinus thrombosis. Based on these findings the ceftriaxone was changed to piperacillin/tazobactam for Pseudomonas coverage and an emergent mastoidectomy was performed. Surgical specimen cultures were notable for Fusobacterium necrophorum but no additional organisms. Antimicrobial coverage was narrowed to clindamycin and ciprofloxacin. CSF gram stain was notable for PMNs but culture was negative. Blood cultures were negative for fusobacterium. Given the known association of this microorganism with Lemierre syndrome, a CT angiogram of the neck was obtained, which was notable for extensive right jugular veno-occlusive disease. There was no evidence of septic emboli in the brain or other end-organ septic embolic complications. The patient was initiated on enoxaparin for anticoagulation with significant symptomatic improvement and was discharged to complete a 6-week course of antimicrobials and anticoagulation. After completion of 6 weeks of anticoagulation therapy, she had improvement but not resolution of her dural venous sinus thrombus, and was continued on an additional 6-week course of anticoagulation, with repeat imaging pending. |
A 26-year-old male presented to Accident and Emergency following assault with a baseball bat. His past medical history consisted of longstanding Hepatitis C infection only, he was on no regular medications nor had any known allergies.\nOn admission, the patient had complained of bilateral pleuritic pain on inspiration with visible, tender bruising to the posterior thoracic region bilaterally. There were no urinary, bowel or neurological symptoms reported and no open wounds were visible on examination. The anterior abdomen was soft and non-tender to palpation.\nA CT scan of chest (performed at 30s post i.v. injection) showed acute fractures of left 10th/11thand right 8th/9th ribs, but no evidence of pneumothorax or lung contusion. A splenic laceration was not suspected clinically and the timing of the scan, optimized for the chest, only showed subtle splenic changes which were not identified by the original reporter ().\nLater that day, the patient complained that their pain had spread from the chest down to the right and left flanks. This prompted a CT scan of abdomen/pelvis (performed at 70 s), which demonstrated a contained splenic laceration with no evidence of capsular breach (). Both the rib fractures and splenic injury were treated conservatively with bed rest and analgesia.\nThe patient abruptly deteriorated becoming hypotensive and tachycardic in keeping with hypovolaemic shock. A further CT scan showed splenic capsular rupture, active bleeding and large-volume intraperitoneal haemorrhage ().\nThis was managed urgently with endovascular embolisation. While the patient gave informed consent for the procedure he indicated that he was unlikely to be compliant with some aspects of his aftercare and likely to self-discharge very soon after completion of the procedure.\nFollowing local anaesthesia an ultrasound guided puncture of the right common femoral artery was performed and the splenic artery selectively catheterized with a Sim 1 catheter (a reverse angled catheter). Angiography revealed three pseudoaneurysms, two arising from a second order upper pole branch and one from a third order equatorial branch (). These were then superselectively catheterized and embolized with a series of 3, 4 and 5 mm microcoils (Nester & Vortex, Nester = Cook Medical, Bloomington, IN, USA, Vortex = Boston Scientific, Watertown, MA, USA), delivered through a microcatheter. This abolished filling of the pseudoaneurysms but there was more devascularisation of the upper half of the spleen than envisaged when the decision to perform superselective embolisation was made. The right common femoral artery was then closed with a Starclose vascular closure device (Abbott Medical).\nThe decision to perform superselective embolisation was made with the intention of achieving definitive haemostasis in a patient who was likely to leave hospital at the earliest possible opportunity and not comply well with the procedural aftercare (). There was no further bleeding.\nDay 1 post-procedure, the patient developed a post-embolisation syndrome (PES) characterized by fever, nausea and left abdominal pain. A fourth CT scan was performed. This showed splenic necrosis, parenchymal and intravascular gas, which is an expected appearance post-embolisation. No fluid collection was demonstrated. Conservative resuscitation was initiated with i.v. fluid and antibiotic administration.\nThere was no indication for surgical drainage at this point as the CT scan showed only splenic necrosis, with no evidence of abscess formation. The patient’s temperature and clinical features were also settling, further reassuring stabilisation of his condition ().\nThe patient recovered from this episode a few days later, and deemed medically fit, was subsequently discharged home.\n3 weeks later, the patient presented to Accident & Emergency once again; this time with episodes of frank haemoptysis. He had an associated fever, and left upper quadrant (LUQ) pain.\nBlood tests showed elevated inflammatory markers, suggesting an infection, thought to be of likely respiratory origin at this time. |
A 31-year-old female visited the Department of Oral Medicine and Radiology with the chief complaint of pain and swelling over the left side of face since 13 months. History of presenting illness revealed that similar type of swelling was present on the right side of jaw since 6 months, which resolvoed spontaneously. There was difficulty in chewing food because of presence of multiple root stumps. There was no history of recent weight loss, facial paraesthesia and trauma in past. Past medical history revealed the presence of bilateral renal calculi since 1.5 years. The patient was known hypertensive for which she was on medication since 2 years. Past dental history revealed that patient underwent extraction of 36 and 37 four months back from a local dentist. The patient was married, with two children.\nOn general physical examination, patient was anemic with paleness of skin and palpebral conjunctiva. Except systemic temperature of 100°F, all vitals were within normal limits. Extraoral examination showed solitary diffuse swelling over left side of face causing gross facial asymmetry []. It extends from ala tragus line superiorly up to 4 cm below the inferior border of body of mandible inferiorly. It extends from midline anteriorly upto posterior border of ramus of mandible posteriorly. The overlying skin was intact and of normal color. On palpation, the swelling was tender; overlying skin was pinchable with no localized increase in temperature. The regional lymph nodes were not palpable. Intraoral examination showed large round, oval-shaped solitary diffuse swelling measuring 6.5 cm in maximum dimensions over the left mandibular alveolar ridge, extending anteriorly from canine region to retro molar regions posteriorly. The overlying mucosa was intact without any discharge. [] On palpation, it was hard in consistency, non-fluctuant, and tender presenting with egg shell crackling. Root stumps 13, 14, 15, 16, 26, 37, 38, 43, 44 and 45 were noted.\nBased upon history and clinical examination, aprovisional diagnosis of giant cell lesion of left body mandible was made and differential diagnosis of central giant cell granuloma, ameloblastoma, aneurysmal bone cyst, cherubism and brown tumor SHPT due to renal disease were considered.\nHematological investigation showed an RBC count of 3.02 × 106/μL, Hb of 6.6 g/dL, erythrocyte sedimentation rate (ESR) of 15 mm/1st hour and blood urea nitrogen of 29.9 mg/dl (N = 5-21 mg/dl).\nSerological investigations revealed raised serum calcium 14.3 mg%, alkaline phosphatase level of 1963 U/L (N = 108-306 U/L) and serum creatinine of 1.6 mg% (N = 0.5-1.2 mg %). Serum sodium and potassium were within normal limits.\nUrine examination showed clumped pus cells of about 80-90 cells/HPF.\nThyroid profile by fully automatic chemiluminescent immunoassay showed raised intact parathyroid hormone (PTH) level of 234.1 pg/mL (N = 15-68.30). Total T3 and total T4 were within normal limits.\nPanoramic radiograph showed multilocular, well-defined radiolucencies with corticated margins involving body of mandible crossing midline and ascending ramus of mandible of left side. There is generalized loss of lamina dura around all the teeth. The trabecular pattern showed ground glass appearance of numerous, small, randomly oriented trabeculae []. Mandibular cross-sectional occlusal radiograph showed expansion of both buccal and lingual cortical shapes.\nThe ultrasound of kidney showed 2.4 cm renal calculus in the right calyx and large staghorn calculus in the left sided kidney suggestive of SHPT []. The ultrasound of thyroid and parathyroid glands showed no abnormality except increased vascularity.\nCT scan showed 62 × 48 mm large expansile bony lesion with cortical expansion of bone and eccentric location at anterior and left lateral aspect of mandible. Bony lesion was causing displacement of teeth with loculated cystic lesion extending to left lateral side and approaching orbit [].\nComplete skeletal scan was performed showing no abnormality.\nIncisional biopsy was performed, which showed multiple spindle to oval shaped scattered multinucleated osteoclast-like giant cells along with areas of hemorrhage and scattered lymphocytes in the stroma and loose fibrillar matrix [Figures and ].\nBased upon the history, clinical, laboratorial, imaging and histopathology investigations the final diagnosis of craniofacial brown tumor because of SHPT due to chronic renal disease was made and the patient was referred for treatment in higher medical centre where a multidisciplinary approach was planned including nephrologist, endocrinologist and oral and maxillofacial surgeon. |
Medical history and demographics
A 76-year-old lady was admitted to hospital when she was discovered to have severe anemia (hemoglobin level of 61 g/L, packed cell volume of 0.191 L/L, mean cell volume of 94.6 fl) while on oral anticoagulant therapy (prothrombin time ratio of 3.7). She had a four-week history of non-specific left-sided abdominal pain, nausea, reduced appetite, passing dark stools, and breathlessness on exertion. The patient was given 10 mg of oral vitamin K and two units of packed-cell blood transfusion followed by an esophagogastroduodenoscopy, which did not reveal any source of upper gastrointestinal bleeding or abnormality. Four days later while an inpatient, she became acutely confused and unwell with symptoms and signs of a chest infection including fever and severe hypotension, which did not respond to intravenous antibiotics and intravenous fluids. Further investigations revealed results, which prompted endocrinology assessment. The patient’s past medical history consisted of hypothyroidism for which she took levothyroxine 125 µg daily and systemic lupus erythematosus (SLE) for which she took warfarin tablets 3 mg daily for previous cranial vasculitis and thromboembolic episodes. She had been tried on hydroxychloroquine in the past but this was stopped because of visual side effects, and her SLE was under control. Apart from that, the patient was usually fit and well. There was no past history of prolonged high-dose corticosteroid usage, but she had received a short course (prednisolone 10 mg daily for one week) two years prior to this presentation.
On the day of admission, she was pale and had mild tenderness in the left iliac fossa on abdominal examination. The rectal examination did not reveal any source of bleeding. Repeat examination demonstrated a heart rate of 98/min and hypotension (blood pressure 75/50 mmHg). She had a temperature of 38°C, respiratory rate of 18 breaths per minute with an oxygen saturation of 95% on room air |
The patient is a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility to be evaluated for CNS vasculitis. She was admitted to this outside facility for a four-week period prior to being transferred to the primary facility for further evaluation over a subsequent 23-day period. Total duration of hospitalization at both the facilities was close to 7.5 weeks. Approximately one week into the initial four-week admission, her family found that she was very lethargic with diminished responsiveness and pronounced difficulty speaking. In the emergency room (ER), her blood pressure was measured at 243/129 mmHg with a blood glucose value greater than 400 mg/dL. She was started on aggressive antihypertensive therapy and underwent a series of diagnostic tests. Dual antiplatelet therapy (DAPT) consisting of aspirin and clopidogrel was initiated in combination with high-dose atorvastatin. With respect to her lethargy and fluctuating cognition, there was concern that she may be experiencing complex partial seizures, so lacosamide was also started.\nA baseline computed tomography (CT) scan of the head without contrast showed multiple indeterminate lacunar infarcts involving the head of the right caudate nucleus and left corona radiata. The same day, a magnetic resonance imaging (MRI) was performed and elicited similar findings with the addition of bilateral punctate infarcts of the left thalamus, right periventricular white matter, and right centrum semiovale. Magnetic resonance angiography (MRA) done on the following day showed high-grade stenosis of the left middle cerebral artery (MCA), in addition to markedly diminished caliber of the right MCA and high-grade stenosis involving the left posterior inferior cerebellar artery (PICA). Bilateral carotid ultrasounds showed very mild plaques. An angiogram exhibited an occluded left posterior cerebral artery (PCA) distally and was also suggestive of advanced intracranial atherosclerosis (more so than would be expected in CNS vasculitis). There was no evident change from day two to day six of this hospital course. A spinal tap performed at the end of the first week demonstrated elevated protein and IgG synthesis rate (16.4), which was concerning for CNS vasculitis. Appreciating the contrast between the imaging and spinal tap findings, CNS vasculitis could not be ruled out. The patient was started on intravenous (IV) corticosteroids briefly, however, the medication was discontinued due to worsening hyperglycemia that was progressively difficult to control. Near the end of the third week of hospitalization, a repeat MRI showed a new small stroke in the left subcortical parietal white matter.\nThe patient was transferred to the primary facility after this initial month of hospitalization, at which time the patient had a National Institutes of Health Stroke Score (NIHSS) of seven. She was alert and oriented to person only and able to follow simple commands. Significant findings on subsequent blood testing revealed leukocytosis (12.2), elevated absolute neutrophil count (ANC) at 11.3, hyperglycemia (314 mg/dL), HbA1c of 9.6%, mildly elevated erythrocyte sedimentation rate (ESR) at 36, positive herpes simplex virus type one (HSV1), and the presence of IgG and hepatitis B core antibody (HBcAb). Workup for hypercoagulable state was negative for Factor V Leiden and antithrombin deficiencies, though notably protein C was elevated. A repeat spinal tap on hospital day one showed elevated levels of protein (122), but also demonstrated an elevation in myelin basic protein (6.09). Otherwise, the patient was afebrile and hemodynamically stable on admission. On hospital day two, rheumatology was consulted. In order to confirm the suspected diagnosis of CNS vasculitis, the specialist recommended a leptomeningeal biopsy and IV corticosteroids in the interim. Although angiography is very sensitive, it is nonspecific as it cannot distinguish between vasculitis and reversible cerebral vasoconstriction syndrome (RCVS). Consequently, the angiography that the patient had undergone earlier in her hospital course could not provide us with a definitive diagnosis, thus warranting the biopsy of the brain.\nA baseline transthoracic echocardiogram (TTE) obtained on hospital day three revealed a left ventricular ejection fraction (LVEF) of 57 +/-5 percent with mild dilation of the left atrial cavity. Repeat imaging showed much of the same findings, however, radiology recommended further workup for underlying CNS vasculitis. Over hospital day four to six, the working diagnosis was “multifocal bihemispheric strokes with no clear etiology with an encephalopathic process.” The patient’s cardiovascular risk factors continued to be treated and monitored (lipids, blood pressure, and sugars), and a more extensive rheumatological workup was ordered. Continuous electroencephalogram (EEG) monitoring on hospital day eight also showed evidence of diffuse encephalopathy although there were no epileptiform changes or seizures recorded. Over the second week of hospitalization, a new left cerebellar infarct was detected on MRI, at which time steroids were tapered down and a transesophageal echocardiogram (TEE) was ordered. The results of this echocardiogram were unchanged in comparison to the baseline TTE. There was no thrombus detected in the left atrium, ruling out cardioembolic etiology of the new stroke. The CT studies of the chest and abdomen were negative for any findings pertinent to the patient’s chief complaint.\nDuring the third week of hospitalization, a repeat head CT without contrast revealed additional recent infarcts. A four-vessel angiogram showed 50% stenosis in the petrous and cavernous segments of the left internal carotid artery (ICA), a completely occluded M1 segment of the left MCA, and multiple alternating foci of narrowing within the M2 and M3 branches of the right MCA as well as the P2 and P3 branches of the PCA. In consideration of these findings and given the fact that there is a considerable overlap between the imaging appearance of vasculitis and atherosclerotic disease, neither diagnosis could be excluded. A second rheumatology consult recommended that a leptomeningeal biopsy be considered prior to starting cyclophosphamide, effectively ruling in CNS vasculitis versus ischemic stroke. The neurosurgery team agreed to conduct the biopsy of the meninges and brain. However, after discussing the details of the procedure with the patient's family, her family decided against her having the procedure due to the risks associated with brain surgery and the debilitating neurologic deficits already suffered by the patient. |
A 40-year-old female patient underwent liver transplantation in February 2016 for cirrhosis secondary to nonalcoholic steatohepatitis. The patient's additional comorbidities included hypertrophic cardiomyopathy managed by myomectomy in 2013, hypothyroidism on replacement, Type II diabetes mellitus (controlled, HbA1c 5.4 g/dl), and chronic kidney disease (baseline creatinine 1.2 mg/dl). Her immunosuppressive regimen in the immediate posttransplant period included tacrolimus, mycophenolic acid, and prednisone. In July 2016, a diagnosis of possible tacrolimus-related posterior reversible encephalopathy syndrome was made on the basis of clinical and radiologic findings, and her immunosuppression was changed to everolimus-based therapy. In October 2016, the patient underwent liver biopsy, which showed severe acute cellular rejection and was managed with high-dose steroids and thymoglobulin. The patient was transitioned to cyclosporine for persistent clinical evidence of rejection.\nIn November 2016, the patient was admitted to Intensive Care Unit (ICU) for the management of adult respiratory distress syndrome and septic shock related to Legionella pneumophila pneumonia. Her ICU course was complicated by respiratory failure, including failed extubation, requiring more than 10 days of ventilatory support, acute on chronic renal failure requiring CRRT, and worsening graft dysfunction requiring up-titration of immunosuppression. Propofol was used as the primary sedating agent along with opioids. She was continued on cyclosporine, which required aggressive up-titration due to subtherapeutic levels.\nOn day 13 of her ICU stay, the patient had issues with her CRRT filter clotting multiple times during the day shift, despite heparin infusion at set rate of 500 units/h with an activated partial thromboplastin time (aPTT) goal of 50–70 s. The anticoagulation was initially ordered for right radial artery thrombus but had helped prevent the patient's CRRT circuit from clotting until day 13. The patient's aPTT was stable between 48 s and 64 s and was therapeutic at the time of clotting on day 13. Due to the change in patient's response to propofol, in addition to the continuous 5 days of propofol infusion, a TG level was obtained and was found to be 3745 mg/dl. Six months prior, the patient's TG level was 156 mg/dl. Her filter and circuits were changed and propofol was discontinued. That evening, the patient had further clotting, and the bedside nurse noticed a change in color of the line and filter []. Her repeat TG level was found to be 3865 mg/dl with normal lipase level. She was also found to have rising serum potassium of 5.8 mmol/L, serum bicarbonate of 20 mmol/L, and pH of 7.2. Adjunctive therapy with intravenous insulin at the rate of 0.1 units/kg/h and heparin at the rate of 500 units/h was initiated, and the patient was planned for emergent therapeutic plasma exchange (TPE). TPE was initiated within 6 h of nonfunctioning CRRT circuits through the patient's hemodialysis vascular access [] using 5% albumin as replacement fluid and a citrate anticoagulant to whole blood ratio of 10. CRRT was able to be resumed with a new filter following TPE and her acid–base balance and electrolytes normalized. She remained on heparin infusion at 500 units/h.\nDespite being off of propofol in the days after, the patient continued to have elevated TG levels up to 708 mg/dl, with increasing serum lipase levels (peaking at 240 mg/dl) and complaints of abdominal pain concerning for pancreatitis. She subsequently required two additional TPE sessions, on day 16 (TG decreased from 708 to 186 mg/dl) and day 21 (TG decreased from 499 to 212 mg/dl) to maintain goal TGs <500 mg/dl. The patient was continued on cyclosporine with escalating doses due to persistent subtherapeutic serum levels and liver graft dysfunction. Both cyclosporine and underlying liver dysfunction were suspected as the culprits for her recurrent and persistent hypertriglyceridemia. She was also started on oral fibrate and fish oil as preventive measures to avoid pancreatitis. Approximately 8 weeks after the patient first required TPE for hypertriglyceridemia, she underwent combined liver–kidney transplant. She received propofol for sedation intraoperatively and remained on cyclosporine postoperatively. Immediately following the second transplant, her TGs level was 154 mg/dl, the lowest it had been since her baseline level months prior. Her TGs level has remained well-controlled despite continued use of cyclosporine. |
In late 2016, a 61 year old non-smoking female presented with exertional shortness of breath, mild pedal edema, distended neck veins, and a recent weight gain of 15 lbs over the prior 2 weeks. It was initially suspected that the patient was experiencing pulmonary embolism based on her clinical presentation and elevated D-dimer, but further testing revealed that she was experiencing cardiac tamponade with mild pulmonary hypertension and heart failure. Pericardiocentesis was performed without definitive diagnosis. In February 2017, the patient exhibited worsening respiratory symptoms without fever, hemoptysis, sputum production, B symptoms, or extremity edema. Baseline chest PET-CT images were reviewed by radiologists at both UCLA Oncology and Texas Tech University Health Sciences Center, demonstrating a large middle mediastinal mass encasing the main pulmonary artery, with pericardial and left pleural effusion (Figure & Figure ). Scattered pulmonary nodules and hypodense lesions were identified in the right lobe of the liver, consistent with a diagnosis of metastatic disease (Figure ). Biopsy of the mediastinal mass revealed a high grade undifferentiated malignant neoplasm composed of highly proliferative (ki67 staining ~50%) pleomorphic anaplastic epithelioid malignant cells with large areas of necrosis and fibrosis. Immunohistochemistry revealed strong antigenicity for CD31 and CD34, and weak antigenicity for D2-40 and Factor VIII indicating a diagnosis of angiosarcoma that was corroborated at both UC San Diego Health and MD Anderson.\nConventional treatment options were recommended, however the patient declined these based on low reported survival rates, and instead, requested the non-selective beta blocker propranolol as a single agent therapy. In May 2017, 40 mg/kg propranolol was administered daily and PET-CT scans were performed at regular intervals to assess the response of the tumor to propranolol. Assessment of tumor response was based on 18F-fluorodeoxyglucose (FDG) tracer uptake and measurements/assessments of the primary tumor and distant metastases. After 12 months of propranolol as a single agent therapy, significant debulking and decreased size of the residual mediastinal mass was observed on PET-CT scans, with resolution of pericardial effusion (Figure & Figure ). Pulmonary nodules were stable to regressed, and the nodules in the right lobe of the liver had completely resolved (Figure ). There was no evidence of residual hyper-metabolic activity based on FDG measurements in the primary lesion or in metastatic sites in the chest, abdomen, or pelvis on PET-CT. |
An ill-appearing 19-year-old male with the one-year history of asthma presented to the emergency room with non-specific symptoms including fatigue, dyspnea, numbness in the right leg, nausea, vomiting, and dizziness. Two months prior to presentation, he had a sinus surgery and within few days after this surgery, he developed cough and dyspnea, so he was admitted to outside hospital for possible pneumonia. He was treated with cefuroxime, Tamiflu, and oral prednisone. He improved momentarily with steroids. Two weeks later, he returned to the outside hospital complaining of right foot plantar numbness and dyspnea, he was discharged home on Levaquin as they thought he may have some residual sinus disease left. One week later he was seen by a pulmonologist at outside hospital and they noticed that one of the cultures grew staph, hence started on Bactrim. He took Bactrim for three days and his mother noticed that he developed some mental status changes, hence Bactrim was stopped. After this, no more symptom of mental status change was noticed. Over the next few weeks, the patient noticed tachypalpitations, continued to have fatigue, shortness of air, and fatigue so the family decided to come to our hospital's emergency department for further workup. While in the emergency room, he was found to be in atrial fibrillation with the rapid ventricular response and elevated troponins. The patient spontaneously converted into sinus rhythm within 10 minutes. His vital signs were stable except for tachycardia with a heart rate of around 100 beats per minute. Physical examination was unremarkable with a normal sensation on right leg and foot. He was admitted to cardiac intensive care unit for further workup due to elevated troponin.\nSalient laboratory values and electrocardiogram\nThe patient’s initial complete blood count was remarkable for white blood cell of 28,800/ul with eosinophil count of 12,960/ul (45%) in spite of the use of low-dose oral corticosteroids for a few days prior to admission. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were both elevated at 35 and 4.14, respectively. His admission troponin was 16.28. His initial electrocardiogram (ECG) showed atrial fibrillation with a heart rate of 161 beats per minute, non-diagnostic Q waves in the inferior leads, T-wave inversions in the inferior leads and no significant ST segment changes noted (Figure ). His repeat ECG 10 minutes later when he converted to sinus rhythm showed sinus tachycardia with a heart rate of 100 beats per minute, Q and T changes as noted earlier, as well and no significant ST segment changes noted (Figure ). Other labs, including TSH, UDS, BNP, lactate, and renal function, were unremarkable. Rheumatological workup including anti-nuclear antibody (ANA), perinuclear antineutrophil cytoplasmic antibody (p-ANCA), cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA), rheumatoid factor, myeloperoxidase (MPO) antibody, serine protease antibody 3, and anti-cyclic citrullinated peptide (anti-CCP) IgG was inconclusive. However, the patient’s IgE and IgG were both markedly elevated. Several infectious causes, such as histoplasma, coccidioides, strongyloides, cytomegalovirus (CMV), human immunodeficiency virus (HIV), tuberculosis (TB), Epstein-Barr virus (EBV), hepatitis B, and hepatitis C, were explored and all were negative.\nImaging\nTransthoracic echocardiogram revealed an ejection of 55% with some apical hypokinesis. The transesophageal echocardiogram showed no evidence of endocarditis, thrombus, shunt, or atherosclerosis. Computed tomography angiography (CTA) of the chest with and without contrast showed moderate mediastinal and bilateral hilar adenopathy in addition to bilateral axillary lymphadenopathy, bilateral peribronchial thickening, and patchy ground-glass opacities most predominantly in the posterior lower lobes. There was no evidence of pulmonary embolism (Figure ). Cardiac magnetic resonance (CMR) showed several areas of delayed enhancement within the left ventricular myocardium and decreased perfusion in the mid to apical septal and inferior segments throughout the apex. It also revealed a small pericardial effusion and minimal hypokinesis of the lateral apical wall (Figures -). Due to the abnormal myocardial enhancement, a CT of the heart with coronary CTA was ordered which showed normal coronary artery anatomy with no evidence of stenosis, calcified plaque, or soft plaque (Videos -). Due to his reported neurologic symptoms, CT of the head without contrast was ordered and showed two areas of low-attenuation within right frontal white matter. MRI of the head was subsequently performed which showed many small bilateral punctate infarcts throughout cerebrum and a few additional ones in the cerebellum.\nBiopsies\nA bone marrow biopsy showed normocellular bone marrow for age and no concern for dysplasia; however, both the bone marrow biopsy and peripheral blood smear showed marked eosinophilia with leukocytosis. Several transbronchial cryobiopsies were taken from the left lower, upper lobes and lingula of the lung which showed patchy areas of eosinophilic venulitis with dense eosinophilic infiltrates involving many of the small venules. This process was happening in the background of chronic bronchiolitis with abundant eosinophils within small airways, smooth muscle hypertrophy, and goblet cell metaplasia (which suggests asthma). All these findings taken into consideration together suggested EGPA (Figure ).\nThe patient was initially started on 1000 mg of intravenous methylprednisolone for three days and then 1 mg/kg/day of oral prednisone for several months with a gradual taper. He was also started on cyclophosphamide for three to six months. Additionally, due to the patient’s young age, arrangements for sperm preservation were made prior to starting cyclophosphamide. The patient responded well to the treatment and at his one-month rheumatology follow-up, the patient continued to improve. His troponin-I reduced to 0.08 at one month visit. |
A 23-year-old Caucasian woman who suffers from WS was evaluated in the emergency room after being injured in a fall. She had left her home during the night without being noticed by her family. She fell next to a highway near her house and fractured her leg; a driver found her, took her to the nearest hospital and contacted her relatives. She was evaluated by orthopedics and then sent home with her family with orders to be medicated with acetaminophen 1000mg three times a day (t.i.d.) alternated with ibuprofen 600mg t.i.d. At home, she presented with psychomotor agitation; her family decided to take her back to the hospital only a few hours after she was discharged. She was presenting with persecutory delusions, auditory and verbal hallucinations, soliloquies, and psychic and motor agitation, and was hospitalized.\nApart from the WS, she had no known medical illness and was not taking any medication before being admitted to the hospital. Nevertheless, she was submitted to extensive medical investigation in order to exclude possible organic causes for the symptoms. A cerebral computed tomography scan did not reveal any lesions. Abdominal ultrasonography showed a slight increase of diffuse liver echogenicity, with no other changes. Her total and direct bilirubin levels were higher than normal (1.8mg/dL and 0.6mg/dL, respectively). No abnormalities were found in her renal function (creatinine, urea and glomerular filtration rate), electrolytes, liver function tests and C-reactive protein. A full blood count showed normal values, as did thyroid-stimulating hormone, free thyroxine and haptoglobin. Viral markers for human immunodeficiency virus 1 and 2, hepatitis C virus and hepatitis C virus were negative. Her toxicology screening was negative as well. Meanwhile, haloperidol 6mg daily was introduced. Liaison psychiatry later confirmed the presence of these symptoms and haloperidol was therefore maintained. As no acute organic cause for her symptoms was found, she was transferred to our psychiatric unit 2 days after being hospitalized. A mental examination was difficult to execute due to the patient’s intellectual disability, but family members provided considerable valuable information. She was still presenting with persecutory delusions and auditory-verbal hallucinations at this time.\nOn the third day of receiving haloperidol 6mg daily, she developed medication-specific side effects: sialorrhea, excessive somnolence and cogwheel rigidity, primarily on her upper left limb. The haloperidol was replaced with risperidone 2mg daily. The side effects improved, but as she was still presenting with excessive somnolence, the risperidone dosage was decreased to 1.5mg daily.\nApproximately 1 year before this episode, she had been expelled from a geriatric care unit after she began to exhibit inappropriate behavior. Her family also reported that she quite frequently had tried to leave home until 2 months before being hospitalized, when her behavior changed. She began to be more suspicious and afraid of being alone. She would look behind doors and to the ceiling as if she expected to find something there. Her mother said in an interview, “She spoke very often to herself, but in a manner that looked like there was someone else there with her.” Her mother associated these changes in her behavior with specific events. Namely, she said this behavior began when the patient’s younger brother (who had looked after her during the past year) resumed school and when another brother and her stepfather fought, resulting in the latter being hospitalized with injuries. The patient had presented with learning difficulties since early age and was referred to a school for children with intellectual disabilities. The diagnosis of WS was made when she was 15-years old. She demonstrates an “elf-like” face, supravalvar aortic stenosis (evaluated in the past by cardiology) and growth retardation, which are present in up to 80% of individuals with WS []. She has also mental retardation, with learning deficits, social disinhibition and an unusual aptitude for music.\nThere is no evidence of prior psychiatric illness or symptoms. There is also no case of mental or neurologic disease in her family.\nThe psychotic symptoms improved gradually, and on day 7 at our unit they were no longer present. She was discharged 21 days after her admission to our unit. |
A 62-year-old female with no significant past medical history presented to the emergency department in November of 2017 with complaints of arthralgias, most notably in her right knee, left shoulder, and bilateral thighs that made it difficult for her to ambulate. She was also admitted due to a headache that was triggered primarily by coughing. Vital signs on admission were as follows: a blood pressure of 202/90 mmHg, a heart rate of 137 bpm, a respiratory rate of 20, and a temperature of 36.6 Celsius. Physical exam revealed Janeway lesions. She was found to have a neutrophilic leukocytosis, with white blood cell count at 20.4 cells/mm3 and neutrophils at 17.4 bil/L. Troponin was elevated at 1.85; this was deemed to be noncardiac in nature as the patient's pain was relieved with ibuprofen and her EKG showed no acute findings. ESR and CRP were elevated at 95 mm/hr and 24.8 mg/dL, respectively. A computed tomography of the brain showed a high-density mass in the right occipital lobe, with surrounding vasogenic edema. The patient continued to deny any visual changes or symptoms other than what was discussed above. An ophthalmologist was consulted to perform a dilated fundus exam, which was positive for small intraretinal hemorrhages that were deemed to be secondary to the patient's hypertension and less likely positive for Roth's spots. There was no evidence of disc edema. A brain MRI with and without gadolinium showed multiple small punctate bilateral areas of acute or subacute infarctions indicative of embolic phenomenon. The hemorrhagic area in the right occipital lobe was again identified, with subtle surrounding enhancement; the differential diagnosis consisted of neoplasm, vascular malformation, or embolic infarction with hemorrhagic conversion. A transthoracic 2D echo was without vegetation, so a transesophageal echo was ordered, and vegetation was shown on the posterior leaflet of the mitral valve. Two blood cultures from admission then came back positive for Rothia dentocariosa. Infectious disease was confirmed, and the patient's current antibiotics, which consisted of vancomycin and ceftriaxone, were switched to penicillin G on a continuous pump. The patient remained largely asymptomatic during her admission and was deemed to be stable for discharge from the hospital after a nine-day stay with penicillin G via a continuous pump for a total of six weeks and was planned for a follow-up MRI in three weeks. The repeat MRI came back showing new subacute strokes. The patient was reported, again, to be asymptomatic but was directed to come straight to the emergency department. A repeat transesophageal echo was done and showed the known vegetation on the mitral valve with new vegetation seen on the PICC line and an abscess between the mitral and aortic valves extending into the ascending aorta. The patient then requested transfer to another institution for further evaluation. A repeat transesophageal echo was completed at this outside institution which showed small anterior and posterior mitral leaflet vegetation with no significant destruction and no abscess. A cardiac MRI was then performed which showed a focal delayed enhancement in the apical inferior and lateral wall, likely secondary to coronary arterial embolization. The patient went on to complete the full six weeks of penicillin therapy, remained asymptomatic, and refused a mitral valve replacement. Her follow-up was continued in the cardiology clinic. |
A 31-year-old primigravida presented to a maternity unit with shoulder tip pain two days after an uncomplicated spontaneous vaginal delivery. She was born in Nigeria and moved to the United Kingdom when she was seven years old. She had a normal gestation and childhood. Her family’s medical history was unremarkable. She had a past medical history of gastro-oesophageal reflux disease and uterine fibroids. In the month prior to her presentation she had sustained a traumatic comminuted fracture of her right third metacarpophalangeal joint which was managed conservatively with splinting. Her first pregnancy was complicated by worsening of her fibroid-related pain in the first trimester and she was commenced on regular analgesia, antiemetics and aspirin (due to concerns of predicted low birth weight). Following delivery she was not on any regular medications. There was no history of drug allergies. Following assessment at the maternity day unit it was concluded that her shoulder tip pain was related to her fibroids and she was discharged with analgesia and safety-netting.\nTwo days later, she was admitted as a priority call to our emergency department with a working diagnosis of possible massive pulmonary embolism from the ambulance service. On arrival she was in extremis. Her airway was patent. She was unable to speak with a respiratory rate of 56 and oxygen saturations of 56% on non-rebreather mask (15L of oxygen). Her trachea was central and there was poor air entry bilaterally. She was in shock with a heart rate of 170 beats per minute and unrecordable blood pressure. Point-of-care echocardiography carried out by the emergency department team demonstrated good biventricular systolic function without right ventricular dilatation or pericardial effusion. There was absent pleural sliding bilaterally on thoracic ultrasound. Chest X-ray demonstrated large bilateral pneumothoraces (see Figure ). Fifty milligrams of intravenous ketamine was given to provide analgosedation for surgical thoracostomies and chest drain insertion. Both pneumothoraces were under tension with audible hiss and rapid physiologic improvement. Subsequent radiograph demonstrated substantial resolution with ex-vacuo pulmonary oedema and new subcutaneous emphysema.\nFollowing stabilisation she underwent computed tomography which additionally demonstrated signs of interstitial lung disease (see Figure ). There was an 8.3 by 6 cm pedunculated uterine leiomyoma.\nShe was transferred to a tertiary cardiothoracic centre for further assessment and care. Due to persistent left-sided air leak she underwent unilateral video-assisted thoracoscopic surgery. Apical bullous disease was noted with normal appearances of the horizontal fissure and diaphragm and she underwent left apicectomy and parietal pleurectomy. Histopathological assessment found areas of subpleural fibrosis, patchy interstitial fibrosis and emphysematous changes with very occasional collections of bland spindle-shaped cells at the margins of dilated airspaces. These findings in conjunction with immunohistochemistry (Smooth Muscle Actin and Progesterone Receptor positivity with equivocal to negative Human Melanoma Black 45) suggested an underlying diagnosis of pulmonary lymphangioleiomyomatosis.\nSerial imaging demonstrated persistent basal left-sided hydropneumothorax and on the sixth post-operative day she had a 12-French Seldinger chest drain inserted by interventional radiology to good effect. She was discharged with outpatient cardiothoracic surgical and respiratory medicine follow-up to evaluate the need for follow-on right-sided video-assisted thoracic surgery and further assessment of her interstitial lung disease. |
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