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A 23-year-old man (weight 65 kg, height 175 cm, and BSA 1.8 m2) with a diagnosis of primitive right atrial enlargement from foetal age was referred to our Centre for cardiological evaluation. Cardiac examination showed increased heart size on percussion and a grade II/VI Levine systolic murmur. No significant pathological findings were found on pulmonary examination. Electrocardiography showed a regular sinus rhythm with a rate of approximately 60 beats/min associated with an abnormal morphology and duration of P wave (enlargement of P wave with duration of 130 msec), together with a low amplitude of QRS complexes in the limb leads. All routine laboratory studies were within normal limits. Chest radiography showed an abnormal cardiac silhouette with increased convexity in the lower half of the right cardiac border and cardiomegaly ().\nTransthoracic two-dimensional echocardiography demonstrated a huge right atrium of about 6.2 cm and a volume of 230 ml/m2, with a thick smoke pattern and mild tricuspid regurgitation. The pulmonary arterial pressure was normal (). The tricuspid valve was normal without significant annular dilation. No stenosis or abnormal displacement of the tricuspid valve leaflets was detected. No significant regurgitation of the tricuspid valve was found despite a partial distortion of the anterior leaflet and compression of the right ventricle inflow. The right ventricle appeared small and compressed anteriorly by the right atrium (area of RV: 11 cm2).\nCardiac magnetic resonance imaging showed a marked right atriomegaly (right atrium area: 66.50 cm2, volume: 220 ml/m2) and normal size of the left atrium (left atrium area: 7.02 cm2). The right ventricle was regular in size and global contractility but was partially compressed and dislocated posteriorly, due to the massive enlargement of the right atrium. The left ventricle was regular in dimension, thickness of the wall, and global/segmental contractility (FE VS = 61%). No evident transvalvular jets or areas of late gadolinium enhancement were found. The pericardium was visualized without focal abnormalities or pericardial effusion ().\nDue to the high risk of arrhythmias and thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, the patient underwent cardiac surgery. Through a median sternotomy, cardiopulmonary bypass was established with standard aorta and bicaval cannulation. After the pericardium was opened, the entire anterior surface of the heart was found to be covered with a thin wall in continuity with the right atrium. No atrial appendage as such was apparent. The right atrium was fully opened. The inferior border of the atriotomy was sewn around the anterior part of the tricuspid annulus, and the superior border was brought over the lateral wall of the right atrium as a flap and sewn near the interatrial groove. This provided adequate reduction of the atrial size and reinforcement of the atrial wall ().\nThe histology of the resected atrial wall showed focal hyperplasic areas of smooth muscle cells with polymorphic nuclei surrounded by a few scattered areas of hypertrophic fibrous tissue.\nPostoperative transesophageal echocardiogram showed a significant reduction of the right atrium area (23 cm2, volume: 93 ml).\nThe patient was extubated 11 hours after surgery. Complications arose postoperatively with the early appearance of pericardial effusion with leukocytosis and elevated inflammatory markers. This was resistant to conventional medical therapy, which in the end required surgical drainage. Medical therapy of the postpericardiotomy syndrome (ibuprofen 600 mg/TID and colchicine 1 mg/OD) was continued over the subsequent 6 follow-up months without further recurrence of pericardial effusion. |
A 63-year-old Chinese gentleman was admitted with a 4-day history of progressive left lower limb swelling and pain. There was no history of trauma to the leg. He did not have any fever, chest pain, breathlessness, nor constitutional symptoms.\nThis was his first episode presented as such. He had a past medical history of hypertension and hyperlipidaemia, when he was on diet control with no chronic medications. There was no family history of DVT. He had no recent prolonged flights or immobilization, although he was working as a private car hire driver with ten-hour shifts. He was a never smoker.\nHe was comfortable and afebrile with normal vitals and oxygen saturation. The cardiovascular and respiratory examinations were unremarkable. His left lower limb was warm and mildly tender, associated with swelling up to the midthigh measuring 4 cm more than the right (). There was no evidence of stasis dermatitis, varicosities, or phlegmasia cerulean or alba dolens. His peripheral distal pulses were well felt.\nInitial blood investigations including full blood count, renal panel, and coagulation profile were normal; fasting glucose for diabetes screening was negative. Electrocardiogram showed sinus rhythm with no heart strain. Doppler compression ultrasonography of the left lower limb showed partial DVT extending from the external iliac (EIV), common femoral (CFV), to the popliteal veins (Figures –).\nIn view of the extensive nature of the thrombosis, a contrasted CT of the thorax abdomen and pelvis was performed both to rule out an intrabdominal pathology as well as look for pulmonary complications in the same sitting. His CT eventually showed compression of the left common iliac vein (CIV) against the lumbar vertebrae by the overlying right common iliac artery (CIA), just caudal to its confluence with the right CIV (), with no definite filling defect identified in the IVC. In addition, filling defects in the right upper lobe and lower lobe pulmonary arterial lobar/segmental branches were noted, consistent with pulmonary embolism without evidence of right heart strain. In the absence of any suspicious pelvic mass, these features were suggestive of MTS. He was started on LMWH enoxaparin.\nDuring the operation, a temporary IVC filter was first inserted via a retrograde right femoral common femoral vein approach (Bard Denali) to mitigate the risk of massive pulmonary embolism and cardiovascular collapse during wire and device manipulation through the iliac thrombus. The left posterior tibial vein at the ankle was the initial vascular access site to allow full evaluation and thrombus removal of the entire femoropopliteal segment in addition to the iliac veins. Intraoperative venogram demonstrated extensive intravenous thrombus from the left CIV to proximal superficial femoral vein (SFV) (). This was treated with AngioJet pharmacomechanical thrombolysis/thrombectomy (Boston Scientific, Massachusetts, United States of America) using the ZelanteDVT thrombectomy catheter and a recombinant tissue plasminogen activator (alteplase 20 mg in 100 ml normal saline). Vascular access was then obtained via the left mid-distal femoral vein for diagnostic intravascular ultrasound (IVUS) which confirmed compression at the level of the CIV (cross-sectional area∼126 mm2) followed by iliac vein stenting using a 16 × 60 mm ABRE (Medtronic, Dublin, Ireland) bare metal stent. There was uninterrupted contrast flow through the left CIV upon completion of the procedure (Figures –) with resolution of the compression and increased CIV cross-sectional area (∼198 mm2) on IVUS.\nPostoperatively, he was placed on thromboembolic deterrent stockings and reviewed regularly by physiotherapists to prevent deconditioning. He was initially observed in the high dependency ward, with improvements of his lower limb swelling, and was subsequently transferred to the general ward where he remained well. His enoxaparin was switched to a Factor Xa inhibitor Apixaban (10 mg BD loading dose for 7 days followed by 5 mg BD maintenance dose), which he was discharged with to complete a total of 1 year duration. On his 1-month follow-up, the patient remained well with resolution of his lower limb swelling and pain. An ultrasound duplex iliac scan showed a patent left CIV stent with subacute to chronic venous thrombosis partially occluding the left CFV. He subsequently underwent an uneventful elective IVC filter retrieval, with plans for a 1-year repeat ultrasound and follow-up. |
A 35 year-old female, gravida 3 para 2, presented to the emergency department with her first ever episode of angina pain 9 weeks and 3 days following an uneventful caesarian section. The patient noted constant chest tightness with radiation to both arms while getting ready for work. The pain was associated with diaphoresis but she denied dyspnea or nausea. Six years prior she underwent catheter pulmonary embolectomy for a thromboembolism thought to be related to oral contraceptive use. Since then she had been taking warfarin and warfarin was restarted postpartum. Otherwise the past medical history was only significant for hypertension. The family history was notable for a younger sister who was diagnosed with cardiomyopathy six weeks postpartum and a grandmother who died of unknown causes suddenly at age 42 without a relationship to pregnancy.\nUpon physical examination the pulse rate was 83, respiratory rate 16 and blood pressure 130/67. The EKG demonstrated evidence of anterior ischemia. Serial troponin-t peaked at 1.17 ng/mL. The INR measured 1.9 IU. Initial treatment consisted of loading with 300mg clopidogrel. Emergent cardiac catheterization showed left anterior descending (LAD) coronary artery dissection complicated by extensive thrombus (Figure , Additional file ). Otherwise the coronary anatomy was without lesions in the left main coronary artery, the circumflex coronary artery or the right coronary artery (RCA) (Figure Panel A, Additional file ). The dissection affected the mid LAD to the distal LAD with irregular severity. Intravascular ultrasound demonstrated subintimal thrombosis but there was no evidence of a free dissection plane. Three everolimus eluting stents (Xience, Abbott Laboratories, USA) measuring 2.5 × 18 mm, 2.5 × 23 mm and 2.5 × 18 mm were deployed and dilated to 3.5 mm proximally. However, an edge dissection of the most distal stent became apparent after treatment of the target lesion. This was covered with an additional 2.5 × 18 mm stent. Notably, the post-intervention EKG demonstrated no evidence of ischemia in the RCA territory (Figure ). The post-intervention course was unremarkable until a witnessed episode of ventricular fibrillation arrest occurred the following day. Cardiopulmonary resuscitation was undertaken for 7 minutes and unsynchronized cardioversion with 200 joules restored a normal sinus rhythm. The EKG showed new T wave inversion in the lateral leads (Figure ). Upon repeat catheterization it was discovered that her non-dominant RCA had newly dissected and was occluded with thrombus (Additional file ). A wire was passed distally, but it was unclear whether this was through the true or false lumen and no stents were placed. However, improvement of distal perfusion was noted on angiogram (Figure Panel B, Additional file ). In view of this, as well as the recent myocardial infarction, the patient was treated conservatively. Transthoracic echocardiography the following day demonstrated a low normal left ventricular ejection fraction of 50-55% as well as apical hypokinesis. This was confirmed by cardiac magnetic resonance imaging. She was discharged five days later on aspirin, warfarin, prasugrel, metoprolol, atorvastatin, and magnesium oxide. The patient made an un-eventful further recovery. Follow-up transthoracic echocardiography after four months demonstrated an improved left ventricular ejection fraction of 55-60% without definite regional wall motion abnormalities. The patient remains asymptomatic. |
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 72-year-old man was referred to our hospital for suspected right pulmonary effusion on chest X-ray. For approximately three years around the age of 30, he dealt with serpentine asbestos in research. Seven years before he was referred to our hospital, he visited another hospital for suspected left pleural effusion on chest X-ray, and his chest computed tomography (CT) showed left pleural effusion and ipsilateral pleural thickening; after two years of follow-up, the pleural effusion had resolved without treatment (Fig. ). Two years later, he had similar abnormalities pointed out on chest X-ray, and his chest CT revealed unchanged left pleural thickening; with no apparent change, his follow-up was finished after one year (Fig. ).\nAt the first visit to our hospital, there were no specific findings on physical examination as well as laboratory tests, except for slightly low serum haemoglobin level. Chest CT showed bilateral pleural thickening accompanied with calcification on the left pleura (Fig. ). Over the next four years, thickening and calcification of the pleura showed gradual progression, and accordingly his lung volumes declined (Fig. ).\nDuring this progression, he was admitted to our hospital for acute respiratory failure caused by bacterial pneumonia. Despite resolution of pneumonia, he developed hypercapnic chronic respiratory failure, and therefore home oxygen therapy and non-invasive positive pressure ventilation (NPPV) were initiated. Subsequently, he experienced recurrent bacterial pneumonia, and 53 months since the first visit he was again hospitalized for acute-on-chronic respiratory failure. Thickening and calcification of the pleura progressed furthermore (Fig. ). Despite treatment, he died on the 94th day of admission; autopsy was performed with the consent of his family. On gross finding, the bilateral lungs were entirely covered with white, hard, and fibrous membrane and adhered to the chest wall. Microscopically, the membrane consisted of hyalinized fibrous tissue with patchy infiltration of lymphocytes. No obvious asbestos body was detected. (Fig. ) Asbestos body concentration was measured at the Kobe Rosai Hospital. Tissue samples were derived from the right lower lobe and processed using standard methods. Asbestos body concentration was 1086/g dry lung tissue, indicating moderate asbestos exposure. |
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. |
Case 1: Mrs. A was a 27 year old patient in her first pregnancy who was referred at 15 weeks gestation from a physician with a diagnosis of RHD. She had just been diagnosed with severe MS 3 weeks prior to presentation although she had been symptomatic since childhood. An echocardiogram showed mitral valve area (MVA) of 1cm2, Ejection Fraction (EF) of 59.3% and a moderately dilated left atrium of 55-60mm diameter with no pulmonary hypertension. A multidisciplinary team (MDT) was set up comprising a physician, cardiothoracic surgeon, obstetricians and an anaesthetist. The cardiothoracic surgeon offered open commissurotomy as percutaneous balloon valvuloplasty (PBV) was not available. She declined the in view of the procedure related risks. At 20 weeks gestation she was commenced on propranolol to treat a persistent tachycardia and prophylactic warfarin. She was switched from warfarin to low molecular weight heparin (LMWH) at 36 weeks gestation. At 38 weeks, an echocardiogram showed an MVA of 0.9cm2 and EF of 58%. Her functional status was New York Heart Association (NYHA) class II. The estimated foetal weight (EFW) was 1950g, with normal middle cerebral artery (MCA) and umbilical artery (UA) flow. In order to time the required lapse period between last dose of LMWH and the epidural procedure, she had elective induction of labour. She developed foetal bradycardia 6 hours after initiating induction. An emergency caesarean section was done under general anaesthesia as the epidural catheter which had been timed for established labour had not yet been inserted. She was given frusemide and antibiotics peri-partum. A 2020g live baby was delivered. She was discharged from hospital on day 7 on warfarin and was stable when reviewed by both physician and obstetrician 14 days postpartum. She was admitted to the Coronary Care Unit 4 weeks postpartum with shortness of breath, severe supraventricular tachycardia and hypotension and died 3 days later. |
The child was a boy aged 6 years and 6 months. He was admitted to hospital due to snoring at night with buccal respiration for 15 days. A physical examination revealed that there was a 1.0 × 1.0 cm mass, protruding from the midline to the left in the right posterior palatopharyngeal arch. The surface of the mass was smooth and soft, and there was no tenderness; no fistula or mass was found in the neck. Electronic nasopharyngoscopy showed that there was a cystic mass of ~2 × 2.5 × 1.0 cm with a smooth surface in the left eustachian cushion, which passed though the posterior margin of the soft palate to protrude into the oropharynx and midline (). Pharynx CT revealed mass-like soft tissue density shadow from the right nasopharyngeal wall to the oropharyngeal wall, and the adjacent airway was compressed and narrowed (). An electro-otoscope was used to show that the bilateral external auditory canals were clean, the tympanic membranes were intact, and the marker was clear. Acoustic immittance exhibited a type A curve, and pure tone audiometry indicated that the hearing in both ears was normal. After a satisfactory level of general anesthesia was achieved, an opener was used, and a catheter was inserted through the double inferior meatus of the nose and through the nasopharynx. It was then taken out through the oral cavity, and the soft palate was pulled up to enlarge the cavity of the pharynx. Endoscopic examination showed that the base of the mass was located on the right eustachian cushion, the size of the mass was ~2 × 2.5 × 1.0 cm, and its distance from the pharyngeal opening of the eustachian tube was ~0.3 cm. The mass was well-exposed, and then punctured, and ~3 ml of light-yellow viscous liquid was extracted. The mass was pulled to the left and down with a clamp through the mouth, and the part of the capsule wall protruding into the cavity of the pharynx was resected with laryngeal scissors and a plasma knife. It could then be seen that the lateral lacuna was depressed outwards, and the surface of the deep capsule wall was smooth, which was not treated. The pathological diagnosis was a bronchogenic cyst (). The child was followed-up for 1 year after the operation, and there was no recurrence. |
A 60-year-old man with a past surgical history of C5–C7 anterior arthrodesis for cervical fracture 17 years ago was found on the floor after an unwitnessed ground-level fall, resulting in a head strike. He was taken to the local hospital in an Aspen collar with a GCS of 15 and no neurologic deficits. Routine ECG and laboratory evaluation was unremarkable except for elevated serum alcohol level. Full cervical spine computed tomography (CT) scan was performed which showed an Anderson and Montesano classification (20) type III left occipital condyle fracture (), a Levine and Edwards classification (21) type III (Jefferson) fracture with mild lateral subluxation of bilateral C1 masses (), and an asymmetric widening and slight anterior subluxation of the right AOJ (). A head CT was performed which showed no acute intracranial abnormalities.\nThe patient was immediately transferred to a level I trauma center for further management. In the emergency department, he reported severe midline neck pain, worse with movement, and unchanged paresthesia to bilateral upper extremities which he states is his baseline. Physical exam was unremarkable except for a right forehead hematoma with overlying abrasions and tenderness to palpation over the midline posterior neck. He exhibited full strength and unchanged baseline sensation to all extremities. He denied bowel or bladder incontinence and had strong rectal tone and intact perianal sensation. Through his clinical course, he became increasingly altered and agitated, refractory to medication. He was unable to maintain spinal precautions due to his agitation, and in an attempt to protect his cervical spine, he underwent endotracheal intubation.\nMRI of the cervical spine was performed and showed ligamentous injury at the craniocervical junction (CCJ) (Figures and ), asymmetric left odontoid-lateral mass widening, widening and subluxation of the right AOJ (), and edema of the paravertebral soft tissues around the fracture. CT and MRI established the diagnosis of OCD in a neurologically intact patient. Due to the unstable nature of the fracture, the patient was taken to the operating room for emergent occiput-C2 posterior instrumentation and fusion ().\nAn Aspen collar was in place at all times until surgical stabilization. Immediately, after removing the collar, Mayfield tongs were applied. Prior to patient positioning, baseline somatosensory evoked potentials (SSEPs) were obtained. Using a Jackson table turning frame, the patient was rotated into prone position. Fluoroscopic imaging was used during manual manipulation of the Mayfield apparatus to ensure no further displacement of the fracture.\nOcciput to C2 posterior instrumented arthrodesis was performed. Neuromonitoring remained stable during the procedure. The patient was discharged to home five days after presentation. |
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. |
A 68-year-old man, known for HCM and nonsevere functional MR, suffered 2 incidents of acute pulmonary edema, following his atrial fibrillation ablation, in 2018 and 2019, the last of which was complicated by cardiogenic shock requiring transient mechanical support with an IMPELLA device (Abiomed, Danvers, MA). Transthoracic echocardiography (TTE) at that time showed a sudden deterioration of the MR, quantified as severe, with a centrally directed jet, possibly caused by procedural volume overload and the appearance of a SAM, causing the hemodynamic instability. The patient was stabilized and discharged with mild MR on the TTE. In January of 2020, the patient was hospitalized for another cardiac decompensation and transferred to our centre for evaluation. After diuretic treatment, transesophageal echocardiography (TEE) showed a mild-to-moderate central MR on an annular dilation (41 mm) caused by LA enlargement and no SAM. Despite optimized medical therapy, the patient had another fulminant cardiac decompensation in our centre that required respiratory support. A combination of a severe functional MR with a centrally directed jet and a subvalvular apparatus SAM causing LVOT obstruction was confirmed by TTE. The visualization of a central MR jet on the TTE is in favour of a severe functional MR caused by an annular dilation and not completely explained by the subvalvular apparatus SAM ().\nFacing this “eclipsed” MR and the fragility of the patient, the heart team estimated the risk of mortality or morbidity to be more than 15% and proposed the least invasive intervention. The unstable MR being explained by a mixed mechanism (annular dilation and a SAM), the team opted for a MitraClip procedure rather than an advanced surface ablation to treat both mechanisms at the same time. We proceeded with the installation of 2 NTR clips under general anaesthesia and 3-dimensional TEE guidance. The first clip was installed in zone 2 (A2-P2), leaving a moderate residual, posteriorly directed MR jet on an incomplete subvalvular apparatus SAM, where a second clip was installed (). The procedure was successful, with a postprocedural TTE showing mild residual MR. The patient's symptoms were significantly improved, and he was discharged to a rehabilitation centre. At 6-month follow-up, the patient has not been readmitted, and the TTE showed stable MitraClips with residual mild MR. |
The patient was an 82-year-old female who is a nursing home resident presented to an outside hospital with a two-week history of jaundice that had progressively worsened. She was brought to the emergency department and found to have hyperbilirubinemia with a total bilirubin of 10.7 and a direct bilirubin of 8.7. She was afebrile, and white blood cell count was 8.9. A CT scan of her abdomen and a MRCP were diagnostic for Mirizzi syndrome, revealing large stones compressing the common bile duct (CBD) with proximal dilation. She was transferred to our hospital for a higher level of care with hepato-pancreatico-biliary surgeons. Of note, her past medical history was significant for morbid obesity with a BMI of 42, dementia, diabetes mellitus, hypothyroidism, deep vein thrombosis (DVT), depression, anemia, tobacco use, and congestive heart failure (CHF). Her surgical history consisted of an appendectomy.\nUpon arrival at our hospital, the patient underwent an endoscopic retrograde cholangiopancreatography (ERCP) with the removal of small stones in the common bile duct, sphincterotomy, and two stent placements with ends terminating in the distal right and left hepatic ducts. However, despite this, there was still tapering in the common hepatic and common bile ducts noted from extrinsic compression from the large stones in the gallbladder and a significant filling defect through the cystic duct (). Additionally, the patient developed a fever and leukocytosis and persistently elevated bilirubin, with the most likely cause being an obstruction of the biliary stents causing persistent cholangitis and cholecystitis. The ERCP was repeated, and a third stent was placed. The patient's fever was controlled, and the bilirubin began to trend down.\nOnce the patient was stabilized, she was taken to the operating room for a cholecystectomy. The procedure began laparoscopically, but dense adhesions in the right upper quadrant involving the liver, gall bladder, omentum, and transverse colon prevented the development of adequate and safe dissection planes, and thus, the procedure was converted to open. The lysis of adhesions continued to be difficult, but once the gallbladder was dissected out, two large, firm masses were palpated within the fundus and the neck of the gallbladder. Given the difficulty with the anatomy to safely do a cholecystectomy with the gallstones en bloc, the decision was made at this time to do a partial cholecystectomy with the removal of the stones. The anterior gallbladder wall was first opened near the fundus, and the gallbladder wall was first dissected off the stone and then the stone from the posterior wall. When removed, the stone measured approximately 3.5 centimeters (), and pus and necrotic tissue were found within the gallbladder.\nAttention was then turned to the neck of the gallbladder where the anterior wall of the gallbladder was again incised, revealing another a large 4-centimeter stone () that was dissected off the posterior wall and removed. It was noted at this time that there was significant arterial bleeding with a loss of approximately 300 cc of blood and the development of mild hypotension and tachycardia. Given the new symptoms and the patient's baseline hemoglobin of 8.4, the patient received two units of red blood cells intraoperatively. The arterial bleeding was controlled with sutures, and attention was turned to the biliary tree upon which a defect in the common hepatic duct and the common bile duct was noted, presumably where the cystic duct previously entered and from the cholecystocholedochal fistula formation. The defect measured approximately three centimeters in length and eight millimeters in width after debridement of necrotic tissue.\nGiven the fragility of the tissue and the size of CBD defect, it was not closed primarily. However, the patient's hemodynamic instability and overall health status made a biliary-enteric anastomosis a very high-risk operation with significant morbidity and mortality for the patient. Thus, the decision was made to use a dissected, viable gallbladder wall piece to form a free flap to repair the defect in the biliary tree (). The mucosal surface of the gall bladder wall was juxtaposed to the lumen of the common ducts and secured in place with a running 5-0 PDS suture. The remnant gallbladder stump was closed and oversewn in a continuous fashion. The area was extensively irrigated given the pus and necrosis that was discovered during the cholecystectomy. One drain was placed within the closed gallbladder remnant and the second one outside the closed gallbladder remnant. The abdomen was then closed in the usual manner.\nImmediately, postoperatively, the patient was managed in the intensive care unit given her instability during the operation but was transferred to a step down unit shortly after on postoperative day two. She was observed off of antibiotics with normalization of leukocytosis, remained afebrile and hemodynamically stable, and had continuously down trending bilirubin levels throughout the remainder of her hospital stay. Clinically, she recovered well and was stable for discharge on postoperative day seven with drains remaining in place and to be removed on a follow-up as an outpatient. On a subsequent follow-up, the patient was noted to remain clinically stable and follow-up CT scans revealed no intrahepatic biliary duct dilation, a normal caliber common bile duct, no intraluminal filling defect, a patent stent, and no biliary leak (). Laboratory evaluation revealed no leukocytosis and normalization of liver enzymes and total bilirubin levels. Given these results, her surgical drains were removed, but her biliary stents remained in place, and despite multiple contact points and counseling to have a follow-up with the gastroenterologists for ERCP and removal, she was noncompliant and the stents remain in place to date. She however remains stable and healthy during her one follow-up appointment as of this writing and continues to be monitored closely with phone check-ins with no evidence of further hepatobiliary symptoms or complaints of incisional hernias. |
Our patient was a 70-year-old Caucasian male with an established diagnosis of DD since his childhood years. He had had innumerable exacerbations in the past and multiple admissions for secondary bacterial infections. He presented to the hospital with worsening of coalescing, firm, greasy, and bright red to skin-colored macules and papules located around his gluteal clefts, back, scalp, and forehead causing severe debilitating pain. The gluteal lesions were the most clinically relevant symptom and they were causing profound distress. He had severe physical deconditioning and had fallen several times prior to his arrival. A few weeks before his hospitalization, he had been diagnosed with intraductal papillary mucinous neoplasm (IPMN) of the pancreas and had undergone a total pancreatectomy and splenectomy with hepaticojejunostomy and Billroth II gastrojejunostomy, which had contributed to his fatigue and weakness. He had no abdominal symptoms and his abdominal exam was initially benign. His vitals were stable, and there was no systemic or laboratory evidence of an infectious process at the time of admission. As the clinical course progressed, his skin lesions and clinical status continued to worsen. He developed bacteremia secondary to Streptococcus dysgalactiae and then Pseudomonas a few days later. A week later, he developed bacterial peritonitis with Enterococcus faecalis, Klebsiella pneumoniae, Candida glabrata, and E. coli. He received multiple courses of broad-spectrum antibiotics and antifungals. The dermatology service was closely following the patient and had started him on acitretin with a dose of up to 50 mg daily. He was also started on systemic steroids. Although he had intermittent days of temporary improvement and relief, his overall progress was mired with worsening skin lesions, sepsis causing hypotension, and functional decline.\nPain management, palliative care, infectious diseases, burn surgery, surgical ICU, social work, cardiology, and endocrinology were involved in his care and followed him closely. The patient had had recurrent exacerbations of his skin disease in the past, especially following a stressful event like surgery. Acitretin controlled it for a limited time, only to be followed by another exacerbation. He had suffered multiple exacerbations with superimposed Streptococcal and Pseudomonas cellulitis over the years. The patient felt physically and emotionally exhausted, and his quality of life was significantly impaired. He was also emotionally drained. He decided to adopt comfort measures alone and ultimately passed away within a few months. |
A 31-year-old man experienced sudden onset of abdominal pain and tenderness in the right hypochondrium. There was no history of trauma, and no history of significant disease in the past. He underwent laparotomy at a primary health care centre, owing to a misdiagnosis of ruptured liver abscess with peritonitis. On exploration, hemoperitoneum with active bleeding from the liver surface was found. Due to lack of diagnosis of the possible cause of bleeding, perihepatic packing was done with sponges, and the patient was referred to our tertiary care institute. The patient received three units of packed cells prior to shifting to our center. On arrival, patient was pale and in hemorrhagic shock. The blood pressure was 76/50 mmHg, pulse was 124 beats per min and feeble. Abdomen was distended and diffusely tender. Laboratory investigation revealed hemoglobin of 6.8 g/dl, hematocrit 26%, and high transaminase levels. After initial resuscitation with intravenous fluids and packed red blood cells (RBC), the patient underwent a contrast enhanced computerized tomography (CECT) scan on a spiral computerized tomography (CT) scanner. CT scan demonstrated a peripherally enhancing lesion in right lobe of liver, located in segments VI and VII []. The largest dimension of the lesion was 11 cm in the craniocaudal direction, and transverse and antero-posterior measurements were 9.7 and 7.3 cm respectively. The site of rupture was clearly seen as a rent in the enhancing margin of the lesion at the postero-superior aspect [ – arrows]. On delayed scans, the lesion showed characteristic centripetal filling-in of contrast, typical of a hepatic hemangioma, with a small central non-enhancing fibrous scar []. Delayed images also revealed hyperdensity tracking along the liver surface and settling in the dependent part [], indicating the presence of active contrast extravasation. There were associated hemoperitoneum and mild bilateral pleural effusions. Remnant liver parenchyma was normal with no other focal lesion. The sponges from previous laparotomy were also identified. Based on CT scan, a diagnosis of giant hepatic hemangioma with rupture and hemoperitoneum was made and patient was shifted to angiography suite within 12 hours of arrival at our center after adequate resuscitation. The patient was normotensive but tachycardic at the time of shifting to the angiography suite.\nDigital Subtraction Angiography (DSA) revealed typical angiographic findings of a liver hemangioma with pooling and puddling of contrast in the right lobe lesion. Arterial contrast extravasation was noted on the celiac angiogram, after which a selective right hepatic arteriogram was done that confirmed active contrast extravasation from the hemangioma [].\nThe hemangioma was embolized in a single session, first using PVA particles for embolisation of tumor interstices, and then the principal arteries were occluded using two steel coils []. PVA particles 500–750 microns (Cook Bloomington Inc U.S.A) were used for embolization and were delivered using a Cobra catheter. Deep catheterization obviated the need for using a microcatheter. The vitals of patient immediately stabilized, and a check angiogram showed successful embolisation with no contrast leak.\nThe patient received a total of six transfusions perioperatively. When the general condition of the patient stabilized, he underwent laparotomy for removal of surgical packs. After pack removal, surgeons did not note any active bleed, and a large hemangioma involving segments VI and VII, which was ruptured on the posterior surface was noted []. The hemangioma could be completely enucleated without significant blood loss. Histological examination revealed a cavernous hemangioma of 11.5×10 cm diameter. The postoperative course was largely uneventful except for mild fever and right pleural effusion which necessitated tube thoracostomy. The patient fully recovered and discharged three weeks following surgery. Patient was asymptomatic on follow-up at two months. |
A 58-year-old man was admitted to our hospital because of acute cardiac decompensation. A transthoracic echocardiogram showed increased left ventricular end-diastolic volume and diameter (160 mL and 55 mm, resp.), severely compromised left ventricular ejection fraction (LVEF 35%) with diffuse hypokinesia, and diastolic grade I dysfunction. A mild mitral regurgitation (MR) was noticed, while aortic valve leaflets and function were normal. Left cardiac catheterization showed 50% stenosis of left main and chronic total occlusion of the right coronary artery; therefore surgical myocardial revascularization was scheduled. The medical history of this patient was significant for impaired glucose tolerance, previous smoking, and Hodgkin's disease (HD) when he was 33 years old. Because of this, he was submitted to mediastinal radiation therapy (RT), with negative follow-up thereafter. In consideration of history and clinical presentation, we decided to perform OPCAB, harvesting left internal thoracic artery (LITA) and a segment of saphenous vein (SV). Despite the presence of firm adhesions at harvesting, LITA flow and caliber were proper, so that this conduit was successfully grafted to left anterior descending artery (LAD). Ascending aorta (AA) and pulmonary artery were partially covered by fibrotic tissue, and dissection to allow side clamping of the aorta was demanding. Finally, we did not succeed in punching the aortic wall, because it was abnormally hyperplastic; we decided to change strategy, preparing a composite Y-graft (SV to LITA) for obtuse marginal branch. During the operation it was not possible to achieve our standard target activated clotting time (ACT) for OPCAB (>250 sec), despite high doses of heparin and antithrombin III. The immediate postoperative course was characterized by an intense inflammatory response. On first postoperative day dual antiplatelet therapy with acetylsalicylic acid and clopidogrel (100 mg and 75 mg daily, resp.) was started. The patient moved to the Cardiologic Rehabilitation Ward on the third postoperative day, and postoperative course was uneventful. He was discharged 15 days after surgery. Two months later, the patient underwent thoracic and coronary computed tomography (CT) scan that confirmed severe thickness of the ascending aortic wall (5.6 mm versus 2 mm normal value) and a localized wall disruption at the site of aortic punching (). All grafts were patent (). |
A 50-year-old man presented with progressive hearing loss in the right ear and vertigo for the past 6 months and facial numbness and unsteady gait for the past 15 days. Upon physical examination, he was found to have right ear sensory hearing loss, ataxia, diminished sensation in the right face (supplied by the third branch of the trigeminal nerve), and high frequency hearing loss in the right ear, as revealed by brain stem auditory evoked potential examination. MRI examination revealed a lesion in the CPA with solid and cystic components, which compressed the brain stem and the cerebellum. The anterior portion of the lesion was solid and showed signs of cystic changes, whereas the posterior portion of the lesion was cystic. The solid component of the lesion showed hyper- and isointensity on T1WI images and mixed hyper- and hypointensity on T2WI images, and it was significantly enhanced after contrast administration. The size of the solid component of the lesion was about 2.2 cm × 2.2 cm × 2.3 cm (Figure ). Surgery was performed via a right suboccipital retrosigmoid approach, and intraoperative monitoring of cranial nerves was conducted. The lesion was revealed to be red, well margined, firm, vascular, anteriorly solid with cystic changes, and adherent to the brain stem and the cerebellar hemisphere, the trigeminal nerve, and facial and acoustic nerves. Following separation of the lesion from adjacent nerves and tissues along the border of the lesion under microscopy, the lesion was totally resected in a partitioning manner. The xanthochromic fluid in the back of the lesion was drained during surgery. The patient recovered well after surgery and presenting symptoms were significantly relieved. Postoperative CT scans demonstrated that the lesion was completely resected (Figure ). The histopathological features of the lesion were consistent with a CM (Figure ). At 1-year follow-up, this patient's symptoms at presentation had resolved. |
A 23 year old male was referred to our intensive care unit from an outside hospital with hepatic and renal failure (serum bilirubin 41 mg/dl, International Normalized Ratio (INR) 1.7, Blood Urea Nitrogen (BUN) 108 mg/dl, serum creatinin 5.3 mg/dl).\nThree weeks earlier the patient had presented to an outside hospital with increasing fatigue and cervical swelling over several weeks. Biopsy of the cervical lymph nodes had established the diagnosis of nodular sclerosing classical HL. Staging was done by whole body MRI scan (Figure ) and revealed a large cervical lymph node bulk (8 × 3.3 cm) on the left side and slightly enlarged cervical, paratracheal and paraaortal lymph nodes on the right side. Moreover, there were two small masses in the left lung (13 mm and 7 mm in diameter, respectively) as well as multiple hepatic nodules. Further laboratoy work-up was notable for increased Erythrocyte Sedimentation Rate (ESR) of 96 mm/h, and immunodeficiency (lymphocytopenia, T helper cell and B cell count significantly decreased, IgG4 deficiency). Histological examination of the bone marrow showed no infiltration by HL.\nThe patient was also known to suffer from NBS. He was diagnosed at birth and registered in the NBS Registry as patient No. 45. In accordance with this diagnosis he displayed mild mental retardation (he had been working as an auxiliary cook up to this point), microcephaly, characteristic facial features as well as an immune defect. He was known to suffer from selective IgG4 deficiency and his fibroblasts and lymphocytes had been found to be hypersensitive to induction of DNA breaks (The International Nijmegen Breakage Study Group []). Up until this young adult age he had no history of severe infections.\nDuring the initial evaluation the patient was withdrawn from further medical care by his parents. When he developed B symptoms and his condition rapidly deteriorated he was readmitted and transferred to our hospital. The patient measured 1,52 m with a weight of around 75 kg; he presented awake and oriented, but severely icteric, anuric and edematous. On admission, a little over two weeks after the diagnosis had been established, he was in acute renal and hepatic failure. Treatment with Prednisone 100 mg i.v. daily had been started.\nUltrasound-guided biopsy of liver nodules was performed immediately and showed multiple small nodular infiltrates of classical Hodgkins lymphoma (Figure ). Therefore, diagnostic stage had to be corrected to IVB and systemic chemotherapy similar to CHOP schedule was started, however adapted to poor renal and hepatic function. He received reduced CHOP-like chemotherapy including cyclophosphamide 375 mg/qm (50%), doxorubicin 12.5 mg/qm (25%) and vincristin 2 mg abs. on day 3 of the hospital stay.\nCitrate hemodialysis was initiated immediately and continued for the following four weeks. During this time renal function started to improve. Additionally, after application of the first cycle of chemotherapy, albumin dialysis was applied several times until hepatic function started to recover.\nWhile organ functions were improving following application of chemotherapy, the patient developed aspergillus fumigatus and candida albicans pneumonia, confirmed by culture of bronchioalveolar fluid, and had to be ventilated for ten days. White blood counts were stimulated using granulocyte colony stimulating factor (G-CSF) starting on day 4 after chemotherapy application. Twenty-one days after the first chemotherapy, the patient received the second cycle, this time an attenuated ABVD schedule (adriamycin d1 75% and d15 100%, bleomycin d1 25% and d15 50%, vinblastin d1 75% and d15 100%, dacarbazin d1 50% and d15 75%). During the second cycle he again became septic from Candida urinary tract infection but recovered in due time with caspofungin treatment and G-CSF support to receive the next dose of chemotherapy on day 15. Overall, we applied four cycles of chemotherapy, three of them ABVD d1 and d15, and were able to escalate the doses of adriamycin, bleomycin and vinblastin to 100%, and of dacarbazin to 75% of the regular adult schedule. In detail, the following doses (given as total or per m2 body surface area) were applied. Cycle 1: cyclophosphamide 375 mg/m2, doxorubicin 12,5 mg/m2, vincristin 2 mg total dose; cyclophosphamide was reduced to limit hematotoxicity considering renal failure; doxorubicin dose was adjusted to poor liver function. Cycle 2 (3 weeks later), d1: doxorubicin 19 mg, bleomycin 2,5 mg, vinblastin 4,4 mg, dacarbazin 194 mg, all per m2. doxorubicin was still applied in a lower dose to account for hepatic dysfunction, bleomycin was thought to be particularly toxic since it induces DNA breaks, especially in kidney failure. Since the patient was still recovering from severe sepsis also vinblastin and dacarbazin were reduced to limit the risk of neutropenic infection. Pegylated G-CSF was given d4 and d19. Cycle 2, d15: doxorubicin 25 mg, bleomycin 5 mg, vinblastin 6 mg, dacarbazin 281 mg, all per m2. Cycle 3 and cycle 4, d1 and d15: doxorubicin 25 mg, bleomycin 10 mg, vinblastin 6 mg, dacarbazin 281 mg, all per m2.\nHowever, the patient was affected with severe intensive care polyneuropathy leading to lower extremities paresthesias and paralysis. The situation was further complicated by grade IV anal ulcer and eventually a depressive episode. The resulting refusal to co-operate prevented mobilisation as well as implementation of supportive measures and staging procedures. Therefore, and in consideration of the dramatic clinical response already obtained it was decided together with his family to terminate chemotherapy. After fourteen days on an inpatient psychiatric ward undergoing intensive psychotherapy the patient was referred to a rehabilitation facility for the following eight weeks and then discharged home.\nFollow-up MRI was performed at 3, 6, 12 and 18 months after initial diagnosis and continued to show complete remission. At 20 months after the initial diagnosis, the patient has now regained full mobility, does not exhibit any clinical sequelae of the disease and has started to work again as an auxiliary cook. His mental condition has returned to baseline without pharmacologic intervention. |
A 9-year-old boy accompanied by his parents reported to the Department of Pediatric Dentistry with the chief complaint of forwardly placed upper front teeth. Parents reported history of active thumb sucking by the child since childhood, sucking his left thumb during sleep only. Child's mother revealed that he was unable to refrain from the habit even after repeated motivation from them. Clinical examination revealed the following features: Early mixed dentition stage, narrow and V shaped maxilla, proclined maxillary central incisors, mesio labial rotation of 11, 21. Median diastema of about 2 mm was also present [Figure and ]. Patient exhibited an overjet of 9 mm, a negative open bite of 0.5 mm and bilateral posterior crossbite extending up to primary canines.\nCephalometric evaluation revealed a skeletal Class II tendency with normodivergent facial pattern [Figure and ]. The anterior dentition presented with mild dentoalveolar proclination []. Analysis of the cast revealed adequate arch length in both maxilla and mandible with arch dimensions depicted in . Based on the investigations, the case was diagnosed as skeletal Class II (border line) and dental Class I with bilateral posterior crossbite and anterior open bite.\nThe child was counseled in the same visit regarding the deleterious effect of digit-sucking habit on dental occlusion, facial esthetics and he was self-motivated to stop the habit by himself. However, he expressed inability to refrain from the habit. Then, we planned intercepting the habit with a modified design of quad helix appliance.\nMolar separation was achieved using orthodontic separators. After banding the maxillary molar, an alginate impression was made with the bands in position and the cast was prepared. A modified quad helix crib appliance was fabricated with 0.036 inch stainless steel wire. Anterior component of the quad helix was modified to form 3 cribs, which are continuous with the anterior helices and the posterior component retained the conventional design. The expansion arms extended up to the primary canine region. The wire component was soldered to the molar bands in situ [].\nThe appliance was tried intra orally before cementation to ensure optimal fit and extension of the crib. It was cemented in the passive form and was not activated until 2 weeks, which allowed the child to acclimatize. The presence of crib in the appliance made it extremely difficult for the child to place the thumb in the mouth. Thus it acted as a deterrent to eliminate the habit. After 2 weeks, the appliance was activated for transverse expansion of maxillary arch using 3-prong plier at the inner leg []. The activation expanded the appliance close to 2 mm generating 100-150 g force. The correction of posterior crossbite was monitored every 3 weeks and the appliance was activated until overcorrection was achieved. Crossbite correction was achieved in 6 months along with successful interception of habit [Figure and ]. The magnitude of expansion achieved in inter-canine and inter-molar width is demonstrated. Post-treatment cephalometric evaluation revealed marked improvement in the upper incisor inclination, interincisal angle, palatal plane and increased vertical dimension. No change was noted in the sagittal relation of the jaws [ and ]. A simple Hawley's retainer was prescribed as retention appliance. |
A previously healthy 34-year-old Korean female was admitted to a regional hospital for fever, myalgia and severe headache that had started 2 weeks ago. Brain MRI showed a high T2 signal intensity change and diffuse swelling of the right temporal lobe, insula and hippocampus (Fig. ). Lumbar puncture showed lympho-dominant pleocytosis. The patient was started on intravenous (iv) dexamethasone and acyclovir under a high suspicion of viral encephalitis.\nShe was transferred to our institution 4 days later due to persistent headache despite treatment. A noncontrast computed tomography (CT) brain scan taken at our institution showed a hemorrhagic transformation of the right temporal lobe, which was not observed on the initial MRI (Fig. ). Follow-up lumbar puncture showed 510 white cells per mm3 (82% lymphocytes), 144 mg/dL protein and 61 mg/dL glucose. CSF culture studies were negative for bacteria, fungi and tuberculosis. PCR of the CSF confirmed the presence of HSV1. The patient was free of neurological symptoms, with a Glasgow Coma Scale of E4M6V5, and was admitted for close observation and continuation of iv acyclovir. Corticosteroid treatment was discontinued upon her admission. On day 3 of hospitalization, the patient presented with a sudden onset of vomiting and severe headache. Brain CT showed an increased amount of temporal lobe hemorrhage and a leftward shift in the midline (Fig. ). Mannitol was administered but did not seem to have a significant effect. The patient became increasingly drowsy, and her right pupil became dilated. She underwent emergency right decompressive craniectomy, expansile duraplasty and ICP monitor insertion. Postoperative brain CT showed alleviation of midline shifting (Fig. ). The patient recovered fully 5 days after the surgery. Apart from mild intermittent headache and dizziness, she did not show any other significant clinical symptoms, including neuropsychological problems. There were no significant neurologic deficits upon neurological examinations performed by the attending neurosurgeon and neurologist. The patient was discharged after completion of 2 weeks of acyclovir and returned 2 months later for cranioplasty. She was followed up 3 more times after cranioplasty. She was stable, without any neuropsychological problems or neurologic deficits, and was able to successfully return to work as a public official. |
A nine-year-old boy was referred to the Department of Medical Genetics for growth and developmental delay. The parents were first cousins and he was the first child of the family. He was born at term by Cesarean section due to breech position. Bilateral congenital cataracts (operated on at age two years), cryptorchidism on the right side, hypertonia and seizures were observed after birth. However, no further seizures occurred for about twelve months. The patient was reported to have severe developmental delay. He never learned to crawl or walk and did not say any words. He was fed by nursing bottle. His height and weight were within the normal range at birth, but below -2 standard deviations at all times after age 2 months.\nRhizomelic shortening of the arms, contractures at elbows and wrists, dolichocephaly, a flat occiput, maxillary hypoplasia, a long flat philtrum, a wide nasal base and hypoplasia of the anterior nasal spine were the dysmorphic features noted in the patient (). His younger sibling had died following surgery for kidney disease at age 2 months, but this sibling did not have the clinical features of RCDP (see pedigree, ). Atrial septal defect (ASD) (3x3.5 mm), a left to right shunt, a small secundum ASD and peripheral pulmonary stenosis on the left side were detected by echocardiography at age 2 years. Cranial computerized tomography performed at age 3 years was reported as normal. Digital electroencephalography (EEG) was performed at age 5 years when he was awake and resting. No focal or paroxysmal abnormality was noted on the EEG. Liver, portal vein, hepatic vein, gallbladder, spleen, kidneys and adrenal glands were all reported as normal in an abdominal ultrasonography performed at age 6 years.\nAt presentation, the patient was 9 years old. Rhizomelic shortening of the arms and calcifications at the elbow were observed. Ulnar hypoplasia and distal radio-ulnar dyplasia were detected on the X-rays (). Gas chromatography/mass spectrometry (Shimadzu GCMS QP 2010 SE) performed at Dicle University revealed the blood levels of very-long-chain fatty acids (VLCFA) as C22: 68.7 µmol/L (N: 0-96.3 µmol/L), C24: 61.5 µmol/L (N: 0-91.4 µmol/L), C26: 0.8 µmol/L (N: 0-1.3 µmol/L) and pristanic acid 0.12 µmol/L (N: 0-2.98 µmol/L), values which were in the normal range (normal levels given in parenthesis). Phytanic acid level was 125.85 µmol/L (N: 0-9.88 µmol/L), a value which was very high. We elected to sequence the PEX7 gene in the patient and his parents. Ethylenediaminetetraacetic acid blood samples were sent to the Research Institute of the McGill University Health Centre in Montreal, Canada, in the context of a research protocol (). Molecular genetic analysis was performed by Sanger sequencing of polymerase chain reaction amplicons of all 10 PEX7 exons and flanking intronic regions according to reported methods (). The results indicated that the patient was homozygous for an unreported PEX7 variant, c.192delT (p.F64Lfs*10). This variant was found in the heterozygote state in both parents (). It was not found in public exomes databases and is considered highly likely to be pathogenic and consistent with the diagnosis of RCDP type 1. The parents were informed about this disease and genetic counseling was provided. The family gave informed consent to use the X-rays, the patient’s photograph and test results for publication. |
A 60-year-old African-American male with a past medical history of a stab wound to the abdomen, post laparotomy years ago, presented to the emergency department with abdominal pain and distention for seven weeks. The pain was characterized as continuous, involving all quadrants, nagging, nonradiating, 7/10 in intensity, associated with decreased appetite, bloating, constipation, and weight loss of 50 lbs over seven weeks. The patient denied fever, chills, night sweats, nausea, vomiting, and blood in the stool. The patient endorsed shortness of breath on exertion and disturbed sleep due to abdominal distention. The patient had colonoscopy 1.5 years ago and reported it as normal. The patient denied tobacco, alcohol, illicit drug abuse, and he worked as a security guard. The patient’s vital signs were within normal limits and his physical examination was remarkable for abdominal distention with mild tenderness on deep palpation in all quadrants with rebound tenderness. Laboratory findings showed mild anemia with hemoglobin of 11.8 g/dL, hematocrit of 37%.\nThe patient first underwent computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast that showed an 8 cm mesenteric mass with surrounding stranding and poorly defined borders, no bowel ileus or obstruction but thickening of the descending colon through the sigmoid, with mild surrounding stranding (Figure ).\nOncology and general surgery services were consulted and the decision was made to proceed with exploration laparotomy and biopsy of mesenteric mass with frozen section. No metastatic disease or ascites were noted. There was a mass at the base of the mesentery involving the transverse mesocolon overlying the middle colic as well as the superior mesenteric artery. The mass area was ligated and wedge resection was performed and sent for frozen section. Tru-Cut biopsies of the retroperitoneal area were also sent. The pathologist was unsuccessful in identifying a diagnosis intraoperatively as what was mostly seen was fibrosis. More specimen was required, hence careful dissection was started. The base of the mesentery was opened and the mass was then carefully dissected. Resection was continued along the mesentery to the small bowel where the mass, as well as the small bowel, was excised. The results of the preliminary biopsy of the mesenteric mass and omentum showed fibro-adipose tissue with lymphoid hyperplasia, vague nodular collections of foamy histiocytes with giant cell reaction, marked chronic inflammation, fat necrosis, and prominent sclerosis/fibrosis (Figure ).\nImmunohistochemistry showed CD20 and paired box containing (PAX) 5-stained lymphoid follicles and scattered B cells, CD3-stained interfollicular areas and scattered T cells, and CD56 stained histiocytes (foam cells). Pan-cytokeratin stain was negative. Methenamine silver and acid-fast stains were negative for fungal and mycobacterial organisms respectively. Flow cytometry showed a mixed population of polytypic B-cells (32%) and T lymphocytes (49%) with no pan-T cells, no pan-T cell antigen deletion or B cell light chain restriction detected. Consultation with John Hopkins reference laboratory was obtained and immunohistostains done there showed increased IgG4-positive plasma cells (focally over 40 in one high power field) (Figure ).\nA Movat stain highlighted areas of phlebitis (Figure ).\nIn addition, there were pockets of tissue eosinophilia and focal storiform fibrosis (Figures -).\nEven though there were focal areas of fat necrosis, the overall features argued in favor of IgG4-related disease. Plasma IgG4 level was measured a few days after the resection of the mass and it was normal at 47 mg/dL (reference range 4.0-86.0 mg/dL). The patient had a protracted hospital course requiring two additional laparotomies for resection of the ischemic small bowel.\nThe patient was discharged with oncology outpatient follow up. A CT scan of the abdomen with contrast at three months showed a residual soft tissue mass-like density within the mesenteric fat anterior to the third portion of the duodenum measuring 6.4 x 2.7 cm. A CT scan of the abdomen repeated six months after the surgery showed that the mass-like density in the mid-abdomen had mostly resolved. |
A 73-year-old woman with no previous comorbidities or family history of hematological disorders or hypercoagulability was admitted to the Infectious Disease Clinic due to suspected HFRS and dehydration. Two weeks prior to disease onset, she had been exposed to bank voles while cleaning out a cabin. For 6 days following disease onset, she had been ill with fever, chills, weakness, low urine production, and difficulties eating and drinking. The patient had positive serology for Puumala virus thereby confirming the HFRS diagnosis. Laboratory tests taken the day before admission revealed thrombocytopenia (platelet count: 48 × 10\n9\n/L), impaired renal function (creatinine: 278 μmol/L), and leucocytosis (white blood cell count: 14 × 10\n9\n/L). Upon admission to the hospital, the platelet count had increased to 61 × 10\n9\n/L and creatinine increased to 370 μmol/L indicating clinical progression to the oliguric stage of HFRS. However, the platelet levels decreased to 12 × 10\n9\n/L on days 8 to 9. The treating physicians decided to transfuse platelets on days 8, 9, and 10 due to the high risk of spontaneous bleeding. Despite transfusion with three platelet units, the patient remained severely thrombocytopenic with platelet counts below 50 × 10\n9\n/L during days 8 to 13. The case is summarized in\n. Criteria for disseminated intravascular coagulation (DIC) were fulfilled from day 8 (see\nfor an overview of criteria).\nOn day 13 (2 days after the last platelet transfusion and a platelet count of 27 × 10\n9\n/L), the patient falls ill with abdominal pain which increases in severity during the evening. An abdominal computed tomography (CT) shows congestion and ischemia in the terminal ileum due to a thrombus in the superior mesenteric vein (SMV) reaching up to the portal vein (PV). The hematologist advised against thrombolysis due to thrombocytopenia in combination with a known mild VHF, which could increase the risk of bleeding. A national coagulation expert is consulted for further advice, who recommends anticoagulant treatment with heparin in a “careful” dose. Heparin at a dose two-thirds the national recommended dose is initiated with the aim of APTT 1.5 times the baseline value (40–50 s). The patient therefore receives a bolus dose of 4,000 units heparin followed by transfusion of 24,000 units heparin per day. The following day (15), the patient has gastrointestinal bleeding and a decrease in hemoglobin values from 100 to 89 g/L. According to the surgeon consultant, the stasis caused by the SMV and PV thrombus damages the intestinal mucosa leading to the observed gastrointestinal bleeding. Heparin is therefore continued in the same careful dose and the patient receives one unit red blood cells (RBCs) that day and the following day (16). The following criteria had to be fulfilled before mesenteric phlebography and thrombolysis via catheter could be considered: (1) platelet levels greater than 100 × 10\n9\n/L, (2) no bleeding, and (3) the patient can tolerate a full-dose heparin. On day 19, the patient fulfilled these criteria and underwent mesenteric phlebography via interventional radiology. Using the percutaneous transhepatic route to the PV, a hydrolyser 7F, double lumen, over-the-wire thrombolysis (Hydrolysis, Cordis Europe NV, Roden, the Netherlands) was used to perform a mechanical thrombolysis of the PV followed by pharmacological thrombolysis with tPA (Actilyse infusion: 0.8 mg/hour). A notation from the surgery department states that the previous heparin treatment aiming for 1.5 times APTT had been unsuccessful in decreasing the size of the SMV and PV thrombus. A control angiography 6 hours postsurgery shows that the thrombus distally in the SMV has been removed. There is still a thrombus between the portal and the splenic vein; therefore, the catheter is moved further into the area of thrombosis and thrombolysis by Actilyse administration is continued. At this time, there is no contrast leakage as a sign of hepatic bleeding. The patient stays in the intensive care unit (ICU) with local hydrolysis via the catheter. The following day (20), the patient becomes hypotensive with systolic blood pressure down to 75, and has signs of peritonitis. The levels of the fibrin degradation product D-dimer increases to 20 mg/L and fibrinogen decreases to 0.69 g/L. A CT thorax/abdominal scan shows an ongoing expanding hepatic intraparenchymal arterial bleeding. In addition, the CT scan shows presence of pulmonary emboli. Since the patient has a propensity for bleeding and thromboembolism, arterial intervention via the femoral artery into the aorta and then out into the common hepatic artery with coiling was not an option. Instead an emergency surgery procedure is performed with ligation of the right hepatic artery in the hepatoduodenal ligament, which stops the bleeding. In addition, the anticoagulant therapy is discontinued that day and the patient is tended in the ICU in a respirator. On day 21, the anticoagulant therapy is readministered at a low dose. The patient is extubated on day 22 and the anticoagulant therapy is increased to a target of APTT 60s due to remaining portal thrombi and peripheral pulmonary emboli. On the evening of day 25, the patient develops acute dyspnea, and oxygen saturation decreases to 88% with 4 L of oxygen and tachycardia. A CT pulmonary angiography shows pulmonary emboli in the right and left pulmonary arteries and peripherally in the pulmonary lobe arteries. The patient is transferred that evening to the ICU with heparin treatment at a target of APTT 85s. An ultrasound of the peripheral extremities (day 26) shows bilateral deep vein thrombosis in the posterior tibial veins. Since the APTT remains difficult to adjust (ranging from over 180s to the therapeutic target of 80s) and the propensity to develop thrombosis despite anticoagulation, it is decided to change the treatment from heparin to low-molecular-weight heparin (Fragmin 16,000 IU/day) on day 32. After day 55, the patient receives warfarin as prophylaxis against further thromboses and is discharged to her home on day 61. To rule out other causes for the thromboembolic complications, she was tested and found negative for activated protein C resistance. |
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 28-year-old male patient, a single child from a consanguineous marriage, reported to us for a routine dental checkup. He presented with hoarseness of voice since childhood. He gave history of occasional recurrent self-limiting oral ulcerations since 2 years. He gave no history of seizure episodes, visual disturbances, or any other systemic symptoms. Patient's family and medical history were noncontributory.\nOn general physical examination he was found to be moderately built and nourished. There was presence of waxy beaded papules on the margins of both eyelids () and areas of hyperkeratosis on the dorsal surface of the hands.\nOn intraoral examination, macroglossia was evident with crenations along the margins and there was absence of papillae on the dorsum surface. On palpation the tongue and the sublingual frenum were found to be stiff and limited mobility of the tongue was noted (Figures and ).\nThere was presence of diffuse yellowish waxy papular lesions throughout the oral mucous membrane which appeared nodular and rough on palpation ().\nA lateral skull view and posterioanterior view did not delineate the presence of any calcifications. On the basis of history and clinical appearance differential diagnoses considered were amyloidosis, lipoid proteinosis, myxoedema, and erythropoietic protoporphyria.\nComplete hemogram and thyroid profile values were found to be within the normal range. An incisional biopsy from the buccal mucosa and ventral surface of the tongue was performed. Similar histological findings and staining patterns were observed in the sections from both sites. Hematoxylin and eosin stained sections revealed stratified squamous parakeratinized epithelium and it was hyperplastic in nature. The tongue specimen showed spongiosis of epithelial cells in few areas. The connective tissue showed hyalinized fibrous stroma with fibers, fibroblasts, blood vessels, and proliferating endothelial cells. Numerous eosinophilic amorphous aggregates were seen in the connective tissue (). Similar type of deposit was also found around the blood vessels and the proliferating endothelial cells. Special staining with Congo red and crystal violet was found to be negative which ruled out the presence of amyloid tissue (). Periodic acid Schiff staining (PAS) was found to be positive, which showed deposition of pink hyaline PAS-positive material in the connective tissue and around the blood vessels ().\nAfter clinicopathologic correlation a final diagnosis of lipoid proteinosis was rendered. Patient was educated and made sentient about his condition; however patient was lost to followup. |
A 50-year-old woman presenting with back pain was referred to our center with a 2.5-cm nodule in the right middle lung field on a chest X-ray. The patient had a past history of surgical excision of an odontogenic tumor at a local dental clinic 17 years previously. A chest computed tomography (CT) scan revealed a 2.6-cm lobulated, heterogeneous enhancing nodule and a 1.5-cm satellite nodule in the anterior segment of the right upper lobe (RUL) (). Therefore, we performed CT-guided percutaneous needle biopsy for the tissue diagnosis of the main nodule. The specimen showed a cohesive tumor island composed of squamoid cells, with central keratin pearl-like material. There was only one event of mitosis in the whole field (), and the patient was diagnosed with squamous cell carcinoma. Bronchoscopy, brain magnetic resonance imaging, and positron emission tomography scanning were performed for further evaluation and staging, based on the impression of primary lung cancer. Then, we performed a planned RUL lobectomy and mediastinal lymph node dissection through thoracotomy upon the diagnosis of clinical stage IA (T1bN0) or IIB (T3N0) lung cancer. The resected specimen showed two separate nodular lesions that shared the same histopathological features. However, the histopathological diagnosis after the operation was different from that of preoperative biopsy. Postoperative histopathological examination revealed densely packed tumor islands. These tumor islands showed peripheral palisading and loosely arranged central cells, which resembled stellate reticulum. The peripheral palisading cells were columnar and hyperchromatic. Little mitotic activity and cellular pleomorphism were observed (). The overall features suggested a follicular pattern of ameloblastoma. We contacted the patient's former dentist and requested her medical records, but we could not obtain any information related to the odontogenic tumor. Finally, the diagnosis of metastasizing ameloblastoma was made based on the patient's past history and histopathological studies. The postoperative course was uneventful. Although the patient had no associated symptoms, panoramic radiography and facial CT were performed after consulting a dentist to identify local recurrence at the oral cavity. There was fibrotic scar formation but no evidence of recurrence at previous odontogenic tumor site. No adjuvant therapy was performed. The patient was doing well without any evidence of recurrence or metastasis during regular follow-up. |
A-66-year-old male, who was exsmoker, presented to the ER for a change in mental status. As per the family, the patient who had a normal mental status in the past was noticed to be progressively worsening for the last couple of months, with disorientation mainly found over phone discussions. At the time of presentation he was awake and alert, but disoriented to time and place. He denied any pain and was unable to provide any history. Physical exam was normal except for the presence of facial ecchymosis. The neurological part showed a normal motor, sensory, and cranial nerve exam. Also, his gait was stable.\nPast medical history was significant for hypertension, hypercholesterolemia, aortic valve replacement (nonmetallic), atrial fibrillation (on warfarin), incomplete heart block, pacemaker, and IgG- IgA, kappa type Monoclonal Gammopathy of Undetermined Significance (MGUS). Family history was not relevant.\nMGUS was diagnosed 9 months ago on serum protein electrophoresis (SPEP) as patient was referred to our institution's center for cancer and blood related diseases for hyperglobulinemia on routine blood work. At that time the patient underwent extensive work up to rule out any plasma cell dyscrasias. The Urine protein electrophoresis (UPEP) was negative for M spike. Immunohistochemistry and flow cytometry from peripheral smear as well as from bone marrow biopsy were negative for a clonal plasma cell or malignant B-cell. Chromosomal analysis and cytogenetics were also normal.\nIn the ER, a head CT scan revealed multiple parenchymal hemorrhagic lesions suspicious for metastases mainly in the frontal part bilaterally and in the left parieto-temporal region, with edema and compression mainly on the left lateral ventricle. Also, an interventricular lesion at the foramen of Monro causing mild hydrocephalus was seen. CT chest, abdomen and pelvis was done to find a possible primary malignancy and revealed numerous ground-glass and solid nodules in the lungs. Warfarin was discontinued and the international normalized ratio (INR) of 2.2 was reversed with fresh frozen plasma. Patient was started on steroids and phenytoin. Lower extremity duplex was negative.\nThe clinical status continued to deteriorate with cold, cyanotic fingers developing on day twelve of hospitalization. At this point he was seen by a vascular surgeon in order to rule out arterial emboli. A transesophageal echocardiogram was done for possible vegetations causing infectious emboli. All results were negative, and no infiltrative cardiomyopathy was found. Bronchoscopy and broncho-alveolar lavage with cultures (including nocardia) was also negative. CT guided lung biopsy showed H&E (hematoxylin and eosin) and PAS (periodic acid-schiff) positive amorphous eosinophilic material suspicious for amyloid (). Initial staining with congo red was negative. However, it stained positively with Thioflavin T stain and had weak green birefringence under polarized light. Immunostains for kappa and lambda chains showed stronger staining for kappa subtype. Brain biopsy was positive for eosinophilic material (similar to the lungs) but negative for congo red and thioflavin T stain (). Later, the patient had a respiratory failure secondary to health care acquired pneumonia (HCAP) and was intubated. Serial blood cultures and serologies for Cryptococcus and histoplasmosis, serology for antineutrophil cytoplasmic antibody (ANCA), antinuclear antibody (ANA), rheumatoid factor (RF), cryoglobulin, and Antiglomerular basement membrane antibodies (antiGBM) were all negative. Only seen was a positive blood culture for candida on day eighteen.\nPatient was initially treated with ampicillin and gentamycin for possible endocarditis. Later, he was switched to vancomycin, meropenem, and ciprofloxacin for possible health care acquired pneumonia. Also, he was started on amphotericin B after blood cultures revealed candida. Whether a more diffuse disease such as systemic amyloidosis is present was not confirmed, as the family refused any further diagnostic procedures that are not relevant to the acute care of the patient in a context of a deteriorating clinical picture. Despite all antifungals and supportive measures, he failed weaning trials and remained on ventilator support. A tracheostomy was suggested. However, after extensive discussions, the family refused. On day 29, health care proxy decided to withdraw all life support measures based on the patient's previous wishes who did not want to be kept alive on a ventilator. Finally, the patient had a terminal wean after these events, and the family refused any autopsies. |
Patient presentation
A 21-year-old female college student with a history of asthma presented to the neurosurgery office for consultation complaining of mass on the left side of her skull associated with increasing size over the past two days and intermittent headaches for the past two to three weeks. The left-sided headache included her upper jaw. She also reported a history of cellulitis and urinary tract infections, in addition to surgical removal of an impacted wisdom tooth in 2016. Family history was positive for diabetes mellitus (DM) type II in both her father and her grandfather and colon cancer and coronary artery disease in her other grandfather. She admitted to drinking alcohol one to two times per week but denied use of tobacco and drugs. At the time, she was taking Viorele birth control to regulate her menses. Review of systems was otherwise negative.
Clinical findings
Physical examination revealed a well-developed, well-nourished female in no acute distress. She was awake, alert and oriented to person, place and time with a Glasgow Coma Score (GCS) of 15. A soft left frontal lesion associated with tenderness to palpation, without erythema or drainage, was palpated slightly off midline. Her cranial nerves II-XII were intact. Strength in both upper and lower extremities was five out of five bilaterally. No pronator drift was noted. Sensation to light touch was intact bilaterally in V1-3, upper extremity, and lower extremity distributions. Her reflexes were symmetric. Her gait was within the normal limits.
Imaging
CT of the head without contrast (Figure ) revealed an expansive soft tissue mass with beveled edges and dimensions measuring approximately 3.5 x 2.1 x 2.3 cm in the left frontal calvarium. Bony destructive changes of the inner and outer table of the left frontal calvarium were apparent. Extension of the mass into the dura was noted. The mass did not extend into the |
We present a 10-years-old girl with a history of tissue swelling involving the third digit of left hand, bilateral wrists and ankles. At 2 years of age, she first developed soft tissue masses above both wrists and ankles without pain, warmth or redness. She did not receive any treatment and the soft tissue masses enlarged slowly. Two years later, she underwent surgery to remove the ankle masses and pathology was reported to be consistent with chronic bursitis. However, swelling gradually recurred near the surgical sites within 1 year.\nAt 6 years of age, a new mass appeared on the palmar aspect of the left hand at the base of the third digit (). The mass was resected and histology illustrated hallmark features of GCTTS including an abundance of multinucleated giant cells (; see for full images). This diagnosis was confirmed by three independent pathologists that reviewed the histology. One year later, the patient started experiencing joint pain and gradual decline in the range of motion of wrists and ankles. Pain and swelling affected additional joints including bilateral elbows and the fifth PIP joint of left hand.\nWith progression of symptoms, the patient was referred to our Rheumatology Clinic for evaluation. Physical examination was notable for symmetric soft tissue masses along the anterior tibialis tendon of ankles and extensor tendons of the wrists (). She had tenderness and reduced range motion of those joints, suggestive of active synovitis. Laboratory investigations including complete blood count, C-reactive protein, erythrocyte sedimentation rate, ferritin, HLA-B27, T-Spot, rheumatoid factor, anti-cyclic citrullinated peptide antibodies and anti-nuclear antibodies were all unremarkable. Serum creatinine, transaminases, and lipid profile were within normal limits. X-ray showed early bone destruction of the left fifth PIP and the left wrist (). Musculoskeletal ultrasound revealed nodules with enhanced blood flow in the wrists and ankles. Re-evaluation of slides from her previously resected ankle masses revealed chronic bursitis with multinucleated giant cells and rare granuloma-like structures ().\nMRI of wrists showed synovial thickening with abnormal signals around the tendon sheath, with evidence of wrist bone erosions (). Ophthalmology and dermatology examination did not reveal any abnormalities. Given the unusual features and progressive tenosynovitis, whole exome sequencing (WES) was performed for the patient and parents. The patient was started on etanercept (25 mg SC weekly) and methotrexate therapy (15 mg PO weekly) for possible polyarticular juvenile idiopathic arthritis while results were pending.\nWES revealed a de novo heterozygous mutation in NOD2 (nucleotide-binding oligomerization domain protein 2; exon 4; c.1001G>A; p. Arg334Gln) that was not found in either parent (). This mutation is a known pathogenic variant responsible for EOS/Blau syndrome (, ), an autoinflammatory syndrome characterized by granulomatous arthritis, uveitis and dermatitis. The de novo mutation and lack of family history favor the nomenclature of EOS, which typically describes sporadic cases while Blau syndrome refers to cases with familial inheritance. Given the early onset of symptoms and overlapping pathology findings, EOS likely explains the multifocal inflammation with multinucleated giant cell involvement in this patient. The patient's therapeutic response was also similar to others with EOS/Blau syndrome as treatment with etanercept and methotrexate led to steady improvement of arthritis and reduced soft tissue swelling near the wrists and ankles. After 1 year, her physical examination showed no evidence of joint swelling, joint tenderness or restricted range of motion. Marked improvement of tendinous inflammation in the anterior tibialis tendon was also demonstrated by MRI (), although residual enhancement was still evident despite the lack of symptoms or physical exam findings. |
A 75-year-old man visited our emergency department complaining of cough and sputum lasting one month. There was no definite mediastinal widening on a simple chest X-ray, but a prominent aortic arch was observed in comparison to the past two years. A three-dimensional computed tomography scan revealed multiple aneurysmal dilatations with pseudoaneurysm and thrombi in the aortic arch and proximal abdominal aorta. They were measured to be 2.7×1.1 cm (external diameter: 5.1 cm) and 1.5×3.6 cm, respectively, in size. Another focal aneurysmal dilatation was observed in the distal abdominal aorta just above the iliac bifurcation (). Upon admission, transthoracic echocardiography showed moderate aortic regurgitation and minimal mitral regurgitation without regional wall motion abnormalities. We planned a staged operation (thoracic endovascular aortic repair for aortic arch aneurysm first and aortic valve replacement later) as the patient was considerably old and had poor pulmonary function.\nThoracic endovascular aortic repair (36×100 mm, SEAL thoracic limb stent graft; S&G Biotech, Seongnam, Korea) was performed as previously described []. There was a 12-mm-long landing zone between the left subclavian artery and aortic arch aneurysm with an aortic diameter of 30 mm. Considering the possible type Ib endoleak caused by the mild aneurysmal dilatation of the descending thoracic aorta, we planned to position two overlapping stent grafts strategically (one just distal to the left subclavian artery, and the other covering the descending thoracic aorta). Balloon angioplasty was not performed. The patient was monitored in the intensive care unit postoperatively, transferred to the general ward on the first postoperative day, and discharged on the fifth day without any specific symptoms or signs. No endoleaks were found on the computed tomogram on the second day (). The patient follow-ups in the outpatient department revealed no symptoms except for the retrograde type A aortic dissection observed on the three-month computed tomogram performed as a routine follow-up study ().\nThe patient underwent aortic valve replacement and ascending aorta replacement under deep hypothermic circulatory arrest and selective unilateral antegrade cerebral perfusion. The aortic valve was replaced with a Carpentier-Edwards no 23. tissue valve (Edwards Lifesciences, Irvine, CA, USA). The intimal tear had originated from the bare metal structure in the anterior direction, which was in contact with the anterior wall of the ascending aorta. This bare metal structure was partially exposed after the excision of the dissected aorta. Distal aortic anastomosis was performed without the removal of the stent graft and reinforced circumferentially with pledgeted sutures. Distal aortic anastomosis sutures partly included the proximal bare metal structure of the stent graft. The pump time, aortic cross clamp time, circulatory arrest time, and selective antegrade cerebral perfusion time were 234, 144, 40, and 32 minutes, respectively.\nThe patient was extubated on the first postoperative day, transferred to the general ward on the third postoperative day, discharged on the eighteenth day, and followed up for 24 months without specific symptoms. Endoleaks were observed in the junction of the distal anastomosis site and the bare metal structure of the stent graft on the computed tomogram on the seventh day, but had no significant interval changes on the computed tomogram after 20 months (). |
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 38-year-old man visited our hospital complaining of anterior chest pain. He had no significant medical or family history, and the vital signs were stable. Ischemic events were not observed in electrocardiography, but chest X-ray and computed tomography (CT) showed a cystic lesion (6.0 × 7.0 × 10.0 cm) in the anterior mediastinum (Fig. a). Although the cystic capsule demonstrated contrast enhancement, its fluid component had low radiation absorbance. Based on these findings, we suspected the mass to be a thymic cyst. Blood tests indicated the presence of inflammation (white blood cell count 11,200/μL and C-reactive protein 3.38 mg/dL).\nTwo days after hospitalization, the patient developed dyspnea and his chest pain worsened. Subsequent chest CT showed that the cystic lesion had become inhomogeneous and the radiation absorbance of the cyst’s fluid component had increased (Fig. b). The cyst wall became thickened, and bilateral effusion was observed. Blood tests indicated that hemoglobin levels had decreased from 15.8 to 12.8 g/dL, and levels of inflammatory markers had increased, with the fever exceeding 38.5 °C. Needle aspiration biopsy and tumor wall biopsy with a small skin incision were performed; however, we could not obtain a diagnosis. One week after admission, general condition and laboratory data of the patient gradually improved. A chest CT on day 13 showed that the tumor had become small in size with a thickened wall (Fig. c). The effusion on the right side had decreased and that on the left side had disappeared.\nThe patient had recovered enough to undergo surgery; the tumor was resected by sternotomy on day 18. The tumor was found to be encased in a smooth, yellow, and elastic coat. The tumor was densely adhered to the junction of the left brachiocephalic vein and superior vena cava, and it was required to detach the tumor from the dense adhesion site carefully. The right phrenic nerve was preserved, and the right pleural effusion was serous. The tumor and thymic tissue were resected en bloc. The operative time was 288 min, and the estimated blood loss was 521 mL. The resected tumor was covered with a thick, fibrous capsule, and the lumen was filled with necrotic tissue and hemorrhagic material (Fig. a, b). The postoperative course was uneventful, and he was discharged on day 26.\nThe pathological findings showed a fibrotic cyst wall; the cyst was filled with necrotic tissue. The slight proliferation of lymphocytes was confirmed in the necrotic tissue and around the cyst wall (Fig. a, b). The tumor was diagnosed as type B1 cystic thymoma (Fig. c). As the tumor did not appear to have spread beyond the capsule, it was determined to be at Masaoka stage I. Nevertheless, the dense adherence of the tumor to its surrounding tissue indicated the possibility of invasion, and postoperative radiotherapy (50 Gy) was administered.\nTwo years after the surgery, recurrent metastasis of the tumor was found on the right pleura and the left upper lobe of the lung. The patient was treated with chemotherapy, radiotherapy, and local resection. The patient remains alive 12 years after the first surgery. Following an analysis of the tissue obtained from the resected recurrent tumor, the pathological diagnosis was changed to type B3 thymoma. |
A 26-year-old nulliparous lady presented to the gynecology department with lower abdominal pain, fever, and foul smelling discharge per vaginum since 2 days. She had undergone dilatation and evacuation for missed abortion 2 weeks back. It was a spontaneous conception, 2 years following marriage, and early pregnancy evaluation at 6 weeks revealed a viable singleton pregnancy in the right horn of a bicornuate uterus and a 9 cm × 7 cm cystic lesion with internal echoes in the region of left adnexa, posterior to urinary bladder. Repeat scan at 8 weeks revealed absent cardiac activity and evacuation was carried out at a local hospital. Immediate postabortal period was uneventful.\nShe was febrile with significant tachycardia and no pallor. Abdominal examination revealed tenderness in left iliac fossa and hypogastrium with no palpable mass. Vulval inspection revealed asymmetry at the introitus with a bulge on the left side and unhealthy vaginal discharge. Swab was taken for C and S. Per speculum examination not carried out due to severe tenderness. On bimanual examination cervix felt high-up and to the right side, cephalad and posterior to a tender cystic mass of about 10 cm × 8 cm on the left side, uterine body normal in size, and tender on palpation. Lab parameters showed normal Hb, TLC – 12,500 with neutrophilia, and highly elevated CRP. USG was reported as uterus didelphys (as the uterine horns were widely separated) with a large collection of 10.3 cm × 6.8 cm with pockets of air seen on the left side, posterolateral to the bladder, normal ovaries and absent left kidney. An obstructive mullerian anomaly was suspected and MRI was carried out to confirm the same [Figures –].\nPatient was started on antibiotics and planned for surgery. Local examination findings were reconfirmed before starting the procedure. Left lateral vaginal mass was palpated and Foley's catheter placed to define the limits of the bladder. There was a tiny fistulous opening on the vaginal septum 4 cm above the hymenal ring. Diagnostic laparoscopy was carried out [Figures and ]. The extent of the left paravaginal mass noted and limits of the bladder were identified []. Cruciate incision was made transvaginally, at the site of the tiny fistulous opening on the longitudinal vaginal septum, and around 200 cc of foul smelling, frothy, purulent material drained. Specimen was sent for C and S. Digital palpation through the cruciate incision helped in defining the extent of the vaginal septum. It was excised using electro cautery until the left cervix was reached. Fine absorbable sutures were placed on the resected septum to attain hemostasis. Post-operative period was uneventful and she conceived 3 months after surgery. |
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 25-year-old female patient was referred from an outside hospital in November 2011 for the management of dysphagia. The patient initially presented to a different hospital in 2008 with a 4-year history of heartburn and acid regurgitation and was diagnosed with GERD. The patient did not complain of dysphagia or globus symptoms at the time, and the esophagogastroduodenoscopy (EGD) performed at the previous hospital showed grade B erosive esophagitis according to LA classification. A 24-hour intraesophageal pH study showed a DeMeester score of 33.1 (normal value, <14.2), a total fraction time of pH <4 of 9%, and abnormal acid regurgitation when the patient was upright (upright fraction time of pH <4, 24%). Preoperative esophageal manometry showed normal lower esophageal sphincter (LES) relaxation during swallowing (resting LES pressure 14 mm Hg to LES relaxation 2 mm Hg), and no peristalsis was observed in the esophageal body (). A favorable response to medical treatment (proton pump inhibitor) was not achieved, and subsequent laparoscopic Nissen fundoplication was performed in September 2009 in a previous hospital. Thereafter, the patient developed postoperative complications such as solid and liquid dysphagia, a sensation of inability to belch, and a sticking sensation in her lower to mid chest. Approximately 2 to 3 weeks after the operation, the patient's symptoms showed improvement. However, regurgitation recurred and was soon aggravated to dysphagia. Dysphagia was worse with solids than with liquids, and these symptoms occurred whenever the patient swallowed food. Medical therapy with proton pump inhibitors and prokinetics was attempted in the previous hospital but was ineffective. The patient was then referred to our hospital. EGD performed at our hospital showed postfundoplication status, and the endoscope could pass through the gastroesophageal junction without any resistance (). The previously observed erosive esophagitis was improved. Esophageal mucosal biopsies ruled out eosinophilic esophagitis. Abnormal barium stasis in the esophageal body was found on barium esophagography (). A paraesophageal hernia was observed on abdominopelvic computed tomography (), which was performed to evaluate the patient for postoperative organic causes of dysphagia. Esophageal manometry showed aperistalsis in the esophageal body, and the resting pressure and percent relaxation of LES were 5 mm Hg and 81%, respectively, which were within the normal range (). In our hospital, medical treatment (prokinetics, mosapride 15 mg; proton pump inhibitor, esomeprazole 40 mg; calcium channel blocker, nifedipine 5 mg) was initiated and continued for 2 months; however, a favorable outcome was not obtained. Pneumatic dilatation with a 30-mm balloon was used for symptom relief, but the symptoms did not improve. Therefore, additional pneumatic dilation with a 35-mm balloon was performed 2 weeks later. Barium esophagography performed after the second pneumatic dilatation showed improved barium passage through the esophagus, but the solid and liquid dysphagia, and globus sensation symptoms were not improved (). We considered esophageal motility disorder or paraesophageal hernia that developed after fundoplication as the most likely cause of the dysphagia. Therefore, we recommended a revision operation that would involve taking down the wrap or Toupet fundoplication. However, the patient refused to consent to a second operation and is currently undergoing medical treatment. |
A 65-year-old Caucasian female with prior history of hypertension and hyperlipidemia presented with unsteady gait, weakness in her left leg, and blurry vision. Patient had intermittent symptoms for last few weeks; however, she did not seek any medical attention. In view of her persistent symptoms, she was evaluated by medical team for a possible neurological cause. Review of systems was otherwise unremarkable. She takes simvastatin and hydrochlorothiazide for dyslipidemia and hypertension, respectively. There were no other significant past medical or surgical history to contribute for the presenting complaints. Clinical examination revealed decrease in power in left lower extremity with preserved reflexes. All routine blood tests were within normal limits. A magnetic resonance imaging (MRI) (Figures , , and ) scan of the brain revealed four enhancing brain masses largest in the right parietal lobe and others in right frontal and temporal lobe and left parietal lobe, respectively. Further staging workup included examinations of the eyes, head, and neck mucosa, total skin, gynecological evaluation, bone scintigraphy, and computed tomography (CT) scans of abdomen and pelvis () that showed a filling defect in terminal ileum as the only pathological finding. Colonoscopy revealed a partially pigmented polypoid lesion in the terminal ileum and a biopsy done was suggestive of malignant melanoma (). Further pathological evaluation revealed it as epithelioid variant with ulceration and negative for BRAF oncogene (). Patient underwent resection of terminal ileum with end-to-end anastomosis. After resection she underwent chemotherapy with ipilimumab and dacarbazine every three weeks for 4 cycles. After chemotherapy she received 30 Gys of whole-brain radiotherapy (WBRT) for ten sessions. Followed by this she had a Cyberknife, (Accuray Inc., Sunnyvale, CA) an image-guided robotic radiosurgery. The treatment procedure included CT image acquisition based on skull-bone landmarks, planning, and radiation dose delivered at 18–20 Gys based on size of lesion for three treatments. Despite aggressive treatment patient succumbed to the disease in 14 months. Our index case accounts for the less than 2% of cases reported in literature as a rare presentation of primary malignant melanoma in the gastrointestinal tract (GI) without evidence of any primary skin lesion or any other sites with multiple brain metastases. |
A seven and half year-old boy visited the outpatient clinic of Pediatric Dentistry Department, Faculty of Dentistry, Cairo University in June 2015 with a chief complaint of pain on the lower right molar area. The patient’s mother stated that the pain was at times throbbing in nature, and child is not able to chew on this side.\nClinical examination showed a badly decayed, lower second primary molar with related localized intraoral abscess, where the lower first primary molar was intact. The patient had poor oral hygiene; he had not received any professional dental care, and was very apprehensive.\nRadiographic examination revealed root resorption and bone rarefaction related to lower second primary molar. The interesting finding was a considerable amount of root resorption of the distal root of the adjacent lower first primary molar (\n).\nThe case was managed by performing pulpectomy\n to the lower second primary molar, with root canals filled with calcium hydroxide paste with iodoform (Metapex, Meta Biomed, Republic of Korea). The tooth was then restored with high viscosity glass ionomer (GC Fuji IX GP capsule, GC corporation, Tokyo, Japan) (\n). The lower first primary molar was not touched and instead monitored. No antibiotics or analgesics was prescribed.\nUnfortunately, the patient’s mother did not want follow-up appointments in person, however, she was contacted on the phone, after 2 weeks, 3 months and 6 months, and she said everything was fine and there was no swelling or pain.\nAt about 8 months from the treatment appointment, the patient’s mother visited the outpatient clinic with the patient for other reasons, and decided to pass by the Pediatric Dentistry Department for patient follow-up. Clinical examination showed no signs or symptoms, occlusal restoration was intact, and radiographic examination revealed arrested root resorption, on both molars, and an increase in the density of bone although this was not at a normal level yet (\n).\nshows the patient’s timeline of symptoms, treatment and follow-up. |
The deceased donor was a 67-year-old man with a kidney Doppler ultrasound (DUS) that was negative for any nodular lesion. As part of the routine postoperative follow-up management, the recipient underwent DUS to assess the patency of the graft on postoperative day 1. The DUS finding was suspicious for an acute arterial thrombosis but did not reveal any focal irregularities. Consequently, a computed tomography (CT) scan was urgently obtained but it did not show any arterial complications. However, it serendipitously revealed a 2.4-cm lesion on the upper pole of the renal allograft which was not detected during the back-table or ultrasonography monitoring. A biopsy of the lesion was performed, and its histology revealed an epithelial proliferation of large cells with finely granular cytoplasm and medium round nucleus vesicular acidophilus, arranged tubules, and alveoli and cords immersed in a connective tissue stroma. This picture was consistent with oncocytoma. However, because the eosinophilic variant of chromophobe renal cell carcinoma (RCC) may morphologically resemble renal oncocytoma, immunohistochemical staining was performed using Ki-67 antibodies and RCC antigens. The results were negative, ruling out chromophobe RCC. The therapeutic options and potential related outcomes were clearly discussed with the patient. Given the low risk of malignant transformation in an oncocytoma [], we found no reason for resection of the lesion or an allograft nephrectomy. Consequently, we opted for active surveillance of the benign tumor with ultrasonography, every 2 months, for the first year and, then, with magnetic resonance imaging (MRI), every year (Fig. ). The patient received mycophenolate-mofetil, tacrolimus, and prednisone throughout the 5-year follow-up period and the regimen for immunosuppression was not changed despite the presence of the renal mass. After 60 months of active surveillance, we report that radiological studies have shown no growth, regression, or any other interim morphological changes to the lesion, and the patient is alive and well (Fig. ). |
A 36-year-old Brazilian male patient was admitted to the hospital with a palpable lump in his right breast, located at the junction of the upper quadrants of the right breast (Fig. ). On physical examination, the lesion appeared firm with irregular margins. Axillary lymphadenopathy was negative and there were no palpable supraclavicular nodes. On breast imaging, ultrasonography showed a hypoechoic mass with partially defined contours measuring 4.0 × 3.0 cm, located at the upper region of the right pectoralis major muscle at the 12 o’clock position with muscle infiltration (Fig. ). Histological examination of core biopsy samples revealed a malignant tumor. Preoperative exams, such as X-rays and chest CT scan, abdominal US did not show any signs of disease. Radical mastectomy was then performed, due to pectoralis major muscle infiltration, consisting in removal of the breast along with the major and minor pectoralis muscles. Biopsy of the sentinel lymph node was performed. Gross examination revealed a solid tumor measuring 3.7 × 3.5 cm with a yellowish-tan cut surface and local foci of hemorrhage. Histopathology showed intravascular papillary proliferation of endothelial cells, spindle cell areas and necrosis, atypia and prominent mitotic figures, consistent with the diagnosis of high-grade angiosarcoma with areas of infiltration of the pectoralis major muscle (HE staining, magnification of 400×) (Fig. ). Histopathology also demonstrated a surgical specimen with clear margins, absence of angiolymphatic and perineural invasion, in addition to sentinel lymph node free of metastasis. Immunohistochemical study revealed a tumor positive for CD31 marker (Fig. ), confirming the vascular nature of the tumor. At the two-week follow-up of the surgical procedure, adequate wound healing was observed, without any evidence of the disease. The patient was transferred to the clinical oncology department, where he presented with severe headache and seizures after the second cycle of adjuvant chemotherapy with paclitaxel. Magnetic resonance imaging of the brain was ordered, revealing a right frontal parasagittal lesion, measuring 1.3 × 1.1 cm with a hemorrhagic component and perilesional edema, suggestive of brain metastasis. The disease progressed rapidly, culminating in the patient’s death at 20 days after the onset of neurological symptoms. |
In August 2010, a 30-year-old male financial adviser presented to the hospital having awoken with painless weakness and patchy numbness of his left upper limb. He had slept the previous night with his girlfriend sleeping on his left shoulder. Prior to this, he had been well without any significant neurological disease. His 59-year-old father had been diagnosed with Parkinson's disease 5 years ago and had peripheral neuropathy as well as carpal tunnel syndrome. He also had a 32-year-old sister who was in good health.\nOn examination, he was a tall, lean man, with a height of 195 cm. He had weakness of the muscles of the proximal left upper limb, particularly the shoulder girdle. He was unable to abduct his left shoulder and had weakness on elbow flexion. Maximum weakness was observed in the supraspinatus, infraspinatus, deltoid, rhomboid, and triceps muscles, with a relative sparing of other muscles in the distal upper limb. Reflexes were preserved. The working diagnosis was that of a brachial plexopathy.\nThe MRI scan of his spinal cord and brachial plexus, performed pre- and post-gadolinium, were normal. Nerve conduction studies, which were performed on August 9 2010, indicated an underlying peripheral neuropathy with mixed features to suggest an axonal and demyelinating polyneuropathy (table ). Median and ulnar sensory nerve action potentials (SNAPs) recorded using both palmar stimulation and ring electrodes were absent. The radial SNAP was also absent. The distal median motor latencies were mildly prolonged bilaterally with borderline conduction velocities; F-waves prolonged in latencies on the right and absent on the left. The ulnar motor studies showed borderline distal motor latencies from stimulation at the wrist, mild slowing of conduction in the forearm without slowing above the elbow segments on both sides (data not shown); the F-waves revealed a latency prolongation – more marked on the left than on the right. Right common peroneal response showed a mildly prolonged distal motor latency from stimulation at the ankle with reduced amplitude and moderate slowing of conduction in the leg. F-waves were prolonged in latency, even when considering the patient's height. Right posterior tibial response had a normal latency, amplitude, and moderately reduced conduction velocity with prolongation of the F-wave latencies. Both right medial plantar and right sural SNAPs were absent. The F-wave prolongations were not consistent with the patient's height of 195 cm (where relevant).\nElectromyography (EMG) showed a markedly reduced recruitment pattern, without features suggestive of active denervation, in most of the muscles sampled, in particular the left deltoid, abductor digiti minimi, supraspinatus, and rhomboid muscles. His father's nerve conduction studies also showed a mixed picture compatible with axonal degeneration and demyelination.\nThe clinical picture, nerve conduction studies, and EMG raised the possibility of HNPP as a diagnosis, and gene testing confirmed a PMP22 deletion on chromosome 17. Advice was given in regard to the protection of nerves from external pressure, in particular, avoiding his partner leaning or sleeping on his arms, not leaning his elbows on chairs or desks, and avoiding below-knee boots which might compress the common peroneal nerves below the fibula heads. He was also informed about the genetic nature of HNPP. A genetic review was organised, and as the patient expressed an interest in discussing family planning issues, particular emphasis was placed on advising about the genetic risk to his children as well as the implications of HNPP. Written informed consent was obtained from the patient for the publication of this case report. |
A chest CT scan of a 63-year-old, 62 kg, 156 cm female patient revealed a lung tumor. She was admitted into hospital for a left lower lobectomy. Her medical history only showed well-managed hypertension and diabetes mellitus. The preoperative blood tests appeared normal. A simple chest X-ray revealed a left lower lobe solitary pulmonary nodule and a calcified nodule in the right lung.\nAfter inducing general anesthesia, endotracheal intubation was performed with a double-lumen tube for one-lung ventilation. After confirming the modified Allen test, an arterial catheter was inserted at the right radial artery for continuous monitoring of the blood pressure and arterial blood gas analysis (ABGA). Subsequently, a central venous catheter (CVC) was inserted into the left internal jugular vein. When operating on the left lung, placing the patient in the right lateral recumbent position allows manipulation of the left internal jugular venous catheter. In addition, it is possible to quickly recognize any mechanical damage to the operation site when inserting the CVC.\nTo insert the CVC, the patient's head was lowered 5° and the neck was rotated approximately 15° to the right. The internal jugular vein was punctured with an 18-gauge needle with ultrasound guidance. After vein puncture, a J-tip guide wire was inserted and the needle was removed, the insertion site for the catheter was dilated with a venodilator, and a 7 Fr. central vein catheter (Two-lumen Central Venous Catheterization Set with Vantex®, Edward Lifescience Co., USA) was inserted. Subsequently, the aspiration of blood by the two lumens of the catheter was observed, and the catheter was secured at a depth of 18 cm below the skin after checking the CVP waves.\nThe patient was placed in the right lateral recumbent position for surgery. Surgery was performed after performing one lung ventilation. The concentration of sevoflurane and the dose of the remifentanil infusion were adjusted accordingly to the vital signs, and the ventilator was set in pressure-control mode (peak air way pressure at 21 mmHg, respiratory rate at 11, inspiratory to expiratory time ratio at 1 : 2). After starting one lung ventilation, the ABGA was pH 7.47, PaCO2 31 mmHg, PaO2 264 mmHg, and bicarbonate (BE) 22.6 mmol/L at FiO2 1.0. The respiration rate was adjusted so that PaCO2 would be between 36 and 40 mmHg. There were no abnormal findings during surgery, and the surgeon inserted the left chest tube. The total time under anesthesia care was 7 hours and 40 minutes. The CVP was kept at 6-11 mmHg, and no changes in the wave forms were observed. Blood loss during surgery was estimated to be approximately 50 ml with a urine output at 350 ml. The total amount of fluid administered was 1,500 ml, of which 200 ml was a saline and 400 ml was pentastarch (Pentaspan®, Jeil Pharm, S. Korea) injected into the central vein.\nImmediately after surgery, a chest X-ray was taken with the patient in the supine position which led to an abnormal finding in the right lung (). Atelectasis was found in the right upper lobe apex after obtaining the opinion of a thoracic surgeon. Accordingly, the right bronehus was checked with a fiberoptic bronchoscope. There was no secretion or blood clot, nor was there any blockage found in the airway found, so positive-pressure ventilation was performed. An additional chest X-ray showed normal findings (). Consequently, the muscle relaxants were reversed, spontaneous respiration was restored, and the double-lumen tube was extubated. The patient was provided oxygen via a mask and transported to the intensive care unit (ICU) for observation.\nOn the day of surgery, the patient in the ICU reported no other pain or discomfort other than that at the operation site. Her vital signs were normal. She did not complain of breathing difficulty. On postoperative day 1, her vital signs were stable. However, on a simple chest X-ray, the opacity of the right lung increased and the mediastinum was shifted to the left (). Consequently, a thoracentesis was performed. 700 ml of pleural fluid was drained. The glucose level was 107 mg/dl with a protein level <1 g/dl, so it was estimated to be effusion fluid. Two days after surgery in the morning, the breathing sounds from the right lower region reduced. A chest X-ray revealed an increase in opacity of the right lung. A 28 F. chest tube was inserted and 1,400 ml of pleural fluid was drained. The cause of the right-side pleural effusion was believed to be due to movement of the central vein catheter, so the catheter was removed. After observing a decrease in pleural fluid leaking into the chest tube, the patient was moved to the general ward. In the ICU, the CVP was stable at 7-12 mmHg and no changes in the wave forms were ob served. Eight days after surgery, the patient had no other complications and was discharged from hospital. |
On August 27, 2020, a 28-year-old woman, was admitted to the chest clinic in Rania Medical City (RMC) private hospital, she presented with gradual swelling in the left supraclavicular region, at the beginning the mass was painless, but within 1 month the pain started with no past medical and surgical history.\nSubsequently, physical examination revealed a hard, painful, tender, non-pulsative mass at the left supraclavicular region (), on the course of the external jugular vein, the mass in the first month was enlarging during lying on the bed. The skin overlying the mass did not have any signs of inflammation. The vital signs within normal limits. Regarding laboratory findings, there was no significant alterations. Additionally, there was no history of cervical trauma, cannulation of the neck vessels, prior hospital admission, or previous thromboembolic or oncological diseases. There was no cervical lymphadenopathy. The patient did not have symptoms in the otorhinolaryngological, thoracic or abdominal areas. She did not have night sweat, and neither weight loss, nor fever, no traveling recently. Chest, abdomen examination, and blood tests were normal. The patient was referred for a confirmatory US evaluation from a qualified radiologist, which revealed a 2 cm cystic mass without any increase in size with Valsalva maneuver, was not compressible. The mass was diagnosed as a branchial cyst in the neck.\nOn September 3, 2020, after explaining the procedure to patient the cardiothoracic and vascular surgeon with his team decided to surgery to remove a branchial cyst. On September 3, 2020, the patient underwent surgery by the first author, with local anesthesia in the supine position, with tilting of the neck toward right side and some extension. After good disinfection, and covering of the patient, through a transverse incision, the cyst was looks like venous aneurysm, black, uncompressible, and connected to the external jugular vein proximally. The cyst was extended toward the chest under clavicle. Due to an inflammatory reaction, there was adhesion around the cyst had been removed. Proximal and distal control of the external jugular vein without resection of the clavicle was performed. Ligate and trans-fixation of the external jugular vein was done without any complications. The cyst was excised, after opening the cyst intraoperatively it was clotted. Histopathological examination was made, and diagnosed as thrombosed external jugular vein aneurysm. On September 12, 2020, the stiches have been removed.\nAfter two days remained at critical care unit, and the patient was discharged to home for outpatient follow-up. Additionally, after 2 months, the patient did not have any additional local or general events during the follow-up period, and there was no recurrent swelling. |
A 10-year-old boy presented with five days of right sided abdominal pain associated with vomiting, diarrhoea, and anorexia. The patient's medical history included craniosynostosis, attention deficit hyperactivity disorder, asthma, and migraines.\nOn examination, the patient had tenderness and voluntary guarding in the right upper quadrant, and McBurney's sign was negative. The patient was afebrile and was not jaundiced. His admission blood tests showed a high white cell count (21.4 × 109/L), with no left shift. Liver function tests, C-reactive protein, and amylase were unremarkable.\nThe abdominal ultrasound showed no evidence of cholelithiasis or cholecystitis, and the diameters of the intrahepatic and extrahepatic bile ducts were within normal range. The appendix was not visualised and no free fluid was present. At the time of reporting, there were no abnormalities identified in the area of the liver or the falciform ligament.\nThe patient was admitted and observed for possible early appendicitis. The inflammatory markers returned to normal, but the abdominal pain persisted. Given the atypical presentation, computed tomography (CT) of the abdomen and pelvis with intravenous and oral contrast was performed. The CT showed hazy increased density of fat and inflammatory changes centred around a focal area of fat, which was anterior and inferior to the left lobe of the liver and adjacent to the falciform ligament. These features suggested incarcerated fat with inflammatory change. These radiological signs are similar to those recognised for epiploic appendagitis and omental infarction, which appear as areas of focal fat infarction and local inflammatory changes, elsewhere in the abdomen. In view of the site and the relevant literature, this CT was in keeping with torsion of a fatty appendage of the falciform ligament (Figures and ).\nObservation of the patient continued, with oral analgesia used to control pain. The patient did not have an exploratory laparotomy or laparoscopy. The pain resolved after four days, and the patient was then discharged. On review two months later, the pain had mostly resolved. |
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. |
A 25-year-old female presented to the emergency department (ED) for evaluation of persistent productive cough of yellowish sputum over the last four week and mild exertional dyspnea over the last two years. Her past medical history was unremarkable and she took no regular medications. There was no personal or family history of multiple endocrine neoplasia type 1 (MEN1) syndrome. She was in no distress on presentation to the ED with a resting hemoglobin oxygen saturation of 97% while breathing room air. Her physical examination was remarkable for absent breath sounds and decreased tactile fremitus on the left middle and lower lung fields. No wheezing or stridor were heard. Laboratory data were within normal limits.\nA chest x-ray (CXR) in the ED demonstrated opacification of the left middle and lower lung fields, hyperinflation of the right lung and deviation of the trachea to the left (Fig. ). A computerized tomography (CT) scan of the chest showed complete left lung atelectasis due to a mass obstructing the left main bronchus and excessive mediastinal deviation to the left with substantial herniation of the hyperdistended right lung into the left hemithorax (Fig. ). There was no evidence of tracheobronchial narrowing in the right lung or esophageal compression. The mass was well demarcated and of soft-tissue quality, demonstrating homogeneous contrast enhancement, starting 2.8 cm distal to the main carina, measuring 4.4 × 2 × 2.8 cm (Fig. ). Abdominal and head CT scans showed no abnormal findings. The patient subsequently underwent a diagnostic flexible bronchoscopy which revealed a pale hypervascular polypoid mass completely obliterating the left main bronchus which was biopsied using forceps (Fig. ). Histopathological examination of endobronchial biopsies disclosed a carcinoid tumor with a Ki-67 index of approximately 10%.\nFollowing thoracic surgery consultation, an open left pneumonectomy with concurrent complete lymph node assessment and dissection was performed. During surgery, the left lung was found completely atelectatic with adhesions between the pericardium and the left pleura which were dissected. No attempt of repositioning the mediastinum or placement of tissue expanders was performed, due to the absence of airway compression in the right bronchial tree during previous bronchoscopy and CT scan. The patient recovered well after surgery and no complications were noted. Post-operative histopathology disclosed an atypical carcinoid with a Ki-67 labelling index of 10% but no areas of necrosis (Fig. ). There was a radical resection of all tumor with clear operative margins, the periphery of the left main bronchus was infiltrated by tumor, but there was no invasion of the visceral pleura, and no infiltration of resected lymph nodes from lymph node stations 5, 7, 9 and 10 by carcinoid cells.\nPre-operative spirometry was as follows: FEV1: 1.51 lit (44% predicted), FVC: 1.54 lit (39% predicted), FEV1/FVC: 98%. Spirometry and static lung volumes 12 months after surgery were as follows: FEV1: 1.93 lit (58% predicted), FVC: 2.34 lit (61% predicted), FEV1/FVC: 82%, TLC: 3.28 lit (63% predicted), RV/TLC: 118% predicted. Although spirometry appears to be significantly improved after surgery, spirometry before surgery triggered fits of coughing and therefore preoperative values might not be representative.\nPostsurgical follow-up has included the following: Initial chest CT scan was carried out 2 months after surgery. Parathyroid hormone (PTH) and prolactin levels were within normal limits 1 year after surgery. The following investigations were carried out at 6 months and then every 6 months for the first 5 years: Chest CT scan, abdominal ultrasound, chromogranin A measurement and standard laboratory testing including complete blood count, renal function, liver function, calcium and glucose. Abdominal CT scan and fiberoptic bronchoscopy were carried out 1 year after surgery and then will be carried out annually for the first 5 years. Bronchoscopy would be performed earlier for any symptoms or imaging findings suggestive of local progression. Repeat chest CT scans after surgery showed no changes in mediastinal rotation compared to those prior to surgery, and no signs of tracheobronchial or esophageal compression. Repeat bronchoscopy showed a normal-appearing surgical stump of left main bronchus and no airway compression of the right bronchial tree. The remaining studies listed above have been normal. The chronic mild exertional dyspnea reported by the patient before surgery completely resolved on hospital discharge, 7 days after pneumonectomy. The patient has been asymptomatic for the last 16 months after surgery with excellent performance status. |
A three year-old girl presented with abdominal pain and jaundice of one month duration. The pain was epigastric in origin, and worsened after eating. Her history was unremarkable except for blunt trauma following a car accident. The child was sitting on her father’s lap while he was driving whenthe accident occurred.After trauma the child was visited by a physician because of vomiting. When ultrasound and physical examination of the child was normal she was discharged and given metoclopramide drops. Over the next three days, she had nonbiliary vomiting which stopped thereafter. The child complained of occasional abdominal pain which was finally complicated by icterus. She appeared healthy and tolerated feeding well, although occasionally complaining of postprandial abdominal pain. She did not have a fever and her vital signs were normal. The physical examination showed a soft abdomen with no tenderness but revealed jaundice. The laboratory data results wereas follows: WBC = 12600 mm3, Hb = 12 g/dL, ESR = 25 mm/h, total bilirubin = 8.4 mg/dL, conjugated bilirubin = 6.9 mg/dL, aspartate aminotransferase (AST) = 559 IU/L, alanine aminotransferase (ALT) = 475 IU/L and alkaline phosphatase = 938 IU/L. A new ultrasound examination showed remarkable dilatation of the common bile duct to 8 mm with a terminal stricture. Barium meal showed a normal stomach and an open duodenum. Magnetic resonance cholangiopancreaticography (MRCP) demonstrated severe dilatation of the both intrahepatic and extrahepatic bile ducts ().\nThe patient was operated with a diagnosis of obstruction of the end of the common bile duct. The severe dilatation was obvious upon operation. The common bile duct was explored. A small catheter was passed with difficulty which pointed to severe stricture and fibrosis,not amenable to dilatation. The patient underwent choleducoduodenostomy. On the third day postoperatively, the patient started oral feeding. On the fifth day postoperatively, the serum bilirubin decreased significantly and the patient was discharged in good general condition. |
A 50-year-old man visited our hospital with a complaint of weak stream, residual urine sensation, and perineal discomfort. Previously, he had been diagnosed with benign prostatic hyperplasia and had been treated with medication for more than two years at local urologic clinics. Otherwise, he was generally in good health. For these months, he had been experiencing worsening weak stream and urethral pain without any hematochezia or change in bowel habits. Digital rectal examination revealed non-specific findings for the prostate, except that it was apparently enlarged. The serum level of prostate-specific antigen was normal (0.85 ng/mL). Transrectal ultrasonography revealed that a huge prostatic mass measuring 97×88×84 mm was well capsulated with internal hemorrhage and that the mass was isolated from the surrounding structures (). The radiologist recommended abdominopelvic computed tomography (CT) and magnetic resonance (MR) imaging for an evaluation of the prostatic mass. CT scan showed direct invasion to adjacent organs with no metastasis. MR imaging of the prostate showed an enlarged prostatic mass with hemorrhagic necrosis. The prostatic mass was large (110×85×86 mm) with heterogeneous enhancement, displacing the bladder anteriorly and rectum posteriorly (). This implied that the tumor was mainly localized within the prostate and there was no definite evidence of a direct invasion of adjacent organs. The patient then underwent five-core prostate biopsy guided with transrectal ultrasound. Histologically, the tumor was composed of spindle cells with vesicular nuclei and eosinophilic cytoplasm. These cells were arranged in a whirling or fascicular pattern. There was no significant nuclear pleomorphism (). Mitotic counts were more than five per 50 high-power fields. The tentative diagnosis was prostatic stromal sarcoma. Tumor cells from the biopsy specimen showed strong and diffuse immunohistochemical reactivity to KIT (CD117) and CD34, while negative immunohistochemical staining results were obtained for desmin, smooth muscle actin, cytokeratin, and S-100 (). These outcomes are concordant with the diagnosis of GIST. Therefore, we concluded that the final diagnosis was primary prostatic extra-GISTs. Tumor genotyping was not carried out due to the high costs of this examination. To evaluate the tumor stage, the patient underwent gastroscopy and colonoscopy for the primary lesion, and chest CT and bone scan for the distant metastasis, but there were no abnormal findings in these examinations. We did not start neoadjuvant treatment with imatinib because of its high cost and the patient's lack of medical expense insurance. Therefore, we planned to perform radical prostatectomy routinely; if the tumor had involved the rectum, we would have additionally performed colostomy after complete tumor resection. The patient changed his mind a day before the operation; he refused to undergo radical surgery and left the hospital against our advice. Therefore, we have not followed-up on him since then. |
On April 23, 1986, a 3-year-old girl was brought to our institution after a 1.5-ton truck ran over her left lower limb. She suffered a floating knee injury with fractures at the supracondylar level of the femur and middle third of the tibia. An above the knee and near-total amputation was sustained at the supracondylar level, with a complete degloving of the thigh up to the inguinal area (Fig. ). There were no other injuries.\nReplantation started at 9 h after injury. The supracondylar fracture was fixed with Kirschner wires, followed by intramedullary nailing of the tibial fracture using a Steinmann pin. The popliteal artery was anastomosed using a 5-cm vein graft from the contralateral saphenous vein after which two popliteal vein anastomoses were performed. The peroneal and tibial nerves were then repaired followed by repair of the quadriceps femoris, hamstrings and triceps surae. The degloved skin was defatted and was re-applied as a full-thickness skin graft to the thigh.\nThe patient was stable postoperatively. A few days after surgery, skin and muscle necrosis of the gastroc-soleus complex developed, requiring repeated debridement before a split-thickness skin graft could be applied (Fig. ). Two months after the index surgery, the patient had begun rehabilitation exercises for the lower extremity. After removal of the Steinman pin, an anteromedial external fixator was applied for two and a half months on the tibia in order to facilitate range of motion and weight-bearing exercises. The patient was discharged 1 month later.\nAt 4 years after the injury, the patient was ambulatory without assistance and no leg length discrepancy was noted at the time; knee flexion was noted at more than 90° and ankle dorsiflexion was to 10°. Six years after injury, the patient was able to stand on the affected limb without support, but soft tissue contractures had developed in the ankle and knee joints. No intervention was done at this time. When patient was 11 years old, 8 years after the injury, the knee flexion contracture was severe and a Z-plasty of the popliteal area to release the scar tissue contracture (Fig. ) was carried out. With continued rehabilitation, the patient was able to regain full knee extension though flexion was limited to 90°. She was ambulatory without support but left with an equinus deformity of the left ankle. The leg length discrepancy had become more pronounced at this time of a growth spurt. She underwent her last major surgery when she was 12 years old; a femoral osteotomy was done, and an external fixator was applied for gradual lengthening to correct the 6-cm shortening of the left lower extremity.\nAt the last review in September 2009, the patient was 25 years old. At 21 years after the incident, she remained freely ambulatory but with an equinus deformity of the left ankle. The knee range of motion was maintained at 0–90 degrees of flexion, the foot was sensate, and she was also able to move her toes (Fig. ). Radiographs taken showed left lower extremity was shorter but the fractures and osteotomy sites had completely remodeled (Fig. ). In 2012 through a telephone interview, the patient described her knee movement as unchanged and had no new complaints. She was satisfied with the functional outcome of her left lower limb despite it not being esthetically pleasing. Aside from the operative scars from the skin grafts, the limb was thinner from severe reduction in muscle bulk over the leg where debridement had been done. The patient was content that she was walking with her own limb with intact sensation and had avoided a prosthesis. |
A 28-year-old Asian woman (G3P1) who had undergone emergency cesarean delivery owing to a compound presentation at full term was referred to our institution with a suspicion of abnormally located gestational sac. She had undergone laparoscopic cholecystectomy and open appendectomy previously. She did not have any medical, family, or psychosocial history. She had missed her menstrual period without any other symptom and visited a private obstetrical clinic to confirm the pregnancy. However, she was diagnosed as having an abnormal pregnancy such as cervical or CSP by USG.\nAt our institution, she reported that her last menstrual period was just 5 to 6 weeks prior. However, USG revealed a gestational sac in the anterior lower uterine segment with a fetus measuring 4.83 cm crown-rump length (CRL) with positive cardiac activity, corresponding to 11 weeks and 6 days of gestation. Color/power Doppler images depicted a hyperechoic rim of a choriodecidual reaction with excessive vascularity (Fig. ). Although we could observe a definitive abnormally located gestational sac, our patient did not have any pain during the physical examination. She admitted that her last menstrual period was different from her usual menstrual periods. Because CSP or cervical pregnancy was suspected, we performed computed tomography (CT) for a definitive diagnosis. The CT scan showed an intrauterine gestational sac in the lower uterine segment bulging through the anterior uterine wall at the site of the cesarean scar. No invasion of the urinary bladder was observed (Fig. ). On presentation, her β-human chorionic gonadotropin (β-hCG) level was 66,536.8 IU/L (Day 1). Initially, we injected 50 mg of methotrexate (MTX) mixed with 9 mL of normal saline in the amniotic sac through a 22-G needle transabdominally under USG guidance. Simultaneously, 2 ml of amniotic fluid was aspirated for termination of the pregnancy. However, fetal cardiac activity was still observed 2 days later (Day 3), without significant changes in the serum β-hCG levels (65,342.5 IU/L). We decided on laparotomy instead of laparoscopy because of the large CRL (Day 4). The intraoperative finding showed bloody amniotic fluid, blood clot, placenta, and a fetus at the lower segment of the uterus. A transverse uterine incision was made at the lower segment of the uterus (Fig. ). The gestational sac was removed, as well as most of the trophoblastic tissues that were adherent and invading the wall of the lower uterine segment. The fetus and placenta showed no definitive abnormalities (Fig. ). The estimated blood loss was 1.2 L at intra-operation, without immediate complication. The uterine defect was repaired into two layers by using 2–0 Vicryl sutures. Our patient received 3 units of packed red blood cells (PRBC) at the ward postoperatively. The serial β-hCG level was 1958 IU/L at 4 days after the surgery (Day 8). She was discharged in good condition 5 days after the operation (Day 9). After 1 month (Day 39), her β-hCG levels returned to normal (2.8 IU/L). She was very satisfied with the fact that she had recovered well without the need for intensive care or further treatment without the need for hysterectomy. |
This is a case of 70 years old female, with no known co-morbidities, who presented to the Otolaryngology clinic with complaints of increased somnolence and reduced appetite for past the 3–4 years. These complaints were not associated with any other associated symptoms. Her past medical history and family history were unremarkable. She had a history of the cesarean section under spinal anesthesia 30 years ago. On examination, she was well oriented to time, place, and person. She had a heart rate of 64 beats per min, a respiratory rate of 16 breaths per min, a blood pressure of 110/70 mm Hg, and she was afebrile. Her general physical and systemic examination was also unremarkable. Her serum calcium was 14.1mg/dl (8.8–10.6) and serum parathyroid hormone showed a value of 457mg/dl (7-53). Her vitamin D level, serum albumin, and creatinine were all within a normal range. 24-hour urinary calcium and phosphorus were also within normal limits. She underwent neck ultrasonography which showed a complex solid and a cystic nodule on the left side close to the lower pole of the thyroid measuring 3.6 × 2.8 cm ().\nThe clinical picture was suggestive of a parathyroid adenoma and she was admitted electively for its resection. After relevant investigations and anesthesia fitness, she underwent complete excision of the left parathyroid lesion and left lobectomy of thyroid gland under general anesthesia. Intra-operative findings were enlarged left parathyroid gland which was inseparable from the multi-nodular left lobe of the thyroid gland. A recurrent laryngeal nerve was identified and saved. Neck drain was placed and the sample was sent for histopathology (). The lesion was not involving any other structure other than the thyroid gland. No level VI lymphadenopathy was observed intraoperatively.\nThe patient remained hemodynamically stable with no signs of recurrent laryngeal nerve palsy. Her post-op serial serum calcium monitoring was done which decreased to normal range and remained normal. No other signs of hypocalcemia were noted and serum calcium was within a normal range. The patient remained stable and hence was discharged as post-op day 2 on oral calcium supplements and analgesia.\nAt 1-week follow-up her serum calcium was 8.9mg/dl and serum parathyroid hormone was 16.3pg/ml. Histopathology revealed a neoplastic lesion composed of closely space clusters of medium-sized ovoid to polygonal cells with pale to mildly eosinophilic cytoplasm, distinct cell borders, and centrally placed round to ovoid nuclei showing prominent nucleoli. Moderate cytological atypia and nuclear pleomorphism were seen. Occasional mitotic figures were consistent with parathyroid carcinoma. The tumor was seen invading into the thyroid parenchyma 0.4cm away from the resection/capsular margin ().Her repeat serum calcium was also within the normal range. MEN syndrome workup was done which was negative. The patient was seen regularly for 1 year and then has been on regular follow up yearly for past three years. She has been doing well with no symptoms and signs for recurrence of disease. Hence she has an overall disease free survival of three years with only complete surgical resection of tumor and no chemotherapy or radiotherapy. |
An 82-year-old male was admitted to our hospital because of cough with blood-streaked phlegm. These symptoms had been sustained for two weeks. He had smoked 30 cigarettes per day for the past 60 years. The patient did not experience dyspnea, fever, chest pain, body weight loss, or poor appetite. His medical history and family history were unremarkable. Physical examination on admission revealed a clear breathing sound; moreover, abnormalities in neck size as well as supraclavicular lymph nodes were not detected. The results of the laboratory tests were within the normal range except for a mild normocytic anemia.\nOn performing routine chest radiography, a mass lesion on the right upper lobe was discovered (). Contrast-enhanced CT revealed a lobulated soft-tissue mass that measured 3.7 cm in the largest dimension on the axial plane in the medial region of the right upper lobe (RUL). In addition, the image showed encasement of the RUL bronchus, mediastinal invasion, and enlarged lymph nodes in the pretracheal retrocaval space, subcarinal space, and the right hilar region (). Bronchoscopic examination revealed extensive submucosal and lymphangitic infiltration with partial obstruction of the right upper lobe orifice and a whitish keratinized tumor, which bled easily upon touch, and partially occluded the right anterior segment of the lower lobe bronchial lumen (RB7, right anterior segment of the lower lobe bronchial lumen) (, ).\nPathologic examination of the pulmonary specimen of the RUL bronchus revealed small cell lung cancer (SCLC), which was positive for chromogranin-A and negative for CD45. The pulmonary specimen of the bronchus of RB7 showed moderately to poorly differentiated non-small cell lung carcinoma (NSCLC). In addition, the morphological features and immunohistochemical results of the tumor cells from the two distinct regions of the right lung were different (). These results were not consistent with the characteristics reported for metastatic cancer of pulmonary origin. However, both pathologic specimens showed a strong positive reaction for p53, implying that similar carcinogenesis caused these two different types of lung cancers. We also performed an abdominal sonography and a Tc-99m whole body bone scan. The findings were unremarkable. The patient refused further examinations, was discharged and was not available for a follow-up visit. |
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. |
The patient is a 50-year-old female with a history of hypertension, hyperlipidemia and obesity that presented to an outpatient gastroenterologist for routine screening colonoscopy. The patient was found to have a lesion, measured at ~2.5 centimeters in size, near the ileocecal valve. Biopsies were taken at the time of colonoscopy, which demonstrated evidence of a well-differentiated neuroendocrine tumor with invasion into the submucosa. There was no increased mitotic activity (Ki-67 < 3%). The patient was referred to surgery clinic for further evaluation.\nOn assessment, the patient denied any symptoms related to her neuroendocrine tumor, including flushing, increased sweating, increased heart rate, wheezing, shortness of breath, diarrhea, weight loss or appetite changes. The only significant family history was a paternal and maternal grandfather with colon cancer. Imaging results were significant for computed tomography (CT) of chest demonstrating very small, but multiple, pulmonary nodules. A CT abdomen and pelvis demonstrated the known neuroendocrine tumor near the ileocecal valve () as well as two poorly visualized liver lesions (–). The magnetic resonance imaging (MRI) demonstrated two separate one-centimeter lesions in segment 5 and 7 of the liver ( and ). The portal vein lacked normal left and right bifurcation; there was a circumferential right portal vein, which coursed anteriorly and superiorly and ultimately to the left lobe of the liver.\nAfter workup was completed, the patient was consented for laparoscopic right hemicolectomy for removal of the primary tumor. The procedure was performed in a lateral to medial fashion and a hand-sewn extracorporeal anastomosis was performed after removal of the specimen. The operation was uneventful and the patient recovered appropriately in the immediate postoperative period. She was discharged home tolerating a regular diet and having normal bowel function. Final pathology demonstrated a 3 × 2 × 1.7 cm well-differentiated neuroendocrine tumor. Margins were clear and 8/19 lymph nodes were positive. There were 1/10 mitoses per high power field. Ki-67 was noted to be <1%. The final stage was T3N1M1 (stage IV) by the grading of American Joint Committee on Cancer (AJCC), and the tumor was G1 based on WHO classification.\nThe patient was seen postoperatively in clinic and has continued to recover well. She underwent repeat imaging that demonstrated stability in the size and location of metastatic disease. She remains asymptomatic. Oncology will continue to follow the patient with yearly bloodwork and repeat imaging. Discussions regarding the next step in management are ongoing, with the understanding that if she develops symptoms or demonstrates enlargement of the metastatic lesions on imaging, the need for intervention becomes more acute. |
A 62-year-old man with a reported past medical history of uncontrolled hypertension was seen at our hospital emergency department due to worsening confusion over the last 24 hours with an acute exacerbation of confusion, witnessed by friend 30 minutes prior to the initial evaluation. Upon arrival, he had no specific complaints. He was on a daily 81 mg aspirin and multiple blood pressure medications. He denied any other past medical or surgical history. He had no family history of diabetes; he had no history of coagulopathy but was unsure about any family history of any neurological disease or cancer. Social history was significant for smoking two cigarettes a day for 15 years but quit 10 years ago. On exam two hours after initial emergency department evaluation, he was alert and oriented to person, place, time, and event, but did elicit some confusion and had a Glasgow Coma Scale (GCS) of 14 (eye-opening - 4, verbal response - 4, motor response - 6). Cranial nerves II-XII were grossly intact, yet horizontal gaze was noted to be mildly abnormal. The remainder of his neurological exam was unremarkable with pupils equal, round, and reactive to light; sensation to light touch intact to upper and lower extremities; 5/5 grip strength as well as in the upper and lower extremities; deep tendon reflexes (DTRs) 2+/4 in upper and lower extremities bilaterally; no dysdiadochokinesia appreciated; and no truncal ataxia. The heart was found to be at a regular rate but bradycardic. No heart murmur was noted, and blood pressure was not elevated.\nSTAT computed tomography (CT) brain without contrast showed parenchymal hemorrhage of the right basal ganglia and intraparenchymal hemorrhage of the left lentiform nucleus (Figure ). Comprehensive metabolic panel (CMP), complete blood count (CBC), and lipid panel were all within normal limits, except HDL of 25 mg/dL. A urine drug screen was negative. EKG demonstrated bradycardia at 55 bpm, but otherwise a normal EKG. A portable chest X-ray showed no acute cardiopulmonary disease.\nThe patient was admitted to the ICU overnight and recovered an additional 48 hours on the telemetry floor. His hospital course was unremarkable except for one dose of hydralazine PRN, and his systolic blood pressure remained less than 160. Neurosurgery was consulted, and no neurosurgical intervention was indicated. A magnetic resonance imaging (MRI) brain scan was obtained on the first day of admission demonstrated that hematoma was subacute in the bilateral basal ganglia, as seen on the initial scan (Figure ) with no evidence of arteriovenous malformation (AVMs). MR neck angiogram with and without contrast showed evidence of mild atherosclerotic luminal narrowing of the right internal carotid origin and a hypoplastic left vertebral, which is usually developmental. An echocardiogram with a bubble study demonstrated a left ventricular ejection fraction greater than 60% with no wall abnormalities, no significant valvular regurgitation or vegetations, no thrombi identified, and no bubble contrast shunt from the interatrial septum. The patient was discharged after reaching the maximal benefit of hospital stay and is following in a hospital-associated outpatient clinic with the primary care provider (PCP) and neurology. |
Mr. B is a 63-year-old Caucasian male with a history of an ID and epilepsy; he has lived in a group home setting since he was 20 years old. A chart review showed that Mr. B had previously been able to independently perform many activities of daily living until the fifth or sixth grade. He was noted to have had gradual neurological deterioration from age 13 until age 17, with impaired speech, loss of the ability to walk independently, and a change in his seizure pattern. His intelligence quotient (IQ) was estimated to be around 30 at that time.\nWhen he was 25 years old, Mr. B was noted to have periods of hostility, agitation, and aggression for which he was prescribed sodium amobarbital intramuscular injection as needed and oral thioridazine 300 mg/day. He was reported to engage in property destruction such as tearing linens, taking light fixtures and air conditioning units off of the wall, throwing filters on the floor, taking all the drawers out of his dresser and emptying the contents on the floor, turning over furniture, and lifting his bed high enough that the mattress would fall off. Some of these episodes were reported to occur while he was withdrawn, quiet, and sullen, with poor eye contact, psychomotor retardation, hypersomnia, decreased appetite, low energy level, low interest level, and lack of motivation.\nWhen he was 33 years old, he was found with a toothpick pushed into his genitalia that caused bleeding. At that time, Mr. B was on mesoridazine, orally 75 mg/day, and benztropine, orally 4 mg/day. When he was 38 years old, he was admitted for the first time to a psychiatric hospital for evaluation. At that time, he was found unclothed on the sun deck of the group home in what appeared to be an attempt to jump off the brick railing on the third floor. He also was noted to be increasingly withdrawn, staying mostly in bed and refusing to get up, eat, or take his medications. He would stay under his bed for days, refusing to come out of his room. Mr. B was started on carbamazepine and was discharged back to his group home after a month-long hospitalization. At age 41, he was started on doxepin for “recurrent destructive attempts.” The following year, he was evaluated by a psychological consultant after increasingly unusual and bizarre behavior. His speech was marked by delusional themes concerning “the cross” and being crucified. He was also noted to have periods of “posturing and waxy flexibility.” He was described as noncommunicative and rigid, holding his arms up in the air for long periods of time. He also had periods of staring off into space or appearing distracted and not reality-based. Mr. B would also refuse to talk, eat, take his medications, or participate in daily hygiene and grooming. At that time his diagnosis was changed to schizoaffective disorder. Over the next ten years he was tried on multiple different medications including haloperidol, risperidone, venlafaxine, and paroxetine for psychosis and mood lability but continued to demonstrate episodes of withdrawal, abnormal posturing that the staff would call “statue man,” and social isolation, followed by periods of intense agitation with intermittent self-injurious behaviors and aggression towards staff.\nSeven months prior to evaluation, Mr. B had an episode of agitation that was rated by the staff using the KANNER scale [] and was given a score of 88 for Part 2. At that time, he was taking haloperidol, aripiprazole, lamotrigine, and valproic acid. The episode of agitation lasted about ten hours and ended within minutes after he received an intramuscular injection of lorazepam 1 mg (after two previous 0.5 mg doses of oral haloperidol that did not appear to work). During this episode, he was noted to be lying in the middle of the living area naked, trying to bite and kick staff and scratching his shoulder, and he had defecated and smeared feces in his bed, was constantly moving himself off of the safety mats, and was pounding his feet in a rhythmic motion on the floor and was also refusing to eat. Additionally, Mr. B was noted to have a “general blank stare on his face” with pursed lips and squinted eyes and tense facial muscles. He required four staff members to provide him with adequate hydration, nutrition, and medications.\nOver the next seven months, Mr. B had at least five milder episodes of similar behavior, all of which showed improvement in behavior after intramuscular injections of lorazepam. Seven days prior to evaluation, he was started on oral lorazepam 3 mg/day (1 mg three times daily). This resulted in a six-week period free from such episodes until he had an episode where he was found to be lying on the floor, mute, and uncooperative. His repeat KANNER score was rated at 46 for Part 2.\nA psychiatric consultation with the senior author was requested regarding the frequent episodes of agitation that were not controlled with his prior medication regimen. The treating psychiatrist could not wait one week for the evaluation and had started lorazepam. He requested the consult to have his diagnosis of catatonia verified and the chronic treatment with lorazepam approved. After seven days on 3 mg/day of lorazepam, the patient no longer had any catatonic signs. It was determined that during the prior seven months he had met the following eight out of the twelve criteria for catatonia: stupor, mutism, negativism, posturing, mannerism, stereotypy, agitation not influenced by external stimuli, and grimacing. If the DSM-5 had been available at that time, he would have met criteria for catatonia associated with a neurodevelopmental disorder. The patient had no history of depressive or manic episodes and no history of specific schizophrenia symptoms such as bizarre delusions or the typical auditory hallucinations. Additionally, similar to the patient in the previous case, this patient also was noted to have a generalized tremor in his trunk, head, and arms and was diagnosed with a cerebellar tremor.\nMr. B had no relapse in catatonic symptoms for four years after getting stable doses of lorazepam up to 8.5 mg/day. |
A 51-year-old male weighing 131 kilograms (kg) presented to the emergency department (ED) via ambulance with altered mental status and slurred speech after undergoing cervical epidural injection with two milliliters (mL) of 2% lidocaine (40 milligrams [mg]) under fluoroscopic guidance in an ambulatory setting. He became unresponsive during the injection with subsequent brief convulsive activity for which he was given 2 mg of midazolam. Emergency medical services was called and found him obtunded with shallow breathing and low oxygen saturations requiring ventilation assistance. On arrival to the ED his breathing was spontaneous and erratic with low oxygen saturations. He remained somnolent with slurred speech, unable to answer questions appropriately or follow commands. Preparations were made for intubation given altered mental status and low oxygen saturations; however, oxygen saturations and mental status improved within the first 10 minutes of arrival and ultimately intubation was not required.\nOn cardiopulmonary monitor he was noted to have an irregularly irregular heart rhythm. Electrocardiogram showed atrial fibrillation with a rate of 82 beats per minute. Hemodynamically he was stable. He converted to normal sinus rhythm 20 minutes later. He reported no history of atrial fibrillation. Within 60 minutes of ED arrival the patient’s mental status was back to baseline without recollection of the events that had occurred after the start of the procedure. He only had chest wall pain, possibly from sternal rub or from any bystander chest compressions that may have been performed when he became unresponsive. Imaging studies obtained included the following: chest radiograph, computed tomography (CT) of the head, CT angiogram of the head and neck, and CT of the chest with intravenous contrast. No pertinent imaging abnormalities were identified. Serum/plasma levels of lidocaine and its primary active metabolite, monoethylglycinexylidide (MEGX) were obtained 15 minutes after patient arrival. Both levels returned undetectable. The patient was observed overnight in the hospital and remained asymptomatic and without any further dysrhythmia. He was discharged home the following day on aspirin 325 mg daily and with a referral to outpatient cardiology. |
A 74-year-old male patient suffering from severe back and both legs radiating pain was transferred to our hospital. The magnetic resonance imaging (MRI) taken at the previous hospital showed an epidural mass at L1-2. Besides, there was a spinal stenosis at L4-5 and a spondylolytic spondylolisthesis at L5 (). The patient had suffered from neurogenic claudication for the last 10 years and had received epidural steroid injections from time to time. Eleven days before his transfer he was troubled due to multiple tryouts of an epidural injection. Severe back pain and both legs radiating pain were developed after that night. MRI was done the next day and SEH compressing the cauda equina was found at L1-2. The conservative treatment failed to ameliorate the symptoms. Thus, he was transferred to our hospital. On neurological examination, there was a diffuse sensory decrease in the left lower extremity, the motor power was intact. On laboratory findings, platelet count, bleeding time, clotting time, and prothrombin time were within normal limits. Activated partial thromboplastin time was slightly extended with 36.4 seconds (). As the patient had neither a past history of abnormal bleeding nor any medication of anti-platelet agents and there was nothing specific in his laboratory findings, we couldn't consider any bleeding disorder. His SEH was suspected to be related to the unsuccessful epidural injections. Carelessly, we did not give attention to the fact that the location of the hematoma was different from the site of epidural injections. We performed the hematoma evacuation and the surgery for L4-5-S1 simultaneously. All surgical procedures were uneventful. The symptoms had improved after the operation. However, the pain in the right leg re-developed after 6 hours. So, the MRI was retaken. During the MRI, his blood pressure dropped to 90/60 mm Hg and a complete paraplegia developed. The MRI showed large hematomas at L1-2 and L4-S1 compressing the cauda equina (). An emergency evacuation of hematomas was done. The bleeding tendency increased compared to the previous surgery. After the revision, neurological symptoms began to improve immediately. The repeated SEH raised our doubts about his bleeding tendency. Thus, we examined the clotting factors and got to know that factor VIII was 28% of normal, indicating a mild hemophilia A. The clotting factor was not replaced because there was no sign of neurological deterioration any more. At day 14, the MRI was repeated. There were still hematomas on both sites, but there was no compression to the dura mater anymore (). The neurological symptoms were fully recovered at month 2. |
A 25-year-old woman was referred for review of a long-standing history of exertional dyspnoea since infancy.\nShe had a history of recurrent lower respiratory tract infections in her first year of life, associated with respiratory distress and diffuse interstitial changes. There was no family history of respiratory illness and both parents were healthy. On each admission, she was administered oxygen and antibiotics for presumed aspiration pneumonia. At five months, she was readmitted to a paediatric unit; multiple investigations were performed, including fibre-optic bronchoscopy, immunoglobulins, sputum cultures, sweat electrolytes, and milk precipitins, which were all unremarkable. A radionuclide gastro-oesophagram (milk scan) only revealed moderate-to-gross reflux in the prone position without evidence of pulmonary aspiration.\nAn open lung biopsy was obtained at 11 months of age which demonstrated desquamated pneumocytes and a few foam cells (Fig. ) as well as fibrous thickening of alveolar septa and epithelialization of lining cells (Fig. ) with immunohistochemistry for CD68 highlighting prominent intra-alveolar macrophages (Fig. ) and cytokeratin showing prominent enlarged pneumocytes (Fig. ) leading to a histopathological conclusion of pulmonary interstitial fibrosis of uncertain aetiology. She was treated with daily oral prednisolone from 11 months to four years of age at a starting dose of 2 mg/kg/day. When she was five years old, her younger brother was born with similar but milder clinical features and did not require hospitalization during his childhood. However, he was also treated with daily oral prednisone from five to seven years of age.\nHer quality of life appeared near normal apart from some exercise limitation in her adolescent years. At age 25 years, she was seen by an adult respiratory physician having had a diagnosis of a paediatric ILD. Physical examination revealed finger clubbing and bilateral lower zone fine inspiratory crackles on auscultation. An arterial blood gas sample on room air demonstrated moderate hypoxaemia with partial pressure of oxygen (PaO2) 61 mmHg, partial pressure of carbon dioxide (PaCO2) 33 mmHg, and arterial oxygen saturation (SaO2) 95%.\nIn view of her unusual history and clinical features, a surfactant protein deficiency disorder was suspected. DNA from both siblings was extracted from whole blood and sent to the Department of Pediatrics Research Laboratory at Johns Hopkins University School of Medicine which revealed compound heterozygosity for a known ABCA3 mutation (c.3609_11delCTT; F1203del) and a second, previously unknown, missense mutation in ABCA3 exon 5 (c.127 C>T; p.R43C) [4]. Genetic testing of parents showed that her mother was heterozygous for the F1203del mutation and her father was heterozygous for the p.R43C mutation.\nHer serial pulmonary function tests demonstrated an obstructive pattern with forced expiratory volume in 1 sec (FEV1) gradually decreasing from 78% predicted at age 10.5 years to 52% predicted at age 39 years, but forced vital capacity (FVC) remained within the normal range at 93% predicted at age 39 years. Diffusing capacity was severely reduced below 25% predicted from age 10.5 years and did not change over time (Fig. ). Plethysmographic lung volumes did not reveal any lung restriction. A high-resolution chest computed tomography (HRCT) scan at age 28 years showed severe diffuse lung disease with extensive distribution of cystic change throughout the lungs (Fig. ). In the lower lobes there was extensive vascular attenuation, and at the bases were multiple well-defined cysts up to 20 mm size (Fig. ). Comparison to an HRCT chest three years prior showed no interval change. Transthoracic echocardiography at age 27 years showed an elevated pulmonary pressure at 55 mmHg with mild right ventricular and right atrial enlargement but normal contractility.\nHer brother had a similar obstructive pattern on serial lung function testing but had developed more airflow obstruction over time in conjunction with a higher residual volume and diffusing capacity (Fig. ). His HRCT chest at age 30.5 years also demonstrated extensive thin walled cysts measuring from a few millimetres to 60 mm. There were areas of reticulation and parenchymal distortion consistent with fibrosis with air trapping on expiratory series (Fig. ).\nWith genetic counselling and in view of the autosomal recessive nature of ABCA3 deficiency, the patient sought pregnancy at age 27 years, despite her cardiorespiratory disease. She was managed in a high-risk obstetrics unit from 30 weeks gestation, and given inhaled iloprost two weeks pre-partum and immediately post-partum. Elective lower segment caesarean section delivered a healthy male baby without any lung disease.\nShe was managed conservatively with supplemental oxygen after she developed resting hypoxaemia from age 27 years. No specific drug therapy for ABCA3 deficiency was trialled and pulmonary hypertension medications were unable to be continued due to patient-perceived side effects. She developed right heart failure at age 39 years and was subsequently referred for consideration of a lung transplant. Her brother was also diagnosed with pulmonary hypertension and had been managed in a pulmonary hypertension clinic since age 28 years. He has remained on room air and is maintained on tadalafil and macitentan as well as fluticasone, umeclidinium, and vilanterol inhaler. |
Patient presentation
A 21-year-old female college student with a history of asthma presented to the neurosurgery office for consultation complaining of mass on the left side of her skull associated with increasing size over the past two days and intermittent headaches for the past two to three weeks. The left-sided headache included her upper jaw. She also reported a history of cellulitis and urinary tract infections, in addition to surgical removal of an impacted wisdom tooth in 2016. Family history was positive for diabetes mellitus (DM) type II in both her father and her grandfather and colon cancer and coronary artery disease in her other grandfather. She admitted to drinking alcohol one to two times per week but denied use of tobacco and drugs. At the time, she was taking Viorele birth control to regulate her menses. Review of systems was otherwise negative.
Clinical findings
Physical examination revealed a well-developed, well-nourished female in no acute distress. She was awake, alert and oriented to person, place and time with a Glasgow Coma Score (GCS) of 15. A soft left frontal lesion associated with tenderness to palpation, without erythema or drainage, was palpated slightly off midline. Her cranial nerves II-XII were intact. Strength in both upper and lower extremities was five out of five bilaterally. No pronator drift was noted. Sensation to light touch was intact bilaterally in V1-3, upper extremity, and lower extremity distributions. Her reflexes were symmetric. Her gait was within the normal limits.
Imaging
CT of the head without contrast (Figure ) revealed an expansive soft tissue mass with beveled edges and dimensions measuring approximately 3.5 x 2.1 x 2.3 cm in the left frontal calvarium. Bony destructive changes of the inner and outer table of the left frontal calvarium were apparent. Extension of the mass into the dura was noted. The mass did not extend into the |
We obtained written informed consent from the patient’s parents. The case is compliant to the SCARE guidelines []. A 10-month-old girl was taken to our hospital by her parents due to recurrent maxillofacial infections accompanied by swelling or abscess of the left cheek and purulent discharge from the preauricular pit for 4 months. On physical examination, a swollen erythematous maxillofacial lesion was observed in the left cheek (Fig. ). A pit with white purulent secretions in the intertragic notch and another small cutaneous dimple in the left parotid gland region were observed (Fig. A). EAC contained no fistula track. All other head and neck examinations were unremarkable. Ultrasound performed for the maxillofacial area demonstrated a lesion with inflammatory changes. The other auxiliary examinations, including otoscopy, pure tone audiometry and renal ultrasound, were without abnormalities. There was no history of previous incision or drainage procedures. No particular family history was recorded. A 3D-computed tomography (CT) fistulogram and magnetic resonance imaging (MRI) were performed to delineate the course of the tract and the extent of the lesion. The results revealed two conjunctive tracts: one tract arose from the skin surface anteroinferior to the EAC, beneath the facial nerve, and passed into the deep lobe of the parotid gland and projected to the masseter; the other tract extended from the superficial lobe of the left parotid to the intertragic notch (Figs. and –).\nOriginally, the infection could be controlled by intravenous antibiotic administration, which resulted in maxillofacial infection improvement (Fig. B). However, recurrent inflammation had been persisting for one year. Finally, after obtaining parental consent, the patient underwent surgical management under general anaesthesia at 2 years old. The surgery was performed under facial nerve monitoring. First, a methylene blue staining agent was injected into the cutaneous pit of the intertragic notch. Then, a modified Blair incision encompassing the pit was made. The facial nerve was identified first, which allowed the cyst to be incised easily. During superficial parotidectomy, one cyst lying in the superficial lobe of the parotid and cartilage of the intertragic notch were removed. Blunt dissection was then used to follow the other larger fistula tract, which was located deep inside the facial nerve and projected medially to the posterior aspect of the masseter (Fig. A). Using a microscope, the tract was carefully separated from the facial nerve, which formed a blind pouch alongside the masseter (Fig. B). Finally, a suction drain was placed and kept until 48 h postoperatively (Fig. C). The patient recovered well, and no complications occurred in the following days. The final pathology of the excised tissue was consistent with that of type II FBCA (Fig. D). At follow-up over one year postoperatively, the surgical site was well healed, and the patient had no facial palsy or recurrence by coronal and axial MRI imaging (Fig. D–F). |
A 76-year-old man was referred to our hospital for having lower back pain for 5 months, which was suspected to be L2/3 spondylitis on magnetic resonance imaging (MRI). He had previously undergone cervical laminoplasty for ossification of the posterior longitudinal ligament and diffuse idiopathic skeletal hyperostosis 19 years earlier. He had a history of hypertension and diabetes. Although a history of BCG vaccination was unknown, he had no previous history of tuberculosis infection. He had also undergone transurethral resection of a bladder tumor (TUR-Bt) and had been treated with intravesical mitomycin C (MMC) for the bladder cancer 1.5 years earlier. Four months later, he again underwent TUR-Bt, received intravesical MMC, and started BCG therapy for the recurrence of bladder cancer. After the sixth course of intravesical BCG therapy, he was aware of systemic weakness and loss of appetite, and was unable to walk. He appeared to have septic shock and therefore was treated in the intensive care unit. In spite of systemic analyses, the source of his infection and the causative bacteria could not be identified. He was subsequently diagnosed with hypercytokinemia caused by BCG therapy. He showed clinical improvement without the administration of antituberculosis drugs, and was discharged 9 months before he came to our hospital.\nExcept for a temperature of 37.2 °C, his vital signs were within normal limits. Although there were no motor and sensory disturbances in the legs, he was unable to walk owing to lower back pain. Physical examination demonstrated vertebral tenderness at the L2/3 level. Laboratory analysis demonstrated a normal white blood cell (WBC) count of 6300 /μL, a high erythrocyte sedimentation rate (ESR) of 53 mm/h, and a high C-reactive protein (CRP) level of 2.7 mg/dL. There were no other abnormal laboratory findings regarding anemic changes, kidney function, or liver function. The patient did not receive tuberculin skin testing.\nThere were no notable findings on electrocardiogram or chest X-ray. X-ray of the lumbar spine displayed collapsed endplates of L2/3. Sagittal T1-weighted MRI displayed a decreased signal in the L2/3 disc and the vertebral bodies (Fig. ). Sagittal T2-weighted MRI displayed an increased signal in the L2/3 disc and fluid collection in the anterior part of the vertebral bodies (Fig. ). Axial T2-weighted MRI displayed an increased signal around the posterior area of the vertebral bodies, which extended into the left epidural space and reached the peripheral muscle tissue and the area near the aorta (Fig. ).\nOn the second day of hospitalization, the patient underwent an L2/3 disc biopsy, but the general bacteria culture was negative, and the smear was negative for Ziehl-Neelsen staining. The patient’s blood culture was negative, and the result of T-SPOT.TB® (T-SPOT), which is a type of interferon-gamma release assay (IGRA), were also negative; the number of spots for both 6-kDa early secretory antigenic target (ESAT-6) and 10-kDa culture filtrate antigen (CFP-10) was 5 or less. The causative bacteria remained unidentified and therefore he was started on empirical therapy with intravenous ceftriaxone. On the fifth day of hospitalization, a plain computed tomography scan, which was performed for systemic examination, showed soft tissue development in the adjacent abdominal aorta at the L3 level (Fig. ), which was suspected to be an infectious aortic aneurysm. A 2-stage operation was planned for the spondylitis with adjacent infectious aortic aneurysm, to prevent rupture of the infectious aortic aneurysm, obtain spine stabilization, drain the abscess and make a diagnosis. On the tenth day in hospital, because the risk of rupture was considered to be low, the patient underwent an L2/3 laminectomy followed by posterior fixation using percutaneous pedicle screws at T12, L1, L4, and L5 as the first stage. Specimen cultures of the lumbar vertebrae, yellow ligament, necrotic tissue, etc., were negative for general bacteria, specimen smears were also negative for Ziehl-Neelsen staining, and there were no pathological findings of caseating granuloma or necrosis. On the fiftieth day of hospitalization, because the infection had been controlled but the inflammatory response was sustained, he underwent replacement of the aneurysm with a synthetic graft by vascular surgeons, and lesion curettage and L2/3 anterior interbody fusion by iliac bone transplantation as the second stage. Spinal surgery was performed in the transabdominal approach owing to the risk of rupture. On pathological analyses, the L2/3 intervertebral disc, vertebral bone, and tissue surrounding the vertebral bone and aorta showed caseating granuloma and necrosis with multinucleated giant cells and epithelioid cells upon hematoxylin-eosin staining, and positive bacilli upon Ziehl-Neelsen staining. The tuberculosis-polymerase chain reaction (Tb-PCR) result of the tissue was also positive, using COBAS® TaqMan® MTB Test, which is a real-time PCR system targeting the 16S rRNA gene region of Mycobacterium tuberculosis complex DNA. Owing to the patient’s history of BCG therapy, negative T-SPOT, pathological findings, and positive Tb-PCR, the pathogenic bacteria of the spondylitis was considered to be BCG. We then started multidrug therapy with antituberculosis drugs, including isoniazid (INH), rifampin (RFP), and ethambutol (EB), because BCG is typically resistant to pyrazinamide (PZA). PCR-based genomic deletion analysis was performed using the specimens to distinguish BCG from the other M. tuberculosis complexes. Specifically, multiplex PCR was performed utilizing region of difference 1 (RD1), which is present in the DNA of other M. tuberculosis complexes but is deleted in the DNA of BCG []. Primers ET1, ET2 and ET3 bind and amplify a 190-bp region in BCG, whereas a 160-bp region is amplified in the other M. tuberculosis complexes, as observed by electrophoresis on an agarose gel. A clinical isolate sample from our patient was identified as BCG with a deletion in RD1 (Fig. ). Specimen cultures from the first-stage and second-stage operation were later identified as members of the M. tuberculosis complex using a mycobacteria growth indicator tube. After therapeutic intervention, the patient’s WBC count, ESR level, and CRP level were improved and MRI displayed no signs of active infection in the spine, epidural space, peripheral muscle tissue, or aorta. On the ninetieth day after hospitalization, the patient was discharged from our hospital and transferred to a different hospital for physical rehabilitation. |
A 76-year-old Caucasian man presented with a long history of a left temporal lesion that had progressively enlarged only recently. Initial surgical excision yielded a mass measuring 2.5 × 2.2 × 1 cm. Histological examination revealed tumor islands infiltrating the dermis and connecting to the epidermis with a lobulated morphology (Figure ). Since the deep resection margin was positive for malignant cells, a re-excision was performed and negative margins were verified microscopically. Eight months later, our patient presented with a 2 cm firm mass overlying the left parotid gland with minimal mobility on physical examination (Figure ). Whole body imaging in the form of a positron emission tomography (PET) scan showed increased uptake in the left parotid gland, which was consistent with metastasis. Our patient underwent left parotidectomy with facial nerve preservation and cervical lymphadenectomy. Surgical pathology specimens revealed a moderately differentiated carcinoma with growth pattern and morphological features consistent with porocarcinoma. Microscopically, nests of polygonal malignant cells were seen infiltrating the papillary and reticular dermis (Figure ). A surrounding dense fibrous stroma could be visualized (Figure ). The cervical lymph nodes (14 in total) were negative.\nGiven the aggressive behavior of EPC described in the literature and evidence for the effective use of radiation therapy [], our patient was offered intensity-modulated radiation therapy (IMRT). After initial consultation at the Head and Neck Radiation Oncology Clinic of the Medical University of South Carolina (MUSC) Hollings Cancer Center, a consent form was obtained. Treatment planning consisted of a contrasted head and computed tomography (CT) scan of the neck with our patient lying supine, using a head and neck thermoplast mask with bite block immobilization. Thin (3 mm) axial images were imported into the ADAC Pinnacle planning system (ADAC Laboratories, Milpitas, CA, USA). In this case, IMRT was designed using an inverse-planning algorithm.\nThe six-beam heterogeneous plan entailed delivering a total of 60 Gy in 30 treatment fractions over six weeks. A CT scan with isodose distributions and a dose-volume histogram for the final treatment plan are shown in Figure . Particular attention was directed to regions of interest such as the left inner ear and optic nerve, to minimize radiation exposure well below the reported tolerance doses of 40 Gy and 50 Gy, respectively [].\nWeekly on-site treatment visits were documented during the treatment phase of our patient, followed by scheduled three-month follow-up visits. Our patient tolerated the radiation treatment well, and developed only mild xerostomia and mild paresthesias. Further follow-up revealed that our patient remains disease-free 10 months after completion of the treatment course. |
Patient is an 11-year-old female sixth-grade student. She was referred to the psychiatric clinic by her pediatrician for mood swings, irritability, and stubbornness. Parents were concerned about her school refusal. Mother reported that she refuses to follow instructions, and these symptoms are getting worse. She was afraid of visiting hospitals, and during her initial visit she had difficulty in engaging and had minimal interaction with the clinicians. During her assessment, the mother reported that she was born preterm at eight months of gestation with immediate birth cry. The patient had no developmental delays, and the speech was acquired at 1.3 years of age. The patient had deep interest in mathematics, science, and technology but did not like going to school. She did not like to interact with kids of her age. She struggled to make friends, engaging in reciprocal play, and going to a new place. She refused to go on the school bus with other children. She had a deep knowledge about dogs and their breeds. She liked playing with building blocks and watched the same TV series multiple times. She was previously diagnosed with obsessive compulsive disorder (OCD) and anxiety disorder and received treatment from two different psychiatrists. There was no relevant medical history. During assessment she was shy and attempted to make eye contact but could not sustain it. She had sensory issues like sensitivity toward rough textured clothing, strong smell, and aversion toward loud noises. She did not wear undergarments till three years of age and avoided haircuts. IQ assessment was conducted with some difficulty since it was challenging to develop a rapport. Wechsler Intelligence Scale for Children - Fourth Edition (WISC-IV) was conducted for IQ testing, which revealed a scattered profile with full-scale IQ in average intellectual functioning range with low processing speed. She scored 38 on childhood autism rating scale high functioning (CARS2-HF), signifying severe symptoms of autism spectrum disorder. The patient was initiated on aripiprazole 2 mg once daily for irritability and started behavioral therapy three times a week. The parents were provided psychoeducation about her diagnosis and treatment strategies. At four weeks follow-up visit, the family reported reduction in irritability and improvement in overall behaviors and academic performance. |
An 80-year-old man with a background of hypertension, gout, osteoarthritis and cerebrovascular disease presented following an injury to his left big toe. He developed confusion and sepsis secondary to deep infection of his left big toe. As part of the initial septic screen, he had a CT scan which did not show any evidence of hydrocephalus (Fig. a). He was started on intravenous antibiotics and the decision was made to undertake amputation of the infected toe. After extubation, he had persistently reduced level of consciousness. A CT head scan performed post-operatively showed new onset acute hydrocephalus with no evidence of haemorrhage or infarction (Fig. b).\nHe was urgently transferred to a tertiary neurosurgical centre with a GCS of 11 (E4, V2, M5) and underwent an emergency external ventricular drainage (EVD) insertion under local anaesthetic. Intra-operatively, his CSF was under moderate pressure and it was clear and colourless. His CSF results showed a white cell count of 28 × 106/L, red blood count of 3 × 106/L with no organisms. Post-operatively, he recovered to a GCS of 14 (E4, V4, M6). However, he still had persistent hydrocephalus on the repeat CT head scan. Apart from raised inflammatory markers and mild hyponatremia, his blood tests were unremarkable.\nDue to crowding of his foramen magnum, a decision was made to have a MRI head and spine. This showed erosion of the odontoid with marked degenerative changes at the atlantodental joint. Interestingly, a large tissue mass presumed to be a pannus was also seen causing severe compression at the cervico-medullary junction and obstruction to CSF flow at this level (Fig. ). Prior to this presentation, the patient had never suffered from any neck pain or limb weakness.\nOn further examination, he was not myelopathic but had widespread tophi in his hands and feet. He was started on treatment for gout with Colchicine and Allopurinol. His uric acid levels were normal at 289 umol/L. Radiographs of the hands and feet disclosed mixed osteoarthritis and gouty athropathy. He was reviewed by the Rheumatology team who felt that the odontoid pannus was most likely secondary to gout or pseudo-gout and not rheumatoid arthritis. Rheumatoid factor and anti-CCP IgG antibodies were both negative.\nDue to persistent hydrocephalus on the repeat scan, he subsequently underwent ventriculo-peritoneal shunt insertion and made good recovery both cognitively and physically. The patient initially declined definitive surgical intervention and was discharged to his local hospital for ongoing rehabilitation. Following discharge to his local hospital, he developed new onset weakness in his left arm and a repeat MRI spine showed significant canal stenosis at C1 level secondary to the pannus causing cord compression.\nHe was transferred back to our neurosurgical unit and underwent posterior C1 and C2 decompression and C0–C3 posterior fixation. Post-operatively, he regained full power in his left upper limb and improved his mobility with physiotherapy input. He was reviewed 12 months after his initial presentation and was able to mobilize independently. |
A 32-year-old man undergoing treatment for a depression with serotonin–norepinephrine reuptake inhibitors (SNRIs) and with one prior admission to the hospital due to rightsided abdominal pain was admitted to our department after 4 h with severe abdominal pain. The pain was acute in onset, stabbing and located in the whole right side of the abdomen, radiating to the back and to the right groin. The pain was associated with nausea and restlessness, and could not be reduced with either NSAID or high doses of morphine. On presentation, he was ill looking, sweating, restless and in agony. He was ABC stable, his abdomen distended with generalized tenderness and in his upper right quadrant he had a 10 × 10-cm hard very sore mass, with positive rebound tenderness. There were normal bowel sounds.\nParaclinical tests showed leucocytosis (10,1 × 109/l) and normal serum lactate. A computed tomography (CT) scan showing gastric retention, encapsulated dilated small intestines in the right upper quadrant with pneumatosis intestinalis and collapsed small intestines distally from this area interpreted as intestinal obstruction (Figs and ).\nAn emergency laparotomy was performed 12 h after the first appearance of the symptoms and 7 h after admission to the hospital. This revealed severe small bowel obstruction. Approximately 1 m of small bowel was found distended, herniated and strangulated through a 2-cm defect in the mesentery of the transverse colon. Furthermore, it was capsuled in the mesenteric peritoneum, resembling a balloon ready to burst. The intestines were greyish in colour, but vital without signs of necrosis or ischaemia (Fig. ). The peritoneal capsule was then removed and the defect widened, before the herniated small bowel could be loosened and retracted back through the hernia. The defect was sutured. The postoperative course was smooth, and the patient was discharged 4 days later. The patient has not been readmitted to the hospital at 18 months follow-up, but did complain of mild pain in the upper abdomen the first few weeks after discharge. |
This is about a 76-year-old heavy smoking male patient who presented with a history of 4-day duration of fever, cough, and purulent sputum. He was admitted to hospital because of presence of an irregular rather compact elongated shadow in the right upper zone of his chest X-ray. The patient had been a farmer for all previous years. His past medical history included an operation for inguinal hernia 15 years ago. Three months ago, he underwent colonoscopy for abdominal discomfort and investigation of chronic microcytic anemia which proved to be due to diverticuli in descending colon and sigmoid. An abdominal Computed Tomography (CT-scan) further revealed small cortical cysts on his left kidney as well as a tiny calculus in the middle calyx of the same kidney. All full blood and biochemical tests were within normal limits except for mild hypochromic anemia (Htc = 38%), a moderate neutrophilic leucocytosis, and elevation of C-reactive protein (CRP = 18.9 mg/L). A chest CT-scan showed an elongated irregular shadow with traces of air-bronchogram stripes in it () for which the patient underwent fiberoptic bronchoscopy (FOB) to exclude malignancy. Upper airways and trachea were normal () but right main bronchus was roughened by multiple unequal sized nodules with no signs of infiltrated mucosa along the bronchus' entire length (). No other abnormal findings were detected in the bronchial tree down to the level of subsegmental branches. Washings and brushings were obtained from the apical segment of right upper lobe (RUL) where the compact lesion was located. Several biopsies were also taken from the right main bronchus mucosal nodules. The patient was administered (iv) Cefuroxime Sodium, 750 mg tid for 5 days, and continued with per os Cefuroxime axetil 500 mg bid for another 5 days with almost radiological clearance of the RUL shadow. Biopsy from nodules of right main bronchus was sent for histopathologic diagnosis. Microscopy revealed that the nodules consisted of cartilage situated between the normal cartilage and the surface epithelium of the bronchus. Step sections showed that there was indeed continuity through narrow pedicles, in concordance with the view that the condition represented multiple ecchondroses of the bronchial cartilages (). These findings posed the diagnosis of TO while RUL lesion proved to be pneumonia. |
A 28-year-old male came to our Emergency Department with vision loss in his right eye following an accident that occurred an hour earlier at his workplace. The patient is a farmer who plans to hunt eels in rice fields. He was using a 4.5mm air rifle with projectiles consisting of metal arrows made from motorcycle wheel spokes. When the gun jammed, he checked it through the gun barrel, and the accident happened when the rifle was accidentally triggered. A metal arrow entered his head and penetrated his right infraorbital region.\nOn arrival, his vital signs were normal limit and his mental status was alert. Primary and secondary surveys did not reveal additional injuries, and no significant medical history or drugs, tobacco, or alcohol abuse was recorded. The patient reported having a headache but no nausea, vomiting, or convulsions. In addition, he exhibited normal body temperature, a Glasgow Coma Scale score of 15 points, clear consciousness and speech, and cooperation during the physical examination. The muscular strength and tension of the limbs were normal. Bilateral Babinski signs were not induced. He was given a preoperative test immediately after admission, as well as an intravenous broad-spectrum antibiotic, anti-tetanus injection, and anti-convulsant medications. Blood analyses and other biochemical parameters were within normal limits.\nLocal examination revealed a perforating injury at the entry location of the foreign body and penetration below the right infraorbital margin measuring 0.5 × 0.5 cm in size with bleeding (A). His right pupil was dilated and not reactive to light, and eye movement was limited. Only minimal ecchymosis and hyphemia were seen in the anterior chamber of the right eye. There was a penetrating wound on the right inferior medial eyelid or Zone 3C of the Turbin pattern measuring 1 × 0.5 cm (B). Using a slit lamp, we found a vitreous hemorrhage (C), and ocular ultrasound revealed a discontinuity of the inferior wall of the eyeball (A).\nA plain skull radiograph showed that a metallic foreign body that looked like an arrow had penetrated above the right orbital roof (B). The tip of the foreign object was in the right parietal lobe as revealed by non-contrast computerized tomography (CT) (C) and a three-dimensional CT (3D CT) of his brain (A). No lesions of the major cerebral vessels were noted on non-contrast CT angiography (CTA) (B).\nAs there was no evidence of vascular injury and the neurosurgeon recommended anterior orbital rather than transcranial surgery, it was considered reasonable and safe to remove the foreign body anteriorly. Subsequently, with preparing and planning surgery having established a multidisciplinary approached, the patient was transferred to the operating room 2 h after arriving in the Emergency Department. We (the neurosurgeon and ophthalmologist) performed retrograde removal through the penetration wound with C-arm radiography fluoroscopic guidance (C). We used fluoroscopic guidance during the surgery to determine the precise location of the base of the arrow through the penetration or entrance wound and to perform retrograde removal gently. There was a minimal amount of brain tissue accompanying the arrow from the entry wound without CSF leakage. The length of the homemade metal arrow was 10 cm (D). Following this procedure, we irrigated the wound with a standard 0.9% saline solution containing antibiotics to eliminate debris and control bleeding in the entrance wound and wound track as far as could be attained.\nPost-surgery, the patient was admitted to the critical care unit, where broad-spectrum antibiotics and anticonvulsant drugs were administered for seven days. His intracranial pressure (ICP) was monitored by the neurosurgeon in the postoperative period for two days and was within normal limits. The patient fully recovered and was discharged on postoperative day 10. The long-term, post-operative evaluation six months later, as well as the surgical wound and the patient's general condition, were satisfactory. The CT brain scan with and without contrast showed a slight ischemia on the right-frontal lobe and a globe rupture on the right eye (A). The CTA detected no abnormal vascular brain structure (B). Meanwhile, the patient's vision had not changed, with enophthalmus in the right eye (C). |
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 46-year-old non-smoking Caucasian G4P2022 presented for evaluation of chronic pelvic pain first noted following HS in July 2011. The Essure procedure was completed under ketorolac intravenous sedation in an ambulatory surgery center setting. Three months later, the patient was informed that bilateral tubal occlusion was confirmed by hysterosalpingogram, but a detailed review of findings was not provided. Soon after HS the patient reported increased, irregular vaginal bleeding and dyspareunia. Within 4 to 6 weeks of her Essure procedure the patient developed intermittent right lower quadrant pain, headaches, insomnia, lower extremity paresthesia, intolerance to cold, and fatigue.\nAlthough the patient was in excellent general health before HS and only visited the doctor for annual check-ups, following the Essure procedure she had approximately 36 clinic appointments (partial year). No specific diagnosis or treatment was offered. Her symptoms persisted during subsequent years and insurance records showed a sharp uptick in frequency of health visits following the Essure procedure: 34 billed encounters in 2012 to discuss pelvic pain issues with various healthcare providers, 22 consultations in 2013, 28 visits in 2014, and 54 appointments in 2015 (partial year). In addition to consultations, these visits included multiple in-office ultrasounds, three computed tomography scans, and blood tests with no abnormal findings identified from these evaluations. The patient obtained a flat panel abdominal X-ray (anterior-posterior view) in May 2015 and this study showed the left Essure implant located within the corresponding fallopian tube, although the right device was in two asymmetric parts.\nIn September 2015, the patient had a follow-up hysterosalpingogram which showed a normal endometrial cavity and bilateral tubal occlusion. Consistent with the confirmatory hysterosalpingogram performed after HS, the right Essure device appeared disrupted with a small portion possibly corresponding to the medial terminal marker near the right cornu, but the majority of the implant was situated at the lateral pelvis.\nIn November 2015, the patient underwent diagnostic hysteroscopy and 5-mm triple-port laparoscopy with partial bilateral salpingectomy for removal of Essure implants. The intrauterine compartment appeared grossly normal and both tubal ostia were visualized. At laparoscopy, both ovaries were normal appearing and the outer coil of the right Essure device could be seen protruding from tubal serosa lateral to the right uterotubal junction (). This was removed without difficulty using bipolar cautery and circumferential dissection, as previously described []. However, a component of the right Essure device was fluoroscopically identified laterally and cephalad to the uterine fundus, although the fragment could not be seen during laparoscopy. The left implant was entirely contained within tubal tissue and was removed using the same technique, with the left implant closely inspected during laparoscopy to verify presence of all terminal markers. Here, it was noted that the inner rod's terminal (medial) marker was absent (). Intraoperative fluoroscopy was performed and this permitted retrieval of the device remnant (). Postoperative computed tomography was performed which showed the left cornual region was free of any foreign body, but the right Essure fragment was lodged near intestinal serosa at the proximal colon. Because the procedure ended in the late afternoon, the patient stayed overnight and was discharged home from the outpatient surgery floor the following morning. Her postoperative course has been uncomplicated and she continues to do well. |
A 45-year-old male was admitted to our clinic due to new onset right leg pain. The patient had undergone an L5-S1 lumbar total disk replacement two years prior with an SB Charitė disk (Depuy Spine, Raynham, MA, USA) as a result of discogenic back pain. Following surgery, he reported a significant relief of his initial symptoms but developed new, atraumatic onset right leg pain over the six months prior to visiting our clinic. The radicular pain had failed to significantly improve with a course of nonoperative treatments consisting of physical therapy, nonsteroidal anti-inflammatory medications, and epidural steroid injections. He denied paresthesias or the loss of motor function, and he also denied a prior history of HO formation, trauma, or inflammatory arthritis.\nA physical examination revealed a well-healed abdominal surgical scar, and the lumbar range of motion was limited in forward flexion secondary to right leg pain, with near full motion in all other planes. A straight leg raise reproduced the patient's symptoms, and a neurologic examination revealed 5/5 motor strength in all major muscle groups with intact sensation to light touch in all lower extremity dermatomes. The patellar and achilles reflexes were 2+ and symmetric. On presentation, radiographs demonstrated implant encroachment into the spinal canal with heterotopic bone formation outside the margins of the disc (). A computed tomography myelogram () revealed compression of the traversing nerve root secondary to the inferior endplate of the implant that resided posterior to the margin of the vertebral endplate as well as an associated posterior bone growth further into the canal.\nSince the symptoms failed to improve with non-operative management, the patient elected to undergo a revision surgery to extract the implant and to perform an L5/S1 anterior lumbar interbody fusion. Ureteral stents were placed to facilitate mobilization and identification of the ureters intraoperatively. The implant was then removed, and a polyetheretherketone interbody spacer filled with an autologous iliac crest bone graft was placed anteriorly. This was followed by a posterior hemilaminotomy and decompression of the traversing S1 nerve root with removal of heterotopic bone originating from the superior endplate of S1. The heterotopic bone of this location and small islands of HO were decompressed with kerrison rongeur and curettes. The pedicle screw instrumentation was placed to supplement the interbody fusion. The patient tolerated the procedure well, with a blood loss of 400 mL, and was discharged to return home on postoperative day 3. During his 6-week postoperative visit, the patient reported complete relief of his radicular leg pain, and during his most recent 2-year follow-up, the patient continued to note improvements in both leg pain and quality of life. The radiographs at that time demonstrated a solid fusion without subsidence or recurrence of heterotopic bone formation (). |
An 80-year-old Chinese woman presented with progressive weakness in the proximal limbs and exercise intolerance for 3 months. She had difficulties in climbing stairs and getting up from a chair and needed to rest after walking about 100 m. Her symptoms continued to worsen. Two months before admission, it was difficult for the patient to raise her arms to collect objects or comb her hair. She could only walk about 10 m unassisted. She also complained of numbness in her toes and fingers. She had no ptosis and no difficulty chewing or swallowing. She exhibited no skin rashes, muscle pain, or weakness fluctuations. The patient had lost about 10 kg of weight in the past year. She had a 10-year history of hypertension. She used sertraline to treat anxiety for about 3 years. She had no history of myopathy or taking statins. Her family history was unremarkable. Neurological evaluation showed a waddling gait and muscle weakness in the patient’s neck flexors and bilateral proximal limbs (4/5 by Medical Research Council scale). No sensory abnormality was found. Serum creatine kinase was slightly elevated at 361 U/L (reference range 25–170 U/L), and lactate dehydrogenase was also elevated at 500 U/L (100-240 U/L). Myositis-specific antibody and associated antibody tests showed that the anti-signal recognition particle antibody and anti-Ku antibody were weakly positive (Supplementary Figure S). A nerve conduction study showed that the conduction velocity of bilateral median and superficial peroneal nerves was decreased. The electromyography and repetitive nerve stimulation were normal. Computed tomography of the chest and abdomen showed no signs of tumors. Magnetic resonance imaging of thigh muscles revealed mild fatty infiltration and edema changes (Supplementary Figure S).\nThe initial diagnosis was myositis, and a muscle biopsy was performed to confirm the diagnosis and rule out other myopathies. However, a biopsy of the vastus lateralis muscle showed many cytoplasmic vacuoles under hematoxylin and eosin staining that were confirmed as lipid droplets when stained with oil red O. These mainly appeared in type 1 muscle fibers when stained with ATPase pH 4.4, indicating lipid storage myopathy without any features of myositis (Fig. ). The plasma acylcarnitine profile under the fed state showed increased medium-chain and long-chain acylcarnitine species (decanoyl (C10) carnitines, 0.54umol/L (normal range: 0–0.50umol/L); tetradecadienoyl (C14) carnitine, 0.09umol/L (0–0.04umol/L) and hexadecenoyl (C16) carnitine, 0.48umol/L (0.02–0.42umol/L)), consistent with the diagnosis of MADD. The patient received oral vitamin B2 (riboflavin) at a dose of 20 mg three times a day. Her muscle weakness gradually improved. After a month of treatment, she regained her normal muscle strength. The patient refused a further genetic test to confirm the genotype. |
A 77-year-old female with hypertension, untreated hyperlipidemia, hypothyroidism, but without prior history of CAD or angina symptoms was referred to a cardiologist's office for a treadmill exercise test secondary to new onset palpitations. She denied any chest pain or pressure, shortness of breath, exertional dyspnea, or leg swelling. She quit smoking 36 years ago and has no family history of early cardiovascular diseases. She has a very distant cardiac work-up years ago, including a stress test and an echocardiogram, which the patient reported were unremarkable. Vital signs prior to the test were a blood pressure of 140/78, heart rate of 80, and a respiratory rate of 14. Physical exam was unremarkable except for a systolic ejection murmur that was graded II/VI at the base. EKG was at baseline with a normal sinus rhythm, normal axis, and occasional premature ventricular complexes (PVCs).\nThe patient underwent an exercise stress test using the Bruce protocol and was able to complete stage 1 with exercise for three minutes at a speed of 1.7 mph and a 10% incline. The test was terminated due to dyspnea and fatigue without chest pain. She reached a heart rate of 141 beats per minute which was 98% of predicted for her age. She accomplished 4.5 metabolic equivalents of exertion. With exercise, she had occasional atrial premature complexes and PVCs with a ventricular couplet in recovery. She started to notice tightness in her chest. Her peak blood pressure at the time was 218/90.\nThe patient was transferred onto a stretcher, and an IV line was started. She was given sublingual nitroglycerin, 325 mg of aspirin to chew, and one 5 mg IV push of metoprolol tartrate. She then received nitroglycerin paste and metoprolol tartrate IV every 5 min for two more doses. At that time, her EKG on the stretcher showed ST elevations in leads I, aVL, V5, and V6 with ST depressions in leads III, aVF, and V1-V3 consistent with a lateral wall evolving myocardial infarction (). She was transferred urgently to our institution for cardiac catheterization.\nThe patient underwent an emergent cardiac catheterization with left ventriculography and intravascular ultrasound (IVUS) within 2 hours after onset of symptoms. Troponin-I levels prior to the catheterization increased to 11.17 (normal less than 0.05 ng/ml). The rest of the laboratories were within normal limits including a thyroid-stimulating hormone (TSH) level. Coronary angiography showed nonobstructive coronary artery disease (pLAD 40%) and highly tortuous coronary arteries. IVUS of the proximal LAD revealed a minimal lumen area of 5.2mm2, and no ruptured plaques. Left ventriculogram revealed a left ventricular ejection fraction (LVEF) of 20% and severe mid-cavitary hypokinesis with basal and apical hyperkinesis (Figures and ). To our knowledge, this is the first case of treadmill exercise testing-triggered mid-left ventricular ballooning variant of takotsubo cardiomyopathy, whereby obstructive epicardial CAD and ruptured plaques were excluded with angiography and IVUS, respectively.\nThe patient was started on medical management with standard therapy for heart failure. A follow-up echocardiogram was done two days after the event which redemonstrated mid-left ventricular ballooning, with an improved LVEF of 35%. The patient remained asymptomatic during the course of her hospitalization and troponin levels trended down from a postcardiac catheterization peak of 16.06 ng/ml. An echocardiogram was repeated during an outpatient follow-up two weeks later which showed resolution of wall motion abnormalities and an LVEF of 45-50%. |
A 38-year-old male with no significant past medical history presented to the ER with complaints of exertional dyspnea associated with flu-like symptoms, including low-grade fevers, chills, occasional episodes of diarrhea, chest pain, and cough productive of yellow to gray sputum for two weeks. The patient initially went to an urgent care facility five to six days prior to admission where he was diagnosed with pneumonia (CXR not available). The patient completed a course of azithromycin and amoxicillin-clavulanate without improvement in symptoms and thus presented to the ER. He reported exposure to sick contacts (children with an upper respiratory infection) and a history of vaping until two to three weeks prior to admission, which he stopped due to the symptoms. History was unremarkable for any exposure to birds, occupational exposures, immunocompromised status, outdoor activities, or recent travel. On presentation, vital signs were remarkable for temperature 38.2°C, heart rate of 115, respiratory rate 33, and oxygen saturation of 76% requiring 3 liters of oxygen via nasal cannula. Lung examination revealed rhonchi on bilateral bases with associated egophony. Laboratory studies revealed a WBC count of 18.3 with left shift, thrombocytosis, and a lactic acid level of 2.2 mg/dL. CXR demonstrated bilateral lower lobe consolidation (Figure ).\nThe patient was admitted for severe sepsis secondary to bilateral pneumonia and was initiated on levofloxacin, which was later changed to vancomycin and ceftriaxone. Infectious disease was consulted due to the failure to respond to antibiotics and the possibility of fungal etiology. Over the course of the next few days, the patient did not show significant improvement. Imaging was repeated and included a CT scan of the chest, along with a CXR, for a more detailed evaluation of the lung parenchyma. This showed a rapidly worsening airspace opacities (Figures , ). Antibiotics were adjusted to include doxycycline, and ceftriaxone (dose was increased to 2 g every 24 hours). Work-up, including streptococcal pneumonia antigen, Legionella urinary antigen, Mycoplasma pneumonia, blood cultures, sputum cultures, and methicillin-resistant staphylococcus aureus (MRSA) nasal swab, was negative. A procalcitonin level was 0.18 ng/mL, as well as a C-reactive protein at 27.8 mg/dL.\nDue to a lack of improvement, persistent fevers, increasing oxygen requirements up to 8 liters via nasal cannula, and worsening leukocytosis, pulmonary service was consulted. The patient underwent a bronchoscopy, which revealed pitting of the bronchial mucosa due to bronchitis. Bronchoalveolar lavage was sent for culture, cytology, differential, bacterial, and fungal studies which came back negative. The patient was started on methylprednisolone, 0.5 mg/kg twice daily, with remarkable improvement (Figure ). Antibiotics were discontinued, the patient was weaned off oxygen, and discharged home with a steroid taper over four weeks. A follow-up CXR two weeks following discharge revealed marked improvement in airspace opacities to almost complete resolution (Figure ). |
A 59-year-old European man crashed his car into a concrete dam (Fig. ). Bystanders attending to the accident found him in cardiac arrest and started cardiopulmonary resuscitation (CPR) immediately. Sufficient CPR efforts were continued until the emergency services had arrived. The first recorded heart rhythm was ventricular fibrillation (VF). On inspection, no signs of injury were immediately visible and no skid marks were found. CPR was continued by physician-staffed emergency medical services (EMS) according to the current advanced life support (ALS) guidelines []. Return of spontaneous circulation (ROSC) was achieved after 30 minutes. He remained unconscious without any sign of muscular activity. He was intubated, mechanically ventilated, and treated with catecholamines during and post CPR.\nAlthough the car was severely damaged, the prehospital physician deemed a traumatic cause for out-of-hospital cardiac arrest (OHCA) unlikely. Based on findings indicative of myocardial ischemia in a post-ROSC electrocardiogram (ECG), acute coronary syndrome was suspected as the etiology of cardiac arrest. After telephone consultation with the trauma leader of the regional trauma center, the patient was transported to the trauma center with percutaneous coronary intervention (PCI)-capability primarily within 120 minutes of the accident.\nOn arrival at the trauma center, the patient appeared clinically stable. His heart rate was 65 per minute, systolic blood pressure was 150 mmHg, oxygen saturation measured by pulse oximetry was 94%, and body temperature was 34.2 °C. Signs of myocardial ischemia were found in the ECG (Fig. ). His pupils were found to be equal, round, and reactive to light.\nAfter primary evaluation in the emergency room a whole-body CT scan revealed findings listed in Table . An MRI scan (Fig. ) of his head and neck was obtained immediately due to the severity of the CT findings. Additional findings in the MRI scan are summarized in Table .\nThe medical and social history of our patient were provided by his family. Subjective overall health assessment found the married man, who was a father and grandfather, to be in good health. He had suffered a fall leading to a fractured scapula 8 years before this accident, which was treated non-operatively. Two years ago, he was assessed for suspected coronary heart disease by a specialist in cardiology, who could not substantiate this suspicion.\nHe was transferred to the intensive care unit (ICU) for further treatment. Halo fixation was installed because only ligamentous structures were disrupted in this case. This procedure is common and adequate in AOD when no cervical spine fractures are present [].\nDue to several episodes of severe bradycardia, transient transvenous pacing was conducted. Cardiac diagnostics showed an ischemic cardiomyopathy with recurrent episodes of ventricular tachycardia. Assessment via echocardiography was performed in the trauma room, 3 weeks and 2 months after the accident and revealed akinesia of the left anterior descending coronary artery (LAD) region and hypokinesia of the inferior wall after a suspected myocardial infarction and VF. Early coronary angiography could not be performed due to severe brain injuries.\nAlthough he was initially assessed to have a poor neurological prognosis from the perspective of the neurologists and neurosurgeons because of his severe brain injuries, he could be discharged from the ICU after 23 days; he was responding to verbal contact and was able to move all his extremities.\nAfter 23 days of treatment at the trauma center he was transferred to a hospital close to his home. Further in-patient treatment was continued by local protocol for further 33 days (timeline in Table ).\nHe was discharged to a neurological rehabilitation facility, where care and rehabilitation efforts were continued with great success. Three months after the incident the tracheostomy was surgically closed.\nCoronary angiography was performed 4 months after the primary event and revealed no coronary artery disease. Subsequently, he had to wear a life vest due to arrhythmia. He was defibrillated once by the LifeVest® 3 months after the trauma during his stay at the neurological rehabilitation facility. Finally, 6 months after wearing the life vest an implantable cardioverter-defibrillator (ICD) was installed.\nSix months after the trauma, he was fully conscious, spontaneously breathing, independent of help in everyday life, and mobile with walking crutches. However, he was unable to swallow granular feed due incomplete bilateral paresis of the hypoglossal nerve. His neurologic status is continuously improving; treating neurologists attested a high potential of restitution. |
A 77-year-old right-handed man was admitted to our department with recurrent episodes of transient amnesia. The patient had no other notable past medical or psychiatric disease. Two days before admission, the patient experienced an episode of transient amnesia that began abruptly during breakfast. He could not remember his job and kept asking why he had to go to work despite his wife’s repeated explanations. The symptoms lasted for about 10 minutes and then disappeared. Similar symptoms were observed again on the next day. The patient did not remember his nephew’s wedding, which had taken place 4 days prior. He continued to ask when the nephew was married and why he was married so early. The patient’s memories gradually returned over a period of 20 minutes. After these episodes, however, the patient remembered what happened during those events. During the above episodes, the patient’s family did not observe any other cognitive dysfunction other than amnesia. The patient was alert, fluent, and not disoriented to place or person.\nRoutine physical and neurological examination revealed no abnormalities. The patient had a score of 29 on the Korean version of the mini-mental examination (K-MMSE) (2 out of 3 on memory recall test, could recall the other when cued). Magnetic resonance imaging (MRI) and MR angiography revealed no abnormalities in the hippocampi or other structures related to memory. On electroencephalographic (EEG), epileptiform discharges were observed in the right temporal leads, including F8 and T2 (). Brain single-photon emission computed tomography revealed no significant increase or decrease of perfusion in the temporal lobes. Routine blood tests, including blood cell counts, routine chemistry tests, and tests to determine the levels of ammonia and creatine phosphokinase revealed no significant abnormalities. The patient was treated with 15 mg/kg of carbamazepine controlled-release (CR), followed by maintenance dose of 200 mg twice per day. He has been free of memory disturbances for 3 months. |
A 67-year-old woman presented to our tertiary care center for treatment of a small basilar artery aneurysm. The patient was started on aspirin and clopidogrel for stent assisted coiling of the aneurysm. A 5F 10-cm sheath was inserted into the right common femoral artery. During the exchange from the 5F to a 6F 80-cm guide sheath, access to the right common femoral artery was lost and the 6F guide sheath was dislodged superiorly and medially within the soft tissue. Manual pressure was held over the right common femoral artery access site while new access through the left common femoral artery was obtained. An angiogram of the right common femoral artery demonstrated a large hematoma with contrast extravasation (). Despite 40 minutes of manual pressure held over the right common femoral artery, the hemorrhage persisted. The dome of the hematoma was accessed through the original access site under ultrasound and fluoroscopic roadmap guidance using a 21G micropuncture needle and a microwire. A 6F, 10-cm sheath was inserted through the hematoma into the common femoral artery (, A). Once the sheath location was confirmed within the right common femoral artery, a Mynx-Grip closure device was inserted into the sheath (, B). The balloon was inflated inside the artery and pulled back against the origin of the puncture and contrast extravasation site. A right femoral artery angiogram was performed demonstrating exclusion of the hemorrhage by the balloon of the closure device. The sheath was pulled out and the collagen plug was deployed and pressed against the outside the vessel to obliterate the puncture site. A final angiogram demonstrated complete obliteration of the puncture site with no evidence of extravasation or vessel dissection (, C). On follow-up 3 months after the index procedure, the patient does not demonstrate any signs of infection. Duplex ultrasound imaging demonstrates no pseudoaneurysm, dissection, or arteriovenous fistula formation. |
A 66-year-old Japanese man who had no past medical or medication history complained of gross hematuria and visited a nearby hospital in October 2013. He had no habit of drinking alcohol or smoking tobacco. He was diagnosed as having a right renal tumor and underwent right nephrectomy laparoscopically. The pathological diagnosis was right renal cell carcinoma (RCC), clear cell carcinoma, pT1bN0M0, v1 (Fig. ). One and half years later, lymph node swelling was detected at hepatic portal region and he underwent lymphadenectomy. The pathological diagnosis was a metastasis from RCC. Two years after diagnosis, he was suspected of lung metastases and started treatment with interferon α. Three years later, the multiple lung metastases grew larger and were determined as progression despite interferon α therapy. At this point, he was referred to our hospital in October 2016. There were no abnormalities on physical examination and his vital signs were normal. He started treatment with sunitinib 50 mg/day on a schedule of 4 weeks on treatment and 2 weeks off; however, adverse events including grade 3 thrombocytopenia (platelet count, 49,000/μL), gum swelling, and hoarseness became intolerable 2 weeks after starting sunitinib. Four weeks after cessation of sunitinib 50 mg/day, he was started on a dose of sunitinib 25 mg/day on a schedule of 2 weeks on and 1 week off. Computed tomography (CT) findings in January 2017 revealed that his lung metastases had shrunk; however, he continued to experience the same adverse events. Therefore, the dose of sunitinib was further reduced to 12.5 mg/day on a schedule of 2 weeks on and 1 week off. CT findings in May 2017 revealed new metastases in the pleura, diaphragm, and the right paracolic gutter (Fig. a, b). As a result, the treatment was changed from sunitinib to axitinib and he started treatment with axitinib at 10 mg/day; however, adverse events including gum swelling, dysphonia, hypertension, diarrhea, and thrombocytopenia became intolerable (Fig. ). Two weeks after cessation of the drug, the dose of axitinib was gradually reduced from 6 mg/day to 4 mg/day. CT findings in September 2017 revealed the metastases had diminished in size and lung metastases were maintained at a diminished size (Fig. c, d); however, the adverse events could not be controlled and he discontinued axitinib treatment. His adverse events improved after discontinuation of axitinib; however, CT findings in December 2017 revealed the size of metastases had increased again (Fig. e, f). Consequently, he was started on fourth-line therapy with nivolumab (3 mg/kg every 2 weeks) and did not experience any adverse events. However, after he had received eight cycles of nivolumab, his metastatic lesions had grown, peritoneal dissemination appeared in his pelvic region, and pleural effusion appeared (Fig. g, h), so nivolumab was discontinued. After giving a detailed explanation of treatment options to our patient, he decided to rechallenge with axitinib 4 mg/day. However, adverse events including gum swelling and dysphonia became intolerable. After that, the dose of axitinib was reduced to 2 mg/day, and he experienced relief of adverse symptoms except for hoarseness. CT findings in August 2018 revealed metastases in lungs, pleura, diaphragm, and the right paracolic gutter had diminished in size (Fig. i, j). He has been continuously receiving a low dose of axitinib at 2 mg/day for 10 months with metastases maintained at reduced size. |
A 64-year-old male, two years status-post orthotopic heart transplant for ischemic cardiomyopathy, presented with an agminated cluster of deep red-to-brown firm, indurated erythematous nodules on his left hip and groin (). The patient’s immunosuppressive medications included mycophenolate sodium 1440 mg and cyclosporine 175 mg. He had no constitutional symptoms at the time. The patient was referred to dermatology for evaluation, and the lesions on his left buttock and hip were biopsied (). Pathology results showed a diffuse interstitial infiltrate of atypical lymphoid cells that were large and pleomorphic with mitotic figures. Immunohistochemistry staining of the atypical cells was positive for CD4 and CD30. Stains for S-100, pancytokeratin, myeloperoxidase, and CD56 were negative. The biopsy was consistent with a CD30+ lymphoproliferative disorder. A PET-CT was done and was negative for nodal disease or other foci beyond the primary lesions on the hip. Flow cytometry and T-cell rearrangement studies were performed on the patient’s blood. These results were negative for clonality and only notable for a depressed CD4 to CD8 ratio. Given these findings, immunosuppression was reduced with mycophenolate sodium decreased to 360 mg and prednisone 7.5 mg/day was added. The patient underwent radiation therapy with a total of 4600 Gy of radiation to the affected area. However, during the course of radiation, he developed new lesions in his left popliteal fossa and ankle. His immunosuppressive regimen could not be lowered further due to risk of graft rejection. A repeat biopsy was performed of the newly developing lesions. This also showed a CD30+ lymphoproliferative disorder with signet ring cell features (). The dermis contained a dense infiltrate of enlarged mononuclear cells organized in nodules, cords, and strands embedded in a sclerotic stroma. No lymphocytes were highlighted by CD20 and in situ hybridization for EBV was negative. However, the patient did have an EBV viremia with a titer of 6398 one month prior to the onset of the lesions. Due to the multifocal nature of these newly-developing scattered smaller lesions, the decision was made to treat with the addition of methotrexate 15 mg/week. The larger plaques resolved following local radiation treatment at a follow-up visit three months after initial radiation therapy. The smaller lesions on the left popliteal fossa and ankle remained stable with the addition of methotrexate. |
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 55-year-old female with a history of renal cell carcinoma of the left kidney metastatic to the bony pelvis, lungs, mediastinum, and spleen presented to the emergency department with shortness of breath, pleuritic chest pain, and left scapular pain. She presented to the same emergency department one week prior with pleuritic chest pain but was discharged home after pulmonary embolism was ruled out.\nShe was diagnosed with renal cell carcinoma of the left kidney five years prior after presenting with gross hematuria. At that time, she underwent left radical nephrectomy. One year later, she developed a metastatic lesion in the bony pelvis for which she underwent radiation therapy. She as treated with pazopanib for two years with stable disease but stopped due to gastro-intestinal toxicity. Therapy was switched to nivolumab, which was discontinued after six months due to grade four pancreatitis and grade two rash. Eight months prior to her current presentation, she underwent radiation treatment to metastatic lesions in the left pubic symphysis and spleen. The patient initiated therapy with cabozantinib, a tyrosine-kinase-inhibitor used to treat renal cell carcinoma, three months prior to her current presentation.\nOn physical examination, she was wheezing in all lung fields and hypoxemic requiring supplemental oxygen. She had prior 12-pack-year smoking history but no formal diagnosis of chronic obstructive pulmonary disease (COPD). A chest x-ray revealed a small left pleural effusion and left basilar atelectasis. Laboratory workup, including complete blood count, renal and hepatic panels, and troponin, was unremarkable. An electrocardiogram (ECG) revealed sinus tachycardia without signs of ischemia. CT was not repeated due to her negative CT angiogram one-week prior. Given radicular and left scapular pain, an MRI of the spine was done, which revealed no pathologic metastases in the thoracic or lumbar spine but did reveal a new sacral lesion. Given her progressive stridor, she underwent laryngoscopy, which revealed a normal upper airway. A bronchoscopy showed significant trachea-bronchomalacia and thick purulent secretions in the left upper lobe, lingula, and right upper lobe.\nTwo days after admission, repeat chest X-ray revealed near complete opacification of left lung and large pleural effusion, a remarkably different radiograph from admission (Figure ).\nSubsequent CT chest revealed a large left pleural effusion with partial loculation as well as partial atelectasis of the left upper lobe and complete atelectasis of the left lower lobe. A right perihilar metastasis and perisplenic metastases were reported. The study was negative for pulmonary thromboembolism.\nThoracentesis revealed cloudy straw colored exudative effusion. A four French pigtail catheter was placed. Approximately 400 milliliters of yellow-green fluid was immediately drained. Pleural fluid studies revealed a white blood cell count of 33,000/μL (97% neutrophils), pH of 6.44, LDH of 4760 U/L, and an amylase of 394 U/L. She was started on vancomycin, cefepime, and metronidazole for presumed empyema. Pleural fluid cultures showed heavy growth of lactobacillus species, heavy growth of anaerobic gram negative cocci, and moderate growth of Candida krusei. Antimicrobial therapy was subsequently narrowed to ertapenem and anidulafungin. Given lack of improvement and continued significant chest tube output over the following week, further CT imaging was obtained, revealing a gastro-pleural fistula (via the left diaphragm and superior posterolateral stomach) with associated complex pleural effusion containing contrast material and gas (Figure ). This process abutted the known splenic metastases.\nAn esophagogastroduodenoscopy (EGD) revealed a 1.5-cm fistula in the posterolateral stomach that opened to the pleural space (Figure ).\nEndoscopic suturing was attempted to close the fistula with limited success (partial closure noted on imaging, with methylene blue dye taken via mouth visualized in the chest tube drainage catheter on water seal; Figure ).\nFor complete closure, the authors attempted a novel approach utilizing a venting gastrostomy tube and chest tube to water seal to facilitate closure of the fistula over the ensuing six weeks. Enteral feeding via jejunostomy tube to aid closure of the fistula was employed. The patient was continued on ertapenem and anidulafungin. She was also initiated on a proton pump inhibitor. She was discharged to a rehabilitation facility with plans to repeat imaging and methylene blue swallow in six weeks.\nUnfortunately, CT scans after six weeks showed that the fistula remained patent. A second attempt was made at endoscopic closure, which was again unsuccessful. One month later, during a hospitalization for electrolyte abnormalities, the patient decided to pursue elective surgical repair of the fistula in hopes of regaining the ability to resume normal oral intake. Four months after her initial presentation, she underwent laparoscopic surgery for fistula repair. The surgeon visualized extensive radiation fibrosis involving the stomach, spleen and retro-peritoneum. Given these findings and to avoid splenic bleeding, they pursued a conservative surgery whereby they stapled the stomach to ligate the gastro-pleural fistula anatomically. This approach is novel and was successful in our patient. A fluoroscopic upper GI series with oral contrast three days after surgery demonstrated no leakage of contrast outside of the GI tract or into the pleural space, and CT five days after surgery revealed no evidence of communication between the stomach and pleural space (Figure ).\nShe tolerated an oral diet. Gastrostomy tube, jejunostomy tube, and chest tube were removed without complication. |
A 37-year-old man was admitted to our hospital for resection of a presumed liver metastasis. Three months earlier, the patient had undergone a right hemicolectomy for transverse colon cancer and a wedge resection for a solitary metastasis in Couinaud's segment VI of the liver. At that time, the TNM classification for the pathologic staging of colon cancer was T4 (serosal invasion), N1 (three regional lymph node metastasis), and M1 (liver metastasis). A CT scan performed prior to the current admission revealed a small ovoid, well defined, hypodense mass with peripheral contrast enhancement in the subcapsular region of segment VI adjacent to the previous resection site (). These CT findings suggested a liver metastasis. On the T1-weighted MR images, the mass was hypointense relative to the liver parenchyma, while it was hyperintense on the T2-weighted MR images. In addition, the T2-weighted MR image revealed a hypointense rim at the peripheral portion of the hyperintense mass (). Furthermore, the mass showed persistently peripheral enhancement on the gadolinium-enhanced MR images (). Two weeks after the CT scan, a PET/CT was performed to evaluate other occult metastases, including this lesion. The results of the PET/CT scan revealed the presence of a solitary liver mass with markedly increased accumulation of FDG (SUVmax, 9.6) (). The patient underwent a partial resection of the liver for the mass. Histology of the resected mass revealed a localized necrosis with a foreign body granuloma surrounded by a fibrous wall (). When correlated with the imaging findings, the central necrotic areas and the granulation tissues combined with the peripheral fibrous wall corresponded to areas of low attenuation and peripheral enhancement, respectively, on the CT images and to areas of hypointensity and peripheral enhancement, respectively, on the gadolinium-enhanced MR images (). The outer fibrous wall was especially well visualized as a peripheral hypointense rim on the T2-weighted MR image (). |
A 70-year-old woman was brought to the emergency department (ED) after being found down by her son in her home. She had a history of an old right posterior cerebral artery stroke which left her with mild left hemiparesis as well as a history of diabetes, coronary artery disease, and chronic atrial fibrillation for which she was anticoagulated on warfarin.\nOn arrival to her local ED, her mental status was intact, but she was febrile to 39.5°Celsius and hypotensive with a blood pressure of 70/53. Heart rate was tachycardic at 120 beats per minute and tachypneic at 28 breaths per minute. She was admitted to the intensive care unit, where she was aggressively fluid resuscitated and started empirically on intravenous meropenem and vancomycin. Her blood pressure stabilized and she transferred to the floor, where multiple sets of blood cultures grew group B Streptococcus agalactiae. Transesophageal echocardiogram demonstrated a 2 cm mobile mass on the atrial surface of her posterior mitral valve leaflet (), consistent with vegetation due to IE. She was then transferred via ambulance to our tertiary hospital for management of IE.\nEn route to our hospital, she developed acute right-sided weakness and difficulty speaking. Upon arrival at our ED, she was noted to have both expressive and receptive aphasia with marked right hemiparesis. She was seen immediately by a neurologist and her NIH Stroke Scale score was determined to be 24. Physical exam demonstrated garbled speech, right hemiparesis, right sensory loss, right pronator drift, and a right Babinski sign. She became drowsy and developed respiratory distress, prompting intubation. Head CT without contrast revealed evidence of an old right occipital lobe infarct but no acute process. After hemorrhage was excluded, CT angiography (CTA) and CT perfusion (CTP) imaging were performed to evaluate for potentially salvageable ischemic penumbra or acute thrombus. CTA () revealed complete abrupt occlusion of a large anterior left M2 segment. CTP () demonstrated an extensive area of diminished cerebral blood flow in the left middle cerebral artery (MCA) distribution, predominantly in the left frontal and parietal lobes. A moderate-sized penumbra of preserved cerebral blood volume was seen peripherally.\nEndovascular neuroradiology performed emergent intraarterial mechanical thrombectomy using the Solitaire FR revascularization device. Full recanalization was successfully achieved using the Solitaire retrievable stent (). There were no procedural complications and post-procedure CT scan revealed no evidence of hemorrhage.\nShe experienced significant clinical improvement following the procedure. Her right-sided weakness began to recover within a few hours, and after one day she was able to move all extremities against gravity. By day 4, she exhibited no residual signs of her left MCA stroke.\nShe was deemed to be a poor surgical candidate due to her poor baseline functional status. Antibiotic regimen was deescalated to intravenous gentamicin and ceftriaxone. She was discharged to a skilled nursing facility to continue her rehabilitation and complete her course of antibiotics. |
We present the case of a 48-year-old male, who was evaluated by the medical genetics service because he had noticed weakening of his voice with a high pitch since age 35, associated with premature graying since his 30s and skin lesions since about the age of 40. At the age of 32, bilateral cataracts were diagnosed and at 44 he was diagnosed with diabetes mellitus, currently on oral hypoglycemic agents. Additionally, he has hypothyroidism and hypertriglyceridemia in management and calcification of the Achilles tendon. Patient endorses lack of an early adolescent growth spurt; however, final stature is similar to his other 3 siblings (164 cm). Patient reports he had no child by choice.\nPatient is product of the union of consanguineous parents (second cousins) and has a 49-year-old brother with similar clinical characteristics, including voice changes since the age of 28, bilateral cataracts at age 29 (subsequently presents complications from corneal ulceration and is currently legally blind), and premature graying since age 33, moreover, scleroderma-like skin changes since his 30s and diagnosis of type 2 diabetes mellitus at age 35. His brother also endorses no child by choice. No other complications such as atherosclerosis, dyslipidemia, hypertension, osteoporosis, or tumors were reported.\nUnfortunately, patient's brother and parents declined genetic testing. There are no other relatives with clinical suspicion of WS.\nPatient states maternal aunt has unspecified type leukemia and father with a history of acute myocardial infarction at age 65 and a diagnosis of melanoma at age 85. Maternal uncle diagnosed with lung cancer at age 72 and maternal grandfather with prostate cancer diagnosed at age 73.\nOn initial physical examination, he appeared much older than his age with “bird-like” facial appearance, beak-shaped nose, and bilateral cataracts, his voice was high-pitched and his hair and eyebrows were scarce and markedly gray. He had thin upper limbs with decreased subcutaneous fat and truncal obesity (). Moreover, we found short stature, hypogenitalism, lower limbs with markedly atrophied skin and subcutaneous fat, abnormal pigmentation of the skin and hyperkeratosis, and flat feet (Figures and ).\nWRN gene sequencing identified the homozygous variant NM_00553.4: c.2581C>T (NP_000544.2: pGln861Ter). WRN gene sequencing report can be found in Supplementary . This variant generates a stop codon at position 861 and has been classified as pathogenic and previously described in homozygous status in a Caucasian patient from the United States in 2006 [].\nLaboratory findings included normal renal function, high blood glucose (164 mg/dl), elevated glycosylated hemoglobin (9.4%), and elevated triglycerides (324.6 mg/dl) with normal cholesterol (162.4 mg/dl). EKG showed an elevation of the J point by early repolarization. Abdominopelvic CT-scan showed bilateral renal cysts, small umbilical hernia, and no fatty liver. Testicular ultrasound showed decreased bilateral testicular volume mainly left side.\nRegular screening for malignancies is recommended for patients with WS, due to the high risk of early-onset neoplasms. Also, it is very important to rule out cardiovascular and metabolic diseases during the follow-up of these patients. Our patient is still under periodic clinical observation and follow-up. Currently, he is on treatment with oral hypoglycemic agents for DM2 with adequate glucose control and in treatment of hypertriglyceridemia. Until now no signs of atherosclerosis or cardiovascular disease have been detected. However, he was recently diagnosed with refractory cytopenia with multilineage dysplasia, a form of myelodysplastic syndrome, which has required multiple transfusions.\nAccording to a clinical history, the patient's brother is being monitored for inadequate control of diabetes mellitus and severe skin lesions that have been difficult to treat, but no cancer has been documented. |
A 77-year-old man is a victim of hypertension and diabetes under insulin control at our outpatient clinic. He complained of chest tightness and dyspnea on exertion for a period. He had a history of tobacco used but quit for decades. Echocardiography was performed on 2011 September and the result showed normal left ventricular function without regional wall motion abnormality. Due to frequent chest tightness complained on exertion and the symptom exacerbated. He visited our emergency department for help. At emergency department, CXR showed cardiomegaly. Cardiac enzyme was checked and it was within normal limit. Under the impression of coronary artery disease with angina, he was treated with dual antiplatelet therapy (aspirin 100 mg/day, clopidogrel loading 300 mg, then 75 mg/day) at emergency department and catheterization was suggested after admission. Three days later, catheterization was done and coronary angiography demonstrated left anterior descending artery (LAD) with critical lesion and left circumflex (LCX) atherosclerotic lesion. Angioplasty was performed and two drug-eluting stents (BIOMATRIX 3.0 × 18 and 2.5 × 18) were deployed on LAD and LCX, respectively, with clopidogrel loading 300 mg (). The hospital course was uneventful and he was discharged on aspirin and clopidogrel on the following day.\nHe was transferred to emergency room again at the noon on the next day after discharge, and he complained of severe acute onset chest pain with diaphoresis. On admission, his blood pressure was 150/96 mmHg, and pulse beats 110/min. His electrocardiography (ECG) showed ST elevation in leads V1-V5. Over the next 10 minutes, his blood pressure dropped to 65/48 mmHg with complete atrioventricular block by ECG monitor, and he was transferred immediately for emergency coronary angiography. After implantation of a temporal pacemaker, coronary angiography revealed totally occluded mid LAD and mid LCX at the same time, the site over the prior stents (). Balloon angioplasty restored Thrombolysis in Myocardial Infarction (TIMI) 2 flow in both sites. Because of low left ventricular ejection fraction and a large thrombus burden by SETMI secondary to stents thrombosis, re-occlusion developed later resulted in failure from primary PCI. |
An otherwise healthy 27-year-old woman presented with severe upper abdominal pain, multiple episodes of vomiting and absolute constipation for the previous 12 hours. The pain was acute in onset, severe and continuous. It was initially located in the epigastrium but became generalized with progression of time. It was severe enough to warrant a continuous intravenous infusion of meperidine. The pain was associated with multiple episodes of nonbilious vomiting. She had no other comorbid conditions and had not undergone any operations in the past. On examination, she was drowsy with a pulse of 110/minute, blood pressure of 90/50 mm Hg, temperature of 37°C and respiratory rate of 23/minute. She was dehydrated. Abdominal examination revealed diffuse abdominal tenderness and voluntary guarding in the right lower quadrant. Bowel sounds were audible. A reducible paraumbilical hernia with a 2 × 2 cm fascial defect was also noticed. Breath sounds in both lungs were equal and vesicular. Results of laboratory investigations were normal except for a borderline elevated white blood cell count []. A CT scan of the abdomen was done. This revealed dilated distal small bowel with features of bowel ischemia and significant free fluid []. After initial resuscitation, the abdomen was explored through a midline incision. Approximately 1.5 liters of reactionary peritoneal fluid was drained. The proximal ascending colon and two thirds of small intestine had herniated through a large mesenteric defect. Fifteen centimeters of mid-ileum was grayish-black in color with loss of peristalsis and absent pulsations. The terminal ileum was viable. Pulsations of mesenteric vessels were intact. On splaying out the bowel, a large mesenteric defect measuring approximately 12 × 15 cm was noted. The arcades in the mesentery were intact running in close proximity to the mesenteric edge of the bowel. Distal to the proximal jejunum, true mesentery was absent. There was no evidence of malrotation. A redundant sigmoid colon was also appreciated. The gangrenous segment of small bowel was resected and a stapled side-to-side functional anastomosis created. The previously described mesenteric defect was closed. This resulted in a configuration that would have a very high likelihood of twisting. To minimize this risk, the small bowel loops were systematically positioned side by side. The arrangement was secured by intermittent tacking sero-muscular sutures placed between bowel loops. Care was taken to avoid kinking of the small bowel [Figures , ]. The patient tolerated the procedure well. Bowel function resumed on the third postoperative day. Diet was initiated and tolerated. She was discharged on the sixth postoperative day, the delay being due to social issues. On follow-up visit at 2 months, she was doing well. |
An 18-year-old girl admitted in our department on February 2014 with the complaints of pain and swelling over medial aspect of upper one-third of the left forearm [] for the past 6 months, and the mass was gradually increasing in size, for which incisional biopsy was done outside and referred with biopsy report as glomangioma or epithelioid haemangioendothelioma. On examination, there was a palpable firm mass on the medial volar aspect of the left forearm measuring about 7 cm × 5 cm × 3 cm with surgical scar with few dilated veins over the swelling with no bruit. There was no distal neurovascular deficit. Hand functions were normal. Magnetic resonance imaging of the upper forearm showed the mass with high vascularity, involving mainly the muscular plane. The biopsy report showed the possibility of glomangioma or epithelioid haemangioendothelioma, which was a benign condition, and so excision biopsy was done.\nA lazy 'S' incision was made over the swelling. Highly vascular mass was found encapsulated within the flexor digitorum superficialis (FDS) muscle at the common flexor origin [ and ]. The ulnar nerve was found closely adhered to the capsule of the mass and was dissected after dividing its muscular branches []. Mass was excised along with part of FDS muscle up to the musculotendinous junction; the remaining tendinous part was attached to flexor digitorum profundus muscle with adequate tension.\nHistopathology examination showed nests of large polygonal cells with abundant eosinophilic cytoplasm having pseudoalveolar pattern [ and ], separated by delicate vascular structures. All the findings confirmed ASPS.\nThe patient was screened for metastasis post-operatively. Computed tomography (CT) chest showed multiple coin-like opacities in both the lung fields, more in lower lobes []. CT brain, CT abdomen and whole-body skeletal survey were normal.\nAfter discussion with oncology department (radiation oncology, medical oncology and surgical oncology), we decided to give cycles of chemotherapy and radiotherapy.\nThe patient received 6 cycles of chemotherapy with adriamycin, cyclophosphamide and vincristine as suggested by the oncologist. The patient was closely followed for 11 months. There was no locoregional recurrence or metastasis elsewhere. Full range of movements in the hand and elbow was possible.\nAfter 11 months, the patient had good functional improvement [ and ], due to financial constraints, PET scan and other investigations were not done on subsequent follow-up. |
A 24-year-old pregnant woman (G2P1) was referred to us due to suspected bilateral ovarian cysts at 8 weeks of gestation. She had undergone ovarian cystectomy twice under open surgery: left and right ovarian cystectomy for mature cystic teratoma and mucinous cystadenoma, respectively. She had no additional medical history or familial medical history. Transvaginal ultrasound and magnetic resonance imaging (MRI) (Figures and ) revealed two pelvic cysts. The left-sided unilocular cyst was 9 cm in diameter. The right-sided multilocular cyst was 5 cm in diameter. We diagnosed this condition as bilateral ovarian cysts.\nAlthough the serum levels of tumor markers (CA125, CA19-9, and CEA) were normal for a pregnant woman, considering the large size of the cyst, cyst resection was attempted at 14 weeks; however, it was converted to probe laparotomy. Marked adhesion around the cysts, posterior uterus, and Douglas' pouch made cyst resection impossible as extensive adhesiolysis may cause uterine damage and also uterine contractions after surgery. Gross examinations revealed no metastatic lesions or lymph node swelling. Abdominal fluid cytology revealed no malignant cells.\nAt 32 weeks of gestation, MRI revealed that the left-sided cyst size had increased to 27 cm in diameter (Figures and ), although she was asymptomatic. As shown in , the right-sided multilocular cyst became very close to the left monocytic cyst. At this stage, the left large monocytic cyst appeared to merge with the smaller right multilocular cyst, forming a large cyst occupying the entire pelvic cavity, which was later confirmed by laparoscopic findings.\nThis large cyst showed no solid-part or papillary growth. The serum levels of tumor markers remained normal. Malignant ovarian tumor could not be ruled out but was considered less likely. We weighed merits and demerits between relaparotomy for tumor resection during pregnancy and a wait-and-see approach for several weeks; the former is likely to require extensive adhesiolysis and may cause preterm delivery. We decided on the latter strategy, since resection should be performed in the event of a size increase or images indicative of malignancy. The fetus normally developed without fetal growth restriction.\nCesarean section and tumor resection were performed at 37+4 weeks of gestation, yielding 3,012-g male infant with Apgar score 8/9 at 1/5 minutes, respectively. The infant did not have congenital abnormalities. After the completion of cesarean section, we ruptured the wall of this large cyst, with care to avoid the cyst content entering into the abdominal cavity. A large amount of serous fluid was drained. This large cyst was a multicystic cyst (5 cm), considered to be the right multicystic ovarian cyst that had been observed from the first trimester. The wall of the large cyst showed marked adhesion to the peripheral peritoneal cavity. We resected it as widely as possible together with right salpingo-oophorectomy (Figures and ). The left ovary was macroscopically normal, and thus there was no evidence of the left ovarian tumor. The resected tumor consisted of a large unilocular cyst with serous fluid and a mucinous cystadenoma (Figures and ). In the former, lining epithelium was absent in many parts () and mucinous epithelium was occasionally found in continuity with the cyst wall of the latter (right ovarian cystadenoma). No malignant cells were found in the resected specimen. Immunohistochemistry revealed focally positive staining for estrogen and progesterone receptors on the resected cyst wall (Figures and ). At 12 months after the delivery, left ovary remained normal and the retention cyst did not recur. An informed consent for this reporting was obtained from this patient. |
A 12-year-old boy presented to the Pediatric Emergency Department shortly after receiving an electric shock at a nearby apartment building. As the boy, his mother, and younger brother were waiting outside the building for friends to “buzz them in” (), an individual exited the building and held the door open. The mother and younger brother entered without touching the door. As our patient entered the building, he grasped the metal door knob with his left hand while at the same time keeping his right hand on a metal stair railing that was adjacent to the entrance. This action occurred just as the door entry mechanism was activated from upstairs. As a result, the patient sustained an electrical shock inducing upper extremity tetany, prohibiting him from releasing his grip. The shock lasted approximately 5 seconds and was witnessed by the patient's mother. The patient retained consciousness throughout the event and did not fall afterwards.\nInitial evaluation in the Emergency Department centered on the potential cardiac, myopathic, and renal aspects of the injury. The patient was admitted for telemetry monitoring in the Pediatric Intensive Care Unit. No arrhythmias were noted. The following morning, the patient complained of bilateral wrist pain. He had mild swelling and erythema, therefore an orthopaedic surgery consult was requested for evaluation.\nThe child was able to use both hands, favoring the right over the more painful left. There were no burn marks or abrasions, but mild, bilateral, distal radius volar angulation deformities were present. Anteroposterior, lateral, and oblique radiographs of both wrists revealed bilateral, buckle-type, apex-dorsal angulated fractures of the distal radial metaphyses (Figures and ). The patient was placed in bilateral volar splints to reduce wrist motion, preserve hand function, and permit examination of his skin. He was discharged home directly from the PICU on hospital day three and followed an uneventful course to clinical and radiographic healing. |
A 55-year-old healthy Caucasian female with no history of ocular disease was observed in the emergency department with fever, myalgia, nasal congestion with 3 days of evolution, and a generalized rash for 1 day. She had contact with dogs. Physical examination revealed a cooperating and well-oriented patient with stable vital parameters and a tympanic temperature of 38.2°C. There was a maculopapular rash on the upper limbs, abdomen, and the dorsal region. A tache noir, a small crust surrounded by a violet halo, was visible in the region of the left thigh. There were no meningeal signs and no cardiorespiratory or abdominal changes. Serology for R. conorii was positive with an IgM titer of 1/320. Autoimmune and thrombophilia studies were negative. Hypercholesterolemia and systemic hypertension were excluded. The patient was admitted to our department and therapy with oral doxycycline 100 mg every 12 h was initiated. Oral doxycycline was given for 10 days.\nOn the first day of hospitalization, the patient reported decreased visual acuity (VA) of the right eye (RE). A CT scan was performed which was normal. No ophthalmologic examination was requested.\nThere was a systemic improvement and the patient was discharged after 6 days. Five days thereafter, the patient returned to the emergency room with complaints of worsening of RE VA. Observation revealed an RE VA of 20/100 and there was a superior temporal artery branch occlusion with perivascular sheathing in the ocular fundus as well as cotton wool spots and edema (fig. ). The retinal edema affected the macular region (fig. ). Campimetric examination revealed an inferior nasal scotoma, consistent with fundus lesions (fig. ).\nIntravitreal injection of triamcinolone acetonide 3.2 mg/0.08 ml was given only once and showed significant improvement of the retinal edema and a recovery of RE VA to 20/25 after 1 month. The vascular sheathing and the occlusion persisted (fig. ), and so did the corresponding scotoma in the visual field.\nThe total follow-up period is 1 year. No ocular side effects related to intravitreal triamcinolone were detected. |
A 44-year-old female patient presented to the Outpatient department with a chief complaint of swelling in the lower jaw since 6 months. There was difficulty in speech, mastication, and deglutition. There was no associated pain. She had no contributing medical history.\nOn extra oral examination a large well-defined swelling was noticed in the mandibular anterior region crossing the midline causing facial asymmetry [].\nThe swelling extended below the inferior border of the mandible and the skin over the swelling was stretched and smooth. She had difficulty in opening the mouth. No lymph nodes were palpable.\nIntraoral examination showed a large swelling, which extended completely into the floor of the mouth and completely obliterating the lingual and buccal vestibules mediolaterally [].\nThe lingual frenum was pushed back. Anteriorly, it extended from the labial sulcus to the ramus posteriorly. There was no surface discharge present, the mucosa over the swelling was normal and its color was same as that of the normal tissue. All the mandibular incisors were missing. Generalized extrinsic stains were present.\nOn palpation the inspectory findings were confirmed. The swelling was bony hard, non-tender, and immobile. None of the teeth present showed mobility.\nRoutine blood and urine examination was normal. Fasting and post-prandial sugar levels were also normal.\nComputed tomography showed a large multilocular osteolytic lesion extending from 37 to 47 region crossing the midline, destruction of both cortices and pathologic fracture was seen []. Soap bubble and honeycomb patterns were appreciable. A multi-centric growth pattern was seen showing a permeative type of destruction [].\nA chest radiograph was taken to rule out any primaries in the lung.\nA provisional diagnosis of ameloblastoma was established. A differential diagnosis of odontogenic keratocyst was made.\nAn incisional biopsy was performed under local anesthesia and microscopic examination revealed odontogenic islands infiltrating the connective tissue, the peripheral tall columnar cells showed proliferation and peripheral palisading of basal cells with reverse polarity of the nucleus. Stellate reticulum was scanty [].\nHigh power view showed cells with atypical features of pleomorphism, hyperchromatism, altered nuclear-cytoplasmic ratio, and mitotic figures [].\nA final diagnosis of ameloblastic carcinoma was established.\nA total mandibulectomy was carried out under general anesthesia []. An immediate reconstruction was done []. |
A previously healthy 39-year-old woman was referred to our hospital because of a cystic lesion in the liver demonstrated by abdominal ultrasonography (US). Laboratory studies, including liver function tests, and tumor markers were also within the normal limits. Serological markers for hepatitis B or C viral infection were undetectable. Abdominal US revealed a well demarcated, heterogeneously low-echoic mass 170 mm in diameter in right lobe of the liver. Abdominal computed tomography (CT) during hepatic arteriography (CTHA) revealed early ring enhancement in the peripheral area in the arterial phase and slight internal heterogeneous enhancement in the delayed phase (Figures and ). Magnetic resonance imaging (MRI) showed that the tumor had low signal intensity on T1-weighted images and some foci of high signal intensity on T2-weighted images. Gadolinium ethoxybenzyl (Gd-EOB) MRI revealed no uptake in the corresponding area (Figures , , and ). Abdominal angiography demonstrated a large avascular region in the liver corresponding to the tumor, although no typical features of cavernous hemangioma were evident (). 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) revealed no abnormal FDG uptake. With these radiological findings, malignant liver tumor could not be excluded, such as biliary cystadenocarcinoma, cholangiocarcinoma, mesenchymal tumors, and hepatocellular carcinoma associated with cystic formation.\nThe patient underwent posterior sectionectomy. Intraoperative examination revealed a relatively soft dark red tumor (); the resected specimen weighed 1.1 kg and measured as 170×100×80 mm. The cut surface of the tumor revealed a white, solid, and cystic mass that was elastic, soft, and homogeneous with a yellowish area considered to be myxoid degeneration (). Histological examination showed that the tumor mostly consisted of sclerotic area and cavernous hemangioma area is partly observed (). Sclerotic area presents diffuse fibrosis () and the typical histology of cavernous hemangioma was confirmed in some parts. In addition, marked increase and dilation of medium sized veins with cavernous form were frequently noted in the surrounding areas of tumor (). The increased and dilated veins show positivity of CD31 immunostaining being a marker of endothelium (). The pathologic features were consistent with sclerosing hemangioma. The postoperative course was uneventful, and the patient was discharged on postoperative day 10. |
A 41-year-old pregnant Japanese woman, gravida 4, para 3, underwent three consecutive cesarean sections. She became aware of abdominal distention 3 months after her third cesarean section, which was performed in a private clinic. Therefore, she visited the general hospital, and she was diagnosed as having an abdominal wall incisional hernia (Fig. ). She underwent an operation of hernia repair with use of monofilament polypropylene mesh 7 months after her third cesarean section. However, a surgical site infection associated with the mesh occurred 2 months after the hernia repair. Although she received intravenous infusions of antibiotics for 14 days, pus continued to discharge from a cutaneous fistula. She was found to be pregnant at that time.\nThe patient and her spouse did not desire termination of the pregnancy. She was referred to Kobe University Hospital at 13 weeks and 2 days of gestation, at which time she was found not to have fever, abdominal pain, or peritoneal irritation signs. Her body mass index prior to pregnancy was 24.4 kg/m2. Her white blood cell count (8800/μl) and serum C-reactive protein level (0.87 mg/dl) were slightly increased. A cutaneous fistula was present in the lower abdominal wall, and pus discharged from the fistula (Fig. ). Streptococcus agalactiae and Peptostreptococcus micros were isolated by bacterial culture of pus. Magnetic resonance imaging performed at 14 weeks of gestation clearly demonstrated an accumulation of pus surrounding the surgical mesh (Fig. ).\nSurgeons performed an operation to remove the infected mesh at 16 weeks and 3 days of gestation. During the surgery, it was found that an infection had spread to the soft tissue surrounding the mesh and that the infected tissue strongly adhered to the bladder, omentum, and small intestine. The bladder wall and the serosa of the small intestine developed defects during the adhesiolysis, resulting in a 4-cm, full-thickness opening in the dome of the bladder and a defect of the serosa of the small intestine with a 2-cm width. The bladder mucosa was repaired by using interrupted sutures with 0.2-mm polydioxanone monofilament sutures, and the muscular layer and the serosa of the bladder was repaired by using a continuous suture with 0.2-mm multifilament absorbable sutures. The defect of serosa of the small intestine was repaired by interrupted sutures with 0.2-mm multifilament absorbable sutures. An interrupted one-layer mass closure with 0.35-mm polydioxanone monofilament sutures was used on the peritoneum and abdominal fasciae, and a vertical mattress suture with 0.2-mm nylon sutures was used for skin closure.\nThe patient received intravenous infusions of ampicillin/sulbactam (12 g/day) and ritodrine hydrochloride for prophylaxis of uterine contraction for 2 weeks. After this operation, the pus discharge completely stopped. The patient was discharged from the hospital 32 days after the operation, when her white blood cell count and serum C-reactive protein level had returned to normal without a uterine contraction or a fever. Neither hernia nor infection at a surgical site recurred during the rest of her pregnancy as well as during the puerperal period.\nA cesarean section with an upper abdominal incision, followed by a transverse uterine fundal incision, was planned with the patient’s informed consent. A female newborn weighting 2478 g with Apgar scores of 9 (1 minute) and 9 (5 minutes) was delivered successfully by cesarean section at 37 weeks and 3 days of gestation. The same procedure used in the previous operation to remove the infected mesh was performed for the upper abdominal wall closure. The patient’s blood loss volume was 820 ml. The patient and her child were discharged 8 days later, and the remainder of the hospital course was uneventful. However, an incisional hernia of the upper abdominal wall occurred 4 months after this cesarean section (Fig. ). Seven months after the cesarean section, surgeons repaired this hernia by open surgery without use of surgical mesh and by the same procedure for abdominal wall closure used in the previous operations. The incisional hernia of the abdominal wall did not recur for at least 10 months thereafter. |
A 78-year-old Japanese woman presented to our hospital because of colicky left abdominal pain. She had no history of any hip or neuromuscular disease. Her past medical history was only a Cesarean section. Physical examination revealed her height as 155 cm and weight as 35 kg (body mass index 14.5), and she was afebrile and in good general health. Blood examination and urinalysis was normal. She complained of left costovertebral angle tenderness. Computed tomography (CT) revealed dilated left ureter with herniation of the ureter into the sciatic foramen (). Drip infusion pyelography (DIP) identified that the left ureter was dilated and ran an unusual and convoluted course laterally through the pelvis (). We diagnosed her case as left ureteric sciatic hernia on the basis of these findings.\nThe patient's symptoms spontaneously are resolved before long. We followed up her closely without treatment for four months. However, the patient's symptoms recurred occasionally, and the left hydroureter remained unchanged. We decided to laparoscopically repair the hernia.\nOperative Management. The patient was positioned in a head-down right semilateral position after general anesthesia. A balloon trocar was placed superior to the umbilicus. Three accessory ports (5 mm) were placed under direct laparoscopic control, and a three-dimensional (3D) laparoscopy system (Shinko Optical, Tokyo, Japan) was used. The peritoneum overlying the common iliac artery was elevated and transected, and the left ureter was identified on the artery. The ureter was mobilized all the way to the sciatic foramen by blunt dissection. The herniated ureter was reduced with light traction. A small defect was identified and repaired by suturing the edges of the surrounding connective tissue in order to close the hernia sac. The patient had an uneventful recovery. A month after the laparoscopic repair, DIP revealed that the left ureter had returned to its normal anatomical position without ureteral obstruction. Eight months after the surgery, the patient remains asymptomatic. |
An 89-year-old man presented with sudden onset of bilateral ptosis on November 2, 2005. He had a one week history of xerostomia. Three days prior to hospitalization, the patient was diagnosed with diabetes mellitus and had a history of surgery for gastric cancer, which occurred four years ago. His fasting glucose level and postprandial (2 hr-after-meal) glucose level were 196 mg/dL and 300 mg/dL, respectively.\nHis visual acuity was 20/100 in both eyes. The right pupil was mid-dilated and had no light reflex. Complete ophthlamoplegia of the right eye and superior rectus palsy of the left eye were observed. Bilateral ptosis, mild proptosis, and periorbital puffiness were also observed. His anterior segment examination was notable for moderate nuclear sclerotic cataracts and mild chemosis, while fundoscopic examination revealed no specific abnormal finding.\nBrain and orbit magnetic resonance imaging (MRI) revealed no specific abnormal findings in the cavernous sinus and orbit, except bilateral midbrain infarction and mild diffuse sinusitis (). Based on the brain MRI finding, the bilateral ptosis and superior rectus palsy of the left eye were regarded to be caused by 3rd nuclear palsy. The results of CSF analysis showed that white blood cell count and protein were elevated and intravenous antibiotics were given for a central nervous system infection.\nOn November 3, 2005, the visual acuity of both eyes decreased to perception of hand motion and bilateral complete ophthlamoplegia developed (). Anterior segment examination revealed no specific change compared with the previous finding. Fundoscopic examination revealed that the optic disc was pale and the posterior pole was generally edematous in both eyes. A cherry-red spot was found in the left eye (). Fluorescein angiography (FAG) demonstrated that retinal vessels were not imaged, even 10 minutes after injecting the dye.\nChoroidal perfusion was not found in the right eye and partial choroidal fluorescence was observed in the left eye (). Thus, he was diagnosed with simultaneous bilateral ophthalmic artery occlusions. On the same day, rhinologic examination revealed a destructed nasal septum, black necrotic lump in the nasal cavity and an ulcer on the hard palate, which was confirmed as murcomycosis upon biopsy ().\nBased on the clinical findings and biopsy result, ROCM was diagnosed and Amphotericin B (50 mg/day) was intravenously administered. Although extensive local excision was required, the patient and his guardian rejected to have surgery. On November 6, 2005, the patient died. |
A 44-year-old man complaining of intermittent constipation for 3 years presented to his local hospital with an increased severity of constipation of 3-month duration. Computed tomography (CT) scan showed a large presacral mass measuring 9 cm in diameter, and he was then referred to our hospital. His past medical history was significant for colostomy performed just after birth for anal atresia and anorectoplasty with stoma closure when he was 3 years old. He had occasionally, several times a year, suffered from incontinence. In his family history, his father died of lung cancer at the age of 65, and his mother was 80 years old and healthy. He had no siblings or children. He had no family history of hereditary diseases. He was not receiving regular medications.\nPhysical examination of the abdomen showed a surgical scar extending from the left upper to lower quadrants without palpable masses. At the perineum, the rectal mucosa was anastomosed to the skin by the previously performed anoplasty. Digital rectal examination showed that the aperture of the rectum (plastic anus) was hard and that the lumen of the rectum was narrow. An elastic hard presacral mass was palpable at 4 cm from the anal verge. He could voluntarily constrict the anal sphincter. The rest of the physical examination, including urinary and genital systems, did not reveal any abnormalities.\nLaboratory analyses were normal, including tumor markers (carcinoembryonic antigen, carbohydrate antigen 19–9, anti-p53 antibody, alpha-fetoprotein, prostate-specific antigen, neuron-specific enolase, and squamous cell carcinoma antigen).\nAnorectal manometry revealed an impaired anal function; the maximum static pressure was 22 mmHg (normal range 26–64 mmHg), and the voluntary contraction pressure was 43 mmHg (normal range 59–206 mmHg).\nColonoscopy and contrast enema showed that the lower rectum was stenotic but with intact mucosa.\nCT scan showed a partial defect in the right side of the sacrum with deformity, a 60 × 30 × 21-mm meningocele which was protuberated anteriorly from the level of the S3 vertebra, and a 90 × 85 × 68-mm cystic mass which was located anterior to the meningocele, compressing the rectum ventrally (Fig. ). In addition, double renal pelvises were observed in the right kidney, and the inferior mesenteric artery branched from the superior mesenteric artery. On magnetic resonance imaging (MRI), the inside of the cystic tumor showed heterogenous high intensity in T2-weighted image, and an almost homogenous low intensity in T1-weighted image, with the wall showing enhancement with Gadolinium (Fig. ). The existence of the fat component highly suggested teratoma as a preoperative diagnosis. The levator ani muscle appeared intact, except for partial atrophy.\nFluorodeoxyglucose-positron emission tomography showed no abnormal uptake. Myelography showed an inflow of the contrast agent into the meningocele, but not into the presacral tumor.\nResection of the presacral mass was performed, based on a diagnosis of Currarino syndrome associated with presacral teratoma and meningocele. A postsacral midline incision was made with the patient in the jackknife position. After S3-4 laminectomy, the meningocele was dissected by ligating the root at the level of the dura. Then, the removal of the coccyx with an extended incision provided excellent visualization of the teratoma (Fig. ). The posterior wall of the most distal area of the rectum was severely adherent, resulting in tumor rupture during dissection. We completely removed the clay-like content and the capsule. The peritoneal cavity was not opened. The wave of the anal sphincters, monitored by motor-evoked potential (MEP), did not attenuate throughout the operation. The operation time was 8 h 56 min and intraoperative blood loss was 430 ml (including spinal fluid).\nPathological examination revealed a mature teratoma (Fig. ). The cyst wall was composed of keratinizing stratified squamous epithelium surrounded by fat, sweat glands, and peripheral nerves. The content, 200 g in weight, was composed of a histologically keratinized substance. The coccyx showed no abnormality.\nOral intake was started on the fourth postoperative day. He was discharged from the hospital on the 15th postoperative day with no morbidity. His constipation dramatically improved postoperatively. No urological or rectal complications, or lower extremity neuropathy, were observed. |
A 12-year-old female patient was referred to the Department of Oral and Maxillofacial Surgery with a complaint of painless swelling of the lower jaw. The patient had noticed the swelling 4 years back which gradually increased to the present size. Extraoral examination revealed the presence of an asymmetric swelling of the mandible causing deviation of the chin to the right side. The skin over the swelling was normal. The jaw movements and the temporomandibular joint (TMJ) movements were normal []. On intraoral examination, the swelling was seen to extend from the right angle to the ramus on the left side. The labial and buccal mandibular sulci were obliterated and buccolingual expansion was evident. The teeth present were 33, 36, 41, 42, 43, 45 and 46 with multiple missing teeth and displacement of 33, 41, 42 and 43 [].\nOrthopantomograph (OPG) revealed a multilocular radiolucency extending from the right ramus to the left ramus region with an expansile swelling in the lower border of the mandible []. Apart from the teeth present clinically, impacted second permanent molars on both sides as well as an impacted paramolar on the right side were discerned. Expansion of the cortical plates buccally and lingually was appreciated. Displacement of 33, 41, 42 and 43 and resorption of roots of 41, 42, 43, 36 and 46 could be appreciated.\nVisor incision was placed, layer by layer dissection was performed at the subplatysmal layer and flap was elevated to explore the mandible from the right to left condylar region. Facial artery and vein were preserved for the reconstruction procedure. Genioglossus and geniohyoid muscles were resected. The affected portion of the body of the mandible was resected from right to left subcondylar region after making bilateral osteotomy cuts. Right fibular graft harvested by the plastic surgery team was osteotomized at the bilateral canine region, adapted to the titanium reconstruction plate and fixed with screws. Microvascular anastomosis was done. Glove drain and Ryles tube were placed. It was closed with 3-0 vicryl suture and 4-0 ethilon sutures. The resected tumor mass was sent for histopathological examination.\nA specimen x-ray was taken which revealed similar radiologic findings as the preoperative OPG []. Multiple sections were grossed from different areas of the specimen []. The cut section shows areas of yellowish white coloration along with pale hemorrhagic areas with rough borders. It was friable and soft in consistency measuring 4.5 × 5.5 cm in dimension. Brownish fluid oozed out while grossing (suggestive of cystic degeneration). Fibrous areas intermixed with fatty areas were seen. Right posterior border of the specimen was grossed. It was creamy white in color, hard in consistency, measured 2.7 × 2.3 cm in dimension and contained the second permanent molar.\nThe histopathological examination of the soft tissue revealed a richly cellular stroma comprising of plenty of evenly distributed multinucleated giant cells []. Few areas of pathologic resorption of bone were also evident []. High power view showed multinucleated giant cells with agglomeration of around 25–50 nuclei in center surrounded by clear cytoplasm, the proliferating stromal cells were hyperchromatic and pleomorphic [Figures and ]. Decalcified section of the specimen with impacted teeth showed dentin, bony trabeculae and myeloid tissue. The serum calcium levels and alkaline phosphatase levels were evaluated to rule out hyperparathyroidism.\nBased on the characteristic appearance of giant cells, stromal cells findings and lab investigations; a diagnosis of GCT was given. |
A 69-year-old African American female presented in February 2015 in our department with the chief complaint of gross hematuria and dysuria that started in December 2014. Prior to urology evaluation, she had received two courses of antibiotics without resolution for her presenting symptoms. The patient denied history of urologic trauma, nephrolithiasis, chronic Foley catheter, family history of genitourinary (GU) malignancy, or previous GU surgeries. The patient had a history of stage IA adenocarcinoma of the right upper lung in 2011 and a 20-pack year history of smoking.\nCystoscopy revealed a large complex bladder mass on the right lateral wall and right trigone involving the right ureteral orifice. Abdominal and pelvic CT scan revealed right-sided bladder mass involving the right ureterovesical junction, right hydronephrosis and right-sided pelvic lymphadenopathy. Transurethral resection of the bladder tumor (TURBT) was performed and pathologic examination showed a prominent inflammatory background with admixed high-grade undifferentiated tumor cells arranged in sheets with ill-defined cytoplasmic borders imparting a syncytial appearance diagnostic of the LEL variant of urothelial carcinoma (Fig ). Foci of urothelial carcinoma in situ were also noted involving the surface urothelium. Muscularis propria invasion was present.\nPatient was treated with four cycles of neoadjuvant chemotherapy of gemcitabine and cisplatin. Repeat CT two weeks after the last round of chemotherapy revealed smaller right-sided bladder mass (6.1 x 2.9 cm vs. 6.9 x 3.4 cm). Two months after the last round of chemotherapy, the patient underwent a radical cystectomy with ileal conduit diversion and pelvic lymph node dissection in July 2015. The operation was complicated by extensive adhesions from previous appendectomy, hysterectomy, and hernia repair with ventral mesh placement necessitating small bowel resection.\nFinal pathology report showed three high-grade tumor foci in the bladder with the largest being high-grade urothelial carcinoma located in the right lateral wall measuring 2.8 cm, another tumor located in the posterior wall measuring 0.9 cm with areas of squamous differentiation, and the smallest tumor located at the dome measuring 0.6 cm with pathology consistent with LEL urothelial carcinoma. The LEL variant of urothelial carcinoma is rare and diagnosed by the presence of high-grade/poorly differentiated tumor cells admixed with a prominent inflammatory cell infiltrate. The tumor cells have high nuclear:cytoplasmic ratios and indistinct cytoplasmic borders imparting a syncytium-like appearance. The overall appearance is similar to the lymphoepitheliomas typically seen in the nasopharyngeal region. They can be seen in the bladder either in the pure form or admixed with more usual forms of high-grade urothelial carcinoma, as seen in the present case. One obturator lymph node was positive for metastatic urothelial carcinoma (1/17 nodes positive). All surgical margins were negative. Final pathology staging was pT3bN1MX.\nPatient had an uneventful recovery and was discharged on post-operative day 12 to a skilled nursing facility. Repeat abdominal and pelvis CT at 9 weeks post-op showed no mass, lymphadenopathy, or destructive osseous lesions. Patient reported improvement in appetite and normal bowel movement with persistent mild abdominal pain. However, lung cancer follow-up chest CT in September 2015 revealed a new 3 mm left upper lobe nodule not present in preoperative chest CT in June 2015. Repeat chest CT in February 2016 showed left upper lobe nodule enlarged to 12 mm. CT-guided needle biopsy showed CK7+, p40+, and GATA3+ tumor cells similar to morphology of previous bladder cancer, consistent with metastatic urothelial carcinoma. Multiple new liver lesions were present on repeat CT in April 2016. Patient unfortunately died with metastases in October 2016. |
A 62-year-old woman was referred to our outpatient clinic in February 2013, after visiting two different neurologists in 2012 who diagnosed her with CD, followed by an unsuccessful treatment episode in a rehabilitation clinic. The patient reported the following progressive problems since 2009: loss of power in the left arm and leg, and problems keeping her balance, sometimes resulting in a drunken gait and falling. In the 4 years before referral to our clinic, the patient had fallen spontaneously five times, without losing consciousness and with the ability to stand up immediately afterward. Furthermore, double vision occurred during fatigue, and the patient had difficulty articulating, difficulty finding words, pain in almost all joints and muscles, poor memory function, and difficulty in concentration due to which she could no longer read or solve puzzles – her favorite pastime. As a result, the patient experienced sleeping problems, low mood, no desire to perform activities, and a passive death wish.\nIn 2014, the patient again visited the neurological outpatient department. Anamnesis revealed progressive difficulties with speech and swallowing as well as falls due to balance disorder since 2009. On neurological examination, we saw a patient with a surprised look. Dysarthria was present, as well as a vertical upward supranuclear gaze palsy and slowing of downward vertical saccades. There was a prominent postural instability and a positive pull test. Snout reflexes were present. A left-sided striatal toe was seen. Rigidity and bradykinesia were present in legs more than arms. The applause sign was positive. Neither apraxia nor cortical sensory deficit was found, nor was there dysmetria or evidence of autonomic failure. Together with evidence of mesencephalon atrophy on MRI of the cerebrum and results on cognitive testing (described further), we concluded that the patient fulfilled the National Institute for Neurological Disorders and Stroke and Society for PSP criteria (NINDS-SPSP) criteria for probable PSPS. |
A 21-year-old Nepalese man presented to the emergency department with a 2-day history of abdominal pain and repeated vomiting. The initial vital signs and the clinical examination were unremarkable except for diffuse vague tenderness in the abdomen. Laboratory studies (summarized in Table ) revealed renal failure and high anion gap metabolic acidosis. An urgent CT abdomen revealed mild perirenal fat stranding. Urine microscopy was unremarkable. On day 5 of the illness, clinical examination revealed bilateral facial nerve palsy. Subsequently, the patient developed bulbar symptoms as well as bilateral palsies of cranial nerves III, IV, and VI. He had distal neuropathic pains associated with distal lower limb weakness, diminished reflexes, and downgoing plantars. Brain CT scan was normal. Upon repeated questioning, the patient gave a history of ingestion of around 60 mL of furnace fuel. The high anion gap metabolic acidosis persisted and the patient went in anuria requiring hemodialysis (HD). Brain MRI done at this stage (nearly 2 weeks after ingestion) was essentially normal including magnetic resonance spectroscopy (MRS). Ten days after admission, a nerve conduction study (NCS) was performed, which showed predominately moderate axonal sensorimotor peripheral neuropathy (Table ). Subsequently, he developed aspiration pneumonia and was transferred to the medical intensive care unit (MICU) with a decrease in the level of consciousness. He was intubated and treated with intravenous antibiotics. An EEG revealed moderately severe slowing in the theta and delta ranges, but no epileptiform discharges. Eventually, the patient underwent tracheostomy and was weaned off the ventilator but remained HD dependent. He was fully conscious and cooperative but had residual facial paresis, dysphonia, and moderate weakness of all 4 limbs with bilateral foot drops as well as persistent areflexia.\nRepeat brain MRI (Figure ) after 3 months showed evidence of mild generalized brain atrophy (Table ). Repeat NCS showed bilaterally absent conduction in tibial, peroneal, and sural nerves. The upper limbs showed a predominately demyelinating sensorimotor neuropathy. Visual evoked potentials (VEPs) and brain stem auditory evoked potentials (BAEPs) were normal. |
An 86-year-old woman (2 gravida, 2 para), who was presented with an increased pelvic mass, was referred to our facility. She had undergone surgery to repair an umbilical hernia one year previously, and a large abdominal mass with broad calcification, measuring 16 × 13 × 13.1 cm (longitudinal × transverse × anteroposterior), was identified by plain computed tomography (CT) before the hernia repair surgery. The mass was consistent with a uterus containing calcified uterine fibroids (). The patient felt the increased pelvic mass and experienced gastric discomfort related to the stomach being compressed by the mass, resulting in complete appetite loss for the previous week. She was also presented with continuous uterine bleeding and urinary discomfort. The latest CT examination showed that the myomatous uterus significantly increased in size, measuring 27 × 16.5 × 17 cm (), and the calcified lesion was largely displaced (Figures and ).\nOn admission, her height was 150 cm and body weight was 60 kg. Her body temperature was 35.8°C, pulse rate was 81/min, and blood pressure was 88/40 mmHg. Although she was lucid, her Eastern Cooperative Oncology Group (ECOG) performance status was level 3, accompanied by disabling fatigue []. Her abdomen was distended by an irregular hard mass equivalent to 28 weeks' gestation. There was mild tenderness without rebound tenderness on the mass. On pelvic examination, the portio was normal and no vaginal bleeding was observed. Transabdominal ultrasound showed a large pelvic mass with peripheral calcification suggestive of uterine fibroids. Both ovaries were undetectable and minimal ascites was observed. Both kidneys were normal, with no dilatation of the renal pelvis and ureter. Plain CT images revealed that the uterine cervix was elongated and moved upward compared with its position one year before ().\nBlood tests showed an elevated white blood cell count (32,500/μL), low hemoglobin levels (9.9 g/dL), elevated C-reactive protein (16.4 mg/dL), and high serum creatinine (2.45 mg/dL). We initially suspected a malignant tumor arising from the uterus or ovary, such as uterine leiomyosarcoma, carcinosarcoma, or ovarian cancer, based on the patient's advanced age, abnormal uterine bleeding, rapidly growing abdominal mass, and ascites. Therefore, we performed cervical cytology, endometrial cytology, and a puncture of the pouch of Douglas to assess the cytology of the ascites. The results of all cytological examinations were negative for malignancy. Transvaginal ultrasonography revealed that the endometrium was thin; therefore, we did not perform an endometrial biopsy. On the second hospital day, the patient's vital signs were stable, but her ECOG performance status fell to level 4. She was completely confined to the bed and could not carry out any self-care tasks; this change in status was accompanied by delirium. Paralytic ileus developed and her bowel was decompressed with a gastric tube. A small amount of uterine bleeding was observed, and her urine volume gradually decreased. Her hemoglobin level decreased (8.5 g/dL), and her white blood cell count (37,800/μL) and serum creatinine levels (3.5 mg/dL) were elevated. On the third hospital day, she remained in a state of delirium. Hemorrhagic fluid was collected from the pouch of Douglas by a needle aspiration. The preoperative diagnosis was degeneration and infection of the large myomatous uterus; however, the relationship between the diagnosis and the deleterious change in her general condition remained unknown. During emergency surgery, the uterus was found to be rotated 360 degrees clockwise at the level of the isthmus (). The rotated uterus had suffered total infarction. A small amount of hemorrhagic fluid was present in the peritoneal cavity. A part of the small intestine was compressed by the uterus. We performed a total abdominal hysterectomy and bilateral salpingo-oophorectomy. The weight of the uterus was 3.8 kg. The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. Histopathology showed fibroids and extensive uterine and adnexal hemorrhagic infarcts consistent with uterine torsion. |
A 52-year-old female patient reported with a chief complaint of an enlarging but painless, gingival growth in relation to the lower front tooth region []. The growth was present for 2 years and was slowly increasing in size. There was no history of trauma and/or, surgery to the concerned region. The patient's medical and social history was noncontributory and she did not take any medications. Radiographic examination was unremarkable. On examination, the growth was polyploidal, nodular, sessile, firm, nontender with regular and nonindurated borders measuring around 2 cm × 0.7 cm in dimensions, and appeared to be arising from the free and attached gingival region of mandibular anterior teeth with evidence of mild tooth mobility. Routine laboratory investigations were found to be normal. Based on the medical history and clinical features, a provisional diagnosis of pyogenic granuloma was made. The clinical differential diagnosis was fibroma, peripheral giant cell lesion, and peripheral ossifying fibroma. The lesion [] was surgically excised with electrosurgical unit to minimize bleeding and postoperative discomfort [] and subjected to histopathological examination. Vestibuloplasty was also performed to correct inadequate vestibular depth []. Histopathological examination revealed a dense fibrovascular connective tissue with sheets of mixed inflammatory cell infiltrates comprising predominantly of plasma cells and few lymphocytes with an overlying parakeratinized stratified squamous epithelium with anastomosing rete pegs [Figures and ]. The tissue was, also, subjected to immunohistochemical analysis. Immunohistochemical examination revealed kappa and lambda light chain immunoglobulin markers [Figures and ] which are used to differentiate reactive from benign and malignant lesions as was confirmed in our case, with a positive reaction revealing the lesion to be reactive rather than being benign or, malignant in nature. Based on the above findings, a final diagnosis of PCG of gingiva was confirmed. The patient was re-called after every 3 months [] for 1 year for a follow-up. Follow-up examination revealed no clinical evidence of any possible recurrence. |
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