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A 32-year-old man was admitted to our hospital with appetite loss. He had a history of traumatic transverse cervical spinal cord injury at the C5 level due to suicide attempt at the age of 18. As a result of cervical spinal cord injury, he was paralyzed in the lower body. Contrast-enhanced computed tomography (CT) revealed a late-onset traumatic diaphragmatic hernia with strangulated ileum (Fig. ). The small intestine, transverse colon, and omentum were displaced into the left thoracic cavity, and some portions of these organs showed a decrease in blood flow. Left lung collapse and a compressed right lung with mediastinal shift were evident. The patient underwent emergency surgery. After replacing the incarcerated organs to their original positions, scattered areas of necrosis were identified in the small intestine, transverse colon, and omentum (Fig. ). By using interrupted sutures with non-absorbable 1–0 monofilament, the diaphragmatic orifice was closed. Wedge resection with primary closure was performed for the colonic necrosis in two places. Partial resection, 45 cm long, with end-to-end anastomosis was performed for the small intestine. The necrotic omentum was removed (Fig. ). In addition, a gastrostomy tube was placed since delayed initiation of oral intake was expected. The patient developed severe septic shock postoperatively. Treatment-resistant critical hypotension with non-compensatory tachycardia developed, likely due to parasympathetic nervous system damage related to the cervical spinal cord injury. On postoperative days (POD) 3 and 6, cardiac arrest occurred. Fortunately, he was rescued by cardiopulmonary resuscitation with administration of large doses of vasopressin and catecholamine. However, peripheral vasoconstriction, increased intra-abdominal pressure, and ischemia of the gastrointestinal tract developed, which resulted in colonic anastomotic leakage with diffuse peritonitis, abdominal wound dehiscence, and collapse of gastrostomy on POD 6 (Fig. ). The patient was unable to undergo surgical repair because of his poor general condition with continuing severe septic and neurogenic shock. Therefore, he underwent AVS through the open abdominal wound and it was the first procedure at the intensive care unit. The procedure of AVS was as follows: 1. the open wound and peritoneal cavity were rinsed with normal saline and necrotic and/or contaminated tissues were debrided (Fig. ); 2. wound dressing materials (DUOACTIVE® ConvaTec, New Jersey, USA) for protecting healthy skin around the open wound were patched along the abdominal wound in piecemeal fashion so as to adjust dressing materials to the complicated shape of the wound (Fig. ); 3. two drainage tubes with multiple side holes, up to 30 cm from the tip, were placed in the abdominal cavity through the open abdomen and the enteric contents were suctioned through the drainage tubes using a Continuous Suction Unit MERA Sacuum (Senko Medical Instrument Manufacturing CO, Tokyo, Japan) set to 50–75 mmHg continuous negative pressure; and 4) the entire wound was filled with saline-moistened gauzes and covered with polyurethane drape (Fig. ). The colonic anastomotic leakage showed gradual healing over the course of 2 months, followed by contraction and closure of wound dehiscence (Fig. ). Because the gastric fistula remained, a gastrostomy balloon catheter was placed through the gastric fistula. The patient resumed oral intake on POD 112 and left the hospital on POD 190 with the gastrostomy balloon catheter and without incisional hernia. |
A 77-year-old right-handed man undergoing ipilimumab treatment for recurrent metastatic melanoma presented with multiple falls that started three days after his third ipilimumab infusion. The symptoms began with hoarseness of voice, right arm weakness, and tingling in the bilateral hands which progressed over the next two weeks to near complete loss of movement and sensation in all four extremities. The patient had no bowel or bladder incontinence. He also denied any fever, sick contacts, recent travel, insect bites, or gastrointestinal symptoms.\nOn exam, his mental status and all cranial nerves were intact except for significant hoarseness and hypophonia. The tone was flaccid in his distal extremities and decreased proximally but no atrophy was present. Proximal strength was reduced overall in all extremities except during left shoulder abduction, left elbow flexion, and left elbow extension. Sensation was decreased in all extremities (distal > proximal). Reflexes were also absent in all extremities. Toes were mute to plantar stimulation bilaterally.\nA magnetic resonance imaging (MRI) scan of the entire spine with and without contrast was negative for metastatic disease, cord compression, cord edema, or other etiology. The MRI brain scan with and without contrast was also negative for malignancy or any other acute pathology. The first electromyogram (EMG) of the right upper and lower extremity is shown in Figure which was done seven days after the onset of the symptoms (ten days after his last ipilimumab dose). The EMG demonstrated no evidence of definite myopathy. In the right upper extremity, there was evidence of chronic neurogenic changes as well as decreased recruitment of all muscles tested. In the right lower extremity, there was a concern for polyneuropathy or polyradiculopathy. Repeat EMG shown in Figure was done 21 days after the onset of the symptoms and 24 days after his last ipilimumab/nivolumab dose. It demonstrated electrophysiologic evidence consistent with AIDP with secondary axonal features.\nThe cerebrospinal fluid (CSF) analysis showed elevated immunoglobulin G (IgG). The serum paraneoplastic panel was negative except for high titers of striatal antibody. Other pertinent serum findings were elevated glutamic acid decarboxylase (GAD65), negative IgG and immunoglobulin M (IgM) for Lyme disease, elevated erythrocyte sedimentation rate (ESR), normal C-reactive protein (CRP), normal creatine kinase (CK) and elevated myoglobin. Because of hoarseness in his voice, there was a concern for bulbar dysfunction which was evaluated by speech pathologists and ear, nose, and throat (ENT) service. Swallow evaluation and modified barium swallow were also performed. They demonstrated moderate to severe oropharyngeal dysphagia complicated by trace penetration and silent aspiration with all liquid consistencies. The ENT service also conducted a bedside flexible fiberoptic nasolaryngoscopy which demonstrated reduced motor and sensory function of the larynx, poor glottic closure, and right vocal cord weakness. |
A 62-year-old man with a past history of severe fibrotic lung disease requiring long-term oxygen therapy was hospitalised with typical angina pain with no resolution despite sublingual glyceryl trinitrate use. ECG demonstrated dynamic anterolateral ST depression with T wave inversion in anterior and lateral leads (). High-sensitivity troponin I assays (normal: < 5 ng/L) measured 99 and 250 ng/L. Transthoracic echocardiography revealed a left ventricle ejection fraction of 52% and anterolateral hypokinesis (video ) with a high likelihood of pulmonary hypertension (estimated systolic PA pressure 110 mmHg), indicating likely group III pulmonary hypertension (secondary to hypoxic lung disease). The main PA diameter was measured at 5.0 cm (). Cardiac catheterisation assessment as part of a lung transplant work-up 7 years earlier had shown no significant coronary disease () with invasive measurements demonstrating only mild pulmonary hypertension (mean systolic PA pressure 30 mmHg). The differential diagnosis included pulmonary thromboembolism and pulmonary/aortic dissection, although the clinical picture was most consistent with acute MI. The patient was commenced on pharmacological treatment for non-ST elevation myocardial infarction. In view of the significant lung disease, an initial conservative approach was taken, but after continual chest pain despite optimal medical therapy, invasive coronary angiography was necessitated. The transradial coronary angiogram showed a tight narrowing of the LMCA ostium (). The smooth tapering appearance () raised the possibility of external compression. This was subsequently confirmed with the use of intravascular ultrasound (IVUS) which showed dynamic external compression of LMCA with a slit-like lumen and absence of atheroma (, video )—suggesting that the patient had developed a type 2 NSTEMI due to demand ischaemia. A gated CT scan confirmed normal origin and course of the LMCA; however, it was compressed by a grossly dilated PA measuring 58 mm (normal ~30 mm) at its maximal diameter (), compared to 33 mm 7 years ago ().\nThe patient was not felt to be a suitable candidate for surgery and underwent IVUS-guided percutaneous coronary intervention with a 5 × 20 mm everolimus drug-eluting stent deployed directly without any predilatation. Postdeployment, IVUS confirmed a well-apposed stent (video ) with resolution of extrinsic compression and restoration of the LMCA lumen (Figures and ). The patient was rendered pain free and discharged home after a short period of observation and remained angina free on subsequent follow-up. |
A 74-year-old woman visited our station with squamous cell carcinoma (SCC) on the right buccal mucosa. Her past medical history included chronic obstructive airways disease, hypertension, and diabetes mellitus. The patient is a current smoker, with a history of 20 pack-years. Preoperative chest radiography, electrocardiogram, full blood count, and serum biochemistry were within the normal range. After being diagnosed with SCC as a result of incisional biopsy, the patient underwent the resection of SCC on the right buccal mucosa of the mandible, modified radical neck dissection, and primary reconstruction with a fibula-free flap. Tourniquet pressure was 300 mm/Hg, and its application time was 60 min. Total on-table time was approximately 7 h. Upon admission to the SICU after the 7-h operation, hypothermia and hypotension were noted. On the first postoperative day, the patient exhibited oliguria and proteinuria and elevation of CK, AST, ALT, and LDH. Together with the nephrology and neurology staff, we tried to figure out our patient’s symptoms and clinical findings. We thought that her clinical picture was based on an impression in which acute renal failure was diagnosed as secondary to rhabdomyolysis. Thus, she was managed with high-dose loop diuretic therapy. Additionally, we gave her hepatotonic to recover her liver function. The patient was supplemented with 150 to 250 mL/h of lactated Ringer’s solution and 0.9% NaCl. When the volume was full, urine output of above 100 mL/h was maintained by 20 mg intravenous injection with furosemide.\nHer urine output for the first hour is at 20 mL/hour, but after the medication, her urine output began to improve on day 4 with a corresponding reversal in the serum creatinine. After postoperative day 4, the muscular enzyme showed a downward trend. We treated the patient with medication and hydration, and then the result became favorable. In the end, she was able to recover fully from the symptoms. Figures and show the change in serum enzyme levels during hospitalization (Figs. and ). |
An 80-year-old woman presented with a 3-year history of localized swelling on the left medial eyebrow area and an aching, dysesthetic pain typically radiating to the left medial forehead, especially when the head was inclined. The dysesthetic pain developed in association with the development of the localized swelling on the medial eyebrow area and progressively worsened with time. The pain distributed along the cutaneous distribution of left supraorbital area to the frontal forehead. Eventually, she suffered from extreme difficulty and pain during daily life in tasks including eating, face washing, brushing, and reading because the pain was instantly aggravated with the face and head in dependent position.\nOn physical examination, there was no local heat or tenderness and no thrill was felt on palpation of the swollen area. No discoloration and no signs of inflammation were evident. The eye examination was normal. There was no neurologic deficit, except mild hypesthesia in the distribution of the left supraorbital nerve and a sensorineural hearing loss in the left ear. In the medical history, she underwent a gamma knife radiosurgery for a small, left-sided, vestibular schwannoma 5 years before presentation, which had been incidentally detected during an evaluation for left-sided hearing loss and tinnitus. She had been medicated for 10 years with calcium channel blocker for an essential hypertension. There was no history of exposure to antiplatelet or anticoagulation drugs and no history of physical trauma and shingles over the left supraorbital area. She denied discoloration or swelling presented in the left supraorbital area before the development of swelling and pain 3 years ago. Laboratory examination including the coagulation profile was normal.\nMagnetic resonance imaging (MRI) showed an approximately 21 mm × 18 mm-sized, irregularly-marginated, highly-enhancing subcutaneous mass in the left supraorbital area. Low signal intensity was produced on both T1 and T2-weighted images, and strong enhancement with gadolinium was evident [Figure and ]. There was no involvement in the adjacent left orbit. Considering the progressive growing nature and medical intractability, surgical resection was planned. On exposure through a supraorbital, eyebrow incision, poorly circumscribed, multiple vascular channels encircling the supraorbital and supratrochlear nerves were encountered. Dissection of the supraorbital and supratrochlear nerves from the vessels was impossible because the nerves were embedded within the vascular channels and profuse venous bleeding was observed. En bloc excision of the vascular channels including the involved nerves was performed. Histological examination showed lobulated and dilated, blood-filled vessels lined by CD31 positive endothelial cells [Figure and ]. No cellular atypia was observed. The pathological features were consistent with a diagnosis of formerly-called cavernous hemangioma (slow-flow VM).\nThe incident, dysesthetic supraorbital pain on inclining the head disappeared immediately after resection. However, a new denervation dysesthetic pain according to the supraorbital neurectomy developed and lasted 9 months postoperatively. The pain eventually subsided with gabapentin therapy. Moderate hypesthesia and mild dysesthesia in the distribution of the left supraorbital nerve was evident, and no recurrence was observed 2 years postoperatively. |
A 69-year-old asymptomatic female was found an abnormal shadow on a chest X-ray during a regular health check-up. Then she was referred to Shandong Provincial Hospital Affiliated to Shandong University for further investigation.\nShe described her history of hypertension, diabetes, and depression and taken oral drugs to control the blood pressure, blood sugar, and the depression symptoms. The patient denied history of smoking and lung disease and had no respiratory complaints or other physical symptoms. She received the enhanced chest computerized tomography (CT) scan that showed a tumor located in the left lingular lobe (Figs. and ). Given that trans-bronchial lung biopsy or bronchial alveolar lavage might lead to a false-negative result, the percutaneous lung biopsy was undertaken to diagnosis precisely, and the biopsy pathology showed sarcomatoid cancer. Some other preoperative evaluations were also conducted including physical examinations, cardiac, and pulmonary function test. In addition, CT scans of the brain and abdomen and bone scintigraphy were performed to rule out distant metastasis. According to all the preoperative examinations, the clinical stage was classified as Ib stage and there was no obvious contraindication. The basic bronchoscopies were performed preoperatively to confirm the extent of tumor invasion. Based on all the examinations, the video-assisted thoracic surgery left lingular lobe with mediastinal lymph node dissection was carried out. The resected specimen showed a peripheral tumor measuring 4.0 cm in maximum diameter. Microscopic images showed that it was comprised 50% spindle cells, 30% giant cells, and 20% adenocarcinoma cells (Fig. ). The final diagnosis was PC. Dissected hilar and mediastinal lymph nodes were free of metastatic disease. Postoperative adjuvant chemotherapy was not performed due to the patient's refusal and has been living cancer-free during the 12 months’ clinic visits after the surgery without any chief complaint. This study was approved by the Ethics Committee of Shandong Provincial Hospital Affiliated to Shandong University (Jinan, China). |
A 56-year-old male was admitted to our hospital for an increasing swelling mass at the root of his right neck (Figure ). He had an active smoking history, but no history of chest pain or trauma. Physical examination found a large pulsating mass on the right neck, without other significant clinical findings. Angio-CT scan revealed a large thrombosed aneurysm of 65 x 60 x 170 mm, originating from the innominate trunk; there was no involvement of the origin of the right carotid and subclavian arteries (Figure , Figure ). The electrocardiogram revealed a previous inferior wall myocardial infarction with negative T-waves in the inferior leads (Figure ). Transthoracic echocardiography showed severe depressed left ventricular function [ejection fraction (EF): 35%] (Figure ). Coronary angiography revealed atherosclerotic coronary disease with chronic occlusion of the right proximal coronary artery (RCA), severe stenosis of the main of the diagonal artery, and severe stenosis of the circumflex artery. Myocardial viability study confirmed the presence of viable myocardium in the lateral wall and the anterior wall with non-viable myocardium of the inferior wall. A CT angiogram scan of the cerebral arteries showed a permeable Willis polygon.\nThe decision was made to perform a simultaneous operation of the IAA resection and coronary artery bypass grafting (CABG).\nUnder general anesthesia, peripheral femoral arterial cannulation was performed along with the catheterization of the left carotid artery; a median sternotomy extending into the right side of the neck along with the medial edge of the sternocleidomastoid muscle was performed. Cardiopulmonary bypass (CPB) circuitry was completed with cannulation of the superior and inferior vena cava, and the patient was cooled to 25 °C. The myocardial arrest was achieved by cold crystalloid cardioplegia. The fragile part of the ascending aorta was clamped and resected over the coronary ostia at the level of the sinotubular junction (Figure ).\nA Dacron aortic graft was anastomosed proximally to the aortic sinotubular junction with external reinforcement by a Teflon strip. CPB flow was stopped. The IAA was completely resected from its origin in the arch to its distal bifurcation. The Dacron graft was inserted and the distal lateral ends ligated. The distal end of the graft was anastomosed to the proximal aortic arch and reinforced with external Teflon bands. The other end of the graft was anastomosed to the distal innominate artery (Figure ).\nUnilateral anterograde selective cerebral perfusion was instituted via a cerebral perfusion catheter placed into the left common carotid artery. Cerebral monitoring was achieved using transcutaneous cerebral oximetry.\nWe proceeded with the coronary bypass with retrograde cardioplegia, with a circulatory arrest time of 42 minutes. Resumption with reheating for the preparation for aortic unclamping was disastrous with myocardial fibrillation not recoverable after electric shock or with supplemental doses of cardiotonic drugs. Without the possibility of a heart transplant immediately, we declared the patient’s death. |
A 10 year old white girl with severe (class 3: BMI ≥140% of the 95th percentile for age and sex) obesity and otherwise normal development presented to the Pediatric Weight Management Clinic with her mother. The mother reported that the patient had been at the 75th percentile for height and weight for most of the patient's life but she experienced a “20 to 30 pound” weight gain over the past year. The mother further explained that this recent weight gain coincided with treatment of seasonal allergies with montelukast and she wondered if this may have been the cause of the weight increase. The patient had no prior weight loss attempts.\nThe patient was born full term, weighing 3.18 kg. The mother's pregnancy was uncomplicated, as was the patient's newborn course. Aside from seasonal allergies, the patient was healthy. She had no history of hospitalizations, surgeries, or mental health concerns. She was not taking any medications.\nThe patient was eating regularly-spaced meals consisting primarily of highly processed foods and simple carbohydrates (e.g., pastries for breakfast, potatoes with cheese for dinner). The family was eating fast food three times per week on average. The patient endorsed having a big appetite and feeling hungry all the time. She was eating while watching TV and when bored. She denied binge eating, loss of control eating, emotional eating, sneaking/hiding food, or eating during the night. Her physical activity was limited to gym class at school three times per week.\nThe patient was living with her mother and her mother's partner. The patient's parents divorced when she was very young and the mother's partner had been living with them since the patient was a toddler. The patient saw her biological father rarely. She had no siblings. She was attending fourth grade and enjoyed reading and writing. The mother and her partner worked full-time and the patient was cared for by a baby sitter after school a few times per week. They had no food insecurity. The family history was notable for obesity in both biological parents and type 2 diabetes in the maternal grandmother.\nThe patient's review of systems was negative. She reached menarche several months prior to presentation. On physical examination, her weight was 70.31 kg (155 lbs.), height was 142 cm (4'8”), and BMI was 34 kg/m2 (145% of the 95th percentile). Her blood pressure was 105/65 mmHg and pulse was 74 beats per minute. Her physical examination was normal. The results of her fasting labs were: total cholesterol 176 mg/dL (normal: < 170 mg/dL), HDL-c 49 mg/dL (>45 mg/dL), LDL-c 96 mg/dL (< 110 mg/dL), triglycerides 157 mg/dL (< 90 mg/dL), ALT 27 (< 50 U/L), AST 29 (< 50 U/L), glucose 98 mg/dL (70-99 mg/dL), and HbA1c 5.5% (0-5.6%). Her Pediatric Symptom Checklist (routinely obtained in the Pediatric Weight Management Clinic) score was 8 (> 28 is considered abnormal).\nThe patient and family were started on a program of lifestyle modification therapy and responded particularly well with decreasing fast food consumption and liquid calories. Further, the patient started bringing her lunch to school instead of eating the school fare and was able to keep a food log almost daily. The patient's physical activity, however, continued to be limited. Over the course of 5 months, the patient's BMI decreased 5 units (15%), from 34 kg/m2 to 29 kg/m2 (145% of the 95th percentile to 125% of the 95th percentile).\nAt the end of the 5 month period, coinciding with the end of the school year and beginning of summer vacation, the patient's sleep/wake cycle became irregular. Because she did not like the hot weather, she chose to stay inside all day. Her mother left prepared meals for the patient to encourage healthy eating while mom was at work. Despite this, the patient's BMI began to trend upward from 29 kg/m2 to 31 kg/m2 over the summer months. Upon school resuming in the fall, the patient's sleep/wake cycle normalized and eating behaviors and patterns improved, returning to those of the previous school year. The patient's BMI stabilized for a few months but then increased further. The patient expressed frustration because she believed that she was eating well, which was indeed reflected in her daily food logs. She continued to attend monthly visits with the Pediatric Weight Management Clinic dietician, psychologist, and pediatrician with specialized training in obesity medicine. Yet, the patient's BMI continued to increase such that by 2 years after her initial appointment, the patient's BMI returned to baseline (135% of the 95th percentile) (see Figure ).\nSuspecting that metabolic adaptation was causing the patient's weight rebound, adjunct pharmacotherapy was recommended. Orlistat was considered but not started because of concern about gastrointestinal side effects and lack of insurance coverage. Metformin may have been another reasonable option but the patient's fasting glucose and HbA1c were in the normal range and she did not have acanthosis nigricans on physical examination which would have suggested insulin resistance. She was ultimately started on topiramate 75 mg daily in addition to ongoing LSMT. She and her mother were cautioned that although topiramate is not FDA-approved for the indication of obesity (in children or adults), multiple studies have demonstrated clinically-meaningful weight loss efficacy in adults. Additionally, it was explained that the side effect profile in children is well established stemming from its use for epilepsy treatment.\nAfter 4 months of treatment with topiramate, the patient's BMI trajectory plateaued yet was not decreasing as was desired. Recognizing that the combination of topiramate and phentermine is the most effective weight loss medication currently available for adult obesity, phentermine 15 mg daily was added to the topiramate 75 mg daily. The patient and mother were informed that phentermine is FDA-approved only for individuals older than 16 years and for “short-term use.” With combination treatment for ~22 months, the patient experienced good BMI reduction, from 34.1 to 25.7 kg/m2. Her blood pressure and heart rate were monitored regularly and though her blood pressure did not increase, her heart rate increased slightly from 60 to 70 s, in line with the mechanisms of action of phentermine (stimulant-like effects). Later, the patient reported that she was experiencing some “memory” issues but noted no change in her academic performance. Although it seemed unusual for this type of symptom to emerge 10 months after starting topiramate, the topiramate dose was decreased from 75 to 50 mg daily and the memory issues resolved. Written informed consent was obtained from the parent of the patient for the publication of this case report. |
A 50-year-old right-handed female was admitted with a 4-year history of recurrent acute onset of neurological deficits. The first attack came up in 2009. She suddenly developed difficulty speaking and referred to a local hospital. Brain magnetic resonance imaging (MRI) demonstrated diffusion-weighted imaging (DWI) hyperintensity in the cortex of left frontal lobe (Fig. A). No stenosis of hemodynamical significance was found on brain and neck computed tomography angiography (CTA). Acute cerebral infarction was considered. Aspirin was given but discontinued after a short period of time. The second and third ischemic events followed in 2010 and 2011, respectively, presenting with similar motor and speech deficits. Multiple lesions of fluid-attenuated inversion recovery sequence image (FLAIR) hyperintensities were revealed in the left frontal and parietal lobes and right temporal lobe (Fig. A), attributed to the previous several attacks of ischemia. During that time, antiplatelet agent was intermittently used. The latest episode of symptomatic stroke was in May 2013, with sudden onset of expressive dysphasia and right hemiplegia. DWI revealed massive restricted diffusion in the left frontal, parietal, and temporal lobes. Antiplatelet therapy and rehabilitation were given and her symptoms got partial recovery. Brain magnetic resonance angiography disclosed that left anterior cerebral artery and bilateral posterior cerebral artery originated from the communicating arteries (not shown). The patient was then admitted to our hospital for identifying the cause of recurrent stroke.\nShe had varicose veins in lower extremities for 30 years. She had no history of hypertension, ischemic heart disease, diabetes or hyperlipidemia and had no relevant family history.\nOn examination, heart rate was 60 beats per minute, blood oxygen saturation was 97% on room air and the respiratory rate was 16 breaths per minute. Neurological examination revealed combined aphasia. Left Babinski sign was present. Cardiovascular examination was unremarkable.\nBlood tests including hemoglobin, liver function, cholesterol, homocysteine, and arterial blood gas were within normal ranges. Multiple fresh silent infarcts were demonstrated on brain MRI shortly after admission (not shown).\nThrombophilia screen (proteins C and S, antithrombin, activated protein C resistance assay, factor V Leiden, prothrombin and lupus anticoagulant, antinuclear, and anticardiolipin antibodies) was normal.\nElectrocardiogram (ECG) revealed a normal sinus rhythm. Atrial fibrillation was further ruled out at a normal 24-hour Holter ECG. No evidence of patent foramen ovale or other possible cardioembolic phenomena was found on transthoracic and transesophageal echocardiogram (TEE). Aortic CTA was unremarkable. Nonetheless, contrast transcranial Doppler ultrasound (TCD) detected a typical “curtain” appearance of microbubbles, indicative of a right-to-left shunt (Fig. B).\nComputed tomography pulmonary angiogram (CTPA) was then initiated and a pulmonary arteriovenous malformation (PAVM) in the left lower lobe was found (Fig. C). The left PAVM was then confirmed using catheter angiography. Screening Doppler ultrasound of systemic venous system revealed calf muscular venous thrombosis of the right lower limb. The patient underwent percutaneous closure of PAVM. Afterward, warfarin was commenced because of the high risk of further thromboembolic complications in the following weeks to months. Post-treatment CTPA demonstrated successful closure of PAVM. No microbubble signals were detected on post-treatment contrast TCD study (Fig. D). Warfarin was maintained with INR between 1.8 and 2.5. The patient suffered no further embolic events during 3-year follow-up. No recanalization or new PAVMs were detected on CT scan. |
A 60-year-old female was referred to us for the left lateral access mandibulotomy for an open biopsy to a left infratemporal lesion found on CT scan in June 2014. She had a history of toothache at a left mandibular molar tooth which was extracted approximately a year earlier. The patient subsequently developed hypoesthesia 2-week postextraction at the left angle of mandible to the neck. She previously underwent transoral alveolar bone biopsy in a nearby hospital of which the report came back as osteomyelitis and she was treated with multiple long duration of antibiotics. Her condition worsened further with progressive trismus and left otalgia. A left mastoid exploration was performed in February 2014 under the ear, nose, and throat department. She developed House–Brackmann Classification V left facial nerve palsy complication from the surgery.\nOn examination, she had left facial asymmetry. She was unable to close her left eye fully but no conjunctival ulcer present. She had left lower lip hypoesthesia and drooling. Her mouth opening was 2 cm measured intrinsically. Her gag reflex was positive. There was no mandibular bowing at the angle region, no shallowing of buccal sulcus or tenderness bilaterally at the mandible. The mucosa at the left mandibular posterior alveolar ridge was intact.\nThe panoramic radiograph [] showed multiple root treated teeth with complex crown and bridgework. There was ill-defined, enlarged radiolucency along the left mandibular canal measuring about 7.7 mm.\nMeanwhile, the CT scan showed hyperintense lesion over the left infratemporal fossa mass involving the pterygoid muscle and deep lobe of the left parotid gland. There was widening of the mandibular canal region extending to the mental foramen. There was cortical irregularity with periosteal reaction of the medial aspect of the left mandible [].\nThe MRI showed ill-defined heterogeneous enhancing mass in the left infratemporal fossa. The mass extended down to the left angle of mandible with extension along the mandibular canal [].\nBoth alveolar cortical bone and the left infratemporal lesion were concurrently biopsied and reported as differentiated squamous cell carcinoma. She subsequently underwent left hemimandibulectomy and superficial parotidectomy. She, however, finally succumbed to her disease. |
An 80-year-old man with known cervical canal stenosis due to ossification of the cervical posterior longitudinal ligament was transferred to an emergency room soon after a falling accident. The patient was alert and had complete tetraplegia, sensory deficits below the C4 cervical sensory level, and progressive restrictive respiratory failure that required assisted ventilation, and, eventually, a tracheostomy. Cervical spine magnetic resonance imaging showed cervical cord compression that was worse at the C3–C4 intervertebral level, and an intramedullary high signal at C3 and C4 vertebral levels, and the patient was managed conservatively. During the conventional weaning process of trials of intermittent spontaneous breathing in the ICU, an unexpected cardiac arrest made both the patient and medical staff reluctant to proceed with further weaning. Fifty-eight days after cervical cord injury, the patient was transferred to our hospital for further rehabilitation. He was fully conscious and received pressure-support mechanical ventilation with back-up rates of 16 breaths. He had total tetraplegia below shoulder girdle muscles with a preserved sensation from C2 to C3 on both sides, and a urinary obstruction. The limbs and trunk were stiff with contracture, and muscle stretch reflexes of the limbs were abolished. Neurological level of injury was C3 according to the ISNCSCI, with a total sensory score of four. There was no sensory sacral sparing, while the deep anal pressure was preserved, and the American Spinal Injury Association scale graded B. Chest roentgenogram and computerized tomography showed bilateral, widespread atelectasis. The patient occasionally showed signs of pulmonary infection. Intensive pulmonary care and rehabilitation, including physical support for expectoration, mechanically assisted removal of tracheal secretions (CoughAssist E70®, Philips Japan, Tokyo), ventilator muscle training, and management of the spasticity of the abdominal wall, resulted in the improvement of the roentgenological findings. His expiratory tidal volume (VT) measured 150 mL.\nAlthough the neurological status according to the ISNCSCI was unchanged, the patient was willing to be weaned from mechanical ventilation as his general condition improved. After informed consent was given, we attempted automated weaning from mechanical ventilation using IntelliVent®-ASV on and after 131 days of injury. IntelliVent®-ASV (Hamilton Medical AG, Switzerland) is a closed-loop ventilation mode that adjusts the pressure support in terms of the percentage of the ideal minute volume (%MV). The ideal MV (100%MV) is calculated from the patient’s height and gender. Based on Otis’ equation [], adaptive support ventilation will select the best VT–respiratory rate (RR) coupling for the optimal work of breathing. IntelliVent®-ASV has interrelated functions: an auto-adjustment for carbon dioxide (CO2) elimination, an auto-adjustment for oxygenation, an auto-weaning tool named Quick Wean (QW), and a spontaneous breathing trial (SBT). The setting of the target %MV is automated based on either the monitored end-tidal CO2 or monitored spontaneous breathing rate. The auto adjustment of fraction of inspiratory oxygen or positive end-expiratory pressure is based on the monitored oxygen saturation fraction of hemoglobin with a pulse oximeter (SpO2) []. The QW mode is an optional automated weaning, and progressively reduces the pressure support, monitors for readiness-to-wean criteria, and provides the option to automatically conduct a fully controlled SBT. In the QW mode, when the spontaneous rate is less than the upper limit of the predicted target range, %MV is automatically decreased gradually to the selected level. To facilitate the recovery of VT by means of the loading work on respiratory muscles, we set the level of support to 70%MV for the QW mode. When spontaneous breathing satisfied the pre-determined conditions of oxygenation and ventilation for 1 min, the SBT mode was used. We operated the SBT mode during the day from 9:00 a.m. to 6:00 p.m.\nIn the QW mode, the duration of 70%MV and SBT time (25%MV) increased gradually (Figs. and ). Twenty-two days after the introduction of the QW and SBT modes in association with intensive pulmonary rehabilitation, SBT was running almost fully during the daytime. At this time, the expiratory VT was increased to 350 mL from 150 mL upon admission to our hospital. Thereafter, we withdrew the pressure support ventilation during the nighttime 39 days after the introduction of the auto-weaning mode, and the patient’s weaning process was completed. |
A 59-year-old man presented to the urgent care clinic at the School of Dentistry complaining of an upper lip mass for one-year duration. The mass started out as a small bump and had grown steadily since then. 3 months prior to his presentation, a draining parulis developed on the mass. The patient had no history of systemic symptoms such as fever, chills, weight loss, or fatigue. He was aware of a dark-colored “dead tooth” for several decades in the area of concern but denied any previous history of swelling in the area. The patient had recently moved to the United States from Nigeria and had previously been without access to adequate dental care. The patient said that a doctor in Nigeria told him that the lesion was likely cancerous.\nOn exam, there was a large, painless, fibrous, exophytic mass in the anterior maxillary labial vestibule (Figures and ). The base of the mass approximated the apex of tooth #8. A yellow purulent material was observed draining from the parulis (). Tooth #8 was discolored and was confirmed to be nonvital on pulp testing. There was a significant gap between teeth #7 and 8. Tooth #8 was displaced medially and was extruded relative to the adjacent dentition.\nA periapical radiograph revealed a large unilocular radiolucency associated with the apex of tooth #8 (). Cone-beam computed tomography again demonstrated a large cystic-appearing defect in the anterior maxilla with perforation of the buccal and palatal cortices . The lesion extended to the nasal floor on the ipsilateral side.\nThe patient was referred to the oral surgery department for excisional biopsy. After tooth #8 was removed, an incision was made around the base of the stalk that connected the mass to the labial and alveolar mucosa. Sharp dissection was used to free the mass, and the specimen was sent for histopathologic analysis. The mass communicated with a cystic lesion of the maxilla. The cyst was enucleated with a curette and also sent for pathology. Perforation of the cyst through the buccal and palatal cortices was noted during the procedure. Slight undermining of the wound margins allowed for closure with resorbable sutures.\nAt the patient's one-week follow-up (), he was doing very well. He reported minimal pain, no neurosensory disturbances, and no systemic or local symptoms of infection. He and his family were very relieved to learn that the lesion was benign. He was happy with his appearance after having the mass removed. |
A 24-year-old male patient was presented to the outpatient department with a history of right lateral knee snapping and recurrent sensation of discomfort for the past 2 years. Snapping was elicited upon extending the knee and could be reproduced by applyng direct pressure on the posterolateral knee. There was no actual or previously sustained trauma noted on this patient.\nThe patient was initially and preliminarily diagnosed with knee joint plica syndrome and underwent arthroscopic surgery in a previous institution. This however did not relieve his symptoms. As the snapping continued, the patient became unable to tolerate physical activities or prolonged walking.\nUpon thorough physical examination, full active range of motion was observed to be intact, but a reproducible audible and palpable snapping of the lateral knee when moving from flexion to extension. However, this was not consistently reproducible with passive range of motion. (see Additional file ) Other special tests were negative except for the Cabot sign, which clearly produced a snapping sensation.\nX-rays presented a round osseous structure in the posterolateral part of the joint, similar to the normal sesamoid (Fig. ). CT scanning and MRI were additionally conducted. No pathological results were obtained apart from a clearly visible ovoid-shaped bone that was located posterior and superior to the proximal musculo-tendinous intersection of the popliteus muscle. The sesamoid bone articulates non-cartilagenously with the lateral dorsal femoral condyle (Figs. and ). The popliteus muscle and tendon presented signs of inflammation. A steroid injection was administered into the snapping point but did not relieve his symptoms.\nA posterior approach was used to incise the bone which lead us to discover that the biceps femoris tendon presented no pathological changes. Deeper in the popliteus tendon, a large cyamella was found. With the knee in a passive range of motion, we found there was snapping of the popliteus tendon over the cyamella. (see Additional file ) An incision was made directly over the located area and the sesamoid bone was excised (see Additional file ). The cyamella located near the musculo-tendinous intersection of the popliteus muscle (Fig. ). The specimen measured 15*7*9 mm (Fig. ). Radiographs following the procedure demonstrated removal of the cyamella (Fig. ).\nPostoperatively, the patient recovered well and had immediate relief after treatment. He was able to return to physical fitness activities at 8-week follow-up. |
A 28-year-old female with insignificant past medico surgical history presented with one day of acute onset pain in the periumbilical region that later migrated and confined to the RIF. She had associated intermittent fever, nausea, and loss of appetite. She did not have any urinary symptoms, bowel irregularities, or gynecological complaints. Abdominal examination was performed by two senior surgeons at two different occasions; the same day had findings of guarding and rebound tenderness at RIF. Hematological tests showed polymorphonuclear leukocytosis with left shift. Biochemical tests and urinalysis were normal. Urinary pregnancy test was negative. Abdominal radiographs were unremarkable. USG could not visualize appendix and was inconclusive except for probe tenderness in RIF. CT scan of the abdomen could not be done due to unavailability. A clinical diagnosis of acute appendicitis was made assigning an Alvarado score of 9/10. Laparotomy was performed using the Lanz incision in RIF. Intraoperatively appendix was found to be normal without evidence of inflammation or infection in RIF. In view of symptoms and signs, a possibility of other pathology was thought. Walking the bowel proximally up to 3 feet (1 m) did not show a Meckel's diverticulum or any other small bowel lesions. There were no obvious mesenteric lymph nodal enlargement and pelvic organs looked pristine. Approaching closure, just when the medial edge of the incision was retracted superomedially, a hemorrhagic lesion seemed to appear little deeper in the mid abdomen. Therefore, the incision was extended transversely from the medial edge to explore further. Entire bowel was explored and this revealed an ulcerated lesion measuring 7 × 5 cm arising from the antimesenteric border of the ileum 8 feet (2.5 m) from ICJ with localized interloop hemoperitoneum and inflammatory exudates as shown in . Resection of ileal segment containing the lesion was performed followed by restoration of bowel continuity and peritoneal toileting. The lesion was subsequently reported to be an ulcerated malignant ileal GIST.\nHistopathologically, gross examination confirmed the operative findings, and the cut section revealed a nodular lesion protruding out of the serosal surface measuring 7 × 5 cm along with 2 lymph nodes each measuring 2 × 1 cm.\nMicroscopically, the growth from the ileum had villous lining epithelium with focal ulceration. The submucosal region had a circumscribed nodule with proliferation of loosely cohesive spindle cells; some of which were arranged in vague storiform pattern and others in long fascicles. There were areas with epitheloid cells forming small anastomosing nests and cords. The areas in between these showed skenoid fibers along with focal areas of hemorrhage, infarction, and congestion as shown in . The mitotic figures were seen (8/50 high-power field). The lymph nodes were microscopically identified to be reactive, and the resected margins of the ileum were free of tumor.\nBased on tumor size and mitotic activity, possibility of a malignant GIST was suggested along with immunohistochemical analysis (CD117 and CD34) for further confirmation. The patient had an uneventful recovery and was discharged on the 8th postoperative day. She was advised to review a week later at the outpatients but failed to report. All possible contacts were used to trace her, but she remained inaccessible and lost to follow-up. |
In May 2016, a 30-year-old woman was addressed to our department for multiple inflammatory collections on the face 18 days following HA injections by her dermatologist. She was in good health and not known for allergy. Five years previously, she had received HA injection in the chin. To improve the facial contour, she received HA injections: 0,6 ml of Voluma® (Allergan) and 0.8 ml of Volift® (Allergan) in the cheeks, mandible and chin. She also presented a sore throat the following day and received amoxicillin treatment. Treatment was switched to clarithromycin and ciprofloxacin at day 4 due to the occurrence of multiple small pustules over the entire body. At day 10, the patient presented erythema and swelling of the white inferior lip and the chin. The patient received a prednisone regimen of 60 mg/day for 5 days with temporary improvement. At day 16, the patient presented erythema and swelling at different injection sites. An echography showed subcutaneous collections at the chin. A puncture showed the presence of pus and a sample was sent for bacterial analysis and culture. The patient was referred to our department at day 18 after HA injection. She presented with erythema, swelling and painful palpable subcutaneous collections at the chin, over the right cheekbone and the left angle of the mandible, without fever. The lower white lip was indurated and painful. The white blood cell count was 12.9 G/l and C-reactive protein was 15.2 mg/l. Magnetic resonance imaging confirmed subcutaneous facial collections in different localisations (). The patient was hospitalised and incision and drainage were performed under general anaesthesia that showed a greyish viscous discharge with coagulated blood, mainly in the chin (∼4 ml) and over the right cheekbone (∼1 ml). Bedside drainage and antiseptic rinsing were continued twice daily and then daily until necessary for 10 days. The patient was treated with intravenous piperacillin tazobactam for 6 days post-operatively. Due to the occurrence of multiple pustules over the entire body, the antibiotic therapy was switched to oral ciprofloxacin and clindamycin for one month. The clinical and biological evolution was favourable and the patient went home after 9 days of hospitalisation. Bacterial analysis and culture were negative. She presented an early recurrence of the subcutaneous collection at the chin, necessitating drainage and antiseptic rinsing for one additional week. The patient presented a chronic palmoplantar pustolosis 10 days after the last antibiotic treatment, which was finally diagnosed as a psoriasis variant and treated with ultraviolet therapy. At three months, she did not present any aesthetic sequelae, but the lower white lip was still indurated at palpation. An acute generalised exanthematous pustulosis hypersensitivity reaction to aminopenicillins or HA was suspected, but both were negative following patch skin and intradermal testing. In vitro lymphoproliferation test was not interpretable due to a non-specific immunological reaction against HA. |
A 69 year-old female, having arterial hypertension since 5 years as medical history, with a sedentary lifestyle, obesity (BMI: 30.5 kg/m2) and abdominal obesity, with no drug nor family history. She was admitted with symptoms of sudden pain and paresthesia of the left upper limb, started 6 hours before her consultation. During the previous 20 days, she had a progressive dyspnea (class III of the New York Heart Association's classification) with productive cough, causing important limitation of physical activity, for what only symptomatic treatment was taken; this dyspnea had quickly evolved to a class IV of the NYHA's classification, during the last 3 days before her admission. Her family history was negative for any thromboembolic disorders and she denied any history of taking contraceptive drugs.\nThe physical examination find polypnoea with 24 cycle/min of respiratory rate, and a SatO2 = 91%, blood pressure at 130/90 mm Hg, heart rate at 115 beats per minute (bpm). Cardiopulmonary examination was unremarkable, and extremity examination did not demonstrate any edema, however, a lack of pulse and sensibility with pallor on a cold left upper limb was noticed. The rest of the examination was unremarkable.\nThe electrocardiogram showed sinus rhythm at 115 bpm, with a right bundle branch block and a negative T wave in V1,V2.\nSince the findings on examination of the left upper limb were suggestive of arterial ischemia, an upper limbs angio-CT was performed. The primary interpretations showed a significant arterial thrombosis of the left humeral artery extended to the radial and ulnar arteries. The patient was directly taken to the operating room, where a trans-humeral embolectomy was performed successfully by a junior resident with 4 years of specialised training (using local anesthesia). The intervention adherence and tolerability were well during the whole course of his treatment. Moreover, no other interventions were performed.\nOtherwise, the final interpretation of the angio-CT showed in addition to the arterial thrombosis of the left humeral artery, a massive bilateral Pulmonary Embolism (PE) with a thrombus straddling the two main branches (see ).\nLower limb compression ultrasonography showed deep venous thrombosis (DVT) in the right popliteal artery.\nClinical and radiological data gave the suspected diagnosis of paradoxical embolism through a PFO, so a transthoracic echocardiography was performed.\nThe transthoracic echocardiography showed a dilated, non-hypertrophied right ventricle with a mild systolic dysfunction, an Interatrial septal aneurysm (of 10 mm) and a tricuspid regurgitation allowing calculating the systolic pulmonary pressure: SPP: 67 + 5 mmHg.\nThe TTE did not detect the PFO, so we completed by a trans-esophageal echocardiography (TEE) with a bubble test, which showed an Interatrial septal aneurysm and, at an angle for visualization around 50°, a PFO with a spontaneous positive bubble test (without the Valsalva maneuver) (see , ).\nThe diagnosis of right popliteal DVT and massive PE was proved, with paradoxical embolism in the right upper limb due to a PFO. A medical treatment with enoxaparin 1 mg/kg every 12h was early established, relayed then with direct oral anticoagulants (DOA):Rivaroxiban 20mg/Day.\nThere were no complications during the hospitalization with a clear clinical improvement. One week after discharge, Transthoracic echocardiography showed improvement of the right ventricular function and the SPP decreased to 40 + 5 mmHg. The electrocardiogram showed a regression of the right bundle branch block.\nThe patient underwent a thrombophilia screening: Factor V Leiden, antithrombin III, Protein C and Protein S, anticardiolipin immunoglobulin G/immunoglobulin M, and anti-dsDNA antibodies were all negative, but it showed a mutation of the Factor II.\nA lifetime anticoagulation with Rivaroxiban was indicated.\nAfter 2 years of follow-up, the patient had no more thrombotic events, with the persistence of PFO with a spontaneous positive bubble test at the TEE and a SPP at 29 + 3 mmHg. No hemorrhagic complications have been noticed during this two years. |
A 50-year-old female with end-stage renal disease (ESRD), secondary to amyloidosis on hemodialysis, presented to the emergency department (ED) with dyspnea for 4 days. Her medical history included cirrhosis secondary to HCV genotype 1a infection, two failed kidney transplants, and posttransplant diabetes mellitus. Her chest examination suggested the presence of a large right-sided pleural effusion.\nChest X-ray [] showed massive right pleural effusion. Computed tomography scan of the chest and abdomen revealed small ascites and splenomegaly but no evidence of parenchymal lung pathology. Her characteristics and laboratory details are shown in . A pigtail pleural catheter was inserted and the right lung fully re-expanded after intermittent drainage of the effusion. Pleural fluid analysis results were consistent with a transudate. Echocardiogram revealed normal left ventricular ejection fraction with mild left ventricular hypertrophy and diastolic dysfunction.\nAttempts at treating her effusion with frequent hemodialysis and ultrafiltration were unsuccessful as she had frequent and symptomatic hypotension. She was discharged home 9 days following the removal of the pleural catheter but returned to the hospital the following week with dyspnea. Chest X-ray showed reaccumulation of the right pleural effusion. Again, there was no evidence of intravascular fluid overload.\nShe was readmitted 3 weeks later with worsening right pleural effusion and severe dyspnea. A pleural catheter was reinserted, again with repeated drainage of the pleural fluid for the following 3 weeks.\nA 24-week course of ombitasvir, paritaprevir, and ritonavir plus dasabuvir was started and was well tolerated. HCV-RNA became negative 2 weeks after discharge. After 2 more weeks, she still had a moderately large effusion on X-ray and was mildly symptomatic. Her dyspnea progressively improved and resolved. Three months after DAA initiation, the right pleural effusion had diminished and completely resolved over the following 6 months []. She had no further hospital admissions or ED visits during the subsequent 9 months []. HCV-RNA quantification was 875,091 IU/ml before the initiation of antiviral therapy and became undetectable at 12 and 24 weeks' posttreatment confirming SVR. Her most recent laboratory values are shown in . |
A 36-year-old male, a seasoned cyclist with no past medical history, presents to the emergency department with complaints of lightheadedness and diaphoresis after a bicycle fall. Patient was participating in a bicycle race when another rider ahead of him fell causing the patient to swerve to avoid him. Patient states that he fell on his left side and hit a tree with his right leg. Patient was wearing a helmet and did not suffer any chest or head trauma. After the fall, he felt lightheaded and diaphoretic and complained of mid back pain. Patient denied any chest pains or shortness of breath. Patient was subsequently brought to the hospital directly following the accident by ambulance.\nIn the emergency department, patient was noted to be in no acute distress; initial blood pressure was 128/69 mmHg with pulse of 65 beats per minute. He was afebrile, not tachypneic, and well appearing with marked right thigh swelling and tenderness to his medial thigh. Given the dizziness and diaphoresis initially, patient had an ECG performed which showed lateral ST segment elevation () and had a subsequent troponin I that was positive, 0.49ng/mL, with a Creatine Phosphokinase (CPK) of 617 U/L.\nThere was initial concern for a possible cardiac contusion, although the patient had no chest wall trauma and thus was admitted for further evaluation. As an inpatient, an echocardiogram was performed demonstrating normal right and left ventricular function and trace pericardial effusion while the patients troponin continued to trend upwards towards a maximum of 21ng/mL. He was loaded with Aspirin and Clopidogrel as well as initiation of a heparin infusion, Lisinopril, and a Beta Blocker. Coronary angiography was subsequently performed demonstrating a spontaneous coronary artery dissection of left anterior descending coronary artery. No further diagnostic study was performed at that time. Further history revealed that he took multiple caffeine Jello shots and drank a large cup of coffee prior to participation in the race. He denied cocaine, amphetamine, or other performance enhancing drug use ().\nThe patient's CPK and troponin trended downwards on conservative medical management and his back pain resolved; therefore a stent was not placed. The patient was visiting from outside the area; discharge planning included repeat coronary angiography in 6 weeks and instructions that he will not be able to perform competitive cycling again. Should his dissection extend at that period of time or patient become symptomatic, stent placement would be considered. Patient was to continue the Aspirin and Clopidogrel until the repeat angiography was performed. Patient was discharged with plans to follow up with a cardiologist in his home state. Multiple follow-up phone calls made us unable to reach the patient and he was subsequently lost to follow-up. |
An 86-year-old female with a history of metastatic ovarian cancer presented to the ED with painful bilateral lower extremity edema and a left lateral leg ulceration. Her metastatic ovarian cancer had been diagnosed by malignant pleural effusion five months earlier, and she had completed neoadjuvant chemotherapy with carboplatin and Taxol approximately one week prior to this presentation. She was admitted to the hospital and started on cefazolin for left lower extremity cellulitis on hospital day one.\nOn admission, plain films and ultrasound did not reveal any evidence of osteomyelitis, fracture, DVT, or abscess to the left lower extremity. On exam, she had 3+ pitting edema below the knee bilaterally as well as chronic venous stasis changes. The patient also had a venous ulcer (approximately 2 cm in diameter) on the anterolateral aspect of the distal third of her left lower leg. At the time of admission, this venous ulcer had some serous weeping but no purulent drainage or fluctuance on examination. Her initial Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score was 4, suggesting a low risk for necrotizing fasciitis; however, on hospital day 3, her CRP began to uptrend and she became febrile. At this point, her antibiotics were switched from cefazolin to vancomycin to cover MRSA.\nOn hospital day five, the patient was noted to have a new erythematous area over the anterior left knee, inferior to the patella (). Ultrasound revealed a small fluid collection superficial to the patellar tendon in the infrapatellar region measuring 3.3 × 2.5 × 0.4 cm (). The infrapatellar bursa was aspirated and sent for culture. The patient was started on piperacillin-tazobactam, given the patient's immunocompromised status and subsequent risk for atypical and gram-negative organisms.\nAn MRI was performed on hospital day seven (this was delayed due to the patient's pacemaker) but did not reveal any evidence of osteomyelitis. The patient was clinically improved after starting piperacillin-tazobactam, and vancomycin was discontinued on hospital day seven. On hospital day eight, aspirate cultures returned with Pseudomonas aeruginosa; she was stable for discharge at that time and was sent out with a ten-day course of levofloxacin (culture was pan-sensitive) and close follow-up with infectious disease. |
A 71-year-old Caucasian female patient presented to the general surgeon appointment complaining of dysphagia, after initial evaluation in the general practice clinic. She is retired but used to work in a textile manufacturer. The patient had a previous medical history of arterial hypertension, osteoarthritis, and incomplete CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome and was medicated with glucosamine, calcium supplements, and amlodipine (10 mg, once daily). She had no known medication allergies and no history of tobacco or alcohol consumption; the patient also denied chronic use of nonsteroidal anti-inflammatory drugs. The familial medical history was unremarkable.\nThe dysphagia was present for two years for solid foods only. The patient avoided this kind of food but otherwise had no other restrictions on her diet. She also complained of sporadic dyspeptic symptoms. The patient had no other symptoms namely nausea, vomiting, sialorrhea, anorexia, weight loss, or abdominal pain. The physical exam was unremarkable.\nThe diagnostic workup started in the general practice clinic with an upper endoscopic study and esophageal manometry. The endoscopy was incomplete due to stenosis at the cricopharyngeus not allowing passage of the standard endoscope; no biopsies were taken. The esophageal manometry showed dysmotility of the distal two-thirds of the esophagus. The patient was then referred to a general surgery appointment in our institution.\nA second endoscopic study was performed to achieve mucosal sampling and try to surpass the stenosis. This was possible with a 55 mm scope and the mucosa of the proximal esophagus showed trachealization with no other relevant alterations. The stomach and the duodenum were normal. Biopsies were taken from the stenosis and the adjacent mucosa of the esophagus (both proximally and distally).\nA barium swallow study and a second manometry were also performed. The former did not reveal any esophageal diverticula, stenoses or hiatus hernia and both showed no signs of esophageal motility disorders (Figure ). There was no anemia, abnormal leucocyte count or elevated C-reactive protein in the blood analysis.\nThe biopsies revealed mucosa with a stratified squamous epithelium with lymphocytic infiltrate in a peri-papillary distribution and spongiosis. No granulocytes, namely eosinophils, were observed (Figure ). There were no signs of malignancy or dysplastic alterations. The pathologic study concluded that there was chronic inflammation of the mucosa compatible with lymphocytic esophagitis.\nThe patient was started on esomeprazole 40 mg, once daily. Four months later, she referred symptom improvement being able to swallow solid foods, complaining only of sporadic heartburn. Nowadays she maintains acid-suppressing therapy and follow-up by a general surgeon. She has an upper endoscopic procedure and repeated mucosal sampling scheduled at one year after the initial evaluation. |
A 55-year-old Japanese woman was referred to our hospital because of hepatic tumors detected by abdominal ultrasonography during a screening examination. Her medical and family history was unremarkable; her occupational and residential history showed no apparent exposure to asbestos. She received no medical drugs, and neither smoked tobacco nor drank alcohol. On admission, her general status was unremarkable; her temperature was 36.2 °C and her blood pressure was 110/70 mmHg with a regular heart rate of 80/minute. A physical examination revealed no abnormal findings; neurological examinations were unremarkable. All laboratory data, including levels of the serum tumor markers carcinoembryonic antigen, cancer antigen 19-9, alpha fetoprotein, protein induced by vitamin K absence or antagonist-II, and cancer antigen 125 were within normal ranges (Table ). An abdominal enhanced CT scan revealed four hypervascular, round hepatic tumors. One tumor, which was 32 mm in diameter, was in contact with the lateral liver segment and the hepatic hilus; the other three tumors were in contact with the diaphragm and measured 7 mm in segment 4, 17 mm in segment 7, and 15 mm in segment 8 (Fig. a–d). There was no pleural effusion or ascites, and no other nodular lesions in her chest or abdomen were observed. Similar to the CT findings, magnetic resonance imaging (MRI) revealed a tumor of lower intensity than the liver on T1-weighted images, and with higher intensity than the liver on T2-weighted and diffusion-weighted images (Fig. a–c). Upper and lower gastrointestinal endoscopic examination findings were normal. We hypothesized that the tumor arose from her liver, and diagnosed our patient as having multiple hepatic hemangiomas, at first. However, imaging findings were not typical of hepatic tumor: all tumors were on the edge of the liver and the tumor margins were clear. Moreover, the tumor in segment 7 appeared continuous with the diaphragm with similar high-density contrast medium (Fig. d). We then performed CT angiography to obtain further details.\nCT findings during arterial portography showed enhancement in none of the tumors (Fig. a and b), but CT during hepatic arteriography revealed strong enhancement in the tumor in the lateral segment and that this tumor was fed by the left gastric artery. In contrast, the remaining three tumors had no enhancement and were fed by the right inferior phrenic artery (Fig. c and d). Imaging findings indicated that the liver was not the origin of these tumors. Moreover, all tumors exhibited increased uptake of 18F-fluorodeoxyglucose, with a standardized uptake value of 7.8 with positron emission tomography (PET)-CT (Fig. ). We considered these tumors to be peritoneal malignant tumors, preoperatively, and performed surgical resection. Laparotomy revealed that the tumor in the lateral segment was clearly in contact with the hepatoduodenal ligament, and that the other three tumors were on the diaphragm (Fig. a–c). We performed tumorectomy with left liver lobectomy (Fig. ) and partial diaphragmatic resection. Histopathological and immunohistochemical examinations of the resected specimens were positive for CD34, CD31, D2-40, and calretinin, which confirmed epithelioid mesothelioma (Fig. a–h). Our patient was not given any medication except a general drip infusion and prophylactic antibiotics; she was discharged 10 days after surgery without complications. CT and PET-CT revealed no recurrence 20 months after surgery without adjuvant therapy. |
A 78-year-old woman was presented to the hospital without prior colorectal cancer screening. Physical examination was significant for Virchow's node (Troisier's sign). Colonoscopy revealed innumerable uniform polyps most heavily concentrated in the cecum. Biopsy revealed mantle cell lymphoma. A 78-year-old woman with history of hypertension and hyperlipidemia presents to the surgical clinic for colonoscopy. Upon review of systems, patient reports dysphagia. Her physical examination was remarkable for a soft nontender anterior cervical lymphadenopathy, and a left supraclavicular lymph node enlargement (Virchow's node; Troisier's sign). Upper endoscopy and colonoscopy were scheduled within the next week. Upper endoscopy revealed a partially occluding oropharyngeal submucosal lesion. There were no signs of ulceration or any mucosal involvement. Some polyps were observed within the esophagus, but the stomach and duodenum were unremarkable. Colonoscopy revealed numerous polyps were increasing in quantity from the transverse colon to the cecum. On colonoscopic inspection, polyps were uniform in size and shape resembling a polyposis syndrome. Multiple biopsies were obtained in the cecum, and ascending and transverse colon, and then sent to pathology. Initial pathology report returned with the polyps as atypical lymphocytic infiltrate, which was later identified to be mantle cell lymphoma. Outside of the lymphatic system, mantle cell lymphoma is seen as a multiple lymphomatous polyposis, or lymphomatous intestinal polyposis. Its detection and identification are of utmost importance due to the aggressive nature of the disease. This is a patient who presented with a physical examination concerning for malignancy, which was later confirmed via biopsies in the colon and in a cervical lymph node. Mantle cell lymphoma is known as a late-presenting aggressive cancer. Outside of the lymphatic system, it can present as lymphomatous intestinal polyposis or multiple lymphomatous polyposis in the colon. This phenomenon should be recognized and incorporated into the differential workup of polypoid diseases of the colon.\nA 78-year-old woman with past medical history significant for hypertension and hyperlipidemia presents to the surgical clinic for colonoscopy. The patient denied history of a colorectal screening examination. Upon review of systems, the patient reported dysphagia. Her physical examination was remarkable for a soft nontender anterior cervical lymphadenopathy and a left supraclavicular lymph node enlargement (Troisier's sign; Virchow's node; Figure ). Due to the concerning physical examination findings, upper endoscopy and colonoscopy were scheduled within the next week. Upper endoscopy revealed an oropharyngeal submucosal lesion in the lumen as seen in Figure .\nDue to the precarious location, biopsy was not performed at that time. There were no signs of ulceration or any mucosal involvement. Some polyps were observed within the esophagus, but the stomach and duodenum were unremarkable. Colonoscopy revealed numerous polyps increasing in quantity from the transverse to the right colon. On colonoscopic inspection, polyps were uniform in size and shape resembling a polyposis syndrome. These are depicted in Figures and . Multiple biopsies were obtained in the cecum, and ascending and transverse colon and then sent to pathology. Initial pathology report returned with the polyps as atypical lymphocytic infiltrate, which were later identified to be mantle cell lymphoma with increased uptake of Ki-67 and positive fluorescence in situ hybridization (FISH) translocation with chromosomes 11 and 14. High magnification of cecal biopsy slide is located in Figure .\nComputed tomography (CT) scan soft tissues in the neck, CT chest, abdomen, and pelvis, along with positron emission tomography (PET) scan were performed (Figure ). CT neck results showed multiple left-sided pathologic nodes along with mass effect in the left-sided laryngeal region and supraglottic region (refer to Figure ). Sclerotic lesion in the vertebral body of C4 concerns for metastasis. CT abdomen pelvis was remarkable for periaortic lymph nodes, the largest of which was 1.3 cm. Excisional biopsy of cervical 1.5 cm cervical lymph node located posterior to the sternocleidomastoid and deep to the platysma was dissected from the surrounding tissue and sent to pathology for analysis (refer to Figure ). Provisional report identified the biopsy to exhibit CD5-positive B-cell non-Hodgkin lymphoma, later confirmed to be mantle cell lymphoma compatible with FISH analysis of biopsies from the colon. Morphologic features, increased mitosis and high Ki-67 proliferation index (80%), indicate aggressive variant with blastoid and pleomorphic features. |
A 61-year-old woman presented to a knee surgeon with a 6-month history of pain in the distal anterior left thigh accompanied by ipsilateral knee instability and stiffness. Pain was exacerbated by walking up and down stairs, squatting, kneeling and was worse at night, whereas resting the knee in extension alleviated pain. The patient was otherwise well and without history of previous knee injury. She had power walked 6 miles daily for many years. Salient medical history included shock-wave treatment for left-sided rotator cuff calcification many years previously. On examination, there was tenderness 3–4 cm proximal to the left patella associated with effusion and quadriceps wasting.\nPlain radiographs and an MRI scan of the left knee were undertaken.\nRadiographic appearance and MRI signal change confirmed calcification rather than ossification or enthesopathy. An unusual diagnosis of dystrophic calcification of the left quadriceps tendon was made (Figs –).\nElectrolytes and renal function, bone profile, parathyroid hormone, ESR and CRP were unremarkable.\nDespite conservative management, her symptoms continued and she was offered surgery.\nThe surgical procedure began with an arthroscopy. Synovitis was identified in the supra-patella pouch where foci of calcified material protruded from the quadriceps tendon. An arthroscopic shaver was used to excise calcified deposits, prior to open operation.\nUsing a 12 cm longitudinal incision in the distal anterior thigh, the quadriceps tendon was exposed and ~10% of the tendon was excised to remove as much of the visible calcification and surrounding paste-like material as possible. Calcific foci in the deeper part of the tendon involving vastus intermedius were removed preferentially. The synovial layer was closed, followed by tubularization of the remaining tendon and closure of the wound. The knee was splinted in extension and the patient was discharged from hospital, fully weight-bearing on the left leg, on Day 3 following surgery. Her bracing was diminished gradually, enabling progressive flexion over 4 weeks. Two weeks post-operatively, the patient was more comfortable than prior to surgery.\nHistology of the excised material revealed chronically inflamed synovium as well as fibrous tissue with aggregates of calcific material without evidence of dysplasia or malignancy.\nTen months following surgery the patient was pain free and could flex her left knee to 120°. The Fulkerson modification of the Lysholm score was 99/100 []. |
A 79-year-old Caucasian male, with a past medical history of atrial fibrillation on warfarin and metoprolol, and coronary artery disease on atorvastatin with previous coronary artery bypass grafting and placement of a dual-function pacemaker/ implantable cardioverter defibrillator (ICD), was on a motor boat in a remote location. The patient’s boat went over a wake of a larger boat passing by. He bounced off his seat in a vertical direction and subsequently landed on his tailbone. After the high impact fall, he complained of both immediate lower back and diffuse abdominal pain but did not seek out urgent medical help.\nTwo days after the initial incident, he started to become pale and diaphoretic; additionally, his ICD delivered three shocks over a 30-min period. He presented via ambulance service to a local community hospital in hemorrhagic shock with a blood pressure of 63/22 and heart rate of 118 beats/min. A primary survey was pertinently positive for hemodynamic instability and diffuse abdominal and lower thoracic spine tenderness.\nHe was resuscitated with 1 L of normal saline leading to an improvement of his pressure to 106/88. Initial laboratory investigations included a hemoglobin of 95 g/L, lactate of 6.1 mmol/L, creatinine of 129, and a supratherapeutic INR of 8.8. An initial non-contrast CT abdomen and pelvis showed moderate hemoperitoneum with sentinel clot in the left upper quadrant and pericolic gutter, as well as the area adjacent to the posterior wall of the stomach. An additional finding of a severely comminuted, minimally displaced burst fracture of the T10 vertebral body was noted (). Further interventions included INR reversal with 3 mg of Vitamin K and 3000 units of prothrombin complex concentrate, and administration of 2 units of packed red blood cells and 2 L of normal saline. Based on clinical severity, the patient was transferred to the trauma service at a tertiary-care Level 1 trauma center.\nPrimary survey revealed a protected airway, spontaneous and bilateral air entry, and hemodynamic stability with a blood pressure of 100/60 and a heart rate of 88 beats/min. His abdomen continued to be mildly distended and tender without peritoneal signs, however the patient reported it had improved since his original presentation to the local hospital. Repeat laboratory investigations revealed a stable hemoglobin of 94 g/L, and correction of his INR to 1.2. Given his stable condition, he underwent a CT RIPIT (Rapid Imaging Protocol in Trauma) [] and CT angiogram (CTA) of the abdomen and pelvis. His imaging revealed pseudoaneurysms of the left gastric artery measuring up to 6 mm with another 9 mm rounded area of increased attenuation along the lesser curve of the stomach (, ). No extravasation was seen. Decision was made to monitor the patient closely with serial abdominal exams and repeat imaging in 72 h, or sooner if the patient exhibited any signs of deterioration.\nOver the next 72 h, the patient’s vital signs and abdominal exams, improved and his hematological profile remained stable. Given the patient’s CHADS score of 2, it was decided to hold therapeutic anticoagulation until the patient’s bleeding risk decreased, however deep venous thrombosis prophylaxis was initiated. A repeat CTA was performed to follow the evolution of the two pseudoaneurysms. The imaging study revealed unchanged pseudoaneurysms but noted the distal aspect of the left gastric artery was attenuated in keeping with a focal dissection and intramural thrombus. Secondary to the dissection, the patient was started on 81 mg of aspirin daily. Over the next few days the patient continued to improve clinically, and was discharged home. The patient lived outside of the local area, and arrangements for close follow up were made. |
A 39 years old woman with a background history of left atrial myxoma with total tumor excision done on November 24, 2017, presented to us on June 21, 2018 with progressive headache associated with blurred vision, nausea and vomiting which was lasting for 1 month. There was no history of fever, trauma or seizure. Brain computed tomography (CT) showed heterogeneous hemorrhagic lesions surrounded by edema. The lesions were spread through both hemispheres with the largest one in the left frontal lobe about 2.4*2.2 cm in diameters (Fig. ). Magnetic resonance imaging including MRI, MRA and SWI confirmed the presence of multiple brain metastases of myxoma and cerebral aneurysm formation from myxoma involving the bilateral anterior cerebral artery (ACA) and middle cerebral artery (MCA), right posterior cerebral artery (PCA) and superior cerebellar artery (SCA) (Fig. ). Subsequent 18F-FDG PET-CT was performed to exclude malignancy (Fig. ). The echocardiographic showed no recurrence of the atrial myxoma.\nOn July 12, 2018, the patient was admitted to the Beijing Tiantan Hospital for treatment. A left frontotemporal craniotomy was performed. The left ACA arterial aneurysms, which were responsible for intracranial hemorrhage in the left frontal lobe lesion, were neurosurgical clipping and a little bit of the frontal tissue (4*4*8 mm) was excised for histological examination. For safety of the patient, other lesion sites involving the right ACA and the bilateral MCA, right PCA and SCA were not treated by surgery. And they found myxoma cells in the brian tissue by histological examination confirming the cerebral metastases despite 7 months having passed since the cardiac myxoma was resected. Hematoxylin and eosin staining of both tissue disclosed typical atrial myxoma cells, embedded in a loose myxoid matrix in the resected tissue (Fig. ).\nAfter the surgery, the patient received antiepileptic, antiemetic and rehydration treatment and other symptomatic treatments. Her headache, blurred vision, nausea and vomiting disappeared gradually. Ten days later, she had a very good recovery and discharged from the hospital. After then, she received antiplatelet treatment and a follow-up observation. In May, 2019, she received reexamination of magnetic resonance imaging including MRI and MRA. Results showed that the brain lesions became smaller, but edema around them became larger than before and the metastatic aneurysms rarely changed (Fig. ). However, no headache, nausea, vomiting, and other neurological symptoms appeared, so she kept the antiplatelet treatment and follow-up observations. |
In November 2006, a 36 years old male presented with severe plaque type psoriasis, to our hospital clinic. He has had psoriasis for the last 13 years; first it was localized to the scalp and periodically spread to other small areas of his body so he could control his disease with topical therapy. However, for the last six years the disease spread and covered the majority of his integument. He denied prior history of psoriatic arthritis. He had a positive family history of psoriasis, which affected his father, sister and paternal grandmother, but they all had a milder disease. His past treatments included various topical agents and several years ago he had a course of UVB with significant improvement, however he could not resume phototherapy because he lost his health insurance. Otherwise his past medical history was unremarkable. He had no known drug allergies and did not smoke. His current medications included over the counter iron pills. He denied any other systemic medications.\nExamination revealed severely erythematous scaly, excoriated thick plaques involving approximately 80% of his body surface area, and pitting of his nails. The rest of his physical examination was unremarkable. After completion of the screening tests which were within normal limits except from mild normocytic anemia (HGB = 12.0 gr/d) and elevated platelets count (470 × 103/mc, normal range 180-400 × 103/mc) and receiving informed consent he was treated with efalizumab (Raptiva) 0.7 mg/kg SC once weekly and subsequently 1 mg/kg as per label. Under treatment his skin condition has gradually improved. However, after 3 months of treatment he had started to complain of arthralgia first involving his right ankle and latter on extending to 2 fingers in both hands and eventually to his left ankle. Treatment with Ibouprofen 600 mg twice daily did not relieve his pain. X-ray analysis confirmed the diagnosis of psoriatic arthritis therefore treatment with Efalizumab was interrupted after 9 months. In addition during the 9 months of efalizumab therapy his platelets count rose substantially (Figure ). Then, etanercept (Enbrel) 50 mg twice weekly was started. After an initial flare of his skin condition, which was expected after cessation of efalizumab, a significant improvement of both his skin and joints ensued. Interestingly during the sixteen months of etanercept treatment his platelets count returned to normal. But unfortunately due to health insurance coverage problems he had to stop etanercept and consequently his skin conditioned dramatically deteriorated and resembled his baseline condition when he first reported to our clinic. Therefore cyclosporine (Neoral) 200 mg twice daily was introduced for approximately 2 months till he received his insurance approval for adalimumab (Humira) and again his skin and arthritis gradually improved. His platelets count rose while being off etanercept and even when he was treated with cyclosporine and his skin condition started to improve his platelets counts remained persistently high and only after adalimumab was started they returned to normal values. |
A 44-year-old man was referred to our institution for evaluation of bulging on his right chest wall. Bulging had started spontaneously without any event of trauma to the chest wall. On history, he had received silicone prosthesis insertion 14 years ago for correction of Poland syndrome at a different institution. Except for the chest wall, he did not show additional deformities related to Poland syndrome, such as hand or upper arm hypoplasia. Otherwise his medical history was unremarkable.\nOn physical exam, fluctuation and slight erythema was persistent over the whole right chest wall.(Fig. ) Radiologic exam was performed for baseline evaluation.(Fig. ) CT revealed absence of right pectoralis muscle, and a subcutaneous dome-shaped attenuation measuring 16.2 cm in its base diameter, highly suggesting periprosthetic fluid collection. The fluid was seen on both the superficial and deep plane of the prosthesis. Inside the prosthesis, numerous full-thickness perforations of regular diameter were found, which explained the massive fluid collection on both planes around the prosthesis. Under the impression of late periprosthetic seroma formation, syringe aspiration was done. About 500CC of serous fluid was evacuated and sent out for cytology. Cytology showed mixed inflammatory cells which was consistent with chronic active inflammation. Immunohistochemistry for CD30 showed negativity. However, fluid collection relapsed and required additional aspirations. After 2 rounds of office-based aspiration, surgical management with implant removal and complete capsulectomy was planned.\nUnder general anesthesia, a 6 centimeter-sized oblique incision was designed slightly above the right costal margin. Upon incision and subcutaneous dissection, serous fluid was drained. After evacuation of remnant fluid, capsule was revealed. The capsule and implant was removed en-bloc with minimal injury. (Fig. ) Gross examination showed a very thick capsule, measuring over 4 millimeters in thickness. Multiple, atypical nodular growth over the whole implant capsule was found. The capsule was sent out for histology. The pocket was thoroughly irrigated and closed with negative suction drains. Postoperative course was uneventful.\nHistology revealed chronic inflammation with fibrosis. Fortunately, no evidence of implant-associated malignancy was found. (Fig. ) Up to 1 year, He is being followed up with no signs of recurrence. (Fig. ) Informed written consent was obtained from the patient for publication of the images. |
A 67-year-old male with a past medical history of congestive heart failure, non-ischemic dilated cardiomyopathy, atrial fibrillation, and pacer-dependent heart block, presented to the University of Miami emergency department with refractory complex partial seizures. Due to the fact that the patient was pacemaker-dependent, he was unable to undergo MRI. Therefore, a contrasted CT scan of the brain was performed, which demonstrated a heterogeneously enhancing left frontal mass just anterior to the left motor cortex thought to be either a primary central nervous system tumor or a metastatic lesion (Figure ).\nA full metastatic workup was conducted with no primary lesion detected. Given the location of the lesion, further imaging was thought to be necessary for two reasons: 1) to further elucidate the differential diagnosis of the lesion and 2) to help safely guide resection or biopsy of a lesion in close proximity to eloquent brain tissue. As a result, cardiothoracic surgery was consulted for the possible replacement of the patient’s current pacemaker with an MRI-compatible alternative. The patient subsequently underwent the removal of their pacemaker and replacement with a Surescan Medtronic DDD Pacemaker RVDR01 (Minneapolis, MN, US). Following this procedure, an MRI was performed and demonstrated a multi-lobulated, relatively uniformly enhancing left frontal lesion (Figure ). Due to the lack of a definitive diagnosis, the decision was made to perform an awake craniotomy with MRI-assisted stereotactic guidance and intraoperative electrocorticoraphy for a definitive diagnosis. The lesion was able to be resected with the initial pathology suggestive of Mycobacterium tuberculosae brain abscess. The patient was placed on anti-tuberculosis medication and intravenous (IV) antibiotics and discharged home. Approximately one year later, he returned to the hospital after suffering a partial seizure with subsequent right upper extremity weakness. He was found to have a recurrence of his left frontal lesion and was again taken to the operating room for surgical resection. Repeat imaging at one year did not demonstrate any further recurrence of his previously seen lesion. |
A 50-year-old Caucasian woman presented with generalized bone pain and weakness. She reported a subtotal thyroidectomy operation for her multinodular goiter 20 years before admission. Her medical history included hypertension, for which she was taking amlodipine 10 mg daily, and recurrent nephrolithiasis. Her physical examination was normal, and she seemed to be euthyroid at presentation.\nHer biochemical findings were suggestive of PHPT (Table ). Abdominal ultrasonography demonstrated bilateral pelvicaliectasis and microcalculi of the right kidney. Bone mineral density was osteopenic at her spine and hip. Neck ultrasonography showed heterogeneous thyroid parenchyma and a hypoechoic lesion at the inferior region of her right thyroid lobe compatible with a parathyroid adenoma.\nUnder a presumptive diagnosis of PHPT, she underwent excision of the enlarged right inferior parathyroid adenoma. The excised mass was well defined with a yellow-pink cut surface and was measured as 20 mm × 15 mm × 10 mm on gross examination. Histopathological examination revealed a parathyroid adenoma with foci of epithelioid granuloma representing non-caseating granulomas (Figure ). There were epithelioid histiocytes and multinuclear giant cells tending to come together, with lenfoid cells surrounding the granulomas (Figures and ). Two of her lymph nodes were compatible with reactive hyperplasia.\nPossible underlying pathophysiological disorders for non-caseating granuloma formation were sought thoroughly. She was completely asymptomatic for tuberculosis, and a polymerase chain reaction (PCR) assay for Mycobacterium tuberculosis of the homogenates of the parathyroid tumor was found to be negative. QuantiFERON tuberculosis testing was reactive; her tuberculin skin test was 15 mm, which was considered normal in our country. Computed tomographic scans of the neck and chest did not exhibit any abnormality. To exclude sarcoidosis, her serum angiotensin-converting enzyme level was measured and was detected as normal. Her fundus examination was also normal. Her medical history and clinical findings for berylliosis, coccidioidomycosis, and histoplasmosis were all negative.\nNine months following the operation, the patient's serum calcium level remained within the normal range (Table ). She is still asymptomatic and has no complaints in terms of any disease. The etiology of the non-caseating granulomas has not been determined yet. The patient is keen on attending regular visits, so we choose to follow her only with watchful waiting rather than perform additional tests. |
An 81-year-old male presented for consideration of vertebral augmentation due to diagnosis of stage IV, metastatic prostate adenocarcinoma, and worsening back pain. Lupron therapy was initiated at diagnosis four months prior. Docetaxel treatment was planned for six cycles but was subsequently stopped after the first cycle secondary to side effects. No radiation therapy was previously given. PSA level was 120.73 at diagnosis and 0.6 before radiofrequency ablation.\nAt the first appointment, the patient reported mild back pain and required a walker but was able to ambulate without difficulty. He did have pain upon palpation of the thoracolumbar junctional level. He did not have any neurologic deficit at presentation. Computed tomography (CT) scans showed 40% compression deformity of T12. Magnetic resonance imaging (MRI) showed pathologic involvement of T12 and L1 and metastatic involvement of the epidural component, resulting in 40% spinal canal stenosis (Figure ). At this time, vertebral augmentation was recommended although it was believed the epidural component would not be addressed and Radiation Oncology would need to be consulted. In a short period of two months, the patient’s condition deteriorated where he was wheelchair bound due to severe pain, not controlled with NSAIDS or opioids. In addition, repeat studies showed further tumor infiltration involving T11, prompting augmentation of T11, in addition to T12 and L1.\nThe procedure was performed under monitored anesthesia care (MAC) and fluoroscopic guided imaging. Under this image guidance, 10-gauge introducer needles were advanced into the T11, T12, and L1 vertebral levels using a bilateral transpedicular approach (Figure ). A drill and osteotome were used to create cavities at the anterior aspect of the vertebral bodies. Bilateral 17-gauge bipolar radiofrequency probes were advanced into the vertebral cavities and simultaneous application of radiofrequency energy was performed as part of the protocol for volumetric ablation of the vertebral bodies. These were done in serial at T11, T12, and L1 vertebral bodies for approximately 15 minutes for each level. Lastly, methylmethacrylate was injected into the vertebral bodies of T11, T12, and L1 (Figure ) for vertebral stability. No complications occurred during the surgery and the patient was discharged the same day.\nThe patient reported no pain at the three-week follow-up and he was able to ambulate without assistance and continued to increase daily activities. He also no longer required any pain medication. He continued to be pain-free at the eight-week follow-up and repeat MRI showed stable vertebral changes and complete resolution of epidural disease at the T12 and L1 level (Figure ). At nine-months post-op, the patient still had no pain and returned back to his normal activities. |
A 38-year-old woman with no relevant medical history was brought into our emergency department for urgent neurological evaluation. Her relatives described a sudden language disturbance which had started less than three hours earlier. They also explained that throughout the previous day she had complained of gradually worsening holocranial headache and had suffered vomiting. There was no prior history of migraine or toxic abuse. On examination, the patient was slightly agitated and showed global aphasia without any other neurological deficit. The National Institutes of Health Stroke Scale (NIHSS) scored 6 points, all of them from the area of language.\nA non-contrast cerebral CT scan, performed 185 minutes after the aphasia onset, was normal, revealing no signs of brain ischemia or hemorrhage. A transcranial Doppler study (TCD), immediately carried out after CT scan, showed velocity asymmetry between both middle cerebral arteries (MCA) compatible with a left MCA TIBI 3 pattern. An embolic stroke was suspected and intravenous thrombolysis was proposed. As the time elapsed since the aphasia onset was now over 3 hours, a multimodal brain MR study was made following our center´s protocol. Although the images revealed extensive delayed perfusion in the whole left hemisphere, including the territory of anterior (ACA), MCA and posterior (PCA) cerebral arteries, there were no altered diffusion-weighted images (Figure ). In the MR-angiography the diameter of left MCA and its main branches was reduced with respect to the contralateral artery (Figure ). There was no fetal origin of left PCA or flow alteration in terminal internal carotid artery. The existence of a complete hemispheric perfusion/diffusion mismatch, together with the clinical picture including the presence of headache, led to a presumed diagnosis of HaNDL (ICHD-II 7.8). A lumbar puncture was performed with a CSF opening pressure of 18 cmH20, 80 cells/mm3 (95% lymphocytes), 0.50 g/L proteins and 82 mg/dL glucose. These results were congruent with the suspected diagnosis. Invasive procedures or treatments were avoided, and the patient was admitted to a follow-up of her evolution. Eighteen hours later her symptoms had improved, and 2 days after admission her NIHSS scored 0. A new TCD study showed symmetric MCAs velocities.\nCSF stains and cultures were normal. Additional tests performed (CBC, ESR, biochemistry, thyroid hormones, vitamin B12 and cold agglutinin) showed normal values. Borreliosis, syphilis, brucellosis, mycoplasma, hepatitis B, C and HIV serologies were negative. Studies of autoimmunity were carried out (ANA, anti-DNA, anti-Ro, anti-LA, anti-RNP, p-ANCA, c-ANCA, IgG and IgM cardiolipin), all yielding negative results. Given the presence of CSF pleocytosis and the patient's clinical evolution, we did not consider a high probability of reversible cerebral vasoconstriction syndrome or primary CNS vasculitis. Moreover, since it has been described that cerebral angiography could worsen neurological deficits in HaNDL, conventional arteriography was not performed.\nAfter 30 days the patient suffered another episode. She came back to the emergency department with worsening headache, which had started 10 hours earlier, accompanied by vomiting without fever or other neurological deficits. The patient recalled the intensity and characteristics of headache as identical to that of the previous event. CT without contrast was again performed, showing no ischemic or hemorrhagic lesions (images not shown). A new TCD study displayed no abnormalities in mean blood flow velocity (MFV) of intracranial arteries. Lumbar puncture was not done because there was no neurological deficit and the suspicion of HaNDL recurrence was high. The episode was resolved with 50 mg of intravenous dexketoprofen. There were no further recurrences. We repeated TCD and multimodal-MRi four months later. These explorations were completely normal, including FLAIR images, with symmetric findings in both hemispheres (Figures and ). |
A 28-year-old man presented to the emergency department of our hospital complaining of sudden painful loss of vision in the left eye. He gave a history of hit by a sharp metallic object on the left eye while hammering an iron plate. His left eyelids were mildly swollen, but the orbital rim was intact with no crepitation. Visual acuity in the left eye was limited to the perception of hand motion, while the visual acuity in the right eye was 20/20, and the left intraocular pressure (IOP) was not measurable. Slitlamp examination revealed a full thickness scleral laceration of 4.0 mm length with prolapsed uveal tissue, a shallow anterior chamber, and a traumatic cataract. The details of the posterior segment could not be visualized. Computed tomography (CT) demonstrated an intraorbital foreign body with intensity of iron that had passed through the left eyeball and was located in the intraorbital space close to the optic nerve (Fig. ). The right eye was normal.\nThis study was conducted at the Shandong University Qilu Hospital and the procedures used were approved by the Ethics Committee of the Shandong University Qilu Hospital. The procedures conformed to the tenets of the Declaration of Helsinki.\nThe primary repair of the scleral perforation with abscission of the prolapsed and necrosed uveal tissue was done on the emergency basis. The conjunctiva was incised along the limbus cornea, the sclera was exposed, and the scleral laceration was confirmed and sutured. We actually attempted to maneuver the foreign body behind the eyeball with the use of a magnet, but this was not successful. We therefore severed medial rectus muscle. An iron foreign body was found and was removed in a single piece (Fig. ). From outside of the eye, the exit laceration could not be confirmed. The operation was completed without a scleral suture of the exit laceration. Postoperatively, the intravenous antibiotics were administered, topical antibiotics and steroids with cycloplegics. Visual acuity in the left eye was perception of hand motion, the wound was healthy with intact sutures, the anterior chamber was formed, and the lens was cataractous with no view of the retina. The IOP in the left eye was 13 mm Hg. Removal of the foreign body was confirmed by a postoperative CT scan.\nTwelve days later, the patient underwent pars plana lensectomy and 3 ports (23-gauge) pars plana vitrectomy. We observed a scleral exit laceration near the optic disc, but did not see any objects. The full-thickness posterior pole defect was closed. With endolaser photocoagulation and silicone oil injection, the patient had his retina reattached. On follow-up after 4 weeks, the visual acuity was always limited to hand motion perception, scleral sutures were intact. The anterior chamber was formed. The IOP was 15 mm Hg. At 3 months after the operation, visual acuity in the left eye was the perception of hand motion and the left IOP was 15 mm Hg (noncontact tonometer). There were no postoperative complications (including retinal detachment, proliferative vitreoretinopathy, infection, sympathetic ophthalmia, and hemorrhage). |
A 70-yr-old woman, previously in good health was transferred to our hospital with the history of left sided chest pain, non-bilious vomiting and shortness of breath for four days. Six days before admission, the patient had a routine health check-up including chest radiography and upper gastrointestinal study (UGIS) using a gastric bloating agent at a local clinic. According to the referring clinic, the chest radiography and UGIS were normal (, ). However, after taking the gastric bloating agent the patient developed increasing epigastric pain and nausea.\nOn admission, the respiratory rate of the patient was 38 breaths/min and the oxygen saturation was 91% in room air. Physical examination showed absent breath sounds and dull to percussion findings at the left lung base. The chest radiography revealed gastric air-fluid levels and bowel loops in the left thoracic cavity. After the placement of a nasogastric tube, the chest radiography showed displacement of the tip of the tube to the dilated stomach located in the left thoracic cavity (). An UGIS using gastrograffin was performed without a bloating agent (). The UGIS showed no transit of the gastrograffin through the duodenum and a hook-like image was noted at the medial portion of the herniated stomach. A chest CT revealed absence of the left side of the diaphragm (). The impression was that the patient had a mesenteroaxial gastric volvulus with a diaphragmatic hernia. The patient underwent surgical exploration.\nA left posterolateral thoracotomy was done at the sixth intercostal space. We examined the surface of the entire lung field but could not find any diaphragmatic tissue. The abdominal organs (stomach, spleen, splenic flexure of colon) were present in the left thoracic cavity (). We attempted to rearrange the contents for devolvulation but could not achieve this due to the presence of severe pleural adhesions. Warm saline irrigation and a left lower lobe adhesiotomy were performed. There was no diaphragmatic tissue; we could not repair the diaphragm and return the abdominal organs, from the thorax, to the abdomen. The pleural cavity was closed in the usual manner. Conservative treatment was continued with nothing per oral and nasogastric drainage. Follow-up UGIS was performed 10 days after surgery (); the abdominal organs remained in the left thoracic cavity but there was no disturbance of gastric passage. The patient improved by 12 days after surgery, on a soft diet. Thirteen days after surgery the chest radiography showed resolution of the herniation but mild haziness remained at the left lower lung field (). The patient was discharged 20 days after admission. During the 1 yr of follow-up, the patient continued to do well. |
The third case is of a 68-year-old Irish woman who presented to the MMUH in April 2016 with acute laryngitis. She had a background of bipolar affective disorder which had been stable for the past 30 years on monotherapy with lithium. There had been a recent history of lithium toxicity secondary to a deterioration of her renal function, which had been managed at her local psychiatric hospital. After the episode, she had been restarted on a low dose of lithium as well as a low dose of valproate.\nOn presentation to the MMUH she was initially treated jointly by the ear, nose, and throat (ENT) team and medical team and was managed in an ICU environment due to respiratory compromise. She had no oral intake for multiple days. Once stabilized she was transferred to an acute medical ward but an acute onset confusional state with bizarre behavior was noted over a period of 2 days. Due to her psychiatric history the Liaison Psychiatry service was consulted. On review she was severely thought disordered and confused. She was only able to produce a word salad and showed echolalia. She had motor retardation, increased tone, negativism, and posturing on examination. The impression was that she was suffering from acute catatonia. Brain imaging did not reveal acute abnormalities. She was diagnosed as having bipolar I disorder with catatonia as per DSM-5 (Table ).\nAdvice was given to treat her with paliperidone. Her mental state improved slightly as a result, but she remained severely thought disordered and confused for 2 weeks. Eventually, lithium was cautiously reintroduced under close monitoring of her renal function. The reintroduction of lithium was well tolerated and she improved significantly over a 2-week period. At discharge she was no longer thought disordered, she was well orientated, and back to her fully independent baseline. She continues to live independently to date. |
A 7-year-old boy visited to our hospital complaining of a slowly growing mass in his right posterior auricular region for 3 months. On physical examination, about a 3 cm sized pulsating soft mass in the posterior auricular region was palpated. There was no definite abnormality on the laboratory findings.\nPlain radiographs of the skull revealed a relatively well-defined, osteolytic lesion in the right temporal bone. No evidence of sclerotic margin or periosteal reaction was found (). Temporal bone CT revealed about a 5 cm sized, soft tissue density mass with marked bone destruction that mainly involved the mastoid portion of the right temporal bone (). The mass showed dense heterogeneous enhancement and intracranial extension with a large area of necrosis.\nOn T1-weighted MR images (TR/TE, 509/14), the mass was heterogeneously hypointense with some high signal foci and signal void dots (). The T2-weighted MR images (TR/TE, 4225/100) revealed a heterogeneous signal intensity mass with multiple signal void dots (). After an intravenous infusion of contrast media, the main mass involving the temporal bone was intensely enhanced (). This lesion appeared to extend intracranially at its superior aspect, and there was associated peritumoral edema in the adjacent temporal lobe. The intracranial portion of the mass showed intermediate signal intensity on the T1-weighted images and homogenous high signal intensity on the T2-weighted images, and there was peripheral rim enhancement that represented necrosis ().\nA large, ill-defined, hypervascular mass involving the right temporal region was seen on the early arterial phase of the external carotid arteriogram, and the mass was mainly supplied by petrosal branches of the middle meningeal artery (). The lesion still remained hypervascular on the late venous phase of the arteriogram, and this was due to delayed washout of contrast media (). No arteriovenous shunting could be observed.\nThe preoperative diagnosis was malignant tumor of a vascular origin and the differential diagnosis included other sarcomas such as rhabdomyosarcoma.\nThe patient underwent an incomplete tumor resection due to massive bleeding via the right temporal approach. The removed tumor was a brown colored fragile soft tissue mass that measured up to 4.5×5.0×6.5 cm. The intracranial portion of the mass was noted to have hemorrhagic necrosis. Histologically, the tumor was composed of short strands or solid nests of pleomorphic and highly atypical spindle cells. The cells showed relatively abundant eosinophilic cytoplasm, bizarre nuclei and frequent mitotic figures including the atypical forms. The tumor showed focal luminal differentiation filled with erythrocytes (). A vascular origin for these cells was confirmed by the positive test for CD34, a specific endothelial marker. This tumor was finally diagnosed histologically as a grade III hemangioendothelioma.\nFollowing the surgery, the patient underwent radiotherapy. At one year after the initial disease presentation, the boy ominously returned the emergency room due to dyspnea, and the chest radiograph we took showed bilateral pneumothorax with multiple cavitary lung metastases. Sadly, two years after the initial disease manifestation, our patient succumbed to his illness despite our best efforts using radiotherapy and concurrent chemotherapy. |
An 11-month-old Malay boy presented with a 6-month history of an intensively pruritic scaly rash characterized by crusting and excoriation over the body (Figures and ). The infant was seen at 8 months of age by his family physician. He was misdiagnosed to have atopic dermatitis with secondary bacterial infection at 10 months of age, and he was treated with topical mometasone furoate cream daily for two weeks, oral cloxacillin for 7 days, and an emollient several times a day. In spite of the treatment, there was no improvement of the eruption and no relief of the itch. A serum immunoglobulin E (IgE) was performed and was found to be normal. Because of the intense pruritus and the lack of improvement with the current therapy, the infant was referred to one of us (KFL) at 11 months of age.\nPast medical history revealed that the infant was born to a 26-year-old primigravida woman at 39 weeks gestation following an uncomplicated pregnancy and delivery. He was exclusively breastfed for 6 months, at which time solid food was introduced. The developmental milestones were normal. His past medical history was otherwise unremarkable, and he had not been on any medications until 8 months of age which was 3 months after onset of the rash.\nFamily history revealed that both parents had an intensely pruritic erythematous papular eruption affecting the interdigital web spaces and lateral aspects of fingers approximately 2 to 3 months after the onset of the eruption in the infant. The parents did not have any crusted lesions. They were seen by a dermatologist, who made the diagnosis of scabies and treated with 5% permethrin cream with reduction of the pruritus and improvement of the lesions. On further questioning, the babysitter was found to have crusted scabies. She was seen and treated by a dermatologist.\nOn examination, the infant was well nourished and not in distress. His weight was 8.8 kg, height 74 cm, temperature 37°C, heart rate 78 beats per minute, and respiratory rate 28 breaths per minute. Diffuse, scaly, crusted, hyperkeratotic, erythematous patches and plaques were seen over the body. Some of the lesions were excoriated. The lesions were accentuated on the groins, palms, and soles (). The rest of the physical examination was normal.\nDirect microscopic examination of skin scrapings revealed numerous scabies mites and eggs. A skin biopsy was performed on one of the lesions which revealed the scabies mite within the epidermis (). A diagnosis of crusted scabies was made. His complete blood cell count, differential count, T-cell and B-cell subsets, quantitative immunoglobulins, and HIV test were all normal.\nThe infant was treated with overnight application of topical 5% permethrin cream to the entire body weekly for a total of 6 weeks. There was complete resolution of cutaneous lesions at the end of the treatment (Figures and ). |
A 64-year old female was admitted to the hospital with symptoms of abdominal discomfort, diarrhea, and weight loss. The patient also had intermittent nausea and vomiting for few months treated as gastritis. Her past medical history included breast carcinoma which was diagnosed seven years earlier on screening mammography and was treated with lumpectomy and axillary node clearance followed by adjuvant radiotherapy and hormonal treatment (Tamoxifen) for five years. This was a 32 mm grade one infiltrating lobular carcinoma (T2 N0 M0) and hormone receptor (Estrogen (ER) positive, progesterone (PR) negative). Patient remained well with no clinical or radiological evidence of recurrence on her regular oncology follow-up visits. Patient was not on any current medications at this time of admission. She is nonsmoker. No family history of breast or gastrointestinal carcinomas.\nPhysical examination was remarkable for pale sclera, mild lower abdominal tenderness with no rebound tenderness and bowel sounds were present. Rectal exam was normal but fecal occult blood was positive.\nInvestigations showed normocytic normochromic anemia (Hemoglobin 9.4 grams per deciliter, Mean corpuscular volume 88.0 femtoliters, and Mean cell hemoglobin 29 picograms). White blood cell count was 11, 000 × 109/l. Upper and lower endoscopy were performed which revealed multiple areas of nodular thickening involving the gastric antrum proximal duodenum and the hepatic flexure of the colon (). Biopsies showed infiltrating adenocarcinoma with signet ring cell morphology suspicious for primary gastric tumor (). However, Immunoperoxidase stain of the specimen revealed cytokeratin (CK) 7 and ER both were positive () while CK-20 and CDX2 were negative suggestive of metastatic breast carcinoma. PR and HER2 were negative. Staging investigations showed extensive metastasis to the upper and lower GI tract. No pulmonary or hepatic lesions were identified and nuclear bone scan was normal.\nThe patient was managed initially with palliative hormonal treatment (Letrozole) which was changed to palliative chemotherapy due to disease progression. However, patient did not tolerate the side effects of chemotherapy and it was stopped. After seven months of her diagnosis with the breast cancer metastasis to the GI tract; the patient level of care was changed to comfort care and symptomatic treatment only and was transferred to inpatient hospice. |
A 68-year-old female with primary biliary cirrhosis received a liver transplantation from a deceased heart-beating donor for end-stage liver disease with a MELD of 24. Preoperative study showed no evidence of hepatic artery abnormalities. The donor was a 75-year-old female that was pronounced brain-dead after a stroke. The donor's only comorbidity was significant history of cigarette use. During the donor surgery the celiac artery did not have any atherosclerotic disease and the hepatic arterial anatomy was normal. Of note, the iliac arteries were severely diseased with hard circumferential plaque.\nLiver transplantation was performed by using the Piggy-back technique with restoration of vena caval and portal vein blood flow before the arterial reconstruction. It was then discovered that the native hepatic artery was dissected (Figures and ). In this situation we routinely use a cadaveric iliac jump graft to the infrarenal aorta to reconstructe the hepatic artery; however, the donor's vessels were unusable []. The decision was made to anastomose the donor celiac artery with aortic carrel patch directly to the supraceliac aorta (Figures and ).\nThe length of the donor celiac artery was adequate without tension. The gastrohepatic ligament had already been ligated during the hepatectomy and the stomach was retracted laterally. The diaphragmatic cura were divided in a muscle splitting fashion in an attempt to preserve them starting just below the insertion of the aorta through the diaphragm. The supraceliac aorta was exposed inferiorly for approximately 3-4 cm which allowed cross-clamping with two straight vascular clamps above the celiac artery origin. We did not dissect out the aorta circumferentially to avoid the posterior lumbar arteries in that area of the aorta. An arteriotomy was made and enlarged with two 4.8 mm aortic punches. The anastomosis was created with 5–0 prolene in running fashion. Cross-clamp was 17 minutes.\nPostoperatively, the patient's creatinine rose to 1.6 mg/dL on POD1 and returned to a baseline level of 0.8 mg/dL by POD4. Transaminases reached 1000 (U/L) on POD1 and came down immediately. They did not seem affected by the arterial reconstruction and were probably a result of the age of the donor. The patient was started on TPN from POD1 to POD 9 and had a nasogastric tube until POD 2. TPN was used as a precaution because we were concerned that the patients' age combined with ischemia time to the gut from a supraceliac aortic clamp would cause a significant ileus. She did have some delay in bowel function. We left her nasogastric tube in for two days and then advanced her diet slowly over the next few days. During this time we left her on TPN until she had significant caloric intake by mouth. She tolerated a regular diet on POD 9. She had no evidence of neurological dysfunction []. The arterial anastomosis was patent by CT angiogram on POD 9 (Figures and ).\nBoth the short and long-term outcomes are excellent. She is now a year out and has had no complications including no episodes of rejection. The graft is functioning well and she did not need a retransplant. |
A 14-year-old girl without any previous clinical history presented with a painful, swollen right knee joint after playing volleyball. She was first treated conservatively for knee sprain at another institute, but the pain persisted. She was brought to our clinic for a second opinion 2 weeks after the injury.\nThe physical examination of the right knee showed loss of full extension, swelling during the range of motion, and tenderness. Initial radiographs showed lateral subluxation of the right patella with one fragment in the knee joint (Figures –). Magnetic resonance imaging (MRI) demonstrated an osteochondral defect over the central aspect of the patella with a large loose body (Figures and ). Surgery was suggested.\nOn the third posttrauma week, open reduction and internal fixation were performed. Under general anesthesia with the patient in the supine position, we performed a medial parapatellar arthrotomy to approach the fractured patella. The patella was flipped laterally, and the articular surface of the patella was exposed. Debridement and irrigation of the fracture site with normal saline were conducted. Finally, reduction was performed, and the fracture was fixed with two headless screws (Acutrak®, Acumed, Beaverton, Oregon) (). The medial patellofemoral ligament was also repaired. Intraoperative findings revealed the osteochondral fragment (30 × 25 × 10 mm3) from the central aspect of the patella (), an intra-articular loose body, hemarthrosis, and rupture of the medial patellofemoral ligament, which caused lateral subluxation of the patella. The calculated surgical time from incision to wound closure was 1 hour.\nPostoperatively, partial weight bearing was immediately allowed and a dynamic functional knee brace was applied. The knee was fixed in full extension for 2 weeks, gradual knee flexion was allowed, and physical therapy was prescribed.\nAfter 2 months postoperatively, the patient showed good patella gliding without pain. She could do squats and sports activities as well as she could before the injury (Figures and ). Follow-up radiographs also demonstrated healing of the osteochondral fracture (Figures and ). After 1 year, implant irritation was noted, and implant removal was suggested.\nSecond-look arthroscopy was performed and showed resorption of the cartilage over the screw head. The screw head and some metallosis were found. There were no significant degenerative changes in the cartilage (Figures –). After removal of the implant, her discomfort disappeared. |
A 55-year-old male underwent pancreaticoduodenectomy as an initial treatment for biliary carcinoma in March, 2009 without preoperative chemoradiotherapy. Histopathological examination revealed papillary adenocarcinoma infiltrating to the fibromuscular layer with no metastasis to the regional lymph nodes, with the pathologic stage of the tumor defined as T1 N0 M0. The drainage tubes were placed at the anterior aspect of the pancreaticojejunostomy and the dorsal space of the hepaticojejunostomy. On the 11th day after surgery, the patient had a spiking temperature of up to 38.5°C with purulent and amylase-rich (>5000IU/l) exudates from the bilateral drainage tubes. The initial clinical impression was a leakage of the pancreaticojejunostomy. Continuous lavage from the drainage tubes using 1.0 L saline /day was begun. Although the fever disappeared after three days, the drainage output of turbid fluids with necrotic tissues persisted. On the morning of the 16th day after surgery, the patient had an episode of hemorrhage from the drainage tubes with a duration of a few minutes, but it spontaneously disappeared with the patient remaining hemodynamically stable. A computed tomography (CT) was performed which demonstrated neither an abscess nor a hematoma around the pancreaticojejunostomy (Figures and ). The stump of the gastroduodenal artery (GDA) showed an outgrowth with a length of 6.0 mm without formation of pseudoaneurysm (, arrow). The stump of the GDA was immediately adjacent to the tip of the drainage tube placed at the anterior aspect of the pancreaticojejunostomy (, arrowhead). The sentinel bleeding disappeared just after transfer to the CT room. However, on the evening of the 16th day after surgery, the patient had an episode of back pain, followed by release of 500 mL of hemorrhagic fluid from the drainage tubes. There was no aneurysm or hematoma in the peritoneal cavity visualized with a second CT. The remnant stomach was markedly dilated, being filled with intralumanal fluids. The patient did not vomit, but had severe, burning epigastric pain. An aspiration of the gastric contents yielded 300 mL of hemorrhagic fluids. During the period between the 16th and 17th day postsurgery, the patient had been hemodynamically stable. On the evening of the 17th day postsurgery, the patient vomited large amounts of coffee-grounds liquid with fresh blood in the abdominal drains. Despite the patient being hemodynamically stable, a drop in hemoglobin necessitated transfusion of 2 units of packed RBC. Upper endoscopy revealed a stomach filled with blood but the bleeding point was unable to be identified around the gastrojejunostomy or pancreaticojejunostomy (Figures and ). The patient was kept hemodynamically stable during the 12 hours after the upper endoscopy without bleeding from abdominal drains or hematemesis. On the morning of the 18th day postsurgery, following the appearance of blood in the abdominal drains and hematemesis, copious bloody diarrhea was observed. The resistance of the abdominal wall had increased and a pulsatile mass was palpable at the left upper quadrant. In view of the patient being hemodynamically unstable, a visceral angiogram was performed immediately after fluid resuscitation and blood transfusion of 4 units of packed RBC. The patient underwent emergency angiography using the standard Seldinger technique and an angiography catheter. A selective celiac angiogram showed that the splenic artery and the left gastric artery were all patent with good organ perfusion. A 2.7 cm diameter pseudoaneurysm arising from the stump of the GDA was identified (, arrow). It is generally accepted that gastroduodenal artery pseudoaneurhysm embolization with stent grafting of the common hepatic artery have significant clinical benefits to maintain liver blood flow [–]. However, stent-graft materials adapted to the common hepatic artery could not be prepared for the patient under the emergency conditions. Therefore, coil embolization, both proximal (common hepatic artery) and distal (right and left hepatic artery) of the pseudoaneurysm was performed with microcoils (MWCE-18S-TORNADO) (Figures and . The clinical course and schematic presentation are shown in Figures and . Bleeding from the abdominal drains and gastric tube disappeared about 48 hours after coil embolization. The hemoglobin value had decreased to 6.2 g/dL but gradually returned to 8.0 g/dL without blood transfusion. On the 3rd day postcoil embolization, the patient suffered a fever (up to 39°C). Coagulation tests on day 5 postcoil embolization revealed a marked elevation of fibrin degradation product (FDP). CT of the abdomen revealed that segments 2/3 of the liver were replaced by a low density area located near the edge of the liver (). The source of high fever was proved to be a hepatic infraction of segments 2/3. Because the right main branch of the portal vein was clearly visualized with a high value of portal vein peakvelocity (PVPV) on a doppler ultrasonography (), we believed that severe hepatic failure would not occur. Symptomatic treatment with an initial dose of an antibiotic and vasodilator was started. High fever around 38°C to 39°C persisted for 10 days. Although the high fever resolved spontaneously over the following days, there was a high fever relapse of up to 40°C on the 16th day after coil embolization. CT demonstrated that segments 2/3 of the liver were replaced by fluid-level components with accumulation of gases (). We concluded that the hepatic infarction had led to an intrahepatic biloma and abscess formation. High output of purulent fluids from a decompression tube placed intraluminally at the hepaticojejunostomy was observed, suggesting that the formation of the hepatic abscess was associated with a delayed ischemia of the biliary ducts caused by the embolization of the hepatic artery. Conservative treatment with antibiotics was given based on cultures of fluids from the decompression tube and the patient became afebrile on the 30th day postcoil embolization. On the 32nd day after coil embolization, a CT scan revealed that segments 2/3 of the liver were replaced by a homogenous low-density area with thick capsule formation (). In addition, anastomotic leakage persisted with a high-output pancreatic fistula from the drain adjacent to the pancreaticojejunostomy (). Continuous irrigation from the drain with saline and enteral nutrition from the jejunostomy catheter yielded complete relief of symptoms associated with anastomotic leakage on the 34th day after coil embolization (). A solid diet was started on the 40th day after coil embolization. All drains and tubes were removed until the 50th day postcoil embolization. On the 68th day after coil embolization, the patient suffered high fever with epigastric pain. A hepatic abscess was localized by CT in the edge of segments 2/3 penetrating to the lesser sac, which was immediately treated by percutaneous drainage (). The patient recovered uneventfully and was discharged on the 101st day after coil embolization. Two months after discharge a CT scan showed disappearance of segments 2/3 of the liver with no abscess formation present (). The patient underwent no postoperative adjuvant therapy. The patient is in good condition without any recurrence in the 13 months since recovery. |
A 42-year-old female, Caucasian, patient was referred to our institute suffering from a large, pedicled, painless, slow-growing mass, located on the anterior thoracic wall. In addition the patient was complaining of fatigue and weakness for the past 3 months. The lesion developed as a long-standing tumour with duration of approximately 22 years. There was a history of incomplete previous resection of the tumour twice in the past, resulting in locoregional recurrence. The tumour was diagnosed according to the first histological report as a dermatofibrosarcoma protuberance (DFSP) and according to the second one as a hemangiopericytoma. In addition to recurrence, neglect on the part of patient, patient's fear, and embarrassment for her disease may have played a role in the development of this chronic large tumour.\nOn physical examination, the tumour appeared as an exophytic pseudolobulated tan-pink mass, measuring 18 × 10 cm, with multiple hemorrhagic foci, yellow and black necrosis, and localized infection with purulent exudates (). There was no evidence of regional lymph node involvement. Laboratory examination revealed hypochromic microcytic anemia with hemoglobin serum level of 5.2 mg/dl, probably due to the intermittent bleeding of the tumour. The iron-deficiency anemia was relieved with the administration of 2 blood units. Magnetic Resonance Imaging (MRI) studies revealed no bone or cartilaginous involvement, apart from the chest wall soft tissue infiltration. The additional studies, including chest X-ray and abdominal ultrasound that were performed, were unremarkable with no evidence of metastatic disease.\nBecause of the high vascularity of the tumour prior to incisional biopsy, a preoperative selective embolization of the right internal thoracica artery was performed in order to reduce vascularity and minimize intraoperative hemorrhage. The patient underwent wide local resection of the tumour by a surgical team consisting of thoracic and plastic surgeons (). For the reconstruction of the wide defect both pectoralis major advancement flaps were mobilized and covered with a partial-thickness skin graft ().\nThe histological examination of the biopsy and resection specimen revealed a spindle cell tumour with strong diffuse CD34, Bcl-2, and vimentin positivity but negativity for S-100, c-kit, smooth muscle actin, and cytokeratin (AE1 + AE3). Immunohistochemically, the tumour cells were stained also negative with CD99. Microscopically spindle cells were arranged patternless, with a characteristic hemangiopericytoma-like morphology, and there were areas with very high cellularity (Figures and ). The spindle cells had moderate cytologic atypia and 9 mitoses per 10 HPFs. Foci of superficial necrosis were also identified (coagulative tumour necrosis). The tumour appeared centered on subcutaneous tissues. Surgical resection margins were not involved. The diagnosis of histologically malignant extrapleural SFT was confirmed. The differential diagnosis of this tumour, in particular, exclusion of a DFSP— tumour's original diagnosis—was made on the basis of its characteristic microscopic appearance in conjunction with immunohistochemical features. Histologically, SFTs present a typical, although not diagnostic, hemangiopericytoma-like morphology with patternless arrangement of spindle cells in a collagenous background whereas DFSPs are characterized mostly by a storiform pattern. In addition, DFSP stains frequently positive for CD34 but negative for Bcl-2.\nPostoperatively the patient received adjuvant radiotherapy (Intensity-Modulated Radiation Therapy) and was closely followed up. In the ensuing 12 months after surgery and radiation treatment, the patient has remained asymptomatic and without clinical or radiological evidence of recurrence or distant metastasis. |
Our patient is a 58-year-old male with past medical history of hypertension and distant right femur fracture who was involved in a motocross accident suffering left- (1st-12th) and right-sided (7th, 9th, and 12th) rib fractures with bilateral hemopneumothoraces requiring bilateral chest tube placements. The patient also suffered a right intertrochanteric and a peri-implant femur fracture as well as a nondisplaced ulnar styloid process fracture. The patient was transferred from an outside hospital for further care. Upon arrival the patient was remarkably asymptomatic, in normal sinus rhythm, hemodynamically and respiratory stable, maintaining an O2 saturation between 95% and 100% with minimal supplemental O2 via nasal cannula. The patient denied significant chest pain or shortness of breath despite his significant injury burden. ECG findings showed some ST wave abnormalities suggestive of early repolarization. An initial troponin level of 0.15 normalized within 24h of admission. Given the patient's injury pattern and troponin leak in the absence of known coronary artery disease (CAD), congestive heart failure (CHF) pulmonary embolism (PE), or shock, a formal TTE was obtained. TTE revealed severe tricuspid regurgitation secondary to flail anterior tricuspid valve leaflet with preserved right ventricular geometry and systolic function. The cardiac surgery team recommended outpatient follow-up for elective repair in two months with repeat TTE.\nThe femur fracture was repaired on hospital day two under general anesthesia and the patient recovered well. The chest tubes could be sequentially removed without recurrence of pneumothoraces over the course of the admission. On hospital day three the patient experienced an episode of atrial fibrillation with rapid ventricular response that responded well to a single 5 mg intravenous bolus of metoprolol. After a few hours the patient converted back to normal sinus rhythm, in which he remained until the day of discharge on hospital day seven. The patient continued to deny any palpitations, shortness of breath, or radiating chest pain. By the time of discharge, the patient was able to ambulate, and his pain was well controlled with oral analgesics. Unfortunately, by one year after hospital discharge, the patient had not followed up with regard to his newly diagnosed tricuspid regurgitation. |
The propositus was a 39-yr old father of one child, healthy, morphologically normal Korean male who demonstrated mixed field hemagglutination on cell typing by both the manual tube and gel card techniques at the time of blood donation (). This mixed field agglutination pattern is consistent with an ABO subtype that is relatively common in Korea known as B3 and is caused by a series of mutant B alleles known collectively as B alleles.\nTo investigate the hypothesis that the mixed field agglutination in the propositus was caused by a B allele, allele-specific polymerase chain reaction (AS-PCR) and direct sequencing of exons 6 and 7 of the ABO gene were performed using previously described methods (, ) on the propositus' genomic DNA. Initially these investigations demonstrated only the O01/O02 genotype. However, a careful review of these chromatograms revealed very small B-allele specific peaks at nucleotides 526, 657, 703, 796, 803, and 930 (). The guanosine residue at position 261 was not detected. The former single nucleotide polymorphisms (SNP) discriminate common O and A alleles from B alleles, while the 261delG SNP is only present in common O alleles.\nIn an effort to demonstrate a B allele in the propositus, haplotype-specific templates were prepared using B allele-specific primer pairs. Haplotype sequencing revealed the presence of a normal B101 allele. This normal B101 allele was also detected when exons 6 and 7 of the propositus' ABO gene were cloned and sequenced. Thus 3 ABO alleles, B101, O01 and O02, were conclusively demonstrated to be present in the propositus. This confirmed that the mixed field agglutination observed with anti-B reagent indicated the simultaneous presence of two distinct RBC populations (group B and group O) in the propositus.\nThe propositus denied receiving a stem cell transplant or transfusion and did not have a known twin, thus chimerism or mosaicism was suspected to explain the presence of the 3 ABO alleles.\nTo discriminate chimerism from mosaicism, nine short tandem repeat (STR) loci were tested on DNA extracted from blood, buccal mucosal cells, and hair from the propositus, and on DNA isolated from blood on his immediate relatives (father, mother, and older brother). Out of 9 STR loci, 4 loci (D3S1358, D5S818, D13S317, and D18S51) demonstrated a pattern consistent with a double paternal DNA contribution confirming the presence of dispermic chimerism (). To investigate the extent of the propositus' chimerism, ABO typing as well as sequence analysis of ABO exon 6 and 7 was performed on his immediate relatives (). Both parents were blood type B and shared the B101 allele while the O01 allele was also detected in the propositus' mother, and the O02 allele in his father. His brother was also had blood type B with the B101/O02 genotype.\nUsing sequence-based typing on genomic DNA, one paternal and one maternal HLA class I (A, B) and II (DRB1) haplotype was identified in the propositus (). A double paternal DNA contribution was thus not identified. Analysis of 50 metaphases indicated that the propositus was also a karyotypic mosaic: 32 (64%) metaphases exhibited a normal male karyotype, 46,XY, while 18 (36%) metaphases demonstrated 47,XYY. No structural chromosomal abnormalities were present. |
A 35-year old male patient was admitted to the Department of Otorhinolaryngology with a compensated inspiratory dyspnea and biphasic stridor. Video fibroscopy revealed a severe subglottic stenosis grade III according to the Cotton-Myer scale. Computed tomography scan of the neck and chest confirmed an hourglass-shaped subglottic stenosis which carried down to the second tracheal ring (). Three months prior to hospitalization, the patient underwent aortic valve replacement because of severe regurgitation after bacterial endocarditis. The patient was intubated in an intensive care unit for 5 days. A month after this procedure, the first signs of breathing problems began to appear. The cardiac status before our intervention displayed good functionality of the replaced valve but moderate insufficiency of the mitral and tricuspid valves, with an ejection fraction of 28%. The patient showed a decrease in functional capacity - the Duke Activity Status Index score was 4 points. According to the American Society of Anesthesiologists was grade IV. The patient's informed consent was obtained according to the guidelines of the local medical ethics committee.\nGeneral anesthesia was induced and patient was ventilated through a laryngeal mask airway. Low collar incision was performed and the subplatysmal flap was raised. The trachea and cricoid cartilage were dissected from the soft tissues surrounding them, while protecting the recurrent nerve. The first two tracheal rings were shrunk, with a loss of cartilaginous support. Through an incision just beneath the pathological section, the patient was intubated with a tube and afterwards the stenotic segment, the cricoid arch and the first two tracheal rings were resected. The lumen of the stenosis was 2.5 mm (). Severe adhesions were found and resected posteriorly enveloping the corresponding segment of the esophagus. Afterwards, the trachea was released from the upper mediastinum and thyrohyoid release of the larynx was performed. Thyrotracheal anastomosis was performed using MonoPlus® (B. Braun, Melsungen, Germany) (polydioxanone) sutures; the latter was verified for air leaks via laryngeal mask ventilation. At the level of the collar incision (approximately 4 cm beneath the anastomosis), an intercartilaginous incision of the trachea was performed and both superior and inferior skin flaps were sutured with the tracheal edges creating a tracheostomy, as shown in 3, without placing a cannula. The postoperative period was uneventful. Crusts were removed meticulously from the tracheostomy and the latter was closed under local anesthesia at the end of the first month. One additional finding of interest was a significant post-operative increase in the ejection fraction up to 41%. The follow-up period was one year and no signs of restenosis or notable granulation were registered in the area of anastomosis or at the level of the temporary tracheostomy. |
A 51 year-old male was diagnosed with chronic pancreatitis of seven months duration, having an obscure etiology. He was a non-drinker and a non-smoking. His chief symptom included several episodes daily of severe abdominal pain, and unable to stand without narcotic analgesics. The pain was mainly epigastric, but radiating to the rear. It was more severe after meals, and was relieved by leaning forward. Patient had lost weight (from 90 kg to 65 kg), and was diabetic.\nThe diagnosis of chronic pancreatitis was made based on the clinical presentation, radiological workups and percutaneous biopsy at another center. He had a metallic biliary stent for biliary stenosis. Endoscopic examinations revealed gastritis. A percutaneous celiac axis blockage was required for severe episodes of pain. Before referring to our center, patient had undergone laparotomy for pancreatic resection; however, because of the portal hypertension, the procedure had been capped off. The pancreatic tru-cut biopsy at this laparotomy confirmed the diagnosis of chronic pancreatitis without evidence of malignancy. He was then referred to us for surgery for pain relief.\nAt the time of admission, he was daily using narcotic analgesics, proton pump inhibitor, pancreas enzyme extracts and antidepressant drugs. He had mild anemia, slightly elevated liver function tests, and CA-19-9 was 202 U/ml. Computed tomography was compatible with chronic pancreatitis (heterogenous parenchyma, dilated pancreatic duct), and a 4 cm pseudocyst was seen at the tail of the pancreas ( and ). Positron emission tomography revealed the pancreatic activity as 5.1 SUVmax.\nWe intended to make a pylorus-preserving pancreaticoduodenectomy with portal vein replacement. Before surgery, the patient and his relatives were informed of the possible surgical methods. A reverse “L” incision was used. Adhesions of the prior surgery and venous collaterals complicated the procedure. A cholecystectomy was performed, and the common bile duct was transected for bilio-enteric anastomosis to replace the biliary stent. The metallic stent in the distal bile duct was checked, but was found to be stuck and could not be removed. Subsequently, the common bile duct stump was closed. The dilated venous collaterals, particularly around the head of the pancreas, resulted in substantial blood loss during the dissection, and 3 red pack cells were transfused during the procedure (). It was then decided to pursue with the Frey procedure instead of pancreatic head resection. The inflamed pancreatic head was excavated, and the cavity at the pancreatic head was expanded to the distal pancreas ( and ). The enlarged pancreatic duct became visible at the body and tail of the pancreas; however, the pancreatic duct at the head of the pancreas was not visible (). A Roux-en-Y pancreaticojejunostomy was created by 3-0 running polypropylene suture. Bilio-enteric anastomosis was done 10 cm distal of the pancreatic anastomosis with 4-0 polydiaxone interrupted sutures. Entero-enterostomy of the Roux limb was created in the usual way (). Totally, 40 ml absolute alcohol was injected around the celiac plexus for pain relief. Laparotomy was closed after insertion of two abdominal drains. The total operative duration was 490 minutes.\nDuring the early postoperative period, patient was delirious in the intensive care unit, but the attacks were relieved after medications. Fortunately, the remaining postoperative course was uneventful, except for prolonged abdominal ascites drainage and intractable diarrhea that commenced on day 11, and was treated conservatively (). The drain amylase levels were continually in the normal ranges. Oral liquid and semisolid nourishments were started on days 5 and 7, respectively. Patient required only one dose of 60 mg meperidine in the postoperative period. He was discharged on day 28. Unexpectedly, pathological examination of the pancreatic specimen reported chronic pancreatitis accompanied with pancreatic cancer. He was then referred to the medical oncology department. |
A 44-year-old male, Chinese businessman, mainly for multi-joints swelling and pain for more than half a year, was admitted to Department of Nephrology and Rheumatology in our hospital on January 9, 2018. Six months before admission, he presented successively multiple joint arthralgia and mild swelling, mainly involving shoulder joints, the PIP joints of both hands, the second and third of MCP joints of both hands, double wrist joints, with morning stiffness for a few minutes. In the course of the disease, he had no symptoms of dry eyes, blurred vision, mouth ulcer, rash and hair loss, etc. He accepted “three oxygen therapy” in Horqin First Hospital, which made swelling and pain of above joints partially relieved. However, his right shoulder joint pain got worse one week before admission, and he was admitted to our hospital. His previous medical history included progressive hearing loss for several years and microhematuria for one year by a physical examination. His father died of colorectal ulcer. His mother is relatively healthy without hearing loss and obvious kidney diseases, and she never had a urine test. His daughter also had a history of microhematuria found by a medical examination for enrollment one year ago. On physical examination, his vital signs including blood pressure, body temperature, respiration rate and pulse rate were within normal range, and no abnormalities were found in lungs, heart and abdomen. The PIP joints of both hands, the second and third of MCP joints of both hands, and his right shoulder joint had mild tenderness with no obvious swelling.\nLaboratory data was as follows: blood routine was normal; urinalysis revealed hematuria (3+) with 100% of deformed red blood cells by phase difference microscopic examination and microalbuminuria (0.101 g/24 h); blood chemistry showed normal function of liver, serum creatinine within normal range (106 μmol/L) and moderately increased level of blood uric acid (526 μmol/L); inflammatory markers including C-reactive protein and ESR were normal; serological studies revealed an elevated level of RF (46.0 IU/ml), a dramatically elevated level of ACPA (>250RU/ml), normal levels of immunoglobulin and complement; ANA and ANCA were negative; serum hepatitis B surface antigen and anti-hepatitis C virus antibody were negative. The X-ray of both hands was normal. There were no abnormalities on chest computed tomography and electrocardiogram. Renal ultrasound showed diffuse changes in double renal parenchyma and double renal multiple cysts with partial cystic wall calcification. The results of hearing test suggested binaural sensorineural deafness, and no abnormalities were found in the eye examination.\nThe patient with swelling and tenderness of muti-joints over six weeks, double-positive for RF and ACPA, according to 2010 ACR/EULAR classification criteria for RA [], was definitely diagnosed with early RA. However, a history of microhematuria could not be completely explained by RA, as microhematuria occurred before RA. To further clarify the cause of hematuria, a renal biopsy was performed on January 18, 2018. Light microscopy showed glomerular minimal change with chronic tubulointerstitial disease, and immunofluorescence microscopy showed mild stainings for IgM(+) and IgA(±), with others negative (Fig. ). Electron microscopy revealed thin GBM (130-200 nm), epithelial foot process segmental fusion, no dense deposit, with edema and lymphocytes infiltration of interstitium (Fig. ). The diagnosis of electron was TBMN with tubular interstitiallesion (Fig. ). Combining with microhematuria, hearing loss and TBMN, we highly suspected that he had a very rare disease for Alport syndrome. Mutational Analysis of Alport syndrome genes (COL4A3, COL4A4 and COL4A5) by Beijing Genomics Institute revealed the mutation in COL4A5 (c.1351 T > C, p.Cys451Arg, hemizygous, missense mutation) encoding α5 of type IV collagen. In silico analyses using Database of Non-synonymous Functional Predictions (dbNSFP) predicted that p.Cys451Arg is functionally “tolerated”(0.07, scores less than 0.05 are classified as “damaging”) by Sorting Intolerant From Tolerant (SIFT), “possibly damaging” (0.603, Polymorphism Phenotyping 2 HumVar), “probably damaging”(0.971, Polymorphism Phenotyping 2 HumDiv), “probably damaging”(0.963, Mutation Taster), and.\n“deleterious” by Likelihood Ratio Test. Above results further proved the diagnosis of X-linked Alport syndrome.\nThe patient has been regularly receiving the treatment of total glucosides of paeony and leflunomide for RA, and Cozaar 50 mg for the protection of kidney so far. During the 10-months follow-up, swelling and tenderness of the joints in this patient had been generally relieved, but there was no obvious improvement in microhematuria and a slight increase in proteinuria. |
A 60-year-old man with a history of asthma, benign prosthetic hypertrophy, and hyperlipidemia presented 1.5 years after an uncomplicated primary right TKA done by an outside surgeon. He had been complaining of 4 months of increased pain in his right knee. An aspiration had been attempted, yielding 1 mL of sanguinous fluid which had not been sent for analysis. The patient continued to have swelling and increased pain in the knee, and an MRI was obtained demonstrating “pseudotumor” (Figure , A–C). He was then referred to our orthopaedic oncology office for further evaluation and management.\nAfter review of initial radiographs (Figure , A and B) and CT (Figure , A–C), the patient underwent an open biopsy of his right tibial lesion adjacent to the tibial baseplate one week after presentation to the office. Pathology from his initial biopsy was consistent with GCTB. One week following his open biopsy, the patient underwent a complex reconstruction of his proximal tibia as well as patellar tendon (Figure , A and B).\nIntraoperatively, complete destruction of the medial cortex of the tibia was noted, with the area infiltrated extensively by tumor. After the initial anterior exposure through the previous TKA incision, the area was extensively curettaged. A high-speed burr and argon beam coagulator was then used to complete the resection at the edges of the cavity. Following the removal of the mass, we noted that the tibial baseplate was mechanically stable even after the extended curettage. An intraoperative determination was made to preserve the primary arthroplasty components and to reinforce the tibia with cement and Steinmann pin fixation. Steinmann pins were fired distally into the tibia, which allowed buttressing of the tibial baseplate proximally. The entire excisional cavity was then packed with polymethyl methacrylate (PMMA) cement. Intraoperative examination demonstrated that the construct had excellent stability and strength afterward.\nFollowing reconstruction of the proximal tibia, attention was turned toward the patellar tendon. We noted that the destructive process had eroded much of the patellar tendon and reconstruction was required. Marlex mesh was used in the technique described by Browne and Hanssen. The mesh was layered into a construct with approximate width as the patellar tendon and then weaved into the remnant of the native patellar tendon into normal tendon tissue. #5 Ethibond suture was used to reinforce the closure and attachment of the Marlex mesh to the tendon, avoiding the placement of mesh adjacent to skin.\nBefore discharge, the patient was placed in a long leg bivalved cast. Three weeks postoperatively, the patient was transitioned into a hinged knee brace, which is locked in extension while upright. The patient was then instructed to allow for bed dangles with the knee. At 6 weeks post-op, the patient began physical therapy for gentle range of motion of the knee, still with brace locked in extension while ambulating. At 7 weeks, the patient was placed on Keflex for 1 week after he noticed a small amount of discharge from his distal incision site after a scab was removed, with resolution of symptoms. Three months post-op, the patient was allowed to weight bear as tolerated on his extremity. At this time, he was started on a trial of denusumab (Amgen Manufacturing Limited) adjuvant chemotherapy under the medial guidance of his oncologist. The patient developed a rash after two doses and was changed to zoledronic acid (Zometa; Novartis Pharmaceuticals Corporation) for a total of 6 months of diphosphonate therapy. He completed the course without further incident. Radiographs taken at 16 months demonstrated maintained alignment without evidence of component subsidence or implant failure (Figure , A and B). At a 20-month follow-up, the patient was weight bearing on the extremity without assistance, using a cane only for long distances. |
A 69-year-old man visited our clinic in July 2010 with complaints of blurred vision and night blindness in both eyes. Our initial examination found that his best-corrected visual acuity in decimal units was 1.0 in the right eye and 0.6 in the left eye. His refractive error was +3.0 D and +3.5 D for the right eye and left eye, respectively. Full-field rod ERGs were absent and cone-rod mixed responses were the negative type, indicating ON bipolar cell dysfunction (). From the symptoms and ophthalmic examination, he was suspected of having PR.\nA general physical examination revealed small cell carcinoma of the lung and Western blot of the patient’s serum showed autoantibodies against TRPM1. We diagnosed this patient with melanoma-associated retinopathy and retinal ON bipolar dysfunction due to TRPM1 autoantibody and have reported his findings in more detail elsewhere. After diagnosis, chemotherapy was started using cisplatin and etoposide combined with radiation for the lung cancer in August 2010. The patient achieved a complete remission, but underwent cranial irradiation in March 2011 to prevent brain metastasis.\nWe have followed this patient ophthalmologically for more than 2 years since his initial visit and his symptoms have not changed. His best-corrected visual acuity decreased slightly to 0.8 in the right eye and 0.5 in the left eye due to progression of cataracts. His visual field has not constricted and his ERGs have not changed (see ). Rod responses were nonrecordable in 2010 and 2012. The amplitudes for a-wave of cone-rod mixed maximum response were 240 μV and 220 μV in 2010 and 2012, respectively, and those for b-wave were 150 μV and 120 μV, respectively. The amplitudes of cone response were about 25 μV for the a-wave and 50 μV for the b-wave at both times in 2010 and 2012. The amplitudes of the 30 Hz flicker ERGs were 14 μV in 2010 and 11 μV in 2012. The difference in the ERGs was most likely within the error of measurement and not due to differences in retinal function. However, fundus photographs taken in October 2012 showed diffuse choroidal hypopigmentation in both eyes (). We also found that the choroidal vessels were more visible than those obtained at the initial examination ().\nSpectral domain optical coherence tomography (Heidelberg Engineering, Heidelberg, Germany) showed a slight reduction in retinal thickness during the 2 years of follow-up, but the morphological architecture of the retina appeared almost normal at both times (). Consistent with the choroidal hypopigmentation of the fundus, spectral domain optical coherence tomography showed a marked decrease in choroidal thickness over the 2-year period (, arrowhead). Subfoveal choroidal thickness in the right eye measured manually was 250 μm in 2010, 180 μm in 2011, and 110 μm in 2012, and for the left eye was 380 μm in 2010, 230 μm in 2011, and 100 μm in 2012. |
An 8-year-old girl presented with a chief complaint of watery discharge bilaterally since birth. Slit lamp examination of the anterior segment and indirect ophthalmoscopy of the posterior segment were unremarkable. Motor development was normal. The absence of puncta and canaliculi was noted bilaterally on examination of the lacrimal system. An opening was present 6–7 mm from inner canthus on the cheek. Syringing of the cutaneous opening of this cheek fistula revealed communication with the nose [Figures and ]. The presence of atresia of puncta and canaliculi bilaterally was confirmed.\nSurgical treatment was performed under general anesthesia. The site of the puncta was marked in both eyes. A lacrimal probe was passed from the fistulous opening into the lacrimal sac with a 23-gauge intracath with insertion of a teflon sleeve at the site of the lower punctum which was directed downward and slightly posterior once it contacted the probe [Figures and ]. The trocar was removed, and fluid in the sleeve was expressed freely at the fistulous opening. The probe was inserted through the skin opening into the nose in the direction of the nasolacrimal duct and a tube was directed toward the nasolacrimal duct to confirm the patency. The passage of fluid from the nose to the throat was confirmed by the presence of blue stain on endoscopy. A teflon tube was fixed at the lid margin with an 8-0 vicryl suture. A fistulectomy was performed and the opening closed with 6-0 catgut, and the skin was sutured with 6-0 Vicryl.\nThe child was examined periodically to ensure the patency of the lacrimal drainage system of both eyes. On last examination at 3 years postoperatively, both lacrimal passages were functional. The patency was tested with lacrimal sac syringing and fluoresceine dye. Radio-imaging with dye confirmed the patency of the lacrimal drainage system bilaterally. The child claimed resolution of epiphora postoperatively. |
A 22-year-old woman was admitted to our hospital with a history of intermittent colicky right hypochondrial pain not relieved by any medications for the past 3 months. Initially, she was given proton pump inhibitors, but her pain was not relieved. She had no other symptoms. Her past medical and family histories were not significant. The result of her clinical examination was normal. Upper gastrointestinal (GI) endoscopy showed globular swelling at the medial wall of D2 (Fig. ). The ampulla was situated at the summit of swelling.\nComputed tomography (CT) with oral contrast agent showed dilation of the intramural part of the common bile duct (CBD). A cyst of size 2.4 × 2.3 cm was noted in the second part of the duodenum (Fig. ). On the basis of the above findings, it was reported as type 3 choledochal cyst. The patient was further investigated with magnetic resonance imaging, which showed dilation of the intramural part of the distal CBD. A 2.4 × 2.3 cm cyst was noted in the ampullary region, again consistent with a type 3 choledochal cyst (Fig. ). Then the patient was planned for endotherapy, but owing to difficulty, it could not be negotiated into the ampulla. Finally, the patient was referred to the surgical gastroenterology department for operative management. After multidisciplinary team discussion, an abdominal ultrasonogram (USG) was done, which showed a clear thick wall cyst measuring 4.6 × 2.6 cm between the second part of the duodenum and the head of the pancreas with gut signature (Fig. ). Opening of the CBD into the duodenum was seen just distal to the cystic lesion. There was active peristalsis noted all around the cyst, suggestive of duodenal duplication cyst.\nAfter the diagnosis was confirmed, the patient was taken for elective laparotomy. Intraoperative findings were a 5 × 3-cm cyst over the medial wall of the duodenum extending proximal and distal to the ampulla and displacing it posteriorly (Fig. ). The cyst was communicating with the ampulla by a small opening in its medial wall (Fig. ). So, cyst secretions were drained via the ampulla, which avoided retention of cystic fluid. Part of the cyst wall was shared with duodenal musculature. Upon needle aspiration, the cyst wall contained bile due to communication with the ampulla. The duodenum was Kocherized, and a longitudinal duodenotomy was made for about 5 cm. Partial excision of the cyst was done (Fig. ) because it was closely associated with the ampulla. Marsupialization of the remaining cyst wall was done. The duodenotomy was closed horizontally. Feeding jejunostomy (FJ) was done using a modified Witzel method. A flank drain was kept in place. The postoperative period was uneventful. The patient was started on oral medications on the third day after surgery. The FJ tube was removed after 6 weeks. Histopathology showed the cyst wall was lined by duodenal mucosal epithelium with focal areas of ulceration and composed of tall columnar cells with goblet cells on either side of a common (shared) muscular layer. The submucosa showed lymphoid aggregates with Brunner glands. The common muscular layer showed congested vessels. Histopathological features were suggestive of duodenal duplication cyst (Fig. ). At her 9-month follow-up visit, the patient had no complaints. |
A 60-year-old male reported at the Postgraduate Clinic, Department of Restorative Dentistry, King's College, London, for the fabrication of a new obturator to replace his prosthesis which was approximately 20 years old. The patient had a maxillectomy done after being diagnosed with oral squamous cell carcinoma of the maxilla, resulting in resection of the premaxilla. Extraorally, there was evidence of scar tissue in the perioral region, which reduced the capacity to retract the upper lips. The patient also had a canted smile line which appeared to show more of the upper right incisors both at rest and when smiling [].\nOn the other hand, intraoral examination revealed a classic Class VI defect, which was a large defect in the premaxillary region, with an oroantral communication. He was missing all of the upper anterior teeth and the upper first right premolar tooth [Figure and ]. His existing prosthesis was also a two-piece obturator and removable partial denture, but the retention of both prostheses was poor. There was a lack of tissue contact and seal between the obturator and soft tissues at the region of the defect and an incomplete extension into the soft-tissue undercuts. This resulted in poor retention of the obturator. The obturator had been fabricated using cold-cured acrylic, and it had not been replaced for many years which resulted in the material becoming hard causing inflammation to the soft tissues in the region of the defect. The periodontal condition of the patient was quite poor with generalized probing depths between 4 and 5 mm in both the upper and lower remaining dentition. The patient's neglect of oral and denture hygiene was clinically visible with plaque and calculus deposition both intraorally and on the prostheses [Figure and ]. The free gingival margins and interdental papilla were inflamed due to chronic periodontal tissue disease.\nInitially, the patient was given oral hygiene counseling to improve plaque control aside from nonsurgical periodontal therapy to manage the deep pockets. The patient's existing denture and obturator were relined with a soft liner (GC Reline™ Soft, GC Europe, Leuven, Belgium) to allow healing of the soft tissues in the region of the defect before making the final working impression. Apart from that, the retention of the existing prostheses was temporarily improved as the soft liner managed to engage into the soft-tissue undercuts in the region of the defect. The area of the obturator and denture which needed to be relined was relieved with a tungsten carbide bur and thoroughly cleaned and dried. GC Reline™ Primer R was applied to the cleaned surfaces and allowed to gently dry. Subsequently, GC Reline ™ Soft was applied to the fitting surfaces of the denture and obturator. These prostheses were then fitted into the patient's mouth one at a time, with the obturator being inserted first followed by the denture. They were allowed to set in situ before the excess was trimmed for the patient's comfort.\nTo overcome the difficulty of inserting a one-piece hollow bulb obturator, the guarded motivation, the condition of soft tissue in the region of the defect, and given the patient's age, the authors decided to provide the patient with a two-piece obturator denture, utilizing the lock-and-key mechanism.\nFirst, a primary impression was taken using irreversible hydrocolloid material (Aroma Fine Plus Normal Set, Alginate Impression Material, GC Corporation, Tokyo, Japan) with a piece of gauze placed across the defect to prevent the impression material from gaining access into the nasal cavity.\nA wax-up (Metrodent No. 2 Modelling Wax, England) of the obturator was done on the resulting cast, and a keyhole was incorporated into the obturator, for the “lock” to allow for the fit of the removable prosthesis, which acted as the “key” []. The completed waxwork was then flasked before finally processing with a heat-cured resilient acrylic, Molloplast B® (Molloplast B, Regneri GmbH and Co. KG, Karlsruhe, West Germany) [].\nThe patient was recalled for a try-in of the resilient obturator intraorally, to ensure that it was able to fit and engage the soft-tissue undercuts without traumatizing the tissues []. Once the patient felt, it was comfortable, and the fit was established, the obturator was used in the laboratory to aid the subsequent fabrication of the conventional heat-cured removable prosthesis.\nA final working impression of the remaining dentition and the surrounding soft tissues including the depth and width of the buccal sulci was recorded using a special tray (Metrodent, Light Curing Tray Material, Germany) and irreversible hydrocolloid material (Aroma Fine Plus Normal Set, Alginate Impression Material, GC Corporation, Tokyo, Japan). The obturator was fitted on the resulting master cast, and this was followed by the fabrication of the definitive baseplate. During the wax-up (Metrodent No. 2 Modelling Wax, England) for the baseplate, an extension of the wax was incorporated into the keyhole simulating the lock-and-key mechanism.\nThe next clinical step was to record the retruded jaw relationship of the patient []. It was then followed with a wax try-in to evaluate the esthetics and phonetics of the patient []. Once the patient was completely satisfied with the appearance and could comfortably articulate, we proceeded with the final processing to produce the conventional heat-cured acrylic (Meliodent® Heat Cure, Heraeus Kulzer GmbH, Hanau, Germany) removable partial denture.\nThe final removable partial denture was designed to be an acrylic denture, engaging into the interdental undercuts with maximum extension of the baseplate for additional retention, resistance, and stability.\nDuring the final fit, the denture was polished, and instructions were given to the patient on how to insert and remove the prostheses. A significant improvement in the patient's appearance could be noticed after insertion of the obturator and denture. There was a slight midline shift toward the right to accommodate the missing teeth in the edentulous space [Figure and ].\nWhen compared with the previous denture, the occlusion of the new prosthesis was maintained as a Class I incisor relationship but with an increased overbite, and the patient was more satisfied with the given denture. Furthermore, through regular oral hygiene counseling, the patient's plaque control had improved tremendously, and the periodontal condition was at the maintenance phase after nonsurgical periodontal therapy. The patient was also advised on how to maintain and clean the prostheses so that it can remain well fitted, functional, and esthetically pleasing. The patient was recalled 1 and 4 weeks postinsertion to examine the health of the soft tissue in the region of the defect as well as the fit of both the obturator and denture. During the first recall visit, minor adjustments were made to the extension of the baseplate of the denture in areas which showed soft-tissue trauma at the buccal sulci. Otherwise, the authors were satisfied with the progress of patient's oral health and handling of prostheses. The patient was advised to come for regular maintenance appointments at 6 monthly intervals. |
A 36-year-old Caucasian, Greek man presented to the out-patient clinic of KAT hospital complaining of weakness and limited range of motion of his right shoulder. He had noticed, over the past two months, that abduction and elevation of the joint had gradually become limited while he was carrying his newborn child in a baby basket. He denied any neck or shoulder pain and could not recall a specific precipitating traumatic event or any recent episode of respiratory infection. However, he reported that his job was a heavy manual one, requiring lifting and carrying heavy objects on his shoulders. Our patient had no significant medical history.\nPhysical examination revealed a winged scapula and asymmetry of his shoulders, with right shoulder depression (Figure ). He was unable to abduct his right arm above 80° in the frontal or scapular plane while his forward elevation was slightly reduced. His passive range of motion was comparable to the normal left side. Scapular winging was marked during abduction and disappeared in forward elevation, while it was only slightly evident at rest (Figure ). Furthermore, there was a marked wasting of his right trapezius muscle with decreased shrugging of the affected shoulder. Both wasting and weakness were not observed in the ipsilateral sternocleidomastoid muscle, and a neurological examination did not reveal other cranial nerve deficits. No brachial plexus neurological signs were detected. Our patient's rotator cuff was judged to be intact.\nResults of his complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum biochemistry were all normal. The initial X-rays of his right shoulder region were unremarkable. Computed tomography (CT) of the shoulder girdle of our patient revealed significant diffuse trapezius muscle wasting (Figure ). Magnetic resonance imaging (MRI) of his cervical spine, right shoulder joint and skull base identified no pathology except mild atrophy of his right sternocleidomastoid and trapezius muscles (Figure ).\nNerve conduction study of our patient's right spinal accessory nerve, with surface stimulation along the posterior border of the sternocleidomastoid muscle and recording from the trapezius, produced no compound muscle action potential. A needle electromyography (EMG) of the right trapezius muscle revealed signs of active denervation (fibrillation potentials and positive sharp waves), atrophy (marked diminution of insertional activity), and severe axonal damage (no recruitment of motor units). Meanwhile, EMG of his right sternocleidomastoid muscle showed findings suggestive of mild axonal injury (decreased recruitment, polyphasicity and prolonged duration with increased amplitude of motor unit potentials). His levator scapulae, serratus anterior and rhomboid muscles were normal. Electrophysiological findings suggested an axonal degeneration of his right spinal accessory nerve that was mainly distal to the innervation of the sternocleidomastoid muscle, and an irreversible denervation of his right trapezius muscle. Consequently, a diagnosis of spontaneous chronic right spinal accessory nerve palsy was established, although not affecting some fibers to the sternocleidomastoid muscle.\nThe chronic nature of our patient's lesion and the denervation of his trapezius muscle with severe loss of most of its motor units suggested the appropriate treatment procedure was dynamic muscle transfer using the levator scapulae and the rhomboid muscles. Our patient refused the recommended treatment, since he felt that his painless disability did not justify this highly demanding procedure. Instead he followed a specific program of physiotherapy, focusing on resistance exercises to progressively strengthen the adjacent scapular muscles and on exercises to preserve the maximum range of motion of his shoulder joint.\nThereafter, he was followed up every month for assessing and modulating the progress of his rehabilitation program and for subsequent EMG investigations. At the last follow-up, eight months after the onset, a slight improvement in the active abduction of his arm and in his neck asymmetry was observed. Significantly, his scapular winging remained painless, with no associated neurological deficits. Our patient was also able to perform his manual work, at approximately the same level as before. A repeat EMG did not show any alterations from the initial electrophysiological findings. |
A 78-year-old male with nephrotic syndrome was referred to our center by his primary care physician. The patient had a history of hypertension since the age of 45 years, hypothyroidism since the age of 68 years and diabetes mellitus since the age of 70 years. He also had a history of percutaneous coronary intervention for coronary artery disease and femoropopliteal bypass surgery for peripheral arterial disease at the age of 74 years, and a history of Wallenberg syndrome since the age of 77 years. He was being treated with an angiotensin II receptor blocker, a calcium-channel blocker, pitavastatin, aspirin and levothyroxine sodium hydrate. Diabetic control by diet and an oral α-glucosidase inhibitor were sufficient. There was no evidence of retinopathy or albuminuria before the episode of nephrosis. His urinalysis on admission was positive for proteinuria (2.5 g/day) and red blood cells (1–4/hpf). His blood chemistry findings were as follows: albumin 3.2 g/dl, creatinine 1.11 mg/dl and HbA1c 6.1%. A kidney biopsy revealed 21 glomeruli, of which 2 were globally sclerotic. The glomeruli showed increased mesangial matrices and mild GBM thickening. Massive foam cells had infiltrated into the glomerular capillaries and mesangial region (fig. ). These foam cells stained positive with Oil Red O (fig. ) and immunohistochemically stained positive for CD68 (fig. ). Transmission electron microscope images also revealed foam cell infiltration mainly located in the capillaries, and some mesangial cells showed scattered lipid droplets in their cytoplasm (fig. ). Mild interstitial fibrosis and tubular atrophy (20% of the cortical region) as well as moderate arteriosclerosis and arteriolar hyalinosis were found. The foamy change was seen in some tubular epithelial cells but no interstitial foamy cells were found. Immunofluorescence studies revealed segmental deposits of IgM and C3 in the mesangial area and slightly peripheral IgG deposits in the glomeruli; however, no significant deposit was found by electron microscopy. He had an apoE2 homozygote phenotype and the Arg158Cys genotype with no other mutations. After the diagnosis, ezetimibe was added to his statin therapy. The patient's most recent serum creatinine was increased to 2.1 mg/dl after 2 years. |
A 23-year-old Greek man who had episodes of hematemesis and hematochezia was admitted to the emergency department of our hospital. Clinical signs of anemia and splenomegaly were recognized on our patient. His medical history revealed that he was missing an FVL. His peripheral blood examinations revealed the following: hematocrit, 22.2%; hemoglobin, 7.5 g/dL; international normalized ration (INR), 1.22. Results of his liver function tests, as well as the rest of his biochemical examinations, were within normal limits.\nAn emergency endoscopy performed on our patient showed enlarged bleeding gastric varices but no esophageal varices. This led us to consider that the enlarged varices may be secondary to splenic vein thrombosis. We used a Sengstaken-Blakemore tube on our patient, but this failed to restrain his bleeding.\nUltrasound and CT scan of our patient revealed his enlarged spleen and an engorged splenic artery with a diameter of 1 cm, and a fusiform dilated splenic vein measuring 5 × 6 × 9 cm (Figures and ).\nAn angiography was performed using the Seldinger technique on the femoral artery of our patient. Selective celiac angiography and superselective splenic arteriography with frontal and bilateral oblique projections were also performed. A venous phase follow-up examination demonstrated that our patient had a completely occluded splenic vein. GVs and dilated gastroepiploic veins were also noted.\nAn emergency embolization of our patient's splenic artery was subsequently performed. A guidewire was directed into his splenic artery, and a wedge balloon catheter was passed over the guidewire using several giant Gianturco steel coils. Initially, a coil with a diameter of 10 mm and a length of 10 cm was used because his splenic artery measured around 10 mm in diameter on CT images. Subsequently, smaller coils (5 mm × 5 cm) were used to occlude the lumen of the 10 mm × 10 cm coil (Figure ). The procedure was uneventful and the bleeding of his GVs eventually subdued.\nOur patient was discharged one week later without any symptoms. An elective surgical splenectomy was also scheduled. |
A 51-year-old Indian female presented to the emergency department of her local hospital with a history of sudden-onset, severe bilateral occipital headache. Her headache had started suddenly three days earlier with no prodromal symptoms or preceding visual aura. The headache was continuous, exacerbated by coughing and relieved when she was recumbent. The pain radiated across the top of her head to the temporal region and was associated with nausea and mild photophobia. There was no relief with simple analgesia. A history of a recent coryzal illness was elicited but that had fully resolved by the time of presentation.\nHer past medical history was unremarkable and she was on no regular medications. There was no history of migraine and no family history of headaches, cerebrovascular accident or sudden death.\nOn examination, the patient was alert and her vital signs were within normal limits. Neurological examination was normal and Kernig's sign was negative. There was no neck stiffness and no rash. Fundoscopy showed no signs of raised intracranial pressure.\nInitial investigations revealed a mild leucocytosis with a neutrophilia but inflammatory markers were normal (erythrocyte sedimentation rate 32 mm/hour; C-reactive protein level <5 mg/L).\nHer history of acute-onset severe headache suggested a diagnosis of subarachnoid haemorrhage. A contrast-enhanced computed tomography (CT) brain scan proved normal. A lumbar puncture showed no evidence of red cells in the cerebrospinal fluid (CSF) and the opening pressure was normal at 11 cm/H2O. Spectrophotometric examination of the CSF was negative for xanthochromia. A magnetic resonance angiogram was performed to exclude the presence of an intracranial aneurysm. This was normal.\nIn light of the negative investigations and symptomatic improvement with bed rest and weak opioid analgesia, a tentative diagnosis of migraine was made and the patient was discharged.\nTwo months later, although symptomatically improved, the patient still had persistent headache and sought a further opinion. A repeat magnetic resonance imaging (MRI) brain scan revealed new bilateral thin (<1 cm) subdural collections suggesting the possibility of reduced intracranial pressure (Figure ). An MRI of the spinal cord demonstrated a large right-sided spinal arachnoid cyst at the level of T10/11 extending out through the neural foramen (Figure ). A right-sided pleural effusion was also noted suggesting that the cyst had ruptured into the pleural space.\nIn retrospect it seems likely that the patient's initial presentation was precipitated by spontaneous rupture of the spinal arachnoid cyst. Her persistent headache was the result of ongoing CSF hypotension.\nTwelve months after initial presentation our patient is well and her headache has completely abated. Her right-sided pleural effusion has resolved and she has not required any neurosurgical intervention. |
A previously healthy 57-year-old woman, with no significant past medical history, presented to the surgical department of our hospital for definite management of a primary pancreatic leiomyosarcoma, after being treated with adjuvant chemotherapy.\nOne year before her last admission, she was initially admitted to our emergency department due to abdominal pain, fatigue, and weight loss. She was totally healthy prior to these symptoms. She then underwent magnetic resonance imaging (MRI) that was indicative of a pancreatic head lesion along with possible metastatic liver lesions, superior mesenteric vein occlusion, and portal vein infiltration (Fig. a, b). The decision was to undergo an endoscopic ultrasound (EUS) biopsy in order to determine the exact nature of the lesion. EUS report was indicative of pancreatic leiomyosarcoma.\nMultidisciplinary team’s decision was to use gemcitabine- and docetaxel-based chemotherapy as up-front treatment to assess tumor response. Follow-up CT scan and magnetic resonance imaging (MRI) after the completion of chemotherapy regimen showed downsizing of the pancreatic mass, as well as downsizing of suspicious for malignancy segment III liver lesion (Fig. c, d).\nBased on the response to chemotherapy, tumor characteristics, and physical status of the patient, multidisciplinary team’s decision was to proceed to surgical exploration. Due to local expansion of the pancreatic tumor, its relation with the superior mesenteric and portal vein, and the underlying SMV thrombosis, excision of the pancreatic tumor was not feasible. Intraoperatively, a small piece of tumor was excised in order to be sent for histopathology. Surgeon’s decision was to ablate the tumor with irreversible electroporation (Fig. ). Metastatic liver lesions were identified with the use of intraoperative ultrasound. Segment III liver lesion was resected, while smaller lesions of the right lobe were ablated using microwave ablation.\nThe patient had an uneventful postoperative recovery and complete resolution of her symptoms. Histopathological examination of pancreatic lesion as well as segment III liver lesion revealed sarcomatous tissue of high cellularity with fascicular pattern, increased mitotic activity, and diffuse cytoplasmic immune reactivity for SMA, desmin and h-Caldesmon, and chromagen DAB (Figs. and ). Surprisingly enough, pathological report of a smaller liver lesion was indicative of angiomyolipoma staining positive for HMB45 and Melan-A. The lesion was a benign hamartomatous, circumscribed but unencapsulated hepatic mass composed mainly by mature lipocytes and limited mesenchymal component (smooth muscle cells), showing no marked atypia and thick-walled vasculature. Myoid component was positive for ΗΜΒ-45 and Melan-A. Based on the histopathological report, tumor board decided that the patient should be treated with adjuvant therapy for leiomyosarcoma after surgery. A regimen with anthracycline and olaratumab was used for 3 months. Follow-up imaging in 6 and 12 months showed no progression of the disease (Fig. a–d). |
Case 1: Twenty two years old male who sustained closed fracture of midshaft of both bones of forearm due to a fall from motorcycle (). Patient was taken up for surgery the next day under regional block after proper preanaesthetic check up. In our technique) a transverse incision was made over radial styloid along Langer’s lines. Care was made over radial styloid along Langer’s lines. Care was taken to protect the sensory branch of radial nerve and thumb tendons. Entry point was created with awl or 3.2 mm drill bit making it big enough to allow passage of two nails. Tip of flexible nail was prebent into a gentle curve. Nail was inserted into radial canal manually with a T handle. Insertion typically encounters resistance every few centimeters. Resistance can be overcome by minimally rotating the nail. Nail was passed up to fracture site. Closed reduction was achieved using C arm. Nail was passed across fracture site but not completely so as to allow manipulation for reduction of ulna fracture. For ulna, entry point was made on the radial aspect of ulnar shaft near olecranon (eccentric). A prebent nail was inserted in to the ulnar canal up to fracture site. Closed reduction was achieved and the nail was passed across up to ulnar head. Similarly radial nail was passed up to radial head. One more additional nail was passed in both radius and ulna (2 in radius and 2 nails in ulna). The nails were cut flush with bone. Reduction was found to be stable on table during prono-supination movements (). No immobilization was given post-surgery and physiotherapy was started the next day and patient was comfortably performing prono-supination from third day post-surgery (). Patient was followed up at 2 weeks, 6 weeks, 3 months, 6 months and 1 year. Patient had minimal pain post operatively and achieved full prono-supination and wrist and elbow range of movements by 2 weeks. He was able to return to his computer work in 2 weeks. Callus was seen by 3 months and fractures were completely united by 6 months (). At one year follow up the bone showed good union and functionally patient was able to do all activities (). |
A 25-year-old female with no known co-morbidities or history of previous illness or trauma presented to the neurosurgery outpatient department. According to her, she was well three months back, when she suddenly developed episodes of severe left frontal headache which were continuous for hours to days. It was throbbing in character not associated with symptoms of nausea, vomiting, dizziness or fits. At many occasions, she complained of double vision with severe headache. For the same length of time, she also noticed that her left eye was bulging outwards along with pulsation in the same protruded eye which gradually increased and since one month it had been so obvious that anyone looking at her could appreciate it.\nHer general physical examination was unremarkable. She was conscious and well oriented to time, place and person. Her higher mental functions, gross motor, fine motor and sensory functions were intact. On local examination of the left eye, there was non-axial pulsatile proptosis with outward depression of the left eyeball. On visual acuity, she was assessed to be emmetropic with diplopia in her left eye. Ocular movements in both eyes were normal. There was no tenderness on palpation but pulsations were felt on left eye. No bruit was heard on auscultation.\nComputed tomography (CT) scan of the brain with contrast study showed deficient bone on left orbital roof and floor with left frontal gliotic brain compressing the eyeball causing it to move out and down (Figure ). Her magnetic resonance imaging (MRI) brain and orbit with contrast study showed an asymmetrical deformity of the skull and left cerebral hemisphere which was bulging towards the left orbit, causing extrinsic pressure resulting in proptosis of the eyeball (Figure ). It also showed widened cortical sulci due to decreased volume. There was indirect evidence of widening of left lateral ventricle and Sylvian fissure due to hemi-atrophic changes. Contrast studies did not elucidate any abnormal enhancement. Right orbit, right hemisphere, brainstem, and cerebellum appeared normal. There was no evidence of demyelination or any intracranial mass. The affected orbit was larger and downwards than normal. There was an absence of floor and roof of the left orbit.\nWith this information, a working diagnosis of orbital meningoencephalocele was made and a maxillofacial consult was taken. The patient and her family were counseled regarding the condition and about our plan of surgical intervention that included a two-staged procedure. Left frontal craniotomy was done as a part of the first stage. Per-operatively after opening the dura, frontal lobe was retracted without violation of the arachnoid. The retraction assisted in determining the bony defect. Then a titanium mesh was used to reconstruct the orbital roof and was fixed with screws over the inner surface of superior orbital rim. Duroplasty was performed and the frontal bone flap was secured. A subgaleal drain was then placed and the wound was closed in layers. The drain was removed at the first post-operative day and she was mobilized and discharged on third post-operative day. Her post-operative CT scan with three-dimensional (3-D) reconstruction confirmed the appropriate placement of metallic prosthesis covering the bony defect in the roof of the left orbit (Figures -).\nAt her two-week follow-up, her diplopia and headache had considerably improved and the proptosis had resolved with no visible pulsations. She is planned for second stage surgery after three months in which we plan to reconstruct the orbital floor. |
A 33-year-old woman presented at the emergency department with a subacute progressive bilateral leg weakness. The neurological examination revealed a severe paraparesis, as well as hypesthesia of both lower limbs in the absence of sphincter incontinence or radicular pain. Reflexes were hyperactive in both legs, with no evidence of clonus or Babinski sign. The patient had been diagnosed 13 months before with a temporal lobe GBM (Fig. ). She had undergone surgical gross total tumor resection and postoperative MRI scans had revealed a very small residual contrast-enhancing abnormality (Fig. ). Postoperatively, the patient had no other remarkable neurological deficits besides a light remaining right hemiparesis and had undergone concomitant radio- and chemotherapy followed by 6 cycles of adjuvant temozolomide. Nevertheless, the doses of temozolomide had to be reduced due to a severe thrombocytopenia developed by the patient during the treatment.\nBrain MRI at the time of consultation due to paraparesis showed no evidence of a local intracranial recurrence (Fig. ). However, MRI of the spine demonstrated a homogeneous contrast-enhancing mass within the spinal canal at the Th8/9 level causing significant spinal cord compression (Fig. ). A lumbar puncture was performed and cytological examination of the cerebrospinal fluid (CSF) did not reveal the presence of neoplastic cells.\nDue to the spinal cord compression and progressive paraparesis, we suggested surgery with the aim of decompressing the spinal cord and if possible remove the tumor. Therefore, we performed a decompressive laminectomy at the Th8/9 level. After opening the dural sack, an intradural intramedullary high vascularized infiltrating tumor was identified (Fig. ). Due to its adherent and infiltrating pattern with no recognizable edges to the normal tissue, it was unreasonable to attempt a radical tumor excision so we carefully performed a biopsy and interposed a dural graft to expand the spinal canal, therefore reducing the pressure against the spinal cord (Fig. ).\nUnfortunately, neurological deficits did not improve during the postoperative follow-up and the patient refused to undergo further oncologic therapies. She underwent best supportive care and died 5 months later due to complications of pneumonia acquired in the context of a highly debilitated general state of health. |
A 68-yr-old man was transferred to the emergency room because of sudden postprandial chest pain, which had been aggravated during the previous two days. He had a history of stroke, hypertension and diabetes mellitus for 3 yr, during which time he had been taking antihypertensive, antiplatelet, and antidiabetic medications. Blood pressure was 110/70 mmHg, pulse 120 bpm, and temperature 39℃. Physical examination demonstrated a subcutaneous emphysema in the neck. Laboratory data included white blood cell count of 16,400/µL, hemoglobin value 11.3 g/dL, and platelet count 273×103/µL. Chest radiograph revealed a small air shadow in the neck (). Chest computed tomography showed periesophageal fluid collection in the lower esophagus, more prominent air collection at the left lateral aspect of the lower esophagus, and bilateral minimal pleural effusion (). The radiologist suggested an esophageal perforation and recommended an esophagogram with gastrographin. The esophagogram revealed a perforation with a bulge of 2.5 cm in width in the lower thoracic esophagus (). The patient was emergently operated on, with primary closure for perforation, and proper drainage with insertion of thoracostomy tubes. At the time of surgery, after esophageal dissection and debridement, a perforated esophageal diverticulum was identified and the perforation was revealed in the borderline between mucosal protrusion and muscular fiber (). The mucosal edge of the perforation was identified and approximated using interrupted 4-0 vicryl sutures, and the muscular edge was closed using interrupted 3-0 silk sutures. The closure was reinforced by stripping a large flap of pleura from the posterolateral thoracic cavity and stitching it to the muscular coat of the esophagus all round the sutured perforation. The chest was closed with two thoracostomy tubes. Despite the use of broad spectrum antibiotics postoperatively, the patient's condition deteriorated and follow-up radiography revealed markedly aggravated pulmonary edema, cardiomegaly, and widened mediastinum () until the 2nd postoperative day. After using cardiotonic and diuretics, the patient was improved his symptom and radiography. A gastrographin swallow study obtained on the 14th postoperative days showed the disappearance of the diverticulum, although there remained small and confined leak from the esophagus (). The patient was managed with non-surgical treatments, such as intravenous hyperalimentation and broad-spectrum antibiotics. A gastrographin swallow study performed on the 42nd postoperative day showed the absence of leaks, and the patient was discharged 2 months after the operation. |
A 68-year old male with a history of seminoma, currently with no evidence of disease, was referred to our surgical group after a routine check-up abdominal ultrasound (US) revealed a tumor in the lesser curvature of the stomach. An abdominal CT scan with oral and IV contrast confirmed a 5 cm homogenous mass in the lesser curvature of the stomach; there was no suspicious lymphadenopathy and no other suspicious lesions were identified. Patient was completely asymptomatic at the time, referring no weight loss, no changes in diet or appetite, as well as normal GI function. The patient’s case was discussed in a multidisciplinary tumor board and a laparoscopic resection was recommended ().\nUnder general anesthesia, a laparoscopic exploration confirmed a 5 cm mass in the lesser curvature of the stomach, no suspicious lymphadenopathy and no other lesions were identified. An intraoperative upper GI endoscopy did not identify any other suspicious lesions on the upper GI tract. Using the laparoscopic technique, the gastroesophageal junction was retracted anteriorly and, using a harmonic scalpel, access to the lesser omentum was obtained. Using a 60 mm endoscopic stapler, the tumor was completely resected with 1.5 cm margins, and extracted with an endoscopic bag. Intraoperative pathology confirmed a spindle cell neoplasia with negative margins. A new upper GI endoscopy confirmed that the diameter of the gastric lumen had not been significantly reduced and that the staple line was airtight. No complications were reported. The patient was started on a soft diet the following day. His postoperative recovery was uneventful, and he was discharged home on postoperative day 3 (, , ).\nThe pathology report confirmed a 5.1 × 2.1 cm, lobulated, white-yellow tumor, with well-defined margins. In the immunohistochemical analysis, the tumor was positive for PS100 and SOX10, and negative for CD117 and DOG-1, confirming diagnosis of gastric schwannoma. The tumor was completely resected, and all margins were confirmed clear of tumor. At a one-year follow-up, the patient remains completely asymptomatic and a follow up CT shows no evidence of tumor recurrence. |
A 27-year-old Caucasian woman was admitted to the Emergency Department of our Institution because of bloody diarrhoea – up to 10 bowel movements per day – during the last month, 3 weeks after quitting smoking. Physical examination showed no abnormalities but confirmed haematochezia on digital rectal examination. Colonoscopy showed continuous severe colonic inflammation with small ulcers from the anus to the descendent colon, classified as grade 3 in Mayo endoscopic sub-score and 3 points in Ulcerative Colitis Endoscopic Index of Severity (UCEIS); complete examination was not performed because of the risk of perforation. Empirical antibiotic treatment with ciprofloxacin and metronidazole, as well as oral and rectal mesalamine were started and partial symptomatic improvement was achieved. Venous thrombosis prophylaxis with subcutaneous enoxaparin, 40 mg per day, was started. At admittance, haemoglobin, white cell count, platelets, fibrinogen and C reactive protein (CRP) were within the reference range. Stool cultures were negative. Cytomegalovirus (CMV) infection was also ruled out in colonic biopsies (polymerase chain reaction – PCR – and, later, immunohistochemistry). As bloody diarrhoea persisted 48 h later, and histopathological examination of colonic biopsies showed crypt distortion, a mixed inflammatory infiltrate of the lamina propria and crypt abscesses suggesting the diagnosis of UC, intravenous methylprednisolone (1 mg per kg of weight, daily) was started. After 3 days of corticosteroids the patient achieved partial clinical response (6 bowel movements per day, Edinburgh index 2 points, CRP within the normal range); nevertheless, 2 weeks later infliximab therapy (5 mg/kg of weight) was started due to sustained clinical activity, with 10 bloody bowel movements per day and a progressive increase of CRP levels, up to 10 mg/dL. Three days after the first dose of infliximab, the patient presented a massive lower bleeding with haemodynamic instability and severe anaemia; CT scan showed active arterial haemorrhage from ascendant colon; a subsequent arteriography demonstrated active arterial bleeding from a colic branch of the superior mesenteric artery; selective transcatheter embolization with platinum microcoils (MicroNester©, Cook Medical) was performed with immediate technical success; nevertheless, the patient persisted with rectal bleeding 2 days after embolization, requiring laparoscopic subtotal colectomy and ileostomy. Pathological evaluation of the colon confirmed the diagnosis of UC. Eight days after surgery the patient was discharged. |
A 1-year-old girl was presented to a hospital in the northeastern part of Ghana with a 1-week history of pustular rashes on her scalp and neck, which occasionally ruptured, along with discharge of yellowish purulent fluid. The child is of Mole-Dagbon ethnicity and hails from the northern part of Ghana. The patient had also experienced recurrent episodes of fever for 3 days, which had been controlled with the use of acetaminophen. The child had no known history of human immunodeficiency virus (HIV) infection, tuberculosis, or other underlying medical condition and lived with her parents, whose primary occupation was farming. The child lives in a rural area about 20 km from the hospital. On examination, the patient weighed 8 kg, was pale and febrile with a temperature of 39.7 °C, and well-hydrated. Her heart rate was 132 beats per minute, and her heart sounds were normal. Her lungs were clear, and her respiratory rate was 35 breaths per minute. A provisional diagnosis of sepsis and malaria was made.\nPending the results of laboratory tests of collected blood, urine, and stool samples, the patient was empirically treated with 270 mg of intravenous cefuroxime three times daily and 40 mg of intravenous gentamicin daily for 48 hours. Intravenous artesunate 24 mg was also administered for the first 24 hours (Table ).\nComplete blood count investigations showed a hemoglobin concentration of 6.6 g/dl and a total white blood cell count of 8800 cells/μl (lymphocytes 52.1%, neutrophils 37.4%). The result of a malaria rapid diagnostic test was positive for malaria. The patient was given a hemotransfusion of 240 ml of whole blood over a period of 4 hours.\nResults of both urine and stool cultures were negative for any bacterial growth. The blood culture sample was flagged as positive after 24 hours of incubation in an automated BD BACTEC 9050 blood culture system (BD Biosciences, San Jose, CA, USA), and a Gram stain of the culture broth revealed gram-negative rods. The broth was subcultured on blood agar, chocolate agar, and MacConkey agar (Oxoid Ltd, Basingstoke, UK) and incubated at 37 °C and 30 °C. Growth characteristics at 37 °C were poor, with grayish nonhemolytic colonies on blood agar and nonlactose fermentation on MacConkey agar. Plates incubated at 30 °C showed good pure growth on MacConkey agar and nonhemolytic colonies on blood agar. Results of biochemical investigations were negative for oxidase and urease and positive for citrate. The isolate was subjected to analytical profile index (API) 20NE and 20E (bioMérieux, Marcy-l’Étoile, France), which yielded unacceptable results.\nMolecular identification was performed by extracting deoxyribonucleic acid (DNA) from a pure culture of this bacterium using SpheroLyse DNA extraction kit (Hain Lifescience GmbH, Nehren, Germany) according to the manufacturer’s instructions. The 16S ribosomal DNA (rDNA) was amplified using the primer pair 8F and 1492R []. The polymerase chain reaction conditions and the subsequent steps of Sanger sequencing we used were based on those described by Liu et al. []. The resulting 16S rDNA sequence was checked using DECIPHER (version 2.2.0; R Foundation for Statistical Computing, Vienna, Austria) to verify that it was not a chimera [].\nThe procedure for molecular phylogenetic inference was based on that described by Ku et al. []. Briefly, the 16S rDNA sequence of the isolated strain was used as the query for sequence similarity search against the GenBank 16S rDNA sequence database. Highly similar sequences representing closely related species were selected for phylogenetic inference. All sequences were aligned using Multiple Sequence Comparison by Log-Expectation (MUSCLE) [], and the resulting multiple sequence alignment was used to infer a maximum likelihood phylogeny using PhyML (version 3.0) []. The bootstrap support values were inferred based on resampling 1000 iterations.\nThe isolated strain reported in this study was found to be most similar to Pseudomonas oryzihabitans IAM 158 [GenBank accession NR_115005] with nucleotide sequence identity of 1455/1460 (99.7%). The molecular phylogeny also indicated that the new sequence is clustered with other P. oryzihabitans strains, which strongly supports the findings displayed in Fig. . The purified bacterial strain has been assigned the strain name Agogo to reflect its geographic origin and the sequence deposited in the GenBank database [accession number KX812763].\nAntimicrobial susceptibility testing was done on the isolated bacteria using the Kirby-Bauer disk diffusion method following Clinical Laboratory Standards Institute guidelines []. The isolate was found to be susceptible to co-trimoxazole, chloramphenicol, ciprofloxacin, and gentamicin but resistant to ampicillin and cefuroxime. The rash on the patient’s scalp and skin, as well as her temperature spikes, resolved after continuous treatment with gentamicin, and her condition improved clinically. She was discharged on flucloxacillin and folic acid therapy 1 week after admission. A final diagnosis of septicemia secondary to bacterial skin infection with malaria coinfection was made. |
Case of patient 1
An 18-year-old male with no past medical or surgical history presented to a free-standing clinic with the chief complaint of right testicular pain. Symptoms started one hour prior to arrival, was exacerbated by palpation, relieved by nothing, was sharp and non-radiating, rated 10/10, and was constant, with no associated symptoms. The patient was sitting at home playing video games and drinking a few beers when he got up and started having intense right testicular pain. He was asymptomatic before that time and had no history of trauma, minor or otherwise. He came straight to the emergency department after symptoms developed. A scrotal ultrasound was ordered immediately. Ultrasonography (Figures -) demonstrated the right testicle to have no sonographic demonstration of color flow, enlargement of the right epididymis, and hydrocoele. The left testicle demonstrates arterial and venous signals on color flow, a small left epididymal cyst, and a small left hydrocele.
The transfer center was called and the patient was sent to our facility for urology consultation. The patient arrived at our facility four hours after symptoms started and urology was consulted immediately. The review of systems was positive only for right testicular pain. Physical examination was abnormal only for a hard, high-riding right testicle with swelling and absent cremasteric reflex. The patient was taken straight to the operating room where the right testicle was found to be dark and rotated 720 degrees. After detorsion, the testicle started taking back some color so a bilateral orchidopexy was performed and the patient was discharged home the next day with an improvement of symptoms.
Case of patient 2
A 24-year-old male with no past medical or surgical history presented to our hospital with the chief complaint of right testicular pain. Symptoms started 24 hours prior to arrival, exacerbated by movement, relieved by nothing, sharp, radiating to the right flank, rated |
The proband was a 35-year-old male from Hebei province in the north of China. He is of Han ethnicity and was born to consanguineous parents. His family pedigree is shown in Fig. . The proband was normally delivered after a full-term pregnancy, and birth weight and length were within normal ranges. The initial signs and symptoms appeared when he was 6 years old. Deformity of interphalangeal joints initially appeared in the fingers. Hips, knees, and wrists were then gradually involved. Diagnosis of JRA was considered by local hospitals, and glucocorticoids were prescribed without any efficacy. As he grew up, his symptoms deteriorated. He had to walk with crutches at 16 years of age because of arthritis of the lower extremities. At 26 years of age, he first experienced progressive pain with numbness radiating down his entire left leg and right thigh. At 34, he started to have mild difficulty in urination. Thereafter, his leg pain progressed and he became immobile. Treatment with tramadol, physical therapy, and spine injection were tried but were not effective. He had a younger brother with a similar clinical presentation but who also had mild neurological impairment (Fig. ).\nThe proband’s height and weight were 162 cm and 72.5 kg when he was admitted to our hospital. His visual analogue scale (VAS) score was 9. He did not have behavioral difficulties and was not retarded in his intellectual development. Physical examination showed multiple malformations of the major limb joints, especially of the knees and hands (Fig. ). Amyotrophy of both lower limbs was obvious. Cervical and lumbar movements were limited with compensatory kyphosis. The muscular strength of all four limbs was normal. Dysesthesia was found in the posterolateral left calf, dorsolateral left foot, and perineal area. Bilateral knee-jerk reflexes and ankle reflexes were hypo-induced. The erythrocyte sedimentation rate (13 mm/h) and C-reactive protein level (2 mg/L) were both within the normal range. Tests for rheumatoid factors were negative.\nSpinal x-rays showed flat and osteoporotic vertebral bodies. Pedicles were short, and end plates were irregular. Bone bridges were seen at many levels. Kyphosis was detected in both the cervical and upper thoracic spine. Magnetic resonance imaging showed multilevel Schmorl nodes. Multilevel disc herniation and hypertrophic ligamentum flavum caused lumbar canal stenosis from L2 to S1 (Fig. ). |
A 7-yr-old girl visited our outpatient clinic complaining primarily of shortening of her left upper extremity and limitation of the right shoulder motion, which were detected 2 yr ago. She had been taken care of by her college-educated mother since birth, who stated that the patient had been very healthy without any serious illness. Her medical record did not show any significant health problem. Her height was 124 cm (70% among the age matched normal population), and her weight was 31 kg (95%). She had a round face and short neck, and her teeth showed signs of yellowish discoloration. Her hands and feet were short and broad, and all of her fingers and her 3rd, 4th and 5th toes of both sides were short. More than 10 subcutaneous calcifications were detected in the hands, chest wall and abdominal wall. A physical examination at age 8 yr and 10 months revealed 10 degrees active and 40 degrees passive forward flexion of the right shoulder. The active and passive abductions of the right shoulder joint were 50 and 100 degrees, respectively. Passive external rotation was 50 degrees on the right and 90 degrees on the left. The distance from the acromion to the cubital crease measured 16 cm on the right side and 21 cm on the left side (). Radiographic examination showed that the round but flattened humeral head was retroverted with a varus orientation (). All of the metacarpals and the phalanges of the hand were short and broad with coned epiphyses, while the 3rd, 4th and 5th metatarsals were short as compared with the 1st and 2nd metatarsals (). There was no calcification in either the brain or the kidney, as determined by MRI and ultrasonography.\nBiochemical study revealed hypocalcemia (6.9 mg/dL), hyperphosphatemia (8.1 mg/dL) and the serum level of intact parathyroid hormone was 552 pg/mL (reference, 10-65 pg/mL). The thyroid function test showed the presence of a euthyroid state while the serum level of thyroid stimulating hormone (TSH) was elevated to 13.0 IU/mL (reference, 0.4-4.1 IU/mL). Serum levels of lutenizing hormone (LH) and follicle stimulating hormone (FSH) were normal. Her skeletal age was advanced for her chronologic age, and her intelligence quotient was 75 at age 10 yr.\nSurgical treatment was performed at age 8 yr and 10 months. Medical treatment with calcium carbonate and active vitamin D3 was started on the day before the operation and continued thereafter. Proximal humeral valgus osteotomy was fixed with Ilizarov apparatus, and the humerus was lengthened at the distal corticotomy site. Under image-intensifier the position of the shoulder joint to obtain the optimal glenohumeral articulation was deteremined to be valgization 65 degrees, internal rotation 30 degrees and forward flexion 10 degrees. Osteotomy was performed at the surgical neck, and the amount of planned correction could be achieved by controlling the Ilizarov rings. Distal corticotomy was made at the distal metaphysiodiaphyseal junction via anterolateral approach, identifying and protecting the radial nerve. After 7 days of latency period, gradual lengthening at a rate of 0.75-1.0 mm/day was carried out. The external fixator was removed at postoperative 5.5 months with an overall length gain of 10 cm, resulting in a healing index of 0.55 month/cm (). After four years of follow-up, both proximal humeral physes were closed, and the residual shortening of the right humerus was 1.2 cm (). She could actively abduct her right shoulder to 75 degrees, flex forwardly to 60 degrees. Passive abduction was 120 degrees and passive forward flexion was 150 degrees. The range of motion of the right elbow was not decreased. The patient stated that she had experienced an improvement in her daily life such as face washing, eating and combing. |
The second case refers to a 48-year-old woman, a busy manager with a history of depression and sleep disturbance. She has had three terminations of pregnancy and one delivery by cesarean section. She smokes approximately ten cigarettes per day and has high cholesterol serum levels. She takes several medications: a selective serotonin reuptake inhibitor (escitalopram), two benzodiazepines (delorazepam and clonazepam), and a statin. She reports a four-year history of urinary symptoms: daily UUI episodes, mild stress urinary incontinence (SUI), and two episodes of nocturia per night. She wears pads every day. The urology consultation revealed some degree of pelvic pain, especially during vaginal examination. The urine dipstick was negative and there was no PVR. No specific causes of the symptoms such as urine tract infection were identified. The patient also complained of mild dyspareunia and occasional constipation. The urine culture turned out to be sterile, with no blood in urine, and the pelvic ultrasound scan and urine cytology were also negative. The cystoscopy, which was performed as a result of the presence of storage symptoms and to rule out a bladder tumor in this current smoker, was normal.\nIn OAB patients, it is of utmost importance to consider all comorbidities. Anxiety and depression may play a role, feeding a vicious circle. Moreover, medications to treat neurological or psychiatric disorders can influence OAB and be responsible for side effects [, ]. Gastrointestinal disorders are frequently associated with OAB, such as constipation in this case, but patients rarely raise the topic. An overlap exists between irritable bowel syndrome and OAB [].\nThe patient was prescribed a β3 agonist, pelvic floor muscle training (PFMT) and bladder retraining. Four months later, she noticed some degree of improvement, but had stopped the treatment as she felt that she had no time for PFMT. She was not compliant with the bladder drill either, and soon stopped the β3 agonist because she did not sense any real improvement. She also felt that she did not have the time to complete a bladder diary. She was prescribed fesoterodine 8 mg for three months. In parallel, her general practitioner asked for vaginal and urethral culture swabs, which were negative. After three months, her urinary urgency improved, but she said that the few remaining episodes of urgency were “killing her life” and that she did not want to be on pills for her whole life. Therefore, she refused to continue the treatment and requested an “easy fix”. Her reaction highlights the need for careful consideration of the consequences of incontinence in terms of QoL. A publication from Vaughan et al. [] reported that OAB and incontinence synergize to reduce QoL, especially in the domains of sleep, elimination, usual activities, discomfort, distress, vitality, and sexual activity.\nConsistent efficacy on urgency symptoms with a significant decrease in UUI and urgency episodes has been reported with fesoterodine at doses of 4 and 8 mg compared to placebo () [, , , ]; however, some patients may react differently. Patient satisfaction is an important driver of treatment success []. Patient expectations should be considered carefully in the context of OAB management. The achievement of patients' goals was measured in the Study Assessing FlexIble-dose fesoterodiNe in Adults (SAFINA study) [], a 12-week multicenter open label study with 331 OAB adults, using the Self-Assessment Goal Achievement (SAGA) questionnaire. Fesoterodine treatment resulted in 81.3% of patients declaring that their goals were “somewhat achieved/achieved” or that the result “exceeded/greatly exceeded their expectation”.\nOur case patient had very specific expectations; she refused to have an implant (neuromodulation), saying “I'm not going to be an android!” She accepted botox injections, and so a first set of injections was performed under local anesthesia. She found the injections “a little painful” and “a big annoyance”, but at the one-month follow-up visit after botox injection she reported no more UUI episodes and an improvement in frequency and the number of urgency episodes, as well as in QoL. Even though she stated that she did not like the idea of being a patient for the rest of her life, she accepted subsequent injections.\nThe clinical points that can be learned from this case are as follows:All OAB cases are different, and a thorough evaluation is mandatory to adequately address each case. It is important to assess other aspects, such as functional and psychological disorders that may influence symptoms, and to consider nonneurogenic OAB as a multifactorial disease. The major goal of initial therapy is to meet the patient's expectations regarding the reason for their visit, to improve their satisfaction, and their QoL. Due to fesoterodine's characteristics and flexible dosage, improvement of symptoms and achievement of the patients' goal are usually high with this medication. When patients have specific requirements, all options should be discussed and the patient's agreement obtained. A customized approach is a crucial factor for treatment success. OAB management should be personalized; beware of a simplistic application of a standardized treatment algorithm. |
A 79-year-old man with right hemiparesis was admitted to our hospital for examination of a growing abnormal shadow on a chest radiograph. He had experienced rickets as a child and had been treated for hypertension, mild chronic obstructive pulmonary disease, cerebral infarction, and cerebral hemorrhage for 20 years. His height, weight, and body surface area were 133 cm, 44.2 kg, and 1.25 m2, respectively. He also had pigeon breast. Computed tomography (CT) angiography demonstrated a saccular aneurysm of the descending thoracic aorta with a maximum diameter of 55 mm. The thoracoabdominal aorta adjoining the aneurysm possessed a bend of 180°, and the right brachiocephalic artery was extremely elongated and tortuous (Fig. ). Transthoracic echocardiography revealed moderate aortic valve regurgitation. Considering respiratory failure by left thoracotomy and his medical history such as cerebral infarction and cerebral hemorrhage, the patient was deemed unsuitable for performing a traditional graft replacement. After informed consent was obtained from the patient, we selected TEVAR under a ministernotomy.\nAlthough the iliac arteries were suitable for the introduction of a sheath for the chosen stent graft, we were concerned that the tortuous thoracoabdominal aorta would prevent safe retrograde delivery of the stent graft to its intended location. The right brachiofemoral through-and-through wire technique was not feasible because of a high risk of vascular injury associated with the severely tortuous right brachiocephalic artery. The use of a transapical approach was considered difficult because of the hemodynamic collapse caused by aortic regurgitation. After considering the available options, we decided to deliver the stent graft from the ascending aorta, using the ascending aortofemoral through-and-through wire technique.\nUnder general anesthesia and transcranial Doppler and cerebral oximetry monitoring, we performed an inverted T ministernotomy below the second intercostal space. Because the diameter of the ascending aorta was >40 mm, we prepared the conduit in the ascending aorta under partial cardiopulmonary bypass. A 10-mm woven Dacron graft was anastomosed in an end-to-side fashion to the ascending aorta, using 4–0 monofilament sutures under partial clamping. A 5-Fr sheath was placed in the graft, and a 400-cm, 0.035-inch hydrophilic guidewire was inserted through a pigtail catheter to the descending aorta. The wire was snared within the abdominal aorta and externalized through the right common femoral artery. Subsequently, the 5-Fr sheath in the graft was exchanged to a 22-Fr sheath (dry seal sheath, W.L. Gore & Associates, Inc.). Aortography was performed using the pigtail catheter, which was introduced beside the stent graft into the large sheath. A 31 × 150-mm conformable GORE TAG stent graft (W.L. Gore & Associates, Inc., Flagstaff, AZ, USA), which was designed to be released from the middle position, was advanced in the antegrade direction across the aortic arch and subsequently deployed distal to the left subclavian artery by repeated pull-loosen technique with both sides of the wire (Fig. ). No endoleak was detected by angiography after performing touch-up via inflation of a balloon (tri-lobe balloon catheter, W.L. Gore & Associates, Inc.). The patient’s postoperative course was uneventful and without major complications such as cerebrovascular accidents, vascular access issues, respiratory failure, cardiac failure, wound infection, or paraplegia. Postoperative CT angiography demonstrated complete exclusion of the saccular thoracic aneurysm (Fig. ). The patient has been discharged from hospital and is now undergoing monthly follow-up examinations.\nCurrently, thoracic endovascular aortic repair is the first treatment of choice in patients with aneurysms of the descending thoracic aorta, depending on the condition of the patient, demographic factors, and suitable anatomy for stent-graft deployment. When there is an abnormal anatomical characteristic, such as severe aortic tortuosity, technical success may not be possible using ordinary maneuvers involving a stiff guidewire alone and delivery of stent grafts from femoral arteries in an antegrade direction. Furthermore, a catastrophic situation caused by thrombus or plaque embolization and rupture of calcifications can occur. In such anatomically challenging cases, it is important to carefully consider the choice of access route, the use of adjuvant guidewire technique, and the need for adjunctive surgical interventions. Several adjuvant techniques have been reported as means of overcoming these issues. The use of stiff “buddy” wires and the introduction of a coaxial sheath should be performed primarily to straighten an excessively tortuous aorta, but delivery of the stent graft can be difficult. The through-and-through wire technique, which pulls both sides of an externalized guidewire with adequate tension and straightens the severely tortuous aorta, is a powerful means of delivering stent grafts []. The through-and-through wire technique from the right brachial artery to the femoral artery was not feasible in the present case because the right brachiocephalic artery was severely tortuous []. The left common carotid artery and left subclavian artery did not allow the use of an externalized guidewire because of multiple severe calcifications around the supra-aortic vessels. Joseph et al. described a technique to create a transseptal externalized guidewire loop []. It was thought that this technique would result in acute deterioration of valve regurgitations caused by the indwelling guidewire. Furthermore, Saouti et al. described the efficacy of a transapical approach, which is often available in transcatheter aortic valve replacement []. This approach can lead to several complications such as respiratory failure by left thoracotomy, hemodynamic collapse due to aortic regurgitation, and ventricular pseudoaneurysm formation. Ultimately, we decided to perform antegrade TEVAR from the ascending aorta with a tube graft conduit.\nBecause the maximum diameter of the ascending aorta was >40 mm, we decided to prepare the conduit to prevent uncontrollable bleeding and unexpected aortic dissection. To reduce the risk of intraoperative stroke, we ensured that the tip of the large sheath did not damage the wall of the atherosclerotic aorta. Debranching of the supra-aortic vessels in combination with TEVAR is performed during median sternotomy and right anterior minithoracotomy. The right anterior minithoracotomy approach is appropriate in selected high-risk patients with prior cardiac surgery []. The application of a ministernotomy for preparing a conduit appeared to be a safe and reliable method, particularly in the present case with multiple comorbidities, although the potential advantage of endovascular treatment declined in the view of less invasive surgical options.\nChen et al. reported that high tortuosity of the thoracic aorta is associated with higher occurrence of endoleaks and lower survival in patients undergoing TEVAR for atherosclerotic aneurysms []. Moreover, it has been speculated that perioperative complications related to TEVAR show significant associations with tortuousity. It was conceivable that the use of a hydrophilic guidewire would contribute to the compatibility of stent graft placement for a tortuous aorta compared with the use of a stiff guidewire. Furthermore, it was considered that delicate manipulation by repeating pull-loosen with both sides of the hydrophilic guidewire could play an important role in minimizing the damage of the atherosclerotic aorta by the stent graft.\nWith respect to the precise deployment of the stent graft to its intended position, there are several adjuncts including right ventricular rapid pacing, administration of adenosine or vasodilators, balloon inflation in the inferior vena cava, and through-and-through bowing technique []. In addition, it was difficult to determine the accurate proximal sealing zone when performing the reversed deployment of the stent graft. Although the use of a hydrophilic guidewire prevented the stent graft from pressing against the greater curvature of the thoracic aorta, the longer stent graft coverage was thought to obviate migration of the stent graft and the occurrence of endoleaks by taking advantage of the anatomical characteristics in the present case, i.e., the aneurysm existed at the top of a sharply curved thoracic aorta. |
The patient was a 44-year-old woman with a lifetime history of low VWF and clinically significant bleeding manifestations. Her disease manifested with excessive bleeding including menorrhagia and epistaxis. Laboratory evaluation confirmed type 1 VWD with ristocetin cofactor activity of 38%, von Willebrand antigen level of 41%, factor VIII level of 60%, and a normal von Willebrand factor multimer distribution. The patient had type A blood and abnormal Platelet Function Assay (PFA) markedly prolonged clotting times with both collagen/epinephrine and collagen/ADP stimulation. The patient previously underwent a DDAVP challenge test which more than doubled her ristocetin activity after administration of 20 mcg of intravenous DDAVP. This challenge test was not followed by any complications. She presented to the pain clinic with a long-standing history of low back and bilateral lower extremity pain. Over the years, she had tried a variety of medications, multiple episodes of physical therapy, and other conservative care without significant improvement. MRI imaging of the lumbar spine revealed an annular tear and disc bulge at L5-S1. After discussion of risks versus benefits of the procedure and the need for consideration of DDAVP to reduce the risk of bleeding, the patient underwent an infusion of DDAVP 0.3 mcg/kg receiving a total of 22 mcg over a period of 30 minutes immediately before the procedure. The patient was not given any specific instructions for fluid restriction postoperatively. Bilateral L5-S1 transforaminal epidural steroid injections were carried out. Once needle position and good contrast spread along the nerve roots were confirmed, an injection of 1 mL of 0.25% bupivacaine and 40 mg methylprednisolone was carried out on each side. Although the procedure was performed without incident, the patient experienced persistent dizziness and a nonpositional headache early the next day. Thorough investigation, including serial serum and urine electrolyte evaluations, revealed findings consistent with hyponatremia (serum sodium: 125 mmol/L), which was a decrease from baseline serum sodium of 138 mmol/L. Further investigation also revealed excessive water intake of 80 ounces per day that may have contributed to the acute onset of hyponatremia. The electrolyte disorder was gradually corrected over a period of 24 hours through fluid restriction and administration of sodium with complete resolution of symptoms. Serum sodium went from 125 mmol/L to 128 mmol/L at the 6th hour after diagnosis, to 134 mmol/L at the 10th hour, and then to 139 mmol/L at the 18th hour after diagnosis and remained at 136–139 mmol/L for the next several days to weeks on outpatient follow-up. |
A 67-year-old man with a history of chronic obstructive pulmonary disease, cerebral vascular accident, necrotizing pancreatitis complicated by pseudocyst requiring splenectomy and heart failure with preserved ejection fraction was transferred to our hospital following one month of treatment for pneumonia. He was a distant alcoholic but had since gone through rehabilitation and admitted to drinking one time per week and smoking four cigarettes a day. He had previously presented to his primary care physician with fever and malaise and was diagnosed with community-acquired pneumonia. He was treated with five days of azithromycin. He continued to worsen, and was admitted to an outside hospital with hypoxemia and right lower lobe pneumonia for which he was started empirically on vancomycin and piperacillin-tazobactam. His hospital course was complicated by respiratory failure requiring intubation for three days and a recurrent exudative right lung loculated effusion that required decortication and placement of a catheter that remained in place for two weeks. All blood and pleural fluid cultures were negative.\nOn transfer to our hospital for physical rehabilitation, the patient complained of mild shortness of breath. He denied hemoptysis, chest pain, orthopnea, nausea, chills or night sweats. Physical exam was significant for bilateral rhonchi with signs of consolidation in the right lower lobe. His labs were notable for a white blood cell count (WBC) of 17,000 cells/mcl with 87% neutrophils, and a chest radiograph revealed a right middle lobe infiltrate. He was continued on intravenous (IV) vancomycin and piperacillin-tazobactam at admission. Over the next two days his WBC climbed to 21,000 cells/mcl. Computed tomography scan of the chest revealed a right-sided empyema with extensive bilateral airspace disease consistent with severe pneumonia. A new chest tube was placed, which drained dark brown exudative fluid with gram-positive cocci on gram stain. The fluid was cultured and grew E. faecium resistant to ampicillin and vancomycin but sensitive to linezolid, gentamicin and streptomycin. The patient was started on linezolid and improved over the next two weeks, with resolution of the chest tube drainage. |
A 25-year-old woman with poor vision in her left eye since early childhood and high myopia in her right eye (refractive error; -11 D) presented with a history of sudden deterioration of vision in her left eye for two days. Her medical and family histories were unremarkable.\nFull ocular examination was performed. The best corrective visual acuity of her right eye was 20/20 with correction of -11 D, while that of her left eye was hand motion with correction of -19.75 D. Intraocular pressure was 15 mmHg in the right eye and 17 mmHg in the left eye. Slit lamp examination showed clear corneas in both eyes, with diameters of 13.5 mm in the right eye and 10 mm in the left eye. The left eye also had a coloboma of the iris, which manifested as a small notch in the inferonasal pupillary margin () and shallow chamber depth. Keratometry and A-scan ultrasonography were performed. Upon A-scan ultrasonography, axial length was 28.16 mm in the right eye and 29.65 mm in the left eye. Anterior chamber depth was 3.75 mm in the right eye and 2.34 mm in the left eye. Upon keratometry, correction was 43.38 D in the right eye and 40.0 D in the left eye. Fundus examination of the right eye showed a myopic tigroid fundus with tilted disc and peripapillary atrophy. Fundus examination of the left eye showed an inferior choroidal coloboma for about six disc areas, extending to the optic disc, macula and above the temporal arcade. Total bullous detachment of the retina was also observed, including detachment of the macula and the other areas of the retina. Findings suspicious for retinal break were visible in the inferotemporal margin of the choroidal coloboma ().\nThe patient subsequently underwent repair of the retinal detachment. Standard three-port of the pars plana vitrectomy was initially performed with sclerotomy sites 2.5 mm posterior to the limbus. By using the operating microscope, fluid-air exchange was performed with a tapered needle placed over the ecstatic abnormality of the colobomatous zone. A retinal break was noted due to the subretinal viscous fluid passing through the break. The retina was gradually flattened by evacuating subretinal fluid through the retinal break. An endolaser was applied over the flattened retina around the colobomatous area by diode laser. To achieve internal tamponade, 5,000-centistoke silicone oil was then injected into the vitreous cavity. The patient was encouraged to maintain a face-down position for two weeks.\nAt a six-month follow-up period, the patient had visual acuity of hand motion, and the retina remained attached. |
A 21-years-old Caucasian woman presented to a private dental clinic with a chief complaint of asymptomatic swelling in the gingiva observed four years prior. A gradual increase in size and no history of previous treatment were also reported during the anamnesis. The patient signed the informed consent, which represents the ethical approval of the faculty committee. Her medical and socio-economic histories were not contributory. The extra-oral evaluation did not reveal changes. The intraoral examination revealed a sessile nodule with a color similar to that of the mucosa and a focal erythematous area with a fibro-elastic consistency measuring 1.5 cm in the largest diameter extending from the inferior right lateral incisor to the inferior right first premolar. The lesion involved the vestibular and lingual gingiva, causing displacement of the inferior right canine (Fig. ).\nPanoramic reconstruction and parasagittal slices of the Cone Beam Computed Tomography (CBCT) showed a slightly superficial hypodense area between the inferior right lateral incisor and inferior right canine with reabsorption of the alveolar crest (Fig. ). Based on the clinical and immunological aspects, the main diagnosis hypotheses included peripheral ossifying fibroma, peripheral giant cell lesion, and ancient pyogenic granuloma. The peripheral odontogenic tumors were also included as a differential diagnosis. An excisional biopsy was performed and a clear separation was noted between the lesion and mandible bone during the trans-surgical approach. The histopathological analysis revealed a well-circumscribed proliferation comprising numerous islands and strands of epithelial polyhedral cells with well-defined borders and marked round nucleus in the connective tissue under the mucosal epithelium. Numerous nests, cords, and small islands of polyhedral cells with clear and vacuolated abundant cytoplasm were observed interspersed with the amorphous eosinophilic deposits (Fig. ). Immunohistochemistry was performed, which yielded positive results for CK-19 in the epithelial cells, except for the clear cells. Congo red staining showed the presence of amyloid-like deposits with apple-green birefringence under polarized light (Fig. ). A final diagnosis of a peripheral CEOT rich in clear cells was reached. No complications were observed in the postoperative appointment and a follow-up schedule was established. The patient has had no recurrence after 22 months (Fig. ). |
Female, 22 years old, the first born to healthy, unrelated Dutch parents. She has one healthy sibling. At the age of 1 year she was evaluated because of growth failure, abnormal chest shape and limb shortness. Skeletal radiographs showed a small, narrow thorax, brachydactyly of the fingers, short and broad diaphyses, and wide metaphyses of the arms and legs, and short iliac bones with spiky protrusions. There was no history of respiratory difficulties. At 3 years of age she was admitted because of a respiratory infection. Blood gas revealed a metabolic acidose, serum creatinine was elevated, urinalysis showed proteinuria and her blood pressure was 200/170 mmHg. Her renal function deteriorated (serum creatinine 422 μmol/l, ureum 16.8 mmol/l) and at the age of 4 years CAPD was started. A renal transplant was performed when she was 5 years old and 5 weeks later bilateral nephrectomy was performed because of persistent hypertension. At the age of 12 years she developed progressive renal failure, as a result of chronic rejection, and she was transplanted for a second time. Until now, her renal function remained stable. Ophthalmic examination showed narrow blood vessels without clinical problems. At the age of 22 years she is living independently and working as a nurse with mildly impaired exercise tolerance. Physical examination at the age of 22 years showed a height below the third centile, a sitting height/height ratio above the 97th centile, a chest circumference below the third centile, a hand length below the third centile and a foot length below the third centile. The chest radiograph showed an abnormal position of both ribs and claviculae with a CTR of 0.53 (reference < 0.50; []). Pathological examination of the native kidneys showed that most of the glomeruli were totally sclerotic and the remainder showed capillary collapse. Periglomular fibrosis, tubular atrophy and dilatation with interstitial lymphocyte infiltration were seen. |
A 39-year-old Caucasian woman presented in our emergency department with acute abdominal pain associated with nausea, vomiting, and signs of intestinal occlusion. The clinical history of the patient highlighted two other admissions for the same clinical signs. During the first admission, she was given an abdominal computed tomography (CT) scan that demonstrated only the presence of free fluid localized in the pouch of Douglas and the perihepatic region. In relation to these signs, she was given an emergency, explorative laparotomy, with lavage of the abdomen. The laparotomy demonstrated only hyperemic jejunal and ileal handles. She was discharged after nine days without any complications. Two weeks after the patient was readmitted to the same hospital with similar symptoms, and she was treated with corticosteroids, mesalazine, and metronidazole with a complete resolution of the symptoms. Five days later, the patient was admitted to our unit. At admission, she had leukocytosis (WBC, 19.960 × 106/L) and normal levels of the coagulation parameters. She was given abdominal ultrasonography in association with Doppler ultrasonography (Esaote Megas GPX 7.5-MHz convex probe), highlighting a thrombosis of the SMA. As a result of this clinical picture, she underwent an abdominal CT scan (Figures to ), demonstrating the presence of a partial thrombosis of the celiac trunk, a thrombosis of the SMA for a 25- to 30-mm tract, and the lack of a splenic artery. She immediately underwent an explorative laparotomy, showing ischemic, but viable handles, and a tree revascularization by thromboendarterectomy with a Fogarty catheter was performed. In the following postoperative days, she was given a scheduled second and third look, showing necrotic handles (the first jejunal handle, the last ileal handle, and about 20 cm of the medium ileum) in the first procedure, and another necrotic tract of small bowel (the other 10 cm of the first jejunal tract) in the last procedure. During that surgical procedure, we performed duodenojejunal and three other laterolateral anastomoses to reestablish the bowel continuity. A T-tube was inserted to protect the duodenojejunal anastomosis. A cholecystectomy and biliary diversion were performed to reduce the biliary output. In relation to the risk of dehiscence, we performed a colonostomy in the right flank. During all the surgical procedures, we performed intraoperatory Doppler ultrasound of the SMA and celiac trunk to control the arterial flow without evidence of a new thrombosis. The patient stayed in the ICU for 27 days with total parenteral nutrition and antibiotics therapy. A coagulation screening demonstrated a thrombophilic state for a protein-S (16%) deficiency with normal values of VIII, IX, and XI factors. The search for antiphospholipid antibodies was negative, and the genetics test for factors II to V and methylenetetrahydrofolate reductase (MTHFR; the deficiency of this enzyme is associated with an increased risk to develop massive thromboembolic events) was negative (no mutations). She was discharged from our unit after 37 days without any complications. After three months, the patient had a surgical procedure for restoring the bowel continuity. The patient was evaluated after one week, and one, three, and six months after discharge with blood and coagulation examinations, abdominal ultrasonography, Doppler ultrasound, and abdominal CT scan. She was asymptomatic and stayed well. At one year, we had successfully restored the bowel continuity without complications. |
A 37-year-old Caucasian male with a known history of aplastic anemia (AA), presented to a rural hospital after a ground level fall. AA was diagnosed 10 months earlier after he was investigated for pancytopenia. A bone marrow biopsy showed cellularity of only 10% and the presence of a small paroxysmal nocturnal hemoglobinuria clone (less than 0.2%). He received standard combination treatment for AA with cyclosporine 225 mg orally twice daily, horse anti-thymocyte globulin (ATG) 40 mg/kg daily for 4 consecutive days, and prednisone 1 mg/kg daily. His other medications included daily Pantoloc 40 mg orally, daily Valtrex 500 mg orally, and daily Dapsone 50 mg orally for Pneumocystis jirovecii prophylaxis due to a reported allergy to trimethoprim/sulfamethoxazole. He had recently quit smoking and denied alcohol use but actively used other recreational drugs, including marijuana, cocaine, and methamphetamine. He was unemployed. He had no known other medical co-morbidities and was taking no other medications prior to developing AA. The etiology of AA was felt to be idiopathic because he had no improvement after an initial trial of sobriety. AA improved following immunosuppressive therapy and, although human leukocyte antigen typing was performed, a subsequent bone marrow transplant was deferred not only because of the medical therapeutic response but also due to his ongoing recreational drug use. Although he was no longer transfusion dependent a month after starting immunosuppressive therapy, his treatment compliance waned overtime due to regular ongoing recreational drug use of cocaine and methamphetamines. He routinely used unsterilized tap water for illicit drug injections, but he denied other exposure to fresh or salt water sources at home or in the community.\nOn presentation to the emergency department he was not in distress, with a heart rate of 90 bpm and a blood pressure of 116/59. Severe pallor was noted upon examination, as well as a petechial rash and mild ecchymoses (Fig. ). The rest of his physical assessment was normal, including a neurological examination. Admission bloodwork revealed severe pancytopenia with hemoglobin of 22 g/L, a platelet count of 1 × 109/L, a white blood cell count of 3.7 × 109/L, and an absolute neutrophil count of 0.2 × 109/L (reticulocytes were not sent at admission, but 2 weeks into his hospitalization his absolute reticulocyte count was 12 × 109/L with a reticulocyte percentage of 0.5). All other admission blood work was normal, including liver function tests (total bilirubin 9 μmol/L (reference < 21 μmol/L), alanine aminotransferase 13 μmol/L (reference < 41 μmol/L), alkaline phosphatase 66 U/L (reference 30–130 U/L)) and renal function tests (creatinine 63 μmol/L (reference 59–104 μmol/L), glomerular filtration rate 120 mL/min (reference < 59 mL/min)). He was stabilized and transferred to a tertiary care center where he was restarted on treatment for relapsed AA with a regimen that included cyclosporine (5 mg/kg/day) and prednisone 30 mg daily in addition to five doses of ATG. He remained transfusion dependent throughout his hospitalization.\nOn day 10 after admission, he developed generalized, mild (3/10), colicky abdominal pain with an associated fever > 38.5 °C. He was started empirically on piperacillin-tazobactam (PTZ) 3.375 gm intravenously every 6 hours. Two sets of blood cultures, each consisting of an anaerobic and aerobic BacT/Alert bottle (bioMérieux, Laval, Quebec), were collected peripherally and from his central line. E. coli grew in each bottle set at 10 and 11 hours, respectively. He then developed watery, non-bloody bowel movements, 3–4 times a day, associated with rectal pain. Real-time PCR for Clostridium difficile A/B toxin on a stool sample was negative. Computerized tomography of the abdomen and pelvis was also unremarkable. Repeat blood cultures were negative at 24 and 48 hours after the initial positive set. He improved dramatically after 7 days of intravenous PTZ and was stepped down to oral ciprofloxacin 500 mg orally twice daily to complete a further 7 days of therapy.\nOn day 19 of admission he developed acute continuous severe (9/10), non-radiating dull rectal pain, associated with a high-grade fever (40.4 °C). Vancomycin 1.5 g intravenously every 12 hours and metronidazole 500 mg orally twice daily were empirically started and ciprofloxacin was continued in the same dosage. Blood cultures that were collected from peripheral venipuncture and a peripherally inserted central catheter line grew A. hydrophila at 11 hours. The peripherally inserted central catheter line was immediately removed the next day (day 20 after admission). The same day he also began to complain of vague, mild, bilateral leg pain. Delayed serum sickness due to recent ATG administration was considered a possible cause for his new symptoms because clinical examination did not show erythema, edema, or deformities on either of his legs. However, sustained bacteremia was diagnosed by recovery of A. hydrophila from repeat blood cultures (i.e., one anaerobic and aerobic bottle set from two peripheral venipunctures) positive after 11 and 16 hours of incubation. Bilateral leg pain steadily worsened in intensity (10/10) over the next 48 hours, and the area of distribution of pain extended to the lateral aspect of the right thigh although physical examination remained unremarkable. Creatinine kinase was increased at 470 U/L (normal range for males, 0–195 U/L). Ultrasound venous Doppler of both legs also showed no evidence of deep venous thrombosis. However, magnetic resonance images of both legs showed extensive bilateral patchy multi-compartment muscular and fascial inflammatory changes highly concerning for NF (Fig. , ).\nUrgent initial surgical debridement was performed that evening. An extensive four-compartment fasciotomy, debridement, and myomectomy were performed on both legs. Extensive ‘dishwater’ purulent material was found in multiple compartments of both legs, including (1) the superficial posterior compartment between the gastrocnemius and soleus muscles, and (2) the lateral deep compartment. There was also clinical evidence of severe muscle necrosis of the tibialis anterior muscles in the anterior compartment of both legs. He was admitted to the Intensive Care Unit post-operatively. After consultation with the Infectious Diseases service and review of the antibiotic susceptibility profile of the previously isolated A. hydrophila strain, antibiotics were changed to meropenem 1000 mg intravenously every 8 hours and clindamycin 600 mg intravenously every 8 hours. High dose intravenous immunoglobulin (2 g/kg) was also given. All prior antibiotics were discontinued.\nGram stain of tissue samples from the right tibialis anterior muscle showed no neutrophils but that gram-negative bacilli were present, and subsequently grew a heavy amount of A. hydrophila. Gram stain and anaerobic culture from the right vastis lateralis muscle also did not show the presence of neutrophils or organisms but grew scant amounts of A. hydrophila. A genus-level identification as Aeromonas was obtained for all isolates from blood and tissue samples by matrix-assisted laser desorption ionization-time of flight (MALDI-TOF) mass spectrometry using a VITEK MS (bioMérieux, Laval, Quebec, Canada); since this technique has an accuracy of identification rate of 80–90% for species-level identification of Aeromonas [], all isolates were also analyzed using in-house bi-directional 16S rRNA gene cycle sequencing of the V1-V3 (approximately first 500 bp), as previously described []. Broth microdilution susceptibility panel testing was performed and interpreted using published guidelines []. All isolates were multidrug resistant to ampicillin, ceftriaxone, ciprofloxacin, and trimethoprim/sulfamethoxazole but susceptible to meropenem and tetracycline. The isolates were confirmed to produce an extended-spectrum β-lactamase (ESBL) using published guidelines and the Mast Disc Test (Mast Group Ltd., Merseyside, UK) []. Production of an AmpC β-lactamase was shown by resistance to cefoxitin disk (30 μg) testing and the Mast Disc test (Mast Group Ltd.).\nTwo additional extensive surgical procedures for removal of necrotic tissue from both legs were undertaken in the next 24 hours. Bilateral above-knee amputations were performed during the last debridement as a life-saving measure because of extensive rapid progression of bilateral leg necrosis, and the patient’s rapid clinical deterioration with severe unremittent hemodynamic instability during the operation. Post-operatively, he required aggressive resuscitation for septic shock in the Intensive Care Unit with intractable hyperkalemia and severe acidosis, and anuric acute kidney failure (creatinine 210 μmol/L; normal range for males, 50–120 μmol/L). Despite all therapeutic interventions, the patient went into cardiac arrest and passed away within 2 hours after the final surgery.\nPost-mortem examination at autopsy revealed findings related to the underlying AA, and evidence of septic shock secondary to extensive bilateral lower limb necrotizing myofasciitis. The bone marrow was markedly hypocellular and there was splenic enlargement at 331 g. The heart was enlarged (536 g). Cardiomegaly was likely a compensatory response to the AA due to the absence of atherosclerotic and hypertensive cardiovascular disease. In keeping with the patient’s severe septic shock, there was marked centrilobular necrosis of the liver, as well as petechial hemorrhages of the skin, heart, pleural surfaces, kidneys, and liver capsule. Histologic examination of skin and muscle from the left thigh showed necrosis of the muscle and deep subcutaneous adipose tissue, admixed with dense collections of gram-negative bacilli (Fig. , ). However, in keeping with the AA, there was notably an absence of an acute inflammatory response. |
An 88-year-old man was referred to our hospital for emergent massive hemoptysis. His medical history was remarkable for chronic heart failure, moderate mitral regurgitation, atrial fibrillation, and chronic kidney disease. He was undergoing treatment with apixaban and pilsicainide for atrial fibrillation. He was severely hypoxic (SpO2: 80 under O2 15 L/min by oxygen mask) and hypotensive (systolic blood pressure: 80 mmHg) on admission. In the emergency department, we performed intubation into the right main bronchus through guided bronchoscopy; this was followed immediately by right side one-lung ventilation as portable chest radiography showed consolidation in the left upper lung. Bronchoscopy showed that the trachea was almost obstructed by haemorrhage and haematoma. He experienced cardiopulmonary arrest immediately after the airway was maintained. However, spontaneous circulation was restored by cardiopulmonary resuscitation. Contrast computed tomography (CT) demonstrated an aortic aneurysm at the aortic arch, which penetrated the upper lobe of the left lung (Fig. A,B). We suspected that it would be difficult to perform emergent surgery because of the patient’s poor general condition. Furthermore, we believed that there was no indication for endovascular stenting due to the following reasons: (1) the root of the left brachiocephalic artery was close to the penetrating portion of the aneurysm, at a distance of 12 mm. Thus, there might have been a high risk of obstructing blood flow to the brachiocephalic artery; (2) a risk of aortic injury might have been induced by stenting because the aortic arch was highly calcified. He was admitted to the intensive care unit, and we controlled blood pressure using nicardipine and discontinued anticoagulation therapy and performed platelet and fresh frozen plasma (FFP) transfusion for haemostasis. Bleeding from the APF decreased gradually due to astriction by haematoma. On the 17th hospital day, we performed bronchoscopy for the suction of haematoma, except in the bronchi of the left upper lobe, and adjusted the intubation tube for conversion to bilateral lung ventilation. On the 18th hospital day, we performed endobronchial occlusion with EWS to prevent fatal hemoptysis despite the risk of atelectasis. We inserted the EWS into each target bronchus with haematoma, with consideration of the risk of re-bleeding due to the removal of the haematoma. EWS sizes were as follows: (B1 + 2a: 7 mm; B1 + 2b: 7 mm; B3b + c: 7 mm; and B3: 7 mm) (Fig. A). B1 + 2c did not undergo EWS insertion because this bronchus was not responsible for bleeding on CT findings. There was no massive hemoptysis after bronchial occlusion with the EWS; subsequent mild hemosputum was controlled by a haemostatic drug. The patient was successfully extubated on the 22nd hospital day and was discharged on the 47th hospital day without complications and free from oxygen. Radiography showed that EWS had promptly fixed each bronchus (Fig. B). |
This was a 25-year-old male with known spina bifida who presented with a chronic infection of his left acetabulum. He had been previously managed for several years for a non-healing pressure ulcer of the left greater trochanter, having undergone a partial femoral head resection and prior flap placement with subsequent failure. He presented with large volume drainage from a small ulceration over his left trochanter with CT imaging demonstrating an abscess in the gluteus muscle with osteomyelitis in the abutting femoral head. He underwent a left Girdlestone procedure. Intraoperative findings included heterotopic ossification with necrotic bone in the femoral head. Cultures grew MRSA, Proteus mirabilis, and mixed microorganisms. The surgical wound was treated with a Cleanse NPWTi-d utilizing normal saline. Three days later, he underwent partial delayed primary closure over closed suction drains with the placement of a negative pressure dressing over the incision and ongoing wound, as complete primary closure was not possible due to the dimensions of the resulting wound. He was discharged eight days after the initial procedure on ertapenem. He was not readmitted in the first 30 days after discharge.\nAt his one-month follow-up, it was noted that his left-sided osteomyelitis had not recurred nor progressed. At his two-month visit, the wound continued to be clean and closed, with no sign of breakdown. However, at this time, he developed the worsening of a previously existing stage IV right ischial pressure ulcer, which was treated with operative debridement. He has not had a recurrence of his left hip osteomyelitis and his wound is nearly completely healed. Figure below depicts his chronic trochanteric ulcer, the wound after Girdlestone resection, placement of negative pressure wound therapy over the closed incision, and the resultant healing wound.\nPatient 7: right side\nThis was a 29-year-old male with a history of paraplegia who developed several stage IV ischial and sacral pressure ulcers on his right side, resulting in a dislocation of his femoral head on the right and progression of the infection into the acetabulum and iliacus muscle. His ulcer progressed despite appropriate treatment, and he also developed severe protein malnutrition; he was thus treated with a right Girdlestone procedure. Intraoperative findings were significant for necrotic exposed acetabulum and femoral head. Cultures grew MRSA and Staphylococcus epidermidis. The resulting wound bed, including the acetabulum, was dressed with a Cleanse Choice® (KCI, San Antonio, Texas, USA) NPWTi-d utilizing normal saline. Three days later, he underwent a partial delayed primary closure over closed suction drains with the placement of a negative pressure device over the incision. He was discharged 14 days after the initial procedure on doxycycline and trimethoprim-sulfamethoxazole. He was not readmitted in the first 30 days after discharge.\nAt his three-month follow-up visit, his wound was healing well, with no sign of recurrent osteomyelitis on the right side. However, he did have progressive ulceration of his previously existing left greater trochanter ulcer and was found to have invasive osteomyelitis in the left hip. Figure demonstrates the pre-operative ulcer, resection specimen, and resultant healing wound.\nPatient 7: left side\nDue to the success of the right Girdlestone procedure, the patient underwent a left Girdlestone approximately three months later. Like the right side, he had developed a chronic ulcer over the left greater trochanter with subsequent femoral head osteomyelitis. Intraoperative findings were also similar, with a necrotic femoral head and resultant cultures growing no organisms, though previous cultures grew Pseudomonas. The wound was dressed with a Cleanse Choice NPWTi-d (see Figure below). Three days later, he underwent a partial delayed primary closure over closed suction drains with the placement of a negative pressure device over the incision. He was discharged eight days after the initial procedure on doxycycline and trimethoprim-sulfamethoxazole and was not readmitted in the first 30 days after discharge.\nThe patient was readmitted at 60 days with concern for the protrusion of his left distal femur into the ongoing wound bed and was taken to the operating room for excisional debridement and bone biopsy. The biopsy was negative for invasive osteomyelitis. In addition, at month four, he presented with a stage IV ulceration of his sacrum. Adequate offloading, wound care and nutritional support, and intravenous antibiotics were not able to be achieved in the postoperative care of this patient due to numerous factors. He was discharged in this state five days later on trimethoprim-sulfamethoxazole with the intent to heal by secondary intention and has since re-presented with progressive malnutrition and dry gangrene of the toes of his right leg. He has refused ongoing medical care. He has not required ongoing treatment for the infection in either hip and his surgical wounds continued to decrease in size. |
In April 2015, a 23-year-old male was referred to our Faculty (Oral and Maxillofacial disease department of Mashhad university of Medical sciences). His chief complaint was an ulcer of the uvulae from three months ago, oropharyngeal dysphagia, pharyngitis, and mild fever with unintentional 20-pound weight loss over the course of 4 months. Also he was on a mechanical soft diet. Over the past four years, he had severe episodes of Pharyngitis without response to the usual administered drugs, as well as a history of severe dental infection involving the brain. He did not have any systemic disorders and no history of smoking, abusing drugs or drinking alcohol. He was a construction worker and often dealt with rock wool. Upon physical examination, two painful destructive ulcers were observed. One of them was about 2cm × 1 cm, extending from the soft palate to hard palate, exposing the underlying bone and another one was destructing the uvulae (). The ulcers were covered with a thick fibrinoleukocyter membrane with erythematous borders. He had no history of such lesions. His oral hygiene was in good condition. Cervical lymph nodes were not appreciable. Disseminated hypopigmented maculopapular rash throughout the skin, from nine years ago, was also observed. Examination of vital signs revealed a blood pressure of 110/70 mm Hg, a heart rate of 80 beats per minute, and a body temperature of 100°F. The rest of his physical examination did not raise any concerns.\nThe patient had been initially admitted to the Ear, Nose and Throat department two months ago. Due to suspicion of an infective condition (Trench mouth), some antibiotics such as Ceftriaxone, Metronidazole and Clindamycin were administered for about one month but without significant improvement.\nBecause of the negative response to the drugs mentioned above the suspicion of an immunosuppressive condition was raised. Therefore, the patient was admitted to the allergy and immunology department. After consultation with an infectious disease specialist, the administered antibiotics were altered to Ceftazidime, Vancomycin, and Metronidazole. However, despite taking these remedies for about three weeks, no improvement was observed. Along with these treatments, some laboratory tests and paraclinical modalities had been requested below:\nThe blood test only showed Neutrophilia (about 80% of the white blood cell count) and Lymphocytopenia. Erythrocyte sedimentation rate was high and C-reactive protein was positive. Serum chemistry parameters were in normal range. All of the virology, microbiology and parasitology tests; such as Anti-HIV, Tuberculin skin testing, etc.; were negative. He had a high Lactate dehydrogenase level of about 595 IU/L. Given the patient's fevers, history of recurrent upper respiratory tract infection with depigmented skin rashes, Infectious mononucleosis was a concern when white blood cell morphology was studied and viral type Lympho-Mono were seen. Also viral capsid antigen (VCA)-IgM and VCA-IgG were dramatically high in the patient’s serum. The results of nitro blue Tetrazolium test NBT and Dihydrorhodamine Flow cytometric Assay (DHR) and other immunological laboratory findings were in normal ranges, except for high IgE (2375 µ/dl) and a slightly low IgM (25 mg/dl). Consequently Immunodeficiency and autoimmune disease were not confirmed. Paraclinical modalities such as Abdominal, Inguinal and Neck ultrasonography, Chest X-ray, Posterior-Anterior Skull X-ray were requested and no abnormalities were observed, except for multiple reactive lymph nodes up to 12 millimeters in size at the anterior cervical lymph node chain, which were detected by ultrasonography. Computed tomography (CT) scan of the head and neck with contrast medium revealed nothing of note. In addition, biopsy from palatal ulcers with fungal culture revealed a non-specific ulcer with extensive necrosis and bacterial colonies, fibrin deposition and vasculitis. Dysplasia, malignancy any fungal elements were not evident.\nA biopsy of the palatal ulcers was repeated and the histopathological examination of the specimen showed very dense lymphocyte infiltration. In the background of the tissue, lymphoid cell with atypia, irregular and prominent nuclei were seen. The benign inflammatory cell infiltration with geographic necrosis in the background was evident (). After consultation, the patient was referred to our Faculty and two clinical provisional diagnoses were made: The first one was a Lymphoproliferative disease such as Non-Hodgkin lymphoma and the second one was necrotizing stomatitis accompagnied by immunocompromised conditions and viral infections like EBV, cytomegaloviruses (CMV) and Herpes simplex viruses (HSV). Hence immunohistochemical analysis of paraffin blocks was requested. CD3 was expressed in some background lymphoid cells, CD56 was positive in some lymphoid cells, CD20 was positive in some background lymphocytes, and the Ki-67 marker was positive in 60% of background of Lymphoid cells. Terminal deoxynucleotidyl transferase (TdT) was negative and finally the diagnosis of ENKL was confirmed (). Clinical staging of the lymphoma according to the Ann Arbor classification was determined as stage IE (4). The patient was referred to the oncologist and a CHOP regimen of chemotherapy was administered every three weeks. After the third cycle of CHOP therapy, the patient unfortunately died due to sepsis and infection (about 3 months after treatment). |
B. A., a 71-year-old man, presented with dyspnoea under moderate exercise, without angina. His clinical history was characterized by a silent inferior-basal myocardial infarction detected by a control ECG that presented inferior Q waves, and he had not previously presented symptoms. The infarct was referable to maximum a couple of months before especially basing on symptoms worsening. So he had not any previous diagnosis or treatment of the acute event.\nFunctional NYHA Class was III, but it was I two months before, and symptoms were rapidly worsening in the last 20 days.\nThe patient was a smoker, affected by chronic obstructive pulmonary disease (COPD), hypertension, and peripheral vascular disease, presented in sinus rhythm. Medical therapy was based beta blockers, ASA, and a diuretic with some relief of symptoms.\nA transthoracic echocardiogram (TTE) showed a dyskinetic posterior wall of the left ventricle, mild mitral regurgitation, with moderate reduction of the ejection fraction (EF = 44%). An aneurysmatic enlargement was noted in the posterior wall, but with few signs to distinguish a true aneurysm from a pseudo-aneurysm. The patient underwent a coronary angiographic examination that revealed patent anterior descending and circumflex arteries and a right coronary artery that was completely closed and perfused by hetero-coronaric circulation. Contrast ventriculography showed an enlargement of the left ventricle with a large dyskinetic cavity localized in the diaphragmatic region, suggesting the presence of a pseudo-aneurysm. (Fig. )\nA second TTE was done, with results similar to the first one, but a thrombus in the posterior wall was also disclosed.\nAt the time of surgery a large true aneurysm of the posterior wall of the left ventricle was found (Fig. , ). The aneurysm consisted of a very thin myocardium layer; inside there was a thrombus about 6 cm × 4.5 cm. (Fig. )\nSurgery was performed via medial sternotomy. A normothermic cardiopulmonary by-pass (CPB) was carried out with cannulation of the aorta and the right atrium, on a beating heart without cardioplegic arrest and without clamping of the aorta. We performed the Dor repair, as described by Dor et al. [], after creating a neck of healthy muscle with 2/0 Prolene suture. The defect was closed, using the thin autologous aneurysmatic excised myocardium layer as patch. It was sutured with 3/0 continuous Prolene.(Fig. ) Two Teflon pledgets were used to reinforce the ventricle suture on the outside.(Fig. ) The CPB was 78 minutes long. Intra-operative TEE showed no mitral regurgitation (MR) and satisfactory post-surgical remodelling.\nThe patient was discharged 6 days after surgery without complications. At his 3 month control, the patient was completely restored to a normal life, an echocardiography showed good results in left ventricular remodelling with mild mitral regurgitation and an improved ejection fraction (EF = 55%). |
A 68-year-old man who lived in Venezuela for the last four decades presented to our clinic with inexplicably incremental renal and hepatic failure with an obviously reduced general and nutritional condition for further diagnostic and therapy. Due to severely reduced creatinine-clearance (MDRD = 14 mL/min), he was scheduled for continuous ambulatory peritoneal dialysis (CAPD) both to drain his up to six litre nonmalignant, persistent hydroperitoneum as well as for dialysis. Patient's history was inconspicuous apart from a single vessel coronary heart disease that had lead to an acute myocardial infarction (AMI) interventionally treated by implantation of a bare metal stent into the LAD three years ago. One-month post-AMI, the patient complained of progressive abdominal heaviness and pressure as well as shortness of breath, which was nebulously explained by hepatic failure due to thienopyridine treatment. In contrast to the assumed hepatic cirrhosis, multiple abdominal ultrasound studies did not reveal cirrhotic hepatic tissue texture as well as excluded a veno-occlusive Budd Chiari disease. Shortly before the admission to our hospital echocardiography study was conducted externally with reduced acoustic windows to exclude an underlying cardiac disease and showed a moderately reduced left ventricular function, a mild aortic and mitral regurgitation as well as anterior hypo- to akinesia with potential apical adhesions. These findings were in far contrast to a holosystolic harsh loud murmur accompanied by a thrill being present in the third and fourth left intercostal space. For further clarification of morphology, function, the extent of myocardial scar, and exclusion of constrictive pericarditis, a gadolinium contrast enhanced and cine MRI was conducted. Cine short axis, two- and four-chamber MRI imaging showed a dilated right and left ventricle with an almost severely reduced left and right ventricular function (LV ejection fraction = 35%), a pronounced apical post-AMI aneurysm with transmurally infarcted myocardial tissue () as thin as 1.5 mm together with a moderate to severe mitral and tricuspid regurgitation. Short axis imaging () revealed a hemodynamically highly significant 12 mm large apical interventricular septal defect (IVSD) with an MRI estimated regurgitation fraction of 52%. Therefore, having come to the diagnosis of a post-AMI ventricular IVSD, we consequently conducted a left and right heart transseptal catheterisation. Heart X-ray confirmed MRI results and revealed a left to right ventricular shunt of almost 63%, a severe LAD in-stent restenosis, and a severe mitral regurgitation. Despite the administration of more than 180 mL of X-ray contrast agent, patient's renal function was preserved due to an intensified CAPD regime. Heart surgery was performed where the apical aneurysm was excised, the mitral valve was reconstructed, the IVSD was closed and the subtotally in-stent occluded LAD was bypassed with an internal mammaria coronary artery bypass graft. Post-surgery, the ascites were significantly reduced. CAPD therapy could be terminated since the renal function gradually improved (MDRD = 25 ml/min).\nThe overall incidence of post-AMI IVSD is hard to assess because clinical and autopsy series differ considerably []. A large autopsy study in 1989 suggests that the incidence of myocardial interventricular rupture has increased since the late 1960s, with a rate of 31 per cent among necropsied cases []. The prior use of corticosteroids or nonsteroidal anti-inflammatory agents has been implicated as predisposing to rupture as a result of impaired healing. Conversely, the early use of thrombolytic therapy appears to reduce the incidence of cardiac rupture [], an effect that is responsible in part for improved survival with effective thrombolysis. The size of the defect determines the magnitude of the left to right shunt and the extent of hemodynamic deterioration []. The likelihood of survival depends on the degree of impairment of ventricular function and the size of the defect []. Recently a non-ischemic surgical repair was described but was not suitable in this patient due to the complex situation this newer approach without aortic occlusion, systemic hypothermia and cardiolplegic arrest.\nIn our case, due to right-sided heart failure with increased right atrial pressure and tricuspid regurgitation, subsequent congestive hepatomegaly associated ascites was extensively treated with diuretics and consequently led to hepatorenal syndrome. Although echocardiography might not always suggest IVSD due to reduced acoustic windows, simple and inexpensive auscultation is almost pathognomonic. An IVSD is characterized by the appearance of a new harsh, loud holosystolic murmur at the lower left sternal border and is usually accompanied by a thrill. Although biventricular failure generally ensues within hours to days, in our case the patient astonishingly survived more than three years with accretive signs of hepatic and renal failure. Operative intervention is mostly successful and should not be delayed in patients with a correctable lesion [].\nOur case report has two important issues. To our knowledge, for the first time we report successfully practiced CAPD catheter drainage of hydroperitoneum due to congestive liver and renal failure in combination with CAPD hemodialysis. Secondly, simple auscultation of the heart lead to the diagnosis of IVSD that, if correctly performed earlier, could have greatly relieved the patient's symptoms, his reduced physical state, and his commencing hepatic and renal failure. Furthermore cardiac TEE or MRI as not invasive and radiation-free methods should be considered in case of limited acoustic windows for echocardiography to exclude an IVSD. |
A 55-year-old man developed sudden-onset right-sided hemiplegia and aphasia. His colleagues at work immediately called the emergency services. The patient had several cardiovascular risk factors, including hypertension, hyperlipidemia, and previous myocardial infarct.\nHe arrived at the CT lab in our emergency room (ER) 17 min after symptom onset. The NIHSS score was 19. The blood pressure was 149/93 mm Hg, and plasma glucose level was 92 mg/dL (76–113 mg/dL). CT showed “dense artery sign”. CTA revealed a thrombus in the M1 segment of the left middle cerebral artery and preserved blood flow in the peripheral part of the middle cerebral artery due to collaterals.\nWe do not wait for the results of all the blood tests before performing thrombolysis, unless there is a specific reason to do so. Intravenous thrombolysis could therefore be initiated with a door-to-needle time of 15 min. The patient was immediately taken to our angiography suite for a mechanical thrombectomy.\nHe was slightly agitated, and general anesthesia was considered necessary to safely perform the procedure. Onset-to-groin puncture time was 62 min. We gained access from the right groin, and angiography confirmed a total occlusion of the proximal left M1 segment (Fig. ). A microcatheter was advanced into and presumably beyond the occlusion. A Solitaire 2 (6 × 30 mm) revascularization device was introduced through the microcatheter and expanded with the proximal part of the device aligned with the proximal part of the occlusion. The Solitaire device was kept in place for 5 min for the radial forces of the device to work through the thrombus. The thrombus was successfully removed 85 min after symptom onset. The following angiography showed full recanalization of the left middle cerebral artery (Fig. ).\nOn waking up from general anesthesia, the patient could move all 4 extremities and had regained normal language function. NIHSS score shortly after the procedure was 0. A brain magnetic resonance imaging (MRI) scan performed on the same day was completely normal, with no signs of infarct on diffusion-weighted imaging (Fig. ). The patient underwent further investigations to identify the etiology of stroke. Blood tests for thrombophilia were normal – no factor V (Leiden) or prothrombin mutation, no lupus anticoagulant, and normal protein S and C. Carotid duplex ultrasound revealed a small calcified plaque at the carotid bifurcation on the left side, without significant stenosis. Transthoracic echocardiography showed hypertrophic left ventricle with inferolateral hypokinesia and ejection fraction around 50%. Three-day Holter monitoring was normal. Accordingly, we concluded that the patient had stroke with a “cardioembolic etiology” based on TOAST criteria []. The patient was discharged symptom free 5 days later with appropriate secondary prevention according to the guidelines.\nAn ultra-early MRI scan implies a risk of false-negative diffusion-weighted imaging in acute ischemic stroke []. The MRI of our patient was performed within 24 h of symptom onset. We therefore performed a follow-up MRI scan 6 weeks after the event. The follow-up scan was also normal, with no signs of infarct on FLAIR imaging. At that time, the patient had returned to his work. |
A 71 year-old man was admitted due to exertional dyspnea (New York Heart Association functional class III). He had a past medical history of pericardiectomy due to effusive-constrictive pericarditis 29 years ago and mitral valve replacement with tricuspid annuloplasty due to severe mitral and tricuspid regurgitation 11 years ago. His electrocardiogram showed atrial fibrillation with controlled ventricular rhythm. Transthoracic echocardiography showed abnormal shunt flow from the aorta to the right atrium, a tissue defect in the mitral annulus with moderate PVL (distal jet area of 7 cm2), mild tricuspid regurgitation with mild resting pulmonary hypertension (maximal velocity of tricuspid regurgitation jet=3.2 m/s), and an enlarged left ventricle with near normal systolic function (end-diastolic dimension of the left ventricle=67 mm, ejection fraction=51%). Transesophageal echocardiography (TEE) also revealed continuous abnormal shunt flow through a fistula tract from the aorta (noncoronary cusp) to the right atrium (), and about a 4-5 mm sized tissue defect in the mitral annulus with moderate PVL (). On computed tomography, about a 3 mm PVL was observed at the lateral aspect of the mitral annulus (). His calculated logistic EuroSCORE and STS risk score were 16.7% and 7.0%, respectively. After discussion with both the surgical and interventional teams, we decided to perform staged transcatheter closures for both the fistula tract and mitral PVL.\nThe first procedure was conducted under general anesthesia with fluoroscopic and TEE guidance. A 7 French (Fr) sheath was inserted through the right femoral vein and 6 Fr sheaths were placed in both femoral arteries. A 6 Fr pigtail catheter was inserted through the right femoral artery. After a peripheral angiogram with a pigtail catheter, we confirmed the existence of a fistula tract between the aorta and right atrium. The fistula tract could be crossed with a 260 cm long 0.032 inch Terumo wire, and it was confirmed by TEE. A 6 Fr Cournand catheter was advanced into and passed through the defect. Another 6 Fr Cournand catheter was introduced into the right atrium (). Using a Curry intravascular retriever, the guidewire was captured and withdrawn through the right femoral sheath. The guidewire was exchanged for a 0.035 inch stiff wire using a 6 Fr Cournand catheter. The 6 Fr Cournand catheter was replaced with an 8 Fr delivery sheath that was advanced into the ascending aorta. Because the sizes of the tissue defect on TEE were 9 mm in length and 4 mm in width, we selected an 8/6 mm sized Amplatzer duct occluder. After proper device positioning, which was confirmed using fluoroscopy and TEE, the device was deployed ( and ). Immediate TEE showed a well-positioned device, and disappearance of the abnormal shunt flow between the aorta and right atrium ().\nAfter the first procedure, his symptoms improved slightly and a follow-up echocardiography two months after the first procedure showed slightly decreased left ventricular diastolic dimension with reduced ejection fraction (from 51% to 46%) and persistent resting pulmonary hypertension. Percutaneous closure of the PVL was attempted to decrease left ventricular volume overloading. Under general anesthesia, a 7 Fr sheath was inserted through the right femoral vein and a 6 Fr sheath was inserted through the right femoral artery. A TEE was also used for guidance. A pigtail catheter was placed into the posterior cusp of the aortic valve through the right femoral artery. The sheath in the right femoral vein was exchanged for a Mullin sheath and dilator, which was advanced over a 0.032-inch guidewire into the superior vena cava. Thereafter, the guidewire was removed and a Brockenbrough needle was gently advanced to within a few millimeters of the tip of the dilator, and the needle was flushed and connected to a manifold for continuous pressure monitoring. With fluoroscopic guidance (RAO 45 degrees), the interatrial septum was punctured at the site of the fossa ovalis. The Mullin sheath was advanced into the left atrium. The stainless steel wire was crossed over the tissue defect in the mitral annulus. Using a 6 Fr multipurpose catheter, the stainless steel wire was exchanged for a 0.035 inch stiff wire (). After several dilatations with a septal dilator, the 8 Fr delivery sheath was placed in the left ventricle. Because the defect size on the TEE was about 4-5 mm, we also chose an 8/6 mm sized Amplatzer duct occluder. After proper device positioning was confirmed on the TEE, the device was deployed (). The TEE that was performed immediately afterwards showed a well-positioned device and a trivial PVL (). At 24 hours post-procedure, transthoracic echocardiography also showed well-positioned devices and no remnant shunt flow or PVL. Echocardiography immediately after the procedure showed persistent left ventricular dysfunction (ejection fraction=47%) but disappearance of resting pulmonary hypertension. |
A 76-year-old male with no prior cardiac history was hospitalized after chest wall and pelvic trauma following a fall from a ladder. On the third hospital day, a pause lasting 2.6 seconds was seen on telemetry while the patient was awake (). Progressive PR prolongation is visible in the first two cardiac cycles. The T wave of the second cycle is deformed suggesting a superimposed premature atrial contraction with atrioventricular block. A junctional beat is visible 2.6 seconds after the preceding QRS, followed by a sinus beat with a prolonged PR interval and another junctional beat. There was no associated lightheadedness, presyncope, or syncope. Interestingly, the patient reported having a violent bout of hiccups (which he described as being the worst of his life) during the time the bradyarrhythmia was recorded on telemetry.\nThe patient was normotensive and his pulse rate was noted to be in the sixties. The cardiac examination was normal. Patient’s medications included metoprolol 50 mg orally twice daily (started during this hospitalization for episodes of atrial tachycardia) and morphine sulfate intravenously as needed for pain control. Laboratory data demonstrated no electrolytes disturbances and a normal thyroid stimulating hormone level. Transthoracic echocardiogram was performed, which showed normal left ventricular function, no significant valvular abnormality, normal aortic root, and no evidence of cardiac contusion. Based on these findings, the etiology of the atrial tachycardia was likely from an increased catecholamine state from his recent trauma. Of note, the patient last received metoprolol 12 hours prior to the pause and morphine sulfate three hours prior.\nIncreased vagal tone/parasympathetic activity can be associated with hiccups, and hiccups have been reported to cause bradyarrhythmias. Due to the transient nature of the patient’s hiccups and the absence of bradycardic symptoms a permanent pacemaker was not indicated or offered to the patient. The metoprolol dose was continued at the same dose and the patient experienced no further bradyarrhythmias or hiccups for the remainder of the hospitalization. An outpatient Holter monitor did not reveal any recurrence of the bradyarrhythmias and the patient remains asymptomatic. |
A 64-year-old male presented with a two month history of difficulty urinating and was found to have a fungating penile mass involving 50% of his penis. The mass was hard and fixed and extended from the glans proximally up the shaft. He also had bilateral palpable inguinal lymphadenopathy. There were no associated constitutional symptoms. Given there was a high suspicion for malignancy, the patient underwent partial penectomy within a month of presentation. Biopsy results confirmed a pT2 tumor with invasive keratinizing squamous cell carcinoma, poorly differentiated, and tumor size of 5 × 4 × 2.5 cm, with corpus spongiosum and lymphovascular involvements.\nFollowing the procedure, the patient had PET-CT for staging, and imaging revealed enlarged hypermetabolic bilateral axillary lymph nodes concerning for metastatic disease. In addition, there was a large centrally necrotic lymph node conglomerate in his left groin that had increased FDG avidity. The patient had left inguinal and bilateral pelvic lymph node dissections revealing metastatic squamous cell carcinoma in multiple lymph nodes. The left inguinal mass was also found to be metastatic well-differentiated SCC. His diagnosis was staged at T2N3M0.\nAfter his surgical procedures, patient was started on adjuvant chemotherapy. He began first line chemotherapy with paclitaxel, ifosfamide, and cisplatin (TIP). He underwent 4 cycles of TIP but eventually developed disease progression on repeat imaging. At this point, the patient was started on cetuximab given EGFR amplification on tumor analysis with the FoundationOne testing platform. However, the patient had an allergic reaction to cetuximab, so his treatment was changed to panitumumab. The patient had stable disease and a progression-free survival of 6 months with anti-EGFR treatment, which is clinically significant given that this treatment was given in the second-line setting for an aggressive tumor type that other than chemotherapy there is no other approved drug to date.\nThe patient was ultimately started on the PD-1 inhibitor nivolumab. He had initial response to immunotherapy followed by stable disease, so he had a disease control rate of an additional 6 months with this investigational agent at that time. Ultimately, he was placed on hospice and passed away two years from the the time of diagnosis. |
A 59-year-old gentleman with end-stage kidney disease due to hepatitis C with focal segmental glomerulosclerosis, on hemodialysis for seven years, underwent deceased donor renal transplantation. The donor kidney had a KDPI of 60%. There was a 4 antigen HLA mismatch with Class II panel reactive assay (PRA) of 62.31%. Class I PRA was 0%. His donor specific antibody testing was positive for an antibody to DQ7. The donor had died as a result of anoxic brain injury following cardiopulmonary arrest resulting from anaphylactic shock. The cold ischemic time was 12 hours, 7 minutes and the warm ischemic time was 51 minutes. Basilixumab was used for immunosuppression induction and the patient underwent early steroid withdrawal. His maintenance immunosuppression was tacrolimus (targeting trough levels 5-8) and mycophenolate sodium 720 mg BID.\nThe patient's postoperative course was complicated by delayed graft function, necessitating continuation of renal replacement therapy. He was hemodynamically stable throughout his hospital course. A renal allograft ultrasound showed good blood flow to the entire kidney with a resistive index of 0.64. A repeat flow crossmatch was negative, but he remained positive for a persistent low-level donor specific antibody to DQ7 (1000 MFI). He underwent a renal transplant biopsy on his 4th day postoperatively. This showed extensive acute tubular necrosis with associated peritubular capillaritis and interstitial nephritis (). Oxalate crystals were seen in several tubules. One large caliber artery showed active endothelialitis, but no tubulitis or glomerulitis seen. C4d staining was negative in the peritubular capillaries. Electron microscopy revealed minimal effacement of podocyte foot processes. The patient received methylprednisolone 500 mg x 3 doses to treat moderate acute cellular rejection. His tacrolimus dose was also optimized as his trough levels had been running low at between 3.5 and 6. He continued to take mycophenolate sodium at a dose of 720 mg BID. A decision was made not to treat for antibody-mediated rejection given that no glomerulitis was seen and that there was minimal capillaritis with a negative C4d stain.\nHe remained dialysis dependent with minimal urine output for three weeks after transplantation. A urine protein : creatinine ratio was elevated to 1070 mg/g when assessed after hospital discharge when his urine output started to gradually improve. His urine microalbumin : creatinine ratio was 450 mg/g. A decision was ultimately made to readmit the patient on day 12 after transplantation for IV thymoglobulin 1.5 mg/kg to treat his Banff Type IIa T cell mediated rejection, given his lack of response to pulsed IV steroid treatment. Given lack of improvement in renal function and urine output by day 16 after transplantation, a second renal allograft biopsy was performed. This revealed residual acute tubular necrosis with associated mild peritubular capillaritis and interstitial nephritis (). Extensive oxalate crystals were now visible in many tubules. His plasma oxalate level was concurrently elevated to 19.3 μmol/L (reference range ≤ 1.9 μmol/L). No signs of persistent acute antibody- or cell-mediated rejection were seen. Again, there was minimal segmental effacement of the podocyte foot processes seen on electron microscopy and no evidence of recurrent focal segmental glomerulosclerosis present.\nHe commenced calcium citrate along with dietary oxalate restriction to manage his hyperoxaluria and his serum creatinine improved to a nadir of 1 mg/dl (88 μmol/L), with a concomitant gradual reduction in his proteinuria and microalbuminuria to undetectable levels. Of note, the patient had no history of malabsorptive intestinal disease and denied any GI symptoms throughout this time period. He had never suffered from renal calculi. At three weeks after transplantation, his urine output and creatinine clearance had recovered sufficiently to enable him to become dialysis independent. A decision was made to continue low-dose oral steroids in the long-term given the presence of early acute cellular rejection on his first allograft biopsy. An interval renal biopsy performed 2 months later showed no ongoing evidence of oxalate deposition, tubular necrosis or cellular rejection. A concurrent repeat plasma oxalate level showed interval reduction to the normal range. |
A 40-year-old lady with unknown co-morbidities presented with a 10 days history of multiple red raised painful lesions over body. Lesions were initially noted over forearms and in the next 2-3 days, new lesions appeared over arms and back. Patient complained of increased pain and burning sensation in the lesions while working in sunlight. Associated mild to moderate grade fever, intermittent in nature was present for the same duration. There was a history of malaise, pain over large joints, decreased appetite and redness of eyes. No history of oral ulcers, weight loss or any drug intake prior to her symptoms was present.\nGeneral examination revealed that she was febrile with an axillary temperature of 102°F. She also had pallor. Her systemic examination was essentially within normal limits. Dermatological examination revealed erythematous papules coalescing to form plaques with a pseudovesicular appearance over the extensor aspect of forearms and photo-exposed areas on back [Figures and ]. There was a sharp cut-off between the lesions and the photo-protected areas, where there were absolutely no lesions []. The plaques were tender. Conjunctival congestion was also present.\nInvestigations revealed anemia with haemoglobin of 9.5 gms/dl, and neutrophilic leukocytosis with 86% neutrophils out of a total of 13,000/mm3. She had an ESR of 36 mm fall in the first hour by the Westergren's technique. C reactive protein was positive. Antinuclear antibodies ANA and ELISA for HIV were negative. Extensive radiological, biochemical and hematological investigations did not reveal any underlying malignancy or any systemic association. Skin biopsy revealed edema of the papillary dermis along with a heavy neutrophilic infiltrate in the upper dermis. Furthermore, coalescing infiltrate of neutrophils in the lower dermis especially around blood vessels with absence of vasculitis was seen []. There was evidence of endothelial swelling with few fragmented neutrophilic nuclei but no fibrinoid necrosis []. She responded dramatically to a course of oral steroids in the form of Prednisolone 40 mg/day and topical broad spectrum photo-protection. Within seven days, her fever had subsided and within the next two weeks most of her skin lesions regressed. The oral steroids were tapered off in another 4 weeks. There was no recurrence of lesions after three months of follow-up. |
A 57-year-old man with cough and minimal whitish expectoration was diagnosed with adenocarcinoma after a biopsy was conducted on the upper lobe mass of his left lung on March 4, 2016. Mutational analysis revealed that he lacked EGFR mutations or ALK gene rearrangements. CT scans showed that this patient had developed multiple metastases, including mediastinal and hilar lymph nodes, and also to the right intrapulmonary lymph nodes. He received 6 cycles of Pem-Cis chemotherapy, followed by thoracic radiation. Review of the CT scan at completion of sequential chemoradiotherapy treatment resulted in a significant shrinkage in the primary tumor in his left lung, while simultaneously resulting in a slight increase in metastatic lymph nodes (Fig. ). In February 6, 2017, CT scans showed that the tumor progressed rapidly. He was then scheduled to receive gemcitabine plus nedaplatin (Gem-Ndp) chemotherapy. However, the patient had to discontinue the planned 2nd cycle of this regimen as he developed moderate pneumonia. After systemic antibiotic treatment, the patient improved symptomatically and became increasingly energetic. However, CT scans on March 29 revealed that his tumors had progressed further. Owing to his poor physical condition after Gem-Ndp treatment, the chemotherapy regimen was changed to Pem-Cis. However, CT scans on May 11 failed to detect any shrinkage in his tumor. In addition, lymph node metastases increased post-treatment. The patient subsequently started standard of care pembrolizumab treatment at 2 mg/kg intravenously every 3 weeks for 6 cycles. During hospitalization, the general condition of the patient was good, with signs of fatigue only present at the first 2 days after each pembrolizumab infusion. The treatment failed to result in an anti-tumor response. The patient then received 2 cycles of initially used chemotherapy regimen Pem-Cis. Interestingly, we detected a significant shrinkage in the enlarged mediastinal and hilar lymph node metastases, with the primary site in the left lung exhibiting no further progression.\nRecent studies have suggested that clinical responses are associated with several potential biomarkers, including PD-1, PD-L1, and CD3.[ To investigate the clinical association of these factors with the efficacy of PD-1 blockade, we performed immunohistochemistry analysis of PD-1, PD-L1, and CD3 in specimens obtained from this patient. The results showed that the tumors from exhibited CD3+ T cell infiltration, but no PD-1 or PD-L1 expression (Fig. ). |
A 6 year-old boy was born at 34 weeks’ gestation to healthy parents with birth weight of 3,540 g after an uneventful pregnancy. He had no family history of epilepsy or focal neurologic sign, but he had delayed development in fine motor and language areas since before 12 months of age. His first seizures, myoclonic seizures, occurred at the age of 8 months, and he was started on antiepileptic drugs (AEDs). At the time of his admission to our hospital at the age of 27 months, he was suffering from multiple types of seizures including drop attacks, generalized tonic seizures, and atypical absence seizures. His EEG showed generalized slow spike and wave discharges with multifocal spikes (), and he was diagnosed as having LGS. Brain magnetic resonance imaging (MRI) showed no abnormalities. Despite various antiepileptic drugs (valproate, topiramate, zonisamide, and clobazam) and a ketogenic diet, his seizures remained uncontrolled. Long term video EEG monitoring for 48 hours showed a dozen seizures a day. The seizure semiology consisted of suddenly dropping his head with losing his tone, thus suggesting myoclonic-atonic seizures. Ictal EEG showed diffuse generalized bursts of polyspike discharges with very brief electromyographic activities. His social quotient was reported to be 27. At the age of 36 months, he underwent anterior 2/3 corpus callosotomy. He achieved a post-operative seizure-free state for 3 months, after which he developed a relapse of his seizures. EEG performed at post-operative 10 months revealed frequent generalized spike and wave discharges and generalized paroxysmal fast activities. His seizures did not respond to either additional vagus nerve stimulation or to medical treatment at that time. As diffusion tensor imaging showed remaining callosal fibers in the splenium, we decided to perform a second total callosotomy (). After the second total callosotomy, the patient maintained a seizure-free state for 27 months and with EEG normalization (). He is still on the medication of valproic acid, topiramate, zonisamide, and clobazam. Developmental test performed after the second surgery showed no remarkable progress by that time. However, according to his parents, his cognitive function is improving compared to that of his pre-operative state. |
A 79-year-old man was admitted emergently with a 3-week history of fever, chill, and abdominal pain. His past history was remarkable for hypertension and diabetes. He also had a history of receiving acupuncture on his back about a month before the onset of symptoms. Clinical examination revealed tender abdomen, tachycardia of 98 beats per min, and temperature of 38℃. Laboratory evaluation revealed a white blood cell count of 28,580/mm3 with 95.6% neutrophils. Abdominal computed tomogram performed at local clinic confirmed the presence of an abdominal aortic pseudoaneurysm from infrarenal to the level of both common iliac arteries with periaortic gangrenous gas formation (). Because of the presence of retroperitoneal pseudoaneurysm and probable rupture, an emergent explo-laparotomy was performed. At emergency laparotomy, a pseudoaneurysm containing gas-forming inflamed friable tissue with hematoma was observed, and the adventitia of the aorta was almost completely destroyed (). The hematoma was partly evacuated and the cavity was debrided. During removal of the infected adventitial tissue, a part of the abdominal aorta suddenly ruptured. By careful management of the patient's vital signs, the ruptured aorta was firmly compressed and the uncontaminated portion of infrarenal abdominal aorta and both common iliac arteries were secured for cross clamping. After the proximal and distal parts were cross clamped, the infected friable aortic walls and pseudoaneurysm were completely removed. Since there was on going inflammation and bacterial infection was highly suspected, we decided to resect the entire infected abdominal aorta and perform an extra-anatomical correction. The aorta was resected obliquely abovel the pseudoaneurysm, preserving the right renal artery. The left renal artery had to be ligated, and both common iliac arteries were also obliterated by applying continuous sutures of prolene 4/0. In addition, right axillary artery to right femoral artery bypass grafting and right femoral to left femoral artery bypass grafting were performed using 8 mm ringed Goretex grafts.\nHistological examination of the resected abdominal aorta showed far advanced atherosclerosis, which was complicated with ulceration, severe aneurysm, and superimposed necrotizing inflammatory infiltrates involving mural and adventitial soft tissue, consistent with necrotizing aortitis. The cultures of the aortic wall and atheroma revealed many Escherichia coli and methicillin-resistant Staphylococcus aureus (MRSA).\nPostoperatively, the recommended antimicrobial therapy with vancomycin, meropen, and metronidazole was continued during hospitalization. Postoperative follow-up abdominal CT performed after one month revealed organized fibrotic tissue at the aortic resection area and the patent bypass grafts (). After 30 days of ICU treatment because of acute renal failure, pneumonia, and 46 days of hospitalization, the patient was discharged. |
A 64-yr-old man was admitted to the hospital because of an abnormal shadow, about 1-cm sized solitary pulmonary nodule (SPN), on chest radiography. He was diagnosed as chronic obstructive pulmonary disease (Global Initiative for Chronic Obstructive Lung Disease criteria class II) in 1998 and had taken medical treatment for the disorder. He was a 30 pack-year smoker.\nChest radiographs showed a round, smooth-margined SPN in the left upper lobe (). Chest CT showed an SPN measuring 12×11×11 mm in the anterior segment of left upper lobe (). Dynamic contrast-enhanced CT scans also revealed that the SPN was well enhanced above 60 Hounsfield Unit (HU) in early phase and showed an early washout pattern ().\nThe patient underwent a left thoracotomy and a wedge resection was performed for the pulmonary tumor with diagnostic and curative purpose. The tumor was well-circumscribed, grayish-white on cut surface and measured 12×10 mm in diameter (). There was no necrosis or hemorrhage. The tumor was not encapsulated but it was relatively easy to separate from the surrounding pulmonary parenchyma.\nHistologically, the tumor consisted of sheet of neoplastic cells surrounded by thin-walled blood vessels with various sizes. Relatively numerous clefts resembling bronchiole or metaplastic alveoli were recognized (). The vasculature was prominent and often tumor cells were arranged in a radial fashion around blood vessels. However, there was no vessel with a thick muscular wall. The tumor cells were polygonal with abundant clear to eosinophilic granular cytoplasm and distinct cytoplasmic membranes (). Few mitotic figures were recognized in 50 high power fields (HPF). The clear cytoplasm contained numerous glycogen granules as demonstrated by PAS staining. The immunohistochemical studies showed strong immunoreactivity for HMB-45 in most tumor cells () and some cells showed a positive reaction to S-100 protein, but no reactivity for cytokeratin. The diagnosis of the benign clear cell sugar tumor of the lung was established. For exclusion of the metastatic renal cell carcinoma, abdominal, and pelvic CT was performed and showed no abnormal findings.\nNo postoperative complications occurred. At 2-month follow-up, he had no complaints and no evidence for the disease was found. |
A 46-year-old female patient of schizophrenia (DSM-IV) criteria came to OPD of psychiatry in Indira Gandhi Government Medical College and Hospital, Nagpur. This institute is also a regional center for reporting an adverse drug reaction for central India. The patient was suffering from schizophrenia since last 18 years and was on fluphenazine (depot) administered intramuscularly (25 mg) once in a month and without any evidence of movement disorder. Since last 6 months, she developed continuous chewing, lip licking, and pouting movements, and diagnosis of TD was done by a psychiatrist. She scored 7 on the Abnormal Involuntary Movement Scale. Her routine biochemical profile and hemogram was in normal limits. Then, fluphenazine administered intramuscularly was discontinued and the psychiatrist decided to start clozapine tablet in the dose of 50 mg twice daily, and after 1 week, the dose was increased to 100 mg twice daily. The total blood count was determined weekly. The patient returned after 2 weeks for follow-up with reduced symptoms of TD, no symptoms of schizophrenia, but complaining of excessive sedation because of clozapine.\nKeeping in mind that sedation may be due to higher dose of clozapine, the psychiatrist decided to reduce the dose of clozapine to 150 mg (half tablet of 100 mg in the afternoon and 1 tablet at night). Again, 2 weeks later, the patient visited the OPD and exhibited increased symptoms of TD. A complete past history from relatives was taken regarding compliance of drugs. She was taking medication regularly and properly. The patient had decreased symptoms of TD after 10 days when the dose of clozapine again increased to 200 mg/day. The biochemical investigation and hemogram were normal. The patient is presently maintained on clozapine 200 mg/day without any re-emergence of TD since 3 months.\nTill now, very few reports of clozapine-related TD are available in the existing literature. In one rare case, it was found that after 1 year of treatment with clozapine a patient developed TD.[] Above-mentioned findings suggest that clozapine at the dose of 200 mg reduces symptoms of TD but when 150-mg dose of the same drug was given, symptoms of TD again re-emerged. A similar case of TD was reported with reduction in the dose of clozapine.[] There is no proven effective treatment for TD. The available literature supports the use of clozapine in the management of TD.[] The mechanism for TD is not well understood but the most commonly accepted explanation is super-sensitivity of post-synaptic dopaminergic receptors due to chronic dopaminergic blockade by antipsychotics. Older antipsychotics are more likely to cause this phenomenon, as they have stronger and irreversible dopamine blockade. Newer antipsychotic have serotonin- as well as dopamine-blocking property and slightly lower risk of TD.[] All first-generation antipsychotic agents are associated with a risk of TD. Studies of newer antipsychotic agents suggest that TD liability is much lower with second-generation agents and clozapine is associated with substantial lower risk for the development of TD than other antipsychotic medication.[] Although family and twin studies have elucidated the possible role of genetic factors, there is no significant association between single-nucleotide polymorphism and TD.[]\nIn this patient, due to reduction in the dose of clozapine, the post-synaptic dopaminergic receptors may not be completely blocked and the increased amount of natural dopamine reaching these super-sensitized receptors leads to dysregulated movements giving rise to TD. However, the exact pathophysiological mechanisms are still not clear and more data are required to know the typical characteristics and risk factors associated with TD due to reduction in the clozapine dose. |
A 62 year-old male had displayed bradykinesia and tremor of his right limbs for one year, during which he was able to perform limited fine movements such as dressing himself, lacing up his shoes and brushing his teeth. His tremors were aggravated by nervousness and relieved when asleep. He had had a history of hypertension and took a daily dose of 5 mg amlodipine. The patient had no history of any other chronic illnesses and was not on any other type of medication. Neither the electrocardiogram nor the Holter monitor showed any abnormalities. His baseline recumbent-upright blood pressure (BP) and heart rate (HR) were normal prior to treatment with piribedil, as shown in Table . He was diagnosed with PD based on the Movement Disorder Society clinical diagnostic criteria []. Initially, he received dopamine replacement therapy of 50 mg piribedil per day. Although there was no significant improvement in symptoms neither did he feel any discomfort. Therefore, starting the first dose change of piribedil, he added extra 50 mg to his dose. About two hours later after the first change in dose, the patient experienced symptoms of dizziness and sweating; he collapsed half an hour later. Whilst in a sitting position, the patient’s BP and HR were measured immediately. The BP reading was 85/48 mmHg and HR was 45 beats/min. His symptoms continued for the duration of the day with sitting BP fluctuating between 80–95 mmHg to 45–68 mmHg. Because his head computerized tomography examination found no abnormalities, the patient received 500 mL of 0.9% sodium chloride solution, after which his symptoms improved. Due to adverse drug reactions (ADRs), the patient was started on a second dose change of piribedil, i.e. an extra dose of 25 mg piribedil was to be taken in the afternoon in addition to the existing 50 mg taken in the morning. After two and a half hours, the patient experienced dizziness, sweating, nausea and vomiting, with a BP reading of 70/45 mmHg and a HR of 47 beats/min when sitting. His BP recovered to 105/65 mmHg and HR to 60 beats/min after elevating his lower limbs and resting for 20 mins. As a result of these incidences, the patient was switched to pramipexole to replace piribedil. After taking pramipexole 0.125 mg or 0.25 mg three times a day (tid), the symptoms of hypotension and bradycardia disappeared but a reduced amplitude of his right arm swing was still observed. Finally, after the pramipexole dose was increased to 0.375 mg tid, the patient showed marked improvements in motor symptoms. The changes in BP and HR are shown in Fig. . |
A 56-year-old woman reported a 5-year history of otitis media of the right ear and subjective hearing loss. She was previously treated elsewhere with ventilation tubes and topical antibiotics. The patient presented to us complaining of intermittent purulent otorrhoea of the right ear, with otalgia. She denied any vertigo, imbalance or tinnitus. Her past medical history was unremarkable.\nPhysical examination revealed purulent otorrhoea of the right ear, with the ventilation tube in situ. Rinne testing was negative on the right. Neurologic examination, including evaluation of the cranial nerves, and head and neck examination were normal.\nAudiometry showed a mixed hearing loss of the right ear with a 40–50 dB conductive component. During the follow-up there was no significant improvement of signs and symptoms, and therefore, additional diagnostic imaging was suggested.\nHigh resolution Computed Tomography (CT) in the axial plane with coronal reconstructions of the petrous bones revealed on the right side a soft tissue mass filling up the entire middle ear cavity and mastoid cells (Fig. ). The ossicular chain was intact and not displaced. There was no evidence of destruction of the petrous bone or mastoid. These findings were primary compatible with chronic otitis media in which a cholesteatoma could not be ruled out.\nA middle ear and mastoid exploration was performed via a combined aproach tympanotomy (CAT). After mastoidectomy, a soft tissue mass was found filling the antrum and middle ear, which bled easily on touch. A clear boundary between bone and tumor mass was not observed, suggesting infiltration. The tumor was removed as radical as possible, with preservation of the ossicular chain and facial canal. After surgery, the patient recovered promptly and improvement of hearing was seen.\nHistopathological examination of the tumor revealed a meningioma with numerous intracellular pseudolumina with round eosinophilic periodic acid Schiff (PAS), epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA) positive bodies, so-called pseudopsammomabodies (Fig. ). This entity is known as a secretory meningioma.\nMagnetic resonance imaging (MRI) of the head was performed to assess the extent of the tumor. In the middle cranial fossa on the right side, a homogeneous hyper-intense mass on the axial contrast enhanced T1-WI could be appreciated, in close contact to the temporal bone and with extension into the sphenoid sinus (Fig. a). Thickening and hyper-intense signal intensity on the T1–WI of the tentorium on the right side represent a so-called “dural tail”. On the coronal and sagittal T1–WI the extension of the mass into the petrous bone with spread to the epitympanum could be seen (Fig. b, c). The brain parenchyma was unremarkable.\nAt 6 months follow-up, MRI showed a slight increase of tumor volume in the temporal bone without any change of tumor enhancement of the tentorium. Follow-up at 1 year showed no changes in tumor volume or extension on MRI. No new symptoms developed 1 year after surgery. The patient will be followed for symptoms and signs at regular intervals with six monthly repeat of MRI. |
A 39-year-old female presented with a history of abdominal hysterectomy 10 years back followed by exploratory laparotomy and adhesiolysis for adhesive intestinal obstruction 5 years back, development of incisional hernia, and repair by open onlay mesh hernioplasty 2 years back. Physical examination showed a supra- and infraumbilical incisional hernia with a periumbilical midline scar (). She underwent ultrasonography of the abdomen; further radiological investigations could not be done. There was a defect 11 cm long and 6 cm wide around the umbilical region containing omentum. We decided to perform a laparoscopic e-TEP Rives repair in March 2018. The operative technique has been followed according to the technique put forward by Belyansky et al., except for the transfascial suture reconstruction of the linea alba []. The omentum was reduced, the midline defect was closed with interrupted transfascial 1-0 polypropylene sutures, and the defect in the posterior layer was closed with 2-0 polygalactin continuous without any tension. Then, a polypropylene mesh measuring 21 cm long and 14 cm wide was placed in the retrorectal space to ensure adequate overlap of the mesh edges. The previous onlay mesh did not need removal because it was in a completely different plane. She had an uneventful postoperative recovery and was discharged on the 4th postoperative day (). In 1-year follow-up, she is completely free of any complaints.\nA 45-year-old female had a history of open cholecystectomy 19 years back and developed hernia in the medial part of the incision at the right subcostal region. She underwent an onlay mesh repair with a polypropylene mesh 8 years back which recurred after 2 years. She underwent intraperitoneal onlay (IPOM) mesh repair with a proceed composite mesh (Ethicon) tacked with a securestrap (Ethicon) and transfascial sutures 4 years back; after 6 months, it recurred again along with another infraumbilical port site hernia. She was subjected to a contrast-enhanced computed tomography of the abdomen, which showed the hernias: right subcostal hernia measuring 6 × 6 cm and the infraumbilical port-site hernia about 4 × 4 cm in size (Figures and ). In June 2018, we did a retrorectal Rives-Stoppa repair with right-sided transversus abdominis release (TAR) following the principles of Belyansky et al. [] (Figures –). After reduction of the hernia, the hernia defects were approximated with no. 1 polypropylene, the peritoneum was opposed with 2–0 polygalactin continuous (), and retromuscular polypropylene mesh was placed (). A component separation in the form of TAR was needed in this patient due to the unusual location of the hernia, viz. subcostal; TAR provided the space to be extended beyond the costal margin on the right side; closure of the posterior layer without tension and retromuscular/retrorectal mesh placement was possible only with the addition of TAR. Though we witnessed the previous remains of the IPOM mesh, it was found densely adhered to the omentum and intestines, so we did not try to dissect it further or to remove it. She was discharged on the 6th postoperative day without any events. At 10-month follow-up, she did not have any complaints.\nA 30-year-old male had epigastric hernia for the last 10 years; he had undergone IPOM repair with Composix™ E/X mesh (polypropylene/e-PTFE prosthesis for laparoscopic ventral hernia repair, Bard) 1 year back which recurred after 3 months. On examination, he had 6 cm × 6 cm epigastric hernia. A preoperative computed tomography scan showed the hernia with the same dimensions along with the radiopaque e-PTFE layer mesh (Figures and ). In August 2018, we did laparoscopic Rives-Stoppa repair, the removal of the previous mesh with a placement of the retrorectal polypropylene mesh (). He developed a collection detected after 5 days, which started increasing (); so we put a drain on the 10th day; about 600 ml of serosanguinous fluid that came out the drain was kept for 8 days more after which it was removed. The drain wound healed completely on the 20th day postoperatively. Repeat CECT abdomen showed a well-apposed mesh in the parietal wall. He is completely satisfied at the 6th month of follow-up. |
An 82-year-old lady was referred to gynaecology outpatients in June 2007 with a one month history of post menopausal bleeding. Her past gynaecological history included a negative hysteroscopy in 1998, and previous use of hormone replacement therapy. She had previously given birth to two children. The patient was fit and well, with no significant past medical history apart from hypertension for which she took bendroflumethiazide and atenolol.\nPhysical examination revealed a bulky uterus with no adnexal masses. A pipelle biopsy demonstrated only tiny fragments of blood clot. A subsequent transvaginal ultrasound scan showed a large endometrial mass with calcification (Figure ). The ovaries appeared normal. She underwent a hysteroscopy in July 2007 when a 6 cm uterine fibrotic polyp, which filled the uterine cavity, was removed.\nMicroscopy demonstrated polypoid tissue with a variably cellular and fibrotic stroma, focal adipose and possible chondroid metaplasia, but no malignant features. The glands showed focal mucinous and keratinising sqaumous epithelial metaplasia. There was focal nuclear atypia, focal mitotic activity and occasional cribriform gland fusion. These features were in keeping with either atypical complex hyperplasia within an endometrial polyp associated with metaplastic changes, or a polypoid uterine teratoma.\nImmunohistochemistry showed positive staining of the small crowded epithelium for the epithelial marker cytokeratin (CK)-7 and the thyroid and lung marker TTFI. There was positive staining of the chondroid area for S100 protein, focal staining of dilated gland epithelium and stromal cells for oestrogen receptor and progesterone receptor, and staining of stromal cells for smooth muscle α-actin (SMA). Thyroglobulin, desmin, CK20 and CDX2 staining was negative. A diagnosis of benign teratoma with thyroid gland and cartilaginous elements was therefore made.\nFollowing hysteroscopy, the bleeding continued. A repeat ultrasound scan revealed that the teratoma had grown back almost completely filling the uterine cavity. A magnetic resonance imaging (MRI) scan in November 2007 showed the tumour filling and distending the endometrial cavity and extending down into the cervix (Figure ). There was evidence of posterior wall myometrial invasion but there was no lymphadenopathy and the ovaries appeared normal. Tumour markers including alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA) and Ca19-9 were within normal limits. Serum Ca125 was slightly elevated at 42 U/ml (normal range 0–35 units (U)/ml) and lactate dehydrogenase (LDH) raised at 372 IU/L (normal range 125–250 U/ml).\nThe patient proceeded to a total abdominal hysterectomy and bilateral salpingo-oophorectmy in December 2007. At operation, the uterus was found to contain a haemorrhagic polypoid tumour (110 × 80 × 70 mm) arising from the posterior aspect of the endometrial cavity (Figure ). Uterine size was equivalent to that of a 12-week gestation uterus.\nMicroscopically the tumour was a teratoma containing mature and immature elements with mixed malignant transformation (Figure ). The tissue types found included squamous and glandular epithelium, thyroid parenchyma, smooth muscle, connective and adipose tissue. In addition there were areas of immature bone, invasive adenocarcinoma, and papillary thyroid carcinoma. There was extensive lymphovascular invasion and deep myometrial, but not serosal, involvement. The omentum, cervix, fallopian tubes and ovaries were free of tumour. Immunohistochemistry showed that the malignant epithelial components were positive for CK-7 and TTF-1, but negative for CK20 and thyroglobulin. One area of the tumour stained positive for desmin but not for SMA, S100 or CD10, suggesting that this is likely to be a small focus of myogenic sarcoma.\nThe histopathological conclusion was of a poorly differentiated adenocarcinoma and a focal myogenic sarcoma arising in a polypoid uterine teratoma with mature and immature elements. A post-operative computer tomography (CT) scan of the thorax, abdomen and pelvis found no evidence of distant disease giving an overall International Federation of Gynaecology and Obstetrics (FIGO) disease stage 1C.\nThe patient recovered well from surgery and was referred for oncological follow up. Given her age and performance status a surveillance approach was taken with regular clinical examinations, serial tumour markers and routine CT scans. Initially in remission, six months post-operatively para-aortic lymphadenopathy was detected on CT although she remained asymptomatic with an Eastern Cooperative Oncology Group (ECOG) performance status of 0. In view of her age and wishes for a treatment with acceptable toxicity, the patient was commenced on an initial dose of cisplatin (20 mg/m2) and etoposide (100 mg/m2). This was well tolerated so one week later treatment was continued with a fortnightly alternating regimen of paclitaxel (135 mg/m2) and etoposide (150 mg/m2), followed by paciltaxel (135 mg/m2) and cisplatin (60 mg/m2). This treatment was chosen based on our experience of its effectiveness and tolerability in the treatment of relapsed germ cell tumours and gestational trophoblastic disease [,].\nAfter three cycles of chemotherapy there was a reduction in the size of the para-aortic mass, but an increase in the cystic component suggesting possible differentiation towards a mature teratoma. Consequently she underwent a retro-peritoneal lymph node dissection in October 2008. Histology from this confirmed the presence of metastatic teratoma. Unfortunately she had a turbulent post-operative course and, although she recovered well enough to return home a month later, she sadly died shortly thereafter. |
A 42-year-old Saudi male presented to our emergency room with a history of upper gastrointestinal bleeding in form of hematemesis and melena for 1 day prior to his presentation. It was preceded by 2 days of persistent nausea and repeated vomiting. He denied symptoms of chronic dyspepsia, NSAIDs or alcohol consumption. There was no history of abdominal trauma or background of chronic liver disease. Upon assessment, he was fully conscious, oriented, nonicteric, and dehydrated, with no peripheral or central stigmata of chronic liver disease. Two hours after the arrival, he experienced another attack of hematemesis, a total amount of around 150 mL. Room air oxygen saturation was maintained at 98%, his pulse was 110 beats/min, and his blood pressure was 102/68 mm Hg. There was no significant postural drop of blood pressure. Chest examination showed a decreased air entry in the right lower zone. Cardiovascular system examination was within normal limits. His abdomen was soft with mild epigastric tenderness; there was no rigidity or rebound tenderness. His initial routine laboratory test is presented in Table . After the patient's condition had been stabilized with initial resuscitation, blood transfusion was carried out and PPI infusion was initiated. Urgent gastroscopy revealed a normal esophagus; the stomach was obscured with fresh blood and blood clots, and intubation of the pylorus was difficult due to anatomical distortion (organoaxial volvulus). However, intubation of the pylorus achieved after several attempts. The second part of the duodenum was partially compressed with normal mucosa. Gastric (fundal) mucosa was severely inflamed with variable sizes of multiple ulcers (Fig. ). Some of them were actively oozing blood, which required adrenaline injection to achieve homeostasis. The patient was admitted to the intensive care unit and observed closely for any clinical deterioration, sign of perforated viscus or peritonitis. Chest X-ray (Fig. ) showed a raised right hemidiaphragm with homogenous haziness in the right lower lung lobe and obliteration of the right costophrenic angle. Erect abdominal X-ray showed a dilated stomach with an air/fluid level and a gas-filled bowel loop behind the cardiac shadow blunting the cardiophrenic recess (Fig. ). Contrasted CT scan was carried out early after recovered acute renal injury and revealed pancreatitis with prepancreatic collection (Fig. ), splenic vein thrombosis and intrathoracic herniation of the fundus of the stomach, antrum, and pylorus bowel loop along with an omentum into the posterolateral segment of the chest cavity (Fig. ). Pancreatitis was assumed to be related to impaired blood flow and pressure effect. A surgical approach was considered early. The patient was kept under very close observation in the intensive care unit.\nHis clinical and biochemical parameters improved and a second endoscopy evaluation was done after 5 days of admission showing that the previous gastric ulcers had significantly healed. He was subjected for corrective reconstructive surgery. Surgical intervention was done through a thoracotomy approach to reduce the abdominal content and repair the hernial defect. His postoperative course was smooth. Postoperative chest X-ray (Fig. ) was almost normal. He was discharged a few days after surgery with complete clinical recovery. |
An 8-month-old female child with single ventricle physiology (double-inlet left ventricle) with hypoplastic mitral valve (9 mm, Z score = −2.44) and intact atrial septum and unobstructed pulmonary blood flow needed PA banding and creation of an inter-atrial communication.\nAfter detailed evaluation of the child and taking informed consent from her parents, the child was prepared under anesthesia as for open-heart surgery and the procedure was carried out through a median sternotomy. The patient was heparinized. A purse-string suture was made on the right atrial (RA) appendage. A stab was made within the purse string, and through this, thoracoscopy scissors [] was introduced in the RA. Under TEE guidance, the closed tip of the instrument was used to tent the fossa ovalis below the limbus [] and a small perforation was created. It was then dilated by stretching with the opening of the blades of the scissors []. The orifice was assessed and found to be restrictive with a peak gradient of 4 mmHg and a mean gradient of 2 mmHg.\nThe scissors was then rotated to face inferiorly with one blade on either side of the inter-atrial septum, and two cuts were made in the inferior and lateral directions, away from the AV valves and conduction tissue, creating an orifice of maximum size 9 mm with free laminar flow across the resulting defect []. PA banding was done as per Trussler's formula to 27mm.\nThe child recovered uneventfully and was discharged from the hospital on the 6th postoperative day. The PA band gradient on the pre-discharge echo was 60 mm Hg. At 1 month follow-up, the child was well and symptom free with an oxygen saturation of 92%. The child had gained 0.5 kg in the 1 month following discharge. The follow-up echo at 1 month showed a band gradient of 50 mm Hg. The atrial septal opening measured 9 mm with laminar flows across the defect. A bidirectional Glenn shunt is planned in the subsequent 3-6 months. |
A 61-year-old man, previously radically resected for a renal cell carcinoma, was diagnosed with an 11-cm tumour located at the stomach 15 years ago (November 2005) (shown in Fig. .1; Table ). This gastric tumour was primarily resected with free margins. Histopathology reported an epithelioid GIST, immunohistochemically positive for CD117 and CD34. A mitotic count of 20 per 50 high-power fields under lineated high risk of progression was found according to the AFIP criteria [].\nIn April 2006, a recurrence in the lymph nodes in the lower pelvic and in the inguinal was discovered on imaging. First-line treatment with imatinib 400 mg orally per day was initiated. The disease progressed in May 2006, and thereby the dose of imatinib was increased to 400 mg orally twice daily. A week later, the patient developed epidermolysis. The treatment was stopped in July 2006 after 3 months of treatment due to imatinib intolerance. Due to the skin reaction, prednisolone 50 mg orally was administered. The following PET-CT showed regression of the disease, and therefore it was concluded that the presumed recurrence most likely had been a reactive change in the lymph nodes since it disappeared while on treatment with prednisolone.\nSixteen months after stopping imatinib, PET-CT revealed a recurrence with 3 intra-abdominal tumours of 4.5 cm, 3.1 cm close to the liver and 2.6 cm close to the gall bladder (shown in Fig. ), and it was verified by biopsy. Treatment with imatinib 200 mg orally once daily was reintroduced. Again, the patient developed a severe skin reaction after a few days of treatment. Second-line treatment with continuous sunitinib 25 mg orally once daily was initiated covered by 3-week treatment with prednisolone 25 mg daily. The dose was then increased to 37.5 mg sunitinib orally daily.\nAfter 3 months of treatment with sunitinib, the disease progressed on PET-CT scan in 2 tumours intra-abdominally. Third-line treatment with nilotinib was initiated, 400 mg orally twice daily. After 1 month of treatment, a new CT scan of the abdomen revealed progression of one of the existing tumours in the abdomen and in the left liver lobe with 2 new tumours. In lack of other treatment options, imatinib 200 mg orally once daily was reintroduced with steroid coverage with prednisolone 100 mg orally once daily. Unfortunately, the patient developed a urticarial rash, and the treatment was interrupted. Again, due to lack of treatment options, a second go with nilotinib was tried.\nIn June 2008, the disease progressed, but the treatment continued until the next CT scan. In August 2008, a cystic tumour process with a peripheral solid component in the pelvis was drained for 3 L of bloody fluid. A CT scan confirmed further progression in August 2008 (shown in Fig. ). At that time, treatment with sorafenib became available and was administered as 400 mg orally twice daily. After 1 month, the dose was reduced to 400 + 200 mg orally due to toxicity. On treatment with sorafenib, the patient had partial regression with measurable targets according to Response Evaluation Criteria in Solid Tumours (RECIST) 1.1 regressing from 13.7 cm to 9.5 cm during a 1-year period (shown in Fig. ) []. In September 2009, the patient underwent surgery including cholecystectomy, partial liver resection, and resection of peritoneal disease. No residual tumour was left behind. Postoperatively, sorafenib was restarted at 200 mg orally twice daily.\nOver the next years, full dose of sorafenib was not tolerated due to side effects, especially diarrhoea. In May 2013, the dosage was 200 mg orally per day.\nEven though the dose of sorafenib was reduced, the patient had 2 non-ST segment elevation myocardial infarcts. Therefore, it was necessary to perform a coronary artery bypass graft in 2012 and a percutaneous coronary intervention in 2015. In addition, an investigation was carried out due to proteinuria which was concluded to be caused by a mild chronic kidney insufficiency. The cardiac and kidney affection was treatment related.\nIn April 2014, a mutation analysis was performed on archived material from the primary tumour from 2005 which revealed a deletion in exon 18 of PDGFRA involving c.2527_2538delATCATGCATGAT, p.Ile843_Asp846del (p.I843_D846del), and wild-type c-KIT. At primary diagnosis in 2005, mutation analysis was not part of daily practice.\nIn September 2017, a recurrence of GIST in relation to the ileum was discovered on CT imaging which retrospectively could be detected already in August 2016, and biopsy was carried out (shown in Fig. ). The disease had then been stable, without recurrence, in 95 months during adjuvant treatment with sorafenib after resection of recurrence. Histopathology confirmed the diagnosis of GIST and an unchanged mutation status. Sorafenib was increased to 400 mg orally once daily. Three months after dose escalation, image evaluation showed a 15% reduction in tumour size (shown in Fig. ). Since the patient was in performance status 0, resection of the tumour of 2.7 cm was performed in February 2018. Resection was complete with free margins.\nAfter tumour resection, sorafenib 400 mg orally once daily was reintroduced, and the patient is still in treatment with the same dose of sorafenib in March 2021. The patient had acceptable side effects such as diarrhoea and hypertension and is still without signs of recurrence (shown in Fig. ). |
A 26-year-old man with general weakness, peroneal atrophy, and deformities in the extremities presented at our hospital. The patient's parents and 2 elder sisters were healthy and free of any neurological diseases. The patient reported that he was born at term in his home by normal spontaneous vaginal delivery, and the birth was assisted by a midwife. His birth weight, height, and head circumference were not noted. The patient's bilateral thumbs and feet were weak at birth. The patient could walk by the age of 6 years, although he had an unstable gait and fallen arches in his feet. The arches in his feet developed gradually. The patient underwent bilateral straightening of the feet at a local hospital at the age of 16, which resulted in greater improvement in his right foot. The patients had visited multiple hospitals, but the etiology remained unknown. His parents reported that he had no history of seizures. The patient had a history of chronic gastritis for 10 years, while he was at junior high school; however, he had no other previous history of any disease and was not addicted to either alcohol or tobacco.\nWe examined the patient in August 2019. His vital signs, measured at the time of admission, were normal (body temperature: 36.5°C; pulse rate: 104 beats/minute; respiratory frequency: 20 breaths/minute; blood pressure: 123/85 mm Hg). The patient walked with a myopathic gait because of weakness in the lower extremities. We observed variable scoliosis, deformities of both thumbs and both feet (pes cavus), and symmetric atrophy of the bilateral thenar muscles, bilateral hypothenar muscles, and bilateral muscles of under knees. Muscle strength in the bilateral upper distal limbs, bilateral upper proximal limbs, bilateral lower distal limbs, and bilateral lower proximal limbs was grade IV, grade IV+, grade IV, and grade IV−, respectively. Tendon areflexia in the extremities, deep sensory dysfunction of the right leg, and the Romberg sign were all also observed.\nResults from the CSF protein test were positive, and further laboratory tests showed that albumin, IgA, and IgG were elevated in the CSF. The concentration of serum neuron-specific enolase was slightly elevated (24.29 μg/L; ≤16.30 μg/L is normal), and serum creatine kinase was very elevated (529 U/L; ≤190 U/L is normal). Analyses of routine blood and urine, stool, as well as hepatic and renal function, and tumor markers showed no clinically significant abnormal values related to the syndrome. Additionally, all antibodies of autoimmune diseases were negative.\nCranial routine MR showed no obvious abnormalities in the brain. Routine MR of both the right and left hand demonstrated marrow edema at the end of the first metacarpal bone, malunions of the first metacarpophalangeal joint, and a small number of effusions in the first metacarpophalangeal and wrist joints. Routine MR of the left foot revealed joint effusions in the ankle, intertarsal, tarsometatarsal, and metatarsophalangeal joints, as well as a slight narrowing of both the talocalcaneal and calcaneocuboid joint spaces. In addition, edema of the posterior tibial muscle, sole muscles, and fascia were observed, as well as swelling of the ankle joint and soft tissues around the foot. Routine MR of the right foot revealed joint effusions in the ankle, taloscaphoid, talocalcaneonavicular, and metatarsophalangeal joints, as well as swelling of the ankle joint and soft tissues around the foot, and severe narrowing of the talocalcaneal joint space. MR images of bilateral hands and feet are shown in Figure .\nEMG results revealed that nerve conduction was not elicited in the motor and sensory bilateral median nerves, bilateral ulnar nerves, bilateral tibial nerves, and common peroneal nerves. Spontaneous potential was observed from the right anterior tibial muscle, and EMG results revealed neurogenic damage. Spontaneous potentials were also observed from the right abductor pollicis brevis, but motor unit potentials were not recorded. Detailed EMG results are shown in Tables and . Above EMG results indicated that the patient has a polyneuropathy.\nGenetic analysis of the patient's blood sample revealed a heterozygous mutation in PMP22 (NM_153322, c.54_55insGTGCTG, p.(L19delinsVLL)), which has uncertain significance according to the American College of Medical Genetics and Genomics (ACMG).[ Other than that, no other pathogenic mutations were found in WES. The verification results of the positive mutation using Sanger sequencing are shown in Figure . The PROVEAN program analyzed the variant to be “deleterious”, with a score of -9.286.[ The wild-type and mutation structure of PMP22 were created via SWISS-MODEL program (Fig. a and b). By comparing Figure a and Figure b, it is easy to find that one of alpha helixes structure was disrupted according to the predicted 3D structures. |
An 83-year-old man consulted his family doctor due to dyspnea. The dyspnea increased in the last weeks and occurred mainly during physical activity. No other symptoms were reported. Apart from impaired hearing, the patient’s history was uneventful. So far, no colonoscopy was performed. Furthermore, the patient had no previous abdominal surgery. Although he was a retired farmer, he continued to work on his farm and in former years, he regularly went to the forest. Physical examination of the patient was inconspicuous. Laboratory analysis revealed a microcytic, hypochromic anemia. For further assessment of the anemia, a colonoscopy was performed and revealed a non-stenosing carcinoma of the ascending colon. No distant metastasis were found by staging computer tomography scan. During primary surgical resection, routine intraoperative ultrasound showed a 10 mm hepatic lesion in segment V (Fig. a). Due to a suspected liver metastasis of the colon cancer, resection of the lesion was performed. A 76-year-old woman consulted her family doctor for stool irregularities. No further symptoms were reported. A normocytic, normochromic anemia was diagnosed but otherwise, the examination of the family doctor showed no abnormality. A colonoscopy was initiated and a stenosing mass extending from the distal colon to the proximal sigmoid colon were found. After histological confirmation of an adenocarcinoma, preoperative staging by computer tomography showed a focal liver parenchymal change in segment VI with a metastasis-compatible contrast enhancing (Fig. b). Intraoperatively, an approximately 6 mm, hyperechogenic structure in segment VI could been visualized by sonography. A left hemicolectomy was performed with an excisional biopsy of the hepatic mass.\nThe pathological workup of both colorectal cancers showed a moderately differentiated pT3 adenocarcinoma with no lymph node metastasis. The examination of the two liver resection specimens revealed a central necrotic cystic lesion with a granulomatous and lymphocytic rim (Fig. ). Further histochemical staining highlighted the Periodic-Acid-Schiff (PAS)-stain positive germinal layer consistent with echinococcal cysts (Fig. , inserts) []. Vital parasites (scolices) could not be detected, so that the diagnosis was further confirmed by molecular tests using specific polymerase chain reaction (PCR). These tests revealed Echinococcus multilocularis (E. multilocularis) and Echinococcus granulosus (E. granulosus)- DNA in the lesion of the male and female patient, respectively.\nFollowing consultation with the infectious disease specialist, no anti-helminthic treatment was initiated in the male patient due to advanced age, complete resection of the parasitic cyst and lack of vital scolices in the surgical specimen. The female patient was treated with albendazole 400 mg twice per day []. Both colon cancers were nodal negative (AJCC/UICC tumor stage II) and therefore no adjuvant chemotherapy was applied. At three years follow-up, both patients were free of cancer and there was no evidence of parasitic disease. |
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