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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. ). |
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. |
This is a description of a 55-year-old male, banker by profession and of Asian descent, an Urdu speaking Pakistani. He was a long-standing smoker, having smoked 3 packs per day for 30 years prior to presentation. He denied any form of alcohol intake and had been found to have type 2 diabetes 17 years prior to presentation. There was no known history of tuberculosis or any form of calcium disorder in parents, siblings or children. He weighed 47 kg and was 1.65 mt tall.\nHe presented in December 2007 with a 6-month history of unplanned weight loss, of up to 13 kg, associated with recent onset weakness and drowsiness, low grade fever but no cough. Clinical examination revealed a normotensive, lean individual, with crackles in his left upper chest but no focal neurological deficit.\nAn X- ray of his chest revealed a left lingular infiltrate. Brochoalveolar lavage produced a smear positive and culture positive tuberculosis that was pan sensitive. Biopsy of the infiltrate was negative for malignancy.\nHis serum calcium level was high, (), with the initial corrected calcium level at 13.2 mg/dl and a low parathormone level, with normal 25-OH Vitamin D levels and a high 24 hour urine calcium. His serum phosphate was normal. A diagnostic work up for drowsiness and hypercalcemia, (), revealed a normal serum sodium. Serum magnesium was initially low and was corrected to a normal level. Thyroid function tests were initially consistent with hyperthyroidism, with the thyroid scan showing negligible uptake. They subsequently normalized within 4 weeks. Serum protein electrophoresis, liver function tests, angiotensin converting enzyme (ACE) levels and a morning cortisol level were normal.\nMRI brain was negative for tuberculomas. CSF D/R revealed a high protein content, while routine and AFB cultures of the CSF were negative. Vasculitic work up was negative.\nHence, a diagnosis of hypercalcemia secondary to pulmonary tuberculosis was made.\nThe patient was hydrated aggressively, followed by diuresis with intravenous furosemide, 40 mg, once the patient had been well hydrated. Intravenous pamidronate 90 mg was administered twice over a period of two months, as well as daily calcitonin injections at 240 IU subcutaneously twice daily. A nephrology consult revealed that dialysis was not feasible because of non availability of a calcium free dialysate. Subsequently, steroids were started at 40 mg/day, while his anti tuberculous medication was continued, including isoniazid 300 mg, rifampicin 600 mg, ethambutol 1200 mg, pyrazinamide 1500 mg per day.\nSerum calcium fluctuated between 12.0 - 14.2 for a period of two and a half months and his drowsiness, disorientation & hypercalcemia failed to resolve with the initial measures. Once steroids were started, the calcium levels gradually began to resolve (), as did the patient’s confusional status. |
A 25-year-old lady presented to hospital 36 days after giving birth via an uncomplicated lower segment caesarean section. She stated that the scar was well healed since the fifth day post-surgery and she had begun breast feeding. There was no history of nipple trauma or mastitis. She complained of a 15 day history of progressive pain, swelling and decreased range of motion in her right knee. Systemic examination revealed pyrexia of 38.5 degrees Celsius and a tachycardia of 120 beats per minute. Examination of the knee demonstrated a red, hot, swollen, tender joint with minimal range of motion. Radiographs of the knee were unremarkable. The knee was aspirated under aseptic conditions and 35 mls of turbid fluid was collected.\nBlood laboratory results were as follows: white cell count 15.8 × 109/L, CRP > 250 and ESR 75. Initial gram stain of the aspirate showed 3+ of white cells but no organisms.\nIn view of the clinical picture, the patient underwent urgent arthroscopic washout of the knee. It was noted that the synovium was thickened and inflamed, and biopsies were taken. Furthermore, there was a significant amount of pus in the suprapatellar pouch. The knee was washed out with nine litres of normal saline.\nCultures of the pus taken from the knee subsequently grew Staphylococcus aureus sensitive to flucloxacillin. Cytology of the fluid was negative for crystals. The biopsies taken revealed acute synovitis with extensive ulceration of the synovial lining lined by inflammatory exudate composed of fibrin, polymorphs and scattered macrophages.\nThe patient was given intravenous benzylpenicillin (1.2 g QDS) and flucloxacillin (1 g QDS) post-operatively and the patient clinically improved. She underwent a further arthroscopic washout of the knee three days after the original procedure. It was found at the second operation that there was some pus in the lateral arthroscopy portal wound though no pus within the knee joint itself.\nIn total, the patient completed a week's course of intravenous antibiotics and four further weeks of oral antibiotics (Penicillin V 500 mg QDS, Flucloxacillin 500 mg QDS). She was seen in the out-patient clinic 11 weeks after her presentation where she was noted to be doing well with an improved range of motion in her knee of 0-80 degrees. |
A 52-year-old woman diagnosed with PAH and anteroseptal MI was referred to our institute, where she presented with progressive dyspnea on exertion and peripheral edema. She had worked as a nurse without difficulty until she was 45 years old, when she began to experience increased general everyday fatigue. She had never smoked and she denied any illicit drug use.\nIn 2007, the patient had undergone cardiac catheterization at another hospital (pulmonary arterial pressure [PAP]: systolic/diastolic/mean = 72/27/45 mmHg, pulmonary artery wedge pressure [PAWP] = 14 mmHg, cardiac index = 2.16 L/min/m2, pulmonary vascular resistance [PVR] = 9.3 Wood units). Her left ventricular ejection fraction was 67%, left atrial dimension was 32 mm, and lung function was normal. She had subsequently begun combination therapy through that hospital with 125 mg bosentan twice daily and 20 mg sildenafil three times daily.\nIn 2009, the patient presented to her original hospital with prolonged chest pain and low blood pressure; she was considered to be in cardiogenic shock due to acute MI. Emergent coronary angiography revealed 99% stenosis of the left main coronary artery in an abnormally high position because of pressure from an aneurysm of the left coronary sinus of Valsalva (); consequently a Cypher stent (Cordis Corporation, Miami Lakes, FL, USA) was implanted at the patient’s original hospital during intra-aortic balloon pumping (). The aneurysm was not noted at the time during the procedure but was found retrospectively on review of the imaging findings at our hospital. For this reason, no additional examinations were conducted at the time of the procedure.\nIn April 2014, the patient was hospitalized with progressive heart failure and was then referred to our hospital for treatment and management of her pulmonary hypertension (PH). We promptly performed cardiac catheterization (mean PAP = 47 mmHg, PAWP = 21 mmHg, right atrial pressure = 12 mmHg, cardiac index = 2.36 L/min/m2 [Fick principle], PVR = 7.4 Wood units). Physical examination on admission revealed an increased P2 component of S2 and dilated jugular veins. No ascites was present, but the patient’s lower extremities were slightly edematous. An electrocardiogram revealed right ventricular hypertrophy with ST change. The plasma brain natriuretic peptide (BNP) concentration was 450 pg/mL. Transthoracic echocardiography showed that the anteroseptal wall was thin and akinetic and the left ventricular ejection fraction was 37% (); the inferior vena cava was dilated (27.7 mm) (). Pulmonary perfusion scintigraphy revealed no abnormalities. We performed echocardiography and magnetic resonance imaging. Multiple samples of whole blood were withdrawn during cardiac catheterization to measure O2 saturation to rule out other underlying heart diseases, such as primary left heart disease and congenital heart disease. There was no cardiac shunt, valvular disease, or lung disease. In addition, the results of both pulmonary function testing and high-resolution computed tomography (CT) were normal. Together, these findings supported the diagnosis of PH due to left heart disease (group 2 of the Dana Point 2008 classification). In addition, 64-slice multidetector CT at admission showed marked dilation of the main pulmonary artery (47.7 mm) (), no restenosis of the left main coronary artery, and aneurysm of the left coronary sinus of Valsalva (36.0 mm) (). The stent previously placed in the left main coronary trunk was located between the main pulmonary arterial trunk and the left coronary sinus of Valsalva ( and ).\nBefore the patient’s referral to our institution, her treatment had comprised oxygen therapy, bosentan (125 mg twice daily), sildenafil (20 mg three times daily), warfarin (4 mg daily), azosemide (60 mg daily), and spironolactone (25 mg daily); tolvaptan (7.5 mg daily) was added after referral. Thereafter, her pulmonary congestion decreased, as did her plasma BNP level (to 170 pg/mL) and mean PAP (to 38 mmHg). After one month of treatment with tolvaptan, the patient was discharged home. She has been followed through our outpatient clinic: at last review, her PH was in World Health Organization (WHO) functional class II and her plasma BNP level had remained unchanged since discharge.\nThe patient’s elder sister had also been diagnosed with PAH (mean PAP = 35 mmHg, PAWP = 11 mmHg, PVR = 4.8 Wood units). The chest radiographs of the two women were similar in regard to the degree of pulmonary artery dilatation ( and ). However, the sister had no left main trunk stenosis and no aneurysm of the left coronary sinus of Valsalva (), and the left ventricle was of normal size and had normal contractility. There were no signs of left heart disease, congenital heart disease, or lung disease. Because we suspected that these sisters had HPAH, we suggested professional genetic testing. Conventional Sanger sequencing and multiplex ligation-dependent probe amplification analysis of the genes encoding bone morphogenetic protein receptor type 2 (BMPR2) and activin A receptor type II–like kinase 1 (ACVRL1) failed to detect any pathogenic variants, but subsequent whole-exome sequencing revealed a novel heterozygous splicing mutation in the FLNA gene, namely IVS2-2A > G (c.374-2A > G in NM_001456) () in both sisters but not in their father (aged 89 years). This mutation changed the 3’ splice site consensus sequence from AG to GG and was expected to cause aberrant splicing. A four-generation pedigree of the family was generated from their responses to a questionnaire (). |
A 48-year-old Caucasian man, weighing 108 kg (BMI 38) () was scheduled for dental treatment under general anaesthesia. He had been diagnosed with DOOR syndrome as an infant and had typical features, for example: malformations of distal phalanges of the fingers and nails (). Other manifestations during childhood included epilepsy with frequent grand-mal seizures, deafness and significantly delayed psychomotor development. Aside from features associated with DOOR syndrome and obesity, the patient also had left sided cerebral palsy, recurrent chest infections, asthma, gastroesophageal reflux and hypertension. It was difficult to elucidate any history of obstructive sleep apnoea due to his learning difficulties. There was no cardiac defect or urogenital abnormities. His medication included carbamazepine, omeprazole, salbutamol and budesonide. He had no allergies. At anaesthetic preassessment the only findings noted were a thyromental distance of 7.5 cm and good neck mobility. The neck circumference was measured as 40 cm. Further airway assessment, such as the Mallampati score, was impossible. Basic observations were performed, including a blood pressure reading of 150/90 mmHg and a heart rate of 72 bpm. Oxygen saturation on room air was 95%. He was extremely uncooperative, rendering an awake dental examination unfeasible. A plan was therefore made to use short sedation for dental assessment including X-rays and then to administer general anaesthesia for definitive treatment. The patient was premedicated with 4 mg of buccal midazolam and sedated with titrated intravenous midazolam to a total dose of 5 mg and fentanyl 100 mcg. Depth of sedation was assessed using entropy monitoring (GE Healthcare, Chalfont St. Giles, UK). The baseline values of entropy were inside the normal range - SE 87, RE 98. During intravenous sedation the levels dropped to 60 (SE) and 75 (RE) which corresponded with values seen in patients without learning difficulties. The procedure lasted 25 minutes and was uneventful. Based on X-ray examination, the patient was scheduled for multiple extractions and fillings under general anaesthesia. The patient was fasted for 6 hrs, and had his usual dose of antiepileptic and inhalers. Premedication with 4 mg of buccal midazolam was given, after which a 20 G i.v. cannula was placed and ranitidine 50 mg and metoclopramide 10 mg administered intravenously. We routinely use these drugs in learning disability patients as the fasting interval may not be adhered to. Induction was with propofol (2.5 mg.kg−1) and fentanyl (1 mcg.kg−1). After a trial of bag mask ventilation, atracurium 0.5 mg.kg−1 was administered. Due to his specific facial features and short neck a McCoy blade was used in the first instance. The laryngeal view was a Cormack and Lehane grade II and the trachea was intubated at the first attempt with a size 8.0 cuffed RAE tube and a cotton throat pack was inserted. The difficult airway trolley was available and our plan ‘B’ involved the insertion of a laryngeal mask airway. Maintenance of anaesthesia was with sevoflurane in an air/oxygen mixture. Further doses of fentanyl were administered for analgesia to a total dose of 200 mcg. The procedure lasted 100 minutes and included two complicated surgical extractions and four fillings. The dentist infiltrated local anaesthetic. The patient was extubated fully awake without complications. In the recovery ward, twenty minutes after extubation, he developed mild partial seizures affecting his right forearm and hand. At the time of these seizures, the patient was fully awake, with SpO2 97% on oxygen mask, and was haemodynamically stable. The seizure activity lasted 10 minutes and abated spontaneously. Other postoperative complications included a short period of decreased saturations and moderate nausea. The patient was discharged home that same evening, 4 hours post-procedure. Oral paracetamol was prescribed for post operative pain relief. |
A 36-year-old Caucasian woman was evaluated with chief complaint of gluteal pain radiating to her leg. Her medical history was remarkable with gunshot injury to the affected leg with multiple pellets dispersed into her pelvis and proximal part of the thigh, as shown in Figs. and . She had gunshot injury 20 years ago. She was previously diagnosed as having lumbar disc herniation at L4–5 level. She underwent a previous discectomy outside our institution 2 years ago. A radiological examination revealed the presence of recurrent disc herniation, as well as multiple shotgun bullets in her pelvis and thigh. One of those bullets was deep into the sciatic nerve inside her quadratus femoris muscle.\nElectromyography (EMG) showed the presence of chronic sciatic nerve injury. Since it was clinically impossible to distinguish lumbar disc herniation from the sciatic injury, we decided to proceed with removal of the foreign object and neurolysis of the sciatic nerve followed by L4–5 discectomy and fusion. We decided to perform those procedures in two different settings. The first surgery included access to the sciatic nerve in the upper portion of her thigh and exposing the nerve fibrotic bands around the nerve. The dissection proceeded deep into the nerve within a muscle, where a bullet was found and removed. The distance from the bullets to the nerve was approximately 2 cm. Muscle tissue around the bullets was excised for analysis. For comparison, another specimen was obtained from the gluteal muscle, superficially away from the nerve and all the bullets. Two weeks later, she underwent scheduled L4–5 discectomy and fusion. Her postoperative course was uneventful. On follow-up examination at 6 months, she was essentially symptom free.\nA scanning acoustic microscope (AMS-50SI) developed by Honda Electronics (Toyohashi, Japan), whose schematic setup is shown in Fig. , was used in AI mode. It has a transducer with quartz lens, a pulser/receiver, an oscilloscope, a computer, and a display monitor. An 80 MHz transducer is installed within the microscope, which generates the signals and collects the reflected acoustic waves. Water is the coupling medium between the quartz lens and the substrate. For two-dimensional scans, an X-Y stage, controlled by a computer, is used. An oscilloscope analyzes the reflected signals from both the reference and target material after being collected by the transducer. As a result, acoustic intensity and impedance maps of the region of interest with 300 × 300 sampling points are obtained.\nThe principle of SAM in AI mode is demonstrated in Fig. . Distilled water is widely used as reference. The signal reflected from the target is\nwhere, S0 is the generated signal by the 80 MHz transducer, Ztarget is tissue’s AI and Zsub is the polystyrene substrate’s AI (2.37 MRayl). The tissue’s AI is calculated by combining the reflected signals from the tissue and the reference. The signal reflected from the reference iswhere Zref is the AI of water (1.50 MRayl). Then, the target’s AI is written aswith a constant signal S0 [] generated by the transducer.\nElectron microscopy-based imaging and chemical analysis studies were performed in a JEOL JIB-4601 focused ion beam scanning electron microscope (FIB-SEM) multi-beam platform coupled with an Oxford X-MaxN EDS system, as shown in Fig. .\nBlood samples were collected in test tubes containing ethylenediamine-tetraacetic acid (EDTA) and no anticoagulant on the day of the first surgery (foreign object removal) prior to the procedure. Then, 2 ml of 20% trichloroacetic acid (TCA) was supplemented into the blood samples to release the red blood cells (RBC) and other ingredients. The supernatant part was received from blood with TCA by centrifugation at 4000 revolutions per minute (rpm) for 20 minutes for the analysis of Pb and cadmium (Cd) within total blood. Coagulation of blood samples enabled serum trace element analysis: chromium (Cr), Fe, Cu, magnesium (Mg), manganese (Mn), selenium (Se), and Zn. The serum specimen was prepared using Hettich Universal centrifuge by centrifugation at 3000 rpm for 15 minutes, separating from cells immediately after and storing at − 20 °C until the analysis [].\nAfter weighing the left sciatic nerve tissue samples, they were digested with 2 ml of 65% nitric acid (HNO3) at 180 °C in the incubator for 1 hour. Then, 2 ml of 65% perchloric acid (HClO4) was added into the cooled mixture. Then, the mixture was digested at 200 °C in the incubator until the volume was halved. Digested materials were vortexed and diluted in water to a total volume of 10 ml. Concentrations were given in micrograms per gram (μg/g) wet tissue weight [].\nAll glassware were maintained at 10% (volume/volume; v/v) HNO3 before use, cleaned with deionized water, and dried in an incubator at 100 °C overnight. Pb, Cd, Cu, Cr, Fe, Mn, Se, and Zn elements were detected by inductively coupled plasma optical emission spectrophotometer (ICP-OES 6000, Thermo, Cambridge, United Kingdom). Measurements for each element were done three times and averaged. The ICP-OES was operated with argon carrier flow rate of 0.5 L/minute, plasma gas flow rate of 15 L/minute, sample flow and elusion rate of 1.51 L/minute, and peristaltic pump speed of 100 rpm, selecting the suitable wavelength for Pb, Cd, Cr, Cu, Fe, Mn, Se, and Zn, which were 220.353 nm, 228.802 nm, 267.716 nm, 324.75 nm, 285.213 nm, 357.610 nm, 196.090 nm, and 206.200 nm, respectively. Transport lines were obtained using 1.25 mm internal diameter polytetrafluoroethylene tubing. Element levels were indicated in micrograms per deciliter for serum (μg/dl) and μg/g for wet tissue. The standard concentrations for standard graph calibration were arranged from standard stock solutions of 1000 μg/ml for each analyzed element [].\nThe tissue samples were investigated by using AI mode of SAM. Figure shows the AI map of the tissue obtained away from the gunshot. The map was constructed by collecting the reflections of acoustic signals, generated by the transducer within SAM, from surfaces of the reference (water) and the tissue sample on the polystyrene substrate. At specific locations within the sample, the AI was calculated to be higher than 2 MRayl, indicating accumulation of elements with different elastic properties. Figure shows the AI map of the tissue obtained close to the gunshot. As can be seen in this image, almost everywhere had an AI of greater than 2 MRayl.\nSEM images of the tissue far away from the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. Similarly, SEM images for the tissue close to the gunshot were obtained at magnifications of 5000 × and 500 ×, as shown in Figs. and , respectively. The images were acquired at 5 keV energy for both tissue samples.\nThe SEM images show that the tissue far away from the gunshot keeps its original structure, whereas the tissue close to the gunshot seems to be deformed and torn up. These results demonstrate the degree of damage the impact of gunshot causes on soft biological tissues.\nTable represents the EDS measurements in SEM, carried out for determining the elemental distribution differences in the deformed tissues. The measurements show the percentages of the residue elements detected on tissues far away from the gunshot and close to the gunshot. According to the results, among all residue elements, Pb, Cr, Fe, and Mn are found to be higher in weight content in the region close to the gunshot, when compared to distant region. Cd and Cu levels do not differ much; however, Zn level is lower in the tissue close to the gunshot.\nWe determined Pb, Cr, Cd, Cu, Fe, Mn, Zn, and Se levels in both tissue samples. Pb, Cr, Fe, Se, and Mn levels were higher in the tissue close to the gunshot, conversely, Zn level was lower in this sample (Table ). Blood Pb and blood Cd, and serum Cr, Cu, Fe, Mn, Se, and Zn levels of our patient were also analyzed and the results are shown in Table , however, we did not observe significant differences when compared to reference values. |
A 37-year-old female presented to our department after noticing progressive enlargement of her jaw, ears, nose, and fingers. Physical examination and comparison to previous photographs confirmed acromegaly. Further neurological examination including formal visual field testing was within normal limits. A full spectrum of pituitary axis investigations was performed, which revealed increased levels of IGF-1 (1109 ng/ml) as well as increased levels of GH (15 ng/ml). All other hormonal laboratory results were within the normal reference range.\nHigh-resolution magnetic resonance imaging (MRI) of the brain with contrast media was performed, which revealed a 17.5 mm × 15.7 mm × 17.4 mm pituitary macroadenoma with stalk displacement. The patient was admitted to our department for elective transsphenoidal surgery. During the preoperative review of MRI films, a subtle area of flow void was noticed within the right anterior aspect of the tumor [].\nThe decision was made to perform a magnetic resonance angiography (MRA) for a more detailed visualization of the vasculature, which revealed a 2.5-mm cavernous ICA aneurysm pointing towards the sella.\nA detailed discussion was then carried out with the patient about the natural history of adenomas and all available treatment options, including endovascular treatment of the aneurysm followed by TSS. We also discussed associated risks and benefits as well as the risks of leaving the adenoma untreated. The patient elected to first pursue medical treatment for pituitary adenoma with close follow-up for the aneurysm.\nShortly after initiation of monthly intramuscular injections of 30 mg of octreotide depot and twice a week administration of 0.25 mg oral cabergoline, the patient noticed a significant decrease in the soft tissue swelling of her face and extremities. IGF-1 levels showed a steady decline from 1109 ng/ml to 353.1 ng/ml over the follow-up period of 1.5 years. The efficacy of medical therapy was also confirmed by a steady decline in tumor size from 17.5 mm × 15.7 mm × 17.4 mm to 10.9 mm × 4.9 mm × 8.9 mm [Figure –]. Simultaneously, there was a gradual increase in the aneurysm size from 2.7 mm to 4 mm [Figure –].\nSix months later a CT angiography was performed at an outside hospital where the patient was seen for a second opinion [].\nGiven the steady increase in the size of the aneurysm and considering the absence of reliable data about the natural history of intratumoral aneurysms, the patient was again offered endovascular treatment and subsequently underwent a successful stent-assisted coil embolization of the aneurysm without complications []. The stent was placed due to the relative low dome-width to neck-width ratio as well as to secure the coil in place in case subsequent TSS was required. One-year follow-up angiogram revealed complete occlusion of the aneurysm along with continuous decrease of IGF-1 levels []. Currently, the patient remains asymptomatic and we shall follow-up with clinical, radiographic, and laboratory surveillance at regular intervals. |
A 20-year-old male was subjected to a home invasion by members of an illegal organisation and suffered multiple targeted GSW to both lower legs. Whilst restrained, he suffered a single GSW to the medial aspect of the right leg (below the knee) and a single GSW to the dorsum of the left foot. An emergency ambulance was requested by his relatives, after the departure of the persecutors, which transported him to a nearby Level 1 Trauma Centre. On arrival, he was noted to have a single entry wound to his right leg laterally at the midpoint of the fibula with supra-medial trajectory but no exit wound, and an expanding pulsatile haematoma medially below knee and acute limb ischaemia. He had a “through-and-through” GSW with an entry wound on the medial aspect of the dorsum of the left foot and an exit wound on the plantar surface. On examination, external haemorrhage had ceased and the vascularity of the left foot was satisfactory. He was initially resuscitated and underwent a plain radiograph of both legs, which demonstrated right fibula and tibia injury and a retained deformed bullet foreign body, in addition to skeletal trauma to the left first meta-tarsal bone (Fig. ). He underwent a vascular assessment and proceeded to have an emergency open exploration of the right leg arteries below knee via a medial approach. When haemostasis was achieved, there was evidence of disruption of the right popliteal artery and tibio-peroneal (TP) trunk. A fragmented bullet was lodged with open comminuted fracture of the right fibula and tibia. He had intraluminal vascular shunts placed from the popliteal artery to TP trunk, and underwent short segment bypass from the popliteal artery to the distal TP trunk using interposition autologous (harvested contralateral long saphenous vein) reversed vein graft repair. He had an on-table angiogram which demonstrated an occluded right anterior tibial artery (without bleeding) and a patent bypass with run-off via the posterior tibial artery to the foot. We elected to manage his left foot conservatively as the bleeding had stopped, and despite occlusion of the left dorsalis pedis artery, his foot was well-collateralised and vascularly intact. He was also assessed by the Orthopaedic Surgeons, and had an external fixator to his right tibia. He made an uncomplicated recovery and regained good functional status. |
A 56-year-old man presented to our department with headache, vomiting, and gait disturbance (for 1 month). He had a past medical history of hepatitis type B virus infection and hepatic failure. He had been medically treated for hypertension and hepatitis for the previous 4 years. On admission, he had an impaired consciousness [Glasgow coma scale (GCS), 14]. Cerebellar ataxia and gait disturbance were evident. Diffusion weighted imaging (DWI) demonstrated multiple cerebellar infarctions at several intensities with perilesional edema of the left cerebellar hemisphere (Fig. ). Brain magnetic resonance imaging (MRI) did not reveal any prominent meningeal gadolinium enhancement or nodule (Fig. ). MR angiography revealed no abnormal findings. The main venous sinuses were confirmed to be patent by 3-dimensional reconstructions of MRI with gadolinium (Fig. ). Chest X-ray did not reveal any abnormal lesions, and the results of serum examination for infectious diseases, including human immunodeficiency virus (HIV), were negative except for hepatitis B virus surface antigen.\nSubsequently, the patient was diagnosed with subacute cerebellar infarction due to arteriosclerosis and was administered glycerol to control the intracranial pressure; however, 1 week after admission, his GCS decreased to 11. Computed tomography confirmed worsened cerebellar edema and hydrocephalus. External and internal decompression surgery were performed to control the intracranial pressure (Fig. ). A section of the swollen cerebellar hemisphere was removed and submitted as a surgical specimen. Additionally, external continuous ventricular drainage was performed to control hydrocephalus. Lumbar puncture to collect cerebral spinal fluid (CSF) was not performed until this time because of the risk of cerebral herniation. CSF from continuous ventricular drainage demonstrated mild inflammation (cell count, 36 /mm3; protein, 16 mg/dl; glucose, 113 mg/dl). C. neoformans was detected in CSF as well as in the surgical specimen of the cerebellum.\nHistopathologic examination of the surgical specimen revealed strong hyperplasia of the arachnoid mater (Fig. ). Fungi were mainly localized in the subarachnoid space and rarely in the parenchyma (Fig. ). Lymphocytes and multinucleated giant cells forming granulomata invaded the arachnoid and subarachnoid spaces and pia with heavy fibrosis (Fig. ). Small arteries were occasionally observed to be occluded with internal endothelial proliferation. While there were arteries in the sample, veins were rarely observed in the subarachnoid space (Fig. ). In addition, venules in the parenchyma were frequently observed to be congested. The patient was diagnosed with granulomatous meningitis due to C. neoformans and was immediately treated using liposomal amphotericin B and fluconazole; however, the ischemic lesion of the cerebellum continued to bilaterally worsen along with worsening perilesional edema (Fig. ). The patient’s course subsequently deteriorated. He developed renal failure and ultimately died 25 days after admission.\nAutopsy confirmed that the pathological changes were confined to the central nervous system and predominantly localized at the surface of the cerebellar hemisphere. Fungal bodies were widely spread along the surface and bilaterally into the deep sulcus of the cerebellum (Fig. ). Few fungi were observed in the supratentorial and intraparenchymal lesions. The lesion in the arachnoid mater of the cerebellum was roughly the same as that in the surgical specimen although it was more deeply spread into the peripheral sulcus and the granulomatous inflammation was not as severe (Fig. ). |
A 39 year old Kosovan Albanian woman was referred to the Division of Abdominal Surgery at the University Clinical Center of Kosovo for an 18-month history of chronic (intermittently colicky) pain in the right upper quadrant, often accompanied by nausea. There was no history of jaundice. She had been treated by her primary care physician with antispasmodics, H2 receptor blockers and antibiotics, without resolution. On admission, physical examination showed no abnormal abdominal findings except mild tenderness in the right upper quadrant. Routine blood tests such as CBC, renal and liver panel proved unremarkable. Chest x-ray showed no signs of cardio-respiratory disease. Plain radiograph of the abdomen showed a calcified opacity at the level L2-L3 vertebrae on the right. The diagnosis, supported by ultrasound and computed tomography, was a calcified hydatid cyst of the liver with involvement and deformation of the gallbladder; the architecture and the size of biliary ducts were normal. The patient underwent right subcostal laparotomy. Intra-operatively, a calcified primary hydatid cyst of the fundus and body of the gallbladder was found with its pericyst attached to the liver (Figure ). The inflammatory response of the liver tissue against the cyst was extensive and formed the structural part of the posterior wall of the pericyst. Complete pericystectomy along with cholecystectomy was performed. No other cysts were found during careful exploration of the peritoneal cavity. On opening the gallbladder, a calcified hydatid cyst (dimensions 7 cm × 5 cm) was found, located in the body and fundus of the gallbladder (Figure ). Macroscopically, the hydatid cyst was part of the gallbladder. The cyst had reduced severely the lumen of the gallbladder and had grown entirely submucosally (Figure ). The histopathology confirmed the presence of calcified hydatid cyst of the gallbladder (Figure ). The patient's postoperative course was uneventful and she was discharged in good condition on the seventh postoperative day. She received two 21-day courses of oral Albendazol 400 mg/day with 14 days pause in between. At five-year follow up, she has had no recurrence of hydatid disease. |
A 27-year old, obese Somali gentleman was admitted with a history of acute onset of hallucinations and delirious behaviour. He was found by his relatives with suspected olanzapine overdose and had been on the floor for more than two days. He had a past history of depression and psychosis for which he was on this medication. The patient also had a longstanding history of "Khat" abuse (a flowering plant with amphetamine-like effects). On admission, he was noted to be disorientated and confused. He was afebrile, tachycardic and had a blood pressure of 110/70. His oxygen saturations were normal. Cardiovascular, respiratory, abdominal and neurological examinations were unremarkable. There were no signs of meningism.\nBlood tests revealed no significant abnormalities apart from elevated Creatine Kinase of 4,800 u/l, which was presumed to be due to rhabdomyolysis secondary to lying on the floor for a prolonged period. Arterial blood gases and chest X-RAY were normal, whilst his ECG showed sinus tachycardia. A CT scan of his brain did not reveal any abnormalities. Urine dipstick showed no evidence of a urinary tract infection. Serial ECGs showed the development of widespread flattened T waves and right axis deviation. A CT pulmonary angiogram was performed 48 hours after admission and revealed extensive pulmonary thrombo-embolic disease and right lower lobe consolidation.\n• Cardiac event\n• Drug-induced arrhythmia\n• Pulmonary embolus\n• Lower respiratory tract infection\nHe was started on intravenous antibiotics for possible aspiration pneumonia and anticoagulation for pulmonary embolism.\nHe was subsequently reviewed by the psychiatrists and admitted to storing 90 olanzapine tablets and taking these with a clear suicidal intent following a severe depressive episode. He denied having taken any other medications and no other empty packaging was found, apart from that of his olanzapine tablets. Following medical treatment for the pulmonary embolus and lower respiratory tract infection, he was discharged to the care of the mental health team. He has since recovered completely. |
A 54-year-old previously healthy Caucasian female with otherwise unremarkable past medical history presented to emergency department with one-day history of hematochezia and abdominal pain. The patient described crampy left lower quadrant pain with no aggravating or relieving factors. She had a total of five bowel movements since symptom onset with the first bowel movement containing stool mixed with bright red blood followed by predominantly bloody stools. She took no medications on a regular basis and denied having a screening colonoscopy for colorectal cancer at age 50. She reported symptoms of upper respiratory tract infection (cold, sneeze, and cough) for which she took three doses of 120 mg pseudoephedrine purchased from a local grocery store for 1 day prior to symptom onset. Her maternal grandfather had prostate cancer but there was no significant gastrointestinal tumor history in the family. She was a nonsmoker and reported drinking socially (roughly one standard drink) once a week.\nHer admission vitals were within normal limits. Physical examination was consistent with mild tenderness on the left side of abdomen and hypoactive bowel sounds. Rectal examination showed bright red blood without any stool in the rectal canal. Her laboratory values were significant for mild anemia with hemoglobin of 11.5 mg/dl, hematocrit of 34.5%, erythrocyte sedimentation rate 31 mm/hr, and C-reactive protein 2.15 mg/dl. A computed tomography scan revealed mild to moderate mural thickening of the descending/sigmoid colon consistent with colitis without pericolonic abscess, ascites, or free air (). An infectious workup was obtained including blood cultures, stool cultures, gastrointestinal panel for Clostridium difficile, and gastrointestinal viruses but was negative. She was resuscitated with intravenous fluids.\nThe patient underwent colonoscopy which demonstrated segmental moderate inflammation in the sigmoid colon, descending colon and splenic flexure along with internal and external hemorrhoids. There was evidence of submucosal hemorrhages with mild edema in the aforementioned segments of the colon (). Endoscopic findings were highly suspicious of ischemic colitis. Several biopsies were obtained from the inflamed areas which exhibited focal lamina propria eosinophilic change with mild crypt attenuation and loss of goblet cells consistent with mild ischemic changes. There was no evidence of chronic inflammation.\nShe was observed in the hospital for 3 days and her diet was progressed slowly. Her bloody bowel movements ceased after 1 day in the hospital and patient was counseled and educated regarding avoidance of pseudoephedrine and over the counter medications for symptomatic management. |
A 54-year-old male patient presented with a two-day history of severe left-sided, lower back pain which disappeared with the appearance of left-sided lower limb pain felt deep inside the whole lower limb and described by the patient as intense internal pressure (VAS 8/10). On examination, power was 5/5 with no sensory deficit and normal reflexes. The patient received NSAID painkillers and active bed rest was advised. However, on the patient's insistence, a lumbar spine X-ray and MRI were done which showed mild IVD protrusion. The patient was reassured and sent home. A week later the patient presented to the ER with an 18-hour history of heaviness and difficulty in raising the left foot when walking, with numbness along the lateral part of the leg and dorsum of the foot. The pain was moderate (VAS 5/10). No sphincter-related symptoms were observed. According to the patient, the decreased pain caused the delay in presentation, against the instructions on first evaluation. On examination of the foot dorsiflexion was 2/5 (movement on gravity alleviation) with decreased sensation along the left L5 dermatome. There were normal reflexes and sphincters. The patient underwent immediate, new lumbar MRI which showed a large disc sequester with disc migration. His status was fully explained; he was admitted and underwent microdiscectomy L4/5.\nThe initial back pain lasted for 2 days, mostly caused by stretching and pressure on the weakened annulus fibrosis. This type of pain disappears or decreases once the annulus opens and nucleus pulposus leaks to the spinal or root canal. Initial left lower limb pain is a typical description of painful radiculopathy where mass pressure and inflammatory irritation of the nerve root and dorsal root ganglion cause neuropathic pain. This is different from nociceptive and referred pain types. Dorsal root ganglion hosts the cell bodies of sensory nerves with bidirectional connections (to the periphery and spinal cord). Injury to the nerve root motor fibers causes weakness.\nThe majority of patients with lumbar disc and radicular pain improve with conservative treatment. Surgical indications are acute or progressive motor weakness, sphincter dysfunction, intractable pain not responding to analgesia, and pain affecting patient daily life, not responding to 6 weeks of conservative treatment in the absence of acute surgical indications. In all cases, there should be a correlation between the clinical picture and MRI findings. In the current case, the patient has left L5 nerve root symptoms and signs (dorsiflexion weakness, normal reflexes, and dermatologic radiculopathy). The expected IVD prolapse occurred on either the left posterior-lateral L4/5 or left extreme lateral L5/S1 disc. Although there is a debate about dermatologic sensory distribution in root-originating symptoms and variations in clinical presentation (i.e., the L4/5 disc causing S1 root symptoms via compressing the root in higher position, groin pain with lower lumbar discs due to paravertebral sympathetic ganglion pathway entering at L1 or L2 nerve), we presented the commonly encountered scenarios. |
A 40-year-old female, G7P4+2, was admitted for elective cesarean section at 38 weeks. Her medical history included gestational diabetes mellitus (GDM) during her current pregnancy that was controlled on metformin (500 mg, three times daily), four previous cesarean sections, two early pregnancy losses at six-week gestation, hypothyroidism, and previous eye surgery at childhood for eye squint. Her family history was positive for diabetes and hypertension.\nThe patient had an elective cesarean section under spinal anesthesia and gave birth to a living female. It was noticed that she has been omental to the anterior abdominal wall adhesions and omental to the anterior uterine wall adhesions. There were no intraoperative complications and estimated blood loss was about 500 cc.\nOn the first postoperative day [POD1], the patient looked well with stable vital signs. System review was within normal, and physical examination showed soft and lax abdomen with audible bowel sounds. The patient was started on the liquid diet. The patient passed flatus and was started on the soft diet. The same day at night, she developed mild abdominal distension, bowel sounds still audible with stable vital signs, and the patient was advised to mobilize. The patient mentioned she used to have more abdominal distension after each caesarian delivery.\nOn POD2, the patient started to have more abdominal distension despite passing stool, and bowel sounds become sluggish then nonaudible. Patient was kept NPO; serum electrolytes were requested and showed mild hypokalemia 3.29 mmol/L. Patient was encouraged to mobilize and was started on potassium chloride infusion and NGT was inserted. She initially was diagnosed to have paralytic ileus, but her general condition eventually deteriorated dramatically, and she developed tachycardia and shortness of breath.\nThe patient was transferred to the Intensive Care Unit (ICU), reviewed by ICU and surgical team. Abdominal X-ray was performed and showed distended abdomen with pneumoperitoneum (see Figures and ). CT scan was requested and showed small amount of free fluid collection in the subphrenic area with subhepatic longitudinal mass and large pneumoperitoneum suggesting possible bowel perforation and dilated proximal small bowel loops without obvious transitional zone. The patient was transferred to OR for exploration laparotomy.\nExploration laparotomy performed through longitudinal abdominal incision. There was gangrenous changes of the caecum and right colon with its anterior wall showing multiple ischemic areas and necrosis; some of them are perforated with gross picture of ischemic changes, others thinned out and were about to perforate in subhepatic area; right hemicolectomy and iliostomy were performed till the area of normal color of the colon was reached (see Figures and ). Peritoneal lavage was performed afterwards, 2 abdominal drains were inserted, and the incision site was closed with staples. The patient was properly hydrated all through the surgery, fluid input/output were properly calculated, and urine output was adequate and clear. The uterus and both adnexa were normal.\nThe patient was transferred back to ICU. The patient received broad-spectrum antimicrobial agents; she was under close monitoring, multidisciplinary team management and discharged to regular room 6 days postoperatively. The postoperative course passed otherwise uneventful. The multidisciplinary team shared in plan of care were surgeons, pulmonologists, ICU intensivists, obstetricians, and cardiologist. Thrombophilia screening was suggested and hyperhomocysteinemia was found; homocysteine level was 14.26 Umol/L. She was discharged in a good general condition 12 days postoperatively. |
Our patient is a 54-year-old Caucasian male with a history of cardiac and pulmonary sarcoidosis, hypertension, premature ventricular contractions (PVCs), and obesity who presented with acute onset right hemianopsia, memory recall difficulty, and alexia without agraphia. He was in his normal state of health and doing yard work when the symptoms began.\nIn the past year, incidental PVCs were found on 12-lead electrocardiogram (ECG) during a preoperative evaluation for dental work. Holter monitoring revealed a 12% PVC burden over 24 hours, indicating an indeterminate degree of ventricular dysfunction. Cardiac evaluation of the PVCs included transthoracic echocardiogram which revealed hypokinesis of the left inferior ventricular wall with an ejection fraction of 35%. Cardiac catheterization for investigation of structural blockages of coronary vessels yielded no significant CAD.\nThese findings were suspicious for an infiltrative process. This hypothesis was supported by cardiac MRI showing sarcoid infiltrates on T2-weighted images and by discovery of noncaseating granulomas on pulmonary node biopsy. He was diagnosed with cardiac sarcoidosis three months after initial presentation. During this time, he showed no clinical symptoms of systemic sarcoidosis or heart failure. An implantable cardioverter defibrillator was placed for primary prevention of arrhythmias secondary to cardiac sarcoid. He was doing well for one year until he presented with stroke symptoms.\nOur patient endorsed decreased vision on the right and described the words on his lawn mower being visible but not readable. He also acknowledged trouble with recalling names and specific events.\nUpon examination, our patient demonstrated normal speech and language. He was asked to write a simple sentence and performed the task without difficulty. When asked to read the sentence, he was unable to do so, representing alexia without agraphia. He showed right homonymous hemianopia. The remainder of the physical exam was normal.\nAt admission, initial computed tomography (CT) scan was negative. Subsequent brain magnetic resonance imaging (MRI) and magnetic resonance angiogram (MRA) confirmed a left posterior cerebral artery (PCA) infarction (). MRA of the neck was unremarkable. The stroke was suspected to be cardioembolic in origin due the PVC burden and reduced ejection fraction of 35% promoting possible thrombus formation. Transesophageal echocardiogram revealed no thrombus or patent foramen ovale (PFO) and supported the previous finding of hypokinesis of left inferolateral ventricular wall (). Hypercoagulable workup was nonrevealing. There was no family history of sarcoidosis or early age stroke.\nBy the time of discharge, our patient's visual symptoms returned to baseline and he was given high dose aspirin and atorvastatin for secondary stroke prophylaxis. Cellcept and prednisone were prescribed for management of sarcoidosis. A LINQ device was placed for continuous ECG monitoring. Lisinopril and metoprolol were maintained for pressure and rhythm control. |
A 27-year-old man presented to the emergency department with nausea and vomiting for a few days. The vomiting was non-projectile, non-bilious, and contained ingested food materials. The patient had no concomitant diarrhea and denied any constitutional symptoms.\nThe patient described experiencing similar episodes of vomiting over the last seven years. He reported that the typical episode started upon waking up in the morning with no preceding stressors and was associated with intense abdominal pain, lethargy, and nausea. Then, the abdominal pain would be followed by multiple episodes of vomiting up to 10 times within a few hours. Every episode might last for five to six days and would reoccur every two to three months. The patient was doing well between episodes and was able to resume his job as military personnel.\nHe had experienced many emergency presentations and multiple admissions and had been treated symptomatically throughout without reaching a conclusive diagnosis. His past medical history was unremarkable apart from a laparoscopic appendectomy performed seven years back.\nThe patient had achieved his developmental milestones at normal ages and had grown up in a middle-class family consisting of his parents, brothers, and sisters. He denied a previous diagnosis of any psychiatric disorders such as post-traumatic stress disorder. His siblings were healthy, except for his younger sister, who was known to have an intellectual disability. He was married and had a one-year-old child; he worked as a civil officer in the armed forces and denied alcohol consumption, smoking, or drug abuse. Clinical examination revealed normal vital signs, delayed capillary refill, and dry mucus membrane. The abdomen was soft, with mild epigastric tenderness. Other systemic examinations were unremarkable.\nThe patient was investigated thoroughly and evaluated for possible gastroenterological, immunological, neurological, metabolic, and psychiatric disorders. Biochemical investigations were normal, except for lactic acidosis, ketonuria, and proteinuria attributed to the normal physiological response to vomiting. Also, the urine toxicology screen was negative. An extensive gastrointestinal workup including abdominal X-ray, abdominopelvic CT, gastro-endoscopy, and colonoscopy was unremarkable. Neurologically, he was evaluated with brain MRI, magnetic resonance spectroscopy, and electroencephalography, and all were normal. The patient was also screened for various metabolic disorders, including urea cycle defect, porphyria and Fabry disease, organic aciduria, fatty acid oxidation disorder, and amino acid disorder; all of them were excluded. Other workups for the immunological and autoimmune disorders were unremarkable. He had undergone multiple psychiatric assessments during different admissions, which had been unable to establish any psychogenic cause of the vomiting.\nAfter excluding all the above possibilities, we considered CVS, and the patient's presentation fulfilled the Rome IV criteria for the diagnosis of CVS. The patient was started on amitriptyline 25 mg (0.5 mg/kg) as a prophylactic measure. The patient responded to the treatment well; he has had a five-month symptom-free period and has remained well during outpatient follow-up visits. |
A 64-year-old, 87 kg female presented for clipping of a 4 mm wide neck unruptured saccular anterior communicating artery aneurysm which was discovered incidentally during the evaluation of headaches and memory difficulties. Her past medical history was significant for remote breast carcinoma, hypertension, obstructive sleep apnea, and gastroesophageal reflux disease. She was a lifelong nonsmoker and notable preoperative medications included aspirin, furosemide, and propanolol. Preoperative imaging had no evidence of prior infarcts. After induction of anesthesia, a right internal jugular central venous catheter and radial arterial catheter were placed and maintenance of anesthesia was performed with a combination of 0.5 MAC sevoflurane, propofol 50 mcg/kg/min, and remifentanil 0.125 mcg/kg/min infusions. Mannitol 1 gram per kilogram for a total of 80 grams was administered. The neurosurgeon placed an 80 cm closed-tip, barium impregnated lumbar drainage catheter with a 0.7 mm inner diameter (Medtronic USA, REF 46419) at the L3-4 level prior to the start of surgery.\nThe surgery commenced and was progressing unremarkably. Prior to opening of the dura, 20 mL of CSF was drained over 15 minutes per neurosurgeon request. After dural opening and during dissection of the aneurysm, the surgeon requested the lumbar drain to be opened allowing further drainage of CSF. After approximately 15 minutes, the blood pressure sharply increased over the course of 1 to 2 minutes from a baseline systolic blood pressure of 130 mmHg to over 205 mmHg with an associated decrease in heart rate from 60 bpm to 50 which resolved over the course of minutes. This acute hypertension was treated with a number of interventions including 250 mg propofol and 1 mcg/kg remifentanil boluses aimed at treating light anesthesia as well as a bolus of 7.5 mg labetalol. At the time of the hypertensive event, the propofol, remifentanil, and Sevoflurane dosing had been stable and unchanged for over an hour. There was also no change in the level of surgical stimulation at this time as the dura had been incised and the neurosurgeons were using the operative microscope to expose the aneurysm. After the aneurysm was successfully clipped, the lumbar drain was closed and the surgery was completed without further episodes of hypertension. A total of 60 mL of CSF was drained via the lumbar drain during the case in addition to the losses from the surgical field.\nAt conclusion of the operative procedure, the patient remained comatose and unresponsive despite an hour in the operating room awaiting emergence from anesthesia. A postoperative head CT was obtained prior to transport to the intensive care unit which demonstrated mild cerebral edema and borderline inferior transtentorial herniation, but no significant hemorrhage or focal abnormalities. She was then transported to the Neurocritical Care Unit intubated and ventilated. Initial arterial blood gas analysis on arrival to the ICU did not reveal a cause to her delayed emergence (pH 7.35, pCO2 47 mmHg, pO2 323 mmHg, glucose 204 mg/dL, and sodium 137 mEq/L). She did not receive any benzodiazepines during the case, but did receive 50 mcg of fentanyl on induction and 1 gram of levetiracetam in addition to the propofol, remifentanil, and Sevoflurane maintenance. Initial neurological exam demonstrated midline and equal 4 mm pupils which were reactive to light bilaterally. She withdrew to painful stimuli in all four extremities. On postoperative day (POD) 1, her mental status continued to be depressed with a Glasgow Coma Scale of 7T (E2:V1T:M4). That day, an MRI was obtained which revealed bilateral thalamic infarctions on the diffusion weighted imaging which can be seen in . Supportive care was continued and over the course of several days, her mental status slowly improved to GCS of 10 (E4:V1:M5) on POD 5. This allowed for safe extubation that day. She continued to improve and was alert and oriented to person, place, time, and situation with some memory and attention difficulties by POD 16. A timeline of her in-hospital recovery can be found in . She was discharged home with referrals for home physical, occupational, and speech therapy on POD 19. She continued her gradual neurological improvement and by over a year postoperatively, Neurology records indicated she was living independently and her Montreal Cognitive Assessment score had recovered to a normal 27/30. |
A 24-year-old Caucasian man presented with a several month history of muscle pain, fatigue and insidious onset of pitting edema to his lower extremities. His symptoms progressed to include bilateral arm swelling, muscle pain to the thenar eminence of both hands, and paresthesias to his hands. He did not have a rash, joint pain, Raynaud phenomenon, oral ulcers, fever, hardening of the skin or weight loss, and he denied any cardiac, respiratory, genitourinary, or gastrointestinal symptoms. Prior to symptom onset, he had traveled to South America and reported possible ingestion of undercooked meat as well as swimming in a river and lake. He had frequent visits to the Northeastern United States and had recently spent an extended period of time outdoors in Rhode Island. Medical, surgical, and family histories were unremarkable, and he was not taking any medications. He had a history of mild alcohol intake, no history of smoking, and some marijuana use. On examination, his vital signs revealed a blood pressure of 104/57 mm Hg, pulse of 56 bpm, and normal temperature. He had no significant findings on head, neck, cardiovascular, respiratory, or abdominal exam. He had no cervical, axillary, or inguinal lymphadenopathy. He had significant pitting edema on his feet and legs extending up to his knees as well as non-pitting edema on the dorsum of both hands. Although there was edema, the skin was soft without any significant hardening and was without any overlying erythema. There were no signs of skin dimpling or grooves. His neurological examination revealed normal strength.\nHis initial laboratory work was significant for a mild eosinophilia of 700 with a normal white blood cell count, hemoglobin, and platelets. He had normal calcium, creatinine, and electrolyte levels. His alanine aminotransferase was slightly elevated and total protein slightly low, but he had normal albumin and bilirubin. His thyroid-stimulating hormone was slightly increased, but his free T4 and total T3 were normal. His urinalysis was normal. He had a normal level of creatine phosphokinase, sedimentation rate, and C-reactive protein. Further workup revealed a positive anti-nuclear antibody with a titer of 1:160 with a speckled pattern. His extractable nuclear antigen panel was negative, including Scl-70. His ANCA, myeloperoxidase and proteinase-3 serum studies were also negative. His ACE level and complement levels were normal. Infectious workup revealed negative stool studies for culture, ova, and parasites. Antibodies for HIV, CMV, and Trichinella were negative. Serum for histoplasmosis, cryptosporidium, coccidiomycosis, and interferon gamma release assay were negative. A blood smear for parasites was negative. An ELISA IgG/IgM test for Lyme disease was positive with subsequent testing with Western blot strongly positive for IgG (eight out of ten bands positive) and also positive for IgM (two out of three bands positive).\nThe patient had extensive imaging done with a normal CT scan of his neck, chest, abdomen, and pelvis. An echocardiogram was also normal. Due to a previous negative workup along with persistent pain and swelling, an MRI of his right lower extremity was performed (see Figure ). Imaging demonstrated extensive circumferential edema with enhancement of the superficial soft tissues, superficial fascia, and, to a lesser extent, deep fascia of the lower leg. Taking into account the mild peripheral eosinophilia and the imaging findings, a diagnosis of eosinophilic fasciitis was considered. To complete the workup, a biopsy of the fascia, muscle, and adipose tissue of the left calf was taken (see Figure ). The biopsy did not include the dermis. Surprisingly, there was no evidence of eosinophilic fasciitis. Instead, the specimen illustrated a striking granulomatous fasciitis and vasculitis. The fascia showed exuberant granulomatous inflammation (Figure A) with an inflammatory infiltrate that was made up predominately of histiocytes and CD3-positive T cells with very rare eosinophils (Figure B). The granulomatous inflammation centered primarily on small- to medium-sized blood vessels and was non-necrotizing. While the vessels did not display overt fibrinoid necrosis, they did appear damaged with loss of endothelial cells confirmed with CD31 immunostaining. The inflammatory infiltrate was seen extending into adipose tissue and particularly around blood vessels within the fat. The adjacent skeletal muscle also showed perivascular inflammation and vasculitis in both the perimysial and endomysial compartments. There was no endomysial fibrosis, fatty infiltration, or inflammation surrounding muscle fibers. AFB (acid-fast bacteria) and Wade-Fite stain were negative for mycobacterial organisms. GMS (Grocott-Gomori's methenamine silver) stain was negative for fungal organisms. Due to the positive Western blot for Lyme, a Warthin-Starry silver nitrate stain was performed to evaluate for spirochetes; however, no definitive organisms were seen. A Borrelia PCR analysis of the tissue was performed as well, but no DNA was detected.\nThe patient was given a diagnosis of granulomatous fasciitis along with a diagnosis of Lyme disease. He was first treated with doxycycline for 42 days straight due to initial Lyme serology being positive. The calf biopsy revealing fasciitis was not performed until the patient was 3 weeks into the doxycycline course. Once fasciitis was diagnosed, he was started on a prednisone taper starting at 1 mg/kg/day for a week with taper by 10 mg every 2 weeks. After 2 months of treatment with prednisone, the patient had near resolution of symptoms. A repeat MRI was performed 82 days after the initial MRI with the previously seen changes consistent with fasciitis nearly completely resolved with only a thin sliver of edema over the superficial fascia. On re-evaluation of the patient, he reported skin changes to his left upper arm at 20 mg of prednisone per day and skin changes to his left forearm at 7.5 mg per day. Examination of the upper arm revealed an atrophic patch with some overlying erythema and examination of the forearm showed indurated, bound-down, tense skin with a slight groove and minimal overlying hyperpigmentation. The rest of the dermatological examination was within normal limits. Skin biopsy revealed marked septal thickening with sclerosis, sparse lymphoplasmacytic infiltrate along the dermal subcutaneous junction, and swollen, homogenized collagen fibers with diminished spaces between the fibers (see Figure ). The clinical examination along with pathology revealing dermal and subcutaneous sclerosis was consistent with a diagnosis morphea profunda. Lyme serology was repeated, but Western blot for IgG was negative. A scleroderma antibody panel was negative as well. He was started on methotrexate and a higher dose of prednisone. |
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. |
The patient is a 37-year-old Hispanic female who initially presented for a firm mass on her right breast that had enlarged and became painful over one year. Her medical history is significant for Sjogren’s syndrome with no systemic treatment, hypothyroidism, parity, a right breast benign adenoma, and bilateral breast augmentation with subsequent removal of the implants due to infection. A fine-needle aspiration (FNA) biopsy showed GM (Figure ). Right axillary lymph node contained lymphoid tissue with sinus histiocytosis. There was no evidence of cancer, fungus, or tuberculosis. A diagnosis of idiopathic GM was made. However, no treatment was given as she was lost to follow-up.\nThree months after the FNA, her incision site did not fully heal while the mass continued to grow. There was worsening erythema, induration, and pain associated with the mass. Ultrasound (US) of the mass showed complex partially fluid-filled parenchymal changes directly under the indurated area with extension into the biopsied GM lesion suspicious for fistularization with possible abscess or phlegmon formation (Figure ). US findings did not indicate that the mass was drainable. Although her labs did not indicate an active infection, the patient was placed on oral linezolid 400 mg twice daily for 14 days and asked to follow-up in the outpatient surgical clinic.\nPrior to the completion of her antibiotic course, she presented with thick drainage from the site of the mass. This presentation was consistent with a breast abscess, for which the patient underwent an incision and drainage (I&D) with packing. During the post-op evaluation, the site of I&D showed granulation and sanguineous drainage without any signs of infection which was supported by her labs and vital signs. Post-I&D US showed resolution of parenchymal fluid and confirmation of a persistent path between the I&D site and the biopsied GM lesion (Figure ). Differential diagnoses from US included progression of GM with fistularization and a superimposed infection with more evidence for the former diagnosis. One notable drawback during the investigation for her case is the lack of a bacterial culture; hence, we could not definitively rule out infection as a complication associated with her GM. A week after I&D, the patient reported some improvement in the I&D site. |
A 48-year-old woman, a musician and piano teacher for more than 20 years, complained of a relapsing and remitting attack of left thumb weakness that had occurred 3 times over the previous 2 years. In each attack, she was completely unable to extend her left thumb and the weakness lasted for 3-7 days. During attacks, she could still move her thumb towards her palm or grasp objects properly. There was no associated pain, sensory change or joint pain of her left hand. A milder right thumb weakness, which spontaneously recovered within 24 h, occurred during the second and third attacks. She denied having a high-calorie or carbohydrate diet, emotional change, or infection before the attack. Stage II hypertension (classified according to Joint National Commission 6 criteria) had been diagnosed 3 years previously and was well controlled by amlodipine besylate. She denied having had recent craniofacial injury, nutrition or herbal supplement, illicit drug usage, or consumption of alcohol. There were no familial diseases, thyroid disorder or neuromuscular disorder known in 3 generations of her family.\nShe presented 4 h after the onset of her fourth attack. On presentation, she was oriented and cooperative. Her vital signs were normal. She could not extend or elevate her left thumb (fig. ). However, she could normally and powerfully move her left thumb towards her palm, flex her left fingers, extend or flex the wrist, elbow and shoulder. No significant weakness of the right thumb was detected. Pinprick pain, fine touch, and cold/warm sensation were normal. Tendon reflexes were symmetric and normoactive. There were no other abnormal neurologic findings. A drop thumb was diagnosed. Nerve conduction studies did not show conduction velocity slowing in the median, ulnar or radial nerves. Needle electromyogram did not show abnormal spontaneous activity in the extensor pollicis brevis and extensor pollicis longus muscles. Laboratory tests, including biochemistry, muscle enzymes, hematology, serology, autoimmune indices, and serum concentration and daily urine excretion of magnesium and calcium, were within reference range, except hypokalemia [1.9 mEq/l (reference range: 3.4-5.0)] and mild metabolic alkalosis. Thumb weakness was dramatically reversed by a replacement of potassium to 2.6 mEq/l.\nBased on profound hypokalemia, episodic focal muscle paresis, and a rapid reversal of muscle paresis after potassium replacement, hypoPP was considered. Thyroid function and pituitary-adrenal gland axis were normal. The plasma renin activity was 0.3 ng/ml/h (reference range: 0.5-3.3) and serum aldosterone level was 84 ng/dl (reference range: <31) in the supine position. The aldosterone/renin ratio was 28. Conn's syndrome was diagnosed. Abdominal computerized tomography demonstrated a left adrenal mass (fig. ). The patient underwent surgical removal of her adrenal mass. Pathologically, the mass was shown to be an adrenal adenoma. After surgery, the patient did not experience any further episodes of thumb weakness, and her serum potassium and aldosterone levels and plasma renin activity were normalized. She discontinued antihypertensive drugs 6 months later as her blood pressure remained normal. During a 12-year follow-up, she remained well without recurrence of weakness, hypertension or hyperaldosteronism. |
A 10-years-old boy, resident of city Panjgur, the state of Balochistan, presented in an outpatient clinic with complaints of pain in the left thigh for 15 days along with fever and difficulty in walking for seven days. His past history was unremarkable, except that the child had a history of recurrent episodes of lower limb pain since the age of four years, for which no specific workup or treatment was done. Transfusion history was also negative. He was born to non-consanguineous parents whose family history was negative for haemoglobinopathies or blood transfusions. On physical examination, child was conscious and oriented with no dysmorphic features; he was febrile at the time of examination, with a temperature of 38.3°C, and he was mildly anaemic. Anthropometric measurements were lying at the 5th percentile on the growth chart developed by National Centers for Health Statistics - CDC for age and gender. There was diffuse swelling over the anteromedial surface of the middle third part of the left thigh. Skin overlying swelling was warm with no overlying colour changes. The area was tender on palpation and passive and active movements. The examination of both hip and knee joints was unremarkable. On abdominal examination, the liver was palpable 2 cm below the right costal margin with a total span of 12 cm and the spleen was not palpable. His rest of the systemic examination, including the cardiovascular, respiratory, central nervous system, and musculoskeletal, was also unremarkable.\nPlain radiograph of left leg indicates mild periosteal reaction and periosteal elevation with sub-periosteal collection and displacement and blurring of fat planes, suggestive of infective/inflammatory process (). Magnetic resonance imaging (MRI) with contrast shows marrow signal abnormality in the mid-shaft diaphysis of the left femur with post-contrast peripheral soft tissue signal abnormality and abscess formation in soft tissues, consistent with osteomyelitis ().\nHis complete blood count shows haemoglobin 6.8 g/dL, haematocrit 21.7%, total leucocyte count 13.5 cells per microliter, comprising of 70% neutrophils and 22% lymphocytes, platelet count of 111,000 per microliter of blood, red cell indices: mean corpuscular volume (MCV) 80.5 fL, mean corpuscular haemoglobin concentration (MCHC) 37.5 g/dL, mean corpuscular haemoglobin (MCH) 30.2 pg, reticulocyte count 7.5% (corrected reticulocyte count 5.0%). Peripheral smear showed anisocytosis, poikilocytosis, target cells, and rouleaux formation. Erythrocyte sedimentation rate of 80 mm/hour (<5 normal) and blood culture revealing no bacterial growth. Haemoglobin electrophoresis result was positive for compound heterozygote for sickle cell SD disease ().\nDiagnosis of osteomyelitis of left femur secondary to compound heterozygous SDC (Hb SD) was made, and the patient was given a course of intravenous (IV) antibiotics and hydration. The patient was started on hydroxyurea once the osteomyelitis got resolved, and follow up was planned in the ambulatory clinic of paediatric haematologist after two weeks. The patient’s family was counselled for the screening of the whole family for haemoglobinopathy. The patient’s follow-up remained uneventful. |
A 53-year-old man presented to the Surgical Outpatient Department with a 2-month history of rectal bleeding, black tarry stools and associated episodes of fainting. His past medical history was unremarkable. There was no family history of gastrointestinal disease. Physical examination revealed pallor. Haematological investigations showed normocytic anaemia and thrombocytopenia: other haematological and biochemical parameters were normal. A computed tomographic (CT) scan of the abdomen and pelvis showed a diffusely thickened stomach wall suggestive of linitis plastica with perigastric lymphadenopathy. Multiple osteolytic lesions were noted in the dorso-lumbar vertebrae. Endoscopy showed a big fundal mass infiltrating the greater curvature up to one third of the stomach.\nHistopathological examination of the gastric biopsy was reported as infiltrating adenocarcinoma of the signet ring cell type and was negative for H. pylori infection. Bone scintigraphy and bone marrow examination showed no evidence of metastasis. The patient was given a total of 4 sessions of chemotherapy (5FU + leucovorin LF1) with 3-week intervals between each session. Anti-H. pylori treatment was not given as the initial gastric biopsy was negative for the same. A follow-up contrast-enhanced CT scan of the abdomen and the pelvis showed an ulcerated irregular heterogeneous soft tissue density mass (9 × 7 × 6 cm) in the body of the stomach, causing circumferential irregular wall thickening extending towards the gastric fundus with perigastric lymphadenopathy. An endoscopy showed the upper margin of the previously diagnosed gastric tumour reaching the gastro-oesophageal junction. A PET-CT scan revealed increased uptake of the tracer FDG within the stomach lesion.\nIn view of the disease progression and unresponsiveness to chemotherapy, a total gastrectomy was performed. The gastrectomy measured 35.5 cm along the greater curvature and 11.5 cm along the lesser curvature and showed a diffusely thickened wall with ulcerated mucosa involving fundus to antrum (fig. ). The resection margins appeared free from the lesion. Lymph nodes were dissected along the lesser and greater curvatures. The related histopathological findings showed intermingled areas of poorly differentiated signet ring cell carcinoma and diffuse monotonous sheets and nodules of small lymphoid cells with centrocyte morphology (fig. ). There was transmural involvement of the gastric wall by both components. At immunohistochemical assay, the lymphoid cells were diffusely positive for CD20 (fig. ) and BCL2 and focally positive for CD43. They were negative for CD10, cyclin D1, CD5, CD23 and Bcl-6. The Ki-67 proliferation index was 5%. Of the 60 perigastric lymph nodes, 54 (90%) showed metastatic carcinoma. The adjacent gastric mucosa showed severe chronic active H. pylori (Warthin-Starry stain and H. pylori immunostain positive) associated gastritis with intestinal metaplasia. The lesser omental margin was positive for signet ring cell carcinoma. The carcinomatous areas showed no definite response (grade 3) to chemotherapy. A final diagnosis of a collision tumour, signet ring cell carcinoma and MALT lymphoma (ypT4aN3bMx) was made. The pre-operative biopsy was reviewed and it showed foci of signet ring cell carcinoma admixed with a monotonous lymphoid population whose features were consistent with MALT lymphoma. The patient survived the immediate post-operative period and had a follow-up endoscopy 2 months later, which showed an inflammatory polyp at the anastomotic site confirmed at biopsy. Thereafter, the patient was lost to follow-up. |
The patient is a 28-year-old male with prior history of a grade 3 astrocytoma, who presented to our emergency department (ED) after having a general tonic-clonic seizure (GTC). Initially, his disease was appreciated four years prior when he presented with a GTC and was found to have a grade 3 astrocytoma of the left occipital lobe (Figure ). He underwent maximal safe resection followed by adjuvant EBRT to 45 Gy in 25 fractions at an outside institution. Due to reasons outside the patient’s control, he received only two cycles of adjuvant temozolomide following EBRT. He developed disease recurrence, presenting as a GTC six months prior to this ED presentation, where imaging demonstrated tumor progression with increased extension into the parietal lobe (Figure ). He subsequently underwent a second maximal safe resection later that month. Final pathology returned as GB. Follow-up imaging four months afterward demonstrated disease progression and he was started on bevacizumab as well as a tumor treatment field (TTF) device. He unfortunately only tolerated TTFs for one month, ending one month prior to the ED presentation.\nUpon admission to our hospital from the ED, MRI demonstrated progression of disease in the left occipital-parietal lobes with extension into the splenium and anterior-inferior extension into the left thalamus and basal ganglia (Figure ). His physical exam was notable for mild right-hand weakness, but he was otherwise neurologically intact. Despite changes to his antiepileptic medication, he had seizure recurrence a few weeks following admission. Presuming that the area of tumor recurrence received a definitive dose in the past, the consensus decision was to proceed with repeat maximal safe resection with GammaTile placement. A dose of 60 Gy was prescribed to a 5 mm depth using a total of eight tiles, each containing four Cesium-131 3.5U seeds, to line the post-operative cavity volume of 17.6 cc. A significant portion of the occipital-parietal disease was debulked, with final pathology again demonstrating a grade 4 astrocytoma with molecular studies indicating an IDH-mutated, ATRX mutated, and MGMT promoter methylated phenotype with hypermutation. Postoperatively he was noted to have right upper and lower extremity weakness/spasticity with right foot drop and mild right face weakness. He required a cane to assist with ambulation and reported word-finding difficulties and decreased short-term memory. He was planning to start adjuvant temozolomide; however, he developed a severe GTC with increased muscle weakness and altered mental status two months later. MRI following this episode demonstrated mildly increased enhancement to the tissue surrounding the surgical bed with the progression of disease in the splenium and left thalamus/basal ganglia (Figure ).\nGiven this symptomatic disease progression, a treatment plan was made to take the progressive regions of disease outside the irradiated GammaTile volume to 35 Gy in 10 fractions. As shown in Figure , an initial planning target volume (PTV) was delineated consisting of the T1 post-contrast-enhancing disease with a 5 mm margin. The volume that received greater than 35 Gy from the GammaTile treatment was excluded from the PTV. The PTV was then separated into two portions by subdividing the remaining volume between tissue that received less than 17.5 Gy (PTV1), and that which received 17.5-35 Gy (PTV2). Using a volumetric modulated arc therapy plan with five arcs, one non-co-planar, a homogeneous dose of 35 Gy in 10 fractions was delivered to PTV1. This dose was calculated to have a biologically equivalent dose in 2 Gy fractions (EQD2) of 45 Gy. Dose painting gradually decreased the dose from 35 Gy to as low a dose as achievable approaching the resection bed border of PTV2 (Figure ). A composite of the GammaTile dose volume (Figure ) with a dose delivered from PTV1 and PTV2 resulted in a homogeneous dose of approximately 50 Gy EQD2 to the residual disease extending into the splenium and the left thalamus/basal ganglia (Figure ). Digital imaging and communications in medicine (DICOM)-RT data from the initial 45 Gy delivered from the outside institution were obtained, and cumulative dose to critical organs at risk (OARs), including the brainstem, ocular structures, and cochlea, were within established constraints.\nDue to the hypermutation phenotype of his tumor, the patient was started on CCNU (100 mg/m2) and completed his EBRT with no issue or progression of his current neurological symptoms. He was last seen in follow-up by our team three months after his most recent EBRT, at which point he reported significant improvement in the right leg tremors/spasms and improved headache. He demonstrated decreased right foot drop but continued to have word-finding difficulties and short-term memory deficits. He has not developed any new areas of muscle weakness or paresthesia, visual loss, or other forms of altered sensoria. The current plan is to complete six cycles of CCNU with alternative systemic therapy thereafter upon disease progression. While temozolomide could be used to this effect, the high mutational burden found in his most recently resected disease would permit entry into currently available trials of immunotherapy. |
A 25 year old male who is a hospital ward attendant by occupation presented to emergency ward with the chief complaints of left sided chest pain, productive cough and low grade fever with night sweats for the last one month. This was associated with significant loss of his weight and appetite. There was no history of swelling of his limbs, jaundice and diarrhea. He was a non diabetic, non hypertensive and there was no past history of tuberculosis or any major febrile illness in the past. There was no history of intravenous drug abusers or high risk behaviors. On examination of the patient, he was febrile, with a pulse rate of 106 beats/min, blood pressure of 110/70 mmHg, and respiratory rate of 30/min. Respiratory system examination had a dull note on percussion with decreased breath sounds on auscultation suggesting the presence of a left-sided pleural effusion. Other systems yielded no clinical abnormalities. His chest x ray was suggestive of bilateral miliary mottling with a left sided moderate pleural effusion which later resolved with intercostal drainage (ICD) [Figure and ]. Laboratory evaluation showed exudative pleural effusion by Light's criteria with pleural fluid protein of 5.5 mg/dl, pleural fluid lactate dehydrogenase 1194 mg/dl, gram-stain showing no pus cells, with lymphocyte prominent cells on cytology and high adenosine deaminase levels of the pleural fluid 216 μIU/ml. Contrast enhanced computed tomography chest revealed multiple miliary nodules in the left lung, consolidation in the right lung with relative subpleural sparing []. The hematological profile showed mild anemia with high erythrocyte sedimentation rate. Except for his mild liver dysfunction, rests of the biochemistry results were normal. His viral markers (hepatitis B surface antigen, anti-hepatitis C virus, HIV) were negative. Blood, urine, and sputum culture were sterile, and sonography of abdomen was normal. With the above evidence, the patient was started on antitubercular therapy, isoniazid, rifampicin, pyrazinamide, and ethambutol under directly observed treatment, short-course regimen. On the 4th day, the patient had respiratory distress with oxygen saturation to 60% at room air and the arterial blood gas was suggestive of low PO2 40% with a PF ratio of 200. His chest X-ray was suggestive of acute respiratory distress syndrome (ARDS) [], and transthoracic two-dimensional echocardiography showed a good left ventricular function. The patient was given noninvasive ventilation with the initiation of steroids (prednisolone 1 mg/kg). After 3 days, patient oxygenation improved with facial mask and a repeated chest X-ray showed clearing of the infiltration in both lung fields []. The patient was continued on antitubercular medication; his liver enzymes were normalizing, and steroids were continued on tapering doses. After 15 days of admission, the patient was discharged with the continuation of antitubercular medication for 6 months. |
A 51-year-old Chinese male had undergone living-donor kidney transplantation for end-stage renal disease (ESRD) due to 8 years of nephrotic syndrome in 2011 at the age of 46 years. His immunosuppression regimen included a combination therapy with tacrolimus 2.5 mg/d, mycophenolate mofetil 1.5 g/d, and prednisolone 10 mg/d. The blood concentration of tacrolimus (FK-506) was monitored regularly at a target level of 4 to 10 ng/mL. No colonoscopy had been performed prior to transplantation, and he did not receive colonoscopic surveillance postoperatively. Renal graft function remained stable in him without any rejection episodes.\nFour years after transplantation, the patient presented with recurrent hematochezia (bright red blood per rectum mixed with stools), and a change in bowel habits manifested by frequent loose stools. He did not pay attention to these abnormal conditions until he began to suffer from progressive general malaise and weight loss of 3 kg during the period of 1 month. A colonoscopy was taken subsequently in January 2016, which revealed a near-circumferential mass at the middle rectum about 8 cm from anal verge accompanied by moderate luminal stenosis. The friable lesion proved to be adenocarcinoma by further biopsy. Having made a definite diagnosis, the patient was admitted to our institution in February 2016. He had a previous history of hypertension for 7 years that could be generally controlled by medication, and cigarette smoking for more than 30 years. Regarding his family history, there were no remarkable findings.\nOn admission, detailed physical examination revealed a temperature of 36.5°C, pulse of 80 bpm, and blood pressure of 106/60 mm Hg. No special signs were noted except for a healed surgery scar on his right lower abdominal wall. The lower margin of a solid, irregular mass was touched about 6 cm from anal verge through a digital rectal examination. Laboratory tests showed hemoglobin was 133 g/L, fecal occult blood test was positive, serum creatinine was 88 μmol/L, liver function test was normal, and most serum tumor markers (AFP, CEA, CA19-9, and CA72-4) were within the normal range but CA242 was slightly elevated. The following computed tomography (CT) scan demonstrated a soft tissue density mass protruding into the lumen of upper-middle rectum, with the wall thickening and peripheral lymph node metastasis and the transplant kidney was located in the right pelvic cavity (Fig. ). No evidences of distant metastasis were suggested. FK-506 was 4.7 ng/mL at that time. According to recommendation of interdisciplinary team including urologists, medical physicians, and general surgeons, immunosuppressive therapy with previous regimen was extended perioperatively. Meanwhile, 100 mg of intravenous hydrocortisone was added before anesthesia induction, and turned to a dose of 50 mg/8 h postoperatively for 1 day to prevent acute renal failure caused by the attack of surgery.\nWithout any surgery contraindications, a laparoscopic assisted low anterior resection with total mesorectal excision (TME) was performed then. After induction of general anesthesia, the patient was positioned in the lithotomy position. Complete exploration of the abdominal cavity suggested that there was no liver and peritoneal carcinomatosis. The tumor was identified locating at the middle-lower rectum. Specific surgical procedures started with lysis of abdominal adhesions from previous surgery. The sigmoid mesocolon and mesorectum were dissected along the inner side of the ureters by harmonic scalpel, and the vessels and lymphatics were ligated at the root of the superior rectal vessel with Hem-o-lock. The distal rectum was transected intracorporeally 3 cm from the distal margin of tumor with a EC45-A laparoscopic linear stapler (Johnson & Johnson, Cincinnati, OH, USA). After that, a 6 cm left supraumbilical incision was made to remove the proximal rectum, the distal sigmoid colon, and the surrounding tissues of the rectum 10 cm from the proximal margin of tumor. The specimen was obtained for further pathological evaluation (Fig. ). Reconstruction was performed intracorporeally in the manner of straight end-to-end colorectal anastomosis using a 28 mm transanal circular stapler (COVIDIEN, Mansfield, MA, USA) (Fig. ). Finally, 2 drainage tubes were placed in the pelvic cavity surrounding the anastomosis site. In order to prevent postoperative anastomotic leakage, a loop transverse colostomy was performed (Fig. ). The operation was completed successfully within 2 h with a proximate blood loss of 100 mL. Histopathology revealed a well-moderately differentiated adenocarcinoma measuring 4.2 × 5 × 2.3 cm, with invasion through the muscularis propria into pericolorectal tissues (Fig. ). The resection margins were free of tumor. Among the removed 14 lymph nodes, 3 contained metastatic cancer, indicating the tumor stage of pT3N1M0. Furthermore, immunohistochemistry showed the tumor was negative for MLH-1, MLH-2, MLH-6, and PMS-2.\nApproximately 4 h after surgery, the patient developed archorrhagia (dark red blood and clots per rectum), and the amount of blood loss increased up to 200 mL within 1 h. After exploration per anus, a bleeding wound near the anastomosis was identified and the control of hemorrhage was performed timely. There was no recurrence of archorrhagia ever since. The further postoperative course was uneventful. Graft function stayed well, and serum creatinine levels were always within the normal limits, ranging from 80 to 104 μmol/L. The ostomy was opened on the third postoperative day, and flatus was passed then. The patient went on a liquid diet 4 days after surgery and made a recovery soon. He stayed in hospital for 9 days after surgery, and drainage tubes were removed at discharge.\nDuring 4 months of follow-up period, immunosuppressive therapy with tacrolimus 2.5 mg/d, mycophenolate mofetil 1.5 g/d, and prednisolone 10 mg/d continued, no allograft rejection and complications were observed. Considering his tumor stage, he received adjuvant chemotherapy with a regimen of FOLFOX (oxaliplatin/5-fluorouracil/calcium folinate). After 8 cycles of chemotherapy, repeated CT scan indicated no evidences of local recurrence and distant metastasis. To date, the patient was in a good condition. |
A 35-year-old female with a diagnosis of chronic myeloid leukemia for seven years presented with a worsening weakness to the emergency department. The patient was diagnosed with CML in 2010 when she presented with fatigue, weakness, and headache. At that time, her complete blood count was significant for Hb of 5.9 g/dl with 400,000 WBC and massive splenomegaly. Bone Marrow was hypercellular with entire marrow replaced by myeloid lineage cells in various stages of maturation and less than 5% blasts with RT-PCR and peripheral FISH with 80% t (9,22) translocation (BCR-ABL). She was in remission on imatinib mesilate however, she stopped taking the medicines during her pregnancy in 2014. QPCR showed recurrence of the disease and imatinib mesilate was restarted. The patient was found to have a loss of molecular response on imatinib mesilate in 2017 and she was switched to nilotinib. Around the same time, the patient started complaining of weakness with difficulty climbing stairs. This weakness started before the patient was switched to nilotinib and progressed. Patient first started complaining of spasms and stiffness in multiple muscle groups followed by lower extremity weakness more pronounced on the left side than the right. Patient’s weakness continued to worsen, and she presented to the emergency department after a fall. At that time, the weakness involved her bilateral lower extremities and left upper extremity with loss of hand grip. No data supports such presentation of motor neuron weakness secondary to tyrosine kinase inhibitors which have a reported side effect of peripheral neuropathy. Physical exam was significant for signs of both upper and lower motor neuron disorder. An extensive workup was done as her presentation warranted a broad differential. Her complete blood count and comprehensive metabolic panel showed no abnormalities. Her serum CK level was 193U/L (24–195), CRP of 9.32 mg/dl (1.0–4.0), Vitamin B 12 levels were 382 pg/ml, methylmalonic acid level of 172 nmol/L Vitamin B1 of 90.3 nmol/L, serum copper level of 163 ug/dl (72–166) and zinc 70 mcg/dl (60–130). Her serum protein electrophoresis showed a normal pattern with no monoclonal bands identified on immunofixation. Multiple Cerebrospinal fluid studies were found to be normal. Her initial CSF exam showed clear, colorless CSF fluid with normal opening pressure, normal CSF cell count, CSF protein of 20 mg /dl and CSF glucose was 69 mg /dl. CSF cytology was negative. CSF ACE level was < 5 U/L with multiple repeat CSF studies with similar findings. CSF flow cytometry for myeloid and lymphoid disorders was negative. CSF immunofixation showed no monoclonal bands. CSF viral panel for West Nile/HSV/enterovirus/HIV/CMV/EBV and VZV was negative. Serum HTLV EIA screen with western blot was found to be negative as well. The patient was also tested for multiple different autoantibodies. Her serum acetylcholine receptor binding Ab was 0.0nmol/L, MuSK antibody levels of 0.0nmol/L, negative cryoglobulins, undetected serum levels of NMO Antibodies. MRI of the cervical, thoracic and lumbar spine performed using the following sequences: sagittal STIR, T1 weighted and T2-weighted images; axial T1-weighted and T2-weighted images for possible concern of extramedullary disease involvement but found to be an unremarkable study. Brain MRI showed nonspecific foci of T2/FLAIR signal hyperintensity in the subcortical white matter involving the left frontal lobe, bilateral parietal lobe and left basal ganglia. CT scan chest, Abdomen, and pelvis with IV contrast was essentially normal. The patient was treated multiple times with plasmapheresis and IVIG but did not improve her symptoms. The plan was then made to proceed with a sural nerve biopsy but patient went into pulseless ventricular tachycardia immediately after getting anesthesia. Later, nerve conduction studies and electromyography results were significant for electrophysiological evidence of diffuse pathology of motor neurons and their axons. After an extensive workup to rule out various pathologies and no substantial evidence, her constellation of symptoms was considered secondary to atypical presentation of ALS. Patient’s overall functional status deteriorated over the course of the next few months with worsening respiratory status with eventual tracheostomy and discharge to a skilled nursing facility. |
A 70-year-old male with PMH of CML in remission, peripheral arterial disease on apixaban, hypothyroidism, COPD, HLD, gout, a chronic indwelling catheter who was chronically bedbound presented from a skilled nursing facility with altered mental status and abdominal pain. While in the emergency department, he was hypotensive with an initial blood pressure of 96/53 mmHg and tachycardic. Patient's chronic Foley catheter appeared blocked and upon removal of the catheter purulent drainage was noted. Initial laboratory testing was significant for leukocytosis of 43,400 cells/mL, lactic acidosis with initial lactic acid of 2.3 mMol/L and an acute kidney injury with a creatinine of 2.92 mg/dL and an elevated INR of 2.3, which was secondary to apixaban therapy. The patient's blood pressure did not improve after 30 mL/kg of fluid resuscitation and patient was started on a vasopressor infusion. CT abdomen pelvis without contrast performed in the emergency department revealed gastric pneumatosis with adjacent left upper quadrant portal venous gas along with branching portal venous gas throughout the liver (). The patient was started on broad-spectrum antibiotics with cefepime, vancomycin and meropenem and urine cultures, as well as blood cultures, were sent. The patient was transferred to the step-down unit for closer observation.\nThe general surgery and gastroenterology teams were consulted for further evaluation while the patient was in the step-down unit. Given the patient's therapeutic INR and lack of any small bowel findings on the initial CT scan, embolism causing mesenteric ischemia was thought to be unlikely. However given the acute renal failure, CT angiography was not performed. Esophagogastroduodenoscopy was performed revealing proximal stomach ischemia from the midbody to the fundus with some necrosis with normal appearing distal stomach and duodenum (). Biopsies revealed acute hemorrhagic gastritis secondary to ischemia with recommendation of surgical resection (). The patient was deemed to not be a surgical candidate by the general surgery service given his multiple comorbidities and was managed conservatively with intravenous acid suppression therapy, intermittent nasogastric tube suction, bowel rest with total parenteral nutrition along with broad-spectrum antibiotic therapy for his urosepsis. Patient's blood and urine cultures grew extended-spectrum beta-lactamase Proteus mirablis.\nDays later after the patient's acute renal failure resolved, CT angiography was performed which revealed mild narrowing of the celiac artery with patent flow. The superior and inferior mesenteric arteries, as well as the left gastric artery, were all found to be patent and the prior noted gastric pneumatosis and portal venous gas were resolved. The patient began to show clinical improvement and repeat EGD 1 week later revealed gastritis in the proximal stomach with healing pink mucosa and no signs of ischemia or necrosis. The patient was then slowly started on an oral diet and after completion of his antibiotic course was transferred to the medical floor and later discharged to a long-term acute care facility. |
A 59-year-old Caucasian male developed 3 weeks of progressive lower extremity edema and was found to have proteinuria on urinalysis. Twenty-four-hour urine protein was 16 g and serum protein electrophoresis showed a monoclonal immunoglobulin G lambda type. A renal biopsy established the diagnosis of lambda light chain amyloidosis. Evaluation for other organ involvement including serum troponin, echocardiogram, esophagogastroduodenoscopy and colonoscopy with random biopsies was negative for amyloid involvement. Treatment included melphalan- and dexamethasone-based chemotherapy followed by autologous stem cell transplantation. Despite therapy, total serum-free light chain levels and massive nephrotic syndrome persisted. Three months after his stem cell transplant, he received an additional 5-month course of bortezomib therapy with no hematological response. The following 9-month period was marked by increasing proteinuria (15.8–24.4 g/24 h) and autonomic dysfunction with disabling orthostatic hypotension with postural syncope such that he became wheelchair dependent. Renal function worsened with 24-h creatinine clearance of 15 m/min and 24.4 g of urinary protein excretion in 24 h. Serum albumin fell to 1.2 g/dL. He was treated with alternate day albumin infusion with temporary symptomatic improvement. Due to poor functional status thought to be related to ongoing proteinuria, the decision was made to pursue therapeutic renal ablation to decrease daily urinary protein loss and restore plasma oncotic pressure. A trial of naproxen therapy did not improve his proteinuria. He was referred for consideration of surgical nephrectomy. As an alternative approach, with the aim of minimizing hypotension related to nephrectomy, the patient underwent hand-assisted laparoscopic ligation of both native ureters. Urine output and protein losses ceased immediately. Post-operative renal ultrasound confirmed mild hydronephrosis. Recovery was uneventful and the patient did not complain of pain associated with the procedure. Blood pressure, serum albumin and postural symptoms gradually improved (). During 12-day hospitalization, nutritional and functional status improved and he was dismissed home on long-term hemodialysis requiring minimal assistance for ambulation. |
A 58-year-old male, who was otherwise in good health, presented to us with a history of gradual onset of a slowly progressive painless swelling in the upper nasal quadrant of the orbit for 2 years. It was not associated with any pain or other ocular symptoms. The patient did not give history of trauma or any other systemic ailments or swellings elsewhere in the body.\nOcular examination revealed a best-corrected visual acuity of 20/20 in both eyes with normal intraocular pressure and posterior segment examination in both eyes. The left orbit showed a diffuse mass measuring 20 mm × 10 mm in the upper nasal quadrant []. The eyelids were free from involvement and had complete movements. There was no proptosis and ocular movements were full and complete in both eyes. A plain computerized tomography scan of the brain and orbit was performed which showed the presence of a soft-tissue density mass lesion (HU 40–60) with few internal fat densities in the superonasal quadrant of orbit, measuring 12 mm × 8 mm in size. There was no evidence of internal calcification or scalloping/erosions of the underlying bone. Rest of the structures of eye and brain were found to be normal []. The patient underwent complete excision of the mass under local anesthesia. Profuse bleeding was noted during surgery and was attributed to the presence of a feeder blood vessel which was ligated before complete excision in toto [].\nThe excised intact mass was sent for histopathological examination which revealed a well-circumscribed tumor mass measuring 20 mm × 10 mm, composed of small delicate papillae projecting into the lumen []. The papillae were composed of a single layer of endothelial cells surrounding a collagenized core. A diagnosis of IPEH was made. The patient was followed up for 20 weeks and was free from symptoms and showed no signs of recurrence []. |
A 25-year-old multiparous woman presented in November 2015 with eight months' history of bilateral breast enlargement. The chief complaint was painful and swollen breasts, accompanied by multiple palpable nodules and erythema. She reported use of birth control implant by two years at the age of 23 years, after her last pregnancy.\nOn clinical examination, the woman showed bilateral and symmetrical breast enlargement and a palpable mass of 4 cm diameter in the lower inner quadrant of the right breast, while no palpable lesions were found in the left breast. The breast skin was red and swelling, with peau d'orange appearance, whereas nipple areola complex was normal. Edema was also observed in both breasts (). Notably, an axillary lymph node was palpable in the right axilla. At the interview, she stated no familiar or personal history of breast cancer.\nSince inflammatory breast carcinoma was suspected, a bilateral mammography was initially recommended. Nevertheless, the diagnosis was not conclusive due to the extremely dense breast tissue. Then, a complementary ultrasound was performance, revealing heterogeneous breast echotexture with hypoechoic areas and multiple circumscribed solid masses in both breasts, ranging in diameter from 3 to 8 cm (Figures –). In addition, ultrasound examination revealed two suspicious axillary right lymph nodes with increased cortical thickness. After imaging examination, this case was categorized as BI-RADS 4A.\nTo the pathologic diagnostic, fragments of the large mass from the right breast were obtained by percutaneous core needle biopsy. Also, a fine needle aspiration biopsy of the right axillary lymph node was performed. Pathologists reported breast parenchyma that was composed of ductal-lobular unit and breast stromal tissue containing a complex pattern of linear spaces lined by endothelial-like spindle cells. Those cells exhibited strong positivity for CD34, actin, and calponin (Figures –) and negativity for estrogen and progesterone receptors. The diagnosis was benign breast lump with features of a diffuse PASH. Meanwhile, fine needle aspiration biopsy of right node reported lymphoid hyperplasia.\nDue to the extension of the disease, the patient was subjected to a bilateral mastectomy with immediate reconstruction. A magnetic breast resonance was performed for surgical planning, confirming multiple bilateral circumscribed masses with avid and persistent enhancement after gadolinium administration, which is usually related to a benign etiology (Figures –). After the referred surgery, and taking into account the imaging features of the lesion, the diagnosis was changed from diffuse PASH to tumoral PASH in a careful interdisciplinary review of the case.\nCurrently, the patient is tumor-free on one-year follow-up with excellent cosmetic outcome and breast symmetry. |
A 68-year-old Japanese man with a history of distal partial gastrectomy for gastric cancer 10 years earlier was admitted for surgical treatment of intrathoracic esophageal cancer (T3, N2, M0, stage III). He underwent subtotal esophagectomy with three-field lymph node dissection and removal of the remnant stomach with the abdominal lymph nodes. The alimentary continuity was reconstructed with a pedicled jejunal limb through the antethoracic route. When we separated the diaphragm from the esophagus and removed xiphoid surgically to prevent a pedicled jejunal limb injury, the pericardium was opened. Anastomosis of the esophagus and jejunum was carried out instrumentally with a circular stapler. A postoperative enteral contrast examination showed smooth passage and no deformity of the reconstructed jejunum. The patient was discharged about 4 weeks postoperatively. About 6 months after the esophagectomy, the patient was readmitted to our hospital because of abdominal discomfort and vomiting. His vital signs were stable and unremarkable. He was thin, with a body mass index of 18.6 kg/m2. Physical examination revealed that the pedicled jejunum through the antethoracic space was markedly dilated and the abdomen was soft and flat (Fig. ). Laboratory data showed only leukocytosis with no other remarkable findings. A chest roentgenogram revealed an increased cardiothoracic ratio of 70%, and an enteral contrast study showed a bird’s beak deformity. The swallowed barium remained static in the reconstructed jejunum. Computed tomography of the thoracoabdominal region showed the reconstructed jejunum within the pericardium anterior to the heart (Fig. ). We diagnosed the patient with IPDH after esophagectomy and performed an emergency laparotomy. Upon opening the abdominal cavity, we found that the reconstructed jejunum had herniated into the pericardium through an approximately 4-cm-diameter diaphragmatic defect without a hernia sac (Fig. ). The herniation was easily reduced, and the congestion of the incarcerated jejunum resolved. There was no evidence of bowel ischemia. Primary closure of the diaphragmatic defect was accomplished using vertical mattress sutures with 1–0 silk (Fig. ). Because the diaphragm adhered to the pericardium around the diaphragmatic defect, we closed these together. Moreover, to reinforce the closure of the diaphragmatic defect, we used a graft harvested from the rectus abdominis posterior sheath. Interrupted sutures with 3–0 nylon were placed to fix the 8 × 6 cm graft of the rectus abdominis posterior sheath to the diaphragmatic defect, preventing recurrence of the hernia (Fig. ). Postoperatively, an upper gastrointestinal study confirmed free flow of contrast medium from the cervical esophagus into the intra-abdominal jejunum through the pedicled jejunum. The postoperative course was uneventful, and the patient was discharged on the seventh postoperative day. |
A 35-year-old male patient reported with the complaint of a scar and hollowing on the right side of forehead, following road traffic accident 3 months back. He had received conservative treatment only. On examination, there was a “S-” shaped concavity present at the junction of lateral one-third and medial two-third of right eyebrow extending upward, approximately of about 6 cm × 4 cm in size, in the right lateral forehead [Figure –]. Computed tomography scan revealed comminuted frontal bone fracture in the right side without any dural tear [Figure –]. Thus, a cosmetic correction of the defect was planned.\nA stereolithographic model of the defect area of the skull was reconstructed. The undercuts were blocked with modeling clay. An impression of addition silicone impression material in light body consistency was made and poured in Type III dental stone. A wax pattern was fabricated with base plate wax so as to provide proper bony contour of the depressed defective area alongside covering the gaping bony defect [Figure and ]. Care was taken to match the external bony contour of the affected region with that of the unaffected contralateral side. The wax pattern was then acrylized with double heat-cured medical grade PMMA of the colorless variety. Due to its less residual monomer, it is supposed to cause least tissue irritation [Figure and ].\nCare was taken to gradually blend the margins of the acrylic fabrication with the edges of the defect. Holes were drilled around the edges of the acrylic fabrication and on the surface with a number 8 round bur which would be helpful to secure the prosthesis with screws to the underlying bone. Once satisfactory fit was achieved, the prosthesis was finished and polished and made ready for surgical placement.\nOroendotracheal intubation was done. Coronal flap was elevated. Dissection was done to the pericranium. Disposable Raney clips aid in hemostasis. After definite reflection of the temporal region, the PMMA implant was tried in and was fixed with two 2 mm × 8 mm screws and two 2 mm × 11 mm screws. These screws were placed without damaging the dura. One screw was placed at the edge of the implant while the others were placed at the center. Few holes were kept open for fibrous encapsulation of the implant with the pericranium. Proper flushing of the implant margins with the cranium was ensured before closure. Closure was done in layers [ and ]. |
A 38-year-old man experienced sudden onset of lower back pain when he bent down to pick up a newspaper off the floor. There was no history of trauma, cardiovascular disease, and bleeding disorders as well as no experience with drugs. The lower back pain worsened, and bilateral leg pain developed within 3 days. The subject received a medical examination and a magnetic resonance imaging (MRI) scan of the lumbar spine at a local hospital. MRI images revealed a large subdural hematoma, extending from L1 to S1 in the sagittal view and presenting as hyperintensities on T1 weighted sequences and hypointensities to isointensities on T2 weighted sequences (Fig. ). A conservative treatment plan was decided upon at a local hospital because there were no severe neurological deficits. However, the symptoms worsened progressively with lower extremity muscle weakness, gait disturbance, and numbness in the saddle area 15 days after onset. Therefore, the subject was transferred to our center for further examination and management.\nA neurological examination demonstrated paresthesia and pain below the L4 dermatome and motor weakness at grade 4 on the right lower limb and grade 3 on the left lower limb. The bilateral Achilles’ tendon reflex decreased, and the straight-leg-raising test was positive for both lower limbs. There was no evidence of bowel and bladder disturbances, and no pathological reflexes were identified. Laboratory tests revealed an acceptable platelet count and normal coagulation. A repeated MRI scan of the lumbar spine revealed an increase in the size of the subdural hematoma from L4 to S1 in the sagittal views, and the cauda equina was dorsally compressed in the axial views (Fig. ). Furthermore, there was a change in the signal intensity of the subdural hematoma, which presented as hyperintensities on both T1-weighted and fat-suppressed T2-weighted sequences (Fig. ).\nSubdural evacuation of the hematoma was performed immediately to improve the neurological symptoms. After bilateral L5-S1 laminectomy, the ligamentum flavum sustained hypertrophy and turned brown, and it was resected intraoperatively (Fig. ). A pathological examination showed degeneration and formation of a new hematoma within the ligament (Fig. ). The dura mater was tough and discolored (Fig. ). After opening the dura with a longitudinal midline incision, dark brown blood drained spontaneously (Video 1). The region was irrigated with saline until the cerebrospinal fluid was clear and the nerve roots were visible through the intact arachnoid membrane. There was no evidence of a vascular abnormality and no bleeding from the subarachnoid space. After closing the dura with running lock sutures, its pulsatile motion was restored (Video 2).\nPostoperatively, the lower limb pain improved immediately. An MRI revealed complete drainage of the chronic SDH (Fig. ). The patient was discharged 1 week after surgery. At the 6-month follow-up, the pain and numbness of the lower limb pain disappeared, and the muscle strength of both legs recovered completely with normal gait. |
A 44-year-old Japanese woman with a 6-year history of insulin-dependent diabetes mellitus and an 11-year history of central diabetes insipidus presented with a pain in the genital area worsening over 2 weeks, general fatigue, and loss of appetite. Two months earlier, patient underwent a urinary catheter insertion as a management for urinary frequency, but it was removed during the previous hospital stay, a month before her recent hospitalization, for possible urinary infection. She has had frequent hospital admissions (6 times/year) and was hospitalized 3 months before her recent admission because of edema of the pelvic area and lower limbs. The patient developed diabetes mellitus after undergoing total pancreatectomy for nesidioblastosis, a surgical procedure which involved the removal of patient’s pancreas including the spleen and gallbladder. Her sister was also diagnosed with idiopathic central diabetes insipidus; thus, a family etiology was suspected. The patient had a surgery for suspected tongue cancer 2 years ago and was also suspected of non-alcoholic steatohepatitis with episodes of hepatic encephalopathy. Although she was on multiple medications including subcutaneous insulin injections and desmopressin tablets, her glycemic and hydration status were poorly controlled.\nFour days prior to the present admission, she visited a gynecologist for her inguinal pain. No uterine tenderness or exudate was observed, and she was prescribed gentamicin and lidocaine ointments for possible local infection. She developed edema in the pelvic area with loss of appetite, and her home doctor consulted the university department 1 day before the present admission.\nUpon admission, the patient appeared weak but was alert and had low-grade fever (37.4 °C) under a regular use of acetaminophen (1500 mg/day) and diclofenac (75 mg/day). Her blood pressure was not significantly different from previous measurements (88/42 mmHg) but a sinus tachycardia (heart rate 125/min) was noted. She complained of continuous abdominal pain and tenderness in all four quadrants. No abdominal guarding or rigidity was observed, but she had severe edema in the pelvic and bilateral femoral areas without necrotic skin discoloration. Laboratory investigations revealed a white cell count of 16,310/μL with neutrophilia (90.8%), elevated C-reactive protein of 22.18 mg/dL, and no serum sodium or potassium abnormalities. Serum aspartate aminotransferase and alanine aminotransferase were elevated at 466 U/L and 148 U/L, respectively. The patient’s international normalized ratio was high (2.26), but disseminated intravascular coagulation score did not meet the criteria. The patient’s HbA1c level was 8.8%, and blood sugar at admission was 316 mg/dL. She had low serum albumin concentration (1.7 g/dL), elevated serum ammonia concentration (154 μg/dL), and elevated lactate level (10.3 mmol/L). No ketonuria was noted, but significant pyuria was observed.\nThe abdominal ultrasound was unrevealing; thus, an intravenous treatment with ceftriaxone (1 g every 8 h) was initiated empirically after obtaining the blood and urine culture samples. A CT scan performed the following morning revealed the presence of air in the soft tissue of the inguinal and pelvic areas, such as pectineal and psoas major muscles (Fig. ). Immediate infectious and surgical consultations were made, and the antibiotics were changed to meropenem (1 g every 8 h), vancomycin (1 g every 12 h), and clindamycin (600 mg every 8 h). Gram-positive cocci and gram-positive rods were found in the initial blood cultures. In the evening of hospital day 2, a surgical debridement of the extraperitoneal pelvic tissue with colonostomy was performed, and the CT image after the operation suggested a complete resection of the affected tissue. However, hypernatremia (a serum Na concentration of 160 to 170 mEq/L) ensued as the use of nasal desmopressin could not effectively control the patient’s central diabetes insipidus after the operation.\nNo bacteria could be cultured from the debridement tissues (Table ). The blood culture isolates were finally identified as Streptococcus constellatus using superoxide dismutase A sequencing and C. ramosum by 16S ribosomal DNA sequencing []. The minimum inhibitory concentrations (MICs) of various antibiotics were tested using Etest for C. ramosum [] and the broth microdilution method [] except imipenem and meropenem (Etest) for S. constellatus (Table ). These results were interpreted using the Clinical & Laboratory Standards Institute M11-A8 document [] for C. ramosum and M100-S24 document for S. constellatus. Both strains were susceptible to penicillin, meropenem, and clindamycin. Despite the continued use of susceptible antibiotics and intensive care, disseminated intravascular coagulation and pancytopenia developed, and the patient died on hospital day 8. |
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 55-year-old man presented to our hospital with a hard mass in his neck. He noticed that the mass had been gradually increasing in size for six months. In the last month, he reported of having experience of hoarseness and dyspnea. In addition, his previous medical history was unremarkable. Clinical examination showed not only the hard mass with a size of 3 × 4 cm and limited movement but also bilateral cervical lymph nodes.\nNeck ultrasound and 128-slice computed tomography scan (as per ) revealed a diffusely enlarged thyroid gland, whereas the right lobe size was approximately 16 × 29 mm and the left lobe size was about 28 × 25 mm. The tumor was found to infiltrate the tracheal wall and narrow the tracheal lumen by 90%. Besides, there were suspected metastatic lymph nodes in levels II, III, and IV in both sides with the largest dimension roughly 14 × 10 mm. Flexible fiberoptic laryngoscopy demonstrated limited movement of bilateral vocal cords. Tracheobronchoscopy indicated that the mass invaded the upper part of the trachea but still was covered by normal tracheal mucosa. This mass caused the difficulty in the evaluation of the lower part of the trachea. Other examinations were not particular.\nThe clinical stage was classified as cT4aN1bM0, stage III (according to AJCC, 8th edition, 2017) and stage IV (according to the classification of McCaffrey et al.). Because endotracheal intubation was not applicable due to constricted trachea, tracheostomy was performed for anesthesia. Intraoperative evaluation has shown that (i) the tumor invaded the trachea in the length of approximately 7 cm and (ii) reconstruction of the remaining parts of the trachea after resection was impractical. Eventually, total thyroidectomy, resection of the involving part of the trachea, and permanent tracheotomy (as per ) were performed. This patient was discharged after 14 days. Subsequently, RAI ablation was administered. During one-year follow-up, evidence of tumor recurrence and complication was not found. |
A 51-year-old Korean man with a past medical history of hypertension and hyperlipidemia was referred on Jan 7, 2014, to the Yonsei University Health System for treatment of a known GCT. The colonoscopic finding was an approximately 2.0-cm-sized, hard, rectal mass 10 cm above the anal verge (). Histopathology findings indicated a GCT composed of round tumor cells having abundant granular cytoplasm with no evidence of malignancy. The patient had no other symptoms when he visited a local hospital on December 13, 2013, for a screening colonoscopy. He had no specific family history, but his sister had had breast cancer. He had a 10 pack/yr history of smoking and a body mass index of 25.7 kg/m2.\nOn admission, the patient appeared well. The laboratory findings were all within normal limits. Although the computed tomography (CT) findings made us suspicious of T3 rectal cancer (), subsequent magnetic resonance imaging (demonstrated a probable submucosal rectal mass with mesorectal fat infiltration above 8 cm from anal verge (Rb), but without circumferential resection margin threatening, extramural vessel invasion, or regional lymph-node enlargement (). His chest x-ray showed neither active lung disease nor evidence of lung metastasis.\nWhen we reviewed the English literature, we were able to find only a few cases involving a GCT that had extended to the level of the muscularis propria. Our colorectal team, including colorectal surgeons, oncologists, gastroenterologists, and pathologists, discussed an appropriate treatment for this rare case. Even though we first considered a low anterior resection and lymph-node dissection, we finally decided to perform a TEO, with which we had had plentiful experience for patients with early rectal cancer. The surgery was performed on January 14, 2014. The patient had an uneventful postoperative course and was discharged 6 days after surgery. Two weeks later, the patient visited our clinic, and the final pathology revealed that the tumor consisted of a 1.7 × 1.5-cm GCT (), which had been positively stained for the S-100 protein () and had invaded the subserosa with clear margins (). The patient exhibited no other risk factors for malignancy according to Fanburg-Smith criteria []. Because of the rarity of this disease entity and the lack of experience with a GCT with subserosal invasion, short-term follow-up was recommended. The CT scan and sigmoidoscopy performed nine months later showed no evidence of recurrence. |
A 66-year-old man with no history of concomitant conditions was admitted to our hospital. He had been operated on due to a tumour on the distal third of the left arm as a result of a closed trauma without fracture 20 years before. Several debridement surgeries were also performed on his lesion, but no detailed information is available. Physical examination revealed new lumps with seropurulent discharge 7 years after the first surgery. Subsequent blood analysis did not show alteration in acute phase reactants, and hematimetric indexes were within normal ranges. For the next 20 years, he continued to experience similar episodes of sporadic reactivation of the lumps.\nBefore 4 years, he was admitted to our hospital due to painless lumps fixed on the subcutaneous tissue of the left arm (). Radiographs of the left humerus revealed an alteration of the bone structure with distal diaphyseal involvement and thickening and cortical irregularity, which extended up to the metaphysis (). A computed tomography (CT) scan disclosed an alteration in the osseous morphology of the metaphysis–diaphysis in the distal third of the humerus at an intramedullary and cortical level, with osteolysis, periosteal reaction, diffuse involvement of adjacent soft tissues and several lower density, superficial, poorly defined liquid areas (). A magnetic resonance imaging (MRI) scan confirmed the multiple liquid deposits on the thick walls of the soft tissues and muscular oedema related to joint effusion ().\nAspiration was performed, and the extended coloured (Diff-Quick) haematoxylin–eosin, pap-test showed lymphocytes, leukocytes, macrophages, multinucleated giant cells and accumulations of bacillary structures (Actinomyces), consistent with a granulomatous inflammatory process (). Cultures (glucose asparagine agar, arginine-glycerol and tyrosine agar mediums) were subjected to a prolonged incubation of 21 days under aerobic and anaerobic conditions and were negative.\nThe patient underwent a surgical resection of necrotic tissue and insertion of an antibiotic cemented rod (vancomycin) through a lateral approach of the arm ().\nSamples were cultured and sent to pathological anatomy. The patient developed a radial palsy, which resolved spontaneously in 6 weeks. Cultures were also negative, and pathological anatomy revealed the presence of gram-positive filamentous bacilli consistent with bacterial colonies of Actinomyces. At the osseous level, histological sections evidenced important morphological changes in the fibrofatty medullary bone with lymphocytes, plasma cells and polymorphonuclear cells. The patient received intramuscular penicillin G, 4,000,000 IU every 6 h for 6 weeks, followed by oral amoxicillin for 6 months. Clinically, his symptoms improved, but new active cutaneous fistulas reappeared after 2 years.\nHe underwent a reoperation, and the antibiotic cement was replaced, followed by excision of the necrotic cortical tissue. The antibiotic therapy consisted of 2 g of intravenous ceftriaxone every 24 h for 4 months.\nDespite this last procedure, fistula formation continued a year later. Radiology studies and an MRI scan evidenced that the disease had spread to the metaphysis of the proximal humerus ().\nConsequently, wide surgical resection of the humerus was performed, in which the proximal metaphysis was preserved. An antibiotic cemented rod with vancomycin was inserted ( and ). The new results from pathological anatomy continued to disclose the presence of colonies of Actinomyces. Cultures were still negative.\nSubsequently, two new debridement surgeries of the fistulas were carried out. After 6 months, symptoms continued, the rod was replaced with a new one and antibiotic beads were inserted in the fistular areas (). Cultures were negative for Actinomyces, but the patient developed a Staphylococcus epidermidis infection, which further complicated his clinical condition. The new samples sent to pathological anatomy continued to disclose the presence of colonies of Actinomyces. The future therapeutic plan includes eventual total humerus reconstruction with prosthesis, once the infection has been completely eradicated. |
A normally fit and well right hand dominant twenty-six-year-old man sustained a left Rockwood grade V acromioclavicular joint dislocation from a rugby injury in June 2015, complaining of pain and a grinding sensation from his left shoulder on abduction when seen in January 2016. represents the preimplant radiographs. After discussion, he elected to undergo surgery to correct the position of his distal clavicle.\nAn uncomplicated LARS procedure was performed in March 2016 with satisfactory intraoperative reduction and reasonable postprocedure radiographs. However, three months after operation, he re-presented with an acute history of the distal clavicle again becoming prominent and elevated with no intervening history of trauma, and subsequent radiographs demonstrated the loss of position, as seen in . He opted for a revision procedure, performed in July 2016.\nIntraoperatively, the clavicular screws were secure. represents the removed LARS intraoperatively. On removal, the ligament was found to be tightly held within the bone canal (on the right in ; however, the LARS was found to be frayed deep to the coracoid, as seen on the left in ). There was no apparent cause for the LARS to fray under the neck of the coracoid, as no sharp bony prominence was found that could have eroded the LARS. A revision double LARS ligament repair was completed uneventfully. The patient was seen in clinic in August 2016. Radiographs, as seen in , demonstrated satisfactory reduction and the patient reported good function. The patient remained satisfied with the clinical outcome at repeat follow-up in September 2016 with full range of motion in the shoulder and radiographs showing a slight superior clavicular migration of 0.5 cm. Heterotopic ossification medial to the medial clavicular screw was visualized on postoperative Figures and but not identified intraoperatively and was considered to not be implicated in the failure.\nThe second patient is a forty-seven-year-old male builder who had sustained a grade V Rockwood ACJ dislocation after falling from a motorbike. The ACJ dislocation was successfully reduced using LARS. The ligament seemed to have failed spontaneously, when three weeks after surgery he awoke with the distal clavicle elevated. On exploration, the LARS had frayed at its medial limb, as seen in , between its position at the clavicle and coracoid. |
An 11-month-old boy was referred to a tertiary center for failure to thrive, poor muscle tone, short neck, kyphosis, and unusual spacing between teeth. He was diagnosed with infantile HPP after repeated low ALP activity test results and radiographic assessment of severe rickets-like skeletal changes and tongue-like lucencies projecting into the metaphyses. He was hospitalized multiple times for pneumonia likely related to musculoskeletal manifestation of HPP, which required treatment with intravenous antibiotics. At age 9 years, he developed persistent headaches; a magnetic resonance imaging (MRI) cranial scan confirmed craniosynostosis with Chiari malformation and cerebellar tonsillar herniation. He underwent craniovertebral decompression, with removal of the posterior arch of C1; a ventriculoperitoneal shunt was inserted to relieve intracranial pressure. He subsequently underwent 2 shunt revisions.\nThe patient experienced multiple fractures, starting at age 17 years, when he sustained bilateral femoral fractures when jumping off a wall; this required bilateral intramedullary rod insertion. At age 18 years, he sustained a right tibial fracture while jumping. Bone healing was delayed, but the fracture eventually healed satisfactorily. At age 20 years, he sustained bilateral femoral fractures when he rolled off his bed during a seizure and required rehabilitation for approximately 8 months.\nOver the first 18 years of life, the patient was hospitalized 8 times for a total of 43 days (Table ). Of these hospitalizations, 5 separate admissions required a stay of ≥5 days; the stay for insertion of a ventriculoperitoneal shunt was 14 days.\nOutpatient specialist visits represented a significant proportion of the healthcare resource utilization by this patient (Fig. ). Most of the outpatient specialist visits required consultation with providers in 12 specialties, including pediatric dentist (38 visits), pediatric endocrinologist (32 visits), neurosurgeon (27 visits), and general pediatrician (19 visits; Table ). Outpatient management consisted of diagnostic imaging procedures (Table ). The most frequent procedures were radiography of the limbs and spine, performed on 18 and 12 occasions, respectively; the patient also underwent 14 MRI cranial scans. Dental surgery and tooth extraction were performed as day case procedures for management of dental carries and malocclusion on 3 occasions. |
A 12-month-old Thai girl was brought to our emergency department with high-grade fever, cough, and dyspnea. Her underlying disease was gastroschisis. She underwent primary abdominal closure at birth without complications.\nInitial findings showed hypoxia, suprasternal and subcostal retraction, and crepitation on chest auscultation. According to these signs and symptoms, an initial provisional diagnosis of ARDS and septic shock had been made. All later investigations led to a diagnosis of severe bacterial pneumonia as a primary cause of ARDS. Upon examination, she had a body temperature of 39.0 ºC with a heart rate of 170 beat per minute, a respiratory rate of 70 breaths per minute, an arterial blood pressure of 70/30 mmHg, and an oxygen saturation of 80%. She looked drowsy with dyspnea. Mild cyanosis was seen on her lips. In addition to the abnormal sound found with stethoscope, we noticed that there was an abdominal paradoxical respiration. With respect to the conditions, she was intubated and supported with conventional mechanical ventilation (CMV) at a mode of pressure control with positive end-expiratory pressure of 12 mm H2O and total inspiratory pressure of 30 mmHg. Despite the aggressive management, she still had severe hypoxia with a partial pressure of oxygen (PaO2) of 82 mmHg. Consequently, she was transitioned to HFOV after 6 hours of CMV.\nDuring the first 2 days of support with the maximum amplitude of HFOV and mean airway pressure of 32 mmHg, she was able to be maintained with an oxygen saturation of over 88%. Her condition seemed to uneventfully improve. During a weaning of mean airway pressure on day 7 of HFOV support, however, she developed an abrupt onset of inadequate ventilation and oxygenation. A radiography revealed left tension pneumothorax requiring intercostal drainage (ICD). A few hours after ICD, she suffered from a markedly distended abdomen with subcutaneous emphysema across her entire upper torso. Again, plain thoracoabdominal film showed pneumothorax with pneumomediastinum and a markedly large amount of pneumoperitoneum (Fig. ). Her abdominal circumference suddenly increased from 40 to 50 cm. She underwent a bedside abdominocentesis withdrawing approximately 1 L of air to release intraabdominal pressure, partially improving her hemodynamic instability. Her abdomen was still tense and her hemodynamics was unstable. Subsequently, to exclude diaphragmatic injuries secondary to ICD, hollow viscus perforation, or abdominal compartment syndrome as a primary cause, she was transferred for surgery. As she had financial support from the government, there was no financial challenge for her further management.\nWith an upper transverse incision, soft tissue emphysema was found in several places, including esophagogastric junction, mesenteric root, and terminal ileal serosa (Fig. ). There was no fluid or blood intraabdominally. There was no intestinal perforation and the lesions were left in place. There were no lesions on both sides of her diaphragm. Therefore, the presence of intraabdominal air was certainly due to emphysematous leakage at the aforementioned sites not a passage through diaphragm or intestinal perforation. There was no complication in the postoperative period. After 20 days in an intensive care unit, she was extubated and transferred to a pediatric ward. She was on oxygen therapy, on chest physical therapy, and kept hemodynamically stable until she had recovered. She was doing well at the time of 2-week interval follow-up. Currently, 12 months later, she is well without any sequelae from surgery. |
This patient is a 14-year-old male, who felt a popping sensation and significant right knee pain while jumping and colliding with another player during a basketball game the previous day. Following the injury, he was evaluated in an outside emergency department, where anterior, posterior, and lateral radiographs obtained in the emergency department demonstrated a tibial fracture consisting of two primary components (). He was placed in a cast and sought a second opinion regarding findings and management.\nUpon presentation to the clinic the following day, he reported mild pain (3/10) and noted no normal function of his leg. A physical exam was performed but was limited due to pain. Following the review of radiographic imaging, an MRI was performed, which demonstrated a type IIIB tibial tubercle avulsion fracture and complete tear of the patellar tendon from its distal attachment site, as well as a hematoma at the fracture site (). After discussing the findings with the family, the patient was scheduled to undergo open reduction internal fixation of a type IIIB fracture and repair of the patellar tendon three days following the initial injury.\nAn 8-centimeter anterior incision was made at the superior aspect of the tibial tubercle and extended distally. At the patellar tendon insertion site on the tibia, the tendon was noted to be completely avulsed from the bone cortex distally, while proximally, the tendon remained attached to the displaced tubercle. The tendon remained attached to the inferior pole of the patella. The anterior tibial plateau fragment was anatomically reduced using two fully threaded noncannulated screws (Arthrex, Naples, FL), while the tibial tubercle fragment was reduced via bicortical fixation with a 50 mm fully threaded 3.5 mm cortical screw (Arthrex, Naples, FL).\nThe distal patellar tendon was completely avulsed through two-thirds of its length. To restore the native footprint of the patellar tendon, a 4.5 mm PEEK (polyetheretherketone) corkscrew anchor (Arthrex, Naples, FL) was placed slightly lateral to the anatomic insertion site to avoid a stress riser on the anterior tibial cortex. The anatomic repair of the patellar tendon was completed with two mattress sutures and tied.\nIn addition to the avulsion of the patellar tendon and periosteum, it was noted that fascial tissue with tibialis anterior muscle belly avulsed through the injury site causing subacute extensive bleeding within the anterolateral compartments (). This scenario raised concern for impending compartment syndrome, and an anterolateral compartment release was planned.\nThree 3-centimeter incisions were made along the anterolateral aspect of the leg. The first was located 3 centimeters distal to the neck of the fibula, the second was located 10 centimeters above the distal fibula tip, and the third was located at the midpoint between the two. Under endoscopic visualization, the intramuscular septum was identified and Metzenbaum scissors were used to cut through the fascial compartment beginning in the anterior compartment and extending proximally then distally to the midtibia (). The fascial incision was extended posteriorly into the peroneal compartment and then was extended proximally and distally to the midtibia. These steps were repeated for the midpoint and distal incision sites. Distally, the course of the superficial perineal nerve was identified and the nerve itself was protected during the distal release of the anterior compartment. It was believed that the impending compartment syndrome occurred due to damage to the surrounding bony and muscular tissue. A medium Hemovac drain was placed along the length of the lateral compartment, exiting in the posterolateral proximal leg. The patient was placed in a hinged knee brace which was locked in extension. He was discharged home later that day.\nOn postoperative day number two, the patient's Hemovac drain was removed by a family member. The patient was seen 1 week postoperatively and noted moderate pain (6/10) and 0% normal function. On physical examination, incisional sites were clean, dry, and intact and a small fracture blister was noted on the posterior aspect of the knee—which was cleaned and redressed. Radiographic imaging revealed well-positioned screws, no evidence of new fractures or foreign bodies, and early evidence of callus formation. Two and a half weeks after surgery, the patient presented to the clinic for evaluation. He reported that he had no pain (0/10) and had 5% of his normal function at this time. On physical examination, he noted no tenderness to palpation of the knee joint, and he had 40 degrees of knee flexion. Anterior-posterior and lateral X-rays were taken which showed evidence of callus formation in the bone (). At this time, it was recommended that the patient begin gentle active range of motion exercises with extension and light flexion. He was also encouraged to become full weight-bearing with the brace until its removal two months postoperatively.\nFive months postoperatively, the patient reported no pain (0/10) and possessed 95% of his normal function at this time. On physical examination, he was nontender to palpation along the joint line. There was no laxity with varus or valgus stress. He demonstrated 5/5 quadriceps strength with no evidence of an extensor lag. He had an active range of motion from 0 to 130 degrees of flexion, and there was no lag with straight leg raise. Repeat anterior-posterior and lateral X-rays demonstrated a well-reduced tibial tubercle fracture as well as well-positioned and nondisplaced hardware (). |
A 55-year-old white British man very fit and well, presented with complaints of central chest tightness. It was non-radiating and lasted about 30 minutes. He usually walks two miles and does frequent cycling and swimming. He has not had chest pain previously and has always been in excellent health. He had a day case surgery for nasal polyp removal one week previously. He did not have any medical problems in the past and was not on any medications. He was a non-smoker. On examination all observations were normal and systematic examination was normal. All blood tests were normal. Electrocardiogram (ECG) and chest X-ray were normal. The Troponin-T test later came back raised at 0.14 micrograms per litre. A diagnosis of NSTEMI (non-ST elevated myocardial infarction) was made and he was started on appropriate treatment. The next day he had an in-patient coronary angiogram, which was normal (). The cardiologist concluded that a diagnosis of myocardial infarction was unlikely in his case, all treatment was stopped and the patient was allowed to go home with no further follow-up.\nFour weeks later the patient again presented, this time with a history of left-sided chest pain. He described a sharp stabbing pain initially in the left rib cage and then in both sides later. Pain was made worse on inspiration, not related to posture or exertion and was only partially relieved with analgesics. He felt short of breath on walking which was unusual for a man of his fitness. On examination his observations were all normal. Systemic examination was normal. ECG and chest X-ray were normal. Arterial blood gases (ABG) showed- pH of 7.45, pCO2 of 4.3, PaO2 of 10.4, bicarbonate of 26 and saturation of 96%. Initially it was thought by the attending physician that the pain may be musculoskeletal. A diagnosis of pulmonary embolism (PE) was considered very unlikely in his case as he had no risk factors and was very fit, but in view of a slightly low PaO2 (though pCO2 was not low as would be expected in PE) for his level of fitness it was decided to do a CT pulmonary angiogram (CTPA) the next day. A troponin level on this occasion was normal but D-dimer came back as positive. No prediction score like Wells or Geneva was used at time of admission to determine the probability of PE. It is likely that even any such score had been used it would have shown a low probability given the absence of any risk factors for thromboembolism. To our surprise the CTPA showed bilateral pulmonary emboli in both the main pulmonary arteries and the segmental branches ( and ). The patient was started on warfarin following initial low molecular weight heparin. Once the INR was therapeutic, the patient was allowed to go home and was advised to continue on warfarin for 6 months. |
We report the case of a 21-year-old woman with cardiac transplant performed in 2007 owing to progressive right ventricular failure following Mustard surgery for transposition of the great arteries. Reviewing the patient's chart, we found no evidence of acute episodes of heart failure associated with regional wall motion abnormalities, suggesting no previous episodes of TS. The patient developed progressive severe systemic right ventricular failure, a known complication after the Mustard palliation surgery. After successful cardiac transplant, the patient was followed up regularly by clinical evaluation, laboratory testing, and echocardiography every 3 months, while coronary angiography and intravascular ultrasound was performed every 12–18 months, according to current guidelines, in order to establish progression of coronary disease (i.e. Stanford score). During follow-up, one hospitalization was reported in 2014 for bacterial pneumonia, while no acute heart failure events and/or cardiac rejection occurred. The last follow-up visit before the reported event was performed in November 2016. Clinical and echocardiographic examination showed optimal clinical conditions, New York Heart Association class I, normal biventricular function with absence of wall motion abnormalities, and no pericardial effusion. Angiography demonstrated mild-to-moderate coronary artery disease (Stanford II) with absence of significant coronary artery stenosis.\nIn mid-January 2017, the patient was admitted to the hospital owing to acute worsening of her clinical conditions characterized by reduced tolerance to activity and fatigue. The day before the onset of symptoms, she reported having an angry debate. Physical examination evidenced fair general clinical conditions, borderline tachycardia with a heart rate of 100 b.p.m., an oxygen saturation of 98%, and a cuff blood pressure of 130/87 mmHg. The electrocardiogram showed right bundle branch block (already present in her previous tracings) with new evidence of negative T waves in all precordial leads (Figure\n). Echocardiography revealed a dilated left ventricle with severely depressed ejection fraction (34%); there was evident asymmetry of regional function with typical akinetic mid-to-apical segments and normally contracting basal segments (Figure\n; ), resembling the traditional Japanese octopus trap (i.e. ‘takotsubo’). Right ventricular function was low to normal (fractional area change of 34%) with no pericardial effusion. Troponin was still normal at admission but turned moderately positive within the following hours, reaching 108 pg/mL (normal range 0–17 pg/mL), with markedly elevated BNP (1780 pg/mL). With the aim of ruling out ischaemic cardiomyopathy and/or acute rejection, epicardial vessels coronary angiography and myocardial biopsy were performed. No evidence of coronary obstruction was found (Figure\n); thus, with acute graft rejection being suspected, patient was started on i.v. milrinone and diuretics, to sustain circulation and prevent the expected development of right ventricular dysfunction, as previously described in acute cardiac rejection patients. After 2 days, cardiac histological examination was available showing no signs of acute rejection. Accordingly, diagnosis of TS cardiomyopathy was made, and the patient was switched to oral therapy with enalapril and furosemide. As expected, within 7 days, heart rate decreased to 80 b.p.m., and echocardiography showed normal left ventricular volume and improved systolic function with 55% ejection fraction, with a significant reduction in apical hypokinesia. After 20 days, follow-up evaluation showed fully recovered systolic function with a 65% ejection fraction and no more apical dysfunction (Figure\n; ); thus, preventive treatment with carvedilol and enalapril was undertaken. At present, patient undergoes regular follow-up visits. No additional acute events have been reported, and she is currently asymptomatic and in good general clinical conditions. |
A 10-year-old female child was admitted to Pediatric Intensive Care Unit through Emergency Department with a history of two to three episodes of massive hemoptysis for the past 2 days. She had such intermittent episode of hemoptysis for the past 2 months. She had no fever, breathlessness, loss of weight, joint pain, or renal symptoms. She had no significant past or family history of tuberculosis. On arrival in Emergency Department, she was in hemorrhagic shock. The patient was resuscitated with fluid, and whole blood transfusion was given.\nHer investigation yielded hemoglobin was 4 gm% with normocytic normochromic type of anemia, an erythrocyte sedimentation rate of 20 mm at end of 1t hour. Her total leukocyte count and differential leukocyte count were within normal limits, while her tuberculin test was negative. Chest radiograph was suggestive of well-circumscribed mass on upper left side with infective changes []. High-resolution computed tomography (CT) thorax revealed infective changes in both lung fields with well-circumscribed mass of size 4 cm × 4 cm × 4 cm on the left main bronchus and extending on lingual lobe suggestive of thymolipoma []. CT-guided fine-needle aspiration cytology shows fat cells. Intraoperatively, there was a mass of size 4 cm × 4 cm × 4 cm in the anterior mediastinum and communicating with left main bronchus and lingual lobe with cheesy puttaceous material coming out of it and adhere to aorta, pericardium, and pleura. Mass was excised, and partial lobectomy was done. Histopathologically, on cut surface, mass was solid grayish white in color with many cysts of size 1 cm × 1 cm × 1 cm filled with brownish friable material and yellowish fatty area. On microscopy, elements of all three germ layer namely epidermis, skin adnexal structure, adipose tissues in sheets and groups, mature cartilage, muscle tissues, and respiratory epithelium suggestive of mature teratoma were seen [Figures and ]. |
A 49-year-old African American female patient with a history of untreated HIV infection presented to the emergency room with a two-week history of left ear pain, headache, fever, and blurred vision. Physical exam was significant for crusted vesicles in the left ear and a vesicular skin rash on the left side of the face consistent with herpes zoster. Ophthalmic examination showed normal visual acuity (VA) in the left eye. VA in the right eye was decreased to 20/100 and an afferent pupillary defect in the right eye was also observed. The fundoscopic examination was unremarkable bilaterally. CSF analysis revealed lymphocytosis consistent with viral meningitis. VZV polymerase chain reaction (PCR) was positive confirming VZV meningitis. She was started on intravenous acyclovir (). MRI of the brain revealed enhancement of the right optic nerve and chiasm consistent with retrobulbar optic neuritis. Laboratory assessment showed a low cluster of differentiation 4 (CD4) count and high HIV viral load consistent with uncontrolled AIDS (). The patient was started on tenofovir, emtricitabine, and boosted darunavir for AIDS management and on high-dose methylprednisolone for optic neuritis. She experienced clinical resolution of her fever and headache. She was discharged on valacyclovir and prednisone taper.\nFour weeks later, the patient presented to the emergency room with sudden painless loss of vision in her left eye. A cerebral angiogram revealed stenosis of the left ophthalmic artery. tPA was administered which resulted in an improvement in her visual acuity from 20/400 to 20/100 in her left eye. She was started on acyclovir and steroids. Fundoscopic examination revealed the progression of the necrosis ultimately involving the whole retina (see ). Fluorescein angiography was consistent with PORN. PCR performed on the vitreal fluid was positive for VZV. Intraocular ganciclovir and IV cidofovir were started to help prevent the progression of PORN. Laser walling was done subsequently to prevent retinal detachment.\nThe patient responded well to the antiretroviral treatment, her CD4 count six months later was 317 cells/cubic milliliter, and her viral load decreased to 50 copies/ml. Her visual acuity after one year showed significant improvement to 20/40 bilaterally. |
A 14-year-old South Asian boy from rural Bengal (India), born of a second degree consanguineous marriage, with normal birth and development history, presented with abnormal brief jerky movements involving his trunk and limbs, with recurrent falls for 10 months. The jerks were neither stimulus sensitive nor present during sleep. No loss of consciousness was reported to occur with these jerky movements. Recurrent convulsions involving the left half of his body, without impairment of awareness, was present for 8 months. It was followed by insidious onset of mild weakness of the left half of his body for 7 months. Subsequently he suffered progressive decline in his general ability to maintain average daily activity independently for 5 months. He had to discontinue schooling because of his failing cognitive functions. For 2 months prior to presenting to us, he developed rapid dance-like movements involving all four limbs that flowed from one muscle to the other in a more or less continuous fashion. Occasionally it would become somewhat flinging particularly in his upper limbs. There was no history of similar illness in the family. He received all the scheduled vaccines as was stated by his mother.\nThe height of the boy was 150 cm and he did not have any dysmorphic facial features. A clinical examination revealed generalized choreiform movements as the most obvious finding. These movements intermittently became flinging in nature, resembling ballism. Generalized myoclonic jerks were seen embedded inside the flurry of chorea-ballism. When he was asked to protrude his tongue, besides motor impersistence, oromandibular dystonia was also found. He had severe dysarthria with apparently preserved comprehension. A limited cognitive assessment revealed reduced attention span as well as short-term memory impairment. Rigidity was obvious in all four limbs along with dystonia in both lower limbs. Weakness in the left half of his body along with brisk reflexes and extensor plantar on left side was also detected on motor system evaluation.\nRoutine laboratory parameters revealed impaired fasting glucose (120 mg/dl), mildly raised liver enzymes and creatine phosphokinase (CPK) level of 820 IU/L. Other blood and urine parameters were within normal limits. Screening investigation for Wilson’s disease, storage disorders, and metabolic disorders were all negative. A routine cerebrospinal fluid (CSF) study was unremarkable and anti-measles antibody was negative. Anti-nuclear antibody in blood was also negative. His serum level of lactate was 36 mg/dl (2–19 mg/dl) while CSF lactate was 42 mg/dl. Shortening of PR interval (0.10 second) was found in electrocardiography. Two-dimensional echocardiography was devoid of any abnormality. Serial brain imaging was done at different centers throughout the course of his illness. On studying his MRI brain images sequentially, a relapsing remitting pattern of lesions was detected. On T2/fluid-attenuated inversion recovery sequence (FLAIR) there were hyperintense lesions that mainly involved subcortical white matter in frontoparietal areas (Fig. ). An area of diffusion restriction was found in the right capsule-ganglionic region (Fig. ) that temporally coincided with the onset of left hemiconvulsions and hemiparesis. Magnetic resonance spectroscopy (MRS), done at our center, showed the presence of lactate peak in brain lesions. Brainstem auditory response revealed bilateral prolonged latency. Electromyography (EMG) showed short duration low-amplitude polyphasic motor unit action potential which was suggestive of myopathic pattern. Spike-wave discharges were observed arising from bilateral frontal areas on electroencephalography (Fig. ). A muscle biopsy, which was done from left vastus lateralis, revealed ragged red fibers (Fig. ), suggestive of mitochondrial failure and deposition of abnormal mitochondria below the plasma membrane of muscle fibers.\nAccording to the clinical criteria, MELAS syndrome was the most probable diagnosis in our case and we needed to confirm the diagnosis. As a facility for analysis of respiratory chain enzymes in the muscle was not available, we decided to search for underlying genetic abnormality in mtDNA. A polymerase chain reaction (PCR) method was employed for this purpose. Amplification of DNA in whole blood sample of our patient was performed for detection of mutations 3243A>G, 3271T>C, and 3251A>G in mitochondrial tRNA leucine 1(MT-TL1), by using appropriate wild type and mutant type specific primers for each and a common reverse primer for all. Genetic analysis result was as following: A>G point mutation at position 3251 of MT-TL1 gene of the mtDNA with heteroplasmy of 70%.\nAfter reaching the diagnosis, valproate was taken off and lamotrigine was introduced. He was put on co-enzyme Q supplement and haloperidol for abnormal movements. Six months into follow-up his seizures and abnormal movements were controlled significantly with slight improvement of cognitive abilities. |
In January 2021, a 2-year-old boy of white Caucasian origin presented to his local ophthalmologist for acute unilateral sixth nerve palsy and was subsequently transferred to our pediatric emergency department for further evaluation. The patient, generally being fit and well, had developed a sudden dysfunction in lateral movement of his left eye, resulting in a continuous abduction deficit with consecutive fixated turn of the head to the left side. His medical history was unremarkable for trauma, headache, vomiting or fever. He had not received any vaccinations within the last few weeks. Apart from a mild gait instability, there were no concomitant symptoms or other focal neurological deficits on clinical examination. The patient did not suffer from any chronic diseases and did not take any regular medication; his vaccination status was complete according to national recommendations.\nThree weeks prior to onset of symptoms the patient had experienced a respiratory tract infection resulting in an increased respiratory rate, dry cough, intermittent fever and loss of appetite, lasting for 2 weeks. Symptomatic treatment was initiated by his local pediatrician, who attributed the patient's symptoms to a common cold rather than COVID-19. Thus, no oropharyngeal swab for SARS-CoV-2 or other viruses was obtained. At the same time, the patient's father and his uncle developed cough, dyspnea, sore throat and muscle aches; the uncle tested positive for SARS-CoV-2 on PCR from oropharyngeal swab (). The child's uncle does not live in the same household but had been in close contact to the patient 4 days prior to his positive test for several hours due to an indoor-birthday party. The patient's relatives were unvaccinated as at that time the COVID-19 vaccines were still unavailable for the general public.\nOn admission, laboratory inflammatory markers including C-reactive protein were negative. Full blood count showed mild thrombocytopenia (186 109/) but was unremarkable otherwise. Cranial contrast-enhanced magnetic resonance imaging (MRI) showed an hypoplastic left abducens nerve and atrophy of the corresponding left lateral rectus muscle compared to the contralateral side (). There were no signs suggesting any inflammatory intracranial process or elevated intracranial pressure, no papilledema. A lumbar puncture was performed. The cerebrospinal fluid (CSF) opening pressure was 24 cmH2O corresponding to the upper limit of normal range () thus diagnostic lumbar puncture was followed by therapeutic drainage of 8 ml CSF. Routine CSF laboratory parameters yielded a normal result; no oligoclonal bands were detected on CSF/serum. Multiplex-PCR (Filmarray, BioFire, Biomerieux Lyon, France) from CSF was negative for cytomegalovirus (CMV), enterovirus, herpes simplex viruses 1 and 2, human herpesvirus 6, human parechovirus, varicella zoster virus, Cryptococcus neoformans and gattii, E. coli K1, Haemophilus influenzae, Listeria monocytogenes, Neisseria meningitides as well as Streptococcus agalactiae and pneumoniae. An additional multiplex-PCR performed on an oropharyngeal swab sample yielded a negative result for adenovirus, coronaviruses 229E, HKU1, NL63 and OC43, human metapneumovirus, human rhino-/ enterovirus, influenza virus A and B, Middle East Respiratory Syndrome Coronavirus (MERS-CoV), SARS-CoV-2, parainfluenza virus 1–4, respiratory syncytial virus, Bordetella pertussis, Bordetella parapertusssis, Chlamydophila pneumonia and Mycoplasma pneumoniae. Testing for Borrelia burgdorferi showed no antibodies in neither serum nor CSF. An EEG was unremarkable. Repeated ophthalmologic examinations revealed incomitant squint angles due to left-sided sixth nerve palsy and a significant abduction deficit of the left eye, consistent with the diagnosis of left abducens nerve palsy. An underlying retraction syndrome was considered unlikely due to the sudden onset of symptoms and absent globe retraction. Optic nerve examination was unremarkable.\nReal-time reverse transcriptase PCR (rRT-PCR) test for SARS-CoV-2 (oropharyngeal swab sample) was negative on admission, while serology turned out to be positive for SARS-CoV-2 anti-spike IgG (Euroimmune, Germany). Of particular note in this context, SARS-CoV-2 specific IgG was also detected in CSF. Pathogen-specific antibody index as an indicator for potential intrathecal antibody production was negative, suggesting involvement of central nervous system being secondary to systemic infection rather than direct viral infection (). An rRT-PCR for SARS-CoV-2 from CSF was negative.\nGiven the boy's history of recent respiratory tract infection, COVID-19 very likely in his father and proven in his uncle, and detection of SARS-CoV-2-IgG antibodies in the patient's serum and CSF, post-infectious abducens nerve palsy appeared to be the most likely diagnosis. During inpatient stay, symptoms already showed spontaneous mild improvement without therapeutic measures. Following discharge, the boy was regularly seen for ophthalmologic follow-ups. Three months following onset of abducens nerve palsy, the family noticed a distinct improvement in eye movement and the child eventually made a full recovery 2 weeks later. |
An 18-years old male was referred by his general dental practitioner to the Department of Oral and Maxillofacial Surgery, Aalborg University Hospital, Denmark, due to a progressive asymptomatic impairment of mouth opening interfering with eating, speaking, and maintaining oral hygiene. The patient´s medical history was unremarkable and there was no history of facial trauma or surgery. The patient had previously undergone orthodontic treatment.\nOn physical examination, the temporalis and masseter muscles were slightly tender on palpation, but no masticatory muscle pain and temporomandibular joint pain or sounds were observed. The maximal interincisal mouth opening was 22 mm with lateral movements to the right and left of 5 mm and 6 mm, respectively (Fig. ). Attempts to increase mouth opening by manipulation was impossible. Intraoral clinical examination revealed intact dental arches and an Angle class I occlusion. A panoramic radiography showed bilateral enlargement of the mandibular coronoid process projecting into the infratemporal fossa (Fig. ). Cone beam computed tomography with three-dimensional recons-truction revealed bilateral elongated mandibular coronoid processes impinging upon the zygomatic bone during mouth opening (Fig. ). On the basis of the clinical and radiographic findings, a working diagnosis of MCPH was made. Due to the restricted mouth opening and impingement of the coronoid processes with the zygomatic arches, the decision was made to perform bilateral coronoidectomy.\nThe surgical procedure was conducted in general anaesthesia with nasotracheal intubation, supplemented by local anaesthesia. An intraoral incision, similar to the surgical approach for a sagittal split osteotomy, provided the necessary access to the coronoid processes. The periosteum and the masseter muscle were elevated over the ascending ramus. The insertion of the temporal muscles was released of the temporalis attachment on the anterior border of the mandibular ramus and the tendon attachment was cut from the coronoid process. A channel retractor was placed into the sigmoid notch and a ramus clamp was secured over the coronoid process before a low coronoidectomy was performed from the sigmoid notch to the anterior oblique ridge with a reciprocating saw (Fig. ). The coronoid process on both sides was removed with forceps and surgical recontouring of the mandibular ramus was performed with a drill (Fig. ). The wound was irrigated with saline and closed with resorbable continuous sutures. The interincisal mouth opening increased to 42 mm in the operation room (Fig. ). The patient was discharged later the same day. Healing was uneventful. Histopathologic examination of the resected coronoid process revealed normal bone, confirming the diagnosis of primary bony hyperplasia of the coronoid processes.\nPost-operative panoramic and three-dimensional CT demonstrated successful bilateral coronoidectomy with no interferences between the mandible and zygomatic bone during mouth opening (Figs. and ). Vigorous physical therapy was initiated one week after surgery using TheraBite Jaw Motion Rehabilitation System. However, the patient was not particularly motivated for physiotherapy due to pain and a tightening sensation in the cheek. Three months after the operation, a satisfactory mouth opening of 32 mm was achieved (Fig. ). |
A 6 year old, 33 kg female spayed Wheaten Terrier mix presented to a local veterinary clinic due to severe lethargy and weakness after vomiting a large amount of clear fluid. No prior health issues were reported; however, the dog had been less energetic for a week prior to presentation, according to the owner. The physical examination at that time identified tachycardia and muffled heart sounds during thoracic auscultation. Thoracic radiographs revealed an enlarged cardiac silhouette and an ultrasound examination showed pericardial effusion. Pericardiocentesis was performed and 110 mL of hemorrhagic pericardial fluid was removed. The patient received intravenous fluid therapy and was referred to the small animal hospital at the Atlantic Veterinary College (AVC) for further evaluation.\nOn initial evaluation at the AVC, the patient was lethargic but ambulatory. Physical examination revealed muffled heart sounds, mild tachycardia and weak femoral pulses. The dog was slightly dyspneic but not in distress. No accurate systolic blood pressure measurement could be obtained on initial presentation using Doppler ultrasonography, likely due to peripheral vasoconstriction and/or hypotension. A complete two-dimensional, M-mode and Doppler echocardiogram revealed moderate anechoic pericardial effusion and a broad-based, 3.3 × 4.6 cm mass of mixed echogenicity involving most of the lateral wall of the right auricle. Diastolic collapse of the right atrium was present, consistent with cardiac tamponade. M-mode measurements of the left ventricle obtained from the right-parasternal short axis view were most consistent with concentric pseudohypertrophy secondary to cardiac tamponade and ventricular underloading. No evidence of distant metastasis was noted in three-view thoracic radiographs and abdominal ultrasound examination. Due to recurrence of pericardial effusion and the location of the right auricular mass, the owner was informed of treatment options and attendant advantages and drawbacks; the owner requested surgical excision of the mass.\nA right lateral thoracotomy was performed in routine manner through the fourth intercostal space with the patient under general anesthesia. The pericardium was incised along its craniocaudal aspect ventral to the right phrenic nerve and a pericardial basket was created by suturing it to the thoracotomy incision. A 4 cm diameter mass was palpated in the right auricle and Satinsky vascular forceps were placed as close to the right atrium as possible to isolate the right auricle with the widest possible margin. The mass was excised and the auriculectomy incision was closed with three mattress sutures with pledgets using 4-0 polypropylene over-sewn with a simple continuous pattern using 3-0 polypropylene. Five small dark round masses ranging from 5 mm to 12 mm in diameter were adhered to the visceral surface of the parietal pericardium and were suspected to represent locoregional metastasis. They were carefully dissected with tenotomy scissors after the owner was notified of their presence and again provided consent. No other lesions were seen in the other areas of the pericardium. The pericardium was loosely apposed and sutured in a simple interrupted suture pattern using 3-0 polydioxanone. The right thoracotomy was closed in a routine fashion after a 24 Fr thoracic tube had been placed through the seventh intercostal space. Recovery from anesthesia was uneventful. The dog was hospitalized in the intensive care unit with analgesia (Fentanyl IV CRI 1–3 µg/kg/h, tramadol 3 mg/kg orally every 12 h, and meloxicam 0.1 mg/kg orally every 24 h) and supportive care. The dog experienced mild aspiration pneumonia on the fifth day postoperatively and was discharged with oral antibiotics (amoxicillin/clavulanic acid, 13.75 mg/kg every 12 h) 7 days after surgery.\nHistopathological examination of the right auricular mass revealed locally invasive infiltrates of plump spindloid cells which formed small blood-filled channels and/or coalescing sieve-like clefts supported by small amounts of a fine collagenous stroma. These infiltrates multifocally breached the wall of the atrium and invaded the adjacent pericardial fat. The tumor cells had round to oval nuclei with finely stippled chromatin, often with a prominent nucleolus, and had small amounts of poorly defined cytoplasm. These findings were consistent with hemangiosarcoma. Anisokaryosis was moderate. Mitotic figures were 1–2 per high power field. Neoplastic infiltrates extended to all tissue margins. Sections of the pericardial masses also consisted of large areas of hemorrhage, occasionally interspersed with variably sized infiltrates of the tumor cells seen in the right auricular mass ().\nFifteen days postoperatively, intravenous injectable chemotherapy was initiated with doxorubicin 30 mg/m2. This protocol was repeated every 3 weeks for five treatments with repeat staging consisting of a complete blood count and serum biochemistry profile along with three-view thoracic radiographs, a complete abdominal ultrasound and echocardiographic evaluations at the time of the third doxorubicin treatment. No sign of metastasis was noted in the thoracic radiographs, abdominal ultrasound examination, or echocardiogram. The dog experienced grade 3 gastrointestinal adverse events [] 3 days after the first treatment, which resolved with supportive care without the need for hospitalization. Maropitant (Cerenia, Pfizer Animal Healthm Parsipany-Troy Hills, NJ; 1 mg/kg subcutaneously once followed by 2 mg/kg orally every 24 h for 4 days) was administered with each doxorubicin administration and the dog tolerated all remaining treatments without notable signs of toxicosis. After five treatments with intravenous doxorubicin (118 days postoperatively), metronomic chemotherapy was initiated with piroxicam (Novo-Piroxicam, Novopharm Ltd. Stouffville, Whitchurch-Stouffville, Canada; 0.3 mg/kg orally every 24 h) and cyclophosphamide (Procytox; Baxter Corporation, Mississauga, Canada; 20 mg/m2 orally every 24 h). Concurrent treatment with a polysaccharopeptide bioactive agent from the mushroom Coriolus versicolora (I’m Yunity®, Integrated Chinese Medicine Holdings, Ltd. HongKong, China, 100 mg/kg orally every 24 h) was administered starting 21 days postoperatively and continued lifelong. No abnormalities were noted in physical examinations and imaging diagnostics until the 229th postoperative day, at which time multiple soft tissue opacities were visible on thoracic radiographs, representing suspected pulmonary metastases.\nThe dog presented to the AVC emergency service with a week’s history of lethargy and vomiting, as well as hematochezia for 2 days prior to presentation (318 days postoperatively). The dog was tachypneic, dyspneic, and recumbent on presentation. Progressive pulmonary nodules were radiographically present. Given the likelihood of progressive metastatic malignant neoplasia despite treatment, the owner elected humane euthanasia. A complete necropsy was declined by the owner, but the heart and a part of the lungs were examined grossly with the owner’s permission. The heart and lungs showed multiple dark red, firm, infiltrated nodular masses ranging in size from 1 mm to 3 cm. The pericardial sac was distended with serosanguineous fluid and a large, 12–15 cm, firm, red, multi-lobulated mass extended from the heart base involving the right atrium and pericardium (). |
A 75-year-old female with a history of stage IIIB squamous cell cancer of the cervix, which had been treated and in remission for about 2 years, presented in February 2016 with several weeks of decreased coordination and decreased balance with weakness and clumsiness noted especially on her left side in addition to a left facial droop. Magnetic resonance imaging (MRI) of her brain showed a solitary 4.6 cm × 3.4 cm × 4.1 cm heterogeneous solid mass at the right temporo-parietal junction with surrounding edema, mass effect, and early uncal herniation suggestive of either a metastasis or high-grade primary lesion [Figure and ]. Computed tomography (CT) of her abdomen and pelvis did not show any primary or metastatic lesion. The patient received dexamethasone, which improved her symptoms, and then underwent surgical resection of the tumor in March 2016. Histopathological examination of the resected tumor revealed an epithelial neoplasm with squamous differentiation and extensive keratinization. The tumor cells displayed considerable anaplasia, and mitoses were numerous [Figure and ]. There was a sharp demarcation between the tumor tissue and the surrounding compressed cerebral parenchyma, which showed gliosis and nerve fiber degeneration []. Immunohistochemical stains revealed strong positivity for cytokeratin (CK) 7 and CK5/6 [Figure and ], and also immunopositive for human papilloma virus (HPV), which was confirmed by in situ hybridization for HPV [].\nPostoperative MRI [Figure and ] of her brain showed gross total resection of the lesion. The patient experienced no neurological complications postoperatively and was recovering well at the time of discharge. In April 2016, a positron emission tomography (PET)/CT scan of the patient's head, neck, chest, abdomen, and pelvis showed no evidence of recurrent or metastatic disease. The patient had a repeat MRI [Figure and ] of her head in April 2016, which showed no evidence of tumor progression and significantly improved edema around the resection area. Clinically, she was back to independent living without any neurological deficits. She was subsequently treated with stereotactic radiosurgery (SRS) to the resection cavity with a dose of 18 Gy to the 50% isodose curve.\nIn July 2016, the patient had a left-sided focal clonic seizure and an episode of left-sided weakness. An MRI showed a new single metastatic tumor measuring 2.3 × 3.5 cm2 noted in the right temporo-parietal area with significant surrounding edema within temporal lobe and extending into right parietal and occipital lobes [Figure –]. Given her excellent performance status and only solitary recurrence, she underwent resection of this second metastatic lesion in July 2016 with a postoperative MRI that showed successful tumor resection with residual edema causing minimal left midline shift [Figure and ], and another treatment of SRS to the resection cavity in August 2016. Another PET/CT scan of her head, neck, chest, abdomen, and pelvis was obtained; it showed small bilateral lung nodules in the right middle lobe and ligula likely of inflammatory origin but still concerning of metastases. Given the size and the imaging characteristics of the lesions, decision was made not to biopsy the lesion and obtaining repeat imaging in 6 months that reported stable nodules with no signs of progression; therefore these lesions were unlikely to be metastases. Serial repeat MRIs showed no evidence of disease progression and clinically she remained independent without any neurological symptoms. The plan for the patient is continued monitoring symptoms along with repeat MRI every 3 months. |
A 37 years-old unmarried premenopausal female presented in the emergency of our hospital with complaints of bleeding from ulcerative lesion in the left breast for a couple of hours. The patient had history of ulcer in the left breast for past 2–3 weeks which developed over the underlying huge breast lump. The lump was present for past 5 months but rapidly grew over the last 3 months to current size. There was no history of coagulopathy, trauma, chronic liver disease or history of similar illness in first degree relatives. The patient attained menarche at the age of 15 years. She is a non-smoker and denies history of contraceptive use or alcohol intake.\nOn examination in our emergency there was severe pallor with tachycardia. However the blood pressure was maintained. There was an oozing ulceration in upper outer quadrant of the left breast with underlying huge lump measuring 15 × 15 cm occupying the upper outer, lower outer and upper inner quadrants with deformed shape of the breast (a). The skin surrounding the ulcer was erythematous and edematous. Axilla did not show lymphadenopathy.\nBefore the development of breast lump, the patient had itchy lesion at the same site. The patient unfortunately attributed all these events to that itchy lesion and sought help late. There was no history of headache, shortness of breath, abdominal distension or bone pain.\nOn investigating the patient, she was severely anemic with hemoglobin of 5 gm% with normal total and differential counts. Coagulation parameters and liver function tests were normal. Chest X-ray showed no evidence of metastasis other than dense breast shadow on left side due to huge breast lump(a).\nMammogram revealed homogeneous radiodense lobulated mass occupying whole of left breast with subcutaneous thickening on the background of dense breast (b). Ultrasound (USG) of abdomen and pelvis did not reveal any abnormal findings. Trucut biopsy from the breast mass showed necrotic areas with fibrocollagenous tissue with proliferation of oval to elongated spindle cells showing mild degree of atypia.\nAfter discussion about the possibility of metaplastic versus malignant phyllodes tumor, the patient underwent modified radical mastectomy (MRM). Histopathological examination showed maximum tumor size of 14 cm with increased stromal cellularity, loss of stromal-epithelial balance and frequent mitoses more than 45 per 10 high power fields () typical of malignant phyllodes. All the resected margins, nipple and areola were free of tumor. Lymphovascular and perineural invasions were not identified. Seventeen axillary nodes retrieved were free of tumor.\nPostoperatively patient gained weight and was doing well. Three months after surgery, the patient started developing progressively increasing headache, nausea and vomiting for which she got admitted in our center. There was no local recurrence. However on further investigations, she had a huge cystic lesion with mural nodule in her brain (b,c,d). Contrast enhanced computed tomography (CECT) of chest, abdomen and pelvis showed lesions in lower lobe of left lung (a) of size 3.9 × 3.6 cm of +40 Hounsfield unit (HU) in posterobasal segment and right adrenal gland () of 5.8 × 5.1 cm of +30HU with significant enhancement in post-contrast images. Functional evaluation for the adrenal mass with urinary metanephrines and serum cortisol was negative. Patient refused biopsy from right adrenal gland and lung, but while preparing for burrhole biopsy from brain, patient succumbed to the disease. The patient underwent serial ultrasound of abdomen and pelvis during hospital stay which showed increase in size of 4 cm of adrenal mass in 15 days (tumor velocity). |
Mr. N, a 26-year-old farmer, was brought with a one-day history of loss of strength in his lower limbs. The previous day, hostile farmers from a nearby village had raided his fields and destroyed his crops while his family was away; instead of defending his land, he had hidden in his house. When his family returned, he was discovered lying in his bed, unable to move his lower limbs.\nOn examination, he was found to have no apparent power in any muscle group in his lower limbs. Superficial and deep reflexes, however, were normal. A diagnosis of conversion disorder with motor deficit (DSM-IV 300.11) was made. Over the subsequent week, attempts were made to provide insight into the nature and cause of the symptom, and to effect symptom removal through the use of reassurance, placebo, suggestion, electrical stimulation of the muscles of the lower limbs, removal of secondary gain, and other methods; none proved successful.\nSystematic enhancement of functioning was then attempted. He was encouraged to move the great toe on one foot, and was lavishly praised when, after much time and effort, a flicker of movement was elicited. This movement was practiced until he was able to move the toe consistently. He was then encouraged to move the great toe of the other foot. Afterwards, he was encouraged to dorsiflex and plantarflex each foot, then swing his legs at the knees as they dangled freely over the edge of a chair, and so on, until he was able to perform most movements of most muscle groups in the lower limbs.\nHe was subsequently encouraged to walk. Initially, he was only able to make stepping movements while supported by a ward boy on each side; however, he gradually progressed to weight-bearing with support from the ward staff, and then weight-bearing with support from furniture and railings. He eventually walked on his own, hesitantly, but without support. Successful restitution of functioning through systematic enhancement took about 2 weeks. |
An 81-year-old Asian man who was hit by a car while riding on a bike was transferred to our emergency room. He had no neurological symptoms except numbness in the right C6 dermatome area. Computed tomography (CT) of his cervical spine showed a right minimally displaced facet fracture of C6. The fracture fragment size was 6 mm, involving 25% of the height of the intact lateral mass (Fig. ). CT angiography (CTA) at initial survey revealed a right blunt VAI, which was overlooked at initial admission (Fig. ). CTA was used because it is integrated into a whole-body CT protocol for patients with high energy or multiple traumas. His blood data showed no abnormal findings of coagulation or platelet count. The fracture fragment was small, and the fracture was considered stable and the patient was treated with a Philadelphia collar and discharged four days after admission. We ordered magnetic resonance imaging (MRI); however, the patient could not undergo MRI during his first hospital stay. In addition to the MRI reservation being full, an MRI was deemed less urgent as he had no motor deficit. A visit to the outpatient department, including an MRI, was scheduled nine days after injury. However, 9 days after the injury, he developed quadriplegia gradually and was re-admitted to the hospital. He presented with complete paralysis of the lower extremity and bilateral motor weakness of the upper extremity including the elbow flexors and extensors, wrist extensors, and the finger abductors and flexors. Muscular power was graded as 0/5 to 4/5 by manual muscle testing (MMT). His neurological level of injury (NLI) was C4, and American Spinal Injury Association (ASIA) Impairment Scale was A. MRI at re-admission showed a disc injury at C5/6 with spinal cord compression from a posterior epidural mass accompanied by intramedullary signal intensity changes at the same level (Fig. ). Immediately after admission we surgically performed a mid-splitting laminoplasty with instrumented fusion of C5–6 using lateral mass screws (Fig. ). At surgery, fibrous scar tissue compressing the posterior aspect of the cord was observed and removed. We consulted a neurosurgeon about the blunt VAI, and the patient underwent endovascular stenting. At four months after surgery motor function in his upper extremities had improved and he was able to eat by himself using an assistive device but motor loss persisted in both of his legs. |
A 67-year-old man who was diagnosed with osteopetrosis more than 20 years ago was admitted to our hospital for systematic treatment on 25 August 2014. Over 1 month ago, persistent pain emerged with no obvious injury in his left hip but did not influence his walk. Thus, he not only ignored this symptom but also rejected any treatment. After suffering from aggravating pain, he was unable to ambulate and sought medical advice in the hospital. Before this incident, 4 femoral fractures occurred respectively in 1986, 1993, 2002, and 2011, which explicated in Table . The patient denied both consanguineous marriage on his parental side and familial genetic disease. None of his family members (3 paternal and 2 maternal uncles, 2 sisters, and his daughter) had a condition as this patient.\nThe physical examination revealed that tumefaction appeared in his left hip and restricted his range of motion; additionally, pressing pain existed in the upper part of the left thigh. The lever force disappeared and deformity appeared during adduction and abduction. It was no distinct tenderness in the right hip and knee, but restriction of movement was the same as the left hip within the angle ranging from 0 to 30 degrees. In addition, lower extremities had unequal length in appearance.\nA radiological examination was carried out simultaneously through x-ray check. Radiographs proved that bones had calcified variously in pelvis, diaphysis, metaphysis, which characteristically identified osteopetrosis. Besides, a left subtrochanteric femoral fracture was observed in the film, along with a bilateral fracture formerly treated by internal fixation. In the meantime, we found that overlap extent of fracture was about 3 centimeters that surrounded some callus. (Fig. A–C). It was in sore need of reduction for femoral subtrochanteric fracture.\nBiochemical examination parameters were illustrated in Table , in which could find out abnormity of hepatic and hematopoietic function. Osteopetrosis could be further confirmed by hematological indices about liver, hemocytes, and bone calcium metabolism. Excessively high alkaline phosphatase (ALP) implies that osteal diseases have been progressing. However, low red blood cell count (RBC) and red blood cell distribution width (RDW), as well as decreased hemoglobin (Hb), mainly relate to anemia that probably is an accompanying sign with osteopetrosis. Besides, hepatosplenomegaly sometimes seen in the disease could arouse increase in alanine aminotransferase (ALT), aspartic acid transaminase (AST), and γ-glutamyltransferase ggt (γ-GGT). For comparison easily, we list associated data in Table .\nOn 28 August 2014, ORIF (with LISS plate-screw fixation) was conducted for this patient. Epidural anesthesia was chosen for the subject during surgery. As expected, it was a great quantity of callus surrounding the fracture and seemed to be tough to resume their position ascribed to soft tissue contracture and malunion. As a result, we took amputation by 3 centimeters in the distal part of fracture ends as planned, which made fragments return to appropriate position smoothly and equilibrated length between lower extremities. As to fixing the fracture, LISS plate partly covered on the previous plate after removing 2 screws from the former fixture (Fig. ). In spite of one drill bit unavoidably broke in the process of digging cannelure, the operation completed ultimately. After this surgery, cephalosporin was appropriately used to prevent infection. Since the date he was discharged from the hospital, a long-term follow-up had been implemented with return visit every 2 weeks and 1, 3, 6, and 12 months. During a 12-month nursing, the patient is capable of walking and bearing weight without pain. Moreover, radiographs displayed as below also reflected recovery stage of the fracture (Fig. D–F). Unfortunately, we eventually lost contact with the patient when an inspection was carried on the 13th month. |
A 26-year-old previously healthy female sought medical attention in January 2015 with a 2-month history of weight gain, facial acne, and hirsutism. She was seen by an endocrinologist who obtained a 24-h urinary cortisol at 483 mcg/mL (normal range: 10–100 mcg/mL). Computed tomography (CT) scan of the thorax and abdomen revealed multiple lobulated pulmonary nodules of soft tissue density measuring between 5 and 15 mm as well as a solid heterogeneous mass in the left adrenal gland measuring 14 × 8 × 7 cm, with dystrophic calcifications, contrast enhancement, central area of necrosis, and extension into the left adrenal vein. The lesion was in contact with the superior kidney pole, displacing it inferiorly, but without signs of invasion. There were three nodular foci in the liver compatible with hemangiomas. Magnetic resonance imaging (MRI) of the abdomen confirmed a solid lobulated mass in the left adrenal, measuring 14 × 9 × 7 cm, with high signal on T2 sequences and heterogeneous contrast enhancement. There was invasion into the left adrenal vein with associated thrombosis. On 28 January 2015, left adrenalectomy with en bloc radical nephrectomy and adrenal vein tumor thrombus excision was performed. Postoperative recovery was uneventful. Histopathologic exam confirmed a 13 × 10 × 5 cm ACC with solid and diffuse architecture pattern, high nuclear grade, mitotic rate of more than 15 per 10 high power fields with atypical mitosis, extensive necrosis, clear cell component of 25%, the presence of capsular invasion, and neoplastic invasion into the left adrenal vein. Immunohistochemistry showed positivity for alpha-inhibin and synaptophysin, as well as a Ki-67 of 40% (). The patient was first seen at our Oncology Center in February 2015. Due to the high mitotic count and Ki-67, she was started on mitotane combined with chemotherapy (doxorubicin, cisplatin, and etoposide). After three cycles, a positron emission tomography-CT (PET-CT) scan demonstrated stable disease. However, due to the side effects (especially nausea, vomiting, malaise, and alopecia), the patient decided to stop chemotherapy.\nTo explore additional treatment options, the patient travelled to California and underwent a surgical biopsy of a peripheral lung nodule that provided viable tissue for the ex vivo analysis of programmed cell death (EVA/PCD). This phenotypic platform provides drug response profiles that have been shown to correlate significantly with response, time to progression, and survival. The platform has been the subject of prior review with methods described in previous publications. In summary, sterile surgical specimens are submitted from the operating room in modified RPMI 1640 media. Following mechanical and enzymatic disaggregation, micro-spheroids of desired size (50–70 cells) are isolated by Ficoll-Hypaque density gradient. Cell suspensions are washed twice and adjusted to desired cell density with viability determined by initial Trypan blue. A volume of 90 µL of cell suspensions are then distributed into 96-well plates with cytotoxic agents distributed as 10 µL aliquots with continuous drug exposures for 72 h. Cell death events are examined using various endpoints including adenosine triphosphate (ATP) content by luciferase, mitochondrial metabolism by 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT), caspase activation, and delayed loss of membrane integrity measured by staining and morphology. As metformin actions are metabolic in nature, we utilize the membrane integrity endpoint to measure its activity to prevent possible artifacts being introduced by non-lethal perturbations in mitochondrial signal.\nPhenformin had previously been selected in our platform as the index biguanide. Active concentration ranges (500–31 µΜ) have been determined in training sets and calibrated to provide clinically relevant signals in over 350 prior phenformin human tumor analyses. Five-point dose–response curves, in duplicate, were averaged and interpolated to provide the patient LC50 value of 39.7 µΜ. This fell in the most sensitive range for phenformin as determined by modified Z-score. The results also identified activity for the cyclin-dependent kinase 4/6 inhibitor, palbociclib, and the phosphoinositol α-specific kinase inhibitor BYL-719 with moderate activity for everolimus and sunitinib. Phenformin was included in this patient’s tumor as a comparator molecule in ongoing research being conducted at the time on a structurally unrelated inhibitor of mitochondrial complex I (unreported observation). Based upon this favorable finding with phenformin, the structurally similar and clinically available biguanide, metformin, was recommended.\nIn June 2015, the patient was started on oral metformin at 500 mg twice daily and continued on mitotane. After 3 months, a repeat CT scan of the thorax showed marked reduction in the size of the dominant nodules, with complete disappearance of several smaller nodules ( and ). The measurable response continued for approximately 9 months.\nWith her family history positive for a retroperitoneal sarcoma (mother) and a lung cancer (grandmother), the patient was referred for genetic counseling and tested positive for LFS as a pathogenic mutation was found in TP53: c.1010G>A (p.Arg337His) (het). Exon codons and the exon–intron boundaries of the CHEK2 (22q12.1) and TP53 (17p13.1) genes were amplified and processed for next-generation sequencing (NGS). The sequencing results were analyzed in the Variant Caller software (Ion Torrent; Life Technologies) and compared with the version of the GRCh37/HG19 genome. The nomenclature of the detected mutations was made following the recommendations of HUman Genome Organization (HUGO) and Human Genome Variation Society (HGVS). The reference sequences of the detected genes were NM_000546.5 (TP53) and NM_007194.3 (CHEK2). In this analysis, a pathogenic mutation was detected according to the information deposited in the consulted databases (ClinVar, dbSNP, and/or IARC LOVD).\nWritten informed consent for patient information and images was obtained from the patient before publication. Hospital Alemão Oswaldo Cruz does not require ethical approval for reporting individual cases or case series. |
A 69-year-old male patient presented with the chief complain of prominence of the left eye associated with periocular swelling, redness, and gross diminution of vision of 1 month duration.\nHe was a known case of primary open angle glaucoma on anti-glaucoma medication. Trabeculectomy in the left eye was performed, after which his symptoms developed on post-operative day 3. He was diagnosed to have hemorrhagic choroidal detachment and was treated with steroids with little benefit. He was a known case of diabetics and hypertension on regular medical therapy.\nOn examination, his best corrected visual acuity was 6/9 and N6 in the right eye and no light perception (PL negative) in the left eye. Extraocular movements were full and free in the right eye but were limited in all directions of gazes in the left eye. There was periorbital edema and near total ptosis in the left eye. Hertels exophthalmometry revealed reading of 21 mm in the right and 30 mm in the left eye, with a base reading of 110.\nSlit lamp examination of the right eye was normal except for the early cataractous changes in the lens. In the left eye, there was conjunctival congestion, chemosis associated with dilated episcleral vessels. Anterior chamber was flat with total cataract. Intraocular pressure in OD was 24 mm of Hg and 30 mm of Hg in the left eye by Goldman Applanation Tonometry. Fundus examination revealed a vertical cup: disc ratio (C:D ratio) to be 0.7 with neuroretinal rim thinning in the right eye with the rest of the fundus being normal. No view of the fundus could be obtained in the left eye. USG-B scan was done which revealed thickened extraocular muscles with optic nerve head cupping, shallow 360 degree choroidal detachment with no evidence of mass lesion, and a dilated superior ophthalmic vein on the left side (Figure 1 ).\nBased on the clinical signs, symptoms, and initial investigations, carotid cavernous fistula was suspected and further investigated. A MRI was performed which showed prominent left cavernous sinus with abnormal flow with early opacification of the left superior ophthalmic vein on dynamic contrast and enhanced MR angiogram. A four vessel angiogram revealed type D CCF (Figure 2 ).\nVisual evoked potential was done which revealed normal P100 amplitude and latency in OD and grossly diminished P100 amplitude and latency in OS (Figure 3 ). Perimetry was performed in OD to document the visual field loss.\nIn view of nil visual prognosis in OS, he was advised conservative management and for OD was prescribed anti-glaucoma medication and regular follow-up in the glaucoma clinic. He was referred to an interventional radiologist for opinion and further management as required. The patient subsequently was lost to follow-up. |
A 35-year-old female Caucasian patient palpated a mass in her right breast in February 2011. Staging examinations revealed a T1c primary tumor, axillary lymph node metastases, and multiple liver metastases up to 45 mm throughout the liver (Figure ).\nHistology confirmed poorly differentiated invasive ductal carcinoma, ER and PR negative, HER2 positive, and the patient was negative for germline BRCA 1 and 2 mutations. The serum tumor marker CA 15-3 was elevated to 620 kIE/l. Emerging biomarkers such as circulating tumor cells were not assessed. The patient was otherwise healthy and in excellent performance status. First-line systemic treatment with weekly docetaxel and trastuzumab was initiated in March. Treatment with docetaxel was stopped in August because of peripheral neuropathy. All sites of disease were in complete clinical remission at that time. CA 15-3 was no longer elevated (10 kIE/l). The patient continued on trastuzumab, now every three weeks. In September 2012, after increasing problems with headaches, multiple supra- and infratentorial parenchymal brain metastases were diagnosed. Computed tomography revealed six lesions with a maximum diameter of 40 mm of solid or cystic appearance (Figure ).\nNo other active sites of disease were present. CA 15-3 was still normal. The patient's performance status was excellent. Whole-brain radiotherapy was initiated (10 fractions of 3 Gy). Trastuzumab was continued. When evaluated for delayed radiosurgery to residual brain metastases, all lesions had resolved completely. The clinical course was unremarkable until May 2016 when mammography showed recurrent tumor in her right breast (after 86 courses of trastuzumab), again without accompanying increase in CA 15-3. No lymph node or other metastases were detected. A mastectomy was performed and the histology was identical to the initial findings from 2011. Postoperatively, systemic therapy was changed to paclitaxel, trastuzumab, and pertuzumab. The patient is currently still on active treatment with this regime (last follow-up March 06, 2017). No serious cardio- or neurotoxicity has occurred. |
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 17-year-old man was involved in a road accident in which he suffered the open fractures of the right femur and tibia. At the arrival to the Emergency Dept (ED), he was alert and hemodynamically stable and the Glasgow Coma Scale (GCS) was 15; the initial alignment of the fractured ends was performed in the ED with a gentle traction performed under sedation with iv. ketamine; a total body CT did not demonstrate other injuries. Approximately two hours after the admission the patient was taken to the surgical theatre for the external fixation of the fractured bones; at entering the operating room, the GCS was 8, the arterial pressure was 115/80 mm Hg, the heart rate was 115 bpm, and the arterial oxygen saturation (SPO2) was 85 at room air; the procedure was performed under general iv anesthesia with propofol and remifentanyl; the standard monitoring included the ECG, the noninvasive arterial pressure, the SPO2, and the end-tidal CO2 (ETCO2); during the intervention, the SPO2 rose to 100% at a FIO2=40% and all the other variables remained stable throughout the procedure after the 3-hour-long intervention in which the complete alignment of the bony ends was achieved; the patient was transferred to the Intensive Care Unit (ICU) still intubated and mechanically ventilated; the iv anaesthetics were gradually tapered until the complete suspension. Two hours later, the SpaO2 and the ETCO2 slightly decreased and anisocoria was observed; and an urgent CT scan of the head demonstrated a diffuse cerebral edema and the herniation of the cerebellar tonsils (Figures and , respectively). At this time, the pupils became bilaterally mydriatic and the EEG was almost isoelectric; due to the severity of the conditions, a MR scan was considered unnecessary. On the basis of the clinical and radiologic findings repeated boluses of iv. mannitol and steroids were given in the following hours aiming to reduce the intracranial pressure. An echocardiogram demonstrated a severe right ventricular depression with an ejection fraction of 20%. On the following day, the patient was declared brain dead according to the current Italian law.\nAt the autopsy, the cerebral microvascular network appeared diffusely plugged with BME (Figures –) and ischemia-related microcalcifications were scattered throughout the brain (); other organs were less extensively involved; no PFO was demonstrated. |
A 72-year-old woman was admitted to our hospital for the treatment of a BAA, which was incidentally detected during the observation of malignant lymphoma. She had a medical history of steroid use for rheumatoid arthritis for more than 20 years. At the age of 57, she underwent cerebral aneurysm clipping surgery due to a subarachnoid hemorrhage. She had no particular symptoms at the time of admission. Her general condition was poor, and she required wheelchair assistance due to moderate hemiplegia. An enhanced computed tomography (CT) angiogram revealed a round, 30 mm-diameter fusiform BAA adjacent to the distal aortic arch (). This aneurysm had two inflow arteries, one being the left bronchial artery and the other being a tortuous and thin artery branching from the lesser curvature of the aortic arch. Some indented outflow vessels drained into the pulmonary artery.\nGiven the patient’s general status and frailty, conventional open chest surgery seemed to have a high operative risk. Therefore, we selected a less invasive endovascular approach. Because there were multiple, thin, and tortuous inflow arteries, coil embolization was considered technically demanding. Finally, we decided to use an aortic stent graft alone to block the blood flow into the aneurysm.\nUnder general anesthesia, the right common femoral artery was exposed, and a 6F sheath was inserted into the left femoral artery percutaneously. After heparin was administered, a 34 mm×157 mm thoracic aortic stent graft (Cook Zenith TX2, Cook Medical, Bloomington, IN, USA) was inserted from the left femoral artery. With fluoroscopic guidance, the stent graft was deployed at zone 3 across the orifice of the feeding artery and dilated with a balloon catheter (Cook Zenith Coda balloon). A post-procedure angiogram showed a small but not negligible type IA endoleak so we added a proximal extension device (Cook Zenith TX2 34 mm×77 mm) at zone 3 to cover the proximal portion of the device. After this procedure, blood flow into the aneurysm halted. The postoperative course was uneventful, and CT angiography at the time of hospital discharge demonstrated complete thrombosis of the aneurysm. Two years after the treatment, the patient was doing well. A repeat CT scan revealed no endoleakage, and the size of the aneurysm was reduced to 8 mm (). |
A 72-year-old sexually active para 2 living in Southern Thailand presented at the outpatient department of our clinic with a 1-month history of an incarcerated uterine prolapse along with an infected wound at the anterior wall of the uterus. She had a 3-year history of a reducible prolapsed uterus and had experienced no symptoms of urinary discomfort or difficulty with defecation. During the month before the current presentation, she had attempted to treat herself with hot stone therapy similar to that practiced by some postpartum women. She believed that placing a heated stone on her uterus would return it to the normal position. However, her uterus was burned. It became swollen, incarcerated, and finally infected because of several underlying illnesses including hypertension, dyslipidemia, and especially poorly controlled diabetes mellitus. Pelvic examination revealed a stage IV genitourinary prolapse according to the POP-Q classification. An ill-defined ulcer measuring 6.5 × 4.5 cm was present in the anterior vaginal wall, and a 2.0 cm diameter ulcer was present in the right posterior vaginal wall (). The prolapsed uterus was nonreducible due to the inflamed, edematous vaginal mucosa. The patient was treated symptomatically with broad-spectrum antibiotics, local estrogen therapy, analgesic and anti-inflammatory agents, and antiseptic dressing of the ulcerated area. After alleviation of all symptoms, the ulcer almost completely healed (). She was advised to undergo definitive surgical treatment for the prolapsed uterus. Six weeks after presentation, the patient underwent a vaginal hysterectomy with McCall culdoplasty and anterior and posterior repairs under spinal anesthesia. Intraoperative cystoscopy showed multiple calculi within the dependent portion of the bladder. The bladder was copiously irrigated. The patient was doing well at her 3-month postoperative visit without recurrence of the prolapse and her postvoid residual urine volume was <50 ml. A follow-up cystoscopic examination revealed no bladder calculi. The pathologic examination of the uterus showed no evidence of malignancy. |
A 42-year-old female presented during the second trimester with severe depressive episode without psychotic symptoms. The depressive episode did not respond to adequate trials of psychotropics and patient became suicidal. Following this, she was admitted to the inpatient unit and managed with six ECTs, started during the 24th week of pregnancy, with which she should significant improvement. She maintained well for later half of pregnancy and gave birth to a healthy male baby at term.\nAll these patients were administered brief pulse, bilateral modified ECT, two to three times per week, by using an indigenously manufactured brief-pulse, constant-energy machine (Medicaid Systems, Chandigarh, India). For all these patients, the frequency was fixed at 70 Hz, and the pulse width was fixed at 1 s. Initial current stimulus varied from 0.4 to 0.6 s with initial charge of 48–72 coulombs. All patients received proton pump blockers to reduce the chances of gastric reflux. Premedication for ECT involved the use of glycopyrrolate (0.2–0.3 mg), and induction was done using thiopental sodium (100–300 mg) and succinylcholine was used for muscle relaxation. All patients received intravenous fluid in the form of saline before and during the ECT procedures. All the patients were properly preoxygenated with 100% oxygen before administration of stimulus. Seizure duration was estimated using cuff method, and a motoric seizure lasting for at least 15 s was considered as an indicator of an effective ECT. In three of the five cases, in which ECT was used during the third trimester, patients were given injection betamethasone 12 mg intramuscular on two consecutive days before starting of the first ECT to promote fetal lung maturity. Injection progesterone 250 mg was given 30 min before every ECT to promote uterine relaxation. The three cases, in which ECT was administered during the third trimester, the procedures were done in the operation theater with multidisciplinary teams involving psychiatrist, gynecologist, anesthetist, and neonatologist. In all the cases, patients were monitored for uterine contractions before and after the ECTs. In all the cases, ECT was given by placing the patients in the left lateral position. Patients were intubated after the procedure as per the requirement. |
A 4-year-old male child with double outlet right ventricle (RV) and pulmonary stenosis with superior-inferior arrangement of the ventricles submitted to right modified systemic pulmonary Blalock–Taussig shunt at the age of 3 months and surgical repair at the age of 2 years showed an abrupt clinical deterioration late after surgery. During the early postoperative period, neither significant clinical nor instrumental findings were reported. However, about 9 months after a seemingly successful surgical repair, he started to complain progressive worsening of effort tolerance and showed a significant increase of cardiac enlargement as compared to the postsurgical baseline chest X-ray. There were no clinical, hematological or echocardiographic features suggestive of infective endocarditis in the recent past or at the time of admission. In particular, white cell count was within normal values, so blood cultures were not done. The electrocardiogram (EKG) was unremarkable with respect to the early postsurgical picture, showing mild tachycardia and complete right bundle branch block as sole relevant findings. On transthoracic echocardiogram (TTE), a rupture of the right SV into the RV outflow tract, measuring 4 mm, was seen on color Doppler analysis, causing significant left-to-right shunt and resulting in significant RV dilatation with global functional impairment. Based on clinical and anatomic findings, percutaneous treatment of this complication was planned as a lower risk option with respect to surgical repair. After parental informed consent and agreement with the surgical team, the procedure was performed under general anesthesia with TTE guide. At cardiac catheterization, moderate RV hypertension (RV/left ventricular pressure ratio 0.6) and severe RV dilatation were found []. Aortic angiography showed a 3-mm large rupture of the right coronary sinus into the RV outflow, resulting in moderate significant left-to-right shunt (QP/QS 1.6:1) []. As a cost–benefit approach, device closure from the aortic side was chosen. The ruptured sinus was then negotiated with a coronary guidewire (Abbott Vascular, Santa Clara, CA, USA) and a multipurpose 4 Fr catheter. Over an exchange 0.035” guidewire, the dedicated delivery system (AMPLATZER™ TorqVue™ LP Delivery System, St. Jude Medical Inc., St. Paul, Minnesota, USA) was tracked into the RV outflow. Then, an ADO II-AS™ 5–2 mm (St. Jude Medical Inc., St. Paul, Minnesota, USA) was deployed under fluoroscopic and echocardiographic guide. Angiography was used for the precise deployment of the device. Procedural and fluoroscopic times were 70 and 13 min, respectively. After deployment, aortic root angiogram revealed mild foaming across the device [] without any aortic valve malfunction or right coronary artery compression. The postprocedural course was uneventful, and the child was discharged after 48 h without new-onset EKG anomalies or residual shunt at echocardiographic evaluation. Over a 3-month follow-up, both clinical condition and RV size and function improved significantly. |
A 59-year-old female patient was admitted with history of abdominal pain for a year and rectal bleeding for a month. At colonoscopy, a tumor mass in scirrhous feature, narrowing the lumen and complicating the passage of the endoscope, was detected on sigmoid colon. Mucosa was normal through the last 10 cm of terminal ileum and the entire colon except tumoral tissue detected at sigmoid colon (). Pathological finding of the biopsy specimens obtained from the mass was reported as colorectal carcinoma (). At computed tomography (CT), asymmetric wall thickening on the rectosigmoid region, pollution of the adjacent mesenteric fat tissue and millimetric lymph nodes were observed. Low anterior resection was performed and pathological diagnosis was moderately differentiated adenocarcinoma with intact borders. Tumor was determined as T3N0M0. Oral capecitabine 3 gm/day for 14 days per month was administered. Two months later, she was admitted to the medical oncology clinic with complaint of a bloody diarrhea and bowel frequency of approximately 10 times/day. Fever and tachycardia was determined at physical examination. Other biochemical parameters were normal with erythrocyte sedimentation rate at 45 mm/hour. Stool examination revealed abundant leukocytes and erythrocytes with no parasitic cysts and ova. Oral capecitabine therapy was stopped. Intravenous ciprofloxacin and metronidazole treatments were administered. However, symptoms of the patients were not resolved on the seventh day of treatment (hemoglobin: 8.4 gm/dl, white blood cells: 5000/mm3, platelets: 283,000/mm3), colonoscopy was performed. On colonoscopy, although mucosa of the terminal ileum was normal, the mucosa of the cecum, ascending, transverse and descending colons and rectosigmoid region was hyperemic, edematous, spontaneous fragile and erosive (). The biopsy specimens obtained from the colonic mucosa was compatible with chronic active colitis with distinct inflammation, moderate activation, crypt abscess, distortion and destruction (). Diagnosis was considered as ulcerative pan colitis with severe clinical and endoscopic activation. Clinic remission was achieved with oral mesalazine 4 gm/day, mesalazine enema 4 gm/ day and metilprednisolone 32 mg/day. After induction of remission metilprednisolone was tapered and stopped. Oral mesalazine 4 gm/day and mesalazine enema 4 gm/ day were chosen for maintenance therapy. Colonoscopy 1 year after the low anterior resection revealed normal colo-nic mucosa with no recurrence of colorectal carcinoma.\nPathophysiology of the ulcerative colitis includes genetic susceptibility influenced by the luminal microbiota, which provides antigens and adjuvants that stimulate either pathogenic or protective immune responses. Environmental triggers as diet, the use of antibiotics and nonsteroid anti-inflammatory drugs, colonic ischemia stress or infection are necessary to initiate or reactivate disease.\nTakakura et al reported an ulcerative colitis case occurring 10 days after resection for sigmoid colon cancer and reviewed 8 other ulcerative colitis cases in the literature triggered by surgery for colorectal carcinoma. Interval for development of ulcerative colitis has been shown between 2 weeks and 8 years. While region that the colorectal carcinoma originates was sigmoid colon at 4 cases, rectum was the site of colorectal carcinoma at 4 cases. Surgical procedures were low anterior resection or sigmoidectomy. Chemotherapy background of the cases was indefinite.\nTo date only two definitive reasons were given for a direct relationship between the colonic operation and the pathogenesis of ulcerative colitis. Takakura et al postulated that an ischemic condition induced by the operation might have caused the development of ulcerative colitis. In fact, it has been reported that the connection of microcirculation could be the etiology of ulcerative colitis, or strong emotional stress from the operation may be the cause as some reports suggest that psychological stress might lead to the pathogenesis, exacerbation, or relapse of ulcerative colitis, possibly triggering 40% of all cases of ulcerative colitis; Lim et al postulated that diversion colitis might have caused the development of ulcerative colitis as leukocytes were sensitized and activated in the vasculature of the inflamed diverted colon, circulated and recruited to the mucosa through the phenotypically similar vascular endothelium of the instream large intestine, finally causing an inflammatory process, which developed into ulcerative colitis.\nAnother possibility is the presence of silent microscopic colitis as the etiological factor of colorectal carcinoma and triggering of manifest ulcerative colitis by perioperative factors. Katsanos et al reported a case of a silent ulcerative colitis adjacent to a regular sigmoid carcinoma. In his case at colonoscopy tumor mass was seen and the remaining colon mucosa was normal. Ulcerative colitis was detected on pathologic examination. This case may indicate that a normal looking mucosal ulcerative colitis increases the risk of colon cancer.\nCapecitabine is an orally administered prodrug of 5-fluorouracil commonly used in the treatment of advanced breast cancer, gastric cancer, colorectal cancer and esophageal cancer. Capecitabine induced colitis is rare with only four cases have been reported in the literature. The mechanism leading to colitis with ischemic features is unclear. Vasoconstriction induced colonic ischemia, mesenteric artery thrombosis due to injury to the endothelium and disruption of factors involved in fibrinolysis, direct mucosal injury and shift in intestinal micro flora which leads to activation of proinflammatory cytokines that results in mucosal necrosis may be the contributing factors.\nExcept adjuvant chemotherapy regimen, the case we present; location of the tumor, interval for development of ulcerative colitis and surgical procedure for treatment of colorectal carcinoma were similar with the cases described in literature. Factors triggering ulcerative colitis after treatments for colorectal cancer might be the presence of silent microscopic colitis, capecitabine induced colonic ischemia and perioperative stress factors. |
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). |
Our patient was a 73-year-old man with an Eastern Cooperative Oncology Group (ECOG) performance status of 2, being followed for prostatic hyperplasia at another hospital as an outpatient since 2009. Around January 2017, he began having a progressive swelling from the right side of the jaw to the neck and, therefore, visited the other hospital. Right cervical lymph node fine-needle aspiration cytology revealed class V, while CT demonstrated right submaxillary gland swelling and right cervical lymph node swelling, leading to a diagnosis of right submandibular gland cancer with right cervical lymph node metastases. Around this time, the patient's serum PSA had also increased from 7 to 30 ng/mL, based on which concurrent prostate cancer was suspected. Bone scintigraphy showed multiple vertebral body metastases and bilateral iliac bone metastases, for which multiple bone metastases from prostate cancer were considered at the other hospital. His right submandibular gland cancer was clinically assessed as stage II, and radical surgery was performed.\nSubsequently in May 2017, the patient was referred to our hospital for a definite diagnosis and treatment of prostate cancer, in the context of complication by submandibular gland cancer. On CT performed at our hospital, the multiple bone metastases showed osteolytic changes. An MRI of the prostate gland detected no apparent abnormalities. Prostate biopsy showed no findings of malignancy. On the basis of these findings, prostate cancer and multiple bone metastases from prostate cancer could be ruled out. On the other hand, tissue specimens from the submaxillary gland showed findings of submaxillary duct carcinoma (salivary duct carcinoma; SDC), and immunostaining revealed CK7(+), CK20(–), androgen receptor(+), GCDFP-15(+), and PSA(+) (Fig. ). On the basis of these findings, although postoperatively, the patient's SDC was re-staged as pT2N3M1 stage IV, with the presence of multiple cervical lymph node metastases and multiple bone metastases.\nThereafter, chemotherapy for advanced submandibular gland cancer was initiated. After 2 cycles of chemotherapy consisting of carboplatin + fluorouracil + cetuximab, his serum PSA level showed an obvious decreasing trend (from 30.5 to 6.0 ng/mL), supporting that the serum PSA levels in this case derived from SDC. |
A 62-year-old female patient who is heavy smoker presented with a burning sensation and discomfort in her left breast that has been recurring over a month prior to admission to the hospital. No fever, chills, or any other symptoms were described. She reported a past medical history of hypertension and a surgical history of hemorrhoidectomy, dilation and curettage surgery, colonoscopy, and gastroscopy.\nPhysical examination revealed a palpable left breast mass (measuring approximately 3 × 3 cm) in the upper quadrant with no overlying skin changes. The right breast exam was normal. No palpable locoregional lymphadenopathy (axilla and supraclavicular lymph nodes) was noticed. Routine blood tests (complete blood count with differential, electrolytes, prothrombin time, partial prothrombin time, and international normalized ratio), chest X-ray, and electrocardiogram (ECG) were all normal.\nMagnetic resonance imaging (MRI) of the left breast showed an ill-defined deep retroareolar spiculate lesion extending over 3 × 1.5 cm revealing early enhancement peak with associated architectural distortion. There were no axillary lymph nodes or abnormal bone signal intensity. No cutaneous thickening or retraction was seen. Findings were suggestive of BIRADS type IV lesion ().\nAn excisional biopsy was performed and revealed breast tissue with extensive lymphocytic infiltrate intermixed with neoplastic epithelial cells (). Immunohistochemistry results were positive for CK AE1/E3 antibody in the neoplastic epithelial cells with no expression of estrogen or progesterone receptors, and HER2/neu was not overexpressed (). The lymphocytes in the background stained positive for both CD3 and CD20 (Figures and ).\nThe patient underwent a left modified radical mastectomy. Eleven lymph nodes were dissected and free of tumor. The mastectomy specimen showed a 3.5 × 3 × 3 cm cavity at the site of the previous excisional biopsy. On histological examination, apocrine metaplasia was identified but no residual tumor was detected. To note, apocrine metaplasia is a very common incidental benign finding that is considered part of or associated with fibrocystic changes, and hence, does not affect prognosis and management []. Accordingly, no adjuvant hormonal therapy, chemotherapy, or radiotherapy was given to the patient.\nNo evidence of recurrence was noted on a 2-year follow-up. |
A 38-year-old female underwent a medical check-up at our institution. The patient’s medical profile had no history of weight loss, anorexia, or weakness. She had had a splenectomy 20 years prior for traumatic splenic rupture. Laboratory tests, including complete blood count, liver function test, and alpha-fetoprotein levels, were within the normal range. An abdominal CT scan without contrast enhancement revealed the absence of a normal spleen, with a homogeneous mass situated at the site (A and B). The mass exhibited heterogenous enhancement in the arterial phase (C), but homogeneous enhancement in the venous phase (D). The mass was suspected to have originated from the pancreas tail (A and C). There was no fat stranding surrounding the mass (). Coronal CT image revealed that this mass compressed the left renal (A). The mass was fed by several branches of the splenic artery (B). An abdominal MRI was performed. This confirmed that the mass was located in the normal site of spleen and compressed the left kidney (A) and that the mass was round, with well-defined borders and without fat stranding. The mass presented as hyperintense compared to liver parenchyma on T2-weighted image (B). T1-weighted images showed that the mass was hypointense compared with liver parenchyma and did not lose signal on T1 out-of-phase images (). Part of the mass was suspected to be connected to the pancreas tail (A and C). T1-weighted image in the arterial phase revealed that the mass enhanced markedly and inhomogeneously (A). The normal left adrenal gland was observed clearly (B). The mass exhibited homogeneous enhancement in the venous phase (C). It was clear that the mass showed restricted diffusion, since it was hyperintense on diffusion-weighted image (DWI) and had hypointense apparent diffusion coefficient (ADC) values on an ADC map compared to liver parenchyma (). The preoperative diagnosis was a tumor of the pancreas tail. The patient underwent an operation to remove the mass by a general surgeon with 10-year experience. Histopathology showed white and red pulps, as well as connective tissue that presented within the mass as trabeculae that carry the arteries and veins (A). The white pulps included periarterial lymphatic sheath, lymphatic follicles, and the marginal zone (B). It was surrounded by a capsule composed of dense fibrous tissue (C). The lymphatic follicles contained benign lymphocytes (D). Histopathology of the mass revealed the appearance of a spleen parenchyma. The final diagnosis was an enlargement of AS after splenectomy. This patient was discharged from the hospital 4 days after surgery without further complications or treatment. The patient was lost to follow-up later. This case was reported in line with the SCARE 2020 criteria []. |
A 57-year-old male with a history of type 2 diabetes and hypertension presented to the emergency department (ED) with low back pain radiating into his legs worsening over the prior three days. The patient was not able to identify any clear precipitating factors or trauma. He described the pain as moderate in intensity and of a “burning” quality. Prior to being seen in our ED the patient was seen at an outside clinic in Mexico where he was given a “Toradol shot and steroids” and diagnosed with sciatica. The patient denied any other symptoms including fever, cough, shortness of breath, chills, or weakness.\nThe patient’s vital signs on presentation were normal except for a heart rate of 101 beats per minute. He was noted to have a blood pressure of 119/79 millimeters of mercury (mm Hg), a pulse oxygen saturation of 96%, and a temperature of 98.2° Fahrenheit (F). The patient’s exam was unremarkable except for pain to palpation of bilateral lower extremities. He was not noted to have any weakness or neurological deficit and had normal bilateral dorsalis pedis pulses. The patient had a blood glucose within normal limits. He was given pain medication and steroids and felt improvement of his symptoms. As the pain seemed to improve, his symptoms were attributed to sciatica and he was discharged home.\nTwo days later the patient returned to the ED reporting worsening pain in his flank and legs and bilateral lower extremity weakness. He reported moderate to severe pain with movement, which had limited his ability to ambulate. The patient complained of some dysuria but continued to deny other symptoms including fever, cough, shortness of breath, chest pain, nausea, vomiting, diarrhea, numbness or inability to urinate. At this presentation, the patient had a heart rate of 66 beats per minute, blood pressure was 120/79 mm Hg, oxygen saturation was 95% on room air, and his temperature was 97.4°F. He reported that his pain on arrival was 10/10. He was found to have full range of motion, normal sensation, and equal dorsalis pedis pulses bilaterally in his lower extremities. He was noted to have pain with movement of his lower extremities, and endorsed tingling. He had normal rectal tone. His lower extremities were noted to be hyporeflexic.\nThe patient was given pain medication and his laboratory results revealed an elevated white blood cell count of 23.5 × 109/liter (L) (reference range 4.0–10.0 × 109/L) with normal hemoglobin, hematocrit, and platelets. His comprehensive metabolic panel was normal with the exception of bicarbonate of 22 milliequivalents (mEq)/L (23–30 mEq/L), an elevated blood urea nitrogen of 60 milligrams per deciliter (mg/dL) (7–20 mg/dL), an alanine aminotransferase of 242 units (U)/L (7–56 U/L), and an aspartate aminotransferase of 617 U/L (normal 0–35 U/L). His creatine phosphokinase was markedly elevated at 26,818 U/L (20–600 U/L). His D-dimer was 562 nanograms per milliliter (ng/mL) (< 250 ng/mL). His lactate dehydrogenase was 2601 U/L (140–280 U/L). The urinalysis demonstrated 2+ blood and 30 red blood cells. Based on his urinalysis, a computed tomography (CT) abdomen and pelvis was also ordered to evaluate for nephrolithiasis.\nThe CT of his abdomen did not demonstrate any significant intra-abdominal abnormalities, but he was noted to have diffuse patchy infiltrates in his lower lungs. A chest radiograph demonstrated patchy diffuse bilateral infiltrates. These findings prompted COVID-19 testing, which resulted as positive despite lack of any upper respiratory symptoms. A lumbar spine CT was completed, which was also unremarkable. While in the ED, his pain and weakness worsened and ultimately a magnetic resonance imaging (MRI) of his lumbar spine and a venous duplex of his lower extremities were also obtained and he was admitted for pain control and further workup. The MRI was unremarkable and his lower extremity venous duplex ultrasonography did not demonstrate any acute deep vein thrombosis. Within two hours of his ED stay, an arterial duplex of his lower extremities was performed and demonstrated no blood flow in the right dorsalis pedis artery or left posterior tibial, anterior tibial, or dorsalis pedis arteries. A computed tomographic angiography (CTA) of the aorta and lower extremities demonstrated diffuse arterial insufficiency with thrombosis of distal abdominal aorta and occlusion of bilateral iliac arteries (). He was then placed on a heparin drip. The patient’s respiratory status deteriorated over the next few days and he required endotracheal intubation for acute respiratory failure. He was transferred to an outside hospital for possible thrombectomy.\nThe thrombectomy was successful; however, the patient’s lower extremities were not salvageable and he required bilateral above knee amputations. His hematology workup revealed slight decrease in protein S 39% (reference range 66–143%) with normal protein C, complement, antithrombin 3, homocysteine, and cardiolipin antibodies, and no evidence of disseminated intravascular coagulation (DIC). Ultimately, the patient’s respiratory status improved and he was extubated. At the time of writing he remained hospitalized on enoxaparin with discharge planning ongoing. |
A 30-year-old Malaysian woman who had bilateral upper lobe lung bullae underwent bullectomy in May 2008 for rapidly enlarging bullae causing respiratory compromise. Post-operatively, she was intubated for 6 days. Upon trial of extubation at day 6, she developed shortness of breath. She was re-intubated for another 5 days before extubation was successful and she was discharged 20 days post operatively.\nShe was well for the next one month until July 2008 when she developed a sudden onset of shortness of breath and stridor. Intubation attempt with a 4 mm endotracheal tube was unsuccessful leading to emergency tracheostomy. Flexible bronchoscopy in the operating room revealed a membranous web-like concentric stenosis without cartilage involvement 3 cm below the vocal cords. Bronchoscopy through the tracheostomy showed normal distal airways. A neck CT scan confirmed the presence of a short segment tracheal stenosis (less than 1 cm) [figure ].\nIn August 2008, she underwent rigid bronchoscopy using a 12 mm Dumon rigid tracheal tube. This was followed by examination using a small diagnostic flexible bronchoscope via the rigid bronchoscope. A pin hole stenosis of the trachea 3 cm distal to the vocal cords was noted with areas of granulation tissue. We failed to pass the flexible bronchoscope through the stenosis.\nInitially argon plasma coagulation (APC) was used to devitalize the granulation tissues surrounding the stenosis. Rigid forceps and cryoprobe were used to remove the necrotic tissues. The APC and cryo probes were inserted through the side-port of the Dumon rigid bronchoscope instead of through the working channel of the flexible bronchoscope to minimize damage to the working channel of the flexible bronchoscope. Bougies was then used to dilate the stenotic area in incremental size to a final size of 1 cm. Pledgets soaked with diluted Mitomycin C (concentration 0.2 mg/ml) were introduced using rigid forceps through the rigid bronchoscope to the raw surface of the mucosa and direct pressure applied via the rigid forceps [figure ]. Pressure was applied for 2 minutes until blanching of the mucosa was seen. The procedure was repeated circumferentially until all mucosa was treated. A total of 4 applications or 8 minutes of contact time was needed [figure ]. Hemostasis was then secured with pledgets soaked with adrenaline and the rigid bronchoscope was removed. The whole procedure took 110 minutes.\nIntravenous dexamethasone 8 mg tds was given for 3 days. At 2 weeks, a flexible bronchoscopy showed a patent airway and she was weaned off the tracheostomy tube after she tolerated spigotting for 48 hours [figure ].\nRepeat flexible bronchoscopy was done at 1 month [figure ] and then 4 month [figure ] after the procedure. The trachea remained patent. A small granulation nodule was seen on the anterior wall of the trachea but a 6 mm therapeutic flexible bronchoscope could easily be passed through the stenosis.\nThere is no significant worsening of the narrowing between 1 month and 4 month. She did not complain of stridor or difficulty in breathing and was back to work. |
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. |
An 80-year-old man complaining of drowsiness was brought to the emergency room by his family. He was in his usual state of health in the previous week and started having influenza-like (flu) symptoms with on-and-off fever, body ache, lethargy, and generalized weakness. His family noticed that, for the past two days, he was sleepier than usual and remained in bed. This was mentioned as very unusual because he maintains an active lifestyle for his age. His family continued that he had recently completed a successful hunting season. He was drowsy on presentation, but oriented to all the spheres without any focal neurological deficits. A chest X-ray showed possible right lower lobe infiltrates, and he was admitted with the initial diagnosis of aspiration pneumonia. Despite appropriate antibiotics, his mental status continued to decline and developed severe encephalopathy responding only to painful stimuli. A computed tomography (CT) scan of the head did not show any acute changes. Arterial blood gas showed evidence of hypercapnic respiratory failure ().\nThe patient was started on bilevel positive pressure ventilation (BIPAP) for carbon dioxide narcosis. A CT chest angiogram did not show any pulmonary embolism, nor any evidence of pneumonia. Despite meticulous titration of BIPAP settings, the patient continued to have persistent hypercapnia with compensated respiratory acidosis (Table1). Based on previous serum bicarbonate levels there was no evidence of chronic respiratory failure. The CT of the chest did not show any structural defect of the pulmonary system, and the patient’s wife denied any symptoms of sleep apnea. In view of persistent encephalopathy despite compensated acidosis, further evaluation was performed. An electroencephalogram did not show any seizure activity. A lumbar puncture showed elevated protein levels (367 G/L) in the cerebrospinal fluid (CSF) and a total cell count of 13/cc with lymphocyte predominance (92%) suggesting meningoencephalitis. With supportive care, his mental condition improved, but the patient could not be weaned from BIPAP. Any interruption of BIPAP would lead to hypoxic hypercapnia respiratory failure. Meanwhile, the CSF viral panel came back as positive for West Nile virus (WNV) antibodies, both IgM and IgG. Upon further questioning, the patient’s wife revealed their recent trip to El Salvador one month ago.\nAs the patient was dependent of BIPAP, neurological respiratory failure due to WNV was considered. The patient did not show any evidence of acute flaccid palsy. In view of his overall condition, fluoroscopic examination for diaphragmatic movements, and detailed pulmonary functions tests to assess respiratory muscle strength were not performed. Manometry showed reduced maximum inspiratory pressure to 25 cm of water (normal: >60), and an ultrasound of the thorax showed absent movements of both diaphragms with deep inspiration (Video 1). Due to a lack of resources, electrophysiological studies of the phrenic nerve and diaphragm were not conducted.\nBased on these corroborative findings, neurological respiratory failure due to WNV encephalitis with isolated phrenic nerve involvement was diagnosed. The patient was continued on BIPAP therapy with improvement in their mental and respiratory condition, however, he succumbed to the terminal illness within two months of disease onset. Isolated phrenic nerve palsy due to WNV leading to respiratory failure is rare, and has only been reported in two previous cases in the literature [,]. |
Three years ago, a 59-year-old male Moroccan presented to the hospital with a dyspnea, dry cough, and a chest pain that had started 6 months before. He had smoked about 30 cigarette packs a year. He was diagnosed non-insulin dependent diabetes 1 year before and was under oral anti-diabetics. He had undergone a successfully surgical intervention for priapism 4 years before the current episode of sickness. His sister is known to have a breast adenocarcinoma. Physical examination at admission revealed a patient with performance status according to World Health Organization (WHO) quote one, and dyspnea type II according to NYHA (New York Heart Association). The pulmonary examination was poor. Head and neck examination found a right thyroid nodule without clinical signs of hypothyroidism or hyperthyroidism. Chest and neck computed tomography (CT) scan were performed and revealed a tumor in the left upper lobe of the lung, in contact with the pulmonary artery (Fig. a: chest axial cup CT-scan pulmonary window). The pulmonary tumor measured 5.5 × 6 cm without mediastinal lymph nodes (Fig. b: chest axial cup CT-scan mediastinal window). The neck CT-scan showed one nodule in the right lobe of the thyroid plunging into the superior mediastinum measuring 8 × 4 cm with homolateral cervical lymph nodes (Fig. ). The level of the thyroid stimulating hormone (TSH) was normal. For the pulmonary tumor, we performed transbronchial biopsies. Pathological examination of the biopsy revealed an adenocarcinoma of the lung, positive thyroid transcription factor (TTF-1).\nOther explorations conducted such as brain CT and bone scan and abdominal ultrasound were normal. We concluded a diagnosis of pulmonary adenocarcinoma classified T4N0M0 with a suspected nodule of the thyroid right lobe. We performed a repeated fine needle aspiration biopsy (FNAB) of the thyroid right lobe but the anatomopathologic results were negatives. We decided to performed a total thyroidectomy and lymph node dissection after extemporaneous anatomopathologic exam, confirming the malignancy of the thyroid right lobe nodule. The pathologic examination revealed a moderately differentiated tubulopapillary adenocarcinoma (Fig. a, b) and three lymph nodes with extracapsular effraction. The immunohistochemistry showed positive tumor cells with TTF1 and cytokeratin (CK) 7 (Fig. a) but negative tumor cells with thyroglobulin and CK20 (Fig. b). Thus, the pulmonary tumor was classified stage IV (T4N0M1) according to the 2009 UICC (Union for International Cancer Control) staging. The multidisciplinary team found that lung tumor was inoperable because it was in contact with the pulmonary artery. The decision to administrate chemotherapy followed by the chemoradiation was retained because there was lack of places on the linear accelerator. A chemotherapy based on the combination of cisplatin and etoposide was administrated along with supportive care. The chemotherapy consisted of the combination of cisplatin and etoposide that were administered according to the following schema: for every cycle, cisplatin 75 mg/m2 on day 1 and etoposide 100 mg/m2 on day 1–3. The length of time between the treatments was 21 days. In total, two cycles of this chemotherapy were performed. We didn’t report any side effect regarding this chemotherapy. After the two cycles, the patient presented dizziness and headaches. A brain magnetic resonance imaging (MRI) was performed and showed multiples brain metastases. The cranial palliative radiotherapy 10 × 3 grays including five fractions per week was realized with best supportive care. This radiation therapy was well tolerated. Particularly, there was no deleterious effect on cognition. After the radiation therapy, we decided to go on with the chemotherapy administration. But the performance status of the patient was poor. Unfortunately, despite reanimation measures and best supportive care, the patient died 2 months later after brain radiotherapy. |
A 72-year-old white male presented to the emergency center with a complaint of right-sided chest pain. He had been treated over the past month for bronchitis with persistent coughing. During a recent coughing spell he developed severe right-sided chest pain and a palpable bulge with localized ecchymosis. The pain worsened and became relentless 12 hours before his visit to the emergency center.\nHis past medical history was significant for obesity, diabetes, hypertension and renal insufficiency. He quit smoking a few years ago and he remembered sustaining a right-sided rib fracture secondary to a fall five years ago. Vital signs were normal on admission; however, discomfort was obvious especially with coughing or movement. Chest exam revealed a painful, palpable bulge with ecchymosis in right posterior chest wall, which enlarged with coughing. The remainder of his physical exam was unremarkable except for moderate central obesity and a small reducible umbilical hernia.\nChest radiography revealed a radiolucent shadow in the right lower posterior chest wall at the level of the diaphragm consistent with an intercostals lung herniation (Fig ). There was no evidence of a right-sided pneumothorax. There were old healed fractures of the right 8th and 9th ribs with minimal deformity. Comparison films from previous admissions confirmed the new finding of a right-sided lucency. Computer tomography of the chest confirmed the findings of the CXR and revealed a posterolateral right lung herniation (Fig ).\nDue to the increasing pain and concern for incarceration, the patient was taken urgently to the operating room for repair. Utilizing a 10 cm posterolateral muscle-sparing thoracotomy incision, we entered the right chest through the 5th intercostal space. Exploration revealed a 4 cm × 2 cm posterior intercostal defect between the 8th and 9th ribs extending anteriorly in a less well defined manner in the form of attenuated intercostal musculature. The diaphragm formed a cul-de-sac with this area of lax intercostal muscles. The lung had spontaneously reduced and its evaluation revealed a mildly indurated area with no hemorrhage or necrosis.\nA piece of polypropylene mesh was fashioned to cover the defect with good overlap. Using number 2 polypropylene sutures (Ethicon, Inc.; Somerville, NJ) and a GORE Suture Passer (Gore, Inc.; Newark, DE), the needle was inserted intrathoracically through the polypropylene mesh and back out through the chest for external tying at the subcutaneous plane. Excellent tension-free repair of the defect was achieved. The diaphragm was re-approximated to the chest wall to complete the repair by obliterating the cul-de-sac described above. A single chest tube was placed and the thoracotomy incision closed. The patient did well post operatively and was discharged from hospital without further sequelae on postoperative day six. At follow-up, the patient remains alive with no hernia recurrence at 5 years. |
A 19-year-old female patient reported to the Department of Oral Medicine and Radiology, K.S.R Dental College and Hospital with the chief complaint of missing teeth in the upper and lower jaw since childhood; she had difficulty in mastication and was feeling esthetically ill. Her medical history revealed that in her childhood she had recurrent history of fever followed, which she visited a physician and the investigation was carried out and diagnosed to have Langerhans cell histiocytosis. History reveals that she had lesions in the skull and mandible at the age of 3 years and was treated with a combination of radiotherapy and chemotherapy radiotherapy of 50 Gy was given in divided doses for a period of 1 year and chemotherapy for about 1 year, patient was under review for about 10 years with no recurrence. Her dental history was not contributory; she gave a history of spontaneous exfoliation of her few deciduous teeth, followed by delayed eruption of permanent teeth. On general examination, patient was normal in stature, appearance and gait. On extra oral examination facial asymmetry was noticed on the left side of the face (the radiation exposed side) [] temporomandibular joint movements were normal and on right and left side deep sigmoid notch was present but more prominent on the left side. On intra oral hard tissue examination revealed clinically present 11, 13, 15, 16, 17, 23, 26, 31, 33, 34, 36, 41, 42, 43, 46, 47 and RS in 62. Hence, there was hypodontia, microdontia of all the teeth and spacing between the teeth [Figures and ].\nOrthopantomogram revealed (a) hypodontia (b) hypoplasia of mandible (c) deep sigmoid notch (d) reduced vertical height of ramus (e) stunted roots of all permanent teeth (f) inversed and impacted 34 and 44 []. Lateral and posteroanterior view revealed no bony lesions, but there was hypoplasia of mandible on the left side with multiple missing permanent teeth [Figures and ].\nPatient was subjected to complete hemogram, serum calcium and phosphorus, which were in normal limits.\nOn the basis of clinical history and radiographic examination, a diagnosis of radiation induced biological changes in bone and teeth were made. |
The patient is a 13-year-old Igbo boy from the South-Eastern region of Nigeria in Sub-Saharan Africa. He had been a resident of a destitute home managed by a Catholic Reverend Sister in Enugu, Nigeria. The destitute home provides habitation for mainly abandoned children and vagrant psychotic patients. The first psychiatric service contact with the patient had been during a community mental health service sponsored by the Rotary Club of Independence Layout, Enugu, Nigeria in which the authors volunteered. The patient was abandoned in a refuse dump a few days after delivery and he had grown up with other abandoned children who found habitation in the destitute home because his parents could not be traced.\nThe patient was born with oculocutaneous albinism and on growing up he had been a child in a world of his own. He rarely played with other children in the destitute home and failed to develop like other children of his age. He was unable to develop speech and incapable of verbal communication; he only screamed sharply if in distress or in need of attention. He avoided eye to eye contact and he often appeared to be looking into space focusing on an unseen object. He failed to reciprocate any social gestures extended to him by his multiple care-givers over the years. He did not turn around if his name was called and he was most of the time preoccupied with playing with his fingers. He often snatched away other children's meal and snacks after finishing his own. Presently at the age of 13 years the patient could not utter a word, only shouting and screaming sometimes without apparent reason. He did not respond to instructions and appeared distant when attempts were made to interact with him. He however often responded to the word 'take', especially if the individual interacting with him was holding a biscuit or any other snacks, which he usually snatched away forcefully and ate voraciously. Associated behavioral problems included destructive tendencies, screaming without apparent reason and running around the destitute home in a circle, which gave him delight as he almost always resisted any attempt to stop him and often required forceful intervention to redirect him.\nThere was no history of any major medical illness that could influence normal neurological development in the patient during childhood and throughout the period of his stay at the destitute home.\nGross motor development was said to have been normal when compared to other children in the destitute home. Developmental impairments were restricted to the areas of communication, cognition and social interaction. The patient had not been exposed to any form of schooling and he had had no social interaction outside the destitute home where he had lived for thirteen years.\nNo information is available about family history.\nHe appeared to be oblivious of his environment and was staring into space, focusing on an unseen object. He did not respond to any questions or instructions during interaction and made no speech of his own. His attention and concentration were impaired during interaction. His attention was however drawn with the word 'take' to which he snatched away forcefully the biscuit from the examiner's hand and ate voraciously as if the biscuit would be taken away from him if he was not fast enough. After this, he was once again in his own world. There was no abnormal motor activity.\nPhysical examination revealed a young boy with features of oculocutaneous albinism (Figures and ). There were no other specific skin lesions. He was small in stature for his age. Gross examination of the Central Nervous System (CNS) revealed no hearing or vision impairment. There was no motor abnormality or sensory deficit. Examinations of other systems were essentially normal.\nFormal Intelligent Quotient (I.Q) test was not carried out on this patient because of the confounding variation that socio-cultural influences have on standardized I.Q tests. However, empirical judgment based on interaction with the patient in the milieu of his socio-cultural environment showed that he was severely retarded with mental age estimated at around 3 to 4 years.\nBased on the history, physical examination and clinical interaction with the patient, co-morbid diagnoses of oculocutaneous albinism (E 70.3) and childhood autism (F 84.0) with severe mental retardation (F 72) were made based on World Health Organization (W.H.O) International Classification of Diseases, 10th Edition (ICD – 10) []. Identified problems in the patient were communication impairment, poor social interaction, and behavioral problems characterized by unwarranted screaming, unruly behavior of snatching away other children's snacks, hyperactivity that was often displayed by running around the destitute home in a circle and destructive tendencies. He was referred to the Child and Adolescent Psychiatry unit of Federal Neuro-Psychiatric Hospital, New Haven, Enugu, Nigeria for further evaluation and management.\nA multi-disciplinary approach to the management of children with autism is desirable, however there are limited facilities for children with autism in a third world country like Nigeria.\nHowever, the behavioral problems were managed with oral Haloperidol 2.5 mg daily on which the patient had been on for three weeks with reduction in hyperactivity and unruly behavior toward other children. |
A 74-year-old female presented to the emergency department (ED) with upper abdominal pain and melanotic stools. She had an elective open juxtarenal abdominal aortic aneurysm repair a month before her index presentation. She was hemodynamically stable. Her pertinent initial labs showed a hemoglobin of 6.7 g/dl (baseline 9.6 g/dl) with a hematocrit of 23%. Patient did not have any fever or leukocytosis. A CT abdomen with contrast done in the ED for abdominal pain showed nonspecific findings, i.e, irregularity of the “aneurysmal sac” with a small amount of fluid around the sac (see ) which was read by the radiologist as early postsurgical changes. She was admitted and was started on proton pump inhibitors. An esophagogastroduodenoscopy (EGD) was performed that revealed mild duodenitis. Her hemoglobin remained stable the next couple of days, and she was discharged home with a 6-8-week course of proton pump inhibitors. Two months later, she presented again with similar complaints with a drop of hemoglobin. A repeat EGD was performed that did not reveal any obvious source of bleeding, and she was discharged home after stabilization.\nA month later, she came for the third time into the ED with abdominal pain, hematochezia, and profound hypotension. Her pertinent laboratory findings include leukocytosis, low hemoglobin and hematocrit, thrombocytopenia, and transaminitis. She was resuscitated with IV fluids and blood transfusions. She was started on broad spectrum antibiotics after blood cultures were drawn. A CT abdomen and pelvis was performed which showed tiny foci of air at the anterior aspect of the native aneurysm wrap just inferior to the location where duodenum crosses (see ). At that time, a decision was made to perform push enteroscopy instead of simple EGD to evaluate second and third portion of duodenum which showed an aortoduodenal fistula with infected graft adherent to the bowel wall and extruding purulent exudate (see ). She underwent emergent surgical excision of the infected graft and bypass grafting to restore vasculature. Her blood cultures and cultures from the graft revealed methicillin-resistant Staphylococcus aureus (MRSA) and Streptococcus agalactiae. Aggressive management was continued with proper antibiotics in the intensive care unit, but her condition deteriorated, and she expired within several days. |
A 66-year-old man presented with severe dysphagia, weight loss and recurrent pulmonary infections due to an esophago-tracheal fistula. Due to squamous cell carcinoma of the esophagus 17 month ago, he had undergone esophageal resection and interposition of a gastric tube with cervical anastomosis. This treatment was followed by adjuvant radio-chemotherapy (50 Gy with 5-FU and Cisplatin). The first signs of dysphagia developed 8 weeks before admission to our hospital. Initial endoscopic therapy revealed local tumor recurrence beginning at 21 cm from front incisors, but failed to provide palliation of dysphagia. The distal end of the stenosis could not be measured precisely due to high grade stenosis which could not be passed endoscopically. Though intravenous hyperalimentation was administered, the patient kept on losing weight. Furthermore recurrent pulmonary infections occurred and swallowing of salvia, without coughing became impossible. For palliative surgical treatment the patient was transferred to our institution. Unfortunately we found the esophageal lumen to be completely obstructed by recurrent tumor. Moreover the tumor had invaded the trachea and had caused an esophago-tracheal fistula. The fistula itself could not be seen endoscopically, but was found by gastrographin swallow and CT-scan (Figure , Figure ). According to CT-scan we estimated it to be located at about 2–3 cm distal from the beginning of the stenosis. The recurrent mediastinal tumor was estimated to have a length of 6 cm and infiltrated the gastric tube.\nAll endoscopic attempts to pass the obstruction failed, because the guide wire only entered the associated esophago-tracheal fistula. Therefore a retrograde endoscopic approach was undertaken. According to the previous esophageal resection with interposition of a gastric tube, radiologically guided percutaneous gastrostomy techniques [] had to be rejected. Retrograde access to the esophageal lumen was obtained by open surgery and a duodenotomy (Figure ). Through an endoscope a guide wire (Terumo, RF-GA35403M Standard, 0.035 inch) was pushed up and the esophageal obstruction was traversed, which simultaneously was monitored by a transnasal endoscope. Using a guiding catheter the esophageal stenosis was dilated and a naso-jejunal triluminal feeding tube was placed into the first jejunal loop. Subsequently the longitudinal duodenal incision was closed in a transverse fashion. Before closure of the abdominal wall a jejunostomy catheter was implanted to ensure sufficient enteral nutrition. 72 hours later, in a second step further endoscopic guided dilatation of the esophageal stenosis was repeated twice. Using a stiff wire (0.035 inch) placed under fluoroscopic control subsequent guide wired dilatation, up to 12.8 mm according to the method of Savary, was performed. In a third step a nitinol self-expanding fully covered stent, the so called Choo stent (M.I. Tech/MTW), was placed across the fistula under radiological and endoscopic control (Figure ). After successful placement of the stent the upper end was located directly proximal from the stenosis at about 20 cm from frontal incisors and completely traversed the whole stenosis. As a result the patient felt neither foreign body sensation nor pain. A follow up contrast study, performed on the 4th day after stent placement, showed the stent to be almost completely expanded without any signs of persisting leakage. Thereafter the patient was allowed to swallow liquid food, although only a small volume could be swallowed at a time. A few days later swallowing of semi solids and hypercaloric liquid food was possible and the patient was discharged.\nFollow-up analyses revealed that the patient died 158 days after our treatment due to severe pleural effusion and diffuse pulmonary metastasis. |
A 65-year-old man presented with a history of mild low back ache pain 1-month duration, radiating to flanks and anterior aspect of abdominal wall. He also had burning sensation and paresthesia in both lower limbs (below groin) of 1-month duration. He had dull abdominal pain for the last 3 weeks, which was associated with loss of appetite. There was no history of fever, trauma, or any focal weakness. There was no history of bowel and bladder disturbances. There was no history of diabetes, hypertension, or tuberculosis. There was no history suggestive of contact with tuberculosis. He was not a smoker or alcoholic. His nutritional status and general and systemic examination were normal. His abdominal examination was unremarkable, and there was no tenderness or hepatosplenomegaly. On spine examination, there was tenderness over L3–L4 region. There was no gibbous or deformity. Higher mental functions and cranial nerves were normal. Motor and sensory examination was normal. Deep tendon reflexes in the upper and lower limbs were normal. Planters were bilateral flexors. X-ray of the chest and lumbar spine was normal. His blood investigations except erythrocyte sedimentation rate (40 mm in 1 h) were within normal range. Ultrasound abdomen showed a lobulated measuring 5.8 cm × 4 cm × 7.3 cm in the left retroperitoneal paraspinal region lateral to the aorta at L3 level. The patient was further investigated with magnetic resonance imaging (MRI) of the lumbar spine which showed a heterogeneous mass at L3 level, mainly located in the left paraspinal area and involving the left psoas muscle. The lesion was extending into the neural foramina with signal changes in vertebral bodies; there was compression of the thecal sac and obliteration of the subarachnoid space [Figures –]. The mass was heterogeneously enhancing after contrast administration. Based on imaging findings and short clinical history, a diagnosis of the left paraspinal malignant tumor was suspected. The patient underwent L3–L4 unilateral partial laminotomy, there was grayish, relatively avascular lesion in the left paraspinal region, involving the left psoas muscle and going into the neural foramina, and a subtotal resection of the lesion could be performed. There was no pus in the lesion. Bony elements were relatively healthy on inspection. The patient recovered well after surgery. Histopathological examination of the excised tissue showed caseous necrosis with adjacent granulomas containing epithelioid cell, Langhans giant cells, and lymphocytes suggestive of tuberculosis []. The patient was advised to bear thoracic corset and started on antitubercular treatment (rifampicin, isoniazid [INH], pyrazinamide, and ethambutol for 3 months followed by rifampicin and INH for further 9 months) and doing well at 9-month follow-up. |
A 42 years old woman presented with a frontal scalp subcutaneous nodule that gradually became larger during 7 months. Her past medical history was unremarkable. Physical examination showed a 6 cm scalp nodule at right frontal area.\nA brain magnetic resonance image (MRI) and computed tomography (CT) scan were obtained that showed a large lytic lesion arising from right frontal bone with destruction of outer and inner table and diploic space and evidence of soft tissue mass. No calcification was seen in the soft tissue component ( and ). The patient underwent surgery. The mass was completely resected with free margins; and the frontal bone defect was reconstructed.\nGross examination of the specimen showed a creamy-brown-colored mass measuring about 6 cm in diameter rested on a fragment of bone. Bone invasion was evident in step sectioning.\nHistologic study showed multiple well-formed microfollicles lined by single layer of cuboidal cells with centrally located nuclei. Invasion of bone trabeculae by the follicles was confirmed on microscopic study ().\nFollicular cells were immunoreactive for cytokeratin and thyroglobulin.\nThe patient was asked for history of any thyroid problem. She did not mention any history of thyroid enlargement, pain, or other symptoms of thyroid disease. Thyroid examination was normal.\nThyroid function test showed normal hormone levels. Then, thyroid ultrasonography was done which revealed a well-defined hyperechoic mass in deep part of left lobe, suspicious for malignancy. So, total thyroidectomy was performed.\nGross examination of the thyroid showed enlargement of left lobe. Multiple cut sections revealed two well-defined creamy-gray nodule in left lobe of thyroid. The larger nodule is measuring about 3 cm and the other is measuring about 0.5 cm in greatest diameter ().\nMicroscopic examination of the nodule showed a follicular neoplasm with various, sized microfollicles and area of capsular invasion; nuclear features of papillary carcinoma (intranuclear inclusions, grooves, and ground-glass appearance) were absent. Representative sections (10 sections) show no vascular invasion (Figures and ). So, the patient diagnosis was follicular thyroid carcinoma with skull metastasis. |
A 5-year-old girl with acute myeloid leukemia (AML) with t(8;21) underwent relatively standard AML chemotherapy, which consisted of 2 cycles of induction chemotherapy with daunorubicin, cytarabine, and etoposide, followed by 2 cycles of consolidation chemotherapy with high-dose cytarabine 3 gr/m2 per dose ×6, in each cycle. Intrathecal therapy was also carried out. The patient was in remission by morphology and flow cytometry assessment after the first cycle and in molecular remission after the second cycle by flow cytometry assessment. Four months after the end of therapy, she presented progressive radicular pain of the lower right extremity and gait claudication. Neurological assessment revealed symmetrical depressed reflexes of the lower limbs and reduction of right knee extension. The posterolateral regions of the right buttock and thigh were painful to the touch. Laboratory evaluation showed normal blood counts. The imaging study evaluation with an MRI of the spine revealed a soft tissue intracanalar epidural mass located from L5 to S4, which extended through multiple sacral neural foramina to the presacral space. The tissue involved sacral nervous roots () and the sciatic nerve (). The infiltrating mass was hypointense in T1- and T2-weighted images and enhanced after contrast (). Based on the MRI findings, the authors suggested the diagnosis of chloroma. The chest X-ray was normal. In order to confirm the leukemic relapse, a bone marrow aspiration with biopsy and a minimal invasive biopsy of the sacral mass, guided by Computed Tomography, were performed. Bone marrow evaluation showed a hypercellular marrow with 2% myeloid blasts with fluorescent in situ hybridization (FISH) testing and was positive for the t(8;21) ETO/AML1 fusion. Histologic findings of the sacral mass revealed connective tissue without signs of invasion by previously diagnosed neoplasia. Flow cytometry could not verify the etiology of the mass, but ETO/AML1 translocation was confirmed by PCR in the biopsied sacral mass. The 2% of blasts in the bone marrow and the myeloid sarcoma as an epidural mass suggested the diagnosis of relapse combined with AML.\nFollowing the institutional guidelines for relapsed AML, the patient started FLAG-IDA as a relapse protocol. Intrathecal chemotherapy was not performed in the first cycle due to the mass's large volume.\nThe patient was in remission by morphology and flow cytometry assessment after the first cycle of FLAG-IDA. Neurological assessment revealed normal and symmetric reflexes of the lower limbs, as well as symmetric and normal knee extension. At that time, the patient did not have pain in the right buttock or thigh. The MRI evaluation revealed a significant tumor mass volume reduction at that time ().\nTriple intrathecal therapy was performed at the beginning of the second FLAG-IDA cycle and there were no blasts detected in the cerebrospinal fluid. Following the second cycle, the patient experienced a four-month period of hematologic aplasia, which postponed the allogeneic stem-cell transplant (allo-HSCT). Meanwhile, she remained in remission but did not fully recover from the hematologic aplasia, while the platelets levels remained low. She started maintenance chemotherapy, in addition to intrathecal therapy (no blasts in the CNS fluid) as a bridge to allo-HSCT. At this point, the patient is waiting for allo-HSCT. The decision to undergo radiotherapy has been postponed until further evaluation after the transplant, as there was no growth of the sacral mass during the prolonged bone marrow aplasia. |
An 86-year-old woman with known history of coronary artery disease and sick sinus syndrome was admitted to the hospital with dyspnea, orthopnea, and exertional dizziness. Physical exam revealed bibasilar crackles, a grade 5 crescendo-decrescendo murmur, elevated JVP, and lower extremity edema. Laboratory findings were pertinent for creatinine of 1.73, BUN of 45, and NT-pro-BNP of 10k. The rest of her physical exam and laboratory was normal. Electrocardiogram showed normal sinus rhythm with known left bundle branch block. 2D echocardiogram demonstrated severe aortic stenosis with mean aortic valve pressure gradient of 68.6 mmHg and peak velocity of 5.15 m/s with preserved systolic function. She received intravenous diuretics with some clinical improvement. She was seen and evaluated by cardiothoracic surgeon for evaluation of aortic valve replacement but deemed high risk for surgical aortic valve replacement with an estimated surgical mortality by Society of Thoracic Surgeons score of 8.1%. Cardiac computed tomography angiography was done as part of transcatheter aortic valve replacement evaluation and showed severely calcified aortic leaflets with short and low left coronary system with coronary ostial height of 8.4 mm (). Special attention was paid to the left coronary leaflet calcification and the short and low coronary ostium. Coronary angiography revealed 50% LAD stent restenosis, diffuse distal LCX disease, and 90% proximal RCA stenosis. A drug-eluting stent was implanted to the right coronary artery. The decision then was made to proceed with TAVI utilizing coronary protection technique during the procedure. TAVR was then undertaken from the right femoral artery through a 14F Edwards arterial sheath. Two 300 mm long Prowater wires were advanced to the LAD and LCX arteries. With rapid ventricular pacing over a long Amplatz extra stiff wire, balloon aortic valvuloplasty using an Edward 4 × 23 mm balloon with simultaneous root aortography to see how the left main flow was performed (). There was flow compromise in the left main system to TIMI-2 flow; the wires were deformed and the large piece of calcium in the left cusp moved right over the left main coronary artery (). We therefore made decision to place two 3.5 × 12 mm Robel bare-metal stents in the LAD and LCX. An Edward 23 mm SAPIEN 3 valve with rapid ventricular pacing was then deployed followed by deploying the two stents in a kissing fashion, and an excellent result was obtained (). Position of the valve and function was confirmed by aortography and transesophageal echocardiography (TEE). The left main coronary flow was excellent. The patient tolerated the procedure well and recovered uneventfully and was discharged 2 days after the procedure without complications. At 30-day follow-up, she has notable improvement of her symptoms and physical activity with NYHA class I from class IV symptoms. |
A 29-year-old male, with a history of excessive alcohol intake and chronic pancreatitis, presented to the emergency department at The Chrissie Tomlinson Memorial Hospital, Cayman Islands. He exhibited a 24-hour history of abdominal pain, which was increasing in intensity, without a history of significant trauma. He also had associated vomiting. An abdominal examination revealed upper abdominal tenderness but no signs of peritonism.\nThe patient was investigated with ultrasound scan (USS) that revealed a PP (). A computed tomography (CT) scan of the abdomen confirmed the presence of a PP in direct contact with the inferior pole of the spleen (). The patient was not known to have a PP prior to admission. A heterogenous hypodense area of the splenic hilum was noted and there was a grossly enlarged liver with a normal gall bladder (). Fat stranding was identified at the tip of the pancreatic tail with a thickened lateral conal fascia. A small fluid collection was seen between the upper pole of the spleen and the left liver. His serial serum amylase throughout this period remained within normal range, making acute pancreatitis less unlikely [].\nOver the following 24 hours, the patient became haemodynamically unstable and showed signs of peritonism. An USS revealed intra-abdominal haemorrhage (), which was confirmed by CT as a result of a splenic rupture (). The patient was transfused and underwent an emergency laparotomy with splenectomy and distal pancreatectomy. The spleen was completely disintegrated and the parenchyma had shelled out of the capsule. The tail of the pancreas was densely adherent to the hilum of the spleen. The histology report did not demonstrate acute pancreatitis but identified variable inflammation, reactive changes, and focal necrosis, consistent with chronic fibrosing pancreatitis. Following surgery, the patient went on to make a full recovery. We believe that the PP directly contributed to atraumatic splenic rupture within this individual (ASR). |
A 53-year-old Caucasian woman was referred to our institution because of the suspicion of peritoneal carcinomatosis, raised by the findings of ascites at a transvaginal ultrasound performed as a yearly routine exam; a pre-surgical staging exam with computed tomography (CT) scan show thickening of the gastric walls, multiple omental nodules and ascites ().\nHer previous personal history was unremarkable and she denied any clinical symptom or cancer history.\nAt our hospital, she underwent an esophagogastroduodenoscopy and colonoscopy, with results negative for gastric/colon cancer.\nHer comprehensive metabolic profile revealed mild liver dysfunction with an alanine transaminase of 77 U/L and aspartate transaminase of 71 U/L. When tumour markers were assessed, CA125 demonstrated increased levels of 290 U/mL (normal values <35 U/mL), whereas carcinoembryonic antigen, CA 19.9 and other immunohistochemical markers were within the normal ranges. Serological assessment of HIV, hepatitis C virus and hepatitis B virus were negative.\nTen days later, the patient underwent an ultrasound-guided biopsy () with a diagnosis of suspicious carcinoma from the an unknown primary site.\nAfter 2 weeks, the patient received a CT scan of the thorax (to complete pre-operative staging), demonstrating a spontaneous (with no therapy) dimensional and numerical reduction of peritoneal lesions in the upper abdomen, partially included in the chest CT scan, as well as resolution of peri-hepatic and peri-splenic ascites ().\nSince there was no evidence of primary cancer at pre-operative examinations and the second CT scan revealed a partial resolution of peritoneal implants and ascites without therapy, the suspicion of an infectious disease was raised.\nThe pathological evaluation of the biopsies performed on the omentum and peritoneum revealed the presence of lymphoid aggregates with a central core of epithelioid cells with large eosinophilic cytoplasm, without atypia or mitosis () and with no immunohistochemical marker of oncologic malignancy. Due to the presence of necrotic nodules with histiocytes and giant cells, a Ziehl–Neelsen stain was performed to identify bacilli (), whose presence was then confirmed with molecular assays.\nBecause of the uncertain result of the biopsy and the conflicting results of the 2 CT scans, in order to rule out malignancy with certainty, the patient underwent a laparoscopic surgery in 2 weeks.\nDefinitive histological diagnosis excluded the presence of malignant cells and reported a necrotising inflammation caused by non-tuberculous mycobacteria. Hence, the patient was sent to a hospital with expertise in infectious diseases.\nA follow-up CT scan performed 1 year later confirmed a complete recovery of peritoneal findings. |
A 64-year-old man with liver cirrhosis, advanced hepatocellular carcinoma (HCC) that invaded the main portal vein, and adrenal metastasis was admitted for the treatment of giant rectal varices due to portal hypertension. During the preceding 6 years he had undergone partial hepatectomy, six sessions of transcatheter arterial chemoembolization, and radiation therapy (39 Gy) for his HCC. He had no history of melena.\nOn admission, his functional reserve of the liver was Child-Pugh grade B. Colonoscopy showed huge tortuous rectal varices. Contrast-enhanced computed tomography (CT) revealed giant varices with tori inside the rectal lumen (Fig. a). Flow from the dilated inferior mesenteric vein was into the rectal varices that drained primarily into the left internal iliac vein. On inspiratory CT the diameter of the inferior mesenteric vein and left internal iliac vein was 10 mm.\nAfter obtaining informed consent from the patient and his family we attempted interventional therapy to prevent rupture of the varices. The plan was to fill the varices with a sclerotic agent via the IVM using balloon assistance. As tumor thrombosis into a main portal vein ruled out the percutaneous transhepatic approach we chose an ileocolic vein approach after laparotomy under general anesthesia. Portal venography via the superior mesenteric vein (SMV) confirmed that the rectal varices were supplied by the inferior mesenteric vein and mainly drained into the left internal iliac vein (Fig. b–d). Inflation of a 6-Fr balloon catheter (Selecon MP Catheter, Terumo Clinical Supply, Gifu, Japan) with a 2-cm diameter balloon in the inferior mesenteric vein failed to produce congestion in the varices. Consequently, we inflated a 6-Fr balloon catheter placed in the main trunk of the left internal iliac vein via the right common iliac vein (CIV) to block the in- and outflow of the rectal varices. However, we could not obtain congestion in the varices and contrast material in the varices drained into the collateral circulation of the left and right internal iliac vein. Thinking that flow in the rectal varices could be reduced by balloon occlusion of the left common iliac vein after coil embolization of the left internal iliac vein, we embolized the main trunk of the left internal iliac vein with nine microcoils. A 3-Fr microcatheter (Renegade, Boston Scientific, Natick, MA, USA) was advanced through the inflated 6-Fr balloon catheter placed into the orifice of the left internal iliac vein to prevent coil migration. First, one interlocking detachable microcoil (diameter 12 mm, length 30 mm) (InterlockTM, Boston Scientific, Cork, Ireland) was introduced as an anchor coil. Then we intertwined six pushable microcoils (diameter 8 mm, length 14 cm) (Micronester, Cook, Bloomington, IN, USA) with the anchor coil. Lastly two InterlockTM coils (diameter 10 mm and 12 mm, length 30 cm) was placed to hold the other seven coils and to obtain embolization.\nPostprocedure portal venography obtained under double balloon inflation in the inferior mesenteric vein and left common iliac vein revealed flow reduction in the rectal varices. However, as drainage via collateral vessels in the pelvis persisted 5 mL of absolute ethanol and 40 mL of a glucose solution were injected via the inflated balloon catheter in the IMV to embolize these drainers. This also failed to obtain complete congestion in the varices. As filling the rectal varices with a sclerotic agent was difficult under the existing conditions we attempted to embolize the varices with liquid glue. A 3-Fr microcatheter was advanced through the inflated balloon catheter in the internal mesenteric vein into the left superior rectal vein as close as possible to the rectal varices. Then 5.5 mL of a 1:10 mixture of N-butyl cyanoacrylate (NBCA) (Histoacryl, Aesculap, Tuttlingen, Germany) – lipiodol (André Guerbet, Aulnay-sous-Bois, France) was injected via the microcatheter under inflation of the balloons in the IMV and left CIV. As portal venography revealed that the right superior rectal vein flowed into residual rectal varices we injected 40 mL of 50% glucose and 12.5 mL of 5% ethanolamine oleate (Oldamin, Takeda Pharmaceutical, Osaka, Japan) with iopamidol (Iopamiron 300, Bayer HealthCare, Osaka, Japan) as a sclerotic agent through the microcatheter in the right superior rectal vein. Then eight pushable microcoils (diameter 8 mm) (Micronester) were placed in the internal mesenteric vein under double balloon inflation. As portal venography confirmed the complete obliteration of the rectal varices we removed the catheters (Fig. e, f). A plain radiograph obtained 30 min later confirmed that the coils remained in the left internal iliac vein. Throughout these procedures the patient's condition was stable and he was under continuous observation by anesthesiologists.\nDuring extubation the patient suffered a paroxysm of coughing and immediately after extubation he developed dyspnea and shivering. His partial pressure of arterial oxygen and percutaneous oxygen saturation slightly fell to 87 mmHg and 95% under oxygen inhalation of 5 L/min. Under continuous oxygen inhalation his symptoms abated somewhat and he was placed under observation with oxygen inhalation.\nOn the first postoperative day his dyspnea disappeared and the percutaneous oxygen saturation was 98% in room air. There were no respiratory symptoms. Although the liver function deteriorated transiently he developed no acute complications. A CT study performed on the fifth postoperative day confirmed complete obliteration of the rectal varices (Fig. a). However, all nine metallic coils placed in the left internal iliac vein had migrated into a lower branch of the right pulmonary artery (Fig. b and c). As this elicited no respiratory symptoms and as we considered the removal of the coils by interventional procedures inadvisable at that time, he was discharged 10 days after undergoing the procedures.\nHe subsequently received hepatic arterial infusion chemotherapy for HCC and endoscopic treatment for aggravated esophageal varices. Although colonoscopy showed shrinkage of the rectal varices, CT obtained 3 months later revealed progression of his intrahepatic HCC, enlargement of the adrenal metastasis, and newly developed lung metastasis. He died of HCC 5 months after the obliteration of the rectal varices. |
A 31-year-old female patient presented with blurred vision in both eyes which was more severe in her left eye. The problem had started 3 years prior to presentation. The condition had been slowly progressive 6 months prior to her first visit. The patient did not use glasses or any optical aids.\nFamily and past history were unremarkable. Best corrected visual acuity (BCVA) was 2/10 and counting fingers at 2 meters with -18.0-5.0 × 70 and -19.0 in her right and left eyes respectively.\nSlit lamp biomicroscopy showed inferior corneal thinning bilaterally about 2 mm above the limbus with a stromal scar involving the central cornea, extending to the inferior area of thinning in her left eye. The remainder of the ocular examination was unremarkable bilaterally. Corneal topography, showed a crab- claw pattern in the right eye and a very irregular pattern in the left one ().\nPK was performed in the left eye and a 7.50 mm sized recipient bed received a 7.75 mm donor with small inferior graft decentration.\nOn the first postoperative day, the patient was found to have a positive Seidel test and mildly shallow anterior chamber (AC); the microleakage was from a suture tract located at the 6 o’clock position of the host-graft junction. There was no apparent wound gap but a small host-graft mismatch was present. The patient was followed while receiving steroid and antibiotic drops; a few days later the leakage ceased and the AC was fully formed.\nThe rest of her postoperative course was uneventful up to 8 months when her last sutures including the one at 6 o’clock were removed. Several days later, the patient developed acute hydrops at the inferior host-graft area mostly involving the recipient cornea (). Seidel test was positive but the AC was formed. Antibiotic drops were started and the eye was patched; 2 days later the leakage stopped and edema in the inferior recipient cornea decreased. One week later there was no leakage and the hydrops disappeared with final uncorrected BCVA of 2/10 (). |
A 39-year-old woman presented for evaluation of difficulty swallowing and epigastric pain. Her medical and familial histories were unremarkable. Upper gastrointestinal endoscopy showed a smooth elevated lesion located 19 to 24 cm from the incisor teeth (Fig. ). Endoscopic ultrasonography showed a large tumor mass of low echogenicity in the esophageal wall. The tumor mass originated in the muscle layer, and we performed a boring biopsy of the tumor mass. However, we were unable to obtain a tissue specimen from the tumor.\nA chest enhanced computed tomography (CT) scan revealed a tumor mass (39 × 28 × 56 mm) at the upper thoracic esophagus with internal heterogeneity. No metastasis to the lymph nodes or other organs was seen on the CT scan (Fig. ). FDG-PET CT showed a hypermetabolic appearance matching the tumor mass. The maximum standardized uptake value (max SUV) was 5.5 (Fig. ).\nMagnetic resonance imaging of the chest revealed that the boundary of the tumor was clear and smooth. The mass exhibited isointensity or low intensity compared with the muscles on T1-weighted images and high intensity on T2-weighted images. There was no invasion into the surrounding tissue.\nWe performed endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS-FNAB) with the patient under general anesthesia. Because the specimen was small, we were unable to establish a definitive diagnosis. We suspected an esophageal schwannoma, leiomyoma, or gastrointestinal stromal tumor (GIST).\nThe patient underwent surgery because of her difficulty swallowing and our suspicion of malignant potential. We performed thoracoscopic surgery with the patient under general anesthesia and single-lung ventilation.\nFirst, we attempted enucleation of the tumor. However, the mass was larger than 5 cm, and enucleation was difficult without creating an extensive defect of the esophageal wall. Therefore, subtotal esophagectomy was performed with thoracoscopic assistance.\nThe resected specimen measured 55 × 45 × 24 mm. The cut surface was solid milky white (Fig. ). Histopathological examination revealed spindle-shaped cells in a fasciculated and disarrayed architecture in the proper muscle layer. Immunohistochemical studies showed S100 protein expression and the absence of CD34 and c-kit protein expression. Nuclear division was inconspicuous, and the MIB-1 labeling index was <10 %. Finally, we diagnosed the tumor as a benign schwannoma. The patient’s postoperative course was uneventful, and she had no evidence of recurrence at the time of this writing. |
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 79-year-old woman, with no significant past medical history, had a fall landing on her flexed left knee. As a consequence, she was unable to actively extend her knee and straight-leg raise. The patient walked with difficulty using a stick and did not seek medical attention until a year later. The patient was of short stature had a body mass index (BMI) of 31. There was gross quadriceps wasting, the patella tendon was not palpable, and the patella was proximally displaced. She had no active knee extension. Plain radiographs revealed a patella alta. An ultrasound scan confirmed complete deficiency of the patella tendon. It was agreed to reconstruct her tendon using synthetic ligaments.\nThe operation was performed by the senior author (DG), with the use of a thigh tourniquet inflated to 300 mm Hg. Antibiotic prophylaxis was given. A midline skin incision was used. Findings were complete absence of the patella tendon and deficiency of the adjacent retinacular tissue. The remaining tissue was unsuitable for primary repair. Two bundles of LARS ligament were used. The rounded end was fixed to the tibial tuberosity with an interference screw (Fig. ). The flattened end passed through tunnel prepared deep to the fibrous tissue covering the anterior aspect of the patella. Each bundle was secured with three Ethibond sutures, after the patella had been reduced to a satisfactory position. The repair and the adjacent retinacular tissue were reinforced with a Vicryl mesh. The wound was closed in layers. No drain was used. Operating time was 45 min.\nThe knee was splinted (cricket pad splint) in extension for 6 weeks. In the meantime, the patient was mobilizing as able with crutches and was encouraged to perform straight-leg raising exercises to strengthen the quadriceps muscle. Free mobilization and physiotherapist-guided knee flexion were initiated 6 weeks postoperatively. The patient was reviewed regularly in the outpatients’ clinic for 1 year.\nThe Insall/Salvati ratio [] improved from 1.9 preoperatively to 1.3 postoperatively (Fig. a, b). The patient was able to perform active straight-leg raising as early as 2 weeks after her operation, when examined in the clinic. At 1-year follow-up, she was able to mobilize unrestricted, had regained full knee flexion (Fig. a), and had full power of extension (Fig. b). |
We report the case of a female patient, 1.55 m, 49 kg, 25 years old, with suggestive medical history for congenital nystagmus, severe scoliosis, and visual disability. Further information about the patient, such as past medical history, allergies, medication, and social and family history, is shown in .\nTreatment started with passive lip seal, acceptable facial asymmetry, and closed nasolabial angle. There was prevalence of horizontal growth (). The intraoral evaluation revealed the presence of severe crowding in both upper and lower arches, upper canine in buccal-version, and left upper lateral incisor palatal tipped in corssbite position. There are half cusp (1/2) class II malocclusion on the right side and 3/4 cusp class II on the left side and overjet of 2 mm and overbite of 5 mm ().\nAfter assessing the panoramic radiography and teleradiography (), the patient was diagnosed with maxillary retrusion, thus causing natural compensatory proclination of upper incisors. Mandible was normal with lower incisors well positioned in the symphysis ().\nThe options given to the parents of patients were exodontia of third upper molars and first upper premolars (teeth 14 and 24) even with the prevalence of horizontal growth, considering that the amount of crowding would fill the extraction space and the retraction of the anterior quadrant would be small, thus not aggravating the overbite. However, parents refused this option because of the high number of extractions and the potential lack of patient cooperation during surgical therapy. The second option, approved by the parents, was distalization with pendulum supported in mini-implant as skeletal anchorage. As such, the main objectives of the treatment would be achieved: distalization of first upper molars toward Class I position and improvement of dental and facial esthetics.\nThe treatment had a few steps. First, exodontia of teeth 18 and 28 was requested subsequently under local anesthesia (2% lidocaine hydrochloride with 1 : 50,000 norepinephrine hemitartrate); then, 2 titanium mini screws (SIN, São Paulo, Brazil) of 1.6 cm in diameter and 8 mm in length were installed in the hard palate area, not parallel to each other. After installation, an alginate molding was made and forwarded to the production of the pendulum's anchorage unit, where it would be bonded with photoresin ().\nAfter 1 month from the start of distalization of first upper molars, fixed orthodontic appliances were bonded to the upper and lower arches: Roth prescription (Iceram, Orthometric, Marília, SP, Brazil), 0.022′′ × 0.0028′′ slot with 0.014′′ Flexy Super Elastic, Orthometric, wire. A bracket was not bonded to tooth 22 for lack of space in the dental arch (). In the fifth month of treatment, the first molars were in Class I, with accentuated buccal torque () and decreasing upper and lower crowding. In this step, the pendulum was removed along with the anchorage unit. Then, space opening for tooth 22 started, with spring (JS) produced with a 0.018′′ steel wire. In the tenth month of treatment, the remaining spaces were closed with chain elastics and with the installation of a 0.019′′ × 0.025′′ steel wire in the upper arch, improving the torque in the first upper molars (); a 0.018′′ steel wire was installed in the lower arch. Folds (offset) were applied in the area of teeth 33 and 43, seeking the proper lateral movement ().\nAfter 11 months from the start of the treatment, the intercuspation procedure began. By the end of intercuspation and occlusal adjustment, the orthodontic fixed appliance was removed and retainers were produced. A Hawley plate was installed in the upper arch and a 3 × 3 fixed retainer was installed in the lower arch. Orthodontic therapy lasted 12 months (Figures and ).\nIn the first posttreatment control, one month after the removal of the fixed appliance, the upper anterior teeth were rebonded for the repositioning of tooth 22, which presented lingual relapse () due to the lack of patient cooperation in using the upper removable retainer. Releveling lasted 3 months, and after removal a fixed retainer was installed on teeth 21, 22, and 23 (). |
A 43-year-old female initially presented to her general practitioner with rigours and a red, swollen right ankle joint. She was treated with oral antibiotics. She rapidly deteriorated, became confused, pyrexial and developed a diffuse erythematous rash of her extremities. She was admitted to the Intensive Therapy Unit (ITU) and required intubation due to profound shock, cardio-respiratory failure and renal failure. Streptococcus pyogenes (b-haemolyticus Group A) was isolated from blood cultures and aspirate of the right ankle joint. A diagnosis of Streptococcal Toxic Shock Syndrome was made and she was started on high dose intravenous antibiotics.\nShe was referred for ophthalmology assessment on day 36 of her admission to ITU as her right eye appeared injected and her right pupil was unresponsive to light. The visual acuity was reduced to hand movements (HM) on the right and was 6/6 on the left. The right eye was comfortable despite significant ciliary injection. She was examined by the bed side using a hand held slit lamp. On examination, the anterior chamber was deep and appeared quiet. The corneal surface appeared irregular with three distinct zones of sub-epithelial opacification. There was no corneal staining with 2% fluorescein. The right iris appeared atrophic and paler in colour. The pupil was fixed in mid-dilation with extensive posterior synechiae at 360 degrees (Fig ). There were no transillumination defects or evidence of rubeosis iridis. There was no hypopyon. The intraocular pressure was elevated measuring 24 mmHg in the right eye. On indirect ophthalmoscopy the view of the fundus was clear with no evidence of inflammatory membranes in the vitreous. The retinal vessels appeared normal and there were no areas of intraretinal haemorrhage or pallor. The right optic disc was pale. The left eye was normal except for a small cotton wool spot above the left optic disc. A diagnosis of right optic atrophy in association with post-streptococcal uveitis was made and she was commenced on g maxidex hourly, g. cyclopentolate tds and g Timolol 0.25%. On day 48 the vision was perception of light (PL) in the right eye. Thinning of the superior sclera was noted and she was maintained on topical steroids (Fig ). By this stage she had undergone extensive limb amputations including two below-knee amputations and bilateral amputations of all digits due to extensive vasculitic necrosis. The medical team were reluctant to treat her with oral steroids due to the risk of secondary infections in the healing wounds.\nBy the fourth month a large anterior staphyloma had formed in the right eye between 11 and 4 o'clock positions (Fig ). The eye remained comfortable and she was treated with g.maxidex qid and g timoptol 0.25% bid. At the last review, six months after presentation, the eye was comfortable. The vision remained PL on the right and 6/6 on the left. The intraocular pressure was 12 mmHg on g timoptol 0.25% b d on the right. Specific immunological tests conducted 5 months following the septicaemia showed normal levels of IgA, IgB, IgC, CD19/B cells and CD16/K cells. Pneumococcal antibodies were noted to be below the normal range. |
An 89-year-old Caucasian woman presented to our outpatients clinic with a left-sided neck lump which she had had for six months. It had started as a small pea-sized lump and developed into a 3 cm × 4 cm smooth, fixed mass (Figure ). It had grown relatively slowly and the woman has been otherwise healthy throughout. She reported no pyrexia, dysphagia, weight loss, night sweats or hoarseness. Accompanied by her niece, our patient made it clear that, if it was malignant, she wanted it treated conservatively.\nA further history revealed that she had a three-year history of recurrent neck swellings that progressed to abscesses, continuously discharging and healing slowly.\nOn closer physical inspection, there was visible scarring from a previous abscess approximately 5 cm inferior to this mass on her left side. As can be seen in Figure and Figure , on our patient's right side there was an old, slowly healing abscess that has been present for over a year.\nOur patient had always enjoyed good health, with the only other significant past medical history being a splenectomy, resulting in taking life-long daily penicillin. The abscesses were treated by her general practitioner with recurrent antibiotics and frequent dressings. Previous swabs from the neck abscess, sent by her general practitioner, did not grow the tuberculous organism.\nOur patient agreed to have further investigations. A chest X-ray and fine needle aspiration (FNA) were performed. The chest X-ray showed chronic fibrotic changes and no evidence of TB infection. The FNA contained necrotic debris and foam cells; acute inflammatory cells were not prominent and granulomata were not seen. Ziehl-Neelsen staining was performed and no acid-alcohol fast bacilli were seen. The features found represented a partially treated skin abscess but the negative staining did not exclude the possibility of TB. Part of the aspirate was submitted to microbiology for conventional and TB culture.\nThe differential diagnosis included neoplasia, tertiary syphilis, deep fungi (for example, sporotrichosis, actinomycosis) and chronic granulomatous disease.\nThree weeks after submitting the aspirate to microbiology for culture, TB was confirmed. The infectious disease team took over our patient's care immediately. Our patient underwent various tests, including liver function tests, urea and electrolytes test, HIV screening and visual acuity prior to commencing the anti-TB regime.\nOur patient was started on ethambutol 700 mg to be taken in the morning, rifampicin 450 mg, isoniazid 300 mg, pyrazinamide 900 mg and pyridoxine 20 mg. Within two months she showed a significant improvement, and after four months there were visible scars only. |
A 43-year-old previously healthy Sri Lankan Sinhala man was admitted with an acute febrile illness lasting for three days. He had high fever associated with severe myalgia, arthralgia and headache. He also had vomiting associated with a severe loss of appetite for two days. There was no photophobia. He did not complain of cough, chest pain or shortness of breath. There was no dysuria, increased frequency of urination, loin pain, diarrhea or abdominal pain. He did not have any bleeding manifestations. He had no history of skin rash or joint swelling. He had not noticed any significant reduction of urine output and his urine color was normal.\nOn examination, our patient was febrile and appeared ill. He was not icteric and not pale. There was no cervical lymphadenopathy. There was a mild conjunctival injection but no conjunctival hemorrhages. There were no peripheral stigmata to suggest bacterial endocarditis. Neck stiffness and Kernig's sign were absent. There were no features of joint inflammation but his muscles were mildly tender. No skin rashes or eschars were observed.\nA cardiovascular examination found our patient to be tachycardic with a regular pulse rate of 104 beats per minute. His jugular venous pressure was not elevated. His blood pressure was 110/68 mmHg with no postural decline. His heart was in dual rhythm and there were no murmurs. His apex beat was not deviated.\nHe was not dyspnoic and had a respiratory rate of 16 breaths per minute. His breath sounds were symmetrically vesicular and there was no evidence of consolidation. No rhonchi or crepitations were observed.\nHis abdomen was soft to superficial palpation. There were no abnormal masses on deep palpation and his liver and spleen were not palpable, nor were there any ballotable masses. There was no free fluid.\nA nervous system examination revealed an alert and conscious middle-aged man who was mildly distressed due to headache and myalgia. A cranial nerve examination and neurological examination did not reveal any abnormality and he had a normal gait.\nBasic laboratory investigations were sent and our patient was initially managed as having an unspecified viral fever pending results of the investigations. A full blood count revealed his hemoglobin level to be 13.4 g/dL, his white blood cell count (WBC) 4400/μL (neutrophils, 76%; lymphocytes, 24%) and a platelet count of 48,000/μL. His serum creatinine was 83 μmol/L, serum sodium was 141 mmol/L and potassium was 3.7 mmol/L. His liver enzyme results were as follows: aspartate transaminase, 153 U/L, alanine transaminase, 207 U/L; and alkaline phosphatase, 387 U/L. His total protein level was 70 g/dL with albumin of 43 g/dL. His international normalized ratio was 0.97 and activated partial thromboplastin time 32 seconds.\nThe initial investigations were compatible with dengue fever. Blood was taken to screen for the dengue antibody and blood cultures were sent. His fluid intake was maintained according to the national guidelines on dengue fever, as there was a widespread dengue epidemic with hundreds of patients getting admitted daily. His fever was treated symptomatically with paracetamol.\nHis fever settled on the fourth day of fever and our patient was symptomatically better. However, he developed mild slurring of speech six days after the onset of fever. On the seventh day, he had marked slurring of speech and he found it difficult to walk due to severe unsteadiness. An examination revealed a bilaterally impaired finger-to-nose test and heel-to-shin test. He had dysdiadochokinesia and an ataxic gait. There was no nystagmus. An examination of the fundus of his eye did not reveal any abnormality. An examination of his cranial nerves continued to be normal. There were no peripheral sensory or motor deficits. His deep tendon reflexes and plantar reflexes remained normal.\nDue to the sudden onset of bilateral cerebellar signs, an urgent non contrast computed tomography scan was performed. There were bilateral hypodense areas in his cerebellum but his ventricles and cerebral hemispheres appeared normal. A magnetic resonance imaging (MRI) scan of his head was performed, which revealed bilateral diffuse hyperintense areas in his cerebellar hemispheres spreading across the vermis (Figures and ). The appearance in the MRI scan was compatible with acute postinfective cerebellitis. Serological studies were performed for the possible viral etiologies that can cause cerebellitis. Hepatitis B and C serology, herpes simplex virus serology and Japanese encephalitis immunoglobulin M were negative. An enzyme linked immunosorbent assay (ELISA) for human immunodeficiency virus was negative. Dengue virus immunoglobulin M (IgM) and immunoglobulin G (IgG) were both detected in our patient's serum on day six after the onset of the symptoms. EBV IgM antibody was detected in his blood by ELISA on the eighth day of illness. Serological studies for Salmonella typhi and paratyphi were negative. A cerebrospinal fluid (CSF) analysis showed a WBC of 10 cells/μL (lymphocytes, nine cells and neutrophils, one cell), red blood cells 1 cell/μL, proteins 0.88 g/dL and sugar 3.1 mmol/L with a random blood sugar of 5.4 mol/L, all of which were compatible with viral central nervous system infection. Cerebrospinal fluid cultures were negative and viral studies were negative for Herpes simplex virus, EBV antibody and Japanese encephalitis virus. Dengue IgG was detected in his CSF while IgM was negative. Dengue virus non-structural protein 1 antigen was not detected in his CSF.\nDependent on the clinical findings and serology, a diagnosis of post-infectious cerebellitis was made. Our patient was managed with maximum supportive care and physiotherapy combined with gait training. Speech therapy was also commenced. At discharge two weeks after the onset of symptoms, our patient was able to walk without support and his speech was markedly improved. His platelet count, WBC and differential counts were within normal limits at the time of discharge. |
A 56-year-old woman was admitted as an emergency, after a sudden loss of consciousness. The multislice CT (MSCT) of the brain performed in a local medical centre showed the presence of intracerebral hematoma 7x4cm in the temporobasal region, with the penetration of blood into the venticular system (Fig. ). On admission, her Glasgow Coma Scale (GCS) score was 8 with right-side hemiplegia and sensorimotor aphasia was observed. Urgent angiography revealed a duplicated left MCA, which merged in the area of the M2 segment and then immediately divided in the dorsal and ventral branch. In the dorsal branch of the MCA duplication, 6x4mm aneurysm was detected. In addition, in the area of the ICA bifurcation itself, between the dorsal branch of the DMCA and the origin of the left ACA, a 4x3mm aneurysm and another aneurysm on the distal part of the left ACA were detected (Fig. a). Emergency surgical intervention was performed by using the pterional approach to the left, with the opening of the Sylvian fissure. The intraoperative finding was in correlation with the angiographic finding. A clear MCA duplication was found, with both branches of the MCA and the initial segment of the ACA being of almost identical diameters (Fig. b). The branches of the DMCA merged at the level of the insular segment of the MCA and, immediately thereafter, the normal branching of the MCA in the dorsal-frontal and ventral-temporal branches was found. In the central part of the dorsal branch of the DMCA, the 6x4mm ruptured aneurysm was located. It had a perforating branch arising from the neck. Two smaller aneurysmal changes were observedone in the ICA bifurcation area between the initial segment of the ACA and the dorsal branch of the DMCA, and another one on the distal part of the ACA (Fig. b). All three aneurysms were clipped. At the very origin of the ICA bifurcation, in the area between the MCA duplications, a caruncule was observed with the perforating branches arising from it. This lesion was wrapped. The intracerebral hematoma was removed. A second angiogram was performed 7 days after surgery, the aneurysms were occluded but there was evidence of vasospasm (Fig.c). In the postoperative period, recovery of consciousness occurred with a preoperative neurological deficit in the form of hemiplegia and motor aphasia. After stabilization, the patient was transferred to a rehabilitation center. Examination performed after 6 months showed hemiparesis and aphasia. |
A 67-year-old, 85 kg man with a prior history of left anterior descending artery (LAD) stent placement was brought by emergency medical services (EMS) to the emergency department (ED) of an academic, community-based hospital. He complained of numbness in his left arm that radiated into his chest. He took 325 mg of aspirin 20 minutes prior to EMS arrival, and EMS gave a single 0.4 mg sublingual nitroglycerin while in transport with full relief of pain. Electrocardiogram (ECG) performed by EMS showed normal sinus rhythm. As the patient was undergoing his initial nursing assessment, he reported that he “felt funny;” his upper extremities began to shake, and then he became unresponsive with agonal respirations, followed by apnea. At this point, no pulse was present and the monitor displayed VF. Chest compressions were started and he received biphasic defibrillation at a dose of 200 J. The first attempt at intubation was esophageal, so the endotracheal tube was promptly removed and ventilation resumed via bag-valve mask (BVM) with excellent chest wall rise. The resuscitation continued with administration of epinephrine 1 mg intravenous bolus approximately every three minutes with four total doses given. In addition, he received a total of 450 mg of amiodarone. The patient received a total of five defibrillation shocks, the first four at 200 J and the fifth at 300 J, biphasic.\nAfter failing to successfully terminate the VF in the first 15 minutes, it was decided to attempt dual axis defibrillation and esmolol administration, so a second defibrillator was brought to the room. A STAT request was made for pharmacy to send esmolol. The paddles of the second defibrillator were placed in an anterior – posterior central position (). Coordination of dual discharge using the original pads and the additional second device and pads occurred “on the count of 3,” and 300 J were simultaneously delivered from each device in the 15th minute of resuscitation. There was no change from VF with this intervention, so CPR continued. While cardiopulmonary resuscitation (CPR) was performed, the patient received a bolus of 80 mg of esmolol IV push and an infusion of 0.1 mg/kg/hr was initiated at the 18th minute of the resuscitation attempt. After allowing time for the esmolol to circulate with CPR, there was persistent VF, and a second simultaneous dual defibrillatory shock was delivered after 21 minutes of resuscitation in the same manner as the first. With that attempt, there was return of spontaneous circulation with a room air pulse oximetry of greater than 90%.\nA second attempt at intubation was initiated at the 23rd minute of the resuscitation attempt, but was aborted when the patient became more alert with the insertion of the laryngoscope. Following resuscitation, the ECG demonstrated atrial fibrillation with 2–5 mm ST elevations in leads I, AVL, and V2–V6 consistent with an ST-segment elevation myocardial infarction (STEMI). Laboratory analysis showed a mild hypokalemia, mild elevation in Troponin I, and mild anemia. A repeat ECG approximately 30 minutes after the resuscitation, and just prior to going to the catheterization laboratory, was still consistent with a STEMI (). At this time the patient was awake, speaking in full sentences, and was breathing room air with stable vital signs. He received a heparin bolus and drip and was successfully transferred to the catheterization laboratory where, with minimal sedation, he was found to have a mid-left anterior descending (LAD) lesion. A drug-eluting stent was placed.\nThe patient had an otherwise uneventful inpatient stay and was discharged on hospital day 4. He was seen again one week later in the outpatient cardiology clinic. He complained of some chest wall soreness, and mild dyspnea on exertion, but otherwise felt well. At follow up with the patient after completion of cardiac rehabilitation, he had no known long-term sequela and was riding his bicycle over eight miles a day. He provided permission for this case report. |
A 23-year-old primigravida at 17 weeks of gestational age presented to the emergency department complaining of worsening cough with intermittent hemoptysis, shortness of breath, and chest pain which had been present for several weeks and exacerbated by light physical activity. Her past medical history was significant for SLE with secondary Sjögren's syndrome diagnosed 6 years before based on findings of arthritis, malar rash, positive antinuclear antibodies (ANA), elevated anti-double-stranded DNA (anti-dsDNA), and the presence of anti-Ro/SSA antibodies. She had no prior surgery and was not taking any medications. She reported no SLE flares in the past few years and had discontinued hydroxychloroquine (HCQ) approximately one year earlier. Her obstetrician referred her for a maternal-fetal medicine consultation in early pregnancy, at which time she acknowledged that she was no longer under the care of a rheumatologist. She did not receive any risk assessment or counseling prior to conception. Two months prior to pregnancy, her serum creatinine was 0.74 mg/dL and her systolic and diastolic blood pressures ranged from 100 to 120 mm Hg and from 70 to 80 mm Hg, respectively. Of note, she was treated with antibiotics for pneumonia three times in the past 14 months and was known to have a recent negative purified protein derivative (PPD) skin test.\nUpon arrival to the hospital, she was awake and alert and in no apparent distress. Her heart rate was 116 beats per minute. She was afebrile and normotensive with a respiratory rate of 16 breaths per minute and an oxygen saturation of 100% on room air. Abdominal exam noted a gravid, nontender uterus. Her respiratory effort appeared normal and her lungs were clear to auscultation. A subtle malar rash and diffuse, white ulcerative plaques were visualized on the buccal mucosa and palate. Bilateral lower extremity pitting edema was noted. The physical exam was otherwise unremarkable. Abdominal ultrasound demonstrated an intrauterine pregnancy with a fetal heart rate of 150 beats per minute. Laboratory evaluation was significant for a hemoglobin and hematocrit of 6.1 g/dL and 18.5%, respectively. The white blood cell (WBC) count was 3,300/uL and the platelet count was 101,000/uL. Serum creatinine was 1.04 mg/dL and urine protein/creatinine ratio was 4.7. A 24-hour urine collection contained over 3 grams of protein. Antiphospholipid antibody testing was performed. On hospital day (HD) 1, anticardiolipin IgG and IgM were 24 GPL and 8 MPL, respectively, and repeat testing on HD2 yielded 34 GPL and 11 MPL, respectively (medium or high titer typically defined as >40 GPL or MPL). Anti-beta2-glycoprotein I antibody screen was negative. Lupus anticoagulant assays, which included dilute Russell viper venom time (dRVVT) and silica clotting time (SCT), were negative. An electrocardiogram (EKG) showed sinus tachycardia. Chest radiography featured indistinct increased markings and a patchy opacity in the right lower lung (). Chest computed tomography angiography (CTA) found no evidence of pulmonary embolism but did reveal a patchy infiltrate in the right middle and lower lobes with small bilateral pleural effusions and enlarged hilar lymph nodes (). After initial evaluation by the obstetrical and emergency medicine teams, consultations were requested with pulmonology, infectious disease, rheumatology, hematology, and nephrology to assist in further management. The decision was made to admit the patient for treatment of suspected community-acquired pneumonia (CAP), new-onset nephrotic syndrome likely secondary to lupus nephritis, and an active SLE flare associated with pancytopenia, malar rash, oral ulcers, and lymphadenopathy.\nThe patient was started on intravenous ceftriaxone and azithromycin for CAP and HCQ and pulse methylprednisolone for the SLE flare. Thromboprophylaxis was initiated with enoxaparin. She received a transfusion of two units of packed red blood cells (PRBCs) for severe anemia described as normocytic and direct coombs test positive. Indirect bilirubin and lactate dehydrogenase (LDH) were increased, serum haptoglobin was decreased, and a peripheral smear showed a few schistocytes and some spherocytes; these findings were most consistent with autoimmune hemolytic anemia. Overall, her pancytopenia was thought to be multifactorial. In the coming days, there was evidence of continued hemolysis despite steroid administration with a persistently elevated LDH and low serum haptoglobin (<20 mg/dL), as well as repeat peripheral smears demonstrating numerous schistocytes. Of note, a repeat direct coombs test was negative. Collectively, these findings were concerning for other (nonimmune) causes of hemolysis such as microangiopathic hemolytic anemia (MAHA) or thrombotic thrombocytopenic purpura (TTP). A normal ADAMTS13 activity level subsequently ruled out the latter.\nOn HD5, the patient was started on supplemental oxygen via nasal cannula after it was noted that her oxygen saturation on room air decreased to below 90%. Over the next few days her oxygen requirements gradually increased to 6 L/min. Pulmonary edema was suspected based on diffuse b-lines on lung ultrasound and repeat chest radiograph noting increased bilateral pleural effusions. All intravenous fluids were discontinued, and furosemide was administered. Improvement in lower extremity edema and good urine output were noted. However, she continued to experience shortness of breath with oxygen saturation as low as 80% on supplemental oxygen. A repeat chest CTA noted progressive ground-glass opacities bilaterally (), which, in the setting of continued hemoptysis, increased concern for diffuse alveolar hemorrhage (DAH). On HD8 she was transferred to the intensive care unit for worsening hypoxia despite diuresis, antibiotic therapy, and steroids. Therapeutic plasma exchange (TPE) was performed with a plan for 4 additional sessions. Fiberoptic bronchoscopy with sequential bronchoalveolar lavage (BAL) demonstrated grossly hemorrhagic fluid consistent with DAH.\nGiven the severity of maternal disease, poor prognosis for the fetus, and recommendation for more aggressive therapy, the patient was counseled for termination of pregnancy. The patient understood that the risk of early-onset preeclampsia and extreme preterm delivery was high even if her pulmonary and renal status were to improve significantly. She was induced and delivered vaginally on HD10. Cyclophosphamide, an alkylating agent associated with gonadal toxicity and teratogenicity, was administered after a suboptimal response to steroids and TPE.\nIn the first few days after delivery, her blood pressures were persistently elevated (≥140/90 mm Hg) but generally below severe range (160/110 mm Hg). A repeat 24-hour urine collection on HD 14 was found to have a total protein of more than 17 grams. Serum creatinine increased to 1.02 mg/dL. On HD22, a kidney biopsy was performed, which revealed lupus nephritis, diffuse global proliferative type, class IV-G (A), and an active thrombotic microangiopathy (TMA) in several arterioles and glomerular vascular poles. The patient was discharged home on HD25 on prednisone and HCQ for lupus, losartan for hypertension, and furosemide for edema associated with nephrotic syndrome. She was also to continue cyclophosphamide infusions outpatient.\nLess than two weeks after discharge, the patient was readmitted with symptomatic anemia and progressive lower extremity edema. She was also noted to have worsening hypertension (systolic blood pressure 160–200 mm Hg), acute kidney injury (creatinine 1.7 → 2.5 mg/dL), and worsening thrombocytopenia (platelet count 81,000 → 52,000/uL). Serum LDH increased to 1,028 U/L. These findings were suspicious for an SLE flare-induced TMA exacerbation versus atypical hemolytic uremic syndrome (aHUS). Pulse dose steroids were again administered. Nicardipine infusion was started for blood pressure optimization. Approximately one hour after a transfusion of PRBCs was started, the patient was found to have altered mental status. She then experienced a series of generalized tonic-clonic seizures in quick succession without recovery between episodes. This was followed by a postictal state. Lorazepam and levetiracetam were administered. She was intubated and mechanically ventilated for airway protection and transferred to the ICU for status epilepticus. Urgent brain MRI demonstrated symmetrical bilateral hyperintensities on T2-weighted imaging and fluid-attenuated inversion recovery (FLAIR) sequences consistent with cerebrocortical edema (); there was no evidence of acute infarct or hemorrhage. Neurology was consulted. Posterior reversible encephalopathy syndrome (PRES) and neuropsychiatric SLE (NPSLE) were highest in the differential diagnosis. Eclampsia was thought less likely at more than 3 weeks postpartum, after delivery of a 17-week fetus. After an inadequate hematologic response to pulse dose steroids, eculizumab, a monoclonal antibody and complement inhibitor, was started. Over the next few days, the platelet count and LDH began improving, suggesting a response to this treatment. The patient received a total of 5 units of PRBCs and 3 doses of eculizumab and was successfully extubated on HD5 after readmission. Repeat antiphospholipid antibody testing was performed. Anticardiolipin IgG and IgM decreased to 4 GPL and 3 MPL, respectively. Anti-beta2-glycoprotein I antibody screen and lupus anticoagulant functional assays remained negative. She was discharged home on HD13 on labetalol and amlodipine for blood pressure control, HCQ and prednisone for lupus, levetiracetam for seizure prophylaxis, and cefuroxime for meningitis prophylaxis given that eculizumab increases susceptibility to Neisseria infection.\nOver the next several months, she experienced continued seizure activity and frequent headaches which required intermittent hospitalization. |
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. |
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