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A 49-year-old Caucasian male presented with a two-week history of sinusitis, nonproductive cough, and sore throat, which progressed to a fever up to 38.9 °C unrelieved by acetaminophen. The patient also experienced generalized myalgia, and had a two-day history of decreased appetite, diarrhea and frontal headache. He tested positive for SARS-CoV-2 by polymerase chain reaction (PCR) assay and was quarantined and medically managed at home for seven days prior to hospital admission. He was admitted due to hypoxemia with oxygen saturation in the low 80 s.\nThe patient’s past medical history was significant for mixed connective tissue disease, rheumatoid arthritis, and systemic lupus erythematosus. The patients’ rheumatological symptoms were controlled by a combination of methotrexate weekly and rituximab every six months since 2008. However, the prolonged course of rituximab resulted in chronic pansinusitis which led to a diagnosis of acquired humoral deficiency. The patient was referred by an otolaryngologist for an immunological evaluation because his chronic pansinusitis did not resolve with septoplasty and bilateral sinus surgery followed by several rounds of clarithromycin. His laboratory results revealed poor humoral function as demonstrated by peripheral lymphopenia with zero absolute CD19 count with an absence of IgA and IgM, but a normal level of IgG (). The IgG serology showed no titer response following a pneumococcal vaccination. Because rituximab was the key in management of his rheumatological conditions, intravenous immunoglobulin (IVIG) was initiated. Initially, the IVIG treatment and dosage were targeted at 400 mg/kg per dose every 4 weeks, increased to 600 mg/kg per dose every 4 weeks, then eventually to 600 mg/kg per dose every 3 weeks along with prophylactic azithromycin daily to effectively manage his chronic sinus infections. The patient’s last IVIG and last dose of rituximab was seventeen days prior to hospitalization and two days prior to the start of a non-productive cough.\nOn admission, the patient presented with tachypnea and visible respiratory distress. His pulse oximetry was 96% on 2 L/min of oxygen via nasal cannula. The vital sign measurements were as follows: temperature of 37.6 °C, blood pressure of 119/87 mmHg, heart rate at 82 beats per minute, and respiratory rate at 20 breaths per minute. The auscultation of the lungs revealed fine bibasilar inspiratory crackles with symmetric chest expansion. The initial chest X-ray revealed cardiomediastinal silhouette enlargement with increased pulmonary vascularity and mild bibasilar atelectasis. The complete blood count demonstrated leukopenia and lymphocytopenia, which were both normal in January 2020 (). At this time, the patient was experimentally given hydroxychloroquine and azithromycin, but the lack of pulmonary infiltrate on the initial chest X-ray did not qualify him for the remdesivir trial.\nOn hospital day four, the patient was transferred to the intensive care unit due to worsening hypoxemia. The laboratory results in demonstrated persistent leukopenia and lymphocytopenia with a new elevation of IL-6, C reactive protein, D-dimer, ferritin, glucose, alanine aminotransferase, aspartate transaminase, and lactate dehydrogenase levels. On day five, IVIG was given at 600 mg/kg followed by another 600 mg/kg dose two weeks later. Supplemental oxygen via high flow nasal cannula was initiated at 15 L/min and titrated up to 20 L/min by day twelve. A repeat chest X-ray revealed multifocal ground-glass consolidative opacity that was more significant in the left lower lung with worse aeration compared to prior study.\nHis respiratory condition was complicated by abdominal distention with diarrhea and ileus, necessitating a nasogastric tube placement. An abdominal computed tomography study was completed on day twenty-six revealing thickening of the colon. On day twenty-seven, he was discharged from the intensive care unit. Cultures of Histoplasma, Pneumocystic jiroveci, Staphylococcus aureus, Rhinovirus, Metapneumovirus, Adenovirus, and Clostridium difficile were found negative for respiratory and gastrointestinal symptoms; however, SARS-CoV-2 remained positive via PCR assay. A computed tomography of the chest was performed on day thirty-three showing diffuse patchy infiltrates in both lungs, worse in the right upper and middle lobes as shown in . The patient was discharged to home after forty-one days of hospitalization with oxygen supplementation only with ambulation and a complete resolution of diarrhea despite still being tested positive for SARS-CoV-2. The patient eventually was cleared of SARS-CoV-2 after three different PCR assays at three weeks post hospital discharge. |
Case 3 was a 68 years old male with two rather small and slowly growing peripheral tumours in the lung, one in the right upper lobe (5 mm in size) and one in the right lower lobe (1.8 cm in size). There was no evidence of lymph node or distant metastases or malignant pleural exudate. Both tumours were surgically removed with wedge resections and were TTF-1 positive non-mucinous adenocarcinomas. The growth pattern was 50% papillary, 25% acinary, 20% lepidic and 5% mucinous (mostly lepidic) in the larger lower lobe tumour and 60% acinary and 40% lepidic in the upper lobe tumour. See Fig. . Both tumours exhibited some spreading through air spaces (STAS) and the tumour in the lower lobe also had pleural invasion but without any detected extension to the pleural surface (i.e. PL1). Furthermore, in the lower lobe there were a couple of additional, separate very small foci (<1 mm) of minimally invasive adenocarcinoma with lepidic and acinary growth, also detected during the surgical procedure.\nThe findings of the lower lobe was judged to be a tumour with metastasis within the same lobe (pT3), but it was discussed whether the tumour of the upper lobe represented a separate synchronous tumour (pT1a) or a metastasis (pT4). Targeted NGS analyses showed the same KRAS and SMAD4 mutations in both the tumour of the upper lobe and one of the tumours in the lower lobe. See Table for full NGS data. IHC staining for ALK was negative in both tumours. A later analysis of one of the very small tumours of the lower lobe (not of relevance for the clinical handling of the patient) revealed the same KRAS mutation while the SMAD4 mutation was not seen.\nConsequently, although the patient had a very small separate tumour with partly different growth pattern in the upper lobe and no lymph node or distant metastases, the case was considered to be a metastasis to another lobe (pT4) based on the identical molecular genetic profile. The patient suffered from severe renal insufficiency which limited the extent of surgical and chemotherapy treatment. However, the patient was still alive 18 months after surgery. At the time, a CT scan showed suspicious growth of a lung nodule and mediastinal lymph node enlargement, but EBUS-guided cytology was negative. |
A 30-year old woman, primigravida, was referred to the Fetal Medicine Clinic of our department at 37 weeks of gestation for evaluation of a fetal scrotal mass visualized on ultrasound. Previous obstetric ultrasound examinations at 12, 21, and 30 weeks had not shown any evident fetal abnormalities. Fetal karyotyping had not been performed.\nThe ultrasound examination was performed using a 4.0–8.0 MHz multifrequency 3D transducer (Voluson 730 Expert, General Electric). Fetal biometry was within the normal ranges for the gestational age with normal biparietal diameter and head circumference and femur length and abdomen circumference. The amount of amniotic fluid was in the normal range.\nThe study of the scrotum revealed an echogenic mass (50 × 46 mm) with mixed echostructure and regular walls, containing few small echo-free cystic areas in the right side () and hydrocele in the contralateral side (). During the examination, no intestinal peristalsis was seen in the scrotal mass and there was no evidence of an associated bowel obstruction or meconium peritonitis. No other fetal abnormalities were found. Color Doppler examination showed the presence of regular vessels in the testicular sac.\nA pediatric surgeon counselling was requested after our examination. The consultant advised for clinical evaluation at birth, and emergency surgery in case of complications.\nThe following ultrasound examination, performed a week later, found no modification in size of the mass and an increasing number of echo-free cystic areas, without signs of complications (). In addition, bowel peristalsis was observed in the scrotal mass allowing us to make diagnoses of inguinal scrotal hernia.\nA caesarean section was performed with no complications at 38w + 6d, for podalic presentation.\nThe neonate weight was 3700 g with an Apgar score of 8 and 9 at 1 and 5 minutes, respectively. The pediatric examination confirmed the presence of right inguinal scrotal hernia ().\nThe neonate was dismissed on the fourth day after birth and was admitted in the Pediatric Surgery Department of our hospital, where, on day 10, a laparoscopic hernia repair was performed without complications. During surgery, a similar contralateral defect of the inguinal canal was observed and repaired. This was the cause of the left hydrocele, seen in utero ().\nNo bowel symptoms on follow-up examination at 3 months were found. |
A 41-year-old female with extensive medical history including hypertension, lupus nephritis, anti-phospholipid antibody syndrome, coronary artery disease, and previously treated Libman-Sacks endocarditis presented to the ED with persistent lightheadedness for one week and two days of recurrent nausea and vomiting with decreased oral intake. She had a pertinent surgical history of coronary artery bypass graft and aortic valve replacement secondary to the endocarditis. She was anticoagulated on warfarin and required hemodialysis. The patient also reported two days of right calf pain that occurred only when ambulating. She did not complain of chest pain, back pain, or abdominal pain.\nThe patient’s initial vital signs included a blood pressure of 171/91 millimeters of mercury, heart rate of 92 beats per minute, respiratory rate of 18 breaths per minute, and oral temperature of 37.1 degrees Celsius. Her oxygen saturation was 99% on room air. On initial evaluation in the ED, the patient appeared in no distress and was alert and oriented to person, place, and time. She answered questions appropriately, and her neurologic examination showed no focal weakness or sensory deficits. Lungs were clear and cardiac exam was noted as regular rate and rhythm without murmur. The patient’s abdomen was soft, non-tender, and non-distended. Her lower extremities were warm and well perfused with normal range of motion and no swelling or calf tenderness. Her peripheral pulses were intact and symmetric bilaterally.\nBased on her history and physical examination, the treating physicians were most concerned for an acute viral process or foodborne illness. Nonetheless, given her complaint of right calf pain in the context of a chronic pro-coagulant state, they decided to evaluate for a DVT in the right lower extremity. The patient underwent a POCUS two-point compression examination of the right leg, which showed normal compression of the right femoral and popliteal venous systems. However, an abnormal intraluminal echogenic signal was seen in the right femoral artery, which had the appearance of an intimal flap.\nColor Doppler was used to confirm differential flow on either side of the flap (). The ultrasonographers proceeded to interrogate the abdominal aorta, and a dissection flap was noted in the transverse view (). A computed tomography (CT) angiogram of the chest, abdomen, and pelvis with run-off to the lower extremities was then performed, which showed an intimal flap starting in the distal abdominal aorta and extending into the right common iliac, external iliac, and superficial femoral arteries ().\nA subsequent review of the patient’s medical chart showed that she had been admitted to our institution one month prior for acute coronary syndrome and had been taken to the cardiac catheterization suite three times during that hospitalization. The hospital record noted that she was canalized in her femoral region three times, twice via her left femoral artery and once via her right femoral artery. The patient was assessed by the vascular surgery team in the ED. Their impression was this dissection was iatrogenic given her history of recent catheterization, and they recommended strict blood pressure control and admission. Given her extensive and complicated cardiovascular history she was ultimately admitted to the cardiac intensive care unit. Her blood pressure medications were adjusted, and she was discharged home three days later. |
A 24-year-old Caucasian man, a full-time student, 1.8 m tall, 77 kg in weight, with no significant medical history, a non-smoker, taking no medications and with no substance misuse, was riding a motorcycle while wearing a helmet; he collided with a moving automobile and was ejected over 30 m into the air. He sustained multiple injuries including a large chest wall avulsion and a severe partial amputation of the left arm. The limb was not salvageable, requiring amputation, with a small residual fragment of the left scapula remaining (Figure ). Left scapulothoracic dislocation and severed left brachial plexus were also found intra-operatively. His head, right arm and lower extremities were grossly intact.\nHe received 10 weeks of acute care in our surgical medical unit, where surgical intervention included repair of the chest wall and internal organs, after which he was transferred to the acute rehabilitation unit where, almost immediately, phantom limb pain became his major issue.\nHe reported his pain episodes as variable in number, ranging from three to six per day. Described as searing, aching or cramping as if his missing hand was clenched in a fist formation, the pain episodes often occurred at random intervals during the day, ranging from 15 minutes to up to an hour and a half. On average, he rated the pain at between 8 to 10 out of 10 on a visual analog scale (VAS).\nAs his entire left upper limb was missing, including the shoulder and parts of the clavicle and scapula, 'stump' pain did not actually apply to his description. Instead, he consistently experienced the feeling that his left fist was severely clenched and he could not release it from the cramping that became a burning, searing pain.\nThis persisted despite a series of aggressive pain management methods through the administration of naproxen 250 mg three times a day, tramadol 50 mg four times a day, extended release morphine 150 mg twice a day, hydrocodone/acetaminophen 5/500 mg every four hours as needed, lidocaine patches (two patches every 24 hours), gabapentin 400 mg four times a day and the use of a transcutaneous electrical nerve stimulation (TENS) unit. At this point our pain clinic was consulted for possible nerve block, which was deemed not appropriate. The pain was so severe that it affected patient's blood pressure as well. He required treatment with clonidine 0.4 mg twice daily, metoprolol 125 mg twice daily, and lisinopril 20 mg once daily. Over the course of two weeks, it was suggested that the employment of mirror therapy might provide some measure of relief. A vertically supported mirror in a frame was fashioned for easy positioning against his midline chest with him seated in a chair. In leaning slightly forward, he was able to watch the reflection of his right arm during motions as if doing biceps curls, opening and closing the fist, pronating and supinating the outstretched 'arms', while attempting to concentrate on doing these movements as if bilaterally. He performed these maneuvers for 15 minutes at a time at least twice daily. Although not significant in the first week to week and a half, he began to report some decrease in the intensity of the left upper extremity phantom limb pain by the end of the second week of the mirror therapy. He rated his maximal pain as 6 out of 10 on the VAS. All pain medications except gabapentin were gradually discontinued over two weeks of mirror therapy. Gabapentin was decreased to 400 mg three times a day.\nHis blood pressure also decreased after two days of mirror therapy. At the end of the third week he was only on lisinopril 20 mg daily.\nDuring the mirror therapy course his mother participated by clapping her hands in synchrony with his movement of his hand towards the mirror, giving the illusion of not only seeing but also hearing hand clapping. We encouraged this form of auditory feedback and it was continued throughout his acute rehabilitation stay. Although MVF was started initially for the treatment of this patient's PLP, auditory feedback, at first performed unintentionally by his mother, was thereafter simultaneously performed along with the mirror therapy.\nHis other rehabilitation goals were met sooner than initially projected, and he was determined to be appropriate for discharge home with continuation of out-patient mirror and auditory feedback therapy, as well as further out-patient therapy care. |
A 50-year-old male patient presented with a 2-year history of left-sided typical HFS. Painless irregular clonic contraction of the facial muscles began initially in the orbicularis oculi muscle of the lower lid. It gradually spread to other muscles innervated by the facial nerve on the left side of the face, including platysma. The paroxysm was induced or aggravated by emotional tension, stress, and voluntary and reflexive movements of the face. He had significant difficulty in his work and social life despite 2 times of botulinum toxin injection. Medical treatment with carbamazepine (up to 600 mg) and baclofen (30 mg) was not effective. He was referred for surgical treatment. His medical history was unremarkable. His physical and neurologic examinations were normal, including hearing. No tinnitus or discernible noise heard in his left ear was found. Only typical nature of clonic hemifacial spasm was evident. Abnormal synkinesis between the orbicularis oculi and orbicularis oris muscles was found by the electromyographic examination of the blink reflex. Despite typical HFS, there was no discernible vascular structure in the REZ of left facial nerve (). However, a meatal loop of AICA abutting to the cisternal portion of the facial nerve was found.\nUnder the impression of HFS caused by neurovascular compression of distal facial nerve, standard microsurgical procedure was performed as described previously [, , ]. In addition to intraoperative monitoring of BAEPs, LSR, which is an abnormal muscle response demonstrated by EMG recordings from mimic muscles that are innervated by a different branch of the facial nerve [], was also monitored throughout the operation. The entire course of the facial nerve and offending arteries were exposed under microscopic vision. Upon exposure of the REZ of the facial nerve, there was no offending vessel in the REZ as expected (). The distal, cisternal segment of the facial nerve was found to be bent by a meatal loop of the AICA (). A small piece of Teflon felt was interposed between the facial nerve and the meatal loop of the AICA with extreme care not to stretch the internal auditory artery and the distal facial nerve (). After interposition of Teflon felt, LSR immediately disappeared and BEAP was stable also (). The closure of the dura and wound was performed in routine manner. The HFS resolved completely following the surgery. The postoperative course was uneventful with no signs of facial weakness or hearing impairment by pure-tone audiometry. No recurrence of HFS or neurologic sequelae was evident at a 12-month follow-up. |
The patient, a 62-year-old male, came from Hengshui Wuyi county and was referred to our department due to dizziness and gait instability that had persisted for >10 days. The patient staggered from side to side, fell several times due to the gait instability, did not dare to stand and exhibited continuously worsening symptoms. The patient also had a dull expression and hypomnesia. Three years previously, the patient had undergone cardiac carcinoma surgery and was prescribed long-term oral ranitidine and furazolidone. The family members of the patient complained that his food intake had decreased significantly and that he had recently suffered from delusions, following which his appetite had reduced further. The patient had no history of poison contact or drinking or drug abuse.\nPhysical examination of the patient revealed that he was lucid when conscious, but exhibited slurred speech, apathy, cognitive impairment and poor calculation and memory. The bilateral pupils of the patient were round and equal, his light reflex and eyeball motion were normal, and the patient did not exhibit nystagmus. His bilateral frontalis and nasolabial groove were approximately symmetrical, and his tongue was in the center. The muscle strength of the patient’s extremities was grade 5, with normal muscle tone. The patient had no sensory disturbance, and his physiological reflexes were present without pathological reflex. The finger-to-nose and fast alternating movement tests showed the patient to be slightly clumsy, and MRI showed symmetric abnormal signals in the splenium of the corpus callosum (). Blood lipid tests revealed a total cholesterol level of 6.29 mmol/l and a low-density lipoprotein level of 3.96 mmol/l. Routine blood, urine, liver and kidney function and blood coagulation tests showed no obvious abnormalities, and no abnormalities were found with thoracic and abdominal computed tomography. The diagnosis was MBD. The patient was told to consume a diet rich in vitamins to improve the brain blood and oxygen supply, and was prescribed vitamins B1 and B6, methylcobalamin, and folic acid treatments. Two weeks after admission, the slow responses and delusions of the patient had improved markedly. Following discharge, the patient was followed-up for two months. He could walk freely and live on his own. |
A 64-year-old man who had no symptoms was diagnosed with thoracic superficial esophageal cancer that was detected by screening upper endoscopy. He had a history of hypertension. He had also been found to have a vascular abnormality (DAA) as an adult and was observed in an asymptomatic state.\nPhysical examinations showed no unusual findings, and the laboratory examination data, including tumor markers, such as squamous cell carcinoma-related antigen and carcinoembryonic antigen, were all within normal ranges. Chest X-ray demonstrated a widening in the upper mediastinal silhouette, reflecting the superior right aortic arch. An endoscopic examination revealed superficial esophageal cancer located in the left side of the wall in the upper thoracic esophagus and the invasion of the submucosa (Fig. ). A histological examination of biopsy specimens confirmed the presence of squamous cell carcinoma. Enhanced computed tomography showed a swollen lymph node in the right upper mediastinum, which was diagnosed as metastatic (Fig. ). No distant metastasis was detected. Computed tomography also confirmed the DAA. The right aortic arch was dominant, and the descending aorta was located at the right side of the post-mediastinum, as is common in cases of DAA (Fig. ). The patient was therefore diagnosed with upper thoracic esophageal cancer of cT1bN1M0 Stage IIB (UICC-TNM 7th) and a DAA.\nHe underwent neoadjuvant chemotherapy prior to sub-total esophagectomy with three-field lymphadenectomy. The neoadjuvant chemotherapy regimen was 2 courses of 5-FU (800 mg/m2) and cisplatin (80 mg/m2) every 3 weeks.\nWe planned to perform radical subtotal esophagectomy with three-field lymph node dissection after neoadjuvant chemotherapy. We first planned to perform cervical procedure in a supine position before the thoracic procedure in order to identify the bilateral inferior laryngeal nerves and avoid causing them injury or inducing palsy. We also planned to perform upper mediastinal lymph node dissection during this preceding procedure because the DAA was expected to interfere with upper mediastinal dissection attempted via either side of a transthoracic approach. We then planned to perform lymph node dissection via a left-thoracoscopic approach below the left aortic arch, as we worried that the right-sided descending aorta might interfere with a right-thoracic approach (Fig. ). The laparoscopic procedure was planned to be performed via an abdominal procedure in a supine position. Reconstruction would use the gastric tube pulled up via the retrosternal route with cervical esophago-gastric anastomosis.\nIn the preceding cervical procedure performed in a supine position, we identified the bilateral inferior laryngeal nerves, which were thought to be recurrent at each side of the aortic arch (Fig. ). After upper mediastinal dissection was performed, the left thoracoscopic procedure in a prone position was performed for middle and lower mediastinal lymph node dissection below the left aortic arch. We first confirmed that the right-sided aortic arch and descending aorta would interfere with the usual right thoracic approach (Fig. a). Upper mediastinum dissection was also deemed impossible via a bilateral thoracic approach because of the bilateral aortic arches and subclavian arteries, as expected preoperatively (Fig. a, b). Postmediastinal reconstruction also seemed impossible. The port position for the left thoracoscopic procedure was set symmetrically to our normal right thoracoscopic procedure for middle to lower mediastinal dissection, as shown in Fig. . No major anatomical findings other than those noted preoperatively were observed during the left thoracoscopic procedure. We were unable to identify where the thoracic duct ascended because of the preservation of the thoracic duct. We were also unable to confirm the details concerning both recurrent laryngeal nerves around each aortic arch.\nThe abdominal procedure in a supine position was performed laparoscopically with the simultaneous cervical procedure for bilateral supraclavicular lymph node dissection. Reconstruction was performed with cervical esophago-gastric tube anastomosis. The gastric tube was pulled up through a retrosternal route as planned. Three-field lymph node dissection and complete resection (R0) were achieved. The operative time was 8 h 9 min, and the total bleeding was 70 ml. No vocal cord palsy was observed on flexible laryngoscopy after the operation.\nThe patient’s postoperative course included minor leakage that was cured conservatively after 2 weeks, and he was discharged at postoperative day 29. The pathological diagnosis was ypT1bN0M0 Stage IA (UICC-TNM 7th edition). The patient was followed for 2 years with no signs of cancer recurrence. |
A 36-year-old caucasian woman was admitted to our hospital due to a moderate to severe retrosternal pain, commencing 24 hours ago, radiating to the back and being aggravated with deep breathing. There was no fever, history of recent viral infection or any other accompanying symptoms. The woman had been suffering for the last 5 months from a low-grade fever, diffuse myalgias and worsening fatigue. She had been fully investigated during the previous month and was considered to suffer from an autoimmune disease, yet no definite proof was available. While waiting for the results of a skin-muscle-vessel and liver biopsy she was empirically set on corticosteroids and was discharged from the hospital. A few days later she presented to us with the previously described pain. From her medical history she had no significant cardiovascular risk factor.\nOn admission she looked ill. She had no dyspnoea and from her physical examination she had sinus tachycardia, normal first and second heart sounds, a fourth heart sound, a mild apical mid-systolic murmur, no pericardial friction rub and clear lung fields. On chest x-ray the heart size was normal and there were not any signs of pulmonary congestion. The admission ECG revealed sinus rhythm, normal QRS axis, a Q-wave at I, aVL and ST-segment elevation at leads I, aVL, V3-V6 with reciprocal ST-segment depression at leads III, aVF (figure ). Cardiac enzyme levels (troponin, creatin phosphokinase-MB) were increased. Echocardiography showed a left ventricle with normal internal dimensions, an apparent regional hypokinesia of the posterior, the middle and apical segments of the lateral wall and a mildly compromised global systolic function.\nThe electrocardiographic and echocardiographic findings, as well as the enzymic activity, were typical of an acute myocardial infarction. However, the young age of the patient, the atypical features of chest pain, the lack of significant risk factors for coronary artery disease and the underlying autoimmune disease, led us to reconsider our diagnosis towards that of acute focal myocarditis. After a lot of consideration, we decided to initiate high-dose intravenous corticosteroid treatment, instead of immediate reperfusion with intravenous thrombolysis (as emergent coronary angiography and possible primary angioplasty were not available in our setting).\nOn the following days ST-segment elevations gradually decreased and negative T-waves appeared at the same leads, following the pattern of acute myocardial infarction (figure ). However, on the third day of hospitalization, a second echocardiogram revealed the presence of a mural thrombus attached to the previously recognized hypokinetic area (figure ). Antithrombotic therapy was initiated (with low molecular weight heparin and acenocoumarol) and three days later the thrombus gradually disappeared (figure ) without any embolic sequela.\nSerial echocardiographic assessment showed that the segmental wall motion abnormality gradually improved and ten days later the global contractility of the left ventricle were normal. Similarly, the cardiac enzymes that were elevated on admission returned to normal, following the pattern of an acute myocardial infarction. The patient underwent coronary angiography, which revealed normal coronary arteries and normal systolic function of the left ventricle.\nA few days after her hospital discharge, the previous taken skin-muscle-vessel biopsy was proved to be diagnostic of polymyositis. Twelve months later, while being on corticosteroids, the patient remains asymptomatic, with a normal ECG (figure ), and a normal echocardiogram. |
A 62-year-old hypertensive, obese, diabetic, male patient with short neck presented with episodic falls, difficulty in walking, and brief loss of consciousness to our hospital. Weakness of both hands and progressive weakness of all four limbs were present since 1 week. The patient was diagnosed as complex partial seizures clinically. There was no history of trauma at the presentation or in the past. The patient had a history of subarachnoid haemorrhage 6 months back for which conservative management was done elsewhere. Magnetic resonance (MR) imaging of the brain showed mild effacement of the sulcal spaces on T1 weighted images (T1WI) and T2 weighted images (T2WI) (Figures and ). There was a positive phase shift and blooming along the sulcal spaces on phase contrast and maximum intensity projection (MIP) susceptibility weighted images (arrow heads in Figures and ) suggestive of superficial siderosis. There was no evidence to suggest the possible source of haemorrhage in the MR images of the brain. The remainder of general physical and systemic clinical examination was also unremarkable. Screening of the spine was done to locate the possible source of the bleeding leading to superficial siderosis.\nCervical spinal magnetic resonance imaging (MRI) revealed an intradural extramedullary mass occupying the anterior intradural space which displayed hypointense signal on T2WI and hyperintense signal on T1WI (Figures , , , and ). Contrast enhancement of the mass was not evidently revealed by visual assessment on postcontrast fat saturated T1 weighted images () due to strong T1 hyperintensity of the mass on unenhanced images. Mild peripheral heterogeneous enhancement of the tumor was verified by the subtracted images (). The lesion was compressing and displacing the spinal cord posteriorly (arrow in Figures , , and ). The PET CT images showed moderately FDG avid extramedullary intradural mass (arrow in ) and no other foci of FDG avidity were noted elsewhere in the body. The patient underwent C3–C5 laminectomy and excision of the intradural extramedullary mass lesion. The Lesion was placed anterior to the cord and was firm, smooth surfaced, and blackish in colour. Retrospectively, examinations of the skin and the fundus of the eye did not reveal any melanotic lesions. Hence the lesion was treated as primary cervical spinal melanocytoma which was confirmed on histopathological examination which revealed diffusely pigmented tumor with peritheliomatous arrangement which obscured the cytological details (H and E 40x) (). After bleaching, tumor had pleomorphic round to oval nuclei with inconspicuous nucleoli and moderate amount of cytoplasm (H and E 40x) (). Tumor on higher magnification had a mild nuclear pleomorphism, moderate to abundant cytoplasm with few containing blackish brown pigment. No significant increase in mitosis (<1 mitosis per 10 high power fields) was seen (H and E 200x) (). The proliferation index Ki-67 was low (<1%) which is diagnostic of melanocytomas. The patient improved symptomatically in the postoperative period. |
A 67-yr-old man presented to the hospital, complaining of dark-colored urine intermittently for approximately 4 yr. He had also experienced intermittent bilateral flank pain over the previous 2 months. His medical history included a total gastrectomy for advanced gastric cancer 2 yr earlier and chemotherapy with paclitaxel and cisplatin. There was no evidence of a radiologic abnormality in the urinary tract at that time or on the most recently obtained follow-up abdominal computed tomography (CT) image. The physical examination of the patient was unremarkable. On laboratory examination, there was microscopic hematuria. The blood chemistry included serum creatinine of 10.7 mg/dL and serum potassium of 5.1 mM/L. Renal ultrasound (US) showed moderate hydronephrosis in both kidneys and dilatation of both upper ureters. It was thought that the bilateral obstructive uropathy was due to direct extension or external compression of both ureters by advanced gastric cancer metastases. Therefore, percutaneous nephrostomy catheters were placed bilaterally on an emergency basis and antegrade pyleograms were obtained. The third day after the urinary diversion, the serum creatinine had decreased to 1.3 mg/dL. The antegrade pyleograms revealed moderate dilatation in both renal pelvises and upper ureters, and the mid to lower ureter could not be visualized, even after 1 hr, implying complete ureter obstruction (). Bilateral retrograde ureterograms (RU) were attempted after a normal cystoscopic examination, which revealed a narrowed ureter caliber bilaterally, suggesting long segmental strictures from the upper to middle ureter bilaterally (). Since the percutaneous nephrostomy tubes would reduce the patient's quality of life and cause a number of problems, such as discomfort and tube displacement, we inserted double-J ureteral stents in both ureters and then we clamped both nephrostomy catheters to ensure proper urine drainage through the ureteral stent. Unfortunately, the patient developed anuria immediately after removing both percutaneous nephrostomy tubes, and this progressed to hydronephrosis and flank pain. Urine cytology from the nephrostomy catheters and both ureteral catheters was negative for malignancy. We could not find any ureteral or peri-ureteral lesion on either side, except for moderate hydronephrosis and dilatation of the ureter on abdominal CT. Surprisingly, ureteroscopic evaluation showed over 20 small, separate FEPs from the middle to upper ureters bilaterally (). Furthermore, on cystoscopy, the lumens of the double-J stents were filled with small polyps without urine passage. We could not remove all of the lesions because the polyps were located diffusely over the entire ureter wall and we could not find any normal ureter mucosa. Furthermore, the ureter lumens were so narrow that we could not resect the polypoid lesions while avoiding ureter injury. We were concerned about whether the remaining ureters would function normally without a long segmental ureteral stricture after the polyps were removed, even if it proved possible to remove all of the lesions. That is why we decided to maintain the percutaneous nephrostomy catheters. Histological examination of the resected specimen showed FEPs consisting of a core of fibrovascular tissue covered by a normal layer of transitional epithelium. Mild chronic inflammatory cell infiltration was seen in the stroma (). |
The patient was a 4-year-old boy and the only child of nonconsanguineous parents, with no family history of inherited or neurological diseases. He exhibited dilated lateral ventricles at 30 weeks of gestation. He was born spontaneously at full term. At birth, he weighed 3085 g (50th percentile), was 48 cm long (25th percentile), and occipitofrontal circumference was 32.5 cm (25th percentile). The perinatal history was uneventful. The patient had a left esotropia and moderate developmental delay and was brought into the clinic at age 4 years. His spontaneous movement, sensation, and the deep tendon reflex of the limbs were normal and symmetric. He had no dysmorphic facial features including ptosis and pupil abnormalities. However, reduced sweating was observed on the whole right side of his body, including the face. His parents were aware of this symptom from infancy. This symptom had not deteriorated and caused the patient no disability. Blood and urine screening produced normal results.\nMagnetic resonance imaging of the brain disclosed cerebellar vermian dysplasia, dysmorphic and hypertrophic basal ganglia with fusion between the putamen and caudate nucleus, not affecting the anterior limb of the internal capsule, moderate hypoplasia of the right brain stem, and ventriculomegaly (-). Diffusion tensor imaging revealed disorganization of the pyramidal fibers (). An electroencephalogram showed a few epileptic discharges in the right occipital regions, but no clinical seizures had been reported.\nThese clinical features are similar to those that have commonly been described in patients with TUBB3 mutations, particularly patients with cortical dysplasia, complex, with other brain malformations type 1. Therefore, the authors performed a mutation screening of TUBB3 after approval from the institutional review board of Yamagata University, Faculty of Medicine, and obtaining written informed consent from the parents. Sequencing was done by polymerase chain reaction–direct sequencing according to the standard methods using originally designed primers. The results showed a missense mutation, c.862G>A (p.E288K), which was not detected in both parents, indicating a de novo mutation.\nSince he showed unilateral hypohidrosis, the authors surveyed his autonomic nerve function. Orthostatic hypotension was not detected in the patient; the heart rate was normal and increased on standing. Thermoregulatory sweat testing was performed by applying a starch and iodine paste to the thigh, which turns purple in the presence of sweat. This test revealed a decrease in sweating on the right thigh. The amplitude of the sympathetic skin response was low on the right thigh, which led to a diagnosis of right autonomic dysfunction associated with reduced sweating (). Tests of heart rate and blood pressure showed normal cardiovascular autonomic functioning (). For cardiac autonomic tests, myocardial metaiodobenzylguanidine uptake was measured and found to be normal. |
An 86-year-old male with a history of seropositive rheumatoid arthritis for 30 years. The treatment for his rheumatoid arthritis included methotrexate initially, but due lack of control, adalimumab 40 mg subcutaneously every 2 weeks was added with excellent control of his arthritis of hands and feet. This lasted for few years. However, he later on experienced activity of disease and he was switched from adalimumab to Tocilizumab 162 mg subcutaneous injections every 1 week. His arthritis was controlled on the latter regimen apart from his shoulders. At this time his lab investigations were normal including complete blood panel (CBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), Liver function test (LFT), Renal function test (RFT), and Bone profiles. He experienced an increasing pain and stiffness in his shoulders, in addition to an increasing limitation of shoulder movement in all directions. His other medical conditions include type 2 diabetes mellitus and hypertension. We are unaware of any history of adhesive capsulitis. His other medications are Methotrexate 10 mg oral once weekly, hydroxychloroquine 200 mg, alendronate 70 mg once weekly. X-rays of shoulders at the time (May 2012 and are shown in ). They showed punctate and linear calcification around the capsule. The patient received multiple intra-articular steroid injections with no notable benefit. Over the years of follow-up visits, his condition progressed, with increasing limitation of his shoulders movements. The Oxford Shoulder Score (OSS) were used to assess degree of pain and disability. Score for both shoulders were 7, indicating a great degree of disability.\n Repeated imaging on March 2017 was performed and is shown in and showed hypertrophic, global plaque-like calcification in the capsule. Meanwhile, X-ray of hands did not reveal any chondrocalcinosis or erosions and knee x-rays showed advanced osteoarthritis. Magnetic Resonance Imaging (MRI) of left shoulder was done in November 2020 and is shown in and revealed severe arthritis with remoulding deformity with extensive capsular calcification, intra-articular loose bodies and large joint effusion resulting in dislocation. In addition, he had severe rotator cuff which atrophy reflects chronic complete tear with retraction and atrophy. The patient was offered surgical intervention, but he refused. |
A 72-year-old woman presented with a 2-month history of mild dyspnea on exertion. A computed tomography (CT) scan showed a low-attenuation defect in the lumen of the right main pulmonary artery (). Pulmonary artery thromboembolism was suspected. Anticoagulation therapy with apixaban was administered; however, the defect in the pulmonary artery did not improve after 4 weeks of anticoagulation treatment. A positron emission tomography (PET) scan revealed the uptake of fluorine-18 fluorodeoxyglucose (FDG) in the intraluminal defect with a maximum standardized uptake value (SUVmax) of 4.35, suggesting a malignant tumor. To confirm the diagnosis and selection of a treatment, we performed endovascular catheter aspiration biopsy of intravascular mass using a 6 Fr Envoy catheter (Codman, Raynham, Massachusetts, USA). Vacuum suction of the mass was performed several times by careful manipulation (). Histological examination revealed pulmonary artery sarcoma. No distant metastasis was identified. A right pneumonectomy with arterioplasty of the pulmonary arterial bifurcation with the use of cardiopulmonary bypass was planned.\nThrough median sternotomy, the surgery was performed. The tumor completely filled the right main pulmonary artery. No invasion to extravascular tissues was identified. Cardiopulmonary bypass was established with standard techniques using cannulas placed in the ascending aorta and the right atrial appendage. The main pulmonary artery was incised. The solid tumor filled the right main pulmonary artery and extended to the main pulmonary artery trunk. The right pulmonary artery and a part of the main pulmonary artery from the supravalvular to the left main pulmonary artery were resected. Macroscopically, the surgical margin was negative. The main pulmonary artery trunk and left pulmonary artery were reconstructed with bovine pericardium. The patient was successfully weaned from the cardiopulmonary bypass, and a right pneumonectomy was performed. The operation time was 429 min, and the cardiopulmonary bypass time was 73 min. The blood loss was 920 mL. The gross specimen showed that yellowish solid mass, 9 × 5 × 3 cm in size, was occluding the right main pulmonary artery (). Histological examination of the tumor revealed intimal sarcoma. Immunohistochemical staining was positive for vimentin, murine double minute type 2 homologue, and alpha smooth muscle actin (), negative for desmin, factor VIII, CD34, and CD31. Tumor cells were invading outside pulmonary artery. A few tumor cells were found at the resected margin of the left distal pulmonary artery. The patient was discharged 28 days postoperatively without any complications. The patient received radiotherapy postoperatively. A series of subsequent chest CT scans did not show signs of recurrence of the tumor 6 months postoperatively. |
A 66-year-old woman presented at a hospital with chest pain. Her history included diabetes, hypertension, and hyperlipidemia. On examination, the patient had a pulse of 100 beats/minute and blood pressure of 150/80 mmHg. Her electrocardiogram, echocardiogram, and blood test results were normal. Multislice computed tomography (CT) showed a saccular LMCA aneurysm and significant stenosis in the LAD artery (Fig. ). Coronary angiography revealed a saccular LMCA aneurysm measuring 9.8 × 7.5 mm with 75% stenosis in the proximal portion of the LAD artery. The operation was performed under general anesthesia. A median sternotomy was performed, and after a longitudinal pericardial opening was made, the heart was inspected. The LITA was removed from the inner chest wall in a skeletonized fashion using electric cautery. A distal segment of 1.5–2 cm was procured and reserved for use as a patch repair. Before aortic cannulation, the ascending aorta was dissected from the pulmonary artery. Under cardiopulmonary bypass, coronary artery bypasses of the left internal thoracic artery to the LAD artery were constructed in the beating heart. After aortic cross-clamping, the LMCA saccular aneurysm was exposed without main pulmonary artery transection. The saccular LMCA aneurysm was carefully dissected and completely excised. There was no thrombus in the lumen. Then, the LITA was longitudinally divided and trimmed to fit the incised LMCA. The small internal thoracic artery patch was sutured to the normal and firm lateral coronary arterial wall with a continuous 7–0 Polypropylene suture. Resection of the saccular aneurysm and closure using a small internal thoracic artery patch was then complete. The aortic cross-clamp time was 120 min, and the CPB time was 147 min. The patient had an uneventful hospitalization and was discharged on aspirin therapy. Follow-up multislice CT 10 days after the operation revealed the complete disappearance of the aneurysm and a successful repair with no luminal stenosis by the internal thoracic artery patch. The LITA graft was also found to be patent (Fig. ). The patient has been followed up yearly since 2009. Fortunately, at the 9-year follow-up, the patient was still asymptomatic, and there were no changes in the ECG and UCG. The patient included in the follow-up had preserved preoperative left ventricular function, and there was no coronary incompetence. Pathology of the aneurysm revealed that the aneurysm wall was very thin due to a lack of trilaminar arterial structure from the remarkable atherosclerotic changes (Fig. ). |
A 65-year-old Caucasian female with ASA physical status 2 sustained an intraparenchymal brain hemorrhage with rapidly progressive neurologic deterioration that required the airway to be secured. Tracheal intubation was performed prior to arrival at the admitting referral institution. A chart review of placement of the endotracheal tube (ETT) did not document any difficulty or injury with the procedure.\nA chart review after admission documented preanesthetic airway assessment for unrelated surgery seven years prior. She was classified as a Mallampati class 2 airway. Tracheal intubation at that time was hindered by small mouth opening after induction, an anteriorly positioned glottic opening with a grade 2 laryngoscopic view by the Cormack-Lehane criteria, and inability to place an ETT through the glottis without the assistance of a gum elastic bougie.\nDuring the current admission to the intensive care unit, it was noted that the ETT pierced the patient's tongue. Anesthesiology and otolaryngology services were consulted for ETT exchange and evaluation of the tongue injury. Inspection of the oral cavity was restricted by limited mouth opening, a short thyromental distance, and crowded dentition (). A 7.0 mm internal diameter ETT on the right side of the oral cavity pierced through the body of the tongue with a 4 mm pedicle of tissue bridging around the ETT. Ecchymosis and edema of the surrounding oropharyngeal tissue made further evaluation difficult but the ETT was in correct position in the trachea (). Anteroposterior radiographs of the facial structures demonstrated the ETT to be deviated to the right side of the mandible instead of that in the midline position that the ETT bite block maintains for patients mechanically ventilated with an ETT (). Oxygenation and ventilation remained normal at all times during the hospital stay.\nThe patient's brain injury had resulted in loss of the gag reflex but the cough reflex remained intact. Consent to perform elective tracheostomy followed by operative direct laryngoscopy (DL), removal of the ETT, and repair of injury to the oropharyngeal structures was obtained, and the patient was transferred to the operating room on hospital day 2. DL demonstrated the ETT traversing the floor of the mouth through a laceration at the junction of the floor of the mouth and the posterior tongue on the right side. The ETT appropriately entered the laryngeal opening (). Both the Lindholm and anterior commissure laryngoscopes allowed visualization of the epiglottis, but tilting of the epiglottis to expose the laryngeal inlet or visualization of the laryngeal opening was not possible ().\nThe patient had normal tracheal anatomy, and a standard tracheotomy with placement of a number 4 cuffed Shiley tracheostomy tube was performed. After securing the tracheostomy tube, the ETT was removed and repeat DL revealed no additional injuries. There was no significant bleeding from the tongue or floor of the mouth. The patient had scant drainage of serosanguinous secretions from the laceration site that resolved spontaneously. The patient was placed on palliative care due to the severity of the brain injury and expired on the fifth hospital day. |
The blind and painful right eye of a 38-year-old man was eviscerated in September 2016. The patient stated that his right eye had been blind since early childhood due to a unilateral congenital anomaly complicated by secondary glaucoma. He received the diagnosis of SO in January 2017 after he experienced visual loss in his only seeing (left) eye. At the time of diagnosis, the patient was admitted to the hospital and meticulously investigated for possible infectious and noninfectious causes to rule out other uveitic entities, but without any positive findings. At that time, his best-corrected visual acuity was 6/10. Slit-lamp examination yielded some vitreous cells in the left eye. Fundoscopy showed a few scattered pigmented chorioretinal scars and discrete yellowish round choroidal lesions throughout the left fundus (). Fluorescein angiogram delineated the active lesions as early hypofluorescent () with late staining. Left macular contour was normal on optical coherence tomography (OCT) examination (). He was started on oral prednisolone (64 mg) for 2 weeks with gradual tapering of 8 mg per week. Despite initial visual improvement, he experienced another episode of visual decline while taking 32 mg of prednisolone. His best-corrected visual acuity decreased to 2/10 and he had grade 4 vitreous haze according to the Miami grading. Fundus examination showed marked yellowish-white discoloration of the macula with some evidence of intraretinal hemorrhage ( and ). He was hospitalized and treated with pulse methylprednisolone 1 g (250 mg 4 times daily) for 3 days. Following pulse therapy, 64 mg oral prednisolone and 150 mg (50 mg 3 times daily) azathioprine were co-administered. Two weeks after the completion of pulse therapy, his visual acuity was still 2/10 despite a significant reduction in vitreous haze. Fluorescein angiogram and OCT demonstrated type 2 choroidal neovascularization (). Five intravitreal 2 mg aflibercept injections were given within a period of 8 months. His final visual acuity was 6/10 with a stable-looking macula ( and ) and he was continued on a treatment regimen of 150 mg azathioprine and 8 mg prednisolone daily. |
A 78-year-old male with multiple comorbidities, including hypertension, hyperlipidemia, and type II diabetes mellitus, presented to our emergency setting with complaints of recurrent bouts of abdominal pain and fluctuating fevers for the previous two weeks. The patient reported that the pain is a new manifestation of a previously dull aching pain that had waxed and waned over the last decade. His description alluded to a pain that was sharp and intermittent with localization in the right upper quadrant. He could not attribute the intermittent nature of his predicament to any aggravating or relieving influences. The pain was associated with fluctuating low-grade fevers (99°F-100°F), anorexia, and an associated 13-pound weight loss, which culminated in a visit to our clinical setup.\nFurther interrogation disclosed that the patient underwent a laparoscopic cholecystectomy in 2003. The ensuing year was relatively pain-free but was followed by recurrent bouts of right upper quadrant pain, albeit less upsetting than his current presentation. He was subsequently diagnosed in 2005 with gallstone spillage. The patient chose conservative treatment for his abdominal pain, rather than invasive interventions, which included the administration of acetaminophen and non-steroidal anti-inflammatory drugs (NSAIDs). This treatment modality was sufficient for the duration of a decade. He presented to another medical facility with similar complaints of fever and abdominal pain in 2016. A computed tomography (CT) scan of his abdomen disclosed the presence of a necrotic phlegmon, which was subjected to aspiration. Its composition included a combination of fibrous material, granulation tissue, and inflammatory infiltrate. The aspiration provided considerable relief of symptoms and he was discharged on a gabapentin prescription that was well-tolerated and produced sustained amelioration of his pain, with only occasional wavering with respect to his baseline.\nThe initial assessment showed an elderly gentleman, who was alert and well-orientated but under considerable distress due to the abdominal pain and accompanying chills. He had a fever of 104°F, a heart rate of 120 beats per minute, a blood pressure of 95/70 mm Hg, and a respiratory rate of 19 per minute. An abdominal exam revealed a non-protuberant, soft, and tender abdomen with marked sensitivity in the right upper quadrant. He had perceptible bowel sounds, an absence of dullness on percussion, and a clear rectal vault. Pertinent initial laboratory investigations included an elevated white blood cell (WBC) count of 16,900/µL, C-reactive protein (CRP) of 6.5 mg/dL, and a random blood glucose (RBG) of 280 mg/dL. The patient subsequently underwent a whole body CT scan, which revealed a large 19-cm sub-diaphragmatic, right retroperitoneal abscess, which was inferior and posterior to the right hepatic lobe (Figure ).\nIn lieu of these findings, the patient was admitted and started on a combination of intravenous fluids, intravenous (IV) vancomycin and piperacillin-tazobactam due to an underlying suspicion of sepsis secondary to a hepatic and/or perihepatic liver abscess caused by a lack of gallstone retrieval following his cholecystectomy. He was subsequently subjected to a percutaneous CT-guided drain placement, which allowed for the evacuation of approximately 700 mL of grossly purulent material and provided prompt pain relief. The fluid sample was sent for a gram stain, culture, and a bilirubin assay. The gram stain of the abscess fluid revealed branching gram-positive rods concerning for Actinomyces, Nocardia, and Streptomyces; therefore, the patient remained on IV vancomycin and piperacillin-tazobactam, with the addition of trimethoprim-sulfamethoxazole (TMP-SMX) to the antibiotic regimen. Remarkably, the culture growth was positive for Propionibacterium and ampicillin-sulbactam was added to the list of antibiotics. Two days later, the patient was shifted to the intensive care unit (ICU) following unrelenting fever and persistent tachycardia, even after his abdominal drain placement was supplemented with vigorous antibiotic and antipyretic therapy. His ICU stay was complicated by episodes of dyspnea, tachypnea, and pleuritic chest pain, while a chest auscultation unveiled a decrease in breath sounds at the right basal region. A repeat CT scan confirmed the reduction in the size of the abscess and showed a new right-sided pleural effusion, which explained the patient’s breathing difficulties (Figure ).\nThe patient underwent an ultrasound-guided chest tube placement, which evacuated 10 mL of purulent fluid, followed by two doses of lytic therapy with tissue plasminogen activator (tPA) via the catheter. The following morning, he described an aggravation in his pleuritic chest pain, and an episode of shortness of breath, whereby, the oxygen saturation dipped to 90%. A new chest CT scan was ordered, which showed an interval increase in the right basilar opacity with a moderately sized pleural effusion (Figure ).\nHe was subsequently upgraded to a larger catheter via a CT-guided approach that evacuated a total of 900 mL of purulosanguinos fluid. The patient tolerated subsequent lytic therapies well and showed vast clinical improvement following this procedure, with a reduction in dyspnea, pleuritic chest pain, and fever, as well as a down-trending of his inflammatory markers.\nA repeat CT scan was performed on the 10th day of admission, which revealed a near-complete resolution of his right-sided pleural effusion, and a decrease in his right-sided retrohepatic intrabdominal abscess (Figure ).\nThe chest drain was removed and the patient was subsequently discharged with a transhepatic drainage catheter, daily intravenous ampicillin-sulbactam, and acetaminophen, as needed for pain control. The patient was shifted to oral amoxicillin-clavulanate a month after the resolution of his pain, fever, and stabilization of inflammatory markers. The transhepatic percutaneous drain was left in place in order to allow for the formation of a chronic drainage tract, with hopes of localizing the perpetrating gallstones at its base.\nTwo months after discharge, the patient underwent a procedure for upsizing the drain in order to accommodate for a choledochoscope into the abscess cavity and the maturing sinus tract. He subsequently underwent an abscess cavity endoscopy, whereby a catheter and scope were advanced into the cavity for exploration. An abscess catheter study was performed through the existing catheter and showed no filling defects, after which the catheter was removed and an endoscope was inserted for direct visualization of the main cavity, as well as the two “fingers” branching out of the abscess cavity. Two small stones were expelled after flushing the main cavity multiple times. Following the expulsion of the gallstones, a replacement catheter was advanced into the cavity and a second catheter study was performed to rule out the possibility of any other stones. The patient tolerated this intervention well and had no further drainage from the intra-abdominal drain.\nFollowing a lack of drain output and resolution of symptoms after his last endoscopic procedure a month ago, the patient's intra-abdominal drain was removed. The patient is currently stable and is being followed as an outpatient to date. |
The patient was a 20-year-old male with a history of chronic gastritis and a hiatus hernia. He had presented complaining of episodes of frequent abdominal pains, sometimes debilitating, associated with nausea and vomiting, with onset around 10 days prior to hospital admission.\nOn physical examination he was in good general health, with good color, hydrated, acyanotic, no sign of jaundice, free from fever, with normal blood pressure and peripheral pulses present. He had diffuse abdominal pains on palpation and attenuated bowel sounds.\nAfter assessment by the general surgery team, the patient underwent computed tomography of the abdomen, which showed a gastroduodenal artery aneurysm and significant stenosis of the celiac trunk (). The vascular surgery team was called in and angiotomography was performed, showing the gastroduodenal artery aneurysm with a maximum diameter of 3.6 cm, length of 9.5 cm and no signs of rupture (\n).\nAfter careful consideration of the case and the patient’s general status, endovascular treatment of the aneurysm was proposed. The right common femoral artery was catheterized, a 5 French introducer was inserted and a 5 French Cobra catheter was positioned within the superior mesenteric artery (access to the aneurysm via the celiac trunk was ruled out because of the stenosis). A Rebar® microcatheter was then inserted and advanced along the path of the pancreaticoduodenal artery to access the gastroduodenal artery. Embolization was performed using a total of 19 Concerto® coils of varying sizes (6/20 mm, 8/30 mm, 9/30 mm, and 10/30 mm) and also Onyx® embolizing agent () and angiographic results after the procedure were satisfactory ().\nThere was significant remission of the patient’s pain after the procedure and he was discharged 2 days after surgery in a satisfactory general condition. Six months after the procedure, the patient underwent angiotomography once more, which showed complete exclusion of the aneurysm and no complications related to the procedure (). The celiac trunk stenosis was not treated because around 12 months after the procedure the patient was still stable and asymptomatic. |
A 56-year-old Chinese woman diagnosed with bipolar I disorder for the past 28 years was transferred from a tertiary psychiatric hospital in 2017 to our medical hospital for bacterial endophthalmitis. She was also experiencing a manic relapse concurrently, presenting with elated and labile mood, irritability, flight of ideas, disinhibition, and grandiose delusions of being a beauty pageant contestant. Her mental state affected her insight into and compliance with all medical treatment, and as such, she was mentally and medically unwell.\nHer bipolar disorder was previously stabilized on dual mood stabilizers (lithium carbonate at 400 mg per day, and sodium valproate at 1,000 mg per day). Since the diagnosis of her medical comorbidities in 2010, including CKD approaching ESKD secondary to her poorly controlled diabetes mellitus and hypertension, hyperlipidemia, and obstructive sleep apnea, her lithium was ceased due to CKD, switching to antipsychotics (including sulpiride at 400 mg per day, and risperidone at 4 mg per day) and sodium valproate at 1,200 mg per day. However, she continued to have multiple relapses, necessitating three admissions between the years of 2011–2014 for mania. Despite inpatient treatments, her condition was not stabilized, and she was trialed on intramuscular antipsychotics (zuclopenthixol at 400 mg every 4 weeks) due to poor compliance with oral medications.\nOur patient was first hospitalized in our medical hospital for 5 months, complicated by five intensive care unit admissions due to decompensated type 2 respiratory failure. Although there were initial improvements in the mental state with sodium valproate and risperidone, she developed bicytopenia on sodium valproate, and had repeated episodes of drowsiness, prolonged corrected QT interval (QTc), and bradycardia likely secondary to antipsychotics. Electroconvulsive therapy (ECT) was considered, but the patient was deemed to be of high general anesthetic risk. She also had a permanent pacemaker implanted after a diagnosis of sick sinus syndrome by the cardiologist. She was then discharged to the rehabilitation hospital with aripiprazole at 10 mg per day but continued to display residual manic symptoms, including irritability and distractibility. She was admitted again 2 months after her discharge from the rehabilitation hospital for a relapse of manic symptoms, presenting with irritability, elated and labile mood, poor sleep, flight of ideas, and pressured speech. She also had persistent diarrhea and poor management of her fluid overload. |
A 67-year-old Caucasian man presented to the emergency room because of a 4 days’ history of abdominal pain, with one episode of vomiting.\nThe patient’s past medical history was significant for colonic diverticulosis and an episode of gastrointestinal bleeding one year before. The event had been investigated by two different gastroscopies, a colonoscopy and a MDCT, which produced inconclusive results. Ten months later he was newly admitted because of abdominal pain and fever at 38.0 C°, with valid urination and defecation. A CT of the abdomen was performed, which confirmed the colonic diverticulosis and revealed the presence of multiple diverticula of the small intestine, fat stranding, signs of inflammation as well as a small amount of free liquid in the abdomen. The patient was hence diagnosed with jejunal diverticulitis and managed conservatively with intra-venous antibiotics, with an apparent complete recovery.\nHe re-presented to the emergency department two months later with acute abdominal pain. The pain was described as severe and constant, localized mainly in the lower abdomen with clinical signs of peritonitis. No change in bowel habits nor urinary symptoms were complained. His vital signs were stable, with a temperature of 37.2 C°; he appeared fully oriented and not in any acute distress. Laboratory examination reported a hemoglobin of 121 g/dL, a WBC count of 12.2 × 10E9/L and a CRP of 249 mg/L. Other laboratory data were within normal limit. An abdominal and pelvic contrast-enhanced computed tomography, with administration of oral contrast, was performed. Jejunum and ileum showed several diverticula as well as an inflammatory thick-walled mass involving different loops of the intestine. In addition, free fluid in the abdomen and a small amount of subdiaphragmatic air were reported (, ). On the basis of these findings, the diagnosis of perforated diverticulitis was hereby proposed.\nThe patient underwent a diagnostic laparoscopy which revealed plenty of purulent yellowish liquid collected in the right abdomen and a conglomerate of intestinal inflamed loops. We hence decided to convert immediately the procedure to laparotomy. Large multiple diverticula were found covering a section of small intestine approximately 2.5 m long, without signs of obvious macro perforation. Among the middle distal tract of the jejunum and the middle distal tract of ileum, strong adhesions were identified (). The involved segments of jejunum and ileum were connected by an intestinal loop free of signs of diverticulosis (). There were no signs of bowel ischemia. Adhesiolisis was partially carried out, however, because of difficulties associated with the procedure, we opt to perform a double enterectomy, removing only those segments involved in the intestinal conglomerate and deeply affected by the pathology. Roughly 25 cm of ileum and 80 cm of jejunum were resected. Bowel continuity was restored with an ileo-ileal and a jejuno-jejunal anastomosis. Almost 700 cl of pus were drained and the peritoneal cavity was washed with 10 L of saline solution. The postoperative recovery was uneventful and the patient was discharged 8 days later. There were no signs of malignancy in the resected intestine. |
A 35-year-old man was referred to the hospital with an eye injury in which one prong of a double prong fishhook had stabbed through the upper eyelid and cornea and was embedded in the temporal area of the right eyeball (Fig. ). The accident occurred while he was a bystander observing a fishing competition. No attempt was made to remove it. On initial presentation, the patient reported visual acuity of hand movement in the injured eye. Detail evaluation of anterior and posterior segments could not be properly performed as the upper eyelid was stuck with the fishhook. Intravenous ceftazidime dosing 1 g every 8 h and vancomycin dosing 1 g every 12 h were given to the patient.\nUnder general anesthesia, the hook shank was firmly grasped and a wire cutter was inserted beneath the upper lid, above the cornea, to cut the bend of the fishhook (Fig. ). The proximal part of the fishhook was removed backward through the entry wound of the upper eyelid and it was, then, possible to open the eyelid fully (Fig. ). On examination, the hook was seen to have perforated the temporal peripheral cornea and traversed through the iris and lens zonule and was tightly entrapped within the ciliary body inside the eyeball. No exit wound was observed. The point of the hook had adhered approximately 3 mm posterior to the limbus. Traumatic cataract and hyphema were noted. Conjunctival peritomy was performed at the temporal limbal site, from 7 to 11 o'clock, corresponding to the traumatic conjunctiva. The remaining end of the bent hook was gently pressed outward against the sclera to locate the embedded hook. Incision was made through the tented sclera starting from the point of the fishhook to a limbus using a straight surgical blade No. 15. The entire barbed hook was, then, advanced forward and rotated out through the scleral wound. The iris and vitreous were noticed at the surgical site. The corneal wound was repaired with 10.0 nylon, while the limbal and scleral wounds were sutured with 8.0 nylon. The iris was repositioned and the anterior chamber was reformed using balanced salt solution. The vitreous was managed. The lacerated eyelid was approximately repaired. During the final step, intravitreal injections of vancomycin (1 mg/0.1 mL) and ceftazidime (2.25 mg/0.1 mL) were given. The appearance of the fishhook is shown in Figure . After the operation, 1% of prednisolone acetate and 0.5% of levofloxacin eye drops were given topically four times a day and tapered over a period of 4 weeks. In addition, intravenous ceftazidime (1 g every 8 h) and vancomycin (1 g every 12 h) were administered continuously for 5 days. Due to high prevalence of post-traumatic infection in this region, systemic steroid was not prescribed.\nOn day 1 postoperatively, anterior segment examination revealed secured corneal and scleral wounds, a peak pupillary margin, hyphema, and a traumatic cataract (Fig. ). The B-scan showed signs of localized retinal detachment without choroidal injury. Then, oral steroid was not prescribed. A week after the primary operation, a combined lensectomy and pars plana vitrectomy were done. A mid-peripheral retinal tear, from a point of the fishhook, and a surrounded localized inferotemporal retinal detachment were repaired. A 5,700-centistoke silicone oil was used as a tamponade at the end of surgery. The removal of the silicone oil with secondary intraocular lens implantation were carried out 6 months after injury. At month 10, the best corrected visual acuity was 20/20. Temporal peripheral corneal scar was seen. The retina appeared completely attached with inferotemporal retinal scar (Fig. ). No other significant complication was noted. |
A 76-year-old woman visited our gynecologic department with masses in her lower abdomen and left inguinal region for seven months. She also had experienced intermittent vaginal spotting over the last three years. Upon physical examination, a large, hard abdominal mass that extended from the pelvis to 7 cm above the umbilicus was noted. In addition, a tense, painless mass 5 cm in size was detected in her left inguinal region. The inguinal mass was not reduced by manual pressure in the supine position, nor was it changed in size by Valsalva's maneuver. Also, no evidence of inflammation or incarceration was noted. The serum cancer antigen 125 level was elevated to 106.0 U/mL (normal range, < 35 U/mL), and the white blood cell counts were within the normal limit.\nTransabdominal ultrasonography revealed a huge, 21×12×10 cm, solid and cystic mass with increased blood flow within the solid and septal parts of the abdominal cavity. High-resolution ultrasonography with a 12-5 MHz linear-array transducer showed a well-circumscribed, oval cystic mass with a hypoechoic solid portion (3×3×6 cm) in the left inguinal region. Also, a stalk-like structure was seen in the cranial aspect of the mass (). The mass did not show peristalsis or any bowel content. Color Doppler sonography showed flow signals in the solid portion and in the wall of the mass ().\nThe contrast-enhanced MDCT with multi-planar reformatted images showed the large abdominal mass arising from the right adnexa and extending to the upper abdominal cavity. The left inguinal cystic mass was abutted to the extraperitoneal portion of the round ligament, which was unusually thickened, and had an irregular enhancing solid portion in the wall of the cystic mass. The presumed preoperative diagnosis was a malignant right ovarian mass with metastasis in the left round ligament. Herniation of the left ovary cases were excluded from the patient population because the reformatted CT images revealed more cranially located ovarian vessels within the pelvic cavity ().\nAt surgery, the large mass originated from the right ovary, and the left ovary was atrophied. A cystic mass in the inguinal region was attached to the hypertrophic left round ligament.\nUpon histopathologic examination, the right ovarian mass was confirmed as a Sertoli-Leydig cell tumor. The inguinal mass was a multi-locular cyst lined by mesothelial cells, and combined with diffuse stromal edema and chronic inflammation (). However, no evidence of malignancy was observed. |
A 42-year-old male presented with recurrent palpitations for the last 10 years. The 12-lead electrocardiogram was normal, and echocardiography showed no significant structural abnormalities. Holter monitoring and an exercise test revealed intermittent preexcitation, suggesting a left-sided AP (). As the patient was highly symptomatic, EPS and possible radiofrequency catheter ablation (RF ablation) were recommended.\nEPS was performed with a steerable duo-decapolar catheter positioned in the right atrium and inside the coronary sinus (CS), and two quadripolar catheters positioned at the right ventricular apex and the His bundle region. Basic intervals were normal. Dual AV nodal physiology was demonstrated by single atrial extra-stimulation. Anterograde conduction via an AP was not observed. At a driving pacing cycle length (CL) of 600 msec, the effective refractory periods (ERPs) of the fast and slow AV nodal pathways were 430 msec and 320 msec, respectively. During incremental ventricular pacing, 1:1 ventriculoatrial (VA) conduction over the AP in the left free wall occurred at 280 msec. The ERP of retrograde VA conduction over the AP was 250 msec and the ERP of retrograde VA conduction over the AV node was less than 230 msec.\nTachycardia with CL varying from 393 to 420 msec was reproducibly induced by single atrial extra-stimulation with AH prolongation (). The AH and HV intervals were 284 msec and 53 msec, respectively. The shortest VA interval of 56 msec was noted in the proximal CS recording. The sequence of retrograde atrial activation was eccentric, suggesting atrial fusion. Changing the site of earliest retrograde atrial activation to the distal CS with single premature ventricular pacing during tachycardia at a time of His-bundle refractoriness hindered discrimination of tachycardia ().\nWith slight prolongation of CLs of the tachycardia, there was transition of tachycardia to the other type of tachycardia with a CL ranging from 479 to 505 msec. The AH interval was 356 msec and the HV interval was 59 msec. Retrograde atrial activation was eccentric and the shortest VA interval was 70 msec in the distal CS recording ().\nA 4-mm deflectable tip ablation catheter (Irvine Biomedical, Inc., Irvine, CA, USA) was used to deliver RF current with 40 W power and temperature limit of 60℃. After ablation of the AP in the left free wall (), VA conduction over the AV node was recorded and AVNRT with concentric retrograde atrial activation was reproducibly induced by single atrial extra-stimulation with AH jump (). Successful slow pathway modulation was achieved with an anatomic approach (). |
A 56-year-old female without comorbidities and a 50-pack-year smoking history was referred to the emergency department with a severe right frontal headache. The onset was abrupt and the preceding symptoms and signs included nausea and ataxic gait. Neither cranial nerve abnormalities nor focal limb weakness was spared. Initial laboratory values were within the reference range. The patient underwent a magnetic resonance imaging (MRI) which revealed multiple nodular lesions throughout both cerebral and cerebellar hemispheres with an unusual lamellated pattern of enhancement. The demonstrated significant vasogenic edema did not led to midline-shift (Figures –). Chest X-ray detected opacity with a lobulated contour in the right upper lobe (). A contrast-enhanced chest computed tomography (CT) showed an ill-defined mass in the anterior segment of the right upper lobe and right hilar lymphadenopathy (). No additional abnormalities were observed on subsequent detailed metastatic work-up including abdominal CT and whole body bone scintigraphy. Due to the neurological symptoms, the patient underwent whole-brain radiotherapy (WBRT). Fractionation of 3 Gy per day was used, reaching a total dose of 30 Gy in two weeks; in addition, the patient received intravenously dexamethasone during WBRT. The performed bronchoscopy detected a bulging lesion located at the membranous portion of bronchus of the anterior segment of the right upper lobe. The lesion did not appear to be covered with normal bronchial mucosa. Subsequently, a biopsy was performed (), and the histopathology showed infiltration by atypical melanocytes containing melanin pigmentation (Figures and ). Positive immunohistochemical staining of MART-1, S-100 protein, and HMB-45 and lack of expression of cytokeratin (CK), epithelial membrane antigen (EMA), synaptophysin (Syn), and high molecular weight cytokeratin confirmed the diagnosis of pulmonary melanoma. Molecular profiling was conducted which failed to detect any mutations.\nTo rule out the diagnosis of melanoma metastasis from a primary site, an extensive skin and fundoscopic examination was carried out. Endoscopy of both upper and lower gastrointestinal tract as well as cystoscopy did not detect any additional abnormalities. Indeed, a panendoscopy of the upper airway failed to demonstrate extrapulmonary disease. Based upon the histological characteristics, immunohistochemical features, and clinical data, the diagnosis of primary pulmonary malignant melanoma was established.\nSurgical resection of the lung mass was not indicated due to the brain metastases. The completion of WBRT resulted in transient remission of the neurological symptoms and the patient was switched to dacarbazine (DTIC) in combination with recombinant interferon alpha-2a (rIFN-α-2a). Due to unacceptable toxicity rIFN-α-2a was discontinued three weeks after being administered.\nThe treatment did not provide any clinical benefit; instead patient's condition deteriorated and ipilimumab was added to DTIC. The evaluation of response was planned to be performed after four cycles of the administered combination therapy. Indeed, the patient was admitted to the hospital with progressive exertional dyspnoea and persistent chest tightness. She died five months after the initial diagnosis. Massive pulmonary embolism was determined as the cause of death in the performed autopsy. |
A 67-year-old woman with a history of hypertension was admitted to our hospital with acute chest pain. Under the diagnosis of unstable angina, anticoagulant and dual antiplatelet therapy was initiated with intravenous heparin (25000/IU), aspirin (75 mg/day), and clopidogrel (75 mg/day). Laboratory tests showed a partial thrombin time of 58.3 seconds and a prothrombin time of 11.6 seconds with an international normalized ratio of 13.24. One day after commencing anticoagulant therapy, she developed sudden severe back pain and paraplegia (Grade I/V).\nAlthough she also had hypoesthesia below the T2 dermatome, her consciousness was completely normal. Thoracic T2-weighted MRI showed intradural extramedullary low signal intensity at the T2-3, which was consistent with spinal subarachnoid hematomas. High signal intensity was also observed from C5 toT4 in the spinal cord (). Surgical exploration was then undertaken to decompress the spinal cord. Prior to the operation, heparinization was reversed and antiplatelet administration was discontinued. With total laminectomy at T1-4, we could not find any hematoma in the subdural space. However, thick hematoma compressing the spinal cord was found beneath the arachnoid membrane (). The hematoma extended into the anterior, upper and lower subarachnoid space around the spinal cord. Much of the hematoma was removed sufficiently and the spinal cord became soft. Postoperative MRI demonstrated reduced spinal cord edema. The next day, the patient complained of a severe headache and brain CT revealed SAH on both parietal lobes (). For prevention of vasospasm, nimodipine was administered to prevent cerebral vasospasm intravenously. Hypervolemic and hypertensive treatments were not started because of her poor heart function. Nevertheless, her consciousness decreased over time and blurred vision developed with hemiplegia seven days after surgery. Brain CT and MRI revealed multiple cerebral infarctions in the right posterior cerebral artery territory, left parietal lobe and right watershed area (). Conventional cerebral angiography showed diffuse severe vasospasm of the intracranial arteries, which was most prominent in the right middle cerebral artery and temporo-occipital branches. Perfusion defects were also noted in the bilateral parietal, occipital, and temporal lobes on perfusion CT scan (). After one month of intensive care, she was referred to the rehabilitation department. After six months, she displayed partial improvement of right lower extremity motion, cognition and vision. However, there was no improvement of weakness in her left extremities. |
A 45-year-old Haitian American female with no significant medical history presented with a six-month history of progressive solid food dysphagia and a one-month history of odynophagia. The patient had lost 15 pounds and was only able to tolerate pureed food or liquids. She had experienced no symptomatic relief on omeprazole 40 mg twice daily for the past two months and did not use tobacco, alcohol, or illicit substances. Her initial blood count and metabolic panel were unremarkable. An esophagogastroduodenoscopy (EGD) revealed erythematous and friable mucosa with ulcerations in the proximal esophagus (). There was a stricture encountered at 15 cm from the incisors through which the gastroscope could not be traversed. Biopsies were taken from the inflamed esophageal mucosa and the proximal lumen of the stricture. Brush cytology was collected through the stricture as there was a concern for malignancy. A subsequent barium esophagram and upper GI series demonstrated 2 cm irregular narrowing in the cervical esophagus, but no abnormalities in the rest of the esophagus, the gastroesophageal junction, stomach, duodenum, or proximal jejunum. Contrast-enhanced computed tomography (CT) of the chest showed no acute esophageal, mediastinal, pulmonary, or cardiac pathology. The esophageal biopsy indicated acute and chronic inflammation with filamentous sulfur granules consistent with Actinomyces; rare fungal hyphal elements were additionally identified (Figures , , and ). The cytology was negative for malignant cells and the acid-fast bacilli (AFB) stain was negative, ruling out Nocardia as a potential pathogen. The patient was started on intravenous (IV) Penicillin for a diagnosis of esophageal actinomycosis and oral Fluconazole for a presumed Candida coinfection given concurrent fungal elements. Her human immunodeficiency virus (HIV) status was negative and her fasting blood glucose was within the normal range. She was discharged on Fluconazole 200 mg daily for 2 weeks and IV Penicillin G 3 million units every 4 hours for 6 weeks followed by oral Penicillin to complete a total of 6 months of antibiotics. Her esophageal culture eventually grew normal oropharyngeal flora and rare Candida albicans. The patient returned to the hospital 6 weeks later due to an acute right upper extremity deep venous thrombosis (DVT) associated with her peripherally inserted central catheter (PICC). Her odynophagia had improved but she was still not able to advance her diet. Repeat EGD was performed, which showed resolution of her esophagitis, but a remaining stricture in the proximal esophagus (). During an attempt to dilate the stricture, a small mucosal tear was induced. The dilation had to be deferred at that point pending mucosal healing. A repeat barium esophagram illustrated the previously identified stricture with no perforation. The patient's PICC was removed and she was discharged on oral amoxicillin 875 mg twice daily to complete the 6-month course. The patient was given a follow-up appointment in our GI clinic where she would be reassessed for esophageal stricture dilation. Unfortunately, she was lost to follow-up. |
A 51-year-old, right-handed male visited the outpatient clinic for right fingers-drop. It occurred spontaneously a month prior. The patient in this case was considered to have a brain lesion and disc disease and visited an orthopedic as an outpatient a month after seeing a neurologist. He denied any history of trauma or recent illness. During a general physical examination, the patient’s fingers, including the thumb, were not extended at the metacarpophalangeal joint. Active extension of all five fingers on wrist extension revealed motor power of grade zero to one. Active motion of the right wrist was available. There was no accompanying dysesthesia or decrease of touch sensation below the elbow joint. There was no palpable mass or tenderness around the pathway of radial nerve. Plain radiographs and all routine blood studies were normal. The electromyography (EMG) and nerve conduction velocity (NCV) study were consistent with PIN. Further evaluation was performed with magnetic resonance imaging (MRI) to identify any space-occupying lesions or possible soft tissue lesions around the radial nerve pathway. On MRI imaging, a ganglion cyst about 1.8 cm in diameter was observed on the proximal part of the superficial layer of the supinator muscle (or Arcade of Frohse) ().\nWe proceeded with surgical removal of the cystic lesion for PIN decompression because MRI findings and the EMG-NCV study revealed definite PIN compression with a space-occupying lesion, although the sudden onset paralysis motor symptom had a short duration. Under general anesthesia, a modified anterolateral approach was used with a small, 4 cm-sized incision. Instead of using the typical anterolateral approach for PIN syndrome, the cyst was reached through the space between the brachioradialis and common extensor with forearm pronation because the cyst was easily palpated just lateral to border of the brachioradialis muscle belly. The ganglion cyst was found at the proximal part of the superficial layer of the supinator muscle. Compression of the PIN by the ganglion cyst was noted. The superficial branch of the radial nerve was also visualized, and no compression was noted. Surgical excision was done at the base of the stalk of the ganglion cyst which seemed to be connected to the proximal radioulnar joint capsule (). The palsy had completely resolved when the patient was observed by the outpatient department a month after the operation. Patient did not complain of any motor weakness or history of recurrence during the one-year follow-up period. |
We present the case of 32-year-old female patient who presented to the urologist with fever lasting for the previous two months, usually 37°C to 38°C, with occasional temperature leaps to 40°. Laboratory blood tests showed leukocytosis with cell counts up to 19,000. The patient did not complain of lumbar pain. Urinalysis showed leukocyturia, erythrocyturia, and slight positivity for protein. The patient was treated with a combination of amoxicillin, clavulonic acid, and cefuroxime. Abdominal ultrasound and multiphase CT depicted a large, expansive, heterogenous mass in the lower pole of the left kidney measuring 92 × 59 × 76 mm. CT scans showed a renal neoplastic mass with increased peripheral inhibition and poor central inhibition, possibly due to central necrosis. The tumor was unsharply demarcated from the surrounding parenchyma and pelvic system (). Regional lymphadenopathy was present, but there were no signs of propagation in the main renal vein or perinephric fat tissue, and there were no signs of liver or bone metastasis. Chest CT showed no signs of lungs metastasis or mediastinal lymphadenopathy. In order to determine the final histopathological diagnosis, histopathological and immunohistochemical analyses were performed, complemented by reverse transcryptase-polymerase change reaction (RT-PCR). Gross examination of the slitted specimen showed a sharply circumscribed tumor measuring 9 × 6 × 6 cm and replacing a large part of the kidney. The renal tumor displayed infiltrated parenchyma but no expansion into the pelvic system and ureter or signs of spreading into the main renal vein. Gross appearance of the tumor was white-yellow with large necrotic areas. The microscopic analysis of perinephric fat tissue, regional lymph nodes, and the left adrenal gland revealed no signs of metastasis. Microscopic analysis showed that the tumor was composed of solid nests and stripes of tumor cells with small oval and round nuclei, and some of the nuclei contained small nucleoli (). The tumor cell cytoplasm was scanty and pink with coarser appearance of chromatin. Mitotic figures were also found. Immunohistochemical analysis included MIC2 gene product/CD 99 (), neuron-specific enolase (NSE) (), CD3, CD 20, chromogranin, synaptophysin, vimentin, and neurofilament. Immunohistochemical staining revealed positive expression of CD99 and NSE in all tumor cells. The tumor cells did not stain with CD3, CD 20, chromogranin, synaptophysin, vimentin, or neurofilament. RT-PCR analysis for EWS-FLI1 fusion proteins was positive for type II 191 pb and negative for type I 125 pb. Diagnosis of rPNET set on the basis of characteristic morphological characteristics and results of immunohistochemical analysis was confirmed by RT-PCR. After nephrectomy, the patient received 14 cycles of a VAC/IE regiment adjuvant chemotherapy comprised of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Our patient was disease-free for nine years and eight months. Blood and urine laboratory test values were in the normal range.\nThe study was approved by the institutional ethical committee and conducted according to the principles of the declaration of Helsinki of the world medical association. The authors declare that they have no conflicts of interest. The authors did not receive any grants for writing this article. |
A 64-year-old woman presented to the gastroenterologist with abdominal pain, unintentional 10 pound weight loss over 3 months, and reflux symptoms. Pertinent medical history included hypertension treated with olmesartan 20 mg once a day for the past 7 years, hyperlipidemia treated with simvastatin, gastroesophageal reflux disease treated with pantoprazole 20 mg twice a day, and osteoporosis treated with denosumab injections every 6 months. Esophagogastroduodenoscopy (EGD) performed in the office showed granular mucosa in the proximal duodenum and nodular mucosa in the stomach with numerous linear ulcers. On withdrawal of the scope from the duodenum, a large amount of fresh blood was noted in the stomach with clotted blood near the cardia. The bleeding was believed to be due to either the underlying gastric ulcers or a Mallory Weiss tear. The patient was transferred to the emergency department for further evaluation.\nOn admission, the hemoglobin and hematocrit were normal, and an emergent EGD was performed. The gastric fundus and body appeared atrophic with friable and severely inflamed mucosa and multiple linear ulcers without active bleeding (). The antral mucosa appeared erythematous. Biopsies of the stomach and duodenum were performed. The biopsies of the antrum and body showed increased subepithelial collagen deposition confirmed on a trichrome stain, chronic inflammation in the lamina propria, surface epithelial regenerative changes, and erosions, consistent with collagenous gastritis (). The gastric body mucosa was atrophic with near-total loss of parietal cells. Immunostain for H. pylori organisms was negative, and a Congo red stain for amyloid was negative. The biopsies of the duodenum showed partial villous atrophy and increased subepithelial collagen deposition confirmed on a trichrome stain, consistent with collagenous duodenitis/sprue (). The patient was started on high dose acid suppression with pantoprazole 40 mg twice a day, and the olmesartan was discontinued.\nFollow-up EGD 7 weeks later showed somewhat atrophic gastric mucosa with few residual linear areas of erythema and complete resolution of the ulcers, inflammation, and mucosal friability (). The duodenal mucosa appeared normal. Colonoscopy was unremarkable. Repeat biopsies showed marked improvement of the collagenous gastritis with resolution of the epithelial injury and only rare residual foci of subepithelial collagen. A mild nonspecific chronic inactive gastritis remained present. The biopsies from the duodenum and left colon showed normal mucosa without evidence of collagenous duodenitis or collagenous colitis. |
In June 2007, a 70-year-old non-smoking female was seen at our hospital because of a 3-month history of progressive dysponea at rest. CT scan revealed a mass at the right lower lobe and moderate pleural effusion on the right side. Metastasis to right adrenal gland was observed on abdominal CT. There was no evidence of extra thoracic metastasis on brain MRI and bone ECT scans. Lung needle aspiration revealed adenocarcinoma and the patient was diagnosed with NSCLC stage IV. She started 4 cycles of systemic chemotherapy with cisplatin and gemcitabine in Jul 2007. CT scan showed a stable disease of the carcinoma and marked clinical improvement was noted as dyspnoea disappeared and the patient reported a general feeling of wellness. The patient then received two cycles of docetaxel for maintenance therapy. In the following six months, the patient was well without any evidence of local or systemic recurrence. In June 2008 a routine follow-up bone ECT and brain MRI revealed bone and brain metastatic lesions, and the patient commenced erlotinib (150 mg daily ), which she tolerated well and only experienced grade 1 skin rash without requiring dose adjustment. After 4 weeks of erlotinib, the patient showed complete response in her intracranial disease and a partial response in her lung disease. After 12 months of erlotinib therapy, tumor at the right lower lobe progressed, and two cycles of carboplatin and paclitaxel were administered. CT imaging confirmed stable disease in the right lower lobe tumor. However, the patient refused further cytotoxic chemotherapy because of severe treatment-related diarrhea. She was re-challenged with erlotinib (150 mg daily ) in October 2009, and experienced grade 3 skin rash without dose modification. Fortunately, she improved clinically with her right lower lobe tumor showing partial response after 4 weeks of erlotinib treatment and the treatment was continued for eleven more months(CT scans were shown in Figure ). |
A 26-year-old lady patient presented to our emergency department complaining of exacerbated headache and swelling of the left upper eye lid. On examination, a pulsatile soft swelling near the eye led and the left frontal scalp was found without neurological deficits. MRI and CT scan revealed left frontal and periorbital soft swelling with soft tissue signal and subcutaneous flow void signs. Accordingly, six-vessel cerebral angiography was conducted and demonstrated left frontal scalp AVM fed from the external and internal carotid arteries. From the external carotid artery, it supplied from right and left superficial temporal. On the other hand, from the internal carotid artery, it is fed from the left supraorbital artery, a branch of the ophthalmic artery. The venous drainage reaches the superior sagittal sinus through a pore from the bony skull (–).\nUnder sedative anesthesia and analgesia, through the endovascular procedure (starting from the right femoral artery), bilateral superficial temporal arteries were occluded with LESA. Post-embolization, angiography was conducted and revealed complete resolution of the nidus with remaining of the supraorbital artery as the single main feeder for the AVM (–). Two days following, the dilated supraorbital artery was ligated and excised through left eye-brow incision. In addition, left fronto-temporal skin incision was created and the flap was dissected and retracted in sub-periosteal fashion, leaving the AVM attached to the flap. During the retraction, the main venous drainage and bone pore was coagulated and occluded with bone wax. Dissection and retraction of the flap was continued until the superior orbital rim. The AVM was sealed and dissected from the peri-galeal plane to be totally resected (–). The wound was closed and a drain was left beneath the flap. No intraoperative vascular clips were utilized, nor blood transfusion was needed, with blood loss of about 200mL (–).\nPostoperatively, the patient was discharged without complications. After one-year, cerebral angiography revealed no recurrence of the AVM nidus with normal intact superior sagittal sinus flow ( and ). |
A 44-year-old Asian female, gravida 2, para 2, with no significant previous medical history, presented to urologist and complained that she had experienced dyspareunia over the preceding several months. The nature of the pain was a deep pain, which she experienced every time she attempted intercourse. She did not have vaginal discharge. No psychological factors can be found for her painful intercourse. She denied any episodes of gross hematuria, fever, chills, or irritative urinary symptoms. A pelvic bimanual examination was difficult to perform because of the patient's discomfort. No significant findings were detected on physical examination. Urinary tract infection was excluded as results of urinalysis and urine cytology were normal. The results of other laboratory tests including renal function, liver function, complete blood cell count, and coagulation function were also within the normal range.\nA pelvic ultrasonography revealed a 6.6 × 5.8 cm2 mass lesion located in the trigone of urinary bladder. The mass lesion was confirmed on contrast-enhanced CT to be an enhanced 7.2 × 6.1 cm2 soft tissue mass in the right paravaginal area. CT scan also revealed a lobulated and enlarged uterus consistent with uterine leiomyoma (Fig. ). There was no evidence of thickening of the urinary bladder wall and locoregional invasion. Flexible office cystoscopy was attempted and revealed a rounded, smooth, submucosal mass arising from the trigone of urinary bladder distorting the medial margin of both ureteric orifices. Double J stents could be passed with ease bilaterally. Retrograde pyelogram demonstrated no filling defects or dilation of the both ureters. The biopsies taken with a transurethral resection (TUR) loop showed a benign proliferation of smooth muscle in a connective tissue stroma suggestive of leiomyoma. After a careful discussion about the 2 options of further treatment, open surgical excision, and repeated TUR, the patient opted to receive open surgical excision. Then, an open local excision of this well-encapsulated tumor was performed, in which a 7.3 × 6.1 × 5.8 cm3 grayish-white tumor was removed (Fig. ) and hysteromyomectomy was also performed at the same time. Histological examination revealed intersecting fascicles of smooth muscle (Figs , ) without any evidence of malignant change and thus diagnosis of leiomyoma was confirmed. The postoperative period was uneventful, and the patient was discharged on postoperative day 10. The urinary catheter was removed after 2 weeks. Over an 8-month follow-up period, the patient made no complaint of dyspareunia and no bladder leiomyoma was detected by pelvic ultrasonography and cystoscopic examinations. |
Patient II.1 is the oldest brother (9 years old). Although he is similarly affected, he was originally misdiagnosed for cerebral palsy because of prematurity and early lower limb spasticity. Postnatally, he was admitted to the NICU because of respiratory distress; however, no mechanical ventilation was required. By the age of 4 months, his lower limbs were markedly spastic with scissoring. As a 2-year-old, his speech and motor developmental delay were notable. He could not sit unsupported for long periods, but could take a few steps while holding onto objects with evident lower limbs spasticity and marked truncal hypotonia. He started to speak when he was 3 years old. On examination, he was alert and cooperative despite delay in active speech. His gait, performed with difficulty for few steps, demonstrated tiptoe walking with limited knee and hip extension and trunk flexion.\nAt 2 and 3 years old, he was admitted to an inpatient pediatric rehabilitation and training program for 4 months each, which aimed to support motor and speech development. The response was good, as gross motor functions, including sitting, crawling and kneeling, standing, and walking for a short distance without support or orthopedic aid, became possible. He showed a more secure and significantly improved gait, and was able to pick up objects from the ground and walk up to 600 meters without supportive aids. His Gross Motor Function Measure (GMFM) chart, at the age of 3 years showed continued improvements with the percentage of sitting 100%, standing 74% and walking 56%. However, as a 7-year-old, he underwent tenotomies for his spastic hips and ankles.\nFollow up at 9 years showed him to have an unsupported gait with primary forefoot contact, knee in flexion and lumber lordosis. Features of truncal hypotonia, markedly limited ankle dorsiflexion and ankle clonus were notable.\nHis speech showed mild dysarthria. He has learning difficulties and is attending a mainstream school with extra support. Multiple and variable-sized areas of brownish skin discoloration were present at different places on his body (Fig. .1).\nof the two affected boys showed dysgenic/TCC and white matter cystic changes (Fig. .2). Images of the unaffected brother (heterozygous for the mutation) showed well-developed Corpus Callosum.\nThe clinical characteristics of our patients in comparison to other NT5C2-related patients are summarized in Table \n. |
B is a 9-year-old African boy who was born at term after a normal pregnancy. He was placed in foster care at the age of 3 years old due to severe physical and psychological violence within the family and severe neglected care. He barely had contact with his mother and his father. His family background is marked with a psychotic disorder in one first-degree relative. He quickly presented a delay in language and motor skills. An ear, nose, and throat (ENT) exploration and audiometry were performed at the age of 4 years old without any abnormalities. He did not receive speech therapy or psychomotor therapy. His academic performances were severely impaired due to his global and severe neurodevelopmental delay. School was quickly impossible and interrupted due to repetitive admission in psychiatry for tantrums and severe outbursts. Around the age of 8 years old, B presented an increase of behavioral disorders occurring at school and at home, leading to several hospitalizations for severe outbursts, self-harm, and aggressive behaviors. At the same time, he reported a progressive onset of visual and auditory hallucinations. Risperidone was initiated at 2 mg/day but without any efficacy.\nAt the admission in our unit, B presented delusional ideas, hallucinations, and mental automatism. He exhibited a disorganized and dissociative motor behavior, disorganized processes of thinking with speech disorder, and cognitive impairment. A clinical examination did not find any abnormality, and the result of a standard blood workup (CBC and liver and kidney function) was normal. After an extensive screening panel (see for details of the screening panel), no organic cause was retrieved, and the diagnosis of EOS was retained. Aripiprazole was introduced at 15 mg/day and associated with levomepromazine at 15 mg/day. However, the patient's tolerance was questioned with a severe sedation and a worsening of delusions and hallucinations. The aripiprazole plasma level showed an overdose at 835 ng/mL (therapeutic reference range: 150–500 ng/mL) regardless of the proper dosage–weight ratio. Aripiprazole was then switched to haloperidol gradually at up to 1.5 mg/day. There was no improvement regarding positive symptoms. The haloperidol and levomepromazine plasma levels showed an underdose, respectively, at 1.1 and 5 ng/mL. After excluding all known food or drug interactions, a heterogenous genotype CYP metabolism was suspected. Pharmacogenetic testing for cytochromes was performed, and it identified CYP2D6*1/*10 and CYP2D6*1/*41 heterozygous genotypes and a CYP3A5*1/*1 homozygous genotype (see ). These genotypes are associated with a partial deficiency in CYP2D6 activity and an ultra-rapid metabolizer phenotype CYP3A5, respectively. Therefore, these genotypes are genetic explanations of the high aripiprazole plasma level and low haloperidol plasma level. Given the resistance to two atypical antipsychotics and one typical antipsychotic and the severity and the burden of the psychotic features, the off-label use of clozapine was legitimate. However, during a progressive increase of clozapine dosage (300 mg/day), we reported a low clozapine plasma level (124 ng/mL). After a multidisciplinary staff consensus, we decided to add on fluvoxamine to clozapine. A few days after fluvoxamine's introduction (50 mg/day), the patient presented an extreme sedation and QTc prolongation, requiring a transfer to the pediatric cardiology department for continuous monitoring and discontinuation of the drugs. Indeed a relatively high levomepromazine plasma level (140 ng/mL; therapeutic reference range in adults: 5–25 ng/mL) was retrieved due to CYP2D6 inhibition by fluvoxamine. The clozapine plasma level was low (139 ng/mL). Therefore, the extreme sedation was secondary to an overdose of levomepromazine, leading to the discontinuation of the drug. Clozapine was introduced once again in association with a low dose of fluvoxamine (25 mg/day). Within the next days after fluvoxamine's introduction, the plasma clozapine level dramatically increased (see ), which was associated with an important improvement of the clinical state regarding positive symptoms (66% reduction in the SAPS) and a reduction of aggressive behavior. Regarding tolerance, besides the drug interactions described above, we did not report any other side effect, and no hematological adverse event was reported (see ). |
A 61-year-old female with medical history of hypertension, diabetes mellitus type 2, and chronic kidney disease stage V was transferred to our institution from an outside hospital for further evaluation and definitive management of a migrated intracardiac stent.\nShe initially presented to the outside facility with progressive dyspnea on exertion, orthopnea, and bilateral lower extremity edema. She was initially diagnosed with acute heart failure and pneumonia and treated with diuretics and antibiotics. Subsequently, a transthoracic echocardiogram was performed, which revealed a foreign body within the right ventricle. On transfer to our facility, a transesophageal echocardiogram revealed a long stent straddling the tricuspid valve from the right atrium with the other end lodged in the trabeculation of the right ventricle with severe tricuspid regurgitation ().\nOn further investigation, we learned that the patient had undergone peripheral endovascular intervention for May-Thurner syndrome with placement of a self-expanding Nitinol Protege (14 mm × 60 mm) stent to the left iliac vein 6 months prior to presentation.\nA percutaneous endovascular approach with a 35- mm Medtronic-Covidien Amplatzer Gooseneck Snare was initially attempted to retrieve the migrated stent. However, the snared proximal segment fractured, leaving behind 2 stent fragments. After ensuring there was no myocardial perforation or pericardial effusion with intracardiac ultrasound, the patient was referred for surgical extraction via median sternotomy with use of cardiopulmonary bypass.\nDuring the operative procedure, the stent was found to be densely adherent to the tricuspid leaflets and the subvalvular apparatus, with majority of the primary chords to the anterior and posterior leaflets ruptured (). After successful extraction of the stent and native tricuspid valve, she underwent valve replacement with a 29-mm Carpentier-Edwards bioprosthetic valve. Her postoperative course was complicated by hemopericardium secondary to anticoagulation resulting in cardiac tamponade that was drained percutaneously, and small thromboembolic cerebellar stroke from atrial fibrillation. She was discharged to an inpatient rehabilitation facility and did well on 8-month follow-up. |
A 31-year-old white man of European ancestry who was on insulin treatment for diabetes mellitus type 1 since childhood presented with right-sided back pain. Computed tomography (CT) disclosed a 7 × 11 cm retroperitoneal mass compressing the vena cava and additional mediastinal lymphadenopathy of 2.1 cm in size. Further clinical evaluation revealed a right-sided testicular mass and a thrombosis of the vena cava with extension of the intravascular clotting into both iliac veins (). The serum level of beta human chorionic gonadotropin (bHCG) was increased to 36.6 U/L (upper limit of norm, 2.5 U/L); alpha fetoprotein (AFP) and lactate dehydrogenase (LDH) were within normal limits. The management consisted of antithrombotic treatment and inguinal orchiectomy. Histologic evaluation revealed pure testicular seminoma (). Subsequently, the patient received chemotherapy with 3 courses of cisplatin, etoposide, and bleomycin. Upon restaging, the bHCG serum level had normalized and the mediastinal mass had disappeared, while the retroperitoneal mass had shrunk to 3 × 3 cm. One year thereafter, the residual mass had completely disappeared. With respect to the thrombotic occlusion of the vena cava, multiple collateral veins had evolved in the retroperitoneum. Later, during follow-up, the patient developed hypogonadism with the need for testosterone replacement therapy. Nine years after initial treatment, the patient presented with abdominal pain. Abdominal CT revealed an oval mass sized 5 × 6 cm located in the retroperitoneum caudal to the left renal vein and between vena cava and abdominal aorta (). No other neoplastic deposits were observed. The serum levels of bHCG, AFP, and LDH were within normal limits. The findings were considered to represent a late relapse of seminoma mainly because the newly evolved mass was located right at the site of the initial metastasis 9 years priorly. Accordingly, the patient received a chemotherapy regimen consisting of three cycles of cisplatin, ifosfamide, and etoposide. Upon restaging with CT, no significant change of the retroperitoneal mass was noted. Therefore, surgical excision was performed. Despite the complex intraabdominal venous collateral network, a complete surgical excision of the mass was accomplished. Postoperative recovery was uneventful, and, 6 months thereafter, the patient is in good condition and is pursuing his work again.\nCut section of the specimen revealed a firm white-greyish mass with several bone-like appearing tiny spots and a clearly demarcated rim (). Histological evaluation disclosed loosely whorled and short intersecting fascicles composed of cells with round to oval nuclei, with small nucleoli and a small-to-moderate amount of mostly clear cytoplasm. Along the edge, some mature bone formation was observed. Noteworthy, in several areas of the specimen, the cells were more crowded, the nuclei showed atypia, and mitotic activity was observed. Immunohistochemical stains with glial fibrillary astrocytic protein (GFAP) demonstrated positive cytoplasmic staining; S100 showed focal nuclear and cytoplasmic positive staining. Germ cell tumour markers (Oct3/4, PLAP, SALL4, Glypican 3, and CD30), pancytokeratin (AE1/AE3), and neurofilament muscle specific markers (Desmin) as well as CD34, CD117, SOX10, MDM2, and EMA were all negative. The proliferation marker Ki-67 was positive in less than 5% of cells. Given the overall rather benign neurogenic differentiation with focal increased cellularity, nuclear atypia, and mitotic activity, the diagnosis of neurofibroma with transition into a low-grade malignant peripheral nerve sheath tumour (MPNST) was made (Figures and ). No components of seminoma or other germ cell tumours were detected in the completely embedded specimen. Fluorescence in situ hybridization (FISH) examination revealed excess genetic material of chromosome 12p (isochromosome 12p) (). This finding was considered as evidence for a somatic type malignancy occurring on the basis of a germ cell tumour metastasis in the retroperitoneum rather than a primary soft tissue tumour. |
A 51-year-old female with the history of ESRD on home hemodialysis, antiphospholipid syndrome on warfarin, systemic lupus erythematosus, diabetes mellitus, hyperparathyroidism, and bowel obstruction followed by the surgical repair in the past admitted to the hospital with severe pain and hardness in the abdominal wall as shown in . The patient was initially diagnosed with abdominal wall hematomas and was advised to stop the warfarin. Even with warfarin on hold, the lesions increased in size, became more painful, and started to ulcerate as showed in . The patient has been on home hemodialysis for 2 years prior to the onset of symptoms and undergoes 5 times a week dialysis treatment. The medications include calcitriol 0.25 µg daily, ergocalciferol 50 000 IU weekly, omeprazole 40 mg daily, oxycodone-acetaminophen 10-325 mg every 6 hours as needed for pain, ferric citrate 210 mg 2 tablets with meals and 1 tablet with snack, pentoxifylline 400 mg daily, prednisone 5 mg daily, febuxostat 80 mg daily, hydroxychloroquine 200 mg daily, warfarin 2 mg daily, insulin glargine 25 units every day, and insulin sliding scale. The patient has been on warfarin for 10 years because of antiphospholipid syndrome.\nThe patient was seen at home dialysis clinic 2 weeks after the hospital admission, and clinical diagnosis of calciphylaxis was made. The vital signs on presentation were temperature of 97°F, pulse rate of 96 beats per minute, respiratory rate of 18 breaths per minute, and blood pressure of 145/82 mm Hg. Patient was in distress from pain, abdominal examination revealing an eschar of 13 cm × 6 cm on right lower abdomen with purulent drainage and surrounding erythema as shown in . Firm subcutaneous lumps were felt bilaterally on the lower abdomen, which are tender to palpation. Rest of the examination was nonsignificant.\nThe laboratory data revealed white blood cell count 10.9 × 103 cells/µL, hemoglobin 10.6 g/dL, sodium 135 mEq/L, potassium 4.5 mEq/L, carbon dioxide 23 mEq/L, blood urea nitrogen 69 mg/dL, creatinine 7.7 mg/dL, parathyroid hormone 333 pg/mL, calcium 9 mg/dL, phosphorus 5.7 mg/dL, calcium phosphorus product elevated at 51.3, vitamin D level 16.4 ng/mL, and albumin level 3.1 g/dL. Patient was meeting the goal of dialysis adequacy. The laboratory data before, during, and after presentation are summarized in . Home hemodialysis prescription is summarized in .\nThe patient’s calcium supplements were stopped. Patient was started on sodium thiosulfate 3 times a week with home hemodialysis. The home dialysis frequency was increased to 6 times a week. Patient was referred to the dermatology, hematology, endocrine surgery, and wound care. The patient was resumed back on warfarin as the benefits of being anticoagulated outweighed the risks. Hematology agreed with plan to continue warfarin. Dermatology confirmed the diagnosis of calciphylaxis and deferred the biopsy because of extensive necrosis. The patient was managed meticulously with wound care with frequent dressing changes and periodic debridement. The course of wound healing was described in images with timeline (, b, and c). Endocrine surgery deferred parathyroidectomy as the parathyroid hormone was in acceptable range. The ulcers completely resolved in 4 months (), wound care was discontinued, sodium thiosulfate infusions were stopped, and the patient’s home dialysis frequency was decreased to 5 times a week. |
A 10 year-old girl presented with the complaint of palpitation to a cardiologist. She had normal physical examination and laboratory tests, except tachycardia (heart rate = 130 per minute) and low TSH levels (0.005) with normal T3 (9.46) and T4 (145). She was referred to endocrinologist for possible hyperthyroidism evaluation. The thyroid gland was normal size, with no nodularity. She was diagnosed with possible thyrotoxicosis, but due to the normal physical examination, she underwent thyroid scan to rule out possible thyroiditis, which did not show any uptake in the thyroid gland, while there was an increased uptake in the right ovary (). Pelvic trans-abdominal sonography showed a heterogeneous complex solid mass of 113 × 112 × 100 mm with volume of 670 cc in the right ovary with no ascites. The patient had no complaint of abdominal pain or pelvic pain or abnormal uterine bleeding.\nShe was treated with methimazole 10 mg daily and propranolol 40 mg daily and were candidate for surgery after being euthyroid. The patient was referred to a gynecologist with the possible diagnosis of struma ovarii for further evaluation. She underwent right oophorectomy with the presumption of teratoma combined with thyroid-stimulating hormone (TSH)-suppressive therapy following treatment with I131. Total thyroidectomy was performed to permit evaluation for metastatic disease and monitoring for recurrence by thyroglobulin levels. The pathology report of the ovary mass indicated teratocarcinoma with 60% well-differentiated follicular thyroid carcinoma and 40% well differentiated follicular-variant with tumor necrosis, microscopic capsular invasion and peritumoral lymphovascular invasion, considering stage IC of PTC () and the thyroid gland did not show pathologic features of PTC.\nFurther evaluation with whole body scan with Iodine 123 (I123) showed metastasis to lymph nodes. She had high levels of thyroglobulin and received iodine therapy (150 mCi) twice. In the follow-up whole body scan, there was no trace of iodine uptake and the patient was symptom free.\nThe patient is now under treatment with levothyroxine 0.1 mg daily. Following 8 months after surgery and iodine therapy, she is totally symptom free. |
A 72-year-old man with a previous history of schizophrenia, acute subdural hematoma, duodenal ulcer and paralytic ileus was referred to our hospital for PEG. He has been hospitalized in a psychiatric institution for the past 40 years and has been bed-ridden for the past 5 years due to the subdural hematoma. His oral intake gradually decreased and recurrent episodes of aspiration pneumonia prompted his attending physician to recommend PEG.\nPhysical examination and blood laboratory markers were unremarkable and he was tube-fed with a nasogastric tube (NGT) for 7 days before undergoing the PEG procedure. Enteral feeding via NGT was well tolerated. Endoscopic imaging before the procedure revealed a deformed duodenal bulb with moderate stenosis () and gastric ulcer scars (). PEG was performed using the push technique on the anterior wall slightly towards the greater curvature of the stomach ().\nAlthough the procedure ended uneventfully, emesis of liquid infusion into the gastrostomy tube occurred the following day, causing aspiration, asphyxiation and cardiac arrest. Cardiopulmonary resuscitation was successful and the patient received mechanical ventilation and total parenteral nutrition until he was stable. After receiving intensive care, the patient’s condition stabilized and he was weaned from mechanical ventilation on day 70 after PEG procedure. After multidisciplinary consultation and discussion, the legal guardian chose enteral nutrition via PEG-J over total parenteral nutrition (TPN) via a central venous access port.\nPEG-J was performed on day 83 (post-PEG) using a 20-Fr size all-silicone jejunal tube with gastric decompression function (Cliny PEG-J Catheter by Create Medic Co., Ltd, Yokohama, Japan). The procedure was performed in an interventional radiology suite with the use of fluoroscopy. An ultrathin endoscope was inserted into the gastric lumen through the gastrostomy puncture site after removal of the PEG tube and the PEG-J tube was placed so that the tip of the tube goes beyond the ligament of Treitz. shows an abdominal X-ray image taken after the procedure, revealing the PEG-J tube’s position after placement. The green arrowhead indicates the entry point of the PEG-J tube through the gastrostomy puncture site, the yellow arrowhead points to the radiopaque marker before the pylorus and the red arrowhead shows the tip of the PEG-J tube in the jejunum.\nTube feeding resumed in a gradual manner until gravity-controlled drip feeding was achieved. As shown in , improvement of nutritional biomarkers such as total lymphocyte count (TLC), serum cholinesterase (ChE), total cholesterol (TC), triglyceride (TG), total iron binding capacity (TIBC), total protein (TP) and albumin levels (Alb) after PEG-J procedure was observed. After achieving stable tube feeding, the patient was discharged without further complications. |
We report of a 58-year-old woman, who did not suffer from any pre-existing medical conditions, with the only exception of being mildly overweight (BMI 31.25). In particular, she did not smoke, she had no history of migraine, and she did not receive any hormone replacement therapy after menopause, that occurred at age 51. One hour after the execution of her first-time (later revealing as negative) nasopharyngeal swab, while driving home, she likely experienced amnesia: the bystanders reported that the woman pulled over the car, lowered the window and called for rescue, telling she was unable to find the right direction; when she was asked where she was going, she answered she did not remember and began to behave anxiously. The bystanders reported that she could tell her name and other personal information, and that she could herself remember the unlock code of her phone and call her husband. The husband noticed she was disoriented, repeatedly asking the same questions about where she was and what she was doing. This episode lasted for about two hours then reverted; the day after, she was conducted to our attention for evaluation. A memory gap for the episode persisted: she could remember having had the swab tested, then having paid for the test, having left the hospital, and reached the car after the procedure, but she could not remember the following events, including those witnessed by the by-standers and including the call to her husband and its arrival on the place where she had left her car.\nNeurological examination was normal. Brain MRI, performed one day after TGA, revealed a hippocampal punctate area of DWI restriction (Fig. ), confirming the clinical suspect. Supra-aortic vessels Duplex ultrasonography and EEG were negative, ruling out mimics. The strict temporal relationship between nasopharyngeal swab and TGA may have been simply coincidental, but we are usually prone to investigate each time carefully the triggers for TGA, since TGA recent anamnesis is often full of peculiar, even bizarre, but usually biologically fitting antecedents. |
A 22-year-old Caucasian female with no past medical history presented to the emergency department after being found by her boyfriend unconscious at home. The patient’s last known normal was 11 h prior. The mother reported that the patient had complaints of generalized malaise and fatigue over the last 2 months, but otherwise she was healthy. No sick contacts were reported. Family history was significant for Hashimoto’s thyroiditis in the mother and celiac disease in the father. Patient had a two pack year history of smoking and had recently quit 3 months ago. The only medication patient was taking was an oral contraceptive. She was an occasional alcohol and marijuana user but did not use any other recreational drugs.\nOn presentation to the emergency department, blood pressure was 90/61 mm Hg, heart rate was 127 beats per minute, temperature was 38.9 °C, respiratory rate was 28 breaths per minute and saturation was 87% on room air. The patient was subsequently intubated for airway protection. On physical exam, pupils were equal and reactive, 2/6 holosystolic murmur was heard on the left lower sternal border, and pulses were diminished throughout. Extremities revealed cold, dusky fingers with mottling of toes bilaterally. On neurological exam, there were no spontaneous movements of left upper extremity and minimal movements of the left lower extremity.\nLabs on admission are listed in .\nThe 2D transthoracic echocardiogram showed an ejection fraction of 15% and severe mitral regurgitation. Given the altered mental status, CT of head was obtained which showed extensive multifocal areas of hypoattenuation throughout the bilateral frontal, parietal, occipital and right temporal lobes consistent with multifocal infarction concerning for cardioembolic etiology ().\nGiven the fever, leukocytosis and suspected cardioembolic phenomenon causing infarctions in the brain, there was concern for bacterial endocarditis. Hence, transesophageal echocardiogram (TEE) was performed which showed a 1 cm vegetation at the anterior mitral leaflet (, Supplementary videos 1 and 2, www.cardiologyres.org). The patient was started on broad spectrum antibiotics, vancomycin, and ceftriaxone for presumed bacterial endocarditis with systemic emboli. However, blood cultures obtained from two different sites (peripheral and antecubital) prior to administration of antibiotics remained negative throughout the hospital stay.\nAutoimmune workup was negative. Hematological workup showed an isolated lupus anticoagulant with normal anti-cardiolipin antibodies. Repeat lupus anticoagulant a few days later was negative. Patient likely had disseminated intravascular coagulation that was resolving. Thus, hypercoagulable workup was not performed with presumption of false positive findings in the setting of critical illness along with a vegetation that was showering emboli.\nOn day 8, metabolic laboratory abnormalities improved but patient’s neurological status worsened and she no longer opened her eyes spontaneously. That she had multifocal infarctions based on her initial head CT scan was already known. Although her initial head CT had shown multiple infarctions, her abrupt neurological decline was unclear. This prompted us to perform an MRI of the brain. It showed increased signal on DWI throughout bilateral frontal, parietal, and occipital lobes consistent with new/ongoing acute infarcts (). After discussion with family and consultants, we decided to start her on therapeutic anticoagulation with heparin.\nOn day 22, patient’s clinical status continued to deteriorate. There was no neurological improvement. Despite therapeutic anticoagulation, patient was found to have a left upper extremity arterial thrombus requiring emergent thrombectomy.\nHemoglobin remained stable but her platelets continued to drop. Heparin-induced thrombocytopenia was on the differential given the drop in platelets while the patient was on heparin. Therefore, heparin was changed to argatroban. Serotonin release assay was negative but PF4 antibody was positive.\nOn day 35, patient’s clinical status continued to deteriorate further with ongoing systemic clotting. She now had bilateral internal jugular vein clots as well as cyanotic extremities. After a palliative care meeting with the family, decision was made to withdraw care and the patient expired. An autopsy was performed given the complexity of the case. Autopsy findings were the following: ischemic necrosis of most organs including the liver, lungs, and adrenal glands; cardiomegaly with atrial and ventricular thrombi, mitral vegetation with pericardial effusion; right lung hemorrhage infarct with vascular emboli; hepatomegaly with congestion, necrosis; kidneys with extensive necrosis and vascular emboli; mitral valve vegetation, 10 cm in size, had lots of necrosis and the mitral valve tissue showed focal necrosis without the presence of bacterial or fungal organisms; myocardium was normal without lymphocytic infiltrates or granulomas; clusters of fibrinous thrombi with lymphoid myeloid cells adjacent to the myocardial tissue possibly related to extensive vegetations. These findings were consistent with non-bacterial thrombotic endocarditis (). |
A 60-year-old woman who underwent abdominal computed tomography (CT) for a routine health check-up was found to have a 1.8-cm cystic lesion in the tail of the pancreas. The patient was admitted to our center for further examination. She had no symptoms related to the lesion. Her abdomen was soft and flat, and no tenderness or rebound tenderness was observed. Complete blood count, electrolytes, and liver function tests were within normal limits. There was no evidence of pancreatitis. Both the serum carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) levels were normal. A dynamic CT of the pancreas showed multiple cystic lesions in the tail of the pancreas and the largest cystic lesion showed a lobulated contour without communication with the pancreatic duct. The lesion measured 2.2 cm in maximal diameter and had several small calcifications. There were no indications of invasion into the surrounding tissues or dilatation of the main pancreatic duct (). Magnetic resonance cholangiopancreatography revealed a 2.2-cm round cystic lesion in the tail of the pancreas which had no communication with the main pancreatic duct. The main pancreatic duct was not dilated (). Endoscopic ultrasonography revealed a 2.2×1.3 cm-sized cystic lesion and a 5×2.5 mm-sized mural nodule; the septation within the cyst and the cyst wall was thin; and the cyst was well-demarcated from the surrounding tissues. The pancreas showed normal parenchymal echogenicity and the main pancreatic duct had a normal caliber (). We diagnosed the cystic lesion as an intraductal papillary mucinous neoplasm.\nTo treat this lesion, we performed laparoscopic spleen-saving distal pancreatectomy. During the operation, the cystic lesion, approximately 3 cm in diameter, was found arising from the border between the body and tail of the pancreas without any infiltration into the surrounding tissues. Most of the lesion was located within the pancreatic tissue, and its border was well-defined. There was no peripancreatic lymph node enlargement. The preplanned surgery was performed uneventfully. The patient showed the usual routine postoperative course and was discharged 10 days after surgery. The patient quickly returned to her normal activities.\nOn examination of the resected specimen, the cystic lesion of the pancreas (2×1.8×1.5 cm) was multi-locular with thin septa (). A well-defined, oligo-locular cystic mass was in the pancreatic tail without a solid portion. Inner surface of the cyst was smooth and glistening and the cyst contained serous fluid. No connection with the main pancreatic duct was identified. Histologic examination revealed that the cysts had linings ranging from flat squamoid cells to transitional cells with non-keratinization (). No tall columnar mucinous cells were identified. No associated lymphoid or splenic tissue was present. The cyst walls were composed of relatively thin fibrous issue. The epithelia of the cysts reacted negatively with MUC 1 and MUC 6 (a marker for intercalated duct centroacinar cells), and positively with CK7 and p63, and negatively with inhibin. Ki-67 represented about 1% (). The results of these immunohistochemical stains are summarized in . The final diagnosis was confirmed as squamoid cyst of the pancreas. |
An 11-day-old girl with an uncomplicated birth was noted by her parents to have a lump on her lower back, prompting further workup by her pediatrician. A physical exam revealed a 4–5 cm soft, nontender mass in the lumbar spine with hyperpigmented changes []. An ultrasound was performed, which showed the absence of sacral lamina and dorsal elements with an associated intraspinal mass and bilateral hydronephrosis. She subsequently underwent an MRI showing a spinal dysraphism of the sacrum associated with a large cystic intraspinal mass, concerning for a lipomyelomeningocele versus a terminal lipomyelocystocele. Her motor exam showed normal strength in both lower extremities but poor deep tendon reflexes and a slightly distended abdomen. Further urological workup revealed the presence of grade 4 vesicoureteral reflux for which she was started on a clean intermittent catheterization protocol. After a thorough discussion about the risks and benefits of operative intervention, she was taken to the operating room (OR) for debulking of the lipomatous mass and untethering. Electrical stimulation and neuromonitoring were used to monitor function. The dural tube was reconstructed using a synthetic patch duroplasty. No cyst was encountered during the debulking. There were no intraoperative complications, however, 1 week postoperatively she developed a cerebrospinal fluid (CSF) leak, which precipitated a Klebsiella wound infection. She returned to the OR for irrigation and debridement, placement of a lumbar drain, and revision of the dural closure. Postoperatively, she remained intubated and prone for 1 week, at which point the lumbar drain was removed and she was extubated. She had no further leakage from the wound and was discharged approximately 5 weeks after her first surgery. At 18-months’ postoperative follow-up, her wound was well healed, and she retained normal strength in her bilateral lower extremities. She progressed to grade 5 vesicoureteral reflux with moderate hydronephrosis without urinary tract infections, managed by clean intermittent catheterization every 3 hours. |
A 35-year-old female gravida 9 para 5 presented to the emergency department with right lower quadrant pain and fever for two days. She was postpartum day 8 and had given birth to twins after an uncomplicated spontaneous vaginal delivery. She was an active smoker and smoked half pack per day for 20 years, had a history of substance abuse, and was a hepatitis C carrier. Her vital signs and physical examination was unremarkable except for tenderness on palpation of the right lower quadrant of the abdomen. Pelvic examination revealed uterus, cervical, and right adnexal tenderness. A transvaginal ultrasound was performed that showed a small amount of fluid and blood products within the endometrial cavity. Computed tomography (CT) of the abdomen and pelvis with contrast revealed a markedly dilated right ovarian vein containing abnormal soft tissue attenuation material throughout, with fat stranding along the course of the vein signifying thrombophlebitis. A right ovarian vein thrombus extending into the inferior vena cava at its insertion, measuring 1.5 cm was found (Figure ). There was no past medical history or family history of hypercoagulability or blood clots, except for blood clots in a sister who was on oral contraceptive pills and an uncle who had cancer. With an initial diagnosis of septic thrombophlebitis, the patient was started on enoxaparin 70 mg every 12 hours and piperacillin-tazobactam. On day 4, piperacillin-tazobactam was stopped, as she was afebrile for 48 hours.\nThe option of bridging to warfarin was offered but due to the necessity of transportation for international normalized ratio (INR) monitoring it was declined by the patient. Rivaroxaban was not an option as she was breastfeeding. Therefore, it was recommended to continue enoxaparin for at least three months, with outpatient follow-up with hematology. The patient did not follow up as outpatient. She took enoxaparin for three months and then stopped by herself. After six months from her diagnosis of right ovarian vein thrombosis, she presented to the emergency department for right lower quadrant pain. Her transvaginal ultrasound was unremarkable. A CT of the abdomen and pelvis showed a normal caliber of the right ovarian vein with resolution of the intraluminal thrombus. |
A 45-year-old, multiparous woman (gravida 3, para 1, G3P1) presented with a 5-year history of large amounts of vaginal discharge. The ThinPrep cytology test revealed moderate inflammation. Several transvaginal ultrasonography scans revealed an edematous cervix and multiple cysts with a honeycomb appearance (). The inner cervical wall was not smooth and the tumor marker levels were within the normal range. Following cervical conization for the cervical cysts, the biopsies revealed chronic cervical inflammation with the presence of retention cysts and squamous metaplasia in the fundic portion of the cervix. Subsequently, the patient underwent laparoscopic cystectomy and biopsy, hysteroscopy, fractional curettage and cervical biopsy. The histopathological examination revealed chronic inflammation of the cervical mucosa. However, the vaginal discharge did not subside. The patient then underwent a pelvic magnetic resonance imaging (MRI) examination, which revealed multiple cervical cysts and hydrops in the pelvic cavity. Medically the patient was in good condition and her history only revealed an appendectomy performed in 1983. Following admission to our department, the gynecologic examination showed large amounts of vaginal discharge and cervical hypertrophy, with no other abnormal findings. The patient underwent total abdominal hysterectomy and the fast-frozen cervical biopsy revealed the presence of adenocarcinoma; thus, bilateral salpingo-oophorectomy and pelvic lymphadenectomy was also performed.\nGrossly, the cervix was thickened and hard with multiple retention cysts, with no other abnormal macroscopic findings (). The microscopic examination revealed cervical mucilaginous glands that were irregular in size and shape with increased apophysis, part of the glands were surrounded by a loose edematous or desmoplastic stromal response, the glands typically exhibited deep invasion of the cervical wall and were adjacent to the cervical adventitia. The glandular epithelial cells exhibited foci of heteromorphism. The parametrium and the pelvic lymph nodes showed no evidence of malignancy. The tumor was staged as Ib2 MDA according to the FIGO classification. Subsequently, cervical and pelvic radiotherapy was performed. At the last follow-up the patient was disease-free. |
This case corresponds to a 16-year-old Caucasian male, who was diagnosed with PGC at the age of 5 and was brother of case 2. He was on medical treatment (one drug) for elevated IOP from age 5. At presentation in HCSC, an enlarged corneal diameter was the only sign detected. At the age of 16 a goniotomy in the OS was performed because of poor IOP control despite being on three topical glaucoma medications. Five months later, a trabeculectomy was required in the OS, achieving IOP control since then without medication. The IOP in the OD has been controlled up to the present with three topical glaucoma medications, and no previous surgical procedures were required.\nThe trabeculectomy from the OS of this patient contained two pieces of tissue, one corresponding to a part of the sclera and another containing the rest of the sclera, the SC and the TM. In semithin sections, we observed a SC with an open lumen, a quite compact CTM, and an UTM with well-developed intertrabecular spaces “”. In either pieces of sclera, we found no collector channels. The TEM revealed that most of the SC endothelial cells had disappeared with only debris of necrotic cells being observed “”. The JCT was composed of alternating layers of abundant ground substance, elastic-like fibers, fibrillary collagen and necrotic stellate cells “”. The CTM was difficult to recognize because the tissue was quite disorganized “”. In some areas the trabecular beams were separate and the zones that would correspond with the inter-trabecular spaces were occupied by the remains of necrotic cells and by collagen that had been detached from the trabeculae “”. In other areas the trabecular beams were fused, the boundary between them being recognized by the presence of necrotic cells debris “”. The trabecular beam had basal membranes, many elastic-like fibers, fibrillary collagen and abundant ground substance “”; the latter gave to the trabecular beam an “empty” appearance “”. The endothelial cells that delineate the trabecular beams were mostly necrotic “”, and those remaining showed autophagic activity “”.\nAs occurs in the CTM, the UTM core had an “empty” appearance in some regions due to the presence of abundant ground substance “”. In addition, there were elastic-like fibers and abundant type VI collagen, which intermingled with the collagen of the basal membranes (type IV) “”. In some zones the whole trabecular beam was filled up by collagen VI “”. The type VI collagen was degraded in the periphery of the trabecula and broken away from it “”. All endothelial cells lining the trabecular beams had disappeared due to cell death by necrosis “”. In this surgical piece of trabeculectomy the ciliary muscle was not included. |
A 5-yr-old girl was admitted to Sanggye Paik Hospital due to chronic productive cough and greenish sputum. Eight months prior to the current hospitalization, she developed cough, sputum, rhinorrhea, and fever which were associated with frequent respiratory tract infection.\nShe was born by vaginal delivery. A meconium staining of the amniotic fluid was observed at delivery, but the evidence of meconium aspiration was not clear. Her birth weight was 2.45 kg, which was small for her gestational age (40 week). She had an older brother while the CF in family history or CF-related symptoms were found negative from her older brother. The patient had been suffered from diarrhea persisted from 3 months of age, which led to hospitalization and poor weight gain since then. She had been hospitalized for three times from 2 yr of age due to recurrent pneumonia and respiratory problems. Her body weight and height on admission were 12.2 kg (<3 percentile) and 95.9 cm (10-25 percentile), respectively.\nChest radiographs and computed tomographic (CT) scans showed bilateral diffuse bronchial wall thickening and symmetric bronchiectasis in both lung fields (). The diseases caused by immunodeficiency or allergic reactions were ruled out by her normal complete blood cell count, serum immunoglobulin levels and specific IgE levels. P. aeruginosa was cultured from her sputum. Abdominal ultransound and pancreaticobiliary CT scan revealed moderate to severe fatty liver and atrophy of the pancreas. Because she had chronic diarrhea, poor weigh gain, and atrophy of the pancreas as well as pulmonary symptoms, the patient was suspected to have CF.\nTo confirm the diagnosis, the sweat chloride concentration was measured by a quantitative pilocarpine iontophoresis sweat test recommended by the National Committee for Clinical Laboratory Standards, USA (National Committee for Clinical Laboratory Standards. Sweat Testing: Sample Collection and Quantitative Analysis. Villnova, Pa: National Committee for Clinical Laboratory Standards; 1997 NCCLS document C34-A.). The test was repeatedly performed at Yongdong Severance Hospital, Yonsei University on two separate days. The average sweat chloride concentrations on both forearms were 78.3 mM/L and 99.0 mM/L in two separate measurements with a one-week interval. These values were over the reference limit (<40 mM/L) and highly suggestive of CF.\nAn initial genetic screening was performed on the 11 mutation/polymorphism loci of CFTR gene previously identified in the Korean population () and on the 10 most common disease-associated loci in Caucasians using the SNaP Shot method (Applied Biosystems, Foster City, CA, USA). However, none of the above mutations were identified in the patient's gene. Therefore, we next scanned the genetic variations in the CFTR gene using the denaturing gradient gel electrophoresis (DGGE) and subsequent nucleotide sequencings to find unknown CFTR mutations as previously detailed (). Interestingly, a non-synonymous L441P mutation of CFTR was identified in one allele by DGGE and consecutive nucleotide sequencings (). Although we performed a comprehensive search for the entire coding regions and exonintron splicing junctions of CFTR gene, no additional mutations were found. Among family members, patient's mother was available for genetic tests. However, no CFTR mutations including L441P were found in the blood samples from patient's mother (). Therefore, the L441P mutation was assumed to come from patient's father.\nIt has been reported that the L441P mutation of CFTR is associated with CF in Japan and its allele frequency was estimated around 4.5% among CF patients in Japan (). However, the molecular pathogenic mechanisms of the L441P mutation of CFTR are currently unknown. Therefore, we investigated the disease-causing mechanism of L441P using an integrated molecular and physiologic study. The CFTR plasmid carrying a L441P mutation was constructed by sitedirected mutagenesis using QuickChange kit (Stratagene, La Jolla, CA, USA) with pCMV-CFTR plasmid according to the manufacturer's protocol. The residue thymine base at 1454 of CFTR was substituted with cytosine and the mutagenic sequence was confirmed by the DNA sequencing. The total amount of CFTR protein was compared in HEK 293 cells transfected with plasmids for wild type CFTR or CFTR carrying the L441P mutation by immunoblotting of total cell lysates. In addition, the amount of surface-expressed proteins was examined by the immunoblotting of surface biotinylated proteins.\nDuring secretory pathway, the polypeptidic chain of CFTR undergoes post-translational modifications at its glycan moiety in the Golgi complex to produce the fully glycosylated mature form, also known as band C, of about 170-180 kDa (). In immunoblotting of cell lysates, most of the wild type CFTR protein was detected as the fully glycosylated mature form, whereas virtually all of L441P mutant proteins appeared as the ER core-glycosylated form of about 150 kDa, also known as band B (). These results imply that the L441P mutant CFTR protein has a defect in the ER-to-Golgi trafficking of the secretory pathway of membrane protein. Consequently, the surface biotinylation results revealed that the L441P mutant protein failed to reach the plasma membrane, whereas the fully glycosylated form of wild type CFTR was expressed on the cell surface ().\nTo investigate the intracellular localizations of the mutant CFTR, immunostaining was performed in HEK 293 cells transfected with plasmids for wild type CFTR or CFTR carrying the L441P mutation. The CFTR protein was stained with anti-CFTR 24-1 antibodies (R&D Systems, Minneapolis, MN, USA) and fluorescein isothiocyante-conjugated secondary antibodies. The ER-resident calnexin protein was stained with anti-calnexin antibodies (Abcam, Cambridge, MA, USA) and rhodamine-conjugated secondary antibodies, and then images were obtained with a Zeiss LSM510 confocal microscope. As shown in , wild type CFTR was mainly localized on the plasma membrane (arrow heads). On the other hand, L441P mutant protein was found in the ER, which was confirmed by co-localization with calnexin, an ER membrane protein ().\nIt is well known that CFTR protein has a cAMP-activated Cl- channel function (). Thus, the cAMP-activated chloride channel activities of CFTR-expressing HEK 293 cells were measured in the whole cell configuration. Treatments with the adenylyl cyclase activator forskolin (FSK) produced a large inward current in NMDG-Cl solutions (). The I-V relationship measurements revealed the typical CFTR current of a linear I-V relationship when a ramp pulse from -120 mV to +120 mV was applied at peak current (). The current density of wild type CFTR at a -30 mV holding potential was 37.48±6.20 pA/pF. However, the cAMP treatment (5 µM FSK) failed to activate the Cl- currents in cells transfected with L441P mutant CFTR (). |
A 61-year-old non-symptomatic Japanese housewife presented to our hospital because of an abnormality on chest radiography undergone during a lung cancer screening program in her city of residence. She had no family history of breast cancer and was taking no medications. She had a painless 1.5-cm elastic hard palpable mass in her left breast C area. There were no abnormalities in the clinical laboratory examination. Chest computed tomography (CT) showed old left pulmonary inflammation, and also revealed an ovoid homogenous mass with regular margin measuring 12 mm in her left breast C area (Fig. a). Mammography showed a well-defined high-density ovoid-shaped mass without calcification that measured 14 mm × 11 mm in the M area on the mediolateral oblique view (Fig. b) and measured 16 mm × 11 mm in the O area on the craniocaudal view. US showed a thick, disk-shaped, slightly inhomogeneous low-echogenic tumor (Fig. c–e). The tumor had a defined margin with lateral echo. The tumor size was 15.8 mm × 14.1 mm × 7.5 mm.\nFive years before this consultation, she had undergone a mammographic examination following a breast cancer screening program performed by her city residence that incidentally revealed a density mass in her of left breast that measured 16 mm × 12 mm in the M area on the mediolateral view and 15 mm × 14 mm in the O area on the craniocaudal view (Table ). She then underwent a breast cancer check-up at our hospital. Ultrasound showed a tumor size of 14.6 mm × 14.2 mm × 6.6 mm. It was diagnosed as class II on fine needle aspiration biopsy.\nThree years before this consultation, she had gone to another hospital to undergo another breast cancer screening program performed by the city (Table ). Mammography at that time showed no remarkable change in the size or density of her left breast tumor. Dynamic CT imaging revealed a well-defined homogenous tumor that showed a pattern of gradual increase, which is known as the benign pattern (Fig. ). A core needle biopsy performed that same day showed FA with a pericanalicular pattern but no pattern of malignancy even when assessed retrospectively. The specimen showed the proliferation and dissociation of small ductal epithelial cells (Fig. a) and a reduction in the number of myoepithelial cells on p63 immunohistochemical staining (Fig. b).\nAt the present consultation, mammography showed no remarkable change in the size or density of the tumor, although the secular change in background mammary tissue was reduced (Table ). However, because the size of the tumor by US was slightly enlarged, we decided to perform MRI. Dynamic MRI showed a pattern of malignant enhancement, that is, a pattern of rapid enhancement and washout (Fig. f). The tumor showed high signal intensity on T2-weighted and diffusion-weighted images. So, we decided to perform a core needle biopsy, and the subsequent histopathological findings such as those of dense, epithelial proliferation with small ductal or cribriform growth pattern favored malignancy over its benign counterparts. The clinical stage was T1N0M0, Stage I. Then, breast-conserving therapy with sentinel lymph node biopsy of the left breast tumor was performed. The gross appearance of the excised specimen showed a well-encapsulated ovoid tumor measuring 15 mm in diameter with a gray-white, slightly firm, and homogenous cut surface (Fig. a). Histologically, a final diagnosis of low-grade DCIS within FA was obtained. The tumor encapsulated with thin fibrous tissue was composed of an intense proliferation of ductal cells showing cribriform, tubular, and solid growth patterns with fibroadenomatous myxoid stroma (Fig. b–f). The specimen's immune profile was estrogen receptor (ER), +(90%); PgR, −(3%); human epidermal receptor-2 (HER-2), −(membranous reaction, 0+); MIB-1 labeling index, 1% to 3%. Thus, the final specimen showed low-grade DCIS within a FA.\nThe patient underwent postoperative breast tangential irradiation therapy of 50 Gy for 5 weeks without any side effects and showed no signs of recurrence for 2 years. |
A 63-year-old male presented to the emergency department of our institution in January of 2011 with an open pilon fracture of his left tibia along with a fracture of the distal fibula (). His medical history was significant for diabetes mellitus being treated with insulin therapy. The patient's fracture was immediately treated in the operating room with an open reduction and internal fixation with plates and screws. Postoperatively ( and ), the patient received intravenous antibiotic therapy with ceftriaxone, amikacin, and metronidazole for 3 days and the patient was discharged from the hospital 1 week after the initial operation.\nOne month after the operation, the patient presented with a skin lesion at the side of the incision () with a postoperative angiography revealing a partial obstruction of the fibular artery (). Three months after the initial operation, the patient developed skin necrosis and infection at the site of surgical intervention. At that time, all retained hardware was surgically removed and the patient received oral antibiotic therapy for the following 3 months.\nSix months after the first operation, the patient was diagnosed with a septic non-union of the tibia. At that time, an extensive surgical osseous and soft tissue debridement was performed in addition to skeletal stabilization and Ilizarov external fixation. The skin defects were treated with the Papineau technique (). Intraoperative cultures were positive for Streptococcus mitis, Escherichia coli, and Stenotrophomonas maltophilia, and biopsies were positive for inflammation. Postoperatively, the patient was allowed immediate full weight-bearing and received oral antibiotics for an additional 3 months.\nSix months after the Ilizarov external fixation, there was radiographic evidence of fracture healing ( and ). The skin defect was healed () and the Ilizarov external fixation was removed. One year after the operation, the patient presented without any pain, had full range of motion at the ankle, and had returned to his daily activities. |
A 26-year-old male patient was referred to our dental clinic with limitation of mouth opening ever since childhood. His medical anamnesis was unremarkable. He had no musculoskeletal anomalies, congenital bone dysplasia or acromegaly. Clinical examination revealed a 15 mm measurement of mouth opening. In panoramic radiography, elongation of the coronoids could not be seen clearly. For a further diagnosis, digital images with CBCT were taken using an ILUMA CBCT scanner (Imtec Corporation, OK, USA) with an amorphous silicon flat-panel image detector and a cylindrical volume of reconstruction up to 19×24 cm2. Images were obtained at 120 kVp, 3.8 mA, and a voxel size of 0.2 mm, with an exposure time of 40 seconds. Axial scans and 3D reconstructions were created by reformatting the axial CBCT scans on a local workstation using the ILUMA dental imaging software in accordance with the manufacturer's instructions. Before imaging, written informed consent forms were obtained from the patients. Elongation of the mandibular coronoid processes were easily detected bilaterally in both 3D images ( and ) and axial scan (). For the measurements, the orientation of the 3D view was made based on the Frankfort plane (a line passing horizontally from the superior border of the external auditory meatus to the inferior border of the orbital rim). A second line passing from the top of the condyle that was parallel to the Frankfort plane was used for the length of the coronoid. Vertical measurement between the second line and the top of the coronoid process revealed the length of the elongated coronoid ( and ). The length of the coronoid was measured as 6.1 mm on the right and 5.4 mm on the left side. On the axial scan, the distance between the coronoid process and inner face of the frontal part of the zygomatic bone was 2.9 mm on the right and 2.7 mm on the left side. The patient was informed about the treatment plan and referred to a surgeon for operation. |
A 21-year-old woman was admitted to the emergency room in her second week of puerperium because of headache, seizures and decreased alertness. A computed tomography (CT) scan showed no remarkable alterations. The patient had no significant medical history. In the initial neurological evaluation, we found her to be poorly reactive and confused, with eye-opening to pain, symmetric and reactive pupils, brain stem reflexes present and localized pain with movement of the extremities as well as bilateral Babinski signs. A Glasgow Coma Scale (GCS) of 11 points was calculated.\nMagnetic resonance imaging (MRI) with a 3D time of flying (3D TOF) sequence showed thrombosis of the sagittal superior sinus with bifrontal venous infarcts (). Therapeutic anticoagulation with low-molecular-weight heparin was started. The patient remained stable during the following days, but neurological function deteriorated with the absence of pupillary reflexes, and a GCS of 7 was observed at 5 days. A new CT scan showed haemorrhagic transformation of the bilateral infarcts accompanied by severe oedema. Lesions on both sides were large and associated with midline shift greater than 5 mm (). Because of the clinical deterioration and length of the lesions, the patient underwent bifrontotemporal decompressive craniectomy ().\nAfter the procedure, she remained under sedation with mechanical ventilation support, and 3 weeks later was extubated. She maintained good clinical evolution and stable vital signs and was discharged 1 month after the procedure. Upon hospital discharge, she had quadriparesis and required support for all her activities of daily life. A modified Rankin score (mRs) of 5 points was calculated.\nGenetic tests for MTHFR mutations and other studies such as those evaluating antithrombin III, anticardiolipin antibodies, lupus anticoagulant and anti-beta 2 glycoprotein antibodies were performed, and the results were reported as normal.\nTreatment with dabigatran was maintained for the following months. By the 6-month follow-up, the patient was able to walk without assistance and attend to her basic needs, and an mRs of 3 was calculated. A new CT scan with venography revealed partial recanalization of the sagittal superior sinus (). |
A 37-year-old female patient presented to the emergency department with a complaint of central chest pain since 7 days, with the pain radiating to her left shoulder. She also had a history of hemoptysis since 8 days, at a frequency of 10–15 episodes per day. Also, she had one episode of vomiting 2 days prior to presentation with streaks of blood in the vomitus. There was no associated diaphoresis, palpitation, dyspnea, syncope, melena, or fever. She had a history of severe pulmonary valvular stenosis which was treated with balloon valvular dilatation during her childhood. She was detected to have VWF deficiency when she developed a hematoma during the post-operative period. She was not on any regular follow-up or treatment for long periods and did not have any significant bleeding manifestations which warranted medical attention. Her last menstrual period was 16 days back. Examination revealed a heart rate of 71 beats per minute, all peripheral pulses felt, with a blood pressure of 200/130 mmHg, respiratory rate of 18 breaths per minute, and blood oxygen saturation of 99% in room air. She was pale on general examination. Auscultation of the heart revealed an ejection murmur in the left 2nd parasternal area. Her respiratory, abdominal, and neurological examinations were within normal limits. Electrocardiogram (ECG) revealed ST-segment depression in leads I, aVL, II, III, aVF, and V4–V6 ().\nHer troponin I level was reported as 0.09ng/ml. Echocardiography revealed severe pulmonary stenosis and right ventricular hypertrophy with no regional wall motion abnormality and good left ventricular systolic function. The patient was transferred to the cardiac intensive care unit. VWF deficiency and presence of bleeding manifestations during the past 8 days was considered as a contraindication for starting the patient on unfractionated heparin and antiplatelet agents. Since echocardiography showed no regional wall motion abnormality and taking into consideration the previous history of complications during balloon valvular dilatation, PCI was withheld. She was started on nitroglycerine infusion, angiotensin II receptor blockers, and calcium channel blockers along with trimetazidine. Her chest pain and ECG changes settled after 2 days (), and she was discharged in a stable condition. |
A 30 year old male was referred to our department with a 4 years history of pain (pain scale VAS 80) and crepitus in the left TMJ during mastication, increased left facial pain, and limited functional mandibular movements.\nBruxism was reported by the patient for a period of about 18 months. He had natural molar contacts in each dental quadrant, and no parodontal disorders.\nIntraoral examination revealed a bilateral Class II molar relationship and a severe overjet [Figure ]. The lower dental midline deviated to the left of the upper by 4 mm. Moderate crowding was observed in both arches.\nClinical examination confirmed acute muscular pain, lateral deviation of the mandible to the left during opening and closing of the mouth, persistent pain and crepitus in left TMJ, limited opening (interincisal distance 20 mm), lateral movement to the right (3 mm), lateral movement to the left (7 mm), and difficulty protruding the mandible [Figure ]. Crepitus and pain were determined by palpation of both joints during maximal protrusion and maximum mouth opening.\nA panoramic radiograph of the patient's jaws prior to removal of the mandibular left third molar, revealed left CR [Figure ]. This type of radiographic examination does not offer as clear and reliable images as those of other techniques such as computerized or linear tomography, but does demonstrate the condyles with a degree of clarity [].\nRoutine haematological analysis did not reveal any evidence of underlying systemic bone disease such as rheumatoid arthritis.\nTreatment was anterior repositioning of the mandible with a hard acrylic splint in the maxilla.\nThe splint was 3 mm thick, and it was constructed with an inclined plane for mandibular advancement of 2,5 mm, re-centring the lower deviated dental midline [Figure ]. The splint surface was adjusted to obtain a balanced muscular activity, and checked with conventional clinical control of the dental contacts.\nThe splint was used consistently, though due to work commitments, not in the mornings. The patient was reviewed every month and showed progressive symptomatic improvement on each occasion. After 8 months, a new panoramic radiograph confirmed new bone formation on the condylar surface [Figure ]. Clinical features were improved, with reduced pain (pain scale VAS 20) and an increase in mouth opening (30 mm), although deviation of the mandible and crepitus were still evident during mastication [Figure ]. After 18 months there was complete resolution of the symptoms, with no pain, and similar morphology of both condyles [Figure , ]. At completion of treatment, there were no occlusal abnormalities. |
A 62-year-old woman was referred to the authors' hospital for evaluation of a corneal ulcer in her left eye. With the patient's consent, a review of the clinic record was conducted. Ten years previously she received an allogeneic hematopoetic stem cell transplant for treatment of myelodysplasia syndrome. Her course was complicated by the development of cGVHD affecting the liver, skin, esophagus, mouth, and eyes. Severe keratoconjunctivitis sicca had been treated with a PROSE lens for more than 2 years prior to developing this corneal ulcer. Her medical status was compromised by systemic steroid dependence and by steroid induced diabetes mellitus.\nAfter presenting with a corneal ulcer in her left eye, the patient was empirically treated with topical 0.5% moxifloxacin (Vigamox, Alcon) that was applied 6 times a day (once before the PROSE lens was inserted in the morning, 4 times during the day inside of the PROSE lens reservoir, and once again at night after the PROSE lens was removed). After failure to improve on 4 days of this treatment, she was referred to Massachusetts Eye and Ear Infirmary for additional corneal ulcer evaluation, culture and modification of treatment. Upon presentation, the central cornea was opaque and neovascularized with tissue loss of approximately 60% of the corneal thickness. There was an epithelial defect that measured 2.5 mm by 1 mm, with an underlying 2 mm by 1 mm infiltrate. Microbiological smears and cultures were performed with the specimen from corneal scraping. No bacteria or fungi were evident with Gram stain or Calcofluor white stain. At the time, frequency of application of topical moxifloxacin was increased to every 2 hours while awake (approximately 8 times per day), delivered as one drop added to the PROSE reservoir after removal and cleaning reinsertion of the device and replenishment of the reservoir with preservative free saline (B).\nFour days after culture and modification of antibiotic delivery regimen, the corneal ulcer resolved, with re-epithelialization of the cornea surface and resolution of the infiltrate. A strain of E. coli cultured from the lesion in 5% sheep blood agar media exhibited resistance to fluoroquinolones, trimethoprim/sulfamethoxazole and ampicillin and ampicillin-sulbactam according to breakpoints set by the Clinical Laboratory Standard Institute-CLSI (). The strain carried multiple single point mutations in the quinolone resistance-determining region (QRDR) of gyrA (Ser83Leu, Asp87Asn), parC (Ser57Thr, Ser80Ile) and parE (Leu416Phe) genes. These mutations were correlated with varying levels of resistance to the fluoroquinolones: ciprofloxacin (MIC 256 μg/mL), levofloxacin (8 μg/mL) and moxifloxacin (16 μg/mL), as determined by reference broth microdilution. |
A 43-year-old Pakistani man was admitted to Rigshospitalet, University Hospital of Copenhagen, in January 2013, with complex mechanical mitral valve- and native aortic valve endocarditis, complicated by aortic root abscess. Medical history included heart failure after rheumatic fever with mitral stenosis and mild aortic insufficiency, treated with mitral valve replacement and a minor commissurotomy of his fused aortic valve cusps in 2009. The patient was otherwise healthy and had no risk factors for coronary artery disease. Preoperative coronary angiography was normal (Figure and B). The patient was emergently operated with implantation of a biological mitral valve (St. Jude Medical, Epic, size 27 mm) and a full root FB (size 23 mm) in the aortic position. A FB was chosen since the tissue quality and irregularity of the revised root including the presence of a rigid biological mitral valve prosthesis did not allow for implantation of the rigid suture-ring of a stented (mechanical or biological) aortic valve prosthesis. Furthermore, a homograft was not available. Reimplantation of the coronary ostia was performed in a standard fashion with the button technique. The angle of the porcine coronary ostia is 90° relative to the centre of the aortic root, whereas the human coronary ostia assume an angle of 120°. The FB was therefore oriented so that the left coronary artery was reimplanted in the left porcine ostium, while the right coronary artery was reimplanted higher and further to the right in the right sinus of the FB, to avoid kinking or stretching of the coronary (. The patient was discharged after 6 weeks of targeted intravenous antibiotic therapy, with atrial fibrillation, and newly diagnosed diabetes mellitus type 2. He was prescribed warfarin (target international normalized ratio 2–3), enalapril 2.5 mg, metformin 1000 mg, and metoprolol 50 mg. Follow-up transoesophageal echocardiography 41 days postoperatively showed a well-functioning FB valve in the aortic position.\nIn June 2017, the patient presented with chest oppression, pain in the left arm and hand, as well as shortness of breath. Physical examination revealed a discrete heart murmur along with an irregular rhythm. Lung auscultation presented normal vesicular breath sounds and extremities were without oedema. Except from the chest oppression, the general condition was good and further physical examination normal. Electrocardiogram showed atrial fibrillation and new ST depressions and T-wave inversions in I, II, V5, V6, and ST elevation in aVR (Figure ). Troponin T samples showed increasing concentration reaching a maximum of 196 ng/L (normal range < 14 ng/L). A contrast-enhanced cardiac computed tomography (CT) showed significant left- and right coronary ostial stenosis but no sign of atherosclerosis (Figure ). Preoperative coronary angiography confirmed this finding, showing 90% stenosis in both ostia and otherwise normal coronary arteries (Figure and D, Videos 1 and 2). Preoperative transthoracic echocardiogram showed left ventricular ejection fraction of 50%, and excellent prosthetic aortic- and mitral valve function. Due to the risk of non-dilatable ostial tissue and stents protruding into the aortic lumen, the case was not found suitable for percutaneous coronary intervention (PCI). The patient underwent emergent reoperation with the aim to either replace the FB or to perform revascularization. Intraoperatively, after partial opening of the distal anastomosis between the FB and the ascending aorta, we found pseudointimal glass-like membranes, which covered the distal anastomosis between the FB and the ascending aorta as well as both coronary ostia, leaving the latter with high-grade stenoses (Figure ). The membranal tissue was brittle, not invading the FB and could thus be peeled of the FB tissue in strips. Since the pseudointimal membranes extended from the anastomotic sites and into both coronary arteries, and since surgical detachment of the membrane tissue could only be done radically in the proximal parts of the vessels, we considered replacement of the FB and subsequent reimplantation to carry a considerable risk of dissection between the pseudointimal tissue and native tissue, and subsequent coronary occlusion by the remaining membrane tissue. Likewise, PCI could potentially also dislodge the membrane and cause occlusion. Therefore, after collecting a tissue sample of pseudointimal membrane, the patient had coronary artery bypass grafting (CABG) performed with separate venous grafts to the left anterior descending artery and the right coronary artery (RCA). The patient recovered uneventfully, aside from a smaller procedure due to epigastric fascial rupture.\nAcetylsalicylic acid 75 mg was added to previous medication and metoprolol was reduced to 25 mg. Histological examination of the explanted pseudointimal membranes showed fibro-intimal thickening with areas of inflamed granulation tissue. There was no sign of acute inflammation, calcifications, foreign bodies, or amyloidosis.\nIn September 2017, a follow-up cardiac CT and transthoracic echocardiography, performed due to complaints of chest pains, showed a small (2.3 mL) pseudoaneurysm arising from partial rupture of the last opened (distal) anastomosis between the FB and the aorta (Figure ). Due to the patient’s numerous previous reoperations, and the small size of the pseudoaneurysm, we chose a conservative, non-surgical strategy. Repeat follow-up cardiac CT, performed 3 months later, showed reduction of the pseudoaneurysm to 1.4 mL. and video 3\nDuring follow-up in November 2019, the patient still suffered from exercise-induced chest pains. Myocardial perfusion scintigraphy from 2018 showed exercise-induced myocardial ischaemia of 6–8%, probably due to previous myocardial infarction. Coronary angiography from 2018 confirmed complete revascularization. Transthoracic echo showed mild-moderate stenosis of the FB valve and well-functioning biological mitral valve. |
A 39-year-old woman, gravida 1, para 0, was diagnosed with invasive squamous cell carcinoma of the cervix following conization. Pathological findings showed carcinoma consistent with FIGO stage IA1 with lymphovascular invasion. She was referred to Keio University Hospital. The patient and her husband were informed of the treatment options, including AmRT and pelvic lymphadenectomy. The patient was told that the outcome of this procedure could not be guaranteed because an insufficient number of these procedures have been performed worldwide to yield reliable conclusions. The patient wished to preserve fertility, and she and her husband signed a written consent form agreeing to this treatment. Pathological findings after AmRT and pelvic lymphadenectomy showed no residual tumor and no lymph node metastasis. There was no finding of an ovarian tumor before surgery. A left ovarian cyst of 4 cm was identified during postoperative follow-up.\nAt 3 years and 6 months after surgery, the patient underwent IUI and then had fever and pain in her left lower abdomen 10 days later. At her first visit, her temperature was mildly elevated to 37.5°C. The patient's pregnancy was denied because a qualitative urine human chorionic gonadotropin (hCG) test was negative. A tumor with tenderness was palpated in the left adnexal area. A cystic tumor of 64x 41 mm was found by transvaginal ultrasonography (). Blood tests showed increases in white blood cell (WBC) count to 11900/μL and C-reactive protein (CRP) to 22.80 mg/dL. The patient was diagnosed with PID with ovarian cyst infection and hospitalized for treatment. Conservative treatment with antibiotics was initially used, but her symptoms did not improve. On hospital day 8, blood tests showed a further increase in WBC count to 23900/μL and CRP to 28.17 mg/dL, and pelvic CT showed that the ovarian cyst had grown to 10 cm in size ().\nWe decided to perform laparoscopic left ovarian cystectomy on day 8. Since the patient had a history of open surgery, adhesion was likely in the abdominal cavity. Before surgery, we asked the urologist to insert bilateral 6 Fr ureteral catheters because of possible difficulty identifying the ureters. The catheters were fixed to the thigh with tape (). The uterine manipulator was not inserted before surgery to avoid the risk of uterine perforation.\nCO2 pneumoperitoneum was established at 10 mmHg. Laparoscopic left ovarian cystectomy was performed using typical trocar placement. The left ovary was swollen to 10 cm and the fluid contents were purulent (). The left adnexa, posterior uterine wall, and retroperitoneum were firmly adhered (). The bilateral fallopian tubes were firmly adhered to the surrounding tissue and were unable to be identified. During surgery, by moving the catheter manually back and forth from outside the body, we were able to identify the ureters visually (). The 7 cm uterine manipulator was inserted during surgery under a laparoscopic view. The left ovarian cyst was excised, leaving the normal part of the ovary (). The operation time was 2 h and 58 min, and blood loss was 550 mL. No complications occurred during or after surgery. After the operation, symptoms improved rapidly and the patient was discharged 8 days after surgery. The excised specimen was pathologically an endometriotic cyst. A bacterial culture of the cyst fluid was positive for Prevotella bivia, Prevotella species, and Finegoldia magna. |
A 22-year-old man presented to our department for correction of refractive errors. The ophthalmological examination revealed uncorrected visual acuity of 0.25 and 0.3 in the right and left eyes, respectively. The central corneal thickness of the right eye was 500 µm, and that of the left right was 502 µm. Treatment with Zeimer Femto LDV-assisted laser in situ keratomileusis (LASIK; Zeimer, Port, Switzerland) was proposed. The size of the ring of negative pressure was set at 9.0 mm. The pre-set depth of the valve was 110 µm. The surgery in the right eye was successful. In the left eye, however, a small, eccentric flap with a diameter of approximately 6.5 mm was noted in proximity to the nasal side (). The surgery was therefore aborted, and redo surgery was proposed after an interval of 3 months, at which time the corneal profile would have stabilized. The patient, however, insisted on undergoing redo surgery immediately as he wished to participate in a civil servant physical examination the next day. The patient was informed about the potential risks, and he gave written informed consent.\nThe second flap was made using the IntraLase technique. Prior to the redo surgery, anterior optical coherence tomography was performed, which revealed that the depth of the first flap was 119 µm (). The corneal depth for the present surgery was then pre-set at 140 µm, and the diameter of the flap was 8.6 mm (). Following the redo surgery, the patient was required to wear a bandage contact lens. The uncorrected visual acuity for both eyes was 0.5 at 2 h postoperatively. The corneal flap location was satisfactory, with a slit lamp revealing only slight edema (). The margins of the flap had healed completely at 2 days postoperatively (). The uncorrected visual acuity for each eye was 1.2 at 5 days after surgery, and the profile of the cornea was more regular than previously (). The patient was followed up for more than 12 months, with a satisfactory prognosis regarding vision and refraction. |
A 61-year-old Caucasian male army veteran had been treated as an outpatient for the last 2 years for post-traumatic stress disorder (PTSD), alcohol dependence, and depression not otherwise specified (NOS). Before this, he had been symptomatic, but did not present for treatment. He was admitted to a residential PTSD/alcohol dual diagnosis treatment program. His medications upon admission were fluoxetine 20 mg every morning for depression, nortriptyline 25 mg at bedtime for sleep and depression, quetiapine 50 mg at bedtime and every 6 hours as needed for anxiety, prazosin 1 mg at bedtime for nightmares, pramipexole 0.5 mg at bedtime for restless legs syndrome, terazosin 5 mg at bedtime for benign prostatic hypertrophy, atenolol 50 mg daily, and spironolactone 50 mg daily, for hypertension. He had been on all these medications for at least 6 months, with no reported adverse effects. He had smoked 2 packs of cigarettes per day for the past year, and had previously smoked four packs per day, for about 30 years. The patient's diagnosis of restless legs syndrome was made before any treatment he received for mental illness, and was not thought to be a medication adverse effect. His laboratory studies were negative and he did not have anemia (his hemoglobin level was 13.5 just before his enrolment in the treatment program).\nThe patient ceased using alcohol 1 month before entering the treatment program. He denied using any alcohol throughout the duration of the residential program, and no such evidence was found to the contrary by his health care providers. Three weeks before completion of the program, he was prescribed varenicline for smoking cessation, at the manufacturer recommended titration. This was prescribed by a provider at the treatment program. Subsequently, the patient reduced his smoking to two cigarettes per day.\nUpon starting varenicline, he reported experiencing visual hallucinations. This was unusual to him as he reports that he had never experienced visual or other hallucinations in the past. On increasing the medication to 1 mg twice daily, his visual hallucinations became more frequent and more vivid. He reported seeing ropes dangling in the air and birds flying around the room. Nineteen days after the commencement of varenicline, he was discharged to home. Initially, he had not reported his symptoms to his providers or family, fearing that they would think he was "crazy". On the trip home, his wife noticed his behavior had changed. She reported that on the drive home he was leaning over as though avoiding something and it appeared to his wife that he was "seeing things". His wife reported that he was also reaching out in the air as if to grasp something.\nUpon arrival at home, he read through the drug information provided by the pharmacy and noted that hallucinations could be a side effect. He immediately discontinued varenicline, without consulting his physician, and the hallucinations reduced, and resolved over a period of 3 days. He reported these symptoms to his regular mental health physician provider at his next office visit. According to DSM-IV-TR criteria, a diagnosis of Psychotic Disorder Not Otherwise Specified would be appropriate []. No further investigations were performed at this time as he was asymptomatic.\nOn applying the Naranjo causality scale, a score of 6 was obtained, indicating a probable adverse drug reaction to varenicline []. It is important to note that we took into consideration that this patient was on pramipexole, a dopamine agonist, which could have contributed to his symptoms. However, the patient had been using this medication for many months and had not reported signs or symptoms of psychosis. |
This study was conducted under approval of the Ethics Committee of First Hospital of Jilin University, and the protocol was accorded with its standards.\nThis patient was a 50-year-old woman admitted to our department due to a posterior mediastinal mass detected by computed tomography (CT) scan 7 days ago. The symptom of oppression in chest did not improve after anti-inflammatory treatment. At admission, her blood pressure, pulse rate, respiratory rate and body temperature were all in the normal range. She had no cough and expectoration, no fever, no significant chest and back pain, and no muscle weakness. She had no hypertension, heart disease, diabetes, any infectious disease or drug allergy history. At the age of 15 years old, she was diagnosed of multiple neurofibroma. At the age of 48 years old, she received the fibormectomy at a local hospital because of worsened symptoms of the left lower extremity. At day 1 after admission, the chest-enhanced CT revealed, a round soft tissue density sized 8.4 × 4.0 cm with unclear boundary with esophagus and heterogeneous enhancement in posteriormediastium, and multiple nodule-like soft tissue density shadows in subcutaneous chest and back (Fig. ). Meglumine diatrizoate angiography of upper gastrointestinal tract revealed abnormal changes in the lower esophagus. Magnetic resonance imaging (MRI) revealed thoracic bone hyperplasia and disc deformation. Laboratory examination showed that the plasma level and urinary excretion of epinephrine, norepinephrine and dopamine were all in the normal range. The liver function and kidney function were normal.\nBefore operation, the patient was treated with intravenous fluid resuscitation for a week and prepared for a surgery. A single-stage thoracoscopic mediastinal mass resection was performed. The patient was placed in the right supine position and anesthetized by double lumen tracheal intubation. A about 1.5 cm-long incision in the 9th intercostal axillary midline was made and thoracic exploration was performed by thoracoscope first. Then, a standard 24 cm-long lateral incision in the 7th intercostal space on the left was made. After each layer was cut, switch to single lung ventilation. No effusion or adhesions in the thoracic cavity and no space occupying lesion in pulmonary lobe were observed. The lower mediastinal pleural bulged and a mass adhered to surrounding tissue could be felt behind the esophagus and in front of the aorta. No obvious enlarged lymph nodes were found in the mediastinum. Mediastinal mass was diagnosed intraoperatively. Then, the posterior mediastinal pleura was open. After fully dissociating mass with surrounding tissues, the mass was completely resected. Rapid intraoperative pathology reported posterior mediastinal tumor. The tumor was measured about 11 × 7 × 5 cm in size with abundant blood supply and incomplete capsule (Fig. a). The tumor location was marked by titanium clip and a drainage tube was placed and fixed in the 9th intercostal space of the left axillary midline. Finally, suture the chest wall layer by layer. A total of 1000 mL fluid without transfusion was given. The whole operation went smoothly and the patient was back to ward safely.\nPostoperative histopathological examination showed the tumor was MPNST and measured 9.0 × 7.0 × 4.8 cm in size (Fig. b). Hematoxylin–eosin (H&E) staining showed obvious tumor cell atypia and irregular nuclei > 10/10 HPF (High power field) (Fig. ). Immunohistochemistry revealed CD34(−), Desmin(−), Ki-67(+ 30%), SMA( +), Vimentin( +), S-100( +), CD99( +), Bcl-2(−), CD117(−), EMA( +), and Calretinin( +) (Fig. ). Moreover, histopathological examination showed the mass in the body surface was benign neurofibroma and immunohistochemistry revealed Ki-67(+ 1%) and S-100( +). Three months later, the CT scan of lungs did not show relapse of tumor (Fig. ). |
A 62-year-old woman presented with a one-month history of sudden painless visual loss in the right eye. On examination, best corrected visual acuity (BCVA) was 20/20 in both eyes. Intraocular pressure was 21 mmHg in both eyes. Dilated funduscopic examination in the right eye revealed retinal emboli inferior to the optic disc obstructing a small arteriole associated with retinal ischemia (Figures and ). The left eye was unremarkable. There was no prior past ocular history. The patient had hypertension and hypercholesterolemia and had an extensive smoking history.\nFluorescein angiography revealed delayed retinal perfusion along the inferior arcade in the right eye (Figures and ). Optical coherence tomography showed normal foveal contour with inner retinal ischemia and thickening, consistent with an acute inferotemporal branch retinal artery occlusion (). The patient was started on Latanoprost at nighttime in the right eye to lower the intraocular pressure in hopes to increase reperfusion of the retina. An extensive cardiovascular workup was done, and significant carotid artery stenosis of less than 70% was found. She was started on plavix and aspirin by her cardiologist. The patient continued with BCVA 20/20 OU vision and persistent highly refractile peripapillary emboli; retinal ischemia resolved. Her OCT showed inner retinal atrophy (Figures and ).\nUnexpectedly, the patient presented with a three-day history of sudden painless visual loss OD a year and a half after her initial presentation. BCVA was counting fingers (CF) in the right eye. Fundus exam revealed new superotemporal retinal ischemia associated with two new emboli. OCT demonstrated thickening and hyperreflectivity of the inner retinal layers consistent with an acute BRAO OD (). The patient refused fluorescein angiography at that time. Urgent workup revealed worsening stenosis to 80% in her right common carotid artery. The vascular surgery referral prompted a right carotid endarterectomy with a carotid stent placement approximately two months following her presentation with acute vision loss.\nAfter endarterectomy, vision in the right eye improved from counting fingers to 20/200 and 20/250 at 2 months and 6 months, respectively. Postoperatively, retinal whitening resolved and reduced intraretinal edema was noticed (). One year after endarterectomy, visual acuity was 20/30. The retinal swelling had resolved (). |
A 66-year-old male underwent colostomy for large bowel obstruction due to advanced rectal cancer and continued chemotherapy. The patient was referred to our hospital for Fournier gangrene due to the penetration of the advanced rectal cancer during chemotherapy. The perineum and buttocks around the anus exhibited swelling and skin necrosis with severe pain and a septic odor. Laboratory testing showed leukocytosis (11,500/μl) and elevated C-reactive protein (38.94 mg/dl). Abdominal computed tomography revealed a necrotizing soft tissue infection with large amounts of gas throughout the perineum (Fig. ).\nEmergency surgical debridement was performed on the areas of the perineum and buttocks surrounding the rectal cancer (Fig. ). The infected wound around the rectal cancer was washed, and intravenous antibiotics were administered; however, the tumor was exposed by the wound, and exudate persisted. Tumor resection was considered necessary to treat the infectious wound, and radical surgery was performed on day 15 after debridement surgery. General anesthesia was induced, and a skin incision was made in the lower abdomen. Pelvic exenteration was performed due to the infiltration of the tumor into the bladder and prostate. Tumor resection resulted in a large defect in the pelvic floor, increasing the likelihood of internal organs herniating into the infectious wound. A colostomy was constructed through the left rectus abdominis muscle, and an ileal conduit was constructed through the right rectus abdominis muscle. The omentum was adhered and atrophied, due to previous surgery, and was not sufficient to fill the large pelvic defect.\nA 15-cm incision was made and a fascia lata free flap (15 × 9 cm) was created from the left femur (Fig. a, b). The ventral, lateral, and back sides of the flap were fixed to the edge of the peritoneum along the resected bladder, bilateral external iliac arteries, and ileal conduit, respectively, (Fig. c). After the rectum was resected, the pelvic space was left and the perineal wound was not sutured. However, the perineal wound was separated from the abdominal cavity.\nHistological examination revealed moderately differentiated tubular adenocarcinoma of the rectum with direct invasion of the prostate and vas deferens. Severe lymphatic and vascular invasion were present, and four metastases of the regional lymph nodes were identified. A swollen right inguinal lymph node was dissected and diagnosed as a metastasis from rectal cancer. The tumor was diagnosed as stage IV, pT4b (prostate and vas deferens) N2 M1 (inguinal lymph node metastasis) according to the Japanese Classification of Colorectal Carcinoma. The tumor was resected with negative proximal and distal margins; however, this case had a high risk of local recurrence, and so, postoperative chemotherapy and chemoradiotherapy were planned.\nThe patient ceased fasting on postoperative day 2. There was no postoperative intraabdominal inflammation or bowel obstruction. The perineal wound was managed as an open wound. The wound exudate became clear, and the wound was considered to be healed on postoperative day 35 (Fig. c). Postoperative computed tomography showed maintenance of the fascia lata free flap. There was no weakness in the left leg, and the patient maintained the same walking ability as preoperatively. Outpatient chemotherapy was continued after the patient was discharged from hospital. Radiotherapy of the pelvic area was performed postoperatively. Irradiation of the pelvic space was performed, while minimizing irradiation of the small bowel (Fig. ). The pelvic space had disappeared by 8 months postoperatively (Fig. d). Positron emission tomography performed 1.5 years postoperatively showed no accumulation of 18F-fluoro-deoxy-glucose in the pelvic area. The patient has had no further complications in 2 years of follow-up. |
A 40-year-old male presented with a 2-day history of epigastric pain. He once had a 2-year history of epigastric pain after fatty meals, and a family history of positive chronic hepatitis B virus for 20 years (the patient's father and brothers were both died of liver disease). On examination, all observations were within normal limits. His abdomen was mildly tender in the epigastrium without any palpable masses or evidence of peritonitis. Laboratory data showed elevated leukocytes 12.4 × 109/L (normal, 4.0–10.0 × 109/L), Hepatitis B antigen positive (+). An ultrasound scan revealed a distended and thickened gallbladder containing gallstones. Computed tomography of the abdomen was performed, which showed the presence of gall stones, acute cholecystitis, and liver cirrhosis. After preparation, he was taken to the operation theater and underwent laparoscopic cholecystectomy, which was technically successfully. The gallbladder was removed intact from the gallbladder fossa and placed in a specimen retrieval hag for extraction without complication. At the time of surgery, there was a small amount of yellow effusion accumulated under the right side of the diaphragm. A silastic close-suction drain tube was placed in the right subhepatic space at the completion of the operation (Fig. and Fig. ). The patient tolerated the procedure well and resumed oral intake later on the day of surgery. Antiinfective and other supportive treatment were given after the operation. Postoperative pathology: acute cholecystitis with cholelithiasis.\nThe effluent from the drainage was noted milky and odorless on the second day after surgery. The initial output of the effluent was about 500 mL per day, but increased day by day, to the seventh day postoperative, it began significantly up to and maintained 8000 to 9000 mL per day. The patient had abdominal discomfort, bloating, and nausea, but had no fever. A CT scan was performed, revealing significant ascites. The effluent's laboratory analysis revealed elevated levels of triglycerides (1245 mg/dL), protein (28.5 g/L), and leukocytes (685 × 106/L), consistent with chyloperitoneum. Conservative management was provided initially on the third day post-LC. The patient was given a total parenteral nulrition (TPN) in combination with medium-chain triglyceride supplementation, and with no oral intake. Simultaneously, liquid recovery, electrolyte and albumin replenishment, antibiotic prophylaxis, plasma transfusion, diuretic, somatostatin continuously intravenously infusion, and other effective treatment were also afforded to maintain the patient's liquid, electrolyte, and acid-base balance. Frustratingly, after a week of conservative therapy, output from the patient's abdominal drain did not diminish. So we decided to give surgical intervention for this refractory case without any delay. The patient was taken to the operation room again for laparoscopic reexploration at the tenth day postoperative. At the time of reexploration, an active chyle leak was identified from the base of the gallbladder fossa, emanating directly from the parenchyma of the liver bed, but no evidence of injury to bowel, lymphatic vessels or structures within the portahepatis was identified. Then we turned to open the abdominal cavity, 3–0 Prolene suture was placed in figure-of-eight fashion encompassing the area of leakage, fibrin glue was then placed as well after stitching, with resolution of the leak. We carefully observed to confirm no obvious leakage after the reoperation (Fig. and Fig. ). The patient was returned to ICU ward after the second surgery, and was still given continuous conservative therapy. The next day after the reoperation, the drainage output diminished from about 9000 mL per day down to about 5000 mL per day. After a long period of conservative treatment (lasting almost 7 months), the drainage gradually decreased to zero. Abdominal sonography on the eighth postoperative month revealed no fluid accumulation in abdominal cavity, especially in the right subhepatic space, and then the drain was removed. The patient was discharged home without further difficulty during the 1-year follow-up. |
A 47-year-old male with a past medical history notable for hypertension on metoprolol succinate, morbid obesity, and pre-diabetes presented to the emergency department (ED) with a chief complaint of generalized weakness. The patient tested positive for COVID-19 and exhibited mild unspecified respiratory symptoms. He was subsequently discharged home to recover in isolation per the CDCs COVID-19 response protocols. One week later, the patient returned to the ED for ongoing symptoms and was admitted requiring supplemental oxygen for hypoxia. Upon admission to the hospital, the patient was noted to have difficulty standing and ambulating. Two days into his inpatient stay, the patient developed urinary retention issues requiring intermittent catheterization. Three days later, he developed facial weakness and numbness. The clinical diagnosis of GBS was suspected and subsequent EDX studies reported AIDP. The patient was treated with a five-day course of intravenous immune globulin (IVIG). Three days after the completion of his IVIG treatment, the patient noted improvement with right upper extremity anti-gravity strength.\nThe patient was transferred to inpatient rehabilitation with significant proximal lower extremity weakness. The bilateral upper extremities demonstrated slight weakness. The bilateral lower extremities demonstrated a significant loss of strength, 1/5 dorsiflexion, and 3/5 plantar flexion bilaterally. The patient also reported diminished sensation to light touch in bilateral upper extremities in all dermatomes. His blood pressure upon rehabilitation admission was 110/73 mmHg.\nPrior to hospitalization, the patient was independent with mobility and all activities of daily living. Upon evaluation in the inpatient rehabilitation, the patient was at a significant functional decline from baseline, requiring dependent assistance with toileting hygiene, showering, upper body dressing, lower body dressing, footwear management, rolling left and right, and all transfers. The patient was unable to ambulate due to his level of impairment. In inpatient rehabilitation, the patient completed three hours total of physical, occupational, and speech therapy per day five days a week with exercises aimed to improve balance, mobility, activities of daily living, fine motor skills, cognition, and breath support.\nAfter completing four weeks of inpatient therapy, the patient was independent with supine to sit, upper and lower body dressings, and rolling right and left in bed. He required minimal assistance to lift the left lower extremity into the bed and moderate assistance with wheelchair-to-bed transfers and bed mobility. He was able to stand with the assistance of his spouse and by using significant reliance on bilateral upper extremity support on a walker for stability and offloading lower extremities. He required a power wheelchair for mobility and was unable to transfer in and out of the car thereby requiring a wheelchair van for transportation. Final muscle strength grading was not documented. His blood pressure remained stable throughout his stay on metoprolol succinate and was 121/65 mmHg on discharge. |
A 16-year-old male complained of inability to flex his left elbow since 1 year prior to admission. One and a half year before, he fell down and hit his elbow during football practice. He felt pain and there was swelling on his elbow. However, he didn't seek for medical treatment. He had his elbow massaged every week for 5 months but there was no improvement. His elbow became fixed in extended position. A month later, he went to an orthopaedic surgeon and underwent x-ray examination which revealed a fracture and dislocation on his left elbow. He was then referred to our institution for further treatment.\nFrom clinical examination, range of flexion-extension of the elbow was 300-00 with normal pronation-supination. There was no neurological deficit (). From radiological examination, there was a malunion of medial epicondyle with subluxation of left proximal ulna (). From 3D CT reconstruction, there was a deformity and malunion fracture in humeral capitellum with radial and ulnar postero-superior dislocation (). The patient was diagnosed with extension contracture of the left elbow due to malunion of left capitellum, neglected dislocation of the radiohumeral joint, and neglected dislocation of the ulnohumeral joint. The patient was scheduled to have a contracture release, open reduction and internal fixation, and ulnar interposition.\nIntraoperatively, we did a posterior approach to the elbow. The ulnar nerve was identified and preserved. The fibrotic tissues and heterotopic ossification were excised. We did a contracture release and open reduction and internal fixation using K-Wire. The flexion and extension of the elbow were evaluated and we managed to get 300 - 130° of flexion-extension ROM. Afterwards, ulnar interposition was performed to prevent ulnar impingement. The wound was closed and a single drain was placed. The elbow was immobilized with back-slab in 900 flexion position for two weeks.\nAfter 1 week, the patient went back to our hospital for follow-up examination. In the 1st evaluation, we tried to remove the back slab and moved the elbow passively. The movement is restricted due to pain and the patient went back home with the back slab on. In the 2nd week follow-up, we permanently remove the back slab and the stitches. At that time, the pain still persisted and the patient was planned to have physical rehabilitation.\nOn the 4th week after surgery, the surgical wound was infected. We performed debridement, implant removal, and manipulation under general anesthesia. Two weeks later, patient came back to our hospital. We removed the stitches and started rehabilitation. Later on, he continued his rehabilitation in his previous hospital.\nAfter 6 months, he visited our outpatient clinic for medical checkup. From physical examination, the elbow flexion-extension ROM was 1100 - 300 (). The patient is able to do normal daily activities (). |
A 42-year-old Caucasian Finnish woman was scheduled for laparoscopic cholecystectomy due to typical symptoms and radiological findings of gallstones. She was obese (89 kg, BMI = 33), even though she had managed to lose weight by 20 kg six months prior to presentation. She was using sibutramine and oral contraceptives as regular medication.\nSurgery was uneventful, but on the second postoperative day, the general state of our patient started to deteriorate, resulting in anuria and difficulty of breathing, admission into the intensive care unit (ICU), endotracheal intubation, and mechanical ventilation. Endoscopic retrograde cholangiopancreatography (ERCP) was performed upon ICU admission because of suspected biliary tract leakage. However, no signs of leakage were observed. Computed tomography (CT) examination revealed fluid around her liver, while her pancreas could not be visualized. Her plasma amylase concentration was elevated, thus confirming the diagnosis of pancreatitis.\nDue to decreased renal function, she was commenced on continuous hemodiafiltration therapy on the third day and continued until the 10th postoperative day. Propofol infusion for sedation, supplemented with intravenous oxycodone boluses, was started as part of our hospital's standard therapy in order to facilitate mechanical ventilation and other treatment procedures. Propofol sedation was continued until the 36th postoperative day. Upon the decision of weaning, her attending physician decided to add dexmedetomidine infusion into the sedation regimen 17 days after her surgery.\nWeaning was not successful, and tracheostomy was performed on the 18th postoperative day. On the 19th postoperative day, esophagogastroduodenoscopy and explorative laparotomy were performed because the general condition of our patient again started to deteriorate. Hemorrhagic pancreatitis with severe inflammation of her abdominal cavity was discovered. This deterioration prevented further weaning. Our aim was to stop propofol infusion after starting her on dexmedetomidine, but in order to achieve the desired level of sedation (light to moderate, Sedation-Agitation Scale (SAS) levels 3 to 4 []), propofol infusion had to be continued despite the already high dose of dexmedetomidine she was receiving.\nOur patient later required yet another laparotomy because of elevated abdominal pressure. This surgery did not reveal any additional findings, but this time her abdominal wall had to be left open, and sedation had to be continued. Vacuum-assisted closure therapy was established on the 22nd and continued until the 34th postoperative day. Weaning was started again on the 36th postoperative day. Clonidine infusion was started on the 40th postoperative day, while the dexmedetomidine infusion was discontinued on the following day, on the 24th day of its administration.\nOur patient's abdominal wall was finally closed on the 47th day, and the clonidine infusion was stopped on the 51st postoperative day. The tracheostomy cannula was removed on the 54th day and she was admitted to a surgical ward on the 55th postoperative day for further recovery.\nDuring dexmedetomidine sedation, her plasma albumin level was low (8.4 g/L to 11.6 g/L) and her creatinine level was slightly elevated (23 μmol/L to 126 μmol/L). Meanwhile, her bilirubin level and international normalized ratio (INR) were both normal at 5 μmol/L to 16 μmol/L and 1.1 to 1.4, respectively.\nAt 14 months later, her recovery is still incomplete. Already in the ICU she complained that her vision is impaired. According to the consulting ophthalmologist, this symptom is likely due to ischemic optic neuropathy. At present she can only see movement and light with her right eye. The vision of her left eye is also severely impaired, but she is able to read using special equipment for the visually impaired. Additionally, her everyday life is harmed by numbness and weakness of her extremities, which is caused by critical illness polyneuropathy. Despite these impairments, her aim is to return to work.\nSamples for the measurement of dexmedetomidine concentrations in her plasma were drawn at 8-hour intervals as directed by the plan of the pharmacokinetic study she was recruited in. The concentrations were determined using reversed-phase high-performance liquid chromatography with tandem mass spectrometric detection (PE Sciex API4000 instrument; PE Sciex, Foster City, California, US) as described previously [].\nThe rates of the dexmedetomidine and propofol infusions, as well her plasma concentration results of dexmedetomidine, are presented in Figure . In calculating the dexmedetomidine dose, we used her preoperative weight of 89 kg.\nBecause the dose rate of dexmedetomidine remained constant for relatively long periods of time during three separate intervals, we calculated the plasma clearance of dexmedetomidine during these intervals by dividing the infusion rate by the plasma concentration. The calculated clearance was 55 L/h, 92 L/h and 87 L/h during the 2nd to 6th, 14th to 20th and 21st to 23rd day of the dexmedetomidine infusion, respectively. A list of drugs administered during her ICU stay is presented in Table . |
A 55-year-old woman presented three days after a sudden onset of right-sided chest pain, pleuritic and positional in nature, associated with an acute onset of shortness of breath. She had gone to her primary care physician, who performed a chest X-ray and urged her to come to the hospital. Upon presentation at the emergency department, her oxygen saturation was above 95% on room air, and she was not in any respiratory distress, but her exam was significant for decreased breath sound on the right. A chest X-ray confirmed a large right-sided pneumothorax with small pleural effusion. A chest tube was inserted on the right side for the resolution of the pneumothorax, and subsequent computed tomography (CT) scan of the chest revealed bilateral diffuse bullous disease of the lung with multiple cysts (Figure -). The patient underwent video-assisted thoracoscopic surgery for right thoracoscopic wedge resection of a lung bleb and talc pleurodesis. Gross examination of the specimen revealed several dilated air-like spaces ranging from 0.2 cm to 0.4 cm in size. The hospital course was complicated by postsurgical pneumonia, but she recovered fully and was discharged to home with only minimal symptoms of dyspnea on exertion. Upon further investigations, she was found to have multiple small lesions of angiomyolipoma on the right kidney with diffuse retroperitoneal lymphadenopathy. One of the lymph nodes was biopsied, and pathology revealed predominantly spindle cells positive for HHF35 and smooth muscle actin, consistent with the diagnosis of leiomyoma. At the eight-month follow-up at the pulmonology clinic, her pulmonary function test (PFT) showed normal vital capacity and forced expiratory volume in one second (FEV1), but moderately reduced diffusion capacity, which may also be related to LAM. At her 12-month and 24-month follow-up visits, her PFT results showed improvements in peak flow and diffusion capacity, and the patient continues to report no symptoms other than minimal dyspnea on exertion. |
The Patient, a 60-years old Caucasian male found unconscious in a trailer park of gypsies, was carried to Emergency Department at night, intubated and haemodynamically stable. At physical examination he presented a head gunshot wound, with an only visible bullet-hole located at the back of the neck. No other lesions were found. The total body contrast-enhanced CT scan performed at the Emergency Department revealed a large subdural haematoma, a retained bullet in the brain (Fig. ) and another retained projectile in the left lung (Fig. ), without any evidence of thoracic wall wounds; there was no air outside the bowel (Fig. ) even if little air bubbles could be recognized near to the pubis (Fig. ). These findings were difficult to be interpreted, both by the radiologist, the surgeon and the anaesthesiologist, as patient’s examination performed in the shock room after the CT scan confirmed the only presence of a single bullet-hole located at the back of the neck. As the Patient had no clinical and radiological signs of bowel perforation, it was decided for a “wait-and-see” attitude, scheduling a second CT scan for the following day.\nAbout 14 h later the second CT scan showed the presence of hematoma near the pubis, peritoneal air bubbles outside the bowel (Fig. , ) and diffuse peritoneal free fluid (Fig. ). These findings were suspected for bowel perforation, thus the patient was translated into operative room: by laparotomy, the haemoperitoneum was drained and multiple perforations of small bowel were identified, associated with a single perforation of the upper part of the rectum, a double perforation of the stomach and a small hole in the left diaphragm, throughout which the bullet had reached the left lung. Multiple sutures of the rectum, the bowel, the stomach and the diaphragm and a small bowel resection were performed; the bulled retained in the lung was not reached nor removed. The only possible explanation for the trajectory of the bullet was that it was shot through the anus; evidently, after the blow on the head, the patient had fallen face down and the assailant shot him throughout the anus. This interpretation was later confirmed by aggressor interrogation by the Police.\nThe following course of the patient was uneventful; he was estubated after 3 days, having a Glasgow coma scale of 11, and he was discharged to rehabilitation 13 days after the incident; 4 months later the head bullet was removed by anterior approach, while the lung bullet was left in place. |
A 35-year-old British Caucasian woman was referred by her general practitioner to our breast symptomatic services following a 3-week history of a lump below her left nipple. She also complained of some itchiness and a hot feeling in the same region. She had undergone bilateral breast augmentation using subglandular cohesive gel silicone implants 5 years previously.\nClinical examination revealed soft healthy implants which were clinically intact. There was a 2 mm mobile lump behind the left nipple.\nInitial ultrasound investigation showed both implants to be intact but there were multiple hypoechoic areas at the symptomatic site in the left retroareolar region which appeared superficial to the implant. Mammography showed an irregular contour of the left implant. A magnetic resonance imaging (MRI) scan of both breasts was suggestive of both intracapsular and extracapsular rupture of the left breast prosthesis.\nThe patient was seen in the breast clinic with the results of the radiological investigations (Figures , and ). On this occasion, she complained of a tender lump in the left axilla after a flu-like illness. Clinically, the lump was thought to be a lymph node and following review a month later, she was listed for excision biopsy of the axillary lesion.\nBefore the excision biopsy, the patient was reviewed as an outpatient by the plastic surgeon who had performed the original augmentation procedure. At this time, a similar tender lump to the left axillary mass was found in the right mastoid region.\nA combined procedure involving excision biopsy of the left axillary lesion and replacement of the ruptured implant was performed. Pus-like fluid was seen to surround the ruptured implant and ooze from the axillary node. Subsequent histological analysis showed that the axillary lymph node contained large amounts of silicone and demonstrated a lipogranulomatous reaction (Figures , ).\nTwo weeks postoperatively, the patient had clinically improved with resolution of her operative discomfort. |
The patient is a 69-year-old woman. She was infected with pneumonia at the age of three and had a high fever for a long period. After the fever abated, she became aware of hearing loss. Because she lived in a mountain village, she rarely visited medical facilities. Since then, she had never worn a hearing aid, had gone to a school for the deaf from elementary school to high school, and communicated with people in sign language. Her husband also used sign language, so she had not used oral communication from elementary school to the present. In 2011, family stress triggered the onset of tinnitus. Because around the same time, she began suffering from sleep onset disorder (it took about one hour until sleep onset), nocturnal awakening, and palpitations, she went to a nearby psychosomatic medicine clinic. She received medication at the clinic but her tinnitus did not improve, so she was referred to our department in 2014.\nVarious questionnaires were given at her consultation. The result of THI (Tinnitus Handicap Inventory) [], which is used to evaluate the severity of tinnitus, was a high of 94, which indicates the most severe form of tinnitus. Also, an SDS (Self-rating Depression Scale) [] score, which is used to measure depression tendency, was as high as 61, showing that she had a tendency toward depression. The STAI (State-Trait Anxiety Inventory) [], which is used to measure anxiety tendency, returned a State Anxiety (STAIs) score of 69 and a Trait Anxiety (STAIt) score of 67, indicating that she had an extremely high anxiety tendency. As one of the personal features of her tinnitus, she had no concept of the “loudness of tinnitus” because she had been deaf since childhood.\nIn imaging tests, there were no abnormal findings except for a slight enlargement of the inner ear canals observed by temporal bone CT scan. Head MRI showed no abnormal findings within the skull or in the internal auditory canals.\nFor treatment, we started oral administration of a serotonin reuptake inhibitor (SSRI) (paroxetine hydrochloride, Paxil®, 12.5 mg, started as one tablet a day, increased to three tablets a day) and a sleep-inducing agent (suvorexant, Belsomra®, 15 mg, one tablet a day). One and a half months later, the sleep onset disorder and nocturnal awakening improved, but early morning arousal persisted. Then, a benzodiazepine anxiolytic was added (etizolam, Depas®, 0.5 mg, one tablet a day). At four months after the initial visit, THI was 84, SDS 43, STAIs 50, and STAIt 48, which showed that her psychological condition had improved, although the tinnitus distress level did not change.\nAt this time, there was no improvement in perceived palpitations, and “pulsatile tinnitus” that seemed to synchronize with the heartbeat became the chief complaint concerning tinnitus, which led us to suspect that she had autonomic disorders. Six months after the initial visit, she started to receive psychotherapy (autogenic training). After the start of the treatment, we treated the patient with psychotherapy once a month, which continued until the 7th therapy session was completed. At the end of psychotherapy, our test results showed THI at 60, SDS 45, STAIs 32, and STAIt 43, showing a further improvement trend. The THI score was still high at 60, but the subjective tinnitus distress became “not so annoying,” and the “echoing tinnitus” that was the cause of the patient’s discomfort at the time of the initial visit disappeared. Only the pulsatile tinnitus, which seemed to be related to palpitations, remained.\nOne year and seven months after the initial visit, palpitations and pulsatile tinnitus, as well as anxiety and insomnia, were aggravated due to work stress. She restarted psychotherapy. At the same time, SSRIs were replaced by noradrenergic and specific serotonergic antidepressants (NaSSAs) (mirtazapine, Reflex®, 15 mg, started as one tablet a day, increased to two tablets a day). As a result, improvement of the palpitations and insomnia gradually occurred, and two years and one month after the initial visit, our test results showed THI at 40, SDS 47, STAIs 40, and STAIt 46.\nBy three years after the initial visit, the symptoms had stabilized and the anxiolytic drug was discontinued, but oral administration of the NaSSA and sleep induction drugs continued. The subjective tinnitus and palpitations at the time of sleep almost disappeared, and the sleep onset disorder and nocturnal awakening rarely occurred.\nNow 4.5 years have passed and she is taking only a low dose NaSSA (mirtazapine, Reflex®, 15 mg, 0.5 Tablets a day). The latest test results were THI 0, SDS 43, STAIt 47, and STAIs 50, indicating that the tinnitus distress had disappeared completely. |
A 9-year-old boy was admitted to the hospital with left-sided hydronephrosis for one week.
Due to abdominal pain, the patient underwent an ultrasonic examination in the out-patient department and was diagnosed as having "left hydronephrosis". It was suspected that the left side had the indication of a renal malformation. He was admitted to the hospital with "left hydronephrosis".
The patient exhibited abdominal flatness, a soft abdomen, normal bowel sounds, no varicose veins in the abdominal wall, no tenderness, no rebound pain, no abdominal masses, and slight percussion pain in the kidney.
The following characteristics were observed via an ultrasound (abdominal) exam-ination: The left kidney was enlarged (with a size of approximately 12.6 cm × 6.3 cm × 5.5 cm), the cortex was thin (with a thickness of approximately 1.0 cm), the pelvis was visibly separated, the lower part of the pelvis was obvious and separated within a range of approximately 7.8 cm × 5.9 cm × 5.4 cm, and the upper part of the pelvis was separated, both before and after treatment, with a diameter of approximately 2.2 cm. The size and morphology of the right kidney were normal, with a uniform cortical echo, a clear renal internal structure, no separation of the renal pelvis, and no clear occupying lesions. The upper segment of the left ureter was dilated (approximately 2.6 cm wide), and no significant dilation was observed in the middle and lower segments. No dilation was observed in the right ureter. Bladder filling was optimal, the wall was not thick, but smooth, and there was no abnormal echo inside of the kidney.
The following characteristics were observed via an intravenous pyelography (Figure ): No positive stones were observed in the abnormalities of the ureters and the bladder on both sides. After the injection of the contrast agent, bilateral ureters and bilateral kidneys were developed. The left renal pelvis was |
A 39-year-old Caucasian female with a history of cerebral palsy with spastic quadriplegia and congenital hydrocephalus with VP shunt presented to our institution for rapidly developing abdominal distension, pain, and coffee-ground emesis. On examination, the patient was lethargic and in obvious discomfort. Her abdomen was markedly distended with rebound tenderness and decreased bowel sounds. Abdominal ultrasound revealed severe free fluid in the abdomen which was confirmed on CT of the abdomen. Upper endoscopy was negative for active bleeding, varices, or ulcers. Diagnostic paracentesis showed serum-ascites albumin gradient (SAAG) > 1.1, total protein (TP) < 2, and clear yellow fluid. There was no evidence of cirrhosis on imaging with patent hepatic vein, splenic vein, and inferior vena cava (IVC). She had an echocardiogram that did not show any cardiomyopathy or restrictive pericarditis. Fibrosure showed F0, no hepatic fibrosis. Repeat CT later on in that admission showed large amount of intra-abdominal partially loculated fluid suspicious of CSFoma (Figure ).\nNeurosurgery was consulted and did not feel that CSFoma was likely. She followed up with GI outpatient for workup of the ascites with all workup for hepatic or portal vein etiology being negative, including a transjugular liver biopsy. It was determined that the fluid collection was not hepatic in nature and that CSFoma needed to be further investigated. She continued to experience worsening distension and foot swelling and again presented to the ED one week following discharge. She was seen by neurosurgery who requested additional abdominal imaging. Upon review of imaging studies, including CT of the abdomen/pelvis (A/P), it seemed CSFoma was caused by loculations and septations around the peritoneal end of the VP catheter. She was taken to the operating room for attempted laparoscopic drainage of her intraperitoneal CSF cysts. That was followed by open conversion to repair a loop of bowel that was inadvertently dissected during the laparoscopic procedure. There were multiple adhesions seen during the procedure with several anastomoses created. At the same time, one nonfunctioning peritoneal shunt was removed, and a second was replaced in the right upper quadrant. She recovered very well from her surgery and there have been no additional complications following the procedure. |
A 19-year-old male of Caucasian origin was admitted to our center as a polytrauma after a road traffic accident. He was previously fit and well, a nonsmoker with an alcohol intake of approximately 10 units per month. The accident, in which his motorcycle collided with an oncoming vehicle, caused him to sustain multiple significant injuries including unstable pelvic fractures and femoral fractures. He had bilateral pneumothoraces, extensive pulmonary contusion, and a splenic hemorrhage. He presented in extremis with signs of hypovolemic shock. He was intubated and resuscitated using local major hemorrhage protocols to achieve a blood pressure of 159/93, receiving ten units of packed red cells and four units of fresh frozen plasma in the emergency department.\nHe underwent an emergency laparotomy and splenectomy and was subsequently transferred to the intensive care unit, where he became increasingly hypoxic with features of adult respiratory distress syndrome (ARDS). This culminated in him receiving veno-venous extracorporeal membrane oxygenation (ECMO) from day 15 of his admission for 21 days. He returned to theater on day 21 for a massive haemothorax which required an emergency thoracotomy. After being decannulated from the ECMO circuit, he was stepped down to the general intensive care unit on day 36 and was transferred to the ward on day 55 before being discharged after a 4-month admission including a prolonged rehabilitation and recovery period.\nAfter presentation and commencement of ECMO, there was a relatively modest change in liver function tests. Alkaline phosphatase (ALP) increased from 55 to 143 IU/L between day 1 and day 6 of hospital admission and no persistent alanine transaminase (ALT) rise until after decannulation. Proceeding decannulation, there was a sequential increase in ALP peaking at 2335 IU/L on day 113. ALT rose to a lesser extent, peaking at 781 IU/L on day 52. The bilirubin did not rise above 57 μmol/L. The pattern of liver function tests is summarized in Figure . Autoantibody screen, immunoglobulins, and viral hepatitis serology were negative.\nSerial ultrasound, computerized tomography (CT), and magnetic resonance imaging excluded biliary stones and sludge. The liver on CT at day 1 showed normal liver and biliary structure (Figure ), and it was not until 10 months after the admission, the repeat magnetic resonance cholangiopancreatography (MRCP) demonstrated a multistenotic pattern of disease within the intrahepatic ducts (Figure ).\nIn the absence of significant casts within the biliary tree and no evidence to suggest biliary sepsis, endoscopic retrograde cholangiopancreatography (ERCP) was felt not to be helpful. A conservative management approach was taken, and the patient was instigated on ursodeoxycholic acid to help improve cholestasis. With bilirubin improving, the patient was closely monitored as an outpatient upon discharge for progressive liver disease and dysfunction. Repeat imaging and noninvasive fibrosis assessments were undertaken. Despite having evidence of SSC, the synthetic liver function has remained excellent, cholestasis markers have improved, and there have been no episodes of cholangitis or biliary sepsis for over 1 year. |
A now 22-year old woman was diagnosed with mutation negative cryopyrin-associated periodic syndrome (CAPS)/chronic infantile neurologic, cutaneous, articular (CINCA) syndrome at the age of 10. She had been suffering from rash, chronic meningitis, bone dysplasia and growth retardation since birth.\nFrom the first year of life recurrent episodes of pyelonephritis had been present, associated with vesicourethral reflux, leading to impaired renal function and proteinuria. These had been progressive since the age of 17.\nDirectly after she was diagnosed with CAPS, she was started on anakinra. 8 years later she switched to the selective IL-1β antibody canakinumab. Two years later she developed end stage renal disease due to a combination of recurrent pyelonephritis, use of NSAIDs and hypertension. Kidney biopsy was contraindicated because of small kidney size, but there were no other signs of AA amyloidosis. A pre-emptive renal transplantation was planned and 2 months before, canakinumab was switched back to anakinra 100 mg on alternating days; the shorter half life of the latter makes it more easy to stop in case of complications. This patient received a renal transplantation of a living related donor almost 2 years ago. She was started on immunosuppressive therapy with MMF, tacrolimus and prednisone, while anakinra was continued. Kidney function increased rapidly and she could be discharged 6 days after the transplantation. Because of incomplete control of inflammation 4 months after the transplantation the dose of anakinra was increased stepwise to 100 mg each 36 h and later to 100 mg daily.\nShe is now doing well. She has been admitted three times since the transplantation: 3 months post-transplantation because of influenza and primo-Epstein Barr virus (EBV) infection (2 days) and both 8 and 11 months after the transplantation because of diarrhea, with positive norovirus PCR during the last episode. This may be related to her job at a children's day care center. MMF was switched to azathioprine because of diarrhea. She still uses anakinra 100 mg each day. |
The patient is a 24-year-old male, Igbo by tribe, and an undergraduate, resident in Enugu, Southeast Nigeria. He was referred from the Enugu State University Teaching Hospital staff clinic on account of persistent hiccups of 4 days and confusion of 2 days' duration. The patient was in apparent good health until about 14 days before presentation when he was involved in a road traffic accident, in which he sustained minor blunt injuries, bruises, and lacerations but no fractures. On presentation, he was seen, admitted, and managed by the General Surgical Unit of University of Nigeria Teaching Hospital, Enugu, Nigeria. Four days into the hospital admission in the surgical unit, he developed progressive weakness and worsening of the body pain, and hiccups which became so severe that the patient could not eat without fear of discomfort. As a result of the weakness, he was unable to walk around or bath himself. Two days later, the patient developed nausea and recurrent vomiting, which smothered on until he developed progressive confusion and altered sleep pattern. The patient later developed facial puffiness which was closely followed by bilateral swelling of the legs. The leg swelling progressed to the thigh; he had no associated fever or seizures. His urine volume was noticed to have reduced and a collected urine sample turned dark brown after 6 h. There was no history of hematuria or frothy urine. He had no bleeding from any orifice. There was no history of hypertension or diabetes mellitus. He had no known risk factor for muscular disease from history obtained, and there is no family history of muscular disease.\nPhysical examination revealed an acutely ill-looking young male. He was in respiratory distress, was afebrile (temperature 37.1°C), not pale, and not anicteric. There was no significant peripheral lymphadenopathy. He had bilateral pitting lower limb edema. Asterixis was present.\nThe significant observations on examination of the systems were that the patient was drowsy with flapping tremor and bilateral pitting lower limbs and facial edema. He had a pulse rate of 102 beats/min which was of small volume, with a blood pressure of 88/56 mmHg and respiratory rate of 34 cycles/min. Four lacerations and bruises, all dressed; they were neat and not offensive. He had a bilateral basal crepitation.\nThe following investigations were carried out: urinalysis, urine microscopy culture and sensitivity (MCS), full blood count (FBC), prothrombin time, activated partial thromboplastin time, serum electrolytes, urea, and creatinine (SEUC), serum calcium, serum phosphate, serum uric acid, fasting blood sugar, serum protein, serum CK, serum myoglobin, electrocardiogram (ECG), chest X-ray, and renal ultrasound scan. The results are shown in Tables -.\nA diagnosis of rhabdomyolysis complicated by AKI was made.\nHemodialysis was commenced for the patient, intravenous fluid 5% dextrose/saline 1 L 8 hourly for 72 h, ciprofloxacin 200 mg 12 hourly for 72 h, and later converted to oral ciprofloxacin 500 mg 12 hourly for 7 days. The patient was also on tramadol 50 mg 12 hourly for 5 days. He regained full consciousness after the first session of hemodialysis. However, he received two further sessions of hemodialysis. His edema resolved within 15 days. The results are shown in Tables -. He was discharged after 18 days on admission. His first clinic check-up after 2 weeks showed that he had no edema and had normal BP. Laboratory evaluation at day 32 showed FBC, SEUC, urinalysis, serum CK, and serum myoglobin, and ECG results displayed in Tables -. He was stable and doing well. Further follow-up of this patient continued but was not captured, as this report was written 2 weeks after his discharge from the hospital. |
The patient was a 3-year 7-month-old female referred by her speech-language pathologist, presenting with oromyofascial dysfunction characterized by speech sound production errors, difficulty swallowing, open mouth breathing, and noisy breathing during sleep. With respect to sleep, there were reports of difficulties going to sleep, waking up two to three times per night to drink water, getting up to go to the bathroom, open mouth breathing while asleep (), snoring during sleep, and sweating more than usual during sleep. She experienced wheezing that was associated with asthma, which was treated with an Albuterol sulfate inhaler. There were reports of difficulty with effective chewing. In addition, the patient would eat around 50% of her meals, before refusing the rest. She experienced chronic cough and recurrent upper respiratory tract infections.\nPhysical examination () of the patient showed her to be well developed, well nourished, and to appear the stated age. The patient was alert, oriented, able to communicate, and respond appropriately to questions. During the nasal examination, the nose had no external deformity. The nasal septum was straight, and the inferior turbinates were grade 2 bilaterally. There were class 3 dental occlusion and a class 3 facial-skeletal relationship characteristic of anterior-posterior maxillary deficiency. Oropharyngeal examination showed grade 3 modified Mallampati tongue position and grade 2 tonsils. The patient was found to have a restrictive class 2 upper labial frenulum with tethering of the upper lip () and restrictive grade 4 lingual frenulum [] ().\nBased on the patient history and the physical examination, the assessment revealed that the patient had functional and structural mouth breathing due to open mouth posture and low tongue posture in the setting of restrictive labial and lingual frenulum. The risks and benefits of lingual and labial frenuloplasty to facilitate lip closure and proper tongue resting posture were discussed with the parents and included, but were not limited to pain, inflammation, bleeding, scarring, need for revision surgery, and failure for significant improvement. An informed consent document was signed by the parents.\nThe maxillary labial frenuloplasty was performed under general anesthesia. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the maxillary labial frenulum via a 27-gauge needle. Pressure was applied lateral to the frenulum to locate the point of maximum tension. The maxillary labial frenulum was incised at the base of attachment with the use of sharp scissors. A V-to-Y lip closure was performed with a 4-0 chromic suture applied in a simple interrupted fashion ().\nThe lingual frenuloplasty procedure was then performed. A 2-0 silk suture was applied to the tip of the tongue as a retraction stitch. Local anesthesia was achieved by applying 1 cc of 0.25% Marcaine with 1 : 200000 epinephrine to the lingual frenulum via a 27-gauge needle. The tongue was retracted in the anteroposterior direction extending to the roof of the mouth and maxillary central incisors. Tension was applied to the floor of the mouth to protect the floor of mouth salivary glands, as well as Wharton's duct. A hemostat was used to clamp the restrictive lingual frenulum 5 mm above the attachments of the sublingual gland duct. The fibrous band was gently excised with the use of iris scissors. The underlying myofascial fibers of the genioglossus muscle were dissected further, with blunt cotton tips and sharp iris scissors being used to release the muscle from the overlying mucosa. The dissection was continued until there was adequate improvement to the tongue range of motion such that the tongue could extend up towards the maxillary central incisors in maximal mouth opening position. Simple interrupted sutures were used to close the diamond-shaped defect into a vertical line, as a means to lengthen the ventral tongue, with 4-0 chromic suture applied in a simple interrupted fashion. In total, the tongue was released from a grade 4 restricted range of motion to a grade 1 range of motion (). All wounds were hemostatic at the completion of the procedure. The patient was gently awoken from anesthesia and taken to recovery in stable condition.\nThe patient returned to the clinic four days after the procedure. The wound sites were healing appropriately, and there were no postoperative complications observed. The patient's mother reported that within the first day of returning home, the patient's issues with chewing had improved significantly, and she was more interested in eating different foods. In addition, her appetite appeared to have increased, and the patient would complete her entire meal before asking for more food (as compared to having only eaten around 50% before treatment). By the fourth day after surgery, the patient exhibited closed-mouth nasal breathing while asleep (). There were no longer any observed events of snoring and/or noisy breathing. The mother reported that the patient had remained compliant with myofunctional and speech therapy.\nThe patient returned for a 2-month postoperative visit. During the examination at this visit, no scar tissue was observed, and the wound sites had closed. Grade 1 tongue range of motion was observed ().\nThe patient's family wrote in a letter to the clinic approximately six months after the procedure, mentioning that the patient was doing very well with no complications. In addition, the patient had been reported to have completely stopped mouth breathing and snoring while asleep. However, the patient was reported to still have occasional episodes of cold and cough, as well as one episode of asthma exacerbation. Finally, the patient was reported by her family to have made progress with myofunctional and speech therapy, but the goals of eliminating the tongue thrust, achieving proper resting posture, and improving speech sound production errors were not met due to early discontinuation of treatment. |
A 16-year-old male with a past medical history of Marfan syndrome with severe pectus excavatum, significant scoliosis, and generalized anxiety disorder presented for PSIF from T3 to L4 to correct his scoliosis deformity. Preoperative evaluation demonstrated a left lumbar curve of 64 degrees and lumbar kyphosis of 14 degrees. His curve corrected to 40 degrees with a fulcrum bend. Evaluation 1 year prior to the surgery revealed no cardiovascular abnormalities with a normal ejection fraction of 56%.\nInduction of anesthesia and intubation were uncomplicated. His preprocedure vitals were stable and within a normal range. The patient was placed in a prone position. Due to the ongoing hemodynamic instability and need for abundant amounts of vasopressor support, the chest pads were repositioned which improved his hypotension for a time. The surgical aspects of the procedure proceeded uneventfully. The patient had transverse process pedicle hooks placed bilaterally at T3 and had navigated pedicle screws placed T4-L4. Facetectomies were performed at each level and a Cortrel-Dubousset derotation maneuver was performed to correct the scoliosis. Sequential distraction and compression was performed to correct the deformity in combination with in situ rod bending in both the coronal and the sagittal plane. Throughout the procedure, he had a significant vasopressor requirement. The patient received adequate crystalloid and colloid in addition to 4 units of fresh frozen plasma and approximately 600 cc of cell saver. The case lasted 10 hours and was complicated with 1.4 L of blood loss.\nAt the conclusion of the case, the patient was flipped supine and transferred to the postanesthesia care unit (PACU) intubated and on mechanical ventilator support in hopes to later extubate once his anesthetic dissipated. He was slow to emerge from anesthesia, and initial vital signs showed significant hypotension (systolic blood pressure 66 mmHg with a mean arterial pressure (MAP) of 54) and tachycardia into the 140s. The patient was started on phenylephrine and was transferred to the Surgical Intensive Care Unit (SICU) for ongoing management.\nOver the next few hours, the patient became interactive and was extubated uneventfully; however, his hypotension continued despite phenylephrine which resulted in a transition to a norepinephrine infusion. His vitals continued to demonstrate a narrowed pulse pressure with hypotension and tachycardia, concerning for cardiac tamponade and/or active postsurgical bleeding. Laboratory investigations demonstrated a hemoglobin of 12.5 g/dL, international normalized ratio (INR) of 1.6, activated partial thromboplastin (APP) time of 30, and a fibrinogen of 155 mg/dL which improved to 187 mg/dL without intervention. He was given fluid boluses and continued on norepinephrine with minimal improvement. His initial postoperative labs were significant for a lactate of 3.6 mmol/L and a creatinine (Cr) of 0.8 mg/dL. His lactate peaked at 5.0 mmol/L. Given continued concern for myocardial depression, tamponade physiology, or active bleeding, he was assessed with an emergent formal bedside transthoracic echocardiogram and CT angiogram of the chest and abdomen. These demonstrated no active sources of bleeding, no pneumothoraces, and a relatively unfilled, hyperdynamic left ventricle with a postoperative Haller index that increased from 8.7 to 11.3 ().\nOver the next several hours, surveillance labs showed an evolving acute kidney injury and signs of global hypoperfusion as his lactate continued to rise. We suspected that his pectus excavatum had compromised his cardiac function by mechanical obstruction and that this compromise was exacerbated by significant intraoperative blood loss, fluid deficit, and residual effects of his anesthetic. Cardiothoracic surgery was consulted and gave consideration to immediate surgical intervention should the patient not improve with medical management. We utilized fluid boluses and vasopressor support over the course next 24 hours (). He slowly improved hemodynamically. His acute kidney injury, lactate, and urine output improved, and he was ultimately transferred out of the ICU on postoperative day two without additional surgical intervention. Preoperative and postoperative radiographs demonstrating the spinal instrumentation and deformity correction are provided (). |
A 58-year-old African-American female presented to a hospital emergency room with a superotemporal marginal corneal ulcer of the left eye two clock hours in length, with mild corneal thinning. Uncorrected visual acuity on the initial exam was 20/30 in the right eye and 20/50 in the left. Both eyes showed diffuse punctate corneal epitheliopathy in the palpebral fissure. Her past medical history included alcohol use since the age of 14, serious alcoholism since the age of 34, and chronic pancreatitis and diarrhea since the age of 52. She reported that she drank 6–12 beers per day. She had a smoking history of >50 pack-years as well as a history of gastrointestinal bleed, liver cirrhosis, cholecystectomy, chronic obstructive pulmonary disease, and anaphylactic reaction to peanuts. She had lived in urban USA since birth.\nAfter corneal culture, the patient was started on hourly topical moxifloxacin 0.5% eyedrops in the left eye as well as artificial tears in both eyes. Culture was positive for alpha-hemolytic streptococcus and catalase-positive Gram-positive cocci in groups with preliminary identification as coagulase-negative Staphylococcus. She returned 3 days later, with an unchanged exam except for mild injection of the right eye, and was maintained on the same antibiotic drops but with an increase in artificial tears in the right eye. Three days after that (6 days after the initial visit), she presented to an outpatient office with bilateral marginal corneal ulcers (fig. , fig. , fig. ), both with a thinning >50% and parallel to the limbus. The left eye also had a small hypopyon. Uncorrected visual acuity was 20/40 right, 20/80 left. She did not complete workup or treatment and was lost to follow up for over 1 week. She returned to the emergency room 17 days after initial presentation, with a complaint of bilateral severe vision decrease and painful swelling of the left lower lid of 2 days duration. She was hospitalized at that point.\nOn admission, vision was hand motions in both eyes. The right eye had deep marginal thinning for 6 clock hours inferiorly. The left cornea had a marginal area of deep stromal thinning temporally for 5 clock hours, and the inferior lid showed tender erythema and edema. Diffuse severe bilateral corneal edema and hypopyon (1.5 mm in the right eye and 1.0 mm in the left eye) were present. B-scan ultrasound of the fundus of both eyes was unremarkable.\nAfter corneal culture, the patient was placed on bilateral topical ocular application of fortified vancomycin eyedrops (25 mg/ml) every hour (for Gram-positive coverage) and moxifloxacin 0.5% eyedrops every hour (for Gram-negative coverage). Cultures from admission grew Pseudomonas aeruginosa and Staphylococcus hominis from the right eye and Staphylococcus warneri and Staphylococcus lugdunensis from the left eye. On the third day of hospitalization, when culture results were available, the eyedrops were changed to fortified vancomycin every 2 h and fortified tobramycin (14 mg/ml) every 2 h. Tobramycin was started because of its potency against Pseudomonas. The presence of three Staphylococcus species in the cultures warranted continuation of the vancomycin eyedrops, but we decreased the frequency to every 2 h around the clock to reduce epithelial toxicity and patient stress. The left lower lid cellulitis did not yield any material for culture, but the patient received IV cefazolin (1 g every 8 h) for 2 weeks with resolution of the cellulitis. Due to the severe corneal ulceration, bilateral amniotic membrane grafts (AmbioDisk; IOP Ophthalmics, Costa Mesa, Calif., USA) with an overlying 16.0-mm Kontur bandage contact lens (Kontur Kontacts, Hercules, Calif., USA) were placed on the fourth day of hospitalization. A urinary tract infection found during hospitalization was culture-positive for vancomycin-resistant Enterococcus and was treated with linezolid.\nTesting revealed a marked vitamin A deficiency, with a level of 0 μg/dl (reference range 18–77). Vitamin A analysis was performed by LabCorp using high performance liquid chromatography. The patient had mild protein malnutrition with total protein 6.5 g/dl (reference range 6.6–8.7). Her BMI was 21.4 (normal range 18.5–25). Laboratory analysis showed hypokalemia, hyponatremia, hypomagnesemia, and hypocalcemia. Testing ruled out HIV, tuberculosis, syphilis, sarcoidosis, rheumatoid arthritis, hepatitis A, B, C, and Wegener granulomatosis. The ANA titer was elevated (40) with a fine speckled pattern, but not to the level of significance (80). Sjogren syndrome testing for SS-A/Ro, SS-B/La, Smith antibody, and ribonucleoprotein antibodies were all negative by microparticle multiple immunoassay. Erythrocyte sedimentation rate and C-reactive protein were normal.\nIntramuscular vitamin A was not available, so it was repleted with 100,000 IU orally for 3 days, followed by 50,000 IU orally every day for 2 weeks. After initial treatment of the infections and after ruling out systemic tuberculosis, systemic steroids were given (IV methylprednisone starting at 50 mg daily and tapered over 2 weeks). The patient also received thiamine, folate, ascorbate, calcium, vitamin D, multivitamins, topical cyclosporine 0.05% eyedrops, and intensive eye lubrication. The patient's diarrhea, pancreatic enzyme deficiencies, electrolyte abnormalities, and protein malnutrition were treated, and she received extensive nutritional and alcoholism counseling. |
A 54-year-old female with a past history of left lower extremity deep vein thrombosis on Apixaban was transferred to our hospital due to a 1-week complaint of rectal bleeding. Associated symptoms included moderate to severe central abdominal pain for 1 month. Upon our evaluation, the patient looked in mild distress with poor functional status. Her vital signs were normal, but the abdomen was diffusely tender on palpation and remarkable for shifting dullness. The basic laboratory workup showed microcytic anemia with a hemoglobin level at 9.6 g/dL and mild acute kidney injury with BUN at 20 mg/dL and creatinine of 1.9 mg/dL. Cancer antigen 125 and carcinoembryonic antigen were elevated at 147.8 and 2,135 ng/mL, respectively. Septic screen was unremarkable.\nColonoscopy was done which showed a polypoid rectosigmoid mass and the biopsy findings were consistent with well-differentiated adenocarcinoma in a background of tubulovillous adenoma (Fig. ). Staging CT scans revealed moderate ascites and multiple findings including multiple variable sized liver lesions with contrast enhancement, bilateral solid ovarian masses measuring 8 × 5 × 7 cm on the left side and 5 × 5 × 4.6 cm on the other side, small basal pulmonary nodules and small omental deposits.\nThe hospital course was complicated by progressive respiratory distress requiring ICU transfer for monitoring. Chest X-ray and arterial blood gases did not show significant abnormalities that can explain her new complaint. EKG showed sinus tachycardia and CT angiogram was negative for pulmonary embolism. Transthoracic echocardiography revealed a large mass in the right atrium measuring 3.8 × 3 cm, which seems to be attached to the interatrial septum and protrudes into the right ventricle through the tricuspid valve during diastole (Fig. ). Cardiac MRI with contrast was performed for better characterization of the mass (Fig. ). It showed a heterogenous mass of irregular and multilobed shape with contrast enhancement that is consistent with atrial metastasis.\nThroughout the patient hospitalization, the anticoagulation treatment had been stopped, which led to resolution of rectal bleeding. However, inferior vena cava filter was placed with no complications due to the risk of embolization. The patient was not fit for chemotherapy or any surgical intervention given her poor prognosis and functional status in the setting of advanced stage of her disease. After discussion with the patient and her family, a decision was made to change her code status to DNR (do not resuscitate) and focus on palliative treatment of her disease. Ultimately, she was discharged home and planned for home hospice before she died within 2 weeks of her date of discharge. |
A 15-year-old girl in good health presented to the Department of Endodontics, Wuhan University Hospital of Stomatology, in November, 2014, with a chief complaint of cold water pain on her left upper posterior tooth for the last 5 days. Three months earlier, she had caries filled in the same region. Intraoral examination revealed that tooth #13 had an existing distal occlusal composite restoration. Tooth #13 showed sensitivity to pulp test and no sensitivity to palpation and percussion. Radiographic examination showed distal a filling shadow close to the pulp and a fully developed root and closed apex (). On the basis of the results of the clinical and radiographic examinations, the diagnosis for tooth #13 was chronic pulpitis.\nAn informed consent was obtained from the patient and her parent for regenerative endodontic treatment. An access cavity preparation was performed after local anesthesia with 2% lidocaine and rubber dam isolation. The canal was irrigated copiously with 5.25% sodium hypochlorite and 17% EDTA. The canal was partially dried with sterile paper points and then dusted with a mixture powder of ciprofloxacin and metronidazole. The tooth was then temporized with zinc oxide eugenol cement.\nAt 2-week follow-up, the patient was asymptomatic. The tooth was not sensitive to percussion and palpation. After local anesthesia by using 2% lidocaine and rubber dam isolation, the temporary restoration was removed. The antibiotic mixture was completely removed with 5.25% sodium hypochlorite. The apical foramen was enlarged up to 0.6 mm with a size #60 hand file (K-file). Seventeen percent EDTA was used as a final irrigating agent and left in the canal for about 1 minute. Then, the canal was dried with sterile paper points. A size #25 K-file was introduced into the root canal to irritate the periapical tissue and to create some bleeding into the root canal. The mineral trioxide aggregate (MTA) was placed approximately 2 mm below the cementoenamel junction. The tooth was restored with glass ionomer cement ().\nAt 3-month recall, the patient was asymptomatic. The tooth was not sensitive to percussion or palpation and not responsive to pulp testing. The radiograph showed nearly a normal periodontal ligament space (). The tooth was restored with composite resin after removal of the glass ionomer cement.\nAt 6-month follow-up, tooth #13 regained pulp sensibility and responded positively to the electric pulp tester (EPT) and Endo-Ice at a similar extent as the control tooth. The radiographic results showed narrowing of the canal space with radiopaque shadows ().\nAt 12-month recall, there was no pain on percussion and palpation. Tooth #13 was normally responsive to Endo-Ice and EPT. The radiographic results revealed evidence of root wall thickening ().\nAt 30-month recall, tooth #13 still showed normal pulp vitality compared to the control tooth, and no periapical lesion was found on the X-ray film (). The recovery of pulp vitality at different follow-up time points was summarized in . |
A 44-year-old Hispanic man was diagnosed in October 2006 with a primary mediastinal seminoma complicated by superior vena cava (SVC) syndrome. He was started on a first-line systemic therapy of bleomycin, etoposide and cisplatin (BEP). Bleomycin (30 units) was administered on days 2, 9 and 16; etoposide (100 mg/m2 intravenously) on days 1 to 5; and cisplatin (20 mg/m2 intravenously) on days 1 to 5 every three weeks for a total of four cycles. The total cumulative bleomycin dosage was 360 units with the last dose of bleomycin administered on 29 December 2006. Following chemotherapy, the patient achieved a complete response to treatment with resolution of the SVC syndrome. His anterior mediastinal mass decreased substantially in size, with a complete normalization of the standardized uptake value (SUV) by computed tomography (CT) and positron emission tomography (PET); his beta human chorionic gonadotropin (β-HCG) level decreased from 5452 to an undetectable level; and his alpha fetoprotein (AFP) level remained within the normal range. He tolerated the chemotherapy without any adverse side effects.\nThree months after the treatment, he presented at the emergency department at Stony Brook University Medical Center, having suffered from progressive shortness of breath for three days but without any other obvious precipitating factors. He was not on any medication and he did not have any gastrointestinal symptoms. Physical examination revealed tachycardia, tachypnea, hypoxia and decreased breath sounds with fine crackles bilaterally. Chest X-ray showed a right lower lobe infiltrate. Interestingly, his eosinophil count had increased from a baseline level of 2% to 10%, although his total white blood cell count was within the normal range. Subsequent CT of his chest showed extensive patchy ground-glass opacities in the right upper lobe, middle lobe and left lung without evidence of any pulmonary embolism (Figure ). He was treated with ceftriaxone and azithromycin empirically for community acquired pneumonia. Because he did not respond to a four-day course of the antibiotic treatment and showed worsening dyspnea, our patient was admitted to the medical intensive care unit, and underwent a thoracoscopic right middle lobe wedge biopsy to investigate possible bleomycin-induced lung toxicity. Pathological examination of the lung tissue revealed severe widespread organizing pneumonia with accompanying eosinophil-rich inflammatory infiltrates (Figure ). Cultures and stains of the tissue showed negative for any infectious agents including Mycobacterium tuberculosis, viral, fungal or Pneumocystis jirovecii infection. There was also no evidence of seminoma recurrence.\nOur patient was started on oral prednisone 70 mg daily, and his respiratory status slowly improved over the next few days. Treatment continued with a high-dose prednisone for one month. A follow-up CT of his chest revealed a complete resolution of the bilateral ground-glass opacities (Figure ). Furthermore, his peripheral eosinophilia level decreased from 10% to <2%. The prednisone was then tapered off over a period of three months. Our patient has remained symptom-free and disease-free for two years. |
A six-year-old, male child with intubation status was transferred to us from another hospital. The patient had no medical history except for having had atopic dermatitis. He had been admitted to the previous hospital five days earlier due to fever and dyspnea. He was diagnosed with asthma and suspected pneumonia and was treated with antibiotics, bronchodilator nebulizers, and steroids. However, these treatments were ineffective. The patient was transferred to the intensive care unit after intubation was performed as his dyspnea had intensified with carbon dioxide (CO2) retention, as seen on arterial blood gas analysis (ABGA). Following admission to our hospital, the patient experienced severe respiratory failure even though he was on conventional ventilator support (). We decided to perform an early application of venovenous extracorporeal membrane oxygenation (ECMO) for respiratory support. The patient's body weight was 20 kg, and his height was 119 cm. An outflow catheter (22 Fr; DLP, Medtronic Inc., Minneapolis, MN, USA) was inserted via the right internal jugular vein, and an inflow catheter (16 Fr; RMI, Edwards Lifesciences LLC, Irvine, CA, USA) was inserted via the right femoral vein (). Pump flow was maintained at 100% to 120% of the cardiac index (2.4 L/min/m2), and ECMO operated smoothly during the entire time of its use. A chest X-ray and the ABGA results showed marked improvement two days after application of the ECMO. The ECMO weaning was possible due to the significant improvement in the ABGA results along with the fact that the ventilator was on the moderate setting three days following the application of the ECMO (). The total support time was 62 hours. Due to the patient's continued improvement, extubation was performed the day after weaning from the ECMO was complete. The patient made a full mental recovery, and no neurological deficit was noted on clinical evaluation. A brain computed tomography and electroencephalography revealed no specific abnormalities. A Doppler sonography was performed on the catheter insertion sites, which revealed smooth luminal narrowing but showed patent vascular flow in the right internal jugular vein. The right femoral vein showed luminal narrowing and mild wall thickening, but it had patent vascular flow at the catheter insertion site. The patient's transfer to the general ward was possible on the next day following extubation. Because of the continued improvement in his general condition, the patient was discharged without other complications (). |
A 35-year-old male with a history of hyperlipidemia and seizure disorder presented to an outside hospital following a breakthrough seizure, where he was incidentally also found to have punctate areas of acute cerebral infarcts in multiple vascular territories. Additional workup revealed the presence of a left atrial thrombus and newly diagnosed atrial fibrillation. He was ultimately discharged to home on apixaban. The patient then re-presented a month later for evaluation of transient diplopia, expressive aphasia, daily right temporal headaches, and right facial and left leg weakness. MRI of the brain showed new areas of diffusion restriction in the left cerebellar hemisphere and left medial occipital lobe (Figure ). CTA showed no signs of carotid occlusion or stenosis. The etiology of his multifocal strokes was thought to be related to his newly diagnosed atrial fibrillation and left atrial thrombus.\nThe patient was then transferred to our hospital for further evaluation. His initial NIH stroke scale was 8 (primary deficits were including unilateral facial palsy, bilateral lower extremity pronator drift and ataxia). Stroke labs, including lipid panel and hemoglobin A1C, were unremarkable. MRI of the brain with contrast showed a new infarct in the splenium of the corpus callosum in addition to prominent generalized meningeal enhancement (Figure ). MRI of the spine with contrast showed possible meningeal enhancement as well as punctate areas of encephalomalacia in the C3-4, C7, and T3 spinal levels. A bedside LP revealed a mildly elevated opening pressure of 24 cm H20, lymphocytic pleocytosis (WBC 150 K/cumm, 61% lymphocytes), protein 170 mg/dl, hypoglycorrhachia of 15 mg/dl, and presence of cryptococcal antigen. Other notable CSF labs included the presence of 11 oligoclonal bands. He was diagnosed with cryptococcal meningoencephalitis and started on a four-week course of amphotericin B and flucytosine. A repeat LP after several days of treatment showed a normal opening pressure of 14 cm H20, mildly improved pleocytosis (WBC 130 K/cumm, 84% lymphocytes), protein 172 mg/dl, and glucose 14 mg/dl. He did not require any additional lumbar punctures, and his symptoms (including headaches and left lower extremity weakness) gradually improved. The patient was discharged to an inpatient rehabilitation facility prior to returning home.\nThough our patient in Case 1 had a history of an unknown autoimmune disease, our patient in Case 2 had no history of autoimmune disease or other existing immunodeficiency. Both patients developed multiple cerebral infarcts in multiple vascular territories in the setting of cryptococcal meningoencephalitis, though our patient in Case 2 also had recently diagnosed with atrial fibrillation which further confounds the underlying etiology of his strokes. |
A 54-year-old male patient reported with a chief complaint of pain, swelling and mobility of the lower right back tooth for 4 months. On inspection, the lesion was a well-defined oval intraoral swelling of 2 cm × 1 cm on the buccal aspect of 43, 44 and 45 []. On palpation, the swelling was firm to hard in consistency. 44 exhibited Grade I mobility and found to be nonvital. The patient had an unremarkable medical history.\nCoexisting with the intraosseous lesion, an oval shape, soft tissue swelling of size 3 cm × 1 cm was evident on the lingual aspect of the attached gingival of 41, 42, 43 and 44. No clinical attachment was exhibited by the two lesions [].\nOrthopantomogram (OPG) and intraoral periapical radiograph (IOPA) revealed a triangular multilocular radiolucency in relation to 43,44 regions. Associated bone loss and divergence of roots of 43 and 44 were also observed. Root resorption was present for 44 [Figure and ]. Buccal cortical plate expansion was also noticed. A provisional diagnosis of lateral periodontal cyst, odontogenic myxoma and peripheral ameloblastoma was considered.\nIntraoperatively, a firm multilocular swelling measuring about 2.5 cm × 3 cm was found in buccal aspect extending from the alveolar process to half way down the lower border of the mandible superioinferiorly and from the distal side of the right canine region to the mesial side of the right mandibular first premolar tooth anteroposteriorly. Surgical excision was planned under local anesthesia. On elevation of the mucoperiosteal flap, a multilocular appearance with irregular bony margins was noted [Figure and ]. Curettage of the buccal aspect and complete excision of the lingual lesion was done and sent for histopathological examination. Lingual cortical bone was found to be intact.\nHistopathological examination revealed numerous variable sized tumor islands of odontogenic epithelium dispersed in a matured connective tissue stroma []. The islands consisted of peripheral layer of flattened or low cuboidal cells and central squamous cells [Figure and ], along with attempting keratin formation in some foci []. Cystic degeneration was also observed in some islands.\nThe lingual lesion showed an ameloblastomatous cystic lining with granular changes in superficial cells associated with a moderately fibrous cystic capsule [Figure -]. Ameloblastomatous follicles with peripheral tall columnar cells exhibiting reversal of polarity and subnuclear vacuolization with central round to oval-shaped granular cells' changes were observed. The granular cells exhibited coarse eosinophilic granular cytoplasm with peripherally arranged pyknotic, hyperchromatic nuclei [Figure and ]. The granular cells were also seen dispersed within the connective stroma []. The granular cells stained positive for Periodic acid–Schiff staining [].\nA histopathological final diagnosis of squamous odontogenic tumor (SOT) was established along with a synchronous association with peripheral cystic GCA. No recurrence on re-examination was observed following a 2 years of follow-up. |
A 52-year-old female presented with generalized grade three pruritus of six months duration. There was no history of any skin lesions, fever, abdominal pain, jaundice or fatigue. Neither did she have any history of leg swelling, facial puffiness, decreased urine output or gastrointestinal bleed. She was detected to be hypertensive one year back for which Amlodipine was prescribed. Examination revealed a 5 cm hepatomegaly more in the epigastric region along with separate masses in both lumbar regions which were bimanually palpable and ballotable. Liver function test revealed normal bilirubin and aminotransferases but a more than three times elevation of serum alkaline phosphatase and γ-glutamyl transferase. Renal function was normal and there was no proteinuria.\nInitial ultrasound scan was followed by CT scan of abdomen () which showed multiple noncommunicating cysts of varying size in both lobes of liver. There was mild dilation of intrahepatic biliary radicles in both lobes. Common hepatic duct and common bile duct (CBD) appeared normal. Both kidneys were bulky with multiple noncommunicating cysts.\nMRI abdomen () with MRCP () showed multiple T2WI high signal parenchymal cysts mostly in the left lobe of liver, the largest measuring 9.8 × 6.7 cm. Mild dilation of intrahepatic bile ducts was seen. A few small cysts in communication with the dilated bile radicles were also noted. The proximal CBD was not visualized due to compression by the cysts at the porta hepatis. The distal CBD was normal in caliber. Spleen and pancreas were normal. Both kidneys were markedly enlarged with parenchyma totally replaced by innumerable cortical cysts, the largest measuring 7×5.6 cm. The large cysts in the left lobe of liver and porta hepatis compressing the bile ducts were not communicating with the biliary system. A diagnosis of polycystic liver disease with Caroli’s disease and biliary obstruction was made. The patient also had polycystic kidney disease. Hence our patient presented for the first time with longstanding pruritus as the only manifestation of polycystic liver disease.\nThe patient was planned for laparoscopic deroofing of liver cysts. However she was lost to follow up. |
A 68-year-old male presented a two-week history of malaise, left-sided abdominal pain and intermittent fever. The only apparent incident of significance prior to presentation was a colonoscopy performed two weeks ago for investigation into his recent change in bowel habit. The colonoscopy was performed with adequate bowel preparation and revealed a 10 mm pedunculated rectal polyp. Apart from a rectal polypectomy with snare diathermy, 3 mm-diameter biopsies were taken randomly from the terminal ileum, ascending colon and descending colon. Hemostatic clips and adrenaline injection were not required as there was no significant bleeding. There was no significant past medical, surgical or dental histories. He does not take any regular medications and has no known allergies. There was no recent travel, sick or animal contacts. He does not smoke and only drinks alcohol socially. He denies any form of illicit drug use.\nHistological examination confirmed the rectal polyp as a tubular adenoma. He was well the next day following polypectomy but presented four days later with left upper quadrant pain radiating to the back and minor rectal bleeding. Six days following colonoscopy and polypectomy he developed sepsis, supported by leukocytosis of 18.1 (range 4-11), CRP of 234 (normal range <5) and ESR of 55 mm/h (normal 2-14). He was immediately commenced on amoxycillin/clavulanic acid and treated as colitis by his general practitioner. Due to a lack of clinical improvement despite oral antibiotic, a contrast CT scan of his abdomen and pelvis was performed. There were CT evidence of distal splenic artery aneurysm with complete obliteration distally as well as splenomegaly with most of the spleen occupied by multiple cystic lesions ().\nBlood cultures and CT-guided splenic abscess aspiration were collected immediately, followed by administration of intravenous antibiotics (ceftriaxone and metronidazole). Multiple sets of blood cultures and splenic aspirate cultures were positive for Enterococcus faecalis on day one, sensitive to amoxycillin and vancomycin. Transthoracic echocardiogram showed moderately severe aortic regurgitation and an aortic valve vegetation (1.4 × 0.9 cm) situated at the right coronary cusp with localized leaflet prolapse and perforation. Apart from that, no other valvular lesion was detected and both ventricular systolic functions were well-preserved. These findings were confirmed on a subsequent transesophageal echocardiogram.\nOpen splenectomy was performed via a roof-top incision. Macroscopically, the spleen was grossly enlarged with multiple loculations of abscess (). The infected aneurysm of splenic artery was densely adherent to the retroperitoneum and pancreas, rendering its excision challenging. A decision was then made to suture-ligate it proximally. Post-splenectomy recovery was uneventful. Bioprosthetic aortic valve replacement was performed one week after splenectomy. Culture of the native aortic valve was positive for Enterococcus faecalis. Recovery post-cardiac surgery was uncomplicated. He was then discharged on ampicillin 2 g every four hours for a total of six weeks. He remained well and asymptomatic at his follow up eight weeks after discharge. |
A 43-year-old woman presented to the emergency department with a two-day history of left shoulder pain. The patient described the shoulder pain as sharp, constant, and non-radiating. She did not have any known past medical or surgical histories, and her only medication was OCP pills. On review of systems, she reported epigastric discomfort without nausea, vomiting, lack of appetite or recent weight changes. On physical exam, the patient was tachycardic with otherwise stable vital signs. Abdominal exam revealed epigastric tenderness with no guarding or rebound. Initial laboratory work is depicted in Table .\nInitial computed tomography (CT) scan of the abdomen revealed a large heterogeneously enhancing mass arising from the anterior wall of the stomach with a small amount of free fluid surrounding the liver and a moderate amount of fluid in the pelvis (Figure ).\nContrast-enhanced magnetic resonance imaging (MRI) of the abdomen demonstrated an hypoenhancing exophytic mass from the anterior wall of the stomach with evidence of small volume internal and adjacent hemorrhage. The mass measured 4 x 7.2 x 8 cm in maximum anteroposterior, transverse, and craniocaudal dimensions (Figure ).\nSubsequently, the patient underwent an exploratory laparotomy, which revealed free blood with a large amount of blood clots occupying the greater curvature between the stomach and spleen. An infarcted, hemorrhagic, ovoid mass was seen between the fundus of the stomach, diaphragm, and spleen, which was separate from spleen, liver, and greater curvature slightly attached to the diaphragm. Removal of intraabdominal mass was done successfully and followed by biopsies taken from the omentum. Patient had an uncomplicated surgery and remained asymptomatic in the following days. Initially, the lesion was thought to be an accessory spleen on gross examination and the vicinity. The pathological exam of the surgical piece revealed an infarcted, hemorrhagic mass composed of cords of degenerated epithelioid cells with vacuolated and occasionally lipid-laden cytoplasm (Figure ). Large, dilated vessels with thrombi within some of them and papillary endothelial hyperplasia were noted. Fibro- and myofibroblastic tissue surrounded the mass. Morphologically the epithelioid cells were reminiscent of hepatocytes, and the provided immunohistochemical stains showed them to be diffusely positive for HerPar-1 (hepatocyte origin), cytokeratin (CK) AE1/AE3 and negative for cluster of differentiation (CD)117. There was focal immunoreactivity for CD31 and CD34 between the cell cords. These findings were most consistent with an infarcted inflammatory HCA associated with the reactive papillary endothelial hyperplasia. |
An eighteen-month-old boy was brought with the complaint of a gradually progressive painless mass in the left eye since 16 months. According to the parents, the child was unable to see with both the eyes since birth. General and systemic examination of the child was normal. Ocular examination revealed phthisical right eye () and a cauliflower like mass arising from the limbus (360 degrees) in the left eye (). The entire anterior surface of the left eye was covered by the mass, obscuring the view of anterior and posterior segments. Engorged, tortuous conjunctival feeder vessels were found surrounding the mass.\nUSG B scan showed unidentifiable phthisical right eye and normal posterior segment of the left eye. CT scan of the orbit showed right eye phthisis () and a lobulated soft tissue mass measuring 27 × 7 mm2 in preseptal region extending from lateral canthus to medial canthus of the left eye abutting the anterior globe and lacrimal gland with loss of intervening fat plane (). The mass was completely covering anterior segment of the left globe.\nAfter considering these findings, the left eye was enucleated under general anaesthesia. Gross examination of the postoperative specimen revealed a 28 × 22 × 2 mm3 solid greyish-white firm mass extending across the entire perilimbal area, cornea, and anterior segment (). The optic nerve stump appeared to be normal ().\nHistopathology of the excised mass revealed tissue lined by keratinized stratified squamous epithelium. The subepithelium was composed of spindle, oval, and stellate cells in fibrocellular stroma (). There was presence of myxoid areas in the stroma. Dilated prominent vascular channels were observed. There were interstitial infiltration and perivascular infiltration by inflammatory cells composed of lymphocytes and plasma cells (). Mitosis and necrosis were absent and optic nerve was free of lesions. The histopathological findings were suggestive of IMT.\nThe postoperative period was uneventful and the child is on regular follow-up with no evidence of recurrence till date. The parents were counselled about rehabilitation of the child and use of prosthetic eye for cosmetic purpose.\nInformed consent was obtained from the parents of the child for use and publication of the photographs and other related materials in scientific journal. |
A 10 year-old girl presented with the complaint of palpitation to a cardiologist. She had normal physical examination and laboratory tests, except tachycardia (heart rate = 130 per minute) and low TSH levels (0.005) with normal T3 (9.46) and T4 (145). She was referred to endocrinologist for possible hyperthyroidism evaluation. The thyroid gland was normal size, with no nodularity. She was diagnosed with possible thyrotoxicosis, but due to the normal physical examination, she underwent thyroid scan to rule out possible thyroiditis, which did not show any uptake in the thyroid gland, while there was an increased uptake in the right ovary (). Pelvic trans-abdominal sonography showed a heterogeneous complex solid mass of 113 × 112 × 100 mm with volume of 670 cc in the right ovary with no ascites. The patient had no complaint of abdominal pain or pelvic pain or abnormal uterine bleeding.\nShe was treated with methimazole 10 mg daily and propranolol 40 mg daily and were candidate for surgery after being euthyroid. The patient was referred to a gynecologist with the possible diagnosis of struma ovarii for further evaluation. She underwent right oophorectomy with the presumption of teratoma combined with thyroid-stimulating hormone (TSH)-suppressive therapy following treatment with I131. Total thyroidectomy was performed to permit evaluation for metastatic disease and monitoring for recurrence by thyroglobulin levels. The pathology report of the ovary mass indicated teratocarcinoma with 60% well-differentiated follicular thyroid carcinoma and 40% well differentiated follicular-variant with tumor necrosis, microscopic capsular invasion and peritumoral lymphovascular invasion, considering stage IC of PTC () and the thyroid gland did not show pathologic features of PTC.\nFurther evaluation with whole body scan with Iodine 123 (I123) showed metastasis to lymph nodes. She had high levels of thyroglobulin and received iodine therapy (150 mCi) twice. In the follow-up whole body scan, there was no trace of iodine uptake and the patient was symptom free.\nThe patient is now under treatment with levothyroxine 0.1 mg daily. Following 8 months after surgery and iodine therapy, she is totally symptom free. |
A 48-year-old woman with no clinical history of interest, and gynaecological history of menarche at 14-years-of-age, regular menstrual cycles with a duration of 5 days every 28 days. Parity of 2-0-0-2. She is currently climacteric with amenorrhoea for 6 months and has not received any hormone replacement therapy. She presented with clinical symptoms of pelvic pain in the right iliac fossa region of 3 months' evolution. Initially, the patient was evaluated for acute appendicitis, tubal pregnancy, and other inflammatory processes. That pain was occasional, dull, not related to movements, and sometimes referred as a puncture. A suprapubic abdominal ultrasound scan showed an enlarged uterine corpus that measured 140 × 65 × 43 mm and, in anteversion, a well-defined hyperechogenic 52 × 32 mm nodular intramural formation was highlighted on the anterior face (), as well as an endometrial polyps in the uterine fundus with a maximum diameter of 20 mm. Uterine adnexa were not seen because of the uterine size. Using Doppler, the uterine wall nodule appeared avascular. With the diagnosis of hypertrophic and myomatous uterine corpus, the patient was submitted to surgery for total hysterectomy and bilateral adnexectomy. Patient was previously evaluated and was considered that the suitable treatment should be one only surgical procedure. For this reason, endometrial polypectomy was not performed previously. The postoperative evolution was without complications and with subsequent remission of the pelvic pain. Patient had endovenous treatment for the pain during three days and after oral treatment for 5 more days. At 1 year follow-up, patient only refers some skin paresthesias and no abdominal pain.\nThe pathological study revealed a hypertrophic uterine corpus of 140 grams and 14 × 7 × 5 cm. Dissection revealed an intramural tumorous development of 52 × 32 mm, which bulged into the endometrial cavity, with a smooth cut surface and somewhat elastic consistency and yellowish coloration (). Microscopically, it was almost entirely comprised of mature adipocytes occasionally defined by fibrous tracts containing capillary vessels (). Peripherally, the lesion was surrounded by a fibrous capsula. By using immunohistochemical techniques, positivity was observed for markers such as vimentine, and focally for actin in the smooth muscle of the fibroconnective tracts around the capillary vessels. On the contrary, other markers such as CD10, CD99, CD34, and HMB45 were negative. The remaining findings were centred on a 20 × 3 mm endometrial polyps implanted in the uterine fundus with a histology characteristic of glands with irregular morphology, some cystic, on a dense stroma rich in thick-walled vessels. The left ovary was 80 × 55 mm, of hard consistency with a yellowish, trabecular surface. Microscopically, it consisted of fibroblasts, partly vacuolar, on a hyaline stroma, edematous and occasionally collagenized, characteristic of ovarian thecomas. |
A thirty-seven-year-old male presented with persistent right should pain after a car accident 2 months earlier. The patient was a nonsmoker with a BMI of 32, a history of dyslipidemia and hypertension, and no prior shoulder problems. Initial physical exam revealed good strength in the flexor pollicis longus, flexor digitorum profundus, and intrinsic muscles of the hand, but the patient had pain upon resisted external rotation and abduction of the shoulder. He had pain with palpation over the bicep tendon and deltoid insertion. MRI revealed a partial thickness supraspinatus tear along with fluid around the biceps tendon. The patient was diagnosed with a partial rotator cuff tear, bicep tendonitis, and a deltoid strain. Surgery was scheduled after he had failed conservative treatment which included a corticosteroid injection and physical therapy for two months without significant improvement. On the day of surgery, the patient was given a preoperative, ultrasound-guided, interscalene nerve block with 25 mL of 0.5% ropivacaine administered with a 21 gauge, 90 mm, echogenic short bevel needle. Next, the patient underwent induction of general endotracheal anesthesia. The patient was placed in the lateral decubitus position with the head in a neutral position, and a boom was used to apply 15 pounds of traction throughout the surgery. Glenohumeral debridement and a subacromial bursectomy were performed arthroscopically, and arthroscopic inspection revealed extensive synovitis along the long head of the biceps tendon. Following the arthroscopic procedure, the weighted traction was released and an open subpectoral long head biceps tenodesis was performed. The total case time was one hour and thirty-eight minutes.\nOne week postoperatively, the patient presented with difficulty flexing the DIP joint of the index finger and the IP joint of his thumb. At three weeks postoperatively, the patient was found to have profound weakness of FPL and FDP and he was diagnosed with AIN palsy of the right hand. An electromyography and nerve conduction study was done seven weeks postoperatively and revealed no major abnormalities in the median nerve proper, suggesting that the insult was primarily isolated to the AIN nerve. At eight weeks postoperatively, the patient's AIN palsy was clinically improving and weakness had decreased. |
A 61-year-old female with medical history of hypertension, diabetes mellitus type 2, and chronic kidney disease stage V was transferred to our institution from an outside hospital for further evaluation and definitive management of a migrated intracardiac stent.\nShe initially presented to the outside facility with progressive dyspnea on exertion, orthopnea, and bilateral lower extremity edema. She was initially diagnosed with acute heart failure and pneumonia and treated with diuretics and antibiotics. Subsequently, a transthoracic echocardiogram was performed, which revealed a foreign body within the right ventricle. On transfer to our facility, a transesophageal echocardiogram revealed a long stent straddling the tricuspid valve from the right atrium with the other end lodged in the trabeculation of the right ventricle with severe tricuspid regurgitation ().\nOn further investigation, we learned that the patient had undergone peripheral endovascular intervention for May-Thurner syndrome with placement of a self-expanding Nitinol Protege (14 mm × 60 mm) stent to the left iliac vein 6 months prior to presentation.\nA percutaneous endovascular approach with a 35- mm Medtronic-Covidien Amplatzer Gooseneck Snare was initially attempted to retrieve the migrated stent. However, the snared proximal segment fractured, leaving behind 2 stent fragments. After ensuring there was no myocardial perforation or pericardial effusion with intracardiac ultrasound, the patient was referred for surgical extraction via median sternotomy with use of cardiopulmonary bypass.\nDuring the operative procedure, the stent was found to be densely adherent to the tricuspid leaflets and the subvalvular apparatus, with majority of the primary chords to the anterior and posterior leaflets ruptured (). After successful extraction of the stent and native tricuspid valve, she underwent valve replacement with a 29-mm Carpentier-Edwards bioprosthetic valve. Her postoperative course was complicated by hemopericardium secondary to anticoagulation resulting in cardiac tamponade that was drained percutaneously, and small thromboembolic cerebellar stroke from atrial fibrillation. She was discharged to an inpatient rehabilitation facility and did well on 8-month follow-up. |
A 70-year-old female presented to our hospital with a periumbilical abdominal lump associated with intermittent colicky abdominal pain. There was a rapid increase in size of the lump in the past few weeks. On evaluation, the abdominal examination revealed a well-defined, freely mobile, firm, intra-abdominal lump approximately 20 × 15 cm in size, occupying the left hypogastrium and paraumbilical region. Ultrasonography (USG) followed by contrast enhanced computed tomography (CECT) scan revealed a heterogeneous enhancing mass lesion, arising from the mesentery of small bowel, without any obvious involvement of the bowel (\n).\nExploratory laparotomy revealed a mobile, lobulated, highly vascular mass of variegated consistency in the mesoileum, about two feet proximal to ileocecal junction. The adjoining bowel was pushed to the periphery of the mass, without evidence of any gross involvement. A few other smaller nodules were present in the adjacent mesentery, near the mass, mimicking enlarged mesenteric lymph nodes without any evidence of involvement of other adjacent organs. En bloc resection of the mass along the mesenteric nodules and the adjacent small bowel was done followed by an end to end anastomosis (\n). Multiple other smaller nodules in the adjacent mesentery of the small bowel were left behind assuming them to be enlarged lymph nodes (\n). Histopathological examination of the tumor revealed long interlacing fascicles of malignant spindle cells with plump to cigar shaped blunt end nuclei with granular chromatin, moderate amount of eosinophilic granular cytoplasm, and ill-defined cytoplasmic border (\nand\n). Average mitotic rate was more than 5/50 high-power fields with large areas of necrosis, hemorrhage, and cystic degeneration. The overlying bowel wall was free from the tumor (\n). The nodule which was sent separately assuming it to be lymph nodes was having same features of malignant eGIST without any lymphoid tissue. Immunohistochemical examination revealed the tumor to be strongly positive for cluster differentiation (CD)117, whereas negative for SMA (smooth muscle actin) and S-100, to rule out tumors with neural differentiation (\n). Based on these findings a diagnosis of primary malignant eGIST of the mesentery of the high-risk category was made.\nFluorodeoxyglucose positron emission tomography (FDG-PET) scan was done in the postoperative period to look for other sites of metastasis. There was increased activity in the tumor bed area, probably because of the residual nodules, left in the small bowel mesentery, which were intraoperatively assumed to be mesenteric lymph nodes (\n). As the patient was elderly and frail, so was planned for adjuvant treatment. The patient was discharged on postoperative day 7 with imatinib mesylate (400 mg/day; Glivec, Novartis Pharma AG), as adjuvant chemotherapy and advice to follow-up at regular intervals. After 1 year of surgery, the patient is having a stable disease both clinically and on CECT scan. |
A 67-year-old female, with a typical presentation of carpal tunnel syndrome in the right hand, presented initially with numbness in digit IV and V a year later. A few months later, she developed numbness in all five fingers on the left side. Furthermore, the patient mentioned that she had had a burning sensation in both feet for a few years. Another few months later she complained of weakness in all four limbs and was sent for a neurological consultation with electromyography. Clinical examination revealed a Hoffmann-Trömner reflex on the left side, mild loss of strength in both hands, loss of vibratory sense and hypoesthesia in the distal end of all four limbs and loss of proprioception in both legs. The electromyography showed disturbed sensorimotor signals in the left hand and mildly disturbed sensorimotor signals in the right hand. The disturbed electromyography was attributed to carpal tunnel syndrome, which was presumably less severe on the right side due to treatment with long acting corticosteroid injections. The paresthesia in digit IV, digit V and both feet, however, could not be explained with the diagnosis of carpal tunnel syndrome. Therefore, magnetic resonance imaging (MRI) of the cervical spine was performed. Imaging showed an extra-dural soft tissue mass posterior to the odontoid process of the axis. The mass extruded through the transverse ligament of the atlas with severe compression of the myelum and myelomalacia at the level of C1 (Figures , and ).\nA plain radiograph of the cervical spine was performed to check for atlanto-axial instability. The radiograph during flexion shows a slightly widened atlantodental interval, measuring 4 mm (normal value: <3 mm) (Figure and ).\nPre-operative imaging of the cervical spine also revealed an anatomical variant of the craniocervical junction, atlanto-occipital assimilation of the massa lateralis bilaterally (Figure ).\nThe patient was treated with laminectomy of C1 to decompress the spinal canal followed by posterior fixation of C1-C2 for stability. |
A 40 year old male who was known to have type I (insulin-dependent) diabetes mellitus, presented with complaints of productive cough, breathlessness, fever, weight loss, and hemoptysis for three years. His previous chest radiograph revealed left upper lobe cavitary infiltrate []. His routine investigation revealed uncontrolled blood sugar and negative sputum smear for acid-fast bacilli at that time. Patient had received two years of antituberculosis treatment along with insulin from a private practitioner, though his sputum did not show any acid-fast bacilli. Despite adequate antitubercular treatment, he deteriorated clinically as well radiologically.\nOn admission, general examination revealed grade III clubbing of fingers and toes. Examination of respiratory system revealed bronchial breath sound over left mammary area and coarse crepts in bilateral suprascapular region. Review of his serial chest radiograph revealed progressive increase in cavitation with the evolution of fungus ball and progression of disease on right side []. His hematological and biochemical investigations were with in normal limits except uncontrolled blood sugar (fasting blood sugar: 202mg/dl and post prandial: 368mg/dL). Enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus was negative. Multiple sputum smears revealed no bacteria and acid-fast bacilli. The culture by BACTEC did not show any mycobacteria. The sputum on fungal culture grew Aspergillus fumigatus. A CT scan of the chest revealed a crescent-shaped lucency (air crescent sign) with in the area of consolidation in the left upper lobe and right middle lobe [Figures and ]. Fiberoptic bronchoscopy showed purulent secretions coming from both upper lobe bronchi and left lower lobe bronchi. Bronchoalveolar lavage smears did not reveal any bacteria or acid-fast bacilli but the growth of Aspergillus fumigatus. Thus, he was diagnosed as a case of chronic necrotizing pulmonary aspergillosis with diabetes mellitus.\nHe was given insulin for glycemic control and oral itraconazole 200mg twice daily. After one month of itraconazole therapy, the patient started showing clinical improvement, became afebrile, gained weight, and the volume and the purulence of sputum also reduced considerably. |
A 32-year-old female with a history of hyperthyroidism status after radiation resulting in hypothyroidism and no history of seizures presented with an acute onset of behavioral changes and witnessed seizure activity. Family history is remarkable for thyroid disease in multiple relatives, but negative for seizure or psychiatric disorders. Behavioral changes included uncontrolled laughter, screaming, signs of agitation, spitting on the floor, complete lack of appetite, and speaking in a British accent. A further history revealed that the patient is of Caucasian descent, was born in Germany, moved to the United States when she was a baby, and has no ties to Britain.\nShe had two seizures both involving tongue biting and postictal confusion with combative behavior. Her workups for seizures, including but not limited to head CT, urine drug screen, and electrolyte levels, were all within normal limits. She, as well as her family, refused MRI and subsequently was discharged on levetiracetam for new onset seizures. On the fourth day of illness (DOI), the patient was admitted to a local community hospital with continued behavioral changes, where an MRI and lumbar puncture (LP) were found to be unremarkable, with a WBC count of 1 cell per mm3. Other CSF parameters include a RBC count of 10 cells per mm3, glucose of 53 mg per dL, and protein of 26 mg per dL. She was subsequently transferred to our hospital care on the fourteenth day of illness because of persisting and worsening behavioral changes. Upon revisiting the initially unremarkable impression of the MRI, abnormal T2 flair hyperintensity in the mesial temporal lobes was noted, with left lobe hyperintensity greater than that of the right lobe (). These features raised suspicion for limbic encephalitis.\nTiters for serum anti-NMDAR and paraneoplastic antibody panel were sent on clinical suspicion. Video EEG () showed frequent focal onset electrographic seizures from the left frontocentral and left frontotemporal region. Some of these electroclinical seizures showed delta brushes (). With strong clinical suspicion for an anti-NMDAR or paraneoplastic antibody related encephalitis, before even receiving antibody titer results, the patient was started on five-day IV steroids on the fourteenth DOI, and IVIG course started on the fifteenth DOI.\nAt this point, multiple differential diagnoses were being considered including autoimmune encephalitides. During hospital admission, the patient continued to remain afebrile. Repeat LP showed lymphocytic pleocytosis, with a quantitative value of 54 cells per mm3 with 98% lymphocytes, after which she was started on empiric acyclovir until HSV PCR was later confirmed to be negative. All other CSF findings were insignificant, with a normal protein level of 16.8 mg per dL, no RBCs, and an elevated glucose level of 95 mg per dL. She continued to have seizures requiring Lacosamide followed by an addition of Lamotrigine. On the fifth day of IVIG course and twentieth DOI, NMDAR antibody was found to be positive in the serum. CT of the chest, abdomen, and pelvis showed no evidence for neoplasms and a transvaginal ultrasound was negative for ovarian teratoma. CSF was positive for NMDAR antibody with a titer of 1:64. After completion of IV steroids and IVIG, there was no significant clinical improvement. She was started on rituximab on DOI 27 for a total of 4 weeks given weekly, with subsequent clinical improvement in addition to no clinical seizures on EEG and resolution of her new onset British accent.\nThe patient continued to have subclinical focal seizures, catatonia, and orofacial dyskinesias with subsequent gradual improvement in her behavior at discharge. When seen for follow-up as an outpatient, she still was found to have some residual memory and cognitive processing deficits. The patient continues to follow up in the hospital for rituximab infusions which is controlling her anti-NMDAR encephalitis and she has now returned back to baseline health. |
A 55-year-old woman with a medical history of chronic viral hepatitis presented to our clinic after sudden onset of headache with vomiting. We did not find any neurologic deficit and her mentality was drowsy. Her CT scan showed a diffuse SAH and mild hydrocephalus (). CTA confirmed an upper basilar abnormality (). Cerebral angiography obtained two hours later failed to disclose a vascular abnormality (). The patient received an external ventricular drain (EVD) for symptomatic hydrocephalus on posthemorrhage day 2. The patient's clinical condition improved and her level of consciousness improved to alert immediate after EVD. On the second day after EVD, her mentality suddenly deteriorated to semicoma. Immediate CT scan showed an increased amount of SAH, which suggested rebleeding. A repeat CTA after rebleeding did not show an abnormal vascular lesion. However, cerebral angiography at the same time confirmed a small cylinder like contrast stagnation from the upper basilar artery at late arterial phase, indicating pseudoaneurysm formation (). We inserted two Solitaire stents (4 × 20 mm, ev3, Irvine, CA, USA) for flow diversion and anticipating thrombus formation of the pseudoaneurysm. The patient's clinical course was uneventful, however, one-month follow up CTA still showed a saccular lesion from the upper basilar artery. Therefore, we decided to perform DSA and intervention. DSA still showed contrast stagnation at late arterial phase on the upper basilar artery and superselective microcatheter angiography confirmed a pseudoaneurysm arising from a small perforating artery of the superior cerebellar artery (SCA) (). Due to concern about cerebellar infarction, we could not sacrifice the SCA. To facilitate thrombus formation of the pseudoaneurysm, we performed repeated temporary balloon occlusion using a Sceptor XC balloon (4 × 10 mm, Microvention, Tustin, CA, USA) and achieved decreased contrast inflow to the pseudoaneurysm (). On follow-up at 11 months, the patient had no neurologic deficit, and she was able to live independently. Follow up DSA performed 11 months after hemorrhage confirmed no contrast stagnation and abnormal vascular lesion (). |
A 33-year-old man was referred to the eye clinic with a chief complaint of progressively decreasing vision in his right eye. The patient reported that he first noted the change in vision 3 months previously, without any other remarkable features. He also reported that when he moved his right eye, he felt a sense of discomfort and aching pain. One month later, a small palpable, round, movable, nontender bump was found in his left chest wall, which was getting bigger in size from 2×2 cm2 to 3×3 cm2 in recent months. Excluding the change in vision and the bump in the chest wall, further detailed ophthalmic examination and general physical assessment showed no significant abnormalities. He had no history of trauma or any other underlying systemic and ophthalmologic disease. He had a smoking history of 10 pack-years and a history of alcohol use. A cranial and nasopharynx computed tomography (CT) showed a right solid tissue orbital mass in the retrobulbar space, pressing on the optic nerve; there was no involvement of the bony orbital wall, and the brain was normal. The initial clinical impression was that of orbital displacement, and it was difficult to differentiate the benign tumors (capillary hemangioma, optic nerve glioma, cavernous hemangioma, and meningioma), inflammatory pseudotumor, or malignant tumor, all of which show a similar unclear shadow on plain scan. Before further investigative examinations were ordered, the ophthalmologist recommended the patient to a surgical clinic for evaluation of left chest wall neoplasm to determine if it was a primary malignancy or metastasis. A subcutaneous resection was performed, and the histopathology examination of the excision showed poorly differentiated metastatic adenocarcinoma. Furthermore, immunohistochemical studies showed negativity of TTF-, NapsinA, S-100, HMB45, and Melan-A and positivity of CK, CK7, and Vim. Four weeks after his first doctor visit, the patient came to our radiation oncology clinic, complaining of decreasing visual acuity, proptosis of his right eye and aching pain without lid ptosis or other relevant focal neurological deficits in contrast to the left eye, and onset of multiple aches from head to back. The laboratory tests showed cytokeratin fragment antiogen 21-1: 181.5 ng/mL (normal, 0.0–3.3) and neuron-specific enolase: 72.97 ng/mL (normal, 0–17). On the basis of the findings, his left chest wall neoplasm was regarded as a metastasis from an unknown primary source, and there was a large suspicion that the retrobulbar mass was also a metastasis. An investigative systemic oncologic workup was initiated. In the chest CT, an enhancing mass () was found in the lower lobe of the right lung, with right hilum and mediastinal lymph node metastases, and enhancements in multifocal muscular neoplasms (), suggestive of metastatic involvement. A CT-guided percutaneous fine-needle aspiration biopsy (FNAB) of the lung mass was performed, and it provided a histopathological diagnosis of adenocarcinoma, which is consistent with the known subcutaneous tumor (). By this time, visual acuity and pain in the right eye progressed in severity. A spontaneous subcapsular hemorrhage was observed in the right eye. A diagnostic magnetic resonance imaging (MRI) of the nasopharynx was repeated, and it demonstrated multifocal skeletal muscular metastases and a right retro bulbar extraconal mass (20×15×20 mm3) that was increasing in size, pushing the eyeball forward (), and two enhancing nodules in brain (). The experienced medical radiologist reported that all the lesions showed similar patterns on different series, highly supportive for the metastatic involvements. A bone scan showed no abnormal values. After a multidisciplinary discussion with the physicians (including oncologists, radiologists, and surgeons), we did not perform an invasive biopsy because of the high risk of complications, which would further affect his potential vision because of the close anatomical relationships, as well as the patient’s poor condition and refusal. According to the clinical, radiological, and histopathological evidence, our patient was considered to have stage IV (T2N2M1) non-small-cell lung cancer (NSCLC) with brain, orbital, subcutaneous, and multifocal skeletal metastases. Once the diagnosis was certain, the patient was referred to the special ward for the palliative chemoradiotherapy. Tyrosine kinase inhibitors (TKIs) were denied as a first-line treatment because the histopathology report from our biopsy was negative for epidermal growth factor receptor (EGFR). He received first-line chemotherapy with two cycles of pemetrexed and cisplatin; in addition, palliative radiotherapy, 50 Gy in 25 fractions to relieve the pain and the decreasing visual acuity, was performed on the retrobulbar mass. In a repeated MRI scan, the orbital metastasis showed a significant response to the treatment () and two more lesions were seen in the brain (). Because of the progression of the neoplasm to the brain, radiation to the whole brain was initiated, 50 Gy in 25 fractions. During the second cycle of chemotherapy, the skeletal muscular metastases enlarged rapidly, with pain escalating out of medical control (). Being judged as a process of progression according to the Response Evaluation Criteria In Solid Tumors (RECIST), our patient underwent second-line chemotherapy with docetaxel and cisplatin. Additionally, radiation to the skeletal muscle metastases was initiated. When the treatment was completed, he reported relief from the pain in the metastatic sites and improvement in visual acuity (). No relevant side effects of radiotherapy were found by CT scan and physical examinations. The patient was subsequently lost to follow-up.\nThis study was approved by the Institutional Review Board of Radiation Oncology, Shandong Cancer Hospital and Institute. The patient provided written informed consent. |
A 60-year-old Caucasian woman presented with an acute onset right upper abdominal pain and a rapidly enlarging upper abdominal mass. She had a previous history of resected early-stage melanoma. Clinical examination revealed tender hepatomegaly, and a contrasted computed tomography (CT) scan of the thorax, abdomen, and pelvis showed a large solitary heterogeneous mass in segment V1 of the liver with evidence of intratumoural bleeding (). She proceeded to laparotomy and at operation a large liver mass was evident and the tumour was found to be grossly adherent to a number of intraabdominal organs with evidence of tumour perforation and intraabdominal spillage. A lateral segmentectomy of the liver along with cholecystectomy was performed and she made an uneventful postoperative recovery.\nThe tumour measured 15 × 11 × 20 cm and cut section revealed massive necrosis and haemorrhage consistent with the preoperative CT scan (). The tumour extended to the liver capsule and to within 2 cm of the margin of excision. There was extensive vascular and perineural invasion. Histological examination of the tumour showed a malignant biphasic neoplasm consisting of a moderately differentiated adenocarcinoma intermingled with a malignant mesenchymal component (). The latter consisted of pleomorphic spindle cells arranged in sheets intermixed with bizarre multinucleate osteoclast like giant cells. Reams of osteoid surrounded by osteoblasts were also characteristically seen. The sarcomatous component made up to 70% of the whole tumour. The adenocarcinoma cells showed apparent mucus production as demonstrated by PAS and alcian blue staining. They neither displayed trabecular arrangement nor bile production, which are characteristics of a hepatocellular carcinoma. Immunohistochemical study of carcinomatous component revealed positive staining for Cam5.2, EMA, AE1/AE3, CK7, CK19, and CEA and negative staining for HePAR1. This immunoprofile was in keeping with cholangiocarcinoma. The sarcomatous component was positive for vimentin but negative for epithelial markers. S100, HMB45, MELAN A, desmin, inhibin, and CD117 were negative in both components. Both MiB1 and p 53 showed strong positivity in both epithelial and sarcomatous component (>80%). Electron microscopy showed presence of basement membrane and microvilli in the tumour cells which excluded their hepatocyte origin. It also demonstrated presence of tight junctions and desmosomes in the sarcomatous component indicating their epithelial origin. Based on histological, immunohistochemical and electron microscopy findings, a diagnosis of sarcomatoid cholangiocarcinoma was made and the patient was referred for consideration of adjuvant chemotherapy.\nA postoperative contrasted CT scan at 5 weeks confirmed rapid onset disease recurrence with multiple peritoneal deposits and multifocal liver metastasis (). Patient was started on gemcitabine (1000 mg per m2 on days 1 and 8) and cisplatin (60 mg per m2 on day 1) chemotherapy in a 21-day cycle which she tolerated well with minimal side effects. Patient received a total of six cycles of chemotherapy and a restaging CT scan at the end of 6 cycles confirmed partial response (). Apart from grade 1 nausea that settled with antisickness medications, the patient tolerated chemotherapy very well. The patient reported improvement in appetite, weight gain, and resolution of abdominal swelling during chemotherapy. Following completion of treatment, she was started on clinical follow-up. A reassessment CT scan after 3 months of follow up showed further marginal disease size reduction. She continued under regular clinical follow-up until 10 months later; she developed further disease progression was rechallenged with gemcitabine and cisplatin at the same doses. A reassessment CT scan after chemotherapy showed partial response. She remains well and is now 29 months post initial presentation. |
A 39-year-old man presented at our hospital with a thyroid nodule in the neck region, which the mass had been detected during a routine medical check-up. FNA were performed twice prior to the visit to our hospital, but the results of the cytological evaluations at both instances were atypia of undetermined significance (AUS). The patient had no clinical symptoms and the laboratory results were in normal range. Ultrasonography, performed at our hospital, revealed the presence of a relatively well circumscribed mass (2.2 cm in diameter) with microcalcifications on the right lobe of the thyroid gland (Fig. a). Additionally, another FNA conducted at our hospital. The cytologic evaluation showed some follicular cells with enlarged nuclei and pale chromatin pattern. However, the cytologic findings were not sufficient to diagnose a specific tumor, thereafter we diagnosed the cytologic specimen as AUS (Fig. b). Subsequently, the patient underwent right hemithyroidectomy for diagnostic and treatment purposes.\nThe results of the cytology could not confirm whether the mass was benign or malignant. Hence, histologic evaluation of a frozen section using hematoxylin-eosin (H&E) staining was performed. A relatively well-circumscribed mass comprising cells that were mostly arranged in a trabecular or solid sheet-like pattern and intersected by homogenous eosinophilic material was noted (Fig. ). The tumor cells consisted of amphophilic cytoplasm and round to oval-shaped nuclei with condensed chromatin and indistinct nucleoli (Fig. b). The eosinophilic material was suspected to be amyloid deposition and the mass was diagnosed as a medullary thyroid carcinoma. Neck lymph node dissection was additionally performed due to the results of the frozen biopsy. And the patient has been warned that if the results of permanent biopsy was confirmed to medullary thyroid carcinoma as of the frozen biopsy, total thyroidectomy is necessary.\nSubsequently, a permanent section obtained from the patient was evaluated. The tumor showed well margination with thin fibrous capsule (Fig. a and b). The basic growth pattern and morphology of the tumor cells were similar to those seen in the frozen section (Fig. c). Additionally, follicular growth pattern was identified in the permanent section (Fig. e). Also, infarction-like necrotic area and hemorrhage were recognized (Fig. d). Congo Red staining of the homogenous eosinophilic material failed to show the characteristic apple-green birefringence under polarized light microscopy (Fig. a). And blue staining of the homogenous material using Masson’s trichrome staining method confirmed that the homogenous eosinophilic material was fibrosis (Fig. b). Immunohistochemical staining showed diffuse immunopositivity for thyroglobulin and thyroid transcription factor-1 (TTF-1) (Fig. c and d, respectively). Conversely, no staining for synaptophysin or calcitonin, known to be expressed in medullary thyroid carcinoma, was observed (Fig. e and f, respectively). Knowing the patient’s history of multiple FNA procedures during the diagnosis, we suspected that the fibrosis, hemorrhage and necrosis was due to the injury from the FNA. Additionally, there was a needle tract-like structure in low-power field (Fig. a, arrow). Therefore, based on above findings the mass was diagnosed as follicular adenoma with extensive fibrosis. The patient is alive without recurrence or metastasis, after 3 years of follow up. |
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