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In August 2010, a 57-year-old Chinese male presented with epistaxis and decreased hearing for 1 month. No additional symptoms, such as a neck mass, nasal obstruction, headache, diplopia or other cranial nerve palsies, were noted. The patient had no history of previous or synchronous tumours or any family history of cancer. Nasopharyngoscopy revealed a large exophytic tumour that was covered by smooth mucosa, which grew from the right posterolateral nasopharyngeal wall in the right posterior naris. Magnetic resonance imaging (MRI) scans of the nasopharynx and neck using gadolinium enhancement demonstrated a 2.0 × 1.5 × 2.0 cm well enhanced mass over the right posterior nasopharynx with right retropharyngeal node enlargement. The tumour extended across the right parapharyngeal space and infiltrated into the medial pterygoid muscle. In addition, skull base erosion was detected with right alar lamina involvement (Fig. ). Cervical lymph node metastasis was not observed. Hematologic, hepatic and renal function tests revealed no abnormalities. The patients underwent chest and abdomen computed tomography (CT) as well as a bone scintigram, and no distant metastasis was found. A biopsy of the nasopharynx was performed.\nIn the biopsy specimen, normal salivary tissue was not present. The tumours were ill demarcated without encapsulation. Tumour cells were arranged in nests and nodules. Two morphologic patterns of the tumour cells were observed. Some small round cells exhibited dark nuclei and scant cytoplasm. Other large cells contained round to oval pale nuclei and eosinophilic to amphophilic cytoplasm. In the central region of the tumour cell nests, large cells displayed a solid growth pattern. Small dark cells were clustered at the periphery of the tumour cell nests and appeared palisaded. Prominent nucleoli and mitosis can be observed, and an average of three mitotic figures were observed per 10 high-power fields (original magnification × 400).\nIn the immunohistochemical analysis, the tumour cells were immunoreactive with P63, vimentin, and cytokeratin (CK7 and CK14) antibodies and focally immunoreactive with a calponin antibody. This case of BCAC was not positive for smooth muscle actin or CD117. The proliferative index as demonstrated by Ki-67 was approximately 10%. Based on the immunohistochemistry results and the pathological findings, which included tumour islands with solid proliferation, basaloid-like cells containing large pale and small dark cells, an infiltrative margin, cellular and nuclear pleomorphism, and prominent mitosis, the patient was diagnosed with a solid-type minor salivary gland BCAC (Fig. ).\nBased on the 2002 American Joint Committee on Cancer (AJCC) Tumor, Node, Metastasis (TNM) staging system [], the tumour was classified as stage III (T3N0M0).\nIn our case, the patient received intensity-modulated radiation therapy (IMRT) with 6 MV X-rays. The delineation of the gross tumour volume (GTV) was based on the primary tumour volume determined from the physical and imaging examinations. The clinical target volume (CTV) was defined as the whole nasopharyngeal cavity, the clivus, the skull base, the pterygoid plates, the parapharyngeal space, the sphenoid sinus, the posterior one-third of the nasal cavity, the maxillary sinus, and the drainage of the upper neck (levels II, III, and Va. A total dose of 70.4 Gy/32 F/6.2 W was administered based on the planning target volume (PTVg) (GTV with 0.5 cm margin). The PTV60 was defined as 60 Gy/30 F (CTV with 0.5 cm margin) (Fig. ). After radiotherapy, MRI and nasopharyngoscopy revealed complete disappearance of the tumour (Fig. ). The patient was followed up every 3 months for the first 2 years, every 6 months for another 3 years, and then every 12 months. A follow-up at 72 months did not detect any evidence of disease recurrence. The patient developed moderate mucositis as an acute adverse event. However, he did not exhibit any grade 3/4 late adverse events, such as xerostomia, dysgeusia, or hearing impairment. |
A 76-year-old man was referred to our hospital for having lower back pain for 5 months, which was suspected to be L2/3 spondylitis on magnetic resonance imaging (MRI). He had previously undergone cervical laminoplasty for ossification of the posterior longitudinal ligament and diffuse idiopathic skeletal hyperostosis 19 years earlier. He had a history of hypertension and diabetes. Although a history of BCG vaccination was unknown, he had no previous history of tuberculosis infection. He had also undergone transurethral resection of a bladder tumor (TUR-Bt) and had been treated with intravesical mitomycin C (MMC) for the bladder cancer 1.5 years earlier. Four months later, he again underwent TUR-Bt, received intravesical MMC, and started BCG therapy for the recurrence of bladder cancer. After the sixth course of intravesical BCG therapy, he was aware of systemic weakness and loss of appetite, and was unable to walk. He appeared to have septic shock and therefore was treated in the intensive care unit. In spite of systemic analyses, the source of his infection and the causative bacteria could not be identified. He was subsequently diagnosed with hypercytokinemia caused by BCG therapy. He showed clinical improvement without the administration of antituberculosis drugs, and was discharged 9 months before he came to our hospital.\nExcept for a temperature of 37.2 °C, his vital signs were within normal limits. Although there were no motor and sensory disturbances in the legs, he was unable to walk owing to lower back pain. Physical examination demonstrated vertebral tenderness at the L2/3 level. Laboratory analysis demonstrated a normal white blood cell (WBC) count of 6300 /μL, a high erythrocyte sedimentation rate (ESR) of 53 mm/h, and a high C-reactive protein (CRP) level of 2.7 mg/dL. There were no other abnormal laboratory findings regarding anemic changes, kidney function, or liver function. The patient did not receive tuberculin skin testing.\nThere were no notable findings on electrocardiogram or chest X-ray. X-ray of the lumbar spine displayed collapsed endplates of L2/3. Sagittal T1-weighted MRI displayed a decreased signal in the L2/3 disc and the vertebral bodies (Fig. ). Sagittal T2-weighted MRI displayed an increased signal in the L2/3 disc and fluid collection in the anterior part of the vertebral bodies (Fig. ). Axial T2-weighted MRI displayed an increased signal around the posterior area of the vertebral bodies, which extended into the left epidural space and reached the peripheral muscle tissue and the area near the aorta (Fig. ).\nOn the second day of hospitalization, the patient underwent an L2/3 disc biopsy, but the general bacteria culture was negative, and the smear was negative for Ziehl-Neelsen staining. The patient’s blood culture was negative, and the result of T-SPOT.TB® (T-SPOT), which is a type of interferon-gamma release assay (IGRA), were also negative; the number of spots for both 6-kDa early secretory antigenic target (ESAT-6) and 10-kDa culture filtrate antigen (CFP-10) was 5 or less. The causative bacteria remained unidentified and therefore he was started on empirical therapy with intravenous ceftriaxone. On the fifth day of hospitalization, a plain computed tomography scan, which was performed for systemic examination, showed soft tissue development in the adjacent abdominal aorta at the L3 level (Fig. ), which was suspected to be an infectious aortic aneurysm. A 2-stage operation was planned for the spondylitis with adjacent infectious aortic aneurysm, to prevent rupture of the infectious aortic aneurysm, obtain spine stabilization, drain the abscess and make a diagnosis. On the tenth day in hospital, because the risk of rupture was considered to be low, the patient underwent an L2/3 laminectomy followed by posterior fixation using percutaneous pedicle screws at T12, L1, L4, and L5 as the first stage. Specimen cultures of the lumbar vertebrae, yellow ligament, necrotic tissue, etc., were negative for general bacteria, specimen smears were also negative for Ziehl-Neelsen staining, and there were no pathological findings of caseating granuloma or necrosis. On the fiftieth day of hospitalization, because the infection had been controlled but the inflammatory response was sustained, he underwent replacement of the aneurysm with a synthetic graft by vascular surgeons, and lesion curettage and L2/3 anterior interbody fusion by iliac bone transplantation as the second stage. Spinal surgery was performed in the transabdominal approach owing to the risk of rupture. On pathological analyses, the L2/3 intervertebral disc, vertebral bone, and tissue surrounding the vertebral bone and aorta showed caseating granuloma and necrosis with multinucleated giant cells and epithelioid cells upon hematoxylin-eosin staining, and positive bacilli upon Ziehl-Neelsen staining. The tuberculosis-polymerase chain reaction (Tb-PCR) result of the tissue was also positive, using COBAS® TaqMan® MTB Test, which is a real-time PCR system targeting the 16S rRNA gene region of Mycobacterium tuberculosis complex DNA. Owing to the patient’s history of BCG therapy, negative T-SPOT, pathological findings, and positive Tb-PCR, the pathogenic bacteria of the spondylitis was considered to be BCG. We then started multidrug therapy with antituberculosis drugs, including isoniazid (INH), rifampin (RFP), and ethambutol (EB), because BCG is typically resistant to pyrazinamide (PZA). PCR-based genomic deletion analysis was performed using the specimens to distinguish BCG from the other M. tuberculosis complexes. Specifically, multiplex PCR was performed utilizing region of difference 1 (RD1), which is present in the DNA of other M. tuberculosis complexes but is deleted in the DNA of BCG []. Primers ET1, ET2 and ET3 bind and amplify a 190-bp region in BCG, whereas a 160-bp region is amplified in the other M. tuberculosis complexes, as observed by electrophoresis on an agarose gel. A clinical isolate sample from our patient was identified as BCG with a deletion in RD1 (Fig. ). Specimen cultures from the first-stage and second-stage operation were later identified as members of the M. tuberculosis complex using a mycobacteria growth indicator tube. After therapeutic intervention, the patient’s WBC count, ESR level, and CRP level were improved and MRI displayed no signs of active infection in the spine, epidural space, peripheral muscle tissue, or aorta. On the ninetieth day after hospitalization, the patient was discharged from our hospital and transferred to a different hospital for physical rehabilitation. |
The patient was a 36-year-old male, who was admitted to the hospital due to persistent abdominal pain. This patient had no remarkable medical history. Starting in July 2015, the patient experienced repeated episodes of unexplained upper abdominal pain and experienced no other accompanying symptoms. After review of the patient’s past medical records, multiple laboratory tests at other hospitals indicated elevated levels of pancreatic enzymes ranging from 213-341 IU/L (less than 3-fold of the upper limit of the normal range [35-140 IU/L]) combined with slight exudation around the pancreas, and gallstones. His condition improved when he was treated for pancreatitis. As pancreatitis caused by gallstones could not be excluded, cholecystectomy was performed. After surgery, his symptoms repeatedly relapsed. In October 2015, right eyelid oedema occurred intermittently, which was not treated. In April 2016, the right eye became increasingly swollen until protrusion of the eyeball occurred. Orbital computed tomography (CT) and magnetic resonance imaging (MRI) were performed at other hospitals and indicated space-occupying lesions, but no further treatment was given. In May 2016, the patient again experienced abdominal pain and was diagnosed at the Department of Gastroenterology at a hospital specializing in the standardization of residency training. Laboratory tests showed slightly elevated liver and pancreatic enzymes and normal routine blood and autoantibody test results. Levels of carbohydrate antigen 19-9, carcinoembryonic antigen (CEA), and other tumour markers were also normal. A contrast-enhanced abdominal CT scan indicated a blur gap between fat around the pancreatic tail and between the spleen and stomach. A circle-like cystic low-density shadow was observed in the gap between the stomach and pancreas without clear enhancement. The pancreatic duct and the bile ducts inside and outside the liver were also slightly dilated (Figure ). The possibility of pancreatitis was considered. However, the patient’s abdominal pain relapsed repeatedly, and the evidence for “pancreatitis” was not sufficient. His medical history and related tests excluded the possibility of pancreatitis caused by drugs, metabolism, and autoimmunity. Therefore, endoscopic ultrasound was performed, the results of which indicated an uneven echo of the pancreatic tail and body. Echo-free fluid exudation was observed around the pancreatic tail, and a hypoechoic, solid space-occupying lesion was visible at the uncinate process with uneven echo. Scattered hyperechoic areas were also observed. A pancreatic head space-occupying lesion and degeneration due to pancreatitis were considered. Needle aspiration biopsy of the space-occupying lesion of the pancreatic uncinate process was performed using an endoscopic ultrasound. The pathologic analysis revealed local tissue necrosis, and the patient exhibited obstructive jaundice after the operation. Endoscopic retrograde cholangiopancreatography was performed to place a metallic stent, and dark-red tissue discharge was observed during the procedure. The tissue was sent for pathological examination, but no tumour cells were detected. The patient’s symptoms were treated, and he was discharged after they improved.\nIn July 2016, the patient’s abdominal pain recurred. Positron emission tomography CT (PET-CT) indicated low-density lesions resembling lumps in the pancreatic tail. The shadow had a ring shape and glucose metabolism was abnormally high. Therefore, the lesion was considered an inflammatory pseudotumour. The pancreatic head region exhibited shadowy patches, glucose metabolism was slightly changed, and another CT performed using the same machine showed a space-occupying lesion in the corresponding region as well as an abnormal density change. Thus, the lesion was considered a benign lesion (possibly an inflammatory lesion). Glucose metabolism was abnormally high in the capsule area that formed a cord outside the right orbit, and a benign lesion was diagnosed (possibly an inflammatory pseudotumour). The aetiology of the lesion in this patient remained unclear, and the possibility of a haematologic malignancy was considered. A bone marrow biopsy was performed, but obvious abnormalities were not detected. Therefore, percutaneous needle aspiration biopsy of the hypoechoic area of the pancreatic tail was conducted under ultrasonography guidance. The result showed heterotypic cells with a flaky appearance arranged in cord-like shape indicating infiltrate growth (Figure ), and the pathologic analysis indicated a malignant tumour. In addition, the morphology suggested a poorly differentiated adenocarcinoma. However, considering the long medical history of the patient and because tumour markers and imaging findings did not support the diagnosis of pancreatic cancer, immunohistochemistry was performed after a consultation with pathologists. The results showed that the lesion was negative for CEA(M), CEA(P), CK(AE1/AE3), CD56, CgA, Syn, Bcl-2, Bcl-6, CD10, CD20, CD3, and MUM1, and the lesion was positive for Ki-67 (+ 80%), P53 (approximately 50%), CD43, and MPO (Figure ). These results supported the diagnosis of myeloid leukaemia with pancreatic involvement. Bone marrow puncture and biopsy were performed again, and the results were almost normal. No further treatment was administered.\nIn October 2016, the patient’s right eye protruded significantly and was accompanied by severe pain; the eyelids were also unable to close. The patient underwent surgical treatment at another hospital, and the pathologic analysis suggested a malignant tumour. Although the combined immunodetection did not support an epithelial-derived tumour, myeloid leukaemia and osteosarcoma were still considered. After a consultation with pathologists at our hospital and the addition of a myeloperoxidase (MPO) (+) test, which was combined with other immunohistochemical results, myeloid sarcoma was considered. By then, the diagnosis of myeloid sarcoma was clear, and the patient was transferred to the Department of Haematology for treatment. On November 23, 2016, a CT examination revealed a mass shadow with a diameter of approximately 8.6 cm in the pancreatic tail. The density was not uniform, and a contrast-enhanced CT scanning showed significant enhancement. The demarcation between the lesion and the posterior wall of the stomach and the splenic hilum was not clear. The intrahepatic general bile duct exhibited dilation and pneumatosis, and spongy-like changes were observed in the portal vein (Figure , C). An IDA chemotherapy regimen was initiated in December. After three cycles of chemotherapy, the disease progressed. Radiochemotherapy (the lesion in the pancreatic tail, 24Gy/12F) was then given. However, in April 2017, the patient experienced pain due to the distention of the right orbit. An orbital MRI scan (T1-weighted imaging (Figure ) and T2-weighted imaging (Figure ) showed a uniform-signal intensity mass in the right lateral rectus area and right optic nerve compression with displacement, suggesting recurrence. No abnormalities were observed on bone puncture. The patient was then treated with radiotherapy again, during which multiple masses developed throughout the body. Ultrasound results suggested the possibility of the infiltration. Bone puncture was performed again, and the result suggested that primary granulocytes accounted for 38.4% of the lesion. Consequently, the patient was switched to DAC (decitabine) + CAG chemotherapy regimen. By February 2018, bone puncture results indicated remission, but the extramedullary disease continued to progress. |
The patient was a 37-year-old primipara woman. She had a history of uterine fibroids and cystectomy due to rupture of ovarian endometrial cyst. Transvaginal ultrasound performed at 5 weeks of gestation revealed a 6 cm fibroid in the fundus of the uterus.\nAt 16 weeks and 6 days of gestation, the patient developed abdominal pain and genital bleeding and was admitted to our hospital. On speculum examination, the cervix was impossible to visualize, and slight bleeding was observed. On vaginal examination, a solid mass was palpated in the pouch of Douglas, and the external uterine orifice was displaced above the symphysis pubis. Based on these findings, uterine incarceration was suspected. To reduce the incarceration and relieve her symptoms, she was instructed to assume a knee-to-chest position after micturition. However, at 18 weeks and 4 days of gestation, the abdominal pain and genital bleeding persisted and the physical findings remained unchanged. At 18 weeks and 5 days of gestation, magnetic resonance imaging (MRI) was additionally performed to obtain more detailed findings. MRI showed a large fibroid engaged in the pouch of Douglas and a cranioventrally stretched cervix. The uterus was strongly retroverted; therefore the fundus and the posterior wall of uterus were entrapped in the pelvis between the sacral promontory and pubic symphysis (). Based on MRI findings, the patient was diagnosed with uterine incarceration and threatened abortion.\nAfter that, she remained in the hospital and continued with the same management; however, the maneuver was unsuccessful. Therefore, manual reduction of the incarceration was planned. However, at 19 weeks and 5 days of gestation, IUFD occurred. Transvaginal and transrectal manual reduction was immediately attempted under general anesthesia to achieve vaginal delivery; however, the attempts were unsuccessful. An expectant management approach was planned, expecting a decrease in the uterine blood flow, leading to reduction in the size of the uterine cavity. We planned to follow up the patient once a week on an outpatient basis by speculum and pelvic examination for less than 4 weeks. Blood tests during the expectant management showed no signs of infection or coagulopathy. The minimum blood fibrinogen level before delivery was 335 mg/dl.\nAt 22 weeks and 3 days of gestation (19 days after IUFD), the cervix was visually recognized on speculum examination. On pelvic examination, the fibroid in the pouch of Douglas was still palpated, but the external uterine orifice was palpated in the normal position. At 23 weeks and 5 days of gestation (28 days after IUFD), the incarcerated uterus of the patient resolved spontaneously with reduction of the uterus; subsequently, labor was induced with gemeprost vaginal suppository after mechanical dilatation of the uterine cervix. The macerated fetus was successfully delivered. The patient had a favorable course after delivery and was discharged uneventfully. MRI performed 3 months after delivery showed a large fibroid in the fundus of the uterus (). Uterine fibroid may cause recurrence of an incarcerated uterus on the next pregnancy; hence, we performed laparoscopic myomectomy and adhesiolysis of the adhesion between the uterine posterior wall and the rectum. |
A 71-year-old Japanese postmenopausal woman was referred to us for evaluation of a tumor in the right breast. She had suffered from malignant lymphoma (diffuse, medium to large B-cell lymphoma) treated with chemotherapy consisting of eight cycles of R-CHOP and radiotherapy (total 46 Gy) to the head for the previous 3 years. She had undergone hysterectomy for myoma of the uterus at the age of 41 years. In addition, she had a medical history of hypertension and hyperlipidemia. There was no remarkable family history.\nPhysical examination demonstrated an elastic hard lump on palpation located in the lower lateral quadrant of the right breast. The tumor size was 2.0 cm in diameter, and the borderline was unclear. No skin change or dimpling was seen. Nipple charge was not evident. No axillary lymph node swelling was found. All laboratory data were unremarkable, and there was no increase of tumor markers such as CEA and CA15-3.\nMammography of the right breast showed an unclear mass with accumulation of calcification (Fig ). Ultrasonography showed a hyperechoic lesion within a hypoechoic area, with rough surface (Fig ). Enhanced magnetic resonance imaging revealed a mass of high intensity in the right breast (Fig ).\nHistopathological evaluation of the core needle biopsy material revealed an invasive adenocarcinoma with metaplastic change, but definitive histological diagnosis could not be determined. There was no proliferation of atypical lymphocytes.\nThe patient underwent right mastectomy and sentinel lymph node biopsy. The frozen section of the sentinel lymph node was found to be free of disease by intraoperative diagnosis.\nMacroscopically, cut sections revealed a white, solid, and well-circumscribed tumor measuring approximately 17 × 15 mm (Fig ). Histopathologically, the tumor was composed of cancer cells forming papillary or tubular structures with an abundant mucus cytoplasm, which was positive for periodic acid-Schiff staining, and accompanied by psammoma bodies (Fig , Fig ). Squamoid cancer cells proliferated in sheet-like patterns, but intracellular bridges or keratinization were not seen (Fig ). Intermediate cells were also seen (Fig ). In the stroma, many inflammatory cells proliferated around the tumor.\nImmunohistochemical findings showed that the tumor cells were positive for cytokeratin 7, cytokeratin 5/6, cytokeratin 14, epidermal growth factor receptor, p63, and MUC-1, and negative for gross cystic disease fluid protein-15, estrogen receptor, progesterone receptor, and human epidermal growth factor receptor-2. Ki-67 labeling index was 22%. The mitotic index was one mitosis per 10 high-magnification fields. No vascular or perineural invasions were observed. Histological examination determined MEC of the breast, intermediate histological grade. |
A 50-year-old male was admitted to the hospital with ongoing chest pain. Eleven years ago, he underwent implantation of a mechanical aortic valve prosthesis due to degenerated bicuspid valve with normal coronaries on preoperative angiography. His medical history included treated hypercholesterolaemia and current smoking. There were no previous records on atrial fibrillation, left ventricular dysfunction, coagulation disorders, or recent cancer. At admission, the cardiopulmonary exam was normal, the blood pressure was 95/65 mmHg, heart rate 79 b.p.m., temperature 36.2°C, and oxygen saturation of 99% on room air.\nThe electrocardiogram showed sinus rhythm with anterior ST-segment elevation (Figure ). Emergent angiography revealed total occlusion of the proximal left anterior descending artery (LAD, Figure , Videos 1 and 2). Thrombus was aspirated; however, no histopathological analysis of the extracted material was undertaken. Due to high thrombus burden and absence of the blood clotting status at the time of angiography, bolus of intravenous glycoprotein IIbIIIa inhibitor was administered. Except the apical part of the LAD showing distal embolization, coronary flow was completely re-established with no evidence of stenosis or significant atherosclerosis (Figure , Video 3). As an additional finding, first diagonal branch showed a filling defect possibly resulting from embolization or minimal iatrogenic dissection. An OCT of the LAD was performed, which revealed at the site of occlusion a normal vessel wall without relevant atherosclerotic plaque that could explain an erosion or plaque rupture, the middle part of the vessel showed minimal intimal thickening s(). Thus, an embolic event was the only reasonable explanation for the coronary obstruction. Accordingly, no stent was implanted. Transoesophageal echocardiography was performed and confirmed a floating structure measuring 3.5 mm in the left ventricular outflow tract (LVOT, Figure , , suggesting that an embolus originating from the prosthetic aortic valve obstructed the LAD. Except the moderate dysfunction of the left ventricle [left ventricular ejection fraction (LVEF) 40%], resulting from apical septal hypokinesia, no other abnormalities were observed, especially no dysfunction of the prosthetic aortic valve. Left atrium appendix thrombi and persistent foramen ovale were ruled out by transoesophageal echocardiography including a bubble study with Valsalva manoeuver. Left ventricular thrombus was not shown in the transthoracic echocardiography (TTE); however, it cannot be definitely ruled out since no contrast ultrasound agent was used. Although, according to Duke Criteria the diagnosis of infective endocarditis (IE) was possible (one major and two minor criteria, respectively, positive echocardiographic diagnosis, predisposing cardiac condition, and vascular phenomena embolism), in the absence of fever and laboratory inflammation parameters, we considered IE as unlikely, therefore neither blood cultures were obtained nor antibiotics were given. Additionally, the further course of the patient including 3 months of follow-up revealed no events suggesting of an endocarditis.\nThe international normalized ratio (INR), gained after angiography, measured 4.3, however, 2 days prior to presentation, the INR was 1.9 and the patient administered 5 mg warfarin. Target INR was increased to 2.5–3.5. Due to previously subtherapeutic INR suggesting suboptimal anticoagulation control and possible minimal non-flow limiting dissection in the diagonal branch aspirin 100 mg was given additionally only for 1 month. A month later, the floating structure in the LVOT was no longer detectable in the transoesophageal echocardiography, prosthetic aortic valve functioned normally and left ventricular function normalized (LVEF 55%). Computed tomography thorax likewise showed neither evidence of thrombus in the LVOT nor any other intrathoracic pathology. The patient was advised to take part in the INR self-testing training. |
Peter, a 12-year-old boy, accompanied by his maternal aunt, presented at the local university Dental Hospital (Pediatric Dental Clinic) in Kenya in October 2012, with a complaint of a large, painful left facial swelling related to the left upper incisors. He had been referred from a local rural hospital where he had been taken by the same aunt, for treatment of the swelling. The swelling had occurred only 2 days prior to visiting the local hospital, and 4 days before presenting himself at the University Dental Hospital. Enquiry about the causes of the swelling provided unclear answers. Family history indicated that the young boy was a first-born among three siblings (9-year-old girl, 5-year-old boy), and that their single parent (mother) had been deceased for 6 years due to HIV-related complications. The three children had moved to live with their maternal grandparents and their seven sons. The patient had no adverse past medical history and had never consulted a dentist previous to the present problem. The boy was in grade seven in a local primary school and had the aspiration of becoming a medical doctor in future. It was not possible to establish from the aunt or the boy the situation of the patient's other siblings.\nAn extra-oral examination showed a young boy with a normal gait, sickly, unkempt, rather withdrawn, and small for his age. He had asymmetrical face due to the swelling involving his left submandibular region and spreading upwards to the inferior orbital margin, febrile (39.1°C), a marked submandibular lymphadenopathy on the left side, the skin overlying the swelling was warm, shiny and fluctuant, and the lips were dry and incompetent (2 cm) and as shown in [Figures –]. However, the temporomandibular joint movements were normal. The patient was also found to have a big, healing scar on the dorsal surface of the left foot, the cause of which was also unclear [].\nIntra-oral examination revealed a young boy in the permanent dentition with un-erupted third permanent molars, poor oral hygiene with heavy plaque deposits on the tongue and a generalized but moderate inflammation of the gingiva. There was a grade three mobility in relation to 11, 12, 21, 22 and a grade two mobility in relation to 23, 24, 25 (Miller mobility index). There was intramucosal swelling in relation to 21-24 extending labially/buccally (measuring 4 cm × 3 cm) and palatally (measuring 3 cm × 2 cm). On elevation of the upper lip, active discharge of pus mixed with blood and some black granules could be seen emanating from the abscess. There were no alveolar/bone fractures elicited, but carious lesions were present on 46 (occlusal), 47 and 37 (buccal). Orthodontic evaluation showed Angles class I molar relation on the left and edge to edge tending to class II on the right side. The canines were in class I relationship bilaterally. There was an anterior over-jet of 3 mm (11/21), an overbite of 20%, coincidental dental/facial midline and crowding on the upper right arch with 15 palatally displaced as can be seen in Figure –.\nFor investigations, orthopantogram, intra-oral periapical 11, 12, upper and lower standard occlusal and bite wing radiographs were taken and examined. In addition, clinical photographs, study models, and vitality tests for the traumatized teeth were undertaken. A diet and nutrition assessment, full blood count, stool microscopic analysis for ova and cyst and bacterial culture and sensitivity were also undertaken.\nThe results of the radiographs showed un-erupted with potential impaction of 48 and 38, an upper midline radioluscence, widened periodontal space in relation to 11, 21 (with a mesial tilt), 22, occlusal caries on 46 and buccal caries on 47 and 37. There was the presence of root fractures involving the apical one-third of 21, 22. Vitality tests conducted on the traumatized incisors showed false positive (may be due to the presence of infection). The blood analysis showed the presence of neutrophilia (suggestive of bacterial infection), mild iron deficiency, but he was sero-negative. From the diet chart, the boy was generally on a noncariogenic diet that lacked the intake of fruits and animal proteins. Nutritional assessment revealed a boy with a height of 144 cm, a weight of 28 kg, and a body mass index (BMI) of 13.5 Kg/m2 (below 5th percentile (given the ideal BMI should be 17.8 Kg/m2 in the 50th percentile).\nFrom the history adduced and the results of the investigations, a diagnosis of child abuse and neglect was reached, with the boy having suffered traumatic injuries resulting in facial cellulitis, Ellis class VI fracture involving 21, 22 associated dentoalveolar abscess and subluxation of 11, 12. In addition, there were dental carious lesions on 46 (occlusally), 47 and 37 (buccally) and a relatively severe malnutrition. The patient had also moderate plaque induced gingivitis, mild anemia (microcytic and iron deficiency), mild dental fluorosis, potentially impacted 48 and 38 and crowding in the upper right and lower anterior arches.\nThe objective of treating the boy was to eliminate the pain, infection, improve the general and oral health, restore carious teeth, improve esthetic and report the child abuse and neglect to the relevant authorities. In the initial phase of treatment, the patient was admitted for 4 days and placed on dexamethasone 8 mg stat, cefuroxime 750 mg 3 times a day, metronidazole 500 mg 3 times a day, diclofenac 50 mg tablets alternating 4 hourly with oral paracetamol 1000 mg 3 times a day, to run for 5 days. Patient was also placed on chlorhexidine mouthwash 10 ml twice daily for 7 days and ranferon (hematinics) 10 ml to be used twice a day for 1 month. The second phase of treatment included incision and drainage of the abscess, followed by the splinting of the mobile teeth in the upper dental arch using semi-rigid splint of 0.6 mm stainless steel round wire for 4 weeks while. Root canal treatment of 11, 21, 12, and 22 followed thereafter.[] A referral of the patient was made the child support center in the main referral hospital, plus the patient was placed on future recalls to determine whether the patient would have overcome the problem and the oral health was maintained in good condition.\nThe third phase of treatment involved interceptive orthodontics with the extraction of 15 to relieve the crowding in the area. Oral hygiene instructions were availed to the patient and the guardian, placement of fissure sealants was done for the premolars and molars to help reduce plaque retention on these teeth, preventive resin restorations were placed on 37, 46, and 47. The root fractures involving the apical one-third of 21 and 22 meant that the two teeth were to be initially dressed using non setting calcium hydroxide, and after healing, root canals are filled in the usual manner [].\nNutrition evaluation had initially been done and when the patient was re-evaluated after 1 month, he had gained bodyweight up to 1 kg. The child support center continued to carry out psychotherapy, and during one of the sessions, the patient confessed to having undergone physical abuse and threatened not to divulge any information by one of the uncles. The center considered placing the boy into a children's home, probably together with his siblings. Radiographic examinations evaluation after 3 months indicated some external apical root resorption taking place on 21 and 22. Further follows-ups were to continue.[] After 10 months, the oral health and general heath of the patient had remarkably improved as shown in . |
A 73-year-old woman with no previous comorbidities or family history of hematological disorders or hypercoagulability was admitted to the Infectious Disease Clinic due to suspected HFRS and dehydration. Two weeks prior to disease onset, she had been exposed to bank voles while cleaning out a cabin. For 6 days following disease onset, she had been ill with fever, chills, weakness, low urine production, and difficulties eating and drinking. The patient had positive serology for Puumala virus thereby confirming the HFRS diagnosis. Laboratory tests taken the day before admission revealed thrombocytopenia (platelet count: 48 × 10\n9\n/L), impaired renal function (creatinine: 278 μmol/L), and leucocytosis (white blood cell count: 14 × 10\n9\n/L). Upon admission to the hospital, the platelet count had increased to 61 × 10\n9\n/L and creatinine increased to 370 μmol/L indicating clinical progression to the oliguric stage of HFRS. However, the platelet levels decreased to 12 × 10\n9\n/L on days 8 to 9. The treating physicians decided to transfuse platelets on days 8, 9, and 10 due to the high risk of spontaneous bleeding. Despite transfusion with three platelet units, the patient remained severely thrombocytopenic with platelet counts below 50 × 10\n9\n/L during days 8 to 13. The case is summarized in\n. Criteria for disseminated intravascular coagulation (DIC) were fulfilled from day 8 (see\nfor an overview of criteria).\nOn day 13 (2 days after the last platelet transfusion and a platelet count of 27 × 10\n9\n/L), the patient falls ill with abdominal pain which increases in severity during the evening. An abdominal computed tomography (CT) shows congestion and ischemia in the terminal ileum due to a thrombus in the superior mesenteric vein (SMV) reaching up to the portal vein (PV). The hematologist advised against thrombolysis due to thrombocytopenia in combination with a known mild VHF, which could increase the risk of bleeding. A national coagulation expert is consulted for further advice, who recommends anticoagulant treatment with heparin in a “careful” dose. Heparin at a dose two-thirds the national recommended dose is initiated with the aim of APTT 1.5 times the baseline value (40–50 s). The patient therefore receives a bolus dose of 4,000 units heparin followed by transfusion of 24,000 units heparin per day. The following day (15), the patient has gastrointestinal bleeding and a decrease in hemoglobin values from 100 to 89 g/L. According to the surgeon consultant, the stasis caused by the SMV and PV thrombus damages the intestinal mucosa leading to the observed gastrointestinal bleeding. Heparin is therefore continued in the same careful dose and the patient receives one unit red blood cells (RBCs) that day and the following day (16). The following criteria had to be fulfilled before mesenteric phlebography and thrombolysis via catheter could be considered: (1) platelet levels greater than 100 × 10\n9\n/L, (2) no bleeding, and (3) the patient can tolerate a full-dose heparin. On day 19, the patient fulfilled these criteria and underwent mesenteric phlebography via interventional radiology. Using the percutaneous transhepatic route to the PV, a hydrolyser 7F, double lumen, over-the-wire thrombolysis (Hydrolysis, Cordis Europe NV, Roden, the Netherlands) was used to perform a mechanical thrombolysis of the PV followed by pharmacological thrombolysis with tPA (Actilyse infusion: 0.8 mg/hour). A notation from the surgery department states that the previous heparin treatment aiming for 1.5 times APTT had been unsuccessful in decreasing the size of the SMV and PV thrombus. A control angiography 6 hours postsurgery shows that the thrombus distally in the SMV has been removed. There is still a thrombus between the portal and the splenic vein; therefore, the catheter is moved further into the area of thrombosis and thrombolysis by Actilyse administration is continued. At this time, there is no contrast leakage as a sign of hepatic bleeding. The patient stays in the intensive care unit (ICU) with local hydrolysis via the catheter. The following day (20), the patient becomes hypotensive with systolic blood pressure down to 75, and has signs of peritonitis. The levels of the fibrin degradation product D-dimer increases to 20 mg/L and fibrinogen decreases to 0.69 g/L. A CT thorax/abdominal scan shows an ongoing expanding hepatic intraparenchymal arterial bleeding. In addition, the CT scan shows presence of pulmonary emboli. Since the patient has a propensity for bleeding and thromboembolism, arterial intervention via the femoral artery into the aorta and then out into the common hepatic artery with coiling was not an option. Instead an emergency surgery procedure is performed with ligation of the right hepatic artery in the hepatoduodenal ligament, which stops the bleeding. In addition, the anticoagulant therapy is discontinued that day and the patient is tended in the ICU in a respirator. On day 21, the anticoagulant therapy is readministered at a low dose. The patient is extubated on day 22 and the anticoagulant therapy is increased to a target of APTT 60s due to remaining portal thrombi and peripheral pulmonary emboli. On the evening of day 25, the patient develops acute dyspnea, and oxygen saturation decreases to 88% with 4 L of oxygen and tachycardia. A CT pulmonary angiography shows pulmonary emboli in the right and left pulmonary arteries and peripherally in the pulmonary lobe arteries. The patient is transferred that evening to the ICU with heparin treatment at a target of APTT 85s. An ultrasound of the peripheral extremities (day 26) shows bilateral deep vein thrombosis in the posterior tibial veins. Since the APTT remains difficult to adjust (ranging from over 180s to the therapeutic target of 80s) and the propensity to develop thrombosis despite anticoagulation, it is decided to change the treatment from heparin to low-molecular-weight heparin (Fragmin 16,000 IU/day) on day 32. After day 55, the patient receives warfarin as prophylaxis against further thromboses and is discharged to her home on day 61. To rule out other causes for the thromboembolic complications, she was tested and found negative for activated protein C resistance. |
A 64-year-old caucasian male with an unknown past medical history presented to the Bayfront Hospital ED in St. Petersburg, Florida, in early November. The average outside temperature that night was in the mid to upper 60s. The patient was initially found by local police officers lying unconscious in a ditch next to his bicycle. When EMS arrived on scene the patient was cold, unresponsive, GCS 3, covered in ants and without any other obvious signs of skeletal or traumatic injury. The patient was fully immobilized in a cervical collar and on a backboard prior to transport to the ED and was intubated secondary to his low GCS. The patient's vitals at the time showed sinus bradycardia at a rate of 30-40s, palpable pulse but an unobtainable blood pressure on multiple attempts.\nAt 08:22 the patient arrived at the ED and upon transfer to the trauma bed was noted to be in ventricular fibrillation. At that time AED pads were attached and advanced cardiac life support (ACLS) protocol was initiated. Trauma protocol labs were sent which consisted of CBC, CMP, serum cortisol, serum ETOH, amylase, TSH, cardiac enzymes, and a coagulation profile.\nDuring the resuscitation the emergency room physician and trauma surgeon noted that the patient was very cold to the touch. A rectal temperature was performed but the temperature did not register. Per trauma protocol, the patient was being given warm IV fluids as well as warm humidified air via mechanical ventilation. It was not until 09:06 that the patient's temperature was accurately recorded to be 24.9°C (76.9°F). At approximately 09:22 the patient had been undergoing ACLS protocol for one hour and had been defibrillated 13 times and given five rounds of epinephrine, two rounds of atropine, and two rounds of amiodarone. With the patient still in ventricular fibrillation, it was decided to continue cardiopulmonary resuscitation with chest compressions and mechanical ventilation until the patient's core temperature increased. Supplemental active re-warming interventions including warm water lavage via OG tube and Foley catheter were started at that time.\nThe patient remained in ventricular fibrillation for more than three hours until 11:45, when his rectal temperature was 30.1°C (86.2°F) he was successfully converted into normal sinus rhythm at 66 bpm with a manual blood pressure of 180/120. As the patient's temperature continued to rise he went in and out of atrial fibrillation and sinus tachycardia but eventually returned and remained in normal sinus rhythm.\nAfter cardiac stabilization, a complete secondary survey was performed that showed no other obvious signs of trauma or skeletal injury. A central line and arterial line was placed and he was started on a dopamine drip as his blood pressure decreased to 88/56. Once the patient's temperature reached 31.2°C (88.2°F) his OG and Foley catheter lavage were discontinued. The patient eventually became lucid and responsive to verbal commands. Although unable to remember the accident, or any events leading up to the accident, he did appropriately respond to some basic questions. It was at this that the patient's motor deficits were noticed as he was unable to feel anything from the neck down or move any of his extremities.\nThe patient underwent a scan of his head, neck, abdomen, and pelvis. The scans showed no head injury or C-spine fractures and noted only a small 1 mm broad-based disc bulge at the level of C5-6 and C6-7, moderately dilated loops of bowel consistent with an ileus and two small bilateral pleural effusions. Although a toxicology screen was not obtained, he did have an ETOH of 0.183 g/dl. The patient was transferred to the ICU, and subsequently underwent an MRI of his C-Spine which demonstrated diffuse edema of his cervical spine. After a prolonged hospital stay the patient's mental status did improve to baseline but his paralysis did not as he remained a tetraplegic. The patient was then transferred to a rehab facility for further care. |
To our knowledge, this is the first reported case of an index finger MCP joint dislocation surgically treated by a lateral approach.\nThe authors describe a case of a 16-year-old male who suffered a fall onto his outstretched right hand during a soccer game. The patient presented to the ER with pain and deformity of the index finger MCP joint. Volarly, the prominence of the second metacarpal head was evident ().\nRadiographs confirmed a dorsal index finger MCP joint dislocation and showed a small dorsal osteochondral fragment (Figures and ).\nAfter multiple unsuccessful reduction attempts under ring block by different physicians, the patient was referred to surgery.\nUnder general anesthesia, a lateral surgical approach () was performed on the MCP joint. A straight longitudinal incision was made over the lateral aspect of the MCP joint; the volar neurovascular bundle and the dorsal branch of the digital nerve were identified and retracted with Farabeufs.\nInterposition of the volar plate () preventing the reduction was observed. Applying gentle traction and flexion, the MCP joint was reduced, and proximal volar plate reinsertion with a 4-0 Vicryl suture was performed.\nThe posterior joint capsule was identified and split longitudinally, above the collateral ligament. Once adequately exposed, a small osteochondral fragment was found (). Reduction and retrograde fixation of the osteochondral fragment with a 1.7 mm screw were performed, burying the screw head in the cartilage.\nThe joint capsule, subcutaneous layer, and skin were closed using appropriate sutures. Reduction was confirmed by intraoperative fluoroscopy.\nThe patient was placed in a volar splint with approximately 45° of flexion and discharged on postoperative day zero without any complications.\nImmobilization was removed by week 3. Radiographic control revealed joint congruence, and the patient was encouraged to actively mobilize the finger.\nAt week 6, the fracture was consolidated (Figures and ). The joint was painless and presented slight stiffness (ROM 0-70°). The patient could return to competition with protective syndactyly.\nOne year postoperative, there was no pain, growth disturbance, or joint stiffness, with full ROM of the index finger. |
The proband was a 34 year-old right-handed man. From the age of 26 years, involuntary movements of the bilateral lower limbs, associated with dysarthria, grinding teeth and drooling, appeared and gradually worsened. At 31, he suffered from epileptic seizures, which were considered to be generalized tonic-clonic seizure, but antiepileptic drugs had never been administered. One year later, involuntary movements spread to his upper limbs and orofacial automatisms including abnormal tic-like facial movements, tongue protrusion and biting his lips appeared. Then he was treated with haloperidol (2 mg three times a day) and baclofen (10 mg three times a day) for 2 years for his choreic and dystonic problems, but he responded poorly to drug treatments. At age of 34, his involuntary movements gradually spread to his whole body and epileptic seizures increased in frequency. Since the disease onset, the patient had never suffer from psychiatric problems. Neurological examination revealed poor muscle tone and absent deep tendon reflexes in all limbs. Additionally, right positive babinski sign was elicited. Laboratory data revealed elevated creatine kinase level in the peripheral blood. Acanthocytes were found in 4% of cells on the peripheral blood smear test. Doppler ultrasound examination revealed splenomegaly. Brain magnetic resonance imaging (MRI) showed progressive, symmetrical, mild atrophy of the caudate heads (Figure ). His 24-h continuous electroencephalography (EEG) showed generalized asynchronous theta and epileptiform activity, which mostly originated from the right temporal lobe. A nerve conduction study showed a polyneuropathy, which revealed the right peroneal nerve, right median nerve and bilateral ulnar nerves were partly damaged. His score of Mini Mental Status Examination (MMSE) was 27. The father of the proband did not show any neurological abnormalities and died from pneumonia at 65 years old (Figure ). The mother of the proband (II-3), a 65-year-old woman, showed mild involuntary movements in her limbs since the age of 45 years (Figure ). The proband's uncle (II-5), a 52-year-old man, showed mild cognitive impairment (MMSE 24), characterized by memory impairment and had seizures history of 31 years, which were simple partial seizures and treated with antiepileptic drugs (Figure ). His another uncle (II-1) and two sisters (III-1, III-2) had no neurological clinical symptoms. Brain MRI and peripheral blood smears of the proband's mother, his uncles and two sisters are normal. The clinical picture of the proband was suggestive for ChAc, but the inheritance mode of this family seems to be autosomal dominant.\nAll patients were of Han nationality from Hunan province, China. Blood specimens and genomic DNA were obtained from family members and 100 control subjects after informed consent. The 73 exons and flanking intronic splice consensus sequences of VPS13A were amplified by polymerase chain reaction (PCR) (, ). By sequencing, we identified a novel homozygous nonsense mutation c.8823C > G (p. Tyr2941*) in exon 65 of VPS13A in the proband (Figure ). Five members of the family including the proband's mother (II-3) and his uncle (II-5) were detected to be heterozygous for mutation c.8823C > G (Figures ). The homozygous nonsense mutation c.8823C > G (p. Tyr2941*) causes the loss of TPR10 domain of the vacuolar protein sorting 13A protein. This homozygous nonsense mutation c.8823C > G was not detected in 100 healthy controls, thus representing a novel etiology in an ChAc Chinese family. Besides, the mutations in genes responsible for Huntington's disease and McLeod disease were screened and the results were negative. |
A 9-year-old girl was admitted to the Bone Marrow Transplant Unit for a second haematopoietic stem cell transplantation (HSCT) procedure for SAA from her HLA-matched sister. Her blood count had returned to normal after her first transplant but she became pancytopenic again following a primary parvovirus infection 18 months after initial transplantation. This resulted in severe neutropenia for six months prior to admission whilst awaiting viral clearance. She was maintained on prophylactic cotrimoxazole and itraconazole throughout this period.\nPrior to admission the patient had been nonspecifically unwell for a number of weeks, with a persistent cough, but was afebrile with a CRP of less than 10 mg/dL. Due to these symptoms she was thoroughly investigated on admission. Echocardiogram, lung function tests, and CT scan were all normal (the latter shown in ). The patient underwent one week of conditioning chemotherapy followed by HSCT and tolerated this well.\nShe remained well in herself (apart from her mild cough) until day 5 after transplant when she became febrile. Antibiotics were administered (piptazobactam, gentamicin, meropenem, and vancomycin in sequence according to local febrile neutropenia guidelines) but pyrexia worsened and CRP rose to 133 mg/dL on day 7 after transplant (clinical parameters shown in ). Chest radiograph (CXR) at this stage was clear (). Daily intravenous liposomal amphotericin (1 mg/kg) was commenced in view of the previous protracted neutropenia. Additional symptoms of increasing tachypnoea and reduced breath sounds on examination prompted CT scanning on day 9 after transplant (). This demonstrated left upper lobe collapse and a small left pleural effusion but no parenchymal nodules suggestive of fungal infection; nevertheless, caspofungin antifungal therapy was added due to the severity of the clinical situation.\nDyspnoea progressed and a CXR on day 11 after HSCT identified progressive lung collapse () with lack of an air bronchogram, noted to resemble foreign body obstruction, just proximal to the bronchus leading to the lingula lobe. In retrospect this could also be visualised on the CT image (). An extrabronchial mass was deemed unlikely; therefore, an endobronchial mass was suspected, resulting in a high suspicion of an occluding EBFI. Although Aspergillus antigen testing at this time was negative, concern about clinical progression led to increased antifungal therapy: voriconazole (optimal first line therapy for invasive aspergillosis) was added to caspofungin and an increased dose of liposomal amphotericin (5 mg/kg) was used.\nThe patient initially exhibited a good response to triple antifungal therapy (pyrexia and dyspnoea resolved), supported by a rising neutrophil count (). However, on day 19, she developed shoulder tip pain and by day 21 redeveloped a fever. Following exclusion of the more common causes of febrile illness seen in patients recovering their neutrophil count after HSCT, bronchoscopy was performed on day 27. This revealed a white plaque completely occluding the left main bronchus; slight suction on this mass produced profuse bleeding and bronchoscopy was aborted. Microscopy of bronchoscopic samples identified fungal hyphae and Aspergillus fumigatus was grown in culture. The patient remained afebrile but with a persistent CRP between 20 and 40 and was therefore commenced on additional nebulized amphotericin (5 mg BD, preceded by nebulised salbutamol) on day 38. Her CRP fell to 12 on this regime by day 48.\nWhilst left lower lobe atelectasis resolved, the upper lobe collapse remained and lobectomy was required subsequently. This entailed a complex, eight-hour procedure which involved extensive bronchovascular dissection because of adherence of nodes to the hilar structures including opening of the pericardium and the left main bronchus. The patient remained well and free of Aspergillus infection five years later. |
A 60-year-old man with a past surgical history of C5–C7 anterior arthrodesis for cervical fracture 17 years ago was found on the floor after an unwitnessed ground-level fall, resulting in a head strike. He was taken to the local hospital in an Aspen collar with a GCS of 15 and no neurologic deficits. Routine ECG and laboratory evaluation was unremarkable except for elevated serum alcohol level. Full cervical spine computed tomography (CT) scan was performed which showed an Anderson and Montesano classification (20) type III left occipital condyle fracture (), a Levine and Edwards classification (21) type III (Jefferson) fracture with mild lateral subluxation of bilateral C1 masses (), and an asymmetric widening and slight anterior subluxation of the right AOJ (). A head CT was performed which showed no acute intracranial abnormalities.\nThe patient was immediately transferred to a level I trauma center for further management. In the emergency department, he reported severe midline neck pain, worse with movement, and unchanged paresthesia to bilateral upper extremities which he states is his baseline. Physical exam was unremarkable except for a right forehead hematoma with overlying abrasions and tenderness to palpation over the midline posterior neck. He exhibited full strength and unchanged baseline sensation to all extremities. He denied bowel or bladder incontinence and had strong rectal tone and intact perianal sensation. Through his clinical course, he became increasingly altered and agitated, refractory to medication. He was unable to maintain spinal precautions due to his agitation, and in an attempt to protect his cervical spine, he underwent endotracheal intubation.\nMRI of the cervical spine was performed and showed ligamentous injury at the craniocervical junction (CCJ) (Figures and ), asymmetric left odontoid-lateral mass widening, widening and subluxation of the right AOJ (), and edema of the paravertebral soft tissues around the fracture. CT and MRI established the diagnosis of OCD in a neurologically intact patient. Due to the unstable nature of the fracture, the patient was taken to the operating room for emergent occiput-C2 posterior instrumentation and fusion ().\nAn Aspen collar was in place at all times until surgical stabilization. Immediately, after removing the collar, Mayfield tongs were applied. Prior to patient positioning, baseline somatosensory evoked potentials (SSEPs) were obtained. Using a Jackson table turning frame, the patient was rotated into prone position. Fluoroscopic imaging was used during manual manipulation of the Mayfield apparatus to ensure no further displacement of the fracture.\nOcciput to C2 posterior instrumented arthrodesis was performed. Neuromonitoring remained stable during the procedure. The patient was discharged to home five days after presentation. |
An 82-year-old woman presented with a 2-week history of a sudden onset of acute lower back pain and worsening mobility. She did not report any other significant medical history. A clinical examination revealed a patient who was clinically dehydrated. She was pyrexial (39°C), tachycardic (pulse rate of 105/minute) and hypotensive (blood pressure of 90/50 mmHg on admission). Abdominal, chest and musculoskeletal examinations and urine analysis did not reveal any significant findings that could be attributed to her worsening sepsis. She had a leucocytosis level of 19,600, which increased to 40,700 in 24 hours, accompanied by worsening pain and pyrexia. She was also noted to be oliguric. Her lower back pain worsened and resulted in her becoming bedridden within 24 hours.\nAppropriate resuscitative measures were initiated upon admission. She was also commenced on broad spectrum intravenous antibiotics. An ultrasound scan and a computed tomography (CT) scan of her abdomen were performed. These revealed an abnormal infrarenal aorta with evidence of stranding of periaortic fat planes and a localised perforation resulting in a contained leak into the surrounding soft tissue (Figures and ). The iliacs were not involved and there was no evidence of an aneurysm. The blood cultures that were taken on admission confirmed the presence of Staphylococcal aureus. The overall findings were consistent with abdominal aortitis. A white cell scan was not performed as this would have caused a delay to the operation on this patient. She was classified as high-risk for a general or spinal anaesthetic, therefore an endovascular aortic repair (EVAR) was carried out under local anaesthetic. Her common femoral artery was exposed and punctured under direct vision and a sheath was introduced. A 20-mm tube stent graft (Zenith® Endovascular Graft Leg Extension) was then cantered over the site of perforation in the infrarenal aorta. The distal landing zone was proximal to the bifurcation. A check angiography was performed pre and post deployment. This confirmed the correct positioning of the stent and the exclusion of the leak.\nAfter the procedure, our patient had a full complement of pulses in both lower limbs and minimal pain control was required. Added risks and complications of a general or spinal anaesthetic were avoided. Antibiotics (Linezolid ®) of 600 mg IV twice daily were started after the operation and continued for approximately 3 weeks. The antibiotics were selected following positive blood cultures and sensitivities.\nOur patient's problem with sepsis was resolved and her overall clinical condition continued to improve with the signs of inflammation returning to normal levels. A CT scan (Figures and ) performed 2 weeks after the operation revealed that the endoluminal stent had effectively sealed the aortic leak. The patient was asymptomatic and well at a follow-up check-up one year later. A CT scan (Figure ) revealed no evidence of aortitis or leak. |
A 51-year-old male was diagnosed with muscle-invasive urothelial cell carcinoma in May 2015. Standard neoadjuvant chemotherapy was given followed by radical cystoprostatectomy. Final pathology showed pT3 N0 M0, stage III disease. He remained disease free until April 2016, at which point he developed new exertional dyspnea and a small lung nodule was noted on imaging. During cardiac clearance for a biopsy, a Mobitz 2 heart block with bradycardia was noted. Transthoracic echocardiogram (ECG) demonstrated a mass in the right ventricular outflow tract, which was additionally found to be fluorodeoxyglucose (FDG)-avid on positron emission tomography (PET) computed tomography (CT) (Figure , upper panel).\nThe patient developed progressive dyspnea with minimal exertion and then experienced a syncopal episode prompting hospitalization. Biopsy of the right ventricular mass demonstrated poorly differentiated carcinoma, consistent with urothelial origin (Figure ).\nThe mass was not felt to be respectable and chemotherapy was not felt to offer rapid disease control in the setting of progressive symptoms. He was offered palliative radiotherapy. His bradycardia progressed to a complete heart block, likely due to the growth of the mass, necessitating placement of a dual chamber pacemaker. The right ventricle mass was treated with 45 Gy in 18 fractions (3D conformal photons for 5 fractions, followed by intensity-modulated radiotherapy for the remaining 13 fractions (to reduce dose to the left ventricle). Figure demonstrates the dose distribution.\nProton radiation was considered to spare the uninvolved myocardium. He had substantial tumor thromboembolic disease to his lungs during radiotherapy, requiring medical intensive care unit (MICU) admission for symptom management. He ultimately completed the full course of radiotherapy which improved his functional status.\nFollowing radiotherapy, he began immune checkpoint therapy with the PD-L1 antagonist atezolizumab (1200 mg every three weeks), which was well tolerated. A PET/CT scan seven months after completing radiation demonstrated a complete metabolic response in the right ventricle mass and no other sites of progression (Figure , lower panel). The previously noted pulmonary nodules were stable. He resumed normal activity and returned to work. Eight months after radiotherapy and six months into his immune therapy, he developed progressive shortness of breath. He had heart failure and the ejection fraction reduced to 20%-30%. Cardiac magnetic resonance imaging (MRI) revealed a focal area of subendocardial delayed enhancement, potentially representing myocarditis. Cardiac catheterization revealed extensive coronary artery disease and complete occlusion of the right coronary artery; however, the left to right collaterals were intact. Atezolizumab was held with an initiation of high dose steroids to treat potential immune-mediated myocarditis with minimal effect. Electrophysiology testing demonstrated severe cardiac dyssynchrony. Cardiac resynchronization therapy was recommended and a biventricular pacer was placed with rapid symptom relief. He returned to work with overall minimal symptoms. Atezolizumab was resumed, and a total of 18 cycles (54 weeks) of therapy were completed without issue.\nIn the months that followed the placement of his biventricular pacer, he was evaluated several times for shortness of breath and atypical chest pain, however with no clear etiology. PET/CT imaging in August 2017 demonstrated increased FDG-avidity in his right ventricle concerning for progression. An MRI was ordered and in early September 2017, he was admitted for workup of an exacerbation of chest pain. A contrast-enhanced CT of the chest showed significant disease progression in the area of prior treatment, extension toward the left ventricle and along the heart wall leading to increased right heart pressure (Figure ), and probable encasement of his left anterior descending (LAD) artery by disease progression (Figure ). He had acute progression of chest pain associated with an increased oxygen requirement, shortly after being admitted. His ECG showing ST elevations in the anterior leads, consistent with an LAD infarct (Figure ). The cardiology service was consulted to consider palliative LAD stenting; however, the patient underwent cardiac arrest in the interim and did not wish to be resuscitated. He died 17 months after his disease recurrence. |
We describe the case of a 15-year-old right-handed boy, who one day after a swim at the local beach decided to take a nap. When he woke up, he noted blurring of vision of the left half of his visual field. He moved his left arm and noted it was moving in slow motion. He did not compare the left and right arm. He raced to report this phenomenon to his mom and he noted that his sister's babysitter was walking in slow motion. It was not clear if he distinguished the slow motion of his left arm from bona fide weakness. However, his mom noted that he was clumsy while grasping a cup with his left hand, potentially an optic ataxia. He complained of similar symptoms to his left leg, yet again we were unable to ascertain definite clumsiness, in addition to slow motion, the latter being definite. He noted fleeting and transient paresthesias of his left arm and leg. His speech remained fluent but his mental acuity was blunted. He was an avid reader with an eidetic memory for languages and culture, a bona fide savant. But his mother had noted that he was unable to grasp information magnetically, as he did before. He remained remarkably calm during this whole ordeal. By 12 hours, the visual blurring and slow motion perception had resolved.\nHis past medical history was significant for autism spectrum disorder and obsessive compulsive disorder. It was also noted that his symptoms, specifically the blurring of vision, were magnified with the intrusion of obsessive thoughts. His family history was negative for premature coronary artery disease or cerebrovascular disease. His father had a history of deep venous thrombosis. His medications included aripiprazole, pimozide, asenapine and metaformin. The metformin was included as an antidote to the diabetic inducing properties of these three atypical neuroleptics. The pediatric psychiatrist deemed it absolutely necessary for the patient to remain on three dopamine antagonists in order to control his obsessive thoughts and behavior. After a long struggle at school, this highly intelligent young man was schooled at home.\nOn examination, his height was six feet (93rd centile), his weight was 172 pounds (92nd centile), BMI of 23.3 (82nd centile), BP of 115/75 mmHg, temperature 95.5 degrees Fahrenheit and pulse of 110. Precordial examination with a stethoscope did not reveal a cardiac murmur, and there was absent bruits upon auscultation of the skull and carotid arteries. His gait was perfectly normal, and he could easily tandem and stand effortlessly on either leg. His mental state was sound. He was a little withdrawn, not his usual inquisitive and talkative nature. He seemed a little distracted but answered questions appropriately. His two-minute delayed memory was one out of three words. His digit span was five out of seven. Visuomotor skills were preserved with pantomime. No limb kinetic apraxia or ideomotor apraxia was noted bilaterally with coin deftness and transitive actions, respectively. His speech was fluent, naming intact and understanding effortless. No dysarthria was noted. Pertinent cranial nerve examination revealed full ocular motion with preserved accommodation and absent ptosis. Visual fields were full to confrontation and visual field neglect absent with sequential unilateral and bilateral visual field confrontation. No facial weakness was noted. His tongue protruded to the midline.\nMotor examination using Medical Research Council (MRC) scale was 5/5 throughout all the muscles of the upper and lower limbs, with one exception; sequence motion of the fingers of the left hand was subtly clumsy, and finger spreaders of the left hand were graded at 4/5. His reflexes were lively throughout the upper and lower extremities. Pertinent sensory examination revealed normal stereognosis and graphesthesia of the left hand, with intact two-point discrimination of the left index finger. Touch, pressure, pin prick and kinesthesia were normal in the index fingers of both hands. Cerebellar examination revealed normal alternating hand motion and the absence of dysmetria or intention tremor of the arms.\nDiffusion-weighted MRI revealed hyperintensity of the banks of the right POs. The ischemic nature of this lesion was confirmed by a matching hypointensity on gradient echo (GRE) imaging (Figure ).\nTo confirm the location of the infarct on the right POs, we compared it to the left POs, on sagittal T1-weighted images (Figure ).\nThe fluid-attenuated inversion recovery (FLAIR) MRI image confirms further that the ischemic infarct is in the vicinity of the POs (Figure ).\nA CT angiogram of the brain and carotid duplex scan were normal. The usual embolic infarct workup was carried out. The patient was telemetry monitored, and a transthoracic echocardiogram showed no evidence of valvular vegetations, cavitary thrombus or atrial septal defect. The pediatric cardiologist deemed that a transthoracic echocardiogram was adequate interrogation of the heart for a 15-year-old boy. A lumbar puncture revealed a non-inflammatory cerebrospinal fluid. A full thrombophilia panel included protein C, S, anti-thrombin III activity, prothrombin G20210 A mutation, IgG and IgM antiphospholipid antibodies, beta-2-glycoprotein and lupus anticoagulant, all of which were entirely normal, except for a factor V Leiden mutation. Fasting blood lipids, including cholesterol, low-density lipoprotein (LDL) cholesterol, high-density lipoprotein (HDL) cholesterol and triglycerides, were normal. Lipoprotein A was elevated at 99 (<75 nmol/l). Two covid-19 polymerase chain reaction (PCR) tests of nasal swabs were negative on two separate occasions, five days apart. Based upon these findings, a diagnosis of a cryptogenic ischemic infarct was made.\nAlthough an ischemic infarct in a 15-year-old boy is significant enough to warrant an exhaustive evaluation for a specific etiology and may in itself constitute a case report, this is not the focus of this article. This case report highlights the phenomenon of Zeitraffer and localizes this phenomenon for the first time to a circumscribed lesion of the banks of the POs, which is in keeping with the literature, specifically an interruption of the dorsolateral visual stream. In our case report, we have a specific strategically localized lesion along this pathway. Other reports emphasize lesions of V6, MIP, the superior parietal lobule and en mass lesion of the whole area []. |
A 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. He had a medical history of hypertension and hyperlipidemia and a surgical history of the right inguinal hernia. The patient's laboratory findings were within normal limits. Abdominal computed tomography (CT) revealed a well-demarcated oval isodensity mass of 25 mm at the tip of his appendix. Contrast-enhanced CT revealed a lesion with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase (). No abnormal findings were found in the root to the middle of the appendix. Abdominal ultrasonography (US) revealed a well-demarcated hypoechoic tumor. The tumor size was 22 mm × 18 mm × 18 mm, with some cystic area and blood flow (). Colonoscopy findings were normal. The patient's symptoms naturally alleviated during examination period.\nPreoperative diagnosis indicated appendiceal neuroendocrine tumor (NET) G1 or gastrointestinal mesenchymal tumors, such as GIST. Malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was recommended. Intraoperative findings revealed a well-demarcated tumor at the tip of the appendix, with no invasion into the surrounding tissue. This observation was similar to the preoperative imaging findings. According to another intraoperative finding, dissecting the adhesion between the terminal ileum and the peritoneum, which was the effect of the past herniorrhaphy, was necessary. The operation time was 167 min, and the amount of blood loss was 100 ml.\nPathological findings revealed a well-demarcated tumor originating from the muscular layer at the tip of the appendix and spindle-shaped heterotypic cells proliferating in a bundle. Vascular invasion and lymph duct invasion were not detected. No tumor cells were found in the dissected lymph node. Immunohistochemical studies revealed negative values for KIT and CD34 and positive values for S-100 protein (), which confirmed the schwannoma of the appendix. The patient was discharged on the 9th day after surgery without any complication requiring medical treatment. The patient is presently doing well without any evidence of recurrence at 3 months after surgery. |
A 64-year-old man was referred to our hospital for a middle mediastinal tumor detected incidentally by a CT scan. Chest CT showed an entirely cystic mass with a thick capsule slightly enhanced in the middle mediastinum between the bilateral brachiocephalic vein and trachea (Fig. ). At CT scan, the thymus is of normal size and located in the anterior mediastinum as a low-density triangle area. The mass had no solid component. T2-weighted MRI revealed that the main tumor had a heterogeneous isodense signal intensity and that the tumor was encapsulated by a low-signal area (Fig. ). There was no gadolinium-enhanced area in this tumor. This radiologic finding indicated the possibility of the mass being a hemorrhagic cyst, bronchogenic cyst, neurogenic tumor, or teratoma, with a small proportion of fat component.\nThe patient was placed in the left lateral decubitus position, and a right thoracic approach with three-port video-assisted thoracoscopic surgery (VATS) was performed. The tumor was surrounded by the trachea, right main bronchus, brachiocephalic artery, superior vena cava (SVC), and left brachiocephalic vein. It was greatly adherent to the lateral trachea, right main bronchus, and recurrent nerve. Due to the complicated adhesiolysis in the anterior brachiocephalic artery and tumor involving the recurrent nerve, we decided to change the procedure to open thoracotomy. The tumor and recurrent nerve whose length was about 10 mm were removed while keeping them encapsulated. The tumor measured 45 × 35 × 30 mm and contained red–brown necrotic tissue surrounded by a fibrous capsule. Cross-sectional slices showed a small solid component (8 mm). The tumor nodule existed along a fibrous capsule multiply (Fig. ).\nMicroscopic findings showed a proliferation of markedly atypical polygonal epithelial cells having hyperchromatic nuclei associated with extensively necrotic and hemorrhagic areas (Fig. , ). The recurrent nerve was involved with the carcinoma cells. Immunohistochemically, the carcinoma cells were positive for AE1/AE3, CAM5.2, p40, p63, CK34betaE12, CD5, and bcl-2 but negative for CK5/6, TTF-1, c-kit, AFP, and CD30. This feature indicated poorly differentiated, thymic squamous cell carcinoma in pathological T3N0M0 stage III. The margin of the resected tumor was free of disease.\nAdjuvant concurrent chemoradiotherapy was performed. We administered monthly carboplatin plus paclitaxel for four courses, and the radiation therapy dose was 50 Gy. There was no recurrence 6 months after surgery. |
A 28-year-old woman was screened for LFS after her 10-year-old daughter tested positive for germline TP53 gene mutation while undergoing evaluation for newly diagnosed sarcoma. She was found to carry familial heterozygous missense TP53 gene mutation in exon 7 causing nucleotide change c.733G>A with corresponding amino acid change p.G245S in the gene protein. Her two other children from the same partner, aged fourteen and four years, also carried the same mutation. Her partner did not carry the mutation and she had no family history of cancer.\nHence she was enrolled in the high-risk cancer screening program, eighteen months later, she presented with a cough with white sputum and upper back pain. Symptoms did not improve with the course of antibiotics, therefore, she underwent further evaluation. Computed tomography (CT) scan of the chest (Figure , left panel) showed a right upper lobe mass. Bronchoscopy and transbronchial biopsy of this mass were diagnostic of lung adenocarcinoma with EGFR exon 19 deletion mutation, clinical stage was T3N2M1, stage IV. Staging positron emission tomography (PET) scan revealed fluorodeoxyglucose uptake in the osteoblastic lesions involving the thoracic spine. Evaluation by neurosurgery deemed the patient not a candidate for surgical intervention, therefore she was treated with external beam radiation therapy of 30 Gray to the thoracic spine in ten fractions for impending cord compression.\nAs the tumor was EGFR mutated, treatment with erlotinib 150 mg by mouth once daily was recommended. Within two weeks of the treatment, she reported improvement in cough and back pain. Later, she developed flares of moderate cutaneous toxicity (as defined under international EGFR inhibitor dermatological toxicity grading system) to erlotinib. It was treated with the periodic use of topical corticosteroids, topical urea, and two-week courses of doxycycline 100 mg capsules twice daily. Besides cutaneous toxicity, she had no other significant adverse effects from erlotinib. Follow up CT scan six months later showed (Figure , right panel) response evaluation criteria in the solid tumor, RECIST 1.1 partial response.\nTwelve months later she complained of unremitting left hip pain causing her to limp. CT scan of the left hip (Figure , left panel) revealed a new lytic lesion. Restaging CT of the chest showed new lung nodules (Figure , right panel) consistent with progressive disease. Peripheral blood analysis tested positive for T790M. She was referred for palliative radiation to the left hip while further treatment option with osimertinib in light of T790M positivity was being pursued. |
A 50-year-old, gravida 2, para 2 perimenopausal woman presented with abnormal uterine bleeding requiring transfusion. The patient's past medical history is significant for anemia and psoriasis with an Eastern Cooperative Oncology Group performance status of 0. She had a recent negative pap smear 3 years prior and no history of abnormal pap results. Gynecologic consultation revealed a large, firm, bleeding, 6 cm mass in the cervix without clear evidence of parametrial or vaginal involvement. Biopsy under colposcopy confirmed high-grade invasive cervical adenocarcinoma. Transvaginal ultrasound showed a cervical mass with increased color Doppler flow and hypoechoic components. Computerized tomography of the abdomen and pelvis demonstrated a large heterogeneous mass at the cervix measuring 5 × 5 × 7 cm in size, with no obvious lymphadenopathy or distant disease.\nThe pretreatment biopsy () contained sheets of malignant glandular cells. At higher power, the majority of tumor is composed of signet-ring adenocarcinoma cells (, arrow), so named because the mucin-filled cytoplasm displaces the nucleus to the periphery of the cell giving the appearance of a signet ring. This is a highly unusual variant of cervical mucinous adenocarcinoma. An immunohistochemistry stain for P16, a marker for high-risk human papillomavirus, was strongly positive consistent with the diagnosis of cervical primary.\nAt this point, it was recommended that the patient undergo external beam radiation therapy with chemosensitization followed by a brachytherapy boost. She underwent a course of concurrent chemoradiation with weekly cisplatin to the pelvis: 4500 cGy in 25 fractions followed by 5 fractions of high-dose brachytherapy. Brachytherapy consisted of 600-670 cGy per fraction to the high-risk clinical target volume (HRCTV) prescribed to cover 90% of the target (D90). The use of real-time MRI guidance on 2 fractions, and combined intracavitary/interstitial techniques, resulted in significant tumor shrinkage during brachytherapy that was evident between fractions 1 and 4 ().\nOn pelvic exam at a one-month follow-up after brachytherapy completion, it was noted that the patient had a 1.5 cm, firm, nodular mass within the cervical canal suspicious for residual tumor. The cervix and uterus were freely mobile with no involvement of parametrial tissue, vaginal extension, anterior extension towards the bladder, or posterior extension towards the rectum. The tumor, cervix, and uterus were determined to be resectable with only minimal required resection of the paracervical tissue and vagina, and not necessitating a radical or exenterative approach []. A PET/CT revealed a standardized uptake value (SUV) of 13.97 in the cervix consistent with residual tumor, as seen in . There remained no evidence of metabolically active lymph nodes or distal metastases.\nAfter a multidisciplinary discussion of the institutional dedicated gynecology oncology tumor board which included specialists in gynecology oncology surgery, chemotherapy, and radiation, the options reviewed included adjuvant hysterectomy or continued close observation. The risks of adjuvant resection after chemoradiation were discussed and reviewed with the patient, including risk of poor wound healing and potential loss of function injury to bladder, rectum, and/or bowel. Close observation while having the possible benefit of not requiring further intervention and allowing further tumor regression was not favored by the tumor board or patient preference, given the high clinical suspicion of residual tumor that may ultimately require pelvic exenteration. The patient underwent adjuvant hysterectomy in the form of an exploratory laparotomy, total abdominal hysterectomy, and bilateral salpingo-oophorectomy. The hysterectomy specimen revealed residual cervical tumor measuring up to 1.6 cm in greatest dimension. Sections through this tumor demonstrated a mixture of conventional mucinous adenocarcinoma () along with residual signet-cell adenocarcinoma (, arrow). The relative predominance of the conventional mucinous adenocarcinoma in this specimen may represent a posttreatment alteration in the tumor.\nAt a 3-month postsurgery visit, the patient had no complaints, is without residual pelvic pain, and has maintained normal bowel and bladder function. Twelve months postsurgery, she continues to show no evidence of disease and the patient continues to feel well with no complaints. |
An asymptomatic 44-year-old man presented to Matsushita Memorial Hospital to seek medical follow-up after moving. The patient was found to have an abnormal electrocardiogram (i.e. left ventricular hypertrophy) at a medical checkup several years ago and was diagnosed with HCM at another hospital. He reported to have received bisoprolol 5 mg per day, which was discontinued for his preference. His previous medical history was otherwise unremarkable. Cardiac auscultation at the apex showed a fourth sound (S4), a grade 3 systolic ejection murmur, and a third heart sound (S3). The remainder of the examination was normal. There was no family history of HCM or premature sudden death.\nEchocardiography showed myocardial hypertrophy in the interventricular septum, anterior and lateral walls, and the apex of the left ventricle (). The left ventricular ejection fraction was 71%, the left atrium dimension was 38 mm, the peak transmitral E wave velocity and the deceleration time of the transmitral E wave were 0.73 m/s and 153 ms, and the transmitral E/A ratio and E/early diastolic mitral annular velocity ratio were 2.35 and 20.85, respectively. Mid-ventricular obstruction with a peak velocity of 3.2 m/s was observed in the left ventricle, along with a diastolic paradoxic jet flow (). Of note, right ventricular hypertrophy was also suspected, and a tiny flow from the right ventricular apex to the base during early diastole was detected (). The unique flow started approximately 110 ms after the onset of the second heart sound (S2) and lasted to the end of the S3 or for almost 95 ms (). The onset was likely to be later than the diastolic paradoxic jet flow in the left ventricle, and the duration seemed similar to that of the left ventricle, although the tiny flow was difficult to assess by pulsed Doppler echocardiography.\nCardiac magnetic resonance revealed myocardial hypertrophy in the ventricular septum, the right ventricular free wall, and left ventricular anterior and lateral walls (). Prominent trabeculation was not present in the left ventricle as well as in the right ventricle. Extensive late gadolinium enhancement was detected mainly in the areas of myocardial hypertrophy. Medical treatment including beta-blockers or anticoagulants was recommended but declined by the patient. He had been doing well without any medication for months and later lost to follow-up. |
A 65-year-old male presented to our department with a 3-month history of slowly growing prominence of the right eye. On physical examination, the right eye showed painless proptosis without diplopia. Ocular motility of the right eye was mildly limited both in lateral and downward directions. Hertel exophthalmometer values of the right eye and left eye were 23 mm and 13 mm, respectively. Visual acuity measured with Snellen chart was 0.4 for the right eye and 0.8 for the left eye. Anterior segment examination revealed a clear cornea with grade 1 nuclear sclerosis in both eyes. Fundus examination showed choroidal folds in the right eye.\nOrbital magnetic resonance imaging revealed a well-defined mass measuring 32 mm × 31 mm × 25 mm in intraconal region of the right eye, which was isointense relative to the adjacent soft tissue in T1 signal and hyperintense in T2 signal [Fig. and ]. There was no sign of bone erosion or infiltration to adjacent soft tissues. Lateral orbitotomy through upper lid skin crease incision was performed to gain access to the posterior orbit. The dissection extended through orbicularis muscle and deep fascia to the periosteum of the orbital rim. Periosteum along the lateral orbital rim was cut and elevated from the lateral orbital wall. The bone was cut with an oscillating saw, angling the cut slightly inferiorly and parallel to the orbital roof. The cut was made into the thin bone along the sphenozygomatic suture line. A second cut was made through the orbital rim just above the zygomatic arch. Small holes were drilled on either side of each cut near the rim to facilitate later replacement of the bone. The bony rim fractured outward. The periorbita was then opened. A well-circumscribed, encapsulated, and highly vascular lesion was seen. To improve exposure, inferior subciliary approach for inferior orbitotomy was performed following canthotomy and cantholysis. Then, the tumor was freed up entirely with blunt dissection and removed in total []. Macroscopically, the tumor was ovoid, highly vascular, white-gray with smooth borders []. Microscopic examination revealed richly vascular, well-circumscribed, patternless spindle-shaped cells arranged in sheets. Floret-like multinucleated giant cells often lined the pseudovascular spaces []. Immunohistochemistry was performed; spindle cells as well as the giant cells showed reactivity for Vimentin, CD34, and bcl-2 []. Neural crest marker S100, desmin, KI67 and ALK-1 were all negative. The morphology and immunohistochemical profile was consistent with the diagnosis of GCA. Immediate postoperative period was uneventful [Fig. and ]. Proptosis regressed completely with recovery of full extraocular motility. No recurrences were detected over 2-year follow-up with good cosmetic and functional results. |
A 66-year-old woman was referred to colonoscopy for a positive fecal occult blood test which was performed in the national colorectal cancer screening program. Her medical history was unremarkable. She denied prior overt gastrointestinal bleeding or other gastrointestinal symptoms. Her laboratory tests were unremarkable, including the levels of CEA and CA 19.9 which were normal. Colonoscopy revealed a 70-mm-wide sessile polyp in the rectum located 5 cm from the ano-cutaneous verge. A total of 5 mL of SPOT® endoscopic marker was injected circumferentially around the polyp base. Pathological examination of the colonoscopic biopsies revealed tubulovillous adenoma with low-grade epithelial dysplasia and surgical resection was recommended due to its size. As a part of the preoperative planning, the patient underwent magnetic resonance imaging (MRI) of the pelvis that was performed 3.5 months after the colonoscopy. MRI showed a 7 cm large tumor formation of the rectum with restriction of diffusion and focus of the probable invasive growth with penetration of the whole thickness of the bowel wall and incipient infiltration of the mesorectal fat (Fig. ). Mesorectal fascia was intact and the lymph nodes were unsuspicious (Fig. ). There were no signs of vascular invasion present. The radiological report was signed out as a large adenoma with probable cT3N0 rectal carcinoma. Since pathological and radiological reports were inconsistent, the patient was introduced to a multidisciplinary team consisting of gastroenterologist, radiotherapist, and surgeon. Since the lesion was large, reliability of repeated colonoscopic biopsies could be questionable in such a case and MRI showed signs of mesorectal invasion, short regimen irradiation (5 × 5 Gy) of the affected rectal segment was performed and was followed by surgical resection 4 days after the completion of radiotherapy.\nPathological examination of the resection specimen revealed a tubulovillous adenoma with high-grade dysplasia and a focus of invasive adenocarcinoma (Fig. ) that penetrated the lamina muscularis mucosae to a depth of only about 1.5 mm. There was no vascular invasion, lymphangiosis or perineural invasion. There were no areas of fibrosis after tumor regression. However, there were areas of fibrosis combined with abundant deposition of coarse clumps of black pigment, corresponding to the carbon-based dye that was previously used as a marker during the endoscopic procedure (Fig. ). The pigment was partially extracellular and partially engulfed by macrophages that formed numerous foreign body type granulomas (Fig. ). The area of dye deposition spanned the entire thickness of the rectal wall and focally involved some areas of perirectal fat (Fig. ). The macrophage infiltration was accompanied by a mild lymphocytic infiltrate and there were no granulocytes. |
A 67-year-old female with history of chronic tobacco use, chronic obstructive pulmonary disease, hypertension, and hyperlipidemia, presented to the ED with symptoms of TIA. The patient described the acute onset of left-sided facial weakness that waxed and waned, recurring several times throughout the day, and lasting 2–3 minutes at a time. The left facial weakness was also associated with mild, left-arm weakness and “clumsiness” involving fine motor function of her left hand. She noted lightheadedness but denied leg weakness, headache, visual changes, chest pain or shortness of breath. She also noted that symptoms were brought on by use of her upper extremities and when she changed her body position from lying to sitting. She denied any similar symptoms previously or stroke history. Of note, she noticed a rapid improvement in her symptoms to resolution just prior to ED presentation.\nOn examination, her blood pressure (BP) was 183/86 millimeters of mercury (mmHg). She was awake, alert, oriented, and able to describe a detailed history. Her cranial nerves were intact, motor strength was 5/5 bilaterally, and fine motor movements in both her hands were normal. There was no ataxia, extraocular muscle dysfunction, or indication of posterior circulation involvement.\nJust after her initial asymptomatic presentation to the ED, her symptoms recurred when her systolic BP dropped by 20 mmHg upon standing from a supine position. Emergent computed tomography angiogram (CTA) of the head and neck demonstrated a severe flow-limiting lesion of the innominate artery (). Further investigation with magnetic resonance imaging demonstrated decreased signal intensity within the right internal carotid artery at the cavernous sinus and petrous segments, a finding that potentially represented slow flow ().\nThe patient subsequently underwent emergent cerebral angiogram, which demonstrated occlusion of the proximal innominate artery () at the aortic arch with resultant left to right vertebral artery steal phenomenon supplying the right subclavian artery (). The distal brachiocephalic artery flow was reconstituted via the subclavian artery and secondary steal phenomenon occurred into the right common carotid artery, causing delayed flow to the right cerebral hemisphere ().\nThe patient was maintained on a norepinephrine bitartrate infusion to increase BP, and her symptoms subsequently resolved. The symptoms recurred when she was positioned supine, but upon being placed in the Trendelenburg position her symptoms again resolved. The patient was therefore maintained with systolic BP goals between 160 and 210 mmHg. She remained asymptomatic during this period of elevated BP management. For definitive care, she underwent elective left carotid to right carotid “necklace” bypass surgery with complete and permanent resolution of her symptoms. |
In 2006, a 73-year-old Caucasian man presented with nodular lesions on the left chest wall while receiving mitoxantrone and prednisolone for metastatic hormone refractory prostate cancer with skeletal metastases. At the time, mitoxantrone was the standard systemic chemotherapy for this condition, this gentleman was first diagnosed with prostate cancer 9 years previously. Past treatments included androgen deprivation, strontium, carboplatin–etoposide, radiotherapy to the chest wall for pain in the manubrium and sternum; and also with cisplatinum–phenoxidiol (as part of a phase 1 clinical trial). He initially responded well to mitoxantrone with a fall in the serum prostate specific antigen (PSA) and resolution of bone pain.\nAfter the eighth cycle of mitoxantrone, the patient reported having skin lesions on his chest. The lesions were non-tender, pink, raised and occurred in irregular clusters just lateral to the nipple towards the mid axillary line, there were no epidermal changes. A clinical suspicion of cutaneous metastases was considered along with the differential diagnoses of a drug eruption and other dermatoses.\nA punch biopsy was performed on a sample lesion. The pathology showed metastatic adenocarcinoma in the dermis that stained for cytokeratin, PSA and prostatic acid phosphatase (PAP) consistent with prostate cancer (Figures and ). He was also found to have a lower leg deep vein thrombus and was commenced on enoxaparin then warfarin.\nChemotherapy was ceased due to progressive disease, thereafter, serum PSA levels continued to rise. The PSA increase coincided with increasing extent, size and pain in the nodules on the chest wall. The lesions close to a previous sternal ulcer became friable and bled easily (Figure ). Two months from when the lesions were first noticed, palliative radiotherapy of 18 Gy in 3 fractions using electrons was delivered to the left chest wall to good clinical effect (Figure ).\nThe PSA continued to rise, however, and further symptoms developed including fatigue, leg swelling and dyspnoea. A computed tomography (CT) scan revealed bilateral pleural effusions, the larger effusion on the left was drained. The cytology of the fluid revealed adenocarcinoma cells staining with anti-cytokeratin antibody CAM 5.2, PSA and PAP consistent with metastatic disease.\nThe treated cutaneous lesions did not cause further problems but new lesions were found out of the field of radiotherapy treatment (Figure ). He continued to decline and required a second pleurocentesis. At this time there were also changes on CT consistent with pulmonary lymphangitis, he subsequently died in hospital 2 weeks later. |
A 21-year-old male without any notable past medical history presented to our hospital with numbness in the left upper extremity. Symptoms emerged a few months before the patient presented at our hospital and had been gradually deteriorating. Upon admission, a neurological examination revealed bilateral abducent palsy with partial impairment of adduction in the right eye when the patient looked to the left (one and a half syndrome), dysphagia, and left-sided hemiparesis, dysesthesia, and ataxia. His family history, including schwannomas and meningiomas, was unremarkable. Brain magnetic resonance imaging (MRI) showed a 4-cm mass in the dorsal pontomedullary area adjacent to the fourth ventricle (Fig. a–f). The contrast-enhanced mass was accompanied by a large cyst behind the solid component. No contrast enhancement was observed on the cyst wall. There were no other lesions such as vestibular schwannoma or spinal schwannoma in this patient. With 18F-fluorodeoxyglucose positron emission tomography, uptake in the lesion was non-significant, indicating minimal or no malignancy. A thorough survey of malignancy in the trunk did not reveal any lesions. Preoperative diagnostic angiography did not show tumor staining, which suggested a differential diagnosis of pilocytic astrocytoma. The fourth ventricle partially collapsed due to the mass effect, and signs of intracranial hypertension caused by obstructive hydrocephalus gradually emerged; thus, urgent tumor resection was planned.\nThe location of the motor and sensory tracts was confirmed by tractography with diffusion tensor imaging and Q-ball imaging prior to surgery. The motor and sensory tract was anteriorly and laterally compressed by the tumor, respectively. An approach from the posterior side was planned, based on the results of tractography (Fig. a, b).\nA midline suboccipital craniotomy with a transcerebellomedullary fissure approach was performed. Immediately after the craniotomy, an external ventricular drain was inserted. The posterior wall of the cystic component was visible along the midline of bilateral facial colliculi (Fig. c). After suction of the yellowish liquid from the cyst, the anterior solid component of the tumor was identified.\nBradycardia and hypotension occurred during removal of the ventrocaudal component of the tumor, which might have been due to stimulation of the dorsal nucleus of the vagus nerve. Thus, the procedure was terminated, resulting in incomplete removal of the tumor.\nA tracheotomy was performed immediately after tumor removal to prevent postoperative respiratory adverse events. Obstructive hydrocephalus resolved after tumor resection, and external ventricular drainage was removed on postoperative day (POD) 10. The ventrocaudal residual mass was followed up with gadolinium-enhanced MRI (Fig. g). The image on POD 29 showed slight enlargement of the contrast-enhanced area. The radiographical change was initially regarded as a postoperative inflammatory reaction, but MRI performed on POD 43 showed apparent growth of the solid component (Fig. h). Local fractionated irradiation was initiated at 50 Gy/25 Fr from POD 50. Subsequent follow-up MRI demonstrated no tumor regrowth. Three months after surgery, the tracheostomy was closed, and the patient was transferred to a rehabilitation facility. Left-sided facial paralysis and abducent palsy persisted even after discharge from the facility, but left-sided hemiparesis improved. Currently, no tumor regrowth has been observed 4 years after surgery (Fig. i). |
We present a 77-year-old male with a past medical history of NHL/SLL diagnosed almost 10 years ago, who presented to the hospital with abdominal swelling, altered mental status, and difficulty in urinating associated with hematuria. On physical examination, diffuse bulky lymphadenopathy was found in the cervical, axillary, and inguinal areas. Detailed oncologic history and treatment regimens that were taken by the patient have been well explained in Table .\nLaboratory workup showed hypokalemia, hypophosphatemia, and elevated lactate dehydrogenase levels. During the hospitalization, computed tomography (CT) scan head was done that showed negative findings for any acute events. Due to altered mental status with underlying worsening of NHL and metabolic disturbances, the toxic and metabolic encephalopathy were the differentials under consideration. Metabolic derangements were corrected during hospitalization and that improved his mental status as well. Idelalisib treatment was discontinued abruptly a week prior to patient’s presentation to the hospital due to pancytopenia and a mixed response on the CT scan imaging. Upon admission, a repeat CT of the abdomen and pelvis showed diffuse bulky lymphadenopathy in the abdomen; one of the nodes in the anterior para-aortic region was measured about 5 cm × 5 cm × 8 cm (Figure ). Bilateral iliac, inguinal, and retroperitoneal lymphadenopathy was also significantly increased in size compared with prior CT scan. There was diffuse lymphadenopathy along with axillary and cervical regions as well (Figures -).\nBone marrow biopsy was done that showed a cluster of differentiation 5 (CD5) and CD23 positive B-cell population (37% of the lymphoid gate), lambda-restricted. Almost all of the B-cells showed immunophenotypic expression of CLL/SLL with lambda light chain restriction that was found in previous cases of this patient. Interestingly, a kappa light chain restricted population of monoclonal plasma cells co-expressed with CD56 (1.1% of total events) is also identified. Urology was consulted for urinary complaints of difficult voiding and hematuria. However, the patient was further diagnosed with paraphimosis and scheduled to have an elective circumcision that alleviated his urinary complaints later. Idelalisib treatment for SLL/NHL that patient took for almost one year was stopped a week prior to current clinical symptoms. Based on progressive SLL/NHL, the patient was started on venetoclax (B-cell lymphoma 2; BCL-2 inhibitor) 20 mg/day for seven days and 50 mg/day afterward. The patient did not develop any tumor lysis syndrome after starting the therapy and is tolerating the treatment well based on both imaging and clinical finding. |
A 28-year-old previously healthy female patient has attended our surgical clinic complaining of a chronic dull aching left-sided abdominal pain and mild abdominal distension for 1 year. During the course of investigations, she had an abdominal and pelvic CT scan and was found to have a 16.8 × 16 × 20 cm solid and cystic mass in the left retroperitoneum in the suprarenal area containing bone and multiple soft tissue densities (). The tumour had calcifications, was not well enhanced, and was without any evidence of distant metastasis. Further assessment was performed using abdominal magnetic resonance imaging (MRI) (). Preoperatively, we diagnosed the tumour as having originated in the left adrenal gland, because the normal adrenal gland could not be recognised by CT or MRI. Tumour markers, such as serum alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), lactate dehydrogenase (LDH), carcinoma antigen 125 (CA 125), neuron-specific enolase (NSE), and carbohydrate antigen 19-9 (CA 19-9), were examined because we also thought it might have been possible that the tumour did not originate from the adrenal gland.\nThe patient underwent resection of the lesion through a midline laparotomy approach. The retroperitoneal dissection was tedious and difficult, but the mass was excised in its entirety (). The macroscopic examination revealed a well-circumscribed mass measuring 230 mm × 200 mm × 120 mm, with intact surface. The specimen weighed in excess of 2 kg. The capsule was intact and the residual adrenal gland was noted on the external surface measuring 60 × 45 mm and was distinct from the mass. On slicing the specimen, the mass was completely filled with sebaceous-like material with identified hair and protuberances. On slicing, bony areas were noted.\nMicroscopically, the mass was composed of a mixture of mature components including bone, squamous epithelium, glandular epithelium, and stroma including muscle with no identified undifferentiated or primitive neuroectodermal tumour- (PNET-) like elements.\nAdjacent to the tumour was a normal adrenal gland, which appeared to be distinct from the tumour. Ovarian tissue was not seen. Adjacent paraganglial tissue and ganglioneuronal tissue were also noted. There were no malignant features and the tumour appeared completely excised.\nThe conclusion of the histopathological examination was that of a mature teratoma, which might have arisen in the retroperitoneum and become attached to the left adrenal gland.\nBecause these tumours usually represent metastasis from other primary sites, additional imaging with CT of the chest and breast and ovarian ultrasonography was performed. No other primary tumour was identified. Therefore, we diagnosed the mass as a primary retroperitoneal teratoma. The patient is currently under clinical and imaging follow-up, to identify any future possible recurrence. |
A 69-year-old male patient presented to our hospital due to visual loss and metamorphopsias on his left eye. Best corrected visual acuity (BCVA) at presentation was 9/10 in his right eye and 9/10 in his left eye with his hyperopic correction (+1.00 sph +0.50 cyl 180, OD, and +0.75 sph, OS). Slit lamp examination of the anterior segment and ocular motility examination did not reveal any significant abnormality. Fundus examination showed the presence of yellowish deposits (fleck-like) in the parafoveal area bilaterally and a grayish subretinal membrane was observed on the temporal side of the fovea of the left eye. Fundus autofluorescent images showed hyperautofluorescent deposits and a mottled loss of autofluorescence in the temporal parafoveal area of the left eye (). An occult choroidal neovascular membrane of the left eye was observed on fluorescein angiography that was subsequently obtained (). Optical coherence tomography scans of the macula showed focal RPE distortion on the right eye and the presence of intraretinal cysts and subretinal fluid on the temporal aspect of the fovea of the left eye (). The working diagnosis was that of a patient with the reticular type of pattern dystrophy, complicated by a leaking occult choroidal neovascular membrane of his left eye.\nDetailed information regarding the nature of his condition and the therapeutic options was given to the patient, but since the central visual acuity was not severely affected, he elected to be observed rather than receiving anti-VEGF intravitreal treatments. Repeat ophthalmic examination and OCT testing, 6 months later, revealed complete resolution of subretinal fluid and restoration of foveal architecture of the left eye (). Mean central macular thickness of the left eye decreased from 298 μm to 266 μm (at 6 months), while macular thickness at the temporal side of the fovea (that showed thickening at the initial exam), decreased from 85 μm to 69 μm, respectively, compared to the baseline values (). Baseline BCVA remained unchanged at 9/10 in both eyes in all follow-up visits. Repeat examination and OCT scan at 12-month follow-up visit showed a stable clinical picture and absence of subretinal and intraretinal fluid on OCT scans (). |
A 42-year-old male was referred to his ophthalmologist regarding the complaint of sudden blurriness of vision in his right eye. He was diagnosed five months ago with a case of ENKTCL, nasal type, stage IV-B with testicular and central nervous system involvement with 23% natural killer (NK) cells on cerebrospinal fluid (CSF) flow cytometry. He was on an active therapeutic plan consisting of cycles of chemotherapy (i.e., SMILE protocol: dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide), to which there was a complete response in the form of negative flow cytometry and computed tomography (CT) of the chest, abdomen, and pelvis. Five months after the initial diagnosis, prior to the fourth cycle of treatment, a complaint of blurriness of right eye vision was made. Brain CT was done aiming to rule out ocular involvement, which turned out to be unremarkable. At the time of the complaint, visual acuity was 20/30 -2 in the right eye as opposed to 20/20 in the left eye. The right eye showed relative afferent pupillary defect. Extraocular muscle movements were competent in both eyes. On examination of the anterior chamber, +1 cells were visualized in the right eye only. On dilated fundus examination, there was vitritis in the right eye which obscured the vision. Left eye examination was insignificant. B scan ocular ultrasonography revealed retinal detachment in the right eye. An optic CT revealed vitritis in the right eye; an impression of disease infiltration of the eye was made. The therapeutic plan was a diagnostic vitrectomy followed by systemic therapy. A pars plana vitrectomy was made and the vitreous specimen was sampled revealing the presence of 10% viable lymphoid cells expressing CD2 and CD56 on immunohistochemistry stain. An orbital magnetic resonance imaging (MRI) was ordered revealing evidence of an enhancing retinal lesion centered on the optic disc with diffuse restriction consistent with lymphomatous infiltration; minimal proptosis of the right eye was noted (Figure ). Fundus photography of both eyes was performed postoperatively with the retina flat under the silicon in the right eye as seen in Figure and minimal disease involvement of the left eye which further confirmed the diagnosis (Figure ). In addition, CSF flow cytometry revealed immunophenotypic evidence of disease (75% mature T cells and 20% NK cells expressing CD2, CD16 and CD56). Follow-up examination revealed 6/9 vision in the left eye, as opposed to no light perception in the right eye. The patient received 35 Gray units of radiotherapy to the optic apparatus and posterior globe of both eyes and the entire cranium down to the third cervical spine region. Post-radiotherapy MRI revealed interval regression of the disease in the form of resolution of bilateral optic disc infiltrative nodule. Despite medical efforts, the patient’s condition deteriorated, and he passed away. |
A 27-year-old male patient presented with blurred vision and floaters in the right eye for three days with no improvement. His ophthalmological history was clear, while his medical history included sickle cell SC type hemoglobinopathy.\nAt presentation, his best corrected visual acuity (BCVA) was 6/18 in the right eye and 6/6 in the left eye, intraocular pressure was 13 mmHg in both eyes, and anterior segment slit-lamp examination was also unremarkable in both eyes. Dilated fundoscopy revealed vitreous hemorrhage in the right eye (stage IV proliferative sickle cell retinopathy) and normal left eye. The patient was counselled on the nature of the problem and referred to the medical retina department of our hospital. A fluorescein angiography was performed to evaluate the extent of retinopathy, confirming leakage from the sea fan neovascularization in the right eye, with ischemia in the periphery (). In the left eye, only small ischemic areas in the temporal periphery were detected (). Optical coherence tomography (OCT) was normal in both eyes (Figures and ).\nTreatment alternatives were discussed with the patient and he was offered an off-label intravitreal ranibizumab injection in the right eye. Written informed consent was obtained from the patient for off-label use of ranibizumab, after explaining the potential complications of such a treatment. After placement of a sterile lid speculum and topical 5% iodine povidone, an intravitreal injection of 0.5 mg ranibizumab was administered in the right eye 4 mm posterior to the limbus using a 30-gauge needle.\nOne week after injection, his BCVA was 6/9 in the right eye and fundus examination demonstrated improvement of vitreous hemorrhage, as well as regression of the sea fan neovascularization, confirmed by fluorescein angiography (). One month after injection, the BCVA was 6/6 in both eyes, the vitreous hemorrhage was totally absorbed, and the retinal neovascularization further regressed (). Scatter laser photocoagulation was also applied to the area of nonperfusion in the temporal periphery of the right eye. Three months later, there was no recurrence of the neovascularization, as it is depicted on fluorescein angiography () and the BCVA was 6/6, remaining stable at the 9-month follow-up (), while no adverse events were observed. |
A 57-year-old right-handed female presented with a 4-year history of right leg weakness with equinovarus, and a reduction in grip strength in the right hand. Weakness was such that she had to lift her right leg in and out of her car with her hands. The following year, she began to experience numbness in the right hand as well as low back pain and urinary urgency. A course of intravenous methylprednisolone provided no benefit. Her condition slowly progressed but remained unilateral after 18 years, with no evidence of bulbar dysfunction. There have been no persistent sensory symptoms, though she has complained of cold extremities and acrocyanosis.\nThe patient was an ex-smoker. Her only past medical history of note was of curative (local) treatment for ductal breast carcinoma (11 years after onset of neurological symptoms). There was no family history of neurological disease.\nThe gait was spastic and hemiparetic, but ambulation was unaided. There was a pyramidal catch in the right upper limb and obvious spasticity in the right lower limb. Mild pyramidal weakness (Medical Research Council (MRC) grade 4) and hyperreflexia were noted in the right upper and lower limb.\nThere was an asymmetrical spastic paraparesis, worse on the right, and requiring a frame to ambulate. There were early flexion contractures of the fingers in the right hand with marked hypertonia in the right upper and lower limbs. Pyramidal weakness was noted in the right upper (MRC grade 4 proximally and grade 3 distally) and lower limb (MRC grade 3). Pathological hyperreflexia was now also evident in the left lower limb, but the left plantar response was flexor whereas the right was extensor.\nThe patient had begun using a wheelchair after fracturing the right radius and ulna in a fall, and had been catheterised due to impaired mobility. She had evolved significant amyotrophy in the right hand and forearm. |
A 32-year-old Kashmiri woman was referred to our hospital with chief complaints of hemoptysis, cough, and fever of 2-year duration. She was diagnosed as pulmonary tuberculosis and took antitubercular treatment for 6 months. But gradually she started developing chest pain and dyspnoea. There was a past history of fibroadenoma of right breast. There was no history of nausea, vomiting, diarrhea, or weight loss. She was a nonsmoker. Laboratory investigations revealed hemoglobin of 9.0 g/dl with normal total leucocyte and platelet counts. Erythrocyte sedimentation rate was 53 mm at the end of first hour. Liver and kidney function tests were within normal range. HIV, hepatitis B virus, and hepatitis C viral serologies were negative. Chest X-ray showed consolidation and collapse of left upper lobe. Contrast-enhanced computed tomography (CECT) chest showed a well-circumscribed homogeneously enhancing mass lesion in the apical segment of left lower lobe occluding the left lower main bronchus. There was no evidence of any calcification or necrosis on CT scan []. Radiological diagnosis of pulmonary hamartoma or intrapulmonary hydatid cyst was made. Fiberoptic bronchoscopy done revealed a polypoidal growth arising from the lower lobe-left causing occlusion of left main bronchus and suggested the possibility of a carcinoid tumor. Finally preoperative diagnosis of carcinoid tumor arising from the left lower lobe bronchus involving the apical segment of the lower lobe was made. A left lower lobectomy was performed via left posterolateral thoracotomy and specimen was sent for histopathological examination. Macroscopic examination of the resected specimen revealed a whitish solid mass, 5.5 cm in maximum diameter, arising close to the bronchus and compressing the adjacent lung parenchyma. The cut surface showed mucoid material without hemorrhage or necrosis. On close examination, several small cystic spaces were noted []. Microscopically, the tumor was well circumscribed, lined by respiratory epithelium, and consisted of numerous irregularly arranged cysts, tubules, and glands lined by bland columnar, cuboidal, or flattened, mucus secreting cells. Tumor was found projecting into the bronchial lumen, however, not invading the underlying cartilage [ and ]. There was no cytological atypia or mitoses. No intermediate cells or squamous component of mucoepidermoid carcinoma was found. The intervening stroma consisted of delicate connective tissue and lymphoplasmacytic infiltrate []. The lumen contained mucus that was alcian blue positive []. The Ki-67 proliferation index was low (less than 1%). Immunohistochemistry for thyroid transcription factor-1 was negative []. Taken together, these observations led us in making a diagnosis of a bronchial mucous gland adenoma. The postoperative course was uneventful and the patient made a complete recovery. |
A 12-year-old boy of Indian ethnicity reported to the Department of Periodontics with a chief complaint of inability to move his tongue like all his other school friends. He also complained of slurred speech. History revealed that he had congenital cleft lip (not associated with cleft palate) and was surgically treated for the same soon after birth [].\nOn oral examination, the patient had thick inferior lingual frenum attached 3 mm from the tip of the tongue []. Restricted tongue movements like protrusion, lateral movements and inability to touch the palate with the tip of the tongue were observed. On protrusion, a bifid or heart shaped tip [] of the tongue was observed. According to Kotlow's classification, he was allocated under Class III “severe ankyloglossia” which accounts for the movement of the tongue between 3 mm and 7 mm. According to Hazelbaker's assessment tool, the appearance score was 2 (which was <8) and the functional score was 5 (which was <11) hence was indicated for frenectomy.[]\nA routine hematological examination of hemoglobin percentage, bleeding time, clotting time, random blood sugar, total leukocyte count and differential count was taken and was found to be within the normal range hence was planned for surgery.\nAfter application of topical anesthetic gel, adequate local anesthesia (2 ml of 0.2% lignocaine hydrochloride in 1:200,000 adrenaline) was given as infiltration around the lingual frenum. A tissue forceps was used to clamp the frenum. A soft tissue diode laser of power 1 W was used to relieve the lingual frenum approaching from either side of the tissue forceps []. No bleeding was observed on the surgical site. A 3–0 silk suture was used to approximate the dorsum of the tongue []. The patient was given adequate antibiotics and analgesics (amoxicillin 250 mg TDS and paracetamol 250 mg BD) along with postoperative instructions and was asked to report after 1-week for suture removal and review.\nThe Hazelbaker's appearance score after suture removal post 1-week was 10 and the function score was 12 [Figures and ]. The parents were instructed to consult a speech therapist for improvement in the articulation of speech.\nThe patient was called after 1-month for a review []. The Hazelbaker's appearance score and function score were 10 and 13 respectively. |
This study describes a case of a 43-year-old female with a history of traumatic brain injury secondary to a motor vehicle accident in June 2004. The patient had no history of psychiatric illness. She sustained multiple fractures, including a right-sided occipital fracture, C2 fracture, right scapula fracture and multiple rib fractures. She presented to our outpatient clinic complaining of right-sided headaches and neck and shoulder pain. She complained of pain which was 8/10 on the visual analog scale (VAS) in her neck, right shoulder and right side of the head. She described pain as a tight vice like gripping with paresthesias. The pain was alleviated by heat and massage and aggravated by increased activity and sleep. She was taking morphine 30 mg two times per day. She was followed in our clinic for 14 years and was trialed on multiple medications such as Flexeril, gabapentin and Elavil, which did not give her relief. She was also increased on her narcotic pain medications. She was placed on MS Contin 45 mg two times per day and morphine immediate-release 30 mg two times per day for breakthrough pain. This was equal to 150 morphine milligram equivalents (MME) per day. The pain was controlled with narcotic pain medications for over 10 years. She states the medications decreased her pain from 8/10 on the VAS to 4/10 on the VAS.\nOver the years, the patient expressed her concerns about becoming addicted to narcotic pain medication. On 6 April 2016, the state of Pennsylvania legalized medical cannibus, and on 15 February 2018 medical cannibus became available for patients in Pennsylvania. The patient was educated on medical cannibus as an alternative to opioid medications. We came up with a weaning protocol. We first decreased the long-acting MS Contin by 15 mg per week until she was only on morphine immediate-release. She did complain of some increased pain and withdrawal symptoms such as chills and diarrhea but was able to wean off in 1 month. Once off the long-acting narcotic medications, we began to decrease her immediate-release medications. In the next week, we decreased her immediate relief morphine from 60 mg daily to 30 mg daily or 60 MME to 30 MME. We then started her on medical cannibus. She began using the medical cannibus product called Harlequin 500 mg which had a 2:1 cannabidiol (CBD) to tetrahydrocannabinol (THC) ratio in the vape form. It had 26.3% CBD and 17% THC. These are divided into 2.5 mg doses per inhalation. She stated that with two vape inhalation per day, her pain decreased from 8/10 on the VAS to 2/10. She was able to completely wean off her opioid narcotics and reported no side effects from the medical cannibus. At her 6-month follow-up, she continued to have excellent relief. We confirmed the patient was not on any opioids with a follow-up urine drug screen at 6 months which showed no opioids in her system and was positive for cannibus. |
A 49-year-old Chinese female was admitted to the emergency department of other center for a sudden severe headache, and she was successfully referred to our institution after an enhanced CT scan and a magnetic resonance (MR) showing a round hemorrhagic mass lesion. The mass of 6 cm × 4.8 cm × 6 cm was located in the left deep frontal region with an irregular peripheral enhancement and perilesional edema determining a contralateral midline shift of 8 mm []. The clinical history revealed two previous episodes of a mild headache (2 months before) and a tubal ligation performed several years before.\nAt the admission, the patient was awake, alert, with a GCS score of 15 and a moderate right hemiparesis. No cranial nerve palsy was observed. Desametasone 8 mg twice/day, mannitol 125 ml, levetiracetam 500 mg, and pantoprazole 40 mg were administered.\nThe patient underwent a surgical intervention for the excision of the lesion 2 days later. After the dural opening, some ectatic pathological cortical veins were observed within the left upper Sylvian area, in the fronto-opercular region, close to the Broca's area. The arachnoid was dissected along the margin of the vessels and after the left frontal inferior sulcus was widened a polylobate, capsulated vascular mass was detected, which was surrounded by coagulated blood. A circumferential dissection of the capsule was performed, the mass was completely removed and the specimen sent to our pathology department for histologic examination. The postoperative computed tomography (CT) scan showed no tomographic alterations.\nThe histological examination was performed with hematoxylin and eosin section [] revealed ectatic and fragmented vascular structures. These findings were suggestive for a cavernous malformation.\nThe postoperative course was regular. No complications occurred, and the patient was discharged 6 days later with a good neurological outcome and improvement of the moderate right hemiparesis. A 2 months’ MR follow-up was performed []. |
We present the case of a 47-year-old Caucasian male who initially presented to the primary care physician 6 years ago with the chief complaint of left ear fullness of 2 weeks' duration. The patient also noticed that the left ear fullness was associated with tearing from the right eye. The patient was initially treated conservatively, with no subjective improvement in his symptoms. He underwent imaging studies which revealed a left mastoid/middle ear fossa mass and a right orbital mass (). Imaging studies were followed by left mastoid-mass incisional biopsy and right orbital debulking. Biopsy of the mastoid/middle fossa area revealed Langerhans cell histiocytosis, and biopsy of the right eye orbit revealed non-Langerhans cell histiocytosis. Staging workup including imaging studies demonstrated a sizeable mass of soft tissue density encasing the infrarenal thoracoabdominal aorta and extending caudally into the proximal bilateral iliac arteries leading to right-sided hydroureter and hydronephrosis. Based on the radiographic appearance, this mass was consistent with Erdheim-Chester disease.\nThe surgical resection of the orbital mass was followed by six cycles of vinblastine 6 mg/m2 weekly and prednisone 40 mg/m2. Thereafter, he was started on monthly pulse prednisone and daily 6 mercaptopurine doses as maintenance therapy for 24 weeks.\nThe patient was relatively stable until about three years ago when he started experiencing diplopia in the right eye. CT scans revealed extensive bilateral intraconal enhancing soft tissue, infiltrative soft tissue involving the right sphenoid wing with extension into the pterygopalatine fossa and right temporal fossa, and enhancing soft tissue identified within bilateral frontal sinuses (). Right orbitotomy and excisional biopsy of the orbital lesions confirmed relapse of non-Langerhans histiocytosis. Bone marrow biopsy demonstrated no evidence of histiocytic involvement.\nThe patient was started on PEGylated interferon alpha to be given as 180 mcg subcutaneously weekly. Repeated CT evaluations over the next 3 years demonstrated no evidence of progression of disease on PEGylated interferon alpha-2a therapy ().\nAbout 6 months ago, the patient noticed a swelling on the frontal part of the scalp. MRI brain showed interval development of left calvarial mass lesion extending intracranially, associated with an epidural soft tissue component measuring 4 × 1.5 × 1.5 cm (). The patient underwent resection of the mass with the pathology demonstrating both the skull and dural excisional specimens consistent with Langerhans cell histiocytosis with immunohistochemistry noting the cells as CD1a-positive, S100-positive, and largely CD68-negative (). BRAF mutation was not tested on the biopsy specimen. As a result of this recurrence of Langerhans cell histiocytosis, he underwent PET-CT restaging that showed a stable disease from the prior studies. The patient underwent local radiation to the scalp lesion to consolidate the resection. Besides the use of corrective lens for diplopia, he continues to do fairly well and is largely symptom free on weekly interferon therapy. |
A 24-year-old female, resident of Mexico City, with past medical history of obesity and being exposed to family members with COVID-19, presented to the ED of our tertiary care center with respiratory failure and oxygen saturation of 80%, given the findings she was managed in accordance with national and international guidelines.\nHer family reported she began with pain in the left midface region six days prior, two days later she developed progressive left lid swelling and maxillary hypoesthesia; therefore, a primary care physician suspected infection, beginning treatment with oral antibiotics (amoxicillin-clavulanate 875/125 mg twice-daily) with partial remission of pain. However, on subsequent days she continued with progression of soft-tissue edema, being referred to our center for an extended study protocol and management.\nPhysical examination revealed severe left lid edema with extension to the upper lip and malar region, left proptosis with a hyperemic conjunctiva, and an opaque cornea which precluded further evaluation. Rhinoscopy revealed edema of the left nasal mucosa with an impaired response to vasoconstriction, without evidence of necrotic tissue or purulent discharge. Samples for a direct exam were taken. The oral cavity examination only revealed pallor of hard palate mucosa. We could not assess sensitivity of facial structures due to the patient being sedated.\nA contrast-enhanced CT of head and chest was performed revealing soft tissue swelling of the left inferior turbinate and thickening of the mucosa of the maxillary, ethmoid, and sphenoid sinuses on the ipsilateral side. Periorbital and midfacial structures of the left sides also had soft tissue swelling, with associated proptosis (Figure ).\nThe thorax section had findings suggestive of atypical pneumonia due to SARS COV-2, consisting of multiple parenchymatous zones with an increased density and bilateral distribution with peripheral predominance giving a ground-glass appearance, coexisting with consolidation of lower lobes (Figure ).\nWe present the ancillary studies performed during her management at our center (Table ).\nOur initial diagnosis was complicated rhinosinusitis of probable fungal origin, with the following associated diagnoses: severe diabetic ketoacidosis, diabetes mellitus, severe metabolic acidosis, and highly likely atypical pneumonia due to SARS COV-2. Due to severity of infection, we started imipenem/linezolid and amphotericin B as empirical treatment.\nDirect exam was repeated due to suspicion of a false negative, confirming diagnosis in the next sample, with further culture in Sabouraud media isolating Lichteimia (Absidia) spp (Figure ). COVID-19 was confirmed with reverse transcriptase-polymerase chain reaction (RT-PCR) of bronchioalveolar fluid (Logix Smart™, Co-Diagnostics, Inc., Salt Lake City, UT, USA).\nEven with the established management of the severe diabetic ketoacidosis and mechanical ventilation, the patient had an unfavorable evolution due to refractory metabolic acidosis combined with the pulmonary insult and aggregated acute kidney injury due to disseminated intravascular coagulopathy, which precluded further surgical management, dying of multi-organic failure due to unresponsive septic shock (Figure ). |
A previously well 87-year-old Caucasian woman living in a senior assisted care center presented to the neurology clinic with complaints of six months of slowly progressing left sided weakness. Initial difficulty in ambulating and using the stairs progressed to being wheelchair bound. Neurologic exam revealed diffuse 3/5 left sided weakness, left leg drift, and left facial droop. Brain magnetic resonance imaging (MRI) revealed a large confluent white matter T2-hyperintensity in the right frontal lobe with multifocal nodular enhancement of the left cerebral hemisphere (). Foci of enhancement were also identified in the cerebellum and leptomeninges. The radiologic differential diagnosis included vasculitis, lymphoma, and CNS sarcoidosis as the most probable causes of the multifocal disease process, with glial neoplasm, demyelination, and metastases considered less likely.\nAll sample analysis described below were performed on material obtained by brain biopsy as part of clinical care. All samples were obtained with appropriate consent.\nA biopsy of the mass was performed and revealed extensive parenchymal lakes and vascular and perivascular deposition of amorphous, amyloid like material (). Congo-red positive staining and apple-green birefringence (not shown) of the amorphous material upon polarization confirmed that the amorphous material was amyloid (). Also present in the resected tissue were a number of small intraparenchymal blood vessels with perivascular lymphoplasmacytic infiltrates (). The initial histologic differential diagnoses included cerebral amyloid angiopathy-inflammatory type (CAA-I) and lymphoma associated amyloidoma. To identify the underlying etiology of the amyloid accumulation, a number of additional analyses were performed.\nLiquid chromatography tandem mass spectroscopic analysis identified the amyloid as AL λ-type and not β amyloid or an amyloid associated with a hereditary amyloidosis. Further analysis of the perivascular lymphoid populations was undertaken. Histologically, the monotonous populations of perivascular lymphoid cells demonstrated a lymphoplasmacytic appearance (). Immunohistochemical analysis demonstrated that the lymphoid cells were CD20 positive (). Tumor cells were negative for CD3, CD5, BCL1, and CD23. The tumor Ki67 proliferation index was low (3%). The more plasmacytoid appearing cells were CD138 positive and were shown to be lambda light chain restricted by kappa and lambda chromogenic in situ analysis (Figures and ). An immunoglobulin heavy chain (IgH) gene rearrangement analysis of the brain tissue from this case was positive for a clonal process with a 253-base pair peak in the FR2 region. A MYD88 L265P mutation analysis by PCR-based pyrosequencing on the brain tissue from this case was negative. A diagnosis of a low grade, lymphoplasmacytic lymphoma (LPL) was rendered. The identification of this CNS low grade lymphoplasmacytic lymphoma confirmed the cause of the amyloidoma to be a lambda light chain producing lymphoplasmacytic lymphoma.\nTo determine if an extracranial/systemic lymphoplasmacytic lymphoma was the source of the CNS neoplasm, a bone marrow biopsy was performed. The bone marrow biopsy showed normal trilineage hematopoiesis and no evidence of lymphoma, myeloma, or amyloidosis. Cytogenetics and fluorescent in situ hybridization studies on the bone marrow were negative for genetic aberrations. Urine protein and serum immunoglobulin levels were within normal limits. A biopsy of subcutaneous abdominal adipose tissue was negative for amyloid, demonstrating lack of evidence of systemic amyloid deposition. Interestingly, an IgH gene rearrangement analysis on the bone marrow was positive for a clonal gene rearrangement with two peaks: a 282-base pair peak in FR2 region and a 120-base pair peak in FR3 region in a polyclonal background, which importantly were markedly different from the IgH gene rearrangement identified in the CNS lymphoplasmacytic lymphoma. Since the two-small bone marrow clonal peaks are present in a polyclonal background, their significance is uncertain and may be age related.\nOur patient received one cycle of chemotherapy with Rituximab for Primary CNS lymphoplasmacytic lymphoma. Two months after diagnosis, she developed a hemorrhagic infarct on the left frontal white matter and was transferred to hospice care. |
The patient suffered from recurrent difficulty in voiding and urine retention even underwent surgical intervention for about half-a-year.\nA 65-year-old Asian man presented with difficulty voiding for about 2 years that deteriorated gradually within 6 mo even if received treatment with selective alpha-blocker agent. He visited a local hospital for first aid in 2016. In addition, dry ejaculation has also disturbed for more than one year before medical intervention. The patient did not complain about any episode of hematuria, fever, or loss of weight. TRUS showed an estimated volume of 230 cm3, irregular shape with heterogeneous echotexture, and multiple cysts. A systemic core needle biopsy revealed nodular hyperplasia with inflammation and some atypical glands but no evidence of malignancy. Because of the former failed medical treatments and the recurrent weak urinary stream and urine retention, two transurethral resections of the prostate (TURP) were undertaken during a short 4-month interval (April and July 2016). Both pathology reports revealed nodular hyperplasia and chronic prostatitis with a few atypical glands weighing 5.3 g and 5.2 g, respectively. No malignancy was identified by immunohistochemistry (IHC) staining.\nHowever, the patient continued to have gradually deteriorating obstructive voiding symptoms and acute urine retention for 6 months, even with 5-alpha reductase and alpha-blocker treatment. Therefore, he came to our tertiary hospital and underwent a laser prostatectomy (green-light XPS), with the consideration of TRUS still showing a huge residual prostate gland with an estimated volume of 131.3 cm3 and uroflowmetry disclosing a low peak flow and an average flow rate with a large postvoid residual urine volume. The pathology report on the resected tissues revealed multilocular cystadenoma of the prostate. Nonetheless, the patient experienced progressive difficulty in voiding 1 year after the laser prostatectomy, although the PSA levels were within the range of 4-6 ng/mL.\nHe had been healthy before the low urinary tract symptoms developed.\nHe has no habit of alcohol drinking or cigarette smoking and no specific family history.\nA digital rectal examination revealed a huge elastic prostate with rectal compression, and no tender or palpable induration was noted.\nA baseline biochemistry profile at presentation showed a high PSA level (13.0 ng/mL), with an otherwise normal biochemistry profile. Urinalysis was normal, with no evidence of hematuria. During the period of follow-up at our hospital from August 2016 to January 2018, the PSA levels were within the range of 4-6 ng/mL.\nIn 2016, computed tomography (CT) of the abdomen and pelvis (Figure ) at local hospital showed an 8.2 cm × 8.1 cm × 7.8 cm multiloculated cystic prostate gland with several enhanced focal lesions. At our hospital, TRUS still showed a huge residual prostate gland with an estimated volume of 131.3 cm3 (Figure ) even after twice TURPs at previous hospital. A subsequent magnetic resonance imaging (Figure ) showed a large multilocular retrovesical mass, 8.0 cm × 7.3 cm × 6.4 cm, with solid parts and heterogeneous enhancement, a coexisting malignant part of the tumor, either a cystadenocarcinoma or prostate intraepithelial neoplasm should be suspected, but there were no enlarged pelvic lymph nodes.\nThe gross prostate specimen was a solid mass 11.1 cm × 8.2 cm × 7.1 cm in size, weighing 245 g, and was almost entirely replaced by a multilocular cystic tumor (Figure ). The tumor was nonencapsulated, with mutilocular cysts with focal hemorrhage, watery contents, and smooth cyst walls. There was no grossly invasive focus. Histologically (Figure ), the tumor was composed of variously sized dilated glandular and cystic structures lined by blended prostatic type epithelia. Foci of florid papillary infoldings were seen in the large cysts. Focal squamous metaplasia was seen. There was fibromuscular stroma with focal adipose metaplasia. In addition, there were no atypical features or mitosis observed. The lining epithelia were positive for PSA, PSMA, CK7, and MUC2. The epithelia were negative for CDX2, CK20, MUC1, and MUC5ac on immunohistochemical staining, indicating that the tumor originated from the prostate. Final pathology indicated a giant multilocular prostatic cystadenoma. |
The proband was a 34 year-old right-handed man. From the age of 26 years, involuntary movements of the bilateral lower limbs, associated with dysarthria, grinding teeth and drooling, appeared and gradually worsened. At 31, he suffered from epileptic seizures, which were considered to be generalized tonic-clonic seizure, but antiepileptic drugs had never been administered. One year later, involuntary movements spread to his upper limbs and orofacial automatisms including abnormal tic-like facial movements, tongue protrusion and biting his lips appeared. Then he was treated with haloperidol (2 mg three times a day) and baclofen (10 mg three times a day) for 2 years for his choreic and dystonic problems, but he responded poorly to drug treatments. At age of 34, his involuntary movements gradually spread to his whole body and epileptic seizures increased in frequency. Since the disease onset, the patient had never suffer from psychiatric problems. Neurological examination revealed poor muscle tone and absent deep tendon reflexes in all limbs. Additionally, right positive babinski sign was elicited. Laboratory data revealed elevated creatine kinase level in the peripheral blood. Acanthocytes were found in 4% of cells on the peripheral blood smear test. Doppler ultrasound examination revealed splenomegaly. Brain magnetic resonance imaging (MRI) showed progressive, symmetrical, mild atrophy of the caudate heads (Figure ). His 24-h continuous electroencephalography (EEG) showed generalized asynchronous theta and epileptiform activity, which mostly originated from the right temporal lobe. A nerve conduction study showed a polyneuropathy, which revealed the right peroneal nerve, right median nerve and bilateral ulnar nerves were partly damaged. His score of Mini Mental Status Examination (MMSE) was 27. The father of the proband did not show any neurological abnormalities and died from pneumonia at 65 years old (Figure ). The mother of the proband (II-3), a 65-year-old woman, showed mild involuntary movements in her limbs since the age of 45 years (Figure ). The proband's uncle (II-5), a 52-year-old man, showed mild cognitive impairment (MMSE 24), characterized by memory impairment and had seizures history of 31 years, which were simple partial seizures and treated with antiepileptic drugs (Figure ). His another uncle (II-1) and two sisters (III-1, III-2) had no neurological clinical symptoms. Brain MRI and peripheral blood smears of the proband's mother, his uncles and two sisters are normal. The clinical picture of the proband was suggestive for ChAc, but the inheritance mode of this family seems to be autosomal dominant.\nAll patients were of Han nationality from Hunan province, China. Blood specimens and genomic DNA were obtained from family members and 100 control subjects after informed consent. The 73 exons and flanking intronic splice consensus sequences of VPS13A were amplified by polymerase chain reaction (PCR) (, ). By sequencing, we identified a novel homozygous nonsense mutation c.8823C > G (p. Tyr2941*) in exon 65 of VPS13A in the proband (Figure ). Five members of the family including the proband's mother (II-3) and his uncle (II-5) were detected to be heterozygous for mutation c.8823C > G (Figures ). The homozygous nonsense mutation c.8823C > G (p. Tyr2941*) causes the loss of TPR10 domain of the vacuolar protein sorting 13A protein. This homozygous nonsense mutation c.8823C > G was not detected in 100 healthy controls, thus representing a novel etiology in an ChAc Chinese family. Besides, the mutations in genes responsible for Huntington's disease and McLeod disease were screened and the results were negative. |
A 57-year-old female presented with vision loss in the left eye during the restoration of consciousness after endoscopic DCR surgery for the left eye. In this case, the DCR surgery was performed under general anesthesia. Notably, 2 ml of 1% lidocaine with 1:100,000 epinephrine was injected into the axilla of the middle turbinate and the frontal process of the maxilla using a dental syringe. In this case, the neurosurgical patties soaked in 2 ml of 1:1000 epinephrine were inserted between the inferior turbinate and the nasal septum and in the middle meatus to achieve topical decongestion. In the process of making mucosal flap and incision, the patient had a higher bleeding tendency than was noted with other patients, and a suction diathermy was used meticulously for the incidence of hemostasis. For this reason, it did not lead to a major bleeding in this case.\nThe patient’s medical history was notable for thrombocytopenia and MHA. Upon review, the patient denied temporal headache, pain, or flashes. When tested, the patient’s best-corrected visual acuity (BCVA) was 20/20 in the right eye and light perception in the left eye. Her intraocular pressure (IOP) was 14 mmHg in the right eye and 16 mmHg in the left eye. Her visual field test result was normal for the right eye. However, the test could not be conducted for the left eye due to the incidence of poor vision. When tested with the swinging flashlight maneuver, a relative afferent pupillary defect was found in the left eye of the patient. Her extraocular movements were noted as being full and painless. However, mild periorbital bruising and swelling were detected in the left eye. Additionally, there was mild maxillary sinusitis noted as well. However, it was shown there was no underlying disease in the other sinuses. On the funduscopic examination, there were no obvious abnormal findings in the macula of either eye. The use of a fluorescent angiography did not reveal leakage or a filling defect at the disc. The baseline testing included blood tests to evaluate syphilis, systemic lupus erythematosus, and neuromyelitis optica. Her erythrocyte sedimentation rate and C-reactive protein results were noted as normal. Her pre-operative platelet count was 61 × 103/mm3. A chest x-ray was performed to evaluate sarcoidosis. She was transfused with six units of platelets preoperatively, which increased her platelet count to 123 × 103/mm3. No other cause of optic neuropathy was found in this evaluation.\nThe pattern visual evoked potential revealed delayed P100 latency (Fig. ). Her electroretinogram showed normal electrical activity in the retina. The magnetic resonance imaging (MRI) of the orbit revealed a focal hyperintensity within the intra-orbital segment of the left optic nerve on the T2-weighted image (T2-WI) and flair image. At evaluation, the MRI showed an enhancement on the T1 post-contrast imaging (Fig. ). It did not show any demyelinating disease in the brain. The patient was diagnosed with left optic neuropathy and treated with 1 g/day of intravenous methylprednisolone for 3 days, followed by 1 mg/kg/day of oral prednisone with subsequent dose tapering. It is noted that the patient’s BCVA improved to 20/30 after the treatment. Although her vision improved, she was left with a visual field defect in the left eye. |
A 21-year-old male patient, with an uneventful medical history, came to the emergency department of our hospital after an episode in which he lost consciousness. The patient was hemodynamically stable, with blood pressure and heart rate within normal limits; ECG did not present any abnormalities, either. At the time of presentation, he was in a good neurological condition and did not mention any thoracic or abdominal pain. Blood laboratory investigation showed normal values of hemoglobin, hematocrit, glucose and other markers. He was then admitted to the department of internal medicine for further investigation.\nTwelve hours later, the patient complained of diffuse abdominal pain, while a decrease of hemoglobin was observed (9.5 mg/dL), and hemodynamic instability presented, with an increase in heart rate to 100 bpm. He was initially treated with intravenous fluids; surgical opinion was then seeked. Clinical examination revealed pronounced rebound tenderness on all of the abdominal quadrants and decreased abdominal sounds. In order to investigate the possibility of intraperitoneal bleeding and considering the stability of the patient, an emergency abdominal CT scan with i.v. contrast was indicated. CT scan revealed the presence of a ruptured proximal splenic artery aneurysm measuring 26 mm × 18 mm, 6 cm from its origin ().\nThanks to the rather stable hemodynamic condition of the patient, and the fact that our institution is equipped to provide an endovascular approach, open surgery was excluded as the first therapeutic option; angiography with coil embolization of the aneurysm and proximal splenic artery was then performed. Under local anesthesia (injection of lidocaine solution 2%, Astra Zeneca, Athens, Greece), a percutaneous puncture of the right common femoral artery, and selective catheterization of the splenic artery was performed, using a 4-F Simmons 1 catheter (Cordis, Tipperary, Ireland). Subsequent angiography revealed the aneurysm, but an attempt to pass a microcatheter (Progreat®, Terumo, Leuven, Belgium) distally through the aneurysm failed. Embolization of the aneurysm itself and of the proximal splenic artery was decided, and 2D and 3D mechanically detachable fibered micro coils (Interlock-18, Boston Scientific, Cork, Ireland), and N-butyl-cyanoacrylate (NBCA) glue (Histoacryl®, Brown, Hessen, Germany) were used, preserving all of the collateral vessels for the spleen.\nAfter embolization, the patient was stabilized and no signs of abdominal bleeding were observed on imaging. He was then transferred to our surgical department for further intense monitoring and treatment with intravenous fluids and blood transfusions. His recovery remained uneventful, as he did not develop further bleeding. He was discharged on the 12th day after the embolization in good condition; follow-up examination on an 8th-month basis showed preservation of the spleen, complete elimination of aneurysm and no other abnormal findings (). |
A 51-year-old male was diagnosed with muscle-invasive urothelial cell carcinoma in May 2015. Standard neoadjuvant chemotherapy was given followed by radical cystoprostatectomy. Final pathology showed pT3 N0 M0, stage III disease. He remained disease free until April 2016, at which point he developed new exertional dyspnea and a small lung nodule was noted on imaging. During cardiac clearance for a biopsy, a Mobitz 2 heart block with bradycardia was noted. Transthoracic echocardiogram (ECG) demonstrated a mass in the right ventricular outflow tract, which was additionally found to be fluorodeoxyglucose (FDG)-avid on positron emission tomography (PET) computed tomography (CT) (Figure , upper panel).\nThe patient developed progressive dyspnea with minimal exertion and then experienced a syncopal episode prompting hospitalization. Biopsy of the right ventricular mass demonstrated poorly differentiated carcinoma, consistent with urothelial origin (Figure ).\nThe mass was not felt to be respectable and chemotherapy was not felt to offer rapid disease control in the setting of progressive symptoms. He was offered palliative radiotherapy. His bradycardia progressed to a complete heart block, likely due to the growth of the mass, necessitating placement of a dual chamber pacemaker. The right ventricle mass was treated with 45 Gy in 18 fractions (3D conformal photons for 5 fractions, followed by intensity-modulated radiotherapy for the remaining 13 fractions (to reduce dose to the left ventricle). Figure demonstrates the dose distribution.\nProton radiation was considered to spare the uninvolved myocardium. He had substantial tumor thromboembolic disease to his lungs during radiotherapy, requiring medical intensive care unit (MICU) admission for symptom management. He ultimately completed the full course of radiotherapy which improved his functional status.\nFollowing radiotherapy, he began immune checkpoint therapy with the PD-L1 antagonist atezolizumab (1200 mg every three weeks), which was well tolerated. A PET/CT scan seven months after completing radiation demonstrated a complete metabolic response in the right ventricle mass and no other sites of progression (Figure , lower panel). The previously noted pulmonary nodules were stable. He resumed normal activity and returned to work. Eight months after radiotherapy and six months into his immune therapy, he developed progressive shortness of breath. He had heart failure and the ejection fraction reduced to 20%-30%. Cardiac magnetic resonance imaging (MRI) revealed a focal area of subendocardial delayed enhancement, potentially representing myocarditis. Cardiac catheterization revealed extensive coronary artery disease and complete occlusion of the right coronary artery; however, the left to right collaterals were intact. Atezolizumab was held with an initiation of high dose steroids to treat potential immune-mediated myocarditis with minimal effect. Electrophysiology testing demonstrated severe cardiac dyssynchrony. Cardiac resynchronization therapy was recommended and a biventricular pacer was placed with rapid symptom relief. He returned to work with overall minimal symptoms. Atezolizumab was resumed, and a total of 18 cycles (54 weeks) of therapy were completed without issue.\nIn the months that followed the placement of his biventricular pacer, he was evaluated several times for shortness of breath and atypical chest pain, however with no clear etiology. PET/CT imaging in August 2017 demonstrated increased FDG-avidity in his right ventricle concerning for progression. An MRI was ordered and in early September 2017, he was admitted for workup of an exacerbation of chest pain. A contrast-enhanced CT of the chest showed significant disease progression in the area of prior treatment, extension toward the left ventricle and along the heart wall leading to increased right heart pressure (Figure ), and probable encasement of his left anterior descending (LAD) artery by disease progression (Figure ). He had acute progression of chest pain associated with an increased oxygen requirement, shortly after being admitted. His ECG showing ST elevations in the anterior leads, consistent with an LAD infarct (Figure ). The cardiology service was consulted to consider palliative LAD stenting; however, the patient underwent cardiac arrest in the interim and did not wish to be resuscitated. He died 17 months after his disease recurrence. |
A 30-year-old male patient presented to the neurosurgical services with history of left focal seizure with secondary generalization for the last three to four years along with left sided weakness for the past one month. There was history of slowly progressive decreased vision in both eyes leading to complete blindness in both eyes for the past one month. On neurological examination, patient had left hemiparesis of grade-2/5 with perception of light absent in both eyes. Fundus examination revealed bilateral optic atrophy.\nMagnetic resonance imaging (MRI) of the brain showed a large supratentorial heterogenous multicystic ring enhancing mass lesion involving right frontal lobe, right frontotemporal opercular region, and posteriorly abutting the central sulcus [Figures and ]. The lesion was hyperintense on T2WI and FLAIR images. The lesion was showing multiple cystic components of various sizes with smaller solid component abutting the dura peripherally; largest cyst showing ring enhancement with enhancing mural nodule in the superior aspect of the lesion. There was mild perilesional oedema and marked mass effect seen causing compression of right lateral ventricle and producing significant midline shift towards left side.\nPatient underwent right frontoparietal craniotomy and near total excision of tumor. He received standard preoperative medication, including phenytoin, dexamethasone antibiotic, and mannitol on the morning of surgery. After the scalp was opened, the tumor was visible anteriorly, and there was destruction of galea and bone was present. Craniotomy was performed by using wide margin around the destroyed calavaria with the use of multiple burr holes. After the bone flap was removed, a large tumor mass was found in the extradural space anteriorly. The dura under this mass was completely intact macroscopically. The extradural mass was easily peeled off the dura. The dura was excised widely around the tumor, and attention was focused on intraparenchymal part of the tumor. Tumor was gray, soft, and friable, and at places, it showed cystic components also. It was moderately vascular and there was no clear plane of margin between tumor and normal brain. Near total excision of tumor was done and dura was repaired with bovine pericardium dural substitute. Bone flap was then inspected and the tumor resected with high speed drill and bone flap was replaced and scalp was closed. Postoperatively, patient kept on elective ventilatory support for 24 hours. Histopathological examination of tumor revealed oligodendroglioma WHO grade-2 [].\nPostoperatively, patient received six weeks of external beam radiotherapy to the gross tumor volume along with chemotherapy. There was slight improvement in limb weakness, but there was no improvement in vision and his seizures are well controlled on single antiepileptic drug.\nAt nine month follow-up, there was no recurrence of tumor. |
A 41-year-old female who was 37 weeks pregnant was admitted to the obstetric labor ward. She had a history of only one prior delivery. She had a previous uncomplicated cesarean section for failure to progress. There were no issues or complications during this pregnancy. However, she developed gestational diabetes, which was well-controlled on diet alone. The only other significant medical history was that of hypothyroidism which was under control with L-Thyroxine. Her vitals were normal. Her height was 5.2 feet and her weight was 150 pounds. The decision was to proceed with a repeat cesarean section.\nAt 2:30 am, she was brought to the operating room. She received a spinal anesthetic for the cesarean section. The spinal was preformed with the patient in the sitting position. The needle was inserted midline. Once cerebrospinal fluid (CSF) was identified 1.5 ml of hyperbaric bupivacaine 0.75%, fentanyl 10 mcg and epimorphone 150 mcg were injected into the intrathecal space. There were no complications including no parasthesia or no blood through the spinal needle. The surgery went smoothly, and there were no complications. The estimated blood loss was 800 ml.\nThe mother was transferred to the recovery room at 3:40 am. She spent 2 h in the recovery room. There she received 6 mg of naltrexone orally at 5:30 am for itching. At 5:45 am, she was transferred to the labor ward. On the labor ward, the nurses took hourly vitals as per the hospital's protocol regarding patients who have received intrathecal opioids. Her vitals were all normal including her respiratory rate that ranged from 15 to 19 breaths per minute and her saturation which was 96%–97% on room air. However, the nurses did notice that she was quite drowsy.\nAt 8:40 am, a code blue was called because the patient was unresponsive and cyanotic. Oxygen was applied through ambu bag and 0.4 mg of naloxone was given. She became more responsive and her saturation reached 100% on 10 L of oxygen by facemask. She was still drowsy. Hence, she was transferred to the Intensive Care Unit and started on a naloxone infusion. The naloxone infusion was discontinued the next day at 00:50 am. At 1:06 am, she required hydromorphone for pain relief. |
A 56-year-old male presented to the emergency department (ED) with significant substernal chest pain starting 30 minutes prior to arrival. The patient was immediately brought back to an exam room after an electrocardiogram (ECG) was performed and was seen by a provider within 10 minutes of registration (Figure ).\nThe initial ECG revealed atrial fibrillation with a rapid ventricular response, rate of 147, with minimal ST depression within the lateral leads but was without apparent ST-segment elevation. On initial assessment, the patient had point-of-care labs immediately drawn (a basic metabolic panel and troponin), and a chest X-ray performed to evaluate for a possible aortic dissection which revealed no gross abnormalities (Figure ).\nWith a non-diagnostic chest X-ray alternative diagnoses were pursued. The ultrasound fellow in the department was consulted for an immediate cardiac ultrasound for evaluation of right heart strain secondary to pulmonary embolism. During the bedside cardiac ultrasound, the patient experienced ventricular fibrillation (Video ), and CPR was immediately started.\nThe ACLS algorithm was followed for pulseless ventricular fibrillation, and the patient received multiple rounds of epinephrine, 450 mg of amiodarone (300 mg and then 150 mg), and three conventional defibrillations with increasing joules at 150 J, 200 J, and 200 J (the departmental defibrillators are biphasic and have a maximum output of 200 J). The patient continued with ventricular fibrillation throughout the ACLS algorithm, and the decision was made to attempt DSD. The patient had a second set of pads applied in the anterior-posterior orientation in addition to the conventional right upper chest and left lateral chest with successful conversion of the ventricular fibrillation. The patient was additionally given Lidocaine, 100 mg, due to a wide-complex tachycardia and apparent non-responsiveness to the previously given amiodarone (Figure ). An improvement was noted after the use of Lidocaine, and a Lidocaine drip was started.\nOnce the patient was stabilized, he was taken for computed tomography (CT) imaging to further evaluate for the possibility of a pulmonary embolism. In the CT room, he developed bradycardia and subsequently lost his pulse. CPR was again started, the patient was given atropine, and return of spontaneous circulation (ROSC) was achieved shortly after. The CT scan did not reveal any evidence of aortic dissection or pulmonary embolism and a repeat ECG was performed which showed a persistent wide complex tachycardia with no obvious ST-segment changes. Due to the morphology of the QRS complexes and length of resuscitation time from initial arrest (nearing 90 minutes), tissue plasminogen activator (tPA) was used as a thrombolytic for what was presumed to be a large vessel occlusion myocardial infarction. Hundred milligram of tPA was administered (50 mg as a bolus and 50 mg as a drip given over 60 minutes) with an apparent reperfusion rhythm followed by a "normal" appearing sinus tachycardia. Another ECG was repeated which revealed sinus tachycardia, at a rate of 114, now with ST-segment elevations present in aVR, V1, V2, V3, and V4 with depressions in leads II, III, and aVF (Figure ).\nGiven the patient's persistent elevations despite thrombolytic therapy, interventional cardiology was consulted, and the patient was transferred to a tertiary care facility for cardiac catheterization revealing a thrombotic occlusion in the proximal left anterior descending coronary artery.\nAfter transfer to the tertiary care facility for cardiac catheterization, the patient developed cardiogenic shock. The patient was started on ionotropic medications with no improvement, and he was placed on venous-arterial extracorporeal membrane oxygenation (ECMO) therapy (~20 hours after his cardiac arrest). Before initiation of ECMO, the patient was awake, alert, and following simple commands (although still intubated). Unfortunately, despite ECMO support, his cardiac function did not improve, and the patient was not a candidate for cardiac transplantation. Seventeen days after the patient's initial presentation to the ED, the family decided to withdraw care. The patient was extubated, had ECMO discontinued, and time of death was documented shortly after. |
A 47-year-old woman presented with a painful subungual lesion on the right big toe which appeared one year before as a hyperkeratotic lesion and treated with oral terbinafine, with no improvement. Twelve years ago she underwent laser therapy for a lesion at the same location. A histopathological examination was not performed.\nPhysical examination revealed a painful subungual nodule on her right big toe. There was no regional lymphadenopathy.\nAn excisional biopsy of the nodule was performed. Histopathological examination showed an ulcerated tumor extending in a diffuse pattern from the epidermis to the inferior limit of the specimen. Rounded cells with vesicular nucleus, scant cytoplasm, numerous mitotic figures, dyskeratotic and apoptotic cells were observed ().\nThe presence of nests of large, bizarre chondrocytes embedded in a chondroid matrix was observed in around one third of the lesion, strongly suggesting positivity for Alcian Blue ().\nOn the periphery of the specimen hyperplasia and acanthosis of epidermis were observed as well as intracytoplasmic melanin pigment in small aggregates (). Nests of atypical melanocytes were observed at the dermoepidermal junction without evidence of pagetoid spread, and isolated atypical melanocytes were present in the upper dermis ().\nImmunohistochemistry showed positivity of the junctional component and of melanocytes in the upper dermis for S-100 protein and positivity of the tumor cells for HMB-45 (, and ). The cartilaginous component showed positivity for S-100 protein and for neuron specific enolase (). We diagnosed melanoma with cartilaginous differentiation. The tumor had a Breslow depth higher than 5 mm (Clark level V).\nThe patient subsequently underwent amputation of the distal phalanx of the big right toe. Histopathological examination of the lesion confirmed the biopsy findings of malignant melanoma with cartilaginous differentiation, with a Breslow thickness of 9,9 mm and a Clark level of V. Sentinel lymph node biopsy was also performed, which proved negative for tumor. According to the TNM staging this malignant melanoma was T4b N0 M0, stage II B.\nTwo years after surgery an ulcerated lesion at the same location appeared. This was excised and proved by histopathological examination to be a local recurrence. The big and second toes were amputated. This subsequent wider excision showed no residual melanoma.\nNeither local recurrence nor metastasis were observed during the following three years of followup. |
A 52-year-old female with a history of fibromyalgia, left shoulder osteoarthritis, and chronic pain in her left arm, neck and back presented to the ED with an exacerbation of her chronic pain for two days. She had suffered a fall while in the shower one year prior to presentation and attributed her chronic neck, back, and radicular left arm pain to this injury. She had tried chiropractic manipulation, acupuncture, intraarticular glucocorticoid injections, physical therapy, and topical creams and patches, as well as a variety of over-the-counter medications. Despite these interventions, her pain persisted. Her vital signs on presentation to the ED were all within normal limits, and on physical examination her pain was rated as 10/10 on the left side including her lateral neck, back, circumferential upper arm, and lateral aspect of her elbow.\nShe exhibited allodynia in these regions and had pain-limited range of motion at the shoulder and elbow. There were no areas of skin erythema, induration, or fluctuance. Plain radiographs of her shoulder and elbow revealed no acute findings. She had received 10 milligrams of oxycodone while waiting in the triage area, but this gave her minimal symptom relief, likely due to the severity of her pain. After explaining the risks and benefits to the patient, an ultrasound-guided ESPB was performed with 60 milliliters of 0.25% ropivacaine (–).\nThe block was performed at the level of the second thoracic (T2) vertebrae with the patient in prone position and the ultrasound probe oriented parasagittally. An in-plane approach was used with the needle tip oriented cephalad. During the instillation of the local anesthetic, manual compression caudal to the site of injection was applied to influence spread of the injectate cephalad toward the vertebral levels where the patient was experiencing pain ().\nThe procedure was performed without complication. After 30 minutes, the patient reported complete relief of her neck, shoulder, thorax, and back symptoms and had complete restoration of range of motion. She did not experience any motor blockade and had full strength in her extremity. She rated her pain at a 0/10 and expressed satisfaction at the quality of her pain control. Only minimal elbow pain persisted and after a period of monitoring in the ED, the patient was discharged home. On follow up, she reported that she had complete relief of pain for the next five days, after which her symptoms gradually returned at a more tolerable level. |
A male preterm 980 g by birth weight was born to 25-year-old primigravida with 28 weeks of gestation. The baby was delivered with a cesarean section for the indication of fetal distress. In view of respiratory distress, the baby received single dose of natural bovine surfactant within half an hour of birth and was ventilated with nasal continuous positive airway pressure. A PICL (Vygon, Germany) was inserted electively on the 5th day of life through the right saphenous vein. The chest X-ray confirmed the position of the catheter tip at the junction of inferior vena cava and right atrium just above the diaphragm. On the 7th day of life, the baby suddenly deteriorated in the form of desaturations, tachycardia, and hypotension. On examination, the neonate appeared cyanosed, with saturations of 85% and delayed capillary refill time. On auscultation, the heart sounds were feeble and blood pressure showed a systolic reading of 30 mm mercury. The baby was therefore immediately intubated and was put on mechanical ventilation with pressure control mode and 100% oxygen. Systemic hypotension was corrected with inotropes. In view of this sudden deterioration, a chest roentgenogram was called for, which showed marked cardiomegaly and a cardiothoracic ratio of 0.68 []. In view of the above findings, a two-dimensional echocardiogram was performed, which showed a massive PCE []. There was a diastolic collapse of the free wall of the right atrium, indicating cardiac tamponade. Pericardiocentesis was immediately performed, and 9 ml of yellowish fluid was aspirated through a subxiphoid percutaneous approach. Subsequently, the skin color of the neonate improved rapidly, his vital signs gradually returned to normal, and his oxygen requirement decreased from 90% to 30%. Echocardiography done later showed increased contractility with resolution of the PCE []. The PICL line was immediately withdrawn. Inotropic support was gradually reduced and omitted after 24 h; the pressure support for the baby was also gradually reduced and the baby was weaned off the ventilator the next day. Biochemical analysis of the obtained pericardial fluid revealed a glucose concentration of 1215 mg/dl and total protein of 0.1 g/dl. There were 100 red blood cells/mm3 and 50 white blood cells/mm3. The aspirated fluid was suggestive of total parenteral nutrition infusate. There was no further accumulation of fluid, and a follow-up echocardiogram after a week was normal. The baby was started on trophic feeds 3 days after pericardiocentesis. The patient was subsequently discharged, and a chest roentgenogram done before discharge showed a normal cardiothoracic ratio. |
The patient is a 29-year-old previously healthy female, gravida 1 para 0, who presented for a scheduled induced abortion via dilation and evacuation at 22-weeks gestation, in the setting of a pregnancy complicated by a fetus with trisomy 22 confirmed on amniocentesis and multiple fetal anomalies. Workup prior to the procedure included a complete blood count which revealed a hemoglobin of 12.1 g/deciliter and platelets of 134 × 103/μl. No history of pre-pregnancy thrombocytopenia was noted. The patient’s pre-procedure systolic blood pressures ranged from 80 to 100 s mmHg.\nThe day prior to the procedure the patient had osmotic cervical dilators placed in the office. On the day of the procedure a singleton fetus with fetal heart tones was confirmed by ultrasound, monitored anesthesia care was administered with fentanyl, midazolam and propofol in an operating room, osmotic cervical dilators were removed, and a paracervical block with 1% lidocaine was performed. The patient then underwent ultrasound-guided dilation and evacuation using electric suction curettage and Bierer forceps. All fetal parts and the placenta were accounted for at the end of the case, with an estimated blood loss of 100 ml and no apparent complications. No hypoxia, tachycardia, or hypotension were noted in the operating room at the time of the procedure.\nIn the post-anesthesia care unit 15 min post-operatively, the patient experienced moderate vaginal bleeding (visually estimated to be 200 ml) with normal vital signs. The leading diagnosis for additional blood loss at this time was uterine atony; methylergonovine 200 micrograms intra-muscular was administered and a 500 ml crystalloid bolus was given. Forty-five minutes after the procedure moderate vaginal bleeding (visually estimated to be additional 200 ml) was noted again. On assessment vital signs were normal, transabdominal ultrasound revealed a thin endometrial stripe and no fluid posterior to the uterus to suggest intra-abdominal hemorrhage. Firm uterine tone was noted on bimanual exam. Rectal misoprostol (800 micrograms) was administered.\nFinally, 75 min post-operatively, the patient became acutely hypotensive (54/42 mmHg). Anesthesia and gynecology teams were immediately called to the bedside. An additional crystalloid fluid bolus was initiated and the decision was made to transfer the patient back to the operating room for evaluation, considering a differential diagnosis for bleeding including retained products of conception, uterine atony, cervical laceration, or coagulopathy.\nIn the operating room, a complete blood count, activated partial thromboplastin time (aPTT), and International Normalized Ratio (INR) were obtained. Monitored anesthesia care was again administered. A 1 × 2 cm piece of clot was removed from the endometrial canal on bimanual exam. Firm uterine tone was noted. Gritty texture and no ongoing bleeding was noted with repeated curettage with a suction cannula. No cervical lacerations were noted, however, hemostatic agents were applied to two site of oozing on the external os. Blood loss was estimated to be 750 ml total at this time.\nThe patient was transferred back to the post-anesthesia care unit, where an additional dose of intramusclular methylergonovine 200 micrograms was administered given continued moderate bleeding while awaiting lab results. Intra-operative lab results returned showing mild anemia, worsened thrombocytopenia, and abnormal elevation of INR and aPTT (Table , Lab 1). Clinical suspicion at this time was for possible AFE, given an episode of significant hypotension and coagulopathy out of proportion to blood loss. STAT repeat labs including a fibrinogen level were ordered, in addition to a ROTEM®.\nROTEM® results (Fig. ) showed very low amplitude fibrinogen, reflected in the low amplitude at 10 min (A10) in the FIBTEM channel, and prolonged clotting time (CT) (359 s) and reduced clot amplitude A10 in the EXTEM (16 mm) channel. Concentrated fibrinogen and transfusion of fresh frozen plasma and platelets were initiated immediately. Additional blood products were ordered from the blood bank. Fifty minutes after the initial ROTEM®, a repeat ROTEM® and repeat STAT labs were sent; at this time the full results were not yet available from the previous labs sent for analysis. More than 2 h after the first infusion of concentrated fibrinogen was initiated based on the first ROTEM® results, the first STAT fibrinogen level resulted as less than 25 mg/deciliter (Table , Lab 2). By the time these labs resulted, a second ROTEM® had been run and continued to show severe hypofibrinogemia, as shown in the FIBTEM A10 value (2 mm), and improvement in EXTEM CT (187 s) (Fig. ).\nConcurrently, the patient developed hypoxia, was placed on a non-rebreather mask and the surgical intensivist team was consulted. Labs sent at the time of the second ROTEM® confirmed ongoing coagulopathy (Table , Lab 3). After receiving 4 g concentrated fibrinogen, 2 units of cryoprecipitate, 4 units of fresh frozen plasma, 2 × 5-pack platelets, and 2 g of tranexamic acid, repeat labs were obtained; one unit cryoprecipitate and 2 units of packed red blood cells were given between obtaining the labs and receiving the results. Upon arrival to the surgical intensive care unit, labs showed worsened anemia (not yet reflecting the 2 units of packed red blood cells given after the labs were obtained), improving thrombocytopenia, normalization of aPTT and INR, and normalization of fibrinogen (Table , Lab 4). Repeat ROTEM® showed normalization of fibrinogen levels (FIBTEM A10 = 13 mm), CT (60 s), and EXTEM A10 (45 mm) (Fig. ).\nAfter transfer to the surgical intensive care unit, a chest radiograph showed diffuse hazy opacification predominant in the peri-hilar regions and lower lungs. The patient received heated high flow nasal cannula secondary to desaturations and 2 × 5-pack platelets for thrombocytopenia. She remained hemodynamically stable, her hypoxia resolved, and she was discharged on room air on post-operative day two. She was doing well and asymptomatic at her two-week post-operative appointment. |
A 36-year-old male presented to the outpatient department with a history of low backache for the past one year, associated with typical claudication symptoms, left-sided unilateral numbness, and paresthesia of the foot. He gave no history of radicular pain, but he complained of progressive reduction in claudication distance. He had experienced a sudden deterioration in gait along with urinary incontinence, following a trivial traumatic fall, one week before presentation. There was no history of constitutional features. On examination, he had a bilateral high-stepping gait due to foot drop. Symmetrical weakness of both the L4 and L5 roots (MRC grade 0/5) and partial weakness of the S1 root (MRC grade 3/5) was noted with nondermatomal sensory disturbances. Deep tendon reflexes of the lower limbs were absent bilaterally. Though anal tone was normal, saddle anesthesia was present. Postvoidal ultrasonogram of the urinary bladder revealed a residual urine volume of 250 ml, thus confirming a neurogenic bladder.\nPlain radiography of the lumbar spine did not show any gross feature of instability and was inconclusive (). Magnetic resonance imaging (MRI) of the lumbar spine revealed a solitary intraspinal posterior epidural lesion of 1.8 × 1.5 × 0.5 cm at the L2-L3 level with heterogeneous signal intensities and adjacent epidural fatty hypertrophy contributing to severe canal stenosis (3 mm). The cauda equina was severely compressed and was almost not visible (). Owing to the heterogeneous signal intensities, computerized topography (CT) was performed which revealed the presence of an osseous lesion attached to the right L2 inferior articular process causing severe secondary canal stenosis (). Considering the recent-onset neurological deficit, the patient was advised surgical decompression and excision biopsy at the earliest. The patient was positioned prone on a Relton Hall frame under general anesthesia. A standard midline posterior approach was employed and L2 and L3 lamina were exposed. The spinous process was removed and using a motorized burr, a rectangular trough was created surrounding the lesion. The lamina was thinned out using a burr to avoid further insult to the dural sac, and then using a Kerrison ronguer, laminectomy was completed all around the lesion under microscopic guidance (). A small osteotome was used to remove the attachment on the right side which required partial removal of the facet joint. The lesion was then held, and the adherent soft tissues beneath the lesion were removed, resulting in en bloc removal and complete decompression of the cauda equina. Fusion was performed, as the procedure involved partial facet joint resection. The lesion was sent for histopathological examination. There were no adverse events or postoperative complications. The patient was mobilized with bilateral orthoses, and bladder training was initiated.\nAt 4 weeks, there was an improvement in his urinary symptoms, and by 12 weeks partial neurological recovery (MRC grade 3/5 motor power) in bilateral L4 and L5 was observed. The S1 root power increased by one grade (MRC grade 4/5). His gait improved thereafter, and he was able to return to his normal activities by 6 months with further neurological improvement by 1 grade in all roots.\nThe gross specimen measured 2 cm × 1.5 cm × 1.5 cm. It was greyish white in color and firm to hard in consistency (). It had a well-defined capsule and had the feel of particulate materials on cut sections. Histopathological examination revealed the presence of bone, cartilage and ligamentous tissue, and zones abutting all these composed of mature adult-type encapsulated adipose tissue (Figures and ). This confirmed the diagnosis of benign osteolipoma. |
A 45-year-old Japanese man visited our clinic in September 1996 complaining of decreased vision in his left eye. His vision was decreased to 20/200, and ophthalmoscopy showed a retinal detachment involving the macula and a retinoschisis that extended from the optic disc pit through the macula in the left eye (Figure ). Glial tissue was seen at the optic disc pit but a retinal tear was not seen. Fluorescein angiography showed hypofluorescence of the optic disc pit, and multiple hyperfluorescent spots in the area of the macular lesion (Figure ), but with no dye leakage. In the late phase, the optic disc pit and glial tissue became hyperfluorescent with mild dye leakage. He was diagnosed with optic disc pit maculopathy and vitrectomy was recommended.\nAfter obtaining informed consent, vitrectomy was performed. A posterior vitreous detachment (PVD) was created by suction with a vitreous cutter until the 'fish-strike sign' was no longer seen. However, the vitreous cortex remained firmly attached at the optic disc pit. Neither condensed vitreous strands nor a residual Cloquet's canal was observed. It was decided intra-operatively that the glial tissue at the edge of the optic pit should be removed in order to remove the vitreous traction completely. Tapered forceps with a fine tip were used to avoid contact with the neural tissue at the edges of the optic pit. During this procedure, it was noted that the glial tissue was firmly attached to the temporal wall of the optic pit. An excavated space at the bottom of the optic pit was then clearly observed after removal of the tissue.\nThe vitrectomy was completed with a 14% octafluoropropane (C3F8) gas tamponade, and the patient was instructed to maintain a face-down position for a week. Under these conditions, the retinal detachment and retinoschisis gradually decreased, and the retinal detachment and retinoschisis were absent six months postoperatively (Figure ). Vision improved to 20/20 without any visual field defects (Goldmann perimetry).\nThe patient's vision deteriorated to 20/40 owing to a nuclear sclerosis cataract two years after the vitrectomy, and the lens was extracted. Vision has remained 20/20 for 10 years without any recurrence of the retinal detachment or retinoschisis. Optical coherence tomography (OCT) at this time did not detect a retinal detachment or retinoschisis, but two channels were seen running from the vitreous cavity to the longitudinal space of the optic nerve, possibly the subarachnoid space and the intraretinal space. The exit of these channels to the vitreous cavity was closed (Figure ). |
The patient is a 57-year-old nulligravid female who presented to clinic with symptoms of urinary urgency incontinence. She had a past medical history of endometriosis, autoimmune hepatitis, cirrhosis, and denied any pertinent mental/psychological history or trauma. Her initial symptoms included urgency, voiding up to 5 times an hour, and nocturia up to five times per night. She had no previous vaginal surgeries. Baseline sexual function evaluation was completed at intake with the Female Sexual Function Index (FSFI) questionnaire where the arousal domain equaled 0.9 (range 0-6, with 6 indicating maximal arousal) (). Pelvic examination was significant for vaginal atrophy but no notable clitoral or labial abnormalities were visualized. There were no signs of prolapse or pelvic floor musculature hypertonicity. She was asked to keep a voiding diary and then advised to attempt timed voiding upon its completion. She was also started on vaginal estrogen and a generic anticholinergic agent (oxybutynin 10 XL daily). After 4 weeks of treatment, she experienced significant worsening of anticholinergic side-effects and stopped the medication on her own. She was then started on a beta sympathomimetic (mirabegron 25mg daily). After 6 weeks of this therapy, she did not have any subjective improvement in her symptoms. The dose was increased to 50 mg daily and continued for an additional 4 weeks. Again, she did not have an adequate reduction in symptoms and was counseled on third tier treatment options. Ultimately the decision was made to proceed with SNM.\nThe sacral neuromodulator was implanted per manufacturer instructions after undergoing a peripheral nerve evaluation with >50% improvement in her urgency symptoms. At one-week follow-up, her incisional pain was minimal. She did not require oral analgesics and had reported marked improvement in urinary symptoms consistent with the test phase.\nHowever, near the six-month follow-up appointment, she expressed concerns about persistent arousal symptoms in the vaginal area overall with new onset hypersensitivity localized to the clitoris. She did not have these symptoms prior to or immediately postimplant, but she reported gradual development of arousal symptoms postoperatively over the six-month period. She had not initiated the use of any new medications or therapies during the same time period.\nPelvic examination did not demonstrate engorgement of the clitoris, change from the intake examination, or evidence of hypertonic pelvic floor muscle dysfunction based on digital assessment. To manage her arousal, the four programs that were programmed into the system were alternated with cycling activated. Behavioral modifications were suggested including loose clothing. Upon no change in symptoms, device deactivation was performed. This resulted in no notable improvement of the manifest arousal symptoms. However, her urgency symptoms immediately recurred upon the deactivation. At this point, the plan was to trial a new set of programs to see if her arousal symptoms could be eliminated using different settings. The patient agreed to trial all four new programs, each over at least a 10-day period and assess which one was associated with fewer arousal symptoms. The programming was done at sensory levels. Alternation of pulse width and frequency was performed as well. The patient was instructed to complete a 4-week diary indicating arousal and bladder activity. At follow-up, she had trialed each program and continued to experience sexual arousal symptoms. Her symptoms were present even when the device was turned off and intensified when the machine was on. The symptoms were also present without any clitoral contact by undergarments. It was becoming so bothersome that it was difficult for her to stay asleep at night and creating anxiety. Complete testing of the neuromodulator unit was reperformed with normal values noted for impedance. Reprogramming of the unit with a new set of programs was performed again with subsensory levels used at this point. Additionally, she was offered sexual counseling but it was declined.\nOne year after placement, she elected for removal of the generator and lead. The generator and lead, intact with tip, were successfully removed with no complications. At the 6-week follow-up after explant, she had recurrence of urinary urgency symptoms with persistence of hyper-arousability. One year later, she continues to be sexually active with mild discomfort due to vaginal atrophy (as noted on the pain domain in the FSFI). She reports that the clitoris remains hypersensitive with persistence of the arousal symptoms, although moderately improved from prior to SNM. At the follow-up visits, no changes in medications or new medical diagnoses were reported when compared to prior visits. Furthermore, she denied any new stressors or change in her personal life regarding the relationship with her husband. |
A 52-year-old Korean female visited Seoul Veterans Hospital with a complaint of acute pain and gingival swelling on the right mandibular molar region. The patient was systemically healthy and her physical examination showed no significant abnormality. Intraoral examination revealed completely edentulous state. The overlying gingiva of the right mandibular area showed swelling and inflammation clinically. On palpation, bilateral buccal bony expansion was noted on the posterior mandible and maxilla (). She had a complete denture, however her denture was out of use due to the gingival swelling and pain.\nPanoramic radiograph showed diffuse, lobular, and irregularly shaped radiopacities or cotton-wool appearance throughout the alveolar process of both quadrants of the maxilla and mandible. Multiple sclerotic masses with radiolucent borders were found in the maxilla and mandible, confined within the alveoli at the level corresponding to the roots of the teeth, above the inferior alveolar canal (). Cone beam computed tomography (CBCT) (KaVo 3D eXam, KaVo, Biberach, Germany) images also revealed large radiopaque shadow extending into the mandible and maxilla. The bucco-lingual aspects of the lesions could be visualized on the CBCT images, which demonstrated the relationship of the bony lesions to the cortical plates in the bucco-lingual dimension. High density masses were surrounded by a low density layer. The superior border of the mandibular canal was intact (). Some lesions appeared larger and connected with the buccal and lingual cortical plates. On the parasagittal reformatted images at this level, bucco-lingual cortical expansion was observed (). The radiographic appearance varied from radiolucent to mixed lesions or rather radiopaque masses.\nUnder local anesthesia, incision and surgical curettage was performed on the right mandibular posterior area. Irregular bony defects and inflammatory fibrous tissue were seen in the operation field.\nAfter curettage, the swelling was decreased and the patient could wear her denture. Biopsy was performed and it was histologically diagnosed with cemento-osseous dysplasia. Histological finding of this lesion showed the formations of dense sclerotic calcified cementum-like masses. The lesion was composed of cementum-like substances characterized by islands of calcified deposits and areas of loose fibro-collagenous stroma, which showed the evidence of proliferation. The cementum-like substances mainly showed acellular structure ().\nThe patient's mother and twin daughters had similar lesions in the jaws. The familial history and histological findings suggested a diagnosis of familial gigantiform cementoma. The patient of the present case has been followed up over the last 12 month and FCOD has remained asymptomatic. |
A 53-year-old male patient was admitted to a local hospital with recurrent fever and cough in December 2013. He had a smoking history of 40 years, but there was neither immunosuppressive drug intake nor underlying diseases such as tuberculosis, diabetes, and malignancies. Physical examination and laboratory findings were normal except for a body temperature of 37.8°C. Chest computed tomography (CT) showed a 3 × 3 cm mass in the right middle lobe along with multiple nodules in both lungs (fig. ). A thoracoscopic wedge resection of the lesion was then performed, and the biopsy showed suppurative granulomatous inflammation. The patient's symptoms improved after surgery and 1 week's use of piperacillin-tazobactam.\nHowever, he was readmitted with cough, fever, and new-onset low back pain in May 2014. Chest CT scan indicated an emerging nodular lesion in the left lower lobe (fig. ), and magnetic resonance imaging (MRI) revealed bony destruction in the L5 vertebral body and L2/3 pedicle (fig. ). Biopsies from both tracheoscopy and percutaneous vertebral puncture showed chronic inflammation. Meanwhile, tissue sections of lung specimens were rechecked and the suggestion was suppurative inflammation probably caused by Nocardia. However, no further pathogenic evidence was provided. Although the patient was treated with antituberculosis drugs empirically, he still had persistent back pain and developed bilateral lower extremity weakness, which prompted surgical intervention including drainage, debridement, decompression, and stabilization of the lumbar spine.\nNevertheless, he suffered from a repeated moderate fever after surgery and was then transferred to our hospital. A 2 × 2 cm soft mass was palpable in the right paravertebral area. Muscle strength was 4/5 for both lower extremities. A new MRI showed deterioration of the imaging findings (fig. ). Consequently, we performed a second surgery including removal of the original internal fixation, debridement, decompression, and drainage. A few spore or hypha analogues were visualized in tissue sections with Gomori methenamine-silver stain and periodic acid-Schiff stain (fig. ). Then, the patient was initially treated with voriconazole (a loading dose of 6 mg/kg of body weight on the 1st day followed by 4 mg/kg, every 12 h intravenously). Two weeks later, he had pain relief and recovered his normal temperature. Resolution of the lumbar lesions was also distinctly observed. Notably, specimens from the third lumbar surgery primarily for spinal stabilization revealed septate fungal hyphae, dichotomously branching at acute angles (fig. ). So far, the patient had taken voriconazole for more than 3 months, and no recurrence was detected. However, the muscle strength of both lower extremities did not improve significantly. |
An otherwise healthy 45-year-old Asian female was referred to our institution after hematuria evaluation revealed a leftsided partial staghorn calculus encompassing the central region and lower pole calices (). Stone size precluded ureteroscopic or extracorporeal treatment methods, and PCNL was recommended.\nIn the operating theatre while in the prone position, interpolar renal access was achieved above the level of the 12th rib (). Balloon dilatation of the tract was performed and a 30F sheath was introduced as a working channel. Uneventful rigid nephroscopy with ultrasonic lithotripsy followed by flexible nephroscopy was performed with complete stone removal. A re-entry Malecot nephrostomy catheter was introduced for postoperative drainage. A ureteral stent was not placed. Nephrostogram at the conclusion of the procedure revealed no significant extravasation and prompt transit of contrast down to the level of the bladder. There were no hemodynamic or respiratory issues throughout the procedure and estimated blood loss throughout was ∼100 mL.\nChest radiograph obtained in the recovery room was normal 1 hour after surgery. Postoperative hematocrit declined to 33.0 from 41.3 before surgery; however, vital signs were within normal limits. A noncontrast computed tomography (CT) scan was performed on postoperative day (POD) 1, per our institutional protocol, showing a large left pneumothorax and nephrostomy catheter traversing the spleen (). Subsequent radiograph confirmed the pulmonary findings, showing a large left pneumothorax (). A small perinephric hematoma was also noted; however, no significant intraperitoneal bleed was identified. Pulmonary consultants placed a thoracostomy drainage catheter in the left pleural space. Resolution of the pneumothorax was noted on subsequent imaging.\nAfter chest decompression, the patient had no significant complaints. Interval laboratory values and vital signs were all within normal limits and urinary output showed no sign of gross hematuria. The decision was made to proceed with conservation, nonoperative management of the splenic injury.\nThe patient was observed with serial abdominal examinations and laboratory monitoring. The re-entry catheter was removed at the bedside on POD 6 that was tolerated well by the patient. In advance of planned nephrostomy catheter removal, the patient was kept without food or drink after midnight (NPO) should surgical intervention be necessary. However, the patient's vital signs, laboratory values, and physical examination remained unchanged. Delayed chest tube removal was planned to determine whether nephrostomy removal would produce a recurrence of the left pneumothorax. Once stability of chest imaging was determined, the pigtail catheter was clamped for 24 hours and subsequently removed on POD 7 after chest radiograph showed no evidence of pneumothorax. The patient was discharged the following day. Follow-up visit on POD 15 demonstrated an unremarkable physical examination and stability in hematocrit. |
We present the case of a 13-year-old female patient, known with severe visual field loss, who referred for another opinion regarding the ophthalmological diagnosis.\nAnamnesis at presentation revealed that at the age of 9 years and 3 months, on a routine ophthalmological examination, papillary calcification and retinal hemorrhage were discovered in the left eye. At that moment, the suspicion of intracranial calcifications was raised. The patient underwent clinical neurological examination, EEG, and cerebral MRI, all of them revealing a normal aspect. The patient was also recommended fluorescein angiography, which showed papillary autofluorescence. The diagnosis established then was papillary drusen in both eyes, buried in the right eye and mixed in the left eye and the patient was recommended to keep it under observation, together with a periodical examination of the visual field.\nThe patient had had multiple examinations of the visual field over the time.\nThe first visual field examination of the right eye showed an arcuate nasal defect, in the superior nasal quadrant, sketching an aspect of nasal step, structure of the sensitivity defect that in 3 years time evolved into a quadranopsia.\nIn the left eye, the first visual field examination showed inferior nasal quadranopsia, extended superiorly with a nasal arcuate defect respecting 20° centrally, which after three years evolved into a paracentral diffuse defect with an island of central vision of 5°.\nHowever, over the time, the examination of the visual field was made with different types of machines, and no correlation of the modifications could be made objectively.\nThe patient received several different diagnoses from several different ophthalmologists, among which optic nerve drusen; papillary oedema and hamartoma have to be mentioned.\nAt presentation, the patient’s visual acuity was 20/ 20 with correction for the RE and 20/ 20 without correction for the LE, with a refraction ROD: -1 DSf<> -0.75 DCyl, 179* and ROS: +0.50 DSf<>-0.75 DCyl, 167* and a cycloplegic refraction: OD: -0.75 DSf<> -1 DCyl, 168*, OS: +0.75 DSf<> -1 DCyl, 170*.\nThe intraocular pressure was 19 mmHg GAT in the right eye and 13 mmHg GAT in the left eye.\nSlit lamp examination of the anterior segment revealed no pathological changes for both eyes, and the red-discrimination test was also normal.\nFundoscopy presented only with papillary pathological modifications.\nThe optic disc in the right eye was elevated, with relatively clear margins, pink color, and the absence of cupping. At 5 o’clock meridian, a nodular yellow mass, with irregular outline, could be noticed ().\nIn the left eye, the optic disc was also elevated, pale, of irregular outline, and the absence of cupping was noticed. Nodular, yellow, reflective protrusions, with irregular contour and brambleberry shape could be noticed ().\nThe retinal vessels, the macula, and the retinal periphery presented no pathological changes in either of the eyes.\nThe clinical examination suggested the diagnosis of optic nerve drusen in both eyes. B-scan ultrasonography and optical coherence tomography (OCT) examinations were used for the confirmation of the diagnosis.\nB-scan ultrasonography is considered the gold standard method for the detection of optic disc drusen. In this patient’s case, it showed round, hyperechoic structures, observed at the optic nerves of both eyes. The A-scan mode, which was overlapped on the structure only for the left eye, showed hyperreflectivity at the anterior side of the optic nerve, of supraretinal intensity.\nOptical coherence tomography is a useful examination in the assessment of the structure and the anatomical shape of the drusen, and in the analysis of retinal nerve fiber layer (RNFL) and GCL-IPL complex.\nFor patients under 18 years old, however, there is no normative database regarding the normal values of the analyzed parameters, therefore these analyses are useful only for patient’s follow-ups.\nThe OCT scan of the optic nerve showed a prominent aspect of the optic disc, with a lower value of average RNFL thickness in the left eye compared to the right eye ().\nMacula was structurally normal, with an asymmetry of macular thickness, thinner in the left eye, compared to the right eye ().\nThere was also an asymmetry of thickness regarding the GCL-IPL complex, which was thinner in the left eye compared to the right eye ().\nThe investigations confirmed the diagnosis of optic nerve drusen in both eyes.\nThe differential diagnosis in the case of this patient took into consideration the following pathologies:\n• Papilloedema – excluded by B-scan ultrasound;\n• The existence of an intracranial expansive process – excluded by clinical and imagistic examinations;\n• Optic nerve tumors\no Astrocytic hamartoma – the proliferation of astrocytic cells occurs above the optic disc, whereas optic disc drusen is located in the substance of the optic nerve.\no Optic nerve sheath meningioma – excluded by clinical and imagistic examinations.\n• Leber optic neuropathy – it typically presents with severe loss of central vision.\n• Infiltration of the optic nerve (leukemia, lymphoma) – excluded by normal laboratory tests.\nThe patient’s visual field examination at presentation revealed a superior nasal altitudinal scotoma at the right eye (), and at the left eye an important constriction of the visual field, with the preservation of a small 15* island of temporal paracentral vision ().\nThe patient was not recommended any treatment, but only periodical follow-up with visual field examination at every 4-6 months, and annual OCT.\nThe patient came back a year later for follow-up. At examination, there was no progression of the visual field alterations (,), but the intraocular pressure was at the superior level of the normal range, 21 mmHg GAT for the right eye and 20 mmHg GAT for the left eye.\nTherefore, the patient was recommended the treatment with a prostaglandin analogue to prevent the exacerbation of the visual field loss in order to attenuate the mechanical compression on the ganglion cells axons and to improve the blood flow to the optic nerve head. |
This is a 60-year-old male with past medical history of hypertension, aortic dilation, cirrhosis, and first degree AV block who presented with complaints of sudden onset severe holocranial headache, nausea, left arm numbness and weakness, blurred vision, ataxia, and dyspnea. The patient denied any head trauma preceding the onset of his symptoms. His vital signs were stable at time of presentation. Initial laboratory studies performed in the emergency room (ER) including complete blood count (CBC), complete metabolic panel (CMP), hemoglobin A1c, urinalysis, and urine drug screen were unremarkable. A CT brain was done which revealed no acute intracranial abnormalities. Teleneurology evaluated the patient in the ER and calculated his National Institute of Health Stroke Scale (NIHSS) to be three. Tissue plasminogen activator (tPA) was recommended, however, the patient refused. He was admitted for further evaluation and neurology consultation.\nA CT angiography (CTA) head and neck showed a narrow basilar artery without other apparent vascular abnormalities (Figure ). MRI brain showed no acute infarct or bleed. Based on the patient’s presenting symptoms and imaging, the diagnosis of reversible cerebral vasoconstriction syndrome was suspected. He was started on a prednisone taper 30 mg twice daily with progressively decreasing doses every three days and nimodipine 60 mg every four hours. However, his blood pressure was unable to tolerate the nimodipine. Therefore, he was switched to verapamil 80 mg three times per day, which he tolerated better. A repeat CTA head was performed 48 hours after initiation of therapy. The repeat CTA showed improvement in the basilar artery narrowing as compared to the initial study (Figure ).\nThe patient continued to take verapamil daily. Headaches were noted to persist, although gradual improvement was reported daily. Acetaminophen and hydrocodone were used to supplement headache management. He continued to work with physical therapy and occupational therapy who reported daily improvement in motor and sensory deficits. With his continued progression, the patient was discharged to a skilled nursing facility to continue his rehabilitative therapy with instructions to follow up with neurology for repeat imaging 3-4 weeks after discharge. However, the patient was lost to follow-up. |
The first case is of a 57-year-old man who has undergone elective coronary artery bypass grafting for triple-vessel coronary artery disease and required sternal resuturing for sternal wound infection after 1 month of surgery. After 2 months of surgery, an angioplasty was performed to mid-LAD for 95% stenosis in the segment. Later, after 1 month, he was readmitted with repeated episodes of bleeding from the sternal wound and a small pulsating membrane at the upper part of the sternal wound. He was in congestive cardiac failure at the time of presentation, with bilateral pedal edema and crepitations in the chest. A transthoracic echo was done that showed a 40 mm × 44 mm pseudoaneurysm [] arising from the ascending aorta. A CT angiogram of the chest showed a large trilobed and partially thrombosed pseudoaneurysm from the ascending aorta [] with a narrow neck (9.0 mm), which was abutting the sternum and extending into the left parasternal region and compressing the right ventricular outflow tract and main pulmonary artery. The distance of origin of the neck measured 36 mm from the right coronary origin. After assessing the comorbid condition and the risks of redosternotomy for surgical management, the interventional management by occlusion of pseudoaneurysm opening with ASD Occluder was planned. The left femoral artery (6 F) and left femoral vein (7 F) accesses were obtained. Aortic angiogram was performed with a pigtail catheter (6F, merit), which demonstrated the trilobed pseudoaneurysm with a narrow origin from the ascending aorta, as shown by CT angiography. A right coronary angiographic catheter (Judkins Right Coronary, JR2) was used to engage the orifice of the pseudoaneurysm and selective hand injection was carried out to delineate the pseudoaneurysm anatomy. An exchange length 0.035” J tip Terumo wire (Terumo™) was then positioned into its middle lobe using a catheter. A 9 F ASD Occluder delivery sheath (Searcare, Lifetech Scientific(Shenzhen) Co., LTD, China) was then railroaded over this Terumo wire with its tip in the pseudoaneurysm. The ASD Occluder device was selected based on the size of the neck, which measured 9 mm. A 12 mm ASD Occluder (Seercare, Lifetech Scientific(Shenzhen) Co., LTD, China) spanning the middle lobe and the neck of the pseudoaneurysm and the angiogram showed no residual leak []. The patient made an uneventful recovery with a postprocedure CT scan demonstrating complete occlusion of the neck of pseudoaneurysm [] and collapse of the walls of pseudoaneurysm over the device. The features of congestive cardiac failure also improved over the next 5 days with medical support. The 6-month follow-up echo showed device in position and no residual flow across device. |
A 23-year-old female acquired Lyme disease 9 years ago (serology positive) and developed chronic pain due to PTLDS. Her functionality was severely affected; she was bedbound for approximately 5 years and required a wheelchair. Other past medical history includes fibromyalgia, hypothyroidism, anxiety/depression, and insomnia. The patient was scheduled for extraction of symptomatic, impacted molars under general anesthesia.\nBefore surgery, her pain manifested in skin, spine, muscles, bones, and joints. Her baseline Numeric Pain Rating Scale score was 6–7/10 throughout her body, but mostly in her back. She had severe pain episodes requiring emergency department visits and admissions with notably poor response to opioids but relieved with ketamine. She had tried an extensive list of pain therapy modalities and medications and had a history of sustained use of opioid medication for her pain. Finally, she was prescribed methadone but was able to wean off of this treatment 5 months before surgery. She was on buprenorphine 2 mg tablet for breakthrough pain which she used approximately once every 3–4 weeks. Other home medications were thyroid tablets (60 mg) once a day, clonazepam (0.5 mg nightly), and quetiapine (200 mg nightly). She was referred to the preanesthesia clinic for recommendations regarding her perioperative pain management. At that time, multimodal pain management therapy was discussed.\nAnesthesia was induced with propofol and maintained with sevoflurane. Four molars were extracted uneventfully. The patient received fentanyl 250 mcg intravenous (IV), ketamine 100 mg IV, acetaminophen 1000 mg IV, and ketorolac 30 mg IV for pain control. At the end of surgery, local anesthetic was administered in all four quadrants by the surgeon for postoperative pain relief.\nThe patient was extubated in the operating room and transferred to the recovery room. During recovery, in addition to acute oral pain, she developed an exacerbation of nonspecific musculoskeletal pain. The patient received an additional fentanyl 250 mcg IV, hydromorphone 1.2 mg IV, lorazepam 2 mg IV, gabapentin 600 mg p.o., and ketamine 50 mg p.o. Her pain was still severe and uncontrolled; therefore, a ketamine infusion was started at 5 mcg/kg/min. Opioids seemed to work poorly and at higher doses actually precipitated hypoxia. She was transferred to the Intensive Care Unit (ICU) for further pain management. A multimodal pain regimen was used for 2 days and included: ketamine infusion; acetaminophen 1 g IV four times a day; ketorolac 15 mg IV four times a day; sublingual buprenorphine 2 mg once a day; oxycodone 10–20 mg p.o. as needed; and hydromorphone 0.4–1.2 mg IV as needed. Subsequently, she was weaned off these medications and transitioned to oral medications and was discharged home on day 3.\nDischarge pain medications included ibuprofen 800 mg three times a day, oxycodone-acetaminophen (5/325) two tablets four times a day, ketamine 20 mg four times a day, gabapentin 600 mg three times a day, clonazepam 0.5 mg nightly, sublingual buprenorphine 2 mg once a day, and hydromorphone 4 mg every 4 h as needed. The patient was expected to taper off medications for acute pain over a period of several days as acute pain from her dental procedure was expected to resolve over that period of time. |
This was a case of a 23-year old G4 P3 A3 woman at a gestational age of 39 weeks in April, 2017 who was referred to the labour ward of the University of Calabar Teaching Hospital (UCTH), Calabar, Southern Nigeria, with a history of inability to deliver her baby after 5 days in labour prior to presentation. The patient had been managed in two different traditional birth attendant (TBA) homes. The second TBA recognized that the cause of prolonged labour was abnormal lie of the fetus and attempted to correct the lie by intrauterine manipulation through the vagina. This resulted in uterine rupture and subsequent evisceration of the intra-abdominal viscus through the vagina. The loop of the small intestine was initially mistaken for umbilical cord by the TBA who pulled several lengths of it through the vagina (). Following the failed attempt to deliver the baby and the woman's deteriorating clinical state, the patient was subsequently rushed to UCTH.\nAt UCTH, the patient was resuscitated. Parenteral broad spectrum antibiotics were administered. The extruded loops of bowel were wrapped in sterile guaze soaked with warm normal saline. A general surgeon was invited to take part in the management of the patient. The patient was immediately prepared and taken to theatre for emergency laparotomy.\nA midline incision was made to access the peritoneal cavity. Intra-operatively, a macerated female fetus was found in the uterine cavity. There was a left postero-lateral uterine wall tear extending from the mid portion to the posterior vaginal fornix. The loops of bowel extruded through this opening. The dead fetus was extracted. About 1,200 ml of blood in the peritoneal cavity was suctioned. Total abdominal hysterectomy was performed.\nThe whole intestines were thoroughly examined (Figures and ); about half the length of the intestine was devitalized and therefore, resected and an end-to-end anastomosis was done. The peritoneal cavity was lavaged with warm normal saline before abdominal wall closure. Nasogastric tube was then inserted.\nPost-operatively, the patient was given parenteral nutrition for 5 days. Antibiotics, analgesics and other supportive treatments were continued according to our protocols. The patient opened bowel on the eighth post-operative day. The patient was commenced on graded oral meals which the patient tolerated. Subsequent post-operative days were uneventful. The patient was later discharged home. |
A 64-year-old female patient with prior THA presented with a seven-year history of groin pain and weakness during initial hip flexion. The THA was initially performed at a different hospital to address painful dysplastic osteoarthritis. Ipsilateral groin pain was presented soon after the THA. Moreover, stair climbing and exiting cars were aggravating activities. Pressure on the iliopsoas tendon of the groin was painful, especially during the Stinchfield test. A relatively large cup was implanted (58 mm in diameter) in the shallow hypovolemic acetabulum. Insufficient medialization was observed on the anteroposterior (AP) radiograph from the first outpatient clinic (Fig. ). It was thought that periprosthetic joint infection could be the cause of the pain and was excluded using laboratory tests and ultrasound screening. The pelvis tilted upward posteriorly due to global sagittal malalignment associated with multiple spondylolistheses and degenerative kyphotic deformity in the lumbar spine. The upper border of the pubic symphysis was at the level of the third sacral vertebral body on a standing pelvic AP radiograph. The abduction angle of the acetabular component was 57.3°. On the axiolateral radiograph, the anteversion angle of the cup was 30.3°. The protrusion of the acetabular component was approximately 25 mm on the sagittal computed tomography image (Fig. ).\nNSAIDs and physical therapy failed to manage the pain. Multiple ultrasound-guided injections provided only transient analgesia. After conservative treatment failed, revision THA was offered as a treatment of choice, but she refused a revision arthroplasty because she was not satisfied with the index THA. Informed consent was obtained for arthroscopic tenotomy. It had been sufficiently explained that revision THA is necessary if there is no effect. Finally, she agreed to undergo arthroscopic iliopsoas tenotomy instead of a cup revision as an alternative minimally invasive manner instead of revision THA. Hip arthroscopy was performed using a standard traction device in the supine position. Under sufficient hip distraction confirmed via AP fluoroscopic imaging, we used the standard anterolateral viewing portal and a modified midanterior working portal.\nSixty mmHg of fluid pressure was used with an intermittent increase up to 80 mmHg. After arthroscopic interportal pseudo-capsulotomy, psoas bursitis and iliopsoas tendinitis were observed near the acetabular cup overhang (Fig. ). A bifid iliopsoas tendon was identified and tenotomized using a monopolar hook-shaped diathermy probe under endoscopic visualization without incident. Postoperatively, she was managed in a brace with physiotherapy. Her groin pain during initial hip flexion resolved immediately following arthroscopic iliopsoas tenotomy. She was delighted with the result and the minimally invasive surgery. One month postoperatively, she presented to the emergency department with an anterior dislocation of the THA after sitting on the floor in Buddha’s position (Fig. a). After closed reduction, the anterior apprehension test was positive. Hip bracing of the reduced THA was used as she refused further surgery. When reviewed three months after the arthroscopic tenotomy, she still complained of anterior instability and apprehension and subsequently experienced a second dislocation. She gave informed consent for revision surgery of the acetabular component. Revision THA was performed using the previous posterolateral approach. During revision surgery, tenotomized iliopsoas tendons were observed adjacent to the overhang of the protruding acetabular component (Fig. b).\nThe acetabular component was explanted using a cup removal instrument (X-plant, Zimmer). The hip center was medialized to improve coverage of the cup by reaming toward the medial direction. The femoral component was retained. A porous-coated multihole cup was carefully implanted and fixed using multiple screws. A 36-mm delta ceramic head was used. After the multidirectional stability test was performed, the short external rotators were repaired using transosseous fixation. She recovered entirely by three months postoperatively. At 18 months postoperatively, she had a negative Stinchfield test, and she was pleased with the outcome of no subsequent groin pain, flexion weakness, apprehension, or dislocation (Figs. , and ). |
A 68-year-old man with a known recurrent right inguinal hernia presented as an emergency with a 1-day history of right iliac fossa pain, and acute irreducibility of the hernia. There were no symptoms indicative of bowel obstruction.\nHis past medical history included hypertension and chronic obstructive pulmonary disease, and he was taking multiple medications including atenolol, aminophylline and carbocisteine. He was an ex-smoker. His surgical history included recurrent inguinal hernias, with three elective repairs on the right, and one on the left, all using the open mesh technique. His last operation was 20 years ago.\nHe was apyrexial with no signs of dehydration. Examination of his abdomen revealed a 3 × 3 cm lump in the right groin, which was irreducible and tender to touch.\nHis full blood count showed a raised white cell count of 14.8 × 109/l, neutrophils of 12.2 × 109/l and CRP of 48 mg/l. Plain abdominal x-ray was unremarkable. Ultrasound confirmed a right-sided inguinal hernia, with the presence of incarcerated bowel. There did not appear to be a loop, and the sonographer suggested that the hernia may contain the appendix; clinical correlation was advised.\nAt this point, an incarcerated right inguinal hernia with a possibility of appendix or small bowel in the hernial sac was suspected. The patient was prepared for emergency surgery.\nAn open repair of incarcerated recurrent inguinal hernia was planned with consent obtained for possible laparoscopy or laparotomy. An incision was made in the right inguinal region where long-standing adhesions due to previous surgery were noted. An indirect inguinal hernial sac containing an acutely inflamed appendix was identified (Fig. ). The base of the appendix was not visible and it was not safe to proceed through the deep inguinal ring. Hence, the appendix was reduced into the abdominal cavity and a laparoscopic repair was deemed suitable.\nAn open Hasson technique was used to achieve pneumoperitoneum and standard port placement for appendicectomy was used. During laparoscopy the cecum, terminal ileum and appendix were examined. The appendix was grossly inflamed and thickened with a healthy base. There was no evidence of perforation and there was no free fluid or pus present. Laparoscopic appendicectomy was performed and the appendix was removed in an endoscopic retrieval bag.\nThe hernia repair was then completed with a sutured repair, without mesh, due to the increased risk of infection.\nThe post-operative period was uneventful. The patient discharged himself from hospital after one day and there were no post-operative complications. The histology confirmed the presence of acute appendicitis; the macroscopic length of appendix was 80 mm. |
A 48-year-old Chinese woman was admitted due to non-specific complaints of abdominal distension, weakness and fatigue. This patient had been diagnosed with an oophoritic cyst 11 years ago and right oophorocystectomy. Meanwhile, this patient did not have medical history of weight loss, fever, or changes in bowel habits. Physical examination was notable for only splenomegaly. Abdominal ultrasonographic examination showed the increase in volume and multifocal processes throughout the spleen (Figure ). CT scans confirmed the splenomegaly with multiple round and hyperdense lesions in the spleen. Contrast enhancement revealed an early hypoattenuation on arterial and most early portal phase scans. There was heterogeneous to homogeneous enhancement on late portal phase and delayed images. Some delayed scans have described a complete contrast washout with return to isoattenuation. In general, the CT can show an isoattenuated mass within the superior aspect of the spleen, which is barely perceptible on the noncontrast examination. The results from laboratory examinations were normal and the serologic examination for hepatitis A, B and C was negative. Our preoperative diagnosis was hemangioma. Subsequently, the patient underwent splenectomy. Post-operative rough pathological examination revealed a moderately enlarged spleen with approximately 1020 g in weight and 18 × 10 × 6 cm in dimensions. The capsular surface of the spleen showed multifocal sponge-like vascular spaces (Figure ). The nodules with dark bluish red, smooth and multilobulated surface were ranged from 0.3 to 4.4 cm. In addition, these nodules were characterized as having multiple cystic spaces and structures resembling exaggerated red pulp sinusoids [-]. Histologically, this lesion was described as a vascular neoplasm with anastomosing vascular channels lined by histiocytes with occasional papillary structures, which was consistent with LCA (Figure 3). Immunohistochemistry was positive for factors CD31 and CD68 and negative for CD34 and CD21, thus confirming the diagnosis of LCA, and mitigating the diagnosis of angiosarcoma. The patient's post-operative course was uneventful, and her general condition was improved markedly within 100 days after the operation. |
A 63-year-old African American woman presented to the Plastic Surgery clinic from her nursing home with a 2-year history of a painful distal forearm mass. The lesion was initially thought to be a keloid from a stab wound more than 20 years ago; however, she decided to have it evaluated because of a recent increase in size and intermittent bleeding. She denied any numbness, tingling, or weakness of the hand. Her neurovascular examination had normal findings. Clinically, the mass was located on the volar aspect of the mid-forearm, was pink and ulcerated, and 3 × 3 × 4 cm in size (). There was no associated supracondylar or axillary lymphadenopathy.\nBecause of the unique characteristics of the mass, we further evaluated the lesion with a magnetic resonance image of the right forearm. This image demonstrated a 3.8 × 2.1 × 4.1-cm mass in the subcutaneous tissue without invasion into the fascia or muscle (). The lesion was concerning for malignancy, so she was referred to a surgical oncologist for excisional biopsy. One month later, the mass was excised en bloc with careful dissection of the deep margin to maintain the fascia. We used a 6 × 4-cm elliptical incision, and this defect was closed primarily using suprafascial flaps for a tension-free closure.\nOn final pathology, the entire specimen was 6 × 4 × 0.8 cm. It was found to be an invasive nodular melanoma with positive, deep, and radial margins. This case was discussed at our multidisciplinary tumor board with plans of positron emission tomographic (PET) scan, reexcision, and sentinel lymph node biopsy.\nBefore her postoperative follow-up appointment, she underwent a PET scan, which unfortunately demonstrated a hyperactive node in the right axilla as well as a concerning left breast lesion. At her follow-up appointment, we offered a wide local excision with sentinel lymph node biopsy; however, the patient refused to have further surgery. On physical examination, there was a small, red, scaly lesion in the middle of the surgical site concerning for rapid recurrence. We referred her to a breast surgeon for workup of the left breast lesion, which ultimately was found to be ER/PR+ invasive lobular breast cancer. The patient was scheduled for close follow-up in both clinics but had not made her appointments. After reviewing the medical records, the patient refused to have any further surgical procedure at this time. |
A 66-year-old female with no significant past medical history presented to her primary care physician with new onset hematuria. She had palpated a mass in her abdomen eight months prior to presentation, but did not seek medical care at that time. Physical examination revealed a soft, nontender abdomen with a palpable mass in the deep left upper quadrant. Urinalysis confirmed hematuria but was otherwise unremarkable. An abdominal ultrasound revealed a heterogeneous hypoechoic left renal mass with vascular flow and areas of posterior acoustic shadowing suggestive of calcifications within the mass (Figure ).\nFurther evaluation with a contrast-enhanced abdominal CT (computed tomography) confirmed mass localization to the upper pole of the left kidney (Figure ).\nThe mass was well-circumscribed and contained areas of enhancement as well as central necrosis. Calcifications were noted in the periphery of the mass without evidence of active hemorrhage. There was no CT evidence of vascular invasion or metastases. The right kidney had normal imaging appearance.\nBased on the clinical and imaging features, differential diagnosis of renal cell carcinoma versus oncocytoma was considered. To exclude the rare possibilty of uroepithelial involvement and for treatment planning, the patient underwent cystourethroscopy and ureteroscopy which were negative for urothelial lesions. Left renal pelvis could not be evaluated with ureteroscopy due to the narrowing of the proximal ureter by the mass near the ureteropelvic junction. The patient then underwent a left nephrectomy.\nGross pathologic appearance revealed a solid, encapsulated mass measuring 9.3 x 7.9 x 9.4 cm (Figure ).\nAlong the inferior margin of the mass were coarse calcifications, and the entire mass appeared to be surrounded by a thin rim of renal cortex. The mass caused severe compression of the calyces, renal pelvis, and renal parenchyma but showed no involvement of the renal vein. On cut section, the mass demonstrated a rubbery pink, moderately fleshy whorled surface with central tan-white fibrosis and patchy yellow necrosis.\nHistological examination revealed a neoplasm composed of spindle cells arranged in intersecting fascicles, some of which were in a focal storiform pattern. Areas of thick collagen deposition were present and there were numerous branching, hemangiopericytoma-like blood vessels present throughout the tumor. The tumor was hypercellular with areas of necrosis, but no cellular atypia was present. Immunostaining revealed tumor cells to be diffusely positive for CD34 and BCL2 and focally positive for CD99 (Figure ).\nThe tumor cells stained negative for smooth muscle actin, caldesmon, S-100, AE1/3, HMB45, and CD117. The results of immunostaining were compatible with solitary fibrous tumor of the left kidney.\nA follow up magnetic resonance imaging (MRI) of the abdomen showed no evidence of tumor recurrence or metastatic disease two years after nephrectomy. |
A 28-year-old man fell from a height while working as a construction laborer. Roentgenograms displayed combined bilateral fractures of the scaphoid and distal radius. The scaphoid fractures were type B according to the Herbert classification system, and the distal radial fractures were type C according to the AO classification system (Figure ).\nThe patient also sustained an anterior compression fracture of the L1 vertebrae. Open reduction of the intra-articular distal radius fractures and scaphoid fractures was performed under general anesthesia. A dissection was made between the flexor carpi radialis and palmaris longus tendons, and it was extended 3 cm distal to the wrist flexion crease to expose the scaphoid. The flexor pollicis longus tendon was retracted in the direction of the radius, while the median nerve and other tendons were retracted in the direction of the ulna, revealing the pronator quadratus. Next, the distal and radial borders of the pronator quadratus were raised and retracted in the direction of the ulna to expose the distal radius. First, the scaphoid fracture was fixed with a Herbert screw; next, open reduction of the distal radius was performed with the aid of intrafocal leverage achieved via elevation, traction, and fixation using temporary Kirschner wires. The entry site for the Herbert screw at the distal pole of the left scaphoid was comminuted, and to gain stable screw purchase, the Herbert screw was inserted from the palmar proximal toward the dorsal distal, which is a relatively infrequent procedure. No cast immobilization or bracing was used after the surgery. The patient began passive and active range of motion exercises immediately.\nFinally, the distal radius fractures were fixed with locked volar plates. The results of roentgenographic examination conducted 3 months post injury demonstrated complete union of the scaphoid and distal radius fractures (Figures , ). At 9 months after the injury, the range of wrist motion on the right side was 45° extension to 50° flexion, 20° ulnar deviation and 10° radial deviation, with 80° pronation and 70° supination; that on the left side was 40° extension to 40° flexion, 15° ulnar deviation and 10° radial deviation, with 70° pronation and 70° supination. The L1 compression fracture was treated conservatively. The patient was able to resume work at 3 months post injury. |
A 32-years-old man was admitted with the complaints of severe and diffuse abdominal pain associated with mild fever for last 8 h. He had no bowel movement within last 24 h and respiratory discomfort for one and half an hour. He was hemodynamically stable with optimum oxygen saturation and temperature was recorded 99 °F with some signs of dehydration. His abdomen was not distended but it was tender all over and rigid and digital rectal examination revealed normal. Treatment started with intravenous crystalloid infusion with antibiotics (cefoperazone + metronidazole) and analgesics.\nPlain chest and abdominal radiograph with abdominal ultrasound () appeared normal. Hematological and biochemical parameters revealed neutrophilia, mildly raised serum lipase, normal liver and renal function, and no electrolyte imbalance (). Urine analysis showed insignificant pyuria. On the 2nd day he had no significant improvement. So, abdominal CT was done that showed a significant amount of collection in the right sub-phrenic, sub diaphragmatic, and pelvic area ().\nDiagnostic laparoscopy proceeded with modified direct initial trocar access through umbilicus as our routine practice and other ports were placed as needed (). Huge amount of purulent and turbid collection was noted in all potential spaces (; A–D) with extensive fibrinous exudates over the liver, loops of intestine, and in the pelvic cavity. There was no organized lump in the abdomen and small bowel loops were mildly distended. The appendix was found swollen but no perforation (; E). Peritoneal fluids were sucked out and fibrinous exudates were swept out with gauze piece as much as possible. Peritoneal fluid was sent for culture and sensitivity test. Gastro-duodenal area, gallbladder (; F), and ileum were carefully inspected. The gastro-duodenal area was covered with dens exudates, but no bile leak was noted in closed magnified view. Appendicectomy was performed (; E, G) followed by thorough peritoneal irrigation with 2 L of warm normal saline and finally, a drain tube (DT) was placed in pelvis. Operative time was 90 min (; QR for video link to the ). Anesthetic recovery was uneventful. The patient had symptomatic improvement on first post-operative day (POD) and DT collection was noted 150 mL, which gradually declined. Bowel sound appeared on 3rd POD and oral feeding resumed. Peritoneal fluid culture revealed no growth. Histopathology of the appendix revealed acute appendicitis. He was discharged with DT on 4th POD and did not develop any further complications like recurrent abdominal pain, intestinal obstruction, or any abdominal sepsis within 1 year of post-operative follow-up. |
A 65-year-old woman was referred to the Plastic and Reconstructive Department of the University of Perugia in May 2012 for the management of an inflammatory skin disease of both axillae clinically unresponsive to long-term conventional topical therapy. Symptoms included pruritus, burning paraesthesia and pain.\nThe patient was initially treated with a combination of topical corticosteroids and antibiotics for two weeks without improvement. This was followed by topical imiquimod 5% cream for about 4 weeks with out benefit.\nOn physical examination, the lesion in the left axilla measured 4 × 4 cm and the other side one was much bigger, measuring 12 × 10 cm. Both of them appeared as pink erythematous patches with irregular borders and with localised desquamated erosions together with leukokeratotic foci [].\nBilateral breast examination revealed normal breast consistency without any nodules, nipple modification, excoriation or discharge; mammograms showed a normal breast parenchyma confirming the nodules absence. An 18-FDG Pet-Tc was also performed without showing any internal malignancy.\nThe patient underwent operation under general anaesthesia: in the right side we were able to repair the secondary skin defect by an intradermal suture, due to its moderate dimension. For the left lesion the patient was positioned on the contralateral side; left arm was abducted in 90°. After excising the skin lesion with an apparent tumour-free margin of 2 cm, we provided the skin defect reconstruction using a thoraco-dorsal perforator flap, according to Limberg's design [Figures and ].\nDuring the operation we removed two lymph nodes appearing lightly enlarged and of increased consistency: Histopathological examination revealed no tumour invasion but just signs of reactive histiocytosis secondary to sovra-infections of the lesion.\nThe excision specimen of the cutaneous lesion was sent for histopathological examination: There were nests of atypical cells with melanin pigmentation within their cytoplasm showing pagetoid involvement of the epidermis together with cells with abundant basophilic cytoplasm, with signet ring forms. These cells stained positively with Alcian Blue and PAS-diastase stains, confirming the presence of mucin. On immunohistochemistry, the pagetoid cells stained strongly positively for cytokeratin 7 (CK7), cytokeratin 20 (CK20), carcinoembryonic antigen (CEA), EMA and CGDFP-15. These cells also stained positively for melanocytic markers S-100, Melan-A, condition quite rare with very few cases described in the literature.\nThe presence of positivity for CK 7 and negativity for CK 5/6 (an excellent marker of squamous differentiation, characteristically expressed strongly and diffusely by either squamous carcinomas or benign squamous epithelium) allowed us to rule out, together with the specimen histopathological features (intracellular mucin, signet cells and glandular structures), the hypothesis of Bowen's disease.\nThe acinar formation, the intracellular mucin and the positivity of the anti-EMA and anti-CEA antibodies (usually not labelled by melanocytes) were strongly indicative for Paget's disease rather than melanoma.[]\nThe flap survived completely preserving a complete range of motion of the upper limb and the aesthetic result after 24 months was good. No further adjuvant treatments were given after the excision [].\nThe perineal area was also evaluated but was free from any pathological signs such as redness, swelling, dryness, and the patient denied any discomfort or itching in that area.\nThe patient is currently alive without any sign/symptoms of recurrence after 15 months: she continues to be under follow-up. |
In February 2014, a 50-year-old Caucasian female patient was referred to our department due to suspected chorioretinitis in the left eye. The patient reported metamorphopsia in the left eye lasting about 6 months. Both personal and ocular histories were free of any noteworthy facts. VA was 4/4 (ETDRS) natural in both eyes; intraocular pressure was 26 mmHg in the right eye and 23 mmHg in the left eye. The finding in the anterior segment of both the right and left eyes was quite physiological when examined using the slit lamp. Biomicroscopic examination of the fundus of the left eye revealed a clear, prominent whitish tumour with calcification at the centre (Fig. ) sized 1 disc diameter, up in the temporal region at the edge of the macular area, 1.5 disc diameter from the foveola. No supply vasculature or vascular drainage were visible. Furthermore, parafoveal greying of the retina was visible in the parafoveolar temporal regions in both eyes, with small crystalline deposits and impaired capillary network structure with small telangiectasias (Fig. ), better visible in red-free photographic documentation (Fig. ). Autofluorescence of the fundus in the left eye showed a mild decrease in autofluorescence in the area of the tumour with hyperautofluorescence at the calcification site (Fig. ). The macular regions of both eyes showed a mild increase in autofluorescence. Fluorescein angiography showed mild fluorescein leakage at the tumour site in the left eye in late phases, with accentuation of the mulberry-like structure of the tumour. In the macular regions of both eyes, the telangiectasias were coloured in early phases with fluorescein leakage in the foveola in late phases (Fig. ). SD-OCT of the tumour showed proliferation in the retinal nerve fibre layer with a normal structure of deeper retinal and choroid layers (Fig. ) with centrally located calcification associated with an acoustic shadow. In the area of the foveola with normal contours, small cystoid areas were visible in both eyes, as well as small areas of impaired retinal architecture at the junction of external and internal photoreceptor segments (Fig. ). Ultrasound assessment of the left eye showed a solid, highly echogenic lesion in the temporal region above the central area, prominent by 0.65 mm, with high reflectivity 85-90 % with central calcification and an indicated acoustic shadow (Fig. ). The finding was concluded as macular telangiectasia type 2 associated with a solitary retinal astrocytic hamartoma. No skin alterations were shown in the patient, in the sense of neuroectodermal phacomatoses, and her general condition was not associated with neurological symptomatology either. The additionally collected history confirmed no family burden with the above mentioned diseases. The 85-year-old mother of the patient has been followed at our department due to bilateral active neovascular membrane; however, the advanced stage of the finding at the time of diagnosis prevented us from determining whether it had developed in connection with macular telangiectasia of the mother. To confirm our diagnosis and to rule out familial form of macular telangiectasia type 2 we also examined the 47-year-old brother and, with consent of his parents, 7-year-old son of the patient but found no signs of macular telangiectasia or astrocytic hamartoma.\nConsidering that currently no efficient therapy of macular telangiectasia type 2 is known, and given the stability of the astrocytic hamartoma without any signs of growth after 18 months of follow-up, the patient is only actively followed without any therapy for now. |
Abdominal computed tomography for regular monitoring of uterine fibrosis in a 49-year-old woman found a hypodense lesion 7 cm in diameter with a clear boundary near the gallbladder (Fig. a, b). No obvious change in size and internal density of uterine fibrosis had been observed during these 5 years (Fig. b). She had no previous history of alcohol or drug abuse. Ultrasound revealed a well-defined, non-calcified tumor between the gallbladder and liver (Fig. ). Magnetic resonance imaging demonstrated a hypointense tumor that compressed the gallbladder and liver on precontrast T1 mapping (Fig. a). T2-weighted images revealed hyperintense tumor with delayed enhancement on arterial phase and portal venous phase (Fig. b) followed by a delayed washout on the hepatocyte phase. Clinical evaluation and laboratory results were nonspecific, and serum tumor markers including carcinoembryonic antigen, cancer antigen (CA) 19-9, alpha-fetoprotein (AFP) and squamous cell carcinoma antigen were within their normal ranges. The findings were consistent with a gastrointestinal stromal tumor (GIST) or another benign tumor originating from the liver or gallbladder. Accordingly, we planned firstly laparoscopic partial hepatectomy concomitant with gallbladder resection. Lymphadenectomy among the hepatoduodenal ligament were also planned under the situations where malignant tumor was suspected intraoperatively. Under the laparoscopic view, a soft tumor had expansively progressed behind the gallbladder, with dorsal compression of the liver (Fig. ). The tumor was easily separated from Laennec’s capsule of the liver at the gallbladder neck and body without adhesion, which indicates the tumor is benign. Therefore, operative policy changed to tumor enucleation with cholecystectomy. Frozen sections including the surgical margin of the cystic duct were negative for tumor cells. As the tumor was moderately attached to the liver at the fundus of the gallbladder, the liver parenchyma was partially resected en bloc to ensure that the surgical margins were free of tumor tissue.\nThe resected specimen included soft and white tumor tissue with clear boundaries that was located between the liver and gallbladder (Fig. ). Pathological examination following Hematoxylin and Eosin and immunohistochemical staining of tumor specimens was consistent with SFT. The specimens included spindle-shaped tumor cells with elongated nuclei (Fig. a), present in both tangled and patterned arrangements in the subserosal layer of the gallbladder (Fig. b, c). The tumor tissue had a storiform pattern with alternating hypocellular and hypercellular areas with some showing myxoid degeneration. The tumor cells were positive for CD34, CD99 and B-cell lymphoma (BCL)-2 and negative for S100 and alpha smooth muscle antigen (αSMA) staining (Fig. d), and few cells were positive for the cell proliferation marker Ki-67/MIB-1. The tumor cells were negative for SMA, keratin, cytokeratin (AE1/AE3), CD117, epithelial membrane antigen, and desmin. The pathological diagnosis was SFT originating from the cystic plate.\nThe postoperative course was uneventful. She was discharged on postoperative day 9. Chemotherapy was not considered necessary, and postoperative follow up at 18 months found the patient well without any sign of recurrence. Periodical check-up for uterine fibrosis by gynecologist is also continued. |
A 56-year-old woman presented to our emergency department with a posttraumatic abdominal pain as she was punched and hit with a rock several times on both the right upper quadrant of her abdomen and the lower part of her chest 2 hours prior to her admission to our department. She was otherwise well with a medical history of a surgical excision of a hepatic hydatid cyst at the age of 40.\nThere was no history of recurrent cough, hemoptysis, dyspnea, or chest pain.\nThe temperature was 37, the pulse was 88, the respiratory rate was 20, the oxygen saturation was 99% while the patient was breathing the ambient air, and the blood pressure was 14/9 mmHg.\nOn examination, the patient appeared well. No signs of rib fracture were found. On auscultation the lungs were clear. No abdominal nor chest bruises were found. Liver and spleen were palpable with tenderness at the right hypochondriac region of the abdomen.\nThe laboratory investigations were all within normal limits. Arterial-blood partial pressures of oxygen (PaO2) and carbon dioxide (PaCO2) were 68.9 and 36 mmHg, respectively.\nThe electrocardiogram showed sinus tachycardia with no axis deviation nor T wave or ST segment abnormalities.\nIn the chest X-ray, there was a rounded prominence of the left cardiac border with the presence of a radiolucent rim at its superior aspect (crescent sign) (). In the lateral view, well-defined rounded opacity was in the retrocardiac space ().\nCT scan of the chest revealed () the following:\n(i) 31 cm dilation of the pulmonary trunk with a partial occlusion of the left pulmonary artery and a complete occlusion of the right pulmonary artery by a fluid attenuation lesion ();\n(ii) a well-circumscribed fluid attenuation lesion with homogenous content and smooth, hyperdense walls located in the lower lobe of the right lung measuring 30 by 20 by 27 cm ();\n(iii) a subpleural cyst of the inferior lungular segment measuring 19 mm ().\nAbdominal ultrasonography and CT scan were normal.\nThe radiologic investigations concluded massive pulmonary hydatid embolization with pulmonary echinococcosis.\nA transthoracic echography showed no signs of cardiac cysts and there were no signs of pulmonary hypertension.\nThe patient has been admitted to the Department of Cardiology in University Hospital Farhat Hached for treating the hydatid pulmonary embolism. The cardiac surgeons decided to abstain from a surgical intervention. |
A 28-year-old lady presented with dry cough for 2 years and hemoptysis for 1 week. She had three episodes of hemoptysis (100 ml per episode). There was no history of breathlessness, fever, anorexia or weight loss. She was in the first trimester of her third pregnancy. Her chest examination was unremarkable. A chest radiograph showed a well-circumscribed lesion in the left hilar region. A non-contrast computed tomography scan revealed a left hilar mass lesion causing abrupt cut off of the left lower lobe bronchus []. The fat planes with the adjacent mediastinal structures were well-preserved. The patient was subjected to flexible bronchoscopy, which showed a vascular growth occluding the left lower lobe bronchus. In view of the clinical presentation with massive hemoptysis and the presence of a vascular growth occluding the bronchus, a clinical suspicion of carcinoid tumor was considered. An endobronchial biopsy was obtained using an electrocoagulation-enabled biopsy forceps with the application of electrocoagulation current (40 W for 10 seconds in a monopolar mode). The histopathologic examination of the biopsy specimen showed a tumor composed of a mixture of spindle cells arranged in short and long fascicles []. These cells were positive for smooth muscle actin (SMA) confirming their myofibroblastic nature. The tumor stroma showed an inflammatory infiltrate which was predominantly composed of plasma cells. There were no mitoses or necrosis seen. Immunostaining for anaplastic lymphoma kinase (ALK) antigen was positive [] in the tumor cells and the IgG4 stain did not highlight excess of IgG4-positive plasma cells, thus confirming the diagnosis of an inflammatory myofibroblastic tumor.\nShe had ongoing hemoptysis despite being treated with cough suppressants and antifibrinolytic agents (tranexamic acid). The treatment options were discussed with the patient and she opted for surgical resection following a medical termination of pregnancy (MTP). The resected left lower lobectomy specimen showed a circumscribed greyish white tumor (4 × 4 × 3 cm) occluding the left lower lobe main bronchus []. Histopathological examination showed a tumor with similar morphology and immunohistochemical profile as endobronchial biopsy. The bronchial resection margin and the lymph nodes were free of tumor. The patient is currently asymptomatic and on follow-up. |
In February 2006, a previously healthy 58-year-old Sinhalese woman presented to our hospital with a history of intermittent fever, headache, loss of appetite, loss of weight, dry cough, and arthralgia of 5 weeks’ duration. She had no known past medical conditions or any family history of chronic disorders or similar illnesses. She was a housewife who frequently engaged in gardening and had no history of visits to forest areas or working in paddy fields. She was not alcoholic and was a nonsmoker.\nAt the beginning, she had been hospitalized, had undergone investigations, and had received broad-spectrum antibiotics. Due to the nature of the protracted illness with fever, and because she had an erythrocyte sedimentation rate (ESR) above 100 mm/h and increased C-reactive protein (CRP), a course of antituberculous antibiotics had been given over 2 weeks without definitive confirmation of the diagnosis of tuberculosis. While on antituberculous medications, instead of clinical improvement, she showed further deterioration of her illness and developed new problems, including appearance of a tender lump over the right temporal area over the course of 1 week and another similar lump over the anterior aspect of the left thigh of 5 days’ duration. At this juncture, she left the first hospital at her own wish and secured admission to Teaching Hospital Peradeniya (THP).\nOn admission to THP, she looked very ill and feeble and was febrile and tachypneic. She had a tender lump resembling an abscess over the right temporal area. Furthermore, she had similar lumps over the anterior aspect of the left thigh. She had an inflamed, swollen knee and wrist joints with a tender erythematous rash over both shins that resembled erythema nodosum. Her pulse rate was 110 beats/min with blood pressure of 100/65 mmHg. The results of her respiratory system and abdominal examinations were clinically normal. She was conscious, rational, and had no neck stiffness, with a clinically normal neurological examination result.\nBlood samples were taken for basic investigations and culture at this juncture. Diagnostic needle aspirates from the cystic lumps and knee joint effusions were purulent and were sent for bacteriological investigations. Blood samples for cultures were taken under aseptic conditions. Three separate blood cultures were taken in 30-minutes intervals and drawn into standard blood culture bottles. Knee joint and skin lump aspirates also were drawn into separate culture bottles under aseptic conditions. All the samples were transported to the microbiology laboratory, which is located within the hospital premises, and the cultures were done by a consultant microbiologist. Pending investigations, the patient was treated with intravenous (IV) co-amoxiclav (amoxicillin mixed with clavulanic acid).\nOn the fifth day of the patient’s admission, she became drowsy and developed focal seizures involving the right upper and lower limbs and lasting for around 2 minutes. She became drowsy with a Glasgow Coma Scale (GCS) score of 12/15. She moved all her limbs, and her deep tendon reflexes were normal, but she had an upgoing plantar response. She had no neck stiffness, positive Kernig sign, or focal neurological signs. She had persistent tachycardia with low blood pressure of 90/50 mmHg. Her respiratory rate was 30 breaths/min, and she had bibasal crepitations. She had bilateral knee joint effusions, and her knee joints were swollen, warm, erythematous, and tender. A tentative diagnosis of cerebral abscesses was made, and the patient was transferred to the intensive care unit.\nUrgent contrast-enhanced computed tomography of the brain showed a well-defined cystic mass in the right temporal region outside the skull vault and contrast-enhanced changes in the adjacent brain matter. Furthermore, a 12-lead electroencephalogram (EEG) showed a theta-wave focus over the right temporal area with intermittent epileptiform discharges compatible with structural brain damage. The results of her ultrasound examination of the abdomen and an echocardiogram were normal. Her white blood cell count was 3.3 × 109/L with neutrophils of 83% and lymphocytes of 12%. Her ESR was 120 mm/first hour, and her CRP level was 192 mg/L. Her chest x-ray showed multiple peripherally located, ill-defined cystic areas in both lung fields, more in the lung bases, suggestive of multiple lung abscesses. By that time, the blood culture, knee joint aspirate, and skin lump aspirates grew B. pseudomallei, which was confirmed by the local microbiologists with the help of international reference laboratories []. Thus, a diagnosis of melioidosis was confirmed, and treatment commenced.\nFollowing the confirmed diagnosis her antibiotic regimen was changed and started on IV meropenem 1 g 8-hourly for 8 days and later changed to IV imipenem 500 mg 6-hourly, based on the antibiotic sensitivity pattern of the bacterial isolate. Two more antibiotics were added to the regimen at the same time: IV ciprofloxacin 400 mg 12-hourly and IV ceftazidime 2 g 6-hourly. The patient showed gradual improvement of her general condition, and her fever subsided in a few days. However, all three antibiotics were continued for 30 days and changed to an oral antibiotic course comprising cotrimoxazole 1920 mg twice daily plus doxycycline 100 mg twice daily for 20 more weeks. Her seizures were controlled with sodium valproate 200 mg thrice daily. In follow-up, the patient’s chest x-ray, ESR (50 mm/h), CRP (0.7 mg/dl), and liver and renal profiles showed gradual improvement, and the results of her retroviral screening were negative. Her blood sugar and hemoglobin A1c levels were within normal limits. The result of repeat EEG in 3 months’ time was normal, and the patient’s sodium valproate was gradually tapered. After 6 months of antibiotic treatment, her ESR was 12 mm/first hour; her CRP was < 6 mg/L; and all of her other biochemical parameters were normal. Then, she was advised to come back to our hospital for annual screening to check for recurrence of the disease, including ESR and CRP measurement. She has had no recurrence of her illness during the last 13 years and remains well.\nSubsequently, how she contracted the infection was inquired about. She was able to recall contact with soil a few days prior to developing her illness. The footpath leading to her home had a side drain that was filled with mud and soil. She found that a worker had dug up the drain and piled mud and soil on the footpath. The patient, dismantled the piled-up soil with her bare feet and made the footpath accessible. |
Here, we present an 11-year-old patient with LDS. This girl developed an early and rapid progressive enlargement of the aortic root (maximum aortic root size 30 mm, z-score > 7) and therefore underwent valve-sparing aortic root replacement at the age of 2.9 years using a 22 mm Albograft (AlboGraft® polyester vascular graft, LeMaitre Vascular GmbH, Sulzbach/Ts., Germany).\nFollow-up cardiovascular magnetic resonance imaging (CMR) under sedation in 2010, 2011, and 2013 showed good operative results with no evidence of dissection or aneurysm of the aorta. She was lost to follow-up for 5 years from our centre but was then referred for another CMR scan.\nOn examination, we saw an 11-year-old girl in good general condition. Auscultation revealed a continuous murmur in the suprasternal notch area and at the right side of her neck. In addition, there was a soft diastolic murmur at the left parasternal border. Her chest was clear. Medication at that time consisted of metoprolol and captopril. Cardiovascular magnetic resonance imaging showed a large aneurysm (∼50 × 30 mm) at the origin of the subclavian artery (). Additional magnetic resonance imaging (MRI) of the head and neck vessels as well as the abdominal aorta and iliac and femoral arteries demonstrated considerably elongated neck vessels as well as generalized elongation and slight ectasia of the basal brain vessels (), no other aneurysms were detected. The right vertebral artery was arising from the large subclavian aneurysm (). Magnetic resonance imaging of the head and neck vessels gave the impression that it was less well perfused possibly contributing only very little to the basilar artery flow.\nThe ascending aorta distal to the Albograft prosthesis had a diameter of 27 × 34 mm. The aortic valve showed mild regurgitation (regurgitant fraction 16%).\nAfter a multidisciplinary discussion of the findings with the colleagues of interventional radiology and cardiac and vascular surgery, the indication for surgical resection with prosthetic replacement of the right subclavian aneurysm was made.\nSurgical removal of the aneurysm and prosthetic replacement of the right subclavian from the brachiocephalic trunk to the exit of the right mammary artery with a 7 mm Gore-Tex-Vascular Graft (GORE-TEX®, W. L. Gore & Associates, Inc., AZ, USA) was performed via median sternotomy with the use of heart lung machine on the beating heart in moderate hypothermia (26°C) without any complications. Apart from the experience at our centre, the reason for median sternotomy was that the patient needed revision of the sternum due to a severe form of pectus carinatum. Cooling was performed for cerebral protection because it was not clear at the beginning of cardiopulmonary bypass if the right carotid artery had to be clamped during the procedure. The MRI finding of a small/hypoplastic right vertebral artery was confirmed during the operation, and therefore, the vessel was transected.\nA follow-up CMR scan 1 month after surgery demonstrated a patent and non-obstructive Gore-Tex-Vascular graft which was used for the prosthetic replacement of the subclavian artery (). On her most recent outpatient assessment, the patient was asymptomatic. |
A 70-year-old man was admitted to our hospital for a scheduled invasive coronary angiography due to mild exertional dyspnea followed by an equivocal treadmill stress test. His past medical history was notable for arterial hypertension, mild dyslipidemia, and smoking. He denied any family history of sudden deaths.\nThe patient was transferred to the cardiac catheterization laboratory asymptomatic. His resting ECG showed a slight J point elevation of 1 mm in inferior leads (II, III, aVF) (Figure ). The transradial coronary angiography was performed with Judkins left and Judkins right diagnostic catheters of 6F. The radiocontrast used was iopromide, a non-ionic iodine-based contrast agent of low osmolarity. During the procedure, the patient manifested ventricular fibrillation immediately after the intracoronary injections in both coronary arteries that required electrical cardioversion. Each arrhythmic episode was preceded by a further elevation of the J point in the inferior leads (II, III, aVF) during right coronary artery angiography (Figure ) and by J point elevation in lateral leads (I, aVL) during left main artery angiography (Figure ). Of note, a premature ventricular ectopic beat of the same morphology preceded both ventricular fibrillation episodes. Angiography was then completed by additional non-selective infusions from the aortic root (Figure ,F). Coronary angiography did not reveal any sign of obstructive disease. Mechanical complications of the procedure such as stimulation of the myocardium by catheter manipulation, catheter occlusion, deep intubation into the coronaries or superselective engagement were immediately excluded by the operator. The timing as well as the mode of the initiation of the arrhythmia leaded us to the diagnosis of contrast media-induced ventricular fibrillation.\nEven if the non-spontaneous manifestation of the arrhythmia prohibited the diagnosis of an early repolarization syndrome, the pattern in the resting ECG of the patient raised our suspicion of an underlying substrate that could increase his vulnerability to malignant arrhythmogenesis. The hospitalization of the patient was extended during which he underwent a new cardiac ultrasonography with no abnormal findings, a treadmill stress test with no signs of ischemia or arrhythmia and continuous monitoring with no arrhythmic episodes. A ‘Viskin test’, an isoproterenol test, and a flecainide test were conducted with negative results for long QT syndrome, premature ventricular contractions, or Brugada pattern. The patient was discharged with the diagnosis of contrast-related myocardial toxicity. |
Abdominal computed tomography for regular monitoring of uterine fibrosis in a 49-year-old woman found a hypodense lesion 7 cm in diameter with a clear boundary near the gallbladder (Fig. a, b). No obvious change in size and internal density of uterine fibrosis had been observed during these 5 years (Fig. b). She had no previous history of alcohol or drug abuse. Ultrasound revealed a well-defined, non-calcified tumor between the gallbladder and liver (Fig. ). Magnetic resonance imaging demonstrated a hypointense tumor that compressed the gallbladder and liver on precontrast T1 mapping (Fig. a). T2-weighted images revealed hyperintense tumor with delayed enhancement on arterial phase and portal venous phase (Fig. b) followed by a delayed washout on the hepatocyte phase. Clinical evaluation and laboratory results were nonspecific, and serum tumor markers including carcinoembryonic antigen, cancer antigen (CA) 19-9, alpha-fetoprotein (AFP) and squamous cell carcinoma antigen were within their normal ranges. The findings were consistent with a gastrointestinal stromal tumor (GIST) or another benign tumor originating from the liver or gallbladder. Accordingly, we planned firstly laparoscopic partial hepatectomy concomitant with gallbladder resection. Lymphadenectomy among the hepatoduodenal ligament were also planned under the situations where malignant tumor was suspected intraoperatively. Under the laparoscopic view, a soft tumor had expansively progressed behind the gallbladder, with dorsal compression of the liver (Fig. ). The tumor was easily separated from Laennec’s capsule of the liver at the gallbladder neck and body without adhesion, which indicates the tumor is benign. Therefore, operative policy changed to tumor enucleation with cholecystectomy. Frozen sections including the surgical margin of the cystic duct were negative for tumor cells. As the tumor was moderately attached to the liver at the fundus of the gallbladder, the liver parenchyma was partially resected en bloc to ensure that the surgical margins were free of tumor tissue.\nThe resected specimen included soft and white tumor tissue with clear boundaries that was located between the liver and gallbladder (Fig. ). Pathological examination following Hematoxylin and Eosin and immunohistochemical staining of tumor specimens was consistent with SFT. The specimens included spindle-shaped tumor cells with elongated nuclei (Fig. a), present in both tangled and patterned arrangements in the subserosal layer of the gallbladder (Fig. b, c). The tumor tissue had a storiform pattern with alternating hypocellular and hypercellular areas with some showing myxoid degeneration. The tumor cells were positive for CD34, CD99 and B-cell lymphoma (BCL)-2 and negative for S100 and alpha smooth muscle antigen (αSMA) staining (Fig. d), and few cells were positive for the cell proliferation marker Ki-67/MIB-1. The tumor cells were negative for SMA, keratin, cytokeratin (AE1/AE3), CD117, epithelial membrane antigen, and desmin. The pathological diagnosis was SFT originating from the cystic plate.\nThe postoperative course was uneventful. She was discharged on postoperative day 9. Chemotherapy was not considered necessary, and postoperative follow up at 18 months found the patient well without any sign of recurrence. Periodical check-up for uterine fibrosis by gynecologist is also continued. |
A 10-month-old male infant presented for the first time with his parents expressing concerns about his poor response to sound stimuli by not turning towards soft or loud sound sources. The child’s past medical and surgical history was none contributory with an uneventful antenatal period followed by normal full-term delivery. As reported by the mother, he underwent an otoacoustic emissions test at the time of birth and passed. No family history of hearing loss was reported. Physical examination revealed profound sensorineural hearing loss bilaterally. The auditory brainstem response test showed profound hearing loss at 85 decibels above normal adult hearing level (dBnHL) on the left and 90 dBnHL on the right.\nRadiological imaging was performed to assess inner ear structures and to determine the selection criteria for surgical intervention.\nTemporal bone HRCT was performed with a slice thickness of 0.8 mm. This revealed typical bilateral bony abnormalities affecting the inner ear structures but sparing the middle and external ear structures bilaterally. Both the IACs were bulbous in appearance with diameters of 7 mm on the left and 6.8 mm on the right []. Normal diameter ranges from 2–8 mm with an average of 4 mm. The abnormalities included the symmetrical corkscrew appearance of the cochlea and absent bony separation between the basal turn of the cochlea and the IAC (lamina cribrosa) []. In addition, poorly formed modiolus and interscalar septa were noted []. The facial nerve canal and vestibular aqueduct were prominent but were within normal limits. The semicircular canals were preserved. These CT features are considered characteristic of IP-III.\nThe child was given hearing aids, as per his parent’s preference, although cochlear implantation was advised. No improvement was noted following 18 months of regular follow-up and speech therapy, after which the family consented to proceed with cochlear implantation.\nA perimodiolar electrode was selected following discussion with the otology committee accounting for the cochlear malformation and the short area of the basal turn to achieve full electrode insertion. Cochlear implantation was performed on the right side at the age of three years.\nDuring surgery [], the expected perilymph/cerebrospinal fluid (CSF) gushing occurred on exposing the round window, which gradually stopped after five minutes. The electrode of the implant was inserted and then sealed with periosteum to prevent fluid leakage.\nPostoperative audiological tests showed some positive results in hearing; impedance field telemetry was detected, neural response telemetry (NRT) was satisfactory, and speech recognition threshold was seen in two channels.\nHowever, abnormalities were identified on postoperative imaging. The X-ray revealed an abnormal straight course of the electrode [], and a HRCT scan confirmed mispositioning of the electrode, the tip of which was reaching and hooking medially around the internal auditory meatus (IAM) [].\nThe patient was taken back to surgery within 48 hours. The mispositioned cochlear implant was removed, and a new slim straight cochlear implant was re-implanted with great difficulty due to significant bleeding and perilymph/CSF fluid leak. Complete insertion could not be achieved; only 10 out of 24 channels could be inserted. The electrode position was confirmed by HRCT scanning immediately after surgery while the patient is still intubated [] and the audiological NRT test demonstrated a satisfactory response.\nThe postoperative recovery period was uneventful, and follow-up audiological NRT
testing demonstrated a normal response, representing a significant improvement compared to the
initial test.\nThe child is under regular follow-up and, at five years of age, demonstrated normal attenuation of language milestones. No genetic testing was done for him. |
Patient 5 was a 23-year-old man who sustained a left ankle fracture in a motorcycle accident. Because of direct injury to the medial side of the ankle, the soft tissue was too poor for immediate surgery. Images showed a severely comminuted MMFx of the tibia (). The images showed no malalignment of the talus, but its protection by the medial malleolus of the tibia was gone, which means a medial subluxation of the talus could have occurred. Surgical restoration was planned for anatomical reduction of the medial malleolus of the tibia. Due to the poor soft tissue condition, the operation was delayed for 16 days. Despite confirmation of skin necrosis, a surgical approach to the fracture was planned. A skin incision was performed by avoiding the necrotic area (). The L-shaped incision allowed access to the fracture site. The surgical procedure for the fracture was performed as described above. We first fixed the fracture in the deep area of the joint cartilage. Then, the large fracture fragments were fixed using mini-screws. Stability of the talus was confirmed through intraoperative fluoroscopy. It was possible to cover the area over the screws with healthy skin. The area not covered with skin was covered with a negative pressure wound treatment. Immobilization was performed using a posterior short leg splint (). Union of the medial malleolus was achieved at 7 months postoperatively ().\nThe exfoliated skin area was covered with a split-thickness skin graft 16 days after the first surgery. There were no infections or other problems despite soft-tissue damage. The patient began to walk with crutches with partial weight bearing 3 months after surgery. The X-ray taken 7 months after surgery showed union of the fracture with no arthritic changes. The FAOS score of the patient 22 months after surgery was excellent and the patient had recovered to preinjury levels of activity. |
A 9-year-old boy, with a 5 years history of autistic disorder, was referred to one of the author’s child and adolescent psychiatric clinic (J.A.) for the management of his condition. His parents report his developmental stuttering as sound repetition and sound prolongation on first and middle vowels since age 4. At the time the child psychiatrist prescribed risperidone (an atypical antipsychotic); however, the parents refused giving this medication to their son because of their general worries about the side effects of drugs. Over time, the parents came to conclusion that they need to seek professional psychiatric help for their child’s behavioral and educational difficulties. After a thorough assessment of the patient, memantine 5 mg per day was started and increased to 7.5 mg per day after 7 days. The dose was increased to 10 mg per day once again after 1 week. At the beginning of the third week, he encountered deterioration of stuttering and difficulty for starting to speak. His parents explained that the child could only start to speak after a deep and audible breath. Otherwise, he was not able to talk. This problem was only noted at the beginning of the patient’s speech and vanished after a while. No other new stressful or deteriorating condition was thought to aggravate his speech difficulty. Since memantine was the only drug he was consuming, it was decided to reduce the dose of memantine to 7.5 mg per day. Several days after this dose reduction, the patient did not need to take such a deep, audible breath before speaking. However, the deteriorating symptoms of stuttering caused so much distress for the patient and his family that the treating psychiatrist decided to taper down and finally to discontinue memantine. Three weeks after discontinuation of memantine, aggravated stuttering was reduced to its baseline. Then, risperidone was prescribed for the management of autism. |
A 23-year-old married female presented to our outpatient department with complaints of swelling of the vulva and ulcers on the perineal folds for 5 years. The swelling started in 2011 during 1st trimester of gestation, which was gradually progressive (1–5 cm in size). Few painless ulcers developed on the inner aspect of both thighs after 1 year, which gradually increased in number and size. Gradually, she developed pain in the perineum. There was no history of preceding fever, chronic cough, weight loss, oral ulcers, loose stool, and blood in stool. She gave a history of cesarean section, which was performed due to genital prolapse. Then, she was operated for prolapse, and later, biopsy was taken from the swelling to rule out postoperative lymphangioma. The biopsy showed nonspecific granulomas and chronic infiltrates. She was started on oral azathioprine, antibiotics, metronidazole, and steroids with some improvement in groin ulcers and a decrease in vulvar edema and induration. She was on and off treatment for past 3 years with partial relief at best. Meanwhile, she took antituberculosis treatment for 3 months from the gynecology department of a tertiary hospital but did not show any improvement.\nThe condition worsened, and the patient presented in our OPD with complaints of vulval swelling, fissuring, and typical “knife-cut” ulcers, as shown in .\nOn examination, an irregular firm, lobulated swelling arising from the vulva was appreciated. There were multiple, oval to linear, typical knife cutting deep ulcers with well-demarcated margins, of size varying from 0.5 to 5 cm. The ulcers were tender and showed fresh bleeding.\nWe repeated a biopsy which showed epithelioid cell granuloma in the dermis with dense chronic inflammatory infiltrate comprising lymphocytes and plasma cells, as shown in . Special stains for acid-fast bacillus and reticulin were noncontributory. Our differential diagnosis at this stage included vulvar CD, cutaneous tuberculosis, sarcoidosis, deep fungal infection, and lymphogranuloma venereum.\nColonoscopic biopsy was done from the terminal ileum, which showed only mild inflammatory changes without any features of granulomatous disease. Colonoscopy was normal. Anti-neutrophil cytoplasmic antibody (both p ANCA and c ANCA) and anti-saccharomyces cerevisiae antibodies were negative. Routine hematological investigations and angiotensin-converting enzyme levels were within the normal range. Hepatitis B surface antigen, enzyme-linked immunosorbent assay for HIV, and Venereal Disease Research Laboratory test were negative. Tissue fungal culture was negative. The chest X-ray was normal. Magnetic resonance imaging of the pelvis showed vulvar edema with soft-tissue thickening within superficial subcutaneous layer extending up to the perineum likely inflammatory. In view of the clinical and histopathological features and all other investigations, a diagnosis of CD of the external genitalia was made by the exclusion.\nIn view of the previous nonresponse, we planned to start biologic therapy in form of injection adalimumab 160 mg (8/8/16)–80 mg (22/8/16)–40 mg (6/9/16) s/c at an interval of 2 weeks for 1 year, along with azathioprine 50 mg bid.\nThere was significant healing of the ulcers after six cycles of adalimumab, as shown in . |
A 42-year-old man was referred to our hospital for sudden back and chest pain. He had a history of dyslipidemia. He had no history of traumatic injury, but had received acupuncture treatment on his back. At hospitalization, his blood pressure was 125/69 mmHg and heart rate was 91 bpm. Laboratory examinations revealed white blood cell count of 15,400 cells/µL, hemoglobin level of 13.3 mg/dL, and C-reactive protein level of 0.05 mg/dL. Contrast-enhanced CT showed a post-mediastinal hematoma and unusual vessels, which branched off at the same level as the abdominal aorta (superior mesenteric artery [SMA]) and formed an aneurysm. From the aneurysm, unidentifiable vessels branched to both cranial and caudal sides of the aorta (). The vessel branching off on the cranial side of the aneurysm ran along the aorta, and seemed to be connected to the left subclavian artery. The patient was diagnosed with aneurysm rupture attributed to an unusual vessel leading to the aneurysm. After obtaining informed consent from the patient, we performed emergent angiography to embolize the ruptured aneurysm. The procedure was performed under local anesthesia as follows: A 5-Fr sheath was introduced via the left radial artery, and a 6-Fr sheath via the right femoral artery. At first, to identify the unusual vessels branching off the aneurysm and the point at which the rupture had occurred, an angiogram of the left internal thoracic artery (LITA) was obtained to prevent the sheath from passing through the aneurysm. However, the angiogram obtained from the LITA and left common carotid artery (LCCA) showed neither an unusual vessel nor the aneurysm. However, in the venous phase, the vessel, which was considered to be unusual on a CT image, could be confirmed. Thus, a 6-Fr sheath was introduced from the right femoral artery, and a 4-Fr CXI support catheter (Cook Medical in Japan, Tokyo, Japan) was advanced to the aneurysm branching off the abdominal aorta. Digital subtraction angiography (DSA) images were obtained. With the assistance of a road map, the upper side of the unusual vessel branching off the aneurysm was catheterized using a 0.014-inch guide wire (Cruise, ASAHI INTECC J-sales, INC., Tokyo, Japan). Then, DSA images were obtained again ().\nThis procedure revealed that the unusual vessel branching off the aneurysm was a thoracic duct, which ran into the left subclavian vein. A diagnosis of aneurysmal-thoracic duct fistula associated with aneurysm rupture was made. Therefore, to isolate the aneurysm, both the aneurysmal ostium from the aorta and the thoracic duct had to be embolized. Using a 0.018-inch embolization coil (Tornado®, Cook Medical in Japan, Tokyo, Japan), we embolized both the cranial and caudal sides of the thoracic duct. DSA images then showed that all flow from the distal side of the aneurysm had disappeared. Similarly, using an 8-mm Amplatzer™ vascular plug (AVP, St. Jude Medical, St. Paul, MN, USA), we embolized the proximal side of the aneurysm. Angiography, performed before and after AVP2 placement, confirmed total occlusion of the aneurysm and successful embolization (). A vascular closure device was used to close the right femoral puncture site, and the embolization procedure was completed.\nThe postoperative contrast-enhanced CT showed no blood flow into the aneurysm, revealing disappearance of the post-mediastinal hematoma. The patient made good progress after the operation, and discharged on postoperative day 7. The follow-up CT examination after 3 months revealed total embolization of the aneurysm, with no post-mediastinal or retroperitoneal hematoma. |
An ill-appearing 19-year-old male with the one-year history of asthma presented to the emergency room with non-specific symptoms including fatigue, dyspnea, numbness in the right leg, nausea, vomiting, and dizziness. Two months prior to presentation, he had a sinus surgery and within few days after this surgery, he developed cough and dyspnea, so he was admitted to outside hospital for possible pneumonia. He was treated with cefuroxime, Tamiflu, and oral prednisone. He improved momentarily with steroids. Two weeks later, he returned to the outside hospital complaining of right foot plantar numbness and dyspnea, he was discharged home on Levaquin as they thought he may have some residual sinus disease left. One week later he was seen by a pulmonologist at outside hospital and they noticed that one of the cultures grew staph, hence started on Bactrim. He took Bactrim for three days and his mother noticed that he developed some mental status changes, hence Bactrim was stopped. After this, no more symptom of mental status change was noticed. Over the next few weeks, the patient noticed tachypalpitations, continued to have fatigue, shortness of air, and fatigue so the family decided to come to our hospital's emergency department for further workup. While in the emergency room, he was found to be in atrial fibrillation with the rapid ventricular response and elevated troponins. The patient spontaneously converted into sinus rhythm within 10 minutes. His vital signs were stable except for tachycardia with a heart rate of around 100 beats per minute. Physical examination was unremarkable with a normal sensation on right leg and foot. He was admitted to cardiac intensive care unit for further workup due to elevated troponin.\nSalient laboratory values and electrocardiogram\nThe patient’s initial complete blood count was remarkable for white blood cell of 28,800/ul with eosinophil count of 12,960/ul (45%) in spite of the use of low-dose oral corticosteroids for a few days prior to admission. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were both elevated at 35 and 4.14, respectively. His admission troponin was 16.28. His initial electrocardiogram (ECG) showed atrial fibrillation with a heart rate of 161 beats per minute, non-diagnostic Q waves in the inferior leads, T-wave inversions in the inferior leads and no significant ST segment changes noted (Figure ). His repeat ECG 10 minutes later when he converted to sinus rhythm showed sinus tachycardia with a heart rate of 100 beats per minute, Q and T changes as noted earlier, as well and no significant ST segment changes noted (Figure ). Other labs, including TSH, UDS, BNP, lactate, and renal function, were unremarkable. Rheumatological workup including anti-nuclear antibody (ANA), perinuclear antineutrophil cytoplasmic antibody (p-ANCA), cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA), rheumatoid factor, myeloperoxidase (MPO) antibody, serine protease antibody 3, and anti-cyclic citrullinated peptide (anti-CCP) IgG was inconclusive. However, the patient’s IgE and IgG were both markedly elevated. Several infectious causes, such as histoplasma, coccidioides, strongyloides, cytomegalovirus (CMV), human immunodeficiency virus (HIV), tuberculosis (TB), Epstein-Barr virus (EBV), hepatitis B, and hepatitis C, were explored and all were negative.\nImaging\nTransthoracic echocardiogram revealed an ejection of 55% with some apical hypokinesis. The transesophageal echocardiogram showed no evidence of endocarditis, thrombus, shunt, or atherosclerosis. Computed tomography angiography (CTA) of the chest with and without contrast showed moderate mediastinal and bilateral hilar adenopathy in addition to bilateral axillary lymphadenopathy, bilateral peribronchial thickening, and patchy ground-glass opacities most predominantly in the posterior lower lobes. There was no evidence of pulmonary embolism (Figure ). Cardiac magnetic resonance (CMR) showed several areas of delayed enhancement within the left ventricular myocardium and decreased perfusion in the mid to apical septal and inferior segments throughout the apex. It also revealed a small pericardial effusion and minimal hypokinesis of the lateral apical wall (Figures -). Due to the abnormal myocardial enhancement, a CT of the heart with coronary CTA was ordered which showed normal coronary artery anatomy with no evidence of stenosis, calcified plaque, or soft plaque (Videos -). Due to his reported neurologic symptoms, CT of the head without contrast was ordered and showed two areas of low-attenuation within right frontal white matter. MRI of the head was subsequently performed which showed many small bilateral punctate infarcts throughout cerebrum and a few additional ones in the cerebellum.\nBiopsies\nA bone marrow biopsy showed normocellular bone marrow for age and no concern for dysplasia; however, both the bone marrow biopsy and peripheral blood smear showed marked eosinophilia with leukocytosis. Several transbronchial cryobiopsies were taken from the left lower, upper lobes and lingula of the lung which showed patchy areas of eosinophilic venulitis with dense eosinophilic infiltrates involving many of the small venules. This process was happening in the background of chronic bronchiolitis with abundant eosinophils within small airways, smooth muscle hypertrophy, and goblet cell metaplasia (which suggests asthma). All these findings taken into consideration together suggested EGPA (Figure ).\nThe patient was initially started on 1000 mg of intravenous methylprednisolone for three days and then 1 mg/kg/day of oral prednisone for several months with a gradual taper. He was also started on cyclophosphamide for three to six months. Additionally, due to the patient’s young age, arrangements for sperm preservation were made prior to starting cyclophosphamide. The patient responded well to the treatment and at his one-month rheumatology follow-up, the patient continued to improve. His troponin-I reduced to 0.08 at one month visit. |
A 60-year-old woman underwent transurethral resection of bladder tumor (TURBT) at our institute in 2004; her pathological diagnosis was a high-grade UC with adenocarcinomatous differentiation (pT2a, G2>G3). Radical cystectomy was conducted. Only carcinoma in situ (CIS) was found in the surgical specimen, and the surgical margin was negative. There was no cancer cell infiltration in the resected uterus or anterior wall of the vagina, and no lymph node involvement was detected. The patient developed continuous pain and bleeding from the residual vagina in 2010, and a tumor was found in the residual vagina; magnetic resonance imaging (MRI) showed it to be located on the anterior wall (). A biopsy of the tumor revealed a pathological diagnosis of adenocarcinoma (). Computed tomography (CT) and bone scintigraphy revealed no metastasis. Based on a preoperative diagnosis of a primary adenocarcinoma occurring on the residual vagina, tumor resection was performed (). The sigmoid colon was partially resected as it was strongly adherent to the tumor. On pathological examination, adenocarcinoma and SCC were detected (); on immunohistochemistry, sections of the tumor were positive for the SCC markers CD56, chromogranin A, and synaptophysin and were negative for the urothelial carcinoma markers GATA-3, p63, uroplakin, thrombomodulin, and 34βE12. We then reexamined the original TURBT specimen and confirmed the presence of SCC (). Adenocarcinoma and SCC were mostly present in the superficial layer of the TURBT specimen, while high-grade UC was found in the deeper layers where muscle invasion was present. Based on these findings, the tumor was diagnosed as a recurring bladder tumor. Local recurrence and pelvic bone metastasis were detected via MRI 3 months after the patient underwent surgical resection of the vaginal recurrence, whereupon she underwent radiation therapy (52 Gy, 26 fractions). She developed ileus in January 2011 and underwent release surgery. Subsequently, multiple lung metastases and local recurrence in the pelvis developed in June, and she died of disease progression the following month. |
A 65-year-old African woman presenting with abdominal pain was referred to our hospital. Her medical history and family history were unremarkable. She had no history of previous abdominal surgery. On examination she looked healthy with no clinical jaundice or pallor. An abdominal examination revealed a large upper abdominal mass with thinned overlying skin. It had minimal mobility and was not tender. The rest of the examination was normal. Her hemoglobin, on admission, was 7.0g/dL. She was transfused and underwent an esophagogastroduodenoscopy, which revealed a submucosal tumor at D2 and D3. A biopsy was obtained but was reported as nonspecific. A computed tomography (CT) scan of the abdomen revealed a 12×13cm retroperitoneal mass in the region of the head of the pancreas (Figure ). There was no evidence of metastases to her liver or lung. From these radiographic findings, we diagnosed a submucosal tumor of the duodenum. The patient underwent an elective exploratory laparotomy. No evidence of local invasion of the pancreas or of distant metastases was found. The tumor had greatly decreased in size, and it was thought that the liquid of the tumor had probably emptied into the duodenum through a fistula between the tumor and the duodenum. Considering that the pancreas and major papilla were not involved, a local resection was performed, with a 1cm disease-free margin. A retrocolic Roux-en-Y loop was then created and the edges of the defect in the duodenum joined to the jejunal limb of the Roux-loop by a hand-stitched side-to-side duodenojejunostomy anastomosis using a 3-0 Vicryl. In addition, a resection of the right hemicolon was performed due to tumor infiltration of the right curvature of the colon. An ileotransversostomy was performed to reconstruct the gastrointestinal passage. The operative time was 200 minutes and estimated blood loss was 100mL. Microscopic examination with hematoxylin and eosin staining of the tumor showed spindle shaped and epithelioid cells with mild nuclear pleomorphism (Figure ). Immunohistochemistry revealed that the cells strongly expressed CD117 (Figure ), with focal expression of CD34 (Figure ). Therefore, the final histology was consistent with the diagnosis of a duodenal GIST. Based on the above findings, the tumor was finally diagnosed as a GIST with high-grade malignancy originating from the duodenum. A molecular genetic analysis for KIT protein mutation was not performed because of its unavailability at our institute. After the operation, the postoperative digestive opacification showed no digestive fistula (Figure ). The patient was treated with imatinib. She was doing very well with no evidence of disease recurrence when she was last seen 24 months after her operation. |
Patient B, a 75-year-old man was admitted to the emergency department because of recurrent syncope in the last 2 months. His medical history included hypertension, paroxysmal atrial fibrillation, left ventricular hypertrophy, and angina pectoris. Laboratory evaluation and echocardiography showed no abnormalities. His electrocardiogram revealed sinus bradycardia with a left heart axis and a first degree AV block (PQ duration: 380 ms), as well as a right bundle branch block (QRS duration: 146 ms). A pacemaker was implanted because of this trifascicular block. Postimplantation, a cardiac MRI was performed as part of a clinical trial (Fig. ). Unexpectedly, this MRI showed an endocardial tumor in the right atrium and right ventricle. The tumor also involved the tricuspid valve, thus restricting blood flow from the right atrium to the right ventricle. The patient's syncope was probably caused by this obstruction of blood flow, but arrhythmias could also have played a role. No other tumor localizations were found on FDG-PET/CT. He subsequently underwent intracardiac echocardiography-guided biopsy of the tumor. Cytologic examination showed blast-like cells with nucleoli. Immunohistochemical staining was positive for CD20 and PAX5 confirming the diagnosis of DLBCL. There was not enough tissue for FISH analysis. A staging bone marrow biopsy showed no localization of lymphoma; therefore, he had a stage IE primary cardiac lymphoma. HIV status was negative. The patient had a good left ventricular ejection fraction and was treated with six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) followed by two cycles of rituximab alone. He achieved a complete response.\nTwo years after the initial presentation he experienced rapidly progressive cognitive decline as a result of central nervous system (CNS) relapse with multifocal parenchymatous lesions on MRI. He quickly recovered after starting dexamethasone and subsequently received high-dose methotrexate (3 g/m2). Although initially responding to therapy, he died 2 months after initiation of chemotherapy due to progressive disease. |
A 38-year-old female was brought to the emergency department with a total scalp avulsion injury which happened 8 h ago. She complained of pain in the head and neck. She was conscious, oriented and vitals were stable when she arrived to casualty. The avulsed scalp was brought with an ice pack which was inspected and found to involve bilateral eyebrows, the root of the nose and total scalp []. The scalp dressing was opened in the operation theatre. There were no neurological deficits, but tenderness was noted on the cervical spine. Head and cervical spine computed tomography scan was done and which had shown doubtful instability in the subaxial cervical spine at C4-5 level []. Magnetic resonance imaging (MRI) could not be performed due to progressively prolonging ischemia time of the scalp and also the active bleed, which required immediate attention. Hence, discussion with neurosurgeon, plastic surgeons and anaesthesiologist concluded at the procedure, targeting at minimally mobilising the neck. This decision was also taken considering the absence of neurological deficit, lateral mass and facetal fractures, which provided clue towards the probable lesser degree of trauma to the cervical spine. The decision was made to do the replantation with hard cervical collar in situ and post-operative MRI spine. The patient was anaesthetised with intubating laryngeal mask airway (LMA) with cervical collar in situ. The horseshoe stand was placed for better access to the posterior scalp, and anterior part of the collar was removed and cut a bit upper lateral part. Initial haemoglobin was 11.5 g % with packed cell volume 38%.\nWith two-team approach, the bench dissection was started. Bilateral superficial temporal artery and vein, bilateral supraorbital vein and left supratrochlear vein were identified. The post-auricular and occipital vessels were not dissected as it was not intended to do the anastomosis of these vessels. The other team dissected on patient simultaneously. The scalp was placed and inset was done in post-auricular region bilaterally. The occipital area inset was not possible. Quilting sutures were placed at vertex with 3-0 Vicryl®. The right superficial temporal artery and vein, bilateral supraorbital veins and left supratrochlear veins were anastomosed. The left superficial temporal artery and vein were anastomosed with vein graft. The scalp started to bleed once anastomosis of artery was done. It was difficult to achieve haemostasis on the posterior scalp. Intraoperative blood loss was significant and the patient received 2 units packed red blood cells, four fresh-frozen plasma along with crystalloids. The procedure time was 9 h.\nPost-operative day 1: The haemoglobin dropped to 6.6 g%, which was corrected with 2 units packed red blood cells and was maintained around 10 g%. The patient was extubated uneventfully. On post-operative day 3: MRI of cervical spine was done and showed no evidence of the cervical spine injury. Hence, hard cervical collar was removed. On post-operative day 15, the patient was taken to the operation theatre and necrosed left occipital area was debrided along with the left eyebrow area. Vacuum-assisted closure (VAC) dressing was done. There was progressive necrosis of scalp in occipital extending to the parietal area due to infection. The patient was taken to the operation room, the scalp was debrided and split-thickness skin graft (STSG) with VAC was done on day 27. The scalp and STSG settled with minimal raw area which healed with dressing [Figures and ]. |
A 3-year-old girl was referred to a tertiary metabolic bone disease unit for premature loss of primary teeth with roots intact and low serum ALP activity (123 IU/L; reference range: 230–700 IU/L) []. Routine genetic testing revealed compound heterozygosity (c.350A > G, p.Y117C, c.400_401AC > CA, p.T134H) for different TNSALP missense mutations in exon 5 of the ALPL gene, confirming the diagnosis of HPP. On presentation, radiologic assessment of the left hand and arm showed tongue-like lucencies projecting into the metaphyses consistent with childhood HPP. She did not have any clinical features of skeletal involvement of the lower limbs and no motor developmental delay except for a mild waddling gait as a younger child. The patient had a relatively asymptomatic clinical course until she presented at age 11 years with swelling and tenderness of the left ankle that was nonresponsive to paracetamol or ibuprofen. An MRI scan of the ankle suggested a diagnosis of chronic recurrent multifocal osteomyelitis, which was subsequently confirmed by biopsy. The symptoms of pain and swelling of the lower limb joints showed spontaneous transient improvement at age 13 years. Recurring at age 14 years, the symptoms fluctuated and caused significant pain and disability. These symptoms eventually stabilized when the patient was transitioned to adult care at age 17 years. At age 18 years, she successfully underwent radiofrequency ablation for Wolff-Parkinson-White Syndrome, a cardiac disorder unrelated to HPP. The patient is now 27 years of age and has experienced an episode of metatarsal stress fracture; she also suffers from generalized aches and pain.\nOver 22 years, this patient was hospitalized 3 times for a total of 19 days (Table ). Only 1 hospitalization exceeded 3 days, when the patient was admitted for 14 days to receive intravenous antibiotics for suspected osteomyelitis (Table ).\nOutpatient specialist visits, outpatient procedures, and day case procedures represent the majority of healthcare resources used by this patient (Fig. ). Seven specialists provided care for the patient; a pediatric dentist was seen on 40 occasions. Dental procedures, including restorative dentistry (performed on 3 occasions), were the most common of these. This patient was also seen by a pediatric rheumatologist and psychiatrist (Table ). |
A 68-year-old gentleman presented with a 2-year history of progressive left nasal obstruction. There was no associated dysgeusia or hyposomnia or anosmia. He was a lifelong non-smoker and was not known to have allergies to medications or an atopic type of rhinosinusitis. He did not have any other significant medical or surgical history and no genetic history of nasal pathologies.\nOn examination, he had severely reduced left nasal airflow. Anterior rhinoscopy revealed a large fleshy tumour in the left nasal cavity, which was not papillomatous or vascular (). Nasendoscopic examination showed a large tumour of solid and polypoidal components. It occluded the posterior nasal choanae, filling the inferior aspect of the nose. Visualized from the right nostril, the tumour prolapsed into the postnasal space. There was no contact bleeding. The right nasal passage appeared normal. The nasopharyngeal vault and the remainder of the upper airway appeared normal. He did not have otitis media with effusion in the left ear and there was no palpable cervical lymphadenopathy. Pre-operative imaging was ordered to determine the extent of the lesion ().\nTreatment was local excision under general anaesthetic. The tumour was attached to the lateral wall of the nose at the level of the middle turbinate and was removed in its entirety excision. There was minimal bleeding and the nasal passage looked otherwise normal. Middle meatal antrostomy revealed scant mucus in the antrum, but the mucosa appeared normal. The perioperative course was uneventful, and the patient was discharged home on the sameday.\nThe resected mass was a grey polypoidal tissue with spicules of bone measuring 36 × 33 × 18 mm.\nHistological examination revealed the mass was lined with ciliated respiratory epithelium, which invaginated into the core of the polyp, forming glandular and cystic structures. There was focal calcification within the cysts. There was a component of bland sero-mucinous glands in lobular arrangements. The epithelial elements were encased in a hyalinized fibrous stroma. No features of atypia or malignancy were visualized ().\nOn follow up, he was asymptomatic and had normal nasal airflow, taste and smell. |
A 71-year-old male patient who was a known case of metastatic renal cell carcinoma on treatment with the tablet pazopanib 800 mg OD since 5 years presented to us for the first time in ER on 19.06.2018 with complaints of shortness of breath and easy fatigability.\nOn examination, the patient was breathless and had a sallow complexion with dry skin and peripheral edema with prominent visible neck veins.\nThe patient's blood pressure was 100/70 mmHg with tachycardia and oxygen saturation of 92% at room air. The patient had demonstrable pulsus paradoxus with an inspiratory drop in a blood pressure of more than 16 mmHg.\nBedsides, a 2D echocardiogram showed a large pericardial effusion with right atrial right ventricular diastolic collapse suggestive of cardiac tamponade as shown in .\nA primary working diagnosis of suspected disease progression with pericardial/cardiac metastases was made.\nThe patient was immediately shifted to the Intensive Care Unit and underwent pericardiocentesis. About 350 ml of yellowish golden colour fluid was aspirated which was sent for cytological and biochemical investigation.\nImmediately post procedure, the patient showed significant improvement in cardiopulmonary status. His tachypnea and tachycardia subsided, and the patient maintained adequate oxygen saturation on room air. Post pericardiocentesis, the 2D echocardiogram revealed minimal pericardial fluid with no evidence of cardiac tamponade.\nPericardial fluid analysis showed degenerate and mesothelial cells and no evidence of any malignancy or acid fast bacilli.\nHowever, to rule out disease progression, the patient had an 18-fluorodeoxyglucose (FDG) PET-CT scan () which showed regression of the necrotic mass of the left kidney, stable proximal left renal tumor thrombosis, stable lesion adjacent to tumor thrombosis in the anterior calyx, stable metastatic right lung nodule, and interval development of bilateral pleural effusions and mild to moderate pericardial effusion with pericardial catheter drainage tube in situ.\nConsidering his history and findings, we did a thyroid function test which was suggestive of severe hypothyroidism with high TSH levels (TSH > 100 mIU/L) and low T3 T4 levels.\nThe patient did not have any prior reports of TSH or thyroid hormone levels. Antithyroid peroxidase antibody testing was done which was negative.\nThe patient was treated with the tablet levothyroxine 50 micrograms daily which was later gradually increased to 125 mcg daily. The patient's condition improved significantly and was discharged in a stable condition 1 week post removal of the pericardial drainage tube.\nFollow-up echocardiogram after a period of 2 months showed near total resolution of pericardial effusion. Within a few weeks, all his signs and symptoms completely resolved and the patient is currently continuing treatment on pazopanib tablet as part of his renal cell carcinoma treatment.\nThe patient is on regular follow-up, and his latest 2D Echo on 10.10.2018 showed minimal pericardial effusion with good systolic left ventricular function. The patient is currently hale and hearty on thyroid replacement and pazopanib tablet treatment with a normalized TSH value of 0.97 mIU/L. |
A 70-year-old Asian male presented to the hospital with worsening dyspnea on exertion and severe fatigue. He also reported an “issue with his heart” and a murmur which had been present since childhood. The patient noted absence of chest pain, orthopnea, or palpitations. He had a past medical history consistent with essential hypertension and cataracts but denied any history of diabetes, dyslipidemia or smoking. Family history of cardiovascular-related disease was non-existent.\nOn examination, his vitals were stable. The patient weighed 45 kg with a body mass index (BMI) of 17.2 kg/m2. Cardiovascular exam was significant for a 4/6 systolic murmur with a palpable thrill best heard at the left lower sternal border and a right ventricular heave.\nTransthoracic echocardiography was significant for a left ventricular ejection fraction (LVEF) of 65%, as well as moderate to severe mitral and tricuspid regurgitation (, ). Transesophageal echocardiography revealed a normal left ventricular size and systolic function with an LVEF of 55-65%. It also showed evidence of a septum within the right atrium diagnosed as CTD. The mitral valve had moderate to severe regurgitation and the tricuspid valve had severe regurgitation. There was an intra-atrial septal aneurysm with a small patent foramen ovale and a right-to-left shunt (, ).\nLeft heart catheterization was significant for a vertically rotated heart. He had several redundant large caliber vessels in the left circumflex and right coronary artery, in addition to a large caliber wrap-around left anterior descending coronary artery with no stenosis noted in these arteries, just luminal irregularities.\nThe patient was discharged to be followed up in the cardiology clinic for continued evaluation of his valvular disease and cor triatriatum. Together, they appeared to be the main contributors to his fatigue and shortness of breath. In contrast, his coronary arteries were large in caliber, with no disease that could be attributing to his symptoms. The patient would require monitoring for his mitral and tricuspid regurgitation with the possibility of surgical/percutaneous valve repair if deemed necessary in the future. He would also require monitoring for right ventricular failure secondary to CTD with the possibility of surgical resection/percutaneous disruption of the membrane if needed. |
A 94-year-old woman was transferred to our emergency room because of sudden right inguinal pain. There was swelling in her right groin and computed tomography revealed a right inguinal hernia which required surgical repair. She was previously diagnosed severe aortic stenosis but initially declined to undergo TAVI; however, surgery was not feasible given her severe aortic stenosis. Following re-discussion with the patient, she agreed to undergo intervention. She agreed the procedure this time. Her medical history included hypertension and permanent pacemaker implantation for sick sinus syndrome. On examination, her blood pressure was 131/70 mmHg with a heart rate of 62 b.p.m. On cardiac auscultation, a systolic ejection murmur was present and the intensity of second heart sound was reduced. The carotid pulse had a weak and delayed upstroke. There were no significant findings on respiratory auscultation, but she had jugular vein distention indicating high-central venous pressure. Her cardiac symptom was shortness of breath classified as New York Heart Association II. Transthoracic echocardiography showed a preserved ejection fraction with severe aortic stenosis; aortic valve area of 0.4 cm2, peak velocity of 4.4 m/s, and mean gradient of 47.0 mmHg. The Society of Thoracic Surgeons score was 4.0% and the Logistic Euro Score was 32.1%. After multidisciplinary team discussions, the patient was deemed inoperable for heart surgery because of old age, and considered as a candidate for TAVI. Computed tomography showed no significant coronary artery disease. It revealed a 377 mm2 annulus and compatible iliofemoral arteries; thus, transfemoral TAVI using a 23 mm Sapien 3 was performed. The procedure was under general anaesthesia because our institute was in early experience of TAVI and chose it as a routine method. After the pre-dilatation using a 20 mm balloon, the Sapien 3 was advanced. While crossing the stenotic native valve, we realized that the prosthetic valve and delivery balloon were out of alignment. The delivery system was pulled back to the ascending aorta and re-adjusted. The device crossed the aortic valve again and was deployed under rapid pacing. During the inflation, the delivery balloon slipped into the ventricle probably due to malalignment, and the valve top was not fully expanded (, S1 and S2). Although the paravalvular leak was mild ( and S3), post-dilatation using a 23 mm balloon was decided to prevent recoiling and a small valve area caused by insufficient expansion ( and S4). Just after the post-dilatation, the patient developed shock and arrested. Cardiopulmonary resuscitation was commenced and venoarterial extracorporeal membrane oxygenation (ECMO) was provided. Aortography and haemodynamics indicated severe aortic regurgitation (and ). Transoesophageal echocardiography depicted the absence of leaflets inside the Sapien 3 with severe aortic regurgitation throughout the entire stent (, S5 and S6); thus, we confirmed that all three leaflets were stuck. A pigtail catheter was advanced and manipulated in the prosthetic valve stent, but the leaflets were not recovered. The valve-in-valve procedure was performed using the second 23 mm valve ( and S7). The second valve functioned well with a mild paravalvular leak (, S8 and S9) and her haemodynamics dramatically improved. The ECMO was removed in the operating room and the patient was sent to the cardiac care unit under close observation. She was discharged 20 days later in a stable condition. Four months later, the patient was admitted again to the surgery department and underwent right inguinal hernia surgery. The patient was asymptomatic and stable in the 1 year follow-up period. Transthoracic echocardiography showed an effective orifice area of 1.32 cm2, peak velocity of 2.3 m/s, and mean gradient of 11.4 mmHg. |
The patient was a 61-year-old male without smoking history. In August 2014, without any symptom, a chest computed tomography (CT) showed a mass in the left upper lobe of the lung (maximum diameter approximately 6.0 cm). In 21 August 2014, the patient underwent left upper lobectomy and systemic hilar mediastinal lymph nodules section. Pathologic examination confirmed a moderately differentiated squamous cell carcinoma of central left lung (Fig. ). The margin was negative and no metastasis was found in the lymph nodes. Pathologic stage was pT2bN0M0, stage IIA. The patient then received 4 cycles of postoperative adjuvant chemotherapy with cisplatin 75 mg/m2 on day 1 plus gemcitabine 1250 mg/m2 on days 1 and 8, every 3 weeks.\nSix months later on September 18, 2015, chest computed tomography detected a newly occurring lesion with diameter of 1.5 cm in the left lower lung. CT-guided percutaneous lung biopsy was performed and metastasis of squamous lung cancer was confirmed. Other distant metastasis in liver, bone or brain was not found. The specimen was submitted for detection of EGFR mutation, ROS1 fusion gene rearrangement and ALK fusion gene rearrangement, yet neither mutation nor gene rearrangement was detected. The patient refused to receive systemic chemotherapy and RFA was intermittently performed for 3 times from October 25, 2015 to September 2, 2016 in his local hospital. The lesion at left lower lung decreased in diameter (0.9 cm) and was temporarily in control (Fig. ). On March 30, 2017, routine follow-up of chest computed tomography revealed an enlargement of the lesion at left lower lung with involvement to chest wall, and new nodules in both lungs (Fig. A). The patient received treatment of intravenous Atezolizumab (1200 mg) every 3 weeks from May 12, 2017. No severe adverse reaction was found during treatment. Radiological evaluation on August 3, 2017 showed disease progression in both lungs (Fig. B), however, Atezolizumab was continuously administrated due to his stable physical status. An encouraging partial response was observed at the left lower lung lesion (Fig. C), and stable response was observed at the right upper lung lesion on September 14, 2017, when then another radiological evaluation was performed. The patient went on treatment of Atezolizumab, and his improvements on clinical symptoms, health-related quality of life and radiological examination continued until now. A recent evaluation of chest computed tomography on May 24 2018 illustrated a durable clinical benefit (partial or stable response lasting > 6 months), that the lesion in the left lower lung continued to respond to Atezolizumab significantly, while the lesion in the right upper lung remained stable (Fig. D). |
A 30-year-old healthy male presented to us in September 2007 with complaints of gradual painless diminution of vision in both eyes (BE) since several years, which has worsened over the past 2 months in the right eye (RE). On examination, his visual acuity was counting finger (CF) at one meter in the RE and 20/200, N.18 in the left eye. Anterior segment examination and intraocular pressures were normal in BE. Fundus examination showed yellowish to orange, slightly elevated sub retinal lesion with well-defined geographic margins over the posterior pole in BE [Figure and ]. RE had a small sub-foveal grayish lesion suggestive of CNV with overlying sub retinal hemorrhage. Fundus fluorescein angiography (FFA) showed patchy hyperfluorescence and late staining in BE. RE also showed blocked fluorescence due to sub retinal hemorrhage and minimal leakage from CNV []. Ultrasonography showed minimally elevated lesion over the posterior pole with high reflectivity and orbital shadowing in BE confirming the diagnosis of osteoma []. Optical coherence tomography (OCT) showed diffuse thickening of retinal pigment epithelium (RPE) and choroid with intact inner retinal layers in BE and CNV subfoveally in the RE []. After written consent, he was treated by intravitreal injection of ranibizumab (0.5 mg in 0.05 ml) in the RE at monthly intervals for 3 months. Following treatment sub retinal hemorrhage disappeared, OCT showed resolution of CNV with the restoration of foveal contour and visual acuity improved to 20/200 []. He was under regular follow-up and remained stable. At his regular visit in January 2013, he complained of a recent decrease in vision in the RE, which was found to be reduced to CF at 3 m. Fundus examination of BE revealed enlargement of osteoma with zones of decalcification and no visible CNV [Figure and ]. FFA of the RE showed patchy hyperfluorescence without leakage [Figure and ] while OCT showed subretinal fluid (SRF) subfoveally suggestive of recurrent CNV []. Following an injection of intravitreal bevacizumab (1.25 mg in 0.05 ml), SRF resolved completely, restoring vision to 20/200 and has remained stable for the past 1 year []. |
A 32 year old female Cameroonian gravida 4 para 3 at 34 weeks of gestation presented to the labour and delivery unit of Mbalmayo district hospital with 8 h history of severe generalized headache, expressive aphasia and right sided paralysis in an afebrile context. This was associated with blurred vision but no convulsions. There was no epigastric pain and no difficulty breathing and no history of trauma or fall. For this current pregnancy, antenatal care (ANC) was started at 18 weeks with a booking blood pressure of 100/70 mmHg. She did four ANCs and all were uneventful. During her routine four ANCs here blood pressure was always less than 140/90 mmHg and her urine dipsticks done during the four ANCs were all negative for proteinuria. She refused neurological symptoms such as headache during pregnancy. She has a history of gestational hypertension in her third pregnancy. There was no family history of chronic hypertension, diabetes and chronic kidney diseases. On examination she was afebrile with a blood pressure of 182/126 mmHg and pulse of 112beats/minute. Neurological examination revealed Glasgow coma score of 13/15, right sided hemiparesis and expressive Broca’s aphasia, no signs of meningeal irritation. The abdomen was distended by a gravid uterus with a fundal height of 35 cm, foetus in a longitudinal lie and cephalic presentation. The cervix was long, posterior, soft and closed with a station of − 1. We had a working diagnosis of severe pre-eclampsia complicated by stroke. Shown on Table are laboratory investigations done and their results.\nAn emergency obstetric ultrasound showed a life foetus with an estimated foetal weight of 2300 g at 33 weeks of gestation. Emergency cerebral non contrast-CT scan showed a 3.2 cm hyperdense region in the left parietal lobe with surrounding hypodensity due to clot retraction as shown on Fig. . Emergency management by the obstetrician consisted of MgSO4 using the Pritchard protocol [], which consisted of 14 g loading dose then 5 g maintenance every 6 h until 24 h after caeserean section; bethamethasone 12 mg intramuscular and reduction of blood pressure with nicardipine 5 mg/h. Four hours later an emergency caesarean section was done by the obstetrician under spinal anaesthesia and it let to the extraction of a life female with APGAR 8 and 10 at the 1st and 5th minute respectively and weight 2200 g. The management after caesarean section consisted of hospitalization in the intensive care unit with nicardipine titrated in an electric syringe at 2.5 mg/hour, ceftriaxone 2 g intravenous, Paractamol 1 g 8 hourly, and ringers lactate 6 hourly for 24 h. Post-operative management was done by a multidisciplinary team including a neurologist, cardiologist, intensive care physician, obstetrician, neonatologist and physiotherapist. On postoperative day 2 she was transferred from the intensive care unit to the maternity where she spends five additional days on nicardipine slow release 50 mg 12 hourly and paracetamol 1 g 8hourly and was later release after the ten days on nicardipine 50 mg daily and daily physiotherapy. Six weeks during routine postpartum visit the blood pressure was normal and patient was no longer aphasic and shet has regained the muscle strength partially. The baby was hospitalised in the neonatal unit for 10 days and discharged alongside the mother. |
A 30-year-old female patient presented with complaints of sudden onset painful and rapidly progressive diminution of vision in the left eye of 2 days duration. She gave a history of being administered intravenous drugs multiple times for her episodes of headache over the past 6 months. There was no history of trauma or ocular surgery. There was no history of any systemic illnesses.\nOn examination, best corrected visual acuity (BCVA) was 6/6 in the right eye and finger counting close to face with accurate projection of rays in the left eye. Intraocular pressure was 13 mmHg in the right eye and 10 mmHg in the left eye. On slit lamp biomicroscopy, there was mild ciliary congestion, anterior chamber cells of 4+ with a hypopyon of approximately 1 mm in the left eye. On indirect ophthalmoscopy, there was evidence of 3–4+ vitritis. A solitary elevated yellowish lesion could be hazily seen in the superotemporal quadrant giving suspicion of an abscess. Combined vector ultrasound B-scan confirmed the presence of a subretinal abscess associated with vitreous exudates. There was no evidence of retinal detachment or choroidal detachment.\nOn systemic examination she had evidence of cellulitis of the left lower limb. Blood cultures (aerobic and anaerobic bacterial and fungal) were sent before initiating intravenous antibiotics. Results of all other relevant investigations were normal.\nBased on the clinical picture, a diagnosis of endogenous endophthalmitis with subretinal abscess was made. She was started on intravenous Vancomycin (40 mg/kg/day) and Ceftriaxone (100 mg/kg/day). Pars plana vitreous biopsy was performed along with injection of intravitreal antibiotics (Vancomycin 1 mg/0.1 ml and Ceftazidime 2.25 mg/0.1 ml). Gram stain and culture of the vitreous specimen did not reveal any organism. Blood culture and urine culture also failed to grow any organism. There was further deterioration of visual acuity and media clarity over the next 24 h along with ultrasound evidence of increase in the size of the abscess. A standard 25G pars plana vitrectomy was hence immediately undertaken. After core vitrectomy and sufficient clearing of media, a yellowish subretinal abscess of approximately 8 × 6 disc areas could be visualized in the superotemporal region (Fig. a). Since the abscess was large and had not responded to systemic and intravitreal injections, a decision to administer antibiotics into the lesion was taken. An elevated and relatively less vascular area was identified over the abscess. 41G needle (DORC international, Netherlands) was used to inject Vancomycin (0.05 mg/0.05 ml) directly into the subretinal abscess (Fig. b). After air-fluid exchange, silicone oil (1000 cs) was injected to provide internal tamponade.\nOn the first postoperative day, her best corrected visual acuity (BCVA) was 4/60. There was a track of exudative fluid along the temporal aspect and minimal subretinal bleed adjacent to the abscess (Fig. c). On the third postoperative day, there was marginal reduction in the size of the abscess and appreciable decrease in exudative fluid. On day 10, after surgery the subretinal abscess had shrunk significantly and there was absence of any subretinal fluid (Fig. d). There was no further increase in subretinal hemorrhage. Visual acuity had improved to 6/24 at this visit and intraocular pressure was normal (13 mmHg). Patient was lost to follow up after this and despite repeated telephonic requests did not report for a follow up due to economic constraints. She is apparently under the care of a local ophthalmologist and continues to maintain 6/24 vision in the involved eye. |
A 41-year-old woman with a 14-day history of subacute intermittent right lower quadrant abdominal pain was admitted to our hospital. No accessory symptoms were observed. She experienced similar symptoms approximately 5 years prior to this event. At that time, her symptoms resolved after fasting and the administration of intravenous fluids and antibiotics. Cecal volvulus was not noted.\nShe did not have a history of any surgical procedures and any gynecologic diseases. She had a regular menstrual cycle and had not taken oral contraceptives or progestins. Her physical examination revealed a distended abdomen with localized tenderness in the right lower region without peritoneal signs. Laboratory tests reported a white blood cell count of 4820 cells/μL and C-reactive protein level of 0.06 mg/dL. Plain radiography showed dilated gas-filled loops of the colon. The colon was displaced in the pelvis but there was no evidence of air-fluid levels (Fig. ). Abdominal computed tomography showed both dilated intestinal gas like coffee beans in the pelvis and signs of twisting of the mesentery and mesenteric vessels (whirl sign), although these findings did not indicate which section of the large intestine was twisted (Fig. , ).\nWe suspected sigmoid colon volvulus and performed an emergency colonoscopy. The large intestine was not ischemic and was filled with stool (Fig. ). We successfully repositioned the colon, and the patient’s symptoms resolved soon after the colonoscopic reduction. We scheduled an elective laparoscopic sigmoid resection 14 days after the colonoscopy. Intraoperative exploration showed a significantly distended cecum and ascending colon, which was twisted around a short rope-like adhesion that connected the cecum and transverse colon mesentery. The intestinal wall showed no evidence of ischemia, necrosis, or perforation. Therefore, a laparoscopic ileocecal resection rather than a sigmoid resection was performed (Fig. ). The postoperative course was uncomplicated. Histopathological examination revealed the presence of endometriosis in the short rope-like adhesion (Fig. ). This finding confirmed a diagnosis of cecal volvulus caused by endometriosis. No other focus of endometriosis could be identified. The patient was discharged in good general condition 10 days after the laparoscopy. After the operation, she was diagnosed with endometriosis for the first time. The endometriosis was not found in the other organs on magnetic resonance imaging, and the patient has since been monitored without the need for any further treatment. |
A 12-year-old girl was admitted to the hospital as a case of unusual painless bleeding from the left side of the face (), left eye, and tear duct of two-week duration. The bleeding was described to be spontaneous, unpredictable, and intermittent, with no specific patterns, and it stopped spontaneously. There was no association with mood, activity, or sleep. The bleeding occurred mostly around the orbital regions, the tear ducts, and the face, with intact skin, and no blood or redness, or visual disturbance of the eyes, except for mild perorbital tenderness. Several episodes of bleeding occurred prior to admission, and on admission two episodes occurred while the physician observed. Each episode started like tear drop confluent spots of mild watery secretion over the left side of the face and streak-like drops starting from the corner of eye where tear duct is situated and along the cheeks. This was followed by bright-red colored secretion, lasting for 10–20 minutes, and the patient did not show any signs of distress, siting comfortably in the bed during the episodes. The patient describes occasional gum bleeds when brushing teeth, although she denied hematuria, blood in stools, or blood stained clothes or underwear, and there was no evidence of petechiae, bruising, or ecchymosis. During this admission, the patient had one episode of epistaxis, which was treated with cautery. However, previously, the patient had presented twice in June 2015 with two episodes of epistaxis and on both occasions was treated with cautery.\nCT scan was performed on the patient to be assessed for any sinus disease and showed mucosal thickening in bilateral maxillary sinuses and sinus orifice obstruction consistent with chronic sinusitis. Additionally CT scan showed bony nasal septal deviation to the right.\nPrevious admission was on 2013 (2 years ago) for repeated faint attacks, palpitations, and mild anemia, with normal ECG findings.\nOn system review, patient denied fever, runny itchy nose, nasal discharge, red or painful eye, shortness of breath, cough, chest pain, palpitations, rash, joint pain, mouth ulcers, bleeding PR/urine, menorrhagia, abdominal pain or change in bowel habits, anorexia/recent weight lose, sensory loss or weakness, and lumps or bumps or lymphadenopathy. Her physical examination was unremarkable, except for right nasal septum deviation, enlarged adenoids, hypertrophied bilateral inferior turbinates, and mild periorbital tenderness. Complete blood count (CBC), renal function test (RFT), liver function test (LFT), and coagulation profile were all normal.\nThe secretion at the corner of the eye was collected during an episode of bleeding and we did blood smear examination on the sample. The results showed plenty of red blood cells, and no abnormal cells.\nHematology, dermatology, ophthalmology, ENT, and immunology were consulted to further evaluate and assess the patient, and cause of bleeding. All consults returned inconclusive of any identifiable source or cause of bleeding.\nThe only significant finding on past medical history included menarche at age of 10 years that was regular for four months, then spontaneously stopped for around two years, and returned spontaneously for unknown cause, with no medical attention. Last few menstruations however were regular and of normal flow and no clots or dysmenorrhea.\nPatient is not on any medications and not on over-the-counter drugs. However patient describes allergy to eggs or egg-containing products and sugar candy, where a steak line rash appears on arms.\nThere are no family histories of such events of spontaneous bleeding amongst close relatives. |
A 38-year-old woman was referred to our outpatient ear, nose and throat (ENT) clinic with the right submandibular swelling for about a one-year duration. Besides a mild intermittent ache during meals, she had no other relevant symptoms. The patient did not have any trauma or chronic medical illnesses. Clinical examination revealed a mass of about 2.0 × 2.0 cm at the right submandibular region which was firm in consistency. No tenderness was elicited or warmth felt on palpation. Fine needle aspiration cytology (FNAC) of the mass was ordered and the result was suggestive of sialadenitis. Sialography was then scheduled for further investigation of the lesion.\nDuring sialography, serial dilatation of right submandibular duct was performed, followed by insertion of size 16 F cannula. Furthermore, 3 mL of Ultravist (contrast agent) was injected and serial images were taken. No resistance was felt during infusion of the contrast material. The patient tolerated the procedure well and did not complain about having any pain. Opacification of the distal part of the submandibular duct was noted, followed by fusiform dilatation of the duct and extravasation of contrast (). The patient was then immediately referred to an otolaryngologist.\nOn bimanual examination, a stone could be felt in the floor of the mouth. A nodular mass of about 2.0 cm was palpated at the right submandibular region, which was non-tender. The CT scan of the submandibular gland was suggested by the otolaryngologist. The CT scan showed a radiopaque submandibular stone measuring 11.0 mm × 6.0 mm and right submandibular duct perforation as evidenced by contrast extravasation into soft tissue of the submandibular region (). The patient was treated conservatively with amoxicillin/clavulanic acid (co-amoxiclav) for a week and prednisolone 60 mg for three days. The patient was well on the follow-up session a week later and no tenderness was found on palpation. Repeat lateral neck X-ray showed that the contrast material had disappeared. She was seen again at the ENT clinic, three weeks later and submandibular gland excision was scheduled. |
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