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A 67-year-old male presented to the eye casualty department in December 2008 with a 1-week history of distorted vision in his left eye. His past ocular history was significant in that he had a disciform scar in his right eye leaving him with hand movement vision. He has also been under the care of the medical retinal consultant since January 2008 with a left occult choroidal neovascular membrane for which he had received 5 intravitreal injections of bevacizumab.\nHis past medical history included peripheral vascular disease, essential hypertension, impaired glucose tolerance, early dementia and depression.\nOn examination at the casualty department, his visual acuity was 6/38 + 2 in the left eye with microaneurysms at the fovea with minimal oedema. A fundus fluorescein angiogram (FFA) and optical coherence tomography (OCT) were requested, which revealed the following (fig. , fig. ).\nThe patient was seen in at clinic as a matter of urgency. A decision was made with the patient to start intravitreal ranibizumab due to the presence of only one functioning eye. Bevacizumab was stopped, and treatment was initiated a week later. The patient showed significant improvement after the first 3 injections, and his vision remained stable. Figure shows his OCT image.\nThe patient received a total of 10 intravitreal ranibizumab injections within the first year, with stable vision at 6/24 (40 letters). However, in May 2010, during routine follow-up, the patient presented to the Age-Related Macular Degeneration (ARMD) clinic complaining of reduced vision. Visual acuity was 6/120 + 4 (8 letters), and his OCT scans are shown in fig. . A repeat FFA was organised immediately (fig. ).\nThe decision was made to treat the patient with IVTA, and the patient was given a single IVTA injection. The patient was given the IVTA injection in the first week of June 2010. During the subsequent visits, an immediate improvement in vision was noted as well as improvements in his OCT imaging. His visual acuity improved to 6/48 (25 letters) at the first visit and remained stable for the following 2 months. His OCT scan 1 month after IVTA is shown in fig. .\nHe required no further intravitreal triamcinolone injections, and ranibizumab injections were continued based on OCT appearances on a monthly basis. Since the intravitreal triamcinolone injection, he has received a total of 7 intravitreal ranibizumab injections to the left eye over a 4-year period based on his OCT appearances, with the last injection in October 2013. His vision fluctuates on a monthly basis but it has remained between 6/48 + 3 (28 letters) and 6/30 – 1 (34 letters). OCT images at the 1-, 2-, 3- and 4-year follow-up are shown in fig. .\nCurrently, the patient is still under follow-up at the ARMD clinics. His right eye shows no signs of RAP and is being monitored with OCT imaging. As the patient had an almost immediate improvement with IVTA injection based on vision and intra-retinal fluid, we are able to confirm the inflammatory nature of RAP and the effects of IVTA. |
A 10-year-old female with a history of iris heterochromia and anisometropic amblyopia (diagnosed at age 6) presented to her paediatrician after she had an absent red reflex noted in a photograph. The patient had no notable family medical history and an otherwise unremarkable personal medical history, including no history of cyanotic disease. The patient was referred to our tertiary eye centre for evaluation (Moorfields Eye Hospital, London, UK). Fundoscopic examination revealed healthy optic discs. Tortuous and dilated retinal vessels in the right fundus, and to a far lesser extent on the left were noted, without exudation or hemorrhage present (A). The diagnosis of arteriovenous malformation was made, with no evidence of progression or visual consequence reported over seven years of annual evaluation (B). Visual acuity of the patient was 6/24 and 6/6 for the right and left eye respectively, having high hyperopia in the right eye and no refractive error in the left. Right eye vision had not improved significantly after attempted patching in early childhood. Visual acuity remained stable over time. The eyes were normally aligned, and extra-ocular movements were full. Visual fields to confrontation were normal. Intraocular pressure in both the right eye and left eye was 12 mmHg. Slit-lamp examinations of the anterior segments of both eyes were unremarkable.\nAt 14 years of age at routine follow-up, she complained of an intermittent right-sided headache and a pressure sensation around the left eye. At a follow up three years later, this gradually escalated to throbbing pain and discomfort behind both eyes. This pain was not related to ocular movement and did not wake the patient up at night. Retinal imaging was stable. Magnetic resonance imaging and angiography (MRI and MRA) was requested to exclude intracranial involvement (both at age 13 and 17 years). Neuroimaging was normal, ruling out central nervous system vascular abnormalities and ocular ultrasound had no observable malformations. Ophthalmic blood vessels on both eyes had no obvious vascular abnormalities, in contrast to the retinal vessels.\nColor fundus photos and OCT imaging were in keeping with an AVM in the right eye; with retinal vessels in the right eye being both highly tortuous and dilated (A). Both eyes were imaged with swept-source OCTA (SS-OCTA, Plex Elite, Carl Zeiss Meditec AG). This demonstrated that the macrovessels were not passing through either the macula (C) or the foveal avascular zone (D). OCTA B-scan images of the right and left eye were analyzed in ImageJ to quantify retinal blood perfusion and then compared between eyes, in order to investigate the impact of this unilateral AVM (E). The eye with the AVM had significantly higher retinal blood perfusion than the unaffected eye. The percentage difference of blood flow between eyes reached a maximum of 1102% in the superior arcade (). This trend continued with the percentage difference between the left and right inferior arcade being 836.2% (), and 190.5% for the fovea (). |
A 55-years-old woman presented with the sensation of a foreign body in her throat and hoarseness that had worsened for the 3 months before her presentation. Her occupation was a house keeper. Laryngoscopy revealed a submucosal bulging mass at the left pyriform sinus and this mass extended to the ipsilateral false vocal fold; the left vocal fold was pushed medially and the shape of the mass did not alter with respiration (). A soft-tissue density mass was observed at the level of the glottis on a lateral C-spine X-ray. Computed tomography (CT) showed a 2-cm mass that contained ring-like calcification, and the mass was located in the left supraglottic area (). A well-encapsulated, solid mass was detected when performing laryngomicroscopic surgery (LMS). The frozen biopsy was reported as a schwannoma. Owing to the extensive range and fixation of the mass, we had to conduct a second operation via an external approach to achieve complete removal. After the patient was first discharged, magnetic resonance imaging (MRI) confirmed the presence of a well-defined ovoid mass with isointensity on the T1-weighted images and high signal intensity on the T2-weighted images (). We excised the mass via a lateral thyrotomy without a preliminary tracheostomy in the second operation. A window was created using an electrical saw. After dissecting the soft tissues, the yellow mass was easily separated from the adjacent tissues with finger dissection. The internal branch of the superior laryngeal nerve was found to be the nerve of origin for the tumor. No postoperative complications occurred, and she was discharged on postoperative forth day. Postoperative laryngoscopy, which was performed 4 months after the second discharge, revealed that the bulging findings of the left vocal fold had disappeared and both vocal folds were mobile. The lady's voice recovered completely.\nThe gross tumor specimen measured 2.0×2.5 cm. On microscopic examination, the tumor was characterized by cellular Antoni A areas alternating with myxoid, loose degenerative Antoni B type areas. The immunohistochemical study was positive for S-100 protein in the cellular areas. The overall features of the tumor were consistent with a schwannoma. |
The patient in our study was a 35-year-old female who sustained a low voltage alternating current (110–380 V) electrical injury to her left hand. Left forearm pain, paraesthesia and bluish skin discoloration were features from initial presentation. In the 3 weeks following injury, she developed progressive patchy paraesthesia and dysaesthesia predominantly in the median nerve distribution of the affected hand. An ultrasound showed a normal appearance of the median nerve and of structures within the carpal tunnel and distal forearm. Acute carpal tunnel syndrome was suspected as being responsible for at least part of the presenting symptoms. With the possibility of secondary median nerve compression related to the injury, a carpal tunnel release procedure was undertaken at 3 weeks post injury. The median nerve appearance was macroscopically normal. Postoperatively, some paraesthesia in the territory of the median nerve improved but forearm pain persisted.\nFrom 1 month post injury, the patient's disease continued to progress; all movements and sensory stimulants to the affected hand were grossly intolerable and a diagnosis of CRPS was made. The diagnosis was made based on the patient fulfilling the Budapest criteria, as shown in Table .\nFrom 1 to 10 months post-injury, the patient received specialist therapy and pain management including pharmacological agents such as gabapentin, paracetamol, ibuprofen, tramadol, amitriptyline, ketamine, pamidronate infusions, lidocaine plasters, nabiximols, and capsaicin cream, which all failed to give relief. Supraclavicular catheter blocks, transcutaneous electrical nerve stimulation (TENS), and hand physiotherapy were also ineffective. At 9 months post injury, the patient was experiencing spontaneous skin breakdown with weeping, cellulitis and was hospitalized as a result of these infections. She was unable to clean her skin due to excessive pain. By the end of this period the patient had developed a functional loss of the limb. The patient requested an amputation which was ultimately supported by the multidisciplinary team consisting of plastic surgeons and pain anesthetists. The potential for control of pain was a secondary aim, as the impact of amputation on pain could not be fully predicted.\nTen months post-injury, the patient underwent a below elbow amputation at the level of the proximal third of the forearm, with a dorsally based flap of intact skin used to resurface the residuum. Nine months post-amputation the patient has not had any recurrence of CRPS at the amputation site, which is fully healed. She has had a dramatic response in terms of relief of CRPS symptoms. She has regained her quality of life and is wearing a cosmetic artificial arm prosthesis, with plans for her to progress to use of a myoelectric prosthesis. |
A 17-year-old adolescent nulligravid girl whose last menstrual period was four days back referred with the diagnosis of low lying myoma after being transfused with four units of blood. She presented to the Gynecology Outpatient Department on 22 July 2018, with history of irregular vaginal bleeding of 2-year duration.\nShe gives history of lower abdominal swelling which was small initially and progressively enlarged to attain the current size for the last 13 months. She is sexually active but not married. She has no history of abdominal pain, urinary, or bowel complaint, has no bleeding from other sites, and has no significant past medical and surgical history noted.\nOn examination, patient was conscious, coherent with blood pressure of 100/60 mmHg, pulse 80 /min, and temperature normal and has pale conjunctiva, cardiovascular, and respiratory systems normal. Abdominal examination revealed 20-week sized nontender, firm, irregular, fixed, and lower border unreachable mass. On vaginal examination, a firm huge nontender growth attached to the anterior vaginal wall. It was difficult to reach at the cervix. A clinical diagnosis of cervical myoma was made.\nHer blood investigations on arrival showed hemoglobin of 9.7 gm/dL and were transfused one unite blood and the hemoglobin elevated to 10.7 gm/dL. Ultrasonography showed empty uterus with normal size echotexture and pushed up in to the abdomen. There is 10 by 12 cm hypoechoic mass arising from the cervix filling the vaginal canal with these the diagnosis of cervical myoma was made. The diagnosis necessitated Computed Tomography (CT) scan of the abdomen and pelvis which helped to know the extent of the mass and reported hypodense contrast enhancing mass seen on the uterus arising from the cervical region measures about 10.3 cm by 14.4 cm. The mass grows down in to the vagina and concluded with the diagnosis of contrast enhancing cervical mass likely myoma ().\nUnderstanding the complexity of her surgery and possible postoperative morbidity and mortality, surgical, anesthesiology, nursing, and recovery room teams was assembled with gynecologic oncology team. She was prepared for elective laparotomy the day before the surgery. On 18 August 2018, the patient was taken to operating room, after the general anesthesia given; she was positioned in a supine position. The abdomen cleaned with povidone iodine and draped with sterile towels and midline vertical incision was made. Intraoperatively, bilateral fallopian tubes and ovaries, the uterus, and urinary bladder grossly look normal; there is 10 cm by 15 cm sized firm mass between the vagina and the lower uterine segment (). Vesicouterine peritoneum incised the bladder reflected away from the lower uterine segment and upper vaginal wall, about 4 cm vertical incision was made on proximal anterior vaginal wall, and dissection of the mass away from the anterior vaginal wall was tried but it was difficult to have a clear cleavage line to excise the whole mass. Then another 6 cm longitudinal incision was made on the posterior vaginal wall; sharp and blunt dissection were made to separate the bulk of mass away from the vaginal wall; finally excision of the mass from the base within 1 cm of normal vaginal tissue was performed to carefully remove a 15 × 10 × 7 cm encapsulated mass (). The anterior vaginal wall, extension of incision on the cervix, and posterior vaginal wall closed separately with vicryl number 0 in two layers (). Hemostasis was secured, correct count was reported, the fascia and skin were closed using delayed absorbable stitch. The surgery was completed after 2hrs and 20 minutes.\nThe excised mass was sent for histopathological examination; the patient recovered completely and discharged on 6th postoperative day. The final pathological diagnosis of the mass was vaginal angiofibroma (). |
A 59-year-old male with a history of multilevel lumbar laminectomy was referred to us for trial and permanent placement of epidural spinal cord stimulator for failed back surgery syndrome with persistent low back pain and lumbar radiculopathy. He was cleared by formal neuropsychiatric testing and subsequently underwent routine trial with two Vectris Surescan (Medtronic Sofamor Danek USA, Inc. TN, USA) percutaneous eight contact epidural electrodes under conscious sedation. Saline push technique with Omnipaque epidurogram (GE Healthcare, CT, USA) confirmed epidural placement. Intraoperative fluoroscopy showed both electrodes at the desired levels rostrally at the bottom of the vertebral body of T8, spanning the entirety of T9 and caudally ending at the top of T10. One electrode was slightly off to the right of midline. Sedation was lightened and the system was activated for intraoperative testing. The patient did not complain of any dysesthesias or undesired stimulation and confirmed excellent coverage of his pain. No abnormal parameters were noted and all contacts were working normally during intraoperative interrogation. Final anteroposterior (AP) X-ray at the end of the procedure confirmed proper placement, and the electrodes were secured to the skin in the usual fashion with silastic anchors and silk sutures (Figure ).\nImmediately postoperatively in the recovery unit, he complained of severe and distressing sharp testicular and scrotal pain in the abscence of neurological deficits. He was placed on high dose corticosteroids, which improved his pain. He was evaluated by urology service and was found to have no evidence of intrinsic testicular or scrotal pathology. His bladder function remained normal without urinary retention. His stimulator was turned on the next day, and his overall pain and discomfort markedly improved, and therefore, no further workup or imaging was obtained. He was discharged home for the completion of his trial and eventually returned for permanent placement with a laminectomy style paddle electrode with an open mini-laminectomy approach. His permanent placement postoperative course was unremarkable and he ultimately reported therapeutic stimulation. |
The patient is a 73-year-old male who was transferred from an outside hospital for repair of a right acetabulum fracture involving the femoral head after falling approximately 8-feet from a ladder while painting his house. Past medical history was significant for hyperlipidemia and osteoarthritis. Baseline metabolic equivalents were greater than four. Aside from a cataract extraction, the patient had no other operations. He denied any allergies. Prior to presentation, the patient was on Aspirin 81 mg daily for cardiovascular disease prevention and Atorvastatin 20 mg daily for hyperlipidemia. Computed tomography (CT) without contrast showed an acute, comminuted, and displaced fracture of the right acetabulum involving both posterior and anterior acetabular walls. The right femoral head was superiorly and laterally displaced with impaction fracture to its inferior and medial aspects. There were bone fragments within the right gluteus musculature, and the high attenuation in tissue density represented blood product within. Prior to surgery, the orthopedic team made an effort to reduce the patient's right acetabulum fracture with tibial traction pin under conscious sedation with midazolam, fentanyl, and ketamine in the emergency department. During the closed reduction, the patient experienced a brief period of respiratory depression. For approximately two minutes, his oxygen saturation was 85-86%, which improved to 95% with Narcan reversal. The emergency department record also noted that the patient had premature ventricular contractions on the electrocardiogram (EKG) at this time. After the closed reduction, the orthopedic team opted to monitor the patient on continuous telemetry for 24 hours and take the patient for open reduction and internal fixation of the right acetabulum the next day. He was not on prophylactic anticoagulation for this preoperative period.\nPrior to entering the operating room, the patient's vital signs were stable: blood pressure 120/64, heart rate 73 beats per minute, respiratory rate 18 per minute, oxygen saturation 95%, and temperature 98.6 Fahrenheit. Neurological exam was significant for limited right knee flexion (<30 degrees). Otherwise, overall sensation was intact and right deep tendon reflex was intact. Hemoglobin was 11.6, down from 13.4 on admission. Coagulation tests showed elevated prothrombin time at 12.8, partial thromboplastin time 26.7, and international normalized ratio 1.20. In the OR, the patient was induced with standard dosing of midazolam, fentanyl, lidocaine, propofol, and succinylcholine. Phenylephrine was given preemptively on induction to avoid hypotension. Intubation was uncomplicated. Patient had two 20g peripheral intravenous catheter in place, and a left radial arterial line was placed after intubation. Despite premedication with phenylephrine, the patient became hypotensive with systolic blood pressure (SBP) in the 80s, median arterial pressure < 65. Boluses of ephedrine and phenylephrine were given, and a phenylephrine infusion was started thirty minutes after induction. The patient initially responded appropriately to treatment (SBP > 100). Due to concern for sciatic nerve injury, intraoperative somatosensory evoked potential (SSEP) monitoring was performed, and general anesthesia was maintained with propofol at 100 mcg/kg/min and remifentanil at 0.2 mcg/kg/min, in addition to 0.5% end tidal sevoflurane.\nUpon patient positioning to the right lateral decubitus position from supine, the patient's SBP acutely declined into the 50s and the patient became hypoxic with an oxygen saturation of 88%. His hypotension was no longer responsive to phenylephrine or ephedrine. Norepinephrine and epinephrine drips were started. Also, the FiO2 was increased from 50% to 100%. There was a minimal and transient response to epinephrine administration. The patient was then returned to supine position, and he arrested shortly thereafter. Chest compressions commenced immediately upon loss of cardiac rhythm. The cardiac anesthesia team emergently responded and performed an intraoperative transesophageal echocardiography (TEE), which showed reduced left ventricular ejection fraction and reduced right ventricular systolic function. Due to unsuccessful cardiopulmonary resuscitation, the patient was placed on venoarterial ECMO via right femoral artery and vein; stable hemodynamics were achieved. A total of 8000 units of Heparin was given upon ECMO installation. At this point, the planned surgery was aborted. The patient was transported to the cardiac catheterization lab for pulmonary angiogram, cardiac catheterization, and antegrade perfusion of the right superficial femoral artery. A large thrombus in the left pulmonary artery was discovered on selective angiography (Figures –). Hypothermic protocol was initiated. Heparin therapy was also continued at this point.\nOn hospital day 5, a CT chest with contrast showed multiple bilateral pulmonary emboli and evidence of right ventricular failure. Right heart catheterization and placement of a Swan-Ganz catheter demonstrated that the patient was found to have severe pulmonary hypertension 55/30 and elevated central venous pressure (CVP) of 18. The patient underwent bilateral pulmonary artery embolectomy, exploration of right atrium with removal of clots, discontinuation of ECMO support, and repair of right femoral artery on hospital day 9. His pulmonary artery pressure and CVP improved to 31/12 and 8, respectively. One month later, the patient was discharged to a rehabilitation facility with a plan for conservative management of his fracture. |
A 2-year-old girl was referred for the presence of a subcutaneous palpable mass on the neck. She appeared otherwise healthy with no evidence of lymphadenopathy or organomegaly. Her parents were consanguineously married. She had no significant family history of atypical nevus or melanoma. She has a giant congenital nevus on head but nobody has not noticed that the malignant melanoma nodules were developed on it.\nOn physical examination, on the left sternocleidomastoid region, indurated subcutaneous palpable masses were adjacent to each other and adhered to the skin with erythema formation. These indurations were palpated as solid masses with patchy areas of softening. Initially two biopsy specimens measuring 5 and 6 mm in diameter were excised from this subcutaneous palpable mass. Histopathological examination revealed a highly malignant tumor diffusely infiltrating the soft tissue (). Tumor cells composed of large epithelioid cells arranged in a solid and infiltrative pattern which comprised of atypical tumor cells with abundant mitotic cells (). Tumor cells had enlarged hyperchromatic nuclei with prominent nucleoli devoid of melanin pigment. In immunohistochemical studies, tumor cells revealed positive expressions for HMB-45 (), S-100, and Melan A (MART1) but negative for myeloperoxidase, LCA, pancytokeratin, CD 68, desmin, and CD56. With these findings metastatic malignant melanoma was diagnosed.\nThe requested dermatological examination revealed a large congenital melanocytic nevus completely covering the left half of the scalp, and extending to the left side of the neck and showing heterogeneous dark hyperpigmentation with several nodules measuring 0.5–1 cm in diameter. With these clinical findings, development of malignant melanoma on congenital melanocytic nevus and also soft tissue metastasis was suspected and biopsy of nevus was taken. Microscopically, the similar histopathological morphology was seen. The histology revealed ulcerated amelanotic nodular melanoma composed of medium-large sized malignant cells exhibiting atypical vesicular nuclei and prominent nucleoli but no melanin pigment. The tumor infiltrated into the entire dermis up to the hypodermis and the surgical margin was positive. Numerous and atypical mitotic cells were detected. Immunohistochemically, tumor cells showed reactivity for Melan A (MART1), S100, and HMB-45. Total body contrast-enhanced computed tomography (CT) examination did not reveal any further secondary localizations and metastases. Skin-grafting or total excision was not made because of lesion's size and localization. The final diagnosis was ulcerated metastatic amelanotic nodular malignant melanoma with a congenital melanocytic nevus and the patient was transferred to the department of internal medicine for chemotherapy. However, her family refused additional treatment and she died 9 months later after the diagnosis. |
We report a case of 38-year-old Greek woman who presented to our Emergency Department complaining of abdominal pain during the last week without any other symptoms. Her clinical history was clear and she had not noticed the occurrence of the same symptoms before. A clinical examination revealed only focal tenderness in the left part of her abdomen. Laboratory results were within normal limits. During an ultrasound examination of her abdomen, an anechoic lesion in her upper left abdomen was revealed. In a further investigation with computed tomography (CT), a well-defined hypodense cystic 7.08 × 6.05 cm mass with mild enhancement was noted (Fig. ). Surgical approach was decided after a thorough examination and our patient gave her consent for surgery. A cystic lesion sized 7.08 × 6.05 cm appeared between the layers of small bowel mesentery (Fig. ). The cystic lesion was excised within healthy borders and sent for further pathologic examination (Fig. ). On macroscopic examination, the cyst sized 7.08 × 6.05 cm was unilocular and contained a white, milk-like viscous fluid (chylous), which was drained out by incision. Histopathological investigation showed a thick fibrous wall, pervaded by chronic inflammatory cells (lymphocytes and plasma cells) and lymphoid aggregates. Variously sized vessels could also be observed while immunohistochemically CD31 (platelet endothelial cell adhesion molecule) was positive (Fig. ). CD31 is used primarily to demonstrate the presence of endothelial cells and can help to evaluate the degree of tumor angiogenesis. A definitive inner epithelial lining was not found. On the inner surface, multiple aggregates of foamy macrophages as well as focal foreign-body giant cells were present. (Fig. ) The thickness of the wall varied trivially with small parts of mature fat tissue toward the outer surface, indicating the mesentery. The findings were found to be most consistent with a simple lymphatic (chylous) cyst of the mesentery in combination with features of a non-pancreatic pseudocyst. Her postoperative course was uneventful and patient feeding was started on the second postoperative day. She was discharged on the fifth postoperative day while she had a low-grade fever. A 6-month follow-up with abdomen ultrasound and 1-year CT imaging showed no signs of recurrence. |
A 16-year-old right-hand dominant female presented to our orthopedic department with tolerable pain in upper back associated with “clunking” during active movements of right shoulder. The parents also had noticed an abnormal prominence in the right scapular region. She developed gradual symptoms over a 6 month period before her presentation with no antecedent history of trauma and constitutional symptoms. Other siblings and parents were normal.\nPhysical examination revealed a nontender palpable mass at superior border near medial angle of scapula. A grating sensation, both palpable and audible, was produced during active shoulder range of motion. Vertebral border of right scapula was more prominent than left (). The winging of scapula was noticed even at rest which did not increase as the patient did push against the wall. There were no signs of infection or inflammation. A bony mass was felt over the superomedial angle of the right scapula, which was minimally tender and corresponded to the area from where the crepitus arose. The upper extremity and shoulder girdle muscles were neurologically intact. Laboratory investigations were within normal limits.\nRadiographs showed a large bony tumor arising from superior border close to angulus superior scapulae and directed toward the thorax (). Computed tomography scan images revealed a pedunculated bony mass measuring 5.3×3.0×1.5 cm attached to ventral surface of superior border of scapula by a small stalk (-). Magnetic resonance imaging revealed this mass was extending anteriorly against the ribs and had a characteristic mushroom-shaped appearance with no obvious soft tissue component. A diagnosis of ventral scapular exostosis is made and patient was offered for surgical treatment in view of her complaints and cosmetic concern. Wide, open excision is the treatment of choice; however, there are recent reports of arthroscopic excision [, , , , ].\nThe patient was treated with excision of osteochondroma in prone position under general anesthesia. The shoulder was held in internal rotation thus facilitating lifting of medial border of scapula. A longitudinal incision along the medial border of scapula was used. Trapezius muscle was incised in line with its fibers (). Levator scapulae and rhomboid minor muscles were detached. No bursa was demonstrated and we encountered no technical difficulties during surgery. Extraperiosteal resection of the swelling was done and sent for biopsy (). Histopathological examination confirmed the mass to be an osteochondroma (). a. No malignant transformation was noticed in the cartilaginous cap. Post-operative clinical examination showed painless full shoulder mobility as well as the absence of scapular winging and crepitus. Radiograph confirmed complete removal of the tumor. The arm was immobilized in a sling for 4 weeks during which period only pendulum exercises were permitted. After 4 weeks, she was sent for a rehabilitation program to improve her shoulder range of movements. Her symptoms resolved dramatically in the immediate post-operative period and she felt no abnormal movements of the shoulder at 6 weeks follow-up. There is no recurrence of symptoms till last follow-up at 6 months. |
A 12-year-old Japanese girl, gravida 0 para 0, who was the monozygotic twin sister of the patient in Case 1, visited our gynecologic department for ovarian screening 3 months after the first operation of Case 1. Transrectal echography revealed bilateral ovarian cystic tumors with calcified structures. Tumor marker levels (CA125, SCC, CEA, and CA19-9) were normal. The bilateral ovarian cystic walls were extracted, and normal ovarian tissues were preserved by laparotomy. Pathological analysis of the cyst wall disclosed that there were benign teratomas in both ovaries.\nSixteen months after the first surgery, bilateral ovarian tumors were detected with echogram and pelvic MRI (right side: 4.4 cm, left side: 8.0 cm in diameter) during her regular check-up (). The ovarian tumors were accompanied by a solid part and high echoic part inside. Blood examination of tumor markers revealed a mild, elevated CA125 (37.5 U/mL). Bilateral cystectomy was performed 20 months after the first surgery. Examination of the other pelvic and abdominal organs found no disseminated and metastatic lesions in the surgery. There were no swollen pelvic lymph nodes. The histological diagnosis was an immature teratoma of grade 1 with International Federation of Gynecology and Obstetrics stage I in the right ovary and a benign mature teratoma in the left ovary. The girl's postoperative condition was good, and she was discharged without any complications. An additional surgery and chemotherapy were not planned according to a deliberated discussion with her family.\nAfter being discharged, she continued to be followed every other month. Four months after the last surgery, a right ovarian cystic mass (4 cm in size) was detected, and it enlarged to 6 cm two months later (). The blood CA125 level (32.3 U/mL) was in the normal range. Therefore, a third laparotomy was performed 7 months after the second surgery. The right adnexa, appendix, and greater omentum were resected. Examination of the other pelvic and abdominal organs showed that they did not have disseminated and metastatic lesions. There were no swollen pelvic lymph nodes. The histological diagnosis was an immature teratoma of grade 1 in the right ovary, and metastatic carcinoma was not detected in the other extracted organs (Figures and ). After 30 months, the patient continued to do well and there was neither tumor recurrence nor pelvic lymphadenopathy. Levels of tumor markers, including CA125, were within normal levels.\nApproval of the institutional study board and informed consent of these patients were obtained. |
A 57-year-old female presented with a history of pain and swelling below the left knee, insidious in onset for 4 years with a sudden increase in size over the past 2 months. She also had swelling on the dorsum of the right hand. No significant limitations of movement were noted. No history of injury was given by the patient. An ultrasound of the knee joint showed multiple loculated cystic lesions involving the subcutaneous planes and the popliteal fossa with diffuse internal echoes (). It was suggested that she undergo an MRI of the knee for further evaluation, which showed multiloculated cystic lesions that were hypointense on T\n1 weighted and hyperintense on T\n2 weighted sequences around the knee joint with intra- and extra-articular components ( and ), and subcutaneous extension insinuating between the tendons, muscles and popliteal vessels (). A similar small lesion was seen at the non-weight-bearing surface of the lateral condyle of the femur, suggestive of an intraosseous lesion (). Hoffa’s fat pad was involved (). Underlying degenerative changes were noted in the bilateral knee joints. Similar cystic lesions were found around the right knee joint, right wrist, abductor pollicis longus, extensor pollicis brevis () and overlying the ring finger, in the flexor tendon at the proximal phalanx of ring finger (). Intraosseous involvement of the capitate and lunate were noted (). With all the above findings and in view of multiple joint involvement, the possibility of cystic ganglionosis or multiple giant ganglion cysts was considered. Aspiration of one of the knee lesions was performed, which yielded a gelatinous material. Fine-needle aspiration cytology showed cyst macrophages in clusters against proteinaceous–mucoid background, without any atypical cells. Also, biopsy of the lesion confirmed the diagnosis of a ganglion cyst (as described in ). As the lesion was diagnosed to be harmless, the patient was advised conservative management and follow-up. With multi-joint involvement of the ganglion cysts, the term “cystic ganglionosis” was used. |
A 79-year-old Caucasian female presented to our institution with chest pain, dyspnea, and a rapidly enlarging thyroid goiter. The patient endorsed that she was previously quite healthy aside from stable hyperthyroidism and a nonenlarging thyroid goiter. Her thyroid had been overactive for years, treated with stable dose of 7.5 mg daily of methimazole for at least the past four years. In 2002, 2003, and 2010, she had had thyroid uptake scans indicating heterogeneous elevated uptake consistent with toxic multinodular goiter and a cold area was identified in the lower right lobe. In 2000 and 2010, she had biopsies of the nodules of the right and left lobes with benign results. Since treatment with methimazole, she had been asymptomatic up until three months prior to presentation, living a mostly independent and having active lifestyle that included cooking, gardening, and daily errands.\nThe patient's family became concerned approximately three months before presentation to our facility because the patient lost a significant amount of weight while her goiter rapidly enlarged. Soon after, she had sudden “terrible pain” in the left side of her neck, diagnosed as an internal jugular vein thrombus. At the time of this diagnosis, her thyroid measured 8.4 × 12 cm. She saw her endocrinologist who noted that her thyroid was hyperfunctioning again and no longer controlled with her daily dose of methimazole, which was subsequently increased. A month later, her labs showed that her thyroid was even more overactive. Her dose of methimazole was subsequently increased to 30 mg per day. She was evaluated for surgery the following month, but surgery was deferred due to uncontrolled hyperthyroidism. The day prior to presentation at our institution she developed chest pressure and tightness when lying down. A computed tomography (CT) scan of her chest and neck showed that her thyroid had grown to 9.8 × 13.2 cm.\nUpon admission, her TSH measured <0.003 μIU/L (reference range 0.5–4.70 μIU/mL), free serum thyroxine (T4) measured 1.56 ng/dL (reference range 0.7–1.25 ng/dL), and free serum triiodothyronine (T3) measured 2.4 pg/mL (reference range 1.7–3.7 pg/mL). Ultrasound of her thyroid showed gross enlargement of both thyroid lobes with the largest nodules measuring 9.7 cm in the left lobe and 5.1 cm in the right lobe. Subsequent CT scan of her chest showed the mass extending into the anterior mediastinum with scattered calcifications within the mass (). There was narrowing of the trachea in addition to multiple pulmonary masses throughout the visualized lung fields. She was continued on methimazole (20 mg twice a day) while hospitalized and started on propranolol and prednisone for symptomatic control. Her free T4 improved and free T3 remained within normal limits on this regimen.\nDue to dyspnea and malignancy concerns, she underwent a total thyroidectomy two weeks after admission to obtain a pathologic diagnosis. Intraoperative findings included involvement of the left recurrent laryngeal nerve and tracheal invasion. Intraoperative biopsies sent for frozen section analysis were reported as poorly differentiated carcinoma, suggestive of anaplastic thyroid carcinoma (). As a result, a prophylactic tracheostomy was placed. The final pathology report confirmed undifferentiated anaplastic carcinoma (8.7 cm) involving the left thyroid, positive for lymphatic involvement (2/5 central nodes) and extrathyroidal extension to the pretracheal cartilage. Surgical margins were positive along the left middle and inferior pole. The right thyroid lobe had multiple benign nodes. Subsequent whole-body CT and nuclear medicine scan found multiple bilateral pulmonary nodules with central cavitation and a destructive right intertrochanteric lytic lesion with pathological fracture (). The final staging was pT4b N1b M1 anaplastic thyroid carcinoma (American Joint Committee on Cancer 7th Edition). Chemotherapy was recommended for her ATC, as she was deemed not a candidate for radiation therapy. The patient's family chose to pursue chemotherapy closer to home. The patient quickly deteriorated towards the end of her hospitalization and missed several follow-up appointments with her medical oncologist upon discharge. She passed away at home within a month after leaving the hospital. |
Our patient is a 3-year-old Pakistani female born to first-degree cousins. She presented at the age of 10 months with enlarged cervical lymph nodes and diarrhea. She had multiple admissions for recurrent diarrheal illnesses. The diarrhea started at the age of 7 days, with a frequency of more than 10 times per day of watery consistency but no blood or mucus. She was started on an elemental amino acid-based formula because of a diagnosis of food protein-induced enterocolitis syndrome based on a presentation of severe bloody diarrhea, acidosis and hypotension. She had a sister who had a similar presentation of chronic diarrhea and fever and died at the age of 1 year due to septic shock. Immune deficiency was not suspected and the underlying cause was not investigated. Also, no genetic tests were obtained and the family did not receive any prenatal genetic counseling.\nAfter removing cow’s milk from our patient’s diet and initiating the special formula, her diarrhea settled, but at the age of 1 year, she had a recurrence of diarrhea described as bloody and associated with multiple perianal abscesses. The abscesses were treated with intravenous antibiotics and surgically drained. The culture from the drained pus repeatedly grew extended spectrum β-lactamase-producing E. coli and Klebsiella species. At the age of 2 years, she developed arthritis involving large joints of the lower limb (knees and ankle) and wrist associated with morning stiffness and an inability to walk.\nOn examination, the patient had severe failure to thrive. Multiple enlarged cervical lymph nodes were noted. The scar of the Bacillus Calmette-Guérin (BCG) vaccine was normal, and no axillary lymph nodes were detected. The liver was palpable 3 cm below the costal margins. She had swelling of the left knee joints with a reduced range of movement. Perineal examination revealed inflammation, multiple scars of drained perianal abscesses and a perianal fistula opening (Fig. ).\nLaboratory investigation revealed leukocytosis (29.1 × 109/L), anemia (hemoglobin 8.4 g/dL), elevated inflammatory markers (C-reactive protein 12.0 mg/dL, and ESR 80 mm/h) and elevated immunoglobulin levels. A rheumatological workup was positive for serological markers of autoimmunity, including antinuclear antibodies (ANA) (titer 1:80, speckled pattern), rheumatoid factor (25.1 IU/mL; normal < 15), anti-cyclic citrullinate peptide (anti-CCP) antibody titer (216.7 u/mL; normal < 20) and anti-double-stranded DNA (anti-dsDNA) antibodies (83.6 IU/mL; normal < 35). In addition, these markers were positive on repeated testing. Other markers, including anti-RNP, anti-SSA, anti-SSB, and anti-Sm, remained negative, while C3 and C4 complement levels were not suppressed. Joint fluid sampling was obtained, and an infectious etiology for joint involvement was excluded by culture obtained from the knee joint. Based on the clinical presentation and laboratory workup, a diagnosis of oligoarticular juvenile idiopathic arthritis (JIA) was provided.\nFurthermore, microbiological testing for infectious diarrhea, including stool culture and sensitivity tests, a stool ova and parasite examination, Clostridium difficile toxin test, and Giardia lamblia and Cryptosporidium stool antigen tests, were negative. The celiac panel was also negative, and malabsorption tests, including fecal fat and fecal elastase tests, were normal. Stool examination was positive for fecal occult blood, and fecal calprotectin, an indicator of intestinal inflammation, was elevated at 580 mcg/g (normal < 50.0). Workup for TB was negative, including bone marrow and lymph node examination, using polymerase chain reaction (PCR) assay, cultures, and the TB gold test. In addition, a purified protein derivative (PPD) skin test was performed on the patient and showed no reaction.\nIn view of her presentation and the early colitis symptoms, she was further evaluated, and a diagnosis of CGD was made based on the oxidative burst assay [dihydrorhodamine-1,2,3 (DHR) flow cytometry test], which revealed an absence of NADPH oxidase activity in the patient’s neutrophils upon phorbol myristate acetate (PMA) stimulation (Fig. ). The neutrophil oxidative index (NOI) of the patient was < 5. NOI measures the oxidative ability of phagocytes and their overall integrity and is calculated from the ratio of the fluorescence in stimulated phagocytic cells to the fluorescence expressed in unstimulated phagocytic cells []. The normal value in control specimens is usually > 73 [].\nThe diagnosis of CGD was confirmed by targeted next-generation sequencing using Ion AmpliSeq Designer software (Life Technologies, Carlsbad, CA, USA). The gene panel used analyzed over 200 genes associated with immune deficiency and immune dysregulation, including auto-Inflammatory and Autoimmunity syndromes. The test was performed on venous blood samples collected in EDTA tubes for DNA extraction after obtaining written consent from the parents to perform the genetic diagnostic assay. The test revealed a deletion mutation in exon 10 as a homozygous loss-of-function variant of NCF2 (NCF2: NM_001190789, nucleotide change: c.855_856del:p.T285fs). Both parents were heterozygous for the mutation. We believe this to be a novel variant that has not been previously reported.\nTo prevent serious and life-threatening infections, the patient was started on an antibacterial and antifungal prophylactic regimen consisting of trimethoprim-sulfamethoxazole (trimethoprim 6 mg/kg/day) and itraconazole (5 mg/kg/day), respectively. She remained free of infection. Her arthritis symptoms were initially managed with naproxen followed by oral prednisolone at a dose of 1 mg/kg/day for 4 weeks followed by gradual tapering and then discontinued after 4 months. In addition, hydroxychloroquine was initiated and continued throughout the observation period, which lasted for 18 months following the onset of arthritis. She showed improvement and started to walk with no pain and had no relapse of symptoms during subsequent follow-up visits. However, we failed to establish long-term follow-up. In addition, further endoscopic, histological and imaging studies were planned but not performed because the patient and family traveled to their home country and were lost to follow-up. |
A 47-year-old man with no significant past medical history, and no risk factors for atherosclerosis, presented to us with an anterior ST-elevation myocardial infarction. He reported an event of some minor chest pressure 2 to 3 weeks prior to admission. On the morning of admission, he developed severe chest pain accompanied by diaphoresis and dyspnea. The patient was taken urgently to the catheterization laboratory. During transport to the laboratory he developed ventricular fibrillation, from which he was successfully resuscitated with a single defibrillation. He then had an angiogram, which demonstrated a 100% thrombotic occlusion of the proximal LAD, without any other significant lesions. A mechanical thrombectomy was performed, followed by balloon angioplasty and a drug-eluting stent implantation, with excellent angiographic results. An intravascular ultrasound (IVUS) performed during the procedure identified a MB 23 mm distal to the point of maximal plaque burden. The MB was greater than 12-mm long, had a systolic compression of 14.3%, and an MB thickness (halo) of 0.41 mm (-). Clinical outcome was good; the patient remained stable during the hospitalization and is asymptomatic since then.\nA 41-year-old man, a visitor from Europe and an athlete, with borderline hyperlipidemia and no other past medical history, had completed the San Francisco Marathon on the morning of the index event. He had vomited twice shortly after the race, and then returned to his hotel. Two hours later, he developed substernal chest pain, lasting approximately 30 minutes, accompanied by shortness of breath. Learning about the possible cause of the symptoms from the World Wide Web, he decided to contact emergency medical services. He was brought in to the emergency department, where an EKG (electrocardiogram) showed ST elevations in the anterior and inferior leads, with sinus bradycardia. Coronary angiography was urgently performed, revealing an intermediate lesion in the proximal LAD, as well as a total cut-off of the distal wraparound LAD, likely due to the proximal plaque rupture and distal embolization. The rest of the coronary tree did not demonstrate angiographic evidence of disease. Following mechanical thrombectomy of the embolized thrombus and restoration of TIMI 3 flow, IVUS of the artery demonstrated a proximal plaque rupture, as well as a mid-LAD MB. The bridge was located 10 mm distal to the plaque, and measured 14 mm in length, had a systolic arterial compression of 24.2%, and had a halo of 0.4 mm ( and ). Left ventriculography showed a normal ejection fraction, with anterior and apical hypokinesis. A drug-eluting stent was implanted successfully in the proximal lesion, without complications. The patient later suffered from transient nonsustained ventricular tachycardia, but otherwise was asymptomatic, and had remained so after his discharge. He returned to his home country later that month. |
We describe a case of a 40-year-old female nurse who presented in May 2003 with an 18-month history of pain in the left thumb. Clinical examination showed soft-tissue swelling around the left thumb metacarpal. Radiographs showed an expansile lesion occupying most of the metacarpal with periosteal reaction and cortical destruction. MR scan () showed extensive destruction with expansion of the shaft of the first metacarpal of the left hand, associated with a soft-tissue mass and an extraosseous component, primarily on the dorsal aspect. On T\n1 weighted images, the lesion was of low signal; on fluid-sensitive sequences, the lesion showed high signal with a thin and irregular septae. Following contrast medium administration, inhomogeneous, multilobulated, peripheral enhancement of the lesion was noted but also areas of non-enhancement predominated centrally, consistent with a cartilaginous lesion.\nThe CT scan () demonstrated a lytic lesion involving virtually the entire shaft of the first metacarpal of the left hand and reaching the subchondral bone plate at both ends. The lesion demonstrated a significant expansion of the bone with small areas of punctuate calcification, significant thinning of the cortex circumferentially and a breach in the cortex with some new bone formation on the dorsal aspect. Some soft-tissue swelling was also seen. There was no evidence of pulmonary metastases on the CT scan () at initial diagnosis.\nAn open biopsy was performed in June 2003, the histology of which revealed a grade II chondrosarcoma with evidence of spread beyond the cortex into the periosteum. Vascular invasion was not identified. 1 month later, under general anaesthesia, a first ray amputation was performed with disarticulation of the thumb at the trapezioscaphoid joint. A tourniquet was used at the time of the surgery to exsanguinate the limb. Macroscopic examination revealed an expansion of the metacarpal bone due to a chondroid tumour measuring 35 × 25 mm, which eroded through the cortex but did not cross the metacarpophalangeal joint. Microscopic examination of the sections () confirmed a grade II chondrosarcoma, which showed an extensive permeative growth beyond the cortical bone. There was no de-differentiation and the tumour was contained within the periosteum. The lesion was completely excised, although the closest margin was 1 mm on the dorsal aspect.\nIn April 2004, the patient noticed a superficial, firm swelling in the medial aspect of the left upper arm, which was not particularly painful. There was no clinical or radiographic evidence of local recurrence in the hand. An MRI scan demonstrated a subcutaneous lesion () on the medial aspect above the elbow with signal characteristics similar to the previous chondrosarcoma in the ipsilateral hand. The mass was closely related and in the line of the cephalic vein; it showed high signal on the T\n2 weighted images, intermediate signal on the T\n1 weighted images and peripheral enhancement after intravenous gadolinium administration. Subsequently, an excisional biopsy of the left upper arm lesion was performed. Macroscopically, the soft-tissue mass showed encapsulated grey myxoid tissue, which was centrally cystic and partially haemorrhagic. This was microscopically confirmed to be a grade II chondrosarcoma, most likely representing a metastasis from the previous thumb chondrosarcoma. There was no lymphoid tissue seen that could be associated with the subcutaneous lesion to suggest lymph node metastases. The closest margin was less than 1 mm. At the same time, a biopsy of the hypertrophic scar at the site of ray amputation was performed, which did not reveal any evidence of neoplasia on histological examination. Owing to the close margins of this subcutaneous lump on excision, a wider excision was performed a month later under general anaesthesia.\nIn June 2004, a CT scan of the chest was performed and a solitary tiny nodule in the right middle lobe (), which was suspicious for lung metastasis, was seen. There was no mediastinal or hilar lymphadenopathy seen. The lesion continued to increase in size and the patient had a pulmonary lobectomy in November 2004. Within 2 months, however, she developed further lung metastases. In January 2005, she received palliative chemotherapy, but even after three cycles, the pulmonary lesions continued to increase in size and number () and it was decided to discontinue further treatment. The patient died subsequently from the disease. |
A 21-year-old man (height, 158 cm; weight, 40 kg) was scheduled for wedge resection with video-assisted thoracoscopic surgery for treatment of pneumothorax. The patient had visited our hospital because of sudden chest discomfort the day before admission. He had been diagnosed with CMTD 14 years previously. Surgical treatment of scoliosis had been performed 6 years previously, and chest tube insertion for treatment of right pneumothorax had been performed 3 years previously. The patient had also undergone surgery for CMTD-related bilateral foot deformities and arm dislocation. He reported that the surgery and anesthesia were uneventful at that time. Although the operations for the scoliosis, foot deformities, and arm dislocation had been conducted under general anesthesia, no other information was available because the operation had been performed in another hospital. The patient had muscle weakness in both upper extremities (manual muscle testing grade 2−), and only smartphone manipulation was possible using finger movements. Atrophy and deformation of both feet were noted (), and the patient was unable to stand up on his own. His condition was so severe that even using a wheelchair was not possible. Neck movements were normal, and physical examination showed atrophy of the peroneal muscles of both lower extremities (manual muscle testing grade 1) with normal sensory function. The tendon reflexes were decreased in the knees and ankles. Additionally, atrophy was present in the upper extremities.\nThe patient’s mouth opening width was normal, thyromandibular length was >5 cm, Mallampati classification was class II, and American Society of Anesthesiology physical status was grade III. Preoperative laboratory studies showed no abnormal findings. Chest radiographs showed right chest tube insertion for treatment of right pneumothorax accompanied by fixation of the thoracolumbar spine with scoliosis (, ). Electrocardiography (ECG) revealed signs of left anterior fascicular block, and the patient’s vital signs were as follows: blood pressure (BP), 120/60 mmHg; heart rate (HR), 64 beats/minute; body temperature (BT), 36.6°C; oxygen saturation, 100%; and respiratory rate (RR), 20 breaths/minute.\nPremedication was not given because the patient had CMTD with pneumothorax. The patient was transferred to the operating room and underwent noninvasive BP, ECG, pulse oximetry, and bispectral index monitoring. Additionally, a neuromuscular monitoring (NMT) module (IntelliVue NMT module, Model 865383; Philips, Amsterdam, Netherlands) was set up. Before induction, the patient’s vital signs were as follows: BP, 116/63 mmHg; HR, 84 beats/minute; BT, 36.4°C; oxygen saturation, 100%; and RR, 12 breaths/minute.\nAnesthesia was induced with 2% propofol (Fresofol 2%; Fresenius Kabi, Bad Homburg, Germany) and remifentanil (Ultiva; GlaxoSmithKline, Brentford, UK) administered at an effect-site concentration of 2.5 μg/mL and 2 ng/mL, respectively, using a target-controlled infusion pump (Orchestra Base Primea; Fresenius Vial, Brezins, France) under 100% oxygen inhalation (6 L/min). A neuromuscular block was achieved using intravenous injection of 15 mg of rocuronium. However, as the patient’s respiration continued, 5 mg of rocuronium was administered again after 2 minutes.\nA 35-Fr left-sided double-lumen tube was inserted without difficulty. Anesthesia was maintained with 2% propofol at 1.5 to 2.5 μg/mL and remifentanil at 1 to 2 ng/mL (effect-site concentration) using a target-controlled infusion pump for TIVA, with 1 L/minute of oxygen and 2 L/minute of air.\nFor one-lung ventilation, the pressure-controlled ventilation–volume guaranteed mode was used with a tidal volume of 250 mL, RR of 14 breaths/minute, and peak airway pressure of 25 cmH2O. The patient was switched to the left lateral decubitus position for the video-assisted thoracoscopic surgery. However, the patient’s muscle relaxation was insufficient, and there was a collision between the ventilator and self-respiration. Therefore, 5 mg of rocuronium was administered again.\nNo adverse events occurred during the surgery, and 0.4 mg of glycopyrrolate and 15 mg of pyridostigmine were administered to reverse the neuromuscular block. The endotracheal double-lumen tube was removed after confirming that the patient had recovered spontaneous breathing and consciousness.\nThe total duration of anesthesia was 55 minutes, and that of the surgery was 25 minutes. The patient was transferred to the post-anesthesia care unit, and his vital signs were as follows: BP, 112/78 mmHg; HR, 68 beats/minute; BT, 36.5°C; oxygen saturation, 100%; and RR, 24 breaths/minute. After the patient was monitored for about 1 hour in the post-anesthesia care unit, he was transferred to the ward.\nNo abnormalities were found on the postoperative laboratory studies, chest radiographs or ECG or with the patient’s movement and respiration. The patient was discharged on postoperative day 3.\nThis study was approved by the institutional review board of Eulji University Medical Center and was performed in compliance with the EQUATOR Network guidelines. Written informed consent for publication was obtained from the patient and his parents. |
In our case, a 68-year-old man with a past medical history of end-stage renal disease, atrial flutter, and peripheral vascular disease presented to the hospital with severe, pressure-like chest pain. The initial electrocardiogram (ECG) was abnormal as shown in Figure . Initial laboratory workups, including electrolytes and cardiac enzymes, were within normal limits. The ECG in Figure demonstrates a narrow QRS complex tachycardia with normal axis. Borderline low voltage QRS complexes along with elevated ST-segments in leads II, III, and aVF are also apparent. The patient's ECG and presentation was concerning for an inferior ST-segment elevation myocardial infarction (STEMI). However, careful inspection of the ECG will reveal that the rhythm is in fact atrial flutter with 2:1 conduction to the ventricles at a rate of 106 beats per minute, and the flutter waves (tachycardia cycle length 289 ms) are falling on the ST segment of the ECG. This is best seen in lead V\n1, where the flutter waves are positive.\nIn the index case, however, the patient was taken to the cardiac catheterization lab given high pretest probability of ischemic disease, and no evidence of coronary artery disease was found. During the cardiac catheterization, the patient spontaneously converted to sinus rhythm with resolution of ST segment changes. A previous ECG (Figure ) demonstrates atrial flutter with slow ventricular response. On this tracing, flutter waves are intermittently superimposed on the ST segments (thick arrows), and when not overlapped, clearly demonstrates the ST segment is normal (thin arrows).\nPrevious cases have been reported where the timing of the flutter waves raise the suspicion for ST-segment elevation, mimicking a STEMI. In these cases, reciprocal changes should be carefully evaluated, and patient history may also provide a diagnostic clue. Whenever the diagnosis of myocardial injury is unclear due to the possibility of an artifact or atrial flutter, simply repeating the ECG during carotid sinus massage or administering AV blocking agents may fortuitously dissociate the ST segments from flutter waves just briefly enough to elucidate the correct diagnosis as shown in Figure . Our case illustrates how striking the elevation of ST segments can appear when atrial flutter waves are incidentally superimposed upon the ST segments.\nRecognition of this arrhythmia can potentially expedite treatment (rate control and/or cardioversion) and spare the patient invasive studies. |
A 71 y/o man with an environmental exposure to insulation for 20 years while working at NASA, with less than a 10-pack year history of smoking presented to the community emergency department with severe dyspnea and low grade fever. He was diagnosed and admitted for bacterial pneumonia, treated for two days with IV antibiotics, and discharged on oral antibiotics. His symptoms did not improve prompting two additional hospital admissions over 3 months. Chest CT ultimately demonstrated intraseptal thickening, areas of peribronchial consolidation, and ground-glass opacity involving the lower lobes. A surgical lung biopsy revealed organizing pneumonia associated with alveolar septal inflammation leading to a final diagnosis of COP. The patient was initially treated with Prednisone 80 mg daily that was later tapered to 60 mg 4 weeks later. Azathioprine was also started at 100 mg daily as a steroid sparing agent. Despite these interventions, his dyspnea continued and he was referred to our institution for further evaluation. On presentation, several painful, variably pustular papules and small nodules were present on the face and extremities. He reported an outdoor fall 8 weeks earlier and unsuccessful treatment with both doxycycline and cephalexin given for one month.\nA biopsy by 4 mm punch technique of a fluctuant nodule on the arm () revealed a dermal abscess with gram-positive filamentous rods characteristic of Nocardia species (). A modified acid-fast Kinyoun stain was also positive. Chest CT showed interval development of bilateral upper lung nodules. The patient was directly admitted to the hospital and began treatment with intravenous imipenem in conjunction with oral trimethoprim-sulfamethoxazole (TMP-SMX). Magnetic resonance imaging of the brain and a CT scan of the abdomen and pelvis were negative for other end-organ involvement. Tissue cultures grew Nocardia brasiliensis susceptible to augmentin and TMP-SMX.\nThe patient was discharged 5 days later after showing clinical improvement, with plans to continue TMP-SMX therapy for 6 months. At follow-up 5 weeks after discharge, all cutaneous lesions had cleared, and repeat chest CT showed remarkable improvement with only minimal left upper lobe involvement. The Pulmonary Function Test completed reported minimal airway resistance and negligible airway obstruction. |
The patient was a 1.5-year-old boy who was admitted to the hospital with a primary complaint of fever, diarrhea, and vomiting. At the beginning of the hospitalization, the patient had a generalized clonic-tonic seizure. The patient was dispatched to our intensive care unit on the fourth day of admission due to reduced consciousness.\nPatient is the first child of a family and is resident of the village. He had a history of pneumonia at 6 months of age. There was a history of seizure of fever in his family. From the outset, he was treated with ceftriaxone, vancomycin, phenytoin, and acyclovir. The growth and development were normal.\nThe patient had tachypnea and tachycardia, and fever was 39°C. Percent oxygen saturation without getting oxygen was 98%. It was toxic and had GCS = 8. The patient had a mild tenderness on the right side of the abdomen with a predominance in the RLQ (right lower quadrant). The rectal examination was normal. The amount of urine output was normal. Patients were treated with liquid therapy, and intravenous antibiotics were changed to moropenem and vancomycin. Patient tests: serum electrolytes (sodium and potassium), blood gas analysis, coagulation tests, liver and kidney function tests, vidal test and Albumin were normal.\nSome of the patient's laboratory results were as follows:\nIn addition, he had hypocalcemia and Hypomagnesemia and were treated with sepsis as a common symptom. Due to biliary secretion from the stomach tube, bloody diarrhea, tenderness and abdominal distension, abdominal ultrasonography was performed with suspicion of obstructive problems such as enuresis and acute appendicitis.\nUltrasound findings favored the diagnosis of acute appendicitis: insignificant free fluid in the space between the interleuk and a non-compressible appendix of 65 mm in the lower right quadrant of the abdomen was evident. The patient underwent appendectomy (Figure ). One day after the surgery, the child was alert (Glasgow Coma Scale /Score GCS = 12), but the fever continued. Metronidazole and intraperitoneal ciprofloxacin were administered. Two days after the commencement of these antibiotics and appendectomy, the patient's alertness became normal, and the general condition was satisfactory with erythrocyte sedimentation reaction (ESR) = 32, and platelet count and white blood cell count increased. A pathological report of acute puffiness appendicitis with peri appendicitis confirmed. After two weeks, the patient was discharged with a good general condition. |
A 45-year-old man presented to our otorhinolaryngology outpatient department with the symptoms of persistent odynophagia and hoarseness for 1 week after ingesting a fish bone. There was neither dysphagia nor dyspnoea. At first, he sought medical consultation in a local clinic and cervical X-ray was done. However, it was not conclusive, and there was no sign of presence of foreign body in the pharynx. Due to the worsening of his symptoms, he presented to our clinic on 25 August 2016. Flexible pharyngolaryngoscopy was done, and it was noted that there was left vocal cord immobility, oedematous left aryepiglottic fold, and left pyriform fossa with secretions in it. We could not find any foreign body in his pharynx nor in larynx. However, based on his history, the presence of fish bone impaction in the larynx was suspected. The patient was then admitted to the hospital, and urgent computed tomography (CT) scan of the neck was done and revealed a 3 cm linear foreign body embedded in the soft tissue medial to the left thyroid cartilage with surrounding hypodense soft tissue swelling causing mild indentation of the left vocal cord extending into the supraglottic region up to the left pyriform sinus and downwards minimally extending to the infraglottic region causing mild asymmetric narrowing of the involved laryngeal segments and causing mass effect on the glottis and supraglottic airway causing mild asymmetric narrowing ().\nThe diagnosis of inflammation of the paraglottic region and vocal cord immobility secondary to fish bone impaction in the paraglottic space was made. The patient was then shifted to the operation theatre (OT), and rigid direct laryngoscopy was performed under general anaesthesia. It revealed oedema in the left pyriform fossa and left supraglottic region, and no foreign body was visualized. Also, rigid oesophagoscopy was performed, and it showed the same findings as the laryngoscopy. Therefore, foreign body retrieval endoscopically has failed.\nThen, we proceeded to the external approach, and thyroid cartilage window with a Skeeter drill window of 12 mm [] was made and the foreign body was retrieved (Figures and ). Then, the wound was sutured and a corrugated drain was inserted. The patient was then shifted to the intensive care unit (ICU) for observation, and he was put on mechanical ventilation. We started dexamethasone IV 8 mg TDS to reduce oedema. He was also given Augmentin (amoxicillin/clavulanic acid) IV 1.2 g TDS as a prophylactic, and analgesia was given when required.\nThe progress of the patient was well, and he was extubated. We started him on oral feeds in the ICU before shifting him to the ward. After his condition improved, he was shifted to the ward and laryngoscopy was repeated, and it showed improvement in the mobility of the left vocal cord and no oedema was noted. In the ward, the patient was started on normal feeds and he tolerated them well and was discharged.\nOn the following laryngoscopy two weeks later, the swelling of the left pyriform fossa and the left aryepiglottic fold has subsided, and the function of the vocal cords movement was recovered. |
The patient is a 63-year-old man with a medical history of hypertension, hyperlipidemia, heart failure with preserved ejection fraction, and coronary artery disease who originally presented to our center from an outside hospital with right-sided weakness and speech difficulties. On arrival, his National Institutes of Health Stroke Scale (NIHSS) score was 12 owing to right arm drift, right leg weakness, aphasia, and dysarthria. The drift was thought to be due to inability to follow commands fully. Due to lack of venous access, magnetic resonance imaging (MRI) and magnetic resonance angiography were performed instead of computed tomography angiography. A left anterior cerebral artery (ACA) infarction secondary to a left A1 occlusion was demonstrated. Diffusion-weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) sequences are shown in Figure . Given the completed infarction, no tissue plasminogen activator was administered and thrombectomy was not performed. The patient met sepsis criteria so blood cultures were obtained, and he was admitted to the neurosciences intensive care unit for further management. He was also noted to have atrial fibrillation with rapid ventricular response at that time.\nBlood cultures grew Streptococcus constellatus for which intravenous (IV) antibiotic therapy with ceftriaxone (2 g twice daily) and metronidazole (500 mg every 8 h) was initiated. To investigate hyperbilirubinemia on initial laboratory testing, liver ultrasonography was performed, demonstrating a hepatic abscess. He then underwent image-guided abscess drainage followed by placement of a pigtail drain. A transthoracic echocardiogram demonstrated severe mitral valve regurgitation. Given the high suspicion for endocarditis, a transesophageal echocardiogram was performed. This demonstrated mitral valve vegetations. Following completion of his medical care, the patient was deemed stable for discharge to a rehabilitation facility. The antibiotic plan at that time was 14 days of oral metronidazole (500 mg twice daily) and four weeks of IV ceftriaxone at the previous dose given his downtrending infectious markers. For stroke prevention he was placed on apixiban 5 mg twice daily. He recovered a good portion of his right-sided strength and was discharged home after completing his rehabilitation course.\nThe patient was readmitted to our center four days post-discharge with worsening right-sided weakness (NIHSS score of 3). Brain perfusion imaging demonstrated no new perfusion deficits or large vessel occlusions. Repeat MRI DWI/FLAIR sequences demonstrated evolution of the left ACA territory FLAIR signal and diffusion restriction (Figure ). This was concerning for worsening ischemia or an abscess. Contrast-enhanced MRI was consistent with an intracranial abscess within the previous stroke bed (Figure ).\nBecause the abscess was abutting the frontal horn of the lateral ventricle, the patient was emergently taken to the operating room for drainage. The abscess was localized using Stealth® (Medtronic, Minneapolis, MN, USA) navigation, and an incision site was marked. After craniotomy, a small corticectomy was performed over the abscess. Thick, yellow pus was immediately encountered. Approximately 30 mL was drained; however, it was noted that there was a component of organized phlegmon. Therefore, an IRRAflow® catheter was placed in the cavity in order to irrigate and promote drainage following closure. After the procedure, imaging was obtained (Figure ) prior to the initiation of irrigation (20 mL per hour with 0.9 normal saline). The patient was then placed on IV antibiotics consisting of vancomycin (1.75 g every 12 hours) and cefepime (2 g every 8 hours), in addition to oral metronidazole (500 mg every 6 hours) until culture results were received. On postoperative day 2, the irrigation solution was switched for 50 mg of vancomycin diluted in 1 L of normal saline, and this solution was continuously irrigated through the IRRAflow® system.\nAfter intraoperative cultures revealed Staphylococcus hominis, the systemic antibiotic regimen was changed to ceftriaxone (2 g daily) and metronidazole (500 mg every 8 h). The patient was observed for one week with decreasing infectious markers. Serial head CT scans were performed during this time. As demonstrated in Figure , a small left frontal subdural fluid collection developed. It decreased after the irrigation rate was lowered to 10 mL per hour. Repeat MRI showed significant resolution of the abscess (Figure ). From a neurologic standpoint, the patient experienced a reduction of his NIH to 2 for improved right leg weakness, right arm drift, and aphasia. Given these findings, the drain was removed on postoperative day 8. CT obtained the following day demonstrated continued resolution of the subdural fluid collection.\nThe patient had an extended hospital course due to a cholecystoduodenal fistula that was found on investigation for the cause of his liver abscess. A periampullary mass thought to represent malignancy was found during endoscopic retrograde cholangiopancreatography. These issues were managed by the general surgery, oncology, and medical services. No other acute neurosurgical issues arose during the remainder of his hospitalization, and he was discharged to rehabilitation after finishing his one-month course of antibiotics. He is scheduled to follow-up in clinic following rehabilitation. |
An emaciated 30-year-old female arrived at a residential addiction treatment centre for a six-week program for treatment of a Cannabis Use Disorder. She endorsed escalating use of marijuana in the last few years and estimated smoking one gram per day in recent months. When she first started using marijuana in her early twenties, she only used it socially with friends. She reported that her use escalated in the last few years specifically to control nausea and to increase her appetite. In fact, she could not eat a meal in the last year without first smoking marijuana. She had also been experiencing low mood, anxiety, and panic attacks for the past six years since starting graduate school, for which she was on Cipralex 20 mg daily and Wellbutrin XL 300 mg daily. She endorsed vomiting a few times per week to once daily. This vomiting was not in response to feelings of guilt after having eaten too much but rather a response to feelings in her stomach when she had not eaten enough or when she had eaten without marijuana use. She denied any body image issues. She had lost twenty pounds over the past year, with a current weight of 49.6 kg and a BMI of 17. She had been diagnosed by her Psychologist with Avoidant/Restrictive Food Intake Disorder and had been in outpatient therapy for her eating disorder for the past year. Other aspects of medical history included a diagnosis of hypothyroidism, for which she was taking thyroid hormone replacement.\nUpon arrival at the residential facility, she endorsed having not eaten anything for at least the past two days and was feeling lightheaded and unwell. Her blood pressure measured 80/49. She was subsequently sent to a nearby hospital for assessment. In the Emergency Department, her blood pressure was 88/54. Serum sodium was 126 mmol/l, potassium was 4.8 mmol/l, urea was 8.9 mmol/l, creatinine was 97 mmol/l, and glucose was 3.1 mmol/L. As a result of her symptoms and hyponatremia, she was admitted to the Internal Medicine service. The Endocrinologist who saw her during her admission noticed that her skin had a bronzed hue, and the patient shared that her skin suddenly became tanned nine months earlier. Her potassium level rose to 6.3 mmol/l the following day. Given her symptoms, hyperpigmentation, and lab findings, a presumptive diagnosis of Addison's disease was made. ACTH was measured to be >278 mmol/L, and random cortisol was 25 mmol/L. She was started on Hydrocortisone 10 mg daily and discharged back to the residential facility once her symptoms and lab abnormalities normalized. Within a few days, her eating completely normalized, she denied any nausea or vomiting, and her anxiety resolved. She went on to successfully complete the residential program. |
A 78-year-old woman (body mass index, 20.3 kg∙m−2) presented with pocket hematoma. The patient had a significant cardiac history: pacemaker implant for complete atrioventricular block in 1994, percutaneous coronary intervention and coronary artery bypass graft after acute myocardial infarction in 2004, and combined aortic and mitral valve replacement in 2009. She also had a left ventricular aneurysm. A pocket hematoma was noted after generator change (Figure ). The generator needed to be explanted before it eroded skin. The atrial pacing had been abandoned due to the high impedance. However, insertion of the additional atrial lead with generator replacement was avoided due to the bilateral mastectomy for breast cancer and the necessity of anticoagulation for mechanical valves. We decided to implant a TPS concurrently with the explant of the generator and hematoma.\nThe procedure was performed under therapeutic anticoagulation with warfarin. A delivery catheter was directed toward the right ventricular septum. We reviewed the left anterior oblique view to ensure that the device cup was placed on the septum (Figure ). First, a small amount of contrast media was injected via the delivery catheter. Contrast media pooling was observed before the deployment of the TPS (Video and Figure ). We changed the implantation site. Finally, we deployed the tines of the TPS at the mid-septum, which yielded good electrical data. Although the contrast material was retained in the pericardial space, the patient remained hemodynamically stable, and no effusion was noted with echocardiogram.\nThree days after the implant, a cardiac computed tomography scan was performed. A protruded diverticular structure on the right ventricle was observed, and the contrast material had remained in a pouch within the pericardium (Figure ). The patient continued to take warfarin. She was asymptomatic and discharged although subclinical cardiac perforation from the device cup of the TPS was suspected. The TPS showed normal functionality at 3 months after the implant. |
A 12-year-old, 161 cm, 32 kg, female patient with idiopathic scoliosis in the thoracic vertebra presented for posterior fusion operation. She had no significant medical issues and was not taking any medication. Blood tests before the operation were in the normal range, and no abnormalities except for idiopathic scoliosis were found on the simple chest X-ray. X-rays on the vertebrae revealed severe scoliosis from the fifth thoracic vertebra to the first lumbar vertebra. Prior to anesthesia, atropine 0.3 mg was administrated. Before anesthesia, the pulse oximetry was 100% and the blood pressure was 120/79. The induction of anesthesia included administration of propofol 50 mg and succinylcholine 40 mg, followed by a catheterization. The anesthesia was maintained with N2O 2 L/min, O2 2 L/min, desflurane 5%, atracurium 15 mg. To check collateral circulation in the left hand before the induction of anesthesia, Allen's test was performed, and by inserting a 22 G catheter into the left radial artery, arterial pressure was measured. When a monitoring catheter was inserted into the right internal jugular vein, the central venous pressure was 10 mmHg.\nFor the surgery, the patient was turned from a supine position to a prone position, and then her shoulder joints were abducted at 120° and the elbow joints were flexed at 70° while the neck was maintained in neutral position.\nAfter the transition in position, the blood pressure indicated 100/60 mmHg and the central venous pressure was 10 mmHg, showing no change. However, the systolic blood pressure dropped from 90 to 40 mmHg while inserting an introducer needle after skin incision and muscle dissection. We suspected damage in great vessels and checked the operative field after consulting the surgeon, but not a significant bleeding was observed. The blood loss had amounted to about 500 ml, so 6% hydroxyethyl starch (HES) 500 ml (Salinhes®, Ilsung pharmaceuticals, Korea) was administrated in order to complement the volume in the vessels, and the urine was 90 ml, and the central venous pressure was maintained at 9 mmHg or so. While the operation was ceased, the systolic blood pressure was restored to 90 mmHg. When the operation was resumed, the systolic blood pressure repeatedly dropped back to 40 mmHg. Despite the sudden drop in the arterial pressure, no bleeding was found in the operative field, so we suspected blood pressure lowering caused by the reduction in venous return and cardiac output owing to a pressure on the inferior venacava resulted from a pressure on the vertebrae during the spine surgery. As the surgeon applied a compressing force to the surgical region with the same intensity as in usual surgical process in order to confirm such possibility, a drop close to the previous one was detected. The difference between compressing and not compressing on the region was 40-50 mmHg in the systolic blood pressure, but the central venous pressure was 9 mmHg, not showing much change while the arterial pressure was changing. Then, the possibility of the blood pressure reduction due to such causes was ruled out. While repeating the same process, the arterial pressure was measured at the right upper arm region by a automated noninvasive blood pressure device when the blood pressure in the left radial artery decreased, but the blood pressure did not change when compressing force was applied to the vertebrae. Then, to make a more exact comparison, a monitoring catheter was inserted into the right radial artery. Despite a series of repeated compressing that triggers a drop in the left radial arterial pressure, the blood pressure in the right radial artery did not show a significant change, while the systolic pressure in the left radial artery was lower than in the right one by 40-50 mmHg (). Suspecting a disease responsible for reducing blood flow in the left upper arm, we monitored the blood pressure in the right radial artery. The blood pressure at the radial artery in the left upper limb decreased every time there was a pressing force applied to the surgical site, but the surgery was resumed with close observation because the pulse oximetry on the left middle finger was maintained 100% and no abnormalities except for the blood pressure decline were observed. Thereafter, the surgery was finished without any particular incident and the patient was turned from the prone to the supine position for awakening. After the repositioning, the left radial artery pressure was restored to a similar level to that of the right one and the difference of the systolic blood pressure in the two limbs remained within 5 mmHg. The total length of the surgery was 3 hours and 50 minutes and the anesthesia lasted 5 hours and 15 minutes.\nThe patient was transported to the postanesthetic recovery unit after awakening, and on postoperative day 8, Roos' test was performed to confirm thoracic outlet syndrome. The test revealed a decrease in vascular tone and paleness in the left upper limb. There was no dysesthesia, but she complained about uncomfortable feeling in the left upper limb. On postoperative day 10, an Angio Computed Tomography found that the first rib and the clavicle compressed the right subclavian artery at the same position as in the operation, so we diagnosed her case with TOS (). The blood pressure at the supine position after the operation showed just insignificant difference between the bilateral upper limbs and there was no reproduction of the symptoms that she had felt. Then on postoperative day 18, she was discharged home under follow-up medical observation. |
A 29-year-old female at 37 weeks of an uneventful pregnancy attended antenatal clinic. During her assessment a large predominantly left sided thyroid mass was noted. She was reviewed during that attendance by the local otolaryngology team. She had no history of thyroid pathology, had no lifetime radiation exposure and no relevant family history. There was no palpable cervical lymphadenopathy. Her vocal cords were mobile and symmetrical. Spirometry revealed no intrinsic pulmonary pathology.\nAn emergency Caesarian section was performed at term + 2 days for fetal distress without intra-operative complication. The child was healthy. Six days post partum she developed a tender erythematous lower abdominal wound, was diagnosed with a wound site infection. A Caesarean section wound swab grew mixed skin flora with anaerobes and treatment was initiated with 625 mg co-amoxiclav. During this time she underwent an ultrasound examination of the thyroid and neck, which revealed a 35 × 40 mm solid nodule within the left thyroid lobe. The left lobe was noted to be hyper-vascular with a surrounding fluid density collection. An outpatient Otolaryngology appointment was made.\nShe returned to the emergency department the following day with rapid onset tenderness, oedema and erythema over the thyroid. She became haemodynamically unstable with a white cell count of 14.9×109/l and a C-reactive protein of 301 mg/L and was admitted under endocrinology. She was treated with parenterally with 4.5 mg piperacillin/tazobactam three times daily and gentamicin as per trust microbiology advice.\nThe ultrasound of the thyroid mass was repeated revealing severe expansion of the left lobe with a predominant fluid component. Fine needle aspiration was performed and microbiological assessment again revealed no growth. Cytology however, was suspicious of malignancy. The cytology was repeated and confirmed the likely malignancy.\nDue to a lack of response a computed tomographic scan was performed of her neck (Figure ). A large enhancing soft tissue mass arising from the left lobe of the thyroid with an area of fluid inferolaterally displacing the surrounding structures was reported. The total size of the lesion including the fluid collection was 60 × 43 × 70 mm. Ipsilateral lymphadenopathy was seen in levels II, III and IV. Clinical staging was cT2N1b (according to the International Union Against Cancer staging). During her acute admission the thyroid abscess was incised and drained and a formal biopsy taken of the intra-thyroidal lesion. Systemic improvement was rapid and she completed a further 48 hours of parenteral co-amoxiclav prior to discharge. Histology revealed a papillary thyroid carcinoma (PTC) and a repeat CT for staging was performed (Figure ). The left common carotid artery and internal jugular vein were displaced and compressed, but there was no evidence of encasement. The mass extended into the sterno-thyroid and sterno-hyoid muscles and appeared inseparable but not invading. Maximal axial dimensions were 29 × 26 mm, with a maximal coronal dimension of 48 mm. The previously enlarged nodes had resolved.\nShe subsequently underwent a total thyroidectomy and bilateral level VI clearance. As the previous enlarged nodes had resolved, no other neck dissection was performed. Post-operatively she developed hypocalcaemia and received supplementation, which was reduced over the following 2 months. Histology revealed a 40 mm infiltrative unencapsulated classical PTC with extrathyroid invasion. One of 5 lymph nodes contained metastatic PTC. Her pathological staging was pT3N1a. Post operatively she received radioactive iodine ablation and has made a full recovery. |
A 70-year-old, right hand dominant, otherwise healthy and active, female presented to our clinic three (3) months after falling onto her left shoulder. She was initially treated elsewhere nonoperatively with sling immobilization and limited physical therapy. Upon presentation to us, she complained of persistent lateral shoulder pain and limited function in her left arm. Physical examination revealed her to be neurovascularly intact with no deltoid deficiency. She had limited active motion and painful passive motion associated with crepitation. Radiographs revealed a two-part, varus-angulated malunion with a large spike of bone protruding laterally, without evidence of AVN (). The natural history, prognosis, treatment options, potential complications, and expected outcomes for both operative and nonoperative management were reviewed with the patient. Using a shared-decision making process, she elected to proceed with surgical management.\nThe patient was taken to the operating room for an ORIF procedure using the PH Cage. The patient was positioned in a modified beach chair setup using a shoulder specific table and articulated arm holder for the procedure. C-arm fluoroscopy was positioned “over the top” of the patient to allow for both AP and lateral views using internal/external rotation of the arm during the procedure. An extended deltopectoral incision was used to expose the fracture. The axillary nerve was identified inferiorly and laterally as it wrapped around the humerus close to the fracture site. Since the fracture was well healed, a surgical osteotomy was required for mobilization and reduction of the fracture. A reduction jig, which resembles the contours of a locking plate, was used for initial fracture fixation. The reduction jig comes attached with an optional plate, which may be used per surgeons' discretion. Kirschner wires were used in conjunction with the reduction jig to obtain and maintain provisional reduction and fixation of the fracture. An 8 mm hole was drilled over a guide wire from the distal end of the reduction jig to approximately 5 mm below the articular surface of the head. The metaphyseal area below the head was then prepared using a tool specifically designed to break down the intramedullary cancellous bone without disrupting the subcortical bone. The PH Cage was then inserted in a retrograde manner, deployed, expanded, and then locked in position. The PH Cage is available in three different sizes: small, medium, and large. For this patient, a medium PH Cage was indicated.\nThe distal end of the PH Cage was locked to the plate using two 28 mm screws. At the proximal end, three screws were used to secure the fracture fragments to the PH Cage and the plate construct. One of these screws was a kickstand screw across the fracture line that was stabilized by the plate and the PH Cage on either side of the fracture. One additional screw was added outside of the plate to secure the greater tuberosity to the PH Cage construct. A titanium washer was added to that screw to buttress the screw head as well as to augment fixation of the rotator cuff. Intraop fluoroscopy confirmed adequate reduction and hardware position. The entire construct moved well as a unit under direct visualization and fluoroscopic control.\nPostoperatively, the patient was immobilized in a sling for 6 weeks. She was started on pendulum/Codman exercises on POD1 and formal physical therapy involving gentle passive motion at week 1. The rehab protocol was advanced to active motion after 6 weeks and strengthening after 12 weeks. There were no intraoperative or postoperative complications. Her most recent follow-up X-rays obtained at 6 months after operation revealed a well healed fracture with anatomical alignment and no hardware complications or AVN (). The PH Cage maintained the head and the screws in position, thus preventing varus collapse or intraarticular screw penetration. The patient exhibited excellent range of motion, strength, and function. She had no significant pain at last follow-up (). |
A 28-year-old male patient was admitted to the medical floor of a community hospital due to a painful erection of three days’ duration. He had not experienced this before. The erection was unprovoked and had occurred spontaneously. There was no sexual stimulation involved. He complained of pain in the legs and testicles and he was unable to urinate. The pain was described to the nursing staff as "severe", but no pain scales were used to measure the intensity. There was penile swelling and tenderness on examination. He denied any current illicit drug use, including cocaine and methamphetamine. The patient denied any perineal or penile trauma. He does not have a history of sickle cell anemia, thalassemia, or multiple myeloma. He was not using any medications for erectile dysfunction. While in the emergency room, the patient was afebrile and hemodynamically stable. The base of his penis was cleansed with iodine and anesthetized. The patient underwent aspiration and irrigation followed by intracorporeal phenylephrine injection to achieve detumescence. Approximately 100 mL of blood was removed. The patient tolerated well the procedure, and he noted that the penile pain improved immediately, but the penis remained erect. A Urologist was subsequently consulted by the attending physician. There was no documentation of necrosis, and his penis was no longer erect three hours later. There was no penile edema or ecchymosis observed. There was no further intervention recommended afterward. He reported to his outpatient therapist a week later that he has not experienced erectile dysfunction or recurrence of the priapism. He was scheduled to have a follow-up visit with the urologist who treated him.\nDuring the psychiatric consultation, the patient was calm and cooperative. He denied any depressive, manic, or psychotic symptoms. He was anxious because of his pain symptoms. There were no suicidal or homicidal thoughts. His psychiatric home medications, which included olanzapine, quetiapine, and sertraline, were held. The patient reported a prior psychiatric history and prior psychiatric hospitalization. The patient admitted to a history of mood swings, irritability, rage reactions, sleeping disturbances, racing thoughts, poor impulse control, and psychomotor agitation. He had been taking his home medications for five months. He was compliant with his medication regimen and treatment follow-ups. He was diagnosed with bipolar disorder after a psychiatric hospitalization the year before. He had not experienced any painful erections since his psychiatric medications were started. Because of a recent argument with his girlfriend, the patient experienced worsening irritability. His physician assistant started him on topiramate 50 mg twice a day. He took a week’s dose of topiramate when he noticed the painful erections. |
A 25-year-old pregnant woman was referred at 25 weeks’ gestation for fetal diagnosis of right lung agenesis and right eye hypoplasia, without clear evidence of cardiac malformations.\nA female neonate born at 37 weeks via cesarean delivery (birth weight 2101 g) showed craniofacial abnormalities postnatally, which suggested diagnosis of Goldenhar syndrome. Transthoracic echocardiography revealed abnormal drainage of the left pulmonary veins into the innominate vein, a large atrial septal defect, a small ventricular septal defect, and patent ductus arteriosus. Computed tomography confirmed right lung agenesis (Fig. A–D). Transthoracic echocardiography performed 2 days after birth revealed accelerated pulmonary venous flow between the vertical and the innominate vein. Progressive pulmonary venous obstruction necessitated surgical repair 3 days after birth.\nA median sternotomy was performed to expose the heart, which was rotated counterclockwise owing to right lung agenesis. Cardiopulmonary bypass was established with ascending aortic perfusion and direct bicaval venous drainage. The patent ductus arteriosus was ligated. We performed pulmonary artery venting via the left pulmonary artery, and cardiac arrest was achieved using antegrade cardioplegia infusion. The pulmonary venous chamber was well visualized after counterclockwise rotation of the heart, and we selected the posterior approach intraoperatively. The vertical vein was ligated at its confluence with the innominate vein and was divided and incised toward the pulmonary venous chamber. The corresponding posterior wall of the left atrium was incised longitudinally toward the left atrial appendage. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures (Fig. A–C). The atrial septal defect was directly closed. The aortic clamp time was 48 min. The left and right ventricular function was good with catecholamine support and nitric oxide administration. The heart was weaned off cardiopulmonary bypass at a systemic pressure of 58/35 mmHg, pulmonary pressure of 34/13 mmHg, and central venous pressure of 6 mmHg, followed by sternal closure. Echocardiography and computed tomography confirmed absence of pulmonary venous obstruction, 1 year postoperatively (Fig. A, B). |
A 29-year-old gentleman was admitted to our center with the provisional diagnosis of right nephrolithiasis with right pyelolithiasis with right gross hydronephrosis. He was planned for right-sided PCNL. Preoperative anesthetic evaluation did not reveal abnormal findings except for high total leucocyte count during routine urine examination which was normalized after 5-day course of inj. amikacin. There was no significant medical, surgical, family, and psychosocial history.\nIn the operation theater, anesthesia was induced with propofol and trachea was intubated with size 7.5 mm ID flexometallic endotracheal cuffed tube using vecuronium 7 mg as muscle relaxant. Maintenance was done by isoflurane, oxygen, and intermittent vecuronium top-ups. The initial intrathoracic pressure was 19 cm of H2O. Patient was kept on volume control mode with initial tidal volume of 8 ml/kg. Over the period of surgery of 2 h, a gradual increase in intrathoracic pressure from 19 cm of H2O to 30 cm of H2O was noted. It was ascertained intraoperatively that there was no pneumothorax by auscultation of chest and confirming the presence of breath sounds antero-posteriorly. Other potential causes of rise in intrathoracic pressure such as endotracheal tube displacement, kinking, and obstruction due to secretions were ruled out immediately. The large numbers of stones and their fragments required further surgical time and thus the initial decrease of tidal volume to 6 ml/kg was done to decrease the intrathoracic pressure. The rest of the intraoperative period was uneventful.\nWhen the patient was turned supine, it was noted that patient had a tense abdomen. Initial impression led us to believe that there was intraabdominal extravasation of fluid. Immediately eFAST was done in which the left and right thoracic views were done to exclude hemothorax and pneumothorax. Both right and left quadrants of the abdomen were scanned along with the pericardial and suprapubic scan. It was noted that there was significant fluid collection in the Morison’s pouch. Drainage of fluid was then accomplished in real-time ultrasound guidance. Subsequently, the distension of abdomen decreased. After the return of adequate muscle strength, spontaneous breathing, and normalization of intrathoracic pressure, trachea was extubated and patient was shifted to post-operative ward.\nIn the post-operative ward, patient was managed conservatively with analgesics, antibiotics, fluids, and diuretics. Vitals including abdominal girth were measured continuously until the patient was shifted to ward. A repeat ultrasonogram done by radiologist 2 days later showed fluid collection in Morrison’s pouch, left upper abdomen, and bilateral iliac fossa but the collection was smaller in volume. Patient subsequently improved with conservative management and was discharged. Patient’s condition had normalized in the follow-up visit a week later. |
A 14-month old boy presented with respiratory distress, wheeze and hypoxia. This was preceded by 24 h of coryza, fever and reduced feeding. He was admitted directly to intensive care, where he was diagnosed with pneumonia. Treatment was started with empirical antibiotics. He was intubated and ventilated for 4 days, after which he was extubated and stepped down to high dependency unit (HDU) on continuous positive airways pressure (CPAP) ventilation. He continued to have persistently increased work of breathing, persistent expiratory wheeze and symptoms and signs of air trapping.\nThough he did not progress to respiratory failure, weight gain and oxygenation was achieved only by initiation of Heated Humidified High Flow Nasal cannula therapy (HHHFNC).\nPolymerase chain reaction (PCR) testing of nasopharyngeal aspirates (NPAs) revealed the persistence of rhinovirus and adenovirus for 10 weeks in both NPAs and bronchoalveolar lavages (BALs), with parainfluenza type 3 found in just one NPA sample. Adenovirus was also found in blood by PCR testing. Rhinovirus is not normally tested for in blood samples and there was no validated assay available for this. One BAL and one urine sample were screened for cytomegalovirus (CMV) by PCR also, and found to be negative. One stool sample was tested and found to be negative for rotavirus and adenovirus (despite the persistence of adenovirus in the respiratory samples at this time).\nThere was no significant family history of any genetic diseases and the patient was born at term. Mum is a smoker but states that she did not smoke during her pregnancy. During pregnancy, intrauterine growth restriction was identified. At the time of admission his weight was < 0.4th centile, but this was increasing. His parents report poor feeding since birth and an increased work of breathing from 5 months old. He is known to the allergy services for severe eczema and faltering weight, and has been diagnosed with cow’s milk protein allergy. His eczema medications have included topical tacrolimus, moderately potent topical steroids and emollient therapy.\nHis other medical history includes two previous episodes of bronchiolitis, three and 1 month prior to this admission (including one overnight stay during a trip to Australia). Prior to admission, he had only received two courses of antibiotics in his life – once for an infected BCG vaccine site and once for an ear infection.\nDuring his inpatient stay, he had 2 further intercurrent bacterial respiratory infections, treated by broad spectrum antibiotics. Immunological investigations revealed an IgG and IgM hypogammaglobunemia but a normal IgA. He was up-to-date with his childhood immunisations prior to admission, but has had a poor response to tetanus and twice to haemophilus B, demonstrated by low IgG levels to both. He was also found to have near absent B-cells and a reduced number of T-cells, and prophylaxis for Pneumocystis jiroveci was commenced. HIV testing was negative on two occasions. We withheld any further live virus vaccinations, in view of his immunodeficiency (Table , Fig. ).\nAt this stage, a CT chest was performed (Fig. ). The findings (mosaic pattern attenuation of both lung fields with a combination of air trapping and oligaemia, more pronounced in expiratory images) were consistent with BO which was believed to be secondary to the persistent adenovirus rather than the rhinovirus infection. Reasons for this were mainly that rhinoviruses are predominantly upper respiratory tract infections, whereas AdVs can cause more systemic infections and AdV DNA was detected in the blood. No biopsy or histopathological investigation was performed in order to confirm BO.\nA flexible bronchoscopy showed structurally normal airways. Screening for tuberculosis was negative.\nAfter a multidisciplinary team discussion, it was decided to commence pulse methyl-prednisolone therapy (3 daily doses of 10 mg/kg given every month) and long-term azithromycin (10 mg/kg 3 doses every week) for management of BO. Prior to commencing the steroids, cidofovir was administered to control the adenovirus replication and reduce the dissemination of the adenovirus.\nCidofovir (5 mg/kg, intravenously, with probenecid) was given on fortnightly basis for 4 months, during which he his renal function was monitored weekly. All the patient’s renal function test results remained within our institutional laboratory’s normal ranges (sodium 133–146 mmol/L; potassium 3.5–5.0 mmol/L; urea 1.5–5.0 mmol/L; creatinine 15–31 μmol/L) throughout the treatment period with cidofovir, indicating good tolerance to the drug. Since commencing steroid therapy, it was possible to gradually wean him off his HHHFNC, and he has since been thriving well. He was discharged on supplemental oxygen, which was weaned off over the next 12 months.\nAn array comparative genomic hybridisation (CGH) revealed mosaic tetrasomy of short arm of chromosome 9. There are no reports yet of immunodeficiency in association with mosaic tetrasomy 9p, nor any specific vulnerability to any particular viral infections [], but has previously been associated with auto-immune conditions associated with IFN-1 dysregulation (predisposing to inflammatory myositis and lupus-like syndrome) [].\nA conclusive diagnosis of a specific immunodeficiency/immunomodulation is yet to be made in this case, as this profile does not fit any primary immune deficiency. Molecular genetic testing for mutations in 82 genes associated with primary immunodeficiency and gastrointestinal disorders (TIGER panel, Great Ormond Street Hospital, London) did not identify any clearly pathogenic variants. The patient is currently being given weekly supplemental immunoglobulins.\nMost recently, the child has now cleared the long-term rhinovirus and adenovirus infections, and has been weaned off steroids and long term oxygen therapy. His weight gain has been adequate on air, and he is still too young for spirometry testing. Ongoing problems include recent Pseudomonas aeruginosa infection, with a wet cough, which is likely due to the bronchiectasis component of the BO disease. |
A 72-year-old man was admitted to our institution for uncontrollable hemorrhage from the left groin. The patient was severely hypotensive (70/40 mmHg) presenting with loss of consciousness. Manual compression in the bleeding region was immediately applied while he was resuscitated, intubated, and transferred to the operating theater.\nThe patient had 12 months earlier been treated for an infiltrating squamous cell scrotal carcinoma with non-resectable regional lymph nodes metastasis (Pathological Stage C according to the Ray and Withmore classification) (), with surgical treatment of the primary tumor and was then submitted to fractionated adjuvant radiotherapy in two sessions (performed the first time bilaterally in the groin with technical opposing camps and on the scrotum with direct technique for a total of 50.40 Gy for 2 months, the second time only in the left groin with technical opposing camps for a total of 30.40 Gy). After the last session, the patient developed multiple skin lesions in the left groin and a complete, paraneoplastic, iliofemoral deep vein thrombosis with severe edema of the left leg.\nAfter resuscitation and transfer to the theater, an urgent endovascular approach was planned, as the left CFA was thought to be very frail with high risk of re-bleeding in case of an open surgical repair. A 6 Fr sheath was, therefore, placed in the left common iliac artery from the right groin with a cross-over maneuver and an angiogram was carried out revealing a ruptured left CFA with large extravasation of contrast medium despite manual pressure (). Hemostasis was then immediately achieved by inflating a 10 mm balloon (Advance balloon; Cook, Bloomington, IN, USA) in the external iliac artery. The subsequent angiography through the balloon catheter showed clearly the eroded CFA and the feasibility to repair it deploying a covered stent (). The 6 Fr sheath was then exchanged for a 12 Fr 45 cm sheath (Flexor sheath, Cook Bloomington, IN, USA) and a 10 mm × 4 cm Fluency stent graft (Bard Peripheral Vascular Inc., Tempe, AZ, USA) was advanced over a hydrophilic stiff guidewire and placed covering the erosion. After post-dilatation, completion angiography demonstrated a patent CFA without any leak (). The patient received 4 units of blood during the procedure and was transferred to the ICU where he recovered well within 24 h. Treatment with subcutaneous fondaparinux injections in a daily dose of 2.5 mg was commenced 48 h after this uncomplicated procedure. After 1 month the necrotic infected cavity was stable without recurrent bleeding (); a Duplex scan showed patency of the stent graft but no recanalization of the iliofemoral deep vein thrombosis. |
A 35-year-old male presented to the casualty 30 days after his first episode of seizure with pain in both shoulders and difficulty in movements. Patient had not taken any consultations with any doctor post the episode of seizure. Past history failed to shed any light as to the cause of the seizure with no history indicating earlier episodes involving head trauma, substance abuse/withdrawl or any pre-existing neurological cause. No previous episodes of shoulder dislocation were reported by the patient.\nOn initial evaluation, the patient complained of decreased bilateral shoulder function and motion. On examination, normal shoulder contour was lost, shoulder movement was restricted especially abduction with arm in attitude of external rotation and pain was elicited with movement. Diagnosis of bilateral anterior shoulder dislocation was made clinically. Distal motor, sensory and vascular function was preserved. X-rays showed bilateral anterior shoulder dislocations with displaced greater tuberosity fractures ().\nLeft side closed reduction was done under general anesthesia followed by 3 weeks of immobilization and intermittent physiotherapy (). Closed reduction of the right shoulder had been attempted but the shoulder was locked and attempts proved futile. During second sitting, open reduction was performed. Even after complete release, shoulder did not relocate, the gt was adhered to underlying bone. The gt was freed, repositioned and fixed with ethibond after repositioning, the joint was reduced, capsule repaired, joint was stable and was put in an immobilizer. Details of open reduction- Standard deltopectoral approach was used. Conjoint tendon was retracted. humeral head was seen to pierce through the subscapularis muscle. After subscapularis was released, still there had been difficulty in reduction due to capsular adhesions. After releasing the capsule, reduction was attempted again, but was found to be unstable. Greater tuberosity was malpostioned and was adhered to proximal part of humerus. Adhesions were released and greater tuberosity was brought back into position fixed with ethibond and joint was reduced. Capsullorhaphy was done, subscapularis repaired. Joint found stable and greater tuberosity was well reduced and in position under C arm. Patient was put in a shoulder immobilizer and continued for 3 weeks. For rehabilitation, gentle pendulum exercises were started post operatively, but the patient was non compliant and moved his shoulder joint inspite of instructions. Patient came for follow up after 3 weeks and X- ray showed partial displacement of greater tuberosity. However, for displaced greater tuberosity, we decided to continue rehabilitation and monitor progress. At 6 weeks follow up, patient had active assisted forward flexion of 110 degrees, active abduction of 60 degrees. At 12 weeks follow up active abduction was 150-160 degrees.\nWe report post-operative follow up of 3 weeks and 3 months wherein at the end of 3 weeks the side that underwent closed reduction had full abduction and the side that underwent open reduction had no wound complications, had started pendular movements and was under physiotherapist care for gradual mobilization (, ). At the end of 3 months, the side reduced closed had full abduction, no complaints of pain and the side reduced open had 150-160 degrees range of motion (, , ) with 3 month follow up X-ray also posted showing union of greater tuberosity (Figs. , ). |
A 30-year-old woman came to our clinic for FNAC examination of the thyroid following-up her history of Hashimoto’s thyroiditis. She has been diagnosed with Hashimoto’s thyroiditis 3 years prior, and has been compliant with her scheduled appointments in the clinic. Cytologic evaluation of the thyroid with ultrasound -guided fine needle aspiration from multiple sites revealed normal thyrocytes and presence of macrophages that fell into the Bethesda II category according to WHO (). Thyrocytes were arranged in crowded three dimensional groups and in microfollicular pattern accompanied by a background of sparse macrophages, lymphocytes and stromal elements.\nFour months later she presented to the clinic with discomfort and swelling on the right side of her neck that has been growing for several weeks.\nPhysical examination resulted in a non-tender palpable mass. She was clinically euthyroid. Ultrasound of the thyroid () showed diffuse heterogeneous parenchyma with focal glandular enlargement presenting as hypoechoic nodules varying in size in a background of hyper vascularized thyroid gland. Fine echogenic fibrous septa create a pseudo-lobulated appearance. CT-scan of the neck showed enlarged lymph node with poorly defined nodal margins, classic of extracapsular nodal spread, suggesting metastatic disease. Consequently, the patient underwent surgery of the neck with complete removal of the mass.\nMicroscopic examination () of the mass showed presence of neoplastic cells within the lymph node parenchyma, with extracapsular invasion. The neoplastic cells were arranged in finger-like patterns with central fibrovascular core creating true papilla ( and 5). A diagnosis of metastatic involvement of the lymph node originating papillary carcinoma of the thyroid was made.\nOccult papillary carcinoma was firstly suspected in this patient. Hence, the patient underwent total thyroidectomy with partial neck dissection. Histopathologic evaluation of the continuous sections of the thyroid, demonstrated benign thyroid tissue in a background of Hashimoto’s thyroiditis. The tissues around the thyroid resulted in carcinomatous infiltration, and the two lymph nodes removed were positive for metastatic papillary carcinoma of the thyroid, but there was no presence of malignant cells in the thyroid gland which was entirely sectioned and reviewed, for microcarcinoma foci.\nA diagnosis of ectopic thyroid papillary carcinoma with metastatic disease in bilateral cervical lymph nodes was made. |
A 27-year-old female patient presented with a history of swelling in mandibular left posterior region for 1 year and pain for 1 month []. Intraoral examination revealed a bony hard swelling extending from the left mandibular second premolar till retromolar area. Obliteration of buccal and lingual vestibule was noted. The mucosa overlying the lesion appeared normal. The patient had undergone extraction of mandibular first molar of the same side earlier.\nThe radiographic examination revealed a multilocular radiolucency involving left mandibular region [Figure and ]. The lesion extended from the second premolar and posteriorly it involved the coronoid and the condylar processes. The expansion of buccal and lingual plate of the mandible was evident on occlusal radiographic images. Differential diagnoses of ameloblastoma and keratocystic odontogenic tumor were considered. Incisional biopsy confirmed the diagnosis of follicular ameloblastoma. Based on the histopathological diagnosis, segmental block resection was carried out with the surgical margin 1 cm away from the radiographic boundary of the lesion. The excised specimen was sent for histopathological diagnosis. No evidence of recurrence was noted after 1 year of resection.\nThe gross specimen showed expansion of buccal and lingual plates of mandible and bone erosion in the retromolar area. On grossing, a large cystic lesion was noted involving body and ramus of mandible. Microscopic examination of the excised tissue specimen revealed follicles of ameloblastoma of varying sizes []. The stroma was mature, collagenous and it demonstrated foreign body granulomas with numerous multinucleated giant cells [Figure and ]. On careful examination, it was evident that these foreign body granulomas were associated with multiple, amorphous, eosinophilic masses enclosed in densely hyalinized eosinophilic matrix/rings [Figure and ].\nHistochemical staining was done to understand the nature of hyaline rings. The hyaline rings were periodic acid–Schiff positive []. The peripheral portion of the foreign body was positive for Masson's trichrome stain suggesting it to be condensation of collagen.\nTo prove the similarity of the foreign body and plant cells, grains such as gram, wheat, rice, split pigeon pea were boiled and processed. Moreover, vegetables such as carrot, cabbage were processed raw. The sections were stained with hematoxylin and eosin and periodic acid-Schiff. We observed that a section of a legume [] bore a striking resemblance, to the foreign body we encountered. Both the legume and the foreign body showed peripherally smaller angular to rectangular cells and centrally larger cells enclosing amorphous substance. When viewed under polarized light, the hyaline structures exhibited birefringence [Figure and ] and the fragments of material similar to hyaline rings were noted within the giant cells [Figure and ].\nBased on these observations, the foreign bodies were identified as remnants of food particles of vegetable origin. To rule out other local and systemic granulomatosis, hematological investigations were done, and the results were within normal limits. Tuberculosis was ruled out by normal findings of chest radiography and negative tuberculin test. Hence, the lesion was diagnosed as follicular ameloblastoma with PG. Patient's consent has been obtained for publishing clinical findings and images. |
A 35-year-old Greek female patient, gravida four and para two, presented to our breast unit department due to a non-palpable breast lesion which was revealed via ultrasonic examination, with malignant ultrasonographical features. Her personal medical history and her psychosocial history were uneventful and Pap Smear tests were up to date and all negative. She was non-smoker and consumed alcohol only in social occasions. Her body mass index (BMI) was 30, 48 Kg/m []. Because of her mother’s breast cancer history (diagnosed with breast cancer at the age of 50, but never tested for BRCA), the patient was followed up via transvaginal ultrasonography and breast ultrasonic examination every six months since 2004.\nUpon arrival, physical examination of her breasts did not reveal any palpable mass. Breast ultrasonography showed a hypoechoic lesion of 0,9 × 0,8 cm located in the lower inner quadrant, while breast magnetic resonance imaging confirmed the suspicious and possible malignant finding on her right breast (MRM BIRADS IV) (). The chest x-ray test was normal. Her laboratory workup was all normal.\nUnder general anesthesia, a total excision of the lesion (which had been marked via a hook wire) was performed by a gynecologist specialized in breast surgery with 6 years’ experience in breast surgical procedures. The normal tissue of the breast, the nipple and the areola were conserved. The excised mass was almost 1 cm in diameter. The contralateral breast was normal. Ultrasound guided biopsy was not performed because BIRADS IV was overestimated (not necessarily malignancy). There were no peri-operative complications and the breast healed well. Post-operatively, the patient was followed up in the breast ward. She was administered antibiotics, fluids and painkillers intravenously. Histology confirmed the ultrasonic diagnosis, revealing a central low grade invasive ductal carcinoma and a peripheral in situ ductal breast carcinoma grade III (). The margins of the resected surgical specimen were negative for cancer cells.\nAfter almost a month from the first operation, the patient underwent a new surgical excision of unilateral right axillary lymphadenectomy of the first and second lymph nodes level. Twenty-two excised axillary lymph nodes were negative for metastasizing breast disease (0/22). Immunohistochemical analysis in cellular level showed ER (clone 6F11) stain positive in 100% of tumor cells, PR (clone 636) stain positive in 2% of tumor cells, Ki-67 (clone M1B1) stain positive in 60% of tumor cells and c-erb-2 (clone CB11)/HER 2 showing 3+ positive for cancer. Subsequently, the patient underwent treatment via chemotherapy, radiotherapy, herceptin and hormone therapy for the invasive ductal carcinoma Grade III (TNM staging: T1N0MO).\nGene evaluation for genetic mutations showed a BRCA 1 mutation; gene BRCA1 analysis was positive for mutations predisposing for breast or ovarian malignancy. More specifically the mutation p.Gly1738Arg (HGVS nomenclature)/ G1738R (BIC nomenclature) was detected. Thus, a prophylactic bilateral mastectomy was performed and followed by a successful plastic reconstructive surgery done by an experienced plastic surgeon, allowing for optimal aesthetic results.\nA year after the first operation, the patient underwent a prophylactic laparoscopic bilateral salpingoophorectomy and uterine diagnostic curettage at the age of 37. Before surgery, blood tests including tumor markers were all in normal levels (CEA: 1,3 ng/ml, CA15-3: 6U/ml, CA125: 8U/ml, CA19-9: 30 U/ml, AFP: 1,2 IU/ml). The chest x-ray test was normal and the preoperative magnetic resonance imaging of the upper and lower abdomen did not detect any pathological finding. The histological diagnosis of the surgical specimens was suggestive of a unilateral invasive high grade salpingeal cancer, mostly intraepithelial and minimal invasive (0, 1 cm) serous carcinoma of one salpinx. Immunochemical analysis was positive for P53 and Ki67 (). No other pathologic finding was detected.\nA further surgical evaluation was decided by the oncology council. Thus, the patient underwent an open total abdominal hysterectomy, omentectomy and bilateral pelvic lymphadenectomy by a gynecologist specialized in gynecologic oncology under general anesthesia. Postoperatively, the patient was followed up in a gynecology ward. She was administered antibiotics, fluids and painkillers intravenously. After a hospitalization of 5 days, she was discharged on the 6th postoperative day, in good condition. She was prescribed tinzaparin (u) for 8 days and cefuroxime (u) peros for 7 days. The patient was compliant with the therapeutic program which was well tolerated with no significant side effects. Histology was totally normal without any other pathological findings. Bone scanning was normal and did not reveal areas of increased radionucleide uptake. Similarly, the hip and pelvis x-ray were normal.\nThe patient is in excellent clinical condition and she is followed up via ultrasonography of upper and lower abdomen and breast magnetic resonance imaging every 6 months, without any pathological finding after the last surgery. |
The patient is currently a 93-year-old female who was first referred to us at age 64 because of edema involving both legs to the thighs and above, and 12 g of proteinuria in 24 h. Her renal function was within normal limits. The creatinine clearance was 80 mL/min, her BUN was 14 mg/dL, and her serum creatinine was 0.9 mg/dL. Her serum albumin was 1.1 mg/dL. Her total serum cholesterol was greater than 300 mg/dL. Repeat 24-h proteinuria was 13 g. A renal biopsy was classic for membranous nephropathy by light, immuno-, and electron microscopy criteria. No malignancies were discovered, and tests for systemic lupus and other collagen diseases were negative. She was seronegative for human immunodeficiency virus and hepatitis B and C. She had never received gold salts, nor did she take nonsteroidal anti-inflammatory drugs. Thus, she had idiopathic membranous nephropathy by clinical criteria.\nA conservative trial of furosemide and an angiotensin enzyme inhibitor was attempted with no reduction in her proteinuria. She became orthostatic when the dose of furosemide was increased in an attempt to relieve her symptoms of leg stiffness and difficulty with ambulation. She remained weak and relatively immobilized.\nAfter 4 months of the above treatment, she was treated with the steroid and chlorambucil protocol described by Ponticelli et al. [, ]. There was no response for 2 months, but her serum albumin began to rise to 2 mg/dL by month 3. Her 24-h albumin excretion continued to be 10 mg/24 h. By month 4, her albuminuria was no longer present. Her 24-h protein excretion had fallen to less than 100 mg. She completed the full 6-month course and remained in complete remission for 5 years. There was no proteinuria, and all blood chemistries remained within normal limits.\nFive years after her complete remission, the nephrotic syndrome recurred with edema, proteinuria, hyperlipidemia, etc. The therapy was repeated for the 6-month course of alternating prednisone and chlorambucil exactly as administered previously, but there was no response. Two months later, a course of intravenous immunoglobulin [] was attempted with a 2-month induction period and 4 months of infusions every 3 weeks with no effect on her proteinuria. This protocol was terminated at the patient's request because she had lost patience.\nShe was then treated with 20 mg of prednisone on alternate days plus cyclosporine 4 mg/kg daily []. Within 3 months, she was in complete remission. Her dose of prednisone was tapered to 0 by 6 months, and her dose of cyclosporine was reduced to 25 mg daily.\nShe remained in remission for another 7 years, but her nephrotic syndrome again recurred. The prednisone/cyclosporine regimen was repeated for 6 months with no response. She was then treated with alternate-day prednisone as before plus mycophenolate 500 mg twice daily []. Once again, she had a complete remission after 3 months.\nThe steroids were tapered to 0 after 6 months, and the mycophenolate was continued at 125 mg twice daily. She remained in complete remission until 7 years later when she developed cytomegalovirus colitis that remitted upon discontinuation of the mycophenolate and treatment with ganciclovir. She remains in complete remission 10 years later on no treatment. |
A 29-year-old female patient with end stage renal failure was our recipient. She was considered for a pre-emptive transplant and the only viable living donor was her 59-year-old mother. Upon evaluation for the donor, all the criteria were met except for the detected horseshoe kidney by CT scan. Dynamic CT revealed one renal artery and one renal vein for each side, a long but thin isthmus connecting lower poles with no visible arterial supply (). Sizes of the left and right kidneys were 110 × 38 cm and 117 × 42 cm, respectively. Absence of a connecting caliceal system was established with a descending urography. We did not perform a dynamic renal perfusion scintigraphy to assess the split functions of the kidneys since their sizes, respective vasculature and measured GFR values indicated sufficient capacity for each kidney. Left portion of the kidney was considered for nephrectomy.\nWith a left subcostal incision transperitoneal exploration was performed. The left kidney had indeed one renal artery and one renal vein. There were two ureters for the left kidney. The vessels, ureters were prepared and the kidney was mobilized up to the isthmus. A temporary bulldog clamp was put on left renal artery to determine the demarcation zone and it was shown to form around isthmus (). The artery and vein were divided and the isthmus separation was performed with a 60-mm Endo GIA Black stapler (Medtronic, USA) (). After separation, no bleeding and no urine leakage were detected from transacted surface of the remaining kidney.\nThe graft was perfused with cold lactated ringer–prilocaine–heparin solution. There were no visible leaks (). It was transplanted to right iliac fossa of the recipient. Total cold ischemia duration was 21 min. Ureters were anastomosed to bladder separately side by side. Patient received tacrolimus, mycofenolic acid and methylprednisolone treatment. No collection was formed on the postoperative period, drain creatinine sampling showed no leakage and she was discharged on the 5th day with a serum creatinine level of 0.85 mg/dl. 6 months follow up showed a stable creatinine levels between 0.75 and 0.96 mg/dl.\nNo postoperative complications were observed in donor. Drain was taken out on the 3rd day after creatinine sampling and she was discharged on the 4th day. Her creatinine levels were stable during postoperative 1st, 3rd and 6th months follow-up 0.82, 0.86 and 0.85 mg/dl respectively. Her blood pressure levels, urine output, creatinine levels and GFR values showed a stable and sufficient renal capacity with remaining right kidney. |
E. G. is a fifty-year-old, black female with a past history of Crohn's disease, diagnosed at the age of 19 with subsequent intestinal resection at the age of 21, and a history of ischemic stroke who presented with left sided weakness, mild aphasia, and mild dysarthria (NIHSS = 3) outside the 4.5-hour window for treatment with tPA. At that time she was taking aspirin for stroke prevention and prednisone for a Crohn's disease flare (last flare occurred one month ago). MRI was done and showed right cerebellar and bilateral occipital acute ischemic strokes. MRA revealed a hypoplastic left vertebral artery, but no other vascular abnormalities. Transesophageal echocardiography did not reveal an embolic source. Hypercoagulation panel showed high normal levels of Factor VIII and vWF, but all other tests were normal. ESR and CRP were not elevated which makes it unlikely to have a concomitant Crohn's flare. She was discharged home with NIHSS = 1 on aspirin and clopidogrel for stroke prevention and prednisone for her Crohn's disease.\nTen days after discharge, the patient presented with partial visual field cut, nasolabial flattening, right leg ataxia, and decreased sensation on the left leg and arm (NIHSS = 4). She was not treated with tPA because of her recent stroke. MRI was done and showed additional areas of right occipital acute ischemia. MRA of the brain remained unremarkable. Hypercoagulation panel was ordered and revealed an elevation in Factor VIII; vWF level was not measured at this time. ESR level was normal; however, CRP levels were elevated which may, in addition to a recent episode of loose stool, suggest a flare of her Crohn's disease. She reported episodes of palpitations and lightheadedness, concerning paroxysmal atrial fibrillation; therefore, she was placed on warfarin. She was discharged home with NIHSS = 1.\nTwo months later, the patient presented again with slurred speech (NIHSS = 5) outside the 4.5-hour window for treatment with tPA. Her INR was subtherapeutic 1.3 on admission. MRI was done and showed right frontal and right caudate acute ischemic stroke. Transesophageal echocardiography did not reveal an embolic source. Hypercoagulation panel was ordered and revealed an elevation in Factor VIII; vWF was not ordered at this time. ESR and CRP were not elevated at this presentation. She was discharged home with NIHSS = 3 on warfarin for stroke prevention and prednisone for her Crohn's disease.\nShe visited our stroke clinic 2 and then 8 months later for follow-up. She had no new neurological symptoms or Crohn's disease flares during this period. She continued to receive warfarin and prednisone for stroke and Crohn's disease prevention, respectively. |
A 48-year-old female patient presented with history of repetitive intrusive thoughts of contamination with dirt along with compulsive behavior of washing for over 18 years. She was diagnosed with OCD and treated with various SSRIs, including escitalopram, fluoxetine, and clomipramine in adequate doses for adequate duration. However, she showed limited improvement with these drugs. In year 2008, she was started on paroxetine and dose increased to 75 mg over 2 months and showed significant improvement in her symptom. But over these 2 months, she started experiencing discomfort and engorgement in her breasts. This continued for 2-3 weeks and then she noticed whitish milky discharge from both nipples. Patient was extensively evaluated for the galactorrhea, and her serum prolactin levels were found to be raised (89 ng/ml) and the rest of her investigations (magnetic resonance imaging [MRI] brain, follicle-stimulating hormone, luteinizing hormone, dehydroepiandrosterone-sulfate) were found to be within normal limits. She had not been using any other drug during this period, so the possibility of increased prolactin due to other drugs was ruled out. Local breast examination by the gynecologist did not reveal any pathology. Thereafter, she was lost to follow up and she discontinued paroxetine due to galactorrhea. The discharge stopped over next 7-10 days. Over next 3 years, she would start taking paroxetine on her own whenever her symptoms would exacerbate, but she would take dose up to 25 mg as thereafter she started having breast engorgement and discomfort after approximately 2 months of starting taking medication and did not increase the dose any further. During this period, she had three episodes of discharge of whitish milky fluid from breasts and discharge would stop within 10 days of discontinuing paroxetine. However, her prolactin levels were within normal limits during these three episodes. Last time, she presented to us, she was taking paroxetine 25 mg for 3 months duration and had started experiencing breast engorgement and discharge for last 2 weeks. On evaluation, serum prolactin levels were raised (129 ng/ml), and other hormonal investigations were normal. There was no pathology detected on local and systemic examination. MRI Brain was repeated, which revealed a hyperintense signal on T2 in the pituitary gland, which measured 1.7 mm × 1.5 mm × 1.2 mm. Thereafter, case was evaluated by an endocrinologist and diagnosis of “drug-induced hyperprolactinemia with incidentaloma” was made because patient's reporting of symptoms suggestive of galactorrhea were temporally related to treatment with paroxetine, and she did not have continuously raised prolactin levels, so raised prolactin levels due to the pituitary mass was unlikely. |
A 27-year-old healthy male patient presented to the ED with a history of a 3-meter fall during work in which he landed on his feet. Initial clinical examination of the lower extremities at 13:00 showed intact skin and significant right proximal leg deformity associated with swelling and tenderness with a VAS of 3-4/10. Vascular evaluation of distal pulses revealed that the posterior tibial artery pulse was not palpable. The capillary perfusion was less than 2 seconds. The foot was sensate. Initial assessment also revealed swollen but soft compartments; however, the passive stretch test was negative and did not indicate an ACS. Radiographs showed a severely comminuted tibial plateau fracture (Figure ). Realignment of the tibial plateau fracture was performed under procedural sedation and an above-knee backslab was applied, followed by reassessment of distal pulses, which showed Doppler-able but weak pulses of the posterior tibial and dorsalis pedis arteries (Figure ). Due to the severity of the injury, its natural association with vascular injuries, and the inability to palpate the posterior tibial artery pulses on initial assessment, computed tomography angiography (CTA) was performed, which revealed attenuation of the popliteal artery with patent superficial femoral and anterior and posterior tibial arteries (Figure ). The findings were communicated and thoroughly discussed with the on-call vascular surgeon. He concluded that attenuation was not an injury but a compression caused by the posterior tibial plateau and required only serial distal neurovascular assessment. The patient received a dose of fentanyl (50 mg) upon arrival, followed by morphine (5 mg) two hours later, and was then intravenously administered regular doses of acetaminophen (1 g).\nThe patient was scheduled for application of a knee-spanning external fixator; however, this was delayed due to two life-saving procedures occupying the operating rooms. Re-evaluation of the compartments in the holding bay at 20:00 revealed quite tense compartments, especially the anterior compartment, but the passive stretch test was negative. Therefore, the patient was consented for a spanning external fixator application with/without fasciotomy of the compartments, depending on the intraoperative compartment pressure measurements. The patient was placed in the supine position under general anesthesia, and a spanning external fixator of the tibial plateau fracture was applied under image guidance (Figure ). Compartment pressure testing using the same method revealed a pressure of 66 mmHg within the posterior deep compartment, 54 mmHg in the posterior superficial compartment, 69 mmHg in the anterior compartment, and 45 mmHg in the lateral compartment [,]. Therefore, the decision was made to proceed with a decompressive fasciotomy using dual incisions to decompress all four compartments of the leg. Not surprisingly, the anterior compartment muscles significantly escaped when decompressed and were partially dusky with reduced ability to bleed when tested. Irrigation and debridement were performed. The fasciotomy wounds were approximated by the rubber band lace-up technique to keep the skin partially approximated but tension-free. Postoperative examination revealed a perfused limb with capillary refill of less than 2 seconds, and an intact neurological examination; however, distal pulses were not palpable and could not be detected by a hand-held Doppler device. Thus, an urgent CTA was arranged to reveal the patent arteries.\nTwo days later, the patient underwent irrigation, debridement, and wound closure under spinal anesthesia. Finally, the patient underwent open reduction internal fixation with bone graft and plate and screw fixation of the right tibial plateau fracture in the following week (Figure ). The patient was discharged with a right below-knee backslab for two weeks, after which it was removed and placed on a hinged brace. At two weeks, the wounds were inspected, and the stitches were removed. At four months, the patient was re-evaluated (Figure ). He was pain-free, with healed scars, and a comparable range of motion of the injured knee and ankle joint to the normal contralateral side, without any neurological symptoms. He had mild limitations during moderate-intensity activities. |
A 72-year-old lady was admitted for elective trans-catheter aortic valve replacement (TAVR). Her postoperative course was complicated by hemopericardium that progressed to pulseless electrical activity. As part of resuscitation, she underwent emergent pericardiocentesis and was placed on VA ECMO and intra-aortic balloon pump (IABP) for cardiogenic shock. Over the following two days, she was recognized by the primary cardiology service to have a poor neurological examination including nonreactive pupils and not triggering the ventilator. Her underlying heart rhythm remained pulseless electrical activity. Neurology was consulted for prognostication after presumed anoxic brain injury. Initial evaluation was performed off sedation at body temperature of 36.4C. Mean arterial pressure was sustained above 70 mmHg by VA ECMO, IABP, and norepinephrine with no severe electrolyte or metabolic derangement. There were no fluctuations in heart rate with noxious stimulation. Her pupils were 4 mm and nonreactive bilaterally. She had no corneal reflex. Her eyes remained midline during oculocephalic and oculovestibular testing. She had no cough or gag. She had no evidence of spontaneously initiated breaths. All four limbs were atonic with no withdrawal to painful stimuli. Deep tendon reflexes and plantar responses were absent bilaterally. Given the complexity of cardiopulmonary instrumentation and monitoring, CT head could not be safely obtained. We used bedside TCD to evaluate for cerebral circulatory arrest to support the clinical impression of an irreversible brain injury. The TCD and repeated TCD showed isolated systolic spikes in the anterior and posterior circulation in all insonated vessels consistent with cerebral circulatory arrest (Figure ).\nReversal of systolic spikes in the middle cerebral artery was likely oscillatory waveform from the artificial circulatory support provided by the combination of VA ECMO and IABP. After the clinical examination and TCD, apnea testing was then performed. She was on pressure control mechanical ventilation. The Fraction of inspired oxygen (FiO2) was increased to 100% to preoxygenate. Arterial blood gases (ABG) showed PaO2 of 236 mm of Hg and PaCO2 of 39 mm of Hg. She was then disconnected from the ventilator. A cannula with 4 liters of O2 flow was placed inside the endotracheal tube at the level of the carina. The sweep rate on the VA ECMO was concomitantly reduced to 800cc and monitored with arterial line and pulse oximetry. O2 saturation was sustained at 96 to 100% throughout apnea testing with no change in systolic blood pressure which was supported with vasopressors, VA ECMO, and IABP. After approximately 12 minutes, ABG showed PaCO2 of 68 mm of Hg. She was declared brain dead. |
A five-year-old typically developing female was referred by her primary care provider for evaluation of a rash reported to be sensitive to sunlight and had been reoccurring for the last 4 years. Cutaneous exam revealed fine scale on her trunk and extremities as well as small pink flat papules on lower left leg. She was diagnosed with ichthyosis vulgaris and provided with a treatment plan. She was subsequently referred to gastroenterology for decreased appetite. Two weeks later the child presented to the Emergency Department for right knee pain. Her mother reported her daughter had experienced bilateral lower extremity pain and had been limping. During the evaluation she was able to walk for the physician without reporting pain and no tenderness was reported with palpation. The mother reported her daughter was bitten by a tick about 1 year ago and that no workup or treatment was conducted. Radiographic scans of the right knee and leg showed no abnormalities. Laboratory testing for Lyme disease was conducted and found to be negative. She was discharged with the diagnosis of tenosynovitis. The child presented again to the Emergency Department the following week for left knee and ankle pain. She reported pain in her left calcaneus when asked to bear weight. Migratory arthralgia was noted and further laboratory testing, including a vitamin C level, was ordered. Her vitamin C level was found to be < 5 umol/L (reference range: 23 to 114 umol/L). Her prealbumin level was also low, 14 mg/dl (reference range: 17–36 mg/dl) as was both her ferritin 3.9 ng/ml (reference range: 6.2–137.0 ng/ml) and her iron saturation 15% (reference range: 20–55%). Both vitamin A and vitamin D were found to be within the normal reference range. With the diagnosis of the vitamin C deficiency, the child was started on ascorbic acid and referred to the feeding program.\nThe participant was the youngest of six children and raised by her biological parents in a middle-class household in a small town. She was born full term by caesarian section with a birth weight of 3.88 kg. There were no prenatal or postnatal complications. No delays in development were noted by the primary care provider or parents. She scooted at 6 months and walked before 1 year. No problems were noted with her gait until several weeks prior to the initial visit to the Emergency Department for knee pain.\nAt 1 year of age, she transitioned from infant formula to milk and cereal snacks. She never accepted baby food. For 4 years after transitioning off infant formula her diet consisted of cereal snacks, one type of cracker, and, inconsistently, chocolate pudding, vanilla ice cream, chocolate, and banana. Except for the occasional banana, she never ate fruit, vegetables, or meats. She drank skim milk, water, and, rarely, soda. She refused to taste new foods or drinks. Additionally, at the time of her diagnosis with vitamin C deficiency, her body mass index was at the 1st percentile. Based upon her inadequate growth and extremely limited diet, she was admitted to an intensive day treatment feeding program. During the course of intensive treatment she learned to eat 29 foods from all food groups through the use of an intervention involving gradual repeated exposure to novel foods []. In 6 months after intensive treatment, her weight had increased by four kilograms, her height increased by three centimeters, and her body mass index reached the 61st percentile. At 1 year after completion of intensive treatment, her height had increased by 7.6 cm and her weight had increased by seven kilograms Her body mass index reached the 85th percentile (see Fig. ). Across the span of the 1 year after intensive treatment she continued to be monitored as an outpatient by a feeding therapist who continued to address the child’s diet variety and helped the family maintain the gains made during intensive treatment. At all outpatient appointments, a meal was conducted allowing the therapist to verify the child’s consumption of a variety of foods.\nHow unusual was this case?\nTo determine how this case compared to the existing literature, articles describing cases of scurvy secondary to diet limitations published from 2000 to 2018 were reviewed. PubMed and Google Scholar were searched using the search terms, “scurvy” and “ascorbic acid deficiency”. The reference section and citation listing of each article identified from these searches were then examined to identify additional articles. Sixty-one articles describing either a case study or a case series reported on 77 children diagnosed with scurvy as a result of diet insufficiencies (see Additional file for an alphabetical listing of all studies). Selected demographics from these studies are shown in Table . While the child in the current study was only slightly younger than median age as the children in the existing literature, she differed from most of these children who were predominantly males with special needs, most often, autism spectrum disorder. Of the 77 reported cases, only six were females without special needs.\nWhile all 77 children reported in these studies had limited diets, it is not clear that all could be described as selective eaters or to what degree their diets were the result of refusal to eat a variety of foods. Six of the children exhibited oral motor dysfunction which limited the range of foods they could eat [, ]. While some, or possibly all, of these six children might have obtained sufficient nutrition by consuming lower texture foods, oral supplement, or multi-vitamins, it is probable their parents did not know how to modify their diets to match their oral motor limitations. There were also three children dependent upon tube feeds who either received an insufficient amount of enteral formula [] or received homemade tube feeds deficient in vitamin C []. There were other children whose diets were not adjusted to meet their specific health needs, for example, one child receiving a ketogenic diet was not receiving any vitamin supplementation [] and another child received only a limited diet due to multiple food allergies []. For various reasons, the parents of 18 children, (five children with typical development, 14 children with special needs other than autism) limited their diets to the point these children developed vitamin C deficiency. Of the nine children with typical development with parent-limited diets, all but two were less than 2 years of age.\nFifty-nine of the children described in the existing literature could be described as selective eaters whose scurvy resulted from their limited intake. Of these children, 41% had autism spectrum disorder, 31% had intellectual disabilities, and 86% were male. Four of these 59 children were females with typical development like the girl in the current study. The child in the current case study had a diet similar to the diets of these 59 children identified as selective eaters in the existing literature on vitamin C deficiency. None of these children reportedly consumed vegetables or fruits, most consumed only starches and dairy products, with a few eating a limited number of proteins. The child in our case study was anemic, as were 42% of the children in the existing literature, an expected finding given the role of vitamin C in iron absorption. While the child in our case study did not exhibit signs or symptoms indicative of additional nutrient deficiencies, other deficiencies were considered likely so she was placed on a multivitamin within a day of the initial diagnosis of vitamin C deficiency. Her pediatrician conducted further testing and found her vitamin A and vitamin D levels were within the normal range. Of the 59 children described in the existing literature, 22% were identified with an additional nutrient deficiency beyond anemia (e.g. vitamin A, vitamin D). Given the diets reported for these 59 children, it is possible, if not probable, that more of these children had other nutrient deficiencies, but further deficiencies were either not reported or not identified.\nThe girl described in our case study had a body mass index at the 1st percentile prior to her feeding treatment. Underweight was also a common problem among the 59 children in the existing literature, with 32% being described as underweight. It is not surprising so many of these children were underweight. Many of children had anemia, which decreases appetite and eating was no doubt uncomfortable or even painful for many of these children, 71% of whom exhibited gingival symptoms. |
A 21-year-old woman was consulted in February 2015 for bleeding gingival enlargement evolving for 12 months. She complained of esthetics, discomfort, and difficulties of plaque control. According to medical history, the patient had received a kidney transplantation 2 years earlier (2013). She has been administrating a daily immune suppressor treatment based on cyclosporin A 125 mg, prednisolone 5 mg, and mycophenolate mofetil 500 mg per day as a prophylaxis against organ transplant rejection.\nThe patient had a very poor oral plaque control; the plaque index PI [] and gingival index GI scores [] were high which were, respectively, 2 and 2.75.\nThe clinical examination revealed an erythematous, edematous gingival overgrowth localized at the buccal and lingual side of the anterior teeth. The gingival overgrowth appeared as localized nodular enlargement of the interdental papilla (Figures –).\nThe amount of the gingival overgrowth was obtained according to the GO score of Seymour et al. [].\nA GO score was assigned to each buccal and lingual interdental papilla (gingival unit) of the six anterior upper and lower teeth. Then the sum of the horizontal and the vertical enlargement components was made.\nThe first component measured the degree of gingival thickening (horizontal enlargement) labially and lingually by means of a three-point scale (0 = normal width, 1 = thickening up to 2 mm, and 2 = thickening of more than 2 mm). The second component measured the extent of encroachment (vertical enlargement) of the gingival tissues on the labial and lingual aspects of adjacent tooth crown; it ranged from 0 to 3 (from no clinical evidence of overgrowth to an overgrowth covering three-fourths of the tooth crown). Likewise, a total of 20 papillae are examined, presenting a potential maximum GO score of 100, which could be expressed as a percentage [].\nThe gingival overgrowth is considered as clinically significant if the GO score is ≥30% [].\nIn the present case report, the GO score was 30.5%, so that it was classified as clinically significant gingival overgrowth.\nA suitable probing revealed deep pockets with negative recessions, due to the gingival overgrowth (indicating coverage of clinical crowns ≥ 2 mm). Underlying calculus was localized mainly at the anterior teeth. The pocket values and clinical attachment loss varied from 5 to 7 mm and from 2 to 3 mm, respectively.\nX-ray examination showed a marginal (coronal third) horizontal alveolar bone loss which was more pronounced at the lower incisors (). So the patient had a periodontitis beside the gingival enlargement.\nThe final diagnosis was CsA-induced gingival overgrowth with underlying localized moderate periodontitis stage II grade B. The periodontitis was classified according to the new classification system of periodontal diseases and conditions from the American Academy of Periodontology and the European Federation of Periodontology 2018 [] (Tables and ).\nThe management strategy consisted of a nonsurgical periodontal therapy based, initially, on oral hygiene instruction. On the second-time round, a full-mouth scaling and root planning were performed a week later as well as polishing of all the rough dental surfaces. Extraction of the remaining root of tooth #26 was done at the same appointment.\nThe treatment was conducted under appropriate antibiotic prophylaxis based on amoxicillin plus clavulanic acid 1 g (intraoral) 2 times per day for 8 days as suggested by the patient's nephrologist. The antibiotic prophylaxis was performed in order to cover the infectious risk related to the systemic health status.\nTwo months after the periodontal treatment (hygienic phase), the clinical evaluation showed a successful regression of the inflammation and improvement of periodontal parameters. We have noted a reduction of pockets' depth and plaque and gingival index scores which become, respectively, PI: 0.5 and GI: 0.8.\nThus, a supportive therapy was established including the reinforcement of oral hygiene instruction and full-mouth scaling every 2 months. The whole treatment resulted in the total disappearance of gingival overgrowth without any surgical procedure. The last clinical and X-ray evaluation after 2 years of regular follow-up shows the good stability of the results (Figures –). |
A 25-year-old Caucasian male with no significant past medical history was admitted to the pulmonary service for further workup of hemoptysis in May of 2015. The patient initially presented to an outside hospital in 2014 with three episodes of frank hemoptysis that was two to three tablespoons in quantity. It was associated with shortness of breath on exertion and cough that was productive with yellowish sputum. During that admission, he underwent a CT scan of the chest that showed a 8 cm lobulated mass in right lower lobe with right hilar nodes. He underwent bronchoscopy with mediastinoscopy due to the concern for malignancy. There were no endobronchial lesions seen per the report and pathology showed squamous metaplasia with ulceration. He left against medical advice (LMA) prior to completion of further workup.\nThe patient was seen in our emergency room in May 2015 with a cough with blood-tinged sputum that has gotten worse since his previous admission. He also endorsed 10-pound weight loss and poor appetite. He denied any fever, chills, foul smelling sputum, or recent travel outside the country. He was currently unemployed and smoked one pack of cigarettes daily for last five years. There was no significant family history.\nInformed patient consent was obtained for his treatment. The patient was started on broad-spectrum antibiotics and underwent a CT scan of the chest that showed an irregular mass-like consolidation measuring 9.5 x 5.0 cm in the right lower lobe with adenopathy and right lower lobe bronchiectasis (Figures -). He also underwent a PET/CT that was significant for an FDG-avid mass in the right lower lobe demonstrating an standardized uptake value (SUV) max of 4.5 (Figure ).\nThe patient underwent flexible bronchoscopy that was remarkable for copious purulent secretions in all large airways with friable mucosa (Figure ) and marked mucosal irregularities in the right lower basal segments. Once the secretions were cleared, a gray-colored solid foreign body (approximately 1.0 x 0.5 cm) in right lower lobe was seen that was removed with toothed forceps (Figure ). Pathology was remarkable for non-viable foreign material and underlying submucosa with diffuse lymphoplasmacytic infiltrate admixed with neutrophils, eosinophils, and a few granulomas with no malignancy features.\nOn repeated questioning, the patient admitted to habitual chewing of plastic objects and recalled one episode in 2009 when he may have fallen asleep while chewing on a plastic object. He was given antibiotics for two weeks and was discharged home with a plan to repeat a CT scan in six to eight weeks. However, he was lost to follow-up again. We hypothesize that this episode in 2009 may have caused the unintentional aspiration of the foreign body into the lungs, which the patient was unaware of. This occult foreign body caused foreign body granuloma resulting in a tumor-like mass with intense FDG avidity mimicking malignancy. |
We present a case of a 60-year-old female who presented to the hospital with complaints of dull substernal chest pain. She had chronic atrial fibrillation, chronic obstructive pulmonary disease with home oxygen, osteoarthritis, and anxiety disorder as her comorbid conditions. On examination, she did have chronic dyspnea and was on home oxygen. She did not have any cyanosis, palpitations, paroxysmal nocturnal dyspnea, or orthopnea.\nShe was initially evaluated with a nuclear stress test that did not show any reversible ischemia but dilation of the right ventricle (RV); ejection fraction was identified to be 54%. The patient was further evaluated by a transthoracic echocardiogram (TTE) in order to evaluate the RV dilatation. TTE identified a complete absence of IAS and a CA (). The findings were confirmed with a positive bubble study (). Transesophageal echocardiogram (TEE) was performed that confirmed the absence of IAS, demonstrated free mixing of color flow, moderate to severe tricuspid regurgitation, normal mitral valve structure, normal left ventricular ejection fraction, and enlarged right atrium (RA) and RV.\nThe patient was evaluated with cardiac computed tomography angiogram (CCTA) that demonstrated the right coronary artery to be the dominant artery, all coronary arteries to be ectatic/aneurysmal and measuring up to 8-10 mm, a complete absence of IAS, marked dilation of CA and both ventricles, a coronary arteriovenous fistula (CAF) between the distal left anterior descending and coronary sinus, massive dilation of pulmonary arteries, and no mitral or aortic valvular abnormalities; left ventricular ejection fraction was measured to be 59% (Figures –).\nThe patient underwent an invasive angiography (IA) which demonstrated many abnormal findings. It showed that the patient had coronary artery aneurysms measuring 0.7 cm to 1 cm (). IA was instrumental in taking measurements regarding oxygen saturation and pressure at multiple levels identifying a large interatrial shunt with a 10% increase in oxygen saturation from IVC to RA. IA measured RV pressure to be 98/5 mmHg denoting severe pulmonary hypertension, RV end-diastolic pressure at 12 mmHg, mean RA pressure as 10 mmHg, and left ventricular (LV) end-diastolic pressure as 6 mmHg; oxygen saturation in the inferior vena cava (IVC) was 68.3%; oxygen saturation in RA was 79.8%; oxygen saturation in RV was 79.1%, and oxygen saturation in the femoral artery was 88%.\nThe patient had survived into adulthood with these congenital abnormalities. The patient did not have any muscular, skeletal, ophthalmologic, or vascular abnormalities to signify that her abnormalities were part of any congenital syndrome. Cardiothoracic surgery had been consulted; however, due to the technical implications of surgery, the patient was managed conservatively with no intervention to correct the congenital abnormalities. The patient was not considered for a transcatheter approach of fixing the atrial septal defect as there was a complete absence of the septum. The decision to approach conservatively also included factors such as the age of the patient, comorbid conditions, and the ability of the patient to tolerate this defect (without Eisenmenger syndrome). |
A 55-year-old manual laborer came to our emergency department with a 15-day history of new onset severe holocranial headache that was worse early morning; He did not have a past history of headaches. The headache had worsened and increased in intensity over the past few hours. It was associated with several episodes of vomiting and blurring of vision since day 1. He complained of diplopia on looking to the right. Prior to this, he had been in good health and had been able to work well. He had no known comorbidities. Other than a 6th nerve palsy on the right side, there was no apparent abnormality on general physical examination.\nFrom the history and examination, it was clear that there was raised intracranial pressure, the etiology was not certain. Differentials considered at the time were tuberculous or fungal meningitis, carcinomatous meningitis, a space-occupying lesion in the brain. Magnetic resonance imaging (MRI) of the brain was done, which was reported as normal aside from raised intracranial tension. Lumbar puncture was clearly contraindicated.\nMeanwhile, the patient's neurological symptoms worsened; he developed a VII nerve lower motor neuron palsy and started becoming drowsy. He was started on intravenous Mannitol, hypertonic saline, and dexamethasone. With best possible medical therapy for raised intracranial pressure, his headache persisted and Cushing reflex was present. A neurosurgical consultation was sought and an emergency external ventricular drainage was performed. The patient had significant relief of his headache with the procedure. Cerebrospinal fluid (CSF) from the external ventricular drainage under sterile precautions was sent for biochemical, microbiological, and pathological analysis [].\nCytospin smears of CSF were paucicellular and showed few atypical cells scattered singly and in clusters with eccentrically placed nuclei, granular chromatin, occasional with prominent nucleoli and vacuolated cytoplasm containing mucin, some resembling signet ring cells []. CSF tumor markers showed cancer antigen (CA)-19-9 to be very high. Carcinoembryonic antigen (CEA) in the CSF was elevated as well.\nBecause these are quite specific for intra-abdominal malignancy, computed tomogram (CT) of the abdomen [] done at this time revealed a primary gallbladder malignancy infiltrating the surrounding liver. An ultrasound-guided fine-needle aspiration (FNA) smears from the gallbladder [] showed cellular smears with cohesive clusters, few acinar structures, and few irregular monolayered sheets of polygonal cells with moderate anisokaryosis, occasional prominent nucleoli, and moderate amounts of eosinophilic-to-vacuolated cytoplasm. The background contained necrotic debris and mononuclear inflammatory cells. The patient was offered chemotherapy and radiotherapy. A ventriculoperitoneal shunt (VP) was inserted for relief of raised intracranial pressure. The patient opted for palliative therapy. |
A seventeen-year-old girl was referred for subacute foot pain. In her past medical history, she presented with rhabdomyosarcoma of the left thoracic wall at the age of 8 months treated by surgery and chemotherapy. In her family medical history, her mother developed breast cancer and a leiomyosarcoma diagnosed at the age of 45, two of her maternal aunts in her mother's side were diagnosed for breast cancer, and one of her first-degree cousins with a rhabdomyosarcoma. The genetic analysis found a TP53 mutation c673-2A>G at the splice acceptor site of intron 6.\nHer recent history was marked by the occurrence of a continuous pain on the medial aspect of her right foot. Plain radiographs of the ankle and the foot revealed an ossification of the calcaneus (). The presence of a tumor was confirmed by a CT scan () and a MRI (). The biopsy revealed a high-grade osteosarcoma of the right calcaneus. On the PET scan, three different hypermetabolic lesions were seen: one intense hyper fixation of the right calcaneus (maximum of standardized uptake values SUVmax 7), one moderate hyperfixation of the upper metaphysis of the femur (SUVmax 2.8), and one low hyperfixation of the right sacroiliac (SUVmax 1.9) (). These results were confirmed by scintigraphy (). Based on the radiological aspect and low FDG uptake, the two lesions incidentally discovered were diagnosed as osteochondromas (). It was decided to treat the high-grade osteosarcoma of the right calcaneus and to resect the two lesions after the end of chemotherapy.\nThe initial course of treatment of the high-grade osteosarcoma of the right calcaneus was chemotherapy, and then, the multidisciplinary team proposed a conservative surgery. A resection of the right calcaneus with a reconstruction by iliac graft along with a triple arthrodesis of the hindfoot was performed (Figures and ). The histopathology analysis of the tumor revealed a good response to chemotherapy with less than 5% of residual tumor cells identifiable. The postoperative period was marked by a deep infection of the operative site during chemotherapy requiring surgical treatment with removing the hardware along with the bone graft and adapted antibiotherapy.\nThe two lesions identified on the PET scan were followed up. CT and MRI performed 7 months after the end of chemotherapy showed only a thickening of the femur tumor's cap while the aspect of the sacroiliac lesion stayed stable (Figures and ). In addition to these findings, the patient began to feel pain at the left hip. Then, it was decided to resect both tumors with large resection margins. The surgery of the proximal left femur, performed 2 years after the discovery of the lesions, was a resection of the left proximal femur with a reconstruction by allograft and fibula autograft (). The histopathological analysis of the tumor revealed that it was, in fact, a high-grade surface osteosarcoma. Finally, nine months later, the resection of the tumor of the right sacroiliac joint was performed. The sacral vertebras were conserved, and arthrodesis of the right sacroiliac joint was performed (). The histopathological analysis revealed a well-differentiated surface chondroblastic sarcoma. In both cases, the resection was complete, so no adjuvant treatment has been added.\nAt the last follow-up of eight years, no recurrence had occurred, and the patient was pain-free with an unlimited walking capacity. She was submitted then to annual screening including whole-body MRI and was diagnosed with 3 new tumors (a breast carcinoma at age 28, a melanoma at age 30, and a soft tissue sarcoma at age 31). |
A 37-year-old woman with a past medical history of bacterial endocarditis of the pulmonic, mitral, and tricuspid valves due to intravenous drug abuse, with subsequent tricuspid valve replacement and pulmonary embolism presented to our Emergency Department (ED) complaining of generalized myalgias and shortness of breath. She had no history of congenital cardiac disease. Notes in the medical record indicated that she had baseline oxygen deprivation attributed to a historical diagnosis of pulmonary hypertension. In the ED, she was tachypneic, tachycardic, and had persistent oxygen saturations around 88% on room air mandating the need for oxygen via nasal cannula at 2 l/min. Upon physical exam, she was in moderate respiratory distress and diaphoretic. Cardiovascular examination revealed a harsh, blowing holosystolic grade IV/VI murmur noted at the left upper sternal border. The rest of her physical exam was unremarkable. A complete metabolic panel (CMP) showed no abnormalities and a complete blood count (CBC) showed only mild anemia. No arterial blood gases were tested. An electrocardiogram (EKG) demonstrated a right bundle branch block, but no other marked abnormalities. Due to suspicion for pulmonary embolism, a CT scan of the chest was performed. The CT scan was negative for pulmonary embolism, but did reveal an anomalous vein connecting the left brachiocephalic vein to the left superior pulmonary vein [Figures and and ]. The CT study demonstrated intravenously administered contrast passing from the left brachiocephalic vein through the anomalous vessel into the left superior pulmonary vein to the left atrium, indicating a right-to-left shunt via the anomalous vein. The anomalous vein measured 9 mm in maximum diameter. Subsequently, transthoracic and transesophageal echocardiography was performed. This demonstrated dilatation of the right atrium and right ventricle, severe tricuspid valve regurgitation, and elevated right ventricular pressure estimated at 30 mm Hg. The inferior vena cava was dilated without respiratory variation, also consistent with elevated right atrial pressure. A small fistula between the ascending aorta and the right ventricle was also seen likely secondary to endocarditis. In retrospect, this fistula was not visible on CT due to streak artifacts from the previous tricuspid valve prosthesis. Agitated saline injection showed appearance of bubbles in the left atrium from the left superior pulmonary vein, confirming an intrapulmonary right-to-left shunt. MRI of the anomalous vein was also performed to evaluate the flow characteristics []. As the patient was claustrophobic, only a limited evaluation of the anomalous vein could be performed. The study demonstrated monophasic, inferiorly directed flow within the vessel toward the left superior pulmonary vein, with the flow calculated as 20 ml per heartbeat.\nShortly after admission, treatment was started for bacterial endocarditis and subsequent blood cultures were positive for Escherichia coli. Her symptoms were attributed to bacterial endocarditis and right-heart overload secondary to the severe tricuspid valve regurgitation and the small fistula between the aortic root and the right ventricle. Right-to-left shunting through the anomalous pulmonary vessel was also believed to further exacerbate her hypoxia. No interventions were undertaken with regard to the anomalous vessel during the patient's hospital course. The patient left the hospital against medical advice before completion of therapy and was lost to follow-up. |
A 65-year-old female presented with occasional mild pain in the right upper quadrant of the abdomen for the past 6 months. Pain was nonradiating and not associated with any specific aggravating or relieving factors. She used to have two to three such episodes every month. There was no history of flank pain, hematuria, lower urinary tract symptoms, fever, weight loss, bone pains, or loss of appetite. General physical examination was unremarkable. Abdominal examination revealed a soft, ill-defined smooth, firm lump in the right hypochondriac region extending to the right lumbar region inferiorly and epigastric region medially. Mass was not bimanually palpable but was ballotable. It was not possible to get above the swelling. Ultrasound of the abdomen revealed a large heterogeneous hyperechoic space-occupying lesion arising from the upper and midpole of the right kidney. A contrast-enhanced computed tomography scan of the abdomen was done, which revealed a large heterogeneously enhancing mass measuring 12 cm × 10 cm arising from the upper and midpole of the right kidney, with the involvement of the renal sinus. There was no tumor thrombus in renal vein []. Nearby structures were not invaded. A metastatic workup (liver function tests, serum alkaline phosphatase, and chest X-ray) was done, which was within normal limits. The patient was counseled and taken up for open right radical nephrectomy. Intraoperatively, the mass was closely abutting the liver, from which it was carefully dissected off. The renal artery was doubly ligated and divided, followed by the renal vein in the same fashion. The radical nephrectomy specimen was delivered out and sent for histopathology. The postoperative course was uneventful, and the patient was discharged 4 days after the operation.\nOn gross inspection, the radical nephrectomy specimen with an intact capsule measured 14 cm × 11 cm × 6 cm. The cut surface of the lesion showed variegated rounded growth with hemorrhagic pale yellow and necrotic areas. On microscopy, a thickened fibrocollagenous capsule was seen, which was uninvolved by tumor cells. Cuboidal cells were seen arranged in long cords and tubules and making abrupt transitions to spindle cell morphology at focal places. These epithelial structures were arrayed against the background of lightly basophilic mucinous or myxoid material. The nuclei were spherical or oval with fine chromatin and small nucleoli. Mitotic figures were uncommon. The mucinous background contained mast cells, clusters of foamy histiocytes, and cholesterol clefts. On immunohistochemistry, the tumor cells were positive for CK7, negative for p63, and Alpha-methylacyl-CoA racemase (AMACR) was noncontributory [Figures and ]. Features were consistent with a diagnosis of MTSCC arising from the upper pole of the right kidney. |
A 54-year-old female presented to hospital for new onset of double vision. The patient denied having a similar episode in the past. Additionally, she had persistent left ear ache for 5 months. The patient stated that the earache started as dull, achy pain that had been progressively getting worse requiring multiple office visits. She had been treated with oral and topical antibiotics without any significant relief. Her past medical history includes diabetes mellitus, hypertension, obesity, hypothyroidism, and fibromyalgia. The patient's presenting vitals were stable. The ear exam revealed granulation tissue on the left tympanic membrane and serosanguinous fluid was present. The patient had painful left external ear structure upon gentle traction. The neurologic exam findings were significant for inability to abduct the left eye beyond midline (). When looking left, the patient would have double vision. There were no other focal neurologic deficits, cranial nerve palsies, or abnormal cerebellar signs.\nA CT scan of the head was inconclusive (). A magnetic resonance image (MRI) of the brain showed an enhancement of left skull base that raised the suspicion for active infection (). These findings were further assessed with Gallium-67 citrate (Gallium-67 scan), which revealed a strong possibility of active infection (). Based on imaging and clinical findings, the patient was diagnosed with temporal bone osteomyelitis (TBO) due to malignant otitis externa. She was empirically treated with cefepime for possible Pseudomonas species infection. The patient underwent bone biopsy, which did not grow any organisms. The patient was treated with IV cefepime for 6 weeks. After the completion of antibiotic therapy, a repeat Gallium-67 scan showed improvement in the inflammation (). The patient was then treated with oral ciprofloxacin. The Gallium-67 scan five months after the initial scan showed complete resolution of the inflammation. Her diplopia has markedly improved (). The patient follows up with an ophthalmologist and complete resolution of diplopia is expected. |
A 12-year-old male child presented with history of imbalance on walking with tendency to sway to right side since 1 month. Since last 7 days, patient started having headache with vomiting. On neurological examination, patient was having right sided lower motor type facial paresis with numbness. There was also hearing loss in right ear with right sided lateral rectus palsy. Cerebellar signs were present bilaterally (right > left). Magnetic resonance (MR) imaging of the brain revealed a heterogeneous irregular mass lesion with few cystic areas involving right side of pons, midbrain and middle cerebellar peduncle []. The lesion was extending into right CP angle laterally and causing mass effect on right cerebellar lobe and fourth ventricle. Right side of pons and midbrain was distorted. Post gadolinium images showed irregular ring like enhancement of the margins with perilesional edema [Figure –]. A differential diagnosis of brainstem tuberculoma or exophytic ependymoma was considered. In view of exophytic nature, doubtful diagnosis and mass effect on brain stem a decision to operate was taken. Patient underwent right retro sigmoid suboccipital craniectomy with near total excision of the tumor. The tumor was grayish white in color. It was firm to soft in consistency with gelatinous rubbery areas. Few cystic areas with clear fluid were present. Tumor was densely stuck to VII/VIII nerve complex but was separated meticulously. Medially lesion was indistinctively merging into right side of pons where small part of tumor was left behind. Post operatively patient had worsening of facial paresis. Postoperative MR imaging showed small residual tumor involving right side of pons []. Histopathological examination showed the lesion to be GBM comprising of neoplastic astrocytes with nuclear pleomorphism, hyperchromatism and frequent mitosis. Tumor cells were arranged in sheets with areas of hemorrhages, palisading necrosis, micro vascular proliferation and multinucleated giant cells. Patient received adjuvant radiotherapy. At 1 year follow up, patient was having mild residual paresis of facial nerve. There was no regrowth of small residual tumor on fresh MR scanning. |
We present the case of a 66-year-old male with a several month history of left leg rest pain. Of note, the patient had a remote history of testicular seminoma as a child, for which he underwent a right orchiectomy.\nWorkup began with noninvasive studies of the left lower extremity displaying aortoiliac occlusive disease and infrainguinal disease. Aortogram demonstrated a severely calcified aorta, patent left common iliac and proximal hypogastric arteries, with occlusion of the left external iliac, left common femoral and left superficial femoral arteries (). The right iliac system was patent apart from an occluded right hypogastric artery. A selective left lower extremity angiogram demonstrated reconstitution of the mid-to-distal profunda femoris artery () to the proximal above-knee popliteal artery at the adductor hiatus (). Extensive collateralization was appreciated in the pelvis and lower extremity, between the residual left hypogastric artery and profunda femoris artery. Endovascular revascularization attempts were unsuccessful.\nA right common femoral to left profunda artery bypass was recommended to improve inflow to the left lower extremity with consideration that a left profunda to above-knee popliteal bypass may be needed if symptoms did not resolve.\nThe patient underwent a right common femoral to left profunda femoris artery bypass with a 6-mm ring polytetrafluoroethylene graft. During dissection of the left profunda femoris artery, many collateral vessels were appreciated, with care taken to preserve them. The procedure concluded uneventfully, with newly acquired posterior tibial artery signal in the left foot and continued signals in the right foot.\nOn postoperative day 3, the patient developed bilateral incisional and scrotal erythema with fever and leukocytosis. Despite empiric antibiotic treatment, the patient became increasingly septic. No signs of graft infection were demonstrated on ultrasound or computed tomography scan. A testicular ultrasound duplex displayed the absence of arterial perfusion of the left testis. As a result, simple left orchiectomy was performed. An infarcted testis without evidence of torsion or malignancy was identified on final pathology. Following the procedure, the sepsis resolved, and the patient remained free of rest pain. |
A 53-year-old woman was referred to our outpatient clinic because of recurrent complaints of nausea. In addition, her general practitioner had repeatedly found a low serum sodium concentration. She had a previous history of trigeminal neuralgia, which had proved difficult to treat. Previously, pregabalin, gabapentin and oxcarbazepine had been tried, but either gave serious side effects or insufficient pain relief. The option of microvascular decompression had been discussed with the neurosurgeon, but had been deferred for the time being. Her neurologist had accepted hyponatraemia as an unavoidable side effect of carbamazepine, one of the few drugs that could relieve her trigeminal neuralgia. It was currently under control by a combination of carbamazepine (400 mg b.i.d.) and amitriptyline (10 mg q.d.). She complained of nausea, forgetfulness and difficulty concentrating. Most of these complaints had been present for a number of years. She also admitted gait instability during her weekly game of tennis. She had a normal diet, and was believed to have normal water and salt intake. Her blood pressure was 120/80 mm Hg, her pulse was 60 beats per minute. Physical examination was unremarkable and she neither had oedema nor orthostatic changes (clinical euvolaemia). Her previous laboratory tests showed chronic hyponatraemia with serum sodium levels fluctuating between 124 and 135 mmol/L since 2004. Her current laboratory results are shown in Table . Because of the biochemical data (Table ) together with carbamazepine and amitriptyline use, SIADH appeared the most obvious diagnosis, although adrenal insufficiency could not formally be excluded on the basis of a random cortisol value. Because the neurologist determined that the carbamazepine was essential to the patient, we advised the patient to limit fluid intake to 1 L/day, and to contact the hospital in situations of fever, diarrhoea, vomiting or the prescription of a new drug. We explained the likely connection between her symptoms and her chronic hyponatraemia to her neurologist, and advised to check serum sodium levels at regular intervals. Because this patient was recently seen in our outpatient clinic, the response in serum sodium and symptomatology to fluid restriction remains to be determined. |
A 40-year-old female, height 145 cm, weight 45 kg, gravida 1 para 0, was planned for an elective caesarean section at 38 weeks of gestational age. She has a significant past medical history of thalassaemia minor, migraine and a type I ACM. She presented 5 years ago with persistent left-sided headaches, numbness of the left side of the face, upper limb and neck. The initial MRI had depicted a large syringohydromyelia seen in the entire spinal cord extending from C1 to T12 level with only a small syrinx in the conus medullaris. There was herniation of the cerebellar tonsils below the level of the foramen magnum by about 11 mm. She underwent a foramen magnum decompression. The intraoperative findings were that of a thickened dural band at the foramen magnum, with elongation of tonsils down to the upper border of C1. The follow-up MRI done the following year showed that the CSF space around the craniocervical junction had improved, though the large syrinx still persisted and extended from C1 to beyond T5.\nNeurosurgical consult was made early in pregnancy by the obstetrician and she was deemed suitable to proceed with either a normal vaginal delivery or a caesarean section.\nPre-operative assessment was conducted 1 day prior to the surgery. There was residual left-sided numbness over her face and left upper limb and the headaches were still present but not worsening. There were no other signs or symptoms of raised intracranial pressure (ICP). These symptoms were not aggravated with pregnancy, coughing, or neck movements.\nA physical examination was done, specifically, there was decreased sensation over the left face and left upper limb. Airway examination was unremarkable. There was a slight thoracic scoliosis over her back but the spinous processes were well felt in the lumbar region.\nShe was counselled regarding the risks and benefits of spinal NA versus general anaesthesia (GA) together with the obstetrician, with consideration of the input from the neurosurgeon. A normal vaginal delivery was discussed again as it was not a contraindication. However, she had concerns with lying supine for a prolonged period due to her thoracic scoliosis.\nShe had initially decided to proceed with the caesarean section under a GA. However, after thorough discussion of the risks and benefits of a GA versus spinal NA, the decision was made to proceed with the surgery under spinal NA.\nThe patient was positioned in the sitting position, and a single shot spinal anaesthetic was given at the level of L3/4 interspace. This was a single attempt using a 27-gauge Whitacre spinal needle, with a spinal introducer. Given her relatively short stature, intra-thecal hyperbaric bupivacaine 0.5% 1.6 mL with fentanyl 15 µg and morphine 100 µg was administered. Assessment of block height to cold sensation was performed, and a level corresponding to that of T4 was achieved prior to surgical incision.\nIntra-operatively, the patient had mild discomfort from intraoperative surgical stretching of the abdomen, which was transient in nature. She was given intravenous (IV) fentanyl 50 µg in titrated boluses, together with Entonox, just prior to the delivery of the baby. After the delivery of the baby, she was given IV 1 mg of midazolam. The surgery was completed uneventfully thereafter without further complaints. Post-operatively, she was monitored in the high dependency unit overnight. Follow-up on the patient on the first day following surgery revealed no worsening of her pre-existing neurological symptoms, which was that of a left-sided numbness over her face and left upper limb. There was also no interval development of new neurological symptoms. She was discharged on post-operative day 3 and remained well on her follow-up visit with the obstetrician 2 weeks later. |
A 50-year-old male was admitted to our hospital complaining of slowly progressing weakness. He stated that when he was 4 years old, his mother noticed that he was not to be able to run as fast as his peers. He was the first of three children of his non-consanguineous parents, and both his siblings and his parents were healthy. No other muscular symptoms were noted throughout his childhood. In the 2 years leading up to his evaluation, he noticed mild weakness in his legs and difficulty getting up from a squatting position. These symptoms gradually became more severe, until he presented at age 50 with proximal-to-distal weakness in the muscles of the lower limbs. He has never experienced cognitive or cardiologic problems.\nNeurological examination revealed mild proximal muscle weakness in the lower extremities and the toe extensor muscles (MRC grade 4). The Gower’s sign test was positive and his tendon reflexes remained present but weak. There was no evidence of calf hypertrophy, scapular winging, or facial, and ocular impairment. Laboratory testing revealed slightly elevated creatine kinase levels (2× normal). Electromyography abnormalities of myopathic changes were found in both the vastus medialis and tibialis muscles. Echocardiograph and electrocardiogram results were normal. A magnetic resonance imaging (MRI) of the patient’s muscles revealed a preferential fatty infiltration in the posterior compartment of the patient’s thigh, with marked sparing in the gracilis, sartorius, and quadriceps muscles (). There was more focal involvement in the gastrocnemius muscles, and the remaining muscle groups were mildly involved (). The upper limb muscles demonstrate minimal fatty infiltration and no distinct involvement pattern (-).\nPeripheral blood samples were obtained with informed consent from three members of the family. Genomic DNA was isolated from these samples according to standard techniques. All known protein-encoding regions, including a total of 19,396 genes, were captured and enriched. The enriched libraries were sequenced on an Illumina NovaSeq 6000 (Illumina, USA), which generated data covering 99.9% of the sequence at 300×. The sequencing reads were aligned on a reference human genome (hg19) using Burrows-Wheeler Aligner software. The Genome Analysis Toolkit (GATK) was used to detect single-nucleotide variants and indels of the reads. Whole exome sequencing was performed at Chigene Medical Research Centre (Beijing, China). It turned out that the patient had homozygous deletion of a 2kb region, chr9.hg19: g.119460021_119461983del, in exon 2 of the TRIM32 gene (). The presence of this homozygous deletion was confirmed with quantitative real-time polymerase chain reaction (). The patient’s parents were heterozygous for the mutation. This novel mutation was neither observed in 200 healthy Chinese controls, nor four public databases, including dbVar, ClinVar, the Database of Genomic Variants (), and the Leiden Open Variation Database ().\nAfter written informed consent was obtained from the patient, a gastrocnemius biopsy was conducted. Muscle biopsy revealed marked variation of the muscle fiber diameters, internalized nuclei, as well as necrotic and atrophic myofibers. Small and irregularly slit-shaped vacuoles containing basophilic material were observed throughout the sarcoplasm in scattered myofibers. The connective tissue of the muscle was moderately increased. However, no inflammatory cell infiltration was observed (). Immunohistochemical staining showed that anti-dystrophin-N, -C, -R proteins, anti-α-, β-, γ-, δ-sarcoglycan proteins, calpain-3, and dysferlin levels were normal. |
A 12-year-old boy was hospitalized with complaints of a headache and high fever accompanied by psychosis, delirium, and indistinct consciousness. He was diagnosed with limbic encephalitis, which is an autoimmune disorder characterized by inflammation of the limbic area in the brain. His symptoms became exacerbated, and he required intensive therapies including high-dose steroid and catecholamine administration.\nDespite the continuous therapeutic support mentioned above, he abruptly developed hypotension following diarrhea, fever, and abdominal distension on day 41 after admission. Metabolic acidosis (pH 7.34, base excess − 7.0 mmol/L) was confirmed by blood gas analysis, and highly elevated CPK 11800 U/L, AST 461 U/L, ALT 201 U/L, and LDH 1034 U/L values were revealed by a blood chemistry profile. An emergency CT scan revealed pneumatosis intestinalis localized in the ascending colon and rectum coexisting with portal venous gas (Fig. ). While the root of the SMA and the inferior mesenteric arterial (IMA) flow was maintained, the peripheral blood flow was attenuated adjacent to the non-contrast-enhanced ascending colon and rectum.\nAlthough intraabdominal free air was not detected in the CT scan, the massive ascites and progressing peritoneal signs with muscular guarding required an emergency laparotomy for suspected mesenteric ischemia and bowel perforation. Intraoperatively, skip ischemic lesions were observed in the ascending colon close to the hepatic flexure and the rectum without bowel perforation. Although SMA and superior rectal arterial pulsations were present, the marginal perfusion near the two lesions could not be confirmed. The patient was diagnosed with NOMI based on these operative findings and the rapid progression of the symptoms, which are unlike other vascular disorders or necrotizing enterocolitis. The remaining colon, from the transverse to the sigmoid colon, appeared intact. The color of the unaffected intestinal wall was restored, which suggested intestinal viability (Fig. ). We performed a distal ileostomy without bowel resection because a second-look laparotomy after 24 to 48 h was considered.\nAfter returning to the ICU, the patient required resuscitation for cardiac arrest, septicemia, and DIC. The scheduled second-look laparotomy was canceled, and intensive care including hemodiafiltration was continued. However, the gastrointestinal symptoms did not progress during the intensive treatment.\nOn the 16th and 60th postoperative days, colonoscopies were carried out, and they revealed rectal and ascending colon stenosis with ulceration (Fig. ). The patency was 5 mm in diameter at both strictures. However, normal findings in the transverse colon to the sigmoid colon were observed by colonoscopy.\nA lower gastrointestinal series by gastrografin contrast radiography also demonstrated the patency of the two lesions after laparotomy (Fig. a). Based on successful evacuation of the contrast media and intact mucosal findings around the mild stricture, we scheduled ileal stoma closure. For 1 month prior to the closure, approximately 100 ml of bowel contents that had collected in the ostomy pouch were injected into the anal side of the ileostomy to induce efficient bowel movement. We confirmed the continuous expulsion of feces from the anus and the improvement of transient bowel strictures; therefore, the ileal stoma was closed 14 months after the previous laparotomy.\nCurrently, the patient’s confusional state has prolonged, and he has received enriched liquid nutrition via gastrostomy. The two stenotic lesions are completely resolved, and defecation has been maintained after stoma closure (Fig. b). |
A 55-year-old Caucasian male presented to an outside clinic with a two-month history of fatigue and right bundle branch block (RBBB). At the time of presentation, he exhibited AF with a rapid ventricular response. Symptoms included shortness of breath and chest discomfort during episodes of AF, which lasted 15 to 120 minutes. Cardiac computed tomography (CT) angiography revealed a lesion in the left anterior descending coronary artery, but subsequent cardiac catheterization revealed no evidence of obstructive coronary artery disease. Notably, mildly enlarged lymph nodes in the pericardial region and mediastinum were observed on the CT angiogram. Three years prior, his left ventricular ejection fraction (LVEF) was 55% on cardiac catheterization. Two weeks prior to referral, transthoracic echocardiogram revealed an LVEF of 40%, and the newly depressed LVEF was suspected to be due to tachycardia-induced cardiomyopathy related with AF. The patient was initially started on rivaroxaban and propafenone by the referring physician. He continued to have episodes of AF and was switched to amiodarone. He was referred to our center for AF ablation.\nHis past medical history also included hypertension and hyperlipidemia. At the time of presentation to our center, his medications included metoprolol, rivaroxaban, and amiodarone. Due to a suspected amiodarone-induced cough and the patient’s young age, amiodarone was discontinued and changed to dofetilide.\nElectrocardiogram (ECG) in our clinic showed sinus rhythm with RBBB with a QRS duration of 160 ms and fragmented QRS (. He underwent cryoballoon pulmonary vein isolation (PVI) without complication. The following day, he underwent CMR to evaluate his fractionated RBBB and reduced LVEF seen on transthoracic echocardiogram and intracardiac echocardiogram during the ablation. CMR showed an LVEF of 40% and extensive, patchy delayed enhancement throughout the left ventricular myocardium along with enlarged mediastinal lymph nodes, consistent with cardiac and pulmonary sarcoidosis (.\nOne week later, the patient underwent endobronchial ultrasound-guided mediastinal lymph node biopsy, which revealed poorly formed, non-necrotizing granulomas. Based on these findings, he met the 2014 Heart Rhythm Society Expert Consensus Statement criteria for the diagnosis of CS and subsequently underwent dual-chamber implantable cardioverter-defibrillator (ICD) implantation for primary prevention. The patient was reluctant to use corticosteroids and was instead started on methotrexate with folic acid. A baseline 18F-FDG-PET/CT scan showed extensive heterogeneous 18F-FDG uptake throughout the myocardium that corresponded with perfusion abnormalities on the myocardial perfusion imaging scan (. However, although his shortness of breath resolved, the patient’s complaint of fatigue remained.\nBased on the active inflammation seen on cardiac PET and the patient’s reduced LVEF, the methotrexate dosage was increased from 15 mg to 25 mg weekly; 400 mg infliximab was intravenously administered at baseline, weeks two and six, and then every six weeks thereafter.\nSeven months after cardiac ablation, the patient experienced multiple presyncopal episodes while at work. ICD remote transmission showed five episodes of non-sustained polymorphic ventricular tachycardia (VT), as well as episodes of monomorphic VT (. ECG was performed and showed normal sinus rhythm with frequent premature ventricular contractions (. The patient was admitted to the hospital for initiation of sotalol. On discharge, the frequency of infliximab treatment was increased to every four weeks. No programming changes were made to the ICD.\nTransthoracic echocardiogram revealed stable LVEF of 40%. A cardiac 18F-FDG-PET scan performed 10 months after the initial 18F-FDG-PET scan revealed an overall decrease in 18F-FDG uptake, with a new focus in the mid-inferolateral myocardial wall (. On the current regimen, the patient has remained clinically stable without any further atrial or ventricular arrhythmias. |
A 39-year-old male presented to the clinic with a history of facial redness and swelling and recurrent syncope over the past year that was noticeably worse in the supine or bending forward positions or while doing push-ups/lifting weights. The patient had undergone DDDR [Talos DR, 60/60 beats per minute (bpm) with rate hysteresis of 10 bpm; Biotronik, Berlin, Germany] pacemaker implantation due to cardioinhibitory syncope that resulted in orthopedic injuries four years prior. His syncopal attacks resolved immediately after pacemaker implantation; however, they restarted with a change in nature at about one year later, occurring particularly in relation to him bending down to tie his shoelaces. Physical examination findings were unremarkable, except that, upon bending forward, the patient developed prominent venous collaterals on the neck and anterior chest/abdominal walls and positional syncope was reproduced. A computed tomography scan and venography confirmed SVC occlusion ( with a 14-mmHg gradient across the obstruction and total occlusion of the left subclavian vein (. Following three months of oral warfarin therapy without any clinical benefit, the leads were separated from their middle part during mechanical traction from the subclavian vein and removed by use of a transfemoral snare system (. Subsequently, a balloon angioplasty was performed at a pressure of 8 atm.\nGiven the significant venous recoil, we decided to implant a stent. Using fluoroscopy, we confirmed placement in the proper stent position ( and finalized stent deployment by inflating the outer balloon to a pressure of 6 atm ( and . Follow-up venogram results indicated no collateral flow, and the pressure gradient was found to be reduced from 14 mmHg to 1 mmHg. The patient had no postoperative complications and was discharged from the hospital the next morning, with instructions to take dual antiplatelet agents. Clinical follow-up after one month revealed marked improvement in his symptoms, including resolution of his positional syncope; however, his cardioinhibitory syncopal attacks had resumed. Therefore, two months later, we implanted a Micra™ leadless pacemaker (Medtronic, Minneapolis, MN, USA) ( and . At one year postimplantation, the patient had no syncopal events. |
A 69-year-old Saudi man with diabetes mellitus was admitted to the Neurology Department with an unremitting headache lasting 5 days, episodic confusion, and visual disturbances. According to his family, the headache started gradually over the left side of his head and then became holocephalic and moderate to severe in intensity. The patient reported feelings of nausea and 2 episodes of vomiting. Moreover, his family stated the patient was often seen “bumping” into surrounding objects while ambulating. The patient reported experiencing some visual disturbance during this period. The patient's family felt he appeared confused at times and was not responding to his surroundings. He had no clear history of seizure, according to the family, and his past medical history was unremarkable apart from diabetes. He had sustained a minor head trauma 3 years prior with no concussion; however, his scan was reported to have shown “scattered areas of bleeding” in his brain. He otherwise maintained a healthy life and never required a hospital visit for any medical issues. His family reported the patient had normal cognitive function, especially as someone who ran his own business.\nThe patient was lethargic upon arrival to the accident and emergency department. Given the apparent risk of airway obstruction, the emergency physician intubated the patient. On general examination, we found no facial phakomas. While the patient was sedated, we found tonic eye deviation with nystagmoid-like eye movement and subtle myoclonic jerks of the distal limb suggestive of subclinical seizures. He was given an intravenous (IV) loading dose of phenytoin in addition to a midazolam infusion. Despite this, he sustained several clinical seizures in the subsequent days. Therefore, he required further titration of midazolam infusion (up to 14 mg/hr) and IV levetiracetam was added to optimize the antiepileptic coverage. His electroencephalogram was obtained postictal. His brain MRI was obtained 1 day following suspected subclinical seizures. Laboratory investigations showed peripehral blood cell count, haemoglobin, renal and liver function within reference range, and his blood glucose was elevated (14.7 mmol/L/264mg/dl).\nMoreover, the results of his thyroid function test and his parathyroid hormone and serum vitamin B12 levels were normal. His serum folate level was not available. The screening tests for Hepatitis B antigen, Hepatitis C virus, and human immunodeficiency virus antigens and antibodies were all negative. His anti-TTG immunoglobulin A (IgA) titre was high at 35 U (reference range is up to 20 U), and the screen for antiendomysial antibody was negative, and his anti-Gliadin IgA antibody results were within reference range. Cerebrospinal fluid (CSF) study showed a WBC of 1, red blood cell count of 1, protein level of 36mg/dL, and a glucose level of 7.6mmol/L. The results of the CSF tuberculosis and herpes simplex virus 1 and 2 polymerase chain reaction were negative. We performed a duodenal endoscopic biopsy, but the specimen was not prepared properly; therefore, histopathological examination was suboptimal. We did note, however, increased intraepithelial lymphocytes with normal villous architecture.\nThe computed tomography (CT) of the patient's brain showed bilateral scattered corticosubcortical parietooccipitotemporal calcification with no oedema or mass effect (). The brain MRI with and without contrast showed diffusion-weighted imaging (DWI) restriction over bilateral occipital cortex (more so over the left side) in a gyriform pattern () with concordant area of apparent diffusion coefficient (ADC) hypointensity (). Susceptibility weighted magnetic resonance sequences (susceptibility weighted imaging [SWI], susceptibility weighted angiography [SWAN]) demonstrated hyperintensity corresponding to the area of DWI restriction (). T1-weighted imaging with contrast showed no contrast uptake (), and we saw no oedema or mass effect. We found no cortical atrophy or any deep cerebral vein enlargement. A second brain CT after 1 month () showed no interval change compared to the initial CT and no evidence of residual changes observed in MRI (DWI, ADC, and SWI).\nBilateral cortical calcification has a distinct set of mimickers, and Sturge–Weber syndrome (SWS) is first among the possible differentials. Our patient's brain CT may indeed look identical to SWS. However, it is the constellation of clinical and radiological features that differentiate the two conditions. The important clinically distinguishing feature of SWS is our patient's normal cognition. Psychomotor retardation is seen in 50% of SWS cases [], and our patient lacked facial nevus which is prevalent in most SWS cases (except SWS Roach classification type 2) []. Our patient also had no ocular disease, a presentation found in 77% of SWS cases. Radiologically, SWS has tram line or gyriform-only cortical calcification (and not subcortical, as in our patient). Bilateral calcification (as seen in our patient) occurs in only 25% of SWS patients []. Other radiological findings of SWS not seen in our patient are ipsilateral choroid plexus hypertrophy [], enlarged transcortical (medullary) veins [], ipsilateral cortical atrophy [], enlargement of the ipsilateral ventricle, loss of volume of the ipsilateral cranial cavity [], and pial enhancing angiomatous malformation (usually ipsilateral to the facial angioma). Therefore, many classic SWS features were not identified in our patient, distinguishing his condition from SWS.\nOther conditions known to cause cortical calcification in a similar pattern are, namely, congenital folate malabsorption or the adverse effects associated with methotrexate and antifolate agents. There was no mention of the use of either of these agents. Congenital folate malabsorption would have an associated life-long history of symptoms of malabsorption [] which is not apparent in our patient. Cortical laminar necrosis could account for such radiological presentation. However, this entity is conventionally thought to be a sequela of a remote traumatic brain injury, anoxic-ischaemic injury, or a metabolic insult that is not present in this patient's past history. Moreover, characteristically, cortical laminar necrosis has T1-weighted gyriform hyperintensity in MRI which was not noted in this patient. Although each of these conditions gives rise to calcified shadows in brain imaging, none are anti-TTG IgA-positive which is specific for the diagnosis of CD.\nAntiepileptic treatment with phenytoin and levetiracetam was commenced immediately upon the realisation that the patient has seizures. With the diagnosis of probable CEC, he was started on a strict gluten-free diet.\nThe patient recovered remarkably and regularly submits to follow-up examinations at our neurology clinic. At his last visit (9 months from his presentation), we found no residual neurological deficit other than mild homonymous hemianopia. Currently, he is fully active, self-sufficient, and has not sustained any seizure for the past 9 months. |
A 70-year-old male with myelodysplastic syndrome treated with double cord allogeneic blood stem cell transplant 34 months ago complicated with chronic GVHD-related glomerular nephropathy, adrenal insufficiency, and end-stage renal disease on hemodialysis presented to clinic after 2 weeks of joint pain. Physical exam revealed normal strength, and he was treated with nonsteroidal anti-inflammatory therapy. Four days later, he developed worsening joint pain, lower extremity calf pain, and hoarse voice. Examination at this time was notable for 3/5 strength in his lower extremities. He was admitted to the hospital and treated with 50 mg intravenous hydrocortisone every 12 hours for three days and intravenous fluids for a suspected postviral myositis. His symptoms resolved, and he was discharged home.\nFive days afterwards, he was readmitted to a different hospital for dysphagia and concern of aspiration pneumonia. A gastric tube was placed, he was treated with intravenous ceftriaxone and metronidazole, and he was sent to a rehabilitation facility. Of note, he was not treated with a quinolone or aminoglycoside antibiotic. Another four days later, he presented to our hospital with worsening pneumonia. At this time, physical exam revealed decreased proximal muscle and grip strength, weak palate elevation, diplopia upon prolonged upward gaze, and 3/5 strength in lower extremities. He did not exhibit muscle fatigue from repetitive use. Initial evaluation for this patient was unremarkable for brain or cranial nerve lesions, motor neuron disease, neuromuscular junction disorders (NMJ), or other myopathies (). He was treated with stress-dose steroids (50 mg methylprednisolone every six hours) for concern of myositis, antibiotics, and other supportive measures. Four days after this admission, the antiacetylcholine receptor (AChR) antibody (Ab) panel revealed elevated ACR binding and modulating antibodies correlating with diagnosis of myasthenia gravis. Titers for AChR binding Ab were elevated at 2.15 nmol/L, and AChR modulating Ab was elevated at 45%. He was started on pyridostigmine and plasma exchange (5 exchanges, 3500 ml per exchange). Three days into this therapy, he developed respiratory failure from MG crisis and worsening aspiration pneumonia. Due to worsening symptoms, the patient requested hospice care, and he patient passed away soon thereafter (). |
A 41-year-old Asian man presented to our hospital after an automobile accident in which he sustained a severe crush injury to his right foot that resulted in formation of a soft-tissue lesion in the weight-bearing heel and instep area. After the debridement, the size of a full-thickness defect at the medial and anterior aspects of the heel measured 6cm in length and 4cm in width (Figure ). Because the extensive zone of injury involved the proximal instep area, an ipsilateral instep island flap was not possible. Therefore, a plan was made to cover the defect using a free instep flap.The patient was placed in the prone position, and both legs were abducted for simultaneous flap elevation and preparation of the recipient site. The operation was performed under tourniquet control for better visualization. Debridement of the defect was performed, and the stumps of the posterior tibial artery and vein, as well as the tibial nerve, were indentified and dissected proximal to the defect. In harvesting the instep flap from the contralateral foot, the skin paddle was designed 7cm in length and 5cm in width and marked in the instep area (Figure ), so that it was slightly larger than the defect to allow for flap-moulding. Flap dissection began with an incision along the distal border of the flap down to the fascia, which then was incised and dissected in a plane superficial to the abductor hallucis muscle. The distal branches of the medial plantar artery were carefully indentified and divided. The intermuscular septum between the abductor hallucis muscle and the flexor digitorum brevis containing the medial plantar artery, nerve and its fasciocutaneous perforators was raised in continuity with the flap. During flap dissection, the medial plantar nerve and the cutaneous branches of the first toe were preserved, but cutaneous nerve fascicles were retained within the flap. To obtain adequate length of these fascicles, we performed interfascicular dissection of the medial plantar nerve proximally. To facilitate flap harvest, the abductor hallucis muscle was divided at its origin, exposing the neurovascular pedicle deep into it. The neurovascular pedicle of the medial plantar artery and venae comitans was dissected proximally up to the common origin of the medial and lateral plantar artery from the posterior tibial artery (Figure ). The abductor hallucis muscle was reattached, and the donor site was covered with a split-thickness skin graft taken from the thigh (Figure ).\nSo as not to sacrifice an important artery in the traumatized foot of our patient, inset of the flap into the defect was followed by end-to-side anastomosis of its medial plantar artery to the recipient posterior tibial artery. The venous anastomosis was carried out end-to-end. One artery and two venae comitans were anastomosed. An epineurial window was meticulously made on the medial surface of the tibial nerve to ensure nerve-sprouting from sensory nerve fascicles. The cutaneous nerve fascicles of the flap were then anastomosed end-to-side with the recipient tibial nerve. When the tourniquet was freed, vascularization of the flap was noted (Figure ).The donor and recipient sites healed without any complications during 3 weeks of follow-up (Figure ). Two months after surgery, the patient was able to wear normal footwear and had a normal gait. At his 3-month follow-up examination, the patient subjectively reported an increase in sensation at the recipient site compared to immediately after surgery. Static two-point discrimination was 20mm compared to 18mm of the contralateral normal side at the 6-month follow-up visit. At 30 months after surgery, no ulceration in the flap due to poor contact with the shoe had occurred. |
A 26-year-old woman without any medical history suddenly collapsed while she was walking. Her friends called 119, and basic life support was started in 5 minutes. An automated external defibrillator (AED) was applied three times because the cardiac rhythm showed ventricular fibrillation. Spontaneous circulation returned after 10 minutes of cardiopulmonary resuscitation (CPR). When the patient arrived at the emergency room of Korea University Anam Hospital, a 12-lead electrocardiogram (ECG) showed a sinus rhythm. There was no evidence of cerebral hemorrhage, infarction, or myocardial ischemia on brain computed tomography and 12-lead ECG. The patient was admitted to the intensive care unit (ICU) and underwent targeted temperature management. Forty-eight hours later, the patient regained full consciousness.\nTo evaluate the cause of cardiac arrest, ECG was continuously monitored via telemetry during ICU admission. Frequent premature ventricular contraction (PVC) in the bigeminy pattern and T wave inversion in the inferior leads were observed (). QT was mildly prolonged up to 484 ms. However, an epinephrine challenge test to evaluate long QT syndrome showed a negative result. Echocardiography showed bileaflet mitral prolapse and moderate mitral regurgitation. Both valvular leaflets were thickened with myxomatous change, and the posterior mitral leaflet was elongated. During systole, the mitral annulus was backwardly displaced and separated from the atrial aspect of the left ventricle (mitral annular disjunction), and the posterior mitral ring moved backward exaggeratedly and adjacent to the posterior left ventricular wall (curling). The inferomedial papillary muscle was hypertrophied, and the myocardial figure of the inferior wall looked abnormal (). In cardiac magnetic resonance (MR) imaging, the abnormal movement of mitral annulus, hypertrophied papillary muscles, and abnormal figure of the left ventricular inferior wall were consistently observed with a focally thinned left ventricular basal inferior wall, where it was adjacent to the base of the hypertrophied papillary muscle. Although it was not very prominent, the signal intensity was inhomogeneous in that lesion upon late gadolinium imaging, which suggested the presence of damaged myocardium (). |
A 73-year-old man who had developed pneumonia 1 year previously underwent a detailed pulmonary examination for an abnormal residual chest shadow. He was a taxi driver and had a history of smoking. Chest computed tomography (CT) revealed a solid lesion in the right lower lobe of the lung (approximately 2 cm in diameter) and multiple patchy ground-glass opacities (GGO) in the bilateral lungs (). As primary lung cancer was highly suspected, partial resection of the right lower lobe including the solid lesion was performed. The histological diagnosis of the solid lesion was invasive adenocarcinoma. The patient was discharged postoperatively but was readmitted a few days later because of severe dyspnea. No mass lesion was found in the lungs or other organs on CT. However, diffuse and patchy GGO remained and had worsened. Infection was suspected, but the lesion also exhibited the abnormal uptake of fluorodeoxyglucose (FDG) on positron emission tomography (PET). In addition, the serum levels of soluble IL-2 receptor and lactase dehydrogenase were high (2,098 U/mL and 645 U/L, respectively). Pulmonary malignant lymphoma was suggested, and the previously resected specimen was pathologically re-evaluated.\nIn addition to the solid lesion, which consisted of invasive adenocarcinoma (papillary adenocarcinoma with a lepidic growth pattern), diffuse scattered pulmonary interstitial lesions were observed. In some areas around the solid lesion, adenocarcinoma and interstitial lesions were close, but they did not mix. Detailed observation revealed that the interstitial lesions were histologically divided into two types, although they looked similar at first: 1) lesions located along bronchovascular bundles, characterized by the aggregation of many macrophages and non-atypical small lymphoid cells with anthracosis, and 2) thickened alveolar septa in centrilobular areas, where small aggregates of inflammatory cells were found, but macrophage aggregation and anthracosis were not notable. Many atypical large lymphoid cells were identified in the capillaries of the alveolar septa of the second lesion. These atypical cells were also diffusely detected in the non-thickened alveolar septa (). Immunohistochemically, these atypical cells were positive for CD20 and CD5 (). They were also positive for CD79a, BCL2, and MUM1, and negative for CD3, CD10, and EBER-ISH (data not shown). Of note, these two types of interstitial lesions were not mixed, and no CD20-positive large atypical cells were found in the peribronchovascular lesions. No parenchymal invasion was histologically noted throughout the entire resected specimen. Bone marrow examination performed later for staging revealed CD20-positive large atypical lymphoid cells in small vessels; however, the area involved was limited, and only a few atypical cells were present. Thus, the lung was thought to be the predominant site of the IVLBCL.\nTherapy for IVLBCL was prioritized. The patient underwent six courses of rituximab, cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisone (R-CHOP) therapy. His dyspnea, GGO on CT, and abnormal lung uptake of FDG on PET subsequently dissipated. |
A 64-year-old man with a history of hypertension presented with a 2-year history of shortness of breath. He denied exertional chest discomfort. He did not have features suggestive of a syndromic aortopathy and had no family history of aortic disease. Transthoracic echocardiography showed moderate to severe aortic regurgitation with a severely dilated left ventricle and preserved left ventricular function and an annular diameter of 2.5 cm. The aortic valve was tricuspid. Contrast computed tomography scan demonstrated an anomalous LCX originating from the right coronary artery with a retroaortic course (, A and B), an ascending aortic aneurysm of 6.0 cm, and a dilated aortic root of 5.3 cm. Coronary angiography confirmed an anomalous LCX originating from the dominant right coronary artery, coursing into the obtuse marginal distribution (, C).\nThe patient was brought to the operating theater. Cardiopulmonary bypass was established via proximal aortic arch and right atrial cannulation, and systemic cooling was initiated targeting a nasopharyngeal temperature of 24°C. Following aortic cross-clamping and cardiac arrest achieved by selective cardioplegic perfusion to the left main and right coronary ostium, the aortic root was prepared by resecting the aortic root tissue with a 5-mm cuff and mobilizing the coronary artery buttons. The anomalous LCX was identified at its origin of the right coronary button coursing down toward the noncoronary sinus of Valsalva ( and ). The close association between the aortic annulus and the anomalous LCX would have resulted in compression or suture impingement.\nBecause the aortic annulus was not dilated, we decided to perform a remodeling root replacement to keep the sutures above the annular plane and avoid injury to the anomalous LCX. A 28-mm woven polyester graft was fashioned into 3 tongues and was sutured to the aortic wall remnant with 4-0 polypropylene to create 3 neosinuses. The aortic cusp height was measured using a Schafer caliper, and all 3 cusps had an effective height of >9 mm. Both coronary buttons were reimplanted in a standard fashion. Before completing the aortic root repair, we performed a hemiarch reconstruction under moderate hypothermic circulatory arrest and antegrade cerebral perfusion via the innominate artery.\nPostoperative echocardiography confirmed excellent aortic valve function with no aortic insufficiency and prolapse. The perioperative electrocardiogram was unchanged. |
“Theresa” was a widowed, White British woman in her 50s who saw an advertisement for the study in her local newspaper. Her doctor had suggested that she was depressed 2 years previously and had recommended antidepressants, but she had declined these because she felt that medication would not “solve the problem” of why she was depressed. Theresa had few expectations for the online computer program she had seen advertised but was interested to take part in the study as an opportunity to “do something useful.”\nDuring the study eligibility assessment, Theresa reported a long history of recurrent depression since the age of 21, and estimated her current major depressive episode to be of 4 years duration. The diagnostic interview revealed a number of comorbidities, namely, current posttraumatic stress disorder (from a physical assault at work 10 years previously); social phobia (since her late teens), which was characterized by a fear of coming across as “stupid” in conversations; a specific travel phobia (as long as she could remember), originally limited to public transport but which had recently spread to include driving her car; and generalized anxiety disorder (since her early teens), which was characterized by worry (especially in the evening or at night) about a range of topics from her own health to the life choices of her daughters. Theresa also had a history of alcohol dependence, and although she did not currently meet diagnostic criteria, she felt that she was drinking more than she should do. Theresa was currently receiving no treatment for her depression or anxiety. She was currently taking codeine for chronic pain, which she described as diffuse in nature, affecting many of her muscles and joints.\nDespite her long history of depression and anxiety, Theresa had received little treatment in the past. She had taken Valium briefly in her early 20s and an antidepressant for a few years in her 30s. She had received a few sessions of counseling 5 years previously but had not found it helpful because she did not get on well with the counselor.\nTheresa had held a number of administrative jobs in the past, but recently she had been reducing her hours and was increasingly worried about debt. She lived alone with her mother, who was in the early stages of Alzheimer's disease, and for whom she was the main caregiver. Theresa's husband had died 15 years previously, following a stroke; and although she had two adult daughters, they did not live locally and she saw them only infrequently.\nOn the baseline questionnaire assessment measures (see Table ), Theresa scored in the “severe” range for depression on the Beck Depression Inventory – II (BDI-II; Beck, Steer, & Brown, ), including anhedonic symptoms (rating “I get very little pleasure from the things I used to enjoy” and “I am less interested in other people or things than before”). On the Prospective Imagery Test (PIT; Holmes, Lang, Moulds, & Steele, ), she rated the vividness with which she could imagine positive events in her future as “vague and dim” on average. She scored nearly at ceiling on a measure of negative interpretation bias, the Scrambled Sentences Test (SST; Rude et al., ). Her score on the Behavioural Activation for Depression Scale (BADS; Kanter, Mulick, Busch, Berlin, & Martell, ) indicated very low levels of behavioral activation, consistent with her report that she was “not doing very much around the house” and that she often struggled to go out even to carry out simple errands. See Table for exact scores on the measures and more information on how these are interpreted.\nTheresa presented with a long history of recurrent depression and numerous anxiety disorder comorbidities, suggesting longstanding vulnerability that may present a challenge to a brief intervention. Thus, while the formulation that follows focuses on cognitive processes, these are only one part of a clearly complex picture that includes a number of socioenvironmental stressors that could easily contribute to maintaining depressed mood.\nWithin the cognitive framework that had informed the investigation of positive imagery CBM, two processes can be identified that could contribute to the maintenance of Theresa's depression and be targeted by the intervention. First, Theresa demonstrated difficulty in imagining positive events in her future (as indicated by a low score on the PIT). Imagining future events is thought to contribute to individuals’ estimate of the likelihood of those events occurring, and their evaluation of the potential outcomes of a course of action. Thus, being unable to generate positive mental images (which in itself may be exacerbated by or be a consequence of depressed mood) may contribute to a sense of hopelessness about the future (i.e., low likelihood of positive events occurring) and reduce motivation to engage in potentially rewarding activities (i.e., unable to imagine that they might have positive outcomes), reinforcing depressed mood and behavioural withdrawal (Holmes et al., ). For example, if Theresa was to receive an invitation to a social event, she may be unable to imagine positive possible aspects of attending the event, such as enjoying herself or the other guest being very friendly.\nSecond, Theresa demonstrated a tendency to interpret ambiguous information negatively (as indicated by a high score on the SST). Given the inherently ambiguous nature of many of the situations encountered in everyday life, habitually interpreting these in a negative manner, rather than positively or benignly, would also reinforce depressed mood. For example, Theresa may interpret the invitation as meaning “They're just being polite; they don't really want me to come” rather than “They'd like to see me.”\nWithin such a cognitive framework, if a positive imagery CBM intervention could increase Theresa's ability to generate vivid positive mental imagery and train a more positive interpretation bias, then this could help reduce her symptoms of depression by enabling her to imagine positive possibilities in her future, and by increasing the chance that she may interpret everyday events in a positive or benign, rather than negative, manner.\nThe positive imagery CBM intervention as implemented in the trial (Blackwell et al., ) comprised an initial face-to-face introductory session at the research center followed by 12 sessions completed from home over the Internet. Six sessions used audio presentation of training stimuli, in which participants listened to brief descriptions of everyday situations that were structured so that they started ambiguous as to their potential resolution but always ended positively (for example, “As you are getting dressed in the morning, you think about the day ahead. You anticipate it with pleasure and feel full of energy”; positive resolution in italics). Participants were instructed to imagine themselves in each scenario as if actively involved, seeing it through their own eyes, and in particular to focus on the scenario's outcome.\nIn the other six sessions, training stimuli were ambiguous photographs of mostly everyday scenes, paired with a positive caption of a few words. Participants were instructed to generate a positive mental image that combined the photo and the caption. For example, if presented with a photo showing a cloudy grey sky combined with the caption “brightening up,” the participant may generate an image of the clouds clearing and enjoying a sunny day (see Figure ). The training schedule alternated between the audio and picture-word sessions, with six sessions (one each day) scheduled for the first week, and two sessions (one of each type) scheduled for each of the following 3 weeks. Each session comprised 64 training stimuli, arranged into eight blocks of eight scenarios, with a brief self-paced break in between each block (approximately 20 minutes per session), and no stimulus was repeated over the course of the intervention.\nAs in the preceding experimental (Holmes et al., ) and clinical studies (Blackwell & Holmes, ), the face-to-face introductory session started with a standardized introduction to the imagery CBM program, guided by the researcher following a written protocol (available on request from the first author). This included an introduction to mental imagery and practice in generating mental imagery in response to scripts read aloud by the researcher. The introduction aimed to enhance the extent to which participants could feel actively emotionally involved in the mental imagery, for example, via emphasizing the generation of field (i.e., through one's own eyes) as opposed to observer (i.e., viewing oneself from the outside) perspective imagery, and via instructing participants to try to avoid verbally analysing the scenarios (cf. Holmes et al., ).\nIn addition, in order to reduce the risk that the consistently positive nature of the scenarios might lead to participants rejecting them as unrealistic, the fact that the scenarios might sometimes seem unrealistic was explicitly addressed. Participants practiced imagining obviously unrealistic situations, such as winning the lottery or seeing an elephant flying past through the clouds, to illustrate the point that in generating mental imagery, the realism or otherwise of a situation need not be an issue. Once participants had moved to the computer to start the training session, the researcher then sought feedback after each block of eight scenarios. This provided an opportunity to ascertain that participants understood the task instructions and to facilitate their engagement in the subsequent computerized training session.\nThere was a face-to-face outcome assessment at posttreatment (i.e. 4 weeks post-baseline), and follow-up measures were then completed online 1, 3, and 6 months after the end of the intervention. A final feedback interview, including debriefing, was carried out by telephone following completion of the 6-month follow-up questionnaires.\nIn the face-to-face introductory session, Theresa engaged well with the imagery CBM program, although she initially struggled with generating images while listening to audio scenarios. Via the researcher-guided introduction to mental imagery and feedback elicited during breaks (see previous section), the researcher was able to identify that Theresa was struggling and provided suggestions to help her engage in the imagery. This included normalizing the difficulty Theresa was experiencing, reassuring her that the task was in fact challenging and it was thus not surprising she did not find it straightforward. The aim here was to avoid Theresa feeling like a “failure” or becoming disheartened by her difficulty, but rather to see it as normal part of the process and something that could be overcome. Further, examples of where Theresa had been able to generate a more vivid image were elicited and discussed. A strategy developed with Theresa was to stop trying to generate a detailed visual image of every aspect of the scenario as it unfolded, but rather to see the first section of the scenario as “setting the scene” and focus her efforts on more vividly imagining the (positive) outcome of the scenario and the feeling involved in the outcome.\nTheresa completed all 12 online sessions and noticed her mood improving over the course of the 4-week intervention. She felt that she became gradually better at generating positive imagery during the training, and she found herself thinking more positively about events in her life, consistent with the idea of increasing ability to generate positive mental imagery and the induction of a more positive interpretation bias.\nIn addition, Theresa reported some phenomena that did not appear to be accounted for in the initial conceptualization of the imagery CBM intervention as described in the “Initial Case Formulation.” She noticed that generating positive imagery during the sessions provided an immediate (and very welcome) boost to her mood. Intriguingly, this effect seemed to spread into her daily life via the positive images “popping back” into her mind, for example, if triggered by encountering a situation that was similar to one of the training scenarios. The image would not simply be a visual scene, but included some of the positive emotion generated by imagining it during the session, and some of the associated cognition, such as expectation of a positive outcome.\nTheresa reported that sometimes such an involuntary memory of a training scenario could influence her behavior. She provided a particularly striking example of a training scenario that had involved going for a walk and feeling invigorated. On more than one occasion, this image had popped into her mind, bringing with it a burst of positive emotion (experienced as anticipatory pleasure) and prompting her to then go out for a walk. Noticing the beneficial impact that the positive imagery had on her mood and general outlook prompted Theresa to bring some of the positive images generated during training sessions deliberately to mind during daily life to provide a boost to her mood.\nTheresa's scores on the posttreatment outcome measures (Table ) showed marked improvement, including a reduction in symptoms of depression into the “minimal” range, a lower negativity score on the SST, and improved ability to imagine positive events. She felt that the program had helped her “tremendously” and, in fact, wished that she could continue using it (not possible within the constraints of the trial). Theresa reported general increases in behavioral and social engagement–for example, she had made inquiries about joining a local choir and looked into membership of a gym–and this improvement is reflected in increased score on the BADS (Table ). Theresa reported that others had also noticed the improvements. For example, one of her daughters had commented on how well she looked, which had made her notice that she was starting to care about her appearance again, for example, in how she dressed and her use of make-up. At a hospital appointment, where normally she would be very anxious, a nurse had commented on how calm she seemed. Theresa had also stopped drinking alcohol completely and was pleased and surprised that this had been possible.\nTheresa maintained her improvement over the following 3 months of follow-up, with no anhedonic symptoms and high levels of behavioral activation (Table ). Shortly before the final 6-month follow-up, she experienced some worsening of her symptoms, which she attributed to a difficult family situation. However, she felt that she was coping much better than she would have done previously and was pleased because she had maintained her abstinence from alcohol. Theresa reported that she still brought to mind some of the positive images to help improve her mood, and this also helped to stop her from “hitting the bottle.”\nIn her feedback, Theresa was very positive about the imagery CBM intervention, rating herself as “extremely satisfied” and “extremely confident” in recommending it to a friend with depression (in fact, she commented that she had already done so). She rated herself as “extremely likely” to be willing to try the program again if depressed in the future and said that she wished she had access to it at the current time because she thought it would be beneficial. |
A 55-year-old male not known to have any previous medical problems presented with worsening bilateral lower leg pain which started couple days prior to his presentation for the first time in his life. He also reported chronic deformities in his fingers, toes, and nails which were present since his teenage, became worse as he grew up, and later stabilized. He denied any pain related to them until this presentation. He also denied any morning stiffness, hand joints pain, recent diarrhea, fever, or chills. He reported drinking alcohol since the age of 12, and he currently drinks 2-3 cans of beer per day. He had no history of smoking or illicit drug use. Family history was significant for similar hand/nail changes in his mother though he does not remember the age at which it started.\nUpon physical examination, the patient looked well-nourished; his vital signs were within normal limits. He was found to have severe bilateral clubbing of the fingernails and toenails along with osteoarthropathic changes in the hand joints (as illustrated in ). He had severe tenderness at the distal end of his legs, tenderness at the knee joints bilaterally, and mild left knee effusion. He had an antalgic gait secondary to the pain. Heart, lung, and abdominal examinations were unremarkable. There was no lymphadenopathy or hepatosplenomegaly. His skin examination was unremarkable. The patient's blood count and basic metabolic panels were within normal limits. Liver function tests were normal. An x-ray of his legs showed coarse periosteal thickening along the distal ends of the tibia and fibulae (as shown in ); knee and forearm x-rays () showed periosteal reaction along the long bones with left knee effusion. A chest x-ray and CT scan could not identify any lung pathology or masses. Diffuse fatty infiltration of the liver was noted without evidence of cirrhosis. Due to the presence of severe digital clubbing (Grade 4 as it resembles drumstick appearance) and diffuse long bone periostitis with no apparent secondary cause, the patient was diagnosed to have primary hypertrophic osteoarthropathy, which is also called pachydermoperiostosis. He responded well to NSAIDs and was discharged home in stable condition. |
A 48-year-old woman with end-stage renal disease (ESRD) due to chronic glomerulonephritis received a kidney transplant from a donor with DCD (donated cardiac death). The kidney was successfully transplanted to the recipient and normal serum creatinine levels were observed after 7 days. On the fourth day after transplantation, the patient was treated with teicoplanin, cefoperazone, sulbactam and etimicin due to the development of multiple drug resistance Acinetobacter baumannii in both organ preservation solution and drainage fluid. Tigecycline was administered intravenously at 100 mg for the first dose and was given at 50 mg every 12 h from the eighth day after the operation because of persistent abdominal infection. She felt pain at the transplant kidney area and then the blood pressure dropped to 88/61 mmHg on the twentieth day after transplantation. Emergency ultrasound showed two huge hematoma around the graft. Graft pain relieved after emergency treatment including transplanted kidney exploration, renal hematoma removal, renal vascular reconstruction and ureteral re-implantation. The treatment with tigecycline was continued based on the results of the peritoneal drainage, blood and urine culture. The symptoms did not worsen until approximately 15 days after being initially administered. The patient presented with fever, nausea, vomiting and moderate abdominal pain. Physical examination found moderate tenderness in the upper abdomen. Laboratory analyses were remarkable for leukocytosis and the level of lipase raised to 156 U/L. Other results such as serum amylase level was 424 U/L and drainage amylase was 554 U/L, despite the aminotransferase and alkaline phosphatase were within the normal range. CT scans (Fig. ) suggested acute pancreatitis (AP) with grade D on Balthazar score, no necrosis visible without contrast injection. There was no sign of dilated biliary ducts according to the abdominal ultrasound examination. Since those findings were considered to be related to drug-induced pancreatitis, it was recommended that tigecycline should be discontinued on the 16th day following exposure. Shortly after tigecycline discontinuation, the patient’s symptoms gradually improved. Blood amylase and lipase returned to baseline levels in a week. CT scans (Fig. ) showed a basically normal after tigecycline discontinuation for 14 days. She was discharged from the hospital with a low-fat diet for 3 weeks. One month later, abdominal CT scans on follow-up did not find any abnormalities and showed as normal (Fig. ). Throughout the course of treatment, the immunosuppressive regimen was a triple therapy based on recommended doses including tacrolimus, mycophenolate mofetil and prednisone. Serum tacrolimus concentration was maintained at 6–8 ng/ml. The patient received these medications over the next 6 months, with no discomfort and relapse after stopping tigecycline. Timeline of disease was showed in Additional file : Figure S1. |
A 19-year-old man previously diagnosed with anorexia nervosa (he had history of food restriction, rigorous physical exercise programs, induced vomiting and use of laxatives) lost 80 pounds in just 6 months. After that, he suddenly developed acute symmetrical flaccid lower limb weakness that progressed to the upper limbs in a few days, and was associated with paresthesias in all four limbs. At this time, the patient also suffered from abdominal pain, constipation and recurring episodes of ‘red’ urine. He had positive family history of psychiatric conditions (both his mother and maternal grandmother suffered from major depressive disorder), but no family history of neurologic illness. He was then diagnosed with Guillain–Barré syndrome in a different institution, but he refused treatment with intravenous immunoglobulin or plasmapheresis. He was left with severe disability and was wheelchair bound since then.\nAfter 5 months, he was admitted to the psychiatric unit of UNICAMP with a clinical picture of worsening aggressiveness and antisocial personality disorder, which were present (but not treated) since childhood. At the time, the patient was severely disabled with quadriplegia, reduced muscle stretch reflexes, tactile and vibratory hypoesthesia with a distal gradient, combined with altered conscious proprioception. Nerve conduction studies and needle electromyography performed in the acute phase revealed a sensorimotor axonal polyneuropathy. He had normal vitamin B12 levels and there was no albumin–cytologic dissociation in cerebrospinal fluid analyses. Urinary PBG levels were found significantly increased, so that AIP was finally biochemically diagnosed. We opted not to treat him with hematine, because the clinical picture was stable with no signs of recent neurologic decline.\nHe was discharged with optimized psychiatric treatment that included the introduction of selective serotonin reuptake inhibitor (SSRI), as well as individual cognitive–behavioral therapy and dietary guidelines under the supervision of a nutritionist.\nThe patient continues to be regularly monitored at the psychiatry and neurology clinics at UNICAMP. Although there has been no motor improvement to date, the eating disorder is now stable and he had no further attacks of AIP. |
A 30-year-old Brazilian male was brought to our ED from a local jail because of agitation. He had been arrested by Swiss authorities because of suspected internal concealment of drugs of abuse (body packing), which had been confirmed by abdominal CT scan in our hospital approximately 12 h before (Fig. ). During this first visit, the patient had presented asymptomatic and had admitted to carrying cocaine-containing body packets. In addition, he had reported recreational use of marijuana and cocaine. It was also noted that the patient was transsexual; he had breast implants. The patient’s past medical history was unknown. Standard operating procedure in such a case is to perform a CT scan without a consultation at the ED. During the second visit approximately 12 h later, the patient presented with psychomotor agitation, mydriasis and tachycardia. His heart rate was 116 bpm, blood pressure was 116/68 mmHg, respiratory rate was 40/min, oxygen saturation by pulse oximetry was 99% on ambient air and auricular temperature was 38.0° Celsius. Communication with the patient was impossible because of his altered mental status and because he spoke a foreign language. The physical examination of heart, lungs and abdomen revealed no pathologies. Neurological examination showed symmetrical spontaneous movement of all extremities and symmetrical gaze to both sides prompted by speech or touch. Glasgow coma scale was 11. An electrocardiogram showed sinus tachycardia without signs of ischemia. Due to our knowledge of the ingested body packets, we first suspected cocaine intoxication because of package rupture. We treated the patient with repeated doses of intravenous midazolam and performed an emergency abdominal CT scan to guide potential emergency surgical decontamination. The CT scan showed 60–70 packets in the gastrointestinal tract without signs of gastrointestinal obstruction or perforation. Laboratory results showed an increased C-reactive protein (CRP) level (231 mg/l, norm < 5 mg/l), an increased creatinine level (181 mcmol/l, norm 62–106 mcmol/l), an increased creatine kinase (CK) level (1000 U/l, norm < 190 U/l), a slightly increased troponin level (16 ng/l, norm < 14 ng/l), hypoglycemia (2.8 mmol/l), mild hyponatremia (128 mmol/l, norm 136–145 mmol/l), hyperkalemia (5.8 mmol/l, norm 3.3–4.5 mmol/l) and hyperphosphatemia (1.68 mmol/l, norm 0.87–1.45 mmol/l) (Table ). Urine analysis showed no evidence of urinary tract infection. A qualitative urine toxicological test was positive for cocaine and benzodiazepines.\nGiven the clinical presentation of our patient with remarkable hypotension despite cocaine intoxication and elevated inflammatory markers, we suspected infection rather than massive cocaine intoxication to be the main problem. Thorough review of the abdominal CT scans revealed rapidly progressive pulmonary infiltrates in the lower left lobe and lingula, diffuse lymphadenopathy and hepatosplenomegaly (Fig. ). To assess the extent and morphology of the pulmonary infiltrates and to investigate for thoracic lymphadenopathy or solid tumors, we performed a thoracic CT scan. Compared to the abdominal CT scan 4 h before, it showed progression of the pulmonary infiltrates in the lower left lobe and lingula, multiple nodular pulmonary consolidations in both upper lobes compatible with septic emboli or tuberculous foci as well as diffuse cervical, mediastinal and axillary lymphadenopathy.\nDue to the above named findings, we withheld emergency laparotomy and started broad-spectrum antibacterial treatment with ceftriaxone and clarithromycin according to our hospital’s guidelines for severe pneumonia. Given the rapid progression of the pulmonary infiltrates in the course of few hours on the thoracic CT scan, we considered the possibility of toxic rather than infectious infiltrates. Because of the patient’s poor general condition we therefore decided to broaden the antibacterial treatment as for sepsis with unknown focus – especially taking into account an abdominal source – to piperacillin/tazobactam instead of ceftriaxone according to our hospital’s guidelines. Based on the elevated creatinine level we diagnosed acute kidney injury, most probably caused by dehydration precipitated by infection and cocaine intoxication. The patient also had an elevated CK level. Cocaine-induced rhabdomyolysis could therefore be an additional cause of the acute kidney injury. Therefore we treated the patient with intravenous fluids.\nThe patient was admitted to our intensive care unit and isolated for possible pulmonary tuberculosis. In the course of his hospitalization, further diagnostics were available: Pneumococcal urinary antigen test was positive. Screening and confirmatory test for HIV-1 were positive and CD4+ T-cell count was 144/μl, so we diagnosed CDC stage A3 HIV infection. Blood and urine cultures didn’t show any bacterial growth. The diagnosis of pneumococcal pneumonia and sepsis could thus be confirmed, with underlying untreated HIV infection as risk factor. Active pulmonary tuberculosis was excluded by three negative sputum smears and three negative sputum cultures.\nWith the aid of an interpreter, the patient told us in the course of the hospitalization that one of the drug packages had ruptured in his mouth during swallowing, but that he had been able to spit out most of the cocaine. The exact time of this event remained uncertain.\nUpon diagnosis of pneumococcal pneumonia, the antibacterial therapy was changed from piperacillin/tazobactam back to ceftriaxone. As beta-lactam resistant pneumococci were a concern because of the patient’s geographical origin, vancomycin was added to ceftriaxone. This regimen was continued for 7 days. Clarithromycin was stopped after diagnosis of pneumococcal pneumonia. After 9 days, the patient was discharged into custody of the Swiss authorities. Antiretroviral therapy was established in an outpatient setting after ruling out active pulmonary tuberculosis. The patient recovered uneventfully.\nFigure shows the key diagnostic findings and interventions on a timeline. |
A 30-year-old lady presented in the emergency room with swelling and pain of the right shoulder for the previous 4 days. She reported a minor trauma to her right shoulder a week previously, following which she developed swelling and pain, associated with high-grade fever. She was a known diabetic and had been receiving treatment with oral hypoglycaemic agents for the last 5 years.\nOn clinical examination, the patient was toxic, febrile, tachypnoeic and had tachycardia. On local examination there was a diffuse swelling involving the right shoulder, neck and anterior chest wall, and the overlying skin was inflamed. There was local rise in temperature with tenderness and marked induration. There was gross oedema involving the face and the patient had difficulty in breathing.\nContrast-enhanced computed tomography (CT) scan showed extensive cellulitis and thrombosis of the internal jugular vein and marked necrosis of the underlying muscles (Figs and ). The basic investigations revealed a haemoglobin level of 7.5 g/dl, a random plasma glucose level of 227 mg/dl and the fasting glucose level was 240 mg/dl.\nThe total count was elevated to 16 370 mm3 and arterial blood gas showed metabolic acidosis.\nThe patient was started on intravenous piperacillin tazobactam and was subsequently taken for emergency debridement of the wound on the right shoulder. During surgery, as the wound was being debrided, there was practically no bleeding (Fig. ). There was extensive necrosis involving the scapular, neck and facial muscles, although the subcutaneous tissue and the overlying skin looked apparently normal. The tissue was sent for histopathology and, to our surprise, it turned out to be positive for fungal elements. Examination showed acute on chronic inflammatory cell infiltrate, necrosis and splaying of muscle fibres. Many broad aseptate fungal elements morphologically resembling features of the zygomycetes group were seen (Fig. ). Special staining on periodic acid Schiff (PAS) stain also showed clusters of broad aseptate fungal hyphae.\nCulture swab taken from the wound site isolated Klebsiella pneumoniae and Enterobacter cloacae, and the antibiotics were changed to imipenem and cilastatin. As the patient's condition had not improved, despite antibiotic treatment, she was taken for re-debridement under general anaesthetic. The entire dead tissue was removed until active bleeding was seen (Fig. ) and tissue sent for histopathological examination.\nThe patient was started on amphotericin B after the histopathological report confirmed fungal Infection, but she succumbed to the infection after 2 days. |
A previously healthy 5-year-old boy was admitted to our hospital with a short history of a rash and abdominal pain. His medical history was unremarkable, and no drug intake was reported. His parents reported a palpable non-blanching purpuric rash over his lower extremities that had been present for 2 days. The rash extended to the upper extremities, and the patient complained of abdominal pain on the day before admission. Laboratory data obtained on admission showed a hemoglobin level of 12.5 g/dL, a white blood cell count of 11,020/mm3 with a normal differential count, and slightly elevated IgA (211 mg/dL). Other laboratory data were within normal limits. A urinalysis showed no proteinuria or hematuria. The patient was treated with intravenous fluids as conservative treatment under the impression of HSP. After admission, he complained of pain in the right knee and elbow joint with swelling and was started on 1 mg/kg/day of oral prednisolone.\nThe joint and abdominal pain improved by hospital day 17. However, both sides of the scrotum became edematous and tender with a purpuric rash. The penis also showed swelling and a purpuric rash with a focal reddish mass-like lesion at the penile tip. The reddish mass-like lesion caused skin twisting at the base with mild deviation of the penile axis (). The patient did not complain of pain in the penile shaft or in the reddish mass-like lesion at the penile tip. No history of trauma was reported, and no evidence of a urinary tract infection or hematuria was detected. The phallus was uncircumcised, and the patient voided without difficulty or pain and had a normal urinary stream. The patient was referred for emergency scrotal ultrasound examination. On ultrasonography, bilateral scrotal soft tissue thickening was noted. It was more severe on the right side (), and the right epididymis was swollen with increased vascularity, mimicking bacterial epididymitis (-). The size, echogenicity, and vascularity of both testicles were within normal limits. The penile shaft and foreskin were also swollen (), and there was a well-defined hypoechoic mass-like lesion (dimensions, ~1.4 cm×1.3 cm) without vascularity on the penile tip (). This lesion was not connected with the corpus cavernosum, corpus spongiosum, or glans, and seemed to originate from the tip of the foreskin. Although the lesion did not show vascularity in a Doppler study, the patient did not complain of any pain in the penile area or voiding difficulty. Follow-up ultrasonography was recommended under the impression of HSP-related large ecchymosis or purpura with a blister on the penile tip. The edema, purpuric rash, and pain in the scrotum completely subsided as did the reddish mass-like lesion on the penile tip after 2 days of continuous treatment with oral prednisolone. The penile axis also became normal. Followup ultrasonography showed no scrotal or penile edema and no epididymal swelling (), and the hypoechoic mass-like lesion on the penile tip had disappeared (). |
This is a case of a 59-year-old female presented to our hospital with a five-year history of progressively sharp pain and severe tenderness in the tip of her right middle finger. She had numerous visits to different hospitals in the past, with multiple different diagnoses including neuroma, neuritis, Raynaud’s disease, and menopausal syndrome. She was treated with nonsteroidal anti-inflammatory drugs (NSAID), morphine, and anxiolytics, none of which achieved a response. She even was recommended to receive an amputation of the affected digit. The pain was worse at night or during the heavy physical activity. She was unable to sleep at night due to the worsening pain. Sometimes she forced herself to put finger under her body and sleep on the painful finger. She had no trauma history or prior surgery. Swelling and pain with limited motion were observed in the distal interphalangeal joint (DIP) of the affected finger. The distal nail root appeared dense purple in color due to a long soak in the povidone lodine solution (Fig. ). Partial necrosis of skin in the nail root was seen. The physical examination revealed the following findings: The distal and middle finger was red, hot, swollen and painful. There were erythema, edema and warmth of the skin in the middle finger. Positive Love’s test, which identified the exact location of the tenderness by pressing with the head of a pin or paper clip. In this particular case, the Love’s test was atypical. The whole finger was tenderness,and the radial subungual of middle finger was more painful. Positive Hildreth’s test, which showed that pain subsided after a tourniquet was applied to the base of the finger. Positive cold sensitivity test, which demonstrated an increase of the localized pain when her middle finger was exposed to cold water, based mainly on the past medical history. Laboratory studies showed that the neutral granular cell count was elevated, but erythrocyte sedimentation rate (ESR) and high-sensitivity C-reactive protein (CRP) were all within the normal ranges. Plain X-ray films documented some concavity in the distal phalanx with soft tissue swelling (Fig. ). Magnetic resonance imaging (MRI) revealed the bone cortex of distal phalanx in right middle finger was coarse, the boundary was unclear, soft tissue nearby was swollen. An approximately solid, well-delimited subungual nodule with hyperintense on T2-weighted image. Soft tissue of the distal middle finger was swollen with hyperintense on T2-weighted image. (Fig. ). The lateral, radial and dorsal incision was made according to the appearance of MRI and palpation []. We removed the finger nail. The cuticle was partial necrosis. The nail bed and matrix were incised longitudinally, the phalangette was exposed. We found the ruptured glomus tumor, seropurulent pus and inflammatory granulation tissue and we did the debridement in the subungual cavity. The impression of distal phalanx was seen. The nail bed and cuticle were sutured, the wound was closed primarily. (Fig. ). Surgical procedure was carried out with surgical loupes. Patient who suspected glomus tumor, the clinical diagnosis can be confirmed by MRI and clinical examining (as Love’s test, tourniquet test etc). In this atypical case, we hope to order a tissue biopsy to make a definitive diagnosis. We gave up the biopsy in consideration of pin-track infections. Pathologic findings showed a subungual glomus tumor with glomus cells and chronic inflammatory cell infiltrates. Immunohistochemistry results were SMA (+),desmin (−),CD34 (+),caldesmon (+),vimentin (+) (Fig. ). Glomus cells are normally immunoreactive with SMA and vimentin. Then the endothelial markers such as CD31 will be helpful in distinguishing glomus tumor from hemangiomas that are lined with unique vascular endothelial cells.\nAfter surgery, the patient was pain-free and she reported uninterrupted sleep for the first time in 5 years. She took 200 mg of Celebrex, twice a day for 1 week. The patient received Cefuroxime (Glaxo Wellcome Operations, UK; 0.5 g twice a day) for 1 week after the surgery. She achieved a significant recovery and experienced no tumor recurrence in 3 years after surgery (Figs. , and ). These study protocols were approved by the Medical Ethics Committee of the First Affiliated Hospital, College of Medicine, Zhejiang University. |
A 47-year-old female presented with recurrent haemoptysis. She was on treatment for hypertension and type II diabetes mellitus. She had previously been treated for pulmonary tuberculosis on two separate occasions, including the mycobacterium avium complex. She was infected with the human immune-deficiency virus (HIV) and was on antiretroviral therapy with a CD4 count of 201 cells/mL.\nThe computed tomography (CT) scan of the chest revealed a destroyed right upper lobe with bronchiectasis and cavitation. The ventilation/perfusion scan revealed a nonfunctional right upper lobe.\nThe patient was subjected to a right posterolateral thoracotomy and a right bilobectomy. The procedure was complicated by an iatrogenic injury to the right superior pulmonary vein. There was significant haemorrhage following attempts to repair the injury which also involved compression with a small gauze (Ratex) swab. The patient became haemodynamically unstable, acidotic, hypothermic, and coagulopathic and required inotropic support. The operation was stopped and she was transferred to the intensive care unit (ICU) for stabilization. Concluding swab and instrument count in theatre identified a missing small gauze swab.\nIn ICU, X-rays of the chest and abdomen showed a surgical swab in the aorta at the level of the diaphragm (Figures and ).\nThe vascular surgery unit was consulted and noted that the patient was responding poorly to resuscitation. She had absent leg pulses and established calf compartment rigidity bilaterally. Despite reservations regarding the merits of intervention, we were persuaded to attempt removal of the surgical swab using minimally invasive methods.\nIn the operating theatre, the femoral vessels were exposed in the right groin and on-table angioimaging revealed a surgical swab impacted in the abdominal aorta above the level of the renal arteries. Removal of the swab using a filter retrieval catheter set was attempted. Unfortunately, we only managed to advance the swab down to the aortic bifurcation. The swab had consolidated into a solid, clotted mass making it virtually impossible to advance into the right iliac vessels ().\nThe only option would have been an open conversion to surgically remove the swab. The patient deteriorated significantly and she progressed to multiorgan failure. Conversion to an open procedure to remove the swab was thought to be of too high risk. The decision, by consensus, was that any further attempts at salvage would be futile, and the procedure was terminated. The patient was returned to the ICU where she died a short while later. |
A 50-year-old Tunisian man, diagnosed with BD since the age of 29 years, was hospitalized for angina pectoris with a 2 weeks history of anterior chest pain (Canadian Cardiovascular Society class III). The BD diagnoses were based on oral aphta and genital ulcers with a positive pathergy test and repeated episodes of posterior segment inflammation treated by corticosteroids but complicated by complete blindness. The only cardiac risk factor was smoking. Physical examination upon admission revealed a fever of 37.5°C, pulse rate at 90 beats/ min and blood pressure at 120/70 mmHg. Heart sounds and bilateral lung area were normal. The patient erythrocyte sedimentation rate was at 30 mm/hr, C reactive protein level at 6 mg/dl. The protein C, S, homocyteinemia, creatine kinase, serum glutamic-oxaloacetic transaminase, liver and kidney function tests, serum cholesterol and triglycerides were all within the normal limits. Anticardiolipin antibodies and antinuclear factor were negative. An electrocardiogram revealed only sinus tachycardia. Transthoracic echocardiogram revealed no abnormality. On the base of these data, the patient was diagnosed with instable angina. Treatment was initiated with Colchicine, intravenous nitroglycerin, heparine and beta-blocker therapies which lead to a resolution of the symptoms. Six days after hospital admission, the patient underwent coronary angiography, showing one aneurysm (16/8 mm) located on the distal right coronary artery with a tight stenosis located on the proximal part of the posterolateral branch (Fig. ). Heparine therapy was stopped and the following course of treatment implemented: monthly pulse of intravenous cyclophosphamide, methylprednisolone 1g/day for 3 days followed by oral administration of prednisone at a dose of 80 mg/day leading to improvement of all the symptoms. The dose of prednisolone was gradually decreased. Three years later, the patient was referred to cardiac unit with chest pain; coronary angiography showed a total occlusion of the right coronary with athe rosclerotic lesions and well developed collateral circulation. The electrocardiogram showed no signs of myocardial infarction and echocardiography also didn’t show any abnormalities. Desobliteration of the right coronary artery was attempted but unsuccessful. Therefore, no coronary intervention was attempted and the current medical therapy was continued. |
The second patient was a 47-year-old man, a car driver, with no safety belt, victim of car crash with frontal impact on the steering wheel. He reported closed thoracic trauma with several rib fractures, left pneumothorax and diaphragmatic rupture (). On the acceptance at the Emergency Department, he was alert and responsive, with GCS 15; blood values were normal, the patient was dyspnoeic with the request of high oxygen flow, blood oxygen saturation was 78%, blood pressure 90/60 mmHg, hearth rate 125 bpm. Bedside fast ultrasound examination of the thorax documented the absence of pulmonary sliding sign and a pleural particle effusion; a contrast-enhanced CT scan of the abdomen and thorax confirmed a left diaphgramatic rupture () and gastric displacement in the pleural cavity. In this case the placement of a nasogastric tube was helpful to partially relief the symptom related to lung and mediastinal compression by the herniated stomach.\nThe patient underwent to emergent laparotomy: after reducing the herniated stomach into the abdomen, left phrenic rupture was repaired directly with non-absorbable interrupted sutures using polypropylene. The spleen and the other abdominal viscera were undamaged. Two drainages were placed: a left thoracic tube and a drain in the upper left abdominal quadrant.\nThe post-operative course was dramatic with the occurrence of massive haemothorax () in first post-operative day (1500 mL/5 h blood effusion trough the pleuric drainage) that required a second emergency intervention: a thoracoscopic exploration of the left pleural cavity revealed a large hematoma of the anterior chest wall and several pulmonary contusions of the inferior left lobe which was dissected, but the origin of blood spillage remained unclear. Three left thoracic drainages were left in place.\nThe patient was discharged on 37th post-operative day from intensive care unit and he was transferred to a rehabilitation facility for a complete functional and respiratory recovery that he finally achieved after a month. |
A 22-year old woman presented to our clinic with a palpable mass for 6 months. The mass was painless. Her medical history was not remarkable for any disorder. On physical examination she had a palpable mass filling the left upper quadrant and epigastrium. On laboratory examination she had normal levels of total protein, albumin, globulin, alanine aminotransferase, aspartate aminotransferase, blood urea nitrogen, serum creatinine, carbohydrate antigen 19–9 (Ca19-9), carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP). She also had negative serology for hepatitis B and C viruses. On ultrasonography there was a hypoechoic, solid mass with sharp contours and heterogenous pattern which had a size of 16 x 10 cm and diffuse cystic-degenerative areas and which appears hypervascular on Doppler USG (A). The described mass was considered to reside exophytically in the left lobe of the liver. An urgent abdominal tomography showed a giant solid mass that originated from the inferior part of the medial segment of the left lobe of liver and that extended inferiorly. Its size was approximately 17 × 15 x 11 cm. It had smooth contours and marked hypervascularity. It contained diffuse cystic-degenerative areas. A giant hepatic adenoma was primarily considered in the differential diagnosis, which also included liver tumors of mesenchymal origin or hepatocellular carcinoma on a non-cirrhotic basis (B).\nThe patient’s abdominal cavity was explored with a subcostal incision. There was a mass with smooth contours, measuring 15 x 12 cm in the left lobe of the liver, which grew exophytically. Other parts of the liver were normal. The mass’s portion out of the liver was of hypervascular appearance that compressed adjacent tissues but was easily separable from them. The mass was excised with liver tissue and gall bladder, with a negative surgical margin, with the help of an ultrasonic dissector and cautery. There was no additional lesion in the abdominal cavity ().\nThe macroscopic examination of the hepatic resection material revealed a tumoral lesion with a size of 14 × 12 x 13 cm and a cross-sectional color of yellow, which contained diffuse hemorrhagic and necrotic areas, 2 cm apart from the surgical margin. Sections prepared from the tumor showed that it was separated from the adjacent hepatic parenchyma with a clear border but showed infiltration of the parenchyma in a few foci (A). The tumor was highly cellular, the components of which were spindle in shape from place to place and epithelioid in most areas, and they had round-ovoid nuclei and abundant eosinophilic cytoplasm (B). There were interspersed cells that showed nuclear coarsening. Tumor’s background was highly rich in vascularity and there were interspersed free hemorrhagic foci.\nImmunohistochemical study showed negative staining with Pan-CK, Hep-Par, CD117. There was diffuse cytoplasmic positivity with HMB-45 (C) and SMA (D). The background rich vascular network was positively stained with CD34, CD31 and Factor 8 while tumor cells were not. Two mitotic figures were noted under 50 gross magnification. Morphological appearance and immunohistochemical study results suggested a PEComa. Although the criteria for malignancy have not been clearly defined for hepatic PEComas, considering a tumor size greater than 5 cm, presence of more than 1 mitosis under 50 GMA, and infiltrative growth pattern, which have been associated with tumor recurrence or metastatic process for soft tissue or gynecological tumors, the case was considered a malignant PEComa.\nThe patient was discussed in general surgery and oncology councils, which recommend no therapy. The patient recovered uneventfully, and no additional therapy was recommended. She was discharged 3 days after the surgery. She was put under close follow-up; her tri-monthly control tomographic examinations revealed no pathology. She is under follow-up without recurrence 10 months after the surgery (). |
A 12-year-old female patient reported to the Department of Pedodontics and Preventive Dentistry, Rajasthan Dental College and Hospital with the complaint of discolored upper left anterior tooth with recurrent discharge of pus in relation to the same tooth since the last 4 to 5 months. History revealed that she had a fall 2 years back resulting in fracture\nof upper front teeth. Intraoral examination showed 11 with Ellis class II fracture and 21 with Ellis class III fracture and discoloration. An infected sinus was present in relation to 21 with pus discharge (). There was no pain on percussion and no tenderness in the periapical region. Grade II mobility was present in relation to 21, whereas 22 showed grade I mobility. A nonvital response was elicited from 21 and 22 on using thermal and electric pulp tester.\nMaxillary occlusal view revealed incomplete root formation with 21 and an oval shaped periapical radioluscency about 3 × 2.5 cm with clear-cut borders involving the periapical area and apical one-third of roots of 21 and 22 (). The case was diagnosed to be infected radicular cyst involving 21 and 22. Root canal treatment was initiated in 21 and 22, meanwhile periapical surgery was planned as there was constant pus discharge.\nRoutine blood investigations and oral prophylaxis were carried out. Patient was prescribed prophylactic antibiotic a day prior to surgery. A trapezoidal mucoperiosteal flap was reflected using crevicular incisions with limiting vertical incision on distal aspect of 11 and 23 under local anesthesia. Complete loss of buccal cortical plate was observed with 21 (). Complete curettage of the defect including cystic lining was achieved. After thorough curettage of the granulomatous tissue, the area was thoroughly irrigated using betadine solution. The root canal was obturated with gutta- percha points under proper isolation. Retrograde filling was done using glass ionomer cement.\nSlight bleeding was induced from the bony cavity and PerioGlas® (Nova bone) ceramic crystals were directly compacted into the bony defect (). The mucoperiosteal flap was approximated with interrupted sutures. Periopack was applied on the surgical area.\nImmediate postoperative radiograph revealed satisfactory bone fill in the defect area (). Sutures were removed by the end of first week and there was uneventful soft tissue healing. Clinically, by end of first month there was absence of pus discharge and mobility was reduced to grade I in 21 and was absent in 22. At the end of third month mobility in both teeth had completely disappeared. Subsequently, IOPAs were taken at 1 week, 1, 3 and 6 months interval which demonstrated increasing levels of radiopacity signifying successful uptake of graft and positive bone regeneration. One week postoperative radiograph showed increased radiopacity of the material within the bony defect. Radiograph taken in the 3rd month showed a diffuse radiopaque area and increased calcification surrounding the graft (). Radiopacity with foci of pattern progressing from ground glass to spiculed appearance was observed indicating healing calcifying defect at the end of 6 months (). 11 and 23 were tested for vitality using thermal and electric test and both the teeth tested vital during succeeding visits. |
A 40-yr-old man developed end-stage renal disease secondary to chronic glomerulonephritis that had been diagnosed 18 yr before. Thirteen years previously, he had undergone a living-related renal transplantation to the right iliac fossa without complications. The patient was maintained on prednisone and other immunosuppressants such as cyclosporine and mycophenolate mofetil. The transplanted kidney showed excellent function until the diagnosis of AAA. The serum creatinine level was within normal limits during the follow-up period. An abdominal ultrasound for the regular follow-up evaluation was performed in December 2005. The scan revealed an AAA, and subsequent computed tomography (CT) angiography showed an infrarenal AAA measuring 6.4 cm in diameter (). There was no radiographic evidence of leak or rupture.\nOn preoperative evaluations, myocardial perfusion imaging at rest and under stress revealed an abnormal irreversible perfusion defect in the inferior and basal lateral wall. Coronary angiography showed a focal severe stenosis of the proximal left circumflex coronary artery. However, attempts to pass a balloon failed and he was maintained with medical treatment.\nAfter one month of medical management, he underwent an AAA repair with a tube prosthesis and a temporary aortofemoral shunt. Through a transperitoneal approach, the aorta was dissected up to the renal artery without renal vein division. A separate right groin incision was made to expose the common femoral artery. Because the proximal neck of his AAA was enough to place the temporary shunt, cannulation for a temporary shunt was performed at the level of the perirenal aorta; this shunt was placed in the common femoral artery (). A 16-Fr and a 12-Fr aortic perfusion cannula (Edwards Lifesciences, Irvine, CA, U.S.A.) were used for the temporary shunt, and these two cannulas were connected with a 3/8" connector. After confirming this temporary shunt's function, we performed infrarenal aortic cross-clamping. The total aortic cross-clamping time was 34 min and the renal allograft produced urine throughout the AAA repair with a 16-mm Dacron tube graft. The aortic and femoral cannulation sites were simply closed with previously placed purse-string sutures. The patient recovered without any complications, and the immediate postoperative serum creatinine level was 1.1 mg/dL. During the admission, his serum creatinine level remained within normal limits. He was discharged after a 14-day hospitalization, and subsequent CT angiographic images showed a well-functioning aortic graft with a renal allograft (). His renal function has remained stable over 18 months of follow-up. |
A 43-year-old male with spina bifida occulta presented to the emergency department after a traumatic brain injury followed by a fall, without loss of consciousness. The traumatic brain injury was caused by an accident at work, involving storage boxes falling over the patient. He presented severe cervicalgia, anterior cervical flexion, and generalized loss of active movement and sensation. On physical examination, the patient presented reduced cervical range of motion, short neck, low posterior hairline, apparent cervical kyphoscoliosis, and tetraplegia. Cervical kyphoscoliosis had never been investigated or treated previously. Around two hours after the accident, the patient presented hypoalgesia below T2 level. Muscle strength grading according to the AIS was 3/5 in left finger flexors, 4-/5 in left elbow extensors and finger abductors, 4-/5 in left hip flexors and knee extensors, with no other neurological deficits. According to the American Spinal Injury Association Impairment Scale, the patient had a C7 AIS D lesion []. This classification means that C7 is the neurological level of injury, and it refers to the most caudal segment of the spinal cord with normal sensory and antigravity motor function on both sides of the body []. In this case, the patient had a D lesion, with motor function preserved at the most caudal sacral segments (tested by voluntary anal contraction) and had some sparing of motor function in his upper and lower extremities. To be considered a D lesion, the patient must have a muscle grade equal or superior to 3/5 in at least half of key muscles below the neurological level of injury (C7).\nRegarding radiological findings, the cranial magnetic resonance imaging (MRI) was normal. The patient had both thoracic and lumbar abnormalities with incomplete T1 vertebra and spina bifida occulta (L4 posterior vertebral defect). Cervical magnetic resonance imaging revealed important additional findings, which are described in Figures , .\nSurgical cervical stabilization was attempted but the procedure was aborted due to lack of space for placing the anterior plate safely. During the 22 days of hospitalization, the patient wore a cervical collar and started a rehabilitation program including muscle strengthening, balance, and gait training. At discharge, he maintained a slight deficit of strength in his left upper and lower extremities but was able to walk with a walker. At discharge, the patient maintained a classification of C7 AIS D lesion. Rehabilitation treatment continued in an outpatient clinic and included 12 weeks of physiotherapy and occupational therapy sessions three times a week.\nThe presence of spina bifida, scoliosis, hemivertebrae, and block vertebrae led to the following differential diagnosis: anatomical variant, Klippel-Feil syndrome (KFS), Wildervanck syndrome, VACTERL association, and Aicardi syndrome. The patient did not have deafness or strabismus, which excluded Wildervanck syndrome []. Imaging examination did not find tracheoesophageal, kidney, or cardiac anomalies. The absence of cleft palate, limb abnormalities, cardiac defects, or anal atresia excluded VACTERL association []. Aicardi syndrome was excluded because the cranial MRI did not reveal agenesis of the corpus callosum and physical examination did not show any characteristics of Klinefelter syndrome. Aicardi syndrome only affects women or males with 47, XXY chromosome constitution []. The most likely diagnoses were Klippel-Feil syndrome (KFS) or anatomical variant. During hospitalization, the patient was asked to carefully read and sign an informed consent for publication and use of clinical images. Researchers ensured data confidentiality. |
We present the case of a 65-year-old Caucasian woman with no previous history of psychiatric disease until 2012 (60 years of age). At this point, she complained to her family doctor about low mood, reduced energy, and anhedonia. She was prescribed Sertraline 50 mg once a day and maintained the treatment for about 3 years with partial improvement, mainly in the first year.\nAt 63 years of age, she started complaining to her family doctor about nonspecific limb weakness without movement difficulties. A few months later (April 2015), she was admitted to hospital with a myocardial infarction and was discharged 5 days later with full recovery of coronary perfusion. She was followed in cardiology outpatient clinic for about one year and was discharged because of progressive noncompliance with medical treatment and prescribed medical exams.\nDuring the months that followed the myocardial infarction, her depressive and motor symptoms gradually worsened, leading to eleven visits to the Psychiatric Emergency Room Service (ERS), where she was assessed by Psychiatry and Neurology with complaints of anxiety, low mood, social isolation, deficits in memory retrieval, unspecific pain, and limb weakness with progressive development of abnormal gait. She left the ERS before full medical evaluation multiple times and never accurately followed medication changes that were suggested.\nAfter several visits to the ERS, she was referred to Psychiatry and Neurology outpatient clinics. At Psychiatry consultation, she was, once again, medicated for anxiety and depressive mood, with Duloxetine 30 mg and Gabapentin 300 mg daily. One year later, her condition worsened, which motivated addition of Mirtazapine 30 mg and titration of Gabapentin up to 600 mg, again without proper response.\nSimultaneously, she was evaluated in a Neurology appointment because of diminished force and limitation of limb movements. The neurological examination noted unspecific wide-based gait, enhanced bilateral reflexes, and abolished bilateral postural sensitivity. Blood samples showed low folic acid (3,9 ng/mL), which was considered as partial justification for neurological findings and reposition with folic acid was initiated. Additionally, more exams were requested.\nOne month before the admission at our Acute Inpatient Unit (AIU) that leads to the diagnosis of HD, the patient was, once again, evaluated in the Psychiatric ESR. On observation, the patient showed worsening of mood status, increased pressure of speech, pseudo hallucinations (patient described the following: “a TV host speaks with me, he guides me and tells me to do certain things”), and suicidal ideation. She was started on Valproic Acid 1000 mg and Quetiapine 400 mg and Mirtazapine was suspended. However, behavior changes were maintained and even aggravated (she took her clothes off at the window, broke some lamps at home and was aggressive to her sister), eventually leading to the admission to the psychiatric ward.\nThe mental state examination at our unit showed low mood and partial disorientation in time. She was unable to provide dates of important life events saying repeatedly “I don't remember” and scored 14/30 at Mini-Mental State Examination (MMSE). Because of her depressive symptoms and cognitive changes, diagnosis of Major Depressive Disorder/Pseudodementia was assumed and she was medicated with Mirtazapine 30 mg and Quetiapine 200 mg. She was discharged 12 (twelve) days later with improved mood, but she still presented abnormal sustained gait, which was interpreted as a comorbid Conversion Disorder.\nThe Computed Tomography (CT) and Electromyography did not reveal any significant alterations. Therefore, at the subsequent Neurology appointment, patient was discharged with diagnosis of Conversion Disorder.\nAfter hospitalization, she did not follow the prescribed medication and her abnormal behavior was maintained, such as not cleaning her house and throwing clothes away, so she was referred again to the Psychiatric ERS. At the mental state examination she endorsed soliloquies, depressive mood with catathymic delusions, and suicidal ideation and was disoriented to time and place. For that reason, she was readmitted to the Psychiatry ward on March 2017.\nDuring hospitalization the abnormal gait and movements were still obvious; therefore the case was referred to the Neuropsychiatry Team (NT). The NT considered that the psychiatric symptoms of the patient, in addition to the hyperkinetic motor ones, especially in the upper limbs and cervical area (chorea), were in favor of the diagnosis of HD. Genetic test revealed 39 (±2) triplets on one huntingtin allele, confirming the diagnosis.\nThe chorea was controlled with high doses (30 mg/day) of oral haloperidol. The depressive mood and psychotic symptoms remitted with Haloperidol and Venlafaxine. The Venlafaxine was suspended a few days later, because the patient developed moderate maniac symptoms. Later on, for reappearance of low mood, Sertraline was initiated with good response.\nCognitive functions were assessed using the Clock Drawing Test: she was able to draw the contour but could not draw numbers and clock hands. Mini-Mental State (MMS): 14/30 (the same result) and Montreal Cognitive Assessment (MOCA): 7/30.\nAt the moment of discharge to a special unit for rare diseases, she was euthymic, with proper conduct and without choreiform movements, although she still presented residual symptoms, namely, apathy, cognitive impairment, and overvalued ideas of ruin, conditioning severe limitations in her daily life, which made it impossible to live without continuous support. She will maintain follow-up by the Neuropsychiatry Team. |
A 14-year-old female was admitted to the hospital on June 5, 2016, at 2:20 am. The patient’s symptoms started 12 h after clinical manifestation in her sister (Case 1). She had a 39 °C fever and headache progression for 24 h. She presented with psychomotor agitation, followed by generalized tonic-clonic seizures. She was taken to Axochiapan Morelos Hospital for assessment. On admission, she exhibited neurological deterioration requiring orotracheal intubation and was sent to Children’s Hospital of Morelos, where cranial computerized axial tomography (CAT) revealed severe cerebral edema and uncal herniation (Fig. ). Her laboratory blood tests are presented in Table . She was transferred to the intensive care unit where hemodynamic and antiedema care was provided. On beginning intensive care, clinical data indicated brain death, which was confirmed by three isoelectric EEGs. The patient died in the hospital five days after admission. Serology positive for dengue NS1 by ELISA was reported, and PCR was performed to detect enterovirus in the CSF, with negative results.\nBoth patients underwent anti-doping and immunoglobulin testing on admission (Table ). At the time of interview, family members responsible for both patients were asked about any background that might indicate probable toxic poisoning of the patients, but no positive indications were obtained. When questioned about whether there was another member of the family with the same clinical picture, the mother of the children said that on June 3 of the same year, her 6-year-old daughter had a fever of 38 °C. She was taken to a health center in the community of Axochiapan Morelos where paracetamol was prescribed, which improved the symptoms, and no other complications presented. Due to the history of the complications suffered by the patients, serology for dengue NS1 in the 6-year-old child was performed by ELISA, with a positive result.\nIn our country, the ELISA and PCR tests used for the detection of dengue virus are based on the Epidemiological Diagnostic and Reference guidelines of the Department of Health [], performed as follows. |
A 12-year-old boy was hospitalized with complaints of a headache and high fever accompanied by psychosis, delirium, and indistinct consciousness. He was diagnosed with limbic encephalitis, which is an autoimmune disorder characterized by inflammation of the limbic area in the brain. His symptoms became exacerbated, and he required intensive therapies including high-dose steroid and catecholamine administration.\nDespite the continuous therapeutic support mentioned above, he abruptly developed hypotension following diarrhea, fever, and abdominal distension on day 41 after admission. Metabolic acidosis (pH 7.34, base excess − 7.0 mmol/L) was confirmed by blood gas analysis, and highly elevated CPK 11800 U/L, AST 461 U/L, ALT 201 U/L, and LDH 1034 U/L values were revealed by a blood chemistry profile. An emergency CT scan revealed pneumatosis intestinalis localized in the ascending colon and rectum coexisting with portal venous gas (Fig. ). While the root of the SMA and the inferior mesenteric arterial (IMA) flow was maintained, the peripheral blood flow was attenuated adjacent to the non-contrast-enhanced ascending colon and rectum.\nAlthough intraabdominal free air was not detected in the CT scan, the massive ascites and progressing peritoneal signs with muscular guarding required an emergency laparotomy for suspected mesenteric ischemia and bowel perforation. Intraoperatively, skip ischemic lesions were observed in the ascending colon close to the hepatic flexure and the rectum without bowel perforation. Although SMA and superior rectal arterial pulsations were present, the marginal perfusion near the two lesions could not be confirmed. The patient was diagnosed with NOMI based on these operative findings and the rapid progression of the symptoms, which are unlike other vascular disorders or necrotizing enterocolitis. The remaining colon, from the transverse to the sigmoid colon, appeared intact. The color of the unaffected intestinal wall was restored, which suggested intestinal viability (Fig. ). We performed a distal ileostomy without bowel resection because a second-look laparotomy after 24 to 48 h was considered.\nAfter returning to the ICU, the patient required resuscitation for cardiac arrest, septicemia, and DIC. The scheduled second-look laparotomy was canceled, and intensive care including hemodiafiltration was continued. However, the gastrointestinal symptoms did not progress during the intensive treatment.\nOn the 16th and 60th postoperative days, colonoscopies were carried out, and they revealed rectal and ascending colon stenosis with ulceration (Fig. ). The patency was 5 mm in diameter at both strictures. However, normal findings in the transverse colon to the sigmoid colon were observed by colonoscopy.\nA lower gastrointestinal series by gastrografin contrast radiography also demonstrated the patency of the two lesions after laparotomy (Fig. a). Based on successful evacuation of the contrast media and intact mucosal findings around the mild stricture, we scheduled ileal stoma closure. For 1 month prior to the closure, approximately 100 ml of bowel contents that had collected in the ostomy pouch were injected into the anal side of the ileostomy to induce efficient bowel movement. We confirmed the continuous expulsion of feces from the anus and the improvement of transient bowel strictures; therefore, the ileal stoma was closed 14 months after the previous laparotomy.\nCurrently, the patient’s confusional state has prolonged, and he has received enriched liquid nutrition via gastrostomy. The two stenotic lesions are completely resolved, and defecation has been maintained after stoma closure (Fig. b). |
A 20-year-old male presented to the clinical center in the village with a complaint of pain in the lower back spreading over the left lower limb with paresthesia mimicking a lumbar disk hernia. He was given analgesic drugs that eased the pain several weeks.\nThe patient then noticed a mass bulging from the left lower quadrant of the abdomen and was referred to the hospital by the family physician. He didn't have any other symptom or a significant past medical history. Familial history included hypertension and diabetes mellitus. Physical examination showed a mass in the left iliac region. A CT scan showed a huge lytic mass (longest diameter: 19 cm) occupying the left iliac fossa and pushing the adjacent structures. Some erosion was evident in the iliac crest (see ).\nThe laboratory tests showed an elevated ESR (68 mm/h), elevated INR (1.85), low albumin levels (2.5 mg/dl) with otherwise normal values.\nThe patient refused to perform a biopsy, so an instructor surgeon in the general surgery department performed an exploratory laparotomy. The tumor infiltrated the local structures and the spinal nerves (which explains the previous presentation), so a complete resection was not possible. We performed a debulking procedure of the tumor and sent a biopsy to the pathology. The tumor was so infiltrative that we couldn't specify whether it originated from the iliac crest or from the retroperitoneal soft tissue (see ).\nThe microscopic examination showed malignant proliferation of solid nests and cords of small compact oval and round tumor cells, with wide areas of necrosis. A final diagnosis needed an immunstaining. Current Stains were used: CD99, LCA, CD79 and CD34. CD99 was positive and the final diagnosis was Primitive NeuroEctodermal Tumor (PNET).\nWe consulted the oncology department and transferred the patient to adjuvant therapy; which included a combination of chemotherapy and radiotherapy. The patient took 4 sessions of chemotherapy (mesna, etoposide and cyclophosphamide) followed by 50 Gy of radiotherapy divided to 25 sessions, each of 2 Gy.\nAfter the completion of the treatment, a multislice PET/CT scan showed a few small active remnants around the iliac bone without any nodal or visceral involvement.\nThe patient is followed up by CT scan every 4 month. The last follow up CT scan, after two years of diagnosis, showed complete remission without any evolvement of the tumor compared to the previous images (see ). |
A 61-year-old male patient complained of progressive decrease in visual acuity over 7 months before admission, worse on the left side. He also reported increased urine output for 3 months. His medical history included a left lung lobectomy for lung cancer 25 years earlier. Neurological examination did not show any other abnormalities. Laboratory and endocrinological investigation were normal.\nMagnetic resonance imaging (MRI) revealed a mixed solid-cystic lesion measuring 3.1 × 2.2 × 2.9 cm and appearing with intermediate signal on T1 and T2 sequences and with avid postcontrast enhancement after application of i/v gadolinium. The lesion was centered in the suprasellar cistern at the expected site of pituitary stalk and compressed and displaced the optic chiasm. The tumor extended superiorly toward the floor of the third ventricle, compressing the hypothalamus bilaterally, without an apparent discernable tissue plane separating it from adjacent structures [].\nPosteriorly, the lesion displayed intimate contact with the pituitary stalk and extended into the interpeduncular cistern, displacing the mesencephalon posterior-inferiorly. On T2 and FLAIR sequences, there was increased signal intensity along the pathway of the optic tract and in the hypothalamus which was interpreted as edema. The usual T1-signal increase of the neuropituitary was not visualized and the appearance of adenohypophysis was normal.\nThe patient underwent open microsurgery through a left pterional approach. The lesion was found to be firm and could not be aspirated by suction-irrigation and was rather hypervascular, leading to significant intraoperative blood loss. The tumor appeared to have originated from the pituitary stalk which was infiltrated. The chiasm was elevated and the left optic tract was displaced laterally by the tumor. A good cleavage plane could be identified, and after subtotal removal (limited by hypothalamic infiltration and involvement of perforating arteries of the posterior communicating and the posterior cerebral artery), we achieved a substantial decompression of the optic pathway. The postoperatively period was uneventful, except some transient diabetes insipidus which resolved spontaneously by the fourth postoperative day []. Late postoperative MRI (at 3 months) revealed some residual tumor but adequately decompressed optic pathways []. At this time, campimetry by confrontation test revealed partial recovery in bitemporal hemianopsia, which was still worse at left.\nMacroscopic pathological examination revealed a lesion of whitish-brown discoloration with an elastic consistency. Histopathology revealed a glial neoplasm with an epithelioid pattern. The specimen showed moderate cellularity with sometimes rounded or elongated nuclei and a broad eosinophilic cytoplasm. Absence of cellular pleomorphism, mitotic figures, and any areas of necrosis was noted. The immunohistochemical staining profile showed positivity for GFAP, S-100, Vimentin, TTF-1, ATRX, and BCL-2. Staining was negative for EMA, Synaptophysin, P53, HDI-1, NEUN, and CK8/18; The cell proliferation index (estimated by Ki-67) was low at about 1% []. |
A 43-year-old male patient attended a dental clinic with the prosthetic crown of tooth 24 in hand, with the remaining fractured root core, part of which was inside the root canal (Figures and ).\nClinical and radiographic examination revealed the absence of a root fracture, which might preclude maintenance of the tooth (). It was also observed that the root canal had not been submitted to removal of sealing material up to the adequate length of 2/3 of the dental remnant to the root apex [, ]; the cast metallic core was short, which impaired the intraradicular retention.\nDuring clinical examination, it was observed that the dental remnant presented satisfactory conditions for a new rehabilitation with placement of an intraradicular core and a total prosthetic crown. It was proposed to remove the portion of the cast core that was inside the root canal for later accomplishment of a new intraradicular cast core, using the existing metal-ceramic crown as a reverse template for the coronal portion of this future core.\nThis alternative was possible because there was no need for additional preparation (wear) of the dental remnant at the cervical level, which would impair the adaptation and reuse of the original prosthetic crown. Initially, root canal preparation (buccal and palatal) was performed by instrumentation with Gates Glidden drills at the appropriate length (2/3 of the dental remnant in the largest (palatal root canal), 1/2 of the dental remnant in the other canal (buccal)) [, ].\nThen, the prosthetic crown was internally ground with carbide drills to allow coronal adaptation of the new cast core. For that purpose, the prepared root canals were previously isolated with petroleum jelly and filled with fluid acrylic resin, supported by prefabricated resin rods (Pincanal®) in each canal. Polymerization of the acrylic resin was properly controlled to avoid adhesion of the resin pin inside the root canal ().\nFor preparation of the coronal portion of the core, the prosthetic crown was fitted on the coronal remnant and was internally isolated with petroleum jelly, filled with acrylic resin, and repositioned on the dental remnant with the core in place, thus serving as a reverse template for reconstruction of the coronal portion of this new core (Figures –).\nAfter completion of polymerization, the finished resin core was sent to the prosthesis laboratory for alloy casting (Figures and ).\nThis new core was properly adjusted to the remaining root, and the adaptation of the metal-ceramic crown on it was also verified (Figures –).\nFinally, both pieces (core and crown) were cemented with definitive zinc phosphate cement (Figures and ), and the occlusal contacts were adjusted (). |
During anatomic study of the abdominal cavity, additional renal branches from the aortic axis were revealed in a 97-year-old male Caucasian cadaveric donor. Continued dissection outlined notable anatomic variations in the arterial, venous, and ureteric patterns. No urologic or cardiovascular issues were reported by the donor or family at the time of enrolment in the Deeded Body Program. For this type of study, Institutional Review Board approval is not required for research conducted with cadaveric specimens.\nBoth kidneys were retroperitoneal and similar in size with measurements of 12.3 cm (l) and 12.7 cm (r) in the craniocaudal direction. Despite the fact that the lengths were similar, there were marked differences in the relative positioning of the superior and inferior poles. The superior pole of the right kidney was situated more superiorly. The inferior pole of the left kidney was positioned near the superior border of the left common iliac artery. Although each kidney occupied an extended volume, neither kidney had a pelvic component. Hilar structures entered/exited the organs anteriorly, not with the typical medially projected hilum ().\nA total of five major arteries (3l and 2r) emerged from the aortic and common iliac axes (Figures and ). The superior left renal artery originated from the abdominal aorta and supplied the superior pole. This artery (5.2 mm diameter) branched into two vessels of similar caliber to supply the upper third of the organ. Supply to the left gonad originated from the more inferior of these two branches (Figures and ). The middle left renal artery (6.1 mm diameter) originated from the iliac junction, just anterior to the median sacral artery (Figures and ). The inferior left renal artery (5.4 mm diameter) originated from the common iliac artery, and coursed posteriorly to the kidney before entering the hilum anteriorly (from the lateral aspect of the organ), to supply the inferior pole (Figures , , and ). On the contralateral side, the vasculature to the right kidney consisted of only two renal arteries, both originating from the abdominal aorta. The superior renal artery (6.2 mm diameter) branched laterally from the aorta (at the same level as the left superior renal) and supplied the superior pole. As with the contralateral side, this artery also branched into two vessels of similar caliber, but supplied the superior half of the organ (Figures and ). In contrast to the contralateral side, the right gonadal artery branched directly from the aorta at its generally observed position, just inferior to the (superior) renal artery. Arterial supply to the inferior pole (6.5 mm diameter) originated on the lateral aorta, at a level inferior to the inferior mesenteric artery, and branched into two arteries (Figures , , and ).\nMultiple contributing veins, of different caliber, coalesced into four major renal veins (2l and 2r) that returned blood from the kidneys to the IVC (). The relative positioning of the superior renal veins followed the conventional pattern, entering the IVC through single vessels at a level just inferior to the superior mesenteric artery as it emerged from the aorta. On the right side, three major veins merged into a short segment (3 mm in length) to drain the superior 2/3 of the organ. The inferior 1/3 of the organ was drained through two primary contributors that merged into a single vein that ultimately drained into the IVC at the level of the previously described right inferior renal artery. A visible and substantive anastomotic connection was evident between the superior and inferior venous pathways (). On the left side, the superior renal vein received contributions from the suprarenal gland and the superior half of the kidney. Venous return from the left gonad ultimately merged into the most inferior branch of the three primary contributors to the superior renal vein (Figures and ). The inferior renal vein drained directly into the anterior aspect of the IVC at the junction of the common iliac veins, posterior to the bifurcation of the aorta. The most inferior of the three primary contributors to the inferior renal vein emerged from the posterior lateral aspect of kidney and spanned the hilum to ultimately converge into the inferior renal vein. The middle contributor emerged from the parenchyma, but was unremarkable. As on the contralateral side, a visible and substantive venous anastomotic connection was evident between the superior and inferior aspects of the kidney ().\nUnilateral duplication of the kidney with concomitant ureters was evident for the right side (). Aligned with the arterial pattern of this organ, the superior ureter demonstrated a discreet collecting system with well-formed minor and major calyces draining into a defined renal pelvis. The majority of the superior renal pelvis was positioned posterior to the vasculature (Figures and ). At the inferior portion of the kidney, the hilum was more anteriorly directed with the calyces and renal pelvis more evident given their anterior position relative to the vasculature. Both ureters continued inferiorly and independently to enter the bladder (). Histological investigation of the ureterovesical junctions was not completed; however a distinct orifice near the bladder trigone was evident for each ureter. On the contralateral side, the single ureter emerged from a widened and anteriorly projected hilum (). Major calyces from the peripheral parenchyma were evident and these calyces contributed to an elongated renal pelvis. The remaining course of the left ureter was unremarkable. |
An 86-year-old female with a past medical history significant for hypertension, congestive heart failure, aortic and mitral valve regurgitation status post repair, transient ischemic attack without residual neurologic deficits, and atrial fibrillation not on anticoagulation presented to an urban, academic emergency department (ED) with acute onset bilateral lower extremity pain, weakness, and paresthesia. The symptoms began two hours prior to arrival. The patient denied abdominal pain, back pain, fever, and recent trauma.\nThe patient arrived in moderate distress with an irregularly, irregular pulse of 65 beats per minute and a blood pressure of 187/109 millimeters of mercury. Other vital signs and the patient’s blood sugar were within normal limits. During initial triage, physical examination revealed new onset right (4/5) and left (2/5) lower extremity weakness. Neurological exam was otherwise unremarkable. A stroke alert was initiated immediately. CT head revealed no acute pathology. Upon return to the ED, the treating physician noted cool extremities with absent bilateral dorsalis pedis and posterior tibial pulses. Abdominal exam was benign. No rash or signs of trauma were evident. We then considered alternative etiologies of our patient’s bilaterally lower extremity weakness, including aortic pathologies.\nPOCUS of the abdominal aorta demonstrated an occlusive intraluminal echogenicity originating just proximal to the iliac bifurcation ( and ). Vascular surgery was consulted immediately for AAO. Emergent CT angiography of the inferior abdomen and bilateral lower extremities confirmed the aortoiliac occlusive thrombus (). The soft tissues demonstrated no evidence of myonecrosis. Laboratory values were unremarkable.\nThe patient was taken directly to the operating room where she underwent successful thrombectomy of the aorta, iliac, and femoral arteries. A transesophageal echocardiography did not demonstrate left atrial or ventricular thrombus. The patient had an unremarkable hospital course. Upon discharge to a skilled nursing facility, distal lower extremity pulses were present on Doppler exam, and the patient was ambulatory with mild residual bilateral lower extremity weakness. |
A 58-year-old female presented to our hospital with a four month history of right ocular pain that had progressively worsened. Two years prior, she underwent cataract extraction with IOL implantation, and during the first year postoperatively, she received panretinal photocoagulation therapy. One year ago, she underwent a pars plana vitrectomy for proliferative diabetic retinopathy. The patient was diagnosed with diabetes 17 years ago, but otherwise did not have a significant past medical history.\nAt the initial examination, the best corrected visual acuity of the right eye was 0.08, and the left eye was 0.3. The IOP of the right eye was measured using the Goldmann applanation tonometry and was 35 mmHg; the left eye was 14 mmHg. On slit lamp examination, new vessels on the iris surface were detected, and in the anterior chamber angle examination, new vessels had formed. Due to the continuous proliferation of the fibrovascular membrane, closure of the right anterior chamber angle was detected. Topical beta-blockers and topical carbonic anhydrase inhibitors (Cosopt®, MSD, USA) were continuously administered, and photocoagulation for new vessels in the anterior chamber was performed. However, the ocular pressure could not be controlled; hence, an Ahmed glaucoma valve implantation was performed.\nSurgery was performed by the method identical to the Case 1. In this case, however, due to the small size of her pupil, we examined the location of tube by using a Hirschman iris hook (Bausch & Lomb, Inc. St. Louis, Missouri). Four months after surgery, a slightly elevated IOP level at 20-22 mmHg was maintained. Five months after surgery, the visual acuity of the right eye was 0.08, and the IOP of the right eye stabilized to 17 mmHg. On slit lamp examination, the result of the regression of new vessels in the iris surface was detected (). Ten months after surgery, during the patient's final visit to our outpatient clinic, her visual acuity had not improved, but she did not have hypotony, hyphema, choroidal detachment or other complications, and her IOP was well-maintained at 12-17 mmHg. |
A 57-year-old woman presented to our hospital because of dry cough, intermittent chest wall and back pain for 3 weeks. She complained the pain gradually aggravated but did not influence the length of sleep bouts. Simultaneously, the level of daily physical activity was significantly decreased, with a 3kg unintentional weight loss over the past month. She denied tobacco and tuberculosis exposure history. Physical examination suggests obvious swollen bilateral supraclavicular lymph nodes.\nA chest enhanced computed tomography (CT) scan revealed over 200 uniform size pulmonary nodules in an evenly dispersed pattern at bilateral lungs with a 38×45mm new creature at the dorsal segment of the lower lobe of the left lung (–), in addition to a small amount of left-sided pleural and pericardial effusion. Interestingly, the artificial intelligence (AI) system (The AI software supported by Yitu CT, YITU Healthcare Technology Co., Ltd., China. The AI system of deep learning-based quantification was performed using a novel established lung nodule net based on one automatic segmentation software. This module was developed as a combination of U-net and fully convolutional networks. Approaches for lung nodule segmentation involved the detection of a volume of interest (VOI) covering the nodule area and segmentation inside this VOI., These methods can be generally classified into machine-learning methods based on China big date, which covered more than 10 provinces and more than 50 hospitals), only identified 18 pulmonary nodules (), which are lower than 10% of the real data. Besides, all of these were judged as low-risk nodules. Another enhanced CT scans showed multiple liver and adrenal metastases, multiple bone metastases (including thoracic spine, sternum, ribs and left scapula), as well as apparent lymphadenopathy in the left hilar, bilateral mediastinal and retroperitoneal area. Magnetic resonance imaging (MRI) revealed multiple osteolytic bone destruction in thoracic vertebrae and a T7 vertebral body’s compression fracture. T-SPOT was negative while a significant elevation of the serum tumor markers (including CEA 839.30 ng/mL↑, NSE 60.38 ng/mL↑, Cyfra 21-1 20.11 ng/mL↑, CA125 466.70 U/mL↑, CA153 181.00 U/mL↑, CA199 74.16 U/mL↑ and CA724 151.40 U/mL↑). Biopsy of the left supraclavicular node confirmed metastatic adenocarcinoma () with supportive immunohistochemical staining reaction: positive for TTF-1 (), PCK (), CK7, but negative for P40, Syn, and the Ki 67 index was reported in 70% (). The peripheral blood gene detection showed wild-type epidermal growth factor receptor (EGFR) with no gene mutation sites. In light of clinical and instrumental examinations, the diagnosis of lung adenocarcinoma (T4N3M1 stage IV), EGFR-negative, was made.\nThis patient lost the chance of surgical treatment due to the extensive metastatic transfer of primary tumors to distant organs. The negative gene detection result indicated that she could not benefit from target therapy. We recommended a combination therapy regimen, the subsequent imaging revealed a significant decrease in the size and number of primary tumors and metastases (). |
A 6-month-old female entire domestic shorthair cat was presented to the referring veterinary surgery for neutering. Following an attempt to give parenteral premedication with medetomidine and butorphanol in the neck region of a non-compliant cat using a syringe with a 23 G × 16 mm needle, the cat collapsed, became instantly non-ambulatory and was referred. It was reported that none of the premedication was injected, as the same volume remained in the syringe after the injection attempt. The cat was reported to be previously healthy. Vaccination and worming status were current, and the cat was housed strictly indoors.\nOn referral, physical examination did not reveal any significant findings other than a small erythematous skin lesion at the injection site in the right cranial cervical area. Neurological examination revealed mild obtundation with intermittent horizontal nystagmus, decreased bilateral nasal stimulation response and ptosis of the right upper eyelid. The cat displayed non-ambulatory right-sided hemiparesis. Paw replacement, hopping, tactile and visual placing on the right thoracic and pelvic limbs were absent. There was increased muscle tone on the left thoracic and pelvic limbs, and a decreased withdrawal reflex on the right thoracic limb. The cutaneous trunci muscle reflex (CTR) was absent. There was no evidence of pain on palpation of the vertebral column or on passive flexion of the head and neck.\nBased on the neurological findings, a multi-focal neuroanatomical localisation affecting the brainstem (the vestibular nuclei, the vestibulospinal tract, the sympathetic innervation to the eye, the ascending reticular activating system and the proprioceptive tracts) and C6–T2 spinal cord segments or a single brainstem lesion with spinal shock was suspected. The CTR can be absent in neurologically normal cats. The response to stimulation of the nasal mucosa is a cortically mediated withdrawal of the head and was thought to be absent as a result of impairment of the ascending reticular activating system. Main differential diagnoses included a traumatic iatrogenic lesion, vascular accident or inflammatory disease.\nComplete blood count and serum biochemistry were unremarkable. Prothrombin and thromboplastin times were normal. Serial blood pressure measurements were within the normal interval. Dynamic radiographic study (flexed and extended views) of the cervical vertebral column obtained under general anaesthesia did not show any abnormalities.\nMagnetic resonance imaging (MRI) of the cervical vertebral column and the brain was performed using a 1.5 Tesla unit (Siemens Magnetom Essenza; Siemens). A well-defined, 2.5 mm in diameter, rounded intra-axial lesion was identified at the right ventro-lateral aspect of the myelencephalon. The lesion was hypointense on T2-weighted (T2W) and iso- to hypo-intense on T1-weighted (T1W) images when compared with normal grey matter. A rounded signal void corresponding to the lesion described above was identified on T2*-weighted images. A perilesional rim hyperintensity on T2W and fluid-attenuated inversion recovery images was also observed ( and ). There was no contrast enhancement after intravenous injection of gadolinium (0.1 mmol/kg, Gadovist; Bayer). A caudodorsal to cranioventral linear hyperintense lesion was observed in the muscles of the right dorsolateral aspect of the neck at the level of C1–C2 on parasagittal short tau inversion recovery images in the direction of the cisterna cerebellomedullaris (CMC) (). The above description was consistent with a haemorrhagic brainstem lesion associated with perilesional oedema. Changes observed in the musculature of the neck were compatible with a needle track. Cerebrospinal fluid (CSF) collection was not attempted.\nThe only treatment consisted of fluid therapy, and three consecutive anti-inflammatory doses of dexamethasone to reduce perilesional oedema (0.1 mg/kg IV q24h, Dexadreson; Intervet). Clinical signs gradually improved during hospitalisation and the cat regained ambulation but remained hemiparetic and ataxic by day 4. An intermittent wide base stance was observed during recovery. At the time of discharge, on day 7, the cat remained ambulatory hemiparetic, but postural reactions had improved and withdrawal reflexes and cranial nerve examination had normalised. The cat was re-examined a month and a half after diagnosis. A mild residual right hemiparesis with a mild decrease in right postural reactions were the only abnormalities observed. The owner reported that the cat was able to jump normally on to furniture and a gradual improvement had been observed. |
A 20-day-old male, born prematurely, with a palpable round lump on the upper left eyelid was presented to us for consultation by the department of pediatrics. The upper eyelid was closed due to the mass and could not be opened nor everted. There was no change in the color or the size when the patient's head was placed in a dependent position or when the infant cried (). His mother was in good general health and had not taken any medications during pregnancy. Examination of the infant showed no other congenital malformations.\nUltrasonography (US) revealed a 1.1×1.3 cm round anechogenic cyst in the medial portion of the left orbit. It displaced the left eyeball to the posterior lateral portion. (). Magnetic resonance images (MRI) of the orbits revealed a 1.4×1.3 cm round cystic mass with a low signal intensity in T1WI and a high signal intensity in T2WI. The left eyeball was compressed and displaced to the inferior-posterior portion of the eye socket by the mass. Although the mass was bound by the intracranial and nasal cavities, it was observed that the mild stimulation of the left optic nerve caused slight inferior displacement of the medial rectus muscle ().\nSince there was visual axis encroachment in conjunction with eyeball displacement, surgical intervention was scheduled. The mass was excised via an approach through the skin. It was found to be attached with two stalks to the upper eyelid where it was carefully dissected and removed (). The specimen was a grayish polypoid mass with fibrous band-like stalks and filled with a clear fluid. Histopathologically, the specimen consisted of a solitary cyst with a very thin cystic wall (). Microscopically, it was lined by a double layer of non-keratinizing cuboidal and/or flattened epithelium, with features identical to the glands of Moll. The innermost cells displayed a granular and distinctly eosinophilic cytoplasm with apical expansions, while the outer wall was composed of a fibrous tissue. The diagnosis of a sudoriferous cyst was thus confirmed ().\nThe patient underwent a comprehensive ocular examination one week after the operation. Since there appeared to be little fixation or following efforts towards the mother's face or a hand at near distances with the left eye, the patient was prescribed 30 minutes of occlusion by patch per day. Esotropia was then able to be diagnosed. The angle of deviation was 30 prism diopters (PD) by the Krimsky method. Even with intensive occlusion therapy, examination at four months showed no interval change for fixation and an abduction limitation on left eye was found. Seven months after the operation, occlusion therapy was ceased because of a newly developed poor response to visual stimulation on the right eye, probably due to occlusion time in excess of the prescribed treatment. The angle of deviation was then found to be 50 PD by an alternative prism cover test at a near distance. US revealed no abnormal echogenecity in the left orbital area and no movement during scanning on the left eyeball which was deviated medially (). Nine months after the operation, 30 PD esotropia was determined by the Krimsky method and an abduction limitation was found in both eyes (). Computed tomography (CT) of the orbits revealed no eyeball deformity with normal extra-ocular muscle structure and normal peri-orbital tissue (). |
A 64-year-old Caucasian man visited our out-patient department with symptomatic anteromedial osteoarthritis of his right knee. A physical examination revealed a full range of motion and stable collateral and cruciate ligaments. Plain radiography showed an anteromedial osteoarthritis grade III [] (Figure ). After an initial conservative approach, which included a 1-year period of physiotherapy, non-steroidal anti-inflammatory drugs and three hyaluronic acid injections, he was planned for a UKA (Oxford® Partial Knee).\nPreoperatively, a medium-size femoral component was templated. The surgery was performed by a specialist arthroplasty surgeon. During surgery, a femur of medium size, a tibia size C and an insert size 3 were placed and no complications were experienced. The preoperative diagnosis of anteromedial osteoarthritis was confirmed and the anterior cruciate ligament was found to be stable. The time of blood void was 63 minutes and the bone quality of both tibia and femur was assessed as good. Pulsed lavage was applied to increased femoral cement penetration in combination with the application of retention holes to further enhance cement fixation. Range of motion of the knee after wound closure was 120 degrees of flexion and full extension with stable collateral and cruciate ligaments. The postoperative X-rays revealed no abnormalities and proper positioning of the UKA (Figure ). The postoperative checks at the out-patient department were planned 6 weeks and 3 months postoperatively. At both appointments the patient showed a good clinical recovery and his range of motion observed at 3 months was 120/0/0.\nTwo weeks after the last check he presented at the out-patient department with extreme pain and a locked knee. There was no trauma; however, he reported an acute pain after sitting in a cross-legged position. A physical examination showed that he was in pain with a locked knee in 90/90/0. An X-ray showed a fully migrated and loosened femoral component (Figure ).\nDuring revision surgery, performed by the same surgeon, a crack in the cement of the femoral component was observed (Figures , and ). No other signs were found that could explain failure of the prosthesis. Cultures and biopsies of the medial femur condyle were taken for further examination; however, bacterial infection or osteonecrosis was not found. The tibial component was found to be solid. During revision surgery, a total knee arthroplasty (Genesis II Total Knee System, Smith & Nephew®, Memphis, USA) with a femur size 7 posterior stabilized component was placed in combination with a tibia size 6, an insert size 15 and patella size 26. During surgery no complications were experienced. His range of motion after wound closure was 120 degrees of flexion and full extension with stable collateral ligaments. The postoperative X-rays revealed no abnormalities and a proper positioning of the total knee prosthesis.\nHis recovery after revision surgery was without complications. During the last appointment at the out-patient department, 1 year after revision surgery, he showed a good clinical recovery and the range of motion observed was 120/0/0. |
The patient is a 25-year-old African American male who initially presented with right eye pain and bilateral blurry vision. A month later, he was seen by a retinal specialist and underwent fluorescein angiography that revealed bilateral serous retinal detachments. He was diagnosed with VKHD and was started on prednisone 60 mg daily, with a plan to taper by 10 mg over the course of four weeks.\nHe was initially compliant with his steroid regimen, but due to symptoms of anxiety and jitteriness, he stopped taking it after three weeks. Subsequently, his vision worsened, and a few days later, he developed right eye ptosis, nausea, vomiting, headache, and difficulty swallowing liquids.\nHe presented to the emergency room a few days after. Examination revealed right eye ptosis with a 7-mm unreactive pupil and limited movement in all directions as well as impaired abduction of the left eye. He underwent MRI brain, MRA head and neck, and MR orbits, although only non-contrast images were obtained due to patient anxiety and body habitus, and all imaging sequences were unremarkable. Prednisone was recommenced 60 mg daily.\nAbout two days later, he was run as a stroke code with emergent imaging for new-onset tongue deviation, slurred speech, and diminished hearing in his right ear. MRI brain with and without contrast noted extensive cranial nerve thickening and enhancement, including bilateral cranial nerves II and III, left cranial nerve IV, bilateral cranial nerve V, left cranial nerve VI, right greater than left cranial nerve VII and VIII, and left cranial nerve XII (Figures -). Differential diagnosis at this time included progression of VKHD, infectious or inflammatory granulomatous processes, neoplastic involvement, or an idiopathic inflammatory polyneuropathy with cranial nerve involvement such as Miller Fisher variant versus a hereditary polyneuropathy or neurofibromatosis.\nHe was newly diagnosed with HIV based on positive HIV1 antibody titer, with 63,000 copies/mL and CD4 count 41 cells/μL. Further infectious workup returned positive for Hepatitis A, with negative antibodies for CMV, HSV, VZV, Hepatitis B, toxoplasma, coccidioidomycosis, aspergillus, gonorrhea, and chlamydia, as well as negative RPR and quantiferon. Lumbar puncture was performed and revealed glucose 62, protein 195, WBC 83 with 94% lymphocytes, and RBC 1460. CSF was negative for oligoclonal bands, with normal MBP and IgG index. CSF cytology was negative, and flow cytometry was not obtained. CSF infectious workup was positive for EBV with 150,000 copies/mL and was negative for HSV 1/2, cytomegalovirus (CMV), varicella zoster virus (VZV), HHV 6/7, mumps, measles, West Nile virus, mycobacterium, enterovirus, and cryptococcus. Serum and cerebrospinal fluid (CSF) bacterial, fungal, and AFB cultures were negative. Serum autoimmune panel returned positive for ANA with mildly elevated RNP of 1.1 and elevated SS-A of 2.4. Ganglioside panel was notable only for elevated GD1a antibody of 72. Serum and CSF paraneoplastic panel including LGI1, CASPR2, AMPAR 1/2, GABAR, and NMDAR were negative.\nHe was started on sulfamethoxazole/trimethoprim, fluconazole, and azithromycin prophylaxis for his low CD4 count. Given his symptomatic worsening following cessation of steroids, he was treated empirically for possible VKHD exacerbation with IV methylprednisolone 1000 mg daily for three days and was continued on prednisone 60 mg daily. This was followed by empiric treatment for possible acute inflammatory demyelinating polyneuropathy (AIDP) with IVIG 0.5 g/kg divided over four days. On day 9 of hospitalization following completion of IVIG, he was started on emtricitabine and raltegravir. His right eye ptosis improved, but on day 10, he started to develop a left eye third nerve palsy with restricted eye movements. On day 11, he was noted to have a loss of reflexes. On day 13, he started to complain of dysphagia and dysarthria and developed limb weakness (4-/5 ankle dorsiflexion, 4-/5 hip flexion, 4-/5 finger abduction, 4+/5 elbow extension, and 4/5 shoulder abduction), so he was treated with a five-day course of plasma exchange (PLEX) for presumed AIDP. On day 14, a fiberoptic endoscopic evaluation of swallowing was performed noting right vocal cord paralysis, edema, and copious secretions. On day 16, EMG of the left upper and lower extremity was performed, which revealed only a left ulnar motor neuropathy, and MRI of the cervical and lumbar spine showed thickening and enhancement of multiple ventral and dorsal cervical and lumbar nerve roots. Due to continued dysphagia, he underwent EGD with gastric and duodenal biopsies and percutaneous endoscopic gastrostomy (PEG) placement. He had nominal subjective improvement in strength and return of biceps reflex (1+), therefore was discharged to a rehabilitation center on prednisone 60 mg daily.\nOne week later, he was readmitted due to persistent nausea and vomiting. CT of chest, abdomen, and pelvis areas was negative. Duodenal and gastric biopsy from prior admission revealed an atypical lamina propria lymphoid infiltrate favoring aggressive B-cell lymphoma with MYC rearrangement, scattered cells positive for CMV. Further characterization of the lymphoma cells demonstrated MIB1 approaching 100%, intermediate size, prominent nucleoli, variable nuclear contours, and cytoplasmic vacuolization. MUM-1 was negative as were CD30, CD43, CD5, CD3, and BCL-2. Stains for PAX5, CD79a, BCL-6, CD10, and CD20 were positive. Fluorescence in situ hybridization (FISH) panel showed an MYC gene rearrangement without BCL-2 or BCL-6 abnormalities. Burkitt’s lymphoma was strongly favored, with differential also including large B-cell lymphoma or high-grade B-cell lymphoma not otherwise specified. A bone marrow biopsy was performed and found negative for malignancy. He was started on CODOX-M/IVAC chemotherapy treatment. His course was complicated by febrile neutropenia, septic shock, and appendicitis requiring appendectomy, and a decision was made to withdraw care and transfer to hospice. |
A 29-year-old pregnant woman who was being treated with azathioprine for autoimmune hepatitis consulted at 32 weeks of gestation for decreased fetal movement. Obstetric ultrasound revealed many heterogeneous, hypervascular fetal tumors in the left armpit (), intra-abdominal organs, and subcutaneous space (). There was no sign of fetal cardiac insufficiency or anemia (no pericardial effusion and the middle cerebral artery peak systolic velocity was 50 cm/sec or 1,07 MoM).\nFetal wellbeing was monitored daily and fetal lung maturity was induced at 32 weeks of gestation and 2 days with 12 mg of betamethasone repeated at 24 hours.\nTwo days later, a caesarian section was performed because of signs of fetal distress by fetal heart rate monitoring. The child presented immediately difficulties in adapting to extrauterine life and was cared for in the pediatric intensive care unit (). First aid revealed that the child was anemic at birth (hemoglobin at 11,8 g/dl) with a pulmonary arterial hypertension on cardiac ultrasound. A body scan () and a biopsy of a cutaneous tumor were performed and showed characteristics of a rhabdoid tumor (small round nuclei, granular chromatin, and eosinophilic cytoplasm). The immunohistochemical analysis revealed a loss of SMARCB1 protein expression and pathological examination of the placenta showed an invasion of both fetal and maternal compartments (). Because of prematurity, circulatory failure, and severe renal dysfunction with anuria, chemotherapy was contraindicated, and the decision was made with the parents to withdraw care. The child died at day 5 of multivisceral failure. The mother underwent a whole-body MRI to identify potential metastasis, but none was found.\nThe INI1/SMARCB1 analysis conducted by Sanger sequencing of all exons and exon/intron boundaries and a Multiplex Ligation-dependent Probe Amplification (MLPA) to detect copy number alterations revealed a homozygous deletion of the 9 exons in tumor DNA. This alteration was not found in the child's germ line nor in the parents. Therefore, the hypothesis of an inherited predisposition involving the SMARCB1 gene was excluded, and the hypothesis of a postzygotic (de novo) genetic alteration was retained. |
A 51-year-old man was diagnosed with phospholipase A2 receptor (PLA2R) positive, primary membranous nephropathy. He was initially treated with prednisone and cyclophosphamide without significant improvement. The patient was subsequently started on tacrolimus and became calcineurin inhibitor-dependent. Due to a relapse despite being on tacrolimus, rituximab treatment was recommended. His past medical history was remarkable for multiple episodes of deep venous thrombosis on chronic anticoagulation, hypertension, and dyslipidemia. He did not have any previous cardiac history and denied any concerning preceding cardiac symptoms of chest pain, dyspnea, syncope, orthopnea, or paroxysmal nocturnal dyspnea. The patient had a 33-pack-year history of smoking but quit two years previously. His 12-lead electrocardiogram (ECG) prior to rituximab treatment showed normal sinus rhythm. He worked as a carpenter and had been carrying heavy equipment at work without significant physical limitation. His family history is significant for coronary artery disease in his father at 50 years of age and dilated cardiomyopathy (DCM) in his brother. Due to significant cardiovascular diseases in his family members the patient underwent a cardiolite treadmill stress test which showed normal exercise tolerance and functional class I on Bruce protocol for 15 minutes. There was no evidence of fixed or reversible defect.\nThe patient was 179 cm tall with a body weight of 88 kg and body surface area (BSA) of 2.1 m2. Rituximab was administered at a dosage of 1000 mg intravenous (IV) at a starting infusion rate of 50 mL/h. Oral acetaminophen (1000 mg), oral diphenhydramine (50 mg), and IV methylprednisolone (100 mg) were also given as premedication. He underwent his first rituximab infusion without any immediate side effects. Forty-eight hours after the infusion, the patient presented to the hospital and reported having woken up from sleep with chest tightness and shortness of breath. He also had nausea and emesis. His physical examination was unremarkable with no evidence of heart failure.\nA 12-lead ECG showed a new left bundle branch block (LBBB) as shown in . Troponin I level was 0.08 ng/mL (normal <0.03 ng/mL). Otherwise, Laboratory testing revealed the following values: hemoglobin 11.2 g/dL (reference range 13.5-17.5 g/dL), white blood cell count 9.6×109/L (reference range 3.5-10.5 ×109/L), platelet 157×109/L (reference range 150-450 ×109/L), serum creatinine 1.5 mg/dL (reference range 0.8-1.3 mg/dL), BUN 41 mg/dL (reference range 8-24 mg/dL), sodium 141 mmol/L (reference range 135-145 mmol/L), potassium 5.0 mmol/L (reference range 3.6-5.2 mmol/L), chloride 111 mmol/L (reference range 100-108 mmol/L), bicarbonate 22 mmol/L (reference range 22-29 mmol/L). Computed tomography angiogram of the chest was negative for pulmonary embolism without evidence of significant coronary calcification. An urgent transthoracic echocardiogram demonstrated moderately reduced left ventricular (LV) systolic function with an ejection fraction (EF) of 30% with moderate hypokinesis of the anteroseptal myocardium. There was no significant valvular disease. The patient subsequently underwent left and right heart catheterization with coronary angiography which revealed a cardiac output of 5.9 L/min (normal range: 4-8L/min) and a cardiac index of 2.88 L/min/m2(normal range: 2.6–4.2 L/min/m2). There was no significant atherosclerotic disease noted in the coronary vessels. However, there was moderate diffuse hypokinesis of the LV on ventriculogram. Right heart catheterization demonstrated mildly elevated right and left-sided filling pressures. Thus, the diagnosis of non-ischemic cardiomyopathy was made, and the underlying etiology of his non-ischemic cardiomyopathy was postulated to be due to rituximab treatment.\nThe patient received medical treatment for the cardiomyopathy with carvedilol 3.125 mg orally twice a day as well as lisinopril 10 mg and rosuvastatin 10 mg orally once a day. The patient was also subsequently placed on a life vest due to prevent sudden cardiac death. The patient was restarted on tacrolimus for his treatment of membranous nephropathy. He did not receive any further treatment with rituximab. A repeat echocardiogram one month later still demonstrated severe LV enlargement with a calculated EF of 26%. There was persistent generalized LV hypokinesis with abnormal ventricular septal wall motion due to LBBB. Otherwise, there was a mild valvular heart disease. His follow-up ECG showed normal sinus rhythm with LBBB. Carvedilol was increased to 6.25 mg twice a day and slowly titrated up to 25 mg twice a day. At 9-month follow-up, despite that he had better optimization of his medical therapy, his transthoracic echocardiogram still showed reduced LVEF of 31% with moderate−severe LV enlargement. Thus, the patient was evaluated and underwent cardiac resynchronization therapy with implantable cardioverter defibrillators (CRT-D) implantation. At 3 month after initiation of CRT therapy, the patient had significant improvement in his functional status with LVEF of 52%. Although the patient still had proteinuria at 607 mg/dL/24 hours, his kidney function remained stable with creatinine of 1.6 mg/dL and creatinine clearance of 69 mL/min/BSA. |
A 28-year-old female presented because of brown pigmentation and sclerosis of the right lower limb. She had a past medical history of Caesarean sections (at 22 and 24 years), without particular familial medical history. The history of current illness was as follows: She noticed brown pigmentation of the right thigh in 2008. It gradually expanded to her right lower back, but there was no tenderness, spontaneous pain or pruritus. The patient visited a dermatologist and was administered a vitamin C preparation, but it was ineffective for treating the pigmented lesion. Since sclerosis developed in the pigmented region along the course, the patient visited our clinic in September 2011. Upon first examination, brown pigmentation with a slightly clear boundary was present on the right thigh and crus (fig. ). No general symptoms, such as Raynaud's phenomenon, were noted.\nHer serum IgG level was 1,764 mg/dl, and antinuclear antibody titers (FA method) were 160 times, which was slightly elevated, but no specific autoantibody was detected, and no other abnormal findings were noted. For histopathological examination, biopsy specimens were collected from two sites: (1) In the sclerotic region (fig. ), flattened epidermis and increased collagen fibers directly below the epidermis over the deep dermis were noted, and appendages, such as sweat glands, had been lost. (2) In the pigmented region (fig. ), the sweat glands and adipose tissue had been excluded by increased collagen fibers. Under high magnification, many infiltrating inflammatory cells were noted around sweat glands, vessels, and adipose tissue in the deep dermis. On Fontana-Masson staining, melanin deposits were noted throughout the epidermal layer in the pigmented region.\nBased on clinical and histopathological findings, the patient was diagnosed with localized scleroderma. Oral treatment with etretinate 20 mg/day was initiated. The reasons for etretinate selection were that she did not want more children and that acitretin or isotretinoin are not approved in Japan. The skin sclerosis started to improve 28 days after treatment initiation. Oral treatment was continued for about 5 months, and the skin sclerosis and pigmentation improved. Topical corticosteroids were not administered during this period, and no adverse events, such as pruritus, skin thinning, gastroenteritis, paronychia or angular cheilitis, developed. 15 months after the discontinuation of etretinate treatment, although the brown pigmentation remained, improvements in skin eruption were maintained compared to those upon first examination (fig. ).\nBiopsy results upon first examination, after 4 months of oral treatment and 9 months after discontinuation of oral treatment were compared (fig. ). Under low magnification, epidermal thickening was noted after 4 months of oral treatment and 9 months after treatment discontinuation, and sweat glands were present in the dermis. In addition, collagen fibers in the dermis had become bulky and dense (fig. ). Under high magnification, infiltrating inflammatory cells around vessels and in lobular fat in the deep dermis had decreased and atrophied appendages had recovered. |
A 7-year-old Syrian boy with war-related burn injury was referred to our hospital for reconstructive surgery for burn scars and contractures on his face, neck, and body. A consultation with anesthesia department was held by plastic and reconstructive surgery clinic for the preanesthesia evaluation. Patient was conscious and oriented on examination. He had severe scar contractures involving neck, face, anterior chest, and both shoulders leading to restricted mouth opening, no neck extension, and stooped posture with chin and chest fused together by scars and the neck and head contracted in flexed position. The width from upper incisor to lower teeth was approximately 15 mm and Mallampati class was 3, while thyromental and sternomental distance could not be evaluated due to neck and head being contracted in flexed position. Cardiac, thoracic, and laboratory investigations revealed normal findings. Detailed history of the patient obtained from the parents by the help of a translator revealed that the child had been posted for the reconstructive surgery in another university hospital, while the operation was cancelled due to failure to maintain mask ventilation even after pain relief and induction of anesthesia. The previous anesthesiologist had given two attempts after induction of anesthesia but failed at intubation. Then child was awakened. The day after, he was transferred to our hospital for difficult airway approach and the operation. Awake FFB nasal intubation was planned because of the past history of “cannot intubate and cannot oxygenate” scenario. The necessity and details of the procedure were explained to the patient and his family by the help of a translator. After a 6-hour fasting period, the patient was admitted to our intensive care unit (ICU), accompanied by a family member and translator. Following the routine (NIBP, HR, StO2) monitorization (Nihon Kohden, Japan), patient has been informed again about the details and steps of the procedure with the help of the translator. Premedication and sedation were not applied because of the patient's status. During the initial trial phase, nasal drop of xylometazoline 0.1% was instilled for vasoconstriction in both nostrils. Three puffs of 10% lidocaine were implemented for topical anesthesia. Through a nasal cannula, oxygen was administered at 5 L/min through the left side. Tip of the fiberoptic bronchoscope (FOB, 2.8 mm, Karl Storz-Endoskope, Germany) was inserted into the contralateral nostril. Endoscopy was performed. When the vocal cords were visible, the trial procedure was ended. It was explained to the patient and his family that the same procedure would be repeated on the day of surgery as followed by intubation and induction of general anesthesia. On the day of operation, two days after the initial trial, patient was taken to the surgery room and monitored (Infinity Delta Dräger, Lübeck, Germany) routinely (NIBP, HR, SatO2). A nasal drop of xylometazoline 0.1% was instilled for vasoconstriction. Three puffs of 10% lidocaine spray were implemented for topical anesthesia. It directly sprayed onto the mucosa of the mouth, pharynx, and tongue. Through a nasal cannula, oxygen was administered at 5 L/min through the left nostril. Endoscopy was performed through the right nostril. Two ml of 2% lignocaine was sprayed through the FOB on to the glottis after the vocal cords were seen. The FOB's tip was then passed into the trachea through the laryngeal opening and was stopped just above the carina. Lubricated 5.0 nasotracheal tube was railroaded over the FOB. After three ventilations, position of nasotracheal tube was confirmed by the FOB. Successful tracheal intubation had been achieved while maintaining spontaneous ventilation and was monitored by capnography. Propofol, fentanyl, and rocuronium were used for induction of general anesthesia via intravenous route and maintained with remifentanil 0.1 μg/kg/min and sevoflurane in oxygen (Primus workstation Dräger, Lübeck, Germany). The operation lasted for approximately four hours. The contractures on neck and left axilla were released and graft was placed. The intraoperative course was uneventful. The patient was extubated after complete recovery of consciousness, adequate spontaneous breathing, preventive reflex, and muscle strength [] (). |
A 43-year-old male patient attended a dental clinic with the prosthetic crown of tooth 24 in hand, with the remaining fractured root core, part of which was inside the root canal (Figures and ).\nClinical and radiographic examination revealed the absence of a root fracture, which might preclude maintenance of the tooth (). It was also observed that the root canal had not been submitted to removal of sealing material up to the adequate length of 2/3 of the dental remnant to the root apex [, ]; the cast metallic core was short, which impaired the intraradicular retention.\nDuring clinical examination, it was observed that the dental remnant presented satisfactory conditions for a new rehabilitation with placement of an intraradicular core and a total prosthetic crown. It was proposed to remove the portion of the cast core that was inside the root canal for later accomplishment of a new intraradicular cast core, using the existing metal-ceramic crown as a reverse template for the coronal portion of this future core.\nThis alternative was possible because there was no need for additional preparation (wear) of the dental remnant at the cervical level, which would impair the adaptation and reuse of the original prosthetic crown. Initially, root canal preparation (buccal and palatal) was performed by instrumentation with Gates Glidden drills at the appropriate length (2/3 of the dental remnant in the largest (palatal root canal), 1/2 of the dental remnant in the other canal (buccal)) [, ].\nThen, the prosthetic crown was internally ground with carbide drills to allow coronal adaptation of the new cast core. For that purpose, the prepared root canals were previously isolated with petroleum jelly and filled with fluid acrylic resin, supported by prefabricated resin rods (Pincanal®) in each canal. Polymerization of the acrylic resin was properly controlled to avoid adhesion of the resin pin inside the root canal ().\nFor preparation of the coronal portion of the core, the prosthetic crown was fitted on the coronal remnant and was internally isolated with petroleum jelly, filled with acrylic resin, and repositioned on the dental remnant with the core in place, thus serving as a reverse template for reconstruction of the coronal portion of this new core (Figures –).\nAfter completion of polymerization, the finished resin core was sent to the prosthesis laboratory for alloy casting (Figures and ).\nThis new core was properly adjusted to the remaining root, and the adaptation of the metal-ceramic crown on it was also verified (Figures –).\nFinally, both pieces (core and crown) were cemented with definitive zinc phosphate cement (Figures and ), and the occlusal contacts were adjusted (). |
A 30-year-old gentleman with mild autism was admitted to hospital for a left supracondylar fracture following a fall. He was able to communicate and perform simple daily activities independently. He had a past medical history of epilepsy and allergic rhinitis. He was recently discharged from hospital about a week ago after being treated for pneumonia. His mobility was limited by poor vision. He had had multiple falls, some of them with head injuries. His regular medications included chloral hydrate, ferrous fumarate, calcium, vitamin D supplements, and sodium valproate. He did not smoke or drink alcohol [, ].\nThe fracture was treated conservatively. During the admission, his sodium was noted to be 128 mmol/L. Other blood test results are shown in . He was referred to the medical team for review when the sodium levels subsequently dropped to 120 mmol/L on day 3 of admission. Tracing his previous blood test results, his sodium has always been within the range of 124 to 126 mmol/L, and the chronic hyponatremia was previously attributed to psychogenic polydipsia. The previous tests done to investigate hyponatremia were not available for review. Physical examination did not reveal any significant findings. The patient was apyrexial, had a stable blood pressure of 125/80 mmHg with a heart rate of 80 beats per minute. He was clinically euvolemic. Postural blood pressure and heart rate measurements did not show any significant variation.\nAs there was a drop in sodium levels from his usual baseline, the hyponatremia workup was repeated. His serum osmolality was 248 mOsm/kg, urine osmolality 387 mOsm/kg, and urine sodium 86 mmol/L. Thyroid function tests and 9 am cortisol levels were normal (). Urine osmolality of above 100 mOsm/kg suggested a degree of vasopressin secretion leading to inability to excrete free water.\nThe initial impression was SIADH secondary to sodium valproate, recently treated pneumonia, and pain from the left supracondylar fracture. A CT scan of the brain, thorax, abdomen, and pelvis performed to identify other causes of the hyponatremia was normal. As the patient had a urine osmolality of less than 500 mOsm/kg, he was initially placed on fluid restriction of 800 ml/day which was approximately 500 ml below his daily urine volume [], but the serum sodium level remained between 120–125 mmol/L. He was then given 2 sodium chloride tablets 3 times per day. Each sodium tablet contained 600 mg of sodium chloride. His fluid intake was further restricted to 600 ml/day.\nDespite these interventions, the sodium levels did not improve. He was also trialed on furosemide 20 mg twice daily. His sodium did increase to 130 mmol/L, but the patient was complaining of significant thirst, and his renal function deteriorated. He was subsequently taken off furosemide, and his sodium levels returned to his baseline of 126 mmol/L.\nDue to the history of recurrent falls with head injuries, there was a possibility of cerebral salt wasting. However, the patient was clinically euvolemic and did not display any signs of dehydration at presentation. Also, the patient did not respond to sodium supplementation in the diet which goes against the diagnosis of cerebral salt wasting.\nThe possibility of reset osmostat was considered. A water load test was performed one week after cessation of diuretics. Following an overnight fast, the patient was given 800 ml of water (approximately 15 ml/kg) intravenously. About 720 ml of urine was excreted in 4 hours (220 ml at 1 hour, 340 ml at 2 hours, and 570 ml at 3 hours). The results are shown in and .\nA diagnosis of reset osmostat was made, and the patient was discharged without any sodium tablets and fluid restriction.\nThe patient remained clinically well and the sodium levels stable between 125–130 mmol/L. He is being followed up for 6 months in the clinic to monitor his sodium levels. |
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