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1/556. Pseudocapsulorrhexis in a patient with iridocorneal endothelial syndrome.

    We describe a patient with Chandler's syndrome variant of the iridocorneal endothelial syndrome in whom ectopic Descemet's membrane was found intraoperatively on the anterior surface of the lens. Initially, the membrane was confused with the anterior lens capsule during extracapsular cataract extraction, leading to the performance of a pseudocapsulorrhexis. Electron microscopy disclosed that the epilenticular membrane was composed of multiple layers of abnormal basement membrane consistent with the iridocorneal endothelial syndrome.
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2/556. Ocular changes in mucopolysaccharidosis iv A (Morquio A syndrome) and long-term results of perforating keratoplasty.

    BACKGROUND: The mucopolysaccharidoses (MPS) are an inhomogeneous group of disorders of errors in the carbohydrate metabolism with severe ocular involvement (corneal opacification, retinal degeneration, optic atrophy). PATIENT PRESENTATION: We report on a boy aged 12 years, with Morquio A (MPS IV A) syndrome. Ocular findings: progressive pseudoexophthalmus due to shallow orbits, increasing corneal stromal clouding, intermittent dissociated manifest nystagmus of the left eye, nyctalopia. visual acuity OD cc = 0.16, OS cc = 0.05. electrophysiology: changes suggesting a symptomatic tapetoretinal degeneration and optic atrophy. TREATMENT AND COURSE OF disease: OS: perforating keratoplasty. Postoperative improvement of visual acuity to 0.25 for nearly a year, followed by progressive reopacification of the corneal graft. Both eyes: progressive signs of tapetoretinal degeneration and optic atrophy. visual acuity now reduced to OD 0.05, OS 0.1. CONCLUSIONS: Success of a keratoplasty is limited by (1) reopacification of the cornea, (2) visual impairment due to (a) retinal degeneration and (b) optic atrophy. The indication for perforating keratoplasty has to be thought about very carefully in these multimorbid patients. In our patient, beside progressive visual impairment there is a progressive deafness which dominates his social and school life. Attending school is severely complicated by the double handicap. Perforating keratoplasty enabled the boy to attend a school for physically handicapped without a special low-vision care for another year. Progressive visual loss without further treatment options now renders optical and electronic low-vision aids necessary. Although the time of improved visual acuity lasted less than a year, we think patients with a life expectancy of less than 20 years should have every possible improvement of their situation - even if it does not last permanently. We therefore propose perforating keratoplasty in spite of insufficient long-term results.
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3/556. Unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy in a patient with multiple endocrine neoplasia type 2a syndrome.

    We report the first patient who had bilateral pheochromocytoma associated with multiple endocrine neoplasia type 2a syndrome (MEN 2a) and underwent unilateral laparoscopic adrenalectomy followed by contralateral retroperitoneoscopic partial adrenalectomy 2 years later. The postoperative course was uneventful both times, and the patient was cured of hypertension without any need for steroid replacement. Endoscopic partial adrenalectomy is a minimally invasive procedure for pheochromocytoma with mild symptoms. We believe that this procedure has considerable potential for treating bilateral pheochromocytoma, which is frequently observed in patients with MEN 2a.
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4/556. Undiagnosed Mirizzi's syndrome: a word of caution for laparoscopic surgeons--a report of three cases and review of the literature.

    The mirizzi syndrome is often undiagnosed before surgery and can carry a high risk of iatrogenic damage to the common bile duct when encountered during open or, especially, laparoscopic surgery. Endoscopic management has recently been reported, but this treatment can be performed only when there is a high index of suspicion based on clinical criteria that the condition is present and therefore suggests the indication for endoscopic retrograde cholangiopancreatography (ERCP). This is not always the case. Consequently, in a considerable percentage of patients, the syndrome is discovered only after the bile ducts have been damaged during surgery. Three cases of Mirizzi's syndrome were observed in our experience of 896 laparoscopic cholecystectomies (0.3%). All patients were without typical symptoms, and the syndrome was unsuspected in spite of preoperative intravenous cholangiography. All patients required conversion to an open procedure, with two injuries of the common bile duct (a complete transection and a tear) being promptly repaired. We conclude that when this syndrome is suspected or found during surgery, the surgeon should follow these guidelines: (1) perform intraoperative cholangiography when possible, even through the gallbladder wall; and (2) dissect the gallbladder from above and, if necessary, open it to extract the stone. dissection of Calot's triangle should never be attempted. Great expertise is required to complete the operation laparoscopically. The reported cases in the literature refer to a high percentage of conversion, underlining the technical difficulties and making this syndrome, when undiagnosed and unsuspected, a real challenge for laparoscopic surgeons.
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5/556. Noninsulinoma pancreatogenous hypoglycemia: a novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes.

    In adults, endogenous hyperinsulinemic hypoglycemia is almost invariably due to insulinoma. In these patients with insulinoma, neuroglycopenic episodes exclusively after meal ingestion and negative 72-h fasts are extraordinarily rare. We describe five adults with neuroglycopenic episodes from hyperinsulinemic hypoglycemia within 4 h of meal ingestion and negative 72-h fasts. Each had negative transabdominal ultrasonography, spiral computed tomographic scanning, and celiac axis angiography of the pancreas. However, all showed positive selective arterial calcium stimulation tests indicative of pancreatic beta-cell hyperfunction. At pancreatic exploration, no insulinoma was detected by intraoperative ultrasonography and complete mobilization and palpation of the pancreas. Moreover, the resected pancreata showed islet hypertrophy and nesidioblastosis, but no insulinoma. No definite disease-causing mutation was detected in Kir6.2 and SUR1 genes, which encode the subunits of the pancreatic ATP-sensitive potassium channel responsible for glucose-induced insulin secretion. Four patients who underwent gradient-guided partial pancreatectomy have been free of hypoglycemic symptoms for up to 3 yr follow-up; the other, who underwent a limited distal pancreatectomy, has had brief recurrence of symptoms. The unique clinical features and responses to dynamic testing in these adults with hyperinsulinemic hypoglycemia in the absence of insulinoma may constitute a new syndrome of postprandial hypoglycemia from diffuse beta-cell hyperfunction.
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6/556. Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord.

    A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.
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7/556. scoliosis secondary to cerebrocosto-mandibular syndrome. A case report with surgical management.

    STUDY DESIGN: A case report of scoliosis secondary to cerebrocosto-mandibular syndrome. OBJECTIVES: To bring about awareness of the scoliosis and respiratory problems associated with this rare syndrome. SUMMARY OF BACKGROUND DATA: Of the approximately 50 cases of this syndrome previously reported, only 3 involved scoliosis, and only 1 of these required surgery (but the patient expired). methods: A retrospective chart was made, and a radiologic review were done. RESULTS: A successful surgical outcome was achieved, despite severe preoperative curvature (112 degrees scoliosis) and diminished pulmonary function (21% vital capacity). CONCLUSION: This syndrome can be associated with progressive scoliosis but can be managed successfully with surgery.
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8/556. Cardiac involvement in coffin-lowry syndrome.

    coffin-lowry syndrome is an X-linked recessive syndrome of mental retardation, characteristic facies and skeletal anomalies. In one patient with the syndrome, we observed early recurrent episodes of congestive heart failure with intercurrent normalization and the late development of mitral insufficiency due to annular dilation and congenital abnormalities of the valve apparatus. This unusual course of cardiac involvement, the non-adaptation of the left ventricular contractility to the aggravation of the mitral insufficiency and the postoperative persistence of the ventricular dysfunction, underline the possible role of an associated primary myocardial disease. This clinical observation demonstrates clearly that a mitral valve malformation can occur in patients with the syndrome, but also the role of a dilated cardiomyopathy, which can be secondary to the mitral regurgitation, but is more likely a myocardial disorder occurring as part of the syndrome.
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9/556. Combined endoscopic and surgical management of mirizzi syndrome.

    mirizzi syndrome is a form of obstructive jaundice caused by a stone impacted in the gallbladder neck or the cystic duct that impinges on the common hepatic duct with or without a cholecystocholedochal fistula. This syndrome is a rare complication of cholelithiasis that accounts for 0.1% of all patients with gallstone disease. Preoperative recognition is necessary to prevent injury to the common duct during surgery. We present a patient with a preoperative diagnosis of type I mirizzi syndrome that was confirmed and drained by endoscopic retrograde cholangiography (ERC), followed by subtotal cholecystectomy. A review of the literature covering its clinical presentation, diagnosis, and surgical treatment is also presented.
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ranking = 2
keywords = operative
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10/556. duodenal obstruction by gallstone: case report of Bouveret's syndrome.

    Bouveret's syndrome involves gastric outlet obstruction by gallstone. Herein we describe an unusual case of duodenal bulb obstruction by gallstone. An 80-year-old woman was hospitalized with a fifteen-day history of vomiting. Computed tomography (CT) showed pneumobilia and a round calcified mass in the second portion of the duodenum. upper gastrointestinal tract series demonstrated the same sized oval radiolucency between the bulbus and the second portion of the duodenum. Endoscopic examination revealed a round black mass in the second portion of the duodenum, totally occupying the lumen. Endoscopic removal and destruction of the gallstone was attempted using a dye-laser, but the stone was too hard to crush. Eventually surgical enterolithotomy was successfully performed without cholecystectomy or closure of the fistula. Improved preoperative systemic management and prompt examination allowed earlier surgical intervention and reduced the morbidity. Surgical approach whether fistula closure should be performed remains controversial.
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