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Moyamoya syndrome associated with Basal meningioma successfully treated by the modified transsphenoidal approach: case report.

ABSTRACT: Objective Moyamoya vasculopathy associated with skull base tumors has been rarely reported except for partially removed pituitary adenoma and craniopharyngioma. Only a single case of meningioma resulted in vast cerebral infarction. Surgical treatment carries a high risk of damage to the compensatory collateral circulation through leptomeningeal anastomosis leading to a devastating outcome. Case presentation A 46-year-old woman presented to our hospital with progressive visual disturbance. She had a history of medically treated moyamoya vasculopathy. Head magnetic resonance imaging revealed a well-demarcated skull base tumor extending from the planum sphenoidale to the diaphragm sellae. The patient had no cerebral ischemic symptoms, but iodine-123 N-isopropyl-p-iodoamphetamine single-photon emission computed tomography indicated bilateral hemodynamic compromise. Modified extended transsphenoidal surgery was performed, resulting in successful removal without complications including cerebral ischemia. Conclusion The very low tolerance to cerebral ischemia associated with moyamoya disease manipulation in this location carries an extremely high risk of cerebral infarction. This is the first report of a basal meningioma associated with moyamoya syndrome that was successfully removed. The modified extended transsphenoidal approach is the first-line surgery for skull base meningioma located in the para-midline region.

SUBMITTER: Ogawa Y

PROVIDER: S-EPMC4110121 | biostudies-other | 2014 Aug

REPOSITORIES: biostudies-other

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Project description:To describe a case of atypical hemolytic uremic syndrome induced by influenza A infection with the p.Ile1157Thr C3 mutation.Data sourcesClinical observations of a patient.Study selectionCase reports.Data extractionData extracted from medical records, after patient's consent.Data synthesisFour days prior to presentation to our hospital, a 16-year-old adolescent had a fever and arthralgia with hematuria. He was found to be positive for type A influenza and prescribed oseltamivir and acetaminophen by a primary-care physician. A bleeding tendency and purpura in the extremities and on the trunk developed; therefore, he was transferred to Chiba University Hospital. Hematology revealed severe thrombocytopenia, hyperbilirubinemia, and acute kidney injury. Aspartate aminotransferase, lactate dehydrogenase, and potassium could not be determined because of severe hemolysis. Highly elevated blood urea nitrogen and creatinine levels indicated acute kidney injury. A platelet count of 24,000/μL indicated thrombocytopenia, with low hemoglobin level. Peripheral blood profiling identified schistocytes. Continuous hemodiafiltration and plasma infusion were initiated immediately; however, he became oliguric. Plasma exchange was initiated on ICU day 3, but decreased urine output, hemolysis, and thrombocytopenia persisted. IV eculizumab therapy was initiated on day 7 and resulted in recovery of these symptoms and also successful discontinuation of renal support. The patient showed a stable condition without recurrence of hemolytic findings and acute kidney injury and is currently on maintenance therapy of eculizumab (1,200 mg, every other week) without any relapse of atypical hemolytic uremic syndrome symptoms. A plasma sample collected prior to initiation of plasma exchange showed an disintegrin-like and metalloprotease with thrombospondin type 1 motifs 13 activity level of 104.9%. The absence of both Shiga toxin-producing Escherichia coli in feces led to suspicion of atypical hemolytic uremic syndrome. Subsequent genetic analysis identified a mutation in C3 (p.Ile1157Thr), confirming the diagnosis of atypical hemolytic uremic syndrome.ConclusionsAlthough managing thrombocytopenia secondary to infection, inclusion of atypical hemolytic uremic syndrome in the differential diagnosis at an early stage is important in clinical practice.

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Moyamoya syndrome associated with Basal meningioma successfully treated by the modified transsphenoidal approach: case report.

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