İntrakraniyal kanamalı bir olguda Klippel-Trenaunay sendromu ve Hemofili B birlikteliği

Klippel-Trenaunay Sendromu KTS , kutanöz vasküler malformasyonlar, kemik ve yumuşak doku hipertrofisi ve variköz venler ile karakterize ender konjenital bir sendromdur. KTS’de hem periferik hem de visseral tutulum gözlenebilir ve bu önemli morbidite ve mortalite kaynağı olabilir. KTS de intrakranial kanamaya oldukça nadir rastlanır ve altında genellikle arteriovenöz malformasyonlar veya fistül mevcuttur. Bu olgu KTS’lu bir hastadaki intrakranial kanamanın sebebinin Hemofili B olması nedeniyle sunulmuşur.

Anahtar Kelimeler:

Klippel-Trenaunay sendromu, Hemofili B, intrakranial kanama

An intracranial hemorrhage in a patient with Klippel-Trenaunay syndrome and Hemophilia B association

Klippel-Trenaunay syndrome KTS , a rare congenital disorder, is characterized by cutaneous vascular malformation, soft tissue-bony hypertrophy and varicose veins. KTS does not only show peripheral involvement, but also visceral involvement may be observed which is an important source of morbidity and mortality. Intracranial hemorrhage in KTS is extremely rare and usually traced to an underlying arteriovenous malformation or fistula. We report a case that KTS in a patient presented with intracranial hemorrhage was bound to hemophilia B.

Keywords:

Klippel-Trenaunay syndrome, haemophilia B, intracranial haemorrhage,

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