Telangiectasiaより脳内出血を来たしたRendu-Osler-Weber Diseaseの1例

Rendu-Osler-Weber Disease with Cerebral Hemorrhage due to a Capillary Telangiectasia : a case report

I．はじめに

　Rendu-Osler-Weber disease（以下ROWと略す．）は，別名hereditary hemorrhagic telangiecta-siaと言われる稀な全身性遺伝性血管形成異常症である．一方，中枢神経系には，telangiectasiaは0.04％から0.1％に剖検例では認められると報告されているがそのほとんどは無症候性であり，telangiectasiaより重篤な脳内出血を来たすことは非常に稀である．今回われわれは重篤な脳内出血にて発症し，その原因がtelangiectasiaと考えられ，Rendu-Osler-Weber diseaseと診断された10歳女子の症例を経験したので報告する．

We encountered a case of acute cerebral hemorrhage secondary to capillary telangiectasia in a 10-year-old female. She was diagnosed as having Rendu-Osler-Weber disease (ROW). In this case, the cerebralhematoma did not result in neurologic damage and the final outcome was excellent. ROW is an autosomal dominant disorder characterized by the presence of vascular malformations ofvarying types in several tissues, including the brain, nasal mucosa, lungs, gastrointestinal tract, and liver.Neurological complications occur in 8 to 12 percent of patients with ROW.

The pons is the most common site of capillary telangiectasia, but most of the malformations caused areclinically silent. Massive cerebral hematoma due to capillary telangiectasia is rare.

Cerebral hematoma due to hypertension in a child is less than that found in an adult. So in a child it isimportant to investigate the origin of cerebral hematoma.