Moyamoya disease with repeated intracranial haemorrhage in two consecutive pregnancies.

A 26-year-old woman presented with an intraventricular haemorrhage during the 27th week of gestation in July 1996, and recovered without general or neurological complications after external ventricular drainage. The fetus was delivered spontaneously through the vagina at the 30th week of gestation, but eventually died. Cerebral angiography demonstrated typical appearances of moyamoya disease with occlusion of the supraclinoid portion of both internal carotid arteries and presence of abnormal collateral vessels. Five years later the second haemorrhage, an intracerebral haematoma in the right temporoparietooccipital area, occurred during the 28th week of pregnancy. Emergency craniotomy was performed and the intracerebral haematoma was removed totally. It is still unknown whether repeated pregnancy increases the risk of cerebrovascular accident in moyamoya disease. A long-term follow up study of several patients with haemorrhagic moyamoya disease is needed to clarify risk factors for rebleeding.