[A juvenile onset case of moyamoya disease with intraventricular hemorrhage during pregnancy: case report].

We report a primipara with intracranial bleeding during pregnancy. The patient, a 23-year-old female, had history of cerebral ischemic attack during childhood and was diagnosed as having moyamoya disease at the age of ten. She did not undergo reconstructive vascular surgery but had not suffered from the cerebral ischemic attack in the last ten years. She suddenly lost consciousness in the eighth month of pregnancy and was admitted to our hospital. The brain CT scan revealed right paraventricular hemorrhage with ventricular perforation. After the newborn baby was delivered by cesarean section under spinal anesthesia, an emergency ventricle drainage was performed. The angiography demonstrated the occlusion of the internal carotid arteries at C1 portion with evidence of a moderate basal moyamoya network on both sides. Marked dilatation of the posterior lateral choroidal arteries was also seen on both sides, but no aneurysms were found. We supposed that the source of intracranial hemorrhage was a breakdown of the dilated perforating moyamoya vessels that formed the basal collateral network. We also supposed that the time of onset of intracranial hemorrhage might be related to the hemodynamic and hormonal changes occurring during pregnancy. Appropriate counterplans to pregnancy should be made by females with moyamoya disease, because pregnancy may be risky for them.