A retrospective chart review of the perinatal period in 22 pregnancies of 16 women with Moyamoya disease

Moyamoya disease (MMD) is characterized by severe stenoses of the arteries in the circle of Willis, which predispose the patient to brain ischemia and intracranial hemorrhage. We performed a retrospective chart review of 22 pregnancies in 16 patients with MMD at the Kyushu University Hospital. An uncomplicated Cesarean delivery was performed in nearly all patients. In the 20 pregnancies in patients with pre-existing MMD, two had transient ischemic symptoms in the postpartum period. Two patients not previously known to have MMD developed transient ischemic symptoms postpartum. One of these patients was initially diagnosed and managed as pre-eclampsia. For patients diagnosed with MMD, a good perinatal outcome can be expected with appropriate management. Neurological events, however, may still occur postpartum even in well-managed patients. MMD may mimic the signs and symptoms of other neurological or psychiatric disorders, thereby complicating diagnosis and management.