Abstract
Moyamoya disease (MMD) is a chronic cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries (ICA) and the development of fragile collateral vessels. These abnormal vessels can lead to ischemic or hemorrhagic strokes, with intracranial hemorrhage being a notable complication. Although rare, hydrocephalus may also occur in MMD, particularly in the setting of intraventricular hemorrhage (IVH). We present the case of a 42-year-old male with a history of cerebral palsy (CP) and MMD, diagnosed at the age of seven, who developed IVH and hydrocephalus. Despite prior bilateral craniotomy and encephaloduroarteriosynangiosis (EDAS), the patient presented with seizure activity and was found to have a hemorrhagic stroke with IVH and hydrocephalus on imaging. He initially underwent external ventricular drain (EVD) placement, which was ineffective, necessitating ventriculoperitoneal (VP) shunt insertion. His hospital course was complicated by aspiration pneumonia and chronic respiratory failure, requiring tracheostomy. Although the IVH and hydrocephalus ultimately resolved, the patient remained in a vegetative state. This report highlights the complexities of managing MMD in patients with comorbid CP, underscoring the need for early diagnosis, timely revascularization, and coordinated multidisciplinary care.