Hemorrhagic moyamoya disease in children: clinical features and surgical outcome

The clinical presentation of moyamoya disease (MMD) typically includes cerebral ischemia in children and intracranial hemorrhage in adults. Because of its rarity, the benefit of surgery in the hemorrhagic type of pediatric MMD has not been clearly established. The purpose of this study was to delineate the clinical features and surgical outcome of hemorrhagic MMD in children.

Thirteen patients (six boys and seven girls with a combined mean age of 11.1 years and a range of 4-18 years) were retrospectively confirmed as having MMD with intracranial hemorrhage between 1988 and 2009. All of the patients underwent indirect bypass surgery that followed a relatively uniform scheme at a single institution. We retrospectively reviewed their clinical features and surgical outcome. The mean time of follow-up was 50.3 months (with a range of 3-99 months).

Six of the patients presented with intracerebral hemorrhage (ICH), and six others presented with intraventricular hemorrhage (IVH); the remaining patient presented with both ICH and IVH. Preoperative angiography demonstrated that the patients were in various stages of the disease, but the majority of the patients (i.e., 16 of the 26 hemispheres) were in stage 3 with abundant moyamoya vessels (MMV). Three of four patients who presented with a history of prior hemorrhage or infarct exhibited a poor clinical outcome. Five patients with good revascularization had a decreased degree of MMV in their follow-up angiogram and showed good clinical outcome. Recurrent bleeding occurred after surgery in one patient.

Revascularization surgery may have a role for prevention of rebleeding and cerebral infarction in pediatric MMD patients who present with intracranial hemorrhage.