Long-term Outcome in Children with Moyamoya Syndrome After Cranial Revascularization by Pial Synangiosis

Object: Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral "moyamoya" vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients.

Methods: The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period. There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8% of the population and transient ischemic attacks (TIAs) in 43.4% prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7% per patient; 4% per surgically treated hemisphere) and three severe TIAs. Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging-documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status.

Conclusions: Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.