Pial synangiosis in patients with moyamoya younger than 2 years of age

Abstract

Object Patients with moyamoya who are younger than 2 years of age represent a therapeutic challenge because of their frequent neurological instability and concomitant anesthetic risks. The authors report their experience with pial synangiosis revascularization in this population. Methods The authors reviewed the clinical and radiographic records of all patients with moyamoya in a consecutive series of patients under 2 years of age, who underwent cerebral revascularization surgery using pial synangiosis at a single institution. Results During a 12-year period (1994–2005), 34 procedures (bilateral in 15 patients, unilateral in 4) were performed in 19 patients younger than 2 years (out of a total of 456 procedures in 240 patients). Eighteen of these patients presented with either stroke or transient ischemic attack. The average age of the 19 patients at first surgery was 1.4 years (range 6 months–1.9 years). Unanticipated staged operations occurred in 3 patients, due to persistent electroencephalographic changes during the initial surgery in 2 cases and due to brain swelling during the procedure requiring ventriculostomy in the other. There were 2 perioperative strokes; both patients had postoperative seizures but made clinical recoveries. The average follow-up was 7 years (range 1–14 years). Long term, at follow-up, 13 patients (68%) were clinically independent for their age, with 8 (42%) having no significant deficit. Late complications included subdural hygroma evacuation (1), additional revascularization procedures performed years later for frontal lobe ischemia (2), late infarction (1), and asymptomatic ischemic change on routine follow-up MRI studies (1). All patients who had both pre- and postoperative angiography demonstrated progression of disease. Conclusions Despite the challenges inherent to this population, the majority of children with moyamoya under the age of 2 years have a good long-term prognosis. The data from this study support the use of pial synangiosis as a safe, effective, and durable method for treatment of moyamoya for most children in this potentially high-risk population.