Introduction:
Moyamoya patients under two years of age represent a therapeutic challenge because of their frequent neurologic instability and concomitant anesthetic risks. We report our experience with pial synangiosis revascularization in this population.
Methods:
We reviewed the clinical and radiographic records of all patients with moyamoya syndrome 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), thirty-four procedures (15 bilateral, 4 unilateral) were performed in 19 patients under two years of age (out of a total of 456 procedures in 240 patients). Eighteen of these patients presented with either stroke or TIA. Average age at first surgery was 1.4 years (range 6 months to 1.9 years). Unanticipated staged operations occurred in three patients, two due to persistent EEG changes during the initial surgery and one due to brain swelling during the procedure requiring ventriculostomy. There were two perioperative strokes; both patients had post-operative seizures but made clinical recoveries. The average follow-up was 7 years (range 1-14). In long term 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 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 post-operative angiography demonstrated progression of disease.
Conclusions:
Despite the challenges inherent to this population, the majority of children with moyamoya under 2 years of age have a good long-term prognosis. Our data 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.
Bilateral retinoblastoma: Long-term follow-up results from a single institution
