Effect of Modified Encephalo-Duro-Arterial-Synangiosis on Periventricular Anastomosis in Pediatric Patients with Moyamoya Disease.

Abstract

Periventricular anastomosis (PA) is a risk factor for hemorrhage in moyamoya disease. In pediatric moyamoya disease, the onset of hemorrhage after adulthood worsens its long-term prognosis. At our institution, modified encephalo-duro-arterial-synangiosis (EDAS) is the first-line treatment for pediatric cases. Herein, we examined the outcome of modified EDAS and its effect on the development of PA. A total of 21 patients who underwent revascularization for pediatric moyamoya disease between March 2010 and December 2022 were included. Image analysis was performed on 36 hemispheric surgical cases followed up for ≥ 2 years postoperatively. The degree of PA was classified as grades 0-2 at 4 sites: lenticulostriate artery (LSA), thalamic artery (THA), anterior choroidal artery (AChA), and posterior choroidal artery (PChA) anastomosis, and evaluated using a total score of 8 points. Of 36 surgical cases, perioperative complications included transient ischemic attack, asymptomatic stroke, symptomatic stroke, and postoperative epidural hematoma/infection in 8, 2, 1, and 1 cases, respectively. At a median follow-up of 87.2 months, 34 hemispheres (94.4%) remained event-free. The grade 2 PA observed in each region (LSA 47.2%, THA 16.7%, AChA 38.9%, PChA 30.6%) preoperatively showed regression after revascularization in 70.6% for LSA, 100% for THA, 78.6% for AChA, and 90.9% for PChA, and the PA score improved significantly from 3.7 to 2.0 (P = 0.0001). Modified EDAS in pediatric patients with moyamoya disease was associated with transient perioperative complications and a favorable long-term prognosis. In addition, imaging showed PA regression, which may effectively prevent late hemorrhagic complications.