Encephaloduroarteriosynangiosis with Dural Inversion for Moyamoya Disease in a Pediatric and Adult Population-a Single-Center 20-Year Experience

Moyamoya disease refers to a progressive vasostenotic or vaso-occlusive disease that puts patients at risk for ischemic and/or hemorrhagic events. Surgical revascularization procedures aim to restore cerebral blood flow to mitigate stroke risk and functional decline. Direct and indirect bypass procedures have been proposed. Encephaloduroarteriosynangiosis (EDAS) with dural inversion is a well-accepted indirect procedure.

Patients with moyamoya disease undergoing EDAS with dural inversion between 2000 and 2019 were retrospectively reviewed. Clinical data including short-term and long-term outcome were measured using the modified Rankin Scale. Patient satisfaction was assessed in patients with a minimum of 3 years of clinical follow-up.

Over a 20-year period, 54 patients underwent 88 EDAS with dural inversion procedures. Most patients underwent bilateral surgery (34/54 patients, 63.0%). Median age at surgery was 19 years (range, 1-63 years) with a median follow-up of 6 years (range, 1-20 years). Periprocedural complications occurred in 4 of 54 patients (7.4%). Periprocedural infarction occurred in 3 of 88 operations (3.4%). Functional outcome at 90 days was favorable (modified Rankin Scale score 0-2) in 92.6% of cases. On long-term follow-up, 3 patients experienced ischemic infarction (5.6%), and 1 patient (1.9%) experienced hemorrhagic infarction. Patients' overall satisfaction with the surgical procedure at last follow-up was determined in 36 of 45 patients with at least 3 years of follow-up (response rate 80.0%). Median satisfaction was very good (score 5; range, 3-5).

EDAS with dural inversion is a safe and effective indirect revascularization procedure for pediatric and adult moyamoya disease that is associated with favorable long-term outcomes and high patient satisfaction.