Direct and combined revascularization in pediatric moyamoya disease

Surgical revascularization of moyamoya disease can improve neurological outcomes, compared with the natural history of the disease or the results of medical treatment. Controversy exists regarding whether direct or indirect revascularization yields better outcomes. This study involves a single-center experience with direct anastomosis and is the first North American series using direct revascularization for pediatric patients with moyamoya disease.

Twelve patients (age range, 5-17 yr; mean age, 10.2 yr) underwent direct revascularization of 21 hemispheres. Two patients had experienced failure of previous indirect revascularization procedures, with continued clinical deterioration. Superficial temporal artery-middle cerebral artery anastomosis was performed in 19 hemispheres (with concurrent encephaloduroarteriosynangiosis in 6). Middle meningeal artery-middle cerebral artery anastomosis and omental transposition were each performed in one hemisphere. Follow-up periods ranged from 12 to 65 months (mean, 35 mo), and monitoring included neurological examinations, angiography, magnetic resonance imaging, and cerebral blood flow studies.

The neurological conditions of all patients were stable or improved after surgery. None of the patients developed new strokes, and no new ischemic lesions were seen in magnetic resonance imaging scans. All grafts evaluated by follow-up angiography were patent. Postoperative cerebral blood flow studies showed significantly improved blood flow (54.4 versus 42.5 ml/100 g/min; P = 0.017, n = 4) and hemodynamic reserve (70.3 versus 43.9 ml/100 g/min; P = 0.009, n = 4), compared with preoperative studies.

Surgical revascularization by direct anastomosis in pediatric patients is technically feasible, is well tolerated, and can improve the progressive natural history, the angiographic appearance, and the cerebral blood flow abnormalities associated with the disease. Direct revascularization has the advantage of providing immediate and high-flow revascularization and is particularly useful for patients who have experienced failure of previous indirect revascularization procedures.