"STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis" as a one-staged revascularization strategy for pediatric moyamoya vasculopathy

Moyamoya vasculopathy progressively compromises cerebral blood flow resulting in chronic hypoperfusion. The middle cerebral artery (MCA) territory and the bifrontal areas are the regions most frequently affected. Although most techniques aim to only revascularize the MCA territory, augmentation of blood flow of the bifrontal areas is of importance in the pediatric moyamoya population since these regions play an important role in cognition, intellectual development, and in lower extremity and sphincter function. We recently described a one-staged surgical procedure combining revascularization of three regions, the MCA territory unilaterally and the frontal areas bilaterally. The purpose of this article is to report our surgical experience in eight children and to emphasize the rational for bifrontal revascularization.

We report a case series consisting of eight children where the following surgical strategy was applied: (1) a direct superficial temporal artery-to-middle cerebral artery (STA-MCA) bypass with encephalo-duro-myo-synangiosis (EDMS) for unilateral MCA revascularization; in combination with (2) a bifrontal encephalo-duro-periosteal-synangiosis (EDPS) for bifrontal revascularization. Patients' characteristics and 30-day follow-up data are reported.

The patient group consisted of six girls and two boys (mean age 10.0, range 4.2-17.5 years): six children presented with moyamoya disease, two with moyamoya syndrome. We performed a one-staged revascularization of one MCA territory and both frontal areas in all patients. No significant complications occurred. Two patients experienced postoperative focal seizures, successfully treated with anti-epileptic medication.

The single-staged STA-MCA bypass with EDMS combined with bifrontal EDPS allowed revascularization of three regions (the MCA territory unilaterally and the frontal areas bilaterally) and may serve as an alternative and safe treatment option for pediatric moyamoya patients.