gms | German Medical Science

Objective: Moyamoya is a progressive intracranial angiopathy affecting not only the internal carotid artery (ICA) bifurcation along with the middle cerebral arteries (MCA) but also the anterior (ACA) and posterior (PCA) cerebral arteries. Repeated stroke in these territories and in a few cases hemorrhage are commonly observed. We present the systematic workup, indication and planning of multiple cerebral revascularizations in newly diagnosed cases over the last five years.

Method: 64 symptomatic Moyamoya patients (19 adults, 45 children) (range 1-59 years) underwent preoperative workup and cerebral revascularization between 2011 and 2015. Preoperative angiograms showed bilateral angiopathy in one or more of ICA, ACA, MCA, PCA in all patients with a corresponding decrease of perfusion reserves after Diamox- H215O-PET challenge. Depending on the clinical symptomatology, the number of correlating arterial territories involved and the extent of perfusion reserve deficits observed on PET, the surgical strategy was tailor-made for each individual patient.

Results: A total of 179 (136 pediatric, 43 adult) revascularization procedures were performed. Children Cohort: 45 children underwent multiple revascularizations of the ACA (51), MCA (74) and PCA (11) territories. 67 combined direct and indirect revascularizations (STA-MCA/ACA bypass and indirect with galeaperiost with temporalis muscle) were performed. If the donor/recipient vessel was unavailable or of too small caliber, indirect revascularizations with EDAS in 58 ACA/MCA territories and in 11 PCA territories were undertaken. There were no peri- or postoperative complications. At a mean follow-up of 3.5 years, all children were stroke free. Adult Cohort: 19 adults underwent multiple revascularizations in ACA (5), MCA (36) and PCA (2) territories. Thirty nine were direct STA-MCA/ACA bypasses and four were indirect EDAS procedures. Only one (2,5%) of 39 direct bypasses showed an occlusion caused by intraoperative thrombosis of the donor vessel. The patient there fore underwent a second bypass using the frontal branch of the STA at 6 months after the initial surgery. All remaining bypasses were patent at a mean of 1.5 years. There were no perioperative strokes in the revascularized territories.

Conclusions: Meticulous diagnostic workup, pre-surgical planning and tailor-made surgical strategies for multiple cerebral revascularizations are mandatory for effective and safe prevention of recurrent strokes in cases of Moyamoya.