Indirect Revascularization Procedures for Surgical Treatment of Moyamoya Disease in Pediatric Patients

Background: Many forms of indirect cerebral revascularization had been adapted to establish neovascularization in moyamoya disease (MMD) affecting pediatric age group. Objective: The aim of this study was to analyze the clinical outcome and different angiographic responses following combined indirect revascularization [encephaloduroarteriosynangiosis (EDAS) with bifrontal encephalogaleosynangiosis (EGS)] in pediatric patients with MMD. Patients and Methods: This study was conducted as a retrospective study in the period between January 2013 and January 2015, in Department of Neurosurgery Ain Shams University, on pediatric patients with MMD who underwent combined EDAS and Bifrontal EGS. Clinical outcomes were assessed. Preoperative and postoperative angiograms from twenty-eight treated hemispheres were collected and analyzed as regard good revascularization signs. Follow up ranged from 6 to 24 month. Results: Twenty-nine hemispheres were treated in fifteen patients, age ranged from 1.5 to 10 years with a mean of 5.57- ±2.96. There were nine males and six females. In our study, 93.3% of the patients presented with ischemic neurological symptoms. Eighty % of the patients showed stabilization and improvement of the symptoms after 3 month of the treatment. One patient 6.7% had postoperative major posterior circulation stroke, two patients 13.7% hand ischemic event between the two stages of treatment with good outcome. Mortality was 6.7%. In the twenty-eight hemispheres with follow up, good revascularization was established in 78.6% in the middle cerebral artery (MCA) territory, and in 71.4% in the anterior cerebral artery (ACA) territory. Superficial temporal artery (STA) showed neovascularization in 72.7% of the improved hemispheres and the middle meningeal artery (MMA) in 90%. STA showed increase in caliber in 42.9% and MMA in 50%. There was a decrease or disappearance of moyamoya vessels in 65% of the followed hemisphere. Conclusion: Our results demonstrate that indirect revascularization (EDAS and Bifrontal EGS) with arachnoid opening should be considered as a primary line of treatment once diagnosis of MMD is made in pediatric age group. It is technically feasible, well tolerated, safe with good clinical outcome, decrease in the rate of the strokes, and decrease in morbidity and mortality rates .The combination of EDAS and Bifrontal EGS gives a wide cortical surface covering including MCA and ACA territory with good neovascularization rates and this could alter the progressive natural history and the angiographic appearance of MMD. Outcomes of these procedures are best evaluated clinically and not only angiographically. For more adequate results, a longer follow up with a bigger sample is needed and launching of an Egyptian registry for treatment of pediatric MMD, could do this.