The Role of Encephaloduroarteriosynangiosis in Moyamoya Disease: A Consecutive Case Series From Pakistan

Background Moyamoya disease is a rare cerebrovascular disorder characterized by the occlusion of cerebral arteries due to stenosis. Although the disease lacks a specific treatment approach, various surgical options have been discovered to address ischemic issues, including indirect bypass, direct bypass, and their combination, which aim to enhance cerebral blood flow. Indirect revascularization via encephaloduroarteriosynangiosis (EDAS) is deemed effective in preventing additional strokes, although its use for preventing future hemorrhagic stroke in Moyamoya disease remains a subject of controversy. Objective The aim of this study was to evaluate the clinical outcomes of EDAS in hemorrhagic Moyamoya disease. Methodology This retrospective cohort study included eight consecutive patients with hemorrhagic Moyamoya disease who underwent indirect revascularization via EDAS from January 2019 to March 2024 at the Department of Neurosurgery, Punjab Institute of Neurosciences, Lahore, Pakistan. Of these eight patients, one patient was excluded owing to the non-availability of follow-up data. The data were collected in October 2024 by reviewing patient demographics, clinical characteristics, imaging findings, hematological parameters, treatment options, treatment outcomes, and follow-up data from hospital records. Results The mean age of study participants was 14.7±6.8 years. The mean preoperative Glasgow coma scale (GCS) score and the modified Rankin scale (mRS) score were 12.8±2.3 and 2.3±1.4, respectively. Following EDAS, four (57.1%) patients developed collateral circulation with grade A, whereas three (42.8%) patients had grade B according to the Matsushima grading system. The mean postoperative GCS score was 14.0±1.4, whereas the mean postoperative mRS score was 1.3±1.2. Only two patients developed postoperative ischemic symptoms, while none of them developed recurrent intracranial hemorrhage. Conclusion Appreciating the small size of the study, we recommend the effective utilization of EDAS in preventing strokes and improving functional outcomes in patients with hemorrhagic Moyamoya disease. The favorable role of EDAS is justified due to the lack of recurrent intracranial hemorrhage in our patients during the follow-up period.