A single - center retrospective observational study on patients undergoing Encephalo-Duro-Arterio -Myo-Synangiosis in patients with moyamoya disease

BACKGROUND: Many cases of moyamoya disease are refractory to conventional medical therapy, hence surgical revascularization techniques have emerged as one of the primary choices of treatment. In this study, we present the functional and angiographic outcomes of patients undergoing encephalo-duro-arterio-myo-synangiosis (EDAMS). METHODS: This is a retrospective observational cohort study, done over 8 years (2012–2020) in a neurological center in Eastern India. Data were retrieved from the hospital's electronic system, recorded in a standard data abstract sheet, and analyzed. RESULTS: This study included 75 patients, with a male (n = 42; 56.0%) preponderance. Majority belonged to the pediatric age group (≤18 years) (n = 70; 93.3%); remaining adult population included 5 (6.6%) patients. The most common presenting complaint was that of an ischemic cerebrovascular accident (CVA) (n = 57; 76.0%). Symptomatic hemispheres (n = 69; 92.0%) were treated and later followed if they had progressed to bilateral disease formation. Preoperative DSA showed 50 (71.4%) to have Suzuki grade 3 type of angiographic findings. Postoperative complications included worsening unilateral hemiparesis 4 (40%), slurring of speech (n = 2; 20.0%), hematoma (n = 2; 20.0%), and surgical site infection (n = 2; 20.0%). One patient succumbed to his illness on the second postoperative day. A postoperative angiogram showed regression of moyamoya vessels in the majority (n = 69; 93.3%) of patients. All (n = 74; 100%) had an intensification of transdural vessels; none had a regression. None of the study participants showed an intensification of moyamoya vessels. Many of our patients (83.8%) had a good grade of revascularization (modified Matsushima and Inaba A and B), while 16.2% had low-grade revascularization (grade C). On assessing outcomes using a modified Rankin Score, a large number of our patients had an excellent (n = 45, 60%) neurological outcomes. CONCLUSION: There was a bimodal age distribution with most of them presenting with ischemic CVA. This procedure (EDAMS) had good angiographic (Matsushima and Inaba) and functional (modified Rankin Score) outcomes.