Aim:Moyamoya Disease is a rare vascular condition that can lead to devastating outcomes. Although it can be treated surgically, there is insufficient research on this disease in Turkey. Our goal is to evaluate patients who underwent surgery with a diagnosis of Moyamoya disease and contribute to the literature.
Material and Methods:Patients were evaluated retrospectively based on age, symptoms, history of cerebrovascular events (CVE), genetic disorders, pre-operative (pre-op) radiological stage, surgical technique, post-operative (post-op) improvement, and post-op radiological staging. The absence of new CVEs and reduced seizure frequency were considered indicators of clinical improvement.
Results:A total of 7 patients, 4 of whom had bilateral MD, underwent surgery. The average age was 11.8±5 years. 4 patients (57%) presented with cerebrovascular events as symptoms, and the remaining 3 patients (43%) presented with headaches. Cranial digital subtraction angiography (DSA) revealed that the patients were in advanced stages (Suzuki Stage 4.9±1.1).
EncephaloDuroArterioMyoSynangiosis (EDAMS) surgical technique was performed on 10 hemispheres, and a combined bypass (EDAMS + direct) was performed on 1 hemisphere.
Clinical improvement was observed approximately 6 months postoperatively. During follow-up, disease progression in the contralateral hemisphere with associated symptoms was noted in 4 patients, and these patients subsequently underwent surgery on the contralateral hemisphere. The average time between the first and second surgeries was 15±7.7 months. Post-operative follow-up was conducted with DSA, and radiological success was defined as Lucia Stage 2±0.85.
No clinical difference was observed between craniotomy and craniectomy as surgical techniques. No differences were found between vessel selection and clinical outcomes.
Conclusion:The EDAMS protocol has proven to be an effective treatment method for pediatric patients with MD. Post-operative clinical improvements are rapidly observed, followed by radiological improvements. Patients may experience progression from unilateral to bilateral disease, which can progress quickly.