Revascularization in Pediatric Patients with Moyamoya Disease.

AIM: To evaluate the patients who underwent surgery with a diagnosis of Moyamoya disease (MMD), and to contribute to the literature from a single-center in Türkiye.

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 MMD, 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). Encephalo Duro Arterio Myo Synangiosis (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 followup, 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 MMD. Postoperative clinical improvements are rapidly observed, followed by radiological improvements. Patients may experience progression from unilateral to bilateral disease, which can progress quickly.