Introduction: Sickle cell disease (SCD) patients with Moyamoya syndrome (MMS) represent a rare subset of patients with potentially devastating neurologic outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. We aim to elucidate the effectiveness of surgical intervention in our series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization versus conservative transfusion therapy.
Methods: We retrospectively reviewed our database of Moyamoya patients at the Johns Hopkins Medical Institution from 1990-2013. Pediatric patients (age<18 years) with a confirmed diagnosis of SCD and MMS were included. Baseline and management information was described. Intracranial stroke occurrence during the follow-up period was compared between interventional and conservatively managed patients.
Results: A total of 15 pediatric SCD-MMS patients(28 affected hemispheres) were included in our study, and all were African American. Seven patients(14 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 +/- 4.0 years, with 9 (60.0%) being female. Fourteen(93.3%) patients stroke before diagnosis of MMS with average age at first stroke of 6.6 +/- 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres while no patients undergoing revascularization procedure had any strokes at follow-up (p=0.029).
Conclusions: Our study suggests that indirect revascularization is a safe and effective alternative to best medical therapy in patients with SCD-MMS. Additional studies with larger sample sizes may further clarify optimal treatment regimens for this challenging group of patients.
Patient Care: This is the first study comparing conservative management with surgical revascularization in patients with SCD-MMS. We found that compared on the grounds of conservative management, surgery provided stroke prevention with acceptable surgery-associated complications (3 patients with TIA that eventually resolved). This finding provided comparative evidence to support surgical management in the population of SCD-MMS patients
Learning Objectives: By the conclusion of this session, participants should be able to:
1) Understand that SCD-MMS is a rare disease with unclear natural history
2) Learn that indirect surgical revascularization may be an alternative treatment strategy for SCD patients with recurrent ischemic attacks/strokes
3) Understand that surgical revascularization is effective in preventing subsequent strokes