Sickle cell anemia with moyamoya disease: outcomes after EDAS procedure

Pediatr Neurol. 2003 Aug;29(2):124-30. doi: 10.1016/s0887-8994(03)00047-x.


Moyamoya disease is a relatively uncommon neurovascular complication of sickle cell anemia. We report a case series of six patients with sickle cell anemia who developed moyamoya disease and underwent encephaloduroarteriosynangiosis procedures. These six patients presented with either cerebrovascular accidents, transient ischemic attacks, or seizures, and subsequent magnetic resonance imaging scans were suggestive of moyamoya-like changes in the cerebral vasculature. Conventional cerebral angiography was used to confirm the diagnosis in all six patients. Four of six patients manifested a cerebrovascular accident before surgery, and two of these patients were compliant on a transfusion protocol at the time of their cerebrovascular accident. Bilateral (n = 4) or unilateral (n = 2) encephaloduroarteriosynangiosis procedures were performed without any complications. The patient who was stroke-free preoperatively had a cerebrovascular accident 2 weeks after the procedure; otherwise, all patients have remained free of neurovascular complications with an average follow-up of 33 months. Collateral anastomoses between external and internal carotid arteries were established by magnetic resonance angiography in three patients. The encephaloduroarteriosynangiosis procedure is a safe and effective treatment option in patients with sickle cell anemia who develop moyamoya disease.

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / complications*
  • Cerebral Angiography
  • Cerebral Revascularization* / adverse effects
  • Cerebral Revascularization* / methods
  • Child
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Moyamoya Disease / etiology
  • Moyamoya Disease / surgery*