Single-Photon Emission CT to Access Outcomes in Children with Moyamoya Syndrome and Sickle Cell Disease Before and After Encephaloduroarteriosynagiosis

About 11% of patients with sickle cell disease (SCD) will develop and ischemic stroke by 20 years. Patients with SCD and Moyamoya Syndrome (MMS) are at an especially high risk for developing stroke. Encephaloduroarteriosynagiosis (EDAS) is emerging as a safe and effective method for the treatment of MMS in Pediatric patients with SCD since it provides improved revascularization. Single-photon emission CT (SPECT) has been used in pediatric MMS patients to demonstrate regions of decreased cerebral perfusion and cerebrovascular reserve. The use of SPECT in SCD patients with MMS undergoing EDAS has not been evaluated.

Between 2001 and 2011 we had 45 patients with SCD who developed clinical evidence of cerebrovascular accident (CVA), transient ischemic attack (TIA), or seizure with radiologic evidence (magnetic resonance and or angiography) of MMS. 20 underwent surgical revascularization with EDAS. Vascular reserve and cerebral perfusion was assessed with SPECT before and after EDAS in 9 patients.

6/9 patients had bilateral EDAS while 3/9 had unilateral EDAS. The postoperative clinical outcome of each patient was assigned to one of the following 3 categories based on the SPECT reports: (1) improved if there was increased perfusion and vascular reserve after EDAS, (2) stable if there were no new areas of decreased perfusion or vascular reserve after EDAS and (3) worsened if there were new areas of decreased perfusion or vascular reserve with increased risk for future infarct after EDAS. 6/9 patients showed improvement, 2/9 patients showed stable cerebral perfusion and vascular reserve and 1 patient showed additional areas at risk for future infarct. SPECT was performed on average 5.7 (range 1.5–16.1) months before EDAS and 16.2 (range 1–47.1) months after EDAS.

SPECT performed before and after EDAS of the involved cerebral hemispheres can assess effectiveness of revascularization surgery and may predict the further clinical outcomes of pediatric patients with SCD and MMS.

No relevant conflicts of interest to declare.