ENCEPHALODUROARTERIOSYNANGIOSIS IN A CHILD WITH SICKLE-CELL-ANEMIA AND MOYAMOYA DISEASE

We report a black girl with sickle cell anemia, On prophylactic exchan ge transfusion protocol, she experienced cerebrovascular accidents at 3 and 3.5 years of age, both associated with transient right hemipares is, At 7.5 years of age, she presented with a partial motor seizure an d a left hemiparesis, A cerebral angiogram demonstrated stenosis at th e origins of both middle and anterior cerebral arteries bilaterally wi th extensive basal collateralization. She underwent uncomplicated bila teral encephaloduroarteriosynangiosis (EDAS) procedures using both sup erficial temporal arteries. At age 9 years, the patient presented with a severe headache and tunnel vision secondary to a stenosis of both p osterior cerebral arteries, She underwent bilateral EDAS procedures us ing both occipital arteries, No complication was encountered, Postoper ative cerebral angiogram demonstrated impressive neovascularity at the sites of all four EDAS procedures, Different treatment options of moy amoya disease are discussed.