O. Vernet et al., ENCEPHALODUROARTERIOSYNANGIOSIS IN A CHILD WITH SICKLE-CELL-ANEMIA AND MOYAMOYA DISEASE, Pediatric neurology, 14(3), 1996, pp. 226-230
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.