EDAS Procedure for Sickle Cell Anemia with Stroke: Report of Four Cases.

Abstract

Children with sickle cell anemia (SCA) and stroke receive chronic transfusions to prevent a recurrent stroke. Despite transfusions, 10–20% have a recurrent stroke and 43% develop moyamoya changes. One treatment option for moyamoya changes is encephaloduroarteriosynangiosis (EDAS) procedure. We report four children with SCA and stroke who have undergone EDAS procedure for progressive clinical and/or imaging findings.Case 1. Five -year-old female with SCA presented with left sided weakness. MRI showed acute infract in the right frontal and parietal areas. She was treated with chronic transfusions. Two years later MRI/MRA showed stenosis in bilateral supraclinoid internal carotid arteries (ICA) and bilateral M1 segments. Patient underwent bilateral EDAS procedures 6 months apart. She tolerated both procedures well and is clinically stable 6 months post second surgery.Case 2. 5-year-old female with SCA presented with right-sided weakness. MRI showed acute infarct in the left middle cerebral artery (MCA) distribution. She was treated with monthly transfusions. Twenty-two months after the initial stroke she presented with left sided weakness. MRI/MRA showed an acute infarct in the right frontal lobe with significant stenosis in bilateral ICA's, and left MCA. She was started on Hydroxyurea in addition to transfusions. She underwent EDAS on the right side. Patient tolerated procedure well. She is one year post surgery and is clinically stable. MRI done 10 months post surgery showed no new infarcts.Case 3. Four year old male with SCA presented with right facial palsy and right-sided weakness. MRI/MRA showed an infarct in the left frontal area and left MCA stenosis. He developed multiple alloantibodies after the initial partial exchange transfusion and was started on Hydroxyurea. His HbF increased to 20%. Repeat MRI/MRA 3 years after the initial stroke showed occlusion of the left MCA and prominent transganglionic collaterals. He underwent EDAS procedure on the left side. Five years after the initial stroke patient had a repeat MRI/MRA that showed occlusion of bilateral posterior cerebral arteries. He was still on Hydroxyurea with HbF level at 35%. He underwent EDAS procedure on the right side. He is clinically doing well one month after the second procedure.Case 4. Five-year-old male with HbSS disease presented with seizures and right-sided weakness. An MRI showed right parietal infarct, and occlusion of the supraclinoid ICA's bilaterally. He was treated with monthly transfusions. Despite being compliant with transfusion therapy, he had a repeat stroke 26 months after the initial stroke. MRI revealed marked stenosis of the bilateral ICA's, middle and anterior cerebral arteries. Patient underwent a left sided EDAS procedure. He is on chronic transfusions along with Hydroxyurea. He is clinically stable 6 months post surgery. In summary, we report 4 cases of sickle cell anemia with stroke, who have undergone EDAS procedure. All patients tolerated the procedure well. However, the effect of this procedure on long-term neuropsychological outcome remains to be seen.Clinical characteristics of sickle cell patients undergoing EDAS procedure for strokePatient numberAge at strokeTreatment for strokeRepeat strokeMoya moya changesAge at EDASTreatment after EDASTime elapsed since EDAS (months)15TxNoYes8 (R), 8 (L)Tx12 (R), 6 (L)25TxYesYes8Tx, HU1234HUNoYes7 (L), 9 (R)Tx25 (L), 1 (R)45TxYesYes8Tx, HU6EDAS: encephaloduroarteriosynangiosis, Tx: treatment, HU: Hydroxyurea, R:right, L: left