Stroke in hemoglobin (SD) sickle cell disease with moyamoya: successful hydroxyurea treatment after cerebrovascular bypass surgery

An 11-year-old boy with hemoglobin sickle disease (HbSD), bilateral stenosi s of the intracranial carotid arteries, and moyamoya syndrome had recurrent ischemic strokes with aphasia and right hemiparesis. His parents (Jehovah' s Witnesses) refused blood transfusions. After bilateral extracranial-intra cranial (EC-IC) bypass surgery, hydroxyurea treatment increased hemoglobin F (HbF) levels to more than 30%. During a follow-up of 28 months, flow velo cities in the basal cerebral arteries remained stable, neurologic sequelae regressed, and ischemic events did not recur. This is the first report of s uccessful hydroxyurea treatment after bypass surgery for intracranial cereb ral artery obstruction with moyamoya syndrome in sickle cell disease. The p atient's religious background contributed to an ethically challenging thera peutic task. (Blood, 2001;97:2165-2167) (C) 2001 by The American Society of Hematology.