A RARE CASE OF SICKEL CELL DISEASE (SCD) MOYA MOYA SYNDROME : CASE REPORT

Abstract

BACKGROUND: Moyamoya syndrome (MMS) is a relatively uncommon vascular complication of sickle cell disease (SCD) characterized by progressive stenosis of the supraclinoid carotid arteries and development of typical collaterals. MMS confers a signicant lifetime risk of ischemic stroke, and frequently results in multiple cerebrovascular events leading to poor outcomes. Management of MMS in this population is challenging. Ischemic stroke develops in 7% to 11% of children with homozygous sickle cell anemia (HbSS) CLINICAL DESCRIPTION: We present a case of Moyamoya syndrome disease that was detected in a 7-year-old female who presented with transient altered mental status &leftsided hemiparesis. On MR angiography and further investigation, the child was diagnosed to have Moyamoya diseases with sickle cell disease. MANAGEMENT: Although there is no denitive medicaltreatment, numerous direct and indirect revascularization procedures have been used to improve the compromisedcerebral circulation, with outcomes varying according to procedure type, the treatment goal is toimprove blood ow to hypoperfused cerebral regions bysurgery. Our patient was treated conservatively and referred to a higher Centre for specic neurosurgery. Neurosurgical revascularization process leads to favorable outcome. CONCLUSION: Moyamoya disease can be diagnosed if history, physical exam and brain imaging is highly suspicious. Conventional angiography remains the gold standard for diagnosis and aids in surgical planning for patients with suspected Moyamoya disease