5613251 RAPIDLY PROGRESSING MOYAMOYA SYNDROME IN SICKLE CELL DISEASE -- CASE REPORT

Abstract

Introduction: Moyamoya syndrome (MMS) is an uncommon cerebrovascular complication of Sickle Cell Disease (SCD) characterized by progressive narrowing of large intracranial vessels and development of small-vessel collaterals. This association leads to a significant risk of ischemic stroke and poor neurologic outcomes. Case description: We present a case of a 5-year-old female child, with irregular follow-up at Pediatric Hematology Consultation due to Sickle Cell Disease, who presented to our hospital with a first ischemic stroke. The magnetic resonance angiography (MRA) showed narrowing of the terminal portions of the right internal carotid artery, the proximal segments of the right middle and anterior cerebral arteries and the A1 segment of left anterior cerebral artery, with multiple collaterals, aspects highly suggestive of MMS. She initiated hydroxycarbamide, acetylsalicylic acid and chronic red blood cell exchange (RBCX) aiming HbS<30% and 3 months later she presented with a new transient ischemic stroke. She was submitted to right encephaloduroarteriosynangiosis (EDAS) and was stable, without new ischemic events, until 1 year later when she presented with a new transient ischemic stroke of the left cerebral territory. Since then, she had multiple recurrent ischemic events with total recovery of neurologic deficits. MRA and Computed tomography (CT) showed multiple diffuse areas of cerebral infarction and bilateral severe segmental stenosis of internal carotid, middle and anterior cerebral arteries. Discussion/Conclusion: The literature review suggests that early detection and surgical revascularization in addition to RBCX could significantly reduce stroke recurrence and improve neurocognitive outcome. However, in this case, despite the recommended treatment, it was not possible to prevent the rapid progression of MMS, revealing the complexity of the approach to these patients.