Ischemic infarction following an intra-cerebral hemorrhage in an adult sickle cell disease with angiographic Moyamoya

摘要

A 43-year-old lady, known case of sickle cell disease (SCD) was admitted in sickle cell crises and developed a left frontal intracerebral hematoma. She worsened further neurologically and was found to have developed a large left middle cerebral artery (MCA) infarct. Angiogram showed Moyamoya pattern. The patient was managed conservatively with exchange transfusions and made good recovery. She is being maintained on monthly exchange transfusions and hydroxyurea. Such a presentation has been described infrequently, that too mostly in children. Only once, it has been reported with adult SCD. Diagnostic and management controversies are discussed in the light of available literature.