Unilateral moyamoya disease mimicking intracranial hemorrhage in a pediatric patient: Surgical treatment with encephalo-duro-myo-synangiosis in a progressive disease

Abstract

Abstract Background Moyamoya disease (MMD) is characterized by progressive bilateral internal carotid artery stenosis and its distal branches. When angiographic findings are unilateral, the diagnosis is considered to be probable MMD. Surgery is recommended treatment of patients with deterioration caused by progressive cerebral ischemic events. Different techniques have been described, and the encephalo-duro-myo-synangiosis (EDMS) procedure is highly recommended for patients with rapid deterioration. A case of unilateral MMD mimicking intracranial hemorrhage in a pediatric patient is reported. Case description A 14-year-old girl was referred to our center due to a decreased level of consciousness. Physical examination revealed right-sided hemiparesis; however, a brain CT scan showed multiple hyperdense masses mimicking a hemorrhagic lesion. A brain MRI showed a large hypointensity in the temporoparietal, suggesting a vascular lesion. In all children that exhibit encephalitis, vascular events such as MMD should be considered. An emergency surgical EDMS procedure was performed, and the patient regained consciousness and exhibited no TIAs during the follow-up period. A postoperative brain MRI showed an improvement in brain vascularity. Conclusion After EDMS, a considerable degree of neurological recovery was observed in our patient with rare unilateral MMD mimicking intracranial hemorrhage. We found that the progression of clinical improvement after indirect revascularization in our case was due to EDMS. Surgical treatment with EDMS is reasonable for MMD because it allows for flexible revascularization that adequately addresses the requirement for new blood supply in the ischemic lesion. The EDMS procedure is followed by an observation period to preclude the possibility of the patient developing definite MMD.