Neuroendoscopic Findings of Ventricular Wall in Adult Hemorrhagic Moyamoya Disease: Report of Two Cases

Moyamoya disease usually manifests as ischemic events in childhood, and as more severe hemorrhagic events, including intraventricular hemorrhage, in adults. Recently, the indication for neuroendoscopic surgery has been extended to cast-formation intraventricular hematomas. However, detailed information about the use of neuroendoscopic surgery for the treatment of intraventricular hemorrhage associated with moyamoya disease has not been reported. We describe two cases of intraventricular hemorrhage with moyamoya disease; one in a 62-year-old and another in a 33-year-old women who both presented with severe neurological symptoms. Cerebral angiography revealed unilateral moyamoya disease. Neuroendoscopic surgery to remove the intraventricular hematoma was performed via bilateral frontal burr holes in both cases. Abnormal findings in the ventricle were observed only in the affected side and the intact side was normal. Specific findings of neuroendoscopic observation were dilated and tortuous vessels, intersection vessels, black-brown macules in the subependyma, and rattan blind-like (Japanese sudare) bleeding vessels. These characteristic neuroendoscopic findings may be useful for the exact diagnosis and treatment of intraventricular hemorrhage associated with moyamoya disease. Endoscopic evacuation of the ventricular hematoma may be important for intracranial pressure control in patients with intraventricular hemorrhage in adult moyamoya disease.