Von Hippel-Lindau disease with intracranial hemorrhage due to arteriovenous anastomosis via multiple spinal hemangioblastomas: illustrative case

BACKGROUND: Some spinal hemangioblastomas (HBLs) resemble spinal vascular malformations. Intracranial subarachnoid hemorrhage (SAH) secondary to spinal HBL has rarely been reported. OBSERVATIONS: A 67-year-old man with a prolonged von Hippel-Lindau disease (VHL) history presented with sudden headache and vomiting. Cranial and cervical computed tomography (CT) revealed severe infratentorial, supratentorial, and cervical SAH. Cranial CT angiography and magnetic resonance imaging revealed a mismatch in hemorrhage and intracranial tumor localization, with no vascular lesions that could lead to intracranial SAH. Cervical CT angiography revealed abnormal blood vessels originating from 5 spinal tumors suspected to be HBLs. We considered that the SAH was caused by venous reflex from vascular malformation–like spinal HBLs. Transarterial embolization (TAE) of the feeding artery of HBLs was performed to improve symptoms and reduce rebleeding risk. Nine months after TAE, angiography showed no venous reflux into the intracranial space. Ten months later, the authors excised the T1–2 tumor because the patient complained of progressive paralysis of the right upper extremity. LESSONS: In HBL with prolonged VHL, intracranial hemorrhage due to venous regurgitation via a mimicked vascular malformation may occur. Reducing venous reflux with TAE may improve symptoms and prevent rebleeding.