Developmental venous anomaly presenting as a spontaneous intraparenchymal hematoma without thrombosis

Developmental venous anomalies (DVAs) are cited as the most common cerebral vascular malformations. Still, intracerebral hematomas are rarely thought to be caused by DVAs. In this report, the authors present a unique case of a DVA that hemorrhaged spontaneously, rather than hemorrhaging into a venous infarction following DVA thrombosis as has been more commonly reported.

A 22-year-old previously healthy male presented to the emergency department with a severe headache, confusion, and progressive hemiparesis. A computed tomography (CT) scan demonstrated a spontaneous left parietal intraparenchymal hemorrhage (IPH), with intraventricular extension and acute hydrocephalus. CT angiography did not demonstrate an underlying vascular malformation. The patient was taken emergently to the operating room for a left parietal craniotomy for evacuation of the hematoma. Intraoperative pathology was consistent with a DVA Postoperative magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), and magnetic resonance venography (MRV) did not demonstrate a mass lesion, ischemic stroke, or underlying vascular malformation. An MRI obtained three years previously for headaches was normal. A postoperative diagnostic cerebral angiogram was normal. An MRI/MRA performed six months postoperatively demonstrated two foci of abnormal vessels on susceptibility-weighted imaging (SWI), suggesting the presence of a venous vascular malformation. A diagnostic cerebral angiogram obtained six months postoperatively was again normal, including delayed imaging.

Few reports have cited DVA as the sole cause of intracerebral hemorrhage. While very rare, these reports suggest hemorrhagic conversion of a venous infarction secondary to a thrombosed DVA as a possible etiology, and several provide imaging consistent with this diagnosis. This case study demonstrates a unique presentation of a hemorrhagic DVA in the absence of thrombosis or stroke.