Atypical venous drainage system and distinct vascular characteristics in pediatric intracerebral hemorrhage caused by multiple micro arteriovenous malformations

Hemorrhagic brain micro-arteriovenous malformations (micro-AVMs) are considered to constitute a relatively significant portion of pediatric AVMs, though they are often associated with a low bleeding rate, as seen in hereditary hemorrhagic telangiectasia, which frequently involves multiple micro-AVMs.

We present a rare case of a 10-year-old girl with multiple hemorrhagic micro-AVMs. Intraoperative findings during the emergency operation for hematoma evacuation and post-operative superselective angiography highlighted the unique angioarchitecture of three micro-AVMs (two lesions in the superficial areas and one lesion in the deep-seated area) and the atypical bleeding source due to the complex congestive venous drainage system. One micro-AVM was successfully occluded by a transarterial emboliozation, and remaining two micro-AVMs underwent gamma knife irradiation as a salvage therapy.

Superselective angiography is crucial for detecting micro-AVMs, offering detailed insights into small, localized abnormal vascular drainage systems, and guiding therapeutic strategy. Additionally, micro-AVM-associated unique vascular hypersensitivity, such as vasospasm, requires careful consideration, as invasive procedures may significantly alter the visibility of the entire micro-AVM network.