Pediatric Cavernous Malformations

Pediatric cavernous malformations (CMs) are rare lesions that arise congenitally or de novo secondary to radiation therapy. Most CMs occur spontaneously, with a small portion inherited genetically. Symptomatology relates to location with supratentorial CMs more often presenting with seizures, and infratentorial presenting with neurologic deficits from a small annual hemorrhage risk. CT and MRI have high sensitivities in lesion detection, while angiography is typically negative. Management decisions should largely be based on symptoms, location, hemorrhage risk and seizure control. While a majority of seizures are controlled with medications, up to 30% remain drug-resistant. Surgical resection significantly reduces seizure burden, with about 3/4 achieving Engel Class I overall. A larger proportion experience seizure freedom with resection of the perilesional hemosiderin rim in addition to the lesion itself. Brainstem CMs incur a high complication risk; nevertheless certain attributes, such as large, exophytic lesions or those <2 mm from the pial surface, should prompt consideration for resection. Radiosurgery provides questionable benefit with significant complication and thus should be avoided. Alternative modalities such as magnetic-resonance guided focused ultrasound or laser interstitial thermal therapy hold potential as viable treatment options, although further investigation is required.