Surgical resection of a solid posterior cranial fossa hemangioblastoma causing obstructive hydrocephalus in a...

Hemangioblastomas (HBs) are vascular neoplasms that are most involved with the central nervous system and can be associated with Von Hippel-Lindau disease (VHL). HBs involving pediatric patients are common, but VHL-associated HBs and those that cause obstructive hydrocephalus are rare.

In this case study, a pediatric patient presented with multiple neurological deficits including headaches, ataxia, confusion, and right-sided weakness, as well as a magnetic resonance image revealing a lesion involving the 4^th ventricle causing obstructive hydrocephalus. An external ventricular drain was placed to treat the hydrocephalus acutely, followed by a tumor resection, resulting in some postsurgical bleeding and increased fluid accumulation. Further genetic testing revealed that the patient was positive for a VHL mutation.

There are only a few reports of intraventricular HBs that are found in the 4^th ventricle in pediatric patients with less discussing solid HBs, so evaluation of this case can provide a valuable opportunity to compare and reflect on the nature of this rare presentation and contribute toward understanding the best approaches for treating these rare conditions.