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

BACKGROUND

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.

CASE DESCRIPTION: 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 4th 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.

CONCLUSION

There are only a few reports of intraventricular HBs that are found in the 4th 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.