Adrenergic Storm with Obstructive Hydrocephalus: Atypical Neurological Presentation of Von Hippel-Lindau Disease with Bilateral Pheochromocytoma in an Adolescent.

Pheochromocytoma is often not the first diagnosis considered for many neurological complaints, especially obstructive hydrocephalus. We report a case of Von Hippel-Lindau disease in an adolescent presenting with obstructive hydrocephalus due to adrenergic storm requiring emergency ventriculoperitoneal shunt. This vignette emphasizes the importance of an integral approach, particularly accurate diagnosis that would lead to targeted pharmacological therapy focusing on alphaa-drenergic receptor blockade, which was followed by total curative excision of pheochromocytoma in a timely fashion.