Management of Pediatric Pseudotumor Cerebri Syndrome

Pseudotumor cerebri syndrome (PTCS) is a clinical condition characterized by signs and symptoms of elevated intracranial pressure without any radiographic evidence of a mass lesion in the brain, and no abnormalities of the brain parenchyma and cerebrospinal fluid (CSF) composition. Children with PTCS typically present with various combinations of headache, diplopia, and pulsatile tinnitus. Although this syndrome has long been considered a benign condition, it may lead to permanent visual impairment if not promptly and correctly treated. Complete remission of PTCS is associated with a decrease in intracranial pressure, the disappearance of clinical symptoms, and the reversion of papilledema at the funduscopic examination. PTCS has been associated with several comorbidities, but its exact pathophysiology has yet to be fully elucidated. Because the origin of PTCS remains unclear, an etiologically targeted treatment has not yet been identified. Current medical treatments include diuretics (e.g., acetazolamide, furosemide) able to reduce CSF production and pressure, and CSF diversion procedures in the most severe cases. Future elucidation of the pathophysiologic mechanism(s) underlying impaired CSF dynamics in PTCS should help control more finely the CSF formation by choroid plexus with novel drugs having fewer side effects than the commonly used diuretics (e.g., acetazolamide). In this review, we briefly summarize the current therapeutic options in pediatric PTCS.