Systematic scoping review of papilledema in vestibular schwannoma without hydrocephalus

Background: Vestibular schwannoma is a common pathology encountered by neurosurgeons worldwide. Often vestibular schwannoma presents with obstructive hydrocephalus. Papilledema is present in 8% of the patients with vestibular schwannoma, primarily due to obstructive hydrocephalus. Hyperproteinorrhachia is believed to be responsible for papilledema in the absence of hydrocephalus in vestibular schwannoma. However, there is a paucity of literature on the mechanism of papilledema in vestibular schwannoma patients with hydrocephalus.

Objective: The aim of this study was to conduct a scoping review of scientific literature on papilledema in vestibular schwannoma patients without hydrocephalus.

Methods: Design: This was a systematic scoping review and critical appraisal. Literature Search from PubMed was done following PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews) and Joanna Briggs Institute guidelines for conducting and reporting scoping reviews.

Results: A total of seven studies, including eight patients, were identified for inclusion in the review. The studies were heterogeneous in terms of reporting for various variables. All the included studies were case reports, with the earliest publication in 1954 and the latest publication in 2020. The mean age of the patients in the included studies was 35 years, with a minimum age of 20 years and maximum age of 64 years. Approximately 62.5% were females, and 37.5% were males in the included study. Only three studies have studied cerebrospinal fluid (CSF) proteins levels in these patients.

Conclusions: There is paucity in literature and a lack of evidence to conclusively state hyperproteinorrhachia as an antecedent to the development of papilledema in vestibular schwannoma patients without hydrocephalus. Younger age and female gender are risk factors for developing papilledema in the absence of hydrocephalus in vestibular schwannoma patients. Brainstem compression due to the large size of vestibular schwannoma can still have a patent aqueduct of Sylvius and no obstruction to CSF flow. The development of papilledema in vestibular schwannoma is a complex interplay of multiple factors that must be studied comprehensively for complete understanding.