Objectives This study presents a case report and literature review of acetazolamide use in the pregnant patient to further delineate its role and controversies in the treatment of symptomatic hydrocephalus. Here we present a case from our neurosurgical service which demonstrated safe use and efficacy of acetazolamide in refractory hydrocephalus secondary to a space-occupying lesion.

Design Case report and literature review.

Subjects 33-year-old, primigravid female treated at Charing Cross Hospital, London.

Methods Adaptation of the PRISMA guideline to facilitate an initial PubMed database search for all available full-text, peer-reviewed publications in English. Papers were screened for relevance to pregnant patients with hydrocephalus receiving acetazolamide therapy, as well as relevant papers describing related disorders of cerebrospinal fluid flow.

Results There exist no prospective, randomised-controlled trials for the use of acetazolamide to treat hydrocephalus in pregnancy. Retrospective data in patients with related conditions suggest no significant link between acetazolamide use and teratogenicity in humans.

Conclusions Acetazolamide can be a safe and efficacious therapy in the pregnant patient with refractory hydrocephalus as a bridge to delivery. We agree with the consensus and its appropriate use in the pregnant neurosurgical patient.