Hydrocephalus-related amenorrhoea: A scoping review

Hydrocephalus is a rare, potentially reversible cause of gonadotropic dysfunction, amenorrhoea, and delayed puberty; fewer than 60 cases have been reported. This scoping review aimed to characterize hydrocephalus-associated amenorrhoea, assess time to postoperative menstrual (re)occurrence, and identify factors influencing recovery.

This scoping review followed Joanna Briggs Institute methodology, with a systematic search of PubMed, Embase, Google Scholar, and Web of Science for cases of amenorrhoea associated with hydrocephalus. Two reviewers independently screened records and extracted data, with disagreements resolved by a third reviewer; data were synthesized descriptively using non-parametric tests to explore selected associations.

Fifty-eight cases met inclusion criteria. Aqueductal stenosis was the most common aetiology (30/53; 56.6%), and most patients presented with primary amenorrhoea (38/57; 66.7%). Menstruation returned after treatment in 36/44 cases with known outcome (81.8%), mostly after surgery, at a median of 90 days where reported (IQR 30-210), though delays of almost five years occurred in a minority. Recovery was significantly faster after shunt placement than ventriculostomy, possibly reflecting differences in aetiology and selection bias.

Hydrocephalus-associated amenorrhoea, most often due to aqueductal stenosis, likely reflects hypothalamic GnRH disruption from ventricular dilation. Surgical decompression usually restores menstrual cycles, though timing varies with baseline pubertal status. Early recognition is critical in adolescents with unexplained hypogonadotropic hypogonadism; because menstruation may resume quickly postoperatively, preoperative contraceptive counselling is advised in postpubertal patients. These findings underscore the need for multidisciplinary management and further research into long-term endocrine and reproductive outcomes.