Hypopituitarism due to hydrocephalus: case report and review of the literature.

BACKGROUND

Although rare, chronic hydrocephalus may cause amenorrhea and delayed puberty associated with obesity as the main endocrine manifestations. Since the first operation carried out in 1950 on a patient with amenorrhea and hydrocephalus, fewer than 30 patients with these features have been reported.

METHODS

Our patient represents a teenage case of primary amenorrhea, partial adrenal insufficiency and adult growth hormone deficiency caused by hydrocephalus due to aqueductal stenosis. Our English-language literature search for similar well-documented cases yielded less than 30 cases. Our management consisted of endoscopic third ventriculostomy, which resulted in regular menstrual cycles and normalization of somato- and adrenocorticotropic axes.

CONCLUSIONS

This is a rare case of a 20-year-old woman with primary amenorrhea caused by hydrocephalus due to aqueductal stenosis. Neurosurgical treatment produced relief in hypothalamic hydrodynamics tension, allowing recovery of hormonal function and restoring normal menstrual cycles.