[Primary amenorrhea revealing congenital hydrocephalus]

Among the various endocrine manifestations of chronic hydrocephaly, amenorrhea is not exceptional, whereas late puberty and obesity are the most frequent. The hypothalamic gonadotropic function usually returns to normal within the few months following the ventricular shunt.

A 16 year-old girl, with a von Recklinghausen's disease, developed amenorrhea 2 years after the onset of puberty. X-rays of her skull showed an enlarged sella turcica and a diagnosis of hydrocephalus due to aqueductal stenosis was finally made. As her head circumference was retrospectively shown at +3 SD as early as the first month of life, the stenosis was certainly congenital, a feature known to occur in 2% of patients with von-Recklinghausen's disease.

In symptomatic forms of aqueductal stenosis, the preferred surgical procedure is ventriculocisternostomy under endoscopy. Recovery of intellectual functions is often incomplete. Early diagnosis of hydrocephalus is of paramount importance; ultrasonography through anterior fontanel should be proposed to any infant with head circumference reaching +3 SD.