PRIMARY AMENORRHEA AS THE FIRST MANIFESTA TION OF A CONGENITAL HYDROCEPHALUS

Among the various endocrine manifestations of chronic hydrocephaly, am enorrhea 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. Case report. - A 16 year-old girl, with a von Recklinghausen's disease, dev eloped amenorrhea 2 years after the onset of puberty. X-rays of her sk ull 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 li fe, the stenosis was certainly congenital, a feature known to occur in 2% of patients with von-Recklinghausen's disease. Conclusion. - In sy mptomatic forms of aqueductal stenosis, the preferred surgical procedu re is ventriculocisternostomy under endoscopy. Recovery of intellectua l functions is often uncomplete. Early diagnosis of hydrocephalus is o f paramount importance; ultrasonography through anterior fontanel shou ld be proposed to any infant with head circumference reaching + 3 SD.