Poor outcome of bilateral congenital choroid plexus papillomas with extreme hydrocephalus

Bilateral choroid plexus papillomas, or villous hypertrophy of the choroid plexus, is a very rare entity that presents serious therapeutical problems in the management of secondary oversecretive hydrocephalus. The authors report the cases of 2 infants affected by this condition, and cured with removal of the lateral ventricle papillomas and with cerebrospinal fluid (CSF) ventriculoperitoneal shunt insertions. In both cases the CSF shunting alone or the removal of only 1 papilloma did not suffice in controlling the intracranial hypertension. In both infants late outcome was unsatisfactory. The poor prognosis associated with bilateral choroid papillomas in the infants described in the present report may be explained by the precocious onset of the disease, diagnosed during fetal life, and by the marked cerebral alterations already apparent in the immediate postnatal period on the neuroradiological examinations. Repeated surgical procedures, which were necessary to control the associated hydrocephalus in these patients, might have also played a significant negative role.