Neurocutaneous melanosis with hydrocephalus: report of one case.

Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome and characterized by large or numerous congenital melanocytic nevi and excessive proliferation of melanin-containing cells in the leptomeninges. It is believed to be an embryonic neuroectodermal dysplasia. Patients with NCM may develop severe hydrocephalus and other neurological symptoms with extremely poor prognosis. We report an infant with multiple large congenital melanocytic nevi and hydrocephalus. He was admitted to our hospital due to intermittent projectile vomiting and irritable crying for one week. CSF cytology and brain magnetic resonance imaging revealed central nervous system involvement. His condition was much improved after ventriculoperitoneal shunting. Even though patients with NCM and hydrocephalus may have normal growth and development after shunt insertion, close follow-up for these patients is still warranted.