Neurocutaneous Melanosis and Dandy-Walker Malformation in a Newborn: A Case Report.

Abstract

Neurocutaneous melanosis (NCM) and Dandy-Walker malformation (DWM) are both uncommon congenital condition, involving the central nervous system (CNS). Combined NCM with DWM have been reported in a few studies. Potential consequences, including hydrocephalus and CNS malignancies make these illnesses extremely challenging to diagnose and treat. Occurrences of NCM and DWM together, can result in progressive neurological consequences and patients with this condition seems to have really poor prognosis. This case report describes a 5-day-old girl who was born with several large pigmented cutaneous nevi all over her body. She was born at term to non-consanguineous parents who had no family history of similar conditions. Imaging studies, such as brain CT scan, showed cystic dilatation of the fourth ventricle, hypoplasia of the cerebellar vermis, enlargement of the posterior fossa, and possibly secondary hydrocephalus-all of which are indicative of DWM. A biopsy of the nevi confirmed NCM. The patient had no neurological symptoms or systemic complications at the time of presentation. The combination of NCM and DWM is extremely rare, with fewer than 40 reported cases so far. While the exact connection between these two conditions is not fully understood, their coexistence of these conditions might be linked to their shared embryonic origin. Both melanocytes and parts of the CNS come from the neural crest during early development. Since these conditions can have serious neurological and systemic effects, managing them requires multidisciplinary care because of potential consequences of these disorders.