LAUSR

INTRODUCTION Neurocutaneous melanosis (NCM) is a rare congenital syndrome. Except for some retrospective studies, information on clinical follow-up and management of these patients are limited. This study aimed to review our experience on diagnostic protocol and clinical follow-up of patients with NCM in a referral children hospital in Iran. METHODS Between 2012 and 2019, eight patients with NCM were consecutively managed in our center. Brain magnetic resonance imaging (MRI) and cutaneous biopsy were done in all patients at diagnosis. Follow-up surveillance and the characteristics of disease are described. RESULTS The mean follow-up period was 25.75 ±13.81 months and 75% of patients were male. The most MRI findings were hyper-signal lesions in the temporal lobe (75%), cerebellum (62.5%%), brainstem (50%), and thalamus (12.5%), respectively. Dandy-Walker syndrome (DWS) was found in 4 (50 %) and shunt dependent hydrocephalus in 3patients (37.5%). Cutaneous malignant melanoma and malignant involvement of CNS were found in 2 (25%) and 3 cases (37.5%), respectively. The mortality rate was 37.5%. CONCLUSION There are no specific guidelines for the management of NCM due to rarity of the disease. This study proposed some modifications in diagnostic criteria as well as some recommendations for follow-up surveillance.