• Zohreh Habibi, Hannan Ebrahimi, MeybodiKeyvan TayebiKTDepartment of Pediatric Neurosurgery, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran., Bahareh Yaghmaei, and Farideh Nejat.
• Department of Pediatric Neurosurgery, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran. Electronic address: z-habibi@sina.tums.ac.ir.
• World Neurosurg. 2021 Feb 1; 146: e1063-e1070.

IntroductionNeurocutaneous 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's hospital in Iran.MethodsBetween 2012 and 2019, eight patients with NCM were consecutively managed in our center. Brain magnetic resonance imaging and cutaneous biopsy were done in all patients at diagnosis. Follow-up surveillance and characteristics of the disease are described.ResultsThe mean follow-up period was 25.75 ± 13.81 months, and 75% of patients were male. Most magnetic resonance imaging findings were hypersignal lesions in the temporal lobe (75%), cerebellum (62.5%), brainstem (50%), and thalamus (12.5%). Dandy-Walker syndrome was found in 4 patients (50%), and shunt-dependent hydrocephalus was found in 3 patients (37.5%). Cutaneous malignant melanoma and malignant involvement of the central nervous system were found in 2 (25%) and 3 cases (37.5%), respectively. The mortality rate was 37.5%.ConclusionsThere are no specific guidelines for management of NCM due to the rarity of the disease. This study proposed modifications in diagnostic criteria, as well as recommendations for follow-up surveillance.Copyright © 2020. Published by Elsevier Inc.

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