A Case Report of Neurocutaneous Melanosis with Associated Dandy-Walker Complex

Document Type : Case Report

Authors
Mohamad Hosein Lookzadeh 1
Razieh Sadat Tabatabaie 2
Maryam Saeida- Ardekani 1
Hanie Bakhshayesh 3
1 Mother and Newborn Health Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
2 Department of Obstetrics and Gynecology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
3 Department of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
10.18502/wjpn.v5i2.11996
Abstract
Background: Neurocutaneous melanosis (NCM) and Dandy-Walker malformation (DWM) are two forms of rare congenital neurodysplasia. NCM is a rare dysmorphogenesis characterized by single or multiple giants pigmented cutaneous nevi and the involvement of benign and/or malignant melanocytic tumors of the leptomeninges. DWM is a rare congenital malformation of the brain. Cystic enlargement of the fourth ventricle is its characteristic that communicates with an enlarged posterior fossa, cerebellar dysgenesis, high tentorial insertion, and hydrocephalus. However, these two conditions are rare, and NCM associated with DWC is even more unusual.
Case Report: Here, we report a male newborn with macrocephaly and multiple pigmented nevi over his whole body with regular borders and normal weight, height, and spine. He was finally diagnosed as NCM in association with DWC.
Conclusion: After diagnosing NCM in association with DWC, appropriate follow-up is recommended; however, there is no particular treatment to prevent the malignant change.
Keywords
Neurocutaneous melanosis
Dandy-Walker malformation
Dysmorphogenesis
Neurocutaneous syndrome
Melanoma
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Journal of Integrated Maternal and Pediatric Care
Volume 5, Issue 2
December 2022
Pages 96-98

  • Receive Date 26 December 2022