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

Document Type : Case Report

Authors

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

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

References

  1. Cho IY, Hwang SK, KIM SH. Dandy-Walker malformation associated with neurocutaneous melanosis. J Korean Neurosurg Soc 2011; 50(5): 475-7. 
  2. Swar MO, Mahgoub SM, Yassin RO, Osman AM. Dandy-Walker malformation and neurocutaneous melanosis in a three-month-old infant. Sudan J Paediatr 2013; 13(2): 61-5. 
  3. Elsagh A, Jashni Motlagh AR. Association of Dandy-Walker malformation and neurocutaneous melanosis in a newborn: A case report. Iran J Neonatol 2019; 10(3): 103-7. 
  4. Sarwar M, Tripathy L, Jain H, Basu S, Sengupta A. Neurocutaneous melanosis with hydrocephalus and Dandy-Walker variant. Asian J Neurosurg. 2021; 16(4): 876-80. 
  5. Marnet D, Vinchon M, Mostofi K, Catteau B, Kerdraon O, Dhellemmes P. Neurocutaneous melanosis and the Dandy-Walker complex: uncommonly but not so insignificant association. Childs Nerv Syst 2009; 25(12): 1533-9. 
  6. Ruggieri M, Polizzi A, Catanzaro S, Lo Bianco M, D Praticò A, Di Rocco C. Neurocutaneous melanocytosis (melanosis). Child's Nerv Syst 2020; 36(10): 2571-96. 
  7. Schreml S, Gruendobler B, Schreml J, Bayer M, Ladoyanni E, Prantl L, et al. Neurocutaneous melanosis in association with Dandy-Walker malformation: case report and literature review. Clin Exp Dermatol 2008; 33(5): 611-4. 
  8. Sung KS, Song YJ. Neurocutaneous melanosis in association with dandy-walker complex with extensive intracerebral and spinal cord involvement. J Korean Neurosurg Soc 2014; 56(1):61. 
  9. Kim KH, Chung SB, Kong DS, Seol HJ, Shin HJ. Neurocutaneous melanosis associated with Dandy-Walker complex and an intracranial cavernous angioma. Childs Nerv Syst 2012; 28(2): 309-14.
World Journal of Peri and Neonatology
Volume 5, Issue 2
December 2022
Pages 96-98

Files

History

  • Receive Date: 26 December 2022
  • Accept Date: 26 December 2022

Share

How to cite

Statistics