CC BY-NC-ND 4.0 · Asian J Neurosurg 2021; 16(04): 850-853
DOI: 10.4103/ajns.AJNS_468_20
Case Report

Revisiting dandy–Walker malformation with associated neurofibromatosis

Shreykumar Shah
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
,
Jaypalsinh Gohil
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
,
Krishnakumar Kesavapisharady
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
,
Harihara Easwer
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
› Author Affiliations

This report describes a very rare Dandy–Walker malformation (DWM) associated with neurofibromatosis (NF) and bony defect over torcula emphasizing the role of meticulous follow-up for asymptomatic DWM. The clinical aspects of an adolescent patient with undiagnosed DWM who was asymptomatic until the age of 14 years are being discussed. Computed tomography and magnetic resonance imaging were revealed DWM. To our knowledge, this is the first report from India that describes a patient who has been diagnosed with DWM with associated NF with bony defect over torcula creating a management dilemma.

Financial support and sponsorship

Nil.




Publication History

Received: 14 October 2020

Accepted: 21 August 2021

Article published online:
16 August 2022

© 2021. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Spennato P, Mirone G, Nastro A, Buonocore MC, Ruggiero C, Trischitta V, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst 2011;27:1665-81.
  • 2 Bosemani T, Orman G, Boltshauser E, Tekes A, Huisman TA, Poretti A. Congenital abnormalities of the posterior fossa. Radiographics 2015;35:200-20.
  • 3 Imataka G, Yamanouchi H, Arisaka O. Dandy-Walker syndrome and chromosomal abnormalities. Congenit Anom (Kyoto) 2007;47:113-8.
  • 4 Dandy WE, Blackfan KD. An experimental, clinical and pathological study: Part 1.–Experimental studies. Am J Dis Child 1914;8:406-82.
  • 5 Osenbach RK, Menezes AH. Diagnosis and management of the Dandy-Walker malformation: 30 years of experience. Pediatr Neurosurg 1992;18:179-89.
  • 6 Pascual-Castroviejo I, Velez A, Pascual-Pascual SI, Roche MC, Villarejo F. Dandy-Walker malformation: Analysis of 38 cases. Childs Nerv Syst 1991;7:88-97.
  • 7 Dandy WE. The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg Gynec Obstet 1921;32:112-24.
  • 8 Brodal A, Hauglie-Hanssen E. Congenital hydrocephalus with defective development of the cerebellar vermis (Dandy-Walker syndrome) clinical and anatomical findings in two cases with particular reference to the so-called atresia of the foramina of Magendie and Luschka. J Neurol Neurosurg Psychiatry 1959;22:99-108.
  • 9 Hart MN, Malamud N, Ellis WG. The Dandy-Walker syndrome. Neurology 1972;22:771.
  • 10 Reeder MR, Botto LD, Keppler-Noreuil KM, Carey JC, Byrne JL, Feldkamp ML, et al. Risk factors for Dandy–Walker malformation: A population-based assessment. Am J Med Genet Part A 2015;167A: 2009-16.
  • 11 Kaplan LC. Congenital Dandy Walker malformation associated with first trimester warfarin: A case report and literature review. Teratology 1985;32:333-7.
  • 12 Reefhuis J, Honein MA, Schieve LA, Rasmussen SA; National Birth Defects Prevention Study. Use of clomiphene citrate and birth defects, National Birth Defects Prevention study, 1997-2005. Hum Reprod 2011;26:451-7.
  • 13 Alam A, Chander BN, Bhatia M. Dandy-Walker variant: Prenatal diagnosis by ultrasonography. Med J Armed Forces India 2004;60:287-9.
  • 14 Sautreaux JL, Giroud M, Dauvergne M, Nivelon JL, Thierry A. Dandy-Walker malformation associated with occipital meningocele and cardiac anomalies: A rare complex embryologic defect. J Child Neurol 1986;1:64-6.
  • 15 Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988;45:575-8.
  • 16 Na M, Xie C, Wang H, Shen H, Lin Z. Calvarial defects and Dandy-Walker malformation in association with neurofibromatosis type 1. Chin Med J (Engl) 2014;127:1187-8.
  • 17 Sander A, Dörrler J, von Einsiedel HG, Horch HH. Rare combination of neurofibromatosis and Dandy-Walker syndrome. Dtsch Z Mund Kiefer Gesichtschir 1989;13:433-43.
  • 18 Koul RL, Chacko A, Leven HO. Dandy-Walker syndrome in association with neurofibromatosis in monozygotic twins. Saudi Med J 2000;21:390-2.
  • 19 Arrington DK, Danehy AR, Peleggi A, Proctor MR, Irons MB, Ullrich NJ. Calvarial defects and skeletal dysplasia in patients with neurofibromatosis Type 1: Clinical article. J Neurosurg Pediatr 2013;11:410-6.
  • 20 Jurcă MC, Kozma K, Petcheşi CD, Bembea M, Pop OL, MuŢiu G, et al. Anatomic variants in Dandy-Walker complex. Rom J Morphol Embryol 2017;58:1051-5.
  • 21 Infante JR, Garcia L, Rayo JI, Serrano J, Dominguez ML, Moreno M. PET/CT in a patient diagnosed With Dandy-Walker syndrome. Clin Nucl Med 2016;41:e58-59.
  • 22 Stambolliu E, Ioakeim-Ioannidou M, Kontokostas K, Dakoutrou M, Kousoulis AA. The most common comorbidities in Dandy-Walker syndrome patients: A systematic review of case reports. J Child Neurol 2017;32:886-902.