Journal of Pediatrics Review

Published by: Kowsar

Dandy-Walker Syndrome: A Review of New Diagnosis and Management in Children

Kaveh Haddadi 1 , * , Amirhossein Zare 2 and Leila Asadian 2
Authors Information
1 Department of Neurosurgery, Spine Fellowship Scholar of Boston University Medical Center, Orthopedic Research Center, Mazandaran University of Medical Sciences, Sari, Iran
2 Orthopedic Research Center, Mazandaran University of Medical Sciences, Sari, Iran
Article information
  • Journal of Pediatrics Review: July 2018, 6 (2); e63486
  • Published Online: April 25, 2018
  • Article Type: Review Article
  • Received: October 31, 2017
  • Accepted: November 6, 2017
  • DOI: 10.5812/jpr.63486

To Cite: Haddadi K, Zare A, Asadian L. Dandy-Walker Syndrome: A Review of New Diagnosis and Management in Children, J Pediatr Rev. 2018 ;6(2):e63486. doi: 10.5812/jpr.63486.

Copyright © 2018, Journal of Pediatrics Review. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited
1. Context
2. Evidence Acquisition
3. Results
4. Conclusions
  • 1. Dandy WE. The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka. Surg Gynecol Obstet. 1921;32:113-24.
  • 2. Taggart JK. Congenital Atresia of the Foramens of Luschka and Magendie. Arch Neurol Psychiatry. 1942;48(4):583-612. doi: 10.1001/archneurpsyc.1942.02290100083008.
  • 3. Benda CE. The Dandy-Walker syndrome or the so-called atresia of the foramen Magendie. J Neuropathol Exp Neurol. 1954;13(1):14-29. doi: 10.1093/jnen/13.1.14. [PubMed: 13118372].
  • 4. Raimondi AJ, Samuelson G, Yarzagaray L, Norton T. Atresia of the foramina of Luschka and Magendie: the Dandy-Walker cyst. J Neurosurg. 1969;31(2):202-16. doi: 10.3171/jns.1969.31.2.0202. [PubMed: 5306614].
  • 5. Osenbach RK, Menezes AH. Diagnosis and management of the Dandy-Walker malformation: 30 years of experience. Pediatr Neurosurg. 1992;18(4):179-89. doi: 10.1159/000120660. [PubMed: 1472430].
  • 6. Mohanty A, Biswas A, Satish S, Praharaj SS, Sastry KV. Treatment options for Dandy-Walker malformation. J Neurosurg. 2006;105(5 Suppl):348-56. doi: 10.3171/ped.2006.105.5.348. [PubMed: 17328256].
  • 7. Haddadi K. Outlines and outcomes of instrumented posterior fusion in the pediatric cervical spine: A review article. J Pediatr Rev. 2016;4(1). doi: 10.17795/jpr-4765.
  • 8. Haddadi K. Pediatric lumbar disc herniation: A review of manifestations, diagnosis and management. J Pediatr Rev. 2016;4(1). doi: 10.17795/jpr-4725.
  • 9. Haddadi K. Pediatric endoscopic third ventriculostomy: A narrative review of current indications, techniques and complications. J Pediatr Rev. 2016;4(2). doi: 10.17795/jpr-5074.
  • 10. Alaedini K, Haddadi K, Asadian L. A review of neurobehavioral challenges in children exposed prenatally to intrauterine opioid. J Pediatr Rev. 2017;5(2). doi: 10.5812/jpr.9234.
  • 11. Steiner C, Elixhauser A, Schnaier J. The healthcare cost and utilization project: an overview. Eff Clin Pract. 2002;5(3):143-51. [PubMed: 12088294].
  • 12. Alexiou GA, Sfakianos G, Prodromou N. Dandy-Walker malformation: analysis of 19 cases. J Child Neurol. 2010;25(2):188-91. doi: 10.1177/0883073809338410. [PubMed: 19833975].
  • 13. Grinberg I, Northrup H, Ardinger H, Prasad C, Dobyns WB, Millen KJ. Heterozygous deletion of the linked genes ZIC1 and ZIC4 is involved in Dandy-Walker malformation. Nat Genet. 2004;36(10):1053-5. doi: 10.1038/ng1420. [PubMed: 15338008].
  • 14. Imataka G, Yamanouchi H, Arisaka O. Dandy-Walker syndrome and chromosomal abnormalities. Congenit Anom (Kyoto). 2007;47(4):113-8. doi: 10.1111/j.1741-4520.2007.00158.x. [PubMed: 17988252].
  • 15. Li PG, Tang YN, Zheng SS, Chen WK, Lu LP. Dandy-Walker Malformation and Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episode Syndrome: Is There a Causal or Coincidental Association? Chin Med J (Engl). 2016;129(5):620-1. doi: 10.4103/0366-6999.176984. [PubMed: 26905003].
  • 16. Aldinger KA, Lehmann OJ, Hudgins L, Chizhikov VV, Bassuk AG, Ades LC, et al. FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nat Genet. 2009;41(9):1037-42. doi: 10.1038/ng.422. [PubMed: 19668217].
  • 17. Klein O, Pierre-Kahn A, Boddaert N, Parisot D, Brunelle F. Dandy-Walker malformation: prenatal diagnosis and prognosis. Childs Nerv Syst. 2003;19(7-8):484-9. doi: 10.1007/s00381-003-0782-5. [PubMed: 12879343].
  • 18. Ecker JL, Shipp TD, Bromley B, Benacerraf B. The sonographic diagnosis of Dandy-Walker and Dandy-Walker variant: associated findings and outcomes. Prenat Diagn. 2000;20(4):328-32. [PubMed: 10740206].
  • 19. Shekdar K. Posterior fossa malformations. Semin Ultrasound CT MR. 2011;32(3):228-41. doi: 10.1053/j.sult.2011.02.003. [PubMed: 21596278].
  • 20. Alam A, Chander BN, Bhatia M. Dandy-Walker Variant : Prenatal Diagnosis by Ultrasonography. Med J Armed Forces India. 2004;60(3):287-9. doi: 10.1016/S0377-1237(04)80066-2. [PubMed: 27407651].
  • 21. Guz E, Krzysztof T, Marta C, Elżbieta. B . Dandy-Walker Syndrome - review of patients' health situation in Poland. J Educ Health Sport. 2016;6(8):181-9.
  • 22. Sasaki-Adams D, Elbabaa SK, Jewells V, Carter L, Campbell JW, Ritter AM. The Dandy-Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatr. 2008;2(3):194-9. doi: 10.3171/PED/2008/2/9/194. [PubMed: 18759601].
  • 23. Lavanya T, Cohen M, Gandhi SV, Farrell T, Whitby EH. A case of a Dandy-Walker variant: the importance of a multidisciplinary team approach using complementary techniques to obtain accurate diagnostic information. Br J Radiol. 2008;81(970):e242-5. doi: 10.1259/bjr/77399621. [PubMed: 18796551].
  • 24. Tadakamadla J, Kumar S, Mamatha GP. Dandy-Walker malformation: An incidental finding. Indian J Hum Genet. 2010;16(1):33-5. doi: 10.4103/0971-6866.64936. [PubMed: 20838490].
  • 25. Kohler BA, Ward E, McCarthy BJ, Schymura MJ, Ries LA, Eheman C, et al. Annual report to the nation on the status of cancer, 1975-2007, featuring tumors of the brain and other nervous system. J Natl Cancer Inst. 2011;103(9):714-36. doi: 10.1093/jnci/djr077. [PubMed: 21454908].
  • 26. Blaettner C, Pfaffenberger NM, Cartes-Zumelzu F, Hofer A. Psychiatric misdiagnoses in Dandy-Walker variant. Neurocase. 2015;21(4):499-500. doi: 10.1080/13554794.2014.940978. [PubMed: 25058305].
  • 27. Khatua S, Sadighi ZS, Pearlman ML, Bochare S, Vats TS. Brain tumors in children--current therapies and newer directions. Indian J Pediatr. 2012;79(7):922-7. doi: 10.1007/s12098-012-0689-9. [PubMed: 22294272].
  • 28. Forzano F, Mansour S, Ierullo A, Homfray T, Thilaganathan B. Posterior fossa malformation in fetuses: a report of 56 further cases and a review of the literature. Prenat Diagn. 2007;27(6):495-501. doi: 10.1002/pd.1722. [PubMed: 17367101].
  • 29. McClelland S3, Ukwuoma OI, Lunos S, Okuyemi KS. The natural history of Dandy-Walker syndrome in the United States: A population-based analysis. J Neurosci Rural Pract. 2015;6(1):23-6. doi: 10.4103/0976-3147.143185. [PubMed: 25552847].
  • 30. Mendelson SG. Maternal grand mal seizure leads to a surprising diagnosis of Dandy-Walker variant. J Obstet Gynecol Neonatal Nurs. 2011;40(4):458-62. doi: 10.1111/j.1552-6909.2011.01267.x. [PubMed: 21771071].
  • 31. Salihu HM, Kornosky JL, Druschel CM. Dandy-Walker syndrome, associated anomalies and survival through infancy: a population-based study. Fetal Diagn Ther. 2008;24(2):155-60. doi: 10.1159/000142146. [PubMed: 18648217].
  • 32. ten Donkelaar HJ, Lammens M, Wesseling P, Thijssen HO, Renier WO. Development and developmental disorders of the human cerebellum. J Neurol. 2003;250(9):1025-36. doi: 10.1007/s00415-003-0199-9. [PubMed: 14504962].
  • 33. Paladini D, Volpe P. Posterior fossa and vermian morphometry in the characterization of fetal cerebellar abnormalities: a prospective three-dimensional ultrasound study. Ultrasound Obstet Gynecol. 2006;27(5):482-9. doi: 10.1002/uog.2748. [PubMed: 16619375].
  • 34. Long A, Moran P, Robson S. Outcome of fetal cerebral posterior fossa anomalies. Prenat Diagn. 2006;26(8):707-10. doi: 10.1002/pd.1485. [PubMed: 16764010].
  • 35. Salihu HM, Kornosky JL, Alio AP, Druschel CM. Racial disparities in mortality among infants with Dandy-Walker syndrome. J Natl Med Assoc. 2009;101(5):456-61. doi: 10.1016/S0027-9684(15)30932-9. [PubMed: 19476199].
  • 36. Warf BC, Dewan M, Mugamba J. Management of Dandy-Walker complex-associated infant hydrocephalus by combined endoscopic third ventriculostomy and choroid plexus cauterization. J Neurosurg Pediatr. 2011;8(4):377-83. doi: 10.3171/2011.7.PEDS1198. [PubMed: 21961544].
  • 37. Nigri F, Cabral IF, da Silva RT, Pereira HV, Ribeiro CR. Dandy-walker malformation and down syndrome association: good developmental outcome and successful endoscopic treatment of hydrocephalus. Case Rep Neurol. 2014;6(2):156-60. doi: 10.1159/000363179. [PubMed: 24932176].
  • 38. Simpkins CJ. Ventriculoperitoneal shunt infections in patients with hydrocephalus. Pediatr Nurs. 2005;31(6):457-62. [PubMed: 16411537].
  • 39. Boddaert N, Klein O, Ferguson N, Sonigo P, Parisot D, Hertz-Pannier L, et al. Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 2003;45(5):320-4. doi: 10.1007/s00234-003-0980-6. [PubMed: 12682795].
Creative Commons License Except where otherwise noted, this work is licensed under Creative Commons Attribution Non Commercial 4.0 International License .

Search Relations:



Create Citiation Alert
via Google Reader

Readers' Comments