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Dandy-Walker Malformations

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Handbook of the Cerebellum and Cerebellar Disorders

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Abstract

Dandy-Walker malformation (DWM) is a rare congenital disorder that involves the cerebellum and the fourth ventricle. It is defined as agenesis of cerebellar vermis, cystic dilation of the fourth ventricle, and large posterior fossa with upward displacement of the tentorium, lateral sinuses, and torcular. The exact etiology of DWM remains unknown. Most patients are diagnosed within the first year of life. In nearly 90% of cases there is an accompanying hydrocephalus. DWM may be associated with several central nervous system and extracranial anomalies. Nearly half of these patients will have mental disability and approximately 20% will develop seizures. Treatment often includes surgical management of hydrocephalus.

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References

  • Alexiou GA, Prodromou N (2010) Dandy-Walker malformation in Crouzon syndrome. J Child Neurol 25:653

    Article  Google Scholar 

  • Alexiou GA, Sfakianos G, Prodromou N (2010a) Dandy-Walker malformation: analysis of 19 cases. J Child Neurol 25:188–191

    Article  Google Scholar 

  • Alexiou GA, Varela M, Sfakianos G et al (2010b) Shunting for the treatment of arachnoid cysts in children. Neurosurgery 67:1632–1636

    Article  Google Scholar 

  • Asai A, Hoffman J, Hendrick B et al (1989) The Dandy-Walker syndrome: experience at the Hospital for Sick Children, Toronto. Pediatr Neurosci 5:66–73

    Article  Google Scholar 

  • Bragg TW, St George EJ, Wynne-Jones GA et al (2006) Familial Dandy-Walker syndrome: a case report supporting an autosomal inheritance. Childs Nerv Syst 22:539–541

    Article  Google Scholar 

  • Cakmak A, Zeyrek D, Cekin A et al (2008) Dandy-Walker syndrome together with occipital encephalocele. Minerva Pediatr 60:465–468

    CAS  PubMed  Google Scholar 

  • Cardoso J, Lange MC, Lorenzoni PJ et al (2007) Dandy-Walker syndrome in adult mimicking myasthenia gravis. Arq Neuropsiquiatr 65:173–175

    Article  Google Scholar 

  • Clarren SK, Alvord EC Jr, Sumi SM et al (1978) Brain malformations related to prenatal exposure to ethanol. J Pediatr 92:64–67

    Article  CAS  Google Scholar 

  • Dandy WE, Blackfan KD (1914) Internal hydrocephalus. an experimental, clinical and pathological study. Am J Dis Child 8:406–482

    Article  Google Scholar 

  • Golden JA, Rorke LB, Bruce DA (1987) Dandy-Walker syndrome and associated anomalies. Pediatr Neurosci 13:38–44

    Article  CAS  Google Scholar 

  • Harper T, Fordham LA, Wolfe HM (2007) The fetal Dandy-Walker complex: associated anomalies, perinatal outcome and postnatal imaging. Fetal Diagn Ther 22:277–281

    Article  Google Scholar 

  • Has R, Ermis H, Yuksel A et al (2004) Dandy-Walker malformation: a review of 78 cases diagnosed by prenatal sonography. Fetal Diagn Ther 19:342–347

    Article  Google Scholar 

  • Hirsch JF, Pierre-Kahn A, Renier D, Sainte-Rose C, Hoppe-Hirsch E (1984) The Dandy-Walker malformation. A review of 40 cases. J Neurosurg 61(3):515–522

    Article  CAS  Google Scholar 

  • Kalayci M, CaÄŸavi F, Bayar U et al (2006) Neurocutaneous melanosis associated with Dandy-Walker malformation. Acta Neurochir (Wien) 148:1103–1106

    Article  CAS  Google Scholar 

  • Kalidasan V, Carroll T, Allcutt D et al (1995) The Dandy-Walker syndrome. A 10 year experience of its management and outcome. Eur J Pediatr Surg 5:S16–S18

    Article  Google Scholar 

  • Klein O, Pierre-Kahn A, Boddaert N et al (2003) Dandy-Walker malformation: prenatal diagnosis and prognosis. Childs Nerv Syst 19:484–489

    Article  CAS  Google Scholar 

  • Kumar R, Jain MK, Chhabra DK (2001) Dandy-Walker syndrome: different modalities of treatment and outcome in 42 cases. Childs Nerv Syst 17:348–352

    Article  CAS  Google Scholar 

  • Marnet D, Vinchon M, Mostofi K et al (2009) Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association. Childs Nerv Syst 25:1533–1539

    Article  Google Scholar 

  • Martinez-Lage JF (1991) Dandy-Walker malformation, cranial meningocele, ocular abnormalities, and cleft palate: Walker-Warburg syndrome? Pediatr Radiol 21:319

    Article  CAS  Google Scholar 

  • Menon RK, Nadkarni TD, Desai KI et al (2006) Dandy-Walker malformation associated with polycystic kidneys: Goldston syndrome revisited. J Clin Neurosci 13:875–877

    Article  Google Scholar 

  • Metry D, Heyer G, Hess C et al (2009) Consensus statement on diagnostic criteria for PHACE syndrome. Pediatrics 124:1447–1456

    Article  Google Scholar 

  • Mohanty A, Biswas A, Satish S et al (2006) Treatment options for Dandy-Walker malformation. J Neurosurg 105:348–356

    PubMed  Google Scholar 

  • Mytilinaios DG, Tsamis KI, Njau SN et al (2010) Neuropathological findings in Dandy Walker variant. Dev Neurorehabil 13:64–67

    Article  Google Scholar 

  • Osenbach RK, Menezes AH (1992) Diagnosis and treatment of the Dandy-Walker malformation: 30 years of experience. Pediatr Neurosurg 18:179–189

    Article  CAS  Google Scholar 

  • Papazisis G, Mastrogianni A, Karastergiou A (2007) Early-onset schizophrenia and obsessive-compulsive disorder in a young man with Dandy-Walker variant. Schizophr Res 93:403–405

    Article  Google Scholar 

  • Prakash R, Singh LK, Kour J et al (2009) Psychiatric comorbidities in Dandy-Walker variant disorder. J Neuropsychiatr Clin Neurosci 21:477–479

    Article  Google Scholar 

  • Salihu HM, Kornosky JL, Druschel CM (2008) Dandy-Walker syndrome, associated anomalies and survival through infancy: a population-based study. Fetal Diagn Ther 24:155–160

    Article  Google Scholar 

  • Sawaya R, McLaurin RL (1981) Dandy-Walker syndrome: clinical analysis of 23 cases. J Neurosurg 55:89–98

    Article  CAS  Google Scholar 

  • Tohyama J, Kato M, Kawasaki S et al (2010) Dandy-Walker malformation associated with heterozygous ZIC1 and ZIC4 deletion: report of a new patient. Am J Med Genet A 155:130–133

    Article  Google Scholar 

  • Ugur HC, Torun F, Yilmaz E et al (2005) Trigeminal neuralgia in a patient with Dandy-Walker malformation. J Clin Neurosci 12:815–817

    Article  Google Scholar 

  • Venturini E, Magni L, Pucci G, Mazzinghi F (2017) A late presentation of Dandy-Walker malformation and aortic coarctation. J Cardiovasc Med (Hagerstown). 18(5):381–384

    Google Scholar 

  • Yapar EG, Ekici E, Dogan M et al (1996) Meckel-Gruber syndrome concomitant with Dandy-Walker malformation: prenatal sonographic diagnosis in two cases. Clin Dysmorphol 5:357–362

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, University Hospital of Ioannina, Ioannina, Greece

    George A. Alexiou & Neofytos Prodromou

Authors
  1. George A. Alexiou
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  2. Neofytos Prodromou
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Editor information

Editors and Affiliations

  1. Bruxelles, Belgium

    Mario Manto

  2. Scripps Research Institute Dept. Neuropharmacology, La Jolla, CA, USA

    Donna Gruol

  3. Dept. Neurology, Harvard Medical School Massachusetts General Hospital, Boston, MA, USA

    Jeremy Schmahmann

  4. Gunma University Dept. Integrative Physiology, Maebashi, Gunma, Japan

    Noriyuki Koibuchi

  5. Dept Pathology & Immunology & Neuro, Baylor College of Medicine Dept Pathology & Immunology & Neuro, Houston, TX, USA

    Roy Sillitoe

Section Editor information

  1. Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

  2. CHU-Charleroi, University of Mons, UMons, Charleroi, Belgium

    Mario U. Manto

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Alexiou, G.A., Prodromou, N. (2020). Dandy-Walker Malformations. In: Manto, M., Gruol, D., Schmahmann, J., Koibuchi, N., Sillitoe, R. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-97911-3_86-2

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  • DOI: https://doi.org/10.1007/978-3-319-97911-3_86-2

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-97911-3

  • Online ISBN: 978-3-319-97911-3

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

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