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Genetic and Rare Diseases Information Center (GARD)

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Dandy-Walker complex


Other Names for this Disease
  • Dandy-Walker syndrome or malformation (type of DW complex)
  • Dandy-Walker variant (type of DW complex)
  • DW complex
  • Mega cisterna magna (type of DW complex)
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Overview


Dandy-Walker complex is a congenital brain malformation involving the cerebellum (an area at the back of the brain that controls movement) and the fluid-filled spaces around it. This condition is characterized by an enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord), a partial or complete absence of the cerebellar vermis (the area between the two cerebellar hemispheres), and cyst formation near the internal base of the skull.[1][2] Dandy-Walker complex represents a continuum of developmental abnormalities which includes Dandy-Walker variant, Dandy-Walker malformation, and mega cisterna magna.[2] This condition may present with an increase in fluid and pressure around the skull (hydrocephalus). It may also be associated with developmental delay, and malformations of the heart, face, limbs, fingers and toes.[1][3] 
Last updated: 1/7/2013

References

  1. NINDS Dandy-Walker Syndrome Information Page. National Institute of Neurological Disorders and Stroke (NINDS). December 16, 2011; http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm. Accessed 1/7/2013.
  2. Incesu L, Khosla A. Imaging in Dandy-Walker Malformation . Medscape Reference. May 2011; http://emedicine.medscape.com/article/408059-overview. Accessed 1/7/2013.
  3. 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; http://www.ncbi.nlm.nih.gov/pubmed?term=18759601. Accessed 1/7/2013.
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Basic Information

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Dandy-Walker complex. Click on the link to view a sample search on this topic.