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Information sheet about Dandy-Walker Complex

Other Names

Dandy-Walker complex; Dandy-Walker cyst; Dandy-Walker deformity; Dandy-Walker syndrome; DWM; DWS; hydrocephalus, internal, Dandy-Walker type; hydrocephalus, noncommunicating, Dandy-Walker type; Luschka-Magendie foramina atresia; Dandy-Walker variant; Mega cisterna magna

Categories

Congenital and Genetic Diseases.

Signs and Symptoms

Dandy-Walker Complex (DW complex) is a group of disorders that affects brain development. The part of the brain affected is called the cerebellum, which coordinates movement. The changes in brain development are generally present from birth or within the first year of life.

Sign and symptoms of DW complex overlap between the group of disorders and may include:

  • Dandy-Walker malformation or syndrome – where the central part of the cerebellum (the vermis) is small and may be unusually positioned.
  • Fluid-filled cavity between the brainstem and the cerebellum and the part of the skull that contains the cerebellum and the brainstem are large.
  • Problems with movement, coordination, intellect, mood, and other brain functions.
  • Children may have delay in motor skills such as crawling, walking, and coordinating movements.
  • Experience muscle stiffness and partial paralysis of the lower limbs and seizures.
  • Dandy-Walker variant – where there is a small cerebellar vermis but without other features of DW complex.
  • People with Mega cisterna magna have an enlarged part of the base of the skull with a normally developed cerebellum and it may not cause any health problems.
  • In some cases, some people may have problems with other systems and can include heart defects, malformations of the urogenital tract, extra fingers or toes, or fused fingers or toes, or abnormal facial features.

Cause/Inheritance

  • In most cases DW complex cause is unknown.
  • Mostly thought to be a combination of genetic and environment factors that affect development before birth.
  • In some cases, exposures that occur during pregnancy such as having diabetes or infections such as rubella may increase the risk of a baby developing DW complex.
  • In some cases, there may be an underlying genetic change such as extra or missing pieces of chromosome in each cell of the body.
  • DW complex may also be a symptom of another genetic syndrome.
  • Most cases of DW complex are sporadic, meaning they occur in people with no history of the condition in their family.
  • A small percentage of cases seem to run in families however, it is not understood as to how the condition is inherited.

Treatment and Management

Treatment of DW complex is based on the symptoms present for each person. Treatments may include:

  • Focus on relieving any pressure on the brain that is caused by the brain malformations.
  • Surgery to drain excess fluid in the brain.
  • Medications used to help control seizures.
  • Supportive multi-disciplinary approach to provide special education, occupational therapy or physical therapy and other services to support symptoms such as developmental delay, treatment of coordinating movement, physical and intellectual disabilities.

Support Contacts Australia

Dandy-Walker Australia https://dandywalkeraustralia.wordpress.com/about/

Support Contacts International

Dandy-Walker Alliance http://dandy-walker.org/

Facebook Support

Dandy-Walker Alliance https://www.facebook.com/dandywalker/

Dandy-Walker Australia https://www.facebook.com/Dandy-Walker-Australia-285461195232/

Sources

Genetics Home Reference https://ghr.nlm.nih.gov/condition/dandy-walker-malformation#inheritance

National Organisation for Rare Disorders https://rarediseases.org/rare-diseases/dandy-walker-malformation/

Genetic and Rare Diseases Information Center https://rarediseases.info.nih.gov/diseases/6242/dandy-walker-complex

Orphanet https://www.orpha.net/consor/cgi-bin/Disease_Search_Simple.php?lng=EN

 

Disclaimer: This information is for general use only and is not meant to be a substitute for seeking professional care in the diagnosis, treatment and management of this condition.

Last update July 2018