We have changed our visitation policy for the safety of our patients and staff. Click here for the updated visitation policy and click here for information about COVID-19.

Lahey Health is now part of Beth Israel Lahey Health

(Dandy-Walker Malformation; Dandy Walker Syndrome; Familial Dandy Walker; Dandy Walker Malformation)

Definition

Dandy-Walker syndrome is a rare brain deformity that happens before birth. It affects an area in the back of the brain that controls movement, thinking, and behavior. It also causes a buildup of fluid in ventricles (chambers) around it.

Cerebellum
si2027 97870 1 cerebellum
Copyright © Nucleus Medical Media, Inc.

Causes

The exact cause is not known. Genes may play a role.

Risk Factors

This problem may be more common in people who have a parent with it.

Symptoms

Problems may be:

  • Increased head size
  • Muscles that are very floppy or stiff
  • Delayed development doing things like sitting up and walking
  • Irritability
  • Vomiting
  • Jerky eye movements
  • Problems with coordination
  • Bulging of the back of the skull
  • Breathing problems
  • Seizures
  • Problems controlling facial movements
  • Learning problems

Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.

Diagnosis

The doctor will ask about your child's symptoms and health history. A physical exam will be done.

Images may be taken of the brain. This can be done with:

Treatment

There is no cure. The goal is to manage symptoms. This can be done with physical therapy and education services.

The buildup of fluid in the ventricles may need to be treated. Extra fluid can cause pressure and swelling in the brain. Fluid may be drained by:

  • Shunt—A tube is placed from the brain to the abdomen to allow the extra fluid to drain out of the brain.
  • Ventriculostomy—A connection is made from one ventricle to other areas that contain fluid. It allows extra fluid to drain out of the affected ventricle.

Prevention

There is no known way to prevent this syndrome.

RESOURCES

Genetics Home Reference  https://ghr.nlm.nih.gov 

National Institute of Neurological Disorders and Stroke  http://www.ninds.nih.gov 

CANADIAN RESOURCES

Canadian Neurological Sciences Federation  http://www.cnsfederation.org 

Health Canada  https://www.canada.ca 

References

Dandy-Walker malformation. Genetics Home Reference—National Institutes of Health website. Available at: https://ghr.nlm.nih.gov/condition/dandy-walker-malformation. Updated October 2015. Accessed April 14, 2020.

Dandy-Walker malformation. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/dandy-walker-malformation/. Published 2008. Accessed April 14, 2020.

Dandy Walker syndrome. BrainFacts.org—Society for Neuroscience website. Available at: https://www.brainfacts.org/diseases-and-disorders/neurological-disorders-az/diseases-a-to-z-from-ninds/dandy-walker-syndrome. Accessed April 14, 2020.

Dandy-Walker syndrome information page. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/Disorders/All-Disorders/Dandy-Walker-Syndrome-Information-Page. Updated March 27, 2019. Accessed April 14, 2020.

Hydrocephalus in children. EBSCO DynaMed website. Available at:  https://www.dynamed.com/condition/hydrocephalus-in-children . Updated December 4, 2018. Accessed April 14, 2020.

Kahle KT, Kulkarni AV, et al. Hydrocephalus in children. Lancet. 2016 Feb 20;387(10020):788-799.

Revision Information

  • Reviewer: EBSCO Medical Review Board Rimas Lukas, MD
  • Review Date: 02/2020
  • Update Date: 04/14/2020