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Dandy Walker Syndrome/ Malformation

What is Dandy Walker Malformation? 

Dandy Walker malformation, also often referred to as Dandy Walker syndrome, is a congenital defect affecting the development of the cerebellum. The central part of the cerebellum (vermis) in children with Dandy Walker malformation may either be abnormally positioned, unusually small or completely absent. Conversely, children with fetal Dandy Walker malformation can also have an abnormally large fluid-filled cavity between the brainstem and cerebellum, as well as a larger part of the skull where the brainstem and cerebellum sits.

Babies with Dandy Walker malformation may have difficulty draining excess cerebral spinal fluid (CSF), which can potential lead to hydrocephalus. For more information on this condition, click here.

Dandy Walker Malformation

Causes: Is Dandy Walker Malformation Hereditary?

Many cases of fetal Dandy Walker malformation cannot be linked specifically to one particular cause. Parents are often concerned that the condition is hereditary, but evidence suggests Dandy Walker malformation results from a number of potential causes rather than a genetic identifier. 

Some of these include: 

  • Chromosomal brain developments during pregnancy
  • Exposure to certain medications or toxins during pregnancy
  • Maternal diabetes
  • Viral infections passed from the mother to the baby during pregnancy

How is Dandy Walker Syndrome Diagnosed? Symptoms and Next Steps

Fetal Dandy Walker malformation can be identified during pregnancy (usually in the second or third trimester) with an ultrasound. After birth, Dandy Walker malformation can be identified in a number of ways. Because the cerebellum is the part of the brain that controls movement, behavior and cognitive ability, your child’s physician can diagnose Dandy Walker malformation by recognizing symptoms that include: 

  • Bulging at the back of the skull
  • Delays in motor and language skills (sitting up, walking, talking, etc.)
  • Difficulty with balance and coordination
  • Irregular eye movements
  • Increased head circumference
  • Poor muscle tone
  • Seizures
  • Vision and hearing difficulty

While Dandy Walker malformation symptoms usually present before age one, it is important for both parents and physicians to keep an eye on developmental progress throughout early childhood. 

Dandy Walker Malformation Treatment and Prognosis

If needed, Dandy Walker syndrome treatment mostly involves treating the symptoms associated with each individual’s case. For cognitive issues, Dandy Walker malformation treatment approaches can include speech, physical and occupational therapy to assist in development during the early childhood years and into adolescence. If your child struggles with seizures, your physician may prescribe a number of medications to help alleviate those symptoms. 

Children whose Dandy Walker malformation also includes hydrocephalus may need to have a shunt placed in the brain to drain excess CSF fluid. 

Because each child’s case is different, the long-term prognosis for a child Dandy Walker malformation varies. These can range from little to no impact on cognition and development to developmental and impairment issues in more severe cases.