All That You Need To Know About Dandy-Walker Syndrome

Published on: 31 March 2018 Last Updated on: 05 November 2024

Dandy-Walker Syndrome (DWS), also known as Dandy-Walker Malformation (DWM), is a rare disorder of the human brain. This disorder or malformation impacts the development of the cerebellum, a part of the brain responsible for muscle coordination, balance, posture, and speech.

Due to this disorder, the normal flow of cerebrospinal fluid (CSF) can be affected. This can result in the accumulation of too much CSF in the brain, causing a condition called hydrocephalus. The impact of the Dandy-Walker Syndrome typically lasts lifelong for a patient.

Who can get affected by Dandy-Walker Syndrome?

This disorder is a congenital condition that exists at birth. Infants belonging to both sexes can be affected by Dandy-Walker Syndrome. It is a genetic disorder that can occur sporadically. This syndrome impacts one in every 30,000 infants born alive.

What are the causes of this syndrome?

Dandy-Walker Syndrome is caused due to the partial or total failure of development of the cerebellar vermis that leads to the blockage of the CSF circulation. It is thought that various genetic and environmental factors can be responsible for someone to develop this rare congenital condition.

What are the symptoms of DWS?

The symptoms generally appear within one year after birth. Among the common symptoms of the syndrome are the following:

Delay in development of language and motor skills such as talking, crawling, walking, and sitting up
Poor muscle tone and muscle stiffness
Difficulty in balance and coordination
Unsteady eye movement, including jerky eye movement
Hearing and vision impairment
The occurrence of seizures or convulsions
Breathing difficulties or irregular patterns of breathing
Widened and flat veins visible in the skull
The increasing size of the skull leading to the abnormal growth of the head

How and when is Dandy-Walker Syndrome diagnosed?

The diagnosis of Dandy-Walker Syndrome can be done even before an infant is born. Tests to detect the syndrome before and after birth include the following:

High-resolution ultrasound screening is carried out during the second or third trimester of pregnancy to diagnose the syndrome
Fetal Magnetic Resonance Imaging (MRI) is done to confirm the diagnosis of the condition and to rule out other conditions that may appear to be similar to Dandy-Walker Syndrome in ultrasound screening
MRI after birth is done to confirm the syndrome and determine if any other complications are impacting the baby
Further imaging studies are done after birth, such as X-rays of the skull to determine its enlargement, MRI, and CT scan to check the shape and measure the size of the fourth ventricle, and ultrasound screening to determine hydrocephalus development.

In most cases, this syndrome is diagnosed in children in the early stages of their life. Some babies are born with normal ventricles but the signs of the syndrome start to appear when they reach about one year of age. In many cases, the syndrome is diagnosed in children when they are between three to four years of age. However, this syndrome has been known to be diagnosed in children as old as twelve years as well.

How can the condition be treated?

Treatment for Dandy-Walker Syndrome is aimed towards resolving the underlying symptoms and managing the complications that may arise due to the condition. Among the treatment options are:

Medications provided to check seizures
Physical therapy to help develop muscle tone and coordination
Speech therapy to improve language and speech
Occupational therapy to improve mobility skills such as walking, eating, managing simple daily chores, etc.
A surgical procedure called shunt may be carried out if severe hydrocephalus is observed in the patient. This procedure is done to reduce the intracranial pressure inside the skull by draining off the excess fluid gathered within the brain. It can bring relief to the patient from inflammation and some other problems that may occur due to intracranial pressure.

Since this is a rare condition, treatment needs to be done by knowledgeable doctors who are experienced in dealing with patients suffering from this syndrome. If you are looking for an expert doctor to consult about this syndrome, you can get expert advice and professional help at https://www.xpertdox.com/.

How can Dandy-Walker Syndrome be prevented?

This syndrome is a genetic condition. As such, there is no specific method that can be applied to ensure prevention from it. Expecting parents can undergo genetic testing. Prenatal diagnosis can also be helpful to understand the risks associated with the condition. In case of a family history of the syndrome, genetic counseling can be helpful to evaluate the risks before planning to have a baby.

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All That You Need To Know About Dandy-Walker Syndrome