Dandy Walker Syndrome

Dandy Walker Syndrome is a rare brain disorder that leads to many intracranial problems as well as multiple physical and mental defects. Read and know all about this disease as well as its causes, symptoms, diagnosis and treatment.

What Is Dandy Walker Syndrome?

This is a congenital malformation of the brain that involves the cerebellum and the fourth ventricle. It is a type of Hydrocephalus which is an abnormal condition characterized by accumulation of the cerebrospinal fluid in the ventricles located in the brain.

The disorder is also known as Dandy-Walker Complex.

Dandy Walker Syndrome Incidence

This is a very rare condition that affects one in every 25,000 or 35,000 newborns. It is found to be present in 4-12% cases of Infantile Hydrocephalus.

Dandy Walker Syndrome Symptoms

This condition is marked by discomforting symptoms. Some of the major symptoms of Dandy Walker Syndrome (DWS) include :

• Difficulties in balance
• Increasing intracranial pressure
• Vomiting
• Convulsions
• Mental retardation
• Double vision (Diplopia)

These problems arise as a consequence of pressure build-up within the cranium. The symptoms may be non-existent in infants due to the fact that their head has the ability to grow. The symptoms are generally apparent in older children. The symptoms of Dandy Walker Syndrome usually depend to an extent on the combination of difficulties in maturation in the infant. In some cases, the only symptom may be increase in size of head due to Hydrocephalus. Hydrocephalus is seen to arise in 90 percent of sufferers of this condition.

This brain abnormality is also characterized by a partial or total absence of the Cerebellar Vermis as well as a cyst formation at the base of the skull. Cerebellar Vermis is the space between two hemispheres of the brain. The increase in amount of fluids flowing through the brain puts more pressure on both the skull and the brain.

The condition also alters the physical appearance of many sufferers. The normal positioning of the eyes and nose is commonly affected and sufferers appear to have an upward displacement of sinuses and a bulging skull.

Dandy Walker Syndrome Causes

This deformity of brain happens when apertures that let cerebrospinal fluid enter the space around the brain surface fail to open. This condition may give rise to multiple abnormalities in the central nervous system and the brain.

Medical researchers are still in the dark about the exact causes of Dandy Walker Syndrome. The disorder is supposed to be a combination of environmental and genetic factors. This disease is seen to run in several generations of the same family.

Dandy Walker Syndrome Diagnosis

The brain malformation resulting from this disorder is generally diagnosed with the aid of medical tests like Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and Antenatal Ultrasound. An MRI Scan allows doctors to get a detailed analysis of the lesions formed by brain malformation. This might be followed by Antenatal MRI assessment that can help in confirming the diagnosis and get more detailed information.

This disorder is generally diagnosed in children. Many kids are born with healthy ventricles with normal formation, but suffer from discomforting symptoms of the syndrome only when they are about a year in age. Most of these cases are diagnosed in children aged between three to four years. However, it may be found in kids as old as twelve years as and young as nine months.

Dandy Walker Syndrome Treatment

Treatment for Dandy-Walker Syndrome sufferers usually involves curing the problems related with this condition. Treatment is done only if necessary. In some cases, a special tube may be inserted into the skull in order to drain off extra fluid. This will reduce pressure inside the cranium (part of the skull enclosing the brain) and help manage inflammation. This will also provide relief to sufferers from some of the other problems that may arise from intracranial pressure and inflammation.

Parents of Dandy-Walker Syndrome sufferers may benefit from Genetic Counseling if they wish to have more kids. A number of organizations are available for Counseling and other types of aids for parents of DSW sufferers. Counseling helps parents who find it difficult to understand the problems faced by kids diagnosed with this disorder.

The treatment depends on early diagnosis to a large extent. Some kids may be able to function normally if DSW is diagnosed and cured at a young age. However, this may vary from one suffer to another.

Some kids may experience long-term complications and problems from this disease. Sufferers with more than one defect from this condition may have a reduced life span.

Dandy Walker Syndrome Prognosis

The scope of prognosis is diverse for sufferers of this disorder. Usually, mortality rate associated with this disease is found to very high. This is due to the fact that mortality statistics of DAS are frequently compiled by neurologists who handle worst cases of this disorder that lead to death. This explains bad prognosis or high mortality rate in both prenatal and postnatal DWS infants.

Broadly considered, the prognosis is good in this condition with chances of survival being somewhere around 75 percent. Approximately half of all sufferers of this disease are found to have normal IQ. However, most affected individuals suffer from discomforting symptoms like retarded motor control, stiffness and difficulties in balance. Around 15 percent of sufferers experience seizures due to this disorder.

This condition leads to variable effects on the intellectual development of Dandy Walker Syndrome sufferers. While some kids have normal cognitive powers, others fail to attain healthy intellectual development even after early treatment and correction of the excess fluid accumulation. Children suffering from less serious symptoms of this syndrome may have healthy intellectual development while those with severe malformation may sufferer from mental retardation.

Longevity of DWS sufferers is dependent on the severity of the disease and malformations associated with it. The life span of sufferers may be reduced due to the presence of a number of congenital defects.

Dandy Walker Syndrome Complications

This bran disorder is often found to be associated with other conditions like Hydrocephalus, Central Nervous System disorders, absence of Corpus Callosum as well as malformations of the face, heart, fingers, toes and limbs.

Dandy Walker Syndrome Management

Management of this condition involves managing the discomforting symptoms, such as seizures, associated with the disorder.

Dandy Walker Syndrome Pictures

Here are some Dandy Walker Syndrome photos that you will find quite useful. Take a peek at these Dandy Walker Syndrome images to know how sufferers of this disease look like.

Photo 1 – Dandy Walker Syndrome
Source – drarunlnaik

Picture 2 – Dandy Walker Syndrome
Image Source – dandykidsdocumentary

If you encounter problems similar to DSW in any child in your family, immediately get in touch with a doctor. As aforesaid, early diagnosis can help cure this condition earlier and make the affected child behave normally even in future.