Dandy Walker Syndrome

What is Dandy Walker Syndrome?

The Dandy Walker syndrome or also known as Dandy Walker complex is a medical condition that involves congenital malformation or deformity of the brain. The part of the brain which is affected is the cerebellum and its key fluid protector. There is also a possibiltity that there is partition of the brain absent, even worst is the complete absence of a part. The name Dandy Walker syndrome is made possible by Walter Dandy and Arthur Walker.


This medical condition is involved in the enlargement of one of the ventricles of the brain, called the fourth ventricle. This is also characterized by the absence of the part of the brain that is responsible for a person’s coordination. It has been reported that the condition generally affects 1 out of 25, 000 to 35,000 live births. Women are more affected than men. As also expected, recurrence may happen as for it is considered as a genetically acquired condition.

The condition is classified according to the following:

Dandy walker syndrome malformation

This is believed the most severe form or presentation of the condition. Its complication includes hydrocephalus and spina bifida, affectation of the spine.

Dandy walker mega cisterna

This is characterized by the presence of large quantities of cerebrospinal fluid in the cisterna magna.

Dandy Walker variant

This is a less severe form of malformation. When Dandy Walker syndrome is confused from what classification it belongs to, DWS variant is then the provided as the final diagnosis.

Symptoms

Those who are unfortunate to acquire Dandy Walker syndrome is believed to be characterized by the following symptoms:

  • Depleted motor development in infants
  • Hydrocephalus
  • Increased irritability
  • Nausea and vomiting
  • Convulsions
  • Lack of muscle coordination
  • Possible jerky movements of the eyes
  • Deformities may be present on the patient’s face, limbs, extremities, and heart.

Causes

The direct cause of Dandy Walker syndrome is not known or idiopathic in sense. This has been the major report among cases of Dandy Walker syndrome. But according to studies, the condition is considered as autosomal recessive. Environmental factors have also been noted to be part as a predisposing factor for Dandy Walker syndrome. The proposed environmental factors are the conditions that a pregnant woman acquires during her first trimester are rubella, acquisition of cytomegalovirus, and taking or warfarin.

Diagnosis

Dandy Walker Syndrome can be diagnosed through the following examinations:

  1. MRI – This has been noted as one of the best diagnostic exam for neurological or CNS related conditions.
  2. CT scan – This procedure assists in the diagnosis of Dandy Walker syndrome. There are 3D studies that are good in the evaluation process of DWS.
  3. Angiography –This can demonstrate or provide the features of malformations of Dandy Walker syndrome.
  4. Ultrasonography – The malformation can be identified through the antenatal ultrasound.

Treatment

Treatment for this condition involves surgical intervention such as placing a shunt in the patient’s skull in order to reduce the increasing pressure in the brain, intracranial pressure. Ventriculoperitoneal (treats ventricles) or cystoperitoneal (treats cysts) shunts are the said procedures. This will relieve the excessive CSF in the patient’s skull by draining them out.

The characteristics of the syndrome are treated. Once a patient is having a seizure, management for these episodes should be known. The care for patients who are seizure precautionary are the following:

  • Do not expose the child to bright lights. This can precipitate for a seizure episode.
  • When a seizure is in progress, place the child in a low position in order to avoid drastic results from a fall.
  • Do not attempt to place anything in the mouth of the patient such as tongue depressor once the seizure has taken place, it should be place prior to a seizure episode.
  • During an episode, place the patient in a sidelying position to avoid aspiration.
  • Do not restrain the patient while on seizure.

There are also various therapies provided for clients who are in their recovery phase. These therapies are the following:

Occupational and physical therapy – These therapies will assist the affected as the manifestations of the disease process are highly affecting the coordination and full attainment of movement of the child.

Special education – Children who survived the years of Dandy Walker syndrome requires special education as this condition results to mental affectation.

Speech therapy – As part of the special education, speech therapy is also required so to attain at least part of the goal for assisting patients with Dandy Walker syndrome.

Genetic counseling – This is an essential part of educating the needy. As the condition places a risk to the parents of a child with Dandy Walker syndrome, they need to be aware of this and be counseled.

Life expectancy

Those affected of Dandy Walker syndrome are most likely to have a dependent life expectancy. As research shows, the longevity of their lives depends on the classification of Dandy Walker syndrome. When the condition is at its severe state, then it’s expected to have a poor prognosis and a shorter life expectancy range. As the manifestations of the disease is much grave, then a person shall have a shorter life span. Seizures are the most likely outcome of the condition that can result for the increasing difficulties of the patient. The condition can even result to lifelong complications that lead to an increased mortality of the affected.

Dandy Walker Syndrome Pictures

Photos, Images and Pictures of Dandy Walker Syndrome…

Dandy Walker Syndrome Picture (1)

Source: medindia.net

Dandy Walker Syndrome Picture (2)

Source: medindia.net

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