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Dandy Walker Syndrome - Symptoms and Treatment


Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. It is defined as an enlargement of the fourth ventricle, an absence (partial or complete) of the cerebellar vermis (the narrow middle area between the two cerebral hemispheres), and cyst formation in the posterior fossa (the internal base of the skull). Hydrocephalus (increased intracranial pressure) may also be present. Children with Dandy-Walker syndrome may never have normal intellectual development. The length of their life span depends on the severity of the syndrome and associated malformations. The presence of multiple congenital defects may shorten life span. Dandy-Walker syndrome is frequently associated with disorders of other areas of the central nervous system including absence of the connecting area between the two cerebral hemispheres, and malformations of the heart , face, limbs, fingers and toes. Treatment for individuals with Dandy-Walker syndrome generally consists of treating the associated anomalies, if needed. Also, a ventriculoperitoneal shunt may be inserted to control the hydrocephalus. Genetic counselling may also be needed. The prognosis for patients with Dandy-Walker syndrome is only moderately favorable, even when the hydrocephalus is treated early and correctly. The presence of multiple congenital defects may adversely affect survival. Prognosis for normal intellectual development is variable depending on the severity of the syndrome and associated malformations.

Symptoms of Dandy Walker Syndrome

Some common Symptoms of Dandy Walker Syndrome :

  • Abnormal breathing patterns.
  • Increased head circumference.
  • Problems with the nerves that control the eyes, face and neck.
  • A head bulge at the back of the skull.

 

 


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