Dandy Walker syndrome
Overview
Dandy-Walker syndrome (DWS), or Dandy-Walker complex, is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. The Dandy-Walker complex is a genetically sporadic disorder that occurs one in every 25,000 live births, mostly in females
Symptoms
Symptoms which often occur in early infancy include slow motor development and progressive macrocrania (an abnormally enlarged skull). In older children symptoms of increased intracranial pressure such as irritability, vomiting and convulsions, and/or signs of cerebellar dysfunction such as ataxia and nystagmus (jerky eyes) may occur.
Prognosis
The spectrum of outcomes for Dandy-Walker syndrome are diverse. Mortality statistics are often compiled by neurologists who deal with worst case outcomes, which thus reflect a high mortality rate, or grim prognosis – both pre and post natal – in DWS infants. Children with Dandy-Walker syndrome may never have normal intellectual development, even when the hydrocephalus is treated early and correctly. Longevity depends on the severity of the syndrome and associated malformations. The presence of multiple congenital defects may shorten life span.
Treatment
Treatment for individuals with Dandy-Walker syndrome generally consists of treating the associated problems, if needed. A special tube (shunt) to reduce intracranial pressure may be placed inside the skull to control swelling. Treatment may also consist of various therapies such as occupational therapy, physical therapy, speech therapy or specialized education. Services of a vision teacher may be helpful if the eyes are affected. Parents of children with Dandy-Walker syndrome may benefit from genetic counseling if they intend to have more children