Dandy-Walker Syndrome

Fact Sheet

By Shelly Sumner

Description

Dandy-Walker syndrome is a congenital disorder involving brain malformation and is a common cause of hydrocephalus (increased fluid in the brain. The malformation involves the cerebellum an area in the back of the bran that controls movement the fluid filled spaces around it and an enlargement of the fourth ventricle (a small channel that allows fluid to flow between the upper and lower areas of the brain and spinal cord). It also involves a partial or complete absence of the cerebellar vermis (area between the two cerebellar hemispheres which is important for coordination and voluntary muscel movements, and cyst formation near the base of the skull. The syndrome has a wide range of severety. Some people may not show any symptoms while others result in severe disabilities or even death.

Visual System Affected

Common vision problems include:

  • nystagmus (involuntary back and forth movement of the eyes).
  • Strabismus: misalignment of the eye, surgery may improve eye alignment.
  • Cataracts
  • Retinal dysgenesis: abnormal formation of the retina
  • Chorid Coloboma: abnormal formation of the eye between the sclera (white part of the eye) and the retina.
  • Cortical Visual Impairment

Effects on the condition of the visual system

Vision problems can vary from mild visual deterioration to severe vision loss. Vision problems may also affect perceptual abilities such as eye hand coordination. Children with hydrocephalus (increased fluid pressure in the brain) may also have trouble with visual motor skills such as trouble judging distance and direction or seeing and organizing a sequence of movements or have trouble separating relevant information from a background which can affect their ability to interpret meaning from pictures.

Common Treatments

The primary treatment for Dandy-Walker syndrome is associated with hydrocephalus is placing a shunt in the brain to drain the excess fluid which can be reabsorbed by the body. Other treatments include the use of anti-seizure medication, OT/PT and speech therapy if needed, and surgery to correct the eye alignment.

Congenital or adventitious and prossressive or stable

Dandy-Walker syndrome is congenital (present at birth). The occurance is isolated and sporadic and have little risk of recurrence. It may be inherited as an autosomal recessive trait which would imply a 25% chance of recurrence.

Anticipated Functional Implications of the condition:

The effects of the syndrome vary. Some children will have normal cognition while others may have severe disabilities and a shortened life span.

References

Dandy WalkerAlliance. (2010). DW basics. Retrieved from

Elquist, M. & Demchak, M.A. Fact Sheet-Dandy Walker Syndrome. Colorado

Services to Children and Youth with Combined Vision and Hearing Loss Project. Retreived from

Klein, O., et al. (2001). Dandy-Walker Malformation: Prenatial Diagnosis and

Prognosis. Childs Nervous System. 19 (August 2001), 484-9.

National Institute of Neurological Disorders and Stroke, (May 2010). NINDS

Dandy-Walker Syndrome Information Page, Retrieved from

Polzin, S. J. (2005). Dandy-Walker Syndrome Health Article. Gale Encyclopedia

of Neurological Disorders. Retrieved from

Yilmaz, A. M. DR., Real Brain Section. Dandy-Walker Homepage Personal Web

Page on DWS. Retrieved July 2010 from