Dandy-Walker Syndrome

Dandy-Walker syndrome is a congenital brain malformation involving the fourth ventricle and cerebellum.

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.

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.

The syndrome can appear dramatically or be totally asymptomatic. Other symptoms include increased head circumference, bulging occiput (the back of the head), cranial nerve dysfunction, and abnormal breathing patterns.

Of importance is the high association of Dandy-Walker syndrome with other central nervous system structural anomalies including agenesis of the corpus callosum and mal-formations of the face, limbs, digits and heart.

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.

Information provided by the
National Institute of Neurological Disorders and Stroke,
National Institutes of Health

Each year, Medical College of Wisconsin physicians care for more than 180,000 patients, representing nearly 500,000 patient visits. Medical College physicians practice at Children's Hospital of Wisconsin, Froedtert Memorial Lutheran Hospital, the Milwaukee VA Medical Center, and many other hospitals and clinics in Milwaukee and southeastern Wisconsin.