Disorder - Dandy-Walker syndrome

Organ Systems Involved
Nervous/sensory

Alternative Names
Hydrocephalus with retrocerebellar cyst and aplasia of cerebellar vermis; Dandy-Walker malformation

Presenting Signs
    Dandy-Walker syndrome is caused by a malformation of the brain. The parts involved are the cerebellum (responsible for movement and balance) and the fluid filled spaces surrounding it. The condition results from a build-up of fluid on the brain (hydrocephalus), which causes increased pressure inside the skull. The hydrocephalus also causes abnormal development of the cerebellum and cysts in the fourth ventricle (a space between the medulla and the cerebellum). This can result in the brain tissue becoming compressed, causing serious nervous system problems. Symptoms may develop gradually or suddenly. The first signs may include sudden collapse, bumping into objects, severe lethargy and not eating. Other signs of nervous system dysfunction may be present, including flicking of the eyeballs, failure to blink, lack of co-ordination, head tremors and seizures. The heads of affected animals may appear dome shaped.

Treatment
    Surgical and Medical: An operation called a ventriculoperitoneal shunt is performed to remove the fluid and relieve the pressure on the brain. A small hole is drilled in the skull and a catheter is passed into the brain to drain the fluid. After the operation an anti-inflammatory (prednisolone) and a drug to prevent fluid buildup (frusemide) is used.

Contributor
Megan Prendergast