What is it?
What are common effects?
Dandy-Walker (DW) Syndrome is a rare congenital birth defect of the brain. DW involves the cerebellum, the back
part of the brain, and the fluid filled spaces that surround it. The cerebellum controls movement, behavior, and
cognition. Many people experience difficulties with balance, coordination, voluntary muscle movements, mood and
intellectual disability. DW is often associated with other disorders of the central nervous system including absence
of the corpus callosum and malformation of the heart, face, limbs, fingers, and toes. DW can disrupt the normal
flow of cerebrospinal fluid (CSF) leading to a condition called hydrocephalus. A DW diagnosis may be more
common in females.
Although a small percentage of cases occur in a family, most diagnoses occur sporadically with no familial pattern
of inheritance. In most cases, the cause is a combination of genetic and environmental factors that could include
viral infection or intrauterine exposure to certain toxins or medications. A diabetic mother is also more likely to have
a child with DW.
Prognosis largely depends on severity of DW and its associated congenital defects. Malformations causing key
features associated with DW include:
Some symptoms develop slowly and seemingly unnoticeable over time while others appear dramatically.
Symptoms often beginning in early infancy include slow motor development and progressive skull enlargement.
Children could demonstrate delays in sitting, walking, and talking while intellectual development varies. Diagnosis is
normally made around 3-4 years of age. Other advancing symptoms include:
DANDY-WALKER SYNDROME
The Specialized Health
Needs Interagency
Collaboration (SHNIC)
program is a collaborative
partnership between the
Kennedy Krieger Institute
and the Maryland State
Department of Education.
Enlargement of the 4th ventricle, the area that allows fluid to flow between the brain and spinal cord
Absence or partial development of the cerebellar vermis, the area between the 2 hemispheres
Development of a cyst at the base of the skull
Increase in size of fluid filled spaces of brain
Malformation of heart, face, limbs, fingers and toes
Affected nerves of eyes, face, neck
Jerky, uncoordinated movements, stiffness
Vomiting, irritability
Seizures
Increased head circumference, increased intracranial pressure
Bulging at the back of the skull
Abnormal breathing related to cerebellar dysfunction
There is no cure for DW and treatment focuses on relieving pressure on the brain and
managing the associated symptoms. A ventriculoperitoneal shunt will be surgically
placed to drain excess fluid and reduce swelling and intracranial pressure. Medications
are often prescribed to control seizures.
What is the treatment?
