Dandy walker Malformation / Variants

Dandy walker variants / syndrome is a congenital brain disorder where there is significant brain abnormalities seen. It is said the malformation is due to inflammation or trauma of the brain which blocks the smooth flow of circulation of CSF. 

Incidence

1 in 10,000 to 30,000 newborns
About 3% children with hydrocephalus have Dandy walker malformations

Signs and Symptoms

• Hydrocephaly is a common sign which ids associated with vomiting, convulsion and emotional irritability
• Mostly head size is bigger than normal peer
• Slow in motor skills development
• Movements are uncoordinated and ataxic
• Presence of Involuntary eye movements ( Nystagmus)
• Some children may be intellectually disabled

Types / Variety

Dandy walker variants may be mild, moderate or severe on the basis of malformation of brain and impact on the functional status

Causes

Dandy walker variants are a genetic disorder but the genetic profile is not fully established. 

Diagnosis Making

• Dandy walker variants can be diagnosed before birth with ultrasound due to the presence of hydrocephaly. At birth it is easily diagnosed due to high head circumference and other associated problems. 
• Skull X ray and CT scan can detect the abnormalities in the brain

Treatment / Management

Dandy walker variants are treated and managed with both medical management and therapies.

Medical and Surgical Treatment

The primary problem hydrocephalus is treated with medications and surgical intervention by putting a shunt. Medications for controlling seizure disorder and other associated problems. Hormonal therapy is also suggested

Therapy

All children with Dandy walker variants require physiotherapy and occupational therapy due to developmental delay and movement disorders. On the basis of cognition, special education is also provided. 

Prognosis

Mild to moderately affected individuals live better and partially independent life. A few individuals have very good IQ and very good in communication.

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