Thursday, June 18, 2009

Case history of Dandy-Walker Syndrome Child~ Jaisal Bakshi

Jaisal Bakshi is ten year old Dandy-Walker Syndrome child. He was admitted in Swami Brahmanand Pratishthan, Centre for Mentally Challenged two years ago. During that time, his condition was quite severe. But, he has very loving family, who work very hard on his progress and they co-operate with the teachers at school in helping him cope with his problems. He has shown marked improvement during these two years, he has better coordination of muscle movements, is able to recognize and respond to insructions and loves Bollywood music.

Dandy-Walker syndrome (DWS) is a congenital (present at birth) brain malformation typically involving the fourth ventricle and the cerebellum. The disorder was first described in 1914 by W. Dandy and K. Blackfan and was designated as Dandy-Walker syndrome in 1954 by C. Benda, who also reported familial occurrence. DWS occurs in one out of every 25,000 live births.

The following characteristics have been seen in children with Dandy-Walker syndrome:

• Enlargement of the fourth ventricle
• Absence (partial or complete) of the cerebellar vermis (area between the two cerebral hemispheres)
• Cyst formation in the posterior fossa (internal base of the skull)
• Slow motor development in early infancy
• Progressive macrocrania (abnormally enlarged skull)
• Hydrocephalus
• Seizures
• Intracranial pressure in older children, causing irritability, vomiting, and convulsions
• Cerebellar dysfunction causing ataxia and nystagmus
• Bulging occiput (back of head)
• Cranial nerve dysfunction
• Abnormal breathing patterns
• Agenesis of the corpus callosum
• Malformations of the face, limbs, digits, and heart
• Cleft lip and palate
• Urinary structural abnormalities

The majority of individuals with DWS are diagnosed their first year of life as a result of the commonly associated hydrocephalus. However, the definitive diagnosis can be made by ultrasound, CT-scan, or MRI.

Treatment consists of treating the associated symptoms (i.e., anticonvulsants for those with seizures and the insertion of a shunt for those with hydrocephalus). Balance problems and spasticity may occur and warrant physical therapy. Occupational therapy may be helpful for those with poor fine motor control.

The syndrome can appear dramatically, or be totally asymptomatic. Therefore, the prognosis for normal intellectual development varies depending on the severity of the syndrome and associated malformations. Difficulties in learning occur in 35-70% of children with DWS. Statistics in the literature citing a high rate of mental retardation most likely reflect injury due to inadequate management of the associated hydrocephalus, something which is less likely to occur today than in the past. Many children with DWS can be mainstreamed at school. Pediatricians, pediatric neurologists, pediatric neurosurgeons, geneticists, physical therapists, and educational specialists may be required to systematically follow these children and work towards ensuring that the child is given the best opportunities to reach his or her full potential.


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