Dandy Walker syndrome is a rare condition that is caused when the brain fluid (CSF) flow is disturbed, leading to enlargement of the head size.
A 3-day-old baby who was diagnosed with Dandy walker syndrome (abnormally large brain) was successfully treated at a private hospital in Mumbai. A team of doctors comprising Dr. Ashwin Borkar, Dr Vinod Rambal, Dr. Rajashri Tayshete Bhasale and Dr. Nitu Mundhra performed a complex and critical surgery on the baby who was born with a rare condition. The newborn is doing well now, the doctors said.
The baby boy was diagnosed with hydrocephalus in intrauterine life, a rare condition which causes fluid-build up in the brain increasing the size of the head. The mother came to know about this condition in one of the ultrasound examinations at 26 weeks. Following which she visited Wockhardt Hospitals, Mira Road. Further evaluation showed Arachnoid Cyst (Accumulation of CSF) in posterior fossa which led to hydrocephalus.
Dandy Walker Syndrome: What you need to know
Prevalence of Arachnoid cyst in the foetal period was reported to be approximately 0.2 – 0.9 per cent. The patient was monitored clinically and by ultrasound for increasing size of head. The baby was delivered at 37 weeks through LSCS done by Dr Rajashri Tayshete on 3rd May 2023. The baby was kept in NICU for further evaluation and management under Dr. Nitu Mundhra, Consultant Neonatologist.
An MRI brain with whole spine screening was done and the baby was diagnosed to have Dandy Walker syndrome, a condition associated with large posterior fossa cyst, cerebellar atrophy and gross obstructive hydrocephalus. In this condition, the brain fluid (CSF) flow is disturbed as the CSF outflow pathway is not formed normally since birth leading to trapping of significant amounts of CSF within the brain and enlargement of the head size.
Treatment of Dandy Walker Syndrome
A neurosurgery team comprising of Dr. Ashwin Borkar and Dr. Vinod Rambal operated on the baby on the third day of life with a surgery called Ventriculoperitoneal shunt. In this surgery, a small hole is drilled in the skull, a small shunt tube is passed with one end into the ventricular (brain cavity) to drain CSF into the abdominal cavity at the other end. This procedure provides CSF diversion, relieves brain pressure and helps in normal brain growth.
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“After the successful surgery the baby showed remarkable improvement and both mother and child were discharged in healthy condition,” said Dr Ashwin Borkar, Consultant Neurosurgeon specialized in brain and spine surgery, Wockhardt Hospitals, Mira Road.
The patient’s mother Pooja Khunt noted that timely intervention by the doctors gave her son the second lease of life.
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