HYDERABAD: In one of the rarest of rare procedures, the doctors at Osmania General Hospital performed a liver transplant surgery on a 8-month-old diagnosed with scalp hypotrichosis.The child’s mother works as an Anganwadi Sevika and father is a coolie in Jagtial district. The child was diagnosed with Neonatal Ichthyosis Scalp Alopecia Cholangiohepatitis (NISCH) syndrome. He was suffering from dry scaly skin, no scalp hair, jaundice and water formation in the abdomen since birth.
“Performing liver transplantation surgery on a small child with skin changes was really challenging,” said Dr Ch Madhusudhan, professor of surgical gastroenterology. The surgery lasted for approximately 18 hours.Till now only 18 cases of NISCH syndrome have been reported across the world. Among them, only 4 underwent liver transplantation. This is the first patient with NISCH syndrome who underwent liver transplantation in India.
The syndrome is often found in children of consanguineous couples. The child’s parents were close relatives. Their first child had died immediately after the birth due to the same disease.The operation took place on June 17 at Osmania General Hospital Hyderabad with pediatric support from Niloufer hospital.