Surgical treatment for intractable pruritus in progressive familial intrahepatic cholestasis

Progressive familial intrahepatic cholestasis (PFIC) is one of the causes of childhood end stage liver disease. It is an autosomal recessive disorder, characterized by pruritus, coagulopathy, growth retardation, jaundice, and subsequently cirrhosis and hepatic failure due to impaired bile acid transport and metabolism. Diversion of bile, internally or externally, from the terminal ileum, to decrease re- uptake, is a viable option for relieving pruritus. Four children with PFIC type1 were treated with partial internal biliary diversion (PIBD) from June 2014 To March 2017 in the Unit of Paediatric surgery, Jinnah Hospital Lahore. The ages of patients were from four months to five years. Three were girls and one was a boy. The main symptom common to all was intractable pruritus. There was relief in pruritus, observed within first week postoperatively. They had been able to sleep without pruritis associated awakening episodes. PIBD is an effective technique for relieving the most devastating symptom of pruritus in PFIC.