Pediatrics & Therapeutics
Open Access

Abstract

Long-Term Outcome of Children with Biliary Atresia after Kasei Surgery in Iranian Infants

Mastaneh Moghtaderi, Mojtaba Gorji, Fatemeh Farahmand, Golamhosein Fallahi and Bahar Ashjai

Aim: The aims of the present study were to evaluate the long-term prognosis of children with biliary atresia after the Kasai surgery and to analyze the present status of survivors retaining their own liver function.

Background: Biliary atresia is the most common cause of pathologic infantile jaundice that results from obstructions of extra hepatic bile ducts due to inflammation and fibrosis. It is a progressive disorder and gradually results in cirrhosis, portal hypertension and hepatic failure. The disease progression to cirrhosis and hepatic failure could be prevented with Kasai surgery.

Method and patients: This is a descriptive retrospective cohort study in which we evaluated patients with biliary atresia who admitted between 1998 and 2008 and underwent Kasai surgical procedure.

Results: A total of 49 patients had Kasai surgery. Patients’ survival rates were 14.3%, 8.1% and 2.1%, 2-5, 5-10 and >10 years after the surgery, respectively. The incidence of cholangitis was 59.1% and gastrointestinal bleeding 38.7% following the surgery. Four patients had liver transplantation (8.1%) and only 2 survived (50%). The survival rates varied significantly depending on the age at Kasai operation. Of the 10 patients who underwent Kasai procedure during the first 8 weeks of life, 6 survived (60%) and 4 died. Of the 23 patients who had Kasai surgery between 8-12 weeks of life, 4 survived (17.3%) and 19 (82.6%) died. Of the 16 patients who had kasai operation after 12 weeks of life, 2 survived (12.5%) and 14 (87.5%) died.

Conclusion: This clinical trial confirms previous studies that Kasai surgery is an effective procedure for the treatment of children with biliary atresia and that the long-term survival rate can be improved if Kasai operation is performed before the first 8 weeks of life.