Case report on failed Kasai-portoenterostomy for extrahepatic biliary atresia with neonatal cholestasis in one-month-old infant

Abstract

Biliary atresia is a neonatal onset, obstructive cholangiopathy of the intrahepatic or extrahepatic biliary system, leading to the build-up of bile in the liver. This case discusses a one-month-old infant who was previously diagnosed with jaundice, presenting the symptoms of yellowish eyes and skin, pale stools, and palpable liver. The infant and her mother’s liver enzyme levels were found to be higher and her histopathology studies evidenced atretic gall bladder. The liver biopsy revealed mild periportal ductular reaction and diffuse hepatocanalicular cholestasis. She was diagnosed with neonatal cholestasis- extrahepatic biliary atresia (EHBA) and underwent Kasai-portoenterostomy. She was stable and discharged with gallstone dissolution agents, antibiotics, vitamin supplements, and a barbiturates-liver enzyme inducer. After six months, she presented hepatosplenomegaly with ascites and was found to have transaminitis. She was then diagnosed with EHBA- failed Kasai, probable cholangitis, and planned for living donor liver transplantation. Antibiotics, antiviral, antifungal, anticoagulants, and immunosuppressants were prescribed on discharge. The Doppler study of allograft was performed to check the blood flow after transplantation. Acute graft rejection was monitored on day 5 with liver transplant pack reports. One year later, the infant's condition had shown improvement, evidenced by enhanced food intake, absence of symptoms, and the return of liver function tests to normal levels.