Consultation with the Specialist: Liver Transplantation for Biliary Atresia

Frank R. Sinatra

doi:10.1542/pir.22-5-166

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Frank R. Sinatra, MD^*

^*Editorial Board.

Introduction

Without treatment, biliary atresia is a uniformly fatal disorder. The median survival is approximately 8 months, with the vast majority of affected patients dying during the first 2 years of life. The hepatoportoenterostomy (Kasai procedure), developed in the late 1960s, provided a mechanism of establishing bile flow in approximately 80% of infants undergoing the procedure during the first 60 days of life, resulting in a 10-year survival rate without transplantation of approximately 25% (Figure⇓ ). For the remaining 75% of infants, who either fail to establish initial bile flow or who develop progressive fibrosis despite resolution of jaundice, liver transplantation is a well-established, successful procedure.

Figure 1.

Prognosis following hepatoportoenterostomy (Kasai procedure).

Case Presentation

Kevin is a 9-year-old boy who is brought to the emergency department with a 24-hour history of melena, progressive pallor, and dizziness. On physical examination, he is pale and has tachycardia and postural hypotension. His height and weight are appropriate for age. There is no apparent jaundice. Evaluation of his abdomen reveals a well-healed surgical scar and dilated superficial abdominal veins. His liver is hard and palpable 2 cm below the right costal margin and 6 cm below the xyphoid process. The spleen is palpable 8 cm below the left costal margin. Medical history is remarkable for jaundice at 3 weeks of age and a hepatoportoenterostomy (Kasai procedure) for treatment of biliary atresia at 5 weeks of age. The jaundice resolved following surgery, and the boy has been healthy for the past 9 years. He has had no prior episodes of apparent gastrointestinal bleeding. His pediatrician has noted, however, that his spleen has been increasing in size, and he has had a progressive decrease in both white blood cell and platelet counts over the past 4 years. He has had no episodes of jaundice or abdominal …