Long-term outcomes of biliary atresia with splenic malformation

Background We assessed the long-term outcomes of patients with biliary atresia with splenic malformation (BASM). Methods We retrospectively assessed outcomes of 255 patients who underwent the Kasai procedure (KP) at our hospital between 1972 and 2014. Clinical outcomes of 11 patients with BASM (group A: nine with polysplenia, two with asplenia) and 244 patients with isolated BA (group B) were compared. Results The incidence of early cholangitis and hepatopulmonary syndrome (HPS) was significantly higher in group A than in group B. Of the 11 group A patients, three died of severe cardiac defects during early infancy. Seven became jaundice free following KP, with three patients subsequently requiring liver transplantation (LTx). Four survived with their native livers for 2, 5, 22, and 23 years, respectively. Overall 20-year survival rates were 63.6% and 66.5% and 20-year native liver survival rates were 29.0% and 47.3% in groups A and B, respectively. No significant difference in cumulative survival rates was observed between both groups. Conclusions Long-term outcomes in BASM patients without lethal cardiac defects were comparable to patients with isolated BA. Careful follow-up may be required in patients with BASM because of a potentially higher risk of secondary complications such as HPS.