BILIARY ATRESIA AND THE POLYSPLENIA SYNDROME - ITS IMPACT ON FINAL OUTCOME

Up to 25% of babies with biliary atresia (BA) bear associated malforma tions that most often cluster in the polysplenia syndrome (PS). This a rticle examines the impact of associated PS on the final outcome of th e authors' BA patients and the necessary modifications of surgical tec hnique during orthotopic liver transplantation (OLT) in these patients . The authors studied 88 consecutive infants with biliary atresia oper ated upon during the 10 year period between 1984 and 1993. Biliary dra inage was provided by a Roux-en-Y hepaticojejunostomy. OLT was perform ed in 38. Eleven infants (12%) had three or more components of PS: mal rotation (11), preduodenal portal vein (11), polysplenia (10), situs i nversus (4), absent inferior vena cava (3), cardiac defects (2), and a nomalous hepatic artery supply (2), Bile drainage was achieved in 7/11 infants (63% versus 48% for regular BA patients) 5 of whom (45% versu s 31%) are jaundice-free with a mean follow-up of 105 months. Three pa tients received transplants (2 from living-related donor) and are aliv e and well with normal liver function after a mean follow-up of 35 mon ths. Overall survival was 72% in the BA plus PS and 66% in the BA grou p (P = not significant [NS]). Results show that long-term bile drainag e in these PS patients can be achieved at least as often as in other B A patients (45% versus 31%) (P = NS) and indicate that Kasai operation remains the best initial choice. When OLT becomes necessary, attentio n to vascular anatomy is required to determine the feasibility of reco nstruction. The absence of inferior vena cava may facilitate the impla ntation and reduce postoperative renal dysfunction because the vena ca va is not occluded during the anhepatic phase.