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Biliary atresia and the polysplenia syndrome: its impact on final outcome
J Vazquez1, J C López Gutierrez, M Gámez
1Department of Pediatric Surgery, Hospital Infantil La Paz, Madrid, Spain.
Insights
Biliary atresia (BA) with polysplenia syndrome (PS) has similar outcomes to BA alone. The Kasai operation is the preferred initial treatment, with liver transplantation requiring careful vascular assessment.
Area of Science:
- Pediatric Surgery
- Hepatology
- Congenital Malformations
Background:
- Biliary atresia (BA) affects up to 25% of infants, often associated with polysplenia syndrome (PS).
- Associated malformations in PS can complicate surgical outcomes in BA patients.
Purpose of the Study:
- To evaluate the impact of polysplenia syndrome on biliary atresia patient outcomes.
- To identify necessary surgical modifications for orthotopic liver transplantation (OLT) in BA patients with PS.
Main Methods:
- Retrospective study of 88 infants with biliary atresia between 1984-1993.
- Roux-en-Y hepaticojejunostomy for biliary drainage; OLT performed in 38 patients.
- Detailed analysis of 11 infants (12%) with three or more PS components and their surgical outcomes.
Main Results:
- Bile drainage success was 63% in BA+PS versus 48% in BA alone; 45% remained jaundice-free (vs. 31%).
- Overall survival rates were comparable: 72% for BA+PS and 66% for BA (P=NS).
- Three BA+PS patients underwent OLT and are alive with normal liver function.
Conclusions:
- Long-term bile drainage is achievable in BA patients with PS, comparable to those without PS.
- The Kasai operation is the recommended initial surgical approach for BA.
- Orthotopic liver transplantation in BA+PS requires meticulous attention to vascular anatomy for successful reconstruction.
Abstract:
Up to 25% of babies with biliary atresia (BA) bear associated malformations that most often cluster in the polysplenia syndrome (PS). This article examines the impact of associated PS on the final outcome of the authors' BA patients and the necessary modifications of surgical technique during orthotopic liver transplantation (OLT) in these patients. The authors studied 88 consecutive infants with biliary atresia operated upon during the 10-year period between 1984 and 1993. Biliary drainage was provided by a Roux-en-Y hepaticojejunostomy. OLT was performed in 38. Eleven infants (12%) had three or more components of PS: malrotation (11), preduodenal portal vein (11), polysplenia (10), situs inversus (4), absent inferior vena cava (3), cardiac defects (2), and anomalous hepatic artery supply (2). Bile drainage was achieved in 7/11 infants (63% versus 48% for regular BA patients) 5 of whom (45% versus 31%) are jaundice-free with a mean follow-up of 105 months. Three patients received transplants (2 from living-related donor) and are alive and well with normal liver function after a mean follow-up of 35 months. Overall survival was 72% in the BA plus PS and 66% in the BA group (P = not significant [NS]). Results show that long-term bile drainage in these PS patients can be achieved at least as often as in other BA patients (45% versus 31%) (P = NS) and indicate that Kasai operation remains the best initial choice. When OLT becomes necessary, attention to vascular anatomy is required to determine the feasibility of reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)