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The outcome of the older (> or =100 days) infant with biliary atresia
M Davenport1, V Puricelli, P Farrant
1Department of Paediatric Surgery, King's College Hospital, Denmark Hill, London, England, UK.
Insights
Kasai portoenterostomy may offer reasonable medium-term survival for infants over 100 days with biliary atresia (BA). Age alone should not dictate primary liver transplantation, as some patients achieve good outcomes with surgery.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Increasing age negatively impacts outcomes of Kasai portoenterostomy for biliary atresia (BA).
- Some centers advocate primary liver transplantation for older infants with BA, irrespective of other factors.
Purpose of the Study:
- To evaluate the outcomes of Kasai portoenterostomy in infants with biliary atresia (BA) diagnosed at or after 100 days of age.
- To identify predictive criteria for outcomes in this patient group.
Main Methods:
- Retrospective analysis of infants with BA aged >= 100 days who underwent surgery between 1980-2000.
- Calculation of actuarial survival rates (death and transplantation as endpoints).
- Review of preoperative ultrasonography and liver histology for predictive criteria.
Main Results:
- 35 infants (8.2%) were >= 100 days at surgery; 26 underwent portoenterostomy.
- 5- and 10-year actuarial survival with native liver was 45% and 40%, respectively.
- 35% of patients are alive with native liver, 28% received transplantation, 13% died. Ultrasonography showing heterogeneous parenchyma predicted poor outcome but lacked sensitivity.
Conclusions:
- Approximately one-third of infants aged >= 100 days with BA can achieve reasonable medium-term survival with primary corrective surgery.
- In the absence of accurate discrimination, primary surgery is favored over routine transplantation based solely on age.
Background:
There is a detrimental effect of increasing age on the results of the Kasai portoenterostomy for biliary atresia (BA), and some centers routinely advocate primary liver transplantation for the older infant, irrespective of other criteria. This perception that such infants are indeed irretrievable was tested by retrospective analysis.
Methods:
All infants who had undergone surgery for BA during the period 1980 through 2000 aged > or =100 days were reviewed. Actuarial survival was calculated using 2 end-points (death and transplantation). A retrospective review of their ultrasonography (n = 12) and preoperative liver histology (n = 22) was also undertaken to ascertain possible predictive criteria.
Results:
A total of 422 infants had BA diagnosed during this period, of which 35 (8.2%) were > or =100 days at surgery (median [interquartile range], 133 [range, 108 to 180] days). Surgery included portoenterostomy (n = 26), hepaticojejunostomy (n = 7), and a resection and end-to-end anastomosis (n = 1). A laparotomy only was performed in 1. Five- and 10-year actuarial survival rate with native liver was 45% and 40%, respectively. Currently, 12 (35%) patients are alive with their native liver (8 are anicteric), 9 (28%) have undergone transplantation, and 13 have died. Although there were some survival advantages for types 1 or 2 BA and "noncirrhosis" at time of surgery, neither reached statistical significance. Individual histologic features (eg, degrees of fibrosis, giant cell transformation, bile duct destruction) in the retrospective review of available material were not discriminatory. The finding of a "heterogeneous" parenchyma on ultrasonography was predictive of poor outcome but lacked sensitivity.
Conclusions:
The potential for reasonable medium-term survival is present in about one third of infants 100 days or older coming to primary corrective surgery. In the absence of accurate discrimination, the authors continue to favor this option rather than subject all to transplant simply on the basis of age.
