Is early response to portoenterostomy predictive of long-term outcome for patients with biliary atresia?

Purpose: Therapy for biliary atresia (BA) typically involves portoenterostomy (PE). In light of the development of liver transplantation (LT) as an option for therapy in infancy, the authors reviewed their experience to determine factors that might predict the requirement for LT. Methods: Cases of BA diagnosed between September 1980 and September 1994 were reviewed. Responses to PE were rated as poor (PR; death or LT by 3 years), temporary (TR; LT > 3 years), or good (GR; anicteric). Results: Twenty-nine patients were identified; 24% were native indian or Inuit. Twenty-three had PE; 11 responded. Seven (32%) became anicteric and continue to do well (GR). Four (18%) required LT after age 3 (TR). Twelve patients had PR; 3 underwent LT (average age, 1.3 years), 4 are listed for LTX, and 5 died by age 2.8 years. (Six patients did not have PE; 2 died in infancy and 4 had LT.) All transplant recipients are well. Factors associated with PR were older age at time of surgery (67 ± 7 days v 51 ± 4 days in the GR group), nadir of AST (273 ± 84 U/L v 70 ± 26 U/L in the GR group), and number of post-PE complications (3.6 v per patient v 1 per patient in the GR group). TR was differentiated from GR by bilirubin nadir (46 ± 10 μmol/L v 14 ± 3 μmol/L, respectively) and rate of bilirubin decline (2.6 ± 1.5 μmol/L/d v 10.8 ± 3.0 μmol/L/d, respectively). (P < .05 for all comparisons.) Conclusion: Outcomes are comparable to those of North American series, but the incidence is lower overall and is higher among natives. Factors that correlate with outcome include age at time of surgery, post-PE complications, postoperative decline and rate of decline of bilirubin and AST. The latter had not been reported previously and may prove useful in planning therapy for partial responders after PE.