Macroscopic appearance at portoenterostomy—A prognostic variable in biliary atresia

Abstract

A number of features (eg, age at time of surgery, size of microscopic biliary ductules) have been suggested to be useful predictors of the efficacy of portoenterostomy in the treatment of biliary atresia. However, no previous study has attempted to quantify the macroscopic appearance of the degree of hepatobiliary damage found during initial surgery. Methods: A macroscopic appearance at portoenterostomy (MAP) score was calculated for 30 infants treated consecutively at King's College Hospital, London. The MAP score was summated from four features (liver consistency, size of portal remnants, degree of portal hypertension, and associated extrahepatic anomalies), with the possible range being from 0 to 6. The infants had follow-up prospectively for a median of 32 months. Results: Twenty infants were anicteric at the time of analysis; their median time to clear jaundice was 46 days (Success group; n = 20). Of the remaining infants, eight had undergone transplantation, one had died awaiting transplantation, and one was still jaundiced (Failure group; n = 10). There was a significant correlation between the MAP score and the age at time of surgery ( r s = .57, P = .0005), but only the MAP score was significantly different between the Success and Failure groups ( P = .02). Component analysis showed that the portal-remnant subscore contributed most to discrimination ( P = .007). The time until clearance of jaundice for the Success group was related to liver consistency ( P = .01) and portal remnants ( P = .02). Conclusion: Simple observation by an experienced surgeon (quantified as the MAP score) was a useful indicator of the success of this procedure and may be as valuable as the more sophisticated histological or biochemical tests.