Biliary atresia: should all patients undergo a portoenterostomy?

PURPOSE: The management of noncorrectable extra hepatic biliary atresia includes portoenterostomy, although the results of the surgery are variable. This study was done to develop criteria that could successfully predict the outcome of surgery based on preoperative data, including percutaneous liver biopsy, allowing a more selective approach to the care of these babies.
METHODS: The charts and biopsy results of 31 patients who underwent a Kasai procedure for biliary atresia between 1984 and 1994 were reviewed. Values for preoperative albumin, bilirubin, age of patient at Kasai, and lowest postoperative bilirubin were recorded. Surgical success was defined as postoperative bilirubin that returned to normal. A pathologist blinded to the child's eventual outcome graded the pre-Kasai needle liver biopsy results according to duct proliferation, ductal plate lesion, bile in ducts, lobular inflammation, giant cells, syncitial giant cells, focal necrosis, bridging necrosis, hepatocyte ballooning, bile in zone 1, 2, and 3, cholangitis, and end-stage cirrhosis. Clinical outcome was then predicted.
RESULTS: Success after portoenterostomy could not reliably be predicted based on gender, age at Kasai, preoperative bilirubin or albumin levels. Histological criteria, however, predicted outcome in 27 of 31 patients (P < .01). Fifteen of 17 clinical successes were correctly predicted; as were 12 of 14 clinical failures (sensitivity, 86%; specificity, 88%). Individually, the presence of syncitial giant cells, lobular inflammation, focal necrosis, bridging necrosis, and cholangitis, were each associated with failure of the portoenterostomy (P < .05). Bile in zone 1 was associated with clinical success of the procedure (P < .05).
CONCLUSIONS: Based on the predictive information available in a liver biopsy, we conclude that those patients who will not benefit from a Kasai procedure can be identified preoperatively, and channeled immediately to transplantation.