Perioperative factors affecting the outcome following repair of biliary atresia.

The records of all patients with biliary atresia seen at the Childrens Hospital of Los Angeles during a 14-year period were reviewed. Of the 41 patients who could be evaluated, 32 were treated with trimethoprim and sulfamethoxazole prophylaxis, five were given other agents, and four received no prophylaxis after surgery. At least one episode of cholangitis occurred in nine patients. With one exception, all patients destined to have cholangitis did so within 9 months of surgery. Of the nine patients, five were receiving prophylaxis at the time the disease developed, but two were no longer receiving any prophylaxis. The other two patients in whom cholangitis developed never received antibiotic prophylaxis. In the 24 patients who had a Kasai type of portoenterostomy, cholangitis developed in three of the five (60%) with a Roux-en-Y limb length less than 40 cm and in two of the 19 (10.5%) with limb lengths greater than 40 cm (P less than .02). When performed earlier than 61 days after birth, surgery resulted in adequate bile flow in 64.7% (11/17) of patients who could be evaluated as compared with 31.8% (7/22) for surgery at 61 days or later (P less than .05). Of the patients with adequate biliary drainage 11 had no apparent liver disease, but only two of the patients with poor drainage were free of clinical liver disease. The conclusion from this series is that a combination of timely surgery, intestinal conduit at least 40 cm in length, and subsequent long-term antibiotic prophylaxis favors the best bile flow and reduces the occurrence of cholangitis, resulting in the best outcome.