Biliary atresia associated with congenital structural anomalies.

BACKGROUND/PURPOSE: Although biliary atresia (BA) is rarely associated with other congenital anomalies, the presence of a distinct subgroup of patients with accompanying structural anomalies such as situs inversus, polysplenia, or portal vein anomalies has been postulated. The authors present 7 patients with this association.

METHODS

Of 87 patients with BA treated in the past 19 years, 7 (8.0%) have had multiple congenital structural anomalies.

RESULTS

These anomalies included situs inversus in 4, polysplenia in 5, preduodenal portal vein in 5, absent portal vein in 1, absent inferior vena cava in 2, malrotation of the intestine in 5, and congenital heart disease in 3 patients. In these 7 patients, hepatic portoenterostomy (HPE) was performed at the age from 63 to 158 days with an average of 92 days. The porta hepatis was abnormal in position in 1 patient. The connective tissue at the porta hepatis was diminished in 6 patients. Histologically, liver fibrosis was mild in 2 and moderate in 5 patients. Bile excretion was good initially in all patients but gradually diminished in 5 patients. Five patients had multiple episodes of cholangitis, followed by sepsis, liver failure, or cardiac failure and subsequently died at the age from 2 months to 6 years. Of the other 2 patients who underwent HPE recently, 1 is doing well and the other has had one episode of cholangitis.

CONCLUSIONS

BA in association with other congenital structural anomalies may have a poor prognosis. These patients have poor bile secretion after HPE mainly because of delayed operation.