TOTAL ANOMALOUS PULMONARY VENOUS RETURN COMPLICATING PORTOENTEROSTOMYFOR BILIARY ATRESIA

Cardiovascular anomalies such as absent inferior vena cava and preduod enal portal vein are reported in cases of biliary atresia and make hep atic portoenterostomy a technical challenge. The authors present the c ase of a severe cardiac anomaly that significantly altered the functio nal outcome of a Kasai procedure. Baby M., an 8-week-old boy born with total anomalous pulmonary venous return (TAPVR), underwent hepatic po rtoenterostomy for biliary atresia. Over the next 3 months he remained icteric and febrile, and failed to gain weight. After multiple antibi otic treatments for suspected cholangitis, he underwent reexploration of the portoenterostomy, with no improvement in his overall condition. His prognosis was considered dismal because correction of the cardiac anomaly is associated with a high mortality rate (>90%). The cardiac surgeon agreed to attempt a cure of the TAPVR, provided liver transpla ntation is contemplated if the patient survived. Within 48 hours posto peratively, his hepatic function had improved drastically. He became a febrile, had an improved appetite and weight gain, and was finally dis charged 203 days after admission. One year later, he is thriving and r emains anicteric. The exact reason for this drastic improvement is not well understood, but the right-sided cardiac failure caused by the TA PVR had a significant effect on the functional outcome of the portoent erostomy. Copyright (C) 1994 by W.B. Saunders Company