Congenital Biliary Atresia

Abstract

The prognosis of 97 patients with congenital biliary atresia treated by hepatic portoenterostomy was studied. In 37 of 97 patients (38%), good bile excretion was observed after surgery, and jaundice cleared. Sixteen of the 37 patients developed ascending cholangitis; 11, esophageal varices; and nine died. Seven died of ascending cholangitis and two of hepatic failure. Fourteen patients had an uneventful course. In the remaining 60 patients, bile excretion was scanty, and the disease progressed to biliary cirrhosis; 52 died. Causes of death were hepatic failure (53%), intracranial hemorrhage (16%), rupture of esophageal varices (12%), severe dehydration (9%), and postoperative complications (9%). The age at death ranged from 2 to 52 months, with an average at 16 months.

Long‐term results of surgical treatment of biliary atresia

“…Since the number of infants with successful restoration of bile flow is increasing continuously and the follow-up is longer, it may be easier to appreciate the extent of recovery. Our results are similar to those observed in other major series [3,5,6]. Sustained bile drainage with the disappearance of jaundice is obtained in 20-30% of patients but few have normal liver function, including normal BSP clearance.…”

“…The development of portal hypertension is frequent in all series with a long follow-up [2][3][4][5][6]. Our follow-up studies confirmed this, since progressive splenomegaly was a frequent finding and esophageal varices were discovered by serial esophagos-copy in almost one-half of the patients with splenomegaly.…”

“…In 1959, Kasai and Suzuki [1] devised hepatic portoenterostomy, a surgical procedure to correct extrahepatic biliary atresia of the "noncorrectable" type. Evidence of restoration of bile flow has been obtained in about 40-60% of patients operated on before 4 months of age [2][3][4][5][6]. Successful bile drainage, however, did not equate with cure and severe postoperative complications occurred, such as recurrent cholangitis, progressive liver failure, and portal hypertension.…”

Technique and results of operative management of biliary atresia

“…Cirrhosis, frequently associated with portal hypertension, has been reported by many authors in long-term survivors of operations for biliary atresia [2][3][4][5]25]. In our series, 2 patients showed signs of portal hypertension and another had giant splenomegaly.…”

“…Biliary atresia of the noncorrectable type has become a curable disease by the introduction of hepatic portoenterostomy. Recent reports [1][2][3] on follow-up studies of survivors after hepatic portoenterostomy indicate that many of the long-term survivors showed normal growth and development. Nevertheless, the results of operation for biliary atresia are still unsatisfactory, since sustained drainage of bile has been restored in only 30-45% of patients, and liver cirrhosis resulting in rupture of esophageal varices, hypersplenism, or hepatic in-sufficiency is not uncommon even in patients who were relieved of jaundice by operation [4][5][6].…”