Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese biliary atresia registry

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Abstract

Purpose

The aim of this study was to elucidate the epidemiology and short- and long-term results of biliary atresia in Japan analyzing the data of the Japanese Biliary Atresia Registry (JBAR).

Methods

In 1989, the Japanese Biliary Atresia Society started a nationwide registry, JBAR, to investigate all aspects of biliary atresia. A total of 1,381 patients, 863 girls, 507 boys, and 11 unknown, were registered between 1989 and 1999. JBAR includes an initial and follow-up questionnaires. Using these patients’ data, the incidence, sex distribution, associated anomalies, the type of obstruction, the type of operation, and the surgical results were evaluated. The 5- and the 10-year results of 735 patients who were registered initially in or before 1994 also were analyzed.

Results

The incidence of biliary atresia was 1 in 9,640 live births. One hundred sixty-four patients (11.9%) had type I atresia of the common bile duct, 34 (2.5%) had type II atresia of the hepatic ducts, and 1,162 (84.1%) had type III atresia at the porta hepatis. Congenital associated anomalies were found in 19.6% of the patients including 33 cases associated with polysplenia. Impact of the age at operation on bile flow was not clear until 90 days of age, and after 90 days the bile flow rate worsened. The original Roux-en-Y procedure had been used in more than 50% of the patients since 1995. In 1999, 96% of the patients underwent the original Roux-en-Y procedure or the Roux-en-Y with an intestinal valve, and only 3 patients (3.5%) underwent other modifications. There were no significant differences in either the rate of disappearance of jaundice or the incidence of cholangitis among these 3 procedures. Of the 735 patients registered in or before 1994, 19 patients (2.6%) were lost to follow-up. The 5-year survival rates of patients registered in 1989, 1990, 1991, 1992, 1993, and 1994 were 62%, 64.5%, 61.3%, 59.0%, 58.7%, and 52.7% without liver transplantation (LTx), and 69.4%, 74.2%, 75.2%, 79.5%, 78%, and 78.3% with LTx, respectively. Although the overall 5-year survival rate changed from 69.4% to 78.3%, the difference was not statistically significant. According to the 10-year follow-up results of the 108 patients initially registered in 1989, 72 (66.7%) and 57 (52.8%) survived with and without the aid of LTx, respectively.

Conclusions

The overall 5- and 10-year survival rates were 75.3% (553 of 734) and 66.7% (72 of 108), respectively. In spite of the increasing number of survivors after LTx, there was no significant improvement in the 5-year survival rate. It was shown that the JBAR system was functioning well with only 19 patients lost to follow-up among the 743 patients registered from 1989 to 1994.

Section snippets

Materials and methods

The JBAR, consisting of an initial questionnaire and follow-up questionnaires, were sent to all major institutions (132 institutions) belonging to the Japanese Society of Pediatric Surgeons. The initial questionnaire was designed to obtain patients’ information including gestational history, perinatal history, diagnosis, treatment, and early results. Once a patient is registered, the institution is obliged each year to complete and return the follow-up questionnaire including current results of

Results of the initial questionnaire

The number of patients registered each year is shown in Table 1. Between 100 and 150 patients were registered every year. There was no clear tendency of increase or decrease in these 11 years. Because the total number of live births in Japan between 1989 and 1999 was 13,313,000, the incidence was 1 in 9,640 live births. The sex ratio showed a female to male predominance of 1:0.59 (863 girls and 507 boys). The family history of surgical hepatobiliary diseases was found in 3 patients, 2 siblings

Discussion

A nationwide surveillance like the JBAR was carried out in US-Canada (1976 to 89),1 France (1986 to 96),2 and UK-Ireland (1993 to 95).3 The JBAR is still ongoing and has become the largest one.

The incidence of biliary atresia in Japan was estimated to be 10.4 in 100,000 live births from the current study. According to the report by Chardot’s et al,2 this value is the third highest after the 32 in 100,000 live births in French Polynesia and 10.6 in 100,000 live births in Hawaii. Considering that

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