J Korean Assoc Pediatr Surg.  2013 Jun;19(1):1-13. 10.13029/jkaps.2013.19.1.1.

Biliary Atresia: A Survey by the Korean Association of Pediatric Surgeons in 2011

• Oh JT
• Kim DY
• Kim SC
• Kim IK
• Kim HY
• Kim HY
• Nam SH
• Park KW
• Park WH
• Park JY
• Seo JM
• Lee NH
• Lee MD
• Lee SK
• Lee SC
• Chung SY
• Jung SE
• Chung JH
• Choi KJ
• Choi SO
• Choi SH
• Choi YM
• Han SJ
• Hong J

• KMID: 1427979
• DOI: http://doi.org/10.13029/jkaps.2013.19.1.1

Abstract

The Korean Association of Pediatric Surgeons (KAPS) performed the second nationwide survey on biliary atresia in 2011. It was a follow-up study to the first survey, which was performed in 2001 for the retrospective analysis of biliary atresia between 1980 and 2000. In the second survey, the authors reviewed and analyzed the clinical data of patients who were treated for biliary atresia by the members of KAPS from 2001 to 2010. A total of 459 patients were registered. Among them, 435 patients primarily underwent the Kasai operation. The mean age of patients who underwent the Kasai operation was 66.2+/-28.7 days, and 89.7% of those patients had type III biliary atresia. Only five patients (1.4%) had complications related to the Kasai operation. After the Kasai operation, 269 (61.8%) of the patients were re-admitted because of cholangitis (79.9%) and varices (20.4%). One hundred and fifty-nine (36.6%) of the patients who underwent the Kasai operation subsequently underwent liver transplantation. The most common cause of subsequent liver transplantation was persistent hyperbilirubinemia. The mean interval between the Kasai operation and liver transplantation was 1.1+/-1.3 years. Overall the 10-year survival rate after the Kasai operation was 92.9% and the 10-year native liver survival rate was 59.8%. We had 23 patients for primary liver transplantation without the Kasai operation. The mean age patients who underwent primary liver transplantation was 8.6+/-2.9 months. In summary, among the 458 Kasai-operation and liver-transplantation patients, 373 lived, 31 died, and 54 were unavailable for follow up. One-third of the patient who survived have had complications correlated with biliary atresia. In comparison with the first survey, this study showed a higher survival rate and a greater number of liver transplantation.

Keyword

Biliary atresia; Korea; Kasai operation; Liver transplantation

MeSH Terms

Biliary Atresia
Cholangitis
Follow-Up Studies
Humans
Hyperbilirubinemia
Korea
Liver
Liver Transplantation
Retrospective Studies
Survival Rate
Varicose Veins

Figure

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  Fig. 1 Flowchart showing group of study population. 1° Report; report from the pediatric surgeons who performed the Kasai operation for the reported patients. 2° Report; report from the pediatric surgeons who did not perform the Kasai operation for the reported patients, but took care of them. LT: liver transplantation F/U: follow up

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  Fig. 2 Annual distribution of biliary atresia patients who underwent the Kasai operation

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  Fig. 3 Hospital distribution of biliary atresia patients who underwent the Kasai operation

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  Fig. 4 Patient survival rate analysis for 10 years: A) Overall survival rate after the Kasai operation; B) Comparison of the survival rate between hospitals with a large number of Kasai operations (Group I) and a small number of Kasai operations (Group II); and C) Comparison of the survival rate among six groups, five of which being hospitals (A, B, C, D, and E) with a large number of Kasai operations and the sixth (Group II) being the remainder of the hospitals (F-R). *Group I consists of A, B, C, D and E hospitals †Group II consists of F-R hospitals.

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  Fig. 5 Native liver survival rate analysis for 10 years: A) Overall native liver survival rate after the Kasai operation; B) Comparison of the native liver survival rate between hospitals with a large number of Kasai operations (Group I) and a small number of Kasai operations (Group II); and C) Comparison of the native liver survival rate among six groups, five of which being hospitals (A, B, C, D, and E) with a large number of Kasai operations and the sixth (Group II) being the remainder of the hospitals (F-R). *Group I consists of A, B, C, D and E hospitals †Group II consists of F-R hospitals.

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