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

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

doi:10.13029/jkaps.2013.19.1.1

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

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

Journal of the Korean Association of Pediatric Surgeons 2013;19(1):1-13.
Published online: June 28, 2013

DOI: https://doi.org/10.13029/jkaps.2013.19.1.1

Korean Association of Pediatric Surgeons, Korea.

Correspondence: Jung-Tak Oh, M.D., Department of Pediatric Surgery, Severance Children's Hospital Yonsei University College of Medicine 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea. Tel: 02)2228-2124, Fax: 02)313-8289, jtoh@yuhs.ac

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ABSTRACT
REFERENCES

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.
Keywords: Biliary atresia; Korea; Kasai operation; Liver transplantation

REFERENCES

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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

Fig. 2

Annual distribution of biliary atresia patients who underwent the Kasai operation

Fig. 3

Hospital distribution of biliary atresia patients who underwent the Kasai operation

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.

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.

Table 1

Characteristics of Biliary Atresia Patients who Underwent the Kasai Operation

^*Data from the 435 Kasai operation patients

^†Data from the 404 primary reports of 435 Kasai operation patients

Table 2

Associated Anomalies with Biliary Atresia

Table 3

Characteristics of the Kasai Operation

^*I: atresia of common bile duct

II: atresia of hepatic duct

III: atresia of bile duct at the porta hepatis

Table 4

Postoperative Course of the Kasai Operation

^*Multiple responses

Table 5

Follow-up of the Kasai Operation

^*Multiple responses

Table 6

Follow-up of Patients who Underwent Operation for Biliary Atresia before 2001

Figure & Data

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

J Korean Assoc Pediatr Surg. 2013;19(1):1-13. Published online June 28, 2013

DOI: https://doi.org/10.13029/jkaps.2013.19.1.1

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

J Korean Assoc Pediatr Surg. 2013;19(1):1-13. Published online June 28, 2013

DOI: https://doi.org/10.13029/jkaps.2013.19.1.1

Figure

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

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

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

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

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.

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.

Fig. 1

Fig. 2

Fig. 3

Fig. 4

Fig. 5

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

Table 1

Characteristics of Biliary Atresia Patients who Underwent the Kasai Operation

^*Data from the 435 Kasai operation patients

^†Data from the 404 primary reports of 435 Kasai operation patients

Table 2

Associated Anomalies with Biliary Atresia

Table 3

Characteristics of the Kasai Operation

^*I: atresia of common bile duct

II: atresia of hepatic duct

III: atresia of bile duct at the porta hepatis

Table 4

Postoperative Course of the Kasai Operation

^*Multiple responses

Table 5

Follow-up of the Kasai Operation

^*Multiple responses

Table 6

Follow-up of Patients who Underwent Operation for Biliary Atresia before 2001

Table 1 Characteristics of Biliary Atresia Patients who Underwent the Kasai Operation

^*Data from the 435 Kasai operation patients

^†Data from the 404 primary reports of 435 Kasai operation patients

Table 2 Associated Anomalies with Biliary Atresia

Table 3 Characteristics of the Kasai Operation

^*I: atresia of common bile duct

II: atresia of hepatic duct

III: atresia of bile duct at the porta hepatis

Table 4 Postoperative Course of the Kasai Operation

^*Multiple responses

Table 5 Follow-up of the Kasai Operation

^*Multiple responses

Table 6 Follow-up of Patients who Underwent Operation for Biliary Atresia before 2001

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