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Hong, Jung, Kang, Choi, and Park: Five-year Survival and Prognostic Factors after Kasai Portoenterostomy for Biliary Atresia
Original Article
Journal of the Korean Surgical Society

Journal of the Korean Surgical Society 2010; 79(5): 405-410.

Published online: 12 November 2010

DOI: https://doi.org/10.4174/jkss.2010.79.5.405

Five-year Survival and Prognostic Factors after Kasai Portoenterostomy for Biliary Atresia

A Rum Hong, M.D., Eunyoung Jung, M.D., Yu-Na Kang, M.D.1, Soon-Ok Choi, M.D., Woo-Hyun Park, M.D.

Department of Surgery, Dongsan Medical Center, Keimyung University School of Medicine, Daegu, Korea.

1Department of Pathology, Dongsan Medical Center, Keimyung University School of Medicine, Daegu, Korea.

Corresponding author (whp0903@dsmc.or.kr)

Received 15 June 2010       Accepted 9 August 2010

Copyright © 2010 The Korean Surgical Society

Abstract

Purpose

Long-term survival of biliary atresia patients after Kasai's portoenterostomy is being increasingly reported. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. The authors investigated 5-year survival rates and prognostic factors for survival after Kasai's portoenterostomy using univariate and multivariable methods.

Methods

The authors analyzed 5-year survival rates and prognostic factors in 56 patients who underwent Kasai's portoenterostomy during the period from 1991 to 2005 by the Kaplan-Meier model and Cox proportional hazards model, respectively. Both clinical factors and histological features of the liver and periportal fibrous mass were analyzed as prognostic factors.

Results

Younger ages of less than 90 days at surgery, clearance of jaundice and absence of post-Kasai cholangitis in 6 months were predictive of a favorable outcome. A significant difference between the live and the dead was found with regard to an average of 7 or more bile ductules in periportal fibrous mass in 200 HPF (P=0.013). The external diameter of the longest bile ductule in the periportal fibrous mass is not correlated to the prognosis (P=0.49). Independent factors for good prognosis were jaundice-free in 6 months and over 7 bile ductules density in periportal fibrous mass.

Conclusion

Favorable outcome after Kasai's portoenterostomy for biliary atresia is determined by bilirubin clearance in 6 months after Kasai operation and seven or more bile ductules in periportal fibrous mass. The 5-year post-Kasai survival rate is 77.2%.

Keywords: Biliary atresia, Kasai procedure, Prognosis, Survival

Figures and Tables

Fig. 1
Cumulative post-Kasai survival curves.
jkss-79-405-g001
Fig. 2
Cumulative post-Kasai survival curves by number of bile ductules (×200 HPF) in periportal fibrous mass.
jkss-79-405-g002
Table 1
Summary of patients with biliary atresia
jkss-79-405-i001
Table 2
Clinical variables related to 5-year survival rates
jkss-79-405-i002

*YSR = year survival rate.

Table 3
Independent risk factors for 5-year post-Kasai survival
jkss-79-405-i003

*95% CI = 95% confidence interval; †Bile ductule density = average of number of bile ductules in 200 HPF in 5 random field.

References

1. Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr. 2003. 37:4–21.
2. Rothenberg SS, Schroter GP, Karrer FM, Lilly JR. Cholangitis after the Kasai operation for biliary atresia. J Pediatr Surg. 1989. 24:729–732.
3. Scheuer PJ. Classification of chronic viral hepatitis: a need for reassessment. J Hepatol. 1991. 13:372–374.
4. Park WH, Choi SO. The intrahepatic biliary cyst in biliary atresia after Kasai operation. J Korean Assoc Pediatr Surg. 1999. 5:126–129.
5. Houwen RH, Zwierstra RP, Severijnen RS, Bouquet J, Madern G, Vos A, et al. Prognosis of extrahepatic biliary atresia. Arch Dis Child. 1989. 64:214–218.
6. Karrer FM, Lilly JR, Stewart BA, Hall RJ. Biliary atresia registry, 1976 to 1989. J Pediatr Surg. 1990. 25:1076–1080.
7. Howard ER, Davenport M. The treatment of biliary atresia in Europe 1969-1995. Tohoku J Exp Med. 1997. 181:75–83.
8. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet. 2000. 355:25–29.
9. Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K. Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg. 2003. 38:997–1000.
10. Shinkai M, Ohhama Y, Take H, Kitagawa N, Kudo H, Mochizuki K, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital. J Pediatr Gastroenterol Nutr. 2009. 48:443–450.
11. Chun YS, Kim WK, Park KW, Lee SC, Jung SE. Long-term outcome and prognostic factors after Kasai operation for biliary atresia. J Korean Surg Soc. 1999. 57:1016–1022.
12. Yoon CS, Han SJ, Park YN, Chung KS, Oh JT, Choi SH. Kasai operation for extrahepatic biliary atresia - survival and prognostic factors. J Korean Assoc Pediatr Surg. 2006. 12:202–212.
13. Davenport M, Kerkar N, Mieli-Vergani G, Mowat AP, Howard ER. Biliary atresia: the King's College Hospital experience (1974~1995). J Pediatr Surg. 1997. 32:479–485.
14. Park WH, Choi SO. Recent 9-year experience for biliary atresia with introduction of a new ultrasonographic diagnosis. J Korean Assoc Pediatr Surg. 2000. 6:19–26.
15. Choi SO, Park WH, Lee HJ, Woo SK. 'Triangular cord': a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg. 1996. 31:363–366.
16. Park WH, Choi SO, Lee HJ, Kim SP, Zeon SK, Lee SL. A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg. 1997. 32:1555–1559.
17. Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet. 2009. 374:1704–1713.
18. Altman RP, Lilly JR, Greenfeld J, Weinberg A, van Leeuwen K, Flanigan L. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers. Ann Surg. 1997. 226:348–353.
19. Wu ET, Chen HL, Ni YH, Lee PI, Hsu HY, Lai HS, et al. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome. Pediatr Surg Int. 2001. 17:390–395.
20. Wildhaber BE, Coran AG, Drongowski RA, Hirschl RB, Geiger JD, Lelli JL, et al. The Kasai portoenterostomy for biliary atresia: a review of a 27-year experience with 81 patients. J Pediatr Surg. 2003. 38:1480–1485.
21. Subramaniam R, Doig CM, Bowen J, Bruce J. Initial response to portoenterostomy determines long-term outcome in patients with biliary atresia. J Pediatr Surg. 2000. 35:593–597.
22. Schweizer P, Lünzmann K. Extrahepatic bile duct atresia: how efficient is the hepatoporto-enterostomy? Eur J Pediatr Surg. 1998. 8:150–154.
23. Roy P, Chatterjee U, Ganguli M, Banerjee S, Chatterjee SK, Basu AK. A histopathological study of liver and biliary remnants with clinical outcome in cases of extrahepatic biliary atresia. Indian J Pathol Microbiol. 2010. 53:101–105.
24. Mirza Q, Kvist N, Petersen BL. Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis--a Danish study. J Pediatr Surg. 2009. 44:1344–1348.
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