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. 1996 Sep;224(3):276–287. doi: 10.1097/00000658-199609000-00004

Orthotopic liver transplantation for congenital biliary atresia. An 11-year, single-center experience.

J A Goss 1, C R Shackleton 1, K Swenson 1, N L Satou 1, B J Nuesse 1, D K Imagawa 1, M M Kinkhabwala 1, P Seu 1, J S Markowitz 1, S M Rudich 1, S V McDiarmid 1, R W Busuttil 1
PMCID: PMC1235366  PMID: 8813256

Abstract

OBJECTIVE: The authors analyze a single center's 11-year experience with 190 orthotopic liver transplants for congenital biliary atresia. SUMMARY BACKGROUND DATA: Hepatic portoenterostomy generally is the initial treatment for children with congenital biliary atresia. Despite multiple modifications of the hepatic portoenterostomy, two thirds of treated patients still develop recurrent cholestasis, portal hypertension, cholangitis, and cirrhosis. Therefore, the only hope of long-term survival in the majority of children with congenital biliary atresia is definitive correction with orthotopic liver transplantation. METHODS: The medical records of 190 consecutive patients undergoing orthotopic liver transplantation for congenital biliary atresia from July 1, 1984 to February 29, 1996 were reviewed. Results were analyzed via Cox multivariate regression analysis to determine the statistical strength of independent associations between pretransplant covariates and patient and graft survival. Actuarial patient and graft survival was determined at 1, 2, and 5 years. The type and incidence of post-transplant complications were determined, as was the quality of long-term graft function. The median follow-up period was 3.21 years. RESULTS: The liver grafts were comprised on 155 whole-organ, 24 reduced-size, and 11 living donor organs. Median pretransplant values for recipient age, weight, and total bilirubin were 1.4 years, 12.3 kg, and 13.8 mg/dL, respectively. One hundred sixty-four patients (86%) had undergone prior hepatic portoenterostomy. Eighty-seven patients (46%) were United Network for Organ Sharing (UNOS) status 1 or 2 at the time of liver transplantation. The majority (15/24, 62%) of reduced-size graft recipients were UNOS status I at the time of transplantation. One hundred fifty-nine patients (84%) received a single graft, whereas 31 patients required 37 retransplants. The 1, 2, and 5 year actuarial patient survival rates were 83%, 80% and 78% respectively, whereas graft survival rates were 81%, 77%, and 76%, respectively. Cox multivariate regression analysis demonstrated that pretransplant total bilirubin, UNOS status, and graft type significantly predicted patient survival, whereas recipient age, weight, and previous hepatic portoenterostomy did not. Current median follow-up values for total bilirubin and aspartate aminotransferase levels in the 154 surviving patients were 0.5 mg/dL and 34 international units/L, respectively. CONCLUSION: Long-term patient survival after orthotopic liver transplantation for congenital biliary atresia is excellent and is independent of recipient age, weight, or previous hepatic portoenterostomy. Optimal results are obtained in this patient population when liver transplantation is performed before marked hyperbilirubinemia, and when possible, using a living-donor graft.

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Selected References

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  1. Adelman S. Prognosis of uncorrected biliary atresia: an update. J Pediatr Surg. 1978 Aug;13(4):389–391. doi: 10.1016/s0022-3468(78)80462-x. [DOI] [PubMed] [Google Scholar]
  2. Alagille D. Liver transplantation in children--indications in cholestatic states. Transplant Proc. 1987 Aug;19(4):3242–3248. [PubMed] [Google Scholar]
  3. Andrews W. S., Wanek E., Fyock B., Gray S., Benser M. Pediatric liver transplantation: a 3-year experience. J Pediatr Surg. 1989 Jan;24(1):77–82. doi: 10.1016/s0022-3468(89)80306-9. [DOI] [PubMed] [Google Scholar]
  4. Belzer F. O., D'Alessandro A. M., Hoffmann R. M., Knechtle S. J., Reed A., Pirsch J. D., Kalayoglu M., Sollinger H. W. The use of UW solution in clinical transplantation. A 4-year experience. Ann Surg. 1992 Jun;215(6):579–585. doi: 10.1097/00000658-199206000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Bismuth H., Houssin D. Reduced-sized orthotopic liver graft in hepatic transplantation in children. Surgery. 1984 Mar;95(3):367–370. [PubMed] [Google Scholar]
  6. Broelsch C. E., Whitington P. F., Emond J. C., Heffron T. G., Thistlethwaite J. R., Stevens L., Piper J., Whitington S. H., Lichtor J. L. Liver transplantation in children from living related donors. Surgical techniques and results. Ann Surg. 1991 Oct;214(4):428–439. doi: 10.1097/00000658-199110000-00007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Burdelski M., Schmidt K., Hoyer P. F., Bernsau U., Galaske R., Brodehl J., Ringe B., Lauchart W., Wonigeit K., Pichlmayr R. Liver transplantation in children: the Hannover experience. Transplant Proc. 1987 Aug;19(4):3277–3281. [PubMed] [Google Scholar]
  8. Busuttil R. W., Colonna J. O., 2nd, Hiatt J. R., Brems J. J., el Khoury G., Goldstein L. I., Quinones-Baldrich W. J., Abdul-Rasool I. H., Ramming K. P. The first 100 liver transplants at UCLA. Ann Surg. 1987 Oct;206(4):387–402. doi: 10.1097/00000658-198710000-00001. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Busuttil R. W., Seu P., Millis J. M., Olthoff K. M., Hiatt J. R., Milewicz A., Nuesse B., el-Khoury G., Raybould D., Nyerges A. Liver transplantation in children. Ann Surg. 1991 Jan;213(1):48–57. doi: 10.1097/00000658-199101000-00009. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Esquivel C. O., Koneru B., Karrer F., Todo S., Iwatsuki S., Gordon R. D., Makowka L., Marsh W. J., Jr, Starzl T. E. Liver transplantation before 1 year of age. J Pediatr. 1987 Apr;110(4):545–548. doi: 10.1016/s0022-3476(87)80545-0. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Galumbeck M. A., Sanfilippo F. P., Hagen P. O., Seaber A. V., Urbaniak J. R. Inhibition of vessel allograft rejection by endothelial removal. Morphologic and ultrastructural changes. Ann Surg. 1987 Dec;206(6):757–764. doi: 10.1097/00000658-198712000-00012. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Gautier M., Eliot N. Extrahepatic biliary atresia. Morphological study of 98 biliary remnants. Arch Pathol Lab Med. 1981 Aug;105(8):397–402. [PubMed] [Google Scholar]
  13. HAYS D. M., SNYDER W. H., Jr LIFE-SPAN IN UNTREATED BILIARY ATRESIA. Surgery. 1963 Aug;54:373–375. [PubMed] [Google Scholar]
  14. Hadchouel M., Hugon R. N., Odievre M. Immunoglobulin deposits in the biliary remnants of extrahepatic biliary atresia: a study by immunoperoxidase staining in 128 infants. Histopathology. 1981 Mar;5(2):217–221. doi: 10.1111/j.1365-2559.1981.tb01779.x. [DOI] [PubMed] [Google Scholar]
  15. Inomoto T., Nishizawa F., Sasaki H., Terajima H., Shirakata Y., Miyamoto S., Nagata I., Fujimoto M., Moriyasu F., Tanaka K. Experiences of 120 microsurgical reconstructions of hepatic artery in living related liver transplantation. Surgery. 1996 Jan;119(1):20–26. doi: 10.1016/s0039-6060(96)80208-x. [DOI] [PubMed] [Google Scholar]
  16. Kalayoglu M., D'Alessandro A. M., Knechtle S. J., Eckhoff D. E., Pirsch J. D., Judd R., Sollinger H. W., Hoffmann R. M., Belzer F. O. Long-term results of liver transplantation for biliary atresia. Surgery. 1993 Oct;114(4):711–718. [PubMed] [Google Scholar]
  17. Kalayoglu M., Sollinger H. W., Stratta R. J., D'Alessandro A. M., Hoffmann R. M., Pirsch J. D., Belzer F. O. Extended preservation of the liver for clinical transplantation. Lancet. 1988 Mar 19;1(8586):617–619. doi: 10.1016/s0140-6736(88)91416-x. [DOI] [PubMed] [Google Scholar]
  18. Kalayoglu M., Stratta R. J., Sollinger H. W., Hoffmann R. M., D'Alessandro A. M., Pirsch J. D., Belzer F. O. Liver transplantation in infants and children. J Pediatr Surg. 1989 Jan;24(1):70–76. doi: 10.1016/s0022-3468(89)80305-7. [DOI] [PubMed] [Google Scholar]
  19. Karrer F. M., Lilly J. R., Stewart B. A., Hall R. J. Biliary atresia registry, 1976 to 1989. J Pediatr Surg. 1990 Oct;25(10):1076–1081. doi: 10.1016/0022-3468(90)90222-u. [DOI] [PubMed] [Google Scholar]
  20. Langnas A. N., Marujo W., Stratta R. J., Wood R. P., Shaw B. W., Jr Vascular complications after orthotopic liver transplantation. Am J Surg. 1991 Jan;161(1):76–83. doi: 10.1016/0002-9610(91)90364-j. [DOI] [PubMed] [Google Scholar]
  21. Lynch S. V., Akiyama T., Ong T. H., Pillay S. P., Balderson G. A., Matsunami H., Shepherd R. W., Cleghorn G. J., Patrick M. K., Strong R. W. Transplantation in children with biliary atresia. Transplant Proc. 1992 Feb;24(1):186–188. [PubMed] [Google Scholar]
  22. Mazzaferro V., Esquivel C. O., Makowka L., Belle S., Kahn D., Koneru B., Scantlebury V. P., Stieber A. C., Todo S., Tzakis A. G. Hepatic artery thrombosis after pediatric liver transplantation--a medical or surgical event? Transplantation. 1989 Jun;47(6):971–977. doi: 10.1097/00007890-198906000-00011. [DOI] [PubMed] [Google Scholar]
  23. Meister R. K., Esquivel C. O., Cox K. L., Concepcion W., Berquist W., Nakazato P., deVries P. A. The influence of portoenterostomy with stoma on morbidity in pediatric patients with biliary atresia undergoing orthotopic liver transplantation. J Pediatr Surg. 1993 Mar;28(3):387–390. doi: 10.1016/0022-3468(93)90237-f. [DOI] [PubMed] [Google Scholar]
  24. Millis J. M., Brems J. J., Hiatt J. R., Klein A. S., Ashizawa T., Ramming K. P., Quinones-Baldrich W. J., Busuttil R. W. Orthotopic liver transplantation for biliary atresia. Evolution of management. Arch Surg. 1988 Oct;123(10):1237–1239. doi: 10.1001/archsurg.1988.01400340063011. [DOI] [PubMed] [Google Scholar]
  25. Mor E., Schwartz M. E., Sheiner P. A., Menesses P., Hytiroglou P., Emre S., Kishikawa K., Chiodini S., Miller C. M. Prolonged preservation in University of Wisconsin solution associated with hepatic artery thrombosis after orthotopic liver transplantation. Transplantation. 1993 Dec;56(6):1399–1402. doi: 10.1097/00007890-199312000-00024. [DOI] [PubMed] [Google Scholar]
  26. Ohi R., Hanamatsu M., Mochizuki I., Chiba T., Kasai M. Progress in the treatment of biliary atresia. World J Surg. 1985 Apr;9(2):285–293. doi: 10.1007/BF01656322. [DOI] [PubMed] [Google Scholar]
  27. Ohi R., Ibrahim M. Biliary atresia. Semin Pediatr Surg. 1992 May;1(2):115–124. [PubMed] [Google Scholar]
  28. Otte J. B., de Ville de Goyet J., Alberti D., Balladur P., de Hemptinne B. The concept and technique of the split liver in clinical transplantation. Surgery. 1990 Jun;107(6):605–612. [PubMed] [Google Scholar]
  29. Quinones-Baldrich W. J., Memsic L., Ramming K., Hiatt J., Busuttil R. W. Branch patch for arterialization of hepatic grafts. Surg Gynecol Obstet. 1986 May;162(5):488–490. [PubMed] [Google Scholar]
  30. Ryckman F., Fisher R., Pedersen S., Dittrich V., Heubi J., Farrell M., Balistreri W., Ziegler M. Improved survival in biliary atresia patients in the present era of liver transplantation. J Pediatr Surg. 1993 Mar;28(3):382–386. doi: 10.1016/0022-3468(93)90236-e. [DOI] [PubMed] [Google Scholar]
  31. STARZL T. E., MARCHIORO T. L., VONKAULLA K. N., HERMANN G., BRITTAIN R. S., WADDELL W. R. HOMOTRANSPLANTATION OF THE LIVER IN HUMANS. Surg Gynecol Obstet. 1963 Dec;117:659–676. [PMC free article] [PubMed] [Google Scholar]
  32. Samuel D., Gillet D., Castaing D., Reynes M., Bismuth H. Portal and arterial thrombosis in liver transplantation: a frequent event in severe rejection. Transplant Proc. 1989 Feb;21(1 Pt 2):2225–2227. [PubMed] [Google Scholar]
  33. Shaked A. A., Takiff H., Busuttil R. W. The use of the supraceliac aorta for hepatic arterial revascularization in transplantation of the liver. Surg Gynecol Obstet. 1991 Sep;173(3):198–202. [PubMed] [Google Scholar]
  34. Starzl T. E., Esquivel C., Gordon R., Todo S. Pediatric liver transplantation. Transplant Proc. 1987 Aug;19(4):3230–3235. [PMC free article] [PubMed] [Google Scholar]
  35. Starzl T. E., Groth C. G., Brettschneider L., Penn I., Fulginiti V. A., Moon J. B., Blanchard H., Martin A. J., Jr, Porter K. A. Orthotopic homotransplantation of the human liver. Ann Surg. 1968 Sep;168(3):392–415. doi: 10.1097/00000658-196809000-00009. [DOI] [PMC free article] [PubMed] [Google Scholar]
  36. Starzl T. E., Todo S., Fung J., Demetris A. J., Venkataramman R., Jain A. FK 506 for liver, kidney, and pancreas transplantation. Lancet. 1989 Oct 28;2(8670):1000–1004. doi: 10.1016/s0140-6736(89)91014-3. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. Tan K. C., Yandza T., de Hemptinne B., Clapuyt P., Claus D., Otte J. B. Hepatic artery thrombosis in pediatric liver transplantation. J Pediatr Surg. 1988 Oct;23(10):927–930. doi: 10.1016/s0022-3468(88)80387-7. [DOI] [PubMed] [Google Scholar]

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