Abstract
Objective
To study the clinical, biochemical profile and outcome of patients with biliary atresia (BA) who underwent living related liver transplantation (LRLT) at authors’ institute in the last 5 y (2008–2013).
Methods
Case records of the 20 patients diagnosed with biliary atresia who had undergone living related liver transplantation at authors’ centre in the last 5 y were analysed.
Results
Eighteen patients with BA with a failed Kasai procedure and 2 without a prior Kasai's portoenterostomy received a liver transplant. At a median follow up of 2 y and 6 mo, both the patient and graft survival rates were 90 %. The median age of the recipients at the time of LRLT was 8 mo and 12 (60 %) of the transplanted children were less than or equal to 1 y of age. The male–female ratio was 1.8:1. The median weight was 7.3 kg (5.8–48 kg); two thirds were less than 10 kg. The median pre-transplant total serum bilirubin (TSB) and international normalized ratio (INR) were 12.98 (0.5–48.3) mg/dl and 1.3 (1.0–3.9) respectively. All patients received a living related graft and there was no donor mortality. The median duration of postoperative ventilation was 14 h. The post-operative complications were infection (30 %), vascular complications (20 %) and acute rejection (20 %). The median duration of postoperative hospital stay was 21 d (17–42). Two patients died of combined hepatic and portal vein thrombosis in the early postoperative period. Late rejection was encountered in 1 patient and another developed chronic kidney disease necessitating a renal transplant. There were no late vascular occlusions or development of post transplant lymphoproliferative disease.
Conclusions
Thus, LRLT for BA with or without a prior portoenterostomy, is a feasible and successful treatment modality with good outcomes attained despite the challenges of age and size. This treatment modality is now well established in India.
Similar content being viewed by others
References
Kasai M, Mochizuki I, Ohkohchi N, Chiba T, Ohi R. Surgical limitation for biliary atresia: indication for liver transplantation. J Pediatr Surg. 1989;24:851–4.
Mizuta K, Sanada Y, Wakiya T, Urahashi T, Umehara M, Egami S, et al. Living-donor liver transplantation in 126 patients with biliary atresia: single-center experience. Transplant Proc. 2010;42:4127–31.
Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr. 2003;37:4–21.
Ohi R. Biliary atresia. A surgical perspective. Clin Liver Dis. 2000;4:779–804.
Schweizer P. Treatment of extrahepatic bile duct atresia: results and long-term prognosis after hepatic portoenterostomy. Pediatr Surg Int. 1986;1:30–6.
Cacciarelli TV, Esquivel CO, Moore DH, Cox KL, Berquist WE, Concepcion W, et al. Factors affecting survival after orthotopic liver transplantation in infants. Transplantation. 1997;64:242–8.
Ganschow R, Nolkemper D, Helmke K, Harps E, Commentz JC, Broering DC, et al. Intensive care management after pediatric liver transplantation: a single-center experience. Pediatr Transplant. 2000;4:273–9.
Sundaram SS, Alonso EM, Whitington PF. Liver transplantation in neonates. Liver Transpl. 2003;9:783–8.
Woodle ES, Millis JM, So SK, McDiarmid SV, Busuttil RW, Esquivel CO, et al. Liver transplantation in the first three months of life. Transplantation. 1998;66:606–9.
Poonacha P, Sibal A, Soin AS, Rajashekar MR, Rajakumari DV. India's first successful pediatric liver transplant. Indian Pediatr. 2001;38:287–91.
Sibal A, Bhatia V, Gupta S. Fifteen years of liver transplantation in India. Indian Pediatr. 2013;50:999–1000.
Kaur S, Wadhwa N, Sibal A, Jerath N, Sasturkar S. Outcome of live donor liver transplantation in Indian children with bodyweight 7.5 kg. Indian Pediatr. 2011;48:51–4.
Zhu JJ, Xia Q, Zhang JJ, Xue F, Chen XS, Li QG, et al. Living donor liver transplantation in 43 children with biliary atresia: a single-center experience from the mainland of China. Hepatobiliary Pancreat Dis Int. 2012;11:250–5.
Chardot C, Buet C, Serinet MO, Golmard JL, Lachaux A, Roquelaure B, et al. Improving outcomes of biliary atresia: French national series 1986–2009. J Hepatol. 2013;58:1209–17.
Rao S, D'Cruz AL, Aggarwal R, Chandrashekar S, Chetan G, Gopalakrishnan G, et al. Pediatric liver transplantation: a report from a pediatric surgical unit. J Indian Assoc Pediatr Surg. 2011;16:2–7.
Mizuta K, Urahashi T, Ihara Y, Sanada Y, Wakiya T, Yamada N, et al. Living donor liver transplantation in children with cholestatic liver disease: a single-center experience. Transplant Proc. 2012;44:469–72.
Sun LY, Yang YS, Zhu ZJ, Gao W, Wei L, Sun XY, et al. Outcomes in children with biliary atresia following liver transplantation. Hepatobiliary Pancreat Dis Int. 2013;12:143–8.
Tannuri AC, Gibelli NE, Ricardi LR, Silva MM, Santos MM, Pinho-Apezzato ML, et al. Orthotopic liver transplantation in biliary atresia: a single-center experience. Transplant Proc. 2011;43:181–3.
Chen CL, Concejero A, Wang CC, Wang SH, Lin CC, Liu YW, et al. Living donor liver transplantation for biliary atresia: a single-center experience with first 100 cases. Am J Transplant. 2006;6:2672–9.
Karakayali H, Sevmis S, Ozcelik U, Ozcay F, Moray G, Torgay A, et al. Liver transplantation for biliary atresia. Transplant Proc. 2008;40:231–3.
Wang Q, Yan LN, Zhang MM, Wang WT, Zhao JC, Pu CL, et al. The pre-Kasai procedure in living donor liver transplantation for children with biliary atresia. Hepatobiliary Pancreat Dis Int. 2013;12:47–53.
Alexopoulos SP, Merrill M, Kin C, Matsuoka L, Dorey F, Concepcion W, et al. The impact of hepatic portoenterostomy on liver transplantation for the treatment of biliary atresia: early failure adversely affects outcome. Pediatr Transplant. 2012;16:373–8.
Gallo A, Esquivel CO. Current options for management of biliary atresia. Pediatr Transplant. 2013;17:95–8.
Fishman JA, Rubin RH. Infection in organ-transplant recipients. N Engl J Med. 1998;338:1741–51.
Kim JE, Oh SH, Kim KM, Choi BH, Kim DY, Cho HR, et al. Infections after living donor liver transplantation in children. J Korean Med Sci. 2010;25:527–31.
Deng YH, Guo CB, Zhang MM, Li YC. [Postoperative intensive care of biliary atresia patients treated with living donor liver transplantation]. Zhonghua Er Ke Za Zhi. 2011;49:21–6.
Ryckman F, Fisher R, Pedersen S, Dittrich V, Heubi J, Farrell M, et al. Improved survival in biliary atresia patients in the present era of liver transplantation. J Pediatr Surg. 1993;28:382–5.
Langnas AN, Marujo W, Stratta RJ, Wood RP, Shaw Jr BW. Vascular complications after orthotopic liver transplantation. Am J Surg. 1991;161:76–82.
Hori T, Kaido T, Oike F, Ogura Y, Ogawa K, Yonekawa Y, et al. Thrombotic microangiopathy-like disorder after living-donor liver transplantation: a single-center experience in Japan. World J Gastroenterol. 2011;17:1848–57.
McDiarmid SV. Liver transplantation. The pediatric challenge. Clin Liver Dis. 2000;4:879–927.
Shneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr. 2006;148:467–74.
Bhatia V, Sibal A. Are fathers catching up with mothers in liver donation? Indian Pediatr. 2013;50:158.
Shneider BL, Mazariegos GV. Biliary atresia: a transplant perspective. Liver Transpl. 2007;13:1482–95.
Raghunathan A. An abdominal affair 2012 [19-07-2013]. Available from: http://week.manoramaonline.com/cgi-bin/MMOnline.dll/portal/ep/theWeekContent.do?programId=1073755753&contentId=13007946&tabId=13. Accessed on 1 Oct 2014.
Contributions
SM: Wrote first manuscript, patient care, data collection; AS, VB, AK, SG: Patient care, edited the manuscript; SS, SG, NJ, MW: Patient care. AS will act as guarantor for this paper.
Conflict of Interest
None.
Source of Funding
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Malhotra, S., Sibal, A., Bhatia, V. et al. Living Related Liver Transplantation for Biliary Atresia in the Last 5 years: Experience from the First Liver Transplant Program in India. Indian J Pediatr 82, 884–889 (2015). https://doi.org/10.1007/s12098-014-1687-x
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-014-1687-x