Outcomes of Liver Transplant for Biliary Atresia: A Ten Year Single Centre Experience

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1 Outcomes of Liver Transplant for Biliary Atresia: A Ten Year Single Centre Experience Dr Yentl van Heerden; Dr Andrew Grieve, Professor Jerome Loveland For Paediatric Hepatobiliary and Liver Transplant Symposium University of the Witwatersrand November 2017

2 Introduction Biliary Atresia is the most common indication for liver transplantation in the paediatric population % of patients require liver transplant despite Kasai-portoenterostomy 2,3 Paradox: Earlier on in life (smaller patients, younger patients) with more severe grade of liver dysfunction 1.Esquivel C.O.; Iwatsuki S. et al. (1987) Indications for pediatric liver transplantation. Pediatrics. 111(6): Laurent J, Gauthier F, Bernard O, Hadchouel M, Odivre M, Alagille D (1990) Gastroenterology 99: 1793±179 3.Lopez-Santamaria M, Gamez M, Murcia J, Diez-Pardo J,Diaz M, Leal N, et al. Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy.the importance of sequential treatment.pediatr Surg Int 1998;13:

3 Introduction Hepatotropic viruses: CMV, EBV, Reo virus demonstrate poorer outcomes Complications: conflicting reports on the complication rates when comparing transplant for biliary atresia vs transplant for other indications Enteric complications universally higher in biliary atresia group. Overall survival: Seem to be slightly worse in biliary atresia group, but not statistically significant. Cause and timing of death: No correlation between centres.

4 Introduction Factors associated with poorer survival outcomes (generally) PELD score MUAC/Weight of patients Era of transplantation Experience of centre Type of graft used BASM/Splenic malformation

5 Method Retrospective Record Review WDGPLTU: Under 18 years First liver transplant Excluded: Fulminant Hepatic Failure Biliary Atresia vs Other Indications Survival Outcomes Factors affecting outcomes Complications Enteric Biliary Vascular

6 Data collected Age Gender Race Diagnosis PELD score Previous surgical history (KPE) Weight at transplant Operation notes (BASM/splenic malformation) Complications and treatment of complications

7 Data collected Sources Patient records Lab reports Existing database Redcap (WDGPLTU) Ethics (07/09/2017) M HREC Wits

8 Aims and Objectives Overall Aim To determine if there is a difference between outcomes in patients who have undergone liver transplant for biliary atresia, compared to patients who have undergone liver transplant for other indications. Secondary Aim To ascertain the factors which determine these outcomes

9 Aims and Objectives Objectives To analyse 1 year survival outcomes for patients undergoing liver transplant for biliary atresia vs patients undergoing liver transplant for other indications To evaluate the post-operative complications in the group transplanted for biliary atresia vs other To distinguish the factors affecting these outcomes

10 Demographics Overall 101 first time transplants 67% female 55% black, 35% white, 10% coloured and indian Mean Weight: 18.7kg Mean Age:5.5y Median follow up time: 1.4y Biliary atresia 52 first time transplants 67% female 63% black, 29% white, 8% other Mean weight: 11.2kg Mean Age: 2.7y

11 Indications % of recpients (n=101) biliary atresia budd chiari - veno-occlusive disease auto-immune disease oxalosis alpha-1 antitrypsin deficiency polycystic kidney disease and hepatic fibrosis alagille syndrome (biliary hypoplasia) wilson's disease biliary agenesis hepatoblastoma hepatocellular carcinoma cryptogenic maple syrup urine disease PFIC - progressive familial intrahepatic cholestasis other

12 Overall Survival

13 Graft Survival

14 Complications Complication General Biliary Atresia Enteric 7% 20% Biliary 30% 36% Vascular 12% 10%

15 Suspected Factors associated with poorer outcomes (interim) Age Gender Weight (z-score) Mid Upper Arm Circumference (z-score) Graft type used Transplant era Previous Kasai-Portoenterostomy PELD Score Pre-transplant portal vein thrombosis Pre-transplant portal vein hypoplasia BASM/Splenic malformation

16 Causes of Death % of recpients in each group Infection Graft failure Cardiopulmonary Multi-organ failure PTLD Recurrent tumour Haemmorrhage Other Biliary atresia Other

17 Number of transplants Year of transplant

18 Conclusions No statistical difference in 1 year survival outcomes between the two groups Increase in enteric complications in group transplanted for biliary atresia -2/-3 Z-score for weight is a proven prognostic factor for poorer overall 1 year survival outcomes Living related donor grafts have better graft survival outcomes at 1 year The programme continues to gain momentum

19 Thank you