liver transplant for biliary atresia: a ten year single ... of liver transplant for... ·...

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

10-12 November 2017

Introduction

• Biliary Atresia is the most common indication for liver transplantation in the paediatric population1

• 60-80% of patients require liver transplant despite Kasai-portoenterostomy2,3

• 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): 1039-1045

2.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;13:327-330.

University of Witwatersrand – Wits Donald Gordon Medical Centre

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.


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


Method

• Retrospective Record Review

• WDGPLTU: 2005 – 2016

• Under 18 years

• First liver transplant

• Excluded: Fulminant Hepatic Failure

• Biliary Atresia vs Other Indications • Survival Outcomes

• Factors affecting outcomes

• Complications • Enteric

• Biliary

• Vascular


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


Data collected

• Sources • Patient records

• Lab reports

• Existing database • Redcap (WDGPLTU)

• Ethics (07/09/2017) M170752 HREC Wits


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


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


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


0 10 20 30 40 50 60

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

% of recpients (n=101)

Indications

Overall Survival

Graft Survival

Complications

Complication

General

Biliary Atresia

Enteric 7% 20%

Biliary 30% 36%

Vascular 12% 10%


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


Causes of Death

0 10 20 30 40 50 60 70

Infection

Graft failure

Cardiopulmonary

Multi-organ failure

PTLD

Recurrent tumour

Haemmorrhage

Other

% of recpients in each group

Biliary atresia Other

0

5

10

15

20

25

20

05

20

06

20

07

20

08

20

09

20

10

20

11

20

12

20

13

20

14

20

15

20

16

Nu

mb

er

of

tran

spla

nts

Year of transplant

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

Thank you

liver transplant for biliary atresia: a ten year single ... of liver transplant for... ·...

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