paediatric liver transplant programme wits donald gordon ...paedhpb.org/2017/sunday/paediatric liver...
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Paediatric Liver Transplant Programme
Wits Donald Gordon Medical
Centre
J Loveland, J Botha, R Britz, B Strobele, S Rambarran, A Terblanche , C Kock, P Walabh,
M Beretta, M Duncan et al
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1817
1st clear English language reference to BA
Burns J. Principles of Midwifery, including the diseases of women and children 1817:602
John Burns Regius Professor of Surgery
University of Glasgow
“reveal the presence of”
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“Looking at the problem of biliary atresia from the vantage point of 30 years’ experience with the
lesion, we can say with certainty that the jaundiced baby who has had no extrahepatic bile duct has
been the most disappointing patient for the surgeon in the whole realm of lesions theoretically
correctable by a surgical procedure.”
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• Scientific fields progress in a linear and continuous way
• Laborious grind and dog work
• Predictable “solving of puzzles that are left open in a current field of
knowledge…..a pleasant way to keep busy when one is not up to useful work”
Grosfeld
Davenport
Coran Hind
Puri
Millar
Loveland
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The Structure of Scientific Revolutions
“Normal science with a paradigm and a dedication to solving puzzles; followed by
serious anomalies, which lead to a crisis; and finally resolution of the crisis by a new
paradigm”
Paradigm shift in treatment and outcomes
Ian Hacking Introductory Essay “The Structure of Scientific Revolutions”
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Kasai et al. Journal of Paediatric Surgery 1968 3 (6) 665 -675
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• Alluded to ductal diameter of 150 microns as potentially prognostic for drainage
• 10 of 14 with ducts > 200μ drained • Only 1 of 13 < 150μ
“Size may be of great significance”
• All cured cases surgery before 4 months of age
Kasai et al. Journal of Paediatric Surgery 1968 3 (6) 665 -675
“Not a few cases …. Might be curable if portoenterostomy carried out before 4 months of
age, preferably within 3 months after birth”
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The First
• 1963 C Henry Kempe presented Bennie Solis to Starzl
• Department of Paediatrics University of Colorado
• Infectious Diseases Specialist
• Coined term “Battered Child Syndrome”
• Defender of children
• Starzl suggested transplant – Kempe agreed • 1981 when moved to Pittsburgh, firmly supported moving
program forward
“The Puzzle People”
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• Complementary role of KP and LT now accepted standard treatment BA
• Sequential employment significantly improved prognosis
Otte et al. Hepatology 1994 20(Suppl):41S-48S
Vacanti et al. Journal of Pediatric Surgery 1990 25:149 - 152
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Loveland et al. S Afr Med J 2012;102(4):233-236
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SO WHERE ARE WE TODAY…
• 134 transplants • 8 re-transplants
• 36.57% LDLT (49/134)
• 34.33% whole (46/134)
• 18.66% splits (25/134)
• 10.45% Reduced size (14/134)
• 12 fulminant hepatic failure
• 12 CLKT
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UNIT GROWTH
0
5
10
15
20
25
30
20
05
20
06
20
07
20
08
20
09
20
10
20
11
20
12
20
13
20
14
20
15
20
16
20
17
(1
st 6
m)
Nu
mb
er
of
tran
spla
nts
Year of transplant
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DEMOGRAPHIC OF AETIOLOGY
0 10 20 30 40 50 60
biliary atresia
budd chiari - veno-occlusive disease
oxalisis
auto-immune disease
chronic rejection of liver graft
alpha-1 antitrypsin deficiency
alagille syndrome (biliary hypoplasia)
wilson's disease
polycystic kidney disease and hepatic fibrosis
biliary agenesis
hepatoblastoma
hepatocellular carcinoma
cryptogenic
maple syrup urine disease
PFIC - progressive familial intrahepatic cholestasis
other
% of recpients with chronic liver failure (n=117)
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IMMUNOSUPPRESSION
0
10
20
30
40
50
60
70
80
90
100
steroids tacrolimus MMF other
% o
f re
cip
ien
ts (
n=1
15
)
Immunosuppression at time of transplant
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Entire Series
134
Era 1
31
Era 2 103
Whole Grafts
46
18 (58%)
28 (27.18%)
Reduced Size Grafts
14
10 (32%)
4 (3.88%)
Split Grafts
25
3 (10%)
22 (21.6%)
Living Donor Grafts
49
0
49 (47.57%)
GRAFT UTILIZATION
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0 5 10 15 20 25 30
LDLT
Split
Reduced
Whole
Era 1: 2005 - 2012
Era 2: 2012 - Present
GRAFT TYPE BY ERA
Loveland et al. S Afr Med J 2014 104 (11) 799 - 802
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RECIPIENT AGE
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Entire Series
Era 1
Era 2
Age Range (months)
9 – 213
9 – 213
9 – 214
Mean Age
82.39
100.86
64.73
Median Age
50
101
45
Weight Range (kilograms)
5 – 62
5 – 62
6 – 45
Mean Weight
21
25.21
16.94
Median Weight
14.1
23
12.75
Loveland et al. S Afr Med J 2014 104 (11) 799 - 802
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RECIPIENT WEIGHT
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Entire Series
Era 1
Era 2
Age Range (months)
9 – 213
9 – 213
9 – 214
Mean Age
82.39
100.86
64.73
Median Age
50
101
45
Weight Range
(kilograms)
5 – 62
5 – 62
6 – 45
Mean Weight
21
25.21
16.94
Median Weight
14.1
23
12.75
Loveland et al. S Afr Med J 2014 104 (11) 799 - 802
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0 1 2 3 4 50
10
20
30
40
50
60
70
80
90
100
Time
Perc
en
t su
rviv
al
Patient Survival
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HEPATIC ARTERY THORMBOSIS
• 4.5% incidence (6/134)
• 1 Reduced = HAT Day 1 = Died before ReTx
• 1 Whole = ReTx = Died
• 1 Whole = Revised D1 = NAD
• 1 Whole = HAT Day 9 = ReTx Day 22 = NAD
• 1 Split = HAT Day 10 = ReTx Day 13 = Died
• 1 Whole = HAT Day 21 = Good function to date
• 1 Whole = Rupture D11 = Revised
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VASCULAR COMPLICATIONS
• 7 PVT and 1 PVS
– 1 PVT revised with venous interposition
– 1 PVS required angioplasty and stent
– 1 PVT required splenorenal shunt
• Another planned
• 2 patients venoplasty caval anastomosis
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BILIARY COMPLICATIONS
• Increased incidence after split and LDLT – Cut surface leaks
• Long term increase in strictures – Ischaemia
• Attention to detail: – LDLT harvest
– Back table split
– Biliary reconstruction during implant
– Meticulous arterial reconstruction
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LARGE FOR SIZE SYNDROME
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IMPACT OF LDLT
• Access greater organ pool – Significant ethical issues
• Benefits – Decreasing waiting list death – “elective” transplant – Well matched organ
• may lead to better long-term graft and patient survival
• Donor operation places well individual at risk:
– morbidity overall average 35%
• Decision made without coercion • Psychosocial evaluation • Anatomical assessment
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OF SIGNIFICANCE….
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THE IMPACT OF -3…..
0
10
20
30
40
50
60
70
80
90
100
Weight Height MUAC
% o
f p
atie
nts
age
d <
=5y
3
2
0
-1
-2
-3
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ANOTHER SIGNIFICANT HAZARD
• 3.5 (95% CI 1.2-10) for patient survival
• 3.2 (95% CI 1.2-8.4) for liver graft survival
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NOSOCOMIAL INFECTION
76.3%
16.6%
7.1%
Bacterial Viral Fungal / yeast
0
50
100
150
200
250
Nu
mb
er
of in
fectio
ns
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INTERVENTION – BILIARY ATRESIA
• Identification
• Early referral • Surgery
• Centralization
• Follow up
• Early referral • Transplant assessment
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INTERVENTION – TRANSPLANT • Nutritional support
• Pre-transplant
• Infection control
• Venous access and care
• Antibiotic stewardship
• Long Term outcomes • Immunosuppression
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