39ème Congrès | Groupe Francophone d'Hépatologie-Gastroentérologie et Nutrition Pédiatriques

D I J O N 2 9 – 3 1 M A R S 2 0 1 8

ATRESIE DES VOIES BILIAIRES:

le choix des donneurs en cas de greffe

Dr Roberto TAMBUCCI

Unité de Chirurgie et Transplantation Pédiatriques

Cliniques Universitaires Saint-Luc

Université catholique de Louvain

B R U X E L L E S

BILIARY ATRESIA (BA) is

the most common indication for pediatric liver transplantation

RH SQUIRES et al, HEPATOLOGY, July 2014

At least 80% of patients with BA

are transplanted by 20 years of age,

with the majority transplanted

under 4 years of age.

Indications to LT for BA patients

- ongoing cholestasis, decompensated biliary cirrhosis

- cholangitis,

- portal hypertension with or without variceal hemorrhage,

- poor weight gain,

- fat soluble vitamin deficiencies,

- hepatopulmonary syndrome,

- porto-pulmonary hypertension,

- and rarely hepatocellular carcinoma.

Rapidly

downhill !!!

1000 liver transplantations in 882 children …

1993 - 2015

(since the beginning of our living-donor liver transplantation program)

342 BA patients

out of 609 patients (56,1%)

median age IQR range

Biliary Atresia 342 patients 0,97 yy 0.73-1.89 0.32-15.69

Others 267patients 3.89 yy 1.78-8.13 0.09-22.39

TOT 609 patients

p < 0.0001

Median age at LT

(CUSL data)

77.2% of BA patients : LT before 2 years of age

small child

requires

small graft

Types of liver grafts

1. Whole organ (1963)

Types of liver grafts

2. Reduced size (1984)

Types of liver grafts

3. Split-liver (1988)

Types of liver grafts

4. Living related grafts (1990)

Types of liver grafts

More recent frontiers:

Monosegmental grafts

Types of liver grafts

More recent frontiers:

Donation After Cardiac Death (DCD)

Indications in children???

Size of grafts

How do you determine the appropriate parenchymal mass for adequate function?

• Estimated liver volume

LV (mL) = 706.2 × BSA (m

) + 2.4

LV (mL) = 2.223 × BW (kg)

0.426

× height (cm)

0.682

• Graft to Recipient Weight Ratio (GRWR)

1-3% is optimum

< 0.7% survival will suffer-insufficient liver mass

Size of grafts

Practical Application

• Whole organ – Donor weight 1/3 above or below that of recipient is

appropriate

• Left Latetal Segments = donor/recipient wt ~10:1

• Left lobe = donor/recipient wt ~5:1

• Right Lobe = donor/recipient wt ~1:1

Size of grafts

Recipient Donor

(whole liver)

Donor

(reduced/split)

Segments

(reduced/split)

8kg, without ascites 6-10 kg 40-60 kg II+III

8kg, with ascites 6-15 kg 40-70 kg II+III

20 kg 15-25 kg 40-50 kg

50-70 kg

II+III+IV

II+III

30 kg 25-35 kg 50-80 kg II+III+IV

40 kg 30-50 kg 60-90 kg II+III+IV

Our experience

1. Organ allocation in EuroTranplant:

- Almost no facilitation for pediatric patients

(except: donor < 16 years)

- No facilitation for split-grafts

2. >90% from abroad:

- Not eligible for deceased donor

Our experience

Implementation of

Living Donor Liver Transplantation program

Our experience

2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 2016 2017

Post-mortem donors

Living donors

Living donor liver transplantation for children:

Which added value ?

• For the liver recipient ?

• For the living donor ?

• For the institution ?

• For the community ?

• In a global perspective ?

Living donor liver transplantation for children:

• Alleviates cadaveric organ donor shortage

• Reduces pre-transplant mortality

• Allows nutritional preparation of LT candidates

• Improves post-transplant survival (biliary atresia)

• Allows timely LT in oncological indications

Living donor liver transplantation for children:

Ethical principles of living donation

•Risks (D) and benefits (R)

•Selection of the living donor

•Informed consent

Living donor liver transplantation for children:

Need

Recipient

outcome

Risk to the

living donor

Equipoise

Risks for the living donor

Safety first ….

Quality improvement in living liver donation

(NY State Committee, Dec 2002)

« The well being of the donor should be a primary

consideration of any live donor organ transplantation »

World mortality in living donors

of a left liver lobe:

0,1%

Our series (CUSL, first 350 living donor)

• Mortality: 0%

• Morbidity:

* Blood bank transfusion: 1-2%

* Post-operative pain: 82% !

* Biliary fistula: 6%

* Incisional hernia: 3%

* Pleural effusion: 2%

* Nerve compression: 1%

* Persisting disabibility: 0%

• Hospitalization: 4-11 days

Informed consent in living donation

1st Informed

consent

Living

donation

2nd Informed

consent

Medical/psychosocial

work-up

Medical advocate

Pre-operative work-up in living donor candidates

• Parental relationship with the recipient

• Medical history and physical examination

• Body mass index < 27, no liver steatosis

• Biochemistry (thromphilic work-up)

• Pre-operative work-up (ECG, Thorax Xray, Spirometry,

Anesthesiology)

• Liver imagery (echography, MRI)

• Psychological assessment

• INDEPENDENT MEDICAL ADVOCATE

Benefits for the donor

Emotional consequences

of living donation must be better understood:

•Positive consequences

•Negative consequences ?

Our results in BA recipients

p < 0.0001

BA vs other recipients results (CUSL 1993-2015)

(patient survival)

0 5 10 15 20 25

75%

80%

85%

90%

95%

100%

TIME (years)

<2y - Living D

<2y - Deceased D

>2y - Living D

>2y - Deceased D

0 5 10 15 20 25

75%

80%

85%

90%

95%

100%

TIME (years)

<2y - Living D

<2y - Deceased D

>2y - Living D

>2y - Deceased D

0 5 10 15 20 25

75%

80%

85%

90%

95%

100%

TIME (years)

<2y - Living D

<2y - Deceased D

>2y - Living D

>2y - Deceased D

OVERALL

PATIENT SURVIVAL

OVERALL

GRAFT SURVIVAL

p 0,1607 Overall

p 0,0156 < 2 yy DD vs LD

p 0,8099 > 2 yy DD vs LD

p 0,0008

p 0,0001

p 0,6724

BA recipients results (CUSL 1993-2015)

p=0.041

p=0.512

n=79

n=48

n=27

n=51

Maternal donation

Paternal donation

Maternal donation

Paternal donation

Maternal vs Paternal donation

Take home messages

BA is the most common indication for pediatric liver transplantation

Patients are often transplanted < 2 years of age

Their clinical status could get worse wickly, waiting for an organ

Living donor liver transplantation (LDLT)

is a good and safe alternative to deceased donor

In our series, best results are obteined

when LDLT is performed before the age of 2 years

Merci!

roberto.tambucci@uclouvain.be

raymond.reding@uclouvain.be