Author(s): Denis Devictor [*] 1 , Pierre Tissieres 2 3
Keywords
acute liver failure; biliary atresia; children; cholestatic diseases; immunosuppression; liver graft; liver transplantation; metabolic diseases; neonates; outcome; pediatrics; surgery; results
Pediatric liver transplantation (LT) is one of the most successful solid organ transplants [1-3] . It has become a well-established strategy in treating children with end-stage liver disease as well as children with irreversible acute liver failure (ALF), with excellent success and limited mortality. In most centers, the 1-year actuarial survival rate is higher than 90% in elective patients and higher than 70% in children with ALF [4] . Long-term survival is also excellent, more than 80% of children will survive to become teenagers and adults with excellent health-related quality of life [5-7] . Many aspects have contributed to improved survival in children post-LT, especially advancements in pre-, peri- and post-transplant management [8] . The development of surgical techniques, such as reduction hepatectomy, split-LT and the introduction of living related LT, has extended LT to infants under the age of 1 year and weighing less than 8 kg, which has effectively reduced the waiting list mortality from 25 to 5%. Nowadays, pediatric LT are routinely performed in all developed countries across the world [9] . Reports of experience from single centers provide are certainly encouraging, and the databases of the Studies in Pediatric Liver Transplantation (SPLIT group) and of the pediatric acute liver failure study group (PALF group) are also invaluable sources demonstrating these marked improvements in outcome [5,10-12] . For instance, the data of the SPLIT group allows analysis of currently more than 4000 North-American children who have undergone LT [13-16] . Similarly, the PALF group provides considerable data to improve our understanding on the treatment and outcome of children with ALF and to identify factors to predict need for LT in children with ALF [11] . This article will review the recent advances and will consider the future in pediatric LT.
Prior to the operation
This step includes the evaluation of indications and contraindications for LT, the selection, evaluation and preparation of the candidates and their prioritization.
What are the main indications for LT?
The main indications for LT in the pediatric population can be broadly separated in four groups: cholestatic liver diseases, ALF, metabolic liver disease, and liver tumors (Tables 1 & 2) [1,17] .
Table 1.
Indications for liver transplantation in children and outcomes.
Diagnosis Frequency (%) SPLIT registry [dagger] BicêtreYear of study
1995-2002
1986-2002
Patients (n)
1092
568
Number of transplantations
NA
648
Cholestatic liver disease
66
77
Biliary atresia
42
53
Others
14
34
Alagille syndrome
6
Sclerosing cholangitis
3.5
Progressive familial intrahepatic cholestasis
8
Alpha-1-antitrypsin deficiency
4.5
Acute liver failure
13
11
Metabolic diseases
12
9
Others
13
3
Liver graft survival
75 [double dagger]
65 §
Patient survival
69 [double dagger]
83 §
[dagger]
Data taken from [3] .
[double dagger]
15-year outcome.
§
3-year outcome.
NA: Not available; SPLIT: Studies of Pediatric Liver Transplantation.
Table 2.
Patient characteristics of 2982 children who underwent a first liver transplantation registered in SPLIT from 1995 to 2008.
Characteristics Patients (N = 2982) n % Age at transplantMissing
1
0
0-6 mo
260
8.7
6-12 mo
725
24.3
1-5 y
962
32.3
5-13 y
616
20.7
13+ y
418
14
Race
Missing
47
1.6
White
1668
56.3
Black
464
15.6
Hispanic
494
16.6
Other
299
10
Sex...