Evaluation of the Pediatric Patient for Liver Transplantation

PREAMBLE Current American Association for the Study of Liver Diseases (AASLD) liver transplant evaluation guidelines include both adult and pediatric patients (1). While pediatric liver transplants account for ∼7.8% of all liver transplants in the United States, sufficient differences between pediatric and adult patients seeking liver transplantation (LT) now require independent, yet complementary documents. This document will focus on pediatric issues at each level of the evaluation process. Disease categories suitable for referral to a pediatric LT program are similar to adults: acute liver failure (ALF), autoimmune, cholestasis, metabolic or genetic, oncologic, vascular, and infectious; however, specific etiologies and outcomes differ widely from adult patients, justifying independent pediatric guidelines. Data supporting our recommendations are based on a MEDLINE search of the English language literature from 1997 to the present. Intended for use by physicians, these recommendations suggest preferred approaches to the diagnostic, therapeutic, and preventive aspects of care. They are intended to be flexible, in contrast to standards of care, which are inflexible policies to be followed in every case. Specific recommendations are based on relevant published information. To more fully characterize the available evidence supporting the recommendations, the AASLD Practice Guidelines Committee has adopted the classification used by the Grading of Recommendation Assessment, Development, and Evaluation (GRADE) workgroup with minor modifications (Table 1). The classifications and recommendations are based on 3 categories: the source of evidence in levels I through III; the quality of evidence designated by high (A), moderate (B), or low quality (C); and the strength of recommendations classified as strong or weak.TABLE 1: Grading of Recommendations, Assessment, Development and Evaluation (GRADE)LITERATURE REVIEW METHODS AND ANALYSIS Each association appointed at least 1 author to serve on the writing group. The chair of the writing group was appointed by the AASLD. Members of the writing group were not compensated for their work and served as volunteers throughout the process from concept design through final publication. Writing group members had no financial conflict of interest or financial relationship with commercial entities relevant to the article. Topics relevant to liver transplant evaluation in the pediatric patients were identified through a conference call with all members of the writing group on July 11, 2012 and assignments were distributed among the members based on their particular expertise and interest. The literature databases and the search strategies are outlined below. The resulting literature database was available to all members of the writing group. They selected references within their field of expertise and experience and graded the references according to the GRADE system. Data supporting our recommendations are based on a MEDLINE search of the English-language literature from 1973 to the present. Primary search terms included: liver transplant evaluation, liver transplant, child, pediatric, and liver transplant outcome. In addition, each assessment (eg, anesthesia, hepatology, renal); diagnosis (eg, biliary atresia, organic acidemia, maple syrup urine disease [MSUD], ductal plate malformation) and complication (eg, hepatopulmonary syndrome [HPS], malignancy) was searched in the context of the primary search terms as well as individually when relevant clinical background information was needed. The selection of references for the guideline was based on a validation of the appropriateness of the study design for the stated purpose, a relevant number of patients under study, and confidence in the participating centers and authors. References on original data were preferred and those that were found unsatisfactory in any of these respects were excluded from further evaluation. There may be limitations in this approach when recommendations are needed on rare problems or problems on which scant original data are available. In such cases it may be necessary to rely on less qualified references with a low grading. PEDIATRIC LIVER TRANSPLANT EVALUATION TEAM Children have distinct diseases, clinical susceptibilities, physiological responses, as well as neurocognitive and neurodevelopmental features that distinguish them from adults. In fact, even within the pediatric age group differences can be found between newborns, infants, children, and adolescents. Given the intraabdominal anatomical variations associated with biliary atresia, the most common indication for pediatric LT, as well as the restricted abdominal cavity and small size of blood vessels in infants and young children, surgical teams with exhaustive pediatric experience will benefit the pediatric recipient of an LT. Members of the pediatric LT team (Table 2) use their expertise to tailor the LT evaluation plan (Table 3) to the unique needs of the child. The end product of the evaluation will ensure the elements for an informed decision to proceed to LT are met (2).TABLE 2: Potential members of the pediatric liver transplant teamTABLE 3: Components of the pediatric liver transplantation evaluationRecommendation: A multidisciplinary pediatric LT evaluation team should be skilled in pediatric conditions and properly communicate with the family and the child, when appropriate, the processes, risks, and benefits associated with LT. (2-B) TIMING OF REFERRAL FOR PEDIATRIC LIVER TRANSPLANT EVALUATION Based on the US Organ Procurement and Transplantation Network (OTPN) from January 1, 2011 through May 31, 2013, indications for LT include biliary atresia (32%), metabolic/genetic conditions (22%), acute liver failure (11%), cirrhosis (9%), liver tumor (9%), immune-mediated liver and biliary injury (4%), and other miscellaneous conditions (13%) (Fig. 1). Within these broad categories rest many rare conditions with myriad presentations.FIGURE 1: Indications for pediatric liver transplant.Because timing for referral varies depending on the child's clinical circumstances, referral for LT may be emergent, urgent, or anticipatory. ALF or an acute decompensation of an established liver disease may have a rapid and unpredictable course progressing to death or irreversible neurological damage (3). Children with metabolic liver such as or can experience neurological as a of metabolic Primary and liver are rare in children, with and the most for with on to and surgical for an or a for of with biliary atresia to with their liver the 3 to for those with a with syndrome and 1, and can the child's quality of liver associated with liver disease at for with a pediatric LT should be for with ALF or acute decompensation of an established liver referral for LT evaluation may be Children with metabolic to surgical or evidence of should be for LT evaluation. atresia patients are should be for LT evaluation the 3 from liver transplant evaluation should be in patients between and for LT evaluation should be for with liver disease and evidence of liver by or of LIVER TRANSPLANT EVALUATION and The child's evaluation as it to primary associated and strategies should be and by the primary pediatric for of the child's liver should include clinical of and and surgical and as well as and of the between a of the LT evaluation team and the child's will and that may not be in the or may be identified the LT evaluation A of the by the LT team the LT evaluation will the evaluation and of the primary assessment of and to LT. 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