Chapter 67: Pediatric Liver Transplantation

INTRODUCTION

• Pediatric transplants account for approximately 8% of all liver transplants in the United States.

• In 2014 the American Association for the Study of Liver Diseases (AASLD); the American Society of Transplantation; and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition published their evidence-based recommendations for pediatric liver transplantation diagnostic, therapeutic, and preventive care.

• This chapter provides brief summary of these recommendations based on that publication. For further details, please refer to the Further Reading section.

TRANSPLANT EVALUATION TEAM

• Pediatric transplant candidates should be evaluated by a skilled multidisciplinary team. Risks, benefits, and the process from preoperative evaluation to long-term follow up should be reviewed with the patient (when appropriate) and the family/caregivers in order to determine the candidacy and whether to proceed or not based on an informed decision.

TIMING OF REFERRAL FOR TRANSPLANTATION

• Immediate referral:

  • Acute liver failure

  • Acute decompensation of preexisting liver disease

• Urgent referral:

  • Liver-based metabolic crises nonresponsive to medical therapy

  • Hepatoblastoma or hepatocellular carcinoma not amenable to surgical resection

• Prompt referral:

  • Biliary atresia with total bilirubin >6 mg/dL >3 months after Kasai procedure (hepatoportoenterostomy [HPE])

  • Biliary atresia with total bilirubin 2 to 6 mg/dL

• Anticipated referral:

  • Deteriorating chronic liver disease

    • Failure to thrive

    • Development of ascites

    • Encephalopathy (may present as low school performance, forgetfulness)

EVALUATION AND PRETRANSPLANT MANAGEMENT

• Review available records and documents

• Confirm referring diagnosis, comorbidities, and other factors of relevance to the transplantation process (including potential surgical challenges)

• Manage and optimize conditions in collaboration with the local medical team

• Develop a pre- and posttransplant assessment and treat-ment plan

  • Must address potential recurrence of the primary disease (such as autoimmune hepatitis, primary sclerosing cholangitis [PSC], bile salt excretory pump disease), allograft failure, onset of new diagnoses (such as renal dysfunction associated with chronic immunosuppression), and follow-up evaluations (such as irritable bowel syndrome [IBD], cancer screening, infections, compliance)

• Ascites management:

• Diuretics

  • Aldosterone agonist

  • Furosemide

• Paracentesis

• Transjugular intrahepatic portosystemic shunt (TIPS)/other shunting process

• Abdominal evaluation for surgery

  • Duplex ultrasound of the liver, spleen, portal vein, and its branches

  • Special consideration for biliary atresia with splenosis symptoms

    • Interrupted vena cava

    • Preduodenal portal vein

    • Aplastic portal vein

    • Splenosis

• Nutritional assessment and optimization:

  • Children with chronic liver disease require up to 80% more calories for adequate growth

  • Optimized nutrition is associated with improved recipient, graft, and neurodevelopmental outcomes

• Cardiopulmonary assessment and optimization:

  • Evaluation for hepatopulmonary syndrome and portopulmonary hypertension

  • Transcutaneous oxygen saturation measurement in cases of suspected portosystemic shunting

  • Cardiac Doppler echocardiogram to rule out pulmonary hypertension

    • Right-sided cardiac catheterization if right ventricular systolic pressure >50 mm Hg

  • Pulmonary function tests in instances of cystic fibrosis

• Renal assessment and optimization:

  • Creatinine

  • Glomerular filtration rate (GFR)

    • Cystatin C

    • Revised Schwartz formula:

      • 0.413 3 [sCr (mg/dL) / height (cm)] = GFR(mL/min/1.73 m²)

  • Acute renal injury

    • Modified risk for renal dysfunction, injury, failure, loss, and end-stage ...