eMedicine Specialties > Pediatrics: Surgery > Transplantation
Liver Transplantation
Updated: Oct 2, 2007
Introduction
Liver transplantation is a treatment, used in appropriately selected patients, for acute and chronic liver failure due to any cause. It is not indicated if an acceptable alternative is available or if contraindications, such as some cases of malignancy, terminal conditions, or poor expected quality of outcome, are present.
History of the Procedure
Starzl performed the first human liver transplant in 1963.1 Since then, the evolution of immunosuppression and newer surgical approaches has led to the establishment of 100 transplant centers in the United States. Surgeons currently perform more than 500 pediatric transplantations per year.
Problem
When a pediatric patient is likely to require a liver transplant, the medical management is generally divided into pretransplant and posttransplant periods, with the posttransplant period further separated into early and late time frames.
Pretransplantation care needs to take into consideration potentially prolonged waiting periods and to project far in advance when transplantation might be required. By initiating the pretransplant workup early, one can work toward maximizing the nutritional status, which is a factor impacting both pretransplant and posttransplant outcomes, especially in the pediatric population, because of an increased incidence of cholestatic liver diseases. Cholestatic liver diseases lead to fat malabsorption, which causes a deficiency of calories as well as fat-soluble vitamins.2
Pediatric patients can greatly benefit from caloric assessments and supplemental tube feedings as indicated. Furthermore, parenteral feedings are sometimes warranted in the most nutritionally deprived patients with end-stage liver disease. The optimization of nutritional status in pediatric patients has translated into improved survival after transplantation, fewer infections, and a reduction of surgical complications.3
Frequency
Pediatric patients account for about 12.5% of liver transplant recipients.
Etiology
Patients with biliary atresia comprise 50% of the pediatric patients who require a liver transplant. Other disease states that progress to end-stage liver disease in pediatric patients include metabolic disorders and progressive intrahepatic cholestasis. Examples of metabolic derangements include Wilson disease, alpha 1-antitrypsin deficiency, tyrosinemia, and hemochromatosis. Other metabolic disease states leading to hepatic dysfunction include Crigler-Najjar syndrome, glycogenosis, hyperoxaluria, metabolic respiratory chain deficiencies, familial hypercholesterolemia, and methylmalonyl aciduria.4
Pathophysiology
In children and adults, the liver is the largest solid organ in the body and is responsible for supplying energy during fasting, detoxifying drugs and metabolites, bile production, and supplying various different proteins, including albumin and prothrombin. Approximately 20% of the cardiac output passes through this organ. Failure develops with loss of more than 80-90% of hepatic function.5
Compared with adults, who may suffer from recurrence of their primary illness leading to hepatic dysfunction in their transplanted grafts, children largely do not experience recurrence. This fact is reflected in the overall better allograft survival rates in the pediatric population. Unfortunately, the number of children with liver deficiencies necessitating liver transplantation far exceeds the availability through donation. The mortality rate for children on the United Network for Organ Sharing (UNOS) waiting list is estimated at 17%.6
The stratification of deceased organ donation was formulated by UNOS. This system uses a risk determination based on a 3-month pretransplant assessment risk profile to assign priority and organ allocation to the most severely ill patients. Patients who qualify for liver transplantation are assigned a score based on a nation-wide ranking system. This system is called the Model for End-Stage Liver Disease (MELD) for adults and the Pediatric End-Stage Liver Disease (PELD) for patients younger than 12 years. The MELD score uses a mathematical formula based on a patient's creatinine level, international normalized ratio (INR) (which measures blood clotting time), and bilirubin. The PELD score differs in that its calculation also includes albumin, growth failure, and the patient's age when first placed on the waiting list and does not include the creatinine level.6
Liver transplants have been successfully extended to neonates.7 Neonatal hemochromatosis has been found to be the primary cause of acute liver failure in the neonatal population, leading to a histologic diagnosis of giant-cell hepatitis. Although neonates appear to be more immunotolerant to transplanted organs, their immature immune systems combined with immunosuppression increases the risk for infectious complications. Among neonatal transplant recipients, vascular thrombosis is the major complication. When present, survival is dramatically reduced. Because of size discrepancies between the recipient and the donor pool, partial liver grafts are usually used for this population of patients.8
Presentation
Major clinical features of hepatic failure include the following:9
- Jaundice
- Hypoalbuminemia
- Coagulopathy
- Disseminated intravascular coagulation
- Hyperammonemia
- Fetor hepaticus
- Increased serum levels of hepatic enzymes
- Spider angiomas
- Hepatic encephalopathy
- Hepatorenal syndrome
- Coma
Indications
About 50% of the pediatric patients who require a liver transplant have biliary atresia. Other disease states that progress to end-stage liver disease among pediatric patients and require liver transplantation include metabolic disorders and progressive intrahepatic cholestasis.
Relevant Anatomy
The liver is located in the right upper portion of the abdominal cavity, just beneath the diaphragm, and is protected by the rib cage. It sits on top of the stomach, right kidney, and intestines. It is supplied with blood by the portal vein, which drains the splenic, intestinal, and colonic areas and is a rich source of nutrients and substances absorbed from the gut.
It is also supplied by the hepatic artery (usually a branch of the celiac artery), which provides most of the liver's oxygenated blood. The liver consists of 2 main lobes, which are made up of thousands of lobules. These lobules are connected to small ducts that connect with larger ducts, ultimately to form the common hepatic duct. The common hepatic duct transports the bile produced by the liver cells to the gallbladder and duodenum.5
Contraindications
Transplantation is not indicated if an acceptable alternative is available or if contraindications, such as malignancy, a terminal condition, or poor expected outcome exist.
More on Liver Transplantation |
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| Workup: Liver Transplantation |
| Treatment: Liver Transplantation |
| Follow-up: Liver Transplantation |
| Multimedia: Liver Transplantation |
| References |
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References
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Balistreri WF, Bucuvalas JC, Ryckman FC. The effect of immunosuppression on growth and development. Liver Transpl Surg. Sep 1995;1(5 Suppl 1):64-73. [Medline].
McDiarmid SV. Management of the pediatric liver transplant patient. Liver Transpl. Nov 2001;7(11 Suppl 1):S77-86. [Medline].
Evrard V, Otte JB, Sokal E, et al. Impact of surgical and immunological parameters in pediatric liver transplantation: a multivariate analysis in 500 consecutive recipients of primary grafts. Ann Surg. Feb 2004;239(2):272-80. [Medline].
Cotran RS, Kumar V, Robbins SL. Robbins Pathological Basis of Disease. 5th ed. Philadelphia, PA: WB Saunders Co; 1994:841.
The Organ Procurement and Transplantation Network. National Data Reports. OPTN. Available at http://www.optn.org/latestData/step2.asp.
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Chang FY, Singh N, Gayowski T, et al. Fever in liver transplant recipients: changing spectrum of etiologic agents. Clin Infect Dis. Jan 1998;26(1):59-65. [Medline].
Al-Uzri A, Yorgin PD, Kling PJ. Anemia in children after transplantation: etiology and the effect of immunosuppressive therapy on erythropoiesis. Pediatr Transplant. Aug 2003;7(4):253-64. [Medline].
Bucuvalas JC, Ryckman FC. Long-term outcome after liver transplantation in children. Pediatr Transplant. Feb 2002;6(1):30-6. [Medline].
Roggero P, Cataliotti E, Ulla L, et al. Factors influencing malnutrition in children waiting for liver transplants. Am J Clin Nutr. Jun 1997;65(6):1852-7. [Medline].
Kling K, Lau H, Colombani P. Biliary complications of living related pediatric liver transplant patients. Pediatr Transplant. Apr 2004;8(2):178-84. [Medline].
Araz C, Pirat A, Torgay A, et al. Early postoperative complications of pediatric liver transplantation: experience at one center. Transplant Proc. Jan-Feb 2004;36(1):214-7. [Medline].
Nobili V, Comparcola D, Sartorelli MR, et al. Mycophenolate mofetil in pediatric liver transplant patients with renal dysfunction: preliminary data. Pediatr Transplant. Dec 2003;7(6):454-7. [Medline].
Reding R. Tacrolimus in pediatric liver transplantation. Pediatr Transplant. Dec 2002;6(6):447-51. [Medline].
Guthery SL, Heubi JE, Bucuvalas JC, et al. Determination of risk factors for Epstein-Barr virus-associated posttransplant lymphoproliferative disorder in pediatric liver transplant recipients using objective case ascertainment. Transplantation. Apr 15 2003;75(7):987-93. [Medline].
Takada Y, Tanaka K. Living related liver transplantation. Transplant Proc. Mar 2004;36(2 Suppl):271S-3S. [Medline].
Yersiz H, Renz JF, Farmer DG, et al. One hundred in situ split-liver transplantations: a single-center experience. Ann Surg. Oct 2003;238(4):496-505; discussion 506-7. [Medline].
Lopez-Santamaria M, de Vicente E, Gamez M, et al. Pediatric living donor liver transplantation. Transplant Proc. Aug 2003;35(5):1808-9. [Medline].
Paya CV, Wiesner RH, Hermans PE, et al. Risk factors for cytomegalovirus and severe bacterial infections following liver transplantation: a prospective multivariate time-dependent analysis. J Hepatol. Jun 1993;18(2):185-95. [Medline].
United Network for Organ Sharing. Liver Transplantation. MELD/PELD Calculator. Available at http://www.unos.org/resources/meldPeldCalculator.asp.
Further Reading
Keywords
liver transplantation, hepatic failure, hepatic function, end stage liver disease, end-stage liver disease, model for end-stage liver disease, MELD, pediatric end-stage liver disease, PELD, biliary atresia, metabolic disorders, progressive intrahepatic cholestasis, Wilson disease, Wilson's disease, alpha-1-antitrypsin deficiency, tyrosinemia, hemochromatosis, Crigler-Najjar syndrome, glycogenosis, hyperoxaluria, metabolic respiratory chain deficiencies, familial hypercholesterolemia, methylmalonic aciduria, methylmalonyl-CoA, methylmalonyl-CoA methylmalonic aciduria
