Abstract
This chapter continues the scenario from the chapter on Hepatic Portoenterostomy or Kasai Procedure. The pathophysiology of end stage liver disease is reviewed with its specific effects on the individual organ systems. The authors provide a detailed explanation of the pre-operative evaluation for patients requiring liver transplantation. A detailed description of the 3 main phases of liver transplantation is presented with attention to the related anesthetic considerations.
Three years following his Kasai procedure, a four-year-old male with biliary atresia (see Chapter 27) is posted for orthotopic liver transplantation for end-stage liver disease. He is currently intubated in the Pediatric ICU on dopamine and epinephrine infusions to maintain his blood pressure. Total parenteral nutrition and intralipids are infusing through a right subclavian line. Oxygen saturation on room air is 91%. His international normalized ratio (INR) is 5.6 and has required multiple transfusions of Elsewhere in book this is written as PRBCs. Make consistent to PRBCs, fresh frozen plasma (FFP), and platelets.
Hemoglobin: 8.0, Hematocrit: 23.8, Platelets: 60,000, PT: 49.6, INR: 5.6, PTT 88.3
Na: 129, K: 3.1, Cl: 105, HCO3: 19, BUN: 30, Cr: 2.3, Glucose 65, Ca: 0.85, Mg: 1.9
Albumin: 2.5, Total Bilirubin 3.9.
What Are the Indications for and Underlying Diagnoses Associated with Pediatric Liver Transplantation?
The primary indications for liver transplantation are acute fulminant hepatic failure, chronic end-stage liver disease, and progressive primary liver disease refractory to maximal medical management and metabolic disease. Table 28.1 lists the common underlying diagnoses associated with liver transplantation.
Table 28.1 Relative frequency of the primary indications for liver transplantation in children. Data from Cote and Lerman, eds. A Practice of Anesthesia for Infants and Children. 5th Ed. Elsevier 2013
| Diagnosis | Frequency (%) | |
|---|---|---|
| Cholestatic liver disease | 54 | |
| Biliary atresia | ||
| Alagille syndrome | ||
| Primary sclerosing cholangitis | ||
| Intrahepatic cholestasis | ||
| Biliary cirrhosis | ||
| Fulminant hepatic failure | 14 | |
| Acute liver failure | ||
| Cirrhosis | ||
| Autoimmune hepatitis | ||
| Neonatal hepatitis cirrhosis | ||
| Metabolic disease | 14 | |
| Alpha 1 antitrypsin deficiency | ||
| Urea cycle defects | ||
| Cystic fibrosis | ||
| Wilson’s disease | ||
| Tyrosinemia | ||
| Primary hyperoxaluria | ||
| Crigler-Najjar syndrome | ||
| Glycogen storage disease | ||
| Primary hepatic malignancy | 6 | |
| Hepatoblastoma | ||
| Other | ||
| Other | 6 | |
| Congenital hepatic fibrosis | ||
| Budd–Chiari syndrome | ||
What Is the PELD Score and How Is It Used in Pediatric Liver Transplantation?
The Pediatric End-Stage Liver Disease (PELD) score was implemented in 2002 to prioritize organ allocation to the sickest patients, rather than those who had been waiting the longest. It is used for children 12 years and younger. PELD uses the patient’s values for serum bilirubin, serum albumin, the international normalized ratio for prothrombin time (INR), whether the patient is less than 1 year old, and whether the patient has growth failure (<2 standard deviation) to predict survival. It is calculated according to the following formula:
PELD = 4.80[Ln serum bilirubin (mg/dL)] + 18.57[Ln INR] − 6.87[Ln albumin (g/dL)] + 4.36(<1 year old) + 6.67(growth failure)
The PELD score is used to calculate a numerical value that is an accurate predictor of three-month mortality, independent of portal hypertension and etiology of the liver disease. A higher score correlates with a more critical condition. Thus, liver donations are usually allocated by United Network for Organ Sharing (UNOS) according to the PELD score to maximize the life-saving capability of each donated liver.
What Are the Common Pathophysiologic Derangements of End-Stage Liver Failure?
The pathophysiologic derangements common in patients undergoing liver transplantation are summarized in Table 28.2.
Table 28.2 Pathophysiologic derangements common in patients undergoing liver transplantation
| Organ system | Common findings |
|---|---|
| Cardiovascular | Hyperdynamic circulation: ↑cardiac output, ↓systemic vascular resistance (SVR) |
| Pericardial effusions | |
| Arteriovenous (AV) shunting | |
| Long term: cardiomyopathy, high output heart failure | |
| Pulmonary | Hypoxemia due to V/Q mismatch |
| Impaired hypoxic pulmonary vasoconstriction | |
| Intrapulmonary shunting | |
| ↓ functional residual capacity (FRC) due to ascites | |
| Pulmonary hypertension | |
| Hepatopulmonary syndrome | |
| Pleural effusions | |
| CNS | Encephalopathy |
| Cerebral edema | |
| Renal | Prerenal azotemia |
| Hepatorenal syndrome | |
| GI | Hepatic dysfunction: synthetic, metabolic and excretory |
| Portal hypertension | |
| Delayed gastric emptying | |
| Hematologic | Thrombocytopenia |
| Coagulopathy | |
| Anemia | |
| Hypofibrinogenemia, dysfibrinogenemia | |
| Disseminated intravascular coagulopathy | |
| Laboratory/electrolyte anomalies | Metabolic acidosis |
| Hypokalemia | |
| Hyponatremia | |
| Intravascular volume depletion |
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