Hanan Fathy

Problems Faced by Pediatric Liver Tx

Programs (Recipient Issues)

❏ Still existing pediatric pre-transplant mortality

❏ Late referrals (fulminant hepatic failure)
❏ Lack of extracorporeal organ support
❏ Relatively high complication rate (biliary strictures)
❏ Strive to accomplish a meaningful life longevity of pediatric recipients
(87.8% - 3 yrs)
❏ Indications for liver transplantation in
infants and children include
❏ Acute liver failure (ALF),
❏ Chronic liver failure,
❏ Complications of cholestasis
❏ In young children, the most common liver
disease leading to transplantation is biliary
atresia and it accounts for at least 50 percent
of all liver transplants in children
 Frequency
(% of transplants)
Biliary atresia 50
a-1-Antitrypsin deficiency 10
Familial cholestasis 9
Chronic active hepatitis 6
Fulminant hepatic failure 5
Neonatal hepatitis and perinatal hemochromatosis 4
Wilson's disease 3
Tyrosinemia 2
Glycogen storage disease 2
Primary sclerosing cholangitis 2
Other 7
Biliary atresia

a. Status post failed Kasai procedure

b. Recurrent ascending cholangitis

c. Complications of cirrhosis as listed below

Cirrhosis of any etiology with the following
complications

a. Growth failure
b. Ascites that is refractory to medical
management
c. Episodes of variceal bleeding that are
refractory to sclerotherapy and/or TIPS
d. Hypersplenism causing thrombocytopenia
e. Liver synthetic failure
f. Other major systemic complications
• Fulminant hepatic failure

• Neonatal liver failure

• Inborn errors of metabolism

a. Tyrosinemia
b. Glycogen storage disease
c. Crigler-Nijjar syndrome
d. Ornithine transcarbamylase deficiency
e. Other defects with the potential to cause neurologic or
other major systemic complications

• Unresectable hepatic tumors without extension

 Assessing Severity: MELD

❏ Mayo (Model) End Stage Liver Disease

❏ Bilirubin
❏ Creatinine
❏ INR

❏ Extra points for incidental hepatic tumor

❏ Deceased donor allocation by MELD score
❏ Mean MELD at transplant 26 (UVA)
❏ PELD scoring:
This system is based on the risk or probability of
death within 3 months if the patient does not
receive a transplant.
❏ The PELD score is calculated based on
laboratory data and growth parameters.
❏ The laboratory values used are a patient's
albumin, bilirubin, and INR
❏ These values are used together with the patient's
degree of growth failure to determine a score
that can range from 6 to 40.
 Contraindications to transplant
❏ Acceptable alternative therapy

❏ Expected poor outcome

❏ Impairment of other organ systems

Cardiac
Pulmonary
Intestinal
Renal

❏ Infection

❏ Malignancy
 Living Donor Transplantation:
❏ Donor Advantages
❏ Emotional Gain
❏ Donor Disadvantages
❏ Psychological stress to donor and family
❏ Inconvenience / risk of evaluation process
❏ Operative mortality ( 1/150 liver)
❏ Major postoperative complications (2-10%)
❏ Minor post operative complications (50%)
❏ Possible long term morbidity
❏ Unrecognized covert liver disease
 Living Liver Donor Evaluation

❏ Donors should be related to recipient

❏ Donor:
❏ Age < 55yrs
❏ Compatible blood group
❏ History & physical
❏ Routine labs
❏ Ultrasound of liver
❏ Volumetric analysis by MRA
❏ R. lobe wt. = 1% recipient total wt
 Living Donor Evaluation
❏ Decision to donate must be voluntary
❏ Informed consent
❏ Motivation to donate
❏ Behavioral and psychological health
❏ Donor recipient relationship
Removal of cirrhotic liver
Implanting the new liver
New liver
 Early Post-operative
Management
❏ Extubate early
❏ Remove lines as soon as possible
❏ Immunosuppression
❏ Replace ascitic losses with albumin
❏ Avoid FFP unless
❏ active bleeding
❏ Invasive intervention is required
 Classification of Complications
❏ Graft dysfuntion or non-function
❏ Technical
❏ Immunological
❏ Infective
❏ Extrahepatic
❏ Drug effects
❏ Medical
❏ Recurrent Disease
 Immunological
Acute Rejection
5-10 days post transplant but may occur at any time
Clinical Elevated LFTS
Liver Biopsy Portal tract inflammatory infiltrate
Endothelialitis
Destruction of biliary epithelium
Treatment Steroids
 Infective
❏ Bacterial < 15 days
❏ Commonly lines /chest /urine
❏ Fungal > 15 days
❏ Prolonged hospitalization
❏ Broad spectrum antibiotics
❏ Re transplant
❏ Roux loop
❏ Viral > 4 weeks
❏ CMV – recipients of CMV + organs
 Extra-hepatic
❏ Cardiac failure / infarction
❏ Pulmonary
❏ Neurological
❏ Renal
❏ Endocrine
❏ Diabetes (33%)
❏ Drug effects
❏ Recurrent disease
Causes of liver graft loss in children

❏ Vascular thrombosis 15%

❏ Rejection 8%
❏ Biliary complications 5%
❏ Primary non function 3%