Orthotopic cadeveric liver transplantation (LT) has become a standard of treatment for cirrhosis/end stage liver disease (ESLD) as well as acute liver failure (ALF), primary liver cancer (hepatocellular carcinoma-HCC) and some metabolic diseases.
Cirrhosis comes from the Greek word of kirrhos; orange yellow, +osis, condition. This atrophic liver loses its ability to secrete bilirubin, build up of various proteins (i.e., coagulation proteins, albumin, immune antibodies), clear toxins (i.e., blood ammonia).
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Patients might also bleed from portal hypertensive gastropathy (PHG), which is diffuse bleeding from the surface of the stomach. TIPS will be indicated in these cases as well.
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Severe portal hypertensive
gastropathic changes |
Mild-moderate portal hypertensive
gastropathic changes (mosaic
pattern) of the gastric mucosa |
Gastric varices are also of concern for causing upper GI bleeding. It is usually difficult to stop them bleeding endoscopically, requiring TIPS placement for the control of their bleeding.
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A nipple on top of varix signifies a recent episode
of bleeding and carries a higher risk of rebleeding |
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| Actively bleeding varix |
Hemostasis after rubber banding
of the same varix |
- Ascites in association with collateral venous return and abdominal wall hernia: The fluid collection in the abdominal and thoracic cavity is usually managed with salt, fluid restriction in the diet and diuretics. Large volume paracenthesis with albumin infusions are highly effective. TIPS can be considered in selected advanced cases, unresponsive to the above interventions.
- Encephelopathy: State of confusion induced by high serum ammonia levels. In advanced stages, it can induce hepatic coma. It is managed by changing the intestinal flora by addition of Lactulose as a stool softener. 1 gram /kg body weight of protein intake is essential in these cases to avoid malnutrition. Malnutrition is usually manifested by temporal and overall body muscle wasting.
- Renal compromise: Hepatorenal Syndrome (HRS) reflects the severity of the liver disease and the urgency for LT consideration. Followed by serum creatinine (Cr) levels.
- Hepatopulmonary Syndrome (HPS): These patients usually present with cyanosis and enlargement of the finger tips (clubbing). Home oxygen treatment is required until the time of LT since blood oxygen levels will be decreased due to ill effects of cirrhosis on the lungs. Often reversible following LT.
- Porto-Pulmonary Hypertension (PPH): In mild to moderate cases, urgent LT consideration is required to avoid further cardiopulmanory complications. Patients might be required to be on continuous drip of epoprostenol (prostaglandin) at home, in preparation for the LT. Severe cases usually are not offered LT.
- Hypersplenism: Enlargement of the spleen due to portalhypertension can cause decreased platelets and white blood cells, in the peripheral blood, making the cirrhotics prone to easy bruising and some infections.
- Hypocoagulable state: Induced by deficient production of the blood coagulation factors by the cirrhotic liver. Patients are usually prone to spontaneous bleeding. This activity is reported by the lab as PT/INR(prothrombin time/international ratio).
- Jaundice: Signifies a major secretory dysfunction of the liver. Followed by serum total bilirubin (TB) levels.
- Itching usually becomes a problem in patients with cholestatic liver diseases, i.e., PBC, PSC.
- Recurrent cholangitis (infection originating from the bile ducts) usually develops on the basis of PSC and secondary biliary strictures. These patients will require frequent ERCP or PTC (percutaneous transhepatic cholangiogram) to be able to dilate and place stents in to the bile ducts.
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Plastic drainage
stent placed into the
bile ducts by ERCP |
MELD (Model for Endstage Liver Disease) Scoring System
It is a liver disease severity score derived from the transformation of three major biochemical parameters, i.e., PT/INR, TB and Cr., in a logarithmic formula. It is implemented by UNOS since Feb. 27, 2002 in an effort to prioritize the organ allocation, since it is highly predictive of the next three months’ mortality. Transplant work up is usually initiated when the MELD score reaches around 15 or a major complication is encountered. MELD emphasis is on the acuity of the case rather than the time spent on the waiting list. For MELD score calculation please visit www.unos.org.
LT Candidate Work up Consultation and Procedures
Once the transplant hepatologist ( Liver Transplant Medical Specialist) identifies the LT candidate patient, work up is initiated in coordination with the liver transplant surgical team. All patients and their immediate family members are interviewed by the transplant social worker. In the absence of psychosocial contraindications, work up is pursued with other essential consultations.
All patients undergo cardiopulmonary work up by a transplant cardiologist, regardless of their risk factors. Patients with certain risk factors might also be required to be evaluated by a neurologist. Renal, pulmonary, psychiatric consultations are obtained on an as needed basis.
All patients undergo radiological evaluation by ultra sound (US) and CT scan of the abdomen. US exam also involves Doppler study of the portal vein, to check for its patency. CT scan with three phasic IV contrast is usually a reliable way of searching for HCC. MR of the liver can be ordered for further evaluation, if suspicious lesions are identified on CT/US exams. CT scan also computes the liver volume, which is important for the size match.
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Normal liver on CT scan,
with smooth liver contours;
normal size spleen |
Cirrhotic liver with small
nodular appearance,
enlarged spleen,
ascites around the liver |
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Small liver volume (499 cc)
as computed by CT scan.
Normal liver volume
around 1200 to 1500 cc |
Enlarged spleen volume (1020 cc)
as computed by CT scan.
Normal volume around 250 cc |
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3 cm lesion in a patient with HCV, lighting up during the
arterial phase of the IV contract, consisting with HCC |
Patients with HCC are also required to be seen in consultation with an interventional radiology specialist as well as an oncologist, in preparation for pre LT treatment, i.e., radiofrequency ablation (RFA), alcohol injection, intraarterial chemoembolization.
In case the patient’s condition requires intensive care due to any of the above mentioned major complications, care is delivered in the 701 liver transplant ICU, by the liver intensive care specialists and highly trained liver nursing staff.
After the full preparation period, patient is evaluated by the LT surgeon, before being officially presented to the selection committee.
Selection committee consists of the above mentioned group of physicians as well as the social workers, pre and post LT certified nurse coordinators, hospital ethicist, clergy, financial coordinators and a secretary recording the minutes.
Prior to presentation to the committee, patients are required to undergo the following procedures:
- Esophago-gastro-duodenoscopy (EGD); to screen for esophageal/gastric varices, to identify the extent of portalhypertensive gastropathy (PHG). This is important for bleeding risk strafication while the patient is on the waiting list. Usually the esophageal varices are endoscopicaly ligated with rubber bands.
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Single column of
esophageal varices |
Multiple columns of
esophageal varices
with cherry red spots |
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Single esophageal
rubber band ligation |
Multiple band ligations
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- Colonoscopy: All patients with cirrhosis older than age 35, or younger patients with higher risk (history of colitis or family history of early colon cancer), undergo screening colonoscopy with prostatic digital exam being performed in men at the same time. Polyps are identified and endoscopically removed. Patients with precancerous polyps and with history of colitis will require further screening colonoscopies every few years following LT.
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| Colonic polyp |
After colonic polypectomy |
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Rectal varices
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- Endocopic retrograde cholangio-pancretography (ERCP): Patients with PSC or history of colitis, will require this procedure, to identify the biliary strictures and potential cholangiocarcinoma. Biliary brushings are routinely obtained to rule out malignancy, although the yield is generally low.
All the above mentioned endoscopic procedures are performed by your transplant hepatologist, who is also responsible for the coordination of your work up process.
Liver biopsy is not required for the work up. In rare cases if it is indicated (i.e., cryptogenic cirrhosis, tumor), it will be performed by your hepatologist or the intreventional radiologist with direct ultra sound guidance, with intravenous administration of conscious sedation. In cases of acute liver failure, transjugular approach (neck vein) can be used.
- Ultrasound of liver with vessel patency by Doppler studies
- Computerized tomography (CT) of abdomen and pelvis with oral and intravenous contrast. Liver and spleen volumes are computed, and the spinal bone density is detected during procedure.
HCC cases are also worked up with CT of lungs and brain as well as nuclear bone scan to look for potential metastatic disease.
- Mammogram / pap smear
- Cardiac echo and cardiolyte cardiac stress testing by the transplant cardiologist.
- Chest X-R, sinus X-R, panorex X-R of teeth.
Blood Tests
- Blood type and antibody screen, PRA
- Full hepatitis profile, to include serum HCV-RNA titers, HCV genotype, HBV-DNA, hep B “e” antigen and antibody
- Full autoimmune markers to include iron and copper studies, immune protein electropheresis
- Cancer markers, i.e. alhpa fetoprotein (AFP), CEA, PSA (prostate specific antigen), CA 19-9
- Complete blood count (CBC), complete metabolic panel (CMP) to include magnesium and phosphate
- Coagulation studies, i.e., PT/INR, fibrinogen levels
- Cytomegalovirus (CMV) status, varicella titers, crtyptococcal antibodies
Vaccinations
- Hep A and B vaccination, if there is no evidence of prior immunity.
- Pneunomax (pneumonia vaccine, needs to be repeated every five years).
- Flu vaccination once a year.
- PPD skin test for TB screening is also applied.