What are the stages of cirrhosis?
1. Latent phase (well compensated stage)
2. Advanced phase (decompensated stage)
What are the clinical manifestations and complications of decompensated cirrhosis?
The manifestations of decompensated cirrhosis are usually caused by the development of portal hypertension and include:
1) Gastroesophageal varices and bleeding
Esophageal varices are often managed by sclerotherapy or endoscopic variceal band ligation (EVBL). Gastric varices also cause upper GI bleeding but it is usually difficult to stop them bleeding by endoscopic means. Patients with refractory Gastroesophageal bleeding often require a Transjugular intrahepatic Portosystemic Shunt (TIPS) placement for the control of their bleeding. Patients might also bleed from portal hypertensive gastropathy (PHG), which is diffuse bleeding from the surface of the stomach. TIPS is indicated in these patients as well. Transjugular intrahepatic portosystemic shunting (TIPS) is indicated in some selected cases not responding to EVBL. TIPS is placed by an interventional radiologist between the hepatic vein and portal vein. In some instances, a shunt may have to be created surgically by a liver transplant surgeon, who also specializes in these types of shunt procedures. 2) Ascites
Ascites is the presence of excess fluid in the abdominal cavity and frequently develops in patients with chronic liver disease. Ascites is sometimes seen in association with collateral venous return (caput medusae) and abdominal wall hernia. The fluid collection in the abdominal and thoracic cavity is usually managed with salt restriction to 2 Grams of Sodium a day and in addition to diuretics. Fluid restriction is especially necessary in patients who develop hyponatremia (low serum Sodium levels). Large volume paracentesis with albumin infusions are usually effective. TIPS can be considered in selected patients, refractory to the above interventions.
3) Spontaneous Bacterial Peritonitis (SBP)
SBP is often seen in patients with cirrhosis and ascites and refers to bacterial infection of the ascitic fluid without the presence of a surgically treatable cause. Patients with SBP are treated with antibiotics and may require antibiotic prophylaxis to prevent recurrent infections.
4) Encephalopathy
Hepatic Encephalopathy (HE) refers to the state of confusion induced by high serum Ammonia levels. Signs of the buildup of toxins in the brain include neglect of personal appearance, slow in responsiveness, forgetfulness, trouble concentrating or even changes in sleep habits. In advanced stages, hepatic encephalopathy can induce coma (Hepatic Coma -HE). HE is managed by changing the intestinal flora by addition of Lactulose as a stool softener. Antibiotics like Rifaximine can be useful.1 gram /kg body weight of protein intake is essential in these patients to avoid malnutrition.
5) Hepatorenal Syndrome
Hepatorenal Syndrome (HRS) is decreased renal function as a consequence of cirrhosis and reflects the severity of the liver disease and the urgency for liver transplant consideration.
6) Hepatopulmonary Syndrome
Hepatopulmonary Syndrome (HPS) patients usually present with cyanosis (bluish discoloration of skin) and clubbing (enlargement of the finger tips). Home oxygen therapy is required prior to liver transplant since blood oxygen levels are decreased due to effects of cirrhosis on the lungs. HPS is often reversible following liver transplantation.
7) Porto pulmonary Hypertension
Porto pulmonary Hypertension (PPHTN) is a pulmonary vascular disorder seen in patients with portal hypertension. In mild to moderate cases, urgent liver transplant is required to avoid further cardiopulmonary complications. Patients might be required to be on continuous drip of epoprostenol (prostaglandin) at home, or some other oral medications, in preparation for the liver transplant. Severe cases usually are not offered liver transplantation because of significantly increased morbidity and mortality.
8) Hypersplenism
Enlargement of the spleen due to portal hypertension can cause decreased platelets and white blood cells in the peripheral blood, making the cirrhotic patient prone to easy bleeding and some infections.
Clinical manifestations of cirrhosis unrelated to portal hypertension include:
9) Coagulopathy
This is caused by deficient production of blood coagulation factors by the cirrhotic liver. Patients are usually prone to spontaneous bleeding and easy bruising. The PT/INR is used to follow the hypocoagulable state in a cirrhotic patient.
10) Jaundice
Jaundice is a yellowish discoloration of the skin and eyes that occurs when the diseased liver does not secrete enough bilirubin. This signifies a major secretory dysfunction of the liver and is followed by serum total bilirubin (TB) levels.
11) Itching
Itching usually becomes a significant problem in patients with cholestatic liver diseases i.e., PBC, PSC.
12) Edema
Accumulation of fluid in the arms and legs occurs when the liver loses its ability to make the protein albumin.
13) Hepatocellular Carcinoma
Hepatocellular carcinoma HCC is a primary liver tumor commonly caused by cirrhosis usually in the setting of Hepatitis B and C, although it can occur in cirrhosis from any cause. It has a high mortality rate.
14) Sensitivity to medication
Cirrhosis decreases liver's ability to metabolize medications from the blood. This cause some medications act longer than expected and build up in the body. This causes a person to be more sensitive to medications and their side effects.
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