Many children with mild or moderate liver disease have no symptoms at all. Some patients, however, may experience the following problems:
Ascites is a collection of fluid in the abdomen of the patient with liver disease. The fluid is outside the organs and collects because of a blockage to blood flow through the portal vein into the liver. Since blood cannot get through the liver, it backs up into other tissues which then leak clear fluid into the peritoneum (the lining of the abdomen). This collection of fluid can cause the patient's abdomen to appear quite large, and can cause abdominal pain and poor appetite. This fluid can also become infected in a condition called spontaneous bacterial peritonitis (SBP). Ascites is managed with the use of diuretics, medicines that cause the patient to urinate salt and fluid. Dietary salt restriction may also be used. Sometimes it is necessary for the gastroenterologist to place a small needle into the abdominal cavity and drain out either small volumes of fluid, in order to rule out infection, or large volumes of fluid, in order to make the patient feel better. This procedure is called paracentesis.
Some patients with chronic liver disease experience behavior problems or neurologic symptoms associated with abnormal brain function. This is called encephalopathy. Patients with severe encephalopathy may even be in coma. They may not be able to communicate clearly and may be especially combative or difficult due to an altered state of consciousness. Patients with mild encephalopathy may have more subtle abnormalities. They may "act funny" or do poorly in school. The cause of encephalopathy is not completely clear. It is presumed that some chemical or toxin that should have gone into the liver through the portal vein to be purified is not able to get through the liver because of the scarring of cirrhosis. This substance then backs up into the circulation and may cause changes in behavior and other problems. Ammonia is one of the chemicals that probably fails to be cleaned up by the failing liver, contributing to encephalopathy. Encephalopathy can sometimes be treated by treating the underlying liver problem. Medicines which help the body excrete ammonia may be helpful (lactulose is one of these). Antibiotics are occasionally used to prevent the germs in the intestine from making more chemicals that might contribute to the patient's abnormal mental state. Certain drugs can make encephalopathy worse. These include Versed, Valium and a number of other drugs which act on the central nervous system. Infection or dehydration can also cause a patient to develop increased encephalopathy.
Some children with chronic liver disease develop bleeding into the intestine. Typically, this bleeding occurs because of portal hypertension, increased pressure in the vein leading to the liver. When the liver is badly scarred in a patient with chronic liver disease, blood in the portal vein cannot enter the liver as it should. Instead, the blood backs up into the spleen, and into blood vessels in the esophagus called varices. These blood vessels are like hemorrhoids which line the esophagus. While these veins may not cause any trouble at all, they may sometimes break and bleed. This may cause the child to throw up large amounts of pure blood. The blood may also go through the intestine causing the child to pass black, tarry, and especially bad smelling stool which resembles old blood. Pallor, dizziness and even collapse can result. If GI bleeding occurs and the child appears to be very ill, an ambulance should be called immediately and the medics should be informed the child has chronic liver disease. If the child is relatively stable, the on-call pediatric gastroenterologist can be called for instructions.
When a child presents to the hospital with GI bleeding, first efforts are made to resuscitate the child or to make his/her blood circulation better. This often includes the placement of two large IV's and may include measures to help the child's breathing as well. Sometimes an NG or nasogastric tube is placed to assess the source of bleeding and to remove blood from the intestine. Some physicians elect not to do this as placing the tube can injure varices, causing further bleeding. If the child appears to have lost a lot of blood volume, large amounts of IV fluids are given and the patient is crossmatched to receive blood transfusions if needed. A medication called octreotide may be started intravenously. This drug may help lower the pressures in the varices so that bleeding is less likely to occur. Patients with active bleeding are often monitored in the Intensive Care Unit.
Poor liver function may cause poor blood clotting or coagulopathy. This may contribute to GI bleeding and may need to be treated either with vitamin K or with transfusion of fresh frozen plasma. Decreased platelet counts in patients with chronic liver disease may also contribute to bleeding.
Variceal bleeding often stops spontaneously, although rebleeding frequently occurs in the next days to weeks. Patients are often taken to endoscopy (see chapter on procedures) where the source of bleeding is checked out. The endoscopist makes sure that the bleeding is from varices and not from an ulcer or some other cause. Techniques called variceal band ligation or sclerotherapy can be used through the scope to close the veins from which the bleeding occurred. Variceal banding or sclerotherapy do not treat the underlying problem, the increased pressure in the portal vein due to cirrhosis, but they can prevent bleeding. Sometimes, however, esophageal varices came back after treatment. Varices can even develop in the stomach or intestine. These are much harder to treat than varices in the esophagus.
Medications are sometimes used to prevent rebleeding. Propranolol is a "beta blocker" drug that decreases the heart rate, decreases the blood pressure and decreases the pressure in the varices. Doses of propranolol sufficient to drop the heart rate by 20% have been shown to drop the pressure in the portal vein and decrease the risk of bleeding. This approach to prevention is effective in many adult patients, though is yet unproven in children.
Patients sometimes undergo a procedure called TIPS (transjugular intrahepatic portosystemic shunt) to control the portal hypertension. This procedure is described in detail in the "Procedure" section. Essentially, a connection is made between the portal vein, which carries blood into the liver at high pressures because of the scarring of the liver, and the hepatic vein, which helps blood leave the liver. This shunt creates a "short circuit" that does not require blood to go through the scarred liver tissue. This procedure is performed by an interventional radiologist and can decrease the portal pressures to prevent further bleeding. These shunts usually require later procedures to keep them open.
Sometimes a surgical shunt is needed to correct portal hypertension leading to GI bleeding. There are a variety of operations which shunt the vein flow away from the liver. Instead of blood backing up from the hard liver through the portal vein into varices, the portal vein blood is shunted or short-circuited to another blood vessel so it cannot back up. This surgery works well in patients with GI bleeding due to portal hypertension when the liver still works well, but is very risky in patients who are very sick with their liver disease.
Itching or "pruritus" can be a very difficult symptom of chronic liver disease in some children with bile duct problems. Accumulation of bile acids in the bloodstream can cause itching, for reasons which are not clear. Some patients have poor bile flow and very high bilirubin yet experience no itching at all. Other patients might have a nearly normal bilirubin but might experience terrible itching without any obvious rash on the skin. Children may scratch at their skin anywhere on their body, although the ear canals, the skin on the face, and the bottoms of the feet are common locations. The child may rub the skin repeatedly in an attempt to relieve the itching. This sometimes results in marked thickening of the skin.
Many therapies have been tried to help the itching that can occur in children with liver disease. Certainly, keeping the skin in good condition helps. This means keeping dry skin lubricated. In addition, keeping fingernails short prevents the child from causing further damage to the skin, which might lead to further itching. Antihistamines such as Benadryl and Atarax are commonly used. These probably work mostly by sedating the child, as sleepiness is a common side effect of these medications. Drugs that promote bile flow or change the composition of the bile salt pool are also used. Actigall (ursodeoxycholic acid or "URSO") can make bile flow better and is sometimes used for itching; in some cases, however, this drug may worsen the itching. Cholestyramine is a resin that binds bile salts and makes them come out in the stool. This may stimulate the liver to have better bile flow. An antibiotic called rifampin may help with itching. This drug has to be used carefully since it can injure the liver. Naltrexone, a drug which blocks opiate receptors, has been tried to help the itching in desperate situations. This drug must be used with great caution since it can precipitate liver failure as well as other problems. In some children itching has been so severe that surgical procedures have been done to divert the bile flow away from the liver and intestine. Liver transplantation has also been done in desperate situations where itching was a large obstacle to quality of life.