Cirrhosis is a condition of severe liver scarring, known as fibrosis, that changes the organ’s structure and impairs its function. While often associated with lifestyle factors in adults, cirrhosis in children stems from a distinct set of medical conditions. These underlying issues are often present at birth or emerge during early childhood, leading to progressive liver injury.
Underlying Causes of Cirrhosis in Children
A primary category of causes involves diseases of the biliary tract, which is responsible for draining bile from the liver. Biliary atresia is a prominent example in infants, where the bile ducts are inflamed and blocked shortly after birth. This blockage prevents bile from leaving the liver, causing it to build up, destroy liver cells, and lead to rapid scarring.
Genetic and metabolic disorders represent another significant group of conditions leading to pediatric cirrhosis. Alpha-1 antitrypsin deficiency involves the production of an abnormal protein that becomes trapped in the liver, causing cell damage. Wilson’s disease is another inherited disorder that prevents the body from properly removing excess copper, leading to its accumulation in the liver and subsequent injury. Cystic fibrosis can also contribute to liver problems that may advance to cirrhosis.
Beyond biliary and genetic issues, other factors can cause cirrhosis in children. Autoimmune hepatitis occurs when the body’s own immune system attacks the liver, causing inflammation and damage. Though less common due to widespread vaccination, certain viral infections can also result in chronic liver disease. Non-alcoholic fatty liver disease (NAFLD) has become a growing concern, particularly in older children and adolescents, where fat accumulation in the liver can progress to inflammation and scarring.
Signs and Symptoms in Young Patients
One of the most recognizable physical signs is jaundice, a condition that causes a yellowing of the skin and the whites of the eyes. This occurs because the scarred liver cannot effectively process bilirubin, a yellow pigment produced during the breakdown of red blood cells. Another common sign is a swollen abdomen, known as ascites, which results from fluid building up in the abdominal cavity. Swelling, or edema, can also appear in the legs and ankles.
Poor growth or failure to gain weight is often an early indicator, as the liver’s role in metabolism is compromised. Children may also experience persistent fatigue, muscle weakness, and a poor appetite. Easy bruising or bleeding can occur because the damaged liver produces fewer of the proteins needed for blood clotting.
As the condition progresses, signs related to complications may become apparent. Portal hypertension, a buildup of pressure in the portal vein, is a serious complication. This can cause visibly enlarged veins on the abdomen. In some cases, these swollen veins, called varices, can form in the esophagus and stomach, creating a risk of internal bleeding.
How Pediatric Cirrhosis is Diagnosed
The diagnostic process begins with a physical examination and a review of the child’s medical history. A healthcare provider will look for physical signs like jaundice, abdominal swelling, or an enlarged liver or spleen.
Blood tests are a part of the diagnostic workup. Liver function tests (LFTs) measure levels of specific enzymes and proteins in the blood, such as bilirubin and albumin, which can indicate liver injury and how well the organ is working. Blood tests can also check for factors related to blood clotting.
Imaging studies provide a non-invasive way to visualize the liver and surrounding structures. An abdominal ultrasound is often the first imaging technique used to assess the liver’s size, texture, and blood flow, and can also detect an enlarged spleen or fluid in the abdomen. More detailed images may be obtained through magnetic resonance imaging (MRI) or computed tomography (CT) scans.
A liver biopsy is considered the most accurate method for confirming a diagnosis of cirrhosis. This procedure involves taking a very small sample of liver tissue, usually with a thin needle, which is then examined under a microscope. This direct examination of the tissue allows pathologists to see the extent of scarring and structural damage, confirming the presence of cirrhosis and sometimes helping to identify its cause.
Treatment and Management Strategies
The primary goal in managing pediatric cirrhosis is to address the specific underlying condition causing the liver damage. For instance, if autoimmune hepatitis is the cause, medications to suppress the immune system may be prescribed. In cases of Wilson’s disease, treatments are used to remove excess copper from the body, which can slow or halt further scarring.
Nutritional support is important for managing cirrhosis in children to ensure they receive the necessary calories and nutrients for growth. Children with liver disease often have increased energy needs and may require specialized, high-calorie formulas. Supplementation with fat-soluble vitamins, including A, D, E, and K, is frequently necessary because the damaged liver struggles to absorb them properly. In some situations, continuous feeding through a nasogastric tube may be used to deliver adequate nutrition.
Managing the complications of cirrhosis is another aspect of treatment. For ascites, or fluid buildup in the abdomen, doctors may prescribe diuretics and recommend a low-salt diet to help reduce swelling. Portal hypertension and the associated risk of bleeding from esophageal varices are monitored closely through regular screenings.
When the liver’s function deteriorates to the point of failure, a liver transplant becomes the definitive treatment. This surgical procedure replaces the diseased liver with a healthy one from a donor and can be a life-saving option for children with end-stage liver disease. For many of the genetic and metabolic conditions that cause cirrhosis, a transplant not only treats the liver failure but also cures the underlying disease itself.