What is portal hypertension?
Portal hypertension is an increase in the blood pressure within the portal vein, a blood vessel that carries blood from the stomach, intestine, spleen and pancreas to the liver. This condition can lead to many complications and can require a liver transplant in severe cases.
What causes portal hypertension in children?
Portal hypertension can be caused by any of the following:
- A blockage due to a blood clot (thrombosis)
- A malformation of the portal vein itself
- Scarring on the liver (cirrhosis)
- Any liver disease that blocks the flow of blood through the liver
The increased pressure in the portal vein also causes pressure to increase in other veins. This increased pressure in the veins can force blood to back up into the splenic vein, causing the spleen to enlarge. Increased pressure in the veins of the intestines can cause diarrhea and bleeding of the gastrointestinal (GI) tract.
Portal hypertension can lead to the growth of new blood vessels called varices that connect blood flow from the GI tract, such as the intestine, to the general circulation, bypassing the liver. When this happens, toxins and nutrients that the liver would normally process can travel into the rest of the bloodstream.
Varices form when the body senses the increased portal vein pressure and tries to compensate. Varices are weaker than normal vessels and can bleed easily. They tend to grow around the esophagus, spleen, stomach and colon and can cause life-threatening bleeding in the digestive tract.
Who gets pediatric portal hypertension?
Children may develop portal hypertension if they have a blockage of the portal vein outside of a healthy liver, called extra-hepatic portal vein thrombosis. Portal hypertension also commonly occurs in children with severe liver disease.
What are the signs and symptoms of portal hypertension?
The main symptoms and complications of portal hypertension include:
- Gastrointestinal (GI) bleeding, which can cause black, tar-like stools or blood in the stools; vomiting of blood due to the spontaneous rupture and bleeding from varices (enlarged blood vessels that can develop in the esophagus, stomach or intestines)
- Ascites, which is a buildup of fluid in the abdomen; infection of this fluid can be very dangerous
- Encephalopathy (a brain disorder), which causes confusion and forgetfulness and is caused by poor liver function and the diversion of blood flow away from the liver
- Swelling of the spleen, which can cause pain and swelling of the abdomen
- Growth failure
What tests are used to diagnose portal hypertension?
Children who are suspected to have portal hypertension will undergo a thorough evaluation that can include the following:
- Physical exam
- Blood tests to assess how well the liver is working and if an underlying liver disease exists
- Imaging tests that can include an ultrasound, CT scan or an abdominal angiogram to take pictures of the blood vessels
- Endoscopic evaluation, which uses a small camera to check for GI bleeding
- Liver biopsy
What to expect from an endoscopic evaluation and a liver biopsy
Endoscopic evaluations and liver biopsies are performed in our Gastroenterology (GI) Procedure Center by a board-certified pediatric hepatologist and pediatric anesthesiologist.
The biopsy is performed under general anesthesia, so your child experiences no pain or discomfort. The doctor will remove a small piece of the liver through a tiny cut in the skin using a special needle. A board-certified pediatric pathologist with extensive experience evaluating pediatric liver biopsies will review this sample of liver tissue.
How do we make a diagnosis for portal hypertension?
We make a diagnosis for portal hypertension by examining your child’s medical history, performing a physical exam and verifying imaging tests. The liver biopsy helps the doctor identify how well the liver is functioning and if an underlying liver disease is causing portal hypertension. Common symptoms of pediatric portal hypertension, which would prompt a referral to our Pediatric Liver Center, include upper GI bleeding and an enlarged spleen.
Why choose us to test for portal hypertension?
The Pediatric Liver Center at Children’s Hospital Colorado has extensive experience caring for children with portal hypertension. We have a multidisciplinary team of specialists who can quickly and accurately analyze blood tests and perform imaging tests, endoscopic evaluations, and if necessary, a liver biopsy to evaluate for portal hypertension.
We not only provide diagnostic testing, but also a variety of medical and surgical treatment options to allow us to individualize your child’s care in the short and long term. Some children with portal hypertension and liver disease will require liver transplant. If liver transplantation is necessary, our team in the Pediatric Liver Transplant Program has extensive experience performing transplants in children with portal hypertension, using both deceased donor and live donor liver transplants.
How is portal hypertension treated?
The complications of portal hypertension can be managed with medications, endoscopic therapy, surgery or interventional radiologic procedures.
In addition to immediate treatment of complications such as fluid in the belly and GI bleeding, long-term treatment options are prescribed based on the severity of the symptoms and on how well the liver is functioning. If the portal hypertension is due to liver cirrhosis, liver transplantation may be necessary.