Portal Hypertension
What is portal hypertension?
Portal hypertension is when a child has increased pressure in the portal venous system that carries blood from the intestines and spleen to the liver. It is often a complication of advanced liver disease and overall, considered a rare condition for children.
Causes
In children, the potential causes of portal hypertension include a blockage or blood clot from a clotting disorder or from unknown reasons. In certain cases, it can happen in newborn babies due to an umbilical vein catheter or another problem that develops in their belly.
Signs and symptoms
Symptoms of portal hypertension include:
- An enlarged spleen.
- Low platelet count.
- Too much fluid in the belly (ascites).
- Gastrointestinal bleeding due to growth of varices (unusual or enlarged veins that are weaker than normal veins and bleed more easily) due to lack of portal blood flow.
- Poor liver function.
Symptoms of these complications include:
- Discomfort in the upper left part of the belly.
- Fluid buildup.
- Black or tarry stools.
- Vomiting of blood.
- Poor weight gain or weight loss.
- Confusion or forgetfulness.
Diagnosing portal hypertension
Your child’s care team will examine them, including a blood draw to check liver function and imaging of the abdomen (belly). If needed, they will also use an endoscope to look for varices (unusual veins that grow in the esophagus) to determine the appropriate diagnosis and treatment.
Treatment
Our approach to treating portal hypertension is multidisciplinary. That means a team of different experts all work together to take care of your child. Your care team may recommend medication or procedures to control or prevent the internal bleeding of varices. Depending on the cause and severity of your child’s condition, they may need surgery.
For children with very advanced cases of portal hypertension who have severe symptoms and damage to their liver, they may need a liver transplant.
If their condition is caused by blockage of the portal vein, one surgical intervention to avoid liver transplantation would be placing a restorative shunt, called a Meso-Rex bypass, or “rex shunt,” to restore normal portal blood flow to the liver. A shunt is when surgeons find a way to go around a problem area and create a new path for the blood to flow. Alternative shunts are also an option, depending on your child's needs.
What to expect
After surgery, most children’s complications due to portal hypertension will clear up thanks to the restoration of normal portal blood flow through the liver.
If your child had a surgical procedure, they will have regular follow-up appointments with their care team. If you are traveling from out of town, we can often work together with your local care provider for your child’s follow up care.
During these visits, your child’s care team will check on their recovery using routine procedures such as blood tests and ultrasounds.
Your child’s schedule of follow-up visits depends on their individual recovery, but typically they will come in every 3 to 6 months the first year after surgery and then once a year after that.
Portal Hypertension: Taylor’s Story
After an early fight against cancer as a toddler, Taylor developed life-threatening portal hypertension as a pre-teen. The team in the Children’s Mercy Liver Care Center was able to give her a surgical solution to get her healthy and back home.
