Pediatric Portal Hypertension Program
Portal hypertension means there is high blood pressure in the portal vein. The portal vein is the blood vessel that brings all of the blood from the intestines to the liver. It supplies the liver with 75% of its blood supply and 50% of its oxygen. Pressure in the portal vein can be compared with a person’s normal blood pressure.
We understand that children and their circumstances can vary. More detailed information regarding your child’s care and treatment will be provided by your doctors at the time of your visit.
What to Expect
The initial treatment of extra-hepatic portal vein thrombosis (EHPVT) by the team at Lurie Children’s depends on the severity of your child’s initial symptoms. Blood transfusions are often required if your child has vomited large amounts of blood or lost blood in their bowel movements. Medications may also be given intravenously or by mouth to stop bleeding from varices or decrease the chances of future bleeding from the varices. A procedure to decrease the number and size of the varices in the esophagus may also be recommended (called “banding”). These steps are not a cure for EHPVT and portal hypertension but can be an important first step in controlling the problem.
Your child may be referred for surgery at Lurie Children’s after the initial diagnosis of portal hypertension or later after his symptoms are controlled. There are different surgical options, and the best one will depend on your child’s specific needs, the condition of the liver and the condition of the veins inside the abdomen.
Two basic surgical choices include palliative shunts, which essentially decrease the severity of symptoms, and restorative shunts, which restore normal blood flow through the liver.
The Rex Shunt
Use of the restorative shunt, the meso-rex bypass — or “rex shunt” for short — at our hospital began in 1997, and since then, more than 85 children have been operated on here; they have come from 20 states and five countries. The operation is successful in over 90% of patients. Because the rex shunt restores normal blood flow to an otherwise normal liver, we believe that this is the treatment of choice for children with extra-hepatic portal vein thrombosis (EHPVT).
The shunt bypasses the blocked portal vein and restores venous blood flow to the liver. A vein (usually the jugular vein in the neck) is used to build a bridge around the blockage. Blood flows from the large intestinal veins, across the bridge, around the blockage and back into the liver. Blood can then flow from the intestines into the liver in the “normal” way. If for some reason your child cannot have this procedure, other procedures may be considered. Your surgeon will review the different options with you to determine the best procedure for your child.
The rex shunt was first developed in Europe. We were the first to apply this novel technique in North America, and now have the most extensive experience with this unique surgery. In the last 11 years, the procedure has been refined and modified so that it can be applied to a wider number of children with EHPVT.
Following the surgery, the symptoms of portal hypertension usually resolve very quickly. We also believe that restoration of normal portal blood flow to the liver allows the liver to recover some of the functions that may have been impaired because of the obstruction thereby allowing the child to grow and develop to their full potential.
The Hospital Stay
Children spend an average of seven days in hospital after the surgery. Your child will spend one to two days in the Pediatric Intensive Care Unit (PICU) and then the remainder of the stay will be spent on the post-surgical unit. All children receive blood thinners for the first few days after the operation to ensure that the new venous bridge does not clot off.
After the children are eating, medicines are given by mouth to slow down clot formation. After the operation, routine tests and abdominal ultrasounds are done to monitor the flow of blood in the new shunt and to make sure that no clots form to obstruct the flow of blood. Children are usually sent home about seven days after surgery.
Routine blood tests and ultrasounds are repeated every three months and a CT venogram may be done again one year after surgery. Most children can be monitored at their home centers and return to Chicago for their one-year follow-up whenever possible.
Some families prefer to come to Lurie Children's for their check-ups, and we will do everything possible to make their stay as brief and pleasant as possible.
Riccardo Superina, MD, and his team at Lurie Children's have spent the last 10 years perfecting procedures to help correct portal hypertension in children and educate others on the benefits of early diagnosis and treatment.