If your child has been diagnosed with portal hypertension, you may be wondering what this is and where it can be treated. This information is intended to provide an introduction into pediatric portal hypertension and the various surgical treatment options available.
Dr. Ravi S. Radhakrishnan, MD, at the Children’s Hospital of the University of Texas Medical Branch, has received specialized training to help manage and treat pediatric portal hypertension from the nation’s leading expert in the surgical treatment of pediatric portal hypertension, Dr. Riccardo Superina at Children’s Memorial Hospital in Chicago.
The following sections will provide a brief overview of pediatric portal hypertension and some of the options that are available at our center. Each child’s anatomy and disease process is unique and, after a thorough evaluation by our team, we hope to provide a treatment plan tailored to your child’s specific needs.
Portal hypertension means that there is high blood pressure in the portal vein. The portal vein drains the blood from the intestines and supplies vital nutrients from the intestine to the liver.
Portal hypertension can happen because the liver is damaged and is slowly being replaced with scar tissue, called cirrhosis. Portal hypertension can also happen after a blood clot forms in the portal vein, called portal vein thrombosis or extra-hepatic portal vein thrombosis (EHPVT). This prevents normal blood flow through the portal vein to the liver leading to an increase in portal venous pressure. When portal venous flow is obstructed, it must flow through other vessels, or varices, to return to the heart. As the pressure in the portal vein increases, more varices are formed to try to return all the blood to the heart. These varices are not designed to handle the large volume of blood or the high pressure from the portal vein. As a result, varices can burst, leading to significant bleeding, typically from the esophagus or the stomach, leading to vomiting of blood or bloody bowel movements.
Portal hypertension in children can develop from scar formation in the liver (cirrhosis) or from portal vein blockage (thrombosis). Cirrhosis can be due to a number of conditions, including metabolic abnormalities, genetic diseases, infectious diseases, or congenital abnormalities. While children with cirrhosis have underlying liver disease, the majority of children with portal vein thrombosis have an otherwise normal liver. The risk of EHPVT may be increased in children with hypercoagulable conditions or intravenous catheters placed in the umbilical vein at birth. However, in the majority of children, the cause is unknown.
Children with portal vein thrombosis may have a variety of different symptoms but typically have an enlarged spleen, distended abdomen, vomit blood or pass blood in bowel movements, low platelet counts or low white blood cell counts. While this can be diagnosed in infants or adults, the most common age at diagnosis is 2-4 years old.
EHPVT may be suspected by symptoms and confirmed by a variety of tests, including ultrasound, CT scan, MRI, or angiography.
The initial treatment of EHPVT often involves blood transfusions, medications to decrease the blood flow through the varices, or endoscopic procedures such as banding or sclerosis. These treatments are designed to control bleeding from the varices. However, they do not correct the underlying problem. If your child’s symptoms do not improve after these initial therapies, surgical evaluation may be appropriate.
Your child may be referred for surgery after the initial diagnosis of portal hypertension or later after his symptoms are controlled. Many different surgical options are available and a specific treatment plan will be created for each patient, addressing their particular needs and anatomy. The following paragraphs will briefly discuss a few of these options and is not intended to be a complete list.
Portal hypertension surgery falls into two categories: palliative and restorative. Palliative shunts are effective in decompressing varices and reducing the symptoms of portal hypertension. These include the mesocaval and distal splenorenal shunts that have been performed for many years with high success rates. However, these shunts do not restore the nutrient blood flow to the liver.
The restorative shunt, the meso-Rex bypass — or “Rex shunt” for short — has been successfully performed in the US for the last 14 years, with success in over 90% of patients. In addition to relieving the symptoms of portal hypertension (bleeding and dilated varices), it restores blood flow to an otherwise normal liver and results in reduction in spleen size. 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.
Following the surgery, the symptoms of portal hypertension usually resolve very quickly. In addition, restoring normal portal blood flow to the liver allows the liver to recover some of its functions that may have been impaired, allowing the child to grow and develop to their full potential.
How long will my child stay in the hospital?
Children typically spend seven days in hospital after the surgery. Your child will spend 1 to 2 days in the pediatric intensive care unit (PICU) and then the remainder of the stay will be spent on the general inpatient 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. In some cases, oral blood thinning medications are continued after discharge from the hospital. After the children are eating, medicines are given by mouth to slow down clot formation. Prior to discharge, imaging studies (Ultrasound or CT scan) are ordered to confirm that the shunt is working. Children are usually sent home 1 week after surgery.
Routine blood tests and ultrasounds are repeated every three months and possibly a CT scan one year after the surgery. Most children can be monitored at their home centers and return to Galveston for their one-year follow-up. Some families prefer to come to the Children’s Hospital at UTMB for their check-ups, and we will do everything possible to make their stay as brief and pleasant as possible.
UTMB Pediatric Surgery
301 University Boulevard
Galveston, Texas 77555-0353
Phone: (409) 772-5666
FAX: (409) 772-4253
In April 2012, UTMB opened the doors of a new Children’s Hospital on the 10th floor of John Sealy Hospital. Get details or inquire about a tour.