Congenital Portosystemic Shunt (Abernethy Malformation)
What is Abernethy malformation?
Congenital portosystemic shunt, also called Abernethy malformation, is a rare problem with the blood vessels in the portal venous system. Children with this condition have an abnormal connection between blood vessels in the intestines and the body’s general blood circulation, where the blood does not go through the liver before going back into circulation.
Causes
Abernethy malformation occurs while a baby is growing during pregnancy. Children with Abernethy malformation will have the condition from birth, although it is not always immediately diagnosed.
Signs and symptoms
Abernethy malformation can be diagnosed at any age and impacts each child differently. Some children may have no symptoms, while others may have serious symptoms of liver disease, hepatopulmonary syndrome, or pulmonary hypertension. Children with Abernethy malformation often have problems with their heart, lungs, liver, and brain function that lead to a diagnosis of this blood vessel (vascular) condition.
Your child’s care team will examine them to determine the type of malformation and next steps. The exam usually includes a blood draw to check liver function and imaging of their belly.
Treatment
Treatment strategies depend on where the malformation is located, how severe your child’s symptoms are, and what other complications they have related to the condition. Many times, children need surgery to redirect the blood flow to the liver.
Dr. Bhargava Mullapudi and the vascular malformation team of experts often use one-staged or two-staged repairs based on the severity of your child’s condition. Sometimes, the team can do laparoscopic surgery, with only small cuts to your child’s belly.
What to expect
After surgery, you child will most likely stay in the hospital for a few days or until your doctor feels they are well enough to recover at home.
After surgery, you will have regular follow-up appointments with your child’s 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 watch to see if their liver is working better and has correct blood flow. They will also make sure any complications of Abernethy malformation that affected other body systems are getting better, too. 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.
Related programs and specialty clinics
Abernethy Malformation: Dawson’s Story
Dawson Berg kept having severe infections requiring surgery and other complex care. His family was told that liver transplant may be his only option, until the team at Children’s Mercy diagnosed him with Abernethy Malformation. After a shunt ligation surgery, he’s able to live a normal life, free from serious infections.
