Abernethy Malformation: Dawson’s Story
Dawson Berg spent his 18th birthday skiing with family and friends in Colorado. It’s supposed to be an annual spring tradition, but they’ve often had to cancel the trip due to hospital stays. Dawson had 13 surgeries by the time he was 17, most for seemingly unrelated infections. His Children’s Mercy Kansas City team finally found a solution to give Dawson his active life back: a complex liver surgery that is only available in a handful of hospitals in the U.S.
“Looking back, I feel like there were some signs that there was a liver issue,” said Andrea Berg, Dawson’s mom. When he was born, Dawson’s face was purple and bruised. He stayed in the NICU for a week because his bilirubin levels were too high, but they resolved, and he grew into a healthy toddler. When he experienced unusual hair growth at 5 years old, Dawson’s parents took him to his pediatrician for blood work. She found elevated protein enzymes and referred them to Children’s Mercy for a liver ultrasound.
“That’s when they saw that it didn’t look like blood was going through the portal vein to the liver,” remembered Andrea, “and he was diagnosed with Abernethy Malformation.”
The underlying cause
Abernethy Malformation is when the portal vein — one of the main vessels that takes blood from the intestines to the liver — bypasses the liver to the inferior vena cava, sending blood right to the heart. Instead of being processed by the liver first, the blood goes through the rest of the body, eventually working its way back to the liver for delayed filtering.
Dawson’s care team here helped slow his premature growth, monitored his condition and cautioned his family to tell them if he began experiencing jaundice or shortness of breath. At that time, the only option for treatment was a liver transplant, but he’d have to be very sick to qualify for one.
There's another less well-known symptom of Abernethy Malformation: “When the blood from the intestines doesn’t immediately go to the liver and is passed around the body, there’s an increased risk for different types of rare infections,” explained Ryan Fischer, MD, Chief of Hepatology and Transplant Medicine, who took over Dawson’s care in January 2022 when his previous physician retired.
But when infections began happening to Dawson, it wasn’t clear that his liver physiology was to blame. There was always another more immediate culprit.
In fifth grade, Dawson had a severe staph infection that required surgery on his tibia and knee. They thought it was caused by a cut he had gotten roughhousing with his brother, Hudson. In 10th grade, Dawson’s teeth were injured during a basketball game. A month later, he started having flu-like symptoms, and his eye started to swell.
“I thought I was allergic to something because my eye was swollen shut, and I’d never experienced anything like that before,” said Dawson. “I had no clue what was going on.”
After urgent care and primary care visits, they went back to Children’s Mercy, where tests revealed an infection had spread to his eyes, sinuses and brain. He had seven surgeries in less than two weeks to drain the infection. (And later, when his forehead wasn’t healing properly, he had a 3D-printed skull plate inserted as well.)
“I told Dr. Fischer that when Dawson was five, we were told the only cure was a liver transplant,” said Andrea. “Obviously, we don’t want a liver transplant. He doesn’t seem to be sick now, but he’s having all these surgeries, and I’m worried about him.”
After immune testing didn’t show any abnormalities, Dawson’s team began considering how Abernethy Malformation may be contributing to his illnesses — and what they could do to help.
An uncommon solution
In 2022, Bhargava Mullapudi, MD, Division Director, Transplant Surgery, was recruited to Children’s Mercy to direct the pediatric liver transplant program. He also specializes in complex hepato-pancreato-biliary surgery, vascular-related surgery and complex tumor resections.
“When Dr. Fischer talked to me about Dawson, I could see the clear problem,” said Dr. Mullapudi. “He’s an awesome teenager. He just wanted to get back to his life. He was having these recurring admissions, and he’s starting to feel like, ‘Is this something that’s going to be chronic?’”
Some patients with Abernethy Malformation eventually develop nodules, scarring and even cancer in their livers. But Dawson’s liver was healthy so far; the blood just wasn’t getting to it in the right order and was picking up infections along the way.
Fortunately for Dawson, and other kids like him, Children’s Mercy is one of the few pediatric hospitals in the country that can offer another solution now: a shunt ligation, a complex liver surgery to close the shunt and restore normal blood flow to the liver.
“This is the beauty of our institution and our Liver Care team: We focus on pediatrics day in and day out,” said Dr. Mullapudi. “We can think outside the box.”
Dr. Mullapudi and Richard Hendrickson, MD, FAAP, FACS, pediatric transplant surgeon and Director of Intestinal Rehabilitation, gathered a multidisciplinary team — hepatologists, surgeons, interventional radiologists and the ICU — to make a surgical plan. After a liver biopsy and imaging confirmed his liver was healthy and there was some preserved blood flow to work with, they decided a two-stage surgery was Dawson’s best option. Stage one: Partially close the shunt and give the liver’s blood vessels a few days to adjust to the increased blood flow. Stage two: Finish the closure.
Drs. Mullapudi and Hendrickson performed the two-part surgery in October 2022.
A beautiful outcome
“Now the liver is getting beautiful blood flow, it’s getting all the nutrients it needs, and it’s doing its job as a metabolic factory and filter,” said Dr. Mullapudi. “Dawson was a completely different person when I met with him post-operatively. He just had the sparkle back in him.”
His lab results sparkled, too: His ammonia levels came down to healthy levels within the week. (Ammonia, a waste product of food digestion, is elevated when blood is shunted around and not immediately processed by the liver.) Later ultrasounds confirmed that his portal vein looks normal, and his liver blood flow is excellent. He was on six months of blood thinners to make sure those adjusting blood vessels didn’t clot and has been medication-free ever since. Moreover, he has not had a single life-threatening infection since the shunt ligation.
“After a little bit of time, I felt more energized,” said Dawson, of the surgery’s impact. “I get up a little earlier and feel like I’m more prepared for the day. I just feel better.”
Dawson will have annual follow-ups, but that frequency could taper off in adulthood if his liver continues to function well. Since the surgery, Dawson has become a state-competitive bowler and still likes to play pickup basketball with his buddies, too. He’s going to study mechanical engineering at Kansas State University this coming fall, where he’ll bowl with the K-State club.
He encourages other patients with Abernethy Malformation to consider shunt ligation surgery if possible.
“It’ll change your life for sure, whether you know it or not,” he said. “It’s made my quality of life a lot better. I would 100% recommend getting it if somebody has the option.”
“I don’t feel like we’re looking for something wrong all the time now...this is really life-changing for him and our family,” said Andrea, recounting all the time they had spent in hospitals in the past decade. “We are happy he’s healthy now and very thankful for the doctors at Children’s Mercy over the years!”