Ashlyn Frye hadn’t been feeling her best for the last several years. Teachers said she didn’t pay attention in class and would crawl under her desk to take a nap. At home, the 11-year-old seemed disconnected, says her mom Stacey Frye. “I was used to her being out of breath and tired,” Stacey recalls. “Just low energy. She wore out quickly. She’d just hang out and watch shows or read.” After a series of medical tests, Rady Children’s doctors determined that Ashlyn had celiac disease and portal vein thrombosis, a blockage of the vein that transports blood from the gastrointestinal tract to the liver. The hypersensitivity to gluten could be managed with diet, but the portal vein thrombosis would require intensive surgery to address the blood clot preventing her liver from processing blood. The Fryes were referred to a medical facility in San Francisco, but consenting to a complex procedure on their young child seemed grim. Then they learned Rady Children’s had a new doctor, Henri Justino, MD, who was exploring innovative catheter-directed therapies, including one that might work for Ashlyn’s portal vein thrombosis.
In October 2022, Ashlyn became the first pediatric patient to undergo portal vein recanalization at Rady Children’s Dickinson Image-Guided Intervention Center. The hyper-sensitivity to gluten could be managed with diet, but the portal vein thrombosis would require intensive surgery to address the blood clot preventing her liver from processing blood. Stacey says the results have been nothing short of miraculous.
The standard treatment for portal vein thrombosis in children involves surgically closing off less-desirable paths, rerouting that “traffic” to other major veins in the body and perhaps directing that blood back to the correct vein in a location past the blockage. However, Dr. Justino explains that the bypass method doesn’t filter the blood as intended and doesn’t address the clot. “It is really not a cure because the fundamental problem remains that there’s a portal vein blockage,” he says. “Ashlyn has so much more energy,” she says. “Ever since then, she won’t slow down.” Ashlyn is now interested in soccer and basketball. With a few precautions, she can run, jump and play like other children her age.
UNDERSTANDING PORTAL VEIN THROMBOSIS
The portal vein is a blood vessel that delivers blood to the liver from the intestines, stomach, spleen and pancreas. When that vein narrows or is blocked by a blood clot, the patient will likely experience abdominal pain and nausea, and suffer an enlarged liver or spleen. Dr. Justino likens Portal vein thrombosis is the number one cause of major gastrointestinal bleeding in children.
A NEW APPROACH
Dr. Justino is an interventional cardiologist skilled in treating cardiac and vascular conditions, such as clotting. Before he joined Rady Children’s in 2021, he directed the catheterization laboratories at Texas Children’s Hospital in Houston. He brought with him hands-on expertise in a technique he calls portal vein recanalization. The minimally invasive procedure involves using real-time magnetic resonance the portal vein to a major highway. A blood clot is a messy car accident that backs up traffic for miles. “The portal vein is the main vein that brings blood into the liver,” he explains. “And when that vein gets blocked, the blood that should be going into the liver ends up having to find other ways to flow around the liver or through other paths and ends up causing some significant complications for patients. Portal vein thrombosis is the number one cause of major gastrointestinal bleeding in children,” Dr. Justino says. Portal vein thrombosis in adults is often caused by a tumor. If the tumor can be removed or moved aside, blood flow is restored.
In children, the blockage may have begun when they were newborns. An umbilical venous catheter—used to deliver fluids and medicines to premature babies or those with bowel problems—can cross with the portal vein and cause a clot that’s not discovered for years. Portal vein thrombosis in children can also be caused by a clotting disorder, infection or injury. And in many cases, including Ashlyn’s, the origin of the blockage is never fully determined. imaging to run a small wire and then a balloon-tipped catheter to the portal vein to dig through the blockage. The balloon is then expanded to stretch the site of the clot so blood can flow again. If the vein is prone to collapsing, a stent is placed. “It’s like getting in there and removing the accident, clearing the traffic jam and letting the blood flow through the natural highway, which is what it is meant to do,” Dr. Justino says.
Pediatric patients who’ve undergone this procedure start feeling better soon after. Dr. Justino has spoken at medical conferences and published scientific papers on his work. “We see immediate results. You can see right away that the flow is now appropriately going into the liver,” Dr. Justino says. In Ashlyn’s procedure, Dr. Justino successfully cleared the clot and expanded the vein. “With her ultrasound before, we couldn’t see any blood going into the liver. And now we can see a nice vein that’s opened up, that is draining blood into the liver the way it should,” he says. “I feel like she’s had a very good result.” conditions. Just two centers—his former Texas institution and the Dickinson Center at Rady Children’s—are currently using portal vein recanalization to treat pediatric portal vein thrombosis. Ashlyn stayed in the hospital overnight for observation and ultrasound scans. She was sore but walking and able to go home the next day. Her monthly follow-up appointments have shown that she is thriving.
Pediatric patients who’ve undergone this procedure start feeling better soon after. Ashlyn and her mom, Stacey, are thankful that Rady Children’s found a successful portal vein thrombosis treatment Parent outreach is vital for spreading word of the latest healthcare innovations, he says. Families are educated about the risks and benefits of any procedure, but newer, lesser-known medical practices require more detailed explanation. “I think it’s really important for families— especially if they’re being told about something somewhat new, somewhat innovative—to really help them partner with us in this,” he says. “I view them as partners because they must not just consent to a procedure but they have to be willing to go through something that’s novel.” Stacey says the portal vein recanalization made sense for her daughter because it was less invasive than the traditional treatments available. Even if the blood clot couldn’t be cleared, she was optimistic that MRI images would give doctors a better picture of what was happening inside Ashlyn’s portal vein.
The Fryes are grateful Rady Children’s Hospital physicians came together to find a treatment that worked for Ashlyn. “It’s the best outcome we could hope for,” Stacey says. “I just hope this will help another child, to give them the freedom from a lifetime of hospitals and procedures.” Ashlyn’s mom says that though a thrombosis recurrence is possible, any future fix could be as straightforward as stretching the vein again as Ashlyn grows.
LATEST ADVANCEMENTS
Rady Children’s Dickinson Image-Guided Intervention Center is using MRI technology and catheter procedures to less invasively treat children’s hearts, lungs and brains. Dr. Justino and his interdisciplinary colleagues are brainstorming how these newer approaches can help sick children experience fewer intrusive surgeries. “The portal vein has been for a long time a little bit more challenging to get to because we don’t just go in through a standard access in the groin or in the neck. We have to puncture the spleen with the needle, and that has its challenges,” Dr. Justino says. “Familiarity with these techniques is something that not everyone has learned, but that’s something that we can learn. We’re all teachable.”