Our patients’ stories: New surgical technique helps Cristian avoid transplant

Cristian

Typically, when people mention someone’s “big heart” it’s complimentary. But when doctors at Miami Children’s Hospital talked about the “big heart” of then two-month-old Cristian Colon, the phrase was nothing to smile about—it was the sign of a life-threatening illness.

During a routine check-up, Cristian’s doctors noticed the young boy had a heart murmur. The discovery prompted a trip to the cardiologist where it was revealed that his heart was enlarged, likely the result of midaortic syndrome. (Midaortic syndrome occurs when the aorta, the main artery that delivers oxygen-rich blood throughout the body, is narrowed or damaged.) Cristian’s aorta was so constricted it was straining his heart and severely raising his blood pressure by restricting blood flow to his kidneys (a condition known as renovascular hypertension.)

If left untreated for too long, Cristian’s kidneys would fail completely and he’d eventually need a kidney transplant.

He spent the next three weeks in the hospital, until doctors finally found the perfect balance of blood pressure medication to stabilize his small body. With the right medication Cristian was out of immediate danger, but the real question still loomed: How could doctors permanently fix his artery and correct the blood flow to his kidneys?

Most cases like Cristian’s would be treated with a stent (a small mesh tube that acts as a scaffold to open and support restricted arteries), or with a balloon angioplasty, where a tiny balloon is inserted in the artery and inflated, manually expanding the narrowed passage. Another option would be bypass surgery, where doctors would remove the damaged artery and replace it with plastic tubing.

Cristian and his mother Lucy

Unfortunately, all of these options would require multiple procedures for Cristian over the years—if ballooned, the artery would close naturally and need to be re-expanded; and any stents or plastic tubes used would need to be replaced as Cristian grew.

But Cristian’s Floridian doctors thought there might be another way to help their patient. They had heard of a technique being perfected by Heung Bae Kim, MD, director of Boston Children’s Pediatric Transplant Center, which could fix Cristian’s artery with minimal surgeries, and would require no stents or artificial tubes in his body at all.

It’s known as TESLA (tissue expander stimulated lengthening of arteries), and Kim has been perfecting it since 2010. The mechanics of TESLA are fairly simple: a tissue expander (a balloon-like device, about the size of a fist,) is placed underneath a healthy section of artery, downstream from the narrowed or damaged part, and is wedged between the aorta and a sturdy unyielding part of the body like the backbone. Once in place the expander is gradually filled with saline, inflating it like a balloon being filled with water. As it expands it comes between the backbone and the artery, forcing the flexible artery to stretch outward and grow longer.

After weeks of inflating the expander, the artery is stretched by several inches. The expander is then removed, as is the section of artery that is narrowed or damaged. The “extra” artery created by the TESLA stretching is then used to replace the removed, damaged section.

After the expander was inserted in Cristian, he and his family would come to Boston once every three weeks to have it filled with saline. Monthly travel from Florida to Boston Children’s added an extra hurdle to treatment, but in a few months Cristian would be left with a healthy artery, made of natural tissue, allowing perfect blood flow from his heart to his kidneys. No artificial grafts would be in his body and no follow-up surgeries would be required.

“The idea that the TESLA procedure would fix Cristian and prevent him from having to face a whole series of surgeries throughout childhood was something we felt very strongly about,” says Cristian’s mother Lucy. “Dr. Kim explained the procedure clearly, and I could tell he believed it would work. After talking with him we knew this was what we wanted for our child, no matter what.”

Dr. Kim and Cristian

Cristian’s parents were confident they made the right choice for their son, but the experience wasn’t without its struggles. Over the course of six months his expander came loose twice, requiring Kim to reattach it. At one point he was forced to replace the expander altogether, swapping it with a larger, better-designed model that would fit more securely in Cristian’s body.

But despite the setbacks, so far TESLA appears to have been a complete success for Cristian. His aorta was repaired and the expander was removed last month and since then his blood pressure has almost returned to normal as has his kidney function. Aside from routine check-ups to monitor his progress, Kim believes Cristian won’t require any more surgeries as he grows.

“I’m so glad Cristian won’t need multiple surgeries as he gets older,” says Lucy.

“The quality of life that this procedure will offer over the course of his life makes everything worth it. I can’t wait to see what the future holds for him.”

In some ways, Jayshia Lewis represents much of what Cristian’s future could look like. Jayshia first came to Boston Children’s in 2007 for treatment of midaortic syndrome, which had so severely cut off blood from her kidneys that one had shriveled into complete atrophy. In 2010 Jayshia met with Kim who at that point had conceived of TESLA, but had not met a patient it was appropriate for. After months of research and preparation Kim decided the then 3-year-old girl was a good candidate and she became the first person in the world ever to undergo the procedure.

After TESLA Jayshia Lewis is doing great

Now almost two years out from her TESLA, Jayshia is a happy, healthy 5-year-old who loves coloring and riding her bike. She still sees Kim for regular check-ups twice a year and he has been delighted with her progress. In fact, Jayshia’s recovery is going so well that  her case was recently reported on in the prestigious New England Journal of Medicine, because of its favorable outcome and previously unseen technique.

Allowing her daughter to be the first person in the world to have the TESLA procedure was an understandably difficult decision to make, but Jayshia’s mother Sabrina says she had faith that Kim’s innovative approach would eventually keep Jayshia from needing a kidney transplant.

“Everyone has had that moment of inspiration where they come up with a great idea,” she says. “But Dr. Kim’s moment of inspiration saved my daughter’s life and I’ll be forever thankful for that.”

For information about Boston Children’s Midaortic Syndrome and Renovascular Hypertension (MAS/RVH) Program, or to find out if TESLA may be an option for your child, visit their website and use the information provided in the Contact Us section.

Want to learn more? The Boston Globe published an in-depth article on Kim’s procedure, which can be found here.