The stomach pain 14-year-old Niki Karpouza was experiencing was so excruciating, she couldn’t eat, had to give up gymnastics and ultimately missed 350 days of school in her hometown on the island of Crete, Greece.
The culprit: a rare vascular condition that had led to the narrowing of multiple intestinal arteries and their branches, preventing blood from flowing to her intestines like it was supposed to.
Several doctor visits and medications in Greece didn’t help. Doctors there reached out to the University of Michigan’s C.S. Mott Children’s Hospital that has one of the leading multidisciplinary programs for comprehensive treatment of vascular disease. The program is an international referral center for children with complex vascular disease and handles approximately 80 percent of cases requiring surgery in the U.S.
“They told us our best chance to help Niki was to come all the way to Ann Arbor, Michigan,” Niki’s mom Stella Vogiatzidaki says. “We were just happy to know there was hope to make her better — even if it was so far away.”
The Mott team worked with Niki’s doctors to get governmental approval from Greece for her insurance to cover her care in Michigan. Earlier this year, Niki, along with her mother, father Panagiotis Karpouza and cousin Rena Avtzi made the trek to America. After a four-hour surgery at Mott, Niki woke up to find that the symptoms that had plagued her for the last year were gone.
Niki suffered from what’s called chronic mesenteric ischemia resulting from arterial dysplasia, a vast narrowing of blood vessels that restricts vital blood flow to organs.
“We continue to vigorously study this condition of arterial dysplasia but it’s still a mystery why some kids develop it. Niki’s case was one of the most extensive cases we’ve ever seen, and our team had many conversations about the best treatment options for her,” says Dawn Coleman, M.D., a vascular surgeon at C.S. Mott Children’s Hospital. “We were pleased that we were able to help Niki before her condition worsened and that we could send her home to Greece feeling so much better and able to get back to a normal life.”
Niki’s case was complex. She suffered from advanced arterial dysplasia affecting several vascular territories. She had developed renal vascular hypertension— high blood pressure caused by narrowed arteries not supplying proper blood flow to the kidneys— that responded well to angioplasty (balloon dilation to restore blood flow) in Greece. She also had a pelvic artery narrowing (iliac stenosis) resulting in poor blood flow to one leg that restricted long bone growth. Her shorter leg length has resulted in a curvature of the spine (scoliosis) that still requires treatment.
All three of Niki’s major intestinal arteries had narrowed due to her condition. Two of these vessels – the superior mesenteric artery that provides blood to the gut and the inferior mesenteric artery that supplies blood to the large intestine – had such extensive disease that they could not be repaired. However, surgeons believed they could operate on the celiac artery that carries blood from the heart to the forgut area including the stomach, liver and small intestine.
This artery appeared compressed by overlying nervous tissue and diaphragm muscle, a condition described as median arcuate ligament entrapment.
Surgeons treated the celiac artery narrowing by first dividing the surrounding nerve tissue and muscle fibers. The narrowed artery was then opened and repaired with a patch made from one of Niki’s own vessels, to make the artery wider
“I’m thankful that I feel no more pain,” Niki says.
Mott doctors say the surgery was critical to Niki’s long-term health. As Niki developed severe pain with every meal, she had developed a ‘food phobia’. This fear of eating would have ultimately resulted in extreme life-threatening weight loss and malnutrition. In addition, she was at risk for thrombosis or clotting of the intestinal arteries. This clotting could result in a lack of oxygen-rich blood that could have permanently damaged her intestines. This condition, known as acute mesenteric ischemia, can result in bowel death and is fatal in up to 80 percent of cases.
A multidisciplinary team worked on Niki’s case, including Coleman and fellow vascular surgeon Jonathan Eliason, as well as specialists in pediatric nephrology, interventional radiology, and critical care. Pediatric Social Work also offered support before, during, and following Niki’s stay by coordinating efforts to ensure the family’s travels, admission, and discharge back home went smoothly.
The Vascular Program at Mott draws patients from around the country and world, with less than 10 percent of its patients from Michigan, and many traveling from as far as Asia, South America, and Europe.
“We knew that there were no guarantees but that Mott was the best place to go,” Stella Vogiatzidaki says. “As soon as we got here and met the doctors, we knew everything was going to be OK. We are so thankful to Dr. Coleman and the rest of the Mott team for making Niki better.”
Take the next step:
• Learn more about the vascular program at C.S. Mott Children’s Hospital.
• Watch Elizabeth’s story about her comeback from renovascular disease.
• Read about baby Zofia’s journey with renal stenosis and hyptertension, as reported in the Detroit Free Press.
University of Michigan C.S. Mott Children’s Hospital is consistently ranked one of the best hospitals in the country. It was nationally ranked in all ten pediatric specialties in U.S. News Media Group’s “America’s Best Children’s Hospitals,” and among the 10 best children’s hospitals in the nation by Parents Magazine. In December 2011, the hospital opened our new 12-story, state-of-the-art facility offering cutting-edge specialty services for newborns, children and women.