Mackenzie Hild found relief for her median arcuate ligament syndrome (MALS) at UChicago Medicine
August 2, 2015
In her quest to become a doctor, a crucial step for Mackenzie Hild, 25, was to stop being a patient. This wasn't easy. It took six years, much of that time spent searching for a diagnosis, followed by a tussle with her health insurance provider, then a brief visit to the operating room.
Hild "always wanted to study medicine," she said. She particularly wanted to help the disadvantaged. She started early, working with the people most in need. As a teenager, in 2009, she spent the summer between high school and college volunteering on a medical-humanitarian mission to Africa. She helped care for children in a small rural village in Uganda, one of the poorest nations in the world. One-third of Ugandans live on less than $1.25 a day.
But soon after she arrived in Uganda, her stomach began to hurt every time she ate. Physicians call this postprandial abdominal epigastric pain. Slender to begin with, Hild lost almost 15 pounds. No infectious cause, the prime suspect, was ever found, so she blamed her anti-malarial medicine, doxycycline. She returned from Africa and stopped taking the medicine, but the pain never quite went away.
Her second trip, the summer after her freshman year at Harvard, was to rural Rwanda, where she worked with a Partners in Health team that brought together American clinicians and native traditional healers to integrate their approaches. She also helped a neonatologist develop a protocol for Partners in Health's three rural hospitals there. It became a model for the entire country.
But throughout the trip, she suffered profound postprandial pain. This time she lost 35 pounds -- one-third of her normal weight. Worse yet, the pain didn't ease up after she returned to the United States. Whenever she tried to eat, her stomach hurt for the next four hours.
"Once we were back in Boston, they put me in the hospital," she said. Specialists checked for all possible parasites, any digestive disease, eating disorders. "They gave me every test known," she said, but her doctors never pinpointed a cause. So they sent her home, to Nevada City, California, and her mother's cooking, to recuperate.
She took that year off from college. Unable to make the pain go away, her California doctors inserted a feeding tube into her stomach. That didn't help. Next they tried a "J-tube," which goes through the abdomen and into the jejunum -- farther down the digestive path. This approach takes liquefied food to the middle of the small intestine.
At last, the pain stopped. After that, all her nutrition came from fluids, dripping straight from a latex bag through a pump and into her intestine. She called the device her "jet pack."
This was inconvenient, but it stopped the pain. So Mackenzie, 21 years old, an ambitious second-year student majoring in anthropology at one of the world's most selective, demanding and competitive colleges, didn't eat for the next four years.
Giving up food, one of life's most consistent pleasures, has many downsides, some not immediately obvious. "If you don't eat or drink," she points out, "it's hard to go on a date."
She found other activities. She spent two summers helping reduce chronic disease on an Arizona Navajo Indian reservation. At college, she helped Boston's homeless community deal with their health issues. She graduated from Harvard in May, 2014.
"I have no pain when I eat, for the first time in 5 years. I am so so happy."
After graduating, she found a way to get back to Africa. Her Boston-based gastroenterologist -- Dr. Norton Greenberger at Brigham and Women's Hospital -- made arrangements so that Hild could spend seven months in South Africa, where she studied the impact of the violence and trauma caused by apartheid and how it affected women and children's health.
Meanwhile, she continued to research her own health challenge. The more she knew, the more it seemed to resemble a little-known ailment called MALS, short for median arcuate ligament syndrome. First "observed" in 1917, MALS -- also known as celiac artery compression syndrome or Harjola-Marable syndrome -- has been rediscovered, re-described (1965, 1967) and forgotten so many times that it goes by multiple names.
Diagnosis typically follows a process of exclusion -- meaning doctors try a variety of imaging methods and other tests, hoping to find something familiar. If that fails, they consider MALS.
The disorder results from a tiny anatomical flaw. In a very crowded region of the upper abdomen, one of the parts is a little out of place. The median arcuate ligament, a band of fibrous connective tissue, extends from the base of the diaphragm, crosses over the aorta just above the spot where the celiac artery branches off, then reconnects to the diaphragm on the other side.
In some people, perhaps as many as ten percent, this ligament sits a little lower than it should, compressing the celiac artery. In about one percent of that ten percent, this compression of the artery can cause significant chronic abdominal pain.
It is not clear why. Some physicians suspect that it comes from the reduced blood flow to much of the stomach, liver, lower esophagus, spleen and parts of the duodenum and pancreas. Others think it stems from compression of the nerves in the area. Still other physicians don't believe the compression causes pain at all; there must be another cause. Unfortunately, no matter what the cause, patients experience gut-wrenching pain that lasts for hours. Over time, it can do serious damage.
As her multiple test results piled up and Hild's various doctors ruled out the usual suspects, they began to consider MALS. Testing for MALS, however, is not simple. Hild was worked up for MALS at three different institutions, but it seems that "nobody did it in quite the right way," she said.
Fortunately, when she returned to California after graduation and her time in South Africa, Hild enlisted a fourth-year medical student advocate at the University of California at San Francisco to help figure out her abdominal pain. The advocate contacted vascular specialist Christopher Skelly, MD, associate professor of surgery and head of the MALS team at the University of Chicago, one of the largest such programs worldwide. After some discussion, she sent him Hild's images and test results.
"I received an unassuming email last December titled 'GI mystery case' from Mackenzie's advocate," Skelly recalled. "My first thoughts were to figure out if this was a real patient or a student was trying to get extra help on a classroom assignment."
It turned out to be a very real case.
The classic sign of MALS is a small kink in the celiac artery -- visible on a three-dimensional CT angiogram, and confirmed by ultrasound imaging of blood flow through the artery -- but it's not always apparent. Skelly studied the images carefully and was convinced Hild had MALS and could probably be cured with surgery.
He warned her, however, "your pain may not go away." There is not yet a reliable way to predict how a patient, even one who meets all the diagnostic criteria, will respond to the operation. The standard workup not only includes a visit with a gastroenterologist but also a psychiatrist.
"About 70 to 80 percent of our patients have an excellent outcome," Skelly said, "but we can't always tell in advance who the lucky ones will be." For Hild, however, he felt the odds were better, "more like 85 percent."
This sort of talk, about odds and predictions and uncertainty, grabs the attention of the health insurance industry. Skelly sent Hild's insurance provider her test results, long-term follow-up from previous cases and various publications on MALS diagnosis and treatment. He pointed out why treating it once can be less costly that dealing with the problem indefinitely. Meanwhile, the Hild family spent hours on the phone, chasing approval, day after day. By the time Mackenzie and her mother arrived in Chicago, on March 17, 2015, the issue was "pretty much resolved," Skelly said.
The next morning Makenzie and her mother walked into the University of Chicago's Comer Children's Hospital, where the MALS operation is performed. (Most patients are diagnosed earlier, usually in the middle teen years. Nine out of ten are female.)
The surgical team -- led by Skelly and pediatric surgeon Grace Mak, MD, with help from a surgical resident, two surgical nurses, an anesthesiologist and an ultrasound technician -- confirmed every imaginable detail and repeated a few tests. After a poignant goodbye-good luck with her mother, Hild was rolled into the OR.
The operation, formerly done through a big incision, is now performed laparoscopically, through five small abdominal punctures: two for surgical tools, two for the retractors, and one through the belly button for the biggest item, a high-resolution camera.
Hild's procedure went smoothly; the team has done this more than 150 times. Some adhesions, from a previous operation to remove her gall bladder, delayed the process slightly. The only surprise was some funny looking pale spots on the surface of Hild's liver, probably a consequence of years of exposure to a high-fat low-fiber diet through the J tube. Those should quickly disappear.
The operation is technically simple, but complicated by the endless variety of individual anatomies. Two surgeons, Skelly and Mak, worked together to "take down" the median arcuate ligament using tiny electro-cautery tools, which can cut soft tissues without blood loss. They removed the connective and lymphatic tissues above, around and below the celiac artery. Once that was complete, they used Doppler ultrasound to assess blood flow though the now unencumbered celiac artery. The difference was obvious.
The next morning, patients are offered clear liquids. Later that day, Hild ate a few bites of scrambled eggs, her first meal in more than four years. She was a little out of practice, she admitted. Her stomach felt strange, initially uncomfortable -- it had not been fed for years -- but the mild malaise was a vast improvement over the acute pain she felt when she last ate. And after four years of tube feeding, even hospital food tasted fine.
Over the next few weeks, as her body relearned how to eat, the pain vanished. On May 20, about two months after the procedure, Hild emailed: "I have no pain when I eat, for the first time in 5 years. I am so so happy."
"This is such a relief," she added. "After years of being told that nothing is wrong, it's nice to know you're not crazy."
Still, there were benefits from her former illness. "Having to carry my jet pack internationally, plus weeks or months worth of liquid food wherever I traveled, taught me a lot about planning," she said. "Just getting through security could be a struggle. Now I leave myself extra time."
She began medical school, at University of Washington, in August.
Christopher Skelly, MD
Christopher Skelly, MD specializes in vascular surgery. He performs a full range of open and endovascular procedures, including the treatment of carotid disease, complex aortic pathologies and peripheral vascular disease.Read more about Dr. Skelly
Grace Mak, MD
Dr. Grace Mak has expertise in providing surgical care for children of all ages. She specializes in the surgical treatment of neonatal diseases and congenital malformations, anorectal malformations, inflammatory bowel disease, and pediatric oncology.Learn more about Dr. Mak