Think Like a Doctor: A Terrible Stomachache Solved!

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On Thursday we challenged Well readers to figure out why a 43-year-old woman had such terrible abdominal pain that she lost more than 10 percent of her original body weight over the course of just a few weeks. There were two important clues in her case. First, her pain came on after she ate. And second, the pain improved when she brought her knees up toward her chest. More than 250 of you responded, but only five of you got it right.

The correct diagnosis is:

Median arcuate ligament syndrome, or MALS — also known as Dunbar syndrome or celiac artery compression syndrome.

The first person to solves this puzzle was Dr. Adrian Budhram, a third-year neurology resident at Western University in London, Ontario. This is Dr. Budhram’s second win. He said that when the Mayo Clinic doctor started looking for a bruit at the site of the pain, he remembered this unusual diagnosis.

The Diagnosis

MALS is a rare disorder, affecting mostly middle-aged women, and is characterized by nausea and excruciating abdominal pain, usually after eating.

First described in 1917, then decades later in 1963, MALS is a condition in which the medial arcuate ligament, the fibrous structure connecting the right and left halves of the diaphragm muscle, compresses the celiac artery, reducing blood flow to the stomach and intestines. It was thought that the pain came primarily after eating because that’s when demand for blood and oxygen by the gut is the greatest.

However, MALS a very controversial diagnosis. Some surgeons don’t believe it even exists — or at least as a cause of significant pain. The controversy stems from two observations about the disorder.

First, this same anatomy, where the medial arcuate ligament cuts across the celiac artery causing some compression, has been found, in autopsies, in many people who had no history of abdominal pain.

Moreover, the surgical repair of the problem, in which the ligament is cut away from the artery, doesn’t always get rid of the pain. The operation is successful, on average, only 70 percent of the time. If compression of the artery was the cause of the pain, then eliminating the compression should get rid of it. And it does sometimes, but not always.

New Ideas About the Stomach Pain

A new theory on the problem was suggested 20 years ago and is slowly gathering acceptance. This theory posits that the pain of MALS isn’t caused by a lack of blood to the gut but instead is a result of a loss of blood to and direct injury of the celiac ganglion, the nerve bundle that wraps around the celiac artery in the upper abdomen. Proponents suggest that for full resolution of the symptoms, these injured nerves and the nerve bundle must be cut away along with the compressive part of the ligament.

MALS is considered a diagnosis of exclusion — in part because there is no good test for it. The compression of the artery can be seen on CT and M.R.I., especially while the patient is exhaling. And an ultrasound can show the abnormality in blood flow from this compression. However, we know from autopsy studies that there are people with this anatomy who have no pain. So anatomy alone is not enough.

Other possible causes of pain must be ruled out to be certain that the pain is caused by MALS. And given the 70 percent success rate, some surgeons argue that even that is not enough.

Those who accept that nerve damage is an important component of the disorder sometimes use a different test to identify those who might benefit from surgery. They inject a numbing medicine directly into the celiac ganglion. If that completely relieves the pain, then these surgeons believe that the likelihood that the surgery will succeed is greater so long as the damaged nerves are removed during the surgery.

How the Diagnosis Was Made

This 43-year-old woman had an extensive work-up for the nausea and abdominal pain that frequently came on after eating. She was seen by several gastroenterologists.

Doctors at the Mayo Clinic in Rochester, Minn., finally gave her the diagnosis of gastroparesis, in which the passage of food through the gut is abnormally slow, causing nausea and obstruction. The cause of this slowed movement was not clear, but most of the time the cause of gastroparesis is never discovered. Sometimes the condition resolves on its own. Often, however, it does not.

There are no great treatments for gastroparesis. Patients with this disorder are told to eat frequent small meals and take medicines to stop nausea. If that doesn’t work, they can be put on a liquid diet or prescribed medications that make the intestines move faster. If none of that works, patients are fed through a tube that delivers nutrients directly to the small intestines.

Unsuccessful Treatments

The patient tried all of these treatments. Nothing really worked — not even the tube directly into her intestines. So, the woman ate small bits of food and drank supplements. She took a medicine to relieve the nausea. All that was helpful. But nothing seemed to help the pain that usually started an hour or two after she ate.

The patient and her husband returned to Mayo twice more to try to find the best treatment, but ultimately they resigned themselves to the fact that this was their life now.

Over the next year they consulted other experts, but no one had any answers. And the patient found herself spending more and more time in bed, too weak and tired to work at being a mother, or a wife or the lawyer she used to be.

A Call From a Friend

About a year later, the patient got a phone call that changed her life. Lois Abrams, a friend of her mother’s and someone she’d known since she was 10, called to share something she’d heard. She’d followed the patient’s story through her mother and knew she was suffering. Recently she’d heard about a similar story, but with a much better ending.

She had a friend, Lois explained. And her friend’s niece had something that sounded a lot like what she had. This other young woman also had terrible pain after she ate. Pain so terrible she could hardly eat and was wasting away to just nothing. And she’d also been given a diagnosis of gastroparesis. But that’s not what she had, the woman said excitedly.

And she got an operation and now she’s just fine. You should talk to her. She hadn’t been able to eat for years. Got her food through a tube that went straight into her intestines. And now she’s eating just like everyone else.

So what did she have, the sick woman asked? She had something called MALS, or median arcuate ligament syndrome, Lois announced triumphantly.

A Surgical Test

The patient called the young woman at the center of this remarkable story. Their symptoms matched exactly. She got the name of that woman’s surgeon, who specializes in this rarity, and made an appointment to see him.

From this surgeon she learned about the injection of the numbing medication into the nerve bundle, then underwent the test. She watched as the long needle was inserted right in the spot where she always had her pain. The relief was instantaneous. And in the six pain-free weeks the followed she regained most of her lost weight.

How the Patient Fared

She ended up having surgery a few months later with Dr. Richard Hsu, a surgeon in Danbury, Conn. It was a tough procedure, with an incision that ran from the lower edge of the rib cage down to just a couple of inches above the navel.

The patient took her first few bites of food a few days later. No pain. She’s had no pain at all in the 10 months since.

Most of the time it is a doctor and not a friend who makes the diagnosis in this column. I suspect that in life this is a more common event than in these pages. Lois Abrams is modest about the role she played in helping her friend’s daughter get her health, and her life, back. She says she was just lucky that she heard about these two women and their symptoms. “I just put two and two together.” But of course she’s thrilled to know she made a difference.