“I feel just horrible, and no one knows what to do,” the 21-year-old woman sobbed to her father. In one hand, she held her phone, in the other, a red Solo cup. The pungent smell from the vomit-filled cup wafted through the room. Despite her best efforts, the strange lightness she felt when standing told her that she was dehydrated. And why wouldn’t she be? Everything she ate or drank came back minutes later in terrible heaves that tore at the aching muscles in her chest and abdomen. She filled the cup more than once during this call with her father. And maybe a dozen times earlier that day. And the day before. And the day before that.
She paced around the room as she listened to her father. “You need to go to the emergency room,” he told her. She didn’t want to go. She already went seven times over the past three months since this vomiting became part of her daily routine. Most of the time they just gave her IV fluids and sent her home. They thought it was her anxiety. She was admitted twice. Both times they ran countless tests, then sent her home to vomit there — without any answers.
Nevertheless, the woman took her father’s advice, and her roommate drove her to the Emory University Hospital emergency room in Atlanta. After getting some IV fluids and the anti-emetic Zofran, which hadn’t helped her in the past, she was discharged. She called her father as soon as she got back to her apartment, and he told her to come home to Cleveland. It was the week before Thanksgiving, and lots of flights were full, but she finally found one for that afternoon and packed her bag.
Sideswiped and Whipsawed
Just days after arriving in Atlanta that August to start her junior year at Emory University, she was in a car accident. Another car made an illegal turn and sideswiped hers, and she whipsawed against the door. She felt fine, though, and after they exchanged insurance information, she just went on with her day. But by the next day, she had started throwing up. Everything she ate or drank caused her to retch and vomit. She went to the E.R. Because the vomiting started right after her accident, the emergency-department doctor thought she had a concussion. He gave her some fluids and a medicine to stop the nausea. It should get better in a couple of days, he assured her. But it didn’t. She’d been vomiting every day since then. She felt fine until she ate or drank something — anything. Then, within minutes, she would have an overwhelming sense of nausea, and the wrenching spasms and vomiting would start.
The flight to Cleveland was quick. Her father picked her up at the airport and drove directly to the Cleveland Clinic Children’s hospital. Her regular doctor, Ellen Rome, the head of the Center for Adolescent Medicine there, wasn’t in the office that holiday week but arranged for the young woman to see a pediatric gastroenterologist. She immediately admitted her to the hospital.
The doctor who admitted her that night considered the possible causes of this kind of unremitting vomiting. The patient was taking medications for anxiety, so maybe the doctors in Atlanta were right — maybe this was psychogenic vomiting, caused by her longstanding psychiatric disorder. But there were other possibilities. Regular marijuana use could cause persistent vomiting. Hyperemesis gravidarum — excessive vomiting in pregnancy — was also possible. Those were easy to test for. Hyperthyroidism can cause this kind of vomiting as well. By the next morning results from the testing began to trickle in. She was not pregnant and had no evidence of marijuana in her system. Her thyroid was normal. So were the rest of the more routine studies.
That morning, Rome reached out to the team assigned to care for the young woman. When she was hospitalized at Emory, Rome explained, one of her scans showed an unusual finding. Her celiac artery, which provides blood to many digestive organs, was strangely narrowed, as if being compressed from the outside. That was suggestive of a rare disorder called median arcuate ligament syndrome (MALS), where the connective tissue that anchors the diaphragm to the spine — the median arcuate ligament — impinges on the celiac artery. Although usually characterized by severe abdominal pain, compression of that vital artery could cause the kind of nausea and vomiting she had by starving the downstream nerves and organs of adequate blood when they needed it most — right after eating.
Despite this abnormal scan, the doctors at Emory thought it was much more likely that she had some sort of anxiety-triggered vomiting than this rarity. Even so, they had suggested a specialized type of ultrasound to see if the compression was affecting blood flow through the artery. It hadn’t been done by the time the patient came to Cleveland. They needed to do it now, Rome said. The test was done the following day.
Scan After Scan
Using sound waves, Doppler ultrasound allows doctors to estimate how fast blood is flowing by measuring the rate of change in its pitch or frequency. The diaphragm moves upward when air is being breathed in, and so blood flow through the celiac artery would be normal or, if partly obstructed, faster than normal, the way water moving through a hose increases in speed when you use your thumb to partly block the opening. But when breathing out, the diaphragm moves down, and in MALS, this will reduce or even stop blood flow through the artery, depriving the targeted organs or nerves of the blood and oxygen needed to digest food.
The results of the ultrasound, while not completely normal, were inconclusive. The young woman was frustrated and confused. Part of her was glad there was nothing wrong. Another part worried because they still didn’t know what was going on. She had been in the hospital for three days at that point, and she still couldn’t eat or drink. Finally, the doctors put a tube through her nose into her stomach to provide her with some liquid nutrition and water. She would need to get back to school for classes on Monday, with the feeding tube still in operation. It was uncomfortable and she was embarrassed by it, but it certainly beat the alternative.
That day, Rome came to see her. “We need another CT,” she said. Despite the inconclusive ultrasound, Rome felt certain that the young woman had MALS. But Rome had only been able to read the report of the earlier CT. To make this diagnosis, the physicians needed to see the compression of the artery — they needed to repeat the study. On the day before Thanksgiving, the patient had the CT of her abdomen. During the scan, they took pictures when she inhaled and then again when she exhaled. These pictures finally gave them a conclusive diagnosis: She had MALS.
The next step was to inject the celiac plexus with an anesthetic. That nerve bundle was fed by the obstructed celiac artery. If the drug stopped the nausea and vomiting, that would indicate that surgery to loosen the ligaments could be curative. But that couldn’t be scheduled over the holiday. Instead, she went home to celebrate the day with her family. She had to watch as they ate turkey and stuffing but knew that even a single bite would send her rushing to the bathroom.
She went back to school for the rest of the term. Rome arranged for the gastroenterologist she wanted for the surgery to give the young woman the celiac nerve block over the Christmas holiday. After that injection, the patient could eat and drink for the first time in months, at least for as long as the block lasted. Her mother gave her all her favorite foods: sour cream and onion Pringles, a large iced coffee, ice cream. It was the best day ever. And it gave her hope that the surgery would cure her.
Her surgery was done in February. As soon as she woke up in the recovery room, she had a cup of apple juice. And she felt fine. Finally, it was over. She still doesn’t know what caused her to suddenly develop this rare disorder. It started after the car accident, and there are reports of MALS being caused by physical trauma — but she insists the accident really wasn’t a big deal. Was it enough to jostle her organs and realign her diaphragm and celiac artery? Maybe. Medicine is good at figuring out what a patient has and how to treat it. But the “why” often remains elusive.
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