Surgeons remove 2.5-inch hairball from teen with rare Rapunzel syndrome

After a month of unexplained bouts of stomach pain, an otherwise healthy 16-year-old girl arrived at the emergency department of Massachusetts General Hospital actively retching and in severe pain.

A CT scan showed nothing unusual in her innards, and her urine and blood tests were normal. The same was found two weeks prior, when she had arrived at a different hospital complaining of stomach pain. She was discharged home with instructions to take painkillers, a medication for peptic ulcers, and another to prevent nausea and vomiting. The painkiller didn't help, and she didn't take the other two medications.

Her pain worsened, and something was clearly wrong. When she arrived at Mass General, her stomach was tender, and her heart rate was elevated. When doctors tried to give her a combination of medications for common causes of abdominal pain, she immediately vomited them back up.

So, her doctors set out to unravel the mystery, starting by considering the most common conditions that could explain her abdominal pain before moving on to the rarer possibilities. In a case study recently published in the New England Journal of Medicine, doctors recounted how they combed through a list that included constipation, gastritis, disorders of the gut-brain interaction, delayed stomach emptying brought on by an infection, lactose intolerance, gall bladder disease, pancreatitis, and Celiac disease. But each one could be dismissed fairly easily. Her pain was severe and came on abruptly. She had no fever or diarrhea and no recent history of an infection. Her gall bladder and pancreas looked normal on imaging.

Then there were the rarer causes—mechanical problems. With tenderness and intermittent severe pain, an obstruction in her gut seemed like a good fit. And this led them to one of the rarest and unexpected possibilities: Rapunzel syndrome.

Based on the girl's presentation, doctors suspected that a bezoar had formed in her stomach, growing over time and intermittently blocking the passage of food, causing pain. A bezoar is a foreign mass formed from accumulated material that has been ingested. A bezoar can form from a clump of dietary fiber (a phytobezoar) or from a glob of pharmaceutical products, like an extended-release capsule, enteric-coated aspirin, or iron (a pharmacobezoar). Then there's the third option: a tangle of hair (a trichobezoar).