Dangling danger Surgeons remove 2.5-inch hairball from teen with rare Rapunzel syndrome The teen didn't return for follow-up. Instead, she planned to see a hypnotherapist. Beth Mole Nov 21, 2024 5:02 pm | 32 Credit: Getty | Ada Summer Credit: Getty | Ada Summer Story textSizeSmallStandardLargeWidth *StandardWideLinksStandardOrange* Subscribers only Learn moreAfter 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.Hairy detailsThen there were the rarer causesmechanical 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's 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).Hair is resistant to digestion and isn't easily moved through the digestive system. As such, it often gets lodged in folds of the gastric lining, denatures, and then traps food and gunk to form a mass. Over time, it will continue to collect material, growing into a thick, matted wad.Of all the bezoars, trichobezoars are the most common. But none of them are particularly easy to spot. On CT scans, bezoars can be indistinguishable from food in the stomach unless there's an oral contrast material. To look for a possible bezoar in the teen, her doctors ordered an esophagogastroduodenoscopy, in which a scope is put down into the stomach through the mouth. With that, they got a clear shot of the problem: a trichobezoar. (The image is here, but a warning: it's graphic).Tangled tailBut this trichobezoar was particularly rare; hair from the mottled mat had dangled down from the stomach and into the small bowel, which is an extremely uncommon condition called Rapunzel syndrome, named after the fairy-tale character who lets down her long hair. It carries a host of complications beyond acute abdominal pain, including perforation of the stomach and intestines, and acute pancreatitis. The only resolution is surgical removal. In the teen's case, the trichobezoar came out during surgery using a gastrostomy tube. Surgeons recovered a hairball about 2.5 inches wide, along with the dangling hair that reached into the small intestine.For any patient with a trichobezoar, the most important next step is to address any psychiatric disorders that might underlie hair-eating behavior. Hair eating is often linked to a condition called trichotillomania, a repetitive behavior disorder marked by hair pulling. Sometimes, the disorder can be diagnosed by signs of hair lossbald patches, irritated scalp areas, or hair at different growth stages. But, for the most part, it's an extremely difficult condition to diagnose as patients have substantial shame and embarrassment about the condition and will often go to great lengths to hide it.Another possibility is that the teen had pica, a disorder marked by persistent eating of nonfood, nonnutritive substances. Intriguingly, the teen noted that she had pica as a toddler. But doctors were skeptical that pica could explain her condition given that hair was the only nonfood material in the bezoar.The teen's doctors would have liked to get to the bottom of her condition and referred her to a psychiatrist after she successfully recovered from surgery. But unfortunately, she did not return for follow-up care and told her doctors she would instead see a hypnotherapist that her friends recommended.Beth MoleSenior Health ReporterBeth MoleSenior Health Reporter Beth is Ars Technicas Senior Health Reporter. Beth has a Ph.D. in microbiology from the University of North Carolina at Chapel Hill and attended the Science Communication program at the University of California, Santa Cruz. She specializes in covering infectious diseases, public health, and microbes. 32 Comments Prev story