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

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 tail

But 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 loss—bald 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.