What's Rapunzel syndrome? Definitely no fairy tale

When a middle-aged woman came to doctors with complaints of vomiting every time she tried to eat and months of unexplained weight loss, they were baffled at first. But eventually she was diagnosed with a condition cat owners might be familiar with—a hairball in her belly.

In humans, the disturbing and rare disorder is called Rapunzel syndrome—named after the long-haired heroine in the fairy tale by German storytellers the Grimm brothers. It comes about from sucking on one’s own hair and swallowing it. Over time, the hair builds up in the digestive tract and clogs it.

Yale child psychiatrist Dr. Robert King said it’s a complication of trichotillomania—a hair-pulling disorder in which the person compulsively pulls out their own hair. It’s mostly seen in children and adolescents and can involve hair on any part of the body, including the scalp, eyebrows and eyelashes.

“Some kids pull their hair out and mouth the hairs and of those, a small number even swallow them,” King, an American Psychiatric Association member, told CBS News.

In the case described in this week’s BMJ Case Studies, a 38-year-old woman from Pakistan was experiencing nausea, sudden vomiting and constipation, and her abdomen was enlarged due to gas and fluids. The authors, from Khyber Teaching Hospital in Peshawar, Pakistan, and the University of Arizona, wrote that she had been suffering with some of her symptoms for as long as a year.

When an array of tests offered few clues, surgeons decided to operate and discovered a huge hairball in her stomach measuring 15 by 10 centimeters. A small tail extended beyond the stomach into the beginning of the intestines, and a second hairball, four by three centimeters wide, was discovered further down in the intestines. Both hairballs were removed and the patient left the hospital six days later.

Study author Dr. Faiz Anwer, director of the Adult Blood and Marrow Transplant Program at Banner - University of Arizona Medical Center, told CBS News, “This patient suffered with trichotillomania for many months and was diagnosed very late with severe complications of Rapunzel syndrome.”

King, who was not involved with the current case study but who has seen patients with the condition and has published research on it as well, said the behavior is seen in about one to two percent of children, but it’s less common in adults.

In children he said, “Very often it’s a form of self-soothing. It’s associated often with kids who have some anxiety.”

The woman described in the new case study received psychiatric counseling and was given nutritional support.

The researchers also reviewed 88 previous cases, noting that other serious complications are linked with Rapunzel syndrome, including pancreatitis, stomach ulcers, anemia and peritonitits—an inflammation of the membrane that lines the inner abdominal wall and covers the organs in the abdomen.

“Almost all cases reported in the published medical literature from all over the world regarding this disease required treatment with surgery,” said Anwer who is also assistant professor of medicine in the Division of Hematology, Oncology. He hopes their study will encourage doctors and patients to address the illness earlier if they suspect it exists.

In children, King said hair pulling can be “very chronic” and difficult to treat, but a very specific form of cognitive behavioral therapy, called habit reversal therapy, can help.

“The public health message is, if your kid is pulling hair out to the point of bare spots, it’s a cause for concern,” said King.