Rapunzel syndrome, although its name sounds romantic, is a rare disease caused by trichotillomania, i.e. pulling out one's hair and trichophagia - eating the torn hair. Rapunzel syndrome is one type of intestinal obstruction and can, in extreme cases, be fatal. Read about the causes of Rapunzel syndrome, how it manifests itself and how it is treated.

Rapunzel's bandtook its name from the heroine of the Grimm brothers' fairy tale - locked in a tower by Rapunzel's witch, who had very long and beautiful hair. It was thanks to them and the prince's help that she finally managed to escape from the tower. The girl and the prince lived happily ever after, but this is not always the case for people with Rapunzel syndrome, a condition that most often affects teenagers. So far, only 88 cases of this disease have been described.

Rapunzel's syndrome: causes

Rapunzel syndrome affects people who also suffer from trichotillomania, i.e. forced hair pulling, both from the head, eyebrows or eyelashes. Trichotillomania is an obsessive-compulsive disorder that is also accompanied by headaches, abdominal pain, and sleep problems. Trichotillomania, in turn, is related to trichophagia, which is eating the hair that has been torn off. Both of these conditions lead to Rapunzel syndrome - intestinal obstruction caused by hair in the digestive tract.

Rapunzel syndrome occurs in people who are accompanied by anxiety, a sense of loneliness, who suffer from emotional disorders, suffer from depression and even Tourette's syndrome. The disease also occurs in people with neuroses. Mutations in the SLITRK1 gene, responsible for the formation of connections between neurons, are also mentioned - if the connections are faulty, trichotillomania develops, which in turn leads to Rapunzel syndrome.

Rapunzel syndrome: symptoms

People who pull out and eat their hair may notice the following symptoms:

  • hair loss caused by pulling hair out;
  • abdominal pain (especially epigastric pain);
  • nausea;
  • vomiting after each heavy meal;
  • decrease in appetite;
  • weight loss;
  • gastroesophageal reflux;
  • halitosis (bad breath);
  • presencea hard, non-moveable and painless ball of hair in the mid-abdomen - it is often visible "with the naked eye" because it shapes the body layers;
  • the formation of a ball of hair leads to the obstruction of the large or small intestine.
Worth knowing

Trichobezoar

Trichobezoar is a hair ball - tightly sticky and hard that forms in the stomach and extends to the large or small intestine. Trichobezoars occur not only in people with Rapunzel syndrome, but also in men with beards or their partners, as well as in children who swallowed hair from brushes or dolls, i.e. people who swallow their hair accidentally.

Bezoars can also be produced by taking medications (they are called pharmacobezoars), fruit (phytobezoars), and even paper or fabric - in recent cases they are most common in children.

The name "bezoar" comes from the Hebrew language in which it means "medicine against poison". In the past, bezoars were called magic stones from distant lands, which were supposed to be an antidote to various types of poisons.

Rapunzel syndrome: treatment

The habit of eating hair is difficult to cure - people with trichotillomania are sent for psychotherapy, it may also be necessary to see a psychiatrist and prescribe anti-anxiety and anti-depressant drugs. Trichobezoars are surgically removed, but if the intervention is not performed quickly enough, in rare cases infection may develop and the ulcer rupture, and the patient may die.

Rapunzel syndrome: observed cases

As mentioned above, there are few reported cases of Rapunzel syndrome. One of them is the one concerning a 15-year-old girl from Poland who was diagnosed with a large trichobezoar. The girl complained of abdominal pain and was going bald. First, she was referred for psychotherapy, and after 6 months - when she stopped pulling her hair and eating it - she underwent an operation. The patient was under the constant care of a psychotherapist and no symptoms of trichotillomania were observed.

Another known case is a 12-year-old, also treated with surgery - the girl ate her hair from the age of 6, and the trichobezoar removed from her body took the shape of her stomach.

The most tragic known case of Rapunzel's band is that of Jasmine Beever from Great Britain. The 16-year-old felt sick in class, so the teachers sent her home, and shortly after that she went to the hospital with her parents. She was reanimated there, but she died anyway. An autopsy showed that the girl's death was caused by inflammationperitoneum - the trichobezoar in her stomach led to an infection and an ulcer that eventually ruptured.

Category:

Psychiatry