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For some of these people, they also develop trichophagia, where they then eat the hair they have pulled out. As well as patches of baldness, those with this syndrome develop a trichobezoar, which is a hairball that lodges in the stomach.

If you have ever had the pleasure of being around a long-haired cat, you will undoubtedly know what a hairball is, and how disgusting it is when the cat decides to throw it up. The cause of the hairball is the constant licking of the cat’s fur, which collects in the gut and intestine. For a rare number of people, they too develop hairballs, but unlike the cat, it’s not so easy to get them out.

Rapunzel Syndrome

This is a rare syndrome in humans, named after the fairytale by the same name, and is characterized by a psychiatric condition called trichotillomania, where the person pulls out their own hair constantly and repetitively. For some of these people, they also develop trichophagia, where they then eat the hair they have pulled out. As well as patches of baldness, those with this syndrome develop a trichobezoar, which is a hairball that lodges in the stomach.

Not all patients with Rapunzel Syndrome end up with a hairball, but an estimated 37.5% will. Human hair is not digestible and kind of slippery, so it stays in the stomach. The hairball normally has a tapered tail and this often extends into the jejunum, ileum or colon. Because of the consistency of the hairball, the normal contractions of the muscles of the stomach that moves food through the digestive system is unable to shift the ball of hair. This will often lead to an intestinal blockage.

 Symptoms and Complications

There are a large number of symptoms and complications associated with Rapunzel Syndrome, most of which occur in the gastrointestinal system.

Symptoms can include:

  • Pain in the abdomen
  • Appetite loss
  • Nausea and vomiting
  • Pale complexion
  • Weight loss
  • Bloating
  • Patchy baldness or hair loss

Complications can include:

  • Intestinal and gastric obstruction
  • Gastric ulcer
  • Intestinal bleeding
  • Perforation of the gastrointestinal tract wall
  • Destruction of gastrointestinal tract tissue
  • Gangrene of the intestine
  • Obstructive jaundice
  • Peritonitis
  • Acute pancreatitis
  • Anemia

Diagnostic Procedures

Following a physical examination including blood tests, an x-ray is the next diagnostic procedure usually performed. Sometimes, the physician may skip the x-ray and go straight to the CT scanner or MRI machine. The purpose of these radiological tests is to look for gas in the abdomen, which indicates a bowel perforation, and to see if there is a foreign object in the stomach or intestines.

Another procedure used for diagnosis is a fluoroscopy with barium. Barium is swallowed, and then followed using fluoroscopy as it travels through the gastrointestinal system. This barium can stay in the system for quite some time, and further x-rays may be taken later after the barium has exited the body. If there is a hairball, it will have filled with the barium, making it easy to see.

Who Is At Risk, and How Is It Treated?

Treatment Options for Removal of Hairball

The only way the hairball can be removed, is by various surgical techniques. If the hairball is fairly small, an enzyme can be used to dissolve parts of the hairball and it can then be removed by endoscope. Another surgical technique that is appropriate is by removing the hairball through a laparoscopy, which involves a small incision and scopes used to remove the object.

In cases where the hairball is larger, greater than 20 cm in size, the only option is by open surgery, which is a riskier procedure but is often the best method to remove it. These hairballs can get very big, and one of the largest on record to date weighed a staggering 9lbs and was removed from a girl in her teens.

Who Is At Risk?

It is known that females are more likely to develop trichophagia than males, though it is not understood why. Even though researchers know that trichophagia is a neurological or psychiatric disorder, studies have not identified absolutely what the underlying cause is. However, there have been a number of theories put out there, and the most common theory is that it is caused by an event or trauma that occurred in childhood.

Other theories include the possibility of a genetic factor in developing trichophagia. For those who have a parent with an obsessive compulsive disorder, there may be a genetic link, as the compulsion to pull and eat one’s own hair is in itself an obsessive compulsive disorder. The other most common theory is related to learned behavior, meaning that the child has learned the behavior by watching someone else, therefore imitating.

Treating Trichophagia

The first step in treatment is to take care of any associated medical issues such as anemia, and then surgically remove any hairballs that may be present. Then, like other psychiatric disorders, an evaluation is done to determine if there is an underlying psychiatric illness. Unfortunately, because a large component of trichophagia is related to behavior, it takes more than a medicine to treat it. Patients will typically be prescribed an anti-depressant medication, particularly where there is some evidence of an underlying illness or a historical event such as in the case of post-traumatic stress disorder. Then, treatment focuses on psychotherapy. Sessions with a psychologist or other such therapist are necessary to get to the bottom of why the trichophagia is occurring and how to overcome the compulsive behavior. Because trichophagia most commonly begins in childhood and adolescence, with early treatment, the disorder can generally become well controlled.

Conclusion

Trichophagia or Rapunzel Syndrome is very rare, and most people wouldn’t even know it existed, let alone be able to identify the symptoms. This is why it often gets to the point where a hairball is developed, as the behavior goes undetected for a period of time, sometimes even years. However, it is a serious condition, and if you suspect that you may have trichophagia, or someone you know may have it, you do need to seek medical attention and advice as soon as possible. It is much better to get help before the development of the hairball, so that treatment is less invasive.

The good news is that once the trichophagia has been identified, and treatment has been initiated, the prognosis is excellent. Most patients will learn to manage their symptoms and compulsive behavior, and go on to live a perfectly normal life.

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