Rapunzel Syndrome: Symptoms, Causes And Treatment
Rapunzel syndrome is a very rare disease that manifests itself by the presence of a mass of hair in the stomach or intestine due to the voluntary and compulsive ingestion of the hair itself (trichophagia).
This disorder is associated with trichotillomania and causes serious gastrointestinal complications. In this article, we’ll explain what trichotillomania and trichophagia are, their symptoms, and the most effective interventions.
It is a disorder that primarily affects young women and the causes are not entirely clear, but it is suspected that it is associated with low self-esteem, problems with anxiety and depression, in addition to the aforementioned trichotillomania, which we will discuss later, and other disorders.
Symptoms of Rapunzel syndrome
A trichobezoar is a lump that forms in the stomach or intestine of a person who ingests hair. However, trichophagia does not always cause trichobezoars.
The time from when a person begins to ingest hair and when they start showing symptoms can vary. Some of the symptoms that may indicate the presence of a trichobezoar are:
- Weightloss
- Symptoms of obstruction of the stomach or of the exit of the intestine
- Nausea, vomiting, constant feeling of fullness and unable to eat
- Diarrhea
- Bad breath
- Iron deficiency anemia
- Bloody stools
- Alopecia areata associated with trichotillomania
Diagnosis is difficult, as patients often deny ingestion of hair. The presence of any of the above symptoms can be an indicator of trichophagia.
In addition, the presence of alopecia may indicate that the person has a hair pulling disorder (trichotillomania), sometimes associated with its ingestion, which could help guide the diagnosis.
Definitive diagnosis can be made by stool analysis, ultrasound, MRI or gastroscopy. The latter is the best technique to confirm the presence of trichobezoars.
Trichotillomania and its symptoms
Trichotillomania, or traction disorder, is a compulsive hair bundle. Hair or body hair can come from any area of the body, the most common areas being the scalp, eyebrows and eyelids (eyelashes).
Traction can be associated with different rituals. Like choosing a certain type of hair / body hair, handling them later, putting them in the mouth …
Some people experience strange and uncomfortable sensations before pulling their hair which is relieved afterwards. The fact of hair pulling is usually not painful, although it can cause hair loss, often seen as the “tonsure trichotillomania.”
This disorder most commonly affects women and can occur at any age. Its association with trichophagia is also more common in women. It is a chronic, fluctuating disorder, although it can improve with proper treatment.
Treatment of Rapunzel syndrome
The first step in correcting the disorder could be to apply a treatment for trichotillomania, because if one limits the pulling of the hair, the ingestion of the hair can also be reduced.
The most recommended treatment for trichotillomania is habit reversal, by Azrin and Nunn. Its main components are:
- Awareness: it is about making a description of the problematic behavior (pulling the hair), the antecedents and the consequences, to promote awareness
- Competitive response: it is about training and executing behavior that is incompatible with the problematic behavior
- Motivation: Motivate them to do everything they have learned, by reviewing the downsides of the problematic habit. By seeking social support and organizing public presentations on achievements
- Generalization: repeat the whole process in different areas of the subject’s life
Exposure response prevention can also be used. This consists of exposing the patient to the unpleasant sensations that precede the tearing of the hair. And to block the execution of the response (hair pulling).
The goal is for the person to be able to get used to the sensation without having to give the compulsive response they are used to.
Treatment for Rapunzel syndrome consists of dissolving the masses, either chemically or enzymatically. Or in the extraction of trichobezoars by endoscopy or surgery, as well as in the medical treatment of the possible and serious complications derived from it.
Curiosities about Rapunzel syndrome
It was first described by surgeon Vaughan ED and colleagues in 1968. In the 12th century BC, animal trichobezoars were already ingested because they were believed to have medicinal properties.
It is a very rare disease and currently just over 100 cases have been described worldwide. The name of the syndrome comes from the story of the Brothers Grimm, in which Princess Rapunzel found herself trapped in a tower and had to throw down her long hair for the prince to climb to save her.
A trichobezoar can closely resemble the shape of Rapunzel’s mane and be shaped like a braid or ponytail. Regardless of the color of the hair ingested, the color of the trichobezoar is always a very dark black. It may also contain traces of undigested food. Accumulations of hair can have different sizes, shapes and weights, up to 3 kg.
Our thoughts Ehlers Danlos syndrome refers to a pathology that affects connective tissues and which can be extremely disabling.
