Rapunzel syndrome
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Rapunzel syndrome is an extremely rare intestinal condition in humans resulting from ingesting hair (trichophagia).<ref name="Sah">Template:Cite journalTemplate:Dead linkTemplate:Cbignore</ref><ref name=Ventura>Template:Cite journal</ref> This syndrome occurs when a trichobezoar (hairball) reaches past the small intestine, and sometimes even into the colon producing a long, tail-like extension of hair.<ref name="Ullah16">Template:Cite journal</ref> The syndrome is named after the long-haired girl Rapunzel in the fairy tale by the Brothers Grimm. Trichophagia is also sometimes associated with the hair-pulling disorder trichotillomania.<ref name=Chamberlain>Template:Cite journal</ref> The Rapunzel syndrome is, however, a rare and unusual form of trichobezoar. Since 1968, there have been fewer than 40 documented cases in the medical literature.<ref>Template:Cite journal</ref>
Signs and symptoms
The use of the term 'Rapunzel syndrome' first appeared in the medical literature in 1968.<ref name=":0">Template:Cite book</ref>
Characteristics of the syndrome include:Template:Citation needed
- The body of a trichobezoar (hairball) located in the stomach, and its tail (hence the reference to Rapunzel in the syndrome's name) in the small bowel and/or in the right colon
- Small or large bowel obstruction
- Occurring in psychiatric patients
- Trichotillomania
- Abdominal pain
- Nausea and vomiting
- Gut perforation
- Vitamin B12 deficiency
- Acute pancreatic necrosis
Cause
Rapunzel syndrome is caused by the ingestion of hair. Rapunzel syndrome is characterized by a compulsive disorder of pulling one's own hair and ingesting it. There are several psychiatric disorders that are associated with Rapunzel syndrome, such as trichotillomania, trichophagia, and pica. Trichotillomania is the compulsion to pull out one's own hair; if an individual consumes it after ripping it out as well, it is known as trichophagia. Pica comes from the Latin word for "magpie" and involves the craving of non-food items such as cloth, wool, hair, or even small metallic objects.Template:Citation needed
Diagnosis
Trichobezoar can be preoperatively diagnosed. However, the diagnosis of the Rapunzel syndrome has to consider several aspects such as the patient's history with disorders like trichophagia and trichotillomania.<ref name=":1">Template:Cite journal</ref> This syndrome does not appear in the DSM-5, and will therefore not be given as such, but will have been diagnosed as severe trichotillomania.<ref>Template:Cite book</ref> The syndrome itself is used to describe the manifestation of a trichobezoar which has extended far into the small intestine. It describes the trichobezoar, not the mental health disorder which precipitated it.<ref name=":1" />
The diagnosis of the syndrome is also done by endoscopy. A CT scan is recommended to determine the size and the extension of the trichobezoar.<ref name=":0" /> Upper GI endoscopy is known as the gold standard for the diagnosis of a trichobezoar, however the endoscopy alone might not necessarily detect the co-existing Rapunzel syndrome.
Treatment
Laparotomy has been identified as the standard treatment for Rapunzel syndrome.<ref name="Ullah16"></ref> Because the human gastrointestinal tract is unable to digest human hair, the trichobezoar often has to be treated surgically; this involves removal of the mass by careful extraction from the stomach and duodenum. If the mass is small enough, it can be removed endoscopically. Once the mass surpasses greater than 20 centimeters, it must be removed by laparotomy. It is recommended that general anesthesia with intubation be used when removing the hairball in order to protect the throat from any damage. Patients usually also require psychiatric evaluation and treatment due to the association with impulse control disorders, especially trichotillomania.<ref name=Gorter>Template:Cite journal</ref> Long-term follow up as well as psychiatric consultation is also recommended to prevent the event from repeating.<ref name="Lopes10">Template:Cite journal</ref>
Outcomes
The expected outlook after surgical intervention is very promising. The success rate of removal of the mass is above 90% and the complication rate is only near 10%. Recurrence is highly uncommon but can occur if the patient does not follow up on psychological treatment or counseling.Template:Citation needed
Epidemiology
Rapunzel syndrome is extremely rare, with fewer than 64 cases reported since 1968. It is mainly seen in emotionally or mentally disturbed young or adolescent females. The first known case dates back to a 16-year-old boy in 1779; this was eventually published by Vaughan et al. in 1968. Of the cases reported, the typical age range affected from this syndrome is between 4 and 19 years of age. There is no specific region that is subject to developing this condition, however of the cases reported all of the women came from countries where women traditionally had long hair. Women are more subject to this disorder because women often have longer hair than men. There is only one reported male case of Rapunzel syndrome, but he was eating his sister's hair and not his own.Template:Citation needed
Research
Although this condition is extremely rare, researchers have mentioned that it is absolutely critical that prevention methods are taken after surgery. The most common reason for recurrence in patients is lack of follow-up care and incompletion of psychological treatment.Template:Citation needed