Rapunzel syndrome

Rapunzel syndrome
Specialty	Psychiatry, gastroenterology

Rapunzel syndrome is an extremely rare intestinal condition in humans resulting from ingesting hair (trichophagia).[1][2] The syndrome is named after the long-haired girl Rapunzel in the fairy tale by the Brothers Grimm. Trichophagia is sometimes associated with the hair-pulling disorder trichotillomania.[3] This syndrome is a rare and unusual form of trichobezoar. Since 1968, there are only 64 documented cases in literature.[4] This syndrome occurs when the trichobezoar (hairball) reaches past the small intestine, and sometimes even into the colon producing a long tail-like extension of hair (NCBI, 2016).

Signs and symptoms

The use of the term Rapunzel syndrome first appeared in the literature in 1968.[5]

Characteristics of the syndrome include:

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 & 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, they are trichophagia, trichotillomania, and pica. Tricophagia refers to someone who has a pyschiatric disorder and eats their own hair. Trichotillomania is the compulsion to pull out your own hair, and most will start to consume it after ripping it out. Pica comes from the latin word "magpie" and involves the craving of non-food items such as cloth, wool, or hair.

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.[6][7]

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.[5] Upper GI endoscopy is known as the gold standard for the diagnosis of trichobezoar however the endoscopy alone might not necessarily detect the co-existing rapunzel syndrome.

Treatment

Because the human gastrointestinal tract is unable to digest human hair, the trichobezoar may have 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 gastrotomy. 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.[8] Long-term follow up as well as psychiatric consultation is also recommended to prevent the event from repeating.

Mechanism

No information is available at this time.

Further 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. Researchers have concluded that 92.5 % of all cases reported were treated successfully by laparotomy with a 99% success rate (NCBI,2016). There have been few reports of successful treatment through laparoscopic surgery, however this has only been reported to be done in pediatric patients.

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.

Epidemiology

Rapunzel syndrome is extremely rare, with less than 64 cases reported since 1968. It is more common in young or adolescent females. Rapunzel syndrome is mainly seen in emotionally disturbed or mentally disturbed young 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 4-19 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 naturally women often have longer hair than men. There is only 1 reported male case of Rapunzel syndrome, but he was eating his sisters hair and not his own.

References

Sah DE, Koo J, Price VH (2008). "Trichotillomania" (PDF). Dermatol Ther. 21 (1): 13–21. doi:10.1111/j.1529-8019.2008.00165.x. PMID 18318881.
Ventura DE, Herbella FA, Schettini ST, Delmonte C (2005). "Rapunzel syndrome with a fatal outcome in a neglected child". J. Pediatr. Surg. 40 (10): 1665–7. doi:10.1016/j.jpedsurg.2005.06.038. PMID 16227005.
Chamberlain SR, Menzies L, Sahakian BJ, Fineberg NA (April 2007). "Lifting the veil on trichotillomania". Am J Psychiatry. 164 (4): 568–74. doi:10.1176/appi.ajp.164.4.568. PMID 17403968.
Gonuguntla, Veena; Joshi, Divya-Devi (2009). "Rapunzel Syndrome: A Comprehensive Review of an Unusual Case of Trichobezoar". Clinical Medicine & Research. 7 (3): 99–102. doi:10.3121/cmr.2009.822. ISSN 1539-4182. PMC 2757434. PMID 19625498.
Maloney, William James (2014-09-22). The Medical Lives of History's Famous People. Bentham Science Publishers. ISBN 9781608059362.
Wang, Zhe; Cao, Feng; Liu, Diangang; Fang, Yu; Li, Fei (2016-11-22). "The diagnosis and treatment of Rapunzel syndrome". Acta Radiologica Open. 5 (11): 205846011562766. doi:10.1177/2058460115627660. ISSN 2058-4601. PMC 5122172. PMID 27900201.
Wang, Zhe; Cao, Feng; Liu, Diangang; Fang, Yu; Li, Fei (2016-11-22). "The diagnosis and treatment of Rapunzel syndrome". Acta Radiologica Open. 5 (11): 205846011562766. doi:10.1177/2058460115627660. PMC 5122172. PMID 27900201.
Gorter RR, Kneepkens CM, Mattens EC, Aronson DC, Heij HA (May 2010). "Management of trichobezoar: case report and literature review". Pediatr. Surg. Int. 26 (5): 457–63. doi:10.1007/s00383-010-2570-0. PMC 2856853. PMID 20213124.