Rapunzel syndrome
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.[4]
| Rapunzel syndrome | |
|---|---|
| Specialty | Psychiatry, gastroenterology |
Signs and symptoms
The use of the 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
Cause
Rapunzel Syndrome is caused by the ingestion of 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]
Treatment
Because the human gastrointestinal tract is unable to digest human hair, the trichobezoar may have to be treated surgically. Patients usually also require psychiatric evaluation and treatment due to the association with impulse control disorders, especially trichotillomania.[8]
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.
Further reading
- Duncan ND, Aitken R, Venugopal S, West W, Carpenter R (June 1994). "The Rapunzel syndrome. Report of a case and review of the literature". West Indian Med J. 43 (2): 63–5. PMID 7941500.
- Levy RM, Komanduri S (November 2007). "Images in clinical medicine. Trichobezoar". N. Engl. J. Med. 357 (21): e23. doi:10.1056/NEJMicm067796. PMID 18032760. Lay summary – CNN (2007-11-22).
- Matejů E, Duchanová S, Kovac P, Moravanský N, Spitz DJ (September 2009). "Fatal case of Rapunzel syndrome in neglected child". Forensic Sci. Int. 190 (1–3): e5–7. doi:10.1016/j.forsciint.2009.05.008. PMID 19505779.
- Pul N, Pul M (1996). "The Rapunzel syndrome (trichobezoar) causing gastric perforation in a child: a case report". Eur. J. Pediatr. 155 (1): 18–9. doi:10.1007/bf02115620. PMID 8750804.