- Case Report
- Open Access
'Rapunzel syndrome' trichobezoar in a 7-year-old girl: a case report
© Al Wadan et al; licensee BioMed Central Ltd. 2008
Received: 01 August 2008
Accepted: 02 October 2008
Published: 02 October 2008
Rapunzel syndrome is a rare type of trichobezoar with an extension of the hair into the small bowel. Clinical presentation is deceptive and vague ranging from abdominal mass to gastrointestinal symptoms.
We present a 7 years old girl with Rapunzel syndrome, where the trichobezoar was not suspected at all especially with negative history of trichophagia. In majority of the cases the diagnosis was made very late in the history of the disease, at a stage where surgery is the only cure for this syndrome.
In the paediatric age group with a long history of gastrointestinal symptom, endoscopy is a diagnostic as well as a therapeutic modality and may reduce surgery in trichobezoars.
Bezoar is a tightly packed collection of undigested material that is unable to exit the stomach, Most bezoars are of indigestible organic matter such as hair-trichobezoars; or vegetable and fruit the – phytobezoars; or a combination of both but other rare substances has been also been described in literature. Trichobezoars, commonly occur in patients with psychiatric disturbances who chew and swallow their own hair. Only 50% will have history of trichophagia. Trichobezoars have been described in literature and they comprise 55% of all bezoars. In very rare cases the Rapunzel Syndrome hair extends through the pylorus into the small bowel causing symptom and sign of partial or complete gastric outlet obstruction .
7 year old girl was referred to our paediatric clinic in 2007, with a history of abdominal pain, distension, weight loss and attacks of vomiting which was not persistent but it comes after a meal and fluid intake. This history was on and off for almost one year. She was treated in different primary health care as a case of gastroenteritis and parasitic infestation. Despite the treatment she was receiving there was no improvement in her condition, in fact she was getting worse.
In the paediatric clinic, abdominal palpation revealed an oblong mobile well-defined mass occupying the upper half of the abdomen, the mass was not tender and was firm in consistency. Upper Gastrointestinal endoscopy revealed a Trichobezoar occupying almost the whole gastric cavity, an attempt to remove it by foreign body forceps failed and the forceps was barley pulling few fibres of this huge ball of hair.
The common presentation of trichobezoar is in young females usually with an underlying psychiatric disorder. In our case the presentation is in a very young age with hair extending down to the small bowel, causing symptoms, which could mimic gastrointestinal infections and infestation especially in endemic areas. Rapunzel Syndrome is a rare form of trichobezoar. It is named after a tale written in 1812 by the Brothers Grimm about a young maiden, Rapunzel, with long hair who lowered her hair to the ground from a castle, which was a prison tower to permit her young prince to climb up to her window and rescue her.
In recent years more and more cases has been described . This syndrome was originally described by Vaughan et al. in 1968 . The commonly accepted definition is that of a gastric trichobezoar with a tail extending to the jejunum, ileum or the ileocecal junction.
Majority of cases of trichobezoar present late, due to the low index of suspicion by the physician. Of 131 collected cases of trichobezoar, a palpable abdominal mass was present in (87.7%), abdominal pain (70.2%), nausea and vomiling (64.9%), weakness and weight loss (38.1%), constipation or diarrhoea (32%) and haematemesis (6.1%). The laboratory investigations revealed low haemogiobin in about 62% (average) .
Patient with Trichotillomania (a psychological condition that involves strong urges to pull hair), around 30% will engage in trichophagia, and of these, only 1% will go on to eat their hair to the extent requiring surgical removal . Less than half of the patients give a history of trichophagia. There has been few cases of recurrence following successful surgery .
Endoscopy is diagnostic, in almost all cases while Ultrasound has not much to offer as a diagnostic tool. CT scan with contrast will delineate the extension of trichobezoar.
In the early stages endoscopic removal is not with out risk of bowel perforation and should be resolved for small Trichobezoars only . Other methods including the use of laser ignited mini-explosive technique were used successfully . Laparoscopy has been also used with limited success. Open surgery still remains the corner stone of large Trichobezoar removal especially if it has an extension into the bowel, which might be missed with other methods of treatment.
A long history of gastrointestinal problem, in a pediatric age group with history of trichophagia, early endoscopy is recommended. All patients with Trichobezoar should be referred for psychiatric evaluation after surgery to avoid recurrence.
Written informed consent was obtained from the parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Phillips MR, Zaheer S, Drugas GT: Gastric trichobezoar: case report and literature review. Mayo Clin Proc. 1998, 73: 653-656.View ArticlePubMedGoogle Scholar
- Al-Wadan Ali, Al-Absi Mohamed, Al-Saadi Azan, Abdoulgafour Mohamed: Rapunzel syndrome. Saudi Med J. 2006, 27 (12): 1912-4.PubMedGoogle Scholar
- Vaughan ED, Sawyers JL, Scott HW: The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery. 1968, 63: 339-343.PubMedGoogle Scholar
- Gupta Naik, Naik S, Chaudhary AK, Jain P, Sharma A: Rapunzel Syndrome Reviewed and Redefined. Dig Surg. 2007, 24: 157-161. 10.1159/000107716. [My paper]View ArticlePubMedGoogle Scholar
- Mohite PN, Gohil AB, Wala HB, Vaza MA: Rapunzel Syndrome Complicated with Gastric Perforation Diagnosed on Operation Table. J Gastrointest Surg. 2008Google Scholar
- Ventura DE, Herbella FA, Schettini ST, Delmonte C: Rapunzel syndrome with a fatal outcome in a neglected child. J Pediatr Surg. 2005, 40 (10): 1665-7. 10.1016/j.jpedsurg.2005.06.038.View ArticlePubMedGoogle Scholar
- Pérez E, Sántana JR, García G, Mesa J, Hernández JR, Betancort N, Núñez V: Gastric perforation due to trichobezoar in an adult (Rapunzel Syndrome). Cir Esp. 2005, 78 (4): 268-70.View ArticlePubMedGoogle Scholar
- Frey Ariel, Milissa McKee, King Robert, Martin A: Hair Apparent: Rapunzel Syndrome. Am J Psychiatry. 2005, 162: 242-248. 10.1176/appi.ajp.162.2.242.View ArticlePubMedGoogle Scholar
- Memon SA, Mandhan P, Qureshi JN, Shairani AJ: Recurrent Rapunzel syndrome – a case report. Med Sci Monit. 2003, 9 (9): CS92-4.PubMedGoogle Scholar
- Dumonceaux A, Michaud L, Bonnevalle M, Debeugny P, Gottard F, Turk D: Trichobezoar in children and adolescent. Arch Paediatr. 1998, 5: 996-9. 10.1016/S0929-693X(98)80010-9.View ArticleGoogle Scholar
- Huang YC, Liu QS, Guo ZH: The use of laser ignited mini-explosive technique in treating 100 cases of gastric bezoars. Chung Hua Nei Ko Tsa chih. 1994, 33: 172-174.PubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.