Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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A Case of Rapunzel Syndrome

  • Kim, Joon Sung (Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Nam, Chang Woo (Department of Surgery, Ulsan University Hospital, University of Ulsan College of Medicine)
  • Received : 2013.06.10
  • Accepted : 2013.06.19
  • Published : 2013.06.30
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Abstract

Rapunzel syndrome refers to a very rare condition in which swallowed hair forms a gastric trichobezoar that has a long tail extending into the small bowel. We describe a case of Rapunzel syndrome in an 8-year-old girl who presented with abdominal mass, epigastric pain and vomiting. Abdominal computed tomography scan showed a markedly dilated stomach filled with coarse heterogeneous materials. Upper gastrointestinal endoscopy revealed a huge hairy ball with a tail extending through the pylorus. We performed a surgical laparotomy and successfully removed a huge trichobezoar with a long tail extending into the middle portion of jejunum. Psychiatric consultation with review showed her past history of trichotillomania and trichophagia 4 years ago. But her parents denied further psychiatric therapy and she was lost to the follow-up. Rapunzel syndrome should be included in the differential diagnosis in children with chronic abdominal pain and trichophagia.

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References

  1. Williams RS. The fascinating history of bezoars. Med J Aust 1986;145:613-4.
  2. Gonuguntla V, Joshi DD. Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar. Clin Med Res 2009;7:99-102. https://doi.org/10.3121/cmr.2009.822
  3. Rabie ME, Arishi AR, Khan A, Ageely H, Seif El-Nasr GA, Fagihi M. Rapunzel syndrome: the unsuspected culprit. World J Gastroenterol 2008;14:1141-3. https://doi.org/10.3748/wjg.14.1141
  4. http://www.has.vcu.edu/for/grimm/rapunzel_e.html (accessed 10 May 2013).
  5. Vaughan ED Jr, Sawyers JL, Scott HW Jr. The Rapunzel syndrome. An unusual complication of intestinal bezoar. Surgery 1968;63:339-43.
  6. Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, et al. Rapunzel syndrome reviewed and redefined. Dig Surg 2007;24:157-61. https://doi.org/10.1159/000102098
  7. Jiledar, Singh G, Mitra SK. Gastric perforation secondary to recurrent trichobezoar. Indian J Pediatr 1996;63:689-91. https://doi.org/10.1007/BF02730823
  8. Ventura DE, Herbella FA, Schettini ST, Delmonte C. Rapunzel syndrome with a fatal outcome in a neglected child. J Pediatr Surg 2005;40:1665-7. https://doi.org/10.1016/j.jpedsurg.2005.06.038
  9. Jensen AR, Trankiem CT, Lebovitch S, Grewal H. Gastric outlet obstruction secondary to a large trichobezoar. J Pediatr Surg 2005;40:1364-5. https://doi.org/10.1016/j.jpedsurg.2005.05.021
  10. Park JS, Kim HJ, Chung JY. A case of trichobezoar with small bowel obstruction. Korean J Pediatr Gastroenterol Nutr 2009;12:230-4.
  11. West WM, Duncan ND. CT appearances of the Rapunzel syndrome: an unusual form of bezoar and gastrointestinal obstruction. Pediatr Radiol 1998;28:315-6. https://doi.org/10.1007/s002470050362
  12. De Backer A, Van Nooten V, Vandenplas Y. Huge gastric trichobezoar in a 10-year-old girl: case report with emphasis on endoscopy in diagnosis and therapy. J Pediatr Gastroenterol Nutr 1999;28:513-5. https://doi.org/10.1097/00005176-199905000-00015
  13. Fallon SC, Slater BJ, Larimer EL, Brandt ML, Lopez ME. The surgical management of Rapunzel syndrome: a case series and literature review. J Pediatr Surg 2013;48:830-4. https://doi.org/10.1016/j.jpedsurg.2012.07.046
  14. Fraser JD, Leys CM, St Peter SD. Laparoscopic removal of a gastric trichobezoar in a pediatric patient. J Laparoendosc Adv Surg Tech A 2009;19:835-7. https://doi.org/10.1089/lap.2008.0367
  15. Dorn HF, Gillick JL, Stringel G. Laparoscopic intragastric removal of giant trichobezoar. JSLS 2010;14: 259-62. https://doi.org/10.4293/108680810X12785289144520

Cited by

  1. Trichophagia along with trichobezoar in the absence of trichotillomania vol.5, pp.1, 2013, https://doi.org/10.4103/0976-3147.145204
  2. Trichobezoar Causing Airway Compromise during Esophagogastroduodenoscopy vol.2015, pp.None, 2015, https://doi.org/10.1155/2015/806857
  3. Tricobezoar gastroduodenal o síndrome de Rapunzel en una niña de 6 años vol.87, pp.6, 2013, https://doi.org/10.35366/97687