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Rapunzel syndrome in a 17 years old female presented with ileus

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Rapunzel Syndrome in a 17-year-old Female Presented with Ileus

 

Zuhal Bayramoglu, Ibrahim Adaletli

Istanbul University, Istanbul Medical Faculty, Radiology Department

 

  • Objective 
  • Rapunzel syndrome is an extremely rare intestinal disorder caused by  ingesting hair (trichophagia). Trichophagia is sometimes associated with the hair-pulling disorder trichotillomania and these patients usually also require psychiatric evaluation and treatment (1) .
  • Case Presentation
  • 17-year-old girl was admitted to the emergency department with a complaint of vomiting. Several air fluid levels were observed within small intestine on abdominal x-ray.  Abdominal ultrasonography was performed to investigate the cause of ileus. A mass filling almostly  the whole stomach  with echogenic and smooth margins was found. A similar fragment was seen as an obstructive pathology in the jejunal segments causing intestinal dilatation measuring up to 29 mm in diameter. Intravenous contrast enhanced CT demonstrated mass in the stomach and  similar object within the jejunal lumen including millimetric air bubbles and presenting hyperdense peripheral rim.  Partial contour of both masses were not hyperdense mimicking a broken porcelain.  Laparotomy and excision materials were compatible with trichobezoar and further clinical details supported the  swallowing of her own hair for a long time.  
  • Unique Teaching Points
  • Trichobezoar (gastric hair ball)  would have a a tail-like extension into the small bowel. A part may break off into small bowel and cause small bowel obstruction. The ' intralesional mottled gas pattern’ or ‘compressed concentric rings pattern’ would suggest the trichobezoar and final diagnosis should be made via upper gastrointestinal endoscopy  (2). Laparotomy with extraction of bezoar is done with exploration of rest of the small bowel to look for detached bezoars.
  •  

            1. Grant JE, Odlauq BL. Clinical characteristics of trichotillomania with trichophagia. Compr Psychiatry 2008;49: 579–584.

            2. Coulter R, Antony MT, Bhuta P, et al. large gastric trichobezoar in a normal healthy woman: case report and review of pertinent literature. South Med J 2005;98: 1042–1044.

 

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