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Special Feature
December 2002

Image of the Month

Arch Surg. 2002;137(12):1444. doi:
Answer: Rapunzel Syndrome

Computed tomographic scan of the abdomen showing large bezoar in the stomach.

The Rapunzel syndrome is a rare manifestation of a trichobezoar which was first described by Vaughn et al1in 1968. It is named after the heroine of a Brothers Grimm fairy tale who was rescued from a tower by a prince after letting down her long hair. Features include a gastric trichobezoar with a tail and gastrointestinal symptoms. To date, 17 cases have been described in the literature, to our knowledge.2

Bezoars are concretions of plant or animal material in the gastrointestinal tract. The term bezoar is derived from the Persian "padzahr" meaning counterpoison or antidote.3The classic review of 311 patients was published by DeBakey and Ochsner4in 1939. Symptoms range from early satiety, nausea, vomiting, and epigastric pain to obstruction and perforation. Physical findings include the presence of a nontender palpable mass. Computed tomographic examination may show an intraluminal mass without attachment to bowel wall and presence of air within the mass. Treatment depends on the composition of the bezoar. Plant bezoars (phytobezoars) may be dissolved with various enzymes. Trichobezoars (hair) are often resistant to enzymatic dissolution and require endoscopy for removal. Surgical removal is indicated when enzymatic dissolution and endoscopy are unsuccessful.

This patient had a large trichobezoar extending from the stomach into the small intestine causing a complete bowel obstruction. She underwent exploratory laparotomy and removal of the trichobezoar through an anterior gastrostomy. A psychiatric evaluation was requested for trichotillomania and trichophagia (pulling out hair and eating it). The remainder of her hospital course was unremarkable.

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Article Information

Corresponding author and reprints: Bradford G. Scott, MD, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, One Baylor Plaza, 404-D, Houston, TX 77030 (e-mail: bgscott@bcm.tmc.edu).

References
1.
Vaughn  ESawyers  JScott  H The Rapunzel syndrome: an unusual complication of the intestinal bezoar. Surgery. 1968;63339- 343
2.
Uckun  ASipahi  TIdge  M  et al.  Is it possible to diagnose Rapunzel syndrome preoperatively? Eur J Pediatr. 2001;160682- 683Article
3.
Williams  RS The fascinating history of bezoars. Med J Aust. 1986;145613- 614
4.
DeBakey  MOchsner  A Bezoars and concretions: comprehensive review of literature with analysis of 303 collected cases and presentation of 8 additional cases. Surgery. 1939;5132- 160
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