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Case Reports
. 2016 Apr 14:81:166-9.
doi: 10.12659/PJR.896154. eCollection 2016.

Rapunzel Syndrome: Rare 'Tale' of a Broken 'Tail'

Narvir S Chauhan  1 , Satish Kumar  2 , Rohit Bhoil  1
Affiliations
Case Reports

Rapunzel Syndrome: Rare 'Tale' of a Broken 'Tail'

Narvir S Chauhan et al. Pol J Radiol. .

Abstract

Background: Rapunzel syndrome is a rare and unique form of trichobezoar, in which a cast of hairs is formed in the stomach with its 'tail' extending up to varying lengths into the small bowel. Almost all cases described in literature are of 'classic' and more common form of Rapunzel. Sometimes however, the tail of bezoar is broken into smaller fragments. There is not much literature available on this rarer subset of Rapunzel syndrome.

Case report: In this report we present the ultrasound (USG) and CT findings of a case of Rapunzel syndrome in which the tail had broken into five separate fragments. The patient presented clinically with intestinal obstruction due to the impaction of the distal- most fragment in the ileum.

Conclusions: Our case highlights the fact that although USG features may be suggestive, a careful evaluation of CT images is essential for a confidant preoperative diagnosis of Rapunzel syndrome. In cases of this syndrome with a broken tail, CT is essential for precise count and localization of the separated fragments to ensure their complete removal at the time of surgery. We also propose to name the fragments of the broken tail as 'bezoarlets'. This word aptly describes the tail fragments as it suggests their origin from the larger gastric bezoar and the suffix 'lets' conveys they are smaller in size.

Keywords: Bezoars; Intestinal Obstruction; Tomography, Spiral Computed; Trichotillomania; Ultrasonography.

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Figures

Figure 1
Figure 1
Transverse ultrasound image (A) showing a small bowel bezoarlet as curvilinear hyperechoic arc with posterior acoustic shadowing in a dilated jejunal loop (arrows) and the Doppler image (B) showing the lack of internal vascularity. Free fluid can also be seen (arrowheads).
Figure 2
Figure 2
CT scout view of the abdomen (A) showing the gastric bezoar as a localized area of mottled lucencies with a peripheral crescent of air (arrows) and dilated air- filled small bowel loops in the mid abdomen (arrowheads). Coronal CT MPR image (B) showing the gastric bezoar (arrows) and multiple small bowel bezoarlets (zig-zag arrows)
Figure 3
Figure 3
Axial contrast- enhanced CT image (A) showing the gastric bezoar with air in the interstices and peripheral air crescent (arrows). Axial contrast- enhanced CT image at the level of the pelvis (B) showing the separate small bowel bezoarlets (arrowheads).
Figure 4
Figure 4
Intraoperative photograph showing the gastric bezoar along the small bowel bezoarlets.
See this image and copyright information in PMC

References

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