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

Chauhan NS, Kumar S, Bhoil R - Pol J Radiol (2016)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Dr. Rajendra Prasad Government Medical College, Kangra, India.

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.

No MeSH data available.


Related in: MedlinePlus

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).
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f1-poljradiol-81-166: 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).

Mentions: The abdominal USG revealed presence of a curvilinear echogenic intragastric mass with posterior acoustic shadow (PAS). Similar, albeit smaller echogenic masses with prominent PAS were also seen in segments of dilated small gut loops (Figure 1A). Doppler USG did not reveal any abnormal internal vascularity (Figure 1B). Mild free fluid was present in the peritoneal cavity. When asked specifically, the patient admitted to the habit of eating her own hair (trichophagia). The ultrasonographic findings, coupled with the history of trichophagia led us to a suspicion of intragastric and small bowel trichobezoars with intestinal obstruction. CT of the abdomen was done subsequently to confirm the diagnosis. The CT scout view of the abdomen showed mottled radiolucencies in the stomach with a crescent of air in the periphery along with dilated jejunal loops (Figure 2A). The axial plain and contrast images showed an inhomogeneous attenuation hypodense non- enhancing mass 16×11×8 cm in size, occupying almost the entire stomach with pockets of air in the interstices (Figure 3A). The small bowel loops were dilated with presence of multiple (five) similar but smaller hypodense masses in the jejunum and ileum (Figure 2B, 3B). The largest among the small bowel masses measuring 8.5×3.3 cm was impacted in the distal ileum leading to intestinal obstruction. A mild amount of free fluid was noted in the abdomen and pelvis.


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

Chauhan NS, Kumar S, Bhoil R - Pol J Radiol (2016)

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4835156&req=5

f1-poljradiol-81-166: 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).
Mentions: The abdominal USG revealed presence of a curvilinear echogenic intragastric mass with posterior acoustic shadow (PAS). Similar, albeit smaller echogenic masses with prominent PAS were also seen in segments of dilated small gut loops (Figure 1A). Doppler USG did not reveal any abnormal internal vascularity (Figure 1B). Mild free fluid was present in the peritoneal cavity. When asked specifically, the patient admitted to the habit of eating her own hair (trichophagia). The ultrasonographic findings, coupled with the history of trichophagia led us to a suspicion of intragastric and small bowel trichobezoars with intestinal obstruction. CT of the abdomen was done subsequently to confirm the diagnosis. The CT scout view of the abdomen showed mottled radiolucencies in the stomach with a crescent of air in the periphery along with dilated jejunal loops (Figure 2A). The axial plain and contrast images showed an inhomogeneous attenuation hypodense non- enhancing mass 16×11×8 cm in size, occupying almost the entire stomach with pockets of air in the interstices (Figure 3A). The small bowel loops were dilated with presence of multiple (five) similar but smaller hypodense masses in the jejunum and ileum (Figure 2B, 3B). The largest among the small bowel masses measuring 8.5×3.3 cm was impacted in the distal ileum leading to intestinal obstruction. A mild amount of free fluid was noted in the abdomen and pelvis.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Dr. Rajendra Prasad Government Medical College, Kangra, India.

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.

No MeSH data available.


Related in: MedlinePlus