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Rapunzel Syndrome -

A scary, hairy disorder named after a beautiful fairy tale.

 

Dr Sampurna Roy MD                            June 2016   

 


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The term Rapunzel syndrome was first proposed by Vaughan et all.The Rapunzel syndrome. An unusual complication of intestinal bezoar.

Bezoars are foreign bodies in the stomach of animals and humans that are composed of food or hair that has been altered by the digestive process.

Phytobezoars are concretions of poorly digested fruit and vegetable.

It is rarely noted in the normal stomach except in people who eat many persimmons. The pulp or skin of persimmons contain tannin monomer.

It polymerize at low pH to form a glue-like binding material (tannin-cellulose-protein complex).

After binding with other material it forms a dark, hard, sticky phytobezoar in the stomach.

Phytobezoars have been found in patients with delayed gastric emptying and hypochlorhydria after partial partial gastrectomy.

Trichobezoar is a rare condition in which the swallowed hairs accumulate inside the stomach.  

Being indigestible and slippery, it  becomes entrapped within the stomach.

Large amounts can accumulate over the years forming a hair ball.

In Rapunzel syndrome main body of the hair ball is located in the stomach and the tail extends into the small and large bowel as far as the transverse colon.

This syndrome is usually seen in long haired girls or young women and is often related to psychiatric disorders.

The patients swallow their own hair.

The patient may present with  abdominal pain, loss of appetite, weight-loss, vomiting, loose stools, pancreatitis, jaundice, anemia and hypoalbuminemia.

Clinical characteristics are a movable mass in the epigastrium and alopecia.

These signs and symptoms raise the suspicion of malignancy. Diagnosis can be established either by barium swallow or by CT scan.

Ultrasound might suggest the diagnosis, but sonographic features are definitely not pathognomonic.

The clinician must consider the possible presence of a "tail" in any patient presenting with a bezoar.

The therapy of choice is surgery of the trichobezoar together with the whole intestinal "tail," as in most cases endoscopic removal fails due to the large extension.

Early diagnosis and treatment of the Rapunzel syndrome is of great importance to avoid later fatal complications such as gastric perforation and intestinal necroses.

Intensive psychiatric follow-up is necessary for preventing relapses.

Summary of findings of Rapunzel Syndrome:

Diagnosis of bezoars needs high index of suspicion and it should be considered in the differential diagnosis of bloating in young females.

Trichobezoars are usually seen in healthy patients with normal digestive system but with psychiatric problems such as trichophagia, trichotillomania and/ or mental retardation.  

The body of a trichobezoar is located in the stomach, and its tail in the small bowel and/or in the right colon.

Complications of bezoars include ulcers, perforation of the bowel, obstruction and intussusception.

The Fairy Tale Rapunzel:

Grimm’s Fairy Tales are famous all over world.

Rapunzel was written by Brother Grimms (Jacob and Wilhelm Grimm) in 1812.

Rapunzel the long-haired beautiful heroine of the fairy tale was held in captivity in the high tower against her will.

She was probably depressed and had psychological problems.

There is no mention of whether she pulled and ate her beautiful golden hair.

Rapunzel used to lower her beautiful long golden tresses from the high tower window to the ground for the prince to climb up to her window. 

When hair strands extend from the main mass in the stomach, all along the small bowel and reaches upto the cecum, the condition is termed the Rapunzel Syndrome

 

Reference:

Rapunzel syndrome: diagnosis via radiograph and history.

Rapunzel syndrome: A rare presentation with multiple small intestinal intussusceptions.

A Hairy Tail not a Fairy Tale - Rapunzel Syndrome.

Rapunzel syndrome: not just a "hairy tail".

Rapunzel syndrome: a rare postpartum case.

A case of rapunzel syndrome.

The Rapunzel syndrome: an unusual trichobezoar.

The Rapunzel syndrome: a rare presentation of trichobezoar.

The Rapunzel syndrome: a case report and review of the literature.

The Rapunzel syndrome (trichobezoar) causing gastric perforation in a child: a case report.

[Rapunzel syndrome: a report of a new case].

Trichobezoars as a cause of gastrointestinal obstructions: the Rapunzel syndrome.

[Rapunzel's syndrome (trichobezoar)].

CT appearances of the Rapunzel syndrome: an unusual form of bezoar and gastrointestinal obstruction.

Rapunzel syndrome.

Trichophagia and trichobezoar: case report.

Huge simultaneous trichobezoars causing gastric and small-bowel obstruction.

The Rapunzel syndrome: a hard-to-swallow tale.

Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar.

Fatal case of Rapunzel syndrome in neglected child.

The Rapunzel syndrome. An unusual complication of intestinal bezoar.

An unusual case of the trichobezoar: the Rapunzel syndrome

Gastric trichobezoar and the Rapunzel syndrome].

[Trichobezoars].

Kinder und Hausmärchen, gesammelt durch die Brüder Grimm [Children's and Household Tales - Grimms' Fairy Tales], 7th ed., vol. 1 (Göttingen: Verlag der Dieterichschen Buchhandlung, 1857), story 12, at pages 65-69.

 

Visit:  DRESS Syndrome

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Dr  Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

                                                                                                                                 


 

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