Very rare. Consequences of the presence of a gastric trichobezoar which a part extends into the small intestine. The name comes from the heroine of the tale of Grimm, Rapunzel (Raiponce in french), which had very long hair.

Bezoar: undigested foreign body that accumulates in the digestive tract, usually the stomach. Depending on the nature of the foreign body: trichobezoar (hair), phytobezoar (plant fibres), lactobezoar (curd), pharmacobezoars (tablets)...

A trichobezoar formation is due to the association of trichotillomania (compulsive hair pulling) and trichophagia (eating own hair). More than 90% of cases are young girls.

The accumulation of  non-digested hair in the stomach leads to the formation of a blackish mass (due to the effect of gastric acidity on hair) becoming more and more large. If it extends beyond the pylorus, it can cause: small bowel occlusion, intussusception of the small bowel.

This behavior disorder is often associated with a particular psychiatric context or mental retardation.

Other signs: very bad breath, epigastric pain, epigastric mass, gastric ulceration, gastrointestinal perforation. Occasionally: jaundice, pancreatitis.

Treatment: endoscopy only if the mass is small. Otherwise, it is often necessary to perform a laparotomy.

Anesthetic implications:

intestinal occlusion, "full stomach" (presence of the bezoar + obstacle to gastric emptying), psychological or psychiatric disorders

References:

-         Kim JS, Nam CW. 
A case of Rapunzel syndrome. 
Pediatr Gastroenterol Hepatol Nutr 2013; 16: 127-30.

-         Middleton E, Macksey LF, Phillips JD. 
Rapunzel syndrome in a pediatric patient: a case report. 
AANA Journal 2012; 80: 115-9.

Updated: October 2018