Rapunzel syndrome: A rare presentation with multiple small intestinal intussusceptions

Abstract

Bezoars are usually confined to the stomach which is seen in individuals with psychiatric illness like trichotillomania, trichophagia and gastric dysmotility. Long standing bezoars may extend into the small intestine leading to a condition known as Rapunzel syndrome. Diagnosis can be established by endoscopy, ultrasonography and computed tomography scan. Treatment includes improvement of general condition and removal of bezoar by laparoscopic approach or laparotomy. Psychiatric consultation is necessary to treat and prevent relapse. We report a case of Rapunzel syndrome in a 16-year-old girl with trichotillomania. She presented with history of epigastric mass for three months and recent onset of pain abdomen, vomiting and early satiety. Skiagram of abdomen was showing distended stomach and endoscopy revealed trichobezoar. At laparotomy, stomach was distended with trichobezoar and there were multiple small intestinal intussusceptions. Gastrotomy and manual reduction of intussusceptions with the removal of trichobezoar with its tail was done. Patient recovered completely after the procedure.

Keywords: Rapunzel syndrome; Trichobezoar; Trichotillomania; Intussusception

Core tip: Rapunzel syndrome is a rare condition which occurs in younger age group with trichotillomania and trichophagia. When trichobezoar is diagnosed further work up has to be carried out to rule out the presence of its tail extending into the small intestine causing complications such as intestinal obstruction, bleeding, malnutrition and perforation. We report a case where multiple intussusceptions were present which is rare. Though gastrotomy with enterotomy is advised to remove bezoar, we were able to remove the entire length of intestinal tail through gastrotomy itself and there was no need for enterotomy to remove the intestinal part of bezoar.