Rapunzel Syndrome: A Case of Trichobezoar with Small Bowel Complications

Abstract

Introduction Rapunzel syndrome is characterized by a large trichobezoar in the stomach with a tail extending beyond the pylorus into the small bowel, causing mechanical obstruction of the small bowel. A 7-year-old girl presented to the emergency room with severe epigastric pain. Computed tomography suggested trichobezoar causing jejuno-jejunal intussusceptions, bowel wall thickening, and dilated small bowel loops proximal to the obstruction. On laparotomy, two concealed perforations were noted at the duodenojejunal (DJ) junction and 40 cm distal to the DJ junction. An enterotomy incision was given at the antimesenteric border of the distal jejunal perforation site, and the mass was successfully extracted. Primary repair was done at the DJ perforation site, and resection was followed by an end-to-end anastomosis at the distal jejunal perforation site. Surgery confirmed a complex mass of tangled hair within the gastric cavity with a tail extending into the pylorus of the stomach and small intestine, consistent with trichobezoar. Conclusion Computed tomography is superior to other radiological imaging modalities for diagnosing trichobezoars as it helps diagnose and demonstrate mechanical bowel complications.

A huge gastric trichobezoar presenting with Rapunzel syndrome: A case report

“…Trichobezoars are considered to affect just 1% of trichophagia sufferers. [5,6] Rapunzel syndrome occurs when the hair tail extends beyond the pylorus into the small intestine. It is an extremely uncommon condition, with roughly 110 cases documented in the published literature.…”

“…[9] In addition, pressure ulceration, esophagitis, and even esophageal perforation may occur because the removal of all fragments necessitates numerous instances of the endoscope being introduced. [5] Laparoscopy and mini laparotomy are commonly performed for small- or moderate-sized trichobezoars, with laparoscopy being the preferred method. Giant trichobezoars, endoscopic therapy failures, complicated bezoars, and Rapunzel syndrome are usual indications for surgical removal via gastrotomy or enterotomy.…”

“…[1,3] Rapunzel syndrome is uncommon form of gastric trichobezoar having a tail that extends beyond the pylorus, leading to an intestinal obstruction. [4,5] Here, we report a case of Rapunzel syndrome with a huge trichobezoars managed by laparotomy.…”