Rapunzel Syndrome complicated by dual jejuno-jejunal intussusceptions: a case report

A bezoar is a mass of indigestible material that accumulates in the gastrointestinal (GI) tract. Rapunzel Syndrome is a rare form of trichobezoar in which the hair mass extends beyond the pylorus into the small intestine, often leading to partial or complete GI obstruction.

A 9-year-old male with a significant medical history of non-verbal autism spectrum disorder and global developmental delay presented to the emergency department accompanied by his mother with a 1-week history of lethargy, abdominal pain, anorexia, and constipation. Computed tomography imaging demonstrated small bowel intussusception and a large, heterogeneous mass within the stomach. He was scheduled for urgent exploratory laparotomy with gastrotomy/enterotomy for foreign body removal. A longitudinal gastrotomy was performed, revealing a large gastrointestinal Rapunzel-type trichobezoar with foreign body components measuring 42 cm ×6 cm, extending from the stomach into the jejunum. The mass was successfully removed and sent for pathological evaluation. Intraoperative inspection of the bowel revealed two distinct points of intussusception: one located approximately 30 cm from the ligament of Treitz, and the other at 60 cm. Both were manually reduced, with the bowel appearing pink and well-perfused following the reduction.

The treatment of choice for Rapunzel Syndrome is surgical, often involving a gastrotomy and multiple enterotomies to thoroughly explore the stomach and small intestine for any retained bezoars.

Rapunzel Syndrome is rare in children and has been linked to psychiatric disorders. Parental counseling, along with behavioral therapy and psychiatric follow-up plays an important role in the comprehensive management of these patients.