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

Introduction: 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. Presentation of case: 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. Discussion: 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. Conclusion: 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.