Rapunzel Syndrome Revealed by an Umbilical Hernia: A Case Report and Review of the Literature

Introduction: Rapunzel syndrome is a rare entity defined by the presence of a trichobezoar that can sit throughout the digestive tract. It results in psychiatric disorders, trichotillomania and trichophagia. Hernial strangulation is an exceptional mode of revelation of this pathology. We report a case of Rapunzel syndrome revealed by a recurrent strangulated umbilical hernia. Case report: This was a 22-year-old female patient with a history of umbilical hernia repair 6 months ago, admitted to the emergency department of Idrissa Pouye General Hospital for painful umbilical swelling. Examination on admission revealed a strangulated umbilical hernia with firm contents and an inferior umbilical scar. Biological work-up was unremarkable. Surgical exploration via an inferior umbilical approach revealed a strangulated umbilical hernia with a 3 cm neck, containing a viable small intestine, and the presence of a 50 cm long inert foreign body in the terminal ileum, enclosed at the ileocaecal angle. We performed a 1 cm enterotomy to extract the foreign body, which was made into a trichobezoar, then closed the enterotomy, treated the sac and cured the hernia using aponeurography. Postoperative recovery was straightforward. She was discharged on the third postoperative day. She was referred to the psychiatric ward for psychiatric and psychological follow-up. Conclusion: Rapunzel syndrome is a rare condition. Its mode of revelation by a strangulated umbilical hernia is very rare, even exceptional. The evolution can be marked by serious mechanical complications such as intestinal occlusion.