Rapunzel Syndrome Resulting in Multiple Sites of Simultaneous Intussusception

长发公主综合征导致多处同时发生肠套叠。

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Abstract

We present a case of Rapunzel syndrome in a nine-year-old girl with nonverbal autism, pica, and trichotillomania, who presented for acute worsening of abdominal pain and vomiting. Imaging revealed a bezoar extending from the stomach through most of the small intestine, resulting in multiple small bowel-small bowel intussusceptions. Open laparotomy confirmed the presence and enabled the removal of gastroenteric trichobezoars. Trichobezoars are seen most often in adolescent females with a history of trichotillomania and trichophagia. Trichobezoars can cause serious complications, including obstruction, perforation, and intussusception, most notable in the setting of Rapunzel syndrome. Abdominal computed tomography is the preferred imaging modality for diagnosis and surgical planning. The current case highlights the unique radiologic features of extensive bezoar disease and emphasizes the importance of early recognition and definitive operative management to reduce morbidity.

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