Ileo-ileal intussusception revealing a “Rapunzel syndrome”: Case report and literature review

Abstract

« Rapunzel Syndrome » is a rare type of trichobezoar with an extension of the hair into the small bowel in pediatric patients. Patients may be asymptomatic for a long time before developing symptoms as the bezoar grows in size. The aim of our report was to investigate and describe the mechanism of intussusception in RS. A girl presented to our emergency department with a history of severe abdominal pain and vomiting evolving for two days. She had diffuse abdominal tenderness without signs of peritonitis. The diagnosis of a small bowel-small bowel intussusception was evoked and confirmed by an abdominal ultrasound which revealed an ileo-ileal intussusception. The patient had conservative management in the first time, then, because of the persistence of abdominal pain and ileo-ileal intussusception on the ultrasonography (US), surgical intervention was decided. She had an enterotomy and a gastrotomy with an extraction of a trichobezoar of a 45 cm. The postoperative period was uneventful. The patient was discharged five days after surgery and referred to a child psychiatrist to explore psychological disorders. There are only a few cases in children reported in literature, with the youngest age being 2,5 years. It is essential to learn that intussusception can accompany Rapunzel syndrome so it must be timely diagnosed with early surgical intervention to prevent fatality.