The surgical management of Rapunzel syndrome: a case series and literature review

Background/PurposeThe surgical removal of a trichobezoar is the rare end complication of the psychiatric disorders trichotillomania and trichophagia. The more severe form of the disease is termed Rapunzel syndrome, where the bezoar extends from the gastric body beyond the pylorus into the duodenum. Traditional therapy has included endoscopy, often with subsequent laparotomy, and associated psychiatric intervention. We present the largest and most recent series of patients with trichobezoars managed in a single institution.MethodsA retrospective review of all cases of trichobezoar at our institution from 2003 to 2011 was performed. Demographic data, presenting complaints, imaging, surgical treatment, and subsequent management were collected.ResultsAll 7 patients were female, ages 5 to 23 years (mean, 11.5 years). Although multiple imaging modalities were necessary for preoperative diagnosis, most patients were accurately diagnosed without endoscopic evaluation (85%). All patients required an exploratory laparotomy for definitive treatment. At laparotomy, 5 patients were found to have postpyloric extension of the trichobezoar (71%). One of 7 patients had a wound infection postoperatively. There were no other surgical complications or recurrences requiring further exploration.ConclusionsOur series of trichobezoar patients appear to have a high rate of Rapunzel syndrome, and perhaps postpyloric extension should be considered the rule rather than the exception. Our series demonstrates that diagnosis can be established with a thorough history combined with radiography, and treatment should be a combination of laparoscopy and/or laparotomy with psychiatric consultation.