Rapunzel syndrome and its variants in pediatric patients: Our experience

Abstract

Background: Rapunzel syndrome is an extremely rare form of trichobezoar extending from the stomach to the small intestine and sometimes even beyond the ileocecal valve. Approximately 50 cases of Rapunzel syndrome have been reported in the literature, and most of them being young women. The aim of this study was to review the clinical features and outcome of pediatric patients with Rapunzel syndrome. Patients and Methods: We present a retrospective study performed from January 1995 to December 2013. The study included eight paediatric patients. The clinical features including history of trichophagia, intraoperative findings, bezoar characteristics and operative were analyzed. Results: The age of patients ranged from 4 years to 15 years. Only one (12.5%) child was in teenage group, whereas the rest of the patients (87.5%) were below the teenage group. There were five (62.5%) females and three (37.5%) males. Patients had varied presentations like chronic abdominal pain (100%), abdominal distension (75%), epigastric fullness (75%), features of intestinal obstruction (50%), and anorexia (25%). Trichophagia could not be elicited in four patients (50%). Impending intestinal perforation and Intussusception was seen in one (12.5%) each. Six (75%) patients had trichobezoar, while plastobezoar (plastic bezoar) and mixed bezoar was present in one (12.5%) each.Conclusions: Rapunzel syndrome is also seen in children below the teenage group. Although more common in females, it is seen in both sex. Chronic abdominal pain and distension are main presenting features, while history of trichophagia is absent in 50% cases. It should be included in the differential diagnosis in children, especially females with chronic abdominal pain, even if history of trichophagia is absent.