Death of a young girl with Rapunzel syndrome

Abstract

Introduction: Rapunzel Syndrome is an uncommon condition where a trichobezoar is seen at autopsy. This consists of a ball of hair in the stomach, with strands of hair extending like a tail into the small intestine. Case History: A post-mortem examination was performed on an eleven-year-old girl. History revealed chronic alopecia of over 6-8 months duration with loss of appetite and occasional abdominal pain of 3-4 months duration. She had collapsed suddenly and was pronounced dead on admission to hospital. Autopsy revealed peritonitis secondary to gastric perforation with a trichobezoar in the stomach weighing 600g. Discussion: Trichobezoars usually remain symptomless until they become large. They are often palpable when they are substantial. It is common in young females, usually with an underlying psychiatric disorder. Various imaging modalities help in the detection of bezoars. The main complications are ulceration, perforation, and obstruction. This condition is rare, but late or missed diagnosis could be fatal. Conclusions: Even though Rapunzel syndrome is an extremely uncommon variant of trichobezoar, awareness and early suspicion could prevent fatal complications.