An atypical death from Rapunzel syndrome: a case report

Abstract

Trichotillomania is a psychiatric disorder characterized by recurring urges to pulling out hairs, eyelashes, or down in other parts of the body. Trichophagia, which is the urge to ingesting the pulled-out hairs, can cause Rapunzel syndrome, an unusual disorder where gastric trichobezoars can be found in the small intestine. Trichobezoars, amorphous masses composed of undigested food formed by hairs, can obstruct the gastrointestinal tract up to simulating symptoms typical of bowel obstruction. Rapunzel syndrome, named after Grimm’s tale, may cause death, especially in the pediatric population, being it seldom over the age of 6; moreover, developing countries and environmental and familiar issues are listed as uncertain risk factors. The present case report deals with the death of a 4-year-old female occurred after lunch and following a series of vomit events; while no traumatic or pathological findings were revealed at the external examination, the autopsy revealed three large trichobezoars localized in the stomach and the small intestine. Despite death was due to gastrointestinal obstruction for multiple trichobezoars and collateral bronchoaspiration of dietary material, histological findings were totally non-specific, meaning that it is sometimes difficult to conclude that death is related to the primary pathological condition.

Fatal Rapunzel Syndrome

“…25 Complications of trichobezoar formation, and of bezoars, more generally, include malabsorption, malnutrition, anemia, cachexia, hypoalbuminemia, and intussusception. 26 Fatal cases of trichobezoar are rare and are typically secondary to mechanical obstruction of the gastrointestinal tract (perforation, peritonitis, sepsis, and multiorgan failure) 16,[26][27][28][29] or airways. 30 Radiological investigations are the primary diagnostic modalities for the antemortem identification of trichobezoar and Rapunzel syndrome.…”