Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar

Abstract

An unusual form of bezoar extending from the stomach to the small intestine or beyond has been described as Rapunzel syndrome. Bezoars are concretions in the gastrointestinal tract that increase in size by continuous accumulation of non-absorbable food or fibers. Most bezoars in children are trichobezoars from swallowed hair from the head, dolls, or brushes. Trichobezoars typically cause abdominal pain and nausea, but can also present as an asymptomatic abdominal mass, progressing to abdominal obstruction and perforation. Trichobezoar with Rapunzel syndrome is an uncommon diagnosis in children with less than 40 cases reported. It is predominantly found in emotionally disturbed or mentally retarded youngsters. We present the youngest case of Rapunzel syndrome in the United States, a 5-year-old girl with mental retardation who presented with abdominal pain, vomiting and a non-tender abdominal mass.

Figure 1.

Plain radiograph of the abdomen showing multiple air fluid levels with dilated small intestinal loops and a sizable soft tissue density within the stomach are seen.

Figure 2.

CT showed a 10.8 cm bezoar, outlined by contrast, in the stomach and extends into the small intestine.

Figure 3.

A stomach-shaped trichobezoar with a tapering tail extending into the small bowel is surgically removed en bloc.