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Rapunzel Syndrome: A Comprehensive Review of an Unusual Case of Trichobezoar

Veena Gonuguntla, MD, Department of Pediatrics, Marshfield Clinic, 1000 North Oak Avenue, Marshfield, WI 54449
Divya-Devi Joshi, MD, MMM, Director, Pediatric Hematology/Oncology, Pediatric Residency, Department of Pediatrics, Marshfield Clinic, 1000 North Oak Avenue, Marshfield, WI 54449, Current address: Medical Director, Torrance Hospital IPA, 2355 Crenshaw Blvd, Suite 150, Torrance, CA 90501

Reprint Requests: Divya-Devi Joshi, MD, MMM Medical Director, Torrance Hospital IPA, 2355 Crenshaw Blvd, Suite 150, Torrance, CA 90501, E-mail: djoshi{at}thipa.com

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.

Key Words: Abdominal mass • Emotional disturbance • Mental retardation • Rapunzel syndrome • Trichobezoar