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Rapunzel Syndrome

Rapunzel syndrome is a rare condition associated with the ingestion of human hair forming a large mass in the stomach and intestine that creates intestinal distress often requiring surgery. The symptoms vary from severe nausea to weight loss while the cause is often related to a mental illness known as trichophagia, the purposeful consumption of human hair, resulting in the not only the need for surgery but counseling to alter the harmful behavior.

The name Rapunzel Syndrome comes from the classic fairy tale yet it is far from a fairy tale illness. A patient, often an adolescent girl, appears with symptoms including weight loss, bloating, vomiting immediately following meals, acute gastric pain, fatigue, diarrhea or constipation and patchy hair loss. The actual illness appears when a substantial amount of human hair is consumed, which cannot be digested by the stomach, forming a large hairball that extends into the pylorus tract and into the intestinal tract causing a dangerous blockage.

The tail of the trichobezoar, hairball, often is the cause of this blockage which can further cause obstructive jaundice, small bowel perforation, peritonitis and pancreatitis. The medical professionals will perform ultrasounds, barium studies, cat scans or x-ray to determine the extent of the damage and determine a course for treatment or surgery.

The standard treatment involves laparoscopic surgery to extract the trichobezoar and exploration of the small bowel for perforations, ulcers and infectious damage which may or may not have to be repaired or removed. Further physical treatment is required to correct the anemia and weakness associated with this illness.

As Rapunzel Syndrome is tied to the mental illness trichophagia, a compulsive psychological disorder defined as the compulsive eating of one’s hair, it is an important part of recovery for the patient to receive psychotherapy to prevent future recurrences. Trichophagia is a more progressive form of trichotillomania, the compulsion to pull out one’s hair resulting in hair loss, and about 5-10% of those with trichotillomania progress to trichophagia.

Rapunzel syndrome may have a fairy tale name but it is a life threatening illness that left untreated could result in death. After the physical symptoms have been addressed and corrected, often via surgery, a patient is sent for psychotherapy or cognitive-behavioral therapy, with the possibility of medications being prescribed to help the underlying triggers of trichophagia and prevent the patient from further consuming her own hair resulting in trichobezoars forming in the stomach and intestines.

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