Rapunzel syndrome: A rare condition you could share with your cat

By Naveed Saleh, MD, MS | Fact-checked by Barbara Bekiesz

Published April 26, 2024

Key Takeaways

Patients with Rapunzel syndrome tend to be young women with psychiatric comorbidities, such as OCD.
When a trichobezoar develops to extend into the small intestine, potentially causing GI blockages, the condition is known as Rapunzel syndrome.
Treatment includes the surgical removal of the trichobezoar and psychiatric therapy; medications such as SSRIs and CBT or other psychotherapies can help prevent recurrences.

In the German fairy tale Rapunzel, a suitor heroically rescues a princess by climbing her bizarrely long hair to the top of the tower that imprisons her. While Rapunzel’s long locks save her life, patients with the condition that borrows its name from the classic tale have a much different relationship with hair.

Patients with Rapunzel syndrome have developed a trichobezoar, the tail of which has extended into the small intestine due to ingesting—you guessed it—strands of human hair.

This condition is quite rare, with only about 50 cases reported in the literature.^[]^[]

What is a trichobezoar?

These hair-dense types are the most common type of bezoars, representing 55% of all cases, and they are widespread among people with psychiatric disorders.^[] Other types of bezoars include concretions of indigestible or vegetable fibers and result from disorders in gastrointestinal mobility. In cases of Rapunzel syndrome, hair is pulled and chewed on (ie, trichotillomania and trichophagia, respectively).

Researchers reporting on a series of cases from Northern India explain that ingested hair strands become sequestered in the folds of the gastric mucosa.

The accumulating hair, which does not get moved along by peristalsis, becomes entangled into a ball that is too big to transit from the stomach, thus leading to gastric atony. Rarely, this hairball can infiltrate the small intestine and cause intestinal blockage.

Other reported complications include gastric outlet obstruction and stomach bleeds, as well as stomach necrosis and perforation.

Diagnosing this rare condition

Trichophagia is an obsessive-compulsive behavior characterized by eating hair. In most cases, trichophagia is thought to be preceded by trichotillomania. Other underlying or associated diseases include PTSD, childhood neglect/abuse, and affective disorder. About 10% of patients with trichotillomania also exhibit trichophagia as a compulsive behavior.^[]^[]

Patients usually present with this disorder between the ages of 4 and 19 years, per the literature. Many patients with these conditions also exhibit mental retardation or pica.

Initially, patients complain of nonspecific symptoms, including epigastric pain, discovery of a mass, nausea, and vomiting, among others. With larger bezoars, mucosal erosion, ulceration, and perforation of the stomach or the small intestine have been reported, as well as obstructive jaundice, pancreatitis, protein-losing enteropathy, and intussusception.

Trichobezoars can be visualized using ultrasound or CT. CT findings show a non-enhanced, well-described, heterogeneous, intraluminal mass with a mottled appearance. The heterogeneous and mottled appearance derives from different densities of the components of the mass, such as food debris and air bubbles.

Although CT is highly accurate, ultrasound is also extremely sensitive. Upper GI endoscopy is the gold standard to diagnose intragastric trichobezoars, as it visualizes threads and allows for extraction. Taken together, CT findings, surgical findings, and patient history are needed to definitively diagnose Rapunzel syndrome.

Treatment

Treatment of Rapunzel syndrome involves the removal of the trichobezoar foreign body followed by psychiatric treatment for trichotillomania and trichophagia to prevent compulsive behaviors and recurrence. Trichobezoars are usually removed during laparotomy, although laparoscopic surgery has also been used.

Psychiatric treatment involves psycho- and pharmacotherapy options.^[] Effective drug treatment options include SSRIs, clomipramine, naltrexone, topiramate, and atypical neuroleptics.

Psychoeducation and cognitive skills training can further augment the efficacy of pharmacotherapy. Regular psychiatric follow-up is required to help prevent the recurrence of Rapunzel syndrome.

The management of Rapunzel syndrome is multidisciplinary and involves continued communication between the surgeon, gastroenterologist, psychiatrist, and caregivers.

What this means for you

When a young girl comes in with complaints of abdominal pain, vomiting, and anemia, Rapunzel syndrome should be included on the list of differential diagnoses. Early detection is key and recurrence can be prevented with psychiatric counseling and therapy. Patients who are committed to psychiatric therapy, including CBT to decrease compulsive behaviors, have a good long-term prognosis.