Rapunzel Syndrome

Rapunzel syndrome is a digestive disorder that happens when you have a strong, uncontrollable urge to pull your hair out and swallow it. You may do this without realizing it. Mental health conditions like trichotillomania and trichophagia may lead to Rapunzel syndrome.

Swallowing your hair upsets your digestive system. The hair that you swallow gets stuck in your stomach. When you swallow food, the muscles in your stomach relax and then tighten to move the food for digestion in a process called peristalsis. But your hair is smooth and slippery. The muscles can’t grab it to move it out of your stomach.

Over time, tangles of hair in your stomach mix with mucus and food to make a hairball (trichobezoar). This trichobezoar can get big enough to fill your stomach and move into your small bowel.

Without treatment, the trichobezoar in your stomach and small bowel can affect your health. Treatment is medication or surgery to remove it. You may need mental health support to help you manage the urge to pull your hair out and swallow it.

This syndrome is very rare. Healthcare providers reported the first case of Rapunzel syndrome in 1968. They’ve reported fewer than 100 cases since then. The syndrome typically affects females ages 13 to 19. Rarely, toddlers and young children develop it.

Rapunzel syndrome gets its name from the fairy tale about a young woman with very long hair.

Symptoms of Rapunzel syndrome

Rapunzel syndrome symptoms may include:

• Abdominal (belly) pain that feels like cramps
• Bad breath
• Bald spots on your scalp
• Bloated belly
• Constipation
• Diarrhea
• Hair in your vomit
• Loss of appetite
• Nausea and vomiting
• Unexplained weight loss

You may have Rapunzel syndrome for years before you have symptoms. For example, symptoms like belly pain and bloating happen as a trichobezoar forms in your stomach. But you may not notice changes in your body until the ball of hair grows so large that it fills your stomach and small intestine.

Rapunzel syndrome causes

Rapunzel syndrome can happen if you have both trichotillomania (pronounced “trick-oh-till-oh-main-ee-uh”) and trichophagia (pronounced “trick-oh-fay-gee-uh”). These are mental health conditions.

Trichotillomania is like an obsessive-compulsive disorder. If you have it, you have an intense urge to pull out strands of hair from the same spot on your body. People with Rapunzel syndrome pull hair from their scalp. Experts don’t know what causes trichotillomania.

Trichophagia is when you suck on, chew or eat your hair. It’s a type of pica. If you have pica, you have an urge to swallow things that aren’t food, like dirt and hair. Like trichotillomania, healthcare providers aren’t sure what causes trichophagia (hair eating disorder).

Complications

A large hairball in your gut can cause complications like:

• Bowel obstruction
• Hole (perforation) in your stomach or small bowel
• Jaundice if the trichobezoar blocks your bile ducts
• Malnutrition
• Vomiting blood

How doctors diagnose Rapunzel syndrome

A healthcare provider will ask about your symptoms and do a physical examination. They’ll do tests to see what’s happening in your stomach and small bowel, like:

• Abdominal ultrasound
• CT scan
• MRI
• Upper endoscopy

How is Rapunzel syndrome treated?

You may have surgery to remove the trichobezoar. If surgery is necessary, your surgeon will talk to you about which procedure is best for your situation:

• Endoscopy: Your provider uses a long, thin tube (endoscope) to reach the hairball in your stomach and small intestine. The endoscope has small tools that your provider uses to break up and remove the hairball.
• Laparoscopy: This procedure involves small incisions (cuts) in your stomach. Your provider inserts a tiny camera to locate the hairball and small surgical tools to remove it.
• Laparotomy: Your provider may do surgery that opens your stomach to remove a very large hairball.

Mental health treatment

Your healthcare provider may recommend prescription medication and therapy.

Medications that treat trichotillomania include:

• Antidepressants
• Antipsychotic medications
• Antiseizure medications

Mental health therapies for trichotillomania include:

• Cognitive behavioral therapy (talk therapy)
• Habit reversal training to help you replace behaviors, like pulling hair out

When should I see my healthcare provider?

Contact your provider if you had surgery to remove a trichobezoar and have symptoms that may mean there’s an infection in the incision (cut) in your stomach. Common symptoms include:

• Incision starts to look deeper, longer or wider (wound dehiscence) instead of smaller
• Chills
• Fever (greater than 101 degrees Fahrenheit or 38.4 degrees Celsius)
• Thick, cloudy, white- or cream-colored discharge from the incision
• Redness or color changes in your skin that go beyond the edge of the incision

Rapunzel syndrome happens because you have certain mental health issues that make you have the urge to pull your hair out and swallow it. If you’re receiving treatment for those conditions, contact your provider if it’s hard for you to manage that urge.

What can I expect if I have Rapunzel syndrome?

Typically, a trichobezoar in your stomach and small intestine won’t come back after treatment to remove it. It’s important to remember that a mental health condition may trigger your urge to eat your hair. You may need ongoing mental health support to manage the urge.