Rapunzel syndrome is a very rare condition in which a large hair ball (trichobezoar) gets lodged in your stomach and extends into your small intestine. This causes the hair ball to look like a comma sign. The condition was first described in 1968. It’s named after a fairy-tale character, Rapunzel, who was known for her long hair.
This condition is much more common among women than men. And in about 8 out of 10 cases, it’s seen among children, adolescent girls, and young women under 30.
It’s hard to catch this condition early as you might go many years without having any symptoms. By the time you do, the hair ball is usually large and compacted enough to cause a serious blockage in your stomach. You’ll need surgery to remove it.
How Does Rapunzel Syndrome Happen?
Trichophagia is a condition in which you repeatedly eat hair. It’s often associated with trichotillomania, or the urge to pull out hair from the scalp. These conditions usually go hand in hand. If you happen to have one or both conditions, they’re more likely to cause Rapunzel syndrome.
Long hair has a smooth surface that’s often hard for your stomach to break down and digest. Hair also doesn’t move well through your digestive tract as it contracts and relaxes (a process called peristalsis) to move foods and liquids from your mouth to your anus.
This can cause the hair to get to stuck in your stomach cavity. Over time, eating your hair can cause it to form into a giant hair ball.
As the hair ball starts to grow in weight and size, it’s more likely to get clumped together with food you eat and mucus from the stomach lining. Eventually, the hair ball reaches the limits of your stomach wall. With nowhere left to go, the matted hair may start to jut out through the stomach opening (pylorus) and grow into the small intestine.
Signs of Rapunzel Syndrome
Symptoms are usually caused by the blockage or related health problems. This can include:
- Stomach pain
- Feeling full
- Weight loss
- Low weight and intense fear of weight gain (anorexia nervosa)
- Vomiting after meals
- Pain or discomfort under your ribcage (acute epigastric pain)
- Patchy hair loss on your scalp (alopecia)
- Bad breath (halitosis)
Common Comorbid Conditions With Rapunzel Syndrome
When you have Rapunzel syndrome, you’re more likely to have other mental health conditions at the same time, such as:
- Pica (an urge to swallow things that aren’t food)
- Mental disorders, such as schizophrenia, PTSD, ADHD, and others
- Anorexia nervosa
- Obsessive-compulsive disorder
- Bereavement (grief after losing a loved one)
Researchers have found that many people with Rapunzel syndrome have experienced childhood abuse or neglect.
Complications of Rapunzel Syndrome
As the hair ball grows in your belly, with time it can cause you to have other problems within your body, such as:
- Obstructive jaundice
- Physical blockage in your belly or small intestine
- Erosion of the mucus lining in your stomach and small intestine
- Holes within the small intestine (small bowel perforation)
- Inflammation in the stomach lining (peritonitis)
- Swelling of the pancreas (acute pancreatitis)
How Is Rapunzel Syndrome Diagnosed?
Your doctor will take a detailed history. They’ll ask you if you’ve swallowed certain items like human, pet, or doll hair among other things. They’ll also do a thorough physical exam to check for patchy hair loss and bad breath.
To diagnose Rapunzel syndrome, your doctor may run some imaging tests. This will help them figure out the size of the hair ball growth and how far it reaches.
Tests can include:
- CT scan
- Fluoroscopy barium study (barium swallow test). For this imaging test, you’ll swallow barium, a type of chemical that makes certain areas of your body show up more clearly on an X-ray. Fluoroscopy is a medical procedure in which doctors pass continuous X-ray beams through your body to get a real-time video of a body part.
- Endoscopy. Your doctor passes a thin, flexible tube with a light and camera down your throat to see the inside of your body clearly in real time. It also allows your doctor to take a sample of whatever is causing the blockage in your stomach.
Your doctor may also run blood tests to check for anemia (iron deficiency) or other possible nutritional deficiencies.
How Do You Treat Rapunzel Syndrome?
Rapunzel syndrome is often diagnosed when the hair ball has grown too big and starts to cause symptoms. Your doctor may try to remove it endoscopically through your mouth. But if the hair ball is too big, they’ll need to perform surgery.
Laparotomy. It’s a type of surgery in which a surgeon makes a cut in your belly to open and examine your stomach cavity and take out the hair ball. In some cases, complications might include infection and scar tissue around the cut.
Laparoscopy. This is an alternative procedure to laparotomy. The surgeon will make a smaller keyhole-sized cut on your belly to remove the hair ball. The recovery is much shorter with this method.
How Do You Treat the Cause of Rapunzel Syndrome?
Therapy. Cognitive behavioral therapy or other forms of psychiatric evaluation might help a psychologist or psychiatrist pinpoint and address mental health problems that might be causing you to eat your hair. This might also include counseling for parents.
Medications. Your doctor may also prescribe medications to help you gain control over your mental health.
What's the Outlook?
Research shows that in the long run, most people with Rapunzel syndrome do quite well post-surgery if they get behavioral therapy and go to their follow-up therapy appointments consistently. This will help to keep them from having it again.