What is Rapunzel Syndrome and How is it Treated?

What you need to know

• Rapunzel syndrome occurs when a person repeatedly eats their own hair, forming a large hairball (trichobezoar) in the stomach that extends into the intestines.
• It is most common in young women and is often associated with psychiatric conditions like anxiety, depression, and obsessive-compulsive disorder.
• Symptoms can include abdominal pain, nausea, vomiting, weight loss, and intestinal obstruction.
• Diagnosis is typically made through imaging studies like CT scans.
• Treatment usually requires surgical removal of the hairball, along with psychiatric care to address the underlying hair-eating behavior.

What is Rapunzel syndrome?

Rapunzel syndrome is a rare medical condition where a large hairball, called a trichobezoar, forms in a person’s stomach and extends into their intestines. The name comes from the fairy tale character Rapunzel, who was known for her extremely long hair.

This condition occurs when someone repeatedly eats their own hair over an extended period of time, usually months or years. The hair accumulates in the stomach because it cannot be digested. Over time, it can form a large mass that takes the shape of the stomach. In Rapunzel syndrome, this hairball grows so large that it extends past the stomach and into the small intestine.

Who is at risk for Rapunzel syndrome?

Rapunzel syndrome is most common in young women, with about 90% of cases occurring in females. It is especially prevalent in people under 30 years old.

The condition is closely linked to two psychiatric disorders:

1. Trichotillomania - A compulsive urge to pull out one’s own hair
2. Trichophagia - The compulsive eating of hair

These hair-related behaviors are often associated with other mental health conditions like anxiety disorders, depression, obsessive-compulsive disorder, and anorexia nervosa. The hair pulling and eating may serve as coping mechanisms for emotional distress or anxiety.

What are the symptoms of Rapunzel syndrome?

Rapunzel syndrome can go undiagnosed for years, as symptoms may develop slowly over time. Some common signs and symptoms include:

• Abdominal pain, especially in the upper abdomen
• Nausea and vomiting
• Feeling full quickly when eating
• Weight loss
• A noticeable lump in the abdomen
• Constipation or diarrhea
• Bad breath
• Lack of appetite

In severe cases, it can lead to more serious complications like:

• Intestinal obstruction
• Stomach ulcers
• Perforation of the stomach or intestines
• Acute pancreatitis
• Obstructive jaundice
• Protein deficiency

How is Rapunzel syndrome diagnosed?

Doctors may suspect Rapunzel syndrome based on a patient’s symptoms and history, especially if there are signs of hair-pulling or eating behaviors. However, confirming the diagnosis typically requires imaging studies.

Some ways the condition may be diagnosed include:

• Abdominal X-rays - These may show an opaque mass in the stomach area.

• Ultrasound - Can sometimes visualize the trichobezoar as a bright, curved echo with a shadow.

• CT scan - The most definitive way to diagnose the condition. It can clearly show the hairball’s size and extent.

• Upper endoscopy - Allows direct visualization of the hairball in the stomach.

• Barium swallow X-ray - The trichobezoar appears as a filling defect on these contrast X-rays.

The definitive diagnosis is usually made when the hairball is surgically removed and examined.

How is Rapunzel syndrome treated?

Treatment for Rapunzel syndrome typically involves two main components:

1. Removal of the trichobezoar
2. Psychiatric treatment for the underlying hair-eating behavior

Removing the trichobezoar

In most cases, the large hairball needs to be surgically removed. This is usually done through an open abdominal surgery called a laparotomy. The surgeon makes an incision in the stomach and carefully removes the entire hairball.

In some cases, especially if the trichobezoar is smaller, it may be possible to remove it using minimally invasive techniques:

• Laparoscopic surgery - Using small incisions and a camera to guide instruments
• Endoscopic removal - Using a long, flexible tube inserted through the mouth

However, these techniques are often not sufficient for the large hairballs typical in Rapunzel syndrome.

Psychiatric treatment

Addressing the underlying psychiatric issues is crucial to prevent recurrence. This typically involves:

• Cognitive behavioral therapy to change hair-pulling and eating behaviors
• Treatment for any co-existing conditions like anxiety or depression
• Family therapy to address potential contributing factors
• Possibly medication to help control compulsive behaviors

Long-term follow-up is important to ensure the behaviors don’t return.

A case study of Rapunzel syndrome

To illustrate how Rapunzel syndrome can present and be treated, let’s look at a real case study reported by doctors in Mexico:

A 22-year-old woman came to the emergency room with several concerning symptoms:

• Repeated infections related to her dialysis catheter (she had kidney failure and was on dialysis)
• Nausea and vomiting
• Inability to eat
• Abdominal pain
• No bowel movements for 2 days

The patient had a history of pulling out and eating her hair since she was 4 years old. She also had generalized anxiety but wasn’t following any treatment for it.

When examining her abdomen, doctors felt a large mass in the upper part. They ordered some imaging tests:

• An X-ray showed an enlarged stomach area
• A CT scan revealed a large mass filling the entire stomach and extending into the first part of the small intestine

Based on these findings and her history, the doctors diagnosed her with Rapunzel syndrome. They decided to perform surgery to remove the hairball.

During the operation, they found:

• An extremely enlarged stomach
• A massive hairball measuring 52 cm long and 11 cm wide
• The hairball weighed 1.9 kg (about 4.2 pounds)

They successfully removed the entire hairball. After the surgery, the patient recovered well. She was started on a different type of dialysis and referred to psychiatric care to address her hair-eating behavior and anxiety.

This case demonstrates several key features of Rapunzel syndrome:

1. It occurred in a young woman with a psychiatric history
2. The symptoms developed gradually over time
3. Imaging was crucial for diagnosis
4. Surgery was necessary to remove the very large hairball
5. Psychiatric care was an important part of treatment

Conclusions

• Rapunzel syndrome is a rare but serious condition caused by repeatedly eating one’s own hair.
• It most commonly affects young women and is associated with psychiatric disorders.
• Symptoms can be vague at first but may progress to severe complications like intestinal obstruction.
• Diagnosis is typically made through imaging studies, especially CT scans.
• Treatment usually requires surgical removal of the hairball, followed by psychiatric care to prevent recurrence.
• Awareness of this condition is important for both medical professionals and the public to ensure early diagnosis and treatment.