Surgeons Remove 2.5-Inch Hairball from Adolescent Diagnosed with Uncommon Rapunzel Syndrome

Surgeons Remove 2.5-Inch Hairball from Adolescent Diagnosed with Uncommon Rapunzel Syndrome

Surgeons Remove 2.5-Inch Hairball from Adolescent Diagnosed with Uncommon Rapunzel Syndrome


### Examining Rapunzel Syndrome: A Rare and Disturbing Medical Enigma

Medical enigmas often pose challenges even for the most experienced physicians, and the case of a 16-year-old girl experiencing unexplained abdominal discomfort is a prime example. What began as a routine visit to the emergency room at Massachusetts General Hospital transformed into an unusual diagnosis of **Rapunzel syndrome**, a condition as strange as its designation. This article explores the particulars of her case, the underlying factors contributing to this rare disorder, and the wider implications for both mental and physical health.

### The Case: A Month of Mysterious Discomfort

The adolescent presented at the hospital in intense pain, actively retching, and unable to retain medication. Despite her generally healthy background, her symptoms had persisted for a month, with no identifiable cause. Previous visits to another medical facility had resulted in normal findings from CT scans, blood examinations, and urine tests. Painkillers and medications for peptic ulcers and nausea had been prescribed, but her situation only deteriorated.

When she arrived at Massachusetts General, her elevated pulse and tender abdomen raised concern. Initially, physicians considered common sources of abdominal pain, like gastritis, constipation, or lactose intolerance. However, these possibilities were ruled out based on her symptoms and imaging findings. With no fever, diarrhea, or recent infections, even uncommon conditions such as gallbladder disease and pancreatitis were excluded.

### The Diagnosis: A Hairy Offender

As her medical team investigated less typical options, they focused on mechanical obstructions in her gastrointestinal tract. This led them to suspect a **bezoar**, a foreign mass composed of ingested material. Bezoars can manifest in various types:

– **Phytobezoars**: Formed from indigestible plant fibers.
– **Pharmacobezoars**: Created from pharmaceutical products like extended-release capsules or coated medications.
– **Trichobezoars**: Made of hair.

Hair, distinct from food, resists digestion and can gather in the stomach over time. It can become matted and trap other substances, resulting in a dense, tangled mass. In this instance, doctors conducted an esophagogastroduodenoscopy (EGD), a procedure using a scope to scrutinize the stomach. The findings were surprising: the girl had a **trichobezoar**—a hairball lodged within her stomach.

### Rapunzel Syndrome: When Hair Protrudes Beyond the Stomach

What rendered this case especially unusual was the finding that strands of hair from the trichobezoar extended from the stomach into the small intestine. This condition, known as **Rapunzel syndrome**, is named after the fairy-tale character Rapunzel, who famously let down her long hair. While trichobezoars are already rare, Rapunzel syndrome is even less common. It can result in severe complications, such as:

– **Acute abdominal pain**: Caused by intermittent obstruction of the gastrointestinal tract.
– **Perforation**: Tears in the stomach or intestinal lining.
– **Acute pancreatitis**: Inflammation of the pancreas triggered by obstruction.

In the adolescent’s situation, the trichobezoar was surgically excised using a gastrostomy tube. The hairball measured roughly 2.5 inches in width, with strands extending into the small intestine.

### The Underlying Causes: Mental Health and Behavioral Issues

The development of a trichobezoar frequently indicates underlying psychological conditions. In particular, **trichotillomania** and **trichophagia** are closely associated:

– **Trichotillomania**: A repetitive behavior disorder defined by compulsive hair-pulling.
– **Trichophagia**: The act of ingesting the pulled hair, which can lead to the creation of trichobezoars.

Individuals with trichotillomania often experience shame and embarrassment, complicating diagnosis. Physical indicators, such as bald spots or irritated scalp regions, may offer clues, but many individuals go to great lengths to conceal their behavior.

Another possible explanation is **pica**, a disorder characterized by the chronic consumption of nonfood items. Although the teen mentioned having pica as a child, her doctors were doubtful it accounted for her current condition, as the bezoar was solely composed of hair.

### Treatment and Recovery: A Missed Chance

The surgical removal of the trichobezoar alleviated the teen’s immediate symptoms, but her doctors stressed the necessity of addressing the underlying psychiatric issues to avert recurrence. They referred her to a psychiatrist for continued care. However, the teen chose not to return for her appointments, instead seeking out a hypnotherapist suggested by friends.

This choice underscores a vital obstacle in treating conditions like Rapunzel syndrome: the stigma and hesitation surrounding mental health treatment. Without addressing the fundamental cause, patients remain vulnerable to recurrence and further complications.