Rapunzel Syndrome: Rare Trichobezoar Disorder

Science and Tech

Rapunzel Syndrome: Rare Trichobezoar Disorder

Context

  • Doctors in Kerala recently removed nearly 1 kg of hair from the stomach and small intestine of a nine-year-old girl diagnosed with Rapunzel syndrome.

How Rapunzel Syndrome Develops and Presents

  • Rapunzel syndrome is a rare condition in which a trichobezoar, or mass of swallowed hair, forms in the stomach and extends into the small intestine.
  • Human hair is resistant to digestion, so repeated swallowing allows it to accumulate and gradually form a dense mass.
  • The condition is commonly associated with trichophagia (hair eating) and may occur along with trichotillomania (recurrent hair-pulling behaviour).
  • It is seen mainly in children, adolescents and young women.
  • Common symptoms include abdominal pain, vomiting, bloating, early fullness, poor appetite and weight loss.
  • Large hair masses can cause intestinal obstruction, nutritional deficiency, ulceration or perforation if diagnosis is delayed.
  • Patchy hair loss may provide an external clue when repeated hair pulling is present.

Management and Recurrence Prevention

  • Diagnosis usually combines clinical examination, imaging and endoscopy to identify the size and extent of the hair mass.
  • Small trichobezoars may sometimes be removed endoscopically, while large masses extending into the intestine usually require surgical removal.
  • Early diagnosis is important because symptoms can initially resemble common gastrointestinal disorders, allowing the mass to enlarge unnoticed.
  • Treatment should also address the underlying behaviour where present, through psychological assessment and behavioural therapy.
  • Long-term follow-up is important because recurrence may occur if hair-pulling or hair-eating behaviour continues.
  • The condition therefore requires an integrated approach combining surgical or gastrointestinal treatment with behavioural care.

FAQs

Q1. What is Rapunzel syndrome?
It is a rare condition in which a hair mass forms in the stomach and extends into the small intestine.

Q2. What is a trichobezoar?
It is a compact mass of swallowed hair present in the gastrointestinal tract.

Q3. What is trichophagia?
It refers to the repeated swallowing of hair.

Q4. What are the common symptoms?
They include abdominal pain, vomiting, bloating, early fullness and weight loss.

Q5. How is Rapunzel syndrome treated?
Large masses usually require surgical removal, along with behavioural evaluation where needed.