Rapunzel syndrome
Why in News?
Rapunzel syndrome is in the news because doctors at NIMS Medicity in Kerala, India, recently performed a rare, successful surgery to remove a 1-kg mass of hair from the stomach and small intestine of a nine-year-old girl.
Overview & History
- Definition: It is an extremely rare and severe intestinal condition where a large hairball (known medically as a trichobezoar) forms inside the stomach and develops a long, tail-like extension that projects into the small intestine, colon, or bowel.
- Origin of Name: The syndrome was first described in 1968. It is named after the Grimm Brothers' fairy-tale character Rapunzel, who was famous for her long hair.
- Rarity: It is exceptionally uncommon, with fewer than 100 cases reported worldwide in medical history.
Primary Causes
The condition is driven by two tightly linked psychological or emotional disorders:
The condition is driven by two tightly linked psychological or emotional disorders:
- Trichotillomania: An impulse-control disorder where an individual feels an uncontrollable, repetitive urge to pull out their own hair.
- Trichophagia: A compulsive behaviour where a person swallows the hair they have pulled out or found loose.
- The Digestion Problem: Human hair cannot be broken down by human digestive enzymes. Because it is smooth and slippery, the stomach's squeezing muscles (peristalsis) cannot grab it. Over time, the hair becomes trapped in the folds of the stomach, tangling with food and mucus until it hardens into a dense mass.
Affected Demographics
- Gender: It is predominantly seen in females, who account for roughly 90% of all cases.
- Age: It mostly impacts children, adolescent girls, and young women under the age of 30 (most commonly between ages 4 and 19).
- Mental Health Associations: It is frequently associated with underlying emotional stress, history of childhood trauma, intellectual disabilities, or psychiatric conditions like depression, OCD, and anorexia nervosa.
Common Symptoms
The hairball can remain completely symptomless for years while it grows. Once it expands enough to block the digestive tract, it causes:
The hairball can remain completely symptomless for years while it grows. Once it expands enough to block the digestive tract, it causes:
- Persistent stomach pain and abdominal bloating
- Chronic nausea and vomiting after eating meals
- A constant feeling of fullness and loss of appetite
- Significant, unexplained weight loss or malnutrition
- Physical Clues: Patchy hair loss (alopecia) on the scalp and severe bad breath (halitosis) due to the decaying food trapped in the stomach mass.
Treatment & Prevention
- Medical Removal: While small hairballs can sometimes be broken down and extracted using an endoscope, large masses (like the 1-kg mass found in the Kerala case) require open surgery (laparotomy) to safely cut open the stomach and pull the hair out intact.
- Psychiatric Care: Physical surgery only treats the symptom, not the root cause. Long-term prevention requires ongoing multi-disciplinary mental health treatment, such as Cognitive Behavioural Therapy (CBT) and medication, to help the patient stop the underlying urge to pull and eat hair.
