What is Rapunzel Syndrome?
is a rare condition in which one encounters a gastrointestinal obstruction caused due to trichophagia or a disorder which entails eating of hair. The hair ball in the gastrointestinal tract is referred to as a trichobezoar. It is associated with psychiatric patients who have a hair pulling disorder namely trichotillomania.
Approximately 37.5 % of patients with trichophagia or hair eating disorder ends up having the trichobezoar.
The ingested hair accumulates in a ball which could be located in the stomach with a tapering tail extending onto the small bowel, namely the jejunum, ileum or the colon. Hair being indigestible and slippery accumulates in an ball and escapes the normal peristalsis (the muscular contractions and relaxations that moves the food) occurring in the stomach.
The condition gets its fancy name after the fairy tale character Rapunzel who would let down her long rope of hair down to allow her prince to climb up to her captive dwelling. Since the mass or bolus tapers off into a tail like structure, it is aptly named the same.
The syndrome is much more common in girls than in boys. The condition is very often seen to onset in early childhood or in adolescence.
Symptoms of Rapunzel Syndrome
- Abdominal pain
- Loss of appetite
- Tenderness in abdomen
- Paleness and weight loss
- Bloating and a sense of being full
- Gastric ulcers
- Intestinal bleeding
- Destruction of nearby tissue in the gastrointestinal tract
- Perforation in the wall of the gastrointestinal tract
Diagnostic Tests to detect the condition
- An abdominal ultrasound can reflect the presence of a mass. However, the presence of bezoars is difficult to tell. Hence further investigations become essential.
- An abdominal CT Scan can detect 97% of the times the presence of bezoars. They appear as free floating filled defects in the gastrointestinal tract.
Treatment of Rapunzel Syndrome
- Surgery becomes essential to treat this condition.
- If the trichobezoar is small, then it can be removed with an endoscope after breaking it up into smaller parts or by dissolving it enzymatically.
- Laproscopically, it can be removed with a minimal incision.
- In case of bezoars which are big ( more than 20 cm), they have to be removed with open surgery.