“Rapunzel, Rapunzel, let down your hair…..”How often have we read this popular fairytale to our little ones about a princess being held captive in a high tower and how when her prince comes along she throws down her lovely golden tresses so that he can use her plait as a rope to climb up and be with her.
But how many of us are familiar with Rapunzel Syndrome, a rare illness linked to the eating of one’s own hair and the formation of a Trichobezoar or hairball in the stomach of the victim.
|Just after extrication: Dr. Goonetilleke holding the wig-like hairball
And that’s what Consultant Surgeon Dr. Gamini Goonetilleke of the Sri Jayewardenepura General Hospital found when he opened up the stomach of a 14-year-old girl on June 12. “This is a very rare condition,” stressed Dr. Goonetilleke, explaining that only 27 cases have been reported worldwide.
The first-ever in Sri Lanka is the 28th case globally, he says.
For the Sri Lankan teenager’s family, indications of all not being right came about a year ago, The Sunday Times understands.
The girl living in the outstations was the fourth in a family of four children. Although she had undergone a heart operation (to correct Patent Ductus Arteriosus) at Sri Jayewardenepura Hospital many years ago there were no problems thereafter.
“Suddenly, the girl had lost her appetite. She was feeling nauseous. There were recurrent bouts of pain in her abdomen and she began losing weight,” says Dr. Goonetilleke.
Alarmed, her parents took her to the hospital closest to their home. Many were the hospital visits and medicines prescribed. When the pain became unbearable, the doctors did a stomach scan but that did not reveal anything. As that hospital did not have the facilities to do a CT scan, they referred her to another. But no diagnosis could be made even after that.As the symptoms persisted, the teenager was referred to Dr. Goonetilleke by the doctors at the first hospital, on June 10.
“When she came here she was emaciated and malnourished. She had lost a lot of weight,” says Dr. Goonetilleke, who suspecting that it could be a growth in her stomach which was causing a block, immediately admitted her and intravenously administered medication to reduce the pain.
An upper gastrointestinal endoscopic examination followed.
“The diagnosis was clinched on that,” says Dr. Goonetilleke, adding that this rare condition is mainly reported in textbooks with specimens of Trichobezoar being seen only in pathological museums.
Caused by the psychiatric condition Trichotillomania, it is an obsessive compulsive disorder which makes one eat one’s own hair, he says, explaining that as it is very difficult to eat hair, what the patient usually does is pull out tufts of hair from the head or eyebrows, make them into small balls and then swallow them.
When these balls go into the stomach, the usual digestion process attempts to take place. But as hair cannot be digested, it forms into a kind of mesh or wig, gradually taking the shape of the stomach. When the stomach is full of this wig-like hair clump, it overflows into the duodenum and then even extends into the small intestine. “Then it is called Rapunzel Syndrome,” says Dr. Goonetilleke.
In the worst-case scenario such clumps of hair can cause intestinal obstruction, leading to perforation of the bowel and peritonitis, The Sunday Times understands.
The 45-minute operation on the teenager on June 12, saw 700 grams of hair running a length of 75 centimetres being pulled out of her stomach, duodenum and small intestine. It can recur in rare cases, says Dr. Goonetilleke, adding that the girl needs psychological support. “She has promised her parents that she will never eat hair again.”
Rapunzel Syndrome: It’s no fairytale
Rapunzel Syndrome (also known as Trichobezoar) is an extremely rare intestinal condition in humans resulting from the consumption of hair. It is named after the long-haired character Rapunzel in the fairy tale by the Brothers Grimm, says Wikipedia.
A bezoar is a ball of swallowed foreign material (usually hair or fibre) that collects in the stomach and fails to pass through the intestines. Chewing or eating hair or fuzzy materials (or indigestible materials such as plastic bags) can lead to the formation of a bezoar. The rate is very low and the risk is greater among emotionally disturbed children. Generally bezoars are mostly seen in females aged 10 to 19.
Meanwhile, Trichotillomania or "trich" as it is commonly known, is an impulse control disorder characterized by the repeated urge to pull out scalp hair, eyelashes, facial hair, nose hair, eyebrows or other body hair, sometimes resulting in noticeable bald patches. Trichotillomania though classified as an impulse control disorder may seem, at times, to resemble a habit, an addiction, a tic disorder or an obsessive-compulsive disorder, it is learnt.
Trichotillomania seems to strike most frequently in the pre- or early adolescent years. The typical first-time hair puller is 12 years old, although it has affected people as young as one and as old as seventy.
The name derives from Greek: tricho- (hair), till(en) (to pull), and mania.