Everyone knows the Brothers Grimm fairy tale, Rapunzel, where a maiden trapped in a tower lets down her incredibly long hair so her handsome prince can climb up and rescue her.
In the 21st century, Rapunzel syndrome means something completely different, and it’s no fairy tale. Named after this very tale, Rapunzel syndrome is a rare medical condition where the hair a person has consumed (yes, eaten) becomes tangled and trapped in their stomach, causing the formation of a trichobezoar.
A trichobezoar is a long tail made entirely of hair, which extends into the small intestine.
South Africa currently has five patients with trichobezoars who are being monitored as part of a study conducted by Groote Schuur Hospital, the University of the Cape Town (UCT) and Netcare Christian Barnard Memorial Hospital.
The five female patients range in age from 12 to 27 years old, and have been admitted to hospital with symptoms including early satiety (the inability to eat a full meal), abdominal pain and weight loss. Their individual diagnoses were confirmed by endoscopy and abdominal CT scans amongst other tests.
“Rapunzel syndrome commonly occurs in young females who usually have an underlying psychiatric disorder, with only a single reported male patient who ate the hair of his sisters,” the experts conducting the study said.
Two of the five patients have trichobezoars that consist entirely of artificial hair extensions, with the longest one measuring 1.4 metres in length.
“In view of the infection risk and considerable size of many of these bezoars, an open removal is probably safer than any minimally invasive attempt,” the experts concluded.
In general people with intellectual disabilities and psychiatric disorders are prime candidates who suffer from Rapunzel syndrome, which consists of two forms – trichotillomania and pica.
People with trichotillomania feel compelled to pull out their hair, often to the point of visible hair loss. It’s very common for people to then play with the removed hair strands. For example, nibbling the root of the hair or mouthing the hair along the lips can feel relaxing.
Pica comes from the Latin word for “magpie”, because of the bird’s unusual eating habits. The disorder involves craving and eating non-nutritious, non-food substances such as clay, dirt, paper, soap, cloth, wool, pebbles and hair.
Approximately 85% to 95% of Rapunzel syndrome patients who suffer from a trichobezoar suffer from abdominal pain, nausea and vomiting. Other symptoms include a bloated stomach, reduced appetite, weight loss and constipation or diarrhea. Extreme cases may lead to a punctured bowel, which, in turn, may lead to sepsis – an infection of the blood.
Death has occurred in approximately 4% of extreme cases.
Picture: Unsplash/Bennie Lukas Bester