The human being can develop a wide range of abnormal behaviors, and one of the most striking is trichophagia, or the compulsive ingestion of hair. Usually associated with trichotillomania (hair pulling), trichophagia can trigger a much more serious condition known as Rapunzel Syndrome. In essence, a huge ball of hair forms in the stomach, and without direct intervention, it could cause death…
It All Starts with a Hairball…
The concept of bezoar describes the “accumulation of some non-digestible substance” in the stomach or intestine, and in the specific case of trichobezoar, it is a ball of hair. Anyone who has cats knows the process of expelling a trichobezoar, but it is not exclusive to felines. In fact, these accumulations can also appear in humans, with much more serious consequences.
Today's story takes us to Nottingham University Hospital. Its emergency room received a 17-year-old girl after she fainted twice. The girl explained that she had suffered irregular stomach pains over the last five months, but in the two weeks prior to admission, her condition worsened. The doctors noticed that the girl had several episodes over the last three years of trichotillomania and trichophagia, along with weight loss. That led them to order a CT scan, and they discovered a “gastric mass” completely made of human hair. This massive trichobezoar measured about 48 centimeters, causing perforation of the stomach wall, and abdominal sepsis with several pus accumulations. An emergency laparoscopic surgery removed the huge hairball, and a series of intravenous antibiotics neutralized the infection. Medicine has a name for all this: Rapunzel Syndrome.
The Rapunzel Syndrome
The name is associated with the classic Brothers Grimm story, but its use is relatively modern, with the first documented cases in 1968. The accumulation of hair in the stomach is the most important symptom; however, characteristics such as nausea and vomiting, intestinal obstructions, vitamin B12 deficiency, and pancreatic necrosis are also detected. The variant suffered by the young woman from Nottingham presents a fairly compact and contained hairball, but in other patients it can even reach the colon, forming a long extension (just like in the tale).
Beyond the associated psychiatric problems, this happens because hair is highly resistant to digestive enzymes and just keeps accumulating. Even so, texts highlight Rapunzel Syndrome as a very rare occurrence: The people at NORD (National Organization for Rare Disorders) explain that trichotillomania affects up to 3 percent of the population, and trichophagia is even rarer. Only 1 percent of those who suffer from both conditions end up forming a hairball.
Now, that doesn't eliminate the potentially lethal profile of Rapunzel Syndrome. A 16-year-old girl lost her life in September 2017 due to the general failure of her organs, caused by an infected hairball in her stomach.
Sources: Health.com, BMJ Journals
