By pulling out her hair and swallowing it, a 17-year-old woman developed a trichobezoar, a huge hairball that eventually perforated her stomach. A pathology nicknamed Rapunzel syndrome, of which there are barely sixty cases.
Rapunzel syndrome, named after the Disney princess with very long golden hair, is not a fairy tale. It’s quite a nightmarish case of this strange disease reported in the medical journal BMJ from February 8.
A 17-year-old girl presents to the emergency department Queen Medical Center from Nottingham after two episodes of syncope which led to a fall with facial bruising and a hematoma on the scalp. Doctors, who first want to rule out an intracranial lesion, then notice something strange massmass near his stomach. During questioning, the young girl confirmed that she had been suffering from intermittent abdominal pain for the past five months, which worsened over the past two weeks. She explains that she has lost weight, but denies any change in diet or loss of appetite.
A hairball of 50 centimeters
a scanscan abdomen then reveals a huge hairball measuring 48 x 5 x 7 cm stomachstomach, which perforated the intestinal wall by two centimeters. Multiple pockets of pus and fibrinfibrin (A egg whiteegg white thread-like that forms during the coagulationcoagulation blood ulcers) formed between the stomach, very swollen, the missedmissedTHE apertureaperture and the liverliver LEFT. The mass of hair is so enormous that it forms a kind of ‘cast’ of the stomach and abdomen duodenumduodenum. Doctors are then forced to perform a gastrotomy and then cleanse the abdomen by washing copiously with salt water. The patient is then fed through a jejunostomy, in which a feeding tube is placed directly through the skin and stomach wall. She will finally leave the hospital seven days later, under medical supervision for 30 days.
Trichobezoar: between 0.4% and 1% of people affected
This mass of hair, called trichobezoar (or trichobezoan), did not end up in the young girl’s stomach by chance. She does indeed suffer from Rapunzel Syndrome, which combines trichotillomaniatrichotillomania (compulsive hair pulling) to trichophagia (hair swallowing). Hair is not digestible by enzymesenzymes stomach or eliminated by the peristalsisperistalsis Naturally due to their “slippery” nature, they accumulate in the stomach, forming a ball that can assume impressive proportions: we have sometimes removed masses of hair as large as three kilogramskilogramswith consequences that extend into thesmall intestinesmall intestine.
Did you know ?
Between 0.5% and 3% of people suffer from trichotillomania, but only 10% to 30% of these also develop trichophagia. Rapunzel syndrome mainly affects young women; 79% of cases are younger than 20 years old. According to a 2018 study, approximately sixty cases have been reported in the medical literature since the syndrome was discovered in 1968.
Rapunzel syndrome: symptoms and complications
THE symptomssymptoms Typical symptoms of trichobezoar include halitosis (bad breath), abdominal pain, nausea, vomiting, loss of appetite and weight loss. The trichobezoar can lead to complications such as stomach ulcers, intestinal obstruction or even perforation, diarrheadiarrhea and a shortageshortage in vitamin B12 (due to bacterial colonization of the hair). In 2017, a 16-year-old British girl even died from Rapunzel Syndrome after a… peritonitisperitonitis which led to the failure of his vital organs. A much less happy ending than in the Disney film, where Rapunzel and her prince charming got married and lived happily.