By pulling out her hair and swallowing it, a 17-year-old woman developed a trichobezoar, a huge ball of hair that ended up perforating 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 is rather a nightmarish case of this strange illness that the medical journal reports BMJ from February 8.
A 17-year-old girl presents to the emergency room of Queen’s Medical Center from Nottingham after two episodes of syncope which led to a fall with facial bruising and a scalp hematoma. Doctors, who first aim to exclude an intracranial lesion, then notice a curious massmass at his abdomen. During interrogation, the young girl confirmed that she had suffered from intermittent abdominal pain for the past five months, which worsened over the last two weeks. She explains having lost weight, while denying any change in diet or loss of appetite.
A 50 centimeter ball of hair
A to scanto scan abdominal then reveals a huge ball of hair measuring 48 x 5 x 7 cm in its stomachstomach, which perforated the intestinal wall by two centimeters. Multiple pockets of pus and fibrinfibrin (a proteinprotein filamentous which forms during the coagulationcoagulation blood sores) formed between the stomach, very distended, the missedmissedTHE diaphragmdiaphragm and the liverliver LEFT. The mass of hair is so enormous that it forms a sort of “cast” of the stomach and the duodenumduodenum. Doctors are then forced to perform a gastrotomy, then purge the abdomen with copious washing with saline water. The patient is then fed via jejunostomy, which involves placing a feeding tube directly through the skin and stomach wall. She will finally leave the hospital seven days later, with medical monitoring for 30 days.
Trichobezoar: between 0.4% and 1% of people affected
This mass of hair, called a trichobezoar (or trichobezoan), did not land in the young girl’s stomach by chance. She indeed suffers from Rapunzel syndrome, which combines trichotillomaniatrichotillomania (compulsive hair pulling) to trichophagia (hair swallowing). Hair is not digestible by enzymesenzymes gastric or eliminated by the peristalsisperistalsis natural due to their “slippery” nature, they end up accumulating in the stomach to form a ball which can take on impressive proportions: we have sometimes removed masses of hair of up to three kilogramskilogramswith ramifications extending 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, with 79% of cases being under 20 years old. According to a 2018 study, around sixty cases have been reported in the medical literature since the discovery of this syndrome in 1968.
Rapunzel syndrome: symptoms and complications
THE symptomssymptoms Typical trichobezoar symptoms include halitosis (bad breath), abdominal pain, nausea, vomiting, loss of appetite and weight. The trichobezoar can give rise to complications such as gastric ulcer, intestinal obstruction or even perforation, diarrheadiarrhea and an deficiencydeficiency in vitamin B12 (due to bacterial colonization of the hair). In 2017, a 16-year-old British girl even died of Rapunzel syndrome after a peritonitisperitonitis which led to failure of his vital organs. A much less happy ending than in the Disney film, where Rapunzel and her prince charming married and lived happily.
