Rapunzel, Rapunzel, let down your hair extensions

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RapunzelRapunzel syndrome, the rare and extreme presentation of ingested hair, was found in a series of five patients, reports the SA Medical Journal.

According to a Sunday Times report, two hairballs removed from women’s stomachs in Cape Town consisted entirely of hair extensions. One of the hairballs was 1.4m long, and the 19-year-old schoolgirl who had it removed told doctors she had been eating her extensions for four years.

She arrived at Groote Schuur Hospital with abdominal pain, and when doctors opened her up they found the hairball extended into the second part of her small intestine.

The report says the two extension hairballs are among only four similar cases reported worldwide. They are reported by surgeon Jeremy Plaskett and colleagues at the department of surgery, faculty of health sciences, Groote Schuur Hospital and University of Cape Town.

Hairballs that extend into the small intestine are part of Rapunzel syndrome, a “rare and extreme presentation” of an intestinal condition caused by eating hair. It is named after the Brothers Grimm fairy tale. “Complications include gastric ulceration, perforation with peritonitis, obstructive jaundice, acute pancreatitis and even death,” said Plaskett.

“Rapunzel syndrome commonly occurs in young females, who usually have an underlying psychiatric disorder.” About one in 10 people with trichotillomania – a compulsive desire to pull out their own hair – also had trichophagia, an obsession with eating hair.

The condition leads to hairballs when strands of hair get trapped in the folds of the stomach lining. Eventually, hairballs become too large to pass beyond the stomach.

Plaskett’s hair extension cases, and three involving natural hair, involved “highly functional” women whose symptoms included vomiting and abdominal pain, and a 12-year-old with cerebral palsy and anorexia.

Trichobezoars are intraluminal accretions of ingested hair. Rapunzel syndrome is a rare and extreme presentation, with the trichobezoar extending into the small intestine. It is most frequently reported in children and psychiatric patients. We report a South African series of 5 patients who presented with trichobezoars. Each patient was retrospectively reviewed and analysed with regard to background, demographics, clinical presentation, diagnosis, surgical management and complications. Five female patients with a median age of 19 (range 12 – 27) years presented with clinical symptoms, including early satiety, intermittent vomiting with gastric outlet obstruction, abdominal pain and weight loss. The diagnosis was made by endoscopy, abdominal computed tomography (CT) imaging, barium meal examination or plain abdominal radiography. Two patients presented with sealed/contained gastric perforations, and 1 patient with a small-bowel perforation. All 5 bezoars, 2 of which consisted entirely of artificial hair extensions, extended into the jejunum, the longest measuring 1.4 m. All were removed by laparotomy. While trichobezoars are a rare entity, they may present with significant complications, such as obstructions and perforations. 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.

J Plaskett, G Chinnery, D Thomson, S Thomson, B Dedekind, E Jonas

Sunday Times report
South African Medical Journal abstract

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