One of five patients diagnosed with “Rapunzel syndrome” had a mass trapped in her gastrointestinal system formed from the ingestion of hair measuring 1.4m.
This emerged in a study conducted by experts from Groote Schuur Hospital, UCT and Netcare Christiaan
Barnard Memorial Hospital on five patients with Rapunzel syndrome, a rare and extreme presentation of trichobezoars, over five years.
A trichobezoar is a mass found trapped in the gastrointestinal system following the ingestion of hair.
All masses in the five female patients between the ages of 12 - 27, two of which consisted entirely of artificial hair extensions, were found to extend into the middle segment of the small intestine.
According to the experts, trichobezoars are most frequently reported in children and psychiatric patients.
“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 said.
Each of the five patients were retrospectively reviewed and analysed with regard to background, demographics, clinical presentation, diagnosis, surgical management and complications.
The diagnoses were made using a combination of endoscopy, abdominal computed tomography imaging, barium meal
examination and plain
abdominal radiography.
“Common presenting complaints include abdominal pain, nausea and vomiting, and gastric outlet obstruction. Patients may remain asymptomatic for many years. Less commonly, patients may present with weight loss, anorexia, haematemesis and intussusception. Complications include gastric ulceration, perforation with peritonitis, obstructive jaundice, acute pancreatitis and even death,” they said.
Two of the women presented with sealed/contained gastric perforations, also known as ruptured bowel (a hole in the wall of part of the gastrointestinal tract) and one patient with a small bowel
perforation.
All five patients were
successfully treated with a laparotomy, involving a large incision through the abdominal wall.
All patients had an uncomplicated post-operative course and four were referred for psychiatric evaluation.
“Trichobezoar and Rapunzel syndrome should be considered as a differential diagnosis in young females who present with non-specific symptoms such as epigastric pain, fatigue and weight loss, and an epigastric mass.
"A thorough history regarding any psychiatric comorbidity, as well as a history of trichotillomania/trichophagia, should be sought, although most patients deny this even when specifically asked. Severe halitosis or patchy alopecia may be an indication of the condition,” the experts said.
“The goals of bezoar treatment are the removal of the bezoar and the prevention of recurrence by addressing
the underlying psychiatric/emotional cause.
“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.”