Detangle it – A case of Rapunzel syndrome

13-year-old girl presented with history of abdominal pain on and off since 1 month, vomiting for 5 days and non-passage of flatus and faeces since 1 day. No history of previous abdominal surgery. History of trichotillomania and trichophagia is present.

Ultrasound abdomen revealed telescoping of small bowel loops into one another in left lumbar region. Other solid organs are unremarkable. CT abdomen and pelvis revealed circumscribed heterogenous non-enhancing mass with mottled gas appearance in the stomach and proximal duodenum with resultant gastric and duodenal distension. Telescoping of proximal jejunal loops in left lumbar region and right iliac fossa.

Trichobezoar is common in adolescent girls with trichotillomania and trichophagia and in individuals with gastric motility problems. Bezoar formation occurs due to entrapment of hairs within gastric mucosa. Hair being enzymatically undigestable, eventually mattes together with other indigestible materials and takes on the shape of the stomach over a period of time. Sometimes, its tail may extend into the small intestine, known as Rapunzel syndrome [1]. Symptoms include mass in the abdomen, abdominal pain, nausea, vomiting, weakness, weight loss, constipation, diarrhoea and malnutrition. Rarely, it may complicate as intestinal obstruction, bleeding and perforation [2]. Intestinal obstruction can be the direct effect of the bezoar or secondary to intussusception, wherein hair acts as lead point. The diagnosis can be made out by ultrasound, computerized tomography, barium meal and endoscopy [3]. Endoscopic removal is superior to surgical removal since it is less invasive and cost-effective. Endoscopic retrieval should not be attempted when Rapunzel syndrome is suspected; instead, enterotomy is advised for removal. Laparoscopic approach can be used for large gastric bezoars removal, but it is time-consuming. Trichobezoar could be delivered out through a gastrotomy after reducing the intussusceptions at multiple levels in jejunum and ileum [4]. Psychiatric counselling and follow-up is mandatory to prevent recurrence of this condition [5].  It is a disorder that may be fatal, and early diagnosis and surgical treatment are important [6].

In our case, patient was managed by laprotomy with removal of the bezoar and reduction of volvulus. Patient’s postoperative course was uneventful.