Rapunzel Syndrome-An Extremely Rare Cause of Digestive Symptoms in Children: A Case Report and a Review of the Literature

Abstract

Rapunzel syndrome is an extremely rare condition seen in adolescents or young females with psychiatric disorders consisting of a gastric trichobezoar with an extension within the small bowel. The delays in diagnosis are common since in its early stages, it is usually asymptomatic. We report the case of a 13-year-old girl admitted in our clinic for abdominal pain, anorexia, and weight loss. The clinical exam pointed out diffuse alopecia, a palpable mass in the epigastric area, and abdominal tenderness at palpation, the patient weighing 32 kg. The laboratory tests showed anemia. The abdominal ultrasound showed a gastric intraluminal mass with a superior hyperechoic arc. The upper digestive endoscopy revealed a mass formed by hair, mucus, and food occupying the gastric cavity with the extension into the duodenum confirming the diagnosis of Rapunzel syndrome. The giant trichobezoar of 511 g, measuring 17 × 7 × 6.5 cm with a tail of approximately 3 cm, was successfully removed through laparotomy. Although rare, Rapunzel syndrome must never be forgotten as a differential diagnosis for digestive symptoms since its early detection hinders the occurrence of further complications.

Keywords: Rapunzel syndrome; children; trichobezoar; trichophagia; trichotillomania.

Figure 1

Endoscopic aspect of trichobezoar—at the level of gastric corpus.

Figure 2

Endoscopic aspect of trichobezoar—at the level of gastric corpus and antrum.

Figure 3

Intraoperator aspect of trichobezoar.

Figure 4

Macroscopic aspect of hole trichobezoar.