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Case Reports
. 2018 Apr 4;18(1):125.
doi: 10.1186/s12887-018-1097-8.

Rapunzel syndrome: an infrequent cause of severe iron deficiency anemia and abdominal pain presenting to the pediatric emergency department

Giuseppe Cannalire  1 , Luigi Conti  2 , Maurizio Celoni  2 , Carmine Grassi  2 , Andrea Cella  3 , Giulia Bensi  3 , Patrizio Capelli  2 , Giacomo Biasucci  3
Affiliations
Case Reports

Rapunzel syndrome: an infrequent cause of severe iron deficiency anemia and abdominal pain presenting to the pediatric emergency department

Giuseppe Cannalire et al. BMC Pediatr. .

Abstract

Background: Iron deficiency anemia (IDA) and abdominal pain are commonly seen in a pediatric emergency department (8 and 18% incidence respectively in our center). They are manifestations of a wide variety of diseases ranging from benign to immediately life-threatening. Trichobezoar is an under-diagnosed entity that has to be considered in children and adolescents, expecially female, suffering from trichotillomania (compulsion to pull hair) and trichophagy (compulsion to swallow hair). When undiagnosed, gastric bezoars may cause gastric ulceration, perforation, haemorrhage and obstruction.

Case presentation: To underline the importance of including this pathology in the differential diagnosis of IDA and abdominal pain, we present the case of a 14 year-old girl with a huge trichobezoar which completely filled the stomach and extended into the small bowel. Since trichobezoar has an extension to the small bowel, it is classified as Rapunzel syndrome. As the bezoar couldn't be removed by endoscopy, the girl underwent surgical intervention. The patient passed through a gradual re-feeding, with iron and vitamins supplementation, and through a psychiatric counselling.

Conclusion: The Rapunzel syndrome is a rare entity that may be complicated by life-threatening events. A prompt diagnosis and an appropriate therapy can reduce comorbidities. Gradual re-feeding with supplementation of micronutrients allows adequate catch-up weight with normalization of haematochemical nutritional parameters. Since many of these patients suffer from psychiatric pathology such as PICA with emotional problems and mental retardation, psychological/psychiatric counselling plays an important role in order to prevent bezoar recurrence.

Keywords: Abdominal pain; Case report; Iron deficiency anemia; PICA; Rapunzel syndrome; Trichobezoar; Trichophagia; Trichotillomania.

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Conflict of interest statement

Ethical approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient’s parents for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
CT, Coronal and sagittal view: huge, well defined, multi-layered, heterogeneous, solid appearing, mass filling the gastric fundus and antrum and extending into duodenum. Since trichobezoar has an extension to the small bowel, it is classified as Rapunzel syndrome
Fig. 2
Fig. 2
Gastrointestinal endoscopy: large black and hard matter occupying lumen of stomach
Fig. 3
Fig. 3
Huge, intra-luminal solid mass excised from the stomach
See this image and copyright information in PMC

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