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Pediatric Reports
Volume 10
Issue 2
10.4081/pr.2018.7689
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Case Report

Rapunzel Syndrome: How to Orient the Diagnosis

by
Enrico Finale
1,2,*,
Piergiorgio Franceschini
3,
Cesare Danesino
4,
Michelangelo Barbaglia
1 and
Andrea Guala
1
1
Maternal-Child Department, ASL Verbano-Cusio-Ossola
2
Pediatrics Department, Castelli Hospital, Verbania
3
University of Turin; 4Department of Molecular Medicine, University of Pavia, Italy
*
Author to whom correspondence should be addressed.
Pediatr. Rep. 2018, 10(2), 7689; https://doi.org/10.4081/pr.2018.7689
Submission received: 23 March 2018 / Accepted: 24 June 2018 / Published: 27 June 2018

Abstract

Rapunzel syndrome is a rare form of tricobezoar with a tail extending from the stomach into the small bowel; surgical removal is generally required. About 60 cases have been reported and described in the literature since 1968. We present the case of an 8-year-old girl who, during the course of a genetic consultation, was initially assigned with a clinical suspicion of ectodermal dysplasia. Surgical intervention, which resulted in the extraction of a tricobezoar of enormous size and extension, led us to the diagnosis of Rapunzel syndrome. The possibility of a tricobezoar should be considered in all cases of adolescent patients who present signs of intestinal occlusion or sub-occlusion, suffer from psychiatric disorders, and have a history of trichotillomania. Endoscopic methodology, medical imaging and clinical diagnostics are fundamental for a differential diagnosis. Psychiatric follow-up is advised to prevent recurrence.
Keywords: Rapunzel syndrome; diagnosis Rapunzel syndrome; diagnosis

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MDPI and ACS Style

Finale, E.; Franceschini, P.; Danesino, C.; Barbaglia, M.; Guala, A. Rapunzel Syndrome: How to Orient the Diagnosis. Pediatr. Rep. 2018, 10, 7689. https://doi.org/10.4081/pr.2018.7689

AMA Style

Finale E, Franceschini P, Danesino C, Barbaglia M, Guala A. Rapunzel Syndrome: How to Orient the Diagnosis. Pediatric Reports. 2018; 10(2):7689. https://doi.org/10.4081/pr.2018.7689

Chicago/Turabian Style

Finale, Enrico, Piergiorgio Franceschini, Cesare Danesino, Michelangelo Barbaglia, and Andrea Guala. 2018. "Rapunzel Syndrome: How to Orient the Diagnosis" Pediatric Reports 10, no. 2: 7689. https://doi.org/10.4081/pr.2018.7689

APA Style

Finale, E., Franceschini, P., Danesino, C., Barbaglia, M., & Guala, A. (2018). Rapunzel Syndrome: How to Orient the Diagnosis. Pediatric Reports, 10(2), 7689. https://doi.org/10.4081/pr.2018.7689

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