Rapunzel syndrome: how to orient the diagnosis

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 gen...

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Main Authors: Enrico Finale, Piergiorgio Franceschini, Cesare Danesino, Michelangelo Barbaglia, Andrea Guala
Format: Article
Language:English
Published: MDPI AG 2018-06-01
Series:Pediatric Reports
Subjects:
Online Access:https://www.pagepress.org/journals/index.php/pr/article/view/7689
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author Enrico Finale
Piergiorgio Franceschini
Cesare Danesino
Michelangelo Barbaglia
Andrea Guala
author_facet Enrico Finale
Piergiorgio Franceschini
Cesare Danesino
Michelangelo Barbaglia
Andrea Guala
author_sort Enrico Finale
collection DOAJ
description 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.
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spelling doaj.art-c6aa950ccb16476a971145ee6575d8092022-12-21T22:00:23ZengMDPI AGPediatric Reports2036-749X2036-75032018-06-0110210.4081/pr.2018.76893970Rapunzel syndrome: how to orient the diagnosisEnrico Finale0Piergiorgio Franceschini1Cesare Danesino2Michelangelo Barbaglia3Andrea Guala4Maternal-Child Department, ASL Verbano-Cusio-Ossola; Pediatrics Department, Castelli Hospital, VerbaniaUniversity of TurinDepartment of Molecular Medicine, University of PaviaMaternal-Child Department, ASL Verbano-Cusio-OssolaMaternal-Child Department, ASL Verbano-Cusio-OssolaRapunzel 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.https://www.pagepress.org/journals/index.php/pr/article/view/7689Rapunzel syndromediagnosis.
spellingShingle Enrico Finale
Piergiorgio Franceschini
Cesare Danesino
Michelangelo Barbaglia
Andrea Guala
Rapunzel syndrome: how to orient the diagnosis
Pediatric Reports
Rapunzel syndrome
diagnosis.
title Rapunzel syndrome: how to orient the diagnosis
title_full Rapunzel syndrome: how to orient the diagnosis
title_fullStr Rapunzel syndrome: how to orient the diagnosis
title_full_unstemmed Rapunzel syndrome: how to orient the diagnosis
title_short Rapunzel syndrome: how to orient the diagnosis
title_sort rapunzel syndrome how to orient the diagnosis
topic Rapunzel syndrome
diagnosis.
url https://www.pagepress.org/journals/index.php/pr/article/view/7689
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