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...
Main Authors: | , , , , |
---|---|
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 |
_version_ | 1818667534321188864 |
---|---|
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. |
first_indexed | 2024-12-17T06:21:57Z |
format | Article |
id | doaj.art-c6aa950ccb16476a971145ee6575d809 |
institution | Directory Open Access Journal |
issn | 2036-749X 2036-7503 |
language | English |
last_indexed | 2024-12-17T06:21:57Z |
publishDate | 2018-06-01 |
publisher | MDPI AG |
record_format | Article |
series | Pediatric Reports |
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 |
work_keys_str_mv | AT enricofinale rapunzelsyndromehowtoorientthediagnosis AT piergiorgiofranceschini rapunzelsyndromehowtoorientthediagnosis AT cesaredanesino rapunzelsyndromehowtoorientthediagnosis AT michelangelobarbaglia rapunzelsyndromehowtoorientthediagnosis AT andreaguala rapunzelsyndromehowtoorientthediagnosis |