Challenges in the management of caudal duplication syndrome

Caudal duplication syndrome (CDS) is a rare developmental anomaly in which embryonic cloaca and notochord structures are duplicated [1]. Due to the diverse clinical manifestation and rarity of CDS, it is crucial to report every case and to share experience and outcomes of individually adjusted manag...

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Autores principales: Laine Bekere, Ainars Gilis, Zane Abola, Lasma Lidaka
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Lenguaje:EN
Publicado: Elsevier 2021
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Acceso en línea:https://doaj.org/article/d644981adadb4e35b919ada13ae6e378
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spelling oai:doaj.org-article:d644981adadb4e35b919ada13ae6e3782021-11-24T04:29:59ZChallenges in the management of caudal duplication syndrome2213-576610.1016/j.epsc.2021.102083https://doaj.org/article/d644981adadb4e35b919ada13ae6e3782021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2213576621003043https://doaj.org/toc/2213-5766Caudal duplication syndrome (CDS) is a rare developmental anomaly in which embryonic cloaca and notochord structures are duplicated [1]. Due to the diverse clinical manifestation and rarity of CDS, it is crucial to report every case and to share experience and outcomes of individually adjusted management plans.We report here the case of a 2-year-old girl born with duplication of the urogenital (bladder, urethra, uterus, vagina, vulva) and gastrointestinal (gallbladder, appendix vermiformis) systems. Additionally, coccygeal agenesis, lipomyelomeningocele and vertical talus were present. A thorough examination and urological reconstructive surgery were performed.While there may be a desire from patients, parents and healthcare specialists to modify all malformations to an anatomically correct state, the current opinion is that only anatomical variants that influence function should be modified. Consent from patients should be sought for decisions regarding more sensitive matters such as vulva surgery for cosmetic reasons or correction of anatomical variants without functional consequences.Laine BekereAinars GilisZane AbolaLasma LidakaElsevierarticleCaudal duplication syndromeDouble vulvaDouble urinary bladderReconstructive surgeryPediatricsRJ1-570SurgeryRD1-811ENJournal of Pediatric Surgery Case Reports, Vol 75, Iss , Pp 102083- (2021)
institution DOAJ
collection DOAJ
language EN
topic Caudal duplication syndrome
Double vulva
Double urinary bladder
Reconstructive surgery
Pediatrics
RJ1-570
Surgery
RD1-811
spellingShingle Caudal duplication syndrome
Double vulva
Double urinary bladder
Reconstructive surgery
Pediatrics
RJ1-570
Surgery
RD1-811
Laine Bekere
Ainars Gilis
Zane Abola
Lasma Lidaka
Challenges in the management of caudal duplication syndrome
description Caudal duplication syndrome (CDS) is a rare developmental anomaly in which embryonic cloaca and notochord structures are duplicated [1]. Due to the diverse clinical manifestation and rarity of CDS, it is crucial to report every case and to share experience and outcomes of individually adjusted management plans.We report here the case of a 2-year-old girl born with duplication of the urogenital (bladder, urethra, uterus, vagina, vulva) and gastrointestinal (gallbladder, appendix vermiformis) systems. Additionally, coccygeal agenesis, lipomyelomeningocele and vertical talus were present. A thorough examination and urological reconstructive surgery were performed.While there may be a desire from patients, parents and healthcare specialists to modify all malformations to an anatomically correct state, the current opinion is that only anatomical variants that influence function should be modified. Consent from patients should be sought for decisions regarding more sensitive matters such as vulva surgery for cosmetic reasons or correction of anatomical variants without functional consequences.
format article
author Laine Bekere
Ainars Gilis
Zane Abola
Lasma Lidaka
author_facet Laine Bekere
Ainars Gilis
Zane Abola
Lasma Lidaka
author_sort Laine Bekere
title Challenges in the management of caudal duplication syndrome
title_short Challenges in the management of caudal duplication syndrome
title_full Challenges in the management of caudal duplication syndrome
title_fullStr Challenges in the management of caudal duplication syndrome
title_full_unstemmed Challenges in the management of caudal duplication syndrome
title_sort challenges in the management of caudal duplication syndrome
publisher Elsevier
publishDate 2021
url https://doaj.org/article/d644981adadb4e35b919ada13ae6e378
work_keys_str_mv AT lainebekere challengesinthemanagementofcaudalduplicationsyndrome
AT ainarsgilis challengesinthemanagementofcaudalduplicationsyndrome
AT zaneabola challengesinthemanagementofcaudalduplicationsyndrome
AT lasmalidaka challengesinthemanagementofcaudalduplicationsyndrome
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