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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2021
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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) |
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Caudal duplication syndrome Double vulva Double urinary bladder Reconstructive surgery Pediatrics RJ1-570 Surgery RD1-811 |
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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 |
_version_ |
1718416004253483008 |