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Challenges in the management of caudal duplication syndrome

Authors :
Laine Bekere
Ainars Gilis
Zane Abola
Lasma Lidaka
Source :
Journal of Pediatric Surgery Case Reports, Vol 75, Iss , Pp 102083- (2021)
Publication Year :
2021
Publisher :
Elsevier, 2021.

Abstract

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.

Details

Language :
English
ISSN :
22135766
Volume :
75
Issue :
102083-
Database :
Directory of Open Access Journals
Journal :
Journal of Pediatric Surgery Case Reports
Publication Type :
Academic Journal
Accession number :
edsdoj.644981adadb4e35b919ada13ae6e378
Document Type :
article
Full Text :
https://doi.org/10.1016/j.epsc.2021.102083