Urogenital sinus malformation: From development to management

Yu Ding; Yaping Wang; Yiqing Lyu; Hua Xie; Yichen Huang; Min Wu; Fang Chen; Zhongzhong Chen

doi:10.5582/irdr.2023.01027

This article has now been updated. Please use the final version.

Urogenital sinus malformation: From development to management

Yu Ding, Yaping Wang, Yiqing Lyu, Hua Xie, Yichen Huang, Min Wu, Fang Chen, Zhongzhong Chen

Author information

Yu Ding
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Yaping Wang
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Yiqing Lyu
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clinical Research Center For Hypospadias Pediatric College, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Hua Xie
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clinical Research Center For Hypospadias Pediatric College, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Yichen Huang
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clinical Research Center For Hypospadias Pediatric College, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Min Wu
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clinical Research Center For Hypospadias Pediatric College, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Fang Chen
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clinical Research Center For Hypospadias Pediatric College, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Zhongzhong Chen
Department of Urology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clinical Research Center For Hypospadias Pediatric College, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Urogenital Development Research Center, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

Keywords: UGS, PUGS, urogenital sinus malformation, embryology, management

JOURNAL FREE ACCESS Advance online publication

Article ID: 2023.01027

DOI https://doi.org/10.5582/irdr.2023.01027

The final version of this article is now available: Vol. 12 (2023), No. 2 pp. 78-87

Details

Abstract

Urogenital sinus (UGS) malformation, also known as persistent urogenital sinus (PUGS), is a rare congenital malformation of the urogenital system. It arises when the urethra and vaginal opening fail to form properly in the vulva and fuse incorrectly. PUGS can occur as an isolated abnormality or as part of a complex syndrome, and is frequently associated with congenital adrenal hyperplasia (CAH). The management of PUGS is not well-established, and there are no standardized guidelines on when to perform surgery or how to follow up with patients over the long term. In this review, we discuss the embryonic development, clinical evaluation, diagnosis, and management of PUGS. We also review case reports and research findings to explore best practices for surgery and follow-up care, in hopes of increasing awareness of PUGS and improving patient outcomes.

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