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Case Reports
. 2022 Sep-Oct;27(5):610-612.
doi: 10.4103/jiaps.jiaps_150_21. Epub 2022 Sep 9.

Persistent Urogenital Sinus with Ambiguous Genitalia and Urethral Duplication in a Pubertal Female

Sunil Kumar  1 , Rudra Prasad Ghorai  1 , Shivcharan Navriya  1 , Preeti Usha  2 , Satish Kumar Ranjan  1
Affiliations
Case Reports

Persistent Urogenital Sinus with Ambiguous Genitalia and Urethral Duplication in a Pubertal Female

Sunil Kumar et al. J Indian Assoc Pediatr Surg. 2022 Sep-Oct.

Abstract

Urogenital sinus (UGS) and cloacal malformations are the spectra of disease affecting mainly females, resulting in an unusual confluence of the genital and urinary tract with or without the involvement of the gastrointestinal tract. Successful reconstruction of these anomalies depends on the accurate preoperative delineation of abnormal anatomy with the help of cross-sectional and other contrast studies like genitourogram along with cystourethroscopy wherever indicated. We hereby report a case of a 14-year-old female who presented with irregular cyclical hematuria and was diagnosed with persistent UGS with urethral duplication. After a thorough evaluation, the patient was successfully managed with surgical reconstruction, described in this study. Persistent UGS is a complex developmental anomaly. Complete characterization of anomaly requires a thorough evaluation such as hormonal assessment, endoscopy, cross-sectional, and radiological contrast study. Surgical reconstruction needs individualization and may need clitoroplasty, labioplasty, and urethral and vaginal mobilization. Morphological and functional outcome is satisfactory in a well-planned surgical reconstruction.

Keywords: Ambiguous genitalia; urethral duplication; urogenital sinus.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
A 14-year-old female presented with irregular cyclical hematuria, (a and b) The appearance of the external genitalia, a note was made of significant clitoromegaly, (c and d) Magnetic resonance imaging pelvis showing clitoromegaly (arrow), (e) Postvoid film of intravenous urogram showing pooling of contrast in the vagina (arrow), (f) Genitourogram showing contrast in the vagina as bladder capping (arrow)
Figure 2
Figure 2
Surgical steps of urogenital sinus reconstruction, (a) Schematic diagram urogenital sinus low confluence vaginal type with ambiguous genitalia and inverted “y” type of urethra, (b and c) Clitoroplasty and labioplasty, (d) The final appearance of external genitalia
See this image and copyright information in PMC

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