Persistent urogenital sinus with ambiguous genitalia and urethral duplication in a pubertal female

• Main Authors: Sunil Kumar (Author), Rudra Prasad Ghorai (Author), Shivcharan Navriya (Author), Preeti Usha (Author), Satish Kumar Ranjan (Author)
• Format: Book
• Published: Wolters Kluwer Medknow Publications, 2022-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 0971-9261; 1998-3891; 10.4103/jiaps.jiaps_150_21
• Keywords: ambiguous genitalia; urethral duplication; urogenital sinus; Pediatrics; RJ1-570; Surgery; RD1-811
• Source: Journal of Indian Association of Pediatric Surgeons, Vol 27, Iss 5, Pp 610-612 (2022)

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.