Expanded classification system for urethral duplication in the female: A literature review and case report
Caleb Q Ashbrook1, Craig A Peters2
1Division of Pediatric Urology, Vanderbilt Children's Hospital, Nashville, TN, USA; Department of Urology, UT Southwestern Medical Center, Dallas, TX, USA.
Introduction:
Urethral duplication in females is a rare entity with variable presentation and associated abnormalities. Several classification systems have been proposed; however, there exists a need to further delineate female urethral duplication.
Objective:
We sought to propose a new simplified classification system for isolated female urethral duplication based on bladder neck anatomy and meatal configuration, focusing on clinical and surgical outcomes.
Study Design:
A literature review of female urethral duplication up to August 2025 in the PubMed and OVID Databases was performed. We identified 39 articles with 44 patients; clinical characteristics and surgical outcomes were collected. One patient from our institution was included.
Results:
Anatomic classification was based on bladder neck anatomy: separate bladder neck (A), common bladder neck (B) or lambda bladder neck (λ; single bladder neck, duplication extends from the common channel). The distal anatomy was defined by location of the urethral openings - anterior-orthotopic (1), anterior-posterior (2), orthotopic-posterior (3) and orthotopic-orthotopic (4). Incomplete duplicated urethra was identified with the subscript (i). All A1 and A2 reported urinary incontinence and the A bladder neck configuration subset of patients reported UTI in 36% versus 17% with B and 17% with λ. Despite similar bladder neck configuration, A2 (38%) configuration had more genital physical exam abnormalities and anomalies compared to A1 (0%). Surgical intervention was variable across groups - the preponderance of secondary and tertiary operations for type 2 distal anatomy may suggest increased surgical complexity to achieve "normal" voiding outcomes - 4/8 patients with A2 underwent a second surgery and third surgery, 1/4 with B2 and 4/7 with λ2.
Discussion:
Our classification system, based on the bladder neck and distal anatomy, is defined after typical work-up. Despite small populations, there are meaningful differences driven both by the origin of the urethra and meatal location. The system reflects this by separating groups by plane, bladder neck configuration, distal meatal location, and includes the λ configuration.
Conclusion:
Urethral duplication in females is a rare entity, and management decisions must be individualized. To the best of our knowledge, we have compiled the largest collection of reported female urethral duplication with descriptions of clinical, surgical and associated abnormalities. Through this data, we propose a classification system based on bladder neck and distal anatomy, focused on meaningful clinical differences between groups that may help guide counseling and management decisions.
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