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This article has been cited by other articles in PMC. Abstract Urethral duplication UD in females is a rare congenital anomaly and requires a high degree of clinical suspicion for diagnosis.
The preoperative evaluation requires thorough investigations to delineate anatomy which is imperative for surgical reconstruction to provide excellent functional and cosmetic outcome. We describe the successful management of a 6-year-old girl with UD presented as ambiguous genitalia and urinary incontinence along with a review of pertinent literature.
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The UD anatomy is different in males and females and hence the surgical management. The male UD divided into three types according to Effman's classification. The child had normal voiding and dribbling of urine from the abnormal perineal orifice vaginal orifice and had abnormal genitalia hypertrophied clitoris.
Physical examination revealed posterior displacement of the vaginal introitus and prominent clitoris with a narrow opening at the tip. Bekanntschaften kehl scrotal or labial structures were present and the perineum appeared flat and fused [ Figure 1 ]. On further evaluation, the child had female karyotype 46XX and sonography revealed normal Mullerian structures with normal kidneys and bladder.
Retrograde urethrogram RGU was attempted through the opening at the clitoris but catheter could not be passed through it.
Subsequently, evaluation under anesthesia, cystogenitoscopy, and contrast study was done; we could identify one tiny opening at the clitoral tip, which was very narrow and only admitting guide wire for 2 cm.
During vaginoscopy, the vagina was roomy with the normal cervix. A urethral opening was present on the anterior wall of the vagina 2 cm proximal to vaginal orifice and it sa black single ladies led into the normal bladder.
Urethrogram and genitogram were done under fluoroscopy and delineated the anatomy, suggestive sa black single ladies UD [ Figure 1Inset]. Subsequently, the patient underwent feminizing genitoplasty with urethral reconstruction.
The dorsal urethra was dissected, the distal 2 cm of the urethra was very narrow and atretic hence excised. The proximal portion of the dorsal urethra was well developed good caliberjoined to the ventral urethra near its confluence into the vagina [ Figure 2a ].
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The ventral urethra was dissected till communication to the vaginal wall and disconnected. The rent in the anterior vaginal wall and ventral urethra was repaired and soft tissue interposed in between the two suture lines [ Figure 2b ]. The distal continuity of the ventral urethra with the dorsal urethra good caliber was maintained and neourethra i.
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Postoperative period was uneventful and the child was discharged after removal of all the catheters. On subsequent follow-up, the child had developed urethrovaginal fistula of 0.
Six months later, urethrovaginal fistula was repaired successfully from the perineal approach.