Investig Clin Urol.  2016 Sep;57(5):367-371. 10.4111/icu.2016.57.5.367.

An unusual cause of urinary incontinence: Urethral coitus in a case of Mayer-Rokitansky-Kuster-Hauser syndrome

• Sharifiaghdas F
• Daneshpajooh A
• Sohbati S
• Mirzaei M

• KMID: 2427017
• DOI: http://doi.org/10.4111/icu.2016.57.5.367

Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare anomaly in women and is characterized by congenital aplasia of the uterus and vagina, with normal development of secondary sexual characteristics and a normal karyotype. We report a case of a 38-year-old women with MRKH syndrome that had experienced urethral sex for many years. She presented with urinary incontinence and dyspareunia. The patient's secondary sexual characteristics were normal, and examination revealed a widely open incompetent megalourethra and an absent vagina. Laboratory studies confirmed a 46, XX karyotype. Imaging included ultrasonography and magnetic resonance imaging, which indicated bilateral normal ovaries and a rudimental bicornuate uterus. After confirming the diagnosis of MRKH, the patient underwent urethroplasty by urethral plication, antiincontinence surgery by autologous fascial sling of the bladder neck, and the creation of a neo-vagina using a urethral flap. After 3 months, voiding cystourethrography and uroflowmetry confirmed normal voiding. There were no postoperative symptoms of urinary incontinence, and the patient was completely satisfied.

Keyword

Plastic surgery; Urethra; Urinary incontinence

MeSH Terms

46, XX Disorders of Sex Development/*complications/surgery
Adult
*Coitus
Congenital Abnormalities/surgery
Female
Humans
Mullerian Ducts/*abnormalities/surgery
Suburethral Slings
Surgically-Created Structures
Syndrome
Urethra/abnormalities/surgery
Urinary Incontinence/*etiology/surgery

Figure

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  Fig. 1 Megalourethra in a case of Mayer-Rokitansky-Kuster-Hauser syndrome.

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  Fig. 2 Open bladder neck in cystourethroscopy.

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  Fig. 3 (A) Urethral reconstruction: The superior mucosal layer of ventral lip of the urethral wall was plicated over the 16-Fr Foley catheter. (B) Inferior layer was prepared for vaginoplasty.

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  Fig. 4 The rectus fascia graft was prepared for pubovaginal sling. (B) The sling was positioned at the level of bladder neck.

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  Fig. 5 (A) Creation of neo-vagina in the rectovaginal space that was lined by urethral wall flap. (B) Final result of urethroplasty and vaginoplasty.

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