Management of the High Urogenital Sinus—Risk of Overexposure?

Management of the High Urogenital Sinus—Risk of Overexposure? THE confluence of the female urethra with the vagina (urogenital [UG] sinus) into a single channel that opens in the vulva or perineum may result from the lack of reabsorption of the distal UG sinus (persistent UG sinus), from alterations in septation (persistent cloaca), or from the influence of endogenous or exogenous androgenic substances.1 The distinction between the first 2 causes and the latter is crucial because it has a profound influence on the choice of surgical intervention needed to correct the anomaly. When the persistent UG sinus results from virilization, the distal part of the UG sinus persists, and the otherwise normal female urethra elongates and may indeed reach the glans clitoris. In these cases, representing the majority of girls with persistent UG sinus, simple caudal mobilization of the sinus and the vagina and urethra en bloc (total urogenital mobilization [TUM]) is effective, and does not affect urinary continence.2–4 In 1969 Hendren and Crawford advanced the concept that in some children with congenital adrenal hyperplasia (CAH) the confluence of the urethra and vagina can be located above what they called the external sphincter.5 They suggested that performing a cut back of the sinus might result in incontinence, but this idea was based on endoscopic rather than anatomical observations. The anatomical validation for this concept was lacking. The observation that the vagina enters the verumontanum was incorrectly interpreted because in XX individuals there is never a verumontanum. What is often observed is a mound resembling the veru at the point of confluence. In practice we have never seen a patient with CAH become incontinent after TUM. The degree of virilization affects the length of the common channel distal to the confluence but not the length of the urethra proximal to it, which is always normal.6 The advantages of TUM compared to techniques based on the separation of the urethra from the vagina are clear to anyone who has performed both procedures. It also makes anatomical sense since the urethral sphincter complex is omegashaped, incomplete posteriorly.7 Separation of the urethra from the vagina, and not en bloc mobilization of the vagina and urethra, incurs the risk of damage to the continence mechanism. 0022-5347/12/1873-0787/0 THE JOURNAL OF UROLOGY® © 2012 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION

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In our experience TUM can always be accomplished perineally with the patient in the dorsal lithotomy position. For the reasons mentioned we have completely abandoned techniques that separate the urethra from the vagina and have not observed any alterations in voiding or continence.1,8 The situation is totally different in cases of UG sinus not associated with virilization. In these cases the urethra may be short and the surgical correction needs to be individualized. In addition, in these cases continence and voiding may be compromised by associated neurological abnormalities.1 The anterior sagittal transrectal approach described by Salle et al in this issue of The Journal (page 1024) is based on the time-honored York-Mason operation for prostatorectal fistulas.9 In children this approach can be useful in reoperations for membranous urethral strictures, high vaginal atresia when the uterus is present, posttraumatic situations and other challenging cases.10 We recommend that pediatric urologists become familiar with this approach. Nevertheless, we consider it not only unnecessary but potentially harmful for the correction of UG sinus associated with virilization. In the report by Pippi Salle et al 1 girl with CAH needed a diverting colostomy because of a perineal infection. We applaud the technical expertise of the authors and are thankful to Dr. de Castro for having popularized this approach for pediatric urology. At the same time we urge caution, critical analysis of the published literature, and understanding of anatomy and function before embarking on new surgical adventures. Surgeons should tailor the magnitude of the operation to the needs of the patient and do no more than is needed to achieve the intended goal. Ricardo González and Barbara Ludwikowski Kinderkrankenhaus auf der Bult Hanover, Germany and Children’s Hospital of the University of Zurich Zurich, Switzerland

Vol. 187, 787-788, March 2012 Printed in U.S.A. DOI:10.1016/j.juro.2011.12.002

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REFERENCES 1. González R and Barthold JS: Urogenital sinus and cloacal anomalies. In: Pediatric Urology Practice. Edited by ET Gonzales and SB Bauer. Philadelphia: Lippincott-Raven 1999; chapt 38, pp 673– 686. 2. Ludwikowski B, Oesch Hayward I and González R: Total urogenital sinus mobilization: expanded applications. BJU Int 1999; 83: 820. 3. Jenak R, Ludwikowski B and González R: Total urogenital sinus mobilization: a modified perineal approach for feminizing genitoplasty and urogenital sinus repair. J Urol 2001; 165: 2347.

4. Kryger JV and González R: Urinary continence is well preserved after total urogenital mobilization. J Urol 2004; 172: 2384. 5. Hendren WH and Crawford JD: Adrenogenital syndrome: the anatomy of the anomaly and its repair. Some new concepts. J Pediatr Surg 1969; 4: 49. 6. Ganesan A, Smith GH, Broome K et al: Congenital adrenal hyperplasia: preliminary observations of the urethra in 9 cases. J Urol 2002; 167: 275. 7. Ludwikowski B, Oesch Hayward I, Brenner E et al: The development of the external urethral sphincter in humans. BJU Int 2001; 87: 565.

8. González R and Fernandes ET: Single-stage feminization genitoplasty. J Urol 1990; 143: 776. 9. Hadley DA, Southwick A and Middleton RG: YorkMason procedure for repair of recto-urinary fistulae: a 40-year experience. BJU Int, Epub ahead of print, October 28, 2011. 10. Mauermann J, González R, Franc-Guimond J et al: The anterior sagittal transrectal approach for traumatic urethrovaginal fistula closure. J Urol 2004; 171: 1650.