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Review
. 2013 Nov 21:1:41.
doi: 10.3389/fped.2013.00041.

The Surgical Correction of Urogenital Sinus in Patients with DSD: 15 Years after Description of Total Urogenital Mobilization in Children

Barbara M Ludwikowski  1 , Ricardo González  2
Affiliations
Review

The Surgical Correction of Urogenital Sinus in Patients with DSD: 15 Years after Description of Total Urogenital Mobilization in Children

Barbara M Ludwikowski et al. Front Pediatr. .

Abstract

Total urogenital sinus mobilization has been applied to the surgical correction of virilized females and has mostly replaced older techniques. Concerns have been raised about the effect of this operation on urinary continence. Here we review the literature on this topic since the description of the technique 15 years ago. Technical aspects and correct nomenclature are discussed. We emphasize that the term "total" refers to an en-bloc dissection and not to the extent of the proximal dissection. No cases of urinary incontinence have been reported following this operation. It is yet too early to evaluate results regarding sexual function but it is likely that the use of a posterior skin flap to augment the introitus will minimize the development of introital stenosis.

Keywords: cloaca; congenital adrenal hyperplasia; disorders of sex development; feminizing genitoplasty; urogenital sinus.

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Figures

Figure 1
Figure 1
Contrast study in patient with typical anatomy in a Prader 3–5 CAH. B, bladder; U, urethra; V, vagina. The blue arrow indicates the distance between the confluence and the perineal skin. The red arrow indicates the length of the UGS which is not relevant to the difficulty of the mobilization. The excess tissue can be used to create a mucosa lining of the vestibule during vulvoplasty. (6). Because of this, we no longer restrict TUM to UGS less than 3 cm in length.
Figure 2
Figure 2
Artist’s representation of the anatomy and planes of dissection. B, bladder; U, urethra; V, vagina; P, pubis; RVF, rectovaginal fascia. Anteriorly the dissection should be close to the pubis, posteriorly close to the vagina, and laterally a few millimeters away from the vaginal wall.
Figure 3
Figure 3
Appearance of the vulva 6 years after TUM with posterior Ω flap. The patient was virilized (Prader 4) because of maternal exposure to androgens.
See this image and copyright information in PMC

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