Total urogenital sinus mobilization for ambiguous genitalia

    Total urogenital sinus mobilization for ambiguous genitalia Vinicius Menezes Jesus, Francisco Buriti, Rodrigo Lessa, Maria Betˆania Toralles, Luciana Barros Oliveira, Ubirajara Barroso Jr. PII: DOI: Reference:

S0022-3468(17)30505-5 doi: 10.1016/j.jpedsurg.2017.08.014 YJPSU 58250

To appear in:

Journal of Pediatric Surgery

Received date: Revised date: Accepted date:

20 February 2017 11 August 2017 11 August 2017

Please cite this article as: Jesus Vinicius Menezes, Buriti Francisco, Lessa Rodrigo, Toralles Maria Betˆania, Oliveira Luciana Barros, Barroso Jr. Ubirajara, Total urogenital sinus mobilization for ambiguous genitalia, Journal of Pediatric Surgery (2017), doi: 10.1016/j.jpedsurg.2017.08.014

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ACCEPTED MANUSCRIPT 1 Total urogenital sinus mobilization for ambiguous genitalia

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Vinicius Menezes Jesus¹ Francisco Buriti¹, Rodrigo Lessa¹, Maria Betânia Toralles¹, Luciana

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Barros Oliveira¹ Ubirajara Barroso Junior¹

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¹Ambulatory of Disorders of Sexual Development, Division of Urology and Genetics, Federal

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University of Bahia

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Corresponding author: Ubirajara Barroso Junior ([email protected])

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Av. Alphaville 1 Pajuçara Street Lot J01-08

41701015

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Alphaville 1

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Salvador, Bahia, Brazil.

ACCEPTED MANUSCRIPT 2 Abstract Introduction: Genital ambiguity is a very common phenomenon in disorders of sex development (DSD). According to the Chicago Consensus 2006, feminizing genitoplasty, when indicated,

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should be performed in the most virilized cases (Prader III to V). Advances in the knowledge of

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genital anatomy in DSD have enabled the development and improvement of various surgical techniques. Mobilization of the urogenital sinus (MUS), first described by Peña, has became

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incorporated by most surgeons. However, the proximity of the urethral sphincter prompts

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concern over urinary incontinence, especially for full mobilization of the urogenital sinus.

Objective: To retrospectively evaluate the short-term surgical results of feminizing genitoplasty

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with total mobilization of the urogenital sinus in patients with DSD.

Methods: Review of medical records of all patients undergoing feminizing genitoplasty with

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mobilization of the urogenital sinus. We evaluated the rates of complications from surgery and of urinary incontinence, as well as cosmetic results, according to the opinion of the surgeon and the

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family.

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Results: A total of 8 patients were included in the study. The mean age at surgery was 51 months. Congenital Adrenal Hyperplasia (CAH) was diagnosed in six patients, and gonadal

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dysgenesis in the other two. The vagina was separated from the urethra, with suitable distance in all cases. No patient had urinary incontinence after surgery. The mean follow-up of patients was 20 months (3-56 months). In all cases, surgeons recorded being satisfied with the aesthetic result of post-surgical genitalia. The family was recorded as satisfied with the aesthetic result of the genitalia after surgery. In every case, there was no need for a second surgical procedure.

Conclusion: The total mobilization of the urogenital sinus is a feasible and safe technique. The technique permits good cosmetic results, and urinary incontinence is absent.

Keywords genitoplasty; urogenital sinus; disorders of sex development; ambiguous genitalia; intersex; congenital adrenal hyperplasia

ACCEPTED MANUSCRIPT 3 Introduction

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Genital ambiguity is a very common phenomenon in disorders of sex development (DSD).

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Exposure to androgen excess such as high testosterone and dihydrotestosterone concentrations causes virilization of the external genitalia in degrees that range from mild clitoromegaly without

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fusion of the labioscrotal folds (Prader I) to complete masculinization of the external genitalia

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(Prader V) [1-3]. According to the 2006 Chicago Consensus Statement, feminizing genitoplasty (clitoroplasty, labiaplasty and vaginoplasty), when indicated, should be considered only in cases

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of severe virilization (Prader III-V) and should be performed in specialized centers, with the

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focus being on future sexual function, not just cosmetic appearance [3].

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Improved accuracy in the assessment of genital anatomy in patients with DSD has led to the development of new surgical techniques and the refinement of previous ones [4-15]. Urogenital

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sinus mobilization, first described by Peña et al. and subsequently used by Ludwikowski in cases

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of genital ambiguity, has been widely adopted, since this technique allows the surgery to be performed as a one-stage procedure and permits local flaps from the urogenital sinus to be used to expand the vagina or reconstruct the vaginal vestibule [10,11]. Notwithstanding, the proximity to the urethral sphincter prompts concern regarding the possible development of urinary incontinence, particularly in cases of total urogenital sinus mobilization [14-22]. Few papers have been published on this subject, and they fail to clarify whether the procedure performed indeed consisted of partial or total urogenital sinus mobilization.

ACCEPTED MANUSCRIPT 4 In the case of total urogenital sinus mobilization, the puboprostatic ligament must be incised and the membranous urethra dissected; therefore, concern with respect to the preservation of urinary

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continence increases [12]. Although, by definition, total urogenital sinus mobilization does

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involve sectioning the pubourethral ligament, this has not been systematically reported in the literature. Furthermore, due to the risk of incontinence, some authors who reported having

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the urethra was short; i.e. in the most difficult cases.

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performed urogenital sinus mobilization avoided performing this procedure in patients in whom

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The objective of the present study was to describe a series of patients with ambiguous genitalia submitted to vaginoplasty by urogenital sinus mobilization including sectioning of the

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pubourethral ligament and dissection of the urethra up to the bladder neck.

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1. Material and Methods

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This is a retrospective study of consecutive patients submitted to feminizing genitoplasty with total urogenital sinus mobilization, followed up regularly at the interdisciplinary DSD outpatient clinic at this institute. Patients with DSD of any etiology, any karyotype and a minimum followup period of 6 months were included in the study. Exclusion criteria consisted of loss to followup or incomplete medical records. The institution’s internal review board approved the study protocol.

Visits were scheduled for 1, 3 and 6 months after surgery and then every 6 months thereafter for clinical follow-up, hormone measurement and post-operative recovery assessment. The data

ACCEPTED MANUSCRIPT 5 from the patients’ surgical reports and follow-up visits were retrieved from their medical records. All patients were later invited to return to the office to enable the cosmetic outcome of surgery to

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be assessed and to evaluate any symptoms of lower urinary tract dysfunction. Evaluation of the

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cosmetic outcome of surgery took the position and shape of the clitoris and the appearance of the labia minora and majora into consideration. With respect to the functional outcome of the

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surgical procedure, the criteria evaluated were whether the urethra and the vagina were separated

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and suitably sized. If vaginal stenosis occurred because of postsurgical fibrosis, vaginal dilation should be performed. The children’s parents and/or guardians were asked directly about their

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satisfaction with the cosmetic outcome of surgery. Surgical complications such as the number of subsequent surgical procedures and the nature of these procedures, the development of urinary

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fistula, infection, dehiscence, vaginal stenosis and urinary incontinence were also investigated.

The surgical technique has been described in detail in previous studies [8-23]. Surgery was

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performed with the patient in the extreme lithotomy position. A Fortunoff flap was used in all

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cases (Figure 1). Genitoplasty was performed by urogenital sinus mobilization. The pubourethral ligament was sectioned completely in all cases. The decision regarding whether to divide the pubourethral ligament totally or partially was made during surgery. Although the urogenital sinus and the distance between the bladder neck and the urethrovaginal confluence were measured, it is the capacity of mobilization of the vagina during surgery that will define the extent of the en bloc mobilization of the urogenital sinus. Dissection of the urethra extended beneath the pubis up to the bladder neck (Figure 2). Excess urogenital sinus was used to rebuild the vaginal vestibule (Figure 3). The vagina was externalized to the skin and sutured with the

ACCEPTED MANUSCRIPT 6 Fortunoff flap. Finally, the labia minora and majora were reconstructed (Figure 4). Long-term

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results are shown in Figure 5.

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In no case was it necessary to use the sinus to reconstruct the upper vaginal wall, as described by Passerini-Glazel [13]. Overall, in the patients with DSD treated at this institute, irrespective of

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the size of the urogenital sinus or urethra, partial or total urogenital sinus mobilization is

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performed using the perineal approach, with the patient in the lithotomy position. The anterior

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sagittal trans-anorectal approach (ASTRA) was not used in any of these patients.

Urinary continence was evaluated by asking the patients and/or their parents/guardians whether

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there had been any unintentional day or night urine loss and if they had experienced urgency to

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2. Results

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urinate over the preceding month.

Eight consecutive patients who had been submitted to feminizing genitoplasty by total urogenital sinus mobilization over the 6-year period from 2008 to 2014 were included in the study. Three other patients were excluded due to loss to follow-up. Six patients were diagnosed with congenital adrenal hyperplasia (CAH) and two had mixed gonadal dysgenesis. Of these patients, seven had 46,XX karyotypes and one had the 46,XY karyotype. The mean age of the patients at surgery was 51 ± 51.5 months (± standard deviation [SD]). Measurements of the phallus (as shown in Table 1) and the urogenital sinus were performed immediately prior to surgery. In all the patients, the distance between the skin and the urethrovaginal confluence was > 2.5 cm.

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The procedure was successfully completed in every case, with no need to perform pull-through

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vaginoplasty. The vagina was separated from the urethra in all cases and there was no need for

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vaginal dilation in any patient. None of the patients developed urinary incontinence following surgery. Immediately after surgery, a small dressing was placed on the genitalia. Pain was

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controlled with analgesics and most patients were discharged from hospital on the second day

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after surgery. Mean duration of follow-up was 20 months (range 6 to 56 months). With respect to the cosmetic outcome of surgery, the patients’ medical records showed that the surgeons and the

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families had both reported being satisfied with the appearance of the genitalia following surgery in all cases (Table 1). The vagina was separated from the urethra by a suitable distance in every

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case. A second surgical procedure was not required in any cases, since there was no incidence of

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urinary fistula, vaginal stenosis, urinary incontinence or early suture dehiscence. One patient had dehiscence of the surgical wound on the labia resulting from an infection of the surgical wound.

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That patient progressed well, and the cosmetic outcome was satisfactory after the wound had

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healed. All the patients remained continent and there were no reports of urgency or lower urinary tract symptoms.

3. Discussion

This paper reports on the results of feminizing genitoplasty performed using total urogenital sinus mobilization. The findings show that this technique, when indicated, is feasible and effective in achieving the objectives of this type of surgery; i.e. adequate separation between the

ACCEPTED MANUSCRIPT 8 vagina and urethra to achieve high urethrovaginal confluence, a well-positioned clitoris and a

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satisfactory external appearance of the vagina.

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Peña described the technique of urogenital sinus mobilization in patients with cloaca, using the posterior sagittal route [10]. The cosmetic outcome with this technique was superior to that

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achieved with previous techniques. Subsequently, Ludwikowski et al. used urogenital sinus

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mobilization for the treatment of genital ambiguity in two children with CAH [11]. There was concern regarding the possibility of urinary incontinence after this more "aggressive" dissection

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of the sinus. In response to this concern, Rink et al. performed urogenital sinus mobilization, without, however, severing the pubourethral ligament. That technique was referred to as partial

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urogenital sinus mobilization [12]. Fifteen patients were operated on using that variant of the

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technique. No intra or post-operative complications developed and the cosmetic outcome was satisfactory. No cases of urinary incontinence were recorded. Unlike Ludwikowski et al. who

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performed the entire surgery via the perineal route, Rink et al. operated by the posterior sagittal

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route without sectioning the rectum and, later, by the perineal route [12].

With the aim of preventing the theoretical risk of urinary incontinence when mobilizing a long urogenital sinus and maximizing exposure to improve mobilization of the vagina, Salle et al. developed ASTRA for the surgical management of the high urogenital sinus [15]. Those authors evaluated 16 patients with a high urogenital sinus secondary to CAH who were submitted to ASTRA, and concluded that the technique was excellent, resulting in low complication rates and facilitating separation of the urogenital sinus structures. All the patients remained continent after surgery; however, sepsis developed in one patient, requiring a second surgical procedure.

ACCEPTED MANUSCRIPT 9 Despite the low risk of incontinence and good surgical exposure, ASTRA is a more aggressive procedure, involving opening the rectum. Furthermore, it is more complex because the patient's

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position needs to be changed.

The present findings show that it is possible to separate the vagina from the urethra using the

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perineal route, even when urethrovaginal confluence is high (partial or total urogenital sinus

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mobilization). In our opinion, such an approach is possible with no need to resort to ASTRA, which may be associated with more severe complications. We believe that surgery with posterior

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sagittal access is more suitable for long urogenital sinuses unassociated with genital ambiguity.

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We do perform ASTRA, however, in certain cases of pure urogenital sinus and cloaca variants.

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There is no evidence in the medical literature supporting a risk of urinary incontinence following urogenital sinus mobilization, although a few papers have been published on this technique. As

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shown in Table 2, a considerable number of patients have been submitted to partial or total

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urogenital sinus mobilization without developing urinary incontinence.

The first group of investigators to describe total urogenital sinus mobilization for DSD reported that dissection of the urethra was conducted in an anterior direction up to the upper border of the symphysis pubis; however, no mention was made regarding whether or not the pubourethral ligament was sectioned [17,20]. That same group later updated the series and reported urinary continence, as evaluated by data obtained from the patients’ medical records and from telephone interviews in all toilet-trained patients; however, again there was no mention of whether or not the pubourethral ligament had been disturbed. In the series reported by Gosalbez et al., it is

ACCEPTED MANUSCRIPT 10 unclear how many of the patients were submitted to partial and how many to total urogenital sinus mobilization [18]. Those investigators stated that it is often necessary to divide the

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pubourethral ligaments anteriorly and disrupt the endopelvic fascia to allow caudal displacement

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of the urethra and bladder neck. They probably do not perform total urogenital sinus

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mobilization in patients with a short urethra because of the risk of urinary incontinence.

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Camanni et al. performed a retrospective evaluation of six patients with CAH who had been submitted to total urogenital sinus mobilization. Continence was reported as 100%; however, the

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surgical technique was not described [21]. Palmer et al. described total urogenital sinus mobilization as a dissection that “continues under the pubic bone through the pubourethral

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ligaments and to the level of the bladder neck” [22]. As shown here, not all papers on total

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urogenital sinus mobilization include a description of how the pubourethral ligament was

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sectioned and very few define the level at which sectioning is performed.

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In patients with ambiguous genitalia (Prader III-V), the urethral anatomy is more similar in appearance to a male rather than a female urethra. Between the perineum and the glans, the urethra appears as a hypospadic male urethra in cases of Prader III-IV or as a normal male urethra in cases of Prader V. When the urethra is inserted into the perineum as far as the urethrovaginal confluence, it resembles a bulbar urethra that bends and goes deeply inwards towards the bifurcation of the corpora cavernosa. Therefore, in these patients, the pubourethral ligament resembles that of males. Steiner published a highly detailed description of the male urethral ligament [23]. The pubourethral ligament consists of three segments. The anterior segment links the pendulous urethra to the anterior part of the bone. It consists of fibers from the

ACCEPTED MANUSCRIPT 11 suspensory ligament of the penis, the fascial reflection of the perineal membrane, slips of muscle fascia from the adductor longus muscle, and the terminal fibers of Buck’s fascia. In other words,

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this part of the ligament is anterior to the membranous urethra and is not related to the urethral

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sphincter. Therefore, an incision in this ligament cannot harm the sphincter. The intermediate pubourethral ligament is less obvious and consists of the arcuate and transverse ligaments. It is

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linked to the membranous urethra and distal part of the urethral sphincter. The posterior

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pubourethral ligament is pyramidal in shape and consists of the medial reflection of the transversalis fascia and the entire puboprostatic ligament. This part of the pubourethral ligament

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attaches the external striated urethral sphincter. The only way the urethral sphincter could be

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injured is if the intermediate and the posterior parts are incised.

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However, the papers available in the literature fail to stipulate whether the pubourethral ligament was completely or partially transected. This fact precludes comparison between studies.

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Papers on this type of procedure should necessarily include more complete descriptions with

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respect to the pubourethral anatomy. To the best of our knowledge, this is the first paper to be published in the literature on a series of patients submitted to vaginoplasty in which the complete sectioning of the pubourethral ligament is clearly reported.

In our opinion, there are several advantages with urogenital sinus mobilization compared to other techniques. Whether or not to sever the pubourethral ligament is a mere technicality that will depend on the severity of the problem and will not interfere with the urinary continence rate. The advantages of the technique include: 1) The use of one single route, the perineal route, one with which most urological surgeons are accustomed; 2) The possibility of using the urogenital sinus

ACCEPTED MANUSCRIPT 12 to construct the vaginal vestibule or urethral wall; 3) The simplicity of the technique, with no need to separate the vagina and the urethra/bladder neck in most cases; 4) Not having to open the

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rectum, which would increase the risk of the procedure; and 5) Not having to open the anal

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sphincter, which could theoretically increase the risk of fecal incontinence. In our experience, ASTRA has not been necessary in any case of ambiguous genitalia. 6) We do not believe that

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total urogenital sinus mobilization is better than partial urogenital sinus mobilization. These

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techniques are not mutually exclusive. In this institute, partial urogenital sinus mobilization is performed in milder cases and total urogenital sinus mobilization in the more severe cases with

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longer sinuses and a deeper vagina. The cases described in the present report are the more severe

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cases.

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There are some limitations associated with this study. First, the sample size was small, affecting the accuracy of the results, and overall, the duration of follow-up was short. However, the

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exclusive focus on total urogenital sinus mobilization for patients with a disorder of sex

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development constitutes a strongpoint. Indeed, there are as yet few published studies on the subject matter, while some studies have simultaneously included patients with DSD and patients with cloacal malformation, although there are significant anatomical differences between these two groups. Second, although the vagina is presently separated from the rectum in all cases, patients may develop vaginal stenosis and require further surgery or vaginal dilation in the future. Third, urinary incontinence was evaluated using a structured questionnaire (as shown in Table 3), and surveys on symptoms involve inherent subjectivity. Nevertheless, the question regarding whether a child experiences accidental loss of urine during the day or night is straightforward and simple for patients and/or parents to answer. Finally, another limitation of

ACCEPTED MANUSCRIPT 13 this study involves the measurement of the urogenital sinus. Since this measurement was available for only a few patients, it was decided to remove these data from the analysis; however,

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the distance from the skin to the urethrovaginal confluence was greater than 2.5 cm in all cases

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and the depth of the vagina is reflected in the need for total urogenital sinus mobilization.

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4. Conclusions

Total urogenital sinus mobilization is a feasible and safe technique. There were no cases of

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urinary incontinence, even in cases of longer urogenital sinuses. In our opinion, the perineal

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route could be used even in those cases in which the urethrovaginal confluence is higher.

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Conflicts of interest: None.

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Funding: This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.

ACCEPTED MANUSCRIPT 14 References

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[1] New MI. Inborn errors of adrenal steroidogenesis. Mol Cell Endocrinol 2003;211:75–83.

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[2] Damiani D, Setian N, Kuperman H, Manna TD, Dichtchekenian V. Genitália ambígua: diagnóstico diferencial e conduta. Arq Bras Endocrinol Metab 2001;45:37–47.

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[3] Hughes IA, Houk C, Ahmed SF, Lee PA; Lawson Wilkins Pediatric Endocrine

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Society/European Society for Paediatric Endocrinology Consensus Group. Consensus statement on management of intersex disorders. J Pediatr Urol 2006;2:148–62.

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[4] Lattimer JK. Relocation and recession of the enlarged clitoris with preservation of the glans: an alternative to amputation. J Urol 1961;86:113–6.

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[5] Kogan SJ, Smey P, Levitt SB. Subtunical total reduction clitoroplasty: a safe modification of

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existing techniques. J Urol 1983;130:746–8. [6] Canning DA. Genital sensation after feminizing genitoplasty for congenital adrenal

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hyperplasia: a pilot study. J Urol 2005;173:982–3.

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[7] Minto CL, Liao LM, Woodhouse CR, Ransley PG, Creighton SM. The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study. Lancet 2003;361:1252–7. [8] Pippi Salle JL, Braga LP, Macedo N, Rosito N, Bagli D. Corporeal sparing dismembered clitoroplasty: an alternative technique for feminizing genitoplasty. J Urol 2007;178(4 Pt 2):1796– 800. [9] Baskin LS, Erol A, Li YW, Liu WH, Kurzrock E, Cunha GR. Anatomical studies of the human clitoris. J Urol 1999;162:1015–20.

ACCEPTED MANUSCRIPT 15 [10] Penã A. Total urogenital mobilization – an easier way to repair cloacas. J Pediatr Surg 1997;32:263–7.

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[11] Ludwikowski B, Oesch Hayward I, González R. Total urogenital sinus mobilization:

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expanded applications. BJU Int 1999;83:820–2.

[12] Rink RC, Metcalfe PD, Kaefer MA, Casale AJ, Meldrum KK, Cain MP. Partial urogenital

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mobilization: a limited proximal dissection. J Pediatr Urol 2006;2:351–6.

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[13] Passerini-Glazel G. A new 1-stage procedure for clitorovaginoplasty in severely masculinized female pseudohermaphrodites. J Urol 1989;142:565–8.

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[14] Rink RC. Total urogenital mobilization (TUM). Dial Pediatr Urol 2000;23:2–4. [15] Salle JL, Lorenzo AJ, Jesus LE, Leslie B, AlSaid A, Macedo FN, et al. Surgical treatment of

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high urogenital sinuses using the anterior sagittal transrectal approach: a useful strategy to

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optimize exposure and outcomes. J Urol 2012;187:1024–31. [16] Braga LH, Silva IN, Tatsuo ES. [Total urogenital sinus mobilization in the repair of

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ambiguous genitalia in children with congenital adrenal hyperplasia]. Arq Bras Endocrinol

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Metab 2005;49:908–15.

[17] Kryger JV, González R. Urinary continence is well preserved after total urogenital mobilization. J Urol 2004;172:2384–6. [18] Gosalbez R, Castellan M, Ibrahim E, DiSandro M, Labbie A. New concepts in feminizing genitoplasty – is the Fortunoff flap obsolete? J Urol 2005;174:2350–3. [19] Bailez MM, Cuenca ES, Dibenedetto V. Urinary continence following repair of intermediate and high urogenital sinus in CAH. Experience with 55 cases. Front Pediatr 2014;2:67.

ACCEPTED MANUSCRIPT 16 [20] Jenak R, Ludwikowski B, González R. Total urogenital sinus mobilization: a modified perineal approach for feminizing genitoplasty and urogenital sinus repair. J Urol 2001;165:2347–

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[21] Camanni D, Zaccara A, Capitanucci ML, Mosiello G, Iacobelli BD, De Gennaro M. Bladder after total urogenital mobilization for congenital adrenal hyperplasia and cloaca - does it behave

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the same? J Urol 2009;182:1892–7.

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[22] Palmer BW, Trojan B, Griffin K, Reiner W, Wisniewski A, Frimberger D, et al. Total and partial urogenital mobilization: focus on urinary continence. J Urol 2012;187:1422–6.

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[23] Steiner MS. The puboprostatic ligament and the male urethral suspensory mechanism: an

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anatomic study. Urology 1994;44:530–4.

ACCEPTED MANUSCRIPT 17 Figure captions Figure 1. Initial incision in a patient with congenital adrenal hyperplasia.

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Figure 2. Extensive dissection of the urethra beneath the symphysis pubis. The arrow points to

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the bladder neck in a case in which the corpora cavernosa was preserved.

Figure 3. Urethrovaginal confluence (arrow). As shown, the sinus is opened dorsally as it will

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later constitute the vaginal vestibule.

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Figure 4. Final appearance.

Figure 5. Longer-term results in the same patient. A: Long-term appearance of the external

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genitalia. B: Long-term appearance following gentle traction on the labia majora.

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Table legends

Table 1: Characteristics retrieved from patients’ pre- and post-operative medical records

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Table 2: Studies from the literature on urinary continence in patients with a disorder of sex

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development submitted to total or partial urogenital sinus mobilization Table 3: Urinary continence questionnaire

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Figure 1

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Figure 2

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Figure 3

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Figure 4

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Figure 5A

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Figure 5B

ACCEPTED MANUSCRIPT 24 Table 1: Characteristics retrieved from patients’ pre- and post-operative medical records Opinion of the Age in months at Pre-surgical phallus

Post-surgical family regarding the

in cm

complications

8

4

No

B

14

1.5

No

C

16

1.5

No

D

29

3.5

E

42

4

F

43

3

G

99

H

157

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outcome of surgery

Satisfied Satisfied Satisfied Satisfied

No

Satisfied

Wound dehiscence

Satisfied

2

No

Satisfied

8

No

Satisfied

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No

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A

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time of surgery

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Patient

ACCEPTED MANUSCRIPT 25 Table 2: Studies from the literature on urinary continence in patients with a disorder of sex development submitted to total or partial urogenital sinus mobilization Diagnosis

Number of surgeries

Results

Braga et al. [16]

Congenital adrenal

10 cases of total urogenital sinus

100% urinary

hyperplasia

mobilization

continence

Kryger and

9 cases of congenital

All 13 cases submitted to total urogenital

González [17]

adrenal hyperplasia;

sinus mobilization

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Authors

continence

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2 cases of mixed gonadal

100% urinary

dysgenesis,

dysgenesis,

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1 case of ovotesticular

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1 case of gonadal

disorder of sex development Congenital adrenal

[18] Bailez et al. [19]

[21] Palmer et al. [22]

100% urinary

hyperplasia

sinus mobilization

continence

Congenital adrenal

55 patients submitted to total urogenital

100% urinary

hyperplasia

sinus mobilization

continence

Congenital adrenal

6 patients submitted to total urogenital

100% urinary

hyperplasia

sinus mobilization

continence

14 cases of congenital

10 patients submitted to total urogenital

100% urinary

adrenal hyperplasia

sinus mobilization;

continence

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Camanni et al.

7 patients submitted to total urogenital

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Gosalbez et al.

4 patients submitted to partial urogenital sinus mobilization

ACCEPTED MANUSCRIPT 26 Table 3: Urinary continence questionnaire Items

Yes

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Do you have to rush to the bathroom to avoid wetting your clothes?

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Do you have to strain to urinate?

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Do you wet the bed?

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Do you ever wet your clothes during the day?

No