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Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?
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Journal of Pediatric Urology (2014) xx, 1e7
Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?* Halil Tugtepe a,*, David Terence Thomas b, Serap Turan c, Filiz Cizmecioglu d, Sukru Hatun d, Abdullah Bereket c, E. Tolga Dagli a a Marmara University, School of Medicine, Department of Pediatric Surgery, Division of Pediatric Urology, Istanbul, Turkey b Marmara University, School of Medicine, Department of Pediatric Surgery, Istanbul, Turkey c Marmara University, School of Medicine, Department of Pediatrics, Division of Pediatric Endocrinology, Istanbul, Turkey d Kocaeli University, School of Medicine, Department of Pediatrics, Division of Pediatric Endocrinology, Kocaeli, Turkey
Received 29 August 2013; accepted 19 February 2014
KEYWORDS Congenital adrenal hyperplasia; Urogenital sinus mobilization; Common channel
Abstract Objective: Partial/total urogenital sinus mobilization (UGSM) is one of the recommended techniques for treatment of female congenital adrenal hyperplasia (CAH). In this study we compared the length of common channel (CC) and type of operation performed in CAH patients. Patients and methods: We retrospectively analyzed data of patients receiving surgery for female CAH. Patients were separated into three groups: group 1 had partial UGSM, group 2 had total UGSM, and group 3 had total UGSM plus the vaginal anterior wall was made from CC. Age at surgery, length of CC, surgical time, follow-up time, and complications were compared. Results: There were a total of 29 patients. For groups 1, 2, and 3, the average age at surgery was 47.2 months, 14.4 months, and 21.3 months, respectively, and the average CC length was 1.25 cm, 3.1 cm, 4.3 cm, respectively. The average time of surgery was 165 min, 193.1 min, 282.5 min, respectively. The average follow-up time was 34.7 months, 36.3 months, 28.3 months, respectively. There were two complications (UGS flap necrosis and opening of sutures) in the third group.
*
Presented at the 24th Congress of ESPU in Genoa. * Corresponding author. E-mail address: [email protected] (H. Tugtepe).
http://dx.doi.org/10.1016/j.jpurol.2014.02.012 1477-5131/ª 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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H. Tugtepe et al. Conclusion: We advise the use of partial UGSM for CC of 0.5e2 cm, total UGSM for CC of 2.5e3.5 cm, and total USM with use of CC as the anterior vaginal wall in CC 4 cm in length. Good cosmetic and functional results are obtained with this approach. ª 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction
Surgical techniques
Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder of cortisol biosynthesis. It leads to phenotypic changes due to excessive endogenous androgen production during fetal development in females. Characteristic findings are an enlarged clitoris, partly fused and rugose labia majora, and a common urogenital sinus in place of a separate urethra and vagina [1]. Examination of the external genitalia reveals the degrees of virilization. Prader stages IeV describe increasing verification from a phenotypic female with mild cliteromegaly (stage I) to a phenotypic male with glandular hypospadias or normal penis appearance (stage V) [2]. Cliteroplasty, vaginoplasty, and labioplasty are the components of feminizing genitoplasty surgery of female CAH patients [3,4]. However, the surgical treatment of female CAH patients is still challenging and at times controversial. After Pena [5] described total urogenital sinus mobilization (UGSM) in patients with cloaca in 1997, partial/total UGSM became one of the recommended techniques in CAH. The urogenital sinus and vaginal confluence are described as being “high” or “low” according to the location when compared to the external sphincter, and vaginal “high insertion” is accepted as an urethral length of 1.5e2 cm [6]. Despite no objective criteria accepted as standard in the literature, these cystoscopic findings are important to determine the type of surgery that will be performed. The aim of this study was to compare the length of common channel (CC) and the type of UGSM operation that was performed in female CAH patients.
Measuring the common channel
Patients and methods The clinical and operative files of 29 patients who underwent surgery for female CAH due to 21-hydroxylase deficiency between October 2008 and May 2013 at our institute were retrospectively analyzed. CC lengths of all patients were measured by cystoscopy prior to major genital surgery. Patients that were diagnosed with CAH during the newborn period had cystoscopy at 6e7 months and total/partial UGSM around 1 year of age, whereas patients with late diagnosis (over 1 year) had cystoscopy and major genital surgery soon after diagnosis. When retrospectively analyzed, the patients were found to have had three surgical methods performed (PUSM, TUSM, or TUSM plus CC as the vaginal wall) and were therefore divided into three groups for comparison of CC length. The age of patients was recorded and surgery times were analyzed. The short-term results and complications were also recorded.
Before cystoscopic examination or UGSM, all patients received medication for stress coverage. In the lithotomy position, cystoscopy is performed. The CC is examined and the location of the vaginal confluence noted. To measure the CC, the cystoscope is advanced into the vagina. A 3F ureter catheter is inserted through the cystoscope. The cystoscope is reintroduced and performed alongside the catheter. The catheter is adjusted so that the first centimeter mark is at the level of the confluence. The length of the CC, the distance from the vaginal confluence to the perineal meatus, is measured as the cystoscope is withdrawn. A Foley catheter is introduced into the bladder. A Fogarty catheter is placed as a guide in the vagina in preparation for UGSM.
Cliteroplasty The operation starts by placing a traction suture in the glans. A subcoronal circumferential incision is then made 0.5 cm proximal to the glans. The shaft skin is degloved and dorsal foreskin is split longitudinally; the flaps obtained are used for construction of the labia minora later. The dorsal neurovascular bundle and the glans are separated from the corporal body as described by Poppas et al. [7]. The CC is transsected from the corporeal bodies by bipolarcauthery. Corporal bodies at 1.5e2 cm distal to the bifurcation are sutured and distal parts of corporeal bodies are resected by cautery. According to the age of the patient and the size of the glans, glans reduction is done either by wedge resection or by de-epithelization. The glans is settled onto the rest of the corporeal bodies by sutures.
Partial UGSM We begin with a midline and U-shaped perineal flap incision. After cliteroplasty described as above, CC dissection is performed circumferentially to the pubic bone. Anteriorly we reach the pubourethral ligament but do not dissect it. Posteriorly the Fogarty catheter balloon is palpated. The CC is opened at the 6 o’clock position to the vaginal confluence. The posterior vaginal wall is opened and the Uflap skin is sutured. The CC mucosa is tailored to the clitoris by sutures (Fig. 1).
Total UGSM In this group, for further mobilization when PUSM is not sufficient, the pubourethral ligament is dissected anteriorly
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?
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Figure 1 Partial urogenital sinus mobilization. (a) Preoperative appearance, (b) partial mobilization of common channel, (c) postoperative appearance.
towards the pubic bone, and further dissection of the vagina from rectum is achieved posteriorly until the Fogarty catheter is felt. After adequate mobilization, the CC is opened at the 6 o’clock position to the vaginal confluence. The posterior vaginal wall is opened and the U-flap skin is sutured to it allowing a wide vaginal opening. CC mucosa is tailored to the clitoris by sutures (Fig. 2).
Total UGSM with common channel used as anterior vaginal wall In this group, the sinus is mobilized as much as possible using the technique described above. When this dissection is deemed to be inadequate, the vagina is dissected from the sinus and the urethral opening is sutured. After the CC is opened at the 12 o’clock position longitudinally, the sinus flap is folded back and sutured to the anterior margin of vagina as the anterior vaginal wall [8e11]. The posterior vaginal wall is opened and the U-flap perineal skin is sutured to it, allowing a wide vaginal opening (Fig. 3).
Labioplasty The phallic skin is brought down forming the labia minora and its ends are sutured to the sides of the vagina. Labia majora flaps as formed from the initial VeY incisions are repositioned posteriorly to form a normal looking vestibule. A transanastamotic small tampon coated with antibiotics is left for 3 days and a Foley catheter is left in the bladder for 7 days. A broad spectrum antibiotic is given until removal of the Foley catheter. All cases are examined cystoscopically under general anesthesia at the third postoperative. During the follow-up time, all patients were evaluated by physical examination and urinary system questioning every 6 months, unless they presented with symptoms.
Results Patients were separated into three groups according to the surgery performed: partial UGSM, total UGSM, and total
USGM where the CC was used as the anterior vaginal wall. Results are shown in Table 1. There were 11 patients in the partial UGSM group. Their average age at surgery was 47.2 months. The average length of the CC was 1.25 cm (range 1e2 cm). The average time of surgery was 165 min. The follow-up time for these patients was 34.7 months. No complications were observed in this group. There were 10 patients in the total UGSM group. Their average age at surgery was 14.4 months. The average length of their CC was 3.1 cm (range 2.5e3.5 cm). The average time of surgery was 193.1 min. The follow-up time for these patients was 36.3 months. No complications were observed in this group. There were eight patients in the total UGSM group where the CC was used as the anterior vaginal wall. Their average age at surgery was 21.3 months. The average length of their CC was 4.3 cm (4e5 cm). The average time of surgery was 282.5 min. The follow up time for these patients was 28.3 months. One patient had UGS flap necrosis and one patient had dehiscence of the U flap due to wound infection. As of the end of this study’s follow-up time, 17 patients over 4 years of age were all continent and had no urological symptoms. Of these, six of seven patients had normal urodynamic evaluation and one had overactive detrusor.
Discussion Reconstructive surgery for patients with CAH has three components: cliteroplasty, vaginoplasty, and labioplasty. CAH can cause virilization of external genitalia in different degrees; therefore, various approaches have been described to solve these problems, especially for a vaginoplasty operation. Unfortunately, there is still a serious lack of data to provide adequate guidance as to the best timing and choice of surgical approach [6,9,12]. Indeed, cliteroplasty timing is better investigated than vaginoplasty in the literature. In the presence of marked clitoromegaly, clitoral reduction is usually undertaken in infancy. Lesser degrees of clitoral enlargement may be left until puberty when the child can be involved with the decision making [6,13].
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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H. Tugtepe et al.
Figure 2 Total urogenital sinus mobilization (a), preoperative appearance (b), degloving of the phallus (c), mobilization of the urogenital sinus (d), opening of the common channel at 6 o’clock (e), Postoperative appearance (f).
The timing of any vaginoplasty is dependent on the anatomy of the internal and external genitalia and influenced by local practice [3]. The urogenital sinus and vaginal confluence are described as being “high” or “low” according to the location when compared to the external sphincter, and vaginal “high insertion” is accepted as a urethral length of 1.5e2 cm while “low take-off” vagina cases are technically easier to address surgically [6]. The most challenging aspect of the surgical treatment of CAH is dealing with a high inserting vagina. For vaginoplasty, the procedures range from a simple flap vaginoplasty to more complex surgical interventions such as partial or total UGSM, vaginal pull through, autologous buccal mucosa vulvovaginoplatsy [5,14,15]. The choice of procedure depends on the anatomical configuration of the vagina and length of urethra. The timing of surgery of intersex patients and CAH are a matter of debate and, recently, recommendations have been made to postpone surgical interventions until the child can decide for his/herself. Cultural reasons are well known to influence the choice of gender role [16], and in Turkey families request that surgery is performed at early ages for cultural reasons as families believe it is better to perform genital surgery before the child gains awareness of “abnormal” appearing genitalia.
After Alberto Pena [5] first described total urogenital sinus mobilization in patients with persistent cloaca in 1997, Rink [17] applied this procedure successfully in patients with urogenital sinus anomalies as total or partial mobilization of sinus. Several authors have reported good cosmetic results with few complications [8,9], although there was no mention of CC lengths or measurements of any structures. It has been suggested that the critical factor in vaginoplasty is not the length of the CC [13]. In one study, the authors suggest cliteroplasty and vaginoplasty in the same session for less severe cases (CC 1 cm) and a two-stage approach for patients with greater virilization or CC 3 cm [12]. Our study shows that the choice of surgical technique is closely related to CC length. All patients were approached perineally and did not have any perioperative or postoperative complications. Partial USGM was adequate for CCs measuring up to 2 cm, whereas for lengths of 2.5e3.5 cm total UGSM was used, and for CC lengths over 4 cm total UGSM with the CC as the anterior vaginal wall was used. In a series by Braga et al. [18] 24 patients with Prader IIIeV were successfully corrected using partial UGSM. However, in these patients the CC length was 3.0 cm, except for one patient with a CC of 3.5 cm. We performed
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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Figure 3 Total urogenital sinus mobilization with common channel (CC) used as the anterior vaginal wall (a), preoperative appearance (b), degloving and separation of the neurovascular bundle and glans (c), urogenital sinus mobilization (d), dissection of the vagina (e), opening of CC at 12 o’clock (f), formation of the posterior vaginal wall (g), formation of the anterior vaginal wall with CC flap (h), postoperative appearance (i).
total UGSM on patients with a CC length 2.5e3.5 cm because we have observed that partial UGSM results in a hypospadiac appearance of the urethra. Therefore, when making surgical decisions, the CC length should play an important role in choosing the surgical technique of female CAH patients. While there are several classification systems for comparing the genital anatomy of our patient group, none is universally accepted. A common criticism of the Prader classification is that there is no correlation between the degree of external virilization and the level of confluence between the vagina and urethra [19]. While we included
Prader classifications in the first analysis of our data, we found no correlation between Prader and the surgery performed; therefore, we have not included these data in our manuscript. Some authors suggest that genital correction surgery should be performed after puberty when high hormone levels could potentially decrease the rate of complications [20,21]. Braga et al. [18] anecdotally reported that in postpubertal patients, the operation is more difficult due to the deepness of the pelvis, limited exposure, and increased tissue vascularity. We have also observed these difficulties in our patients. However, we generally found it difficult to
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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H. Tugtepe et al. Table 1
Characteristics of patients.
No of patients Age at surgery Length of common channel Surgery time Follow-up time Complications
Partial UGSM group
Total UGSM group
Total UGSM group (common channel used as anterior vaginal wall)
11 47.2 months 1.25 cm (1e2 cm)
10 14.4 months 3.1 cm (2.5e3.5 cm)
8 21.3 months 4.3 cm (4e5 cm)
165.0 min 34.7 months None
193.1 min 36.3 months None
282.5 min 28.3 months UGS flap necrosis (1) Opening of sutures (1)
UGSM Z urogenital sinus mobilization.
separate the vagina from the sinus because of a thin vaginal wall. Recently, the use of estrogen cream for such patients has been suggested [6], and we have started to implement this also. Patients undergoing total UGSM or partial UGSM are at risk for developing complications such as urinary incontinence, urethrovaginal fistula, and vaginal or urethral stenosis. It has been suggested that UGSM can have an adverse effect on the bladder neck, as a result of its downward displacement. Hamza et al. [8] stated that the length of the urogenital sinus was longer than that of a normal urethra. Although we did not measure the urethral length at followup, we believe that the postoperative urethra remains longer than normal anatomy. However, Kryger and Gonzalez [22] showed that six of 13 patients that were dry preoperatively remained dry postoperatively. Podesta and Urcullo [23] found that one of 12 had postoperative stress incontinence that was treated with transurethral injection. Seventeen of our patients were aged over 4 years at follow-up and all were found to be continent and without urinary symptoms. The remaining group when questioned were found to have dry periods, suggesting that none of our patients has urinary incontinence. Of patients who had urodynamic evaluation, six of seven had normal urodynamic findings and one had an overactive detrusor. Vaginal stricture is reported as a rare complication. In this series, we observed no cases of vaginal stricture. Our follow-up time was an average of 3 years. Despite this length of time, there are reports in the literature demonstrating that this complication can be seen years after surgery [18,24,25]. In our series, we had two complications. The patient with UGS flap necrosis was the first patient in the third group. At postoperative day 5, the color of the flap changed and the vaginal opening was found to be closed 10 days later. We noticed the necrosis of the CC at cystoscopic examination. The postoperative appearance was no different from the preoperative appearance. A year later we performed the ASTRA (anterior sagittal transrectal approach), and she remains very well today. Our second complication was dehiscence of the labia majora and U-flap sutures due to wound infection. The vaginal opening was closed but the urethra was totally normal at control cystoscopy. She is to undergo a second operation. The literature reports concerns with regard to the longterm effects of total UGSM on the urinary tract and sexual
function, as this region is important, including clitoral innervation, neuromuscular anatomy of the bladder neck, and the intricate network of supporting tissue [9,26]. Theoretically, the more limited dissection of partial UGSM is thought to limit damage to these structures. However, our study has shown that with meticulous dissection and surgical technique, total and partial UGSM both have good cosmetic and functional results. In a similar finding, Palmer et al. [27] found that there was no difference in terms of urinary incontinence between total and partial UGSM. It is important to note however that patients with CAH are more likely to have urinary symptoms than normal controls [28]. It is not clear whether this is a result of surgery or an effect of CAH [29]. Our study is limited by its retrospective design, limited number of patients, and absence of long-term observational follow-up. Also, only one of our patients had reached puberty, so it was no possible to assess sexual function after surgery. The strength of this study is the fact that we examined all patients and performed cystoscopy under general anesthesia, at the third postoperative month.
Conclusions Total and partial UGSM are safe techniques for genital surgery of female CAH patients and results in good cosmetic and functional outcome. Despite less dissection in partial UGSM, the rates of complications are similar to total UGSM. The length of the CC should be used as a guide for the choice between total and partial UGSM. Further long-term studies are required to assess urinary and sexual functions.
Conflict of interest None.
Funding None.
References [1] Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet 2005;365:2125e36.
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients? [2] Von Prader A. Der genitalbefund beim pseudohermaproditus feminus des kongenitalen adrenogenitalen syndromes. Helv Pediatr Acta 1954;9:231e48. [3] Ogilvy-Stuart AL, Brain CE. Early assessment of ambiguous genitalia. Arch Dis Child 2004;89:401e7. [4] Creighton S. Surgery for intersex. J R Soc Med 2001;94:218e20. [5] Pena A. Total urogenital mobilization e an easier way to repair cloacas. J Pediatr Surg 1997;32:263e8. [6] Creighton S, Chernausek SD, Romao R, Ransley P, Salle JP. Timing and nature of reconstructive surgery for disorders of sex development e introduction. J Pediatr Urol 2012;8:602e10. [7] Poppas DP, Hochsztein AA, Baergen RN, Loyd E, Chen J, Felsen D. Nerve sparing ventral clitoroplasty preserves dorsal nerves in congenital adrenal hyperplasia. J Urol 2007;178: 1802e6. discussion 1806. [8] Hamza AF, Soliman HA, Abdel Hay SA, Kabesh AA, Elbehery MM. Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia. J Pediatr Surg 2001;36:1656e8. [9] Rink RC, Metcalfe PD, Kaefer MA, Casale AJ, Meldrum KK, Cain MP. Partial urogenital mobilization: a limited proximal dissection. J Pediatr Urol 2006;2:351e6. [10] Gonza ´lez R, Ludwikowski B. Management of the high urogenital sinuserisk of overexposure? J Urol 2012;187:787e8. [11] Rink R, Kaefer M. Surgical management of intersexuality, cloacal malformation, and other abnormalities of the genitalia in girls. In: Walsh P, Retik A, Vaughn E, Wein A, editors. Campbell’s Urol. 8th ed. Philadelphia: WB Saunders; 2002. pp. 2428e68. [12] Escala Aguirre JM, Cadena Y, Lo ´pez P-J, Angel L, Retamal MG, Letelier N, et al. Feminizing genitoplasty in adrenal congenital hyperplasia: one or two surgical steps? Arch Esp Urol 2009; 62:724e30. [13] Rink RC, Adams MC, Misseri R. A new classification for genital ambiguity and urogenital sinus anomalies. BJU Int 2005;95: 638e42. [14] Samuelson ML, Baker LA. Autologous buccal mucosa vulvovaginoplasty for high urogenital sinus. J Pediatr Urol 2006;2: 486e8. [15] Rink RC, Adams MC. Feminizing genitoplasty: state of the art. World J Urol 1998;16:212e8. [16] Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. J Pediatr Urol 2006;2: 148e62.
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[17] Rink R. Total urogenital mobilization (TUM). Dial Pediatr Urol 2000;23:2e4. [18] Braga LHP, Lorenzo AJ, Tatsuo ES, Silva IN, Pippi Salle JL. Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for congenital adrenal hyperplasia. J Urol 2006;176:2199e204. [19] Malone PS, Hall-Craggs MA, Mouriquand PDE, Caldamone AA. The anatomical assessment of disorders of sex development (DSD). J Pediatr Urol 2012;8:585e91. [20] Creighton SM. Long-term outcome of feminization surgery: the London experience. BJU Int 2004;93(Suppl. 3):44e6. [21] Minto CL, Liao L-M, Woodhouse CRJ, 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;361:1252e7. [22] Kryger JV, Gonza ´lez R. Urinary continence is well preserved after total urogenital mobilization. J Urol 2004;172:2384e6. [23] Podesta M, Urcullo J. Perineal mobilization of the common urogenital sinus for surgical correction of high urethrovaginal confluence in patients with intersex disorders. J Pediatr Urol 2008;4:352e8. [24] Hendren WH, Atala A. Repair of the high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome. J Pediatr Surg 1995;30:91e4. [25] Krege S, Walz KH, Hauffa BP, Ko ¨rner I, Ru ¨bben H. Long-term follow-up of female patients with congenital adrenal hyperplasia from 21-hydroxylase deficiency, with special emphasis on the results of vaginoplasty. BJU Int 2000;86:253e8. discussion 258e9. [26] Carroll PR, Dixon CM. Surgical anatomy of the male and female urethra. Urol Clin N Am 1992;19:339e46. [27] 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;187:1422e6. [28] Hannestad YS, Rortveit G, Sandvik H, Hunskaar S. A community-based epidemiological survey of female urinary incontinence: the Norwegian EPINCONT study. Epidemiology of Incontinence in the County of Nord-Trøndelag. J Clin Epidemiol 2000;53:1150e7. [29] Davies MC, Crouch NS, Woodhouse CRJ, Creighton SM. Congenital adrenal hyperplasia and lower urinary tract symptoms. BJU Int 2005;95:1263e6.
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Journal of Pediatric Urology (2014) xx, 1e7
Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?* Halil Tugtepe a,*, David Terence Thomas b, Serap Turan c, Filiz Cizmecioglu d, Sukru Hatun d, Abdullah Bereket c, E. Tolga Dagli a a Marmara University, School of Medicine, Department of Pediatric Surgery, Division of Pediatric Urology, Istanbul, Turkey b Marmara University, School of Medicine, Department of Pediatric Surgery, Istanbul, Turkey c Marmara University, School of Medicine, Department of Pediatrics, Division of Pediatric Endocrinology, Istanbul, Turkey d Kocaeli University, School of Medicine, Department of Pediatrics, Division of Pediatric Endocrinology, Kocaeli, Turkey
Received 29 August 2013; accepted 19 February 2014
KEYWORDS Congenital adrenal hyperplasia; Urogenital sinus mobilization; Common channel
Abstract Objective: Partial/total urogenital sinus mobilization (UGSM) is one of the recommended techniques for treatment of female congenital adrenal hyperplasia (CAH). In this study we compared the length of common channel (CC) and type of operation performed in CAH patients. Patients and methods: We retrospectively analyzed data of patients receiving surgery for female CAH. Patients were separated into three groups: group 1 had partial UGSM, group 2 had total UGSM, and group 3 had total UGSM plus the vaginal anterior wall was made from CC. Age at surgery, length of CC, surgical time, follow-up time, and complications were compared. Results: There were a total of 29 patients. For groups 1, 2, and 3, the average age at surgery was 47.2 months, 14.4 months, and 21.3 months, respectively, and the average CC length was 1.25 cm, 3.1 cm, 4.3 cm, respectively. The average time of surgery was 165 min, 193.1 min, 282.5 min, respectively. The average follow-up time was 34.7 months, 36.3 months, 28.3 months, respectively. There were two complications (UGS flap necrosis and opening of sutures) in the third group.
*
Presented at the 24th Congress of ESPU in Genoa. * Corresponding author. E-mail address: [email protected] (H. Tugtepe).
http://dx.doi.org/10.1016/j.jpurol.2014.02.012 1477-5131/ª 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Tugtepe H, et al., Does common channel length affect surgical choice in female congenital adrenal hyperplasia patients?, Journal of Pediatric Urology (2014), http://dx.doi.org/10.1016/j.jpurol.2014.02.012
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H. Tugtepe et al. Conclusion: We advise the use of partial UGSM for CC of 0.5e2 cm, total UGSM for CC of 2.5e3.5 cm, and total USM with use of CC as the anterior vaginal wall in CC 4 cm in length. Good cosmetic and functional results are obtained with this approach. ª 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction
Surgical techniques
Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder of cortisol biosynthesis. It leads to phenotypic changes due to excessive endogenous androgen production during fetal development in females. Characteristic findings are an enlarged clitoris, partly fused and rugose labia majora, and a common urogenital sinus in place of a separate urethra and vagina [1]. Examination of the external genitalia reveals the degrees of virilization. Prader stages IeV describe increasing verification from a phenotypic female with mild cliteromegaly (stage I) to a phenotypic male with glandular hypospadias or normal penis appearance (stage V) [2]. Cliteroplasty, vaginoplasty, and labioplasty are the components of feminizing genitoplasty surgery of female CAH patients [3,4]. However, the surgical treatment of female CAH patients is still challenging and at times controversial. After Pena [5] described total urogenital sinus mobilization (UGSM) in patients with cloaca in 1997, partial/total UGSM became one of the recommended techniques in CAH. The urogenital sinus and vaginal confluence are described as being “high” or “low” according to the location when compared to the external sphincter, and vaginal “high insertion” is accepted as an urethral length of 1.5e2 cm [6]. Despite no objective criteria accepted as standard in the literature, these cystoscopic findings are important to determine the type of surgery that will be performed. The aim of this study was to compare the length of common channel (CC) and the type of UGSM operation that was performed in female CAH patients.
Measuring the common channel
Patients and methods The clinical and operative files of 29 patients who underwent surgery for female CAH due to 21-hydroxylase deficiency between October 2008 and May 2013 at our institute were retrospectively analyzed. CC lengths of all patients were measured by cystoscopy prior to major genital surgery. Patients that were diagnosed with CAH during the newborn period had cystoscopy at 6e7 months and total/partial UGSM around 1 year of age, whereas patients with late diagnosis (over 1 year) had cystoscopy and major genital surgery soon after diagnosis. When retrospectively analyzed, the patients were found to have had three surgical methods performed (PUSM, TUSM, or TUSM plus CC as the vaginal wall) and were therefore divided into three groups for comparison of CC length. The age of patients was recorded and surgery times were analyzed. The short-term results and complications were also recorded.
Before cystoscopic examination or UGSM, all patients received medication for stress coverage. In the lithotomy position, cystoscopy is performed. The CC is examined and the location of the vaginal confluence noted. To measure the CC, the cystoscope is advanced into the vagina. A 3F ureter catheter is inserted through the cystoscope. The cystoscope is reintroduced and performed alongside the catheter. The catheter is adjusted so that the first centimeter mark is at the level of the confluence. The length of the CC, the distance from the vaginal confluence to the perineal meatus, is measured as the cystoscope is withdrawn. A Foley catheter is introduced into the bladder. A Fogarty catheter is placed as a guide in the vagina in preparation for UGSM.
Cliteroplasty The operation starts by placing a traction suture in the glans. A subcoronal circumferential incision is then made 0.5 cm proximal to the glans. The shaft skin is degloved and dorsal foreskin is split longitudinally; the flaps obtained are used for construction of the labia minora later. The dorsal neurovascular bundle and the glans are separated from the corporal body as described by Poppas et al. [7]. The CC is transsected from the corporeal bodies by bipolarcauthery. Corporal bodies at 1.5e2 cm distal to the bifurcation are sutured and distal parts of corporeal bodies are resected by cautery. According to the age of the patient and the size of the glans, glans reduction is done either by wedge resection or by de-epithelization. The glans is settled onto the rest of the corporeal bodies by sutures.
Partial UGSM We begin with a midline and U-shaped perineal flap incision. After cliteroplasty described as above, CC dissection is performed circumferentially to the pubic bone. Anteriorly we reach the pubourethral ligament but do not dissect it. Posteriorly the Fogarty catheter balloon is palpated. The CC is opened at the 6 o’clock position to the vaginal confluence. The posterior vaginal wall is opened and the Uflap skin is sutured. The CC mucosa is tailored to the clitoris by sutures (Fig. 1).
Total UGSM In this group, for further mobilization when PUSM is not sufficient, the pubourethral ligament is dissected anteriorly
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Figure 1 Partial urogenital sinus mobilization. (a) Preoperative appearance, (b) partial mobilization of common channel, (c) postoperative appearance.
towards the pubic bone, and further dissection of the vagina from rectum is achieved posteriorly until the Fogarty catheter is felt. After adequate mobilization, the CC is opened at the 6 o’clock position to the vaginal confluence. The posterior vaginal wall is opened and the U-flap skin is sutured to it allowing a wide vaginal opening. CC mucosa is tailored to the clitoris by sutures (Fig. 2).
Total UGSM with common channel used as anterior vaginal wall In this group, the sinus is mobilized as much as possible using the technique described above. When this dissection is deemed to be inadequate, the vagina is dissected from the sinus and the urethral opening is sutured. After the CC is opened at the 12 o’clock position longitudinally, the sinus flap is folded back and sutured to the anterior margin of vagina as the anterior vaginal wall [8e11]. The posterior vaginal wall is opened and the U-flap perineal skin is sutured to it, allowing a wide vaginal opening (Fig. 3).
Labioplasty The phallic skin is brought down forming the labia minora and its ends are sutured to the sides of the vagina. Labia majora flaps as formed from the initial VeY incisions are repositioned posteriorly to form a normal looking vestibule. A transanastamotic small tampon coated with antibiotics is left for 3 days and a Foley catheter is left in the bladder for 7 days. A broad spectrum antibiotic is given until removal of the Foley catheter. All cases are examined cystoscopically under general anesthesia at the third postoperative. During the follow-up time, all patients were evaluated by physical examination and urinary system questioning every 6 months, unless they presented with symptoms.
Results Patients were separated into three groups according to the surgery performed: partial UGSM, total UGSM, and total
USGM where the CC was used as the anterior vaginal wall. Results are shown in Table 1. There were 11 patients in the partial UGSM group. Their average age at surgery was 47.2 months. The average length of the CC was 1.25 cm (range 1e2 cm). The average time of surgery was 165 min. The follow-up time for these patients was 34.7 months. No complications were observed in this group. There were 10 patients in the total UGSM group. Their average age at surgery was 14.4 months. The average length of their CC was 3.1 cm (range 2.5e3.5 cm). The average time of surgery was 193.1 min. The follow-up time for these patients was 36.3 months. No complications were observed in this group. There were eight patients in the total UGSM group where the CC was used as the anterior vaginal wall. Their average age at surgery was 21.3 months. The average length of their CC was 4.3 cm (4e5 cm). The average time of surgery was 282.5 min. The follow up time for these patients was 28.3 months. One patient had UGS flap necrosis and one patient had dehiscence of the U flap due to wound infection. As of the end of this study’s follow-up time, 17 patients over 4 years of age were all continent and had no urological symptoms. Of these, six of seven patients had normal urodynamic evaluation and one had overactive detrusor.
Discussion Reconstructive surgery for patients with CAH has three components: cliteroplasty, vaginoplasty, and labioplasty. CAH can cause virilization of external genitalia in different degrees; therefore, various approaches have been described to solve these problems, especially for a vaginoplasty operation. Unfortunately, there is still a serious lack of data to provide adequate guidance as to the best timing and choice of surgical approach [6,9,12]. Indeed, cliteroplasty timing is better investigated than vaginoplasty in the literature. In the presence of marked clitoromegaly, clitoral reduction is usually undertaken in infancy. Lesser degrees of clitoral enlargement may be left until puberty when the child can be involved with the decision making [6,13].
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H. Tugtepe et al.
Figure 2 Total urogenital sinus mobilization (a), preoperative appearance (b), degloving of the phallus (c), mobilization of the urogenital sinus (d), opening of the common channel at 6 o’clock (e), Postoperative appearance (f).
The timing of any vaginoplasty is dependent on the anatomy of the internal and external genitalia and influenced by local practice [3]. The urogenital sinus and vaginal confluence are described as being “high” or “low” according to the location when compared to the external sphincter, and vaginal “high insertion” is accepted as a urethral length of 1.5e2 cm while “low take-off” vagina cases are technically easier to address surgically [6]. The most challenging aspect of the surgical treatment of CAH is dealing with a high inserting vagina. For vaginoplasty, the procedures range from a simple flap vaginoplasty to more complex surgical interventions such as partial or total UGSM, vaginal pull through, autologous buccal mucosa vulvovaginoplatsy [5,14,15]. The choice of procedure depends on the anatomical configuration of the vagina and length of urethra. The timing of surgery of intersex patients and CAH are a matter of debate and, recently, recommendations have been made to postpone surgical interventions until the child can decide for his/herself. Cultural reasons are well known to influence the choice of gender role [16], and in Turkey families request that surgery is performed at early ages for cultural reasons as families believe it is better to perform genital surgery before the child gains awareness of “abnormal” appearing genitalia.
After Alberto Pena [5] first described total urogenital sinus mobilization in patients with persistent cloaca in 1997, Rink [17] applied this procedure successfully in patients with urogenital sinus anomalies as total or partial mobilization of sinus. Several authors have reported good cosmetic results with few complications [8,9], although there was no mention of CC lengths or measurements of any structures. It has been suggested that the critical factor in vaginoplasty is not the length of the CC [13]. In one study, the authors suggest cliteroplasty and vaginoplasty in the same session for less severe cases (CC 1 cm) and a two-stage approach for patients with greater virilization or CC 3 cm [12]. Our study shows that the choice of surgical technique is closely related to CC length. All patients were approached perineally and did not have any perioperative or postoperative complications. Partial USGM was adequate for CCs measuring up to 2 cm, whereas for lengths of 2.5e3.5 cm total UGSM was used, and for CC lengths over 4 cm total UGSM with the CC as the anterior vaginal wall was used. In a series by Braga et al. [18] 24 patients with Prader IIIeV were successfully corrected using partial UGSM. However, in these patients the CC length was 3.0 cm, except for one patient with a CC of 3.5 cm. We performed
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Figure 3 Total urogenital sinus mobilization with common channel (CC) used as the anterior vaginal wall (a), preoperative appearance (b), degloving and separation of the neurovascular bundle and glans (c), urogenital sinus mobilization (d), dissection of the vagina (e), opening of CC at 12 o’clock (f), formation of the posterior vaginal wall (g), formation of the anterior vaginal wall with CC flap (h), postoperative appearance (i).
total UGSM on patients with a CC length 2.5e3.5 cm because we have observed that partial UGSM results in a hypospadiac appearance of the urethra. Therefore, when making surgical decisions, the CC length should play an important role in choosing the surgical technique of female CAH patients. While there are several classification systems for comparing the genital anatomy of our patient group, none is universally accepted. A common criticism of the Prader classification is that there is no correlation between the degree of external virilization and the level of confluence between the vagina and urethra [19]. While we included
Prader classifications in the first analysis of our data, we found no correlation between Prader and the surgery performed; therefore, we have not included these data in our manuscript. Some authors suggest that genital correction surgery should be performed after puberty when high hormone levels could potentially decrease the rate of complications [20,21]. Braga et al. [18] anecdotally reported that in postpubertal patients, the operation is more difficult due to the deepness of the pelvis, limited exposure, and increased tissue vascularity. We have also observed these difficulties in our patients. However, we generally found it difficult to
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H. Tugtepe et al. Table 1
Characteristics of patients.
No of patients Age at surgery Length of common channel Surgery time Follow-up time Complications
Partial UGSM group
Total UGSM group
Total UGSM group (common channel used as anterior vaginal wall)
11 47.2 months 1.25 cm (1e2 cm)
10 14.4 months 3.1 cm (2.5e3.5 cm)
8 21.3 months 4.3 cm (4e5 cm)
165.0 min 34.7 months None
193.1 min 36.3 months None
282.5 min 28.3 months UGS flap necrosis (1) Opening of sutures (1)
UGSM Z urogenital sinus mobilization.
separate the vagina from the sinus because of a thin vaginal wall. Recently, the use of estrogen cream for such patients has been suggested [6], and we have started to implement this also. Patients undergoing total UGSM or partial UGSM are at risk for developing complications such as urinary incontinence, urethrovaginal fistula, and vaginal or urethral stenosis. It has been suggested that UGSM can have an adverse effect on the bladder neck, as a result of its downward displacement. Hamza et al. [8] stated that the length of the urogenital sinus was longer than that of a normal urethra. Although we did not measure the urethral length at followup, we believe that the postoperative urethra remains longer than normal anatomy. However, Kryger and Gonzalez [22] showed that six of 13 patients that were dry preoperatively remained dry postoperatively. Podesta and Urcullo [23] found that one of 12 had postoperative stress incontinence that was treated with transurethral injection. Seventeen of our patients were aged over 4 years at follow-up and all were found to be continent and without urinary symptoms. The remaining group when questioned were found to have dry periods, suggesting that none of our patients has urinary incontinence. Of patients who had urodynamic evaluation, six of seven had normal urodynamic findings and one had an overactive detrusor. Vaginal stricture is reported as a rare complication. In this series, we observed no cases of vaginal stricture. Our follow-up time was an average of 3 years. Despite this length of time, there are reports in the literature demonstrating that this complication can be seen years after surgery [18,24,25]. In our series, we had two complications. The patient with UGS flap necrosis was the first patient in the third group. At postoperative day 5, the color of the flap changed and the vaginal opening was found to be closed 10 days later. We noticed the necrosis of the CC at cystoscopic examination. The postoperative appearance was no different from the preoperative appearance. A year later we performed the ASTRA (anterior sagittal transrectal approach), and she remains very well today. Our second complication was dehiscence of the labia majora and U-flap sutures due to wound infection. The vaginal opening was closed but the urethra was totally normal at control cystoscopy. She is to undergo a second operation. The literature reports concerns with regard to the longterm effects of total UGSM on the urinary tract and sexual
function, as this region is important, including clitoral innervation, neuromuscular anatomy of the bladder neck, and the intricate network of supporting tissue [9,26]. Theoretically, the more limited dissection of partial UGSM is thought to limit damage to these structures. However, our study has shown that with meticulous dissection and surgical technique, total and partial UGSM both have good cosmetic and functional results. In a similar finding, Palmer et al. [27] found that there was no difference in terms of urinary incontinence between total and partial UGSM. It is important to note however that patients with CAH are more likely to have urinary symptoms than normal controls [28]. It is not clear whether this is a result of surgery or an effect of CAH [29]. Our study is limited by its retrospective design, limited number of patients, and absence of long-term observational follow-up. Also, only one of our patients had reached puberty, so it was no possible to assess sexual function after surgery. The strength of this study is the fact that we examined all patients and performed cystoscopy under general anesthesia, at the third postoperative month.
Conclusions Total and partial UGSM are safe techniques for genital surgery of female CAH patients and results in good cosmetic and functional outcome. Despite less dissection in partial UGSM, the rates of complications are similar to total UGSM. The length of the CC should be used as a guide for the choice between total and partial UGSM. Further long-term studies are required to assess urinary and sexual functions.
Conflict of interest None.
Funding None.
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[17] Rink R. Total urogenital mobilization (TUM). Dial Pediatr Urol 2000;23:2e4. [18] Braga LHP, Lorenzo AJ, Tatsuo ES, Silva IN, Pippi Salle JL. Prospective evaluation of feminizing genitoplasty using partial urogenital sinus mobilization for congenital adrenal hyperplasia. J Urol 2006;176:2199e204. [19] Malone PS, Hall-Craggs MA, Mouriquand PDE, Caldamone AA. The anatomical assessment of disorders of sex development (DSD). J Pediatr Urol 2012;8:585e91. [20] Creighton SM. Long-term outcome of feminization surgery: the London experience. BJU Int 2004;93(Suppl. 3):44e6. [21] Minto CL, Liao L-M, Woodhouse CRJ, 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;361:1252e7. [22] Kryger JV, Gonza ´lez R. Urinary continence is well preserved after total urogenital mobilization. J Urol 2004;172:2384e6. [23] Podesta M, Urcullo J. Perineal mobilization of the common urogenital sinus for surgical correction of high urethrovaginal confluence in patients with intersex disorders. J Pediatr Urol 2008;4:352e8. [24] Hendren WH, Atala A. Repair of the high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome. J Pediatr Surg 1995;30:91e4. [25] Krege S, Walz KH, Hauffa BP, Ko ¨rner I, Ru ¨bben H. Long-term follow-up of female patients with congenital adrenal hyperplasia from 21-hydroxylase deficiency, with special emphasis on the results of vaginoplasty. BJU Int 2000;86:253e8. discussion 258e9. [26] Carroll PR, Dixon CM. Surgical anatomy of the male and female urethra. Urol Clin N Am 1992;19:339e46. [27] 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;187:1422e6. [28] Hannestad YS, Rortveit G, Sandvik H, Hunskaar S. A community-based epidemiological survey of female urinary incontinence: the Norwegian EPINCONT study. Epidemiology of Incontinence in the County of Nord-Trøndelag. J Clin Epidemiol 2000;53:1150e7. [29] Davies MC, Crouch NS, Woodhouse CRJ, Creighton SM. Congenital adrenal hyperplasia and lower urinary tract symptoms. BJU Int 2005;95:1263e6.
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