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Issues with the External and Internal Genitalia in Postpubertal Females Born with Classic Bladder Exstrophy: A Surgical Series
Pediatric Adolescent Urology Issues with the External and Internal Genitalia in Postpubertal Females Born with Classic Bladder Exstrophy: A Surgical Series Marco Castagnetti MD, PhD 1,*, Alfredo Berrettini MD 1, Evisa Zhapa MD 1,2, Waifro Rigamonti MD 1, Filiberto Zattoni MD 2 1 2
Section of Paediatric Urology, Department of Oncological and Surgical Sciences, University Hospital of Padova, Padua, Italy Urology Unit, Department of Oncological and Surgical Sciences, University Hospital of Padova, Padua, Italy
a b s t r a c t Study Objective: To report our experience with surgical management of gynecological issues in postpubertal female patients born with classic bladder exstrophy (BE). Design: Retrospective review of clinical charts. Setting: Tertiary pediatric urology unit. Participants: 16 postpubertal female BE patients. Interventions: Cosmetic surgery to the external genitalia, widening of vaginal introitus, and treatment of pelvic organ prolapse. Main Outcome Measures: patient satisfaction and additional gynecological problems during follow-up. Results: Eight cases (14e43 years old) underwent cosmetic procedures. All resulted in improved cosmesis, but one case complained of reduced erogenous sensitivity after clitoridoplasty. Five cases (17e20 years old) underwent widening of the vaginal introitus. The modification was as short as possible to avoid any foreshortening of the dorsal vaginal wall. Three cases 33-45 years old presented with pelvic organ prolapse. All were sexually active. One had already given birth. All the three had previously been submitted elsewhere to a posterior vaginal cutback and one to hysterectomy. Two are still awaiting further treatment due to recurrent prolapse. Conclusion: Female BE patients can seek advice to improve the appearance of the external genitalia at any ages. Clidoridoplasty should be considered carefully, because it may harm erogenous sensitivity. Problems with the vaginal introitus typically present at around 20 years of age, probably when BE patients become sexually active. Opening too widely the introitus can cause a foreshortening of the posterior vaginal wall, predisposing to pelvic organ prolapse. Treatment of pelvic organ prolapse is difficult. Key Words: Bladder exstrophy, Gynecology, Vaginal stenosis, Uterine prolapse
Introduction
Bladder exstrophy is a complex genitourinary malformation. Historically, preservation of renal function and achievement of urinary continence were considered the main goals of treatment. Nowadays, these goals can consistently be achieved using different surgical strategies.1,2 Accumulating long-term series, however, have highlighted other aspects that require attention in the management of these patients, such as the cosmetic results of the reconstruction or the problems related to the genitalia. These aspects, often disregarded during the initial treatment, can become paramount, as the patient grows old, and particularly after puberty.3,4 In female BE cases, three major problems related to the genitalia have been defined.5,6 Patients can be unsatisfied with the appearance of the external genitalia, can have problems with a stenotic vaginal introitus noncompliant for intercourse, and can experience pelvic organ prolapse. All these problems have an anatomical basis.5,7,8 Indeed, in uncorrected cases, all the midline structures lie apart due to
* Address correspondence to: Marco Castagnetti, MD, Section of Paediatric Urology, Urology Unit, Department of Oncological and Surgical Sciences, University Hospital of Padova, Via Giustiniani 2, 35128e Padua, Italy E-mail address: [email protected] (M. Castagnetti).
the midline lower abdominal wall defect. The clitoris is bifid, the pubic areas lie on either side of the midline, and the labia majora, often rudimentary, lack superior fusion. Additionally, the vaginal introitus is often stenotic. The internal genitalia are anteriorly displaced following the herniation of the bladder. The vagina has a horizontal axis with the posterior wall parallel to the perineum. The uterus, in an almost normal position, enters the vagina in its anterior wall close to the introitus. Following the pubic diastasis, also the pelvic muscles diverge anteriorly so that they do not provide support to the pelvic organs. Here, we report on our experience with the surgical management of gynecological issues in postpubertal and adult female patients born with classic BE. Materials and Methods
We retrospectively reviewed the charts of 16 postpubertal female patients born with classic BE referred to our institution between 2002 and 2009 to undergo surgery of the internal or external genitalia. Median patient age was 20 (range 14e45) years. Procedures were subdivided into 3 groups, (1) cosmetic procedures to the external genitalia, (2) procedures for widening the stenotic vaginal introitus, and (3) procedures for pelvic organ prolapse.
1083-3188/$ - see front matter Ó 2011 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. doi:10.1016/j.jpag.2010.05.003
M. Castagnetti et al. / J Pediatr Adolesc Gynecol 24 (2011) 48e52
49
Fig. 1. Pre (a, c) and postoperative (b, d) appearance of two cases undergoing reconstruction of the lower abdominal wall and external genitalia. Note the midline depressed scar and labia configuration before and after surgery (a) and the distribution of pubic hairs (b).
Cosmetic procedures to the external genitalia mainly included excision of the midline scar, mobilization, and reapproximation of the subcutaneous fat, and skin closure. This allows for recreation of the mons pubis, reconfiguration of the escutcheon, and fusion of the anterior ends of the labia majora. The two clitoral halves were generally reapproximated in the midline, but two cases underwent formal clitoridoplasty with de-epithelialization of the midline surface and suturing together of the two clitoral halves. Results of such procedures were evaluated only by patient report. The widening of the vaginal introitus consisted of vaginal cutback without interposition of skin flaps. An episiotomy was never associated. In general, the introitus widening was as small as possible to avoid any foreshortening of the dorsal vaginal wall. A variety of procedures were used in patients with pelvic organ prolapse, as detailed in the results. Results
Eight cases with an age between 14 and 43 years were subjected to surgery of the external genitalia. All procedures
resulted in improved cosmesis, as reported by the patients (Fig. 1). One of the two cases undergoing formal clitoridoplasty complained of reduced erogenous sensitivity after surgery. Five cases were treated because of a narrow vaginal introitus. The age range in this group was 17 to 20 years. None presented with difficulties in draining vaginal secretion and menstrual fluids before surgery, and the indication was mainly the need to make the introitus compliant for intercourse. All the patients reported successful intercourse after surgery. Three further cases presented with pelvic organ prolapse. These were 33, 43, and 45 years old, respectively. All were sexually active. One had already given birth. All the three had previously undergone a generous posterior vaginal cutback plus episiotomy elsewhere. One patient was referred to our institution for a recurrent prolapse after previous hysterectomy and vaginal stump suspension (Fig. 2). The prolapse recurred within 6 months from surgery and this patient is currently awaiting further treatment. In the second case, we performed a fixation of the uterus to the anterior abdominal wall. This was a patient already submitted elsewhere to cystectomy and
50
M. Castagnetti et al. / J Pediatr Adolesc Gynecol 24 (2011) 48e52
Fig. 2. Recurrent vaginal prolapse after hysterectomy and vaginal stump suspension.Ă
urinary diversion in the sigmoid colon. Also in this patient, prolapse recurred within 6 months of surgery and she is currently awaiting further treatment. The third patient, finally, underwent a transvaginal sacro-spinous hysteropexy with a mesh (Fig. 3). She is currently well and has not experienced any recurrence after a follow-up of 3 years. Discussion
The present series offers an overview of possible issues related to the genitalia that female BE patients can
experience from puberty onward. Patients can seek treatment to improve the appearance of their external genitalia at any age. The presence of a narrow introitus usually prompts referral after the onset of puberty. Finally, older patients can experience pelvic organ prolapse. Many surgical techniques have been devised to improve the appearance of the lower abdominal wall and external genitalia in female BE patients.9e11 These mainly involve excision of the midline scar and re-approximation with superomedial rotation of the hair bearing tissue on either side.10 This allows reconfiguring the mons pubis and escutcheon, and fusion of the labia majora anteriorly. No single technique, however, appears to be universally applicable. The severity of pre-existing surgical scars and tissue properties are the two major variables influencing the decision-making.11 The possibility to achieve a tensionfree re-approximation of tissue is the key factor for success. For this reason Vanderbrink et al11 recommended the use of Z-plasty rather than linear sutures for skin closure. Mathews et al,6 instead, emphasized the role of osteotomy to reduce midline tension. Unfortunately, by the age these patients require surgery, the pelvic diastasis has usually recurred irrespective of the use of an osteotomy at initial bladder closure and a new osteotomy seems to be overwhelming.12,13 In our patients, extensive mobilization of skin and subcutaneous fat was generally enough to achieve tension-free closure irrespective of the use of a linear suture. In cases with severely scarred abdomens, use of tissue expanders has been reported. Again, however, skin stretching can alter tissue properties and quality.5 Among the possible cosmetic procedures, clitoridoplasty should be considered carefully. Although Vanderbrink et al14
Fig. 3. Intraoperative picture of the case undergoing sacro-spinous hysteropexy with mesh.Ă
M. Castagnetti et al. / J Pediatr Adolesc Gynecol 24 (2011) 48e52
suggested that it might improve the appearance of the external genitalia, one of our two patients who underwent clitoridoplasty reported loss of erogenous sensitivity after the procedure. In our opinion, accurate fusion of the labia anteriorly disguises the cosmetic abnormality recessing the bifid clitoris in a more concealed position making formal clitoridoplasty unnecessary.10 The ideal timing for reconstruction of the external genitalia remains controversial. Intuitively, reconstruction during initial surgery would avoid additional procedures, but this is not always possible, because additional continence surgeries may become necessary at a later stage. Additionally, in a few cases, accurate reconfiguration can be possible after pubic hair distribution becomes apparent.5 Likewise, there is much controversy on the ideal timing for widening of the vaginal introitus. Stein et al9 proposed a policy of a double cutback, one at 1 year of age to allow for drainage of vaginal secretions and menstrual fluids, and a second one after puberty to allow for intercourse. While we agree that, in a few patients, it is impossible for intercourse to take place unless the introitus has been widened, we disagree on the need for an early widening to allow for drainage of vaginal secretions and menstrual fluids. This was never an issue in our patients. Furthermore, vaginal stenosis can quickly recur in the absence of regular dilatations or intercourse.5 Hence, in keeping with Cervellione et al,15 we consider an age around 20 years to be the best for this procedure. If an introital widening is undertaken, it is well recognized that excessive widening should be avoided, because this can cause a foreshortening of the vagina, already shorter than normal in these patients, predisposing to pelvic organ prolapse. Stein et al9 reported uterine prolapse in 4 out of 23 of their patients shortly after vaginal cutback. In the present series, all the 3 patients experiencing prolapse had undergone a generous widening with episiotomy. Pregnancy is the other major acquired risk factor for prolapse in BE patients, with series reporting prevalence up to 100%.5,7e9,16e19 Nevertheless, prolapse can also occur in patients who have never had intercourse or pregnancy, as in two of our cases.6 Indeed, congenital anatomical factors such as a defective pelvic floor, an open pelvic ring with a wide transverse diameter, and a poor uterine support predispose BE patients to prolapse.5,6,16e20 In consideration of these factors, surgical techniques have been developed to attempt to improve the anatomy of the internal genitalia from initial neonatal repair already. Among these, total urogenital mobilization of the vagina and extrophic bladder template allows repositioning the vagina in a more posterior position,21 whereas radical mobilization and reconstruction of perineal soft tissues allows recreating a more normal support to pelvic organs.22 The use of osteotomy during initial closure, instead, has not proven to prevent subsequent pelvic organ prolapsed.6 Besides prophylactic hysteropexy techniques have been devised to prevent the occurrence of prolapse. These include fixation of the uterus to the anterior abdominal wall and rotundum psoas hitching.20 They can be used in patients who underwent cystectomy plus intestinal urinary diversion, and functional reconstruction, respectively. When pelvic organ prolapse
51
occurs, however, it is one of the most troublesome gynecological issues to treat in BE patients.5,7,14 Our case experiencing organ pelvic prolapse after hysterectomy and vaginal stump suspension confirms that the uterus should not be removed, because it is the only solid organ in the pelvis that has any prospect of supporting the pelvic floor.5 In one patient, we performed a sacrospinous hysteropexy23 that proved successful. This is, to our knowledge, the first report of this kind of hysteropexy in this context. Of note, the procedure proved quite difficult due to the abnormal pelvic anatomy. In keeping with recent literature, we believe that hysterosacropexy with a mesh remains the technique of choice for treatment in these patients, although the presence of dense intra-abdominal adhesions might make also this procedure difficult.4,17 It is important to note that for all the techniques mentioned above, the possibility of a pregnancy after treatment remains almost unknown. Cesarean section should, therefore, always be recommended.5,6,19 As with any surgical series, also the current series has limitations. To begin with, it includes only a limited number of patients. Nevertheless, this is almost always an issue with BE patients and particularly with females, because these are a minority of the already small BE population.24 Additionally, the present series included only those cases requiring surgery, so we lack a denominator for the study population. Finally, we did not address the issues related to pregnancy and delivery in our patients. Conclusions
This experience offers an overview of possible issues with the external and internal genitalia in BE patients after puberty. The appearance of the external genitalia can prompt the patient to seek treatment at any age. Several procedures can improve cosmesis, but clidoridoplasty should be considered carefully, because it can harm erogenous sensitivity. Problems with a stenotic vaginal introitus typically present at around 20 years of age probably in relation to sexual debut. A cutback of the introitus can fix the problem, but surgeons should avoid opening too widely the introitus because, given the anatomy of BE patients, this can cause a severe foreshortening of the posterior vaginal wall, increasing the already high risk of uterine prolapse at an older age. Treatment of the latter is difficult. References 1. Purves JT, Baird AD, Gearhart JP: The modern staged repair of bladder exstrophy in the female: a contemporary series. J Pediatr Urol 2008; 4:150 2. Gobet R, Weber D, Renzulli P, et al: Long-term follow up (37-69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: uro-nephrological outcome. J Pediatr Urol 2009; 5:190 3. Meyer KF, Freitas Filho LG, Martins DM, et al: The exstrophy-epispadias complex: is aesthetic appearance important? BJU Int 2004; 93:1062 4. Wilson C, Christie D, Woodhouse CR: The ambitions of adolescents born with exstrophy: a structured survey. BJU Int 2004; 94:607 5. Woodhouse CR: The gynaecology of exstrophy. BJU Int 1999; 83(Suppl 3):34 6. Mathews RI, Gan M, Gearhart JP: Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int 2003; 91:845 7. Stec AA, Pannu HK, Tadros YE, et al: Pelvic floor anatomy in classical bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol 2001; 166:1444 8. Gargollo PC, Borer JG, Retik AB, et al: Magnetic resonance imaging of pelvic musculoskeletal and genitourinary anatomy in patients before and after complete primary repair of bladder exstrophy. J Urol 2005; 174:1559
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9. Stein R, Fisch M, Bauer H, et al: Operative reconstruction of the external and internal genitalia in female patients with bladder exstrophy or incontinent epispadias. J Urol 1995; 154:1002 10. Cook AJ, Farhat WA, Cartwright LM, et al: Simplified mons plasty: a new technique to improve cosmesis in females with the exstrophy-epispadias complex. J Urol 2005; 173:2117 11. VanderBrink BA, Stock JA, Hanna MK: Aesthetic aspects of bladder exstrophy: results of puboplasty. J Urol 2006; 176:1810 12. Sponseller PD, Jani MM, Jeffs RD, et al: Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am 2001; 83A:184 13. Castagnetti M, Gigante C, Perrone G, et al: Comparison of musculoskeletal and urological functional outcomes in patients with bladder exstrophy undergoing repair with and without osteotomy. Pediatr Surg Int 2008; 24: 689 14. Vanderbrink BA, Stock JA, Hanna MK: Aesthetic aspects of reconstructive clitoroplasty in females with bladder exstrophy-epispadias complex. J Plast Reconstr Aesthet Surg 2010.10.1016/j.bjps.2010.02.005 15. Cervellione RM, Phillips T, Baradaran N, et al: Vaginoplasty in the female exstrophy population: Outcomes and complications. J Pediatr Urol 2010. 10.1016/j.jpurol.2010.01.011 16. Blakeley CR, Mills WG: The obstetric and gynaecological complications of bladder exstrophy and epispadias. Br J Obstet Gynaecol 1981; 88:167
17. Muir TW, Aspera AM, Rackley RR, et al: Recurrent pelvic organ prolapse in a woman with bladder exstrophy: a case report of surgical management and review of the literature. Int Urogynecol J Pelvic Floor Dysfunct 2004; 15:436 18. Rose CH, Rowe TF, Cox SM, et al: Uterine prolapse associated with bladder exstrophy: surgical management and subsequent pregnancy. J Matern Fetal Med 2000; 9:150 19. Mantel A, Lemoine JP, Descargues G, et al: Bladder exstrophy: gynecological and obstetrical characteristics with reference to three cases. Eur J Obstet Gynecol Reprod Biol 2001; 94:296 20. Boemers TM, Schimke CM, Ludwikowski B, et al: Rotundum psoas hitch: a new method for colpohysteropexy in girls with bladder exstrophy. J Pediatr Urol 2005; 1:337 21. Kropp BP, Cheng EY: Total urogenital complex mobilization in female patients with exstrophy. J Urol 2000; 164:1035 22. Jarzebowski AC, McMullin ND, Grover SR, et al: The Kelly technique of bladder exstrophy repair: continence, cosmesis and pelvic organ prolapse outcomes. J Urol 2009; 182(4 Suppl):1802 23. Dietz V, Schraffordt Koops SE, van der Vaart CH: Vaginal surgery for uterine descent; which options do we have? A review of the literature. Int Urogynecol J Pelvic Floor Dysfunct 2009; 20:349 € ller T, Mu € ller M, et al: Epidemiological survey of 214 families 24. Gambhir L, Ho with bladder exstrophy-epispadias complex. J Urol 2008; 179:1539
Section of Paediatric Urology, Department of Oncological and Surgical Sciences, University Hospital of Padova, Padua, Italy Urology Unit, Department of Oncological and Surgical Sciences, University Hospital of Padova, Padua, Italy
a b s t r a c t Study Objective: To report our experience with surgical management of gynecological issues in postpubertal female patients born with classic bladder exstrophy (BE). Design: Retrospective review of clinical charts. Setting: Tertiary pediatric urology unit. Participants: 16 postpubertal female BE patients. Interventions: Cosmetic surgery to the external genitalia, widening of vaginal introitus, and treatment of pelvic organ prolapse. Main Outcome Measures: patient satisfaction and additional gynecological problems during follow-up. Results: Eight cases (14e43 years old) underwent cosmetic procedures. All resulted in improved cosmesis, but one case complained of reduced erogenous sensitivity after clitoridoplasty. Five cases (17e20 years old) underwent widening of the vaginal introitus. The modification was as short as possible to avoid any foreshortening of the dorsal vaginal wall. Three cases 33-45 years old presented with pelvic organ prolapse. All were sexually active. One had already given birth. All the three had previously been submitted elsewhere to a posterior vaginal cutback and one to hysterectomy. Two are still awaiting further treatment due to recurrent prolapse. Conclusion: Female BE patients can seek advice to improve the appearance of the external genitalia at any ages. Clidoridoplasty should be considered carefully, because it may harm erogenous sensitivity. Problems with the vaginal introitus typically present at around 20 years of age, probably when BE patients become sexually active. Opening too widely the introitus can cause a foreshortening of the posterior vaginal wall, predisposing to pelvic organ prolapse. Treatment of pelvic organ prolapse is difficult. Key Words: Bladder exstrophy, Gynecology, Vaginal stenosis, Uterine prolapse
Introduction
Bladder exstrophy is a complex genitourinary malformation. Historically, preservation of renal function and achievement of urinary continence were considered the main goals of treatment. Nowadays, these goals can consistently be achieved using different surgical strategies.1,2 Accumulating long-term series, however, have highlighted other aspects that require attention in the management of these patients, such as the cosmetic results of the reconstruction or the problems related to the genitalia. These aspects, often disregarded during the initial treatment, can become paramount, as the patient grows old, and particularly after puberty.3,4 In female BE cases, three major problems related to the genitalia have been defined.5,6 Patients can be unsatisfied with the appearance of the external genitalia, can have problems with a stenotic vaginal introitus noncompliant for intercourse, and can experience pelvic organ prolapse. All these problems have an anatomical basis.5,7,8 Indeed, in uncorrected cases, all the midline structures lie apart due to
* Address correspondence to: Marco Castagnetti, MD, Section of Paediatric Urology, Urology Unit, Department of Oncological and Surgical Sciences, University Hospital of Padova, Via Giustiniani 2, 35128e Padua, Italy E-mail address: [email protected] (M. Castagnetti).
the midline lower abdominal wall defect. The clitoris is bifid, the pubic areas lie on either side of the midline, and the labia majora, often rudimentary, lack superior fusion. Additionally, the vaginal introitus is often stenotic. The internal genitalia are anteriorly displaced following the herniation of the bladder. The vagina has a horizontal axis with the posterior wall parallel to the perineum. The uterus, in an almost normal position, enters the vagina in its anterior wall close to the introitus. Following the pubic diastasis, also the pelvic muscles diverge anteriorly so that they do not provide support to the pelvic organs. Here, we report on our experience with the surgical management of gynecological issues in postpubertal and adult female patients born with classic BE. Materials and Methods
We retrospectively reviewed the charts of 16 postpubertal female patients born with classic BE referred to our institution between 2002 and 2009 to undergo surgery of the internal or external genitalia. Median patient age was 20 (range 14e45) years. Procedures were subdivided into 3 groups, (1) cosmetic procedures to the external genitalia, (2) procedures for widening the stenotic vaginal introitus, and (3) procedures for pelvic organ prolapse.
1083-3188/$ - see front matter Ó 2011 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. doi:10.1016/j.jpag.2010.05.003
M. Castagnetti et al. / J Pediatr Adolesc Gynecol 24 (2011) 48e52
49
Fig. 1. Pre (a, c) and postoperative (b, d) appearance of two cases undergoing reconstruction of the lower abdominal wall and external genitalia. Note the midline depressed scar and labia configuration before and after surgery (a) and the distribution of pubic hairs (b).
Cosmetic procedures to the external genitalia mainly included excision of the midline scar, mobilization, and reapproximation of the subcutaneous fat, and skin closure. This allows for recreation of the mons pubis, reconfiguration of the escutcheon, and fusion of the anterior ends of the labia majora. The two clitoral halves were generally reapproximated in the midline, but two cases underwent formal clitoridoplasty with de-epithelialization of the midline surface and suturing together of the two clitoral halves. Results of such procedures were evaluated only by patient report. The widening of the vaginal introitus consisted of vaginal cutback without interposition of skin flaps. An episiotomy was never associated. In general, the introitus widening was as small as possible to avoid any foreshortening of the dorsal vaginal wall. A variety of procedures were used in patients with pelvic organ prolapse, as detailed in the results. Results
Eight cases with an age between 14 and 43 years were subjected to surgery of the external genitalia. All procedures
resulted in improved cosmesis, as reported by the patients (Fig. 1). One of the two cases undergoing formal clitoridoplasty complained of reduced erogenous sensitivity after surgery. Five cases were treated because of a narrow vaginal introitus. The age range in this group was 17 to 20 years. None presented with difficulties in draining vaginal secretion and menstrual fluids before surgery, and the indication was mainly the need to make the introitus compliant for intercourse. All the patients reported successful intercourse after surgery. Three further cases presented with pelvic organ prolapse. These were 33, 43, and 45 years old, respectively. All were sexually active. One had already given birth. All the three had previously undergone a generous posterior vaginal cutback plus episiotomy elsewhere. One patient was referred to our institution for a recurrent prolapse after previous hysterectomy and vaginal stump suspension (Fig. 2). The prolapse recurred within 6 months from surgery and this patient is currently awaiting further treatment. In the second case, we performed a fixation of the uterus to the anterior abdominal wall. This was a patient already submitted elsewhere to cystectomy and
50
M. Castagnetti et al. / J Pediatr Adolesc Gynecol 24 (2011) 48e52
Fig. 2. Recurrent vaginal prolapse after hysterectomy and vaginal stump suspension.Ă
urinary diversion in the sigmoid colon. Also in this patient, prolapse recurred within 6 months of surgery and she is currently awaiting further treatment. The third patient, finally, underwent a transvaginal sacro-spinous hysteropexy with a mesh (Fig. 3). She is currently well and has not experienced any recurrence after a follow-up of 3 years. Discussion
The present series offers an overview of possible issues related to the genitalia that female BE patients can
experience from puberty onward. Patients can seek treatment to improve the appearance of their external genitalia at any age. The presence of a narrow introitus usually prompts referral after the onset of puberty. Finally, older patients can experience pelvic organ prolapse. Many surgical techniques have been devised to improve the appearance of the lower abdominal wall and external genitalia in female BE patients.9e11 These mainly involve excision of the midline scar and re-approximation with superomedial rotation of the hair bearing tissue on either side.10 This allows reconfiguring the mons pubis and escutcheon, and fusion of the labia majora anteriorly. No single technique, however, appears to be universally applicable. The severity of pre-existing surgical scars and tissue properties are the two major variables influencing the decision-making.11 The possibility to achieve a tensionfree re-approximation of tissue is the key factor for success. For this reason Vanderbrink et al11 recommended the use of Z-plasty rather than linear sutures for skin closure. Mathews et al,6 instead, emphasized the role of osteotomy to reduce midline tension. Unfortunately, by the age these patients require surgery, the pelvic diastasis has usually recurred irrespective of the use of an osteotomy at initial bladder closure and a new osteotomy seems to be overwhelming.12,13 In our patients, extensive mobilization of skin and subcutaneous fat was generally enough to achieve tension-free closure irrespective of the use of a linear suture. In cases with severely scarred abdomens, use of tissue expanders has been reported. Again, however, skin stretching can alter tissue properties and quality.5 Among the possible cosmetic procedures, clitoridoplasty should be considered carefully. Although Vanderbrink et al14
Fig. 3. Intraoperative picture of the case undergoing sacro-spinous hysteropexy with mesh.Ă
M. Castagnetti et al. / J Pediatr Adolesc Gynecol 24 (2011) 48e52
suggested that it might improve the appearance of the external genitalia, one of our two patients who underwent clitoridoplasty reported loss of erogenous sensitivity after the procedure. In our opinion, accurate fusion of the labia anteriorly disguises the cosmetic abnormality recessing the bifid clitoris in a more concealed position making formal clitoridoplasty unnecessary.10 The ideal timing for reconstruction of the external genitalia remains controversial. Intuitively, reconstruction during initial surgery would avoid additional procedures, but this is not always possible, because additional continence surgeries may become necessary at a later stage. Additionally, in a few cases, accurate reconfiguration can be possible after pubic hair distribution becomes apparent.5 Likewise, there is much controversy on the ideal timing for widening of the vaginal introitus. Stein et al9 proposed a policy of a double cutback, one at 1 year of age to allow for drainage of vaginal secretions and menstrual fluids, and a second one after puberty to allow for intercourse. While we agree that, in a few patients, it is impossible for intercourse to take place unless the introitus has been widened, we disagree on the need for an early widening to allow for drainage of vaginal secretions and menstrual fluids. This was never an issue in our patients. Furthermore, vaginal stenosis can quickly recur in the absence of regular dilatations or intercourse.5 Hence, in keeping with Cervellione et al,15 we consider an age around 20 years to be the best for this procedure. If an introital widening is undertaken, it is well recognized that excessive widening should be avoided, because this can cause a foreshortening of the vagina, already shorter than normal in these patients, predisposing to pelvic organ prolapse. Stein et al9 reported uterine prolapse in 4 out of 23 of their patients shortly after vaginal cutback. In the present series, all the 3 patients experiencing prolapse had undergone a generous widening with episiotomy. Pregnancy is the other major acquired risk factor for prolapse in BE patients, with series reporting prevalence up to 100%.5,7e9,16e19 Nevertheless, prolapse can also occur in patients who have never had intercourse or pregnancy, as in two of our cases.6 Indeed, congenital anatomical factors such as a defective pelvic floor, an open pelvic ring with a wide transverse diameter, and a poor uterine support predispose BE patients to prolapse.5,6,16e20 In consideration of these factors, surgical techniques have been developed to attempt to improve the anatomy of the internal genitalia from initial neonatal repair already. Among these, total urogenital mobilization of the vagina and extrophic bladder template allows repositioning the vagina in a more posterior position,21 whereas radical mobilization and reconstruction of perineal soft tissues allows recreating a more normal support to pelvic organs.22 The use of osteotomy during initial closure, instead, has not proven to prevent subsequent pelvic organ prolapsed.6 Besides prophylactic hysteropexy techniques have been devised to prevent the occurrence of prolapse. These include fixation of the uterus to the anterior abdominal wall and rotundum psoas hitching.20 They can be used in patients who underwent cystectomy plus intestinal urinary diversion, and functional reconstruction, respectively. When pelvic organ prolapse
51
occurs, however, it is one of the most troublesome gynecological issues to treat in BE patients.5,7,14 Our case experiencing organ pelvic prolapse after hysterectomy and vaginal stump suspension confirms that the uterus should not be removed, because it is the only solid organ in the pelvis that has any prospect of supporting the pelvic floor.5 In one patient, we performed a sacrospinous hysteropexy23 that proved successful. This is, to our knowledge, the first report of this kind of hysteropexy in this context. Of note, the procedure proved quite difficult due to the abnormal pelvic anatomy. In keeping with recent literature, we believe that hysterosacropexy with a mesh remains the technique of choice for treatment in these patients, although the presence of dense intra-abdominal adhesions might make also this procedure difficult.4,17 It is important to note that for all the techniques mentioned above, the possibility of a pregnancy after treatment remains almost unknown. Cesarean section should, therefore, always be recommended.5,6,19 As with any surgical series, also the current series has limitations. To begin with, it includes only a limited number of patients. Nevertheless, this is almost always an issue with BE patients and particularly with females, because these are a minority of the already small BE population.24 Additionally, the present series included only those cases requiring surgery, so we lack a denominator for the study population. Finally, we did not address the issues related to pregnancy and delivery in our patients. Conclusions
This experience offers an overview of possible issues with the external and internal genitalia in BE patients after puberty. The appearance of the external genitalia can prompt the patient to seek treatment at any age. Several procedures can improve cosmesis, but clidoridoplasty should be considered carefully, because it can harm erogenous sensitivity. Problems with a stenotic vaginal introitus typically present at around 20 years of age probably in relation to sexual debut. A cutback of the introitus can fix the problem, but surgeons should avoid opening too widely the introitus because, given the anatomy of BE patients, this can cause a severe foreshortening of the posterior vaginal wall, increasing the already high risk of uterine prolapse at an older age. Treatment of the latter is difficult. References 1. Purves JT, Baird AD, Gearhart JP: The modern staged repair of bladder exstrophy in the female: a contemporary series. J Pediatr Urol 2008; 4:150 2. Gobet R, Weber D, Renzulli P, et al: Long-term follow up (37-69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: uro-nephrological outcome. J Pediatr Urol 2009; 5:190 3. Meyer KF, Freitas Filho LG, Martins DM, et al: The exstrophy-epispadias complex: is aesthetic appearance important? BJU Int 2004; 93:1062 4. Wilson C, Christie D, Woodhouse CR: The ambitions of adolescents born with exstrophy: a structured survey. BJU Int 2004; 94:607 5. Woodhouse CR: The gynaecology of exstrophy. BJU Int 1999; 83(Suppl 3):34 6. Mathews RI, Gan M, Gearhart JP: Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int 2003; 91:845 7. Stec AA, Pannu HK, Tadros YE, et al: Pelvic floor anatomy in classical bladder exstrophy using 3-dimensional computerized tomography: initial insights. J Urol 2001; 166:1444 8. Gargollo PC, Borer JG, Retik AB, et al: Magnetic resonance imaging of pelvic musculoskeletal and genitourinary anatomy in patients before and after complete primary repair of bladder exstrophy. J Urol 2005; 174:1559
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9. Stein R, Fisch M, Bauer H, et al: Operative reconstruction of the external and internal genitalia in female patients with bladder exstrophy or incontinent epispadias. J Urol 1995; 154:1002 10. Cook AJ, Farhat WA, Cartwright LM, et al: Simplified mons plasty: a new technique to improve cosmesis in females with the exstrophy-epispadias complex. J Urol 2005; 173:2117 11. VanderBrink BA, Stock JA, Hanna MK: Aesthetic aspects of bladder exstrophy: results of puboplasty. J Urol 2006; 176:1810 12. Sponseller PD, Jani MM, Jeffs RD, et al: Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am 2001; 83A:184 13. Castagnetti M, Gigante C, Perrone G, et al: Comparison of musculoskeletal and urological functional outcomes in patients with bladder exstrophy undergoing repair with and without osteotomy. Pediatr Surg Int 2008; 24: 689 14. Vanderbrink BA, Stock JA, Hanna MK: Aesthetic aspects of reconstructive clitoroplasty in females with bladder exstrophy-epispadias complex. J Plast Reconstr Aesthet Surg 2010.10.1016/j.bjps.2010.02.005 15. Cervellione RM, Phillips T, Baradaran N, et al: Vaginoplasty in the female exstrophy population: Outcomes and complications. J Pediatr Urol 2010. 10.1016/j.jpurol.2010.01.011 16. Blakeley CR, Mills WG: The obstetric and gynaecological complications of bladder exstrophy and epispadias. Br J Obstet Gynaecol 1981; 88:167
17. Muir TW, Aspera AM, Rackley RR, et al: Recurrent pelvic organ prolapse in a woman with bladder exstrophy: a case report of surgical management and review of the literature. Int Urogynecol J Pelvic Floor Dysfunct 2004; 15:436 18. Rose CH, Rowe TF, Cox SM, et al: Uterine prolapse associated with bladder exstrophy: surgical management and subsequent pregnancy. J Matern Fetal Med 2000; 9:150 19. Mantel A, Lemoine JP, Descargues G, et al: Bladder exstrophy: gynecological and obstetrical characteristics with reference to three cases. Eur J Obstet Gynecol Reprod Biol 2001; 94:296 20. Boemers TM, Schimke CM, Ludwikowski B, et al: Rotundum psoas hitch: a new method for colpohysteropexy in girls with bladder exstrophy. J Pediatr Urol 2005; 1:337 21. Kropp BP, Cheng EY: Total urogenital complex mobilization in female patients with exstrophy. J Urol 2000; 164:1035 22. Jarzebowski AC, McMullin ND, Grover SR, et al: The Kelly technique of bladder exstrophy repair: continence, cosmesis and pelvic organ prolapse outcomes. J Urol 2009; 182(4 Suppl):1802 23. Dietz V, Schraffordt Koops SE, van der Vaart CH: Vaginal surgery for uterine descent; which options do we have? A review of the literature. Int Urogynecol J Pelvic Floor Dysfunct 2009; 20:349 € ller T, Mu € ller M, et al: Epidemiological survey of 214 families 24. Gambhir L, Ho with bladder exstrophy-epispadias complex. J Urol 2008; 179:1539