Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex
The authors from Copenhagen write about their 15-year consistent strategy in the treatment of antenatally suspected PUJ obstruction. The group deals with this controversial subject in some detail, and they outline data which they feel are helpful for urologists giving advice to parents about the advisability of having the condition treated by operative or conservative means.
The Cohen technique for the treatment of bilateral VUR, using a common submucosal tunnel, over an 18-year period is described by authors from Athens; they found that the technique offers excellent long-term results, and that crossing one ureter upon the other within the tunnel does not predispose to obstruction.
To review of the sexual and urogynaecological issues faced by a large cohort of women with the exstrophy-epispadias complex (EEC).
PATIENTS AND METHODS
The study comprised 83 women and girls with EEC; a confidential survey was mailed to identify their social and sexual concerns. Fifty-six women had classical bladder exstrophy (CBE), 13 had female epispadias (FE) and 14 had cloacal exstrophy (CE). Data on the initial method of reconstruction and urogynaecological problems were obtained from a review of the hospital records. Information on continence, infection and sexual function was obtained from 34 completed surveys.
The bladder was closed in 51 patients with CBE and 13 with CE. Urinary calculi developed in 10 patients with CBE, two with FE and three with CE. Vaginal and uterine prolapse occurred an earlier age in patients with EEC. Eight women had 13 pregnancies, eight of which resulted in normal healthy children. Overall continence was achieved in 85% of the women surveyed. Urinary tract infections remained a frequent problem for women with EEC; only 27% of respondents indicated that they were infection-free. Women aged> 18 years (24) who responded indicated that they had appropriate sexual desire; 16 were sexually active and the mean age for commencing sexual activity was 19.9 years. Six patients had dyspareunia and 10 indicated that they had orgasms. However, five additional patients indicated that they had restricted intercourse, as they were dissatisfied with the cosmesis of their external genitalia.
Sexual and gynaecological issues become increasingly important in patients with EEC as they become adults. Understanding these issues faced by patients with EEC as they mature will permit better counselling of future patients.
Girls with the exstrophy-epispadias complex (EEC) form the minority of patients with this significant abnormality. As these children become adults after successful reconstruction, physicians are increasingly confronted with the management of obstetric and gynaecological issues. The continued counselling of these girls and women about aspects of maturation and sexuality is also important for their successful integration into society. Few series have studied women with the EEC to determine the long-term management issues [1–3].
Most children with the EEC now undergo functional staged [4,5] or single-stage  reconstruction. The reconstitution of the pelvic floor with functional reconstruction can potentially affect later gynaecological, obstetric and urological function . We retrospectively reviewed the long-term results of management in a large cohort of girls and women with the EEC, to determine potential strategies for improving their management and assisting with future counselling of both parents and patients.
PATIENTS AND METHODS
The hospital records of 83 women and girls with EEC were reviewed (mean follow-up 14.1 years, mean age 24 years, range 13–52); of whom 56 (67%) were born with classical bladder exstrophy (CBE), 13 (16%) with female epispadias (FE) and 14 (17%) with cloacal exstrophy (CE). A confidential survey was mailed to all of the patients in the database and an age-adjusted survey to girls aged < 18 years; 34 (41%) completed the survey. The results of the survey were used to identify social and sexual concerns in the patients, and after review additional data were collected using follow-up telephone interviews. The institutional review board approved the study. The data were analysed using standard statistical methods.
The data were collected from two sources, i.e. on surgical reconstruction and stone formation from reviewing hospital records (83), and on sexual function, continence, UTIs, pregnancy and complications from completed surveys. Of the 34 completed surveys received, 24 were from patients with CBE, four from patients with FE and six from patients with CE. Ten patients were < 18 and 24 were ≥ 18 years old.
The initial method of surgical reconstruction was primary reconstruction or urinary diversion; 51 patients with CBE, all with FE and 13 with CE had undergone an initial attempt at reconstruction. In the 56 patients with CBE the bladder was closed initially in 51, in 19 by osteotomies (most with posterior iliac osteotomies), in 31 with no osteotomy and in one the use of osteotomy was not known. In 18 patients the initial closure failed (three with osteotomy, 15 without). Of these, 15 underwent a second attempt at bladder closure (nine with osteotomy). Three patients in whom the initial closure failed (with no osteotomy) had a urinary diversion with an ileal conduit (two) or rectosigmoid bladder (one). The bladder neck was reconstructed in 35 patients; 27 required later bladder augmentation or urinary diversion. Further genital surgery, as vaginoplasty, vaginal cutback, episiotomy or monsplasty, was performed in 41 of these 51 patients; five had initial urinary diversion, as an ileal conduit in four and a ureterosigmoidostomy in one. Three of these patients required additional genital reconstruction.
In the 13 patients with FE, all had initial reconstructive surgery, with later bladder neck reconstruction in 11. Augmentation or urinary diversion was used in three patients, in all of whom the bladder neck reconstruction had failed; additional genital surgery was required in seven patients.
Bladder closure was attempted in 13 of the 14 patients with CE, three with osteotomies; in nine the initial closure failed and in five of them the bladder was successfully closed with osteotomies. One patient had urinary reconstruction with a Kock pouch and another had initial urinary diversion with an ileal conduit. The bladder neck was reconstructed in five patients and 10 had a later bladder augmentation or urinary diversion, two of whom had had unsuccessful previous bladder neck reconstruction. Six patients underwent additional genital reconstructive surgery.
Twenty-four patients with CBE provided information on urinary continence; two had a cystectomy and were dry with bladder replacement (continent stoma or ileal conduit). Another 12 patients were completely dry; five who had a staged reconstruction only, four who had augmentation cystoplasty and three with a continent urinary diversion. Six patients reported occasional stress incontinence and one had only nocturnal enuresis. Three patients had continuous incontinence; two leaked urethrally despite having augmentation and bladder-neck plasty, and one leaked through a continent stoma. None of the four women with FE reported continuous incontinence, although two had an augmentation, one of whom had urinary diversion. Two women reported stress incontinence. One of the six patients with CE who responded had an incontinent urinary diversion. The other five had urinary reconstruction with augmentation cystoplasty and/or continent urinary diversion, one of whom had stress incontinence despite a catheterization regimen.
Ten patients with CBE (20%) developed urinary calculi; seven of these had augmentation cystoplasty with or without continent diversion, but three only had bladder closure. None of the five patients who had an initial incontinent diversion developed urinary calculi. Two patients with FE developed stones, one of whom had augmentation cystoplasty. Stones developed in three patients who had primary closure of CE; two of these also had bladder augmentation or urinary diversion. The patient who had initial urinary diversion with an ileal conduit also developed urinary calculi.
VAGINAL, UTERINE AND RECTAL PROLAPSE
Vaginal and uterine prolapse developed in 10 patients with CBE, the mean age at the time of prolapse being 16 years. Eight of these patients had a staged reconstruction and two had primary urinary diversion. Six of the patients who had a staged reconstruction had posterior iliac osteotomy, at a mean age of 2.1 years. Methods used to manage uterine and vaginal prolapse included uterine suspension in five patients, hysterectomy in one and partial hysterectomy with cervical stump suspension in one. One patient is awaiting suspension and two have had no treatment. Two women who had initial uterine suspension had a recurrence postpartum, and required a hysterectomy. One woman has failed two additional attempts at suspension and has recurrent prolapse.
Two patients with CE had vaginal prolapse at age 21 and 24 years, one of which was postpartum; an osteotomy was undertaken in both. The prolapse was corrected at the time of vaginal reconstruction in one patient and the other had a hysterectomy. Although posterior sacropexy has been used for managing vaginal and uterine prolapse, recurrent prolapse occurs frequently.
Of the whole group, three patients had a rectal prolapse; two had CBE and one CE, the latter having bladder closure when aged 5 years.
PREGNANCY AND COMPLICATIONS
Six women with CBE who had initial closure had 11 pregnancies, of which two were terminated by abortion and two by miscarriage. Five women delivered seven children, six by Caesarean section and one vaginally, the latter being uneventful. One patient had uterine prolapse during pregnancy and required surgery because a pessary failed. Two women had UTIs during pregnancy. One woman who had an initial urinary diversion had a spontaneous abortion. One woman with FE had a termination of pregnancy and one with CE who had an initial diversion delivered a child by Caesarean section. The latter was complicated by perforation of the Kock pouch and postpartum uterine prolapse. She also developed postpartum stool leakage and incontinence from her urinary stoma, which has become progressively worse. All of the infants were reported to be in good health, with none having exstrophy.
Of patients with CBE, nine responding to the questionnaire indicated that they had frequent UTIs, of whom five had urinary diversion/augmentation. Another seven patients have occasional UTIs and only six indicated that they were infection-free. Three of four patients with FE reported having occasional or frequent UTIs. Five of the six patients with CE indicated that they had occasional or frequent UTIs; all had undergone augmentation and/or urinary diversion.
Twenty-four patients with CBE responded to questions about sexual reconstruction and function; eight had no further genital reconstructive surgery after initial bladder closure, and 10 had further genital reconstruction, indicating satisfaction with the results. Five additional patients who had further reconstruction were dissatisfied with the results, the reasons given including the appearance of the genitalia in four and uterine prolapse in one. One other patient was ambivalent about the appearance of her genitalia.
Sixteen patients aged> 18 years answered questions about sexual activity; all had appropriate sexual desire. Five patients were not sexually active, although none implicated the appearance of their external genitalia to be a factor in their lack of sexual activity. Among the 10 patients who were sexually active, the mean age for starting sexual activity was 19.9 years. Three patients indicated that they restricted their sexual activity because of the appearance of the external genitalia. Three patients reported dyspareunia with all sexual activity, and two reported occasional dyspareunia. Seven patients indicated that they had orgasms, three were not sure if they had experienced orgasm and one woman reported none. Overall, seven women reported being satisfied and four dissatisfaction with their sexual lives (three because of cosmesis and one dyspareunia).
Of four patients with FE, three had successful follow-up genital surgery to improve cosmesis. Two of three patients aged> 18 years were sexually active; the third cited religious beliefs for abstinence. Both sexually active patients reported having orgasms and satisfactory sexual lives.
Four of six patients with CE had follow-up genital surgery. Three of five women aged> 18 years had sexual intercourse. One had stopped because of uterine prolapse and discomfort associated with intercourse. The other two patients indicated sexual satisfaction despite the presence of a urinary stoma in both. The two patients who were not sexually active reported reluctance because of the appearance of the genitalia in one and the presence of a stoma in the other.
The reconstruction of the EEC has developed, improving the functional and cosmetic outcome . As girls become adults, issues of sexuality and urogynaecological function become increasingly important. Knowing the long-term results of reconstruction will allow the development of better management strategies and improve the counselling of girls who have undergone reconstruction.
Staged functional reconstruction is the time-tested method for reconstructing bladder exstrophy [4,8], although recent success was reported with single-stage reconstruction of bladder and cloacal exstrophy [9,10]. Girls with CBE have not always had their genitalia reconstructed at the same time as bladder closure in all centres (as a clitoroplasty, bringing the clitoral halves together). In our unit the clitoris, labia minora, clitoral hood and mons are reconstructed at initial surgery, aided by the nearly routine use of pelvic osteotomies. Early urinary diversion, without bladder preservation, is still recommended in some centres , although initial ileal conduit urinary diversion is currently rarely used.
Similarly, the initial surgical reconstruction of FE has included genital reconstruction, as indicated above. The management of infants with CE is complex, involving management of the spine, bowel and bladder. Initial efforts are directed towards managing the spine and bowel, with genital reconstruction typically at the time of bladder closure . These patients have significant associated issues of bowel and lower extremity function that are important in later function and cosmesis.
Bladder neck reconstruction in the context of a staged closure in the EEC is reserved for those patients who remain incontinent on follow-up  and develop adequate bladder capacity. Continence is possible in most patients with CBE and FE after staged reconstruction, but most with CE require additional reconstructive efforts. Continence in staged reconstruction depends on successful initial bladder closure, with repeated closures leading to poorer continence . Bladder augmentation and urinary diversion are sometimes indicated to permit continence, but unfortunately have associated problems [14,15]. Inadequate emptying of mucus may be associated with the development of bladder calculi [16,17] or UTIs . Also, depending on the segment used, there can be electrolyte imbalances . Most patients who developed urinary calculi in the present study had undergone bladder augmentation and/or continent urinary diversion, but stones also developed in a few patients who had only staged reconstruction. However, the present incidence of UTIs did not seem to be significantly greater in patients who had augmentation cystoplasty than in those who had intact bladders with no bowel included in the urinary tract.
Vaginal and uterine prolapse was a significant problem in the present patients with CBE and CE, occurring at younger ages. Previous studies suggested that posterior extension of the perineal incision during genital reconstruction was the cause of prolapse . Many of the patients who had prolapse in this group were young and had had no further perineal reconstruction to account for this problem. Pubic diastasis associated with the EEC contributes to compromising the pelvic floor . This lack of an appropriately closed pelvic floor might be important in permitting the vagina and uterus to prolapse through the pelvic floor. In the present patients, functional reconstruction that included reconstitution of the pelvic ring seemed to have no protective effect, although few patients were assessed. The use of osteotomy to reconstitute the pelvic ring certainly improves the results of abdominal and bladder closure [19,20], but after early osteotomy pubic diastasis can recur ; this can be reduced by osteotomy later in life , although there is significant morbidity associated with this management. Efforts to correct prolapse can be difficult surgically; typically suspension to the sacrum, when present, is recommended. Uterine and vaginal prolapse can also significantly complicate pregnancy and delivery. Interestingly, rectal prolapse was not a significant problem in this group.
Genotypic females with the EEC have the potential to reproduce; there were 13 pregnancies in this group, eight of which proceeded to delivery, seven by Caesarean section. Although one patient had uncomplicated vaginal delivery, Caesarean section is recommended in those patients who have had a successful functional reconstruction . Also, the presence of a urologist with expertise in reconstructive surgery can decrease the morbidity associated with inadvertent injury to the reconstructed bladder.
All patients aged> 18 years had normal interest in sexual activity, the start of which was significantly later than normal . Although some patients expressed dissatisfaction with the appearance of their genitalia after reconstruction, only one with CE blamed the appearance as a reason for completely avoiding sexual activity. Also, some patients reported restricting their sexual activity because of the genital appearance. An improvement in genital appearance with later monsplasty has been reported . After initial reconstruction the pubic hair remains divided because of the dysplastic tissue covering the region of pubic diastasis , and some patients with CE may have residual clitoral separation after reconstruction . Dyspareunia was also a significant issue in 18% of the present patients, which has been reported in previous reviews, and correction with an episiotomy has been advised . As noted, correcting with an episiotomy should be cautious, to prevent the iatrogenic development of vaginal and uterine prolapse. Most of the sexually active patients expressed satisfaction with their sexual lives. Factors that contributed to sexual satisfaction were the presence of a steady relationship, and the partner's acceptance of the reconstructive history of the individual. The presence of continent or incontinent urinary diversion did not seem detract from sexual satisfaction.
In conclusion, women with the EEC after successful reconstruction can lead productive and satisfactory lives. The goal of reconstruction should be to improve the cosmetic and functional outcomes. Understanding some of the issues faced by patients as they become adults will allow better counselling of these patients, and understanding these factors will improve the management and development of strategies to pre-empt some of these issues.
classical bladder exstrophy
Urogynaecological and obstetric issues are important in women with the EEC, but not many authors have assessed them, with only a few reports focusing on this aspect. As girls mature into adulthood the cosmesis of the external genitalia, sexual function, possible complications affecting the internal and external genitalia, and the subject of pregnancy and delivery, become increasingly important. This report from a centre with one of the largest groups of such patients worldwide makes a significant contribution. Unfortunately fewer than half the patients answered the questionnaire and the data on sexual function were obtained from only 16 patients aged> 18 years. So although conclusions must be cautious, several important points can be highlighted.
In the sexually active group the cosmesis of the external genitalia was important and consequently the external genitalia should be reconstructed as carefully as they are in boys. Women with the EEC are prone to uterine prolapse and this raises the question of whether the uterus should be fixed prophylactically during other surgery (e.g. bladder augmentation, urinary diversion). Pregnancies are possible and many physicians prefer to use Caesarean section, to avoid complications.
Interestingly, there were many UTIs, with only a quarter of patients free of infection; it would be interesting to know how many had uncomplicated UTI and how many had febrile pyelonephritis. The incidence of stones was also high (20% of those with classical exstrophy), especially after bladder augmentation. Together with the infection rates, this suggests insufficient emptying of the reconstructed or augmented bladder.
This report further underlines the need of patients with EEC for a lifelong follow-up, with appropriate regard and care at particular ages and stages of development. These patients have many problems, achieving continence being only one; the others should not be underestimated. Despite the many patients assessed in this report, the authors should try to complete the results and follow these patients into adulthood, as this is the only way to learn more about the specific problems and demands of these patients.
Department of Urology, General Hospital,