Int Urogynecol J DOI 10.1007/s00192-013-2285-6
CASE REPORT
Bladder exstrophy combined with uterovaginal prolapse and its surgical management: case report and literature review F. Israfil-Bayli & M. Belal & P. Toozs-Hobson
Received: 5 July 2013 / Accepted: 17 November 2013 # The International Urogynecological Association 2013
Abstract We describe a case of a postmenopausal woman with a corrected bladder exstrophy (BE) who presented with a uterovaginal prolapse complicated by calcified fibroids and discuss its surgical management. A 51-year-old woman presented with a uterovaginal prolapse. She had previously had one full term delivery by caesarean section 20 years ago. She had an ileal conduit performed for her BE at birth. A computed tomography scan revealed significant pelvic anomaly and the uterus contained multiple calcified fibroids. Due to the complexity of her condition the surgical management was planned in two stages: initial examination under anaesthetic in conjunction with a consultant urologist and then the definitive planned reconstructive surgery. This case describes the need for careful evaluation and planning of complex surgery and the successful surgical management option for correction of uterovaginal prolapse in patients with BE. A vaginal approach seemed to be a most sensible route to correct the prolapse in view of her previous multiple abdominal surgeries.
Keywords Uterovaginal prolapse . Bladder exstrophy (BE) . POP-Q classification . Sacrospinous fixation
Introduction Bladder exstrophy (BE) is a rare congenital abnormality occurring once in every 10,000–50,000 live births affecting males and females equally [1]. The condition arises from a failure of midline closure resulting in a defect in the tissues and absence of anterior bladder wall. As a result posterior bladder protrudes on the anterior abdominal wall. It is commonly combined with anomaly of the anterior bony pelvis and external genitalia [2]. In females the vagina may be displaced anteriorly and coexists with epispadias. BE is usually corrected surgically straight after birth. Long-term problems in patients with BE include incontinence, recurrent urinary tract infections, reflux, uterine prolapse and adenocarcinoma of bladder and colon.
Case report Electronic supplementary material The online version of this article (doi:10.1007/s00192-013-2285-6) contains supplementary material, which is available to authorized users. F. Israfil-Bayli (*) : P. Toozs-Hobson Department of Urogynaecology and Pelvic Floor Medicine, Birmingham Women’s Hospital NHS Foundation Trust, Mindelsohn Way, Birmingham B15 2TG, UK e-mail: [email protected] F. Israfil-Bayli e-mail: [email protected] F. Israfil-Bayli e-mail: [email protected] M. Belal University Hospital Birmingham, Birmingham, UK
A 51-year-old postmenopausal woman presented to the urogynaecology clinic complaining of “lump in vagina”, discomfort in perianal region and inability to have sexual intercourse. She did not have any bladder or bowel symptoms. She had had an ileal conduit constructed as a result of correction of her BE at birth. The conduit was functioning without any problems. Twenty years previously she had had one full term pregnancy and was delivered via caesarean section. Otherwise medically she was fit and well other than mild arthritis affecting her knees and wrists. Physical examination, including Pelvic Organ Prolapse Quantification (POP-Q) was performed (Aa+2, Ap+2, TVL 6, Ap −1, Bp −1, C+4, D −1, PB 2, GH 3). Tissues were
Int Urogynecol J
Fig. 1 CT scan performed prior to examination under anaesthetic
extremely atrophic and macerated. The whole anatomy of the vulval region was distorted due to absent symphysis pubis and scars from previous surgery for correction of BE. Conservative measures such as physiotherapy and vaginal pessaries had been unsuccessful due to the severity of the prolapse and extreme nature of the distortion to the pelvic anatomy. As a result a decision was made to correct uterovaginal prolapse surgically. Prior to undertaking this a computed tomography (CT) scan was performed to give more information about the patient’s anatomy. The CT confirmed an incompletely formed sacrum and absent coccyx. Also there was an incomplete fusion of some of the posterior sacral elements, particularly on the left side. The pubic rami were incomplete anteriorly with only the lateral aspects present, so no symphysis was seen. Iliac wings and both hip joints were normal. The uterus
also contained multiple (>10 cm) fibromas which were calcified (Fig. 1). In view of the above finding we decided on a two-stage approach. The first step was examination of the patient in theatre together with a consultant urologist with CT images of the pelvis available at hand to identify what could potentially be achieved. The examination under anaesthetic revealed extensive scarring of the lower abdominal area and confirmed the absent pubic bones. The procidentia was reducible but there was thickening of the anterior vaginal wall. POP-Q performed in theatre confirmed findings described above. It was felt that the uterus was contributing to her symptoms and a vaginal hysterectomy approach most appropriate so she was planned for vaginal hysterectomy with possible sacrospinous fixation. The second step involved definitive surgery to correct the uterovaginal prolapse (Fig. 2).
Fig. 2 Intraoperative images. The specimen needed to be delivered in piecemeal to allow volumetric reduction of the uterus so that the fundus could be safely dissected of the adhesions from the previous paediatric reconstructive surgery and caesarean section. Vaginal hysterectomy was followed by sacrospinous fixation. Conventional trocar mesh kits could
not be used and there was inadequate space for a Miya Hook™ because of the anatomy. A Capio™ was used to secure level 1 support to suspend the cystocele due to the lack of ventral support of the pubocervical fascia. Then she had a modified anterior repair and vault was closed. Total vaginal length after surgery was 6 cm with C at −6
Int Urogynecol J
The patient made a good recovery and was reviewed in the outpatient clinic 8 weeks and 8 months respectively following surgery. She did not have any symptoms of prolapse and was happy with the outcome of surgery. POP-Q was performed and at both reviews was the same (Aa −2, Ba −2, C −6, PB 2, GH 3, TVL 6, Ap −2, Bp −2, D absent). We have used the electronic Pelvic Floor Assessment Questionnaire (ePAQ) before and after surgery. Summary of e-PAQ response postoperatively revealed improvement in pain and sensation of the vaginal domain and general improvement in sex life in the sexual domain.
Discussion On reviewing the literature there are several case reports on surgical management of uterovaginal prolapse in patients with BE [3, 4]. However, there are no reports on vaginal hysterectomy where the uterus was significantly enlarged with calcified fibroids to 10 cm in diameter and delivered vaginally, or where sacrospinous fixation has been employed to secure level 1 support. There are several surgical techniques that have been proposed to treat prolapse in this cohort of patients. In this case the surgical approach was complicated by the distorted anatomy including absent symphysis, absent coccyx and partial sacrum, shortened vagina and abnormal muscles. This resulted in gross distortion of the anatomy, with a reduction in the normal spaces with the pelvis utilized in complex reconstructive surgery so that access and space to insert sutures into the sacrospinous ligament safely were dramatically reduced rendering the more commonly used techniques such as a Myer Hook redundant. Reported abdominal procedures involve sacral colpopexy or rotundum psoas hitch [5]. Reported vaginal procedures involve sacrospinous hysteropexy with uterine preservation or insertion of the Prolift mesh [2]. There are certain limitations of pelvic floor surgery in patients with BE due to absence of anterior bony pelvis and distorted fibromuscular support. We decided on the vaginal route because of the history of previous abdominal surgeries and as a result high likelihood of intraoperative organ damage and/or complications to the ileal conduit. There was no intention to perform uterine preserving procedure as the patient was menopausal and the uterus had significant fibromatous
changes. So in order to provide apical support sacrospinous fixation was performed using the Capio® device to compensate for the reduced access from the distortion of the anatomy.
Conclusion This case describes the need for careful evaluation and planning of complex surgery in patients with BE and the successful surgical management corrections of uterovaginal prolapse. The case involves utilization of both relevant surgical specialties and highlights where a modified vaginal approach may be preferable in women with a history of previous abdominal surgeries. Sacrospinous fixation gains level 1 support and would prevent recurrence of the prolapse in the absence of appropriate anterior bony structures and congenitally weakened ligamental support. In this case this is particularly important as there was a reduction in level 2 and 3 support due to the anatomical defects.
Conflicts of interest None. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
References 1. Nelson CP, Dunn RL, Wei JT, Gearhart JP (2005) Surgical repair of bladder exstrophy in the modern era: contemporary practice patterns and the role of hospital case volume. J Urol 174(3):1099–1102 2. Bartelink LR, Feitz WF, Kluivers KB, Withagen MIJ, Vierhout ME (2011) Surgical treatment of uterine prolapse in women with bladder exstrophy: report of two cases with modified Prolift™ procedure. Int Urogynecol J 22(7):889–891 3. Muir TW, Aspera AM, Rackley RR, Walters MD (2004) 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 15(6):436–438 4. Barr S, McLennan M (2008) The anatomic and surgical challenge of treating a patient with stress incontinence, uterovaginal prolapse, and a history of congenital bladder exstrophy. J Pelvic Med Surg 14(4):449 5. Boemers TM, Schimke CM, Ludwikowski B, Ardelean MA (2005) Rotundum psoas hitch: a new method for colpohysteropexy in girls with bladder exstrophy. J Pediatr Urol 1(5):337–341. doi:10.1016/j. jpurol.2005.02.004
CASE REPORT
Bladder exstrophy combined with uterovaginal prolapse and its surgical management: case report and literature review F. Israfil-Bayli & M. Belal & P. Toozs-Hobson
Received: 5 July 2013 / Accepted: 17 November 2013 # The International Urogynecological Association 2013
Abstract We describe a case of a postmenopausal woman with a corrected bladder exstrophy (BE) who presented with a uterovaginal prolapse complicated by calcified fibroids and discuss its surgical management. A 51-year-old woman presented with a uterovaginal prolapse. She had previously had one full term delivery by caesarean section 20 years ago. She had an ileal conduit performed for her BE at birth. A computed tomography scan revealed significant pelvic anomaly and the uterus contained multiple calcified fibroids. Due to the complexity of her condition the surgical management was planned in two stages: initial examination under anaesthetic in conjunction with a consultant urologist and then the definitive planned reconstructive surgery. This case describes the need for careful evaluation and planning of complex surgery and the successful surgical management option for correction of uterovaginal prolapse in patients with BE. A vaginal approach seemed to be a most sensible route to correct the prolapse in view of her previous multiple abdominal surgeries.
Keywords Uterovaginal prolapse . Bladder exstrophy (BE) . POP-Q classification . Sacrospinous fixation
Introduction Bladder exstrophy (BE) is a rare congenital abnormality occurring once in every 10,000–50,000 live births affecting males and females equally [1]. The condition arises from a failure of midline closure resulting in a defect in the tissues and absence of anterior bladder wall. As a result posterior bladder protrudes on the anterior abdominal wall. It is commonly combined with anomaly of the anterior bony pelvis and external genitalia [2]. In females the vagina may be displaced anteriorly and coexists with epispadias. BE is usually corrected surgically straight after birth. Long-term problems in patients with BE include incontinence, recurrent urinary tract infections, reflux, uterine prolapse and adenocarcinoma of bladder and colon.
Case report Electronic supplementary material The online version of this article (doi:10.1007/s00192-013-2285-6) contains supplementary material, which is available to authorized users. F. Israfil-Bayli (*) : P. Toozs-Hobson Department of Urogynaecology and Pelvic Floor Medicine, Birmingham Women’s Hospital NHS Foundation Trust, Mindelsohn Way, Birmingham B15 2TG, UK e-mail: [email protected] F. Israfil-Bayli e-mail: [email protected] F. Israfil-Bayli e-mail: [email protected] M. Belal University Hospital Birmingham, Birmingham, UK
A 51-year-old postmenopausal woman presented to the urogynaecology clinic complaining of “lump in vagina”, discomfort in perianal region and inability to have sexual intercourse. She did not have any bladder or bowel symptoms. She had had an ileal conduit constructed as a result of correction of her BE at birth. The conduit was functioning without any problems. Twenty years previously she had had one full term pregnancy and was delivered via caesarean section. Otherwise medically she was fit and well other than mild arthritis affecting her knees and wrists. Physical examination, including Pelvic Organ Prolapse Quantification (POP-Q) was performed (Aa+2, Ap+2, TVL 6, Ap −1, Bp −1, C+4, D −1, PB 2, GH 3). Tissues were
Int Urogynecol J
Fig. 1 CT scan performed prior to examination under anaesthetic
extremely atrophic and macerated. The whole anatomy of the vulval region was distorted due to absent symphysis pubis and scars from previous surgery for correction of BE. Conservative measures such as physiotherapy and vaginal pessaries had been unsuccessful due to the severity of the prolapse and extreme nature of the distortion to the pelvic anatomy. As a result a decision was made to correct uterovaginal prolapse surgically. Prior to undertaking this a computed tomography (CT) scan was performed to give more information about the patient’s anatomy. The CT confirmed an incompletely formed sacrum and absent coccyx. Also there was an incomplete fusion of some of the posterior sacral elements, particularly on the left side. The pubic rami were incomplete anteriorly with only the lateral aspects present, so no symphysis was seen. Iliac wings and both hip joints were normal. The uterus
also contained multiple (>10 cm) fibromas which were calcified (Fig. 1). In view of the above finding we decided on a two-stage approach. The first step was examination of the patient in theatre together with a consultant urologist with CT images of the pelvis available at hand to identify what could potentially be achieved. The examination under anaesthetic revealed extensive scarring of the lower abdominal area and confirmed the absent pubic bones. The procidentia was reducible but there was thickening of the anterior vaginal wall. POP-Q performed in theatre confirmed findings described above. It was felt that the uterus was contributing to her symptoms and a vaginal hysterectomy approach most appropriate so she was planned for vaginal hysterectomy with possible sacrospinous fixation. The second step involved definitive surgery to correct the uterovaginal prolapse (Fig. 2).
Fig. 2 Intraoperative images. The specimen needed to be delivered in piecemeal to allow volumetric reduction of the uterus so that the fundus could be safely dissected of the adhesions from the previous paediatric reconstructive surgery and caesarean section. Vaginal hysterectomy was followed by sacrospinous fixation. Conventional trocar mesh kits could
not be used and there was inadequate space for a Miya Hook™ because of the anatomy. A Capio™ was used to secure level 1 support to suspend the cystocele due to the lack of ventral support of the pubocervical fascia. Then she had a modified anterior repair and vault was closed. Total vaginal length after surgery was 6 cm with C at −6
Int Urogynecol J
The patient made a good recovery and was reviewed in the outpatient clinic 8 weeks and 8 months respectively following surgery. She did not have any symptoms of prolapse and was happy with the outcome of surgery. POP-Q was performed and at both reviews was the same (Aa −2, Ba −2, C −6, PB 2, GH 3, TVL 6, Ap −2, Bp −2, D absent). We have used the electronic Pelvic Floor Assessment Questionnaire (ePAQ) before and after surgery. Summary of e-PAQ response postoperatively revealed improvement in pain and sensation of the vaginal domain and general improvement in sex life in the sexual domain.
Discussion On reviewing the literature there are several case reports on surgical management of uterovaginal prolapse in patients with BE [3, 4]. However, there are no reports on vaginal hysterectomy where the uterus was significantly enlarged with calcified fibroids to 10 cm in diameter and delivered vaginally, or where sacrospinous fixation has been employed to secure level 1 support. There are several surgical techniques that have been proposed to treat prolapse in this cohort of patients. In this case the surgical approach was complicated by the distorted anatomy including absent symphysis, absent coccyx and partial sacrum, shortened vagina and abnormal muscles. This resulted in gross distortion of the anatomy, with a reduction in the normal spaces with the pelvis utilized in complex reconstructive surgery so that access and space to insert sutures into the sacrospinous ligament safely were dramatically reduced rendering the more commonly used techniques such as a Myer Hook redundant. Reported abdominal procedures involve sacral colpopexy or rotundum psoas hitch [5]. Reported vaginal procedures involve sacrospinous hysteropexy with uterine preservation or insertion of the Prolift mesh [2]. There are certain limitations of pelvic floor surgery in patients with BE due to absence of anterior bony pelvis and distorted fibromuscular support. We decided on the vaginal route because of the history of previous abdominal surgeries and as a result high likelihood of intraoperative organ damage and/or complications to the ileal conduit. There was no intention to perform uterine preserving procedure as the patient was menopausal and the uterus had significant fibromatous
changes. So in order to provide apical support sacrospinous fixation was performed using the Capio® device to compensate for the reduced access from the distortion of the anatomy.
Conclusion This case describes the need for careful evaluation and planning of complex surgery in patients with BE and the successful surgical management corrections of uterovaginal prolapse. The case involves utilization of both relevant surgical specialties and highlights where a modified vaginal approach may be preferable in women with a history of previous abdominal surgeries. Sacrospinous fixation gains level 1 support and would prevent recurrence of the prolapse in the absence of appropriate anterior bony structures and congenitally weakened ligamental support. In this case this is particularly important as there was a reduction in level 2 and 3 support due to the anatomical defects.
Conflicts of interest None. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
References 1. Nelson CP, Dunn RL, Wei JT, Gearhart JP (2005) Surgical repair of bladder exstrophy in the modern era: contemporary practice patterns and the role of hospital case volume. J Urol 174(3):1099–1102 2. Bartelink LR, Feitz WF, Kluivers KB, Withagen MIJ, Vierhout ME (2011) Surgical treatment of uterine prolapse in women with bladder exstrophy: report of two cases with modified Prolift™ procedure. Int Urogynecol J 22(7):889–891 3. Muir TW, Aspera AM, Rackley RR, Walters MD (2004) 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 15(6):436–438 4. Barr S, McLennan M (2008) The anatomic and surgical challenge of treating a patient with stress incontinence, uterovaginal prolapse, and a history of congenital bladder exstrophy. J Pelvic Med Surg 14(4):449 5. Boemers TM, Schimke CM, Ludwikowski B, Ardelean MA (2005) Rotundum psoas hitch: a new method for colpohysteropexy in girls with bladder exstrophy. J Pediatr Urol 1(5):337–341. doi:10.1016/j. jpurol.2005.02.004
