Pregnancy After Repair Of Bladder Exstrophy and Epispadias Complicated by Uterine Prolapse

Author Information

Sikarwar R*, Hatkar PA**, Satia MN ***.
(* Third Year Resident, ** Associate Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)

Abstract

We report the successful obstetric and perinatal management of a patient with third degree uterovaginal descent with childhood surgery for epispadias with bladder exstrophy and urinary incontinence. The patient had presented to our outpatient department (OPD) for primary infertility and third degree uterovaginal prolapse. She conceived on follicular monitoring and ovulation induction. Pregnancy was monitored throughout her antenatal period. Elective lower segment cesarean section (LSCS) was done at term in view of  history of Young Dee’s repair for epispadias with Kohen’s ureteric reimplantation with clitoroplasty. Despite difficulties like dense adhesions between bladder and lower uterine segment, she had an uneventful post-operative course with complete recovery. 

Introduction

Congenital bladder exstrophy is a very rare condition. 1 in 125000 to 250000 females are affected by the same. Bladder exstrophy comprises of absence of anterior abdominal wall leading to failed fusion of pubic symphysis, defective pelvic floor and exposed urethral orifice, i.e. epispadias. It may also be associated with failed fusion of clitoris. As bladder neck is almost always affected, it usually causes urinary incontinence specially during coughing and straining.  There are significant differences in the pelvic floor anatomy between normal subjects and those with bladder exstrophy. There are also other differences in the bony pelvic structure, connective tissue supports and vaginal axis and length. These may be responsible for the commonly associated problem of pelvic organ prolapse in patients with bladder exstrophy. Due to its rarity, there is a paucity of literature on obstetric management of women with repaired bladder exstrophy. Pregnancy may have complications like preterm labor and recurrent urinary tract infections. Although uterine prolapse is a common gynecologic condition, pregnancy with prolapsed uterus is uncommon.[1] We report a rare case of pregnancy with third degree uterovaginal descent, in a previously operated case of bladder exstrophy.

Case Report

A 30 year old, married since 3 years, presented with third degree uterovaginal descent which developed over 3-4 years. At 3 years of age, she had complaint of urinary incontinence; on examination she had small bladder capacity with low ureteric opening with short urethra, along with pubic symphysis which was separate, suggestive of epispadias with bladder exstrophy. She underwent surgery for epispadias and urinary incontinence in the form of Young Dee’s repair for epispadias with Kohen’s ureteric reimplantation with clitoroplasty. After the surgery, she continued to follow up in outpatient clinic (OPD) and her complaints of urinary incontinence and decreased bladder capacity improved over a period of time.
The patient presented to our gynecology OPD at 29 years of age with complaints of third degree uterovaginal descent with primary infertility. On per abdominal examination there was a puckered midline vertical scar extending from mons pubis to clitoris, and on per speculum examination third degree uterovaginal descent was seen with no cystocele or rectocele. On per vaginal examination the uterus was retroverted and normal sized. A clinical diagnosis of nulliparous prolapse was made and patient was advised conservative line of management for prolapse by placing a vaginal pessary, as she did not wish to have operative treatment. She conceived with ovulation induction. She registered in antenatal OPD and had regular follow up and an uneventful pregnancy. She was hospitalised at 37 weeks of gestation for elective lower segment cesarean section, the indication being history of prior bladder surgery and bilateral ureteral implantation, along with a urologist as a standby. Uterovesical fold was densely adherent to the lower uterine segment and was advanced hence, incision was directly taken slightly higher on the lower segment of uterus without dissecting the UV fold, 2 cm above the upper extent of the bladder. She delivered a male child of 3.198 kg. Postpartum period was uneventful, she had no bladder or bowel complaints. She was counseled to follow up after six weeks for management of uterovaginal prolapse.

Discussion

Bladder exstrophy is usually diagnosed at birth or soon afterwards. Symptoms become more evident once toilet training is done, and the patient may present with complaints of urinary incontinence, urinary tract infections and symptoms due to reflux nephropathy. In most of the cases, the symptoms resolve with surgery. Usually, fertility of the female is not affected as reproductive organs are normally developed. Some babies with bladder exstrophy may also have  other congenital anomalies of genitourinary tract. Some patients may have congenital prolapse or a tendency to develop the same in future because pelvic floor anatomy and structure of the bony pelvis may be altered in patients with bladder exstrophy, along with alterations in the  connective tissue support, vaginal axis and length.[2] These could be the causes of nulliparous prolapse in our patient. Various surgeries are available for treatment as definitive management. Other contributing factors are situations which lead to increased intraabdominal pressure like straining, constipation, heavy weight lifting and chronic obstructive airway disease. Preexisting prolapse is related to complications like infertility, spontaneous abortions, and preterm labor. Due to venous obstruction and stasis, cervical edema occurs which may lead to mechanical trauma and cervical ulceration. Various studies report increased chances of urinary retention and thereby urinary tract infections in cases of pregnancy with uterine prolapse.[3] If such patients go in labor there are more chances of cervical dystocia. Uterine rupture in lower segment has also been reported.[4] Pregnancy with prolapsed uterus is rare. Management of pregnancy with uterine prolapse with history of bladder exstrophy repair though not different from any other patient with prolapse, needs to be individualized. Obstetricians must keep in mind the above stated complications while deciding the right approach for these patients. Bed rest with slight Trendelenburg position is advisable for reposition of prolapsed cervix and reduction of cervical edema. Perineal hygiene should be advised and in case of cervical ulcerations, local antibiotic application  can be recommended. After reduction of cervical edema, pessary may be used during first trimester to keep the uterus reposited in its anatomical position.[3] Induction of labor with dinoprostone gel or oxytocin drip should be avoided.[5] Elective cesarean section can be planned in cases of pregnancy with prolapsed edematous and elongated cervix. Individualized approach and careful monitoring is the key to manage such patients.

References
  1. Rose CH, Rowe TF, Cox SM, Malinak LR. Uterine prolapse associated with bladder exstrophy: surgical management and subsequent pregnancy. J Matern Fetal Med. 2000; 9(2):150-2.
  2. Van Dongen L. The anatomy of genital prolapse. S Afr Med J. 1981; 609(9):357-9.
  3. Piver MS, Spezia J. Uterine prolapse during pregnancy. Obstet Gynecol. 1968; 329(6):765-9.
  4. Daskalakis G, Lymberopoulos E, Anastasakis E, Kalmantis K, Athanasaki A, Manoli A et al. Uterine prolapse complicating pregnancy. Arch Gynecol Obstet. 2007; 276(4):391-2.
  5. Tukur J, Omale AO, Abdullahi H, Datti Z. Uterine prolapse following fundal pressure in the first stage of labour: a case report. Ann Afr Med. 2007; 6(4):194-6.
Citation

Sikarwar R, Hatkar PA, Satia MN. Pregnancy After Repair Of Bladder Exstrophy and Epispadias Complicated by Uterine Prolapse. JPGO 2017. Volume 4 No.7. Available from: http://www.jpgo.org/2017/07/pregnancy-after-repair-of-bladder.html