Elsevier

Journal of Pediatric Urology

Volume 11, Issue 4, August 2015, Pages 213.e1-213.e6
Journal of Pediatric Urology

Successful pregnancy in patients with exstrophy–epispadias complex: A University of Washington experience

https://doi.org/10.1016/j.jpurol.2015.04.019Get rights and content

Summary

Introduction

With advances in genitourinary reconstructive surgery, women with exstrophy–epispadias complex (EEC) have improved health and quality of life, and may reach reproductive age and consider pregnancy. Despite literature suggesting impaired fertility and higher risk with pregnancy, childbirth is possible. Medical comorbidities, including müllerian anomalies, contribute to increased risk of obstetric and urologic complications during pregnancy.

Objectives

We reviewed our experience with EEC patients who achieved pregnancy to investigate (1) urological characteristics of women who achieved pregnancy; (2) pregnancy management, complications, and delivery; and (3) neonatal outcomes. We developed recommendations for managing pregnancy in women with EEC.

Study design/Results

This was a retrospective chart review of 36 female patients with EEC seen at our institution between 1996 and 2013. Female patients less than 18 years, and patients who did not have documented pregnancy were excluded. This resulted in a total of 12 patients with 22 pregnancies. All women with successful pregnancy had bladder exstrophy. The majority had undergone prior bladder augmentation (75%) and were on self-catheterization programs (92%). Thirty-six percent had symptomatic urinary tract infections (UTIs) during pregnancy. Five women had more than one pregnancy. There were four terminations of pregnancy. Of 18 desired pregnancies, there were four spontaneous abortions (SABs) (22%) and 16 live births (78%). The cesarean delivery (CD) rate was 100% (14/14), of which the majority were vertical (classical) uterine incisions with a paramedian skin incision. With the exception of one patient, there were no CD surgical complications. The mean gestational age at delivery was 36 weeks (Range 25 4/7 to 39 4/7 weeks) among eight pregnancies with known gestational age. There were no stillbirths, one neonatal death and no birth defects.

Discussion

Women with EEC can have successful pregnancies, though at increased risk for preterm delivery and SABs. In our cohort, the rate of SAB is similar to that described in prior studies. Symptomatic UTIs likely due to self-catheterization were common. Cesarean delivery using a paramedian skin incision and classical uterine incision were not associated with major complications in this cohort. Limitations include reliance on retrospective data and small sample size. The strength of this study is the longitudinal detailed management of pregnancies in EEC women by a single team over time. A multidisciplinary approach to providing a continuum of care from pediatrics through adolescence to adulthood optimizes successful transitions, reproductive health, and successful pregnancies. Based on our experience, an algorithm providing guidance for pregnancy management was developed.

Table. Pregnancy resulting in live birth versus spontaneous abortion or termination.

Outcomes of 22 pregnancies in 12 EEC patients
OutcomeTotal pregnancies (n = 22)Bladder exstrophy pregnancies (n = 20)Cloacal exstrophy pregnancies (n = 2)
Total pregnancies22 (100%)202
Spontaneous abortion <24 weeks4 (18%)3 (15%)1 (50%)
Terminations4 (18%)3 (15%)1 (50%)
Live births14 (64%)14 (70%)0

Introduction

Bladder exstrophy (BE) and cloacal exstrophy (CE) occur in approximately 1/10,000 to 50,000 and 1/130,000 live births, respectively [1], [2], [3]. Advances in reconstructive surgery have led to better preservation of renal function and improved urinary continence. As patients progress from adolescence to adulthood, sexuality and reproduction become important factors in quality of life. Some women with EEC report poor body image and self-esteem, possibly related to genital cosmesis and scars, embarrassment about stomas, and dyspareunia due to shortened vaginas and displaced cervices [4], [5]. Despite these issues, half of adults with EEC report sexual activity [4].

Müllerian anomalies occur more frequently in patients with EEC and may contribute to difficulty maintaining pregnancy. In BE, the uterus, fallopian tubes, and ovaries are generally thought to be unaffected, although in the largest study of pregnancy outcomes in 19 women with BE and pregnancy, 12% had müllerian duplication [6]. In CE, most female patients have variation in müllerian anatomy. This, in addition to medical comorbidities and adhesions related to prior operations, has resulted in only a small percentage of CE patients reporting pregnancy [4].

The first successful pregnancy in a patient with BE was described in 1724, followed by several reported series of patients with EEC and pregnancy [7], [8], [9], [10], [11], [12]. In the largest series to date, Deans and colleagues [6] found a mean gestational at delivery of 36 weeks and a spontaneous abortion rate (SAB) of 35% in 57 pregnancies among 19 women. All studies had a higher SAB rate than the 14–17% in the general population [13]. Mathews and colleagues [12] recommended a multidisciplinary surgical team and cesarean delivery (CD) to protect the reconstructed anatomy and minimize complications.

We conducted a review of pregnancies in women with EEC at our institution to investigate (1) urological characteristics of women who achieved pregnancy; (2) pregnancy management, complications, and delivery; and (3) neonatal outcomes. As our urology and maternal fetal medicine (MFM) providers were consistent throughout this period, we explored the evolution of our management practices over this period. We developed recommendations for a multidisciplinary continuum of care approach to women with EEC desiring pregnancy.

Section snippets

Methods

This was a single institution retrospective study. Approval was granted by the Institutional Review Board.

Inclusion criteria included female patients with the primary diagnosis of EEC seen at our institution between 1996 and 2013, age >18 at time of evaluation, and self-reported history of pregnancy. Exclusion criteria included primary diagnosis other than EEC, no self-reported history of pregnancy, and age <18 at time of evaluation. Women in this study were identified through a three-step

Results

Thirty-six women with EEC were identified. Twenty-four patients were excluded because there was no self-reported history of pregnancy or children (n = 21), or report of child rearing but no confirmation of biologic parenthood (n = 2). One patient was 46XY cloacal exstrophy born without a uterus, raised as female. Of the 12 women who achieved pregnancy, 10 had a primary diagnosis of classic BE (83%), and two had CE (17%). The majority of women in the study were white (10/12, 83%). Of these 12

Discussion

The main limitation of this study is the small number of women, the use of retrospective data, and incomplete information about pregnancies and deliveries performed at other institutions. The strength of our experience lies in the multidisciplinary approach with consistent providers over 17 years. This allowed for evolution and development of a standardized program of pregnancy preparation and management for women with EEC. This study provides an in-depth description of a center's longitudinal

Conclusion

As young women with EEC progress through adolescence, conversations about gynecologic health, sexuality, and reproduction are an essential part of transitional care. This is an ideal time for introduction to a transition team with a close working relationship: an adult reconstructive urologist, an adolescent gynecologist, and an MFM specialist when considering pregnancy. Preconception counseling may begin here, providing guidance for effective contraception until ready for a desired pregnancy,

Conflict of interest

None.

Funding

None.

Ethics approval

Not required.

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