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