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Introduction
Epispadias-exstrophy complex (EEC) is frequently associated with müllerian anomalies. This is a complex medicosurgical problem that requires a specialized team approach from preconception counseling to delivery. This study aims to describe and reflect upon this experience at our institution.
Materials & Methods
A retrospective review was performed over 15 years, evaluating EEC patients who have carried successful pregnancies. A description of their prior reconstructions and comorbidities is included. Also described is their pregnancy course, antenatal and postnatal outcomes including urologic and obstetric complications. Inclusion criteria: patients with EEC with a history of complex genitourinary reconstruction who have had successful pregnancy between 1996-2014, age >=18 years. Exclusion criteria: patients with genitourinary anomalies other than EEC, minors. IRB approval was obtained.
Results
Seven women with EEC had 12 pregnancies resulting in 9 live births; the other 3 resulted in fetal loss or abortion. All were naturally conceived. Complications during the 9 pregnancies included recurrent urinary tract infection in 6, difficulty catheterizing per Mitrofanoff channel in 2, with complete obstruction requiring nephrostomy drainage in one. Seven neonates were delivered by planned cesarean section, one emergently; one was delivered vaginally at an outside institution. There were two preterm deliveries; the remainder carried to term. There were no major surgical complications at the time of each delivery.
An algorithm for treatment is described. This begins with preconception counseling and early pregnancy planning involving the high risk obstetrician and urologist. An understanding of the patient’s reconstructed anatomy is important. Discussions about routine obstetric and gynecologic care, and unique risks of infection, obstruction, and changes in continence are initiated. Patients are counseled on the likelihood of requiring planned cesarean section via classical uterine incision. Whether the patient has urinary tract colonization vs. infection must be established early, treating more conservatively in patients with prior recurrent UTIs or complications. In the second and third trimesters, consequences of an expanding gravid uterus arise, including changes in the ability to self-catheterize through reconstructed channels. Surgical approach for C-section is described, with key utilization of the Alexis O C-section Retractor which provides circumferential atraumatic resection. The delivery is usually a planned cesarean at 37-39 weeks in an operating room with full surgical capacity, with both Materal Fetal Medicine (MFM) and Urology present.
Conclusions
Women with epispadias-exstrophy complex can have full reproductive lives, though at higher risk for spontaneous abortion, as shown by our experience. According to Center for Disease Control data, there was 1 spontaneous abortion per every 6-7 births in the general population in 2008. There was 1 abortion per every 3 births in our cohort. Our patients’ pregnancies were planned when possible and followed closely by dedicated specialists in MFM and urology. Surgical delivery of EEC patients has become less challenging with increased standardization of pediatric reconstructive approaches. The Alexis Retractor has significantly improved exposure during cesarean section. The algorithm described has evolved from the experiences caring for these women through each step of pregnancy, preempting and treating complications, and delivering their babies safely.