Management of pregnancy in patients with exstrophy-epispadias sequence: a case series and literature review

Abstract

Exstrophy

A case of congenital cloacal exstrophy/omphalocele-exstrophy-imperforate anus-spinal defects syndrome and a successful pregnancy

“…At 35 weeks, the patient developed preeclampsia with proteinuria, necessitating delivery via primary cesarean section with ovarian cystectomy and/or possible salpingo-oophorectomy. Given her extensive adhesions from past corrective procedures and because of the large ovarian cyst requiring excision, cesarean section was performed via a midline vertical abdominal incision with a classical uterine incision [1,6].…”

“…While OEIS, exstrophy epispadias complex (EEC), EES, and cloacal exstrophy are often used interchangeably, the OEIS acronym specifically refers to omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects. OEIS is a very rare congenital defect resulting in an array of pathological phenotypes, with the most common being bladder and colon exstrophy, leading to a myriad of anomalies affecting multiple organ systems (Figure 1) [1,2]. Duplication of multiple sex organs is an additional phenotypic variant some-times seen in OEIS patients.…”

“…Duplication of multiple sex organs is an additional phenotypic variant some-times seen in OEIS patients. OEIS is estimated to affect 1 in 200,000 to 400,000 live births [1,3]. EEC refers to epispadias, classic bladder exstrophy, and cloacal exstrophy complex, with cloacal exstrophy being the most severe of the three abnormalities.…”

“…Generally, early surgical intervention is preferred due to better outcomes relating to urinary continence, infection control, physical appearance, self-esteem, sexual function, and fertility [1]. Despite these better outcomes, most OEIS patients are rarely able to achieve a viable pregnancy because of their severe malformations.…”

“…However, if they do achieve pregnancy, many risks are imposed [4]. The relatively fragile, reconstructed sexual organs typically cannot withstand the birthing process, and these patients also usually require additional postpartum operations [1]. These factors compel extensive obstetric and surgical evaluations throughout pregnancy, often warranting caution when an OEIS patient is considering conception.…”