Clin Exp Reprod Med.  2022 Sep;49(3):215-218. 10.5653/cerm.2021.05148.

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

• Seat M
• Boxwalla M
• Hough A
• Goodwin G

• KMID: 2540232
• DOI: http://doi.org/10.5653/cerm.2021.05148

Abstract

Herein, we report an exceptionally rare case of a 25-year-old woman with cloacal exstrophy/omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) syndrome achieving a viable pregnancy despite many gastrointestinal and genitourinary malformations and multiple respective corrective operations. The patient was born with two vaginas, two uteruses, four ovaries, an imperforate anus, a large omphalocele including bowel and bladder exstrophy, and diaphysis of the pubic rami. This patient is the only documented OEIS patient not to have tethered spinal cord as an anomaly, perhaps contributing to her successful pregnancy. After experiencing preeclampsia with severe features at 35 weeks, the baby was born via cesarean section.

Keyword

Omphalocele; Exstrophy of cloaca; Imperforate anus and spinal defects (OEIS), Cloacal exstrophy, Exstrophy epispadias sequence, Exstrophy epispadias complex