Browse > Article
http://dx.doi.org/10.5653/cerm.2021.05148

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

Seat, Mara (Nova Southeastern University Dr. Kiran C. Patel College of Osteopathic Medicine)
Boxwalla, Munira (Department of Obstetrics and Gynecology, Staten Island University Hospital)
Hough, Arielle (Nova Southeastern University Dr. Kiran C. Patel College of Osteopathic Medicine)
Goodwin, Glenn (Department of Emergency Medicine, Aventura Hospital and Medical Center)
Publication Information
Clinical and Experimental Reproductive Medicine / v.49, no.3, 2022 , pp. 215-218 More about this Journal
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.
Keywords
Cloacal exstrophy; Exstrophy epispadias complex; Exstrophy epispadias sequence; Exstrophy of cloaca; Imperforate anus and spinal defects; Omphalocele;
Citations & Related Records
연도 인용수 순위
  • Reference
1 Gnade CM, Williams HR, Kowalski JT, Andrews JI, Anaya H. Management of pregnancy in patients with exstrophy-epispadias sequence: a case series and literature review. Proc Obstet Gynecol 2018;8:1-9.
2 Phillips TM. Spectrum of cloacal exstrophy. Semin Pediatr Surg 2011;20:113-8.   DOI
3 Thomas JC, Adams MC. Female sexual function and pregnancy after genitourinary reconstruction. J Urol 2009;182:2578-84.   DOI
4 Gezer A, Guralp O, Yesilbas C, Madazli R. Spontaneous pregnancy and birth with corrected cloacal exstrophy. Acta Obstet Gynecol Scand 2011;90:413-4.
5 Naiditch JA, Radhakrishnan J, Chin AC, Cheng E, Yerkes E, Reynolds M. Fate of the uterus in 46XX cloacal exstrophy patients. J Pediatr Surg 2013;48:2043-6.   DOI
6 Deans R, Banks F, Liao LM, Wood D, Woodhouse C, Creighton SM. Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study. Am J Obstet Gynecol 2012;206:496.e1-6.
7 Dukhovny S. ilkins-Haus L. Neural tube defects: overview of prenatal screening, evaluation, and pregnancy management [Internet]. UpToDate; 2022 [cited 2022 Aug 28]. Available from: https://www.uptodate.com/contents/neural-tube-defects-overview-of-prenatal-screening-evaluation-and-pregnancy-management.
8 Catti M, Paccalin C, Rudigoz RC, Mouriquand P. Quality of life for adult women born with bladder and cloacal exstrophy: a longterm follow up. J Pediatr Urol 2006;2:16-22.   DOI
9 Mathews RI, Gan M, Gearhart JP. Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int 2003;91:845-9.   DOI
10 Ziegler MM. Cloacal exstrophy. In: Puri P, Hollwarth M. editors. Pediatric surgery. Berlin, Heidelberg: Springer; 2009. p. 635-42.
11 Dy GW, Willihnganz-Lawson KH, Shnorhavorian M, Delaney SS, Amies Oelschlager AM, Merguerian PA, et al. Successful pregnancy in patients with exstrophy-epispadias complex: a University of Washington experience. J Pediatr Urol 2015;11:213.e1-6.
12 Hyun SJ. Cloacal exstrophy. Neonatal Netw 2006;25:101-15.   DOI
13 Mitrofanoff Support. The Mitrofanoff procedure [Internet]. Wokingham: Mitrofanoff Support; 2022 [cited 2022 Mar 3]. Available from: https://www.mitrofanoffsupport.org.uk/.
14 Stephenson CD, Lockwood CJ. Body stalk anomaly and cloacal exstrophy [Internet]. UpToDate; 2022 [cited 2022 May 17]. Available from: https://www.uptodate.com/contents/body-stalkanomaly-and-cloacal-exstrophy#!.
15 Vlangos CN, Siuniak A, Ackley T, van Bokhoven H, Veltman J, Iyer R, et al. Comprehensive genetic analysis of OEIS complex reveals no evidence for a recurrent microdeletion or duplication. Am J Med Genet A 2011;155A:38-49.