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Cloacal Exstrophy

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Atlas of Genetic Diagnosis and Counseling

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Abstract

Cloacal exstrophy is a rare congenital malformation resulting in exstrophy of the urinary, intestinal, and genital systems and is associated with anomalies of other organ systems. The term OEIS complex (omphalocele, exstrophy of the bladder, imperforate anus, and spinal defects) is used to describe the spectrum of malformations in cloacal exstrophy (Carey and Greenbaum 1978; Kaya et al. 2000). The incidence of cloacal exstrophy is estimated to be 1/200,000–1/400,000 live births (Carey et al. 1978; Hurwitz et al. 1987; Dick et al. 2001), although the true incidence may be as high as 1 in 10,000–50,000, taking into account the lack of diagnosis in stillborn infants (Keppler-Noreuil 2001).

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Authors and Affiliations

  1. Medical Genetics, Shriners Hospitals for Children, Perinatal and Clinical Genetics, Department of Pediatrics, LSU Health Sciences Center, Shreveport, LA, USA

    Harold Chen

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  1. Harold Chen
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Chen, H. (2017). Cloacal Exstrophy. In: Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2401-1_45

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  • DOI: https://doi.org/10.1007/978-1-4939-2401-1_45

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-2400-4

  • Online ISBN: 978-1-4939-2401-1

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