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Register Based Study of Bladder Exstrophy-Epispadias Complex: Prevalence, Associated Anomalies, Prenatal Diagnosis and Survival

Lookup NU author(s): Don Jayamanne, Mary Bythell, Dr Martin Ward Platt, Professor Judith Rankin


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Purpose: We describe the prevalence, associated anomalies, prenatal diagnosis and survival of patients with bladder exstrophy-epispadias complex. Materials and Methods: Data were extracted from the Northern Congenital Abnormality Survey for patients delivered during 1985 to 2008. This survey collects data on congenital anomalies in fetuses, stillbirths and live-born infants of mothers residing in Northern England (Northumberland, North Cumbria, Tyne and Wear Durham, Darlington and Teesside). Results: A total of 43 cases were identified from 824,368 registered births for a total prevalence of 5.22 per 100,000 (95% CI 3.77-7.03). Excluding 1 twin with cloacal exstrophy, 42 cases occurred in singleton pregnancies. A total of 29 cases (69%) were isolated and 13 (31%) were associated with other anomalies, of which 11 (26%) were other structural and 2 (5%) were chromosomal. Male-to-female ratio was 2.2: 1 for all singleton cases and 1.4: 1 for isolated cases. Total prevalence of bladder exstrophy-epispadias complex singleton cases was 5.10 per 100,000 registered births (95% CI 3.67-6.89) and overall live birth prevalence was 4.63 per 100,000 live births (95% CI 3.28-6.36). Total prevalence of isolated cases of bladder exstrophy-epispadias complex was 3.52 per 100,000 births (95% CI 2.365.05) and live birth prevalence was 3.29 per 100,000 (95% CI 2.17-4.79). Accuracy of prenatal diagnosis was low, with 4 cases (10%) being detected prenatally by routine ultrasound (bladder exstrophy in 3, cloacal exstrophy in 1). Overall survival of all infants at 1 year was 95%. Conclusions: This population based study demonstrates a prevalence rate similar to other studies, a low prenatal diagnosis rate and high survival.

Publication metadata

Author(s): Jayachandran D, Bythell M, Platt MW, Rankin J

Publication type: Article

Publication status: Published

Journal: Journal of Urology

Year: 2011

Volume: 186

Issue: 5

Pages: 2056-2060

Print publication date: 01/11/2011

ISSN (print): 0022-5347

ISSN (electronic): 1527-3792

Publisher: Elsevier Inc.


DOI: 10.1016/j.juro.2011.07.022


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