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Epidemiology of Partial Urorectal Septum Malformation Sequence (or 'persistent cloaca'): a population-based study in seven regions of England and Wales, 1985-2010

Lookup NU author(s): Peter Tennant, Dr Svetlana Glinyanaya, Professor Judith Rankin

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Abstract

Background: Partial urorectal septum malformation (pURSM) sequence (or 'persistent cloaca') is a rare congenital anomaly characterised by a joining of the urethral, anal, and genital openings into a single common channel. This study describes the epidemiology of pURSM sequence in England and Wales including prevalence, additional anomalies, and pregnancy outcomes. Methods: All cases of pURSM sequence prospectively notified to seven congenital anomaly registers in England and Wales during 1985-2010, whether delivered as live births, spontaneous fetal deaths (≥20 weeks’ gestation), or elective terminations of pregnancy for fetal anomaly (TOPFA, any gestation), formed this population-based cohort. The risks of spontaneous fetal and infant death were examined by Kaplan-Meier analysis. Differences in prevalence over time, and between regions, were examined by multilevel Poisson regression. Results: 117 cases were recorded among 4,251,241 total births. Six (5%) pregnancies resulted in spontaneous fetal deaths, 53 (45%) in TOPFA, and 58 (50%) in live births. The prevalence was 2.8 (95% CI: 2.3-3.4) per 100,000 total births, increasing significantly over time (p=0.002) and differing significantly between regions (p=0.005). 77 cases (66%) had at least one additional major congenital anomaly outside the perineum, including 67 (57%) renal, 29 (25%) musculoskeletal, 26 (23%) digestive system, and 24 (21%) cardiovascular anomalies. The risks of spontaneous fetal and infant death were estimated as 8.9% (95% CI: 4.1-18.8) and 26.3% (95% CI: 15.1-43.4) respectively. Conclusion: This is the largest study of the epidemiology of pURSM sequence. The information will be valuable for families and health professionals whenever a case of pURSM sequence is diagnosed.


Publication metadata

Author(s): Tennant PWG, Glinianaia SV, Wellesley D, Draper ES, Kurinczuk JJ, Tonks AM, Tucker DF, Wreyford B, Rankin J

Publication type: Article

Publication status: Published

Journal: Archives of Disease in Childhood: Fetal & Neonatal Edition

Year: 2014

Volume: 99

Issue: 5

Pages: E413-E418

Print publication date: 01/09/2014

Online publication date: 28/05/2014

Acceptance date: 29/04/2014

ISSN (print): 1359-2998

ISSN (electronic): 1743-0593

Publisher: BMJ Publishing Group

URL: http://dx.doi.org/10.1136/archdischild-2014-306027

DOI: 10.1136/archdischild-2014-306027


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