OEIS complex: Prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program

OEIS is the acronym of a malformations complex association including omphalocele, exstrophy of bladder or cloaca, imperforate anus, and spinal defects. It has a very low prevalence, ranging from 1/82,000 to 1/200,000 live births (LB). The etiology of OEIS is unknown. Virtually all cases are sporadic...

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Bibliographic Details
Published in:	Birth defects research Vol. 111; no. 11; pp. 666 - 671
Main Authors:	Arteaga‐Vázquez, Jazmín, Luna‐Muñoz, Leonora, Morales‐Suárez, Juan José, Mutchinick, Osvaldo M.
Format:	Journal Article
Language:	English
Published:	Hoboken, USA John Wiley & Sons, Inc 01-07-2019 Wiley Subscription Services, Inc
Subjects:	Adult Anus Anus, Imperforate - complications Anus, Imperforate - epidemiology Anus, Imperforate - mortality Birth defects Bladder bladder esxtrophy Blastogenesis Case-Control Studies cloacal Congenital defects Defects Epidemiology Etiology Female Hernia, Umbilical - complications Hernia, Umbilical - epidemiology Hernia, Umbilical - mortality Humans Infant, Newborn Infections Influenza Male Mexico - epidemiology OEIS complex Phenotypes Pregnancy Prevalence Risk analysis Risk factors Scoliosis - complications Scoliosis - epidemiology Scoliosis - mortality Statistical analysis Twins Urogenital Abnormalities - complications Urogenital Abnormalities - epidemiology Urogenital Abnormalities - mortality Zygosity Mexico epidemiology OEIS complex cloacal bladder esxtrophy
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Summary:	OEIS is the acronym of a malformations complex association including omphalocele, exstrophy of bladder or cloaca, imperforate anus, and spinal defects. It has a very low prevalence, ranging from 1/82,000 to 1/200,000 live births (LB). The etiology of OEIS is unknown. Virtually all cases are sporadic, and specific associated risk factors uncertain. Objectives This study aimed to determine the prevalence, clinical spectrum, possible early pregnancy exposures, and demographic characteristics as potentially associated risk factors in a sample of Mexican cases. Methods We conducted a multihospital based case–control study on 12 cases with the OEIS complex identified in 1,195,020 LB born from January 1978 to December 2015. All comparisons performed were matching 1:3 the relation of cases and controls, respectively, considering the p‐value of ≤.05 as statistically significant. Results The prevalence of OEIS was 1.004/100,000 (1/99,585) LB. The frequency of bladder/cloacal exstrophy was 75 and 25%, respectively, omphalocele was 83.3%, and imperforate anus and spinal defects, 75.0% each. Two pairs of twins discordant for the defect exhibited the severest OEIS phenotype. Except for the higher frequency of maternal first pregnancy trimester influenza infection, early perinatal mortality and a twining trend association, none other variable differed significantly. Discussion The prevalence of OEIS in our sample is within the highest reported worldwide. First‐trimester pregnancy maternal influenza infection and twining emerge as associated risk factors for OEIS. Although twin zygosity was not defined, the observed severest phenotypes in twins endorse the hypothesis that OEIS and monozygotic twinning are features of disturbances on early blastogenesis.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	2472-1727 2472-1727
DOI:	10.1002/bdr2.1512