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Evaluation of co\u2010occurring birth defects in children with A\/M could identify patterns indicative of undiagnosed\/unrecognized syndromes. We obtained information on co\u2010occurring birth defects among infants with A\/M for delivery years 1999\u20132014 from the Texas Birth Defects Registry. We calculated observed\u2010to\u2010expected ratios (OER) to identify birth defect combinations that occurred more often than expected by chance. We excluded children diagnosed with genetic or chromosomal syndromes from analyses. Birth defects and syndromes\/associations diagnosed \u22641\u00a0year of age were considered. We identified 1310 infants with non\u2010syndromic A\/M, of whom 38% (<jats:italic>N<\/jats:italic>\u00a0=\u2009492) were diagnosed with co\u2010occurring major defects. Top combinations included: hydrocephalus, ventricular septal defect, and spinal anomalies (OER 58.4); microphthalmia and anomalies of the aorta (OER 55.4); and cleft lip with or without cleft palate and rib or sternum anomalies (OER 32.8). Some combinations observed in our study may represent undiagnosed\/atypical presentations of known A\/M associations or syndromes, or novel syndromes yet to be described in the literature. Careful evaluation of infants with multiple birth defects including A\/M is warranted to identify individuals with potential genetic or chromosomal syndromes.<\/jats:p>","DOI":"10.1002\/ajmg.a.63081","type":"journal-article","created":{"date-parts":[[2022,12,21]],"date-time":"2022-12-21T10:04:18Z","timestamp":1671617058000},"page":"805-812","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Patterns of co\u2010occurring birth defects in children with anotia and microtia"],"prefix":"10.1002","volume":"191","author":[{"given":"Jeremy M.","family":"Schraw","sequence":"first","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6406-4247","authenticated-orcid":false,"given":"Renata H.","family":"Benjamin","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, Human Genetics &amp; Environmental Sciences UTHealth School of Public Health  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles J.","family":"Shumate","sequence":"additional","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8827-1881","authenticated-orcid":false,"given":"Mark A.","family":"Canfield","sequence":"additional","affiliation":[{"name":"Birth Defects Epidemiology and Surveillance Branch Texas Department of State Health Services  Austin Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1460-5169","authenticated-orcid":false,"given":"Daryl A.","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Molecular and Human Genetics Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott D.","family":"McLean","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hope","family":"Northrup","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth)  Houston Texas USA"},{"name":"Children's Memorial Hermann Hospital  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9025-3276","authenticated-orcid":false,"given":"Angela E.","family":"Scheuerle","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Texas Southwestern Medical Center  Dallas Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2148-7490","authenticated-orcid":false,"given":"Christian P.","family":"Schaaf","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph W.","family":"Ray","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics and Metabolism University of Texas Medical Branch  Galveston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Han","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, Human Genetics &amp; Environmental Sciences UTHealth School of Public Health  Houston Texas USA"},{"name":"Center for Precision Health, School of Biomedical Informatics University of Texas Health Science Center at Houston  Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5874-4155","authenticated-orcid":false,"given":"A. 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