Hospitalizations from Birth to 28 Years in a Population Cohort of Individuals Born with Five Rare Craniofacial Anomalies in Western Australia

Abstract

To describe trends, age-specific patterns, and factors influencing hospitalizations for five rare craniofacial anomalies (CFA). Data on livebirths (1983-2010; n=721,019) including rare CFA (craniofacial microsomia, mandibulofacial dysostosis, Pierre Robin sequence, Van der Woude syndrome, and fronto-nasal dysplasia), episodes of death, and demographic and perinatal factors were identified from the Western Australian (WA) Register of Developmental Anomalies, Death Registrations and Midwives Notification System. Information on incident craniofacial and non-craniofacial related admissions, length of hospital stay, and intensive care and emergency related admissions were identified using principal diagnosis and procedural codes were extracted from the Hospital Morbidity Data Collection and linked to other data sources. Associations of hospitalizations by age groups as well as demographic and perinatal factors were expressed as incidence rate ratio (IRR). The incident hospitalizations were three times as high for rare CFA (IRR 3.22 - 3.72) throughout childhood into adolescence than those without. Children with rare CFA had three to four times as many potentially preventable hospitalizations until 18 years of age than those without. Specifically, respiratory infections (IRR 2.13-2.35), ear infections (IRR 7.92 - 26.28), and oral health-related conditions contributed for most non-craniofacial admissions until the adolescence period. Higher incidence of non-craniofacial related hospitalizations was observed among Indigenous children, births with intrauterine growth restrictions and families with high socioeconomic disadvantage. Throughout childhood, individuals with rare CFA had higher hospital service use, specifically for potentially preventable conditions, than those without. These population level findings can inform new preventive strategies and early disease management targeted towards reducing preventable hospitalizations.