RESUMEN
Orofacial clefts are birth defects that require a multi-disciplinary approach for repair and ongoing management as there are often concomitant chronic health issues. Orofacial clefts can occur as an isolated finding, in combination with other anomalies, or as part of a genetic syndrome. When occurring as part of a genetic syndrome, the complexity of management increases and has lifelong implications for these individuals, their families, and their health care providers. Understanding factors related to the occurrence of syndromic orofacial clefting is important for birth defect research and for health care needs assessment and planning. Many research groups have addressed these issues by studying different populations and focusing on different questions. This study was a retrospective chart review of children with orofacial clefts cared for at a pediatric tertiary care center in Hawai'i to evaluate the proportion of isolated and syndromic clefts in the unique population of Hawai'i. The prevalence of syndromic and isolated clefts were then correlated with ethnicity and compared to the prevalence in other studies. Our goal was to increase knowledge about orofacial clefting in the population of Hawai'i. The proportion of isolated orofacial clefting in a population of patients with orofacial clefting cared for at a craniofacial clinic is similar to birth defect registry data for the Hawaiian Islands (59% vs 58%). Pacific Islanders in our study and prior study have a lower proportion of isolated clefts, suggesting that there are more craniofacial patients with syndromic and complex needs in this population. Further study is needed to clarify the etiologic factors.