Epidemiology and Incidence of Pierre Robin Sequence in the Maltese Islands: A Population-Based Study.

Abstract

Background: Pierre Robin Sequence (PRS) is a congenital condition characterized by a triad of micrognathia, glossoptosis, and cleft palate. This study aims to investigate the incidence and epidemiology of PRS in the Maltese population, providing valuable insights into its prevalence, clinical characteristics, and surgical management.

Method: A population-based retrospective analysis was conducted utilizing data from the years 2000 to 2019. Aggregate data were collected from the Maltese Public Health Department, capturing registered PRS cases in the Maltese population. Incidence rates were calculated, and demographic factors, gestational distribution, and associations with cleft palate were evaluated.

Results: The incidence of PRS in the Maltese population was found to be 12.5 per 100,000 births, higher than global estimates. Among the registered cases, 63.6% were male. Gestational distribution analysis revealed a concentration of PRS cases born at 39 weeks. Furthermore, 90.9% of PRS cases were associated with cleft palate.

Conclusion: This study highlights a higher prevalence of PRS in the Maltese population compared with global estimates, indicating a unique epidemiological profile specific to the Maltese Islands. The findings provide valuable insights for plastic surgeons and health care professionals involved in the management of PRS patients. Further research is needed to explore the underlying factors contributing to this increased prevalence and to optimize treatment strategies for PRS patients in the Maltese Islands.