Abstract
Robin sequence (RS) is a rare condition associating retrognathia and glossoptosis causing upper airway obstruction. For half of the teams, prone positioning (PP) is the first-line treatment considering the benefit experienced on breathing, sleep, and neurodevelopmental outcome of infants with RS. However, this strategy is debated because of the risk of sudden infant death syndrome (SIDS) associated with PP. This study analyzed the occurrence of SIDS in children < 1 year old with RS during the last 30 years in France to clarify the benefit/risk balance of PP. We gathered data from the French national registry for SIDS. We also collected responses to a survey on SIDS sent to all French national RS reference centers for rare diseases. Finally, we collected data from the national epidemiologic database for causes of deaths (CépiDc). During the last 10 years, the French registry for SIDS documented 1 case of SIDS in a child with RS who died while awake in the back position. Among about 3000 children followed during the last 30 years, the survey to RS centers identified 2 cases of SIDS. Regarding CépiDc data, the proportion of SIDS cases among all causes of death in the RS population was lower than in the general population. CONCLUSION: In this preliminary work, SIDS was not more common in the RS population than in the general population in France, even if most patients are treated with PP. Taking into account the benefits of this therapy, PP may be proposed in selected patients, with strict cautions. WHAT IS KNOWN: • Prone sleeping is a well-known risk factor of SIDS. • In approximately 50% of centers worldwide, prone positioning is used as the first-line treatment of upper airway obstruction in children with Robin sequence. WHAT IS NEW: • No child with Robin sequence died from SIDS while in the prone position in the last 10 years in France (SIDS French registry). • The occurrence of SIDS among all causes of death for children with Robin sequence is not higher than in the general population in France even if prone positioning is the first-line treatment.