Developmental outcome of children with Robin sequence treated with the current Paris protocol

Area of Science:

• Pediatric Development
• Genetics and Developmental Biology
• Respiratory Medicine

Background:

• Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
• Continuous positive airway pressure (CPAP) is a primary intervention for managing upper airway obstruction in infants with RS.
• Understanding the long-term developmental trajectory of children with RS is crucial for early intervention and support.

Purpose of the Study:

• To assess the developmental outcomes in children with Robin sequence (RS) managed primarily with CPAP.
• To identify risk factors associated with developmental delays in this population.
• To evaluate the impact of RS severity and maternal education on child development.

Main Methods:

• A cohort of children with isolated RS or RS associated with Stickler syndrome, aged 15 months to 6 years, was studied.
• The French version of the Child Development Inventory was utilized to assess developmental quotients (DQ) across eight domains and globally (DQ-global).
• Statistical analysis was performed to identify determinants of developmental delay risk.

Main Results:

• 71% of the 87 children exhibited normal developmental evolution (DQ-global ≥86).
• 29% were at high risk of delay (DQ-global <86), and 3% at very high risk (DQ-global <70).
• Expressive and receptive language DQs were lower than the general population but showed improvement with age; maternal education level was a significant risk factor for delay.

Conclusions:

• The majority of children with Robin sequence managed with CPAP demonstrate typical developmental progression.
• Language development, while initially delayed, shows improvement with growth.
• Lower maternal education levels are associated with an increased risk of developmental delay in children with RS.