Primary aerodigestive presentations of Pierre Robin sequence/complex and predictive factors of airway type and management.

Objective: To document the mode and age of primary aerodigestive presentation of Pierre Robin sequence/complex (PRS/C) children to the otolaryngologist and to explore predictive factors of upper airway type and management.

Methods: This is a retrospective cohort study conducted in a tertiary pediatric referral center. A prospective surgical database was searched for children who were diagnosed with PRS/C. Demographics, presenting complaint, secondary diagnoses, type of upper airway obstruction, secondary airway lesions, presence of cleft palate, and airway interventions were collected. Multiple linear regression analysis was performed to predict upper airway obstruction type and intervention.

Results: Seventy-seven potentially eligible patients were identified. Forty-six were included (20 females). Mean age at presentation was 20.4±36.9 months (range 1-191.25 months). Twenty-three primarily presented with respiratory failure, 14 with sleep disordered breathing, and nine with swallowing dysfunction. Children with presentations other than respiratory failure were older (p=0.004). Nineteen were syndromic. Overt cleft palate was more common in those presenting with respiratory failure (p=0.01). The type of airway obstruction encountered and use of tracheostomy were positively predicted by the primary presenting feature of respiratory failure (p<0.05) and male gender (p<0.05).

Conclusion: A substantial number of PRS/C patients present later than the neonatal period with presentations other than respiratory failure. Both male gender and presentation with respiratory failure predicted a more severe airway obstruction type and the need for trachesotomy.