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

Nirupan Vipulananthan1, Timothy Cooper2, Manisha Witmans3

  • 1Faculty of Medicine and Dentistry, University of Alberta Hospitals & Stollery Children's Hospital, Edmonton, Alberta, Canada.

Insights

Many children with Pierre Robin sequence/complex (PRS/C) present later than neonates, often with issues other than respiratory failure. Male gender and respiratory failure at presentation predict severe airway obstruction and tracheostomy needs in PRS/C patients.

Area of Science:

  • Pediatric Otolaryngology
  • Craniofacial Anomalies
  • Airway Management

Background:

  • Pierre Robin sequence/complex (PRS/C) is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
  • Early identification and management are crucial for improving outcomes in affected children.
  • Understanding presentation patterns and predictive factors can optimize care.

Purpose of the Study:

  • To document the age and mode of initial aerodigestive presentation of children with PRS/C to otolaryngologists.
  • To identify factors that predict the type of upper airway obstruction and subsequent management needs.

Main Methods:

  • Retrospective cohort study at a tertiary pediatric referral center.
  • Analysis of a surgical database for children diagnosed with PRS/C.
  • Data collected included demographics, presenting complaints, diagnoses, airway obstruction type, airway lesions, cleft palate presence, and interventions; regression analysis was used for prediction.

Main Results:

  • Forty-six children with PRS/C were included, with a mean age at presentation of 20.4 months.
  • Respiratory failure was the primary presentation in 23 patients, followed by sleep-disordered breathing (14) and swallowing dysfunction (9).
  • Later presentation (non-respiratory failure) and overt cleft palate were associated with respiratory failure; male gender and respiratory failure predicted more severe airway obstruction and tracheostomy.

Conclusions:

  • A significant proportion of PRS/C patients present after the neonatal period with non-respiratory failure symptoms.
  • Male gender and presenting with respiratory failure are indicators of more severe upper airway obstruction and a higher likelihood of requiring tracheostomy.
Abstract

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