Current management of Pierre Robin syndrome

Abstract

Introduction: Airway obstruction in children with Pierre Robin syndrome (PRS)could be an important determinant of the prognosis and quality of life of the child.

Objective: to analyze mandibular distraction as an early management in children with PRS and airway obstruction.

Method: PRS patients treated between January 1998 and March 2003 at the Luis Calvo Mackenna Hospital were included. Clinical evaluation, lateral cephalograms, nasoendoscopy, oxygen saturation and polysomnograms were used as parameters in the decision to perform mandibular distraction. Long term follow up included evaluation of complications, nutritional evolution, facial growth and development.

Results: 23/31 PRS patients underwent mandibular distraction, relieving airway obstruction in all the cases. 2 cases underwent tracheotomy at birth that were removed during the process. 2 patients had pulmonary hypertension and reversed clinical signs after distraction. Their weight charts improved significantly after the operation.

Conclusion: Mandibular distraction is a successful method for young patients with PRS to relieve airway onstruction, improve feeding and avoid tracheotomy or early decannulation in previously tracheotomized patients.