Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol

Abstract

Purpose: The aim of the study was to evaluate the largest number of Pierre Robin sequence (PRS) cases to date and its treatment outcome.

Design: This is a retrospective study.

Settings: The study was conducted in a tertiary care university hospital.

Patients: One hundred eighty-eight patients with PRS have been subjected to analysis, defined by the clinical triad of glossoptosis, retro/micrognathia, and cleft or agenesis of the palate, for incidence, risk factors, associated syndromes, other concomitant abnormalities, airway and feeding difficulties, and outcome regarding the neonatal and perinatal surgical and nonsurgical management.

Results: The incidence of PRS was 6.02%. Risk factors were found in 41.5% of mothers with children with PRS. Eleven other syndromes/appearances were associated. Feeding difficulties were seen in 51.8% of the patients. Glossopexy (6.9%) and tracheotomy (2.13%) for airway management were performed very seldom. The suction and drinking plate as well as the surgical treatment protocol may be considered to be the reasons for reduced airway (up to 91.0%), feeding problems (up to 79.9%), and mortality rate (2.1%).

Conclusion: The Pierre Robin sequence, as seen as a heterogeneous group, presents with variation of the cleft palate defects with glossoptosis and concomitant micrognathic mandible. The surgical management and certain prepalatal intervention as nonsurgical management with an innovative treatment strategy were evaluated.