Do patients with Pierre Robin sequence have worse outcomes after cleft palate surgery?

Background: Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often clefting of the soft and hard palate. The purpose of this study was to compare oronasal fistula rates, long-term speech outcomes, and rates of secondary surgery for velopharyngeal insufficiency (VPI) after primary cleft palate repair using the modified Furlow technique between nonsyndromic patients with and without PRS. This study also sought to determine if type of airway management in infancy correlated with speech outcomes in patients with PRS.

Methods: A retrospective review was performed of all nonsyndromic patients with clefts of the palate only who underwent repair between 1981 and 2006 at The Children's Hospital of Philadelphia using the modified Furlow technique. Patient outcomes were evaluated by the rate of postoperative oronasal fistula, speech scores at a minimum of age 5 years using the Pittsburgh Weighted Values for Speech Symptoms Associated with VPI, and the need for secondary pharyngeal surgery for VPI.

Results: Fifty-five patients with PRS and 129 without PRS were included in this study. There was no significant difference in oronasal fistula rate between groups. Speech outcomes were worse in patients with PRS with 52.7% demonstrating a competent velopharyngeal mechanism, and 30.9% a borderline mechanism, compared to 72.1% and 19.4% in the non-PRS group, respectively (P = 0.035). In addition, only 76.3% of patients with PRS had no or mild hypernasality compared to 91.5% of patients without PRS (P = 0.01). The rates of nasal emission, articulation errors associated with VPI, and secondary surgery for VPI did not differ between groups. Of the patients with PRS, 36 were managed in infancy with positioning alone and 15 underwent surgical intervention for airway obstruction, and there were no significant differences in speech scores or rates of secondary surgery for VPI between these subgroups.

Conclusions: Nonsyndromic patients with PRS had worse speech outcomes after modified Furlow cleft palate repair, but no significant differences were seen in the rates of secondary surgery for VPI or postoperative oronasal fistula. In addition, initial airway management in patients with PRS did not correlate with speech outcomes.