The occurrence of velopharyngeal insufficiency in Pierre Robin Sequence patients

Children born with Pierre-Robin Sequence (PRS) have cleft palate, micrognathia, and macroglossia. After the repair of the cleft palate, velopharyngeal insufficiency (VPI) can occur in a subset of patients. We hypothesize that the need for the surgical correction of VPI in PRS children is no different than cleft palate only (CPO) patients.

A retrospective study of 21 children with non-syndromic PRS who were matched to 42 non-syndromic, CPO controls for age and sex. We reviewed incidence of VPI, the need for secondary speech surgery, and speech outcomes post-operatively.

Secondary surgery to correct VPI was necessary in 3 of 21 (14.29%) PRS patients (2 Pharyngeal Flaps, 1 Z-plasty), vs. 10 of 42 (23.81%) CPO (9 Pharyngeal Flaps, 1 Z-plasty) controls. Mean age for VPI surgery for PRS vs. controls: 5.33 vs. 6.41 years, respectively. For final speech outcomes, 73.68% of PRS vs. 71.88% of controls showed no evidence of hypernasality, 89.47% of PRS patients vs. 93.75% of controls showed no evidence of hyponasality, and 76.47% of PRS patients vs. 78.13% of controls had normal velopharyngeal competence (p>0.90 for all three measures).

Our findings suggest that children born with a Pierre-Robin Sequence do not have a higher rate of post-operative VPI after cleft palate repair and are no more likely to require additional surgical intervention.