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Abstract

Purpose: Newborns with Pierre Robin sequence (PRS) suffer
from mandibular hypoplasia, glossoptosis and possibly cleft
palate. Respiratory distress is the leading clinical symptom after birth. The facial appearance of the newborns is characteristic with a severely retruded chin. The aim of this study was to analyze 3-dimensionally the development of the face of PRS children.
Patients and Methods: In a prospective, cross-sectional study 344 healthy children and 37 children with PRS and cleft palate were scanned 3-dimensionally at the age of 0 to 8 years. The images were then superimposed. Growth curves for normal facial development were calculated. The facial morphology of PRS children was compared to healthy children. For statistical analysis the Pearson test was applied.
Results: Facial growth of PRS children in the transversal direction was normal. In the vertical dimension there was a significant underdevelopment of the chin. Up to the age of 36 months 13 of 27 children had a sagittal position of pogonion below the third percentile. Except for the orbital landmarks and nasion, however, also all landmarks of the midface demonstrated a significant deficit. This difference to healthy children remained constant throughout the observation period.
Discussion: The anticipated mandibular deficit was confirmed. A catch-up growth could not be observed. A sagittal deficit of the midface was evident in all ages. This indicates that PRS children also suffer from a very early, severe and persistent midface underdevelopment. Early orthodontic and speech therapy is necessary.