Facial Skeletal Morphology in Growing Children with Pierre Robin Sequence

Abstract

The purpose was to compare the jaw size, jaw relationship, and facial proportions of children with nonsyndromic Pierre Robin sequence with children with nonsyndromic or isolated cleft palate. This is a retrospective cohort study comparing radiographic findings in children with Pierre Robin sequence or isolated cleft palate at two time intervals: ages 4 to 7 years (T1) and ages 10 to 13 years (T2). Linear and angular measurements were obtained using cephalograms; the cephalometric values were compared with unpaired t tests, assuming unequal variances. The study was conducted at the Center for Craniofacial Anomalies at the University of California, San Francisco. The sample included 13 children with Pierre Robin sequence and 14 children with isolated cleft palate who were followed at University of California, San Francisco, craniofacial anomalies clinic during the period from 1971 to 2007 and met the inclusion criteria. The outcome measures were mandibular length, maxillary length, and sagittal jaw relationship in Pierre Robin sequence and isolated cleft palate children at T1 and T2. During early childhood (T1: 4 to 7 years), mandibular length (Co-Gn) was similar in both Pierre Robin sequence and isolated cleft palate groups, as was the maxillary length (Co-A). In older children (T2: 10 to 13 years) mandibular length was significantly shorter in the Pierre Robin sequence group compared with the isolated cleft palate group (difference = 10.9 mm, p = .009). Maxillary length in Pierre Robin sequence and isolated cleft palate groups was similar at T2 but significantly shorter in comparison to age-matched norms with a difference of 14.5 mm (p = .037) for Pierre Robin sequence and 12.4 mm (p = .045) for isolated cleft palate. Children with Pierre Robin sequence did not show a sagittal jaw discrepancy due to a proportionate deficiency in maxillary and mandibular length; whereas, children with isolated cleft palate showed a greater sagittal jaw discrepancy due to normal mandibular length and deficiency in maxillary length. Our study confirms the findings of some previous studies that suggest that children with nonsyndromic Pierre Robin sequence have a proportionate retrusion of the maxilla and mandible, resulting in a convex facial profile by early adolescence.