Objective To analyze the morphology of the maxillary crest in infants with Pierre Robin sequence using an anthropometric coordinate system and to compare the data with those of healthy infants. Setting The study was performed at a craniofacial center servicing a large geographic area. Participants The study involved eight infants aged 1–28 days (average, 7 days) with an established diagnosis of Pierre Robin sequence and six healthy infants aged 1–43 days (average, 22 days). Main outcome Measures Physical models of the maxilla and face obtained by alginate replication were analyzed by computer morphometry yielding the three-dimensional topology of the maxillary crest. Results The maxillary crest of children with Pierre Robin sequence shows an increased inclination relative to the transverse plane (30 ± 3.9 degrees) as compared with that of healthy infants (20 ± 2.9 degrees). The maxillary crest of the patients is shortened in the sagittal direction by comparison with healthy controls. Conclusions The increased inclination of the maxilla in infants with Pierre Robin sequence may aggravate the retroposition of the mandible and may thus be a pathogenetic factor contributing to the severe respiratory problems.

We propose that the morphologic deviations are in part caused by the neuromotor activity of the tongue and of the interrupted M. orbicularis oris. The data can serve as the starting point for a longitudinal study of craniofacial development in children with cleft palate and for studies on the efficacy of different therapeutic approaches.

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