Three-Dimensional Assessment of Facial Development in Children With Pierre Robin Sequence

Abstract

Newborns with Pierre Robin sequence (PRS) have mandibular hypoplasia, glossoptosis, and possibly cleft palate. Their facial appearance is characteristic. The further facial development is controversial. The aim of this study was to analyze the facial development of children with PRS.In a prospective, cross-sectional study, 344 healthy children and 37 children with PRS and cleft palate younger than 8 years were scanned three-dimensionally. Twenty-one standard anthropometric landmarks were identified, and the images were superimposed. Growth curves for normal facial development were calculated. The facial morphology of children with PRS was compared with that of healthy children.The facial growth of children with PRS in the transversal and vertical direction was normal. In the sagittal direction, the mandibular deficit was confirmed. Except for the orbital landmarks and nasion, all landmarks of the midface demonstrated a significant sagittal deficit. This difference to healthy children remained constant for all ages.Our study cannot support the theory of mandibular catch-up growth. The sagittal deficit of the midface could be observed in all ages. This indicates that children with PRS have a very early, severe, and persistent underdevelopment of this part of the face. We conclude that this disturbance must be addressed in early childhood with orthodontic and speech therapy.

Diagnostik und Therapie der Pierre-Robin-Sequenz

“…Bei zusätzlichen syndromalen Erkrankungen können komplexere Pathophysiologien mit pharyngealer Dysfunktion hinzukommen [19]. Zwar steht blickdiagnostisch bei Neugeborenen die Retrogenie im Vordergrund, es bestehen bei PRS aber auch Auffälligkeiten der skelettalen Entwicklung des gesamten Mittelgesichts (Mittelgesichthypoplasie) mit weiterer Einengung des oberen Atemwegs [1,13], ähnlich wie sich dies auch bei Trisomie 21 und Kraniosynostosesyndromen findet.…”

Robin Sequence: From Diagnosis to Development of an Effective Management Plan

“…More recently, three-dimensional data from digital surface photogrammetry have been used to investigate craniofacial morphology. 61 Measurement of the maxillary-mandibular discrepancy on physical examination is a simple, objective measure used to quantify the small or posteriorly displaced jaw and monitor alveolar relationship after interventions and over time, but this must be done in the context of maxillary development 62 (Fig 3).…”

Craniofacial disorders associated with airway obstruction in the neonate

“…Distinct from other forms of palatal clefting with or without cleft lip, the cleft palate associated with PRS most often is wide and U-shaped, thought to be the result of failure of the palatal shelves to fuse in early gestation due to the presence of an abnormally positioned tongue [21]. There is growing evidence that midface hypoplasia may be an important finding in patients with PRS [22], and is probably more pronounced in some infants than others.…”