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Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model
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Midface Hypoplasia and Cranial Base Morphology in Syndromic Craniosynostosis: A Comparative Analysis Study Using a Predictive Regression Model

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Three-dimensional assessment of facial development in children with Pierre Robin sequence

Michael Krimmel1, Susanne Kluba, Martin Breidt

  • 1Department of Oral and Maxillofacial Surgery, University of Tübingen, Germany. Michael.Krimmel@med.uni-tuebingen.de

The Journal of Craniofacial Surgery
|November 3, 2009
PubMed

Insights

Children with Pierre Robin sequence (PRS) show normal facial growth vertically and transversally. However, a persistent midface sagittal deficit, not catch-up mandibular growth, characterizes their development, requiring early intervention.

Area of Science:

  • Craniofacial development
  • Pediatric genetics
  • Medical imaging

Background:

  • Pierre Robin sequence (PRS) is characterized by mandibular hypoplasia, glossoptosis, and potential cleft palate.
  • Facial development patterns in PRS patients are not fully understood and remain controversial.
  • Early identification and intervention are crucial for managing PRS-related craniofacial abnormalities.

Purpose of the Study:

  • To analyze and compare the facial development of children with PRS against healthy controls.
  • To investigate the sagittal, transversal, and vertical growth patterns in PRS patients.
  • To evaluate the theory of mandibular catch-up growth in children with PRS.

Main Methods:

  • Prospective, cross-sectional study involving 3D scans of 37 children with PRS and 344 healthy children under 8 years.
  • Identification of 21 standard anthropometric landmarks and image superimposition.
  • Calculation of normal facial growth curves and comparison of PRS facial morphology.

Main Results:

  • Facial growth in the transversal and vertical planes was normal for children with PRS.
  • A significant and persistent sagittal deficit was confirmed in the midface, excluding orbital landmarks and nasion.
  • The study did not support the theory of mandibular catch-up growth in PRS patients.

Conclusions:

  • Children with PRS exhibit early-onset, severe, and persistent midface underdevelopment.
  • The observed sagittal deficit remains constant across all studied ages.
  • Early orthodontic and speech therapy are recommended for addressing craniofacial disturbances in PRS.

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