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Analysis of Craniomaxillofacial Malformations in Mice Using Three-dimensional Microcomputed Tomography

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The Pierre Robin Mandible is Hypoplastic and Morphologically Abnormal.

Elizabeth G Zellner¹, Russell R Reid, Derek M Steinbacher

¹*Division of Plastic and Reconstructive Surgery, The Hospital for Sick Children, Univesity of Toronto, Toronto, ON, Canada †Section of Plastic and Reconstructive Surgery, Department of Surgery, University of Chicago, Chicago, IL ‡Section of Plastic and Reconstructive Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT.

The Journal of Craniofacial Surgery

|September 23, 2017

Summary

Pierre Robin sequence (PRS) affects mandibular morphology, causing smaller volumes and altered angles. Mandibular distraction osteogenesis (MDO) improves PRS mandible size and shape, normalizing angles but altering ramus and body lengths.

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Area of Science:

Craniofacial surgery
Pediatric orthopedics
3D imaging analysis

Background:

Pierre Robin sequence (PRS) is associated with significant mandibular hypoplasia.
Detailed 3D characterization of PRS mandibular morphology and the impact of mandibular distraction osteogenesis (MDO) remain incompletely understood.
Comparison with age-matched controls is essential for understanding PRS-related skeletal differences.

Purpose of the Study:

To quantitatively assess 3D mandibular morphology in infants with PRS compared to controls.
To evaluate the effects of MDO on mandibular dimensions and morphology in PRS patients.
To provide a comprehensive analysis of PRS-related craniofacial skeletal discrepancies.

Main Methods:

Retrospective analysis of pre- and postoperative CT scans from PRS infants undergoing MDO.
Inclusion of an age-matched control group with CT scans from a PACS database.
Measurement of mandibular volume, ramus length, body length, and angles (anterior symphyseal and mandibular).

Main Results:

PRS patients exhibited significantly reduced mandibular volume, ramus length, and body length compared to controls.
A reduced anterior symphyseal angle was observed in PRS patients, while the mandibular angle was maintained.
Post-MDO, mandibular volumes normalized, anterior symphyseal angles were indistinguishable from controls, but mandibular rami remained shorter and bodies longer.

Conclusions:

The mandible in PRS is characterized by dysmorphia, including smaller volume, shorter ramus, and an obtuse symphyseal angle.
MDO effectively improves mandibular volume and normalizes the symphyseal angle in PRS patients.
MDO leads to specific changes in mandibular dimensions, namely a longer body and shorter ramus, compared to controls.