Mandibulofacial dysostosis--variability in facial morphology and growth: a long-term profile roentgenographic and roentgen stereometric analysis of three patients.

OBJECTIVE

To monitor and compare facial morphology and growth in three individuals with variable expression of mandibulofacial dysostosis (MFD) in terms of changes in the skeletal profile and in terms of growth in the circummaxillary sutures and temporomandibular joints (TMJs).

DESIGN

Retrospective conventional profile roentgenography (mean age 9 to 18 years) and prospective roentgen stereometric analysis (RSA) (mean age 7 to 17 years).

SETTING

Center for Craniofacial Anomalies and Department of Plastic and Reconstructive Surgery, Malmö University Hospital, Sweden.

PATIENTS: The first three MFD patients seen by one of the authors (B.R.).

INTERVENTIONS: Surgery was performed at the Department of Plastic and Reconstructive Surgery. Implants were inserted at surgery under general anesthesia. Roentgen examinations were performed in connection with continued clinical evaluations and treatment.

MAIN OUTCOME MEASURES: All profile roentgenograms were traced and measured by one of the authors (K.-V.S.) using a conventional point-based analysis.

RESULTS

The more afflicted patient showed a greater total difference in profile morphology and growth from the norm and more pronounced effects of articular growth restriction. Little change in the skeletal profile was associated with considerable displacement of the jaws.

CONCLUSIONS

The variability in MFD expression and surgical procedures in our patients is reflected less in the skeletal profile morphology and growth and more in the displacement of the jaws.