Facial Asymmetry in Nonsyndromic and Muenke Syndrome–Associated Unicoronal Synostosis: A 3-Dimensional Study Based on Facial Surfaces Extracted From CT Scans

Author:

Öwall Louise1ORCID,Darvann Tron A.12,Hove Hanne B.34,Heliövaara Arja5ORCID,Dunø Morten6,Kreiborg Sven17,Hermann Nuno V.17

Affiliation:

1. 3D Craniofacial Image Research Laboratory (School of Dentistry, University of Copenhagen, Center of Head and Orthopedics, Copenhagen University Hospital Rigshospitalet, and DTU Compute, Technical University of Denmark), Copenhagen, Denmark

2. Department of Oral and Maxillofacial Surgery, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark

3. Center for Rare Diseases, Department of Pediatrics, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark

4. The RAREDIS Database, Center for Rare Diseases, Department of Pediatrics, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark

5. Cleft Palate and Craniofacial Center, Department of Plastic Surgery, Helsinki University Hospital, Helsinki, Finland

6. Center for Rare Diseases, Department of Clinical Genetics, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark

7. Department of Pediatric Dentistry and Clinical Genetics, School of Dentistry, Faculty of Health and Medical Science, University of Copenhagen, Copenhagen, Denmark

Abstract

Objective: To quantify soft tissue facial asymmetry (FA) in children with nonsyndromic and Muenke syndrome–associated unicoronal synostosis (NS-UCS and MS-UCS), hypothesizing that MS-UCS presents with significantly larger FA than NS-UCS. Design: Retrospective cohort study. Patients and Methods: Twenty-one children (mean age: 0.6 years; range: 0.1-1.4 years) were included in the study (NS-UCS = 14; MS-UCS = 7). From presurgical computed tomography scans, facial surfaces were constructed for analysis. A landmark guided atlas was deformed to match each patient’s surface, obtaining spatially detailed left-right point correspondence. Facial asymmetry was calculated in each surface point across the face, as the length (mm) of an asymmetry vector, with its Cartesian components providing 3 directions. Mean FA was calculated for the full face, and the forehead, eye, nose, cheek, mouth, and chin regions. Results: For the full face, a significant difference of 2.4 mm ( P = .001) was calculated between the 2 groups, predominately in the transverse direction (1.5 mm; P < .001). The forehead and chin regions presented with the largest significant difference, 3.5 mm ( P = .002) and 3.2 mm ( P < .001), respectively; followed by the eye (2.4 mm; P = .004), cheek (2.2 mm; P = .004), nose (1.7 mm; P = .001), and mouth (1.4 mm; P = .009) regions. The transverse direction presented with the largest significant difference in the forehead, chin, mouth, and nose regions, the sagittal direction in the cheek region, and the vertical direction in the eye region. Conclusions: Muenke syndrome–associated unicoronal synostosis presented with significantly larger FA in all regions compared to NS-UCS. The largest significant differences were found in the forehead and chin regions, predominantly in the transverse direction.

Funder

Lippmann foundation

Publisher

SAGE Publications

Subject

Otorhinolaryngology,Oral Surgery

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