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Orbitofacial morphology changes with different suture synostoses in Crouzon syndrome.
Xiaona Lu1, Antonio Jorge Forte2, Alexandra Junn1
1Division of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT, USA.
Summary
Different cranial vault suture fusions in Crouzon syndrome impact orbital development. Tailored surgical interventions, beyond initial skull expansion, may improve oculo-orbital disproportion.
Area of Science:
- Craniofacial Surgery
- Pediatric Plastic Surgery
- Medical Imaging
Background:
- Crouzon syndrome is a craniosynostosis disorder characterized by premature fusion of cranial sutures.
- Cranial vault suture synostoses significantly impact craniofacial morphology, particularly orbital and periorbital development.
- Understanding the specific effects of different suture fusions is crucial for targeted surgical planning.
Purpose of the Study:
- To investigate the influence of distinct cranial vault suture synostoses on orbital and periorbital morphology in Crouzon syndrome patients.
- To compare the morphological changes associated with different types of synostoses (bicoronal, sagittal, pansynostosis, perpendicular combinations, bilateral squamosal).
- To determine if individualized surgical interventions based on synostosis subtype can mitigate oculo-orbital disproportion.
Main Methods:
- Computed tomography (CT) scans of 80 Crouzon syndrome patients and 72 normal controls were analyzed.
- Patients were subgrouped based on specific cranial vault suture synostoses (Type I-V).
- Measurements of orbital bony cavity volume, globe volume, retrobulbar soft tissue, and maxillary length were performed using Materialise software.
Main Results:
- Orbital bony cavity volume was reduced in all synostosis subgroups (16-24%).
- Globe volume reduction was observed in bicoronal synostosis (Type I), and retrobulbar soft tissue decreased in pansynostosis (Type III).
- Globe volume projection beyond the orbital rim significantly increased in all groups, especially Type IV.
Conclusions:
- The type of cranial vault suture synostosis influences the pattern and severity of orbital and periorbital morphological changes in Crouzon syndrome.
- Bilateral squamosal synostosis has a less pronounced effect compared to major vault sutures.
- Individualized surgical strategies, considering specific suture involvement, may offer improved outcomes for oculo-orbital disproportion.
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