Bone Dysmorphogenesis in Patients with Non-Syndromic Unilateral Coronal Synostosis
Christopher P. Bellaire, BA1; Alex Devarajan, BS1; James G. Napoli, BS, MS2;John W. Rutland, BA1;Helen Liu, BS1;Farah Sayegh, MD1;Bradley N. Delman, MD, MS3; Peter J. Taub, MD, MS1
1 Division of Plastic and Reconstructive Surgery, Icahn School of Medicine at Mount Sinai, New York, NY 2 Division of Paleontology, American Museum of Natural History, New York, NY 3 Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, New York, NY
Unilateral coronal craniosynostosis is a congenital disorder resulting from the premature fusion of one of the coronal suture. Premature fusion of the coronal suture on either the left or the right side leads to complex primary and compensatory morphologic changes in the shape of the calvarium and skull base. This deformity typically requires surgery to correct the shape of the skull and prevent neurologic sequelae, including increased intracranial pressure, sensory deficits, and cognitive decline.The present multi-center study sought to reverse-engineer the bone dysmorphogenesis seen in non-syndromic unilateral coronal craniosynostosis using a geometric morphometric approach. Computed tomography scans for 26 non-syndromic unilateral coronal synostosis patients were converted to three-dimensional mesh models. 236 unique anatomical landmarks and semi-landmarked curves were then plotted on each model, creating wireframe representations of the patients' skulls. Generalized Procrustes superimposition, Principal Component Analysis, and heatmaps identified significant superior displacement of the ipsilateral orbit ("harlequin" eye deformity), anterior displacement of the ear ipsilateral to the fused coronal suture, acute deviation of midline skull base structures ipsilateral to the fused coronal suture and flattening of the parietal bone with failure to expand superiorly.The described technique illustrates the impact of premature coronal suture fusion on the development of the entire skull and proposes how bone dysmorphology contributes to the patients' neurologic sequelae. By bridging novel basic science methodologies with clinical research, the present study quantitatively describes craniofacial development and bone dysmorphogenesis.
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