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Crouzon Syndrome: Basic Dysmorphology and Staging of Reconstruction

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Craniomaxillofacial Reconstructive and Corrective Bone Surgery

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Abstract

Crouzon syndrome is the most frequent form of craniofacial dysostosis.1–6 It is characterized by multiple anomalies of the craniofacial skeleton. Its manifestations are generally less severe than those of Apert syndrome, and there is no involvement of the extremities. Typically, the cranial vault presentation is a brachycephalic shape to the skull caused by premature synostosis of both coronal sutures. Cranial vault suture involvement, other than coronal, may include sagittal, metopic, or lambdoidal in isolation or in any combination. The cranial base and upper face sutures are generally involved, resulting in a variable degree of midface hypoplasia with an angle class III malocclusion. The orbits are hypoplastic, resulting in a degree of proptosis with additional orbital dysplasia that may produce a mild to moderate orbital hypertelorism and flatness to the (transverse) arc of rotation of the midface.7–13

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  1. Jeffrey C. Posnick
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Editors and Affiliations

  1. Division of Oral and Maxillofacial Surgery, Columbia University School of Dental and Oral Surgery, New York, NY, USA

    Alex M. Greenberg DDS (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending) (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending)

  2. Division of Oral and Maxillofacial Surgery, Mount Sinai School of Medicine, New York, NY, USA

    Alex M. Greenberg DDS (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending) (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending)

  3. Division of Oral and Maxillofacial Surgery, Beth Israel Medical Center, New York, NY, USA

    Alex M. Greenberg DDS (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending) (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending)

  4. Division of Oral and Maxillofacial Surgery, St. Luke’s/Roosevelt Hospital, New York, NY, USA

    Alex M. Greenberg DDS (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending) (Assistant Clinical Professor, Clinical Instructor, Assistant Attending, Associate Attending)

  5. Clinic for Reconstructive Surgery, Kantonsspital Basel, CH-4031, Basel, Switzerland

    Joachim Prein MD, DDS (Professor of Maxillofacial Surgery; and Head) (Professor of Maxillofacial Surgery; and Head)

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Posnick, J.C. (2002). Crouzon Syndrome: Basic Dysmorphology and Staging of Reconstruction. In: Greenberg, A.M., Prein, J. (eds) Craniomaxillofacial Reconstructive and Corrective Bone Surgery. Springer, New York, NY. https://doi.org/10.1007/978-0-387-22427-5_59

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  • DOI: https://doi.org/10.1007/978-0-387-22427-5_59

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