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What Is the Skull Structure Influence of Squamosal Suture Synostosis in Nonsyndromic and Syndromic Crouzon Craniosynostosis?

Lu, Xiaona, MD*; Chen, Guocheng, MD; Forte, Antonio Jorge, MD, PhD; Cabrejo, Raysa, BS§; Singh, Anusha, BS§; Kyle, Gabrick, MD§; Steinbacher, Derek M., MD, DMD§; Alperovich, Michael, MD§; Alonso, Nivaldo, MD, PhD||; Persing, John A., MD§

doi: 10.1097/SCS.0000000000005396
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Background: The morphologic effect of squamosal synostosis has not been clarified, due to its low occurrence rate, and its inclination to be combined with premature closure of other major sutures, especially in syndromic synostosis. This study's goal is to explore the morphological influence of squamosal synostosis in both syndromic and nonsyndromic craniosynostosis patients.

Methods: Twenty-five computed tomography (CT) scans from nonsyndromic squamosal synostosis (NSS), Crouzon syndrome with squamosal synostosis (CSS), Crouzon syndrome without squamosal synostosis, and normal controls were included. Three-dimensional (3D) cephalometry, entire cranial volume and cranial fossa volume were measured using Materialise software.

Results: The entire cranial volume of all groups was similar. The NSS developed a gradual reduction in the severity of reduced segmental volume from the anterior cranial fossa, to the middle cranial fossa, and to the unaffected volume of posterior cranial fossa. The CSS developed the most severe volume reduction of middle cranial fossa (12608.30 ± 2408.61 mm3 vs 26077.96 ± 4465.74 mm3, 52%), with proportionate volume reduction of the anterior (7312.21 ± 2435.97 mm3 vs 10520.63 ± 2400.43 mm3, 30%) and posterior cranial fossa (33487.29 ± 5598.93 mm3 vs 48325.04 ± 14700.44 mm3, 31%). The NSS developed a narrower anterior cranial fossa, a clockwise rotated Frankfort horizontal plane, and a retracted chin (pogonion).

Conclusion: Squamosal synostosis may alter the development of cranial fossa volume, especially the volume of middle cranial fossa. However, the development of cranial base, and related facial features are determined largely by major vault sutures and associated syndromic conditions, rather than the squamosal suture alone.

Level of Evidence: II

*Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital, Beijing

Department of Plastic Surgery, The First Affiliated Hospital, School of Clinical Medicine of Guangdong Pharmaceutical University, Guangzhou, Guangdong, China

Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida, Jacksonville, FL

§Section of Plastic and Reconstructive Surgery, Yale School of Medicine, New Haven, CT

||Department of Plastic Surgery, University of São Paulo, São Paulo, Brazil.

Address correspondence and reprint requests to John A. Persing, MD, Section of Plastic and Reconstructive Surgery – Yale School of Medicine, 330 Cedar Street, 3rd floor Boardman Building, New Haven, CT 06520, USA; E-mail: john.persing@yale.edu

Received 15 October, 2018

Accepted 27 December, 2018

The authors report no conflicts of interest.

Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.jcraniofacialsurgery.com).

© 2019 by Mutaz B. Habal, MD.