Describing Crouzon and Pfeiffer syndrome based on principal component analysis

Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery
Femke C R StaalDavid Dunaway

Abstract

Crouzon and Pfeiffer syndrome are syndromic craniosynostosis caused by specific mutations in the FGFR genes. Patients share the characteristics of a tall, flattened forehead, exorbitism, hypertelorism, maxillary hypoplasia and mandibular prognathism. Geometric morphometrics allows the identification of the global shape changes within and between the normal and syndromic population. Data from 27 Crouzon-Pfeiffer and 33 normal subjects were landmarked in order to compare both populations. With principal component analysis the variation within both groups was visualized and the vector of change was calculated. This model normalized a Crouzon-Pfeiffer skull and was compared to age-matched normative control data. PCA defined a vector that described the shape changes between both populations. Movies showed how the normal skull transformed into a Crouzon-Pfeiffer phenotype and vice versa. Comparing these results to established age-matched normal control data confirmed that our model could normalize a Crouzon-Pfeiffer skull. PCA was able to describe deformities associated with Crouzon-Pfeiffer syndrome and is a promising method to analyse variability in syndromic craniosynostosis. The virtual normalization of a Crouzon-Pfeiffer skull i...Continue Reading

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Citations

Feb 18, 2017·American Journal of Obstetrics and Gynecology·Andrea Dall'AstaChristoph C Lees
Nov 16, 2018·The Journal of Craniofacial Surgery·Kosuke KuwaharaShunsuke Nosaka
Nov 2, 2019·International Journal of Computer Assisted Radiology and Surgery·F AngulliaD J Dunaway
May 28, 2017·International Journal of Computer Assisted Radiology and Surgery·Naiara Rodriguez-FlorezSilvia Schievano
Jan 1, 2018·International Journal of Computer Vision·James BoothStefanos Zafeiriou
Mar 18, 2020·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Jebrane BouaoudRoman H Khonsari
Mar 14, 2021·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Pam HeutinckAlessandro Borghi
Apr 18, 2021·Clinical Oral Investigations·Jan Aart M SchipperEppo B Wolvius

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