Cranial morphology associated with syndromic graniosynostosis: A potential detection of abnormality in patient’s cranial growth using angular statistics

Introduction: Apert, Crouzon, and Pfeiffer syndromes are common genetic syndromes related to syndromic craniosynostosis (SC), whereby it is a congenital defect that occurs when the cranial growth is distorted. Identifying cranial angles associated with these 3 syndromes may assist the surgical team...

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Bibliographic Details
Main Authors: Nur Syahirah, Zulkipli, Siti Zanariah, Satari, Norli Anida, Abdullah, Wan Nur Syahidah, Wan Yusoff, Abdul Ghapor, Hussin
Format: Article
Language:English
Published: Sage 2022
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Online Access:http://umpir.ump.edu.my/id/eprint/35467/1/2022%20Zulkipli%20et%20al%20cleft.pdf
http://umpir.ump.edu.my/id/eprint/35467/
https://journals.sagepub.com/doi/abs/10.1177/10556656221107524
http://10.1177/10556656221107524
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Summary:Introduction: Apert, Crouzon, and Pfeiffer syndromes are common genetic syndromes related to syndromic craniosynostosis (SC), whereby it is a congenital defect that occurs when the cranial growth is distorted. Identifying cranial angles associated with these 3 syndromes may assist the surgical team to focus on a specific cranial part during the intervention planning, thus optimizing surgical outcomes and reducing potential morbidity. Objective: The aim of this study is to identify the cranial angles, which are associated with Apert, Crouzon, and Pfeiffer syndromes. Methods: The cranial computed tomography scan images of 17 patients with SC and 22 control groups aged 0 to 12 years who were treated in the University Malaya Medical Centre were obtained, while 12 angular measurements were attained using the Mimics software. The angular data were then divided into 2 groups (patients aged 0 to 24 months and >24 months). This work proposes a 95% confidence interval (CI) for angular mean to detect the abnormality in patient’s cranial growth for the SC syndromes. Results: The 95% CI of angular mean for the control group was calculated and used as an indicator to confirm the abnormality in patient’s cranial growth that is associated with the 3 syndromes. The results showed that there are different cranial angles associated with these 3 syndromes. Conclusions: All cranial angles of the patients with these syndromes lie outside the 95% CI of angular mean of control group, indicating the reliability of the proposed CI in the identification of abnormality in the patient’s cranial growth.