New diagnostic approach of the different types of isolated craniosynostosis

Sophia A J Kronig,Henri A Vrooman,Jifke F Veenland,Léon N A Van Adrichem,Otto D M Kronig

doi:10.1007/s00431-020-03860-9

Abstract

In this study, we diagnose skull shape deformities by analysing sinusoid curves obtained from standardized computed tomography (CT) slices of the skull for the common craniosynostoses (scaphocephaly, brachycephaly, trigonocephaly, right- and left-sided anterior plagiocephaly). Scaphocephaly has a high forehead peak and low troughs, in contrast to brachycephaly. Anterior plagiocephaly has asymmetry and shifting of the forehead peak. Trigonocephaly has a high and narrow frontal peak. Control patients have a symmetrical skull shape with low troughs and a high and broader frontal peak. Firstly, we included 5 children of every group of the common craniosynostoses and additionally 5 controls for extraction and calculation of characteristics. A diagnostic flowchart was developed. Secondly, we included a total of 51 craniosynostosis patients to validate the flowchart. All patients were correctly classified using the flowchart.Conclusion: Our study proposes and implements a new diagnostic approach of craniosynostosis. We describe a diagnostic flowchart based on specific characteristics for every type of craniosynostosis related to the specific skull deformities and control patients. All variables are expressed in number; therefore, we are able to use these variables in future research to quantify the different types of craniosynostosis.What is Known:• Premature fusion of one or more cranial sutures results in a specific cranial shape.• Clinical diagnosis is relatively simple; however, objective diagnosis based on distinctive values is difficult.What is New:• Using external landmarks and curve analysis, distinctive variables, and values for every type of craniosynostosis related to the specific skull deformities were determined and used to create a diagnostic flowchart for diagnosis.• Validation with an independent data set of 51 patients showed that all patients were correctly classified.

Highlights

Craniosynostosis is the premature fusion of one or more of the cranial sutures
We describe a diagnostic flowchart based on specific characteristics for every type of craniosynostosis related to the specific skull deformities and control patients
All variables are expressed in number; we are able to use these variables in future research to quantify the different types of craniosynostosis

Summary

Introduction

Craniosynostosis is the premature fusion of one or more of the cranial sutures. Four sutures are considered major: the metopic, coronal, sagittal, and lambdoid. Premature fusion of one or more of the sutures results in characteristic anatomic malformations of the skull. The type of craniosynostosis is classified according to the synostotic suture(s) involved, the presence or absence of a skull-facial syndrome, and the (typical) shape of the cranial deformation. (sagittal synostosis) has a keel-like shape with an elongated anteroposterior and narrowed transverse dimension, whereas brachycephaly (bicoronal synostosis) has a reduced length with a retrusive forehead, orbits, and occiput and increased width [2, 3]. (metopic synostosis) has a characteristic triangular shape of the forehead and orbits, and anterior plagiocephaly (unilateral coronal synostosis (UCS)) is characterized by forehead and orbital asymmetry [2, 4, 5]

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Discussion

Conclusion