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Purpose: Unilateral coronal synostosis (UCS) results in an asymmetrical skull, including shallow and asymmetrical orbits, associated with reduced orbital volume and high prevalences of ophthalmic sequelae. Aim is to link orbital volumes in patients with UCS to severity according to UCSQ (Utrecht Cranial Shape Quantifier) and presence of ophthalmic sequelae. Methods: We included preoperative patients with UCS (≤ 18 months). Orbital volume was measured on CT scans by manual segmentation (Mimics software (Materialise, Leuven, Belgium)), and severity of UCS was determined by UCSQ. Orbital volume of affected side was compared to unaffected side using Wilcoxon signed rank test. Orbital volume ratio was calculated (affected/unaffected volume) and compared to the category of UCSQ by Kruskal-Wallis test. Opthalmic sequelae were noted. Results: We included 19 patients (mean age 7 months). Orbital volume on affected side was significantly lower (p = 0.001), mean orbital volume ratio was 0.93 (SD 0.03). No significant differences in group means of orbital volume ratio between different levels of severity of UCSQ were found (Kruskal-Wallis H (2) = 0.873; p > 0.05). Ophthalmic sequelae were found in 3 patients; one had adduction impairment and strabismus (mild UCS), one had astigmatism (moderate UCS), and one had abduction impairment (on both ipsi- and contralateral side) and vertical strabismus (severe UCS). Conclusion: No association between orbital volume ratio and severity of UCS was found. Side-to-side asymmetry in orbital volume was noted. No association between either preoperative orbital volume ratio or severity of UCS and the presence of preoperative ophthalmic sequelae was found.

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.

Objectives: Severity of unilateral coronal synostosis (UCS) can vary. Quantification is important for treatment, expectations of treatment and natural outcome, and education of the patient and parents. Design: Retrospective study. Setting: Primary craniofacial center. Patients, Participants: Twenty-three preoperative patients with unilateral coronal craniosynostosis (age < 2 years). Intervention: Utrecht Cranial Shape Quantifier (UCSQ) was used to quantify severity using the variables: asymmetry ratio of frontal peak and ratio of frontal peak gradient. Main Outcome Measures(s): The UCSQ variables were combined and related to visual score using Pearson correlation coefficient; UCSQ and visual score were additionally compared to Di Rocco classification by one-way analysis of variance or Kruskal-Wallis test. All measurements were made on computed tomography scans. Results: Good correlation between UCSQ and visual score was found (r = 0.67). No statistically significant differences were found between group means of UCSQ in the 3 categories of Di Rocco classification (F2,20 = 0.047; P >.05). Kruskal-Wallis test showed no significant differences between group means of visual score in the 3 categories of Di Rocco classification (Kruskal-Wallis H (2) = 0.871; P >.05). Conclusions: Using UCSQ, we can quantify UCS according to severity using characteristics, it outperforms traditional methods and captures the whole skull shape. In future research, we can apply UCSQ to 3D-photogrammetry due to the utilization of external landmarks.