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Purpose: To assess the role of FGFR2 mutations and sutural synostotic patterns on facial skeleton dysmorphology in children with syndromic craniosynostosis., Methods: Preoperative high-resolution CT images in 39 infants with syndromic craniosynostosis were evaluated. Patients were divided into infants with and without FGFR2 mutations; each group was split according to synostotic involvement of minor sutures/synchondroses: isolated or combined involvement of middle (MCF) and posterior cranial fossae (PCF). Quantitative analysis of the midface and mandible measures was performed. Each subgroup was compared with a group of age-matched healthy subjects., Results: Twenty-four patients with FGFR2 related syndromes were clustered in 3 subgroups: MCF + PCF (8 patients, 5.4 ± 1.75 months), MCF (8 patients, 3.62 ± 1.68 months), and PCF (8 patients, 2.75 ± 0.46 months). Fifteen no-FGFR2 patients were clustered in 2 subgroups: MCF + PCF (7 patients, 9.42 ± 0.78 months) and PCF (8 patients, 7.37 ± 2.92 months). Both FGFR2 and no-FGFR2 groups with involvement of minor sutures coursing in MCF showed more facial sutural synostoses. Children with minor suture/synchondrosis synostosis of MCF (MCF-PCF and MCF subgroups) showed altered position of glenoid fossa and mandibular inclination ([Formula: see text]), but children in the FGFR2 group had also reduced midfacial depth and maxillary length ([Formula: see text]). Children with minor suture/synchondrosis synostosis of PCF (PCF subgroups) had reduced posterior mandibular height, but those children in the FGFR2 group also showed reduced intergonion distance ([Formula: see text])., Conclusions: In children with syndromic craniosynostosis, both skull base and facial suture synostosis affect facial dysmorphology/hypoplasia. FGFR2 mutations may worsen facial hypoplasia both acting on bone development and causing an earlier premature closure of facial sutures., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: We performed a retrospective qualitative and quantitative evaluation of the sutural changes during the physiological growth to define the age-related ossification stages of major and minor skull sutures or synchondroses., Methods: A total of 390 healthy subjects, examined for cranio-facial trauma and whose CT scans turned out to be normal, were clustered into homogenous age-matched groups ranged from birth to 90 years. High-resolution CT was used to assess the degree of sutural closure according to a 3-grade scoring system, the sutural pattern, the width, and the density of the gap calculated as the average of two or three ROIs along each suture/synchondrosis., Results: The identification of a definite pattern depended on the suture's type, the closure degree, and the width of the gap (p < 0.001). The interdigitation process was more intricate for most of vault sutures than the skull base sutures/synchondroses. Closing grades 1, 2, and 3 were associated to an identifiable sutural pattern and the cutoff value of 1.45 mm of the gap width allowed to detect an identifiable sutural pattern with the best combination of sensitivity (97%) and specificity (98%). Age and sutural closing degree were inversely related to gap width while positively related to the gap density (p < 0.001)., Conclusion: The sutural ossification is an age-related process, distinctive for each suture, and synchondrosis; it occurs neither according to a predefined order along sutural arches nor following a sequential distribution in the cranial fossae, and some sutures continued their growth process during lifetime., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The aim of this study was to identify quantitative tools to classify the severity of trigonocephaly to guide surgical management and predict outcome., Methods: We reviewed high-resolution computed tomography images of 59 patients with metopic synostosis. We assessed the craniofacial sutural pattern as well as interfrontal and metopic angles, and we related the frontal angulation degree with the sutural pattern, the surgical management, and clinical outcome., Results: We identified 3 groups according to the severity of trigonocephaly. No difference was found between the sutural pattern of nasion complex and severity, whereas the closure of zygomatic maxillary sutures increased with the severity degree (P < 0.05). The operative management was related to the severity degree (P < 0.001) and to the reduced age (P = 0.009)., Conclusions: Interfrontal and metopic angles are complementary measurements to evaluate with high accuracy the degree of frontal angulation. In preoperative assessment, they may guide surgery decision in particular when the choice is not straightforward.

Purpose: The effects of premature fusion of one coronal suture cause skull and orbital alterations in term of side-to-side asymmetry. This study aimed to quantify the cranio-orbital complex changes related to the severity of skull base dysmorphology in patients with unicoronal synostosis., Methods: Twenty-four infants affected by unicoronal synostosis were subdivided in three subgroups according to the severity of skull base deformity and their high-resolution CT images were quantitatively analyzed (groups IIa, IIb, III). Dimensions of cranial fossae, intracranial volume (ICV), ICV synostotic and ICV non synostotic side, whole brain volume (WBV), orbital volumes (OV), ICV/WBV, ICV synostotic /ICV non-synostotic , and OV synostotic /OV non-synostotic were evaluated., Results: Asymmetry and reduction in the growth of the anterior and middle fossae were found in all groups while asymmetry of the posterior cranial fossa was found only in IIb and III groups. In all groups, ICV, WBV, and ICV/WBV were not significantly different while ICV synostotic/ ICV non-synostotic and OV synostotic/ OV non-synostotic resulted significant difference (p < 0.05). ICV synostotic side resulted reduction only in group III. OV on the synostotic side was not significantly reduced although a trend in progressively reducing volumes was noted according to the severity of the group., Conclusion: Skull and orbital changes revealed a side-to-side asymmetry but the effects of the premature synostosis were more severe in group III suggesting an earlier timing of premature unicoronal synostosis in group III with respect to the other groups. The assessment of the skull base deformity might be an indirect parameter of severity of skull orbital changes and it might be useful for surgical planning.

Purpose: Craniosynostostic syndromes are due to multisuture synostoses and affect the entire craniofacial skeleton. This study analyzed the facial complex and airways to quantify the relationship between insufficient facial growth, airways obstruction, and the sutural pattern of the splanchnocranium and cranial fossae., Methods: Preoperative high-resolution CT images in 19 infants with syndromic craniosynostosis were quantitatively analyzed. Because all children showed involvement of minor sutures/synchondroses coursing in the posterior cranial fossa, they were divided into three groups according to the synostotic involvement of "minor" sutures/synchondroses coursing in anterior (ACF) and middle (MCF) cranial fossae: group 1 (ACF), group 2 (MCF), and group 3 (ACF-MCF). Analysis of the facial complex and airway was performed. Each group was compared with age-matched healthy subjects., Results: Premature closure of skull base synchondroses of ACF and MCF was found only in groups MCF and ACF-MCF. Group MCF showed synostosis in the posterior branch of the coronal ring and reduced anterior hemifossae lengths while group ACF-MCF showed synostosis in the anterior branch of the coronal ring and reduced middle hemifossae lengths. No group showed reduced maxillary or mandibular volumes but group MCF showed synostosis of the zygomaticomaxillary sutures and maxillary retrusion. All groups showed reduced airway volume but group 2 had a higher degree of airway hypoplasia., Conclusion: The skull base synostotic process drives the changes in facial complex growth and airway obstruction. Premature closure of synchondroses/sutures in the posterior branch of the coronal ring causes insufficient facial growth, maxillary retrusion, and more severe airway reduction.

Purpose: The study aimed to identify premature synostosis of "major" and "minor" sutures of the coronal sutural arch and splanchnocranium sutures to evaluate the relationship between craniofacial dysmorphology and the sutural pattern in children with anterior plagiocephaly., Methods: A quantitative analysis of the skull base and facial changes was performed on preoperative high-resolution CT images in 18 children with anterior synostotic plagiocephaly and compared with imaging findings in 18 age-matched healthy subjects., Results: All patients had patent splanchnocranium sutures. Fifteen out of 18 children showed early and isolated synostosis of the unicoronal suture (the major suture of the coronal ring) and were classified in groups II and III according to the classification scheme of anterior synostotic plagiocephaly based on the severity of craniofacial dysmorphology. Premature fusion of the unilateral coronal suture in groups II and III caused a marked asymmetry and reduced growth of the anterior and middle fossae on the synostotic side and a secondary varying severity in terms of asymmetric growth of the facial complex. Although both groups showed anterior displacement of the mandibular articulation on the synostotic side, group II showed only maxillary asymmetry, while group III showed maxillary and mandibular asymmetry., Conclusions: In anterior synostotic plagiocephaly, the severity of skull base changes and asymmetric growth of the facial complex is not caused by skull base sutural synostotic involvement but is probably related to the different timing of unilateral coronal suture closure.

Purpose: Craniosynostosis is a condition characterised by the premature fusion of one or more of the cranial sutures. The aim of the study was to identify, by multidetector computed tomography (CT), the involvement of vault sutures as well as of the skull base sutures (named "minor" sutures). The latter ones are involved in development of craniofacial and skull base deformities., Materials and Methods: We retrospectively reviewed 27 children with complex synostosis (n = 21) and anterior synostotic plagiocephaly (n = 6). High-resolution CT images with bone definition algorithm and tridimensional volume rendering reconstructions were assessed., Results: In 27 children we found different sutures involved in the synostotic process, including both major and minor skull suture synostosis, and synostosis of synchondroses. Superior orbital rim deformity, nasal root deviation, anterior endocranial axis deviation (ethmoidal axis) are found in children with coronal arch synostosis, while reduced size of the posterior fossa and Chiari 1 malformation are noted in children with lambdoid arch synostosis., Conclusions: High-resolution CT allows an accurate identification of both "major" and "minor" skull base suture synostosis and it represents the gold standard for the diagnosis of craniostenosis and for planning the proper surgical approach.