Journal of cranio-maxillo-facial surgery : official publication of the European Association for Cranio-Maxillo-Facial Surgery · 2026
Morphological analysis of posterior fossa in Apert and Crouzon syndromes before and after posterior cranial vault expansion.
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Dong Ruonan, Liang Ce, Evans Susan E, Johnson David, Shui Wuyang, Duncan Christian, Khonsari Roman H, Paternoster Giovanna, Moazen Mehran
Аннотация
BACKGROUND: Syndromic craniosynostosis, such as Apert and Crouzon syndromes, involve premature fusion of cranial sutures, leading to cranial deformities and potential neurological complications. Posterior cranial vault expansion (PCVE) is commonly used to address intracranial volume (ICV) anomalies in these cases, especially in the posterior fossa region. However, the morphological variation of this region between normal, Apert and Crouzon individuals remains understudied. This study aimed to carry out a detailed morphological analysis of the posterior fossa between the aforementioned groups before and after PCVE.
METHODS: Retrospective computed tomography (CT) data from 28 children with Apert (n = 13) and Crouzon (n = 15) syndromes, alongside 51 age-matched controls, were analyzed. Intracranial and posterior fossa volumes (ICV and PFV) were computed pre- and post-operatively. Morphometric shape analyses were conducted for posterior fossa and intracranial cavity using principal component analysis (PCA).
RESULTS: Pre-operatively, Apert syndrome patients exhibited larger PFV and ICV compared to Crouzon syndrome patients and controls (e.g., p = 0.009 for pre-operative Apert vs. controls), while Crouzon syndrome patients showed more variability in PFV and ICV. Post-operatively, Apert patients demonstrated significant increases in both PFV and PFV/ICV ratios (e.g., post-operative vs. pre-operative PFV: p < 0.001). In contrast, Crouzon patients exhibited limited improvements in PFV, with PFV/ICV ratios often declining post-surgery (p = 0.890; median post-operative-pre-operative difference = -0.095). PCA revealed distinct intracranial and posterior fossa shape differences between Apert and Crouzon patients.
CONCLUSIONS: While PCVE increased posterior fossa volume, it did not appear to improve posterior fossa shape in either Apert or Crouzon patients. Future studies are required to address the limitations of small sample sizes, lack of post-operative data on Chiari anomaly, and surgical variability to further explore possible correlations between the aforementioned parameters.
METHODS: Retrospective computed tomography (CT) data from 28 children with Apert (n = 13) and Crouzon (n = 15) syndromes, alongside 51 age-matched controls, were analyzed. Intracranial and posterior fossa volumes (ICV and PFV) were computed pre- and post-operatively. Morphometric shape analyses were conducted for posterior fossa and intracranial cavity using principal component analysis (PCA).
RESULTS: Pre-operatively, Apert syndrome patients exhibited larger PFV and ICV compared to Crouzon syndrome patients and controls (e.g., p = 0.009 for pre-operative Apert vs. controls), while Crouzon syndrome patients showed more variability in PFV and ICV. Post-operatively, Apert patients demonstrated significant increases in both PFV and PFV/ICV ratios (e.g., post-operative vs. pre-operative PFV: p < 0.001). In contrast, Crouzon patients exhibited limited improvements in PFV, with PFV/ICV ratios often declining post-surgery (p = 0.890; median post-operative-pre-operative difference = -0.095). PCA revealed distinct intracranial and posterior fossa shape differences between Apert and Crouzon patients.
CONCLUSIONS: While PCVE increased posterior fossa volume, it did not appear to improve posterior fossa shape in either Apert or Crouzon patients. Future studies are required to address the limitations of small sample sizes, lack of post-operative data on Chiari anomaly, and surgical variability to further explore possible correlations between the aforementioned parameters.