Individual Influence Of Bicoronal Synostosis, Apert Syndrome And Crouzon Syndrome On Cranial Morphology
Xiaona Lu1, Antonio Jorge Forte2, Michael Alperovich1, Derek M. Steinbacher1, Nivaldo Alonso3, John A. Persing1.
1Yale University School of Medicine, New haven, CT, USA, 2Mayo Clinic Florida, Jacksonville, FL, USA, 3University of São Paulo, São Paulo, Brazil.
The association of isolated craniosynostosis and the influence of syndromic forms, confound the understanding of craniofacial morphological development. This study attempts to clarify the individual influences of isolated bicoronal synostosis, Apert syndrome and Crouzon syndromes on skull base morphology.
One hundred and seventeen CT scans were included (nonsyndromic bicoronal synostosis, n=36; Apert syndrome with bicoronal synostosis, n=25; Crouzon syndrome with bicoronal synostosis, n=11; controls, n=45). Cephalometric measurements were analyzed using Materialise software.
Nonsyndromic bicoronal synostosis patients developed a shortened cranial base length, with a significantly shortened distance between nasion and sella (p=0.005). The cranial base angles of nonsyndromic bicoronal synostosis in both the cranial side (N-S-BA) and facial side (N-SO-BA) significantly increased, by 17.04 degrees (p<0.001) and 11.75 degrees (p<0.001), respectively. However, both the N-S-BA and N-SO-BA angles of Apert syndrome and Crouzon syndrome were narrowed more than that of nonsyndromic bicoronal synostosis (by 12.11°, p<0.001; 12.44°, p<0.001 in Apert syndrome, and by 11.66°, p=0.007; 13.71°, p=0.007 in Crouzon syndrome). However, there is no significantly statisitcal difference of these 2 angles between Apert syndrome and Crouzon syndrome, when they only associated with bicoronal synostosis. Contrary to the relatively normal subcranial space of nonsyndromic bicoronal synostosis, both Apert and Crouzon syndromes developed a reduced subcranial space.
Isolated bicoronal synostosis resulted in a flattened cranial base, while Apert syndrome and Crouzon syndrome developed normal cranial base angle, when only associated with bicoronal synostosis. This suggesting the tendency of folding cranial base of the syndromes. The syndromic skulls had additionally significantly reduced subcranial space.
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