Skull Base Growth in Craniosynostosis

Abstract

Although considerable scientific work has been published on the role of the skull base in craniosynostosis, the changes with age throughout childhood have not been fully outlined. The realisation that little attention has been paid to the posterior skull in craniosynostosis, resulted in renewed interest in skull base growth. The availability of computer-based image analysis provides a new accurate method of study in three dimensions. Using three-dimensional visualisation techniques, 34 points of the skull base were identified on CT scans of 50 children with craniosynostosis of various types, aged from 1 month to 5 years. Several distances and angles between the various landmarks were measured in an attempt to quantify the growth of skull fossae with age. Comparisons were made with normal controls. In children with craniosynostosis, the anterior fossa was overdeveloped in the males, whereas in the females remained underdeveloped throughout the first 2 years of life. The body of the sphenoid showed moderate underdevelopment in the first 2 years in both sexes, the effect being more prominent in the males. The middle fossae showed overdevelopment in both sexes in the first 2 years of life. The posterior fossa was underdeveloped in both sexes in the first 2 years of life, the effect being more prominent in the females. Craniosynostosis seems to affect both sexes to a similar degree, but there are regional differences in the growth pattern. Better understanding of the normal growth pattern of the skull base and the effect of craniosynostosis upon it may assist our approach to surgical treatment and in particular the role of anterior and posterior skull expansive surgery.

The role of the posterior fossa in developing Chiari I malformation in children with craniosynostosis syndromes

“…Studies that included the cerebellum and PF consisted of cephalometric analyses of radiographs or CT scans. Those few studies that have focussed on the PF, presented a smaller anterior-posterior diameter in bicoronal craniosynostosis patients, suggesting its volume to be smaller (Richtsmeier et al, 1991;Sgouros et al, 1999). However, since the skull is deformed in craniosynostosis patients, the brain and cerebellum are deformed as a consequence.…”

Occipital plagiocephaly: unilateral lambdoid synostosis versus positional plagiocephaly

“…This condition can lead to altered venous drainage or higher intravenous pressure and hydrocephalus. In non-syndromic craniosynostosis, the skull base synchondroses are typically not involved 40. Nevertheless, regular follow-ups, including a hydrocephalus workup, should be mandatory.…”

Foramen Magnum Size and Involvement of Its Intraoccipital Synchondroses in Crouzon Syndrome

“…22,23 In addition, animal studies have revealed that FGFR2 is expressed at the neural tube and cranial base during embryogenesis. [24][25][26][27] Several studies reported abnormalities of the skull 2,5,9,28,29 and skull base 9,13,30 in Crouzon syndrome in humans and in mice. [31][32][33] Thus, it is likely that FGFR2 gene mutations have an effect on hindbrain development and give rise to abnormal growth of its surrounding bone plates (i.e., the foramen magnum).…”