Squamous Suture Craniosynostosis

Unilateral, non-syndromic frontosphenoidal with squamous suture craniosynostosis is a rare form of synostosis, which results in a variable pattern of skull deformities. This paper reviews the spectrum of synostosis, including the involvement of the minor sutures. A case report of frontosphenoidal with squamous suture craniosynostosis is described and the merits of virtual surgical planning discussed.

The sutures of the cranial vault are patent at birth. A thin layer of fibrous tissue connects the bones, which facilitates malleability of the skull through the birth canal, as well as rapid brain growth during the first two years of life. There are major and minor cranial sutures across which growth must occur synchronously to achieve normal skull, skull base and fronto-orbital development. Normal growth is in a plane perpendicular to the suture line. Virchow’s Law describes that premature closure of the suture results in absent growth in the perpendicular plane with compensatory growth parallel to the fused suture. Should one or more sutures close early, inappropriate cranial shape will ensue. Furthermore, restriction of growth may have repercussions for neurological development including intracranial hypertension as explained by the Monroe-Kellie …show more content…
The most common associated syndromes are Crouzon, Apert’s and Pfeiffer; each associated with defective fibroblast growth factor receptor (FGFR) genes. A comprehensive way to view all the sutures is by considering the four arches classification, as depicted in Figure 1. The sagittal arch includes the sagittal, metopic and ethmoidal-frontal. The coronal arch; spheno-squamous, spheno-petrosal, spheno-parietal, frontal-sphenoidal, ethmoido-sphenoidal and coronal. The lambdoid arch consists of the lambdoid, occipito-petrosal and spheno-petrosal. The parieto-squamosal arch includes the parieto-squamous and parieto-mastoid