Nonsyndromic Craniosynostoses

Abstract

The subtypes of nonsyndromic ('isolated') craniosynostosis are denominated according to the predominant deformity resulting from premature fusion of one of the major cranial sutures: scaphocephaly, trigonocephaly, anterior plagiocephaly, brachycephaly, posterior plagiocephaly, and oxycephaly. In most cases the underlying causes remain unknown although there is some overlap with syndromic craniosynostosis suggesting heterogeneous etiologies. In contrast to the oversimplified nomenclature, isolated craniosynostosis may involve two or more sutures at the same time or may progress with increasing age, thus indicating that the basic nature of craniosynostosis is a failure of growth regulation of the skull rather than a malformation.

CLARITY: Co‐occurrences in achondroplasia—craniosynostosis, seizures, and decreased risk of diabetes mellitus

“…This contrasts dramatically with craniosynostosis in the general population, in which it is estimated that about 90% of occurrences are single sutural (Collmann et al, 2011). In addition, sutural involvement in achondroplasia seems quite different from the distribution in the general population.…”

“…If one accepts the assumptions noted above, then of the instances described here 6 and possibly 7 of 9 are multisutural, and of those in the literature, not including the duplicated case described by Albino et al (2015) 8 of 11 were similarly complex craniosynostoses. This contrasts dramatically with craniosynostosis in the general population, in which it is estimated that about 90% of occurrences are single sutural (Collmann et al, 2011). In addition, sutural involvement in achondroplasia seems quite different from the distribution in the general population.…”

“…In addition, sutural involvement in achondroplasia seems quite different from the distribution in the general population. In the latter, isolated sagittal sutural involvement accounts for about ½ of all craniosynostosis (Collmann et al, 2011; Greenwood et al, 2014; Lattanzi et al, 2017), while lambdoidal involvement, for example, is quite rare (Greenwood et al, 2014). This is in contrast to what is shown in Tables 1 and 3.…”

Isolated Sagittal Craniosynostosis: A Comprehensive Review

“…At the same time, the results of the various studies are very heterogeneous, as children with different initial stages of deformity, different ages, and different surgical techniques were included in the studies. In any case, all studies describe an improvement in CI after surgery, and it is generally recognised that the lateral, biparietal head circumference increases after the surgery while the anteroposterior head circumference decreases to a lesser extent [74].…”

“…In the second series, which included 181 children operated for ISS, eleven children had to be reoperated due to proven recurrent synostosis [54,[57][58][59][60]. Clinical and radiological evidence of increased intracranial pressure was found in six cases [74]. It should certainly be noted that in some of these children, sagittal craniosynostosis was part of the syndromic disease and that the predisposition for the occurrence of increased intracranial pressure was, therefore, increased in these children.…”