Clinical Features of Syndromic Craniosynostosis

Rice, David

doi:10.1159/000115034

Cited by 41 publications

(15 citation statements)

References 47 publications

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“…However, most facial sutures remain patent until late adulthood, e.g. the frontomaxillary, nasomaxillary and zygomaticomaxillary, which start to fuse until the 7th or 8th decade of life (Rice, 2008). Interestingly, the facial skeleton continues growing into adulthood, whereas the neurocranium and cranial base by that time have already stopped growing Edwards et al 2007;Holton & Franciscus, 2008).…”

Section: Postnatal Changes In the Growth Dynamics Of The Human Facementioning

confidence: 99%

Postnatal changes in the growth dynamics of the human face revealed from bone modelling patterns

2013

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Human skull morphology results from complex processes that involve the coordinated growth and interaction of its skeletal components to keep a functional and structural balance. Previous histological works have studied the growth of different craniofacial regions and their relationship to functional spaces in humans up to 14 years old. Nevertheless, how the growth dynamics of the facial skeleton and the mandible are related and how this relationship changes through the late ontogeny remain poorly understood. To approach these two questions, we have compared the bone modelling activities of the craniofacial skeleton from a sample of subadult and adult humans. In this study, we have established for the first time the bone modelling pattern of the face and the mandible from adult humans. Our analyses reveal a patchy distribution of the bone modelling fields (overemphasized by the presence of surface islands with no histological information) reflecting the complex growth dynamics associated to the individual morphology. Subadult and adult specimens show important differences in the bone modelling patterns of the anterior region of the facial skeleton and the posterior region of the mandible. These differences indicate developmental changes in the growth directions of the whole craniofacial complex, from a predominantly downward growth in subadults that turns to a forward growth observed in the adult craniofacial skeleton. We hypothesize that these ontogenetic changes would respond to the physiological and physical requirements to enlarge the oral and nasal cavities once maturation of the brain and the closure of the cranial sutures have taken place during craniofacial development.

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Section: Postnatal Changes In the Growth Dynamics Of The Human Facementioning

confidence: 99%

Postnatal changes in the growth dynamics of the human face revealed from bone modelling patterns

2013

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“…Even though sutures seem to have little functional roles when compared with the long bone joints such as knees or wrists, they are major actors in the morphogenesis of the head of newborns. In fact, after birth, most skull growth is owing to the action of sutures [2], and clinicians are well aware that abnormal suture maintenance and premature fusion can lead to remarked skull deformations known as craniosynostoses [3].…”

Section: Introductionmentioning

confidence: 99%

A mathematical model for mechanotransduction at the early steps of suture formation

Khonsari

Olivier²,

Vigneaux

et al. 2013

Proc. R. Soc. B.

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Growth and patterning of craniofacial sutures is subjected to the effects of mechanical stress. Mechanotransduction processes occurring at the margins of the sutures are not precisely understood. Here, we propose a simple theoretical model based on the orientation of collagen fibres within the suture in response to local stress. We demonstrate that fibre alignment generates an instability leading to the emergence of interdigitations. We confirm the appearance of this instability both analytically and numerically. To support our model, we use histology and synchrotron X-ray microtomography and reveal the fine structure of fibres within the sutural mesenchyme and their insertion into the bone. Furthermore, using a mouse model with impaired mechanotransduction, we show that the architecture of sutures is disturbed when forces are not interpreted properly. Finally, by studying the structure of sutures in the mouse, the rat, an actinopterygian (Polypterus bichir) and a placoderm (Compagopiscis croucheri), we show that bone deposition patterns during dermal bone growth are conserved within jawed vertebrates. In total, these results support the role of mechanical constraints in the growth and patterning of craniofacial sutures, a process that was probably effective at the emergence of gnathostomes, and provide new directions for the understanding of normal and pathological suture fusion.

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“…Clinical signs of CS and subsequent craniostenosis usually include increase in the in tracranial pressure, mental retardation, protrusion of the eyeballs, hypoplasia of the jaw and even hydrocephalus (Kobus and Łątkowski, 2005;Laskowska-Ziętek and Misiuk-Hojło, 2007;Rice, 2008).…”

Section: Discussionmentioning

confidence: 99%