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Chapter 24 - Craniofacial Neurosurgery

from Section 2 - Clinical Neurosurgical Diseases

Published online by Cambridge University Press:  04 January 2024

By
John T. Smetona  and
John A. Persing
Edited by
Farhana Akter ,
Nigel Emptage ,
Florian Engert  and
Mitchel S. Berger
Farhana Akter
Affiliation:
Harvard University, Massachusetts
Nigel Emptage
Affiliation:
University of Oxford
Florian Engert
Affiliation:
Harvard University, Massachusetts
Mitchel S. Berger
Affiliation:
University of California, San Francisco
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Summary

Craniosynostosis is a condition associated with the pathologic premature fusion of one or more cranial sutures. Physiologically, the metopic suture closes in infancy, while the remaining sutures close years later, even into adulthood. In craniosynostosis, characteristic calvarial deformity first appears on ultrasound in the second trimester and precedes identifiable suture fusion by 4–16 weeks. When this premature closure occurs, it is associated with restriction of calvarial growth perpendicular to the fused suture, with compensatory increase in growth at the remaining sutures. Previously there was debate as to whether the suture fusion itself drives this restriction of growth, or whether a cranial base deformity drives the abnormal development through tension bands in the dura. Currently there is a preponderance of evidence from human and rabbit studies supporting the idea that suture fusion is at least a significant contributor to the overall skull shape abnormality. The natural history of the disease is such that the deformity observed in infancy increases in severity if not surgically corrected.

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Neuroscience for Neurosurgeons , pp. 326 - 334
Publisher: Cambridge University Press
Print publication year: 2024

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