Overview of craniosynostosis
- Edward P Buchanan, MD
Edward P Buchanan, MD
- Assistant Professor
- Baylor College of Medicine
- Larry H Hollier, Jr, MD
Larry H Hollier, Jr, MD
- Baylor College of Medicine
- Section Editors
- Leonard E Weisman, MD
Leonard E Weisman, MD
- Section Editor — Neonatology
- Professor of Pediatrics
- Baylor College of Medicine
- Helen V Firth, DM, FRCP, DCH
Helen V Firth, DM, FRCP, DCH
- Section Editor — Genetics
- Consultant Clinical Geneticist
- Addenbrooke's Hospital, Cambridge, UK
Interruption of normal embryologic craniofacial differentiation can produce a wide variety of craniofacial abnormalities. Many of the more profound craniofacial deformities occur secondary to craniosynostosis or premature fusion of one or more cranial sutures. Reconstruction of craniofacial structure is typically required when physical or mental well-being becomes affected.
The pathogenesis, diagnosis, and surgical management of craniosynostosis are reviewed here. Specific syndromes associated with craniofacial abnormalities are discussed separately. (See "Craniosynostosis syndromes" and "Syndromes with craniofacial abnormalities".)
The newborn infant's skull is composed of bony plates separated by sutures. This arrangement accommodates transient skull distortion during birth and permits future growth of the brain, the volume of which quadruples during the first two years of life. There are four major sutures: the metopic, coronal, sagittal, and lambdoid. Three additional sutures that contribute to calvarial development are considered minor: the frontonasal, temporosquamosal, and frontosphenoidal. The sagittal, coronal, and metopic sutures meet at the anterior of the skull to form the anterior fontanelle, palpable just behind the forehead at the midline. The posterior fontanelle is formed by the intersection of the sagittal and lambdoid sutures (figure 1).
The osseous cranial base is embryologically derived from a cartilaginous framework (endochondral bone) that undergoes a proliferative growth pattern. In contrast, the calvarium consists of membranous bone, which has no cartilaginous phase. The calvarium grows by depositing new bone along suture lines in response to the distending forces of the rapidly growing brain. During the first two years after birth, the brain increases in size to 75 percent of its adult volume (figure 2). The remaining 25 percent of growth occurs during the next 18 years.
Fontanelle and suture closure occurs in a specific pattern (table 1 and table 2). At two months of age, the posterior fontanelle closes, followed by anterior fontanelle closure at approximately two years. The anterolateral and posterolateral fontanelles close at three months and one year, respectively. While the metopic suture typically closes at two years of age, all remaining patent sutures close in adulthood following completion of craniofacial growth.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CRANIAL ANATOMY
- CATEGORIZATION OF CRANIAL DEFORMITIES
- Other deformities
- Computed tomography
- DIFFERENTIAL DIAGNOSIS OF FACIAL ASYMMETRY
- Positional flattening (positional plagiocephaly)
- Congenital torticollis
- Other causes of facial asymmetry
- Increased intracranial pressure
- SURGICAL APPROACH
- Surgical planning
- - Scaphocephaly
- - Plagiocephaly
- - Trigonocephaly
- - Kleeblattschädel
- Surgical complications