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Virtual Pediatric Hospital: Paediapaedia: Craniosynostosis Paediapaedia: Neurological Diseases

Craniosynostosis

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Irregularly shaped skull.

Etiology/Pathophysiology:
Is premature closure of a suture. Can be either primary (idiopathic) or secondary (due to skeletal dysplasias and syndromes, metabolic disease, hematologic disorders, ventricular shunting, and miscellaneous malformations). The primary ones develop in utero and are apparent at birth or shortly thereafter. There is a 3:1 male predominance.

Pathology:
Not applicable

Imaging Findings:
The skull is altered in shape. The involved suture may appear narrowed, demonstrate parasutural sclerosis, sharpening and straightening of the sutural edges, and development of bony bridges. There may be partial or complete fusion of a suture, but a short segment of synostosis often produces the same deformity as a complete fusion. Multiple sutures are involved 11% of time. Plain films detect 89% of craniosynostosis while CT detects 94% of craniosynostosis.

Sagittal craniosynostosis (dolichocephaly, scaphocephaly) accounts for 56% of cases and has an elongated cranial vault.

Unilateral coronal synostosis (plagiocephaly) accounts for 11% of cases and has a harlequin appearance to the affected orbit.

Bilateral coronal synostosis (brachycephaly) accounts for 11% of cases and has a shortened, widened cranial vault and has a harlequin appearance to both orbits.

Metopic craniosynostosis accounts for 7% of cases, and lambdoid craniosynostosis accounts for 1% of cases.

DDX:

References:
See References Chapter.

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