Craniosynostosis refers to premature closure of the cranial sutures. The skull shape then undergoes characteristic changes depending on which suture(s) close early.
- brachycephaly : bicoronal and/or bilamboid sutures
- scaphocephaly / dolichocephaly : sagittal suture
- plagiocephaly : unilateral coronal and lambdoid sutures
- trigonocephaly : metopic suture
- pachycephaly : lamdoid suture
- oxycephaly / turricephaly : sagittal, coronal and lambdoid sutures (Tower like skull)
- cloverleaf skull / Kleeblattschadel : intrauterine sagittal, coronal, lambdoid sutures : most severe
- harlequin eye : ipsilateral coronal suture
The sagittal suture is most commonly involved ( ≈ 50%), where lateral growth of the skull is arrested while anteroposterior growth continues, producing a narrow elongated skull known as scaphocephaly (meaning boat-shaped) or dolichocephaly.
The next commonest sutures in terms of involvement are
- coronal : ~ 20%
- lambdoid : ~ 5 %
- metopic : ~ 5 %
Causes are primary, or secondary to certain haematologic disorders, metabolic disorders, bone dysplasias and syndromes. There is a 3:1 male predominance.
Most occur as isolated anomalies but syndromic associations can be seen a small proportion of cases (~ 10%) . They include
- acrocephalosyndactyly / acrocephalopolysyndactly types
- choanal atresia
Treatment is often with a cranioplasty.
- 1. Blickman JG, Parker BR, Barnes PD. Pediatric radiology, the requisites. Mosby Inc. (2009) ISBN:0323031250. Read it at Google Books - Find it at Amazon
- 2. Dähnert W. Radiology review manual. Lippincott Williams & Wilkins. (2007) ISBN:0781738954. Read it at Google Books - Find it at Amazon
- 3. Benson ML, Oliverio PJ, Yue NC et-al. Primary craniosynostosis: imaging features. AJR Am J Roentgenol. 1996;166 (3): 697-703. AJR Am J Roentgenol (abstract) - Pubmed citation
- 4. Glass RB, Fernbach SK, Norton KI et-al. The infant skull: a vault of information. Radiographics. 24 (2): 507-22. doi:10.1148/rg.242035105 - Pubmed citation
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