Functional implications of single suture craniosynostosis

Abstract 

The benefit of craniofacial surgery in terms of form and function in the craniofacial dysostoses, such as Apert and Crouzon syndromes, as well as in severe multiple suture synostosis, is seldom questioned. However, the role of surgery in isolated single suture craniosynostosis (and non-syndromic bicoronal synostosis) is less defined. It is often misperceived as being merely for ‘cosmetic’ benefit.

This article addresses the question of functional implications in these patients. Direct comparison of studies in the literature is difficult, due to low numbers, differences in systematic data presented, as well as differences in definitions used. It documents that while the benefit in terms of ‘Form’ is substantial, single suture craniosynostosis is a complex clinical entity with more at stake than purely the ‘cosmetic’ appearance of the child. The risk of ‘subclinical’ raised intracranial pressure as well as effects on psychosocial development and function (including potential learning disability and behavioural deficits) are highlighted. The possibility of a reversible underlying cerebral hypoperfusion defect is discussed. The potential consequences of late referral in terms of functional risks and surgical management are presented.

Based on the evidence, a plea is made for early referral for assessment and regular follow-up by an experienced multidisciplinary team. This should minimize the risk of inappropriate conservative treatment as well as minimizing the morbidity and optimizing the results in cases where surgery is appropriate.

Keywords: craniosynostosis, craniofacial, functional outcome, intracranial pressure, cognitive function, intelligence, coronal, sagittal, metopic, scaphocephaly, plagiocephaly, brachycephaly, trigonocephaly, cerebral perfusion studies

No full text is available. To read the body of this article, please view the PDF online.

• f1 Correspondence to: SAW. Tel.: +44 (0)1865 224100; Fax: +44 (0)1865 311673