Da Costa, Annette Celine (2004) Neuropsychological profiles of children and adolescents with craniosynostosis. Other Degree thesis, Victoria University of Technology.

Abstract

Craniosynostosis refers to the premature fusion of one or more cranial sutures, and typically occurs in utero. The major craniofacial disorders that feature craniosynostosis are the syndromic craniosynostoses, in which raniosynostosis occurs within a broader symptom complex (e.g. cardiac, respiratory and hearing impairments), and the nonsyndromic craniosynostoses, in which craniosynostosis presents in isolation. Reconstructive surgery to remodel the cranium is required in most cases, normally between 6 to 12 months of age. The risks of adverse central nervous system and cognitive sequelae are well documented in these conditions. Whilst the syndromic craniosynostoses have historically been associated with varying degrees of intellectual impairment, the nonsyndromic forms have been regarded to have relatively benign cognitive sequelae. There is limited empirical literature addressing the long-term neuropsychological consequences of these disorders. Of that available, cognitive fuctions beyond global intelligence have seldom been examined, and no prior studies have comprehensively evaluated the wide array of cognitive skills that are important for adaptive functioning utilising standardised psychometric tools. This cross-sectional study sought to address these gaps in the developmental literature on the craniosynostoses and comprehensively describe the neuropsychological profiles of 34 children and adolescents with syndromic (n=13) and nonsyndromic (n=21) craniosynostosis. Children of between 7 and 16 years of age (mean age 11 years) completed a battery of neuropsychological tests, measuring intelligence, attention, memory and leaming, academic, executive and social and behavioural functioning.

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