title: Congenital syndromes in paediatric anaesthesia: what is important to know.
abstract: The purpose of this review is to re-evaluate our approach to the perioperative management of children with congenital syndromes, as a result of recent advances in the understanding of the role of genetic factors in the aetiology of specific syndrome complexes, and in accordance with current paediatric anaesthetic practice. Recent information elucidating the genetic basis of these syndromes will be examined and made relevant to paediatric anaesthetists in their emerging roles as perioperative physicians. Diagnostic testing and family counseling, as well as alterations in reactions to drugs will be considered. Finally, the current year of clinical practice at this hospital will be reviewed to determine the frequency that congenital syndromes are encountered, the most common clinical challenges they present, and to provide examples of the management of anaesthesia in selected cases. The review aims to facilitate the anaesthetist's preoperative assessment of a child with a congenital syndrome, suggest approaches to clinical anaesthetic management, and be a basis for further study of the influence of genetics in anaesthesia.
authors.affiliation: Department of Anaesthesia, British Columbia's Children's Hospital, Vancouver, Canada.
authors.forename: J C
authors.initials: JC
authors.lastname: Bevan
mesh_terms: D000758:Anesthesia / Q000379:methods*; D002648:Child; D002869:Chromosome Aberrations; D000013:Congenital Abnormalities / Q000601:surgery*; D005786:Gene Expression Regulation; D015894:Genome, Human; D006801:Humans; D013577:Syndrome