RESUMEN
Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics administered in 29 patients. Twelve of the anaesthetics (11.1%) were associated with cardiac complications including cardiac arrest in two cases (1.85%). Of the two cardiac arrests, one patient died within the first 24 hours postanaesthetic and the other patient survived, giving an overall mortality of 0.9% (3.4%). We conclude that Williams syndrome confers a significant anaesthetic risk, which should be recognised and considered by clinicians planning procedures requiring general anaesthesia.
Asunto(s)
Anestesia/efectos adversos , Paro Cardíaco/etiología , Síndrome de Williams/complicaciones , Adolescente , Adulto , Niño , Preescolar , Femenino , Hemodinámica , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
This cross-sectional survey was designed to evaluate the current practice of anaesthetists in Australia and New Zealand with regard to aseptic technique and needle type during the performance of single-shot caudal blocks. Professional bodies suggest that full aseptic precautions be taken during the administration of caudal or epidural blocks. It has been suggested that using an intravenous cannula or a styletted needle may obviate the occurrence of epidermoid tumours. A total of 202 members of the Society for Paediatric Anaesthesia in New Zealand and Australia were invited to participate in this internet-based survey. Eighty-four responses were received. Most respondents used some form of antiseptic handwash (81%), wore sterile gloves (85.7%), used antiseptic skin preparation (100%) and draped the site (57.1%). When performing caudal blocks, 43.1% used unstyletted needles, 27.2% used styletted spinal needles and 29.6% used intravenous cannulas. However, 11.9% did not wash hands, 10.7% did not wear gloves and 42.9% did not drape the site. Three respondents reported neither handwashing, wearing gloves or draping, instead only using an alcohol swab for skin preparation. The majority of respondents in our region appear to use some level of aseptic precautions, albeit to a variable degree. Published recommendations may either be perceived as overly cautious or as ambiguous in that they do not specify caudal practice as distinct from other epidural blocks. There is a need for clearer professional guidance to support a minimum level of aseptic precaution for single-shot caudal epidural blocks.