Experiences with a reference directory for the perioperative care of children with rare diseases

Abstract

SummaryA computerized reference data bank for the anaesthetic management and postoperative care of children with syndromes and rare disorders was established two years ago. It contains data on some 2000 syndromes and rare diseases. Updating of the directory is done by regular review of the literature and dialogue with the users. Between January 1991 and January 1993 this interdisciplinary service received 169 telephone, fax or postal calls for advisory service from physicians of all specialities connected with the field of operative paediatrics. The consultations concerned 79 patients with dysmorphias, 58 infants with metabolic disorders and 32 patients with complex or unclassified syndromes. About 58% of the patients were aged under three years. Although the structure of the data bank allows for an advisory service tailored to the individual needs, only 63% of the consultations could be given based upon published anaesthetic experiences of similiar cases. In the absence of anaesthetic case reports, advice for the other cases had to derived from the principles of general paediatric anaesthesia practice. In only 48 of the consultations (28%) was the user's experience reported and could then be stored in the directory.