To print: Select File and then Print from your browser's menu --------------------------------------------------------------------------------------- Title: ASA: Conscientious Planning Needed for Anaesthetic Management of Children With Proteus Syndrome URL: http://www.pslgroup.com/dg/23CC2A.htm Doctor's Guide October 22, 2003
By Jill Stein SAN FRANCISCO, CA -- October 22, 2003 -- When planning an anaesthetic for a patient with Proteus syndrome, the anaesthesiologist must be extremely knowledgeable about the nature of the disease and of potentially fatal, disease-related postoperative complications, researchers have advised. The National Institutes of Health (NIH), Bethesda, Maryland, United States, made the recommendations here on October 15th at the 2003 Annual Meeting of the American Society of Anesthesiologists. Dr. Zenaide Quesado and associates from the NIH reviewed findings in 12 children and adolescents with Proteus syndrome who underwent 22 diagnostic and/or surgical procedures requiring anaesthesia. All patients had an abnormal facial phenotype and craniofacial deformities, eleven patients had disproportionate limb overgrowth, four patients had visceral disproportionate overgrowth and nine had vascular malformations. Physical examination and imaging studies showed that all patients had spinal abnormalities. Anaesthesia was induced in eight cases with sevoflurane and in 14 cases with propofol. In 18 procedures, patients underwent endotracheal intubation; one intubation was described as difficult by a paediatric anaesthesiologist. All intra-operative courses were uncomplicated. Postoperatively, all patients did well except for one patient who developed multiple pulmonary emboli 24 hours after an upper and lower gastrointestinal endoscopy and was discharged home after a lengthy postoperative course. Dr. Quezado said the results underscore the importance of careful anaesthetic planning in the patient with Proteus syndrome. "Craniofacial deformities resulting from bone and soft tissue overgrowth can lead to challenging airway management," he said. "In addition, because of the occurrence of adenoid and tonsillar hypertrophy, it is important to be aware of the possibility of sleep apnoea and airway obstruction during anaesthesia induction." Because bone abnormalities can lead to severe asymmetry of limbs, the rib cage and pelvis, careful positioning of these patients is extremely important, he added. Regional anaesthesia should probably be avoided in these patients because of the frequency of vertebral, bone and vascular abnormalities. Nonetheless, Dr. Quezado emphasised that once all the possible co-morbidities in patients with Proteus syndrome are defined and addressed, the intra-operative anaesthetic course in their patients was uncomplicated. However, it is important to have a high index of suspicion for the possibility of deep venous thrombosis and pulmonary emboli, which has been associated with sudden death in this population. [Study title: Anesthetic Management of Children and Adolescents with Proteus Syndrome. Abstract A-1440] --------------------------------------------------------------------------------------------- Copyright © 1999 P\S\L Consulting Group Inc. All rights reserved. Republication or redistribution of P\S\L content is expressly prohibited without the prior written consent of P\S\L. P\S\L shall not be liable for any errors, omissions or delays in this content or any other content on its sites, newsletters or other publications, nor for any decisions or actions taken in reliance on such content. --------------------------------------------------------------------------------------------- This news story was printed from *Doctor's Guide to the Internet* located at http://www.docguide.com --------------------------------------------------------------------------------------- Return to News Story Page This site is maintained by webmaster@pslgroup.com Please contact us with any comments, problems or bugs. All contents Copyright (c) 1998 P\S\L Consulting Group Inc. All rights reserved.