V Jensen1. 1. Department of Anaesthesia, Walter Mackenzie Centre, University of Alberta Hospitals, Edmonton.
Abstract
PURPOSE: This case report presents a patient with Emery-Dreifuss Muscular Dystrophy and describes the anaesthetic considerations. CLINICAL FEATURES: The features of Emery-Dreifuss Muscular Dystrophy are contractures, humeroperoneal muscle weakness and cardiomyopathy. The anaesthetic considerations for this syndrome are difficult tracheal intubation, difficult spinal anaesthetic, heart block, gastric reflux, rhabdomyolysis, and unproved malignant hyperthermia susceptibility. CONCLUSION: The major anaesthetic problem for the patient with Emery-Dreifuss Muscular Dystrophy could be a life-threatening cardiomyopathy.
PURPOSE: This case report presents a patient with Emery-Dreifuss Muscular Dystrophy and describes the anaesthetic considerations. CLINICAL FEATURES: The features of Emery-Dreifuss Muscular Dystrophy are contractures, humeroperoneal muscle weakness and cardiomyopathy. The anaesthetic considerations for this syndrome are difficult tracheal intubation, difficult spinal anaesthetic, heart block, gastric reflux, rhabdomyolysis, and unproved malignant hyperthermia susceptibility. CONCLUSION: The major anaesthetic problem for the patient with Emery-Dreifuss Muscular Dystrophy could be a life-threatening cardiomyopathy.
Authors: A Staiano; E Del Giudice; A Romano; M R Andreotti; L Santoro; G Marsullo; P G Rippa; A Iovine; M Salvatore Journal: J Pediatr Date: 1992-11 Impact factor: 4.406
Authors: Frank Schuster; Carsten Wessig; Christoph Schimmer; Stephan Johannsen; Marc Lazarus; Ivan Aleksic; Rainer Leyh; Norbert Roewer Journal: Case Rep Anesthesiol Date: 2012-09-03