Consensus guideline from the European Malignant Hyperthermia Group on perioperative management of patients with an inherited primary disorder of skeletal muscle.

Abstract

Perioperative abnormalities presenting in patients with inherited primary disorders of skeletal muscle can share similar characteristics to those in a malignant hyperthermia crisis. These patients are often referred to malignant hyperthermia units for perioperative advice as clinicians are accessing conflicting and non-current literature. To address this, the European Malignant Hyperthermia Group (EMHG) has conducted a review of the current literature and used a formal consensus process, with review at annual EMHG meetings and workshops, to provide guidelines for the perioperative management of patients with these myopathies. The guidelines include general recommendations and suggestions applicable to all patients with myopathy requiring anaesthesia or procedural sedation and additional specific recommendations, suggestions and main concerns for patients with specific primary myopathies (ICD-11-CM) with inheritable pathology distal to the neuromuscular junction, including congenital, mitochondrial and metabolic myopathies; muscular dystrophies; myotonias; and familial periodic paralyses.