Limb girdle muscle dystrophies are clinically and genetically heterogeneous group of disorders characterised by progressive weakness and wasting of muscles of the pelvic and shoulder girdles. The prevalence of LGMD has been mapped to 15 chromosomal loci. The incidence is 40 per million. Our objective was to identify the anaesthesia related problems such as respiratory muscle weakness, increased risk of pulmonary aspiration, cardiomyopathy, arrhythmias and risk of rhabdomyolysis. Intraoperative goals were to minimize risk of malignant hyperthermia.

A 30 year old male diagnosed with intestinal obstruction was posted for emergency laparotomy. The patient gave history of progressive weakness of all four limbs since 10 years. Clinical examination revealed presence of waddling gait and decreased power (3/5) in proximal muscles of upper and lower limb. Investigations including blood and urine analysis ECG, echocardiogram, creatine phosphokinase levels were within normal limits. A muscle biopsy which was taken intraoperatively revealed deficiency in alpha and beta subunits and dystrophin. Introperatively standard monitoring including EtCO2, core temperature measurement and neuromuscular monitoring was done. Anaesthesia was maintained with propofol infusion. Use of non-depolarising muscle relaxants and time of reversal were guided by neuromuscular monitoring.

The intraoperative and postoperative period was uneventful

In muscular dystrophies, mutations in transmembrane proteins lead to sarcolemmal instability and predispose to rhabdomyolysis and malignant hyperthermia when succinylscoline or inhalational anaesthetics are used. TIVA and high levels of perioperative monitoring makes administration of anaesthesia safer in these patients.