Anaesthesia for a child with α-dystroglycan-related congenital muscular dystrophy

Abstract

α-Dystroglycanopathy is a newly emerging subcategory of autosomal recessive inherited muscular dystrophies which encompasses a wide spectrum of clinical severity. Mutation of at least 18 genes which are responsible for O-mannose glycosylation of α-dystroglycan has been linked to these congenital muscular dystrophy phenotypes. α-Dystroglycan-related congenital muscular dystrophy (αDG-CMD), which may be associated with multisystem involvement, poses a challenge in perioperative management. Yet, there is a dearth of resources available for reference. We report successful anaesthesia for a 6-year-old child with αDG-CMD who underwent bilateral hamstring lengthening, left Achilles tendon lengthening, and above-the-knee fibreglass for bilateral hamstring tightness. Anaesthesia was performed using total intravenous anaesthesia (TIVA) without muscle relaxant. Bilateral sciatic nerve blocks were performed for postoperative pain control, allowing opioid-free analgesia. The patient was extubated at the end of the surgery. Perioperative considerations in αDG-CMD include anticipation of difficult airway, maintenance of thermoregulation and precautions against malignant hyperthermia with the employment of TIVA, techniques that avoid opioids and neuromuscular blocking agents (particularly suxamethonium), as well as minimizing the risk of aspiration and of raised intracranial pressure.