Spinal Muscular Atrophy Type II: Anesthetic Challenges and Perioperative Management

Abstract

AbstractSpinal muscular atrophy (SMA) is a group of genetic diseases that cause weakness and wasting in voluntary muscles of infants and children and more rarely in adults. It is caused by inadequate production of a protein called survival motor neuron (SMN) protein coded by SMN1 gene on chromosome 5. Anesthetic challenges in patients of SMA include their sensitivity toward induction agents such as thiopental, opioids, muscle relaxants, and their incomplete reversal with anticholinesterases. They also have weakness of respiratory muscles and inadequate laryngeal reflexes, which make them prone to aspiration. Although spinal and epidural anesthesia have been described in adults with SMA, but general anesthesia in these patients is very challenging. In this case, successful perioperative management of such a patient scheduled for correction of dislocated hip has been described.