Anesthesia and Spinal Muscle Atrophy
Highlights
- Spinal muscle atrophy (SMA) is an autosomal recessive Neuromuscular disorder and one of the most common recessive inherited lethal diseases in children. It is characterized by progressive symmetrical muscle weakness (View Highlight)
- Muscles are affected proximally more than distally, legs more than arms (View Highlight)
- Pulmonary complications are the leading cause of anesthetic mortality and morbidity among patients with SMA I and II, but are only rarely seen in patients with SMA III. (View Highlight)
- Pulmonary complications are the dominating perioperative problem (View Highlight)
- Limited mobility of the cervical spine is caused by joint contractures and/or previous spinal surgery (View Highlight)
- Reduced mouth opening is associated with age and is caused by ankylosis of the mandibular joint (View Highlight)
- Gastroesophageal reflux is a phenomenon seen in all patients with SMA I, many with SMA II and some with SMA III (View Highlight)
- Patients with SMA are prone to develop both hypo- and hyperglycemia (View Highlight)
- it is strongly recommended that Suxamethonium be withheld from any patient with Neuromuscular disorder due to the risk of hyperkalaemia. This does apply to patients with SMA, but it should be noted that there are no reports in the literature of hyperkalemic or other adverse responses to succinylcholine in these patients. (View Highlight)
- Prolonged effects of nondepolarizing neuromuscular blockers have been reported, even after reversal (View Highlight)
- neuromuscular monitoring can be unreliable in patients with SMA (View Highlight)