Anesthesia in pediatric patients with spinal muscular atrophy

Overview

Modern causal therapies have significantly improved motor function and reduced the risk of pulmonary complications in patients with spinal muscular atrophy. However, the genetic basis of the disease remains unchanged, and therefore these patients continue to represent a high--risk group requiring individualized perioperative management. Progressive muscle weakness and skeletal deformities lead to restrictive ventilatory dysfunction and airway rigidity. Preoperative evaluation therefore focuses on pulmonary function, cardiac status, airway management (risk of difficult intubation), and nutritional status (risk of hypoglycemia and mitochondrial dysfunction during fasting). When selecting anesthesia, a multimodal approach and ultrasound-guided regional techniques are preferred to minimize opioid use. The administration of succinylcholine is contraindicated due to the risk of hyperkalemia. Non-depolarizing neuromuscular blockers carry a risk of prolonged effects; the safe standard is a combination of rocuronium with targeted reversal using sugammadex under continuous train-of-four monitoring. The postoperative period is the highest-risk phase of perioperative care for pediatric patients with spinal muscular atrophy due to the threat of respiratory failure. Monitoring in the intensive care unit, active physical therapy, the use of noninvasive ventilation, and capnography monitoring are crucial, as excessive oxygen therapy can mask hypoventilation. Safe care is ensured by an experienced multidisciplinary team.

Keywords:

spinal muscular atrophy – perioperative care – pediatric anesthesia