Background <p>Spinal muscular atrophy (SMA) linked to chromosome 5q demonstrates an autosomal recessive inheritance pattern. SMA is characterized by diffuse, symmetric, proximal muscle weakness, with lower limb predominance in the lower limbs than in the upper limbs, and by absent or markedly diminished deep tendon reflexes. The disease incidence ranges 0.005–0.013% among live births. Pediatric anesthesia management in SMA remains scarcely documented. There are scarcely documented of anesthesia management in patients with SMA, particularly among children and adolescents. Given the generalized muscle atrophy in these patients, anesthesiologists are often cautious about administering general anesthesia with neuromuscular blocking agents (NMBAs). Consequently, many anesthesiologists have explored alternative anesthetic techniques that avoid the use of NMBAs while ensuring that there is no interference with the surgical procedure. Particularly for laparoscopic procedures, which require a certain degree of muscle relaxation, the use of NMBAs remains underreported, with no relevant cases documented in pediatric patients.</p> Case presentation <p>We report the successful administration of general anesthesia in a child with SMA type II at our institution, in which an NMBA was used and the child was uneventful extubation without associated respiratory complications.</p> Conclusions <p>Pediatric patients with SMA may experience unexpected airway difficulties, necessitate careful preparation and consideration of awake intubation. NMBAs should be used cautiously, while nondepolarizing NMBAs and sugammadex demonstrate clinical viability. The benefits of fast-track anesthesia warrant further study, and anesthetic plans should be tailored on the basis of a thorough assessment of the patient’s condition.</p>

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Laparoscopic fundoplication under general anesthesia in a child with spinal muscular atrophy type II: a case report

  • Lufeng Yang,
  • Zugang Yi,
  • Fang Chen

摘要

Background

Spinal muscular atrophy (SMA) linked to chromosome 5q demonstrates an autosomal recessive inheritance pattern. SMA is characterized by diffuse, symmetric, proximal muscle weakness, with lower limb predominance in the lower limbs than in the upper limbs, and by absent or markedly diminished deep tendon reflexes. The disease incidence ranges 0.005–0.013% among live births. Pediatric anesthesia management in SMA remains scarcely documented. There are scarcely documented of anesthesia management in patients with SMA, particularly among children and adolescents. Given the generalized muscle atrophy in these patients, anesthesiologists are often cautious about administering general anesthesia with neuromuscular blocking agents (NMBAs). Consequently, many anesthesiologists have explored alternative anesthetic techniques that avoid the use of NMBAs while ensuring that there is no interference with the surgical procedure. Particularly for laparoscopic procedures, which require a certain degree of muscle relaxation, the use of NMBAs remains underreported, with no relevant cases documented in pediatric patients.

Case presentation

We report the successful administration of general anesthesia in a child with SMA type II at our institution, in which an NMBA was used and the child was uneventful extubation without associated respiratory complications.

Conclusions

Pediatric patients with SMA may experience unexpected airway difficulties, necessitate careful preparation and consideration of awake intubation. NMBAs should be used cautiously, while nondepolarizing NMBAs and sugammadex demonstrate clinical viability. The benefits of fast-track anesthesia warrant further study, and anesthetic plans should be tailored on the basis of a thorough assessment of the patient’s condition.