Congenital Muscular Dystrophy (CMD)

Congenital muscular dystrophy (CMD) has many different forms which may lead to serious problems associated with surgery and anesthesia (1). Although, the sole use of central neuraxial anesthesia and peripheral nerve blocks seem to be safer than general anesthesia in adult patients with CMD), the majority of these techniques require deep sedation or general anesthesia in children (2).
A 6.5-year-old girl with the diagnosis of merosin-positive CMD (weight 23 kg, height 123 cm) was scheduled for surgical correction of bilateral congenital hip subluxation. At preoperative assessment, physical examination revealed muscle atrophy and decreased muscle tone (4/5) at both upper and lower extremities. Preoperative creatine kinase, alanine aminotransferase …show more content…
Depolarizing relaxants are associated with life threatening hyperkalemia and non-depolarizing neuromuscular blocking drugs may lead to a prolonged effect (3). Some controversy about the use of volatile anesthetics and their potential to cause a malignant hyperthermia-like syndrome or rhabdomyolysis exists (4). Therefore, regional anesthesia may offer advantages for avoiding many of these problems with reduced drug doses, avoidance of tracheal intubation and decreased postoperative opioid requirement (5, 6). We used a caudal block which has been shown to have beneficial impact on respiratory physiology in healthy spontaneously ventilating children under propofol infusion with LMA (7). Additionally, we used propofol infusion, because dystrophic patients who are exposed to volatile anesthetics may develop a malignant hyperthermia-like syndrome or rhabdomyolysis (8). To the best of our knowledge, this is the first report in the existing literature to use caudal block combined with general anesthesia using LMA in spontaneously ventilating child with CMD. Neither endotracheal intubation nor neuromuscular agents were required. Additionally, it provided excellent postoperative analgesia with a comfortable