Pressure-support ventilation in a child with merosin-deficient congenital muscular dystrophy under sevoflurane...

Merosin-deficient congenital muscular dystrophy (MD-CMD) is the most common and severe form of congenital muscular dystrophy and is characterized by progressive severe hypotonia due to the absence of the merosin chain around muscle fibers. The main anesthetic concerns include a possible association with malignant hyperthermia, the risk of anesthesia-induced rhabdomyolysis, a difficult airway and postoperative respiratory failure. We report the case of an uneventful general anesthesia (GA) in a two-year-old boy with MD-CMD for the placement of an implantable venous access system. The goal of our anesthetic management was to reduce the risk of respiratory depression. We considered the possibility of loss of spontaneous ventilation against the known, but rare, risk of rhabdomyolysis and we choose for a balanced GA with sevoflurane, short acting opioids and a pressure support ventilation mode instead of a trigger-free anesthesia. Our anesthetic management and the perioperative concerns for this particular syndrome are described.