Author/Authors :
Puangsuvan, Neesann University of Missouri - School of Medicine, USA , Mester, Robert A University of Missouri - School of Medicine, USA , Ramachandran, Venkataraman University of Missouri - School of Medicine - Departments of Surgery, USA , Tobias, Joseph D University of Missouri - School of Medicine - Department of Anesthesiology, Department of Pediatrics, USA
Abstract :
Ullrich congential muscular dystrophy (UCMD) is a severe form of congenital musculardystrophy manifesting axial muscle contractures and distal joint hyperlaxity. Severe hypotonia andassociated respiratory failure may occur early in the disease process. Given the various associatedorthopedic conditions, anesthetic management may be required during surgical interventions to correct skeletal deformities or these patients may present with surgical conditions unrelated to their primary illness. We present a 4-year-old with UCMD who required operative intervention for a ruptured appendix. Anesthetic care implications included the need for a rapid airway control to limit the risks of aspiration due to the intra-abdominal process, choice of neuromuscular locking agent for rapid sequence intubation, associated airway issues related to micrognathia and imited mouth opening, and the potential for involvement of the cardiovascular and respiratory systems. The perioperative management of patients with UCMD is discussed including the use of propofol and remifentanil for rapid sequence intubation to avoid the need for neuromuscular blocking agents.
