title: Perioperative care of a child with Ullrich congenital muscular dystrophy.
abstract: Ullrich congential muscular dystrophy (UCMD) is a severe form of congenital muscular dystrophy manifesting axial muscle contractures and distal joint hyperlaxity. Severe hypotonia and associated respiratory failure may occur early in the disease process. Given the various associated orthopedic 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 blocking agent for rapid sequence intubation, associated airway issues related to micrognathia and limited 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.
authors: University of Missouri School of Medicine, Columbia, Missouri, USA.
authors: Neesann
authors: N
authors: Puangsuvan
authors: Robert A
authors: RA
authors: Mester
authors: Venkataraman
authors: V
authors: Ramachandran
authors: Joseph D
authors: JD
authors: Tobias
mesh_terms: D018686:Anesthetics, Intravenous / Q000627:therapeutic use; D001062:Appendectomy / Q000379:methods*; D001064:Appendicitis / Q000601:surgery*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D009136:Muscular Dystrophies / Q000150:complications*; D019990:Perioperative Care; D010880:Piperidines / Q000627:therapeutic use; D015742:Propofol / Q000627:therapeutic use; D000077208:Remifentanil; D012422:Rupture, Spontaneous
chemical_list: D018686:Anesthetics, Intravenous; D010880:Piperidines; D000077208:Remifentanil; D015742:Propofol