title: Anesthesia for videolaparoscopic cholecystectomy in a patient with Steinert disease. Case report and review of the literature.
abstract: OBJECTIVE
  Myotonic dystrophies are autosomal dominant neuromuscular diseases. Among them, myotonic dystrophy type 1 (MD1), or Steinert disease, is the most common in adults, and besides muscular involvement it also has important systemic manifestations. Myotonic dystrophy type 1 poses a challenge to the anesthesiologist. Those patients are more sensitive to anesthetics and prone to cardiac and pulmonary complications. Besides, the possibility of developing malignant hyperthermia and myotonic episodes is also present.
  
  
  METHODS
  This is a 39-year old patient with DM1 who underwent general anesthesia for videolaparoscopic cholecystectomy. Total intravenous anesthesia with propofol, remifentanil, and rocuronium was the technique chosen. Intercurrences were not observed in the 90-minute surgical procedure, but after extubation, the patient developed respiratory failure and myotonia, which made tracheal intubation impossible. A laryngeal mask was used, allowing adequate oxygenation, and mechanical ventilation was maintained until full recovery of the respiratory function. The patient did not develop further complications.
  
  
  CONCLUSIONS
  Myotonic dystrophy type 1 presents several particularities to the anesthesiologist. Detailed knowledge of its systemic involvement along with the differentiated action of anesthetic drugs in those patients will provide safer anesthetic-surgical procedure.
authors.affiliation: UFTM. flora@mednet.com.br
authors.forename: Flora Margarida Barra
authors.initials: FM
authors.lastname: Bisinotto
authors.forename: Daniel Capucci
authors.initials: DC
authors.lastname: Fabri
authors.forename: Maida Silva
authors.initials: MS
authors.lastname: Calçado
authors.forename: Paula Borela
authors.initials: PB
authors.lastname: Perfeito
authors.forename: Lucas Vieira
authors.initials: LV
authors.lastname: Tostes
authors.forename: Gabriela Denardi
authors.initials: GD
authors.lastname: Sousa
mesh_terms: D000328:Adult; D000758:Anesthesia / Q000379:methods*; D017081:Cholecystectomy, Laparoscopic / Q000379:methods; D006801:Humans; D008297:Male; D009223:Myotonic Dystrophy; D020535:Video-Assisted Surgery