Anesthesia for videolaparoscopic cholecystectomy in a patient with Steinert disease. Case report and review of the literature.
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.
Bisinotto
Flora Margarida Barra
FM
UFTM. flora@mednet.com.br
Fabri
Daniel Capucci
DC
Calçado
Maida Silva
MS
Perfeito
Paula Borela
PB
Tostes
Lucas Vieira
LV
Sousa
Gabriela Denardi
GD
D000328:Adult; D000758:Anesthesia / Q000379:methods*; D017081:Cholecystectomy, Laparoscopic / Q000379:methods; D006801:Humans; D008297:Male; D009223:Myotonic Dystrophy; D020535:Video-Assisted Surgery