RESUMO
BACKGROUND AND PURPOSE: Myotonic dystrophy type 2 (DM2) is an adult-onset progressive multisystem disease. There have been no reported risks for anesthesia in DM2. METHODS: We assess the frequency, type, and severity of peri-operative complications under general and local anesthesia in genetically proven DM2. A retrospective multicenter study was conducted. RESULTS: Out of 320 DM2 patients, 134 participated by completing questionnaires (41, 88%), which were delivered by mail, and their clinical records were reviewed (class III evidence). A total of 121 patients had 340 operations in general anesthesia at an average age of 40.5 years (range 18-82); 132 (38.8%) general anesthesia were performed prior to DM2 onset, 187 (55.9%) after disease onset. A total of 212 (62.4%) of the interventions were performed without known DM2 diagnosis. In 120 (35.3%) interventions, DM2 was already diagnosed. The locations of surgery were lower abdomen (47%), peripheral extremities (46.8%), upper abdomen (3.8%), thorax (1.8%), and brain (0.6%). The overall frequency of severe complications was 0.6% (2 of 340). One incident was a post-operative development of rhabdomyolysis, hyperthermia, muscle weakness and renal failure; the others, prolonged muscular weakness and renal failure. Minor complications related to a general anesthesia were reported by 27 participants (20.2%). In 116 patients (86.6%), 342 interventions were performed in regional anesthesia. Minor complications were reported by 20.2% participants such as nausea (6.7%), muscular weakness and pain (5.9%), prolonged anesthesia (5.2%), circulatory insufficiency (2.9%), and shortness of breath (2.9%). CONCLUSION: The overall lower risk seems to be predominantly related to the minor respiratory involvement in DM2, than in myotonic dystrophy type 1 (DM1).
