TABLE 3.

Specific anaesthesia recommendations for myotonic dystrophy types 1 and 2

Recommendations	SIGN	Median score, modified Delphi process	Respondents who scored ≥7, n	Abstained from voting, n
Preoperative recommendations
Preoperative screening should include ECG, echocardiography, and lung function tests (in sitting and the supine position), with review of current medication and therapies (e.g., pacemakers, noninvasive ventilation). A neurological examination should be performed to document the degree of muscle weakness, as proximal weakness indicates a greater risk of complications in DM1 [72, 73].	2−	9	19/19 (100%)	1
CNS involvement manifesting as lack of initiative and compliance may complicate preoperative counselling [74].	2−	9	19/19 (100%)	1
Consider antacid treatment as aspiration prophylaxis due to gastrointestinal dysmotility [42, 43].	4	7	13/17 (76.5%)	3
Intraoperative
Regional anaesthesia, TIVA, and volatile anaesthetics have been used effectively and safely, although rigidity cannot always be prevented [42, 43, 75, 76].	3	9	15/15 (100%)	5
Use of opioids should be avoided if possible, as there is increased sensitivity to such drugs, with an increased risk of respiratory [77] (and possibly intestinal) complications [72]; if this is not possible, use short‐acting opioids.	3	8	14/17 (82.4%)	3
Pain, cold, electrical or mechanical stimulation, and drugs that increase muscle membrane excitability may occasionally trigger myotonia [76], which may be resistant to pharmacological treatment.	3	8.5	18/18 (100%)	2
Postoperative
Avoid the use of opioids if possible [72, 77].	4	9	17/19 (89.5%)	1
Monitoring should include CO2 measurements, as the hypercapnic response is diminished, also considering that excessive sleepiness may be part of the disease spectrum [74].	4	9	16/19 (84.2%)	1
Early home discharge is not recommended, as there is an increased risk of having prolonged ileus due to intestinal dysmotility.	4	9	16/18 (88.9%)	2
Specific considerations for Myotonic Dystrophy type II (DM2)
The same recommendations as for DM1 as outlined above apply; however, the overall complication rate appears to be lower [78, 79].	2‐	9	15/15 (100%)	5
In a few cases, a postoperative CK rise and increased muscle complaints have been reported [78].	2‐	9	14/14 (100%)	6

Note: For each recommendation, the level of evidence according to the SIGN criteria and the level of agreement are given by the median voting results and the percentage of respondents with a voting result ≥7.

Abbreviations: CK, creatine kinase; CNS, central nervous system; DM1, myotonic dystrophy type 1; DM2, myotonic dystrophy type 2; ECG, electrocardiography; SIGN, Scottish Intercollegiate Guidelines Network; TIVA, total intravenous anaesthesia.