Case Report

Anesthetic Considerations in a Patient with Myotonic Dystrophy for Hip Labral Repair

Table 1

Summary of DM effects on organ systems.

Organ systemEffect

Musculoskeletal(i) Myopathy, atrophy, myalgias
(ii) Myotonia: triggers include stress and cold as well as specific medications
(i) DM1: tends to affect facial muscles e.g. distal muscles
(ii) DM2: tends to affect proximal muscles e.g. hip flexors

Nervous System(i) Cognitive impairment
(ii) Mental retardation more common in DM1
(i) Axonal sensorimotor polyneuropathy
(ii) Sensorineural hearing loss

Eye(i) Cataracts
(ii) Proptosis

Cardiac(i) Arrhythmias
(a) AV block, bundle branch block most common
(b) Atrial flutter and fibrillation
(i) Cardiomyopathy: Hypertrophy, dilation, systolic dysfunction

Pulmonary(i) OSA
(ii) Hypersomnia/excessive daytime somnolence
(iii) Increased risk of aspiration pneumonia
(i) Respiratory muscle weakness
(ii) Increased sensitivity to respiratory depressants

Gastrointestinal(i) Dysphagia
(ii) GERD
(iii) IBS-like symptoms
(i) Gallstones

Endocrine(i) Primary Hypogonadism
(ii) Diabetes, Insulin resistance
(iii) Hyperthyroidism
(i) Hyperparathyroidism
(ii) Hyperhidrosis
(iii) Male pattern baldness

Reproductive(i) Low sperm count secondary to hypogonadism(i) Higher risk of miscarriage, preterm labor

Cancer(i) Increased risk for cancers of endometrium, brain, ovary, colon, and skin