| Systemic manifestations | Commonly associated congenital myopathy | Preoperative Tests | Anaesthetic implications |
| Respiratory Insufficiency | Multi/Minicore disease Myotubular myopathy | Pulmonary function test | Sensitive to opioids, Benzodiazepines Avoid Suxamethonium Non depolarising muscle relaxants and magnesium can cause prolonged block |
| Corpulmonale Cardiomyopathies, MVP | Multicore disease Nemaline myopathy | ECG, 2D ECHO | Exaggerated cardiac decompensation with anaesthetic induction |
| Scoliosis, osteopenia, previous corrective surgeries, Spine deformities | Central Core disease Multi/Minicore disease Evans Syndrome King-Denborough disease | USG of spine Preoperative baseline Creatine Kinase, Serum Potassium | Careful positioning, moving and handling Technical difficulties in performing central neuraxial block Risk of Rhabdomyolysis |
| Facial dysmorphism elongated face, high arched palate, dolichocephaly | Nemaline myopathy |
| Difficult intubation can be anticipated |
| Bulbar symptoms-difficulty swallowing, coughing | Nemaline myopathy |
| Risk of aspiration |
| Life threatening Emergency MH AIR | MHS Central core disease Evans Syndrome King-Denborough Disease AIR All Congenital myopathies | Genetic diagnosis | Avoid triggers—Succinylcholine, Inhalational anaesthetics Prepare Anaesthetic machine Keep Dantrolene available for reversal |