Case 2: Spine Surgery and Duchenne Muscular Dystrophy

Question 1

What anesthetic drugs would you avoid in DMD? Why?

Answer 1

Avoid succinylcholine and Inhalational agents due to increased risk of rhabdymyolysis and Hyperkalemia

Question 2

What diseases are linked to Malignant hyperthermia?

Answer 2

Central core disease Multiminicore disease King Denborough

Question 3

What diseases are linked to Malignant hyperthermia?

Answer 3

Central core disease Multiminicore disease King Denborough disease

Question 4

What is duchenne muscular dystrophy?

Answer 4

Most common and severe muscular dystrophy with an incidence of 1 in 3000 male newborns; it is caused by a mutation in the dystrophin gene located on chromosome Xp21 Characterized by progressive skeletal muscle weakness with an early onset in childhood. Muscle remodelling with fibrous and fatty tissue leads to loss of ambulation at a mean age of 10 years.

Question 5

Types of Anesthesia that can be provided?

Answer 5

AVOID inhalational! TIVA + Regional can be provided

Question 6

Additional Preoperative testing?

Answer 6

Cardiac function - EKG, Echo performed to evaluate presence of cardiomyopathy Lung function - Lung volumes, blood gas analysis should be done to evaluate grade of pulmonary involvement.

Question 7

Anesthetic concerns with duchenne muscular dystrophy and scoliosis? Ie. the patient has shortness of breath

Answer 7

Abnormal production of dystrophin leads to

1. cardiomyopathy, ventricular dysrhythmias, mitral regurgitation
2. decreased pulmonary reserves, ineffective cough, retained pulmonary secretions, and recurrent pneumonia d/t chronic respiratory muscle weakness
3. chronic aspiration
4. Macroglossia

Question 8

Premedications for aspiration prophylaxis?

Answer 8

1. Metoclopramide
2. An H2 receptor antagonsis
3. Non particulat antacid due to delayed gastric emptying and diminished laryngeal reflexes associated w/ DMD
4. Give anti sialogogue to reduce oral secretions that could inhibit airway management