Medicine (Connective Tissue Disorders)

Question 1

What is systemic lupus erythematous?

Answer 1

A multisystem chronic inflammation disorder characterized by autoantibody production responsible for immune-mediated tissue damage
-Renal, pulmonary, cardiac, maxillofacial, hematologic, neuropsychiatric manifestations

Question 2

What is your management for a patient with SLE?

Answer 2

-Consult with patient’s rheumatologist
-Delay surgery until flare-ups have resolved
-CBC, aPTT, BMP (r/o coagulopathies/thrombocytopenia)
-EKG

Question 3

What is rheumatoid arthritis?

Answer 3

-Chronic systemic inflammatory disease, leads to progressive destruction of joint, bone and articular cartilage

Question 4

What is obesity hypoventilation syndrome?

Answer 4

-Episodic central apnea events due to desensitization of hypercarbia. Light sedation can cause airway collapse

Question 5

What is Duchenne Muscular dystrophy?

Answer 5

-X-linked recessive neuromuscular disease (dystrophin gene), progressive necrosis of muscle fibers
-Calf muscle pseudohypertrophy

Question 6

What are Becker and myotonic muscular dystrophy?

Answer 6

-Becker: Milder form of Duchenne, X linked recessive
-Myotonic: Autosomal dominant

Question 7

How are patients with muscular dystrophy managed?

Answer 7

-EKG
-Succinylcholine contraindicated (rhabdomyolysis/hyperkalemia)
-Risk of malignant hyperthermia
-Post-op respiratory failure (be ready for ICU), limit narcotics

Question 8

What is myasthenia gravis?

Answer 8

-Autoimmune disorder affecting postsynaptic neuromuscular transmission of nicotinich ACH receptor from autoantibodies
-Weakness and fatigue

Question 9

How is myasthenia gravis managed peri-op?

Answer 9

-Reduce/avoid sedatives/opioids (reduced respiratory reserve)
-Warn patient of possible prolonged ventilatory support
-Avoid NMB (resistent to succinylcholine and sensitive to non-depolarizing agents)
-Propofol safe

-Remi-prop TIVA best

Question 10

What is Ehlers-Danlos Syndrome?

Answer 10

-Inheritable disease resulting in a defect in synthesis or structure of fibrillar collagen. Hypermobile state

Question 11

What is the management for Ehler-Danlos Syndrome patients?

Answer 11

-Resistance to local anesthetics
-Occipitoatlantoaxial instability
-Cardiac evaluation
-Hematology plan

Question 12

What is Marfan’s syndrome?

Answer 12

-Autosomal dominant connective tissue disorder affecting cardiac, skeletal and ocular tissue

Question 13

What is the management for Marfan syndrome patients?

Answer 13

-Cardiology consult to evaluate valvular function and aortic root dilation
-Control BP and blood volume
-Pulmonary function evaluation
-Anticipate difficult airway