Exam 2 Musculoskeletal Diseases

Question 1

What is scleroderma?

Answer 1

Inflammation and autoimmune disease that causes vascular injury and vascular obliteration. Chronic hardening and tightening of the skin and connective tissues.

Tissue fibrosis and organ sclerosis.

Question 2

What are the 3 forms of scleroderma?

Answer 2

1. Localized scleroderma- skin, face, distal limb
2. Limited cutaneous systems sclerosis - CREST syndrome, has prominent skin manifestations
3. Diffuse cutaneous system sclerosis- generalized skin involvement and cardiac complications.

Question 3

The limited symptoms of scleroderma are referred to as CREST. What does CREST stand for?

Answer 3

Question 4

S/S of Scleroderma
Skin:
MS:
Nervous System:
CV:

Answer 4

Skin: Taut skin
MS: Limited mobility/contractures, skeletal muscle myopathy
Nervous System: Nerve compression
CV: Systemic and pulmonary HTN, dysrhythmias, vasospasm in small arteries of fingers, CHF

Question 5

S/S of Scleroderma
Pulmonary:
Renal:
GI:

Answer 5

Pulmonary: Diffuse interstitial pulmonary fibrosis, decreased pulmonary compliance (stiff lungs)
Renal: Decreased renal blood flow and systemic HTN
GI: Xerostomia, poor dentition, fibrosis of GI tract (no Reglan, will not stimulate fibrotic GI tract), reflux

Question 6

Scleroderma Treatment

Answer 6

ACE inhibitors for renal protection - only treatment that has been proven to alter the course of scleroderma

Question 7

SclerodermaAnesthesia Management and Considerations
Airway:
IV:
Pulmonary:
GI:
Eyes:
Other:

Answer 7

Airway: Mandibular motion, small mouth opening, neck ROM (do not manipulate neck while asleep), oral bleeding

CV: IV/arterial line access may be difficult, use US.

Pulmonary: Decreased pulmonary compliance and reserve, avoid increasing PVR (do not make them hypoxic)

GI: Aspiration caution, increase gastric pH by using PPI.

Eyes: tape eyes, ointment, gauze, goggles

Other: Regional anesthesia may be best for these patients, keep patient warm, VTE prophylaxis, Positioning with care, Pulse ox difficulties (ear probe)

Question 8

Patients with schleroderma may already be on steroids. What should you give them because of this?

Answer 8

A stress dose of hydrocortisone. 100mg Q6-8 hrs

Question 9

What is Pseudohypertrophy Muscular Dystrophy/ Duchenne Muscular Dystrophy (DMD)

Answer 9

It is a form of muscle wasting.

Mutation in the dystrophin gene (x-linked recessive) causes fatty infiltration leading to pseudohypertrophic muscles.
Common in 2-5 y/o boys
By age 8-10 Wheelchair bound
Usually live until ages 20-25 years (CHF, pneumonia)

Question 10

Initial symptoms of Duchenne Muscular Dystrophy

Answer 10

Initial symptoms: waddling gait, frequent falling, difficulty climbing stairs, tiptoes.

Question 11

Duchenne Muscular DystrophyS/S
CNS:
MS:
CV:
Pulmonary:
GI:

Answer 11

CNS: Intellectual disability

MS: Kyphoscoliosis, skeletal muscle atrophy, serum CK 20-100x normal d/t increase muscle permeability

CV: Sinus tachycardia, cardiomyopathy, EKG abnormalities (tall R-wave in V1)

Pulmonary: OSA, weakened respiratory muscles, and cough

GI: Hypomotility, gastroparesis

Question 12

Duchenne Muscular DystrophyAnesthesia Management
Airway:
Pulmonary:
CV:
GI:

Answer 12

Airway: weak laryngeal reflexes and cough (You want these patients “singing” on the tube during emergence.)

Pulmonary: weakened muscles

CV: Pre-op EKG and/or echo based on the severity

GI: Delayed gastric emptying, aspiration risk

Question 13

Other anesthesia management for Duchenne Muscular DystrophyAnesthesia:

What drug do you want to avoid?

When do you want to extubate these patients?

What type of incidence are higher in these patients?

What type of anesthesia may be best?

Answer 13

Avoid succinylcholine can cause hyperkalemia and rhabdomyolysis. Use NDMR (Rocuronium).

Pharyngeal and respiratory muscle weakness, secure the airway. Make sure the patient is fully awake.

MH – increased incidence. Have Dantrolene ready. Use TIVA as an alternative

Regional Anesthesia may be best than GA.

Question 14

Avoid succinylcholine in Muscular Dystrophy

Answer 14

They already have muscle wasting, and giving them sux will increase rhabdomyolisis and cause hyperkalemia.

Question 15

What is Myasthenia Gravis?

Answer 15

A chronic autoimmune disorder involving the NMJ. There will be decreased functional post-synaptic AChreceptors.

Muscle weakness w/ rapid exhaustion of voluntary muscles. Partial recovery with rest.

ACh receptor-bindingantibodies are linked to thymus abnormalities.

Question 16

Myasthenia GravisS/S
Eyes:
Oral:
Pulmonary:
Skeletal:
Heart:
Endocrine:

Answer 16

Ptosis (droopy eyelid), diplopia (double vision)

Dysarthria (slurred speech), dysphagia, and difficulty handling saliva

Isolated respiratory failure (rare)

Arm, leg, or trunk muscle weakness

Myocarditis

Autoimmune diseases associated
RA, SLE, pernicious anemia, hyperthyroidism

Question 17

Differentiate between Myasthenia GravisMyasthenic Crisis vs Cholinergic Crisis.

Answer 17

Myasthenic crisis:
D/t drug resistance or insufficient drug therapy
S/S: severe muscle weakness and respiratory failure

Cholinergic crisis (SLUDGE):
D/t excessive anticholinesterase treatment
S/S: muscarinic side effects – profound muscle weakness, salivation, miosis, bradycardia, diarrhea, abdominal pain

Question 18

What is the effect of the Edrophonium/Tensilon Test in each condition?

Answer 18

Edrophonium/Tensilon Test:
1-2 mg IVPof edrophonium will improve the myasthenic crisis and makes the cholinergic crisis worse.

Question 19

Myasthenia GravisTreatment
Anticholinesterases (first-line treatment):
Immunosuppression:
Immunotherapy:

Answer 19

Anticholinesterases:
The first line of treatment
Pyridostigmine last longer than neostigmine

Immunosuppression
Corticosteroids, azathioprine, cyclosporine, mycophenolate

Immunotherapy (short-term effect)
Plasmapheresis will remove antibodies from circulation.
Immunoglobulin will have a temporary effect.

Question 20

The first line of treatment in Myastenia Gravis is Anthicholinergics. Why is this a problem for anesthesia?

Answer 20

Anticholinesterases:
The first line of treatment
Pyridostigmine last longer than neostigmine

This is a problem because they are used to the drugs that we use to reverse muscle paralysis, so we may need to give more of that drug than normal to effect them.

Question 21

What surgical intervention can be done for Myastenia Gravis?

Answer 21

Thymectomy:
Induces remission
Reduced use of immunosuppressives
Reduces ACh receptor antibody levels
Full benefit delayed (2-6 months)

Question 22

Myasthenia GravisAnesthesia Management

Weakened ________ effort

Marked sensitivity to ______

Resistance to ____________

Answer 22

Weakened pulmonary effort (Aspiration risk)

Marked sensitivity to nondepolarizing muscle relaxants (decrease amount). Use nerve stimulator and titrate to nerve stimulator

Resistance to succinylcholine (might need a higher dose for effect, but this can lead patients into MG Crisis, just avoid using Sux)

Question 23

NMDRs and Myastenia Gravis

Answer 23

Marked sensitivity to nondepolarizing muscle relaxants (decrease amount). Use nerve stimulator and titrate to nerve stimulator

Question 24

What is Osteoarthritis?

The is the most common joint disease in the ____?

OA is characterized by _________ inflammation.

Does stiffness fade throughout the day?

Answer 24

Degenerative process affecting articular cartilage, the most common joint disease in the elderly.

Characterized by minimal inflammation usually a result of chronic joint trauma (sports), biomechanical stresses, joint injury, abnormal joint loading, neuropathy, ligament injury, muscle atrophy, and obesity.

Pain present with motion,relieved by rest. Morning stiffness fades throughout the day. (T-rex)

Question 25

What joints will osteoarthritis affect?

Answer 25

Weight-bearing and distal interphalangeal joints. **Heberden nodes**- Distal interphalangeal joints, first knuckle (*pictured*) Protrusion of the nucleus pulposus (herniated disc) can lead to compression of nerve roots. Degenerative disease – vertebral bodies and intervertebral disks. OA will affect the breakdown of the cartilage of the joints and discs in the neck and lower back. **Middle to lower C-spine and  L-spine.**

Question 26

OsteoarthritisTreatment 

Answer 26

PT and exercise Maintaining muscle function Pain relief - heat, simple analgesics, anti-inflammatory drugs, transcutaneous nerve stimulator, acupuncture. **OA patients are typically NOT on steroids** Joint replacement surgery - necessary when pain is debilitating

Question 27

OsteoarthritisAnesthesia Management 

Answer 27

Airway  **Be aware of limited ROM, and keep them comfortable.** **Document the patient's position on the OR table.** Don't lean on the patient.

Question 28

What is Rheumatoid Arthritis?

Answer 28

An autoimmune-mediated, systemic inflammatory disease that usually affects the proximal interphalangeal and metacarpophalangeal joints (2nd & 3rd knuckle). Rheumatoid nodules at pressure points (elbows) Rheumatoid factor (IG antibody present in 95% of patients with RA)

Question 29

Characteristics of Rheumatoid Arthritis 

Answer 29

Single or multiple joints Painful synovial inflammation, swelling, and increased fluid **Morning stiffness like OA, but remains stiff throughout the entire day.** Symmetrical distribution of several joints. **Fusiform swelling - Joints become enlarged and the fingers crooked** (*pictured*) Synovitis of the temporomandibular joint (decrease mouth opening) Affects nearly all joints (except the t-spine and lumbosacral spine)

Question 30

Rheumatoid Arthritis  S/S Atlantoaxial subluxation: Cricoarytenoid arthritis: Osteoporosis NM

Answer 30

Atlantoaxial subluxation instability (C1 and C2): Odontoid process (dens) protrudes into the foramen magnum. The instability can place pressure on the transverse ligament and spinal cord. Instability can also impair vertebral artery blood flow. Cricoarytenoid arthritis : Acute – hoarseness, dyspnea, and **stridor w/ tenderness over the larynx; swelling and redness of arytenoids** Chronic – asymptomatic or variable degrees of hoarseness, dyspnea, and upper airway obstruction  Osteoporosis: More common in women than men NM: Weakened skeletal muscles (peripheral neuropathies)

Question 31

Rheumatoid ArthritisS/S CV: Pulmonary: Hematology: Dryness:

Answer 31

CV: Pericarditis, accelerated coronary atherosclerosis Pulmonary: **Restrictive lung changes**, decrease Vt  Hematology: anemia, neutropenia, elevated platelets Dryness: **Keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth)**

Question 32

Rheumatoid ArthritisTreatment

Answer 32

NSAIDS Corticosteroids DMARDs *May take 2-6 months to see effects* Tumor necrosis factor (TNF-alpha) inhibitors and interleukin (IL-1) inhibitors.  **TNF-alpha inhibitors work *faster than* DMARDs.** IL-1 inhibitors – slower onset and less effective. Surgery is usually held off until the patient is in intractable pain, has impairment of joint function, and is in need of joint stabilization. Usually a total replacement surgery.

Question 33

Rheumatoid ArthritisAnesthesia Management 

Answer 33

Airway- **Atlantoaxial subluxation** ,TMJ limitation, Cricoarytenoid joints. **(handle w/care)** Severe rheumatoid lung disease Protect eyes - **Keratoconjunctivitis sicca (dry eyes)** Stress dose - give before surgery, may have a decrease amount of endogenous cortisol.

Question 34

What is Systemic Lupus Erythematosus? Typical Manifestation:

Answer 34

Multisystem chronic inflammation characterized by antinuclear antibody production. Typical manifestations (Usually will have 3 out 5 of the following): 1. **Antinuclear antibodies** 2. **Malar  rash (*butterfly rash*)** 3. Thrombocytopenia 3. Serositis- inflammation of a serous membrane around the organ 5. Nephritis

Question 35

Systemic Lupus ErythematosusS/S Join/Spine: CNS: CV: Pulmonary: Renal:

Answer 35

Joint/Spine: **Polyarthritis and dermatitis, symmetrical arthritis (most common sx)**, no spinal involvement, avascular necrosis of the femoral head or condyle CNS: Cognitive dysfunction, psychological changes CV: Pericarditis, coronary atherosclerosis, Raynaud’s Pulmonary: Lupus pneumonia, **restrictive lung disease**, vanishing lung syndrome Renal: Glomerulonephritis, decreased GFR, risk of renal failure 

Question 36

Systemic Lupus ErythematosusS/S GI/Liver: NM: Hematology: Skin:

Answer 36

GI/Liver: ABD pain, pancreatitis, elevated liver enzymes NM: Skeletal muscle weakness Hematology: Thromboembolism, thrombocytopenia, hemolytic anemia, Skin: **Butterfly-shaped malar rash**, discoid lesions, alopecia

Question 37

Systemic Lupus ErythematosusTreatment

Answer 37

NSAIDs or ASA - will treat arthritis and serositis Anti-malarial (*Hydroxychloroquine and quinacrine*) - will treat arthritis and dermatological manifestations Corticosteroids- By reducing inflammation, corticosteroids can help to improve the production and survival of platelets in the bone marrow. Immunosuppressants will be used if steroids are not effective (*Methotrexate, azathioprine*)

Question 38

Systemic Lupus ErythematosusAnesthesia Management

Answer 38

Based upon manifestations and organ dysfunction **Airway: recurrent laryngeal nerve palsy (common), cricoarytenoid arthritis**  **Stress dose of corticosteroids**

Question 39

What is recurrent laryngeal nerve palsy? How does lupus cause recurrent laryngeal nerve palsy?

Answer 39

A condition where the nerve that controls the muscles of the larynx (voice box) is damaged, which can lead to problems with speaking, breathing, and swallowing. In lupus, the immune system attacks healthy tissues, including the nerves. This can lead to **inflammation of the laryngeal nerve or blood vessels, which can cause them to become compressed or damaged.**

Question 40

What is Malignant Hyperthermia?

Answer 40

Hypermetabolic syndrome involving a genetic mutation to the **Ryanodine receptor - RYR1 gene or Dihydropyridine receptor** **Caused by exposure to inhaled VA and succinylcholine (*50% mortality, investigate family history*)** Uncontrolled elevation of sarcoplasmic calcium Sustained activation of muscle contraction Rhabdomyolysis

Question 41

Malignant HyperthermiaNon-triggering Agents

Answer 41

Question 42

Malignant HyperthermiaS/S

Answer 42

Early Sign: Masseter, Acidosis, T-peaked, CO2 increase, HR increase, Rigidity, RR increase. *MATCHRR*

Question 43

Malignant HyperthermiaTreatment

Answer 43

D/C all triggering gas/drugs Hyperventilate with 100% O2 at 10 L/min Change breathing circuit and soda lime Dantrolene (*Have someone else do this, hard to mix*) 20 mg + 3 G mannitol (*Mix with 60 mL sterile water*) Initial dose 2.5 mg/kg Max upper limit 10 mg/kg Treat arrhythmias ICE, cool them down Monitor urine output

Question 44

Malignant HyperthermiaPost-Op

Answer 44

Transfer to ICU 24-48 hours Report to MH registry MH testing for pt and family members: **Muscle biopsy contracture testing (Halothane plus caffeine contracture test)**

Question 45

What type of anesthesia would be used if you know the patient has a history of MH?

Answer 45

Use TIVA and go slow.