Exam 2 Musculoskeletal Diseases Flashcards
3 characteristics of scleroderma?
What is it also called?
autoimmune inflammatory vasculitis
fibrosis of skin, internal organs, and deposition of extracellular collagen
tissue fibrosis and organ sclerosis
AKA systemic Sclerosis
What are the 3 forms of scleroderma?
- Localized scleroderma- skin, face, distal limb
- Limited cutaneous systems sclerosis - CREST syndrome, has prominent skin manifestations
- Diffuse cutaneous system sclerosis- generalized skin involvement and cardiac complications.
The limited symptoms of scleroderma are referred to as CREST. What does CREST stand for?
S/S of Scleroderma
Skin:
MS:
Nervous System:
CV:
Skin: Taut skin
MS: Limited mobility/contracture, skeletal muscle myopathy, elevated CK, mild inflammatory arthritis
Nervous System: Nerve compression, trigeminal neuralgia, keratoconjunctivitis sicca
CV: Systemic and pulmonary HTN, dysrhythmias, vasospasm in small arteries of fingers, CHF tx dig, pericarditis, effusions, raynauds tx CCB**
S/S of Scleroderma
Pulmonary:
Renal:
GI:
Pulmonary: pulmonary fibrosis, decreased compliance, pulmonary HTN
Renal: Decreased renal blood flow and systemic HTN
GI: Xerostomia (dry mouth), poor dentition, fibrosis of GI tract (no Reglan, will not stimulate fibrotic GI tract), reflux, treat hypomobility with somatostatin analogue (octreotide***)
Scleroderma Treatment
Alleviating symptoms…
ACE inhibitors for renal protection - only treatment that has been proven to alter the course of scleroderma
SclerodermaAnesthesia Management and Considerations
Airway:
IV:
Pulmonary:
GI:
Eyes:
Other:
Airway: Mandibular motion, small mouth opening, neck ROM (do not manipulate neck while asleep), oral bleeding
CV: IV/arterial line access may be difficult- femoral?
Pulmonary: Decreased compliance, high risk atlanto-pcc. dislocation- avoid increasing PVR, hypoxemia
GI: Aspiration risk- NG, PPI, H2 antagonist
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
What is Pseudohypertrophy Muscular Dystrophy/ Duchenne Muscular Dystrophy (DMD)
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, pulmonary 30%)
Initial symptoms of Duchenne Muscular Dystrophy
waddling gait, frequent falling, difficulty climbing stairs
affecting proximal skeletal muscles of the pelvic girdle
Duchenne Muscular DystrophyS/S
CNS:
MS:
CV:
Pulmonary:
GI:
CNS: Intellectual disability
MS: Kyphoscoliosis, skeletal muscle atrophy, serum CK 20-100x normal
CV: tachycardia, cardiomyopathy by age 18, EKG abnormalities (short PR, V1tall R waves, limb leads deep Q waves)
Pulmonary: OSA, weakened respiratory muscles, and cough
GI: Hypomotility, gastroparesis
Duchenne Muscular DystrophyAnesthesia Management
Airway:
Pulmonary:
CV:
GI:
Airway: weak laryngeal reflexes and cough… DO NOT OVER SEDATE
Pulmonary: weakened muscles, longer vent time up to 36 hrs post-op, avoid NMB
CV: Pre-op EKG and/or echo based on the severity
GI: Delayed gastric emptying, aspiration risk
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?
Avoid succinylcholine can cause hyperkalemia, rhabdo, MH. 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 (ketamine, precedex)
Regional Anesthesia may be best than GA.
What is Myasthenia Gravis?
chronic autoimmune disorder at NMJ- decreased up to 80% of alpha subunit post-synaptic AChreceptors.
Muscle weakness w/ rapid exhaustion of voluntary muscles. Partial recovery with rest.
ACh receptor-bindingantibodies are linked to thymus abnormalities.
Myasthenia GravisS/S
Eyes:
Oral:
Pulmonary:
Skeletal:
Heart:
Endocrine:
60% Ptosis (droopy eyelid), diplopia (double vision)
Dysarthria (slurred speech), dysphagia
Isolated respiratory failure (rare)
Arm, leg, or trunk muscle weakness
Myocarditis- A fib, HB, cardiomyopathy
Autoimmune diseases associated
RA, SLE, pernicious anemia, hyperthyroidism
Differentiate between Myasthenia GravisMyasthenic Crisis vs Cholinergic Crisis.
What is the effect of the Edrophonium/Tensilon Test in each condition?
Myasthenic crisis:
D/t drug resistance or insufficient drug therapy
S/S: severe muscle weakness and respiratory failure
- common when they travel: miss doses or body can’t keep up
Cholinergic crisis (SLUDGEM):
D/t excessive anticholinesterase treatment (too much ACh)
S/S: muscarinic side effects – profound muscle weakness, salivation, miosis, bradycardia, diarrhea, abdominal pain
Edrophonium/Tensilon Test:
1-2 mg IVP edrophonium
- improve the myasthenic crisis - worsen cholinergic crisis
Myasthenia Gravis Treatment (4)
Anticholinesterases:
The first line of treatment
Pyridostigmine (max 120 mg Q3hr) last longer and less side effects than neostigmine
Thymectomy:
- Induces remission
- Reduced use of immunosuppressives
- Reduces ACh receptor antibody levels
- Full benefit delayed (2-6 months)
- Type 2A or 2B start thymectomy b/c of cardiac compression
- thymus located near the heart
Immunosuppression:
Corticosteroids, azathioprine, cyclosporine, mycophenolate
Immunotherapy (short-term effect)
- Plasmapheresis will remove antibodies from circulation.
- Immunoglobulin injections
Myasthenia GravisAnesthesia Management
Weakened ________ effort
Marked sensitivity to ______
Resistance to ____________
Weakened pulmonary effort (Aspiration risk)
H2, metoclopramide
Marked sensitivity to nondepolarizing muscle relaxants (decrease amount). Use nerve stimulator and titrate to nerve stimulator
Resistance to succinylcholine by pyridostigmine ihibits true cholinesterase and impairs plasma cholinesterase (might need a higher dose for effect, but this can lead patients into MG Crisis, just avoid using Sux)
- REMIFENTYL high dose works instead
- RSI
tolerance to NMB and neostigmine reversal due to daily neostigmine
Obicularis oculi may overestimate degree of NMB
What is Osteoarthritis?
The is the most common joint disease in the ____?
OA is characterized by _________ inflammation.
Does stiffness fade throughout the day?
Degenerative process affecting articular cartilage, 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.
*** hips, knees, shoulders most common
Pain present with motion,relieved by rest. Morning stiffness fades throughout the day.
What joints will osteoarthritis affect?
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. - breakdown cartilage of joints and discs in neck and lower back. Middle to lower C-spine (6,7) and L-spine.
lean forward causes compression of disk
OsteoarthritisTreatment
- PT and exercise
- Pain relief - heat, simple analgesics, anti-inflammatory drugs, transcutaneous nerve stimulator, acupuncture. OA patients are typically NOT on steroids
- stem cell therapy, platelet rich plasma, prolotherapy
Joint replacement surgery - necessary when pain is debilitating
OsteoarthritisAnesthesia Management
Airway
**Be aware of limited ROM especially cervical spine
worry about neck extension
usually dont see contraction in these patients
What is Rheumatoid Arthritis?
autoimmune-mediated, systemic inflammatory disease that usually affects the proximal interphalangeal and metacarpophalangeall joints (2nd & 3rd knuckle) hands and feet
Rheumatoid nodules at pressure points (elbows)
Rheumatoid factor: IG antibody present in 90% of patients
Characteristics of Rheumatoid Arthritis
Painful synovial inflammation, swelling, 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)
*common cervial
single or multiple joints
Rheumatoid Arthritis S/S
Atlantoaxial subluxation:
Cricoarytenoid arthritis:
Osteoporosis
NM
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
NM: Weakened skeletal muscles (peripheral neuropathies)
