Exam 2 Musculoskeletal Diseases Flashcards
What is scleroderma?
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.
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/contractures, skeletal muscle myopathy
Nervous System: Nerve compression
CV: Systemic and pulmonary HTN, dysrhythmias, vasospasm in small arteries of fingers, CHF
S/S of Scleroderma
Pulmonary:
Renal:
GI:
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
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, 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)
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)
Initial symptoms of Duchenne Muscular Dystrophy
Initial symptoms: waddling gait, frequent falling, difficulty climbing stairs, tiptoes.
Duchenne Muscular DystrophyS/S
CNS:
MS:
CV:
Pulmonary:
GI:
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
Duchenne Muscular DystrophyAnesthesia Management
Airway:
Pulmonary:
CV:
GI:
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
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 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.
What is Myasthenia Gravis?
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.
Myasthenia GravisS/S
Eyes:
Oral:
Pulmonary:
Skeletal:
Heart:
Endocrine:
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
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
Cholinergic crisis (SLUDGE):
D/t excessive anticholinesterase treatment
S/S: muscarinic side effects – profound muscle weakness, salivation, miosis, bradycardia, diarrhea, abdominal pain
Edrophonium/Tensilon Test:
1-2 mg IVPof edrophonium will improve the myasthenic crisis and makes the cholinergic crisis worse.