Musculoskeletal disorders Exam 2 Flashcards
What is the pathophysiology of scleroderma?
- Autoimmune disease, excessive collagen production
- Hardening/tightening of the skin and connective tissue
- vascular injury/obliteration
- tissue fibrosis and organ sclerosis
What are the 3 types of scleroderma?
- Localized - skin, face, trunk, limbs
- limited cutaneous system - localized + CREST syndrome
- diffuse cutaneous system - rapidly progressive, CV involvment
What mnemonic guides the main symptoms associated with scleroderma?
CREST
* Calcinosis (Ca deposits)
* Raynaud’s
* Esophageal dysfunction
* Sclerodactyly (tight hands)
* Telangiectasia’s (small dilated blood vessels).
What skin and musculoskeletal abnormalities might be seen with scleroderma?
- Taut skin
- Contractures
- myopathy
What can happen to nerves with scleroderma?
Nerve Compression
What airway and pulmonary considerations exist for scleroderma?
- Pulmonary fibrosis
- Decreased airway compliance
What CV considerations exist for scleroderma?
- Systemic HTN and PH
- Dysrhythmias
- Small artery vasospasm’s (Raynaud’s)
- CHF
What treatments are used for scleroderma?
- Symptom alleviation
- ACE-inhibitors (renal crisis protection)
- Digoxin (improve CO)
- Steroids
- octreotide/PPIs
What GI symptoms exist for scleroderma?
- Xerostomia (dry mouth)
- GI tract fibrosis
- Poor dentition
- GERD
What dose of metoclopramide would be utilized for GI tract fibrosis from scleroderma?
Trick question. Metoclopramide is not effective in these patients.
What scleroderma anesthesia management considerations are there?
- avoid increasing PVR
- IV/A-line access
- Aspiration
- normothermia
What is Duchenne’s Muscular Dystrophy (DMD)?
- Genetic disorder - mutation of dystrophin gene (X chromosome)
- affects Skeletal muscles
What is dystrophin? What does it do?
- a large protein that stabilizes the muscle membrane
- without it muscle integrity breaks down
- progressive muscle loss
Who does it commonly affect and at what age? What are the symptoms?
- boys ages 2-5 years old
- waddling gait, frequent falling, can’t climb stairs, Gower’s sign
What is the progression of DMD?
- W/C bound by 8-10 y/o
- Deterioration in muscle strength
- Death at 20-25 y/o
- CHF and/or pneumonia
What s/s are seen with DMD?
- CNS - intellectual disability
- MS - kyphosis, muscle atrophy, ↑ CK, long bone fx
- CV - ↑ HR, cardiomyopathy, short PR
- Pulm - weakened respiratory muscles and weak cough, OSA
- GI - hypomotility & gastroparesis.
What are the anesthetic concerns and interventions relevant to DMD patients?
* Airway
* Pulmonary
* CV
* GI
- Airway - weak laryngeal reflexes & cough
- Pulm - weakened muscles, secretions
- CV - Get pre-op EKG & echo
- GI - delayed gastric emptying.
What drug should be avoided with DMD patients?
- Succinylcholine (Rhabdo & ↑K⁺)
- use NDMBs
What type of anesthesia is preferable for a DMD patient?
Regional (vs GA)
Why might one use less volatile gases with DMD patients?
- ↑risk of malignant hyperthermia
- Ensure you have Dantrolene
What is the pathophysiology of myasthenia gravis?
- Autoimmune: antibodies against post-synaptic AChR
- α-subunits of AChR antibodies
- Muscle weakness w/ rapid exhaustion of voluntary muscles
What is the classic symptom of MG?
- muscle weakness and fatigue that worsens with activity
- improves with rest
What are the classifications of MG?
Type I: limited involvement of extraocular muscles
Type IV: severe muscle weakness (progression from Type I or II)
Type III: acute onset < 6months, high mortality
What organ is linked with the production of anti-ACh receptor antibodies?
Thymus.