Musculoskeletal Diseases Flashcards

(94 cards)

1
Q

Ankylosing Spondylitis (AS)

A
  • Chronic, progressive inflammatory disease involving joints of spine and adjacent soft tissues
  • Associated with Human Leukocyte Antigen (HLA) B27 in most cases
  • Male to female ratio 4:1
  • Important anesthesia implications both articular and non-articular
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2
Q

SLE Manifestations: Heart

A
  • Pericarditis (auscultate friction rub)
  • Valvular disease
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3
Q

Achondroplasia Anesthesia Considerations:

Cardiac and Pulmonary involvement

A
  • Restrictive ventilatory defects may occur and lead to pulm HTN
  • Pulm HTN leading to cor pulmonale is most common CV disturbance that develops
  • Central sleep apnea (related to brainstem compression by foramen magnum), and upper airway obstruction
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4
Q

Scleroderma multi-system effects: Skin

A
  • Thickened taut skin
  • Limited mobility
  • Flexion contractures
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5
Q

Myasthenia Gravis Anesthesia Considerations: Emergence

A
  • At great risk for postop respiratory failure
  • Advise pt may awaken w/ ETT in place
  • Careful eval of ventilatory function prior to extubation
  • Close obs in recovery
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6
Q

Systemic Lupus Erythematosus (SLE): patho/onset/incidence

A
  • Multisystem inflammatory dz characterized by immune-mediated tissue damage
  • Predominantly occurs in females (1:1000)
  • Presence of HTN & nephritis = poor prognosis
  • Onset may be drug induced (milder form, lupus like syndrome):
    • Hydralazine, procainamide, isoniazid, methyldopa
    • Slow acetylators at ↑ risk
  • Physiologic stress can exacerbate disease
    • Surgery; Infection; Pregnancy
      • Poor fetal outcomes (especially if HTN & nephritis)
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7
Q

Mild SLE Treatment

A
  • NSAIDs for joint symptoms and pleurisy
  • Low dose corticosteroids such as prednisone
  • Anti-malarial drugs (hydroxychloroquine/quinacrine) and low-dose corticosteroids for thrombocytopenia, hemolytic anemia, skin, arthritis symptoms
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8
Q

Scleroderma multi-system effects: CV

A
  • Myocardial tissue replaced w/ fibrotic tissue→ conduction abnormalities
  • Major vascular changes
  • High incidence pulm HTN
  • Vasospasms- Raynaud’s (poor arterial perfusion; increased r/f infection)
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9
Q

SLE Drugs for Altered Renal Function

A
  • Most commonly used drugs in anesthesia are at least partly dependent on renal excretion for elimination
    • Dose/frequency modification may be needed to prevent accumulation of drug OR active metabolites
  • Intravenous Agents
    • Pk of Propofol and Etomidate not significantly affected by renal impairment (OK)
    • Benzos undergo hepatic metabolism and conjugation prior to elimination in urine (OK)
    • Opioids: accumulation of morphine and meperidine metabolites prolong respiratory depression in some renal failure
  • VA’s – ideal d/t lack of dependence on kidney for elimination
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10
Q

Myasthenia Gravis Anesthesia Considerations

A
  • Elective surgery during remission
  • Premed/opioids minimized or avoided
    • Prone to weakness
    • Aminoglycoside antibiotics can enhance weakness
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11
Q

Muscular Dystrophy Anesthesia Considerations: Induction

A
  • Succinylcholine contraindicated
    • Risk of inducing severe hyperkalemia, rhabdomyolysis, v-fib and cardiac arrest
    • MH risk: Unclear if true MH or “MH like” rxn w/ rhabdomyolysis etc.
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12
Q

Myasthenic Syndrome or Myasthenia Gravis?

Sensitive to Sch and NDMR

A

Myasthenic Syndrome

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13
Q

Stress Dose Steroids in Periop Setting

A
  • If pt already on steroids, continue at usual dose if possible
  • Data supporting administration of stress doses limited, decision to administer depends on procedure
  • Small procedures (dental work, skin biopsies):
    • no stress dose necessary
  • Moderate procedures:
    • give 25 mg hydrocortisone q8hr, taper over 1-2 days
  • Major surgery:
    • give 50 mg hydrocortisone q8hr, taper over 2-3 days
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14
Q

SLE Manifestations: Joints/Muscle

A
  • Symmetrical arthritis (90%)
  • Cricoarytenoid arthritis
  • AVN (avascular necrosis)– can lead to pain
  • Myopathy
  • Tendon ruptures
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15
Q

Scleroderma multi-system effects: ENT

A
  • dry eyes
  • dry mouth
  • oral/nasal tanglectasias (r/f bleeding)
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16
Q

Myasthenic Syndrome: Anesthesia Considerations

A
  • May be undiagnosed… suspect in bronchoscopy. Mediastinoscopy, or thoracoscopy for suspected lung CA.
  • Unlike myasthenia gravis, LEMS pts are very sensitive to both depolarizing (Sch) and NDNMB’s
    • Anticholinesterase drugs not helpful in this dz and may not effectively reverse clinical effects of NDMR because defect is pre-synaptic
  • VA’s alone often sufficient to provide muscle relaxation for intubation and most surgical procedures
  • Only give NMBA’s in small increments and w/ careful neuromuscular monitoring
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17
Q

Ankylosing Spondylitis Clinical Manifestations:

Cardiac involvement

A
  • Aortic regurgitation (avoid sudden↑ in SVR/ keep HR >90 bpm/low normal BP)
  • Conduction abnormalities- BBB
  • Cardiomegaly
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18
Q

Myasthenic Syndrome or Myasthenia Gravis?

Muscle pain common

A

Myasthenic Syndrome

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19
Q

Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:

Clinical Manifestations: Other musculoskeletal

A

Progressive and severe kyphoscoliosis that results from progressive weakness and contractures

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20
Q

SLE Manifestations: Lungs

A
  • “Lupus pneumonia”
  • Restrictive pattern
  • Recurrent atelectasis (Phrenic nerve neuropathy)

Chart: pleuritis, pneumonitis, PE, pulm hemorrhage

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21
Q

Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:

Clinical Manifestations: GI

A
  • Reduced intestinal tract tone
    • Delayed gastric emptying and ↑ r/f aspiration
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22
Q

Marfan’s Syndrome: Clinical Manifestations (other than CV)

A
  • Long tubular bones: long legs/arms/fingers
  • Hyperextensibility of joints
  • High arched palate (difficult intubation)
  • Crowded teeth
  • Pectus excavatum
  • Kyphoscoliosis
  • Pulmonary
    • High incidence PTX
    • Spinal/ sternal deformities –> restrictive lung
  • Ocular
    • Dislocation of ocular lens; Retinal detachment
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23
Q

Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:

Clinical Manifestations: Cardiopulmonary dysfunction

A
  • Degeneration of cardiac muscle
  • Chronic weakness of inspiratory muscles = ↓ ability to cough/clear secretions
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24
Q

RA Additional Clinical Manifestations: Pulmonary

A
  • Pleural effusion and restrictive lung dz d/t rheumatoid nodules in lung tissue
  • ↓ lung volume
  • Pulmonary fibrosis (rare)
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25
Myasthenia Gravis: Patho
* Autoimmune **destruction of postsynaptic Ach receptors** at NMJ (up to 80% loss) * Characterized by **weakness & rapid exhaustion** of skeletal muscle * Particularly those innervated by cranial nerves (ocular, pharyngeal, laryngeal) -- *eye drooping, r/f dysphagia and aspiration* * Course marked by **exacerbations & remissions** * 1:7500 (women age 20-30 and men \>60) * Surgery, infection, stress, and pregnancy often lead to exacerbations * Association w/ **thymic hyperplasia and thymoma**
26
RA: Management of Anesthesia
* Anemia of chronic disease * Effect of meds on platelet function * With lung dz – **PFT**/ intraop **ABG**/post-op **ventilatory support** * May need **stress dose** corticosteroids * **Extubate w/ caution** if cricoarytenoid arthritis present *(d/t post-extubation swelling)*
27
**Myasthenic Syndrome or Myasthenia Gravis?** Co-existing pathologic condition of thymoma
**Myasthenia Gravis**
28
**Myasthenic Syndrome or Myasthenia Gravis?** Proximal limb weakness (legs more than arms)
**Myasthenic Syndrome**
29
OA Anesthetic Considerations: Positioning/drugs/etc.
* _Positioning:_ Support affected joint(s) and minimize r/f injury * _Drugs_ used in treatment * Corticosteroids NOT recommended * NSAIDs and ASA → Potential bleeding issues * Reconstructive joint surgery → Total hip or knee replacement
30
**Myasthenic Syndrome or Myasthenia Gravis?** Muscle pain uncommon
**Myasthenia Gravis**
31
SLE Anesthesia Considerations: Anesthesia management and drug selection
* Interactions w/ drugs used in SLE treatment * Degree of organ dysfunction * Impaired renal * Hepatic clearance of drugs * Cardiopulmonary involvement * Strict **asepsis** w/ invasive procedures * ↓ r/f infection * Maintenance of **normothermia** * May ↓ r/f infection * Lessening impact of Raynaud’s if present
32
SLE Manifestations: Kidneys
**Glomerulonephritis** leading to nephrotic syndrome and renal failure
33
**Myasthenic Syndrome or Myasthenia Gravis?** Co-existing pathologic conditions of small cell lung cancer
**Myasthenic Syndrome**
34
Scleroderma multi-system effects: Renal
renal HTN (ACE inhibitors are effective)
35
Muscular Dystrophy--Duchenne’s/Pseudohypertrophic: Clinical Manifestations: Cardiac
* _Cardiac muscle degeneration_ 1. **ECG** abnormalities * Atrial arrhythmias * Prolonged PR interval 2. **↓ myocardial contractility** and cardiomyopathy 3. **Mitral regurgitation** 2º to papillary muscle dysfunction
36
Muscular Dystrophy Anesthesia Considerations: Maintenance
* Marked **cardiopulmonary depression** may be seen w/ VA’s * Normal to **prolonged response to NDMR** * Anticipate **post-op pulmonary dysfunction**
37
**Myasthenic Syndrome or Myasthenia Gravis?** Reflexes normal
**Myasthenia Gravis**
38
SLE Manifestations: Skin and membranes
* Butterfly rash w/ nasal erythema (50%) * Oral and pharyngeal ulcers
39
Muscular Dystrophy--Duchenne’s/Pseudohypertrophic: Clinical Manifestations--hallmark
* Clinical hallmark is **muscle weakness pronounced in proximal extremities** * Wheelchair confined by age 8-10; Often fatal by 20.
40
Ankylosing Spondylitis Clinical Manifestations: Airway Considerations
* Difficult intubation is associated when AS involves the **c-spine** * Increased difficulty d/t **limited mouth opening when TMJ** involved * Significant r/f neurological injury w/ any excessive **neck extension** * Progressive **kyphosis and spine fixation** may limit intubation * Risk of **occult cervical fracture** w/ minimal trauma * **Cricoarytenoid arthritis** * Cords susceptible to trauma *(use small ETT and consider awake fiberoptic intubation)*
41
SLE Manifestations: Airway
* Laryngeal involvement in ~ 1/3 pts * Mucosal irritation * **Cricoarytenoid arthritis** * **Recurrent laryngeal nerve palsy** *(hoarseness, dysphagia)*
42
**Myasthenic Syndrome or Myasthenia Gravis?** Exercise causes fatigue
**Myasthenia Gravis**
43
SLE Manifestations: Inflammation and vasculitis
* Vessel wall thickening, weakening, narrowing, and scarring: CAD, Stroke risk, etc. * HTN * +/- Pulmonary HTN– *avoid high CO2 because it can cause vasoconstriction→ lead to acute RHF* * Thromboembolism, Hypercoagulable state * Hemolytic anemia * Frequent fevers
44
Muscular Dystrophy **Duchenne’s/Pseudohypertrophic**
* Most common and severe form of MD * Other dystrophies are less severe, onset at a later age, slower progression * Almost exclusively **male** and presents at age 2-5 yrs. * Characterized by **symmetric** **proximal muscle weakness** manifesting in gait disturbance * Pelvic girdle * _Pseudohypertrophic_: fatty infiltration
45
Severe SLE Treatment
* **High-dose** corticosteroids (Stress dose?) * Immunosuppressive/chemotherapy drugs * Methotrexate, cyclophosphamide, azathioprine, mycophenolate
46
Rheumatoid Arthritis
* Chronic, systemic **inflammatory** disorder/auto-immune w/ **articular** & systemic involvement * Characterized by **exacerbations and remissions** * Affects about 1% of adults (**females** \> males)
47
SLE Anesthesia Considerations: Airway management
* **Laryngeal** involvement * Laryngeal **erythema** and edema common * Mucosal **ulceration**→ *pain, bleeding* * **Cricoarytenoid arthritis** * **Recurrent laryngeal nerve palsy**→ *r/f aspiration*
48
Ankylosing Spondylitis Anesthesia Considerations
* Anesthetic management influenced by severity of disease * Upper airway involvement * Presence of restrictive lung disease * Degree of CV involvement * May be using neurologic monitoring during corrective spinal surgery (anesthetic limitations) * **Awake fiberoptic** tracheal intubation if spinal deformity extensive * Spinal and epidural anesthesia is technically difficult * May result in an increased risk of complications * Consider paramedian approach
49
Scleroderma multi-system effects: CNS
* Thickened connective tissue around nerve sheath leading to **neuropathy**; high incidence **trigeminal neuralgia**
50
Myasthenic Syndrome: Patho
* **Lambert-Eaton** myasthenic syndrome (LEMS) * Develops in association w/ a **neoplasm**, but also seen w/ other occult malignancies or idiopathic autoimmune * Usually **small cell lung CA** * Disorder results from presynaptic side defect of neuromuscular transmission * **IgG Antibodies form against the presynaptic voltage gated Ca++ channels** = ↓ release of Ach * If tumor present, antibodies are directed at tumor but cross-react w/ Ca++ channels
51
SLE Anesthesia Considerations: Neuraxial and regional nerve blocks
* Currently taking **anticoagulants or known coagulopathy?** * Presence of a **peripheral nerve lesion?**
52
Osteoarthritis Clinical Features
* Degenerative changes are most significant in **middle/lower cervical and lower lumbar** * Associated protrusion (herniation) of nucleus pulposus (slipped disk) resulting in nerve root compression * Occurs in **weight bearing joints** or in conditions that put undue stress on joints * **Obesity** * **Joint deformity**
53
OA Anesthetic Considerations: Pneumatic Tourniquets
Pneumatic Tourniquets * Provides bloodless field that greatly facilitates surgery * **Inflation pressure ~ 100 mmHg over SBP** * Prolonged inflation (\>2h) = pain, nerve damage * Be careful not to over-administer opioids for tourniquet pain! * Deflation * **Hemodynamic changes** (*↑CO2 and hypotension with “wash-in”)* * Washout of accumulated metabolic wastes
54
**Myasthenic Syndrome or Myasthenia Gravis?** Extraocular, bulbar, and facial muscle weakness
**Myasthenia Gravis**
55
Achondroplasia
* Most common cause of **Dwarfism** * Caused by **premature ossification** of bones combined w/ normal periosteal bone formation * Results are characteristic appearance of short limbs and relatively normal cranium * Often present to OR for sub-occipital craniectomy for foramen magnum stenosis, laminectomy (spinal stenosis/nerve root compression), VP shunt * Consider need for **VAE monitoring** (VAE sitting crani) * **Evoked potential monitoring** (limits anesthetic options)
56
Marfan's Clinical Manifestations: CV
* **Aortic aneurysm and dissection** remain most life-threatening manifestations (↑ risk in pregnancy) * Cardiac Valve Prolapse/dysfunction/regurg * Mitral Valve * Aortic valve – incompetence often related to dilation of ascending aorta * Arrhythmias- BBB * Aortic and atrioventricular valves prone to calcification
57
Scleroderma
* Inflammation & autoimmunity, vascular injury, fibrosis * Females * Pregnancy may accelerate symptoms * Unknown etiology; no treatment available * Can lead to CREST syndrome * **C**alcinosis, **R**aynaud phenomenon, **E**sophageal hypomotility, **S**clerodactyly, **T**anglectasia
58
Myasthenia Gravis Anesthesia Considerations: Maintenance
Deep anesthesia w/ VA can provide sufficient relaxation for many sx procedures
59
Scleroderma multi-system effects: Musculoskeletal
* Myopathy * Proximal skeletal muscle weakness
60
Lupus (SLE) Manifestations: CNS
1/3 of pts have cognitive symptoms (can be vague; agitation)
61
**Myasthenic Syndrome or Myasthenia Gravis?** Resistant to Sch, sensitive to NDMR
**Myasthenia Gravis**
62
Myasthenia Gravis: Treatment
* Usually oral **anticholinesterase +/- steroid** therapy * Cholinesterase inhibitors – PO Pyridostigmine * Onset 30 min, peak 2 hrs, DOA 3-6 hrs * Higher doses paradoxically enhance muscle weakness- “cholinergic crisis” * Corticosteroids – Prednisone limits antibody production (second line therapy after failure of above) * Thymectomy – surgical approach * Median sternotomy or mediastinoscopy * Plasmapheresis – removes antibodies during crisis (temporary) * Depletes plasma esterase levels * **Prolonged effect of succ, mivacurium, ester-linked LA’s, etc.**
63
**Myasthenic Syndrome or Myasthenia Gravis?** Reflexes absent or decreased
**Myasthenic Syndrome**
64
Achondroplasia Anesthesia Considerations: General (other than airway, cv, pulm)
* Normal response to anesthetic agents and NMB * Difficult IV and CVC access * Short neck * Excess skin and SQ tissue
65
Ankylosing Spondylitis Clinical Manifestations: Pulmonary abnormalities
* Pulmonary fibrosis * Apical cavity lesions * Pleural thickening (similar to TB) * Decreased compliance of chest wall * Decreased vital capacity
66
Muscular Dystrophy--Duchenne’s/Pseudohypertrophic: Clinical Manifestations: Respiratory
* Respiratory muscle degeneration * Progressive **respiratory muscle weakness** * **Inadequate cough** and retention of secretions = Frequent pulmonary **infections** * Kyphoscoliosis and muscle degeneration → severe **restrictive** disease pattern * Sleep apnea common * **Pulm HTN** w/ disease progression
67
Muscular Dystrophies
* A range of congenital muscular disorders characterized by progressive weakness and degeneration of muscle * Affected individuals produce **abnormal dystrophin** * Protein found on the sarcolemma of muscle fibers * Increased permeability of skeletal muscle precedes symptoms * Painless degeneration * Atrophy of skeletal muscle * Classified according to inheritance * X-linked: **Duchenne’s** (pseudohypertrophic), Becker * Autosomal recessive: limb-girdle, congenital * Autosomal dominant: facioscapulohumeral, oculopharyngeal
68
RA: Airway Evaluation and Management
* **Document pre-op ROM limits, baseline symptoms of pain, numbness, weakness** * ***_Consider awake fiberoptic intubation_*** * **Determine head positions awake that can be tolerated** * **Ask about tingling hands/feet, pain, assess ROM** * **Avoid excessive movements during laryngoscopy** * _Temporomandibular joint (TMJ)_ * Assess mouth opening, may be limited * TMJ w/ cervical spine immobility may limit DL visualization * _Cricoarytenoid joints_ * Fixation may present as voice changes or hoarseness * Arthritis and inflammation can make **glottic opening difficult** to identify or stenotic * **Cricoarytenoid arthritis** = ↓ ETT size * Minimal edema may lead to airway obstruction postop * _C-Spine_ * **Atlantoaxial subluxation** (AAS) * Distance b/t anterior arch of atlas to odontoid process \>3mm on xray (during neck extension) * Occurs in 25% of severe RA pts * R/f cervical spinal cord/medulla compression by odontoid process and interference w/ vertebral artery blood flow
69
Scleroderma multi-system effects: Pulmonary
* Fibrosis * Restrictive pattern * ↓ compliance
70
Achondroplasia Anesthesia Considerations: Pregnant patient
* **C-section** required * Cephalopelvic disproportion * **Kyphoscoliosis** and narrow epidural space, spinal canal, osteophytes, vertebral disk/body deformity **increase technical difficulty** * No evidenced based dosing guidelines (epidural might be better so that you can titrate LA slowly)
71
**Myasthenic Syndrome or Myasthenia Gravis?** Affects males more often than females
**Myasthenic Syndrome**
72
Myasthenic Syndrome: Clinical Features
* Presents w/ **proximal muscle weakness** * Typically **begins w/ lower extremities**, spreads to upper limbs, bulbar, respiratory muscles * Change in gait, ability to stand, climb stairs * **Exercise improves strength** * **Autonomic dysfunction** very common * Hemodynamic variability * Dry mouth * Urinary hesitancy *improves with repeated effort*
73
Myasthenia Gravis Anesthesia Considerations: Induction
* Short-acting agents (Propofol) * Consider intubation *without* muscle paralysis * Intubation w/ deep VA may be sufficient * Topical lidocaine application to airway * If must use NDMB, use PNS at orbicularis oculi (may overestimate block) * **NDMB potency ↑ 2X: ↓dose 33-50% if using** * **Aspiration risk - consider RSI** * Sch dose may be **↑ to 2 mg/kg** to overcome resistance, but anticipate **prolonged effect** * If Sch used - **do not use NDMR** until muscle function has returned
74
Muscular Dystrophy Anesthesia Considerations
* Muscle weakness, CV & pulmonary involvement * Preop sedation/opioids best **avoided** * Respiratory muscle and laryngeal reflex weakness * Gastric hypomotility * **Aspiration prophylaxis** * Antacid with H2 blocker or PPI * Prokinetic like metoclopramide to ↓ volume * Antisialogogue like glycopyrrolate if sections are an issue * **Positioning** can be complicated by kyphoscoliosis and flexion contractures * Regional anesthesia may be preferable
75
Myasthenia Gravis: Clinical Features
* Presentation varies from **mild weakness of limited** muscle groups (class I or ocular MG) to **severe weakness of multiple** muscle groups (class IV or severe generalized MG) * **Ocular muscles** most commonly affected * Ptosis, dysphagia and diplopia (most common initial complaints) * **Bulbar** involvement/Laryngeal and pharyngeal muscle weakness * Difficulty clearing secretions → Pulm aspiration * Myocarditis possible * A-Fib, heart block, cardiomyopathy * Severe dz associated w/ **asymmetrical proximal muscle weakness** (no atrophy though) * Neck, shoulders, respiratory muscles
76
Rheumatoid Arthritis: presentation
* **Symmetrical poly-arthritis** in joints * Early disease * Hands, Wrists * Feet, Ankles * Later progression * Shoulders, Elbows, Knees * Temporomandibular joint (**TMJ**) * Cervical spine (**atlantoaxial** instability and cord compression)– *keep a neutral spine; use video laryngoscope; let pt position themselves*
77
RA Treatment Options
* Meds to treat **pain and inflammation** * ASA, NSAIDS (associated blood loss) * Corticosteroids (for acute periods only) * **Surgical** Treatment to **r****elieve pain**and**restore joint function** * Tendon release procedure, synovectomy, joint replacement * **DMARDS** – alter immune response, slow progression * Methotrexate (bone marrow suppression, cirrhosis) * Azathioprine * Sulfasalazine (anti-inflammatory, mild immunosuppressant) * Antimalarial drugs * Minocyclin * TNF inhibitors/monoclonal antibodies (de-myelinating syndromes, infection??)
78
Achondroplasia Anesthesia Considerations: Airway Management
* Challenges with airway management * Facial features can lead to **difficult mask** management * Large protruding forehead, short maxilla w/ long mandible, flat nose, large tongue * Larynx may be small and intubation occasionally difficult * Difficult to expose glottis * **Range of ETT sizes** and **difficult airway cart** available * **Weight** rather than age is best guide for predicting proper size * **Foramen magnum stenosis**, fusion of the atlantooccipital joint with the odontoid, atlantoaxial instability, bulging discs, cervical kyphosis common * Avoid hyperextension during intubation
79
RA Additional Clinical Manifestations: Cardiac
* **Dysrhythmia** d/t rheumatoid nodules in cardiac conduction system * Cardiac valve fibrosis * **Pericarditis** * Myocarditis * Coronary artery arteritis * Dilation of aortic root- aortic regurgitation
80
Osteoarthritis Pathogenesis
* Commonly referred to as Degenerative Joint Disease (DJD), ‘wear and tear’ * Hallmark is **degeneration of articular cartilage** * Most commonly **hip and knee** * Differs from RA in that there is **minimal inflammatory reaction**
81
SLE Manifestations: Liver
* Biliary cirrhosis * Autoimmune hepatitis
82
Marfan’s Anesthesia Considerations: CV and Pulm
* Pre-op assessment should concentrate on **CV abnormalities** * Continue **beta blockade** peri-op * Continuously monitor for **PTX** * **Hemodynamic Stability is critical** * Prevent sudden ↑ in myocardial contractility as this produces an ↑ in aortic wall tension * Avoid excessive endogenous catecholamine production * Control pain and anxiety * Perform hemodynamic stable induction * **Treat HTN** immediately * VA’s can ↓ force of cardiac ejection = ↓ r/f aortic dissection
83
**Myasthenic Syndrome or Myasthenia Gravis?** Affects females more often than males
**Myasthenia Gravis**
84
Ankylosing Spondylitis Treatment
* Management of AS patients * **Relieve pain, reduce inflammation, and maintain good posture and function** * Traditional treatments include **NSAIDs**, education, exercise, and physical therapy * Exercise and PT to **maintain joint mobility and flexibility** * Early diagnosis/treatment essential to **prevent permanent posture and mobility loss** * **Surgery** considered in pts w/ severe, advanced dz associated w/ **refractory pain and disability**
85
Marfan's Syndrome: Treatment
* **Beta Blockers** = standard of care * Reduce workload of heart * Delay/prevent aortic aneurysm and dissection * Surgical procedures * Elective surgery to repair aortic root recommended when maximum aortic diameter reaches 4.5 cm * Spinal fusion for scoliosis
86
Marfan’s Anesthesia Considerations: Airway eval and management
* Airway evaluation and management * High arched palate w/ crowded teeth * Visualization of larynx during DL rarely difficult * TMJ dislocation * Tendency for joint laxity * Avoid extreme movement of mandible * **Tracheomalacia** * Floppy tracheal cartilage leading to **tracheal collapse**
87
What disease is characterized by symmetric proximal muscle weakness manifesting in gait disturbance?
Duchenne's
88
**Myasthenic Syndrome or Myasthenia Gravis?** Poor response to anticholinesterases
**Myasthenic Syndrome**
89
OA Anesthetic Considerations: Bone Cement
* Bone Cement (polymethylmethacrylate or PMMA) * “Cement” misnomer (not bonding 2 things together). Space-filler creates tight space & holds implant against bone, more like ‘grout’. * **Bone cement implantation syndrome** (fat/marrow embolisms) * Hypoxia (increased pulmonary shunt) * Hypotension * Pulm HTN * Dysrhythmias (heart block and sinus arrest) * ↓ CO
90
**Myasthenic Syndrome or Myasthenia Gravis?** Exercise improves strength
**Myasthenic Syndrome**
91
Marfan's Syndrome
* Multi-System disorder of **connective tissue** caused by mutations in extracellular matrix protein **fibrillin 1** * Typically involving **CV, skeletal, and ocular** systems: Classic manifestations include proximal aortic aneurysm, dislocation of ocular lens, long-bone overgrowth
92
Systemic Lupus Erythematosus (SLE): Diagnosis
* Antinuclear antibodies (95% of patients with SLE) * Nephritis * Rash * Raynaud’s * Serositis * Thrombocytopenia
93
Scleroderma multi-system effects: GI
* Dysphagia * Hypomotility of lower esophagus & small intestine * ↓ LES tone *(r/f GERD)* * Malabsorption
94
**Myasthenic Syndrome or Myasthenia Gravis?** Good response to anticholinesterases
**Myasthenia Gravis**