Musculoskeletal Diseases Flashcards
Ankylosing Spondylitis (AS)
- 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
SLE Manifestations: Heart
- Pericarditis (auscultate friction rub)
- Valvular disease
Achondroplasia Anesthesia Considerations:
Cardiac and Pulmonary involvement
- 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
Scleroderma multi-system effects: Skin
- Thickened taut skin
- Limited mobility
- Flexion contractures
Myasthenia Gravis Anesthesia Considerations: Emergence
- 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
Systemic Lupus Erythematosus (SLE): patho/onset/incidence
- 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)
- Surgery; Infection; Pregnancy
Mild SLE Treatment
- 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
Scleroderma multi-system effects: CV
- 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)
SLE Drugs for Altered Renal Function
- 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
Myasthenia Gravis Anesthesia Considerations
- Elective surgery during remission
- Premed/opioids minimized or avoided
- Prone to weakness
- Aminoglycoside antibiotics can enhance weakness
Muscular Dystrophy Anesthesia Considerations: Induction
-
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.
Myasthenic Syndrome or Myasthenia Gravis?
Sensitive to Sch and NDMR
Myasthenic Syndrome
Stress Dose Steroids in Periop Setting
- 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
SLE Manifestations: Joints/Muscle
- Symmetrical arthritis (90%)
- Cricoarytenoid arthritis
- AVN (avascular necrosis)– can lead to pain
- Myopathy
- Tendon ruptures
Scleroderma multi-system effects: ENT
- dry eyes
- dry mouth
- oral/nasal tanglectasias (r/f bleeding)
Myasthenic Syndrome: Anesthesia Considerations
- 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
Ankylosing Spondylitis Clinical Manifestations:
Cardiac involvement
- Aortic regurgitation (avoid sudden↑ in SVR/ keep HR >90 bpm/low normal BP)
- Conduction abnormalities- BBB
- Cardiomegaly
Myasthenic Syndrome or Myasthenia Gravis?
Muscle pain common
Myasthenic Syndrome
Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:
Clinical Manifestations: Other musculoskeletal
Progressive and severe kyphoscoliosis that results from progressive weakness and contractures
SLE Manifestations: Lungs
- “Lupus pneumonia”
- Restrictive pattern
- Recurrent atelectasis (Phrenic nerve neuropathy)
Chart: pleuritis, pneumonitis, PE, pulm hemorrhage
Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:
Clinical Manifestations: GI
- Reduced intestinal tract tone
- Delayed gastric emptying and ↑ r/f aspiration
Marfan’s Syndrome: Clinical Manifestations (other than CV)
- 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
Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:
Clinical Manifestations: Cardiopulmonary dysfunction
- Degeneration of cardiac muscle
- Chronic weakness of inspiratory muscles = ↓ ability to cough/clear secretions
RA Additional Clinical Manifestations: Pulmonary
- Pleural effusion and restrictive lung dz d/t rheumatoid nodules in lung tissue
- ↓ lung volume
- Pulmonary fibrosis (rare)
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
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)
Myasthenic Syndrome or Myasthenia Gravis?
Co-existing pathologic condition of thymoma
Myasthenia Gravis
Myasthenic Syndrome or Myasthenia Gravis?
Proximal limb weakness (legs more than arms)
Myasthenic Syndrome
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
Myasthenic Syndrome or Myasthenia Gravis?
Muscle pain uncommon
Myasthenia Gravis
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
SLE Manifestations: Kidneys
Glomerulonephritis leading to nephrotic syndrome and renal failure
Myasthenic Syndrome or Myasthenia Gravis?
Co-existing pathologic conditions of small cell lung cancer
Myasthenic Syndrome
Scleroderma multi-system effects: Renal
renal HTN (ACE inhibitors are effective)
Muscular Dystrophy–Duchenne’s/Pseudohypertrophic:
Clinical Manifestations: Cardiac
-
Cardiac muscle degeneration
-
ECG abnormalities
- Atrial arrhythmias
- Prolonged PR interval
- ↓ myocardial contractility and cardiomyopathy
- Mitral regurgitation 2º to papillary muscle dysfunction
-
ECG abnormalities
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
Myasthenic Syndrome or Myasthenia Gravis?
Reflexes normal
Myasthenia Gravis
SLE Manifestations: Skin and membranes
- Butterfly rash w/ nasal erythema (50%)
- Oral and pharyngeal ulcers
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.
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)
SLE Manifestations: Airway
- Laryngeal involvement in ~ 1/3 pts
- Mucosal irritation
- Cricoarytenoid arthritis
- Recurrent laryngeal nerve palsy (hoarseness, dysphagia)
Myasthenic Syndrome or Myasthenia Gravis?
Exercise causes fatigue
Myasthenia Gravis
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
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
Severe SLE Treatment
- High-dose corticosteroids (Stress dose?)
- Immunosuppressive/chemotherapy drugs
- Methotrexate, cyclophosphamide, azathioprine, mycophenolate
Rheumatoid Arthritis
- Chronic, systemic inflammatory disorder/auto-immune w/ articular & systemic involvement
- Characterized by exacerbations and remissions
- Affects about 1% of adults (females > males)
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
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
Scleroderma multi-system effects: CNS
- Thickened connective tissue around nerve sheath leading to neuropathy; high incidence trigeminal neuralgia
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
SLE Anesthesia Considerations: Neuraxial and regional nerve blocks
- Currently taking anticoagulants or known coagulopathy?
- Presence of a peripheral nerve lesion?
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
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
Myasthenic Syndrome or Myasthenia Gravis?
Extraocular, bulbar, and facial muscle weakness
Myasthenia Gravis
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)
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
Scleroderma
- Inflammation & autoimmunity, vascular injury, fibrosis
- Females
- Pregnancy may accelerate symptoms
- Unknown etiology; no treatment available
- Can lead to CREST syndrome
- Calcinosis, Raynaud phenomenon, Esophageal hypomotility, Sclerodactyly, Tanglectasia
Myasthenia Gravis Anesthesia Considerations: Maintenance
Deep anesthesia w/ VA can provide sufficient relaxation for many sx procedures
Scleroderma multi-system effects: Musculoskeletal
- Myopathy
- Proximal skeletal muscle weakness
Lupus (SLE) Manifestations: CNS
1/3 of pts have cognitive symptoms (can be vague; agitation)
Myasthenic Syndrome or Myasthenia Gravis?
Resistant to Sch, sensitive to NDMR
Myasthenia Gravis
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)
- Cholinesterase inhibitors – PO Pyridostigmine
- 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.
- Depletes plasma esterase levels
Myasthenic Syndrome or Myasthenia Gravis?
Reflexes absent or decreased
Myasthenic Syndrome
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
Ankylosing Spondylitis Clinical Manifestations:
Pulmonary abnormalities
- Pulmonary fibrosis
- Apical cavity lesions
- Pleural thickening (similar to TB)
- Decreased compliance of chest wall
- Decreased vital capacity
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
- Progressive respiratory muscle weakness
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
- Affected individuals produce abnormal dystrophin
- Classified according to inheritance
- X-linked: Duchenne’s (pseudohypertrophic), Becker
- Autosomal recessive: limb-girdle, congenital
- Autosomal dominant: facioscapulohumeral, oculopharyngeal
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
-
Atlantoaxial subluxation (AAS)
Scleroderma multi-system effects: Pulmonary
- Fibrosis
- Restrictive pattern
- ↓ compliance
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)
Myasthenic Syndrome or Myasthenia Gravis?
Affects males more often than females
Myasthenic Syndrome
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
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
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
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
Rheumatoid Arthritis: presentation
-
Symmetrical poly-arthritis in joints
- Early disease
- Hands, Wrists
- Feet, Ankles
- Later progression
- Shoulders, Elbows, Knees
- Early disease
- Temporomandibular joint (TMJ)
- Cervical spine (atlantoaxial instability and cord compression)– keep a neutral spine; use video laryngoscope; let pt position themselves
RA Treatment Options
- Meds to treat pain and inflammation
- ASA, NSAIDS (associated blood loss)
- Corticosteroids (for acute periods only)
-
Surgical Treatment to relieve painandrestore 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??)
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
- Facial features can lead to difficult mask management
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
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
SLE Manifestations: Liver
- Biliary cirrhosis
- Autoimmune hepatitis
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
Myasthenic Syndrome or Myasthenia Gravis?
Affects females more often than males
Myasthenia Gravis
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
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
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
- High arched palate w/ crowded teeth
What disease is characterized by symmetric proximal muscle weakness manifesting in gait disturbance?
Duchenne’s
Myasthenic Syndrome or Myasthenia Gravis?
Poor response to anticholinesterases
Myasthenic Syndrome
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
Myasthenic Syndrome or Myasthenia Gravis?
Exercise improves strength
Myasthenic Syndrome
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
Systemic Lupus Erythematosus (SLE): Diagnosis
- Antinuclear antibodies (95% of patients with SLE)
- Nephritis
- Rash
- Raynaud’s
- Serositis
- Thrombocytopenia
Scleroderma multi-system effects: GI
- Dysphagia
- Hypomotility of lower esophagus & small intestine
- ↓ LES tone (r/f GERD)
- Malabsorption
Myasthenic Syndrome or Myasthenia Gravis?
Good response to anticholinesterases
Myasthenia Gravis