Anesthesia and Musculoskeletal Diseases Flashcards

1
Q

What is Ehlers-Danlos Syndrome?

A
  • Joint hypermobility and lacity d/o
  • congenital
  • bleeding tendency
  • can have spontaneous rupture bowel, uterus, large arteries
    • increased risk during pregnancy
  • risk for pneumo
  • regional is not recommended d/t high incidence of hemtatome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Overview of scleroderma?

A
  • Inflammation and autoimmunity, vascular injury, fibrosis
  • generally affects females
  • pregnancy may accerlearte symptoms
  • unknown etiology, no treatment available
  • Can lead to CREST syndrome
  • Multi system effects
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is CREST syndrome?

A

afflict scleroderma patient

  • Calcinosis
  • Raynaud’s phenomenon
  • Esophageal hypomotility
  • Sclerodactyly (hand stiffness)
  • Tangelectasia (bunch of blood vessels inside body/skin lesion)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How does scleroderma affect skin and musculoskeletal system?

Anesthesia implications?

A
  • Skin- limited mobility; flexion contractures; thickened taut skin
    • dififcult IV placement
    • difficult regional
    • small oral aperture- difficult to open mouth
      • potential difficult airway- may need fiberoptic
  • Musculoskeletal: myopathy, proximal skeletal muscle weakeness
    • neck ROM issue, positioning issues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are some CNS effects of scleroderma?

ENT effects?

Anesthesia implications?

A
  • Thickened connective tissue around nerve sheath leading to neuropathy, high incidence trigeminal neuralgia
    • may be on opioids for trigeminal neuralgia, may have upregulated opioid receptors
    • if there’s chronic wasting/weakness in muscles, may need avoid succiniylcholine if poor activity tolerance
    • document any existing neuropathies
  • ENT- dry eyes, oral/nasal tanglectasia, dry mouth
    • avoid OPA/NPA
    • Protect eyes!
    • fiberoptic intubation
    • patient will most likely have sore throat when waking up
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

CV effects scleroderm?

Pulmonary?

Anesthesia implications?

A
  • CV:
    • myocardial tissue replaced with fibrotic tissue,
    • conduction abnormalities
    • contracted vascular volume
      • from book- may produce hypotension during induction of anesthesia when anesthetic drugs with vasodilating properties exert their effects
    • high incidence of pulm hypotension,
      • avoid n2o, anything that increases pulmonary vascular resistance (avoid Hypercarbia, hypoxia)
    • vasopasm’s- raynauds
      • keep patient warm to prevent vasospasm’s
      • may see vasospasm on a-line
        • if you lose art line with no change ETCO2, probably art line issue
        • if lose art line and ETCO2 down- then bad news! probably cv collapse
  • Pulmonary:
    • fibrosis,
      • may have baseline o2 requirement
    • restrictive pattern,
    • decreased compliance
      • may need decreased TV and faster RR
    • careful with opioids!
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some GI effects of scleroderma?

Renal effects?

Anesthesia implications?

A
  • GI:
    • dysphagia
      • increased risk aspiraiton
    • hypomotlity of lower esopagus and small intestine
    • lower esophageal tone decreased= regurg symptoms and increased risk aspiration
      • give prophylactics (antacid, PPI)
      • May need RSI
    • malabsorption
      • may need vit K if absorption issues
  • Renal
    • renal hypertension (ACE inhibitors effective)
      • assess renal preop, may need to change antibiotics
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Broad overview lupus?

A
  • Multisystem inflammatory disease characterized by immune-mediated tissue damage
  • Mainly females (1:1000)
  • Presence of HTN and nephritis: poor prognosis
  • Onset of SLE may be drug induced (milder form)
    • hydralazine, procainamide, isoniazid, methyldopa
    • slow acetylators at risk
  • Physiologic stress may exacerbate the disease
    • sx, infection, pregnancy
      • poor fetal outcomes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Diagnosis of SLE?

A

Dx

  • Antinuclear antibodies (95% of pt with SLE) (aka ANA)
  • Rash
  • Thrombocytopenia
  • Serositis
  • Nephritis
  • Raynaud’s
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are some general effects in the body from inflammation and vasculities from SLE?

A
  • Vessel wall thickening, weakeneing, narrowing, scarring
    • CAD, stroke risk
  • HTN
  • Pulm HTN
  • Thromboembolism
  • Hypercoagulable state
  • femolytic anemia
  • frequent fevers
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are some skin, joint and muscle manifestations of SLE?

Anesthesia implications?

A
  • Skin
    • Butterfly rash with nasal erythema (50%)
    • oral and pharyngeal ulcers
      • avoid OPA/NPA
  • Joints/muscle
    • symmetrical arthritis (90%)
      • no spinal involvement
      • migratory and episodic
    • circoarytenoid arthritis
      • dififcult intubation
    • AVN
    • Myopathy
    • tendon ruptures
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Airway effects of SLE? Pulmonary effects?

Consideration for anesthesia?

A
  • Laryngeal involvement in up to 1/3 patients
    • mucosal irritaiton
    • laryngeal erythema and edema common
    • circoarytenoid arthritis
    • recurrent laryngeal nerve palsy
  • Lungs
    • “lupus pneumonia”
      • diffuse pulmonary infiltrates, pleural effusion, dry cough, dyspnea, arterial hypoxemia
    • restrictive pattern
      • lower TV and higher RR may be needed for ventilation
    • Recurrent atelectasis (phrenic nerve neuropathy)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What effect does Lupus have on renal system? Hepatic effects?Anesthesia considerations?

A
  • Glomerulonephritis leading to nephrotic syndrome (protein in urine) and renal failure
    • check renal status, may need to alter abx other drugs
  • Liver
    • biliary cirrhosis
    • autoimmune hepatitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are some CNS effects of lupus?

A

1/3 have cognitive symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Treatment for mild SLE? Severe?

A

Mild

  • NSAIDS for join symptoms and pleurisy
  • low dose corticosteroids such as prednison
  • antimalarial drugs (hydroxycholoroquine/quinacrine) and low dose corticosteroids for thrombocytopenia, hemolytic anemai, skin and arthritis symptoms
    • from John’s Hopkins: Anti-malarial medications help to control lupus in several ways by modulating the immune system without predisposing you to infection. Anti-malarials can protect against UV light and sometimes even improve skin lesions that do not respond to treatment with topical therapy

Severe

  • High dose coritocsteroids (need stress dose?)
    • chronic steroids- CAD accelerated
  • Immunosuppressive/chemo drugs
    • methotrexate, cyclophosphamide, azathioprine, mycophenolate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the current data on stress dose steroids in periop setting?

A
  • If patient already on steroids, continue at usual dose if possible
  • data supporting admin of stress dose limites, deicison to administer stress dose depends on procedure
  • small procedure (dental work, skin bx) no stress dose necessary
  • moderate proceudres give 25 mg hydrocortisone every 8 hours, then taper over 1-2 days
  • Major sx give 50 mg hydrocortisone every 8 hours, then taper over 2-3 days.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are some considerations for neuraxial and regional nerve blocks in SLE?

A
  • Currently taking atnicaog or known caogulopathy
    • want to see PLT, coags before regional
  • presence of peripheral nerve lesion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Anesthetic considerations for SLE?

(non-med related?)

A
  • Interactions with drugs used in SLE treatment
  • degree of organ dysfunction
    • impaired renal
    • hepatic clearance of drugs
    • cardiopulmonary involvement
  • Strict asepsis with invasive procedures
    • reduce the already increased risk of infection
  • Maintain normothermia
    • may reduce risk of infection
    • lessening impact of raynaud’s if present
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How can some of your anesthesia drugs be affected in SLE?

A
  • Most commonly used drugs in anesthesia are at least partly dependent on renal excretion for elimination
    • dose/frequency may need to be modified to prevent accumulation of drug
  • IV Agents
    • pharmacokinetics prop/etomidate- not sig affected by renal impairment
    • Benzos- undergo hepatic metabolism and conjugation prior to elimination in urine
    • Opioids- accumulation of morphine and meperidine metabolites prolong resp depression
  • Volatile agents- ideal due to lack of dependenc eon kidney for elimination
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is rhuematoid arthritis?

A
  • Chronic, systemic inflammatory disorder/auto-immune with articular and systemic involvement
    • characterized by exacerbations and remissions
    • affects about 1% of adults (females>males)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is RA’s manifestations in joints/spine?

A

Symmetrical poly-arthritis in joints

  • Early disease- may need special protection/padding for joints
    • hand
    • wrists
    • feet
    • ankles
  • Later progression- may need special protection for joints
    • knees
    • elbows
    • shoulders
  • TMJ
  • Cerival spine (atlantoaxial instability and cord compression)
    • make sure patient goes into sniffing position on own or maintain head neutral. may need glidescope
    • tenderness on laryngeal palpation, hoarseness can be indication of cricoarytenoid arthritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are some extra-articular manifestations of rheumatoid arthritis?

A
  • Skin- special protection needed?
    • raynaud’s- keep warm
    • digital necrosis
  • eyes
    • scleritis
    • corneal ulceration- protect eyes
  • kidney- Look at BUN/Cr
    • interstitial fibrosis
    • glomerulonephritis
    • amyloid deposition
  • peripheral nervous system
    • compression syndromes
    • mononeuritis
  • Central nervous system
    • dural nodules
    • necrotizing vasculities
  • liver
    • hepatitis
  • blood
    • anemia- assess CBC
    • leukpenia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are some cardiac manifestations of rheumatoid arthritis?

A
  • Dysrhythmia from rhumatoid nodules in the cardiac conduction system
  • cardaic valve fibrosis
  • pericarditis
  • myocarditis
  • coronary artery arteritis
  • dilation of aortic root- aortic regurg
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What are some pulmonary manifestations of rheumatoid arthritis?

A
  • Pleural effusion and restirctive lung disease due to rheumatoid nodules in the lung tissue
    • decreased lung volume
      • look at PFTs
    • pulmonary fibrosis (Rare)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What are treatment options for rheumatoid arthritis?
* **Drugs to treat inflammation/pain** * ASA * NSAIDS * chronic nsaid use might affect coagulation * corticosteroids (acute periods only) * **DMARDS**- alter immune response and slow disease progression. **DO NOT GIVE DMARD WITH NSAID** * methotrexate\< **check LFT/CBC** * azathioprine * antimalrial drugs * minocyclin * TNF inhibitors/MAD * **SX treatment** * relieve pain and restore joint funciton * tendon release procedure, synovectomy, joint replacmenet
26
What are some considerations when evaluating/managing the airway of someone with RA?
* Document preop ROM limits, baseline symptoms of pain, numbness, weakness * consider awake fiberoptic intubation * TMJ- * assess mouth opening * may be limids * TMJ with 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 ID or stenotic * cricoarytenoid joint arthritis requiring decreased tube size * minimal edema may lead to airway obstruction postoperatively * Atlantoaxial instability
27
What are some considerations for the c-spine in patient with RA?
* Atlantoaxial subluxation (AAS) * distance between ant arch of atlas to odontoid process \>3mm on radiologic imaging (during neck extension) * occurs in 25% severe RA patients * risk of cervical spinal cord/medulla compression by odontoid process and interference with vertebral artery blood flow * **determine head positions AWAKE that can be tolerated** * **enquire about tingling of hands/feet, pain and assess ROM** * **AVOID EXCESSIVE MOVEMENT DURIGN LARYNGOSOCPY**
28
How does RA affect management of anesthesia?
* Anemia of chronic disease * effect of meds on PLT function * with lung DX * PFT/intraop ABG/post op vent support * may need corticosteroids * extubate with caution in those with cricoarytenoid arthritis
29
What is the pathogenesis of osteoarthritis?
* commonly referred to as degenerative joint disease (DJD)"wear and tear" * Hallmark is degerneration of articular cartilage * most commonly hip/knee * **differ from RA in that there is minimal inflammatory reaction** * typically in middle/lower cervical and lower lumbar * associated protrustion of nucleus pulposus resulting in nerve root compression * occurs in weight bearing joints or conditions that put undue stress on joint * obesity * joint deformity
30
Anesthetic considerations for osteoarthritis?
* positioning * support affected joints * position to minimize risk of injury * drugs used in treatment * corticosteroids not recommended * NSAIDS and ASA * potential bleeding issues
31
What are anesthetic considerations specifically during reconstructive joint surgery?
* Bone cement (polymethylmethacrylate or PMMA) * "cement" is a misnomer, not bonding two things together * Bone cement implantation syndrome (fat and marrow embolisms) * hypoxia (increased pulm shunt * hypotension * dysrhythmia * pulm hypertension * decreased CO
32
What are anesthetic considerations with tourniquet use?
* Provides a bloodless field that greatly facilitates surgery * inflation pressure usually aboug 100 mmHg over systolic blood pressure * prolonged inflation \>2h, pain, nerve damage * be careful not to over administer opioids for touniquet pain! * *better to admin esmolol to temporarily decrease BP* * Deflation * hemodynamic changes * washout of accumulated metabolic wastes * can cause drop in BP
33
What is pathophys of myasthenia gravis?
Autoimmune destruction of postsynaptic acetylcholine receptors at NMJ (up to 80% loss) * characterized by weakness and rapid exhaustion of skeletal muscle * particularly those innervated by cranial nerves (ocular, pharyngeal and laryngeal) * course marked by exacerbations & remissions * 1:7500 (women 20-30 and men \>60 yo) * sx, infection, stress and pregnancy lead to exacerbations * association with thrmic hyperplasia and thymoma
34
What are some clinical features of myasthetnia gravis?
* Mild to severe weakness * Class I = mild * class IV= severe * ocular muscle most commonly affected * ptosis, dysphagia, and diplopia (most common initial complains) * Bulbar involvement/laryngeal and pharyngeal muscle weakness * difficulty clearing secretions * pulm aspiraiton * myocarditis possible * afib, heart block, CMP * Severe dx associated with asymmetrical proximal muscle weakeness (no atrophy though) * neck, shoulder, respiratory muscles affected
35
Treatment for myasthenia gravis?
* Usually oral anticholinesterase with or without steroid therapy * **Cholinesterase** **inhbiitors** (PO Pyridostigmine) * onset 30 min, peak 2 hours, DOA 3-6 HR * Higher dose can paradoxically enhance muscle weakeness= "cholinergic criss" * **Corticosteroids**- prednisone limits antibody production (second line therapy after failure of above) * Thymectomy- sx approach * can be cure for some pts * median sternotomy or mediastinoscopy * Plasmapheresis- removes antibodies during crisis (temporary) * depletes plasma esterase levels * prolonged effect of succ, mivacurium, ester linked LA etc
36
Anesthetic considerations for mysthenia gravis?
* Elective surgery only during remission * premedication/opioids minimized or avoided * prone to weakness * aminoglycoside antibiotics can enhance weakness * **Induction** * **​**short acting agents (ie prop) * consider intubation without muscle paralysis * intubation with deep volatile agent may be sufficient\<-- synergistic effects with MR * topical lidocaine application to airway * if using NDMB, should use PNS at obicularis oculi (may overestimate block) * Aspiration risk- consier RSI * may need higher does succinylcholine (2mg/kg) to overcome resistance, but prolonged effect should be anticipated * if succinylcholine is used- do not use NDMR until muscle function has returned--\> phase II block
37
Anesthetic considerations for mainetnace and emergence with myasthetnia gravis?
* Maintenance * deep anesthesia with volatile agent can provide sufficient relaxation for many surgical procedures * Emergence * at great risk for postop respiratory failure * advise patient may awaken with ETT in place * careful evaluation of vent function prior to extubation * close observation in recovery
38
What is myasthenic syndrome?
* Referred to as Lambert-Eaton myasthenic syndrome (LEMS) * presents with proximal muscle weakeness * develops in association wiht a neoplasm, but also seen with other ocult malignancies or idiopathic autoimmune * **usually small cell carcinoma of lung** * Disorder results form **presynaptic** side defect of NM transmsission * **IgG** antibodies form agianst presynaptic voltage gate calcium channel * decreased release of ACh * If tumor present, these antibodies are directed at the tumor but cross-react with calcium channels * when tumor resolves, symptoms resolve
39
What are some clinical features of myasthenic syndrome? Where is weakness typically noted? How are hemodynamics potentially affected?
* Proximal muscle weakness (most central muscles) * typically begin with lower extremities, spreads ot upper limbs, bulbar, respriatory muscles * change in gait, ability to stand, climb stairs * exercise improves strength * Autonomic dysfunction very common * hemodynamic instability * dry mouth * urinary hesitancy
40
What are anesthesia considerations for mysthenic syndrome?
* May be undiagnosed. suspect in bronc, mediastinosocpy, thoracoscopy for suspected lung Ca * unlike myasthenia gravis, LEMS pts are very sensitive to both depolarizing and nondepolarizing NM blocker * AChe drugs not helpful in these disease and may not effectively reverse clinical effects of NDMR * Volatile agnets alone often sufficient to provide muscle relaxation for both inbuation and surgical procedures * NMBAs should only be givne in small increments and with careful NM monitoring
41
What are manifestation, affected gender, co-existing patho conditions, and response to muscle relaxants in myasthenic syndromes vs myasthenia gravis
**MYASTHENIC SYNDROME** * Manifestations- proximal limb weakenss (legs more than arms), exercise improves strength, muscle pain common, reflexes absent/decreased * **Gender-** affects male more htan femlas * **Co-exist condition: small cell lung Ca** * **Response to muscle relaxants** * **​**sensitive to sucicnylcholine AND non depolarizing MR * Poor response anticholinesterases **MYASTHENIA GRAVIS** * **Manifestations**- extraocular, bulbar and facial muscle weakness; exercise causes fatigue; muscle pain unommon * **Gender:** affects females more often than males * **Co-existing patho conditon-** thymoma * **Reponse to MR-** * **​resistant** to succinylcholine (need more! up to 2mg/kg) * **sensitive** to nondepolarizing MR * **Good** response to anticholinesterases
42
What is pathophys behind muscular dystophy?
* A range of congenital muscular disorders characterized by progressive weakness and degeneration of muscle * affected individuals produce abnormal dystrophin * protein found on sarcolemma of muscle fibers * **increased permeability of skeletal muscle precedes symptoms** * Painless degeneraiton * atrophy of skeletal muscle * Classified according to inheritane * x-linked: Duchenne's (psudohypertrophic) and Becker * most common * autosomal recessive: limb-girdle, congenital * autosomal dominant: facioscapulohumeral, oculopharyngeal
43
What is the most common and severe form of MD? gender affected? characterized by? Prognosis?
* Duchenne's (pseudohypertrophic) * ALmost exclusively male * incidence 1-3 per 10,000 live male births * presents between 2-5 yo * Characterized by symmetric proximal muscle weakness manifesting in gait disturbance * pelvic girdle * psudohypertrophic- fatty infiltration * Kyphoscoliosis results form progressiv eweakness and contractures * muscle weakness pronoucnced in proximal extremities * wheelchair by age 8-10 * fatal by 20 yo
44
What are some cardiac dysfunctions associated with muscular dystrophy? Anesthesia considerations?
* Degeneration of cardiac muscle- *may see cardiopulmonary depresison with volatile anesthetics* * ECG abnormalities * atrial arrhythmias * prolonged PR interval * Decreased myocardial contractility and CMP * Mitral regurg secondary to papillary muscle dysfunction
45
What are some pulmonary issues associated with muscular dystrophy?
* Chonic weakness of inspiratory muscles * decreased ability to cough and clear secretions * Frequent **pulmonary infections** * **​*anticipate post op pulmonary dysfunction*** * **kyphoscoliosis** and muscle degernation lead ot severe restrictive disease pattern * **sleep apne**a is common * **pulmonary hypertension** with disease progression
46
Preop Anesthetia considerations with muscular dystrophy?
* Muscle weakness, cardiac and pulm involvement * preop sedation/opioids best avoided * resp muscle and laryngela reflex weakness * gastric hypomotility * aspiration prophylaxis * antacid with H2 blocker or PPI * Prokinetic like reglan to reduce volume * antisialogogue like glyco if secretions are an issue * Positioning can be complicated by kyphoscoliosis and flexion contractures
47
Anesthesia considerations during induction for muscular dystophy?
* Succinylcholine contraindicated * risk of inducing severe hyperkalemia, rhabdomyolysis, v fib and cardiac arrest * malignant hyperthemia risk
48
Anesthesia considerations during maintenance for muscular dystophy?
* Marked cardiopulmonary depression may be seen with voltaile anesthetics * normal to prolonged resposne to NDMR * Anticipate post op pulm dysfunction * Regional anesthesia may be preferable
49
What is marfan syndrome?
* Multi system disorder of the connective itssue caused by mutations in extracellular matrix protein fibrillin 1 * classic marfan's syndrome occurs in about 4-6 our of 100,000 people * involve cv, skeletal and ocular systems * classic manifestations include proximal aortic aneurysm, dislocation of ocular lengs and long-bone overgrowth * *need to see recent echo, cardiac workup*
50
CV manifestations of marfan's syndrome?
* Aortic aneurysm and dissection remain most life-threatening manifestations (increased risk in pregnancy) * Cardiac valve prolapse/dysfunction/regurg * mitral valve * aortic valve- incompetence often related to dilation of ascending aorta * arrhythmia- BBB * aortic and atrioventricular valves prone to calcification
51
Pulmonary manifestations of marfan's syndrome?
* High incidence pneumothorax * spinal/sternal deformities can lead to restrictive lung disease
52
Ocular manifestations of marfan's syndrome?
* Dislocation of ocular lengs * Retinal detachment
53
What is standard treatment for marfan's?
* Beta blockers is standard of care * reduce workload of heart * delay/prevention aortic aneurysm and dissection * Surgical procedures * elective aoritc repair * may electively repair aortic root when \>4.5 cm * spinal fusion for scoliosis * *may be prone and that increases risk of ocular dislocation*
54
Marfan syndrome anesthesia consideration for airway?
* Preop assessment should concentrate on cardiac abnormalities * airway evaluation and management * high arched palate with crowded teeth * visualization of the laryngx during direct larngosocpy rarely difficult * TMJ dislocation * tendency for join laxity * avoid extreme movement of mandible * Tracheomalacia * floppy tracheal cartilage leading to tracheal collapse * Beta blockade- should b econtinued perioperativley * Continuous monitor for pneumothorax!
55
What is critical to maintain during anesthesia for marfan's syndrome?
hemodynamic stability! * Prevent sudden increase in myocardial contractility * this produces an increase in aortic wall tension * Avoid excessiv eendogenous catecholamine production * control pain/anxiety * hemodynamic stable inudction should be performed * Treat hypertensive episodes immediately * volatile anesthesia can decrease force of cardaic ejection * decrease risk of aortic dissection
56
What is ankylosing spondylitis?
* Chronic, progressive inflammatory disease involving joints of spine and adjacent soft tissues * associated with HLA B27 in most cases * male to feamle ratio 4:1 * important anesthesia implications both articular and non-articular
57
What is standard treatment for anklyosing spondylitis patients?
* Relieve pain, reduce inflmmation, maintain good posture and function * traditional treatments include NSAIDS, education, exercise, and PT * exercise/PT maintain joint mobility and flexibility * early dx is essential * surgery considered in patients with sever,e advanced dx associated with refractory pain or disability
58
Airway manifestations of ankylosing spondylitis?
Difficult intubation associated when AS invovles cervical spine * increased difficulty when TMJ involved * limited mouth opening d/t TMJ involvement * sig risk of neuro injury with any excessive neck extension * progressive kyphosis and spine flexion may limit intubation * risk of occult cervical fracture with minimal trauma * cricoarytenoid arthritis * cords susceptible to trauma
59
Cardiac involvement/anesthesia concerns for ankylosing spondylitis?
* Aortic regurgitation **(avoid sudden increases in SVR/keep H R\>90 bpm, low normal BP)** * Conduction abrnomalities- BBB * Cardiomegaly
60
Pulmonary abnormalities in ankylosign spondylitis?
* Pulmonary fibrosis * apical cavity lesions * pleural thickening (similar ot TB) * decreased compliance of chest wall * Decreased VC
61
Anesthetic management for ankylosing spondylitis?
* Anesthetic management influence by severity of dx * upper airway involvement * presence of restrictive lung disease * degree of cardiac involvement * may be using neuro monitoring during corrective spinal sx * Awake fiberoptic tracheal intubaiton if spinal deformity is extensive * spinal and epidural anesthesia is technically difficult * may result in an increased risk of complications * consider paramedian approach
62
What is achondroplasia?
* Most common cause of dwarfism * caused by premature ossification of bones combined with normal periosteal bone formation * results in characteristic appearance of short limbs and a relatively normal cranium * often present to OR for sub-occipital creaniectomy for foramen magnum stenosis, laminectomy, VP shunt * consider need for VAE monitoring (sitting crani) * may need right atrial catheter * evoked potential monitoring (limits anesthetic options) * ensures good cerebral circulation
63
Anesthetic considerations for achondroplasia?
* Normal response to anesthetic agents and NMB * C-section required * cephalopelvic disproportion * kyphoscoliosis and narrow epidural space, spinal canal increase tehcnical difficulty * no evidence based dosing guidlines- epidural may be better so you can titrate local anesthetic slowly * Difficult intravenous and central line access * short neck * excess skin and SQ tissue * Cardiac/pulmonary * pulm HTN leading to cor pulmonale is most common disturbance * restrictive bent defects may occur and lead to pulm htn * central sleep apnea (related to brainstem compression by foramen magnum) and upper airway obstruction
64
Challenges with airway management for patient with achondroplasia ?
* Facial features lead to dififcult mask management * large protruding forehead, short maxilla with long mandible, flat nose and large tongue * larynx may be small and intubation occasionally difficult * difficult to exposre glottis * range of tube sizes and dififcult airway cart avilable * weight rather than age is best guide for predicting porper size * Foramen magnum stenosis, fusion of atlantooccipital joint with odontoid, atlantoaxial instabilty, buling disc, cervical kyphosis common * **avoid hyperextension during intubation**