Anesthesia considerations for skin and MSK diseases – Flashcards
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What is epidermolysis bullosa?
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Group of genetic diseases of mucous membranes and skin- especially oropharynx and esophagus. Occurs due to mutation in the genes encoding keratin and collagen
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What are the s/s of epidermolysis bullosa?
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Blister/bulla formation d/t intracellular separation within epidermis followed by fluid accumulation -Occurs when lateral shearing forces are applied to skin -restricts esophageal/oral opening, causes fusion of digits
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What is the treatment for epidermolysis bullosa?
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Treatment is supportive, based on symptoms. Corticosteroids are often used.
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What are the anesthetic considerations for epidermolysis bullosa?
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Regional over GA Use propofol/ketamine to avoid airway manipulation No increased risk of hyperK with succs, no known contraindications to use of V.A. Avoid trauma to skin/mucous membranes
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What types of things can precipitate bulla formation during anesthesia for a patient with epidermolysis bullosa?
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Trauma from tape, BP cuffs, tourniquets, EKG electrodes, alcohol wiping skin Anything touching the patient should be padded Gentle pressure with face mask during preoxygenation- lubricate with cortisol ointment Minimize upper airway instrumentation- no oral/nasal airways or esophageal stethoscope
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Why is intubation potentially difficult in patients with epidermolysis bullosa?
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Chronic scarring- narrowed tracheal lumen
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What is pemphigus?
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Chronic autoimmune blistering diseases that may involve extensive areas of skin and mucous membranes May be r/t underlying malignancy- lymphoreticular cancer
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How is pemphigus treated?
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Immunosuppressive tx, rescue therapy with immune globulin Corticosteroids
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What are anesthesia considerations for patients with pemphigus?
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Similar to epidermolysis bullosa Avoid infiltration with LA d/t increased risk of skin sloughing and bulla formation at injection site
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What is psoriasis?
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Chronic dermatologic disorder d/t accelerated epidermal growth resulting in inflammatory erythematous papules covered with loosely adherent scales
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Do people with psoriasis always have skin lesions?
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Not necessarily, lesions are remitting and relapsing
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Where do psoriatic lesions generally occur?
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Elbows, knees, hairline and pre-sacral region
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What is the name of the condition that is a rare form of psoriasis and is complicated by hypoalbuminemia, sepsis and renal failure?
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Generalized pustular psoriasis
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How is psoriasis treated?
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Goal is to slow the rapid proliferation of epidermal cells Coal tar + UV phototherapy inhibits enzymes, can irritate normal skin Topical corticosteroids- don't stop pre-op
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What are the anesthesia considerations for psoriasis?
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Evaluate drugs being used to manage psoriasis- steroids and chemotherapeutic drugs Skin trauma from IV/incision can increase psoriasis Altered thermoregulation- use an esophageal temp probe
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What is mastocytosis?
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Rare disorder of mast cell proliferation; degranulation of mast cells Urticaria pigmentosa-benign/asymptomatic- occurs in children but resolves by adulthood
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What is systemic mastocytosis?
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Mast cells proliferate in all organs- especially bone, liver, and spleen. Does not occur to CNS cells
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What are the s/s of mastocytosis?
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Histamine release from mast cells and prostaglandins- anaphylactoid responses with pruritus, urticaria, and flushing with hypotension, tachycardia Low bleeding/bronchospasm risk
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What is the anesthetic management of mastocytosis?
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Influenced by possibility of intra-op mast cell degranulation and anaphylactoid reaction- have epi readily available Contrast dyes- profound hypotension- pretreat with H1-H2 blockers and glucocorticoids Oral cromolyn pre-op
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What medications are safe to use in mastocytosis?
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V.A., fentanyl, propofol, vecuronium
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What medications should be avoided in patients with mastocytosis?
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Succs and meperidine- may cause mast cell degranulation
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What are characteristics of acute urticaria?
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Hives and angioedema involving mucous membranes- mouth/pharynx/larynx
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What is the treatment for acute urticaria?
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Anti-histamines
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What are characteristics of chronic urticaria?
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Circumscribed wheals, intensely pruritic
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What agents should be avoided in chronic urticaria?
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ACE inhibitors and NSAIDs
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What are triggers of cold urticaria?
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cold air currents, rain, aquatic activities, snow, consuming cold food/beverages, contact with cold objects
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What are clinical consequences of cold urticaria?
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Laryngeal edema, bronchospasm, hypotension
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What are anesthesia considerations for patients with urticaria?
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Avoid drugs that evoke histamine release, warm IV fluids/meds that are stored in fridge, increase OR temperature, pre-op H1 and H2 antagonists + corticosteroids recommended
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What is scleroderma?
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systemic sclerosis with inflammation, vascular sclerosis, and fibrosis of skin and viscera Causes tissue edema, lymphatic obstruction and ultimately fibrosis
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What are the components of CREST syndrome?
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Calcinosis- calcium deposits in the skin Raynaud's phenomenon- spasm of blood vessels with cold or stress Esophageal dysfunction-acid reflux and decreased esophageal motility Sclerodactyly- thickening/tightening of skin on fingers/hands Telangiectasias- dilation of capillaries causing red marks on skin surface
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What are the s/s of scleroderma in the skin?
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Mild thickening, non-pitting edema, skin becomes taut
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What are the s/s of scleroderma in the MSK system?
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Myopathy- weakness mostly in proximal skeletal muscle groups
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What are the s/s of scleroderma in the nervous system?
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Nerve compression leading to peripheral or cranial nerve neuropathy Keratoconjunctivitis sicca AKA dry eyes- increased risk of corneal abrasions
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What are the s/s of scleroderma in the cardiac system?
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Sclerosis of small coronary arteries and the conduction system, replacement of cardiac muscle with fibrous tissue leading to cardiac dysrhythmias, cardiac conduction abnormalities and CHF
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What are the s/s of scleroderma in the pulmonary system?
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Interstitial pulmonary fibrosis leading to pulmonary HTN and decreased diffusion capacity as well as arterial hypoxemia
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What are the s/s of scleroderma in the renal system?
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Renal artery stenosis d/t arteriolar intimal proliferation
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What are the s/s of scleroderma in the GI tract?
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Dryness of oral mucosa, progressive fibrosis of GI tract. Hypomotility of lower esophagus and small intestine. Dysphagia is a common complaint
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What are the anesthetic considerations for patients with scleroderma?
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Fiberoptic intubation d/t difficult airway Difficult IV/Aline access d/t dermal thickening Fluid replacement for hypotension RSI + OGT d/t GI involvement Ventilation with higher airway pressures Temperature management- OR temp/IV fluids
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What 2 things are patients with scleroderma sensitive to?
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Respiratory depressants- opioids Drugs that are excreted renally
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What is pseudoxanthoma elasticum?
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Rare hereditary disorder of elastic tissue
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How is pseudoxanthoma elasticum diagnosed?
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Angioid retinal streaks
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What are the s/s of pseudoxanthoma elasticum?
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Yellowish, rectangular, elevated xanthoma-like lesions on skin- esp neck, axilla and inguinal regions
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What are the anesthesia considerations for pseudoxanthoma elasticum?
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CV derangements- EKG + NIBP Caution with OGT or esophageal stethoscope No specific recommendations for anesthetic drugs or techniques
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What is the cause of Ehlers-Danlos syndrome?
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Abnormal production of procollagen and collagen
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What are the s/s of Ehlers-Danlos syndrome?
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MSK- joint hypermobility, MSK discomfort, susceptibility to osteoarthritis Skin- fragile or hyperelastic- bruising/scarring Increased risk of spontaneous rupture of bowel, uterus, vasculature OB-premature labor/excessive bleeding Respiratory- dilated trachea, increased risk of pneumothorax
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What are the anesthesia considerations with Ehlers-Danlos?
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Bleeding- avoid IM injections, excessive instrumentation of nose/esophagus, avoid Aline/MML, no regional anesthesia Respiratory- keep low airway pressures d/t risk of pneumothorax
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What is Marfan's syndrome?
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Inherited autosomal dominant trait with long-tubular bones
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What are the skeletal abnormalities with Marfan's?
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High-arched palate, pectus excavatum, kyphoscoliosis and hyperextensibility of the joints
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What are the 2 respiratory complications of Marfan's?
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Early development of emphysema High incidence of spontaneous pneumothorax
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What are the ocular complications of Marfan's?
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Lens dislocation, myopia and retinal detachment -Occur in more than half of patients
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What is the cause for nearly all premature deaths in Marfan's patients?
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CV abnormalities Defective connective tissue in aorta/heart valves lead to aortic dilation, dissection or rupture and prolapse of valves-esp mitral valve Increased risk of bacterial endocarditis Cardiac conduction abnormalities-BBB
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What are the anesthesia considerations of Marfan's syndrome?
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Focus on CV abnormalities Avoid temporomandibular joint dislocation Avoid sustained increase in BP d/t increased risk of aortic dissection Invasive monitoring-including TEE in some patients High risk of pneumo
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What are the two types of myositis?
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Polymyositis- multisystem inflammatory myopathies Dermatomyositis- skin changes + muscle weakness
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What are the s/s of polymyositis and dermatomyositis?
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Skeletal muscle weakness d/t skeletal muscle destruction. Increased serum creatine kinase levels GI- dysphagia, pulmonary aspiration and pneumonia Resp- diaphragm/intercostal muscle weakness causing insufficient ventilation
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Is the neuromuscular junction affected in polymyositis and dermatomyositis?
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No
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What are the treatments for polymyositis and dermatomyositis?
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Corticosteroids and immunosuppressive tx IVIG
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What are the anesthesia considerations for polymyositis and dermatomyositis?
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Risk for pulmonary aspiration, responses to both NDNMBA and DNMBA normal
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What is muscular dystrophy?
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Group of hereditary diseases characterized by painless degeneration and atrophy of skeletal muscles. Progressive, symmetrical, skeletal muscle weakness/wasting but no denervation. Sensation/reflexes remain intact
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What are the initial symptoms of Duchenne's muscular dystrophy?
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Waddling gait, frequent falling, difficulty climbing stairs. Mental retardation is often present. Serum creatine kinase concentrations are 20-100x normal
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What does the EKG show in a patient with Duchenne's Muscular dystrophy?
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Tall R waves in V1 Deep Q waves in the limb leads Short PR interval and sinus tachycardia
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What are the anesthesia considerations for Duchenne's muscular dystrophy?
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Decreased cardiopulmonary reserve GI hypomotility + weak laryngeal reflexes = high aspiration risk Succs- contraindicated d/t hyperkalemia, rhabdomyolysis and cardiac arrest. NDNMB response is normal Rhabdo can be seen if VA are used with no succs Dantrole available- increased incidence of MH RA avoids risks of GA Delayed pulmonary insufficiency- up to 36 hours post-op
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Details about Limb-Girdle MD
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Slowly progressive but benign Onset in 2nd to 5th decade Shoulder or hip girdle muscles are involved
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Details about fascio scapulo humeral MD
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Slowly progressive, onset in adolescence Facial, pectoral and shoulder girdle muscle wasting
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What are the anesthesia considerations for Limb-Girdle and Fascio-Scapulo humeral MD?
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No CV involvement, long life is likely Use atracurium d/t faster metabolism
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What are signs and symptoms of Nemaline Rod muscular dystrophy?
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Slowly progressive symmetrical dystrophy of skeletal and smooth muscles -Delayed motor development, muscle weakness; hypotonia, abnormal gait and loss of DTRs -Micronagthia, dental malocclusions -Kyphoscoliosis and pectus excavatum Dilated cardiomyopathy leading to cardiac failure Normal mentation
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What are the anesthetic considerations for nemaline rod muscular dystrophy?
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Difficult tracheal intubation d/t micronathia and high arched palate; Awake FOI may be needed -respiratory depressant effects of drugs may be exaggerated d/t respiratory muscle weakness and chest wall abnormalities -V/Q mismatch increased; ventilatory response to CO2 may be blunted Response to Succs and NDNMB unpredictable No MH reported Regional anesthesia high motor block could = respiratory compromise Lumbar lordosis/kyphoscoliosis increases neuraxial technical difficulty and positioning concerns
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What is myotonic dystrophy?
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Persistent contracture (myotonia) after voluntary contraction of a muscle or following electrical stimulation -Peripheral nerves and NMJ not affected -intracellular ATP fails to return calcium to the sarcoplasmic reticulum-unsequestered calcium available to produced sustained muscle contraction -Onset in 2nd or 3rd decade; death at 6th decade d/t PNA or CHF
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What are the s/s of myotonic dystrophy?
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Facial weakness- expressionless face Wasting/weakness of sternocleidomastoid muscles, ptosis, dysarthria, dysphagia, inability to relax hand grip Triad of mental retardation, frontal baldness and cataracts Endocrine gland- gonadal atrophy, DM, hypothyroidism, adrenal insufficiency Delayed gastric emptying, intestinal pseudoobstruction Sleep apnea > hypersomnolence Increased incidence of cholelithiasis, especially in men Pregnancy exacerbates symptoms 1st degree AV block- often present before clinical onset of disease- MVP in up to 20% of patients Sudden death may be r/t complete heart block
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What is the treatment of myotonic dystrophy?
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Phenytoin, quinine, or procainimide- to depress sodium influx into skeletal muscle and delay return of membrane excitability -LA infiltration may induce relaxation, and quinine 300-600mg IV may be effective
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What are the anesthesia considerations for myotonic dystrophy?
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GA, regional and NMB do not prevent or relieve contraction -Increased OR temp may decrease myotonia severity Consider cardiomyopathy Succs will have prolonged skeletal muscle contraction, but the response to NDNMB response is normal Sensitive to the respiratory depressant effects of barbiturates, opioids, benzos and propofol Myotonic contraction during surgical manipulation and/or use of electrocautery may interfere with surgical access High concentrations of VA may decrease contractions but will also cause myocardial depression
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What is periodic paralysis?
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Intermittent acute attacks to skeletal muscle leading to weakness or paralysis
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What is the cause of periodic paralysis?
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Hypo or Hyperkalemia
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What are the anesthesia considerations for periodic paralysis?
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Acetazolamide, mannitol, frequent peri-op K monitoring, administer glucose containing solutions, avoid beta agonists, avoid potassium solutions, avoid anectine
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What is myasthenia gravis?
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Chronic autoimmune disorder caused by a decrease in functional acetylcholine receptors at the NMJ resulting from their destruction or inactivation by circulating antibodies -Down regulation of AcH receptors - Thymic hyperplasia is present in 2/3 of patients with MG and 10-15% of these patients have thymomas
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What are the s/s of MG?
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Hallmark- weakness and rapid exhaustion of voluntary muscles with repetitive use, partial recovery with rest; muscle strength is normal when well rested. Initial symptoms- ptosis, diplopia, and dysphagia High aspiration risk-weak pharyngeal, laryngeal muscles = dysphagia, dysarthria and difficulty handling saliva Myocarditis can result in Afib, heart block or cardiomyopathy Other autoimmune diseases- hyperthyroidism, RA, SLE and pernicious anemia Infection, electrolyte abnormalities, pregnancy, emotional stress, and surgery may precipitate or exacerbate muscle weakness ABX-especially aminoglycosides can aggravate muscle weakness
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What is the treatment for MG?
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Pyridostigmine, thymectomy, immunosuppressive tx, plasmapheresis
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What are the anesthesia considerations for MG?
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Increased sensitivity to NDNMB- decrease dose by half to two-thirds -resistance to Succs, need 2.6x ED95 -VA have musculoskeletal relaxation properties -postpone extubation- ventilator support post-op
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What is the other name for myasthenic syndrome?
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AKA Eaton-Lambert syndrome
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What is Eaton-Lambert syndrome?
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Resembles MG, IgG antibodies attack calcium channels
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What is the treatment for myasthenic syndrome?
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- NOT pyridostigmine -3,4 Diaminopyridine -IgG for 6-8 weeks
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What are the manifestations of myasthenic syndromes?
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Proximal limb weakness, Legs > arms, exercise improves strength, muscle pain is common, reflexes absent or decreased
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What is the response to muscle relaxant agents in Eaton-Lambert syndrome?
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Sensitive to both NDNMB and succs.
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What are the anesthesia considerations for myasthenia syndrome?
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Sensitive to depolarizing and NDNMB Antagonism of NMB with anticholinesterase agents may be inadequate -Potential presence of myasthenic syndrome and need to decrease doses of muscle relaxants should be considered in patients undergoing bronchoscopy, mediastinoscopy, or thoracoscopy for suspected lung cancer
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Details about osteoarthritis
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Most common joint disease in US Leading chronic disease of elderly, major cause of disability Degenerative process that affects articular cartilage Pain with motion, relieved with rest Knees and hips are most common sites Pain relieved by heat, APAP and anti-inflammatory drugs
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What is rheumatoid arthritis?
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Most common chronic inflammatory arthritis
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What is the etiology of RA?
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Unknown- but is symmetrical polyarthropathy and significan systemic involvement. Proximal interphalangeal and metacarpophalangeal joints of the hands and feet
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What is the hallmark sign of RA?
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Morning stiffness
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What are the common coexisting sequella with RA?
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Cervical spine involvement- atlanto-axial subluxation, TMJ limitation Cricoarytenoid arthritis Neuropathy Visceral ischemia- bowel perforation, MI and CVA possible Pleural effusion Rheumatoid nodules are typically present at pressure points, particularly below the elbows
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What is the treatment for RA?
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Relieve pain, preserve joint function and strength, prevent deformities and attenuate systemic complications. NSAIDS/corticosteriods
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What is DMARD?
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Disease modifying antirheumatic drug- group of drugs that have the potential to modify, slow or halt the course of RA. Examples- methotrexate given 1x/week, sulfasalazine, anti-malarials
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What are the anesthesia considerations for RA?
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Consider multiorgan involvement and drug SE pre-op evaluation for airway involvement, compromise of airway may occur at the C spine, TMJ and cricoarytenoid joints -Atlantoaxial subluxation may be present- care must be taken to minimize movement of head/neck during DL to avoid further displacement of the odontoid process and damage to the spinal cord -Involvement of the cricoarytenoid joints by arthritic changes is suggested by the pre-op presence of hoarseness or stridor and/or observation of erythema/edema of vocal cords during DL
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What is kyphoscoliosis?
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Spinal deformity- anterior flexion and lateral curvature of vertebral column -idiopathic in 80% of cases, begins late in childhood, progresses in severity during rapid skeletal growth
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What are neuromuscular diseases that may be associated with kyphoscoliosis?
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Polymyelitis, cerebral palsy, muscular dystrophy
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What are s/s of kyphoscoliosis?
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Severe-spinal curvature >40 degrees, physiologic derangements in cardiac and pulmonary function such as decreased vital capacity, dyspnea on exertion, increased WOB and increased airway resistance. -Principle cause of death is restrictive lung disease and pulmonary HTN progressing to cor pulmonale -Pulmonary HTN d/t increased PVR r/t compression of lung vasculature and response to arterial hypoxemia -Poor cough contributes to frequent pulmonary infection
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What are the pre-op anesthesia managements for kyphoscoliosis?
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PFT and ABG- check for pulmonary infection from chronic aspiration Correct infection or bronchospasm before elective surgery
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What are the intra-op anesthetic considerations for kyphoscoliosis?
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N2O may increase PVR -surgery to correct the spinal curvature- consider potential for blood loss and risk of surgically induced spinal cord damage - caution with controlled hypotension to decrease blood loss- can increase risk of ischemic optic neuropathy and spinal cord ischemia
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What is SLE?
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Multisystem chronic inflammatory disease characterized by antinuclear antibody production -exacerbated by infection, pregnancy, surgery -Onset can be drug induced
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What are the drugs implicated in causing SLE?
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Procainamide, hydralazine, isoniazide, D-penicillamine and alpha-methyldopa
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What are s/s of SLE?
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Pleural effusions, heart problems, lupus nephritis, arthritis, Raynaud's, Butterfly rash
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What is the treatment of SLE?
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Corticosteroids, immunosuppressive tx, bone marrow transplant
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What is the anesthesia management with SLE?
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Laryngeal involvement, including mucosal ulceration, cricoarytenoid arthritis and RLN palsy may be present in up to 1/3 of patients
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What is achondroplasia dwarfism?
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Disproportionate dwarfism, kyphoscoliosis common. Premature fusion of the bones at the base of the skull resulting in shortened skull base and stenotic foramen magnum. May be functional fusion of the antlanto-occipital joint with odontoid hypoplasia, antlantoaxial instability, bulging discs and severe cervical kyphosis.
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What is the most common CV problem r/t achondroplasia dwarfism?
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Pulmonary HTN causing cor pulmonale
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What is the anesthetic management for a patient with achondroplasia dwarfism?
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Potential airway difficulties, cervical spine instability and potential for spinal cord trauma with neck extension. -facial features- large protruding forehead, short maxilla, large mandible, flat nose and large tongue = difficult obtaining a good mask fit and maintaining a patent upper airway FOI may be needed -small epidural space may cause difficulty passing epidural catheter. Osteophytes, prolapsed intervertebral discs or deformed vertebral bodies may contribute to difficulty with neuraxial blockade -No data of appropriate LA doses -Epidural anesthesia is > spinal- permits titration of LA to achieve desired sensory blockade level
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What is the anesthesia management for rotator cuff repair?
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Brachial plexus block via interscalene approach -Ipsilateral hemidiaphragmatic paralysis always occurs with interscale r/t phrenic nerve proximity. Avoid in patients with COPD or NM diseases r/t weakness of respiratory muscles -Wound infiltration or lavage with solutions containing long-acting local anesthetics such as bupivicaine or ropivicaine can provide post-op analgesia after major surgery
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What is tracheaomegaly?
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Marked dilation of the trachea and bronchi d/t congenital defect in elastin and smooth muscle fibers in the tracheobronchial tree or d/t destruction after radiotherapy
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How is tracheomegaly diagnosed?
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CXR with tracheal diameter >30mm
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What are symptoms of tracheomegaly?
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Chronic productive cough and frequent pulmonary infection- can be r/t chronic aspiration -tracheal and bronchial walls are abnormally flaccid and may collapse during vigorous coughing
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What is an anesthetic concern for tracheomegaly?
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Increased aspiration risk during GA if maximal inflation of the ETT cuff doesn't produce a seal
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What is Prader-Willi syndrome?
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Manifests at birth as hypotonia, associated with weak cough, swallowing difficulties and upper airway obstruction. -Obesity + endocrine abnormalities including hypogonadism and DM -Micrognathia, a high-arched palate, strabismus, a straight ulnar border and congenital dislocation of the hip may be present. -Dental caries d/t chronic regurgitation of gastric contents -Seizures are r/t to this syndrome but cardiac dysfunction does not accompany this syndrome
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What are anesthetic considerations for Prader-Willi?
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Weak skeletal musculature = poor cough and increased risk of PNA -intra-op monitoring of BG necessary- exogenous glucose adminstration may be needed d/t patient's body using circulating glucose to manufacture fat rather than meet basal energy needs -Decreased NMB needs d/t decreased muscle mass -No MH link established
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What is macroglossia?
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Infrequent but potentially lethal post-op complication that is most often r/t posterio fossa craniotomy performed in the sitting position
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What are possible causes of macroglossia?
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Arterial compression, venous compression from excessive neck flexion or head down position and mechanical compression of the tongue by the teeth, oral airway or ETT -may also have a neurogenic origin
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What is the risk r/t macroglossia?
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May be delayed for 30min or longer. Then there is a risk of complete airway obstruction occurring at an unexpected time in the post-op course
