Skin Disorders

Question 1

Bullae

Answer 1

large blisters on skin filled with clear fluid

Question 2

Hemorrhage from ruptured oral bullae has been treated successfully by what

Answer 2

epi soaked gauze directly to bullae

Question 3

can you use tape with bullae

Answer 3

no

Question 4

scleroderma

Answer 4

inflammation and autoimmunity, vascular injury with eventual vascular obliteration, and fibrosis accumulation of XS matrix in many organs and tissues

Tissue fibrosis and organ sclerosis, widespread capillary loss and vascular obliteration and leakage of serum proteins into the interstitial space

Often see CREST syndrome

Question 5

onset of scleroderma

Answer 5

20-40yrs (women)

pregnancy accelerates about half of patients

Question 6

CREST syndrome

Answer 6

Calcinosis 
Raynauds 
Esophageal dysfunction 
sclerodactyly 
telangiectasia

Question 7

calcinosis

Answer 7

painful lumps of calcium in the skin

fingers, body

Question 8

raynauds phenomenon

Answer 8

white or cold skin on the hands and feet when you’re cold or stressed. caused by BF problems.
Absence of BF due to vasoconstriction

Question 9

esophageal dysfunction

Answer 9

problems swallowing/reflux

caused by scarring in the esophagus

Question 10

sclerodactyly

Answer 10

tightness and thickening of finger or toe skin. can be hard to bend

Question 11

telangiectasia

Answer 11

red spots on hands, palms, forearms, face, and lips. caused by widened blood vessels

Question 12

signs and symptoms of scleroderma

CV, renal, GI

Answer 12

predisposed to corneal abrasions lube eyes

Replace cardiac muscle with fibrous tissue = dysrhythmias, conduction abnormalities, CHF
-Pulm HTN –> cor pulmonale
Arterial hypoxemia

renal artery stenosis and decreased RBF

fibrosis GI tract - hypomotility of lower esophagus and SI

dysphagia = LES tone decreased, increased reflux

malabsorption syndrome/coagulation disorders

Question 13

10-15% of people who develop a scleroderma renal crisis can be treated effectively with what?

Answer 13

ACEi

Question 14

xerostomia

Answer 14

dryness oral mucosa

seen in scleroderma

Question 15

what is good with treating malabsorption syndrome/vitK

Answer 15

broad spectrum ABx

Question 16

scleroderma anesthesia management

Answer 16

fiberoptic intubation
no NPA or OPA (nasal and oral mucosa bleed easy)
IV access impaired by dermal thickening
avoid increase in PVR (respiratory acidosis, arterial hypoxemia)
sensitive to respiratory depressant opioids

Question 17

Marfan syndrome

Answer 17

long, tubular bones (Abe) 
high-arched palate
emphysema 
spont pneumo 
aortic dilation, dissection or rupture and prolapse the cardiac valves, esp mitral 
pregnancy triggers dissection 
BBB = common

Question 18

What is common in Marfan syndrome and what is a prophylactic therapy

Answer 18

BBB and prophylactic BB therapy

Question 19

anesthesia considerations marfan syndrome

Answer 19

focus on cardiopulmonary
abnormalities

AVOID any sustained increase in BP (risk aortic dissection). this includes DL or painful surgical stim

invasive monitoring good option (ex TEE)

Question 20

Ehlers Danlos Syndrome

Answer 20

inherited CT disorder caused by abnormal production of pro collagen and collagen

joint hypermobility, skin fragility, hyper elasticity, bruising and scarring, musculoskeletal discomfort, osteoarthritis

Question 21

What is the form of Ehlers Danlos Syndrome associated with an increased risk of death

Answer 21

vascular type

-complicated by rupture of large blood vessels, the bowel, and uterus

Question 22

Common issues with Ehlers Danlos

Answer 22

Dilation of trachea and higher incidence of pnemo. Use a bigger ETT and keep cuff pressures down to minimize bleeding

Question 23

Management Anesthesia Ehlers Danlos

Answer 23

avoid IM 
avoid anything in nose 
gentle during DL 
a-line and c-line can cause hematoma 
maintain low airway pressure during assisted or controlled mechanical ventilation otherwise pneumothorax 
don't do regional

Question 24

Pseudohypertrophic Muscular Dystrophy (Duchenne Muscular Dystrophy)

Answer 24

muscles more weak and flexible over time
stiff joints

DMD most common type

almost always affects boys and Sx begin in childhood

also degenerates cardiac muscle

Question 25

ECG of Duchenne Muscular Dystrophy

Answer 25

tall R waves in V1 Deep Q waves in limb leads Short PR sinus tachycardia

Question 26

pseudotrophy

Answer 26

muscle tissue eventually replaced by fat and CT lose ability to walk by 12

Question 27

Management anesthesia for DMD

Answer 27

always full stomach use of Six is contraindicated because risk hyperK, rhabdomyolysis, CA normal response to NDMBs rhabdomyo has been noted with volatile agents even without use of sux dantrolene should be available (increased MH)

Question 28

Duchenne think what?

Answer 28

Dantrolene!

Question 29

management anesthesia if you hear "myopathy"

Answer 29

``` micrognathia and a high arched palate = difficult intubation resp muscle weakness v/q mismatch much higher CO2 to trigger response unpredictable response to sux and NDMBs myocardial depression ```

Question 30

Kearns Sayre syndrome mitochondrial myopathy

Answer 30

mitochondrial myopathy skeletal muscle energy metabolism disorder abnormal fatigue •Progressive external ophthalmoplegia, retinitis pigmentosa, heart block, hearing loss, short stature, peripheral neuropathy, and impaired ventilatory drive •Dilated cardiomyopathy and congestive heart failure may be present.

Question 31

Kearns Sayre syndrome anesthesia management

Answer 31

drug induced myocardial depression risk development of cardiac conduction defects hypoventilation

Question 32

alcoholic myopathy floppy infant syndrome anesthesia

Answer 32

increased sensitivity to NDMBs Sux: hyperK and CA ketamine can be useful

Question 33

myopathy, think of what?

Answer 33

MR and K

Question 34

what is the most common NMJ disease

Answer 34

Myasthenia Gravis

Question 35

myasthenia gravis

Answer 35

decrease in final ACH receptors at NMJ resulting from destruction or inactivation by circulating antibodies can loose up to 80% of functional receptors women 20-30 most affected eye deviation and drooping eyelid

Question 36

anesthesia for MG

Answer 36

sensitive to ND MR use small doses of Roc start with 1/5 the dose (decreased number of functional ACH-R) patients treated with pyridostigmine demonstrate resistance to sux induce with short acting agent don't need MR to intubate usually - weak oMonitoring these responses at the orbicularis oculi muscle may over-estimate the degree of neuromuscular blockade but may help to avoid unrecognized persistent neuromuscular blockade in these patients

Question 37

classifications of MG (5 types)

Answer 37

``` T1 = extraocular muscles T2a= slowly progressive, mild sk. m. weakness that spares respiration muscles T2b = rapidly progressive, more severe form and may involve respiratory muscles T3 = acute onset and rapid deterioration of sk m strength within 6mo T4 = severe form that results from progression of T1 or T2 MG ```

Question 38

if a patient has thymomas, what is likely

Answer 38

MG | seen in 10% of MG pts

Question 39

what drug causes a resistance to sux

Answer 39

pyridostigmine

Question 40

what muscle may overestimate the degree of NM blockade

Answer 40

orbicularis oculi

Question 41

myasthenia syndrome

Answer 41

from a carcinoma in the lung skeletal muscle weakness with a small cell carcinoma in the lung •IV immunoglobulin, plasmapheresis, immunosuppressive therapy, and treatment of the underlying cancer can all cause improvement in this condition. •Eaton-Lambert syndrome resembles myasthenia gravis

Question 42

myasthenia syndrome anesthesia

Answer 42

sensitive to NDMB and SUX | antagonism of NMB with anti cholinesterase may not work

Question 43

osteoarthritis

Answer 43

most common joint disease degenerative process that affects articular cartilage knees and hips common

Question 44

kyphoscoliosis

Answer 44

spinal deformity anterior flexion (kyphosis) and lateral curvature (scoliosis) of vertebral column ANT and LAT begins in late childhood females 4x more

Question 45

kyphoscoliosis management anesthesia

Answer 45

ABG, PFT assess restrictive lung disease ABG: hypoxemia? acidosis? pulm HTN often have pulmonary infon from chronic aspiration •Nitrous oxide may increase pulmonary vascular resistance leading to pulmonary hypertension

Question 46

Rheumatoid arthritis

Answer 46

most common inflammatory arthritis 1% of adults •Diagnosis is primarily clinical and notable for morning stiffness and metacarpophalangeal involvement (unlike OA which affects the distal interphalangeals) •Nearly every joint is affected except for the thoracic and lumbosacral spine. •Cervical spine involvement is frequent and can result in pain and neurologic complications oRange of neck motion should always be evaluated preoperatively by asking the patient to demonstrate head movement or positioning. NARROWED GLOTTIS

Question 47

what drugs are RA patients often on

Answer 47

NSAIDs and or glucocorticoids oThe effect of aspirin or NSAIDs on platelet function must be considered. Corticosteroid supplementation may be indicated in patients being treated long term with these drugs.

Question 48

Anesthesia for RA

Answer 48

airway - look for atlantoaxial subluxation •When atlantoaxial subluxation is present, care must be taken to minimize movement of the head and neck during direct laryngoscopy to avoid further displacement of the odontoid process and damage to the brainstem or spinal cord oMinimal movement of neck!! Narrowed glottis fiberoptic intubation

Question 49

why is atlantoaxial subluxation bad and important to look for with RA

Answer 49

This abnormality is important because the displaced odontoid process can compress the cervical spinal cord or medulla or occlude the vertebral arteries

Question 50

systemic lupus erythematosis anesthesia

Answer 50

``` •Assess magnitude of organ system dysfunction and the drugs used to treat SLE oSteroids oNSAIDS oASA oAntimalarial drugs •Laryngeal involvement omucosal ulceration ocricoarytenoid arthritis orecurrent laryngeal nerve palsy omay be present in 1/3 of patients ```

Question 51

ankylosing spondylitis considerations

Answer 51

stiff neck - Spinal column can be stiff and deformed and prevent appropriate cervical spine motion for endotracheal intubation •Aortic regurgitation - keep the heart rate at 90 beats per minute or higher and the systemic vascular resistance lower than normal o Sweeping water off porch = fast helps = >90bpm HR

Question 52

What does the presence of HLA-B27 mean

Answer 52

genetic marker that have inflammatory arthritis of spine and joints (not osteoarthritis) associated with seronegative spondyloarthropathies

Question 53

seronegative spondyloarthropathies

Answer 53

+ HLAB27 ankylosing spondylitis (AS), psoriatic arthritis, and Reiter's syndrome (also called reactive arthritis) •HLA-B27 is present in about 90% of people with AS, but the gene can also be seen in people with no sign of arthritis or inflammation.

Question 54

dwarfism 2 forms

Answer 54

proportionate | disproportionate (limited bone development)