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S5 - Advanced Patho > Pheochromocytoma, Splenic, Carcinoid, Blah Blah > Flashcards

Pheochromocytoma, Splenic, Carcinoid, Blah Blah Flashcards

1
Q

What is a pheochromocytoma?

A

Cathecholamine secreting tumor from the chromaffin cells of the adrenal system

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2
Q

What do chromaffin cells produce?

A

Epinephrine and norepinephrine

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3
Q

Can pheochromocytoma be cured?

A

HTN can be cured by resection

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4
Q

Pheo patient demographics

A
  • Equal male and female
  • Usually 30-50 years old
  • 10% in children
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5
Q

What is familial pheo associated with?

A
  • Can be a part of Multiple Endocrine Neoplastic (MEN) syndrome
  • Almost 100% of MEN II pts have or will have a pheo
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6
Q

In what part of the adrenal gland are most pheos found?

A

80% in the adrenal medulla

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7
Q

Are pheos malignant or benign?

A

Can be either

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8
Q

What do pheos secrete?

A
  • Can secrete norepinephrine and/or epinephrine
  • Usually secrete norepi 85% and epi 15% (normal ratio reversed)
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9
Q

How does neurogenic control of pheos occur?

A

It usually doesn’t

Pheos secrete catecholamines without neurogenic control

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10
Q

Hallmark symptom of pheo

A

Hypertension

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11
Q

How often do patients have symptoms with a pheo?

A

Infrequently to several times daily (very unpredictable)

Usually last a few minutes to several hours

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12
Q

S/S of pheo depend on what they are secreting the most

What are the signs of norepinephrine secretion?

A
  • Increased SBP
  • Increased DBP
  • Reflex bradycardia
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13
Q

S/S of pheo depend on what they are secreting the most

What are the signs of epinephrine secretion?

A
  • Increased SBP
  • Decreased DBP
  • Tachycardia
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14
Q

Effects of pheo on SVR, CO and plasma volume

A
  • Increased SVR
  • Normal CO
  • Slightly decreased plasma volume
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15
Q

EKG changes with pheo

A
  • ST changes (increased or decreased)
  • Flat/inverted T waves
  • Prolonged QT
  • Peaked P waves
  • L axis deviation
  • Dysrhythmias
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16
Q

Cardiomyopathy is common with pheo..

Which chamber usually?

A

LV

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17
Q

blood glucose in pheo patients (prior to resection)

A

Increased due to inhibited insulin release

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18
Q

How do you diagnose a pheo?

What is most sensitive?

A

24 hours metanephrines and catecholamines

Most sensitive is plasma free metanephrines

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19
Q

What are the two plasma free metanephrines?

A

Metanephrine and normetanephrine

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20
Q

What is normetanephrine?

What is considered elevated?

A

Norepinephrine metabolite

>400 pg/mL

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21
Q

What is metanephrine?

What is considered an elevated level?

A

Epinephrine metabolite

>220 pg/mL

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22
Q

test to perform if plasma free metanephrines questionable?

A

clonidine suppression test or glucagon stimulation test (if DBP <100)

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23
Q

imaging for a pheo

A
  • CT
  • MRI
  • PET scan
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24
Q

What pheo test may be done in the cath lab?

A

Taking catecholamine samples from adrenal vein

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25
Q

Differential diagnoses of pheo

A
  • MH
  • Thyroid storm
  • Carcinoid crisis
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26
Q

Where are catecholamines from pheos metabolized?

What is the significance?

A

Metabolized within chromaffin cells

plasma free metanephrines are more accurate for diagnosing pheo than plasma epi and norepi levels

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27
Q

T/F - mosty pheos secrete more epi than norepi

A

false - most pheos secrete norepi more than epi

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28
Q

Effects of alpha blockade treatment for pheo

A
  • Decrease BP
  • Increase volume
  • Prevent HTN episodes
  • Re-sensitize receptors
  • Decrease myocardial dysfunction
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29
Q

Most common alpha blocker for pheo

MOA?

A

Phenoxybenzamine (Dibenzyline)

MOA: non competitive, non-selective irreversible a-blocker

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30
Q

adverse effect of alpha blocker overtreatment

How to avoid in pheo pts?

A

Orthostatic hypotension

D/C 24-48 hours pre-op to avoid refractory intra-op hypotension

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31
Q

Two shorter acting pure alpha-1 blockers

Benefits vs. phenoxybenzamine

A
  • Prazosin (Minipress) and doxazosin (cardura)
  • Benefits: less tachycardia, easier to titrate
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32
Q

How do you treat phenoxybenzamine induced tachycardia?

A

With non-selective B-blockade

  • Usually Propanolol
  • Also atenonol, metoprolol, labetalol
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33
Q

When treating pheo, do you want to block alpha or beta first?

A

Alpha

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34
Q

Why would you want to use EXTREME CAUTION with beta-blockade prior to alpha-blockade?

A

Beta blockade–> unopposed alpha effects –> vasoconstriction and HTN crisis

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35
Q

adjunct treatments for pre-op pheo

A
  • Metyrosine
  • Ca++ channel blockers
  • ACE inhibitors
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36
Q

How does metyrosine treat a pheo?

A

Metyrosine is a tyrosine inhibitor so it blocks catecholamine synthesis

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37
Q

2 primary intra-op goals for a pheo

A
  • Avoid catecholamine release (drugs, stimulation)
  • Maintain CV stability with short-acting drugs
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38
Q

What occurs with ligation of tumor-related vessels with pheo intraop?

A

Significant hypotension

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39
Q

Monitoring intraop for pheo

A
  • Standard monitoring
  • Arterial line
  • Conisder CVP, PA, and TEE also
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40
Q

T/F You want your patient slightly hypovolemic for pheo

A

FALSE

Avoid hypovolemia

Would cause catecholamine release? maybe

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41
Q

drugs to avoid in a pheo pt

A
  • Morphine
  • Atracurium
  • Atropine
  • Succinylcholine
  • Pancuronium
  • Ephedrine
  • Ketamine
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42
Q

Most common approach for pheo excision

Disadvantages?

A
  • Laparoscopic adrenalectomy
  • Insufflation and tumor manipulation cause HTN
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43
Q

What is required for excision of large pheos?

A

Open excision

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44
Q

Name some times during surgery that we would cause increased catecholamine release with pheo

A
  • Laryngoscopy
  • Incision
  • Light anesthesia
  • Emergence
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45
Q

Is pre-op alpha blockade always effective for preventing hypertension?

A

Nope

SBP >200 is common intra-op regardless of pre-op alpha blockade

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46
Q

Drugs to have prepared for pheo patient?

A

“Have potent, rapid onset drugs prepared and ready”

  • Nipride - drug of choice
  • Phentolamine
  • Nitroglycerine
  • Labetalol
  • Mag sulfate
  • Esmolol
  • Diltiazem
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47
Q

caution with using increased anesthetic depth to treat HTN with pheo

A

Increases risk of hypotension with venous ligation

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48
Q

What drugs should you have available for ventricular dysrhythmias with pheo?

A
  • Lidocaine
  • Beta-blockers (propanolol, esmolol)
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49
Q

How to prevent and treat hypotension associated with venous ligation of pheo

A
  • Prevent: pre-treat with cyrstalloids
  • Treat: pressors and inotropes if needed
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50
Q

T/F Cell saver is a good option for pheo patients to prevent hypotension with venous ligation

A

False

Cell-saver is high in catecholamines

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51
Q

What happens to blood sugar after resection of pheo?

How do you treat?

A
  • Increased insulin and decreased glucose
  • Begin dextrose solution after resection
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52
Q

When does hypertension resolve after pheo resection?

A

Most patients eventually become normotensive

  • Approximately 50% with be hypertensive for several days
  • 25% remain hypertensive for life
  • ?? about the other 25%
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53
Q

How long does it take for plasma catecholamine levels to normalize?

(after pheo resection or what?)

A

7-10 days

This doesnt make sense because she said plasma catecholamine levels are not what you really look at but okkkkk - i think theyre still elevated just not the most accurate for diagnosing?

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54
Q

Most frequent post-op cause of death after pheo resection

How to treat? (if they havent died yet)

A

Hypotension due to sudden decrease in catecholamines with refractory vasodilation

Treat with increased fluids, pressors if necessary

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55
Q

How to treat hypoadrenalism after pheo resection

A

Steroids

56
Q

What may occasionally be first indication of an undiagnosed pheo?

A

Unexpected intra-op hypertension and tachycardia during manipulation of abdominal structures

57
Q

What may be unmasked in volume expansion treatment for pheo?

A

Underlying anemia

58
Q

When is art line particularly useful in pheo excision?

Is CVL always necessary?

A

During induction and manipulaiton

CVL not needed in young patients with minimal or no heart disease

59
Q

T/F An awake intubation is indicated in pheo patient

A

FALSE

Intubation should not be attempted until deep level of GA has been established, possibly even LA of trachea

60
Q

What occurs in the spleen until the 5th gestational month?

A

Hematopoiesis

61
Q

Functions of the spleen

A
  • Blood filtering
  • Erythrocyte morphology
  • Immunity related to blood-borne antigens
62
Q

The spleen is a reservoir for what?

A
  • Red blood cells
  • Platelets
63
Q

What can macrophages engulf that can lead to anemia, splenomegaly, and spenic infarcion?

A
  • Abnormal blood cells
    • Sickle cell
    • Thalasemia
    • Spherocytosis
  • Older blood cells (>120 days)
64
Q

What according to these notes play important roles in immunity?

A
  • Macrophages
  • Histiocytes
  • IgM
65
Q

Reasons elective spleenectomies are done (9)

A
  • Hyperspleenism
  • ITP
  • TTP
  • Hereditary spherocytosis
  • Hereditary hemolytic anemia
  • Hemoglobinopathies
  • Malignancies
  • Abcesses
  • Cysts
66
Q

Why are emergent splenectomies performed?

A
  • Splenic trauma
67
Q

What percent of CO does the spleen recieve?

A

5%

68
Q

Is splenic bleeding usually venous or arterial?

A

Arterial

69
Q

What patients are at high risk of splenic trauma?

A

Blunt abdominal trauma + LUQ pain

Commonly from fractured rib

70
Q

How is splenectomy usually performed?

What should you have available?

A
  • Usually laparoscopically
  • Laparotomy if HD instability
  • Have blood available
71
Q

What are asplenic patients at higher risk for?

A
  • Infectious processes
    • bacteremia
    • pneumonia
    • meningitis
  • Thromboembolism
72
Q

Where do most carcinoid tumors occur?

A
  • Bronchus
  • Jejunum/ileum
  • Colon/rectum
73
Q

What do carcinoid tumors secrete?

A

GI peptides and vasoactive substances that may or may not cause symptoms

74
Q

Large amounts (of carcinoid tumors or GI peptides, unsure) can cause

A

Carcinoid syndrome

75
Q

S/S of carcinoid syndrome

A
  • flushing
  • diarrhea
  • pruritis
  • tearing
  • facial edema
76
Q

what precipitates carcinoid syndrome?

A
  • stress
  • alcohol
  • exercise
  • diet
  • drugs (catecholamines, SRIs)
77
Q

how to treat diarrhea with carcinoid syndrome?

A

serotonin antagonists

78
Q

how to treat flushing and pruritis with carcinoid syndrome

A

H1/H2 blockers

79
Q

What life threatening condition can carcinoid syndrome lead to?

A

Carcinoid crisis

80
Q

S/S of carcinoid crisis

A
  • Flushing
  • Diarrhea
  • Abdominal pain
  • CV instability
81
Q

What drugs can precipitate carcinoid crisis?

A
  • Succinylcholine
  • Benzyisolquinolines (except Cis)
  • Catecholamines
  • Dopamine
  • Isuprel
  • Thiopental
82
Q

only cure for preventing/treating carcinoid syndrome

A

surgical excision of carcinoid tumor

83
Q

What drug do patients with carcinoid tumors need 24-48 hours pre-op and continued through the procedure?

What anesthetic plan do these patients need?

A

S-statin analogues

  • Octreotide
  • lanreotide

GA with art line; can use epidural if treated with pre-op octreotide

84
Q

Pre-op instructions and prophylactic drugs for carcinoid tumors

A
  • Avoid precipitating conditions
  • Diet/supplements
  • 5-HT3 antagonists
  • Somatostatin analogues
  • H1/H2 blockers
85
Q

Why may patient with carcinoid tumor/syndrome have delayed awakening from anesthesia?

A

Increased serotonin levels

86
Q

Characteristics of fibromyalgia

A
  • Widespread musculoskeletal pain
  • Fatigue
  • Sleep, memory and mood issues
87
Q

Current theory of what causes fibromyalgia

A

Repeated painful stimuli ➡️ increased neurotransmitters ➡️ hyperactive response to pain

88
Q

Other issues that frequently occur with fibromyalgia

A
  • Headache
  • TMJ disorders
  • IBS
  • Anxiety
  • Depression
89
Q

Fibromyalgia is more common in which gender?

A

Females

90
Q

Cure for fibromyalgia?

Treatment?

A

No cure. Treat with:

  • Exercise
  • Stress reduction
  • Alternative medicine (acupuncture, massage therapy, yoga)
  • Meds (NSAIDs, anti-depressants, gabapenatnoids)
  • Pregabalin (Lyrica)
91
Q

First FDA approved drug for fibromyalgia

A

Pregabalin (Lyrica)

92
Q

What is sarcoidosis?

A

Interstitial granulomatous disease usually in lungs, lymphatics, bone, liver, CNS

93
Q

What type of lung disease is sarcoidosis?

Characteristics?

A

Chronic, restrictive lung disease

  • Dyspnea
  • Cough
  • Pulmonary HTN
  • Cor pulmonale
94
Q

How is sarcoidosis usually diagnosed?

A

Radiography

Many are asymptomatic at time of diagnosis

95
Q

Common manifestations of sarcoidosis

A
  • Hypercalcemia (due to uncontrolled synthesis of vitamin D3 enzyme by macrophages)
  • Facial nerve paralysis (neurosarcoidosis, parotid gland)
96
Q

How is biopsy done for sarcoidosis?

A

Mediastinoscopy

97
Q

Treament for sarcoidosis

A

corticosteroids for symptoms and hypercalcemia

98
Q

Anesthetic implications for sarcoidosis

A
  • Patients have poor tolerance to apnea due to small FRC
  • Keep PIPs as low as possible to minimize barotrauma
99
Q

What is systemic lupus erythematosus?

A

a multi-system chronic inflammatory condition caused by antibody production

100
Q

which gender is systemic lupus erythematosus more common in?

A

female

101
Q

stressors of lupus exacerbation

A
  • Surgery
  • Pregnancy
  • Infection
  • Certain meds
102
Q

systemic lupus erythematosus can be drug induced

What drugs cause it?

A
  • Procainamide
  • Hydralazine
  • Isoniazid
  • Penicillamine
  • Methyldopa
103
Q

Symptoms of systemic lupus erythematosus

A
  • Arthralgias
  • Maculopapular (butterfly) rash
  • Fever
  • Anemia
  • Leukopenia
  • Systemic: CNS, heart, lungs, kidneys, liver, hematologic, MSK, and skin (?)
104
Q

What two things worsen prognosis with systemic lupus erythematosus?

A

Nephritis and/or hypertension

105
Q

Treatment for systemic lupus erythematosus

A
  • Based on systems affected and symptoms
  • ASA
  • NSAIDs
  • Chemo
  • Steroids
106
Q

Anesthetic implications of systemic lupus erythematosus

A
  • Consider organ dysfunction
  • Arthritic neck
  • Recurrent laryngeal nerve palsy
  • Drugs (?)
107
Q

What is scleroderma (systemic sclerosis)?

A

Disorder of collagen/ autoimmune disease

Causes inflammation, vascular sclerosis, fibrosis of skin and organs

108
Q

What does vascular injury in scleroderma cause?

A

Leaking proteins ➡️ edema ➡️ fibrosis in skin, MSK, CNS, CV, lungs, kidneys, GI tract

109
Q

What is CREST syndrome?

What can cause it?

A
  • Calcinoses
  • Raynaud’s phenomenon
  • Esophageal hypomotility
  • Sclerodactyly
  • Telangiectasis

Scleroderma can become CREST syndrome

110
Q

T/F CREST syndrome has poor prognosis

A

tru

111
Q

CV complications of scleroderma

A
  • conduction abnormalities
  • contractility abnormalities
  • Pulm artery HTN
  • cor pulmonale

Consider eval pre- op

112
Q

Lung considerations for scleroderma patient

A
  • May need increased PIPs
  • Acidosis/hypoxia
  • Increased pulmonary vascular resistance
  • Sensitive to opioids
113
Q

Renal abnormalities with scleroderma

Treatment?

A
  • HTN
  • Irreversible renal failure

Treat with ACE- inhibitors

114
Q

GI abnormalities with scleroderma

A
  • Decreased motility
  • Dysphagia
  • GERD
  • Vitamin K malabsorption and resulting coag problems
115
Q

Treatment for GI issues with scleroderma

A
  • Octreotide
  • Prokinetics are ineffective
116
Q

What is telangiectasis?

(Remember this is the T in CREST syndrome)

A

Dilation of blood vessels in mucosa

Causes oral/nasal bleeding with intubation

117
Q

AIs for skin abnormalities in pts with scleroderma

A
  • Difficult IV access
  • Peripheral vasoconstriction
  • Hypothermia in OR
118
Q

Regional use in scleroderma patients

A
  • Difficult but can be advantageous
    • peripheral vasodilation
    • opioid sparing
119
Q

What is porphyria?

A

A group of inborn errors of metabolism resulting from specific enzyme deficiency in heme synthetic pathway overproduction of porphyrins

120
Q

How is porphyia classified?

A
  • As either hepatic or erythropoietic
  • Either acute or non-acute
121
Q

Pathophysiology of porphyria

(long so sorry)

A
  • Overproduction of po
  • It binds with proteins to make Hgb and CYP-450 isoenzymes
  • Heme production is regulated by aminolevulinic acid (ALA) synthetase
  • ALA is easily induced by drugs using P-450 system
  • In porphyria, increased heme requirements causes ALA stimulation
  • Causes accumulated intermediates (compounds preceding site deficiency)
122
Q

What are the only forms of porphyria relevent to anesthesia management?

A

Acute porphyrias

  • Plumboporphyria
  • Acute intermittent porphyria
  • Hereditary coproporphyria
  • Variegate porphyria

(Vampires Always Have Porphyria)

123
Q

What can happen with anesthesia in a patient with acute porphyria?

A

Certain drugs can cause life-threatening reactions

For example: thiopental

124
Q

What type of genetic condition is porphyria?

A

Inherited, autosomal dominant

125
Q

What demographic is porphyria more frequent in?

A

Women in 30s-40s

Rare after menopause

126
Q

What triggers porphyria?

A

Stress

  • Childbirth
  • Fasting
  • Dehydration
  • Illness
  • Infection
127
Q

Silent or latent porphyria may show first symptoms when?

A

After perioperative trggering drugs

128
Q

What is more high risk for triggering porphyria, single exposure or multiple inducers?

A

Multiple inducers duh

129
Q

Potential drug triggers of porphyria to avoid

A
  • Barbiturates (thiopental, methohexital)
  • Sevo
  • Etomidate
  • Pentazocine (Talwin)
  • Ephedrine
  • Ketamine
  • Traditional seizure meds
130
Q

S/S of acute porphyria exacerbation

A
  • Skeletal muscle weakness
  • HTN
  • Tachycardia
  • Abdominal pain
  • Seizures
  • Electrolyte imbalance (esp. hyponatremia)
131
Q

What common aspect of periop instructions can trigger porphyria?

A

NPO status

Consider glucose IVF

132
Q

How to rule out acute porphyria crisis before surgery

A

Urine porphobilinogen level

133
Q

What drugs are good for porphyria patient?

A
  • Short acting drugs
  • N2O, isoflurane, desflurane, opioids, benzos, muscle relaxants all ok to use
134
Q

Considerations with propofol for porphyria patients

A

Avoid propofol infusion

Total dose and length of exposure increases risk of triggering

135
Q

Is regional an option for porphyria patients?

A

Yes after careful neuro assessment

136
Q

Consderations for treating acute porphyria crisis (4)

A
  • Stop any possible triggers
  • Fluid/glucose loading, monitor electrolytes
  • Consider analgesics, anti-emetics, B-blockers, benzos or propofol for seizures
  • Heme (arginate) infusion, somatostatin (Octreotide) to decrease ALA synthetase, plasmapheresis
137
Q

s/s of pheo

A
  • HTN
  • Headache
  • Sweating
  • Pallor
  • Palpitaitons
  • Orthostatic hypotension

(SHOPP)

S5 - Advanced Patho (6 decks)

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