Pheochromocytoma, Splenic, Carcinoid, Blah Blah Flashcards
(137 cards)
1
Q
What is a pheochromocytoma?
A
Cathecholamine secreting tumor from the chromaffin cells of the adrenal system
2
Q
What do chromaffin cells produce?
A
Epinephrine and norepinephrine
3
Q
Can pheochromocytoma be cured?
A
HTN can be cured by resection
4
Q
Pheo patient demographics
A
- Equal male and female
- Usually 30-50 years old
- 10% in children
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
6
Q
In what part of the adrenal gland are most pheos found?
A
80% in the adrenal medulla
7
Q
Are pheos malignant or benign?
A
Can be either
8
Q
What do pheos secrete?
A
- Can secrete norepinephrine and/or epinephrine
- Usually secrete norepi 85% and epi 15% (normal ratio reversed)
9
Q
How does neurogenic control of pheos occur?
A
It usually doesn’t
Pheos secrete catecholamines without neurogenic control
10
Q
Hallmark symptom of pheo
A
Hypertension
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
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
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
14
Q
Effects of pheo on SVR, CO and plasma volume
A
- Increased SVR
- Normal CO
- Slightly decreased plasma volume
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
16
Q
Cardiomyopathy is common with pheo..
Which chamber usually?
A
LV
17
Q
blood glucose in pheo patients (prior to resection)
A
Increased due to inhibited insulin release
18
Q
How do you diagnose a pheo?
What is most sensitive?
A
24 hours metanephrines and catecholamines
Most sensitive is plasma free metanephrines
19
Q
What are the two plasma free metanephrines?
A
Metanephrine and normetanephrine
20
Q
What is normetanephrine?
What is considered elevated?
A
Norepinephrine metabolite
>400 pg/mL
21
Q
What is metanephrine?
What is considered an elevated level?
A
Epinephrine metabolite
>220 pg/mL
22
Q
test to perform if plasma free metanephrines questionable?
A
clonidine suppression test or glucagon stimulation test (if DBP <100)
23
Q
imaging for a pheo
A
- CT
- MRI
- PET scan
24
Q
What pheo test may be done in the cath lab?
A
Taking catecholamine samples from adrenal vein
25
Differential diagnoses of pheo
* MH
* Thyroid storm
* Carcinoid crisis
26
Where are catecholamines from pheos metabolized?
What is the significance?
Metabolized within chromaffin cells
plasma free metanephrines are more accurate for diagnosing pheo than plasma epi and norepi levels
27
T/F - mosty pheos secrete more epi than norepi
false - most pheos secrete norepi more than epi
28
Effects of alpha blockade treatment for pheo
* Decrease BP
* Increase volume
* Prevent HTN episodes
* Re-sensitize receptors
* Decrease myocardial dysfunction
29
Most common alpha blocker for pheo
MOA?
Phenoxybenzamine (Dibenzyline)
MOA: non competitive, non-selective irreversible a-blocker
30
adverse effect of alpha blocker overtreatment
How to avoid in pheo pts?
Orthostatic hypotension
D/C 24-48 hours pre-op to avoid refractory intra-op hypotension
31
Two shorter acting pure alpha-1 blockers
Benefits vs. phenoxybenzamine
* Prazosin (Minipress) and doxazosin (cardura)
* Benefits: less tachycardia, easier to titrate
32
How do you treat phenoxybenzamine induced tachycardia?
With non-selective B-blockade
* Usually Propanolol
* Also atenonol, metoprolol, labetalol
33
When treating pheo, do you want to block alpha or beta first?
Alpha
34
Why would you want to use EXTREME CAUTION with beta-blockade prior to alpha-blockade?
Beta blockade--\> unopposed alpha effects --\> vasoconstriction and HTN crisis
35
adjunct treatments for pre-op pheo
* Metyrosine
* Ca++ channel blockers
* ACE inhibitors
36
How does metyrosine treat a pheo?
Metyrosine is a tyrosine inhibitor so it blocks catecholamine synthesis
37
2 primary intra-op goals for a pheo
* Avoid catecholamine release (drugs, stimulation)
* Maintain CV stability with short-acting drugs
38
What occurs with ligation of tumor-related vessels with pheo intraop?
Significant hypotension
39
Monitoring intraop for pheo
* Standard monitoring
* Arterial line
* Conisder CVP, PA, and TEE also
40
T/F You want your patient slightly hypovolemic for pheo
FALSE
Avoid hypovolemia
Would cause catecholamine release? maybe
41
drugs to avoid in a pheo pt
* Morphine
* Atracurium
* Atropine
* Succinylcholine
* Pancuronium
* Ephedrine
* Ketamine
42
Most common approach for pheo excision
Disadvantages?
* Laparoscopic adrenalectomy
* Insufflation and tumor manipulation cause HTN
43
What is required for excision of large pheos?
Open excision
44
Name some times during surgery that we would cause increased catecholamine release with pheo
* Laryngoscopy
* Incision
* Light anesthesia
* Emergence
45
Is pre-op alpha blockade always effective for preventing hypertension?
Nope
SBP \>200 is common intra-op regardless of pre-op alpha blockade
46
Drugs to have prepared for pheo patient?
"Have potent, rapid onset drugs prepared and ready"
* Nipride - drug of choice
* Phentolamine
* Nitroglycerine
* Labetalol
* Mag sulfate
* Esmolol
* Diltiazem
47
caution with using increased anesthetic depth to treat HTN with pheo
Increases risk of hypotension with venous ligation
48
What drugs should you have available for ventricular dysrhythmias with pheo?
* Lidocaine
* Beta-blockers (propanolol, esmolol)
49
How to prevent and treat hypotension associated with venous ligation of pheo
* Prevent: pre-treat with cyrstalloids
* Treat: pressors and inotropes if needed
50
T/F Cell saver is a good option for pheo patients to prevent hypotension with venous ligation
False
Cell-saver is high in catecholamines
51
What happens to blood sugar after resection of pheo?
How do you treat?
* Increased insulin and decreased glucose
* Begin dextrose solution after resection
52
When does hypertension resolve after pheo resection?
Most patients eventually become normotensive
* Approximately 50% with be hypertensive for several days
* 25% remain hypertensive for life
* ?? about the other 25%
53
How long does it take for plasma catecholamine levels to normalize?
(after pheo resection or what?)
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?
54
Most frequent post-op cause of death after pheo resection
How to treat? (if they havent died yet)
Hypotension due to sudden decrease in catecholamines with refractory vasodilation
Treat with increased fluids, pressors if necessary
55
How to treat hypoadrenalism after pheo resection
Steroids
56
What may occasionally be first indication of an undiagnosed pheo?
Unexpected intra-op hypertension and tachycardia during manipulation of abdominal structures
57
What may be unmasked in volume expansion treatment for pheo?
Underlying anemia
58
When is art line particularly useful in pheo excision?
Is CVL always necessary?
During induction and manipulaiton
CVL not needed in young patients with minimal or no heart disease
59
T/F An awake intubation is indicated in pheo patient
FALSE
Intubation should not be attempted until deep level of GA has been established, possibly even LA of trachea
60
What occurs in the spleen until the 5th gestational month?
Hematopoiesis
61
Functions of the spleen
* Blood filtering
* Erythrocyte morphology
* Immunity related to blood-borne antigens
62
The spleen is a reservoir for what?
* Red blood cells
* Platelets
63
What can macrophages engulf that can lead to anemia, splenomegaly, and spenic infarcion?
* Abnormal blood cells
* Sickle cell
* Thalasemia
* Spherocytosis
* Older blood cells (\>120 days)
64
What according to these notes play important roles in immunity?
* Macrophages
* Histiocytes
* IgM
65
Reasons elective spleenectomies are done (9)
* Hyperspleenism
* ITP
* TTP
* Hereditary spherocytosis
* Hereditary hemolytic anemia
* Hemoglobinopathies
* Malignancies
* Abcesses
* Cysts
66
Why are emergent splenectomies performed?
* Splenic trauma
67
What percent of CO does the spleen recieve?
5%
68
Is splenic bleeding usually venous or arterial?
Arterial
69
What patients are at high risk of splenic trauma?
Blunt abdominal trauma + LUQ pain
Commonly from fractured rib
70
How is splenectomy usually performed?
What should you have available?
* Usually laparoscopically
* Laparotomy if HD instability
* Have blood available
71
What are asplenic patients at higher risk for?
* Infectious processes
* bacteremia
* pneumonia
* meningitis
* Thromboembolism
72
Where do most carcinoid tumors occur?
* Bronchus
* Jejunum/ileum
* Colon/rectum
73
What do carcinoid tumors secrete?
GI peptides and vasoactive substances that may or may not cause symptoms
74
Large amounts (of carcinoid tumors or GI peptides, unsure) can cause
Carcinoid syndrome
75
S/S of carcinoid syndrome
* flushing
* diarrhea
* pruritis
* tearing
* facial edema
76
what precipitates carcinoid syndrome?
* stress
* alcohol
* exercise
* diet
* drugs (catecholamines, SRIs)
77
how to treat diarrhea with carcinoid syndrome?
serotonin antagonists
78
how to treat flushing and pruritis with carcinoid syndrome
H1/H2 blockers
79
What life threatening condition can carcinoid syndrome lead to?
Carcinoid crisis
80
S/S of carcinoid crisis
* Flushing
* Diarrhea
* Abdominal pain
* CV instability
81
What drugs can precipitate carcinoid crisis?
* Succinylcholine
* Benzyisolquinolines (except Cis)
* Catecholamines
* Dopamine
* Isuprel
* Thiopental
82
only cure for preventing/treating carcinoid syndrome
surgical excision of carcinoid tumor
83
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?
S-statin analogues
* Octreotide
* lanreotide
GA with art line; can use epidural if treated with pre-op octreotide
84
Pre-op instructions and prophylactic drugs for carcinoid tumors
* Avoid precipitating conditions
* Diet/supplements
* 5-HT3 antagonists
* Somatostatin analogues
* H1/H2 blockers
85
Why may patient with carcinoid tumor/syndrome have delayed awakening from anesthesia?
Increased serotonin levels
86
Characteristics of fibromyalgia
* Widespread musculoskeletal pain
* Fatigue
* Sleep, memory and mood issues
87
Current theory of what causes fibromyalgia
Repeated painful stimuli ➡️ increased neurotransmitters ➡️ hyperactive response to pain
88
Other issues that frequently occur with fibromyalgia
* Headache
* TMJ disorders
* IBS
* Anxiety
* Depression
89
Fibromyalgia is more common in which gender?
Females
90
Cure for fibromyalgia?
Treatment?
No cure. Treat with:
* Exercise
* Stress reduction
* Alternative medicine (acupuncture, massage therapy, yoga)
* Meds (NSAIDs, anti-depressants, gabapenatnoids)
* Pregabalin (Lyrica)
91
First FDA approved drug for fibromyalgia
Pregabalin (Lyrica)
92
What is sarcoidosis?
Interstitial granulomatous disease usually in lungs, lymphatics, bone, liver, CNS
93
What type of lung disease is sarcoidosis?
Characteristics?
Chronic, restrictive lung disease
* Dyspnea
* Cough
* Pulmonary HTN
* Cor pulmonale
94
How is sarcoidosis usually diagnosed?
Radiography
Many are asymptomatic at time of diagnosis
95
Common manifestations of sarcoidosis
* Hypercalcemia (due to uncontrolled synthesis of vitamin D3 enzyme by macrophages)
* Facial nerve paralysis (neurosarcoidosis, parotid gland)
96
How is biopsy done for sarcoidosis?
Mediastinoscopy
97
Treament for sarcoidosis
corticosteroids for symptoms and hypercalcemia
98
Anesthetic implications for sarcoidosis
* Patients have poor tolerance to apnea due to small FRC
* Keep PIPs as low as possible to minimize barotrauma
99
What is systemic lupus erythematosus?
a multi-system chronic inflammatory condition caused by antibody production
100
which gender is systemic lupus erythematosus more common in?
female
101
stressors of lupus exacerbation
* Surgery
* Pregnancy
* Infection
* Certain meds
102
systemic lupus erythematosus can be drug induced
What drugs cause it?
* Procainamide
* Hydralazine
* Isoniazid
* Penicillamine
* Methyldopa
103
Symptoms of systemic lupus erythematosus
* Arthralgias
* Maculopapular (butterfly) rash
* Fever
* Anemia
* Leukopenia
* Systemic: CNS, heart, lungs, kidneys, liver, hematologic, MSK, and skin (?)
104
What two things worsen prognosis with systemic lupus erythematosus?
Nephritis and/or hypertension
105
Treatment for systemic lupus erythematosus
* Based on systems affected and symptoms
* ASA
* NSAIDs
* Chemo
* Steroids
106
Anesthetic implications of systemic lupus erythematosus
* Consider organ dysfunction
* Arthritic neck
* Recurrent laryngeal nerve palsy
* Drugs (?)
107
What is scleroderma (systemic sclerosis)?
Disorder of collagen/ autoimmune disease
Causes inflammation, vascular sclerosis, fibrosis of skin and organs
108
What does vascular injury in scleroderma cause?
Leaking proteins ➡️ edema ➡️ fibrosis in skin, MSK, CNS, CV, lungs, kidneys, GI tract
109
What is CREST syndrome?
What can cause it?
* Calcinoses
* Raynaud's phenomenon
* Esophageal hypomotility
* Sclerodactyly
* Telangiectasis
Scleroderma can become CREST syndrome
110
T/F CREST syndrome has poor prognosis
tru
111
CV complications of scleroderma
* conduction abnormalities
* contractility abnormalities
* Pulm artery HTN
* cor pulmonale
Consider eval pre- op
112
Lung considerations for scleroderma patient
* May need increased PIPs
* Acidosis/hypoxia
* Increased pulmonary vascular resistance
* Sensitive to opioids
113
Renal abnormalities with scleroderma
Treatment?
* HTN
* Irreversible renal failure
Treat with ACE- inhibitors
114
GI abnormalities with scleroderma
* Decreased motility
* Dysphagia
* GERD
* Vitamin K malabsorption and resulting coag problems
115
Treatment for GI issues with scleroderma
* Octreotide
* Prokinetics are ineffective
116
What is telangiectasis?
(Remember this is the T in CREST syndrome)
Dilation of blood vessels in mucosa
Causes oral/nasal bleeding with intubation
117
AIs for skin abnormalities in pts with scleroderma
* Difficult IV access
* Peripheral vasoconstriction
* Hypothermia in OR
118
Regional use in scleroderma patients
* Difficult but can be advantageous
* peripheral vasodilation
* opioid sparing
119
What is porphyria?
A group of inborn errors of metabolism resulting from specific enzyme deficiency in heme synthetic pathway overproduction of porphyrins
120
How is porphyia classified?
* As either hepatic or erythropoietic
* Either acute or non-acute
121
Pathophysiology of porphyria
| (long so sorry)
* 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
What are the only forms of porphyria relevent to anesthesia management?
Acute porphyrias
* Plumboporphyria
* Acute intermittent porphyria
* Hereditary coproporphyria
* Variegate porphyria
(Vampires Always Have Porphyria)
123
What can happen with anesthesia in a patient with acute porphyria?
Certain drugs can cause life-threatening reactions
For example: thiopental
124
What type of genetic condition is porphyria?
Inherited, autosomal dominant
125
What demographic is porphyria more frequent in?
Women in 30s-40s
Rare after menopause
126
What triggers porphyria?
Stress
* Childbirth
* Fasting
* Dehydration
* Illness
* Infection
127
Silent or latent porphyria may show first symptoms when?
After perioperative trggering drugs
128
What is more high risk for triggering porphyria, single exposure or multiple inducers?
Multiple inducers duh
129
Potential drug triggers of porphyria to avoid
* Barbiturates (thiopental, methohexital)
* Sevo
* Etomidate
* Pentazocine (Talwin)
* Ephedrine
* Ketamine
* Traditional seizure meds
130
S/S of acute porphyria exacerbation
* Skeletal muscle weakness
* HTN
* Tachycardia
* Abdominal pain
* Seizures
* Electrolyte imbalance (esp. hyponatremia)
131
What common aspect of periop instructions can trigger porphyria?
NPO status
Consider glucose IVF
132
How to rule out acute porphyria crisis before surgery
Urine porphobilinogen level
133
What drugs are good for porphyria patient?
* Short acting drugs
* N2O, isoflurane, desflurane, opioids, benzos, muscle relaxants all ok to use
134
Considerations with propofol for porphyria patients
Avoid propofol infusion
Total dose and length of exposure increases risk of triggering
135
Is regional an option for porphyria patients?
Yes after careful neuro assessment
136
Consderations for treating acute porphyria crisis (4)
* 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
s/s of pheo
* HTN
* Headache
* Sweating
* Pallor
* Palpitaitons
* Orthostatic hypotension
(SHOPP)
