- HA - sweating - pallor - palpitations - orthostatic HoTN

Exam IV: Pheo, Spleen, Syndromes Flashcards by Linsley Lloyd

what is a pheochromocytoma

Catecholamine secreting tumor from the chromaffin cells of the adrenal system

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Chromaffin cells produce ______ and ______

epinephrine and norepinephrine

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Pheo can be _______

lethal

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Pheo resection in the OR can ______ _______

cure HTN

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Pheos found equally in _____ & ______

males and females

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usually in adults ___-___ years but ___% in children

30-50 years
10%

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Familial pheo can be part of ______ _____ ______ (_____) Syndrome

Multiple Endocrine Neoplastic (MEN)

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Almost _____% of MEN II pts have or will have a pheo

100

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pheo - ____% in the adrenal medulla

80%

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pheo can be _____ or _____

malignant or benign

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pheo can secrete _____ and/or _____

NE and/or epi

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Most common pheos secrete norepi:epi at a ____:____ ratio. Normal (non-pheo) numbers are _______.

85:15
reversed

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most pheos secrete without ______ control

neurogenic

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pheo symptoms occur _____ to ______ times a day - a few minutes to several hours

infrequently to several

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pheo hallmark symptom

HTN (test question)

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other pheo symptoms:

HA
sweating
pallor
palpitations
orthostatic HoTN

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pheo norepi effects: increased ______ and ______ with reflex _______ (alpha agonist)

SBP and DBP
bradycardia

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pheo epi effects: increased _____ and decreased ______, tachycardia (____ agonist)

SBP
DBP
beta

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pheo - increased _____, normal CO, slightly decreased ____ _____

SVR
plasma volume

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pheo - cardiomyopathy usually _____

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pheo EKG changes - _____ changes, flat/inverted T waves, prolonged _____, peaked _____ waves, L axis deviation, dysrhythmias

ST
QT
P

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pheo - increased _____ d/t _____ insulin release

BG
inhibited

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Pheo - Diagnosis

24 hr _____ ______ and catecholamines

urine metanephrines

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Pheo - Diagnosis

most sensitive test

plasma free metanephrines

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Pheo - Diagnosis Normetanephrine (norepinephrine metabolite) >_____ pg/mL and/or

400

Pheo - Diagnosis Metanephrine (epinephrine metabolite) >_____ pg/mL

220

Pheo - Diagnosis If questionable results: ______ ______ test or _____ ______ test (if DBP <100)

Clonidine suppression glucagon stimulation

Pheo - Diagnosis location found by

- CT - MRI - PET

Pheo - Diagnosis functionality by ______ (concentrates in catecholamine-secreting tumors)

MIBG

Pheo - Diagnosis catecholamine samples from _____ _____ (cath lab procedure)

adrenal vein

Pheo - Diagnosis differentials - must differentiate from 3 other pathologies

- MH - thyroid storm - carcinoid crisis

Pheo - Diagnosis * Catecholamines produced by pheos are metabolized within ______ cells. So plasma free metanephrine levels are ____ _____ for diagnosing a pheo than plasma epi and norepi levels.

chromaffin more accurate

Reminder: Most pheos secrete ↑ _____ ( ___ _____ ).

norepi (α-agonist)

____-blockade to ↓ BP, ↑ volume, prevent HTN episodes, re-sensitize receptors, ↓ myocardial dysfunction

Most common: ______ (____) – non-competitive, non-selective (α1 and α2 ) irreversible α-blocker

Phenoxybenzamine (Dibenzyline®

overtreatment of a pheo can lead to _____ ______

orthostatic hypotension

D/C alpha blockade ____-____ hours pre-op to avoid refractory hypotension

24-48

Prazosin (Minipress®) & doxazosin (Cardura®) - ____ ____ blocker, shorter acting, less tachycardia, easier to titrate

pure α1

Treat phenoxybenzamine-induced tachycardia with:

non-selective β-blockade (usually propranolol; also atenolol, metoprolol, labetalol).

EXTREME CAUTION with ___-blockade before ___-blockade

β α (β2 blockade leads to unopposed α effects which causes vasoconstriction and HTN crisis)

Other adjuncts: Metyrosine (tyrosine inhibitor causes blocked catecholamine synthesis), ____ channel blockers, ____ inhibitors

Ca++ ACE

primary intra-op goals of pheo:

Avoid catecholamine release (drugs, stimulation*) Maintain CV stability with short-acting drugs *Think about laryngoscopy, incision, light anesthesia, emergence…

Ligation of tumor-related vessels leads to _____ ______

significant HoTN

Standard monitoring + ____ ____ (consider CVP, PA cath, TEE)

arterial line

pheo - intra op you must avoid ______

hypovolemia

avoid drugs with known ____ effects:

CV MSO4, atracurium, atropine, sux, pancuronium, ephedrine, ketamine

______ ______ most common approach but insufflation and tumor manipulation causes HTN.

Laparoscopic adrenalectomy

_____ ______ required for large tumors

open excision

Common with pheo: ____ ____ intra-op regardless of pre-op α-blockade

SBP >200

Have _____, ____-____ drugs prepared and ready for pheo intra-op

potent, rapid-onset

_____ is drug of choice; phentolamine, NTG, labetolol, MgSO4, esmolol, diltiazem.

Nipride

↑ anesthetic depth leads to ↑ risk of hypotension with ____ _____

venous ligation

Lidocaine and/or β-blockers (propranolol, esmolol) for _____ _____

ventricular dysrhythmias

Common: SBP ____ after venous ligation Pre-treat with crystalloids; pressors and inotropes if needed.

< 80

avoid ____ ____ blood because it's high in ______

cell saver catecholamines

after pheo resection, increased _____ and decreased _____. Begin _____ solution after resection

insulin glucose dextrose

With complete resection, most pts eventually become _____tensive.

normo

Plasma catecholamines levels do not normalize for __-__ days.

7-10

Approx 50% will be hypertensive for ____ _____; approx. 25% remain hypertensive for ____.

several days life

Most frequent immediate post-op cause of death:

Hypotension d/t sudden ↓ catecholamines with refractory vasodilation. (treat with fluids and pressors if necessary)

steroids for ______

hypoadrenalism

Functions of the Spleen

Blood filtering, erythrocyte morphology, immunity related to blood-borne antigens

Hematopoiesis until the ___ _____ month

5th gestational

Macrophages engulf abnormal (SC dz, thalassemia, spherocytosis) and older (>120 days) blood cells which can lead to ____, _____, ______ ______.

anemia, splenomegaly, splenic infarction

Splenic macrophages, histiocytes and IgM play important roles in ______.

immunity

spleen - Minor role as red blood cell and platelet _____

reservoir

______ ______ done for hypersplenism, ITP, TTP, hereditary spherocytosis, hereditary hemolytic anemia, hemoglobinopathies, malignancies, abscesses, cysts.

Elective splenectomies

Emergent splenectomies done for splenic _____.

trauma

The spleen receives ___% of cardiac output.

splenic bleeding is usually _____

arterial

Blunt abdominal trauma + ____ ____ _____ pain = high risk of splenic trauma (commonly from fractured ____)

left upper quadrant rib

splenectomy - Usually done ______ but with hemodynamic instability, ______ may be approach of choice.

laparoscopically laparotomy

for splenectomy, have _____ available

blood

Asplenic pts have increased risk for _____ processes (bacteremia, pneumonia, meningitis) and _____ for life.

infectious thromboembolism

Most carcinoid tumors occur in the

bronchus, jejunum/ileum or colon/rectum.

carcinoid tumors secrete ___ ____ and ____ substances that may or may not cause symptoms.

GI peptides vasoactive

large amounts cause

carcinoid syndrome

carcinoid syndrome symptoms

- flushing - diarrhea (can also have pruritus, tearing, and facial edema)

carcinoid syndrome precipitated by:

stress, ETOH, exercise, diet, drugs (catecholamines, SSRIs)

Serotonin antagonists used for ______; H1/H2 blockers for ______ and _______.

diarrhea flushing and pruritus

carcinoid syndrome can cause life threatening ______ ______

carcinoid crisis

CC symptoms:

flushing, diarrhea, abd pain, and CV instability

CC can be precipitated by:

sux, benzylisoquinolines (except cisatracurium), catecholamines, dopamine, isuprel, thiopental

Preventing/Treating Carcinoid Syndrome Avoid _____ conditions. may need ___/_____

precipitating diet/supplements

Preventing/Treating Carcinoid Syndrome _____ antagonists, ______ analogues, ___/___ blockers S-statin analogues (octreotide, lanreotide) for 24-48 hrs pre-op and continued throughout procedure.

5-HT3 somatostatin H1/H2

Preventing/Treating Carcinoid Syndrome Only cure: _____ _____ of the carcinoid tumor

Surgical excision

Preventing/Treating Carcinoid Syndrome GA with art line, epidural with monitoring if treated pre-op with ______.

octreotide

Preventing/Treating Carcinoid Syndrome May have delayed awakening d/t:

↑ serotonin levels.

Fibromyalgia Widespread MSK pain with _____, _____, ______ and mood issues

fatigue, sleep, memory

Fibromyalgia Current theory: Repeated _____ _____ causes increased neurotransmitters which leads to a hyperactive response to _____

painful stimuli pain

Fibromyalgia frequently associated with:

H/A, TMJ disorders, irritable bowel syndrome, anxiety, depression

Fibromyalgia _____ more than _____, no ______. Treat with exercise, stress reduction, alternative medicine (acupuncture, massage therapy, yoga), meds (NSAIDS, anti-depressants, gabapentinoids).

females males cure

Fibromyalgia ______ (____): 1st FDA approved drug for fibromyalgia

Pregabalin (Lyrica®)

Sarcoidosis Autoimmune, interstitial, granulomatous, usually in ____, ____, ____, ____, ______

lungs, lymphatics, bone, liver, CNS

Sarcoidosis ____ _____ lung dz causing dyspnea, cough, pulmonary HTN and cor pulmonale.

Chronic restrictive

Sarcoidosis Usually diagnosed by ______; many asymptomatic at time of diagnosis.

radiography

Sarcoidosis Common manifestations: _______ (uncontrolled synthesis of a vitamin D3 enzyme by macrophages) and ____ ____ _____ (neurosarcoidosis, parotid gland)

Hypercalcemia facial nerve paralysis

Sarcoidosis biopsy done with _______

mediastinoscopy

Sarcoidosis Treatment: ______ for symptoms and hypercalcemia

Corticosteroids

Sarcoidosis AIs: Pts have poor tolerance to apnea d/t ____ _____. Keep PIPs as low as possible to minimize ______ risks.

small FRC barotrauma

Systemic Lupus Erythematosus Multisystem, chronic inflammatory, caused by _____ _____

antibody production

Systemic Lupus Erythematosus Usually _____ _____, exacerbated by ______ (surgery, pregnancy, infection)

young females stressors

Systemic Lupus Erythematosus Can be drug induced (usually... 5)

procainamide, hydralazine, isoniazid, penicillamine, methyldopa

Systemic Lupus Erythematosus symptoms

Arthralgias, maculopapular (butterfly) rash, fever, anemia, leukopenia

Systemic Lupus Erythematosus _____ and/or hypertension worsens prognosis.

Nephritis

Systemic Lupus Erythematosus Systemic involvement: CNS, _____, _____, ______, liver, hematologic, MSK and skin

heart, lungs, kidneys

Systemic Lupus Erythematosus Treatment based on systems affected and symptoms with:

ASA, NSAIDs, chemo, steroids

Systemic Lupus Erythematosus AIs: Consider organ dysfunction, _____ neck, ____ _____ nerve palsy, drugs

arthritic recurrent laryngeal

Scleroderma (Systemic Sclerosis) _____/______ dz causes inflammation, vascular sclerosis, fibrosis of skin and organs

Collagen/autoimmune

Scleroderma (Systemic Sclerosis) Vascular injury leads to leaking proteins causing _____ and ______ in skin, MSK, CNS, CV, lungs, kidneys, GI tract

edema and fibrosis

Scleroderma (Systemic Sclerosis) Can become _____ _____ (Calcinoses, Raynaud’s phenomenon, Esophageal hypomotility, Sclerodactyly, Telangiectasis) with poor prognosis.

CREST syndrome

Scleroderma (Systemic Sclerosis) CV: Conduction, contractility, ____ ____ HTN, cor pulmonale (Consider eval pre-op.)

pulm artery

Scleroderma (Systemic Sclerosis) Lungs: May need ↑ _____, acidosis/hypoxia leading to increased pulm VR, sensitive to _____

PIPs opioids

Scleroderma (Systemic Sclerosis) Renal: HTN, irreversible renal failure (Tx: ____ _____)

ACE inhibitors

Scleroderma (Systemic Sclerosis) GI: ↓ motility, dysphagia, GERD, Vit K malabsorption causing coag problems (Tx: ______. ______ ineffective.)

Octreotide Prokinetics

Scleroderma (Systemic Sclerosis) _______: Oral/nasal bleeding with intubation

Telangiectasis

Scleroderma (Systemic Sclerosis) Skin: Difficult ____ _____, peripheral vasoconstriction causes ______ in OR

IV access hypothermia

Scleroderma (Systemic Sclerosis) _______ may be difficult but can be advantageous (peripheral vasodilation, opioid sparing).

Regional

What is Porphyria?

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

Heme is a ______

porphyrin

Binds with proteins to make _______ and ______ ______ isoenzymes

hemoglobin and cytochrome P-450

Heme production is regulated by _____ _____ (___) ______

aminolevulinic acid (ALA) synthetase

ALA is easily ______ by drugs using P-450 system

induced

With porphyria, ↑ _____ requirements causes _____ stimulation which leads to accumulated ______ (compounds preceding the site of the deficiency)

heme ALA intermediates

porphyria classified as ______ or ______; acute and non-acute

hepatic or erythropoietic

only forms of porphyria relevant for anesthesia

acute

certain drugs cause _____-_____ _____

life-threatening reaction

acute porphyrias are inherited, _____ dominant

autosomal

acute porphyrias more frequent in women in there ____s or ____s, and is rare after ______

30s or 40s menopause

acute porphyrias are also triggered by ____

stress (childbirth, fasting, dehydration, illness, infection)

acute porphyrias (4)

- plumboporphyria - acute intermittent porphyria - hereditary coproporphyria - variegate porphyria

“Silent” or “latent” porphyria pts may show ____ _______after perioperative triggering drugs.

1st symptoms

porphyria - ______ “inducers” more high risk than _____ exposure

Multiple single

porphyria avoid list

Barbiturates (thiopental, methohexital), sevoflurane (?), etomidate (?), pentazocine (Talwin), ephedrine (?), ketamine (?), traditional seizure meds

porphyria - acute exacerbation symptoms

Skeletal muscle weakness, HTN, tachycardia, abdominal pain, seizures, electrolyte imbalance (especially hyponatremia)

porphyria - NPO status can trigger. Consider _____ _____

glucose IVF

porphyria - Rule out acute crisis with _____ _______ (____) level.

urine porphobilinogen (PBG)

Short acting drugs tolerated well; avoid _____ _____. (Total dose and length of exposure ↑ risk of triggering.)

propofol infusion

porphyria - Regional OK after careful _____ assessment.

neuro

drugs that are OK for porphyria

N2O, isoflurane, desflurane, opioids, benzos, MRs OK.

treating acute porphyria crisis:

- Stop any possible triggers - Fluid/glucose loading, monitor electrolytes - Consider analgesics, anti-emetics, β-blockers, benzodiazapines or propofol for seizures - Heme (arginate) infusion, somatostatin (Octreotide®) to ↓ ALA synthetase, plasmapheresis