SEMESTER-V CHILD HEALTH NURSING-UNIT-1-INTRODUCTION.pdf
PHEOCHROMOCYTOMA.pptx by dr kavya dnb anesthesia
1. ANAESTHETIC MANAGEMENT OF PATIENT POSTED FOR
PHEOCHROMOCYTOMA
MODERATOR : DR.K.L.SUBRAMANYAM
PROFESSOR
PRESENTOR :Dr ROHINI
II YEAR PG
1
2. ANATOMY OF ADRENAL GLAND
• AKA Suprarenal glands are pair of endocrine glands situated
on the upper pole of the kidneys.
• They are made of two parts- the outer cortex , inner medulla
• Cortex is composed of 3 zones:
- Outer, Zona glomerulosa – produces mineralocorticoids
- Middle, Zona fasciculata – produces glucocorticoids
- Inner, Zona reticularis – produces sex hormones
2
3. • The medulla is composed of chromaffin cells that secrete adrenaline and
noradrenaline.
• Arterial supply:
• Superior suprarenal artery( branch of the inferior phrenic artery ),
• Middle suprarenal artery( branch of the abdominal aorta),
• Inferior suprarenal artery( branch of the renal artery)
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4. • Venous drainage:
The right suprarenal vein → inferior vena cava
Left suprarenal vein → left renal vein
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5. CHROMAFFIN SYSTEM
• Adrenomedullary cells are called chromaffin cells or pheochromocytes.
• Cytoplasmic granules turn dark when stained with chromic acid because of the
oxidation of epinephrine and norepinephrine to melanin.
• Differentiate in the center of the adrenal gland in response to cortisol; some cells
migrate to form paraganglia, located on both sides of the aorta.
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6. • The largest cluster of chromaffin cells outside the adrenal medulla is located
near the level of the inferior mesenteric artery and is referred to as the organ
of Zuckerkandl
• Prominent in the fetus and is a major source of catecholamines during the
first year of life.
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7. CATECHOLAMINE AND SYNTHESIS
• The term catecholamine - contain catechol (ortho-dihydroxybenzene) and side
chain with an amino group—the catechol nucleus
• Epinephrine synthesized & stored in adrenal medulla released into systemic
circulation.
• Norepinephrine is synthesized & stored not only in adrenal medulla but also in
peripheral sympathetic nerves.
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8. • Dopamine, the precursor of norepinephrine, is found in the adrenal medulla and
peripheral sympathetic nerves and acts primarily as a neurotransmitter in the
central nervous system.
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11. • BETA 3 - lipolysis
• D1 receptors : stimulation causes vasodilation vascular beds.
• D2 receptors : are presynaptic;
Stimulation of D2 receptors in inhibits the release of
norepinephrine, inhibits ganglionic transmission, and inhibits
prolactin release
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12. PHEOCHROMOCYTOMA AND
PARAGANGLIOMA
• Catecholamine-secreting tumors arise from chromaffin cells of adrenal medulla
referred to as pheochromocytomas
• Arising from sympathetic ganglia catecholamine-secreting paragangliomas
• Both have similar clinical presentations and treated with similar approaches
12
13. • Distinction between pheochromocytoma and paraganglioma is an important one
because of implications for associated neoplasms, risk for malignancy, and genetic
testing
• Catecholamine-secreting tumors are rare,
• Annual incidence of 2 to 8 cases per 1 million people.
• Estimated at 0.1% to 0.6%.
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15. PHEOCHROMOCYTOMA
HISTORY
• The association between adrenal medullary tumors and symptoms was first
recognized by Fränkel in 1886.
• He described Fraulein Minna Roll, age 18 years, who had intermittent attacks of
palpitation, anxiety, vertigo, headache, chest pain, cold sweats, and vomiting.
• She had a hard, noncompressible pulse and retinitis.
• Despite champagne therapy and injections of ether, she died.
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16. • At autopsy, they were initially thought to be angiosarcomas, but later a
positive chromaffin reaction confirmed pheochromocytoma.
• A subsequent study published in 2007 documented the presence of a
germline RET proto-oncogene mutation in four living relatives of Fraulein
Roll—proving that the original patient and her family had multiple endocrine
neoplasia type 2
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17. PHEOCHROMOCYTOMA
• The term pheochromocytoma, proposed by Pick in 1912
• Comes from the Greek words
• phaios (dusky),
• chroma (color), and
• cytoma (tumor)
• describe the dark staining reaction caused by the oxidation of
intracellular catecholamines exposed to dichromate salts.
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18. RULE OF TEN
• 10 % extra adrenal
• 10 % in children
• 10 % familial
• 10 % bilateral or multiple
• 10 % recur
• 10 % malignant
• 10 % incidentally found
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19. • inherited as a familial autosomal dominant trait.
• It may be part of the multiple endocrine neoplasia (MEN) IIA or IIB,VHL,NF
• MEN IIA includes:
• Aka sipple syndrome
• medullary carcinoma of the thyroid,
• parathyroid hyperplasia, and
• pheochromocytoma
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20. • MEN IIB consists:
• Aka gorlin syndrome
• medullary carcinoma of the thyroid,
• pheochromocytoma, and
• neuromas of the oral mucosa
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22. CLINICAL PRESENTATION
• Equal frequency in men and women,
• Primarily in the third, fourth, and fifth decades.
• Rare in children, and when discovered, they may be multifocal and associated
with a hereditary syndrome.
• Caused by the pharmacologic effects of excess concentrations of circulating
catecholamines
22
23. SIGNS AND SYMPTOMS
• The five P”s
• Pressure [HTN]
• Pain [headache] CLASSICAL TRIAD
• Palpitation
• Perspiration
• Pallor
• Paroxysms [the sixth P]
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24. PAROXYSMS- SPELLS
• 10-60 min duration
• Frequency : daily to monthly
• Spontaneous
• PRECIPITATING FACTORS:
• Any activity displacing abdominal contents
• Diagnostic procedures : contrasts
• Drugs : opioids,
• unopposed beta blockade
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26. • forceful heartbeat, pallor, tremor, headache, and diaphoresis.
• spell may start with a sensation of a “rush” in the chest and sense of
shortness of breath, f/b forceful heartbeat and a throbbing headache.
• Peripheral vasoconstriction associated with a spell results in cool or cold
hands and feet and facial pallor.
• Increased sense of body heat and sweating occur toward the end of the
spell
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37. • A pheochromocytoma is excluded :
• if the normetanephrine level is <112 pg/Ml
• and the metanephrine level is < 61 pg/mL
• Plasma catecholamine levels:
plasma total catecholamine levels > 2000 pg/ml
• Clonidine suppression test:
• lower plasma catecholamine levels in normal persons
• no effect on catecholamine levels in those with pheochromocytoma
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38. • Glucagon stimulation test:
• safest and most specific provocative test.
• acts directly on the tumor to induce release of catecholamines.
• positive response : plasma catecholamine > 2000 pg/mL
• performed only in patients with a diastolic blood pressure below 100 mm Hg.
• Genetic testing
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39. • Imaging :
• CT abdomen
• MRI – sensitive than CT for extra adrenal pheochromocytoma
• MIBG :
• Iodine 231/311 labelled metaiodobenzylguanidine
• For cases diagnosed biochemically but no tumour on CT/MRI
• Inject MIBG ,scan at 24hr,48hr,72hr
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40. • False negatives :
• labetolol,
• reserpine,
• TCA [hold these 4-6 week prior to scan]
• ECG : Left ventricular hypertrophy and nonspecific T-wave E/O acute myocardial
infarction or tachyarrhythmia
• Chest radiograph : cardiomegaly
• Blood count : elevated hematocrit consistent with a reduced intravascular volume
and hemoconcentration.
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45. PREOPERATIVE EVALUATION
• should be multidisciplinary, including the surgeon, anesthesiologist, and
endocrinologist.
• Once the diagnosis is confirmed, the patient must be evaluated for possible end
organ damage , and preoperative medical preparation should be started
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47. PREOPERATIVE PREPARATION
• Arterial pressure control
• Reversal of chronic circulating volume depletion
• HR and arrythmia control
• Assessment and optimization of myocardial function
• Reversal of glucose and electrolyte disturbance
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48. ARTERIAL PRESSURE CONTROL
• Phenoxybenzamine- irreversible long-acting (24 to 48 hours),
• non-specific α blocker, started at doses of 10mg od or bd.
• Dose increased by 10-20 mg every 2-3 days as needed
• Most patients need between 20-100 mg/day.
• Side effects- orthostatic hypotension, fatigue, nasal congestion
• Selective alpha 1 blockers
• Prazosin, terazosin, doxazosin
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49. • Not used routinely – incomplete blockade
• Less orthostasis and reflex tachycardia
• Used more for long term Rx [inoperable/malignant types]
• Calcium channel blockers:
• MOA : inhibit norepinephrine induced calcium influx
• Not recommended as monotherapy
• Nifedipine 30 – 90 mg /day
• Nicardipine 30mg BD
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50. HEART RATE AND ARRYTHMIA CONTROL
• Beta blockade :
• Control reflex tachy and prophylaxis of arrythmia
• Started only after effective alpha blockade
• Propranolol : 40mg po BD upto 480 mg/day in divided dose IV 1-2 mg bolus
• S/E : cardiac failure ,bronchospasm
• Atenolol : 50-100mg/day po [max 300mg/day]
• IV 2.5-10mg/day
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51. • Esmolol :[selective beta 1 ]
• IV bolus 250-500 mcg/kg/min;
• Infusion :25-250 mcg/kg/min
• Labetolol :[mixed alpha + beta]
• 50-200 mg po/day
• IV 0.25 mg/kg
• ASSESMENT AND OPTIMIZATION OF MYOCARDIAL FUNCTION
• Echocardiography mandatory
• Impaired cardiac function improve when catecholamine reach normal
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52. METYROSINE
• inhibits catecholamine synthesis by blocking the enzyme tyrosine hydroxylase.
• sedation, depression, diarrhea, anxiety, nightmares, crystalluria,urolithiasis,
galactorrhea,extrapyramidal signs –side effects
• DOSE: 250 mg every 6 hours on day 1
• 500 mg every 6 hours on day 2
• 750 mg every 6 hours on day 3
• 1000 mg every 6 hours on the day before the procedure
• with the last dose (1000 mg) given on the morning of the procedure.
• Reversal of glucose and electrolyte disturbance
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53. ASSESSMENT OF ADEQUATE PREOPTIMIZATION
• Started atleast 10-14 days preop
• Allow sufficient time for re expansion
• Effectiveness of medical preparation is assessed by twice daily orthostatic
blood pressure (BP) measurement
• seated BP of 120/80 mmHg
• standing systolic blood pressure (SBP) ≥90 mmHg
• Heart rate between 60 and 70 beats/minute (bpm) seated
• Heart rate70 to 80 bpm standing
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54. PREOPERATIVE OPTIMISATION
• ROIZENS CRITERIA
• BP not more than 160/90 mmhg
• No orthostatic hypotension < 80/45 mm hg
• Not more than 5 PVC per minute
• ECG free of ST segment ,T wave changes
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56. • General anesthesia, with or without epidural anesthesia
• If epidural anesthesia is used the resulting sympathectomy and vasodilation may
exaggerate hypotension during stage II of the surgery, especially for patients who
have received phenoxybenzamine for alpha adrenergic blockade.
• We place the epidural prior to induction of anesthesia and administer a test dose
with or without epinephrine
• We do not activate the epidural during surgery in order to avoid the
sympathectomy that results.
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57. MONITORING
• Standard ASA monitoring
• SpO2, ETCO2,temperature monitoring,urine output
• Arterial line,EKGmonitor,CVP line
• Swan ganz catheter [CHF,CAD,low EF]
• Oesophageal doppler in peds
• LIDCO,PICO
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58. ANAESTHETIC MANAGEMENT
• Informed and written Consent to be taken
• Nil per oral status to be adequate
• PREMEDICATION :
• Anxiolytic sedative – BDZ – reduce catecholamine release
• Opioids : morphine is avoided dt histamine release
• fentanyl sufentanyl are safe
• Atropine or glycopyrrolate omitted dt tachycardia
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59. • INDUCTION
• Thiopentone sodium/ propofol – widely used
• Etomidate
• Ketamine is not recommended
• Multimodal : BDZ in combination with synthetic opioids with induction agent.
• ATTENUATION OF PRESSOR RESPONSE :
• Lignocaine 2 percent 1-1.5 mg/kg
• Esmolol 50-100mcg/kg/min
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60. • NEUROMUSCULAR BLOCKADE
• DOC: vecuronium [cardiostable]
• Suxamethonium –avoided causes fasciculations and rise in IAP
• Atracurium /mivacurium avoided cause histamine release
• Cisatrac and rocuronium –safe cardiostable and least histamine release
• MAINTENANCE :
• Isoflurane used- doesn’t sensitize myocardium to catecholamines
• Halothane [arrythmogenic] desflurane avoided
• Sevoflurane used
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61. SURGERY
• The laparoscopic approach to the adrenal gland is currently the procedure
of choice for patients with solitary adrenal pheochromocytomas < 8 cm
• Lap should be converted to open adrenalectomy in cases of difficult
dissection, invasion, adhesions, or surgeon inexperience
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62. STAGES OF SURGERY
• Phase I
• tumor dissected and vascular supply isolated, before clamping of effluent vein.
• characterized by periods of severe episodic hypertension and arrhythmias as
adrenal manipulation releases catecholamines.
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63. • MINIMIZING HAEMODYNAMIC RESPONE TO TUMOUR HANDLING
• Sodium nitroprusside :
• a direct vasodilator, is the agent of choice in hypertensive crisis.
• infusion : 0.5 to 5 mcg/kg/minute and adjusted every few minutes for target BP
• To prevent thiocyanate toxicity, infusion should not exceed 3 mcg/kg/minute.
• Reflex tachycardia treated with infusion of a short
• acting beta blocker
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64. • Phentolamine
• a competitive α-adrenergic blocker and direct vasodilator, is effective
• test dose of 1 mg , followed by a 5mg bolus and/or continuous infusion 0.5 to
1 mg/minute IV
• S/E tachyphylaxis and tachycardia
• Nicardipine
• 5 mg/hour and ↑ by 2.5 mg every 15 minutes to desired effect, not exceeding
15 mg/hour
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65. • Labetalol – combined alpha and beta blocker
• 5 to 20 mg IV
• Esmolol – ultrashortacting selective beta adrenergic blocker.
• bolus 10 to 50 mg IV
• infusion 25 to 250 mcg/kg/minute
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66. • Magnesium
• vasodilator that inhibits catecholamine release from the adrenal medulla
• useful for patients with arrhythmias associated with pheochromocytoma who
are intolerant of beta blockers.
• Reserved magnesium for patients whose BP is particularly difficult to control.
• bolus : 2 to 4 g IV over 20 minutes after induction and intubation
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67. • infusion at 1 to 2 g/hour IV
• The infusion is discontinued once the venous drainage from the tumor is ligated
or the tumor is removed.
• magnesium potentiates the effects of neuromuscular blocking agents dosing
and reversal of NMBAs should be guided by a peripheral nerve stimulator.
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68. • Supraventricular tachycardia is common
• IV lidocaine,
• beta blockade with esmolol
• amiodarone
• catecholamines associated with intubation can be prevented or minimized with
deep anesthesia,
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69. • Phase II —
• after the effluent vein is clamped.
• precipitous hypotension can occur because of the sudden drop in endogenous
catecholamine levels,
• Chronic downregulation of alpha adrenergic receptors,
• presence of alpha blocking medication, and
• intravascular volume depletion
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70. • TREATING HYPOTENSION AFTER TUMOUR DEVASCULARIZATION
• treated with aggressive administration of IV fluid,
• discontinuation of vasodilator infusions,
• administration of rapidly acting vasopressors.
• Phenylephrine – drug of choice
• bolus: 40 to 160 mcg IV
• infusion : 10 to 200 mcg/minute IV, titrated to effect.
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71. • Ephedrine:
• avoided before tumor removal.
• Given occurs after tumor resection,
• bolus 5 to 25 mg IV, repeated as needed
• Norepinephrine
• infusion : 2 to 20 mcg/minute IV titrated to effect
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72. • Vasopressin
• infusion :initial dose 0.01 to 0.03 units/minute;
• maintenance dose 0.03 to 0.04 units/minute
• used in cases of refractory hypotension after tumor removal,
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73. Postoperative disposition
• Most patients can be extubated at the end of surgery and can be transferred
to the (PACU) for further monitoring.
• Patients who require vasoactive medications to maintain adequate BP are
subsequently transferred to the (ICU).
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74. POSTOPERATIVE CARE
• Hemodynamic instability –
• Transient postoperative hypotension is common due to :
• downregulation of alpha adrenergic receptors
• residual effects of long acting antihypertensive medications
• hypovolemia
• 50 percent of patients remain hypertensive for 1-3 days after excision of
pheochromocytoma
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75. • Hypoglycemia
• can occur intraoperatively or postoperatively
• dt rebound increase in insulin secretion, as the catecholamine inhibition of insulin
secretion is eliminated with tumor removal
• Hypoglycemia should be suspected when patients are slow to emerge from
anesthesia or are lethargic postoperatively.
• Blood glucose should be measured every 6 hours postoperatively
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76. • Adrenal insufficiency
• Patients who undergo bilateral adrenalectomy are at risk for acute
postoperative adrenal insufficiency
• require glucocorticoid replacement.
• typical regimen is as follows:
• Hydrocortisone 100 mg IV with induction of anesthesia
• Hydrocortisone 100 mg IV every eight hours for 24 hours
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77. • Hydrocortisone taper over three days to maintenance dose
• hydrocortisone 25 mg IV or [PO] twice daily, or
• prednisone 10 mg PO daily
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78. PHEOCHROMOCYTOMA IN PREGNANCY
• Pheochromocytoma in pregnancy can cause the death of both the fetus and
the mother
• MRI (without gadolinium enhancement) is the preferred imaging modality,
• 123I-MIBG scintigraphy and 68Ga-DOTATATE PET/CT are contraindicated.
• use of nitroprusside should be avoided.
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79. • Removed in the second trimester
• Third trimester: medical management is indicated and delivery completed by
cesarean section.
• Spontaneous labor and delivery should be avoided.
• Tumour resected post partum
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80. REFERENCES
• WILLIAMS TEXT BOOK OF ENDOCRINOLOGY 14 TH EDITION
• MILLERS 9 TH EDITION
• STOELTINGS COEXISTING DISEASE 7 TH EDITION
• BARASH
• B.D. CHAURASIA BOOK OF ANATOMY
• ANAESTHESIA FOR ADULT WITH PHEOCHROMOCYTOMA ARTICLE [WOLTERS
KLUWER]
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