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PHAEOCHROMOCYTOMA

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17 views

PHAEOCHROMOCYTOMA

Uploaded by

Zubair
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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DR FAHAD BUTT DR AHMED SHAHBAZ

PHAEOCHROMOCYTOMA

A phaeochromocytoma is a functionally active, catecholamine-secreting tumor of the neuroendocrine


chromaffin cells found in the sympathetic nervous system, accounting for about 0.1% of cases of
hypertension. They are usually benign and localized to an adrenal gland. These tumors are important to
the anesthetist because they can present unexpectedly peri-operatively and the mortality is high.

They may secrete any combination of

o Noradrenaline
o Adrenaline
o Dopamine
o Vasoactive intestinal peptide (VIP) or ACTH.

PRESENTATION:

The commonest presentation is sustained hypertension.

The classic presentation is of paroxysms of sympathetic crises

o Severe hypertension
o Flushing
o Sweating
o Palpitations
o Headache
o Anxiety
o Weakness
o Lethargy

Paroxysms may be precipitated by multiple factors:

o Simple activities such as exercise


o Maneuvers that increase intra-abdominal pressure such as sneezing, voiding and defecating.
o Histamine releasing drugs and succinylcholine
o Anesthetic procedures (particularly intubation)

If undiagnosed, it may present with end-organ damage

o Heart failure
o Pulmonary edema
o Myocardial ischemia
o Cerebro-vascular events

Presentation may be determined by the dominant catecholamine secreted:

o Mainly Noradrenaline
o Hypertension, headaches, Slow, thudding palpitation
o Mainly Adrenaline
o Tachycardia’s Anxiety attacks
DR FAHAD BUTT DR AHMED SHAHBAZ

o Mainly Dopamine
o Nausea and vomiting

EFFECT OF ADRENO-RECEPTORS:

α1 o Vasoconstriction
o Sweating
o ↓ insulin and glucagon release
α2 o Inhibition of noradrenaline release
β1 o Chronotropy
o Inotropy
o Renin secretion
β2 o Smooth muscle relaxation
o bronchi
o vascular wall
o uterus
o ↑ insulin and glucagon release

DIAGNOSIS:

o Clinical suspicion on the basis of history or hypertension.


o Traditionally by measuring urinary catecholamine’s or their metabolites (vanillylmandelic acid
[VMA] and metanephrine).
o Clonidine Suppression Test – lack of suppression suggests phaeochromocytoma (prevents
noradrenaline reuptake).
o Genetic testing may identify familial cases.
o CT can localise adrenal tumours with 93%–100% sensitivity.
o MRI scan of the abdomen has a slightly higher sensitivity for extra-adrenal tumours.
o An isotope scan may help to localise an extra-adrenal tumour.
o A radio-labelled iodine isotope, 123I-metaiodobenzylguanidine (MIBG), is taken up by the
chromaffin cells.
o Selective vena caval sampling

RULE OF 10
o 10% are extra-adrenal and can be anywhere along the sympathetic chain from skull base to pelvis.
o 10% are bilateral.
o 10% are malignant.
o 10% occur in children.
DR FAHAD BUTT DR AHMED SHAHBAZ

ASSOCIATION WITH OTHER CONDITIONS:

o Multiple endocrine neoplasia (MEN) Syndrome Type 2


o Type 2A – medullary thyroid carcinomas and parathyroid adenomas
o Type 2B – medullary thyroid carcinomas and Marfanoid features.
o Von Hippel–Lindau disease
o Neurofibromatosis Type I
o Familial carotid body tumors

PERIOPERATIVE INVESTIGATIONS, 10 WEEKS BEFORE SURGERY

o The aim of pre-operative management is to:


o Determine the site of the tumor and what it secretes.
o Normalize the blood pressure.
o Allow the resolution of catecholamine cardiomyopathy.
o Correct hypovolemia (contracted intravascular volume).
o An echocardiogram is useful to assess left ventricular function and to exclude cardiomyopathy
o ECG
o Alpha and Beta-adrenoceptor blockade Controls the symptoms and hypertensive swings
o Reduces the hypertensive surges associated with tumor handling.
o Alpha -adrenoceptor blockade should be instituted prior to Beta-blockade.

ALPHA BLOCKERS:

o PHENOXYBENZAMINE is a non-selective alpha1 and alpha2 blocking drug


o It binds covalently and irreversibly to the receptors.
o It has a long duration of action and may contribute to post-operative hypotension.
o Alpha 2 blocking can inhibit pre-synaptic noradrenaline re-uptake
o Usually necessitates the use of beta-blockade to treat secondary tachycardia

o PRAZOSIN and DOXASOSIN are shorter acting competitive alpha1 selective blocking drugs
o Less tachycardia
o May not require adjuvant Beta blockade unless the tumor is secreting adrenaline

ARE CARDIOSELECTIVE B BLOCKER NECESSARY?

In theory, avoiding β2 blockade will allow β2-mediated vasodilatation to continue but in practice a
selective β1-blocker is not necessary. β-blockers are usually added to control tachycardia secondary to -
blockade. Selective β1- blockers may be used for adrenaline secreting tumors or to treat the tachycardia
associated with the use of phenoxybenzamine
DR FAHAD BUTT DR AHMED SHAHBAZ

WHAT ARE THE SURGICAL TECHNIQUES AVAILABLE FOR RESECTION

OPEN Lateral retroperitoneal approach

ADVANTAGES:
Quicker
Fewer catecholamine surges

DISADVANTAGES:
More painful
LAPAROSCOPIC APPROACH ADVANTAGES:
Shorter recovery time

DISADVANTAGES
Greater surgical manipulation – more instability
Long operation

HOW TO ANESTHETIZE THIS PATIENT.


o Sedative pre-medication
o Invasive arterial and central venous monitoring is essential.
o Large bore IV access
o The patient may be intravascularly depleted after prolonged sympathetic over-stimulation.
o Large fluid shifts are possible.
o Cardiac output monitoring is useful in those with cardiomyopathy
o Induction with remifentanil and propofol. Remifentanil may be useful in this instance as it has short-
acting sympatholytic properties.
o Vecuronium to paralyze the patient to avoid the potential histamine (and therefore catecholamine)
release associated with other agents.
o Intubate the patient and maintain anaesthesia with a mixture of oxygen, air, sevoflurane and
remifentanil.
o Opioid-based analgesia is reasonable for a laparoscopic technique.
o Thoracic epidural for an open procedure.
DR FAHAD BUTT DR AHMED SHAHBAZ

HOW TO CONTROL INTRA-OPERATIVE BLOOD PRESSURE SWINGS/ WHICH DRUGS NEED TO BE


IMMEDIATELY AVAILABLE

o Phentolamine (a non-selective α-antagonist) may be given as an infusion or a bolus (1–2 mg


increments). It acts within one circulation time.
o The heart rate should be kept below 100 bpm. Short-acting β-blockers such as Esmolol or
Metoprolol may be useful for this.
o Magnesium blocks catecholamine release and the adrenoceptor response to noradrenaline. It also
has anti-arrhythmic activity.
o Labetalol is a combined α and β blocker (∼1:10 α:β activity). Onset in 5 minutes but effect may last
hours.
o There can be hypotension during these procedures too, particularly once the tumor has been
removed.
o There may be profound hypovolemia necessitating several liters of fluid.
o Vasopressors such as epinephrine and metaraminol should also be immediately

TREATMENT OF POSTOPERATIVE HYPOTENSION:

o Post-operative hypotension is a common problem as the source of catecholamines has been


removed but the adrenergic blockade remains.
o Assessment of pre-load/volume status, cardiac function, inotropy and the peripheral vascular
resistance should guide treatment.
o Catecholamine infusions in ‘normal’ doses may be ineffective, therefore fluid and posture may need
to be the mainstays of treatment.
o Early extubation helps by negating the need for sedation.
o Adrenaline can be useful if there is evidence of left ventricular failure.
o Noradrenaline or Vasopressin may be useful in refractory vasodilatation.
o When both adrenals have been removed, hydrocortisone is required immediately.

SPECIFIC PROBLEM IN POSTOPERTAIVE PERIOD:

o Hypoglycemia, symptoms of this may be masked by -blockade so the glucose should be measured
regularly
o Glucocorticoid and mineralocorticoid deficiency requiring supplementation with hydrocortisone
and fludrocortisone.
o Electrolyte and fluid imbalance

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