Anesthetic management

in Conn’s Syndrome and

Pheochromocytoma

- Dr. Shashikant . Y
 Conn’s Syndrome
 Conn’s Syndrome is a primary
hyperaldosteronism state.
 There is excess secretion of aldosterone
from a functional tumour (aldosteronoma)
independent of physiological stimulus.
 More common in women. Rare in children
 May be assoc. with pheochromocytoma,
acromegaly or primary
hyperparathyroidism.
 Secondary hyperaldosteronism is present when
increased circulating serum concentrations of
renin, as in renovascular hypertension, stimulate
the release of aldosterone.
 Conn’s syndrome is assoc. with hypertension,
whereas aldosteronism in Bartter’s syndrome is
not accompanied by systemic hypertension.
 The prevalence of primary aldosteronism in
patients with essential hypertension appears to
be < 1%
 Signs and Symptoms

 Nonspecific signs and symptoms; some

asymptomatic.
 Headache due to hypertension
 Polyuria
 Nocturia
 Skeletal muscle cramps
 Skeletal muscle weakness
 Hypertension is usually more of a Diastolic
hypertension (DBP often >100-125 mmHg)
 Its due to aldosterone induced Na retention and
resulting ↑ed ECF volume
 Aldosterone ↠ renal excretion of K ↠ hypokalemic
metabolic alkalosis.
 ↑ed Urinary excretion of K >30 mEq daily in the
presence of hypokalemia suggests primary
aldosteronism.
 Hypokalemic nephropathy can result in polyuria and
inability to concentrate urine optimally.
 Diagnosis and Treatment
 Spontaneous hypokalemia with systemic hypertension.
 Plasma Renin activity
 Reduced ↠ prim. aldosteronism
 Increased ↠ secondary aldosteronism
 A ratio of serum aldosterone (in ng/dL) to plasma renin
activity (in ng/mL per hour) > 30 and an absolute level of
aldosterone >15 ng/dL suggest primary aldosteronism.
 Failure to suppress plasma aldosterone (to 5 ng/dL after 500
mL/h of normal saline x 4 h)
 A syndrome with all features of hyperaldosteronism ↠ may
result from chr. Ingestion of Licorice.
 Initial treatment ↠ supplemental K,
competitive aldosterone antagonist –
Spirinolactone
 Anti hypertensive medications
 Drug induced diuresis ↠ accentuation of
hypokalemia ↠ use K sparing diuretic such as
triamterene
 Definitive treatment ↠ surgical excision.
 Anesthetic management
 Aim – good preoperative control of hypertension
and correction of hypokalemia
 Hypokalemia can modify responses to NDMR.
 All routine monitoring like ECG, pulse oximetry,
NIBP, temp. monitoring. In addition invasive
arterial BP is recommended before induction.
 Inhaled or IV agents can be used.
 Use of sevoflurane questionable ; if hypokalemic
nephropathy or polyuria exists preoperatively.
 CVP and PA catheter ↠ for adequate evaluation of
the intravascular fluid volume and response to IV
fluids.
 Aggressive preoperative preparation can convert
excessive intravascular fluid volume status of these
patients to unexpected hypovolemia ↠ manifesting as
hypotension.
 Orthostatic hypotension should be looked for.
 ABG intraoperatively.
 Exogenous cortisol.
 Pheochromocytoma
 Catecholamine secreting tumours originating
from adrenal medulla or in chromaffin tissues
along the paravertebral sympathetic chain, from
pelvis to base of skull.
 Conventionally adrenal tumours are termed
‘pheochromocytomas’ and extra-adrenal ones
‘paraganglionomas’
 > 95% found in abdominal cavity and about 90%
originate in adrenal medulla.
 Rule of Ten

 10% malignant
 10% extra-adrenal sites
 10% Bilateral
 10 % familial
 10 % Asymptomatic
 Occurs in the age MEN IIA MEN IIB
group of 30- 50 yrs. Medullary Medullary
About 1/3rd in children carcinoma of carcinoma of
thyroid thyroid
usually males. Pheochromocyto Pheochromocyto
ma ma
 They may be a part of
polyglandular Parathyroid Mucosal
syndrome referred to hyperplasia neuromas

as MEN IIA or IIB. Marfanoid habitus

Von Hippel –
lindau disease
 Although fewer than 0.1% of patients of
systemic hypertension have
pheochromocytoma, nearly 50% of deaths
in pts. with unsuspected
pheochromocytoma occur during
unrelated anesthesia and surgery or
parturition.
 Signs and symptoms
 Pheochromocytomas usually present with the triad of
 headache,
 sweating and
 palpitations, singly or in combination.
 The sensitivity of diagnosing a pheochromocytoma in a
hypertensive patient with all the three symptoms is more
than 90%.
 Very often, symptoms of co existing disease are
predominant.
 Hypertension in phaeochromocytoma may be
paroxysmal [48%, suggestive of predominant
epinephrine(E) or mixed nor epinephrine (NE)-E tumour],
or sustained (29%, suggestive of a NE tumour). Nearly
13% patients are normotensive.
 No correlation has been observed between plasma
levels of catecholamines and hypertension. Patients with
sustained high catecholamine levels may be
normotensive;
 Patients with recurrent crises may have normal
circulating catecholamines.
 The presynaptic sympathetic nerve terminals are loaded
with NE and E vesicles which get discharged across the
synaptic cleft following even minimal stimulation, causing
a severe haemodynamic response with little or no
increase in circulating catecholamines.
 On the other hand, sustained levels of NE and E are
responsible for chronic signs of vasoconstriction,
haemoconcentration, cardiomyopathy and myocarditis.
 Neuropeptide Y may potentiate NE action and be
responsible for alpha blockade-resistant
phaeochromocytoma.
 In view of the diverse and unpredictable nature
of symptoms and the potentially lethal nature of
the disease, it is important to have a high index
of suspicion for ‘high risk’ individuals:
 Patients with episodic headache, tachycardia and
sweating, with or without hypertension
 Previous unexplained adverse cardiovascular
response to surgery/parturition/straining.
 Adverse cardiovascular response to histamine,
succinylcholine, metoclopramide.
 Patients with adrenal ‘incidentaloma’ and family h/o of
pheochromocytoma or MEN II syndrome.
 Hyperglycemia reflects a predominance of
alpha adrenergic activity (inhibitn of insulin
release, glycogenolysis)
 Enhanced coagulation may accompany
chronic catecholamine excess.
 Orthostatic hypotension – reflects
decreases in intravasc. Fluid volume
assoc. with sustained systemic HTN.
 Haematocrit > 45% - hypovolemia caused
by chronic increased in systemic BP
 Diagnosis
 Biochemical
 Accurate tools like High Performance Liquid
Chromatography (HPLC) are now available
for plasma catecholamine estimation.
However plasma catecholamines suffer from
several drawbacks. Their sensitivity is low
(84%) as discussed earlier.
 Measurement of free norepinephrine in a 24
hr urine collection provides a more sensitive
index than the metabolites
(VMA,metanephrines,normetanephrines)
 Clonidine suppression test
 Radiological localization
 Both CT and MRI have high sensitivity and
specificity and can detect tumours <2 cm in
size.
 131 Meta Iodo Benzyl Guanidine (MIBG) scan
is performed routinely on all
pheochromocytoma patients to detect
multicentricity and metastatic disease.
 The newer modalities are PET scan using
11C hydroxyephedrine and octreotide scans
for paragangliomas.
 ECG – for LVH and non specific T wave
changes.
 Chest radiograph may reveal
cardiomegaly.
 Treatment
 Surgical excision – definitive treatment
 Before Surgery , its important to establish
alpha blockade
 To stabilize systemic BP
 To expand intravascular volume
 To normalize myocardial performance
 Most studies recommend patients receive
alpha blockers usually phenoxybenzamine
10-20 mg PO twice daily.
 Most patients require 80-200 mg /day
 α adrenergic blockade – attenuates or prevents
catecholamine induced HTN.
 Return to normotension facilitates increase in
intravascular fluid volume, as reflected by
decreases in hematocrit.
 Persistence of tachycardia or cardiac
dysrythmias despite presence of α blockade –
indication for β blockade.
 Propranolol 40mg PO twice daily can be used.
 Avoided in pts. with cardiomyopathy – may
precipitate heart failure.
 α-methyltyrosine inhibits enzyme tryrosine
hydroxylase – rate limiting step in catecholamine
biosynthesis.
 To date, there is no study for optimal preop
duration of phenoxybenzamine therapy. Most
pts. require treatment for 10-14 days.
 The following criteria for an optimal pre-op
condition are recommended:
 No “ in hospital” BP reading > 160/90 mm Hg should
be evident for 24hrs before Sx.
 Orthostatic hypotension with readings above 80/45
mm Hg should be present.
 ECG should be free of ST-T abn. for atleast a week. If
abn. 2D-ECHO should reveal no evidence of RWMA
or Global myocardial abn. that cannot be attributed to
a permanent deficit.
 The patient should have no more than 1 VPC every
5mins.
Drugs used in pheochromocytoma
 Alpha blockers – Phentolamine, prazosin,
phenoxybenzamine
 Beta blockers – propanolol, esmolol,
atenolol
 Labetalol
 Vasodilators – Nitroprusside, magnesium
sulfate
 Ca channel blockers –Diltiazem
,nicardipine
 Anesthetic Management
 Preoperative preparation
 The aim of pre operative preparation is to provide
symptom control and cardiovascular stability.
 Continuation of α blockers until the day of Sx is
recommended.
 β blockers should also be continued until the day of
Sx.
 The times of significant intraoperative hazard to the
patients.
• During tracheal intubation
• During manipulation of tumour
• After ligation of tumour’s venous drainage
 Premedication
 Reassurance and familiarity with the patient
are the best premedicants.
 Benzodiazepines can be given at night.
Scopolamine can also be with BZDs
 If B/L adrenalectomy is anticipated,
supplemental cortisol T/t may be instituted at
the same time as preoperative medication.
 Sites for arterial and CVP cannulation are
inspected and the patient is explained and
prepared mentally about insertion of cannulae
under local anesthesia.
 Intraoperative Management
 Anesthetic, vasopressors and vasodilators
should be all prepared and available before
induction of anesthesia.
 Routine Monitoring + invasive BP recording
should be available, placement of a PA
catheter is useful for monitoring of
intravascular fluid volume.
 Induction: often with iv administratn of
barbiturate, etomidate or propofol.
 Depth of anesthesia increased with nitrous
oxide + volatile anesthetics.
 Choice of volatiles based on ability of drug to
decrease sympath. Nervous system activity.
 Isoflurane is very widely used. However sevo
and des also tried.
 Avoid histamine releasing NDMR
 Scoline is avoided.
 Laryngoscopy and intubation response can be
minimized with IV lignocaine 1-2 mg/kg IV.
 Adequate depth of anesthesia is ensured before
attempting intubation.
 Maintenance of Anesthesia
 Nitrous oxide + volatile anesthetics
 Continous iv infusion of nitroprusside may be
necessary, if HTN persists despite delivery of
maximal conc. of volatile anesthetic drugs
(1.5- 2.0 MAC)
 Reflex tachycardia accompanying SNP can
be treated with continuous infusion of
esmolol.
 Once veins draining tumour are ligated,
hypotension can occur
 Once veins draining tumour are ligated, hypotension can
occur.
 Hypotension is multifactorial in origin- residual a
blockade, suppression of contralateral adrenal function,
desensitization of a2 receptors, loss of elastance of
arterioles and volume loss have all been implicated.
implicated
 Treated by
 Decreasing the conc. of volatiles
 Rapid IV infusions of crystalloids and/or colloids
 Rarely , a continuous infusion of phenylephrine or
norepinephrine is required until SVR adapts to decreased levels
of endogenous alpha adrenergic stimulation.
 Monitoring ABG, blood glucose conc. and electrolytes is
recommended intraoperatively.
 Points to consider during laparoscopic
excision of phaeochromocytoma
 Positioning:
 Phaeochromocytomas can be excised through the
transperitoneal (supine or lateral) or the
retroperitoneoscopic (lateral or prone) techniques.
 Whatever the position, IV access should be
satisfactory. The internal jugular catheter may need to
be placed on the ipsilateral side to avoid kinking and
allow free access to the central circulation for
administration of vaso active drugs.
 For posterior retroperitoneoscopic procedures, the
prone position is necessary. The endotracheal tube
should shoud be fixed well. The eyes and pressure
points should be padded and protected. The
abdomen should be free to move and airway
pressures kept under constant check.
 Intra operative haemodynamic events
 Pneumoperitoneum- increases systemic vascular
resistance, mean arterial pressure and induce
catecholamine release, all of which could theoretically
be exaggerated in the presence of
pheochromocytoma.
 Absorption of increasing amounts of CO2 from the
retroperitoneal/ peritoneal space and its
consequences has also been a contentious issue .
However, these fears are largely unfounded,
especially if insufflation pressures are kept at 8-12
mm Hg.
 Tumour manipulation during laparoscopic surgery is
invariably associated with short lived but steep rises
in blood pressure.
 Postoperative management
 Invasive monitoring continued in
postoperative period.
 Persistent Hypotension refractory to volume
replacement is a common complication.
 Persistent hypertension
 Hypoglycemia should be looked for.
 Neuraxial opioids for postop pain relief.
 Regional Anesthesia
 Can be used for excision of
pheochromocytoma
 Despite sympathetic blockade, post synaptic
alpha receptors still respond to direct effects
of sudden increase in catecholamines
 Disadvantages
• Absence of sympathetic activity, if hypotension
follows vascular isolation of tumour
• Also ability to maintain spontaneous ventilation
during intra-abdominal manipulation and retraction.
 Regional anesthesia is practical if only in
supine position.
Thank You