the patient with endocrine disease

PERIOPERATIVE MANAGEMENT OF CO-MORBID CONDITIONS

The patient with endocrinediseaseAmi Jones

Sue Catling

AbstractEndocrine diseases are increasingly common and are often either the

primary indication for surgery or coexist in patients undergoing unrelated

surgical procedures. Surgeons should have a clear understanding of the

problems that the patient with endocrine disease encounters during

the perioperative period and how to manage any related emergencies

that may occur. This article covers the perioperative management of dia-

betic, thyroid and adrenal disease, including preoperative preparation

and postoperative complications. It also discusses the emergency

management of related endocrinological crises that may occur in patients

with undiagnosed or diagnosed endocrine disease.

Keywords Adrenal; diabetes; endocrine; perioperative; phaeochromo-

cytoma; thyroid

Introduction

The endocrine system comprises a series of specialized tissues

that coordinate the synthesis and release of hormones. Endocrine

disease occurs when there is over- or under-production of

hormones. It is important that the surgeon has a sound under-

standing of the various different types of disease, either because

it is the primary indication for surgery, or because it coexists in

patients undergoing unrelated procedures. Such conditions can

have significant impact on the perioperative period, so thorough

preoperative investigation and optimization are vital.

Diabetes mellitus

The term ‘diabetes mellitus’ refers to the polyuria that often

occurs when the condition is untreated. It is derived from the

Greek verb diabaı́nein (diabaί�ei�), meaning ‘to stand with legs

asunder’, from which the word for a siphon (diabήth2) is

derived, and the Greek word for honey. Diabetes is characterized

by hyperglycaemia that in the long term is complicated by vas-

culopathy leading to retinopathy, neuropathy, nephropathy,

ischaemic heart disease, peripheral vascular disease and stroke.

There are currently some 171 million people with diabetes

Ami Jones BM BS BMedSci FRCA EDIC is a Specialist Registrar in Anaesthesia

and Intensive Care Medicine on the Welsh School of Anaesthesia

Rotation, UK. Conflicts of interest: none declared.

Sue Catling BA MB BS FRCA is a Consultant Anaesthetist at Singleton

Hospital, Swansea, UK and is an examiner for the Primary FRCA

Examination. Conflicts of interest: none declared.

SURGERY 28:9 446

worldwide, a number the World Health Organization (WHO)

predicts will have more than doubled by 2030.1

Diagnosis

The WHO diagnostic criteria for diabetes is a fasting blood

glucose >7 mmol/litre or a blood glucose of >11.1 mmol/litre

after ingestion of 75 g glucose. There are further diagnoses

related to intermediate hyperglycaemia known as impaired

glucose tolerance (IGT) and impaired fasting glucose (IFG) which

require a glucose tolerance test (GTT) but they are beyond the

scope of this article.

Patients who give a history of thirst, polyuria or weight loss

and those with slow-healing wounds should be investigated for

diabetes. In children, the first presentation of the disorder may be

acute abdominal pain. Patients over the age of 40 undergoing

major surgery should also have a random glucose performed.

In known diabetics, measuring levels of glycated haemoglobin

(HbA1c) will indicate adequacy of glycaemic control over the

previous 8 weeks. HbA1c <6.5% indicates excellent control,

6.5e8%indicatesadequate control and>8%indicatespoor control.

The optimal level at which blood glucose should be main-

tained has been the subject of several recent studies. In 2001

a prospective randomized controlled trial showed that mainte-

nance of blood glucose at 4.4e6.1 mmol/litre by intensive insulin

therapy reduced mortality in critically ill surgical patients to

4.6% compared with 8% in those treated with conventional

insulin regimes who had blood glucose levels 10.0e11.1 mmol/

litre.2 This trial, known as Leuven-1 e after the hospital in which

it was performed e influenced practice for several years, and

‘tight glycaemic control’ became a therapeutic goal. In 2009 this

was refuted by the NICE-SUGAR trial, which enrolled 6000

intensive care patients and was the largest study of its type

undertaken.3 It showed that tight glycaemic control increased

mortality from 24.9% to 27.5%. In particular, this trial showed

that hypoglycaemia was increased two- to threefold with inten-

sive insulin regimes. Target glucose levels in the surgical patient

therefore should be 5e10 mmol/litre, to avoid both hypo-

glycaemia and hyperglycaemia.

Classification and treatment

Type I diabetes (insulin-dependent diabetes) tends to present

within the first three decades of life and is caused by autoim-

mune destruction of the pancreatic islets of Langerhans. Patients

require subcutaneous insulin therapy, which is typically

provided as a combination of both short- and long-acting prep-

arations. Subcutaneous continuous infusions are also becoming

more widely used, especially in younger patients.

Type II diabetes (non-insulin-dependent diabetes) presents later

in life, although with increasing levels of obesity in childhood it

is being seen at younger ages. It is associated with reduced

insulin production and insulin resistance. Although dietary

modification and oral hypoglycaemic drugs (Table 1) can be used

to manage the majority of patients, around a third will eventually

require insulin.

Perioperative management

Ensure that diabetic patients are first on the operating list and

that the anaesthetist is aware of them. Perioperative management

� 2010 Elsevier Ltd. All rights reserved.

http://dx.doi.org/10.1016/j.mpsur.2010.05.003

Oral hypoglycaemic drugs and their actions

Biguanides

(e.g. metformin)

Enhance the peripheral effects of insulin by increasing the number of low-affinity binding sites for insulin in red cells,

adipocytes, hepatocytes and skeletal muscle cells, and have no effect in the absence of circulating insulin.

Intestinal absorption of glucose is inhibited and glucose utilization is decreased by decreased gluconeogenesis and

increased anaerobic glycolosis.

Metformin is frequently used in obese patients and is fast acting. It does not cause hypoglycaemia but can be associated

with a profound lactic acidosis. It is excreted unchanged in the urine and therefore should be used in caution in patients

with renal impairment.

Sulphonylureas

(e.g. gliclazide)

Act on the pancreatic beta cells to stimulate insulin secretion and can cause hypoglycaemia, and are associated with

numerous drug interaction problems due to their high protein binding.

Glitazones

(e.g. rosiglitazone)

Bind to nuclear receptors in adipose, muscle and liver cells promoting gene transcription of insulin signalling pathways,

which enhances the effectiveness of endogenous insulin by increasing glucose uptake.

Table 1


will be determined by consideration of the patient’s usual

diabetic medication, the duration of preoperative fasting, the

magnitude of surgery proposed and how quickly normal diet will

be established postoperatively.

Diet-controlled diabetics should be fasted as normal and have

regular monitoring of blood glucose. They are not at risk of

hypoglycaemia but hyperglycaemia may require medical

intervention.

Patients taking oral hypoglycaemics may be at risk of hypo-

glycaemia during perioperative fasting, particularly if they are

taking long-acting preparations. Patients undergoing minor

surgery should omit their usual diabetic medication on the

morning of surgery and have regular blood glucose monitoring

until eating and drinking. Patients undergoing major surgery

should omit their morning diabetic medication and be placed

onto an insulin sliding scale/glucose infusion. Usual diabetic

medications can be restarted postoperatively once the patient is

eating and drinking.

Insulin-dependent diabetics can undergo minor surgery with

omission of insulin until after the procedure and a non-

Continuous insulin and glucose infusion

1. At the time of set-up, measure blood glucose.

2. Separate infusions of glucose and insulin should run into a single ve

3. Prescribe and administer 1 litre of 5% glucose (with 27 mmol KCl) at

4. Prescribe 50 units of soluble insulin (Actrapid) in 50 ml 0.9% NaCl a

5. Titrate this between 0.5 and 10 units/hour as described below.

6. Aim to keep blood glucose level between 5 and 10 mmol/litre.

7. Check blood glucose level every 30e60 minutes before/during surger

blood glucose 5e10 mmol/litre. Leave at same rate.

blood glucose >10 mmol/litre. Increase rate by 1 unit/hour.

blood glucose <5 mmol//litre. Reduce rate by 0.5 units/hour and not

blood glucose <3 mmol/litre. Stop Actrapid for 30 minutes. Notify sup

8. Once the patient is eating and drinking, give normal dose of insulin/

9. The blood glucose and rate of insulin infusion should be recorded on

Box 1

SURGERY 28:9 447

carbonated glucose drink 2 hours prior to the procedure. Those

undergoing major surgery should be placed onto an insulin

sliding scale/glucose infusion. Most hospitals will have their

own protocol for insulin sliding scales e an example is given in

Box 1.

Diabetic emergencies

Diabetic ketoacidosis (DKA) is characterized by the triad of

hyperglycaemia, ketoacidaemia and fluid and electrolyte deple-

tion. DKA may be the initial presentation of type I diabetes, but

can also be triggered in a known diabetic by insults such as

sepsis and myocardial infarction. Patients typically present with

a history of polyuria and polydipsia and often exhibit Kussmaul’s

breathing (rapid and deep), smell of ketones and may have signs

of cardiovascular shock or reduced conscious level. Hyper-

kalaemia, which is worsened by acidosis, typically masks

depleted whole body potassium stores. Management includes

fluid administration, insulin and replacement of potassium and

phosphate along with careful blood glucose monitoring (Box 2).

An excessively rapid reduction in blood glucose can cause

nous cannula using anti-siphon and anti-reflux valves.

125 ml/hour.

nd start at 2 units/hour.

y (hourly after surgery) and if:

ify supervising doctor

ervising doctor. Restart at 1 unit/hour if blood glucose >5 mmol/litre.

tablets and discontinue intravenous glucose/insulin infusion.

the blood glucose chart on an hourly basis.



Emergency management of diabetic ketoacidosis4

C Assess airway, breathing, circulation (ABC) and conscious level

C Calculate degree of dehydration and commence fluid resuscitation with normal (0.9%) saline: 1 litre over 1 hour, 1 litre over 2 hours, 1 litre

over 4 hours, 1 litre over 6 hours, 1 litre every 8 hours to an average total of 6 litres in the first 24 hours.

C Give soluble insulin 0.1 U/kg as an intravenous bolus, followed by 0.1 U/kg/hour. Monitor glucose hourly, and potassium and pH hourly. Halve

insulin infusion rate if glucose concentration falls below 5 mmol/litre.

C Maintain serum potassium concentration at 3.3e5.5 mmol/litre by adding KCl 20 mmol to each litre of saline. If potassium <3.3 mmol/litre

give KCl 40 mmol over 1 hour. If potassium >5.5 mmol/litre omit supplemental KCl.

C Once glucose concentration is <11 mmol/litre switch from saline to 5% dextrose or dextrose saline.

C Phosphate concentration should be checked 6 hourly and supplemented as necessary.

Box 2


significant fluid shifts and put patients at risk of cerebral oedema.

In addition to managing the physiological and biochemical

derangements, an underlying cause (e.g. infection, infarction,

trauma, neglect) should be sought and treated.

Hyperosmolar non-ketotic (HONK) state or hypergylcaemic

hyperosmolar state (HHS) is less common than DKA and typi-

cally occurs in middle-aged or elderly type II diabetics. The blood

glucose concentration tends to be much higher than that seen in

DKA (typically >50 mmol/litre) but there is no accompanying

ketosis or acidosis. HONK is associated with severe dehydration

and can present with cardiovascular collapse or coma. Patients

require fluid resuscitation, correction of electrolyte abnormalities

and insulin therapy (Box 3). The mortality associated with HONK

can be as high as 30% and two-thirds of patients will be previ-

ously undiagnosed diabetics.

Thyroid disease

The thyroid gland is the largest endocrine organ. In response to

thyroid-stimulating hormone (TSH) from the pituitary gland, the

thyroid takes up iodine and produces two main hormones triio-

dothyronine (T3) and thyroxine (T4). T3 is the active form with

around 80% produced by deiodination of T4. Thyroid hormones

have actions upon growth, nervous system development and

metabolic rate. In addition they are involved in normal bone

turnover and are sympathomimetic. Abnormalities of thyroid

function (principally hypothyroidism) are often encountered in

patients presenting for elective and emergency surgery.

Enlargement of the thyroid by tumour or goitre has implications

Emergency management of hyperosmolar non-ketotic(HONK) state or hypergylcaemic hyperosmolar state(HHS)

C Treat as for diabetic ketoacidosis, but give dextrose saline if

sodium >150 mmol/litre

C Give insulin infusion 0.05 units/kg/hour

C High risk of thromboembolic disease e ensures prophylactic

anticoagulation (e.g. enoxaparin) given

Box 3

SURGERY 28:9 448

for airway management during anaesthesia and requires further

investigation.

Hyperthyroidism is characterized by a low TSH and high levels

of T3 and T4. Patients present with anxiety, heat intolerance,

palpitations and weight loss. Physical signs include tachycardia,

hypertension, lid lag, tremor and muscle weakness. The com-

monest causes are; autoimmune (Graves’) disease, toxic nodule

and a toxic multinodular goitre (Plummer’s disease). Other

causes, such as post-partum thyroiditis and viral thyroiditis, are

relatively rare. Therapeutic intervention includes control of

symptoms (beta blockade), drugs that interfere with hormone

production (prophylthiouracil, carbimazole), radioactive iodine

and thyroidectomy.

Hypothyroidism may be caused by pituitary disorders (reduced

TSH) or thyroid disease (elevated TSH). The onset may be

insidious and patients typically complain of fatigue, weight gain,

cold intolerance, constipation, hair loss and depression. The

diagnosis is often ‘missed’ and may only be made by exclusion in

the postoperative period. The commonest causes are autoim-

mune (Hashimoto’s) thyroiditis and iatrogenic e secondary to

drug therapy or surgical resection. Rarer causes include iodine

deficiency and congenital hypothyroidism (cretinism). Replace-

ment therapy with thyroxine and intermittent measurement of

TSH are the cornerstones of management.


Patients with treated thyroid disease should undergo thyroid

function tests (TSH, T4) prior to surgery. Stable patients, who

have had a satisfactory test results in the previous 6e12 months,

are unlikely to benefit from re-testing. Symptoms of hypo/

hyperthyroidism should prompt preoperative testing, particularly

in patients not known to have thyroid disease.

To ensure optimal pharmacological control, the opinion of an

endocrinologist should be sought when a patient is due to

undergo thyroid surgery for uncontrolled hyperthyroidism.

Patients with a palpable goitre should have further imaging of

their neck with a computed tomography (CT) scan (Figure 1) as

there may be significant compression and distortion of the

trachea which may make intubation very difficult.

Following thyroidectomy, recurrent laryngeal nerve damage

or tracheal collapse (tracheomalacia) may cause stridor or

airway obstruction. Stridor secondary to acute haemorrhage and



Figure 1 Computed tomogram of cervical region showing severe tracheal

compression (arrow) by very enlarged thyroid gland.5


haematoma formation is caused by venous congestion and

tracheal mucosal oedema rather than external tracheal

compression. Ideally, patients with a very large thyroid or those

with significant tracheal involvement should be managed in

a high dependency or intensive care unit. Hypocalaemia,

secondary to damage or removal of the parathyroid glands, may

lead to acute laryngospasm. Serum calcium concentration should

be checked on the day after surgery and replacement therapy

instituted where appropriate.

Thyroid emergencies

Thyrotoxic crisis tends to occur during episodes of serious

illness or at the time of thyroid surgery, although it can occur

independently of this. Clinically it manifests as hyperpyrexia,

tachycardia (often with atrial fibrillation), dehydration, diar-

rhoea, tremor and delirium and is associated with a very high

mortality. Rapid diagnosis is essential, as it may easily be

confused with sepsis, malignant hyperpyrexia and drug over-

dose. Emergency management is summarized in Box 4.

Emergency management of thyrotoxic crisis4

C Check airway, breathing, circulation (ABC), administer 100% oxygen.

C Rehydrate with intravenous (IV) normal saline and glucose (may have

C Treat hyperpyrexia with passive/active methods and paracetamol. Do

C IV propanolol in 1 mg increments up to 10 mg or esmolol 250e500 mg

cardiovascular monitoring aiming to reduce pulse to <90 beats/minut

C Hydrocortisone 200 mg IV bolus followed by 50 mg IV 6-hourly to tre

C Dantrolene sodium can be used in an effort to inhibit pathological me

10 mg/kg (average 3 mg/kg required).

C Propylthiouracil, 1 g loading dose via nasogastric tube followed by 20

C Following propylthiouracil, IV sodium iodide 500 mg 8-hourly or potas

five to ten drops 6-hourly via nasogastric tube.

Box 4

SURGERY 28:9 449

Myxoedemal coma is the end stage of untreated or inadequately

treated hypothyroidism. It can be precipitated by a number of

conditions including sepsis, myocardial infarction, gastrointes-

tinal haemorrhage and trauma. Patients present with hypo-

thermia, hypotension, reduced conscious level and the

complications of water retention e pleural and pericardial effu-

sions and hyponatraemia. Typically levels of T4 are low and TSH

high. Pituitary hypothyroidism should be considered if TSH is

low or normal. It should be borne in mind that hypothyroidism

may mask panhypopituitarism. Management is summarized in

Box 5.

Adrenal disorders

The adrenal glands are triangular shaped organs located on the

upper poles of the kidneys. Each is divided into an outer cortex

and an inner medulla, the latter secreting adrenaline and

noradrenaline under the control of the sympathetic nervous

system. The cortex is further subdivided into three layers. The

outermost layer, the zona glomerulosa, produces mineralocorti-

coids (mainly aldosterone). The middle layer, the zona fascicu-

late, produces glucocorticoids (mainly cortisol). The innermost

layer, the zona reticularis, is responsible for androgen produc-

tion. While primary adrenal failure (often haemorrhagic) and

hormone secreting tumours (e.g. adenoma, phaeochromocy-

toma) are uncommon, a significant number of patients will be

receiving glucocorticoids prior to surgery.


Stimulation of the hypothalamic-pituitary axis (HPA) and release

of adrenal hormones is a normal and vital response to stress,

trauma and surgery. Patients with adrenal insufficiency and

those on long-term steroid therapy are at risk of a perioperative

hypoadrenal (Addisonian) crisis as a result of chronic HPA

suppression. While it is possible to assess HPA function by

measuring cortisol and the response to synthetic adrenocortico-

tropic hormone (ACTH) administration, it is usually simpler to

treat patients at risk with glucocorticoids during the periopera-

tive period.

In an adult, cortisol secretion is in the order of 50 mg/day

following minor surgery and up to 150 mg/day following

large fluid and glycogen store losses).

not give aspirin or non-steroidal anti-inflammatory drugs.

/kg bolus followed by continuous infusion of 50e100 mg/kg/hour with

e

at adrenal insufficiency and reduce thyroid hormone release.

chanisms occurring in sarcoplasmic reticulum, 1 mg/kg boluses up to

0 mg 6-hourly, to reduce thyroid hormone release.

sium iodide five drops 6-hourly via nasogastric tube or Lugols iodine



Emergency management of myxoedemal coma4

C Assess airway, breathing, circulation (ABC) and conscious level, tracheal intubation and mechanical ventilation may be required if respiratory

effort is inadequate.

C Immediate intravenous (IV) thyroid hormone replacement with cardiovascular monitoring. Load with 500e800 mg of IV levothyroxine followed

by 50e100 mg/day. Consider reduced dose in the elderly or those with significant cardiovascular disease as treatment may induce cardiac

ischaemia.

C Rewarm passively if possible, however patient with cardiovascular instability may require active rewarming.

C Fluid resuscitation if hypotensive. Inotropes and vasopressors are occasionally required.

C Where possible, fluid restrict if hyponatraemic. Severe hyponatraemia or seizures may require treatment with hypertonic saline.

C Avoid sedatives where possible as metabolism may be significantly slowed.

Box 5


major surgery. The extent and duration of elevated cortisol

secretion corresponds to both the magnitude of surgery and

the length of recovery. Steroid replacement therapy should,

therefore, be tailored to both the patient and the surgical

procedure. Patients who have received a regular daily dose of

more than 10 mg prednisolone (or equivalent e see Table 2)

in the 3 months prior to surgery should be deemed to be ‘at

risk’. Patients taking lower doses of prednisolone have been

shown experimentally to have clinically normal HPA

responsiveness.

It is recommended that patients ‘at risk’ undergoing minor

surgery receive a single dose of intravenous hydrocortisone

(25 mg) at induction of anaesthesia. For moderate surgery (e.g.

hysterectomy, major joint replacement) patients should take

their usual steroid dose before surgery, receive hydrocortisone

(25 mg) on induction and a further dose of hydrocortisone (100

mg) on the day after surgery, and resume their normal pre-

operative steroid.

For major surgery (thoracotomy, cardiac surgery, surgery

causing prolonged gastrointestinal dysfunction) it is recommended

that patients take their usual steroids preoperatively, then receive

hydrocortisone (25 mg) at induction of anaesthesia and hydrocor-

tisone (25 mg 6-hourly) for 2e3 days, and resume normal oral

therapy once normal gastrointestinal function has returned.

Acute adrenal insufficiency

Emergency management of hypoadrenal crisis4

This syndrome typically occurs in patients with chronic

adrenal insufficiency. Primary causes include autoimmune

adrenal destruction, malignancy, tuberculosis and acute hae-

morrhage. Secondary causes include hypothalamic and pitui-

tary disease. It can be precipitated by sepsis and trauma, by

Equivalent corticosteroid doses6

Prednisolone 10 mg z Betamethasone 1.5 mg

Cortisone acetate 50 mg

Dexamethasone 1.5 mg

Hydrocortisone 40 mg

Deflazacort 12 mg

Methylprednisolone 8 mg

Table 2

SURGERY 28:9 450

acute steroid withdrawal in a patient on long-term steroids or

by drugs that increase steroid metabolism or reduce steroid

synthesis.

Patients present with hypotension and shock, dehydration

and hyperpyrexia, and may complain of abdominal pain. The

classical biochemical picture is that of hyponatraemia and

hyperkalaemia with cortisol levels lower than expected during

a period of stress. A short ACTH stimulation (Synacthen) test

aids diagnosis but acute treatment (Box 6) should not be delayed

until the results become available. In addition to mineralocorti-

coid and glucocorticoid administration, any underlying precipi-

tating illness should be identified and treated.

Phaeochromocytoma

The word phaeochromocytoma in Greek means ‘dusky coloured

tumour’ referring to the colour which it turns when stained with

chromium salts. It is a tumour of chromaffin cells within the

adrenal medulla that secrete excessive amounts of noradrenaline,

adrenaline and, rarely, dopamine. Approximately 20% of

tumours are bilateral, 10% are malignant and approximately

15% are found outside the adrenal gland, typically in the

abdomen. As many as 25% may occur in the setting of type II

multiple endocrine neoplasia.

The classical presentation is episodic attacks of palpitations,

anxiety, sweating and headaches, although the clinical picture

correlates with the relative proportions of adrenaline or

C Assess airway, breathing, circulation (ABC), administer 100%

oxygen.

C Intravenous (IV) fluid resuscitation to replace blood volume,

sodium deficit and correct hypoglycaemia.

C Hydrocortisone 200 mg IV bolus followed by 100 mg IV

6-hourly.

C Vasoactive drugs as required. Note that these drugs may be

less effective or ineffective if cortisol replacement is

inadequate.

Box 6



Emergency management of undiagnosed phaeochromocytoma4

C Assess airway, breathing, circulation (ABC), administer 100% oxygen.

C Phentolamine (alpha blocker) 1e2 mg increments up to 20 mg with invasive cardiovascular monitoring to control hypertension, titrate to

effect.

C Magnesium sulphate can be given in addition as a slow intravenous (IV) bolus dose of 5 g (20 mmol) followed by 2 g/hour by infusion to

achieve therapeutic magnesium levels of 1.5 mmol/litre.

C If heart rate >100 beats/minute despite alpha blockade or frequent ventricular ectopic beats, give labetolol 5e10 mg increments IV.

C Further tachyarrhythmias should be treated with esmolol boluses of 1.5 mg/kg.

C Ongoing hypertension can be treated with infusion of sodium nitroprusside starting at 0.5e1.5 mg/kg/minute and increasing to 3e5 mg/kg/

minute.

Box 7


noradrenaline secreted. In adrenaline secreting tumours, beta

adrenergic effects (i.e. hypertension and tachycardia) predomi-

nate, whereas in noradrenaline secreting tumours alpha adren-

ergic effects (i.e. hypertension and reflex bradycardia) occur. A

dopamine-secreting tumour typically causes nausea and vomit-

ing. Untreated cases can present with cardiac failure due to

a chronically increased systemic vascular resistance, hyper-

glycaemia and stroke secondary to cerebral haemorrhage.

No single test is diagnostic test as catecholamine levels may

not be elevated at the time of blood/urine testing. Free cate-

cholamine measurement from a 24-hour urine collection, plasma

or urine catecholamine, urinary vanillyl mandelic acid (VMA)

levels or a clonidine suppression test can all be used to confirm

biochemical abnormalities. Localization of the tumour with CT

or magnetic resonance imaging (MRI) will suffice in most cases.


Perioperative mortality in phaeochromocytoma is approximately

2e3%.Aspecialistmultidisciplinary teamof experienced surgeons,

anaesthetists and endocrinologists therefore best manages preop-

erative optimization of the patient. Patients are typically started on

alpha blockers (e.g. phenoxybenzamine 30 mg 12-hourly or dox-

azosin 16mgdaily) in advance of any surgical intervention to lower

blood pressure, allow a compensatory increase in intravascular

volume and to reduce the chances of intraoperative hypertensive

crisis. Once alpha blockade is achieved, a beta blocker is added to

control tachycardia. It is essential that beta blockers are not given

before alpha blockade is achieved, as worsened hypertension and

myocardial failure can occur.

Handling of the tumour during surgery can cause release of

enormous amounts of catecholamine, as can pneumoperitoneum

in laparoscopic cases and adrenal vein ligation. It is essential that

the anaesthetist be alerted prior to these events so that they are

ready to counteract any surges in blood pressure. Following

adrenal vein ligation there is often a sudden fall in catecholamine

levels that may result in a refractory hypotension. Again, the

SURGERY 28:9 451

anaesthetist should be alerted in good time that ligation is soon

to occur. It should be borne in mind that acute cardiovascular

collapse in the setting of left ventricular hypertrophy, hypo-

volaemia and reduced afterload may be caused by dynamic left

ventricular outflow tract obstruction. Transoesophageal echo-

cardiography allows rapid diagnosis and aids management,

whereas traditional haemodynamic monitoring invariably

prompts (inappropriate) diuretic, vasodilator and inotrope

administration.

Patients are typically managed in the high dependency or

intensive care unit after surgery as they may require significant

fluid resuscitation and cardiovascular support. Patients under-

going bilateral adrenalectomy require life-long steroid replace-

ment therapy. A summary of emergency management is shown

in Box 7. A

REFERENCES

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4 Allman KG, McIndoe A, Wilson IH, eds. Emergencies in anaesthesia.

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5 Monkhouse S, Skene A. Acute airway obstruction due to metastatic

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ispub.com/journal/the_internet_journal_of_urology/volume_4_

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the patient with endocrine disease

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