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444
Cryopreservation of parathyroid glands
• Patients who became a parathyroid after re-explorations
specially in MEN syndrome, are candidates for cryopreservation.
• Here 200 mg of cut parathyroids tissue is frozen in
solutions containing 10% dimethyl sulfoxide, 10%
autologous serum and 80% Weymouth solution.
Exploration of the neck and parathyroidectomy
• Preoperative localisation of gland is preferable.
• Rapid intraoperative PTH measurement after minimally
invasive parathyroidectomy should be done.
• Fall in PTH is highly indicating of the success.
• More often it is a single adenoma—it is removed and
rest normal glands are left undisturbed.
• If one cannot identify parathyroids glands, look for
ectopic glands—posterior and deep to thyroid gland, in
the tracheoesophageal grooves, posterior to inferior
thyroid vessels between carotid artery and oesophagus.
Manipal Manual of Surgery
3. Carcinoma: All four glands should be removed
along with ipsilateral thyroid lobe with modified
radical neck dissection in the presence of lymph
node metastasis (Key Box
38.9)—radical para-
thyroidectomy.
Key Box 38.9
Preoperatively Suspect
Parathyroid Carcinoma when
Severe symptoms
Serum calcium levels greater than 14 mg/dl
Significantly elevated PTH levels
Swelling of parathyroid gland.
Follow-up
• Estimation of calcium should be done in the
postoperative period to assess the functioning of the
parathyroid tissue. Very often, after surgery for
adenoma, there is a sudden drop in the levels of
calcium because of absorption of the calcium by the
bones. This is described as ‘hungry bone syndrome’.
This is seen in patients who have generalised bone
disease. In one of our patients, calcium levels
dropped down to 4 mg% with severe tetany. It
took 7–10 days for it to return to the normal levels.
She required 24
hr constant infusion of calcium
(Fig. 38.12).
• Absorption of calcium can be enhanced by oral
administration of 1,25-dihydroxycholecalciferol,
which is the most active metabolite of vitamin D.
Vitamin D stimulates the absorption of calcium and
Section II • General Surgery
phosphate.
Fig. 38.12: Hypercalcaemia responding after excision of
parathyroid adenoma
RECENT ADVANCES IN PARATHYROID SURGERY
Cer
vicoscopy
1. Minimally invasive endoscopic parathyroidectomy
(MIP)
• This is possible today because of localisation by
ultrasound/sestamibi combined with SPECT
• Popularised by Gagner
• Indicated for a single adenoma
• Can be done under general anaesthesia or
regional—cervical block by using xylocaine (1%)
with adrenaline
• 3–4 trocars are required
insufflation is required
• CO
2
2. Video-assisted parathyroidectomy—minimally
invasive
• It is popularised by Parlo Miccoli from Italy
• Ideal for a single adenoma without thyromegaly
• No previous neck surgeries
• It can be done without CO2 insufflation—
gasless
• Bleeding, recurrent laryngeal nerve injuries are not
uncommon
• Can be done for bilateral cases
• No trocars, a small 2–3 cm incision
3. Totally endoscopic parathyroidectomy by lateral
approach by Henry, et al. It is the choice for
adenoma deeper to thyroid gland. Not for bilateral
cases. 10 mm endoscope is required. Require CO
insufflation.
• Refer to Key Box 38.10 and clinical notes.
2
Parathyroid and Adrenals
445
Key Box 38.10
Primary Hyperparathyroidism
(PHPT)—Interesting ‘Most’
Mostly it is sporadic.
Mostly it is due to single adenoma.
Most abundant cation in the body is calcium.
Most common and earliest manifestation of MEN
type I is PHPT.
Most common presentation of PHPT is asymptomatic
hypercalcaemia.
Most widely used and accurate investigation for
localising parathyroid gland is sestamibi scan.
Most commonly done surgery for PHPT is excision of
adenoma.
A 20-year-old boy attended dental outpatient department
for a loose tooth. He was diagnosed to have a loose tooth
and a cyst in the lower jaw as confirmed by X-ray. Patient
also had genu valgus deformity with backache (Fig. 20.5).
An ortho opinion was sought for. Investigations revealed
high calcium levels. An ultrasound neck revealed a
parathyroid adenoma of 6 cm. At exploration a single
adenoma was found arising from right inferior parathyroid
and was excised. Incidentally, X-ray abdomen also
revealed bilateral nephrocalcinosis. This case illustration
has been given to highlight the various presentations of
hyperparathyroidism. High degree of suspicion is
necessary for a clinician to consider the possibility of
hyperparathyroidism.
Treatment
1. Restore fluid volume urgently: 300 to 500 ml/hr of
0.9% sodium chloride (normal saline) 5 to 10 L/day
IV should be given. It not only helps in volume
restoration but also helps in renal excretion of
calcium. Inj. furosemide (80 to 100 ml) can be given
after volume restoration.
2. Bisphosphonates—disodium pamidronate slow
15–60 mg single IV infusion or over 2–4 days.
Maximum dose is about 90 mg. This drug stops
mobilisation of calcium from the bone.
3. Mithramycin: It is produced by Actinomyces, Strepto-
myces plicatus. Given in high doses of 20–25 µg/kg/
IV over 4 to
6 hours/day for 3 to 4 days. Can have
beneficial effects. However, effect seems to be temporary. Used in all cases of malignant hypercalcaemia.
Avoid thiazide diuretics. Cases which do not respond
to corticosteroids can be treated with mithramycin.
• Mithramycin rapidly controls hypercalcaemia
of malignant disease in most of the patients.
This control sometimes is temporary, and
intermittent administration of the antibiotic was
required.
. Steroids (in cases of vitamin D intoxication and
4
sarcoidosis).
5. Gallium nitrate therapy is highly effective and
superior to maximally approved doses of calcitonin
for acute control of cancer-related hypercalcaemia.
It inhibits bone resorption increasing calcium containing bone in majority of patients.
ACUTE HYPERCALCAEMIC CRISIS
• Also called parathyroid crisis.
• It presents with severe abdominal pain and effortless
vomiting. Dehydration, oliguria, renal failure follow
soon. Drowsiness and confusion are other features.
• Untreated cases develop coma and cardiac arrest.
• Profound muscular weakness is characteristic.
Causes
1
. Hyperparathyroidism
• Sudden increase in PTH levels occur due to
spontaneous bleeding in a parathyroid tumour or
rupture of a cystic parathyroid tumour. Severe
dehydration also precipitates a crisis.
2. Disseminated carcinoma with bony metastasis
(usually from carcinoma of the breast).
3. Parathyroid carcinoma.
Investigations
• Hypercalcaemia (16 to 20 mg/dl)
• Azotemia abnormal high level of urea, cratinine, etc.
CALCIPHYLAXIS
Etiopathogenesis
It is also called calcific uremic arteriolopathy. It is seen
in end stage kidney disease patients or patients with
secondary hyperparathyroidism who are undergoing
parathyroidectomy. A few other conditions which can
predispose to calciphylaxis are inflammatory bowel
diseases, autoimmune diseases, etc. Obesity, diabetes,
corticosteroids, chronic liver disease are a few risk
factors. It occurs due to extensive disseminated calcification of vessels and skin necrosis.
Clinical Features
Patients develop expanding painful cutaneous lesions
which are purpuritic. Extremities and areas wherein fat
contents are more such as breast, buttocks and
abdominal wall, gets affected often. Due to high levels
of calcification, ischemic necrosis occurs, and it results
in gangrene. Once gangrene sets in sepsis and death
occur.
Section II • General Surgery
446
Manipal Manual of Surgery
Diagnosis
It is based on the clinical features and high levels of
calcium, phosphate and with or without high levels of
PTH.
Treatment
Renal transplantation is the definite treatment for
secondary hyperparathyroidism. A few drugs used to
treat this are calcimimetic drugs. They reduce the
stimulation of parathyroid glands and thus lower the
PTH levels. If it is associated with persistent high levels
of PTH—more than 500 pg/ml total parathyroidectomy
with autograft in the nondominant forearm brachioradialis muscle or subtotal parathyroidectomy wherein
three and half parathyroid are removed.
ADRENAL GLANDS/SUPRARENAL GLANDS
SU23.1: Describe applied anatomy of adrenal glands.
Anatomy
• Weight of a normal gland is 4 g.
• Yellowish in colour.
• Surrounded by perinephric pad of fat.
• Right side Left side
Pyramidal in shape
Crescent shaped
More apical More medial to superior half
of the kidney
Anterolateral to Related to spleen, tail of
right crus pancreas and left crus of
diaphragm
• Parts: Two parts—cortex and medulla.
Blood Supply
• Superior suprarenal arteries arise from inferior
phrenic arteries.
• Middle suprarenal arteries arise from abdominal aorta.
• Inferior suprarenal arteries arise from renal artery.
• Suprarenal arteries branch before entering the
gland so that 50 to 60 arteries penetrate the capsule.
• Venous drainage is through a single large supra-
renal vein. On the right side, it is short and drains
into IVC. On the left side, it is longer and joined by
inferior phrenic vein and empties into left renal vein.
Surgical Importance
One has to clearly identify and ligate suprarenal vein
especially on the right side because of its short course.
Avulsion of right suprarenal vein can be catastrophic.
Physiology (Table 38.1)
Table 38.1 Histology and function
Cortex Hormone Functions
1.Cells of zona glomerulosa • Aldosterone • Regulates sodium and potassium haemostasis
2.
Cells of zona fasciculata and • Cortisol • Gluconeogenesis
reticularis
Medulla • Adrenaline • Activation of CVS
Cortex
• Derived from mesoderm
• Secretes corticosteroids, androgens, aldosterone.
Medulla
(corticosteroids) • Lipolysis, fat distribution, immunological response
(epinephrine) • Bronchodilatation
• Noradrenaline • Increases BP
(norepinephrine) • Splanchnic vasoconstriction
• Dopamine • Vasodilatation in skeletal muscle
• Increases heart rate
• Chromaffin cells of the medulla are related to
sympathetic system.
• These cells secrete catecholamines (mostly epine-
phrine).
• Derived from neural crest cells.
• It is a mass of nervous tissue associated with
Section II • General Surgery
sympathetic system.
Lymphatics
Drain into caval or aortic lymph nodes.
Parathyroid and Adrenals
SU23.2: Describe the aetiology, clinical features and
principles of management of disorders of adrenal gland.
DISORDERS OF ADRENAL CORTEX
447
Classification
• ACTH—dependent (70% pituitary or ectopic ACTH
producing tumours).
• ACTH—independent: Adrenal adenoma or hyper-
plasia.
PRIMARY HYPERALDOSTERONISM (PHA)
• Triad of PHA is hypertension, hypokalaemia and
hypersecretion of aldosterone (Key Box 38.11).
• PHA can be due to a single adrenocortical adenoma
wherein it is called Conn’s syndrome.
• It can also be due to bilateral micronodular hyper-
plasia.
• Clinical features are headache, hypertension, hypo-
kalaemia and weakness, commonly in women.
• Polyuria, polydypsia, nocturia are other features.
• The diagnosis is established by measuring potassium
level and aldosterone to plasma renin activity
ratio.
• CT scan is the most important test to locate the lesion
(usually it is 1–2 cm).
• Treated by spironolactone and antihypertensive
medications in bilateral hyperplasia.
• Laparoscopic adrenalectomy is an effective treatment
in unilateral cases.
Key Box 38.11
Aldosterone
Angiotensin II increases aldosterone secretion.
Hyperkalaemia is another potent stimulator of
aldosterone.
It functions mainly to increase sodium reabsorption
and potassium and hydrogen ion excretion.
It acts at distal convoluted tubules.
CUSHING’S SYNDROME
• Refers to various clinical manifestations resulting
from increased secretion of corticosteroids.
• When it is due to pituitary adenoma secreting
large amount of ACTH—it is called Cushing’s
disease.
• Ectopic ACTH producing tumours causing Cushing’s
syndrome are:
Small cell lung cancers
–
– Foregut carcinoid
– Medullary carcinoma thyroid
– Neuroendocrine pancreatic tumour.
Clinical Features (Key Box 38.12)
They are mainly due to increased corticosteroids,
aldosterone and androgens. The net result is typical
cushingoid facies—with facial plethora, buffalo hump
and moon face.
Key Box 38.12
Clinical Features
Central obesity
Unusual site obesity: Supraclavicular space, posterior
neck
Secondary osteoporosis: Buffalo humps
Hypertension, Hyperglycaemia, Hypokalaemia, Hirsu-
tism
Irregularity in menstruation
Neurological: Depression, mania
Gain in weight
Skin changes: Abdominal striae, ecchymosis, acne,
plethora due to thinning of subcutaneous tissues
Remember as CUSHINGS
Investigations
1. Morning and midnight plasma cortisol levels are
increased.
. Serum ACTH levels: If increased, it is from pituitary
2
source or ectopic ACTH.
3. CT chest and abdomen to assess not only adrenals
but also to detect ectopic sites.
Treatment
1. Medical: Ketoconazole or metyrapone can reduce
steroid synthesis. Thus, symptoms can be controlled.
They are also indicated if surgery is not possible.
2. Trans-sphenoidal resection of ACTH producing
pituitary tumours.
3. Unilateral adenoma: Adrenalectomy.
4. Bilateral tumours: Bilateral adrenalectomy. Postoperatively cortisol should be given.
Stereotactic radiosurgery, which uses CT guidance to deliver
high doses of radiotherapy to the tumour (photon or gamma
knife) is being increasingly used to treat pituitary adenomas.
Section II • General Surgery
448
Manipal Manual of Surgery
ADRENAL INSUFFICIENCY
Definition: Loss of function of adrenal cortex is also
called Addison’s disease.
• Diseases associated with Addison’s disease include:
. After bilateral adrenalectomy
1
2. Tuberculosis
3. Metastases
4. Haemorrhage
5. Amyloidosis, Wilson’s disease
6. HIV infection
Types
1
. Acute adrenal insufficiency
• Shock
• Fever
• Abdominal pain, vomiting confused for acute
abdomen
Waterhouse-Friderichsen syndrome. It is a
bilateral adrenal infarction associated with
meningococcal sepsis.
2. Chronic adrenal insufficiency
• Anorexia, weakness and nausea
• ACTH and POMC—pro-opiomelanocortin levels
increase—resulting in hyperpigmentation of skin
and oral mucosa
• Hypotension, hypercalcaemia and hyponatraemia.
Diagnosis
• Basal ACTH levels are increased
• Cortisol levels are decreased
• ACTH tests: No rise in cortisol levels following
administration of ACTH.
Treatment
• Acute cases: IV hydrocortisone 100 mg 6th hourly,
saline transfusion, control of infections and cardiac care.
2
• Chronic cases: Oral hydrocortisone (10 mg/m
body
surface area) and fludrocortisone (0.1 mg).
• Lifelong treatment with glucocorticoid and mineralo-
corticoid replacement.
SU23.3: Describe the clinical features, principles of
investigation and management of adrenal tumours.
E. Stromal tumours (myelolipoma and schwannoma),
F. Haematological tumours, and secondary tumours.
II. Tumours of the adrenal medulla and extra-adrenal
paraganglia:
A. Pheochromocytoma,
Paraganglioma (head and neck paraganglioma and
B.
sympathetic paraganglioma),
C. Neuroblastic tumours (neuroblastoma, nodular
ganglioneuroblastoma, intermixed ganglioneuroblastoma, and ganglioneuroma),
D. Composite pheochromocytoma, and composite
paraganglioma.
Adrenocortical carcinoma, neuroblastoma and
phaeochromocytoma are discussed in more details here.
ADRENOCORTICAL CARCINOMA
• Incidence: One in 1,000,000 cases.
• Age: Bimodal with a first peak in childhood and
second between fourth and fifth decades.
• 60% of patients present with Cushing’s syndrome.
• Diagnosis of malignancy is (>6 cm) by size of the
tumour, presence of necrosis or haemorrhage,
capsular or vascular invasion.
• CT—heterogeneous tumour, irregular margins,
haemorrhage, lymphadenopathy, metastasis in liver.
• MRI—angiography to exclude tumour thrombus in
vena cava.
• Open radical tumour resection (R
)—en bloc removal
o
of tumour lymph nodes and involved organs is the
treatment of choice.
• Adjuvant chemotherapy—mitotane alone (derivative
of insecticide DDT) is used. It has adrenolytic activity.
• Etoposide, cisplatin and doxorubicin are also used.
• Steroid hypertension is managed by ketoconazole.
I. Neoplasm of the sympathetic neurons
1. Ganglioneuroma: It is a benign neuronal tumour,
commonly arising from retroperitoneal lumbar
sympathetic trunk. FNAC, ultrasound followed by
surgical excision is the management.
2. Neuroblastoma
II. Neoplasm of the chromaffin cells
• Phaeochromocytoma
TUMOURS OF THE ADRENAL GLAND
I. Classification of the tumours of the adrenal cortex
A. Cortical carcinoma,
B
. Cortical adenoma,
C. Sex cord stromal tumours,
D. Adenomatoid tumour, mesenchymal and
Section II • General Surgery
NEUROBLASTOMA
• It is a malignant tumour, derived from sympathetic
nervous system and adrenal medulla.
• Since it originates from the neural crest, it may be
found from orbit to pelvis where sympathetic
nervous tissue is found.
Parathyroid and Adrenals
449
• It occurs in 1 in 10,000 live births.
• It is the most common solid tumour of infancy in
childhood.
• Adrenal gland is the most common site of neuro-
blastoma.
• As the name suggests, the tumour occurs due to
malignant proliferation of the neuroblasts or failure
of maturation of primitive sympathetic nerve cells,
the neuroblasts (Key Box 38.13).
Key Box 38.13
Neuroblastoma
Most common solid tumour in infancy and child-
hood.
Adrenal gland is the most common site.
Mass abdomen and metastasis are common presenting
features.
Surgical excision is the best treatment.
Younger the child, better the prognosis.
Highest incidence of spontaneous remission.
Pathology
• Characterised by presence of immature cells
derived from neuroectoderm of sympathetic nervous
system.
• It is pale with grey surface and well-encapsulated.
• A few areas of calcification are seen (Key Box 38.14).
• Necrosis and haemorrhage are late features.
• Microscopically, uniform round cells containing
hyperchromatic speckled nucleus, Homer-Wright
rosettes with central fibrillar core.
Clinical Features
• 50% of children present to the hospital under the age
of one year and 80–90% are less than 3 years of age.
• An abdominal mass is the most common presenting
feature. The mass has all the features of a renal mass
but location is slightly higher. It is firm to hard,
nodular and fixed.
• The child is sick with weight loss, fever, abdominal
distension, anaemia, etc.
Key Box 38.14
Tumours with Calcification
Neuroblastoma
Chondrosarcoma
Papillary carcinoma thyroid
Phaeochromocytoma (10%)
• Functional tumours produce diarrhoea and
hypokalaemia due to release of vasoactive intestinal
polypeptide (VIP), sweating and flushing due to
release of catecholamines.
• Proptosis and periorbital swellings are due to bony
metastasis and subcutaneous nodules are quite
common (Key Box 38.15).
• Secondaries in retro-orbital region result in infra-
orbital ecchymosis—Raccoon’s eye sign.
• Posterior mediastinal neuroblastomas can produce
cord compression and even paraplegia due to
protrusion within the spinal canal (dumbbell
tumours).
Key Box 38.15
Types of Neuroblastoma
1. Pepper type: Right-sided tumours with secondaries in
the liver.
2. Hutchinson’s type: Left-sided tumour with metastasis in
bones—orbit and skull.
Investigations
• Vanillylmandelic acid (VMA) and homovanillic
acid (HVA) are the byproducts of catecholamines
passed in the urine. 24-hour urinary excretion of
catecholamines and these two metabolites will be
very high.
• Plain X-ray abdomen shows fine stippled calcifica-
tion. X-ray chest is done to rule out cannonball
secondaries.
• Abdominal ultrasound and computed tomography
(CT) can define the mass, nature and extent of the
tumour and can detect hepatic metastasis.
• Magnetic resonance imaging (MRI) is better
than CT scan in detecting the mass as well as
bony metastasis. CT-/MRI-guided core biopsy can
be done.
• Bone marrow aspiration
is positive in around 60–70%
of cases.
Treatment
• Intermediate- and high-risk cases can be managed
by chemotherapy followed by surgery.
• Early cases respond very well to surgical excision.
• However, many children present with metastasis,
and chemotherapy and radiotherapy are given first
to control the disease followed by surgical excision.
Autologous bone marrow transplantation has
improved the outlook for patients with advanced
disease.
Section II • General Surgery
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Manipal Manual of Surgery
PHAEOCHROMOCYTOMA
Definition: These are catecholamine producing
tumours arising from adrenal medulla and sympathetic
ganglia which are derived from chromaffin cells
(Key Box 38.16).
• As the name suggests, phaeo (dusty) chromo
(chromaffin cells)—phaeochromocytoma is a
neoplasm arising from chromaffin cells.
• 90% of the tumour occurs in adrenal glands.
• When phaeochromocytoma occurs in extra-adrenal
sites, it is called paraganglioma.
• Extra-adrenal sites include organ of Zuckerkandl (the
most common site), urinary bladder, renal hilum, chest,
neck, etc. These are the sites of paraganglionic system.
• In about 5% of cases, the tumour can be a component
of multiple endocrine neoplasia (MEN) type IIa or
type IIb.
• Other syndrome associated with phaeochromocytoma
is von Hippel-Lindau syndrome (Key Box 38.17).
• The clinical manifestations are due to release of
adrenaline and noradrenaline. When the level of
noradrenaline is high, symptoms are severe.
Pathology
• It is a soft, highly vascular tumour consisting of large
sympathetic ganglionic cells. Most of the cells are
differentiated.
Key Box 38.16
Phaeochromocytoma
10% Bilateral
10% Malignant
10% Extra-adrenal
10% Multiple
This 10% rule has
been challenged
10% Familial
10% Children
Key Box 38.17
Other Neuroectodermal Anomalies
with Phaeochromocytoma
Neurofibromatosis (NF) type I
Acoustic neuroma
Meningioma, glioma, haemangioblastoma (von
Hippel-Lindau (vHL)) syndrome
Astrocytoma
MEN type II—hereditary phaeochromocytoma
Familial paraganglioma syndrome along with carotid
body tumours
Section II • General Surgery
• Usually, it is small in size and well-encapsulated.
• Sometimes, it can present as a large abdominal mass.
• Microscopic features are polygonal or spheroidal
chromaffin cells within a vascularised fibrous
stroma.
• Presence of high number of Ki-67 positive cells,
vascular invasion or breached capsule and PASS—
phaeochromocytoma of the adrenal gland scale score
suggests metastasis.
Clinical Features
• Sporadic cases peak at 40–50 years, whereas familial
tend to occur early.
• The most common presenting feature is paroxysmal
or persistent hypertension (80–90%). It is associated
with palpitation (50–70%), fever, pallor, tremors,
sweating and severe headache (60–90%).
• The paroxysmal attack may last for a few minutes to
a few hours (Key Box 38.18).
• The factors which stimulate an attack are
Surgery
–
– Anaesthesia
– Invasive procedure
– Late pregnancy
– Drugs—histamine, glucagon, etc.
– Palpation of the mass
– Classical triad consisting of headache, diaphoresis
and palpitations is seen in only a few patients.
• A high index of suspicion is necessary to diag-
nose phaeochromocytoma in a hypertensive
patient.
Investigations
1. Urinary levels of free catecholamines, vanillyl-
mandelic acid (VMA) in excess of 7 mg/24 hours and
meta-adrenaline 1–3 mg/24 hours are suggestive of
phaeochromocytoma.
Key Box 38.18
Hypertension in Phaeochromocytoma
Paroxysmal Persistent
Palpitations Pallor
Profuse sweating Pain abdomen
Palpation of the mass
Observe 7 Ps
Parathyroid and Adrenals
2. Plasma metanephrines: A fractionated plasma-free
metanephrine level may be measured. Blood sample
drawn after 15–20 minutes of IV catheter insertion
(not immediately).
3. Computed tomography
• Noninvasive, safe investigation
• It has a high degree of accuracy
• It can pick up lesions of less than 1 cm in size.
4. MRI is preferred because contrast media used for
CT scan can provoke paroxysms.
131
5.
I Metaiodobenzylguanidine (MIBG) scan
• Iodine labelled MIBG (radionuclide) scan is found
to be very specific for phaeochromocytoma. This
radionuclide scan locates only abnormal adrenal
tissue and is more useful in detecting ectopic sites
of phaeochromocytoma (Fig. 38.13).
Treatment (F
• Surgical removal is the treatment.
igs 38.14 and 38.15)
1
However, a good
preoperative preparation is essential before doing
surgery. It includes control of blood pressure and
tachycardia, by α- and β-blockers (Key Box 38.19).
• Contraction of vascular bed which occurs due to
catecholamines results in hypovolaemia which
must be corrected with IV fluids.
451
Fig. 38.15: Phaeochromocytoma at surgery
Key Box 38.19
Hyperte Phaeochromocytoma
(Preoperative Preparation)
Phenoxybenzamine: 20–60 mg/day for 3–4 weeks
before surgery to control hypertension—increased to
10 mg/day—total of 100–160 mg.
Propranolol: 20–60 mg/day for 5–7 days before
surgery to control tachycardia and arrhythmias.
Plenty of fluids before surgery to correct hypovolaemia.
• First, alpha-adrenergic blockers such as phenoxy-
benzamine is given to control hypertension and to
permit re-expansion of intravascular volume. Only
after the complete alpha-adrenergic blockade, betaadrenergic blockade may be added.
TEN COMMANDMENTS OF PHAEOCHROMOCYTOMA
1. MRI is better because CT scan provokes paroxysm
2. MIBG—
123
I single-photon emission CT will diagnose
about 90% of cases
3. Should not do FNAC/biopsy of adrenals
4. -adrenergic blockers should be started first
5. β-blockade is always given after -blockade
6. CVP and intra-arterial monitoring is a must, as they
may hold a large volume of fluids
7. Should also look on the contralateral side for
phaeochromocytoma
Fig. 38.13: MIBG scan showing
extra-adrenal phaeochromocytoma
(Courtesy: Prof. MG Shenoy, Senior Consultant in Surgery, KMC,
Manipal)
1
Surgery for phaeochromocytoma is like going to a war. Without proper preparation, going to war is a sure failure.
Fig. 38.14: Phaeochromo-
cytoma excised
8. Ligate adrenal vein first
9. Never allow vaginal delivery in patients who have
phaeochromocytoma
10. Laparoscopic adrenalectomy is the ‘Gold standard’
Section II • General Surgery
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Manipal Manual of Surgery
Surgery (see ten commandments given above)
Excision is the treatment. Certain steps of excision
(adrenalectomy) are as follows (Key Box 38.20):
• Sodium nitroprusside must be kept ready to treat
hypertension during surgery as it is a direct arterial
vasodilator (0.5 to 10 µg/kg/minute).
• Thorough search in the abdomen for other sites, due
to the possibility of multiple tumours.
• Postoperative hypotension can be a serious problem
which needs to be treated with large volume of
plasma expanders, blood transfusions, corticosteroids
and vasopressors.
Key Box 38.20
Precautions during Surgery
Midline incision for familial cases
Anterior or posterior approach for sporadic cases
Careful positioning
Ligation of adrenal vein first
Gentle handling
Haemodynamic monitoring—CVP, arterial monitoring
Avoid rupture of the tumour to prevent recurrence
Laparoscopic adrenalectomy is very popular now
MISCELLANEOUS
INCIDENTALOMAS
• Incidentally detected adrenal masses.
• Detected by ultrasound or CT scan (1 to 5% of
patients undergoing CT abdomen for evaluation of
abdominal pain).
• Some patients may have subclinical Cushing’s
syndrome or aldosteronism.
• Nonfunctioning situations may be metastatic cancer
(primary from breast, kidney, melanoma, lung).
• Myelolipoma
• Adrenal cysts
• Majority of such tumours are nonfunctioning cortical
adenomas of no clinical significance.
• Indication to do adrenal gland biopsy is to rule out/
diagnose metastasis.
• Absolute contraindication to do adrenal biopsy in
phaeochromocytoma.
• Any nonfunctioning adrenal incidentaloma should
be resected.
• Majority of incidentalomas are nonfunctional
adenomas.
Refer to clinical notes.
METASTATIC PHEOCHROMOCYTOMA
It was called malignant phaeochromocytoma earlier.
• 10% of phaeochromocytomas are malignant.
• Rate is higher in extra-adrenal tumours.
• Malignancy is suspected when metastasis or
vascular invasion is present or when a capsule is
breached.
• Open adrenalectomy is the choice for excision.
• Debulking should be done even if metastasis is
present, so as to decrease tumour burden and to
control catecholamine excess.
An 18-year-old girl was admitted for tonsillectomy. This
girl suffered from occasional headache. She was
diagnosed to have migraine. Preoperative blood pressure
showed mild elevations which were thought to be due
to anxiety. During tonsillectomy, there was a rise in BP,
which was controlled well. However, in the postoperative
period, there was severe tachycardia, hypertension,
arrhythmias and hypovolaemia. Within 8 hours of
surgery, the patient died even as the diagnosis of phaeochromocytoma was being considered.
Section II • General Surgery
Parathyroid and Adrenals
453
1. Superior parathyroids derive blood supply from:
A. Common carotid artery
B
. Internal carotid artery
C. Inferior thyroid artery
D. Superior thyroid artery
2. Superior parathyroids are found:
A. Above inferior thyroid artery
B
. Below inferior thyroid artery
C. Above superior thyroid artery
D. Below superior thyroid artery
3. Following are true for primary hyperparathyroidism
except:
A. Majority are due to a single adenoma
. Majority are sporadic
B
C. Hypercalcaemia with decreased PTH levels are
often seen
D. Rarely it can be due to carcinoma also
4. Following are true for inferior parathyroid glands
except:
A. Derived from 3rd pharyngeal pouch
B
. It is inferior to inferior parathyroid artery
C. It is superior to inferior parathyroid artery
D. Inferior parathyroids are less constant in position
5. Following are functions of parathyroid gland except:
A. Activates osteoclasts to resorb bone
B
. Increased calcium reabsorption from urine
C. Renal activation of vitamin D
D. Decrease renal excretion of phosphate
6. Following is not the feature of primary hyperparathyroidism:
A. Hypercalcaemia
. Most common manifestation of MEN type I
B
C. Band keratopathy in cornea
D. Dystrophic calcification
7. The best investigation of choice to localise parathyroid gland is:
99m
Tc-labelled sestamibi scan
A.
B. CT scan
C. MRI
D. Ultrasound
8. Which one of the following is not the site of inferior
parathyroid gland?
A. Thyrothymic axis
. Mediastinum
B
C. Within carotid sheath
D. Cricothyroid articulation
9. Following are true for parathyroid hyperplasia
except:
A. It affects all 4 glands
It is not the common cause of hyperparathyroidism
B.
C. Total parathyroidectomy is the treatment of choice
D. Thymectomy need not be done
10. Which one of the following is the treatment of choice
for acute hypercalcaemic crisis?
A. Steroids B. Diuretics
. Vasopressors D. Bisphosphonates
C
11. Which is the first drug to be used in the preoperative
preparation of a patient with phaeochromocytoma?
A. Steroids
. Noradrenaline
B
C. Propranolol
D. Phenylbenzamine
12. Which one of these operative steps is done first in
excision of phaeochromocytoma?
A. Ligation of adrenal vein
. Ligation of inferior phrenic artery
B
C. Ligation of branch of renal artery
D. Isolation from kidney
13. A few landmarks for identification of ectopic sites
of phaeochromocytoma include following except:
A. Urinary bladder
. Along 5th cranial nerve
B
C. Organ of Zuckerkandl
D. Superior para-aortic region—below the
diaphragm and renal poles
14. Following are true for management of phaeochromocytoma except:
A. Phenoxybenzamine is given prior to propranolol
. Intra-arterial monitoring is a must
B
C. It is dangerous to infuse large volumes of fluid
after ligation of adrenal vein
D. Laparoscopic resection is the gold standard
Section II • General Surgery