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- •Series Editors’ preface
- •Editors’ preface
- •Evidence-based practice in surgery
- •Contributors
- •Liver function and failure
- •Hepatic, biliary and pancreatic anatomy
- •Staging and assessment of hepatobiliary malignancies
- •Benign liver lesions
- •Primary malignant tumours of the liver
- •Colorectal liver metastases
- •Non-colorectal hepatic metastases
- •Portal hypertension and liver transplantation
- •Pancreas and islet transplantation
- •The spleen and adrenal glands
- •Gallstones
- •Benign biliary tract diseases
- •Malignant lesions of the biliary tract
- •Complicated acute pancreatitis
- •Chronic pancreatitis
- •Pancreatic adenocarcinoma
- •Cystic and neuroendocrine tumours of the pancreas
- •Hepatobiliary and pancreatic trauma

Chapter 10
indication. Packs are placed behind the spleen to
elevate and bring it towards the midline. The colon
is retracted inferiorly and the stomach is retracted
superiorly to expose the gastrocolic ligament. The
gastrocolic ligament is divided in the avascular
plane to open the lesser sac and expose the splenic
artery coursing along the superior border of the
pancreas. In patients with splenomegaly, early
isolation and ligation of the splenic artery and vein
are recommended at this point. The gastrosplenic
ligament containing the short gastric arteries and
the phrenicocolic ligament are divided to mobilise
the spleen medially and allow elevation to expose
the splenorenal ligament. If the spleen is normal
in size, ligation and division of the splenic artery
and vein using clamps and ties or a vascular linear
stapling device is performed at this point. Once the
specimen is removed, the abdomen is inspected for
accessory spleens, which are removed if found.
Laparoscopic
Since the first report of the procedure in 1991 by
Delaitre and Maignein,
has become the standard approach for removal
of the spleen for most indications. The patient is
positioned in the right lateral decubitus position
flexed at the hip, which allows for maximum
exposure of the left hypochondrium and gravity, to
reveal the ligamentous attachments. The right arm
is extended and the left arm suspended. The surgeon
and assistant face the patient. Video monitors are
positioned at the head of the bed. Four trocars are
generally placed along a linear curve situated below
the left costal margin once pneumoperitoneum
is achieved using a Hassan technique. The optical
trocar is usually inserted in the anterior axillary
line below the left costal margin. Lateral working
ports at the mid-axillary line and posterior-axillary
line, as well as a medial working port at the mid-
clavicular line below the left costal margin are
placed under direct vision. The next step involves
freeing the splenic flexure and mobilising the colon
inferiorly and medially. This facilitates the division
of the gastrosplenic ligament and short gastric
vessels, which mobilises the stomach medially
to reveal the hilum of the spleen and splenorenal
ligament containing the splenic artery and vein. The
spleen is rotated medially and the lateral peritoneal
attachments are divided. The posterior peritoneum
is opened to reveal the vessels, which are then
divided using a vascular endoscopic stapling device
while protecting the pancreas. The freed specimen
is placed in a bag, morcellated and removed. Once
the specimen is removed, the abdomen is inspected
for accessory spleens, which are removed if found.
23
laparoscopic splenectomy
Partial splenectomy
A major long-term risk of total splenectomy is
overwhelming infection, particularly in children
younger than 5 years. Consequently, partial
splenectomy has gained popularity, initially in the
paediatric population, but increasingly in adult
patients. Partial splenectomy may be done open
or laparoscopically, although a minimally invasive
technique is usually the preferred method. The
indications for partial splenectomy include benign
tumours (hamartoma, epidermoidal cyst, or localised
lymphangioma or haemangioma) and haematologic
conditions leading to hypersplenism. Partial
splenectomy may not be performed for tumours
that are centrally located due to the terminal blood
supply of the spleen. The operating room set-up,
patient positioning, trocar placements and initial
dissection are the same as for laparoscopy. However,
careful control of the individual vessels that supply
the different pedicles of the spleen is the most critical
difference. The hilar vessels, leading to an anterior
artery and posterior vein, terminating in the splenic
parenchyma, are meticulously dissected. Depending
on the location of the tumour, the vessels terminating
in the pole or region containing the abnormality are
isolated. Placing a temporary clamp will create an area
of demarcation to confirm the appropriate selection.
The artery and vein are then divided in sequence
(if the upper pole is selected, the short gastrics will
be divided as well). The line of demarcation is then
used to guide the transection along the surface of the
parenchyma. The parenchyma is then cauterised and
divided. The omentum can be placed in contact with
the cut section of the spleen. The remainder of the
procedure is similar to the laparoscopic approach.
Splenectomy vaccinations
Because the spleen is particularly important in
the immune response to encapsulated bacteria,
vaccinations against Haemophilus influenza
B, Streptococcus pneumoniae and Neisseria
meningitidis should be administered perioperatively.
For all elective cases, vaccinations should be
administered at least 2 weeks preoperatively. For
emergency cases, vaccinations should be administered
2–4 weeks postoperatively, although for trauma
patients, just prior to discharge is acceptable due to
a high incidence of loss to follow-up. The purpose of
perioperative vaccination is to avert overwhelming
postsplenectomy infection (OPSI). OPSI is the
development of a fulminant, rapidly fatal bacterial
infection following splenectomy. OPSI is the most
feared complication after removal of the spleen, and
the incidence in the first 2years postsplenectomy is
estimated at 0.9% for adults and 5% for children.
24
172
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The spleen and adrenal glands
aorta
n
Right adrenal vein
Right adrenal
Phrenic
Adrenal
Adrenal
Introduction
The adrenal glands are bilateral neuroendocrine
organs that are situated superiorly in relation to
the kidneys. The word adrenal originated from
the Latin ad renalis, meaning ‘of the kidneys’.
The Roman anatomist Galen is believed to be the
first to describe ‘loose flesh’ in relation to what
is now known as the left adrenal vein. The 16th
century anatomist Bartolomeus Eustachius gave a
more definitive description and illustration of the
adrenal glands in his work Opuscula Anatomica.
Knowsley-Thornton performed the first successful
adrenalectomy in 1889 on a 36-year-old woman
with a 20-pound adrenal tumour.
26
Since that
time, there have been significant advances in
understanding the function of the adrenal glands
and improvements in assessing the indications and
techniques for performing adrenalectomy.
25
Anatomy and embryology
The adrenal glands are retroperitoneal endocrine
organs situated above and slightly medial to the
kidneys within the renal fascia on both sides of
the abdomen (
has a pyramidal shape while the left adrenal gland
Fig. 10.3). The right adrenal gland
is semilunar. The left adrenal gland is bounded
anteriorly by the peritoneum, tail of the pancreas
and splenic artery, posteriorly by the left crus of
the diaphragm and kidney, and medially by the
left inferior phrenic artery and left gastric arteries.
The right adrenal gland is bounded anteriorly by
the inferomedial angle of the bare area of the liver,
posteriorly by the diaphragm and superior pole
of the right kidney, and medially by the inferior
phrenic artery and vena cava. The adrenal glands are
composed of two heterogenous types of tissue that
are arranged into distinct components. The cortex is
the outermost component of the adrenal gland and
is derived from intermediate mesoderm. The cortex
is subdivided into different layers, namely the zona
glomerulosa, zona fasciculata and zona reticularis
Fig.10.4). The medulla is the innermost component
(
of the adrenal gland and is derived from neural
crest cells (
Fig.10.4). The adrenal glands are highly
vascular organs that receive their blood supply from
the superior adrenal artery (a branch of the inferior
phrenic artery), the middle adrenal artery (a direct
branch from the aorta), and inferior adrenal artery
(a branch of the ipsilateral renal artery) (
Fig.10.3).
Venous drainage is different for the right and left
adrenal glands. The right adrenal drains via a
shorter right adrenal vein directly into the inferior
vena cava, while the left adrenal gland drains via a
longer left adrenal vein that is often joined by the
left inferior phrenic vein before emptying into the
artery
Figure10.3 • Gross anatomical features of the adrenals. The adrenal glands are retroperitoneal endocrine organs
situated above and slightly medial to the kidneys, which receive their blood supply from the superior, middle, and inferior
adrenal arteries. The right adrenal drains via a shorter right adrenal vein directly into the inferior vena cava, while the left
adrenal gland drains via a longer left adrenal vein that is often joined by the left inferior phrenic vein before emptying into
the left renal vein.
Right
kidney
Renal
artery
Renal
vein
Inferior
vena cava
artery
Abdominal
Renal
vein
Renal
artery
Inferior
phrenic vein
Left adrenal
Left adrenal vei
Left
kidney
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173

Chapter 10
e
Transverse section Microscopic section
Right
Adrenal
kidney
glands
Left
kidney
Capsule
Adrenal
cortex
Adrenal
medulla
Figure10.4 • Microscopic anatomical features of the adrenals. The adrenal glands are composed of an inner
medulla and outer cortex. The adrenal medulla produces catecholamines. The adrenal cortex is divided into the zona
glomerulosa, where mineralcorticoids are produced, zona fasciculata, where glucocorticoids are produced, and the zona
retularis, where androgens are produced.
left renal vein. Lymphatic drainage from the adrenal
glands flows directly into adjacent periaortic and
paracaval nodes.
Mineralocorticoids
Glucocorticoids
Adrenal
androgens
Catecholamines
Epinephrine
Norepinephrine
progesterone are modified by 17-alpha-hydroxylase,
which can be further modified by 21-hydroxylase
and 11-beta-hydroxylase to produce cortisol, the
Glomerulosa
Fasciculata
Reticularis
Chromaffin cells
Medullary veins
Splanchnic nerves
Capsul
Cortex
Medulla
major glucocorticoid in humans. Cortisol is secreted
directly into the circulation immediately upon its
Physiology
synthesis and circulates in both bound and free
unbound state. The free form passes into target
Two separate endocrine organs, namely the
cortex and medulla, comprise the adrenal gland.
The adrenal cortex metabolises cholesterol to
produce different steroid hormones in each zone
Fig.10.4). In all three zones, the rate-limiting step
(
is the conversion of cholesterol to pregnenolone by
cholesterol desmolase, which is directly regulated
by adrenocorticotropic hormone (ACTH) from
the pituitary, which in turn is regulated by
corticotropin-releasing (CRH) hormone from the
hypothalamus. The zona glomerulosa is the main
site for the production of mineralocorticoids that
regulate salt balance and blood volume. In this zone,
3-beta-hydroxysteroid dehydrogenase, 21-alphahydroxylase, 11-beta-hydroxylase, and aldosterone
synthase to convert pregnenolone to aldosterone.
Aldosterone is the principal mineralocorticoid
that acts by binding nuclear receptors in cells of
the distal convoluted tubules and collecting duct
of the kidneys and drives transcription of genes
that activate basolateral Na/K pumps to increase
reabsorption of sodium and excretion of potassium
and acid. The Na/K pumps create a concentration
gradient that results in water reabsorption into the
blood, thereby expanding intravascular volume and
increasing blood pressure. The zona fasciculata is
the main site for production of glucocorticoids that
have many effects on metabolism. Pregnenolone and
cells by diffusion and binds to cytosolic receptors
found in virtually all cells in the body. Once bound
to its receptor, cortisol not only has glucose-
regulating properties but also exerts a myriad of
effects on gluconeogenesis, glycogenesis, protein
synthesis, lipolysis, mineral homeostasis, vascular
tone, immunosuppression and wound healing. In
addition, cortisol acts on cells of the hypothalamus
and anterior pituitary to negatively regulate
production of CRH and ACTH. The zona reticularis
is the principal site for androgen production. A series
of hydroxylase and dehydrogenase enzymes convert
pregnenolone to androstenedione and testosterone.
These androgens can be modified by aromatases
resulting in oestrone and oestradiol, and by
5-alpha-reductase to produce dihydrotestosterone,
resulting in male and female steroid effects during
development.
There are a number of enzyme defects that can
lead to adrenal dysfunction and congenital adrenal
hyperplasia. The most common form of congenital
adrenal hyperplasia is caused by a 21-hydroxylase
deficiency that leads to excess androgen and
mineralocorticoid, and deficient glucocorticoid
resulting in salt-wasting dehydration and ambiguous
genitalia or virilisation. Dysfunctional 11-betahydroxylase leads to excess mineralocorticoid and
androgen production, which leads to virilisation
174
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The spleen and adrenal glands
and hypertension. Defects in the enzyme 17-alphahydroxylase cause a rare form of congenital adrenal
hyperplasia characterised by glucocorticoid and
androgen deficiency and mineralocorticoid excess.
This condition may lead to ambiguous genitalia at
birth or delayed puberty, as well as hypokalaemic
hypertension.
Like the peripheral sympathetic ganglia, the
adrenal medulla is embryologically derived from
neural crest cells. The medullary chromaffin cells
have rudimentary nerve fibres and the ability
to synthesise, store, and secrete catecholamines,
primarily epinephrine.
27
The rate-limiting step is
the formation of dopa from tyrosine via the enzyme
tyrosine hydroxylase, which is principally regulated
by ACTH. Although peripheral nerve terminals and
the adrenal medulla both have the ability to produce
norepinephrine, only the adrenal medulla contains the
enzyme phenylethanolamine-N-methyltransferase
(PNMT) that converts norepinephrine to epinephrine,
which constitutes 80–85% of the adrenal medulla
secretion. Catecholamines released by the adrenal
medulla bind a large family of transmembranesignalling adrenoreceptors to exert a variety of effects
associated with acute stress responses, including
tachycardia, hypertension, increased peripheral
vascular resistance, gastrointestinal inactivation,
bronchodilatation, and increased metabolism.
28
Indications for adrenalectomy
Adrenal masses may be benign or malignant and
functional or non-functional. The systematic
work-up of any suspected adrenal pathology
should begin with a thorough history and
physical examination investigating the possibility
of functional symptoms and assessment for
the rare possibility of a genetic syndrome. The
history should query for poorly controlled
hypertension, diabetes, oedema, palpitations,
diaphoresis, headaches, flushing or ecchymosis.
The physical examination should inspect for central
obesity, peripheral wasting, oedema, weakness,
dorsocervical lipodystrophy, striae, atrophic skin
and facial plethora. Initial laboratory studies should
include biochemical evaluation of plasma sodium,
potassium, bicarbonate and glucose levels.
Aldosteronoma (primary
hyperaldosteronism)
Primary aldosteronism was first described as a
clinical syndrome by Conn in 1955.
and symptoms of primary hyperaldosteronism are
non-specific and include resistant hypertension
and hypokalaemia, although patients may also
complain of polydipsia, polyuria, muscle weakness
and cramping. Hyperaldosteronism may occur
29
The signs
more commonly than originally thought, with
increasing prevalence in patients with more severe
forms of essential hypertension.
30
In addition,
hyperaldosteronism may occur in the absence of
hypokalaemia, as electrolyte abnormalities may
be a late manifestation of the disease.
31,32
The
most common causes of primary aldosteronism
are a solitary aldosterone-producing adenoma
(APA) and idiopathic hyperaldosteronism (IHA).
Other less common forms include primary adrenal
hyperplasia, familial hyperaldosteronism and
aldosterone-producing adrenocortical carcinoma.
Mutations of the potassium channel gene KCNJ5
have been identified as the cause in APA.
33–35
Other less common somatic mutations in ATP1A1,
ATP2B3, CACNA1D and CTNNB1 have also
been identified in aldosterone-producing lesions.
Biochemical studies that can aid diagnosis are
plasma aldosterone to renin ratio, as a means
to increase the diagnostic accuracy of either
laboratory value alone. In addition, demonstrating
that endogenous aldosterone secretion is not
inhibited by exogenous saline infusion has been
used to confirm the diagnosis. CT can then be used
for localisation.
Cushing syndrome
In 1932, Harvey Cushing attributed the syndrome
of truncal obesity, facial plethora, hypertension,
polyphagia and polydipsia to pituitary adenomas
discovered upon autopsy of patients with these
physical findings. The most common cause of this
syndrome is iatrogenic administration of exogenous
glucocorticoids. Thus, thorough medical and
pharmacological histories are crucial. The most
common non-iatrogenic cause of this syndrome is
Cushing’s disease, adrenal glucocorticoid excess
caused by ACTH-producing pituitary adenoma.
Although the pituitary adenomas of Cushing’s
disease secrete excessive amounts of ACTH,
they generally retain some negative feedback
responsiveness to high doses of glucocorticoids.
In contrast, ectopic ACTH-producing tumours
can also cause the syndrome but are insensitive to
negative feedback by high doses of glucocorticoids.
The major cause of ACTH-independent Cushing’s
syndrome is a glucocorticoid-producing adrenal
adenoma. Because these tumours secrete excessive
cortisol, production of ACTH is suppressed in an
otherwise normal hypothalamus–pituitary–adrenal
axis. The initial step in the diagnosis of Cushing’s
syndrome is to confirm the presence of elevated
cortisol levels by measuring night-time serum or
salivary cortisol, 24-hour urinary cortisol and/or
undertaking an overnight low-dose dexamethasone
suppression test. The next step is to determine
whether the cause of hypercortisolaemia is an
ACTH-dependent or independent process. If serum
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175

Chapter 10
ACTH levels are suppressed, an ACTH-independent
process originating from the adrenal glands is the
likely cause of the syndrome. If serum ACTH levels
are elevated, a high-dose dexamethasone suppression
test is required. If ACTH levels are suppressed with
high-dose dexamethasone, the likely aetiology is
a pituitary tumour. However, if ACTH levels are
unaffected by high-dose dexamethasone, an ectopic
source of ACTH is the likely cause. If an adrenal
cause is suspected, an abdominal CT should be
performed to localise the tumour. Cortisol-secreting
adrenal tumours, particularly those with overt
Cushing’s syndrome and high cortisol levels, have
been associated with somatic activating mutations in
protein kinase A (PKA) catalytic subunit (PRKACA)
and the Gs alpha subunit (GNAS).
36,37
Pheochromocytoma
Pheochromocytoma is a catecholamine-secreting
tumour that arises from the neuroectodermally
derived chromaffin cells of the adrenal medulla.
Paragangliomas are related tumours that arise
from similar cells of the sympathetic ganglia. These
tumours are rare, occurring in <0.2% of patients with
hypertension.
are benign, solitary and unilateral, and occur in
adults. Pheochromocytomas are usually sporadic,
although they can accompany genetic syndromes
including multiple endocrine neoplasia (MEN) 2a
and 2b caused by a number of somatic mutations
in the gene RET that encodes a transmembrane
receptor tyrosine kinase involved in a number
of signalling pathways,
syndrome caused by mutations in the NF1 gene
that encodes neurofibrin 1, a negative regulator
of the ras signal transduction pathway, and von
Hippel–Lindau syndrome caused by a mutation in
the tumour suppressor VHL gene. Patients typically
present with refractory hypertension, palpitations,
headaches and diaphoresis. Symptoms are usually
episodic with variable frequency and duration.
The diagnosis of pheochromocytoma is dependent
on demonstrating elevated catecholamines in the
plasma or urine. Initially, plasma metanephrine
and normetanephrine levels should be obtained.
In addition, 24-hour urine vanillylmandelic acid,
metanephrines, and fractionated catecholamines
may be obtained. Once a biochemical diagnosis
has been made, the tumour can be localised by CT,
magnetic resonance imaging (MRI) or
iodobenzylguanidine (MIBG) scan.
Patients with pheochromocytoma have chronic
hypersecretion of catecholamines that causes
volume-contraction as well as haemodynamic
and glycaemic instability. Thus, they must be
pre-medicated with a selective alpha-adrenergic
receptor-blocker (phenoxybenzamine) for several
weeks before proceeding to adrenalectomy.
38
The majority of pheochromocytomas
39
von Recklinghausen’s
123
I meta-
Postoperatively, patients are susceptible to
vasoplegia and hypoglycaemia, and should be
monitored closely.
Primary adrenocortical carcinoma
Adrenocortical carcinomas are rare tumours that
can be functional and cause hyperaldosteronism,
hypercortisolism, and/or virilisation, or nonfunctional and present as an abdominal mass
or incidental finding. Most adrenocortical
carcinomas are sporadic, but some occur as part
of several hereditary cancer syndromes, including
Li–Fraumeni syndrome caused by inactivating
mutations of the TP53 tumour suppressor gene,
Beckwith–Wiedemann syndrome caused by a
mutation in the insulin-like growth factor 2 (IGF2)
41,42
gene,
and MEN1 caused by inactivating
mutations of the MEN1 tumour suppressor gene.
The majority of patients with adrenocortical
carcinomas present with tumours >
4 cm that
produce a clinical syndrome of hormone excess.
A significant number have distant metastases at
presentation, usually to liver, lungs, lymph nodes
and bone.
44
Initially, a biochemical work-up
similar to those described above must be pursued.
Imaging characteristics on CT can distinguish
adenomas from carcinoma and MRI can accurately
evaluate the extent of vascular or adjacent organ
involvement. Positron emission tomography (PET)/
CT is also useful for determining distant sites of
disease. Adrenocortical carcinoma is staged using
the TNM staging criteria. T1 tumours are <
with no local invasion, while T2 tumours are >5 cm
with no local invasion. T3 tumours are of any size
with invasion into the surrounding fat, while T4
tumours are any size with invasion into adjacent
organs. N0 indicates no positive lymph nodes,
while N1 designates lymph node involvement
with tumour. M0 indicates the absence of distant
metastases, while M1 specifies that the cancer
has spread to distant sites. The 5-year survival
disease-specific survival rate for American Joint
Commission on Cancer (AJCC) stage I tumours
(T1N0M0) is 82%, stage II tumours (T2N0M0) is
58%, stage III tumours (T1/2N1M0 or T3N0M0) is
55% and stage IV tumours (T3N1M0 or T4N0M0
or TxNxM1) is 18%. The 5-year disease-specific
survival for the European Network for the Study
of Adrenal Tumours stage I tumours (T1N0M0) is
82%, stage II tumours (T2N0M0) is 61%, stage
III tumours (T3/4N0M0 or TxN1M0) is 50% and
stage IV tumours (TxNxM1) is 13%.
45
Complete
surgical resection is the only potentially curative
treatment for adrenocortical carcinoma, although
mitotane, an adrenocorticolytic drug, has been
used in the adjuvant setting or for unresectable or
recurrent disease.
46
5 cm
40
43
176
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The spleen and adrenal glands
Incidentaloma
Adrenal incidentalomas are tumours that are
incidentally discovered on imaging performed
during the work-up of unrelated conditions.
Autopsy studies suggest a prevalence of clinically
unapparent adrenal masses of 1–9%, which
increases with age. Moreover, with the widespread
use of cross-sectional imaging in clinical practice,
adrenal masses are serendipitously discovered in
4–10% of patients.
47,48
The work-up of adrenal
incidentalomas must address functionality
and malignant potential of the mass that will
guide treatment or observational approaches. A
thorough history and physical examination must
be performed to evaluate for potential subtle
clues about functionality or malignancy that may
have been overlooked during prior assessments.
Biochemical evaluations as described above should
be performed to rule out aldosterone, cortisol
or catecholamine-producing adrenal masses.
Although most adrenal incidentalomas are nonfunctional, approximately 10–15% secrete excess
adrenal hormones.
49,50
Malignancy is a rare cause
of adrenal incidentalomas. Fewer than 5% of
adrenal incidentalomas are primary adrenocortical
carcinoma or non-adrenal metastases. The size and
imaging characteristics are useful in determining
whether an adrenal mass is benign or malignant.
Non-contrast CT, contrast-enhanced CT with delay
and MRI are useful in evaluating incidentalomas.
PET/CT can also be used in cases where imaging
is equivocal and tissue diagnosis by fine-needle
aspiration (FNA) should only be pursued in selected
cases and only after pheochromocytoma has been
ruled out.
functional lesions, masses >
51
Adrenalectomy is recommended for
4 cm, or tumours
with imaging characteristics concerning for
malignancy. For masses with benign appearances
(<10 HU, washout >50%), small (<
4 cm),
and completely non-functional, imaging and
biochemical surveillance between 3 and 12months
is reasonable.
48–50
Secondary adrenal metastases
Metastases should be suspected in patients with
adrenal masses and a history of extra-adrenal
malignancy. Renal cell carcinoma, melanoma, nonsmall-cell lung cancer, breast cancer, colorectal cancer
and lymphoma have a predilection for spread to
the adrenal glands. Most adrenal metastases are
asymptomatic, and are discovered on surveillance
imaging for cancer. They are usually unilateral,
although a significant percentage may be bilateral.
Although chemotherapy is usually the treatment
for disseminated cancer, open or laparoscopic
adrenalectomy may be performed in patients
52
with otherwise well-controlled disease with either
synchronous or metachronous oligometastases to the
adrenal gland.
53–57
Adrenalectomy
Open
Left
The patient is placed in a slight right lateral
decubitus position. The abdomen is accessed
via an extended subcostal incision. The splenic
flexure of the colon is mobilised and the omental
attachments along the transverse colon are divided
allowing access to the lesser sac. The splenorenal,
splenophrenic and retroperitoneal attachments of
the spleen are divided and the tail of the pancreas
is rotated medially, exposing the adrenal gland in
the retroperitoneum. Gerota’s fascia and the upper
border of the left kidney are identified and divided.
The left renal hilum is dissected to reveal the left
renal vein and its confluence with the left adrenal
vein, which is ligated and divided just cephalad to
its confluence with the inferior phrenic vein. The
retroperitoneal fat and suprarenal tissue are elevated
and dissected off the superior pole of the left kidney,
lateral abdominal wall and left quadratus lumborum
muscle. The three main arterial branches from the
left renal artery, aorta and left phrenic artery are
ligated during the dissection. The medial dissection
is carried as far as necessary to obtain a negative
margin. For oncologic adrenalectomy, para-aortic
lymph nodes are dissected and removed en bloc with
the specimen. The pitfalls of left adrenalectomy are
injury to the spleen, pancreas or diaphragm, rupture
of the capsule of the gland and misidentification of
the vascular anatomy.
Right
The patient is placed in a slight left lateral decubitus
position. The abdomen is accessed via an extended
subcostal incision. The liver is mobilised medially by
dividing the triangular ligament to allow exposure
of the inferior vena cava and the right adrenal
gland. The right border of the vena cava is dissected
caudally to the diaphragm to allow the right adrenal
vein to be identified, ligated and divided. The right
border of the vena cava is dissected rostrally to
reveal the right renal hilum. Gerota’s fascia and
the upper border of the right kidney are identified
and divided. The retroperitoneal fat and suprarenal
tissue are elevated and dissected off the superior
pole of the right kidney, lateral abdominal wall and
right quadratus lumborum muscle. The three main
arterial branches from the right renal artery, aorta
and right phrenic artery are ligated during dissection
of the right suprarenal tissues. If performed for
oncological purposes, an en bloc dissection of the
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177

Chapter 10
associated lymph nodes is also performed as part of
the medial extent of the dissection of the suprarenal
tissue. The major pitfalls of a right adrenalectomy
are tumour rupture, injury to the vena cava and
right diaphragmatic injury.
Laparoscopic
Gagner and his colleagues performed the first
laparoscopic adrenalectomy in 1992.
minimally invasive approaches have become the gold
standard for benign adrenal tumours. Minimally
invasive approaches offer a magnified view of the
operative field, improved control of the vascular
pedicles and smaller incisions that result in reduced
postoperative discomfort, hospital stay and wound
morbidity compared to open approaches. Open
approaches are still considered the standard surgical
management in primary adrenal malignancy.
Although laparoscopic approaches for clinically
unsuspected adrenocortical carcinoma were initially
associated with a high recurrence rate,
data suggest that laparoscopic approaches may be
an option in carefully selected cases that minimise
jeopardising oncological outcomes.
Left
The patient is placed in a right lateral decubitus
position flexed at the hip. The right arm is extended
and the left arm suspended. The surgeon faces
the patient and the assistant is behind the patient.
Video monitors are positioned at the head of the
bed. Four trocars are generally placed along a linear
curve situated below the left costal margin once
pneumoperitoneum is achieved using a Hassan
technique. After exploration of the abdomen, the
spleen is mobilised by dividing the splenorenal
ligament starting at the inferior pole and extending
up to the left crus of the diaphragm. The medial
reflection of the spleen and pancreas is dissected
58
Since then,
60
59
recent
along the splenic vein exposing the left renal and
adrenal veins. The left adrenal vein is dissected
cephalad to reveal its convergence with the inferior
phrenic vein, where it is ligated and divided. Next,
the inferior, middle and superior adrenal arteries
are ligated and divided. The superior, posterior
and lateral aspects of the gland are dissected free.
Cephalad retraction allows dissection of the gland
along its inferior adrenal pedicle, which frees it from
the superior pole of the kidney. The gland is then
placed in a plastic bag and removed.
Right
The patient is placed in a left lateral decubitus position
flexed at the hip. The left arm is extended and the
right arm suspended. The surgeon faces the patient
and the assistant is behind the patient. Video monitors
are positioned at the head of the bed. Four trocars are
generally placed along a linear curve situated below
the right costal margin once pneumoperitoneum is
achieved using a Hassan technique. The right adrenal
gland is situated behind the liver, necessitating its
medial mobilisation by dividing the triangular
ligament. An atraumatic liver retractor is introduced
through the lateral port to hold the liver out of the
way. Mobilisation of the liver allows for identification
of the vena cava, which is the main anatomical
landmark for identifying and dissecting the right
adrenal gland. The right border of the vena cava is
dissected caudally to expose the renal vein, which
constitutes the inferior landmark of the operating
field. The vena cava is then dissected cephalad to
the diaphragm, to expose the main adrenal vein
and, if present, the accessory adrenal vein. Both are
ligated and divided. The arterial blood supply is then
identified, ligated and divided. The gland is then freed
from its fatty and inferior ligamentous attachments
along the superior pole of the kidney. The gland is
then placed in a plastic bag and removed.
Key points
• The spleen is a highly vascularised organ located in the upper left abdomen with important
haematopoietic and immunological functions.
• A number of traumatic, haematologic or neoplastic causes, or infectious disease, require surgical
intervention to remove all or part of the spleen.
• Splenectomy can be performed with open or laparoscopic approaches.
• The adrenal glands are bilateral retroperitoneal neuroendocrine organs situated above the kidneys.
• The adrenal glands are the site for steroid hormone and catecholamine production that have a variety
of tissue targets and physiological functions.
• A number of benign and malignant diseases require surgical intervention to remove one or both
adrenal glands.
• Adrenalectomy can be performed with open or laparoscopic approaches.
178
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The spleen and adrenal glands
Full references available at http://expertconsult.
inkling.com
Key references
5. MooreEE, ShackfordSR, PachterHL, etal. Organ
injury scaling: spleen, liver, and kidney. J Trauma
1989;29(12):1664–6. PMID: 2593197.
The Organ Injury Scaling (OIS) committee was
commissioned at the 1987 meeting of the American
Association for the Surgery of Trauma to devise injury
severity scores for individual organs to facilitate clinical
research. The organ injury scaling has led to validated
algorithms for operative management and clinical
decision-making based on injury severity.
13. KuterDJ, RummelM, BocciaR, etal. Romiplostim
or standard of care in patients with immune
thrombocytopenia. N Engl J Med 2010;363(20):
1889–99. PMID: 21067381.
An open-label, prospective, randomised, double-arm
trial showing improved primary endpoints for patients
who received romiplostim compared to standard of
care for ITP.
14. Godeau B, Porcher R, Fain O, et al. Rituximab
efficacy and safety in adult splenectomy candidates
with chronic immune thrombocytopenic purpura:
results of a prospective multicenter phase 2 study.
Blood 2008;112(4):999–1004. PMID: 18463354.
A multicentre, prospective, open-label, single-arm,
phase II trial demonstrating rituximab as a safe and
effective splenectomy-avoiding option in some adults
with chronic ITP.
24. Mourtzoukou EG, Pappas G, Peppas G, et al.
Vaccination of asplenic or hyposplenic adults. Br J
Surg 2008;95(3):273–80. PMID: 18278784.
Provides evidence-based guidelines for vaccination for
prevention of overwhelming sepsis in asplenic patients.
35. ChoiM, SchollUI, YueP, etal. K+ channel mutations
in adrenal aldosterone-producing adenomas and
hereditary hypertension. Science 2011;331(6018):
768–72. PMID: 21311022.
Identified mutations in the adrenal potassium
channel KCNJ5 that result in constitutive aldosterone
production and cell proliferation leading to aldosterone
producing tumours.
36. Goh G, Scholl UI, Healy JM, et al. Recurrent
activating mutation in PRKACA in cortisol-producing
adrenal tumors. Nat Genet 2014;46(6):613–7. PMID:
24747643
37. Sato Y, MaekawaS, IshiiR, etal. Recurrent somatic
mutations underlie corticotropin-independent
Cushing's syndrome. Science 2014;344(6186):917–
20. PMID: 24855271.
Identified genetic mutations resulting in cyclic adenosine
monophosphate (cAMP)-independent protein kinase
(PKA) activation that led to the development of cortisolproducing adrenal tumours.
Downloaded for Anonymous User (n/a) at Rutgers University - NERL from ClinicalKey.com by Elsevier on March 22, 2019.
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179

11
11
Gallstones
Ian J. Beckingham
Introduction
The gallbladder serves as a reservoir to hold
bile and release it in a bolus when fat is ingested
(Fig.11.1). Fat in the stomach results in the release
of cholecystokinin (CCK) which causes contraction
and emptying of the gallbladder as food enters the
duodenum. Bile helps to emulsify fat within the
small bowel and aid its absorption. Whilst in the
gallbladder, bile is concentrated by the absorption
of up to 70% of the water content.
Many animals do not have gallbladders – all
members of the deer family (except the musk deer),
all of the equine family, camels, giraffes, elephants,
rhinoceroses, whales, some birds (such as doves,
pigeons and parrots), rats and some fish, do not
have gallbladders. It is thought that the presence
of a gallbladder is related to the interval of food
intake. Thus animals, like humans, cats and dogs,
which take in food at intervals, require a larger
amount of bile acids to aid digestion of fats arriving
in a bolus, rather than in a more constant stream.
In some societies the gallbladder is attributed
with more than just physical properties. In Korea,
the flighty nature of deer is blamed on its lack of
a gallbladder, and when a person acts eccentrically
or irrationally Koreans say the person lacks a
gallbladder. Conversely, when someone is brave,
bold and daring, they say the person has a big
gallbladder.
influence of bile and use powdered bovine gallstones
in their traditional medicines as an antipyretic and to
aid sleep and cure diseases of the liver and epilepsy.
Ox gallstones are also used as an aphrodisiac and
bovine gallstones can fetch up to $14000 a kilo on
the commercial market.
1
The Chinese proclaim the calming
Pathogenesis of gallstones
Bile is composed of a complex solution of bilirubin
(the byproduct of effete red blood cells), cholesterol,
fatty acids and various minerals. If one or more of
the major components is present in excess, then the
solution becomes supersaturated and cholesterol
crystals form within the bile (Fig. 11.2). These
eventually coalesce to form cholesterol or ‘mixed’
(cholesterol/bilirubin) gallstones. Cholesterol
supersaturation can result from either excessive
hepatic secretion of cholesterol, or decreased
hepatic secretion of bile salts or phospholipids
with relatively normal cholesterol secretion. In
>90% of patients, supersaturation results from
altered hepatic cholesterol metabolism.
stones to form there is a need for a nidus, and
mucin that is secreted by the gallbladder wall
may serve as a nidus and act as a pro-nucleating
(crystallisation-promoting) protein. Variations in
mucin composition and decreased degradation of
mucin by lysosomal enzymes are associated with a
higher incidence of stone formation.
Loss of gallbladder motility and excessive
sphincteric contraction are also associated with
gallstone formation (Fig.11.3). Hypomotility leads to
prolonged bile stasis (delayed gallbladder emptying)
and decreased reservoir function. If the situation
persists for long enough, crystals coalesce with
formation of biliary sludge and subsequently stones.
Patients with Crohn’s disease, or who have
undergone intestinal resection or total colectomy, are
also more prone to develop cholesterol stones. This
is due to impaired enterohepatic circulation leading
to reduced hepatic secretion of bile salts in the bile
(Fig. 11.4). This results in higher concentration
2,3
For
4
5
180
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Spiral valves of
the cystic duct
Neck/infundibulum
Hartmann’s pouch
Body
Fundus
Common
Duodenal
papillae
Ampulla of Vater
100
0
Percent bile salt
causes crystallisation
precipitation of salts
Mucin acts as a nidus
hepatic duct
Bile duct
Pancreatic
duct
Pancreas
Gallstones
for crystal formation
Stone
formation
Figure11.1 • Anatomy of the gallbladder and bile ducts.
Figure11.2 • Triangular coordinate equilibrium phase
diagram of the cholesterol–phospholipid–bile salt system
in the gallbladder. Bile composition at point P (normal,
non-lithogenic bile); bile salts (80%); phospholipids acids
(15%); cholesterol 5%.
and decreased solubilisation of cholesterol and
its precipitation as crystals, with eventual stone
formation.
Risk factors
As with most diseases, the development of
gallstones is caused by a mixture of genetic and
environmental factors. Patients with cholelithiasis
often have a strong family history, with gallstones
occurring three times more frequently in first-degree
relatives than in spouses or unrelated controls.
has been estimated that genetic factors account for
80
60
Super saturated
cholesterol crystals
and micellar fluid
40
Percent cholesterol
20
Normal bile
0
100 80 60
P
Duodenum
20
Percent phosphatidylcholine
40
60
80
40 20 0
100
6
Supersaturation
Figure11.3 • Components required for gallstone
formation.
approximately 25% of gallstones.
Hypomotility allows
7
Gallstones are
most common in white European and American
populations and least common in black Africans
(Fig. 11.5). Intermediate rates are found in Asian
populations. The highest prevalence is seen in native
American populations with a prevalence of 60% in
the Pima Indian population of Southern Arizona.
Female gender (10:1 female to male ratio), previous
pregnancy and a family history of gallstone disease
are highly correlated with cholelithiasis (Box11.1).
Oestrogen increases cholesterol secretion and diminishes bile salt secretion, increasing the cholesterol
saturation within bile. Diminished gallbladder motility is commonly seen during pregnancy, with a 10–15
times higher incidence of cholelithiasis seen in women
who have had children.
5–30% of pregnant women and definitive gallstones
become established in 5%.
8
Biliary sludge is found in
9
A number of disease processes can result in the
supersaturation of cholesterol in bile, including
rapid weight loss in the morbidly obese patient
(due to excess cholesterol within the bile), total
parenteral nutrition (which induces gallbladder
hypomotility in the presence of high lipid levels),
and drugs that promote cholesterol secretion into
the bile, e.g. fibrates.
Other risk factors include a high dietary intake of
fats and carbohydrates, a sedentary lifestyle, type
2 diabetes mellitus and dyslipidaemia (increased
triglycerides and low HDL). A diet high in fats and
carbohydrates predisposes a patient to obesity, which
increases cholesterol synthesis, biliary secretion of
cholesterol, and cholesterol supersaturation. Patients
It
with a BMI >45 have a 7-fold higher incidence
of gallstones compared with non-obese women.
8
10
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