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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 2years postsplenectomy is estimated at 0.9% for adults and 5% for children.
24
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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
Figure10.3 • 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
Figure10.4 • 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
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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 12months 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. MooreEE, ShackfordSR, PachterHL, etal. 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. KuterDJ, RummelM, BocciaR, etal. 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.
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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
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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
Figure11.1 • Anatomy of the gallbladder and bile ducts.
Figure11.2 • 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
Figure11.3 • 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 (Box11.1). 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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