2. SURGICAL
ANATOMY
1.Gallbladder
It is a pear-shaped structure, 7.5– 12 cm long, with a normal
capacity of about 25–30 mL.
The anatomical divisions are a fundus, a body and a neck that
terminates in a narrow infundibulum.The muscle fibres in the wall
of the gallbladder are arranged in a criss-cross manner, being
particularly well developed in its neck.The mucous membrane
contains indentations of the mucosa that sink into the muscle
coat; these are the crypts of Luschka.
3. SURGICAL
ANATOMY
1.Gallbladder
The gallbladder is entirely surrounded by peritoneum, and is in
direct relation to the visceral surface of the liver.
It lies in close proximity to the following structures:
1. Anteriorly and superiorly – inferior border of the liver and the
anterior abdominal wall.
2. Posteriorly – transverse colon and the proximal duodenum.
3. Inferiorly – biliary tree and remaining parts of the duodenum.
7. SURGICAL
ANATOMY
2.Cystic Duct
The cystic duct is about 3 cm in length but the length is variable.
The lumen is usually 1–3 mm in diameter.The mucosa of the cystic
duct is arranged in spiral folds known as the valves of Heister and
the wall is surrounded by a sphincteric structure called the
sphincter of Lütkens.
The cystic duct joins the supraduodenal segment of the common
hepatic duct in 80% of cases, however the anatomy may vary and
the junction may be much lower in the retroduodenal or even
retropancreatic part of the bile duct
9. SURGICAL
ANATOMY
3.Common
Bile Duct
The common hepatic duct is usually less than 2.5 cm long and is
formed by the union of the right and left hepatic ducts.
The common bile duct is about 7.5 cm long and formed by the
junction of the cystic and common hepatic ducts. It is divided into
four parts:
1. the supraduodenal portion, about 2.5 cm long, runs in the free
edge of the lesser omentum;
2. the retroduodenal portion;
3. the infraduodenal portion lies in a groove, but at times in a
tunnel, on the posterior surface of the pancreas;
4. the intraduodenal portion passes obliquely through the wall of
the second part of the duodenum, where it is surrounded by
the sphincter of Oddi, and terminates by opening on the
summit of the ampulla ofVater.
10. The Biliary
Tree
The biliary tree is a series of gastrointestinal ducts allowing newly
synthesized bile from the liver to be concentrated and stored in
the gallbladder (prior to release into the duodenum).
Bile is initially secreted from hepatocytes and drains from both
lobes of the liver via canaliculi, intralobular ducts and collecting
ducts into the left and right hepatic ducts.These ducts
amalgamate to form the common hepatic duct, which runs
alongside the hepatic vein.
11. The Biliary
Tree
As the common hepatic duct descends, it is joined by the cystic
duct – which allows bile to flow in and out of the gallbladder for
storage and release.At this point, the common hepatic duct and
cystic duct combine to form the common bile duct.
The common bile duct descends and passes posteriorly to the
first part of the duodenum and head of the pancreas. Here, it is
joined by the main pancreatic duct, forming the
hepatopancreatic ampulla (commonly known as the ampulla of
Vater) – which then empties into the duodenum via the major
duodenal papilla.This papilla is regulated by a muscular valve, the
sphincter of Oddi.
15. Vasculature
ofGallbladder
The arterial supply to the gallbladder is via the cystic artery – a
branch of the right hepatic artery (which itself is derived from the
common hepatic artery, one of the three major branches of the
coeliac trunk).
Venous drainage of the neck of the gallbladder is via the cystic
veins, which drain directly into the portal vein.
Venous drainage of the fundus and body of the gallbladder flows
into the hepatic sinusoids.
18. Calot’s
triangle, or the
hepatobiliary
triangle
It was initially described by Calot as the space bordered by the
cystic duct inferiorly, the common hepatic duct medially and the
superior border of the cystic artery.
This has been modified in contemporary literature to be the area
bound superiorly by the inferior surface of the liver, laterally by the
cystic duct and the medial border of the gallbladder and medially
by the common hepatic duct.
21. Lymphatics
The lymphatic vessels of the gallbladder (subserosal and
submucosal) drain into the cystic lymph node of Lund (the
sentinel lymph node), which lies in the fork created by the junction
of the cystic and common hepatic ducts.
Efferent vessels from this lymph node go to the hilum of the liver,
and to the coeliac lymph nodes.
The subserosal lymphatic vessels of the gallbladder also connect
with the subcapsular lymph channels of the liver, and this accounts
for the frequent spread of carcinoma of the gallbladder to the
liver.
22. Surgical
physiology
Bile is produced by the liver and stored in the gallbladder, from
which it is released into the duodenum.
As it leaves the liver it is composed of 97% water, bile salts (cholic
and chenodeoxycholic acids, deoxycholic and lithocholic acids),
phospholipids, cholesterol and bilirubin.
The liver excretes bile at a rate estimated to be approximately 40
mL/hour.
About 95% of bile salts are reabsorbed in the terminal ileum
(enterohepatic circulation).
23. Functions of
the gallbladder
1. Areservoir for bile. During fasting, resistance to flow through
the sphincter of Oddi is high, and bile excreted by the liver is
diverted to the gallbladder.After feeding, the resistance to
flow through the sphincter is reduced, the gallbladder contracts
and the bile enters the duodenum.These motor responses of
the biliary tract are in part affected by the hormone
cholecystokinin.
2. Concentration of bile by active absorption of water, sodium
chloride and bicarbonate via the mucous membrane of the
gallbladder.The hepatic bile which enters the gallbladder
becomes concentrated 5–10 times, with a corresponding
increase in the proportion of bile salts, bile pigments,
cholesterol and calcium.
3. Secretion of mucus – approximately 20 mL is produced per day.
With complete obstruction of the cystic duct in an otherwise
healthy gallbladder, a mucocoele may develop as a result of
ongoing mucus secretion by the gallbladder mucosa.
24. RADIOLOGICAL
INVESTIGATION
OFTHE BILIARY
TRACT
1.Xray Plain radiograph showing
radiopaque stones in the
gallbladder.
Radiopaque stones are rare
(10%).
Porcelain
gallbladder.
Gas in the gallbladder
and gallbladder wall
(Clostridium
perfringens).
Emergency surgery is
indicated.
25. RADIOLOGICAL
INVESTIGATION
OFTHE BILIARY
TRACT
2.
Ultrasonography
Transabdominal ultrasonography is the initial imaging modality
of choice as it is accurate, readily available, inexpensive and quick
to perform. However, it is operator dependent and may be
compromised by excessive body fat and intraluminal bowel gas.
The size of the gallbladder can be seen, the presence of stones or
polyps determined and the thickness of the wall measured.
Additionally, the presence of inflammation around the
gallbladder, the size of the common bile duct and, occasionally,
the presence of stones within the extrahepatic biliary tree can be
determined.
For the patient who presents with obstructive jaundice, it can
identify intra- and extrahepatic biliary dilatation and often the
level of obstruction. In addition, the cause of the obstruction may
also be determined, such as gallstones in the gallbladder,
common hepatic or common bile duct stones, lesions within the
wall of the common bile duct suggestive of a cholangiocarcinoma
or enlargement of the pancreatic head indicative of a pancreatic
carcinoma.
27. RADIOLOGICAL
INVESTIGATION
OFTHE BILIARY
TRACT
2.
Ultrasonography
Endoscopic ultrasonography (EUS) utilises a specially designed endoscope
with an ultrasound transducer at its tip which allows the gastroenterologist to
visualize the liver and biliary tree from within the stomach and duodenum.
It is accurate in imaging the bile duct and detecting the presence of
choledocholithiasis. In addition, it has been shown to be useful in diagnosing
and staging both pancreatic and periampullary cancers. Biopsies can be taken
from suspicious areas for either cytological or histopathological analysis.
28. 3. Cholescintigraphy
Technetium-99m (99mTc)-labelled derivatives of iminodiacetic acid
(HIDA, IODIDA) when injected intravenously are selectively taken
up by the retroendothelial cells of the liver and excreted into the
bile. This allows visualization of the biliary tree and gallbladder. In
90% of normal individuals the gallbladder is visualized within
30 minutes following injection, with 100% being seen within 1
hour.
The bowel is seen, usually within 1 hour, in the majority of
patients. Non- visualization of the gallbladder is suggestive of
acute cholecystitis. If the patient has a contracted gallbladder,
as often seen in chronic cholecystitis, the gallbladder visualization
may be reduced or delayed.
An abnormally low gallbladder ejection fraction may be suggestive
of gallbladder dyskinesia; however, the diagnosis and
interpretation of cholescintigraphy in this context are
controversial.
Biliary scintigraphy may also be helpful in diagnosing bile leaks
and iatrogenic biliary obstruction.
29. 4.Computed
tomography
(CT)
CT is less affected by body habitus
and is not operator dependent. It
allows visualisation of the liver, bile
ducts, gallbladder and pancreas.
It is particularly useful in detecting
hepatic and pancreatic lesions and
is the modality of choice in the
staging of cancers of the liver,
gallbladder, bile ducts and
pancreas. It can identify the extent
of the primary tumour and define
the relationship of the tumour to
other organs and blood vessels.
In addition, the presence of
enlarged lymph nodes or
metastatic disease may be seen.
However, as only 75% of gallstones
are identified by CT, it is not used
as a screening modality for
uncomplicated gallstones.
30. 5. Endoscopic
retrograde
cholangiopancreato
graphy (ERCP)
Using a side-viewing endoscope the
ampulla ofVater can be identified and
cannulated. Injection of water-soluble
contrast directly into the bile duct
provides excellent images of the ductal
anatomy and can identify causes of
obstruction such as calculi or malignant
strictures.
It is especially useful in determining
the cause and level of obstruction.
During ERCP, bile aspirates can be
sent for cytological and
microbiological examination, and
endoluminal brushings can be taken
from strictures for cytological
studies.
Therapeutic interventions such as
stone removal or stent placement to
relieve the obstruction can be
performed.
31. 6. Magnetic
resonance
cholangiopanc
reatography
(MRCP)
Images can be obtained
of the biliary tree
demonstrating ductal
obstruction, strictures or
other intraductal
abnormalities.
Images comparable to
those obtained using
ERCP or PTC can be
achieved non-invasively
without the potential
complications of either
technique.
By use of the water
content of bile, a
cholangiopancreatogram
can be created, which
makes it an excellent
modality for cross-
sectional imaging of the
biliary tree.
32. 7. Percutaneous
transhepatic
cholangiography
(PTC)
Under radiological control (either
ultrasound or CT) a bile duct is cannulated.
Successful entry is confirmed by contrast
injection or aspiration of bile.Water-
soluble contrast medium is injected to
visualise the biliary system. Multiple
images can be taken demonstrating areas
of strictures or obstruction. Bile can be
sent for cytology.
In addition, this technique enables
placement of a catheter into the bile ducts
to provide external biliary drainage or the
insertion of indwelling stents.The scope
of this procedure can be further extended
by leaving the drainage catheter in situ for
a number of days and then dilating the
track sufficiently for a fine flexible
choledochoscope to be passed into the
intrahepatic biliary tree in order to
diagnose strictures, take biopsies and
remove stones
In general, if a malignant stricture at the
level of the confluence of the right and left
hepatic ducts or higher is suspected in a
jaundiced patient, a PTC is preferred to
ERCP because successful drainage is more
likely.
33. CONGENITAL
ABNORMALITIES
OFTHE
GALLBLADDERAND
BILE DUCTS
Absence of the gallbladder
The Phrygian cap
Floating gallbladder
Absence of the cystic duct
usually a pathological, as opposed to an anatomical, anomaly and
indicates the recent passage of a stone or the presence of a stone at
the lower end of the cystic duct, which is ulcerating into the
common bile duct.The main danger at surgery is damage to the bile
duct, and particular care to identify the correct anatomy is essential
before division of any duct.
Low insertion of the cystic duct
An accessory cholecystohepatic duct
34. EXTRAHEPATIC
BILIARY
ATRESIA
The extrahepatic bile ducts are progressively destroyed by an
inflammatory process which starts around the time of birth.
The inflammatory destruction of the bile ducts has been
classified into three main types:
type I: atresia restricted to the common bile duct;
type II: atresia of the common hepatic duct;
type III: atresia of the right and left hepatic ducts.
Associated anomalies occur in about 20% of cases and include
cardiac lesions, polysplenia, situs inversus, absent vena cava and a
preduodenal portal vein.
35.
36. EXTRAHEPATIC
BILIARY
ATRESIA
Clinical features
About one-third of patients are jaundiced at birth. In all, however,
jaundice is present by the end of the first week and deepens
progressively. Liver function tests show an obstructive pattern
with elevated bilirubin and alkaline phosphatase.
The meconium may be a little bile stained, but later the stools are
pale and the urine is dark. Prolonged steatorrhoea gives rise to
osteomalacia (biliary rickets). Pruritus is severe.
Clubbing and skin xanthomas, probably related to raised serum
cholesterol, may be present.
37. Treatment
A simple biliary–enteric anastomosis is not possible in the majority
of cases in which the proximal hepatic ducts are either very small
(type II) or atretic (type III).
These are treated by the Kasai procedure, in which radical
excision of all bile duct tissue up to the liver capsule is performed.
A Roux-en-Y loop of jejunum is anastomosed to the exposed area
of liver capsule above the bifurcation of the portal vein, creating a
portoenterostomy.
The chances of achieving effective bile drainage after
portoenterostomy are maximal when the operation is performed
before the age of 8 weeks, and approximately 90% of children
whose bilirubin falls to within the normal range can be expected to
survive for 10 years or more.
38. Treatment
Early referral for surgery is critical. Postoperative complications
include bacterial cholangitis, which occurs in 40% of patients.
Repeated attacks lead to hepatic fibrosis, and 50% of long-term
survivors develop portal hypertension, with one-third having
variceal bleeding.
Liver transplantation should be considered in children in whom a
portoenterostomy is unsuccessful.
Results are improving, with 70–80% alive 2–5 years following
transplant
39. CONGENITAL
DILATATIONOF
THE
INTRAHEPATIC
DUCTS
(CAROLI’S
DISEASE)
This rare congenital condition is characterised by multiple
irregular saccular dilatations of the intrahepatic ducts, separated
by segments of normal or stenotic ducts, with a normal
extrahepatic biliary system.
In Caroli’s syndrome, the biliary dilatation is associated with
congenital hepatic fibrosis
40. CONGENITAL
DILATATIONOF
THE
INTRAHEPATIC
DUCTS
(CAROLI’S
DISEASE)
The presentation is varied, with many patients presenting with
abdominal pain, cholangitis or end-stage liver disease.
The majority of patients present before the age of 30.
Sex distribution is equal.
Management is multidisciplinary: cholangitis or jaundice are
treated with appropriate antibiotic therapy and endoscopic or
interventional stenting.
Malignancy is a complication of long- standing disease.
42. CHOLEDOCHAL
CYST
Choledochal cysts are congenital dilations of the intra and/or
extrahepatic biliary system.The pathogenesis is unclear.
Anomalous junctions of the biliary pancreatic junction are
frequently observed, but whether or not these play a role in the
pathogenesis of the condition is unclear.
43. Symptoms
Patients may present at any age with jaundice, fever, abdominal
pain and a right upper quadrant mass on examination; Pancreatitis
is not an infrequent presentation in adults.
Patients with choledochal cysts have an increased risk of
developing cholangiocarcinoma with the risk varying directly with
the age at diagnosis.
44. Investigations
Ultrasonography will confirm the presence of an abnormal cyst
and magnetic resonance imaging (MRI/MRCP) will reveal the
anatomy, in particular the relationship between the lower end of
the bile duct and the pancreatic duct. CT is also useful for
delineating the extent of the intra- or extrahepatic dilatation.
45. Classification oftypes of
choledochalcyst.TypeIa
andb: diffusecystic. Note
extension into pancreasof
type Ib.Type
II: diverticulum ofcommon
bile duct.Type III:
diverticulum within
pancreas.
Type IV: extension into the
liver.TypeV: cystic
dilatation only of
the intrahepatic ducts.
46. Treatment
Radical excision of the cyst is the treatment of choice, with
reconstruction of the biliary tract using a Roux-en-Y loop of
jejunum. Complete resection of the cyst is important because of
the association with the development of cholangiocarcinoma.
Resection and roux-en-Y reconstruction are also associated with a
reduced incidence of stricture formation and recurrent cholangitis.
47. TRAUMA
Iatrogenic injury is perhaps more frequent than external trauma.
The physical signs are those of an acute abdomen. Management
depends on the location and extent of the biliary and associated
injury. In the stable patient a transected bile duct is best repaired
by a Roux-en-Y choledochojejunostomy.
Injuries to the gallbladder can be dealt with by cholecystectomy
48. TORSIONOF
THE
GALLBLADDER
This is very rare and requires a long mesentery, and therefore
often occurs in an older patient with a large mucocoele of the
gallbladder.The patient presents with extreme pain and an acute
abdomen. Immediate exploration is indicated, with
cholecystectomy as the only treatment.
49. GALLSTONES
(CHOLELITHIASIS)
Gallstones are the most common biliary pathology.
Gallstones can be divided into three main types: cholesterol,
pigment (brown/black) or mixed stones.
Pigment stone is the name used for stones containing <30%
cholesterol.There are two types: black and brown. Black stones
are largely composed of an insoluble bilirubin pigment polymer
mixed with calcium phosphate and calcium bicarbonate.
Overall, 20–30% of stones are black.The incidence rises with age.
Black stones are associated with haemolysis, usually hereditary
spherocytosis or sickle cell disease.
Brown pigment stones contain calcium bilirubinate, calcium
palmitate and calcium stearate, as well as cholesterol. Brown
stones are rare in the gallbladder.They form in the bile duct and
are related to bile stasis and infected bile.
51. Clinical
presentation
Gallstones may remain asymptomatic, being detected incidentally
as imaging is performed for other symptoms. If symptoms occur,
patients typically complain of right upper quadrant or epigastric
pain, which may radiate to the back.
This may be described as colicky but more often is dull and
constant. Other symptoms include dyspepsia, flatulence, food
intolerance particularly to fats and some alteration in bowel
frequency. Biliary colic is typically present in 10–25% of patients.
This is described as a severe right upper quadrant pain which ebbs
and flows, associated with nausea and vomiting.
Pain may radiate to the chest.The pain is usually severe and may
last for minutes or even several hours. Frequently, the pain starts
during the night and wakes the patient. Minor episodes of the
same discomfort may occur intermittently during the day.
Dyspeptic symptoms may coexist and be worse after such an
attack.
54. Diagnosis
In the acute phase the patient may have right upper quadrant
tenderness that is exacerbated during inspiration by the
examiner’s right subcostal palpation (Murphy’s sign).A positive
Murphy’s sign suggests acute inflammation and may be
associated with a leukocytosis and moderately elevated liver
function tests. A mass may be palpable as the omentum walls off
an inflamed gallbladder.
While the presentation and examination may suggest acute
cholecystitis, a definitive diagnosis can only be made following
appropriate imaging studies (US or CT).
55. Treatment
Prophylactic cholecystectomy may be considered for diabetic
patients, those with congenital haemolytic anaemia and those
patients who are undergoing bariatric surgery for morbid obesity
because it has been found in these groups that the risk of
developing symptoms is increased.
For patients with symptomatic gallstones, cholecystectomy is the
treatment of choice if there are no medical contraindications.
Experience shows that, in more than 90% of cases, the symptoms
of acute cholecystitis subside with conservative measures.
56. Treatment
Non-operative treatment is based on four principles:
●● Nil per mouth (NPO) and intravenous fluid administration until
the pain resolves.
●● Administration of analgesics.
●● Administration of antibiotics. As the cystic duct is blocked in most
instances, the concentration of antibiotic in the serum is more
important than its concentration in bile. A broad-spectrum antibiotic
effective against gram-negative aerobes is most appropriate (e.g.
cefazolin, cefuroxime or ciprofloxacillin).
●● Subsequent management. When the temperature, pulse and
other physical signs show that the inflammation is subsiding, oral
fluids are reinstated, followed by a regular diet..
57. Treatment
Ultrasonography is performed to confirm the diagnosis.
If jaundice is present MRCP is performed to exclude
choledocholithiasis.
If there is any concern regarding the diagnosis or presence of
complications such as perforation CT should be performed.
Cholecystectomy may be performed on the next available list, or the
patient may be allowed home to return later when the inflammation
has completely resolved
59. EMPYEMAOF
THE
GALLBLADDER
Empyema may be a sequel to acute cholecystitis or the result of a
mucocoele becoming infected. The gallbladder is distended with
pus. The optimal treatment is drainage (cholecystostomy) and, later,
cholecystectomy.
60. Acalculous
cholecystitis
Acute acalculous cholecystitis is particularly seen in critically ill
patients and those recovering from major surgery, trauma and
burns.
The diagnosis is often missed and the mortality rate is high.
61. THE
CHOLECYSTOSES
(CHOLESTEROSIS,
POLYPOSIS,
ADENOMYOMATO
SISAND
CHOLECYSTITIS
GLANDULARIS
PROLIFERANS)
Cholesterosis (‘strawberry gallbladder’)
In the fresh state, the interior of the gallbladder looks something like a
strawberry in patients with this condition; the yellow specks
(submucous aggregations of cholesterol crystals and cholesterol esters)
correspond to the seeds. It may be associated with cholesterol stones.
Cholesterol polyposis of the gallbladder
Ultrasound may show a non-mobile defect in the gallbladder lumen
which does not exhibit an associated acoustic shadow.The differential
is an adenomatous polyp, and interval follow-up is indicated to ensure
stability. Surgery is only advised if there is a diagnostic dilemma.
Cholecystitis glandularis proliferans (polyp, adenomyomatosis and
intramural diverticulosis)
A polyp of the mucous membrane is fleshy and granulomatous.All
layers of the gallbladder wall may be thickened, but sometimes an
incomplete septum forms that separates the hyperplastic from the
normal. Intraparietal ‘mixed’ calculi may be present.These can be
complicated by an intramural, and later extramural, abscess and
potentially fistula formation. If symptomatic, the patient is treated by
cholecystectomy
63. Diverticulosis
of the
gallbladder
Diverticulosis of the gallbladder is
usually manifest as black pigment
stones impacted in the
outpouchings of the lacunae of
Luschka. Diverticulosis of the
gallbladder may be demonstrated
by cholecystography, especially
when the gallbladder contracts
after a fatty meal.There are small
dots of contrast medium just
within and outside the gallbladder.
A septum may also be present, to
be distinguished from the
Phrygian cap.
The treatment is cholecystectomy
64. Typhoid
infection of the
gallbladder
Salmonella typhi or Salmonella typhimurium can infect the
gallbladder.
Acute cholecystitis can occur.
More frequently chronic cholecystitis occurs, the patient
becoming a typhoid carrier excreting the bacteria in the bile.
Gallstones may be present (surgeons should not give patients
their stones after their operation if there is any suspicion of
typhoid!).
It is debatable whether the stones are secondary to the
Salmonella cholecystitis or whether pre-existing stones
predispose the gallbladder to chronic infection. Salmonellae can,
however, frequently be cultured from these stones.
Treatment with ampicillin and cholecystectomy are indicated. In
cases of penicillin allergy a quinolone antibiotic can be used.
65. Stricture of the
bile duct
Causes of benign biliary stricture
Congenital
●● Biliary atresia
Bile duct injury at surgery
●● Cholecystectomy
●● Choledochotomy
●● Gastrectomy
●● Hepatic resection
●● Transplantation
Inflammatory
●● Stones
●● Cholangitis
●● Parasitic
●● Pancreatitis
●● Sclerosing cholangitis
●● Radiotherapy
Trauma
Idiopathic
66. Stricture of the
bile duct
Radiological investigation of biliary strictures
●● Ultrasonography
●● Cholangiography via T-tube, if present
●● ERCP
●● MRCP
●● Percutaneous transhepatic cholangiography
●● CT scan
67. PRIMARY
SCLEROSING
CHOLANGITIS
Primary sclerosing cholangitis is an idiopathic fibrosing
inflammatory condition of the biliary tree that affects both
intrahepatic and extrahepatic ducts.
It is of unknown origin but the association of
hypergammaglobulinaemia and elevated markers such as smooth
muscle antibodies and antinuclear factor suggest an
immunological basis.
The majority of patients are between 30 and 60 years of age.
There appears to be a male predominance and a strong
association with inflammatory bowel disease, especially
ulcerative colitis.
68. PRIMARY
SCLEROSING
CHOLANGITIS
Common symptoms include right upper quadrant discomfort,
jaundice, pruritus, fever, fatigue and weight loss.
Investigation reveals a cholestatic pattern to the liver function
tests with elevation of the serum alkaline phosphatase and γ-
glutamyl transferase and smaller rises in the aminotransferases.
Bilirubin values can be variable and may fluctuate.
Imaging studies such as MRCP or ERCP may demonstrate
stricturing and beading of the bile ducts .
A liver biopsy is helpful to confirm the diagnosis and may help
guide therapy by excluding cirrhosis.
The important differential diagnoses are secondary sclerosing
cholangitis and cholangiocarcinoma.
The latter may be very difficult to diagnose and a high index of
suspicion is required especially in the setting of unexplained
clinical deterioration.
69. PRIMARY
SCLEROSING
CHOLANGITIS
Medical management with antibiotics, vitamin K, cholestyramine,
steroids and immunosuppressant drugs such as azathioprine is
generally unsuccessful.
Endoscopic stenting of dominant strictures and, in selected
patients with predominantly extrahepatic disease, operative
resection may be worthwhile.
For patients with cirrhosis, liver transplantation is the best option.
Five-year survival following transplantation in high-volume
centres is in excess of 80%.
70. Immunoglobul
in (Ig)G4-
related
cholangitis
This recently recognised entity presents with diffuse or segmental
narrowing of the intra- or extrahepatic bile ducts.
Its features may make differentiation from primary sclerosing
cholangitis (PSC), cholangiocarcinoma or pancreatic cancer
difficult.
However, patients often have elevated serum IgG4 levels and
concomitant autoimmune pancreatitis, IgG4- related sialadenitis
or retroperitoneal fibrosis.
Biliary biopsies show lymphoplasmacytic sclerosing cholangitis.
Treatment is with systemic steroids.
Failure to respond to steroid therapy should make one reconsider
the diagnosis and exclude an underlying malignancy.
71. Biliary
ascariasis
The roundworm Ascaris lumbricoides commonly infests the
intestines of inhabitants ofAsia, Africa and Central America.
It may enter the biliary tree through the ampulla ofVater and
cause biliary pain.
Complications include strictures, suppurative cholangitis, liver
abscesses and empyema of the gallbladder.
In the uncomplicated case, antispasmodics can be given to relax
the sphincter of Oddi and the worms will return to the small
intestine to be dealt with by anthelminthic drugs.
Operation may be necessary to remove the worms or deal with
complications.
Worms can be extracted via the ampulla ofVater by ERCP.
72. Clonorchiasis
(Asiatic
cholangiohepa
tis)
This disease is endemic in the Far East.The fluke, up to 25 mm
long and 5 mm wide, inhabits the bile ducts, including the
intrahepatic ducts.
Fibrous thickening of the duct walls occurs.
Many cases are asymptomatic.
Complications include biliary pain, stones, cholangitis, cirrhosis
and bile duct carcinoma.
Choledochotomy andT-tube drainage and, in some cases,
choledochoduodenostomy are required.
Because a process of recurrent stone formation is set up, a
choledochojejunostomy with a Roux loop fixed to the adjacent
abdominal wall is performed in some centers to allow easy
subsequent access to the duct system.
73. Hydatid
disease
A large hydatid cyst may obstruct the hepatic ducts. Sometimes,
a cyst will rupture into the biliary tree and its contents
cause obstructive jaundice or cholangitis, requiring appropriate
surgery
74. Benign
tumours of the
bile duct
Benign neoplasms causing biliary obstruction may be
classified as follows:
●● papilloma and adenoma;
●● multiple biliary papillomatosis;
●● granular cell myoblastoma;
●● neural tumours;
●● leiomyoma;
●● endocrine tumours.
75. Malignant
tumours of the
biliary
tract
Bile duct cancer (cholangiocarcinoma)
●● Rare, but incidence increasing
●● Most patients present with abnormal liver function tests or
frank jaundice
●● Diagnosis by ultrasound, CT or MRCP scanning
●● The majority of patients receive palliative care only
●● Complete surgical excision possible in <10%
●● Prognosis poor: 90% die within 1 year, from liver failure or
biliary sepsis
●● Adjuvant chemoradiation therapy has a limited role
77. Clinical
features
Early symptoms of cholangiocarcinoma are often non-specific,
with abdominal pain, early satiety, anorexia and weight loss
commonly seen.
Symptoms associated with biliary obstruction (puritus and
jaundice) may be present in a minority of patients. In these
patients, examination often demonstrates clinical signs of
jaundice, cachexia is often noticeable and a palpable gallbladder
present if the obstruction is in the distal common bile duct
(Courvoisier’s sign).
78. Investigations
Biochemical
investigations will
confirm the presence of
obstructive jaundice
(elevated bilirubin,
alkaline phosphatase and
γ-glutamyltransferase).
The tumour-marker CA
19-9 may also be
elevated.
Imaging studies such as
ultrasound, MDR-CT and
MRI/MRCP are essential
for diagnosis and staging.
These studies allow the
level of biliary
obstruction to be defined
and determine the
locoregional extent of
disease and the presence
of metastases
79. Investigations
Direct cholangiography using ERCP or PTC is also used following
non-invasive studies. Both can define the level of obstruction and
allow access to the biliary system for biopsy and placement of
endobiliary stents for biliary drainage.The choice between the
modalities depends on local availability and the anatomical site of
the tumour.
PTC preferred for more proximal lesions and ERCP favoured for
distal tumours.
Cytology can be obtained from either procedure but it is often
non-diagnostic.
Positron emission tomography (PET) is useful in detecting lymph
node and distant metastases but has limited value in assessment
of local resectability.
81. Treatment
A multidisciplinary approach is required in all cases.The choice of
treatment depends on the site and extent of the disease.
Unfortunately, the majority of patients present with advanced
disease.
Whether or not the disease is resectable depends on patient factors
(comorbidities, presence or absence of chronic liver disease), and
tumour factors (extent of disease within the biliary tree, vascular
involvement, presence or absence of metastatic disease). Depending
on the site of disease, surgery may involve either a standard or
extended hepatic resection with en bloc lymphadenectomy and
reconstruction of the biliary tree. Distal common duct tumours may
require a pancreaticoduodenectomy (Whipple procedure). Local
resection should be avoided.
In selected patients, liver transplantation has been recommended for
those with locally unresectable disease without evidence of distant
metastases.Transplantation is often combined with neoadjuvant
chemoradiation therapy.
Biliary obstruction can be relieved by either endoscopic (ERCP) or
percutaneous (PTC) methods. Surgical bypass rarely has a role apart
from in patients who present with a distal bile duct lesion and are
found to have unresectable disease at operation.
82. Cancer of the
gallbladder
Highest incidence is among Chileans,American Indians and
residents in parts of northern India.
●● Similar presentation to benign biliary disease i.e., gallstones
●● Diagnosis by ultrasound, CT, MRI/MRCP
●● Most patients present with advanced disease
●● Surgical resection in less than 10% – remainder receive
palliative treatment
●● Prognosis is poor – median survival approximately 6 months
Patients may be asymptomatic at the time of diagnosis.
Jaundice and anorexia are late features. A palpable mass is a late
sign.
83. Investigation
Serum CA19-9 is elevated in approximately 80% of patients.
The preoperative diagnosis is often made on ultrasonography, and
confirmed by a CT scan or MRI/MRCP.
Preoperative staging should aim to determine the local extent of
disease and exclude the presence of distant metastases.
A percutaneous biopsy under radiological guidance is often done
to obtain tissue for pathological examination.
In selected cases, laparoscopic examination is useful in staging the
disease. Laparoscopy can detect peritoneal or liver metastases
which would preclude further surgical resection
PET scanning also has a role in detecting metastatic disease.
84. Aims of
staging
gallbladder
cancer
●● Assessment of local disease
●● Detection of metastatic disease:
Liver
Peritoneal
Lymphatic
Extra-abdominal disease
85. Treatment and
prognosis
Cholecystectomy should be performed for all gallbladder polyps
greater than 1 cm.
Polyps less than 1 cm can be followed with serial ultrasonography
to detect any change in size or character as the incidence of
malignancy in polyps less than 1 cm is extremely low.
Radical en bloc resection that may include segmental or extended
hepatectomy, bile duct resection and regional lymphadenectomy
should be considered in selected patients.
86. Treatment and
prognosis
The aim is to remove the tumour entirely and achieve negative
histopathological margins.
Patients can have the disease diagnosed following
histopathological examination of the gallbladder removed for
presumed benign disease.
In these cases, the need for further surgery is determined by the
stage of disease.
For early-stage disease, confined to the mucosa or muscle of the
gallbladder, no further treatment is indicated.
87. Treatment and
prognosis
However, for transmural disease, a radical en bloc resection of the
gallbladder fossa and surrounding liver along with the regional
lymph nodes should be performed.
If the initial procedure was performed laparoscopically, the
surgeon should examine the laparoscopic port sites.
Routine resection of port sites is no longer recommended.
However, it is recognised that the finding of disease at the port
sites is a sign of generalised peritoneal disease and carries a very
poor prognosis.