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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_40_библиотеки_им_акад_М_И_Перельмана
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CHAPTER 4 | BILIARYDISORDERS
make a denitive diagnosis of ADM using ultrasound alone. ADM theoretically requires no specic
treatment, except when symptomatic, with or without gallstones.
Golse N, Lewin M, Rode Aetal. Gallbladder adenomyomatosis:diagnosis and management. J Visc Surg.
207 Oct;54(5):345– 353. Doi:0.06/ j.jviscsurg.207.06.004.
3. A. Cholecystectomy
• Porcelain gallbladder is associated with gallbladder carcinoma
• Spotty mural calcication has much higher risk of malignancy than homogenous complete
calcication
• Current guidelines recommend cholecystectomy for porcelain gallbladder although evidence
is weak
This woman has porcelain gallbladder. Porcelain gallbladder is rare and is detected in less than 0.%
of cholecystectomy specimens. It is associated with the presence of gallstones in 95% of patients,
which are thought to contribute to chronic gallbladder inammation scarring, hyalinization and
calcication. Porcelain gallbladder is associated with an increased risk of gallbladder malignancy but
a poor causal relationship has been established. Patients are usually asymptomatic and the condition
is often discovered incidentally on abdominal imaging. CT can conrm the condition with a high
degree of accuracy.
While early studies described an incidence of carcinoma in calcied gallbladders as high as 20%,
more recent series suggest a far lower rate of ~3%. Spotty mucosal calcication as seen in this case
is associated with a much higher carcinoma rate compared to homogenous wall calcication.
The management of porcelain gallbladder is controversial. European Association for Study of the
Liver (EASL) recommend that asymptomatic patients with porcelain gallbladder may undergo
cholecystectomy although the quality of evidence is very low. However, given that the risk of
carcinoma appears to be lower than previously observed, some experts suggest observation
particularly in those with homogenous wall calcication.
EASL Clinical Practice Guidelines on the prevention, diagnosis and treatment of gallstones. J Hepatol.
206 Jul;65():46– 8. Doi:0.06/ j.jhep.206.03.005.
4. B. ERCP and plastic biliary stent insertion
• Bile leak occurs in <% post- laparoscopic cholecystectomy
• It commonly presents as abdominal pain and leak of bilious uid from wound sites or
surgical drains
• In the absence of peritonism and intrabdominal collections, endoscopic management with
ERCP and plastic biliary stenting is the preferred treatment modality
Bile leak is a well- recognized and serious complication after cholecystectomy, occurring more
commonly after a laparoscopicy (~0.9%) compared with an open approach. The cystic duct
stump is the most common site of leak, followed by the duct of Luschka, CBD, common hepatic
duct, and gallbladder bed. Bile leaks are also reported in the setting of LT with both duct- to- duct
anastomosis and hepatojejunostomy. The most common clinical feature of bile leaks is abdominal
pain and percutaneous leakage of bilious uid either from wound sites or from surgical drains. The
management of bile leaks (not caused by complete CBD transection) usually involves an endoscopic
approach aiming to create a low- pressure ow direction for the bile to drain away from the leak,
which allows it to epithelialize and seal. This is most commonly achieved by endoscopic biliary
sphincterotomy and/ or endoscopic biliary stenting, which leads to bile leak resolution in >90%.
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Choice of treatment at ERCP should be guided on a case- by- case basis. Biliary stenting provides
faster leak resolution than sphincterotomy alone, and it is equally eective whether sphincterotomy
is performed or not. Therefore, in the absence of obstructing biliary lesions (e.g. retained stones),
current European guidelines recommend insertion of a plastic biliary stent without performance of
sphincterotomy, and removal of the stent four to eight weeks later.
Dumonceau JM, Tringali A, Papanikolaou IS. Biliary stenting:indications, choice of stents and
results:European Society of Gastrointestinal Endoscopy (ESGE) clinical guideline. Endoscopy. 208
Sep;50(9):90– 930. Doi:0.055/ a- 0659- 9864.
5. C. ERCP and plastic stent
• ERCP and plastic stent placement without sphincterotomy can be safely performed for biliary
decompression without interruption to antiplatelet therapy
MRCP shows stacked stones in the CBD (Fig. 4.9). While it is safe to continue low- dose aspirin
for all endoscopic therapies (apart from endoscopic submucosal dissection, colonic endoscopic
mucosal resection [EMR] (>2cm), upper gastrointestinal EMR, and ampullectomy), the
management strategy here is complicated by the patient’s clopidogrel. P2Y2 receptor antagonists
(clopidogrel, prasugrel, ticagrelor) increase the risk of bleeding with interventional procedures
but need to be balanced against the risk of coronary stent thrombosis. ERCP for biliary stenting
without sphincterotomy is classied as at low risk of peri- procedural bleeding and represents the
best option in this case, given no improvement on medical therapy and the need for urgent source
control of the sepsis. Percutaneous options are best avoided to reduce the risk of complications
from bleeding. Extracorporeal shock wave lithotripsy can be used for problematic, large CBD
stones as an alternative to surgery but usually requires subsequent ERCP and sphincterotomy to
extract stone fragments. Denitive CBD stone management and a cholecystectomy should be
Fig.4.9 MRCP showing multiple CBD calculi
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planned in this case following discussion with cardiology regarding when it is safe to discontinue the
P2Y2 receptor antagonist.
Veitch AM, Vanbiervliet G, Gershlick AH etal. Endoscopy in patients on antiplatelet or anticoagulant
therapy, including direct oral anticoagulants:BSG and ESGE guidelines. Gut. 206 Mar;65(3):374– 389.
Doi:0.36/ gutjnl- 205- 30.
6. B. ERCP
• Pregnancy should not deter ERCP when it is indicated for cholangitis and systemic
inammatory response syndrome (SIRS)
• Temporary decompression with biliary stenting followed by a completion ERCP after delivery
is often the most appropriate strategy
In this case, there is a high probability of ascending cholangitis requiring either ERCP- directed
stenting or stone extraction. Ultrasound is useful for looking at dilated ducts, but does not always
visualize obstructing stones within the CBD. Patients with ascending cholangitis should undergo
ERCP to relieve the obstruction as soon as possible, particularly those with severe sepsis and SIRS,
as in this case. Pregnancy should not deter one from performing ERCP when it is indicated because,
despite the radiation exposure, in the context of ascending cholangitis, it is life- saving. Positioning of
the patient may be more challenging during ERCP, but is not impossible. Radiation exposure should
be kept to a minimum, and a shield worn to protect the unborn baby. Because of the technical
diculties of performing an ERCP during pregnancy, the denitive aim of duct clearance in CBD
stone disease is often compromised. Temporary decompression with biliary stenting followed by a
completion ERCP after delivery is an appropriate strategy. This also reduces the risk of pancreatitis
at the index ERCP, which may compromise the pregnancy.
Cappell MS. Risks versus benets of gastrointestinal endoscopy during pregnancy. Nat Rev
Gastroenterol Hepatol. 20 Oct 4;8():60– 634. Doi:0.038/ nrgastro.20.
7. A. Biliary microlithiasis
• EUS should be performed to exclude microlithasis in patients with recurrent biliary- type pain
when ultrasound and MRCP have proven non- diagnostic
• MRCP has slightly inferior diagnostic value for microlithiasis than EUS but is more cost
eective
In patients with recurrent biliary- type pain, when ultrasound and MRCP have proven nondiagnostic, an EUS should be considered to exclude microlithiasis before labelling them with a
functional biliary sphincter disorder (formally sphincter of Oddi dysfunction). The MRCP shows
gallbladder calculi (cholelithiasis) but no evidence of CBD stones (choledocholithiasis) (Fig. 4.0).
Cholelithiasis alone does not explain the raised bilirubin. The EUS image shows a 2.7mm CBD
calculi (microlithiasis) (Fig. 4.). This patient should be referred for ERCP and sphincterotomy
followed by a cholecystectomy.
While EUS has a slightly higher diagnostic odds ratio compared with MRCP, the latter has
proven more cost- eective. Therefore, MRCP is often favoured following non- diagnostic
ultrasound, with EUS performed as a third- line investigation when clinical suspicion remains after a
non- diagnostic MRCP.
Fig.4.10 MRCP showing gallbladder calculi
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Fig.4.11 Endoscopic ultrasound image showing CBD microlithiasis
Meeralam Y, Al- Shammari K, Yaghoobi M etal. Diagnostic accuracy of EUS compared with MRCP
in detecting choledocholithiasis:a meta- analysis of diagnostic test accuracy in head- to- head studies.
Gastrointest Endosc. 207 Dec;86(6):986– 993. Doi:0.06/ j.gie.207.06.009.
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8. A. Ascaris lumbricoides
• Ascariasis aects 25% of the global population and is common in India, China, Africa, and Latin
America
• Hepatobiliary and pancreatic infestation leads to ampullary obstruction, biliary colic,
cholangitis, and acute pancreatitis
• Treatment is with antihelminthic agents +/ − removal of nematodes via ERCP
Ascariasis describes human colonization acts with the nematode, Ascaris lumbricoides. It aects
25% of the global population and is ubiquitous in the Indian subcontinent, China, Africa, and Latin
America, although it can rarely be found in Europe and then typically in rural areas. Most infections
are asymptomatic with clinical disease restricted to individuals with heavy worm load, whereby
multiple organ systems can become involved with associated clinical presentations including
the gastrointestinal tract (obstruction, peritonitis, appendicitis), lungs (pneumonia, asthma) and
hepatobiliary and pancreatic ducts (cholangitis, cholecystitis, hepatic abscesses, hepatolithiasis, or
acute pancreatitis). Hepaticobiliary and pancreatic ascariasis (HPA) is caused by proximal movement
of the organisms from the jejunum (its natural habitat) into the duodenum and ampulla of Vater,
causing biliary obstruction and associated complications. In addition, the writhing movements of live
worms induce sphincter spasm causing severe biliary colic. Ultrasound has an excellent sensitivity
for hepatobiliary ascariasis but is less reliable in pancreatic duct disease where MRCP or ERCP may
be required. Antihelmintic drugs (e.g. mebendazole) are the mainstay of therapy with endotherapy
for HPA performed in cases where symptoms do not subside following intensive medical treatment
and/ or ascrides fail to move out of the ductal lumen.
The liver ukes Opisthorchis viverrini, Clonorchis sinensis, and Fasciola hepatica are very thin, tend
to reside in peripheral small and medium- sized bile ducts, and beyond the spatial resolution of
ultrasound.
Khuroo MS, Rather AA, Khuroo NS etal. Hepatobiliary and pancreatic ascariasis. World J Gastroenterol.
206 Sep 7;22(33):7507– 757. Doi:0.3748/ wjg.v22.i33.7507.
9. E. Ursodeoxycholic acid
• Low- phospholipid associated cholelithiasis (LPAC) is caused by mutations in the ABCB4 gene
• It is characterized by gallstone disease <40years, intrahepatic lithiasis, and biliary colic post
cholecystectomy
• Treatment is with urosodeoxycholic acid
Low- phospholipid associated cholelithiasis (LPAC) is a rare genetic disease associated with
mutations in the ABCB4 gene encoding for the biliary carrier protein MDR3. Because of low biliary
phospholipid concentrations, cholesterol gallstone disease develops before the age of 40years
with intrahepatic bile duct and gallbladder cholesterol stones and recurrent biliary symptoms
after cholecystectomy. Further diagnostic clues are provided by a family history of cholelithiasis in
rst- degree relatives and recurrent bile duct stones. While ABCB4 gene sequencing may provide
additional information, it is not necessary to make the diagnosis of LPAC. Treatment revolves
around ursodeoxycholic acid (5 mg/ kg body weight per day), which yields dissolution of biliary
calculi. Rarely, intrahepatic lithiasis can lead to secondary biliary cirrhosis requiring LT.
EASL Clinical Practice Guidelines on the prevention, diagnosis and treatment of gallstones. J Hepatol.
206 Jul;65():46– 8. Doi:0.06/ j.jhep.206.03.005.
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10. D. It is associated with higher rates of cholangitis compared with
conventional ERCP
• Chalangiopancreatoscopy allows direct visualization of the biliary and pancreatic ducts, and can
help with diagnosis of ductal lesions and removal of dicult stones
• The technique carries a higher risk of cholangitis compared with conventional ERCP
Per- oral cholangiopancreatoscopy enables direct endoscopic visualization of the biliary and
pancreatic ductal systems during ERCP and usually requires sphincterotomy. Traditional DOC, the
so- called ‘mother– daughter’ techinique, requires one endoscopist to control the cholangioscope
and a second to control the duodenoscope. Adding cholangioscopic appearance of bile duct lesions
to biopsy/ brush sampling has been shown to improve diagnostic yield. EHL of ductal calculi is the
most common therapeutic application of DOC, and manages to achieve duct clearance in 77%–
96% of cases where standard ERCP has been unsuccessful. Cholangiopancreatoscopy is associated
with increased risk of cholangitis compared with standard ERCP because of intraductal irrigation,
and therefore prophylactic antibiotics are always required. Amore recent innovation is the singleoperator bre- optic cholangioscope system (SpyGlassTM), which has removed many of the
logistical diculties inherent in requiring two endoscopists, although it is limited by reduced image
quality and the small diameter of the working channel. Direct cholangioscopy refers to the use of
ultraslim endoscopes designed to directly enter the CBD. This technique is technically demanding
and the high intrabiliary pressures from air insuation has been associated with paradoxical
embolism of hepatic venous air and cerebrovascular accidents.
Tringali A, Lemmers A, Meves V etal. Intraductal biliopancreatic imaging:European Society of
Gastrointestinal Endoscopy (ESGE) technology review. Endoscopy. 205 Aug;47(8):739– 753.
Doi:0.055/ s- 0034- 392584.
11. B. It occurs in 50% of patients with PSC over the course of their disease
• Dominant strictures (DS) in PSC are associated with increased risk of CCA and mortality
• CA 9- 9 has poor sensitivity for the detection of CCA
• Balloon dilatation should be the initial treatment of choice for DS in PSC
The MRCP image (Fig. 4.2) shows a long DS involving the CBD and common hepatic duct (CHD)
(arrow), hilar stricture, and proximal dilatation of intrahepatic ducts (arrowheads).
DS occur in 50% of PSC patients and are dened as an extrahepatic stenosis ≤.5mm diameter
in the CBD or ≤mm in the main hepatic ducts within 2cm of the hilum. DS are associated
with an increased risk of CCA and mortality. Development of DS have been associated with
polymorphisms in CD4, a key mediator of the innate immune system. ERCP and ductal sampling
should be considered in PSC in the case of (i)worsening symptoms ( jaundice, cholangitis, pruritus);
(ii) rapid increase of cholestatic enzyme levels; or (iii) new DS or progression of existing stricture(s)
identied at MRCP. Antibiotics should be given routinely before ERCP in PSC patients because
they are at high risk for incomplete biliary drainage and cholangitis. For symptomatic DS, dilatation
or stenting at ERCP has been shown to improve survival and may alter the natural history of the
disease. Data are lacking in asymptomatic DS. In a recent randomized trial, both balloon dilatation
and short- term stenting were shown to have equal clinical benet, but there were signicantly
more side eects in the stenting group (pancreatitis and cholangitis). CA 9- 9 in isolation has a low
sensitivity (4%) for detection of CCA in PSC. Currently, there is no proven benecial surveillance
strategy for CCA in DS.
Aabakken L, Karlsen TH, Albert J, Role of endoscopy in primary sclerosing cholangitis:ESGE and EASL
Clinical Guideline. Endoscopy. 207 Jun;49(6):588– 608. Doi:0.055/ s- 0043- 07029.
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Fig.4.12 MRCP showing CBD stricture (arrow) and proximal biliary dilatation (arrowheads)
12. B. Cirrhosis
• The hepatobiliary ukes Opisthorchis viverrini and Clonorchis sinensis are important risk factors
for CCA in Southeast Asia
• PSC is the most common risk factor for CCA in Western populations
• Other risk factors for CCA include hepatolithiasis, cirrhosis, hepatitis B virus (HBV), hepatitis
C virus (HCV) infection, and choledochal cysts
CCAs are biliary epithelial tumours involving the intrahepatic, perihilar, and distal biliary tree,
and they are the second most common hepatic malignancy after HCC. Although most CCAs are
sporadic, there are several established risk factors. Age- adjusted incidence rates are highest in
Hispanic and Asian populations, and lowest in non- Hispanic white and black populations. Some of
the geographical variation may be accounted for by exposure to environmental risk factors. For
example, in Southeast Asia, there is a high prevalence of infection with the hepatobiliary ukes
Opisthorchis viverrini and Clonorchis sinensis, which contribute to chronic biliary inammation and
development of CCA. Fasciola hepatica is another common liver uke in Asia and Africa but,
although it can cause biliary pain and obstruction, it is not an established risk factor for CCA.
Hepatolithiasis is another risk factor for intrahepatic CCA in Asia, and can occur alongside or
independently of uke infection. Meta- analysis has conrmed cirrhosis, HBV and HCV infection as
major independent risk factors for CCA with odds ratios of 23, 5, and 5 respectively. In the West,
PSC is the most common predisposing condition for CCA with an annual risk of development of
CCA between 0.5% and .5%, with a lifetime prevalence of 5%– 0%. Primary biliary cholangitis
is not an established risk factor for CCA. Other risk factors for CCA include choledochal cystic
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diseases, including Caroli’s disease, which is characterized by multiple segmental dilatations of
intrahepatic bile ducts and congenital hepatic brosis.
Rizvi S, Gores GJ. Pathogenesis, diagnosis, and management of cholangiocarcinoma. Gastroenterology.
203 Dec;45(6):25– 229. Doi:0.053/ j.gastro.203.0.03.
13. D. Refer for surgery
• Brush cytology of biliary strictures has a typically low sensitivity but high specicity for the
detection of malignancy
The history of recurrent pancreatitis suggests the possibility of pancreatitis- related benign distal
CBD stricture in the absence of any mass lesion on imaging. However, the brush cytology clearly
shows malignant changes throughout with increased nuclear- cytoplasmic ratio, nuclear crowding,
and overlapping with three- dimensional cell clusters. While sensitivities have been reported to be
variable (6%– 64%) and typically low (4.6% in a meta- analysis), the specicity reaches 98%– 99%.
EUS tissue sampling, or cholangioscopy, is a reasonable next step if the brush cytology was negative
or equivocal. This patient had a localized distal CBD malignant stricture likely to be CCA and should
be referred for a Whipple’s operation.
Burnett AS, Calvert TJ, Chokshi RJ etal. Sensitivity of endoscopic retrograde
cholangiopancreatography standard cytology:0- y review of the literature. J Surg Res. 203
Sep;84():304– 3. Doi:0.06/ j.jss.203.06.028.
14. B. ERCP, brushings, and metal stent
• Biliary decompression in pancreatobiliary cancers should be performed after MDT discussion
on resectability whenever possible
• An uncovered metal stent should be avoided if there is uncertainty in the diagnosis of cancer
because it is dicult to remove endoscopically and makes surgery technically challenging
A CT scan shows intrahepatic bile duct dilation and a large malignant- appearing left para- aortic
lymph node (Fig. 4.3). Given the patient’s presentation, a hepatopancreatobiliary cancer
should be suspected. The para- aortic lymph node suggests metastatic disease and precludes
surgery. APET- CT does not alter management in this case, and is reserved to investigate for
locoregional lymph node involvement and distant metastasis when considering resectability. Biliary
decompression is required before initiating chemotherapy. Therefore, an ERCP allows biliary
sampling and decompression at the same time. The advantage of a metal stent over a plastic stent
is its durability and patency, which reduces the need for repeat ERCP and stent change, particularly
in patients who are expected to survive over three months. The patency rates of metal stents are
signicantly greater than those of plastic stents (2months vs 3months). Initial stent insertion
for biliary obstruction should be a plastic or covered metal stent if the diagnosis and resectability
are undecided. EUS and ne needle aspiration biopsy may improve the diagnosis of CCA if
brush cytology is negative. The ERCP image for this patient is shown in Fig. 4.4. It demonstrates
a common hepatic duct stricture (arrowheads) suggestive of a CCA. This patient has started
chemotherapy after successful biliary decompression.
Khan SA, Davidson BR, Goldin RD etal. BSG Guidelines for the diagnosis and treatment of
cholangiocarcinoma:an update. Gut. 202 Dec;6(2):657– 669. Doi:0.36/ gutjnl- 20- 30748.
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Fig.4.13 CT abdomen and pelvis showing intrahepatic biliary dilatation (arrowheads) and large
para-aortic lymph node (circled)
Fig.4.14 Cholangiogram showing common hepatic duct stricture (arrowheads)
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15. B. Bulky pancreas and hypodense wedge- shaped renal lesions
• IgG4- related sclerosing cholangitis (IgG4- SC) can mimic CCA and/ or PSC
• Diagnosis of IgG4- SC relies on careful interpretation of histopathological appearances,
radiological features, and serological abnormalities in an appropriate clinical scenario (HiSORT
criteria)
This patient has a hilar stricture (Fig. 4.5; arrow head) with intrahepatic duct dilatation (Fig. 4.5;
arrows) on the MRCP image. The main dierential diagnosis is IgG4- related sclerosing cholangitis
(IgG4- SC) and hilar CCA. IgG4- SC has a male preponderance with typical age of 50– 60 at
presentation. It is the most common extrapancreatic manifestation in patients with autoimmune
pancreatitis (AIP type ). Four main cholangiographic subtypes of IgG4- SC have been reported but
are not specic to discriminate from CCA, particularly in localized malignant disease.
Serum IgG4 levels are elevated in 65%– 80% of patients at diagnosis of IgG4- SC. However, they are
also raised in 5%– 25% of inammatory, autoimmune, and malignant conditions. Serum CA 9- 9
can also be raised in up to 63% of IgG4- SC patients. Cross- sectional imaging is important because
it can show thickened bile duct walls, a mass lesion, evidence of other organ involvement, and any
vascular invasion. Brush cytology and FNA have poor sensitivity for the diagnosis of IgG4- SC but
are useful to exclude malignancy. Intraductal biopsies can show characteristic histological features.
This patient had pancreatic and renal involvement on cross- sectional imaging characteristic of IgG4related disease.
Fig.4.15 MRCP image showing hilar stricture (arrowhead) and intrahepatic duct dilatation
(arrows)
Culver EL, Barnes E.IgG4- related sclerosing cholangitis. Clin Liver Dis (Hoboken). 207 Jul 28;0():9– 6.
Doi:0.002/ cld.642.
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