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Diseases of the bile ducts
Chapter 33
Diseases of the bile ducts Ira Jacobson, MD David Purow, MD
Key Points 1 Diseases of the bile ducts usually present with symptoms and signs related to ductal obstruction. These include pain, jaundice, pruritus, fever, and elevation in serum levels of liver tests. 2 Choledocholithiasis is the most common benign disorder of the biliary tree. Predictors of common bile duct (CBD) stones include elevated liver tests, duct dilatation on ultrasonography or computed tomography (CT), stones visualized in the duct on imaging studies, and initial presentation with cholangitis. 3 In the diagnosis and management of CBD stones before laparoscopic cholecystectomy, endoscopic retrograde cholangiopancreatography (ERCP) should be restricted to patients in whom there is a high level of suspicion and in whom a therapeutic intervention is likely. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound (EUS) are used increasingly for diagnosis. 4 Endoscopic sphincterotomy is the most common technique used for removal of bile duct stones, either before or after cholecystectomy. Laparoscopic extraction of CBD stones during cholecystectomy is an important alternative when intraoperative cholangiography is positive and surgical expertise is available. 5 Endoscopic intervention plays an important role in the diagnosis and treatment of complications of cholecystectomy, such as biliary leaks and strictures. 6 Anatomic and congenital anomalies, such as choledochal cysts, can lead to jaundice, pancreatitis, and even biliary carcinoma if not recognized and treated.
Bile Duct Stones Composition 1 Most gallbladder stones are cholesterol-rich; they may migrate to the CBD 2 Bilirubin stones in the gallbladder are seen in hemolytic disorders, e.g., sickle cell anemia; they too may migrate to CBD 3 Pigment stones are rich in calcium bilirubinate associated with chronic stasis in bile ducts, primary sclerosing cholangitis, biliary strictures, markedly dilated ducts, recurrent pyogenic cholangitis
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Clinical presentation 1 Presenting features of CBD stones • pain • cholangitis • pancreatitis • jaundice 2 Pain from bile duct stones resembles pain of gallbladder origin • epigastric or right upper quadrant pain • abdominal tenderness is greater with cholecystitis than CBD stones • obstructive jaundice from stones is usually accompanied by pain • jaundice associated with malignancy is more likely to be painless 3 Features of cholangitis • ‘Charcot’s triad’ – abdominal pain, fever, and jaundice; each feature may not be present in all patients with cholangitis • ‘Reynold’s pentad’ = ‘Charcot’s triad’ plus hypotension and altered mental status • fever may be marked by severe rigors • cholangitis is much more frequent with bile duct stones than with malignant obstruction • severe cholangitis must be considered life-threatening and requires urgent intervention 4 Timing of presentation with bile duct stones is variable; may occur • precholecystectomy • on intraoperative cholangiography • shortly after cholecystectomy • months to many years after cholecystectomy 5 Gallstone pancreatitis (see later) • small gallstones pose a greater risk of pancreatitis than large stones (migrate more easily through the cystic duct)
Laboratory features 1 Elevations in serum liver tests: alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase, gamma glutamyltranspeptidase (GGTP), bilirubin levels • marked elevations in the serum ALT, AST may occur, even >1000 U/L transiently • ALT falls rapidly, even as alkaline phosphatase rises if stone impaction persists • can be confused with hepatitis 2 Elevations in serum amylase suggest acute pancreatitis 3 Elevations in white blood cell (WBC) count occur with cholangitis or pancreatitis 4 Positive blood cultures found with cholangitis
Imaging studies 1 Ultrasonography • excellent for detecting gallbladder stones; less sensitive for bile duct stones • may be limited by obesity, gas in intestine • very sensitive for detecting bile duct dilatation • more sensitive for CBD stones when duct is dilated • absence of bile duct dilatation does not preclude bile duct stones 2 Computed tomography (CT) • sensitivity <50% for bile duct stones • detection relies on presence of calcifications in stones
Diseases of the bile ducts Fig. 33.1 MRCP image showing a large stone in the distal CBD (arrow). The CBD is markedly dilated.
• sensitivity similar to that of ultrasonography for detecting dilated bile duct • avoid oral contrast on initial images – can obscure CBD stones 3 MRCP (see Fig. 33.1) • relies on T2 weighted images • fluid in ducts provides contrast • sensitivity and specificity rates over 90% • less sensitive for small stones • limitations include availability and cost; contraindications include pacemakers or other metallic implants and claustrophobia 4 Endoscopic ultrasound (EUS) • sensitivity and specificity rival those of ERCP • less risk than ERCP, but still an endoscopic procedure requiring sedation • most suitable when EUS and ERCP can be performed at same session 5 ERCP (see Fig. 33.2) • has been the ‘gold standard’ • can miss small stones, especially in dilated ducts • most often done with therapeutic intent • ERCP with diagnostic intent alone is done less commonly now, with availability of less invasive imaging tests, e.g., MRCP • main risk of ERCP is pancreatitis (5%); usually mild but occasionally severe • other complications can arise from sphincterotomy, e.g., bleeding, duodenal perforation; small risk of anesthesia, as with any endoscopic procedure • percutaneous transhepatic cholangiography (PTC) seldom used for evaluation or treatment of CBD stones, except with acute cholangitis when ERCP is unavailable or fails • PTC is occasionally used to facilitate ‘rendevous procedure’ by the radiologist and endoscopist when ERCP fails
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Fig. 33.2 (A) ERCP cholangiogram showing balloon proximal to three CBD stones prior to stone extraction. (B) ERCP image showing clear cholangiogram after stone extraction. (C) Stone adjacent to sphincterotomy site in duodenum after balloon extraction.
Treatment 1 Open choledochotomy • was the standard care prior to ERCP in the 1970s • performed either at time of cholecystectomy or after patient presented with symptomatic choledocholithiasis • in high-risk patients, gallbladder often left intact, as studies showed risk of needing subsequent cholecystectomy was only 10–20% within 5–10 years • currently used only when other modalities fail or are unavailable 2 ERCP and endoscopic sphincterotomy: treatment of choice at most centers since 1970s • successful clearance of bile duct in over 90% of patients • definitive treatment for CBD stones in postcholecystectomy patients • most common treatment for CBD stones when laparoscopic cholecystectomy is planned and CBD stones documented or strongly suspected
Diseases of the bile ducts
• gallbladder may be left intact after ERCP in high-risk patients, as need for subsequent cholecystectomy is 10–20% within 5–10 years 3 Role of preoperative versus postoperative ERCP • no role for ‘routine’ preoperative ERCP • risk factors for CBD stones – elevated liver tests – CBD dilatation on imaging – actual CBD stones on imaging – initial presentation with cholangitis
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• preoperative ERCP is appropriate when suspicion of CBD stones is high • postoperative ERCP is effective therapy for CBD stones confirmed on intraoperative cholangiography (IOC) • trend toward the deferral of ERCP to postoperative period unless preoperative suspicion is high • unsuccessful postoperative ERCP may result in second operation – should seldom occur in experienced centers Laparoscopic cholecystectomy plus extraction of CBD stones • high success rates (80–90%) reported by expert surgeons when IOC is positive for CBD stones • usual approach is transcystic route • laparoscopic choledochotomy also possible • surgical expertise still not widely available • many surgeons prefer preoperative or postoperative ERCP ERCP techniques for choledocholithiasis • initial cannulation with sphincterotome • advancement of sphincterotome over a guidewire into the bile duct • needle-knife pre-cut sphincterotomy when cannulation of the bile duct is difficult Large stones may require more advanced ERCP techniques • mechanical lithotripsy with large baskets • extracorporeal shock wave lithotripsy – rarely used in USA • laser lithotripsy via choledochoscopy – ‘baby scope’ inserted into CBD through channel of side-viewing endoscope • electrohydraulic lithotripsy via ‘baby scope’ Complications of ERCP and sphincterotomy • pancreatitis in 5% may result from either the diagnostic portion of the procedure or from cautery-induced injury to the pancreatic ductal orifice – symptoms of pancreatitis may not occur until 6–12 h following the procedure – management of post-ERCP pancreatitis is similar to that of other forms of pancreatitis – there are no proven pharmacologic means to prevent pancreatitis – pancreatitis is more common with difficult cannulations, patients with sphincter of Oddi dysfunction, and needle-knife precuts – pancreatitis is more common with difficult cannulations, patients with sphincter of Oddi dysfunction, and needle-knife precuts – recent trends to place temporary stents to prevent pancreatitis • bleeding occurs in 2–3% and is usually self-limited – may occasionally require blood transfusions and even angiographic embolization or surgery
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– local measures during ERCP such as injection of epinephrine, balloon tamponade, or electrocautery may stop bleeding • perforation (usually retroperitoneal) occurs in 1% – often responds to nonsurgical management with nasogastric decompression, nasobiliary drainage (if the complication has been recognized during the ERCP), and broad-spectrum antibiotics – surgery for perforation is required if signs of infection cannot be controlled with antibiotics • infection may occur when adequate drainage is not provided following ERCP – nasobiliary tube or endoprosthesis should be placed to provide drainage until duct can be cleared Long-term stent placement • reserved for patients in whom stone extraction is not accomplished by above means • most appropriate for frail or elderly patients • cholangitis occurs in 10–40% in ensuing years The bile salt ursodeoxycholic acid, taken for 6–12 months, can result in significant shrinkage or even dissolution of retained ductal stones, facilitating subsequent attempts to clear the duct after stents have been placed Oral dissolution therapy with bile salts is not appropriate as sole treatment of CBD stones In good operative candidates, surgery should be performed for otherwise unextractable common duct stones Severe cholangitis is an indication for urgent ERCP, which has been shown to be superior to emergency surgery in a randomized trial
Gallstone pancreatitis 1 Related to impaction of a stone in the ampulla of Vater with occlusion of the pancreatic ductal orifice 2 Clinical features at presentation: • epigastric pain radiating directly into the back • nausea and vomiting • low-grade fever • tachycardia • hypotension, if there has been significant sequestration, or ‘thirdspacing’, of fluid • ascites
3 Laboratory features • leukocytosis • elevated liver tests (usually to a greater degree than in alcoholic and other causes of pancreatitis) • elevated serum amylase and lipase • elevated blood urea nitrogen and creatinine if there is sufficient third-spacing to compromise renal blood flow • hypocalcemia in moderate to severe cases • hypoxemia in severe cases; due to pulmonary capillary leak, which may result in acute respiratory distress syndrome
Diseases of the bile ducts
4 Ranson criteria – most quoted of many classification systems used to predict severity of episode of acute pancreatitis Ranson criteria on admission • age >55 • blood glucose >200 mg/dL • WBC count >16 000/mm3 • serum LDH >350 IU/L • AST >250 U/L Ranson criteria at 48 h • hematocrit decrease >10% • serum calcium <8 mg/dL • base deficit >4 mmol/L • BUN increase >5 mg/dL • estimated fluid sequestration >6 L • arterial oxygen tension <60 mmHg Fewer than three Ranson criteria indicates mild pancreatitis Four or more Ranson criteria associated with more severe pancreatitis and higher mortality 5 Medical treatment of gallstone pancreatitis is similar to that for other forms of pancreatitis • strictly nothing by mouth • intravenous hydration • careful recording of intake and output • intravenous antibiotics are recommended to prevent cholangitis or pancreatic abscess; efficacy debated but suggested by published studies (e.g., imipenem 500 mg i.v. every 6 h) • monitoring of laboratory data, including blood counts and electrolytes • serial contrast-enhanced CT scans to monitor patients with moderate or severe pancreatitis for the development of pancreatic necrosis, pseudocysts, or abscesses • some animal data have shown that contrast-enhanced CT scans may exacerbate pancreatitis, but recent trials have shown contrast-enhanced CT scan do not alter course of pancreatitis 6 Three published randomized studies on role of ERCP in gallstone pancreatitis: • no benefit to ERCP in mild gallstone pancreatitis unless there is clear evidence of a retained stone • one study demonstrated reduced risk of local and systemic complications and shorter hospitalizations in severe pancreatitis • another study showed reduced risk of cholangitis • the third study showed no benefit to urgent ERCP 7 Concomitant pancreatitis and cholangitis is a strong indication for urgent ERCP and sphincterotomy
Postcholecystectomy Syndrome Definition Biliary-type pain in a patient who has undergone cholecystectomy.
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Postoperative bile duct injuries and leaks
Differential diagnosis In addition to CBD stones, the following nonbiliary entities should be considered: • irritable bowel syndrome • gastroesophageal reflux disease • esophageal spasm • peptic ulcer • chronic pancreatitis
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Common cause of postcholecystectomy syndrome Clinical criteria for diagnosis: • bile duct dilatation • delayed drainage of contrast from the bile duct beyond 45 min after ERCP • elevated liver enzymes on repeated occasions during episodes of pain Diagnosis • the gold standard for the diagnosis of SOD is an elevated sphincter pressure >40 mm Hg at SO manometry during ERCP • SO manometry is not available at all centers that perform ERCP • SO manometry may add to the risk of pancreatitis during ERCP • noninvasive imaging techniques like nuclear hepatobiliary (e.g., DISIDA) scanning may be useful Type 1 SOD • all three clinical criteria met • SOD almost always present and SO manometry not required Type 2 SOD • one or two clinical criteria met • accounts for about half of patients have SOD • only patients with abnormal manometry have good long-term responses to sphincterotomy • manometry important for planning therapy Type 3 SOD • biliary pain alone • none of clinical criteria • abnormal manometry in up to half of patients • response to sphincterotomy not well studied • treatment decisions should be individualized to each patient SOD can occur in patients with intact gallbladders: • many experts recommend empiric cholecystectomy, not biliary endoscopy, as initial therapy for patients with unexplained biliary-type right upper quadrant pain, even without cholelithiasis
Postoperative Bile Duct Injuries and Leaks Introduction 1 Reported in 0.2–0.5% of cholecystectomies • partially related to experience of surgeon • incidence is declining 2 Bile leaks can cause acute illness due to intraperitoneal bile collections
Diseases of the bile ducts
3 Most dreaded complication is major bile duct injury leading to a stricture • may result in recurrent cholangitis, liver atrophy, and/or secondary biliary cirrhosis 4 Bile leaks are a known complication of liver transplantation
Classification 1 Types of biliary tract injuries during cholecystectomy • bile leak without interruption of ductal continuity • injury to one or more ducts with impairment or complete interruption of bile flow but without a bile leak • combined bile leak and damage to a duct resulting in interrupted flow 2 Classification system proposed by Strasberg et al. (1995) • Type A – bile leak from a minor duct with preservation of continuity between liver and duodenum – examples of Type A leaks include injury to cystic duct remnant or the duct of Luschka, an accessory duct connecting the gallbladder to the liver bed • Type B – occlusion of right hepatic duct or one of its branches – Type B injury occurs because the right hepatic duct is mistaken for the cystic duct during operation due to an anatomic variation in which the cystic duct joins the right hepatic duct rather than the CBD • Type C – transection rather than occlusion of an aberrant right hepatic duct, resulting in a bile leak • Type D – lateral injury to an extrahepatic duct with preserved communication between the biliary tree and duodenum • Type E – Occlusive injury of the CBD at any level from the hepatic bifurcation to the duodenum
Diagnosis 1 May be recognized intraoperatively or delayed for years 2 Presenting symptoms and signs of bile leaks • pain • low-grade fever • abdominal tenderness • leukocytosis • minor liver chemistry elevations 3 Presenting symptoms and signs of major occlusive injuries of bile ducts • jaundice • pruritus • elevated liver chemistries • cholangitis 4 Imaging studies • nuclear DISIDA scan useful for diagnosis of bile leak • ultrasonography or CT scan may detect intraperitoneal bile collection • benign CBD strictures may not result in ductal dilatation on imaging • MRCP can delineate ductal injury • ERCP is the preferred modality for the diagnosis of major bile duct injuries 5 PTC may be necessary in certain situations: • involvement of the biliary tree above the bifurcation in which PTC can better localize the proximal extent and access the injured duct
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Recurrent pyogenic cholangitis
• suspicion of an excluded segment of the biliary tree with absent communication between the liver and the distal bile duct • MRCP is currently best initial step for defineation of proximal biliary injuries and excluded segments
Management 1 Goal of therapy for bile leaks is to reduce outflow resistance in to the duodenum • procedure of choice is ERCP with stent placement, with or without sphincterotomy • stent does not need to bridge the site of the leak 2 Percutaneous or operative drainage may be needed for large bile collections. 3 Antibiotics should be administered until leak is controlled or drained. 4 Surgical treatment: • for major injuries, ligation of bile duct and creation of Roux-en-Y connection between proximal biliary tree and jejunum • smaller injuries may be repaired by suturing bile duct over a T-tube • treatment of a complete transection of the bile duct during surgery by suturing over a T-tube is rarely successful in the long term 5 Endoscopic therapy of postoperative biliary strictures: • no prospective randomized trials • there are reports of successful treatment with stent placement followed by stent exchanges every 3–6 months for at least 1 year • published series differ on the need for balloon dilation of a stricture prior to stent placement • series agree that good long-term results are achieved in 50–80% of patients at expert centers • retrospective comparison of patients treated endoscopically or surgically has revealed similar results; surgery was better in one series • successful treatment of post-liver transplant bile leaks has been reported
Recurrent Pyogenic Cholangitis Overview 1 The syndrome is characterized by primary intrahepatic stones associated with strictures of intrahepatic ducts. 2 Predominantly seen in the Far East; rare in the USA. 3 Stones composed mainly of calcium bilirubinate. 4 The syndrome was known previously as Oriental cholangiohepatitis.
Pathogenesis 1 Syndrome associated with bacterial infection of bile • bacterial beta glucuronidases hydrolyze conjugated bilirubin • unconjugated bilirubin binds with calcium and precipitates as calcium bilirubinate, the major constituent of intrahepatic stones • parasitic infections, such as Ascaris or Clonorchis, may play a role 2 Seen in rural rather than urban areas in endemic countries • low-protein diet prevalent in rural areas decreases biliary glucurolactone, an inhibitor of beta glucuronidase • the effect is enhanced endogenous beta glucuronidase activity with further deconjugation of bile, leading to precipitation of calcium bilirubinate
Diseases of the bile ducts
Presentation 1 Occurs at younger age than Western gallstone disease • may even occur in young adults and children 2 Clinical features • abdominal pain • jaundice • infection 3 Laboratory features • leukocytosis • elevated serum alkaline phosphatase and bilirubin levels 4 Potential consequences • liver abscesses • atrophy of affected liver segments
Diagnosis 1 Ultrasonography or CT scan may reveal focal areas of dilated intrahepatic ducts as well as stones 2 Definitive diagnosis requires ERCP or PTC
Treatment 1 Broad-spectrum antibiotics (e.g., piperacillin/tazobactam 3.375 g, i.v. every 6 h, or ciprofloxacin 500 mg, i.v. every 12 h) to treat episodes of acute cholangitis 2 Long-term relief includes surgical options tailored to individual patient • resection of atrophic hepatic segments (possibly even lobectomy) and diseased ducts draining affected liver portion • anastomosis of jejunum to intrahepatic segments proximal to sites of obstruction • creation of permanent access through which subsequent therapeutic maneuvers may be performed • creation of a T-tube tract or loop of jejunum brought to subcutaneous site to which bile duct has been anastomosed 3 Endoscopic therapy • proximal location of strictures and stones makes endoscopic approach difficult • PTC generally preferred to ERCP • some successful reports of endoscopic therapy
Choledochal Cysts Introduction 1 Anomalies of the biliary tree characterized by cystic dilatation of variable portions of the intrahepatic and/or extrahepatic ducts 2 More common in Asia than elsewhere 3 Female to male ratio of 3:1 4 Primarily affects children and young adults, but reported age ranges vary greatly
Etiology – proposed theories 1 Abnormality in biliary epithelial proliferation when fetal ducts are solid – leads to abnormally dilated portion proximal to a normal or stenotic distal portion 2 Distal CBD stenosis inducing proximal cystic dilatation 3 Intrinsic autonomic dysfunction • based on finding of deficient postcholinergic neurons in some portions of cyst wall
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Choledochal cysts Fig. 33.3 The normal anatomy (left) contrasted with the anomalous pancreaticobiliary function (arrow) believed to be responsible for free reflux of pancreatic enzymes and choledochal cyst formation. Modified from O’Neill JA. Choledochal cysts. Curr Prob Surg 1992; 29:365–410.
4 Anomalous pancreaticobiliary junction (see Fig. 33.3) • may result in lack of normal sphincter function and reflux of pancreatic enzymes into bile ducts, inducting progressive damage and dilatation • common in type I cysts, but not in type II, III, and V
Classification by Todani et al. (1977): the most commonly used system (see Fig. 33.4) 1 Type I – dilatation of the extrahepatic duct alone • most common type 2 Type II – diverticulum of extrahepatic bile duct 3 Type III – choledochocele, involving only the intraduodenal duct 4 Type IVA – multiple extrahepatic and intrahepatic cysts 5 Type IVB – multiple extrahepatic cysts only 6 Type V – single or multiple intrahepatic cysts (Caroli’s disease)
Clinical symptoms and signs 1 Right upper quadrant pain 2 Jaundice • often sole symptom in infants 3 Palpable abdominal mass 4 Fever 5 Epigastric or diffuse abdominal pain if pancreatitis present
Diagnosis 1 Ultrasonography or CT scan may reveal or suggest diagnosis MRCP emerging as diagnostic modality 2 Direct cholangiography by ERCP or THC usually diagnostic • also important for classification and planning therapy 3 THC best for delineating and potentially draining proximal biliary ducts 4 ERCP enables evaluation of pancreatic duct and pancreatic-biliary junction • often reveals anomalous junction, especially in type I cysts • MRCP obviates ERCP in many cases
Complications 1 Stone formation within cysts 2 Cholangitis and liver abscesses 3 Acute pancreatitis, with or without stones • most common with a choledochocele
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Diseases of the bile ducts
Fig. 33.4 Classification scheme of choledochal cysts suggested by Todani et al., 1977. From: Savader SJ, Benenati JF, Venbrux AC, et al. Choledochal cysts: classification and cholangiographic appearance. Am J Rad 1991; 156:328.
4 Secondary biliary cirrhosis 5 Carcinoma • usually occurs within cysts • significant risk after nonresectional surgery • primary cyst excision reduces cancer risk markedly but not completely • preoperative diagnosis of cancer is rare • poor prognosis because of extensive spreading 6 Portal hypertension 7 Cyst rupture with bile peritonitis
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Treatment Medical management is associated with high morbidity and mortality Simple drainage is inadequate Cyst excision and reconstruction of biliary tree is the treatment of choice Type I and Type II cysts • excision followed by reconstruction of the biliary tree with Roux-en-Y hepaticojejunostomy • reports of successful laparoscopic approach 5 Type III cysts (choledochoceles) • successful endoscopic management by sphincterotomy has been described 6 Type IV cysts • excision of the extrahepatic cyst, partial resection of intrahepatic cysts when present, and hepaticojejunostomy • predominant involvement of the left lobe in type IVA disease may necessitate left hepatic lobectomy 7 Type V cysts (Caroli’s disease) • partial hepatectomy for localized disease • Roux-en-Y hepaticojejunostomy with placement of transhepatic stents for diffuse disease • recurrent stones and strictures are treated with percutaneous techniques • liver transplantation may be required for severe diffuse disease 1 2 3 4
Further Reading Abboud PAC, Malet PF, Berlin JA, et al. Predictors of common bile duct stones prior to cholecystectomy: a metaanalysis. Gastrointest Endosc 1996; 44:450–459. Coppola R, Riccioni ME, Ciletti S, et al. Selective use of endoscopic retrograde cholangiography to facilitate laparoscopic cholecystectomy without cholangiography. A review of 1139 consecutive cases. Surg Endosc 2001; 15:1213–1216. Fan ST, Lai ECS, Mok FPT, et al. Early treatment of acute biliary pancreatitis by endoscopic papillotomy. N Engl J Med 1993; 328:228–232. Folsch UR, Nitsche R, Ludtke R, et al. Early ERCP and papillotomy compared with conservative treatment for acute biliary pancreatitis. The German Study Group on Acute Biliary Pancreatitis. N Engl J Med 1997; 336:237–244. Freeman ML, Nelson DB, Sherman S, et al. Complications of endoscopic biliary sphincterotomy. N Engl J Med 1996; 335:909–918. Geenen JE, Hogan WJ, Dodds WJ, et al. The efficacy of endoscopic sphincterotomy after cholecystectomy in patients with sphincter of Oddi dysfunction. N Engl J Med 1989; 320:82–87. Kaw M, Al-Antably Y, Kaw P. Management of gallstone pancreatitis: cholecystectomy or ERCP and endoscopic sphincterotomy. Gastrointest Endosc 2002; 56:61–65. Lauter DM, Froines EJ. Laparoscopic common duct exploration in the management of choledocholithiasis. Am J Surg 2000; 179:372–374. Liu CL, Lo CM, Chan JK, et al. Detection of choledocholithiasis by EUS in acute pancreatitis: A prospective evaluation in 100 consecutive patients. Gastrointest Endosc 2001; 54:325–330. Neoptolemos JP, Carr-Locke DL, London, et al. Controlled trial of urgent endoscopic retrograde cholangiopancreatography and endoscopic sphincterotomy versus conservative treatment for acute pancreatitis due to gallstones. Lancet 1988; ii:979–983. O’Neill JA. Choledochal cysts. Curr Prob Surg 1992; 29:365–410. Park JS, Kim MH, Lee SK, et al. Efficacy of endoscopic and percutaneous treatments for biliary complications after cadaveric and living donor liver transplantation. Gastrointest Endosc 2003; 57:78–85. Pitt HA, Kauffman SL, Coleman J, et al. Benign postoperative biliary strictures. Ann Surg 1989; 210:417. Prat F, Amouyal G, Amouyal P, et al. Prospective controlled trial of endoscopic ultrasonography and endoscopic retrograde cholangiography in patients with suspected common bile duct lithiasis. Lancet 1996; 347:75–79. Ranson JHC, Rifkind KM, Roses DF, et al. Prognostic signs and the role of operative management in acute pancreatitis. Surg Gynecol Obstet 1974; 139:69. Rhodes M, Sussman L, Cohen L, et al. Randomised trial of laparoscopic exploration of common bile duct versus postoperative endoscopic retrograde cholangiopancreatography for common bile duct stones. Lancet 1998; 351:159–161.
Diseases of the bile ducts Savader SJ, Benenati JF, Venbrux AC, et al. Choledochal cysts: classification and cholangiographic appearance. Am J Rad 1991; 156:328. Sperling RM, Koch J, Sandhu JS, et al. Recurrent pyogenic cholangitis in Asian immigrants to the United States: natural history and role of therapeutic ERCP. Dig Dis Sci 1997; 42:865–871. Strasberg S, Hertl M, Soper NJ. An analysis of the problem of biliary injury during laparoscopic cholecystectomy. J Am Coll Surg 1995; 180:101–125. Taylor AC, Little AF, Hennessy OF, et al. Prospective assessment of magnetic resonance cholangiography for noninvasive imaging of the biliary tree. Gastrointest Endosc 2002; 55:17–22. Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases, including cancer arising from the choledochal cyst. Am J Surg 1977; 134:263–269.
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Diseases of the bile ducts Ira Jacobson, MD David Purow, MD
Key Points 1 Diseases of the bile ducts usually present with symptoms and signs related to ductal obstruction. These include pain, jaundice, pruritus, fever, and elevation in serum levels of liver tests. 2 Choledocholithiasis is the most common benign disorder of the biliary tree. Predictors of common bile duct (CBD) stones include elevated liver tests, duct dilatation on ultrasonography or computed tomography (CT), stones visualized in the duct on imaging studies, and initial presentation with cholangitis. 3 In the diagnosis and management of CBD stones before laparoscopic cholecystectomy, endoscopic retrograde cholangiopancreatography (ERCP) should be restricted to patients in whom there is a high level of suspicion and in whom a therapeutic intervention is likely. Magnetic resonance cholangiopancreatography (MRCP) and endoscopic ultrasound (EUS) are used increasingly for diagnosis. 4 Endoscopic sphincterotomy is the most common technique used for removal of bile duct stones, either before or after cholecystectomy. Laparoscopic extraction of CBD stones during cholecystectomy is an important alternative when intraoperative cholangiography is positive and surgical expertise is available. 5 Endoscopic intervention plays an important role in the diagnosis and treatment of complications of cholecystectomy, such as biliary leaks and strictures. 6 Anatomic and congenital anomalies, such as choledochal cysts, can lead to jaundice, pancreatitis, and even biliary carcinoma if not recognized and treated.
Bile Duct Stones Composition 1 Most gallbladder stones are cholesterol-rich; they may migrate to the CBD 2 Bilirubin stones in the gallbladder are seen in hemolytic disorders, e.g., sickle cell anemia; they too may migrate to CBD 3 Pigment stones are rich in calcium bilirubinate associated with chronic stasis in bile ducts, primary sclerosing cholangitis, biliary strictures, markedly dilated ducts, recurrent pyogenic cholangitis
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Clinical presentation 1 Presenting features of CBD stones • pain • cholangitis • pancreatitis • jaundice 2 Pain from bile duct stones resembles pain of gallbladder origin • epigastric or right upper quadrant pain • abdominal tenderness is greater with cholecystitis than CBD stones • obstructive jaundice from stones is usually accompanied by pain • jaundice associated with malignancy is more likely to be painless 3 Features of cholangitis • ‘Charcot’s triad’ – abdominal pain, fever, and jaundice; each feature may not be present in all patients with cholangitis • ‘Reynold’s pentad’ = ‘Charcot’s triad’ plus hypotension and altered mental status • fever may be marked by severe rigors • cholangitis is much more frequent with bile duct stones than with malignant obstruction • severe cholangitis must be considered life-threatening and requires urgent intervention 4 Timing of presentation with bile duct stones is variable; may occur • precholecystectomy • on intraoperative cholangiography • shortly after cholecystectomy • months to many years after cholecystectomy 5 Gallstone pancreatitis (see later) • small gallstones pose a greater risk of pancreatitis than large stones (migrate more easily through the cystic duct)
Laboratory features 1 Elevations in serum liver tests: alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase, gamma glutamyltranspeptidase (GGTP), bilirubin levels • marked elevations in the serum ALT, AST may occur, even >1000 U/L transiently • ALT falls rapidly, even as alkaline phosphatase rises if stone impaction persists • can be confused with hepatitis 2 Elevations in serum amylase suggest acute pancreatitis 3 Elevations in white blood cell (WBC) count occur with cholangitis or pancreatitis 4 Positive blood cultures found with cholangitis
Imaging studies 1 Ultrasonography • excellent for detecting gallbladder stones; less sensitive for bile duct stones • may be limited by obesity, gas in intestine • very sensitive for detecting bile duct dilatation • more sensitive for CBD stones when duct is dilated • absence of bile duct dilatation does not preclude bile duct stones 2 Computed tomography (CT) • sensitivity <50% for bile duct stones • detection relies on presence of calcifications in stones
Diseases of the bile ducts Fig. 33.1 MRCP image showing a large stone in the distal CBD (arrow). The CBD is markedly dilated.
• sensitivity similar to that of ultrasonography for detecting dilated bile duct • avoid oral contrast on initial images – can obscure CBD stones 3 MRCP (see Fig. 33.1) • relies on T2 weighted images • fluid in ducts provides contrast • sensitivity and specificity rates over 90% • less sensitive for small stones • limitations include availability and cost; contraindications include pacemakers or other metallic implants and claustrophobia 4 Endoscopic ultrasound (EUS) • sensitivity and specificity rival those of ERCP • less risk than ERCP, but still an endoscopic procedure requiring sedation • most suitable when EUS and ERCP can be performed at same session 5 ERCP (see Fig. 33.2) • has been the ‘gold standard’ • can miss small stones, especially in dilated ducts • most often done with therapeutic intent • ERCP with diagnostic intent alone is done less commonly now, with availability of less invasive imaging tests, e.g., MRCP • main risk of ERCP is pancreatitis (5%); usually mild but occasionally severe • other complications can arise from sphincterotomy, e.g., bleeding, duodenal perforation; small risk of anesthesia, as with any endoscopic procedure • percutaneous transhepatic cholangiography (PTC) seldom used for evaluation or treatment of CBD stones, except with acute cholangitis when ERCP is unavailable or fails • PTC is occasionally used to facilitate ‘rendevous procedure’ by the radiologist and endoscopist when ERCP fails
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Fig. 33.2 (A) ERCP cholangiogram showing balloon proximal to three CBD stones prior to stone extraction. (B) ERCP image showing clear cholangiogram after stone extraction. (C) Stone adjacent to sphincterotomy site in duodenum after balloon extraction.
Treatment 1 Open choledochotomy • was the standard care prior to ERCP in the 1970s • performed either at time of cholecystectomy or after patient presented with symptomatic choledocholithiasis • in high-risk patients, gallbladder often left intact, as studies showed risk of needing subsequent cholecystectomy was only 10–20% within 5–10 years • currently used only when other modalities fail or are unavailable 2 ERCP and endoscopic sphincterotomy: treatment of choice at most centers since 1970s • successful clearance of bile duct in over 90% of patients • definitive treatment for CBD stones in postcholecystectomy patients • most common treatment for CBD stones when laparoscopic cholecystectomy is planned and CBD stones documented or strongly suspected
Diseases of the bile ducts
• gallbladder may be left intact after ERCP in high-risk patients, as need for subsequent cholecystectomy is 10–20% within 5–10 years 3 Role of preoperative versus postoperative ERCP • no role for ‘routine’ preoperative ERCP • risk factors for CBD stones – elevated liver tests – CBD dilatation on imaging – actual CBD stones on imaging – initial presentation with cholangitis
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• preoperative ERCP is appropriate when suspicion of CBD stones is high • postoperative ERCP is effective therapy for CBD stones confirmed on intraoperative cholangiography (IOC) • trend toward the deferral of ERCP to postoperative period unless preoperative suspicion is high • unsuccessful postoperative ERCP may result in second operation – should seldom occur in experienced centers Laparoscopic cholecystectomy plus extraction of CBD stones • high success rates (80–90%) reported by expert surgeons when IOC is positive for CBD stones • usual approach is transcystic route • laparoscopic choledochotomy also possible • surgical expertise still not widely available • many surgeons prefer preoperative or postoperative ERCP ERCP techniques for choledocholithiasis • initial cannulation with sphincterotome • advancement of sphincterotome over a guidewire into the bile duct • needle-knife pre-cut sphincterotomy when cannulation of the bile duct is difficult Large stones may require more advanced ERCP techniques • mechanical lithotripsy with large baskets • extracorporeal shock wave lithotripsy – rarely used in USA • laser lithotripsy via choledochoscopy – ‘baby scope’ inserted into CBD through channel of side-viewing endoscope • electrohydraulic lithotripsy via ‘baby scope’ Complications of ERCP and sphincterotomy • pancreatitis in 5% may result from either the diagnostic portion of the procedure or from cautery-induced injury to the pancreatic ductal orifice – symptoms of pancreatitis may not occur until 6–12 h following the procedure – management of post-ERCP pancreatitis is similar to that of other forms of pancreatitis – there are no proven pharmacologic means to prevent pancreatitis – pancreatitis is more common with difficult cannulations, patients with sphincter of Oddi dysfunction, and needle-knife precuts – pancreatitis is more common with difficult cannulations, patients with sphincter of Oddi dysfunction, and needle-knife precuts – recent trends to place temporary stents to prevent pancreatitis • bleeding occurs in 2–3% and is usually self-limited – may occasionally require blood transfusions and even angiographic embolization or surgery
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– local measures during ERCP such as injection of epinephrine, balloon tamponade, or electrocautery may stop bleeding • perforation (usually retroperitoneal) occurs in 1% – often responds to nonsurgical management with nasogastric decompression, nasobiliary drainage (if the complication has been recognized during the ERCP), and broad-spectrum antibiotics – surgery for perforation is required if signs of infection cannot be controlled with antibiotics • infection may occur when adequate drainage is not provided following ERCP – nasobiliary tube or endoprosthesis should be placed to provide drainage until duct can be cleared Long-term stent placement • reserved for patients in whom stone extraction is not accomplished by above means • most appropriate for frail or elderly patients • cholangitis occurs in 10–40% in ensuing years The bile salt ursodeoxycholic acid, taken for 6–12 months, can result in significant shrinkage or even dissolution of retained ductal stones, facilitating subsequent attempts to clear the duct after stents have been placed Oral dissolution therapy with bile salts is not appropriate as sole treatment of CBD stones In good operative candidates, surgery should be performed for otherwise unextractable common duct stones Severe cholangitis is an indication for urgent ERCP, which has been shown to be superior to emergency surgery in a randomized trial
Gallstone pancreatitis 1 Related to impaction of a stone in the ampulla of Vater with occlusion of the pancreatic ductal orifice 2 Clinical features at presentation: • epigastric pain radiating directly into the back • nausea and vomiting • low-grade fever • tachycardia • hypotension, if there has been significant sequestration, or ‘thirdspacing’, of fluid • ascites
3 Laboratory features • leukocytosis • elevated liver tests (usually to a greater degree than in alcoholic and other causes of pancreatitis) • elevated serum amylase and lipase • elevated blood urea nitrogen and creatinine if there is sufficient third-spacing to compromise renal blood flow • hypocalcemia in moderate to severe cases • hypoxemia in severe cases; due to pulmonary capillary leak, which may result in acute respiratory distress syndrome
Diseases of the bile ducts
4 Ranson criteria – most quoted of many classification systems used to predict severity of episode of acute pancreatitis Ranson criteria on admission • age >55 • blood glucose >200 mg/dL • WBC count >16 000/mm3 • serum LDH >350 IU/L • AST >250 U/L Ranson criteria at 48 h • hematocrit decrease >10% • serum calcium <8 mg/dL • base deficit >4 mmol/L • BUN increase >5 mg/dL • estimated fluid sequestration >6 L • arterial oxygen tension <60 mmHg Fewer than three Ranson criteria indicates mild pancreatitis Four or more Ranson criteria associated with more severe pancreatitis and higher mortality 5 Medical treatment of gallstone pancreatitis is similar to that for other forms of pancreatitis • strictly nothing by mouth • intravenous hydration • careful recording of intake and output • intravenous antibiotics are recommended to prevent cholangitis or pancreatic abscess; efficacy debated but suggested by published studies (e.g., imipenem 500 mg i.v. every 6 h) • monitoring of laboratory data, including blood counts and electrolytes • serial contrast-enhanced CT scans to monitor patients with moderate or severe pancreatitis for the development of pancreatic necrosis, pseudocysts, or abscesses • some animal data have shown that contrast-enhanced CT scans may exacerbate pancreatitis, but recent trials have shown contrast-enhanced CT scan do not alter course of pancreatitis 6 Three published randomized studies on role of ERCP in gallstone pancreatitis: • no benefit to ERCP in mild gallstone pancreatitis unless there is clear evidence of a retained stone • one study demonstrated reduced risk of local and systemic complications and shorter hospitalizations in severe pancreatitis • another study showed reduced risk of cholangitis • the third study showed no benefit to urgent ERCP 7 Concomitant pancreatitis and cholangitis is a strong indication for urgent ERCP and sphincterotomy
Postcholecystectomy Syndrome Definition Biliary-type pain in a patient who has undergone cholecystectomy.
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Postoperative bile duct injuries and leaks
Differential diagnosis In addition to CBD stones, the following nonbiliary entities should be considered: • irritable bowel syndrome • gastroesophageal reflux disease • esophageal spasm • peptic ulcer • chronic pancreatitis
Sphincter of Oddi Dysfunction (SOD) 1 2
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Common cause of postcholecystectomy syndrome Clinical criteria for diagnosis: • bile duct dilatation • delayed drainage of contrast from the bile duct beyond 45 min after ERCP • elevated liver enzymes on repeated occasions during episodes of pain Diagnosis • the gold standard for the diagnosis of SOD is an elevated sphincter pressure >40 mm Hg at SO manometry during ERCP • SO manometry is not available at all centers that perform ERCP • SO manometry may add to the risk of pancreatitis during ERCP • noninvasive imaging techniques like nuclear hepatobiliary (e.g., DISIDA) scanning may be useful Type 1 SOD • all three clinical criteria met • SOD almost always present and SO manometry not required Type 2 SOD • one or two clinical criteria met • accounts for about half of patients have SOD • only patients with abnormal manometry have good long-term responses to sphincterotomy • manometry important for planning therapy Type 3 SOD • biliary pain alone • none of clinical criteria • abnormal manometry in up to half of patients • response to sphincterotomy not well studied • treatment decisions should be individualized to each patient SOD can occur in patients with intact gallbladders: • many experts recommend empiric cholecystectomy, not biliary endoscopy, as initial therapy for patients with unexplained biliary-type right upper quadrant pain, even without cholelithiasis
Postoperative Bile Duct Injuries and Leaks Introduction 1 Reported in 0.2–0.5% of cholecystectomies • partially related to experience of surgeon • incidence is declining 2 Bile leaks can cause acute illness due to intraperitoneal bile collections
Diseases of the bile ducts
3 Most dreaded complication is major bile duct injury leading to a stricture • may result in recurrent cholangitis, liver atrophy, and/or secondary biliary cirrhosis 4 Bile leaks are a known complication of liver transplantation
Classification 1 Types of biliary tract injuries during cholecystectomy • bile leak without interruption of ductal continuity • injury to one or more ducts with impairment or complete interruption of bile flow but without a bile leak • combined bile leak and damage to a duct resulting in interrupted flow 2 Classification system proposed by Strasberg et al. (1995) • Type A – bile leak from a minor duct with preservation of continuity between liver and duodenum – examples of Type A leaks include injury to cystic duct remnant or the duct of Luschka, an accessory duct connecting the gallbladder to the liver bed • Type B – occlusion of right hepatic duct or one of its branches – Type B injury occurs because the right hepatic duct is mistaken for the cystic duct during operation due to an anatomic variation in which the cystic duct joins the right hepatic duct rather than the CBD • Type C – transection rather than occlusion of an aberrant right hepatic duct, resulting in a bile leak • Type D – lateral injury to an extrahepatic duct with preserved communication between the biliary tree and duodenum • Type E – Occlusive injury of the CBD at any level from the hepatic bifurcation to the duodenum
Diagnosis 1 May be recognized intraoperatively or delayed for years 2 Presenting symptoms and signs of bile leaks • pain • low-grade fever • abdominal tenderness • leukocytosis • minor liver chemistry elevations 3 Presenting symptoms and signs of major occlusive injuries of bile ducts • jaundice • pruritus • elevated liver chemistries • cholangitis 4 Imaging studies • nuclear DISIDA scan useful for diagnosis of bile leak • ultrasonography or CT scan may detect intraperitoneal bile collection • benign CBD strictures may not result in ductal dilatation on imaging • MRCP can delineate ductal injury • ERCP is the preferred modality for the diagnosis of major bile duct injuries 5 PTC may be necessary in certain situations: • involvement of the biliary tree above the bifurcation in which PTC can better localize the proximal extent and access the injured duct
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Recurrent pyogenic cholangitis
• suspicion of an excluded segment of the biliary tree with absent communication between the liver and the distal bile duct • MRCP is currently best initial step for defineation of proximal biliary injuries and excluded segments
Management 1 Goal of therapy for bile leaks is to reduce outflow resistance in to the duodenum • procedure of choice is ERCP with stent placement, with or without sphincterotomy • stent does not need to bridge the site of the leak 2 Percutaneous or operative drainage may be needed for large bile collections. 3 Antibiotics should be administered until leak is controlled or drained. 4 Surgical treatment: • for major injuries, ligation of bile duct and creation of Roux-en-Y connection between proximal biliary tree and jejunum • smaller injuries may be repaired by suturing bile duct over a T-tube • treatment of a complete transection of the bile duct during surgery by suturing over a T-tube is rarely successful in the long term 5 Endoscopic therapy of postoperative biliary strictures: • no prospective randomized trials • there are reports of successful treatment with stent placement followed by stent exchanges every 3–6 months for at least 1 year • published series differ on the need for balloon dilation of a stricture prior to stent placement • series agree that good long-term results are achieved in 50–80% of patients at expert centers • retrospective comparison of patients treated endoscopically or surgically has revealed similar results; surgery was better in one series • successful treatment of post-liver transplant bile leaks has been reported
Recurrent Pyogenic Cholangitis Overview 1 The syndrome is characterized by primary intrahepatic stones associated with strictures of intrahepatic ducts. 2 Predominantly seen in the Far East; rare in the USA. 3 Stones composed mainly of calcium bilirubinate. 4 The syndrome was known previously as Oriental cholangiohepatitis.
Pathogenesis 1 Syndrome associated with bacterial infection of bile • bacterial beta glucuronidases hydrolyze conjugated bilirubin • unconjugated bilirubin binds with calcium and precipitates as calcium bilirubinate, the major constituent of intrahepatic stones • parasitic infections, such as Ascaris or Clonorchis, may play a role 2 Seen in rural rather than urban areas in endemic countries • low-protein diet prevalent in rural areas decreases biliary glucurolactone, an inhibitor of beta glucuronidase • the effect is enhanced endogenous beta glucuronidase activity with further deconjugation of bile, leading to precipitation of calcium bilirubinate
Diseases of the bile ducts
Presentation 1 Occurs at younger age than Western gallstone disease • may even occur in young adults and children 2 Clinical features • abdominal pain • jaundice • infection 3 Laboratory features • leukocytosis • elevated serum alkaline phosphatase and bilirubin levels 4 Potential consequences • liver abscesses • atrophy of affected liver segments
Diagnosis 1 Ultrasonography or CT scan may reveal focal areas of dilated intrahepatic ducts as well as stones 2 Definitive diagnosis requires ERCP or PTC
Treatment 1 Broad-spectrum antibiotics (e.g., piperacillin/tazobactam 3.375 g, i.v. every 6 h, or ciprofloxacin 500 mg, i.v. every 12 h) to treat episodes of acute cholangitis 2 Long-term relief includes surgical options tailored to individual patient • resection of atrophic hepatic segments (possibly even lobectomy) and diseased ducts draining affected liver portion • anastomosis of jejunum to intrahepatic segments proximal to sites of obstruction • creation of permanent access through which subsequent therapeutic maneuvers may be performed • creation of a T-tube tract or loop of jejunum brought to subcutaneous site to which bile duct has been anastomosed 3 Endoscopic therapy • proximal location of strictures and stones makes endoscopic approach difficult • PTC generally preferred to ERCP • some successful reports of endoscopic therapy
Choledochal Cysts Introduction 1 Anomalies of the biliary tree characterized by cystic dilatation of variable portions of the intrahepatic and/or extrahepatic ducts 2 More common in Asia than elsewhere 3 Female to male ratio of 3:1 4 Primarily affects children and young adults, but reported age ranges vary greatly
Etiology – proposed theories 1 Abnormality in biliary epithelial proliferation when fetal ducts are solid – leads to abnormally dilated portion proximal to a normal or stenotic distal portion 2 Distal CBD stenosis inducing proximal cystic dilatation 3 Intrinsic autonomic dysfunction • based on finding of deficient postcholinergic neurons in some portions of cyst wall
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Choledochal cysts Fig. 33.3 The normal anatomy (left) contrasted with the anomalous pancreaticobiliary function (arrow) believed to be responsible for free reflux of pancreatic enzymes and choledochal cyst formation. Modified from O’Neill JA. Choledochal cysts. Curr Prob Surg 1992; 29:365–410.
4 Anomalous pancreaticobiliary junction (see Fig. 33.3) • may result in lack of normal sphincter function and reflux of pancreatic enzymes into bile ducts, inducting progressive damage and dilatation • common in type I cysts, but not in type II, III, and V
Classification by Todani et al. (1977): the most commonly used system (see Fig. 33.4) 1 Type I – dilatation of the extrahepatic duct alone • most common type 2 Type II – diverticulum of extrahepatic bile duct 3 Type III – choledochocele, involving only the intraduodenal duct 4 Type IVA – multiple extrahepatic and intrahepatic cysts 5 Type IVB – multiple extrahepatic cysts only 6 Type V – single or multiple intrahepatic cysts (Caroli’s disease)
Clinical symptoms and signs 1 Right upper quadrant pain 2 Jaundice • often sole symptom in infants 3 Palpable abdominal mass 4 Fever 5 Epigastric or diffuse abdominal pain if pancreatitis present
Diagnosis 1 Ultrasonography or CT scan may reveal or suggest diagnosis MRCP emerging as diagnostic modality 2 Direct cholangiography by ERCP or THC usually diagnostic • also important for classification and planning therapy 3 THC best for delineating and potentially draining proximal biliary ducts 4 ERCP enables evaluation of pancreatic duct and pancreatic-biliary junction • often reveals anomalous junction, especially in type I cysts • MRCP obviates ERCP in many cases
Complications 1 Stone formation within cysts 2 Cholangitis and liver abscesses 3 Acute pancreatitis, with or without stones • most common with a choledochocele
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Diseases of the bile ducts
Fig. 33.4 Classification scheme of choledochal cysts suggested by Todani et al., 1977. From: Savader SJ, Benenati JF, Venbrux AC, et al. Choledochal cysts: classification and cholangiographic appearance. Am J Rad 1991; 156:328.
4 Secondary biliary cirrhosis 5 Carcinoma • usually occurs within cysts • significant risk after nonresectional surgery • primary cyst excision reduces cancer risk markedly but not completely • preoperative diagnosis of cancer is rare • poor prognosis because of extensive spreading 6 Portal hypertension 7 Cyst rupture with bile peritonitis
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Choledochal cysts
Treatment Medical management is associated with high morbidity and mortality Simple drainage is inadequate Cyst excision and reconstruction of biliary tree is the treatment of choice Type I and Type II cysts • excision followed by reconstruction of the biliary tree with Roux-en-Y hepaticojejunostomy • reports of successful laparoscopic approach 5 Type III cysts (choledochoceles) • successful endoscopic management by sphincterotomy has been described 6 Type IV cysts • excision of the extrahepatic cyst, partial resection of intrahepatic cysts when present, and hepaticojejunostomy • predominant involvement of the left lobe in type IVA disease may necessitate left hepatic lobectomy 7 Type V cysts (Caroli’s disease) • partial hepatectomy for localized disease • Roux-en-Y hepaticojejunostomy with placement of transhepatic stents for diffuse disease • recurrent stones and strictures are treated with percutaneous techniques • liver transplantation may be required for severe diffuse disease 1 2 3 4
Further Reading Abboud PAC, Malet PF, Berlin JA, et al. Predictors of common bile duct stones prior to cholecystectomy: a metaanalysis. Gastrointest Endosc 1996; 44:450–459. Coppola R, Riccioni ME, Ciletti S, et al. Selective use of endoscopic retrograde cholangiography to facilitate laparoscopic cholecystectomy without cholangiography. A review of 1139 consecutive cases. Surg Endosc 2001; 15:1213–1216. Fan ST, Lai ECS, Mok FPT, et al. Early treatment of acute biliary pancreatitis by endoscopic papillotomy. N Engl J Med 1993; 328:228–232. Folsch UR, Nitsche R, Ludtke R, et al. Early ERCP and papillotomy compared with conservative treatment for acute biliary pancreatitis. The German Study Group on Acute Biliary Pancreatitis. N Engl J Med 1997; 336:237–244. Freeman ML, Nelson DB, Sherman S, et al. Complications of endoscopic biliary sphincterotomy. N Engl J Med 1996; 335:909–918. Geenen JE, Hogan WJ, Dodds WJ, et al. The efficacy of endoscopic sphincterotomy after cholecystectomy in patients with sphincter of Oddi dysfunction. N Engl J Med 1989; 320:82–87. Kaw M, Al-Antably Y, Kaw P. Management of gallstone pancreatitis: cholecystectomy or ERCP and endoscopic sphincterotomy. Gastrointest Endosc 2002; 56:61–65. Lauter DM, Froines EJ. Laparoscopic common duct exploration in the management of choledocholithiasis. Am J Surg 2000; 179:372–374. Liu CL, Lo CM, Chan JK, et al. Detection of choledocholithiasis by EUS in acute pancreatitis: A prospective evaluation in 100 consecutive patients. Gastrointest Endosc 2001; 54:325–330. Neoptolemos JP, Carr-Locke DL, London, et al. Controlled trial of urgent endoscopic retrograde cholangiopancreatography and endoscopic sphincterotomy versus conservative treatment for acute pancreatitis due to gallstones. Lancet 1988; ii:979–983. O’Neill JA. Choledochal cysts. Curr Prob Surg 1992; 29:365–410. Park JS, Kim MH, Lee SK, et al. Efficacy of endoscopic and percutaneous treatments for biliary complications after cadaveric and living donor liver transplantation. Gastrointest Endosc 2003; 57:78–85. Pitt HA, Kauffman SL, Coleman J, et al. Benign postoperative biliary strictures. Ann Surg 1989; 210:417. Prat F, Amouyal G, Amouyal P, et al. Prospective controlled trial of endoscopic ultrasonography and endoscopic retrograde cholangiography in patients with suspected common bile duct lithiasis. Lancet 1996; 347:75–79. Ranson JHC, Rifkind KM, Roses DF, et al. Prognostic signs and the role of operative management in acute pancreatitis. Surg Gynecol Obstet 1974; 139:69. Rhodes M, Sussman L, Cohen L, et al. Randomised trial of laparoscopic exploration of common bile duct versus postoperative endoscopic retrograde cholangiopancreatography for common bile duct stones. Lancet 1998; 351:159–161.
Diseases of the bile ducts Savader SJ, Benenati JF, Venbrux AC, et al. Choledochal cysts: classification and cholangiographic appearance. Am J Rad 1991; 156:328. Sperling RM, Koch J, Sandhu JS, et al. Recurrent pyogenic cholangitis in Asian immigrants to the United States: natural history and role of therapeutic ERCP. Dig Dis Sci 1997; 42:865–871. Strasberg S, Hertl M, Soper NJ. An analysis of the problem of biliary injury during laparoscopic cholecystectomy. J Am Coll Surg 1995; 180:101–125. Taylor AC, Little AF, Hennessy OF, et al. Prospective assessment of magnetic resonance cholangiography for noninvasive imaging of the biliary tree. Gastrointest Endosc 2002; 55:17–22. Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases, including cancer arising from the choledochal cyst. Am J Surg 1977; 134:263–269.
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