Biliary System Patrick G. Jackson, Stephen R.T. Evans Anatomy and Physiology General Considerations in Biliary Tree Pathophysiology Benign Bil Malignant i Metastatic and Other Tumors ANATOMY AND PHYSIOLOGY ‘Asanatomic variations in biliary anatomy are common, occurring, {n up co 30% of patients, understanding of both normal anatomy and the variations is important for the management of patents with biliary disease. The ampulla of Vater contains che distalmost portion of the common bile duet and inserts into the wall of the duodenum. The pancreatic duct also joins the ampulla and may fuse with the bile duct before passing through the wal of the duodenum or within the wall of the duodenum, or ie may have separate orifice within the ampulla (Fig. 541). The most inferior portion of the common bile duct is encompassed by che head of the pancreas. Superior ro the intrapancreatc portion, che common bile duct is divided into retroduadenal and supraduodenal seg- ‘ments, "The insertion of the eytie duet marks the differentiation of the common hepatic duct above and the common bile duct below. ‘The cystic duct drains che gallbladder, which is divided into the neck, infundibulum with Hartmann pouch, body and fundus. Roughly the size and shape of common light bulb, the gallblad- der holds 30 to 60 ml. of bile as an extrahepatic reservoir. The sallbladder is attached to the inferior surface of the liver and is enveloped by liver for a variable portion of is circumference Although some gallbladders are almost enveloped by liver paren- chyma, others hang on a mesentery, predisposing to volvulus. The attachment of the gallbladder to the liver, known as the gallblad- der fossa, identifies the separation of the left and right lobes of the liver (Fig 54-2), Where the gallbladder attaches ta the liver, Glisson capsule does not form, and this common surface provides most of the venous drainage of the gallbladder. The cystic duet drains ac an acute angle into dhe common bile duct and can range from 1 to 5 cm in length. There are a number of anatomic vari tions in insertion of the cystic duct, including into the right, hepatic duce (Fg. 5-3). Within the neck of the gallbladder and cystic duets lie folds of mucosa oriented in a spial pattern, known asthe spiral valves of Heister, which act to keep gallstones from encering the common bile duct in spite of distention and incra- uminal pressure. The dependent portion of Hartmann pouch ray overlie che common hepatic or right hepatic ducts, thus 1482 placing these structures at risk during he performance of a holecystectomy. “Above the eystic duct lies the common hepatic duct, draining the left and right hepatic duct systems. The confluence of these suctures lies atthe hilar plate, which isan extension of Glisson capsule. The absence of any vascular struccures overlying che bile ‘ducts at this location allows exposure of the bifurcation by inci sion of this layer at the base of segment IV, lifting the liver off these structures, known as lowering the hilar plate this is generally used to expose the proximal extrahepatic biliary tree for resetion Vascular Anatomy “The segmental anatomy of the liver parenchyma is based on the vascular supply and drainage, and the biliary drainage is described, by the corresponding vascular segment. The hepatic parenchyma is divided into lobes, each of which is divided into lobar segments (Fig, 54-4) co define the basic hepatic anatomic resections. The left lobe is composed of medial and lateral segments. ‘The right lobe is divided into posterior and anterior segments. Alternatively, the hepatic parenchyma can be divided into segments based on the specific hepatic venous drainage and portal inflow, allowing a ‘mote precise description of anatomic pathology as described by ‘Couinaud.’ The three hepatic veins divide che liver into four sepa- rate sectors. Each sector is then subdivided by the insertion of the portal vein, resulting in eight segments. In this classification system, the liver is composed of eight segments. Segment I refers, tothe caudate lhe. "The left lahe ofthe liver, supplied by the lefe portal vein, constitutes segments II through IV. ‘The left lobe is further subdivided by the faliform ligament, which separates segments IT and II, also known as the left lateral segment, from, segment IV. Within the lfc lateral segment, segment Iles su rior to the insertion of the portal ein and segment III lies inferior to it, Segment IV is similarly divided into segmene IVA above and segment IVB below the portal vein insertion. The right portal vein supplies the right lobe of ee liver and divides ie into the posterior and anterior sectors. Each sector is then subdivided on the bass, ofits relative location compared with the portal vein, Segmenc V is supplied by the inferior branch of the anterior sector, andFIGURE 54-1 Patterns of bilary ductpencreatic duct juncton and insertion inte the duadenal wal FIGURE 54-2 Laparoscopic photogreph ofthe gallbladder in situ. The ‘galolader is being suspended by the fundus to expose the infundibu- lum and porta nepats. segment VII is supplied by the superior branch, Inthe posterior sector, segment VI is supplied by the inferior branch and segment VII is supplied by the superior branch, There are three major hepatic veins that drain inco the inferior vena cava in addition to ‘number of small veins thar drain direcely from the right lobe. “The right hepatic vein constitutes most of the venous drainage fiom the tight lobe and generally lies in the intersegmental fissure beoween the anterior and posterior sectors ofthe right lobe. The riddle hepati vein drains the medial segment of the left lobe and 4 small amount of the medial portions of segments V and VIIL In most eases, the middle hepatic vein fuses with the left hepatie vein that drains the lft Lateral segment ‘As opposed co the hepasie parenchyma, where most perfusion ‘comes from portal venous flow, the enti biliary tree is supplied solely by the arterial anatomy. This anatomic arrangement makes FIGURE 54.3 Variability in cystic duct anatomy. Knowledge of these variations 's important to ty to avoid inadvertent injury to the biliary tree curing eholecystectorny. ic particularly susceptible to ischemic injury athe intrahepatic and extrahepatic levels. The inferior bile duct, below the level of the duodenal bulb, receives its perfusion from tributaries of the posterosuperior pancreaticoduodenal and gastroduodenal arteries. ‘The smal! branches coalesce to form the two vessels chat run along, the common bile duet at the 3- and 9-o'elock positions. With close dissection of the areolar tissue surrounding che bile duct, these vessels can be damaged, leaving the bile duct at tsk for ischemic injury. The supraduodenal common bile duct, from the duodenal bulb co the eystic duct, and common hepatic ducts receive their blood supply from the right hepatic and eystiearter~ ies. As che proper hepatic artery ascends on the anterior medial side ofthe porta, it divides inco right and left hepatic arteries. In ‘most cases, the right hepatic artery passes posterior to the common hepatie duct co supply the right lobe of the liver. After crossing the duct, che right hepatic artery passes through the triangle of Calot, bordered by the cystic dues, common hepatie duct, and ceige of liver. In this uiangle, the right hepatic artery gives off the etic arery to the gallbladder and is ar risk for injury during a cholecystectomy. An accessory of replaced right hepatic artery ‘when present, passes through the portacaval space and ascends to the right lobe along she lateral aspect of the common bile duct, pulsatile structure on the most lateral aspect ofthe porta during, a Pringle maneuver identifies chis anomaly. In addition, it can be noted on computed tomography (CT) as a vessel passing tans- versely between the portal vein and inferior vena cava behind the head of the pancreas “The cystic artery normally arises from the right hepatic artery, which can pass posterior or anterior to the common bile duct to supply the gallbladder Similar to the variability ofthe eystie duct, the cystic artery may arise from the right hepatic, left hepatic, proper hepatic, common hepati, gastroduodenal, or superior mesenteric artery. Although variable, the cystic artery generally lies superior to the eystie duct and is usually associated with 2 lymph node, known as Calot node (Fig. 54-5). Because this node provides some of the lymphatic drainage of the gallbladder, ican be enlarged in the seuting of gallbladder disease, whether itis inflammatory oF neoplastic.SECTION X_ Abdomen FIGURE 54-4 Couinaud Segmental Anatomy. Segment | isthe caudate lobe. Segments Il and Il are supalied by the itera branch of the let portal vein, with segment Il ving sbeve the passage of the portal vein and segment Ill below it. Segment WV Is supaliea by the medial branch of furher intr ribution ofthe ante ‘super distribution of this branch. Sirlarly, with respect tothe posterior branch of the right portal vein segment VI las inferior fe the portal vain, wheraes segment Vl les superior, FIGURE 54-5 Operative photogragh of Calot nods. This node arrow! is useful for tentfieaton of the common lection ofthe cystic eter Both within the liver and immediately outside the paren- chyma, che bile ducts generally lie superior to the corresponding portal veins, which in turn are superior co the arterial supply (Fig, 54-6). Retaining a longer exctahepatic segment before inserting, inco the liver, che left hepatic duce travels under the edge of segment IV before slipping superior and posterior to che left portal vein, Duting this wanaverse portion, it can receive a few subsegmental branches fiom segment IV. The lefe duce drains segments II, IIL, and IV, with the most distal branch draining segment IVA. Further superolateral, the ducte draining segment IVB aise, and further up the let duct are the ducts for segments Mand IIE, These fused ducts can generally be found juse posterior and lateral to che umbilical recess. The caudate lobe drains through smaller ducts chat eneer the right and lefx hepatic duct systems, ‘The drainage of the right duct system includes segments V, VI, VIL, and VIII and is substantially shorter than the left duct, Difurcating almost immediately. The fusion of ewo sectoral ducts, postetior and antetior, creates this shore right hepatic duct, The anterior sectoral duce runs ina vertical direction to drain segments Vand VIII, whereas the posterior sectoral duct follows a horizon- tal course to drain segments VI and VIL Physiology Bile secretion from the liver serves wo opposing functions, namely, excretion of toxins and metabolites from the liver and absorption of nutrients ftom the intestinal tact. Bile is secreted into bile canaliculi, which enciele each hepatocyte. Within the hepatic lobule, chese canaliculi coalesce to form smal bile ducts, eventually enteringa portal triad. Four to six portal triads combine to create a hepatic lobule, the smallest functional unic of the liver, identified by ts central cerminal hepatic venue, On the opposi aspect from the canalicular surface of the hepatocyte lies the sinusoidal surface, which contacts the space of Disse. In this «area, the hepatocyte is responsible for the absorption of circulating components of bile, an importanc step in the entero hepatic citculation ofbile. Once the bile components are absorbed and secreted into the bile canaliculi, che tight junctions in che biliary tree keep these components within the bile secretory pathway. The secretion of bile components into the biliary Wee is a major stimulus to bile flow, and the volume of bile flow is an ‘osmotic process. Because bile salts combine to form spherial pockets, known as micelles, che salts themselves provide no ‘osmotic activity. Instead, the cations that aze secreted into the biliary Gee along with the bile salt anion provide the osmotic load to draw water into he duet and ro increase flow co keep bile cleetrochemically neutral. For this reason, bile maintains an osmo- Ialty approximately comparable to that of plasmaFIGURE 54-6 Hepatic lobar segmental bilary anatomy, Synthesis (0.2505 9a) Urinary excretion (<05 mais) [- Portal venous return (695% of Bilary secretion) Bilary eecteton = pool x cycles (12-96 gle) (8.9) x (4-1240) Fecal excretion \ | @zaseis FIGURE 54-7 Enrerohepstic circulation Although a small amount of bile flow is bile salt independen. sceving co expel toxins and metabolites from the body, much of the flow is dependent on neural, humoral, and chemical stinouli Vagal actviey induces bile secretion, as does the gastrointestinal hormone secretin. Cholecystokinin (CCK), secreted by the intex tinal mucosa, serves to induce biliary wee secretion and gallblad- der wall contraction, thereby augmenting excretion of bile into the intestines Bile salts, such as cholic acid and deoxycholic acid, are origi- nally created from cholestrol and secreted into bile canalieuli as cholic acid and its metabolite, deoxycholie acid. ‘The liver actually makes only a small amount of the total bile salt pool tused on a daily basis because most bile salts are recycled after tuse in the incstinal lumen, known as the enterohepatic ciccula- tion (Vig. 54-7). After passage into the intestinal eract and reabsorption by the terminal sleum, bile acids are transported back to the liver for reeling bound to albumin. Less than 5% of bile salts are lost each day in the stool, When sufficient quanti- ties of bile sale reach the colonic lumen, the powerful detergent activity of the bile salts can cause inflammation and diarthea.a SECTION X_Abdomen This can sometimes be seen after a cholecystectomy when the speed of the enterohepatic circulation of bile increases and ray overwhelm the ability of the terminal ileum to absorb bile sale. “The passage of reabsorbed bile salts bound to albumin through the space of Disse allows uptake into the hepatocyte in an efficient process thacinvolves sodium cotransport and soditim-independent pathways. In the less specific sodium-independent pathway, a umber of organic anions are transported, including unconju- gated and indirect bilirubin. The transport of bile sats across the Canalicular membrane remains the rate-limiting step in bile salt excretion, Given the vast differences in concenttation of bile salts the transport of bile up an extreme concentration gradient is adenosine triphosphate dependent. In addition tile salts bile contains proteins, lipids, and pig ments, The major lipid components of bile are phospholipids and cholesterol. These lipids not only dispose of cholesterl from low- and high-density lipoproteins bu also serve to protect hepatocytes and cholangiocytes from the toxic nature of bile. The sources of most biliary cholesterol are citculating lipoproteins and hepatic synthesis. Theretore, the biliary secretion of cholestrol actually serves to excrete cholesterol from the body. Although cholesterol, bile salts, and phospholipids play an important role in nuttional homeostasis, bile also serves as a major route of exogenous and endogenous toxin disposal. One such example of the disposal system is thar of bilirubin. Bile pig- rents, sich as bilirubin, ae breakdown produets of hemoglobin and myoglobin. These are uansported in the blood bound albumin and transported into the hepatocyte, Here, they are transferred into the endoplasmic reticulum and conjugated co form bilirubin glucuronides, also known as conjugated or direct bilizubin, Ie is th bile pigments that give the color t bile and, when converted to urobilinogen by bacterial enzymes in che ines” tines, give stool its characteristic color In the fasting state seereced bile will passthrough the biliary tree into the intestine and be reabsorbed. In addition, bile will collect in the gallbladde, which serves as an exttahepatic storage site of secreted bile, To store bile secretions, the gallbladder is extremely efficient in water absorption and thus concentration of bile components. This absorption isan osmotic process performed through the active sodium wansport. With the absorption of sodium and water across the gallbladder epithelium, the chemical composition of bile changes in the gallbladder lumen, Ineveases in cholesterol concentration, in addition to calcium, whieh is not as eflcienly absorbed, then lead co decreased stability of phos pholipid cholesterol vesicles, The reduced vesicle sability predis- poses co nucleation of this stagnant pool of cholesterol and thus to cholesteral stone formation. ‘The gallbladder neck and cystic duct also secrete glycoproteins to help protect the gallbladder from the detergent activity of bile. These glycoproteins also promote cholesterol crystallization, “The gallbladder fills through a retrograde mechanism. With an increas in the tonic activity of the sphincter of Oddi i the fasting state, pressute increases in the common bile duct. This ineseased pressute allows filling of che lower intraluminal pressutegallblad- der, which is capable of storing up to 600 ml of the daily produc- tion of bile. ‘The pasage of fat, protein, and acid into the duodenum induces CCK secretion fom duodenal epithelial cll CCK. as its name suggests, then causes gallbladder contract with ineralumsinal pressures up co 300 mm He, Vagal activity al induces gallbladder emptying but is a less powerful stimulus to sallbladder contraction than CCK. Common tie uct Pancreatic duct FIGURE 54-8 Sphincter of Odd. Because the sphincters resvonsile for contrl of most tile flovs, this sphincter maintains @ hgh onc con. rection but is inhibited by CCK, “The distal portion ofthe bile duct pases through the sphincter of Oddi (Fig. 54-8). The musculature of this sphincter is inde- pendent from that of the duodenal intestinal wall and responds differently to neurohumoral controls, This muscular sphincter, ‘which normally maincains high tonic and phasic activi is inbib- ited by CCK. With CCK-induced relaxation ofthe sphincter, bile flows more readily from the biliary tee. Coordinated with gall bladder contraction, the relaxation ofthis sphincter allows evac ation of up c0 70% ofthe gallbladder contents within 2 hours of CCK secretion. During the fasting state, che oblique passage of the bile duct through the duodenal wall and the tonic activity of the sphincter prevent duodenal contents from refluxing into the biliary cee GENERAL CONSIDERATIONS IN BILIARY TREE PATHOPHYSIOLOGY ‘Symptoms “The Charcot triad of right upper quadrant pain, fever, and jaun- dice describes the three most common symptoms associated with biliary disease. With the blockage of any tubular structure, pain may come from acute increased intraluminal pressure of from inflammation. Obstruction will generally precede infection Decause stasis of bile is an inciting factor of biliary infection along with sufficient quantity of infectious inoculum in a susceptible hos. Pain Postprandial abdominal pain is generally termed biliary colic, actually a misnomer beeause the pattern of pain is not colicky in nature. Because the nerve fibers tothe gallbladder originate in the celiac axis, this pain can be epigastric in origin or may locate in the right upper quadrant as the inflammatory process affects the parietal peritoneum. As a meal containing fat or protein enters the duodenum, CCK is released, causing contraction ofthe gall bladder and increases in bile secretion. When the gallbladderCHAPTER 54_ Biliary System lumen cannot fully empty because of a stone in the gallbladder neck, viscera pain fibers ate activated, causing pain in the epigas rium of tight upper quadrant. The same luminal obstruction of biliary colic bue associated with sufficient stasis, pressure, and bacterial inoculum creates infection and thereby inflammation, therefore progressing to acute cholecystitis. With this infection and inflammation, the right upper quadrant pain of biliary coli will be accompanied by tenderness noted on palpation of the right upper quadrant. Specifically the voluntary cessation of respiration shen the examiner exerts constant pressure under the right costal margin, known as a Murphy siga, suggests inflammation of the visceral and parietal peritoneal surfaces and can be seen in diseases such as acute cholecystitis and hepatcis. Alternatively, biliary colic in the absence of infection and inflammation is not associated ‘with any reproducible physical examination finding or systemic symptom. Fever Whereas biliary colic does not produce ystemie manifestations, infection or inflammation in the gallbladder o biliacy tee will usually cause fever Tecan be seen in a number of inflammatory diseases, but fever associated with right upper quadrant pain is 3 hallmark of an infectious process in the biliary tree. Wich immedi- ate and ditcet access to the metabolically active hepatie pate chyma, infection of the gallbladder and biliary tree induces cytokine seeretion and thereby diect systemic manifestations Jaundice Jaundice, caused by elevation of the serum bilirubin level, can be demonstrated in the sclera, the frenulum of the tongue, or the skin. Serum bilirubin levels above 2.5 mgldl. are necessary to deveet scleral icterus routinely, and levels above 5 mg/dL will be ‘manifested as cutaneous jaundice, Failure co excete bile from the liver inco che intestines is a prerequisite of jaundice. Therefore, although both are associated with fever and pain, acute cholecys- titis does not cause the jaundice seen in infection of the biliary tuce, known as ascending cholangitis. The constellation of fever, right upper quadrane pain, and jaundice, known as Charcot iad, suggests blockage of the biliary secretion from the liver, not just the gallbladder. With the addition of hypotension and altered mental status, known as Reynolds pentad, patients will demon surate the systemic manifestations of shock ftom biliary origin, Jaundice is generally divided into surgical, from obstruction, and medical, from a hepatocellular process Laboratory Tests Although termed liver function tests, the routine hepatic panel for most laboratories tests a number of aspects of metabolic and hepatic activity. The tests most useful for evaluation of biliary physiology include determination of levels of bilirubin and alks line phospharase, seen in any cholestatic proces, and serum tans aminases, suggesting evidence of hepatocellular injury. Bilirubin can be subdivided into the conjugated and unconjugated forms, thereby allowing delineation of cause based on cellular location of derangement. In other words, hyperbilirubinemia may be caused by increased synthesis of bilirubin, impaired hepatocyte uptake of unconjugated bilirubin, decreased intracellular conjuga~ tion, reduced intracellular ransport and excretion of conjugated bilirubin, oF obstruction of che biliary kee. Although this isan oversimplification of a complex process, derangements up co and including conjugation will be manifested as clevated tnconju- sated bilirubin levels Imaging Studies Plain Films Plain radiographs are of limiced use in the overall evaluation of biliary tree disease. Gallstones are not egularly seen by plain films, and even when they are seen, it rarely changes therapy. Therefore the role of plain radiographs in the evaluation of possible biliary disease is imived to exclision of other diagnoses, such as a duo denal ulcer wich free ais, small bowel obstruction, or right lower lobe pneumonia causing right upper quadrant pain, Ultrasound Transabdominal ultrasound is a sensitive, inexpensive, tliabe, and reproducible test to evaluate most of the biliary tee, being able t0 separate paticnts with medical jaundice, in which the source of hyperbiliubinemia is from hemoglobin breakdown through the process of conjugation, ftom those with surgical jaundice, in which the hyperbilieubinemia occurs from a blockage of excretion. Therefor, this modality is seen as the study of choice for the initial evaluation of jaundice or symptoms of biliary disease, Te finding of a dilated common bile duct in the setting of jaundice suggests an obstruction of the duet from stones, casually associaced with pain, or from a tumor, which is commonly painless (Fig. 54-9). Gallbladder diseases are regularly diagnosed by uluasound because che superficial location of the gallbladder ‘with no overlying bowel gas enables its evaluation by sound waves, Ulerasound has 2 high specificity and sensitivity for cholelithiasis, x gallacones. The density of gallstones allows erisp reverberation of the sound wave, showing an echogenic focus with a character istic shadowing behind the stone (Fig, 54-10). Most gallstones, tunlss impacted, wll move with positional changes in the patient “This feature allows ther differentiation from gallbladder polyps. which are fixed, and fiom sludge, which will move more slowly and does not have the sharp echogenic pattern of gallstones, Pathologic changes sen in many gallbladder diseases canbe iden- tified by ultrasound. For example, the gallbladder wall thickening and pericholeeystie fluid seen in cholecystitis are visible by uluae sound (Fig, 54-11). Porcelain gallbladder, with its califed wall, will appear asa curvilinear echogenic focus along the ence gal- wall, with posterior shadowing (Fig. 5412). In addition FIGURE 54-9 Utvasound image of diated bilary tree. The common bile duct (CBD) is alated, As travels paraliel o the portal vein PV helps ensureSECTION X_ Abdomen LONG GB DEC FIGURE 54-10 Ultrasound image of a gallstone inthe galblader neck. Te sharp echagenic wal ofthe galstone faraw/, with the character iste posterior shadowing stipe under the store, helps differentiate it ‘rom otnerntaluminal ining. Xtc FIGURE 54-11 Utrascund image with acute cholecystitis and thick ‘ened galbldcer wal (arows) to division of medical versus surgical jaundice, ultrasound can sometimes identify the cause of obstructive jaundice, showing ‘common bile duct stones of even cholangiocarcinoma, Hepatic Iminodiacetic Acid Scan Although incapable of providing any precise anatomic delinea- tion, biliary scintigraphy, alzo known as a hepatic iminodiacetic acid (HIDA) sean, can be used to evaluate the physiologic secre tion of bile, The injection of an iminodiacesic acid, which is processed in the liver and secreted with bile, allows identification of bile flow. Therefore, the failure to fill the gallbladder 2 hours alter injection demonstrates obstruction ofthe cystic duct, as seen in acute cholecystcis ( and 54-14), In addition, the scan will identify obstruction of the biliary tree and bile leaks, which may be useful in the postoperative esting. HIDA scans can also be used to determine gallbladder function because the injec tion of CCK during a scan will document physiologic ejection of the gallbladder. This may be useful in patients with biliary cract pain but without stoner because some patients have pain from impaired emptying, known az biliary dyskinesia, Az a nuclear ces FIGURE 54-12 Ultrasound image of porcelain gallbladder. The cur near sharp achageric focus (arrow) combined with substantal post rior shadowing helps confirm this dsgnos's. “4 Fe 1-5 Durston 300 s6e yy "F610" Duration: 300388 —é ve FIGURE 54-13 HIDA scon showing filing of the galllodser. Win gallacer filing farcws), the diagnosis of acute cholecystitis is effec. tively eliminated. -é v 21-25 Duration: 300 s0e medicine tert, the test demonstrates physiologic How but docs not provide fine anatomic detail, nor can it identify gallstones. Computed Tomography Although ultrasound is clearly che first test of choice fo tion of biliary disease, CT provides superior anatomic info tion and therefore is indicated when more anatomic delineation is requized, Because most gallstones ate radiographically isodense to bile, many will be indistinguishable from bile. However, because ultrasound is operator dependent and provides no ana- tomic reconstruction of the biliary tree, CT can be used (0CHAPTER 54_ Biliary System ad ee e a FIGURE 54-14 HIDA scan showing nonfling of the gallbladder. With no filing of she galoladser (arows) even on delayed images, HIDA firms eeclusion ofthe cystic duct, tho characteristic feature of elect a of 5 @? FIGURE 54-15 CT scon showing dilsted bilory vee (arrow at the portal confuence, This dilation continued cown to the head of the identify the cause and site of biliary obsteuction (Fig, 54 “When itis performed for the evaluation of hepatic or parenchyma or possible neoplastic processes, CT is im preoperative planning, and the wee of arterial phase, portal venows phase, and delayed phase imaging, known as a wiple-phase CT, has essentially seplaced diagnostic angiography of the liver. Magnetic resonance imaging uses the water in bile vo delineate the biliary tee and thus provides superior anatomic definition of the intrahepatic and extrahepatic biliary tree and pancreas. Although management of most patients with biliary disease FIGURE 54-16 Normal MRC® image. Note the normal comman bile vet [CBD and pancreatic duct PD) does not sequite the fine detail of anacomie evaluation shown by cross-sectional imaging, magnetic resonance imaging is noninva- sive, requires no radiation exposure, and can prove extremely ‘useful in planning resection of biliary ot pancreatic neoplasms oF management of complex biliary disease, By use of the water content of bile, a cholangiopancreatogram can be crcaced (Fig, 54-16), which makes imaging of the biliary eee. an excellent modality for cross-sectional Endoscopic retrograde cholangiopancreatography (ERCP) i an invasive test using endoscopy and luorascopy to inject contrast material through the ampulla co image the biliary eee (Fig. 54-17), Although it does catty a complication rate of up to 10%, its usefulness lies in its ability t0 diagnose and to reat many diseases of the biliary tee. For patients with malignant obstrac- tion, ERCP can be used ¢o provide tissue samples for diagnosis while also decompressing an obstruction, but it does not scage disease accurately. Many benign diseases, such as choledocholi- thiasis, can be easly treated by endoscopic means. ERCP has also proven exttemely useful in the diagnosis and treatment of com- plications of biliary surgery Interventional radiologic techniques can be used in the evaluation, of biliy anatomy. Similar to ERCP, percutancous transhepati cholangiography (PTC) is an invasive procedure used to evaluate the biliary tee. A needle is passed directly into the liver to access fone of the biliary radicals, and the tact is then used for insertion of transhepatic catheters, Usefal for patients with intrahepatic biliary disease or in whom ERCP is not technically feasible, PTC can decompress biliary obstruction, stent obstructions nonopera- tively, and provide anatomic information for biliary reconstrac- tion (Fig. 54-18).SECTION X_ Abdomen FIGURE 54-17 Normal ERCP image. Intraoperative Cholangiography Another imaging tool for the diagnosis of bilisty tact abnormal ‘ies is intraoperative cholangiography. With the injection cathecer inserted through the eystic duct during 2 cholecystectomy of through another point in the biliary sree, intraoperative cholan- giography can help delineate anomalous biliary anatomy, identify choledocholithiasis, or guide biliary reconstruction, Some sur aeons advocate routine cholangiography during cholecystectomy. Advocates for routine cholangiography note that common duct injuries can be identified and managed immediately when chol- angiography is used routinely. However, because it adds operative time and fluoroscopic exposure to the operation, many surgeons use intraoperative cholangiography selectively during the perfor- ance of a cholecystectomy: Although debated, the routine use of intraoperative cholangiography does not reduce significantly the incidence of injury to she biliary ce during laparoscopic cholecystectomy. Indications for the selective use of cholangiog- raphy include pain on the day of operation, abnormal hepatic function panel, anomalous ot confusing biliary anatomy, and alteration in anatomy that precludes che ability co perform ERCP alter cholecystectomy, such as Roux-en-Y gastric bypass, dilated biliary tree, or any preoperative suspicion of choledocholithiasis Endoscopic Ultrasound Although of limied use in the evaluation of gallbladder disease or intrahepatic disease of the biliary eee, endoscopic ultrasound is valuable in the assessment of distal common bile duct and ampulla, Wich the close apposition of the distal common bile duct and pancreas to the duodenum, sound waves generated by endo- scopic ultrasound provide detailed evaluation ofthe bile duet and ampulla: this has proved most useful in assessing tumors for inva sion int vascular structures. Echoendoscopes ate subdivided into those that scan perpendicular to the long axis of che endoscope, known as radial echoendoscopes, and those chat scan parallel, known a linear echoendoseopes. Radial echoendoscopes are most FIGURE 54-18 PIC image of hepatic ilary anatomy, Serene ac) Crier Pain ating of aperation ‘Abormal hepa ution panel Aromabous or contusing bir anatomy Inabty to perform postoperative RCP Dilated ary ee ‘Any suspicion ofeoledocholtiasis ‘useful for providing a tomographic evaluation, whereas linear echoendoscopes can guide interventions such as needle biopsies under rea-rime ultrasound guidance ( ). Fluorodeoxyglucose Positron Emission Tomography Fluorodeoxyglucose positron emission tomography (FDG PET) exploits the mecabolic difference between a highly metabolically active tissue, sich as a neoplasm, and normal tissue, With the injection ofa radiolabeled glucose molecule, FDG PET scans can differentiate benign and malignant lesions, detect recurrence, and identify metastatic disease. Unfortunately, FDG PET is incapable ‘of demonstrating carcinomatosis and, given the high metabolism fof the immune system, ie of limited vale in the setting of infec- tion of inflammation, Bacteriology ‘he biliary tee inserts into the duodenum and therefore cannot bbe considered sruly sterile. Through a low bacterial load, and withCHAPTER 54_ Biliary System FIGURE 54-19 Linear endoscopic ultrasound with biopsy ofa moh rode. the flow of bile, infection in the absence of obstruction is rare However, with the presence of stones or obstruction, the likeli- hood of bacterial infection increases. The most common types of bacceria found in biliary infections are Enterobacteriaceae, such, as Eicherichia col, Klebsiella, and Enterobacter, followed by Entero- occu spp. Prophylactic antibiotics should be used in mos patients under {going interventions in the biliary tree, such as ERCP ot PTC. To cover the most common bacterial species, a fitst- or second- {generation cephalosporin or fluoroquinolone should suffice. For those undergoing clective laparoscopic cholecystectomy for biliary colic, no antibiotic prophylaxis is nevessary. However, antibiotics should be used for any patient with suspected or documenced infection of the biliary re, such as acute cholecystitis or ascend- ing cholangitis, and should be chosen to cover gram-negative bacteria and anaerobes, BENIGN BILIARY DISEASE Calculous Biliary Disease By far, the most common disease state involving the gallbladder and biliary tfee i that of cholelithiasis. Because the gallbladder ‘concentrates bile, the concentration of solutes in che gallblad- der differs from that in the rest of che biliary tee. This increase in solute concentration combined with stasis in the gallbladder beeween meals predisposes to stone formation in the gallbladder. Gallstones can be subelassfied into ewo major subtypes, depend ing on the principal solute that precipitates into a stone. Moze than 70% of gallstones in Ametica ate formed by precipitation of cholesterol and calcium, with pute cholesterol stones accounting, for only a small (<10%) portion, Pigment stones, further subelar~ sified as black or brown stones, are caused by precipitation of ‘concentrated bile pigments, the breakdown products of hemoglo bin, Four major factors explain most gallstone formation: super saturation of secreted bile, concentration of bile in the gallbladder, crystal nucleation, and gallbladder dysmotilty. High concentra- tions of cholesterol and lipid in bile secretion from the liver ‘constitute one predisposing condition to cholesterol scone forma tion, whereas increased hemoglobin processing is seen in most patients with pigment stones. Once in the gallbladder, bile is ‘concentrated further through the absorption of water and sodium, FIGURE 54-20 Gallbladder with charactenstic yellow cholesterol stones, increasing the concentrations of the bile solutes and calcium. Bile salts act to solubilize cholesterol. With respect to cholesterol scones (Fig, 54-20), cholesterol precipitates out into crystals when the concentration in the gallbladder vesicles exceeds the solubility of cholesterol (Fig. 54-21)’ Crystal formation is further acceler- axed by pronucleating agents, including glycoproteins and immu noglobulins. Finally, abnormal gallbladder moclity can increase seass inthe gallbladder, allowing more time for solutes to precipi- tate in the gallbladder. ‘Therefore, increased stone formation can be seen in conditions associated with impaired gallbladder empeying, such as in prolonged fasting states, with use of total parenteral nutrition, after vagotomy, and with wse of somatostatin analogues. Pigment stones can be divided into black stones, as seen in hemolytic conditions and citthosis,and brown stones, which tend «o be found in the bile ducts and are thought to be secondary to infection. The difference in color comes from incorporation of cholesterol into the brown stones. Because black pigmet occur in hemolytic states from concentration of bilirub ae found almost exclusively in the gallbladder. Alternatively brown stones occur within the biliary tee and suggest a disorder of biliary moriley and associated bacterial infection Natural History allstones are asymptomatic, often being identified at the time of abdominal imaging for other reasons or during laparot- omy. To become symptomatic, the gallstone must obstruct a vis- ceral structure, such as the eystic duct. Billary colic, caused by cemporary blockage of the eystie duct, tends to oceur after a meal, in which the secretion of CCK leads to gallbladder contraction Stones that do not obstruct the cystic duct or pass through the entire biliary tree into the intestines without impaction do notSECTION X_ Abdomen 100 ey 60 \—————_»» “0 20 ° nt ble salt > FIGURE 54.21 Triangle of Solubility. With the thee mejor components of ble that determine cholesterl soluilty and stably, each can be quanttiad by melar percentage to show a relative rato tothe ether two, Crolesterols completely soluble in only he small area inthe lef lower corner, where a clear mice sol lion exists, below the closed cices, Just above this, n tho atea betwoen the open and clesed circles cholesterl is supersaturated but stable and thus cxysalizea only with stasis, Inthe reminder of tre andl, Cholesterol is sign{ieantly sugersaturatec ane unstable, In this region, crystals form immediately. (From Adiitand WH, Small DM! The physicochemical basis of cholesterol galstone formatian in man, Clin Invest 447-1003-1082, 1868) cause symptoms. Only 20% to 30% of patients with asymprom- atic stones will develop symptoms within 20 years, and because approximately 1% of patients with asymptomatic stones develop complications of their stones before onset of symptoms, prophy- lactic cholecystectomy is not warranted in asymptomatic patients Certain subsets of patients, howeves, consticute a higher tisk pool, s0 prophylactic cholecystectomy should be considered. ‘Among these are patients with hemolytic anemia, such as sickle cell anemia. These patients have an extremely high rate of pigment stone formation, and cholecystitis can precipitate a crisis, Patients witha calcified gallbladder wall (known as porcelain gallbladder), those with large (2.5 cm) gallstones, and chose with a long common channel of bile and pancreatic ducts all have a higher risk of gallbladder cancer and should consider cholecystectomy. In addition, patients with asymptomatic gallstones undergoing bariatric surgery may alo benefc fiom cholecystectomy. Not only does rapid weight loss favor stone formation, but also, ater gastric bypass, ERCP to remove common bile duct stones in ascending cholangitis is extemely challenging and usually unsuccessful Finally, because severe infection can be life-threatening in the immunocompromised patient, some ansplantation surgeons recommend prophylactic cholecystectomy before receipt of an organ wansplant Nonoperative Treatment of Cholelithiasis Medical erearment of gallstones is generally unsuccessful and is used rarely. Options include dissolution with oral ble sale therapy: contact dissolution, which requires cannulation of the gallbladder and infusion of organic solvent; and extracorporeal shock wave lithotripsy. With the dissolution strategies, unacceptable recur- rence rates of up £0 50% limic cheir application to the most select group of patients, Extracorporeal shock wave lithotripsy hae a lower recuztence tate, approximately 20%, and can be used in patients with single stones 0.5 co 2 em in size, The widespread ‘us, safery, and efficacy of laparoscopic cholecystectomy have rel- ‘gated nonoperative therapy to patients for whom general anes- thesia presents a prohibitively high risk. Chronic Cholecystitis Recusrent attacks of bility colic, with only temporary occlusion, of the cystic duct, can cause inflammation and scatting of the neck of the gallbladder and cystic duct. This process, called chronic chofecystitis, causes fibrosis as histologic evidence of repeated self-limited episodes of inflammation. The diagnosis of chronic cholecystitis lies along a continuum with biliary colic because i¢ results from recurrent attacks, Therefore, the presenta- tion is that of symptomatic cholelithiasis, or biliary colic. Pain occurring after ingestion of a farty meal, with the arcendant inerease in CCK secretion in response to duodenal intraluminal fat, is elasic for biliary coc, though only 50% of patients will 1eport an asvociation with food. Pain ftom stones cends to locate i the epigastrium or right upper quadranc and may radiate around to the scapula, ‘These attacks of pain generally last a few hours. Pain lasting longer than 24 houre or associated with feverUrascund image of cholestealoss suggests acute cholecystitis. The pain of biliary colic, even in the absence of cholecystitis, may also cause other gastrointestinal symproms, such as bloating, nausea, or even vomiting, Symptomatic tones constitute a risk profile different from that of asymptomatic stones, with a higher likelihood of complica ‘ions, Therefore, sympromatie cholelithiasis is an indication for cholecystectomy. To perform a cholecystectomy for symptomatic stones, one needs presence of symptoms and documentation of Diagnosis The diagnosis of symptomatic cholelithiasis, the clinical manifer- tation of chronic cholecystitis, relies on a history consistent with biliary trace disease, Transabdominal ulteatonography teliably documents the presence of cholelithiasis. Ultrasound can provide other important information, suck as common bile duct dilation, gallbladder polyps, porcelain gallbladder, or evidence of hepatic parenchymal procestes, Cholesteroloss, of the accumulation of cholesterol found in gallbladder mucosal macrophages, can also be seen (1 ).Bven in the absence of frank stones, so-called sludge found in the gallbladder on ulerasonography, with appro- priate symptoms, is consistent with biliary coli. Treatment Patients with sufficient symptoms from gallstones should undergo elective cholecystectomy. Cholecystectomy carries a low-risk profile bue is not without complications, so an analysis of risks and benefits is important. Because patients wich mild symptoms havea low rate of complications from gallstones (1% to 39%yea), observation and dietary and lifestyle changes are appropriate in this population, Patients with moze severe of recurtent symptoms have a higher rate of complications of the disease (79blyent), so elective laparoscopic cholecystectomy is warranted. In more than 90% of patients, cholecystectomy is curative, leaving them symptom free, Acute Calculous Cholecystitis ‘he pathophysiologic mechanism of acute cholecystitis is blockage of the eystic duet. When the blockage occurs from an obstructing CHAPTER 54_ Biliary System CT scan of emphysematous choleeysttis. Significant Berenelecysticinlammatary changes and air in the galladcer wall farms) are signs of emphysematous cholecysts scone, the diagnosis is acute calculous cholecystitis, The differentia tion of biliary colic from acute cholecystitis is untesolved blockage of the cystic duct. In biliary colic, che obstruction is temporary and self-limited. In acute cholecystitis, the obstruction does not resolve, and inflammation ensues, with edema and subserosal hhemorthage, Infection of che scagnane pool of bile isa secondary phenomenon: the primary pathophysiologic mechanism is unre~ solved cystic duct obstruction. Without resolution of the obstruc- tion, the gallbladder will progress to ischemia and necrosis Eventually, acute cholecystitis becomes acute gangrenous chole cystitis and, when complicated by infection with a gas-forming organism, acute emphysematous cholecystitis ( ) Presentation The inflammatory changes in the gallbladder wall are manifested as fever, right upper quadrant pain, sendernese to palpation, and guarding in the right upper quadrant. This process will use an crest of inspiration with gentle pressure under ¢ margin, a finding known as Murphy sign, Tens presence of Murphy sign help distinguish acute cholecystitis from biliary colic, in which there is no inflammatory process. Given that che common bile duct is not obstructed, profound jaundice in che seting of a picture of acute cholecystitis is rare and should raise the suspicion of cholangitis, with obstruction of the common, bile duct, or Mirizi syndrome, in which inflammation or a stone in the gallbladder neck leads to inflammation of the adjoining biliary system, with obstruction of the common hepatic duct Mild clevations of alkaline phosphatase, bilirubin, and transam- nase levels and a leukocytosis support the diagnosis of acute cholecystitis Diagnosis Transahdominal ultrasonography is a sensitive, inexpensive, and reliable tool for the diagnosis of acute cholecystitis, with a zensitiv- ity of 85% and specificity of 959, In addition to identifying gallstones, uluasound can demonstrate pericholecystic fluid ( ), gallbladder wall thickening, and even a sonographic Murphy sign, documenting tenderness specifically over the gall- bladder. In most eases, an accurate history and physicalSECTION X_ Abdomen conte FIGURE 54-24 Ultrasound image of ened galblcier wall witn richolacys cholecystitis icholecytic thd. The thick td farow incicates acute idies and an examination, along with supporting laboratory s0 ultrasound examination, make the diagnosis of acute cholecystitis, In atypical cases, an HIDA scan may be used to demonstra obstruction of the eystic duct, which definitively diagnoses acuse cholecystitis. Filling of the gallbladder during an HIDA scan cextentially eliminates che diagnosis of cholecystitis. CT may show similar Gndings to ultrasound with pericholecystic id, gallblad dee wall thickening, and emphysematous changes, but CT is less sensitive than ultrasound for the diagnosis of acute cholecystitis. Treatment Although the primary pathophysiologic event in acute choleeys tii is the obstruction af the cystic duct and infection is a second ary event that follows stasis and inflammation, most eases of acute cholecystitis ae complicated by superinfection of che inflamed gallbladder. Therefore, patients are given nothing by mouth, and incavenous (IV) fluids and parenteral anuibiotics are started fen that gram-negative aerabes are the most common argan- fime found in acute cholecystitis, followed by anaerober and grim postive acrobes, broad-spectrum anubiotis are warranted, Parenteral narcotics ate usualy equited to contol the pain ‘Cholecystectomy, whether open of laparoscopic, i the ereat- ment of choee for acute cholecystitis. "The timing of operative incervention in acute cholecystitis bar long been a source of debate. ln the past, many surgeons advocated for delayed chole cystectomy, with patients managed nonoperatively during their incal hospitalization and discharged home with resolution of symptoms, An interval cholecystectomy was then performed at approximately 6 wecks ali the inital episode. More recent studies have shown tha ealy in the disease process (wiehin the firse week), the operation can be performed lparoscopically with equivalent or improved morbidity. mortality and length of say swell asa similar conversion rte to open cholecystectomy.’ In audition, approximately 209% of patients initially admitted for nonoperative management fled to respond to medial weatment before the planned interval cholecystectomy and required surgical intervention. Initial nonoperative therapy romain a viable option for patients who present in a delayed fashion and should be decided on an individual basis Given the inflammatory process occutsing inthe porta hepati, carly conversion to open cholecystectomy should he considered when delineation af anatomy isnot clear or when progres cannot bbe made laparoscopically. With substantial inflammation, a partial cholecystectomy, tansecting the gallbladder a che infundibulum with eauterizstion ofthe remaining mucosa, i acceptable to avoid injury tothe common ble duct. Some patient present with acute cholecystitis but havea prohibitively high operative risk. For these patients, a pereutancodsly placed cholecystostomy tube should be considered. Frequendy performed with uleasound guidance ‘under local anesthesia with some sedation, cholecystostomy can act asa temporizing measure by draining the infected bile. Percu- taneous drainage results in improvement in symptoms snd physi logy, allowing a delayed cholecystectomy 3 10 6 months after medical optimization. In patents with cholecystostomy tubes, when Baoroscopy shows a patent eytic duct, the cholecystostomy tube can be removed and the decision for cholecystectomy deter mined by the patients ability wo tolerate suegcal intervention, Choledocholithiasis Choledocholithiass, oF common bile duct stones, is classified b the point of origin. Primaty common duct stones arise de novo in the bile duct, and secondary common duct stones pass feom the gallbladder ino the bile duct. Primary choledocholichiass is generally from brown pigment stones, which are a combination of precipitated bile pigments and cholesterol. Brown stones ate mote common in Asian populations and ate asociated with a bacterial infection of the bile duct. The bacteria secrete an enzyme thac hydrolyzes bilirubin glucuronides ro form free bilirubin, which then precipitates. Most common duct stones found in the United States ae secondary, having originated in the gallbladder, and ae vermed retained common duct stones when they are found within 2 years after cholecystectomy. Many common duct stones are clinically silent and may be identified only during cholangiography if is performed routinely during cholecystectomy (Fig. 54-25). Without pain or an abnor- mal liver function panel, a setting in which scleeive cholangiog- raphy isnot performed, 19 to 2% of patients alter cholecystectom will present witha retained stone. When i is performed routine intraoperative cholangiography identifies choledocholithasis in approximately 10% of asympromatic patients, suggesting chat ‘ost choledocholithiais remains clinically silent.*” ‘When not clinically silent, common duct stones may be mani- fesced with symptoms ranging from biliary colic co the clinical manifestations of obstructive jaundice, such as datkening of the urine, scleral icterus, and lightening of the stools. Jaundice with choledocholithiass is more likely o be painful because the onset of obstruction is acute, causing rapid distention of the bile duct and activation of pain fibers. Fever, a common symptom, can be associated with right upper quadrant pain and jaundice, a constel lation known as Charcot trad. This eriad suggests ascending chol- angitis that, if unteated, may progress (© septic shock. The addition of hypotension and mental status changes, both evidence of shock from a biliary source, to Charcot ttiad is known as Reynolds pentad. Diagnosis In the setting of choledocholichiasis, abnormalities of the hepatic fanction panel are common but neither sensitive nor specific, and with superinfection, leukocytosis may alzo he present. UltrasoundFIGURE 54-25 Intraoperative cholangiogram showing choledochal thiasis nan asymptomatic paver, wit no filing of duodenum and autine of stone Yarrow may show choledocholithiasis or only biliary ductal dilation. In patients with biliary pain, gallxones, and jaundice, a dilaced bile dduct (8 men) is highly suggestive of choledocholichasis, even if, common duct stones ate nox documented ultrasonographically Even without