Fistulae Involving the Gallbladder

In Western countries, where cholesterol cholelithiasis abounds, the gallbladder is most often the site of severe inflammation and obstruction. Cholecystenteric fistulae constitute 70% to 85% of all biliary fistulae reported in the world literature up to 1982 (Rau et al, 1980; Safaie-Shirazi et al, 1973). Of these, 55% to 75% are cholecystoduodenal, 15% to 30% are cholecystocolic, and 2% to 5% are cholecystogastric (see Fig. 42B.1). Multiple fistulae (e.g., cholecystoduodenocolic) are very rare (Shocket et al, 1970). Of the 23 cases reported up to 1978, 21 were secondary to gallstone disease, and one case of each was due to duodenal ulcer and a primary carcinoma of the gallbladder (Morris et al, 1978).

Gallstone ileus, a dramatic clinical presentation of a cholecystenteric fistula, is reported in 8% to 20% of large series of patients with biliary-enteric fistulae (Clavien et al, 1990; Heuman et al, 1980; Kasahara et al, 1980; LeBlanc et al, 1983; Rau et al, 1980; Safaie-Shirazi et al, 1973; VanLandingham & Broders, 1982). As a cause of intestinal obstruction alone, gallstone ileus accounts for 1% to 4% of cases, although in patients older than 65 years, gallstone ileus may account for 25% of cases of small bowel obstruction, regardless of a history of prior abdominal surgery (Clavien et al, 1990; Reisner & Cohen, 1994; Rodriguez-Hermosa et al, 2001; Lassandro et al, 2004; Kirchmayr et al, 2005; Chou et al, 2007).

Although most fistulae between the gallbladder and intestinal tract become obvious preoperatively or intraoperatively, cholecystocholedochal fistulae are insidious and may not be appreciated even at surgery. These biliobiliary fistulae develop between the ampulla of the gallbladder or cystic duct and the proximal common hepatic or common bile duct (CBD; see Fig. 42B.1D). In either instance, the mechanism of formation is the same: pressure necrosis into the common duct by a large solitary calculus impacted in either the ampulla of the gallbladder or the intramural portion of the cystic duct. Cholecystocholedochal fistula has been estimated to occur in 1% to 6% of biliary operations for calculous disease (Corlette & Bismuth, 1975). Although this estimate seems high and is not corroborated by other reports, an awareness of the possibility of cholecystocholedochal fistula is important and may help avoid damage to the common duct at operation.

Fistulae Involving the Common Bile Duct, Cystic Duct Remnant, and Other Extrahepatic Ducts

Choledochoduodenal fistulae are classified as either proximal or distal (see Fig. 42B.1E and 42B.1F). A proximal choledochoduodenal fistula is the principal form of abnormal communication between the CBD and adjacent structures and represents 4% to 20% of all biliary-enteric fistulae. At one time, 80% were caused by peptic ulcer erosion from the first portion of the duodenum into the proximal CBD. This cause is now much less common as a result of the development of effective medical therapy with antacid drugs. Other, less common causes of choledochoduodenal fistula include cholelithiasis, operative trauma, duodenal diverticula, echinococcal infection, Crohn disease, and neoplasms of the stomach, distal bile duct, ampullary region, and duodenum (Feller et al, 1980; Sarr et al, 1981; Kuroki et al, 2005). Since the beginning of the acquired immunodeficiency syndrome (AIDS) epidemic, there have only been three known case reports of choledochoduodenal fistula caused by AIDS/human immunodeficiency virus–associated tuberculous infection (Patino et al, 2003).

Distal choledochoduodenal fistulae connect to the duodenum in the distal 2 cm of the CBD, and the fistula opening can be seen during percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP). The incidence of distal choledochoduodenal fistula secondary to cholelithiasis or operative trauma is variable in different parts of the world. With the development of PTC and ERCP studies of pathologic anatomy (see Chapters 18, 27, and 28), it is becoming apparent that many patients with minor or major biliary-digestive complaints and gallstone disease may have a distal choledochoduodenal or parapapillary choledochoduodenal fistula. Large series from Argentina showed the incidence of parapapillary choledochoduodenal fistula to be 0.7%. Jorge and colleagues (1991) reviewed 2012 ERCP studies from 1976 through 1989, and only 14 cases were found: two cases were from stone disease and the other 12 were from iatrogenic causes.

In Japan, where there is a high incidence of primary intrahepatic calculous biliary tract disease, Tanaka and Ikeda (1983) reported a 5.3% incidence of parapapillary fistula in ERCP studies of 1500 patients. The male/female ratio was roughly equal, in keeping with the Japanese sex distribution of gallstone disease. Ikeda and Okada (1975) classified these as type I fistula, characterized by a small fistula opening on the longitudinal fold of the duodenum just proximal to the papilla, probably caused by penetration of a small calculus through the intramural portion of the common duct into the duodenum, and type II fistula, a larger opening of the duodenum wall adjacent to the longitudinal fold, probably caused by a relatively large stone eroding from the extramural portion of a greatly dilated common duct into the duodenum.

In Turkey, Karincaoglu and colleagues (2003a) reported an incidence rate of 4.9% on review of 1347 ERCP studies in 841 patients. This study also found an intriguing association between peripapillary fistula with common duct stones and complications of cholangitis. The incidence of parapapillary choledochoduodenal fistula is probably less in Western Europe, the United Kingdom, and the United States, and perhaps a greater proportion of these fistulae are not due to spontaneous gallstone disease itself but rather to iatrogenic surgical injury or other instrumental damage to the distal common duct in operations directed against choledocholithiasis. Hunt and Blumgart (1980) and Tytgat and colleagues (1979) published their experience of these fistulous complications of biliary tract surgery.

Unusual choledochal fistulae are mentioned only briefly in this context. Spontaneous fistula formation between the common duct and the colon has been recorded only four times in the English literature (Bose & Sastry, 1983; Rawas et al, 1987; Guitron-Cantu et al, 2001; Anees et al, 2008). We are also aware of one case of a choledochocolonic fistula that developed after blunt abdominal trauma (Benson et al, 2001) and another that developed as a complication from diverticulitis (Blanco-Benavides & Rodriguez-Jerkov, 1992). Peptic ulcer disease has rarely produced fistulae between the common duct, duodenum, and pancreas (Aitken et al, 1986). We have observed one case and are aware of several other cases of severe pancreatitis or pancreatic cancer producing a fistulous communication between pancreatic pseudocysts or necrotic pancreatic cavities and the CBD (DeVanna et al, 1983; Ellenbogen et al, 1981; Lebovics et al, 1990; Miller et al, 1988). Chandar and Hookman (1980) reported one case of GI hemorrhage resulting from a choledochocolonic fistula that developed from a cystic duct remnant 28 years after cholecystectomy, and it was associated with a benign stricture in the ampullary segment of the CBD. More recently, a bile duct stricture associated with a choledochocolonic fistula was reported following an uncomplicated open cholecystectomy four months prior (Munene et al, 2006). Woods and colleagues (1992) reviewed four cases of cystic duct remnant fistulization to the GI tract.

Fistulae Involving the Intrahepatic Ducts, Liver, and Lung

Thoracobiliary and bronchobiliary fistulae are quite rare (Boyd, 1977; Chan et al, 1984; Cleve & Correa, 1958); the former refers to communications between the biliary tree and the pleural cavity and the latter to communications between the bile ducts and the bronchial tree. The three major categories of bronchobiliary fistula are 1) those resulting from infection, especially parasitic, or those acquired, such as from iatrogenic injury as a result of biliary tract surgery or, less commonly, from gastric and pancreatic operations; 2) traumatic fistula; and 3) congenital fistula. The many diverse causes of acquired bronchobiliary fistulae have been well referenced by Sane and colleagues (1971) and more recently by Gugenheim and colleagues (1988). The two main factors responsible for the development of bronchobiliary fistulas are “mechanical” biliary obstruction and infection. Worldwide, the principal cause of bronchobiliary fistula in adults is parasitic disease of the liver, either echinococcal or amebic abscess (see Chapters 67 and 68). In developed countries, iatrogenic injury to the biliary ductal system is the most frequent cause of these fistulae.

The hallmark symptom of a bronchobiliary fistula is biliptysis in conjunction with other pulmonary complaints, jaundice, cholangitis, and external biliary fistula or subphrenic abscess. Radiologic confirmation of the diagnosis is possible by a variety of methods. In the presence of an external fistula, injection of contrast solution (fistulogram) is the most facile and direct approach. PTC and ERCP are equally effective in their ability to show the fistula and have the advantage of offering an avenue for potential therapeutic intervention. The choice of one or the other of these techniques depends on the presence or absence of dilated ducts and the expertise available. Bronchobiliary fistulae also have been shown by cholescintigraphy (Gunlemez et al, 2009; Santra et al, 2009; Uramoto et al, 2008; Andalkar et al, 2004; Savitch et al, 1983; Velchik et al, 1991). Computed tomography (CT) and magnetic resonance imaging cholangiopancreatography (MRCP) have been used to assess bronchobiliary fistulae (see Chapters 16 and 17). These imaging techniques, although helpful in assessing the upper abdomen, rarely visualize the fistula tract (Oettl et al, 1999; Yeatman et al, 2004; Kuroki et al, 2005; Ragozzino et al, 2005).

The surgical treatment of parasitic disease of the liver is discussed elsewhere in this book (see Chapters 67 and 68). In large series of surgically treated cases of hepatic echinococcal disease in Greece and Turkey, only 2% were complicated by rupture into the lung or bronchi (Alestig et al, 1972). Amebic abscess of the liver has been reported in association with bronchobiliary fistula in 8% of cases (Razemon et al, 1963). These complications of parasitic disease are more likely to be suspected in patients from Mediterranean countries, North Africa, Mexico, and some of the southern border states of the United States. The successful treatment of these fistulae depends on the use of appropriate surgical drainage or resection in conjunction with appropriate drug therapy.

The incidence of bronchobiliary fistula as a consequence of surgically treated calculous or neoplastic disease of the hepatobiliary tract and adjacent structures has been decreasing, as patients are operated on earlier in the course of their disease and by better-trained surgeons (Ramesh et al, 1991; Warren et al, 1983). Then again, modern surgical techniques and equipment have contributed to a new cause of biliary fistulae: reports of bronchobiliary fistula caused by new liver tumor ablative therapies, such as radiofrequency ablation (RFA), are beginning to emerge (Kim et al, 2005; Tran et al, 2007; Yoon et al, 2009; see Chapter 85A, Chapter 85B, Chapter 85C, Chapter 85D ). In all these case reports, the fistula responded to conservative treatments focused on biliary drainage.

The incidence of bile duct injury in the course of cholecystectomy has increased with the advent of laparoscopic removal of the calculous gallbladder (Davidoff et al, 1992). Most of the injuries are repaired at or shortly after their occurrence. Bronchobiliary fistulae secondary to bile duct injury are a late consequence of the event in patients with neglected or recurrent strictures after unsuccessful operations. Less frequently, gastric or pancreatic procedures may result in injury to the extrahepatic bile ducts.

Untreated choledocholithiasis complicated by repeated episodes of bile duct obstruction and cholangitis is another, albeit infrequent, cause of bronchobiliary fistula. Brem and colleagues (1990) reported the successful treatment of an 87-year-old patient with this clinical situation by endoscopic papillotomy alone. Indeed, bronchobiliary fistulae should be treated first by biliary decompression, either by endoscopic papillotomy or by transampullary drainage to reduce the fistula tract pressure. Some patients with a persistent fistula or infection will require debridement by thoracotomy.

Posttraumatic thoracobiliary fistulae are extremely rare. Although penetrating or blunt trauma to the abdomen and chest is a common enough civilian and wartime occurrence, the number of fistulae resulting has remained small, often because their initial surgical treatment has been excellent, and because no obstruction to proper bile flow existed (Oparah & Mandal, 1978). Ivatury and colleagues (1984) reported on three cases and reviewed 32 previously reported patients. This series included 20 thoracobiliary and 15 bronchobiliary fistulae. Twenty-six patients sustained penetrating injuries, including 16 with gunshot wounds, 6 with stab wounds, and 4 in which the weapon was not specified. Seven patients were admitted because of blunt trauma, and in an additional four patients, the mechanism of injury was not documented. All of the patients in this report had thoracoabdominal injuries, and the causes of the fistulae were nonoperative therapy, missed or overlooked injury to the diaphragm, inadequate drainage, and subphrenic abscess. All patients had pleural effusion, and bile was aspirated by thoracentesis. Successful treatment frequently required pulmonary decortication, repair of the diaphragm, and adequate drainage above and below the diaphragm. More recent publications support the notion that inadequate drainage caused posttraumatic bronchobiliary fistulae (Navsaria et al, 2002; Eryigit et al, 2007; Ball et al, 2009).

Neuhauser and colleagues (1952) first described congenital bronchobiliary fistula in an infant with a tract communicating between the right main stem bronchus and the hepatic duct that passed through the posterior mediastinum. The diagnosis was made antemortem, but the infant died before surgical therapy. Tommasoni and colleagues (2000) described two newborn infants who were promptly diagnosed and successfully treated surgically. These authors emphasized the importance of a high index of suspicion for early diagnosis and recognition of the very high incidence (approximately 36%) of coexisting hypoplasia or biliary atresia. Therefore, all of these patients need laparotomy and thoracotomy for successful treatment (Tommasoni et al, 2000). The fistulous tract in the posterior mediastinum adjacent to the esophagus should also be excised to avoid repeat thoractomy (Gunlemez et al, 2009). Yamaguchi and colleagues (1990) reported one adult patient with a congenital bronchobiliary fistula and summarized the data of 16 previously reported cases, four of them adults. Interestingly, all adults were younger than 32 years (range, 22 to 32 years). The only congentital bronchobiliary fistula in an elderly person was reported recently by Uramoto (2008).

On microscopic examination, the proximal portion of the fistula in most patients resembles the bronchus, and the distal segment resembles the esophagus; the embryologic explanations for this anomaly are conjectural (Dyon et al, 1978; Sane et al, 1971). Transthoracic excision of the fistula and surgical correction of associated biliary anomolies is usually curative.

Plain and Contrast Radiographs

Pneumobilia, the presence of air in the biliary tree, may be noted on plain films of 30% to 50% of patients with gallstone ileus (Fig. 42B.2A). Other, less common causes of pneumobilia include an incompetent sphincter of Oddi, emphysematous cholecystitis, suppurative cholangitis, and prior biliary-enteric bypass surgery. Hricak and Vander Molen (1978) suggested that the low incidence of pneumobilia in gallstone ileus is due to the cystic duct obstruction that led to the acute cholecystitis and perforation into an adjacent viscus; the obstruction also prevents retrograde passage of air into the extrahepatic and intrahepatic bile ducts.

FIGURE 42B.2 A, Plain radiograph shows pneumobilia (arrowhead). B, Plain radiograph shows multiple dilated loops of small bowel (black arrowheads), several calcified gallstones (white arrowhead), three gallstones located in small bowel in the upper abdomen, and one obstructing gallstone in the pelvis.

(A, Courtesy Dr. Rizwan Aslam. B, Courtesy Dr. Fergus V. Coakley.)

Other classic radiographic signs of gallstone ileus are visualization of a calcified gallstone in the peritoneal cavity a distance away from the gallbladder (Fig. 42B.2B), change on repeat films in the position of a previously observed calcification, and a change in the level of mechanical intestinal obstruction—the so-called tumbling obstruction (Day & Marks, 1975; VanLandingham & Broders, 1982). However, only 30% of gallstones are sufficiently calcified to be radiopaque.

A barium meal or upper GI series shows reflux of contrast material into the fistula in 40% of cholecystoduodenal communications and 75% of choledochoduodenal fistulae of peptic ulcer origin (Balthazar & Gurkin, 1976; Kourias & Chouliaras, 1964). If the plain film and barium swallow are used in concert, more than 60% of biliary enteric fistulae are correctly diagnosed preoperatively (see Fig. 42B.2A; Balthazar & Schechter, 1975). A negative upper GI series in the presence of pneumobilia is an indication for a barium enema, which discloses more than 95% of cholecystocolic fistulae. CT scans with contrast material, endoscopy, radionuclide imaging, and sonography are also useful in the diagnosis of biliary fistulae (Becker et al, 1984); and more recently, MRCP has yielded valuable information.

Endoscopy and Iodinated Dye Studies

Direct injection of contrast material is the best way to outline the normal and pathologic anatomy of the biliary tract. Preoperatively, this imaging is accomplished by ERCP or PTC; when an external bile fistula exists, a fistulogram should be performed. Intraoperative or postoperative cholangiograms are often invaluable.

Since 1970, numerous reports have appeared in the literature supporting the efficacy of ERCP in documenting surgical or spontaneous biliary fistulae (Al Nakib et al, 1982; Tanaka & Ikeda, 1983; Tytgat et al, 1979; Van Linda & Rosson, 1984). Endoscopically, the alimentary side of a fistula can be visualized directly, be it gastric (Stempfle & Diamantopoulos, 1976), duodenal, or colic; the fistula itself or the ampulla of Vater can be cannulated to obtain a high-quality radiograph of the communicating biliary anatomy (Moreira et al, 1984; Watkins et al, 1975; Chatzoulis et al, 2007). Similarly, the largely unappreciated and frequently asymptomatic parapapillary choledochoduodenal fistula has been found to be quite common (Hunt & Blumgart, 1980; Karincaoglu et al, 2003b; Tanaka & Ikeda, 1983; Kuroki et al, 2005). There have been far fewer reports of the role of PTC in showing biliary fistulae, but PTC has been helpful in the diagnosis and treatment of bronchobiliary fistula in association with echinococcal disease of the liver and dilated intrahepatic ducts (e.g., cholecystocholedochal fistula; Cornud et al, 1981; Mannella et al, 1999).

Radionuclide Imaging (See Chapter 15)

With the introduction of the imidoacetic acid agents bound to technetium (p-isopropylacetanilido iminodiacetic acid [PIPIDA] and hepatobiliary iminodiacetic acid [HIDA]), radionuclide scans have rapidly become the preferred method of outlining the normal and pathologic anatomy of the extrahepatic bile passages. These relatively noninvasive procedures do not rely on hepatocellular concentration of bile or on short critical time-flow periods for satisfactory visualization of the biliary tree. Prolonged (24 hours) accumulation of radioactivity, measured by patient scanning or quantitative isotopic counts of body secretions (e.g., sputum), has been used to show very small or intermittent fistulae from the biliary tract to the respiratory passages (Fig. 42B.3) and the colon (Benson et al, 2001; Santra et al, 2009; Uramoto et al, 2008; Andalkar et al, 2004; Bretland, 1983; Edell et al, 1981; Henderson et al, 1981; Savitch et al, 1983; Taillefer et al, 1983).

FIGURE 42B.3 A 49-year-old woman with symptoms of pneumonia and clinical evidence of biliptysis. Anterior image from hepatobiliary iminodiacetic acid scan at 40 minutes after injection shows communication of the liver with the right lung and central airways. Radiopharmaceutical is present in the right and left main bronchi (small curved arrows) and in the trachea (large curved arrow), confirming the presence of bronchobiliary fistula.

(Courtesy Dr. Douglas S. Katz.)

Sonography

Sonography is a useful noninvasive diagnostic aid in the preoperative evaluation of a patient with a suspected biliary fistula (Porta et al, 1981; Renner et al, 1982) and even gallstone ileus (Davies et al, 1991; Pedersen et al, 1988; see Chapter 13). Although the radionuclide scan may readily show a fistula, the sonogram indicates the presence of calculi in the gallbladder; common duct stones; and inflammatory, cystic, or infiltrative disease of the liver and pancreas (Griffin et al, 1983). Sonography can readily detect pneumobilia, indicating a high likelihood of a biliary-enteric fistula. In gallstone ileus, sonography can detect an ileal stone not seen on plain films and is increasingly being used by emergency room physicians (Zironi et al, 2007). Ripolles and colleagues (2001) reported that 22 of 23 patients who had undergone surgery for gallstone ileus were found on sonogram to have pneumobilia. This information is valuable in the decision-making process, such as to evaluate the need for or advisability of cholecystectomy and dismantling of a fistula at the time of operation for gallstone ileus in an elderly or high-risk patient. This information may be obtained more rapidly and safely, and probably with equivalent accuracy, by a preoperative sonogram rather than by intraoperative manipulations about the mass of inflamed tissue in the right upper quadrant. Ultrasound (US) has been used to document the persistence or closure of biliary enteric fistulae after initial emergent surgical or combined endoscopic lithotripter treatment of gallstone ileus, and it has aided the decision for or against further surgery (Clavien et al, 1990).

Computed Tomography and Magnetic Resonance Imaging Cholangiopancreatography (See Chapters 16 and 17)

CT and MRCP are better able to show a moderately calcified ectopic gallstone, air in the biliary tree, the presence of additional stones, and a cholecystoduodenal fistula (Swift & Spencer, 1998). CT is useful also for estimating the size of the impacted stone and determining the site of obstruction for planning the operative approach (Fig. 42B.4). Indeed, new multidector CT scanners that use multiplanar or three-dimensional volume-rendering reconstruction will further improve the diagnostic role of CT in detecting fistulae. Already, modern MRI and MRCP scans with improved image resolution and reduced motion artifact have been shown to accuarately detect a small fistulous tract (Chatzoulis et al, 2007). Although such scanning may disclose fusion of the biliary tract to an adjacent viscus, it may not always precisely show a fistula; but MR scanning should be used to show other important pathologic changes, such as distal common duct stones or other obstructive processes, the presence of a subphrenic abscess, pleural effusion, or parasitic disease of the liver (Porta et al, 1981). Scanning also can be used to verify and localize contrast extravasation after interventional procedures, such as PTC or ERCP, or after contrast injection of an existing controlled external fistula (Fig. 42B.5).

FIGURE 42B.4 A 92-year-old woman with recurrent pyogenic cholangitis and increasing abdominal pain, anorexia, constipation, nausea, and vomiting over a 36-hour period. A, Computed tomography through the liver showing extensive pneumobilia (arrowheads). B, In the pelvis, a soft tissue–attenuated, round, 2.5-cm gallstone (arrow) is seen at the transition point between dilated and decompressed bowel.

(Courtesy Dr. Fergus V. Coakley.)

FIGURE 42B.5 A and B, Cholecystograms showing extravasation: percutaneous cholecystostomy tube. C, Axial computed tomographic scan shows contrast extravasation into right upper abdomen (arrowheads).

Gallstone Ileus

Gallstone ileus is the blockage of the intestinal tract by a gallstone large enough to occlude its lumen partially or completely. This dramatic presentation of intestinal obstruction is too indelibly fixed in the medical imagination to permit amendment of the inappropriate term ileus; perhaps this derives from the frequent initial clinical impression of an unexplained ileus, in that many patients with gallstone obstruction are initially seen without any clinical history or physical signs to suggest mechanical intestinal obstruction. Similar to other patients in Western Europe and English-speaking countries, where cholesterol cholelithiasis predominates, patients seen with this manifestation of advanced biliary tract disease are usually elderly, female, and beset by multiple other medical conditions that may delay or complicate prompt diagnosis and appropriate treatment.

When an elderly person presents with typical signs and symptoms of intestinal obstruction or, perhaps less dramatically, an unexplained ileus without obvious incarcerated abdominal wall hernia, scar of a previous laparotomy, and little suspicion of an occult malignancy, calculous obstruction by a gallstone must be considered. Approximately half of such patients presenting with gallstone ileus give a history suggesting prior calculous biliary tract disease. At the time of clinical presentation with gallstone obstruction, however, signs of active gallstone disease—pain, tenderness in the upper abdomen, cholangitis, and jaundice—may be absent. Laboratory data are consistent with fluid and electrolyte disturbances appropriate to the degree and duration of intestinal obstruction, and some abnormalities of liver function tests (LFTs) may suggest chronic disease of the liver and biliary tract in one quarter of patients.

The classic plain abdominal film triad of small bowel obstruction, pneumobilia, and ectopic gallstone is considered pathognomonic of gallstone ileus (Rigler et al, 1941); however, the triad is encountered in only 30% to 35% of cases (Balthazar & Schechter, 1978). Pneumobilia is often not appreciated even in retrospect. If biliary-enteric fistula is suspected, and a barium meal is administered, reflux of barium into the biliary tree yields a correct preoperative diagnosis in 60% of patients (Fig. 42B.6). Calculi large enough to obstruct the intestine usually do so in the last 50 cm of ileum, although sometimes also in the jejunum or duodenum and rarely in the sigmoid colon. Such calculi are usually larger than 2.5 cm; if partially calcified, they are readily apparent on a plain radiographs (see Fig. 42B.2B).

FIGURE 42B.6 Upper gastrointestinal series shows a choledochoduodenal fistula with reflux of contrast medium into the biliary tree.

(Courtesy Dr. Benjamin M. Yeh.)

Abdominal ultrasound, as mentioned earlier, often establishes the diagnosis of gallstone ileus or provides other information relevant to the diagnosis (Clavien et al, 1990; Davies et al, 1991; Pedersen et al, 1988; Ripolles et al, 2001; Zironi et al, 2007), revealing pneumobilia; the site of the fistula; additional stones still in the gallbladder, or more importantly, in the common duct; and occasionally the location of an ectopic calculus. The diagnositic superiority of CT over abdominal plain films and sonography for gallstone ileus is now well established (Ayantunde & Agarwal, 2007; Lassandro et al, 2005; Loren et al, 1994; Reimann et al, 2004; Swift & Spencer, 1998). In fact, the clinical significance of Rigler’s triad has been revitalized by CT, because all three findings are more consistantly detected (approximately 78%) by this method (Kirchmayr et al, 2005; Ayantunde & Agarwal, 2007; Chou et al, 2007).

The clinical presentation of gallstone ileus has not changed in the past 40 years. In patients with intestinal obstruction, preoperative diagnostic accuracy is approximately 75% (Glenn et al, 1981). Preoperative diagnostic accuracy has contributed to an improved outcome of therapy (Deitz et al, 1986). The increased use of CT in patients with symptoms of intestinal obstruction has led to additional improvement in the diagnostic accuracy of gallstone ileus and has resulted in a decrease in the high mortality rate encountered with this disease (Delabrousse et al, 2000; Lassandro et al, 2004; Reimann et al, 2004; Ayantunde & Agarwal, 2007; Chou et al, 2007).

The overriding consideration in patients presenting with gallstone obstruction of the intestine should be relief of the life-threatening cause of obstruction (i.e., enterolithotomy). This surgical emergency should be approached expeditiously and without a period of waiting in the hope that a suspected stone will pass; it will not. The only reason for delay should be to provide adequate preoperative fluid and electrolyte resuscitation of these critically ill patients and, if possible, to assess the presence of other gallstones in the gallbladder and common duct by means of sonography or CT. The use of nasogastric decompression and preoperative antibiotics is strongly recommended to minimize the risks of aspiration and postoperative wound infection.

The terminal ileum is the site of obstruction in 70% of cases (see Figs. 42B.2B and 42B.4B). Unless the obstructed segment is ischemic or has perforated and requires a small bowel resection, the obstructing calculus can be manipulated proximally to a healthy section of bowel, where a safe enterotomy and stone removal may be executed. Jejunal impaction, often by stones larger than 4 cm, occurs approximately 15% of the time, and enterotomy may be made at the site or just proximal to it. Duodenal obstruction, usually in the bulb, is known as Bouveret syndrome (Argyropoulos et al, 1979; Bhama et al, 2002; Cooper & Kucharski, 1978; Koulaouzidis & Moschos 2007; Frattaroli et al, 1997; Maglinte et al, 1987; Thomas et al, 1976), which occurs in 10% of patients and may be handled by duodenostomy or pyloroplasty. It occasionally may be possible to manipulate the stone back into the stomach and remove it via gastrotomy. Rarely, a gastroenterostomy is necessary to protect a duodenotomy or severely traumatized duodenum at the site of impaction.

Bouveret syndrome and other cases of gallstone impaction high in the jejunum have been managed successfully by a combination of endoscopy and electrohydraulic lithotripsy (Fujita et al, 1992; Holl et al, 1989; Moriai et al, 1991; Sackmann et al, 1991). We are aware of one case report of a failed endoscopic extraction that led to spontaneous uneventful passage of the stone (Wagholikar & Ibrarullah, 2004). In rare instances, the sigmoid colon is the site of obstruction of a calculus that has managed to pass through the terminal ileum or enter the colon via a cholecystocolic fistula (Anseline, 1981; Clavien et al, 1990). Almost invariably, some other pathologic process, such as diverticulitis, has produced an area of colonic narrowing. A colostomy is necessary to decompress the proximal bowel. If the impacted stone cannot be manipulated proximally to a transverse colostomy, consideration should be given to exteriorization of the impacted segment with a Hartmann closure of the bowel distal to it (Milsom & MacKeigan, 1985).

Open exploration and enterolithotomy has always been the standard surgical approach for the treatment of classical gallstone ileus. It not only allows removal of the obstructing stone but also permits careful palpation of the entire bowel and gallbladder region, to determine whether other gallstones are in transit more proximally or if any still reside in the diseased gallbladder (see Fig. 42B.2B). These calculi may be poised for passage through the fistula, possibly to induce a recurrent episode of gallstone ileus, a phenomenon estimated to occur in 5% of cases; however, by simple maneuvers, this can be prevented (Clavien et al, 1990; Haq et al, 1981; Levin & Shapiro, 1980). More recently, some authors have described laparoscopic or laparoscopic-assisted treatment for patients with gallstone ileus (Allen et al, 2003; Malvaux et al, 2002; Moberg & Montgomery, 2007; Owera et al, 2008). Because of the limited number of laparoscopically treated patients, it is unclear wherther this approach will reduce the associated high mortality and morbidity rates. Therefore it is our opinion that an open surgical approach should remain the standard of practice and that laparoscopic treatment needs further evaluation.

Considerable debate in the surgical literature surrounds whether cholecystectomy or common duct exploration or both, with dismantling and closure of the cholecystenteric fistula, should accompany enterotomy and relief of the obstruction or await a second operation (Kirchmayr et al, 2005; Muthukumarasamy et al, 2008; Zuegel et al, 1997). Historical data of published reports from the years 1953 through 1993 (Reisner & Cohen, 1994) showed a lower mortality rate of 11.7% in the enterolithotomy-alone group compared with 16.7% for patients who underwent a one-stage operation. Several published reports indicated that operative mortality is lower in these critically ill, elderly patients when only the gallstone obstruction is relieved (Muthukumarasamy et al, 2008; Tan YM et al, 2004; Heuman et al, 1980; Kasahara et al, 1980; VanLandingham & Broders, 1982). This has lead to the general agreement that enterolithotomy alone should be done for fragile patients with significant comorbidities and that the single-stage procedure should be reserved for young, fit, and low-risk patients.

Although some centers have recently published impressively low perioperative mortality rates (0%) for surgical treatment of gallstone ileus, other centers still report rates in the traditional high range (23%; Muthukumarasamy et al, 2008; Tan et al, 2004; Ayantunde & Agarwal, 2007). In a case series from the United Kingdom (Anyantunde & Agarwal, 2007), five deaths occurred in 22 patients; four of the five deaths were patients treated with enterolithomy alone. Seven of 19 patients from YM Tan and colleagues’ (2004) series were clinically unstable and underwent enterolithotomy alone; on long-term follow-up, these patients did not have further complications.

Indeed, careful follow-up of patients treated with enterolithotomy alone indicates that one third to one half will have minimal or no symptoms after relief of the gallstone ileus, and no further treatment will be necessary (Clavien et al, 1990; van Hillo et al, 1987; Tan YM et al, 2004). If a patient has jaundice and intestinal obstruction at the outset, common duct exploration, or at least tube decompression of the common duct, may also be necessary as a lifesaving procedure. For patients who continue to be symptomatic after relief of gallstone ileus, careful preoperative evaluation and bowel preparation can precede a later operation, adding to its safety.

Because common duct stones are found in 40% of patients with biliary-enteric fistula, it is likely that patients who continue to be symptomatic would require endoscopic or open surgical removal of their common duct stones in addition to cholecystectomy and repair of the fistula. As mentioned, operative mortality rate slowly declined to 15% to 25% in large reported series until the early 1970s. Further reductions in operative mortality rates have been possible by limiting treatment to enterotomy and stone removal alone without further surgical therapy in high-risk patients (Ayantunde & Agarwal, 2007; Glenn et al, 1981; Muthukumarasamy et al, 2008; Rodriguez-Sanjuan et al, 1997; Tan YM et al, 2004).

Cholecystoduodenal Fistulae

Most cholecystoduodenal fistulae do not result in gallstone ileus (see Fig. 42B.1A). Rather they are asymptomatic or occur in association with the usual digestive complaints consistent with gastric or biliary tract disease. They may be found during an upper GI barium study or under less welcome circumstances, such as at the time of abdominal surgery for an unrelated problem.

If an asymptomatic or mildly symptomatic cholecystenteric fistula is diagnosed preoperatively, many of the management decisions regarding gallstone ileus discussed previously may apply. Elective surgery may never be necessary in a completely asymptomatic individual, and surgery may present an unfavorable risk-to-benefit ratio in an elderly, minimally symptomatic patient. Along with dismantling of the fistula and common duct exploration, other alternatives to cholecystectomy must be considered, such as a period of expectant management with careful observation, endoscopic papillotomy and stone extraction with the gallbladder left in situ, or interval cholecystectomy if symptoms of pain or cholangitis persist after endoscopic biliary surgery. In a relatively healthy patient younger than 70 years, we believe that open surgical extirpation of the gallbladder, closure of the fistula, and treatment of any common duct pathology promise the best long-term therapeutic result, although some authors advocate laparoscopic treatment of preoperatively diagnosed biliary-enteric fistulae (Chikamori et al, 2001; Crouch & Kuhnke, 2000; Kwon & Inui, 2007; Lee et al, 2004; Rohatgi & Singh, 2006).

If an incidental cholecystoduodenal fistula is discovered at the time of surgery, the major intraoperative decision revolves around the patient’s need for, and ability to tolerate, additional surgical manipulations. If the patient is judged an unsound risk, or if the biliary tract pathology is not believed to be pertinent to the major indication for operation, nothing need be done. Rarely, a cholecystostomy (Fig. 42B.7) and extraction of large calculi can be accomplished with little additional risk. Usually, the gallbladder is shrunken and barely palpable, however, because it is stuck to the duodenum. If the patient can tolerate additional surgery, and sufficient indication exists to proceed at the present operation, rather than at a subsequent one, an attempt should be made to show the biliary-enteric anatomy and state of the common duct by an operative cholangiogram before proceeding with a definitive procedure.

FIGURE 42B.7 Cholecystoduodenal fistula (arrow) shown by cholecystostomy tube cholangiogram 1 month after operation for acute cholecystitis.

Cholecystocolic Fistulae and Choleric Enteropathy

A cholecystocolonic fistula may develop acutely in patients with long-standing mild or moderately symptomatic biliary tract disease and may be heralded by a sudden change in bowel habits with multiple, loose stools and the development of fever, chills, and other signs of cholangitis from colonic bacterial reflux into the biliary tract. Many elderly patients either weather or ignore these symptoms without seeking medical attention, and some develop signs and symptoms that may incriminate the entire GI tract. Increased stool frequency persists, particularly after ingestion of food, and bouts of fever and malaise subside. Other characteristic symptoms then appear, such as eructation, nausea, weight loss, and increasing diarrhea and steatorrhea. These latter symptoms precede the onset of choleric enteropathy, a dramatic complication of cholecystocolonic fistula. This enteropathy also is seen in other major disturbances of bile acid metabolism, such as with major ileal resection or blind loop syndrome (Brandt, 1984).

Choleric enteropathy comprises of a wide spectrum of anatomic, physiologic, and biochemical changes produced by a significant alteration of the enterohepatic circulation of bile acids. The malabsorption syndrome secondary to cholecystocolonic fistula was clinically documented first by Augur and Gracie (1970) and has since been studied by others (Rau et al, 1980). Ordinarily, 95% of bile acids are passed down the jejunum, aiding in fat and cholesterol absorption, before being largely reabsorbed in the terminal ileum as part of an efficent enterohepatic circulation. Two or three cycles of the bile acid pool per meal occur, with further metabolism of bile acids in the colon and very little lost. With a cholecystocolonic fistula, however, a large part of all of the primary bile acid pool is shunted directly into the colon, resulting in a high luminal concentration of bile acids. In the colon, the primary bile acids undergo deconjugation and dehydroxylation by fecal bacteria, and this increased concentration of bile acids causes a water secretory diarrhea.

Interestingly, only the two natural bile acids with two α-hydroxy groups, chenodeoxycholic acid and deoxycholic acid, induce colonic secretion (Keely et al, 2007). Depending on the amount of bile still passing via the common duct into the small bowel, fat absorption is affected, which over time may result in fatty acid diarrhea. More immediately, however, colonic secretion of water and electrolytes is maximally stimulated by the dihydroxy bile acids. Chenodeoxycholic acid gains access to the colon in large quantities, and massively increased amounts of deoxycholic acid are formed by bacterial dehydroxylation of cholic acid. The primary bile acids and their metabolites are no longer actively absorbed and are lost from the body, eventually causing a chronically lowered bile salt pool beyond the liver’s capacity to synthesize new bile acids to replete it. At this point, even with a partial shunt to the colon, the bile acid concentration still normally passing down the common duct and through the small intestine may be too small to effect micellar solubilization of dietary fat. In contrast to a blind loop syndrome, in which bacterial overgrowth contributes to the choleric enteropathy and can be largely corrected by antibiotics, at least temporarily, cholecystocolonic fistula does not respond adequately to antibiotics. Until the fistula is dismantled, massive shunting of bile acids to the intestine persists and promotes continued watery diarrhea, diminished bile salt pool, and a variable degree of fat malabsorption.

Cholangitis seems to be a more prominent feature of cholecystocolonic fistula, when the fistula is narrow and prone to intermittent obstruction of bile flow (Edell et al, 1981; Safaie-Shirazi et al, 1973), whereas diarrhea with a lesser incidence of fever and chills is more common with a wide-open fistula (Lygidakis, 1981); we are also aware of one case report of a patient with a cholecystocolonic fistula who was seen with massive lower GI hemorrhage (Kunasani et al, 2003). In any instance, a fistula to the colon is more likely than other biliary-enteric fistulae to have associated cholangitis; if it does, the serum levels of bilirubin, alanine aminotransferase, and γ-glutamyltransferase may be slightly elevated and may direct attention to the biliary and upper digestive tract.

Because of the unusual initial complaints, full investigations for malabsorption that include upper GI studies, GI sonography, and jejunal biopsy may be undertaken but usually do not clarify the diagnosis. A pathognomonic triad consisting of pneumobilia, chronic diarrhea, and vitamin K malabsorption was recently suggested by Savvidou and colleagues (2009) to aid in the diagnosis of cholecystocolonic fistula. Diversion of bile acids into the colon results in malabsorption of fat and fat-soluble vitamins. In particular, vitamin K deficiency can be clinically significant and is easily detected by a prolonged prothrombin time (PT), and it can be corrected by parenteral supplementation (Savvidou et al, 2009). Although the condition of bile acid malabsorption can be easily diagnosed by the selenium-75-homocholic acid taurine (SeHCAT) test, this study is time consuming and is not widely available (Pattni & Walters, 2009). Plain abdominal radiographs have been reported to reveal air in the biliary tree in only 50% of cases, however, the diagnosis becomes evident only if a barium enema examination is done, preferably with air contrast, because barium (Fig. 42B.8) and air fill the gallbladder and extrahepatic bile ducts. In a recent report, a barium enema showing contrast filling of the biliary tract was used to diagnose a patient with a rare choledochocolonic fistula causing severe diarrhea (Anees et al, 2008). Failure of even a barium enema to show a cholecystocolonic fistula has been reported, but this is quite rare. In such cases, the cholecystocolic fistula is diagnosed by ERCP (Arvanitidis et al, 2004; Schoeters et al, 2002).

FIGURE 42B.8 A and B, Cholecystocolic fistula seen by barium enema.

There is little controversy about the appropriate treatment for cholecystocolic fistulae. Except in the most extenuating circumstances, the fistulae should be dismantled because of the ever-present risk of sepsis. If the diagnosis can be made preoperatively, suitable mechanical and antibiotic bowel preparation reduces the chance of infectious complications of surgery and aids primary closure of the large bowel. Cholecystectomy and, if indicated, common duct exploration should be done at the same time. Centers with significant expertise in laparoscopic surgery have reported successful treatment of preoperatively diagnosed cholecystocolonic fistulae by this method (Chatzoulis et al, 2007; Gentileschi et al, 1995; Hida et al, 1999; Lee et al, 2004; Wang et al, 2006). Regardless of the choice of surgical treatment, preoperative ERCP with sphincterotomy and stone extraction may obviate the need to explore the CBD and, in rare cases, might result in spontaneous closure of the fistula, obviating surgery altogether (Marshall et al, 1990).

Cholecystocholedochal Fistula, Including Mirizzi Syndrome

The pathogenesis of cholecystobiliary fistula is similar to the mechanism of fistula formation between the gallbladder and other adjacent segments of the alimentary tract: the offending calculus remains impacted in the ampulla of the gallbladder or cystic duct, and the resultant inflammation causes adherence and then perforation into the adjacent structure (see Fig. 42B.1D) In 1948, Mirizzi described the clinical picture in detail and called it functional hepatic syndrome. It is now commonly referred to as Mirizzi syndrome (see Chapters 29 and 43). The mechanism of jaundice was postulated by Mirizzi to be due to spasm of the hepatic sphincter secondary to inflammation in the region of the cystic duct junction with the common hepatic duct (Mirizzi, 1948); however, extensive histologic studies have failed to disclose a “sphincter” in the common hepatic duct. The large size of the stone and the acute cholecystitis with marked pressure necrosis and inflammatory reaction at the site of stone intrusion into the common hepatic duct combine to produce jaundice with variable components of extrinsic compression and intrinsic calculous blockage of bile flow.

The early phase of this cholecystobiliary fistula presumably exists when a large gallstone is impacted in the ampulla of the gallbladder or in the intramural segment of the cystic duct, which often courses parallel to the common duct. The jaundice that occurs as a consequence of this pathologic anatomy is clinically indistinguishable from choledocholithiasis. McSherry and colleagues (1982) suggested that this classic picture (Fig. 42B.9) of Mirizzi’s “functional hepatic syndrome” be subclassified as type 1. With progression of the disease, extrusion of the stone into the common hepatic duct may occur, and a fistula may form between the gallbladder and the hepatic duct. This is classified as type 2 (see Fig. 42B.1D), which is characteristically diagnosed intraoperatively by observing a gush of bile on removal of the impacted stone, indicating a biliobiliary fistula. Csendes and colleagues (1989) further subclassified type 2 patients into three categories based on the percentage of the wall of the common duct that was eroded by the offending calculus. In this subclassification, type II is a fistula involving less than one third of the circumference of the bile duct, type III involves two thirds, and type IV is a fistula with complete bile duct destruction. This classification scheme was recently updated to include a type V, which describes cholecystoenteric fistula, with or without gallstone ileus, coexisting with any of the other types (Csendes et al, 2007).

FIGURE 42B.9 A and B, Mirizzi syndrome type 1 (without cholecystocholedochal fistula). Percutaneous transluminal cholangiography shows an impacted cystic stone with extrinsic compression of the proximal hepatic ducts.

The large and solitary nature of the ampullary and cystic duct stones may be a hint to the preoperative diagnosis. The classic findings on ultrasonography include dilation of the biliary system that includes the common hepatic duct above the gallbladder neck, a stone impacted in the gallbladder, and an abrupt change in the caliber of the common duct below the stone (Hayek et al, 1988). Because most of these patients present with jaundice or some abnormalities on LFTs, a preoperative ERCP or PTC frequently is performed. These imaging studies permit a precise delineation of the condition, markedly altering surgical strategy. Cornud and others (1981) showed the appearance of biliobiliary fistulae at PTC, whereas Heil and Belohlavek (1978) relied on ERCP. In the six patients documented by McSherry and colleagues (1982), PTC and ERCP were equally effective in elucidating the cause of common duct obstruction. Although MRCP has been shown to diagnose Mirizzi syndrome preoperatively, it is limited, because it rarely shows the fistula and does not afford therapeutic stenting (Rappeport, 2002).

Biliobiliary fistulae have been referred to as a “trap” in the surgery of cholelithiasis (Corlette & Bismuth, 1975). This term is appropriate, because the presence of such a fistula frequently is not recognized until the time of surgery, often not soon enough to prevent injury to the common duct in an attempt to dissect the ampulla of the gallbladder or the cystic duct. Lygidakis (1981) emphasized the technical problems in operating on these patients. Other authors (Baer et al, 1990; Dewar et al, 1990, 1991; Yip et al, 1992) emphasized the importance of preoperative direct cholangiography. In patients with jaundice, biliary drainage may be necessary in the preoperative management.

If a large adherent mass about the gallbladder and CBD is encountered at surgery, operative cholangiography, although difficult, may delineate the presence of true choledocholithiasis or of the impacted stone without fistula (Mirizzi type 1) or the biliary fistula itself (Mirizzi type 2). In any event, it is unwise to attempt complete cholecystectomy; it is much better to open the gallbladder, remove the stones, and directly assess the presence or absence of a fistula. A portion of the gallbladder wall is sent for frozen section histology to exclude concomitant malignancy, but choledochotomy is seldom necessary, because multiple stones are infrequent in this disease. For Mirizzi type 2 disease, direct choledochotomy may be done, but the obstructing stone usually can be removed via the opened gallbladder. The gallbladder may be dissected 1 to 2 cm proximal to the fistula, between the ampulla and the common hepatic duct, at which point it is transected and sutured closed. T-tube drainage of the common duct may be achieved through the longitudinal choledochotomy, but this is often unnecessary. Alternatively, the opened gallbladder may be anastomosed to the duodenum as described by Hadjis and colleagues (1987) and later advocated by Baer and others (1990) and Mishra and others (1990). Stenosis of the biliary tree often resolves spontaneously in the postoperative period, and choledochotomy is seldom indicated.

Since the introduction of laparoscopic approaches to surgery of the gallbladder and the bile ducts, several cases and small series of laparoscopic treatment of Mirizzi syndrome have been reported (Bagia et al, 2001; Binnie et al, 1992; Chowbey et al, 2000; Paul et al, 1992; Rust et al, 1991; Schafer et al, 2003; Silecchia et al, 1995; Vezakis et al, 2000; Yeh et al, 2003; Rohatgi & Singh, 2006; Kwon & Inui, 2007). To address the utility of laparoscopic treatment of Mirizzi syndrome, Antoniou and colleagues (2010) recently conducted a comprehensive systematic review of the literature on this topic, a query confined to the English and German literature. Because of the strict study criteria, the authors identified only 10 of 66 articles for comparison in the years between 1989 and 2008. The total number of patients treated by laparoscopy was 124, of which 73 (59%) were sucessfully completeted. Conversion to open operation was necessary in 51 (41%) patients. Interestingly, patients from studies reporting a high preoperative diagnosis rate (>80%) had a significantly lower risk for conversion. The main reasons for conversion included technical failure as a result of dense adhesions in the triangle of Calot, unclear anatomy, and unsuccessful stone retrieval. The analysis showed a complication rate of 16%, with residual stones and bile duct injury the most common complications. The authors concluded, and we agree, that laparoscopic treatment of Mirizzi syndome cannot be recommended as a standard procedure because of the high failure rate (Antoniou et al, 2010).

Paul and others (1992) described successful laparoscopic treatment of Mirizzi syndrome by adhering to a luminal approach to the problem, as advocated by Dewar and associates (1991). This approach includes preoperative ERCP and placement of a biliary stent, followed by use of a flexible choledochoscope introduced into the cystic duct high from the open neck of the gallbladder. Stone extraction from the dilated cystic duct via the choledochoscope may avoid carrying the dissection too far down and injuring the common duct. Although such successful laparoscopic approaches to Mirizzi syndrome have been reported, we believe an open approach should be used when the diagnosis is made preoperatively, or a procedure should be converted to open when encountered during laparoscopy. Open technique affords the use of tactile senses, allowing easier removal of the impacted stone and sampling of the gallbladder wall to exclude a coexisting carcinoma (see Chapter 49); an open approach also minimizes the risk of bile duct injury.

Proximal Choledochoduodenal Fistulae Secondary to Peptic Ulcer Disease

Patients with proximal choledochoduodenal fistulae secondary to peptic ulcer disease may be asymptomatic, or they may present with GI complaints suggesting peptic ulcer (see Fig. 42B.1E). Biliary tract symptoms are usually absent, and these patients generally do not have associated cholelithiasis. In rare instances, cholangitis, jaundice, or abnormal LFTs are part of the clinical picture and indicate concomitant biliary tract infection and obstruction. The diagnosis may be suggested by pneumobilia in 15% to 60% of patients. More often, the diagnosis is made as contrast material from a barium meal refluxes up the common duct to outline a normal-sized, functioning gallbladder. Endoscopy with direct visualization of the ulcer and fistula and ERCP are the best studies to confirm the diagnosis and evaluate the extent of disease (Fig. 42B.10).

FIGURE 42B.10 Choledochoduodenal fistula secondary to gallstone disease shown by endoscopic retrograde cholangiopancreatography.

Given the paucity of reported experiences with choledochoduodenal fistula (Kourias & Chouliaras, 1964) and long-standing differences of opinion about the correct approach to peptic ulcer disease, it is not surprising that, until recently, treatment recommendations have been controversial. Excellent discussions of the natural history, clinical presentation, and management of these fistulae have been reported by Constant and Turcotte (1968), Feller and colleagues (1980), and Sarr and colleagues (1981). Most authors now agree that treatment should be directed at the ulcer diathesis and not at the biliary tract or the fistula itself. Medical management with proton-pump inhibitors and therapy for Helicobacter pylori is often sufficient to control the ulcer disease and even bring closure of the fistula.

More recent high success rates of medical management for peptic ulcer disease have resulted in a drastic reduction in the number of case reports of patients with proximal choledochoduodenal fistulae compared with years before 1990 (H’ng & Yim, 2003; Jaballah et al, 2001; Wong et al, 2004). Although choledochoduodenal fistula as a result of peptic ulcer is now extremely rare, clinicians should remain aware of this clinical entity, especially in symptomatic patients with refactory peptic ulcer disease. In such patients, surgery is indicated to prevent major complications such as hemorrhage and death (La Greca et al, 2008). Otherwise, surgery is not generally indicated for severe ulcer disease, except in the presence of uncontrollable hemorrhage, free perforation, obstruction, or intractability.

Many types of ulcer operations have been performed successfully; however, if technically possible, an exclusion type of gastric resection or duodenal bypass procedure, such as a Billroth II gastrectomy or gastroenterostomy, should be performed in addition to a vagotomy. There is no need to close the fistula, and doing so may injure the duodenum or bile duct. Cholecystectomy and biliary enteric reconstruction may be done, but these are reserved in cases of biliary stricture; such patients are younger and healthier than patients with biliary-enteric fistulae secondary to calculous biliary tract disease, and the results of medical and surgical treatment are usually good.

Distal Parapapillary Choledochoduodenal Fistula

Whether caused by spontaneous gallstone erosion or by iatrogenic damage to the distal common duct, parapapillary fistulae have a clinical presentation similar to that of other advanced calculous biliary tract diseases. In the series reported by Tanaka and Ikeda (1983), the following observations were made: a history of biliary symptoms longer than 10 years’ duration in 46%; pain and/or jaundice in 88% and 69%, respectively; prior biliary surgery in 54%; air or barium in the biliary tree in 41%; cholelithiasis in 71%; and choledocholithiasis in 38%. The anatomic diagnosis rests on meticulous endoscopic observation and expertise in the technique of ERCP (Fig. 42B.11; see Fig. 42B.1F). In Japan, the incidence of primary intrahepatic stone disease is reported to be 17% to 30% (Tanaka & Ikeda, 1983; see Chapter 39). Particularly in Japanese patients with parapapillary fistulae, careful and complete evaluation of the intrahepatic ductal system is important.

FIGURE 42B.11 Duodenoscopy showing the papilla of Vater (arrow) and a distal or peripapillary choledochoduodenal fistula (arrowhead).

(Courtesy Dr. Melih Karincaglu.)

The management of these biliary fistulae is still evolving, and a variety of endoscopic techniques have been advocated (Osnes & Kahrs, 1977; Ramsey et al, 1992; Schapira & Khawaja, 1982; Urakami & Kishi, 1978; Van Linda & Rosson, 1984). These techniques involve using endoscopic sphincterotomy to widen the choledochal fistula enough to permit free drainage or to create a common channel between the fistula and natural ampullary orifice. When necessary, open surgical procedures, such as hepaticodochojejunostomy, have been used (Hunt & Blumgart, 1980).

Fistula to the Hepatic Veins or Portal Veins

Biliary-venous fistula is a rare occurrence that results in bilhemia, a condition that can be dangerous (see Chapter 105), and such a fistula can also be associated with interventional transhepatic maneuvers. When a large caliber intrahepatic bile duct is transected, the creation of a transjugular intrahepatic portosystemic stent-shunt (TIPSS) may be complicated by a biliary-venous fistula. The resulting biliary leak plays an important role in the stenosis and occlusion of the portosystemic shunt (Jalan et al, 1996).

Fistulae After Cholecystostomy

The first open cholecystotomy was done by Bobbs in 1867 (Sparkman, 1967), but open cholecystostomy is infrequently performed today. Historically, the procedure was used in high-risk patients with acute cholecystitis. In selected cases, cholecystostomy also was used as the first step of a two-stage operation for obstructive jaundice secondary to malignancy (see Chapter 62A, Chapter 62B ). Percutaneous cholecystostomy, a less invasive procedure that requires only local anesthesia, is currently the procedure of choice in high-risk elderly patients (Byrne et al, 2003; Ito et al, 2004; Winbladh et al, 2009). Percutaneous placement of a catheter into the lumen of the gallbladder can be performed and can even be done under US guidance at the bedside of critically ill patients (see Chapters 28 and 32). A persistent fistula denotes distal biliary obstruction (e.g., a retained gallstone lodged within a Hartmann pouch).

Biliary Fistulae After Invasive Radiologic Procedures

Biliary leak and fistulae may follow any invasive radiologic procedure involving the hepatobiliary system. Usually these are due to distal biliary tract obstruction, a result of either a retained bile duct stone or an unrecognized malignancy (Fig. 42B.12). Published rates for biliary leak after interventional radiologic procedures vary significantly and depend on patient selection and individual practitioner experience. Complications result from suboptimal puncture location, incorrect selection of biliary catheter size, and inadequate postprocedural tube care (Winick et al, 2001). Burke and colleagues (2003) classified bile leakage, sepsis, and infected biloma as major complications that occur at rates of 2% after PTC and biliary drainage procedures (see Fig. 42B.5). The risk of an internal fistula after radiologic intervention resulting in bilhemia was referred to earlier.

FIGURE 42B.12 An 85-year-old man with severe cardiomyopathy (unfit for surgery) was treated by percutaneous cholecystostomy tube drainage. Six weeks after tube insertion, a cholecystogram shows a retained gallstone impacted in the distal common bile duct (arrow). The patient was treated definitively by endoscopic retrograde cholangiopancreatography and papillotomy.

Fistulae After Abdominal Operations

Laparoscopic Cholecystectomy

Laparoscopic cholecystectomy is currently the standard procedure for symptomatic cholelithiasis and for all forms of cholecystitis, including acute cholecystitis, and it is performed even in instances of gangrenous cholecystitis (Kiviluoto et al, 1998; Gharaibeh et al, 2002; Borzellino et al, 2008). However, laparoscopic cholecystectomy is associated with an increased incidence of bile duct injuries, including fistulae, which are reported in 1.3% to 5.5% of cases (see Chapter 34; Adamsen et al, 1997; Kum et al, 1996). Under these difficult circumstances, the procedure should be performed, or at least supervised, by an experienced surgeon. A high conversion rate of 40% in instances of gangrenous cholecystitis should be expected (Eldar et al, 1998; Gharaibeh et al, 2002; Borzellino et al, 2008).

As mentioned previously, the operative treatment of Mirizzi syndrome now includes a laparoscopic option. In accordance with others, however, we believe that when the presence of Mirizzi syndrome, particularly type 2, is suspected or realized, safety demands that the laparoscopic procedure be converted to open laparotomy (Moser et al, 1993; Yeh et al, 2003; Antoniou et al, 2010).

Small amounts of bile leakage may occur occasionally in the immediate postoperative period after inadvertent damage to a subvesical duct (Viikari, 1960), which is present in normal subjects in 20% to 50% of cases (Kune & Sali, 1980). Removal of an intrahepatic gallbladder also may cause damage to tiny bile ducts in the liver around the gallbladder fossa.

Biliary fistula after laparoscopic cholecystectomy may occur as a result of injury to the extrahepatic biliary tree, or it may originate from the cystic duct stump. A leak from the cystic duct stump may occur from burn injury, from pressure necrosis of a metal clip, or in most instances from distal bile duct obstruction caused by a retained stone, resulting in a buildup of pressure and a resultant blowout of the cystic stump. It is important to differentiate patients at risk of having choledocholithiasis. Patients having deranged LFTs or dilated bile ducts on US should undergo imaging of the biliary tree before cholecystectomy. Sphincterotomy and stone extraction should be performed if choledocholithiasis is established.

Most ductal injuries are not recognized during the initial laparoscopic cholecystectomy (Lillemoe et al, 1997) or in the immediate postoperative period, mainly because drains usually are not left in the abdomen, and the patient typically leaves the hospital within 24 hours. The resulting uncontrolled biliary fistula becomes evident within days or sometimes weeks after the operation, with the clinical presentation of abdominal pain, fever, jaundice, and the demonstration of an intraabdominal fluid collection that produces bile on puncture.

Compared with open cholecystectomy, laparoscopic cholecystectomy is associated with an increased rate of bile duct injury (see Chapter 42A; Davidoff et al, 1992; Waage & Nilsson, 2006). Such injuries are common to most reported series of laparoscopic cholecystectomy and may reach an incidence of 0.9%. Reviews by Strasberg and colleagues (2001) and Vecchio and colleagues (1998) that encompass more than 100,000 patients found the incidence of major bile duct injury to be around 0.5%, and this incidence has reached a “steady state” (Walsh et al, 1998; Wherry et al, 1996; Waage & Nilsson, 2006). A large retrospective cohort analysis of nearly 1.6 million Medicare patients in the United States undergoing laparoscopic cholecystectomy from 1992 through 1999 confirmed the incidence rate of bile duct injury at 0.5% (Flum et al, 2003), and a Swedish population-based study of more than 150,000 patients showed a similar bile duct injury rate of 0.4% (Waage & Nilsson, 2006).

The mechanisms resulting in bile duct injuries are now well recognized (see Chapter 42A). The most common injury is caused by misidentification of the common duct for the cystic duct, resulting in complete transection of the common duct, often with some portion of the biliary tree (Fig. 42B.13). A traction injury results from inadvertent lateral traction of the gallbladder and “tenting” of the cystic duct–common duct junction. In this instance, the clip intended for the cystic duct may occlude the CBD, or a portion of the CBD may be removed between clips. An unrecognized anomalous biliary system—in particular, a cystic duct that empties directly into the right hepatic duct or a low-entry right hepatic sectoral duct—may result in similar damage (Fig. 42B.14; see Chapters 1B and 42A; Davidoff et al, 1992; Lillemoe et al, 1997). Other, less common mechanisms include thermal injury resulting from excessive use of the cautery or laser and the application of excessive clips to control bleeding in the triangle of Calot.

FIGURE 42B.13 Injury to the bile duct at laparoscopic cholecystectomy. The common bile duct was misidentified for the cystic duct, resulting in transection of the common bile duct between the clips. A, Endoscopic retrograde cholangiopancreatography shows a transected bile duct at the level of a clip (arrow) with extravasation of contrast material. B, Hepatobiliary iminodiacetic acid scan shows a total uncontrolled fistula originating from the proximal cut end of the choledochus (arrow).

FIGURE 42B.14 Injury to a right sectoral hepatic duct at laparoscopic cholecystectomy. A, Percutaneous transluminal cholangiography. Note the remnant of the duct draining into the common bile duct (solid arrow) and cystic stump (open arrow). Note the low entry of this duct into the common bile duct. B, Tube fistulography. The biliary ductal system of the cut sectoral duct is outlined.

The role of operative cholangiography (see Chapter 21) in the prevention of bile duct injuries remains controversial despite two large, population-based studies demonstrating a protective effect (Flum et al, 2003; Waage & Nilsson, 2006). Intraoperative cholangiography (IOC) may supply information regarding the presence of unsuspected choledocholithiasis and unexpected anomalous anatomy, and its routine use is recommended by several authors (Fletcher et al, 1999; Flum et al, 2003; Kohn et al, 2004; Stuart et al, 1998; Waage & Nilsson, 2006). The use of IOC has not increased; in reports of laparoscopic cholecystectomy in the United States, IOC was performed in about 40% of cases (Flum et al, 2003; MacFadyen et al, 1998). In a population-based study by Flum and colleagues (2003), the rate of bile duct injury was found to be significantly higher when IOC was not used (0.39% vs. 0.58%). The more recent study by Waage and Nilsson (2006) showed that IOC reduced the risk of bile duct injury by 37%.

Before these reports, evidence that IOC prevented major bile duct injury did not exist (Wright & Wellwood, 1998). In a national survey of American surgeons performing laparoscopic cholecystectomy, Archer and colleagues (2001) found that IOC was helpful for intraoperative detection of bile duct injury. Interestingly, of the 1600 surgeons surveyed in the United States, one third had experienced this complication. A single institutional study by Kohn and associates (2004) examined the use of routine versus selective cholangiography among surgeons performing laparoscopic cholecystectomy. This study found that surgeons performing selective cholangiography were less likely to attempt IOC, even if indications arose resulting in significantly worse adverse events. Regardless of surgeon preference for routine or selective IOC, we recommend a low threshold for early conversion to laparotomy, if the ductal anatomy remains unclear or for any other concern. IOC remains the gold standard to define biliary anatomy, but it cannot prevent all bile duct injuries; and in some rare cases, it may even be the cause.

Open Cholecystectomy

The occurrence of unexpected biliary fistulae after cholecystectomy almost always indicates operative injury to a major bile duct. Such fistulae may arise from damage to the CBD (Fig. 42B.15) or to an anomalous sectoral right hepatic duct (see Chapter 42A). A 0.21% incidence of bile duct injuries was found in a study of 42,474 patients who underwent an open cholecystectomy (Roslyn et al, 1993). Bile duct injury is recognized at the time of cholecystectomy in only a few patients; in approximately 25% to 40% of patients with unrecognized bile duct injury, the injury becomes apparent only when the presence of a biliary fistula is recognized (Andren-Sandberg et al, 1985a, 1985b). In the remaining patients, the injury is recognized only later, when a biliary stricture develops (Blumgart et al, 1984). The recognition of biliary ductal anomalies and prevention of biliary injury at cholecystectomy are discussed in detail in Chapters 1B and 42A. Inadequately ligated or sloughed ligatures on the cystic duct are rarely responsible for biliary fistulae, and for this reason, transfixion suturing of the cystic stump is recommended. However, the presence of an unrecognized significant distal obstruction may be followed by a blowout of the cystic duct stump, resulting in a biliary fistula or bile peritonitis.

FIGURE 42B.15 Postcholecystectomy fistulography. Biliary fistula caused by accidental transection of the common hepatic duct (arrow), the most common cause of a persistent external biliary fistula.

Cholecystectomy is sometimes performed under difficult circumstances, as in the presence of a gangrenous gallbladder associated with fibrosis and inflammation in the region of the triangle of Calot. In these instances, proper identification and transfixion of the cystic stump may not be possible, and the patient is left with a temporary biliary fistula (Fig. 42B.16). As described previously, type 2 Mirizzi syndrome may pose significant technical difficulties, and specific surgical techniques have been devised to deal with this situation (Baer et al, 1990).

FIGURE 42B.16 Postcholecystectomy fistulography outlining large irregular cavity (thick arrow) and connecting with a faintly outlined, but apparently normal, biliary ductal system (thin arrow).

If a defect in the bile duct is found during operation, and if immediate repair is impossible, it may be better to end the procedure with adequate drainage, expecting a future controlled fistula. In all these difficult instances in which a biliary fistula is anticipated, it is important to ensure controlled and adequate drainage of bile and to exclude the presence of a distal obstruction to biliary-enteric bile flow. Under these circumstances, most fistulae close spontaneously after conservative treatment.

Common Duct Exploration

The classic open common duct exploration is now less frequently performed, having been replaced by laparoscopic and endoscopic techniques (see Chapter 35; Csendes et al, 1998). Whether it occurs after open or laparoscopic exploration of the CBD or persists after removal of a T-tube, a biliary fistula is almost always due to a residual common duct gallstone (Fig. 42B.17). It is essential to perform cholangiography and rule out the presence of retained stones before removal of a T-tube or biliary stent.

FIGURE 42B.17 Postcholecystectomy T-tube cholangiography shows a retained stone obstructing the distal bile duct (arrow). The stone was removed 6 weeks after surgery via instrumental basket extraction through the T-tube tract, preventing a persistent external biliary fistula.

Less commonly, an overlooked malignant distal obstruction is the causative factor (Fig. 42B.18). In addition, the passage of metal bougies through the papilla during common duct exploration may result in the creation of false tract, which may result in a choledochoduodenal fistula and may cause jaundice, ascending cholangitis, and acute or chronic recurrent pancreatitis. Treatment may be by endoscopic papillotomy or sphincterotomy, joining the fistulous orifice with the papillary opening (Chung & Roberts-Thomson, 2000; Jorge et al, 1991; Karincaoglu et al, 2003b), but a surgical approach may be necessary (Hunt & Blumgart, 1980).

FIGURE 42B.18 Postcholecystectomy fistulography shows the external biliary fistula, which was caused by an extrinsic compression of the pancreatic portion of the bile duct (arrows). At reoperation, choledochal compression was shown to be due to cancer of the head of the pancreas.

Fistula After Liver Injury, Liver Resection, and Liver Transplantation

Liver Injury

Liver injury (see Chapter 102) may be followed by the formation of biliary fistulae. Such fistulae may occur in association with damage to the liver (Fig. 42B.19) and the bile ducts or may follow sequestration and infection of areas of liver necrosis. Blunt or penetrating grade III or IV liver trauma may be complicated by bile collections and biliary fistulae in 0.5% to 14% of patients (Glaser et al, 1994; Goldman et al, 2003; Gourgiotis et al, 2007; Shahrudin & Noori, 1997; Vassiliu et al, 2004; Wahl et al, 2005). Emergency partial hepatectomy for major liver trauma may result in injury to the bile ducts at the confluence with an early biliary leak and, later, a biliary stricture (Bismuth et al, 1986). Complete transection of the CBD requires immediate hepaticojejunostomy, whereas lacerations of the main biliary channel may be sutured after placement of a T-tube.

FIGURE 42B.19 External biliary fistula after blunt injury to the right lobe of the liver and subsequent drainage of a large right intrahepatic hematoma. The injury was associated with damage to the right hepatic duct and a subsequent intrahepatic stricture (arrow). After external drainage, a high-output biliary fistula developed. The tubogram illustrated was obtained after anastomosis of the fistulous tract issuing from the liver to the adjacent mobilized gallbladder. The cavity within the liver has collapsed, and the gallbladder fills and subsequently outlines the common bile duct. The tube was removed, and postoperative recovery was uneventful. The patient remained symptom free and with normal liver function test results 5 years after surgery.

(From Smith EE, et al, 1982: The management of post-traumatic intrahepatic cutaneous biliary fistulas. Br J Surg 69:317-318.)

Although nonoperative management of blunt liver injury is now firmly established as standard therapy for hemodynamically stable patients (Malhotra et al, 2000; Pachter & Hofstetter, 1995; Christmas et al, 2005), caution should be exercised, because this approach can be hazardous sometimes and demands close observation to avoid delayed surgical treatment of potentially fatal complications (Goldman et al, 2003). Most posttraumatic biliary fistulae can be expected to heal spontaneously (Vassiliu et al, 2004); however, in a case series of 32 patients treated nonoperatively, Carrillo and colleagues (1999) found severe biliary complications in up to 85% of patients with a liver abbreviated injury score (AIS) of grade IV or higher. A study specifically examining the diagnosis and management of bile leaks after liver injury by Wahl and colleagues (2005) confirmed that patients with liver injuries higher than grade IV are more likely to develop bile leaks, especially if treated by arterial embolization. These authors proposed a treatment algorithm for blunt liver injury associated with a bile leak, which includes laparoscopic washout for a diffuse leak. Laparoscopic evacuation and lavage as a new treatment option for bile collections has also been advocated by others to prevent the inflammatory sequelae of bile peritonitis (Franklin et al, 2007). Persistent biliary fistulae may occur from a segment of the liver isolated by the injury. Management of this situation is difficult, particularly when the fistula is associated with a distal stricture. Rarely, the fistula can be identified at operation and oversewn. Alternatively, a well-developed fibrous fistulous tract may be anastomosed to a jejunal loop or to the gallbladder (see Fig. 42B.19; Smith et al, 1982).

Biliovenous fistula with subsequent leakage of bile into the venous system (bilhemia) is a rare but serious complication of liver trauma. After the formation of a necrotic cavity within the liver, an open connection between a vein and an intrahepatic bile duct may occur, allowing bile to leak into the venous circulation (see Chapter 105; Haberlik et al, 1992). Rarely, transjugular intrahepatic portosystemic shunting (TIPS; see Chapter 76E) has been reported to be a direct cause of bilihemia (Jawaid et al, 2003; Singh et al, 2007; Singal et al, 2009).

Liver Resection

The incidence of bile leak after hepatic resection ranges from 1.7% to 12% in large series (Lo et al, 1998; Capussotti et al, 2006; Nagano et al, 2003; Vigano et al, 2008; Tanaka et al, 2002; Yamashita et al, 2001; see Chapter 25, Chapter 90A, Chapter 90B, Chapter 90C, Chapter 90D, Chapter 90E, Chapter 90F ). In most patients, bile leak resolves spontaneously, but sometimes a persistent fistula requires reoperation (Honore et al, 2009). Liver resection done for tumor (see Chapter 90A, Chapter 90B, Chapter 90C, Chapter 90D, Chapter 90E, Chapter 90F ) may be followed by biliary fistula, which may result from inadequate ligation of the bile ducts at the cut liver surface (Thompson et al, 1983) or from failure to secure the bile ducts at the hilus (Fig. 42B.20). This failure is more likely after right hepatectomy, in which the anatomy of the right sectoral ducts is variable in the hilar region. Extended left hepatic lobectomy also has been associated with a high incidence of biliary fistula (see Chapter 90A, Chapter 90B, Chapter 90C, Chapter 90D, Chapter 90E, Chapter 90F ; Starzl et al, 1982). In Japan, Yamashita and colleagues (2001) reported a biliary fistula rate of 4% (31 of 781) after hepatic resection. Their analysis identified operative procedures exposing the major Glisson capsule and including the hilum—anterior sectorectomy, central hepatectomy, and caudate resections—to be high-risk operations for development of postoperative bile leakage. Other factors that may contribute to a persistent bile fistula may include underlying cirrhosis or chronic hepatitis that impair wound healing (Tanaka et al, 2002). Once a posthepatecomy biliary fistula is established, if the drainage output exceeds 100 mL per day 10 days after diagnosis, interventional or endoscopic procedures should be considered to promote definitive closure (Vigano et al, 2008).

FIGURE 42B.20 A 61-year-old woman with isolated metastatic ovarian cancer underwent a left hepatic lobectomy and developed a persistent postoperative biliary fistula. A, Tube cholangiogram shows biliary segments without communication to the main biliary tree. B, Endoscopic retrograde cholangiopancreatography confirms noncommunication of the biliary segments. C and D, Computed tomography shows retained contrast medium within operative drain (arrowheads) and accessory segment VI and caudate duct branches (arrow).

(Courtesy Dr. Jeanne Laberge.)

Operative injury to the biliary tract that is likely to result in fistula is more common after resection of lesions involving or close to the hilar structures (Fig. 42B.21). It also is more likely to occur after resection of lesions involving the caudate lobe ducts, because the anatomy of these ducts is significantly variable in the hilar region. Using major hepatic resection, resection of cholangiocarcinoma combined with biliary-enteric reconstruction may be complicated by a biliary fistula originating from the caudate lobe ducts or from the biliary-enteric anastomosis (see Chapter 90A, Chapter 90B, Chapter 90C, Chapter 90D, Chapter 90E, Chapter 90F ). Hepatic cryotherapy (see Chapter 85B), which may be used at the cut liver surface after hepatectomy or for ablation of deep intrahepatic lesions, may also be complicated by biliary fistula (Sarantou et al, 1998). It may be prudent to leave a drain in situ after this procedure.

FIGURE 42B.21 Fistulography obtained via a tube drain shows a biliary fistula after extended left hepatic lobectomy for primary hepatocellular cancer. The tumor was large and involved the hilar ducts; tumor extension was shown within the left hepatic duct. The fistula was managed conservatively, and it closed within 4 weeks. Arrows indicate the fistula and the course of the right hepatic duct and common bile duct.

Surgery for Hydatid Disease

Surgery is an important treatment option for patients with hydatid disease (Dziri et al, 2004). Operative options include pericystectomy and hepatic resection or simply unroofing of the cyst and treatment of the residual cavity. Patients treated by these methods are prone to all the known complications associated with liver resection. Hydatid disease of the liver (see Chapter 68) is associated with biliary involvement by the disease in approximately 10% of instances (Erguney et al, 1991). An expanding liver cyst results in compression and stretching of adjacent liver tissue, including the bile ducts. It may erode into a stretched bile duct, with the establishment of continuity between the cyst cavity and the biliary system. Hydatid material may enter into the biliary tree, or bile may leak into the cyst. As with other hepatobiliary conditions, a laparoscopic surgical management option for hepatic hydatid disease has been introduced, but it is limited to those few centers where the disease is endemic (Duttaroy et al, 2008; Palanivelu et al, 2006; Sharma et al, 2009).

Biliary fistula may develop after operation for hydatid disease in certain situations. First, a communication between the cyst cavity and the biliary system is missed at operation and is not directly secured (Fig. 42B.22). It is prudent to drain all cyst cavities, particularly those of multiloculated hydatid cysts, to ensure that if a biliary fistula were to develop, it would be controlled. Unless a distal obstruction is present, these fistulae usually close spontaneously. Second, and rarely, the presence of hydatid material within the biliary tract produces biliary ductal obstruction (Fig. 42B.23) that results in a persistent biliary fistula that is relieved only when the hydatid material passes or is removed. Removal is achieved by exploration of the CBD with or without a bypass procedure (Ozmen et al, 1992) or by endoscopic methods (Iscan & Duren, 1991).

FIGURE 42B.22 Persistent external biliary fistula after external drainage of a hydatid cyst cavity (arrow) in the left lobe of the liver. Fistulography shows clear communication between the cyst cavity and the left hepatic duct.

FIGURE 42B.23 Fistulography obtained after excision of a right hepatic hydatid cyst. Note the persistent fistula as a consequence of retained hydatid material in the common bile duct (arrow). Treatment was by exploration of the common bile duct and surgical removal of the retained hydatid material. The fistula rapidly closed.

Assessment of the biliary tree, preferably by endoscopic cholangiography, should be performed before surgery in patients with a history of jaundice or cholangitis or in the presence of a large cyst located centrally and abutting the hilar structures. Kayaalp and colleagues (2003) in a series of 113 patients showed that the location of the hydatid cyst near the hilum is a risk factor for the development of a cystobiliary communication and cavity-related complications. When a cystobiliary communication is shown, the biliary system should be cleared of all debris and cyst remnants, and endoscopic sphincterotomy should be performed before surgical intervention (Kornaros & Aboul-Nour, 1996). Percutaneous treatment of hydatid cysts is associated with a 10% incidence of biliary fistula (Men et al, 1999). Such fistulae usually close spontaneously after any biliary distal obstruction is relieved.

Treatment

The principles of management of postoperative biliary fistulae are essentially the same regardless of the initial surgical procedure; these principles are outlined in Figure 42B.28 and are modified slightly according to the initial surgical procedure and the individual patient. ERCP and endoscopic techniques play an integral and indispensable role in the management of biliary fistulae. The individual treatment plan should be made and agreed on jointly by all members of the team involved in the treatment of the patient.

FIGURE 42B.28 Proposed management plan for postoperative biliary fistula. The initial treatment is most important and includes the establishment of a controlled fistula. This is achieved radiologically, by percutaneous drainage of a collection, or surgically, at which time no attempt at primary repair is made. The fistula is evaluated for biliary-enteric continuity. When a total fistula has been diagnosed, it is most important to ensure that it is controlled. Definitive surgical repair is attempted after a reasonable period of conservative treatment. When biliary-enteric continuity is present with free biliary-enteric bile flow, conservative treatment is instituted and results in spontaneous closure in most cases. The presence of biliary-enteric continuity with distal obstruction calls for some form of treatment to relieve the obstruction, and this is done endoscopically or, preferably, percutaneously by balloon dilation. After the establishment of free biliary-enteric bile flow, patients are treated conservatively. In suitable cases, nasobiliary intubation is followed up carefully after fistula closure for early signs of stricture. Highly complex hilar fistulae are treated conservatively with prolonged periods of drainage. The fistula is expected to close, and this usually occurs together with the development of stricture. After a stricture develops, it is treated surgically.

As stated earlier, it is important to establish a controlled fistula; this can be achieved initially by US- or CT-guided drainage of the abscess or collection. Operation is indicated when nonoperative measures are not suitable, such as in a patient with diffuse bile peritonitis, a septic patient with an intraabdominal abscess too large to be drained by the percutaneous route, or a patient with necrotic material and debris within the abscess. Early surgery also is indicated when percutaneous drainage has failed. During the operation, the temptation to attempt primary repair at this stage must be resisted; only good drainage should be established. Occasionally, drainage of large amounts of bile with significant fluid and electrolyte loss may necessitate early operation, when the external fistula may be converted to an internal fistulojejunostomy using a mobilized and approximated Roux-en-Y jejunal loop (see Fig. 42B.19; Smith et al, 1982). In this procedure, the drainage tube is led from the fistula origin through the jejunal loop to the exterior; this allows control, but it creates an almost certain necessity to reoperate for restenosis at a later stage.

It is important to identify the presence or absence of biliary-enteric bile flow, which differentiates a total biliary fistula from a partial fistula with some residual biliary-enteric continuity. Subsequent management is influenced significantly, because operation is usually unavoidable in the former, and a nonoperative approach may be successful in the latter. A total biliary fistula rarely closes spontaneously when an internal fistula develops between the divided upper duct and the gut (Collins & Gorey, 1984).

When biliary-enteric continuity is present, and bile flow is not obstructed distal to the origin of the fistula, a prolonged period of conservative treatment is indicated, because spontaneous closure of the fistula is common. Fistula closure may be facilitated by temporary placement of a stent across the fistulous opening in the bile duct, excluding bile flow through the fistula. This method may be attempted where the CBD is intact above the fistula origin, and the defect in the common duct is not too large. Stenting may be achieved by endoscopic placement of an endoprosthesis or a nasobiliary tube with its tip above the origin of the fistula. When a short period of stenting is anticipated, nasobiliary intubation is preferred, because it enables follow-up cholangiography, damage to the papilla is minimal, and a second endoscopic procedure is avoided (Barthel & Scheider, 1995; Grala et al, 2009). Using this method, some fistulae close within 2 weeks (Toriumi et al, 1989). It is also useful in patients with liver transplant who have undergone duct-to-duct biliary reconstruction. In patients with concomitant CBD stones or stricture, a papillotomy is indicated. Nasobiliary tubes are cumbersome for the patient, and they may occasionally dislodge; if used for prolonged periods, they may enhance metabolic acidosis resulting from bile loss.

Endoscopic treatment of fistulae with intact biliary-enteric flow by sphincterotomy, stenting, or both is effective in most patients (Agarwal et al, 2006; Rauws & Gouma, 2004). In most instances, a biliary endoprosthesis is used and is left in place for several weeks, until the fistula closes. Closure is verified by HIDA scan before removal of the stent. If standard endoscopic therapy fails, an alternative endoscopic technique using n-butyl-2-cyanoacrylate glue occlusion of the fistula has been shown to be effective and helps avoid the need for surgery (Seewald et al, 2004).

Some authors recommend endoscopic sphincterotomy alone with the intention of reducing the pressure gradient between the biliary system and the duodenum (Ponchon et al, 1989). This procedure is unnecessary, because the fistula will close if no distal obstruction is present. Sphincterotomy may also be associated with short-term and long-term septic and obstructive complications (Kracht et al, 1986), therefore it should be avoided unless specifically indicated (e.g., in patients with a papillary stricture). It also has been shown that stenting is more effective than sphincterotomy alone in the resolution of biliary fistulae (Marks et al, 1998). As stated earlier, we believe that although endoscopic approaches are useful, they generally should be delayed for days to a few weeks to allow spontaneous closure of the fistula, which obviates the need for further intervention and its attendant complications.

After an obstruction distal to the fistula has been diagnosed, it should be dealt with, because until it has been managed, the fistula will not close spontaneously. Obstruction is usually caused by a retained stone or a stricture: retained stones usually are removed by endoscopic means, and relief of a benign stricture can be achieved using balloon dilation, applied either endoscopically or by interventional radiology after PTC. The patient is treated conservatively and expectantly for fistula closure or for restricture, and fistula closure can be facilitated by temporary stenting (Fig. 42B.29), if the location and the size of the defect in the bile duct are suitable, and restricture is treated operatively.

FIGURE 42B.29 Combined surgical/endoscopic/radiologic management of a biliary fistula diagnosed 3 weeks after an open cholecystectomy. A, Percutaneous transluminal cholangiography shows a biliary fistula with a significant distal stricture (arrow). B, Percutaneous transhepatic balloon dilation of the stricture. C, Percutaneous transhepatic guide is exchanged for a nasobiliary tube, which is placed endoscopically with its tip above the origin of the fistula. Free biliary-enteric bile flow is obtained. The fistula is still present but is smaller. D, Check-up cholangiography (lateral view) after 2 weeks of nasobiliary drainage shows fistula closure. The nasobiliary drain was removed.

The use of temporary stenting is limited in highly complex hilar fistulae combined with stricture, particularly fistulae with separation of a right and left ductal system. Some authors place an endoprosthesis across the stricture and leave it for months (Ponchon et al, 1989), even for more than 1 year, with periodic replacements (Davids et al, 1992). This practice is not only unnecessary but is often harmful, resulting in obstruction and septic complications; the endoprosthesis should be removed when the fistula has closed.

Management of biliary fistula after laparoscopic cholecystectomy follows the same principles. Because early recognition of the biliary fistula or bile duct damage significantly affects treatment, a high index of suspicion is important. Initial evaluation includes abdominal US and HIDA scan to rule out the presence of an abnormal collection and to establish the presence of free biliary-enteric bile flow. Abnormal findings at either study call for ERCP. Treatment of biliary fistula with or without biliary-enteric continuity follows the guidelines outlined. A biliary fistula associated with malignant distal obstruction is treated with reoperation and surgical excision of the neoplasm or the creation of an appropriate biliary-enteric bypass.

After closure of the fistula, patients are followed up carefully with regular LFTs and HIDA scans to detect early signs of the development of a biliary stricture, which may take months and sometimes years. Stricture is most likely to occur where a stricture has already been present or where a fistula is associated with an injury to a major bile duct (Blumgart et al, 1984). The proposed plan may involve relatively prolonged management, but it improves the chances of a successful and long-lasting bile duct repair, especially after injury at cholecystectomy. In this situation, early and untimely surgical attempts at definitive repair carry a high risk of biliary leak and anastomotic stricture.

Management of the rare patient with excluded or “orphaned” segemental bile ducts following hepatectomy is extremely challenging. This complication results from unrecognized anatomic biliary variations (see Chapter 1B), or when normal anatomic planes are ignored during hepatic transection. The end result is that a portion of liver tissue remains well perfused but entirely disconnected from the biliary tract (see Fig 42B.20). Multiple interventional treatments have been described, the majority consisting of sclerotic agents that are injected directly into the fistula tract. These substances include tetracycline, ethanol, and acetic acid, (Shaw et al, 1989; Shimizu et al, 2006; Park et al, 2005). Other agents such as fibrin glue, thrombin, and n-butyl cyanoacrylate have been used with variable success (Saad & Darcy, 2008; Vu et al, 2006). We are aware of one successful case report of a patient who was treated by percutaneous intraductal laser ablation after failing other treatments (Eicher et al, 2008). When these nonoperative treatments fail, repeat liver resection is necessary and usually definitive (Honore et al, 2009). The authors have successfully used ablation of the liver tissue containing the orphaned biliary segement to treat persistent biliary fistulae.