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Complications of gallstone disease: Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus
THE AMERICAN JOURNAL OF GASTROENTEROLOGY © 2002 by Am. Coll. of Gastroenterology Published by Elsevier Science Inc.
Vol. 97, No. 2, 2002 ISSN 0002-9270/02/$22.00 PII S0002-9270(01)04013-8
CLINICAL REVIEWS
Complications of Gallstone Disease: Mirizzi Syndrome, Cholecystocholedochal Fistula, and Gallstone Ileus Alaa Abou-Saif, M.D., and Firas H. Al-Kawas, M.D. Division of Gastroenterology, Georgetown University Medical Center, Washington, District of Columbia
ABSTRACT Gallstone is a common disease with a 10% prevalence in the United States and Western Europe. However, it is only symptomatic in 20 –30% of patients, with biliary pain “colic” being the most common symptom. Complications of asymptomatic gallstone disease are generally rare, with an incidence of ⬍1%/yr. The most common complications of gallstone disease are acute cholecystitis, acute pancreatitis, ascending cholangitis, and gangrenous gallbladder. Less frequent complications include Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. Mirizzi syndrome and cholecystocholedochal fistula are two manifestations of the same process that starts with impaction of a gallstone in the gallbladder neck that results in obstruction of the bile duct, causing jaundice. The gallstone may erode into the bile duct, causing cholecystocholedochal fistula. Gallstone ileus refers to small bowel obstruction resulting from the impaction of one or more gallstones after they have migrated through a cholecystoenteric fistula. An accurate diagnosis is essential to the management and prevention of further complications. A variety of imaging and endoscopic modalities are used to make the diagnosis once the condition is suspected clinically. Treatment should be tailored to each individual patient. Management choices include ERCP, lithotripsy (endoscopic or extracorporeal), and surgery. Prognosis is frequently related to early recognition, management of any comorbid conditions, and careful selection of treatment modalities. (Am J Gastroenterol 2002;97: 249 –254. © 2002 by Am. Coll. of Gastroenterology)
MIRIZZI SYNDROME Background and Classification In 1948 Doctor Pablo Mirizzi described a syndrome of hepatic duct obstruction—“sindrome del conducto hepatico”—in the setting of cholelethiasis and cholecystitis (1). This syndrome is caused by an impacted gallstone in the cystic duct or the neck of the gallbladder compressing the adjacent bile duct and resulting in complete or partial obstruction of the common hepatic duct. Mirizzi wrongly postulated that the inflammation resulted in a spasm of a physiological and anatomical sphincter comprised of circular muscle fibers within the common hepatic duct. Jaundice
and recurrent cholangitis are the two main clinical manifestations. Anatomically, a cystic duct parallel to the bile duct is one of the main features of this syndrome (2). It is now well known that there is no sphincter in the hepatic duct and that the jaundice is a result of the external compression caused by the impacted stone. Nevertheless, the syndrome has come to bear his name. It should be noted, however, that Kehr in 1905, Ruge in 1908, and Levrat and Chayvialle in 1941 reported external compression of the bile duct after stone impaction in the cystic duct (3, 4). In 1961, Mirizzi reported an additional seven cases of this syndrome encountered in 4000 patients with gallstone disease (5). In 1982, McSherry et al. (6) suggested a subclassification of Mirizzi syndrome into two types: type I involves the external compression of the common hepatic duct by a stone impacted in the cystic duct, and type II occurs when a cholecystocholedochal fistula caused by stone migration into the common hepatic duct is seen. Further modification was suggested by Csendes et al. (7). In this modification, type II is a cholecystocholedochal fistula that involves less than one third of the circumference of the bile duct, type III is a fistula involving up to two thirds of the duct circumference, and type IV is complete obstruction of the bile duct. Cholecystobiliary fistula is a rare entity. It was first reported by Puestow in 1942 (8) when he described a case of spontaneous internal biliary fistula. In 1950 and 1952 Behrend and Cullen and Mirizzi described a total of seven additional cases (9, 10). It is fair to say that Mirizzi syndrome and cholecystocholedochal fistulas are two stages of the same disease process. Initially, a stone is impacted in the neck of the gallbladder compressing the common hepatic duct, causing jaundice and repeated attacks of cholecystitis. Eventually the stone erodes its way into the common hepatic duct, creating a cholecystocholedochal fistula. Pathophysiology Mirizzi syndrome can be caused by either a single large stone or multiple small stones impacted in Hartmann’s pouch of the gallbladder or in the cystic duct. A long parallel cystic duct and/or a low insertion into the common bile duct
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seem to predispose to the development of this syndrome (11, 12). Recurrent cystic duct obstruction will lead to repeated attacks of cholecystitis and will render the gallbladder distended, thickened, and inflamed. If the inflamed gallbladder is in close proximity to the bile duct and fuses with it, then the inflammation and adhesions may further contribute to the obstruction of the bile duct. With time, the stone causes pressure necrosis and erodes into the common hepatic duct, producing a cholecystobiliary fistula. This hypothesis is supported by the fact that in most cases of cholecystocholedochal fistulas, the fistula is occupied by a large gallstone that is impacted at the opening (13). The incidence of Mirizzi syndrome in patients undergoing biliary surgery varies from 0.7% to 1.4% in general to 2.7% in high-risk populations such as Native Americans (14). The incidence of cholecystocholedochal fistulas is reported to be 1.1% (14). In the largest cohort of patients undergoing surgery for gallstone disease, 17,395, described by Csendes et al. (7), 219 (1.3%) had Mirizzi syndrome and/or cholecystobiliary fistulas. Clinical Manifestation Obstructive jaundice is the most common presentation; frequently, it is accompanied by pain and fever. Rarely, patients may be anicteric at presentation. There are no pathognomonic features in either the history or the physical exam (14). The syndrome usually occurs in the setting of longstanding biliary symptoms. A recent history of jaundice is frequently noted. Patients can also present in the setting of acute cholecystitis, acute cholangitis, or pancreatitis. Mirizzi syndrome can complicate gallstone disease in any patient. Csendes et al. noted an increased mean age (62 yr) in patients with type III and IV lesions. Approximately 50 –77% of all patients reported are women (7), reflecting the increased frequency of gallstone disease in this group. Hyperbilirubinemia is the most encountered laboratory abnormality. Elevated ALP and aminotransaminase levels are also common. Leukocytosis is frequently seen when acute cholecystitis, pancreatitis, or cholangitis is present. The differential diagnosis includes other causes of obstructive jaundice such as carcinoma of the gallbladder, cholangiocarcinoma, pancreatic cancer, metastatic disease, and sclerosing cholangitis (14). Diagnosis An accurate diagnosis has a great impact on the management and prevention of further complications. In many patients, making a preoperative diagnosis with certainty may be difficult (12). On ultrasonography, a contracted gallbladder with gallstones, associated with one large or multiple small impacted stones in the cystic duct, may be seen. A dilated intrahepatic biliary tree and common hepatic duct with a normal-sized common bile duct will further confirm the diagnosis. Regional inflammation and excess bowel gas may impair the
Figure 1. Mirizzi syndrome. ERCP showing extrinsic compression of common hepatic duct (arrow) with intrahepatic biliary dilation.
quality of the study (15). CT can identify similar findings. The presence of periductal inflammation can be misinterpreted as carcinoma of the gallbladder. CT may also reveal abrupt narrowing in the hepatic duct with no extrinsic mass. Some authors believe that CT does not provide any additional information beyond that obtained from ultrasonography (16, 17). However, CT may be helpful in excluding malignancy in the porta hepatis area or in the liver. ERCP is the procedure of choice to confirm the diagnosis and to determine whether a fistula is present. Mirizzi type I will present as extrinsic compression of the common hepatic duct by a distended gallbladder with dilation of the intrahepatic biliary tree (Fig. 1). ERCP is also essential in determining the presence of a fistula preoperatively. Percutaneous transhepatic cholangiography (PTC) can provide similar information. However, we believe that ERCP in expert hands is superior in this situation because it can identify a low-lying cystic duct that may be missed by PTC. In addition, PTC may not visualize the distal common bile duct because of obstruction of the common hepatic duct. Finally, as discussed below, ERCP offers the opportunity to exercise a variety of therapeutic options, including stone retrieval and stent placement (18, 19). Treatment The traditional treatment of Mirizzi syndrome has been surgical. Preoperative diagnosis is very important to avoid complications, because a normal-caliber bile duct may be
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ligated if mistaken for a dilated cystic duct that runs parallel to it. Laparoscopic surgery appears to carry a higher risk for this serious complication (18). The common surgical approach is usually an incision in the gallbladder fundus and removal of the impacted stone. If the surgeon notices a gush of bile this usually indicates a fistula, because the cystic duct is usually occluded. If no fistula is seen on intraoperative cholangiography, then a partial cholecystectomy is performed, leaving the neck of the gallbladder in place. Common bile duct exploration can be done intraoperatively if possible, or via postoperative ERCP with stone removal if needed. If a biliary fistula is present, then a cholecystocholedochoduodenostomy may be performed. Direct repair of the fistula over a T tube or by using the gallbladder wall as a flap may also be attempted (20). Recently, endoscopic therapy has been increasingly used in the evaluation and treatment of patients with Mirizzi syndrome. Whether used as primary therapy or as an adjunct to surgical treatment, ERCP is an attractive option and may be the only option for poor surgical candidates (21). Patients with associated cholangitis will benefit from preoperative biliary drainage. In general, endoscopic management includes both biliary drainage and stone removal. Drainage is usually of the common duct with or without gallbladder drainage. Endoscopic sphincterotomy is frequently performed for stone extraction and to facilitate other interventions. Standard stone removal techniques are usually used and include baskets, balloons, and mechanical and electrohydraulic lithotripsy (EHL). Extracorporeal lithotripsy can also be a helpful adjunct. Binmoeller et al. (22) described the use of EHL during ERCP in 14 patients with Mirizzi syndrome. EHL was delivered using retrograde cholangioscopy after failed standard endoscopic stone extraction. The use of a mother/ daughter scope system allowed the application of EHL under direct vision. After EHL, stone fragments were removed using a basket. In the 12 patients with single stones, complete clearance was obtained in one session. Two cases were of multiple stones and required more than one treatment session. In one case, asymptomatic leakage of contrast from cystic duct into the peritoneal cavity was noted after fragmentation of a 3.5-cm stone. Advances in the design of retrograde cholangioscopes (choledochoscopes) may make this option more attractive in the future. England and Martin (23) retrospectively identified 25 patients with cholangiographic diagnoses of Mirizzi’s syndrome. Sixteen had type I and nine had type II. Eleven patients had preoperative endoscopic treatment including sphincterotomy, stent placement, extracorporeal shock wave lithotripsy, and mechanical lithotripsy. No clinical improvement was noted in three patients. All patients underwent cholecystectomy (only one had laparoscopic cholecystectomy); two patients died from biliary causes. Thirteen patients were deemed nonsurgical candidates and underwent endoscopic treatment alone (six type I and seven type II). Com-
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plete stone clearance and stone removal was achieved in three patients using a variety of accessories including mechanical lithotripsy, dissolution therapy with methyl tertbutyl ether, extracorporeal shock wave lithotripsy, and common bile duct stents. Nine patients were treated with long term biliary stenting as stone clearance could not be achieved. Five patients in this group died, two from biliary causes. Complications occurred in four patients and included pneumonia, acute cholecystitis, liver abscess, and secondary biliary cirrhosis. This study demonstrates the role of endoscopic treatment not only as adjunct to surgery but also as primary therapy. In addition, ERCP was also helpful in identifying the fistula preoperatively in all type II patients (23).
GALLSTONE ILEUS Background Gallstone ileus was first described by Bartolin in 1654, as a disease of elderly and debilitated people. Bowel obstruction results from the impaction of one or more gallstones. In 1890, Courvoisier (24) described 131 cases with a surgical mortality rate approaching 50%. In 1896, Bouveret described a syndrome of gastric outlet obstruction caused by an impacted gallstone in the duodenal bulb after the migration of a large stone through a cholecysto- or choledochoduodenal fistula (25–27). Pathophysiology Gallstone ileus is frequently proceeded by an episode of acute cholecystitis. The resulting inflammation and adhesions facilitate the erosion of the offending gallstone through the gallbladder wall forming a cholecystoenteric fistula and allowing the passage of the gallstone. The fistula is most commonly encountered in the duodenum, although it can occur anywhere in the GI tract (colon, stomach, and small bowel). Rarely, the fistula can be iatrogenic after endoscopic sphincterotomy for choledocholethiasis (26, 27) or a surgical choledochoduodenostomy (28). Once in the small bowel, the stone can pass through or cause obstruction or, less commonly, is vomited up. The resulting obstruction is a true mechanical phenomenon— hence, the term ileus is a misnomer. The size of the stone and the presence of an abnormally narrowed bowel will decide whether or not the stone will get impacted. Most authors agree that stones less than 2–2.5 cm pass spontaneously through a normal GI tract. Stones larger than 5 cm are more likely to become impacted. The site of impaction can be anywhere in the GI tract. However, the terminal ileum and the ileocecal valve are the most common locations because of their relatively narrow lumen and potentially less active peristalsis (29, 30). Less common locations include the proximal ileum and jejunum, followed by the stomach and the duodenum (Bouveret syndrome) (Fig. 2), and, lastly, the colon (31–33).
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Figure 3. Gallstone ileus. CT scan showing a large gallstone (arrow) impacted in the ileum.
Figure 2. Bouveret syndrome. (A) CT of the abdomen showing a large gallstone in the duodenal bulb with dilated stomach. (B) Upper endoscopy on the same patient showing the gallstone obstructing the lumen in the bulb.
right upper quadrant pain may be absent in up to one third of patients. Jaundice is uncommon and found in only 15% of patients. Gallstone ileus is a disease of the elderly, with a mean age of 65–75 yr and a female predominance (3–16:1). It is responsible for 25% of all bowel obstruction in patients over the age of 65, compared to 1–3% when all age groups are considered (37). Because gallstone ileus is predominantly a geriatric disease, as many as 80 –90% of patients have concomitant medical illnesses; cardiovascular disease and diabetes mellitus are the most commonly encountered. Clinicians should always be aware of these associated conditions, as they may impact the management and prognosis. The physical examination is usually nonspecific. Signs of acute illness, dehydration, and intestinal obstruction are frequently seen. The exam may be completely normal between attacks.
Clinical Manifestations Symptoms are those of small bowel obstruction and include crampy abdominal pain and vomiting. Prolonged obstruction and bowel distention may inhibit motility, resulting in less cramps (34). The sine qua non of bowel obstruction is obstipation and inability to pass flatus; however, one needs to remember that with partial obstruction those symptoms may be absent (35). Symptoms can be intermittent, as the passing gallstone may lodge at various levels of the bowel (34). Incomplete bowel obstruction, also known as the “tumbling phenomenon,” is responsible for the waxing and waning of symptoms. This can cause a significant delay in making the diagnosis because the patient may not seek medical attention or the physician may be deceived by a benign exam (36). In general, patients present 4 – 8 days after the beginning of symptoms. Associated symptoms like jaundice and
Diagnosis Abdominal films are very important in establishing the diagnosis. The classic roentgenographic signs were first described by Rigler, Borman, and Noble in 1941 (38), and include intestinal obstruction, pneumobilia, aberrantly located gallstone, and change in location of the previously identified stone on serial exams. The most frequent findings are bowel obstruction, pneumobilia, and an abnormally located stone. The presence of any two of the first three findings is pathognomonic and occurs in 40 –50% of patients. Pneumobilia may be be the result of prior surgical or endoscopic biliary manipulations. Therefore, the clinical picture should be taken into consideration when evaluating this roentgenographic sign. In 1978, Balthazar and Schechter (39) suggested that the finding of two adjacent air fluid levels in the right upper quadrant is an additional helpful sign. The medial collection is in the duodenal bulb and the lateral in the gallbladder itself. One cohort showed
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that 24% of patients had this sign on admission (39). The next step is usually ultrasonography or CT. Ultrasonography may be helpful in making the diagnosis by detecting a fistula and the impacted stone; in addition, it will further confirm the presence of residual cholelithiasis or choledocholithiasis. CT can identify large stones, evidence of bowel obstruction, and the level of obstruction (Fig. 3). An upper GI study may help to further identify the biliary enteric fistula and the level of obstruction. Most of the published studies report a correct, preoperative diagnosis in only 50% of cases, with a delay of 3– 4.5 days between admission and surgical intervention. Frequently, the delay is because of the difficulty in establishing the diagnosis, or the need to medically stabilize the patient (40). Treatment The main goal of the treatment is prompt relief of intestinal obstruction. Optimizing the patient’s condition before intervention is essential. Great attention should be paid to fluid and electrolyte balance and to correction of other comorbid conditions, such as heart disease, diabetes, and infection. Surgical intervention is the treatment of choice for the majority of patients. The best surgical approach has been the source of great controversy and continues to stir a very active debate (40). The main question being asked is whether biliary surgery should be carried out at the same time as the relief of gut obstruction (one-stage surgery), performed later (two-stage surgery), or not done at all. Those who advocate one-stage surgery argue that the morbidity of continued biliary symptoms justifies this kind of approach, especially with improved diagnostic and preoperative resuscitation methods (41). However, a one-stage operation requires prolonged anesthesia and extensive tissue dissection in a frequently fragile patient. In addition, many series have shown that a one-stage procedure carries a higher mortality rate, although a statistically significant difference has not been shown (40). Another question is about the need for elective cholecystectomy. If the patient is symptomatic, then cholecystectomy would be indicated. In asymptomatic patients, some authors argue that no further treatment is needed (40). However, potential complications of gallstone disease in this setting include recurrence of gallstone ileus, cholecystitis, acute cholangitis, residual fistula that can lead to malabsorption and weight loss, and acute cholangitis, especially in cholecystocolonic fistulas. All of the above support the need for a cholecystectomy and fistula repair (42). Another factor is the observed increase in incidence of gallbladder cancer among patients with fistulas relative to those undergoing cholecystectomy without fistula. Bossart et al. (43) reported a 15% incidence of gallbladder cancer when a fistula is present, versus 0.8% of all patients having cholecystectomies. However, the argument has also been made that most biliary fistulas in this setting are well tolerated and will close
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spontaneously once the stone has passed and the cystic duct is patent (40). In general, a one-stage surgery is indicated when patients are carefully selected and aggressively resuscitated preoperatively; this will decrease the exposure to anesthesia and the hospital stay. When biliary surgery is not advisable during the initial operation it will be reasonable to plan for an elective biliary surgery at a later date. In some patients no further intervention is needed. The surgery most authorities recommend for relieving the bowel obstruction is enterolithotomy, where a longitudinal enterotomy incision is performed after the stone is identified and milked into a healthy area. The stone is then extracted and the enterotomy closed in a transverse fashion. If the stone is impacted and cannot be moved to a healthy area, then a segmental bowel resection is performed. A search for other stones in both the small and large bowel is mandatory to prevent reobstruction. Examination of the gallbladder and cholecystectoenteric fistula is important for excluding any evidence of leakage, forming abscess, impending gangrene, or additional stones (36, 43– 45). Extracorporeal shock wave lithotripsy has been used successfully in the treatment of gallstone ileus (46 – 49). This method has not been systematically studied such that one can make any general conclusions. However, factors including patient position, method of targeting the stone, number and power of discharges, and the type of shock wave generator used may play an important role in determining the success or failure of this method. In addition, air in the interposing bowel may make it difficult to visualize the stone by ultrasound and may prevent successful stone fragmentation (48). Endoscopic EHL has also been successful in the management of this condition when the stone is within reach of an endoscope (Fig. 2). Bourke et al. (50) describe a case where the EHL probe was placed through the biopsy channel of an upper endoscope and in close proximity to a stone lodged in the second part of the duodenum. Technical difficulties included difficulties in maintaining scope position and keeping the bowel lumen filled with saline for effective spark discharge. In addition, because these stones are much larger than the one usually encountered in the biliary tree, the need to increase the number and duration of shocks applied was also observed (50). Endoscopic mechanical lithotripsy is another option and involves crushing the stone with physical pressure applied using a mechanical lithotripsy basket. This method is usually used in combination with EHL, because large, hard, cholesterol stones may be difficult to capture and crush (51). Overall, whenever possible, endoscopic treatment should be attempted, especially in those elderly patients with associated medical problems (50). Unfortunately, in many patients the stone is beyond the reach of an endoscope, and if extracorporeal shock wave lithotripsy is not available, surgery may be the only option.
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Reprint requests and correspondence: Firas H. Al-Kawas, M.D., Division of Gastroenterology, Georgetown University Medical Center, 3800 Reservoir Road, Northwest, Washington, DC 20007. Received Aug. 10, 2001; accepted Aug. 27, 2001.
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26. Masson JW, Fraser A, Wolf B, et al. Bouveret’s syndrome: Gallstone ileus causing gastric outlet obstruction. Gastrointest Endosc 1998;47:104 –5. 27. Bedogni G, Contini S, Meinero M, et al. Pyloroduodenal obstruction due to a biliary stone (Bouveret’s syndrome) managed by endoscopic extraction. Gastrointest Endosc 1985;31:36 – 8. 28. Lancaster JF, Strong RW, McIntyre A, Kerlin P. Gallstone ileus complicating endoscopic sphincterotomy. Aust N Z Surg 1993;63:416 –7. 29. Oskam J, Heitbrink M, Eeftinck Schattenkerk M. Intermittent gallstone ileus following endoscopic biliary sphincterotomy. A case report. Acta Chir Belg 1993;92:43–5. 30. Foss HL, Summers JD. Intestinal obstruction from gallstones. Ann Surg 1942;115:721. 31. Van Hillo M, Van der Vliet JA, Wiggers T, et al. Gallstone obstruction of the intestine. An analysis of ten patients and a review of the literature. Surgery 1987;101:273– 6. 32. Wakefield EG, Vickers PM, Walters W. Cholecystoenteric fistulas. Surgery 1939;5:674 –7. 33. Heuman R, Sjodahl R, Wetterfors J. Gallstone ileus: An analysis of 20 patients. World J Surg 1980;4:595– 600. 34. Piedad OH, Wels PB. Spontanous internal biliary fistula, obstructive and nonobstructive types. Twenty year review of 55 cases. Ann Surg 1972;175:75– 80. 35. Turrange RH, Bergen PC. Intestinal obstruction and ileus. In: Feldman M, Scharschmidt BF, Sleisenger MH, eds. Sleisenger and Fordtran’s gastrointestinal and liver disease, 6th ed. Philadelphia: WB Saunders, 1998:1799 – 807. 36. Lennon GM, Browne R, Watson RG, O’Connor J. Gallstone ileus following cholecystectomy and side to side choledochoduodenostomy. Ir J Med Sci 1990;159:287– 8. 37. VanLandingham SB, Broders CW. Gallstone ileus. Surg Clin North Am 1982;62:241–7. 38. Rigler LG, Borman CN, Noble JF. Gallstone obstruction: Pathogenesis and roentgen manifestations. JAMA 1941;117:1753. 39. Balthazar EJ, Schechter LS. Air in gallbaldder: A frequent finding in gallstone ileus. Am J Roentgenol 1978;131:219 –22. 40. Calvien PA, Richon J, Burgan S, Rohner A. Gallstone ileus. Br J Surg 1990;77:737– 42. 41. Reisner R, Cohen J. Gallstone ileus: A review of 1001 reported cases. Am Surg 1994;60:441– 6. 42. Warshaw AL, Bartlett MK. Choice of operation for gallstone intestinal obstruction. Ann Surg 1966;164:1051–5. 43. Bossart PA, Patterson AH, Zinite HA. Carcinoma of the gallbladder. Am J Surg 1962;103:361– 4. 44. McLaughlin CW, Raines M. Obstruction of the alimentary tract from gallstones. Am J Surg 1951;81:424. 45. Balch FG. Gallstone ileus. N Engl J Med 1938;218:457– 62. 46. Meyenberger C, Michel C, Metzger U, et al. Gallstone ileus treated by extracorporeal shock-wave lithotripsy. Gastrointest Endosc 1996;43:508 –11. 47. Sackmann M, Holl J, Haerlin M, et al. Gallstone ileus successfully treated by shock-wave lithotripsy. Dig Dis Sci 1991; 36:1794 –5. 48. Holl J, Sackmann M, Hoffmman R, et al. Shock-wave therapy of gastric outlet syndrome caused by gallstone. Gastroenterology 1989;97:472– 4. 49. Dumonceau J-M, Delhaye M, Cremer M. Extracorporeal shock-wave lithotripsy for gallstone ileus. Gastrointest Endosc 1996;44:759. 50. Bourke MJ, Scheider DM, Haber GB. Electrohydraulic lithotripsy of a gallstone causing gallstone ileus. Gastrointest Endosc 1997;45:521–3. 51. Moriai T, Hasegawa T, Fuzita M, et al. Successful removal of massive intragastric gallstone by endoscopic electrohydraulic lithotripsy and mechanical lithotripsy. Am J Gastroenterol 1991;86:627–9.
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CLINICAL REVIEWS
Complications of Gallstone Disease: Mirizzi Syndrome, Cholecystocholedochal Fistula, and Gallstone Ileus Alaa Abou-Saif, M.D., and Firas H. Al-Kawas, M.D. Division of Gastroenterology, Georgetown University Medical Center, Washington, District of Columbia
ABSTRACT Gallstone is a common disease with a 10% prevalence in the United States and Western Europe. However, it is only symptomatic in 20 –30% of patients, with biliary pain “colic” being the most common symptom. Complications of asymptomatic gallstone disease are generally rare, with an incidence of ⬍1%/yr. The most common complications of gallstone disease are acute cholecystitis, acute pancreatitis, ascending cholangitis, and gangrenous gallbladder. Less frequent complications include Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. Mirizzi syndrome and cholecystocholedochal fistula are two manifestations of the same process that starts with impaction of a gallstone in the gallbladder neck that results in obstruction of the bile duct, causing jaundice. The gallstone may erode into the bile duct, causing cholecystocholedochal fistula. Gallstone ileus refers to small bowel obstruction resulting from the impaction of one or more gallstones after they have migrated through a cholecystoenteric fistula. An accurate diagnosis is essential to the management and prevention of further complications. A variety of imaging and endoscopic modalities are used to make the diagnosis once the condition is suspected clinically. Treatment should be tailored to each individual patient. Management choices include ERCP, lithotripsy (endoscopic or extracorporeal), and surgery. Prognosis is frequently related to early recognition, management of any comorbid conditions, and careful selection of treatment modalities. (Am J Gastroenterol 2002;97: 249 –254. © 2002 by Am. Coll. of Gastroenterology)
MIRIZZI SYNDROME Background and Classification In 1948 Doctor Pablo Mirizzi described a syndrome of hepatic duct obstruction—“sindrome del conducto hepatico”—in the setting of cholelethiasis and cholecystitis (1). This syndrome is caused by an impacted gallstone in the cystic duct or the neck of the gallbladder compressing the adjacent bile duct and resulting in complete or partial obstruction of the common hepatic duct. Mirizzi wrongly postulated that the inflammation resulted in a spasm of a physiological and anatomical sphincter comprised of circular muscle fibers within the common hepatic duct. Jaundice
and recurrent cholangitis are the two main clinical manifestations. Anatomically, a cystic duct parallel to the bile duct is one of the main features of this syndrome (2). It is now well known that there is no sphincter in the hepatic duct and that the jaundice is a result of the external compression caused by the impacted stone. Nevertheless, the syndrome has come to bear his name. It should be noted, however, that Kehr in 1905, Ruge in 1908, and Levrat and Chayvialle in 1941 reported external compression of the bile duct after stone impaction in the cystic duct (3, 4). In 1961, Mirizzi reported an additional seven cases of this syndrome encountered in 4000 patients with gallstone disease (5). In 1982, McSherry et al. (6) suggested a subclassification of Mirizzi syndrome into two types: type I involves the external compression of the common hepatic duct by a stone impacted in the cystic duct, and type II occurs when a cholecystocholedochal fistula caused by stone migration into the common hepatic duct is seen. Further modification was suggested by Csendes et al. (7). In this modification, type II is a cholecystocholedochal fistula that involves less than one third of the circumference of the bile duct, type III is a fistula involving up to two thirds of the duct circumference, and type IV is complete obstruction of the bile duct. Cholecystobiliary fistula is a rare entity. It was first reported by Puestow in 1942 (8) when he described a case of spontaneous internal biliary fistula. In 1950 and 1952 Behrend and Cullen and Mirizzi described a total of seven additional cases (9, 10). It is fair to say that Mirizzi syndrome and cholecystocholedochal fistulas are two stages of the same disease process. Initially, a stone is impacted in the neck of the gallbladder compressing the common hepatic duct, causing jaundice and repeated attacks of cholecystitis. Eventually the stone erodes its way into the common hepatic duct, creating a cholecystocholedochal fistula. Pathophysiology Mirizzi syndrome can be caused by either a single large stone or multiple small stones impacted in Hartmann’s pouch of the gallbladder or in the cystic duct. A long parallel cystic duct and/or a low insertion into the common bile duct
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seem to predispose to the development of this syndrome (11, 12). Recurrent cystic duct obstruction will lead to repeated attacks of cholecystitis and will render the gallbladder distended, thickened, and inflamed. If the inflamed gallbladder is in close proximity to the bile duct and fuses with it, then the inflammation and adhesions may further contribute to the obstruction of the bile duct. With time, the stone causes pressure necrosis and erodes into the common hepatic duct, producing a cholecystobiliary fistula. This hypothesis is supported by the fact that in most cases of cholecystocholedochal fistulas, the fistula is occupied by a large gallstone that is impacted at the opening (13). The incidence of Mirizzi syndrome in patients undergoing biliary surgery varies from 0.7% to 1.4% in general to 2.7% in high-risk populations such as Native Americans (14). The incidence of cholecystocholedochal fistulas is reported to be 1.1% (14). In the largest cohort of patients undergoing surgery for gallstone disease, 17,395, described by Csendes et al. (7), 219 (1.3%) had Mirizzi syndrome and/or cholecystobiliary fistulas. Clinical Manifestation Obstructive jaundice is the most common presentation; frequently, it is accompanied by pain and fever. Rarely, patients may be anicteric at presentation. There are no pathognomonic features in either the history or the physical exam (14). The syndrome usually occurs in the setting of longstanding biliary symptoms. A recent history of jaundice is frequently noted. Patients can also present in the setting of acute cholecystitis, acute cholangitis, or pancreatitis. Mirizzi syndrome can complicate gallstone disease in any patient. Csendes et al. noted an increased mean age (62 yr) in patients with type III and IV lesions. Approximately 50 –77% of all patients reported are women (7), reflecting the increased frequency of gallstone disease in this group. Hyperbilirubinemia is the most encountered laboratory abnormality. Elevated ALP and aminotransaminase levels are also common. Leukocytosis is frequently seen when acute cholecystitis, pancreatitis, or cholangitis is present. The differential diagnosis includes other causes of obstructive jaundice such as carcinoma of the gallbladder, cholangiocarcinoma, pancreatic cancer, metastatic disease, and sclerosing cholangitis (14). Diagnosis An accurate diagnosis has a great impact on the management and prevention of further complications. In many patients, making a preoperative diagnosis with certainty may be difficult (12). On ultrasonography, a contracted gallbladder with gallstones, associated with one large or multiple small impacted stones in the cystic duct, may be seen. A dilated intrahepatic biliary tree and common hepatic duct with a normal-sized common bile duct will further confirm the diagnosis. Regional inflammation and excess bowel gas may impair the
Figure 1. Mirizzi syndrome. ERCP showing extrinsic compression of common hepatic duct (arrow) with intrahepatic biliary dilation.
quality of the study (15). CT can identify similar findings. The presence of periductal inflammation can be misinterpreted as carcinoma of the gallbladder. CT may also reveal abrupt narrowing in the hepatic duct with no extrinsic mass. Some authors believe that CT does not provide any additional information beyond that obtained from ultrasonography (16, 17). However, CT may be helpful in excluding malignancy in the porta hepatis area or in the liver. ERCP is the procedure of choice to confirm the diagnosis and to determine whether a fistula is present. Mirizzi type I will present as extrinsic compression of the common hepatic duct by a distended gallbladder with dilation of the intrahepatic biliary tree (Fig. 1). ERCP is also essential in determining the presence of a fistula preoperatively. Percutaneous transhepatic cholangiography (PTC) can provide similar information. However, we believe that ERCP in expert hands is superior in this situation because it can identify a low-lying cystic duct that may be missed by PTC. In addition, PTC may not visualize the distal common bile duct because of obstruction of the common hepatic duct. Finally, as discussed below, ERCP offers the opportunity to exercise a variety of therapeutic options, including stone retrieval and stent placement (18, 19). Treatment The traditional treatment of Mirizzi syndrome has been surgical. Preoperative diagnosis is very important to avoid complications, because a normal-caliber bile duct may be
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ligated if mistaken for a dilated cystic duct that runs parallel to it. Laparoscopic surgery appears to carry a higher risk for this serious complication (18). The common surgical approach is usually an incision in the gallbladder fundus and removal of the impacted stone. If the surgeon notices a gush of bile this usually indicates a fistula, because the cystic duct is usually occluded. If no fistula is seen on intraoperative cholangiography, then a partial cholecystectomy is performed, leaving the neck of the gallbladder in place. Common bile duct exploration can be done intraoperatively if possible, or via postoperative ERCP with stone removal if needed. If a biliary fistula is present, then a cholecystocholedochoduodenostomy may be performed. Direct repair of the fistula over a T tube or by using the gallbladder wall as a flap may also be attempted (20). Recently, endoscopic therapy has been increasingly used in the evaluation and treatment of patients with Mirizzi syndrome. Whether used as primary therapy or as an adjunct to surgical treatment, ERCP is an attractive option and may be the only option for poor surgical candidates (21). Patients with associated cholangitis will benefit from preoperative biliary drainage. In general, endoscopic management includes both biliary drainage and stone removal. Drainage is usually of the common duct with or without gallbladder drainage. Endoscopic sphincterotomy is frequently performed for stone extraction and to facilitate other interventions. Standard stone removal techniques are usually used and include baskets, balloons, and mechanical and electrohydraulic lithotripsy (EHL). Extracorporeal lithotripsy can also be a helpful adjunct. Binmoeller et al. (22) described the use of EHL during ERCP in 14 patients with Mirizzi syndrome. EHL was delivered using retrograde cholangioscopy after failed standard endoscopic stone extraction. The use of a mother/ daughter scope system allowed the application of EHL under direct vision. After EHL, stone fragments were removed using a basket. In the 12 patients with single stones, complete clearance was obtained in one session. Two cases were of multiple stones and required more than one treatment session. In one case, asymptomatic leakage of contrast from cystic duct into the peritoneal cavity was noted after fragmentation of a 3.5-cm stone. Advances in the design of retrograde cholangioscopes (choledochoscopes) may make this option more attractive in the future. England and Martin (23) retrospectively identified 25 patients with cholangiographic diagnoses of Mirizzi’s syndrome. Sixteen had type I and nine had type II. Eleven patients had preoperative endoscopic treatment including sphincterotomy, stent placement, extracorporeal shock wave lithotripsy, and mechanical lithotripsy. No clinical improvement was noted in three patients. All patients underwent cholecystectomy (only one had laparoscopic cholecystectomy); two patients died from biliary causes. Thirteen patients were deemed nonsurgical candidates and underwent endoscopic treatment alone (six type I and seven type II). Com-
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plete stone clearance and stone removal was achieved in three patients using a variety of accessories including mechanical lithotripsy, dissolution therapy with methyl tertbutyl ether, extracorporeal shock wave lithotripsy, and common bile duct stents. Nine patients were treated with long term biliary stenting as stone clearance could not be achieved. Five patients in this group died, two from biliary causes. Complications occurred in four patients and included pneumonia, acute cholecystitis, liver abscess, and secondary biliary cirrhosis. This study demonstrates the role of endoscopic treatment not only as adjunct to surgery but also as primary therapy. In addition, ERCP was also helpful in identifying the fistula preoperatively in all type II patients (23).
GALLSTONE ILEUS Background Gallstone ileus was first described by Bartolin in 1654, as a disease of elderly and debilitated people. Bowel obstruction results from the impaction of one or more gallstones. In 1890, Courvoisier (24) described 131 cases with a surgical mortality rate approaching 50%. In 1896, Bouveret described a syndrome of gastric outlet obstruction caused by an impacted gallstone in the duodenal bulb after the migration of a large stone through a cholecysto- or choledochoduodenal fistula (25–27). Pathophysiology Gallstone ileus is frequently proceeded by an episode of acute cholecystitis. The resulting inflammation and adhesions facilitate the erosion of the offending gallstone through the gallbladder wall forming a cholecystoenteric fistula and allowing the passage of the gallstone. The fistula is most commonly encountered in the duodenum, although it can occur anywhere in the GI tract (colon, stomach, and small bowel). Rarely, the fistula can be iatrogenic after endoscopic sphincterotomy for choledocholethiasis (26, 27) or a surgical choledochoduodenostomy (28). Once in the small bowel, the stone can pass through or cause obstruction or, less commonly, is vomited up. The resulting obstruction is a true mechanical phenomenon— hence, the term ileus is a misnomer. The size of the stone and the presence of an abnormally narrowed bowel will decide whether or not the stone will get impacted. Most authors agree that stones less than 2–2.5 cm pass spontaneously through a normal GI tract. Stones larger than 5 cm are more likely to become impacted. The site of impaction can be anywhere in the GI tract. However, the terminal ileum and the ileocecal valve are the most common locations because of their relatively narrow lumen and potentially less active peristalsis (29, 30). Less common locations include the proximal ileum and jejunum, followed by the stomach and the duodenum (Bouveret syndrome) (Fig. 2), and, lastly, the colon (31–33).
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Figure 3. Gallstone ileus. CT scan showing a large gallstone (arrow) impacted in the ileum.
Figure 2. Bouveret syndrome. (A) CT of the abdomen showing a large gallstone in the duodenal bulb with dilated stomach. (B) Upper endoscopy on the same patient showing the gallstone obstructing the lumen in the bulb.
right upper quadrant pain may be absent in up to one third of patients. Jaundice is uncommon and found in only 15% of patients. Gallstone ileus is a disease of the elderly, with a mean age of 65–75 yr and a female predominance (3–16:1). It is responsible for 25% of all bowel obstruction in patients over the age of 65, compared to 1–3% when all age groups are considered (37). Because gallstone ileus is predominantly a geriatric disease, as many as 80 –90% of patients have concomitant medical illnesses; cardiovascular disease and diabetes mellitus are the most commonly encountered. Clinicians should always be aware of these associated conditions, as they may impact the management and prognosis. The physical examination is usually nonspecific. Signs of acute illness, dehydration, and intestinal obstruction are frequently seen. The exam may be completely normal between attacks.
Clinical Manifestations Symptoms are those of small bowel obstruction and include crampy abdominal pain and vomiting. Prolonged obstruction and bowel distention may inhibit motility, resulting in less cramps (34). The sine qua non of bowel obstruction is obstipation and inability to pass flatus; however, one needs to remember that with partial obstruction those symptoms may be absent (35). Symptoms can be intermittent, as the passing gallstone may lodge at various levels of the bowel (34). Incomplete bowel obstruction, also known as the “tumbling phenomenon,” is responsible for the waxing and waning of symptoms. This can cause a significant delay in making the diagnosis because the patient may not seek medical attention or the physician may be deceived by a benign exam (36). In general, patients present 4 – 8 days after the beginning of symptoms. Associated symptoms like jaundice and
Diagnosis Abdominal films are very important in establishing the diagnosis. The classic roentgenographic signs were first described by Rigler, Borman, and Noble in 1941 (38), and include intestinal obstruction, pneumobilia, aberrantly located gallstone, and change in location of the previously identified stone on serial exams. The most frequent findings are bowel obstruction, pneumobilia, and an abnormally located stone. The presence of any two of the first three findings is pathognomonic and occurs in 40 –50% of patients. Pneumobilia may be be the result of prior surgical or endoscopic biliary manipulations. Therefore, the clinical picture should be taken into consideration when evaluating this roentgenographic sign. In 1978, Balthazar and Schechter (39) suggested that the finding of two adjacent air fluid levels in the right upper quadrant is an additional helpful sign. The medial collection is in the duodenal bulb and the lateral in the gallbladder itself. One cohort showed
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that 24% of patients had this sign on admission (39). The next step is usually ultrasonography or CT. Ultrasonography may be helpful in making the diagnosis by detecting a fistula and the impacted stone; in addition, it will further confirm the presence of residual cholelithiasis or choledocholithiasis. CT can identify large stones, evidence of bowel obstruction, and the level of obstruction (Fig. 3). An upper GI study may help to further identify the biliary enteric fistula and the level of obstruction. Most of the published studies report a correct, preoperative diagnosis in only 50% of cases, with a delay of 3– 4.5 days between admission and surgical intervention. Frequently, the delay is because of the difficulty in establishing the diagnosis, or the need to medically stabilize the patient (40). Treatment The main goal of the treatment is prompt relief of intestinal obstruction. Optimizing the patient’s condition before intervention is essential. Great attention should be paid to fluid and electrolyte balance and to correction of other comorbid conditions, such as heart disease, diabetes, and infection. Surgical intervention is the treatment of choice for the majority of patients. The best surgical approach has been the source of great controversy and continues to stir a very active debate (40). The main question being asked is whether biliary surgery should be carried out at the same time as the relief of gut obstruction (one-stage surgery), performed later (two-stage surgery), or not done at all. Those who advocate one-stage surgery argue that the morbidity of continued biliary symptoms justifies this kind of approach, especially with improved diagnostic and preoperative resuscitation methods (41). However, a one-stage operation requires prolonged anesthesia and extensive tissue dissection in a frequently fragile patient. In addition, many series have shown that a one-stage procedure carries a higher mortality rate, although a statistically significant difference has not been shown (40). Another question is about the need for elective cholecystectomy. If the patient is symptomatic, then cholecystectomy would be indicated. In asymptomatic patients, some authors argue that no further treatment is needed (40). However, potential complications of gallstone disease in this setting include recurrence of gallstone ileus, cholecystitis, acute cholangitis, residual fistula that can lead to malabsorption and weight loss, and acute cholangitis, especially in cholecystocolonic fistulas. All of the above support the need for a cholecystectomy and fistula repair (42). Another factor is the observed increase in incidence of gallbladder cancer among patients with fistulas relative to those undergoing cholecystectomy without fistula. Bossart et al. (43) reported a 15% incidence of gallbladder cancer when a fistula is present, versus 0.8% of all patients having cholecystectomies. However, the argument has also been made that most biliary fistulas in this setting are well tolerated and will close
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spontaneously once the stone has passed and the cystic duct is patent (40). In general, a one-stage surgery is indicated when patients are carefully selected and aggressively resuscitated preoperatively; this will decrease the exposure to anesthesia and the hospital stay. When biliary surgery is not advisable during the initial operation it will be reasonable to plan for an elective biliary surgery at a later date. In some patients no further intervention is needed. The surgery most authorities recommend for relieving the bowel obstruction is enterolithotomy, where a longitudinal enterotomy incision is performed after the stone is identified and milked into a healthy area. The stone is then extracted and the enterotomy closed in a transverse fashion. If the stone is impacted and cannot be moved to a healthy area, then a segmental bowel resection is performed. A search for other stones in both the small and large bowel is mandatory to prevent reobstruction. Examination of the gallbladder and cholecystectoenteric fistula is important for excluding any evidence of leakage, forming abscess, impending gangrene, or additional stones (36, 43– 45). Extracorporeal shock wave lithotripsy has been used successfully in the treatment of gallstone ileus (46 – 49). This method has not been systematically studied such that one can make any general conclusions. However, factors including patient position, method of targeting the stone, number and power of discharges, and the type of shock wave generator used may play an important role in determining the success or failure of this method. In addition, air in the interposing bowel may make it difficult to visualize the stone by ultrasound and may prevent successful stone fragmentation (48). Endoscopic EHL has also been successful in the management of this condition when the stone is within reach of an endoscope (Fig. 2). Bourke et al. (50) describe a case where the EHL probe was placed through the biopsy channel of an upper endoscope and in close proximity to a stone lodged in the second part of the duodenum. Technical difficulties included difficulties in maintaining scope position and keeping the bowel lumen filled with saline for effective spark discharge. In addition, because these stones are much larger than the one usually encountered in the biliary tree, the need to increase the number and duration of shocks applied was also observed (50). Endoscopic mechanical lithotripsy is another option and involves crushing the stone with physical pressure applied using a mechanical lithotripsy basket. This method is usually used in combination with EHL, because large, hard, cholesterol stones may be difficult to capture and crush (51). Overall, whenever possible, endoscopic treatment should be attempted, especially in those elderly patients with associated medical problems (50). Unfortunately, in many patients the stone is beyond the reach of an endoscope, and if extracorporeal shock wave lithotripsy is not available, surgery may be the only option.
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Reprint requests and correspondence: Firas H. Al-Kawas, M.D., Division of Gastroenterology, Georgetown University Medical Center, 3800 Reservoir Road, Northwest, Washington, DC 20007. Received Aug. 10, 2001; accepted Aug. 27, 2001.
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