Br. J. Surg. 1989, Vol. 76, November, 11 39-1 143

A. Csendes, J. Carlos Diaz, P. Burdiles, F. Maluenda and 0. Nava

Department of Surgery, University of Chile, Santiago, Chile, South America Correspondence to: Dr A. Csendes, Department of Surgery, Hospital J. J. Aguirre, Santos Dumont 999, Santiago, Chile, South America

Mirizzi syndrome and cholecystobiliary fistula: a unifying classification

A new classification of patients with Mirizzi syndrome and cholecysto- biliary fistula is presented. Type 1 lesions are those with external compression of the common bile duct. In type 11 lesions a cholecysto- biliary fistula is present with erosion of less than one-third of the circumference of the bile duct. In type I l l lesions the fistula involves up to two-thirds of the duct circumference and in type IV lesions there is complete destruction of the bile duct. A total of 219 patients were identlfied with these lesions from 17 395 patients with benign biliary tract diseases undergoing surgery. The incidence of type I lesions was 11 per cent, type 11 41 percent, type I l l 44 percent and type 1V 4percent. The majority had obstructive jaundice. In type 1 lesions, cholecystectomy plus choledochostomy is effective. In type 1I lesions, suture of the fistula with absorbable material or choledochoplasty with the remnant of gallbladder can be performed. In type I11 lesions suture is not indicated and choledochoplasty is recommended. In type 1 V lesions, bilioenteric anastomosis is preferred. Operative mortality rate increases according to the severity of the lesion, as does postoperative morbidity. During cholecystectomy, partial resection is recommended in order to extract the stones, visualize the common bile duct and define the type and location of the fistula. T tubes should be placed distal to the fistula. Keywords: Cholecystobiliary fistula, Mirizzi syndrome, surgery

Mirizzi' originally described the syndrome that bears his name in 1948, when he found the following features in patients with gallstones: anatomical variation of a parallel cystic duct; gallstones impacted at the neck of the gallbladder or in the cystic duct; partial obstruction of the common bile duct secondary to compression by the stone or surrounding inflam- mation; recurrent cholangitis; and spasm of the circular muscular fibres in the hepatic duct. Since his description, several isolated cases have been reported2-' ' . Communication between the gallbladder or cystic duct and the common bile duct is rarely referred to in the surgical literature' 2-20. After dealing with a number of patients with Mirizzi syndrome and cholecysto- biliary fistula, we have come to realize that these are stages in the evolution of the same pathological condition.

The purpose of the present study is to define the incidence of the Mirizzi syndrome and cholecystobiliary fistula in a series of patients with gallstones, to propose a new unifying classifi- cation that includes both of these conditions, and to establish the surgical treatment for each stage of the pathological process.

Patients and methods The present paper is a combination ofa retrospective analysis (1976-82) and a prospective study (1983-87) of all patients operated on for gallstones in the Department of Surgery, University of Chile. A total of 17 395 patients were operated on during the entire period, so that approximately 1449 patients per year were submitted to cholecyst- ectomy. From this patient population, 219 patients (1.3 percent) were considered to have Mirizzi syndrome and/or a cholecystobiliary fistula (see below).

A special procotol was designed for the study, in which 60 different parameters were included. All data from each patient were documented on a chart and at the end of the study (July 1987), they were analysed by the IBM computer of the Faculty of Medicine (P240 IBM).

Pathological analysis Each resected gallbladder was carefully examined histologically to exclude carcinoma. All patients with benign strictures, stenosis of the

sphincter of Oddi, carcinoma of the common bile duct and biliary- digestive fistulae were excluded from consideration. Chronic cholecystitis corresponded to the presence of a gallbladder with a firm thick wall, and fibrosis. Histologically Aschoff-Rokitansky sinuses and mono- nuclear inflammatory cells were present. Acute cholecystitis corres- ponded to a gallbladder with fibrinous, shaggy, dull exudates with muscular engorgement with oedema and hyperaemia of the wall. Histologically the presence of polymorphonuclear leucocytes was observed.

Acute suppurative cholangitis was defined during surgery as the presence of muddy bile or pus in the common bile duct, instead of clear green bile.

Definitions For the present study, we have defined four evolving stages of the same disease and have classified them as follows (Figure I ) :

1 . Type I lesion: external compression of the common bile duct due to a stone impacted at the neck of the gallbladder or at the cystic duct ( i t . the original Mirizzi syndrome) 2. Type I 1 lesion: presence of a cholecystobiliary fistula (cholecysto- hepatic or cholecystocholedochal) due to erosion of the anterior or lateral wall of the common bile duct by impacted stones, the fistula involving less than one-third of the circumference of the common bile duct 3. Type Ill lesion: presence of a cholecystobiliary fistula with erosion of the wall of the common bile duct that involves up to two-thirds of its circumference 4. Type IV lesion: presence of a cholecystobiliary fistula with complete destruction of the entire wall of the common bile duct.

To determine the late results of treatment, a special questionnaire was completed with reference to pain, jaundice, loss of weight, talerance to food, and whether the patient was content with the surgical result. A total of 167 (76.3 per cent) of cases were followed in this way with a mean follow-up of 5.7 years (range 1-13 years).

For statistical evaluation, analysis ofvariance was used and P(0.05 was considered as statistically significant.

Results Among the 219 patients, 23 (10-5 per cent) had a type I lesion, 90 (41.1 per cent) a type I1 lesion, 97 (44.3 per cent) a type 111

~ ~ ~ ~~ ~~

aOo7-1323/89/1139 05$3 00 0 1989 Butterworth & Co (Publ~shers) Ltd 1139

Mirizzi syndrome and cholecystobiliary fistula: A. Csendes et al.

Chronic T y p e 1 T y p e I1 T y p e I11 T y p e IV cholecy s t i t i s Ex te rna l < ,

compression o f common C holecystobi I i a r y f is tu la b i le d u c t (Mir izz i syndrome)

Figure 1 Stages in the development of the Mirizzi syndrome and cholecystobiliary fistula

Table 1 Clinical findings on admission in 219 patients with external compression of the common bile duct or cholecystobiliary fistula

Classification

Clinical findings Type I Type I1 Type 111 Type IV (n = 23) (n = 90) (n = 97) (n=9)

Mean age (years) Female (%) Duration of symptoms > 8 days (YO) Biliary pain (%) Jaundice on admission (YO) Fever present for previous 5 days (%) Rectal temperature > 38°C (YO)

44 65 43

100 83 70 35

42 77 43 94 84 57 33

53 77 39 97 92 67 53

62 100 55

100 67 67 34

Figure 2 Surgical alternatives for the repair of the common bile duct in patients with type I1 lesions. Option A involves the simple suture, and in option B the cuff of gallbladder is used for choledochoplasty

lesion and 9 (4.1 per cent) a type IV lesion. The main clinical findings in all cases are shown in Table 1. There was an increasing mean age in patients with type 111 and IV lesions. Almost all cases with type I, I1 and III lesions had a typical clinical picture of obstructive jaundice, but this was present in only six of the nine cases with type IV lesions.

Previous oral cholecystography had been performed in 37 patients, showing no gallbladder filling in any patient. Ultra- sonography was performed in 67 cases, demonstrating gallstones in all and a dilated proximal common bile duct in 54 of the cases. A type I lesion was suspected in six cases and a biliobiliary fistula in 12 patients.

The main surgical findings in the gallbladder in the 219 cases are demonstrated in Table 2. Acute cholecystitis was more

frequent in type I lesions, with multiple gallstones in approximately two-thirds of the cases. Chronic cholecystitis was frequently observed in patients with type 11,111 and IV lesions. The cystic duct was absent in all patients with type IV lesions and in two-thirds of patients with type I1 and I11 lesions. Stones were absent from the common bile duct in 92 per cent of cases with type I lesions and from 56 per cent of those with type IV lesions. In some patients with type 11, 111, and IV lesions, intrahepatic stones were also present. Acute suppurative cholangitis was more common in patients with type IV lesions.

Cholecystectomy was performed in all 219 patients, but was frequently partial or incomplete in patients with type I1 and 111 lesions, when a remnant of gallbladder was left in order to perform a choledochoplasty (Table 3). The surgical treatment in patients with type I1 lesions was most often suture closure (Figure 2) of the fistula (chromic catcut 4-0 or Vicryl" (Ethicon Ltd., Edinburgh, UK) 3-0) leaving a T tube distal to thc closure for at least 2 months. In a quarter of type I1 patients choledocho- plasty was performed, especially when the fistula was large. The most frequent method of repair of the fistula in type 111 lesions was also choledochoplasty using the cuff remnant of the gallbladder. The surgical treatment of patients with type IV lesions consisted of an end-to-end repair in five cases, a biliary enteric anastomosis in three, and a double hepaticostomy in one case.

The size of the cholecystobiliary fistula is shown in Table 4 . Most patients with type I1 lesions had a fistula < lOmm in diameter while, in patients with type 111 and IV lesions, the fistula was often larger.

Postoperative course is shown in Table 5 . There was an increasing mortality rate, which was significantly greater when patients with type 111 and IV lesions were compared with other groups ( P <0.02). The late results in the 204 patients discharged from the hospital are shown in Table 6. Two patients with type

1140 Br. J. Surg., Vol. 76, No. 11, November 1989

Mirizzi syndrome and cholecystobiliary fistula: A. Csendes et al.

Table 2 Surgical findings at operation in patients with external compression of the bile duct or cholecystobiliary fistula

Classification

Findings Type 1 (n = 23)

Type I1 (n = 9 0 )

Type I11 (n = 97)

Type IV ( n = 9 )

Histology Chronic cholecystitis Acute cholecystitis

Absent Solitary Multiple

Absent <5mm >6mm

Number of stones

Diameter of cystic duct

Diameter of common bile duct <12mm 13-20 mm >21 mm

Common bile duct stones Absent Present

Acute suppurative cholangitis

9 14

0 7 16

4 16 3

16 7 0

21 2

4

54 36

18 17 55

56 22 12

26 50 14

20 70 32

65 32

10 29 58

68 12 17

28 56 13

37 60

50

7 2

1 3 5

9 0 0

2 4 3

5 4

6

Table 3 Surgical procedures in patients with external compression of the common bile duct or cholecystobiliary fistula

Classification

Operation Type I (n = 23)

Type I1 Type I11 Type IV ( n = 9 0 ) (n = 9 7 ) ( n = 9 )

Biliary tract Cholecystectomy Cholecystectomy plus choledochostomy

Fistula management Suture Choledochoplasty with remnant of gallbladder End-to-end anastomosis Bilioenteric anastomosis Hepaticostomy

0 90

66 24 0 0 0

8 89

5 83

1 8 0

Table4 Size of the cholecystobiliaryfistula at operation in 196 patients

Classification

Type 11 Type I11 Type IV Size of fistula (mm) ( n = 9 0 ) (n = 9 7 ) ( n = 9 )

< 10 1 1-20 21-30 >31

60 21 0 29 51 1

1 14 2 0 11 6

I lesions developed a benign stricture 3 and 4 months after surgery, and underwent hepaticojejunostomy with good results. In patients with type I1 and 111 lesions, 4 per cent developed a benign stricture within a mean interval of 12 months after surgery. All patients with type IV lesions, as well as the patient with a type 111 lesion in whom end-to-end repair was performed, developed a benign stricture 4 months after surgery; hepatico- jejunostomy was performed in all cases with a good clinical outcome to date.

Discussion The Mirizzi syndrome as originally described consisted of the combination of a stone impacted at the neck of the gallbladder or cystic duct and a functional disorder of a putative sphincter

within the common hepatic duct, but without any lesion of the common bile duct'. Mirizzi postulated that a number of factors might trigger or predispose to contraction of this 'sphincter', such as inflammation, aberrant vessels or stones impacted in the cystic duct. External obstruction of the bile duct following impaction of a stone in the cystic duct with associated inflammation was described by Kehr in 1905 and by Ruge in 1908 as a rare form of obstructive j a ~ n d i c e ' ~ . It is now well known that there is no sphincter within the hepatic duct, and the jaundice seen in these patients is due to external compression secondary to impaction of a stone at the neck of the gallbladder or cystic duct. Mirizzi described seven cases with this syndrome drawn from 4000 patients operated on for gallstone disease". Dietrich, cited in References 2, 10 and 11, reported in 1963 that a parallel cystic duct was present in 18 per cent of patients with gallstones studied by operative cholangiography. However, this was observed in only 1.8 per cent of 5800 patients studied by Puente" in our country.

Treatment of the Mirizzi syndrome is based only on the removal of the initiating factors, namely the inflamed gall- bladder, cystic duct and impacted stone. The common bile duct should be visualized and explored, if necessary, to exclude other causes of obstructive jaundice. Most inflammatory strictures will return to normal when the inflammatory process resolves, as occurred in 16 of our 18 followed cases with type I lesions. T-tube insertion will ensure biliary flow and decom- pression during the healing process. We agree with Lubbers'

Br. J. Surg., Vol. 76, No. 11, November 1989 1141

Mirizzi syndrome and cholecystobiliary fistula: A. Csendes et al

Table 5 Postoperative course in patients with external compression of the common bile duct or cholecystobiliary fistula

Outcome

Postoperative morbidity Haemoperitoneum Bile peritonitis External biliary fistula Subphrenic abscess

~ _ _ ~ _ _ _ _

Operative mortality within 30 days of operation

Residual stones

Classification

Type I Type I1 Type I11 Type IV (n = 23) (n = 90) ( n = 97) ( n = 9 )

3 (13) 0 1 2 0

0

2

13 (13) 4 2 0 1 1 9 3 1 0

12 (12) I

3 0

Figures in parentheses are percentages

Table 6 Late results at follow-up of patients with external compression of the common bile duct or cholecystobiliary fistula

Classification

Follow-up status Type I Type I1 Type 111 Type IV Total (n = 23) (n = 88) ( n = 85) ( n = 8 ) (n = 204)

Asymptomatic 16

0 Benign stricture 2 Died owing to biliary disease Lost to follow-up 5

62 3 3

20

54 4 1

26

135 (66) 12 (6) 5 (2)

52 (25)

Figures in parentheses are percentages

that the term Mirizzi syndrome could now be abandoned, as it is only the first stage of a more complex process, as has been shown in our study.

Cholecystobiliary fistula is a rare entity. Puestow" described it for the first time in 1942, when he published details of one case in 16 patients with spontaneous internal biliary fistulae. Later, Behrend and C ~ l l e n ' ~ described it as a separate entity in three patients with cholecystocholedochal fistula. Mirizzi" described four cases in 1954 and others have published descriptions s u b ~ e q u e n t l y ~ ~ , ~ ~ . Some authors in our country have described this type of biliary fistula in 0.14.5 per cent of all patients submitted to surgery for gallstones".12. Corlette and Bismuth" have presented 24 cases treated in a 15-year period and propose a classification based on whether patients have a cystic duct. In our experience the majority of patients did not have a cystic duct and so this classification has little use in surgical practice.

It is important to establish the diagnosis of fistula before operation, in order to plan the surgical procedure. In our prospective study, which began in 1983, ultrasound proved very useful in this regard and we doubt whether CT scanning will be of more value. Preoperative cholangiography is valuable and we have used mainly the percutaneous transhepatic route because endoscopic retrograde cholangiopancreatography (ERCP) has become available only recently. However, in addition to preoperative diagnosis, ERCP also allows drainage to relieve cholangitis and may well improve the eventual surgical results2 5-27 .

We consider that the so-called Mirizzi syndrome and cholecystobiliary fistula are different evolving stages of the same disease process. In patients with stones impacted at the neck of the gallbladder or cystic duct, repeated attacks of acute inflammation produce first distension of the gallbladder and then inflammation and thickening of the involved structures in the area. The distended and inflamed gallbladder comes to lie in close proximity to the common hepatic or common bile duct, and adhesion between the walls of the two structures with compression of the bile duct may produce obstructive jaundice and the type I lesion. The impacted stone may then cause pressure necrosis and further acute inflammation. In time the stone may erode through into the adjacent bile duct, producing the type I1 lesion or cholecystobiliary fistula. The cystic duct

becomes obliterated and disappears in the majority of cases, and this fact may create an even larger fistula, with destruction of the anterior wall of the bile duct (type 111 lesion). As time passes, the stone can slowly migrate into the bile duct, producing a complete necrosis of the duct (type IV lesion). Other internal biliary fistula (cholecystoduodenal, cholecysto- colonic or cholecystogastric) have entirely the same mechanism of formation, but they do not form type I lesions as they communicate with organs with a greater lumen than the bile duct.

The practical importance of our new classification is that we propose different procedures according to the stage reached. At operation, it is always important to proceed with great care. Palpation of the impacted stone can give the impression of the presence of external compression of the common bile duct. We prefer to open the gallbladder, extract the stone(s) and visualize the interior of the gallbladder or the common bile duct. An operative cholangiogram should be performed to confirm the diagnosis, determine the location and size of any fistula, detect ductal stones and establish whether there is loss of integrity of the bile duct wall. In none of our cases was a mural defect created accidentally by over-enthusiastic cholecystectomy, a procedure that can produce a large fistula if care is not taken. Partial cholecystectomy helps to define more precisely the extension of any d e f e ~ t ~ ~ - ~ ~ . If the fistula is small and has eroded less than one-third of the circumference of the common bile duct, the defect can be sutured with fine absorbable sutures and a T tube placed distal to the fistula for 1 or 2 months. If the defect is larger, a cuff of gallbladder is used for fistula closure and a T tube is placed distally. We try never to place the T tube through the fistula, because leakage of bile occurs readily and a benign stricture can appear late after surgery. In some patients who present with stricture of the common bile duct due to this fistula, immediate hepaticojejunostomy may give good late results. End-to-end bile duct anastomosis is inadvis- able; all of our six patients in whom we performed this procedure developed a benign stricture early after operation, necessitating hepaticojejunostomy. We therefore insist on preserving a cuff of gallbladder on the bile duct so as to use it in reconstruction. The greatest challenge is posed by the type IV lesions. Complete section of the common hepatic duct may be present and in one of our patients the lesion involved both

1142 Br. J. Surg., Vol. 76, No. 11, November 1989

Mirizzi syndrome and cholecystobiliary fistula: A. Csendes et al.

right and left hepatic ducts. In such cases, we suggest immediate bilioenteric anastomosis or hepaticostomy, leaving a stent or a double T tube in place for a long period. It has been shown in animal experiments as well as in patients3’ that autorecon- struction of the common hepatic duct can occur if a viable longitudinal string of duct is left in continuity. However, we must stress that our follow-up may be too short to assess such cases and that benign stricture can lead insidiously to secondary biliary cirrhosis.

References I .

2. 3.

4.

5.

6.

7.

8.

9.

10.

11.

12.

Mirizzi PL. Sidrome del conducto hepatico. J Int Cir 1984; 88: 737-71. Lubbers EJ. Mirizzi’s syndrome. World J Surg 1983; I : 78&5. Htoo MM. Surgical implications of stone impaction in the gallbladder neck with compression of the common hepatic duct (Mirizzi’s syndrome). Clin Radiol 1983; 34: 651-5. Montefusco P , Spier N, Geiss AC. Another facet of Mirizzi’s syndrome. Arch Surg 1983; 118: 1221-3. Starling JR, Matallana RM. Benign mechanical obstruction of the common hepatic duct (Mirizzi’s syndrome). Surgery 1980; 88: 73740. Balthazar EJ. The Mirizzi’s syndrome, inflammation of the common hepatic duct. Am J Gastroenterol 1975; 64: 144-8. Koehler RE, Melson GL, Lee JKT, Long J. Common hepatic duct obstruction by cystic duct stone: Mirizzi’s syndrome. A J R

Witte CL. Choledochal obstruction by cystic duct stone: Mirizzi’s syndrome. Am Surg 1984; 50: 241-3. Louagie Y, Pringot J, Haot J, Kestens PJ. Un cas de sindrome de Mirizzi. Acta Gastroenterol Belg 1982; 45: 394400. Dewbury KL. The features of the Mirizzi’s syndrome on ultrasound examination. Br J Radiol 1979; 52: 99G2. Bannura G. Fistula biliobiliar y sindrome de Mirizzi. Reo Chil Cir 1984; 36: 223-31. Vargas R, Correa E. Fistulas biliares internas. Rea MCd Chi!

1979; 132: 1007-9.

1954; 15: 127-33.

13.

14.

15.

16.

17.

18.

19.

20.

21. 22.

23.

24.

25.

26.

27.

28.

29.

30.

Zabaleta DE, Collarini HA, Senatore CM. Fistulas biliobiliares. M i d Arg 1957; 44: 1425-34. Behrend A, Cullen ML. Cholecysto-choledochal fistula: an unusual internal biliary fistula. Am Surg 1950; 132: 297-303. Cinelli AP. Fistulas biliobiliares espontaneas. Prens MCd Arg 1952; 39: 98&7. Mirizzi PL. Les fistules biliobiliares internes spontanees. J Chir

Patt HH, Koontz AR. Cholecysto-choledochal fistula: a report of two cases. Ann Surg 1951 ; 134: 1064-5. Puestow CB. Spontaneous internal biliary fistula. Ann Surg 1942; 115: 1043-54. Corlette MB, Bismuth H. Biliobiliary fistula: a trap in the surgery of cholelithiasis. Arch Surg 1975; 110: 377-85. Corund F. Mirizzi’s syndrome and biliobiliary fistulas: roent- genologic appearance. Gastrointest Radiol 1981 ; 6: 265-8. Mirizzi PL. Fistulae bilio-biliarie. Mem Acad Chir 1961; 87: 84&5. Puente J. Variaciones del conducto cistico. Rev Chil Cir 1985;

Mallet-Guy P , Roget C, Rodi R. Les fistules cholecysto- chodociennes. Lyon Clin 1960; 56: 23145. Kourias B, Tsopes E. Fistules abiliares internes spontanees d’origine lithiasique. J Chir (Paris) 1959; 75: 353-74. Cotton PB. Endoscopic management of bile duct stones (apples and oranges). Gut 1984; 25: 587-97. Classen M, Hagen Muller F. Biliary drainage. Endoscopy 1983; 15: 221-9. Siege1 JH, Yatto RP. Biliary endoprostheses for the management of retained bile duct stones. Am J Gastroenterol1984; 79: 50-4. Rutledge RH. Methods of repair of non-circumferential bile duct defects. Surgery 1983; 93: 33342. Sandblom P, Tabrizinn M, Rigo M, Fluckinger A. Repair of common bile duct defect using the gallbladder or cystic duct or pedicled graft. Surg Gynecol Obstet 1975; 140: 425-30. Michie W, Gunn A. Bile duct injuries: a new suggestion for their repair. Br J Surg 1964; 51: 96-100.

1952; 68: 23-8.

31: 169-16.

Paper accepted 15 March 1989

Br. J. Surg., Vol. 76, No. 11, November 1989 1143