Mirizzi syndrome: classification, diagnosis, treatment

The name "Mirizzi syndrome" is associated with the name of the surgeon from Argentina Mirizi, who is the author of many works related to the physiology of bile secretion, as well as clinical practice in the field of intraoperative choleography.

In 1948, in his scientific work, the doctor described the radiographic semiotics of the hepatic syndrome, the bright signs of which were stasis of bile and contracture of the bile duct. A picture of the calculous syndrome was also presented, which manifests itself in the appearance of a fistula between the gallbladder and hepatitis choledoch.

Existing Contradictions

Not everyone knows such an ailment as Mirizi's syndrome. What is it, we will tell below. But first it is worth noting that until now in medicine the concept itself is not completely determined. So, many doctors believe that the basis of the disease is the narrowing of the lumen in the liver duct. The most detailed formulation of the syndrome assumes the identification of pathology with stenosis of the liver duct or the area of the Hartman's pocket of the calculus, which is accompanied by an inflammatory process in the gallbladder and manifests itself in cholangitis or jaundice.

Mirzizi's syndrome, the classification of which is presented in this article, according to many scientists, is characterized not only by the narrowing of the lumen. The pathological process covers the right lobar and common bile duct.

Absolutely different interpretations of the syndrome of scientists who believe that the basis of the disease is vesicular choledochial fistula. There are also opposing views on the location of the pathological process. Some scientific articles mention the location of the connections between the gallbladder and the common duct of the liver, and in other research works examples of the presence of fistulas between the gall bladder and choledoch are cited.

For example, in the classification of MV. Corlette, H. Bismuth (1975) present bilio-biliary fistulas of two types depending on the localization of pathological anastomosis (above or below the main junction of the bile duct and cystic duct).

Some researchers attribute the manifestation of the disease to a narrowing of the lumen of hepatitis choledoch and the formation of a cholecystocholedochecal fistula.

A diverse interpretation of the essence of the syndrome makes it difficult to perceive its essence and makes it more difficult to find effective methods for its treatment.

Recently, it is often possible to meet a new interpretation of such a pathology as Mirizzi syndrome? Its types are represented by two variants of the course of the disease:

• An acute form, in which the lumen of the hepatitis choledocha is narrowed;
• A chronic form, which provokes the appearance of a fistula between the hepatitis choledoch and the lumen of the gallbladder.

Classic description

The classic description of such a pathological process as Mirizi's syndrome, the photo of which is presented in this article, includes four main points:

• Close parallel position of the duct of the gallbladder and the main duct of the liver;
• The presence of a stone in the duct of the gallbladder or in its neck;
• Obstructive process of the liver duct, which is caused by a fixed concrement in the duct of the gallbladder and the inflammatory process around it;
• Presence of jaundice with cholangitis or without it.

Principles of classification

What kinds of illnesses are divided into such a syndrome as Mirizzi's syndrome? Classification assumes the level of destruction of the wall of the main duct of the liver by the vesicle-choledochal fistula (Csendes):

• I type - compression of the common duct of the liver with a stone of the neck of the gallbladder or its duct.
• Type II - the presence of a vesicle-holedococcal fistula, occupying less than 1/3 of the circumference of the total duct of the liver;
• III type - the presence of a vesicle-choleadocneal fistula, which occupies 2/3 of the circumference of the liver duct;
• IV type - the presence of a vesicle-holedocheal fistula, which occupies the circumference of the liver duct completely, while the duct wall is subjected to complete destruction.

Causes of the syndrome

The main reasons for the development of such a disease as Mirizi's syndrome are:

• Compression of the lumen of the bile duct from the outside, provoked by acute calculous cholecystitis;
• Presence of stricture of the bile duct, which is located outside the liver region;
• Formation of perforation of hepatitis choledochia in the presence of stricture;
• The development of a vesicouleteral fistula with parallel elimination of stricture.

Depending on the structure of the biliary ducts, the size and weight of the stones, as well as the methods of therapy, the process may stop at any of the above stages, but the transformation from slight compression of the bile duct into the vesicoureteral fistula can be observed only in cholelithiasis.

Compression of the bile duct degenerates into stricture, if surgical intervention is postponed, and the disease acquires a chronic form, in which the period of remission is replaced by exacerbation. At the end of the time, the walls of the gallbladder and hepatiko choledocha begin to touch, which is provoked by a large stone in the Hartmann pocket. Under the pressure of his weight, the condition of trophism worsens, there is a decubitus of the wall of the gallbladder and duct. Then, a vesicoureteral fistula is formed.

Through such a pathological message from the gall bladder to the lumen of the hepatitis choledocha get the stones. The fistula increases in diameter by reducing its tissue in the area of compression. As a result, the narrowing of the proximal part of the hepaticocholedochus is eliminated, the gallbladder decreases in size, its neck, Hartman's pocket and most of the body disappear. As a result, the gallbladder becomes like a diverticuloid-like formation, which has a communication with the lumen of the extrahepatic bile duct with the help of a wide anastomosis. As a rule, the bladder duct is absent.

Symptomatics

How is Mirizzi syndrome manifested? Symptoms are characteristic for cholecystitis, occurring in acute or chronic form with the development of the mechanical form of jaundice. The overwhelming number of patients in the history of the disease noted additionally existing cholelithiasis with frequent attacks, followed by periods of mechanical form of jaundice. About scientific data, the most vivid and often manifested symptoms are pain sensations in the upper right abdominal region. Pain and jaundice disturb in 60-100% of cases.

More often jaundice appears in the presence of a vesicle-choleadocneal fistula.
With cholangitis, fever is noted. Sometimes the pain in the hypochondrium, intoxication, development of pancreatitis (acts as a layering on the common disease) disturb. In the blood, the bilirubin, ALT, AST and alkaline phosphatase levels increase.

Who is more likely to be found?

Mirizzi syndrome occurs in 0.1% of patients with cholelithiasis. In operative intervention, 0.7-2.5% of patients are noted. Both men and women of all races and nations are affected. In old age, the ailment is much more common.

Methods of diagnosis

What is the complexity of treating such a pathology as Mirizzi syndrome? Diagnosis and surgical tactics are not fully determined.

In modern medicine there are no generally accepted rules for conducting diagnostic procedures. Despite the progress in the medical imaging of various diseases, a diagnosis before the operation is established with difficulty. This is possible in about 20% of cases. Only single researchers note that the ultrasound examination of the disease before surgery reaches an accurate value in 67.1% of cases, MRI - in 94.4%, intraprostatic echography - in 97% and endoscopic retrograde pancreatocholangiography - in 100%.

All this suggests that modern methods of instrumental diagnosis do not always make it possible to identify the Mirrizi syndrome in the period preceding the operation.

Most often, with ultrasound examination, the following symptoms occur:

• Expansion of the duct inside the liver, as well as its proximal part located in parallel with the unexpanded common duct of the gallbladder;
• The presence of the gallbladder in a shrunken state.

CT scans of Mirizzi's syndrome coincide with the signs revealed in ultrasound diagnosis. Although CT can not provide important information supplementing the ultrasound method, its role in determining the presence of a malignant tumor in the proximal part of the ducts of the gallbladder is quite high, which is of great importance in the differentiation of Mirrizi's syndrome with the presence of cancer.

Magnetic resonance imaging, retrograde endoscopy and pancreato cholangiography (ERPHG) are equivalent diagnostic methods for the detection of stricture elements and cholecystocholedochelial fistula. The received pictures in T1 and T2 mode allow to distinguish inflammatory process from oncology with greater accuracy, which is not always possible under CT and ultrasound. However, due to the high price for MRI examination, this diagnostic method is not used in all medical centers.

Magnetic resonance cholangiopancreatography is a relatively new, but little-studied type of diagnosis. A number of scientists consider it to be the most promising species for referring to a pathology such as Mirrizi's syndrome.

Some scientists in their works note the advantages of laparoscopic ultrasound of the pancreatoduodenal region. When surgical intervention for suspected SM, this diagnostic method makes it possible in real time to build an image of the bile ducts in several planes of the fetus with different angles. However, at present this method remains inaccessible and unexplored until the end.

It should be noted that despite the availability of various diagnostic methods, it is extremely difficult to establish the presence of SM, which can deceive the surgeon and provoke damage to the choledochus, which is mistakenly perceived as a gall bladder or a wide duct. The absence of common diagnostic methods before surgery pushes to the development of optimal methods.

Principles of treatment

How is Mirizi's syndrome eliminated? Treatment involves two main directions: X-ray endoscopy and surgical intervention.

X-ray endoscopic treatment can be used as an initial stage before surgery in preparation for surgery. He acts as an independent method of therapy for patients with the presence of SM in the case of a high level of anesthesia risk.

Many investigators attribute the shortcomings of the REW to:

• Radiation burden on patients and medical staff;
• High price for endoscopic and radiological examination;
• The impossibility of eliminating the narrowing of the lumen of the proximal choledochus.

According to the scientific literature, the methods of operative intervention are very different. There are various options for surgical operations with CM.
Some doctors believe that the treatment of Mirrizi's syndrome by the method of laparoscopy is absolutely contraindicated.

A more common form of surgery for the first type of syndrome is cholecystectomy, which is supplemented by drainage of choledochus.

How does it stop in the presence of a biliary fistula? Mirizzi syndrome? The situational task requires separation and subsequent restoration of the integrity of choledoch. As one of the ways to close the choledoch, which is used by most surgeons, is to remove the defect of its wall through the left part of the gallbladder. However, some doctors believe that abandoned tissue may increase the risk of residual residual choledocholithiasis.

In the presence of cholecystobiliary fistula, it is recommended to perform choledocha plasty on temporary stents. Many scientists explain the necessity of this type of operation by the presence of long-term changes in the inflammatory nature in the hepatoduodenal ligamentous area, so that SM can be perceived as a model for damaging the walls of the bile ducts. With their considerable deformation, the walls of the bile duct are included in the fistula.
During surgical intervention, there is a high level of risk of complications.

Possible complications

Most often after surgery, there is such a complication, as the stricture of choledoch. According to the Russian researcher GI Dryazhenkov (2009), stricture was developed in 6.5 patients from 46 patients who underwent surgery.

What are the results of the operation on the so-called lost drainage, which was done to four patients with the initial form of CM (stenotic appearance)? Researchers VS Saveliev and VI Revyakin (2003) note the positive dynamics of the course of the disease, but explain the removal of the drainage system from the duct area by the following reasons:

• Development of jaundice;
• Obstruction of drainage, provoked by the formation of small concrements;
• Deposition of salts on the drainage walls;
• A cluster of detritus, similar in consistence to putty, which provokes repeated attacks of cholangitis.

The highest degree of difficulty is the operation on patients with a high degree of destruction of the choledocha wall. If the disease is in the third or fourth stage, then there is a higher mortality rate after surgery. With a disease in the third or fourth degree, most surgeons are in favor of conducting choledochojunoanastomosis.

Treatment after surgery

How does Mirizzy's syndrome stop? Treatment after surgery involves the delivery of a general blood test the day after the operation, a week and a day before discharge from the hospital. Sutures are removed on day 10.

The average length of hospital stay is 10-12 days. The total duration of the recovery period is two months.
Usually patients are shown rest in a sanatorium in a rehabilitation department.

Conclusion

To date, Mirizzi's syndrome, classification, diagnosis, treatment described in this article, medicine is considered as one of the complications of cholelithiasis. However, in the field of diagnosis and surgical intervention, a number of unsolved issues remain.

Despite the fact that there is a wide range of different types of surgical intervention, the results of treatment do not always meet the expectations.
During the operation, the level of intra- and postoperative complications increases.

Difficulties in conducting diagnostic activities, the risk of damage to the bile duct, a small number of observations, and a wide range of surgical techniques are prerequisites for a more in-depth study of the problem.

The introduction of modern diagnostic principles and the development of optimal tactics in the field of surgery, depending on the stage of development of the disease, makes it possible to optimize the therapy of patients with this complication of CLS.