Overview
Definition:
Mirizzi syndrome is a condition characterized by extrinsic compression of the common hepatic duct (CHD) or the common bile duct (CBD) by a gallstone impacted in the cystic duct or the gallbladder's infundibulum
This compression leads to partial or complete obstruction of bile flow, mimicking a malignant biliary obstruction.
Epidemiology:
It is a relatively rare complication of cholelithiasis, occurring in approximately 0.1% to 4% of patients undergoing surgery for gallstones
More common in elderly patients and those with chronic gallstone disease
Some studies suggest a slight female predilection.
Clinical Significance:
Accurate diagnosis and appropriate surgical management are crucial to prevent complications such as cholangitis, liver abscess, biliary fistula, and pancreatitis
Failure to recognize Mirizzi syndrome can lead to suboptimal surgical outcomes and increased morbidity.
Clinical Presentation
Symptoms:
Jaundice, often fluctuating or progressive
Right upper quadrant (RUQ) pain, typically dull or colicky
Fever and chills, suggestive of cholangitis
Nausea and vomiting
Pruritus due to cholestasis
Abdominal distension
Weight loss (less common, but can occur in advanced cases).
Signs:
Jaundice (icteric sclerae and skin)
RUQ tenderness on palpation
Palpable gallbladder (Courvoisier's sign) may be present if there is associated distal CBD obstruction
Hepatomegaly in some cases
Signs of cholangitis (fever, tachycardia, RUQ tenderness - Charcot's triad)
Signs of sepsis (hypotension, altered mental status) if severe cholangitis is present.
Diagnostic Criteria:
No universally accepted strict diagnostic criteria exist, but the diagnosis is primarily based on a combination of clinical suspicion, imaging findings, and exclusion of other causes of biliary obstruction
The presence of a gallstone in the cystic duct/infundibulum causing extrinsic compression of the CHD/CBD is the hallmark.
Diagnostic Approach
History Taking:
Detailed history of gallstone-related symptoms (biliary colic, cholecystitis)
Duration and progression of jaundice
Presence of fever or chills
Previous episodes of cholangitis or pancreatitis
History of abdominal surgery
Any known comorbidities.
Physical Examination:
Thorough abdominal examination focusing on RUQ tenderness, guarding, and rebound tenderness
Assessment for hepatomegaly and Courvoisier's sign
Examination for signs of cholangitis and sepsis
General assessment for signs of chronic liver disease.
Investigations:
Liver function tests (LFTs): Elevated bilirubin (direct and indirect), alkaline phosphatase, and GGT
ALT and AST may be elevated
Complete blood count (CBC): Leukocytosis may indicate cholangitis
Ultrasound (USG) abdomen: Initial imaging of choice, may show gallstones, thickened gallbladder wall, dilated intra- or extrahepatic bile ducts, and a stone in the cystic duct/gallbladder neck
Computed tomography (CT) scan: Useful for assessing extent of ductal dilation, presence of stones, and ruling out extrinsic compression by masses
can also identify fistulas
Magnetic resonance cholangiopancreatography (MRCP): The gold standard for visualizing the biliary tree, demonstrating the level of obstruction, the impacted stone, and the presence of a cholecystobiliary fistula
Endoscopic retrograde cholangiopancreatography (ERCP): Primarily therapeutic, but can also be diagnostic
useful for stone extraction and stent placement if surgery is delayed or not immediately feasible
Endoscopic ultrasound (EUS): Can provide detailed visualization of the gallbladder, cystic duct, and proximal CBD.
Differential Diagnosis:
Malignancy of the common hepatic duct or pancreas head
Cholangiocarcinoma
Pancreatic adenocarcinoma
Gallbladder cancer invading the bile duct
Primary sclerosing cholangitis
Benign biliary strictures
Choledocholithiasis with secondary inflammation
Parasitic infestation of the bile ducts.
Management
Initial Management:
Fluid resuscitation and correction of electrolyte imbalances
Broad-spectrum antibiotics for suspected cholangitis
Analgesia for pain control
Nutritional support
Preoperative optimization for surgery.
Medical Management:
Primarily supportive
Antibiotics are essential for treating cholangitis.ursodeoxycholic acid (UDCA) may be considered for symptom management in some cases of cholestasis, though its role in Mirizzi syndrome is limited.
Surgical Management:
Surgery is the definitive treatment
The approach depends on the type of Mirizzi syndrome and the surgeon's expertise
Type I: Gallstone in cystic duct/infundibulum causing extrinsic compression
Standard cholecystectomy is usually sufficient
Care must be taken to avoid avulsion of the CHD
Type II: Gallstone erodes into the CHD/CBD, forming a cholecystobiliary fistula
Requires careful dissection
Options include:
1
Cholecystectomy with management of the fistula: If the fistula is small and the CHD/CBD lumen is adequate, cholecystectomy alone may suffice, with spontaneous closure of the fistula
2
Cholecystectomy with primary repair of the CHD/CBD: If the fistula is large or causes significant ductal defect, primary repair may be necessary
3
Transhepatic or trans-cystic duct stenting: May be used to maintain bile flow across the repaired area
4
Hepaticojejunostomy (Roux-en-Y): Reserved for complex cases with extensive damage to the CHD/CBD or when other methods fail
Laparoscopic vs
Open Surgery: Laparoscopic cholecystectomy can be performed in Type I Mirizzi syndrome
For Type II, it is technically more challenging and may require conversion to open surgery
A cautious approach with intraoperative cholangiography is recommended
Recognition of the fistula is key
Sometimes, a staged approach (e.g., ERCP for decompression followed by surgery) might be considered.
Supportive Care:
Close monitoring of vital signs, fluid balance, and LFTs postoperatively
Pain management
Early ambulation
Gradual resumption of oral intake
Monitoring for complications like cholangitis, sepsis, fistula formation, or bile leak.
Complications
Early Complications:
Bile leak from the cystic duct stump or CHD/CBD
Cholangitis
Pancreatitis
Hemorrhage
Injury to the CHD/CBD or hepatic artery
Formation of a cholecystobiliary fistula if not recognized and managed
Abscess formation.
Late Complications:
Biliary strictures leading to recurrent cholangitis or secondary biliary cirrhosis
Recurrent choledocholithiasis
Cholangiocarcinoma (increased risk due to chronic inflammation)..
Prevention Strategies:
Meticulous surgical technique during cholecystectomy
Careful dissection of the cystic duct and common bile duct
Intraoperative cholangiography to identify anatomical variations and stones
High index of suspicion for Mirizzi syndrome based on preoperative imaging and intraoperative findings
Prompt recognition and management of any intraoperative bile duct injury or fistula.
Prognosis
Factors Affecting Prognosis:
The type of Mirizzi syndrome (Type I generally has a better prognosis than Type II)
The extent of biliary tree damage
Presence of cholangitis or sepsis at presentation
Surgeon's experience
Promptness and appropriateness of surgical intervention.
Outcomes:
With appropriate surgical management, the prognosis is generally good
Most patients with Type I Mirizzi syndrome recover uneventfully after cholecystectomy
Patients with Type II Mirizzi syndrome may have a higher risk of complications and may require more complex reconstructive procedures
Long-term outcomes are usually favorable if complications are avoided.
Follow Up:
Regular follow-up appointments are recommended to monitor for recurrent symptoms, signs of biliary stricture, or other late complications
LFTs may be checked periodically
Imaging (USG or MRCP) may be considered if symptoms recur
Duration of follow-up depends on the complexity of the case and presence of any complications.
Key Points
Exam Focus:
Mirizzi syndrome is extrinsic compression of CHD/CBD by cystic duct stone
Types I (compression) vs
II (fistula)
MRCP is key for diagnosis
Surgery is definitive
Laparoscopic approach is feasible for Type I, challenging for Type II
Be wary of bile duct injury.
Clinical Pearls:
Always consider Mirizzi syndrome in patients with jaundice and gallstones, especially if imaging suggests a stone in the cystic duct/gallbladder neck
Intraoperative cholangiography is invaluable
If a fistula is encountered, do not force dissection
consider conversion to open or staged management.
Common Mistakes:
Misdiagnosing Mirizzi syndrome as a malignant obstruction, leading to unnecessary extensive oncological resections
Aggressive dissection in Type II Mirizzi leading to severe bile duct injury
Inadequate management of the cholecystobiliary fistula
Performing routine cholecystectomy in Type II without considering the fistula, leading to bile leak or stricture.