Mirizzi Syndrome

Thanks to Richard for presenting the case of a middle-aged man who presented with acute onset of lower back pain, intermittent abdominal pain, and emesis, found to be septic, work up revealing Mirizzi syndrome causing acute cholangitis which led to klebsiella bacteremia and L spine osteomyelitis! Whoosh!


Clinical Pearls

  • Klebsiella is found along the GI tract and can cause UTIs, pneumonias, osteomyelitis, GI infections, and surgical site wound infections.
  • Charcot triad of pain, fever, and RUQ pain is found in only ~50% of patients who present with acute cholangitis.  So do not rule out the diagnosis if someone doesn’t have all three.
  • Mirizzi syndrome is rare and can be accompanied by acute chonagitis, acute cholecystitis, or acute pancreatitis.  Management involves antibiotics to treat a concurrent infection in the biliary tree as well as surgical resection of the gallbladder and impacted stone.

Differential for hyperbilirubinemia:

Hyperbilirubinemia breakdown

Remember that the most common reasons for conjugated hyperbilirubinemia are extrahepatic causes and include the following:

  • Stones (30-70%)
  • Malignancy (10-50%)
  • Benign biliary strictures (5-30%)
  • Biliary stent obstruction (~20%)

Cholangitis

Most common bacteria:

  • E coli (25-50%)
  • Klebsiella (15-20%)
  • Enterococcus (10-20%)
  • Enterobacter (5-10%)

Clinical manifestations

  • Charcot’s triad (~50% have all 3)
    • Fever
    • Abdominal pain
    • Jaundice
  • Reynold’s pentad: (rare, ~5%)
    • Above PLUS
    • Hypotension
    • AMS
  • Cholestatic LFT pattern ⇒ can progress to hepatocellular LFT pattern

Assessment of disease severity

  • Severe (suppurative) cholangitis — Acute cholangitis is considered severe if it is associated with the onset of dysfunction in at least any one of the following organs/systems:
    • Cardiovascular dysfunction – Hypotension requiring pressors
    • AMS
    • Respiratory dysfunction – PaO2/FiO2 ratio <300
    • Renal dysfunction – Oliguria, serum creatinine >2.0 mg/dl
    • Hepatic dysfunction – Prothrombin time-international normalized ratio >1.5
    • Hematological dysfunction – Platelet count <100,000/mm
  • Moderate acute cholangitis — Acute cholangitis is defined as moderate if it is associated with any two of the following:
    • Abnormal WBC count (>12,000/mm3, <4,000/mm3)
    • Fever 39°C (102.2°F)
    • Age (≥75 years)
    • Hyperbilirubinemia (total bilirubin ≥5 mg/dl)
    • Hypoalbuminemia
  • Mild acute cholangitis — Mild acute cholangitis does not meet the criteria for moderate or severe cholangitis at initial diagnosis.

Management

  • For moderate to severe cases, consider admission to the ICU and urgent ERCP/GB decompression.
  • For mild cases, admit to the floor and monitor closely
  • Antibiotics
    • To cover gram negatives, narrow based on sensitivities
    • Duration is typically 7-10 days.
  • Address predisposing cause
    • Elective cholecystectomy after infection has resolved in those with gallstones

Mirizzi syndrome

Obstruction of the common bile duct from extrinsic compression, often from swelling or infection in the cystic duct, which can share a sheath with the CBD.

  • Commonly diagnosed intraoperatively in patients undergoing GB surgery
  • Presentations
    • Pain (54-100%)
    • Jaundice (24-100%)
    • Cholangitis (6-35%)
    • Acute cholecystitis (1/3 of patients)
    • Acute pancreatitis (rare)
  • Labs
    • Elevated bili and ALP
    • Leukocytosis if concurrent cholecystitis, cholangitis, or pancreatitis
  • Diagnosis
    • Imaging
      • Dilatation of the biliary system above the gallbladder neck
      • Presence of impacted stone in GB neck
      • Normal diameter below level of stone
    • US (23-46% sensitive)
    • CT abdomen (can r/o malignancy but sensitivity is 42%, specificity 99%)
    • MRCP (highest sensitivity)
  • Management
    • Surgery
    • Sometimes ERCP can be diagnostic and therapeutic as a temporizing measure to surgery or if patient is too high risk and unsuitable for surgery
    • Antibiotics for treatment of concurrent cholangitis or cholecystitis

mirizzi-syndrome-5-638

Source: https://www.slideshare.net/mohamedfazly31/mirizzi-syndrome-70749345

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