Jaundice is NOT Always Present in Mirizzi Syndrome
Jaundice is a common but not universal feature of Mirizzi syndrome, occurring in the majority but not all cases. The clinical presentation varies depending on the degree and duration of bile duct compression by the impacted gallstone.
Clinical Presentation Variability
The evidence demonstrates that while jaundice is characteristic of Mirizzi syndrome, it is not present in 100% of cases:
Mirizzi syndrome presents with obstructive jaundice in most cases due to extrinsic compression of the common hepatic duct by a stone impacted in the cystic duct or gallbladder neck 1, 2, 3.
However, patients may present with acute cholecystitis symptoms without jaundice, particularly in early or partial obstruction 4. The degree of jaundice correlates with the extent of bile duct compression and whether complete obstruction has occurred 2.
Some patients present with abdominal pain and fever as primary symptoms, with jaundice developing subsequently or remaining mild 4. One case series noted that diagnosis requires "a high index of clinical suspicion" precisely because the presentation can be variable 2.
Why Jaundice May Be Absent or Mild
Several factors explain the variable presence of jaundice:
Incomplete obstruction allows some bile flow, preventing significant hyperbilirubinemia 2, 3.
Early presentation before complete obstruction develops may show cholecystitis symptoms without jaundice 4.
Intermittent obstruction from stone movement can cause fluctuating symptoms 1.
Diagnostic Implications
The absence of jaundice should not exclude Mirizzi syndrome from the differential diagnosis:
Ultrasound findings of dilated intrahepatic ducts with an unclear gallbladder should raise suspicion even without jaundice 4.
ERCP remains the gold standard for diagnosis, establishing the diagnosis in nearly all cases where it is performed, regardless of jaundice severity 1, 3.
Laboratory findings may show cholestatic pattern (elevated alkaline phosphatase, GGT) even with minimal bilirubin elevation 5.
Common Clinical Pitfall
The critical error is assuming that the absence of jaundice rules out Mirizzi syndrome. Surgeons should maintain high suspicion when encountering difficult anatomy during cholecystectomy, particularly with stones impacted in the cystic duct or gallbladder neck, even if preoperative jaundice was absent 1, 3. This vigilance helps prevent bile duct injury, which occurs more frequently in Mirizzi syndrome cases 3.