Abstract: Mirizzi syndrome was first described by Pablo Luis Mirrizi. The incidence of this syndrome ranges from 0.05% to 4%. This rare condition is caused by the obstruction of common hepatic duct or common bile duct due to compression caused by several impacted stones or a single large impacted gallstone in Hartmann’s pouch. The clinical presentations may vary from no symptoms to severe cholangitis. It is a rare cause of obstructive jaundice and hence we are reporting a case to emphasize that while evaluating a case of obstructive jaundice, one must consider Mirizzi syndrome as a differential diagnosis.

Case Report: A 49-year-old male presented to us with chief complaints of low-grade fever, yellowish discoloration of urine, itching in body and right upper abdominal pain for 5 days. The patient was a known case of type 2 diabetes mellitus for the past 8 years and was on oral hypoglycemic agents. He had history of jaundice 20 years back. On examination, the patient was conscious, cooperative and oriented to time, place and person. His vital signs were stable and temperature was 99.6°F (oral). He had no pallor, cyanosis, clubbing, lymphadenopathy or edema, but icterus was present. On systemic examination, liver was palpable but spleen and gallbladder were not palpable. We kept the diagnosis as febrile illness, possibly due to malaria, dengue fever, scrub typhus or acute cholangitis.

Patient was further evaluated and his blood biochemistry showed that renal function tests were normal (blood urea - 16 mg/dL, serum creatinine - 0.70 mg/dL). Liver function tests were deranged (total bilirubin - 5.7 mg/dL, direct bilirubin - 2.3 mg/dL, serum glutamic oxaloacetic transaminase [SGOT] - 245 U/L, serum glutamic pyruvic transaminase [SGPT] - 513 U/L, alkaline phosphatase [ALP] - 341 U/L, total protein - 7.5 g/dL, serum albumin - 3.8 g/dL). His lipid profile was also deranged (serum cholesterol - 195 mg/dL, serum triglyceride - 262 mg/dL, high-density lipoprotein cholesterol [HDL-C] - 18 mg/dL, low-density lipoprotein cholesterol [LDL-C] - 130 mg/dL). Complete hemogram showed hemoglobin - 11.5 mg/dL, mean corpuscular volume (MCV) - 60.1 fL, total leukocyte count (TLC) - 9,940/mm³, platelet count - 3.51 × 10³/mm³. His fever profile and viral markers were negative (Malarial parasite quantitative buffy coat [MPQBC], Dengue immunoglobulin M/immunoglobulin G [IgM/IgG], scrub typhus IgM capture ELISA test, hepatitis B surface antigen [HBsAg], anti-hepatitis C virus [HCV], IgM anti-hepatitis A virus [HAV], IgM anti-hepatitis E virus [HEV], human immunodeficiency virus [HIV]). Ultrasonography (USG) showed fatty liver, cholelithiasis with ill-defined hypoechoic area in gallbladder neck region resulting in distended gallbladder with dilated intrahepatic vascular and biliary radicles, possibly indicating cholecystitis. 

On further investigating, contrast-enhanced computed tomography (CECT) abdomen showed evidence of calcified calculus of size 4.4 × 2.56 cm in gallbladder lumen in body and in neck region, associated with dilatation of central (right hepatic duct [RHD] - 7 mm and left hepatic duct [LHD] - 8 mm) and peripheral intrahepatic biliary radical (IHBR). Gallbladder appeared significantly overdistended; however, wall thickness appeared normal. Dilatation of central and peripheral IHBR with overdistended gallbladder likely due to gallbladder neck calculus impact on coronary heart disease or CHD (suggestive of Mirizzi syndrome). Magnetic resonance cholangiopancreatography (MRCP) showed a 42 mm calculus noted in gallbladder neck causing compression over common bile duct (CBD). Mild IHBR dilatation was seen. These findings were suggestive of Mirizzi syndrome with cholelithiasis, as shown in the Figure 1. Hence, the diagnosis of Mirizzi syndrome was confirmed. Patient was advised surgical treatment for this, and therefore, was transferred to the surgical ward for further management.

Mirizzi syndrome can present to us in various forms, including obstructive jaundice. It is a rare cause of obstructive jaundice. So, while evaluating a case of obstructive jaundice, one must consider Mirizzi syndrome as one of the differential diagnoses.

SUGGESTED READING

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About the Authors

Mahesh Dave, Manasvin Sareen, Anuj Goyal, Ram Gopal Saini, Sahil Kharband 

Senior Professor and Ex-HOD

Resident Doctor

Assistant Professor

Dept. of Medicine, RNT Medical College, Udaipur, Rajasthan

Address for correspondence

Dr Manasvin Sareen

Dept. of Medicine, RNT Medical College, Udaipur - 313 001, Rajasthan