Reverse Mirizzi Syndrome

Abstract

A man in his 40s presented to our Hospital with abdominal pain, jaundice, and pruritus. He had a history of Alagille Syndrome treated with cholecystojejunostomy in the neonatal period because of initial misdiagnosis of biliary atresia. Laboratory investigations showed hyperbilirubinemia (total bilirubin 1.76 mg/dL [<1.2 mg/dL]; conjugated 1.06 mg/dL [<0.3 mg/dL]) and cholestasis (GGT 78 U/L [<50 U/L]; ALP 200 U/L [<50 U/L]). Transabdominal ultrasound was limited by aerobilia due to the cholecystojejuno-anastomosis. Subsequent basal CT scan revealed an impacted stone within the patient's native common bile duct (CBD). Aerobilia in intrahepatic bile ducts and gallbladder was reported. Magnetic Resonance cholangiopancreatography confirmed the gallstone in the CBD compressing cystic duct and common hepatic duct, with dilation of the upstream bile ducts. Furthermore, the native CBD was obstructed by other gallstones. In Mirizzi syndrome, gallstones impacted in gallbladder's Hartmann's pouch or cystic duct extrinsically compress CBD. We suggest naming the present condition "Reverse Mirizzi Syndrome" (Renzulli Matteo Syndrome, RMS) because it is the exact opposite of Mirizzi syndrome.

Keywords: Alagille syndrome; Biliary atresia; Cholecystojejunostomy; Cholelithiasis; Gallstone; Mirizzi syndrome.

Fig. 1

(Panel A) Basal CT scan shows a large, partially calcified gallstone (arrow) occupying the patient's native common bile duct. (Panels B and C) Magnetic Resonance cholangiopancreatography (MRCP) shows the gallstone (asterisk) compressing cystic duct (thin arrow, Panel C) and common hepatic duct, with dilation of the upstream bile ducts. The native common bile duct is obstructed by other gallstones (arrowheads, Panel B). Cholecystojejuno-anastomosis is pointed by the empty arrow in Panel C.