MIRIZZI SYNDROME, TYPE V

By Monzur Ahmed

A 63 year old man presented 2 weeks ago with a 10 day history of chest / abdominal pain radiating to the back. Initially, a cardiac cause was suspected and investigations were as follows:
- ECG, CXR, Troponin-T x2 normal
- CRP 120, Bilirubin 6 umol/L, ALT 59, Alk phosp 380 (NR less than 130)
- D-dimer raised
- CTPA: no pulmonary embolus

He was discharged after a couple of days and an out patient U/S scan showed: stones in gall bladder with no biliary dilatation. Whilst a laparoscopic cholecystectomy was being arranged, he was re-admitted with with RUQ pain and jaundice. Further investigations were as follows:
- Bilirubin 115 umol/L, ALT 505, Alk Phosp 1170
- CA19-9: 1300
- MRCP/ CT: Bilateral IHD dilatation, stricture / impingement of CHD; stones in thick walled GB; possible fistula between GB and duodenum
He was referred for an ERCP (see video)…

This case was quite a tricky one. There was an obstruction at the level of the common hepatic duct (CHD). Possibilities included cholangiocarcinoma and extrinsic compression my stone/ inflammatory mass (Mirizzi syndrome). An additional twist was that the inflamed thick walled gall bladder was in contact with the duodenum, possibly causing a cholecystoduodenal fistula. Such a fistula in the presence of extrinsic compression of the CHD by a stone is known as Mirizzi syndrome type V. The ERCP and stenting has bought us some time and the patient has been referred to the local Liver Unit for a cholecystectomy (probably an open procedure). Unfortunately, treatment has been delayed because the patient developed Covid-19 whilst in hospital. — Pablo Luis Mirizzi was a surgeon from Argentina, born in the city of Cordoba in1893; he died in the same city in 1964. Mirizzi performed the first operative cholangiography which is considered his main contribution to modern biliary surgery. The first description made by Mirizzi compatible with the syndrome that bears his name, was published in 1940 in an article dealing with a “physiologic sphincter of the hepatic duct” [1]. The syndrome consisted of an uncommon and benign cause of obstructive jaundice, caused by a gallstone impacted at the Hartmann’s pouch, gallbladder infundibulum or the cystic duct and its associated inflammatory process, thereby causing obstruction of the common hepatic duct [2-5]. Mirizzi, believed that the obstruction of the common hepatic duct was functional, and that the mechanical obstruction of the gallbladder infundibulum and consequent inflammatory process predisposed to the contraction of a “muscular sphincter” located in the common hepatic duct [2-5]. However, current knowledge has dismissed the presence of any sphincter, or even smooth muscle in the common hepatic duct [3,5]. The current concept of Mirizzi syndrome, includes the external compression of the bile duct and the later development of cholecystobiliary and cholecystoenteric fistulas as different stages of the same disease process [6]. Mirizzi syndrome can be caused by an acute or chronic inflammatory condition secondary to a single large gallstone or multiple small gallstones impacted in the Hartmann’s pouch or in the gallbladder infundibulum and cystic duct. The syndrome can be classified as follows [6]: Mirizzi type I is the extrinsic compression of the common bile duct by an impacted gallstone. Mirizzi type II consists of a cholecystobiliary fistula involving one third of the circumference of the bile duct. Mirizzi type III, the cholecystobiliary fistula compromises up to two-thirds of the circumference of the bile duct. Mirizzi type IV, the cholecystobiliary fistula has destroyed the bile duct wall, and comprises the whole circumference of the bile duct. Mirizzi type V corresponds to any type of Mirizzi associated with a bilioenteric fistula with or without gallstone ileus. 

REFERENCES
1.Mirizzi PL. Physiologic sphincter of the hepatic bile duct. Arch Surg. 1940;41:1325–1333.
2.Martnez MA, Granero LE. Pablo Luis Mirizzi. Acta Gastroenterol Latinoam. 2009;39:177–178.
3.Leopardi LN, Maddern GJ. Pablo Luis Mirizzi: the man behind the syndrome. ANZ J Surg. 2007;77:1062–1064.
4.Mirizzi PL. Sndrome del conducto heptico. J Int Chir. 1948;8:731–777.
5.Lai EC, Lau WY. Mirizzi syndrome: history, present and future development. ANZ J Surg. 2006;76:251–257.
​6.Bettran MA , Mirizzi syndrome: History, current knowledge and proposal of a simplified classification, World J Gastroenterol. 2012 Sep 14; 18(34): 4639–4650.