Abdominal imaging
Case TypeClinical Cases
Authors
Robert Henderson, Yuranga Weerakkody
69 years, female
A 69-year-old woman presented with a short history of fevers, painless jaundice and dark urine.
Clinical examination demonstrated a soft abdomen with mild central abdominal tenderness although no guarding or peritonism. The patient was jaundiced with occasional cutaneous spider naevi. Bloods demonstrated an obstructive pattern of hyperbilirubinaemia.
Abdominal computed tomography (CT) demonstrated generalised biliary dilatation due to common bile duct (CBD) stricture from extrinsic compression by a 23 mm partially-calcified gallstone impacted within the proximal cystic duct - features consistent with Mirizzi syndrome (Figure 1A and annotated Figure 1B; Figure 2A and annotated Figure 2B). No pancreatic head mass or choledocholithiasis.
After admission for initial conservative management, an abdominal ultrasound demonstrated generalised intrahepatic biliary dilatation, distal cystic duct and proximal CBD, with extrinsic CBD compression by an impacted gallstone within the proximal cystic duct (Figure 3A and Figure 3B) and generalised gallbladder wall thickening. Additional incidental finding of localised nodular fundal thickening with spectral Doppler artefact consistent with adenomyomatosis.
The patient underwent successful endoscopic retrograde cholangiography (ERCP) which demonstrated a smooth long segment stenosis of the CBD mid-segment and upstream biliary tree dilatation (Figure 4A and annotated Figure 4B). No intrinsic filling defect evident. The procedure was completed with a sphincterotomy and stent insertion, with elective cholecystectomy booked for 8 weeks time.
Gallstones are a common disease with 10% prevalence in developed countries, although only symptomatic in 20-30% of patients [1].
Mirizzi syndrome is an uncommon manifestation of gallstone disease, with clinical incidence in developed counties peaking at 1%, although 4.7-5.7% in developing counties [1]. Incidence increases in Western countries in patients undergoing cholecystectomy (0.3-5%) [2-10] as up to 50% of diagnoses are made intraoperatively, and gallbladder cancer (>25%) [1,3-7,11-23].
Prevalence peaks in the 4-7th decades and there is no gender predilection.
Although recognised in the early 1900s by Kehr [24] and Ruge [25], it was not until 1948 when the term would be adopted after Pablo Luis Mirizzi, an Argentinian surgeon describing the syndrome del conducto hepatico - translated, the hepatic duct syndrome [26].
Pathogenesis of Mirizzi syndrome involves common hepatic duct (CHD)/common bile duct (CBD) stenosis caused by extrinsic compression secondary to an impacted gallstone within the cystic duct, gallbladder neck or Hartmann pouch. Additional causes include chronic inflammation and scar formation, with potential formation of cholecystocholedochal/cholecystoenteric fistulous communication.
Although generalised intrahepatic biliary dilatation is perceivable, gallstones are typically radiolucent (less than 15%) and hence poorly visualised on CT thereby making a diagnosis of Mirizzi syndrome difficult. Typical sonographic findings include shrunken gallbladder and large immobile stone in the region of the gallbladder neck/cystic duct [27].
Magnetic resonance cholangiopancreatography (MRCP) has high sensitivity and specificity in the detecting gallstones and biliary stenosis characterised by cholelithiasis, impacted gallstone within
the gallbladder neck/cystic duct, extrinsic biliary duct compression, intrahepatic and common hepatic ductal dilatation, and a normal distal CBD [26-32].
Recognition of the relationship between impacted stone, cystic duct morphology, point of biliary duct compression and its complications have implications on surgical planning and outcome.
Csendes classification is useful to determine surgical management [33]:
Surgical planning includes partial cholecystectomy and choledochoplasty for types 2/3, total cholecystectomy for type 1, and biliary reconstruction with hepaticojejunostomy for type 4 [33].
This case demonstrates the importance of recognising Mirizzi syndrome as an uncommon complication of gallstone disease and biliary obstruction.
[1] Abou-Saif A, Al-Kawas FH (2002) Complications of gallstone disease: Mirizzi syndrome, cholecystocholedochal fistula, and gallstone ileus. Am J Gastroenterol 97:249-54 (PMID 11866258)
[2] Beltran MA (2012) Mirizzi syndrome: history, current knowledge and proposal of a simplified classification. World J Gastroenterol 18:4639-50 (PMID 23002333)
[3] Wichmann C, Wildi S, Clavien PA (2008) The relationship of Mirizzi syndrome and cholecystoenteric fistula: validation of a modified classification. World J Surg 32:2244-45 (PMID 18709411)
[4] Gomez D, Rahan SH, Toogood GJ, Prasad KR, Lodge JPA, Guillou PJ, Menon KV (2006) Mirizzi’s syndrome - results from a large western experience. HPB (Oxford) 8:474-9 (PMID 18333104)
[5] Erben Y, Benavente-Chenhalls LA, Donohue JM, Que FG, Kendrick ML, Reid-Lombardo KM, Farnell MB, Nagorney DM (2011) Diagnosis and treatment of Mirizzi syndrome: 23-year Mayo Clinic experience. J Am Coll Surg 213:114-9 (PMID 21459630)
[6] Kwon AH, Inui H (2007) Preoperative diagnosis and efficacy of laparoscopic procedures in the treatment of Mirizzi syndrome. J Am Coll Surg 204:409-15 (PMID 17324774)
[7] Xu XQ, Hong T, Li BL Liu W, He X, Zheng C (2013) Mirizzi syndrome: our experience with 27 cases in PUMC Hospital. Med Sci J 28:172-7 (PMID 24074620)
[8] Beltran MA, Csendes A, Cruces KS (2008) The relationship of Mirizzi syndrome and cholecystoenteric fistula: validation of a modified classification. World J Surg 32:2237-48 (PMID 18587614)
[9] Chatzoulis G, Kaltsas A, Danilidis L, Dimitriou J, Pachiadakis I (2007) Mirizzi syndrome type IV associated with cholecystocolic fistula: a very rare condition - report of a case. BMC Sure 7:6 (PMID 17531103)
[10] Yeh CN, Jan YY, Chen MF (2003) Laparoscopic treatment for Mirizzi syndrome. Surg Endosc 15:1753-78 (PMID 12964062)
[11] Zaliekas J, Munson JL (2008) Complications of gallstones: the Mirizzi syndrome, gallstone ileus, gallstone pancreatitis, complications of ‘lost’ gallstones. Surg Clin North Am 88:1345-68 (PMID 18992599)
[12] Kelly MD (2009) Acute Mirizzi syndrome JSLS 13:104-9 (PMID 19366554)
[13] Desai DC, Smink RD (1997) Mirizzi syndrome type II: is laparoscopic cholecystectomy justified? JSLS 1:237-9 (PMID 9876678)
[14] Safioleas M, Stamatakos M, Safioleas P, Smyrnis A, Revenas C, Safioleas C (2008) Mirizzi syndrome: an unexpected problem of cholelithiasis. Our experience with 27 cases. Int Semin Surg Oncol 5:12 (PMID 18495037)
[15] Lai EC, Lau WY (2006) Mirizzi syndrome: history, present and future development. ANZ J Surg 76:251-7 (PMID 16681544)
[16] Chan CY, Liau KH, Ho CK, Chew SP (2003) Mirizzi syndrome: a diagnostic and operative challenge. Surgeon 1:273-8 (PMID 15570778)
[17] Tsuyuguchi T, Sakai Y, Sugiyama H, Ishihara T, Yokosuka O (2011) Long-term follow-up after perusal cholangioscopy-directed lithotripsy in patients with difficult bile duct stones, including Mirizzi syndrome: an analysis of risk factors predicting stone recurrence. Surg Endosc 25:2179-85 (PMID 21184106)
[18] Fontes PR, Teixeira UF, Waechter FL, Sampaio JA, Pereira-Lima L (2012) Mirizzi syndrome in association with serum Ca 19-9 greater than 20,000U/mL: is it possible? Arq Bras Cir Dig 25:69-70 (PMID 22569985)
[19] Antoniou SA, Antoniou GA, Makridis C (2010) Laparoscopic treatment of Mirizzi syndrome: a systematic review. Surg Endosc 24:33-9 (PMID 19466486)
[20] Roesch-Dietlen F, Perez-Morales AG, Martinez-Fernandez S, Remes-Troche JM, Jiminez- Garcia VA, Romero-Sierra G (2013) Mirizzi syndrome experience at Spanish Hospital of Veracruz. Cir Cir 81:232-6 (PMID 23769254)
[21] Gibor U, Petty ZH, Netz U, Glazer Y, Laufer L, Kirshtein B (2015) Ca 19-9 in the presence of obstructive jaundice due to Mirizzi syndrome. Isr Med Assoc J 17:60-1 (PMID 25739181)
[22] Yonetci N, Kutluana U, Yilmaz M, Sungurtekin U, Tekin K (2008) The incidence of Mirizzi syndrome in patients undergoing endoscopic retrograde cholangiopancreatography. Hepatobiliary Pancreat Dis Int 7:520-4 (PMID 18842500)
[23] Palacios-Martinez D, Gutierrez Lopez M, Gordillo Lopez FJ, (2011) Sindrome de Mirizzi una causa infrecuente de ictericia obstructiva. Semergen 37:167-9
[24] Kehr H (1905) Die in einer klinik geübte tecknik de gallenstein operationen, mit einen hinweis auf die indikationen und die daueresolge. Munich: JF Lehman
[25] Ruge E (1908) Deitrage zur chirurgischen anatomie der grossen galenwege (Ductus hepaticus, choledochus, und pancreaticus). Arch Clin Chir 78:47
[26] Mirizzi PL (1948) Sindrome del conducto hepatico. J Int Chir 8:731-77
[27] Strunk H, Teifke A, Menke H (1988) Typical radiologic findings in Mirizzi syndrome (in German). Rontegenblatter 41:345-7
[28] Becker CD, Hassler H, Terrier F (1984) Preoperative diagnosis of the Mirizzi syndrome: limitations on sonography and computed tomography. AJR Am J Roentgenol 143:591-96 (PMID 6331740)
[29] Choi BW, Kim MJ, Chung JJ, Chung JB, You HS, Lee JT (2000) Radiologic findings of Mirizzi syndrome with emphasis on MRI. Yonsei Med J 41:144-46 (PMID 10731934)
[30] Kim PN, Outwater EJ, Mitchell DG (1999) Mirizzi syndrome: evaluation by MR imaging. Am J Gastroenterol. 94(9):2546-2550 (PMID 10484023)
[31] Katabathina VS, Dosyam AK, Dosyam N et al. Hosseinzadeh K (2014) Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics 34(3): 565-86 (PMID 24819781)
[32] Yun EJ, Choi CS, Yoon DY, See YL, Chang SK, Kim JS, Woo JY (2009) Combination of magnetic resonance cholangiopancreatography and computed tomography for preoperative diagnosis of the Mirizzi syndrome. J Comput Assist Tomogr 33(4):636-40 (PMID 19638864)
[33] Csendes A, Diaz JC, Burdiles P, Maluenda F, Nava O (1989) Mirizzi syndrome and cholecystobiliary fistula: a unifying classification. Br J Surg 1989; 76:1139-43 (PMID 2597969)
| URL: | https://www.eurorad.org/case/17541 |
| DOI: | 10.35100/eurorad/case.17541 |
| ISSN: | 1563-4086 |
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
