PSEUDO-MIRIZZI SYNDROME IN ACUTECHOLECYSTITIS

K. Mergener, M.D., R. Enns, M.D., W. S. Eubanks, M.D.,J. Baillie, M.B.Ch.B., and M. S. Branch, M.D.

Division of Gastroenterology and Division of General Surgery,Duke University Medical Center, Durham, North Carolina

Common hepatic duct obstruction secondary to an impactedcystic duct stone is commonly referred to as the Mirizzi syn-drome. Mirizzi syndrome is an uncommon cause of obstructivejaundice and can be mimicked by several other rare condi-tions. We describe a patient with a massively distended gall-bladder due to acute cholecystitis who presented with clinicaland cholangiographic findings simulating the Mirizzi syn-drome. Endoscopists should be aware of acute cholecystitis asa possible etiology of common hepatic duct obstruction. (Am JGastroenterol 1998;93:2605–2606. © 1998 by Am. Coll. ofGastroenterology)

INTRODUCTION

Mirizzi syndrome, defined as common hepatic duct obstructionsecondary to extrinsic compression from an impacted cystic ductstone (1), is an uncommon cause of obstructive jaundice. Theclinical syndrome and cholangiographic appearance of Mirizzisyndrome can be mimicked by several other rare conditions suchas gallbladder carcinoma, carcinoma of the cystic duct, or lymph-adenopathy in the porta hepatis. We describe a patient with acutecholecystitis who presented with clinical and radiographic findingssimulating the Mirizzi syndrome.

CASE REPORT

A 69-yr-old man presented with right upper quadrant pain, darkurine, and intermittent fevers. Physical examination was notablefor scleral icterus and right upper quadrant tenderness withoutrebound or guarding. No organomegaly or palpable masses wereappreciated. Total bilirubin was 4.1 mg/dl, aspartate aminotrans-ferase 273 IU/ml, alkaline phosphatase 776 IU/ml. The white bloodcell count was 11,600/ml. Ultrasonography showed a distendedgallbladder measuring 13 cm in its longitudinal axis. No wallthickening, pericholecystic fluid, or gallstones were seen. Theextra- and intrahepatic bile ducts were not dilated. A technetium-labeled HIDA scan revealed no excretion of the radioactive tracerat 2 h. Endoscopic retrograde cholangiopancreatography (ERCP)demonstrated a smooth, curved stricture of the common hepaticduct, consistent with extrinsic compression from the distendedgallbladder (Fig. 1). A biliary stent was placed resulting in gooddrainage. At the time of laparoscopic cholecystectomy, a massivelydilated, gangrenous-appearing gallbladder was found. Pathologicexamination of the resected specimen showed evidence of acuteinflammation and necrosis but no stones in the gallbladder or cysticduct. The patient made an uneventful recovery. A repeat cholan-giogram at the time of biliary stent removal revealed completeresolution of the biliary stricture.

DISCUSSION

Common hepatic duct (CHD) obstruction secondary to an im-pacted cystic duct stone was first reported by Kehr in 1905 (2). In1948, Mirizzi, an Argentine surgeon, provided a comprehensivedescription of the associated syndrome that now bears his name(1). His classical description included four components: 1) a closeparallel course of cystic duct and CHD, 2) an impacted stone in thecystic duct or neck of the gallbladder, 3) CHD obstruction sec-ondary to external compression by the cystic duct stone (and thesurrounding inflammation), and 4) jaundice with or without recur-rent cholangitis. Patients with Mirizzi syndrome typically presentwith right upper quadrant pain, jaundice and fever (Charcot’striad). After initial imaging with ultrasound or CT, these patientsare frequently referred for ERCP. Because of the close proximityof cystic duct and CHD, preoperative diagnosis of Mirizzi syn-drome is important to reduce the risk of inadvertent bile ductinjury, which is of particular concern in this era of laparoscopicsurgery. The typical cholangiographic finding of Mirizzi syndromeis a smooth stricture caused by lateral compression of the commonhepatic duct. Although this appearance is most commonly causedby a calculus impacted in gallbladder neck or cystic duct, a similarcholangiographic appearance can result from extrinsic compres-sion caused by gallbladder carcinoma (3, 4), carcinoma of thecystic duct (5), or lymphadenopathy in the porta hepatis (6).Intrinsic lesions of the common hepatic duct, such as cholangio-carcinoma or isolated strictures due to primary sclerosing cholan-gitis or operative injury, also need to be excluded. Ippolito (7)presented a case similar to ours of a patient with acute cholecystitisand biliary obstruction as evidenced by the absence of excretion ofthe radioactive tracer on a HIDA scan. However, although theCHD was mildly distorted by extrinsic compression (caused by thedistended gallbladder), the narrowing was,50% of the duct’soriginal diameter, making it unlikely that there was significantReceived Apr. 21, 1998; accepted May 22, 1998.

FIG. 1. ERCP demonstrates a smooth common hepatic duct stricture(arrows) caused by extrinsic compression from the distended gallbladder.

AJG – December 1998 BRIEF CASE REPORTS 2605

mechanical obstruction of bile flow. In our patient, massive gall-bladder distension caused almost complete occlusion of the com-mon hepatic duct. Even before ERCP, there was little doubt aboutthe cause, as several imaging studies had already revealed massivegallbladder distension. However, ERCP was useful in this case toexclude other biliary pathologies and for stenting to relieve theobstructive jaundice and to prevent cholangitis pending surgery.This case reminds us that the radiographic appearance of Mirizzisyndrome can be mimicked by other conditions. Endoscopistsshould be aware of acute cholecystitis as a possible etiology ofcommon hepatic duct obstruction.

Reprint requests and correspondence: Klaus Mergener, M.D., I. Mediz-inische Klinik und Poliklinik (Direktor: Univ.-Prof. Dr. P. R. Galle),Johannes Gutenberg Universitaet, Langenbeckstrasse 1, D-55101 Mainz,Germany.

REFERENCES

1. Mirizzi PL. Sindrome del conducto hepatico. J Int Chir 1948;8:731–7.2. Kehr H. Die in meiner Klinik geuebte Technik der Gallensteinopera-

tionen, mit einem Hinweis auf die Indikation und die Dauererfolge.Munchen: JF Lehmann, 1905.

3. Musher DR, Madayag MA, Tobias J. Carcinoma of the gallbladder: Adiagnosis aided by endoscopic retrograde and percutaneous hepaticcholangiography. Am J Gastroenterol 1976;66:79–83.

4. Baron L, Balfe M, Weyman J. A prospective comparison of the eval-uation of biliary obstruction using computed tomography and ultra-sonography. Radiology 1982;145:91–8.

5. Walker JM, Kanzer BF. Carcinoma of the cystic duct mimicking theMirizzi syndrome. Am J Gastroenterol 1982;77:936–8.

6. Glenn F, Evans JA, Halpern M, et al. Selective celiac and superiormesenteric arteriography. Surg Gynecol Obstet 1964;118:93–100.

7. Ippolito RJ. Acute acalculous cholecystitis associated with commonhepatic duct obstruction: A variant of Mirizzi’s syndrome. ConnecticutMedicine 1993;57:451–5.

FATAL INTRAOPERATIVE PULMONARY EMBOLISMFROM A HEPATIC HYDATID CYST

Markus A. Rothlin, M.D.

Department of-Surgery, Zu¨rich University Hospital, Zu¨rich,Switzerland

A 43-yr-old woman was operated for recurring hydatidcysts of the liver. One of the cysts was located in segment 8adjacent to both inferior vena cava and right hepatic vein.During the operation, after application of traction on the liverthe patient suddenly went into cardiac arrest. After applyingopen heart massage a Trendelenburg operation was per-formed, revealing a massive embolus of echinococcal materialinto the paracentral branches of the pulmonary artery. Resus-citation was unsuccessful. In the literature only four similarcases have been described. The conclusions from these deathsare that an adequate incision is mandatory, no traction on theliver should be necessary, and total vascular exclusion of theliver before cyst drainage and extracorporal bypass are nec-essary. Interventional techniques should be avoided. (Am JGastroenterol 1998;93:2606–2607. © 1998 by Am. Coll. ofGastroenterology)

INTRODUCTION

In most countries in northern and central Europe, as well as inthe United States,Echinococcus granulosusof the liver is a raredisease. Most cases are observed in immigrants from areas wherethis parasite is endemic. The increasing migration of the populationtoday increases the contact between these patients and surgeons orgastroenterologists hitherto inexperienced in the therapy of thedisease. This case illustrates a classical, albeit rare, pitfall in thetreatment of hepatic echinococcosis.

CASE REPORT

A 43-yr-old woman was admitted to our unit for treatment ofthree hydatid cysts in the right lobe of the liver in 1987. The patienthad immigrated to Switzerland from the Mediterranean area. Shehad undergone previous surgery for hepatic echinococcosis else-where. She had no history of pulmonary problems. Recurrence ofher echinococcosis was diagnosed during an attack of acute pan-creatitis. On admission the patient presented with pain in the rightupper quadrant and a palpable liver of increased consistency.Pulmonary auscultation and percutation was normal. Serologicaltests were positive forE. granulosus. Preoperative abdominalsonography and CT-scan demonstrated three lesions in segments 7and 8, the biggest of which was adjacent to the right hepatic vein.The operation was performed through a bilateral subcostal inci-sion. Intraoperative sonography confirmed the position of the bigcyst in segment 8, which did not reach the surface of the liver andcontained a large amount of solid matter (Gharbi type IV lesion).The needle was positioned in the liquid part of the cyst and thecontents were aspirated. A 50%-glucose solution was then injectedinto the cyst. On removal of the needle a drop of murky, yellowliquid appeared at the puncture site. Traction was then applied onthe liver in a caudal direction and the resection was started, whenthe patient suddenly went into cardiac arrest. An anaphylacticreaction was suspected, but medical resuscitation was ineffective.Therefore, the thoracic cavity was opened and open heart massageapplied. The heart seemed congested and defibrillation did notsucceed in reinstating a normal rhythm. The possibility of a pul-monary embolus was then taken into consideration and a Tren-delenburg operation was performed. Echinococcal material wasaspirated with a suction device from the peripheral branches. Afteraspiration of all the material within reach, the pulmonary arterywas sutured and heart massage reinstated. After a further 30 min ofunsuccessful resuscitation the patient was pronounced dead. Au-topsy demonstrated a perforation of the cyst in segment 8 into theright hepatic vein (Fig. 1) and a great number of paracentralpulmonary emboli by echinococcal material (Fig. 2).

COMMENT

Pulmonary embolism caused by the rupture of a hepatic hydatidcyst in close vicinity of the inferior vena cava (1–3) or the hepaticveins is an extremely rare occurrence. The embolism can occurspontaneously (1–3) and often leads to recurring attacks of dys-pnea, cyanosis, and hemoptysis. As a consequence of these re-peated embolies, secondary hydatid cysts of the lung can develop.The rupture in these cases is small and not visible on contrastcavography. If the rupture site enlarges, a fatal pulmonary embo-lism can result (1, 3).

The intraoperative rupture of the cyst causing a massive and

Received Apr. 2, 1998; accepted May 26, 1998.

2606 BRIEF CASE REPORTS AJG – Vol. 93, No. 12, 1998