IntroductionAcute acalculous cholecystitis accounts

for 5% to 11% of all patients with acutecholecystitis and approximately 1% to 2%of all patients undergoing cholecystectomy.Acute acalculous cholecystitis usually oc-

curs in critically ill patients, following trau-ma, burns, long term parenteral nutrition,and major nonbiliary operations such asaortic abdominal aneurysm repair.

The etiology of acute acalculous chole-cystitis remains unclear, although cysticduct stenosis and ischemia have been mostoften implicated as causative factors. Thedisease often has a fulminant course and

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Case ReportMedical Journal of the Islamic Republic of Iran.Vol. 22, No.4, 2007, pp.208-211

Mirrizi syndrome due to acalculous cholecystitis in a 13-year-old girl: a case report and literature review

M. Mozafar, MD.1, M. R. Kalantar Motamedi, MD, FACS.2,H. Khajouei Kermani, MD.3, and, M. Zeynalzadeh, MD.4

Deptartment of Surgery, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

AbstractAcute inflammation of the gallbladder can occur without gallstones.

Acalculous cholecystitis typically develops in critically ill patients in theintensive care unit. Patients on parenteral nutrition, with extensive burns,sepsis, major operations, multiple organ trauma or prolonged illness withmultiple organ system failure are at risk for developing acalculous chole-cystitis. The association of acalculous cholecystitis with Mirizzi syn-drome is very unusual. Mirrizzi syndrome, which is an unusual cause ofobstructive jaundice, is most commonly caused by a stone impacted inHartmann’s pouch, exerting pressure over the common bile duct (CBD)with subsequent erosion into the CBD. The case we are presenting is acase of Mirrizi syndrome type-1 due to acalculous cholecystitis in a 13-year-old girl that presented with intermittent jaundice and RUQ abdomi-nal pain and fever. Intraoperative finding showed Mirizzi syndrome type-1 without gallstones. The cause of jaundice was only pressure of the gall-bladder on the CBD and cholecystectomy with intraoperative cholan-giography was performed. At post operative follow-up, the patient be-came anicteric and all symptoms and signs disappeared.

Keywords: Acalculous cholecystitis, cholecystocholedochal fistula,Mirizzi syndrome, cholecystectomy.

1. Associate professor of general and vascular surgery, Shahid Beheshti Medical College, Tehran, Iran.2. Corresponding author: Professor and Chairman of General and Vascular Surgery. Address: Shohad-e-Tajrish Hospital, Ghods Sq,Tehran, Iran. Tel: (+98 21) 22 71 80 01-14, Fax: (+98 21) 22 72 11 44, email: Houman.KK@gmail.com.3&4. Residents of general surgery, Shahid Beheshti Medical College, Tehran, Iran.

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frequently progresses to gangrene, empye-ma, or perforation.

The symptoms and signs depend on thecondition of the patient, but in the alert pa-tient, they are similar to acute calculouscholecystitis, with right upper quadrantpain and tenderness, fever, and leukocyto-sis. Ultrasonography is usually the diag-nostic test of choice. It can demonstrate thedistended gallbladder with thickened wall,biliary sludge, pericholecystic fluid and thepresence or absence of abscess formation[4,7].

Because these patients are usually ill,about 90% will improve with percutaneouscholecystostomy, however they do not curecompletely, and other steps such as opencholecystectomy may be required [9,10].

Mirizzi syndrome is an unusual and spe-cific cause of obstruction of the commonhepatic duct or common bile duct due tocontiguous inflammation in the gallbladderor the cystic duct or due to compression ofthe common hepatic duct (CHD) by an im-pacted large stone in the adjacent Hart-mann’s pouch or neck of the gallbladder.The stone may simply press on the bileduct, but more commonly, it ulcerates into

the duct, creating a cholecystocholedochalfistula. Patients present with obstructivejaundice and cholangiography shows nar-rowing of the bile duct at the porta hepatis,which can have the appearance of a cholan-giocarcinoma. The true pathology is even-tually identified at surgery [8] but the oper-ation is often extremely difficult because ofsevere inflammation and fibrosis [1,2]. It isbest not to excise the gallbladder, until thestone causing the obstruction is removed. Ifthere is a large gap in the wall of the bileduct, a biliary enteric bypass is needed; 5this can be achieved by anastomosing theneck of the gallbladder to a Roux-en-Ylimb of jejunum. A reconstruction of thebile duct over a T-tube brought out througha separate stab incision is possible for verysmall defects. Therefore the treatment ofcholecystocholedochal fistula depends onthe Mirizzi syndrome type encountered [3, 6].

Type-1 is external compression of thecommon bile duct, without fistula forma-tion. Consequently management is similarto that of gallbladder stone disease. Mirizzitypes II through IV all represent cholecys-tocholedochal fistula. The gallbladdershould not be removed and after the im-

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Fig. 1. MRCP before operation showed a narrowing near the common hepatic duct (CHD).

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pacted stone has been extracted, the CBDdefect can be managed as above [2].

Case reportThe patient was a 13-year-old girl who

developed right upper quadrant abdominalpain, nausea, vomiting, jaundice, pruritusand dark urine three weeks before hospital-ization.

She had experienced similar attacks ofjaundice and pruritus, intermittently, sincefour years ago, and she had used on antibi-otics, hydroxyzine and cholestyramine onand off for the past four years. This timeshe had severe abdominal pain, which didnot improve with antibiotics and anal-gesics. Previous ultrasound studies had re-vealed a dilated, large gallbladder with nostones and biliary tracts were normal. Onexamination, she had low grade fever, clin-ical jaundice, moderate tenderness in theright upper quadrant, but no peritonealsigns. There was no hepatosplenomegaly.

Ultrasound examination revealed a largeand dilated gallbladder with thickenedwall, and no stones. Intrahepatic ducts weredilated, and the CBD was normal.

MRCP showed an enlarged gallbladderwith dramatic size, and intrahepatic bileducts were markedly dilated as well as theproximal portion of CHD. There was shortsegment narrowing (11 mm) of the CBDnear the common hepatic duct (Fig. 1).

The patient underwent laparotomy witha Kocher incision. The gallbladder was

very large, with thickened wall and severeinflammation and a gangrenous patch onthe body.

The gallbladder was intrahepatic caus-ing pressure over the common hepatic duct(CHD). After the dilated gallbladder wasdrained an intraoperative cholangiographywas performed by injecting dye into thegallbladder which showed that intrahepaticducts are dilated and the CBD has a nar-rowing just near the CHD, and there wasnormal passage of contrast into the duode-

Mirrizi syndrome due to acalculous...

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Table 1. Laboratory values of the studied case

Fig. 2. Intraoperative cholangiography after chole-cystectomy showed normal passage of contrast in

the duodenum.

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num. There were no stones in the CBD.Thus, a cholecystectomy was performedand particular attention was paid to the dis-section between the gallbladder and CBD.After cholecystectomy, there was no fistulaand control cholangiography through thecystic duct showed normal passage of con-trast to intrahepatic and extrahepatic ductsand into the duodenum (Fig. 2).

Three days after surgery, liver functiontests rapidly improved. Elevated serumbilirubin and liver function tests continual-ly improved, and one month after surgery,the patient was well with normal liver func-tion tests.

Pathological examination showed acalcu-lous cholecystitis, with patchy necrosis andedema of the serosa and muscular layers.

DiscussionIn the literature, Mirizzi syndrome has

been mainly reported in relation to gall-bladder stones and it is very rare to haveMirizzi syndrome in acalculous cholecysti-tis [1,2,7].

In this paper, we have presented a reporton a patient who suffers from Mirizzi syn-drome type-I due to acalculous cholecysti-tis and recovery has been achieved justwith a cholecystectomy.

The other noteworthy point about thispatient is occurrence of acalculous chole-cystitis in a young girl who was healthyotherwise. Our diagnosis before operationfor the patient was choledochal cyst, whichis by far more common. While performingcholecystectomy with enough time and pa-tience, care should be taken not to injurethe common bile duct in Mirizzi syndrometype-1 [5].

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59(3): 34-7.Xiaodong H, Hongsheng L, Chaoji Z, et al. Diag-

nosis and treatment of the Mirizzi syndrome. ChinMed Sci J 1999; 14(4): 246-8.

Kaushik R, Sharma R. Jejunal serosal onlay flapfor repair of large common bile duct defect in Mi-rizzi syndrome. Trop Gastroenterol 2001; 22(3):169-70.

Adams TW, Foxley EG. A diagnostic techniquefor acalculous cholecystitis. Surg Gynecol Obstet1976; 142(2): 168-70.

Corlette MB. Biliobiliary fistula. Arch Surg1975; 110: 337.

Csendes A, Carlos Diaz J, et al. Mirrizi syndromeand cholecystobiliary fistula: A unifying classifica-tion. Br J Surg 1989; 76: 1139.

Singer AJ. Correlation among clinical, laborato-ry and hepatobiliary scanning findings in patientswith suspected acute cholecystitis. Ann Emerg Med1996; 28: 276.

Hakala T. Microangiopathy in acute acalculouscholecystitis. Br J Surg 1997; 84: 1249.

Hultman CS, Hergst CA, Mc Call JM, et al. Theefficacy of percutaneous cholecystectomy in criti-cally ill patients. Am Surg 1996. 62: 263.

Lo LD, Vogelzang R, Braun MA, et al. Percuta-neous cholecystectomy for the diagnosis and treat-ment of acute calculus cholecystitis. J Vasc Inter-vent Radiol 1995; 6: 629.

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