CASE REPORT
Mirizzi Syndrome in Gallstone Complication
Ario Perbowo *, Dadang Makmun ** * Department of Internal Medicine, Faculty of Medicine, University of Indonesia Dr. Cipto Mangunkusumo General National Hospital, Jakarta ** Division of Gastroenterology, Department of Internal Medicine, Faculty of Medicine, University of Indonesia/Dr. Cipto Mangunkusumo General National Hospital, Jakarta
ABSTRACT Mirizzi syndrome is a rare complication of gallstone which is characterized by the presence of gallstone LPSDFWLRQLQF\VWLFGXFWWKDWOHDGVWRLQÀDPPDWRU\VWULFWXUHLQWKHELOLDU\GXFWDQGUHVXOWVLQREVWUXFWLYH jaundice. In this report, we highlighted the diagnostic approach and management of Mirizzi syndrome in a 58 year-old male complaining of nausea, vomiting, and appearing jaundice. The role of imaging such as abdominal ultrasonography in depicting the characteristics of Mirizzi syndrome was also discussed and compared the ¿QGLQJVZLWKWKHFODVVL¿FDWLRQRIWKHGLVHDVHLQWKHOLWHUDWXUH,QWKLVSDWLHQW0LUL]]LV\QGURPHZDVVXVSHFWHGE\ the appearance acoustic shadow in the gallbladder with dilated cystic duct suggesting the impaction of common bile duct (CBD). We performed endoscopic retrograde cholangiopancreatography (ERCP) as both diagnostic and therapeutic modalities by which we allowed sphingterectomy to evacuate the gallstone. However, due to the risk of further stone evacuation, the procedure was followed by elective cholecystectomy.
Keywords: gallstone, Mirizzi syndrome, complication
ABSTRAK Sindrom Mirizzi adalah komplikasi dari penyakit batu empedu yang jarang terjadi dengan karakteristik DGDQ\DLPSDNVLEDWXHPSHGXGLGXNWXVVLVWLNXVVHKLQJJDPHQJKDVLONDQVWULNWXULQÀDPDVLGLVDOXUDQHPSHGXGDQ menyebabkan ikterik obstruktif berkelanjutan. Dalam laporan kasus ini dibahas pendekatan diagnostik dan tata laksana sindrom Mirizzi pada pasien laki-laki berusia 58 tahun dengan keluhan mual, muntah, dan jaundice. 3HUDQGDULSHQFLWUDDQVHSHUWLXOWUDVRQRJUD¿DEGRPHQGDODPPHQJJDPEDUNDQNDUDNWHULVWLNVLQGURP0LUL]]L MXJDGLGLVNXVLNDQGDQPHPEDQGLQJNDQWHPXDQSDGDNDVXVLQLGHQJDQNODVL¿NDVLSHQ\DNLWSDGDOLWHUDWXU3DGD pasien ini, sindrom Mirizzi diduga dari penampilan bayangan akustik di kandung empedu dengan saluran cystic dilatasi yang mensugestikan impaksi dari saluran empedu. Endoscopic retrograde cholangiopancreatography (5&3 GLODNXNDQVHEDJDLPRGDOLWDVGLDJQRVWLNGDQWHUDSHXWLNGLPDQDV¿QJWHUHNWRPLGDSDWPHQJHYDNXDVLEDWX empedu. Namun dikarenakan risiko evakuasi batu lanjut, prosedur diikuti oleh kolesistektomi elektif.
Kata kunci: penyakit batu empedu, Sindrom Mirizzi, komplikasi
INTRODUCTION treatment. In the United States, every year more than 1 Gallstone disease has been an important health billion USD is spent for gall stone treatment, particularly problem in western countries. Estimated prevalence of for 500.000 cholecystectomy procedures which are JDOOVWRQHGLVHDVHLVDSSUR[LPDWHO\DQGLQFUHDVHV performed every year. Risk of patients with gallstone in elderly. While in Indonesia, the number is still unclear to experience symptoms and complication is relatively due to the limited publications. Most gallstones do not VPDOO+RZHYHUDIWHUWKHVWRQHVWDUWHGWRFDXVHVSHFL¿F cause disease and do not need treatment. Gallstone biliary colic, risk of problems and complication increase disease is the main reason of hospitalization for surgical gradually.1
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Mirizzi syndrome is complication of gallstone blood abnormalities or malignancy. Patient denied the disease which rarely happened. It usually occurs in presence of allergy. Patient did not suffer from diabetes RIDOOSDWLHQWVZKRVXIIHUIURPFKROHOLWKLDVLV7KLV mellitus and hypertension. None of the patient's family V\QGURPHZDV¿UVWO\H[SODLQHGLQ ZLWKVLJQDWXUH also ever suffered from malignancy, diabetes mellitus, characteristics, which was the presence of gallstone or hypertension. Patient did not have smoking habit, impaction in cystic duct or in the neck of gallbladder, alcohol consumption, or intravenous drug user, or which then leads to mechanical obstruction in hepatic blood transfusion history. In daily basis, patient was GXFWDQGFDXVHLQÀDPPDWRU\VWULFWXUHLQWKHELOLDU\GXFW not exposed to radiation or chemical agent. thus further causes continuous obstructive jaundice.2 Physical examination of the patient at the time of Perioperative diagnosis in this condition is important. admission, patient looked moderately ill, consciousness ,QWUDRSHUDWLYH¿QGLQJVPD\UHVHPEOHH[WUDKHSDWLF FRPSRVPHQWLVEORRGSUHVVXUHPP+J KHDUW biliary malignancy, hence require further approach. UDWHEHDWVSHUPLQXWH ESP UHJXODU UHVSLUDWRU\ &KROHF\VWRELOLDU\DQGFKROHF\VWRHQWHULF¿VWXODVGXH rate 20 breaths per minute, regular, abdominothoracal to the gallstone impaction might cause postoperative UHVSLUDWRU\SDWWHUQ D[LOODWHPSHUDWXUHoC. complication which actually could be prevented by Conjunctiva looked pale, icteric sclera, no palpebral monitoring previous condition. Ultrasonography edema. Skin looked icteric. Ears, nose, and throat were (USG) and computed tomography (CT) have currently within normal limits. Jugular venous pressure 5-2 been used as primary evaluation methods in obstructive cmH2O, lymph nodes not palpable. On the chest, no jaundice. Similar condition also applied to endoscopic spider nevi was found. In both lungs, vesicular breath retrograde cholangiography (ERC).2 Currently, sound was found, without rhonchi or even wheezing. In minimally invasive therapy is important to treat gall heart examination, there was no cardiomegaly, normal stone. Doctor has to know various imaging modalities ¿UVWDQGVHFRQGKHDUWVRXQGZLWKRXWWKHSUHVHQFHRI which have been developed. In this case report, we will murmur or gallop. Flat abdomen, with no presence of discuss about the diagnostic approach and management caput medusa, tender, no pain on palpation, timpani of Mirizzi syndrome as one of the forms of obstructive on percussion, negative shifting dullness, and normal jaundice caused by gall stone. positive bowel sound. On extremities, there was no edema or palmar erythema. Laboratory results of the patient upon admission to CASE ILLUSTRATION WKHKRVSLWDOLQFOXGHKHPRJORELQJG/KHPDWRFULW $ \HDUROG PDOH SDWLHQW FDPH WR &LSWR OHXNRF\WHV X/WKURPERF\WHV Mangunkusumo Hospital with the complain of X/0HDQFRUSXVFXODUYROXPH 0&9 I/PHDQ nausea and sometimes accompanied with vomiting corpuscular hemoglobin (MCH) 29.4 pg, mean 1-2 times per day for less than 100-200 mL in the form FRUSXVFXODUKHPRJORELQFRQFHQWUDWLRQ 0&+& of food and yellowish liquid a month before hospital JG/,QGLIIHUHQWLDOFRXQWH[DPLQDWLRQEDVRSKLO admission. Nausea and vomiting was described to be HRVLQRSKLOQHXWURSKLOO\PSKRF\WHV not meal time dependent. Patient denied the presence PRQRF\WHV$VSDUWDWHWUDQVDPLQDVH of stomachache. Pale colored stool and tea-like color $67 8/LQFUHDVHGDODQLQHWUDQVDPLQDVH $/7 urine were also being complained by the patients. 8/LQFUHDVHGWRWDOELOLUXELQPJG/ Patient's eyes and skin also looked yellowish. Patient LQFUHDVHGGLUHFWELOLUXELQ PJG/LQFUHDVHG also complained of fever which present at nights, LQGLUHFWELOLUXELQPJG/XUHXPPJG/ although the temperature was not too high. Patient also FUHDWLQLQH PJG/UDQGRPEORRGJOXFRVHPJ complained of itchiness in the entire body. Patient's G/VRGLXPPPRO/SRWDVVLXPPPRO/ body weight decreased 5 kg in the last month. Because DQGFKORULGH PPRO/)URPSHULSKHUDOEORRG of these complaints, patient visited Internal Medicine smear, normocytic normochromic erythrocytes were Outpatient Ward in Cipto Mangunkusumo Hospital and found, impression of adequate leukocyte count, abdominal USG was performed, and it was concluded normal morphology, increased thrombocyte count, that there was stones in the gall bladder and duct. positive giant thrombocyte, positive clumping, and Patient was advised to undergo endoscopic retrograde thrombocytosiswith leukocytes shift to the left. cholangiopancreatography (ERCP). Reticulocyte level was not increased. Prothrombin Previously patient never experienced liver disease WLPH 37 ZLWKFRQWURODFWLYDWHGSDUWLDO or jaundice. Patient also denied if he ever suffered from WKURPERSODVWLQWLPH $377 ZLWKFRQWURO
126 The Indonesian Journal of Gastroenterology, Hepatology and Digestive Endoscopy Mirizzi Syndrome in Gallstone Complication
XULFDFLGPJG/LQFUHDVHGWRWDOFKROHVWHURO A PJG/1RQUHDFWLYHDQWLKHSDWLWLV$YLUXV +$9 IgM, non-reactive HBsAg, non-reactive anti hepatitis C virus (HCV). In this patient, there was decreased OHYHORIWRWDOSURWHLQOHYHODVPXFKDVZLWK LQFUHDVHGDP\ODVH8/DQGOLSDVH8/1RUPDO &UHDFWLYHSURWHLQ &53 PJ/DQG&$ 8P/ZHUHDOVRZLWKLQQRUPDOOLPLWV From the results of abdominal USG (Figure 1), it was concluded that there was cholelithiasis, distal choledocholithiasis of the common bile duct (CBD) with dilatation of intrahepatic gall duct and CBD.
B
Figure 1. Abdominal ultrasonography revealed hyperechoic lesion in the gall bladder with the diameter of 0,96 cm with acoustic shadow. In intrahepatic gall duct, dilatation was present. The common bile duct was dilated, with hyperechoic lesion with the diameter of 1.16 cm with acoustic shadow
During hospitalization, patient was given low fat, soft diet 1700 kcal. Because the patient's intake was not adequate, he was also given additional parental nutrition Triofusin E 1000 per 12 hours and Asering 500 cc per 12 hours. Empiric antibiotic was administered intravenous Cefoperazone Sulbactam 1 g three times daily for 7 days. Other supportive therapies include intravenous Omeprazole 40 mg twice daily, oral Domperidon 10 mg three times per day, oral Curcuma 200 mg three times daily, oral ursodeoxycholate acid 250 mg three times daily, oral Simvastatin 20 mg once daily, and oral Cetirizine 10 mg once daily. ERCP which was performed a month later revealed dilated common bile duct and left and right hepatic duct. There was small image of stone in CBD and a quite large shadow of stone in further evaluation was suspected to be located in the cystic duct. Gallbladder was visualized accompanied with image of stone Figure 2. Endoscopic retrograde cholangiography exhibited within it and appearance of dilated cystic duct which dilated common bile duct and left and right hepatic duct (A and B) gave impression of suppressing CBD. Sphingterotomy
Volume 15, Number 2, August 2014 127 Ario Perbowo, Dadang Makmun was performed and continued with ductal clearance acute pancreatitis.2-6 using balloon and basket. Only several small stones Recently, Mirizzi syndrome with bile stone ileus came out from the CBD. To the patient, stenting were require special attention from surgeons. In this performed using 10 fr 7 cm double pigtail stents. Post SDWLHQWDOWKRXJKWKHUHZDVQRVSHFL¿FSDLQLQXSSHU stenting, bile oozed. Therefore, it could be concluded right quadrant of the abdomen, but there was nausea WKDWWKHUHZDVREVWUXFWLYHMDXQGLFH VXVSHFWRI0LUL]]L and vomiting, accompanied with not too high fever syndrome, distal CBD stone, and cholelithiasis. for the last month. He also realized the presence of obstructive jaundice, including yellowish eyes and skin, skin itchiness, also tea-like color urine and DISCUSSION pale stool. The most general results of laboratory From USG results in this patient, there was examination was the presence of hyperbilirubinemia hyperechoic lesion appearance with diameter of 0.96 in patient. Other abnormalities were increased level cm with acoustic shadow in the gall bladder, dilatation of aminotransaminase enzymes and presence of of the intrahepatic bile duct, and dilated duct and leukocytosis, also often found acute cholecystitis, hyperechoic lesion with diameter of 1.16 cm with cholangitis, or pancreatitis. Lately, drastic increase acoustic shadow in CBD. Later, in ERCP results, of tumor marker (CA 19-9) was consistently found in there was dilated common bile duct and right and left patients with type II or higher type of Mirizzi syndrome. hepatic duct. There was also small image of stone in Consequently, CA19-9 has to be interpreted with CBD with a quite big acoustic shadow, which in further caution in patients with suspect of biliary malignancy, evaluation, it was suspected to be in the cystic duct. because if Mirizzi syndrome is not eliminated, patient Gall bladder was visualized accompanied with image might be diagnosed to suffer from gall bladder cancer. of stone within it and appearance of dilated cystic In this patient, there was corresponding abnormalities duct which gave impression of suppressing CBD. This in the laboratory results including increased of AST, ZDVLQOLQHZLWKWKHOLWHUDWXUHRQWKHFODVVL¿FDWLRQRI ALT, total bilirubin, direct bilirubin, indirect bilirubin, Mirizzi syndrome, which placed the patient in type I amylase, and lipase. Mirrizi Syndrome, which was external compression The differential diagnosis of Mirizzi syndrome of the bile duct due to the bile stone which adhere to was other obstructive jaundice, including gall bladder the infundibulum of the gall bladder of cyst duct. cancer, cholangiocarcinoma, pancreatic cancer, First mechanism explained that bile stone which metastatic disease, and others. In this patient, gall DGKHUHDQGLWVVHFRQGDU\LQÀDPPDWLRQSURFHVVPD\ bladder cancer, cholangiocarcinoma, and primary close cyst duct, the adhered bile stone will struggle to sclerosis cholangitis can be excluded because from enter into the bile duct and will lead to stress ulcer which the abdominal USG and ERCP, there was no mass in ZLOO¿QDOO\VFUDSHWKHZDOORIWKHJDOOEODGGHUDQGZDOO the wall of gall bladder, the result of CA19-9 was not RIWKHELOHGXFWDQGIRUPHG¿VWXODEHWZHHQOXPHQV increased, and there was no stricture in the intra or Mirizzi syndrome usually occurred at the average even extrahepatic duct. Similarly, pancreatic cancer DJHRI\HDUVROGZLWKPRVWSDWLHQWVZHUH can also be excluded because from the abdominal USG IHPDOHDSSUR[LPDWHO\RIDOOFDVHV+RZHYHU and ERCP, there was no mass in the pancreas and there this syndrome might happen in any age and in all was no increase in the CA 19-9 level. patients with bile stone. Chronic bile stone diseases, Stiff gall bladder accompanied with thickening or with the average of 29.6 years, have been reported as gall bladder with very thin wall accompanied with patients with Mirizzi syndrome. Mirizzi syndrome single large gall stone or even multiple small gall often present in acute form, however it can also be stones which adhered to the infundibulum may be seen. seen in chronic form. Although the clinical symptoms Dilated hepatic duct in the extrahepatic and intrahepatic RI0LUL]]LV\QGURPHLVXQVSHFL¿FWKHPRVWFRPPRQ above the obstruction site, and CBD will have normal form of the symptoms were obstructive jaundice size below the site of obstruction. Diagnostic accuracy DFFRPSDQLHGZLWKXSSHUDQGULJKWXSSHU UHSRUWHGWKURXJK86*LQ0LUL]]LV\QGURPHZDV TXDGUDQWDEGRPLQDOSDLQ DQGIHYHULQ ZLWKVHQVLWLYLW\RIWR4 In this patient, the patients which were known or suspected to suffer from results of the USG were the presence of acoustic bile stone disease. Previous jaundice history might be shadow which showed stone in gall bladder and in present. Frequently, patients with Mirizzi syndrome CBD, there was dilatation of the intrahepatic duct and came with acute cholecystitis, acute cholangitis, or above the site of CBD obstruction.
128 The Indonesian Journal of Gastroenterology, Hepatology and Digestive Endoscopy Mirizzi Syndrome in Gallstone Complication
0RUHWKDQSDWLHQWVZLWK0LUL]]LV\QGURPHZHUH sphingterectomy to be performed for stone extraction diagnosed during surgery. The surgical characteristics DQGIDFLOLWDWHRWKHULQWHUYHQWLRQVXFKDV VWHQWLQJRU include the narrowing of the gall bladder with changes placing nasobiliary tube or other procedures. Candidates or dilatation of the gall bladder with wall thickening IRUVXUJHU\ZKRDUH¿QDQFLDOO\GLVDGYDQWDJHGPLJKW and big stone, or multiple stones, impaction in the have interesting option with the use of ERCP to neck of gall bladder or infundibulum, Calot’s Triangle alleviate bile duct obstruction due to Mirizzi syndrome. closure, and dense adhesion from subhepatic space. 3DWLHQWZLWKFKRODQJLWLVPLJKWEHQH¿WIURPSUHVXUJHU\ ,QWUDRSHUDWLYHFKRODQJLRJUDSK\ZDVGLI¿FXOWWRSHUIRUP biliary drainage as temporary procedure before and persistent dissection in Calot’s Triangle area GH¿QLWLYHVXUJHU\*HQHUDOO\HQGRVFRS\PDQDJHPHQW may cause wound in the gall bladder. Intraoperative bile drainage, stone evacuation, and stenting. Standard ultrasonography is reported to be useful in identifying stone evacuation technique was usually performed, anatomy of biliary duct and help to accurate dissection including balloon facility, mechanic lithotripsy, and IURPELOLDU\GXFWLQWKHVLWHRILQÀDPPDWLRQ electrohydraulic litotripsi. However, the morbidity and Management of Mirizzi syndrome is important for mortality of ERCP have been known, also risk has to surgeons because presurgical diagnosis is not always EHZHLJKHGWRWKHEHQH¿WLQSDWLHQWVVXVSHFWHGWRVXIIHU possible, and because this surgical management is from Mirizzi syndrome. DVVRFLDWHGZLWKLQFUHDVHGVLJQL¿FDQWULVNIURPELOH In this patient, ERCP with sphingterectomy GXFWLQMXU\$GGLWLRQDOO\VHYHUHLQÀDPPDWRU\SURFHVV continued with ductal clearance using balloon and with thick, hard, dense adhesion and associated with basket were performed. Only several small stones tissue swelling. The presence of cholecystobiliary came out from the CBD, later patient was stented ¿VWXODLQFUHDVHVWKHULVNRIELOLDU\GXFWLQMXU\'XULQJ using double pigtail stent 10 fr 7 cm. After stenting, surgery, operating Calot’s Triangle may cause bile duct bile oozed. However, not all stones could be evacuated injury or excessive bleeding, also the appearance of due to impaction and were very risky, therefore it was RWKHUPRUELGLWLHVVXFKDV VHSVLVELOHGXFWVWULFWXUH decided to perform cholecystectomy. Due to evacuation and secondary biliary cirrhosis. Surgical management of bile stone in the cyst duct was not possible with of Mirizzi syndrome does not use the exact standard ERCP, patient was further being consulted for the and has to be tailored individually, depending on the consideration of elective cholecystectomy. Finally, to VLPSOLFLW\RIWKHFDVHDQGSUR¿FLHQF\RIWKHVXUJHRQV the patient, elective cholecystectomy was performed team. However, several guidelines may be pulled out after eleven days of hospitalization. and have been used for the last few years. Abdominal CT might identify gall bladder and REFERENCES measure wall thickness, and size of dilated duct. But, WKHSUHVHQFHRILQÀDPPDWLRQDURXQGWKHGXFWPD\ 1. /HVPDQD/$3HQ\DNLWEDWXHPSHGX,Q 6XGR\R$: Setyohadi B, Alwi I, Simadibrata M, Setiati S, eds. Buku be misinterpreted with the appearance of cancer. Ajar Ilmu Penyakit Dalam. 4thHG-DNDUWD 3XVDW3HQHUELWDQ 5DGLRORJ\LPDJLQJIURP&7LVQRWVSHFL¿F7KHPDLQ 'HSDUWHPHQ,OPX3HQ\DNLW'DODP).8,S use of CT is to exclude the possibility of malignancy 2. 6D¿ROHDV06WDPDWDNRV06D¿ROHDV36P\UQLV$5HYHQDV in the area of portal hepatic or in the liver.In this &6DYLROHV&0LUL]]LV\QGURPH DQXQH[SHFWHGSUREOHPRI cholelithiasis. Our experience with 27 cases. International patient abdominal CT was not performed, because 6HPLQDUVLQ6XUJLFDO2QFRORJ\S initially the presence of Mirizzi syndrome was not %HOWUDQ0$0LUL]]LV\QGURPH KLVWRU\FXUUHQWNQRZOZGJHDQG suspected in the previous physical examination and SURSRVDORIDVLPSOL¿HGFODVVL¿FDWLRQ:RUOG-*DVWURHQWHURO supporting examination. In the previous USG, only the appearance of stone in CBD was seen, therefore ERCP 4. =DOLHNDV-0XQVRQ/&RPSOLFDWLRQVRIJDOOVWRQHV WKH Mirizzi syndrome, gallstone ileus, gallstone pancreatitis, was performed to evacuate the stone. After ERCP was complications of “lost” gallstones. Surg Clin North America conducted, eventually the feature of Mirizzi syndrome was evident. 5. Memon M, Tahir SM, Ali A, Shaikh AR, Muneer A, Similar to abdominal CT, to this patient magnetic 6KDLNK1$0LUL]]LV\QGURPH DQXQXVXDOSUHVHQWDWLRQRI FKROHOLWKLDVLV5DZDO0HG- resonance cholangiopancreatography (MRCP) was not 6. Beltran MA, Csendes A, Cruces KS. The relationship of performed, other than initially the presence of Mirizzi 0LUL]]LV\QGURPHDQGFKROHF\VWRHQWHULF¿VWXOD YDOLGDWLRQ syndrome was not suspected, MRCP examination also RIPRGL¿HGFODVVL¿FDWLRQ:RUOG-6XUJ has lower diagnostic accuracy compared to ERCP. 7. Sanal HT, Kocaoglu M, Bulakbasi N. Mirizzi syndrome In addition to diagnostic tool, ERCP also allow LQDQLFWHULFSDWLHQW 05,DQG05&3¿QGLQJV-%5%75
Volume 15, Number 2, August 2014 129 Ario Perbowo, Dadang Makmun
Chatzoulis G, Kaltsas A, Danilidis L, Dimitriou J, Pachiadakis I. Mirizzi syndrome type IV associated with cholecystocolic ¿VWXOD DYHU\UDUHFRQGLWLRQ%0&6XUJ 9. Aydin U, Yazici P, Ozsan I, Ersoz G, Ozutemiz O, Zeytunlu M, et al. Surgical management of Mirizzi syndrome. Turk J *DVWURHQWHURO 10. Janes S, Berry L, Dijkstra B. Management of post cholecystectomy Mirizzi’s syndrome. J Minimal Access 6XUJ
Correspondence Dadang Makmun Division of Gastroenterology Department of Internal Medicine Dr. Cipto Mangunkusumo General National Hospital Jl. Diponegoro No.71 Jakarta Indonesia Phone: +62-21-3153957 Facsimile: +62-21-3142454 E-mail: [email protected]
130 The Indonesian Journal of Gastroenterology, Hepatology and Digestive Endoscopy