Recurrent pyogenic cholangitis
- Hannah Lee, MD
Hannah Lee, MD
- Assistant Professor of Medicine
- Virginia Commonwealth University
- Medical College of Virginia
- Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
- Director, Liver Care Network and Organ Care Research
- Swedish Medical Center
- Seattle, WA
- Section Editor
- Sanjiv Chopra, MD, MACP
Sanjiv Chopra, MD, MACP
- Editor-in-Chief — Gastroenterology and Hepatology
- Section Editor — General Hepatology; Gallbladder and Biliary Tract Disease
- Professor of Medicine
- Harvard Medical School
- Senior Consultant in Hepatology
- James Tullis Firm Chief
- Beth Israel Deaconess Medical Center
Recurrent pyogenic cholangitis is a disease characterized by intrabiliary pigment stone formation, resulting in stricturing of the biliary tree and biliary obstruction with recurrent bouts of cholangitis. Various other names have been used since the first description in 1930, including Hong Kong disease, biliary obstruction syndrome of the Chinese, Oriental cholangitis, and Oriental infestational cholangitis . The lack of a uniform nomenclature complicates direct comparison of studies.
Recurrent pyogenic cholangitis is found almost exclusively in people who live or who have lived in Southeast Asia [2-7]. However, the incidence in the West appears to be increasing due to the immigration of people from endemic countries. It occurs in equal frequency in men and women with a peak prevalence in the third and fourth decades of life, although it can also be seen in patients over the age of 60 [8-11]. It is more common in the rural population and in lower socioeconomic groups.
Recurrent pyogenic cholangitis is characterized by recurrent cholangitis caused by bile stasis and stone formation proximal to biliary strictures. The pathogenesis of the stone formation and biliary abnormalities is incompletely understood. Stone formation occurs de novo within the intrahepatic bile ducts in contrast with the more common pattern of stone formation within the gallbladder seen in patients with more common forms of gallstone-related disease.
Transient portal bacteremia is thought to introduce bacteria into the biliary ducts, initiating a cycle of infection and secondary stone formation which lead to further obstruction and infection. Common organisms cultured from bile include E. coli, Klebsiella, Pseudomonas, and Proteus species and, less frequently, anaerobes, although the culture of multiple organisms is common [8,12]. Abnormal phospholipid metabolism has also been implicated . (See "Inherited disorders associated with conjugated hyperbilirubinemia".)
The bile ducts are markedly abnormal, characterized by extrahepatic and intrahepatic ductal dilatation with focal areas of stricturing in the intrahepatic biliary tree. The biliary wall is fibrotic with inflammatory cell infiltration. The bile is purulent and filled with debris composed of bile pigment, desquamated epithelial cells, bacteria, and pus.
Subscribers log in hereLiterature review current through: Aug 2017. | This topic last updated: Jan 17, 2017.References
- Digby K. Common duct stones of liver origin. Br J Sur 1930; 17:578.
- Nakayama F, Furusawa T, Nakama T. Hepatolithiasis in Japan: present status. Am J Surg 1980; 139:216.
- GLENN F, MOODY FG. Intrahepatic calculi. Ann Surg 1961; 153:711.
- Miyake H, Johnston CG. Gallstones: ethnological studies. Digestion 1968; 1:219.
- Nakayama F, Soloway RD, Nakama T, et al. Hepatolithiasis in East Asia. Retrospective study. Dig Dis Sci 1986; 31:21.
- Ker CG, Huang TJ, Sheen PC. [Intrahepatic stones. 1. Etiological study (author's transl)]. Taiwan Yi Xue Hui Za Zhi 1981; 80:698.
- ONG GB. A study of recurrent pyogenic cholangitis. Arch Surg 1962; 84:199.
- Carmona RH, Crass RA, Lim RC Jr, Trunkey DD. Oriental cholangitis. Am J Surg 1984; 148:117.
- Yellin AE, Donovan AJ. Biliary lithiasis and helminthiasis. Am J Surg 1981; 142:128.
- Lim JH. Oriental cholangiohepatitis: pathologic, clinical, and radiologic features. AJR Am J Roentgenol 1991; 157:1.
- Chou ST, Chan CW. Recurrent pyogenic cholangitis: a necropsy study. Pathology 1980; 12:415.
- Seel DJ, Park YK. Oriental infestational cholangitis. Am J Surg 1983; 146:366.
- Shoda J, Inada Y, Osuga T. Molecular pathogenesis of hepatolithiasis--a type of low phospholipid-associated cholelithiasis. Front Biosci 2006; 11:669.
- Cosenza CA, Durazo F, Stain SC, et al. Current management of recurrent pyogenic cholangitis. Am Surg 1999; 65:939.
- Lim JH. Radiologic findings of clonorchiasis. AJR Am J Roentgenol 1990; 155:1001.
- Huang MH, Chen CH, Yen CM, et al. Relation of hepatolithiasis to helminthic infestation. J Gastroenterol Hepatol 2005; 20:141.
- Nakayama F, Koga A. Hepatolithiasis: present status. World J Surg 1984; 8:9.
- Nagase M, Tanimura H, Setoyama M, Hikasa Y. Present features of gallstones in Japan. A collective review of 2,144 cases. Am J Surg 1978; 135:788.
- MAKI T. Cholelithiasis in the Japanese. Arch Surg 1961; 82:599.
- Bain VG, Ardao GH, Kowalewska-Grochowska K, et al. Biliary ascariasis. J Clin Gastroenterol 1988; 10:448.
- Schulman A. Non-western patterns of biliary stones and the role of ascariasis. Radiology 1987; 162:425.
- Cetta FM. Bile infection documented as initial event in the pathogenesis of brown pigment biliary stones. Hepatology 1986; 6:482.
- Spivak W, DiVenuto D, Yuey W. Non-enzymic hydrolysis of bilirubin mono- and diglucuronide to unconjugated bilirubin in model and native bile systems. Potential role in the formation of gallstones. Biochem J 1987; 242:323.
- MATSUSHIRO T. IDENTIFICATION OF GLUCARO-1,4-LACTONE IN BILE AS A FACTOR RESPONSIBLE FOR INHIBITORY EFFECT OF BILE ON BACTERIAL BETA-GLUCURONIDASE. Tohoku J Exp Med 1965; 85:330.
- Ostrow JD. The etiology of pigment gallstones. Hepatology 1984; 4:215S.
- Matsushiro T, Suzuki N, Sato T, Maki T. Effects of diet on glucaric acid concentration in bile and the formation of calcium bilirubinate gallstones. Gastroenterology 1977; 72:630.
- Hikasa Y, Nagase M, Tanimura H, et al. Epidemiology and etiólogy of gallstones. Nihon Geka Hokan 1980; 49:555.
- Matsumoto Y, Fujii H, Yoshioka M, et al. Biliary strictures as a cause of primary intrahepatic bile duct stones. World J Surg 1986; 10:867.
- Lindelöf G, Van der Linden W. The role of stasis in experimental gallstone formation. Acta Chir Scand 1965; 130:494.
- Trotman BW, Soloway RD. Pigment gallstone disease: Summary of the National Institutes of Health--international workshop. Hepatology 1982; 2:879.
- IMAMOGLU K, PERRY JF Jr, WANGENSTEEN OH. [Experimental production of gallstones by incomplete stricture of the terminal common bile duct]. Surgery 1957; 42:623.
- IMAMOGLU K, YONEHIRO EG, PERRY JF Jr, WANGENSTEEN OH. Formation of calculi following cholecystectomy attending partial occlusions of the common bile duct. Surg Forum 1957; 8:225.
- Sperling RM, Koch J, Sandhu JS, Cello JP. Recurrent pyogenic cholangitis in Asian immigrants to the United States: natural history and role of therapeutic ERCP. Dig Dis Sci 1997; 42:865.
- Lim JH, Ko YT, Lee DH, Hong KS. Oriental cholangiohepatitis: sonographic findings in 48 cases. AJR Am J Roentgenol 1990; 155:511.
- Chan FL, Man SW, Leong LL, Fan ST. Evaluation of recurrent pyogenic cholangitis with CT: analysis of 50 patients. Radiology 1989; 170:165.
- Choi TK, Wong J. Endoscopic retrograde cholangiopancreatography and endoscopic papillotomy in recurrent pyogenic cholangitis. Clin Gastroenterol 1986; 15:393.
- Khuroo MS, Dar MY, Yattoo GN, et al. Serial cholangiographic appearances in recurrent pyogenic cholangitis. Gastrointest Endosc 1993; 39:674.
- Safar F, Kamura T, Okamuto K, et al. Magnetic resonance T1 gradient-echo imaging in hepatolithiasis. Abdom Imaging 2005; 30:297.
- Jain M, Agarwal A. MRCP findings in recurrent pyogenic cholangitis. Eur J Radiol 2008; 66:79.
- Katabathina VS, Dasyam AK, Dasyam N, Hosseinzadeh K. Adult bile duct strictures: role of MR imaging and MR cholangiopancreatography in characterization. Radiographics 2014; 34:565.
- Wani NA, Robbani I, Kosar T. MRI of oriental cholangiohepatitis. Clin Radiol 2011; 66:158.
- Lam SK, Wong KP, Chan PK, et al. Recurrent pyogenic cholangitis: a study by endoscopic retrograde cholangiography. Gastroenterology 1978; 74:1196.
- Kartheuser AH, Dozois RR, Wiesner RH, et al. Complications and risk factors after ileal pouch-anal anastomosis for ulcerative colitis associated with primary sclerosing cholangitis. Ann Surg 1993; 217:314.
- Mahadeva S, Prabakharan R, Goh KL. Endoscopic intervention for hepatolithiasis associated with sharp angulation of right intrahepatic ducts. Gastrointest Endosc 2003; 58:279.
- Fan ST, Choi TK, Wong J. Recurrent pyogenic cholangitis: current management. World J Surg 1991; 15:248.
- Tanaka M, Ikeda S, Ogawa Y, et al. Divergent effects of endoscopic sphincterotomy on the long-term outcome of hepatolithiasis. Gastrointest Endosc 1996; 43:33.
- Yoshida J, Chijiiwa K, Shimizu S, et al. Hepatolithiasis: outcome of cholangioscopic lithotomy and dilation of bile duct stricture. Surgery 1998; 123:421.
- Sheen-Chen SM, Chou FF, Lee CM, et al. The management of complicated hepatolithiasis with intrahepatic biliary stricture by the combination of T-tube tract dilation and endoscopic electrohydraulic lithotripsy. Gastrointest Endosc 1993; 39:168.
- Chen C, Huang M, Yang J, et al. Reappraisal of percutaneous transhepatic cholangioscopic lithotomy for primary hepatolithiasis. Surg Endosc 2005; 19:505.
- Fan ST, Mok F, Zheng SS, et al. Appraisal of hepaticocutaneous jejunostomy in the management of hepatolithiasis. Am J Surg 1993; 165:332.
- Okugawa T, Tsuyuguchi T, K C S, et al. Peroral cholangioscopic treatment of hepatolithiasis: Long-term results. Gastrointest Endosc 2002; 56:366.
- Gott PE, Tieva MH, Barcia PJ, Laberge JM. Biliary access procedure in the management of oriental cholangiohepatitis. Am Surg 1996; 62:930.
- Cheng YF, Lee TY, Sheen-Chen SM, et al. Treatment of complicated hepatolithiasis with intrahepatic biliary stricture by ductal dilatation and stenting: long-term results. World J Surg 2000; 24:712.
- Jan YY, Chen MF, Wang CS, et al. Surgical treatment of hepatolithiasis: long-term results. Surgery 1996; 120:509.
- Strichartz SD, Abedin MZ, Ippoliti AF, et al. Intrahepatic cholesterol stones: a rationale for dissolution therapy. Gastroenterology 1991; 100:228.
- Dumont M, Erlinger S, Uchman S. Hypercholeresis induced by ursodeoxycholic acid and 7-ketolithocholic acid in the rat: possible role of bicarbonate transport. Gastroenterology 1980; 79:82.
- Renner EL, Lake JR, Cragoe EJ Jr, et al. Ursodeoxycholic acid choleresis: relationship to biliary HCO-3 and effects of Na+-H+ exchange inhibitors. Am J Physiol 1988; 254:G232.
- Erlinger S. Hypercholeretic bile acids: a clue to the mechanism? Hepatology 1990; 11:888.
- Kusano T, Isa TT, Muto Y, et al. Long-term results of hepaticojejunostomy for hepatolithiasis. Am Surg 2001; 67:442.
- Sun WB, Han BL, Cai JX. The surgical treatment of isolated left-sided hepatolithiasis: a 22-year experience. Surgery 2000; 127:493.
- Stain SC, Incarbone R, Guthrie CR, et al. Surgical treatment of recurrent pyogenic cholangitis. Arch Surg 1995; 130:527.
- Choi TK. Intrahepatic stones. Br J Surg 1989; 76:213.
- He XD, Liu W, Li BL, et al. Combined surgical therapy for hepatolithiasis. Chin Med Sci J 2005; 20:123.
- Cheung MT, Kwok PC. Liver resection for intrahepatic stones. Arch Surg 2005; 140:993.
- Li X, Shi L, Wang Y, Tian FZ. Middle and long-term clinical outcomes of patients with regional hepatolithiasis after subcutaneous tunnel and hepatocholangioplasty with utilization of the gallbladder. Hepatobiliary Pancreat Dis Int 2005; 4:597.
- Vetrone G, Ercolani G, Grazi GL, et al. Surgical therapy for hepatolithiasis: a Western experience. J Am Coll Surg 2006; 202:306.
- Uenishi T, Hamba H, Takemura S, et al. Outcomes of hepatic resection for hepatolithiasis. Am J Surg 2009; 198:199.
- Co M, Pang SY, Wong KY, et al. Surgical management of recurrent pyogenic cholangitis: 10 years of experience in a tertiary referral centre in Hong Kong. HPB (Oxford) 2014; 16:776.
- Al-Sukhni W, Gallinger S, Pratzer A, et al. Recurrent pyogenic cholangitis with hepatolithiasis--the role of surgical therapy in North America. J Gastrointest Surg 2008; 12:496.
- Tang CN, Tai CK, Siu WT, et al. Laparoscopic treatment of recurrent pyogenic cholangitis. J Hepatobiliary Pancreat Surg 2005; 12:243.
- Tang CN, Tai CK, Ha JP, et al. Laparoscopy versus open left lateral segmentectomy for recurrent pyogenic cholangitis. Surg Endosc 2005; 19:1232.
- Yang T, Lau WY, Lai EC, et al. Hepatectomy for bilateral primary hepatolithiasis: a cohort study. Ann Surg 2010; 251:84.
- Yoon HK, Sung KB, Song HY, et al. Benign biliary strictures associated with recurrent pyogenic cholangitis: treatment with expandable metallic stents. AJR Am J Roentgenol 1997; 169:1523.
- Chijiiwa K, Yamashita H, Yoshida J, et al. Current management and long-term prognosis of hepatolithiasis. Arch Surg 1995; 130:194.
- Otani K, Shimizu S, Chijiiwa K, et al. Comparison of treatments for hepatolithiasis: hepatic resection versus cholangioscopic lithotomy. J Am Coll Surg 1999; 189:177.
- Yamakawa T, Komaki F, Kitano Y, et al. Intrahepatic stones and postoperative choledochoscopy. Gastroenterol Jpn 1980; 15:577.
- Strong RW, Chew SP, Wall DR, et al. Liver transplantation for hepatolithiasis. Asian J Surg 2002; 25:180.
- Pan GD, Yan LN, Li B, et al. Liver transplantation for patients with hepatolithiasis. Hepatobiliary Pancreat Dis Int 2005; 4:345.
- Saing H, Chan KL, Mya GH, et al. Cutaneous stoma in the roux limb of hepaticojejunostomy (hepaticocutaneous jejunostomy): useful access for intrahepatic stone extraction. J Pediatr Surg 1996; 31:247.
- Chen MF, Jan YY, Wang CS, et al. A reappraisal of cholangiocarcinoma in patient with hepatolithiasis. Cancer 1993; 71:2461.