Recurrent Pyogenic Cholangitis Clinical Presentation
- Author: Willis Parsons, MD; Chief Editor: Julian Katz, MD more...
History
Patients may present with the following: (1) an acute attack of cholangitis, (2) a history of recurrent attacks of cholangitis typified by fevers and right upper quadrant (RUQ) abdominal pain, or (3) complications of pyogenic cholangitis. As the disease progresses, patients may develop cholangiocarcinoma and present with constitutional symptoms, including weight loss, easy fatigability, and jaundice.[1, 2]
- Roughly one third of patients present with an initial episode of RPC. The typical presentation is a patient older than 30 years who is from an endemic region and reports with complaints of fevers, RUQ abdominal pain, and jaundice (Charcot triad). Atypical presentations without all of the components of the triad are somewhat infrequent but may confound the diagnosis, and a high index of suspicion for RPC in the appropriate setting cannot be overemphasized.
- Patients who have experienced recurrent episodes typically report 1-2 episodes of fevers, jaundice, and RUQ abdominal pain per year and a history of prior biliary surgery, endoscopic procedures, or percutaneous biliary drainage procedures.
- Patients may present to the hospital with any sequelae of the complications of RPC, including gram-negative bacteremia/sepsis and organ failure. Perforations of the bile duct can occur with rupture into potential spaces, including the pericardium and the peritoneum. Acute pancreatitis is an infrequent but often-described consequence of recurrent bouts of pyogenic cholangitis. In patients presenting with acute pancreatitis, the pain may be in the epigastrium, as opposed to the RUQ. A pyogenic hepatic abscess may be one presentation of RPC, as is biliary malignancy.
Physical
No specific physical findings are evident in RPC. The history is cardinal in prompting the diagnosis.
- Patients often appear ill, frequently are jaundiced, and usually have tenderness in the RUQ of the abdomen. Not infrequently in RPC, upon careful physical examination, an enlarged tender gallbladder can be palpated in the RUQ.
- Other findings are specific to local and systemic complications of RPC, which can include the following:
- Rupture into the peritoneum can precipitate an acute abdomen.
- Rupture or fistulization into the abdominal wall often presents with pus drainage from cutaneous fistulae.
- Rupture into the pericardium may present with tamponade.
Causes
The underlying mechanism of RPC is unclear. Most experts believe that RPC is initiated by helminthic infection of the bile ducts and/or sludge/stone formation from deficient glucuronidation as a consequence of profound malnutrition. The initial insult(s) to the bile ducts precipitates a cycle of biliary stone formation and infection that results in recurrent episodes of pyogenic cholangitis.
Jeyarajah DR. Recurrent pyogenic cholangitis. Curr Treat Options Gastroenterol. Apr 2004;7(2):91-98. [Medline].
Bass N. Sclerosing Cholangitis and Recurrent Pyogenic Cholangitis. In: Feldman M, Scharschmidt B, Slesinger M, eds. Gastrointestinal and Liver Disease. Vol 1. Philadelphia, Pa: WB Saunders; 1993:1006-25.
Chan FL, Chan JK, Leong LL. Modern imaging in the evaluation of hepatolithiasis. Hepatogastroenterology. Mar-Apr 1997;44(14):358-69. [Medline].
Jain M, Agarwal A. MRCP findings in recurrent pyogenic cholangitis. Eur J Radiol. Apr 2008;66(1):79-83. [Medline].
Heffernan EJ, Geoghegan T, Munk PL, Ho SG, Harris AC. Recurrent pyogenic cholangitis: from imaging to intervention. AJR Am J Roentgenol. Jan 2009;192(1):W28-35. [Medline]. [Full Text].
Al-Sukhni W, Gallinger S, Pratzer A, Wei A, Ho CS, Kortan P, et al. Recurrent pyogenic cholangitis with hepatolithiasis--the role of surgical therapy in North America. J Gastrointest Surg. Mar 2008;12(3):496-503. [Medline].
Lee KF, Chong CN, Ng D, et al. Outcome of surgical treatment for recurrent pyogenic cholangitis: a single-centre study. HPB (Oxford). 2009;11(1):75-80. [Medline]. [Full Text].
Afagh A, Pancu D. Radiologic findings in recurrent pyogenic cholangitis. J Emerg Med. Apr 2004;26(3):343-6. [Medline].
American College of Surgeons. Surgical education and self-assessment program (SESAP). Recurrent pyogenic cholangitis. Can J Surg. Apr 2004;47(2):121. [Medline].
Carmona RH, Crass RA, Lim RC Jr, Trunkey DD. Oriental cholangitis. Am J Surg. Jul 1984;148(1):117-24. [Medline].
Chen MF, Jan YY, Wang CS, et al. A reappraisal of cholangiocarcinoma in patient with hepatolithiasis. Cancer. Apr 15 1993;71(8):2461-5. [Medline].
Chen PH, Lo HW, Wang CS, et al. Cholangiocarcinoma in hepatolithiasis. J Clin Gastroenterol. Dec 1984;6(6):539-47. [Medline].
Cheng YF, Lee TY, Sheen-Chen SM, Huang TL, Chen TY. Treatment of complicated hepatolithiasis with intrahepatic biliary stricture by ductal dilatation and stenting: long-term results. World J Surg. Jun 2000;24(6):712-6. [Medline].
Chijiiwa K, Yamashita H, Yoshida J, Kuroki S, Tanaka M. Current management and long-term prognosis of hepatolithiasis. Arch Surg. Feb 1995;130(2):194-7. [Medline].
Cosenza CA, Durazo F, Stain SC, Jabbour N, Selby RR. Current management of recurrent pyogenic cholangitis. Am Surg. Oct 1999;65(10):939-43. [Medline].
Digby K. Common duct stones of liver origin. Br J Surg. 1930;17:578.
Koga A, Ichimiya H, Yamaguchi K, Miyazaki K, Nakayama F. Hepatolithiasis associated with cholangiocarcinoma. Possible etiologic significance. Cancer. Jun 15 1985;55(12):2826-9. [Medline].
Lam SK. A study of endoscopic sphincterotomy in recurrent pyogenic cholangitis. Br J Surg. Apr 1984;71(4):262-6. [Medline].
Lee SK, Seo DW, Myung SJ, Park ET, Lim BC, Kim HJ, et al. Percutaneous transhepatic cholangioscopic treatment for hepatolithiasis: an evaluation of long-term results and risk factors for recurrence. Gastrointest Endosc. Mar 2001;53(3):318-23. [Medline].
Lee Y, Lee BH, Park JH, Suh CH. Balloon dilatation of intrahepatic biliary strictures for percutaneous extraction of residual intrahepatic stones. Cardiovasc Intervent Radiol. Mar-Apr 1991;14(2):102-5. [Medline].
Lim JH. Oriental cholangiohepatitis: pathologic, clinical, and radiologic features. AJR Am J Roentgenol. Jul 1991;157(1):1-8. [Medline].
Min HK. Clonorchis sinensis: pathogenesis and clinical features of infection. Arzneimittelforschung. 1984;34(9B):1151-3. [Medline].
Nakanuma Y, Terada T, Tanaka Y, Ohta G. Are hepatolithiasis and cholangiocarcinoma aetiologically related? A morphological study of 12 cases of hepatolithiasis associated with cholangiocarcinoma. Virchows Arch A Pathol Anat Histopathol. 1985;406(1):45-58. [Medline].
Ng WT. Pathogenesis of acute cholecystitis and recurrent pyogenic cholangitis. Surg Laparosc Endosc Percutan Tech. Aug 2004;14(4):240-1. [Medline].
Saing H, Chan KL, Mya GH, Cheng W, Fan ST, Chan FL. Cutaneous stoma in the roux limb of hepaticojejunostomy (hepaticocutaneous jejunostomy): useful access for intrahepatic stone extraction. J Pediatr Surg. Feb 1996;31(2):247-50. [Medline].
Schulman A. Intrahepatic biliary stones: imaging features and a possible relationship with ascaris lumbricoides. Clin Radiol. May 1993;47(5):325-32. [Medline].
Sheen-Chen S, Chen W, Eng H, Sheen C, Chou F, Cheng Y, et al. Bacteriology and antimicrobial choice in hepatolithiasis. Am J Infect Control. Aug 2000;28(4):298-301. [Medline].
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. Apr 1997;42(4):865-71. [Medline].
Stain SC, Incarbone R, Guthrie CR, Ralls PW, Rivera-Lara S, Parekh D, et al. Surgical treatment of recurrent pyogenic cholangitis. Arch Surg. May 1995;130(5):527-32; discussion 532-3. [Medline].
Tanaka M, Ikeda S, Ogawa Y, et al. Divergent effects of endoscopic sphincterotomy on the long-term outcome of hepatolithiasis. Gastrointest Endosc. Jan 1996;43(1):33-7. [Medline].
Tang CN, Tai CK, Ha JP, Siu WT, Tsui KK, Li MK. Laparoscopy versus open left lateral segmentectomy for recurrent pyogenic cholangitis. Surg Endosc. Sep 2005;19(9):1232-6. [Medline].
Tang CN, Tai CK, Siu WT, Ha JP, Tsui KK, Li MK. Laparoscopic treatment of recurrent pyogenic cholangitis. J Hepatobiliary Pancreat Surg. 2005;12(3):243-8. [Medline].
Wong WT, Teoh-Chan CH, Huang CT, Cheng FC, Ong GB. The bacteriology of recurrent pyogenic cholangitis and associated diseases. J Hyg (Lond). Dec 1981;87(3):407-12. [Medline].

