DISTAL COMMON BILE DUCT STRICTURE AND SECONDARY. BILIARY CIRRHOSIS DUE TO CHOLEDOCHOLITHIASIS Timothy C. Simmons, MD Inglewood, California
This case report documents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis. Endoscopic or surgical treatment of choledocholithiasis must be considered early in treatment. (J NatI Med Assoc. 1991 ;83:1105-1108.) Key words * choledocholithiasis * bile duct stricture * secondary biliary cirrhosis This article presents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis. The recurrent episodes of cholangitis and the development of a CBD stricture appeared to have accelerated the onset of biliary cirrhosis. Because the CBD stricture could not be dilated to a diameter that would have allowed safe endoscopic stone extraction, surgical management was required. A delay in diagnosis of recurrent or retained choledocholithiasis was, in part, responsible for the untoward sequelae that developed in this patient.
CASE REPORT A 39-year-old white woman presented to a commu-
nity hospital in July 1985 with jaundice. An abdominal From the West Gastroenterology Group and Digestive Disease Centers of Inglewood and Los Angeles, California, and the Daniel Freeman Hospital, Inglewood, California. Requests for reprints should be addressed to Dr Timothy C. Simmons, 633 Aerick St, Inglewood, CA 90301. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
ultrasound revealed extrahepatic biliary dilation, cholecystolithiasis, and choledocholithiasis. The CBD measured 16 mm at its greatest diameter. Cholecystectomy, CBD exploration, and T-tube insertion were performed. An elongated end of the T-tube extended to the level of Vater's papilla; however, unobstructed flow of contrast media into the duodenum was noted at cholangiography. A repeat T-tube cholangiogram, obtained 1 month after surgery, demonstrated prompt emptying of contrast into the duodenum and no retained CBD stones. The T-tube was subsequently removed. The patient remained asymptomatic until January 1986, when biliary colic, chills, fever, intense pruritus, and jaundice recurred. During the following 10 months, she had four episodes of cholangitis. On each occurrence, ampicillin therapy effectively resolved her fever, pruritus, and jaundice. Although an outpatient abdominal ultrasound in May 1986 revealed extrahepatic biliary dilation and retained CBD stones, she was not referred for endoscopic or surgical therapy. In late November 1986, a recurrent episode of chills, fever, acholic stools, jaundice, and abdominal discomfort was noted by the patient. In December, she was admitted to the Daniel Freeman Hospital in Inglewood, California. On admission, she was afebrile and her physical examination disclosed icterus, right upper quadrant abdominal tenderness, and a right subcostal surgical scar. Laboratory tests revealed a white blood cell count of 10 300 per mm3 with 15% bands, serum bilrubin of 5.3 mg/dL (range: 0 to 1.2), serum alkaline phosphatase of 1300 mU/mL (range: 30 to 105), serum aspartate aminotransferase of 97 mU/mL (range: 10 to 40), serum 1105
CHOLEDOCHOLITHIASIS
Figure 1. ERCP demonstration of distal CBD stricture at the open arrow and dilation of proximal CBD.
Figure 2. Nasobiliary tube (small arrow) in the CBD and demonstration of choledocholithia sis at the open arrow.
alanine aminotransferase of 91 mU/mL (range: 10 to 44), and gamma-glutamyl transpeptidase of 810 mU/ mL (range: 15 to 85).
involving the distal CBD at its intrapancreatic portion was observed. Needle aspirations of the CBD mass and an enlarged periductal node were benign at rapid frozen section analysis. Because uncertainty remained with regard to excluding cholangiocarcinoma, a Whipple procedure and liver biopsy were performed. Gross examination of the CBD (open through its length) revealed a dilated proximal and mid-portion of 12 mm and a sharply narrowed segment at its distal end. The effect of prior papillotomy also was noted. The mucosal surface of the CBD was intact throughout its course. Gross sectional study disclosed no suspicious area of induration or discoloration. Microscopic analysis of multiple sections of the CBD showed an intact mucosal surface. The submucosal aspect of the distal CBD stricture revealed focally dense fibrous tissue deposits (Figure 3). Features suggesting overt malignancy were not identified. Microscopic evaluation of the liver biopsy showed bile stasis throughout, disruption of lobular architecture, increased amounts of portal and periportal fibrosis, active regeneration, and an increased number of bile ducts (Figures 4A-B). An increase in chronic inflamma-
An abdominal ultrasound showed a dilated CBD to 13 mm and choledocholithiasis. Endoscopic retrograde cholangiopancreatography (Figure 1) disclosed a 4-cm stricture of the distal CBD, dilation of the proximal CBD and intrahepatic ducts, and choledocholithiasis. Papillotomy and balloon dilation of the stricture were performed. However, the CBD stricture could not be dilated to a diameter that would have allowed safe stone extraction. A nasobiliary tube was inserted (Figure 2) for bile duct decompression and CBD stone dissolution therapy. After a mono-octanoin infusion failed to dissolve the CBD stones, the patient was referred for surgery. At abdominal exploration, the liver was massively enlarged with the right lobe extending below the umbilicus. The tail, body, and head of the pancreas were normal. Extensive scarring of the hepatoduodenal ligament and thickening of the common bile duct were noted. The proximal CBD was dilated with an internal diameter of 12 mm. A firm, rubbery, 4-cm X 2-cm mass 1106
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CHOLEDOCHOLITHIASIS
tory cells and prominence of Kupffer's cells also were noted. Infiltrative or malignant cells were not identified. Following surgery, the patient had an uneventful 2-week postoperative hospital course and was discharged. She remained asymptomatic at follow-up evaluations at 6, 12, 18, and 24 months. At the 6-month follow-up, most liver function tests were within normal limits. Hyperphosphatasemia (alkaline phosphatase >700 mU/mL) was noted at each follow-up visit.
DISCUSSION This patient presented with choledocholithiasis and a CBD stricture. It is of interest that the 1-month postoperative cholangiogram was normal; thus, the choledocholithiasis and the CBD stricture appeared to have developed within 1 year after the initial surgery. Choledocholithiasis and recurrent episodes of cholangitis may have caused this patient's CBD stricture; however, CBD trauma, either occurring during the initial surgery or as a result of T-tube injury, has been reported as the most common cause of CBD strictures.1 The development of choledocholithiasis within 10 months of cholecystectomy and CBD exploration is unusual although not uncommon. In more than 50% of patients who obtain complete gallstone dissolution with bile acids, gallstones recur within 2 years of discontinuing cholelitholytic therapy.2 In addition, CBD stones recur in 1.2% to 2% of patients who undergo endoscopic choledocholithectomy.3,4 Although the development of choledocholithiasis after cholecystectomy in this patient is an interesting speculation, a missed diagnosis of retained CBD stones most likely occurred. Safrany et a14 and others3'5 have clearly shown that endoscopic sphincterotomy is the procedure of choice for the management of postcholecystectomy choledocholithiasis. Nevertheless, this procedure was either not offered or not available to this patient when she was initially diagnosed with retained or recurrent CBD stones. Common bile duct stenosis due to chronic pancreatitis is well established.6-8 In patients with chronic pancreatitis, Littenberg et a18 found a strong correlation between persistent hyperphosphatasemia (alkaline phosphatase > twice normal for 4 weeks or longer) and significant CBD stenosis. Warshaw et a19 noted that without biliary decompression, these patients are at risk for developing secondary biliary cirrhosis. Similarly, Scobid and Summerskill'0 reported that secondary biliary cirrhosis can occur after prolonged bile duct obstruction due to postoperative stricture, choledocholithiasis, and malignant biliary obstruction. After effectively decompressing the biliary tree by endoJOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
Figure 3. Scanning view photomicrograph of the CBD cross section at level of pancreas (H & E, original magnification x40). Note the dense fibrous tissue deposits at the stricture level (arrow). (P=pancreas, L=bile duct lumen.) scopic or surgical means, persistent hyperphosphatasemia may suggest irreversible secondary liver injury, as observed in the patient reported here. Secondary biliary cirrhosis occurs more commonly following benign rather than malignant biliary obstruction.10'11 However, the time interval between biliary obstruction and cirrhosis varies widely between benign and malignant obstruction10: 7.1 years (range: 0.5 to 26) for benign stricture, 4.6 years (range: 0.3 to 17) for choledocholithiasis, and 0.8 (range: 0.2 to 1.5) for malignant stricture. In addition, benign biliary stricture and chronic cholangitis, as noted in the patient reported here, hasten the development of secondary biliary cirrhosis.12 Although patients with malignant biliary obstruction usually die from complications of their cancer before significant secondary biliary cirrhosis develops, in patients with choledocholithiasis, early surgical or endoscopic management conceivably can prevent recurrent episodes of cholangitis, biliary stricture, and secondary biliary cirrhosis. 1107
CHOLEDOCHOLITHIASIS
Figure 4A. Noodle biopsy of the liver revealing cholestasis (arrows) and disruption of lobular architecture (H & E. original magnification
Figure 4B. Photomicrograph of the liver de monstrating fibrosis with bridging (arrows) and active regeneration (H & E, original magnification x 100).
This patient presented with recurrent attacks of cholangitis, choledocholithiasis, and a distal CBD stricture. Histological documentation of secondary biliary ciffhosis was an unexpected but not uncommon finding. A Whipple procedure was performed because the surgeon could not totally exclude cholangiocarcinoma at the time of abdominal exploration. Although the patient's postoperative course has been uneventful, she remains at risk from potential complications of secondary biliary cirrhosis. Early endoscopic or surgical therapy of choledocholithiasis may prevent the untoward sequelae of this condition.
5. Simmons TC, Gletten F, Henderson DR. Endoscopic sphincterotomy in the management of benign and malignant extrahepatic biliary obstruction. J Natl Med Assoc. 1989;81 :421-429. 6. Scott J, Summerfield JA, Elias E, Dick R, Sherlock S. Chronic pancreatitis: a cause of cholestasis. Gut. 1977;1 8:196201. 7. Snape WJ, Long WB, Trotman BW, Marin GA, Czaja AJ. Marked alkaline phosphatase elevation with partial common bile duct obstruction due to calcific pancreatitis. Gastroenterology. 1976;70:70-73. 8. Littenberg G, Afroudakis A, Kaplowitz N. Common bile duct stenosis from chronic pancreatitis: a clinical and pathologic spectrum. Medicine (Baltimore). 1979;58:385-412. 9. Warshaw AL, Shapiro RH, Ferrucci JT, Galdabini JJ. Persistent obstructive jaundice, cholangitis, and biliary cirrhosis due to common bile duct stenosis in chronic pancreatitis. Gastroenterology. 1976;70:562-567. 10. Scobid BA, Summerskill WHJ. Hepatic cirrhosis secondary to obstruction of the biliary system. American Journal of Digestive Diseases. 1965;1 0:1 35-146. 1 1. Gibson WR, Robertson HE. So-called biliary cirrhosis. Arch Pathol. 1939;28:37-48. 12. Blumgart LH, Kelley CJ, Benjamin IS. Benign bile duct stricture following cholecystectomy: critical factors in management. Br J Surg. 1984;71:836-843.
x1I00).
Literature Cited 1. Way LW, Dunphy JE. Biliary stricture. Am J Surg. 1 972; 1 24:287-295. 2. Ruppin DC, Dowling RH. Is recurrence inevitable after gallstone dissolution by bile-acid treatment? Lancet. 1982;1 :181-1 85. 3. Kawai K, Nakajima M. Present status and complications of EST in Japan. Endoscopy. 1983; 15:169-172. 4. Safrany L, Schott B, Balint T. Endoscopic sphincterotomy: the long term results in choledocholithiasis.
Gastrointest Endosc. 1982;28:152. Abstract. 1108
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
This case report documents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis. Endoscopic or surgical treatment of choledocholithiasis must be considered early in treatment. (J NatI Med Assoc. 1991 ;83:1105-1108.) Key words * choledocholithiasis * bile duct stricture * secondary biliary cirrhosis This article presents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis. The recurrent episodes of cholangitis and the development of a CBD stricture appeared to have accelerated the onset of biliary cirrhosis. Because the CBD stricture could not be dilated to a diameter that would have allowed safe endoscopic stone extraction, surgical management was required. A delay in diagnosis of recurrent or retained choledocholithiasis was, in part, responsible for the untoward sequelae that developed in this patient.
CASE REPORT A 39-year-old white woman presented to a commu-
nity hospital in July 1985 with jaundice. An abdominal From the West Gastroenterology Group and Digestive Disease Centers of Inglewood and Los Angeles, California, and the Daniel Freeman Hospital, Inglewood, California. Requests for reprints should be addressed to Dr Timothy C. Simmons, 633 Aerick St, Inglewood, CA 90301. JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
ultrasound revealed extrahepatic biliary dilation, cholecystolithiasis, and choledocholithiasis. The CBD measured 16 mm at its greatest diameter. Cholecystectomy, CBD exploration, and T-tube insertion were performed. An elongated end of the T-tube extended to the level of Vater's papilla; however, unobstructed flow of contrast media into the duodenum was noted at cholangiography. A repeat T-tube cholangiogram, obtained 1 month after surgery, demonstrated prompt emptying of contrast into the duodenum and no retained CBD stones. The T-tube was subsequently removed. The patient remained asymptomatic until January 1986, when biliary colic, chills, fever, intense pruritus, and jaundice recurred. During the following 10 months, she had four episodes of cholangitis. On each occurrence, ampicillin therapy effectively resolved her fever, pruritus, and jaundice. Although an outpatient abdominal ultrasound in May 1986 revealed extrahepatic biliary dilation and retained CBD stones, she was not referred for endoscopic or surgical therapy. In late November 1986, a recurrent episode of chills, fever, acholic stools, jaundice, and abdominal discomfort was noted by the patient. In December, she was admitted to the Daniel Freeman Hospital in Inglewood, California. On admission, she was afebrile and her physical examination disclosed icterus, right upper quadrant abdominal tenderness, and a right subcostal surgical scar. Laboratory tests revealed a white blood cell count of 10 300 per mm3 with 15% bands, serum bilrubin of 5.3 mg/dL (range: 0 to 1.2), serum alkaline phosphatase of 1300 mU/mL (range: 30 to 105), serum aspartate aminotransferase of 97 mU/mL (range: 10 to 40), serum 1105
CHOLEDOCHOLITHIASIS
Figure 1. ERCP demonstration of distal CBD stricture at the open arrow and dilation of proximal CBD.
Figure 2. Nasobiliary tube (small arrow) in the CBD and demonstration of choledocholithia sis at the open arrow.
alanine aminotransferase of 91 mU/mL (range: 10 to 44), and gamma-glutamyl transpeptidase of 810 mU/ mL (range: 15 to 85).
involving the distal CBD at its intrapancreatic portion was observed. Needle aspirations of the CBD mass and an enlarged periductal node were benign at rapid frozen section analysis. Because uncertainty remained with regard to excluding cholangiocarcinoma, a Whipple procedure and liver biopsy were performed. Gross examination of the CBD (open through its length) revealed a dilated proximal and mid-portion of 12 mm and a sharply narrowed segment at its distal end. The effect of prior papillotomy also was noted. The mucosal surface of the CBD was intact throughout its course. Gross sectional study disclosed no suspicious area of induration or discoloration. Microscopic analysis of multiple sections of the CBD showed an intact mucosal surface. The submucosal aspect of the distal CBD stricture revealed focally dense fibrous tissue deposits (Figure 3). Features suggesting overt malignancy were not identified. Microscopic evaluation of the liver biopsy showed bile stasis throughout, disruption of lobular architecture, increased amounts of portal and periportal fibrosis, active regeneration, and an increased number of bile ducts (Figures 4A-B). An increase in chronic inflamma-
An abdominal ultrasound showed a dilated CBD to 13 mm and choledocholithiasis. Endoscopic retrograde cholangiopancreatography (Figure 1) disclosed a 4-cm stricture of the distal CBD, dilation of the proximal CBD and intrahepatic ducts, and choledocholithiasis. Papillotomy and balloon dilation of the stricture were performed. However, the CBD stricture could not be dilated to a diameter that would have allowed safe stone extraction. A nasobiliary tube was inserted (Figure 2) for bile duct decompression and CBD stone dissolution therapy. After a mono-octanoin infusion failed to dissolve the CBD stones, the patient was referred for surgery. At abdominal exploration, the liver was massively enlarged with the right lobe extending below the umbilicus. The tail, body, and head of the pancreas were normal. Extensive scarring of the hepatoduodenal ligament and thickening of the common bile duct were noted. The proximal CBD was dilated with an internal diameter of 12 mm. A firm, rubbery, 4-cm X 2-cm mass 1106
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
CHOLEDOCHOLITHIASIS
tory cells and prominence of Kupffer's cells also were noted. Infiltrative or malignant cells were not identified. Following surgery, the patient had an uneventful 2-week postoperative hospital course and was discharged. She remained asymptomatic at follow-up evaluations at 6, 12, 18, and 24 months. At the 6-month follow-up, most liver function tests were within normal limits. Hyperphosphatasemia (alkaline phosphatase >700 mU/mL) was noted at each follow-up visit.
DISCUSSION This patient presented with choledocholithiasis and a CBD stricture. It is of interest that the 1-month postoperative cholangiogram was normal; thus, the choledocholithiasis and the CBD stricture appeared to have developed within 1 year after the initial surgery. Choledocholithiasis and recurrent episodes of cholangitis may have caused this patient's CBD stricture; however, CBD trauma, either occurring during the initial surgery or as a result of T-tube injury, has been reported as the most common cause of CBD strictures.1 The development of choledocholithiasis within 10 months of cholecystectomy and CBD exploration is unusual although not uncommon. In more than 50% of patients who obtain complete gallstone dissolution with bile acids, gallstones recur within 2 years of discontinuing cholelitholytic therapy.2 In addition, CBD stones recur in 1.2% to 2% of patients who undergo endoscopic choledocholithectomy.3,4 Although the development of choledocholithiasis after cholecystectomy in this patient is an interesting speculation, a missed diagnosis of retained CBD stones most likely occurred. Safrany et a14 and others3'5 have clearly shown that endoscopic sphincterotomy is the procedure of choice for the management of postcholecystectomy choledocholithiasis. Nevertheless, this procedure was either not offered or not available to this patient when she was initially diagnosed with retained or recurrent CBD stones. Common bile duct stenosis due to chronic pancreatitis is well established.6-8 In patients with chronic pancreatitis, Littenberg et a18 found a strong correlation between persistent hyperphosphatasemia (alkaline phosphatase > twice normal for 4 weeks or longer) and significant CBD stenosis. Warshaw et a19 noted that without biliary decompression, these patients are at risk for developing secondary biliary cirrhosis. Similarly, Scobid and Summerskill'0 reported that secondary biliary cirrhosis can occur after prolonged bile duct obstruction due to postoperative stricture, choledocholithiasis, and malignant biliary obstruction. After effectively decompressing the biliary tree by endoJOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
Figure 3. Scanning view photomicrograph of the CBD cross section at level of pancreas (H & E, original magnification x40). Note the dense fibrous tissue deposits at the stricture level (arrow). (P=pancreas, L=bile duct lumen.) scopic or surgical means, persistent hyperphosphatasemia may suggest irreversible secondary liver injury, as observed in the patient reported here. Secondary biliary cirrhosis occurs more commonly following benign rather than malignant biliary obstruction.10'11 However, the time interval between biliary obstruction and cirrhosis varies widely between benign and malignant obstruction10: 7.1 years (range: 0.5 to 26) for benign stricture, 4.6 years (range: 0.3 to 17) for choledocholithiasis, and 0.8 (range: 0.2 to 1.5) for malignant stricture. In addition, benign biliary stricture and chronic cholangitis, as noted in the patient reported here, hasten the development of secondary biliary cirrhosis.12 Although patients with malignant biliary obstruction usually die from complications of their cancer before significant secondary biliary cirrhosis develops, in patients with choledocholithiasis, early surgical or endoscopic management conceivably can prevent recurrent episodes of cholangitis, biliary stricture, and secondary biliary cirrhosis. 1107
CHOLEDOCHOLITHIASIS
Figure 4A. Noodle biopsy of the liver revealing cholestasis (arrows) and disruption of lobular architecture (H & E. original magnification
Figure 4B. Photomicrograph of the liver de monstrating fibrosis with bridging (arrows) and active regeneration (H & E, original magnification x 100).
This patient presented with recurrent attacks of cholangitis, choledocholithiasis, and a distal CBD stricture. Histological documentation of secondary biliary ciffhosis was an unexpected but not uncommon finding. A Whipple procedure was performed because the surgeon could not totally exclude cholangiocarcinoma at the time of abdominal exploration. Although the patient's postoperative course has been uneventful, she remains at risk from potential complications of secondary biliary cirrhosis. Early endoscopic or surgical therapy of choledocholithiasis may prevent the untoward sequelae of this condition.
5. Simmons TC, Gletten F, Henderson DR. Endoscopic sphincterotomy in the management of benign and malignant extrahepatic biliary obstruction. J Natl Med Assoc. 1989;81 :421-429. 6. Scott J, Summerfield JA, Elias E, Dick R, Sherlock S. Chronic pancreatitis: a cause of cholestasis. Gut. 1977;1 8:196201. 7. Snape WJ, Long WB, Trotman BW, Marin GA, Czaja AJ. Marked alkaline phosphatase elevation with partial common bile duct obstruction due to calcific pancreatitis. Gastroenterology. 1976;70:70-73. 8. Littenberg G, Afroudakis A, Kaplowitz N. Common bile duct stenosis from chronic pancreatitis: a clinical and pathologic spectrum. Medicine (Baltimore). 1979;58:385-412. 9. Warshaw AL, Shapiro RH, Ferrucci JT, Galdabini JJ. Persistent obstructive jaundice, cholangitis, and biliary cirrhosis due to common bile duct stenosis in chronic pancreatitis. Gastroenterology. 1976;70:562-567. 10. Scobid BA, Summerskill WHJ. Hepatic cirrhosis secondary to obstruction of the biliary system. American Journal of Digestive Diseases. 1965;1 0:1 35-146. 1 1. Gibson WR, Robertson HE. So-called biliary cirrhosis. Arch Pathol. 1939;28:37-48. 12. Blumgart LH, Kelley CJ, Benjamin IS. Benign bile duct stricture following cholecystectomy: critical factors in management. Br J Surg. 1984;71:836-843.
x1I00).
Literature Cited 1. Way LW, Dunphy JE. Biliary stricture. Am J Surg. 1 972; 1 24:287-295. 2. Ruppin DC, Dowling RH. Is recurrence inevitable after gallstone dissolution by bile-acid treatment? Lancet. 1982;1 :181-1 85. 3. Kawai K, Nakajima M. Present status and complications of EST in Japan. Endoscopy. 1983; 15:169-172. 4. Safrany L, Schott B, Balint T. Endoscopic sphincterotomy: the long term results in choledocholithiasis.
Gastrointest Endosc. 1982;28:152. Abstract. 1108
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 83, NO. 12
