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Congenital Hepatic Fibrosis With Dilation of Intrahepatic Bile Ducts
GASTROJNTEROLOGY 71:839-843. 1976 Copyright @ 1976 by The Williams & Wilkins Co.
Vol. 71. No.5 Printed in U.S.A.
CONGENITAL HEPATIC FIBROSIS WITH DILATION OF INTRAHEPATIC BILE DUCTS A therapeutic approach M. DUSOL, JR., M.D., E. R. SCHIFF, M.D.
J.
U.
LEVI,
M.D., K. GLASSER, M.D.,
AND
Departments of Medicine and Surgery, University of Miami School of Medicine, Miami, Florida
A case of congenital hepatic fibrosis with dilation of the intrahepatic ducts is presented. External drainage and lavage of the biliary tract through a prosthetic "Y" tube and internal drainage accomplished by a Roux en-Y cholangiojejunostomy resulted in prevention of the patient's recurrent bouts of cholangitis and sepsis. Surgical therapy for recurrent cholangitis in this disease is reviewed. Congenital hepatic fibrosis is an infrequent cause of recurrent cholangitis and biliary tract stone formation. Approximately 15 cases of the disease in its pure form have been described. The recognition of this underlying entity in a patient with cholangitis is critical in the proper selection of a surgical reparative procedure. In this report, a case is described in which various surgical internal and external drainage procedures of the biliary tract were undertaken, which eventually resulted in the prevention of the patient's recurrent bouts of cholangitis. Case Report M. G., a 63-year-old Latin woman, presented to Jackson Memorial Hospital with a 3-day history of fever, shaking chills, vomiting, pruritus. and scleral icterus. Physical examination revealed a healthy-appearing female in mild distress; temperature 100°F, pulse 100, respirations 20, blood pressure 130/80. Scleral icterus and a tender. minimally enlarged liver were noted. Initial laboratory tests revealed the following: hemoglobin 13.5 g per 100 mi. hematocrit 39%. WBC 18,000 per mm" with 89% polymorphonuclear leukocytes; total protein 7.0 g per dl. albumin 3.8 g per dl. blood urea nitrogen 17 mg per dl. alkaline phosphatase 265 mIU per mi. and SGOT 45 mIU per ml. Serum a-fetoprotein and hepatitis B surface antigen were negative. Continuing abdominal pain and fever spikes to 102.8°F prompted an exploratory laparotomy, which revealed subacute cholecystitis with cholelithiasis. The surface of the left lobe was covered by multiple small cystic dilations. some ranging up to 20 mm in diameter. The right lobe demonstrated evidence of chronic cholestasis, but no cysts were apparent. A multitude of small granular common duct stones were palpated. The distal common bile duct measured 1 cm in diameter, but both the right and the left hepatic ducts were enlarged to 2 and 3 cm, respectively. Incision and biopsy of the Received August 29. 1975. Accepted May 5, 1976. Address reprint requests to: E. R. Schiff, M.D., Hepatology Section, Veterans Administration Hospital, 1201 N.W. 16th Street. Miami. Florida 33125.
cysts revealed white bile and multiple stones. There was no evidence of portal hypertension at operation, and portal pressure was not measured. Cultures for anaerobes and aerobes were subsequently negative. A cholecystectomy and common bile duct exploration with lavage in an attempt to clear the multitude of small intrahepatic stones and a sphincteroplasty to ensure biliary drainage were performed. Operative cholangiography was not performed. The kidneys were normal. A liver biopsy taken at surgery showed the typical features of congenital hepatic fibrosis with dilation of the intrahepatic bile ducts (figs. 1 and 2), After surgery the patient improved clinically only to develop recurrent fever of 103°F. leukocytosis, jaundice, and hypotension. Blood cultures grew Citrobacter and treatment with cephalothin and gentamicin was instituted. A transhepatic cholangiogram demonstrated dilation of the biliary radicals as well as multiple cystic lesions communicating with the biliary tree (fig. 3). Twelve days after the first operation. a second exploratory laparotomy was performed. Multiple stones and evidence of suppurative cholangitis were found. Pseudomonas aeruginasa was cultured from the bile. A number 18 prosthetic "Y" tube was inserted via a choledochotomy at the bifurcation of the hepatic ducts. The kidneys were palpated and found to be normal. Postoperatively antibiotic therapy was continued and the patient rapidly improved. The patient was clinically well for the next 5 weeks when the temperature spiked to 101°F and a rise of the alkaline phosphatase to 525 mIU per ml was noted. A Y-tube cholangiogram revealed a functional sphincteroplasty but persistence of multiple stones in a dilated biliary tree (fig. 4). In an attempt to provide more effective internal drainage, the patient was taken back to surgery and a Raux en-Y cholangiojejunostomy with a partial left hepatic resection was performed (fig. 5). Culture of the bile again grew Pseudomonas sensitive to gentamicin. The operative Y tube cholangiogram showed a patent anastamosis with good drainage through the common duct and Roux en-Y anastomosis. Daily saline irrigations via the Y tube were performed for 1 week with the intention of clearing the biliary tract of small calculi and draining the cystic dilations. Postoperatively the patient showed good resolution on
8:39
840
CASE REPORTS
Vol. 71, No.5
FIG. 1. Surgical liver biopsy demonstrating the classical picture of congenital hepatic fibrosis (H & E, > 16).
gentamiCin therapy. She was afebrile with a normal WBC count, a bilirubin of 1.6 mg per dl, and an alkaline phosphatase of 200 mIlJ per m!. Subsequent workup of the patient's genitourinary tract with an intravenous pyelogram showed no evidence of cystic disease of the kidneys. Two years since the last surgical procedure, with her permanent Y tube in place, the patient has remained asymptomatic and afebrile and has an excellent appetite. She has gained weight and resumed her previous daily activities. Bile cult ures have been repeatedly negative and since her last operation, chemistries reveal WBC count 7000 per mm', total bilirubin 0.6 mg per dl, SGOT 4,~ mIU per m!. and alkaline phosphata~e llii mIlJ per m!. The Y tube remains clamped and in place to provide a means of irrigating the patient's biliary tract, if ever necessary in the future.
Discussion Alt hough the entity has been recognized for centuries, the term "congenital hepatic fibrosis" was introduced into t he literature by Kerr in 1961. 1 Histologically the portal areas are hypertrophic and are surrounded by proliferating fihrous bands of tissue. These areas have heen referred to hy Popper and Schaffner as "multiple microhamartomas" and contain small dilated hile ducts. 2 The bile ducts and cysts communicate with the biliary tree and are lined by a normal cuboidal epithelium. 3 This disorder is apparently transmitted in an
autosomal recessive fashion in contrast to the adult form of polycystic liver disease, transmitted as an autosomal dominant. Four types of congenital hepatic fibrosis have been described: periportal fibrosis, cholangitic form, mixed form, and latent form. ,-1' There are two main histological varieties. The one originally described by Caroli is that without portal fibrosis, in which cystic dilation of the intrahepatic bile ducts is probably always present and cholangitis is the main clinical feature. 8. 13. 14 The other type is that with portal fibrosis, in which portal hypertension is frequent but cystic dilation of the intrahepatic bile ducts may occur and the clinical course may be complicated with cholangitis, as in this case. Although portal fibrosis was present in this case it was not associated with hepatocellular failure or portal hypertension. The lobular architecture is usually normal. There may be associated complications of hepatic and subphrenic abscesses, sepsis, or pericarditis. 8. 15 The disease is occasionally segmental and limited to one lobe, usually t he left lohe of the liver. 18 ' 18 It is important to recognize the presence of cystic dilation of the hiliary tree in an effort to prevent recurrent houts of cholangitis and to institute the appropriate surgical procedure. Both operative cholangiography and postoperative cholangiography through a
CASE REPURTS
November 1976
841
FIG. 2. Surgical liver hiopsy revealing one of Ihe multiple microcYNlic structures adjacent In the portal area, IH & E . . WI.
FIG. :~. P('rcutaneOlIs transhepat if cholangiogram demonst rating
man\' small
('~'stic
dilations of th,' intrahepatic hiliar\' tn'e.
T-tube predispose the patient to the development of cholangitis. I. Endoscopic retrograde cholangiopancreatography also may be attended by this complication. 20 Transhepatic cholangiography is both a safe and direct means of identifying this entity. Most clinicians feel that the indication for surgical intervention is evidence of obstruction by stones. 21 Nevertheless, once the entity has been diagnosed and recurrent bouts of life-threaten"ing cholangitis have occurred, some definitive surgical approach should be undertaken. Section of the sphincter of Oddi and sphincteroplasty have been proposed for the patients with recurrent cholangitis with the rationale of providing a larger conduit for the passage of small stones formed in the intrahepatic ducts. Our experience as well as that of others has shown that sphincteroplasty alone fails to provide adequate therapy. 7 Drainage of the common bile duct through a T-tube or a choledochoduodenostomy has not been universally successful. but did show improvement in 1 case. 21. Z2 A Roux en- Y cholangiojejunostomy is usually feasi ble because of the caliber of the enlarged ducts. 23 This procedure helps to minimize stasis and infection and also facilitates the evacuation of calculi from the intrahepatic cysts. A left lobe hepatectomy has led to the recovery of a patient with involvement of the intrahepatic hiliar~, tree
842
CASE REPORTS
Vol. 71, No.5
in the left 10be.17 Therefore, it appears that limited hepatic resection should be performed when possible if the disease is limited to one lobe, because the potential for recurrent cholangitis usually persists as long as cystic dilations remain. In our patient this was considered but would have required a hepatic resection with removal of both the medial and lateral segments of the left lobe. This would still not provide adequate drainage of stones that were impacted in the right side of the biliary tree. There are no reports in the literature of the use of the prosthetic Y tube in conjunction with a Raux en-Y cholangiojejunostomy as described in the present case. As in our patient, this combination of therapeutic approaches has led to marked improvement both clinically and chemically, and has prevented recurrent bouts of cholangitis. The retention and impaction of a multitude of stones and gravel in the biliary tree with recurrent cholangitis strongly negated the institution of a more conservative nonsurgical therapeutic approach. The advantage of a permanent Y tube is the allowance for a means of irrigating the biliary tract to clear biliary
FIG. 5. Schematic diagram indicating location of cystic dilations and reconstructive surgical procedures.
calculi and prevent recurrent cholangitis. Antibiotics alone often fail to control this condition. Finally, one should keep in mind when following these patients that cholangiocarcinoma and amyloidosis have been reported as late sequelae of congenital hepatic fibrosis. 24 - 26 REFERE:\( 'ES
FI(;.
L Y tutH..· c.:holangiogram fl·\'t·aling a dilated hiliar~' tree
t'oIllainillg three calculi Inrrou ."il.
1. Kerr D:-';S. Harrison, (,Y. Sherlock S. et al: Congenital hepatic fihrosis. Q.J Med :10:91-117.1961 2. Popper H. Schaffner F: Liver: Structure and Funnion. :\ew York. McGraw·Hill Book Co. 1957, p 586 :1. Potet F, Molas G, Feldmann G. et al: Prohlemes anatomocliniques poses par la fihrose hepatique congenitale. Cah !l.led 12:1015- JO:lO. 1971 4. Fauvert R. Henhamull .JI'. Meyer P: Fihrose hepatiqlle con· genitale. Re\' Fr Etlld C1in Hiol 9::17.', :177. 1964 f,. Schaffner F, Sherlock S. Leevy C!l.l: The Liver and Its Diseases. :\ew York. Stratton Intercontinental Medical H••• k ('orp. 1!cl74. p 28:1288 6. (;rumhach R. Hourillon .J, Allvert .JI': Maladie fihrokystique dll fnie avec hypertension portale chez )'{'nfant, deux observations. Sem Hop Arch Anat Pat hoi :IO:A7477. 19.~4 7. Erlinger S. Sakellaridis DA. !l.1aillard .J:\. et al: Les formes angiocholitiques de la fibrose hepatiqlJe congenitale. Presse Med 77: IIH9 119!. 1969 H. Caroli .J: Diseases of the intrahepati(' biliary tree. C1in Gastro"n· ternl 2:1·IH1:,:I. 197:1 9. Beau A. Prevnt .J. Manciallx M. et al: t'n nouwau cas dt' malformation polykystique congenitale des voies hiliares .. J ('hir IParisl flO:H2 .';61. 19fiO 10. Vic·DlIpont MM. Mignot .J. Halle H: Dilatation kystique des ,"oies hiliares intrahepatiques poussees d'angiocholit,> echelonnees sur trente ans cirrhose terminalI'. So(' !l.led Hop Paris 74:4274:\:1. 19,,8
November 1976
CASE REPORTS
11. Kerneis JP, Ferron A de, Gordeef A, et al: La fibro angioadenomatose du foie avec hypertension portale. II. AnaOOmie, patbologique, physiopathologie. n08Ologie. Presse Med 69: 14061409, 1961
12. Le Tan Vinh: Hypertension portale par lesion hepatique con-
13.
14.
15.
16. 17.
genitale, sea rapports avec la maladie kystique du foie et les alterations du foie au cours de la mucoviscidose. Actual Pediat (Paris) 4: 185-213, 1962 Caroli J, Soupault R, Kossakowski J, et al: La dilatation polykystique congenitale des voies biliares intrahepatiques. Esssi de classification. 8em Hop Paris 34:488-495, 1958 Caroli J, Corcos V: Maladies des Voies Biliares Intrahepatiques 8egmentaires. Paris, Masson 1964, p 59-127 Murray-Lyon 1M, 8hilkin KB, Laws JW. et al: Nonobstructive dilatation of the intrahepatic biliary tree with cholangitis. Q J Med 164:477-489, 1972 Caroli J, Couihaud C: Une effection nouvelle, 88n1l doote congenitale, des voies biliares. La dilatation kystique unilobaire des cahaux hapatiques. Sem Hop Paris 34:136-143,1958 Helou E, Farah M, Nastier W: Hepatectomie gauche pour dilatation congenitale des voies biJiares intrahepatiques. Rev Int Hepa00115:1295-1304, 1965
843
18. Mercadier M. Clot JP, Mellie", D, et al: Dilatation congenitale des voies biliares intrahepatiques. 8em Hop Paris 44:3283-3288. 1968
19. Clermont RJ. Maillard IN. Benhamou JP. et al: Fibrose hepatique congenitale. Can Med Assoc: J 97: 1272-1278. 1967 20. Waldram R. Vahrman J. Williams R: Salmonella Heidelberg infection in Caroli's syndrome. Gastroenterology 68:151-153.1975 21. Rosewarne MD: Cystic dilatation of the intrahepatic bile ducts. Br J Radiol 45:825-827. 1972 22. Guillemin G. Marcy J. Naudin E, et al: La dilatation kystique congenitale des voies biliares intraphepatiques segmentaires. Arch Mal Appar Dig Mal Nutr 54:797-811. 1965 23. Longmire WP Jr. Sanford MC: Intrahepatic cholangiojejunosoomy with partial hepatectomy for biliary obstruction. Ann Surg 130:455-460. 1949 24. Gallagher PJ. Millis RR, Mitchi90n MJ: Congenital dilatation of
the intrahepatic ducts with choillngiocarcinoma. J Clin Pathol 25:804-808. 19i2 25. Jones AW. Shreeve DR: Congenital dilatation of intrahepatic biliary ducts with cholangiocarcinoma. Br Med J 2:227-278, 1970
26. Fevery J. Tanghe R. Kerremans V, et al: Congenital dilatation of the intrahepatic bile ducts associated with the development of amyloid08is. Gut 13:604--r09. 1972
Vol. 71. No.5 Printed in U.S.A.
CONGENITAL HEPATIC FIBROSIS WITH DILATION OF INTRAHEPATIC BILE DUCTS A therapeutic approach M. DUSOL, JR., M.D., E. R. SCHIFF, M.D.
J.
U.
LEVI,
M.D., K. GLASSER, M.D.,
AND
Departments of Medicine and Surgery, University of Miami School of Medicine, Miami, Florida
A case of congenital hepatic fibrosis with dilation of the intrahepatic ducts is presented. External drainage and lavage of the biliary tract through a prosthetic "Y" tube and internal drainage accomplished by a Roux en-Y cholangiojejunostomy resulted in prevention of the patient's recurrent bouts of cholangitis and sepsis. Surgical therapy for recurrent cholangitis in this disease is reviewed. Congenital hepatic fibrosis is an infrequent cause of recurrent cholangitis and biliary tract stone formation. Approximately 15 cases of the disease in its pure form have been described. The recognition of this underlying entity in a patient with cholangitis is critical in the proper selection of a surgical reparative procedure. In this report, a case is described in which various surgical internal and external drainage procedures of the biliary tract were undertaken, which eventually resulted in the prevention of the patient's recurrent bouts of cholangitis. Case Report M. G., a 63-year-old Latin woman, presented to Jackson Memorial Hospital with a 3-day history of fever, shaking chills, vomiting, pruritus. and scleral icterus. Physical examination revealed a healthy-appearing female in mild distress; temperature 100°F, pulse 100, respirations 20, blood pressure 130/80. Scleral icterus and a tender. minimally enlarged liver were noted. Initial laboratory tests revealed the following: hemoglobin 13.5 g per 100 mi. hematocrit 39%. WBC 18,000 per mm" with 89% polymorphonuclear leukocytes; total protein 7.0 g per dl. albumin 3.8 g per dl. blood urea nitrogen 17 mg per dl. alkaline phosphatase 265 mIU per mi. and SGOT 45 mIU per ml. Serum a-fetoprotein and hepatitis B surface antigen were negative. Continuing abdominal pain and fever spikes to 102.8°F prompted an exploratory laparotomy, which revealed subacute cholecystitis with cholelithiasis. The surface of the left lobe was covered by multiple small cystic dilations. some ranging up to 20 mm in diameter. The right lobe demonstrated evidence of chronic cholestasis, but no cysts were apparent. A multitude of small granular common duct stones were palpated. The distal common bile duct measured 1 cm in diameter, but both the right and the left hepatic ducts were enlarged to 2 and 3 cm, respectively. Incision and biopsy of the Received August 29. 1975. Accepted May 5, 1976. Address reprint requests to: E. R. Schiff, M.D., Hepatology Section, Veterans Administration Hospital, 1201 N.W. 16th Street. Miami. Florida 33125.
cysts revealed white bile and multiple stones. There was no evidence of portal hypertension at operation, and portal pressure was not measured. Cultures for anaerobes and aerobes were subsequently negative. A cholecystectomy and common bile duct exploration with lavage in an attempt to clear the multitude of small intrahepatic stones and a sphincteroplasty to ensure biliary drainage were performed. Operative cholangiography was not performed. The kidneys were normal. A liver biopsy taken at surgery showed the typical features of congenital hepatic fibrosis with dilation of the intrahepatic bile ducts (figs. 1 and 2), After surgery the patient improved clinically only to develop recurrent fever of 103°F. leukocytosis, jaundice, and hypotension. Blood cultures grew Citrobacter and treatment with cephalothin and gentamicin was instituted. A transhepatic cholangiogram demonstrated dilation of the biliary radicals as well as multiple cystic lesions communicating with the biliary tree (fig. 3). Twelve days after the first operation. a second exploratory laparotomy was performed. Multiple stones and evidence of suppurative cholangitis were found. Pseudomonas aeruginasa was cultured from the bile. A number 18 prosthetic "Y" tube was inserted via a choledochotomy at the bifurcation of the hepatic ducts. The kidneys were palpated and found to be normal. Postoperatively antibiotic therapy was continued and the patient rapidly improved. The patient was clinically well for the next 5 weeks when the temperature spiked to 101°F and a rise of the alkaline phosphatase to 525 mIU per ml was noted. A Y-tube cholangiogram revealed a functional sphincteroplasty but persistence of multiple stones in a dilated biliary tree (fig. 4). In an attempt to provide more effective internal drainage, the patient was taken back to surgery and a Raux en-Y cholangiojejunostomy with a partial left hepatic resection was performed (fig. 5). Culture of the bile again grew Pseudomonas sensitive to gentamicin. The operative Y tube cholangiogram showed a patent anastamosis with good drainage through the common duct and Roux en-Y anastomosis. Daily saline irrigations via the Y tube were performed for 1 week with the intention of clearing the biliary tract of small calculi and draining the cystic dilations. Postoperatively the patient showed good resolution on
8:39
840
CASE REPORTS
Vol. 71, No.5
FIG. 1. Surgical liver biopsy demonstrating the classical picture of congenital hepatic fibrosis (H & E, > 16).
gentamiCin therapy. She was afebrile with a normal WBC count, a bilirubin of 1.6 mg per dl, and an alkaline phosphatase of 200 mIlJ per m!. Subsequent workup of the patient's genitourinary tract with an intravenous pyelogram showed no evidence of cystic disease of the kidneys. Two years since the last surgical procedure, with her permanent Y tube in place, the patient has remained asymptomatic and afebrile and has an excellent appetite. She has gained weight and resumed her previous daily activities. Bile cult ures have been repeatedly negative and since her last operation, chemistries reveal WBC count 7000 per mm', total bilirubin 0.6 mg per dl, SGOT 4,~ mIU per m!. and alkaline phosphata~e llii mIlJ per m!. The Y tube remains clamped and in place to provide a means of irrigating the patient's biliary tract, if ever necessary in the future.
Discussion Alt hough the entity has been recognized for centuries, the term "congenital hepatic fibrosis" was introduced into t he literature by Kerr in 1961. 1 Histologically the portal areas are hypertrophic and are surrounded by proliferating fihrous bands of tissue. These areas have heen referred to hy Popper and Schaffner as "multiple microhamartomas" and contain small dilated hile ducts. 2 The bile ducts and cysts communicate with the biliary tree and are lined by a normal cuboidal epithelium. 3 This disorder is apparently transmitted in an
autosomal recessive fashion in contrast to the adult form of polycystic liver disease, transmitted as an autosomal dominant. Four types of congenital hepatic fibrosis have been described: periportal fibrosis, cholangitic form, mixed form, and latent form. ,-1' There are two main histological varieties. The one originally described by Caroli is that without portal fibrosis, in which cystic dilation of the intrahepatic bile ducts is probably always present and cholangitis is the main clinical feature. 8. 13. 14 The other type is that with portal fibrosis, in which portal hypertension is frequent but cystic dilation of the intrahepatic bile ducts may occur and the clinical course may be complicated with cholangitis, as in this case. Although portal fibrosis was present in this case it was not associated with hepatocellular failure or portal hypertension. The lobular architecture is usually normal. There may be associated complications of hepatic and subphrenic abscesses, sepsis, or pericarditis. 8. 15 The disease is occasionally segmental and limited to one lobe, usually t he left lohe of the liver. 18 ' 18 It is important to recognize the presence of cystic dilation of the hiliary tree in an effort to prevent recurrent houts of cholangitis and to institute the appropriate surgical procedure. Both operative cholangiography and postoperative cholangiography through a
CASE REPURTS
November 1976
841
FIG. 2. Surgical liver hiopsy revealing one of Ihe multiple microcYNlic structures adjacent In the portal area, IH & E . . WI.
FIG. :~. P('rcutaneOlIs transhepat if cholangiogram demonst rating
man\' small
('~'stic
dilations of th,' intrahepatic hiliar\' tn'e.
T-tube predispose the patient to the development of cholangitis. I. Endoscopic retrograde cholangiopancreatography also may be attended by this complication. 20 Transhepatic cholangiography is both a safe and direct means of identifying this entity. Most clinicians feel that the indication for surgical intervention is evidence of obstruction by stones. 21 Nevertheless, once the entity has been diagnosed and recurrent bouts of life-threaten"ing cholangitis have occurred, some definitive surgical approach should be undertaken. Section of the sphincter of Oddi and sphincteroplasty have been proposed for the patients with recurrent cholangitis with the rationale of providing a larger conduit for the passage of small stones formed in the intrahepatic ducts. Our experience as well as that of others has shown that sphincteroplasty alone fails to provide adequate therapy. 7 Drainage of the common bile duct through a T-tube or a choledochoduodenostomy has not been universally successful. but did show improvement in 1 case. 21. Z2 A Roux en- Y cholangiojejunostomy is usually feasi ble because of the caliber of the enlarged ducts. 23 This procedure helps to minimize stasis and infection and also facilitates the evacuation of calculi from the intrahepatic cysts. A left lobe hepatectomy has led to the recovery of a patient with involvement of the intrahepatic hiliar~, tree
842
CASE REPORTS
Vol. 71, No.5
in the left 10be.17 Therefore, it appears that limited hepatic resection should be performed when possible if the disease is limited to one lobe, because the potential for recurrent cholangitis usually persists as long as cystic dilations remain. In our patient this was considered but would have required a hepatic resection with removal of both the medial and lateral segments of the left lobe. This would still not provide adequate drainage of stones that were impacted in the right side of the biliary tree. There are no reports in the literature of the use of the prosthetic Y tube in conjunction with a Raux en-Y cholangiojejunostomy as described in the present case. As in our patient, this combination of therapeutic approaches has led to marked improvement both clinically and chemically, and has prevented recurrent bouts of cholangitis. The retention and impaction of a multitude of stones and gravel in the biliary tree with recurrent cholangitis strongly negated the institution of a more conservative nonsurgical therapeutic approach. The advantage of a permanent Y tube is the allowance for a means of irrigating the biliary tract to clear biliary
FIG. 5. Schematic diagram indicating location of cystic dilations and reconstructive surgical procedures.
calculi and prevent recurrent cholangitis. Antibiotics alone often fail to control this condition. Finally, one should keep in mind when following these patients that cholangiocarcinoma and amyloidosis have been reported as late sequelae of congenital hepatic fibrosis. 24 - 26 REFERE:\( 'ES
FI(;.
L Y tutH..· c.:holangiogram fl·\'t·aling a dilated hiliar~' tree
t'oIllainillg three calculi Inrrou ."il.
1. Kerr D:-';S. Harrison, (,Y. Sherlock S. et al: Congenital hepatic fihrosis. Q.J Med :10:91-117.1961 2. Popper H. Schaffner F: Liver: Structure and Funnion. :\ew York. McGraw·Hill Book Co. 1957, p 586 :1. Potet F, Molas G, Feldmann G. et al: Prohlemes anatomocliniques poses par la fihrose hepatique congenitale. Cah !l.led 12:1015- JO:lO. 1971 4. Fauvert R. Henhamull .JI'. Meyer P: Fihrose hepatiqlle con· genitale. Re\' Fr Etlld C1in Hiol 9::17.', :177. 1964 f,. Schaffner F, Sherlock S. Leevy C!l.l: The Liver and Its Diseases. :\ew York. Stratton Intercontinental Medical H••• k ('orp. 1!cl74. p 28:1288 6. (;rumhach R. Hourillon .J, Allvert .JI': Maladie fihrokystique dll fnie avec hypertension portale chez )'{'nfant, deux observations. Sem Hop Arch Anat Pat hoi :IO:A7477. 19.~4 7. Erlinger S. Sakellaridis DA. !l.1aillard .J:\. et al: Les formes angiocholitiques de la fibrose hepatiqlJe congenitale. Presse Med 77: IIH9 119!. 1969 H. Caroli .J: Diseases of the intrahepati(' biliary tree. C1in Gastro"n· ternl 2:1·IH1:,:I. 197:1 9. Beau A. Prevnt .J. Manciallx M. et al: t'n nouwau cas dt' malformation polykystique congenitale des voies hiliares .. J ('hir IParisl flO:H2 .';61. 19fiO 10. Vic·DlIpont MM. Mignot .J. Halle H: Dilatation kystique des ,"oies hiliares intrahepatiques poussees d'angiocholit,> echelonnees sur trente ans cirrhose terminalI'. So(' !l.led Hop Paris 74:4274:\:1. 19,,8
November 1976
CASE REPORTS
11. Kerneis JP, Ferron A de, Gordeef A, et al: La fibro angioadenomatose du foie avec hypertension portale. II. AnaOOmie, patbologique, physiopathologie. n08Ologie. Presse Med 69: 14061409, 1961
12. Le Tan Vinh: Hypertension portale par lesion hepatique con-
13.
14.
15.
16. 17.
genitale, sea rapports avec la maladie kystique du foie et les alterations du foie au cours de la mucoviscidose. Actual Pediat (Paris) 4: 185-213, 1962 Caroli J, Soupault R, Kossakowski J, et al: La dilatation polykystique congenitale des voies biliares intrahepatiques. Esssi de classification. 8em Hop Paris 34:488-495, 1958 Caroli J, Corcos V: Maladies des Voies Biliares Intrahepatiques 8egmentaires. Paris, Masson 1964, p 59-127 Murray-Lyon 1M, 8hilkin KB, Laws JW. et al: Nonobstructive dilatation of the intrahepatic biliary tree with cholangitis. Q J Med 164:477-489, 1972 Caroli J, Couihaud C: Une effection nouvelle, 88n1l doote congenitale, des voies biliares. La dilatation kystique unilobaire des cahaux hapatiques. Sem Hop Paris 34:136-143,1958 Helou E, Farah M, Nastier W: Hepatectomie gauche pour dilatation congenitale des voies biJiares intrahepatiques. Rev Int Hepa00115:1295-1304, 1965
843
18. Mercadier M. Clot JP, Mellie", D, et al: Dilatation congenitale des voies biliares intrahepatiques. 8em Hop Paris 44:3283-3288. 1968
19. Clermont RJ. Maillard IN. Benhamou JP. et al: Fibrose hepatique congenitale. Can Med Assoc: J 97: 1272-1278. 1967 20. Waldram R. Vahrman J. Williams R: Salmonella Heidelberg infection in Caroli's syndrome. Gastroenterology 68:151-153.1975 21. Rosewarne MD: Cystic dilatation of the intrahepatic bile ducts. Br J Radiol 45:825-827. 1972 22. Guillemin G. Marcy J. Naudin E, et al: La dilatation kystique congenitale des voies biliares intraphepatiques segmentaires. Arch Mal Appar Dig Mal Nutr 54:797-811. 1965 23. Longmire WP Jr. Sanford MC: Intrahepatic cholangiojejunosoomy with partial hepatectomy for biliary obstruction. Ann Surg 130:455-460. 1949 24. Gallagher PJ. Millis RR, Mitchi90n MJ: Congenital dilatation of
the intrahepatic ducts with choillngiocarcinoma. J Clin Pathol 25:804-808. 19i2 25. Jones AW. Shreeve DR: Congenital dilatation of intrahepatic biliary ducts with cholangiocarcinoma. Br Med J 2:227-278, 1970
26. Fevery J. Tanghe R. Kerremans V, et al: Congenital dilatation of the intrahepatic bile ducts associated with the development of amyloid08is. Gut 13:604--r09. 1972