Scandinavian Journal of Gastroenterology
ISSN: 0036-5521 (Print) 1502-7708 (Online) Journal homepage: http://www.tandfonline.com/loi/igas20
Bile Duct Cysts in Adults: Pitfalls in Diagnosis and Management A. Bakka, A. Bergan & O. Søreide To cite this article: A. Bakka, A. Bergan & O. Søreide (1991) Bile Duct Cysts in Adults: Pitfalls in Diagnosis and Management, Scandinavian Journal of Gastroenterology, 26:2, 197-206, DOI: 10.3109/00365529109025031 To link to this article: http://dx.doi.org/10.3109/00365529109025031
Published online: 08 Jul 2009.
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Date: 30 March 2016, At: 02:44
Bile Duct Cysts in Adults Pitfalls in Diagnosis and Management A. BAKKA, A. BERGAN & 0. SBREIDE Surgical Dept. B, Rikshospitalet, Oslo, Norway
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Bakka A , Bergan A, Soreide 0. Bile duct cysts in adults. Pitfalls in diagnosis and management. Scand J Gastroenterol 1991, 26, 197-206 Six women have been referred to us for treatment of bile duct cysts during the past 6 years. In three patients the disorder was misdiagnosed by the referring institution, and they were admitted because of continuing severe symptoms; two of them had been treated with internal drainage procedures which led to unnecessary treatment delay and complications. Two patients had developed cyst carcinoma, the most feared complication of cyst disease. It is concluded that clinical symptoms are nonspecific, and the diagnosis is only ascertained after complete cholangiographic imaging of the biliary tree. Primary cyst excision and hepaticojejunostomy is the treatment of choice. Key words: Bile duct cysts; carcinoma; surgery Arne Bakka, M . D . , Surgical Dept. B, Rikshospiralet, Pilestredet 32, 0027 Oslo I , Norway
Bile duct cysts are uncommon anomalies. However, more than 3000 cases have now been reported, the majority from Japan. The incidence in Western countries is unknown; estimates range between 1 in 13,000 (1) and 1 in 2,000,000 births (2). Approximately 20% of such cysts are diagnosed in adults (3-5). The cysts lead in most cases to severe symptoms and complications, but these can be abated with correct diagnosis and management. Early reports on operative treatment suggested that internal drainage procedures (choledochocystoenterostomy) or cyst exclusion was satisfactory. However, complications occurred in an unacceptably high proportion of patients undergoing such procedures, and radical cyst excision has emerged as the treatment of choice (1,4, 6-11). Optimal management depends, however, on accurate preoperative diagnosis and classification of the lesion. The described association of bile duct cysts with an anomalous pancreaticobiliary junction (12) further stresses the importance of recognition and investigation of such patients. Despite marked advances in bile duct imaging
modalities over the past decade, the rarity of bile duct cysts in adults in Scandinavia leads too often to misdiagnosis and delayed treatment. The present report elucidates some of the pitfalls in diagnosis and treatment of bile duct cysts, illustrated by case histories of adult patients operated on in our department. PATIENTS AND METHODS Six consecutive patients, all women (mean age, 33 years; range, 21-49 years), with bile duct cysts were treated in our department during the 6-year period 1984-89. The lesions were categorized anatomically in accordance with the Todani modification (13) of the Alfonso-Lej classification system (14), a system based on cholangiographic imaging by either the endoscopic retrograde (ERC) or percutaneous transhepatic (PTC) technique (Fig. 1). The case histories are summarized in Table I. Three of the patients were initially misdiagnosed. Two of these had undergone an inconclusive ERC examination and were referred for investigation and treatment because of cholangitis and pan-
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Fig. 1. Cyst types (Todani classification). I = extrahepatic bile duct cyst; I1 = diverticulum of common bile duct; 111 = choledochocele; IV = combined intrahepatic and extrahepatic bile duct cysts; and V = cysts demonstrating both isolated and diffuse cystic dilatation of intrahepatic bile ducts (Caroli’s disease). Reprinted by permission from Archives of Surgery ( S ) , (copyright 1986, American Medical Association).
creatitis. For the third patient an incorrect diagnosis of pancreatic pseudocyst had been established. Three patients were referred with a correct preoperative diagnosis. Liver function test (LFI’) results before diagnosis and final treatment were normal in all. The pancreaticobiliary junction was visualized in all six patients. In four of them a long anomalous common channel (>1.5 cm) was demonstrated. Two patients had bile duct stones. All patients were operated on with radical cyst excision and bilioenteric reconstruction, using a Roux-en-Y jejunal loop. Operative results were classified as ‘good’ if the patient remained without symptoms and with normal LIT results; ‘fair’ if the patient had occasional episodes of cholangitis or pancreatitis with minor LFT abnormalities; and ‘poor’ if the patient had persistent symptoms requiring treatment.
RESULTS At a mean follow-up period of 38 months (range, 3-77 months) the results were good in all patients. Only Patient 3 needs occasional analgetics because of intermittent pain in the right upper quadrant. None has needed hospitalization due to cyst-related disease. The two patients with carcinoma in the excised cyst have had no recurrence.
DISCUSSION Whether bile duct cysts are congenital or acquired is disputed (8,15). They can develop as a consequence of an embryonic malformation causing weakness of the bile duct wall, and such a defect may be associated with distal obstruction or neuromuscular dysfunction of the sphincter of Oddi, leading to the typical cystic anomaly. An
25
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30
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39
31
1
2
3
4
5
6
Patient Age, no. years
No
Pain Mass
Previous operation
Choledocholithotomy. Choledochoduodenostomy (Fig. 6) No
Pain No NonHodgkin lymphoma
Pancreatitis
Pain
Pancreatitis
Pain
Pancreatitis Hematemesis Pancreas divisiu m Pain Cystojenunostomy Cholangitis
No
Pain Cholangitis Septicemia
Symptoms
Table I. Summary of case histories
Stricture of the anastomosis. Pain
Lifethreatening cholangitis
Further problems
Yes
No
not conclusive 2. Finally conclusive ( E W Yes
Yes
1 . Initially
No
not conclusive 2. Finally conclusive (ERC) Yes
Yes
Yes
1. Initially
misinterpreted (Fig. 3) 2. Finally conclusive (Fig. 4)
1. Initially
Yes
Complete Anomalous cholangiographic common mapping channel Final treatment
Type I with tumor (Fig. 7)
Whipple’s op. with cyst excision
Type-V cyst Left (Caroli’s hepatic disease) resection left lobe (Fig. 2) Initially cyst wrong excision (pancreatic and hepaticopseudocyst). Finally jejunostomy correct (type I) Type IV cyst excision (Fig. 5) and hepaticojejunostomy Type IV cyst excision and hepaticojejunostomy Type I cyst excision and hepaticojejunostomy
Preoperative diagnosis
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Yes
No
NO
No
Yes
No
3
17
19
36
75
77
Bile cyst FOIIOW-UP, carcinoma months
ki:
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Fig. 2. Patient 1. Left-sided Caroli’s disease. A row of intrahepatic cysts filled with calculi (arrows).
alternative model is based on the demonstration of an anomalous pancreaticobiliary junction with a long common channel (>1.5 cm), a feature that precludes the formation and function of a proper sphincter mechanism above the junction (12,16) (Fig. 8). This ‘common channel theory’ is supported by studies indicating that a postganglionic neural dysfunction or oligoganglionosis in the distal choledochus may exist (17-19). In the absence of a well-functioning sphincter mechanism above the junction, reflux of pancreatic enzymes to the bile duct may cause or add to the tendency of dilatation. An abnormal pancreaticobiliary junction is found in 4 0 4 5 % of adult patients (as in our series), predominantly in those with type-I and -1V cysts (20-22). Such an anomaly may even be present in individuals without biliary cystic lesions (23). Even though bile duct cysts are congenital, they are not recognized until adulthood in approximately 20% of patients (3-9,although many of them experience abdominal symptoms from childhood (like three of our six patients). Only a few present with the so-called classical triad of symptoms (epigastric pain, abdominal mass, and jaundice (10,24)). Symptoms in adults are non-
specific and common for various disorders, such as cholelithiasis, cholecystitis, and pancreatitis. Two of our five patients had bile duct stones, a finding not uncommon among adult patients with bile duct cysts. Prevalence estimates range from 2% to 72% (4,22,24,29). Even though acute pancreatitis often complicates bile duct cysts, the association may not be recognized. Three of our five patients had experienced this illness, and it is important to bear in mind its causal relationship (23,25,26), which lends support to the view that ERCP should be carried out in all patients with ‘idiopathic’ acute pancreatitis. Internal drainage of the cyst commonly used in the past has now been superseded by radical cyst excision and bilioenteric reconstruction using a Roux-en-Y jejunal loop (1,4,7-11,24,27). Several arguments favor such a strategy. The mucosa of the cyst is abnormal, and the epithelial lining is, especially in adults, broken by patches of ulceration and bridges of chronically inflamed tissue. If such nonepithelialized surfaces are incorporated into an anastomosis (as in internal drainage without cyst excision), 10-65% will need reoperation due to stricture formation (as in Patient 4)
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Fig. 3. Patient 2. 3A. ERCP that initially had been misinterpreted as a pancreatic pseudocyst. An anomalous common channel (arrows) is demonstrated, as is a connection to the Santorini duct from the major papilla through a not completely separated ventral part of the gland ('incomplete' pancreas divisum). 3B. Endoscope removed; the misinterpreted picture. 3C. CT imaging was also initially misinterpreted.
(1,8,9,20,28), compared with 1 4 % (4,8) if complete cyst excision and anastomosis to proximal normal mucosa is carried out. Whether typeIV cysts should always lead to hepatic resection is debated. As demonstrated in Patient 3 (Fig. 5), there may be some fusiform intrahepatic dilatation of one or more ducts. Generally, hepatic resection is not recommended (7), as the main goal of surgical treatment is to prevent reflux of pancreatic juice, which damages the mucosa.
Complete excision of an extrahepatic cyst and reconstruction can b e carried out with comparable mortality to and less morbidity than drainage procedures (1,4,5,8,28). T h e recently introduced procedure of cyst excision and biliary-jejunoduodenal diversion by an interposed jejunal loop is not used by us but may be an acceptable alternative (29). Bile duct carcinomas occur at least 20 times more frequently in those with cysts than in the
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Fig. 4. Patient 2. Intravenous cholangiography before referral gave the correct diagnosis. The cyst carcinoma was not suspected preoperatively.
general population (28,30,31).The risk of cancer increases with each decade of life (32), and the average patient with such a malignancy is in the early thirties. Seventy per cent are female, which, however, only reflects that cysts occur more frequently in women (31). A correct diagnosis is often missed preoperatively, as in one of our patients with malignancy, and the prognosis is generally poor despite radical cyst excision (32, 34). Hemobilia can infrequently be seen (33) but may also be misinterpreted, as in Patient 2. Of patients with carcinoma arising in a cyst 2050% have until recently had an internal drainage procedure carried out on average 14 years before diagnosis of the malignancy (11,30,31). The malignant process is probably initiated and promoted by the pancreatic juice reflux, which most likely results from the anomalous pancreaticobiliary junction. The reflux theory is further supported by the high risk of carcinoma developing in the gallbladder and other parts of the pancreaticobiliary tree even in patients without cysts but with an identical anomalous pancreatico-
biliary junction (22,35,36).Thus, treatment aims to prevent reflux and to preclude the possibility for bile and pancreatic juice stasis. There is some controversy whether bile duct cysts are associated with congenital hepatic fibrosis or lead to hepatic cirrhosis (37) and secondary portal hypertension (38). Long-standing obstruction complicated by cholangitis, which often follows internal drainage procedures, will ultimately lead to irreversible liver damage. Some authors note that type-V cysts (Caroli type) only affect segmental bile ducts and that they as such have no effect on the portal system and never will lead to portal hypertension or splenomegaly (39). The dreaded complication related to type-V duct dilatations is chronic infection leading to intraductal lithiasis, followed by episodes of suppurative cholangitis, abscesses and septicemia (as in Patient 1). Patients with Caroli-type cysts confined to one liver lobe should undergo excision of the lobe. It is beyond the scope of this presentation to discuss treatment alternatives for type-V cysts further. It
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Bile Duct Cysts in Adults
Fig. 5 . Patient 3 . 5A. Type-IV bile duct cyst. 5B. Schematic drawing (by the radiologist) of cholangiographic findings. Fusiform dilatations of extrahepatic ducts. 1 = cyst d the c o m o a hepatic duct; 2 = cyst of the cystic duct; 3 = common bile duct cyst; 4 = distal normal portion of common bile duct; and 5 = pancreatic duct. 5C. Anomalous long common channel (>1.5 cm; arrows,).
i s important, however, to consider the alternative
of liver transplantation. Consequently', it is mandatory to refrain from invasive diagnostic o r
therapeutic procedures, which increase the risk of infection. Consultation with a livei transplant unit should preferably precede any further invas-
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Fig. 6. Patient 4. Type-IV cyst initially treated with internal drainage. Extremely long common channel with bile duct joining the pancreatic duct at a very high level (arrows).
ive investigative or therapeutic effort in such patients. Patients with a choledochocele (Todani type 111) contrast with those with other types of bile duct dilatations in that they are generally older than 20 years when symptoms start, and there is no femde predominance (40). Such patients constitute less than 5% of reported cases of bile duct cysts (41). None of the patients in the present series had such lesion. Indeed, the pathogenesis for type-I11 cysts remains an enigma, and doubt has been expressed whether it is justified to classify this lesion as part of the bile duct cyst complex. The choledochocele may simply be a membranous obstruction at the papilla or an enteric duplication cyst. In fact, both sides of the choledochocele can be lined with duodenal mucosa ( 5 , 2 0 ) . Recent reports demonstrate that choledochocele can be successfully treated endoscopically, either by removal of the cyst by sling resection or by endoscopic sphincterotomy
(42-44). In conclusion, the symptoms related to cystic dilatation of bile ducts in adults can mimic various disorders in the upper abdomen. A correct diagnosis is often arrived at after considerable delay.
Fig. 7. Patient 6 . 7A. Type-I cyst with contrast defect (arrow) due to a carcinoma. 7B. CT image showing the same cystic dilatation (arrows) of the common bile duct containing a tumor.
Bile Duct Cysts in Adults
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-,I
'
205
'5
DM
(Normal Connection)
(Anomalous Connection)
Fig. 8. Normal and anomalous connections between the common bile duct and the pancreatic duct, with the sphincter as shown. Reprinted by permission from Wurld Journal of Surgery (22).
Proper cholangiographic imaging is the prime diagnostic modality. Excision of the cyst and bilioenteric reconstruction with a Roux-en-Y jejunal loop is the treatment of choice and will most probably prevent later development of malignancy. REFERENCES 1. Olbourne NA. Choledochal cysts. A review of the cystic anomalies of the biliary tree. Ann R Coll Surg Engl 1975, 56, 26-32 2. Howell CG, Templeton JM, Weiner S, Glassman M, Betts JM, Witzleben CL. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg 1983, 18, 387-393 3. Flanigan DP. Biliary cysts. Ann Surg 1975, 182, 635-643 4. Yamaguchi M. Congenital choledochal cyst. Analysis of 1433 patients in the Japanese literature. Am J Surg 1980, 140, 653-657 5. Deziel DJ, Rossi RL, Munson JL, Braasch JW, Silverman ML. Management of bile duct cysts in adults. Arch Surg 1986, 121, 41@415 6. Trout HH, Longmire WP. Long-term follow-up study of patients with congenital cystic dilatation of the common bile duct. Am J Surg 1971, 121, 68-86 7. Powell CS, Sawyers JL, Reynolds VH. Management of adult choledochal cysts. Ann Surg 1981,193,666676 8. Saito S, Ishida M. Congenital choledochal cyst (cystic dilatation of the common bile duct). Prog Pediatr Surg 1974, 6, 63-90
9. Miyano T, Suruga K, Chen S-C. A clinicopathologic study of choledochal cyst. World J Surg 1980, 4, 231-238 10. Tan KC, Howard ER. Choledochal cyst: a 14-year surgical experience with 36 patients. Br J Surg 1988, 75, 892-895 11. Todani T, Watanabe Y, Toki A, Urushihara N. Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 1987, 164, 61-64 12. Babbit DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb. Ann Radio1 1969, 12, 231-240 13. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts. Classification, operative procedures, and review of thirtyseven cases including cancer arising from choledochal cyst. Am J Surg 1977. 134, 263-269 14. Alfonso-Lej F, Rever WB Jr, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Int Abstr Surg 1959, 108, 1-30 15. Shallow TA, Eger SA, Wagner FB. Congenital cystic dilatation of the common bile duct. Case report and review of literature. Ann Surg 1943, 117, 355-386 16. Babbit DP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology. Am J Roentgen01 Rad Ther 1973, 119, 57-62 17. Iwai N , Tokiwa K, Tsuto T, Yanagihara J, Takahashi T. Biliary manometry in choledochal cyst with abnormal choledochopancreatico ductal junction. J Pediatr Surg 1986, 21, 873-876 18. Kusunoki M, Yamamura T, Takahashi T, Kantoh M, Ishikawa Y, Utsunomiya J. Choledochal cyst.
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Its possible autonomic involvement in the bile duct. Arch Surg 1987, 122, 997-1000 19. Kusunoki M, Saitoh N , Yamamura T, Fujita S, Takahashi T, Utsunomiya J. Choledochal cysts. Oligoganglionosis in the narrow portion of the choledochus. Arch Surg 1988, 123, 984986 20. O’Neill JA, Templeton JM Jr, Schnaufer L, Bishop HC, Ziegler MM, Ross AJ 111. Recent experience with choledochal cyst. Ann Surg 1987,205,533-539 21. Ono J, Sakoda K, Akita H. Surgical aspect of cystic dilatation of the bile duct. An anomalous junction of the pancreaticobiliary tract in adults. Ann Surg 1982, 195, 203-208 22. Nagata E, Sakai K, Kinoshita H, Hirohashi K. Choledochal cyst: complications of anomalous connection between the choledochus and pancreatic duct and carcinoma of the biliary tract. World J Surg 1986, 10, 102-110 23. Matsusue S , Kashihara S, Nakamura Y , Masumoto H. Role of unusually long common pancreaticobiliary channel as a cause of relapsing pancreatitis in children. Z Kinderchir 1982, 36, 69-72 24. Nagorney DM, McIlrath DC, Adson MA. Choledochal cysts in adults: clinical management. Surgery 1984, 96, 656-663 25. Altman MS, Halls JM, Douglas AP, Renner IG. Choledochal cyst presenting as acute pancreatitis. Evaluation with endoscopic retrograde cholangiopancreatography. Am J Gastroenterol 1978, 70, 514-519 26. Okada A, Oguchi Y, Kainata S, Ikeda Y , Kawashima Y , Saito R. Common channel syndromediagnosis with endoscopic retrograde cholangiopancreatography and surgical management. Surgery 1983, 93, 634-642 27. Jones PG, Smith ED, Clark AM, Kent M. Choledochal cysts: experience with radical excision. J Pediatr Surg 1971, 6, 112-120 28. Rattner DW, Schapiro RH, Warshaw AL. Abnormalities of the pancreatic and biliary ducts in adult patients with choledochal cysts. Arch Surg 1983, 118, 1068-1073 29. Gonzalez EM, Garcia IG, Pascual MH, et al. Choledochal cyst resection and reconstruction by biliaryjejuno-duodenal diversion. World J Surg 1989, 13, 232-237 30. Flanigan DP. Biliary carcinoma associated with biliary cysts. Cancer 1977, 40, 880-883 31. Kagawa Y, Kashirara S, Kurarnoto S, Maetani S. Received 7 June 1990 Accepted 30 July 1990
Carcinoma arising in a congenitally dilated biliary tract. Report of a case and review of the literature. Gastroenterology 1978, 74, 1286-1294 32. Voyles CR, Smadja C, Shands WC, Blumgart LH. Carcinoma in choledochal cysts. Age-related incidence. Arch Surg 1983, 118, 986-988 33. Sandblom P. Hemobilia (biliary tract hemorrhage). History, pathology, diagnosis, treatment. Charles C Thomas, Springfield. Ill., USA, 1972 34. Rossi RL, Silverman ML, Braasch JW, Munson JL, ReMine SG. Carcinoma arising in cystic conditions of the bile ducts. A clinical and pathologic study. Ann Surg 1987, 205, 377-384 35. Kinoshita H , Nagata E, Hirohashi K, Sakai K, Kobayashi Y. Carcinoma of the gallbladder with an anomalous connection between the choledochus and the pancreatic duct. Report of 10 cases and review of the literature in Japan. Cancer 1984, 54, 762-769 36. Kimura K, Ohto M, Saisho H, et al. Association of gallbladder carcinoma and anomalous pancreaticobiliary ductal union. Gastroenterology 1985, 89, 1258-1265 37. Lake DNW, Smith PM, Wheeler MH. Congenital hepatic fibrosis and choledochal cyst. Br Med J 1977.2. 1259-1260 38. Martin LW, Rowe GA. Portal hypertension secondary to choledochal cyst. Ann Surg 1979, 190,638639 39. Mercadier M, Chigot JP, Clot JP, Langlois P, Lansiaux P. Caroli’s disease. World J Surg 1984, 8, 22-29 40. Ozawa K, Yamada T, Matumoto Y , Tobe R. Carcinoma arising in a choledochocele. Cancer 1980, 45, 195-197 41. Crittenden SL, McKinley MJ. Choledochal cystclinical features and classification. Am J Gastroenterol 1985, 80, 643-647 42. Venu RP, Geenen JE, Hogan WJ, et al. Role of endoscopic retrograde cholangiopancreatograph y in the diagnosis and treatment of choledochocele. Gastroenterology 1984, 87, 1144-1 149 43. Siege1 JH, Harding GT, Chateau F. Endoscopic incision of choledochal cysts (choledochocele). Endoscopy 1981, 13, 200-202 44. Gerritsen JJ, Janssens AR, Kroon HM. Choledochocele: treatment by endoscopic sphincterotomy. Br J Surg 1988, 75, 495-496
ISSN: 0036-5521 (Print) 1502-7708 (Online) Journal homepage: http://www.tandfonline.com/loi/igas20
Bile Duct Cysts in Adults: Pitfalls in Diagnosis and Management A. Bakka, A. Bergan & O. Søreide To cite this article: A. Bakka, A. Bergan & O. Søreide (1991) Bile Duct Cysts in Adults: Pitfalls in Diagnosis and Management, Scandinavian Journal of Gastroenterology, 26:2, 197-206, DOI: 10.3109/00365529109025031 To link to this article: http://dx.doi.org/10.3109/00365529109025031
Published online: 08 Jul 2009.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=igas20 Download by: [New York University]
Date: 30 March 2016, At: 02:44
Bile Duct Cysts in Adults Pitfalls in Diagnosis and Management A. BAKKA, A. BERGAN & 0. SBREIDE Surgical Dept. B, Rikshospitalet, Oslo, Norway
Downloaded by [New York University] at 02:44 30 March 2016
Bakka A , Bergan A, Soreide 0. Bile duct cysts in adults. Pitfalls in diagnosis and management. Scand J Gastroenterol 1991, 26, 197-206 Six women have been referred to us for treatment of bile duct cysts during the past 6 years. In three patients the disorder was misdiagnosed by the referring institution, and they were admitted because of continuing severe symptoms; two of them had been treated with internal drainage procedures which led to unnecessary treatment delay and complications. Two patients had developed cyst carcinoma, the most feared complication of cyst disease. It is concluded that clinical symptoms are nonspecific, and the diagnosis is only ascertained after complete cholangiographic imaging of the biliary tree. Primary cyst excision and hepaticojejunostomy is the treatment of choice. Key words: Bile duct cysts; carcinoma; surgery Arne Bakka, M . D . , Surgical Dept. B, Rikshospiralet, Pilestredet 32, 0027 Oslo I , Norway
Bile duct cysts are uncommon anomalies. However, more than 3000 cases have now been reported, the majority from Japan. The incidence in Western countries is unknown; estimates range between 1 in 13,000 (1) and 1 in 2,000,000 births (2). Approximately 20% of such cysts are diagnosed in adults (3-5). The cysts lead in most cases to severe symptoms and complications, but these can be abated with correct diagnosis and management. Early reports on operative treatment suggested that internal drainage procedures (choledochocystoenterostomy) or cyst exclusion was satisfactory. However, complications occurred in an unacceptably high proportion of patients undergoing such procedures, and radical cyst excision has emerged as the treatment of choice (1,4, 6-11). Optimal management depends, however, on accurate preoperative diagnosis and classification of the lesion. The described association of bile duct cysts with an anomalous pancreaticobiliary junction (12) further stresses the importance of recognition and investigation of such patients. Despite marked advances in bile duct imaging
modalities over the past decade, the rarity of bile duct cysts in adults in Scandinavia leads too often to misdiagnosis and delayed treatment. The present report elucidates some of the pitfalls in diagnosis and treatment of bile duct cysts, illustrated by case histories of adult patients operated on in our department. PATIENTS AND METHODS Six consecutive patients, all women (mean age, 33 years; range, 21-49 years), with bile duct cysts were treated in our department during the 6-year period 1984-89. The lesions were categorized anatomically in accordance with the Todani modification (13) of the Alfonso-Lej classification system (14), a system based on cholangiographic imaging by either the endoscopic retrograde (ERC) or percutaneous transhepatic (PTC) technique (Fig. 1). The case histories are summarized in Table I. Three of the patients were initially misdiagnosed. Two of these had undergone an inconclusive ERC examination and were referred for investigation and treatment because of cholangitis and pan-
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Fig. 1. Cyst types (Todani classification). I = extrahepatic bile duct cyst; I1 = diverticulum of common bile duct; 111 = choledochocele; IV = combined intrahepatic and extrahepatic bile duct cysts; and V = cysts demonstrating both isolated and diffuse cystic dilatation of intrahepatic bile ducts (Caroli’s disease). Reprinted by permission from Archives of Surgery ( S ) , (copyright 1986, American Medical Association).
creatitis. For the third patient an incorrect diagnosis of pancreatic pseudocyst had been established. Three patients were referred with a correct preoperative diagnosis. Liver function test (LFI’) results before diagnosis and final treatment were normal in all. The pancreaticobiliary junction was visualized in all six patients. In four of them a long anomalous common channel (>1.5 cm) was demonstrated. Two patients had bile duct stones. All patients were operated on with radical cyst excision and bilioenteric reconstruction, using a Roux-en-Y jejunal loop. Operative results were classified as ‘good’ if the patient remained without symptoms and with normal LIT results; ‘fair’ if the patient had occasional episodes of cholangitis or pancreatitis with minor LFT abnormalities; and ‘poor’ if the patient had persistent symptoms requiring treatment.
RESULTS At a mean follow-up period of 38 months (range, 3-77 months) the results were good in all patients. Only Patient 3 needs occasional analgetics because of intermittent pain in the right upper quadrant. None has needed hospitalization due to cyst-related disease. The two patients with carcinoma in the excised cyst have had no recurrence.
DISCUSSION Whether bile duct cysts are congenital or acquired is disputed (8,15). They can develop as a consequence of an embryonic malformation causing weakness of the bile duct wall, and such a defect may be associated with distal obstruction or neuromuscular dysfunction of the sphincter of Oddi, leading to the typical cystic anomaly. An
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39
31
1
2
3
4
5
6
Patient Age, no. years
No
Pain Mass
Previous operation
Choledocholithotomy. Choledochoduodenostomy (Fig. 6) No
Pain No NonHodgkin lymphoma
Pancreatitis
Pain
Pancreatitis
Pain
Pancreatitis Hematemesis Pancreas divisiu m Pain Cystojenunostomy Cholangitis
No
Pain Cholangitis Septicemia
Symptoms
Table I. Summary of case histories
Stricture of the anastomosis. Pain
Lifethreatening cholangitis
Further problems
Yes
No
not conclusive 2. Finally conclusive ( E W Yes
Yes
1 . Initially
No
not conclusive 2. Finally conclusive (ERC) Yes
Yes
Yes
1. Initially
misinterpreted (Fig. 3) 2. Finally conclusive (Fig. 4)
1. Initially
Yes
Complete Anomalous cholangiographic common mapping channel Final treatment
Type I with tumor (Fig. 7)
Whipple’s op. with cyst excision
Type-V cyst Left (Caroli’s hepatic disease) resection left lobe (Fig. 2) Initially cyst wrong excision (pancreatic and hepaticopseudocyst). Finally jejunostomy correct (type I) Type IV cyst excision (Fig. 5) and hepaticojejunostomy Type IV cyst excision and hepaticojejunostomy Type I cyst excision and hepaticojejunostomy
Preoperative diagnosis
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Yes
No
NO
No
Yes
No
3
17
19
36
75
77
Bile cyst FOIIOW-UP, carcinoma months
ki:
.k
S’
3i:
9r?
fi
5
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Fig. 2. Patient 1. Left-sided Caroli’s disease. A row of intrahepatic cysts filled with calculi (arrows).
alternative model is based on the demonstration of an anomalous pancreaticobiliary junction with a long common channel (>1.5 cm), a feature that precludes the formation and function of a proper sphincter mechanism above the junction (12,16) (Fig. 8). This ‘common channel theory’ is supported by studies indicating that a postganglionic neural dysfunction or oligoganglionosis in the distal choledochus may exist (17-19). In the absence of a well-functioning sphincter mechanism above the junction, reflux of pancreatic enzymes to the bile duct may cause or add to the tendency of dilatation. An abnormal pancreaticobiliary junction is found in 4 0 4 5 % of adult patients (as in our series), predominantly in those with type-I and -1V cysts (20-22). Such an anomaly may even be present in individuals without biliary cystic lesions (23). Even though bile duct cysts are congenital, they are not recognized until adulthood in approximately 20% of patients (3-9,although many of them experience abdominal symptoms from childhood (like three of our six patients). Only a few present with the so-called classical triad of symptoms (epigastric pain, abdominal mass, and jaundice (10,24)). Symptoms in adults are non-
specific and common for various disorders, such as cholelithiasis, cholecystitis, and pancreatitis. Two of our five patients had bile duct stones, a finding not uncommon among adult patients with bile duct cysts. Prevalence estimates range from 2% to 72% (4,22,24,29). Even though acute pancreatitis often complicates bile duct cysts, the association may not be recognized. Three of our five patients had experienced this illness, and it is important to bear in mind its causal relationship (23,25,26), which lends support to the view that ERCP should be carried out in all patients with ‘idiopathic’ acute pancreatitis. Internal drainage of the cyst commonly used in the past has now been superseded by radical cyst excision and bilioenteric reconstruction using a Roux-en-Y jejunal loop (1,4,7-11,24,27). Several arguments favor such a strategy. The mucosa of the cyst is abnormal, and the epithelial lining is, especially in adults, broken by patches of ulceration and bridges of chronically inflamed tissue. If such nonepithelialized surfaces are incorporated into an anastomosis (as in internal drainage without cyst excision), 10-65% will need reoperation due to stricture formation (as in Patient 4)
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Fig. 3. Patient 2. 3A. ERCP that initially had been misinterpreted as a pancreatic pseudocyst. An anomalous common channel (arrows) is demonstrated, as is a connection to the Santorini duct from the major papilla through a not completely separated ventral part of the gland ('incomplete' pancreas divisum). 3B. Endoscope removed; the misinterpreted picture. 3C. CT imaging was also initially misinterpreted.
(1,8,9,20,28), compared with 1 4 % (4,8) if complete cyst excision and anastomosis to proximal normal mucosa is carried out. Whether typeIV cysts should always lead to hepatic resection is debated. As demonstrated in Patient 3 (Fig. 5), there may be some fusiform intrahepatic dilatation of one or more ducts. Generally, hepatic resection is not recommended (7), as the main goal of surgical treatment is to prevent reflux of pancreatic juice, which damages the mucosa.
Complete excision of an extrahepatic cyst and reconstruction can b e carried out with comparable mortality to and less morbidity than drainage procedures (1,4,5,8,28). T h e recently introduced procedure of cyst excision and biliary-jejunoduodenal diversion by an interposed jejunal loop is not used by us but may be an acceptable alternative (29). Bile duct carcinomas occur at least 20 times more frequently in those with cysts than in the
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Fig. 4. Patient 2. Intravenous cholangiography before referral gave the correct diagnosis. The cyst carcinoma was not suspected preoperatively.
general population (28,30,31).The risk of cancer increases with each decade of life (32), and the average patient with such a malignancy is in the early thirties. Seventy per cent are female, which, however, only reflects that cysts occur more frequently in women (31). A correct diagnosis is often missed preoperatively, as in one of our patients with malignancy, and the prognosis is generally poor despite radical cyst excision (32, 34). Hemobilia can infrequently be seen (33) but may also be misinterpreted, as in Patient 2. Of patients with carcinoma arising in a cyst 2050% have until recently had an internal drainage procedure carried out on average 14 years before diagnosis of the malignancy (11,30,31). The malignant process is probably initiated and promoted by the pancreatic juice reflux, which most likely results from the anomalous pancreaticobiliary junction. The reflux theory is further supported by the high risk of carcinoma developing in the gallbladder and other parts of the pancreaticobiliary tree even in patients without cysts but with an identical anomalous pancreatico-
biliary junction (22,35,36).Thus, treatment aims to prevent reflux and to preclude the possibility for bile and pancreatic juice stasis. There is some controversy whether bile duct cysts are associated with congenital hepatic fibrosis or lead to hepatic cirrhosis (37) and secondary portal hypertension (38). Long-standing obstruction complicated by cholangitis, which often follows internal drainage procedures, will ultimately lead to irreversible liver damage. Some authors note that type-V cysts (Caroli type) only affect segmental bile ducts and that they as such have no effect on the portal system and never will lead to portal hypertension or splenomegaly (39). The dreaded complication related to type-V duct dilatations is chronic infection leading to intraductal lithiasis, followed by episodes of suppurative cholangitis, abscesses and septicemia (as in Patient 1). Patients with Caroli-type cysts confined to one liver lobe should undergo excision of the lobe. It is beyond the scope of this presentation to discuss treatment alternatives for type-V cysts further. It
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Fig. 5 . Patient 3 . 5A. Type-IV bile duct cyst. 5B. Schematic drawing (by the radiologist) of cholangiographic findings. Fusiform dilatations of extrahepatic ducts. 1 = cyst d the c o m o a hepatic duct; 2 = cyst of the cystic duct; 3 = common bile duct cyst; 4 = distal normal portion of common bile duct; and 5 = pancreatic duct. 5C. Anomalous long common channel (>1.5 cm; arrows,).
i s important, however, to consider the alternative
of liver transplantation. Consequently', it is mandatory to refrain from invasive diagnostic o r
therapeutic procedures, which increase the risk of infection. Consultation with a livei transplant unit should preferably precede any further invas-
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Fig. 6. Patient 4. Type-IV cyst initially treated with internal drainage. Extremely long common channel with bile duct joining the pancreatic duct at a very high level (arrows).
ive investigative or therapeutic effort in such patients. Patients with a choledochocele (Todani type 111) contrast with those with other types of bile duct dilatations in that they are generally older than 20 years when symptoms start, and there is no femde predominance (40). Such patients constitute less than 5% of reported cases of bile duct cysts (41). None of the patients in the present series had such lesion. Indeed, the pathogenesis for type-I11 cysts remains an enigma, and doubt has been expressed whether it is justified to classify this lesion as part of the bile duct cyst complex. The choledochocele may simply be a membranous obstruction at the papilla or an enteric duplication cyst. In fact, both sides of the choledochocele can be lined with duodenal mucosa ( 5 , 2 0 ) . Recent reports demonstrate that choledochocele can be successfully treated endoscopically, either by removal of the cyst by sling resection or by endoscopic sphincterotomy
(42-44). In conclusion, the symptoms related to cystic dilatation of bile ducts in adults can mimic various disorders in the upper abdomen. A correct diagnosis is often arrived at after considerable delay.
Fig. 7. Patient 6 . 7A. Type-I cyst with contrast defect (arrow) due to a carcinoma. 7B. CT image showing the same cystic dilatation (arrows) of the common bile duct containing a tumor.
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-,I
'
205
'5
DM
(Normal Connection)
(Anomalous Connection)
Fig. 8. Normal and anomalous connections between the common bile duct and the pancreatic duct, with the sphincter as shown. Reprinted by permission from Wurld Journal of Surgery (22).
Proper cholangiographic imaging is the prime diagnostic modality. Excision of the cyst and bilioenteric reconstruction with a Roux-en-Y jejunal loop is the treatment of choice and will most probably prevent later development of malignancy. REFERENCES 1. Olbourne NA. Choledochal cysts. A review of the cystic anomalies of the biliary tree. Ann R Coll Surg Engl 1975, 56, 26-32 2. Howell CG, Templeton JM, Weiner S, Glassman M, Betts JM, Witzleben CL. Antenatal diagnosis and early surgery for choledochal cyst. J Pediatr Surg 1983, 18, 387-393 3. Flanigan DP. Biliary cysts. Ann Surg 1975, 182, 635-643 4. Yamaguchi M. Congenital choledochal cyst. Analysis of 1433 patients in the Japanese literature. Am J Surg 1980, 140, 653-657 5. Deziel DJ, Rossi RL, Munson JL, Braasch JW, Silverman ML. Management of bile duct cysts in adults. Arch Surg 1986, 121, 41@415 6. Trout HH, Longmire WP. Long-term follow-up study of patients with congenital cystic dilatation of the common bile duct. Am J Surg 1971, 121, 68-86 7. Powell CS, Sawyers JL, Reynolds VH. Management of adult choledochal cysts. Ann Surg 1981,193,666676 8. Saito S, Ishida M. Congenital choledochal cyst (cystic dilatation of the common bile duct). Prog Pediatr Surg 1974, 6, 63-90
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