Congenital Bile Duct Cysts

Classification, Operative Procedures, and Review of Thirty-Seven Cases Including Cancer Arising from Choledochal Cyst
Takuji Todani, MD, Okayama, Japan Yasuhiro Watanabe, MD, Okayama, Japan Mltsuo Narusue, MD, Okayama, Japan Katsusuke Tabuchi, MD, Okayama, Japan Kunio Okajlma, MD, Okayama, Japan

Congenital bile duct cysts, the so-called choledochal cysts, are common in Japan and the Orient. The classification developed by Alonso-Lej, Rever, and Pessagno [I] is widely accepted. However, there have been recent reports on patients with cystic dilatations in both the extra- and intrahepatic ducts that do not come under this classification [2-51. Moreover, the best operative procedure has not been determined, and excision of the cyst or internal drainage is usually performed. The present study describes our experience with congenital bile duct cysts, including two cases of cancer arising from a choledochal cyst, and discusses their classification and the operative procedures used
in treatment. Classification From 1960 to April 1976 we encountered thirty-

these twenty patients, fifteen had an intrahepatic bile duct cyst as well as a choledochal cyst, which we called type IV-A; one had a rare case of multiple cysts confined to the extrahepatic bile duct alone, which we called type IV-B; and the remaining four had single or multiple cystic dilatations in the intrahepatic bile duct only, which we called type V.
Operative Procedures The operative procedures administered at our clinic for

the various types of bile duct cysts are shown in Table rT II*Types Z and IV. External drainage operation: This procedure was performed in three patients. In two of these
patients it was performed as an emergency procedure, one for perforation of the cyst and the other for a deteriorating condition associated with cholelithiasis. Znternal drainage operation: Until eight years ago, our preferred technic was internal drainage. We performed

seven cases of congenital bile duct cysts in our clinic. Seventeen of the patients were less than seventeen years old and twenty were adults. (Table I.) The ratio of female to male was 2.4:1. According to the classification of Alonso-Lej, Rever, and Pessagno [I], seventeen patients in our series had type I cysts, the common type of cystic dilatation of the choledochus: ten children, including one with segmental dilatation [6]; and seven adults, including three with a diffuse subtype. We did not encounter any patients with types II or III cysts. The remaining twenty patients could not be classified under Alonso-Lejs classification. Of

choledcchocystogastrostcmy in one patient, choledochocystoduoclenostomy in ten, choledochocystocluodeostomy with gastrectomy in three, and choledochocystojejunostomy with Roux-en-Y anastomosis in three. Concomitant cholecystectomy was performed in all patients.
TABLE I Age and Sex Distribution of Patients with Bile Duct Cysts
-l 2 3 3 2 3 3 1 17 5 12 Type (no. of patients) IV-A IV-B V Total 2 5 5 5 9 7 4 37 11 26

Age (yr) <l 1-5 6-10 11-17 16-30 31-50 >51 Total Male Female

From the Department of Surgery, Ckayama University Medical School, Ckayama, Japan. Reprint requests should ba addressed to T. Todani. MD, Department of Surgery. Ckayama University Medical School, 2-5 Shikatacho. Okayama City 700, Japan.

2 2 3 5 2 1 15 4 11

1 1 0 1

1 2 1 4 2 2

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TABLE II

Owrative

Procedures for Bile Duct Cysts

Initial Number of Operations Secondary Final

For Types I and IV External drainage operation internal drainage operation Chofedochocystogastrostomy Choledochocystoduodenostomy Chokdochocystoduodenostomy with gastrostomy Choledochocystojejunostomy (ROWen-Y) Excision of bile duct cysts Hepaticocholedochostomy Hepaticoduodenostomy Hepaticojejunostomy (Roux-en-Y) lntrahepatic cystoduodenostomy at the porta hepatis lntrahepatic cystojejunostomy at the porta hepatis Pancreatoduodenectomy fransduodenal sphincteroplasty

3(3)

1WV
l(l) 9@) 2(l) l:(2) l(l) 3(l) 4 2 1 1 2 3 1 outcomes.

2 1 1 9 1 4 2 2

9(l) 4(l) : 20 5 8 4 3 1 2 3 1

For Type V
Partial hepatectomy Choledochojejunostomy (Roux-en-Y)

Note: Numbers in parentheses indicate the number of unsatisfactory l Includes one case with combined excision of the cyst.

Excision of the cyst: During the past eight years, excision of the choledochal cyst has been performed in twenty-one patients. One patient underwent pancreatoduodenectomy initially and nine underwent excision as a secondary operation. This has become our preferred procedure because we experienced two cases of cancer and six cases of ascending cholangitis after the initial drainage operation. The procedures used for reconstruction after excision of the cyst can be divided into five groups: hepaticocholedochosty, which was changed to hepaticoduodenostomy due to anastomotic leakage (1 patient); hepaticoduodenostomy (6); hepaticojejunostomy with Roux-en-Y anastomosis (7); intrahepatic cystoduodenostomy at the porta hepatis (5); and intrahepatic cystojejunostomy at the porta hepatis (3). Pancreatoduodenectomy was performed in a fifty-five year old male because of our inability to dissect the cyst due to severe inflammation of the head of the pancreas associated with pancreatic stones.

There have been no deaths due to the operation and all of the patients have remained well. Transduodenal sphincteroplasty [7]: This procedure was performed with good results in two patients for a type IV-B choledochocele cyst and for diffuse dilatation of a type Ic cyst. Type V. Because of atrophy of the affected lobe, partial hepatectomy could be performed easily in three patients with type V cysts confined to the one lobe. One patient underwent right hepatectomy and two underwent left lateral segmentectomy. The remaining patient underwent choledochojejunostomy with Roux-en-Y anastomosis because of the possibility of intrahepatic gallstones shifting into the intestine. All of the patients were asymptomatic for at least three years after operation. Case Reports of Cancer Arising from a Choledochal cyst Case I. A seventeen year old female had been perfectly well until age fourteen years when she began to suffer from occasional abdominal pains and fever. At age seventeen she underwent choledochocystoduodenostomy at our clinic for type I cyst. At age nineteen she again complained of right upper abdominal and back pains, high fever, and jaundice. She underwent reoperation for suspected anastomotic stricture. The cystic dilatation had reduced remarkably in size but a hard red bean-sized polypoid tumor, diagnosed as squamous cell cancer by frozen biopsy, was found on the posterior wall. (Figure 1.) The cyst could not be resected because of severe inflammation, and only external drainage of the cyst was performed. Two weeks after the operation, her abdomen was reopened for the radical operation, but cancer of the cyst had widely spread to the retroperitoneum, so gastrojejunostomy with Brauns anastomosis was completed. She died ten days after the final operation. Permission for autopsy was denied. The AmericanJournal ol Surgery

Figure 1. Case 1. Squamous cell cancer with pearl arlshtg in the posterior wall of choledochal cyst.

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Case II. A twenty-two year old male had undergone cholecystectomy and choledochal drainage at another hospital for cholelithiasis with abdominal pains and fever at age fourteen years. At fifteen years of age he again underwent choledochal drainage for a similar attack, but at that time it was noted that he had no choledochal cyst. At the age of twenty-two years he was transferred to our clinic because of repeated abdominal pains and fever. A third operation was performed for suspected recurrent bile duct stones. Although type IV-A (multiple) cysts was diagnosed by operative cholangiogram, only a choledochocystoduodenostomy was performed. Four years later he again began to suffer from abdominal pains and fever. A percutaneous transhepatic cholangiogram showed that the dye had not shifted from the intrahepatic cyst to the duodenum. A fourth operation was performed after dissection of extensive adhesions and a hard duck egg-sized, biopsy-proved cancerous tumor was found. Multiple liver metastases and mesenteric dissemination were also noted. (Figure 2.) Only intrahepatic cystojejunostomy was performed, but he died of carcinomatous peritonitis two months postoperatively.

Figure 2. Case Il. Adenocarcinoma arising in the wall of common bile duct cyst.

Comments
Classlficallon of Congenital Bile Duct Cysts

At present, the various anatomic forms of biliary cystic dilatation are reported to occur not only in the choledochus, but in any part of the bile duct between the liver and the duodenum. Therefore, we prefer the term bile duct cyst to choledochal cyst, and we propose to classify these cysts into the following six types based on our personal cases and literature on the subject. (Figure 3.) Alonso-Lejs classification is applicable only to the cyst of the common choledothus. Type I. Common type: (a) choledochal cyst in a narrow sense; (b) segmental choledochal dilatation; and (c) diffuse or cylindrical dilatation. Type II. Diverticulum type in the whole extrahepatic duct. Type III. Choledochocele. Type IV-A. Multiple cysts at the intra- and extrahepatic ducts. Type IV-B. Multiple cysts at the extrahepatic duct only. Type V. Intrahepatic bile duct cyst (single or multiple). Types I to III, which correspond to Alonso-Lejs classification of choledochal cyst, are well known to surgeons. However, it is likely that type I is divided into three subtypes in view of morphologic findings and surgical treatment. In type Ia cysts excision of the cyst is the most desirable procedure; in type Ib successful hepaticocholedochostomy after excision
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of the cyst may be possible; and in type Ic sphincteroplasty may be effective since stenosis of the terminal bile duct appears to play a leading part in its formation. Children with type Ic cyst symptoms tended to be older than children with type Ia symptoms, and the terminal duct of the choledochus was not as small in the former [8]. Type II is not restricted within the common choledochus and similar cysts may be observed in any part of the extrahepatic bile duct [9]. Type IV-A is multiple cysts that have intra- and extrahepatic bile duct cysts. Esquerra-Gomez and Riveros-Gamboa [9] described a patient with an intrahepatic cyst as well as a type II cyst and we classified him under type IV-A. Intrahepatic cysts usually involve hepatic branches of both lobes, but the left lobe seems to be more commonly involved than the right, although there is no explanation for this phenomenon [2,4]. They are commonly seen in older children and young adults. Development of the symptoms of type IV-A cysts may be delayed until the intrahepatic cysts are filled with the undrained and infected bile fluid. Their occurrence is being more frequently diagnosed by the increasing use of operative cholangiograms. Type TV-B is also multiple cysts, but cysts confined to the extrahepatic bile duct alone. Longmire, Mandiola, and Gordon [IO] called this very rare condition the extrahepatic multiple cystic dilatation. Our patient had both types of choledochocele and diffuse dilatation of the choledochus, which might have been induced by the choledochocele. Type V shows single or multiple cystic dilatations of thk intrahepatic bile duct which are not parenchymal [11,12]. In young adults, they may closely relate to intrahepatic gallstones that have resulted from infection and stagnation of bile, and some of them appear to belong to the pure form of Carolis disease without hepatic fibrosis.
265

Figure 3. Top, typical operative cholangtogfams of congenffal bile duct cysts: (left to right ) types la, common type; lb, sq#nantal dttatatlon; k, diffuse dilatation; IV-A, nndtlph3cyst (/Ma- and extfahepatk); types IV-B, nwff&ts cyst (extrahepatlc only); and V, lntrahepatk cysts. 6ottom, associated d/agrams demonstrat/ng typ/ng of ductal cysts: (left to r/ght) types la, lb, k, Ii, Ill, IV-A, IV-t?, and V.

OperativeProcedures

The best procedure for preventing ascending cholangitis as well as the formation of gallstones is free drainage of the bile. We recommend the following operative procedures for the various types of bile duct cysts. Type I. Type la: Ascending cholangitis and anastomotic stricture often develop after choledochocystoduodenostomy. It must be kept in mind that the anastomosis between the denuded epithelium of the cyst and the intestinal mucosa easily develops stricture postoperatively. Recently, choledochocystojejunostomy with Roux-en-Y anastomosis for type I cysts has been widely recommended as it has shown favorable results with long-term follow-up [13-151. Of our ten patients who underwent choledochocystoduodenostomy, two developed cancer of the cyst and four required excision of the cyst or partial gastrectomy as a second operation for postoperative ascending cholangitis. Consequently, we believe that the cyst should be resected [16,17] to prevent malignant change or ascending cholangitis, especiblly in older children and young adults. In the reconstruction procedure for prevention of ascending cholangitis after excision of the cyst, it is still controversial whether hepaticoduodenostomy

or hepaticojejunostomy with Roux-en-Y anastomosis should be performed. We prefer hepaticoduodenostomy with wide stoma in order to have free drainage. This procedure may avoid the development of adhesive intestinal obstruction, which may be the most important factor in the development of ascending cholangitis. Type Ib: Successful hepaticocholedochostomy after excision of the cyst is feasible if there is no stricture at the terminal duct. However, the T tube should be inserted into the choledochus from the porta hepatis, not from the anastomotic portion. Type Ic: Transduodenal sphincteroplasty with concomitant cholecystectomy has been a recommended procedure to insure free drainage of the bile, but in our experience incision up to the dilated choledochus is necessary to make a wide stoma. Types II and III. We do not have any recommendations for the treatment of these types because of the lack of experience, but excision of the diverticulum for type II cysts and transduodenal sphincteroplasty for type III cysts appear to be the treatments of choice. Type IV-A. Usually, excision of the choledochal cyst is the best procedure. However, in some cases the intra- and extrahepatic cysts are joined with the

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Congenital Bile Duct Cysts

narrowed common hepatic duct, as shown in Figure 4. In such cases, partial resection of the intrahepatic cyst with excision of the choledochal cyst should be performed, followed by intrahepatic cystoenterostomy at the porta hepatis. Mere anastomosis of the jejunum and the choledochal cyst or hepaticoenterostomy after excision of the choledochal cyst does not provide effective decompression of the intrahepatic cyst. Type IV-B. We have successfully performed excision of the choledochal dilatation and sphincteroplasty for choledochocele. In multiple cysts of the choledochus and hepatic duct, as in Longmires case [9], excision of the whole extrahepatic bile duct followed by hepaticoenterostomy at the porta hepatis may be an effective procedure. Type V. Although hepatectomy seems to be the treatment of choice for localized type V cysts, many questions on the treatment still remain for multiple types, as in Carolis disease. Recently, hepaticojejunostomy or choledochojejunostomy with wide stoma has been widely recommended but is not effective in all cases. In some cases cholangitis still occasionally develops.
Cancer Arislngfrom CholedochalCyst

Figure 4. Left, intra- and extrahepatic cysts are joined with the narrowed common hepatic duct (arrow). Right, postoperative cholangiogram after completed lntrahepatic cystojejunostomy (Roux-en-Y) with excision of choledochal cyst In the same patient.

The results of thirty-three cases of cancer arising from choledochal cysts [l&43] and three cases of cancer arising from intrahepatic bile duct cysts (type IV-A) [44,45] have been obtained from the current literature and personal communications. (Table III.) Moreover, five cases of hepatic and pancreatic cancer associated with choledochal cysts [46-501 have been reported. Hepatic cancer arising from type V cysts has also been documented recently [51,52]. It is interesting that most of these cases are in young adult and Japanese patients who had undergone interanal drainage, especially choledochocystoduodenostomy, several years previously. In the older children and young adults in the present series, the wail of the cyst usually tended to have inflamed scarred tissue and denuded epithelium. Such degenerated mucosa could develop into cancer as a result of chronic irritation, ulceration, and regeneration of the epithelium. On the other hand, the explanation suggested by Fortner [53] for the development of cancer from a choledochal cyst is very interesting: The structure of some compounds naturally excreted in bile could undergo abnormal changes metabolically to produce carcinogenic hydrocarbons owing to the close structural resemblance between deoxycholic acid and methylcholanthrene. The histologic types of cancer in reported cases vary from adenocarcinoma, squamous cell cancer,

and adenoacanthoma to undifferentiated carcinoma of the bile duct. In conclusion, we emphasize that excision is the treatment of choice for choledochal cysts in view of the likelihood of the development of cancer as well as the possibility of ascending cholangitis, although cancer of the intrahepatic cyst has occurred in one patient described by Gallagher, Millis, and Mitchinson [45] after excision of the choledochal cyst.
Summary

Thirty-seven patients with congenital bile duct cysts, including seventeen children and nine young adults, were encountered from 1960 to April 1976. Since the congenital bile duct cysts were observed in any parts of the bile duct, from the liver to the duodenum, we prefer to use the term bile duct cyst, and we classify these cysts into six types for surgical treatment, in contrast with Alonso-Lejs classification. Based on experience with two patients in whom cancer arose from a choledochal cyst, it seems that excision of the choledochal cyst is always the most desirable operation for types Ia and Ib cysts in older children and young adults. Partial resection of intrahepatic cysts should be added in some cases of type IV-A cysts to achieve free drainage of the bile juice from the intrahepatic cysts.
Acknowledgment: We are grateful to Professor S. C. Hsu, Department of Surgery, National Taiwan University, for his valuable suggestions and presen-

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TABLE Ill

Reported Cases of Cancer Arising from Choledochal Cyst

Sex/Age (yr) Ml30 ?I? F/33 Ml11 ?I? FI? M/11 F/50 F/19 F/38 F/28 F/30 F/26 Ml34 Ml49 F/32 F/68 M/30 F/36 F/48 F/63 F/23 F/34 F/28 F/26 F130 F/35 F/39 Ml33 F/15 F/16 F/17 Ml22 Age at Malignant Change (yr) 30 ? 35 18 ? 24 18 50 25 65 32 35 28 35 49 ? 68 30 63 52 68 25 28 28 26 30 35 45 33 15 20 19 26

Authors Irwin and Morrison [ 181 1944 Ferraris, Navarro, and Malbran [ 791 1944 Wilson, Morrissey, and McConnell [ZO] 1956 Fischer [21] 1958 Mckenzie, Sandy, and Anderson [ 221 1958 Kempter 1231 1959 Nakashima [ 241 1963 Ashbv 1251 1964 Fukuichi [>6] 1965 Macfarlance and Glenn [ 271 1967

Initial Operation Cholecystogastrostomy ? Cystojejunostomy Cystoduodenostomy ? ? Cystoduodenostomy Excision of the cyst Cystoduodenostomy Cholecystectomy and drainage from the cyst Cystojejunostomy Cystoduodenostomy Cystojejunostomy Gastrectomy elsewhere Indwelling catheterization Cystojejunostomy Cholecystectomy and partial hepatectomy Excision of the cyst Cholecystectomy Cholecystectomy Choledochoduodenostomy Cystojejunostomy Cystoduodenostomy with gastrojejunostomy Cystoduodenostomy Excision of the cyst Cystoduodenostomy Hepaticojejunostomy Cystoduodenostomy Cholecystectomy Cystogastrostomy Cystoducdenostomy Cystoduodenostomy Cystoduodenostomy

Pattiology Squamous cell cancer at autopsy Adenocarcinoma Adenocarcinoma Adenocarcinoma ? ? ? Adenocarcinoma at initial operation Papillary adenocarcinoma Papillary adenocarcinoma (type II) Adenocarcinoma Adenoacanthoma Undifferentiated carcinoma Adenocarcinoma Adenocarcinoma with liver metastasis Cancer Cancer at autopsy Adenocarcinoma at initial operation ? Undifferentiated carcinoma at autopsy Adenocarcinoma Adenocarcinoma Adenocarcinoma at initial operation Adenocarcinoma at initial operation Adenocarcinoma at initial operation (type IV-A) Adenocarcinoma at initial operation Adenocarcinoma at initial operation Adenocarclnoma Anaplastic cancer at initial operation Squamous cell cancer at initial operation Adenocarcinoma Squamous cell cancer Adenocarcinoma (type IV-A)

Hizawa et al [ 281 1967 Tanaka [29] 1968 University of Tokyo [30] 1969 Weber et al [37] 1971 Lbrenzo, Seed, and Beal Uchimura [33] 1972 Yoshimura [34] 1972 Glenn et al 1973 Takahashi [35] 1973

[ 321 197 1

Udaka and Komi [36] Hsu [37] 1974 Kakutani [38] 1974 lnugai [39] 1975

1973

Harada 1401 1975 Sat0 [4i] i975 Ohshima 1421 1976 Fukushim& dno, and Mitsuyasu 1976 Present report

[ 431

tation of a case with cancer arising in the choledochal cyst, and to Doctor A. Talbot and Miss A. Connie for their help in the preparation of this manuscript.
References
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14. Lindenauer SM. Lavanway JH: Surgical management of congenital choledochal cyst. Surgery 65: 972, 1969. 15. Trout HH III, Longmire WH Jr: Long-term follow-up study of patients with congenital cystic dilatation of the common bile duct. AmJSurg 121: 68, 1971. 16. lshida M, Tsuchida Y, Saito S, Hori T: Primary excision of choledochal cysts. Surgery 68: 884, 1970. 17. Kasai M, Asakura Y, Taira Y: Surgical treatment of choledochal cyst. Ann Surg 172: 844, 1970. 18. Irwin ST, Morison JE: Congenital cyst of common bile duct containing stones and undergoing cancerous change. Br J Surg32: 319, 1944. 19. Ferraris LV. Navarro A. Malbran JE: Dilatation congenita del hepatocoledoco y adenocarcinoma. 801 Sot Cir Cdrdoba 5: 21. 1944. 20. Wilson FD, Morrissey GE, McConnell RW: Bile duct cyst with malignant degeneration. J lowa Med Sot 46: 72, 1956. 21. Fischer HG: Prim&s Karzinom in der Wand einer angeborenen zystischen Gallengangserweiterung (sog. Hepatikuszyste). Zentrabl Chir 83: 1234, 1958. 22. Mckenzie AD, Sandy JT, Anderson DO: Choledochus cyst with associated carcinoma. Can J Surg 2: 88, 1958. 23. Kempter M: Cited in [ 441. 24. Nakashima S: A case of choledochus cancer with congenital cyst. Kotsuigaku (Tokyo) 16: 593, 1963. 25. Ashby BS: Carcinoma in a choledochus cyst. Rr JSurg 51: 493, 1964. 26. Fukuuchi T: Cancer arising from congenital choledochal cyst receiving internal drainage operation 7 years previously. Gekashinryo (Tokyo) 9: 1134, 1967. 27. Macfarlance JR, Glenn F: Carcinoma in choledochal cyst. JAMA 202: 1003, 1967. 28. Hizawa K. Fukuda F, Akagi G, Kitamuro T, Tao S: Carcinoma associated with idiopathic choledochus cyst. Acta Pathol Jap 17: 101, 1967. 29. Tanaka S: Cancerous change of the choledochal cyst. Rinshogeka (Tokyo) 23: 1215, 1968. 30. University of Tokyo: Personal communication. 31. Weber BB, Soler FJ. Font RG, Nance FC: Carcinoma arising in a choledochal cyst. Dig Dis 16: 1019, 1971. 32. Lorenzo GA, Seed RW, Beal JM: Congenital dilatation of the biliary tract. Am JSurg 121: 510, 1971. 33. Uchimura M: Operative procedures for congenital choledochal cyst. Shujutsu (Tokyo) 26: 577, 1972. 34. Yoshimura M: A case of cancer arising from choledochal cyst. Jap J Gastroenterol( Tokyo) 69: 780, 1972.

35. Takahashi M: Seven cases of the congenital choledochal cysts. J Jap Pratt Surg Sot (Tokyo) 34: 588. 1973. 36. Udaka H, Komi N: Personal communication. 37. Hsu SC: Personal communication. 38. Kakutani T: Personal communication. 39. lnugai I: Cancer of the choledochus with choledochal cyst. Oral presentation at the 5th Meeting, Japanese Gastrointestinal Surgical Society, Tokyo, 1975. 40. Harada H: Personal communication. 41. Sato H: A case of cancer arising in choledochal cyst. Oral presentation at the 1 lth Meeting, Diseases in Biliary Tract, Tokyo, 1975. 42. Ohshima S: Squamous cell cancer arising from huge choledochal cyst. Oral presentation at the 6th Meeting, Japanese Gastrointestinal Surgical Society, Kurume, 1976. 43. Fukushima M, Ono M, Mitsuyasu J: An autopsy case of cancer associated with choledochal cyst. Rinsho to Kenkyu (fukuoka) 52: 807, 1976. 44. Shiewe R, Baudish E, Ehrhardt G: Angeborene intrahepatische Gallengangszyste mit Steinbildung und maligner Entartung. Bruns Beitr Klin Chir 2 16: 264, 1968. 45. Gallagher PJ, Millis RR, Mitchinson MJ: Congenital dilatation of the intrahepatic bile ducts with cholangiocarcinoma. J C/in Pathol25: 804, 1972. 46. Armanio LP: Idiopathic dilatation of the common bile duct with coexistent primary hepatic carcinoma: report of a case. Ann Intern Med 24: 714, 1946. 47. Dextor D: Choledochal cyst with carcinoma of the intrahepatic bile ducts and pancreatic ducts. Br J Cancer 11: 18, 1957. 48. George PA, Maingot R: Choledochus cyst associated with carcinoma in the liver: report of a case. Br J Surg 50: 339. 1962. 49. Kelly TR, Schuster TM: Choledochal cyst with coexistent carcinoma of the pancreas. Am Surg 30: 209, 1964. 50. Thistlethwaite JR, Horwitz A: Choledochal cyst followed by carcinoma of the hepatic duct. South A&d J 60: 872, 1967. 5 1. Jones AW, Schreeve DR: Congenital dilatation of intrahepatic biliary ducts with cholangiocarcinoma. Br Med J 2: 277, 1970. 52. Yamamura T, Matsumori H. Kameyama Y, Hoshino T, Watanabe 0: Congenital bile duct cysts. Gekashinryo (Tokyo)18: 4 17, 1976. 53. Fortner JG An appraisal of the pathogenesis of primary carcinoma of the extrahepatic duct. Surgery 43: 563, 1958.

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