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 ::  Introduction
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Year : 1979  |  Volume : 25  |  Issue : 3  |  Page : 177-180

Choledochal cyst

Department of Paediatric Surgery and Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India

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Department of Paediatric Surgery and Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh
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PMID: 529172

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 :: Abstract 

Choledochal cyst, a cystic dilatation of a part of biliary system, is a rare clinical problem. Three such cases are reported. The etiology, clinical manifestations, diagnostic problems and the Management are discussed.

How to cite this article:
Rao P, Katariya R N, Pathak I C. Choledochal cyst. J Postgrad Med 1979;25:177-80

How to cite this URL:
Rao P, Katariya R N, Pathak I C. Choledochal cyst. J Postgrad Med [serial online] 1979 [cited 2023 May 31];25:177-80. Available from:

 :: Introduction Top

Choledochal cyst, a cystic dilatation of a part of biliary system, is a rare clinial entity. Since the description of the condition a century ago, just over 1000 cases, mostly from Japan, have been re­ported in the world ­ literature. AlansoLej. et al [1] classified the choledochal cyst into three types. Caroli's disease, which is an intra-hepatic cystic dilatation of the biliary ducts, was later added as type IV choledochal cyst by Jones and Olbourn. [6] To the best of our knowledge only three isolated case reports have so far been published in the Indian literature, none being of Caroli's variety. Three cases of choledochal cysts, one of which is of Carob's variety are presented.

 :: Case reports Top

Case I

A 17 year old female was admitted on 1-7-68 with a history of intermittent epigastric pain and fever of 2 years' duration. She also had periodic attacks of dysentery and exertional dyspnoea of the same duration. On examina­tion, she was found to be normotensive, anicteric, but had fever of 99.5°F. Liver was enlarged by 7 cms. below the costal margin in the mid-clavicular line with a smooth surface. Investigations revealed a leukocytosis of 20,800/ cmm with 80% polymorphs. Stool examination for E.H. was negative. A provisional diagnosis of amoebic liver abscess was made. Aspiration of liver was tried with negative results. Inspite of lack of positive evidence for amoebic liver abscess, she was put on Chloroquin and Enter­ovioform to which she responded. Her pain and fever subsided, tenderness decreased and she was discharged on 11-7-68. Ten days later she was readmitted with the same clinical picture as before. This time about 500 ml. of green coloured bile was aspirated from the liver which grew  Salmonella More Details typhi on culture. Con­trast dye was infected into the liver after aspiration and the radiological examination showed widely dilated cysts in both lobes of the liver. On 27-8-68 exploratory laparotomy was carried out which revealed a cystic swelling ors the under surface of the liver which was con­tinuous with what appeared to be a markely dilated common bile duct. An operative cholan­giogram revealed large cystic spaces within the liver. In addition, peripheral biliary channels were also slightly dilated (see [Figure 1] on page 178B). A cystoduodenostomy was done in two layers. Post-operatively, the patient was wel for about one year. On 27-10-69 she was read­mitted with cholangitis. This time liver biopsy showed ascending cholangitis. Blood culture grew E. coli. She was started on chloram­phenicol but on 11-11-1969 she went into endo­toxic shock from which she could not be revivec and she expired on 12-11-1969.

Case 2

P. K., an 11 month old male child was ad­mitted on 2-9-1970 with history of gradually in­creasing abdominal girth and vomiting off and on since birth. On examination, he was found to be afebrile and anicteric. Abdomen revealed a tender cystic ballotable swelling occupying the right hypochondrium and right lumbar region. There was no other organomegaly. With a provisional diagnosis of hydronephrosis of the right kidney an intravenous urography was done which revealed functioning normal kidneys on both sides. Laparotomy done on 8­9-70 revealed a midly enlarged liver and there was a choledochal cyst of 3" diameter involving the common bile duct. The gall bladder and the cystic duct were distended. Roux-en-Y choledochocysto-jejunostomy and liver biopsy were carried out. Liver biopsy showed evidence of cirrhosis. Post-operatively, the child was well for about one year and then was re-ad­mitted with cholangitis, on 7-9-71. At this time he responded well to antibiotics. He was asymptomatic till 1976 when he was readmitted on 20-11-1976 with history of colicky abdominal pain of 11 months' duration. This time a vague non-tender mass was felt in the right hypo­chondrium. Hence he was re-explored on 23­11-1976 which revealed only post-operative adhesions. Lysis of adhesions and operative cholangiogram was carried out which was nor­mal. Post-operatively, the child did well and is asymptomatic to date.

Case 3

N.R., a 28 year old female had been having recurrent attacks of pain in the right hypo­chondrium, vomiting and jaundice since 1970. In September 1970, after about 3 months of initial illness she was explored at another hospi­tal with a provisional diagnosis of cholecystitis. A choledochal cyst was found and a cholecysto­jejunostomy + jejunojejunostomy were carried out elsewhere. This relieved the patient of her symptoms only for about 3 months. An intra­venous cholangiogram done in 1977 revealed a persistant choledochal cyst and the patient was referred to us on 10-8-1977. On examination, she was found to be anicteric and afebrile. There was a right upper paramedian abdominal scar. Beneath the upper part of the scar a vague non-tender mass was felt. There was no organomegaly. Her haematological and bio­chemical investigations including liver function tests were within normal limits. She was ex­plored on 17-8-1977 through the same old in­cision scar. At operation a choledochal cyst of 4.5 x 3.5 cms size involving the supraduodenal portion of the common bile duct and a little of common hepatic duct was noted. The cystic duct was directly opening into the cyst. An operative cholangiogram confirmed the findings (see [Figure 2] on page 176B). A choledochocysto­duodenostomy was done in one layer. Post­ operatively, patient had uneventful recovery and is asymptomatic to date.

 :: Discussion Top

Though it was attributed to Valter to have described the condition in 1723, it was Douglas [3] who gave the documented description of choledochal cyst in a 17 year old girl in 1852. The condition is re­ported to be common in Japan and Korea and relatively uncommon in Europe and America, one third of the cases reported in the literature having been from Japan. [10] Of the three cases reported here, one is of Caroli's disease (Case 1) and two are of type 1 choledochal cysts (Cases 2 and 3). Till to date there have been only 55 documented cases of Caroli's disease in the literature. [5]

The disease is four times more com­mon in females than in males; two of our cases are females. Our patients present­ed to us at the age of 17 years, 11 months and 28 years respectively (Case 1 to 3), though they had been symptomatic for quite some time. It is described that 60% of the cases present in the 1st decade, and another 10-20% in the second decade of the life. [4] It is also stated that intra­hepatic involvement of bile ducts possibly delays the development of symp­toms and hinders an early diagnosis, [13] as was seen in our first case.

The etiology of the condition is not yet established beyond doubt. A number of theories have been put forth to explain the aetiogenesis. Most of the workers be­lieve it to be congenital [6],[9],[12] whereas a few believe it to be acquired. [2] The theory of Yotuyanagi according to which there is inequality in the proliferation of epithelial cells of the primitive choledo­chus during the embryonic stage, and the combined theory according to which there is proximal weakness of the com­mon bile duct wall with distal obstruc­tion, are widely accepted. [9],[11]

Fonkalsrud [4] in 1973 described the classical triad of the diseases; i.e. Jaun­dice, pain and swelling. The classical triad was present only in the third case whereas Case 1 had, pain and swelling and Case 2 had only swelling. The classical triad has been reported to be present only in 19-30% of cases whereas in 46.4% of the cases only two of the three features are present. [8]

A number of investigations like infusion cholangiogram, Rose Bengal scan, ultrasonic echography, endoscopic transduodenal retrograde cholangiogram and arteriogram have been used to establish the diagnosis pre-operatively. Only one of the three cases (Case 1) had a correct pre-operative diagnosis. It is described that patients of Caroli's disease stay as typhoid carrier for a long time in spite of adequate treatment. [5]

Various surgical procedures that have been carried out to deal with choledochi cysts are: (a) primary excision of the cyst with Roux-en-Y choledocho-enterostomy; (b) Roux-en-Y choledochal cysto­jejunostomy and (c) Choledochocysto­duodenostomy. Out of these, the first procedure is claimed to be the most superior, [11] but is not always possible be­cause of the danger to the structures in the porta hepatis, especially when there are repeated attacks of cholangitis leading to adhesion formation. Of the remaining two procedures, Roux-en-Y choledocho­cysto-jejunostomy is by and large pre­ferred over the choledochocysto-duodeno­stomy. [7] Two of our patients had choledo­chocysto-duodenostomy and one of them (Case 1) died as a result of severe cholangitis. The patient (Case 2) who had Roux-en-Y choledochocysto-jejuno­stomy also had mild cholangitis. We can not really comment on the superiority of one procedure over the other as our experience is limited.

 :: References Top

1.Alonso-Lej, F., Rever, W. B. and Pessangno, D. J.: Congenital choledochal cysts: With a report of two and analysis of 94 cases. Int. Abst. Surg., 108: 1-30, 1959.  Back to cited text no. 1    
2.Burnell, R. H. and Markey, G. B.: Choledochal cyst. Arch. Dis. Child., 40: 329-331, 1965.  Back to cited text no. 2    
3.Douglas, A. H.: A case of dilatation of the common bile duct. Monthly J. Med. Sci. (London), 14: 97, 1852. Quoted by Flanigan D. P. Biliary cysts. Ann. Surg., 182: 635-643, 1975.  Back to cited text no. 3    
4.Fonkalsrud, E. W.: Choledochal cysts. Surg. Clin. North Amer., 53: 1275-1281, 1973.  Back to cited text no. 4    
5.Hamlyn, A. N., Douglas, A. P., James, 0. F. W., Lavelle, M. I. and Venebels, C. W.: Caroli's disease with intrahepatic gall stones and Salmonella infection. Postgrad. Med. J., 52: 656-659, 1976.  Back to cited text no. 5    
6.Jones, C. A. and Olborne, N. A.: Choledochal cyst with associated choleli­thiasis diagnosed by infusion cholangiography and tomography. Brit. J. Radial., 46: 711-714, 1973.  Back to cited text no. 6    
7.Klotz, D., Cohn, B. D. and Kottmier, P. K.: Choledochal cysts: Diagnostic and therapuetic problems. J. Pediat. Surg., 8: 271-283, 1973.  Back to cited text no. 7    
8.Lee, S. S., Min, P. C., King, G. W. and Hong, P. W.: Choledochal cyst. Arch. Surg., 99: 19-28,1969.  Back to cited text no. 8    
9.Loubean, J. M. and Steichen, F. M.: Dilatation of intrahepatic bile ducts in choledochal cyst. Arch. Surg., 111: 1384­1390, 1976.  Back to cited text no. 9    
10.Louw, J. H.: Choledochal cysts. S. Afr. J. Surg., 13: 199-205, 1975.  Back to cited text no. 10    
11.Saito, S. and Ishida, M.: Congenital choledochal cyst. Progress in Pediatric Surgery. 6: 63-90, 1974.  Back to cited text no. 11    
12.Stefanini, M., Urbas, J. E. and Crockett, F. L.: Choledochal cyst with associated anomaly of the head of the pancreas. Amer. J. Gastroenterol., 54: 496-499, 1970.  Back to cited text no. 12    
13.Wei-Jaochen, Chan-Hisung and Wen­Tsung Hung: Congenital choledochal cyst. J. Pediatr. Surg., 8: 529-538, 1973.  Back to cited text no. 13    


  [Figure 1], [Figure 2]


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