Choledochal cystPLNG Rao, RN Katariya, IC Pathak
Department of Paediatric Surgery and Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 529172
Source of Support: None, Conflict of Interest: None
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.
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
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.
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.
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.
Though it was attributed to Valter to have described the condition in 1723, it was Douglas  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.  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. 
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.  It is also stated that intrahepatic involvement of bile ducts possibly delays the development of symptoms and hinders an early diagnosis,  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 ,, whereas a few believe it to be acquired.  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. ,
Fonkalsrud  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. 
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. 
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,  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.  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.
[Figure 1], [Figure 2]