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Experience with choledocal cyst
Experience With Choledocal Cyst By J. Valayer and D. Alagille U R I N G T H E 5-yr period between 1967 and 1972, ten children with choledocal cyst were operated upon at Saint-Vincent de Paul's Hospital in Paris. Cases of cystic dilatation of the c o m m o n bile duct, as it may occasionally be seen during surgical exploration for biliary atresia and called Type I correctable form by Kasai? were not included in our series of choledocal cysts. Nevertheless, we feel concerned about the somewhat artificial distinction between these two anomalies, as far as anatomical description goes; yet, if pathogenesis in both conditions remains obscure, it is mostly the age of the patients and the date of appearance of the jaundice that makes the difference. Perhaps the same mechanism brings different effects by different degrees o f obstruction. Apart from the fact that the girls o u t n u m b e r the boys (4/1 ratio) as in other series, we have noted the following points about the clinical symptoms: (1) Although most of the patients were operated upon after 1 yr of age, very few were symptom-free in the first year of life. This was the case in only three patients who presented with jaundice or pain at 2, 3, and 11 yr, respectively. (2) The classic triad of jaundice, mass, and pain was encountered in one case only. This fact is in accordance with data from a recent review of the literature by Kottmeier. 2 (3) Jaundice was usually present to a moderate degree and in three cases associated with fever. About the abdominal mass, another important symptom in choledocal cyst, we should like to emphasize the fact that irregular enlargement of the right lobe of the liver in other causes of cholestasis may lead to false diagnosis of choledocal cyst. As most patients were very young when the first symptoms appeared, it is difficult to assess the frequency of pain. (4) One patient presented with severe bleeding from esophagal varices; long delay in diagnosis was due to the fact that jaundice was intermittent and only moderate. Operation was carried out at 10 yr of age although the first symptoms occurred at age 2. (5) One patient was first operated upon elsewhere for acute bile peritonitis syndrome; the cause and the proper diagnosis were found and made only a few months later when jaundice reappeared and the child was reoperated upon. The correct preoperative diagnosis was made in nine cases, both by clinical and by radiographic means. Intravenous or oral cholangiography was performed in six instances and gave positive findings in five, although some pictures were misleading because of dilution of the dye in a large cavity. Rose
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From the Department of Pediatric Surgery, Hbpital Saint Vincent de Paul, Paris, France. Presented before the XXlst International Congress of the British Association o f Paediatric Surgeons, Berne, Switzerland, August 27-31, 1974. Address for reprint requests: J. Valayer, M.D., Hbpital Saint Vincent de Paul, 74th Avenue DenFert Rochereau, 74014 Paris, France. 9 1975 by Grune & Stratton, lnc. Journal of Pediatric Surgery, Vol. 10, No. 1 (February), 1975
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Bengal scintiscan was done in two cases, but did not appear to us as a very useful adjunct to otherwise meaningful and simpler diagnostic procedures. Except in the case of cirrhosis with portal hypertension mentioned above, and apart from the usually modified bile-excretion tests, the liver function appeared biologically normal. However, the examination of the liver biopsy specimens taken at operation showed a mild to severe degree of fibrosis in five cases; in only two cases were the findings considered normal. The precise anatomic description of the cystic dilatation and its extension to intrahepatic bile ducts should be obtained by preoperative cholangiograms and also by examination of the open cyst and search for the opening of each duct. Cholangiograms should not be obtained by injection through the cyst's wall but rather by insertion of a catheter into each bile duct opening into the top of the pouch. Dilatation of intrahepatic bile ducts was found in one case out of five in which a cholangiogram was done. This association with dilated intrahepatic bile ducts is rather a rare condition and has already been well defined by Ishida. 3 When dilatation is noted, it may be segmental and have more a congenital aspect rather than a retrograde dilatation above some acquired obstruction; in most cases when no dilatation above the extrahepatic cyst can be seen, it may be that the elasticity of the cyst's wall itself acts so as to absorb retrograde pressure. The gallbladder was removed in all cases except one, because the cystic duct was involved in the cystic dilatation. The common bile duct may not always be isolated, although its patency may be proved by colored stools before the operation. It was missed by the cholangiogram in two cases out of five, and curiously enough, it was also missed at operation when a cystectomy was performed in three cases out of five, probably by artifact of dissection. Capacity of the cyst varied from 20 to 600 ml, and intracystic pressure was measured in one case only: it was below 25 cm of water. Cholelithiasis was seen in three cases, and one of these was after recurrent episodes of angiocholitis in a child aged 11 in whom a previous operation had consisted of partial removal of the cyst wall and Rouxen-Y anastomosis. In three out of four pathological examinations of the cyst wall, there were only patches of epithelium lining to be seen, or none at all; this well-established fact is one of the reasons for removal of the cyst. Surgical treatment in our series changed from simple partial excision to complete removal of the choledocal cyst after we had seen this last operation performed by Kasa? in Japan. Our first case was treated by choledocoduodenostomy. Partial excision of the lateral aspect of the cyst, with removal of the gallbladder and anastomosis of the jejunal loop at the lower part of the opening in the cyst wall was done in five cases. One of these was a case with a very thick cyst wall and also a very unusual fibrous cord 1 cm in diameter going across the cystic cavity from one wall to the other. This cord was left in place fearing it would be developed around the right hepatic artery. But as recurrent episodes of angiocholitis took place, a total excision of the cyst had to be performed a
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few months later, with no special difficulties in relation to this fibrous cord, which contained only a small artery. Total excision of the cyst with Roux-en-Y anastomosis to the hepatic ducts was done in five cases. Although no particular incidents occurred during performance of this technique, we are well aware of possible injuries to important proximal structures such as the pancreatic ducts or the hepatic arteries. Hemorrhage from the cyst wall vessels may be rather overwhelming during the dissection of the cyst, but we have noted that the bleeding regularly ceased once the resection was completed. Incidentally, the bleeding was not a problem in the patient with portal hypertension. There was one death, 3 wk after the operation. This child had a very particular history with correction of Rh isoimmunization at birth by exchange transfusions, and persistence of neurological sequelae. Jaundice appeared at 1 mo of age and surgical exploration was done at 7 mo. Operative cholangiogram showed a fusiform dilatation of the common bile duct with extension of the pathologic aspect to both hepatic ducts. Liver biopsy showed an important degree of fibrosis, which was explained by long-term and severe cholestasis. Although the child had slightly colored stools after the operation, the jaundice remained unchanged and death occurred with infection and generalized bleeding. There was one case of postoperative intestinal obstruction, after choledococystoduodenostomy; duodenal opacification for investigation of that incident showed massive reflux of the barium into the biliary tract. The mean follow-up time was just over 1 yr, but that includes two children, native from overseas, whom we lost sight of within 6 and 8 mo, respectively. The six other children were seen for examination between t and 389 after operation. They were all in good clinical state, but it was generally noted that the liver still seemed to be slightly enlarged. No secondary liver biopsies have yet been obtained; liver-function tests, including bromsulfophthaline retention test have been done for every child and have shown normal results. In the child with portal hypertension, it was noted that 2 yr after the operation, the liver was still palpable, but much reduced in volume and not so hard, and also that the spleen remained moderately enlarged. Biologic tests were satisfactory, but the blood count showed some definite degree of hypersplenism. Although splenoportography gave similar pictures of spontaneous portocaval shunting, as in the preoperative examination, the pressure in the spleen was less than in the previous examination. Esophageal varices are still visible at endoscopic investigation. SUMMARY
In a series of ten children operated upon for congenital choledocal cyst, it was noted that the diagnosis had often been delayed because of intermittent or moderate symptoms during the first year of life. We favor resection of the cyst, advocated by many authors recently, but it should be emphasized that this procedure is not without risk and may be quite a difficult undertaking. Late results were satisfactory on t h e whole, even in one child with severe portal
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h y p e r t e n s i o n . H o w e v e r , the m o d e r a t e fibrosis f o u n d at o p e r a t i v e b i o p s y a n d the still slightly e n l a r g e d liver at f o l l o w - u p e x a m i n a t i o n are r e a s o n s for l o n g - t e r m o b s e r v a t i o n o f these p a t i e n t s . REFERENCES
1. Kasai M: Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modifications. Progr Pediatr Surg 6:5, 1974 2. Klotz D, Cohn BD, Kottrneier PU: Choledocal cysts: Diagnostic and therapeutic
problems. JPediatrSurg8:271, 1973 3. Tsuchida Y, Ishida M: Dilatation of the intrahepatic bile ducts in congenital cystic dilatation of the common bile duct. Surgery 69:776, 1971
D
From the Department of Pediatric Surgery, Hbpital Saint Vincent de Paul, Paris, France. Presented before the XXlst International Congress of the British Association o f Paediatric Surgeons, Berne, Switzerland, August 27-31, 1974. Address for reprint requests: J. Valayer, M.D., Hbpital Saint Vincent de Paul, 74th Avenue DenFert Rochereau, 74014 Paris, France. 9 1975 by Grune & Stratton, lnc. Journal of Pediatric Surgery, Vol. 10, No. 1 (February), 1975
65
66
VALAYER AND ALAGILLE
Bengal scintiscan was done in two cases, but did not appear to us as a very useful adjunct to otherwise meaningful and simpler diagnostic procedures. Except in the case of cirrhosis with portal hypertension mentioned above, and apart from the usually modified bile-excretion tests, the liver function appeared biologically normal. However, the examination of the liver biopsy specimens taken at operation showed a mild to severe degree of fibrosis in five cases; in only two cases were the findings considered normal. The precise anatomic description of the cystic dilatation and its extension to intrahepatic bile ducts should be obtained by preoperative cholangiograms and also by examination of the open cyst and search for the opening of each duct. Cholangiograms should not be obtained by injection through the cyst's wall but rather by insertion of a catheter into each bile duct opening into the top of the pouch. Dilatation of intrahepatic bile ducts was found in one case out of five in which a cholangiogram was done. This association with dilated intrahepatic bile ducts is rather a rare condition and has already been well defined by Ishida. 3 When dilatation is noted, it may be segmental and have more a congenital aspect rather than a retrograde dilatation above some acquired obstruction; in most cases when no dilatation above the extrahepatic cyst can be seen, it may be that the elasticity of the cyst's wall itself acts so as to absorb retrograde pressure. The gallbladder was removed in all cases except one, because the cystic duct was involved in the cystic dilatation. The common bile duct may not always be isolated, although its patency may be proved by colored stools before the operation. It was missed by the cholangiogram in two cases out of five, and curiously enough, it was also missed at operation when a cystectomy was performed in three cases out of five, probably by artifact of dissection. Capacity of the cyst varied from 20 to 600 ml, and intracystic pressure was measured in one case only: it was below 25 cm of water. Cholelithiasis was seen in three cases, and one of these was after recurrent episodes of angiocholitis in a child aged 11 in whom a previous operation had consisted of partial removal of the cyst wall and Rouxen-Y anastomosis. In three out of four pathological examinations of the cyst wall, there were only patches of epithelium lining to be seen, or none at all; this well-established fact is one of the reasons for removal of the cyst. Surgical treatment in our series changed from simple partial excision to complete removal of the choledocal cyst after we had seen this last operation performed by Kasa? in Japan. Our first case was treated by choledocoduodenostomy. Partial excision of the lateral aspect of the cyst, with removal of the gallbladder and anastomosis of the jejunal loop at the lower part of the opening in the cyst wall was done in five cases. One of these was a case with a very thick cyst wall and also a very unusual fibrous cord 1 cm in diameter going across the cystic cavity from one wall to the other. This cord was left in place fearing it would be developed around the right hepatic artery. But as recurrent episodes of angiocholitis took place, a total excision of the cyst had to be performed a
CHOLEDOCAL CYST
67
few months later, with no special difficulties in relation to this fibrous cord, which contained only a small artery. Total excision of the cyst with Roux-en-Y anastomosis to the hepatic ducts was done in five cases. Although no particular incidents occurred during performance of this technique, we are well aware of possible injuries to important proximal structures such as the pancreatic ducts or the hepatic arteries. Hemorrhage from the cyst wall vessels may be rather overwhelming during the dissection of the cyst, but we have noted that the bleeding regularly ceased once the resection was completed. Incidentally, the bleeding was not a problem in the patient with portal hypertension. There was one death, 3 wk after the operation. This child had a very particular history with correction of Rh isoimmunization at birth by exchange transfusions, and persistence of neurological sequelae. Jaundice appeared at 1 mo of age and surgical exploration was done at 7 mo. Operative cholangiogram showed a fusiform dilatation of the common bile duct with extension of the pathologic aspect to both hepatic ducts. Liver biopsy showed an important degree of fibrosis, which was explained by long-term and severe cholestasis. Although the child had slightly colored stools after the operation, the jaundice remained unchanged and death occurred with infection and generalized bleeding. There was one case of postoperative intestinal obstruction, after choledococystoduodenostomy; duodenal opacification for investigation of that incident showed massive reflux of the barium into the biliary tract. The mean follow-up time was just over 1 yr, but that includes two children, native from overseas, whom we lost sight of within 6 and 8 mo, respectively. The six other children were seen for examination between t and 389 after operation. They were all in good clinical state, but it was generally noted that the liver still seemed to be slightly enlarged. No secondary liver biopsies have yet been obtained; liver-function tests, including bromsulfophthaline retention test have been done for every child and have shown normal results. In the child with portal hypertension, it was noted that 2 yr after the operation, the liver was still palpable, but much reduced in volume and not so hard, and also that the spleen remained moderately enlarged. Biologic tests were satisfactory, but the blood count showed some definite degree of hypersplenism. Although splenoportography gave similar pictures of spontaneous portocaval shunting, as in the preoperative examination, the pressure in the spleen was less than in the previous examination. Esophageal varices are still visible at endoscopic investigation. SUMMARY
In a series of ten children operated upon for congenital choledocal cyst, it was noted that the diagnosis had often been delayed because of intermittent or moderate symptoms during the first year of life. We favor resection of the cyst, advocated by many authors recently, but it should be emphasized that this procedure is not without risk and may be quite a difficult undertaking. Late results were satisfactory on t h e whole, even in one child with severe portal
68
VALAYER AND ALAGILLE
h y p e r t e n s i o n . H o w e v e r , the m o d e r a t e fibrosis f o u n d at o p e r a t i v e b i o p s y a n d the still slightly e n l a r g e d liver at f o l l o w - u p e x a m i n a t i o n are r e a s o n s for l o n g - t e r m o b s e r v a t i o n o f these p a t i e n t s . REFERENCES
1. Kasai M: Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modifications. Progr Pediatr Surg 6:5, 1974 2. Klotz D, Cohn BD, Kottrneier PU: Choledocal cysts: Diagnostic and therapeutic
problems. JPediatrSurg8:271, 1973 3. Tsuchida Y, Ishida M: Dilatation of the intrahepatic bile ducts in congenital cystic dilatation of the common bile duct. Surgery 69:776, 1971