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Biliary Duct Cysts
Biliary Duct Cysts KENNETH W. WARREN, M.D. GABRIEL A. KUNE, M.B., B.S., F.R.A.C.S. KENNETH J. HARDY, F.R.A.C.S.
Because cystic dilatations of the biliary tract are uncommon, their variable clinical and pathologic features present problems in diagnosis and surgical treatment. The incidence of these lesions is unknown because many are not reported, and we think that many remain asymptomatic. An early description of the pathologic anatomy of a choledochal cyst was given by Douglas5 in 1852. Sporadic reports since then enabled Alonso-Lej, Rever, and Pessagno 1 in 1959 to record 419 instances from the literature. The present study is based on 11 patients with biliary duct cysts treated at the Lahey Clinic Foundation. This report does not include those patients in whom biopsy showed a biliary epithelial lining of the intrahepatic cyst that had no demonstrable connection with the bile ducts. There were nine women and two men in the group. Their ages at the time of presentation ranged from 9 months to 65 years; eight of these patients were under 40 years of age. In three patients there was a cystic dilatation of the left hepatic duct, and one of these three also had other intrahepatic cysts. Eight patients in the series had choledochal cysts. The patients were divided into two groups: those with cysts of the left hepatic duct and those with choledochal cysts. A brief case report is given of each patient in order to illustrate the variability of clinical and pathologic features as well as the nature and results of the various types of surgical treatment that were employed.
CHOLEDOCHAL CYSTS CASE 1. In 1964 a woman of 37 years complained of right upper quadrant pain and weight loss of short duration but no jaundice. At operation, a 5 cm. choledochal cyst was found containing gallstones. Multiple stones and biliary debris also were found in the proximal hepatic ducts and gallbladder. The distal common duct was occluded almost completely by a web-like structure, 1.5 cm. proximal to the sphincter of Oddi. Cholecystectomy and a 60 per cent excision of the choledochal cyst were performed; in addition, the distal common bile duct was enlarged by Bakes dilators. The remainder of the cyst was sutured longiSurgical Clinics of North America- Vol. 48, No.3, June, 1968
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KENNETH W. WARREN, GABRIEL
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J.
HARDY
Figure 1 (Case 1). T-tube cholangiogram showing the recurrent choledochal cyst. The distal common duct is occluded, and no contrast medium is seen in the duodenum.
tudinally over a T tube. Because jaundice developed postoperatively and clamping of the T tube caused abdominal pain, the patient was referred to the Lahey Clinic for further treatment. A T tube cholangiogram showed a choledochal cyst, 5 cm. in diameter, without filling of the distal common bile duct (Fig. 1). At operation, a fusiform choledochal cyst was found, and the distal common duct was stenotic. The cyst and distal common duct were excised, and the proximal duct was anastomosed to the jejunum with an entero-anastomosis and occlusion of the proximalllmb of the loop jejunostomy. The anastomosis was splinted with a rubber Y tube.'7 The patient remains well without symptoms two years later. CASE 2. A 33-year-old woman was seen at the Lahey Clinic in 1948 with a history of repeated upper abdominal pain and a past history of three biliary tract procedures that included cholecystectomy for gallstones, dilatation of a fibrotic sphincter of Oddi, and a side-to-side choledochojejunostomy for recurrent fibrosis of the sphincter of Oddi. Clinical and laboratory investigations of the patient gave negative results. At operation, a large choledochal cyst, stones in the common bile duct, stenosis of the sphincter of Oddi, and a papilloma of the ampulla of Vater were found. In addition, the choledochojejunostomy had stenosed completely. The biliaryintestinal anastomosis was dismantled, the stones removed, and a sphincterotomy made after transduodenal excision of the papilloma. Reoperation was necessary in 1954 because of persistence of pain. The sphincter of Oddi again was stenosed and the choledochal cyst again contained stones and debris. In addition, a choledochal cyst-duodenal fistula was present; the liver was enlarged and cirrhotic. The internal fistula was divided, the duodenum was closed, and a side-to-side choledochocystojejunostomy was made. On the second day after operation, shock occurred that was associated with severe abdominal pain, fever, and a serum amylase value of 1,850 Somogyi units. Anuria developed, and the patient died on the fourth postoperative day. Necropsy was not performed.
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CASE 3. A baby of 9 months, deeply jaundiced since birth and with marked hepatosplenomegaly, was operated on in 1943. A large choledochal cyst was found. The cystic artery passed anterior to the cyst, but the cystic duct was not demonstrated. Incomplete descent of the cecum was present also. The cyst was opened, and a probe was passed into each hepatic duct with ease, but the opening of the distal bile duct was not found. External catheter drainage of the cyst was followed by choledochocystojejunostomy two months later when the general condition of the baby permitted the operation. The baby died two years later from liver failure, but in the intervening period the jaundice cleared clinically. CASE 4. A cystic mass was palpable in the right upper quadrant of the abdomen in addition to hepatomegaly in a 3-year-old boy with jaundice. He had suffered fever, pain, and vomiting before admission in 1955. Liver function tests indicated obstructive jaundice. An upper gastrointestinal barium series showed anterior elevation of the distal part of the stomach and displacement of the duodenal loop (Fig. 2). A choledochal cyst, 10 cm. in diameter, was found at operation. The gallbladder was distended, and the liver was enlarged. The cyst was opened and the proximal hepatic duct probed with ease, but the distal opening of the common bile duct was not found. Choledochocystojejunostomy with a diverting enteroanastomosis was performed. Reexploration was carried out 17 days after operation because fever and the clinical signs suggested subhepatic abscess. However, no abscess was found. The cystojejunostomy was patent, but the cyst was distended with bile. It was decompressed by drainage with a large de Pezzer catheter. Cholangiography of the cavity through the de Pezzer catheter five months later revealed the anastomosis to be satisfactory with a decrease in size of the cyst. The catheter was removed. The patient was in excellent health ten years after operation. CASE 5. A 13-year-old girl was seen in 1965 with a four-month history of right-sided upper abdominal pain and vomiting. Four years previously, a choledo-
Figure 2 (Case 4). Barium meal examination before operation. Considerable anterior displacement of the distal portion of the stomach is seen in the lateral view.
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KENNETH W. WARREN, GABRIEL
A.
KUNE, KENNETH
J.
HARDY
Figure 3 (Case 5). Barium meal examination following choledochal cyst duodenostomy. Barium outlines the anastomosis, cyst, gallbladder, hepatic ducts and part of the pancreatic duct system. Some barium remained in the choledochal cyst for a few hours after this procedure.
chocystoduodenostomy had been performed elsewhere. Barium meal examination demonstrated a patent choledochocystoduodenostomy with barium freely flowing through the anastomosis outlining a choledochal cyst, the gallbladder, the hepatic ducts, and part of the pancreatic duct system, but some of the barium was seen to remain in the choledochal cyst for several hours (Fig. 3). At operation in 1965, a choledochal cyst with a patent choledochocystoduodenostomy was found. The proximal hepatic ducts were normal, but the distal common bile duct was narrowed at its lower end. There was a large gallbladder folded on itself with a dilated cystic duct that opened into the choledochal cyst itself. Most of the cyst was excised, preserving the cystoduodenostomy. The distal common bile duct was dilated with Bakes dilators, and a transduodenal sphincterotomy with a long-limb T tube was combined with cholecystectomy and pyloroplasty. The remainder of the cyst was closed over another long-limb T tube, the vertical limb of which was brought to the surface through a separate incision in the common hepatic duct. In the postoperative period, a biliary fistula developed that healed within a few days. Six months after operation the T tube was removed, and no further symptoms have occurred since the operation. CASE 6. Recurrent attacks of epigastric pain and jaundice had occurred in a woman of 29 years since the age of 7. External drainage of a choledochal cyst when the patient was 17 was followed a year later by choledochocystogastrostomy. This relieved her symptoms for 12 years when she again became jaundiced, requiring cholecystectomy and removal of stones from the choledochal cyst. In 1961 a few months later, reexploration was performed at the Lahey Clinic because of recurrent abdominal pain and jaundice. The cystogastrostomy was almost completely occluded. The cyst was excised, and hepaticojejunostomy was carried out using a Y tube as an internal stent. In 1963, this tube became occluded and was removed. She was well until 1965 when cholangitis developed. At operation, a stricture of the hepaticojejunostomy was found, and a new anastomosis was constructed using a Y tube stent. She was symptomless five months after operation.
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CASE 7. A 38-year-old man in 1953 gave a history of recurrent pain in the right upper quadrant of the abdomen since the age of 6 years. A cholecystectomy and choledocholithotomy had been done in the past with subsequent reoperation for an impacted stone in the lower end of the common bile duct at which time choledochostomy was combined with transduodenal sphincterotomy. Recurrent attacks of jaundice associated with chills and fever followed the second operation. At operation in 1953 a large cystic dilatation was found to involve the common hepatic, left hepatic, and right hepatic ducts. The cystic dilatation of the common bile duct measured 5.0 cm. in diameter. The proximal ducts were probed with ease, but the opening for the distal common bile duct could not be found. A sideto-side choledochojejunostomy was constructed with a stoma of 5.0 cm. together with a proximal entero-anastomosis. The postoperative course was uncomplicated and there have been no further symptoms. CASE 8. Three months before admission in 1957 a 23-year-old woman had a cholecystectomy for gallstones and drainage for a postoperative biliary collection. Jaundice followed this, and the patient lost 30 pounds in weight. At operation at the Lahey Clinic, a large choledochal cyst was found that contained 200 ml. of bile. The proximal hepatic ducts were probed with ease, but the opening of the distal common bile duct was not found. Choledochocystojejunostomy with a proximal entero-anastomosis was performed. Biopsy of the cyst wall was reported as a fibrous-walled cyst with chronic inflammation. The patient remains well ten years later.
CYSTIC DILATATIONS OF THE LEFT HEPATIC DUCT CASE 9. A 27-year-old woman came to the Lahey Clinic in 1946 with jaundice of 15 months' duration, a biliary fistula, and a history of two external drainage procedures for a pancreatic cyst done elsewhere. The jaundice was not relieved by either of these two operations. At operation a dilated, thick-walled gallbladder and dilated common bile duct were found. There was a cystic tumor obstructing the common hepatic duct and attached by a narrow pedicle to the site of entry of the right hepatic duct into the common hepatic duct. There were also multiple intrahepatic cysts of various
BENIGN INTRACYSTIC TUMOR LIVER
Figure 4 (Case 9). Artist's representation of the operative findings.
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KENNETH J.
HARDY
Figure 5 (Case 10). Operative cholangiogram showing cystic dilatation of the left hepatic duct.
sizes confined to the anterior aspect of the midportion of the liver. Cholecystectomy, choledochostomy, and excision of the intraductal tumor were performed. Frozen section examination proved this to be a benign cystic papilloma of the bile duct. The cystic area within the liver was excised with diathermy. Obstructive jaundice and epigastric pain recurred eight months after this operation, and physical examination revealed an enlarged, tender liver. Reoperation showed a cystic mass present in the hilar area of the liver about 8.0 cm. in diameter and containing bile under raised pressure. After choledochostomy, the left hepatic duct was found to communicate with the cyst. Within the cyst there was a cystic papillary tumor on a pedicle protruding through the cyst into the common hepatic duct (Fig. 4). A left hepatic lobectomy and excision of the cyst and tumor were performed. A T tube was inserted into the common hepatic duct. The cystic mass was multiloculated and lined with biliary type of epithelium. The patient remains well ten years after operation. CASE 10. Repeated episodes of right upper quadrant pain without jaundice occurred for 25 years after cholecystectomy for cholelithiasis in a 66-year-old woman. Examination revealed tenderness in the epigastrium and under the right costal margin. At operation in 1963, a large gallbladder remnant, a dilated common bile duct, and a fibrous stenosis of the sphincter of Oddi were found. No stones were apparent in the common bile duct. An operative cholangiogram showed a cystic dilatation of the left hepatic duct (Fig. 5). The gallbladder remnant was excised. Choledochostomy and exploration of the hepatic ducts confirmed the cystic dilatation of the left hepatic duct, but no stones were found in the cavity. A transduodenal sphincterotomy was performed. The patient has remained well in the four succeeding years. CASE 11. A 59-year-old diabetic woman seen in 1955 had a long history of biliary tract symptoms, including cholecystectomy for gallstones in 1931. A
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laparotomy in 1953 for recurrent biliary colic disclosed a dilated common bile duct and dilated common hepatic duct, together with an abscess in the left lobe of the liver. Choledochostomy, transduodenal sphincterotomy, and external drainage of the liver abscess were performed. In 1955, a subhepatic abscess was drained, but as an external biliary fistula followed, the patient was referred to the Lahey Clinic. An enormously dilated common hepatic duct was found at operation with a stricture at the junction of the common hepatic and common bile ducts. There were large stones proximal to the stricture. Almost the whole of the left lobe of the liver was replaced by an abscess cavity. An end-to-end repair of the biliary stricture was combined with left hepatic lobectomy (Fig. 6). The postoperative course was complicated by a subhepatic bile collection which was drained. Six months later, obstructive jaundice developed. At reoperation an operative cholangiogram revealed a cystic dilatation of the left hepatic duct stump as well as two large calculi in the distal common duct. The calculi were removed and the cystic left hepatic duct remnant explored but it contained no stones. In 1965, reexploration was undertaken because of recurrent episodes of upper abdominal pain. At operation, the common duct was 4.0 cm. in diameter and contained gelatinous mucoid material. It was opened widely and the gelatinous material removed. The left hepatic duct remnant was hugely dilated and full of this gelatinous matter. A side-to-side choledochojejunostomy with a 3.0 cm. stoma was constructed in order to provide improved biliary drainage. The postoperative course was uneventful, and she was well five months after operation.
Figure 6 (Case 11). Diagrammatic representation of the operative findings (July 1955).
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J.
HARDY
ETIOLOGY The majority of bile duct cysts are present at birth, as many examples are reported occurring in the fetus, the stillborn, and the neonate. The reported high percentage of associated anomalies of the biliary tract (found also in the present study) further suggests a developmental cause involving hypoplasia of the bile duct wall combined with partial or complete obstruction of the distal common duct. The hypoplasia of the duct wall may be an absence of epithelial lining, as was observed on biopsy in the present series. A biliary cyst forms because of pressure against the thin wall, and secondary complete obstruction to biliary flow from debris or gallstones may follow; this leads to discovery of the cyst because of jaundice, pain, or fever. Distal biliary tract obstruction was present in the eight choledochal cysts reported here, hypoplasia and obliteration of the distal duct were discovered in four, fibrosis of the sphincter of Oddi was seen in three, and a web was present in one patient. In contrast to these views of hypoplasia, Yotuyanagi20 suggests an excessive epithelial proliferation as the mechanism for developing biliary duct cysts. This theory propounds excessive epithelialization when the hepatic bud lumen becomes occluded during development, so that the larger lumen left after recanalization leaves a cystic space. There is little information in the literature regarding the etiology of cysts of the hepatic ducts. One patient (Case 9) probably had multiple developmental anomalies of intrahepatic and extrahepatic cysts in which benign tumors arose. The only other similar case in the literature is that of Gerber,7 who resected a solitary biliary cyst of the liver related to an obstructive benign tumor of the associated bile duct. Two patients (Cases 10 and 11) had cystic dilatations ofthe hepatic duct secondary to distal biliary obstruction. Obstruction from fibrosis of the sphincter of Oddi developed in one patient (Case 10), and a biliary stricture occurred in one other (Case 11). Why this cystic dilatation should affect predominantly the left hepatic duct is unknown. Similar cases of hepatic duct dilatation secondary to distal biliary obstruction have been reported by Jackson and MaxwelllO in 1964.
PATHOLOGY While the size of a choledochal cyst is variable, it is usually sharply demarcated and begins distal to the entry of the cystic duct and terminates above the duodenum. Occasionally the cystic duct opens into the choledochal cyst. Choledochal cysts contain altered bile, often infected by gram-negative organisms. Gallstones are found occasionally within the cyst as was observed in two patients of the present group. Spontaneous or traumatic rupture or malignant change within a biliary duct cyst occurs but was not noted in any of our patients.3 - S• 9. 15. 19 Cholangitis and biliary cirrhosis follow long-standing biliary obstruction as seen in Cases 2 and 3, with both patients dying of liver failure.
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CLINICAL FEATURES The classic triad of clinical features of a choledochal cyst is abdominal pain, jaundice, and an abdominal mass. Among our eight patients, this triad was seen in only two, while the remaining six individuals had one or two features of the triad. The pain is usually in the right upper quadrant and can extend anteriorly or posteriorly. Initially, the jaundice is obstructive and intermittent. The mass is in the right upper abdominal quadrant, smooth, discrete, often mobile, and may vary in size from time to time. Although not diagnostic, roentgenographic studies/can locate the position of the cyst. If the patient is not jaundiced, cholecystography may show a comma-shaped gallbladder lying high above the duodenum, as pointed out by Dickson Wright19 in 1935, and the cyst is occasionally outlined by intravenous cholangiography.l1 Percutaneous cholangiography was not attempted in this group but may well be helpful. Barium meal examination may show an anterior displacement of the stomach and a downward and inward displacement of the duodenum (Case 4). Following biliary intestinal anastomosis, gas shadows and fluid levels may be seen on the plain films of the abdomen, and the cysts may be filled by reflux of barium through the stoma of the anastomosis (Case 5).
OPERATIVE DIAGNOSIS The relationship of the cyst to the biliary tract is carefully defined by blunt and sharp dissection. Operative cholangiography is performed before opening the cyst to demonstrate the ducts both proximally and distally. After opening the cyst, it is explored from within by probing the proximal and distal bile ducts and for any accessory bile ducts that may be present. A siInilar approach is used in patients with cystic dilatation of the hepatic ducts, with care being taken to establish the cause of the biliary tract obstruction which has led to cyst formation.
SURGICAL TREATMENT The treatment of a symptomatic choledochal cyst is operative. Untreated patients usually die, as Tsardakas and RobneW 6 indicated in 1956 when they reported the deaths of 29 of 30 untreated patients. Surgical treatment of choledochal cyst is becoming more radical. We condemn aspiration of the cyst because this does not deal with the underlying abnormality, predisposes to biliary peritonitis, and has a high mortality. External drainage alone should not be used, except as a temporary measure in severely ill patients with jaundice and poor liver function who would not tolerate resection. Dilatation of the distal common bile duct alone is not sufficient as this only partly deals with the underlying pathologic changes. The most successful methods of
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J.
HARDY
treatment at present involve resection or some form of intestinal anastomosis. Opinion is divided about the correct mode of treatment. Cystoduodenostomy is a relatively simple procedure and has numerous advocates.B• 12. 14 This anastomosis is especially indicated in infants and children. The operative mortality of this procedure is relatively low, but stricture of the anastomosis or recurrent cholangitis occurs in approximately 30 per cent of patients. It was unsatisfactory in one individual in this series. Biliary duodenal anastomosis rarely is performed at the Lahey Clinic because we believe that stricture formation is far more common than after a biliary jejunal anastomosis. It is emphasized by all of the advocates of cystoduodenostomy that the anastomosis should be placed in the most dependent part of the cyst in order to assure adequate biliary drainage. Cystojejunostomy was introduced by Japanese surgeons and is widely used as a method of draining choledochal cysts.2. 12. 1B It was the mode of treatment we most frequently employed (five of eight patients) and carries a relatively low mortality, although slightly higher than for cystoduodenostomy. The incidence of stricture formation and subsequent cholangitis, however, is much lower than for cystoduodenostomy. Cyst excision and hepatico-intestinal anastomosis were first carried out by McWhorter 13 in 1924 and advocated more recently by Alonso-Lej, Rever, and Pessagno. 1 The operative risk associated with cyst excision is thought to be higher than with cystojejunostomy because of associated biliary and vascular anomalies leading to operative complications, but the late results of excision are more gratifying. Excision of the cyst was carried out in two of our patients without incident or subsequent sequelae. We advocate cyst excision and hepaticojejunostomy for relatively small choledochal cysts which can be dissected free of surrounding structures. When cyst excision is thought to be hazardous, we advise cystojejunostomy. If a cyst is extremely large and the most dependent portion is retroduodenal, a mushroom catheter should be inserted into the most dependent portion of the cyst. This should then be brought out through a stab wound in the abdominal wall after the cystojejunostomy has been performed. The surgical treatment of cystic dilatations of the hepatic ducts is directed primarily to the relief of the distal biliary obstruction rather than to the cyst itself. This means correction of a biliary stricture, relief of fibrotic stenosis of the sphincter of Oddi, and removal of gallstones or tumors of the bile duct, all of which can be the primary cause of the biliary cyst.
REFERENCES 1. Alonso-Lej, F., Rever, W. B., Jr., and Pessagno, D. J.: Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Internat. Abst. Surg. 108:1-30 (Jan.) 1959. 2. Arthur, G. W., and Sterwart, J. 0.: Biliary cysts. Brit. J. Surg. 51:671-675 (Sept.) 1964.
3. Blocker, T. G., Jr., Williams, H., and Williams, J. E.: Traumatic rupture of congenital cyst of choledochus. Arch. Surg. 34:695-701 (April) 1937.
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4. Dexter, D.: Choledochal cyst with carcinoma of the intrahepatic bile ducts and pancreatic ducts. Brit. J. Cancer 11: 18-25 (March) 1957. 5. Douglas, A. H.: Case of dilatation of the common bile duct. Monthly J. Med. Sci. 14: 97-101 (Feb.) 1852. 6. George, P. A., and Maingot, R: Choledochus cyst associated with carcinoma in the liver. Report of a case. Brit. J. Surg. 50:339-341 (Nov.) 1962. 7. Gerber, A.: Retention cyst of liver due to bile duct polyp. Amer. Surg. 140:906-910 (Dec.) 1954. 8. Gross, R E.: Idiopathic dilatation of common bile duct in children; review of literature and reportof2 cases. J. Pediat. 3:730-755 (Nov.) 1933. 9. Irwin, S. T., and Morison, J. E.: Congenital cyst of common bile-duct containing stones and underlying cancerous change. Brit. J. Surg. 32:319-321 (Oct.) 1944. 10. Jackson, F. C., and Maxwell, J. W., Jr.: Intrahepatic diverticulum-common hepatic bile duct. Arch. Surg. 89:706-708 (Oct.) 1964. 11. Keith, L. M., Jr., Rini, J. M., and Martin, L. H.: Congenital cystic dilatation of the common duct (choledochal cyst); report oftwo cases. Arch. Surg. 75: 143-145 (July) 1957. 12. Maingot, R: Congenital abnormalities of the bile ducts. In Smith, R, and Sherlock, S. (Editors): Surgery of the Gall Bladder and Bile Ducts. London, Butterworths, 1964, pp.52-66. 13. McWhorter, G. L.: Congenital cystic dilatation of common bile duct. Arch. Surg.8:604626 (March) 1924. 14. Swenson, 0: Pediatric Surgery. New York, Appleton-Century-Crofts, Inc. 1958, pp. 279-280. 15. Tagart, R E. B.: Perforation of congenital cyst of common bile-duct. Brit. J. Surg. 44: 18-21 (July) 1956. 16. Tsardakas, E. N., and Robnett, A. H.: Congenital cystic dilatation of common bile duct; report of 3 cases, analysis of 57 cases, and review of literature. A.M.A. Arch. Surg. 72:311-327 (Feb.) 1956. 17. Warren, K. W.: Modification of the Roux-en-Yprocedure. SURG. CLIN. N. AMER. 45:611615 (June) 1965. 18. Warren, K. W., and Polk, R C.: Benign cysts of the liver and biliary tract. SURG. CLIN. N. AMER. 38:707-728 (June) 1958. 19. Wright, A. D.: X-ray appearances produced by congenital cystic dilatation of common bile duct. Brit. J. Radiol. 8:227-230 (April) 1935. 20. Yotuyanagi, S.: Contributions to the aetiology and pathology of idiopathic cystic dilatation of the common bile duct with report of three cases: a new aetiological theory. Gann. 30:601-650 (Oct.) 1936.
Because cystic dilatations of the biliary tract are uncommon, their variable clinical and pathologic features present problems in diagnosis and surgical treatment. The incidence of these lesions is unknown because many are not reported, and we think that many remain asymptomatic. An early description of the pathologic anatomy of a choledochal cyst was given by Douglas5 in 1852. Sporadic reports since then enabled Alonso-Lej, Rever, and Pessagno 1 in 1959 to record 419 instances from the literature. The present study is based on 11 patients with biliary duct cysts treated at the Lahey Clinic Foundation. This report does not include those patients in whom biopsy showed a biliary epithelial lining of the intrahepatic cyst that had no demonstrable connection with the bile ducts. There were nine women and two men in the group. Their ages at the time of presentation ranged from 9 months to 65 years; eight of these patients were under 40 years of age. In three patients there was a cystic dilatation of the left hepatic duct, and one of these three also had other intrahepatic cysts. Eight patients in the series had choledochal cysts. The patients were divided into two groups: those with cysts of the left hepatic duct and those with choledochal cysts. A brief case report is given of each patient in order to illustrate the variability of clinical and pathologic features as well as the nature and results of the various types of surgical treatment that were employed.
CHOLEDOCHAL CYSTS CASE 1. In 1964 a woman of 37 years complained of right upper quadrant pain and weight loss of short duration but no jaundice. At operation, a 5 cm. choledochal cyst was found containing gallstones. Multiple stones and biliary debris also were found in the proximal hepatic ducts and gallbladder. The distal common duct was occluded almost completely by a web-like structure, 1.5 cm. proximal to the sphincter of Oddi. Cholecystectomy and a 60 per cent excision of the choledochal cyst were performed; in addition, the distal common bile duct was enlarged by Bakes dilators. The remainder of the cyst was sutured longiSurgical Clinics of North America- Vol. 48, No.3, June, 1968
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KUNE, KENNETH
J.
HARDY
Figure 1 (Case 1). T-tube cholangiogram showing the recurrent choledochal cyst. The distal common duct is occluded, and no contrast medium is seen in the duodenum.
tudinally over a T tube. Because jaundice developed postoperatively and clamping of the T tube caused abdominal pain, the patient was referred to the Lahey Clinic for further treatment. A T tube cholangiogram showed a choledochal cyst, 5 cm. in diameter, without filling of the distal common bile duct (Fig. 1). At operation, a fusiform choledochal cyst was found, and the distal common duct was stenotic. The cyst and distal common duct were excised, and the proximal duct was anastomosed to the jejunum with an entero-anastomosis and occlusion of the proximalllmb of the loop jejunostomy. The anastomosis was splinted with a rubber Y tube.'7 The patient remains well without symptoms two years later. CASE 2. A 33-year-old woman was seen at the Lahey Clinic in 1948 with a history of repeated upper abdominal pain and a past history of three biliary tract procedures that included cholecystectomy for gallstones, dilatation of a fibrotic sphincter of Oddi, and a side-to-side choledochojejunostomy for recurrent fibrosis of the sphincter of Oddi. Clinical and laboratory investigations of the patient gave negative results. At operation, a large choledochal cyst, stones in the common bile duct, stenosis of the sphincter of Oddi, and a papilloma of the ampulla of Vater were found. In addition, the choledochojejunostomy had stenosed completely. The biliaryintestinal anastomosis was dismantled, the stones removed, and a sphincterotomy made after transduodenal excision of the papilloma. Reoperation was necessary in 1954 because of persistence of pain. The sphincter of Oddi again was stenosed and the choledochal cyst again contained stones and debris. In addition, a choledochal cyst-duodenal fistula was present; the liver was enlarged and cirrhotic. The internal fistula was divided, the duodenum was closed, and a side-to-side choledochocystojejunostomy was made. On the second day after operation, shock occurred that was associated with severe abdominal pain, fever, and a serum amylase value of 1,850 Somogyi units. Anuria developed, and the patient died on the fourth postoperative day. Necropsy was not performed.
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CASE 3. A baby of 9 months, deeply jaundiced since birth and with marked hepatosplenomegaly, was operated on in 1943. A large choledochal cyst was found. The cystic artery passed anterior to the cyst, but the cystic duct was not demonstrated. Incomplete descent of the cecum was present also. The cyst was opened, and a probe was passed into each hepatic duct with ease, but the opening of the distal bile duct was not found. External catheter drainage of the cyst was followed by choledochocystojejunostomy two months later when the general condition of the baby permitted the operation. The baby died two years later from liver failure, but in the intervening period the jaundice cleared clinically. CASE 4. A cystic mass was palpable in the right upper quadrant of the abdomen in addition to hepatomegaly in a 3-year-old boy with jaundice. He had suffered fever, pain, and vomiting before admission in 1955. Liver function tests indicated obstructive jaundice. An upper gastrointestinal barium series showed anterior elevation of the distal part of the stomach and displacement of the duodenal loop (Fig. 2). A choledochal cyst, 10 cm. in diameter, was found at operation. The gallbladder was distended, and the liver was enlarged. The cyst was opened and the proximal hepatic duct probed with ease, but the distal opening of the common bile duct was not found. Choledochocystojejunostomy with a diverting enteroanastomosis was performed. Reexploration was carried out 17 days after operation because fever and the clinical signs suggested subhepatic abscess. However, no abscess was found. The cystojejunostomy was patent, but the cyst was distended with bile. It was decompressed by drainage with a large de Pezzer catheter. Cholangiography of the cavity through the de Pezzer catheter five months later revealed the anastomosis to be satisfactory with a decrease in size of the cyst. The catheter was removed. The patient was in excellent health ten years after operation. CASE 5. A 13-year-old girl was seen in 1965 with a four-month history of right-sided upper abdominal pain and vomiting. Four years previously, a choledo-
Figure 2 (Case 4). Barium meal examination before operation. Considerable anterior displacement of the distal portion of the stomach is seen in the lateral view.
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Figure 3 (Case 5). Barium meal examination following choledochal cyst duodenostomy. Barium outlines the anastomosis, cyst, gallbladder, hepatic ducts and part of the pancreatic duct system. Some barium remained in the choledochal cyst for a few hours after this procedure.
chocystoduodenostomy had been performed elsewhere. Barium meal examination demonstrated a patent choledochocystoduodenostomy with barium freely flowing through the anastomosis outlining a choledochal cyst, the gallbladder, the hepatic ducts, and part of the pancreatic duct system, but some of the barium was seen to remain in the choledochal cyst for several hours (Fig. 3). At operation in 1965, a choledochal cyst with a patent choledochocystoduodenostomy was found. The proximal hepatic ducts were normal, but the distal common bile duct was narrowed at its lower end. There was a large gallbladder folded on itself with a dilated cystic duct that opened into the choledochal cyst itself. Most of the cyst was excised, preserving the cystoduodenostomy. The distal common bile duct was dilated with Bakes dilators, and a transduodenal sphincterotomy with a long-limb T tube was combined with cholecystectomy and pyloroplasty. The remainder of the cyst was closed over another long-limb T tube, the vertical limb of which was brought to the surface through a separate incision in the common hepatic duct. In the postoperative period, a biliary fistula developed that healed within a few days. Six months after operation the T tube was removed, and no further symptoms have occurred since the operation. CASE 6. Recurrent attacks of epigastric pain and jaundice had occurred in a woman of 29 years since the age of 7. External drainage of a choledochal cyst when the patient was 17 was followed a year later by choledochocystogastrostomy. This relieved her symptoms for 12 years when she again became jaundiced, requiring cholecystectomy and removal of stones from the choledochal cyst. In 1961 a few months later, reexploration was performed at the Lahey Clinic because of recurrent abdominal pain and jaundice. The cystogastrostomy was almost completely occluded. The cyst was excised, and hepaticojejunostomy was carried out using a Y tube as an internal stent. In 1963, this tube became occluded and was removed. She was well until 1965 when cholangitis developed. At operation, a stricture of the hepaticojejunostomy was found, and a new anastomosis was constructed using a Y tube stent. She was symptomless five months after operation.
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CASE 7. A 38-year-old man in 1953 gave a history of recurrent pain in the right upper quadrant of the abdomen since the age of 6 years. A cholecystectomy and choledocholithotomy had been done in the past with subsequent reoperation for an impacted stone in the lower end of the common bile duct at which time choledochostomy was combined with transduodenal sphincterotomy. Recurrent attacks of jaundice associated with chills and fever followed the second operation. At operation in 1953 a large cystic dilatation was found to involve the common hepatic, left hepatic, and right hepatic ducts. The cystic dilatation of the common bile duct measured 5.0 cm. in diameter. The proximal ducts were probed with ease, but the opening for the distal common bile duct could not be found. A sideto-side choledochojejunostomy was constructed with a stoma of 5.0 cm. together with a proximal entero-anastomosis. The postoperative course was uncomplicated and there have been no further symptoms. CASE 8. Three months before admission in 1957 a 23-year-old woman had a cholecystectomy for gallstones and drainage for a postoperative biliary collection. Jaundice followed this, and the patient lost 30 pounds in weight. At operation at the Lahey Clinic, a large choledochal cyst was found that contained 200 ml. of bile. The proximal hepatic ducts were probed with ease, but the opening of the distal common bile duct was not found. Choledochocystojejunostomy with a proximal entero-anastomosis was performed. Biopsy of the cyst wall was reported as a fibrous-walled cyst with chronic inflammation. The patient remains well ten years later.
CYSTIC DILATATIONS OF THE LEFT HEPATIC DUCT CASE 9. A 27-year-old woman came to the Lahey Clinic in 1946 with jaundice of 15 months' duration, a biliary fistula, and a history of two external drainage procedures for a pancreatic cyst done elsewhere. The jaundice was not relieved by either of these two operations. At operation a dilated, thick-walled gallbladder and dilated common bile duct were found. There was a cystic tumor obstructing the common hepatic duct and attached by a narrow pedicle to the site of entry of the right hepatic duct into the common hepatic duct. There were also multiple intrahepatic cysts of various
BENIGN INTRACYSTIC TUMOR LIVER
Figure 4 (Case 9). Artist's representation of the operative findings.
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Figure 5 (Case 10). Operative cholangiogram showing cystic dilatation of the left hepatic duct.
sizes confined to the anterior aspect of the midportion of the liver. Cholecystectomy, choledochostomy, and excision of the intraductal tumor were performed. Frozen section examination proved this to be a benign cystic papilloma of the bile duct. The cystic area within the liver was excised with diathermy. Obstructive jaundice and epigastric pain recurred eight months after this operation, and physical examination revealed an enlarged, tender liver. Reoperation showed a cystic mass present in the hilar area of the liver about 8.0 cm. in diameter and containing bile under raised pressure. After choledochostomy, the left hepatic duct was found to communicate with the cyst. Within the cyst there was a cystic papillary tumor on a pedicle protruding through the cyst into the common hepatic duct (Fig. 4). A left hepatic lobectomy and excision of the cyst and tumor were performed. A T tube was inserted into the common hepatic duct. The cystic mass was multiloculated and lined with biliary type of epithelium. The patient remains well ten years after operation. CASE 10. Repeated episodes of right upper quadrant pain without jaundice occurred for 25 years after cholecystectomy for cholelithiasis in a 66-year-old woman. Examination revealed tenderness in the epigastrium and under the right costal margin. At operation in 1963, a large gallbladder remnant, a dilated common bile duct, and a fibrous stenosis of the sphincter of Oddi were found. No stones were apparent in the common bile duct. An operative cholangiogram showed a cystic dilatation of the left hepatic duct (Fig. 5). The gallbladder remnant was excised. Choledochostomy and exploration of the hepatic ducts confirmed the cystic dilatation of the left hepatic duct, but no stones were found in the cavity. A transduodenal sphincterotomy was performed. The patient has remained well in the four succeeding years. CASE 11. A 59-year-old diabetic woman seen in 1955 had a long history of biliary tract symptoms, including cholecystectomy for gallstones in 1931. A
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laparotomy in 1953 for recurrent biliary colic disclosed a dilated common bile duct and dilated common hepatic duct, together with an abscess in the left lobe of the liver. Choledochostomy, transduodenal sphincterotomy, and external drainage of the liver abscess were performed. In 1955, a subhepatic abscess was drained, but as an external biliary fistula followed, the patient was referred to the Lahey Clinic. An enormously dilated common hepatic duct was found at operation with a stricture at the junction of the common hepatic and common bile ducts. There were large stones proximal to the stricture. Almost the whole of the left lobe of the liver was replaced by an abscess cavity. An end-to-end repair of the biliary stricture was combined with left hepatic lobectomy (Fig. 6). The postoperative course was complicated by a subhepatic bile collection which was drained. Six months later, obstructive jaundice developed. At reoperation an operative cholangiogram revealed a cystic dilatation of the left hepatic duct stump as well as two large calculi in the distal common duct. The calculi were removed and the cystic left hepatic duct remnant explored but it contained no stones. In 1965, reexploration was undertaken because of recurrent episodes of upper abdominal pain. At operation, the common duct was 4.0 cm. in diameter and contained gelatinous mucoid material. It was opened widely and the gelatinous material removed. The left hepatic duct remnant was hugely dilated and full of this gelatinous matter. A side-to-side choledochojejunostomy with a 3.0 cm. stoma was constructed in order to provide improved biliary drainage. The postoperative course was uneventful, and she was well five months after operation.
Figure 6 (Case 11). Diagrammatic representation of the operative findings (July 1955).
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ETIOLOGY The majority of bile duct cysts are present at birth, as many examples are reported occurring in the fetus, the stillborn, and the neonate. The reported high percentage of associated anomalies of the biliary tract (found also in the present study) further suggests a developmental cause involving hypoplasia of the bile duct wall combined with partial or complete obstruction of the distal common duct. The hypoplasia of the duct wall may be an absence of epithelial lining, as was observed on biopsy in the present series. A biliary cyst forms because of pressure against the thin wall, and secondary complete obstruction to biliary flow from debris or gallstones may follow; this leads to discovery of the cyst because of jaundice, pain, or fever. Distal biliary tract obstruction was present in the eight choledochal cysts reported here, hypoplasia and obliteration of the distal duct were discovered in four, fibrosis of the sphincter of Oddi was seen in three, and a web was present in one patient. In contrast to these views of hypoplasia, Yotuyanagi20 suggests an excessive epithelial proliferation as the mechanism for developing biliary duct cysts. This theory propounds excessive epithelialization when the hepatic bud lumen becomes occluded during development, so that the larger lumen left after recanalization leaves a cystic space. There is little information in the literature regarding the etiology of cysts of the hepatic ducts. One patient (Case 9) probably had multiple developmental anomalies of intrahepatic and extrahepatic cysts in which benign tumors arose. The only other similar case in the literature is that of Gerber,7 who resected a solitary biliary cyst of the liver related to an obstructive benign tumor of the associated bile duct. Two patients (Cases 10 and 11) had cystic dilatations ofthe hepatic duct secondary to distal biliary obstruction. Obstruction from fibrosis of the sphincter of Oddi developed in one patient (Case 10), and a biliary stricture occurred in one other (Case 11). Why this cystic dilatation should affect predominantly the left hepatic duct is unknown. Similar cases of hepatic duct dilatation secondary to distal biliary obstruction have been reported by Jackson and MaxwelllO in 1964.
PATHOLOGY While the size of a choledochal cyst is variable, it is usually sharply demarcated and begins distal to the entry of the cystic duct and terminates above the duodenum. Occasionally the cystic duct opens into the choledochal cyst. Choledochal cysts contain altered bile, often infected by gram-negative organisms. Gallstones are found occasionally within the cyst as was observed in two patients of the present group. Spontaneous or traumatic rupture or malignant change within a biliary duct cyst occurs but was not noted in any of our patients.3 - S• 9. 15. 19 Cholangitis and biliary cirrhosis follow long-standing biliary obstruction as seen in Cases 2 and 3, with both patients dying of liver failure.
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CLINICAL FEATURES The classic triad of clinical features of a choledochal cyst is abdominal pain, jaundice, and an abdominal mass. Among our eight patients, this triad was seen in only two, while the remaining six individuals had one or two features of the triad. The pain is usually in the right upper quadrant and can extend anteriorly or posteriorly. Initially, the jaundice is obstructive and intermittent. The mass is in the right upper abdominal quadrant, smooth, discrete, often mobile, and may vary in size from time to time. Although not diagnostic, roentgenographic studies/can locate the position of the cyst. If the patient is not jaundiced, cholecystography may show a comma-shaped gallbladder lying high above the duodenum, as pointed out by Dickson Wright19 in 1935, and the cyst is occasionally outlined by intravenous cholangiography.l1 Percutaneous cholangiography was not attempted in this group but may well be helpful. Barium meal examination may show an anterior displacement of the stomach and a downward and inward displacement of the duodenum (Case 4). Following biliary intestinal anastomosis, gas shadows and fluid levels may be seen on the plain films of the abdomen, and the cysts may be filled by reflux of barium through the stoma of the anastomosis (Case 5).
OPERATIVE DIAGNOSIS The relationship of the cyst to the biliary tract is carefully defined by blunt and sharp dissection. Operative cholangiography is performed before opening the cyst to demonstrate the ducts both proximally and distally. After opening the cyst, it is explored from within by probing the proximal and distal bile ducts and for any accessory bile ducts that may be present. A siInilar approach is used in patients with cystic dilatation of the hepatic ducts, with care being taken to establish the cause of the biliary tract obstruction which has led to cyst formation.
SURGICAL TREATMENT The treatment of a symptomatic choledochal cyst is operative. Untreated patients usually die, as Tsardakas and RobneW 6 indicated in 1956 when they reported the deaths of 29 of 30 untreated patients. Surgical treatment of choledochal cyst is becoming more radical. We condemn aspiration of the cyst because this does not deal with the underlying abnormality, predisposes to biliary peritonitis, and has a high mortality. External drainage alone should not be used, except as a temporary measure in severely ill patients with jaundice and poor liver function who would not tolerate resection. Dilatation of the distal common bile duct alone is not sufficient as this only partly deals with the underlying pathologic changes. The most successful methods of
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treatment at present involve resection or some form of intestinal anastomosis. Opinion is divided about the correct mode of treatment. Cystoduodenostomy is a relatively simple procedure and has numerous advocates.B• 12. 14 This anastomosis is especially indicated in infants and children. The operative mortality of this procedure is relatively low, but stricture of the anastomosis or recurrent cholangitis occurs in approximately 30 per cent of patients. It was unsatisfactory in one individual in this series. Biliary duodenal anastomosis rarely is performed at the Lahey Clinic because we believe that stricture formation is far more common than after a biliary jejunal anastomosis. It is emphasized by all of the advocates of cystoduodenostomy that the anastomosis should be placed in the most dependent part of the cyst in order to assure adequate biliary drainage. Cystojejunostomy was introduced by Japanese surgeons and is widely used as a method of draining choledochal cysts.2. 12. 1B It was the mode of treatment we most frequently employed (five of eight patients) and carries a relatively low mortality, although slightly higher than for cystoduodenostomy. The incidence of stricture formation and subsequent cholangitis, however, is much lower than for cystoduodenostomy. Cyst excision and hepatico-intestinal anastomosis were first carried out by McWhorter 13 in 1924 and advocated more recently by Alonso-Lej, Rever, and Pessagno. 1 The operative risk associated with cyst excision is thought to be higher than with cystojejunostomy because of associated biliary and vascular anomalies leading to operative complications, but the late results of excision are more gratifying. Excision of the cyst was carried out in two of our patients without incident or subsequent sequelae. We advocate cyst excision and hepaticojejunostomy for relatively small choledochal cysts which can be dissected free of surrounding structures. When cyst excision is thought to be hazardous, we advise cystojejunostomy. If a cyst is extremely large and the most dependent portion is retroduodenal, a mushroom catheter should be inserted into the most dependent portion of the cyst. This should then be brought out through a stab wound in the abdominal wall after the cystojejunostomy has been performed. The surgical treatment of cystic dilatations of the hepatic ducts is directed primarily to the relief of the distal biliary obstruction rather than to the cyst itself. This means correction of a biliary stricture, relief of fibrotic stenosis of the sphincter of Oddi, and removal of gallstones or tumors of the bile duct, all of which can be the primary cause of the biliary cyst.
REFERENCES 1. Alonso-Lej, F., Rever, W. B., Jr., and Pessagno, D. J.: Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. Internat. Abst. Surg. 108:1-30 (Jan.) 1959. 2. Arthur, G. W., and Sterwart, J. 0.: Biliary cysts. Brit. J. Surg. 51:671-675 (Sept.) 1964.
3. Blocker, T. G., Jr., Williams, H., and Williams, J. E.: Traumatic rupture of congenital cyst of choledochus. Arch. Surg. 34:695-701 (April) 1937.
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4. Dexter, D.: Choledochal cyst with carcinoma of the intrahepatic bile ducts and pancreatic ducts. Brit. J. Cancer 11: 18-25 (March) 1957. 5. Douglas, A. H.: Case of dilatation of the common bile duct. Monthly J. Med. Sci. 14: 97-101 (Feb.) 1852. 6. George, P. A., and Maingot, R: Choledochus cyst associated with carcinoma in the liver. Report of a case. Brit. J. Surg. 50:339-341 (Nov.) 1962. 7. Gerber, A.: Retention cyst of liver due to bile duct polyp. Amer. Surg. 140:906-910 (Dec.) 1954. 8. Gross, R E.: Idiopathic dilatation of common bile duct in children; review of literature and reportof2 cases. J. Pediat. 3:730-755 (Nov.) 1933. 9. Irwin, S. T., and Morison, J. E.: Congenital cyst of common bile-duct containing stones and underlying cancerous change. Brit. J. Surg. 32:319-321 (Oct.) 1944. 10. Jackson, F. C., and Maxwell, J. W., Jr.: Intrahepatic diverticulum-common hepatic bile duct. Arch. Surg. 89:706-708 (Oct.) 1964. 11. Keith, L. M., Jr., Rini, J. M., and Martin, L. H.: Congenital cystic dilatation of the common duct (choledochal cyst); report oftwo cases. Arch. Surg. 75: 143-145 (July) 1957. 12. Maingot, R: Congenital abnormalities of the bile ducts. In Smith, R, and Sherlock, S. (Editors): Surgery of the Gall Bladder and Bile Ducts. London, Butterworths, 1964, pp.52-66. 13. McWhorter, G. L.: Congenital cystic dilatation of common bile duct. Arch. Surg.8:604626 (March) 1924. 14. Swenson, 0: Pediatric Surgery. New York, Appleton-Century-Crofts, Inc. 1958, pp. 279-280. 15. Tagart, R E. B.: Perforation of congenital cyst of common bile-duct. Brit. J. Surg. 44: 18-21 (July) 1956. 16. Tsardakas, E. N., and Robnett, A. H.: Congenital cystic dilatation of common bile duct; report of 3 cases, analysis of 57 cases, and review of literature. A.M.A. Arch. Surg. 72:311-327 (Feb.) 1956. 17. Warren, K. W.: Modification of the Roux-en-Yprocedure. SURG. CLIN. N. AMER. 45:611615 (June) 1965. 18. Warren, K. W., and Polk, R C.: Benign cysts of the liver and biliary tract. SURG. CLIN. N. AMER. 38:707-728 (June) 1958. 19. Wright, A. D.: X-ray appearances produced by congenital cystic dilatation of common bile duct. Brit. J. Radiol. 8:227-230 (April) 1935. 20. Yotuyanagi, S.: Contributions to the aetiology and pathology of idiopathic cystic dilatation of the common bile duct with report of three cases: a new aetiological theory. Gann. 30:601-650 (Oct.) 1936.