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ERCP in evaluation and diagnosis of choledochal cyst: report of five cases
ERCP in evaluation and diagnosis of choledochal cyst: report of five cases Bryan S. Thatcher, Michael V. Sivak, Jr., Robert E. Hermann, Caldwell B. Esselstyn, Jr.,
MD MD MD MD
A choledochal cyst is an uncommon biliary tract malformation thought to be congenital in origin. l It is most frequently found in Orientals and females l ,2; the incidence in Caucasians is estimated at less than one in 200,000. 3 The defect most commonly produces symptoms in the young, but over 20% of patients are more than 40 years of age. 4 An anomalous connection of the pancreatic and biliary ductal systems has been suggested as a factor in the pathogenesis of choledochal cyst. 5 We report five cases of choledochal cyst seen at the Cleveland Clinic from 1978 to 1984. Endoscopic retrograde cholangiopancreatography (ERCP) is of value for diagnosis and preoperative evaluation in demonstrating the integrity of both ductal systems, presence or absence of cystolithiasis, and the anomalous pancreaticobiliary connection.
Figure 1. Retrograde injection through minor (accessory) papilla (white arrow at left) which fills the entire pancreatic ductal system. During this injection, the biliary system was also opacified via its anomalous connection with the main pancreatic duct (middle white arrow). The proximal portion of the extrahepatic biliary system is dilated to cystic proportions. Note stricture of distal bile duct (black arrow).
CASE REPORTS Case 1
A 55-year-old Caucasian woman was admitted for evaluation of right upper quadrant (RUQ) pain. Ultrasonography demonstrated cholelithiasis and a dilated biliary system. ERCP revealed a long common channel with an anomalous pancreaticobiliary junction and a large choledochal cyst. Because of morbid obesity, she was instructed to lose weight and remained well for 13 months, when RUQ pain recurred along with nausea, vomiting, and leukocytosis. Laboratory studies included a bilirubin of 4.6 mg/dl (normal to 1.0 mg/ dl) and alkaline phosphatase of 360 U/liter (normal to 40). The patient underwent cholecystectomy and choledochocystoplasty and had no complaints until 11 months later when RUQ pain recurred with associated fever and chills. Results of routine liver function tests were normal except for an alkaline phosphatase of 174 U/liter. ERCP was accomplished by cannulation of the minor papilla. The contrast material flowed retrograde through the accessory pancreatic duct, entered the main pancreatic duct filling it in opposite directions from the point of junction with the accessory duct, and then entered the biliary system via the anomalous pancreaticobiliary junction. In addition to a long common channel, a distal common bile duct (CBD) stricture near its anomalous junction with the pancreatic duct and a large choledochal cyst were demonstrated (Fig. 1). The patient underwent a choledochojejunostomy and had an uneventful postoperative course.
Case 2
A 36-year-old Oriental woman was evaluated because of RUQ pain. There had been no fever or chills. Examination of the abdomen was unremarkable, and routine liver function tests results were normal. She had had a cholecystectomy with common bile duct exploration 7 years earlier for cholelithiasis; she described the pain as similar to the episodic pain experienced before the operation. A dilated common bile duct with a distal stricture had been identified, and stricture dilation and sphincteroplasty were performed. The patient returned 6 months after our initial evaluation with recurrent abdominal pain. The examination was again unremarkable, and liver function test results were normal. ERCP revealed an anomalous pancreaticobiliary junction with a stricture of the distal CBD at the point of junction with the pancreatic duct, and a markedly dilated CBD consistent with choledochal cyst (Fig. 2). Surgery was recommended but deferred by the patient. Seventeen months later, she returned after an episode of abdominal pain, fever, chills, and gram-negative sepsis. Laboratory evaluation revealed an alkaline phosphatase of 213 U/liter (normal to 85 U/liter). At laparotomy, a large choledochal cyst containing sludge was excised and a choledochoduodenostomy was performed. The patient has been followed for 52 months, and has had no complaints except for a rare episode of abdominal pain. Case 3
From the Departments of Gastroenterology and General Surgery, Cleveland Clinic Foundatwn, Cleveland, Ohio. Reprint requests: Michael V. Swak, Jr., MD, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44106. Dr. Thatcher's current affiliation is the Department of Gastroenterology, Riverside Methodist Hospital, Columbus, Ohio. VOLUME 32, NO.1, 1986
A 69-year-old Caucasian woman was admitted for evaluation of RUQ pain, fever, chills, and jaundice. Laboratory evaluation included an alkaline phosphatase of 800 U/liter and total bilirubin of 1.7 mg/dl. The history included a cholecystectomy and choledochocystojejunostomy 2 years 27
demonstrated a long common channel, a midpancreatic duct stricture with proximal dilation, stenosis of the distal CBD, and marked dilation of the extrahepatic biliary system consistent with a choledochal cyst. She underwent excision of the choledochal cyst with Roux-en-Y choledochojejunostomy followed by an uneventful postoperative course. The patient has since been lost to follow-up.
DISCUSSION
Figure 2. Retrograde opacification of choledochal cyst. Note stricture (arrow) of distal bile duct at the point of anomalous connection with the pancreatic duct.
earlier after evaluation of RUQ pain. An ERCP demonstrated a large choledochal cyst with an anomalous connection between the cyst and pancreatic duct (Fig. 3). At surgery, a choledochal cyst containing biliary sludge was excised, and a choledochojejunostomy was performed. The patient has been followed more than 5 years and has had no biliary-related problems.
Choledochal cyst was classified by Alonso-Lej et al. 6 in 1959 into three types. Type I cysts, accounting for the vast majority of cases, are dilations of the extrahepatic biliary system, usually of the common duct. The rare type II lesion is a diverticulum ofthe common bile duct. A type III cyst, also termed a choledochocele, is a dilation of the segment of the common duct within the muscular portion of the duodenal wall that often bulges into the duodenum as a round smooth defect associated with the papilla of Vater. Caroli's disease, a variant of intrahepatic and extrahepatic biliary cystic dilation, is given the classification of type IV by several investigators. 7- 9 Solitary hepatic cysts have been reported in several patients and classified as type V cysts. l The pathogenesis of choledochal cyst is unclear, although various theories have been proposed. l Babbitt5 suggested an abnormal junction of the pancreatic and common ducts as the primary anomaly. This variant is said to be present when the length of the common channel outside the duodenal wall is greater than 0.6 cm. 9 According to this theory, the
Case 4
A 12-year-old girl was evaluated by her physician for episodic RUQ pain. There was no history of jaundice, and the abdominal examination and laboratory evaluation were unremarkable. Oral cholecystography failed twice to visualize the gallbladder, which was shown to be enlarged on ultrasonography. A cholecystectomy was peformed, and a choledochal cyst was identified and reportedly excised. A Ttube was left in place, and the patient was referred for further evaluation at which time she was asymptomtic. Four weeks later, the T-tube was removed. Initially, she remained well but returned 20 months later with RUQ pain, nausea, and vomiting. Routine liver function test results were normal. An ERCP demonstrated a choledochal cyst with a distal bile duct stricture and an anomalous pancreaticobiliary junction (Fig. 4, A and B). Laparotomy with cyst excision and Roux-en-Y hepaticojejunostomy was performed. The patient remained well during 17 months of follow-up. CaseS
A 20-year-old Caucasian woman underwent cholecystectomy after evaluation of RUQ pain. At surgery, a "cystic" lesion was identified, and the patient was referred for further evaluation. Results of laboratory studies were normal. ERCP 28
Figure 3. Retrograde cholangiogram demonstrates large choledochal cyst containing sludge. The previous choledochal cyst jejunostomy (short arrow) drains poorly. The anomalous connection between the biliary and pancreatic ducts (long arrow) occurs about 1.5 to 2.0 cm from the oriface at the main papilla.
GASTROINTESTINAL ENDOSCOPY
Figure 4. A, Retrograde cholangiogram which demonstrates choledochal cyst with anomalous connection between bile duct and pancreatic duct (arrow). B, Close-up view of the anomalous junction of the biliary and pancreatic ducts. Note relative narrowing of the distal portion of the common duct (arrow).
length of this channel and the absence of a functional distal bile duct sphincter promote reflux of pancreatic juice into the biliary system. This results in biliary inflammation and stenosis. The degree of biliary stenosis above the pancreaticobiliary junction is thought to be critical, and it has been suggested that high grade stenosis leads to cystic dilation of the extrahepatic bile ducts, whereas low grade stenosis results in cylindrical dilation. 2 In two reports by different groups of VOLUME 32, NO.1, 1986
Japanese investigators, 53 of 61 patients had this anomalous connection. 2 ,9 All five of our patients had this anomaly. The pancreatic duct may be abnormal as well. Five of six patients had abnormal pancreatograms in one series of nine patients, and, as in our case 5, the most common finding was a markedly dilated proximal pancreatic duct. lO The classic clinical triad for choledochal cyst of abdominal pain, RUQ mass, and jaundice is useful but is found in only about 40% of patients. 4 A high index of suspicion is required in those with RUQ pain with or without a palpable mass or jaundice. Although choledochal cyst is more common in Japanese and other Orientals, and therefore more readily suspected in these individuals, all but one of our patients were Caucasians. Since the advent of biliary imaging procedures, the number of reported cases has increased, and more than 80% of these cases are now diagnosed preoperativelyY Some investigators have advocated ultrasonography, computed tomography (CT), and radionuclide scanning as useful in establishing the diagnosis/ 2- 17 but these tests do not adequately define the anatomic relationships or delineate the anomalous pancreaticobiliary junction. Percutaneous transhepatic cholangiography has been useful in a few patients,9,18 as has ERCP. 2 ,3,9 The latter was especially helpful in our patients for evaluation of RUQ abdominal pain, delineation of the particular anatomic features, demonstration of cystolithiasis, and preoperative planning. ERCP demonstrated the anomalous anatomic features in all five of our patients. In addition, four of the five also had a stricture of the bile duct at its point of connection with the pancreatic duct. A long common channel was shown connecting the junction of the common bile and pancreatic ducts with the duodenum. Although this ductal segment is referred to as a common channel, the exact nature of the stricture is uncertain. Ordinarily, a common channel for bile and pancreatic juice is formed by the early loss of the septum between the bile and pancreatic ducts as they approach the ampulla of Vater. Occasionally, in normal individuals, both duct systems simultaneously opacify upon cannulation and retrograde injection of contrast medium, although the common channel length should not exceed 0.6 cm. In previous reports 9,18 and in our five cases, the distal CBD entered the duct of Wirsung at a right angle several centimeters from the ampulla of Vater, suggesting that the common ductal segment is actually pancreatic duct. This anomalous anatomic configuration of the pancreaticobiliary ductal system has certain technical implications in regard to management. For example, endoscopic sphincterotomy is probably contraindicated in most cases, and endoscopic access to the biliary system for removal of stones or sludge is therefore prohibited. At operation, an attempt may be made 29
to dilate a distal bile duct stricture, as was present in four of our five patients. This may lead to trauma to the pancreas if the relationship of the bile duct to the pancreatic duct is not appreciated. Although reports are somewhat anecdotal, a variety of biliary abnormalities may be associated with an increased risk of malignancy, including congenital hepatic fibrosis,19 biliary atresia,20 calcified gallbladder,21 and chronic cholangitis. 22 Common factors appear to be prolonged biliary stasis and infection. 23 There is an increased incidence of carcinoma associated with choledochal cyst. This is estimated at 2% to 5% versus 0.012% to 0.40% expected in persons without choledochal cyst. 24 Flanigan 25 found adenocarcinoma to be the most common malignant cell type and the posterior cyst wall to be the most frequent tumor site. Of those cases of cystolithiasis associated with choledochal cyst, approximately one third had biliary carcinoma. None of our patients had developed carcinoma, although the cyst was excised in four, and the longest follow-up in any case was 6 years. Medical therapy is ineffective. Tsardakas and Robnete 6 nearly 30 years ago stated that 29 of 30 patients treated medically died from their disease. Attar and Obeid27 reported a 97% mortality for patients with choledochal cysts treated medically. Death was due to cyst rupture with secondary peritonitis, cholangitis, or liver cirrhosis with secondary complications. Because of the poor results and the increased risk of malignancy, surgery is the recommended therapy, although there is controversy concerning the best surgical procedure. 9 Cyst excision with a drainage procedure or internal drainage alone are the two primary surgical options, although excision of type I cyst is currently recommended if technically feasible. 11 • 28 Drainage of a choledochocele (type III cyst) byendoscopic sphincterotomy has been accomplished in three series of patients, but further evaluation of this approach is required. 29-31 Regression of biliary cirrhosis has been reported following choledochal cyst drainage. 32 Although choledochal cyst is an uncommon lesion and less than half the patients present with the classic triad of symptoms, it is being recognized with increasing frequency. ERCP is of significant value in establishing the diagnosis, defining anatomic and pathologic relationships such as the presence of a bile duct stricture, and identifying the anomalous pancreaticobiliary junction. ERCP should, therefore, be performed early in the evaluation as the primary procedure of choice in patients with suspected choledochal cyst.
REFERENCES 1. Powell CS, Sawyers JL, Reynolds VH. Management of adult choledochal cysts. Ann Surg 1981;193:666-76. 30
2. Todani T, Watanabe Y, Fujii T, Demura S. Anomalous arrangement of the pancreaticobiliary ductal system in patients with a choledochal cyst. Am J Surg 1984;147:672-6. 3. Deeg HJ, Rominger JM, Shah AN. Choledochal cyst and pancreatic carcinoma demonstrated simultaneously by endoscopic retrograde cholangiopancreatography. South Med J 1980; 73:1678-9. 4. Flanigan DP. Biliary cysts. Ann Surg 1975;182:635-43. 5. Babbitt DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb. Ann RadioI1969;12:231-40. 6. Alonso-Lej F, Rever WB, Pessagno DJ. Collective review: congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Surg Gynecol Obstet 1959;108:1-30. 7. Arthur GW, Stewart JOR. Biliary cysts. Br J Surg 1964;51:6715.
8. Engle J, Salmon PA. Multiple choledochal cysts. Report of a case. Arch Surg 1964;88:345-9. 9. Ono J, Sakoda K, Akita H. Surgical aspect of cystic dilatation of the bile duct-an anomalous junction of the pancreaticobiliary tract in adults. Ann Surg 1982;195:203-8. 10. Rattner DW, Schapiro RH, Warshaw AL. Abnormalities of the pancreatic and biliary ducts in adult patients with choledochal cysts. Arch Surg 1983;118:1068-73. 11. Shiloni E, Lebensart P, Durst AL, Freund D, Rivkind A. Type I choledochal cyst in adults. Isr J Med Sci 1983;19:209-11. 12. Klein GM, Frost SS. Newer imaging modalities for the preoperative diagnosis of choledochal cyst. Am J Gastroenterol 1981;76:148-52. 13. Paramsothy M, Somasundram K. Technetium 99m-diethylIDA hepatobiliary scintigraphy in the pre-operative diagnosis of choledochal cyst. Br J Radiol 1981;54:1104-7. 14. Han BK, Babcock DS, Gelfand MH. Choledochal cyst with bile duct dilatation: sonography and 99mTc IDA cholescintigraphy. AJR 1981;136:1075-9. 15. Huang MJ, Liaw YF. Intravenous cholescintigraphy using Tc99m-labeled agents in the diagnosis of choledochal cyst. J Nucl Med 1982;23:113-6. 16. Atkinson GO, Gay BB Jr. Choledochal cysts in children: radiologic features. South Med J 1982;75:1215-21. 17. Araki T, Itai Y, Tasaka A. CT of choledochal cyst. AJR 1980;135:792-34. 18. Efremidis SC, Lehr-Janus C, Yeh H-C, Arthur KS, Aufses A Jr. Choledochal cyst: case report and review of the literature. Mt Sinai J Med 1980;47:45-8. 19. Scott J, Shousha S, Thomas HC, Sherlock S. Bile duct carcinoma: a late complication of congenital hepatic fibrosis. Am J Gastroenterol 1980;73:113-9. 20. Kulkarni PB, Beatty EC Jr. Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia. Am J Dis Child 1977;131:442-4. 21. Polk HC Jr. Carcinoma and the calcified gallbladder. Gastroenterology 1966;50:582-5. 22. Falchuk KR, Lesser PB, Galdabini JJ, Isselbacher KJ. Cholangiocarcinoma as related to chronic intrahepatic cholangitis and hepatolithiasis. Am J Gastroenterol 1976;66:57-61. 23. Voyles CR, Smadja C, Shands C, Blumgart LH. Carcinoma in choledochal cysts-age-related incidence. Arch Surg 1983; 118:986-8. 24. Bloustein PA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 1977;67:40-6. 25. Flanigan DP. Biliary carcinoma associated with biliary cysts. Cancer 1977;40:880-3. 26. Tsardakas E, Robnett AH. Congenital cystic dilatation of the common bile duct. Arch Surg 1956;72:311-22. 27. Attar S, Obeid S. Congential cyst of the common bile duct-a review of the literature and a report of two cases. Ann Surg 1955;142:289-95. 28. Lilly JR. The surgical treatment of choledochal cyst. Surg Gynecol Obstet 1979;149:36-42. 29. Zimmon DS, Falkenstein DB, Manno BV, Clemett AR. Choledochocele: radiologic diagnosis and endoscopic management. Gastrointest Radiol 1978;3:349-51. 30. Siegel JH, Harding GT, Chateau F. Endoscopic incision of
GASTROINTESTINAL ENDOSCOPY
choledochal cysts (choledochocele). Endoscopy 1981; 13:200-2. 31. Venu RP, Geenen JE, Hogan WJ, et al. Role of endoscopic retrograde cholangiopancreatography in the diagnosis and
treatment of choledochocele. Gastroenterology 1984;87:1144-9. 32. Yeong ML, Nicholson GI, Lee SP. Regression of biliary cirrhosis following choledochal cyst drainage. Gastroenterology 1982;82:332-5.
The evolution of the double pylorus
At the third gastroscopy 1 month later a short slit was found at the juxtapyloric site. It was characterized by regular margins and clear liquid and air bubbles passed through it during peristalsis; there was no evidence of ulcer lesions. The pylorus appeared slightly rigid (Fig. 2). The patient did not relate any symptoms of peptic ulcer until March 23, 1983, when he noted postprandial epigastric pain. The fourth gastroscopy 10 months later showed a prepyloric ulcer 1.5 em in diameter, and its margins showed a high degree of edema and congestion. The pylorus appeared undamaged (Fig. 3) 1 month later. At the fifth gastroscopy the lesion appeared unchanged, but at the last (sixth) gastroscopy, the ulcer site had scarred over, while an antroduodenal passage was formed by two round orifices of about the same size, separated by a transverse band. The pylorus appeared patent, regular in shape, and detectable from the newly formed orifice by the presence of peristaltic opening and closing movements (Figs. 4 to 7). Histological examinations performed during these endoscopies evidenced chronic gastritis with atrophy, intestinal metaplasia, and mild dysplasia. A radiologic examination (June 15, 1983) showed a deformed pyloric channel and an antropyloric fistula parallel to it. At this time the patient is asymptomatic.
Silvano Fayenz, MD
The double pylorus is an acquired abnormality marked by the presence of two orifices (the natural pyloric opening and an ulcer-induced one) separated by a band, arising at the margin of the pylorus itself. The first case of double pylorus studied endoscopically was described in 1969. 1 Various anatomical types of gastroduodenal fistula may arise. Gould2 described two types of spontaneous gastroduodenal fistula: the first type originates from the lesser gastric curvature and communicates with the first part of the duodenum; the second type originates from the posterior stomach wall and communicates with the third or fourth part of the duodenum. Drapkin et al. 3 outlined a third type of gastroduodenal fistula connecting pylorus and duodenum, thus forming a pyloric septum. Several forms of this third fistulous type can be found owing to different perforative paths taken by the prepyloric ulcer. 4- 21 Rodhe et al. 22 observed with periodic endoscopic examinations a patient with prepyloric ulcer who, after 11 months, evidenced a gastroduodenal channel paralleling the pylorus. Hegedus et aLB proved with subsequent radiologic examinations the progressive development of a second pyloric channel arising from the penetration of a prepyloric ulcer into the duodenal bulb. We describe a case in which the penetration of a prepyloric ulcer gave origin to the formation of a true double pylorus. We are unaware of other reports including a complete documentation of the sequence of endoscopic findings. CASE REPORT
A 58-year-old man, suffering from alcoholic cirrhosis with ascites, was hospitalized on May 6, 1982, on account of a radiologically diagnosed prepyloric gastric ulcer. The first gastroscopy showed a prepyloric peptic ulcer 1.5 em in diameter. The pylorus was only marginally involved by the ulcerative process (Fig. 1). During the second gastroscopy 5 days later the prepyloric ulcer appeared very small in size. On the border facing the pylorus, a tiny slit was found and from this slit a leakage of clear liquid could be observed during peristalsis. The pylorus was slightly inflamed. From the Service of Digestive Endoscopy, Presidio Ospedaliero di Gorizia, Italy. Reprint requests: Silvano Fayenz, MD, Service of Digestive Endoscopy, Presidio Ospedaliero di Gorizia, Via Vittorio Veneto 171, 34170 Gorizia, Italy. VOLUME 32, NO. 1,1986
DISCUSSION
In our case an unusual sequence of events was observed. A prepyloric peptic ulcer penetrated the gastric wall until it reached and slit the duodenal bulb wall. The ulcer healed, but a thin prepyloric slit was left giving rise to a gastroduodenal fistula. An ulcer recurrence at the fistula site took place about 10 months later and, because of its intensive destructive action, caused the widening of this path. This process produced a pillar made up of peripyloric tissue and, in effect, a double pylorus. Both peristaltic function and a~tivity of the pyloric orifice were maintained, indicating the presence of muscle tissue in the dividing band. Although the double pylorus has been considered congenital in origin,23,24 the evidence is overwhelmingly in favor of it being acquired through the mechanism documented in our case. Except for a few cases, the double pylorus has been evidenced in the elderly, almost always in patients over 50 years old. In a patient who did not undergo surgery, Rozelle et al. 25 observed the spontaneous disappearance of the septum, resulting in a single enlarged pyloric channel. 31
MD MD MD MD
A choledochal cyst is an uncommon biliary tract malformation thought to be congenital in origin. l It is most frequently found in Orientals and females l ,2; the incidence in Caucasians is estimated at less than one in 200,000. 3 The defect most commonly produces symptoms in the young, but over 20% of patients are more than 40 years of age. 4 An anomalous connection of the pancreatic and biliary ductal systems has been suggested as a factor in the pathogenesis of choledochal cyst. 5 We report five cases of choledochal cyst seen at the Cleveland Clinic from 1978 to 1984. Endoscopic retrograde cholangiopancreatography (ERCP) is of value for diagnosis and preoperative evaluation in demonstrating the integrity of both ductal systems, presence or absence of cystolithiasis, and the anomalous pancreaticobiliary connection.
Figure 1. Retrograde injection through minor (accessory) papilla (white arrow at left) which fills the entire pancreatic ductal system. During this injection, the biliary system was also opacified via its anomalous connection with the main pancreatic duct (middle white arrow). The proximal portion of the extrahepatic biliary system is dilated to cystic proportions. Note stricture of distal bile duct (black arrow).
CASE REPORTS Case 1
A 55-year-old Caucasian woman was admitted for evaluation of right upper quadrant (RUQ) pain. Ultrasonography demonstrated cholelithiasis and a dilated biliary system. ERCP revealed a long common channel with an anomalous pancreaticobiliary junction and a large choledochal cyst. Because of morbid obesity, she was instructed to lose weight and remained well for 13 months, when RUQ pain recurred along with nausea, vomiting, and leukocytosis. Laboratory studies included a bilirubin of 4.6 mg/dl (normal to 1.0 mg/ dl) and alkaline phosphatase of 360 U/liter (normal to 40). The patient underwent cholecystectomy and choledochocystoplasty and had no complaints until 11 months later when RUQ pain recurred with associated fever and chills. Results of routine liver function tests were normal except for an alkaline phosphatase of 174 U/liter. ERCP was accomplished by cannulation of the minor papilla. The contrast material flowed retrograde through the accessory pancreatic duct, entered the main pancreatic duct filling it in opposite directions from the point of junction with the accessory duct, and then entered the biliary system via the anomalous pancreaticobiliary junction. In addition to a long common channel, a distal common bile duct (CBD) stricture near its anomalous junction with the pancreatic duct and a large choledochal cyst were demonstrated (Fig. 1). The patient underwent a choledochojejunostomy and had an uneventful postoperative course.
Case 2
A 36-year-old Oriental woman was evaluated because of RUQ pain. There had been no fever or chills. Examination of the abdomen was unremarkable, and routine liver function tests results were normal. She had had a cholecystectomy with common bile duct exploration 7 years earlier for cholelithiasis; she described the pain as similar to the episodic pain experienced before the operation. A dilated common bile duct with a distal stricture had been identified, and stricture dilation and sphincteroplasty were performed. The patient returned 6 months after our initial evaluation with recurrent abdominal pain. The examination was again unremarkable, and liver function test results were normal. ERCP revealed an anomalous pancreaticobiliary junction with a stricture of the distal CBD at the point of junction with the pancreatic duct, and a markedly dilated CBD consistent with choledochal cyst (Fig. 2). Surgery was recommended but deferred by the patient. Seventeen months later, she returned after an episode of abdominal pain, fever, chills, and gram-negative sepsis. Laboratory evaluation revealed an alkaline phosphatase of 213 U/liter (normal to 85 U/liter). At laparotomy, a large choledochal cyst containing sludge was excised and a choledochoduodenostomy was performed. The patient has been followed for 52 months, and has had no complaints except for a rare episode of abdominal pain. Case 3
From the Departments of Gastroenterology and General Surgery, Cleveland Clinic Foundatwn, Cleveland, Ohio. Reprint requests: Michael V. Swak, Jr., MD, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44106. Dr. Thatcher's current affiliation is the Department of Gastroenterology, Riverside Methodist Hospital, Columbus, Ohio. VOLUME 32, NO.1, 1986
A 69-year-old Caucasian woman was admitted for evaluation of RUQ pain, fever, chills, and jaundice. Laboratory evaluation included an alkaline phosphatase of 800 U/liter and total bilirubin of 1.7 mg/dl. The history included a cholecystectomy and choledochocystojejunostomy 2 years 27
demonstrated a long common channel, a midpancreatic duct stricture with proximal dilation, stenosis of the distal CBD, and marked dilation of the extrahepatic biliary system consistent with a choledochal cyst. She underwent excision of the choledochal cyst with Roux-en-Y choledochojejunostomy followed by an uneventful postoperative course. The patient has since been lost to follow-up.
DISCUSSION
Figure 2. Retrograde opacification of choledochal cyst. Note stricture (arrow) of distal bile duct at the point of anomalous connection with the pancreatic duct.
earlier after evaluation of RUQ pain. An ERCP demonstrated a large choledochal cyst with an anomalous connection between the cyst and pancreatic duct (Fig. 3). At surgery, a choledochal cyst containing biliary sludge was excised, and a choledochojejunostomy was performed. The patient has been followed more than 5 years and has had no biliary-related problems.
Choledochal cyst was classified by Alonso-Lej et al. 6 in 1959 into three types. Type I cysts, accounting for the vast majority of cases, are dilations of the extrahepatic biliary system, usually of the common duct. The rare type II lesion is a diverticulum ofthe common bile duct. A type III cyst, also termed a choledochocele, is a dilation of the segment of the common duct within the muscular portion of the duodenal wall that often bulges into the duodenum as a round smooth defect associated with the papilla of Vater. Caroli's disease, a variant of intrahepatic and extrahepatic biliary cystic dilation, is given the classification of type IV by several investigators. 7- 9 Solitary hepatic cysts have been reported in several patients and classified as type V cysts. l The pathogenesis of choledochal cyst is unclear, although various theories have been proposed. l Babbitt5 suggested an abnormal junction of the pancreatic and common ducts as the primary anomaly. This variant is said to be present when the length of the common channel outside the duodenal wall is greater than 0.6 cm. 9 According to this theory, the
Case 4
A 12-year-old girl was evaluated by her physician for episodic RUQ pain. There was no history of jaundice, and the abdominal examination and laboratory evaluation were unremarkable. Oral cholecystography failed twice to visualize the gallbladder, which was shown to be enlarged on ultrasonography. A cholecystectomy was peformed, and a choledochal cyst was identified and reportedly excised. A Ttube was left in place, and the patient was referred for further evaluation at which time she was asymptomtic. Four weeks later, the T-tube was removed. Initially, she remained well but returned 20 months later with RUQ pain, nausea, and vomiting. Routine liver function test results were normal. An ERCP demonstrated a choledochal cyst with a distal bile duct stricture and an anomalous pancreaticobiliary junction (Fig. 4, A and B). Laparotomy with cyst excision and Roux-en-Y hepaticojejunostomy was performed. The patient remained well during 17 months of follow-up. CaseS
A 20-year-old Caucasian woman underwent cholecystectomy after evaluation of RUQ pain. At surgery, a "cystic" lesion was identified, and the patient was referred for further evaluation. Results of laboratory studies were normal. ERCP 28
Figure 3. Retrograde cholangiogram demonstrates large choledochal cyst containing sludge. The previous choledochal cyst jejunostomy (short arrow) drains poorly. The anomalous connection between the biliary and pancreatic ducts (long arrow) occurs about 1.5 to 2.0 cm from the oriface at the main papilla.
GASTROINTESTINAL ENDOSCOPY
Figure 4. A, Retrograde cholangiogram which demonstrates choledochal cyst with anomalous connection between bile duct and pancreatic duct (arrow). B, Close-up view of the anomalous junction of the biliary and pancreatic ducts. Note relative narrowing of the distal portion of the common duct (arrow).
length of this channel and the absence of a functional distal bile duct sphincter promote reflux of pancreatic juice into the biliary system. This results in biliary inflammation and stenosis. The degree of biliary stenosis above the pancreaticobiliary junction is thought to be critical, and it has been suggested that high grade stenosis leads to cystic dilation of the extrahepatic bile ducts, whereas low grade stenosis results in cylindrical dilation. 2 In two reports by different groups of VOLUME 32, NO.1, 1986
Japanese investigators, 53 of 61 patients had this anomalous connection. 2 ,9 All five of our patients had this anomaly. The pancreatic duct may be abnormal as well. Five of six patients had abnormal pancreatograms in one series of nine patients, and, as in our case 5, the most common finding was a markedly dilated proximal pancreatic duct. lO The classic clinical triad for choledochal cyst of abdominal pain, RUQ mass, and jaundice is useful but is found in only about 40% of patients. 4 A high index of suspicion is required in those with RUQ pain with or without a palpable mass or jaundice. Although choledochal cyst is more common in Japanese and other Orientals, and therefore more readily suspected in these individuals, all but one of our patients were Caucasians. Since the advent of biliary imaging procedures, the number of reported cases has increased, and more than 80% of these cases are now diagnosed preoperativelyY Some investigators have advocated ultrasonography, computed tomography (CT), and radionuclide scanning as useful in establishing the diagnosis/ 2- 17 but these tests do not adequately define the anatomic relationships or delineate the anomalous pancreaticobiliary junction. Percutaneous transhepatic cholangiography has been useful in a few patients,9,18 as has ERCP. 2 ,3,9 The latter was especially helpful in our patients for evaluation of RUQ abdominal pain, delineation of the particular anatomic features, demonstration of cystolithiasis, and preoperative planning. ERCP demonstrated the anomalous anatomic features in all five of our patients. In addition, four of the five also had a stricture of the bile duct at its point of connection with the pancreatic duct. A long common channel was shown connecting the junction of the common bile and pancreatic ducts with the duodenum. Although this ductal segment is referred to as a common channel, the exact nature of the stricture is uncertain. Ordinarily, a common channel for bile and pancreatic juice is formed by the early loss of the septum between the bile and pancreatic ducts as they approach the ampulla of Vater. Occasionally, in normal individuals, both duct systems simultaneously opacify upon cannulation and retrograde injection of contrast medium, although the common channel length should not exceed 0.6 cm. In previous reports 9,18 and in our five cases, the distal CBD entered the duct of Wirsung at a right angle several centimeters from the ampulla of Vater, suggesting that the common ductal segment is actually pancreatic duct. This anomalous anatomic configuration of the pancreaticobiliary ductal system has certain technical implications in regard to management. For example, endoscopic sphincterotomy is probably contraindicated in most cases, and endoscopic access to the biliary system for removal of stones or sludge is therefore prohibited. At operation, an attempt may be made 29
to dilate a distal bile duct stricture, as was present in four of our five patients. This may lead to trauma to the pancreas if the relationship of the bile duct to the pancreatic duct is not appreciated. Although reports are somewhat anecdotal, a variety of biliary abnormalities may be associated with an increased risk of malignancy, including congenital hepatic fibrosis,19 biliary atresia,20 calcified gallbladder,21 and chronic cholangitis. 22 Common factors appear to be prolonged biliary stasis and infection. 23 There is an increased incidence of carcinoma associated with choledochal cyst. This is estimated at 2% to 5% versus 0.012% to 0.40% expected in persons without choledochal cyst. 24 Flanigan 25 found adenocarcinoma to be the most common malignant cell type and the posterior cyst wall to be the most frequent tumor site. Of those cases of cystolithiasis associated with choledochal cyst, approximately one third had biliary carcinoma. None of our patients had developed carcinoma, although the cyst was excised in four, and the longest follow-up in any case was 6 years. Medical therapy is ineffective. Tsardakas and Robnete 6 nearly 30 years ago stated that 29 of 30 patients treated medically died from their disease. Attar and Obeid27 reported a 97% mortality for patients with choledochal cysts treated medically. Death was due to cyst rupture with secondary peritonitis, cholangitis, or liver cirrhosis with secondary complications. Because of the poor results and the increased risk of malignancy, surgery is the recommended therapy, although there is controversy concerning the best surgical procedure. 9 Cyst excision with a drainage procedure or internal drainage alone are the two primary surgical options, although excision of type I cyst is currently recommended if technically feasible. 11 • 28 Drainage of a choledochocele (type III cyst) byendoscopic sphincterotomy has been accomplished in three series of patients, but further evaluation of this approach is required. 29-31 Regression of biliary cirrhosis has been reported following choledochal cyst drainage. 32 Although choledochal cyst is an uncommon lesion and less than half the patients present with the classic triad of symptoms, it is being recognized with increasing frequency. ERCP is of significant value in establishing the diagnosis, defining anatomic and pathologic relationships such as the presence of a bile duct stricture, and identifying the anomalous pancreaticobiliary junction. ERCP should, therefore, be performed early in the evaluation as the primary procedure of choice in patients with suspected choledochal cyst.
REFERENCES 1. Powell CS, Sawyers JL, Reynolds VH. Management of adult choledochal cysts. Ann Surg 1981;193:666-76. 30
2. Todani T, Watanabe Y, Fujii T, Demura S. Anomalous arrangement of the pancreaticobiliary ductal system in patients with a choledochal cyst. Am J Surg 1984;147:672-6. 3. Deeg HJ, Rominger JM, Shah AN. Choledochal cyst and pancreatic carcinoma demonstrated simultaneously by endoscopic retrograde cholangiopancreatography. South Med J 1980; 73:1678-9. 4. Flanigan DP. Biliary cysts. Ann Surg 1975;182:635-43. 5. Babbitt DP. Congenital choledochal cysts: new etiological concept based on anomalous relationships of the common bile duct and pancreatic bulb. Ann RadioI1969;12:231-40. 6. Alonso-Lej F, Rever WB, Pessagno DJ. Collective review: congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Surg Gynecol Obstet 1959;108:1-30. 7. Arthur GW, Stewart JOR. Biliary cysts. Br J Surg 1964;51:6715.
8. Engle J, Salmon PA. Multiple choledochal cysts. Report of a case. Arch Surg 1964;88:345-9. 9. Ono J, Sakoda K, Akita H. Surgical aspect of cystic dilatation of the bile duct-an anomalous junction of the pancreaticobiliary tract in adults. Ann Surg 1982;195:203-8. 10. Rattner DW, Schapiro RH, Warshaw AL. Abnormalities of the pancreatic and biliary ducts in adult patients with choledochal cysts. Arch Surg 1983;118:1068-73. 11. Shiloni E, Lebensart P, Durst AL, Freund D, Rivkind A. Type I choledochal cyst in adults. Isr J Med Sci 1983;19:209-11. 12. Klein GM, Frost SS. Newer imaging modalities for the preoperative diagnosis of choledochal cyst. Am J Gastroenterol 1981;76:148-52. 13. Paramsothy M, Somasundram K. Technetium 99m-diethylIDA hepatobiliary scintigraphy in the pre-operative diagnosis of choledochal cyst. Br J Radiol 1981;54:1104-7. 14. Han BK, Babcock DS, Gelfand MH. Choledochal cyst with bile duct dilatation: sonography and 99mTc IDA cholescintigraphy. AJR 1981;136:1075-9. 15. Huang MJ, Liaw YF. Intravenous cholescintigraphy using Tc99m-labeled agents in the diagnosis of choledochal cyst. J Nucl Med 1982;23:113-6. 16. Atkinson GO, Gay BB Jr. Choledochal cysts in children: radiologic features. South Med J 1982;75:1215-21. 17. Araki T, Itai Y, Tasaka A. CT of choledochal cyst. AJR 1980;135:792-34. 18. Efremidis SC, Lehr-Janus C, Yeh H-C, Arthur KS, Aufses A Jr. Choledochal cyst: case report and review of the literature. Mt Sinai J Med 1980;47:45-8. 19. Scott J, Shousha S, Thomas HC, Sherlock S. Bile duct carcinoma: a late complication of congenital hepatic fibrosis. Am J Gastroenterol 1980;73:113-9. 20. Kulkarni PB, Beatty EC Jr. Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia. Am J Dis Child 1977;131:442-4. 21. Polk HC Jr. Carcinoma and the calcified gallbladder. Gastroenterology 1966;50:582-5. 22. Falchuk KR, Lesser PB, Galdabini JJ, Isselbacher KJ. Cholangiocarcinoma as related to chronic intrahepatic cholangitis and hepatolithiasis. Am J Gastroenterol 1976;66:57-61. 23. Voyles CR, Smadja C, Shands C, Blumgart LH. Carcinoma in choledochal cysts-age-related incidence. Arch Surg 1983; 118:986-8. 24. Bloustein PA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 1977;67:40-6. 25. Flanigan DP. Biliary carcinoma associated with biliary cysts. Cancer 1977;40:880-3. 26. Tsardakas E, Robnett AH. Congenital cystic dilatation of the common bile duct. Arch Surg 1956;72:311-22. 27. Attar S, Obeid S. Congential cyst of the common bile duct-a review of the literature and a report of two cases. Ann Surg 1955;142:289-95. 28. Lilly JR. The surgical treatment of choledochal cyst. Surg Gynecol Obstet 1979;149:36-42. 29. Zimmon DS, Falkenstein DB, Manno BV, Clemett AR. Choledochocele: radiologic diagnosis and endoscopic management. Gastrointest Radiol 1978;3:349-51. 30. Siegel JH, Harding GT, Chateau F. Endoscopic incision of
GASTROINTESTINAL ENDOSCOPY
choledochal cysts (choledochocele). Endoscopy 1981; 13:200-2. 31. Venu RP, Geenen JE, Hogan WJ, et al. Role of endoscopic retrograde cholangiopancreatography in the diagnosis and
treatment of choledochocele. Gastroenterology 1984;87:1144-9. 32. Yeong ML, Nicholson GI, Lee SP. Regression of biliary cirrhosis following choledochal cyst drainage. Gastroenterology 1982;82:332-5.
The evolution of the double pylorus
At the third gastroscopy 1 month later a short slit was found at the juxtapyloric site. It was characterized by regular margins and clear liquid and air bubbles passed through it during peristalsis; there was no evidence of ulcer lesions. The pylorus appeared slightly rigid (Fig. 2). The patient did not relate any symptoms of peptic ulcer until March 23, 1983, when he noted postprandial epigastric pain. The fourth gastroscopy 10 months later showed a prepyloric ulcer 1.5 em in diameter, and its margins showed a high degree of edema and congestion. The pylorus appeared undamaged (Fig. 3) 1 month later. At the fifth gastroscopy the lesion appeared unchanged, but at the last (sixth) gastroscopy, the ulcer site had scarred over, while an antroduodenal passage was formed by two round orifices of about the same size, separated by a transverse band. The pylorus appeared patent, regular in shape, and detectable from the newly formed orifice by the presence of peristaltic opening and closing movements (Figs. 4 to 7). Histological examinations performed during these endoscopies evidenced chronic gastritis with atrophy, intestinal metaplasia, and mild dysplasia. A radiologic examination (June 15, 1983) showed a deformed pyloric channel and an antropyloric fistula parallel to it. At this time the patient is asymptomatic.
Silvano Fayenz, MD
The double pylorus is an acquired abnormality marked by the presence of two orifices (the natural pyloric opening and an ulcer-induced one) separated by a band, arising at the margin of the pylorus itself. The first case of double pylorus studied endoscopically was described in 1969. 1 Various anatomical types of gastroduodenal fistula may arise. Gould2 described two types of spontaneous gastroduodenal fistula: the first type originates from the lesser gastric curvature and communicates with the first part of the duodenum; the second type originates from the posterior stomach wall and communicates with the third or fourth part of the duodenum. Drapkin et al. 3 outlined a third type of gastroduodenal fistula connecting pylorus and duodenum, thus forming a pyloric septum. Several forms of this third fistulous type can be found owing to different perforative paths taken by the prepyloric ulcer. 4- 21 Rodhe et al. 22 observed with periodic endoscopic examinations a patient with prepyloric ulcer who, after 11 months, evidenced a gastroduodenal channel paralleling the pylorus. Hegedus et aLB proved with subsequent radiologic examinations the progressive development of a second pyloric channel arising from the penetration of a prepyloric ulcer into the duodenal bulb. We describe a case in which the penetration of a prepyloric ulcer gave origin to the formation of a true double pylorus. We are unaware of other reports including a complete documentation of the sequence of endoscopic findings. CASE REPORT
A 58-year-old man, suffering from alcoholic cirrhosis with ascites, was hospitalized on May 6, 1982, on account of a radiologically diagnosed prepyloric gastric ulcer. The first gastroscopy showed a prepyloric peptic ulcer 1.5 em in diameter. The pylorus was only marginally involved by the ulcerative process (Fig. 1). During the second gastroscopy 5 days later the prepyloric ulcer appeared very small in size. On the border facing the pylorus, a tiny slit was found and from this slit a leakage of clear liquid could be observed during peristalsis. The pylorus was slightly inflamed. From the Service of Digestive Endoscopy, Presidio Ospedaliero di Gorizia, Italy. Reprint requests: Silvano Fayenz, MD, Service of Digestive Endoscopy, Presidio Ospedaliero di Gorizia, Via Vittorio Veneto 171, 34170 Gorizia, Italy. VOLUME 32, NO. 1,1986
DISCUSSION
In our case an unusual sequence of events was observed. A prepyloric peptic ulcer penetrated the gastric wall until it reached and slit the duodenal bulb wall. The ulcer healed, but a thin prepyloric slit was left giving rise to a gastroduodenal fistula. An ulcer recurrence at the fistula site took place about 10 months later and, because of its intensive destructive action, caused the widening of this path. This process produced a pillar made up of peripyloric tissue and, in effect, a double pylorus. Both peristaltic function and a~tivity of the pyloric orifice were maintained, indicating the presence of muscle tissue in the dividing band. Although the double pylorus has been considered congenital in origin,23,24 the evidence is overwhelmingly in favor of it being acquired through the mechanism documented in our case. Except for a few cases, the double pylorus has been evidenced in the elderly, almost always in patients over 50 years old. In a patient who did not undergo surgery, Rozelle et al. 25 observed the spontaneous disappearance of the septum, resulting in a single enlarged pyloric channel. 31