Choledochocele and Recurrent Pancreatitis Diagnosis and Surgical Management
Frederick L. Greene, MD, Columbia, South Carolina J. Jeffrey Brown, MD, Columbia, South Carolina Paul Rubinsteln, MD, Columbia, South Carolina Marion C. Anderson, MD, Charleston, South Carolina
Since the first description of a choledochal cyst by Vater in 1723 and the subsequent description of the pathologic characteristics and symptoms of such cysts by Douglas in 1852, the literature on choledochal cysts as an entity have included case presentations as well as large series of patients [1]. Impressive experience with this particular lesion has been reported in the Japanese literature, since in Japan, large groups of patients have been collected over relatively short periods of time [2-4]. Classifications of choledochal cysts have generally followed the tenets of Alonzo-Lej and co-workers [5l, who described three specific types of cysts. The type I cyst (Figure 1) represents a general dilation of the extrahepatic common bile duct and is, by and large, the most frequent type of cyst noted in most series. The type II cyst (Figure 1) represents a single diverticulum of the common bile duct and has been relatively rare in most collected series. The type III cyst, or choledochocele, has classically been depicted as a dilation of the distal common duct into the lumen of the duodenum (Figure 2). This entity has rarely been described, and no large experience in surgical management currently exists. Our experience with a patient having a rare type III cyst associated with recurrent bouts of pancreatitis prompted this report. The methods of diagnosis and eventual surgical management are presented herein.
Case Report A 35 year old white man was admitted to the Dorn Veterans Administration Hospital in Columbia, South Carolina in July 1982 with recurrent pancreatitis. The patient's history dated to approximately 25 years earlier when he was noted to have recurrent episodes of abdominal pain From the I ~ of Surgeryand Medicine,UnNersltyof South Carolina ScJ'molof Medicine, Colvwnbla,South Carolina and the Department of Surgery. Medica! University of South Carolina, Chark~ston,South Cerollna. Requests for reprints should be addressedto Frederick L. Greene. MD, Surgical Service, Dorn Veterans AdministrationHospital, Columbia,South C.,srollne29201.
that required hospitalization. No documentation of hyperemylasemia was available from the previous hospital. During the most recent 2 year period, the patient had been admitted multiple times with documented acute pancreatitis. There had been no history of peptic ulcer disease, alcohol abuse, abdominal trauma, or hepatitis, although the patient admitted to beingjaundiced for a 2 week period in 1964. Evaluation in the past included endoscopic retrograde cholangiopancreatography performed in another hospital in October 1980. The report described nonvisualization of the pancreatic duct, but no other abnormal anatomic findings. Ultrasonography of the abdomen had shown a normal gallbladder and no evidence of a pancreatic mass, although a double-dose oral cholecystogram showed no gallbladder visualization. In November 1980, the patient had a repeat episode of pancreatitis and was again hospitalized at the Dorn Veterans Administration Hospital. Because of the abnormal cholecystogram, a cholecystectomy was performed in conjunction with an intraoperative cholangiogram(Figure 3). There were no gallstones found, but mild chotecystitis was noted pathologically. The patient had an unremarkable course and was discharged on the sixth postoperative day. In February 1981, the patient was again admitted with severe abdominal pain radiating to the back associated with nausea and vomiting. The serum amylase level was 990 IU on admi~ion and increased to 2,415 IU on the first hospital day. Within 3 days, the serum amylase value returned to normal limits. Repeat abdominal ultrasonography revealed no abnormalities. The patient was discharged after several days with a diagnosis of idiopathic pancreatiffs. In June 1981, the patient was again admitted complaining of epigastric pain over a 12 hour period with a serum amylase level of 2,850 IU. After several days, the serum amylase leveLreturned to normal limits, and he became asymptomatic. In February 1982, hospitaladmission was required because of severe abdominal pain, nausea, and vomiting which were consistentwith the patient'sprevious bouts of pancreatitis. The serum amylase level at that time was 4,275 IU. Over a 48 hour period, the patient again had cessationof pain and his amylase value returned to normal limits. Repeat endoscopic retrograde cholangiopancreatography showed dilation of both the common bile duct and the pancreaticduct,but no dei'mitearea of obstruction.
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Choledochocele and Recurrent Pancreatltls
In July 1982, the patient again was admitted with pancreatitisassociatedwith hyperarnylasemia,leukopenia,and fever.The film of February 1982 was reviewed and reinterpretedas showing a choledochoceleof the common bile duct (Figure 4). A surgicalprocedure was recommended, but because of personalbusiness,the patientelectedto be discharged. T w o weeks later,the patient was readmitted with recurrent pancrcatitis.Physicalexamination was generally unremarkable except that the abdominal examination showed tenderness to palpationin both upper quadrants. At this time, it was thought that the paticnt'srecurrent bou~ of pancreatitismight be relatedto the findingof a choledochocele or type Ill choledochal cyst.The patient agreed to surgicalinterventionand in August 1982, un. derwent operative pancreatography, common bile duct exploration,and sphincteroplasty.O n the sixth postoperativeday, a T-tube cholangiogram was performed, fol. lowcd by removal of the T tube on the seventh postoperativeday. He was dischargedon the ]0th postoperativeday with a normal serum amylase level.The patienthas been seen frequentlysincethisprocedure and has remained free of symptoms for the past 24 months. Comments T h e term choledochocele was coined by Wheeler [6] in the 1940 report of a patient that he had operated on 30 years earlier.Although biliarytract complications for all types of choledochal cysts have been described, there is littlein the surgical or medical literature relating the choledochal cyst to recurrent pancreatitis.Cuschieri and Davies [7] reported a case of acute pancreatitis complicating a type I choledochal cyst located in the supraduodenal portion of the c o m m o n bile duct. This patient was treated by a choledochocystoduodenostomy. Iriyama et al [8] presented a case and reviewed 15 published cases of choledochoceles and found none in which the patient presented with associated pancreatitis. T h e y reported that most of the patients were treated by cyst excision with various forms of internal drainage. In a 1981 report by O l d h a m et al [9],five cases of choledochal cysts were presented, one of which represented a type III cyst or choledochocele associated
i"
Figure 1. Type ! choledoch~l cysf #bowing diffuse dilation of common bile duct (Ieft). Type U cyM shows diverticulum of common bile duct (right).
with recurrent abdominal pain and eventual necrosis of the pancreatic head. This patient was treated with a pancreaticoduodenectomy because of extensive destruction. Stephens and Pauline [I0} reported the case of a young man who presented with acute pancreatitis and a pancreatic pseudocyst who eventually was diagnosed as having a choledochocele. These investigators credited Sterling [11] with first suggesting in 1949 that pancreatitis may be related to cystic dilation of the terminal common duct. The few published cases of choledochocele show that this lesion generally is found in the adult patient after multiple attempts to resolve the cause of vague symptom complexes [12]. Powell and co-workers [13] reported that type II and type Ill cysts are more likely to be located by operative cholangiography performed routinely in patients with symptoms of biliary tract disease who, at the time of cholecystectomy, are found to have an acalculous gallbladder. This axiom was confirmed in our patient when the operative cholangiogfam indeed demonstrated a choledochocele on critical review (Figure 3).
~ 2 . Type/Ks (/of/) and Jib (n~dd~) choledocl',al c)qrls ehow a lar9~ intralurninal choledochocele with common
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the pam:realic and common bile ducts.
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Figure 4. Film showing Intramural choledochoceta (arrow).
Figure 3. Operative cholangiogrem showing dilated distal common bile duct (choledochocelo) and compression of the pancreatic duct (arrow).
Various diagfiostic procedures, including isotopic studies and routine contrast studies of the gastrointestinal and biliary tracts, have been described as helpful in diagnosing choledochal cysts [14,15]. The classic appearance of a choledochocele was seen during endoscopic retrograde cholangiography on our patient and it became the principal study on which the diagnosis rested (Figure 4). Radioisotopic studies were not included in the workup of this patient. The surgical management of choledochal cysts, especially of the more common type I cyst showing diffuse dilation of the common bile duct, has been controversial. As originally described, by Bakes, early management rested primarily on cyst drainage, generally using choledochocystoduodenostomy or a Roux-Y choledochocystojejunostomy [1]. As a result of significant numbers of carcinomas found in cysts after drainage procedures [1,16], a more aggressive operative approach employing excision has recently been recommended. The pathophysiology of the type I and type II cysts shows that there is no true mucosal lining in these cysts and, therefore, recurrent inflammation and irritation are more likely to set up
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a neoplastic process. Type Ill cysts generally include the mucosal lining of the duodenum or bile duct [8] and, therefore, may not be subject to the same pathologic irritantsas the other types of cysts. Since the type Ill cyst is especially rare, there has been no unified approach to itssurgical management. In large series of patients, especially those reported from Japan, the type III cyst has comprised less than 2 percent of all choledochal cysts [17]. In the review by Todani and co-workers [2], which included 37 patients seen over a 16 year period, the type III cyst,or choledochocele, was not encountered. These researchers did believe, however, that the concept of a transduodenal sphincteroplasty m a y be the treatment of choice for the type III cyst. A review by Powell et al [13] has suggested that the appropriate management for a choledochocele should be excision with reanastomosis of the common bile and pancreatic ducts to the duqdenum. We believe that a sphincteropl~sty, as performed in our patient, is a logical procedure to use when the choledochocele is contained in the intramural portion of the duodenum. Therefore, we have expanded the classification of Alonzo-Lej et al to represent the three possible anatomic variants of a choledochocele (Figure 2). Type IIIa represents the choledochocele that presents in the intraluminal duodenum and contains the terminal pancreatic and common, bile
The American Journal of Surgery
Choledochocele and Recurrent Pancreatitis
ducts as a common channel, type IIIb contains separate ductal structures with an intraluminal cyst, and type IIIc (present case) shows a cyst completely contained within the intramural portion of the duodenum. Endoscopic retrograde cholangiopancreatography and the operative findings support the concept that the choledochocele in our patient created an obstruction to the pancreatic duct intermittently when the choledochocele became distended with bile. Since the common channel theory was first promoted by Opie in the early 20th century, recurrent pancreatitis secondary to biliary tract disease has been supported by the fact that 75 percent of patients with a common channel will display this anatomic characteristic within the intramural portion of the duodenum [3]. A moderate stenosis of the ampulla of Vater, as noted in our patient, was also important in retarding bile flow, which contributed to the intermittent dilation of the choledochocele. For these reasons, a sphincteroplasty is the most logical approach to correcting the problem of recurrent pancreatitis secondary to an intramural (type IIIc) choledochocele.
Summary A case history has been presented that reports a rare type III choledochal cyst (choledochocele) and describes the association of a choledochocele and recurrent acute pancreatitis. The most reliable diagnostic technique, as used in our patient and supported by our literature review, is endoscopic retrograde cholangiopancreatography. This case further supports surgical correction employing transduodenal sphincteroplasty. This procedure is highly recommended for management of the intramural choledochocele (type IIIc), especially when associated with recurrent pancreatitis. Finally, patients who present with recurrent pancreatitis without the usual histerical or diagnostic findings of biliary tract
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or alcoholic disease should have a choledochocele included in the differential diagnosis. Acknowledgment: We thank Susan Hiller for preparation of the illustrations and Lucile Moore for preparation of the manuscript. References 1. Spitz L. Choledochal cyst. Surg Gynecol Obstet 1978;147: 444-52° 2. Todant 1", Watanabe Y, Narususe M, ot al. Congenital bile duct cysts. Am J Surg 1977; 134:263-9. 3, One J, Sakoda K, Aklta H. Surgical aspect of cystic dilatation of the bile duct. Ann Surg 1982;195:203-8. 4, Matsumoto Y, Uchlda K, Nakase A, et al. Cltnlcopathologlc classification of congenital cystic dilatation of the common bile duct. Am J Surg 1977;134:569-74. 5. Alonso-Lej F, Rever W, Passagno D. Congenital choledochal cyst, with a report of 2 and an analysis of 94 cases. Int Abstr Surg 1959;108:1. 6. Wheeler W. An unusualcase of obstruction of the common bile duct (choledochocele?). Br J Surg 1940;27:446-8. 7. Cuschlerl A, Davies R. Acute pancreatltls complicating a choledochal cyst. Br MealJ 1969;3:698. 8. Irlyama K, Mori T, Takenaka 1", et aL Choledochocele. Jpn J $urg 1980;10:149-54. 9. Oldham K, Hart M, White 7". Choledochal cysts presenting in late childl~3d and adulthood. Am J Surg 1981;141:568. 10. Stephens F, Pauline G. Choledochocele: an unusual type of choledochalcyst which presented as acute pancreatitis. Aust NZ J Surg 1966;36:124-7. 11. Stlrling J. Diverticula in the terminal portion of the common bile duct. Am J Petho! 1949;25:325-37. 12, Relnus F, Welngartan G. Choledocele of the common bile duct. Am J Surg 1976; 132:646-8. 13. Powell C, Sawyers J, Reynolds V. Management of adult cholodochal cysts. Ann Surg 1981; 193:666-76. 14. Hcang M, Klaw Y. Intravenous cholecystlgraphy rising Tc99m-labeled agents in the diagnosis of choledochal cyst, J Nucl Med 1982;23:113-6. 15. Klein G, Frost S. Newer imaging rnodallties for the preoperative diagnosis of choledochal cyst. Am J GasVoenterol 1981; 76:148-52. 16. Gupta $, Kumar A, Gupta S, et al. Carcinoma in choledochal cyst, J Surg Oncol 1981;16:313-8. 17. Yamaguchi M. Congenital choledocl'~l cyst. Am J Sung 1980;140:653-7.
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