Gastrointest Radiol 2, 1 4 5 - 147 (1977)

Radiology ,9 by Springer-Verlag 1977

Choledochocele Demonstrated on Percutaneous Cholangiography: A Patient with Acute Fulminant Pancreatitis Bronwyn Jones Department of Radiology, Harvard Medical School and Peter Bent Brigham Hospital, Boston, Massachusetts, USA

Abstract. A patient with a choledochocele associated with development of acute i'ulminant pancreatitis is presented. Treatment had been severely compromised by this rare anomaly before it was identified by percutaneous cholangiography. Because it is potentially curable with surgery, identification of the lesion by percutaneous or intravenous cholangiography has important clinical significance. Key words: Choledochocele - Percutaneous transhepatic cholangiography - Obstructive jaundice due to pancreatitis.

Congenital lesions at or near the ampulla of Vater rarely cause symptoms. When they do, however, the consequences are usually recurrent and may be lifethreatening. Although rare, this anomaly is important to exclude because it is potentially curable. The diagnosis of choledochocele is usually made by intravenous cholangiography [1]. In the present patient the diagnosis was made by percutaneous cholangiography after severe upper abdominal pain had remained unexplained for a number of years and had critically compromised treatment efforts for another disease.

Case Report M.R., a 37-year-old C u b a n female, underwent bilateral nephrectomy for progressive renal failure due to scleroderma. Her subsequent course was complicated by recurrent pericarditis, shunt infections, and shunt clotting, which required chronic anticoagulation. Severe hypotension after the nephrectomy required continual steroid therapy. Address reprint requests to: Bronwyn Jones, M.D., Department of Radiology, Harvard Medical School, 25 Shattuck Street, Boston, M A 02115, USA

In the next three years she had m a n y hospital admissions for recurrent upper abdominal pain of undetermined cause. During this time a n u m b e r of gastrointestinal series and an oral cholecystogram were normal. Four years after her initial presentation, she was admitted with acute epigastric pain, a markedly raised serum amylase (2980 Somogyi units; 4 days previously this had been 400 Somogyi units), a bilirubin of 2.7/1.9 mg %, a white blood cell count of 16,800, and a hematocrit of 29.5. Acute pancreatitis was the clinical diagnosis. Sodium warfarin (Coumadin) was ceased due to prolonged and abnormal clotting studies. Over the next week liver function tests and serum amylase returned to near normal levels. An oral cholecystogram which did not delineate the gallbladder raised the question of malabsorption due to scleroderma involving the bowel. As clotting studies returned to near normal levels, her anticoagulant was again commenced. Return of the severe abdominal pain two days later suggested recurrent pancreatitis or c o m m o n bile duct stone. Bilirubin rose from 2.7 mg to 7 mg %, precluding intravenous cholangiography, and the serum amylase rose markedly. The patient began to bleed from the vagina and the sodium warfarin was ceased to prevent converting acute pancreatitis into hemorrhagic pancreatitis. To rule out a surgically correctable condition such as c o m m o n bile duct stone, percutaneous cholangiography was performed (Figs. 1 and 2). It was decided to postpone surgery until her condition had stabilized, but, despite improving liver function tests and decreasing amylase, the patient's condition gradually deteriorated and she died approximately one week later. Post mortem was refused.

Discussion Congenital lesions at or near the ampulla of Vater include choledochal cyst [2], choledochocele [3], enterogenous cyst of the ampulla [4], enterogenous cyst of the common bile duct [5], and diverticulum of the terminal portion of the c o m m o n bile duct [6]. This bewildering array of names reflects the variable types of mucosa found in these lesions, which may be either duodenal, biliary, or a combination of both. Multiple theories exist to explain these anomalies: a possible similarity between the formation ofa ureterocele and a choledochocele, invoking inflammation and partial obstruction of the papilla with swelling


B. Jones: Choledochocele on Percutaneous Cholangiography

Fig. 1. A normal caliber common bile duct with a choledochocele is visible. There is free drainage of contrast into the duodenum Fig. 2. After injecting more contrast medium the pancreatic duct, which also drained into the choledochocele, is demonstrated throughout its length. There is no evidence of chronic pancreatitis or pseudocysts. The Chiba needle is delineated in a normal caliber intrahepatic radicle in the upper left

of the intramural portion of the common bile duct; a congenital duplication cyst at the ampulla; and a congenital diverticulum of the c o m m o n bile duct. Alonso-Lej et al. [7] classified 416 cases of congenital cysts of the c o m m o n bile duct found in the literature : I. Cystic dilatation of the common bile duct II. Diverticulum of the common bite duct III. Cystic dilatation of the ampulla of Vater exclusively affecting the terminal portion of the common bile duct. A literature review by DeOya et al. [8] identified only six cases of type III, of which one case had associated pancreatitis. They added another case with acute pancreatitis but preferred to call the anomaly 'enterogenous cyst of the ampulla of Vater' because of its histologic structure which had appeared to originate from the internal or biliary side of the ampulla. A recent review by Scholz et al. [1] identified t4 cases which they felt fitted their criteria for a choledochocele, namely a terminal club-like dilatation of the intramural portion of the common bile duct which

bulged into the duodenum as an intraluminal mass. Characteristically, these lesions fill on intravenous cholangiography [9] or, as in this patient, on percutaneous cholangiography but present negative defects on barium study. Several of the reviewed patients developed acute pancreatitis and one also had a pseudocyst [10]. Our present patient, with a well-developed class III choledochocele, had had recurrent attacks of abdominal pain for some years with no underlying cause identified. The documented acute pancreatitis apparently deteriorated into hemorrhagic pancreatitis, almost certainly aggravated by her anticoagulant therapy. The altered pressure dynamics and compromised drainage of the biliary and pancreatic ducts by their insertion into the choledochocele was the probable mechanism for development of obstructive jaundice and acute pancreatitis. Stasis and possible incompetence of the sphincter of Oddi may also have played a part. This is analogous to the relationship between diverticula of the duodenum at the ampulla and inflammatory disease of the pancreas [11].

B. Jones: Choledochocele on Percutaneous Cholangiography

Acknowledgment. This work was supported in part by US Public Health Service grant GM 18674. Many thanks to Ms. Debra Sharpe for her typing of this manuscript.

References 1. Scholz F J, Carrera GF, Larsen CR: The choledochocele, correlation of radiological, clinical and pathological findings. Radiology 118.'25 28, 1976 2. Vatero A: Dissertation inauguralis medica, proes. Diss qua scirrhis viscerum disseret c.s.e.z, lerus, 70." 19, University Library, Edinburgh, 1723 3. Wheeler WID: An unusual case of obstruction to the common bile duct (choledochocele?) Br J Surg 27.'446 448, 1940 4. Brooks B, Weinstein A: Cyst of the ampulla of Vater. Am7 Sur~ 117.728 734, 1943 5. Gordimer H, Bluestone L: Enterogenous cyst of the duodenum, a case report. Ann Surg, 132.'1149 1153. 1950

147 6. Sterling JA : Diverticula of the terminal portion of the common bile duct. Am J Path 25.'325 337, 1949 7. Alonso-Lej F, Rever WB, Pessagno D J: Congenital choledochal cyst, with a report of 2, and an analysis of 94 cases. hllernational Ahstracts o[ Surget 3" 108: l 30, 1959 8. DeOya JC, Puentc JL, Villanueva A, Potel J: Enterogenous cyst of the ampulla of Vater. Digestion 2.201 208, 1969 9. Kaftori JK. Munk J, Schramet A, Barzilai D: Intraluminal diverticulum of the duodenum demonstrated by intravenous cholangiography. Br J Radiol 39.390 392, 1966 10. Stephens E. Pauline GJ: Choledochocele: An unusual type of choledochal cyst which presented as acute pancreatitis. Altst NZJSurg36.'124 127, 1966 11. Costopoulos LB, Miller J D R : Insertion of the common bile duct and pancreatic duct into duodenal diverticula. Radiology 89.256 262, 1967

Receired: June 27, 1977: accepted. Jltlv 15, 1977

Choledochocele demonstrated on percutaneous cholangiography: a patient with acute fulminant pancreatitis.

Gastrointestinal Gastrointest Radiol 2, 1 4 5 - 147 (1977) Radiology ,9 by Springer-Verlag 1977 Choledochocele Demonstrated on Percutaneous Cholang...
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