Choledochal cyst: Report of five cases

Clin. Radiol. (1969) 20, 332-336 CHOLEDOCHAL

CYST:

REPORT

OF

FIVE

CASES

S A N G Y. H A N , L O I S C. C O L L I N S , * a n d R O B E R T M. W R I G H T ,

From the Department of Radiology, Baylor University College of Medicine, Houston, Texas, U.S.A. and Department of Surgery, Wallace Memorial Hospital, Pusan, Korea Five cases o f c h o l e d o c h a l cyst occurring in c h i l d h o o d are discussed with pertinent roentg e n o g r a p h i c a n d clinical findings. The clinical manifestations were (1) in early i n f a n c y - - o b s t r u c t i v e jaundice, a n d (2) in c h i l d r e n - - t h e classical t r i a d o f j a u n d i c e , a b d o m i n a l p a i n a n d mass. The m o s t valuable r o e n t g e n o g r a p h i c findings were (1) on p l a i n film o f the a b d o m e n - - m a s s in the right u p p e r q u a n d r a n t . , (2) on b a r i u m m e a l s t u d y - - a n t e r i o r , inferior a n d left sided displacement o f gastric a n t r u m a n d d u o d e n a l bulb, (3) l a t e r a l displacement o f descending d u o d e n u m , a n d (4) inferior displacement o f the third p o r t i o n o f the d u o d e n u m . Direct d e m o n s t r a t i o n o f the cyst m a y be accomplished b y i n t r a v e n o u s cholangiography, direct p e r c u t a n e o u s cystography, a n d possibly oral cholecystography.

INTRODUCTION C h o l e d o c h a l cyst is a congenital cystic d i l a t a t i o n o f any segment o f the e x t r a h e p a t i c biliary ducts; it is caused p r i m a r i l y by congenital weakness o f the wall. Characteristically, the d i l a t a t i o n involves only the c o m m o n duct, b u t m a y involve the extrahepatic p o r t i o n o f the hepatic duct a n d has been r e p o r t e d in one case involving the cystic duct (Gross, 1953; Weinstein, 1965). Usually there is a short segment o f n o n d i l a t e d duct distal to the dilatation, a n d some degree o f o b s t r u c t i o n at the distal end o f the duct. C h o l e d o c h a l cyst is n o t as rare as is generally suggested b y the literature; the n u m b e r o f cases r e p o r t e d has increased in recent years. Since early diagnosis a n d t r e a t m e n t o f the d i s o r d e r can p r e v e n t serious complications, familiarity with signs a n d s y m p t o m s is i m p o r t a n t . I n this c o m m u n i c a t i o n , we present 5 cases o f i d i o p a t h i c d i l a t a t i o n o f the c o m m o n bile duct in K o r e a n TABLE 1 CLINICAL FEATURES OF PATIENTS WITH CHOLEDOCHAL CYST

Case

Age, Yrs.

Sex

Abdominal pain

Abdominal mass

1

7

F

+

+

2

14

F

+

+

+

3

6

F

+

+

+

4

15

M

+

+

+

5

1/12

F

+

Jaundice

children, a n d discuss the clinical a n d roentgenographic manifestations o f this disorder. A summary o f clinical features o f the cases is given in Table 1. CASE REPORTS Case 1.--A seven year-old girl was admitted to the hospital with fever, abdominal pain and mass. She had had several similar episodes during the year before examination. Physical examination disclosed a cystic mass (10 × 10 × 10 cm.) in the right upper quandrant of the abdomen. After percutaneous aspiration of the bile from the cyst, injection of contrast material demonstrated the size and location of the cyst. (Fig. 5). At surgery, cystic dilatation of the common duct with stenosis of the distal part was found. The gall bladder was not dilated. The cyst was drained through a choledochocystoduodenostomy. Follow-up barium meal study demonstrated the residual cyst containing barium, bile, and gas. (Fig. 6). Case 2.-- A 14 year-old girl was admitted with jaundice, intermittent fever and abdominal pain, and mass, all of several years duration. The pertinent physical findings were mild jaundice and a large fluctuating mass occupying the entire abdomen. X-ray examination of the upper gastrointestinal tract demonstrated inferior and lateral displacement, and widening of the duodenal loop. (Fig. 1 and Fig. 2). Colon examination by barium enema showed obstruction, apparently caused by the mass, at the splenic flexure (Fig. 3). At surgery, a large cystic dilation of the common duct with narrowing of the distal part was noted. The cyst was drained through choledochocystoduodenostomy. Case 3.--A six year-old girl was admitted with seven month history of intermittent jaundice, fever and abdominal pain. Physical examination disclosed an egg-sized mass in the right upper quandrant of the abdomen. Intravenous cholangiography opacified the mass. (Fig. 4). Neither gallbladder nor other biliary ducts were visualized. At surgery, cystic dilatation of the common duct with

* Present address: Rosewood General Hospital, 9200 Westheimer Road, Houston, Texas, U.S.A. 332

CHOLEDOCHAL

duodenum.

CYST

--

A r r o w indicates p o s i t i o n o f l i g a m e n t o f Treitz.

Fro. 3

REPORT

OF

FIVE

CASES

FIG. 2

333

334

CLINICAL

RADIOLOGY

stenosis of the distal portion was found. The cyst was drained through choledocholcystoduodenostomy. Case 4.--A 15 year-old boy was admitted to the hospital with abdominal mass, pain and jaundice, all of which had occurred intermittently during the previous two years. Barium study of the upper gastrointestinal tract demonstrated anterior, inferior and lateral displacement of the gastric antrum and duodenal bulb by the mass. At surgery, dilatation of the common duct ("adult fist size") with narrowing of distal part was found. Choledochocystoduodenostomy was performed to drain the cyst. Case 5.-- A one month-old infant was admitted to the hospital because of progressive jaundice since birth. The liver was slightly enlarged. Operative cholangiography demonstrated fusiform dilatation of the common duct (1 '0 cm. in diameter) with severe stenosis of the distal part. Gallbladder and intrahepatic ducts were normally developed and the gallbladder not enlarged. Choledocholcystoduodenostomy was performed.

found to have obstruction of the distal end of the common duct. In most cases, there is inflamma, tory change in the wall of the cyst. The gallbladder is usually displaced but not otherwise involved. The cysts vary greatly in size, some containing several liters (Smith, 1942). A few cases with stones in the cyst have also been described (Alonso-Lej, Rever and Pessajno, 1959). Both diverticulum and choledochocele of the common duct are extremely rare; only a few cases of each have been reported to date. The diverticulum arises from the lateral wall of the common bile duct. Choledochocele is cystic dilatation of the intraduodenal choledochus and is analogous anatomically to ureterocele.

DISCUSSION The etiology of choledochal cyst is not known. The most widely accepted theory assumes two factors in the causation of choledochal cyst: (1) weakness of the wall of the common duct, and (2) obstruction distal to it (Gross, 1953). Although both factors have been considered congenital by most writers, there have been a few reports (AlonsoLej, Rever and Pessajno, 1959) which suggest that the obstruction may be acquired as a result of infection or pressure by the mass of the cyst. It is conceivable that the transient increase in pressure which results from normal function of the sphincter of Oddi may be sufficient to cause the cystic dilatation in a patient with a congenitally weak wall. Some degree of obstruction is usually encountered at operation or autopsy, but since these patients usually have had episodes of infection, the possibility of obstruction having been acquired (rather than congenital) cannot be excluded. Pathologically, three types of idiopathic dilatation of the common duct have been described (AlonsoLej, Rever and Pessajno, 1959): (1) cystic dilatation of the common duct, (2) diverticulum of the common duct, and (3) choledochocele. The majority of cases belong to the first type. All 5 patients in our series are of this group. Dilatation is usually confined to the common duct, thus differentiating it from that caused by stone or tumor in the distal part of the common duct. Although there have been some cases in which dilatation extended to the hepatic and cystic ducts, usually these are spared. The narrowing at the distal end of the common duct has been found to be caused by: (a) stenosis and valve-like structures in the intraduodenal choledochus, (b) angular insertion into the duodenum, and (c) external mass (cyst) around the distal duct. All the patients in our series were

C L I N I C A L ASPECTS Orientals are affected more frequently than any other race. In Caucasians, choledochal cyst occurs four times as frequently in females as in males; among the Japanese there is no difference in sex incidence. The disorder is usually recognised in children and young adults. In a review of 92 cases Alonso-Lej et aI. (1959) found that 18% of the patients were less than one year of age, 45 were less than 20 years and 82 % were under 30 years of age. In infants, the occurrence of obstructive jaundice in association with choledochal cyst has often led to the mistaken diagnosis of biliary atresia. Fonkalsrud and Boles (1965) suggest that choledochal cyst may be a variant of biliary atresia. In case 5 presented here, the preoperative diagnosis was biliary atresia. At operation, dilatation of the common duct with narrowing of the distal end was found, thus distinguishing it from biliary atresia in which the entire bile duct usually is atretic. Clinical manifestations of choledochal cyst depend primarily on the time of onset of symptoms. In early infancy, the disorder presents with jaundice and acholic stools, signs indistinguishable from obstructive jaundice due to biliary atresia. In older children, symptomatology is characterized by the classical triad of jaundice, abdominal pain and mass. This triad occurred in 63 % of the cases reviewed by Tsardakas and Robnett (1956). However, in a recent review of 92 cases, Alonso-Lej et al. (1959) found it in only 21% of the patients. This is attributable to earlier recognition and treatment. The triad was noted in 4 of our patients. The individual components of the triad vary in frequency and severity, depending upon the degree of infection and size of the cyst. Abdominal pain and jaundice are intermittent and are usually associated with infection.

CHOLEDOCHAL

CYST - -

The complications of choledochal cyst are cirrhosis of the liver, cholangitis, and rarely, bile peritonitis due to perforation. Three cases of portal hypertension with bleeding esophageal varices complicating choledochal cyst have been reported (Fonkalsrud and Boles, 1965; Gills and Sergeant, 1962) RADIOLOGICAL MANIFESTATIONS On the plain film of the abdomen, the only finding is a mass in the right upper quandrant; this may vary considerably in size in different patients. In one of our patients, the mass occupied almost the entire abdomen, displacing the bowel inferiorly. No instance of calcification in the cyst has been encountered in our patients or in the literature. Barium meal study of the gastrointestinal tract is useful in demonstrating the mass. The antrum of the stomach and first and second portions of the duodenum are displaced anteriorly and sometimes downward, and to the left. The third portion of the duodenum is displaced inferiorly (Fig. 1). This results in an expansion of the duodenal loop, as seen in anteroposterior projection. On the lateral projection, the loop is displaced far anteriorly (Fig. 2). In one patient in whom the cyst was very large, the colon was obstructed at the splenic flexure by extrinsic pressure (Fig. 3). The changes caused

REPORT

OF F I V E C A S E S

335

by diverticulum of the common duct are similar to those caused by the more common cystic dilatation of the duct. Choledochocele may cause a polypoid defect in the second portion of the duodenum demonstrable by barium meal examination (Alden and Sterner, 1957). Neither cyst nor gallbladder was opacified by the oral cholecystogram in any of our patients. There have been only two instances in the literature in which the gallbladder and cyst were demonstrated by this method (Liebner, 1958; Mosley, 1957). The failure of opacification may be a result of hepatic damage, stasis in the biliary ducts, dilution of the contrast material by a large quantity of fluid in the cyst, and inability of the cyst to concentrate the contrast material. Mosley expressed the view that the incidence of cholecystographic opacification of the gallbladder and the cyst can be increased if examination is delayed until jaundice has subsided, and if films are taken at later intervals than in the usual study. Intravenous cholangiography may demonstrate the cyst in a patient with normal bilirubin (Liebner, 1958; Loring and Strehlinger, 1957), since the greater density of the opaque material excreted through the liver permits its recognition even without the benefit of concentration in the gallbladder. In one patient in our series, the choledochal cyst was demonstrated by this method (Fig. 4).

FIG. 5 FIG. 6 F~G. 5.--Case 1.--Direct demonstration of the cyst by percutaneous injection of the contrast material. FIG. 6.--Case 1.After choledochoduodenostomy, barium meal demonstrates the residual cyst containing (a) barium (lower arrows), (b) bile (white arrows), and (c) gas (upper arrows). The site of communication between the duodenum and cyst is indicated by ~-.

336

CLINICAL RADIOLOGY

Operative cholangiography through either choledochocystostomy or cholecystostomy is mentioned as the most convincing diagnostic procedure for delineation of the anatomical relationship to the contiguous structures (Fonkalsrud and Boles, 1965; Tsardakas and Robnett, 1956). As a matter of fact, this may be the only available method of diagnosing choledochal cyst in young infants. The direct percutaneous puncture of the cyst, aspiration of the contents and injection of contrast material permits specific preoperative diagnosis and demonstration of the size and position of the cyst (Gundersen, 1965; Kjelberg, 1948; Lumpkin and Wilson, 1955). Since this entails some risk of bile peritonitis, surgery should be planned to follow the procedure. Adequate decompression of the cyst before injection of the contrast material is important to prevent leakage of bile. In one patient in this group, this procedure was successfully performed without complication (Fig. 5). TREATMENT The internal drainage of the cyst either through choledochocystoduodenostomy (Gross, 1953) or choledochocystojejunostomy with Roux en Y

anastomosis is the treatment of choice (Attar and Obeid, 1955). In our patients, choledochocysto. duodenostomy was primarily used. Follow up examination of the patients after interval drainage demonstrated gradual diminution in size of the cyst (Fig. 6). REFERENCES ALDEN, J. F. & STERNER,E. R. (1957). Ann. SurE. 145, 269. ALONSO-LEJ,R. B., REVER, W. B. & PESSAJNO,W. B. (1959). Int. Abstr. of SurE. 108a, 1. ATTAR, S. & O~EtD, S. (1955). Ann. SurE. 142, 289. FONKALSRtJD, E. W. & BOLES, E. T. (1965). SurE. Gyn,and Obst. 121,733." GrLLs, D. A. & SERaEAWr,C. K. (1962). SurE. 52, 391. GROSS, R. E. (1953). The Surgery of Infancy and Childhood, Chap. 42. Philadelphia and London: W. B. Sanders Co. GUNDERSEN,J. (1965). Acta Chir. Seand. 129, 606. KJELBERG,S. R. (1948). Aeta Radiol. 29, 307. LrEBNER,E. J. (1958). Am. J. Roentg. 80, 950. LORING, P. & STREHLINGER,L. (1957). Fortsehr. Rontgenstrahl. 86, 395. LUMPION, F. E. & WILSON, J. W. (1955). Am. Surgeon 21, 63. MOSLEV,J. E. (1957). RadioI. 68, 849. SMn~, B. C. (1942). Arch. SurE. 44, 963. TSARDAKAS,E. & ROBNETr, A. H. (1956). Arch. SurE. 72, 311. WEINSTEIN,C. (1965). Arch. Int. Med. 115, 339.

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