Congenital dilatation of the biliary tract

Congenital Dilatation of the Biliary Tract GABRIEL A. LORENZO, MD, Chicago, Illinois RANDOLPH W. SEED, MD, Chicago, Illinois JOHN M. BEAL, MD, Chicago, Illinois

Congenital dilatation of the biliary tract is an uncommon condition. The location, degree, and type of dilatation vary. The extrahepatic portion of the ductal system is involved most frequently and more than 500 cases of this type have been reported. Communicating segmental dilatation of the intrahepatic radicles is rare. The unusual nature of this type of anomaly is emphasized by the finding of only ten similar cases in the world literature. Four patients with congenital dilatation of the biliary tree have been treated at Northwestern University Medical Center during the past three years. These cases illustrate variations in the findings encountered in patients with this anomaly, and have prompted a study of the surgical therapy for such congenital lesions. Case

Reports

CASE I. The patient, a seventeen year old white woman, was admitted to Passavant Memorial Hospital for the second time in December 1966, complaining of severe right upper quadrant pain of four hours’ duration. The pain radiated to the right subscapular area and was associated with vomiting and fever. She had the first of four distinct similar episodes more than a year prior to the present admission. These attacks were not precipitated by the ingestion of fatty foods. The patient had never been jaundiced and the stools had not been light or the urine dark. Physical examination was unremarkable except for right upper quadrant abdominal tenderness with involuntary muscle spasm. Laboratory studies on admission revealed the following: hemoglobin, 13.4 gm per 100 cc ; white blood cell count 8,700 per mm3, with a normal differential. The results of urinalysis were normal. Serum amylase was 95 Somogyi units, total bilirubin 2.2 mg per 100 ml with 1.3 mg per 100 ml direct fraction. A gallbladder series obtained on her first admission one month earlier failed to visualize the gallbladder after a double dose of TeIepaque@. Intravenous pyelogram showed normal findings. From the Department of Surgery, School. Chicago, Illinois 60611.

510

Northwestern

University

Medical

Because the patient failed to improve during the twenty-four hours after admission, exploratory laparotomy was performed with a preoperative diagnosis of acute cholecystitis. The gallbladder was found to be normal in size and texture and did not contain stones. The common duct was markedly dilated. (Figure 1.) An operative cholangiogram showed a partially filled tortuous common duct measuring 11 cm in the greater diameter with a tapered distal end. The cystic duct measured 1.5 cm in diameter. Cholecystectomy was performed. Exploration of the common duct was carried out and a single 8 by 3 mm stone was recovered. Rouxen-Y choledochocystojejunostomy was performed. The postoperative course was uneventful and the patient has been well for two years.

CommenQ: This report is presented as an example of a choledochal cyst in a young patient whose symptoms were compatible with those of acute cholecystitis. The classical triad of abdominal pain, jaundice, and abdominal mass which occurs in approximately two-thirds of the patienks with this anomaly was not present in this patient. The symptoms associated with this anomaly may simulate those produced by more conventional biliary tract disease so that the diagnosis as in this case is made at the time of operation. Common duct stones have been reported in association with choledochal cyst in five instances [l-5]. CASE II. The patient, a nineteen year old mentally retarded white woman, was transferred to Chicago Wesley Memorial Hospital from another hospital in March 1967 for evaluation of an abdominal mass, jaundice, and distention. Four months earlier anorexia, frequent bowel movements, gray colored stools and the onset of jaundice had developed. She had lost 20 pounds in weight. After an episode of hematemesis, she received three units of blood and was transferred to our institution. On ,admission, the patient was lethargic, deeply icteric, and cachectic. The abdomen was distended. Hepatomegaly (16 cm below the right costal margin) was present. A distended gallbladder measuring 8 cm in diameter was palpated. Laboratory studies revaaled hemoglobin of 11.1 gm per 100 cc, white blood

The American

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of Surgery

Congenital

Dilatation

of Biliary Tract

cell count of 5,880 per mm3 ; serum albumin

was 2.5 gm per 100 cc, globulin 5.0 gm per 100 cc; platelets were 192,500 per mm3. Total serm bilirubin was 17.9 gm per 100 cc, with direct fraction of 7.2 mg per 100 cc. Serum alkaline phosphatase was 15 Bodansky units, serum glutamic oxaloacetic transaminase 177 units, and lactic dehydrogenase 270 units. Results of urinalysis were normal. One and a half liters of ascitic fluid were removed the day after admission. The fluid was clear, without abnormal cells, and had a total protein content of 1.1 gm per 100 cc. A liver scan showed markedly enlarged liver with multiple areas of decreased perfusion in the right lobe. An upper gastrointestinal radiologic study showed extrinsic compression upon the duodenum. After suitable preparation, abdominal exploration was performed. At operation a distended gallbladder was found and the common duct was uniformly dilated with a diameter of 4 cm. Operative cholangiogram through the gallbladder (Figure 2) confirmed the dilatation of this structure with a tapered distal end. The contrast agent did not enter the duodenum. Neither the gallbladder nor common duct contained stones. The liver and spleen were enlarged. Choledochocystoduodenostomy was performed. The gallbladder was left in place ,and drained through a separate stab wound. Liver biopsy was performed. A considerable amount of ascitic fluid was drained during the postoperative period. The patient was discharged on the thirty-fourth postoperative day. Twenty months later, the patient was well and free of symptoms.

Comment: The clinical picture of this patient is quite different from the preceding case, although both cases have been classified as type I (congenital choledochal cyst) [ 61. The presence of an abdominal mass and history of jaundice in a young woman support the clinical diagnosis of choledochal cyst, although the mass in this instance was the enlarged gallbladder. In 1966 Strode [?‘I reported an unusual case similar to this one in which the ductal system was diffusely dilated with a distended gallbladder. CASE III. The patient, a thirty-two year old white woman, w(as admitted to Passavant Memorial Hospital for the first time on May 4, 1969, complaining of epigastric and back pain of five months’ duration. The epigastric pain was relieved by antacids and the back pain by analgesics. She did not complain of nausea, vomiting, or change in bowel habits, nor was the pain related to the ingestion of fatty meals. Evidence of jaundice was absent. Physical examination was unremarkable. The patient was thin, but did not appear ill. The abdomen was soft, wJthout masses or tenderness. Laboratory studies of urine
Volume 121, May 1971

Figure 1. Case 1. Common dochal cyst.

duct was represented

by chole-

Figure 2. Case If. Operative cholangiogram demonstrated tapered distal end of common duct and dilated gallbladder and common duct.

Figure 3. Case III. The choledochal cyst was visualized injection of contrast material into the cyst.

by

511

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TABLE

Seed, and’ Beat

Total Number of Cases of Congenital Dilatation of the Biiiary Tree

I

Author Aionso-Lej et al Lee et al Hays et al Reports

not included

in previous

pubii-

cation Present report

Year

Number of Cases

1959 1969 1969 195969

403 97 10 25

1969

Total

4 539

markedly enlarged gallbladder. Chest roentgenogram and electrocardiogram were negative. Abdominal exploration was performed on May 5, 1969. At operation, a cystic mass was found displacing the duodenum anteriorly and medially. The gallbladder was normal in appearance, and was medial and superior to this mass. The cyst was aspirated and filled with 25 per cent Hypaque@. Operative cholangiogram showed the radiopaque material filling this cyst-like structure and measuring 10 by 8 cm. (Figure 3.) The contrast material did not enter the duodenum. A 3 cm opening was made in the cyst. On digital exploration, nodular masses could be felt which did not seem to involve the mucosal surface of the cyst and which were thought to represent lymph nodes adjacent to the cyst along the hepatic artery. Roux-en-Y choledochocystojejunostomy was performed. Lymph node biopsy revealed a poorly TABLE

Choiedochai

ii

differentiated adenocarcinoma. The postoperative course was uneventful and the patient was discharged nine days after operation. The patient has resumed her previous work but continues to have mild back pain six months later.

Comment: This report is an example of congenital choledochal cyst associated with carcinoma. The incidence of carcinoma in congenital choledochal cyst has been reported rarely. However, analysils of previou.sly reported statistics has indicated that this association may have significantly greater incidence than previously recognized. The precise number of cases of congenital cystic dilatation of the biliary tree reported at the present time is difficult to determine accurately because of the large number of single case reports. AlonsoLej, Rever, and Passagno [6] collected 403 cases in 1959. In 1969, Lee et al [8] reported nine new cases and added eighty-eight from the world literature from 1957 to 1968. Hays et al [9] reported ten cases from the Children’s Hospital of Los Angeles during the last twenty years. Sporadic cases not included in the previous three reports amount to twenty-five and the present report adds four more cases. Therefore, at least a total of 539 cases have been reported in the literature. (Table I.)

Cyst Associated with Carcinoma

AgeW Author Irwin and Morison

[I]

Year

and Sex

1944

30,M

Ferraris, Navarro, and Escargel* Armanino [lo] Wilson, Morrissey, and McConnell (111 Dexter 1121 Fischer [13]

1946 1956

17,M 33,F

1957 1958

22,F 18,M

George and Maingot

1962

27,F

]I41 Kelly and Schlueter

1964

30,M

1151 Ashby [16]

1964

50,F

1967

21,M

Case 1

1967

65,F

Case 2

1967

28,F

1969

32, F

Thistlethwaite and Horwitz [17] MacFarlane and Glenn

Follow-Up Data

Pathology Choledochal cyst; squamous cell carcinoma in the cyst; cholecystogastrostomy performed Choledochal cyst: adenocarcinoma of liver and common bile duct: drainage of cyst performed Choledochal cyst; carcinoma of liver: autopsy performed Choiedochal cyst; Roux-en-Y loop performed in 1952; expioration in 1953 showed carcinomatosis but no primary lesion Choledochal cyst; carcinoma of hepatic duct; autopsy performed Choledochai cyst; excision of cyst performed with adenocarcinoma found in the cyst Choledochal cyst; adenocarcinoma in hepatic duct; Roux-en-Y loop performed Choledochai cyst: choledochocystoduodenostomy performed; carcinoma of head of pancreas found at second operation Choiedochal cyst; choledochocystoduodenostomy performed; biopsy showed adenocarcinoma in cyst wall; cyst excised at second operation Choledochai cyst; excision of cyst performed; carcinoma of hepatic duct found at second operation

Died Died Died Died 2 ma postoperatively Died Died 4 mo postoperatively Died 2 yr postoperatively Died 5 mo postoperatively Alive 11 mo postoperatively; patient in poor condition Died 5 mo postoperatively

WI

Present

report

* Quoted

512

by Kelly and Schlueter

Choiedochai cyst; excision of cyst performed; hepatic jejunostomy performed as second operation for carcinoma Choledochal cyst; Roux-en-Y loop performed; at second operation biopsy showed carcinoma Choledochai cyst; Roux-en-Y performed; biopsy showed carcinoma in lymph node

Died 23 mo postoperatively Died 10 days postoperatively Alive 4 mo postoperatively

1151.

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Congenital Dilatation of Biliary Tract

Figure 4. Case IV. lntrahepatic dilatation of the biliary system was detected by operative cholangiography.

Reports of twelve cases of carcinoma in congenital choledochal cysts have been found, in addition to the case reported herein. (Table II.) Of 7,000 cases of cancer registered at Northwestern University Medical Center excluding the skin, only ten cases of bile duct carcinoma have been found, which yield,s an incidence of 0.14 per cent. The incidence of carcinoma in association with congenital dilatation of the biliary system is 2.4 per cent, or approximately twenty times greater than the incidence of bile duct carcinoma in the general population. CASE IV. The patient, a thirty-five year old white man, was admitted for the first time to the Veterans’ Administration Research Hospital in September 1968 with fever preceded by chills, nausea, intermittent diarrhea, and general malaise of one week’s duration. His past history had been unremarkable until five weeks prior to admission, when a similar episode occurred. He was hospitalized elsewhere #and treated with antibiotics with gradual improvement. The patient had not been jaundiced and acholic stools and dark urine had not been noted. During the course of the present illness, he had a 22 pound weight loss. The patient .appeared chronically ill and was diaphoretic. The blood pressure was 110/70 mm Hg, pulse 104 per minute, and the temperature was 106’~ (41.1’~). On physical examination, there was slight tenderness to palpation in the epigastrium and right upper quadrant. Laboratory studies revealed the following: hemoglobin, 13.0 gm per 100 cc; white blood cell count, 17,000 per mm3 ; serum alkaline phosphatase, 32 King-Armstrong units; total bilirubin, 0.9 mg per 100 cc ; serum glutamic oxaloacetic transaminase and blood urea nitrogen levels were within normal limits.

Volume 121, May 1971

Figure 5. Case IV. Histologic study of the liver demonstrated dilated bile canaliculi and fibrosis of the portal space.

Bromsulphalein dye excretion showed a retention of 36.5 per 100 ml at the end of forty-five minutes. Stools were negative for ova and parasites. B,arium meal and intravenous pyelogram showed normal function. Oral cholecystography revealed poor visualization of the gallbladder with a single dose of Telepaque. During the following thirty-six hours, his abdominal symptoms became more pronounced and the total bilirubin rose to 2.5 mg per 100 ml. Abdominal exploration was performed with the preoperative diagnosis of acute cholecystitis. The gallbladder was found to be slightly distended with mild inflammatory changes. The liver was finely granular with patches of fibrosis. The common duct was dilated and choledochotomy performed.

figure 6. Case IV. Cholangiogram after choledochojejunostomy demonstrated persistence of intrahepatic dilatation of biliary tract.

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Lorenzo,

TABLE

Seed, and, Beal

Segmental

III

Dilatation

of the lntrahepatic

Biliary Tree

Segmental

Year and Sex

Author McWorther

Age at First Symptom

[27]

1924, F

Caroli et al 1201

1958,M

Caroli et al [19]

1958,M

Caroli et al [21]

l%O,M

Beau et al [22]

1960,M

Halasz, Jan, Mocsai [5]

1952.M

Eteve, Trad, and Caroli [23]

1963,F 4 yr (right upper

Arthur and Stewart [24] Gilbertini, Lodi, and Torricelli

1964

]251 Esquerra-Gomez and RiverosGamboa [26] Present report

Jaundice

Episodes of upper abdominal pain, fever, vomiting Episodes of abdominal pain, fever, vomiting Right upper quadrant pain, fever Cholangitis

Yes

Autopsy

No

Yes

No No

Confined to the left lobe Yes

idiopathic dilatation of common duct Left hepatic and common bile duct Choledochal cyst

Episodes fever

No

Yes

No (?)

23 yr (fight upper quadrant pain)

Epigastric and right upper quadrant pain

No

Yes

Choledochal cyst (common duct, 5 cm)

Abdominal

pain and fever

No

Yes

Idiopathic dilatation common duct

Abdominal

pain and jaundice

Yes

Yes

Choledochal

No

Yes

No

of abdominal

pain,

pain)

17 yr (abdominal pain, jaundice) 22 yr (abdominal pain)

Episodes of right upper quadrant pain

1%5,M

(3

Upper abdominal

1969,M

36 yr (upper abdominal pain and fever)

Abdominal ing

pain

pain, fever, vomit-

A small amount of gravel-like material was recovered. A Number 6 Bakes’ dilator was passed easily through the spincter of Oddi. The gallbladder was removed and the common duct drained with a T tube. Operative cholangiogram showed the radiopaque material filling multiple communicating segmental dilatations of the intrahepatic ductal system. (Figure 4.) Liver biopsy revealed marked infiltration with inflammatory cells, fibrosis of the portal spaces, and bile stasis. (Figure 5.) The lobular architecture of the liver was preserved. The postoperative course was febrile. The T tube drained an
5x4

Associated Extrahepatic Dilatation

lntrahepatic Biliary Tract Dilatation _

6 mo (abdominal pain) 2 yr (abdominal pain, nausea) 42 yr (right upper quadrant pain) 21 yr (febrile crisis) 2 yr (abdominal pain)

quadrant

1%5,M

Outstanding Clinical Symptoms

finding

Choledochal

cyst

of

cyst

No

Yes

Choledochal

No

Yes

Idiopathic dilatation of the common duct (20 cm)

cyst

daily. Postoperative cholangiogram showed again a dilated common duct and small amounts of radiopaque material passing into the duodenum. Because of the profuse drainage through the T tube, and clinical and chemical evidence of liver deterioration, a third operation was performed on January 20, 1969, nine weeks after the second operation. A Roux-en-Y end to end choledochojejunostomy was performed, and a number 15 F catheter was placed in the largest cystic dilatation. The catheter was brought out through the defunctionalized limb of the jejunum to facilitate free drainage of this cyst. The postoperative period was followed by slow recovery. The drainage diminished to the point that progressive clamping of the tube was tolerated. Antibiotic therapy was continued and the tube was opened when febrile episodes occurred. Postoperative cholangiograms showed a good functioning ,anastomosis (Figure 6), and the opaque material passed into the jejunum. The patient was discharged two months after the third operation. He gained 15 pounds and was free of jaundice. During the eight month follow-up period, the patient had sporadic #attacks of cholangitis. The patient’s weight has remained about the same with minimal fluctuation. He was admitted to the hospital in September 1969 for evaluation of the hepatobiliary system. The patient had been complaining of easy fatiga-

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Congenital

TABLE III

-__-_

of Biliary

Tract

(Continued)

Initial Operation[s]

Excision of cyst Hepaticoduodenostomy Cholecystostomy Cholecystectomy (1953) Choledochoduodenostomy Cholecystectomy Sphincterotomy Cholecystostomy

(1956)

Choledochotomy Removal, small calculi T-tube drainage Cholecystectomy Choledochotomy T-tube drainage Choledochocystoduodenostomy Choledochotomy; arterial hepatic

T tube drainage; neurectomy

1. Cholecystectomy, T tube drainage 2. Sphincterotomy

Postoperative Cholangitis

...

No

No

13 yr

Yes

42 yr 46 yr 59 yr

Yes

Choledochojejunostomy Roux-en-Y (7 mo) Left hepatic lobectomy

Yes

Choledochojejunostomy

5 yr

Yes

26 yr

Yes

Cystojejunostomy (intrahepatic cyst); cholangiomanometry (38 cm H20) at 1 mo Choledochoduodenostomy

Definitive Operation

Asymptomatic (died 15 yr later of an unrelated disease) Occasional crisis of cholangitis (10 mo)

Peri-

choledochotomy,

21 yr

Yes

Choledochojejunostomy Roux-en-Y (1 mo)

34 yr

Yes

Excision of cyst; hepaticojejunostomy; Roux-en-Y No

(3 mo)

27 yr

...

31 yr

?

Choledochojejunostomy(sideto side) (2 mo)

36 yr

Yes

Choledochojejunostomy Roux-en-Y (2 mo)

(2% yr) Asymptomatic

(2jh yr)

Died (postoperative) Died (4 mo postoperatively sepsis) Recurrent gitis

episodes

No follow-up

Asymptomatic

of

of cholan-

study

(8 mo)

(19yr) T tube removed (7 wk postoperatively); no further follow-up Asymptomatic (7 mo)

Recurrent episodes gitis (8 mo)

of cholan-

36 yr

bility on light physical work. Laboratory studies revealed serum alkaline phosphatase of 150 King-Armstrong units, serum glutamic oxaloacetic transaminase of 56 units, and total protein of 8.3 gm per cent with 3.2 per cent .albumin. Total bilirubin was 0.7 mg per cent. Blood urea nitrogen was 22 mg per cent. Bromsulfalein dye excretion showed a retention of 24/100 at the end of forty-five minutes. Intravenous pyelogram showed normal findings. Tube cholangiogram revealed significant enlargement of the cystic dilatations and good functioning of the anastomosis. The choledochus measured 20 mm in diameter.

Comment: This report is presented as an example of a rare variation of congenital cystic dilatation which involves the intrahepatic biliary tree. In 1958 Caroli et al [19] reported the first documented case and within the next two years published two more [20,21]. Since then six case reports have been published [5,22-261. In 1964, Arthur and Stewart [24] reported a similar case and modified the original classification proposed by Alonso-Lej et al [6] by adding a fourth type of anomaly: multiple biliary cysts. The present case seems to be the eleventh of this type reported. The clinical features are summarized in Table III.

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Current Status (Length of Follow-Up)

Age at Surgery

(3 yr)

Cholecystectomy

Volume

Dilatation

1971

Comments Sex and Age. According to the classification of Alonso-Lej [6] types I, II, and III of this anomaly show a preponderance in the female sex, in the ratio of 4 to 1; of the eleven cases of type IV analyzed here, eight patients were male. However, the number of eases is not sufficient for statistical conclusion. The onset of symptoms in nine patients occurred below the age of twenty-five years. Etiology. The congenital origin of cystic biliary dilatation is widely accepted. Of numerous theories that have been proposed to explain these anomalies, most favor that of Yotsuyagani [28], according to whom th’ere is an unequal proliferation of the epithelial cells of the primitive choledochus during the embryonic stage when this structure is a solid cord. This proliferation is more prominent in the upper segment than in the lower segment. When recanalization occurs at a later stage, the upper segment is abnormally wide with a relatively narrow distal end. Based on this theory, it is possible that the same series of events can occur in multifocal areas of the intrahepatic ductal system. However, the existence of a solid

515

Lorenzo,

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stage has not been demonstrated in embryologic preparations. Pathology. The pathologic features of the congenital cystic dilatation of the extrahepatic ductal system have been described in previous reports. Communicating segmental dilatation of the intrahepatic bile ducts provides an accurate description of this anatomic lesion. The dilatations vary in size and shape and involve both lobes of the liver. The communication is established through relatively narrow ductal structures. In the present review, nine of the eleven cases had associated idiopathic dilatation of the extrahepatic ductal system. The lesion was confined to the left lobe of the liver in one case reported by Caroli et al [19]. The microscopic findings are related to the changes occurring in the portal spaces after episodes of cholangiti8s. Infiltration of inflammatory cells, various degrees of fibrosis, reduplication of bile ducts, and cholestasis in the portal spaces are the most consistent findings. The lobular architecture of the liver is preserved. Symptoms. The classic triad of abdominal pain, jaundice, and abdominal mass is reported in about two-thirds of cases with choledochal cysts. In the present report, an abdominal mass and jaundice were present in one case whereas two others had symptoms similar to those produced by inflammatory disease of the gallbladder. Episodes of upper abdominal pain and fever have been the most prominent symptoms in the group of patients who had cystic dilatation of the intrahepatic biliary tree. These episodes were relieved by variable periods of comparable well being. Only two of the eleven patients whose cases were reviewed gave the history of jaundice. Treatment. The surgical treatment of congenital dilatation of the biliary tract varies according to the location and type of dilatation. Two different types of procedures are advocated for the treatment of the congenital choledochal cyst. Internal drainage procedures are the most conservative approach to this problem. Choledochocystoduodenostomy and Roux-en-Y choledochocystojejunostomy are the two most frequent procedures. The anastomosis should be made on the dependent surface of the cyst. Roux-en-Y choledochocystoj ej unostomy has the advantage of diverting the intestinal contents away from the area of anastomosis, thus decreasing the incidence of cholangiti.s. This procedure is easy, is associated with a 10~ mortality, and provides the most satisfactory results. In recent years, more radical surgical procedures have been advocated. Those favoring ex-

516

cision of the cyst report a decreased mortality rate with this procedure and significantly less morbidity than with the internal drainage technic. The mortality rate for all types of operative procedures has decreased from 83 per cent in a collected series of fifty-three cases reported by Lange [29] in 1927 to 23 per cent in fifty-two cases operated upon and reported by Tsardakas and Robnett [30] in 1956, and 12 per cent reported by Alonso-Lej et al [6] in ninety-one surgically treated patients. In a recent collected series of ninety-one cases, Lee et al [8] reported a mortality rate of 5.2 per cent. However, the mortality rate of patients undergoing excision of the cyst has decreased from 40 per cent reported by Hatano and Imoto [31] in 1954 and 29 per cent in seventeen patients treated by surgical excision of the cyst as reparted by Tsardakas, to 15 per cent in the collected series reported by Alonso-Lej [6] in which thirteen of the total of ninety-one patients surgically treated underwent complete exci.sion of the cyst. However, the number of those treated with resection of the cyst is still small to be conclusive. Congenital diverticulum is best treated by excision of the diverticulum without interference of the bile duct. In our present report, choledochocystojejunostomy with Roux-en-Y anastomosis was performed in two patients and one patient has been free of symptoms for two and a half years. The second had carcinoma associated with this anomaly and continues to have pain, but is without evidence of cholangitis. In a third patient, choledochocystoduodenostamy was performed, and he remains free of symptoms two years later. The surgical treatment of the congenital dilatation of the intrahepatic ductal system (case IV) presents a difficult problem. Surgical correction of these anomalies usually is not feasible unless the disease is confined to an area where resection can be performed. Of the eleven patients who have been recorded, nine had an associated idiopathic dilatation of the extrahepatic ductal system, either diffusely or as a choledochal cyst. This extrahepatic biliary anomaly seems to be an important factor in the pathogenesis of cholangitis, interfering with the praper passage of bile into the duodenum. The goal of operation in this group of patients is to improve the hepatic parenchymal function by treating the cholangitis with adequate internal drainage of the di’seased cammon duct. Four of the eleven patients with recorded cases have been asymptomatic for a period of seven

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Congenital

months to fifteen years. Two of these four patients had excision of a choledochal cyst and internal drainage. A third had left hepatic lobectomy and in the fourth, choledochocystojejunostomy was performed as a definitive procedure. Two patients died, one during the immediate postoperative course and the second of sewis four months after operation. Recurrent episodes of cholangitis have been a major complication in three patients in whom internal drainage was performed. Follow-up data were not available in two patients.

12.

13.

14.

15. 16. 17.

Summary

Four ‘cases of congenital cystic dilatation of the biliary tract ‘are reported. The association of carcinoma and congenital dilatation of the extrahepatic ductal ‘system is discussed ,and found to be higher than previously recognized. Eleven cases of congenital dilatation of the intrahepatic ductal system collected from the world literature are analyzed. The etiology, clinical features, and treatment of these types of anomalies are reviewed. References 1. Irwin ST, Morison JE: Congenital cyst of the common bile duct containing stones and undergoing cancerous change. Brit J Surg 32: 319, 1944. 2. Cinelli AP: Dilatation quisteca idiopatica del choledoco su diagnostic0 mediante la colangiografia operatoria. Bol Sot Cir (Cordoba) 5: 264, 1944. 3. Serfas LS, Lyter CS: Choledochal cyst with a report of an intraduodenal choledochal cyst. Amer J Surg 93: 979, 1957. 4. Paulino F: Dilatacao congenita do coledoco. Rev. Brasil Cir 31: 402, 1956. 5. Halasz J, Jan H, Mocsai L: Gallensteinbildung in den kongenitalen intrahepatischen Hohlraumen. Zbl Chir 87: 547, 1962. 6. Alonso-Lej F, Rever WB Jr, Passagno DJ: Congenital choledochal cyst with a report of two and analysis of 94 cases. Surg Gynec Obstet 108: 1,1959. 7. Strode JE: Congenital cystic dilatation of the common bile duct. Int Surg 45: 508, 1966. 8. Lee SS, Min PC, Kim GS, Hong PW: Choledochal cyst: a report of nine cases and review of the literature. Arch Surg 99: 19, 1969. 9. Hays DM, Goodman GN, Snyder WH, Wolley MN: Congenital cystic dilatation of the common bile duct. Arch Surg 98: 457, 1969. 10. Armanino LP: Idiopathic dilatation of the common bile duct with co-existent primary hepatic carcinoma: report of a case. Ann Int Med 24: 714, 1946. 11. Wilson FD, Morrissey GE, McConnell RW: Bi.le duct cyst

Volume 121, May 1971

18. 19.

20.

21.

22.

23.

24. 25.

26.

27.

28.

29. 30. 31.

Dilatation

of Biliary

Tract

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