Surgical Treatment of Congenital Dilatation of the Bile Duct With Special Reference to Late Complications After Total Excisional Operation ByRyoji
Ohi, Seiichi Yaoita,
Takamichi
Kamiyama,
Mohamed
Ibrahim,
Yutaka Hayashi,
and Tsuneo Chiba
Table 1. Operative
Procedures
Sendai, Japan 0 The surgical treatment of 100 cases with congenital dilatation of bile duct with special reference to late complications was analyzed. There were no deaths nor occurrences of malignancy. Among 91 patients who had undergone the standard operation, namely total excision of the dilated exhahepatic bile duct and reconstruction after Roux-en-Y hepaticojejunostomy, there were one early complication (pancreatic juice leakage) and five late complications (four intrahepatic gallstones and one liver abscess). The cause of intrahepatic gallstone formation after a total excisional operation was attributed to the remaining intrahepatic bile duct dilatation and the stenosis located between the intrahepatic bile duct dilatation and the common hepatic duct. Accordingly, these results support the total excisional procedure for this condition: however, with regard to the cases associated with cystic dilatation of intrahepatic bile ducts, completely free bile drainage from the dilated intrahepatic biliary system should be performed at the radical operation. 0 1990 by W.B. Saunders Company. INDEX WORDS: dochal cyst.
Congenital
dilatation
of bile duct: chole-
C one of the most important surgical conditions affecting the biliary tract in infants and children. Total ONGENITAL
DILATATION
of the bile duct is
excision of the dilated common bile duct and the establishment of free bile drainage from the liver to the intestine has been widely accepted as a standard surgical procedure to replace the internal drainage, even in infants and children.iq5 The purpose of this report is to analyze early and late complications in the treatment of this condition, especially the late complications after the total excisional procedure. MATERIALS
AND
METHODS
One hundred cases of congenital dilatation of bile duct that were operated on at Tohoku University Hospital from 1965 through September 1988 were analyzed. Twenty were male and 80 were female. Of 100 patients ranging in age from 7 days to 23 years, 61% were younger than 4 years. Internal drainage operations were performed in five cases before 1962. Thereafter total excision of dilated common bile duct followed by reconstruction was performed in all patients. The reconstruction was in the form of hepaticoduodenostomy in one case, hepaticojejunostomy in Roux-en-Y fashion in 84, end-to-end anastomosis of the hepatic duct and the common bile duct in two (this procedure was employed before introduction of the concept of anomalous arrangement of the pancreatic duct and the common bile duct,6 and Roux-en-Y hepatic portojejunostomy in one. Seven other patients underwent total excision of shrunken cysts 3 to 13 years after the internal drainage operation at other hospitals (Table 1). Early and late complications were analyzed in these Journa/
of Pediatric Surgery, Vol 25, No 6 (June), 1990: pp 613-617
No. of Cases Internal drainage operation
5
Cyst duodenostomy
1
Cyst jejunostomy, Roux-en-Y
4
Total excision and reconstruction Hepaticoduodenostomy Hepaticojejunostomy, Roux-en-Y
88 1 84
Hepaticocholedcchostomy
2
Hepatic portojejunostomy, Roux-en-Y
1
Recperation’
7
Total
100
*Excision of shrunken cyst and hepaticojejunostomy in Roux-en-Y fashion after internal drainage operation at other hospitals.
The follow-up periods of these patients range from 7 months to 33 years, with a mean of 9.8 years after the corrective operations. Because the cystic dilatation of the intrahepatic bile ducts seemed to be participating factors of the sequelae, we reanalyzed the intrahepatic bile duct dilatation in 81 patients of whom intrahepatic bile ducts were able to be evaluated by operative cholangiogram or endoscopic retrograde cholangiopancreatography. patients.
RESULTS
There was no mortality nor occurrence of malignancy in this series. Early and late complications that developed in these patients are listed in Table 2. About 10 years after cystduodenostomy, a patient developed severe cholangitis. Relaparotomy showed an anastomotic stricture and multiple gallstones in the shrunken extrahepatic bile duct cyst. The anastomotic stricture required a reoperation in one patient who had an excision of a localized cyst followed by end-to-end anastomosis of the extrahepatic bile duct one month after the initial operation. In another patient who had undergone total excisional operation, a temporary pancreatic juice leakage was noted due to incomplete
From the Division of Pediatric Surgery, Tohoku University School of Medicine, Sendai. Japan. Date accepted: April 19, 1989. Address reprint requests to Ryoji Ohi, MD, Division of Pediatric Surgery, Tohuku University School of Medicine. I-1. Seiryomachi. Aoba-ku. Sendai 980. Japan. 0 1990 by W.B. Saunders Company. 0022-3468/90/2506-0008$03.00/0 613
614
OHI ET AL Table 2. Early and Late Complications After Surgery Procedure
No. of Cases
Complications
CYD
1
1 (cholangitis + stricture + gall-
CyJ, Roux-en-Y
4
0
HD
1
0
HC
2
stones)
HJ, Roux-en-Y
1 (anastomotic stricture)
84 + 7
5 (1, pancreatic juice leak; 4, intrahepatic gallstones; 1, liver abscess)
HPJ, Roux-en-Y
1
0
Abbreviations: CyD, cyst duodenostomy: CyJ, cyst jejunostomy; HD. hepaticoduodenostomy; HC, hepaticocholedochostomy; HJ, hepaticojejunostomy; HPJ, hepaticportojejunostomy.
closure of the lowest portion of the common bile duct, but the leakage healed spontaneously within 3 weeks after the operation. Four other patients in this group developed intrahepatic gallstones as a late complication (Table.3). These four patients had undergone total excisional operations at the ages of 1, 7, 10, and 5 years, and had recurrent symptoms 15, 7, 12, and 14 years, respectively, after the initial operation. Cystic dilatation of the intrahepatic bile ducts were demonstrated at the initial operation but were left untreated. Figures 1 and 2 show the extrahepatic and intrahepatic bile duct configuration in case 1. Cystic dilatation was seen in the left and right, especially up to the peripheral portion of the left intrahepatic bile duct. Three other patients also had intrahepatic bile duct configuration similar to this picture. The intrahepatic gallstones were detected by ultrasound and computed tomography (CT) scan in all cases, and percutaneous transhepatic cholangiography (PTC) in cases 1 and 3. CT scan in case 4 and PTC in case 3 clearly demonstrated the intrahepatic gallstones (Figs 3 and 4). They underwent reoperations at the ages of 16, 14,22, and 19 years, respectively (Table 4). The gallstones were present in the left intrahepatic bile duct in three cases, and stones were packed diffusely in the bilateral intrahepatic branches in case 4. Other findings included anastomotic stricture at the site of hepaticojejunostomy in case 1 and liver abscess of the lateral segment resulted from suppurative cholangitis of the left intrahepatic biIe duct in case 3. These stones were successfully removed under direct exposure using
Fig 1. A direct cholangiogram of case 1. Huge cystic dilatation of extrahepatib bile duct was demonstrated.
biliary fiberscope, but lateral segmentectomy was required in cases 3 and 4. Of 81 patients subjected to the evaluation of the intrahepatic bile duct, 61 patients (75%) were associated with dilatation of the intrahepatic bile duct (Table 5). The form of extrahepatic bile duct dilatation was cystic in 45 cases and fusiform in 16. Of 45 patients with cystic dilatation of the common bile duct, intrahepatic duct dilatation was cystic in 27 and fusiform in the remaining 18. On the other hand, all 16 cases with fusiform dilatation of the common bile duct had fusiform dilatation in the intrahepatic bile duct (Table 6). DISCUSSION
With regard to the operative procedures for congenital dilatation of bile duct, it has been shown that the incidence of late complications such as suppurative cholangitis, anastomotic stricture, or malignant change
Table 3. lntrahepatic Gallstone Formation After Total Excisional Operation
NO.
Age at 1st OD Iyears1
Interval
RecurrentSymptoms
of oc
lyeari
Pain
Fever
Jaundice
1
1
15
+
2 3
7 10
7 12
+ +
+ +
Yes (cystic) Yes (cystic)
4
5
14
+
+
Yes (cystic)
Abbreviation: Op, operation.
+
lntrahepaticBile Duct Dilatation Yes (cystic)
TOTAL EXCISION OF CONGENITAL BILE DUCT CYST
A selective cholangiogram of the lntrahepatic bile duct Fig 2. of cese 1. Cystic dilatation of the bilateral intrahepatic bile ducts, especlelly up to the peripheral portion of the left intrahepatic bile duct was noted.
of the remaining extrahepatic biliary cyst, was very high in patients who have undergone internal drainage operation. Therefore, total excision of dilated extrahepatic bile duct and the establishment of a free bile drainage from the liver to the intestine has become widely accepted as a standard surgical procedure replacing the internal drainage operation even for infants and children. The introduction of the concept of anomalous arrangement of the pancreatic duct and the common bile duct6 also demonstrated the advantage of the total excisional procedure. The results of this
Fig 3. Computed tomography scan in case 4. Multiple intrahspatic gellstones were noted (arrow).
615
Fig 4. A percutaneous transhepatic cholangiogram of case 3. lntrahepatic gellstons was demonstrated farrow).
experience using the standard excisional operation further support the recent trend of the surgical treatment of this condition. Although there have been a considerable number of publications7S’2 on the late complications after internal drainage operation for this disease, there have been very few reports of those after the total excisional operation. Todani et all3 reported three patients who have required a reoperation due to an anastomotic stricture with intrahepatic involvement. We also encountered late complications such as intrahepatic gallstone formation and liver abscess in patients even after the total excisional operation. The cause of intrahepatic gallstone formation was attributed to the anastomotic stricture in case 1 and to the underlying relative stenosis located between the extrahepatic bile duct and the dilated intrahepatic ducts in the other cases. Of 8 1 patients subjected to the evaluation of the intrahepatic bile duct, 61 patients (75%) were associated with dilatation of the intrahepatic bile duct. Intrahepatic duct dilatation was cystic in 27 and fusiform in the remaining 18 in patients with cystic dilatation of the common bile duct. On the other hand, all 16 cases with fusiform dilatation of the common bile duct had fusiform dilatation in the intrahepatic bile duct. Accordingly, the fusiform dilatation in the intrahepatic bile duct is thought to be secondary, and the cystic dilatation as the primary one.14 Many authors S” showed the intrahepatic bile duct dilatation in patients with congenital dilatation of bile duct. There exist several reports18‘20 on carcinoma of the intrahepatic ducts in patients undergoing extrahepatic cyst excision followed by biliary reconstruction. Todani et alI3 emphasized that bile stagnation in the intrahepatic ducts is possibly responsible for the development of carcinoma. Based on this, they recom-
616
OHI ET AL
Table 4. lntrahepatic
Case No.
Age at Rew (wars)
Sire of Gallstone
1
16
Left
2
14
3
22
4
19
Bilateral
Gallstone Formation
after Total Excisional Operation
Other Findings
OperariveProcedure
Left
Anastomotic stricture -
Removal of stone, reanastomosis Removal of stone, reanastomosis
Left
Liver abscess
Removal of stone, lateral segmentectomy Removal of stone, reanastomosis, lateral seg-
-
mentectomy
Abbreviation: Reop, reoperation.
Table 5. lntrahepatic
Bile Duct Dilatation Dilatation
(IHBDI in Congenital
\
of Bile Duct
relative
stenosis
81
Total cases examined Dilatation of IHBD Yes
61 (75%)
No
20 (25%)
Table 6. lntrahepatic
Bile Duct Dilatation
Dilatation ExtrahepaticBileDuct Cystic Fusiform
45 16
(IHBD) in Congenital
of Bile Duct (n = 61) lntrahepaticBileDuct Cystic
27
Fusiform
18
Cystic Fusiform
0 16
mended hepaticoenterostomy at the porta hepatis with wide anastomotic stoma to prevent anastomotic stricture. We have adopted this surgical procedure for intrahepatic cystic dilatations (Fig 5). In order to achieve a free drainage from the inside of the liver, we recommend the removal of not only the extrahepatic bile duct cyst, but also the relative stenosis between the common hepatic duct and the intrahepatic bile duct cyst. Extremely high anastomosis beyond the relative stenosis or plasty of the intrahepatic bile duct should be performed at the radical operation. Cystic dilatation of the peripheral portion of the intrahepatic bile duct in adults, occasionally managed by lateral segmentectomy of the liver or intrahepatic may develop late complications cystenterostomy, 17P2’*22 such as those noted in these cases. On the other hand, in infants and children having same condition, the
~~ :~~ :..:..
‘I ,,
.... ....,::.:;,.,.:.,, :.: ..:ji:.: ..... .. ((, ,;;t:;‘i;
Extended Anastomosis
iIiiiii:i:iiiiiiil~~~~~::~ ......... .......:.:... ,::j::w
Plasty of Stenotic Portion
Fig 5. Hepaticojejunostomy in a case with intrahepatic bile duct dilatation. Extremely high anastomosis beyond the relative stenosis or plasty of the intrahepatic bile duct should be performed.
incidence of late complications is less than in adults, so excessive surgical intervention should be avoided and surgical approach for them is not always needed at the time of the initial operation. However, these children must be followed carefully for a long time.
REFERENCES 1. Kasai M, Asakura Y, Taira Y: Surgical treatment of choledochal cyst. Ann Surg 172:844-85 1, 1970 2. Kasai M, Suzuki H, Ohi R: Operative procedures for choledochal cyst. Jpn J Surg 7:147-150,1977 3. Ishida M, Tsuchida Y, Saito S, et al: Primary excision of choledochal cyst. Surgery 68:884-888, 1970 4. Spitz L: Choledochal cyst. Surg Gynecol Obstet 147:444-452, 1978 5. Lilly JR: The surgical treatment of choledochal cyst. Surg Gynecol Obstet 149:36-42, 1979 6. Babbitt DP, Starshak RJ, Clemett AR: Choledochal cyst: A concept of etiology. AJR 11957-62, 1973
7. Powell CS, Swayers JL, Reynold VH: Management of adult choledochal cyst. Ann Surg 193:666-676, 1981 8. Todani T, Watanabe Y, Toki A, et al: Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 164:61-64, 1987 9. Flanigan DP: Biliary cysts. Ann Surg 182:635-643, 1975 10. Nakamura S, Kimura S, Ishidoya T, et al: Re-operation of the choledochal cyst. Z Kinderchir 22:41-50, 1977 11. Klotz D, Cohn BD, Kottmeier PK: Choledochal cysts: Diagnosis and therapeutic problems. J Pediatr Surg 8:271-283, 1973 12. Kim SH: Choledochal cyst: Survey by the Surgical Section of
TOTAL EXCISION OF CONGENITAL BILE DUCT CYST
the American Academy of Pediatrics. J Pediatr Surg 16:402-407, 1981 13. Todani T, Watanabe Y, Toki A, et al: Reoperation for congenital choledochal cyst. Ann Surg 207:142-147, 1988 14. Ohi R, Koike N, Matsumoto Y, et al: Changes of intrahepatic bile duct dilatation after surgery of congenital dilatation of the bile duct. J Pediatr Surg 20:138-142, 1985
15. McWhorter GL: Congenital cystic dilatation of the bile and pancreatic ducts. Arch Surg 38:397-411, 1939 16. Tsuchida Y, Ishida M: Dilatation of the intrahepatic bile ducts in congenital cystic dilatation of the common bile duct. Surgery69:776-781,197l 17. Todani T, Narusue M, Watanabe Y, et al: Management of
617
congenital choledochal cyst with intrahepatic involvement. Ann Surg 187:272-280,1977 18. Thistlethwaite JR, Horwitz A: Choledochal cyst followed by carcinoma of the hepatic duct. South Med J 60:872-874, 1967 19. Gallagher PJ, Millis RR, Mitchinson MJ: Congenital dilatation of the intrahepatic bile ducts with cholangiocarcinoma. J Clin Path01 25:804-808,1972 20. Chaudhuri PK, Chaudhuri B, Schuler JJ, et al: Carcinoma associated with congenital dilatation of bile ducts. Arch Surg 117:1349-1351, 1982 21. Engle J, Salmon PA: Multiple choledochal cysts. Arch Surg 88:345-349, 1964 22. Warren KW, Kune GA, Hardy KJ: Biliary duct cysts. Surg Clin North Am 48:567-577, 1968
Ohi, Seiichi Yaoita,
Takamichi
Kamiyama,
Mohamed
Ibrahim,
Yutaka Hayashi,
and Tsuneo Chiba
Table 1. Operative
Procedures
Sendai, Japan 0 The surgical treatment of 100 cases with congenital dilatation of bile duct with special reference to late complications was analyzed. There were no deaths nor occurrences of malignancy. Among 91 patients who had undergone the standard operation, namely total excision of the dilated exhahepatic bile duct and reconstruction after Roux-en-Y hepaticojejunostomy, there were one early complication (pancreatic juice leakage) and five late complications (four intrahepatic gallstones and one liver abscess). The cause of intrahepatic gallstone formation after a total excisional operation was attributed to the remaining intrahepatic bile duct dilatation and the stenosis located between the intrahepatic bile duct dilatation and the common hepatic duct. Accordingly, these results support the total excisional procedure for this condition: however, with regard to the cases associated with cystic dilatation of intrahepatic bile ducts, completely free bile drainage from the dilated intrahepatic biliary system should be performed at the radical operation. 0 1990 by W.B. Saunders Company. INDEX WORDS: dochal cyst.
Congenital
dilatation
of bile duct: chole-
C one of the most important surgical conditions affecting the biliary tract in infants and children. Total ONGENITAL
DILATATION
of the bile duct is
excision of the dilated common bile duct and the establishment of free bile drainage from the liver to the intestine has been widely accepted as a standard surgical procedure to replace the internal drainage, even in infants and children.iq5 The purpose of this report is to analyze early and late complications in the treatment of this condition, especially the late complications after the total excisional procedure. MATERIALS
AND
METHODS
One hundred cases of congenital dilatation of bile duct that were operated on at Tohoku University Hospital from 1965 through September 1988 were analyzed. Twenty were male and 80 were female. Of 100 patients ranging in age from 7 days to 23 years, 61% were younger than 4 years. Internal drainage operations were performed in five cases before 1962. Thereafter total excision of dilated common bile duct followed by reconstruction was performed in all patients. The reconstruction was in the form of hepaticoduodenostomy in one case, hepaticojejunostomy in Roux-en-Y fashion in 84, end-to-end anastomosis of the hepatic duct and the common bile duct in two (this procedure was employed before introduction of the concept of anomalous arrangement of the pancreatic duct and the common bile duct,6 and Roux-en-Y hepatic portojejunostomy in one. Seven other patients underwent total excision of shrunken cysts 3 to 13 years after the internal drainage operation at other hospitals (Table 1). Early and late complications were analyzed in these Journa/
of Pediatric Surgery, Vol 25, No 6 (June), 1990: pp 613-617
No. of Cases Internal drainage operation
5
Cyst duodenostomy
1
Cyst jejunostomy, Roux-en-Y
4
Total excision and reconstruction Hepaticoduodenostomy Hepaticojejunostomy, Roux-en-Y
88 1 84
Hepaticocholedcchostomy
2
Hepatic portojejunostomy, Roux-en-Y
1
Recperation’
7
Total
100
*Excision of shrunken cyst and hepaticojejunostomy in Roux-en-Y fashion after internal drainage operation at other hospitals.
The follow-up periods of these patients range from 7 months to 33 years, with a mean of 9.8 years after the corrective operations. Because the cystic dilatation of the intrahepatic bile ducts seemed to be participating factors of the sequelae, we reanalyzed the intrahepatic bile duct dilatation in 81 patients of whom intrahepatic bile ducts were able to be evaluated by operative cholangiogram or endoscopic retrograde cholangiopancreatography. patients.
RESULTS
There was no mortality nor occurrence of malignancy in this series. Early and late complications that developed in these patients are listed in Table 2. About 10 years after cystduodenostomy, a patient developed severe cholangitis. Relaparotomy showed an anastomotic stricture and multiple gallstones in the shrunken extrahepatic bile duct cyst. The anastomotic stricture required a reoperation in one patient who had an excision of a localized cyst followed by end-to-end anastomosis of the extrahepatic bile duct one month after the initial operation. In another patient who had undergone total excisional operation, a temporary pancreatic juice leakage was noted due to incomplete
From the Division of Pediatric Surgery, Tohoku University School of Medicine, Sendai. Japan. Date accepted: April 19, 1989. Address reprint requests to Ryoji Ohi, MD, Division of Pediatric Surgery, Tohuku University School of Medicine. I-1. Seiryomachi. Aoba-ku. Sendai 980. Japan. 0 1990 by W.B. Saunders Company. 0022-3468/90/2506-0008$03.00/0 613
614
OHI ET AL Table 2. Early and Late Complications After Surgery Procedure
No. of Cases
Complications
CYD
1
1 (cholangitis + stricture + gall-
CyJ, Roux-en-Y
4
0
HD
1
0
HC
2
stones)
HJ, Roux-en-Y
1 (anastomotic stricture)
84 + 7
5 (1, pancreatic juice leak; 4, intrahepatic gallstones; 1, liver abscess)
HPJ, Roux-en-Y
1
0
Abbreviations: CyD, cyst duodenostomy: CyJ, cyst jejunostomy; HD. hepaticoduodenostomy; HC, hepaticocholedochostomy; HJ, hepaticojejunostomy; HPJ, hepaticportojejunostomy.
closure of the lowest portion of the common bile duct, but the leakage healed spontaneously within 3 weeks after the operation. Four other patients in this group developed intrahepatic gallstones as a late complication (Table.3). These four patients had undergone total excisional operations at the ages of 1, 7, 10, and 5 years, and had recurrent symptoms 15, 7, 12, and 14 years, respectively, after the initial operation. Cystic dilatation of the intrahepatic bile ducts were demonstrated at the initial operation but were left untreated. Figures 1 and 2 show the extrahepatic and intrahepatic bile duct configuration in case 1. Cystic dilatation was seen in the left and right, especially up to the peripheral portion of the left intrahepatic bile duct. Three other patients also had intrahepatic bile duct configuration similar to this picture. The intrahepatic gallstones were detected by ultrasound and computed tomography (CT) scan in all cases, and percutaneous transhepatic cholangiography (PTC) in cases 1 and 3. CT scan in case 4 and PTC in case 3 clearly demonstrated the intrahepatic gallstones (Figs 3 and 4). They underwent reoperations at the ages of 16, 14,22, and 19 years, respectively (Table 4). The gallstones were present in the left intrahepatic bile duct in three cases, and stones were packed diffusely in the bilateral intrahepatic branches in case 4. Other findings included anastomotic stricture at the site of hepaticojejunostomy in case 1 and liver abscess of the lateral segment resulted from suppurative cholangitis of the left intrahepatic biIe duct in case 3. These stones were successfully removed under direct exposure using
Fig 1. A direct cholangiogram of case 1. Huge cystic dilatation of extrahepatib bile duct was demonstrated.
biliary fiberscope, but lateral segmentectomy was required in cases 3 and 4. Of 81 patients subjected to the evaluation of the intrahepatic bile duct, 61 patients (75%) were associated with dilatation of the intrahepatic bile duct (Table 5). The form of extrahepatic bile duct dilatation was cystic in 45 cases and fusiform in 16. Of 45 patients with cystic dilatation of the common bile duct, intrahepatic duct dilatation was cystic in 27 and fusiform in the remaining 18. On the other hand, all 16 cases with fusiform dilatation of the common bile duct had fusiform dilatation in the intrahepatic bile duct (Table 6). DISCUSSION
With regard to the operative procedures for congenital dilatation of bile duct, it has been shown that the incidence of late complications such as suppurative cholangitis, anastomotic stricture, or malignant change
Table 3. lntrahepatic Gallstone Formation After Total Excisional Operation
NO.
Age at 1st OD Iyears1
Interval
RecurrentSymptoms
of oc
lyeari
Pain
Fever
Jaundice
1
1
15
+
2 3
7 10
7 12
+ +
+ +
Yes (cystic) Yes (cystic)
4
5
14
+
+
Yes (cystic)
Abbreviation: Op, operation.
+
lntrahepaticBile Duct Dilatation Yes (cystic)
TOTAL EXCISION OF CONGENITAL BILE DUCT CYST
A selective cholangiogram of the lntrahepatic bile duct Fig 2. of cese 1. Cystic dilatation of the bilateral intrahepatic bile ducts, especlelly up to the peripheral portion of the left intrahepatic bile duct was noted.
of the remaining extrahepatic biliary cyst, was very high in patients who have undergone internal drainage operation. Therefore, total excision of dilated extrahepatic bile duct and the establishment of a free bile drainage from the liver to the intestine has become widely accepted as a standard surgical procedure replacing the internal drainage operation even for infants and children. The introduction of the concept of anomalous arrangement of the pancreatic duct and the common bile duct6 also demonstrated the advantage of the total excisional procedure. The results of this
Fig 3. Computed tomography scan in case 4. Multiple intrahspatic gellstones were noted (arrow).
615
Fig 4. A percutaneous transhepatic cholangiogram of case 3. lntrahepatic gellstons was demonstrated farrow).
experience using the standard excisional operation further support the recent trend of the surgical treatment of this condition. Although there have been a considerable number of publications7S’2 on the late complications after internal drainage operation for this disease, there have been very few reports of those after the total excisional operation. Todani et all3 reported three patients who have required a reoperation due to an anastomotic stricture with intrahepatic involvement. We also encountered late complications such as intrahepatic gallstone formation and liver abscess in patients even after the total excisional operation. The cause of intrahepatic gallstone formation was attributed to the anastomotic stricture in case 1 and to the underlying relative stenosis located between the extrahepatic bile duct and the dilated intrahepatic ducts in the other cases. Of 8 1 patients subjected to the evaluation of the intrahepatic bile duct, 61 patients (75%) were associated with dilatation of the intrahepatic bile duct. Intrahepatic duct dilatation was cystic in 27 and fusiform in the remaining 18 in patients with cystic dilatation of the common bile duct. On the other hand, all 16 cases with fusiform dilatation of the common bile duct had fusiform dilatation in the intrahepatic bile duct. Accordingly, the fusiform dilatation in the intrahepatic bile duct is thought to be secondary, and the cystic dilatation as the primary one.14 Many authors S” showed the intrahepatic bile duct dilatation in patients with congenital dilatation of bile duct. There exist several reports18‘20 on carcinoma of the intrahepatic ducts in patients undergoing extrahepatic cyst excision followed by biliary reconstruction. Todani et alI3 emphasized that bile stagnation in the intrahepatic ducts is possibly responsible for the development of carcinoma. Based on this, they recom-
616
OHI ET AL
Table 4. lntrahepatic
Case No.
Age at Rew (wars)
Sire of Gallstone
1
16
Left
2
14
3
22
4
19
Bilateral
Gallstone Formation
after Total Excisional Operation
Other Findings
OperariveProcedure
Left
Anastomotic stricture -
Removal of stone, reanastomosis Removal of stone, reanastomosis
Left
Liver abscess
Removal of stone, lateral segmentectomy Removal of stone, reanastomosis, lateral seg-
-
mentectomy
Abbreviation: Reop, reoperation.
Table 5. lntrahepatic
Bile Duct Dilatation Dilatation
(IHBDI in Congenital
\
of Bile Duct
relative
stenosis
81
Total cases examined Dilatation of IHBD Yes
61 (75%)
No
20 (25%)
Table 6. lntrahepatic
Bile Duct Dilatation
Dilatation ExtrahepaticBileDuct Cystic Fusiform
45 16
(IHBD) in Congenital
of Bile Duct (n = 61) lntrahepaticBileDuct Cystic
27
Fusiform
18
Cystic Fusiform
0 16
mended hepaticoenterostomy at the porta hepatis with wide anastomotic stoma to prevent anastomotic stricture. We have adopted this surgical procedure for intrahepatic cystic dilatations (Fig 5). In order to achieve a free drainage from the inside of the liver, we recommend the removal of not only the extrahepatic bile duct cyst, but also the relative stenosis between the common hepatic duct and the intrahepatic bile duct cyst. Extremely high anastomosis beyond the relative stenosis or plasty of the intrahepatic bile duct should be performed at the radical operation. Cystic dilatation of the peripheral portion of the intrahepatic bile duct in adults, occasionally managed by lateral segmentectomy of the liver or intrahepatic may develop late complications cystenterostomy, 17P2’*22 such as those noted in these cases. On the other hand, in infants and children having same condition, the
~~ :~~ :..:..
‘I ,,
.... ....,::.:;,.,.:.,, :.: ..:ji:.: ..... .. ((, ,;;t:;‘i;
Extended Anastomosis
iIiiiii:i:iiiiiiil~~~~~::~ ......... .......:.:... ,::j::w
Plasty of Stenotic Portion
Fig 5. Hepaticojejunostomy in a case with intrahepatic bile duct dilatation. Extremely high anastomosis beyond the relative stenosis or plasty of the intrahepatic bile duct should be performed.
incidence of late complications is less than in adults, so excessive surgical intervention should be avoided and surgical approach for them is not always needed at the time of the initial operation. However, these children must be followed carefully for a long time.
REFERENCES 1. Kasai M, Asakura Y, Taira Y: Surgical treatment of choledochal cyst. Ann Surg 172:844-85 1, 1970 2. Kasai M, Suzuki H, Ohi R: Operative procedures for choledochal cyst. Jpn J Surg 7:147-150,1977 3. Ishida M, Tsuchida Y, Saito S, et al: Primary excision of choledochal cyst. Surgery 68:884-888, 1970 4. Spitz L: Choledochal cyst. Surg Gynecol Obstet 147:444-452, 1978 5. Lilly JR: The surgical treatment of choledochal cyst. Surg Gynecol Obstet 149:36-42, 1979 6. Babbitt DP, Starshak RJ, Clemett AR: Choledochal cyst: A concept of etiology. AJR 11957-62, 1973
7. Powell CS, Swayers JL, Reynold VH: Management of adult choledochal cyst. Ann Surg 193:666-676, 1981 8. Todani T, Watanabe Y, Toki A, et al: Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet 164:61-64, 1987 9. Flanigan DP: Biliary cysts. Ann Surg 182:635-643, 1975 10. Nakamura S, Kimura S, Ishidoya T, et al: Re-operation of the choledochal cyst. Z Kinderchir 22:41-50, 1977 11. Klotz D, Cohn BD, Kottmeier PK: Choledochal cysts: Diagnosis and therapeutic problems. J Pediatr Surg 8:271-283, 1973 12. Kim SH: Choledochal cyst: Survey by the Surgical Section of
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