Early and late results of excision of choledochal cysts

Early

and Late Results

By Htut

Saing,

Htoo

Han,

K.L.

of Excision Chan,

W. Lam, Hong

Background/Purpose: dochal cyst excision are relatively few.

Reports on the late with hepaticojejunostomy

results

During (mean,

a follow-up 8.5 years),

period ranging from anastomotic stricture,

of choiein children

4 months cholangitis,

to

A

LTHOUGH LONG-TERM results including anastomotic strictures, intrahepatic stone formation, and cholangitis after internal drainage procedures have been well documented, reports on the late results of cyst excision with hepaticojejunostomy are relatively few. With this in mind, we are now reporting our early and late results of excision of choledochal cysts together with our results of internal drainage procedures as a comparison. MATERIALS

AND

METHODS

A review of records and radiological investigations of 84 patients who had choledochal cyst cared for by the Division of Paediatric Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital over a period between 1965 and 1996 was undertaken. The ages of our patients ranged from 2 weeks to 14 years (mean, 3.5 years) and the male:female ratio was 1:3. The follow-up of these patients after surgery ranged from 4 months to 17 years (mean, 8.5 years). Of the 84 patients, 79 have undergone definitive surgery, and the procedures are listed in Table 1. Three are awaiting surgery, one is being observed with Caroli’s disease, and one child’s parents refused surgery. Although internal drainage procedures were performed in the earlier period, since 1972 we have been performing cyst excision and hepaticojejunostomy (EX-HJ) using a 40 cm Roux limb without antireflux valve in 41 patients. Cyst excision was performed as a primary procedure in 39 children and as a secondary procedure after choledochocystojejunostomy (CCJ) in two. CCJ was performed as a temporizing measure because of excessive bleeding encountered during dissection for cyst excision in these two patients who had repeated attacks of cholangitis. Radical cyst excision was performed with standard techniques’ in most cases, whereas endocystic excision2~3 was used in five instances. Intraoperative flexible choledochoscopy at the time of cyst excision Journal

of Pediatric

Surgery,

Vol32,

No 11 (November),

Chan,

W. Cheng,

and

Cysts

P.K.H.

Tam

Kong

Mefhods: Of the 84 patients who had choledochal cyst who came under our care, 79 have had definitive surgery, three are awaiting surgery, one is being observed with Caroli’s disease, and the parents of one child have refused surgery. Thirty-eight patients treated decades ago had internal drainage procedures. Since 1972, 41 patients have had cyst excision with hepaticojejunostomy using a 40-cm Roux loop without an antireflux procedure. Early complications in those who underwent cyst excision with hepaticojejunostomy included anastomotic leak in three patients who required reoperation, cholangitis in two, and fluid collection in the gall-bladder bed that required no intervention in one. Results: 17 years

F.L.

of Choledochal

1997: pp 1563-1566

and intrahepatic stone formation developed in two children after being well for 8 years and over 11 years. These children required additional surgical procedures to overcome their problems. Asymptomatic intrahepatic stones 2 years after cyst excision with hepaticojejunostomy developed in a third child. There was no mortality in the entire group that underwent cyst excision and they are all enjoying a good quality of life. Conclusions: Careful, long-term follow-up is important in children who have choledochal cyst excision with hepaticojejunostomy. J Pediatr Surg 32: 1563- 1566. Copyright 0 1997 by W5. Saunders Company. INDEX WORDS: nostomy.

Choledochal

cyst,

cyst

excision,

hepaticojeju-

was performed in live patients whose intrahepatic ducts were not visualized during intraoperative cholangiography because of technical problems or in those who had repeated attacks of cholangitis before undergoing cyst excision. In addition, in postexcision patients who had intrahepatic stones, intraoperative flexible choledochoscopy played an important role during exploration to extract stones and to visualize intrahepatic strictures, if present, which would then be subjected to balloon dilatation. For patients in whom late strictures and intrahepatic stone formation developed, revision of anastomotic stricture? as well as hepaticocutaneous jejunostomy followed by choledochoscopic stone clearance have been our preferred methods of treatment5 instead of repeated open exploration to remove the intrahepatic stones, which can be very demanding. Although in the earlier years, a variety of radiological investigations were used, since the early 1980s we have used ultrasound scan in all and computed tomography (CT) in 80% of our patients. Percutaneous transhepatic cholangiography (PTC) was used when better delineation of intrahepatic stones and strictures was required postoperatively. In recent years, we have been using helical CT cholangiogram with megulamine iodoxamic acid (Endobil, Bracco, Milan, Italy) as the intravenous contrast, because it is said to have a lower complication rate than other intravenous cholangiogram agents and it also demonstrates

From the Division of Paedintric Surgeq, Departmerzts of Surgery and Diagnostic Radiology, The Urtiversiol of Hong Kong, Queen Maiy Hospital, Hong Kong. Presented at the 30th Armual Meeting of the Pacijic Association of Pediatric Surgeons, Phoenix, Arizona, May 9-13, 1997. Address reprim requests to Professor Htut Saing, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Hong Kong. Copyright 0 1997 by WB. Saunders Company 0022-3468/97/3211-OOIO$O3.00/0 1563

SAING

1564

Table

1. Operative

Procedures

Cyst excision and hepaticojejunostomy Roux loop without antireflux valve

Performed with

Leak

40 cm 41 38

Internal drainage procedures Choledochocystoduodenostomy Choledochorrhaphy

Cholangitis

ET AL

(12)

and choledochoduodenostomy

Choledochocystojejunostomy

Roux-en-Y

(6) (20) 79

Total

intrahepatic stones very clearly (Fig 1). Endoscopic retrograde cholangiography (ERCP) was used in one-third of our patients, being reserved for older children. We have used magnetic resonance imaging (MRI) in two patients. We have consistently performed operative cholangiograms in all patients. Although the procedure is relatively inexpensive and requires little additional operating theater time, it affords useful information of the intrahepatic ducts in patients who have not been subjected to ERCP. The size; shape, and configuration of intrahepatic ducts, the presence or absence of existing intrahepatic strictures at the time of surgery for choledochal cyst are demonstrated. In addition, the common channel and the pancreatic ducts are often shown and information thus obtained is of considerable help in performing safe excision of choledochal cyst.

RESULTS

In the 41 patients who underwent cyst excision, there was no early or late mortality. There was no observed malignant transformation in any of our patients either before or after EX-HJ. Cyst excision was associated with anastomotic leak in our first three (7%) cases, and the sites of leakage were at the hepaticojejunostomy in two, and at the jejunojejunostomy in one. The patients recovered after reexploration, repair of leakage, supplemented by total parenteral nutrition (TPN), intravenous antibiotics, and adequate drainage. A high fever developed postoperatively in two patients (5%) and clinically cholangitis was diagnosed. With intravenous antibiotic therapy, the cholangitis resolved and the patients have remained well. One child was found on postoperative ultrasound (US) study to have a small fluid collection in the gallbladder fossa, which resolved spontaneously.

Cyst excision, Hepaticojejunostomy n=41

Drainage

procedures n=38

Fig 2. Internal drainage procedures are associated with a high incidence of early morbidity and mortality, whereas cyst excision with hepaticojejunostomy is associated with acceptable morbidity and zero mortality.

Internal drainage procedures performed in the early years of this study, however, were associated with significant morbidity and mortality (Fig 2). Anastomotic leak occurred in three of 38 patients (8%), and the sites of leakage were at choledochorrhaphy in two and at cystojejunostomy Roux-en-Y in one. All three children required reexploration, repair of leakage, intravenous antibiotics, and TPN; two recovered well and one died. Cholangitis occurred in four patients (11%) in this group who underwent drainage procedures; three had choledochocystoduodenostomy and one had choledochocystojejunostomy Roux-en-Y. Despite active treatment, all four patients in whom cholangitis developed and one in whom anastomotic leak developed died of septicemia and multiorgan failure. The late results of cyst excision are generally excellent and, 38 (93%) of the patients are enjoying very good health (Fig 3). However, symptomatic major complications following prolonged periods of excellent health developed in two children, the first beginning 11 years and the second 8 years after the initial operation. Intrahepatic stones developed in a third child. This child was entirely asymptomatic but the stones were picked up by a routine ultrasound scan performed as part of the prospective study protocol for patients who have undergone surgery for choledochal cyst. The details of the three patients in whom intrahepatic stones developed (two Stricture, *tone

Cholangitis , stone

Died

Drainage procedures ll=38

Fig 1. Helical CT cholangiogram filling defects in both left and right

shows intrahepatic

intrahepatic ducts.

stones

as

Fig 3. Late results of surgical treatment show that cyst a far better procedure with minimal late complications mortality.

excision and

is no

EXCISION

OF CHOLEDOCHAL

1565

CYST

symptomatic and one asymptomatic), the surgical procedures performed, and the outcome are summarized in Table 2. In Case 1, after revision of the anastomotic stricture and fashioning a hepaticocutaneous jejunostomy, successful stone clearance was achieved after a series of flexible choledochoscopies and the use of grasping forceps and baskets and supplemented by electrohydraulic lithotripsy. The intrahepatic strictures were treated successfully by Gruntiz balloon dilatation, and the child has been symptom free for more than 4 years5 In Case 2, the child was subjected to percutaneous transhepatic biliary drainage (PTBD) followed by dilatation of the anastomotic stricture using a Gruntiz balloon catheter and was then subjected to revision of the anastomotic stricture and hepaticocutaneous jejunostomy. Subsequent choledochoscopic stone extraction and clearance was performed as in Case 1. She has remained symptom free for 1 year. It is of interest that Case 3 remains totally asymptomatic for more than 1 year despite anastomotic stricture and intrahepatic stones. Interventional procedures to revise the anastomotic stricture and extraction of stones for this asymptomatic child are pending. The late results of internal drainage procedures are disappointing in our series (Fig 3). The procedures were associated with a much higher (23%) incidence of cholangitis and there was a late mortality of 11%. DISCUSSION

The safety and desirability of total excision of extrahepatic choledochal cyst has been well established,2,6;7and there have been numerous publications on late complications after internal drainage procedures.8-‘3 However, reports on the long-term results of extrahepatic cyst excision are few.4.‘4-16 Ohi et al4 reported four patients with intrahepatic stone formation 7 to 1.5 years after the initial operation, and all required corrective surgical Table

CSX? NO.

Sex/Age (yr) at -is op

1

F/l

2

F/O.6

2. Late Complications

After

Cyst Excision,

Recurrent Cholangitis After Initial op (yr)

Anastomotic and lntrahepatic

lntrahepatic stones Detected After

Duct Strictures

op iv)

(yr)

11

+

13.5

14.5

10

11

8

procedures. Todani et al’” reported seven of 73 patients (10%) who required reoperation for postoperative complications after cyst excision, five of whom underwent hepaticoduodenostomy, and four underwent hepaticojejunostomy Roux-en-Y after cyst excision at the initial operation. At late follow-up, cholangitis was observed in nine patients (12%) but only three patients who had intrahepatic involvement required reoperation several years after excisional procedures. Recent reports by Ando et alI5 and Uno et alI6 have emphasized the importance of membranous stenoses, which were already present at the time of cyst excision, and which were thought to be congenital in origin as major contributors of congenital intrahepatic duct dilatation and subsequent intrahepatic stone formation. These stenoses were in the form of a diaphragm-like membrane with a narrow orifice or with a bridge of tissue going across the orifice. Intrahepatic duct dilatations were already present proximal to the stenoses and stones were found in these dilated ducts at the time of initial excisioni or years after hepaticojejunostomy.15.16 Of a group of 56 patients who underwent cyst excision, there were three patients who had intrahepatic duct dilatation associated with downstream stenosis of the bile duct but without anastomotic stricture. All these three patients had intrahepatic stone formation and two had associated cholangitis. On the other hand, only one of 29 (3%) without intrahepatic duct dilatation and one of 24 (4%) with intrahepatic duct dilatation but without downstream intrahepatic duct stenosis had intrahepatic stones during a mean follow-up of 13.6 years.‘” In our series of 41 cyst excisions, there were two patients who were symptomatic and presented with repeated attacks of cholangitis and were also proven to have stricture and stone formation 11 and 8 years after cyst excision, respectively, and both required major surgical interventions. Interestingly, our third patient who Management,

and Outcome

Age at Intervention for stones

Surgical Procedures

Remarks

Revision of anastomotic strictu re Hepaticocutaneous jejunostomy

Well for 4 years since last procedure A course of drug therapy given

Endoscopic stone clearance PTBD followed by balloon dilatation of anastomotic stricture

for liver flukes Well for 1 year since dure

last proce-

Revision of anastomotic strictu re Hepaticocutaneous jejunostomy 3

MI4

Asymptomatic

+

6

Awaiting

Endoscopic stone clearance No procedure performed yet

intervention

Abbreviations:

Op. operation;

PTBD, percutaneous

transhepatic

biliary

drainage.

Completely asymptomatic Year since stones were detected

for 1

1566

SAING

had intrahepatic stones was discovered as a result of a prospective study on postoperative choledochal cyst patients. This child has been symptom free for 2 years since initial cyst excision and for 1 year since intrahepatic stones were discovered; she will be offered surgical intervention when she becomes symptomatic. This case, as well as the report of Uno et a1,16 illustrate that intrahepatic stones may form in postexcision patients asymptomatically and without initial cholangitis. In our patients in whom postoperative intrahepatic stones developed, we also note that two were associated with intrahepatic duct dilatation with downstream stenosis and one was not; all three, however, had varying degrees of anastomotic strictures. The high incidence of carcinoma arising in the wall of choledochal cyst as well as in the remnant portion of the cyst wall, or in the intrahepatic portion of the cyst has been well documented.gJ2J4J7-1gMalignant degeneration according to age at initial operation has also been reported, and it has been estimated that the risk of cancer in patients who had choledocal cyst diagnosed in the first decade is 0.7%, whereas in those who had choledochal cyst diagnosed in 11 to 20 years and in more than 20 years is 6.8% and 14.3%, respectively.lg Therefore, an early diagnosis of choledochal cyst during the first decade of life and total excision of choledochal cyst is considered mandatory to prevent the development of carcinoma in the extrahepatic portion of choledochal cyst. To date, we have not encountered malignancy in our patients during the period of follow-up. However, our hepatobiliary surgical colleagues have found cancer in 5 of 15 adult patients who had choledochal cyst during the period 1987 to 1995 (Liu et al, personal communication).

ET AL

In light of the published literature concerning the known high incidence of malignant changes in the residual portions of choledochal cysts,9J2J7J8be it extrahepatic or intrahepatic, every effort must be made to follow-up on these patients for their life time. To prevent anastomotic strictures, most investigators recommend very high anastomoses, beyond the relative stenosis in the common bile duct (CBD), or a plastic operation of the junction of the intrahepatic ducts (IHD) with the CBD.4.14 We fully concur with the recommendation. However, it should be noted that the intrahepatic duct stricture may form not only at the junction of IHD and CBD but also proximally in the intrahepatic bile ducts.15J6 For strictures at these levels, because open surgical procedures will be very challenging, we recommend the construction of a hepaticocutaneous jejunostomy. Balloon dilatation of intrahepatic duct strictures and endoscopic ductal clearance of stones may then be possible.5 If, for example, strictures and stone formation are localized to the left lateral segment, then lateral segmentectomy of the liver or cystenterostomy may be considered.‘J3J6 The possibility of recurrent strictures and recurrent intrahepatic stone formation is forever present even if we are able to manage these complications in the earlier stages by methods described above or by other means. In addition, the possibility of malignant change in the dilated intrahepatic ducts must not be overlooked. Hence, careful long-term follow-up and liver transplantation for progressive liver failure caused by recurrent cholangitis, strictures, and stones or for malignant change in the liver may be considered.

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10. Flanigan DP: Biliary cysts. Ann Surg 182:635-643, 1975 11. Kim SH: Choledochal cyst: Survey by the surgical section of the American Academy of Pediatrics. J Pediatr Surg 16:402-407, 1981 12. Yamaguchi M: Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 140:653-657, 1980 13. O’Neill JA, Jr, Templeton JM, Jr, Schnaufer L, et al: Recent experience with choledochal cyst. Ann Surg 205:533-540,1987 14. Todani T, Watanabe Y, Toki A, et al: Reoperation for congenital choledochal cyst. Ann Surg 207: 142-147, 1988 15. Ando H, Ito T, Kaneko K, et al: Congenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J Am Co11 Surg 181:426-430, 1995 16. Uno K, Tsuchida Y, Kawarasaki H, et al: Development of intrahepatic cholelithiasis long after primary excision of choledochal cysts. JAm Co11 Surg 183:583-588, 1996 17. Todani T, Watanabe Y, Narusue M, et al: Congenital bile duct cysts. Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263-269, 1977 18. Todani T, Tabuchi K, Watanabe Y, et al: Carcinoma arising in the wall of congenital bile duct cysts. Cancer 44:1134-1141, 1979 19. Voyles CR, Smadja C, Shands C, et al: Carcinoma in choledochal cysts. Arch Surg 118:986-988, 1983