Operative Treatment of Congenital Stenoses of the Intrahepatic Bile Ducts in Patients with Choledochal Cysts Hisami Ando, MD, Kenitiro Kaneko, MD, Fuji0 Ito, MD, Takahiko Seo, MD, and Takahiro Ito, MD, Nagoya, Japan
BACKGROUND: Postoperative complications including intrahepatic calculi may develop after the complete excision of a choledochal cyst. Since congenital stenoses of the intrahepatic bile ducts are more likely the cause of intrahepatic calculi, operative procedures for intrahepatic stenoses are reported. METHODS: There were 16 patients with choledochal cysts who underwent surgery for stenoses of intrahepatic bile ducts. The stenoses were excised at the opening of the common hepatic duct. RESULTS: In the 16 patients, 25 of the 26 stenoses that involved an intraluminal membrane or septum could be excised from the divided end of the common hepatic duct at the hepatic hilum. In 1 patient, the stenosis could not be accessed from the hepatic hilum, and a left hepatic lobectomy was required. In postoperative follow-up, all 16 patients were in good health. CONCLUSIONS: Stenoses of the intrahepatic bile ducts should be treated from the divided end of the common hepatic duct at the initial operation for choledochal cysts. The need for a second operation or hepatic lobectomy may thus be avoided. Am J Surg. 1997;173:491-494. 0 1997 by Excerpta Medica, Inc.
holedochal cysts are characterized by dilatation of the common bile duct, and are associated with pancreaticobiliary maljunction.’ After enteric drainage, there is a high incidence of postoperative complications including cholangiocarcinoma, cholangitis, and biliary stones. This frequently complications mandates reoperation.zmh Thus, complete excision of the choledochal cyst and formation of an anastomosis between the hepatic duct and the jejunum is recommended.“4 Despite this approach, a considerable number of patients will develop cholangitis or intrahepatic calculi.“-zO Stricture of the anastomosis with subsequent dilatation of the intrahepatic ducts has been implicated in the formation of stones.S’Li~‘Y In a previous study of choledochal cyst, we found that stenoses of congenital origin were more likely to be associated
C
From the School of Requests Department Medicine, Manuscript vised form
Department of Pediatric Surgery, University of Nagoya Medicine, Nagoya, Japan. for reprint should be addressed to Hisami Ando, MD, of Pediatric Surgery, University of Nagoya School of 65 Tsurumaicho, Showa-ku, Nagoya, 466 Japan. submitted September 22, 1995 and accepted in reMarch 26, 1996.
C 1997 by Excerpta All rights reserved.
Medica,
Inc.
with stones.” We therefore recommend that these stenoses be treated at the time of the operation to excise the cyst. In this manuscript, an operative approach to stenosis of the intrahepatic bile duct is described. The results in 16 patients with choledochal cysts are reported.
PATIENTS
AND
METHODS
Between October 1992 and July 1995, 29 patients with choledochal cysts were treated at our institution. Stenoses of the intrahepatic bile ducts were detected in 24 patients (83%). Of these, 16 were treated surgically, 4 were treated with balloon dilatation,” and 4 were not treated. The 16 patients who underwent surgical resection were the subjects of this study (Table). They ranged in age from 4 months to 45 years (mean 11.8 years). Six patients had previously undergone surgery for choledochal cysts. The operations included cystoenterostomy (n = 2), and cyst excision (n = 4). The time interval between treatment of the stenosis and the previous operation ranged from 1 year and 8 months to 23 years (mean 11.1 years). In 10 patients, the stenosis was diagnosed before the initial operation, and was treated at the time of cyst excision. Intrahepatic stenosis, which was defined as a narrowing of the lumen of the peripheral bile duct compared with the distal bile duct, was diagnosed preoperatively by cholangiography. This was accomplished either by endoscopic retrograde cholangiopancreatography (ERG’) or by percutaneous transhepatic cholangiography (PTC) (Figure 1). Stenosis was confirmed by cholangioscopy and by direct visual inspection during surgery. Two types of stenoses, membranous and septal, were identified. In the latter, a septum or pillar-like structure was noted, the ends of which were connected to the lateral walls of the bile duct, projecting into the lumen (Figure 2). Operative Procedure The common hepatic duct was divided 0.5 cm below to the confluence of the right and left hepatic ducts. A right angled forceps was introduced into the hepatic duct in an attempt to lay the membrane or septum causing the stenosis (Figure 3). Since most stenoses occurred near the hepatic hilum, they could be brought down gently and excised at the opening of the common hepatic duct under direct visual inspection. The cut surface was then sutured with interrupted 5-O ahsorbable material to achieve hemostasis and prevent cicatricial stricture (Figure 4). Septal stenoses were divided and excised close to the lateral wall to widen the lumen (Figure 5). After resecting the intrahepatic stenoses, a fine retrograde transhepatic biliary drainage tube (RTBD tube; Mitsubishi Bakelite Company, Tokyo, Japan) was inserted into the 0002-9610/97/$17.00 PII SOOO2-961 0(97)00013-5
491
OPERATIVE
TREATMENT
OF
CONGENITAL
STE9
TABLE Patients Number
with
Choledochal
of Patients*
Previous
2 (0)
and
lntrahepatic
4 (4)
in parentheses
refers to number
Biliary
Operations
Present
Cystoenterostomy Cyst excision None
10 (0) ‘Numbers
Cysts
of patients
Stenoses Operations
Cyst excision and resection of stenosis Resection of stenosis or left lobectomy Cyst excision and resection of stenosis with compircating
intrahepatic
caicuh
Figure 3. A right angle forceps revealed a stenosis consisting
Figure 1. lntrahepatic the orifice of the right
stenosis, with contrast and left hepatic ducts
medium (arrows).
blocked
inserted into the left hepatic duct of a thin diaphragmatic membrane.
at
er ial ?nl ed
Figure 2. A bridge-like the right hepatic duct.
septum
(arrow)
was
noted
at the orifice
of
common hepatic duct, and passed through the left hepatic duct, out the anterior abdominal wall (Figure 6). The RTBD tube was used for bihary drainage and to confirm the free passage of bile between the hepatic duct and the jejunum following surgery. Biliary reconstruction was carried out using a Roux-en-Y end-to-side hepaticojejunostomy 492
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created with a single layer of interrupted sutures of i-0 absorbable material. The elimination of the stenosis was assessed by repeated cholangiography thrnugh the RTBL) tube. Within 4 weeks of the operation, when free undisturbed bile flow was confirmed, the tube was removed. The intrahepatic bile ducts were then examined by ultrasonogrqhy, scintigraphy, and/ or computed tomography. All patients underwent liver function testing and ultrasonography every 3 months during the first postoperative year, and every 6 months thereafter. RESULTS Stenoses occurred near the orifice of the left hepatic duct (n = lo), in the right hepatic duct (n = 6), in the posterior JUNE
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1OPERATIVE
TREATMENT
OF
CONGENITAL
STENOSIS
OF
INTRAHEPATIC
BILE
DUCT/AND0
/f
Figure 5. The orifice of the right hepatic wider after the septum was divided. This same patient in Figure 2.
RTBD
ET AL
tube
duct (arrow) became photograph is from the
branch (n = 8), or in the anterior branch (n = 2). Nine patients had multiple stenoses. A total of 26 stenoses in 16 patients were studied. Of these, 21 were membranous and 5 were septal. In the 4 patients with intrahepatic calculi, the stones were found proximal to the stenoses, and no stones were found in the common hepatic duct proximal to the site of the hepaticojejunostomy. We were able to treat 25 of the 26 stenoses from the divided end of the common hepatic duct. In 1 patient with intrahepatic calculi, the stenosis could not be accessed from the common hepatic duct. In this patient, a left hepatic lobectomy was performed. Blood loss ranged from 28 to 688 g (mean 133). The average operative time was 5 hours and 29 minutes. There were no mortalities. There were 2 patients who developed minor postoperative complications, hyperamylasemia and wound infection. Postoperative cholangiography performed through the RTBD tube revealed unimpeded bile flow in the intrahepatic bile ducts of all patients (Figure 7). No episodes of cholangitis occurred in any of the patients during the follow-up period (range 7 months to 3.5 years, mean 1.9 years). The liver function tests were normal in all of the patients.
Figure 6. A membrane was pulled gently and excised around the perimeter. The right hepatic duct was visible before resection of the stenosis and the left hepatic duct was visible after resection. A retrograde transhepatic biliaty drainage (RTBD) tube was inserted from the common hepatic duct and passed through the left hepatic duct, out the anterior abdominal wall.
COMMENTS Intrahepatic calculi that developed after complete excision of choledochal cysts were caused by persistent biliary stasis in previous study subjects with dilated intrahepatic bile ducts that contained anastomotic strictures.5.15m”) LaPointe et al” have reported that anastomotic strictures are due in part to the lack of continuity of the mucosa between the cyst and the intestine. They have recommended that the bowel be anastomosed to the normal biliary system following excision of the cyst. Todani et alI5 have stated that a wide anastomotic stoma permitting free drainage of bile into the intestine is imperative for preventing cholangitis. They have described how to create a wide anastomosis by extending the incision along the lateral wall of both hepatic ducts to obtain a hepaticoenterostomy at the hepatic hilum. Ohi et al’” also have recommended a wide anastomosis of the intrahepatic bile duct. However, our 4 patients who had previously undergone cyst THE
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Figure 7. No stenosis was revealed by cholangiography a retrograde transhepatic biliary drainage tube (arrow) fice of the right and left hepatic ducts. This cholangiogram the same patient in Figure 1.
through
at the oriis from
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While anastomotic strictures have been evaluated in many studies, stenosis of the intrahepatic bile ducts has not been studied extensively. Few reports have emphasized the importance of intrahepatic stenoses of congenital origin as the cause of serious postoperative complications such as biliary calculus formation.24 Membranous stenosis has been reported by a few authors, but they described them as rare This type of stenosis can be overlooked occurrences.“-‘@ because of its resemblance to the wall of the bile duct. Our findings indicate that membranous and septal stenoses of the intrahepatic bile duct are common in patients with choledochal cysts and should thus be regarded as an associated anomalies.*’ Since any obstructive lesion that causes biliary stasis may produce cholangitis and calculus formation, such lesions should be excised to maximally widen the lumen. Diagnosis of these frequently occurring lesions prior to the initial operation may reduce morbidity. Once a stenosis has been diagnosed, treatment is straightforward. Although we have used balloon dilatation to treat these membranous stenoses,‘* results have not always been satisfactory. We thus reserve balloon dilatation for stenoses in more distal branches of the intrahepatic bile duct. Matsumoto et al” have cited hepatic resection as the most common procedure for treating biliary stricture. However, since most stenoses occur at the orifice of the right or left hepatic ducts, resection of the stenoses can be performed easily from the hepatic hilus, thus avoiding the need for hepatic resection. This is a small but well-studied series that addresses the problem of intrahepatic bile duct stenoses in patients who have choledochal cysts. If the intrahepatic ductal stenosis is not recognized and corrected at the time of the cyst excision, a subsequent operution will often be necessary. REFERENCES 1. Yotuyanagi S. Contributions to the etiology and pathology of idiopathic cystic dilatation of the common bile-duct with report of three cases. A new etiological theory based on supposed unequal epithelial proliferatiun at the stage of the physiological epithelial occlusion of the primitive choledochus. Gann. 1936;30:601-652. 2. Todani T, Watanabe Y, Toki A, et al. Carcinoma related to choledochal cysts with internal drainage operations. Surg G?necol Obstet. 1987;164:61-64. 3. Trout HH, Longmire WI’. Long-term follow-up study of patients with congenital cystic dilatation of the common bile duct. Am J Surg. 1971;121:68-86. 4. Yamaguchi M. Congenital choledochal cysts: analysis of 1433 patients in the Japanese literature. AmJ Surf. 1980;140:653-657. 5. Deziel DJ, Rossi RL, Munson JL, et al. Management of bile duct cysts in adults. Arch Surg. 1986;121:410-415. 6. Joseph VT. Surgical techniques and long-term results in the treatment of choledochal cyst. J Pediatr Surg. 1990;25:782-787. 7. Kasai M, Asakura Y, Taira Y. Surgical treatment of choledochal cyst. Ann Surg. 1970;172:844-851.
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8. Ishida M, Tsuchida Y, Saito S, lshitobi Y. Prunxy excision of choledochal cysts. Surgery. 1970;68:884-888. 9. Lilly JR. Total excision of choledochal cyst. Surg G>lnecol Obstet. 1978;146:254+256. 10. Filler RM, Stringel G. Treatment elf choledochal cyst by excision. J Pediatr Sq. 1980;15:437-442. 11. Nunez-Hoyt M, Lees CD, Hermann RE. Bile duct cyst. Experience with 15 patients. .4m J Surg. 1982;144:295-299. 12. Okada A, Nakamura T, Okumura K, et al. Surgical treatment of congenital dilatation of bile duct (choledochal cyst) with technical considerations. Surge?. 1987;101:238%243. 13. Lopez RR, Pmson CW, Campbell JR, et al. Variation in management based on type of choledoch,ll cyst. Am J Surg. 1991;161:612-615. 14. Benhidleb T. Munster B, Ridwelski K, et al. Cystic dilatation of the common hlle duct: surgical trearmcnt and long-term results. BrJ Surg. 1994;81:433-436. 15. Todani T, Watanabe Y, Toki ,4, et .11. Reaperation for congenital choledochal cyst. Ann Surg. 1988;207:142-147. 16. Ohi R, Yaoit,l S, Kamiyama T, et ;11. Surgical treatment of congenital dilatation of the bile duct with qwxal reference to late complications after total excisional operation. J Pediatr Surg. 1990;25:613-617. 17. Hata Y. Sas,lki F, Takahashi H, cr al. Surgical treatment of congenital hiliary dilatation associated with pancreaticohiliary maljunction. Surg Gynecol Obstet. 1993;l i&581-587. 18. Chijiiwa K, Tanaka M. Late compllcatlons after excisional operation in patients with choled<,ch~~l cyst. J Am Coil Surg. 1994;179:139-144. 19. Takiff H, Stone M, Fonkalsrud EW. Choledochal cysts: results of primary surgery and need for reoperation in young patients. .4m J Surg. 1985;150:141-146. 20. Lipset PA, Pitt HA, Colomhanl PM, et al. Choledochal cyst disease. A changmg pattern of ptecenration. Ann Swg. 1994;220:644+652.
21. Ando H, lto T, Kaneko K, Sea T. C,‘~rngenital stenosis of the intrahepatic bile duct associated with choledochal cysts. J ,4m Coil Surg. 1995;181:426&430. 22. Ando H, lto T. Balloonplasty for t&x): strictures associated with choledochal cysts. J Jpn Surg Sot. 1992;93: 1135-l 137. 23. LaPointe R, Gamache A, Pare I’. Bile-duct cyst wth cyst lithiasis: a case report. Gun J Surg. 1984;27:171-273. 24. Matsumoto Y, Fujii H, Yoshiuk,r M, et al. Blliary strictures as a cause of prxnary intrahepatic bile duct stoncb. Vl/i,rld J Surg. 1986;10:867-875. 25. Cater RF, Collins HL. Anomalie\ r>f the bile ducts. Report of .4m J Dis Child. two cases with operations and autopsies. 1936;58:150-161. 26. Glenn F. Moody FG. Intrahepatlc calcull. Ann Surg. 1961;153:71 l-724. 27. Melhem RE, Nahra K. Congenital diaphragm of the common hepatic duct. Br J Radial. 1966;39:392-394. 28. Fisher MM, Chen S, Dekker A. Ctmgenital diaphragm of the hepatic duct. Gustroenterolofiy. 1968;54:605-610. 29. Savader SJ. Benenati JF, Venhrux AC, ct al. Choledochal cysts: classification and cholangiographlc appearance. Am J Roentgenol. 1991;156:327-311. 30. Furukawa H, Hara T, Taniguchl T. A case of septum formation of the common hepatic duct combined with an anomalous hepatlc ducr of the caudate lobe. Gastroenterol Jpn. 1992;27:102-107.
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