- Research article
- Open Access
- Open Peer Review
Surgical treatment of congenital biliary duct cyst
© Wang et al; licensee BioMed Central Ltd. 2012
- Received: 14 December 2011
- Accepted: 30 March 2012
- Published: 30 March 2012
It is acknowledged that total cyst excision is a safe and ideal surgical treatment for congenital biliary duct cyst, compared to simple internal drainage. The aim of this study was to determine the optimal operation occasion and the effect of laparoscopy on congenital biliary duct cyst based upon total cyst excision.
From January 2002 to January 2011, 217 patients were admitted to Southwest Hospital for congenital biliary duct cyst. To determine the optimal surgery occasion, we divided these subjects into three groups, the infant group (age ≤ 3 years), the immaturity group (3 < age ≤ 18 years), and the maturity group (age > 18 years), and then evaluated the feasibility, risk and long-term outcome after surgery in the three groups. To analyze the effect of laparoscopic technique on congenital biliary duct cyst, we divided the patients into the laparoscopy and the open surgery groups.
Among the three groups, the morbidity from cholangiolithiasis before surgical treatment had obvious discrepancy (p < 0.05) (lowest in the infant group), and intraoperative blood loss also had apparent diversity (p < 0.05). Furthermore, long-term outcomes (secondary cholangiolithiasis, stoma stenosis and cholangiocarcinoma) showed no significant difference between different groups (p > 0.05).
Similarly, no significant discrepancy was observed in the morbidity from postoperative complications or long-term postoperative complications (p > 0.05) between the laparoscopic and the open surgery groups.
We conclude that total cyst excision should be performed as early as possible. The optimal treatment occasion is the infant period, and laparoscopic resection may be a new safe and feasible minimally invasive surgery for this disease.
- Congenital biliary duct cyst
- Total cyst excision
- Laparoscopic resection
In the present study, we will investigate the optimal timing of total cyst resection and the effect of laparoscopic technique on the disease.
From January 2002 to January 2011, 217 patients were admitted to Southwest Hospital, (Chongqing, China) for congenital biliary duct cyst. Demographic data and preoperative status were collected prospectively and analyzed retrospectively. Of the 217 patients, 187 consecutive cases underwent total cyst excision in our hospital. Data were collected retrospectively from a computerized database and analysis of patients' hospital charts. Supplementary information was obtained from standardized telephone interviews with the patients during the follow-up. This study was approved by the Ethical Committee of Southwest Hospital.
Parameters of the hepatobiliary system were evaluated preoperatively, including demography, age, gender, clinical symptoms, biliary complication, localization of cyst, and imaging findings (ultrasonography, computed tomography (CT) scan, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP)).
All the patients received the same postoperative management by the same team of surgeons. Patients who received surgical treatment were monitored in the intensive care unit (ICU) during the early postoperative period. Subsequently, whether to stay in the ICU depended on the patient's condition.
No type III cyst patient was included in the 187 patients who underwent total cyst excision in our hospital. Treatment of cyst depended on clinical typing. Patients with types I and II cyst underwent total extra-hepatic cyst excision. The division of the biliary tract should be connected to healthy biliary tissues either in the superior or the inferior part of the cyst. Meanwhile, the Roux-en-Y cholangiojejunostomy was also conducted. In this study, the intra-hepatic cysts of types IV and V were localized forms. Thus, patients with type IV cyst also needed total extra-hepatic cyst excision and Roux-en-Y cholangiojejunostomy. If intra-hepatic cysts were localized and complicated with biliary ductal stricture, stones, abscess, or liver atrophy, the partial hepatectomy would be carried out for type IV cysts. Treatment of patients with type V cyst also relied on the location of bile duct abnormalities. Patients with type V cyst should undergo partial hepatectomy and Roux-en-Y cholangiojejunostomy, if complicated with biliary ductal stricture, stones, abscess, or liver atrophy. Otherwise, they only received Roux-en-Y cholangiojejunostomy.
All the patients were followed up by the same team of surgeons after the surgery every 2 to 3 months. Ultrasonography, ERCP, or MRCP was annually conducted for stenosis, stone, or even advanced tumor. Patients with stenosis or cholangiolithiasis were treated conservatively if they had no or mild symptoms. Patients with cholangiocarcinoma were suggested with radical resection. If the patients with cholangiolithiasis and severe life-threatening attack of acute cholangitis did not respond to initial conservative treatments, other definitive operations should be considered.
Statistical analysis was performed using SPSS 18. If there were 2 or more expectations less than 5, continuous data would be assayed with Fisher exact test; otherwise, data were analyzed with chi-aquare and t tests.
Demographic Data and Preoperative Status
28.2 ± 18.8
Female, n (%)
Male, n (%)
Abdominal pain, n (%)
Jaundice, n (%)
Cholangitis, n (%)
Type I, n (%)
Type II, n (%)
Type IV, n (%)
Type V, n (%)
Total cyst excision should be performed as early as possible, and the optimal treatment time is the infant period
Of 80 patients who had intra- and/or extra-cholangiolithiasis before surgical treatment, 2 belonged to the infant group (n = 23), 21 to the immaturity group (n = 58), and 57 to the maturity group (n = 136). The three groups showed significant discrepancy (p = 0.009 < 0.05) in cholangiolithiasis morbidity before surgical treatment. The cholangiolithiasis morbidities in the immaturity and maturity groups were significantly higher than that in the infant group (p = 0.005 < 0.05); however, no discrepancy existed between the immaturity and maturity groups (p > 0.05). In addition, 8 patients had cholangiocarcinoma, of whom 1 belonged to the immaturity group and 7 to the maturity group. However, no significant discrepancy was observed between the three groups (p = 0.459 > 0.05).
Basic characteristics of patients underwent total cyst excision
27.8 ± 18.2
Female, n (%)
Male, n (%)
Type I, n (%)
Type II, n (%)
Type IV, n (%)
Type V, n (%)
Intraoperative and postoperative characteristics of patients underwent total cyst excision
Blood loss (ml)
66.0 ± 34.2
261.4 ± 305.6*
389.4 ± 402.6*#
Operation time (min)
289.0 ± 70.4
341.2 ± 104.3
320.0 ± 120.4
Pulmonary infection (n)
After the surgery, 153 of 187 patients having undergone total cyst excision were regularly followed up. The visited rate was 81.8%, and the visited duration was 10 to 116 months. There were 10 patients with secondary cholangiolithiasis, 4 with stoma stenosis, and 2 with secondary cholangiocarcinoma. Of them, none of the infant patients (n = 18) suffered from intra- and/or extra-cholangiolithiasis, stoma stenosis, or secondary cholangiocarcinoma. In contrast, 1 patient in the immaturity group (n = 46) had cholangiolithiasis, and in the maturity group (n = 89), 9 suffered from secondary cholangiolithiasis, 4 from stoma stenosis, and 2 from secondary cholangiocarcinoma. However, no significant discrepancy existed among the three groups in morbidity from cholangiolithiasis, stenosis or advanced tumors (p > 0.05).
Totally laparoscopic resection may be a new safe and feasible minimally invasive surgery for this disease
Three patients in the laparoscopic group (n = 22) and 14 patients in the open surgery group (n = 165) showed postoperative complications. After a long-term follow-up, 13 patients in the open surgery group (n = 136) reported secondary cholangiolithiasis, stoma stenosis or cholangiocarcinoma, whereas no patient in laparoscopic group (n = 17) reported those diseases. However, no significant discrepancy was observed between the two groups in postoperative complications or long-term follow-up results (p = 0.668 > 0.05, p = 1.0 > 0.05, respectively).
Internal drainage by cyst-enterostomy or partial cyst excision was once considered the preferred surgical treatment for congenital bile duct cysts. However, its poor prognosis (frequent cholangitis, cholangiolithiasis, and even cholangiocarcinoma) confined its application severely . In recent years, total cyst excision is regarded a more ideal and reasonable treatment for this disease. Our previous study has also confirmed that the total cyst excision has better prognosis than single internal drainage . However, the optimal surgical treatment timing is still controversial.
Congenital bile duct cysts cause abnormal bile duct structure, which is the main factor for cholangiolithiasis and cholangiocarcinoma. The dilated bile ducts cause dilatation-stenosis-like biliary stricture, which subsequently leads to the abnormality of bile dynamics, causing cholestasis and then inducing biliary tract inflammation. In addition, cholestasis could further cause cholangitis and cholangiolithiasis , and even cholangiocarcinoma after long-term inflammatory stimulation . The above evidence suggests that the longer the disease course is, the higher the morbidity of complications will be. This study also suggests that obvious discrepancy exists in cholangiolithiasis morbidity among three groups before the surgical treatment. Of all patients, eight suffer from advanced cholangiocarcinoma (the maturity = 7 and immaturity = 1), which did not arise in infancy. No significant difference was abserved among the three age groups, which may be explained by the limited period of observation.
In this study, we domonstrate that the infants with congenital bile duct cyst have lower morbidity of cholangiolithiasis before surgical treatment and lower intraoperative blood loss when undergoing total cyst excision than the immaturity and the maturity groups. In addition, long-term follow-up data indicate that there is no significant difference among the three groups. These findings suggest that total cyst excision should be performed as early as possible, the optimal treatment timing is during infancy, and the totally laparoscopic resection, which is safer and more feasible, may be a new minimally invasive surgery for this disease.
We would like to thank Miss Xie-Wan Chen and Ms Xiao-Qing Zhan (Medical English Department, Third Military Medical University, China) for a critical reading of the manuscript and kindly giving precious advice. This work was supported by State Key Basic Research Development Program (2012CB518103), Natural Science Foundation Programs (81130026). This research is also supported by program of New Century Excellent Talents in University (NCET) from Ministry of Education and intramural grants from the State Key Laboratory of Trauma, Burns and Combined Injury.
- Yamaguchi M: Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg. 1980, 140 (5): 653-657. 10.1016/0002-9610(80)90051-3.View ArticlePubMedGoogle Scholar
- Wiseman K, Buczkowski AK, Chung SW, Francoeur J, Schaeffer D, Scudamore CH: Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment. Am J Surg. 2005, 189 (5): 527-531. 10.1016/j.amjsurg.2005.01.025. discussion 531View ArticlePubMedGoogle Scholar
- Todani T, Watanabe Y, Toki A, Urushihara N: Carcinoma related to choledochal cysts with internal drainage operations. Surg Gynecol Obstet. 1987, 164 (1): 61-64.PubMedGoogle Scholar
- Nagorney DM, McIlrath DC, Adson MA: Choledochal cysts in adults: clinical management. Surgery. 1984, 96 (4): 656-663.PubMedGoogle Scholar
- Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K: Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 1977, 134 (2): 263-269. 10.1016/0002-9610(77)90359-2.View ArticlePubMedGoogle Scholar
- Caroli J, Soupault R, Kossakowski J, Plocker L, Paradowska : Congenital polycystic dilation of the intrahepatic bile ducts; attempt at classification]. Sem Hop. 1958, 34 (8/2): 488-495/SP.PubMedGoogle Scholar
- Yonem O, Bayraktar Y: Clinical characteristics of Caroli's syndrome. World J Gastroenterol. 2007, 13 (13): 1934-1937.View ArticlePubMedPubMed CentralGoogle Scholar
- Kim SH, Lim JH, Yoon HK, Han BK, Lee SK, Kim YI: Choledochal cyst: comparison of MR and conventional cholangiography. Clin Radiol. 2000, 55 (5): 378-383. 10.1053/crad.2000.0438.View ArticlePubMedGoogle Scholar
- Lee HK, Park SJ, Yi BH, Lee AL, Moon JH, Chang YW: Imaging features of adult choledochal cysts: a pictorial review. Korean J Radiol. 2009, 10 (1): 71-80. 10.3348/kjr.2009.10.1.71.View ArticlePubMedPubMed CentralGoogle Scholar
- Ulrich F, Pratschke J, Pascher A, Neumann UP, Lopez-Hänninen E, Jonas S, Neuhaus P: Long-term outcome of liver resection and transplantation for Caroli disease and syndrome. Ann Surg. 2008, 247 (2): 357-364. 10.1097/SLA.0b013e31815cca88.View ArticlePubMedGoogle Scholar
- Söreide K, Körner H, Havnen J, Söreide JA: Bile duct cysts in adults. Br J Surg. 2004, 91 (12): 1538-1548. 10.1002/bjs.4815.View ArticlePubMedGoogle Scholar
- Tian Y, Wu SD, Zhu AD, Chen DX: Management of type I choledochal cyst in adult: totally laparoscopic resection and Roux-en-Y hepaticoenterostomy. J Gastrointest Surg. 2010, 14 (9): 1381-1388. 10.1007/s11605-010-1263-2.View ArticlePubMedGoogle Scholar
- Liu ZP, Dong JH, Bie P, Li ZH, He Y, Wang SG: A comparison study of different surgical procedure for congenital cystic dilatation of the bile duct. J Dig Surg. 2004, 3 (2): 84-86.Google Scholar
- Todani T, Watanabe Y, Toki A, Morotomi Y: Classification of congenital biliary cystic disease: special reference to type Ic and IVA cysts with primary ductal stricture. J Hepatobiliary Pancreat Surg. 2003, 10 (5): 340-344. 10.1007/s00534-002-0733-7.View ArticlePubMedGoogle Scholar
- Edil BH, Cameron JL, Reddy S, Lum Y, Lipsett PA, Nathan H, Pawlik TM, Choti MA, Wolfgang CL, Schulick RD: Choledochal cyst disease in children and adults: a 30-year single-institution experience. J Am Coll Surg. 2008, 206 (5): 1000-1005. 10.1016/j.jamcollsurg.2007.12.045. discussion 1005-1008View ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/12/29/prepub
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