Biliary cysts in adults: Cerrahpaşa experience

Abstract

Objective: Biliary cysts are biliary duct dilatations, with 20% of the cysts being diagnosed in adulthood. Abdominal pain, jaundice and palpable abdominal mass are defined as the classical triad. However, nausea, vomiting, fever, itching and weight loss are frequent complaints. There are several treatment options depending on the type of the cyst. This study aimed to share our experience with biliary cysts and contribute to the literature on this subject.

Material and Methods: Thirty patients, who received treatment for biliary cyst from January 1981 to December 2018 at our clinic, were studied retrospectively. The patients were analyzed based on age, sex, type of the cyst, diagnosis and treatment methods, post-op follow up and complications.

Results: Twenty-seven of the patients were females, and three were males. The patients were aged between 16 and 76 years, and the median age was 41.9 years. All patients presented with abdominal pain, which was accompanied by cholangitis in nine patients, nausea and vomiting in four patients, dyspepsia in three patients and palpable mass in one patient. According to the Todani classification, biliary cyst findings were consistent with Type I in 23 patients, Type V in three patients, Type IV in two patients, Type II in one patient and Type III in one patient.

Conclusion: Diagnosis and treatment are complex in biliary cysts due to anatomical proximity and variations. Therefore, it would be beneficial to refer them to referral centers. Choice of treatment should be based on the type of the cyst.

Keywords: Biliary cysts, choledochal cysts, Todani, surgical treatment

Introduction

Biliary tract cysts are surgical issues typically encountered in newborns and children; however, approximately 20% of the cases are diagnosed as late as adulthood. These lesions are usually referred to as choledochal cysts; however, biliary tract cyst is a more suitable term as the cystic dilatation can develop at any part of the biliary tree and it is not specific to the main bile duct (1,2).

Classification system for extrahepatic biliary tract cysts was first proposed by Alonso-Lej, F. in 1959. This classification was revised by Todani in 1977 to include intrahepatic cysts, and the same author updated the system in 2003 based on an anomalous pancreaticobiliary junction (APBJ) case (1,2).

Todani identified five essential biliary cysts, and cystic dilatation of the cystic duct was added in recent years as Type VI classification (3,4).

The incidence rate of biliary cysts is reported to be between 1/100.000 and 1/150.000. The rates are higher at the south and east of the continental Asia (1/1000), and 1/2-2/3 of the reported cases are from Japan. There are around 4000 patients reported up to date. Biliary cysts are more common in females (80%), and they can be seen in women three to eight times more compared to men. Past cases were seen predominantly in children; however, in new cases, the rates in children and adults are remarkably close (1,5,6).

There are different theories on the formation of biliary cysts. The most widely accepted theory is anomalous pancreaticobiliary junction (APBJ). Anomalous pancreaticobiliary junction (Babbitt theory) is seen in 50-80% of the patients with biliary cysts. This is usually seen together with a long common duct facilitating reflux of the pancreatic juice into the biliary tract (5,7). As the ductal junction is outside the duodenum wall and therefore it is devoid of the sphincter of Oddi, it causes reflux of the pancreatic juice into the biliary tree (7).

Most biliary cyst cases show symptoms and are identified before the age of 10. In 20% of the cases, the complaints do not emerge until adulthood (>16 years). Classical presentation includes the triad of abdominal pain, jaundice and palpable abdominal mass, and it is more frequent in children compared to adults, and most of the patients present only with one or two findings of the triad. Recurrent pain in the epigastric or right hypochondrium region, abdominal tenderness and mild jaundice are the most common onset symptoms (1,5,8).

Cysts are usually suspected during transabdominal US (ultrasound) or CT (computed tomography) imaging of a patient with abdominal pain, jaundice or an abdominal mass. Imaging methods to be utilized for the assessment of biliary cysts are transabdominal US, CT, MRCP (magnetic resonance cholangiopancreatography), ERCP (endoscopic retrograde cholangiopancreatography), EUS (endoscopic ultrasound) and hepatobiliary scintigraphy (HIDA). MRCP has been more commonly preferred in recent years as it is non-invasive (5,8).

The purpose of our study was to draw attention to biliary cysts due to their rare frequency and contribute to the literature on this issue.

Material and Methods

Thirty patients who underwent surgery due to biliary tract cysts between January 1981 and December 2018 were included in the study. The data was retrospectively studied using medical records of the patients. The patients were analyzed based on age, sex, type of the cyst, diagnosis and treatment methods, post-op follow up and complications, and the topic was studied in line with the relevant literature.

All quantitative data were expressed as mean ± standard deviation. Qualitative variables were defined by frequencies (%). This study was approved by the Ethics Committee of İstanbul Universty Cerrahpaşa, Cerrahpaşa Medical Faculty in 17.06.2020 with number 83045809-60401.02.

Results

Twenty-seven of the patients were females, and three were males. The patients were aged between 16 and 76 years, and median age was 41.9 years. Twenty-one patients were diagnosed with biliary cysts before surgery. The remaining nine patients were hospitalized with different diagnoses including jaundice, choledocholithiasis, hydatid cyst and were diagnosed during the surgery. All patients presented with abdominal pain accompanied by cholangitis in nine patients, nausea and vomiting in four patients, dyspepsia (fever, pain, jaundice) in three patients and painful palpable mass in one patient.

According to the Todani classification, choledochal cysts findings were consistent with Type I in 23 patients, Type V in three patients, Type IV in two patients, Type II in one patient and Type III in one patient. Roux-en-Y hepaticojejunostomy reconstruction was performed in 16 patients who underwent cholecystectomy + cystectomy due to Type I choledochal cysts, and continuity was ensured in five patients with hepaticoduodenostomy. One patient with Caroli syndrome limited to the left lobe of the liver received left hepatectomy + cholecystectomy + choledochal T-tube drainage. Of the two patients with Type V solitary liver cyst, one received partial cystectomy + cystoraphy + T-tube drainage, and the other patient with localized cyst in segment III ductus and choledoch stones received segment III resection + choledochotomy + T-tube drainage. One of the patients who previously underwent drainage received cystojejunostomy, and four patients received cystoduodenostomy procedures.

US and CT were more commonly used for diagnosis in early patients (11 patients) and recent patients received US followed by a MRCP (20 patients). Pre-op ERCPs were performed for 12 patients for diagnosis and treatment purposes; however, one of the procedures failed. No other preoperative concomitant anatomical anomalies were noted. Two patients who developed post-operative intra-abdominal abscess and collection underwent percutaneous drainage, and two patients with wound site infection were treated with wound dressing.

One patient, who had undergone cholecystectomy + choledocoduodenostomy and developed recurrent cholangitis due to anastomosis stricture in the long-term follow-up, and underwent Roux-en-Y hepaticojejunostomy. One patient who underwent cholecystectomy + cystectomy + Roux-en-Y hepaticojejunostomy in 2004 developed afferent loop syndrome in 2006 and underwent segmental jejunal resection + reanastomosis procedures. One patient who had been operated with cholecystectomy + cystectomy + Rouxen-Y hepaticojejunostomy developed incisional hernia after one year.

Mean hospitalization period was 10.5 days, and mean follow-up period was 8.1 years. No malignancies were observed in the pathological specimens.

Choledochal cyst was discovered in one patient who was taken into surgery for cholelithiasis at another center; the surgery was terminated without any intervention. The second operation was performed in our clinic, and the patient’s Type I choledochal cyst was resected successfully.

In another case, a 40-year-old male patient had biliary cyst accompanying gallbladder cancer. Gallbladder cancer (T1-stage according to the TNM clasiffication) was incidentally identified in the cholecystectomy piece; gallbladder bed excision and lymphadenectomy were planned for the patient. A type 1 biliary cyst was diagnosed during the preoperative imaging, and it was resected concurrently. No other malignancies were observed in the pathological examination.

One patient, who was diagnosed with Caroli disease based on the imaging studies due to abdominal pain and cholangitis, had stone of the left intrahepatic biliary tracts and mechanical jaundice. Percutaneous transhepatic catheter (PTC) and biliary drainage was performed pre-operatively, and then the patient received left hepatectomy + cholecystectomy + T-tube drainage. The patient later received percutaneous drainage for post-operative intra-abdominal abscess and was discharged on day 17.

After the introduction of hepatopancreatobiliary surgery in 2000, our clinic abandoned drainage procedures, and performed the resection procedures for all cases, and usually preferred Roux-en-Y hepaticojejunostomy for the reconstruction. Since this study included a large group of patients going back to 1981, some surgical operations that we do not prefer today were also included in the series.

Discussion

The classical presentation of biliary cysts includes the triad of abdominal pain, jaundice and palpable abdominal mass, and it is more frequent in children compared to adults. Most patients usually present with one or two findings of this triad; but nausea, vomiting, fever, itching and weight loss are also among the common complaints (9,10).

The patients may present to the hospital with signs and symptoms of biliary cyst complications such as pancreatitis, cholangitis and obstructive jaundice. Although rare, another presentation is acute rupture of the cyst with subsequent bile peritonitis (5). Nine of our patients presented with cholangitis during diagnosis, seven of which were treated with medical therapy, and the remaining two were operated after biliary drainage with ERCP and PTC for concomitant mechanical icterus.

Advances in the imaging methods have contributed greatly to biliary cyst diagnosis and classification. Cystic lesions are mostly identified by US or CT first. Ulltrasound sensitivity differs between 70% and 97%. The best non-invasive imaging method for biliary tract cysts is MRCP. ERCP and PTC give us detailed anatomical information. ERCP can effectively show the relationship between the pancreatic duct and biliary duct. PTC can be preferred when ERCP is inconclusive in intrahepatic and proximal biliary ducts. MRCP was performed for all 20 patients, the records of whom we could access (Figure 1, 2).

Figure 1. MRCP demonstrating extrahepatic biliary duct dilatation in a Type I cyst.

Figure 2. MRCP appearance of extrahepatic and intrahepatic biliary duct dilatation in a Type IVA cyst.

Biliary cysts should be differentiated from pancreatic, mesenteric and hepatic cysts (simple cyst, hydatid cyst) which are not connected to the biliary tract. If there is still suspicion after CT and MRCP, hepatobiliary scintigraphy (HIDA) or ERCP can be performed to establish the connection with the biliary tract (5,8).

Biliary cysts can be mistaken for hydatid cyst in endemic regions. This was the issue of our previous study (11). We focused on this issue after we found a solitary intrahepatic cyst extending outside the surface of the liver in a patient who was taken into operation with hydatid liver cyst diagnosis.

The treatment approach in biliary cysts depends on the type of the cyst. Type I, II or IV cysts are generally eligible for surgical resection. Type I and IV cysts are treated with total excision and Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy (1,8).

Type II cysts are eligible for simple excision. Cholecystectomy may also be used. T-tube is placed if there is a defect on the common bile duct after the excision. Type III cysts are treated with sphincterotomy or endoscopic resection if they are symptomatic. Type V cysts are harder to treat; they require prevention against recurring cholangitis and sepsis. The cysts which are limited to one lobe are treated with right or left hepatectomy, and patients with widespread cysts are transplantation candidates (5,8).

Cases with ascending cholangitis should be treated with antibiotics and bile drainage regardless of the type of the cysts (ERCP or PTC). Of the 21 patients who underwent cystectomy for type I or IV choledochal cysts; 16 received Roux-en-Y hepaticojejunostomy, and hepaticoduodenostomy was preferred for five patients (Figure 3).

Figure 3. Resection of the Type I biliary cyst.

Type V cysts may be solitary or multiple. They are referred to as Caroli cysts in most resources (1). This is true for multiple intrahepatic cysts; however, existence of solitary intrahepatic cysts should also be taken into consideration. There were two cases that met this definition in our case series. The treatment method for solitary cysts can be determined based on localization. Of the two cases that met this definition, one received left lateral segmentectomy due to the cystic dilatation of segment III duct and the stones in it (Figure 4). The other case with exophytic growth from the liver surface received partial cystectomy and choledochal T-tube drainage.

Figure 4. Segment 3 resection of the Type V solitary cyst.

In the current literature, it was recomended that in multiple intrahepatic cysts (Caroli), lobectomy should be performed if the disease is limited to one lobe, and if it is widespread, the recommendation is to treat the cholangitis episodes (antibiotics, PTC) first, and then perform liver transplantation (1,5). There was one patient with multiple intrahepatic biliary cysts (Caroli) in our case series. The patient received left hepatectomy as the disease was localized in the left lobe (Figure 5).

Figure 5. Left hepatectomy due to the Caroli disease.

In biliary cysts, the whole bile tree is under the risk of cholangiocarcinoma due to APBJ and pancreaticobiliary reflux. Removal of the cyst does not eliminate future risk of cholangiocarcinoma development (7,12,13).

We did not encounter any cholangiocarcinoma cases in our series.

As for the classical knowledge that biliary cysts carry a risk of cholangiocarcinoma development, we would like to underline that biliary cysts are most common in the Far East and Southeast Asia. Sastry et al. have demonstrated that cancer developed in 7.5% of patients with biliary cysts (70.4% cholangiocarcinoma, 23.5% gallbladder cancer and 6.1% other cancers) (12,14).

Tyson and El-Serag have reported that the rate of malignancy transformation in biliary cysts is higher in Asians (18%) versus Americans (6%). It is also known that biliary tract parasites (Clonorchis sinensis, Opisthorchis viverrini) are commonly seen in those areas (12,13). In these cases, the risk of cholangiocarcinoma development is certainly associated with concurrent parasites (chronic irritation) rather than biliary cysts.

By their nature, biliary cysts may include some anomalies that could be considered as a complexity for surgeons. Concurrent ductal and vascular anomalies increase the difficulty of surgery. They can be more troublesome when they are detected during surgery rather than preoperative tests (15,16).

An interesting revelation of our series was that some of the cases were not diagnosed with choledochal cysts before surgery. If surgeons with limited experience in this area unexpectedly detect choledochal cysts during surgery, they may choose outdated options for treatment of choledochal cysts. Procedures such as choledocoduodenostomy, cystojejunostomy and T-tube drainage that we came across in our series should be considered within that context.

Hepaticoduodenostomy has been associated with higher gastric and biliary cancer due to bile reflux. Furthermore, metaanalyses comparing RHYJ and hepaticoduodenostomy have found more reflux and gastritis in hepaticoduodenostomy. A wide anastomosis facilitating bile flow to the intestine and reducing anastomotic stricture and bile reflux after cyst excision may prevent complications and development of carcinoma in intrahepatic ducts (8,17).

As long as there are no contraindications, choledochal cysts are resected in order to prevent development of malignancies and complications in the future.

Conclusion

In conclusion, biliary cyst diagnosis and treatment should be referred to specialized, advanced facilities as complications are frequent in this area due to anatomical proximity and variations. In Type I cysts, which is the most common subgroup, surgical methods of choice are total cyst resection and RY hepaticojejunostomy.

Cite this article as: Durgun AV, Ergün S, Turgut BC, Şimşek O, Velidedeoğlu M, Sarıbeyoğlu K, et al. Biliary cysts in adults: Cerrahpaşa experience. Turk J Surg 2023; 39 (4): 315-320.

This study was approved by Cerrahpaşa Faculty of Medicine Deanery Clinical Researches Ethics Committee (Decision no: 83045809-60401.02., Date: 07.07.2020).

Externally peer-reviewed.

Concept - AVD, SP, KS; Design - AVD, SE, BCT, SP; Supervision - SP, OS, MV; Materials - AVD, SP, KS, OS; Data Collection and/ or Processing - SE, BCT; Analysis and/or Interpretation - AVD, SE; Literature Search - AVD, SE, SP; Writing Manuscript - AVD, SE, DS; Critical Review - BP, KS.

The authors have no conflicts of interest to declare.

The authors declared that this study has received no financial support.

References

1. Buxbaum J, Lu SC. Cystic diseases of the liver and biliary tract. In Yamada’s Textbook of Gastroenterology (eds. Wang TC, Camilleri M, Lebwohl B, Lok AS, Sandborn WJ, Wang KK and Wu GD). Wiley-Blackwell; 2022.pp. 1759-72. https://doi.org/10.1002/9781119600206.ch83
2. Khan S, Nagorney DM. Chapter 46-Bile duct cysts in adults, Editor(s): William R. Jarnagin, Leslie H. Blumgart, Blumgart’s Surgery of the Liver, Pancreas and Biliary Tract (Fifth Edition), W.B. Saunders ;2012, pp 707- 718.e2, ISBN 9781437714548, https://doi.org/10.1016/B978-1-4377- 1454-8.00046-1. https://doi.org/10.1016/B978-1-4377-1454-8.00046-1
3. Conway WC, Telian SH, Wasif N, Gagandeep S. Type VI biliary cyst: Report of a case. Surg Today 2009; 39(1): 77-9. https://doi.org/10.1007/ s00595-008-3789-4
4. Maheshwari P. Cystic malformation of cystic duct: 10 cases and review of literature. World J Radiol 2012; 4(9): 413-7. https://doi.org/10.4329/ wjr.v4.i9.413
5. Mesleh M, Deziel DJ. Bile duct cysts. Surg Clin North Am 2008; 88(6): 1369-84. https://doi.org/10.1016/j.suc.2008.07.002
6. Atkinson HD, Fischer CP, de Jong CH, Madhavan KK, Parks RW, Garden OJ. Choledochal cysts in adults and their complications. HPB (Oxford) 2003; 5(2): 105-10. https://doi.org/10.1080/13651820310001144
7. Takeshita N, Ota T, Yamamoto M. Forty-year experience with flowdiversion surgery for patients with congenital choledochal cysts with pancreaticobiliary maljunction at a single institution. Ann Surg 2011; 254(6): 1050-3. https://doi.org/10.1097/SLA.0b013e3182243550
8. Soares KC, Arnaoutakis DJ, Kamel I, Rastegar N, Anders R, Maithel S, et al. Choledochal cysts: Presentation, clinical differentiation, and management. J Am Coll Surg 2014; 219(6): 1167-80. https://doi. org/10.1016/j.jamcollsurg.2014.04.023
9. Martínez Ortiz CA, Jiménez-López M, Serrano Franco S. Biliary cysts in adults. 26 years experience at a single center. Ann Med Surg (Lond) 2016; 11: 29-31. https://doi.org/10.1016/j.amsu.2016.08.016
10. 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): 527-31. https://doi.org/10.1016/j.amjsurg.2005.01.025
11. Durgun AV, Gorgun E, Kapan M, Ozcelik MF, Eryilmaz R. Choledochal cysts in adults and the importance of differential diagnosis. J Hepatobiliary Pancreat Surg 2002; 9(6): 738-41. https://doi.org/10.1007/ s005340200102
12. Madadi-Sanjani O, Wirth TC, Kuebler JF, Petersen C, Ure BM. Choledochal cyst and malignancy: A plea for lifelong follow-up. Eur J Pediatr Surg 2019; 29(2): 143-9. https://doi.org/10.1055/s-0037-1615275
13. Ohashi T, Wakai T, Kubota M, Matsuda Y, Arai Y, Ohyama T, et al. Risk of subsequent biliary malignancy in patients undergoing cyst excision for congenital choledochal cysts. J Gastroenterol Hepatol 2013; 28(2): 243-7. https://doi.org/10.1111/j.1440-1746.2012.07260.x
14. Sastry AV, Abbadessa B, Wayne MG, Steele JG, Cooperman AM. What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management? World J Surg 2015; 39(2): 487-92. https://doi.org/10.1007/s00268-014-2831-5
15. Singh S, Singh NP, Goyal A, Hans S, Khichy S. Choledochal cyst with aberrant right posterior sectoral duct. Indian J Surg 2015; 77(Suppl 2): 744-5. https://doi.org/10.1007/s12262-013-0926-0
16. Lal R, Behari A, Hari RH, Sikora SS, Yachha SK, Kapoor VK. Variations in biliary ductal and hepatic vascular anatomy and their relevance to the surgical management of choledochal cysts. Pediatr Surg Int 2013; 29(8): 777-86. https://doi.org/10.1007/s00383-013-3333-5
17. Shimotakahara A, Yamataka A, Yanai T, Kobayashi H, Okazaki T, Lane GJ, et al. Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better? Pediatr Surg Int 2005; 21(1): 5-7. https://doi. org/10.1007/s00383-004-1252-1