Management of Choledochal Cysts

10 mm > www.co-gastroenterology.com 2016, 32:225–231 well as thestrategy current for choledochal indications cyst. and management Correspondence to Timothy M. Pawlik, MDSurgical MPH Oncology, Professor PhD, Chief, of Division SurgerySurgery, of 600 and N. Oncology, Wolfe Department Street, of BlalockTel: 688, +410 Baltimore, 502 MD 2387; 21287, USA. fax: +410Curr 502 Opin 2388; Gastroenterol e-mail: [email protected] DOI:10.1097/MOG.0000000000000256 BACKGROUND The precise etiology of choledochalunderstood, cysts is although not well anomalousary pancreaticobili- duct unionhypothesis [1]. (APBDU) A is long common a channel generally ( accepted Department of Surgery, DivisionHospital, of Baltimore, Surgical Maryland, Oncology, USA Johns Hopkins proximal to ampullary sphincter) isdispose thought to to pancreatic pre- enzyme refluxtree into with consequent the biliary biliary duct inflammationpressure, and leading to duct dilation [7–9]. The theory ]. && ,5 & ,4]. In both & Sean M. Ronnekleiv-Kelly, KevinTimothy C. M. Soares, Pawlik Aslam Ejaz, and 2016 Wolters Kluwer Health, Inc. All rights reserved. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. ß Treatment of choledochal cystmay aims occur. to More avoid recently, thecyst minimally numerous with invasive hepatic, acceptable approaches pancreatic, morbidity areafter or and being resection biliary mortality. used has complications Moreover, for demonstrated that long-termcholedochal the that follow cyst treatment although up resection, of the of these choledochal riskeven choledochal patients of after cyst may biliary excision. patients remain malignancy at is a significantly slightlySummary decreased increased after risk ofManagement biliary of malignancy choledochal cystand and choledochal the cyst operative subtype. conductexperience However, will of given depend most the upon centers, complexhepatopancreaticobiliary the these nature centers patient patients of familiar comorbidities should choledochal with be cyst management evaluated and of andKeywords limited choledochal treated cyst. at high-volume cholangiocarcinoma, choledochal cyst, minimally invasive choledochal cyst resection Purpose of review Historically, surgical treatment ofexcision choledochal can cyst result consisted in ofAccordingly, recurrent cyst management symptoms enterostomy. of and However, choledochal malignantprovide incomplete cyst transformation a cyst now within review includes the detailing completecyst-type cyst the cyst specific remnant. up excision surgical to whenever approaches, datetherapy, possible. the management and We impact of long-term of choledochal sequelae minimally cysts. of invasive We choledochal surgeryRecent describe cyst in findings choledochal management. choledochal cyst Management of choledochal cysts PINION URRENT O C characteristic (approximately 4 : 1 ratio) [3 adults and children, a female preponderance is We herein review choledochal cystspatient with a presentation focus and on diagnostic modalities, as Conventionally, patientswere with treated choledochal withostomy) cyst cysts decompression in (cystobstruction, order enter- and to jaundicecyst [6]. relieve excision However, with symptoms complete indicated biliary-enteric in of order reconstruction to ameliorate is pain, long-term sequelae. lations (approximately 1 inAlthough 13 000 individuals) predominately [1]. choledochal diagnosed cysts in arequency children, found in with adultsmajority of increasing patients such in fre- recent that series [2,3 adults comprise the Choledochal cysts aremultiple cystic characterized dilatations by ofor the single intrahepatic extrahepatic and/ or occur biliary infrequently ducts. in100 000 Choledochal to Western 1 cysts populations inthe 150 000 (1 considerably individuals) compared in higher with incidence in Asian popu- INTRODUCTION 0267-1379 Copyright

REVIEW

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consequently, many of the results from investi- KEY POINTS gations pertain to these subcategories. Although predominately diagnosed in children, choledochal cysts are found with increasing frequency in adults such that adults comprise the majority of DIAGNOSIS patients in recent series. Nearly 80% of choledochal cysts present in childhood [5&&]. Symptomatic patients typically present MRCP is the diagnostic modality of choice for infants or children and has likely surpassed ERCP as the best with nonspecific abdominal pain. However, com- imaging test for adults given the noninvasive nature plaints suggesting biliary or pancreatic origin and ability to characterize cyst anatomy in relation to including right upper quadrant pain, jaundice, pan- the biliary tree and surrounding structure. creatitis, and nausea and vomiting are also common [2]. Children typically experience abdominal pain, Prior to surgical intervention, control of biliary sepsis or resolution of pancreatitis must occur; additionally, the jaundice or an abdominal mass; however, the classic extent of biliary tree involvement by the choledochal triad consisting of these three aforementioned cyst must be established. symptoms is rare, particularly in adults. Adults generally present with abdominal pain, infectious When determining whether to use a MIS versus open complications, or malignancy (Table 1) [13,14]. A approach, factors to consider include patient age, comorbidity, and type of cyst. recent series evaluating choledochal cysts in pediatric and adult patients identified abdominal Given the complex nature of choledochal cyst and pain as the presenting complaint in 61% of patients, limited experience of most centers, choledochal cyst which was the most common symptom in both patients should be evaluated and treated at high- adults (72%) and children (41%). Pancreatitis and volume HPB centers familiar with management of choledochal cyst. jaundice were also common presentations (18.8% and 18.5%, respectively), whereas 16% of patients were asymptomatic at the time of diagnosis [5&&]. Asymptomatic patients are increasingly identified is substantiated by the increased occurrence of because of frequent use of cross-sectional imaging APBDU among patients with choledochal cyst and currently comprise approximately 10–36% of (approximately 30–96%) compared with 2% in patients with choledochal cysts [15&]. the general population [1,10]. Experimental studies Initial workup for many patients begins with have corroborated the associated between APBDU ultrasound or contrast-enhanced computed tomo- and choledochal cysts; however, this anomaly graphy, and follow-up cholangiography can delin- cannot be solely responsible given the variable inci- eate the cyst type [percutaneous transhepatic dence in patients with choledochal cysts [1,8]. cholangiography, endoscopic retrograde cholangio- The Todani system classifies choledochal cysts pancreatography (ERCP), or magnetic resonance based on anatomic findings and extent of biliary cholangiopancreatography (MRCP)]. MRCP is the involvement (see Fig. 1) [10–12]. Type I cysts are the diagnostic modality of choice for infants or chil- most commonly encountered in both adults and dren. MRCP has likely surpassed ERCP as the best children (60–80%) and demonstrate either cystic imaging test for adults given the noninvasive nature (IA or IB) or fusiform (IC) dilation of the extrahe- and ability to characterize cyst anatomy in relation patic duct. Of note, the intrahepatic biliary tree can to the biliary tree and surrounding structures be dilated in type I cysts as a consequence of biliary [1,11,14,16,17]. However, ERCP and percutaneous stasis. Type II cysts (1–2%) are supraduodenal diver- transhepatic cholangiography are particularly ticulum, whereas type III cysts (choledochocele, beneficial when a therapeutic intervention is 0.5–4%) are intraduodenal. Both malignancy and needed (i.e., in the management of choledochal APBDU are rare in type III choledochal cysts. More- cyst-associated complications such as cholangitis) over, type III choledochal cysts lack the female [11,18]. preponderance characteristic of other choledochal cysts. Type IV cysts are distinguished by multiple extrahepatic (IVB, 1–2%) or both intrahepatic and TREATMENT extrahepatic (IVA, 15–30%) dilation and are the second most common cyst encountered [3&,4]. Type Indications V cysts (Caroli’s disease) involve only the intrahe- Subsequent to diagnosis, treatment of choledochal patic ducts [2]. The vast majority (approximately cysts aims to avoid the numerous hepatic, pancreatic, 90%) of choledochal cysts in both adults and chil- or biliary sequelae that may occur. Acute inflamma- dren are comprised of type I or type IV cysts, and tory conditions include recurrent cholangitis, liver

Type I Type II Type III

Type IVA Type IVB Type V

FIGURE 1. Choledochal cyst classification. Type I cysts are either cystic (IA or IB) or fusiform (IC) dilation of the extrahepatic duct. These are best treated with extrahepatic duct excision and Roux-en-Y hepaticojejunostomy reconstruction. Type II cysts are supraduodenal diverticulum and can be excised via cystectomy or duct excision with restoration of biliary-enteric continuity. Type III cysts (choledochocele) are intraduodenal and should be treated via endoscopic means (smaller cyst) or transduodenal sphincteroplasty. Type IV cysts exist as multiple extrahepatic (IVB) or both intrahepatic and extrahepatic (IVA) dilation. Treatment of these cysts includes extrahepatic duct excision with restoration of biliary-enteric continuity hepatic resection. Type V cysts involve only the intrahepatic ducts and generally require hepatic resection or liver transplantation. (Figure used with permission from Park et al. [11]). abscess, acute pancreatitis, and sepsis; chronic resolution of pancreatitis must occur; in addition, changes, such as hepatic cirrhosis, portal hyperten- the extent of biliary tree involvement by the chol- sion, chronic pancreatitis, and cholangiocarcinoma/ edochal cyst must be established [16]. Although gallbladder cancer may also develop [8,14]. Prior to partial cyst excision with biliary-enteric anastomosis surgical intervention, control of biliary sepsis or may initially alleviate obstructive symptoms, complete duct excision is indicated to circumvent the high rates of pancreatitis, cholangitis, recurrent Table 1. Presenting symptoms in patients with choledochal stricture, and potential malignancy [1]. In high vol- cysts, stratified by age ume series, approximately 90% of patients with Infants (0–1 year) Children Adults malignancy are associated with type I or IVA chol- (1–18 years) (>18 years) edochal cyst, in contrast to the rare occurrence in type II, III, or V [4]. Therefore, the complexity associ- Prenatal diagnosis Abdominal pain Abdominal pain ated with choledochal cyst treatment includes Abdominal mass Jaundice Biliary lithiasis degree and severity of symptoms, type of choledo- Jaundice Cholangitis Jaundice chal cyst present (and perceived rate of malignancy), Acholic stool Asymptomatic Cholangitis operation required for complete cyst excision and Pancreatitis patient comorbidities. Given these considerations Malignancy combined with the rarity of choledochal cyst, oper- Asymptomatic ative management should be determined in a multidisciplinary setting.

Infants and children developing malignancy [2,8]. This risk appears to be Infants (age 0–1 year) may be diagnosed prenatally low in childhood (< 1%), but increases with age to or may present with symptoms resembling biliary an estimated 30–40% of those greater than 50 years atresia including jaundice, hepatomegaly, and [8]. The precise risk of cholangiocarcinoma in acholic stools. The risk of progression to liver fibrosis patients with choledochal cyst is unknown because warrants early excision. In patients who are clini- of the predilection for symptomatic patients to be cally symptomatic (jaundice, acholic stool, emesis, included in studies. and distension), liver damage has been shown In a review examining the occurrence of malig- to occur early despite excision within the first nancy in 5780 patients with choledochal cyst, the 6 months of life [19]. Early cyst excision is also overall incidence of biliary tract cancer was 7.5% appropriate in asymptomatic patients (diagnosed [15&]. The incidence in children (age 0–18) was low prenatally). In a study examining timing of surgery (0.4%) with increased rates in those older than 18 for neonates diagnosed prenatally, those random- years (11%). The incidence steadily increased with ized to cyst excision within 1 month demonstrated age up to 38% in those patients older than 60 [15&]. significantly less fibrosis and cirrhosis compared to Among patients who develop malignancy, 70% those who were randomized to cyst excision after arises as cholangiocarcinoma within the cyst wall 1 month, with no perioperative morbidity or and approximately 24% arises as gallbladder cancer mortality in either group [13]. [15&]. Furthermore, malignant transformation most Children (age 1–18 years) often present with commonly occurs in type I or type IV cysts, with symptoms of abdominal pain, jaundice, or abdomi- decreased frequency in type V cysts. Transformation nal mass, and in contrast to adults, development of is exceedingly rare in type II or III cysts [6]. The malignancy is rare (< 1%) [14]. In a study by Hung association with malignancy also appears to be et al. [14] evaluating infants and children at a single influenced by presence or absence of APBDU. In a institution undergoing cyst excision and hepatico- recent series by Chang and colleagues [9] of 453 jejunostomy (type I or IVA cysts), neonates pre- patients with gallbladder cancer, APBDU was ident- sented with jaundice (100%) and acholic stools ified in 17.2% and all patients with choledochal (75%) versus abdominal pain (82.4%), jaundice cysts who developed gallbladder cancer had APBDU. (29.4%), and abdominal mass (11.8%) in the To better understand the pathogenesis of pediatric population. No patients were identified carcinoma in patients with choledochal cysts, to have malignancy; instead, pathologic evaluation Katabi and colleagues [20] performed a clinicopa- demonstrated only chronic cyst inflammation. thologic study of 36 symptomatic patients who had There were no perioperative deaths and late com- undergone resection (cyst excision pancreatic plications such as stricture, cholangitis, or cholan- resection hepatectomy). The authors noted giocarcinoma did not occur at a follow-up of 8 years. APBDU in 64% of patients and evidence of chronic These results indicate complete cyst excision with inflammation of the cyst wall in 73% of patients. biliary enteric anastomosis is feasible and safe in Forty percent had evidence of metaplasia (pyloric, neonates (both asymptomatic and symptomatic) intestinal, or squamous metaplasia), 29% had and the pediatric population [13,14,19]. biliary intraepithelial neoplasia (six with BilIN-1, two with BilIN-2, and two with BilIN-3) and five patients (14%) had carcinoma. Histologically, meta- Adults plasia was identified in all specimens containing Similar to pediatric patients with choledochal cysts, carcinoma, and three of the five had concomitant the majority of adult patients present with symp- biliary neoplasia and carcinoma in the pathologic toms related to complications from their choledo- specimen. Median survival among patients with chal cyst. However, the most feared complication cholangiocarcinoma was 22 months. Given the remains malignant transformation. Adenocarci- pathologic findings, a metaplasia to neoplasia to noma comprises 73–84% of malignancy associated carcinoma sequence appeared evident for the devel- with choledochal cysts, and additional histologic opment of carcinoma in this patient population subtypes include anaplastic carcinoma (10%), undif- [20]. Although the exact etiology of malignant ferentiated cancer (5–7%), squamous cell carcinoma change remains unclear, pancreatic reflux, biliary (5%), and other (1.5%) [16]. stasis, and mutagenic secondary biliary acids are Cholangiocarcinoma in the general population thought to play a role [21]. is quite rare (incidence of 0.8/100 000 individuals in A recent series by Soares and colleagues [5&&] the United States versus 50–100/100 000 individuals identified 394 patients in multiple Western institu- in Thailand) [7]. In contrast, patients with choledo- tions who underwent operative management of chal cysts are estimated to harbor a 6–30% chance of choledochal cysts. In this series, the majority of

228 www.co-gastroenterology.com Volume 32 Number 3 May 2016 Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. Management of choledochal cysts Ronnekleiv-Kelly et al. patients were adults (65.7%) and the mean age at Among patients who undergo incomplete cyst diagnosis was 45 years for adults and 5.2 years for excision because of an unresected intra pancreatic children. In this patient population, the majority segment, the remnant cyst can still undergo malig- presented with symptoms attributable to the chol- nant transformation. In a retrospective study of 808 edochal cyst (84.5%), whereas 15.5% were asymp- adult patients with choledochal cysts, Lee and col- tomatic. The rate of malignancy at presentation for leagues [4] identified a metachronous extrahepatic the entire cohort was low (3.3%); 91% of the chol- cholangiocarcinoma rate of 5.3% in patients under- edochal cyst-associated cancers arose in patients going cyst enterostomy versus 0.6% in those under- with type I or type IV cysts. An additional 13 going cyst excision (median interval of 52 months). (3.3%) patients (one child, 12 adults) experienced Xia and colleagues [3&] evaluated 41 patients with metachronous malignancy following cyst resection type I or type IVA cysts who underwent incomplete (type I, n ¼ 6; type IV, n ¼ 6; type V, n ¼ 1). Although cyst resection at the initial operation. Approxi- the overall survival for the entire study population mately 14% developed malignancy in the cyst rem- was quite good, patients with cholangiocarcinoma nant as documented at the time of repeat operation experienced poor survival (see Fig. 2) [5&&]. These for completion cyst resection. Repeat operation data emphasize the importance of close follow-up of occurred at a median of 11–12 years following the patients undergoing resection for choledochal cysts. initial resection (performed for symptomatic pur- poses). Of these patients who were found to have malignancy, the majority (83%) expired as a result of their disease [3&]. (a) Age survival analysis Surgical approach 1.0 Historically, the treatment of choledochal cysts 0.8 entailed cyst enterostomy. However, this subopti- mal approach resulted in long-term complications, 0.6 including stricture, jaundice, cholangitis, cholangiocarcinoma, and often times the need for reoper- & 0.4 ation [3 ,22]. To avoid complications from incomplete cyst excision and to minimize the need

0.2 Children (< 18 years) for reoperation, current surgical strategy aims to Log-rank P = 0.09 Adults (≥18 years) Proportion of patients of aliveProportion excise the entire cyst (including gallbladder), fol- 0 lowed by restoration of biliary-enteric continuity. 0 12 24 3648 60 Type I cysts should undergo complete cyst exci- Time (mo) sion with Roux-en-Y biliary-enteric reconstruction. (b) The proximal and distal extent should be identified CCA survival analysis prior to resection to assist with operative strategy. 1.0 Occasionally, the cyst may extend into the pancreatic head in which case the risk of residual chole- 0.8 dochal cyst must be weighed against the risk of pancreaticoduodenectomy [16]. Alternatively, the 0.6 mucosa of a dilated distal common bile duct may be stripped in order to protect the pancreaticobiliary 0.4 junction. Infrequently, because of recurrent epi- sodes of cholangitis, the cyst wall is densely adher- 0.2 CCA ent to the portal vein, precluding safe resection. Log-rank P < 0.001 NO CCA Proportion of patients of aliveProportion When this occurs, resection of the anterior wall with 0 0 12 24 3648 60 careful fulguration of the mucosa of the posterior Time (mo) wall and Roux-en-Y H-J reconstruction can be performed [23]. Depending on the location of the FIGURE 2. Survival analysis. (a) Both children and adults cyst, type II choledochal cyst may undergo simple who underwent resection for choledochal cyst experienced cyst excision or diverticulectomy. Reconstruction excellent 5-year survival (adults 94.6% and children 97.2%). may be necessary if significant narrowing of the bile (b) In contrast, patients who developed cholangiocarcinoma duct is noted. Type III choledochal cyst can be experienced markedly poor overall survival. (Used with managed by endoscopic means (sphincterotomy), permission from TM Pawlik [5&&].) by sphincteroplasty alone, sphincteroplasty with

0267-1379 Copyright ß 2016 Wolters Kluwer Health, Inc. All rights reserved. www.co-gastroenterology.com 229 Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. Biliary tract cyst excision, or pancreaticoduodenectomy. How- lobectomy may be performed. However, in diffuse ever, given the low incidence of malignancy associ- forms, biliary drainage procedures are ineffective at ated with type III cysts, choledochoceles are best preventing recurrent bouts of cholangitis as well as managed with unroofing (endoscopic or transduo- cholangiocarcinoma. In these patients, liver trans- denal sphincteroplasty) or transduodenal excision plantation provides a durable treatment option (larger cysts) [1,6]. [8,25]. Mabrut and colleagues [25] performed a mul- Type IV choledochal cyst is approached differ- ticenter study, which included 155 patients with ently based on presence or absence and location of type V choledochal cysts from Western surgical intrahepatic disease. The extrahepatic bile duct centers. Approximately 90% of patients were symp- should be excised and if the intrahepatic disease is tomatic including abdominal pain, jaundice, GI limited (i.e., left hemiliver), hepatectomy with bleed, and cholangitis. Synchronous cancer was Roux-en-Y reconstruction to the remaining hepatic identified in 5.2% of patients. Appropriate pre- duct can be performed. In a series of 629 patients operative evaluation was critical for determining with choledochal cysts, Xia and colleagues [24] the extent of resection and the authors emphasized identified 198 patients with type IVA cysts. Of these, the importance of complete resection to optimize 59 were able to undergo extrahepatic cyst excision long-term results. The majority of patients possessed with partial hepatectomy and hepaticojejunostomy. unilobar involvement (69%) with a predilection for There was a low rate of perioperative morbidity left-sided disease, whereas 31% had bilobar involve- (15%) and mortality (0%). At a median follow-up ment. Patients underwent either hepatic resection of 42 months, disease-specific survival was 98%, (75%) or liver transplantation (19%), with excellent with a 4.1% reoperation rate and acceptable biliary or good results achieved in 86%. Five-year overall function in 85.8% of patients [24]. However, not all survival was 97% after liver resection and 89% after patients are appropriate candidates for partial hep- liver transplant [25]. atectomy. Those patients with obvious dilations In those with known malignancy, oncologic and stenosis of intrahepatic ducts, intrahepatic principles should apply; patients who can undergo duct stones, or parenchymal atrophy may benefit safe resection with negative margins are appropriate from hepatectomy [4]. These patients appear more for operation. Resection may include hepatectomy susceptible to intrahepatic cholestasis, hepatic with regional lymphadenectomy, extirpation of abscess, recurrent cholangitis, hepaticolithiasis, extrahepatic bile ducts with regional lymphadenec- and potential carcinogenesis [24]. If hepatectomy tomy (and cholecystectomy), or pancreaticoduode- is planned concomitantly with extrahepatic duct nectomy [16]. However, malignancy associated with excision, the distribution should allow removal of choledochal cyst (cholangiocarcinoma or gallblad- all disease (or the vast majority of severe disease) der cancer) is universally associated with poor prog- with adequate future liver remnant. Of note, type I nosis [5&&,25]. choledochal cyst may present with intrahepatic Choledochal cysts can also be addressed via dilatation because of biliary stasis and therefore open excision or minimally invasive surgery differentiating between type I and type IVA chole- (MIS). In general, children are more likely to dochal cyst is critical because of the therapeutic undergo laparoscopic cyst excision compared with implications. However, in patients without indica- adults, and the operative strategy most commonly tions for hepatectomy, type IV choledochal cysts entails Roux-en-Y hepaticojejunostomy. In a multi- can be treated similarly to type I choledochal cysts. institutional investigation of Western patients with This approach is justified by studies demonstrating choledochal cysts, Margonis and colleagues [26&&] that patients who progress to malignancy most noted 36 of 368 patients underwent an MIS commonly develop extrahepatic cholangiocarci- approach, and this cohort was more likely to occur noma or gallbladder cancer (approximately 95% in children (24%) versus adults (2.1%, P < 0.001). of malignancy), whereas intrahepatic cholangiocar- Operations utilized were most often cyst excision cinoma rarely occurs [4]. This consideration may with hepaticojejunostomy (85.4%), cyst excision allow tailoring of surgical resection depending on with hepaticoduodenostomy (7.3%), cyst excision location and distribution in patients with type IV or alone (3.7%), or whipple (2.8%). Complications V choledochal cysts. occurred in 35% of patients, readmission in 27%, Management of patients with Caroli’s disease and reoperation in 12% with no mortality at can be particularly difficult given the location of 30 days. Five-year overall survival was 98.6%. In a cysts and frequent necessity for surgery [consider- propensity-score-matched analysis, there was no able potential for cholangitis, liver complications, difference in complication rate, readmission, or and biliary cirrhosis; moderate potential for neo- reoperation between the MIS group and open plasia (7%)] [1,8,25]. For focal disease, hepatic group, indicating both approaches are safe. When

4. Lee SE, Jang JY, Lee YJ, et al. Choledochal cyst and associated malignant determining whether to use a MIS versus open tumors in adults: a multicenter survey in South Korea. Arch Surg 2011; approach, factors to consider include patient age, 146:1178–1184. 5. Soares KC, Kim Y, Spolverato G, et al. Presentation and clinical outcomes of comorbidity and type of cyst (70% of MIS patients && && choledochal cysts in children and adults: a multiinstitutional analysis. JAMA had type I cyst) [26 ]. In another series by Senthil- Surg 2015; 150:577–584. The study is the largest Western series to evaluate presentation and outcome in nathan et al. [23] 110 patients (55 pediatric and patients with choledochal cysts. The true incidence of malignancy in patients with 55 adult) underwent laparoscopic resection and choledochal cysts is unknown due to the uncertain true denominator. However, this investigation reveals that the incidence is probably lower than expected (3%) reconstruction of type I or type IVA choledochal in those undergoing resection. What is interesting is the number of patients who cysts. The overall mortality was 1%, reoperative rate developed malignancy after cyst resection (3%). Therefore, this study demonstrates that the risk of malignant transformation in Western populations with was 2% and morbidity 10%, demonstrating the choledochal cysts is likely lower than previously thought but that there is a safety and feasibility of laparoscopic approach in significant risk after cyst excision indicating surveillance is appropriate. 6. Martin RF. Biliary cysts: a review and simplified classification scheme. Surg specialized, high-volume centers [23]. Clin North Am 2014; 94:219–232. 7. Tyson GL, El-Serag HB. Risk factors for cholangiocarcinoma. Hepatology 2011; 54:173–184. 8. Soreide K, Soreide JA. 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Todani T, Watanabe Y, Narusue M, et al. Congenital bile duct cysts: cations associated with partial cyst resection classification, operative procedures, and review of thirty-seven cases includ- mandates complete resection of the cyst when feas- ing cancer arising from choledochal cyst. Am J Surg 1977; 134:263–269. 13. Diao M, Li L, Cheng W. Timing of surgery for prenatally diagnosed asympto- ible. Management of the choledochal cyst and the matic choledochal cysts: a prospective randomized study. J Pediatr Surg operative conduct will depend upon the patient 2012; 47:506–512. 14. Hung MH, Lin LH, Chen DF, Huang CS. Choledochal cysts in infants and comorbidities and choledochal cyst subtype. How- children: experiences over a 20-year period at a single institution. Eur J Pediatr ever, given the complex nature of choledochal cysts 2011; 170:1179–1185. and limited experience of most centers, these 15. Sastry AV, Abbadessa B, Wayne MG, et al. What is the incidence of biliary & carcinoma in choledochal cysts, when do they develop, and how should it patients should be evaluated and treated at high- affect management? World J Surg 2015; 39:487–492. This compilation of numerous Eastern and Western studies evaluating choledo- volume hepatopancreaticobiliary centers familiar chal cysts provides insight to the overall rate of malignancy seen (7.5%) in the with management of choledochal cysts. entire population and the association with increasing age. 16. Machado NO, Chopra PJ, Al-Zadjali A, Younas S. Choledochal cyst in adults: etiopathogenesis, presentation, management, and outcome-case series and Acknowledgements review. Gastroenterol Res Practice 2015; 602591:1–10. 17. Sacher VY, Davis JS, Sleeman D, Casillas J. Role of magnetic resonance None. cholangiopancreatography in diagnosing choledochal cysts: Case series and review. World J Radiol 2013; 5:304–312. 18. Kim SH, Lim JH, Yoon HK, et al. 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