REVIEW CURRENT OPINION Management of choledochal cysts Sean M. Ronnekleiv-Kelly, Kevin C. Soares, Aslam Ejaz, and Timothy M. Pawlik Purpose of review Historically, surgical treatment of choledochal cyst consisted of cyst enterostomy. However, incomplete cyst excision can result in recurrent symptoms and malignant transformation within the cyst remnant. 09/20/2020 on BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3pxNPODIEKBpIl4WIJuUC3wZcoMyoyOwWjpQO3AzJmNqjsWy7MP1HEA== by https://journals.lww.com/co-gastroenterology from Downloaded Downloaded Accordingly, management of choledochal cyst now includes complete cyst excision whenever possible. We provide a review detailing the up to date management of choledochal cysts. We describe choledochal from cyst-type specific surgical approaches, the impact of minimally invasive surgery in choledochal cyst https://journals.lww.com/co-gastroenterology therapy, and long-term sequelae of choledochal cyst management. Recent findings Treatment of choledochal cyst aims to avoid the numerous hepatic, pancreatic, or biliary complications that may occur. More recently, minimally invasive approaches are being used for the treatment of choledochal cyst with acceptable morbidity and mortality. Moreover, long-term follow up of choledochal cyst patients after resection has demonstrated that although the risk of biliary malignancy is significantly decreased after choledochal cyst resection, these patients may remain at a slightly increased risk of biliary malignancy by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3pxNPODIEKBpIl4WIJuUC3wZcoMyoyOwWjpQO3AzJmNqjsWy7MP1HEA== even after excision. Summary Management of choledochal cyst and the operative conduct will depend upon the patient comorbidities and choledochal cyst subtype. However, given the complex nature of choledochal cyst and limited experience of most centers, these patients should be evaluated and treated at high-volume hepatopancreaticobiliary centers familiar with management of choledochal cyst. Keywords cholangiocarcinoma, choledochal cyst, minimally invasive choledochal cyst resection INTRODUCTION well as the current indications and management Choledochal cysts are characterized by single or strategy for choledochal cyst. multiple cystic dilatations of the intrahepatic and/ or extrahepatic biliary ducts. Choledochal cysts BACKGROUND occur infrequently in Western populations (1 in 100 000 to 1 in 150 000 individuals) compared with The precise etiology of choledochal cysts is not well the considerably higher incidence in Asian popu- understood, although anomalous pancreaticobili- lations (approximately 1 in 13 000 individuals) [1]. ary duct union (APBDU) is a generally accepted Although predominately diagnosed in children, hypothesis [1]. A long common channel (>10 mm choledochal cysts are found with increasing fre- proximal to ampullary sphincter) is thought to pre- quency in adults such that adults comprise the dispose to pancreatic enzyme reflux into the biliary majority of patients in recent series [2,3&,4]. In both tree with consequent biliary duct inflammation and adults and children, a female preponderance is pressure, leading to duct dilation [7–9]. The theory on & && 09/20/2020 characteristic (approximately 4 : 1 ratio) [3 ,5 ]. Conventionally, patients with choledochal cysts Department of Surgery, Division of Surgical Oncology, Johns Hopkins were treated with cyst decompression (cyst enter- Hospital, Baltimore, Maryland, USA ostomy) in order to relieve symptoms of pain, Correspondence to Timothy M. Pawlik, MD MPH PhD, Chief, Division of obstruction, and jaundice [6]. However, complete Surgical Oncology, Professor of Surgery and Oncology, Department of cyst excision with biliary-enteric reconstruction is Surgery, 600 N. Wolfe Street, Blalock 688, Baltimore, MD 21287, USA. indicated in order to ameliorate long-term sequelae. Tel: +410 502 2387; fax: +410 502 2388; e-mail: [email protected] We herein review choledochal cysts with a focus on Curr Opin Gastroenterol 2016, 32:225–231 patient presentation and diagnostic modalities, as DOI:10.1097/MOG.0000000000000256 0267-1379 Copyright ß 2016 Wolters Kluwer Health, Inc. All rights reserved. www.co-gastroenterology.com Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved. Biliary tract 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 child- hood [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 226 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. 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