COMPREHENSIVE REVIEW OF ADULT PATIENTS DIAGNOSED WITH CHOLEDOCHAL CYST IN THE WESTERN ADULTS: PATIENT CHARACTERISTICS AND MALIGNANCY RATES
Inochi Gonzalez Calvo*1, Grace C. Bloomfield1, Aradhya Nigam2, Pejman Radkani2, Emily Winslow2
1Medical Student, Georgetown University School of Medicine, Washington, ; 2Georgetown University Medical Center, Washington,
Introduction:
The diagnosis and management of patients with choledochal cysts remains controversial with most current practice patterns based on retrospective data from East Asian countries. Due to relatively rare prevalence in Western countries, understanding of the outcomes and rates of malignancy in Western countries remains limited, especially in adults. We aimed to perform a systematic review of adult patients diagnosed with choledochal cysts in Western countries to better guide management and highlight gaps in understanding.
Methods:
This systematic review was conducted in accordance with the PRISMA guidelines. A literature search in the electronic bibliographic databases MEDLINE via Ovid, Embase via Ovid, and Web of Science was performed on May 17, 2022. Articles were prescreened to exclude studies not meeting predefined population or study-type criteria, followed by comprehensive title/abstract and full-text screening for content and relevance with two blinded independent reviewers (figure 1). Studies of fewer of than twenty adults with CC were excluded and the Newcastle-Ottawa Scale (NOS) was employed for quality assessment. Data on baseline characteristics, surgical details, complications, malignancy rates, and follow-up were extracted and analyzed.
Results:
Of 3,488 articles retrieved, 23 clinical surgical studies evaluated Western adult patients diagnosed with choledochal cysts. Amongst 1,437 patients included in the studies (table 1), 77% were female with a mean age of 34 years. Mean follow-up for the cohort was 6.5 years (mean range 1.7-25 years) post-operatively. The most common Todani subtypes included Type I (61.2%) and Type IV (20%). Symptoms at presentation included abdominal pain (74%), jaundice (31%), cholangitis (26.9%), and pancreatitis (15%) while 14.6% were reported to be asymptomatic. Diagnostic testing for choledochal cyst varied by study and involved ultrasound in majority of cases (73.4%) but included CT/MRCP in 37-79% of cases and ERCP in 43-80%. Importantly, the rate of malignancy in pathologically evaluated surgical specimens was 13% (range 3.1-29.0%). Amongst cancer types, cholangiocarcinoma (88%) was the most prevalent, followed by gallbladder adenocarcinoma (9%), while other cancers reported included bile duct sarcoma, biliary squamous cell carcinoma, and undifferentiated/anaplastic carcinoma (~3%).
Conclusion:
This comprehensive review of the published literature to date demonstrates that the prevalence of malignancy in adult patients in Western centers with resected choledochal cysts remains concerning (13%). Currently, the optimal diagnostic approach and management of adult patients with dilated bile ducts remains unestablished and varies by institution. Additional study is needed to aid in accurate diagnosis and help with risk stratification for malignancy in adult patients with choledochal cysts.
Figure 1: A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) Flow Diagram for Identification and Selection of Articles
Table 1: Characteristics and Findings of the 23 Studies Included for Review and Analysis
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