M. M. Dua1, C. W. Jensen1, P. J. Worth1, J. A. Norton1, G. A. Poultsides1, M. Bruzoni2, B. C. Visser1 1Stanford University,Surgery,Palo Alto, CA, USA 2Stanford University,Pediatric Surgery,Palo Alto, CA, USA
Introduction: The Todani classification for choledochal cysts (CC) is based on five anatomical types that include 8 alphanumeric subtypes that are difficult to remember and often misused. Although the Todani classification has been widely adopted for 40 years, it includes a variety of diseases within the biliary tree which carry varying risk profiles for cancer and different surgical treatments. In addition, there are CCs that do not fit this classification system. We hypothesize that the Todani classification has limited use in accurately describing choledochal cysts.
Methods: The clinical and operative findings of 79 adult and pediatric patients who were treated for CCs were retrospectively analyzed. The dates for the adult cases was from 1979 to 2017 and for the pediatric cases was from 1998 to 2013. All available preoperative imaging and radiology reports were reviewed to verify the morphologic condition of the presenting biliary anomalies.
Results: In the adult patients (n=42), the median age was 41 years and 88% were female. In pediatric patients (n=39), the median age was 4 years and 62% were female. The most common presenting symptom in adult patients was pain (71%) and in pediatric patients was jaundice (44%). Eight pediatric patients had a CC in association with biliary atresia and were excluded from categorization into Todani types. Based on the Todani classification, we found 57 cases of type I (78%) and 8 cases of type IVA (11%) which made up 89% of the total cases examined. The remaining CC cases (11%) did not fit into the other categories of Todani type II, III, or V and are listed in Table 1. In 30% of patients, reference to type I and type IVA or “type I/IV” were all used interchangeably in describing the CC despite clear assessment on radiographic imaging.
Conclusion: The Todani classification is based on site of cystic change but is not practical and falsely groups together different abnormalities of the biliary tree. Types I and IV are variations of the same disease and should be combined as the definition of choledochal cysts. The other conditions should be described by their common names rather than referred to as subtypes of CC. The entire spectrum of congenital choledochal cysts are not incorporated into the Todani scheme. A new descriptive characterization is required that can be applied to all biliary anomalies.