Abstract
Background The choledochal cyst (CC) is a rare cystic dilatory condition with malignant tendency, which is more frequently reported in children. Surgical resection of cysts can significantly decrease the risk of malignancy and reduce associated complications. However, CC has been paid lesser attention in adults, and its surgical parameters have been frequently reported to be in dispute. This study aimed to report experience associated with the treatment of an adult with CC and to suggest the appropriate parameters for the surgery, including the extent of excision (complete or not), the length of the Y limb, the diameter of the cholangio-intestinal anastomosis (CIA), and different operative approaches (open, laparoscopic, and laparoscopic converted to open) by comparing the various indicators, including postoperative bile leakage, cholangitis, choledocholithiasis, carcinogenesis, and surgical re-excision. Methods We conducted a single-center noninterventional retrospective study of 69 different congenital choledochal cyst patients who were admitted to our hospital between July 2010 and July 2020. We collected and analyzed their demographic data, clinical presentations, underlying complications, imaging tests, endoscopic interventions, and parameters for the surgery, histological data, and prognostic indicators over a mean 77-month follow-up period. Results We found that out of the 69 cases, the median age at diagnosis was 32 (IQR = 22–45) years. Seven (10.1%) patients were asymptomatic before the diagnosis, with abdominal pain as the primary complaint in 62 (89.9%) patients, whereas nausea/vomiting was observed in 29 (42.0%) patients. CCs were mainly evaluated by using magnetic resonance cholangiopancreatography (MRCP) (n = 47, 68.1%). It was observed that surgery, cholecystectomy, choledochal cysts excision, and Roux-en-Y hepaticojejunostomy (n = 65, 94.2%), and laparotomy (n = 58, 84.1%) were the dominant therapeutic modalities employed. However, seventeen (24.6%) patients were treated with incomplete cyst resection, while 52 (75.4%) patients received complete cyst resection. We also conducted regular follow-ups after the surgery for a mean duration of 77 months. Postoperative complications were found to be experienced by 35 (50.7%) patients, and a further two patients (2.9%) developed malignancy during the follow-up. Moreover, increasing the diameter of cholangio-intestinal anastomosis served as a potential protective factor for postoperative choledocholithiasis (p = 0.040) and a risk factor for cholangitis (p = 0.002). Conclusions Among the 69 CC participants, abdominal pain was their major symptom. Those with an abnormal pancreaticobiliary junction were more likely to have choledocholithiasis and pancreatitis. The diagnosis was found to be highly dependent on the Todani classification scheme and MRCP. Surgical resection remains the key to CC treatment. The results suggested that the complete resection, the length of the Y limb of 40 cm–60 cm, and the diameter of the CIA of 1.0 cm–1.5 cm were appropriate values for predicting the lower risk of postoperative complications.
Highlights
Congenital choledochal cyst (CC) is a relatively rare inborn bile duct abnormality that is predominantly characterized by cystic dilation of the biliary tract that is usually accompanied by an abnormal pancreaticobiliary junction (APBJ) [1]
Ere are five different types of CC as described in the Todani classification system [2], among which type I and IV Journal of Oncology cysts have been found to be more prevalent in Asian populations and more frequently associated with the presence of APBJ [3]. e classical presentations of this disease include nausea/vomiting and other important elements of the triad of fever, abdominal pain, and jaundice
Total cyst resection has been advocated for management [7] because subsequent biliary cancer and postoperative complications such as lithiasis could be prone to develop in the cyst remnant [8, 9]
Summary
Congenital choledochal cyst (CC) is a relatively rare inborn bile duct abnormality that is predominantly characterized by cystic dilation of the biliary tract that is usually accompanied by an abnormal pancreaticobiliary junction (APBJ) [1].ere are five different types of CC as described in the Todani classification system [2], among which type I and IVJournal of Oncology cysts have been found to be more prevalent in Asian populations and more frequently associated with the presence of APBJ [3]. e classical presentations of this disease include nausea/vomiting and other important elements of the triad of fever, abdominal pain, and jaundice. Total cyst resection has been advocated for management [7] because subsequent biliary cancer and postoperative complications such as lithiasis could be prone to develop in the cyst remnant [8, 9]. It might not be technically feasible to completely remove the cysts because of their intrahepatic or intrapancreatic nature [10] In this case, some surgeons prefer to conduct only partial hepatectomy [11] or intramural dissection of the intrapancreatic biliary cyst [12] to achieve complete excision. Erefore, we have focused on the surgical management and clinical outcome of choledochal cysts in Asian grown-ups with an aim to provide an experience reference for the standardization of treatment modalities for adult patients with CC The poor prognosis of CC such as ascending inflammation and anastomotic stricture has been reported to be associated with an inappropriate length of Y limb or diameter of cholangio-intestinal anastomosis [14]. erefore, we have focused on the surgical management and clinical outcome of choledochal cysts in Asian grown-ups with an aim to provide an experience reference for the standardization of treatment modalities for adult patients with CC
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