A. Sekioka1, K. Fukumoto1, M. Yamoto1, T. Takahashi1, K. Nakaya1, A. Nomura1, Y. Yamada1, N. Urushihara1 1Shizuoka Children’s Hospital,Pediatric Surgery,Shizuoka City, SHIZUOKA, Japan
Introduction: The classification of congenital choledochal cysts proposed by Alonso-Lej or Todani has been widely used. However, this classification did not include the concept of pancreaticobiliary maljunction (PBM). The Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) defined a new classification for PBM in 2015. Our aim is to identify the clinical features of the new PBM classification, including the diagnosis and surgical outcomes.
Methods: The hospital records were retrospectively reviewed. From 2001 to 2017, 91 children underwent excision of the extrahepatic duct for congenital choledochal cyst with PBM. According to the new classification, they were divided into 4 types (A, B, C, and D, shown as figure 1), based on the intraoperative cholangiography. The number of patients in each group was 42, 20, 26, and 3, respectively. Statistical test was performed between type A, B, and C about patients’ demographics and operative outcomes. Type D was excluded from the statistics, because the number was relatively small. Additionally, we evaluated the feasibility of the PBM diagnosis by multi-detector computed tomography (MD-CT) since 2008.
Results:There was no significant difference in the background among three groups (age, sex, body weight, and follow-up period). Prenatal diagnosis was only given in type A (36%, p<0.01). Amylase level in the bile was similar in three groups (91300-127000 IU/l, p=0.77). Abdominal pain or vomit was significantly often observed in type B or C (p<0.01). In contrast, jaundice or acholic stool was significantly found in type A (p=0.02, 0.03). Protein plug in the bile duct was more frequently observed in type C (70%, p<0.01). The rate of biliary perforation or impending perforation was also significantly high in type C (35%, p<0.01). There was no significant difference in the operative results (method, operative time, blood loss, postoperative length of stay, and short or long term complications). Regarding type D, 2 out of 3 patients had the comorbidity, such as annular pancreas and trisomy 21. The accuracy of PBM classification by MD-CT was 72% (36/50, between 2008 and 2017). In type A, 7 patients with the large choledochal cyst (45-170cm) could not reveal the PBM. Six patients had unclear CT images of the PBM. Type D (1 patient) could not be identified by MD-CT.
Conclusion:In the new classification, several characteristics were found in each type of PBM. This classification is simple and correlates with clinical features. In the present study, MD-CT is likely to play an important role to classify the PBM.
