Choledochal cysts are related to significant complications, such as pancreatitis, cholangitis, secondary biliary cirrhosis, and spontaneous rupture of the cyst or cholangiocarcinoma. Choledochal cysts generally rupture spontaneously in children aged <4 years.[20,21] To our knowledge, this is the first reported case of spontaneous perforation of concomitant types VI and I choledochal cysts. Pancreatitis, proteinaceous plugs, or stones within a dilated common channel may be related to pancreaticobiliary malunion.[22–24] Komuro et al reported that acute pancreatitis and protein plug formation were found more frequently in children between ages 1 and 15 years than in adults with choledochal cysts.
To date, few studies have discussed the optimal timing of surgery for choledochal cysts complicated with acute pancreatitis in children. Cho et al divided patients aged ≤15 years into 3 groups according to timing of surgery after diagnosis: ≤7 days (group A), 8 to 30 days (group B), and ≥31 days (group C). Length of operation, days of hospitalization, time to commencement of diet after operation, and change in hemoglobin concentration were similar in the 3 groups. The authors suggested that early cyst excision after the development of acute pancreatitis in patients with choledochal cysts reduced patients’ symptoms, especially in instances of pseudopancreatitis. However, at the time of operation in their study, even the highest amylase or lipase level of the patients in the 3 groups did not exceed 309 U/L. In addition, determining the timing of surgery in choledochal cyst complicated with acute pancreatitis and spontaneous perforation is challenging. Here, we report our experience in treating spontaneous perforation of concomitant types VI and I choledochal cysts with pancreatitis.
Choledochal cysts are congenital disorders of the CBD that can cause progressive biliary obstruction and biliary cirrhosis. Type VI choledochal cysts are rarely reported in the literature. To our knowledge, we present herein the first reported case of concomitant types I and VI choledochal cysts complicated with acute pancreatitis and spontaneous perforation.
The authors would like to thank Dr. Jen-Wei Tsai (Department of Pathology, E-Da Hospital, I-Shou University) because of his help of pathological picture and description in this case report.
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