Two cases of chronic pancreatitis associated with anomalous pancreaticobiliary ductal union and SPINK1 mutation
A 4 year 6 month old girl with five days of periumbilical abdominal pain and vomiting visited a local clinic. An initial workup which was remarkable for elevated amylase and lipase (1,135 U/L and 2,950 U/L, respectively) confirmed the diagnosis of acute pancreatitis. She was brought to Severance Children's Hospital for further evaluation of pancreatitis. After conservative care for fifteen days, blood test results showed normal levels of amylase and lipase. After five days of normalization, abdominal pain recurred. Amylase and lipase were re-elevated (251 U/L and 1,259 U/L, respectively). The abdominal pain prompted a comprehensive evaluation for CP. MRCP demonstrated pancreatitis with pancreas swelling and pancreatic duct dilatation. Ectopic insertion of CBD and ventral pancreatic duct and pancreatic divisum was seen (). ERCP was performed and EST of major papilla and minor papilla was done instead of operation. ERCP demonstrated incomplete pancreas divisum and APBDU. The major papilla of the duodenum was not remarkable. Selective deep cannulation of the bile duct was done successfully and the sphincterotomy was performed with pull type sphincterotome. On the cholangiography obtained by the major papilla selective cannulation, the bile duct was observed to have no dilation or obstruction. However, the main pancreatic duct was visualized just 1.5 cm distal to the ampulla and joined with CBD. On the minor papilla, the minor pancreatic duct was communicated to the body and tail of the pancreas with dilation. After two months of EST, MRCP was done again and demonstrated a decreased caliber of the dorsal pancreatic duct and no remarkable changes of CBD, intrahepatic duct, or ventral pancreatic duct. Pancreatic enzymes were checked in an outpatient clinic and the enzyme levels presented in a wax and wane pattern. Genetic testing revealed SPINK1 IVS3+2T>C splicing mutations ().