Two cases of chronic pancreatitis associated with anomalous pancreaticobiliary ductal union and SPINK1 mutation
A 2 year 8 month old girl presented with intermittent severe right upper abdominal pain for six days. In spite of fasting and conservative care, her pain remained. Choledochal cyst was found in ultrasonography. She was referred to our hospital for further evaluation and management of choledochal cyst. Magnetic resonance cholangiopancreatography (MRCP) showed a choledochal cyst (type IVa) with APBDU (P-C type) and bile sludge in the CBD and common channel (). Hepaticojejunostomy with jejunostomy and excision of choledochal cyst was performed. At the routine checkup two years later, blood test results of serum amylase and lipase levels were 210 U/L and 125 U/L. MRCP was done again which showed focal dilatation of the upstream pancreatic duct and pancreatic duct stone. Endoscopic retrograde cholangiopancreatography (ERCP) was performed and the pancreatic duct stone was removed (). To evaluate the etiology of CP, screening tests including infection and autoimmune disorders were performed and a genetic study was done. Genetic testing revealed mutations in CP: p.N34S mutation in the SPINK1 gene ().