Congenital pancreatic cysts are extremely rare in newborns. The case of a neonatal congenital pancreatic cyst with pancreatitis is reported. The rapid increase in cyst size concomitantly with clinical symptoms prompted surgical management. Intraoperatively, the pancreatic head showed signs of chronic pancreatitis, and in attempts to preserve most of the functional pancreatic tissue, a Roux-en-Y cystojejunostomy was performed. Histology demonstrated a true pancreatic cyst with degenerated epithelial lining.
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