Marked recent weight loss is a major clinical manifestation, and unresponsiveness of the ascites to diuretics is an additional diagnostic clue.

If the duct is disrupted posteriorly, the secretions leak through the retroperitoneum into the mediastinum via the aortic or esophageal hiatus.

Once in the mediastinum, the secretions can either be contained in a mediastinal pseudocyst, lead to enzymatic mediastinitis, or, more commonly, leak through the pleura to enter the chest and form a chronic pancreatic pleural effusion.

Serum amylase is often elevated as well, due to enzyme diffusion across the peritoneal or pleural surface.

[1][2] Contrast-enhanced computed tomography and endoscopic retrograde cholangiopancreatography (ERCP) may also assist in diagnosis, with the latter an essential component of treatment.