Recombinant Factor VIIa Use for Endoscopic Retrograde Cholangiopancreatography With Sphincterotomy in a Patient With Choledocholithiasis and Unusual Coagulopathy.

Endoscopic retrograde cholangiopancreatography (ERCP) is a procedure that combines the use of endoscopy and fluoroscopy to diagnose and treat pancreaticobiliary disorders. The risks of ERCP include pancreatitis, infection, bleeding and perforation. Bleeding during ERCP typically develops after sphincterotomy, hence patients should be screened and tested for coagulopathy before undergoing ERCP. Coagulopathy is a major risk factor for ERCP-related bleeding. Inherited factor VII (FVII) deficiency is a rare autosomal recessive hemorrhagic disorder that can lead to significant coagulopathy and severe bleeding if not appropriately recognized and treated preoperatively. Clinically, the disease ranges between an asymptomatic state to lethal hemorrhage and the degree of FVII deficiency does not correlate with the severity of bleeding. The use of FVII replacement therapy has been reported to prevent bleeding during surgery. We present the first report of a patient with a rare cause of coagulopathy due to inherited FVII deficiency who successfully underwent ERCP with sphincterotomy without bleeding where we used recombinant factor VIIa before and after the procedure.