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

Abstract

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.

Tratamento minimamente invasivo da coledocolitíase e suas complicações: relato de caso

“…A técnica de pré corte em si estaria inclusive mais relacionada ao risco de hemorragias do que perfurações (12) . As coagulopatias, histórico de cirrose e a CPRE com fins terapêuticos estariam associados a maior risco de sangramento pós procedimento (13)(14) .…”

ERCP-Related Bleeding

“…The bleeding risk also depends on the patient's underlying disorder and the availability of interventions to enhance hemostasis (e.g., factor replacement for hemophilia, platelet transfusions for thrombocytopenia). Use of recombinant human factor VIIa as a preventive measure has also been reported in a patient with inherited factor VII deficiency who successfully underwent ERCP with sphincterotomy without bleeding (Abdulsamad et al, 2017). Infusion of terlipressin in four patients (Tyagi et al, 2009) and infusion of nitro-glycerine in another case report (Sharma 2010) were effective in controlling hemobilia during therapeutic ERCP in patients with portal biliopathy.…”