Loeys-Dietz Syndrome Presenting as Hemorrhage From Arteriobiliary Fistula: Endoscopic Management of Hemostasis

TL;DR

A patient with Loeys-Dietz Syndrome and a complex vascular condition was successfully treated with a metallic stent to stop severe bleeding from an arteriobiliary fistula.

Contribution

The paper introduces the novel use of a fully covered metallic stent for hemostasis in a challenging case of arteriobiliary fistula.

Findings

A fully covered metallic stent achieved hemostasis in a patient with Loeys-Dietz Syndrome and arteriobiliary fistula.

Embolization of hepatic artery branches failed to control the hemorrhage in this complex case.

Endoscopic retrograde cholangiopancreatography was used to place the stent successfully.

Abstract

Loeys-Dietz syndrome is a rare autosomal dominant connective tissue disorder with an increasing risk of vascular aneurysm formation and dissection. In this report, we discuss the novel therapeutic use of a fully covered metallic stent for management of severe refractory hemorrhage from arteriobiliary fistula in a case complicated with Loeys-Dietz Syndrome and post thoracic aneurysm repair. Recent history of pancreaticoduodenectomy added on the anatomy complexity, and embolization of hepatic artery branches proved unsuccessful. Hemostasis was achieved after a metallic stent placement in the common bile and hepatic duct via endoscopic retrograde cholangiopancreatography.

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