Multivisceral transplantation for portal hypertension and diffuse mesenteric thrombosis caused by protein C deficiency.

Background. Protein C is a vitamin K-dependent glycoprotein synthesized in the liver. Homozygous deficiency usually manifests as purpura fulminans in infancy and is often fatal. An unlikely, but potentially life-threatening, manifestation of protein C deficiency is mesenteric venous thrombosis. Methods. A patient with undiagnosed familial protein C deficiency and a history of intestinal infarction developed refractory duodenal and jejunal variceal bleeding as a result of diffuse visceral splanchnic thrombosis and portal hypertension. Because his life-threatening bleeding was unresponsive to all therapies, we performed multivisceral transplantation. Results. Multivisceral transplantation cured the patient's underlying disease and was lifesaving. Functional protein C, undetectable before transplantation, was normal after transplantation. He was discharged tolerating a normal diet and is alive at home 6 months later. Conclusions. Multivisceral transplantation should be considered as a treatment option for patients with diffuse mesenteric thrombosis, even in the absence of short gut syndrome, when portal hypertension causes life-threatening complications unresponsive to less aggressive therapies.