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