Thomas M. Fishbein

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. 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. 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. 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.

Late technical complications of composite liver/small bowel transplantation procedures are often complex and have not been well defined. Here we describe the unusual presentation and management of two cases of recurrent thrombocytopenia due to hypersplenism resulting from portacaval shunt stenosis. Both patients presented with portal hypertension late after composite liver/small bowel transplantation. One patient presented with recurrent bouts of upper gastrointestinal hemorrhage and was ultimately found to have a stenosis of her native portacaval shunt. After unsuccessful balloon dilatation of the anastomosis, a successful side-to-side distal splenorenal shunt was performed. The second patient presented with severe thrombocytopenia, the etiology of which was determined to be a short segment occlusion of the inferior vena cava between the native portacaval shunt and the piggyback outflow anastomosis of the liver graft. Total caval occlusion prevented balloon dilatation; the patient was relisted for transplantation but died of chronic rejection four months later. Recurrent portal hypertension is challenging in patients who have had combined liver/small bowel transplantation. Surgeons performing intestinal transplantation need to be increasingly aware of these possible late complications.