Portal Cavernoma in a Patient with Crohn’s Disease Associated with Factor V Leiden Mutation and Antiphospholipid Syndrome

Abstract

Portal vein thrombosis followed by the occurrence of a portal cavernoma is a rare condition that requires convergence between systemic predisposition and local factors in order to develop. Among the complications of inflammatory bowel diseases, such eventuality is quite unusual. Ulcerative colitis accounts for the majority of these vascular complications, while in Crohn's disease the occurrence of such events is exceptional. We present the case of a young male patient aged 42, without any particular history, in whom the diagnosis of the first flare of colonic Crohn's disease was accompanied by the discovery of a portal cavernoma, followed by the demonstration of a heterozygous mutation factor V Leiden associated with the presence of an antiphospholipid syndrome with IgG anticardiolipin antibodies. This unusual association of portal vein thrombosis with antiphospholipid syndrome in Crohn's disease, observed according to our knowledge only in one other instance, raises the discussion regarding the convergent action of general thrombophilic (hereditary and acquired) and local factors in the genesis of vascular complications of inflammatory bowel diseases.