Cavernous Transformation of Portal Vein in the Setting of Protein C and Anti-thrombin III Deficiency

Cavernous transformation of the portal vein (CTPV), also known as portal cavernoma, is a sequelae of thrombosis in the portal vein causing its occlusion and portal hypertension. The etiology, however, remains unknown. Gastroesophageal variceal bleeding, splenomegaly, portosystemic collaterals, and ultimate hematologic abnormalities are among the prominent clinical features. Among the causes, predisposing an individual to CTPV is natural anticoagulant protein C and antithrombin III deficiencies. Determination of the etiology of CTPV may also give a direction toward the management plan to not only relieve the patient of the already developed complications but also to treat the primary cause of the pathology  We discuss a case of a nine-year-old male child diagnosed as CTPV secondary to protein C and antithrombin III deficiency who was treated symptomatically for anemia and varices and was referred for transjugular intrahepatic portosystemic shunt (TIPS).