Chronic Extrahepatic Portal Vein Occlusion in a Young Patient.

Extrahepatic portal vein obstruction (EHPVO) is a rare condition characterized by the occlusion or narrowing of the portal vein outside the liver. We present a case report of a patient with EHPVO secondary to combined protein C and S deficiency and pancytopenia secondary to hypersplenism, highlighting the clinical presentation, diagnostic challenges, and management strategies. Early recognition of this condition and prompt initiation of appropriate treatment can prevent life-threatening complications such as variceal bleeding and portal hypertension. This case underscores the need for a high index of suspicion for inherited thrombophilias in patients presenting with portal vein thrombosis, particularly in the absence of traditional risk factors.