특발성 문맥압항진증 환자에게 병발된 간문맥 및 상장간막정맥 혈전증 1 예

Idiopathic portal hypertension (IPH) presents with splenomegaly, pancytopenia, and variceal bleeding, but shows no hepatic dysfunction. Although the absence of extrahepatic portal vein obstruction is required for diagnosis of IPH, cases associated with extrahepatic portal vein thrombosis have been repeatedly reported. We recently experienced a case of IPH, which developed portal and superior mesenteric vein thrombosis (PMVT). A 27-year-old woman, who had presented with dizziness and received splenectomy after diagnosis of IPH at age 22, was admitted for melena. Esophageal and fundic varices were observed. Spiral abdominal computed tomography (CT) showed long segmental PMVT. After endoscopic variceal ligation, anticoagulation therapy was performed for PMVT. Follow-up CT showed almost complete resolution of PMVT. We report a case of IPH associated with extrahepatic portal vein thrombosis for the first time in Korea.