title: Cavernous transformation of the portal vein: etiology determines the outcome.
abstract: OBJECTIVE
  Cavernous transformation of the portal vein is a rare condition with various etiologies and diverse clinical presentations. We aim to assess the disease outcome of different etiologic groups.
  
  
  METHODS
  We retrospectively scrutinized the clinicopathologic characteristics of 35 patients with cavernous transformation of the portal vein, placing special emphasis on the disease extent, progression, and outcome after stratification into different etiologic groups.
  
  
  RESULTS
  There were 20 males and 15 females with a mean age of 40.4 years. The etiologies of these patients could be classified into four groups including hematologic disease (n=12, 34.2%), malignant disease (n=8, 22.9%), non-malignant disease (n=6, 17.2%), and idiopathic (n=9, 25.7%). The hematologic disease group had 9 patients with myeloproliferative disease and 3 patients with protein C or protein S deficiency. The hematologic disease group survived well after medication or surgical management. Hepatocellular carcinoma (n=6) was the leading cause in the malignant diseases group, which had a significantly poorer survival rate than the rest (p<0.05).
  
  
  CONCLUSIONS
  Cavernous transformation of the portal vein is a long-term sequele of portal vein thrombosis in which the etiology determines the outcome. More aggressive management including long-term anticoagulation in patients with myeloproliferative disorder or underlying prothrombotic states and endoscopic therapy in controlling variceal bleeding may be a more effective treatment for these patients.
authors.affiliation: Department of Internal Medicine, College of Medicine National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Chi-Yang
authors.initials: CY
authors.lastname: Chang
authors.forename: Pei-Ming
authors.initials: PM
authors.lastname: Yang
authors.forename: Shih-Pei
authors.initials: SP
authors.lastname: Hung
authors.forename: Woei
authors.initials: W
authors.lastname: Tsay
authors.forename: Lin-Chih
authors.initials: LC
authors.lastname: Lin
authors.forename: Jaw-Town
authors.initials: JT
authors.lastname: Lin
authors.forename: Hsiu-Po
authors.initials: HP
authors.lastname: Wang
mesh_terms: D000328:Adult; D000369:Aged, 80 and over; D006502:Budd-Chiari Syndrome / Q000209:etiology* / Q000473:pathology* / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011169:Portal Vein / Q000473:pathology*; D011379:Prognosis; D016896:Treatment Outcome