Cavernous transformation of the portal vein: etiology determines the outcome.
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.
Chang
Chi-Yang
CY
Department of Internal Medicine, College of Medicine National Taiwan University Hospital, Taipei, Taiwan.
Yang
Pei-Ming
PM
Hung
Shih-Pei
SP
Tsay
Woei
W
Lin
Lin-Chih
LC
Lin
Jaw-Town
JT
Wang
Hsiu-Po
HP
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