Congenital hepatic fibrosis associated with cavernous transformation of the portal vein.
OBJECTIVE
Congenital hepatic fibrosis (CHF), which is one of the fibropolycystic diseases, occurs in various forms. Portal hypertension, a very common clinical feature of this condition, has been attributed to the compression of portal vein radicles in the fibrous bands. We investigated whether there are any other contributing factors in the development of portal hypertension in patients with CHF.


METHODS
A total of 1285 patients with portal hypertension of different etiologies were studied using ultrasonography as the screening test. Forty-seven (including portal vein involvement and/or CHF) of these 1285 patients were prospectively studied to evaluate the etiology of the portal hypertension by portography, abdominal computed tomography, exploratory laparotomy, peritonoscopy, liver biopsy and laboratory tests. The patients with CHF were divided into two groups, according to whether or not they had portal vein involvement.


RESULTS
Eleven (0.8%) of the 1285 patients with portal hypertension had CHF, and 41 (3.2%) had cavernous transformation of the portal vein (CTPV), resulting from different or unknown etiologies. Five patients had both pathologies (CTPV and CHF). In the 11 patients with CHF, there was CTPV in 5 patients, Caroli's disease in 2 patients, cholangiocarcinoma in 1 patient, inferior vena caval obstruction in 1 patient, and CHF in only 2 patients. There were statistically significant differences in the age of the CHF patients at clinical onset, the incidence of bleeding from esophageal varices, and laboratory findings between the 2 groups with and without CTPV. Despite a thorough investigation, we could not distinguish any predisposing factor in 25 of the 41 patients with CTPV. The incidence of CTPV was 48% in patients with CHF and 3.2% in patients with portal hypertension.


CONCLUSIONS
These results suggest that the association of CTPV with CHF is not coincidental, but that CTPV may be associated with CHF and a new possible factor in portal hypertension, and that it can be a major factor in the manifestation of esophageal bleeding from varices.
Bayraktar
Y
Y
Department of Gastroenterology, Hacettepe University School of Medicine, Ankara, Turkey.
Balkanci
F
F
Kayhan
B
B
Uzunalimoglu
B
B
Ozenc
A
A
Ozdemir
A
A
Dündar
S
S
Arslan
S
S
Sivri
B
B
Telatar
H
H
D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D002648:Child; D002760:Cholangiopancreatography, Endoscopic Retrograde; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000503:physiopathology*; D008103:Liver Cirrhosis / Q000151:congenital* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011169:Portal Vein / Q000000981:diagnostic imaging / Q000473:pathology*; D011172:Portography; D011446:Prospective Studies; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography