title: Obliterative portal venopathy: portal hypertension is not always present at diagnosis.
abstract: OBJECTIVE
  Previous studies on obliterative portal venopathy (OPV) have been biased due to the selection of patients with non-cirrhotic portal hypertension. The aim of this study was to clarify the characteristics of OVP diagnosed by liver biopsy.
  
  
  METHODS
  Fifty-nine consecutive patients with OPV were retrospectively selected on strict histological criteria. Clinical, laboratory, portal vein patency, and associated disorders potentially involving vascular alterations were analyzed. The occurrence of complications was recorded during follow-up.
  
  
  RESULTS
  Mean age at diagnosis was 38.5±15 years old. Initial presentation was portal hypertension (64% of patients) and/or extrahepatic portal vein thrombosis (EHPVT) (22%) or isolated abnormal laboratory tests (20%). Associated diseases found at diagnosis were: prothrombotic disorders (30% of patients) and immune-mediated disorders (17%); 53% of patients had no causal factor (idiopathic OPV). During follow-up (median 8.6 years, range 1-23 years), features of portal hypertension worsened in 46% of patients; EHPVT and portal hypertension were finally found in 44% and 88% of patients. Anti-coagulation and beta-blockers were administered in 47% and 59% of patients, respectively. Severe complications (liver transplantation and/or death) occurred in 11 (19%) patients, 8 had idiopathic OPV. Patients with prothrombotic disorders received earlier anticoagulation therapy; all survived without transplantation.
  
  
  CONCLUSIONS
  A confident diagnosis of OPV can be done by biopsy and is conceivable in patients under 40 years without clinically significant portal hypertension. Poor outcome was noted in 19% of patients, most of them affected with idiopathic OPV. Patients with prothrombotic disorders received early anticoagulation and appeared to have a better outcome despite a high proportion of EHPVT.
authors.affiliation: Service d'Anatomie-Pathologique, Hôpital Beaujon AP-HP, Clichy and Institut National de la Santé et de la Recherche Médicale U773, Centre de Recherche Biomédicale Bichat Beaujon CRB3, Université Denis Diderot-Paris 7, France. dominique.cazals-hatem@bjn.aphp.fr
authors.forename: Dominique
authors.initials: D
authors.lastname: Cazals-Hatem
authors.forename: Sophie
authors.initials: S
authors.lastname: Hillaire
authors.forename: Marika
authors.initials: M
authors.lastname: Rudler
authors.forename: Aurélie
authors.initials: A
authors.lastname: Plessier
authors.forename: Valerie
authors.initials: V
authors.lastname: Paradis
authors.forename: Bertrand
authors.initials: B
authors.lastname: Condat
authors.forename: Claire
authors.initials: C
authors.lastname: Francoz
authors.forename: Marie-Hélène
authors.initials: MH
authors.lastname: Denninger
authors.forename: François
authors.initials: F
authors.lastname: Durand
authors.forename: Pierre
authors.initials: P
authors.lastname: Bedossa
authors.forename: Dominique C
authors.initials: DC
authors.lastname: Valla
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D002648:Child; D015331:Cohort Studies; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000628:therapy; D008099:Liver / Q000098:blood supply / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011169:Portal Vein / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D014652:Vascular Diseases / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D020246:Venous Thrombosis / Q000175:diagnosis / Q000628:therapy; D055815:Young Adult