THROMBOSIS OF THE HEPATIC VEINS—CHIARI'S SYNDROME; REPORT OF A CASE WITH BIOPSY AND VENOUS PRESSURE DETERMINATION

Abstract

Obstruction to the hepatic veins leads to a pathological-clinical entity known as Chiari''s syndrome which gives the clinical picture of portal obstruction combined with hepatic degeneration. The authors review the literature and add an example of the acute form. The syndrome is either primary or secondary. The primary form may be acute or chronic. The chronic form results from repeated small thromboses; remissions are due to the development of a collateral circulation. Replacement fibrosis, regeneration, and hypertrophy of the unaffected cells follow with eventual hobnail liver fibrosis. The secondary form may be due to extrinsic pressure, or to perivascular inflammatory constriction, or to polycythemia vera with thrombosis of the hepatic veins. At post-mortem, a liver exhibiting marked atrophy from venous congestion with a normal heart suggests the site of the lesion. All of these patients usually die from hepatic insufficiency within a few mos. In the case described by the author, death resembled that seen in the hepato-renal syndrome. The diagnosis was made by the clinical picture, and by venous pressure detn. Circulation times were normal, but pressure over the liver resulted in a rapid rise above normal in the venous pressure. The authors emphasize this last finding.