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October 1965

Chiari's DiseaseA Clinico pathological Study

Author Affiliations

From the departments of surgery and pathology, the University of Michigan. Resident in Surgery (Dr. Ludwick), Resident in Pathology (Dr. Markel), Professor and Chairman of the Department of Surgery (Dr. Child).

Arch Surg. 1965;91(4):697-704. doi:10.1001/archsurg.1965.01320160151032

THE CLINICAL syndrome associated with thrombosis of the hepatic veins was described in 1845 by Budd.6 In his case this was secondary to adjacent liver abscesses. In 1899 Chiari reported ten cases of primary occlusions of the ostia of the hepatic veins, which were attributed to syphilitic endophlebitis. This etiologic concept is not supported by present-day knowledge. Obstruction of the hepatic venous outflow tracts has been described in association with a number of conditions. Prominent among these are: neoplastic and inflammatory hepatic diseases; primary or secondary neoplastic involvement of the inferior vena cava; polycythemia rubra vera, persistent Eustachian valve, trauma, and Senecio poisoning. In addition, there are cases reported without apparent cause, wherein the pathologic alterations are similar to those described by Chiari. The following case is reported to describe several clinical, pathological, and radiological facets of primary occlusion of the ostia of hepatic veins (Chiari's disease) and to

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