Diagnosis and Treatment of the Budd--Chiari Syndrome in Polycythaemia Vera

Abstract

University Surgical Tutor, St. Vincent's Hospital The Budd-Chiari syndrome is a disease entity which follows occlusion of the hepatic veins. Originally described by Budd in 1845 and subsequently by Chiari in 1899, it had been recorded in 116 cases up to 1952. Later reports have referred to involvement of the inferior vena cava in addition to the hepatic veins. The aetiological factors are numerous (Palmer, 1954). Thrombotic complications being relatively frequent in polycythaemia vera, it is not surprising that the syndrome should have been recorded in that condition. Nevertheless, Sohval (1938) states that the hepatic veins are the rarest thrombotic site in polycythaemia-the first case having been described by Oppenheimer in 1929, and not more than 10 cases had been reported up to 1938. A case is reported which presented the features of the chronic phase of the syndrome as described by Budd and by Chiari, due to hepatic-vein thrombosis in polycythaemia vera. Fi. I.-Infra-red photograph showing abnormal venous pattern. Note also external genitalia.

A change in the sinusoid‐trabecular structure of the liver with hepatic venous outflow block

“…No observations similar to our own are recorded in the following recent case reports: Coronini and Oberson (1937); Konig (1944); Kilsey and Comfort (1945); Meyer (1947); Caputi and Warthin (1952); Jonas and Lawrence (1954); Palmer (1954); Hsiang (1955);Fitzgerald et al (1956); Kibel and Marsden (1956); Norris (1956); Krass (1957); Parker; and Gibson. This discrepancy might be due to the fact that the above reports were based mainly on necropsy and not, as ours, upon biopsy study.…”

Unklare Leberfibrose bei einer 42-j�hrigen Patientin mit Polycythaemia vera

“…Der kausale Zusammenhang zwischen dem Budd-Chiari-Syndrom und einer Polycythaemia vera wurde erstmals 956 von Fitzgerald beschrieben [4]. Thromboembolische Ereignisse wie der Verschluss der großen Lebervenen sind der wichtigste Grund für die erhöhte Morbidität und Mortalität von Patienten mit einer Polycythaemia vera [2].…”

Budd–Chiari syndrome in very young adult patients with polycythemia vera

“…The association between BCS and polycythemia vera was first described by Fitzgerald et al [7]. For a while, it was assumed that polycythemia vera resulted from the hepatic changes caused by the BCS [7,8].…”