Budd–Chiari syndrome

Description

Hepatic venous outflow obstruction causing portal hypertension

History

1842 – Ernest Lambron (1815 – 1882) published a case of portal vein inflammation caused by a fish-bone, which passed through the pyloric extremity of the stomach and the head of the pancreas, and stuck in the superior mesenteric vein.

1845 – George Budd (1808 – 1882) describes inflammation and obstruction of the portal vein secondary to mechanical obstruction [1845: 136-148]. He references the case of Lambron (1842) and adds two further cases. Two patients had multiple intra-hepatic abscesses that involved one of the hepatic veins, with resultant thrombosis. Budd attributed the thrombosis to sepsis in two cases, while in the third one, with ‘adhesive’ inflammation, secondary to alcoholism.

Of note, Budd, pays reference to Rokitansky’s 1842 publication “Rokitansky, who has well described these appearances, states that they are very common in persons who die in the hospitals in Vienna“

1899 – Whilst working as a pathologist in Prague, Hans Chiari (1851 – 1916) described three patients with hepatic vein thrombosis, together with a review of the literature including another seven patients. Chiari advanced Budd’s early description clinical and pathological correlations terming the condition ‘phlebitis obliterans’. The livers appeared to be congested, atrophic, and diffusely necrotic, with congestion of the spleno-portal circulation and large volume ascites.

Associated Persons

• Karel Freiherr von Rokitansky (1804 – 1878)
• George Budd (1808 – 1882)
• Hans Chiari (1851 – 1916)

References

• Lambron E. Observations d’inflammation de veins du foie. Archives générales de médecine. 1842; III(XIV): 129-143
• Rokitansky C. Entzündung der Leber; Entzündung der Pfortader. In: Handbuch der pathologischen Anatomie. 1842; III: 324-332 [Rokitansky disease]
• Budd G. On diseases of the liver. 1845 [Hepatic venous thrombosis of Budd-Chiari; IV: 136-148]
• Chiari H. Erfahrungen über Infarktbildungen in der Leber des Menschen. Zeitschrift für Heilkunde, 1898; 19: 475-512.
• Chiari H. Ueber die selbständige Phlebitis obliterans der Hauptstämme der Venae hepaticae als Todesursache. Beiträge zur pathologischen Anatomie und Physiologie. 1899; 26: 1-18. [Beiträge zur Pathologie 1976; 158(1): 31-41]
• Brancatelli G et al. Budd-Chiari syndrome: spectrum of imaging findings. AJR Am J Roentgenol. 2007; 188(2): W168-76
• Torabi M, Hosseinzadeh K, Federle MP. CT of nonneoplastic hepatic vascular and perfusion disorders. Radiographics. 2008 Nov-Dec;28(7):1967-82.

History

• Zanetto A, Pellone M, Senzolo M. Milestones in the discovery of Budd-Chiari syndrome. Liver Int. 2019;39(7):1180-1185.