Budd–Chiari syndrome

Description

Hepatic venous outflow obstruction causing portal hypertension


History

1842Ernest 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.

Original
English

Observations d’inflammation de veins du foie: 1. Phlébite de la veine-porte, produite par une arête poisson qui traversa l’extrémité pylorique de l’estomac, la tête du pancréas et s’implanta dans le tronc de la mésentérique supérieure; 2. Des veines sus-hépatiques, due au voisinage d’un abcès métastatique: suives de quelques reflexions

Lambron 1842

Observations of inflammation of veins of the liver: 1. Portal vein phlebitis, produced by a fish bone which crossed the pyloric end of the stomach, the head of the pancreas and implanted in the trunk of the superior mesenteric; 2. The hepatic veins, due to the vicinity of a metastatic abscess: followed by some reflections

Lambron 1842

1845George 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


References


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the names behind the name

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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