Milestones in the discovery of Budd‐Chiari syndrome

Abstract

In 1845, George Budd published a brief report regarding three patients who developed an obstruction of the hepatic veins. The condition has never been reported before, and was related to sepsis and alcoholism. Fifty-three years later, Hans Chiari postulated that syphilis was causing the obstruction of the hepatic veins, and enriched the debate with clinical and pathological correlations. Following the hypothesis on the 'phlebitis obliterans', several authors proposed other pathophysiological explanations including congenital causes, chronic trauma and exogenous toxins. RG Parker, in 1959, first recognized the relationship between obstruction of hepatic veins and thrombophilic conditions such as polycythaemia vera, pregnancy and hormonal therapy. Based on that, anticoagulant treatment was attempted, but with unsatisfactory outcome. We need to wait until the mid 1980s to see a widespread adoption of anticoagulants, with a consequent improvement of patients' survival. The fear of haemorrhagic events in patients with liver disease discouraged this therapeutic approach, and other surgical interventions (mainly port-systemic shunts) were conceived, but with high morbidity and mortality. The first liver transplantation in 1976 and the first trans-jugular intra-hepatic porto-systemic shunt in 1993 represented two major cornerstones in the management of Budd-Chiari syndrome (BCS).Such progresses allowed modifying the treatment of BCS until the modern concept of stepwise therapy. The present review thoroughly reviews the major landmarks in the discovery, treatment and clinical management of patients with BCS.

A study of the different parameters in acute and chronic Budd–Chiari syndrome

“…There was no significant difference between the outcomes of the two groups in terms of hospital LOS and mortality. In our patients the most common predisposing factor for BCS were myeloproliferative neoplasms, paroxysmal nocturnal hemoglobinuria, and systemic lupus erythematosus (Table 1) The definitions of acute and chronic of BCS are not clearly defined [9]; however, most studies have classified the BCS with an onset of less than 6 months as acute and more than 6 months as chronic [3,5,6]. When BCS is suspected, color Doppler ultrasound is typically the first imaging procedure with high sensitivity and specificity [8].…”

“…Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder caused by a partial or complete occlusion from small hepatic veins till inferior vena cava including the main hepatic veins [1][2][3]. The true incidence of BCS is unknown due to its rarity, and various results have been reported in different regions [3,4].…”

“…Based on the onset of the disease in most studies, BCS is classified as acute (less than 6 months) and chronic (more than 6 months), and these two types of BCS differ in clinical manifestations, treatment, and prognosis [3,5,6].…”

Transjugular intrahepatic portosystemic shunts in portal vein thrombosis: A review

“…Earlier placement of TIPSS after patients develop refractory ascites is associated with improved rates of post-TIPSS ascites control. 10 Rarer indications for TIPSS include hepatic hydrothorax, 11 Budd-Chiari syndrome 12,13 and its use prior to abdominal surgery to decompress intra-abdominal varices. 14,15 Emerging indications for TIPSS include hepatopulmonary 16,17 and hepatorenal syndromes.…”

A 14-year-old boy presenting with gradual abdominal distension and occasional abdominal pain for 2 years

“…[1][2][3] In the late 1800s, the term Budd-Chiari was first named after a British inter-nist, George Budd, described three cases of hepatic vein thrombosis due to abscess-induced phlebitis and in 1899 Austrian pathologist Hans Chiari, added the first pathologic description of a liver with obliterating endophlebitis of the hepatic veins. 4 The prevalence of Budd-Chiari syndrome is 1/100,000 among the general population worldwide. 5 Compared to adults, it is very uncommon in children.…”