Diagnostic and Interventional Radiology for Budd-Chiari Syndrome

Abstract

Budd-Chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction that involves one or more draining hepatic veins. Its occurrence in populations in the western hemisphere is commonly associated with hypercoagulative states. Clinical manifestations in many cases are nonspecific, and imaging may be critical for early diagnosis of venous obstruction and accurate assessment of the extent of disease. If Budd-Chiari syndrome is not treated promptly and appropriately, the outcome may be dismal. Comprehensive imaging evaluations, in combination with pathologic analyses and clinical testing, are essential for determining the severity of disease, stratifying risk, selecting the appropriate therapy, and objectively assessing the response. The main goal of treatment is to alleviate hepatic congestion, thereby improving hepatocyte function and allowing resolution of portal hypertension. Various medical, endovascular, and surgical treatment options are available. Percutaneous and endovascular procedures, when performed in properly selected patients, may be more effective than medical treatment methods for preserving liver function and arresting disease progression in the long term. In addition, such procedures are associated with lower morbidity and mortality than are open surgical techniques.

Single-access liver floss technique with antegrade hepatic vein access and recanalization in Budd-Chiari syndrome

“…Patients with Budd-Chiari syndrome may be asymptomatic and discovered incidentally or present with symptoms on a spectrum ranging from mild abdominal pain to fulminant liver failure. The severity of disease on presentation is dictated by the degree and rate of onset of obstruction, as well as the development of compensatory collateral outflow (2). Following diagnosis, the goal of treatment is to decompress hepatic venous outflow in a timely manner to minimize damage to hepatocytes.…”

“…These therapies may alleviate symptoms temporarily but are not definitive treatments, and relying solely on medical management is associated with poor longterm outcomes. The well accepted definitive treatments of Budd-Chiari syndrome are endovascular (hepatic vein recanalization, TIPS) and surgical (shunt creation, liver transplant) procedures (2). Endovascular methods of decompression are preferred over surgical shunt creation as they are associated with lower morbidity and mortality, and liver transplantation is limited by donor availability and is reserved for patients with irreversible liver damage and advanced hepatocellular dysfunction.…”

Karaciğerin Vasküler Hastalıkları

“…Primer veya sekonder nedenlerle gelişen sendromda sinüzoidal basınç artmakta ve PV akımı azalmaktadır. Sentrlobüler konjesyona bağlı olarak nekroz ve atrofi gelişir [1,[4][5][6].…”

“…Bu nedenler kemoterapi ve radyasyon, kemik iliği nakli, oral kontraseptif ilaç kullanımı, hamilelik, polistemi ve protein C eksikliği sayılmaktadır. Nontrombotik nedenler ise karaciğer veya karaciğer dışı kitle lezyonlarıdır (4)(5)(6).…”

Development of a canine model with diffuse hepatic vein obstruction (Budd-Chiari syndrome) via endovascular occlusion

“…According to possible sites of obstruction, BCS may be divided into hepatic vein obstruction, inferior vena cava obstruction and a mixture of hepatic vein and inferior vena cava obstructions. Development of effective vascular surgery and interventional treatment approaches for the treatment of BCS associated with diffuse hepatic vein obstruction is difficult (3,4). Multiple studies have shown that portal vein-vena cava surgical shunt does not increase the survival of BCS patients (5)(6)(7)(8).…”