Budd Chiari syndrome

Budd Chiari syndrome

A condition defined by obstruction of the hepatic veins and its clinical manifestations, regardless of the cause (except congestive heart failure), where the obstruction is either within the liver on in the inferior vena cava between the liver and the right atrium.
 
Aetiology
Thrombosis in the hepatic veins (majority of cases) due to coagulation secondary to polycythaemia vera, pregnancy, postpartum, oral contraceptive use, intravascular lesions including malignancy, myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria (PNH), infection (e.g., schistosomiasis), intravascular webs.
 
Clinical findings
Abdominal pain, ascites, jaundice, hepatomegaly, and eventually cirrhosis, liver failure and hepatic encephalopathy.

Lab
Abnormal LFTs, increased liver enzymes.

DiffDx
Liver failure, GI bleeding, starvation.
 
Management
Unsatisfactory.
References in periodicals archive ?
Elias, "Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome," Gut, vol.
Dutta et al., "Celiac disease and Budd Chiari syndrome: Report of a case with review of literature," European Journal of Gastroenterology & Hepatology, vol.
Gender Age RCC Presentation No side 1 F 62 Rt Renal mass, pain, hematuria 2 F 81 Lt Renal mass, pain, hematuria 3 F 72 Lt Renal mass, ascites (Budd Chiari syndrome) 4 M 47 Lt Renal mass, hematuria 5 M 70 Lt Renal mass, pain, hematuria 6 F 67 Rt Renal mass, hematuria 7 M 51 Lt Renal mass, pain 8 M 65 Lt Hematuria, anemia 9 F 62 Lt Renal mass, hematuria, jaundice 10 M 79 Rt Right renal mass, hematuria, left adrenal mass.
Most common etiology was alcohol [n-27; 37.5%], followed by hepatitis B [n-13; 18.05%], hepatitis C [n-7; 9.7%], combined infection with hepatitis B and C [n-2; 2.7%], hepatitis B with alcohol [n-5; 6.9%], hepatitis C with alcohol [n-4; 5.5%], Wilson's disease [n-5; 6.94%], Budd Chiari syndrome [n-7; 9.7%], secondary biliary cirrhosis [n- 2; 2.7%].
Objective: To determine aetiology clinical presentation and predictors of survival in Budd Chiari Syndrome patients.
Conclusion: Budd Chiari Syndrome affected young patients more frequently and was associated with high mortality.
A number of syndromes overlapped with SLE like Sjogren's syndrome, Scleroderma, Rheumatoid arthritis, Antiphospholipid syndrome and Budd Chiari syndrome. Photosensitivity was found to be found in most of the SLE patients overlapping with various syndromes (Figure 3).
Budd Chiari syndrome (BCS) is a rare disorder that results from hepatic venous outflow tract obstruction, occurring anywhere from the small hepatic veins to the suprahepatic inferior vena cava.
The aforementioned act, or failure, raises the suspicion of manslaughter (section 298 of the penal law, 1977), and/or causing death by negligence (sections 304 and 309 (4) of the penal law, 1977) and responsibility for helpless person and violation of obligation of perant or of responsible person (sections 322 and 337 of the penal law).In September 2010, Anas Saleh was diagnosed with a liver disease, Budd Chiari Syndrome (a clinical syndrome resulting from obstruction of the veins in the liver).
The pathology might have started early in the pregnancy which remained latent until the uterine contractions set in and increased abdominal pressure might have compressed the retro hepatic Inferior vena cava accelerating the thrombotic process, causing complete obstruction of the Inferior vena cava, portal vein, leading to acute presentation of Budd Chiari Syndrome. (7,8)
Budd Chiari syndrome (BCS) is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at any level from the small hepatic veins to the atrio cava junction.
Ortiz, Budd Chiari syndrome: illustrated review of current management.