Autopsy and Case Reports
Autopsy and Case Reports
Article / Autopsy Case Report

Budd–Chiari Syndrome: an unnoticed diagnosis

Camila Kruschewsky Falcão; Gustavo C. Freitas Fagundes; Gustavo Checolli Lamos; Aloisio Felipe-Silva; Silvana Maria Lovisolo; João Augusto Martines; Fernando Peixoto Ferraz de Campos

Downloads: 12
Views: 1258


Budd–Chiari syndrome (BCS) encompasses a group of disorders caused by the obstruction to the hepatic venous outflow at the level of the small or large hepatic veins, the inferior vena cava, or any combination thereof. Clinical manifestation of the subacute form is characterized by supramesocolic abdominal discomfort, abdominal distension, fever, and lower limbs edema. Imaging work-up with hepatic Doppler ultrasound and abdominal computed tomography (CT) enables the diagnosis in the majority of cases. Treatment comprises long-term anticoagulation associated with measures that attempt to re-establish the flow in the thrombosed vessel (thrombolysis or angioplasty) or through the venous blood flow bypasses (transjugular intrahepatic portosystemic shunt or surgical bypass); however, the outcome is often dismal. The authors report the case of a 37-year-old woman presenting a 2-month history of dyspeptic complaints and abdominal distention. Fever was present at the beginning of symptoms. The laboratory work-up disclosed mild hepatic dysfunction, and the ultrasound showed evidence of chronic liver disease. Despite a thorough etiologic investigation, diagnosis was missed and, therefore, management could not be directed towards the physiopathogenetic process. The outcome was characterized by portal hypertension and esophageal varices bleeding. The patient died and the autopsy findings were characteristic of BCS, although an abdominal CT, close to death, had showed signs consistent with this diagnosis. The authors highlight the importance of knowledge of this entity, the diagnostic methods, and the multidisciplinary approach. BCS should be considered whenever investigating etiology for chronic or acute hepatopathy.


Budd–Chiari Syndrome, Hypertension, Portal, Liver Cirrhosis, Ascites, Autopsy


DeLeve LD, Valla DC, Garcia-Tsao G, American Association for the Study Liver Diseases. Vascular disorders of the liver. Hepatology. 2009;49(5):1729-64. []. [PMID:19399912]

Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med. 2004;350(6):578-85. []. [PMID:14762185]

Plessier A, Valla DC. Budd-Chiari syndrome. Semin Liver Dis. 2008;28(3):259-69. []. [PMID:18814079]

Mahmoud AE, Mendoza A, Meshikhes AN, et al. Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. QJM. 1996;89(1):37-43. []. [PMID:8730341]

Darwish Murad S, Valla DC, de Groen PC, et al. Determinants of survival and the effect of portosystemic shunting in patients with Budd-Chiari syndrome. Hepatology. 2004;39(2):500-8. []. [PMID:14768004]

Dancygier H. Hepatic veins. In: Dancygier H, editor. Clinical hepatology: principles and practice of hepatobiliary diseases. Berlin, Heidelberg: Springer-Verlag; 2010. v. 2. p. 641-47.

Chung RT, Iafrate AJ, Amrein PC, Sahani DV, Misdraji J. Case records of the Massachusetts General Hospital. Case 15-2006. A 46-year-old woman with sudden onset of abdominal distention. N Engl J Med. 2006;354(20):2166-75. []. [PMID:16707754]

MacNicholas R, Olliff S, Elias E, Tripathi D. An update on the diagnosis and management of Budd-Chiari syndrome. Expert Rev Gastroenterol Hepatol. 2012;6(6):731-44. []. [PMID:23237258]

Valla DC. Hepatic vein thrombosis (Budd-Chiari syndrome). Semin Liver Dis. 2002;22(1):5-14. []. [PMID:11928075]

Horton JD, San Miguel FL, Membreno F, et al. Budd-Chiari syndrome: illustrated review of current management. Liver Int. 2008;28(4):455-66. []. [PMID:18339072]

Bolondi L, Gaiani S, Li Bassi S, et al. Diagnosis of Budd-Chiari syndrome by pulsed Doppler ultrasound. Gastroenterology. 1991;100(5 Pt 1):1324-31. [PMID:2013376]

Copelan A, Remer EM, Sands M, Nghiem H, Kapoor B. Diagnosis and management of Budd Chiari syndrome: an update. Cardiovasc Intervent Radiol. 2015;38(1):1-12. []. [PMID:24923240]

Faraoun SA, Boudjella ME, Debzi N, et al. Budd-Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging. Abdom Imaging. 2015. [Epub ahead of print]. []. [PMID:25687630]

Kim H, Nahm JH, Park YN. Budd-Chiari syndrome with multiple large regenerative nodules. Clin Mol Hepatol. 2015;21(1):89-94. []. [PMID:25834807]

Pavri TM, Herbst A, Reddy R, Forde KA. Budd-Chiari syndrome: a single-center experience. World J Gastroenterol. 2014;20(43):16236-44. [PMID:25473178]

Nishida N, Iwamura S, Ida H, et al. Clinical and histological features of different types of Budd-Chiari syndrome: a comparison of 4 cases. Dig Dis. 2013;31(5-6):408-14. []. [PMID:24281013]

Khuroo MS, Al-Suhabani H, Al-Sebayel M, et al. Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt. J Gastroenterol Hepatol. 2005;20(10):1494-502. []. [PMID:16174064]

De BK, Sen S, Biswas PK, et al. Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression. Gastroenterology. 2002;122(4):897-903. []. [PMID:11910341]


Publication date:

569538f864cc8b1a57053d81 autopsy Articles

Autops Case Rep

Share this page
Page Sections