Budd-Chiari Syndrome

Classification according to ICD-10
I82.0	Budd-Chiari Syndrome
ICD-10 online (WHO version 2019)

As Budd-Chiari syndrome (BCS) refers to a rare disease of the liver , in which there is a complete or incomplete closure of the efferent blood vessels of the liver. In the classic Budd-Chiari syndrome, the occlusion of the hepatic veins primarily affects the large hepatic veins and is nowadays distinguished from diseases that affect the clogging of the small hepatic veins .

The disease got its name after its first descriptions George Budd (1808-1882) and Hans von Chiari (1851-1916). Synonym is also used: English Rokitansky’s disease; von Rokitansky disease .

Symptoms

The acute Budd-Chiari syndrome is characterized by a feeling of pressure or pain in the right upper abdomen that usually sets in within a few hours. In addition, there is often an ascites . Laboratory-chemical partly strong increase in transaminases (GOT / ASAT often higher than GPT / ALAT ). Liver and spleen usually appear enlarged ( hepatosplenomegaly )

causes

The causes of Budd-Chiari syndrome are often not exactly known. In the vast majority of cases, the hepatic vein is blocked by the formation of blood clots ( thrombosis ). However, there are also isolated cases in which, for example, a space-occupying tumor leads to a Budd-Chiari syndrome due to external compression of the hepatic vein. In children, Wilms' tumor can lead to thrombosis of the vena cava and the right auricle .

Diseases that lead to increased blood clotting ( thrombophilia ) represent a risk factor for the occurrence of Budd-Chiari syndrome. A factor V Leiden mutation increases the relative risk by a factor of 11. A protein C deficiency or a mutation of the prothrombin gene also lead to an increased risk of BCS.

There are several possible causes in around 25% of patients. The Budd-Chiari syndrome occurs most frequently in connection with a malignant disease of cells of the blood-forming system ( myeloproliferative syndrome ).

Diagnosis

The diagnosis is made by detecting closed branches of the hepatic vein using Doppler sonography and by diagnosing the underlying disease (e.g. bone marrow puncture ).

The exact extent of the vascular occlusion is determined by liver venography . If the findings are unclear, a liver biopsy can provide further information.

therapy

If left untreated, the Budd-Chiari syndrome can lead to severe liver damage ( necrosis ) or even liver failure , as the occlusion of the liver veins means that adequate blood flow to the liver is no longer ensured. A chronic Budd-Chiari syndrome can lead to increased blood pressure in the liver ( portal hypertension ) and consequently to liver fibrosis .

Drug thrombolysis of the blood clots can be performed to restore adequate blood flow . There is also the option of creating a TIPS (transjugular intrahepatic portosystemic shunt ). In addition, various surgical procedures exist to restore blood flow.

If it is not possible to ensure sufficient blood circulation in the liver in the long term, a liver transplant may also be necessary.

Chronic Budd-Chiari syndrome involves permanent anticoagulation , for example with phenprocoumon , with the aim of preventing the formation of new blood clots.

literature

M. Aydinli, Y. Bayraktar: Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. In: World journal of gastroenterology: WJG. Volume 13, Number 19, May 2007, pp. 2693-2696, ISSN 1007-9327 . PMID 17569137 . (Review).
H. Ferral, G. Behrens, J. Lopera: Budd-Chiari syndrome. In: American Journal of Roentgenology . Volume 199, Number 4, October 2012, pp. 737-745, ISSN 1546-3141 . doi: 10.2214 / AJR.12.9098 . PMID 22997363 .
A. Mukund, S. Gamanagatti: Imaging and interventions in Budd-Chiari syndrome. In: World journal of radiology. Volume 3, Number 7, July 2011, pp. 169-177, ISSN 1949-8470 . doi: 10.4329 / wjr.v3.i7.169 . PMID 21860712 . PMC 3158894 (free full text).
A. Plessier, DC Valla: Budd-Chiari syndrome. In: Seminars in Liver Disease . Volume 28, Number 3, August 2008, pp. 259-269, ISSN 0272-8087 . doi: 10.1055 / s-0028-1085094 . PMID 18814079 . (Review).

Individual evidence

↑ G. Budd: On diseases of the liver. J. Churchill, London 1845, p. 135.
↑ H. Chiari: Experiences about infarct formations in the liver of humans . In: Zeitschrift für Heilkunde Prague, 1898, 19: pages 475–512.
↑ M. Aydinli, Y. Bayraktar: Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. In: World journal of gastroenterology: WJG. Volume 13, Number 19, May 2007, pp. 2693-2696, ISSN 1007-9327 . PMID 17569137 . (Review)
↑ C. Stambolis, R. Döhler , W. Havers: Wilms tumor with Budd-Chiari syndrome and right atrial tumor thrombus . pediatrische praxis 20 (1978), pp. 243-247
↑ HL Janssen, JR Meinardi, FP Vleggaar, et al .: Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study. In: Blood. Volume 96, Number 7, October 2000, pp. 2364-2368, ISSN 0006-4971 . PMID 11001884 .

[1] G. Budd: On diseases of the liver. J. Churchill, London 1845, p. 135.

[2] H. Chiari: Experiences about infarct formations in the liver of humans . In: Zeitschrift für Heilkunde Prague, 1898, 19: pages 475–512.

[3] M. Aydinli, Y. Bayraktar: Budd-Chiari syndrome: etiology, pathogenesis and diagnosis. In: World journal of gastroenterology: WJG. Volume 13, Number 19, May 2007, pp. 2693-2696, ISSN 1007-9327 . PMID 17569137 . (Review)

[4] C. Stambolis, R. Döhler , W. Havers: Wilms tumor with Budd-Chiari syndrome and right atrial tumor thrombus . pediatrische praxis 20 (1978), pp. 243-247

[5] HL Janssen, JR Meinardi, FP Vleggaar, et al .: Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study. In: Blood. Volume 96, Number 7, October 2000, pp. 2364-2368, ISSN 0006-4971 . PMID 11001884 .