[Budd-Chiari syndrome--a rare manifestation of hereditary thrombophilia].
Budd-Chiari syndrome is a rare manifestation of hereditary or acquired thrombophilia. We saw a case of Budd-Chiari syndrome in a 30-year-old woman leading to initial diagnostic difficulties. She underwent surgical side-to-side shunt and 9 weeks later an almost normal liver could be demonstrated on computerized tomography. Budd-Chiari syndrome should be considered if the Chiari triad with abdominal pain, hepatomegaly and ascites occurs in a patient. If necessary, invasive diagnostic procedures (e.g. angiography) must be performed. Therapeutic options are anticoagulative therapy and porto-systemic shunt, either as a TIPS or a surgical shunt. If severe liver failure occurs or liver cirrhosis is present, orthotopic liver transplantation is an additional option which also cures hereditary thrombophilia.
Mönch
C
C
Abteilung für Transplantationschirurgie, Chirurgie von Leber, Gallenwegen und Pankreas, Johannes Gutenberg-Universität Mainz.
Helmreich-Becker
I
I
Düber
C
C
Wolf
H K
HK
Galle
P R
PR
Otto
G
G
D020016:Activated Protein C Resistance / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D000328:Adult; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D003937:Diagnosis, Differential; D005165:Factor V / Q000235:genetics*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D011170:Portasystemic Shunt, Surgical; D019851:Thrombophilia / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery
C095381:factor V Leiden; D005165:Factor V