Venenverschlusskrankheit der Leber
Sinusoidal
obstruction syndrome (veno-occlusive disease) in a patient receiving bevacizumab for metastatic colorectal cancer: a case report. CT scans. (a) Liver metastasis (long arrow). (b) Response with reduction in the size of the liver metastasis (long arrow). (c) Development of varices at the lower oesophagus (long arrow). (d) Splenomegaly (long arrow) and ascites (short arrow).
Successful
treatment of severe sinusoidal obstruction syndrome despite multiple organ failure with defibrotide after allogeneic stem cell transplantation: a case report. (a) Computer tomography scan of the abdomen with hepathomegaly and ascites; (b) liver tissue obtained through transjugular liver biopsy, haematoxylin eosin stained with necrosis and inflammatory round-cell infiltration through transjugular liver biopsy.
Clinical
characteristics, CT signs, and pathological findings of Pyrrolizidine alkaloids-induced sinusoidal obstructive syndrome: a retrospective study. Elderly male patients diagnosed with gynura segetum-induced HSOS received contrast-enhanced CT and MRI scan. a-d images of plain and contrast-enhanced CT scan; a plain CT scan; b arterial phase; c porta-venous phase; patchy liver enhancement (arrow) and heterogeneous hypoattenuation (arrowhead) were shown; d equilibrium phases. e-f: images of pre-contrast and portal-venous phase on dynamic contrast-enhanced MRI scan;e pre-contrast MRI scan; f portal-venous phase of MRI scan. Heterogeneous hypointensity (arrowhead) and patchy enhancement (arrow) were shown
Noninvasive
imaging diagnosis of sinusoidal obstruction syndrome: a pictorial review. Hepatic sinusoid blood flow diagram. Hepatic sinusoids are a complex of vascular conduits to be responsible for the exchange of blood, oxygen, and nutrients in the liver and systemic circulation. The structure of the hepatic sinusoids is composed of the sinusoidal endothelial cells and containing Kupffer cell. Under normal circumstances, the blood supply to the hepatic sinusoid blood come from the inlet flows (i.e., the hepatic artery and portal vein) and venous blood return to the heart through the outlet flows (i.e., the hepatic veins)
Noninvasive
imaging diagnosis of sinusoidal obstruction syndrome: a pictorial review. A 39-year-old man with SOS caused by Tu san qi. a Normal CT scan shows non-uniform density of the liver parenchyma, hepatomegaly, and medium to a large amount of ascites. b Arterial phase contrast-enhanced CT scan shows non-uniform enhancement of hepatic parenchyma. c–e Portal phase contrast-enhanced CT scan shows patchy low-density lesions with a non-uniform decrease of hepatic parenchyma enhancement (the CT value of the liver is lower than that of the spleen), portal hypertension combined with collateral circulation development, gallbladder wall edema, and edema around the portal vein. f The hematoxylin-eosin (HE) staining at × 200 magnification shows expansion and congestion around the central hepatic vein and hepatic sinus, hepatocyte edema, and mild central venous fibrosis, which is confirmed to be SOS
Noninvasive
imaging diagnosis of sinusoidal obstruction syndrome: a pictorial review. A 68-year-old man with SOS caused by Tu san qi. a Normal CT scan shows a non-uniform density of the liver parenchyma and mild hepatosplenomegaly. b Arterial phase contrast-enhanced CT scan shows a small and ill-defined hepatic artery and decrease of hepatic parenchyma enhancement. c Portal phase contrast-enhanced CT scan shows patchy low-density lesions with a non-uniform decrease of hepatic parenchyma enhancement (the CT value of the liver is lower than that of the spleen)
Noninvasive
imaging diagnosis of sinusoidal obstruction syndrome: a pictorial review. a The T1-weighted image (T1WI) shows hepatomegaly, a non-uniform intensity of liver parenchyma with a patchy and nodular low-signal intensity of the liver. b The T2-weighted image (T2WI) shows a non-uniform signal intensity of liver parenchyma with a patchy and nodular high-signal intensity of the liver and ascites. c Arterial phase contrast-enhanced MRI scan shows heterogeneous enhancement of liver parenchyma. d Portal phase contrast-enhanced MRI scan shows a significant heterogeneous enhancement of liver parenchyma with patchy and nodular lesions with decreased enhancement. e HBP of gadoxetic acid-enhanced MRI shows the signal intensity of liver parenchyma is slightly decreased and accompanied by the nodular lesions with lower-signal intensity. f The hematoxylin-eosin (HE) staining at × 200 magnification shows hepatocyte edema and significant hepatic sinus expansion, which is confirmed to be SOS
Noninvasive
imaging diagnosis of sinusoidal obstruction syndrome: a pictorial review. A 48-year-old woman with focal SOS (arrows) developed by chemotherapy after hepatectomy. a, b A nodular lesion in liver segment III with low-signal intensity on the T1-weighted image (T1WI) and high-signal intensity on the T2-weighted image (T2WI). c Arterial phase contrast-enhanced MRI scan shows a nodular lesion in liver segment III with mild peripheral enhancement. d Portal phase contrast-enhanced MRI scan shows the nodular lesion with apparent, uniform, and persistent enhancement. e HBP of gadoxetic acid-enhanced MRI shows the nodular lesion with a low-signal intensity at the center and high-signal intensity around it, which presents as “focal nodular hyperplasia” like lesion. f The diffusion-weighted imaging (DWI) shows an apparent limited diffusion
Young adult
with newly rising ammonia levels after bone marrow transplant. Spectral doppler US of the left, middle and right hepatic veins from 2 days ago (left) shows them to be of normal caliber and to have normal appearing phasicity. Spectral doppler US of the left, middle, and right hepatic veins today (right) shows the hepatic veins to be compressed and difficult to see and to have spectral broadening and loss of phasicity. The flow velocity in the main portal vein had decreased by 50% between the two exams.The diagnosis was sinusoidal obstruction syndrome.
Sinusoidal obstruction syndrome (SOS), previously known as hepatic veno-occlusive disease (VOD), is a condition arising from occlusion of hepatic venules.
Clinical presentation
- right upper quadrant pain
- painful hepatomegaly
- ascites
- abnormal liver function tests
Pathology
Toxic injury to liver sinusoids causes sloughing of endothelial cells that embolize to hepatic venules and cause eventual fibrosis of the venules. This results in hepatic congestion (similar to Budd-Chiari syndrome) and post-sinusoidal portal hypertension.
Etiology
- bone marrow transplantation
- chemotherapy, includes systemic neo-adjuvant chemotherapy in metastatic colorectal cancer, particularly the use of oxaliplatin
- pyrrolizidine alkaloids (Jamaican bush tea)
Radiographic features
Ultrasound
The imaging modality of choice, which may show:
- hepatomegaly
- portal vein abnormalities:
- portal vein dilatation
- portal venous pulsatility
- hepatofugal portal venous flow
- elevated hepatic artery resistive index (> 0.8)
- loss of triphasic hepatic venous flow
- gallbladder wall thickening (> 6-8 mm)
- ascites
CT
- hepatomegaly
- nutmeg liver
- portal vein dilatation
- ascites
MRI
Recent MRI studies with hepatocyte-specific contrast agent show a diffuse hypointense reticular pattern on post-contrast T1 delayed hepatobiliary phase as a highly specific sign for the diagnosis.
Treatment and prognosis
There is no standard treatment regimen but options include:
- anticoagulation
- vasodilators
- supportive care
Preoperative planning for hepatic metastasectomy should include exclusion of SOS after the use of chemotherapy as there is an increased risk of postoperative liver failure.
Approximately half resolve but high mortality in severe cases (up to 90%).
Complications
- hepatic failure
- portal hypertension
- death
Siehe auch:
- portale Hypertension
- Aszites
- Hepatomegalie
- Budd-Chiari-Syndrom
- Venenverschlusskrankheit der Lunge
- bone marrow transplantation
- Muskatnussleber
- Actinomycin D
- hepatic veno-occlusive disease with immunodeficiency
und weiter:
Assoziationen und Differentialdiagnosen zu Venenverschlusskrankheit der Leber:
