Venous occlusive liver disease
| Classification according to ICD-10 | |
|---|---|
| K76.5 | Venous occlusive liver disease [Stuart-Bras syndrome] Excludes: Budd-Chiari syndrome (I82.0) |
| ICD-10 online (WHO version 2019) | |
The hepatic veno-occlusive disease as a form of hepatic veno-occlusive disease or VOD (also Hepatic sinusoidal obstruction syndrome, SOS is known) the result of (incomplete) closure of the small hepatic veins . It is similar to liver failure with ascites , liver enlargement and an increase in the bilirubin level in the blood.
The disease usually occurs as a complication of a higher dose of cytostatic treatment . The drug actinomycin D is often the cause . Other possible triggers are high-dose radiation of the liver, liver transplantation, or plant alkaloids such as pyrrolizidine alkaloids . There is also a rare congenital disease called hepatic veno-occlusive disease with immunodeficiency .
The frequency is given as 5% up to 60% in children after a stem cell transplant . The disease is not limited to this group of patients, but can also occur during the treatment of tumors such as Wilms' tumor , neuroblastoma or rhabdomyosarcoma .
VOD in the lungs is much less common .
Symptoms
Typical indications are weight gain, tight abdomen with ascites, liver enlargement, increase in bilirubin and often kidney failure .
Diagnosis
The suspicion can be confirmed or ruled out using Doppler sonography of the portal vein . Both the color Doppler and a pulsed Doppler are used. VOD can be found depending on the severity of the disease
- a decrease in the normally triphasic flow of the liver veins ,
- an abrogated forward flow of the portal vein ,
- a flow reversal with a hepatofugal (directed out of the liver) flow pattern.
As the flow reverses, the arterial influx into the liver increases at the same time.
treatment
A direct causal treatment is not known, anticoagulant drugs are used regularly, and if the diagnosis is rapid, an improvement is possible. Mortality depends on a. on the severity of the change.
The Budd-Chiari syndrome , which does not affect the small but rather the large hepatic veins and can occur as a side effect of the drug tioguanine, must be distinguished.
swell
- K. Ebel, E. Willich, E. Richter (Eds.): Differentialdiagnostik in der Pädiatric Radiologie, Thieme 1995, ISBN 3-13-128101-4
- Caffey's Pediatric Diagnostic Imaging Vol II, Mosby 2004, ISBN 0-323-01109-8
- Overview in emedicine: Veno-occlusive hepatic disease
Individual evidence
- ↑ H. Wegner: Sinusoidal obstruction syndrome (veno-occlusive disease, VOD / SOS) of the liver after hematopoietic stem cell transplantation: Prospective evaluation of relevant laboratory parameters for the differential diagnosis of VOD / SOS, dissertation 2009, ( online )
- ↑ R. Negrin, P. Bonis Diagnosis of hepatic sinusoidal obstruction syndrome (veno-occlusive disease) following hematopoietic cell transplantation Uptodate (2013)
- ^ Case Report
- ↑ Hakim Azfar Ali, Pulmonary Veno-Occlusive Disease , Medscape
- ↑ MM Zieger, E. Koscielniak: Diagnosis and follow-up of veno-occlusive disease of the liver by use of Doppler ultrasound. A case report. In: Pediatric radiology. Volume 23, Number 2, 1993, pp. 137-139, ISSN 0301-0449 . PMID 8390642 .
- ↑ G.Rettenmeier, K Seitz (ed.): Sonographische Differentialdiagnostik Vol. 1, VHC, ISBN 3-527-15461-2