Hepatic veno-occlusive disease

The Venous occlusive liver disease as hepatic veno - occlusive disease of the shape or VOD ( also Hepatic sinusoidal obstruction syndrome, called SOS ) is the result of an ( incomplete ) closure of the small hepatic veins. It shows itself like a liver failure with ascites, liver enlargement and increase in bilirubin in the blood.

The disease usually occurs as a complication of higher doses of cytostatic treatment. The reason is often the drug actinomycin D. Other possible triggers include high-dose irradiation of the liver, liver transplantation, or plant alkaloids such as pyrrolizidine alkaloids. Furthermore, there is a rare congenital disorder called hepatic veno- occlusive disease with immunodeficiency.

The frequency is given as 5 % to 60 % in children with stem cell transplantation, but is not limited to this group of patients, but can also occur under treatment of tumors such as Wilms' tumor, neuroblastoma or rhabdomyosarcoma.

Much rarer is a VOD in the lungs.

Symptoms

Typical indications are weight gain, strained waist circumference with ascites, enlargement of the liver, bilirubin and often kidney failure.

Diagnostics

The suspicion can be confirmed or excluded by Doppler sonography of the portal vein. It comes in both color Doppler and pulsed Doppler apply. In the VOD can be found depending on the severity of the disease

  • A reduction of the normally triphasic undulating forward flow.
  • A reversed forward flow.
  • A flow reversal with hepatofugal ( directed from the liver out ) flow pattern.

With increasing flow reversal at the same time increases the arterial inflow to the liver.

Treatment

A direct causal treatment is not known anticoagulant drugs are regularly used, with rapid diagnostic finding an improvement is possible. The mortality depends, inter alia, the severity from the change.

To be distinguished is the Budd -Chiari syndrome, which does not affect the small, but the large hepatic veins and can occur as a side effect of the drug thioguanine.

Swell

  • K. Ebel, E. Willich, E. Richter ( ed): Differential Diagnosis in Pediatric Radiology, 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
Budd–Chiari syndrome Renal failure
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