ABSTRACT
We describe a newborn infant with veno-occlusive disease (VOD) of the liver. Prior
to discharge from the hospital, the newborn, who had been treated for suspected neonatal
infection, suddenly developed sepsis-like symptoms. The size of the liver as well
as serum activity of hepatic enzymes increased progressively. Initial Doppler-flow
studies demonstrated an absent flow in the vena portae, a finding that was compatible
with vena portae thrombosis or occlusion of other hepatic veins. A therapy with recombinant
tissue plasminogen activator (rt-PA) was initiated; due to extensive bleedings from
various sides, the fibrinolytic therapy had to be withdrawn 12 hours later, when Doppler-flow
examination revealed a reverse flow in hepatofugal direction. Despite supportive therapy,
the general condition of the patient deteriorated continuously, finally resulting
in liver and renal failure. Our patient died 19 days after birth. The autopsy demonstrated
obliterative lesions of the centrilobular and sublobu-lar hepatic veins, the classical
signs of VOD of the liver. Despite extensive diagnostics and examinations, the etiology
of VOD could not been elucidated in this newborn.
Keywords
Veno-occlusive disease - newborn
