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From question on page 672
As fig 1 shows, there is a large complex shunt present joining a portal vein (PV) and an adjacent hepatic vein (HV) (left). There is focal necrosis of the parenchyma (top left). The right figure shows hepatic vein 0.5 mm in diameter shows luminal obliteration by organised thrombosis. There is severe necrosis of the surrounding liver parenchyma (both 52×, elastic trichrome stain).
The resected liver contained many arteriovenous malformations compatible with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease). In addition, we found ischaemic necrosis and organising hepatic vein thrombosis involving the medium and small veins.
Pregnancy is a risk factor for hepatic vein thrombosis because of enhanced coagulability and compression by the uterus on the vena cava and liver. In the present case, pre-existent arteriovenous malformations may have made the hepatic veins more susceptible to thrombosis because of turbulent flow. Hypovolaemic shock could also have contributed to thrombus formation and hepatic necrosis. The different ages of necrosis and thrombosis in the present liver suggest a stepwise progression of the ischaemic change. Hepatic vein thrombosis leading to lobular necrosis and hepatomegaly probably began about three weeks prior to delivery when she complained of abdominal pain.
The diagnosis of hereditary hemorrhagic telangiectasia was confirmed with the Arg411Trp mutation in exon 8 of the ALK1 gene. The disease is inherited as an autosomal dominant trait with an incidence of 1:2500. The prevalence of clinical liver involvement is 8–31%.
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