A 23-y/o male from a Nairobi slum presents with recurrent upper abdominal pain and leg swelling. He has lived in the slum for a long time and has a history of excessive alcohol consumption. He looks older than stated age, his abdomen is slightly distended and he has bilateral lower extremity pitting edema. Ultrasound findings in Figures 1-4 below.
Fig. 1: Right upper quadrant coronal view. Liver, ascites and pleural effusion
On the differential diagnosis is Hepatic Vena Cava Syndrome (HVCS), a condition that results from chronic stenosis of the IVC close to the outlet of the hepatic veins.1 The disease was initially described in Europe and North America but current disease reports are predominantly from Asia and Africa, where its incidence correlates with areas of poor hygienic conditions.2
Fig. 2: An extensive non-homogenous echogenicity (thrombus) in the IVC
Fig. 3: The thrombus in the IVC extends minimally into the hepatic veins
Fig 4: Minimal residual flow in the IVC
While still under debate, current research indicates that HVCS results from localized thrombophlebitis of the hepatic IVC secondary to bacterial infection, especially in areas of poor hygiene where GI infections and gram-negative bacteremia frequently occur. With time the initial lesion organizes into a thrombus, then a fibrous cord causing stenosis or complete obstruction, associated with collateral venous formations.3,4
Patients can be asymptomatic for decades, then present with acute exacerbations precipitated by recurrent bacterial infections and worsening thrombus formation.5 Increased sinusoidal pressure leads to cirrhosis (with relatively preserved hepatocellular function) and ascites.6 Varices, GI bleed and other sequelae of portal hypertension result. Other features include hepatomegaly, splenomegaly and pleural effusions. The condition affects both sexes and all ages including children.
Current treatment recommendations involve high dose antibiotics for 6-8 weeks combined with diuretics.1 The condition does not respond to anticoagulation. Surgical and endovascular procedures have been used to decompress the IVC.7 HVCS should be considered as a potential cause of early onset liver disease in developing countries. Ultrasound permits the recognition of stenosis or obstruction of the hepatic portion of the IVC and adjacent hepatic veins, and is the recommended initial imaging modality.8 The condition has a good prognosis on prolonged antibiotic treatment.9
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