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Emergency Ultrasound

International Ultrasound: Hepatic Vena Cava Syndrome

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.

 

Fig1 Intl

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

 

 Fig2 Intl

Fig. 2: An extensive non-homogenous echogenicity (thrombus) in the IVC

 

 Fig3 Intl

Fig. 3: The thrombus in the IVC extends minimally into the hepatic veins

 

 Fig4 Intl

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

 

References:

 

1.   Janssen HLA, Gaecia-Pagan J-C, Elias E,et al. Budd–Chiari syndrome: a review by an expert panel. J Hepatol. 2003; 38: 364–71.

2.   Shrestha SM. Membranous obstruction of hepatic portion of the inferior vena cava: is this an underdiagnosed entity in developing countries. Am J Gastroenterol. 1995; 90: 303–6. 


3.   Shrestha SM, Shrestha S. Hepatic vena cava disease: etiologic relation to bacterial infection. Hepatol Res. 2007; 37: 196–204. 


4.   Kage M, Arakawa M, Kojiro M, Okuda K. Histopathology of membranous obstruction of the inferior vena cava in the Budd Chiari syndrome. Gastroenterol. 1992; 102: 2081–90.

5.   Shrestha SM, Ghimire RK, Basnyat PV, et al. Acute on chronic phenomenon in hepatic IVC obstruction: a case re- port. Trop Gastroenterol. 1999; 20: 182–4.

6.   Shrestha SM, Shrestha S. Bacterial peritonitis in the hepatic inferior vena cava disease: a hypothesis to explain the cause of infection in high protein ascites. Hepatol Res. 2002; 24: 42–9.

7.   Lois JF, Hartzman S, McGlade CT, et al. Budd–Chiari syndrome: treatment with percutaneous transhepatic recanalization and dilation. Radiology. 1989; 170: 791–3.

8.   Chawla Y, Kumar S, Dhiman RK, et al. Duplex Doppler sonography in patients with Budd-Chiari syndrome. J Gastroenterol Hepatol. 1999; 14: 904-907. 


9.   Shrestha SM. Liver cirrhosis in hepatic vena cava syndrome (or membranous obstruction of IVC). World J Hepatol. 2015; 7: 874–84.

 

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