Asian Cardiovasc Thorac Ann 1998;6:141-142
© 1998 Asia Publishing EXchange Pte Ltd
Balloon Dilatation of Membranous Obstruction of the Inferior Vena Cava
Hemant Pramod Pathare, MS,
Reshma Manoj Biniwale, MS
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PK Sen Department of Cardiovascular & Thoracic Surgery Seth GS Medical College & King Edward VII Memorial Hospital Mumbai, India
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For reprint information contact: Hemant Pramod Pathare, MS 6 Queen's Croft Society St. Xaviers School Road Ville-Parle (West) Mumbai 400056, India Tel: 91 22 611 3358 Fax: 91 22 611 5958
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ABSTRACT
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Membranous obstruction of the inferior vena cava is a rare congenital anomaly that results in the primary type of Budd-Chiari syndrome. We describe the case of an 8-year-old boy initially diagnosed with intrahepatic portal hypertension, who underwent percutaneous transluminal balloon dilatation of an inferior vena cava membrane located in the suprahepatic inferior vena cava, which resulted in successful palliation of his symptoms.
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INTRODUCTION
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Membranous obstruction of the inferior vena cava is a rare congenital anomaly that results in the primary type of Budd-Chiari Syndrome. Until recently, surgical treatment with its associated morbidity was often the only available option. Percutaneous balloon dilation of the inferior vena cava membrane is now a viable alternative in some patients.
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CASE REPORT
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An 8-year-old boy presented with history of gradual distension of the abdomen associated with anorexia and cachexia over a period of 3 years. He had a single bout of hematemesis of approximately 300 mL, 6 months previously. There were prominent superficial veins over the anterior abdominal wall. Esophageal endoscopy revealed grade II esophageal varices that were sclerosed with 3% phenol solution at this time. The patient was anicteric and afebrile. There was no history of hepatitis. Abdominal ultrasound demonstrated gross hepatomegaly with a coarse echotexture, splenomegaly, and approximately 1 L of ascitic fluid. The kidneys were normal. The inferior vena cava in its abdominal course appeared to be normal. The serum bilirubin level was minimally raised, aspartate aminotransferase, alanine aminotransferase, and prothrombin time were within normal limits. There was mild anemia (hemoglobin 85 g•L–1) and hypoalbuminemia (albumin 25 g•L–1). A 99mTc-phytate scan suggested chronic liver disease. Histopathology showed minimal portal fibrosis but the hepatocytes appeared normal. Normal serum ceruloplasmin and copper levels ruled out Wilson's disease. Thus, a diagnosis of non-cirrhotic portal fibrosis was entertained.
At this stage the boy was referred to our department. Our first diagnosis was hepatic venous thrombosis. Abdominal Doppler ultrasonography revealed narrowing of the diaphragmatic portion of the inferior vena cava with turbulence near its junction with the right atrium. The hepatic veins were dilated. An inferior vena caval angiogram revealed a caval membrane at the junction of the inferior vena cava and the right atrium with a very small central perforation and extensive collateralization via the azygous pathway (Figure 1
). A guide wire was passed through this central perforation and a balloon catheter was threaded over it. The membrane was ruptured with a percutaneous transluminal balloon (12 
40 mm; 5 F; Cook, Bloomington, IN, USA) under fluoroscopic guidance (Figure 2). The child withstood the procedure well and was heparinized for 24 hours afterwards. At the 3-month follow-up, he was well and the hepatomegaly, splenomegaly, and ascites had disappeared. Bilirubin levels were within normal limits and the serum albumin was 32 g•L–1. An abdominal ultrasound study showed the hepatic veins to have decreased in size.
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Figure 1. Angiogram of the inferior vena cava showing abrupt cut-off at the level of diaphragm due to the supra-hepatic membrane.
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Figure 2. Blood in the inferior vena cava flowing unimpeded into the right atrium after successful percutaneous transluminal balloon membranotomy.
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DISCUSSION
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Inferior vena caval membranes are rare entities in medical literature. In our case, the membrane was located above the openings of all 3 hepatic veins into the abdominal inferior vena cava, producing primary Budd-Chiari syndrome, a congenital obstruction of the hepatic veins or the hepatic portion of the inferior vena cava. Such membranes vary from very thin to several centimeters thick and can sometimes occlude the long segment of the retrohepatic inferior vena cava.1 The more common secondary type of Budd-Chiari syndrome is due to obstruction by tumors or thrombi, especially seen in myeloproliferative disorders. Membranous obstruction of the inferior vena cava is the most common cause of primary Budd-Chiari syndrome. It is seen in people of Japanese, Chinese, and Indian descent.2 It manifests clinically in adulthood (20 to 30 years) and less commonly in the second decade. Clinically, the symptoms are suggestive of portal hypertension and inferior vena caval obstruction. An important additional sign is edema of the lower extremities and the lower trunk.
Abdominal ultrasound always reveals dilatation of the hepatic veins as well as the inferior vena cava and this differentiates it from cirrhosis wherein the hepatic veins are small, narrow, and tortuous.3 Doppler ultrasound studies may show an absence or reversal of flow in the portal vein. Magnetic resonance imaging may successfully diagnose a caval membranous obstruction without the need for inferior vena caval angiography.
It was possible to carry out a percutaneous transluminal balloon membranotomy in this case because the balloon catheter was threaded over the guide wire passed through the central perforation in the inferior vena caval membrane. Yang and colleagues2 reported good results from percutaneous transluminal balloon membranotomy. If balloon or blade membranotomy fails or if there is a thrombus that may result in pulmonary embolism, then surgical options remain. These include transatrial membranotomy, transcaval liver resection with hepatoatrial anastomosis, direct reconstruction of the inferior vena cava, a proximal cava-to-right atrial bypass graft or a meso-atrial shunt if the long segment of the inferior vena cava is involved.4 However, prosthetic grafts in the venous circulation are prone to a high rate of occlusion in spite of long-term anticoagulation.
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REFERENCES
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Orloff MJ. Budd-Chiari syndrome and veno-occlusive disease. In: Blumgart LH, editor. Surgery of the liver and biliary tract. New York: Churchill Livingstone, 1988;1425–53.
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Yang XL, Cheng TO, Chen CR. Successful treatment by percutaneous balloon angioplasty of Budd-Chiari syndrome caused by membranous obstruction of IVC. J Am Coll Cardiol
1996;28:1720–4.[Abstract]
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Kamba M, Ochi S, Ochi H, et al. Asymptommatic membrane obstruction of IVC forming intra-hepatic collateral pathways. J Gastroenterol
1995;30:783–5.[Medline]
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Zamboni P, Pisano L, Mari C, et al. Membranous obstruction of IVC and Budd-Chiari syndrome. J Cardiovasc Surg (Torino)
, 1996;37:583–7.[Medline]
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ABSTRACT
INTRODUCTION
