Abstract
A young female presented with Budd–Chiari syndrome due to membranous obstruction of inferior vena cava. Membrane was quite thick with complete occlusion of the IVC. She had a previous unsuccessful attempt at endovascular stenting using conventional CTO wire technique. She was successfully treated with balloon angioplasty and stenting after perforation of the thick membrane with Brokenborough's needle. At three months follow-up she was asymptomatic with patent stent.
Keywords: Angioplasty, Vena cava, Inferior, Budd–Chiari syndrome
1. Case report
A previously healthy 41-year-old female was referred to our center with 2 months history of abdominal pain, abdominal distention, pedal edema and loss of appetite. There was no history of jaundice. She was diagnosed to have hypothyroidism two years back and was on thyroid hormone replacement. Clinical examination revealed markedly distended abdomen with prominent superficial veins and bilateral pedal edema. There was no jaundice and JVP was not elevated. Vitals were stable. Laboratory examination at another hospital revealed a total bilirubin of 1.6 mg/dl and mildly raised liver enzymes. Ultrasound of abdomen showed moderate ascites, hepatomegaly and complete occlusion of proximal inferior vena cava (IVC). Endoscopy revealed oesophageal varices. Contrast enhanced computed tomography was done which revealed a thick web in proximal IVC with complete occlusion (Fig. 1). Patient was referred to us for PTA and stenting of IVC after a failed attempt at another hospital. Patient was already on oral anticoagulation for the last 6 weeks at the time of presentation at our center.
Fig. 1.

Contrast enhanced CT of the abdomen showing complete occlusion of proximal IVC (arrows).
Patient was primed with 300 mg of Clopidogrel and 325 mg of Aspirin. Venous access was obtained via 6 Fr sheaths in right Internal Jugular Vein (IJV) and right Femoral Vein (FV). Unfractionated heparin (5000 units) was given. Venography was performed by simultaneous contrast injections via NIH catheter in right IJV and pigtail catheter in right FV in two orthogonal views. It revealed a short completely occluded segment (thick web) in proximal IVC with post stenotic dilatation (Fig. 2). The thick web was punctured with a Brokenborough's needle. After confirming the position of Brokenborough's needle by contrast injection, Mullin's sheath was advanced over the needle into the IVC beyond the occlusion. Then a 0.032″ J-tip wire was negotiated across the web and was parked in right subclavian vein via superior vena cava. The web was first dilated with septal dilator over the 0.032″ wire and then sequentially with 6 × 15 mm and 12 × 40 mm balloons (Fig. 3). Proximal IVC was then stented with 22 × 45 mm self-expanding wall stent. IVC venogram post stenting showed good stent apposition with brisk flow (Fig. 4). Distended superficial abdominal veins disappeared on table itself. Patient was ambulated the following day and she had marked reduction in abdominal girth. She was given unfractionated heparin for 2 days and was discharged on oral anticoagulation on 3rd post-procedure day. Patient was asymptomatic at 3 months follow up and venous Doppler revealed patent IVC stent.
Fig. 2.

IVC venogram showing a thick membrane in proximal IVC causing complete occlusion.
Fig. 3.

Dilatation of proximal IVC with 12 × 40 mm balloon.
Fig. 4.

Venogram showing restoration of IVC patency after deployment of 22 × 45 mm self-expanding wall stent.
2. Discussion
Budd–Chiari syndrome is defined as hepatic venous outflow track obstruction at any level from small hepatic veins to right atrium-IVC junction, regardless of the cause of obstruction.1,2 Pure IVC obstruction is more common in Asia whereas pure hepatic vein obstruction predominates in Western countries.3 Most common cause of IVC obstruction is by membranous web. Membranous webs were earlier thought to be of congenital origin, but more recent evidence suggests that membranous webs are the sequelae of thrombus formation.4–6
Treatment of BCS varies depending on the etiology, presentation and the level of obstruction. It includes thrombolysis, surgical porto-systemic shunts, transjugular intrahepatic porto-systemic shunt (TIPS), endovascular PTA and liver transplantation. Endovascular angioplasty and stenting is an important modality of treatment in the subset of patients with membranous obstruction of IVC. Angioplasty alone has high recurrence rates due to elastic recoil. Hence, stent placement in IVC after balloon angioplasty is recommended. Angioplasty and stenting may be technically challenging when a thick membrane is encountered as in our case. In such cases, using Brokenborough's needle is a safe and effective way of increasing the success rates of the procedure as demonstrated.
Zhang et al reported the long term effects of stent placement in IVC or hepatic veins in 115 patients. In this study 96.7% of IVC stents remained patent over a mean follow up of 49 months. Absence of anticoagulants after the procedure was associated with higher incidence of stent occlusion.7 In another study by Srinivas BC et al, twelve cases of hepatic vein outflow obstruction who underwent endovascular balloon dilatation ± stenting were followed up for upto 13 years. There was only one case of restenosis.8 Thus, endovascular management of IVC obstruction has long term patency rates.
In BCS with membranous obstruction of IVC, PTA and stenting should be the treatment of choice as it can be accomplished with least morbidity and mortality and excellent long term patency rates.
Conflicts of interest
All authors have none to declare.
References
- 1.Janssen H.L., Garcia-Pagan J.C., Elias E., Mentha G., Hadengue A., Valla D.C. Budd–Chiari syndrome: a review by an expert panel. J Hepatol. 2003;38:364–371. doi: 10.1016/s0168-8278(02)00434-8. [DOI] [PubMed] [Google Scholar]
- 2.de Franchis R. Evolving consensus in portal hypertension report of the Baveno IV Consensus Workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol. 2005;43:167–176. doi: 10.1016/j.jhep.2005.05.009. [DOI] [PubMed] [Google Scholar]
- 3.Valla D. Hepatic venous outflow tract obstruction etipathogenesis: Asia versus the West. J Gastroenterol Hepatol. 2004;19:S204–S211. [Google Scholar]
- 4.Kage M., Arakawa M., Kojiro M., Okuda K. Histopathology of membranous obstruction of the inferior vena cava in the Budd–Chiari syndrome. Gastroenterology. 1992;102:2081–2090. doi: 10.1016/0016-5085(92)90336-w. [DOI] [PubMed] [Google Scholar]
- 5.Kew M.C., Hodkinson H.J. Membranous obstruction of the inferior vena cava and its causal relation to hepatocellular carcinoma. Liver Int. 2006;26:1–7. doi: 10.1111/j.1478-3231.2005.01194.x. [DOI] [PubMed] [Google Scholar]
- 6.Terabayashi H., Okuda K., Nomura F., Ohnishi K., Wong P. Transformation of inferior vena caval thrombosis to membranous obstruction in a patient with the lupus anticoagulant. Gastroenterology. 1986;91:219–224. doi: 10.1016/0016-5085(86)90462-2. [DOI] [PubMed] [Google Scholar]
- 7.Zhang C.Q., Fu L.N., Xu L. Long-term effect of stent placement in 115 patients with Budd–Chiari syndrome. World J Gastroenterol. 2003;9:2587–2591. doi: 10.3748/wjg.v9.i11.2587. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Srinivas B.C., Dattatreya P.V., Srinivasa K.H., Prabhavathi, Manjunathan C.N. Inferior vena cava obstruction: long-term results of endovascular management. Indian Heart J. 2012 Mar–Apr;64:162–169. doi: 10.1016/S0019-4832(12)60054-6. [DOI] [PMC free article] [PubMed] [Google Scholar]
