Clinical Features And Radiological Intervention Of Chronic Budd-Chiari Syndrome

Background:Budd-Chiari syndrome (BCS) is a heterogeneous disorder which is caused by the obstruction of outflow tract of hepatic veins at any level from the small hepatic veins to the junction of inferior vena cava and the right atrium, regardless of the cause of obstruction. The hepatic veno-occlusive disease and the disorders due to the cardiac conditions are excluded from the disease.BCS is classified into fulminant, acute, subacute and chronic form on the basis of its onset. The Onset of BCS was considered acute in the past but recent studies have demonstrated that BCS has chronic onset. Previously BCS was regarded as fatal disease with grave outcome. However, with the development of modern imaging techniques it has become very evident that BCS has an indolent course and an insidious onset. The clinical manifestations of BCS are abdominal pain, ascites, jaundice, hepatomegaly and rapidly progressive hepatic failure.Outcome of the disease depends on its etiology and clinical course. Patients with BCS often have clinical features which can be tolerated for many years, even decades, before the diagnosis is made.With the advancement and modernization in the field of non invasive imaging techniques BCS is easily, timely and accurately diagnosed. Advanced and improved non invasive imaging techniques have enabled us to broaden our treatment spectrum, which results in better outcome of BCS. With the advent of non-invasive imaging techniques, radiological intervention is becoming the treatment of choice and is being accepted by a large number of patients. The choice of radiological interventions depends on the aetiology and vascular anatomic abnormalities. Currently, Percutaneous Transluminal angioplasty PTA with or without stent implantation and Trans-artrial Intrahepatic Porto-systemic Shunt TIPS are the most commonly performed interventional procedures.In this retrospective study, Data of 586 patients with chronic BCS, who were admitted in our hospital from 1993 to 2014, was critically analysed. This large cohort of patients allowed us to have more comprehensive and authoritative recognition and better understanding of BCS in China. The long term follow up of radiological intervention and experience gave us more enlightenment on its treatment options.Materials & Methods:This retrospective study was conducted at the department of gastroenterology,Shandong provincial hospital, Shandong University, Jinan, Shandong, P. R. China from the year 1993 to 2014.In this study we analysed the data of 586 patients including clinical symptoms, laboratory tests, imaging results radiological interventions and follow up records. The underlying aetiology was known only in a few patients.All the patients were required to screen for following biochemical investigations such as, coagulation profile(prothrombin time or PT, international normalized ratio or INR, fibrinogen concentrate),blood routine(the count of red blood cell,white blood cell and platelet), liver function test and Screening for hepatitis B surface antigen (HBVs-Ag) as well as antibody to hepatitis B core antigen of IgM (anti-HBc IgM) at the time of hospital admission. Child-pugh classification was applied on all patients with or without liver cirrhosis.All the patients were diagnosed first by Doppler-ultrasonography,while computed tomography (CT) (n=64,11%), or preferably, magnetic resonance imaging (MRI) (n=18,3%) were used as a second line diagnostic modalities. The diagnosis was usually confirmed by inferior vena cavography (n=392,67%) or trans-hepatic venography (n=82,14%) to find the location, extent and length of the vascular abnormality and to define the treatment strategy. The vascular anatomical abnormalities were divided into three forms according to the findings of the imaging which are pure inferior vena cava (IVC) obstruction, pure hepatic vein (HV) obstruction and IVC-HV combined obstruction. Percutaneous transluminal angioplasty (PTA) with or without stent implantation was used as the choice of intervention for recanalization of obstructed vessels. Trans-jugular intrahepatic port-o-systemic shunt (TIPS) was performed in a few patients with diffuse obstruction of all three HVs.Recanalization of the obstructed vessels was carried out by percutaneous transluminal angioplasty (PTA) with or without stent implantation. PTA in case of obstructed IVC was performed via either right femoral vein route, right jugular vein route or both. For most of the patients the right femoral vein approach was adopted. In patients with a solid lesion, the recanalization of occluded IVC was done via right jugular vein route. However, a combination of trans-femoral approach and right jugular approach was used to ensure a safe recanalization in patients with a long segmental IVC obstruction. Balloon with a diameter of 18-30 mm was used to dilate the lesions of IVC. Balloons of smaller sizes were used initially to dilate the lesion and size was gradually increased until desired dilation was achieved. Expandable metallic sigma stent was implanted in the patients with recurrent stenosis of IVC, when balloon dilation alone was insufficient. The diameter of the stent was equivalent or 2-4 mm wider than the balloon used during PTA, while its length was 2-3 cm longer than the lesion. Two stents were implanted in IVC in the patients with long segmental obstruction.Recanalization of obstructed Hepatic Vein (HV) was carried out via right femoral vein, right jugular vein or direct percutaneous puncture. For the patients with HV stenosis, the trans-jugular or trans-femoral approach was used to access the HV through IVC. When it was not accessible through the IVC while ultrasound showed a patent venous lumen proximal to major HV, the percutaneous trans-hepatic approach was used, and a balloon was inflated as a target in the IVC. The diameter of the balloon used in HV was 10-15 mm. Stent implantation was undertaken in patients with HV obstruction. PTA in accessory hepatic vein was performed when the recanalization of the three main hepatic veins was not technically feasible, In patient with IVC-HV combined obstruction, Stent placement in IVC was carried out first while stent in HV was implanted 1-3 weeks later. The patients were required to receive anticoagulant treatment following the procedure for at least half a year.The follow-up of patients, who received radiological interventions, was done through outpatient visits at 6,12 and 24 months. The subsequent follow-up was carried out through telephonic interviews and voluntary out-patient visits for free examination until April 2015.Results:There were 345 males and 241 females, with a male-to-female ratio of 1.43:1. The mean duration of symptom was 58 months and abdominal distention was described as the main discomfort by 338 patients (57.68). A number of risk factors were found in 112 patients, and the development of BCS in 33% patients may be related to the poor hygiene.206 patients had pure inferior vena cava (IVC) obstruction,76 had pure hepatic vein (HV) obstruction, and 304 had IVC-HV combined obstruction.389 patients (66%) received radiological interventions and technical success rates were 96% and 90% for IVC and HV obstructions, respectively. The patency rates of IVC were 98.1%,90.8%,88.1%,66.0% at 12,24,60,120 months, while the primary patency rates were 93.5%,78.6%,67.4% at 12,24,60 months of HV.As mentioned above, according to the imaging examination,206 patients had pure IVC obstruction,76 had pure HV obstruction, and 304 had IVC-HV combined obstruction. Three hundred and eighty-nine patients received radiological intervention. The longest follow-up was 200 months.Conclusion:The prevalence of BCS is relatively high in China, and its clinical presentation varies from patient to patient. The percutaneous transluminal angioplasty (PTA) with or without stent implantation proved to be an effective treatment strategy and accepted by most of the patients.