Clinical features, image analysis, and laparoscopic and histological liver findings in Budd-Chiari syndrome

BACKGROUND/AIMS: Clinical manifestations and histological features of the liver in Budd-Chiari syndrome (BCS), with or without idiopathic membranous obstruction of the inferior vena cava (MOVC), vary according to whether BCS is acute, subacute or chronic. We clarified the diagnostic features in 6 patients with MOVC and in 1 without MOVC. METHODOLOGY: Five patients with subacute or chronic type BCS with MOVC complaining of epigastric pain, hematemesis and encephalopathy, and signs of portal hypertension or collateral circulation were seen. There was 1 asymptomatic patient with MOVC. One patient with acute type BCS without MOVC revealed hepatic and multi-organ failure. Liver function tests in BCS with MOVC were similar to those in liver cirrhosis, and laboratory data in acute type without MOVC were quite the same as those seen in fulminant hepatitis. Non-invasive image analysis by US, CT and MRI showed thrombi and obstruction of the IVC, and extrahepatic vasculature or communication between hepatic veins and IVC. Vena cavography showed the length of obstruction in IVC and collateral circulation in the extrahepatic or intrahepatic veins. Liver biopsy demonstrated massive hemorrhagic necrosis in acute type without MOVC, and laparoscopy with liver biopsy in asymptomatic, subacute and chronic type with MOVC showed subcapsular hemorrhage, congestion, fibrosis, and cirrhotic features. CONCLUSIONS: The non-invasive image analysis was complementary to vena cavography, and liver biopsy with or without laparoscopy was essential not only for diagnosis of acute, subacute, and chronic BCS, but also for therapeutic decision-making.     

Proposal of a new nomenclature for Budd-Chiari syndrome: hepatic vein thrombosis versus thrombosis of the inferior vena cava at its hepatic portion

Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins, but subsequent reports on various obliterative changes that occur in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices have resulted in a broadened and ambiguous definition. Membranous obstruction of the inferior vena cava has been regarded by many as a congenital vascular malformation, but its relation to the classical BCS has remained obscure. With modern imaging and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to classify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review, we emphasize that primary hepatic vein thrombosis (classical Budd-Chiari) and an obliterative disease predominantly affecting the hepatic portion of the IVC, both of which account for most patients with venous outflow block, are clinically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow block in developing countries such as Nepal, South Africa, China, and India. The latter is frequently complicated by hepatocellular carcinoma (HCC), and primary hepatic vein thrombosis is not. The major cause of thrombosis is a hypercoagulable state in hepatic vein thrombosis, but more of the latter cases are idiopathic. The clinical presentation of the latter is milder, and onset is frequently inapparent, whereas the former is more severe, sometimes causing acute hepatic failure. Markedly enlarged subcutaneous veins over the body trunk characterize the latter. We propose that these two disorders be clinically distinguished with a suggested term "obliterative hepato-cavopathy" for the latter against classical BCS.     

Budd Chiari syndrome and pericaval filariasis

A young girl presented with Budd-Chiari syndrome (BCS) with narrowing of the retrohepatic segment of inferior vena cava (VC). Dorsal cavoatrial bypass (DCAB) was unsuccessful due to thrombotic occlusion of the graft. A limited autopsy revealed occlusion of the retrohepatic segment of IVC and the terminal parts of the three major hepatic veins. Caval occlusion was just above the level of the superior hepatic veins, and caused by a transverse fibrous shelf. An adult filarial worm was found amidst pericaval fibrosis. Filariasis should be included as a possible aetiological factor in chronic BCS.     

Anatomic arrangement of the hepatic veins in the goat

In gross anatomic and angiographic examinations of the hepatic veins of 15 goats, the following were seen: (i) The middle hepatic vein was formed by the contribution of two main branches, one branch coming from the quadrate lobe and the other branch from the right lobe. (ii) There were two right hepatic veins, one of them running along the caudate process and the other formed by branches coming from the dorsal part of the right lobe. (iii) The entrance of the middle hepatic vein, as well as the entrance of the left hepatic vein into the caudal vena cava were partly covered by valvelike membranous flaps.     

Hepatic vein stenting for Budd-Chiari syndrome

This report describes two patients with Budd-Chiari syndrome with intractable ascites due to a tight hepatic vein stenosis while the other hepatic veins were occluded. Percutaneous transluminal angioplasty of the hepatic vein stenosis followed by insertion of expandable metallic stents reduced the pressure gradient across the stenosis to almost zero. In both patients, ascites disappeared and diuretic therapy could be reduced significantly. This treatment has remained effective for more than 1 yr in one case and 2 yr in the other. These cases demonstrate the feasibility of hepatic vein stenting as a therapy for hepatic venous outflow obstruction. This therapy may be used in selected patients to defer and perhaps avoid shunt-surgery or liver transplantation.     

Alveolar echinococcosis of the liver: sequelae of chronic inferior vena cava obstructions in the hepatic segment

BACKGROUND: The clinical pattern and long-term course of chronic inferior vena cava (IVC) obstructions are variable and depend on the underlying cause, the segment involved, and the extension of secondary thrombosis. Pertinent data on IVC obstructions in well-defined series of patients are lacking. We report the sequelae of chronic IVC obstructions in the hepatic segment in 11 consecutive patients derived from a cohort of 104 patients with alveolar echinococcosis of the liver. METHODS: Based on the results of computed tomography scans, 11 patients (7 men, 4 women; mean age, 53.4 years) with IVC obstructions were selected from an ongoing prospective long-term chemotherapy trial comprising 104 patients with alveolar echinococcosis studied at yearly intervals according to a protocol. Obstruction of the IVC in the 11 patients existed for a mean duration of 8.6 years. In these patients, magnetic resonance imaging was performed to assess the morphologic features and extension of the IVC obstruction, as well as the collateral venous pathways. Patency and valvular function of the femoropopliteal veins were analyzed by color-coded duplex ultrasonography. RESULTS: Total occlusions of the IVC were evident in 8 patients (73%) and subtotal stenoses in 3 patients (27%). Only 4 patients (36%) exhibited signs and symptoms of chronic venous insufficiency of the lower extremities; 2 (18%) of the 4 had a history of swelling in the lower extremity. Seven patients (64%) had no lower extremity symptoms. One patient had a history of pulmonary embolism. Abdominal collateral veins were documented in 5 patients (45%) by using magnetic resonance imaging; however, they were clinically evident in only 3 patients (27%). In the 8 patients with IVC occlusion, thrombosis ended at the confluence of the hepatic veins. Obstruction of the IVC was limited to the hepatic segment in 2 patients (18%) and extended to the distal IVC or the iliofemoral veins in 6 patients (54%). Chronic venous insufficiency was present only if the femoropopliteal veins had been involved in the thrombotic process, showing residual venous obstruction, valvular incompetence, or both. Bilateral renal vein thrombosis with moderate proteinuria was observed in 2 patients (18%). The main collateral drainage was achieved through the ascending lumbar, azygos, and hemiazygos veins. CONCLUSIONS: In patients with alveolar echinococcosis, obstruction of the IVC in the hepatic segment often develops asymptomatically and rarely leads to the impairment of renal function. The collateral circulation fully compensates for obstruction of the IVC. Thrombotic involvement and valvular incompetence of the femoropopliteal veins seems to determine the development of chronic venous insufficiency of the lower extremities.     

Budd-Chiari syndrome: a common complication of Beh?et's disease

OBJECTIVE: The Budd-Chiari syndrome is characterized by venous outflow obstruction of the liver, usually occurring as a consequence of thrombosis of the hepatic veins. Vasculitis is a major component of Beh?et's syndrome. The aim of this study was to determine the incidence of hepatic vein thrombosis in patients with Beh?et's disease and to estimate the effect of this entity upon the clinical features and course of Beh?et's syndrome. METHODS: During an 8-yr period from 1985 to 1994, from a total of 493 patients with Beh?et's disease seen at Hacettepe University Hospital, the incidence and effect of hepatic vein thrombosis on the clinical course of Beh?et's syndrome was investigated. The hepatic vein thrombosis in each case was documented by hepatic venography and confirmed by digital subtraction angiography, computed tomography, ultrasonography, and liver biopsy. Coagulation parameters including protein C, protein S, and anti-thrombin III levels were easured in each case. The survival of cases with Beh?et's syndrome complicated by Budd-Chiari syndrome and the effect of the Budd-Chiari syndrome on the survival of individuals with Beh?et's syndrome were determined using the Kaplan-Meier technique. RESULTS: Of the 493 cases of Beh?et's syndrome, 53 (10.8%) were found to have one or more large vessel thrombosis. Of these 53 patients, 14 (26.4%) had hepatic vein thrombosis. Of these 14 patients, 8 had an additional inferior vena cava thrombosis and 4 had portal vein as well as total inferior vena cava thrombosis. Only two patients with isolated hepatic vein thrombosis were identified. These two patients and two additional patients with hepatic vein thrombosis plus thrombosis of the hepatic portion of the inferior vena cava are currently alive. Of the 10 patients with total inferior vena cava and hepatic vein thrombosis (4 also had portal vein thrombosis), all 10 died with a mean survival of 10.3 months. During the same time period, 37 patients obtained from a total of 1494 patients with clinical evidence of either portal hypertension, hepatic venous outflow obstruction or inferior vena caval obstruction without Beh?et's syndrome were found to have a Budd-Chiari syndrome. Of these 37 patients, 19 (51%) had an identifiable underlying disorder responsible for their hepatic vein thrombosis. CONCLUSION: Based upon this experience, it appears as if Budd-Chiari syndrome is a relatively frequent complication of Beh?et's disease. When individuals with Beh?et's syndrome have BCS, concurrent thrombosis of the portal vein and inferior vena cava are often found, if the patency of these vessels is assessed. The clinical course of patients with Beh?et's syndrome complicated by Budd-Chiari syndrome is poor. The extent of the vascular thrombosis within the inferior vena cava rather than the presence of the hepatic vein thrombosis per se is the major determinant of survival.     

Subpulmonary obstruction in congenitally corrected transposition of the great arteries due to ventricular membranous septal aneurysms

The clinical, hemodynamic, and angiographic observations, as well as the surgical approach used for repair in three patients with congenitally corrected transposition of the great arteries and ventricular membranous septal aneurysms, are presented. In two of the three patients the membranous septal aneurysm caused subpulmonary obstruction, with 94 and 125 mm Hg systolic gradients. In each patient the aneurysm was demonstrated by angiocardiography, which also showed differences in size and shape with cardiac systole and diastole. Review of the previously described reports indicates that patients with congenitally corrected transposition often display various forms of pulmonary outflow obstruction and when a ventricular membranous septal aneurysm exists, a significant subpulmonary obstruction is present in most patients. The unique anatomic relationship between the pulmonary artery and a ventricular membranous septal aneurysm in patients with transposition of the great arteries with and without atrioventricular discordance explains why subpulmonary obstruction sometimes develops.     

Calcified thrombus in the inferior vena cava in infants and children

Calcified thrombi in the prerenal (suprarenal) segment of the inferior vena have a characteristic radiographic appearance that permits accurate "plain film" diagnosis. Most have been fortuitously discovered in infants and young children. None of the affected individuals has had clinical evidence of venous obstruction. Vena caval obstruction is usually incomplete. Uncalcified clot caudal to the calcified thrombus caused complete obstruction of the inferior vena cava and renal veins in one of our patients, a healthy infant; since collateral flow was adequate surgery was not advised. We believe that aggressive diagnostic and therapeutic measures are unnecessary in the management of infants and children with calcified thrombi in the inferior vena cava.     

Spiral structures in the wall of the hepatic venous system in the dog

Unique spiral structures, located in the wall of the hepatic venous system in the dog, were examined in the central veins and the hepatic venous branches, utilizing microvascular corrosion casting and freeze-fracture technique in scanning electron microscopy and transmission electron microscopy of tissue sections. The whole hepatic venous system was divided into 4 portions: the central, sublobular, collecting and branches of the hepatic veins. The central vein was spindle-shaped with several compressions. Removing the endothelial cells of the central vein, pathways of venous sinusoids were like a labyrinth. In the sublobular veins, spiral structures distinctly appeared as the diameter increased. Beneath the endothelial cells in the constricted portions, smooth muscle bundles were found. The spiral structures gradually became irregular in the collecting veins and discontinuous to form shallow constrictions in cast thicker branches of the intrahepatic veins. A single, fine spindle of the central vein was formed by the arrangement of liver cells. The spiral structures of the sublobular vein were formed by smooth muscle bundles. Irregularity of the spiral structures in the collecting veins was caused by smooth muscle bundles anastomosing with adjacent ones. Disappearance of the spiral structure in cast thicker branches of the intrahepatic veins was due to absence of muscle bundles.     

Successful treatment by percutaneous balloon angioplasty of Budd-Chiari syndrome caused by membranous obstruction of inferior vena cava: 8-year follow-up study

OBJECTIVES: This study sought to report the long-term result (up to 8 years) of percutaneous transluminal balloon angioplasty (PTBA) for Budd-Chiari syndrome (BCS) caused by membranous obstruction of the inferior vena cava (MOVC). BACKGROUND: We previously reported on this nonoperative form of therapy in a smaller series of patients and found the short-term results to be excellent. METHODS: We studied the long-term results of PTBA in the treatment of BCS caused by MOVC in 42 patients who underwent PTBA with the Inoue balloon catheter between June 1988 and February 1996. There were 28 men and 14 women with a mean age of 35.6 years (range 16 to 56). MOVC was incomplete in 27 patients and complete in 15. PTBA was successful in 38 patients (91%). The longest follow-up period was 8 years. RESULTS: All 38 patients who successfully underwent PTBA showed marked symptomatic improvement. Immediately after PTBA, the diameter of the inferior vena cava at the MOVC increased from 1.7 +/- 2 to 19.9 +/- 3.5 mm (p < 0.0001), the caval pressure below the MOVC decreased from 23.6 +/- 8.5 to 12.0 +/- 6.5 mm Hg (p < 0.0001), and the enlarged liver size decreased from 6.5 +/- 1.5 to 2.0 +/- 1.5 cm below the right costal margin at the midclavicular line (p < 0.0001). Over a follow-up period of up to 8 years (7 to 8 years in 4 patients, 5 to 7 years in 12, 3 to 5 years in 11, 2 to 3 years in 6 and < 2 years in 9), MOVC returned in only 1 patient. This patient, our first, required a second PTBA 3 years later and a third 4.25 years after the second PTBA, in combination with stent placement for recurrence of stenosis. CONCLUSIONS: PTBA with the Inoue balloon catheter is an effective, safe and long-lasting alternative to surgical treatment of patients with BCS due to MOVC.     

Retrohepatic cavoatrial shunt with a ringed polytetrafluoroethylene graft for the Budd-Chiari syndrome. A case report

This report describes a patient with Budd-Chiari syndrome who was operated on successfully by means of shunt formation with polytetrafluoroethylene graft between the inferior vena cava (IVC) and right atrium. The patient is a sixty-two-year-old woman suffering from persistent edema of the lower limbs for four years. The examination disclosed complete obstruction of the IVC at the level of the diaphragm with a patent right inferior hepatic vein. Following the operation, edema of the limbs disappeared, hypersplenism improved, and the serum ammonium concentration decreased to the normal range. In conclusion, a retrohepatic cavoatrial shunt is feasible and useful in treating a patient with the Budd-Chiari syndrome who has patent major hepatic veins.     

Budd-Chiari syndrome. Treatment options and the value of liver transplantation

The Budd-Chiari syndrome encompasses a group of conditions that cause partial or complete obstruction of the hepatic venous outflow tract. This leads to hepatocyte necrosis which can manifest as a devastating fulminant illness, or a more indolent condition that eventually presents with features of portal hypertension. Doppler ultrasonography of the hepatic veins and inferior vena cava has become the initial diagnostic test of choice, but most patients require venography and liver biopsy prior to definitive therapy. Multiple therapeutic modalities have been used for the Budd-Chiari syndrome, including medical, radiological, and surgical approaches. The role, indications, and outcome of liver transplantation for individuals with the Budd-Chiari syndrome are discussed.     

Intermittent complete vascular exclusion of the liver during hepatectomy: technique and indications

BACKGROUND/AIMS: Complete intermittent vascular exclusion of the liver (IVEL) combines clamping of the hepatic pedicle with clamping of the hepatic veins without interruption of the caval flow. The major advantages of this technique are that patient preclamping fluid overload is avoided, major haemodynamic changes due to caval clamping are escaped, and it allows a very long clamping time. Disadvantage of this technique is the necessity of looping the terminal part of the hepatic veins. METHODOLOGY: In this prospective study, 41 cases of IVEL (Representing 19% of the hepatectomies carried out for cancer during the same period) used for difficult hepatectomies were analyzed, and the operative technique is presented. RESULTS: IVEL was feasible in 90% of the 46 attempted cases, and completely controlled the bleeding in 90% of the cases. The mean duration of IVEL was 69.2 minutes (Range: 37 to 140), and was greater than 130 minutes in three patients. No liver failure occurred during the postoperative course. CONCLUSION: We conclude that IVEL without caval clamping is a new, and valuable, technique of vascular exclusion of the liver. Its application is indicated in the following conditions: 1. For patients who should have classical vascular exclusion but cannot tolerate vena cava clamping (18% of the cases), 2. for patients with pathological liver parenchyma when intrahepatic venous pressure is high, 3. for patients with impaired liver parenchyma, requiring conservative surgery that leads to anatomic or non-anatomic resection close to a vein (Example: A tumor located in the dihedral angle of the terminal part of two hepatic veins), 4. for patients with tumors closely located to a hepatic vein that must be preserved and sharply dissected (Example: A left trisegmentectomy that requires pelting of the right hepatic vein), and 5. for the scarce patient with tumors infiltrating the major hepatic veins, constraining a hepatic vein reconstruction to preserve liver function.     

Solitary intra-articular osteochondroma of the fingers in children

Injuries to the hepatic veins and retrohepatic vena cava have a high mortality due to uncontrolled hemorrhage. Successful repair may necessitate interruption of flow through the retrohepatic vena cava. Active bypass of the area is then needed to provide adequate venous return. Published methods for active bypass require cannulation of axillary and femoral veins in addition to clamping of the vena cava above and below the liver, often with limited exposure and significant risk. This report describes active bypass of the retrohepatic vena cava utilizing two right atrial cannulae. The simplicity of establishing the bypass together with the excellent exposure allowed repair of a bullet wound of the vena cava in one patient and the orderly performance of a left trisegmentectomy for a huge hepatic tumor in a second patient.     

Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations

Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and normal pulmonary parenchyma. Of nine previously described necropsy patients with pulmonary hypertension due to fibrosing mediastinitis, seven had severe narrowing of multiple large pulmonary veins and in six of them the pulmonary hypertension was entirely due to pulmonary venous obstruction. In one other patient, the pulmonary hypertension was due to obstruction of one main pulmonary artery and several large pulmonary veins. Each of these seven previously described patients had severe changes in the small intrapulmonary arteries. Of the other two previously described patients with pulmonary hypertension from fibrosing mediastinitis, one had severe narrowing of only the main right pulmonary artery, and the other, of both main pulmonary arteries. Thus, although pulmonary arterial hypertension in patients with fibrosing mediastinitis is usually due to obstruction of multiple large pulmonary veins and to severe secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension by obstructing the right or both main pulmonary arteries.     

Normal and pathological anatomy and physiology of the veins of the lower limbs

Veins of the lower limbs are divided into two regions: 1) the superficial region, composed of two saphenous veins, internal and external, and of their tributaries; 2) the deep region, composed of veins that are satellites (and homonyms) of the arteries (anterior and posterior tibial; peroneal; popliteal; superficial, deep and common femoral; external, epigastric and common iliac). The two networks are linked by perforating (or communicating) veins and by the arches of the two saphenous veins. Valvules are found in the venous network below the inguinal ligament; their number decreases with increasing proximity to the inferior vena cava. The function of the valves is the main factor in the physiology of venous circulation, in association with the force essentially provided by the "muscle pump" of the calf. The various forms of venous insufficiency are due to obstruction or incontinence of the veins.     

Temporal changes in PO2 of R3230AC tumors in Fischer-344 rats

It has been hypothesized that abdominal trauma may be one of the factors involved in membranous obstruction of the inferior vena cava. We present two cases of membranous obstruction of the inferior vena cava associated with trauma. One asymptomatic case, associated with an occult myeloproliferative disorder, developed within 3 years of a violent abdominal trauma. The other case, associated with familial plasminogen deficiency, was discovered at surgery 3 days after a road accident with obvious abdominal trauma, since superimposed extensive thrombosis of the inferior vena cava caused acute Budd-Chiari syndrome. We conclude that underlying prothrombotic conditions are probably necessary for the development of membranous obstruction of the inferior vena cava and that minor trauma may contribute to the development of thrombosis through indirect mechanisms.     

Doppler ultrasound of blood flow in the hepatic veins

The blood flow in the hepatic veins can normally be studied easily by Doppler ultrasound. The pattern of blood flow in normal individuals is described, and its relation to the cardiac cycle and changing pressure in the right atrium. The blood flow shows variations in healthy persons, and may change in cases of heart disease and hepatic disease. Conditions such as atrial fibrillation, tricuspid regurgitation, abnormal relaxation, restrictive cardiomyopathy, constrictive pericarditis and cardiac tamponade are reflected in the hepatic veins, and the pattern of blood flow may help in diagnosis, and in grading the pathology. In cirrhosis and portal hypertension the heart-synchronous variation in velocity is reduced. This is due to increased resistance to blood flow across the liver and the pressure gradient becoming larger than the variations in pressure in the right atrium.     

Acute acalculous cholecystitis associated with common hepatic duct obstruction: a variant of Mirizzi's syndrome

A variation of Mirizzi's syndrome is described in which common hepatic duct obstruction occurs due to extrinsic compression by an acutely inflamed acalculous gallbladder abutting the common hepatic duct. This case suggests that extrinsic compression and inflammation in the absence of stones can result in complete obstruction of extrahepatic biliary flow.