Presentation on theme: "Raika Jamali MD Digestive Disease Research Center"— Presentation transcript:
1Management conference Middle age man with nephrotic syndrome, ascitis and edema Raika Jamali MDDigestive Disease Research CenterTehran University of Medical Sciences
2A 49 years old man with progressive bilateral pedal edema and ascitis from 1 month ago. History of DM for 4 years.Three months ago during the evaluation for excessive proteinuria inappropriate for diabetic nephropathy ,prolongation of PT was detected before kidney biopsy.Viral markers requested and was referred for liver function evaluation.
3EXAM Vital signs were stable. No fever. Mild anemia. Ichterus in sclera.Parallel collaterals in chest and upper abdomen which filled upward.Tense ascitis. liver span 14 cm.Moderate splenomegaly .No signs of chronic liver disease.Bilateral pedal edema.
24mottled appearance to the underperfused liver with collapsed portal veins, ascites (small arrows)extensive retroperitoneal varices (large arrow).enlarged caudate lobe of the liver (large arrowhead)the collapsed small IVC (small arrowhead).
30Follow UpThe patient was treated with diuretic and concomitant albumin.Several abdominal paracentesis were performed.Heparin started and switched to warfarin.Proteinuria decreased during F/U.Ascitis and edema is partially controlled with diuretic.Hypercoagulability states were checked again which showed normal results.
31Budd-Chiari syndrome more common in women third or fourth decade most common symptoms is ascites (84%) and hepatomegaly (76%)obstruction was in the hepatic veins (62%) inferior vena cava (7%)portal vein thrombosis (14%)myeloproliferative disorder was present in 23% (polycythemia vera).
32Major causes of the Budd-Chiari syndrome Myeloproliferative diseasesMalignancy (Hepatocellular carcinoma)Infections and benign lesions of the liverOral contraceptivesPregnancyHypercoagulableBehcet's diseaseMembranous webs of IVCIdiopathic
33Acute (20%) :(2% with fulminant hepatic failure)Subacute (40%):(having signs or symptoms for < 6 months and no evidence of cirrhosis)Chronic (40%):(having signs or symptoms for > 6 months with evidence of cirrhosis)
34Acute most commonly in women (during pregnancy ) pain and hepatomegaly Jaundice and ascites develop rapidlyLiver function can deteriorate quickly, leading to hepatic encephalopathyDDx: ischemic, viral, malignant/infiltrative, and toxic hepatitis
35Subacute and chronic disease clinical manifestations depend upon the extent of occlusion, and the recruitment of collateral circulation.Chronic occlusion of the hepatic veins may be associated with hypertrophy of the caudate lobe.This cause compression of the intrahepatic portion of the IVC, leading to lower extremity edema
37cirrhosis may develop in the chronically congested liver, resulting in portal hypertension encephalopathy is infrequentHepatopulmonary syndrome (28%)liver biochemical tests are usually mildly abnormal
38DIAGNOSISChronic or subacute Budd-Chiari syndrome should be considered in unexplained liver dysfunction, particularly if ascites is a principal feature, or if risk factors for Budd-Chiari syndrome exist.Clinical:Splenomegaly, venous collateralsEdema of the lower extremities suggests occlusion of the inferior vena cavaSigns of right-sided congestive heart failure (such as jugular venous distension)
39Acute : hepatomegaly, RUQ pain, ascites Accuracy of noninvasive imaging modalities depends upon: duration of disease, location of the clot.Portal vein thrombosis limits therapeutic options and has a poor prognosis
40Doppler ultrasonography Screening testhepatomegaly,splenomegaly,ascites,intraabdominal collaterals,caudate lobe hypertrophy,atrophy of other hepatic lobes,compression of IVCThickening, irregularity, stenosis, or dilation of the walls of the hepatic veinsAbnormal flow in the major hepatic veins or IVC
41CT scan Delayed or absent filling of the three major hepatic veins Patchy flea-bitten appearance of the liverRapid clearance of dye from the caudate lobeNarrowing and/or lack of opacification of the inferior vena cava
42Magnetic resonance imaging typical distorted "comma-shaped" intrahepatic collateralsunremarkable ultrasound examination but in whom the suspicion is highVenographyGold standard for diagnosisplan therapeutic interventions .Determine pressure gradient above and below the entrance of the hepatic veins into the inferior vena cava
43Accurately define the extent or characteristics of the hepatic venous flow Compression of the intrahepatic IVC, leads to sluggish flow in hepatic veins. As a result, the hepatic veins can be undetectable during ultrasound Doppler studies, although they may be patent and amenable to therapy
44Liver biopsy Can be diagnostic in the acute or subacute form Features include centrizonal congestion, necrosis, and hemorrhageCirrhosis may be present in the chronic formDetermine prognosis and guide therapyCirrhotics are less likely to benefit from revascularization procedures
45thrombotic process in Budd-Chiari syndrome may not involve all the hepatic veins. Thus, the distribution of the typical pathologic findings may be focal or patchy. As a result, some patients require biopsy of both the right and the left lobes of the liver.laparoscopic approach may be better suitedPerfom Bx when there is confusion regarding the diagnosis and plan treatment accordingly
46TREATMENT Prevent the propagation of the clot Decompress the congested liverPrevent complications (malnutrition, portal hypertension) _________________________________Medical treatment (supportive care, anticoagulation, thrombolysis),Radiologic procedures (angioplasty, TIPS,)Surgical intervention (shunting procedures , transplantation).
47Medical therapy Diuretics and a low sodium diet large-volume paracentesesImprove nutritional statusUnderlying cause should be investigatedMyeloproliferative disorder may benefit from treatment with aspirin and hydroxyurea
48Anticoagulation alone is unlikely to lead to sufficient recanalization of occluded vessels to avoid the progression of liver disease.A trend for a benefit of anticoagulation on survival in less severe disease.Medical therapy :1) Chronic or subacute Budd-Chiari syndrome with well compensated liver disease at the time of presentation.2) When other types of therapy are not feasible
49Risk of anticoagulation should also be considered, especially in patients who present with bleeding complicationsPatients receiving only medical therapy should be monitored closely for disease progression (liver biopsies annually )and portal hypertension complications (looking for varices)
50Thrombolytic therapyIn acute form which blood clots are younger than three to four weeksDo not use thrombolytic agents in:patients who have extensive clot involving the IVCor a clot of unknown age.
52Surgical therapy Restore hepatic venous drainage using shunt surgery Because of the availability of TIPS, few vascular surgeons routinely perform shunt surgery.Underlying cause of the thrombotic diathesis should be identified and treated prior to considering shunt surgery.Unlikely to be beneficial in patients who have cirrhosis, Such patients are best managed with liver transplantation.
53survival following shunt surgery depends upon the extent of liver damage prior to surgery, and the continued patency of the shuntMaintenance of shunt patency often requires anticoagulationdeterioration in patients following shunt surgery should be investigated by angiography to determine whether the shunt has thrombosed, which may be corrected by angioplasty.
54Liver transplantation who are not candidates for radiologic or surgical decompressionor who have decompensated cirrhosisprotein S, protein C, or antithrombin III deficiency may also be cured of their clotting tendency by liver transplantation,Survival following OLT depends upon the underlying cause of the Budd-Chiari syndrome and the patients condition at the time of the transplant
55Budd-Chiari syndrome during nephrotic relapse in a patient with resistance to activated protein C clotting inhibitorAm J Kidney Dis.
56It has long been known that patients with nephrotic syndrome have a hypercoagulable state, which explains the association between nephrotic syndrome, renal vein thrombosis, and thromboembolism.However, the Budd-Chiari syndrome has never been reported in nephrotic patients.This is the first report of such an association that, most likely, depended on a primary resistance to activated protein C
57Budd-Chiari syndrome and inferior vena cava thrombosis in a nephrotic child. Pediatr Nephrol.
58We observed Budd-Chiari syndrome in a boy aged 2 years 6 months with nephrotic syndrome due to hepatic vein and inferior vena cava thrombosis, confirmed by Doppler imaging.Normal values of the routine hemostatic parameters proved that they are of little predictive value for the thrombotic state.
59Immediate heparin infusion was initiated Immediate heparin infusion was initiated. High doses of heparin up to 59 IU/kg per hour were required for efficient anticoagulation.A remission of the nephrotic syndrome was achieved with vincristine.Oral anticoagulation with a vitamin K antagonist was continued for 6 months.Doppler imaging then indicated full re-establishment of the blood flow through the affected vessels.
60The favorable outcome was due to the immediate heparin infusion and prompt remission of the nephrotic syndrome.Doppler imaging was an important tool for non-invasive diagnosis and follow-up.
61Thromboembolic complications in children with nephrotic syndrome in Bulgaria (1974-1996). Pediatr Nephrol.
62Over a period of 22 years, 447 children with nephrotic syndrome (NS) have been retrospectively studied for clinically apparent thromboembolic complications (TEC).The incidence of TEC is 2% (9/447).TEC were predominantly venous (81% venous vs. 19% arterial).The most commonly affected vessels were deep leg veins, IVC, SVC, mesenteric artery, and hepatic veins (Budd-Chiari syndrome).
63Etiology based prevalence of Budd-Chiari syndrome in eastern India J Assoc Physicians India.
64Idiopathic membranous obstruction and stricture of IVC are the commonest cause of BCS in the eastern part of India.Hepatocellular carcinoma is also a common cause, presenting in the fulminant form.Ultrasonography may be a helpful screening test for BCS,IVC and hepatic vein catheterisation is essential for a complete work up of these patients.
65Budd-Chiari syndrome--a case report Nepal Med Coll J.
66A 21year old male presented with abdominal pain for 2 months and abdominal distension and swelling of lower limbs for 1 month.US showed coarse echotexture of liver and intraluminal filling defect of IVCConfirmation of diagnosis was done by inferior venacavography.The patient had nephrotic syndrome as the risk factor for thrombosis.The patient underwent portocaval shunt with significant symptomatic relief.