Gastroenterology Education and CPD for trainees and specialists

Explanation :IVC webs are leading cause in the developing countries. Membranous obstruction of vena cava (MOVC) can account for up to 40% in Asia. Associated Portal vein thrombosis and IVC thrombosis are seen in >30% of patients.Ultrasound Doppler is the investigation of choice. Typical findings are hepatomegaly, inability to visualise the hepatic veins to connect to IVC, absence of waveforms in hepatic veins, hypertrophy of caudate lobe and comma shaped intrahepatic collateral vessels . Caudate lobe is drained by dorsal hepatic veins directly into IVC rather than through the hepatic veins. So in 50% of patients there is compensatory increase in the size of caudate lobe. In acute BCS - centrizonal ( centrilobular or zone 3) congestion with dilatation of perivenular sinusoids, haemorrhage and hepatocyte necrosis ( most prominent in zone 3) are the typical features.

Explanation :Chronic BCS is the commonest form of presentation in all large series. Collateral circulation can be found in most cases sometime with impressive abdominal veins draining bloods from the inferior extremities .Also caudate lobe hypertrophy is seen as venous drainage is different. TIPS is technically challenging as access to liver parenchyma may be blocked by the clot. Most common treatment for patients with chronic BCS with adequate hepatic reserve is surgical shunt. Only acceptable shunts are those that decompresses the portal circulation ( eg side to side shunts). Side to side splenorenal, mesocaval and portocaval shunts are commonly done- the type of shunt is being dictated by the extent of the thrombosis and degree of obstruction of the IVC by the hypertrophied caudate lobe. Myeloproliferative disease is the commonest cause (between 20-50%) in West. Ascites is seen in 84% . The classic triad is abdominal pain, hepatomegaly and ascites (>62%)

Explanation :It is characterised by occlusion of the terminal hepatic venules and hepatic sinusoids. It can be regarded as a subset of BCS but the causes, prognosis and pathophysiologic characteristics are sufficently distinct to classify VOD as a distinct disease entity. The primary lesion of VOD is endothelial toxicity with progressive occlusion of the terminal hepatic nenules and sinusoids. Histologically sinusoidal dilatation and oedema/necrosis of zone 3 hepatocytes are typical which can mimic BCS or congestive hepatopathy. Although no specific mechanism have been identified risk factors recognized are chemotherapy ( cyclophosphamide, busulphan), stem cell transplantation, hepatic irradiation and ingestion of pyrrolizidine alkaloids containing plants ( VOD was first recognized in Jamaica and epidemics related to these have been described in India, Afganisthan and middle east).

Explanation :Insidious onset of portal hypertension (PH) is characteristic of VOD. It occurs within 2 weeks of transplatation reaching a peak at day 17. The other differentials of abnormal LFT in this situation are Graft -versus-host disease (GVHD), drug toxicities, bacteremia and post transfusion hepatitis. GVHD rarely occurs before day 15 and the other two rarely cause hepatomegaly and ascites. Outcome of transplant related VOD varies from acute liver failure to complete recovery in 3 weeks.A wedge hepatic venous pressure gradient or HVPG of >10mm Hg is quite specific for VOD. Primary prevention has been attempted by adjusting doses of busalphan containing chemotherapy, using prophylactic heparin or ursodeoxycholic acid.In establised cases different hamostasis influencing agents ( such as rTPA,urokinase, prostaglandins etc) has been used in various combinations.

Gastroenterology training and education

MCQ On Budd Chiari Syndrome

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