Published Paper Details:

Budd�Chiari syndrome (BCS) encompasses a varied group of disorders with common pathology characterized by hepatic venous outflow obstruction which can occur anywhere at the level of the hepatic venules, the large hepatic veins, the inferior vena cava and the right atrium. The term �hepatic venous outflow tract obstruction� is usually applied irrespective of the level f obstruction or the mechanism of obstruction. With the advances in the speciality of radio diagnosis the diagnosis of budd chiari syndrome is easily made. However because of its varied unusual presentations, sometimes it poses a diagnostic challenge for the treating physician. We present a case report of a woman who presented with trivial symptoms and pancytopenia which on further evaluation later turned out to be having chronic budd chiari syndrome. CASE PRESENTATION: A 42-year-old female presented to our OPD with history of loose stools and easy fatigability for the last 10 days. On evaluation she had mild ascites and pancytopenia. Her liver function tests were within normal range. She had low serum vitamin B12 and iron deficiency anemia. Her USG hepatobiliary system revealed mild ascites. Computed tomography of her abdomen showed non opacified hepatic veins with marked luminal narrowing of suprahepatic veins with prominent azygous and hemiazygous veins with caudate lobe hypertrophy. Further upper gastrointestinal endoscopy revealed presence of varices which were managed with EVL bands. She was diagnosed as a case of chronic budd chiari syndrome. On thorough history taking it there was history of recurrent abortions for which no prior work up was done. Further investigation revealed deficiency of S, raised homocysteine levels with normal JAK2 mutation and APLA workup. She was then started on anticoagulation and invasive procedures were deferred (stent v TIPS) in view of good network of intrahepatic collaterals. DISCUSSION: Budd chiari syndrome have varied presentations ranging from asymptomatic condition detected incidentally (as in our case) to fulminant BCS classical triad of abdominal pain, hepatomegaly, ascites and jaundice. Previously it was the membranous occlusion of IVC that happened to be the main mechanism. However now it is seen that the isolated hepatic vein occlusion is more commonly seen in our country. Also hypercoagulable state being the etiological factor is now more commonly seen than the membranous occlusion of IVC. Both these facts are confirmed by the findings in our case. CONCLUSION: since budd chiari syndrome may present as asymptomatic condition, higher degree of suspicion should be kept if any of the signs of liver failure are note on clinical examination. Similarly a thorough and meticulous history is warranted so as to rule out the genetic and acquired causes. Abbreviations: APLA antiphosholipid antibody;BCS, Budd�Chiari syndrome; CT, computed tomography; EVL,endoscopic variceal ligation;IVC, inferior vena cava;MRI, LMWH, low molecular weight heparin magnetic resonance imaging; OPD, out patient department;TIPS, transjugular intrahepatic portosystemic shunt; UFH, unfractionated heparin;UGIE, upper gastrointestinal endoscopy;USG, ultrasonography; VKA, vitamin K antagonist