A young female who is a known case of myeloproliferative disorder presents with a 3 day history of progressive abdominal discomfort and ascites. Which of the following is the possible diagnosis

Ans. is 'a' i.e., Budd Chiari Syndrome * Young female with myeloproliferative disorder - points towards a procoagulant state* Presentation of acute onset abdominal discomfort and ascites mostly points to involvement of hepatic vein - probably thrombosis of hepatic vein due to the procoagulant state ->> so the most probable diagnosis is - Budd Chiari Syndrome.Budd Chiari syndrome* This is a condition principally affecting young females, in which the venous drainage of the liver is occluded by hepatic venous thrombosis or obstruction from a venous web.* Asa result of venous outflow obstruction, the liver becomes acutely congested, with the development of impaired liver function and, subsequently, portal hypertension, ascites and oesophagealvarices.* In an acute thrombosis, the patient may rapidly progress to fulmi- nant liver failure but, in the majority of cases, abdominal discomfort and ascites are the main presenting features.* If chronic, the liver progresses to established cirrhosis.* The cause of the venous thrombosis needs to be established, and an underlying myeloproliferative disorder or pro-coagulant state is commonly found, such as anti-thrombin 3, protein C or protein S deficiency.* The diagnosis is commonly suspected in a patient presenting with ascites, in whom a CT scan shows a large congested liver (early stage) or a small cirrhotic liver in which there is gross enlargement of segment I (the caudate lobe). This feature results from preservation and hypertrophy of the segment with direct venous drainage to the I VC in the face of atrophy of the rest of the liver due to venous obstruction.* IVC compression or occlusion from the segment I hypertrophy is also a common feature, as is thrombosis of the portal vein.* Confirmation of the suspected diagnosis is by hepatic venography via a transjugular approach, which demonstrates occlusion of the hepatic veins and may allow a transjugular biopsy.