Huma Arshad Cheema, Nadia Waheed, Anjum Saeed, Muhammad Arshad Alvi, Muhammad Nadeem Anjum, Zafar Fayyaz, Syeda Sara Batool Hamdani.
The Budd Chiari Syndrome, Etiology Clinical Presentation Radiological Finding and Outcome in Children Under 16 Years of Age.
Pak Paed J Nov ;42(4):242-6.

Background and Aims: Budd Chiari syndrome (BCS) is hepatic venous out flow obstruction and a rare cause of ascites in children. There is scarcity of data in literature regarding BCS in children therefore it is easily missed. Objective of our research is to study presentation, clinical manifestation diagnosis and outcome of BCS in children. Methods: Children under 16 years of age presenting with ascites underwent color Doppler ultra sonography. BCS was defined as occlusion/obliteration of one or more hepatic veins and/or suprahepatic IVC. Age sex, caste, and duration of symptoms, socioeconomic status, height, weight, z-score, and stigmata of chronic liver disease, dilated veins over abdomen, visceromegaly and ascites were recorded. Ascitic fluid analysis and gadolinium enhanced multiphasic MR scan were performed. Liver biopsy was performed only in patients with normal coagulation profile. All children screened for thrombophilia disorders, paroxysmal hemoglobinuria and myeloproliferative disorder. Results: 19 out of 54 patients fulfilled inclusion criteria. Mean age of presentation in BCS was 9.5±2.58 years with male to female ratio 1:1.8. 100% (19) children presented with distended veins over abdomen and 89.5 % (17) with gross ascites. 63% (12) children had chronic BCS 31.5% (6) sub-acute BCS, procoagulant disorder was found in 63%(12) patients, most common of them was AT-III deficiency 26%(5) followed by protein C 21% (4) protein S 11. One patient had fulminant BCS and three had chronic liver disease needed orthotropic liver transplant. Six of them presented with sub-acute form having pro-coagulant disorder were started on anti-coagulant therapy and doing well. Rests of them are on liver supportive treatment. Conclusions: BCS is a rare but important cause of ascites in children. Mostly children presents with distended abdominal veins and ascites. Procoagulant disorder is the common etiology and common radiological findings are absent flow in hepatic veins, caudate lobe hypertrophy and intrahepatic collaterals.

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