Rare Association of Budd-Chiari Syndrome With Celiac Disease: A Case Report

Budd-Chiari associated with celiac disease is a rare phenomenon in the medical literature with annual incidence of less than five per million. The majority of the cases are reported from the North African region. Our patient presented in the out-patient department with symptoms of progressive abdominal distension, diffuse abdominal pain and shortness of breath for one year. She was a known case of celiac disease for the last three years. The clinical examination revealed ascites, jaundice, decreased air entry in basal segments bilaterally, and multiple hemangiomas all over the body. Haematological and biochemical investigations, including levels of pro-thrombotic factors and homocysteine level, turned out to be normal. However, computed tomography (CT) revealed hepatic vein obstruction. Hence, a diagnosis of Budd-Chiari syndrome was confirmed. The patient was managed with anticoagulants, diuretics and gluten-free diet. Within a month, the patient showed marked improvement with a significant reduction in ascites. To the best of our knowledge, this rare association is the first case to be reported from Pakistan and third from the region of South Asia.