Budd-Chiari syndrome is one of the post-hepatic causes of portal hypertension and a potential obstruction causes liver fibrosis. In pregnancy, obstruction of hepatic veins could occur due to stenosis or thrombosis. Variceal bleeding is the most fatal complication in pregnancy with co-existing Budd-Chiari syndrome, with 29.4% incidence of abortion and 33.3% perinatal mortality. The aim of this case report was to present the management of non-cirrhotic variceal bleeding in pregnant women with Budd-Chiari syndrome in the early second trimester. We report a pregnant female at 13-14 weeks gestation presented to the hospital with profuse hematemesis. Doppler ultrasonography (USG) was utilized to confirm the diagnosis of Budd-Chiari syndrome-hepatic vein occlusion type in pregnancy. Abdominal USG revealed hepatomegaly with hepatic veins dilation, while endoscopy showed grade IV esophageal varices and grade IV gastric varices. Laboratory results indicated disseminated intravascular coagulation due to hemorrhage. The patient was given strict fluid resuscitation and three packed red cells transfusion to stabilize the hemodynamic. Bleeding was successfully managed by intravenous octreotide, tranexamic acid, and vitamin K. The case highlights that the management of non-cirrhotic variceal bleeding in pregnancy with Budd-Chiari syndrome requires a multidisciplinary approach and regular fetal monitoring to ensure optimal outcomes.
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http://dx.doi.org/10.52225/narra.v4i1.245 | DOI Listing |
Eur J Case Rep Intern Med
December 2024
Department of Internal Medicine, Ospedale Regionale di Lugano EOC, Lugano, Switzerland.
Unlabelled: This article describes a case of a 26-year-old female with a history of Evan's syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus.
View Article and Find Full Text PDFBMJ Case Rep
January 2025
Radiodiagnosis and Interventional Radiology, AIIMS Bhubaneswar, Bhubaneswar, Odisha, India.
Budd-Chiari syndrome with obstruction in the inferior vena cava causes increased venous pressure in the azygous-hemiazygous system and paravertebral venous plexus, which is transmitted to the epidural venous plexus, devoid of the valves. It causes epidural venous plexus engorgement and venous congestion and may present rarely with low back pain or radiating pain. However, patients developing lower limb weakness as a complication of Budd-Chiari syndrome is an infrequent and severe presentation.
View Article and Find Full Text PDFActa Gastroenterol Belg
January 2025
Department of Radiology, Antwerp University Hospital, Antwerp, Belgium.
Radiol Case Rep
February 2025
Department of Internal Medicine, An Najah National University Hospital, Nablus, Palestine.
Behçet's Disease (BD) is a multisystem inflammatory disorder that can lead to severe vascular complications, including Budd-Chiari Syndrome (BCS), a rare but life-threatening condition characterized by hepatic vein obstruction. The co-occurrence of BD and inherited thrombophilia, such as Factor V Leiden mutation, significantly increases the risk of thrombosis, complicating the clinical management of affected individuals. In this case, a 16-year-old female initially presented with nonspecific symptoms of generalized fatigue and bone pain, which later progressed to abdominal distension and significant hepatosplenomegaly.
View Article and Find Full Text PDFEur J Haematol
December 2024
Department of Medicine, Division of Hematology/Oncology, University of South Florida, Tampa, Florida, USA.
Aims: Budd-Chiari syndrome (BCS) is managed by interventions aimed at relieving hepatic venous obstruction and anticoagulation. Despite robust data supporting the tolerability and efficacy of direct oral anticoagulants (DOACs) in patients with other venous thromboembolism, its utility in BCS is not well documented. This study aims to evaluate the efficacy and tolerability of DOACs in Primary BCS from the available literature.
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