A rare pediatric case: Budd-Chiari Syndrome and upper gastrointestinal bleeding in a 5-year-old boy.

Abstract

Introduction and importance: Budd-Chiari Syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction, often presenting with ascites, hepatomegaly, and abdominal pain. The diagnosis and management of BCS, especially in resource-limited settings, pose significant challenges that can lead to poor patient outcomes.

Case presentation: This case report describes a previously healthy individual presenting with progressive abdominal distention, hematemesis, and right upper quadrant pain. The patient had a history of trauma and underwent subsequent percutaneous drainage for ascites. Imaging studies revealed non-opacified hepatic veins, splenomegaly, ascites, and a "nutmeg liver" pattern, confirming the diagnosis of BCS. The patient received blood transfusions, diuretics, and nutritional support. A Transjugular Intrahepatic Portosystemic Shunt (TIPS) procedure was planned but ultimately hindered by financial constraints.

Clinical discussion: This case highlights the challenges of managing BCS in resource-limited settings, particularly regarding access to specialized treatments like TIPS. It emphasizes the need for early diagnosis, multidisciplinary care, and the development of cost-effective treatment strategies to improve patient outcomes.

Conclusion: The management of Budd-Chiari Syndrome is complex, particularly in settings with limited resources. This case underscores the importance of timely intervention and the need for innovative approaches to healthcare delivery that can accommodate financial barriers while ensuring patient safety and care quality.