A bleeding sword: Scimitar syndrome in a child with hematemesis due to esophageal varices - a case report.

Abstract

Introduction: Scimitar syndrome, or congenital venolobar syndrome, is a rare congenital heart condition that typically involves partial anomalous pulmonary venous drainage (PAPVD) of the right lung, right lung hypoplasia, and dextroposition of the heart. The incidence is estimated to be between 1 and 3 cases per 100 000 live births. Most patients present with cardiopulmonary symptoms, though rare cases may involve gastrointestinal (GI) complications. We report a unique case of Scimitar syndrome in a 10-year-old boy presenting with hematemesis due to esophageal varices.

Case presentation: A 10-year-old boy presented with massive hematemesis, losing approximately 1 liter of fresh blood. After stabilization, an upper GI endoscopy revealed grade IV esophageal varices, which were ligated successfully. Abdominal ultrasound showed mild portal hypertension, and a unique vessel, identified as the Scimitar vein, was found draining into the left portal vein. Subsequent imaging confirmed right lung hypoplasia, dextroposition of the heart, and PAPVD, leading to the diagnosis of Scimitar syndrome. The patient was transferred abroad for specialized care due to limited medical facilities in Sudan.

Clinical discussion: Scimitar syndrome is often associated with severe symptoms in children, such as respiratory failure and pulmonary hypertension. Upper GI bleeding in this syndrome is rare, likely resulting from portal hypertension caused by the anomalous drainage of the Scimitar vein into the left portal vein.

Conclusion: This case underscores the importance of recognizing the diverse complications of Scimitar syndrome, including its rare association with upper GI bleeding. Timely diagnosis and intervention, especially in resource-limited settings, are critical to managing complex congenital conditions.