Superior sagittal sinus thrombosis in hemoglobin S–D disease with acute pyogenic meningitis: a rare case report

Abstract

Cerebral venous sinus thrombosis (CVST) involving the superior sagittal sinus with associated cerebral infarction is an exceptionally rare neurological complication of hemoglobin S–D disease. The heterogeneity of clinical manifestations, overlapping infective and hematologic features, and dependence on advanced neuroimaging frequently delay diagnosis. Early recognition and targeted therapy are critical to prevent irreversible neurological injury. We report a 19-year-old woman with hemoglobin S–D disease who presented with acute-onset headache, altered sensorium, and behavioral changes. Contrast-enhanced computed tomography and magnetic resonance imaging of the brain, complemented by magnetic resonance venography and angiography, demonstrated thrombosis of the superior sagittal sinus with bilateral frontal lobe venous infarctions. Evaluation for hypercoagulable states was limited because comprehensive thrombophilia testing was unavailable at our institution due to resource constraints. Specifically, lupus anticoagulant, anticardiolipin antibodies, β2-glycoprotein I antibodies, protein C, protein S, antithrombin III levels, factor V Leiden mutation, and prothrombin gene mutation analysis. Therefore, an underlying thrombophilic predisposition cannot be definitively excluded. Nevertheless, hemoglobin S–D disease is recognized as a prothrombotic condition through mechanisms including hemolysis, endothelial dysfunction, increased platelet activation, and hyperviscosity, which may have contributed to thrombosis in the present case. Additional potential contributing factors included dehydration and acute bacterial/pyogenic meningitis. The patient was treated with therapeutic anticoagulation using rivaroxaban, in addition to intravenous hydration and empiric antimicrobial therapy, resulting in marked clinical and radiological improvement. Patients with hemoglobinopathies, including hemoglobin S–D disease, may be predisposed to thrombotic and neurological complications. Although cerebral venous sinus thrombosis has a multifactorial etiology, the underlying hemoglobinopathy and concurrent infection may have acted as contributing factors in this case. A high index of suspicion is warranted in patients with hemoglobinopathy presenting with persistent headache, altered mental status, or focal neurological deficits. Early neuroimaging and prompt initiation of anticoagulation, supported by optimization of hydration and management of concurrent infection, are essential to reduce morbidity and improve neurological outcomes.