P0445 - A Case Report of Dural Venous Thrombus Causing Hemorrhagic Stroke in Sickle Cell Disease

Background/

Aims: Pediatric stroke is a rare and serious occurrence with an estimated incidence of 1.2-13 cases per 100,000 annually. There is a high level of morbidity and mortality; survivors often suffer from long term neurological sequela. The pathophysiology of sickle cell disease (SCD) creates a prothrombotic state, thus SCD patients constitute a unique population of pediatric patients at high risk for cerebral vascular accidents. It has been estimated that by the age of 20, approximately 11% of SCD patients will have a clinically significant infarction. Most SCD infarctions occur in the large arteries including the middle cerebral and carotid arteries; venous infarcts are rarer. We present the case of a patient with a diagnosis of dural venous thrombosis in the left transverse sinus causing a hemorrhagic stroke, including a literature review to discuss acute management strategies.

Case Description: 17-year-old female found unresponsive at home. She was brought into the ED with bradycardia, hypoxia, normotension, and a GCS of 8 requiring bag mask resuscitation. Her mother disclosed a patient history of SCD and a headache of one-week duration prior to presentation. Lab studies revealed a WBC count of 6.8, a hemoglobin of 8.1, normal electrolytes, a hemoglobin S level of 18%, and a glucose level of 160. Head CT revealed a hemorrhagic stroke with midline shift (image 1). Follow-up CT venogram revealed poor enhancement of the left transverse sinus (image 2). She was taken to the OR for an emergent decompressive craniectomy. Post-operatively, management included strict ICP control, blood transfusion, and exchange transfusion. Anti-coagulation therapy was discussed but deemed contraindicated given the presence of hemorrhagic stroke. Supportive care was provided resulting in neurologic recovery.

Discussion: Dural venous thrombus is a rare but serious cause of infarction in SCD. A mechanism is not entirely understood but one possibility includes blood stasis in the venous circulation allowing increased surface contact between the abnormal RBCs.
Mainstays of treatment for SCD stroke patients include emergent decompressive surgery and maintenance of a normal ICP. Hypertonic saline is standard of care therapy to treat elevated ICP. Simple transfusion may be beneficial for low baseline hemoglobin. Exchange transfusion is indicated if the hemoglobin S level is > 30%. Blood transfusion can reduce the long-term risk of future stroke.
Anticoagulation should be considered in patients with SCD and dural venous thrombus. Multiple case reports reveal positive outcome with heparin usage. However, one case report describes death following anti-coagulation utilization4. Our patient achieved a full recovery without anticoagulation therapy.