musaab al ramsi, MD
Pediatric Intensivist- Division Chief
Sheikh Khalifa Medical City, United Arab Emirates
Disclosure information not submitted.
Alyaa Al Ali, MD
Pediatric intensivist
Sheikh Khalifa Medical City
Abu Dhabi, United Arab Emirates
Disclosure information not submitted.
Title: Acute hemorrhagic venous infarct in a patient with sickle cell disease: to anticoagulate or not?
Case Report Body:
Introduction: Cerebrovascular accidents are unusual but devastating complications of Sickle cell disease (SCD). Notably, dural venous sinus thrombosis (DVST) is rarely reported in SCD and pose a therapeutic Dilemmas regarding anticoagulation. Herein, we describe a challenging case of sickle thalassemia admitted with hemorrhagic infract secondary to anterior superior sagittal sinus thrombosis.
Description: A 16-year-old male with sickle thalassemia was brought to ED with history of Left-hand weakness that progressed within 24 hours to left hemiplegia, a severe headache, vomiting and an altered level of consciousness. High Performance Liquid Chromatography showed HbS of 80.5% and HbF of only 9.8%. Computed tomography (CT) of the Brain showed focal hyper density in the right frontal lobe with hyperdense anterior superior sagittal venous sinus, suggestive of acute hemorrhagic venous infarct. CT cerebral venography confirmed superior sagittal venous sinus thrombosis. The patient deteriorated rapidly within a few hours of admission while work up was still in process. He developed a new onset seizure, facial palsy, and worsening weakness. Emergent re-imaging demonstrated new hemorrhagic infarcts. Despite the risks of fatal bleeding, anticoagulation with heparin was started to treat the acute DVST. Neuroprotective measures and exchange transfusion were performed. After 3 days, he was marked improved and was discharged home with significant neurological improvement. His Left hemiplegia improved gradually and fully recovered after 8 weeks.
Discussion: Timely anticoagulation is theoretically crucial for patients diagnosed with DVST to decrease thrombus propagation and prevent further venous infarction. However, presence of a hemorrhage creates a significant reluctance to immediately initiate anticoagulation and might unnecessarily delay this life saving, yet risky treatment. This case highlights that anticoagulation remains the optimal strategy for preventing both neurological morbidity and mortality even in the setting of active bleeding. Further research, or at least a consensus opinion of experts, can be valuable to establish a practical pathway when clinicians are emergently faced with such a rare but life-threatening condition.