We report a case of a 23-year-old male, known case of Down’s syndrome, who presented to our Emergency Department with a complaint of severe headache for three days. It was accompanied by nausea, vomiting, and confusion. There was no history of trauma, seizures, loss of consciousness, change in vision, or relevant signs of virus infection. He was not on any regular medications. His medical background included surgical closure of ventricular septal defects in his childhood. There was no past medical or family history of coagulopathies.\nOn examination, his Glasgow coma score was 14, with normal vital signs. On neurological examination, the patient was confused, with dense right-sided hemiparesis. Liver function test, prothrombin time, partial thromboplastin time, and international normalized ratio were all within normal range. The brain CT scan revealed left-sided subacute SDH with a 12 mm midline shift and uncal herniation ().\nThe patient underwent emergent left-sided craniotomy and SDH evacuation. The blood evacuated was a mixture of dark stained blood and clots. Post-surgery, the patient’s consciousness level returned to normal, and he regained full power in all limbs. His post-operative CT scan showed optimal evacuation of the left-sided hematoma. There was also a new finding of a small right-sided SDH in the parietal region with no significant mass effect (). The CT scans review showed hyperdense areas in the transverse and sigmoid sinus; this raised suspicion of thrombosis. Magnetic resonance venography (MRV) confirmed the diagnosis of CVST in the right transverse and sigmoid sinuses with loss of flow signal ().\nClinically, the patient made an excellent post-operative recovery. After the diagnosis of CVST, we obtained neurology opinion and considered it risky to commence therapeutic anticoagulation in the early post-operative phase. He was started on a prophylactic dose of low molecular heparin. The patient was discharged home one week after surgery. Subsequently, he was readmitted electively two weeks later and was commenced on Apixaban. He was followed up regularly in outpatients and was asymptomatic.\nThe patient re-presented three months later complaining of a headache and recurrent vomiting, with normal consciousness levels. His repeat brain CT showed recollection of hematoma in the left subdural space (). An updated MRV revealed an interval shrinkage of the thrombus in the right sigmoid venous sinus; however, the reduced flow was seen through the sinus. The medical decision was to stop the anticoagulation, and the patient underwent reopening of the left-sided craniotomy and evacuation of the recurrent SDH. He recovered well after surgery, and we elected not to restart anticoagulation and follow-him clinically.\nThe patient remains clinically well. Interval MRI and MRV scans at six months show complete resolution of the SDH, with residual partial thrombosis of the right sigmoid sinus; however, the sinus is patent, and the venous flow is restored ().
A 68-year-old woman with polycythemia vera experienced a global, nonpositional headache for one day. She subsequently became comatose. On emergent evaluation she exhibited decorticate posturing. Her Glasgow coma score was 6. There was no preceding history of trauma, infection, or dehydration. She was not on anticoagulants or antiplatelet agents. Her polycythemia vera had been managed with therapeutic phlebotomy and hydroxyurea (500 mg daily). She had no prior history of thromboembolic events. Noncontrasted head CT showed extensive left 20 mm acute SDH with 16 mm of midline shift []. She underwent emergent craniotomy and hematoma evacuation. On careful inspection of the exposed brain, a prominent and engorged cortical vein observed. No other vascular abnormalities or potential bleeding sources were identified. CT venography and MR venography detected partial superior sagittal sinus thrombosis, which leads to the empty delta sign (the intraluminal thrombus prevents central filling of the sinus; ). She was managed conservatively with hydroxyurea, hydration, and aspirin. Laboratory analyses were notable for normal coagulation panel, normal platelet function assays, hematocrit of 45.9, negative thrombophilia evaluation (protein S, protein C, antithrombin III, anticardiolipin antibody, homocysteine, lupus anticoagulant, beta-2 glycoprotein antibodies, Factor V Leiden mutation, prothrombin gene mutation). Analysis of DNA extracted from her blood revealed the presence of the V617F mutation within the JAK2 gene. Her 3-month outcome was poor (modified Rankin score 4).