Case Descriptions
Case 1
A 5-year-old female with mild hemophilia A, with baseline FVIII level of 28%, presented to the emergency department (ED) twelve hours following a fall of four feet, with trauma to the back of her head8. She developed altered mentation, severe occipital headache, and multiple episodes of vomiting prior to presentation. A computerized tomography (CT) scan without contrast of the head and CT venography (CTV) were obtained, showing extra-axial hemorrhage of the left occipital region and CSVT (Figure 1). Due to worsening headache, and increase in ICH, the patient was started on scheduled intravenous short-acting recombinant FVIII infusions. She was discharged eleven days after admission, and completed total of 14 days of factor therapy. Post discharge, after ICH stabilized on repeat imaging, the patient was started on subcutaneous enoxaparin therapy with concurrent prophylaxis with long acting FVIII. Magnetic resonance imaging/magnetic resonance venography (MRI/MRV) of the head following 6 weeks of anticoagulation therapy showed complete resolution of the CSVT and ICH.
Case 2
A 2-year-old female with no known PMH presented to the ED following head injury due to an accidental fall 8. Initial head CT scan was normal; the patient developed somnolence and recurrent vomiting over the next day, prompting a CTV which showed left sigmoid sinus and internal jugular (IJ) vein thrombosis. The patient was transferred to the pediatric intensive care unit (PICU) and started on unfractionated heparin (UFH) drip and transitioned to enoxaparin treatment after 72 hours with therapeutic anti-Xa level of 0.53 IU/ml. After 3 days of enoxaparin, the patient was found to have an intra-abdominal bleed as evidenced on abdominal ultrasound (US) and abdominal CT. The enoxaparin treatment was held, and to rapidly control the bleed, she was administered protamine sulfate for enoxaparin reversal, IV vitamin K for potential deficiency-related coagulopathy, and red blood cell transfusion for a hemoglobin of 5.3 g/dl. Factor VIII level was later found to be low at 24% without inhibitor, indicating the patient had mild Hemophilia A 8. Follow-up abdominal US performed a week later showed a hematoma at the root of the mesentery, indicating an acceleration-deceleration injury to her abdomen sustained during the fall, leading to a tear in the root of the mesentery. An MRI/MRV performed 2 weeks after presentation showed no worsening of the CSVT. Anticoagulation treatment was not renewed due to the major abdominal bleed. Repeat MRI/MRV scans performed 3 weeks and 4 months following presentation, showed complete thrombus resolution.
Case 3
An 8-year-old male with type 3 von Willebrand disease (VWD), on demand therapy with VW Factor (VWF) concentrate presented to the ED following head trauma due to a fall from a height of 4 feet. His physical exam showed left parietal scalp hematoma, a head CT scan without contrast showed linear left parietal fracture with small subarachnoid contrecoup bleeding (Table 1). The patient was started on IV VWF concentrate, Haemate-P at 50 units/kg, administered three times a week. Five days following the fall, CTV was obtained for worsening headache. Due to development of new CSVT (left epidural and sagittal vein thromboses), VWF concentrate was discontinued, and anticoagulation therapy was withheld due to risk of worsening bleeding. Two days later, the headache subsided and MRV demonstrated no further bleeding with partial improvement of CSVT. Another MRV obtained a month later demonstrated resolution of the hematoma and complete recanalization of the sagittal sinus thrombosis.
Case 4
A 7-year-old male with no significant past medical history was evaluated for increasing headache four days following an occipital head injury due to an accidental fall. Physical exam showed parieto-occipital hematoma with normal neurological function. A head CT scan showed transverse sinus thrombosis and an epidural bleed. The patient was admitted to the PICU for planned anticoagulation treatment with unfractionated heparin (UFH). However, labs prior to initiation of therapy showed prolonged aPTT and anticoagulation therapy was withheld. Further laboratory evaluation confirmed diagnosis of severe FXI deficiency (FXI level of 4%). An MRI of the brain performed after a month showed absorption of the hematoma and complete recanalization of the CSVT.