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.