Download Dural Sinus (Cerebral Venous) - University of Kansas Medical Center

ILLUSTRATIVE CASE
Dural Sinus (Cerebral Venous) Thrombosis in a Pediatric
Trauma Patient
A Rare Complication After Closed Head Injury
Jonathan Wilcher, MD and Michelle Pannell, MD
Objective: Closed head injury rarely predisposes patients, particularly
children, to the development of dural sinus thrombosis. In addition, most
cases of sinus thrombosis are subacute in nature. The following is a case
report of a precipitous course of dural sinus thrombosis after closed head
injury in a pediatric trauma patient.
Description: A 14-year-old African American girl presented to the emergency department as a trauma activation. She was an unrestrained rear-seat
passenger involved in a motor vehicle collision during which she was
ejected. She experienced loss of consciousness and was found unresponsive at the scene. She was intubated and then transported via helicopter
to the level I trauma center. Initial head computed tomography (CT)
revealed no acute intracranial abnormality. The head CT was repeated
15 hours later when nursing noted the patient to be acutely hypertensive
and tachycardic with an 8-mm nonreactive right pupil. The repeated CT
showed interval development of extensive dural sinus thrombosis, cerebellar edema and infarct, as well as mild downward transtentorial herniation. Because of multiple coexistent injuries and complications, treatment
of the dural sinus thrombosis was difficult. The hospital course was complicated and there was very little improvement in the patient's condition.
Conclusions: The multisystem injured trauma patient poses many clinical challenges. Treatment of dural sinus thrombosis is difficult and controversial and requires an investigation into possible risk factors for a
hypercoagulable state. Clinical outcomes vary from excellent to dismal.
Key Words: dural sinus thrombosis, pediatric head injury, pediatric trauma
(Pediatr Emer Care 2015;00: 00–00)
CASE
A morbidly obese 14-year-old African American girl presented to the emergency department as a trauma activation. She
was an unrestrained rear-seat passenger involved in a motor vehicle collision during which she was ejected and severely injured.
At the scene, the patient was unconscious and responsive only
to painful stimuli. Cervical spine stabilization and immobilization
were instituted, intravenous access was obtained, and she was
intubated in accordance with the rapid sequence intubation protocol of the emergency medical services. She was then transported
via helicopter to the level I trauma center. According to emergency
medical services providers, the patient had no significant medical/
surgical history, she was not taking any medications, and she had
no known allergies. Social history and family history were unknown. The patient was unable to provide any history.
On arrival to the emergency department, the patient was
assessed in accordance with the standard advanced trauma life
support protocol. The patient's initial Glasgow Coma Scale was
From the Department of Emergency Medicine, University of Kansas Medical
Center, Kansas City, KS.
Disclosure: The authors declare no conflict of interest.
Reprints: Jonathan Wilcher, MD, Department of Emergency Medicine,
University of Kansas Medical Center, Mail Stop 1045, Kansas City, KS
66160 (e‐mail: [email protected]; [email protected]).
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 0749-5161
Pediatric Emergency Care • Volume 00, Number 00, Month 2015
3TP (eye, 1; verbal, 1; motor 1; intubated; and paralyzed). Her
pupils were noted to be equal and round as well as reactive to
light. The remainder of the physical examination was notable for
a 4-cm laceration on the right frontal scalp, a 1.5-cm laceration
on the left eyelid, a large abrasion on the left side of the face, abrasions on all extremities, a deep wound on the right upper extremity, an absent left radial pulse, and an obvious deformity with an
open fracture of the left radius and ulna. Her initial vital signs were
rectal temperature of 37.5°C, blood pressure of 193/113 mm Hg,
heart rate of 116 beats per minute, respiratory rate of 20 breaths
per minute, and an oxygen saturation of 100%. She was given intravenous fluids, pain medication, and sedation medication, with
return of vital signs to acceptable limits.
After the initial assessment and stabilization, the patient underwent multiple radiographic studies. The initial head computed
tomography (CT) revealed no acute intracranial abnormality, bilateral frontal scalp lacerations, bilateral parietal scalp soft tissue
thickening, mild asymmetric prominence of the right lateral ventricle without hydrocephalus, and no calvarial fracture. Cervical
spine CT was negative for any fracture. Computed tomography
of the abdomen and pelvis demonstrated a grade 3 to 4 spleen
laceration, free fluid in the pelvis, a questionable left kidney laceration versus infarction, and bilateral parenchymal lung densities.
Chest radiograph demonstrated right upper lobe collapse versus
opacification. Multiple films of all extremities revealed a fracture
of the middle one third of the left ulna and radius as well as a
Salter II fracture of the left radius.
The patient was admitted to the pediatric intensive care unit
(ICU) where she remained intubated and received supportive therapy, monitoring, and interventions as appropriate. Suddenly, after
15 hours, she developed hypertensive emergency with a blood
pressure of 240/120 mm Hg, tachycardia to 216 beats per minute,
and a dilated right nonreactive pupil. She emergently underwent
repeated CT imaging. Head CT revealed an interval development
of a hyperdensity consistent with thrombus throughout the superior sagittal sinus, straight sinus, left transverse sinus to the junction of the left transverse and left sigmoid sinus, as well as right
transverse and sigmoid sinuses to the junction of the right sigmoid
sinus and the right internal jugular vein. In addition to the dural sinus thrombosis, there was an interval development of edema at the
cerebellar hemispheres, the right temporal region, and the right
caudate head/internal capsule region. This was consistent with venous infarct/ischemia and was without parenchymal hemorrhage.
Mild hydrocephalus and mild downward transtentorial herniation
were also noted. Head/neck CT angiography was suboptimal but
without evidence for vascular injury. Intracranial pressure monitoring was initiated, and she was treated aggressively with mannitol,
3% saline, and antihypertensive medications. Given her condition and other injuries, she was not a candidate for anticoagulation therapy and interventional radiology thrombolysis was
deemed too risky.
The patient experienced extensive dural sinus thrombosis
and subsequently had a complicated and protracted ICU stay. Secondary to the combined efforts of multiple specialists, the patient
www.pec-online.com
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
1
Pediatric Emergency Care • Volume 00, Number 00, Month 2015
Wilcher and Pannell
left the ICU after 2 months and was eventually transferred to a rehabilitation facility. A repeated head CT scan at that time revealed
a progressive patchy encephalomalacia of the cerebral and cerebellar hemispheres as well as the basal ganglia consistent with venous infarction after dural sinus thrombosis. Persistent thrombus
was noted within the superior sagittal sinus and the left transverse
sinus. She had regained the ability to open her eyes and blink in
response to commands but was unable to perform any other functions. Every attempt was made to determine any underlying cause
for the thrombosis, but no known disorder of coagulation was discovered in the patient or in her immediate family. Unfortunately,
there was very little improvement in her condition and, overall,
her prognosis is poor.
DISCUSSION
Dural sinus thrombosis, also known as cerebral venous
thrombosis (CVT), is a thrombosis or clot within the brain's venous system. This system begins with venules and veins that then
drain into the great dural sinuses. Although the first description of
CVT occurred in the French literature in 1825, the true incidence
is still unknown.1 The first report of posttraumatic thrombosis
occurred in 1915.2 Lacking good epidemiologic data, it is difficult to accurately determine the true incidence. However, the incidence is likely higher than what was originally thought. Bousser
and Ferro3 stated that it is a “rare type of cerebrovascular disease
affecting about 5 people per million and can occur at any age”
and that it is responsible for 0.5% of all strokes. Cerebral venous
thrombosis seems to be slightly more common in women than
in men and there is no apparent predilection for race.4 Thrombosis
is much more common in adults than in children.
Thrombus formation is often multifactorial. The 3 main factors that predispose to clot formation are known as Virchow triad.
This triad consists of endothelial injury, stasis or turbulence of
blood flow, and blood hypercoagulability. Hypercoagulable states
are classified as either genetic or acquired. Known genetic causes
include mutations in factor V, antithrombin III deficiency, protein
C or S deficiency, fibrinolysis defects, homocysteinemia, and allelic variations in prothrombin levels. Acquired causes include
prolonged immobilization, myocardial infarction, tissue damage,
cancer, disseminated intravascular coagulation, heparin-induced
thrombocytopenia, antiphospholipid antibody syndrome, atrial fibrillation, nephrotic syndrome, hyperestrogenic states, oral contraceptive use, sickle cell anemia, smoking, advancing age, and
obesity.5 After formation, a clot will propagate, embolize, dissolve
via fibrinolytic activity, or recanalize. The significance of a venous thrombus is dependent on its location, and detrimental effects are the result of congestion and edema in the vascular beds
distal to the obstruction.5
Dural sinus thrombosis is often difficult to diagnose. Signs
and symptoms are often vague and nonspecific and may include
headache, papilledema, focal neurologic deficit, cranial nerve
palsies, seizure, altered mental status, amnesia, or coma.1,3,6 Symptoms are generally subacute in nature.1,7 In a study of 40 patients
in 1995, Daif et al,1 reported acute (<48 hours) onset of symptoms in 35% of patients, subacute (<1 month) onset in 40% of
patients, and progressive/chronic onset in 25% of patients. Neuroimaging is required to definitively diagnose CVT. Head CT may
be normal in 25% of patients, and magnetic resonance imaging/
magnetic resonance venography is now considered the criterion
standard for diagnosis.3 Treatment occurs on a case-to-case basis
with identification and management of any underlying cause,
anticoagulation and antithrombotic therapy as deemed appropriate, as well as symptomatic treatment of elevated intracranial pressure, seizures, etc.3,8,9 Mortality has been reported
2
www.pec-online.com
to be from 5% to 30%8,9 with a generally good outcome overall.
It has been reported that 15% to 25% of patients will have some
lasting deficit.2 Poor outcomes are more likely to be associated
with intracranial hemorrhage, a decreased mental status defined
as a Glasgow Coma Score of lower than 9, focal neurologic deficits, seizures, and infarct.10 It has been reported that up to 80% of
patients are functionally independent in the long-term.8 Dural sinus thrombosis is less common in children than in adults but
has been reported in the literature to be secondary to dehydration,
metabolic acidosis, sepsis, localized central nervous system infection, and cyanotic heart disease as well as in various hypercoagulable states. Children often have worse functional outcomes.11
This case is reported because of the paucity of literature on
dural sinus thrombosis in pediatric trauma patients and the complexity of treatment. The etiology of posttraumatic dural sinus
thrombosis is unclear. Meena et al7 and D'Alise et al2 reported 2
separate cases of young adults with dural sinus thrombosis after
head trauma from motor vehicle collisions. Both cases describe
a subacute course, with signs, symptoms, and diagnosis occurring
approximately 10 days after the initial injury. Rich et al11 reported
in 1993 of a 4-year-old boy who developed thrombosis of the
straight, superior sagittal, and transverse sinuses 2 weeks after
minor head trauma without loss of consciousness. He was subsequently diagnosed with inherited protein S deficiency. This
literature postulates that endothelial damage and intramural hemorrhage secondary to rupture of the sinusoids at the site of entry
of the draining veins may initiate the thrombotic process after a
closed head injury.2,7,11 It is also postulated that an underlying
inherited pathology will potentiate the formation of a thrombus
precipitated by trauma. The case presented here is unique for 2
reasons. First, the patient experienced a massive thrombosis of
her dural sinus system acutely after an initial negative head CT.
The second reason is that a genetic or an acquired predisposition
to hypercoagulability was not identified. This patient also reinforces the premise of a case-to-case management. This multisystem trauma patient developed an acute dural sinus thrombosis.
Anticoagulation was contraindicated secondary to her other potentially life-threatening injuries, and symptomatic treatment was
simultaneously managed by the trauma surgeons, neurosurgeons,
neurologists, and pediatric intensivists. Unfortunately, this patient
experienced a poor outcome without any clear etiology.
CONCLUSIONS
Closed head injury rarely predisposes patients, particularly
pediatric patients, to the development of dural sinus thrombosis.
In addition, most cases of dural sinus thrombosis are subacute in
nature. However, thrombosis formation is unpredictable and the
diagnosis should be entertained in any symptomatic patient or in
any patient who deteriorates clinically, regardless of the time
frame. Treatment of dural sinus thrombosis is difficult and somewhat controversial, and it requires an investigation into possible risk factors for hypercoagulability. The multisystem injured
trauma patient poses additional clinical challenges, and each case
must be managed individually. Clinical outcomes are not easily
predicted9 and vary from excellent to dismal.
REFERENCES
1. Daif A, Awada A, Al-Rajeh S, et al. Cerebral venous thrombosis in adults.
A study of 40 cases from Saudi Arabia. Stroke. 1995;26:1193–1195.
2. D'Alise MD, Fichtel F, Horowitz M. Sagittal sinus thrombosis following
minor head injury treated with continuous urokinase infusion. Surg Neurol.
1998;49:430–435.
© 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
Pediatric Emergency Care • Volume 00, Number 00, Month 2015
Dural Sinus Thrombosis
3. Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet
Neurol. 2007;6:162–170.
8. Hameed B, Syed NA. Prognostic indicators in cerebral venous sinus
thrombosis. J Pak Med Assoc. 2006;56:551–554.
4. McElveen WA. Cerebral venous thrombosis. Available at: emedicine.com.
Accessed October 3, 2006.
9. Benamer HT, Bone I. Cerebral venous thrombosis: anticoagulants or
thrombolytic therapy? J Neurol Neurosurg Psychiatry. 2000;69:
427–430.
5. Mitchell RN, Cotran RS. Hemodynamic disorders, thrombosis, and shock.
In: Robbins Pathologic Basis of Disease. 6th ed. Philadelphia, PA: W.B.
Saunders Company; 1999:113–138.
6. Preter M, Tzourio C, Ameri A, et al. Long-term prognosis in cerebral
venous thrombosis. Stroke. 1996;27:243–246.
7. Meena AK, Jayalaxmi SS, Murthy JM. Cortical sinovenous thrombosis
following closed head injury. Neurol India. 2000;48:195–196.
© 2015 Wolters Kluwer Health, Inc. All rights reserved.
10. Girot M, Ferro JM, Canhão P, et al. Predictors of outcome in patients
with cerebral venous thrombosis and intracerebral hemorrhage. Stroke.
2007;38:337–342.
11. Rich C, Gill JC, Wernick S, et al. An unusual cause of cerebral
venous thrombosis in a four-year-old child. Stroke. 1993;24:
603–605.
www.pec-online.com
Copyright © 2015 Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
3