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Cerebral sinovenous thrombosis in a child with

Crohn's disease, otitis media, and meningitis
Article June 2015
DOI: 10.1177/1971400915589688

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Case report

Cerebral sinovenous thrombosis in a child with

Crohns disease, otitis media, and meningitis

The Neuroradiology Journal

2015, Vol. 28(3) 274277
! The Author(s) 2015
Reprints and permissions:
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DOI: 10.1177/1971400915589688
neu.sagepub.com

Omer Selvitop, Andrea Poretti, Thierry AGM Huisman and

Matthias W Wagner

Abstract
Pediatric cerebral sinovenous thrombosis (CSVT) is associated with high morbidity and mortality. Severe long-term sequelae
are reported in up to 48% of children. The most frequent location of CSVT in children is the superficial venous system. We
present the neuroimaging findings using both computed tomography and magnetic resonance imaging (MRI) in a 10-yearold child with extensive superficial CSVT. Our report aims to stress the importance of awareness of risk factors in suspecting
and rapidly diagnosing CSVT. The application of targeted conventional and advanced MRI sequences is the diagnostic tool of
choice in children at risk of or with clinically suspected CSVT.

Keywords
sinovenous thrombosis, neuroimaging, children, magnetic resonance imaging, risk factor

Introduction

Case report

Cerebral sinovenous thrombosis (CSVT) is dened by

thrombosis of the supercial (cortical veins, superior
sagittal sinus, transverse sinus, sigmoid sinus, and jugular vein) or deep (inferior sagittal sinus, internal
cerebral veins, vein of Galen, straight sinus) venous
system.1 CSVT occurs in about 1 of 100,000 children
per year and accounts for 1 in 4 cases of pediatric
stroke.2 CSVT is associated with high mortality and
morbidity. The mortality rate ranges between 819%
and severe long-term neurological sequelae occur in up
to 48% of children.2,3 Prognosis is related to the extent
of vessel and brain parenchymal involvement as well to
timeliness of diagnosis and institution of therapy. A
rapid institution of therapy including anticoagulation
may prevent thrombus propagation and involvement
of a larger brain region (e.g. intraparenchymal venous
stasis or venous stroke) and improve the long-term outcome.4 The diagnosis of CSVT must consequently be
made as soon as possible after onset of symptoms or
should be ruled out in children at high risk for thrombosis, even when only minor symptoms are present.
We report on a 10-year-old girl with Crohns disease
who presented with headaches and neck pain and
stiness due to bacterial meningitis and CSVT. We discuss the key role of the clinician and neuroradiologist
to be aware of the multiple risk factors that can increase
the likability of CVST to develop and the diagnostic
signicance of head magnetic resonance imaging
(MRI) in making a rapid diagnosis of CSVT.

A 10-year-old girl presented to the emergency department (ED) of our tertiary childrens hospital with
worsening headache. Based on a normal neurological
exam and transient improvement upon analgesic drugs,
she was discharged home. Two days later, she presented
again to the ED because of severe, pulsating headaches
in the left temporal and periorbital regions. In addition,
she developed neck pain and stiness, photophobia,
and phonophobia. The neurological exam revealed a
positive Brudzinski sign.
On retrospect, the headaches had started about 4
weeks before the presentation at the ED, were intermittent, located in the left temporal region, and associated
with dizziness and blurry vision. One week prior to presentation, the symptoms were worsening with episodes
of nausea and vomiting. At that time, the patient had a
head computed tomography (CT) scan at an outside
hospital, which was reported as normal. In addition,

Russell H Morgan Department of Radiology and Radiological Science, The

Johns Hopkins University School of Medicine, Baltimore, MD, USA
Corresponding author:
Matthias W Wagner, Section of Pediatric Neuroradiology, Division of
Pediatric Radiology, The Russell H Morgan Department of Radiology and
Radiological Science, The Johns Hopkins School of Medicine, Charlotte R.
Bloomberg Childrens Center, Sheikh Zayed Tower, Room 4174, 1800
Orleans Street, Baltimore, MD 21287-0842, USA.
Email: m.w.wagner@me.com

Selvitop et al.
she developed bilateral acute otitis media two weeks to
presentation.
The patients past medical history is notable for
atypical Crohns disease since the age of 5 years. The
medical management of Crohns disease was dicult
and remission was obtained only under continuous
daily steroid therapy. The child became steroid dependent and developed a secondary adrenal insuciency
that needed hydrocortisone substitution. In addition,
the child developed a secondary vitamin D deciency
that needed substitution with cholecalciferol. In an
attempt to reduce the steroid dependency, an immunosuppressive therapy with thalidomide 50 mg daily was
started. Immunosuppression caused recurrent otitis
media and sinusitis requiring antibiotic therapy.
Neck pain and stiness, photophobia, phonophobia,
and a positive Brudzinski sign at the time of ED presentation were suggestive of meningitis, which was
conrmed by a lumbar puncture and cerebrospinal
uid (CSF) exam. CSF as well as peripheral blood culture were positive for Streptococcus anginosus. Bacterial

275
meningitis was considered secondary to otitis media in
an immunosuppressed child. An antibiotic therapy with
ceftriaxone was started. In addition, a brain MRI was
performed that showed a T2 hyperintense and T1 isointense tubular structure with restricted diusion within
the bilateral proximal internal jugular vein as well as
sigmoid and transverse sinus corresponding to an
extensive thrombus (Figure 1). In addition, there was
an area of restricted diusion noted within the left cavernous sinus suggesting partial left sided cavernous
sinus thrombosis. There was no leptomeningeal
enhancement or evidence of infarction or hemorrhage.
A complementary head CT scan revealed bilateral otomastoiditis and a large area of osseous dehiscence in the
left mastoid complex communicating with the left
middle cranial fossa (Figure 2).
Because of the diagnoses of bacterial meningitis and
extensive CSVT, the child was transferred to the pediatric intensive care unit and an anticoagulation therapy
with enoxaparin was started. Although the child had
multiple risk factors for CSVT (otitis media,

Figure 1. MRI of a 10-year-old girl with extensive CSVT: (a) axial T2-weighted image shows hyperintense signal in the left (long arrows)
and right (arrowheads) transverse and sigmoid sinus and mixed hyper- and isointense signal in the left cavernous sinus (short arrows);
(b) sagittal T1-weighted image reveals isointense signal in the left transverse sinus (long arrows); (c) axial trace of diffusion and (d)
apparent diffusion coefficient (ADC) maps show DWI-bright signal in the left (long arrows) and right (arrowheads) transverse and sigmoid
sinus and the left cavernous sinus (short arrows) with matching low ADC values.

276

The Neuroradiology Journal 28(3)

Figure 2. CT of a 10-year-old girl with extensive CSVT: axial (a) and coronal (b) CT images of the skull base reconstructed in bone
algorithm show bilateral mastoid effusion, left > right (long arrows) and an area osseous dehiscence in the left mastoid complex
communicating with the left middle cranial fossa (short arrow).

mastoiditis, meningitis, and Crohns disease), a thrombophilia workup including cardiolipin, B2 glycoprotein, protein C/S, homocysteine, antithrombin III,
and antiphospholipid a/b level and MTHFR and prothrombin 20212 gene mutation was performed and
revealed normal results.
The antibiotic therapy was continued for 6 weeks. A
clinical follow-up exam 6 weeks after diagnosis was
unremarkable. A follow-up brain MRI at the same
time showed recanalization of the right proximal jugular vein, and sigmoid and transverse sinus, while there
was persistent thrombosis of the left transverse and sigmoid sinus.

Discussion
Epidemiology studies suggest that CSVT aects
about 1 of 100,000 children per year and accounts for
1 in 4 cases of childhood ischemic stroke.2 A prompt
diagnosis of CSVT in children is clinically challenging
because of the sometimes subtle and usually non-specic clinical presentation. An early diagnosis however is
essential to eecting the best possible outcome. A high
index of suspicion is needed for clinicians and radiologists who need to be aware of the various risk factors
for CSVT in children.
Risk factors are denable in more than 98% of children with CSVT.5 They are frequently multiple and are
age-related. In newborns and infants, dehydration and
perinatal events are common. Head and neck infections
are present in about one-third of preschool children,
while other associations, including systemic disease
and trauma, are clustered in the older age groups.
Infection is a major risk factor for childhood CSVT.5
Septic CSVT is dened as thrombosis of venous sinuses
associated with head and neck infections, including
otitis media, mastoiditis, sinusitis, and meningitis as

in our patient. Septic CSVT may account for 1850%

of childhood CSVT.5 The common pathomechanism
involves spread of infection from adjacent tissues into
venous sinuses leading to thrombophlebitis. In addition, acute bacterial infection and sepsis impart other
risk factors such as dehydration, systemic inammation, and coagulopathy. Chronic systemic diseases
also increase the risk of childhood CSVT, especially
in older school-age children. In inammatory bowel
disease such as Crohns disease, systemic inammation
may combine with gastrointestinal disease factors, such
as dehydration, medications, and iron deciency.6 In
pediatric inammatory bowel diseases, neurologic
manifestations are rare, occur in about 3% of the children, and are dominated by vascular complications,
including arterial occlusion, CSVT, and vasculitis.6 In
addition, mononeuropathies and abnormalities of the
white matter have been reported.7 Our patient displayed three risk factors for CSVT: (1) a chronic systemic disease (Crohns disease) treated with (2) a
procoagulant drug (thalidomide);8 and (3) an acute
infectious disease of the head or neck (bilateral otomastoiditis and meningitis). Awareness of these risk factors
led to early diagnosis, therapy, and determined the
good outcome of the extensive CSVT in our patient.
After the identication of risk factors, neuroimaging
plays the key role in the diagnosis of CSVT.5 The primary goal of neuroimaging is (1) to visualize and characterize the thrombus, (2) to identify the degree of
impaired ow within the aected venous system, and
(3) to rule out secondary complications such as venous
ischemia or hemorrhage. A number of dierent techniques have been applied in the diagnostic work-up of
CSVT: CT with or without venography, MRI with or
without venography, conventional angiography, and
trans-fontanel Doppler ultrasonography.5 In most circumstances, MRI is the diagnostic modality of choice

Selvitop et al.
in pediatric CSVT. MRI lacks radiation and may provide additional details of both brain parenchyma and the
venous system combining conventional and advanced
MRI sequences such as diusion weighted/tensor imaging (DWI/DTI) and susceptibility weighted imaging
(SWI). MRI sequences sensitive to vasogenic edema
(e.g. uid attenuation inversion recovery (FLAIR),
DWI, and DTI), cytotoxic edema (e.g. DWI and DTI),
and blood products (e.g. SWI) can conrm and characterize CSVT-related parenchymal changes. MRI may
also image thrombosis itself, possibly providing more
information about the age and nature of the thrombus/lesion. Subacute thrombi often appear hyperintense
on T1- and T2-weighted images.
A number of potential pitfalls may challenge the
MRI diagnosis of CSVT.2 Common pitfalls are congenital asymmetry of the transverse sinuses without
cerebral venous pathology,2 and atretic segments in
the transverse sinus with otherwise normal brain MRI
studies. Such ow-gaps occur more frequently in the
smaller or non-dominant sinus.2 Another common pitfall is arachnoid granulations. They are physiologic
small lling defects within the dural sinuses and have
a density (CT)/signal intensity (MRI) identical to cerebrospinal uid. They can be isointense on T1-weighted
sequences and on FLAIR sequences, but should not be
hyperintense on either of these sequences.2,9
A high index of suspicion as well as familiarity with
the neuroimaging ndings is important for an early,
sensitive, and specic diagnosis of CSVT in children.
In conclusion, our case demonstrates that the awareness of risk factors is the most important premise for an
early and accurate diagnosis of CSVT in children and
that neuroimaging is the diagnostic tool of choice to
conrm CSVT and to follow-up patients with conrmed diagnosis.
Funding
This research received no specic grant from any funding
agency in the public, commercial, or not-for-prot sectors.

277
Conflict of interest
The authors report that there are no conicts of interest.

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