Customize your JAMA Network experience by selecting one or more topics from the list below.
A child with venous sinus thrombosis whose initial clinical findings
were consistent with an isolated pseudotumor syndrome without any predisposing
factors is described.
Report of a Case
A 6-year-old white girl was seen at the emergency department of a children's
hospital with complaints of headaches for 3 weeks and diplopia for 2 days.
Three weeks prior to examination, she had 2 episodes of emesis. Findings from
systemic review were negative for fever, neck stiffness, ear pain, or upper
respiratory symptoms. There was no history of tick bites, skin rashes, or
recent travel. She lived in an urban community in Pennsylvania. The remainder
of her medical history was unremarkable.
On examination she was afebrile with normal vital signs, alert, and
cooperative. Visual acuity was 20/20 OU, and color plate test results from
Ishihara plates was normal in each eye. Ocular motility revealed 10% bilateral
abduction deficits. Anterior segment examination results were normal. Fundus
examination findings revealed mild bilateral disc edema. The remainder of
her general and neurologic examination was unremarkable; specifically, no
other cranial nerve deficits were present.
Magnetic resonance imaging scans of the head were normal. Lumbar puncture
disclosed an opening pressure of 450 cm H2O. Cerebrospinal fluid
composition showed a red blood cell count of 2 cells/µL; white blood
cell count, 20 cells/µL (85% lymphocytes, 13% monocytes); and normal
protein and glucose levels. An initial diagnosis of a pseudotumor syndrome
was entertained. However, magnetic resonance venous imaging showed complete
occlusion of right and partial occlusion of left transverse sinus (Figure 1) revealing the diagnosis of venous
Magnetic resonance venous imaging demonstrates complete lack of flow
through the right transverse venous sinus (short arrow) and sluggish flow
through the left transverse sinus (long arrow).
The following laboratory investigation findings were normal: complete
blood cell count, coagulation profile, cardiolipin antibody, antinuclear antibody,
rheumatoid factor, and homocysteine. Serum Lyme titers were positive for IgG
and IgM at 4.1 mg/dL (<0.9 mg/dL, negative; >1.1 mg/dL, positive). Cerebrospinal
fluid polymerase chain reaction and Western immunoblot were positive for Borrelia burgdorferi.
The child was treated with acetazolamide, low-molecular-weight heparin,
and 3 weeks of intravenous ceftriaxone sodium. One month later, the headaches
had resolved and the child had normal ocular motility and normal optic discs.
The incidence of venous sinus thrombosis is estimated to be 3 to 5 new
cases per year at a single hospital.1 The
clinical findings at initial examination may be similar to pseudotumor cerebri.1-3 In children,
however, most cases of venous thrombosis have been associated with evidence
of infection, otitis media, mastoiditis, systemic lupus erythematosus, use
of oral contraceptives, or signs of dehydration.4
Our case demonstrates that even in the absence of identifiable risk factors,
venous sinus thrombosis may occur with only signs of increased intracranial
pressure in children. The outcome is unpredictable with a 10% to 30% mortality
rate; however, anticoagulation may improve neurologic outcome and reduce mortality
in select cases.1
Our patient had positive results on Lyme serologic analysis. Lyme disease
as a possible cause of venous sinus thrombosis has not been previously reported
to our knowledge. Our case suggests that there may be an association between
neuroborreliosis and venous sinus thrombosis.
In conclusion, our case illustrates the necessity of performing magnetic
resonance venous imaging in the presence of normal findings on magnetic resonance
imaging and emphasizes the importance of excluding venous sinus thrombosis
as a cause of a pseudotumor syndrome in children.
Corresponding author and reprints: Kammi B. Gunton, MD, Department
of Ophthalmology, St Christopher's Hospital for Children, Erie Avenue at Front
Street, Philadelphia, PA 19134-1095.
Ansari I, Crichlow B, Gunton KB, Diamond GR, Melvin J. A Child With Venous Sinus Thrombosis With Initial Examination Findings of Pseudotumor Syndrome. Arch Ophthalmol. 2002;120(6):867–869. doi:
Monkeypox Resource Center