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Clinicopathologic Reports, Case Reports, and Small Case Series
June 2002

A Child With Venous Sinus Thrombosis With Initial Examination Findings of Pseudotumor Syndrome

Arch Ophthalmol. 2002;120(6):867-869. doi:

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 sinus thrombosis.

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).

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.

Broderick  JP Cerebral venous thrombosis. MedLink Web site, Neurology section.2nd2001;Available at: http://www.medlink.com.Accessed January 31, 2002.
Lam  BLSchatz  NJGlaser  JSBowen  BC Pseudotumor cerebri from cranial venous obstruction.  Ophthalmology. 1992;99706- 712Google ScholarCrossref
Biousse  VAmeri  ABousser  M-G Isolated intracranial hypertension as the only sign of cerebral venous thrombosis.  Neurology. 1999;531537- 1542Google ScholarCrossref
Lessell  S Pediatric pseudotumor cerebri (idiopathic intracranial hypertension).  Surv Ophthalmol. 1992;37155- 166Google ScholarCrossref