May 31, 2019 Conference

  


Title
Venous Sinus Thrombosis and Vision Loss in a Pediatric Patient with Elevated Lipoprotein (A)

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Author(s)
Meredith Kim (Presenter)
Dr. Wendy Chen
Abstract

Meredith Kim1 MD and Wendy Chen1 MD

Brown University Alpert Medical School, Division of Ophthalmology, Providence, Rhode Island

Title:

            Venous Sinus Thrombosis and Vision Loss in a Pediatric Patient with Elevated Lipoprotein (A)

Purpose:

To present a case of vision loss in a pediatric patient with cerebral venous sinus thrombosis with special attention to workup and management.

Methods:

            Case Report.

Results:

  • An 11-year-old male patient with past medical history of chronic headaches and recent head trauma while playing hockey, presented to neurology with complaint of worsening headache. His initial exam was notable for bilateral disc edema, prompting referral to the Emergency Department and urgent Ophthalmology Consult. Family history was significant for multiple family members with thrombosis, early cardiac events and/or death. On exam, he was noted to have prominent veins on the forehead. Visual acuity was 20/20 OU, IOP was normal, and an APD OS was present. Slit lamp biomicroscopy was notable only for mild diffuse conjunctival injection. On dilated fundus exam, significant bilateral disc edema was confirmed (Figures 1). Visual field testing revealed dense scotomas in both eyes, left worse than right.  MRI demonstrated optic nerve findings consistent bilateral optic nerve edema (Figure 2). MRV revealed extensive but non-occlusive venous sinus thrombosis (Figure 3). Extensive hypercoagulable workup was performed (Figure 4).  Notably, lipoprotein (A) was found to be elevated. The patient was started on heparin and acetazolamide. He underwent multiple lumbar punctures with elevated opening pressures; Each required periprocedural suspension of anticoagulation. He also developed metabolic acidosis, a known adverse effect of acetazolamide. Despite treatment, there was minimal improvement in clinical exam. He had continued severe bilateral optic nerve edema and visual field defects.  His central vision in the left eye declined to 20/30.  Neurosurgery was consulted, and an LP shunt was placed. Post-operative course was complicated by low pressure headaches and cerebellar tonsillar herniation. Optic nerve edema improved following LP shunt placement. Final visual acuity has stabilized at 20/20 OD and 20/70 OS. Repeat MRV 3 months later shows persistent and large clot burden despite continued anticoagulation therapy.

Conclusion:

Cerebral venous sinus thrombosis (CSVT) is a life-threatening condition with the potential for permanent vision loss. Given the often non-specific presenting symptoms, a high index of suspicion is needed in order to establish a diagnosis and initiate treatment early. Elevated lipoprotein (A) has been identified as a new risk factor of CVST.1,2 Despite early treatment, CVST remains difficult to manage.

Figure 1.

Appearance of right and left fundus at symptom onset.

      

Figure 2.

T2-Weighted MRI with fat suppression revealed symmetric dilation of the optic nerve sheaths with fluid, flattening of the posterior globes, and anterior bowing of the optic nerves.

Figure 3.

MRV revealed extensive but non-occlusive thrombosis of the distal superior sagittal sinus, right transverse and sigmoid sinuses. Findings of slow flow in the left transverse sinus with small nonocclusive thrombus at the junction of the left transverse and sigmoid sinuses.

Figure 4. Hypercoagulability Workup

Abnormal

Normal

ESR

92 (0.00 - 15 mm/h)

Anticardiolipin Antibody, IgA

< 9.4 (0.0 - 11.9 APL)

CRP

26.4 (0 .00 - 10.00 mg/L)

Anticardiolipin Antibody, IgG

< 9.4 (0.0 - 14.9 APL)

PT

14.2 (10.2 - 12.0 sec)

Anticardiolipin Antibody, IgM

< 9.4 (< 12.5 APL)

INR

1.3 (0.8 - 1.2)

Alpha-2-Antiplasmin

129 (70-150%)

Fibrinogen

540 (154 - 448mg/dL)

Factor V Leiden Mutation, DNA

Negative

Factor VIII Activity

150 (53 - 131)

Antinuclear antibody

Non-reactive

Lupus Anticoagulant

1.31 (< 1.21)

Ristocetin Co-factor

128 (40 - 180 %)

Fecal calprotectin

283.7 (0.0 – 49.9 mg/kg)

Prothrombin Gene

Negative

Lipoprotein (A)

57 (

Protein C

  1. 80 - 170%)