**3.1.5 Prognosis**

The prognosis of CVT is generally good. At 16 months, approximately 57% of patients with CVT have no signs or symptoms, and a further 22% of patients with CVT have only minor residual symptoms (Ferro, Canhao et al. 2004). Characteristics that are associated with a poorer prognosis include age over 37, male , previously having a coma or mental status abnormality; haemorrhage noted on imaging; deep venous system thrombosis; central nervous system infection and CVT associated with malignancy (Ferro, Canhao et al. 2004).

Approximately 3% of patients die in the acute phase of CVT and 8% of patients die within the first 30 days following initial symptoms of CVT (Canhao, Ferro et al. 2005). Death is often due to transtentorial herniation or due to diffuse oedema and multiple parenchymal lesions (Canhao, Ferro et al. 2005).

Significant visual loss in CVT is rare (Purvin, Trobe et al. 1995), and if present, is often associated with prolonged elevated intracranial pressure. Visual prognosis depends upon the duration and severity of elevation in pressure.

There is often recanalisation after CVT and this commonly occurs within the first four months. The likelihood of recanalisation occurring depends on the location of the CVT (Baumgartner, Studer et al. 2003). Following a CVT, approximately 3% develop a recurrent CVT and 6% develop another form of venous thromboembolism at 6 years (Martinelli, Bucciarelli et al. 2010).

### **3.2 Ophthalmic manifestations of antiphospholipid syndrome**

Antiphospholipid syndrome (APS) is an autoimmune disease associated with both arterial and venous thrombosis. It is characterised by lupus anticoagulant, anticardiolipin antibody and anti-β2 glycoprotein-I antibody (Miyakis, Lockshin et al. 2006).

#### **3.2.1 Prevalence**

The prevalence of ocular involvement in APS is variable, with studies suggesting that ocular features can occur in 8% to 88% of patients with APS (Utz and Tang 2011).

#### **3.2.2 Clinical presentation**

Ocular manifestations of APS include occlusive vascular disease (Miserocchi, Baltatzis et al. 2001; Suvajac, Stojanovich et al. 2007), vasculitis (Miserocchi, Baltatzis et al. 2001) and neuro-

Venous Thrombosis and the Eye 173

Fig. 10. External photograph showing episcleritis of both eyes of the same patient with antiphospholipid syndrome. The right eye (a) is clinically more severe than the left eye (b).

Fig. 11. External photograph showing scleritis of both eyes with the right eye (a) less severe than the left eye (b). The scleritis associated with antiphospholipid syndrome is often a

Fig. 12. Fundus photograph of a branch retinal artery occlusion in the right eye due to vasculitis (a). The superior retina is pale due to infarction and the occluded artery is clearly

visible on the corresponding fluorescein angiogram (b).

necrotising scleritis.

ophthalmic manifestations. Occlusive vascular disease may occur in the form of central and branch retinal artery or vein occlusion, and choroidal infarction (Ang, Yap et al. 2000) (Figure 9). Vasculitis may manifest as anterior uveitis, episcleritis (Figure 10), scleritis (Figure 11) or retinal vasculitis causing retinal artery occlusion (Figure 12). APS may also be associated with cranial nerve III (Genevay, Hayem et al. 2002) (Figure 13), cranial nerve IV (Shin and Lee 2006) and cranial nerve VI (Shin and Lee 2006) palsies, in addition to ischaemic optic neuropathy (Giorgi and Balacco Gabrieli 1999), and optic neuritis (Giorgi and Balacco Gabrieli 1999).

Fig. 9. Fundus photograph showing central retinal artery occlusion with associated choroidal infarction in the left eye (a) and the corresponding fluorescein angiogram (b).

ophthalmic manifestations. Occlusive vascular disease may occur in the form of central and branch retinal artery or vein occlusion, and choroidal infarction (Ang, Yap et al. 2000) (Figure 9). Vasculitis may manifest as anterior uveitis, episcleritis (Figure 10), scleritis (Figure 11) or retinal vasculitis causing retinal artery occlusion (Figure 12). APS may also be associated with cranial nerve III (Genevay, Hayem et al. 2002) (Figure 13), cranial nerve IV (Shin and Lee 2006) and cranial nerve VI (Shin and Lee 2006) palsies, in addition to ischaemic optic neuropathy (Giorgi and Balacco Gabrieli 1999), and optic neuritis (Giorgi

Fig. 9. Fundus photograph showing central retinal artery occlusion with associated choroidal infarction in the left eye (a) and the corresponding fluorescein angiogram (b).

and Balacco Gabrieli 1999).

Fig. 10. External photograph showing episcleritis of both eyes of the same patient with antiphospholipid syndrome. The right eye (a) is clinically more severe than the left eye (b).

Fig. 11. External photograph showing scleritis of both eyes with the right eye (a) less severe than the left eye (b). The scleritis associated with antiphospholipid syndrome is often a necrotising scleritis.

Fig. 12. Fundus photograph of a branch retinal artery occlusion in the right eye due to vasculitis (a). The superior retina is pale due to infarction and the occluded artery is clearly visible on the corresponding fluorescein angiogram (b).

Venous Thrombosis and the Eye 175

Ocular prognosis depends upon the presence of complications such as neovascularisation

In patients with antiphospholipid syndrome, the risk of further thromboembolic

Similar to systemic venous thrombosis, venous thrombosis in the eye is a manifestation of Virchow's triad. Ocular venous thrombosis presents as retinal vein occlusion. The eye can also be affected by systemic venous thrombosis. Accurate diagnosis of venous thrombosis is vital and early treatment should be implemented in order to preserve existing vision and to

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Anonymous (1986). "Argon laser scatter photocoagulation for prevention of

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Anonymous (1995). "Evaluation of grid pattern photocoagulation for macular edema in

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Asherson, R. A., M. A. Khamashta, et al. (1989). "Cerebrovascular disease and

and the primary antiphospholipid syndrome." Am J Med 86(4): 391-399. Baumgartner, R. W., A. Studer, et al. (2003). "Recanalisation of cerebral venous thrombosis."

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**3.2.5 Prognosis** 

**4. Conclusion** 

**5. References** 

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and therefore, regular ocular monitoring is required.

prevent serious visual, and life threatening complications.

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complications ranges from 22% to 29% (Ruiz-Irastorza, Hunt et al. 2007)

Fig. 13. Clinical photograph of all nine positions of gaze in a patient with a right pupil involving cranial nerve III palsy with right ptosis and exotropia on primary gaze.

Patients with retinal vaso-occlusion usually present with pain, unilateral or bilateral decreased visual acuity, visual field defect or amaurosis fugax. Bilateral amaurosis fugax is a very serious condition, and may indicate the presence of central nervous system ischaemia. Those with cranial neuropathy present with acute binocular diplopia.

On examination, patients with APS may have dry eyes, conjunctival telangiectasia (Miserocchi, Baltatzis et al. 2001) and corneal infiltrates or keratitis. Ocular fundus examination may reveal tortuous and dilated retinal veins, intra-retinal haemorrhages, cotton-wool spots, optic disc and macular oedema or neovascularisation.

### **3.2.3 Investigations**

Ocular imaging with fluorescein angiography directly visualizes the retinal vessels and occlusion. An MRI scan may be important if there is visual loss without ocular fundus changes, as it is important to investigate the possibility of CNS ischaemia.

In addition to imaging, a full thrombophilia work-up may be necessary in the absence of traditional thrombophilia risk factors. This would include searching for antiphospholipid antibodies, including lupus anticoagulant, anticardiolipin antibody and anti-β2 glycoprotein-I antibody. Other screening tests should include homocysteine, protein C and S, plasminogen, anti-thrombin levels, activated protein C resistance and factor V Leiden mutation.

#### **3.2.4 Treatment**

Early detection of ocular manifestations of APS is important as it allows treatment and prevention of further systemic disease. This first involves determining the patient's thrombotic risk.

The treatment of the systemic disease is vital as it may reverse the retinal ischaemia (Srinivasan, Fern et al. 2001). This should include reducing modifiable risk factors such as hypertension, hyperlipidaemia, diabetes mellitus, oral contraceptive use, hormone replacement therapy and smoking.

Treatment should also target the cause of the thrombosis. APS with venous thrombosis requires anticoagulation with warfarin while APS with arterial thrombosis requires antiplatelet agents (Ruiz-Irastorza, Hunt et al. 2007).
