Ординатура / Офтальмология / Английские материалы / Moorfields Manual of Ophthalmology_Jackson_2007

Investigations A clinical diagnosis. Fluorescein angiography shows pinpoint areas of enlarging hyperfluorescence with late staining of the disc and subretinal fluid. If a meningo-encephalitic picture occurs, request a neurological opinion, or if the eye lesions are atypical. B-scan ultrasound may help exclude posterior scleritis.

Treatment and follow–up For the treatment of posterior uveitis see page 342. Second-line agents may be required to allow steroid dose reduction if inflammation is severe or prolonged. The length of treatment depends on the optic neuritis. Retinal detachments usually settle rapidly, leaving considerable RPE mottling or a ‘sunset glow’ fundus. Choroidal granulomas leave Dalen-Fuchs’ nodules (Fig. 8.9). It is often possible to stop systemic treatment after 1 year. Recurrences are typically as an anterior uveitis only and respond to topical treatment.

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Sympathetic ophthalmia

Sympathetic Ophthalmia

Background Bilateral panuveitis following penetrating ocular injury, surgery, or nonpenetrating cyclodestructive procedures.

Symptoms Bilateral subacute discomfort or visual loss usually 1–12 months after the inciting event (range 5 days to 60 + years).

Signs Typically bilateral granulomatous panuveitis with diffuse choroidal involvement or multiple yellow-white Dalen-Fuchs’ nodules, often with papillitis or peripapillary oedema (See Fig. 8.9). There is a broad spectrum of disease activity that may appear confined to the anterior segment.

Differential diagnosis Vogt-Koyanagi-Harada syndrome, multifocal choroiditis, phakoanaphylactic uveitis, and sarcoidosis.

Investigations A clinical diagnosis, although in apparently isolated anterior disease consider ICG angiography to exclude choroidal involvement.

Treatment The treatment of posterior uveitis is detailed on page 342. There is no role for enucleation of the traumatized eye if there is visual potential. Use topical steroids alone for inflammation limited to the anterior chamber. Systemic steroids are usually required and second-line agents may also be needed. Recurrences are likely, often with fresh Dalen-Fuchs’ nodules or peripapillary oedema.

Follow–up It is unlikely that treatment can be stopped for at least 1 year. There is a reasonable long-term visual prognosis, but a high risk of cataract and glaucoma.

Presumed Ocular

Histoplasmosis Syndrome

■Presumed ocular histoplasmosis syndrome (POHS) affects all ages with no sex bias.

■The characteristic triad is of peripapillary atrophy, multiple atrophic ‘histo-spots’ (Fig. 8.10), predominately around the posterior pole and often with internal pigment clumping, and serous or haemorrhagic disciform detachment due to choroidal neovascularization (CNV), usually associated with a macular ‘histo-spot’.

■Linear streaks of chorioretinal atrophy are sometimes seen.

■Vitreous cells are absent.

■Usually asymptomatic until CNV develops.

■The diagnosis is clinical but request a fluorescence angiogram if CNV is suspected.

■Focal argon laser is appropriate for juxtaand extrafoveal CNVs. Oral steroids are of no benefit. Photodynamic therapy is unproven. As the CNV is type II, it may benefit from surgical removal. The role of anti-VEGF agents has yet to be fully determined.

■Offer an Amsler chart to monitor the other eye.

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Multifocal choroiditis and panuveitis

Multifocal Choroiditis

and Panuveitis

■Affects all ages.

■Acute, usually bilateral onset of anterior chamber and vitreous inflammation with lesions of varying size and age in the periphery, posterior pole, or both. Acute lesions are pale yellow or grey (Fig. 8.11).

■Exclude sarcoidosis.

■Usually follows a relapsing–remitting course.

■VA is good unless cystoid macular oedema or choroidal neovascularization occur.

■Cystoid macular oedema may respond to systemic steroids.

Punctate Inner Choroidopathy

■Punctate inner choroidopathy (PIC) usually affects young myopic females.

■Presents with bilateral acute blurring of vision or photopsia with small yellow or grey spots around the posterior pole at the level of the choroid or RPE.

■Few if any vitreous cells are present.

■Fluorescein angiography of acute lesions shows early hyperfluorescence and late staining.

■Recurrences are uncommon but can occur.

■There is a high risk of choroidal neovascularization which may respond to systemic or intravitreal steroids, or photodynamic therapy. The role of anti-VEGF agents has yet to be fully determined.

■Provide an Amsler grid and instructions.

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Acute multifocal posterior placoid pigment epitheliopathy (AMPPE)

Acute Multifocal Posterior Placoid

Pigment Epitheliopathy (AMPPE)

■Primarily affects young adults of either sex.

■Causes acute, usually bilateral, blurred vision.

■One-third of cases are preceded by a viral prodrome.

■Cream-coloured, ill-defined lesions occur at the level of the choriocapillaris or RPE, most commonly at the posterior pole (see p. 424).

■A mild vitritis occurs in 50% and anterior chamber cells in a minority.

■Severe hypertension may produce choroidal infarcts that mimic AMPPE so check BP.

■Fluorescein angiography shows early blockage of choroidal fluorescence with late staining. ICG angiography shows areas of choriocapillary hypoperfusion (p. 424).

■The disease is usually self-limiting but may recur.

■The benefit of systemic steroids is unproven but they are often given if VA is reduced at presentation.

■Ask about severe headaches as these suggest cerebral vasculitis, which may be fatal. If present, refer promptly to a neurologist.

Birdshot Chorioretinopathy

■Usually occurs in HLA-A29-positive Caucasians aged 40–70 years.

■Gradual bilateral onset.

■Oval, cream-coloured, ill-defined lesions occur around the posterior pole and midperiphery (Fig. 8.12); these may take years to appear. Choroidal neovascularization may occur.

■Often associated with moderate vitritis, vasculitis, cystoid macular oedema, and disc oedema.

■A chronic progressive course usually requires systemic steroids and second-line agents for visual loss from cystoid macular oedema.

■Loss of retinal function (ERG) precedes VA change and dictates therapy.

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Multiple evanescent white dot syndrome (MEWDS)

Multiple Evanescent White

Dot Syndrome (MEWDS)

■Typically causes acute unilateral visual loss or paracentral scotoma in young adult females, often following a flu-like illness.

■Small white spots occur at the level of the RPE or retina, mostly within and around the vascular arcades and disc, showing early hypofluorescence and late staining on fluorescein angiography. The macula has a granular appearance due to tiny punctate yellowish or orange spots (Fig. 8.13). ICG angiography typically shows multiple hypofluorescent areas in late films which are more extensive than the lesions seen clinically.

■Vitreous cells may be present, as may an RAPD, disc swelling, and blind spot enlargement.

■MEWDS resolves spontaneously over weeks to normal or near normal acuity.

■The fundus changes also resolve, and may be absent unless examined acutely. ICG angiography may, however, be abnormal for several weeks before reverting to normal.

■Recurrences are rare.

■There is no proven treatment.

Serpiginous choroidopathy

Serpiginous Choroidopathy

■Rare, usually bilateral disease mostly affecting Caucasians of either sex. Also called geographic choroidopathy.

■Presents typically as a middle-aged adult with active choroidal

inflammation adjacent to previous scars (Fig. 8.14). May present at a younger age (>10 years) .

■Has a slow stepwise progression with spread from the peripapillary area towards the periphery.

■Active lesions show early blockage with late staining on fluorescein angiography.

■Often treated with systemic steroids for vision-threatening lesions, although not of proven benefit.

■Extrafoveal choroidal neovascularization is sometimes treated with argon laser with steroid cover.