Ординатура / Офтальмология / Английские материалы / Clinical Ophthalmology A Systematic Approach 7th Edition_Kanski, Bowling_2011

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3Presentation in 65% of cases is between 2 weeks and 3 months after initial injury and 90% of all cases occur within the first year.

4Signs in chronological order:

•The exciting eye shows evidence of the initial trauma and is frequently very red and irritable (Fig. 11.73A).

•The sympathizing eye then develops irritation, blurred vision, photophobia and loss of accommodation.

•Both eyes develop anterior uveitis which may be mild or severe and is usually granulomatous. Because the severity of inflammation may be asymmetrical, mild involvement in one eye may be missed.

•Multifocal choroidal infiltrates in the midperiphery (Fig. 11.73B).

•Sub-RPE infiltrates corresponding to Dalen–Fuchs nodules seen on histology.

•Exudative retinal detachment may occur in severe cases.

•Residual chorioretinal scarring may cause visual loss when involving the macula.

•‘Sunset glow appearance similar to VKH (see Fig. 11.71).

5 FA shows multiple foci of leakage at the level of RPE, with subretinal pooling in the presence of exudative retinal detachment. 6 ICGA shows hypofluorescent spots in active disease which resolve with treatment.

7Ultrasound may show choroidal thickening and retinal detachment.

8 Systemic manifestations are the same as in VKH but are less common.

9Treatment

aEnucleation within first 10 days following trauma should be considered only in eyes with a hopeless visual prognosis because the exciting eye may eventually have better vision than the sympathizing eye. Evisceration does not seem to protect against SO.

bTopical treatment of the anterior uveitis with steroids and cycloplegics is given but the inflammation tends to be resistant to this form of therapy (diagnostic clue).

cSystemic steroids are usually effective although ciclosporin or azathioprine may be required in resistant cases. Treatment is often required for at least a year with gradual tapering of the dose to reduce the risk of relapse. With aggressive therapy 75% of sympathizing eyes retain a visual acuity of better than 6/60. Long-term follow-up is mandatory because relapses occur in 50% of cases, and may be delayed for several years.

Fig. 11.72 Histology of sympathetic ophthalmitis. (A) Infiltration of the choroid by lymphocytes and scattered aggregations of epithelioid cells, many of which contain fine granules of melanin; (B) Dalen–Fuchs nodule – a granuloma situated between Bruch membrane and the retinal pigment epithelium

(Courtesy of J Harry)

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Fig. 11.73 Sympathetic ophthalmitis. (A) The exciting eye; (B) multifocal choroidal infiltrates

(Courtesy of W Wykes – fig. A)

Birdshot retinochoroidopathy

1Definition. Birdshot retinochoroidopathy is an uncommon idiopathic chronic/recurrent bilateral disease which predominantly affects females. Over 95% of patients are positive for HLA-A29. The disease involves both the choroid and retina independently, in contrast to the other conditions in this group.

2Presentation is in the 3rd–6th decades with insidious impairment of central vision associated with photopsia and floaters. Nyctalopia and impairment of colour vision occur later.

3Signs

•Multiple ill-defined cream-coloured choroidal patches, less than one disc diameter in size, in the posterior pole and midperiphery.

•The lesions often appear to radiate outward from the disc but usually spare the macula itself (Fig. 11.74A).

•Many of the lesions are ovoid although some are elongated or have an irregular shape.

•Over several years new spots may appear and old lesions may enlarge.

•Inactive lesions consist of well-delineated atrophic spots, which show no tendency to become hyperpigmented (Fig. 11.74B).

•Other features include vitritis and vasculitis.

•Complications include CMO, macular pucker and CNV.

4 FA shows disc staining, hyperfluorescence due to leakage and CMO (Fig. 11.74C).

5ICGA reveals, well-defined oval hypofluorescent spots during the intermediate phase (Fig. 11.74D), most of which become isofluorescent in the late phase. Many more spots can be seen by ICGA than clinically.

6ERG is normal in early disease but with time the b-wave amplitude and then the oscillatory potentials become decreased. A delay in implicit time of the 30 Hz flicker ERG is the most sensitive change. ERG findings seem to reflect intraretinal oedema and for this reason correlate well with the severity of retinal vasculopathy rather than choroidal involvement.

7Treatment. Although a good response may be achieved with systemic steroids optimal treatment may involve a steroid-sparing agent, such as ciclosporin or azathioprine. Periocular steroids may be useful for CMO.

8Prognosis. About 20% of patients have a self-limited course and maintain normal visual acuity at least in one eye. The majority have variable visual impairment in one or both eyes.

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Fig. 11.74 Birdshot chorioretinopathy. (A) Active stage; (B) inactive lesions; (C) late phase fluorescein angiogramshows disc hyperfluorescence and cystoid macular oedema; (D) early phase indocyanine green angiogramshows numerous hypofluorescent lesions

(Courtesy of C Pavésio – fig. B; P Gili – fig. C)

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Miscellaneous anterior uveitis

Fuchs uveitis syndrome

1Definition. Fuchs uveitis syndrome (FUS) or Fuchs heterochromic iridocyclitis is a chronic non-granulomatous specific uveitis entity which is usually unilateral. It has an insidious onset, occurs mostly in the 3rd–4th decades and affects both sexes equally. Although FUS accounts for about 4% of all cases of uveitis, it is frequently misdiagnosed and over-treated. The heterochromia (difference in iris colour between the two eyes) may be absent or difficult to detect, particularly in brown-eyed individuals. The diagnosis is based mainly on ocular signs, which in early cases may be subtle and easily overlooked.

2Presentation

•Chronic, annoying vitreous floaters are often the presenting symptom.

•Gradual blurring of vision secondary to cataract formation is common.

•Colour difference between the two eyes.

•Incidental detection.

3General signs

•Absence of posterior synechiae, except following cataract surgery.

•KP are characteristically small, round or stellate and grey-white in colour. They are scattered throughout the corneal endothelium and are frequently associated with feathery fibrin filaments (Fig. 11.75A).

•Small nodules on the pupillary border and stroma (Fig. 11.75B) are seen in 30% of cases.

•Aqueous humour shows faint flare and mild cellular reaction.

•Vitritis and stringy opacities may be dense enough to reduce vision.

4Diffuse iris atrophy

•The earliest finding is loss of iris crypts.

•Advanced stromal atrophy makes the affected iris appear dull with loss of detail particularly in the pupillary zone where the sphincter pupillae becomes prominent (Fig. 11.75C). The normal radial iris blood vessels appear prominent due to lack of stromal support.

•Posterior pigment layer iris atrophy is best detected by retroillumination (Fig. 11.75D).

5Heterochromia iridis is an important and common sign; it is demonstrated most effectively in daylight.

•The nature of heterochromia is determined by the relative degrees of atrophy of the stroma and posterior pigment epithelium, as well as the patient's natural iris colour.

•It is easily seen in green eyes, but if the iris is blue or deep brown, heterochromia may be more difficult to detect.

•Most frequently the affected eye is hypochromic (Fig. 11.75E).

•In blue eyes, predominant stromal atrophy allows the posterior pigmented layer to show through and become the dominant pigmentation, so that the eye may become hyperchromic (reverse heterochromia).

6Gonioscopy may be normal or may show one of the following:

•Fine radial twig-like vessels in the angle (Fig. 11.75F) which are responsible for the filiform haemorrhages which develop on anterior chamber paracentesis (Amsler sign).

•Small, non-confluent, irregular peripheral anterior synechiae.

7Systemic association. Parry–Romberg syndrome (hemifacial atrophy) is present in a small minority of cases.

8Complications

aCataract is extremely common and is often the presenting feature (see Fig. 11.75E). It does not differ from that associated with other types of anterior uveitis. The results of surgery with posterior chamber intraocular lens implantation are good.

bGlaucoma is a late manifestation which typically develops only after several years of follow-up. It is usually well-controlled on topical therapy, but some patients may require surgery.

9Treatment is only indicated in patients with troublesome vitreous opacities.

aPosterior sub-Tenon injections of a long-acting steroid preparation such as triamcinolone acetonide may be beneficial although improvement is usually temporary.

b Vitrectomy may be considered for severe vitreous opacification that is reducing vision or is very disturbing.

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Fig. 11.75 Fuchs uveitis syndrome. (A) Keratic precipitates; (B) small nodules at the pupil border and in the stroma; (C) stromal iris atrophy rendering the sphincter pupillae prominent, and small pupil border nodules; (D) posterior pigment layer atrophy seen on retroillumination; (E) heterochromia and left cataract; (F) angle vessels and small peripheral anterior synechiae

(Courtesy of C Pavésio – fig. A)

Topical steroids are generally ineffective and mydriatics unnecessary because of lack of posterior synechiae.

Table 11.8 -- Other causes of heterochromia iridis

1Hypochromic

•Idiopathic congenital

•Horner syndrome, particularly if congenital

•Waardenburg syndrome

2Hyperchromic

•Unilateral use of a topical prostaglandin analogue for glaucoma

•Oculodermal melanocytosis (naevus of Ota)

•Ocular siderosis

•Diffuse iris naevus or melanoma

•Sturge–Weber syndrome

Lens-induced uveitis

Lens-induced uveitis is triggered by an immune response to lens proteins following rupture of the lens capsule, which may be due to trauma or incomplete cataract extraction (Fig. 11.76).

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Fig. 11.76 Lens-induced uveitis. (A) Escaping lens material producing an inflammatory reaction; (B) giant cells in relation to extracapsular material

(Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann, 2001)

Phacoanaphylactic endophthalmitis

1Presentation is with abrupt reduction in visual acuity and pain, which is less severe than in bacterial endophthalmitis, days to weeks after rupture of the lens capsule.

2Signs

•Anterior uveitis is granulomatous and of variable severity.

•The IOP is frequently high.

•The posterior segment is not involved.

3 Differential diagnosis is from bacterial endophthalmitis; in doubtful cases vitreous tap may be required.

4Treatment involves removal of all lens material in conjunction with intensive steroid therapy.

Phacogenic non-granulomatous uveitis

1Signs. Anterior uveitis is less severe and more chronic than in phacoanaphylactic endophthalmitis, and develops within 2–3 weeks of lens capsule rupture.

2Differential diagnosis includes low-grade bacterial and fungal endophthalmitis, SO and IOL-induced inflammation.

3Treatment of mild cases is with topical steroids but periocular and systemic therapy will be necessary for more intense inflammation. Removal of remaining lens material may also be necessary.

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Miscellaneous posterior uveitis

Acute retinal pigment epitheliitis

Acute retinal pigment epitheliitis (Krill disease) is a rare, idiopathic, self-limiting condition of the RPE that is unilateral in 75% of cases.

1Presentation is in the 3rd decade with sudden mild disturbance of central vision.

2Signs

•The macula shows 2–4 discrete clusters of subtle small grey spots at the level of the RPE surrounded by hypopigmented yellow halos (Fig. 11.77A).

•These lesions tend to appear 1–2 weeks after the onset of symptoms.

•After 6–12 weeks the lesions resolve and vision returns to normal.

•Recurrences are uncommon.

3 FA may be normal or the halos may show hyperfluorescence without leakage (Fig. 11.77B).

4EOG is subnormal.

5Treatment is not required.

Fig. 11.77 (A) Acute retinal pigment epitheliitis; (B) FA venous phase shows corresponding focal hyperfluorescence

(Courtesy of M Prost)

Acute idiopathic maculopathy

Acute idiopathic maculopathy is a very rare, self-limiting condition which is most frequently unilateral and may be preceded by a flu-like illness.

1 Presentation is in the 2nd–4th decades with a unilateral sudden and severe loss of central vision.

2Signs

•Detachment of the sensory retina at the macula with an irregular outline (Fig. 11.78A); intraretinal haemorrhages may be

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seen in the detached area.

•Smaller, greyish subretinal thickening at the level of the RPE beneath the detachment is frequently present.

•Iritis, papillitis and mild vitritis may be present.

•Within a few weeks the exudative changes resolve.

•A bull's eye appearance may develop following resolution and may be associated with visual loss.

3FA

•Early phase shows irregular mild hyperfluorescence beneath the sensory retinal detachment (Fig. 11.78B).

•Mid-venous (Fig. 11.78C) and late venous (Fig. 11.78D) phases show intense staining of the subretinal fluid.

4Treatment is not required.

Fig. 11.78 Acute idiopathic maculopathy. (A) Detachment of the sensory retina at the macula with an irregular outline; (B) FA early phase shows mild irregular subretinal hyperfluorescence; (C) mid-venous phase shows extensive hyperfluorescence due to staining of the subretinal fluid; (D) late venous phase shows further staining of subretinal fluid

(Courtesy of S Milewski)

Acute multifocal retinitis

Acute multifocal retinitis is a very rare, frequently bilateral, self-limiting condition that typically affects healthy individuals. It may be preceded by a flu-like illness, usually 1–2 weeks before the onset of the visual symptoms. It has been postulated by some that acute multifocal retinitis may be an unusual presentation of cat-scratch disease.

1 Presentation is in the 3rd–4th decades with sudden onset of mild visual loss.

2Signs

•Multiple areas of retinitis posterior to the equator (Fig. 11.79) in both eyes.

•Mild vitritis and disc oedema are frequent.

•A macular star is present in a few cases.

•Small retinal branch artery occlusions occur in a minority.

•After 2–4 months the fundus lesions resolve and vision recovers.

3 Treatment is not required.

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Fig. 11.79 Acute multifocal retinitis

(Courtesy of S Milewski)

Solitary idiopathic choroiditis

Solitary idiopathic choroiditis is a distinct clinical entity that may give rise to diagnostic problems as it can simulate other pathology.

1Presentation is with mild visual loss and floaters.

2Signs

•Vitritis is present during active disease.

•Discrete, post-equatorial, dull-yellow choroidal elevation with ill-defined margins.

•Associated findings include adjacent subretinal fluid and a macular star away from the main lesion.

•As the inflammation resolves the lesion develops a better-defined margin with resolution of subretinal fluid and exudation.

3Treatment of active, vision-threatening lesions is with systemic steroids. Most inactive lesions either remain stable or resolve without treatment.

4Differential diagnosis includes inflammatory lesions such as sarcoidosis, tuberculosis (see Fig. 11.51B), nodular posterior scleritis and syphilis, and amelanotic tumours such as amelanotic melanoma and metastasis.

Frosted branch angiitis

Frosted branch angiitis (FBA) describes a characteristic fundus picture, usually bilateral, which may represent a specific syndrome (primary form) or a common immune pathway in response to multiple infective agents. Secondary FBA may be associated with infectious retinitis, most notably cytomegalovirus retinitis, but it may also occur in association with other conditions such as glomerulonephritis and central retinal vein occlusion. Primary FBA is rare and typically affects children and young adults.

1 Presentation is with subacute bilateral visual loss, floaters and/or photopsia.

2Signs

•Visual acuity is usually very poor.

•Florid translucent retinal perivascular sheathing of both arterioles and venules (Fig. 11.80A).

•Anterior uveitis, vitritis and retinal oedema are common.

•Uncommon findings include papillitis, hard exudates, retinal haemorrhages and venous occlusion (Fig. 11.80B).

3Treatment is with systemic and topical steroids, but the optimal regimen has not been established. The primary form has a good visual prognosis but significant visual loss may result in secondary forms.

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Fig. 11.80 Frosted branch angiitis. (A) Perivascular sheathing; (B) secondary venous occlusion

(Courtesy of J Donald Gass, from Stereoscopic Atlas of Macular Diseases, Mosby 1997 – fig. A; C Barry – fig. B)

Idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome

Idiopathic retinal vasculitis, aneurysms and neuroretinitis syndrome is a rare entity that typically affects one or both eyes of healthy young women.

1Signs

•Anterior uveitis and vitritis.

•Multiple, leaking, tied-knot-like aneurysmal dilatations along the retinal arteriolar tree and optic nerve head that give rise to marked circumpapillary intraretinal lipid deposition (Fig. 11.81A).

•The aneurysms may increase in number although some may spontaneously regress.

•Disc oedema and a macular star.

•Irregular venous dilatation and vascular sheathing.

2FA shows multiple aneurysms at arterial bifurcations and marked variation of arteriolar calibre (Fig. 11.81B). Extensive peripheral capillary non-perfusion is also seen.

3Treatment by laser photocoagulation may be beneficial in eyes with extensive peripheral ischaemia and retinal neovascularization.

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