Chapter 17 Acquired Macular Disorders

Age-related macular degeneration 334 Polypoidal choroidal vasculopathy 341 Age-related macular hole 342 Central serous retinopathy 344 Cystoid macular oedema 345 Macular epiretinal membrane 347 Degenerative myopia 348 Angioid streaks FINAL 350 Choroidal folds 351 NOT Hypotony maculopathy - 351 Vitreomacular traction syndrome ELSEVIER 352 Solar maculopathy OF 352 Idiopathic choroidal neovascularization 352 CONTENT

PROPERTY SAMPLE

334 Acquired Macular Disorders

Age-related macular degeneration Drusen 1. Histopathology (Fig. 17.1) • Discrete deposits of the abnormal material located between the basal lamina of the RPE and the inner collagenous layer of Bruch membrane. • Thickening of Bruch membrane Fig. 17.2 Hard drusen compounded by excessive production of basement mem- brane deposit by the RPE. a

FINAL Atrophy and depigmentation NOT - RPE ELSEVIER OF

Bruch membrane Drusen Thickening

CONTENTb Fig. 17.1 Histopathology of drusen

2. Signs – yellow sub-RPEPROPERTY excrescences distributed symmetricallySAMPLE at both posterior poles. a. Small hard drusen – less than half a vein width in diameter with discrete margins (Fig. 17.2). b. Large soft drusen – vein width or more in diameter with indistinct margins (Fig. 17.3a). Fig. 17.3 (a) Soft drusen; (b) FA c. Calcifi ed drusen – may be hard or soft (Fig. 17.4).

Age-related macular degeneration 335

a

Fig. 17.4 Calcifi ed drusen b

3. FA a. Hyperfl uorescence – window defect due to atrophy of the overlying RPE and late staining (see FINAL Fig. 17.3b). b. Hypofl uorescence – of hydrophobic NOT (high lipid content) drusen. - 4. Drusen and AMD – features ELSEVIER associated with an increased risk of subsequent visual loss include largeOF soft and/or confl uent drusen, and Fig. 17.5 (a) Confl uent soft drusen with RPE changes; (b) FA focal RPE hyperpigmentation (Fig.CONTENT 17.5), particularly if the other eye has AMD. 3. Signs in chronological order: 5. Prophylactic treatment – high-dose PROPERTY • Focal hyperpigmentation or multivitamins and antioxidants can atrophy of the RPE associated decrease the risk ofSAMPLE progression of with drusen. AMD in eyes with the following high • Sharply circumscribed, areas of risk characteristics: visual loss in the RPE atrophy associated with contralateral eye from pre-existing dry variable loss of the choriocapil- or wet AMD, and confl uent soft drusen laris (Fig. 17.6). even in the absence of visual loss. • Enlargement of the atrophic areas (Fig. 17.7). Atrophic (dry) age-related 4. FA – hyperfl uorescence due to an macular degeneration RPE window defect (Fig. 17.8). 5. Treatment – not possible. 1. Pathogenesis – slowly progressive atrophy of the photoreceptors, RPE, and choriocapillaris. 2. Presentation – gradual impairment of vision over months or years.

336 Acquired Macular Disorders

a

Fig. 17.6 Dry AMD b

FINAL

NOT - ELSEVIER OF

Fig. 17.9 (a) PED; (b) ICG Fig. 17.7 Geographic atrophy CONTENT

a PROPERTY b SAMPLE

Fig. 17.8 Window defect in dry AMD

Age-related macular degeneration 337

Retinal pigment epithelial 6. FA – well demarcated oval area of detachment hyperfl uorescence which increases in density but not in size (Fig. 17.11). 1. Pathogenesis – reduction of 7. Course – may follow one of the hydraulic conductivity of the following patterns: thickened Bruch membrane impeding a. Spontaneous resolution – without movement of fl uid from the RPE residua. towards the choroid. b. Geographic atrophy – following 2. Presentation – metamorphopsia and spontaneous resolution. impairment of central vision. c. Detachment of the sensory retina. 3. Signs – circumscribed, dome-shaped d. RPE tear formation. elevation at the posterior pole (Fig. 17.9a). Retinal pigment epithelial tear 4. ICG – oval hypofl uorescence with a faint ring of surrounding hyperfl uores- 1. Pathogenesis – tearing of the RPE at cence (Fig. 17.9b). the junction of attached and 5. OCT – separation of the RPE from detached RPE due to tangential Bruch membrane (Fig. 17.10). stress – mayFINAL be spontaneous, or more commonly, follows laser photocoagulation, PDT or anti-VEGF therapyNOT of CNV. - ELSEVIER2. Presentation – sudden worsening of central vision. OF 3. Signs – crescent shaped RPE dehiscence with a retracted and folded fl ap (Fig. 17.12a). CONTENT4. FA – relative hypofl uorescence over Fig. 17.10 OCT of PED the fl ap with adjacent hyperfl uores- cence due to the exposed choriocap- PROPERTY illaris (Fig. 17.12b–d). SAMPLE a b

Fig. 17.11 FA of PED

338 Acquired Macular Disorders

a b c d

e f g h

Fig. 17.12 RPE tear

5. OCT – loss of the normal dome- shaped profi le of the RPE in the FINAL PED, with hyper-refl ectivity adjacent to the folded RPE (Fig. 17.12e–h). NOT 6. Prognosis – poor for subfoveal tears. - ELSEVIER Neovascular (wet) age-related macular degeneration OF Pathogenesis Fig. 17.13 CNV in wet AMD • CNV originating from the choriocapil-CONTENT laris grows through defects in Bruch membrane (Fig. 17.13). Fluorescein angiography • Initial visual loss isPROPERTY caused by 1. Classic CNV leakage from CNV underSAMPLE the sensory • Well-defi ned membrane which fi lls retina and under the RPE. with dye in a ‘lacy’ pattern during • This is followed by bleeding from the very early phase of dye transit CNV. (Fig. 17.14b), fl uoresces brightly • Permanent visual loss is caused by during peak dye transit (Fig. subretinal (disciform) scarring. 17.14c), and then leaks into the Clinical features subretinal space and around the CNV within 1–2 min. 1. Presentation – metamorphopsia, • Late staining of fi brous tissue positive scotoma, and blurring of within the CNV (Fig. 17.14d). central vision. • Classic CNV is classifi ed 2. Signs – serous retinal elevation, according to its relation to the foveal thickening, CMO, subretinal centre of FAZ as extrafoveal, haemorrhage, and hard exudate subfoveal, and juxtafoveal. formation (Fig. 17.14a).

Age-related macular degeneration 339

• CNV can be further subdivided Course into wholly classic and predomi- The course of untreated CNV is often nantly classic in which 50% or relentless and the prognosis very poor less of the lesion has a classic due to the following complications. component. 1. Haemorrhagic PED 2. Occult CNV – poorly defi ned with • Initially, the blood is confi ned to less precise features on the early the sub-RPE space and appears as frames (Fig. 17.15b) and gives rise a dark elevated mound (Fig. 17.16). to late, diffuse or multifocal leakage • The blood may then break into the (Fig. 17.15c,d). subretinal space and assumes a 3. Fibrovascular PED – combination of more diffuse outline and a lighter CNV and PED in which the CNV red colour (Fig. 17.17). fl uoresces brighter (hot spot) than 2. Vitreous haemorrhage – when the detachment; in other cases, the subretinal blood breaks through into CNV may be obscured by blood or the vitreous cavity; rare. turbid fl uid. 3. Subretinal (disciform) scarring – causes permanent loss of central vision (Fig.FINAL 17.18).

NOT - a ELSEVIERb OF

CONTENT

PROPERTY SAMPLE

c d

Fig. 17.14 FA of classic subfoveal CNV

340 Acquired Macular Disorders

a b

c d

FINAL

NOT - ELSEVIER OF

Fig. 17.15 FA of occult CNV CONTENT

PROPERTY SAMPLE

Fig. 17.16 Sub-RPE haemorrhage Fig. 17.17 Subretinal haemorrhage

Polypoidal choroidal vasculopathy 341

Retinal angiomatous proliferation 1. Defi nition – uncommon type of wet AMD in which neovascularization originates from the retinal vascula- ture and not the choriocapillaris. 2. Signs • Intraretinal and subretinal neovascularization often accompa- nied by haemorrhage and oedema. • CNV associated with fi brovascular Fig. 17.18 Disciform scar PED and retinochoroidal anasto- moses. 3. FA – similar to purely occult or 4. Massive subretinal exudation – due minimally classic CNV. to chronic leakage from CNV 4. ICG – hot FINALspot in mid or late frames. (Fig. 17.19). 5. Treatment – PDT with adjunctive intravitreal triamcinolone. NOT - ELSEVIERPolypoidal choroidal OF vasculopathy 1. Defi nition – bilateral, choroidal CONTENTvascular disease in which the inner choroidal vessels consist of a dilated network with multiple terminal PROPERTY aneurysmal protuberances that have a polypoidal confi guration. SAMPLE 2. Signs a. Exudative – multiple PED, serous Fig. 17.19 Massive exudation RD, and lipid deposits (Fig. 17.20a). b. Haemorrhagic – haemorrhagic PED Treatment and subretinal haemorrhage (Fig. 17.21a). 1. Photodynamic therapy (PDT) – for 3. ICG – polypoidal dilatations beneath subfoveal, predominantly classic CNV the RPE that fi ll slowly and then leak not larger than 5400 µm in eyes with intensely (Figs 17.20b & 17.21b). a visual acuity of 6/60 or better. 4. Course 2. Anti-vascular endothelium growth • Spontaneous resolution in 50%. factor (anti-VEGF) agents – • In the remainder occasional intravitreal bevacizumab (Avastin), repeated bleeding and leakage, ranibizumab (Lucentis) and pegap- resulting in macular damage and tanib (Macugen) are used to treat visual loss. any type of CNV. 5. Treatment – PDT.

342 Acquired Macular Disorders

a a

b b

FINAL

NOT - ELSEVIER OF Fig. 17.20 (a) Exudative polypoidal; Fig. 17.21 (a) Haemorrhagic polypoi- (b) ICG dal; (b) ICG CONTENT

Age-related macular hole • Stage 2 (early hole) – full- PROPERTY thickness defect, less than 1. Pathogenesis – abnormal vitreo- SAMPLE 300 µm in diameter with or without foveolar attachment, with resultant an overlying pseudo-operculum. antero-posterior and tangential • Stage 3 (established hole) – full- traction. thickness defect more than 2. Presentation – in old age. 400 µm in diameter with an 3. Staging (Fig. 17.22) attached posterior vitreous face • Stage 1a (impending) – fl at umbo, with or without an overlying yellow foveolar spot 100–200 µm pseudo-operculum. in diameter with loss of the • Stage 4 – round defect more than foveolar refl ex. 400 µm in diameter surrounded • Stage 1b (occult) – yellow ring with by a cuff of subretinal fl uid and a bridging interface of vitreous tiny yellowish deposits within its cortex. crater, and completely detached vitreous cortex (Fig. 17.23).

Age-related macular hole 343

normal fovea

Fig. 17.23 Full-thickness macular hole stage 1-a. impending hole 4. OCT – useful in the diagnosis and staging of macular holes (Fig. 17.24).

FINAL

NOT - stage 1-b. occult hole ELSEVIER OF

Fig. 17.24 Macular hole with CONTENToperculum

PROPERTY 5. FA – corresponding area of hyper- stage 2. hole fl uorescence. SAMPLE 6. Surgery (Fig. 17.25) • Indicated for stage 2 and above, associated with a visual acuity worse than 6/9. • Following successful surgery, visual improvement is achieved in 80–90% of eyes, with a fi nal visual acuity of 6/12 or better in stage 3. hole up to 65%.

stage 4. hole Fig. 17.22 Staging of macular hole

344 Acquired Macular Disorders

2. Presentation – unilateral relative positive scotoma, micropsia, metamorphopsia, and occasionally macropsia. 3. Signs – round detachment of the sensory retina at the macula that may be associated with small precipitates on its posterior surface (Fig. 17.26)

FINAL

NOT Fig. 17.25 Unsuccessful surgery for - macular hole. Pre-operative appearance ELSEVIER (above); postoperative appearance (below) OF Fig. 17.26 CSR

Macular microhole CONTENT4. OCT – elevation of the sensory 1. Presentation – central scotoma or retinal layer from the highly refl ective reduced reading vision. RPE layer by an optically empty zone 2. Signs – very small,PROPERTY red, well (Fig. 17.27) demarcated intraretinalSAMPLE foveal or juxtafoveal defect that remains stationary with long-term follow-up. 3. OCT – well localized subtle defect that probably indicates the presence of a gap in the photoreceptors, and/ or the RPE.

Central serous Fig. 17.27 OCT of CSR retinopathy

1. Pathogenesis – localized detach- 5. FA ment of the sensory retina at the • Small hyperfl uorescent spot that macula secondary to focal RPE enlarges and ascends vertically defects. and laterally until the entire area

Cystoid macular oedema 345

Cystoid macular oedema

1. Defi nition • Accumulation of fl uid in the outer plexiform and inner nuclear layers with the formation of fl uid-fi lled cyst-like changes (Fig. 17.29a). • Lamellar hole formation in longstanding cases (Fig. 17.29b).

Fig. 17.28 FA of CSR a

is fi lled with dye (smoke stack appearance – Fig. 17.28) • Less frequently the hyperfl uores- cent spot enlarges centrifugally FINAL (ink-spot appearance). b 6. ICG – early phase shows dilated NOT choroidal vessels, mid phases show - multiple areas of hyperfl uorescence ELSEVIER due to choroidal hyperpermeability. 7. Course OF • Spontaneous resolution within Fig. 17.29 (a) CMO; (b) lamellar hole 3–12 months is the rule. • Occasionally the course is CONTENT protracted and results in 2. Signs – loss of the foveal progressive widespread RPE depression, retinal thickening, and changes (chronicPROPERTY retinal pigment multiple cystoid areas in the epitheliopathy). SAMPLE sensory retina (Fig. 17.30). 8. Treatment a. Not required – in majority. b. Argon laser photocoagulation – in eyes with extrafoveal leaks achieves speedier resolution and lowers the recurrence rate but does not infl uence the fi nal visual outcome. c. PDT – in acute CSR with subfoveal leaks and in chronic disease.

346 Acquired Macular Disorders

Fig. 17.30 CMO

3. OCT – collection of hyporefl ective Fig. 17.32 FA of CMO spaces within the retina, with overall macular thickening and FINAL loss of the foveal depression 6. Infl ammatory causes (Fig. 17.31). • Chronic anterior uveitis, NOT -intermediate uveitis, and certain ELSEVIERforms of posterior uveitis. • Treatment – control of uveitis OF with anti-infl ammatory agents; systemic carbonic anhydrase inhibitors may be benefi cial in CONTENT CMO associated with intermedi- ate uveitis. 7. Following cataract surgery Fig. 17.31 OCT of CMOPROPERTY • Risk factors – posterior capsular SAMPLE rupture, vitreous loss and incarceration into the incision 4. FA – late phase shows a ‘fl ower- site, anterior chamber and petal’ pattern of hyperfl uorescence secondary IOL implantation, (Fig. 17.32). diabetes, and CMO in the other 5. Vascular causes eye. • Diabetic retinopathy, retinal vein • Treatment – correction of the occlusion, hypertensive underlying cause, if possible; retinopathy, idiopathic retinal persistent cases may require telangiectasis, retinal artery systemic carbonic anhydrase macroaneurysm, and radiation inhibitors, topical and periocular retinopathy. steroids, topical NSAIDs, • Treatment – laser photocoagula- intravitreal triamcinolone, and tion in selected cases. pars plana vitrectomy.

Macular epiretinal membrane 347

8. Drug-induced – topical adrenaline • The membrane is translucent and 2% (especially in the aphakic eye), best detected with ‘red-free’ light topical latanoprost, and systemic (Fig. 17.33). nicotinic acid. 9. Retinal dystrophies • RP, gyrate atrophy, and dominantly inherited CMO. • Treatment – systemic carbonic anhydrase inhibitors in RP. 10. Vitreomacular traction syndrome (see below). 11. Macular epiretinal membranes (see below). 12. CNV – CMO is an adverse prognostic factor. 13. Tumours – retinal haemangioblas- toma and choroidal haemangioma. FINAL Fig. 17.33 Cellophane maculopathy Macular epiretinal 3. TreatmentNOT – not appropriate. membrane - ELSEVIER Pathogenesis Macular pucker OF • Proliferation of retinal glial cells at 1. Presentation – metamorphopsia and the vitreoretinal interface that have blurring of central vision. gained access to the retinal surfaceCONTENT 2. VA – 6/12 or worse. through breaks in the internal limiting 3. Signs membrane. • Severe vascular distortion, retinal wrinkling and white striae • May be idiopathicPROPERTY or secondary to RD surgery and cryotherapy, retinal (Fig. 17.34). vascular disease, intraocularSAMPLE • Macular pseudo-hole (Fig. 17.35) infl ammation, and trauma. and occasionally CMO.

Cellophane maculopathy 1. Presentation – mild metamorphopsia although frequently the condition is asymptomatic and is discovered by chance. 2. Signs • Irregular light refl ex or sheen at the macula.

Fig. 17.34 Macular pucker

348 Acquired Macular Disorders

Fig. 17.35 Macular pseudohole

Fig. 17.37 FA of macular pucker 4. OCT – highly refl ective (red) layer on the retinal surface associated with FINAL thickening (Fig. 17.36). Degenerative myopia NOT 1. Defi -nition ELSEVIER• Refractive error > −6 D and axial length >26 mm. OF • Affects approximately 0.5% of the general population and 30% of myopic eyes. CONTENT2. Signs • Pale tessellate (tigroid) fundus with visibility of large choroidal PROPERTY vessels (Fig. 17.38). Fig. 17.36 OCT of macular pucker SAMPLE

5. FA – highlights the vascular tortuosity and may show hyperfl uo- rescence if leakage is present (Fig. 17.37). 6. Treatment – removal of the membrane improves or eliminates distortion, and improves visual acuity in about 50% of cases.

Fig. 17.38 Tessellate fundus

Degenerative myopia 349

• ‘Lacquer cracks’ consist of ruptures in the RPE–Bruch membrane–choriocapillaris complex (Fig. 17.39). • Focal chorioretinal atrophy with visibility of the sclera (Fig. 17.40). • Staphylomas due to expansion of the globe and scleral thinning.

Fig. 17.41 Coin haemorrhage

FINAL

NOT - ELSEVIER Fig. 17.39 Lacquer cracks OF

CONTENTFig. 17.42 Fuchs spot

4. FA – fi lling of large choroidal vessels PROPERTY but not of the choriocapillaris (Fig. 17.43). SAMPLE

Fig. 17.40 Chorioretinal atrophy

3. Maculopathy • CNV associated with ‘lacquer cracks’. • Subretinal ‘coin’ haemorrhages from lacquer cracks without CNV (Fig. 17.41). • Fuchs spot – pigmented lesion Fig. 17.43 FA in high myopia after absorption of a macular haemorrhage (Fig. 17.42).

350 Acquired Macular Disorders

5. Ocular complications • Rhegmatogenous RD. • Cataract, which may be posterior subcapsular or early-onset nuclear sclerosis. • Increased prevalence of primary open-angle glaucoma, pigmentary glaucoma, and steroid responsiveness. 6. Systemic associations • Stickler syndrome. • Marfan syndrome. • Ehlers–Danlos syndrome. Fig. 17.45 Angioid streaks with RPE • Pierre–Robin syndrome. hyperplasia

Angioid streaks 3. FA – hyperfl uorescence over the streaks associated with variable 1. Defi nition – crack-like dehiscences in hypofl uorescenceFINAL corresponding to thickened, calcifi ed and abnormally RPE hyperplasia. brittle collagenous and elastic 4. PrognosisNOT – visual loss in 70% of portions of Bruch membrane. cases- due to CNV (Fig. 17.46), 2. Signs ELSEVIERtraumatic choroidal rupture, or foveal • Mottled pigmentation (‘peau OF involvement by a streak. d’orange’). 5. Systemic associations – in 50% of • Grey or dark-red linear lesions patients: with irregular serrated edges CONTENT intercommunicate around the optic disc and then radiate outwards (Fig. 17.44). • Associated RPEPROPERTY hyperplasia in longstanding casesSAMPLE (Fig. 17.45 ).

Fig. 17.46 FA in angioid streaks with CNV Fig. 17.44 Angioid streaks

Hypotony maculopathy 351

a. Pseudoxanthoma elasticum – most common (Groenblad–Strandberg a syndrome). b. Ehlers–Danlos syndrome type 6 (ocular sclerotic) – occasional. c. Paget disease – uncommon. d. Haemoglobinopathies – occa- sional.

Choroidal folds

1. Defi nition – parallel grooves or striae involving the inner choroid, Bruch membrane, RPE, and sometimes the outer sensory retina. 2. Causes b a. Idiopathic – affect both eyes of healthy hypermetropic patients. FINAL b. Orbital disease – retrobulbar tumours and thyroid ophthalmo- NOT pathy. - c. Miscellaneous – choroidal ELSEVIER tumours, chronic papilloedema,OF posterior scleritis, and scleral buckle for RD. 3. Signs – horizontal parallel groovesCONTENT at the posterior pole in which the crest is yellow and less pigmented than the darker trough (Fig. 17.47a). Fig. 17.47 (a) Choroidal folds; (b) FA 4. FA – alternatingPROPERTY hyperfl uorescent and hypofl uorescent streaksSAMPLE (17.47b).

Hypotony maculopathy

1. Causes – very low IOP (usually <6 mmHg) following fi ltration surgery, particularly when adjunctive antimetabolites are used, trauma and chronic anterior uveitis. 2. Signs – irregular chorioretinal folds (Fig. 17.48). 3. Treatment – depends on the cause.

Fig. 17.48 Hypotony maculopathy

352 Acquired Macular Disorders

2. Presentation – within 1–4 hours of Vitreomacular traction solar exposure with unilateral or syndrome bilateral impairment of central vision and a small central scotoma. 1. Pathogenesis 3. Fundus • Vitreous cortex is attached to the • Small yellow or red foveolar spot fovea and the optic disc but which fades within a few weeks. detached temporal to the fovea • The spot is replaced by a sharply and the area of the papillomacular defi ned foveolar defect with bundle. irregular borders or a lamellar hole • This incomplete posterior vitreous (Fig. 17.50). detachment exerts persistent anterior traction on the fovea, which leads to macular changes, notably CMO. 2. Signs • Partial posterior vitreous detach- ment with persistent attachment FINAL of vitreous to the macula. • The macula may show retinal NOT surface wrinkling, distortion, an - epiretinal membrane or CMO. ELSEVIER 3. OCT – is used to confi rm the diagnosis (Fig. 17.49). OF

CONTENT

Fig. 17.50 Late solar maculopathy PROPERTY SAMPLE 4. Treatment – not possible. 5. Prognosis – good with improvement of vision within 6 months.

Fig. 17.49 OCT of vitreomacular traction syndrome Idiopathic choroidal neovascularization 4. Treatment – pars plana vitrectomy. Idiopathic CNV is an uncommon condi- tion which affects patients under the age Solar maculopathy of 50 years. It carries a better visual 1. Pathogenesis – retinal injury caused prognosis than that associated with AMD by photochemical effects of solar and in some cases spontaneous revolu- radiation is caused by directly or tion may occur. The CNV is of type 2 and indirectly viewing the sun (eclipse lies predominantly above the RPE. E retinopathy).

Ch017-S3135.indd 352 2/13/2009 2:22:08 PM