S Chapter Retinal vascular disease 13 RETINAL CIRCULATION 520 DIABETIC RETINOPATHY 520 Introduction 520 Pathogenesis 521 Classification 521 Signs 521 Treatment 529 Advanced diabetic eye disease 536 Diabetic papillopathy 537 NON-DIABETIC RETINOPATHY 538 RETINAL VENOUS OCCLUSIVE DISEASE 538 Introduction 538 Risk factors 538 Systemic assessment 538 Branch retinal vein occlusion 539 Impending central retinal vein occlusion 541 Non-ischaemic central retinal vein occlusion 542 Ischaemic central retinal vein occlusion 542 Hemiretinal vein occlusion 544 Treatment of the complications of CRVO 544 Systemic management in retinal vein occlusion 549 Papillophlebitis 549 RETINAL ARTERIAL OCCLUSIVE DISEASE 549 Systemic assessment 550 Amaurosis fugax 551 Branch retinal artery occlusion 551 Central retinal artery occlusion 552 Cilioretinal artery occlusion 552 Treatment of acute retinal artery occlusion 552 Systemic management following retinal arterial occlusion 555 Asymptomatic retinal embolus 556 OCULAR ISCHAEMIC SYNDROME 556 HYPERTENSIVE EYE DISEASE 557 Retinopathy 557 Choroidopathy 559 SICKLE-CELL RETINOPATHY 559 Sickling haemoglobinopathies 559 Anterior segment 560 Non-proliferative retinopathy 560 Proliferative retinopathy 560 THALASSAEMIA RETINOPATHY 561 RETINOPATHY OF PREMATURITY 561 Active disease 563 Cicatricial disease 565 RETINAL ARTERY MACROANEURYSM 565 PRIMARY RETINAL TELANGIECTASIA 569 Idiopathic macular telangiectasia 569 Coats disease 569 EALES DISEASE 569 RADIATION RETINOPATHY 572 PURTSCHER RETINOPATHY 572 VALSALVA RETINOPATHY 572 LIPAEMIA RETINALIS 574 RETINOPATHY IN BLOOD DISORDERS 574 Leukaemia 574 Anaemia 577 Hyperviscosity 577 Bowling_Chapter 13_main.indd 519 2/25/2015 6:55:25 PM To protect the rights of the author(s) and publisher we inform you that this PDF is an uncorrected proof for internal business use only by the author(s), editor(s), reviewer(s), Elsevier and typesetter Toppan Best-set. It is not allowed to publish this proof online or in print. This proof copy is the copyright property of the publisher and is confidential until formal publication.
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520 Diabetic Retinopathy
RETINAL CIRCULATION
Arterial system• The central retinal artery,anendartery,enterstheoptic
Fig. 13.1 Normal retinal capillary bed. (A) Periarteriolar capillary-free zone – flat preparation of Indian ink-injected retina; (B) endothelial cells with elongated nuclei and pericytes with rounded nuclei – trypsin digest preparation (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001)
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Retinal vascular disease 52113widelyused internationally.Anabbreviatedversion is setout in
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Table 13.1 Abbreviated Early Treatment Diabetic Retinopathy Study (ETDRS) classification of diabetic retinopathy
Category/description Management
Non-proliferative diabetic retinopathy (NPDR)
No DR Review in 12 months
Very mild NPDR Review most patients in 12 months
Microaneurysms only
Mild NPDR Review range 6–12 months, depending on severity of signs, stability, systemic factors, and patient’s personal circumstancesAny or all of: microaneurysms, retinal haemorrhages,
exudates, cotton wool spots, up to the level of moderate NPDR. No intraretinal microvascular anomalies (IRMA) or significant beading
Moderate NPDR Review in approximately 6 monthsProliferative diabetic retinopathy (PDR) in up to 26%,
high-risk PDR in up to 8% within a year• Severe retinal haemorrhages (more than ETDRS standard photograph 2A: about 20 medium–large per quadrant) in 1–3 quadrants or mild IRMA
• Significant venous beading can be present in no more than 1 quadrant
• Cotton wool spots commonly present
Severe NPDR Review in 4 monthsPDR in up to 50%, high-risk PDR in up to 15% within a
yearThe 4–2–1 rule; one or more of:• Severe haemorrhages in all 4 quadrants• Significant venous beading in 2 or more quadrants• Moderate IRMA in 1 or more quadrants
Very severe NPDR Review in 2–3 monthsHigh-risk PDR in up to 45% within a year
Two or more of the criteria for severe NPDR
Proliferative diabetic retinopathy (PDR)
Mild–moderate PDR Treatment considered according to severity of signs, stability, systemic factors, and patient’s personal circumstances such as reliability of attendance for review. If not treated, review in up to 2 months
New vessels on the disc (NVD) or new vessels elsewhere (NVE), but extent insufficient to meet the high-risk criteria
High-risk PDR Treatment advised – see textShould be performed immediately when possible, and
certainly same day if symptomatic presentation with good retinal view
• New vessels on the disc (NVD) greater than ETDRS standard photograph 10A (about 1
3 disc area)• Any NVD with vitreous haemorrhage• NVE greater than 1
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Fig. 13.2 Microaneurysms – histopathology. (A) Two arms of a capillary loop that may fuse to become a microaneurysm – flat preparation of Indian ink-injected retina; (B) an area of capillary non-perfusion and adjacent microaneurysms – flat preparation of Indian ink-injected retina; (C) eosinophilic (dark pink) degenerate pericytes – trypsin digest preparation; (D) microaneurysm with endothelial cell proliferation (cellular microaneurysm) – trypsin digest preparation (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – figs A and C; J Harry – figs B and D)
A B
C
D
Fig. 13.3 Microaneurysms. (A) Microaneurysms and dot/blot haemorrhages at the posterior pole; (B) FA shows scattered hyperfluorescent spots in the posterior fundus
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Fig. 13.4 Retinal haemorrhages. (A) Histology shows blood lying diffusely in the retinal nerve fibre and ganglion cell layers and as globules in the outer layers; (B) retinal nerve fibre layer (flame) haemorrhages; (C) dot and blot haemorrhages; (D) deep dark haemorrhages (Courtesy of J Harry and G Misson, from Clinical Ophthalmic Pathology, Butterworth-Heinemann 2001 – fig. A)
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Fig. 13.5 Exudates. (A) Histology shows irregular eosinophilic deposits mainly in the outer plexiform layer; (B) small exudates and microaneurysms; (C) more extensive exudates, some associated with microaneurysms; (D) exudates involving the fovea, including central crystalline cholesterol deposition – focal laser has recently been applied superotemporal to the fovea (Courtesy of J Harry – fig. A; S Chen – figs C and D)
A B
C D
Fig. 13.6 FA of exudates. (A) Clinical appearance; (B) exudates not shown on FA
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526 Diabetic Retinopathy
Cotton wool spotsCotton wool spots are composed of accumulations of neuronal
debris within the nerve fibre layer. They result from ischaemic
Fig. 13.7 Cystoid macular oedema. (A) OCT shows retinal thickening and cystoid spaces; (B) FA shows leaking microaneurysms and central diffuse hyperfluorescence with a flower-petal configuration – same patient as Fig. 13.6
A
B
Fig. 13.8 Focal diabetic maculopathy. (A) A ring of hard exudates temporal to the macula; (B) FA late phase shows focal area of hyperfluorescence due to leakage corresponding to the centre of the exudate ring
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Fig. 13.9 Diffuse diabetic maculopathy. (A) Dot and blot haemorrhages – diffuse retinal thickening is present; (B) late-phase FA shows extensive hyperfluorescence at the posterior pole due to leakage (Courtesy of S Chen – fig. B)
A
B
Fig. 13.10 Ischaemic diabetic maculopathy. FA venous phase shows hypofluorescence due to capillary non-perfusion at the central macula and elsewhere (Courtesy of S Chen)
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Fig. 13.12 Cotton wool spots. (A) Histology shows cytoid bodies in the retinal nerve fibre layer; (B) clinical appearance; (C) red-free photography showing differing appearance of cotton wool spots and haemorrhages, the latter appearing black – the smaller well-defined white lesions are exudates (Courtesy of J Harry – fig. A)
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Fig. 13.14 Intraretinal microvascular abnormalities. (A) Histology shows arteriolar-venular shunt and a few microaneurysms within a poorly perfused capillary bed – flat preparation of Indian ink-injected retina; phase contrast microscopy; (B) clinical appearance (Courtesy of J Harry – fig. A)
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Fig. 13.15 Disc new vessels. (A) Mild; (B) severe; (C) FA shows leaking disc vessels, with extensive peripheral capillary dropout and a small focus of leaking vessels elsewhere (Courtesy of S Chen – fig. B)
A
B
C
Fig. 13.16 New vessels elsewhere. (A) Mild; (B) severe; (C) associated with fibrosis
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