The haemorrhage originates from the superficial retinal vessels and is located between the retina and posterior vitreous face.

Usually solitary, round and frequently located at the posterior pole, where it obscures all underlying retinal landmarks ( Fig. 13.1 ).

With time the blood may settle with gravity, giving rise to the typical boat-shaped crescentic configuration ( Fig. 13.2 ).

In some cases, a large haemorrhage may break through into the vitreous cavity.

Proliferative retinopathies (e.g. diabetic, following venous occlusion, radiation, sickle cell, ocular ischaemic syndrome, retinopathy of prematurity and vasculitis).

Retinal artery macroaneurysm.

Trauma.

Subarachnoid haemorrhage (Terson syndrome).

Valsalva retinopathy caused by a severe increase in venous pressure brought on by strenuous physical exertion.

Non-accidental injury (‘shaken-baby’ syndrome).

The haemorrhage originates from the superficial pre-capillary arterioles and is located within the retinal nerve fibre layer.

Bright red, usually multiple haemorrhages spreading along the nerve fibre layer ( Fig. 13.3 ).

Retinal vein occlusion.

Hypertensive retinopathy.

Background diabetic retinopathy.

Retinal periphlebitis.

Blood dyscrasias.

Optic nerve disease – acute papilloedema, anterior ischaemic optic neuropathy and glaucoma.

Flame-shaped haemorrhages with white centres ( Fig. 13.4 ).

Severe anaemia.

Leukaemia.

Dysproteinaemia.

HIV microangiopathy.

Bacterial endocarditis.

The haemorrhage originates from the venous end of the deep retinal capillaries and is located within the sensory retina.

Dot haemorrhages are small and round, and located in the compact middle layers of the retina; they may be difficult to distinguish from microaneurysms.

Blot haemorrhages occupy the full thickness of the retina and are larger, darker and deeper than dot haemorrhages.

Large blotchy haemorrhages are usually indicative of retinal ischaemia.

Background diabetic retinopathy.

Retinal vein occlusion.

Radiation retinopathy.

The haemorrhage is located between the photoreceptors and the retinal pigment epithelium (RPE).

Usually large and bright red with slightly indistinct borders ( Fig. 13.6 ).

The retina overlying the haemorrhage is usually slightly elevated, but the retinal vessels are clearly seen.

Choroidal neovascularisation (CNV).

Ruptured retinal artery macroaneurysm.

Coats disease.

Sickle cell anaemia.

Blunt ocular trauma.

The haemorrhage is located between the RPE and Bruch membrane.

Slightly elevated, very dark-red, almost black, with relatively well-defined borders.

They are often solitary and located at the macula ( Fig. 13.7 ).

CNV is by far the most common (haemorrhagic RPE detachment).

Dark red and frequently extensive.

Blunt trauma.

Complication of drainage of subretinal fluid during retinal detachment surgery ( Fig. 13.8 ).

Cotton-wool spots, venous dilatation, and flame-shaped and blotchy haemorrhages ( Fig. 13.10A ).

FA shows venous staining, capillary non-perfusion, blockage by blood and pruning of vessels in the affected area ( Fig. 13.10B ).

Cotton-wool spots, intraretinal microvascular abnormalities (IRMA), venous dilatation and beading, and haemorrhages ( Fig. 13.11A ).

Peripheral arteriolar narrowing, silver-wiring and occlusion.

FA shows extensive capillary non-perfusion, punctate staining of microaneurysms and enhancement of the venous changes ( Fig. 13.11B ).

Cotton-wool spots, arteriolar narrowing, arteriolosclerosis, flame-shaped haemorrhages, retinal oedema, macular star and disc oedema ( Fig. 13.12 ).

A rare condition that has many causes ( Table 13.1 ).

Large cotton-wool spots, some of which may be confluent ( Fig. 13.13 ).

Superficial peripapillary haemorrhages are common.

Cotton-wool spots ( Fig. 13.14 ) occasionally associated with retinal haemorrhages and capillary abnormalities.

Cotton-wool spots, haemorrhages ( Fig. 13.15 ), microaneurysms, macular oedema, hard exudates, proliferative retinopathy and papillopathy.

Cotton-wool spots, venous tortuosity, dot-blot and flame-shaped retinal haemorrhages and Roth spots ( Fig. 13.16 ).

Bilateral peripapillary cotton-wool spots and disc swelling ( Fig. 13.17 ).

Bilateral, hard exudates, microaneurysms and retinal haemorrhages ( Fig. 13.25 ).

Unilateral segmental hard exudates, venous sheathing, collaterals and residual haemorrhage ( Fig. 13.26 ).

Small hard exudates, soft drusen and macular haemorrhage ( Fig. 13.27 ).

Unilateral ring of hard exudates surrounding the lesion ( Fig. 13.28 ).

Associated haemorrhages may be subretinal, intraretinal, preretinal and vitreous.

Intraretinal and subretinal exudates, retinal vascular tortuosity, dilatation and aneurysm formation ( Fig. 13.30 ).

Cotton-wool spots, flame-shaped haemorrhages, arteriolosclerosis and disc swelling in the malignant (accelerated) phase ( Fig. 13.33 ).

Severe bilateral disc swelling ( Fig. 13.34 ).

Mild disc swelling which is subsiding as the macular star forms ( Fig. 13.35 ).

Stellate maculopathy may occur with a lesion on the disc ( Fig. 13.36 ) or in the periphery.

Retinal opacification along the vascular arcades and haemorrhages ( Fig. 13.37 ).

Extensive subretinal exudation which may be unilateral or bilateral ( Fig. 13.38 ).

Extensive subretinal exudation associated with retinal telangiectasia (see Fig. 13.30 ).

New vessels may involve the optic nerve head (new disc vessels – NDV – Fig. 13.39 ) or develop along the major vascular arcades (new vessels elsewhere – NVE – Fig. 13.40 ).

The vessels may be flat or elevated, and they may be bare or associated with fibrosis ( Fig. 13.41 ).

FA shows intensive leakage of dye and capillary non-perfusion ( Fig. 13.42 ).

Proliferative diabetic retinopathy ( Fig. 13.43 ).

Old branch retinal vein occlusion ( Fig. 13.44 ).

Ocular ischaemic syndrome.

Radiation retinopathy.

Retinal vasculitis ( Fig. 13.45 ).

Previous retinal artery occlusion is an uncommon cause.

Carotid–cavernous fistula.

Vitreous haemorrhage that may be subhyaloid ( Fig. 13.46 and see Figs 13.1 and 13.2 ) or intragel ( Fig. 13.47 and Fig. 13.48 ).

Tractional RD ( Fig. 13.49 ).

New vessels at or anterior to the equator ( Fig. 13.50 ).

They are usually located at the junction of perfused and non-perfused retina.

Sickle cell retinopathy has a ‘sea-fan’ configuration ( Fig. 13.51 ).

Eales disease – recurrent vitreous haemorrhage from peripheral new vessels ( Fig. 13.52 ).

Retinopathy of prematurity ( Fig. 13.53 ).

Familial exudative vitreoretinopathy ( Fig. 13.54 ).

Chronic myeloid leukaemia .

Sarcoidosis .

Incontinentia pigmenti (Bloch–Sulzberger syndrome) – characteristic cutaneous lesions (see Figs 9.21 and 9.22 ), retinal dysplasia and leukocoria.

Vitreous haemorrhage.

Tractional retinal detachment.

An uncommon, usually unilateral condition which typically affects elderly hypertensive females.

Localised, fusiform or saccular dilatation usually arising within the first three orders of bifurcation ( Fig. 13.61 ).

More than one lesion in the same eye occurs in about 20% of cases.

Haemorrhage with surrounding hard exudates are common (see Fig. 13.28 ).

An uncommon, developmental vascular anomaly which typically presents at about the age of 8 years with leukocoria (see Fig. 9.34 ) or strabismus.

It is more common in boys than in girls.

Vascular dilatation, tortuosity and aneurysm formation at the posterior pole and periphery ( Fig. 13.62 ).

Retinal and subretinal exudate formation (see Fig. 13.30 ).

Exudative RD and a retrolental mass which may give rise to leukocoria.

Late-onset retinoblastoma.

Toxocara canis which may also be associated with hard exudate formation.

Unilateral, large, aberrant vessel, usually a vein, in the posterior pole that may cross the fovea and horizontal raphe ( Fig. 13.63 ).

Retinal racemose haemangioma (see Fig. 13.66 ).

Unilateral anomaly in which both the vein and artery are dilated ( Fig. 13.64 ).

Location is in the papillomacular bundle or the superotemporal quadrant.

A benign but vision-threatening tumour that may be solitary or multifocal.

Both eyes are affected in 50% of cases.

Round and orange-red tumour.

Associated with dilated and tortuous artery and vein ( Fig. 13.65 ).

Hard exudates near the lesion or at the macula.

Vitreous haemorrhage.

Retinal detachment.

Von Hippel–Lindau syndrome (see Fig. 12.48 ).

A usually unilateral, congenital arteriovenous malformation that may occur in isolation or in association with systemic lesions (Wyburn-Mason syndrome).

Grossly dilated and tortuous arteries and veins which are more numerous than in a normal fundus ( Fig. 13.66 ).

A congenital, innocuous unilateral tumour.

Clumps of thin-walled, grape-like saccular aneurysms filled with blood ( Fig. 13.67 ).

Patchy, fluffy, white haziness surrounding the venous blood column ( Fig. 13.79 ) that may be associated with haemorrhage.

Later there is venous sheathing ( Fig. 13.80 ).

FA shows vascular leakage and staining of the vessel wall ( Fig. 13.81 ).

Sarcoidosis – severe periphlebitis results in perivascular ‘candle-wax drippings’ ( Fig. 13.82 ).

Behçet disease – periphlebitis may be associated with haemorrhage and venous occlusion ( Fig. 13.83 ); in some cases periarteritis may coexist (see below).

AIDS – periphlebitis is frequently associated with CMV retinitis ( Fig. 13.84 ).

Tuberculosis – periphlebitis may lead to severe retinal ischaemia and secondary neovascularisation ( Fig. 13.85 ).

Multiple sclerosis – periphlebitis may be associated with intermediate uveitis.

Cat-scratch fever.

Whipple disease.

Crohn disease.

Initially, patchy, fluffy white haziness surrounding the arteriolar blood column.

Later there is arteriolar sheathing.

Severe involvement may result in multiple branch artery occlusions ( Fig. 13.86 ).

FA shows staining of arterioles and variable occlusion.

Systemic lupus erythematosus.

Polyarteritis nodosa.

Dermatomyositis.

Behçet disease (also periphlebitis).

Acquired syphilis.

Wegener granulomatosis.

Kawasaki disease.

Churg–Strauss syndrome.

A usually bilateral condition that may rarely be idiopathic and more commonly is secondary to CMV retinitis.

Florid translucent perivascular sheathing of arteries and veins.

Anterior uveitis, vitritis and retinal oedema are common.

Venous occlusion ( Fig. 13.87 ).

Papillitis.

Hard exudate formation.

Retinal haemorrhage.

A protozoan infection which typically affects one eye of a young individual.

Anterior uveitis and vitritis.

Solitary focal retinitis adjacent to an old scar ( Fig. 13.88 ).

Inactive lesions are atrophic scars with pigmentation ( Fig. 13.89 ).

A very rare worm infection that is acquired in early childhood; ocular involvement is always unilateral.

Posterior ( Fig. 13.90 ) or peripheral granuloma that may be associated with ‘dragging’ of the disc (see Fig. 12.58 ).

Chronic endophthalmitis (sees Figs 9.32 and 9.33 ).

Retinoblastoma may resemble a posterior-pole or peripheral granuloma.

A fungal infection which is associated with intravenous drug abuse, parenteral nutrition, chronic lung disease and neutropenia.

Solitary or multiple retinal infiltrate.

Vitritis and fluffy white ‘cotton’ balls ( Fig. 13.91 ).

Intermediate uveitis.

Sarcoidosis.

Lyme disease.

Whipple disease.

Seeding of endophytic retinoblastoma.

A common granulomatous multisystem disorder which affects the eyes in about 30% of cases.

Anterior uveitis, which is most frequently chronic and granulomatous.

Periphlebitis (see Fig. 13.82 ).

Discrete, grey-white to waxy-yellow retinal and preretinal nodules (Lander sign – Fig. 13.92 ).

Multifocal choroidal infiltrates, which are more frequent in the inferior fundus ( Fig. 13.93 ).

Vitreous cotton balls.

Black patient with facial palsy due to neurosarcoid ( Fig. 13.94 ).

Multifocal choroiditis with panuveitis.

Birdshot retinochoroidopathy.

An uncommon multisystem disorder which is frequently associated with ocular manifestations which are usually bilateral.

Acute anterior uveitis with hypopyon but a relatively white eye (see Fig. 8.17 ).

Vitritis.

White, necrotic, superficial retinal infiltrates ( Fig. 13.95 ).

Vasculitis and venous occlusion (see Fig. 13.83 ).

Macular oedema.

Vascular attenuation and optic atrophy in end-stage disease ( Fig. 13.96 ).

Superficial thrombophlebitis ( Fig. 13.97 ).

An uncommon idiopathic condition which typically affects pigmented individuals; bilateral ocular involvement is the rule.

Chronic granulomatous anterior uveitis.

Multifocal choroiditis followed by detachments of the sensory retina ( Fig. 13.98A ).

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