Macroaneurysm, sickle retinopathy and retinal periphlebitis

Classify the vessel and the complication

Retinal vascular disease extends beyond diabetes and major arterial or venous occlusion. Retinal arterial macroaneurysms, proliferative sickle retinopathy and inflammatory peripheral venous disease can all cause haemorrhage, exudation or detachment. The discriminating features are the involved vessel, systemic context and angiographic distribution. A haemorrhage obscuring the macula is a complication, not a complete diagnosis.

Record blood pressure, vascular risk, haematological history, inflammatory symptoms and previous episodes. Examine the retinal periphery as well as the macula. Optical coherence tomography identifies the compartment of macular fluid or blood; angiography maps vascular abnormalities and nonperfusion when the view allows. A peripheral lesion can drive central visual loss through vitreous haemorrhage even when the fovea initially looks normal.

Retinal arterial macroaneurysm

An acquired macroaneurysm is a focal dilation of a retinal arteriole, often near a bifurcation. It may be associated with hypertension and can produce haemorrhage in several planes: subretinal, intraretinal, preretinal and vitreous. Circinate exudate may surround a leaking lesion. Distinguish it from choroidal neovascularization, a retinal angiomatous lesion and a venous aneurysm by its vessel connection and imaging.

Some lesions involute and can be observed if they do not threaten vision. Persistent vision-threatening leakage or haemorrhage may justify treatment tailored to location and complication. Options include selected laser, anti-vascular endothelial growth factor treatment and procedures addressing significant premacular or submacular blood. Directly burning a macroaneurysm near the fovea or along an important artery can create additional damage; there is no universal instruction to laser every visible lesion.

Systemic blood-pressure review is part of management, but the ophthalmologist should not assume every haemorrhage proves uncontrolled hypertension. If acute visual loss occurs, establish whether there is a retinal tear, arterial occlusion or another time-critical cause before settling on the visible vascular abnormality.

Sickle cell retinopathy

Sickling can obstruct peripheral retinal vessels and produce ischaemia. Proliferative retinopathy is particularly associated with some sickle genotypes, including HbSC, even when systemic disease appears less severe than HbSS. Do not infer ocular risk solely from the frequency of painful crises. Coordinate with haematology and consider the patient's systemic and perioperative risks.

The traditional Goldberg sequence is peripheral arterial occlusion, arteriovenous anastomosis, sea-fan neovascularization, vitreous haemorrhage and retinal detachment. Nonproliferative signs include salmon-patch haemorrhages, iridescent spots and black sunbursts after resolving blood. Sea fans arise near the border of perfused and nonperfused retina and may autoinfarct; this does not guarantee that another area will remain quiescent.

Selected active proliferative disease can be treated with scatter laser to associated ischaemic retina. Randomized trial evidence supports reduced vitreous haemorrhage and visual loss in treated populations. This does not mean every small involuting sea fan requires immediate identical treatment. Anti-VEGF may have a selected adjunctive role, but traction and recurrence require attention. Vitrectomy may be needed for nonclearing haemorrhage or threatening detachment, with careful systemic preparation.

Eales disease and other periphlebitis

Eales disease describes an idiopathic peripheral retinal periphlebitis with occlusion and neovascular consequences, generally after exclusion of other causes. Tuberculosis-related disease, sarcoidosis, Behçet disease, infection and other inflammatory conditions can resemble it. A positive test of TB exposure alone does not prove that retinal inflammation is caused by active ocular tuberculosis.

Identify whether the immediate problem is active inflammation, ischaemia with neovascularization or an established surgical complication. Corticosteroid or immunomodulatory treatment addresses selected noninfectious inflammation after appropriate evaluation; antimicrobial therapy addresses demonstrated or sufficiently supported infection. Scatter photocoagulation treats neovascular drive from nonperfused retina, not the cause of an active systemic infection. Persistent vitreous haemorrhage or traction may require surgery.

Case comparison

An older hypertensive patient has multilayer haemorrhage centred on a focal arterial dilation: macroaneurysm is plausible. A young patient with HbSC has peripheral sea fans and vitreous haemorrhage: assess proliferative sickle retinopathy. Bilateral peripheral venous sheathing with inflammatory cells requires an inflammatory and infectious differential. In every case, describe the vessel and mechanism, identify the vision-threatening complication and give a systemic referral plan rather than applying diabetic retinopathy treatment to all vascular abnormalities.

Sources: Primary sickle-retinopathy scatter-laser trial and EBO intraocular-disease curriculum.