Ischemic Choroidal Diseases

Abstract

Introduction Ischemic choroidal diseases are an underdiagnosed entity. The clinical pattern varies according to the size and the localisation of the affected vascular structure. Clinical Presentation In eyes with occlusion of the long posterior ciliary arteries, characteristic triangular patches of choroidal ischemia (Amalric sign) are seen, which in the course of time merge into well-defined areas of atrophy of the retinal pigment epithelium. Above the non-perfused choroidal areas, hyperpigmented, grouped lines appear (Siegrist streaks). Circumscribed ischemia of smaller choroidal arterioles and capillary vessels appears as multifocal, yellowish lesions in the posterior fundus (Elschnig spots). Vortex vein occlusion becomes manifest as exudative haemorrhagic choroidal swelling in the periphery. Causes of Choroidal Ischemia Apart from arterial hypertension as a major risk factor, some immunological disorders such as giant cell arteritis and systemic lupus erythematosus and haematological pathologies also affect choroidal perfusion. Furthermore, choroidal ischemia occurs due to local inflammation, as found in eyes with acute multifocal posterior placoid pigment epitheliopathy (APMPPE). Rarely, choroidal infarction is of iatrogenic origin or drug-induced. Recent advances in imaging, such as the introduction of enhanced depth imaging optical coherence tomography (EDI-OCT) and OCT angiography (OCT-A), have improved the visualisation of the choroidal vasculature and complement the classical angiographic procedures. In patients with age-related macular degeneration (AMD) and diabetes, some changes in choroidal blood flow and vascular structure have also been noted. While in AMD the choroidal pathologies correlate with the disease progression and the functional prognosis, the pathophysiological relationship between diabetic choroidopathy and retinopathy is currently unclear. Management and Conclusion With regard to the limited therapeutic options for choroidal ischemia, optimisation of the cardiovascular risk profile and the management of accompanying ocular and systemic diseases are essential.

Analysis of risk and protective factors associated with retinal nerve fiber layer defect in a Chinese adult population

“…In hypertension, vascular resistance increases over time, which can lead to microvascular damage and atherosclerosis. Atherosclerosis caused by hypertension can cause selfregulation disorders of the vascular bed, and a series of pathological changes such as arteriolar endothelial damage, media thickening, and luminal stenosis occurs, leading to perfusion disorders of the posterior ciliary artery [33] . Retinal tissue is one of the most metabolically active tissues in the whole body.…”

Choroidal involvement in systemic vasculitis: a systematic review

“…These triangular zones of choroidal, RPE, and outer retinal infarction later appear as atrophic areas with pigmentary changes (Fig. 3), typically less extensive in size than the area of apparent ischemia [21,26]. Siegrist's streaks are linear pigmentations overlying sclerosed choroidal vessels, first described in hypertensive choroidopathy [21,28].…”

“…Unlike the abundance of studies of retinal vasculitis in the literature, choroidal vasculitis has not been investigated in isolation. There are recent reviews on classification and imaging of immune mediated choroiditis including choriocapillaropathies [19,20] and ischemic choroidal diseases [21]; however, there is a paucity of data on the involvement of choroidal vessels in systemic vasculitides. The aim of this article was to review involvement of the choroid in systemic vasculitic diseases.…”

“…Placoid lesions as in APMPPE may also occur when the choroid is involved in systemic vasculitides. Other signs of choroidal vasculopathy in systemic vasculitis may be indistinguishable from hypertensive choroidopathy which is mostly seen in younger patients with acutely elevated severe arterial hypertension [21]. Occlusion of small choroidal terminal arterioles is manifested as Elschnig spots, appearing as discreet deep retinal paleyellow spots due to focal RPE infarction, particularly in the posterior pole (Fig.…”

“…Later they become irregularly pigmented with a depigmented halo (Fig. 2) [21,[23][24][25]. Occlusion of larger choroidal arterioles cause small geographic lesions.…”