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.

Key words

choroid - angiography - choroidal infarction - vascular occlusion - ischemia

• References/Literatur

• 1 Amalric P. Choroidal vascular ischaemia. Eye (Lond) 1991; 5: 519-527
• 2 Hayreh SS, Servais GE, Virdi PS. Fundus lesions in malignant hypertension. VI. Hypertensive choroidopathy. Ophthalmology 1986; 93: 1383-1400
• 3 Kishi S, Tso MO, Hayreh SS. Fundus lesions in malignant hypertension. I. A pathologic study of experimental hypertensive choroidopathy. Arch Ophthalmol 1985; 103: 1189-1197
• 4 Rezkallah A, Kodjikian L, Abukhashabah A. et al. Hypertensive choroidopathy: Multimodal imaging and the contribution of wide-field swept-source oct-angiography. Am J Ophthalmol Case Rep 2019; 13: 131-135
• 5 Elschnig A. Die diagnostische und prognostische Bedeutung der Netzhauterkrankungen bei Nephritis. Wien Med Wochenschr 1904; 54: 446-450
• 6 AlTalbishi A, Khateb S, Amer R. Elschnigʼs spots in the acute and remission stages in preeclampsia: spectral-domain optical coherence tomographic features. Eur J Ophthalmol 2015; 25: e84-e87
• 7 Bird AC. Pathogenesis of Serous Detachment of the Retina and Pigment Epithelium. In: Ryan SJ, Schachat AP, Hinton DR, Wilkinson CP. eds. Retina. St. Louis: Elsevier; 2006: 971-977
• 8 Song YS, Kinouchi R, Ishiko S. et al. Hypertensive choroidopathy with eclampsia viewed on spectral-domain optical coherence tomography. Graefes Arch Clin Exp Ophthalmol 2013; 251: 2647-2650
• 9 Sakai D, Matsumiya W, Kusuhara S. et al. The choroidal structure changes in a case with hypertensive choroidopathy. Am J Ophthalmol Case Rep 2020; 18: 100710
• 10 Amalric P. Acute choroidal ischaemia. Trans Ophthalmol Soc U K 1971; 91: 305-322
• 11 Siegrist A. Beitrag zur Kenntnis der Arteriosklerose der Augengefässe. Z Augenheilkunde 1899; 2 (Suppl.) S36-S37
• 12 Mantel I, Schalenbourg A, Zografos L. Evanescent vaso-occlusive choroidal pseudo-tumor with acute painful onset: a presumed vortex vein occlusion. Graefes Arch Clin Exp Ophthalmol 2014; 252: 753-759
• 13 Nemiroff J, Phasukkijwatana N, Vaclavik V. et al. The spectrum of amalric triangular choroidal infarction. Retin Cases Brief Rep 2017; 11 (Suppl. 01) S113-S120
• 14 Märker DA, Walter A, Helbig H. Chorioidale Gefäßverschlüsse. Ophthalmologe 2011; 108: 64-67
• 15 Jampol LM, Lahov M, Albert DM. et al. Ocular clinical findings and basement membrane changes in Goodpastureʼs syndrome. Am J Ophthalmol 1975; 79: 452-463
• 16 Goldbaum MH, Galinos SO, Apple D. et al. Acute choroidal ischemia as a complication of photocoagulation. Arch Ophthalmol 1976; 94: 1025-1035
• 17 Moshfeghi DM, Lowder CY, Roth DB. et al. Retinal and choroidal vascular occlusion after posterior sub-tenon triamcinolone injection. Am J Ophthalmol 2002; 134: 132-134
• 18 Thomas EL, Laborde RP. Retinal and choroidal vascular occlusion following intralesional corticosteroid injection of a chalazion. Ophthalmology 1986; 93: 405-407
• 19 Wachtlin J, Behme T, Heimann H. et al. Concentric retinal pigment epithelium atrophy after a single photodynamic therapy. Graefes Arch Clin Exp Ophthalmol 2003; 241: 518-521
• 20 Tsukikawa M, Stacey AW. A Review of hypertensive retinopathy and chorioretinopathy. Clin Optom (Auckl) 2020; 12: 67-73
• 21 Tso MO, Jampol LM. Pathophysiology of hypertensive retinopathy. Ophthalmology 1982; 89: 1132-1145
• 22 Lim S, Cheng CK, Li YH. Amalric triangular sign in a case of central retinal artery occlusion combined with posterior ciliary artery occlusion – Case report. Am J Ophthalmol Case Rep 2018; 11: 149-152
• 23 Hande P, Sinha B, Nayak S. et al. Amalric sign: An augur of ophthalmic artery occlusion. Indian J Ophthalmol 2017; 65: 1033-1035
• 24 Kang HM, Choi JH, Koh HJ. et al. Significant changes of the choroid in patients with ocular ischemic syndrome and symptomatic carotid artery stenosis. PLoS One 2019; 14: e0224210
• 25 Nguyen QD, Uy HS, Akpek EK. et al. Choroidopathy of systemic lupus erythematosus. Lupus 2000; 9: 288-298
• 26 Hsu CT, Kerrison JB, Miller NR. et al. Choroidal infarction, anterior ischemic optic neuropathy, and central retinal artery occlusion from polyarteritis nodosa. Retina 2001; 21: 348-351
• 27 Valmaggia C, Speiser P, Bischoff P. et al. Indocyanine green versus fluorescein angiography in the differential diagnosis of arteritic and nonarteritic anterior ischemic optic neuropathy. Retina 1999; 19: 131-134
• 28 Cohen S, Gardner F. Bilateral choroidal ischemia in giant cell arteritis. Arch Ophthalmol 2006; 124: 922
• 29 Cogan DG. Ocular involvement in disseminated intravascular coagulopathy. Arch Ophthalmol 1975; 93: 1-8
• 30 Hay-Smith G, Sagoo MS, Raina J. Fatal thrombotic thrombocytopenia purpura presenting with choroidal vasculopathy and serous retinal detachment. Eye (Lond) 2006; 20: 982-984
• 31 Kotoula M, Papageorgiou E, Xanthou F. et al. Choroidal vascular occlusion in a young male patient with sickle cell trait. Int J Ophthalmol 2018; 11: 528-532
• 32 Ebrahimiadib N, Maleki A, Fadakar K. et al. Vascular abnormalities in uveitis. Surv Ophthalmol 2021; 66: 653-667
• 33 Park D, Schatz H, McDonald HR. et al. Indocyanine green angiography of acute multifocal posterior placoid pigment epitheliopathy. Ophthalmology 1995; 102: 1877-1883
• 34 Heiferman MJ, Rahmani S, Jampol LM. et al. Acute posterior multifocal placoid pigment epitheliopathy on optical coherence tomography angiography. Retina 2017; 37: 2084-2094
• 35 Carle MV, Roe R, Novack R. et al. Cosmetic facial fillers and severe vision loss. JAMA Ophthalmol 2014; 132: 637-639
• 36 Park SH, Sun HJ, Choi KS. Sudden unilateral visual loss after autologous fat injection into the nasolabial fold. Clin Ophthalmol 2008; 2: 679-683
• 37 Peyman GA, Kazi AA, Unal M. et al. Problems with and pitfalls of photodynamic therapy. Ophthalmology 2000; 107: 29-35
• 38 Hirata A, Kubo M, Okinami S. Severe retinal atrophy due to retinal and choroidal vascular occlusion following triamcinolone injection into the nasal mucosa. Jpn J Ophthalmol 2008; 52: 510-511
• 39 Reddy S, Goldman DR, Hubschman JP. et al. Cocaine and choroidal infarction, revisiting the triangular sign of amalric. Retin Cases Brief Rep 2011; 5: 91-93
• 40 Melancia D, Vicente A, Cunha JP. et al. Diabetic choroidopathy: a review of the current literature. Graefes Arch Clin Exp Ophthalmol 2016; 254: 1453-1461
• 41 Farazdaghi MK, Ebrahimi KB. Role of the Choroid in Age-related Macular Degeneration: A Current Review. J Ophthalmic Vis Res 2019; 14: 78-87
• 42 McLeod DS, Grebe R, Bhutto I. et al. Relationship between RPE and choriocapillaris in age-related macular degeneration. Invest Ophthalmol Vis Sci 2009; 50: 4982-4991
• 43 McLeod DS, Lutty GA. High-resolution histologic analysis of the human choroidal vasculature. Invest Ophthalmol Vis Sci 1994; 35: 3799-3811
• 44 Deutsche Ophthalmologische Gesellschaft, Berufsverband der Augenärzte Deutschlands. Leitlinie von DOG, RG und BVA: Anteriore ischämische Optikusneuropathie (AION). Stand Juli 2001. Accessed March 23, 2021 at: http://augeninfo.de/leit/leit29.htm
• 45 Deutsche Ophthalmologische Gesellschaft, Retinologische Gesellschaft, Berufsverband der Augenärzte Deutschlands. Leitlinie von DOG, RG und BVA: Retinale arterielle Verschlüsse (RAV). Stand November 2016. Ophthalmologe 2017; 114: 120-131
• 46 Biousse V, Nahab F, Newman NJ. Management of Acute Retinal Ischemia: Follow the Guidelines!. Ophthalmology 2018; 125: 1597-1607
• 47 Dattilo M, Newman NJ, Biousse V. Acute retinal arterial ischemia. Ann Eye Sci 2018; 3: 28