Cilioretinal artery hypoperfusion and its association with paracentral acute middle maculopathy

Abstract

Background/aimsTo study the multimodal imaging findings of a large series of eyes with cilioretinal artery obstruction (CILRAO) and describe the systemic associations.MethodsMulticentre, retrospective chart review from 12 different retina clinics worldwide of eyes with CILRAO, defined as acute retinal whitening in the distribution of the cilioretinal artery, were identified. The clinical, systemic information and multimodal retinal imaging findings were collected and analysed.ResultsA total of 53 eyes of 53 patients with CILRAO were included in the study. In 100% of eyes, fundus photography illustrated deep retinal whitening corresponding to the course of the cilioretinal artery. Twenty-eight patients (52.8%) presented with isolated CILRAO (baseline best-corrected visual acuity (BCVA) 20/50, final BCVA 20/25) associated with nocturnal hypotension, 23 patients (43.4%) with CILRAO secondary to central retinal vein occlusion (CRVO) (baseline BCVA 20/40, final BCVA 20/20) and two patients with CILRAO due to biopsy-proven giant cell arteritis (GCA) (baseline BCVA 20/175, final BCVA 20/75). With spectral domain optical coherence tomography (SD-OCT), a hyper-reflective band involving the inner nuclear layer (ie, paracentral acute middle maculopathy or PAMM) was noted in 51 eyes (28/28 eyes with isolated CILRAO and 23/23 eyes with CILRAO+CRVO) corresponding to the retinal whitening. In the two eyes with CILRAO+GCA, SD-OCT illustrated hyper-reflective ischaemia of both the middle and inner retina.ConclusionsIsolated CILRAO and CILRAO secondary to CRVO are the result of hypoperfusion or insufficiency, rather than occlusion, of the cilioretinal artery and are associated with PAMM or selective infarction of the the inner nuclear layer. With GCA, there is complete occlusion of the cilioretinal artery producing ischaemia involving both the middle and inner retina associated with worse visual outcomes.

Multimodal imaging for paracentral acute maculopathy; the diagnostic role of en face OCT

“…closer to the involved artery), hyperreflectivity of both middle and inner retinal layers in B-scan OCT with a globular pattern in en face OCT is observed. Recent studies revealed that micro vortex-like structures in DCP were responsible for venous drainage of middle retinal layers and behaved as a watershed zone area [18][19][20]. Meticulous scrutiny of en face OCT and OCTA imaging has demonstrated that PAMM, whether isolated or not, heralds an ischemic cascade, which begins as a fernlike configuration with a propensity to venular pole at the level of DCP and progresses laterally to the center of ischemia, producing globular pattern.…”

Cilioretinal Artery Hypoperfusion in Association with Paracentral Acute Middle Maculopathy: A Case Report and Review of Literature

“…Recent enface OCT imaging and OCTA studies have shown that micro vortex-vein like structures are available in the deep capillary plexus and these structures are responsible for the drainage of the middle retinal layers, so any occlusion in front of the venous pathway can predominantly affect this area and secondary PAAM can be seen here [4][5][6]8]. So, it seems that deep capillary plexus is a sensitive structure for both arterial and venous insufficiencies and multimodal imaging studies can show this vulnerability to vascular disturbances.…”

“…As opposed to the central retinal artery, the cilioretinal artery arises from the peripapillary choroid or directly from one of the short posterior ciliary arteries and isn't liable to autoregulation. Subsequently, a drop in systemic blood pressure may cause a secondary reduction in the perfusion pressure and relative hypoperfusion or insufficiency of the cilioretinal artery [4,5].…”

Paracentral acute middle maculopathy associated with hypoperfusion of the cilioretinal artery and impending central retinal vein occlusion

“…The cilioretinal artery (CILRA) can be found in one third of normal eyes. 1 This artery originates from the choroid or from one of the short posterior ciliary arteries. 1,2 Conditions of its obstruction (cilioretinal artery obstruction (CILRAO) have been historically subdivided into three groups: (a) CILRAO isolated, (b) CILRAO associated with retinal vein occlusions, and (c) CILRAO associated with giant cell arteritis.…”

“…1,2 Conditions of its obstruction (cilioretinal artery obstruction (CILRAO) have been historically subdivided into three groups: (a) CILRAO isolated, (b) CILRAO associated with retinal vein occlusions, and (c) CILRAO associated with giant cell arteritis. 1…”