Isolated occlusion of a cilioretinal artery]

PURPOSE: Isolated cilioretinal artery occlusion is uncommon but has characteristic features. Based on a case report and a review of the literature, we present the clinical findings and angiographic particularities of this syndrome and discuss controversial pathophysiological data. CASE REPORT AND METHOD: A 56-year-old man had sudden visual loss in the right eye estimated at 3/10 P10. Fundus examination showed areas of retinal interpapillomacular infarction due to cilioretinal artery occlusion. Fluorescein angiography demonstrated delayed filling and emptying of this artery. Left fundus examination was normal. RESULTS: Systemic examinations revealed severe hypertension (240/130) rapidly controlled with a two-drug regimen. The clinical course was good with normalization of fundus and angiography, and visual recovery to 8/10 P3 with a small absolute paracentral scotoma. DISCUSSION: The few cases described would offer an explanation of the low prevalence of cilioretinal arteries and the more frequent association with central retina venous obstruction which can mask arterial occlusion. A relative reversible occlusion explains the generally good prognosis especially if the capillary network is not affected by the occluded artery as was observed in our case. CONCLUSION: Although diagnosis of isolated cilioretinal artery occlusion is made without difficulty, the underlying pathogenic mechanism remains difficult to explain due to the various phenomena revealed by the increased arterial pressure.     

Delayed cilioretinal artery occlusion after a hemiretinal vein occlusion

The purpose of this study is to report a case of combined hemiretinal vein occlusion and cilioretinal artery occlusion (CLRAO), in which the development of the CLRAO was substantially delayed. This interventional case report illustrates the sequential development of a cilioretinal artery occlusion following a hemiretinal vein occlusion with colour fundus and fluorescein angiogram photographs. Systemic examination revealed previously unrecorded hypertension for which her general practitioner commenced treatment. The patient developed an inferior visual field defect however made a visual recovery to 20/40 at 6-month follow-up. In this report, the risk factors and the likely pathogenesis for such an event are studied.     

Central retinal vein occlusion associated with cilioretinal artery occlusion

PURPOSE: To describe the clinical characteristics and pathogenesis of central retinal vein occlusion (CRVO) associated with cilioretinal artery occlusion (CLRAO). METHODS: The study included 38 patients (38 eyes) who had CRVO associated with CLRAO and were seen in our clinic from 1974 to 1999. At their first visit to our clinic, all patients provided a detailed ophthalmic and medical history and underwent comprehensive ophthalmic evaluation, color fundus photography, and fluorescein fundus angiography. At each follow-up visit, the same ophthalmic evaluations were performed, except for fluorescein fundus angiography. RESULTS: Of 38 eyes, 30 had nonischemic CRVO, 5 had ischemic CRVO, and 3 had nonischemic hemi-CRVO. Patients with nonischemic CRVO were significantly younger (mean age +/- SD: 45.3 +/- 16.0 years) than those with ischemic CRVO (72.3 +/- 9.2 years; P = 0.001) and those with nonischemic hemi-CRVO (64.7 +/- 7.5 years; P = 0.018). At least one third of the patients gave a definite history of episode(s) of transient visual blurring before the onset of constant blurred vision. Initially, the ophthalmoscopic and fluorescein angiographic findings were similar to those seen in CRVO and hemi-CRVO, except that all these eyes had retinal infarct in the distribution of the cilioretinal artery; its size and site varied widely. Fluorescein angiography typically showed only transient hemodynamic block and not the typical CLRAO. During follow-up, visual acuity improved markedly in nonischemic CRVO (P < 0.001) and nonischemic hemi-CRVO but deteriorated in ischemic CRVO. Retinopathy resolved spontaneously in 22 eyes with nonischemic CRVO (mean duration +/- SD: 42.0 +/- 101.0 months), in 2 eyes with ischemic CRVO (15.4 +/- 4.5 months), and in 1 eye with nonischemic hemi-CRVO. Retinociliary collaterals developed in 30% of eyes with nonischemic CRVO, in 40% of eyes with ischemic CRVO, and in 66% of eyes with nonischemic hemi-CRVO. CONCLUSION: CRVO associated with CLRAO constitutes a distinct clinical entity. The pathogenesis of CLRAO in CRVO is due to transient hemodynamic blockage of the cilioretinal artery caused by a sudden sharp rise in intraluminal pressure in the retinal capillary bed (due to CRVO) above the level of that in the cilioretinal artery.     

Central retinal vein occlusion combined with cilioretinal artery occlusion

A healthy 65-year-old man with sudden profound visual loss in his right eye presented with clinical signs of central retinal venous occlusion and retinal whitening, indicative of a cilioretinal arterial obstruction. He had been diagnosed with cilioretinal artery occlusion at a private ophthalmology clinic three days before being referred to our department. On fluorescein angiogram of the affected eye, the proximal portion of the retinal arteries filled with dye 27.3 seconds after injection, indicating a delay in retinal arterial filling. Moreover, the cilioretinal artery did not fill at that phase, but went on to fill 45.1 seconds after injection. Over 63.4 seconds after the filling of the retinal arteries, the laminar flow of the retinal venous vessels appeared. This was not until 90.7 seconds after injection. This patient was elderly, had no systemic diseases, and showed non-ischemic CRVO, prolonged retinal arterial filling on fluorescein angiography, and poor prognosis in visual acuity. His clinical course seemed to favor the pathogenetic hypothesis of a primary arterial affection.     

Embolic cilioretinal artery occlusion due to carotid artery dissection

PURPOSE: To report a case of embolic cilioretinal artery occlusion caused by carotid artery dissection. DESIGN: Interventional case report. METHODS: A 38-year-old woman presented with acute visual loss in her right eye. Funduscopy showed a cilioretinal artery occlusion, which was confirmed by a fluorescein angiography. An embolus was found in the distal segment of the vessel. RESULTS: Color Doppler images of right internal carotid artery (ICA) disclosed a pseudolumen, suggesting a diagnosis of carotid dissection. Retrobulbar color Doppler image showed relative low flow velocity in the ophthalmic artery without flow reversal. Magnetic resonance angiography and cerebral angiogram showed total occlusion of the right ICA. Follow-up visual field examination revealed an inferior central defect fed by the cilioretinal artery. CONCLUSION: The pathogenesis of retinal artery occlusion caused by carotid dissection may be embolic or hemodynamic. In our case, a permanent visual defect was related to embolic occlusion of the cilioretinal artery.     

Myelinated nerve fibers associated with cilioretinal artery occlusion

Although the myelinated nerve fibers are considered as benign, cases associated with retinal vascular abnormalities (telangiectasis, neovascularization, vascular occlusions) have recently been reported, suggesting a possible pathogenic correlation between these elements. Our observation presents a 44-year-old patient, with a sudden decrease of visual acuity in the right eye, discal and peridiscal myelinated nerve fibers, associated with cilioretinal artery occlusion. The general clinical and laboratory examination, except for a drug stabilized arterial hypertension, did not reveal other local or general embolic factors. One year later, the decrease in visual acuity had remained unchanged and the area of the myelinated nerve fibers had diminished. Based on the association between the myelinated nerve fibers, the cilioretinal artery occlusion, the young age of the patient, and the absence of other local or general embolic factors, we consider there is a possible pathogenic correlation between these elements. The action of the myelinated nerve fibers can be explained by a mechanical compression, with the disruption of the cilioretinal artery route, a structurally vulnerable artery.     

Pathogenetic mechanisms in combined cilioretinal artery and retinal vein occlusion: a reappraisal

We examined nine patients who presented cilioretinal artery occlusion (CLRAO) associated with retinal vein occlusion (RVO). CLRAO was probably secondary to the raised intraluminal resistance consequent to the RVO in patients showing initially a delayed filling of the cilioretinal artery in fluorescein angiography. Interestingly, these patients presented an ophthalmoscopically more severe form of RVO and had systemic predisposing factors for a RVO. In patients presenting a physiological perfusion of the cilioretinal artery in fluorescein angiography, RVO was a self limited disease and etiologic factors were not found. This may suggest that in these patients the CLRAO probably occurred simultaneously with the RVO after a decrease in perfusion pressure in both retinal and cilioretinal arterial systems. In this combined vaso-occlusive retinopathy the vulnerability of cilioretinal arteries can be explained either by the absence of autoregulation or by their lower perfusion pressure gradient in comparison with retinal arteries.     

Combined central retinal vein and cilioretinal artery occlusion

We present a case of combined central retinal vein and cilioretinal artery occlusion which, due to the absence of the temporal branch retinal artery, was initially misdiagnosed as a combined central retinal vein occlusion and temporal branch retinal artery occlusion. Given that – in contrast to cases of combined central artery and central retinal vein occlusion – the prognosis for cilioretinal artery occlusion with central retinal vein occlusion is quite good, this case illustrates the importance of suspecting an unusual condition in the presence of a combined occlusion.     

Surgical mobilization of an arterial embolus in cilioretinal artery occlusion

We describe an effective surgical approach for the management of cilioretinal artery occlusion. A 23-G pars plana vitrectomy assisted with two soft tip cannulas was performed. One cannula pressed the cilioretinal artery branch directed toward the macula, distal to the location of the embolus, whereas the other cannula was used to gently swipe over the cilioretinal artery proximal to the occlusion. Anatomical and functional outcomes were evaluated by fundus examination, fluorescein angiography, Goldmann visual field, and best-corrected visual acuity (BCVA). It was possible to mobilize the embolus by mechanical displacement with 23-G soft-tip cannulas and disintegrate it, preventing the passage toward the branch directed to the macula. Restoration of retinal circulation was confirmed by fluorescein angiogram. The patient recovered his previous documented BCVA and visual field. The described technique can be considered as a new possibility for achieving a solution to cilioretinal artery occlusion or any other retinal artery occlusion.     

Bilateral combined cilioretinal artery and central retinal vein occlusion

A rare case with bilateral combined cilioretinal artery and central retinal vein occlusion (CRVO) is reported in a 45-year-old man. Work-up was positive only for already-present type 2 diabetes. The patient first developed ischemic-type CRVO and cilioretinal artery occlusion in his right eye, and underwent successful trabeculectomy with mitomycin-C following panretinal and grid macular photocoagulation. The fellow eye developed a nonischemic type of CRVO and cilioretinal artery occlusion 4 years after the first episode with good visual outcome. The authors have stated that they do not have a significant financial interest or other relationship with any product manufacturer or provider of services discussed in this article. The authors also do not discuss the use of off-label products, which includes unlabeled, unapproved, or investigative products or devices.     

Occlusion of a cilioretinal artery associated with occlusion of the central retinal vein

Cilioretinal artery occlusion (CLRAO) may be occasionally associated with central retinal vein occlusion (CRVO). This combined retinal vascular pathology is described in 5 patients. In this clinical setting the vulnerability of cilioretinal vessels can be explained by the difference in regulatory mechanisms between choroidal and retinal blood flow. The type of CRVO may predict whether the CLRAO is primary or secondary to the CRVO.     

Optic disc edema with adjacent cilioretinal artery occlusion in a male with ulcerative colitis

OBJECTIVE: To report a case of optic disc edema with adjacent retinal ischemia in ulcerative colitis. DESIGN: Photo essay. CASE REPORT: A 36-year-old white man presented with visual loss OD. Past medical history was significant for ulcerative colitis. The patient had suffered presumed non-arteritic anterior ischemic optic neuropathy OS one year prior to the episode OD. Ophthalmoscopic exam showed optic disc edema associated with an area of adjacent retinal ischemia that was confirmed on fluorescein angiography OD. An extensive evaluation for infectious, inflammatory, vasculitic, and hypercoagulable etiologies was negative. The presumptive diagnosis of ulcerative colitis-related ischemic optic neuropathy with simultaneous retinal ischemia was made. The patient was treated with antiplatelet therapy and the vision stabilized but did not recover. CONCLUSION: To our knowledge, this is the first report in the English language literature of a simultaneous ischemic event involving the optic nerve and the adjacent retina in a patient with ulcerative colitis.