Angiographic features of polypoidal choroidal vasculopathy using indocyanine green angiography and optical coherence tomography angiography: A comparative study.

BACKGROUND: To compare the vascular lesion size using optical coherence tomography angiography and indocyanine green angiography in eyes with polypoidal choroidal vasculopathy. METHODS: Treatment-naïve cases (46 eyes of 44 patients) with polypoidal choroidal vasculopathy were retrospectively analyzed. The comparison of mean area of branching vascular network and polyp detection rate was done between indocyanine green angiography and optical coherence tomography angiography and correlated with various optical coherence tomography features. RESULTS: The mean age of the study patients was 62.33 ± 10.74 years. The mean branching vascular network size was 7.47 ± 5.74 and 7.51 ± 5.69 mm² in indocyanine green angiography and optical coherence tomography angiography, respectively, with an excellent correlation (r = 0.997). Optical coherence tomography angiography overestimated (mean ± SD: 0.28 ± 0.19 mm²) and underestimated branching vascular network area (0.36 ± 0.33 mm²) in 23 eyes each as compared to indocyanine green angiography. However, the difference in branching vascular network size was not statistically significant (p = 0.53). Indocyanine green angiography and optical coherence tomography angiography could identify polyps in 43 of 46 (93.48%) and 32 of 46 (69.57%) patients, respectively. CONCLUSION: Branching vascular network size measurements with indocyanine green angiography and optical coherence tomography angiography were comparable and showed significant correlation, albeit the polyp identification rate was lower with optical coherence tomography angiography. Optical coherence tomography angiography may serve as a useful substitute to indocyanine green angiography in measurements of branching vascular network for photodynamic therapy and follow-up of polypoidal choroidal vasculopathy eyes.

Changes in peripapillary blood vessel density in Graves' orbitopathy after orbital decompression surgery as measured by optical coherence tomography angiography.

The purpose is to evaluate the utility of optical coherence tomography (OCT) angiography in the evaluation of Graves' orbitopathy (GO) and response to orbital decompression in patients with and without dysthyroid optic neuropathy (DON). This was a single-center, prospective case series in a cohort of 12 patients (24 orbits) with GO and ±DON, (6 orbits) who underwent bilateral orbital decompression. All patients underwent pre- and postoperative OCT angiography of the peripapillary area. Vessel density indices were calculated in a 4.5 mm × 4.5 mm ellipsoid centered on the optic disk using split-spectrum amplitude decorrelation angiography algorithm, producing the vessel density measurements. Mean change in vessel density indices was compared between pre- and postoperative sessions and between patients with and without DON. Patient 1, a 34-year-old male with GO and unilateral DON OD, showed a significant reduction in blood vessel density indices oculus dexter (OD) (DON eye) after decompression while a more modest reduction was found oculus sinister (OS) with the greatest change noted intrapapillary. Patient 2, a 50-year-old male with DON OU, showed worsening neuropathy following decompression OD that was confirmed by angiographic density indices. Patient 3, a 55-year-female with DON, showed a reduction in blood vessel density OD and increased density OS. Patients without DON showed overall less impressive changes in indices as compared to those with DON. Using OCT angiography, response to surgical treatment in GO orbits, more so in orbits with DON, can be demonstrated and quantified using vessel density indices with reproducibility.

Choroidal morphology under pachydrusen.

IMPORTANCE: To reveal choroidal morphological profiles under pachydrusen. BACKGROUND: Drusen in pachychoroid disorders show certain differences from conventional drusen and are recently named as "pachydrusen." This study analysed the specific choroidal morphology under pachydrusen. DESIGN: This study is a cross-sectional case series. PARTICIPANTS: Enrolled were 136 eyes with pachydrusen from 134 patients. METHODS: The presence of pachydrusen in fundus area covered by a 12 × 9 mm2 optical coherence tomography (OCT) image was accessed in eyes with pachychoroid-related diseases or their fellow eyes using colour fundus photography and swept source OCT. Choroidal morphology under the pachydrusen was analysed using OCT B-scans and en face images. MAIN OUTCOME MEASURES: Choroidal thicknesses and topographical correlation between pachydrusen and pachyvessels. RESULTS: A total of 225 pachydrusen in 136 eyes were analysed. The mean number of pachydrusen was 1.65 ± 1.07 per eye. Most pachydrusen were located para- or perifoveally (37 subfoveal, 86 parafoveal and 102 perifoveal). The proportion of Haller's layer to total choroidal thickness was higher at the area of the pachydrusen compared to the subfovea (0.881 ± 0.081 vs 0.765 ± 0.111, P < 0.001). In multimodal image analysis, 90.1% of pachydrusen identified using fundus photography and OCT B-scan were located at the area of a dilated Haller vessel (pachyvessel) seen on en face images. CONCLUSIONS AND RELEVANCE: Choroidal morphology under the pachydrusen showed increased Haller's layer thickness with an attenuated choriocapillaris layer, which is the hallmark of pachychoroid definition. Topographically, their locations correlated with the underlying pachyvessel.

Variation of Retinal and Choroidal Vasculatures in Patients With Age-Related Macular Degeneration.

Purpose: To investigate variations in chorioretinal vasculatures in fellow eyes of patients with unilateral neovascular age-related macular degeneration (nAMD). Methods: We included fellow eyes of consecutive patients with unilateral nAMD from swept source optical coherence tomography (SS-OCT) angiography database. Vascular and nonvascular indices were determined based on SS-OCT and SS-OCT angiography images. Variation of the vascular or nonvascular index was compared between fellow eyes with and without early AMD. Results: In 146 fellow eyes, 88 (60.3%) had early AMD and 58 (39.7%) had a normal fundus. Vascular density (VD) values of the superficial and deep retinal capillary plexus and choriocapillaris were smaller in eyes with early AMD than in those without (P < 0.001, P = 0.001, P = 0.006, respectively). Flow void area in choriocapillaris was greater in eyes with early AMD than those without (P = 0.015). In 88 fellow eyes with early AMD, vascular indices of the retina were correlated with those of choroid while nonvascular indices were not. Fellow eyes of patients with classic exudative AMD had greater foveal avascular zone area and smaller VD values of the deep retinal capillary plexus than those with polypoidal choroidal vasculopathy (P < 0.001, P = 0.004). Conclusions: In addition to vascular insufficiency in the choroid or choriocapillaris, retinal vascular alteration was apparent in eyes with early AMD. It may suggest that retinal vessels were involved in the pathogenesis of AMD even while changes in nonvascular components of the retina were not yet apparent.

Cilioretinal artery occlusion and anterior ischemic optic neuropathy as the initial presentation in a child female carrier of Fabry disease.

PURPOSE: To report the youngest female carrier of Fabry disease, complicated by cilioretinal artery occlusion and anterior ischemic optic neuropathy (AION). METHODS: Case report. RESULTS: An 11-year-old girl was referred to our clinic with painless, acute loss of vision in her right eye. Posterior segment examination and fluorescein angiography revealed cilioretinal artery occlusion and AION. Systemic evaluations were unremarkable, except for a low blood α-galactosidase A enzyme level of 242.27 pmol/spot*20 h (reference range: 450-2000 pmol/spot*20 h). The patient was diagnosed with female carrier of Fabry disease. CONCLUSION: Retinal vascular occlusions are rare in childhood, and Fabry disease may present with retinal vascular occlusion. Ophthalmological examinations may be contributing for early detection of the disease. To the best of our knowledge, this is the first report of a child female carrier of Fabry disease, complicated by cilioretinal artery occlusion and AION.

Optical Coherence Tomography Angiography Features of Iris Racemose Hemangioma in 4 Cases.

Importance: Optical coherence tomography angiography (OCTA) allows visualization of iris racemose hemangioma course and its relation to the normal iris microvasculature. Objective: To describe OCTA features of iris racemose hemangioma. Design, Setting, and Participants: Descriptive, noncomparative case series at a tertiary referral center (Ocular Oncology Service of Wills Eye Hospital). Patients diagnosed with unilateral iris racemose hemangioma were included in the study. Main Outcomes and Measures: Features of iris racemose hemangioma on OCTA. Results: Four eyes of 4 patients with unilateral iris racemose hemangioma were included in the study. Mean patient age was 50 years, all patients were white, and Snellen visual acuity was 20/20 in each case. All eyes had sectoral iris racemose hemangioma without associated iris or ciliary body solid tumor on clinical examination and ultrasound biomicroscopy. By anterior segment OCT, the racemose hemangioma was partially visualized in all cases. By OCTA, the hemangioma was clearly visualized as a uniform large-caliber vascular tortuous loop with intense flow characteristics superimposed over small-caliber radial iris vessels against a background of low-signal iris stroma. The vascular course on OCTA resembled a light bulb filament (filament sign), arising from the peripheral iris (base of light bulb) and forming a tortuous loop on reaching its peak (midfilament) near the pupil (n = 3) or midzonal iris (n = 1), before returning to the peripheral iris (base of light bulb). Intravenous fluorescein angiography performed in 1 eye depicted the iris hemangioma; however, small-caliber radial iris vessels were more distinct on OCTA than intravenous fluorescein angiography. Conclusions and Relevance: Optical coherence tomography angiography is a noninvasive vascular imaging modality that clearly depicts the looping course of iris racemose hemangioma. Optical coherence tomography angiography depicted fine details of radial iris vessels, not distinct on intravenous fluorescein angiography.

Cilioretinal artery: Vasculogenesis might be promoted by plasminogen activator inhibitor-1 5G allele.

BACKGROUND: Cilioretinal arteries (CAs) represent enlargements of microscopic and early established collaterals formed via vasculogenesis between choroidal and retinal circulations. We aimed to investigate whether genetic tendency to thrombosis due to well-known gene polymorphisms may induce CA vasculogenesis in embryonic life. METHODS: We assessed plasminogen activator inhibitor-1 (PAI-1) 4G/5G, methylenetetrahydrofolatereductase (MTHFR), FACTOR V LEIDEN and PROTHROMBIN gene polymorphisms on 130 patients [82/48 females/males; Median age: 57 (18-84) with visible CAs and 100 (64/36: female/male; Median age: 55 (19-90)] without visible CAs. RESULTS: Using multiple logistic regression models, we found PAI-1 4G/5G; MTHFR (C677T and A1298C) polymorphisms to have significant effects on the probability of visible CAs, that having at least one 5G allele would increase the odds of having visible cilioretinal artery by 98.4% [Odds ratio: 1984 (95% CI: 1.320-3.000, p = 0.001)], and having at least one MTHFR C677T or A1298C allele would decrease the odds of having visible CAs by approximately 38% (OR = 0.618, 95% CI: 0.394-0.961, p = 0.035) or 44% (OR = 0.558, 95% CI: 0.354-0.871, p = 0.011), respectively. CONCLUSIONS: This is the first study to test the existence of significant association between presence of enlarged and visible CAs and genetic factors predisposing to thrombosis, according to the literature. Here we suggest that not only the lack of genetic predisposition to thrombosis by MTHFR gene polymorphisms, but also the PAI-1 5G allele might promote vasculogenesis of CAs.

Cilioretinal Artery Territory Infarction Associated With Papilledema in a Patient With Neurofibromatosis Type 2.

Cilioretinal artery territory infarction can occur in isolation or in association with other vascular compromise of the retinal circulation. Our patient, an 18-year-old woman with neurofibromatosis type 2, developed a cilioretinal artery territory infarction in the setting of papilledema. Our case, together with one previous report, suggests that cilioretinal artery territory infarction in the context of papilledema, although rare, is a real entity.

[Cilioretinal artery occlusion in hemochromatosis]. / Oclusión de arteria ciliorretiniana en la hemocromatosis.

CLINICAL CASE: We report a case of a 31 year-old woman with a sudden visual loss due to a cilioretinal artery occlusion. The physical examinination showed hepatomegaly. Serum iron and ferritin and transferrin saturation were unusually high. The doppler scan of carotid arteries showed no relevant signs of atheromatous disease. Dilated cardiomiopaty was revealed in the B-scan with subendocardial calcium deposits. Genetic tests were positive for hemochromatosis. DISCUSSION: Subendocardial calcification due to hemochromatosis could be the embolic source in our patient. This embolic ocular disease is the first presentation of hemochromatosis in this patient.

Ultrahigh-speed swept-source OCT angiography in exudative AMD.

BACKGROUND AND OBJECTIVE: To investigate the potential of ultrahigh-speed swept-source optical coherence tomography angiography (OCTA) to visualize retinal and choroidal vascular changes in patients with exudative age-related macular degeneration (AMD). PATIENTS AND METHODS: Observational, prospective cross-sectional study. An ultrahigh-speed swept-source prototype was used to perform OCTA of the retinal and choriocapillaris microvasculature in 63 eyes of 32 healthy controls and 19 eyes of 15 patients with exudative AMD. MAIN OUTCOME MEASURE: qualitative comparison of the retinal and choriocapillaris microvasculature in the two groups. RESULTS: Choroidal neovascularization (CNV) was clearly visualized in 16 of the 19 eyes with exudative AMD, located above regions of severe choriocapillaris alteration. In 14 of these eyes, the CNV lesions were surrounded by regions of choriocapillaris alteration. CONCLUSION: OCTA may offer noninvasive monitoring of the retinal and choriocapillaris microvasculature in patients with CNV, which may assist in diagnosis and monitoring.

Optic disc dysplasia in poland syndrome.

PURPOSE: To report optic disc dysplasia in a case of Poland syndrome. DESIGN: Non-interventional case report. METHODS: A 2-year-old boy with Poland syndrome was referred for ophthalmic evaluation after abnormal optic discs were found on exam. RESULTS: Physical exam at birth revealed right-sided aplasia of the pectoralis major muscle, symbrachydactyly, hypoplastic scapula, and an abnormal third rib. On dilated examination the optic nerve heads were dysplastic. The findings included multiple cilioretinal vessels, situs inversus, inferotemporal excavation, and surrounding pigmentary disturbances. CONCLUSION: Only one case of optic disc anomaly has been reported in Poland syndrome and was described as morning glory syndrome. The optic discs in our patient do not fit well with other optic disc excavation syndromes but are most reminiscent of those in papillorenal syndrome. As both Poland syndrome and papillorenal syndrome share vascular dysfunction as a possible etiology, this case adds to the literature of vascular dysgenesis in Poland syndrome.

Anterior segment angiography in strabismus surgery.

BACKGROUND: Anterior ciliary arteries travelling along recti muscles provide anterior segment vascularization, which can be compromised by surgery involving more than 2 muscles. PATIENTS AND METHODS: We studied retrospectively the files of 10 patients in whom a fluorescein angiography of the iris had been performed as a pre-operative assessment prior to a second or third oculomotor surgery. RESULTS: The median age of the patients was 47.5 years (range 15 to 73 years). Relative iris ischemia was present in 4 patients following multiple surgeries, none of them presenting any general cardiovascular risk. The initial surgical protocol was modified according to angiographic results in these 4 patients. CONCLUSION: When further surgery has to be performed on previously multi-operated patients, anterior segment angiography can be useful in the planning of surgery in order to minimize the risks of anterior segment ischemia.

Histomorphometry of the circular peripapillary arterial ring of Zinn-Haller in normal eyes and eyes with secondary angle-closure glaucoma.

PURPOSE: To examine the location and size of the peripapillary arterial circle of Zinn-Haller (PACZH) and its associations with other eye measures in normal eyes and eyes with secondary angle-closure glaucoma. METHODS: The study included 29 human globes enucleated because of malignant choroidal melanoma (n = 19) (control group) or because of secondary angle-closure glaucoma (n=10). Anterior-posterior histological sections were morphometrically evaluated. RESULTS: The PACZH was present in all eyes. The glaucoma group and the control group did not vary significantly in the distance from the PACZH to Bruch's membrane (297 ± 67 versus 270 ± 67 µm; p = 0.29), optic disc border (281 ± 103 versus 391 ± 170 µm; p=0.07), optic disc centre (1059 ± 191 versus 978 ± 205µm; p = 0.30) and retrobulbar cerebrospinal fluid space (173 ± 58 versus 172 ± 81 µm; p = 0.97) nor in the minimal PACZH diameter (39± 18 versus 36 ± 18 µm; p = 0.74) and maximal PACZH diameter (78 ±37 versus 65 ± 25 µm; p = 0.36). The PACZH location, measured as distance from Bruch's membrane, disc border, disc centre and cerebrospinal fluid space, was not significantly associated with axial length (p > 0.39), horizontal globe diameter (p > 0.17) and vertical globe diameter (p > 0.22). Both diameters were statistically independent of axial length (p = 0.72 and p = 0.58, respectively), horizontal globe diameter (p = 0.60 and p = 0.41, respectively) and vertical globe diameter (p = 0.64 and p = 0.52, respectively). All parameters were statistically independent of age (p > 0.10) and gender (p > 0.10). CONCLUSIONS: The PACZH was present in all human eyes examined and did not vary significantly in location and diameter between eyes with secondary angle-closure glaucoma and nonglaucomatous eyes, nor between myopic versus hyperopic eyes.

Central retinal and posterior ciliary artery occlusion after intralesional injection of sclerosant to glabellar subcutaneous hemangioma.

The aim of this study is to describe vision loss caused by central retinal artery and posterior ciliary artery occlusion as a consequence of sclerotherapy with a polidocanol injection to a glabellar hemangioma. An 18-year-old man underwent direct injection with a 23-gauge needle of 1 mL of a polidocanol-carbon dioxide emulsion into the glabellar subcutaneous hemangioma under ultrasound visualization of the needle tip by radiologists. He developed lid swelling the next day, and 3 days later at referral, the visual acuity in the left eye was no light perception. Funduscopy revealed central retinal artery occlusion and fluorescein angiography disclosed no perfusion at all in the left fundus, indicating concurrent posterior ciliary artery occlusion. The patient also showed mydriasis, blepharoptosis, and total external ophthalmoplegia on the left side. Magnetic resonance imaging demonstrated the swollen medial rectus muscle. In a month, blepharoptosis and ophthalmoplegia resolved but the visual acuity remained no light perception. Sclerosing therapy for facial hemangioma may develop a severe complication such as permanent visual loss.

OCT and ultrasound biomicroscopic findings in iris arteriovenous malformation.

Iris arteriovenous malformation, also labeled as iris racemose hemangioma, is a rare condition in which abnormal vessels are present on the iris surface. Slit-lamp findings in addition to anterior segment fluorescein angiographic features of such lesions have been described in the past. The authors report anterior segment optical coherence tomography and ultrasound biomicroscopic features in two patients with iris arteriovenous malformation.