PURPOSE\nTo measure macular choroidal thickness in normal eyes at different points using enhanced depth imaging (EDI) optical coherence tomography (OCT) and to evaluate the association of choroidal thickness and age.\n\n\nDESIGN\nRetrospective, observational case series.\n\n\nMETHODS\nEDI OCT images were obtained in patients without significant retinal or choroidal pathologic features. The images were obtained by positioning a spectral-domain OCT device close enough to the eye to acquire an inverted image. Seven sections were obtained within a 5 x 30-degree area centered at the fovea, with 100 scans averaged for each section. The choroid was measured from the outer border of the retinal pigment epithelium to the inner scleral border at 500-microm intervals of a horizontal section from 3 mm temporal to the fovea to 3 mm nasal to the fovea. Statistical analysis was performed to evaluate variations of choroidal thickness at each location and to correlate choroidal thickness and patient age.\n\n\nRESULTS\nThe mean age of the 30 patients (54 eyes) was 50.4 years (range, 19 to 85 years), and 14 patients (46.7%) were female. The choroid was thickest underneath the fovea (mean, 287 microm; standard deviation, +/- 76 microm). Choroidal thickness decreased rapidly in the nasal direction and averaged 145 microm (+/- 57 microm) at 3 mm nasal to the fovea. Increasing age was correlated significantly with decreasing choroidal thickness at all points measured. Regression analysis suggested that the subfoveal choroidal thickness decreased by 15.6 microm for each decade of life.\n\n\nCONCLUSIONS\nChoroidal thickness seems to vary topographically within the posterior pole. The thickness of the choroid showed a negative correlation with age. The decrease in the thickness of the choroid may play a role in the pathophysiologic features of various age-related ocular conditions.

Enhanced Depth Imaging Optical Coherence Tomography of the Choroid in Normal Eyes

http://www.scielo.br/pdf/clin/v66n3/v66n3a03.pdf

Ocular findings in patients with systemic sclerosis

PURPOSE To evaluate the relationships between RPE, photoreceptor, and choroidal degeneration in choroideremia. METHODS Enhanced-depth imaging optical coherence tomography (EDI-OCT), scanning laser ophthalmoscopy (SLO), and autofluorescence (AF) were performed on 39 patients (78 eyes) with choroideremia. The edges of surviving outer retina on OCT and residual AF were aligned. The distribution of outer retinal tubulations was mapped over a range of ages (16-71 years), and comparison made between pre- and postsubretinal gene therapy. Subfoveal choroidal thickness (SFCT) was compared between 23 choroideremia patients (42 eyes) and 20 age- and refraction-matched male controls (40 eyes). RESULTS The edges of RPE AF aligned with a reduction in outer nuclear layer thickness (Spearman's rho = 0.9992). Correlation was also found between the quality of AF and integrity of ellipsoid zone within islands of surviving retina. Tubulations existed in 71 of 78 (91%) eyes with choroideremia and remained stable following gene therapy. Subfoveal choroidal thickness was reduced at baseline in choroideremia (179.7 ± 17.2 μm) compared with controls (302.0 ± 4.8 μm; P < 0.0001), but did not undergo significant thinning until end-stage retinal degeneration (43.1 ± 6.5 μm). CONCLUSIONS The data suggest that RPE loss is the primary cause of photoreceptor degeneration in choroideremia. The choroid is thinner than controls from early stages, in keeping with a mild developmental defect. Photoreceptors appear to lose outer segments following loss of underlying RPE and form tubulations at the edges of degeneration. The preservation of tubulations over time and after subretinal injection would be consistent with these structures maintaining attachment to the inner retina and hence being potentially light responsive (ClinicalTrials.gov, NCT01461213).

Correlation of Optical Coherence Tomography and Autofluorescence in the Outer Retina and Choroid of Patients With Choroideremia.

Purpose To investigate characteristics of the foveal pit and the foveal avascular zone (FAZ) in patients with Alport syndrome (AS), a rare monogenetic disease due to mutations in genes encoding for collagen type IV. Methods Twenty-eight eyes of nine patients with AS, and five autosomal-recessive carriers and 15 eyes from 15 age-similar healthy control subjects were examined using optical coherence tomography (OCT) and OCT-angiography (OCT-A). Foveal configuration and FAZ measures including the FAZ area, circularity, and vessel density in the central 1° and 3° were correlated. Results Foveal hypoplasia was found in 10 eyes from seven patients with either genotype. In contrast, a staircase foveopathy was found in seven eyes of four X-linked AS patients. The average FAZ area did not differ significantly between AS patients and control subjects (mean ± SD 0.24 ± 0.24 mm2 vs. 0.21 ± 0.09 mm2; P = 0.64). Five eyes showed absence or severe anomalies of the FAZ with crossing macular capillaries that was linked to the degree of foveal hypoplasia on OCT images leading to a significant inverse correlation of FAZ area and foveal thickness (r = -0.88; P < 0.001). In contrary, female patients with X-linked mutations exhibited a significantly greater FAZ area (0.48 ± 0.30 mm2 vs. 0.21 ± 0.09 mm2; P = 0.007), in line with OCT findings of a staircase foveopathy. Conclusions The foveal phenotypic spectrum in AS ranges from foveal hypoplasia and absence of a FAZ to staircase foveopathy with an enlarged FAZ. Because the development of the FAZ and foveal pit are closely related, these findings suggest an important role for collagen type IV in foveal development and maturation.

Phenotypic Spectrum of the Foveal Configuration and Foveal Avascular Zone in Patients With Alport Syndrome.

Systemic sclerosis (SSc) is a rare, chronic autoimmune disease with unknown etiology. Its prominent features are fibrosis, vasculopathy and impaired immune response. Disease can also affect eyes leading to various findings in ophthalmological examination. The objective of this study was to determine the prevalence and type of ocular involvement in patients with SSc. A systematic literature review was conducted using electronic databases. A combination of following keywords was used: "systemic sclerosis" and ophthalmology-related search terms, including the keywords "eye", "ocular" and "ophthalmic". In conclusion, eyelid and conjunctival abnormalities and dry eye disease are among the most common ocular manifestations of SSc. Their diversity is connected to complexity of the disease.

https://www.termedia.pl/Journal/-18/pdf-42774-10?filename=Ocular manifestations.pdf

Ocular manifestations in patients with systemic sclerosis

Purpose:To quantitatively describe choroidal thickness in a patient with Alport syndrome and to qualitatively describe an unusual “stair-case” foveal sign.Methods:Retrospective case report.Results:This is the first case, to our knowledge, of the stair-case foveal sign. This is also the first case wh

Choroidal thinning and "stair-case" foveal sign in a patient with Alport syndrome.

Dear Editor Outer retinal tubulations (ORT) have recently been described as a unique but non-specific OCT (optical coherence tomography) finding in age-related macular degeneration, retinal dystrophies and chronic central serous retinopathy [1, 2]. On SD-OCT, these tubulations appear as round to ovoid structures, usually with hyper-reflective walls and relatively hypo-reflective cavity. Zweifel et al. [1] are credited with the first detailed description of ORT, and in their case series of 63 patients with varied pathology, one patient with A3243G macular dystrophy phenotype (mitochondrial inherited disorders) was mentioned. However, the details of that particular patient were not reported. A 70-year-old Caucasian female was referred by her optometrist with suspicion of age-related macular degeneration. She did not report any visual loss, but was concerned about her ‘retinal condition’, especially as the optometrist had raised the concern of wet AMD. She had been diagnosed as having MIDD (maternally inherited diabetes and deafness) about 10 years back, confirmed with mitochondrial DNA mutation A3243G, and ophthalmic consultation had confirmed typical MIDD-associated macular pattern dystrophy, with no diabetic retinopathy changes. She was advised annual ophthalmic review, but was subsequently lost to follow-up. Generally she was fit and well apart from mild hearing loss. Visual acuity was 0.2 LogMAR (6/10 Snellens) unaided in both eyes.. Anterior segment examination was unremarkable, with normal intraocular pressure. Detailed retinal evaluation showed bilateral symmetrical peripapillary and parafoveal RPE atrophy with relative foveal sparing (Fig. 1), but no features of diabetic retinopathy. Fundus autofluorescence, infra-red reflectance near infra-red autofluorescence showed corresponding signal loss in zones of RPE and photoreceptors atrophy. No distinctive feature of ORT is noted on infra-red reflectance or autofluorescence images. Figure 2 shows the SD-OCT (Spectralis) B-scan images of the right eye showing outer retinal tubulations at varying levels above the RPE in zones of atrophic retina. Similar changes were noted on imaging the left eye (not shown). To our knowledge, this is the first case report detailing outer retinal tubulations (ORT) using multimodality autofluorescence imaging and SD-OCT, in a documented A3243G MIDD-associated macular dystrophy. MIDD with macular involvement is a well-known but uncommon cause of diabetes and deafness, and may show A3243G mitochondrial DNA mutation, usually with varying phenotypic variability [3–5]. Two distinct features of MIDD-associated macular dystrophy, noted in our case as well, include the involvement of peripapillary area (a distinguishing feature compared to Stargardt’s disease, in which peripapillary area is relatively unaffected) and absence of significant clinical diabetic retinopathy. The clinical and histo-pathological correlation is yet to confirm whether the ORT are indeed ‘rosettes’ formed by the photoreceptors and theorized in M. S. Raja :C. Goldsmith :B. J. L. Burton James Paget University Hospital NHS Trust, Norfolk, UK

Outer retinal tubulations in maternally inherited diabetes and deafness (MIDD)-associated macular dystrophy.

A 75 year old lady presented with acute central retinal artery occlusion and contralateral cotton wool spots. General physical examination and investigations led to a diagnosis of CREST syndrome; however, association of central retinal artery occlusion with CREST syndrome is not well known. While diabetes, systemic hypertension, carotid atherosclerosis and cardiac pathology are common causes of CRAO it is always important to rule out giant cell arteritis. This case highlights that inflammatory causes of central retinal artery occlusion other than giant cell arteritis should also be considered as a possibility to spare unnecessary use of excessive systemic corticosteroids.

/pdf/acute-central-retinal-artery-occlusion-presenting-as-crest-1lxp0ocww4.pdf

Acute central retinal artery occlusion presenting as CREST syndrome: a case report

Introduction
Pain in scleritis may be difficult to control with conventional immuno-suppressive regimes. Amitriptyline may be an effective option in those circumstances.

/pdf/amitriptyline-for-pain-control-in-scleritis-may-help-to-zi40gkkx7c.pdf

Amitriptyline for pain control in scleritis may help to avoid excessive steroid use in selected cases: a case report.

Iatrogenic retinal detachment (RD) due to cannula slippage is a rare occurrence. Cannula detachment from a syringe creates an intraocular missile capable of causing sight-threatening damage. We recently experienced cannula slippage during the wound hydration stage of phacoemulsification that caused a shallow localized RD despite the use of a luer-lock syringe system. To our knowledge, there have been no previous such reports. Intraoperative cannula slippage is uncommon. Reported sequelae include iris damage, posterior capsule rupture, zonular dialysis, vitreous hemorrhage, and retinal breaks. In a series of 10 230 cases of anterior segment surgery over 15 years, Rumelt et al. report 6 cases of cannula release during cataract extraction. The precautions they implemented included luer-lock syringes, careful orientation of cannula tips away from important structures during injection, and bimanual injection. Cannula slippage occurred despite all these safety measures.

Iatrogenic retinal detachment due to cannula slippage despite use of luer-lock syringe system.

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