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Characteristics and Quantification of Vascular Changes in Macular Downloaded from http://bjo.bmj.com/ on November 15, 2016 - Published by group.bmj.com Clinical science Characteristics and quantification of vascular changes in macular telangiectasia type 2 on optical coherence tomography angiography Lavanya Chidambara,1 Santosh G K Gadde,1 Naresh Kumar Yadav,1 Chaitra Jayadev,1 Devanshi Bhanushali,1 Abhishek M Appaji,2 Mukunda Akkali,1 Aruj Khurana,1 Rohit Shetty1 1Narayana Nethralaya Eye ABSTRACT MacTel type 2 (MacTel 2) is a bilateral disease Hospital, Bangalore, India Background Macular telangiectasia type 2 (MacTel 2) characterised by changes in the capillary network 2BMS College of Engineering, Bangalore, India is a bilateral idiopathic, rare neurodegenerative disease and neurosensory atrophy. Slit lamp biomicroscopy with alterations in the macular capillary network leading reveals reduced retinal transparency, crystalline Corresponding to to vision loss and is the most common of three subtypes. deposits, ectatic capillaries and blunted venules.4 Dr Santosh G K Gadde, Optical coherence tomography angiography (OCTA) is a Complications of this disorder include proliferation Department of Vitreo-retina, non-invasive imaging modality which helps understand of pigment plaques, photoreceptor loss, foveal Narayana Nethralaya, #121, Chord Road, 1st ‘R’ Block, the complex pathological changes, and images the blood atrophy and retinal neovascularisation that may 5 Rajaji Nagar, Bangalore, vessels across different layers based on their flow result in visual loss. Karnataka 560010, India; characteristics. Stereoscopic colour and red-free fundus photog- [email protected] Methods A cross-sectional study was conducted on raphy and fundus fluorescein angiography (FA) Received 7 October 2015 56 eyes of Asian Indian eyes of 28 consecutive patients have been previously used to describe the disease. Revised 23 December 2015 with MacTel 2 studied during a 3-month period in a Optical coherence tomography (OCT) provides Accepted 30 December 2015 tertiary eye care hospital of South India. Clinically useful visualisation of the retinal and choroidal Published Online First diagnosed cases of MacTel 2 underwent fundus changes in patients with MacTel 2. The currently 28 January 2016 photography, spectral domain OCT and OCTA. described spectral domain OCT signs (SD-OCT) of Fluorescein angiography was performed only when MacTel 2 include hyporeflective spaces in the inner clinically indicated. Mean capillary density was calculated and the outer retina (retinal cysts), plaques and using a MATLAB-based automated software. The images breaks in the ellipsoid zone with progressive outer were thresholded and binarised to derive the mean retinal atrophy in which the retinal layers interior value. to the outer nuclear layer seemingly ‘collapse’ Results The mean age at presentation was 60±5.2, through these breaks towards the retinal pigment with a female preponderance of 71.42%. Vascular epithelium (RPE).67Progression of the disease is network on OCTA shows an increase in the intervascular characterised by shrinkage of the outer retinal spaces with progressive capillary rarefaction and layers and reduction in the central foveal thickness abnormal capillary anastomosis. The outer retina and (CFT).8 En face OCT also enables visualisation of choroid were involved during the later stages and the pigment plaques and new vessels in the various showed a prominent vascular network. The mean retinal layers along with the extent of associated capillary density of the superficial and deep layers was exudation.59 39.99% and 39.03% as against 45.18% and 44.21% FA is the gold standard to confirm the diagnosis in the controls, respectively. There was a statistically of MacTel 2 showing telangiectatic capillaries pre- significant difference between the two groups (p<0.01). dominantly temporal to the foveola10 in the early There is a positive and statistically significant correlation phase and a diffuse hyperfluorescence in the late between the superficial and deep layers. phase. The late hyperfluorescence can be seen even Conclusion OCTA helps understand the pathology and in the absence of telangiectatic alterations. Gass disease progression better in MacTel 2. and Oyakawa1 on studying stereoscopic angio- graphic images have suggested that vascular altera- tions mainly affect the deeper capillary plexus and that the late diffuse fluorescence on FA appears to INTRODUCTION originate from the outer retina. The finer morpho- Macular telangiectasia (MacTel) is characterised by logical changes in the vascular network are abnormalities of the capillaries around the fovea. obscured by the diffuse hyperfluorescence and FA The condition can be a result of a retinal vascular fails to provide details about the deeper retinal disease or systemic diseases such as diabetes or layers and finer pathological changes. Besides, FA is hypertension, but is most often idiopathic. MacTel an invasive imaging modality with documented was formerly called idiopathic juxtafoveolar retinal side effects and repeating the test at every visit may telangiectasis and was classified into three not be feasible. 12 To cite: Chidambara L, groups. Recent advances in imaging have OCT angiography (OCTA) is a new imaging Gadde SGK, Yadav NK, allowed better characterisation of the disease to modality that allows high-resolution imaging of the et al. Br J Ophthalmol define it into two distinct forms, type 1 and type 2 retinal morphology, especially of individual vascu- – 2016;100:1482 1488. MacTel.3 lar layers, without the use of an injectable dye. It 1482 Chidambara L, et al. Br J Ophthalmol 2016;100:1482–1488. doi:10.1136/bjophthalmol-2015-307941 Downloaded from http://bjo.bmj.com/ on November 15, 2016 - Published by group.bmj.com Clinical science has an A-scan rate of 70 000 scans/s, using a light source centred on 840 nm and a bandwidth of 45 nm. The tissue reso- lution is 5 μm axially with a 22 μm beam width. Each B-scan contains 512 A scans.11 RTVue XR 100 (Avanti, Fremont, California, USA) uses the split spectrum amplitude decorrelation algorithm to acquire information on the flow in the retinal and choroidal vasculature.12 Objective The aim of the study was to describe the OCTA features of the different stages of MacTel 2. The capillary density of the super- ficial and deep layers of retina were studied and compared with controls. PATIENTS AND METHODS Study design It is a cross-sectional, observational study. OCTA was performed in 56 Asian Indian eyes of 28 consecu- tive patients with clinically confirmed MacTel 2 for a period of 3 months (February–April 2015) in a tertiary eye care hospital in South India. Ten eyes of five age-matched controls with a normal ophthalmic examination were included in the study. The study met the approval of the Institute Research Board and the Institute Ethics Committee and informed consents were obtained from all enrolled subjects. The study adhered to the Figure 1 Offset and default segmentation of normal retina. ILM, tenets of the Declaration of Helsinki. internal limiting membrane; IPL, inner plexiform layer; RPE, retinal All patients underwent a complete ophthalmic examination pigment epithelium. along with fundus photography, OCT (Heidelberg engineering, Spectralis (V.6.0) GmBH) and OCTA (Optovue RTVue XR 100 fi Avanti, Fremont, California, USA). FA (Heidelberg engineering, The nal output gave a measure of the total area of study, capil- fi Spectralis (V.6.0), GmBH) was performed only in advanced lary area and percentage of capillary density in pixels ( gure 2). cases when clinically indicated. The Gass and Blodi classifica- tion4 was used for classifying subjects into the five clinical Statistical analysis stages. For statistical analysis, IBM SPSS Statistics 22 was used. Since all A single technician acquired a mydriatic OCTA with two con- variables studied exhibited a normal distribution, parametric secutive scans of 3 by 3 mm centred on the macula with an tests were conducted. Post-hoc tests were applied wherever internal fixating target. The OCTA images were independently necessary. Pearson’s correlation coefficients were calculated to graded and assessed by two clinicians. Segmentation of the investigate bivariate relationships and partial correlation coeffi- superficial, deep, outer retina and choroidal levels was done cients were calculated when controlling for confounding vari- with a default standard offset with segmentation layers based at ables. We used the 5% level of significance throughout our internal limiting membrane, inner plexiform layer and RPE analysis. Independent samples t-test was performed to compare (figure 1). In advanced cases of MacTel 2 where the retinal the capillary density between patients with MacTel 2 and con- architecture was altered with gross foveal thinning and extensive trols. One-way analysis of variance was performed to evaluate cystic spaces, the offset was manually adjusted to acquire the the difference in the capillary density of the different stages of right segmentation of layers before analysis. the disease. The outer retina showed speckled pattern occasionally sec- ondary to an artefact or noise which was minimised with the RESULTS ‘remove artefacts’ option of the software and by adjusting the The demographic and vision-related data of the 28 subjects offset to the possible limit in case of architectural changes, recruited into the study are summarised in table 1. The age of based on the B scan. Other occasional artefacts included super- the patients in the cohort ranged from 42 to 75 years with a imposed vessel
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