SD-OCT Stages of Progression of Type 2 Macular Telangiectasia in a Patient followed for 3 Years

Coscas, G.; Coscas, Florence; Zucchiatti, Ilaria; Bandello, Francesco; Soubrane, G.; Souied, Eric H.

doi:10.5301/ejo.5000363

Cited by 7 publications

(4 citation statements)

References 7 publications

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“… 8 Hence, it is reasonable that significant time gap between these two events may occur and therefore structural findings including disorganization of the inner layers with fusion of the inner retinal layers associated with progressive atrophy of the outer layers, formation of small intraretinal cystoid spaces in OCT, increased autofluorescence (AF) within 1 disk diameter of the foveal center due to the depletion of luteal pigment, and vascular changes detected in en face OCT and OCTA may precede the vascular phenomena such as leakage in FA. 9 After 6 years, to observe any pathological changes in the other eye, we performed funduscopic examination, infrared reflectance, SD-OCT, FAF, en face OCT, and OCTA. However, normal fundus examination and imaging studies of the fellow eye remained stable during this time ( Fig.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic macular telangiectasia type 2: A six-year study with multimodal imaging of a presumed unilateral case

Zarei

Mazloumi

Karkhaneh

et al. 2018

Journal of Current Ophthalmology

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PurposeTo present a rare manifestation of macular telangiectasia type 2 (MacTel type 2) followed up for over six years.MethodsA 61-year-old woman with one year history of blurred vision of her left eye was referred.ResultsWhereas the funduscopy, spectral-domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and fundus autofluorescence (FAF) were normal in the right eye, they revealed noticeable findings typical of MacTel type 2 in the left eye. After over six years follow-up, OCT-angiography (OCTA) showed no remarkable difference between the two eyes, and en face OCT showed subtle abnormal change in the right eye as well as typical pathological changes in the left eye.ConclusionMacTel type 2 can present unilaterally and remain so for a long time. The role of multimodal imaging in diagnosis and follow-up is of utmost importance.

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Section: Discussionmentioning

confidence: 99%

Idiopathic macular telangiectasia type 2: A six-year study with multimodal imaging of a presumed unilateral case

Zarei

Mazloumi

Karkhaneh

et al. 2018

Journal of Current Ophthalmology

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“…Outer retinal changes include discontinuity and disruption of the ellipsoid zone (EZ) of the photoreceptors, areas of hyporeflectivity in the outer retina, which some have termed as cavities, and neurosensory retinal atrophy (Charbel Issa et al 2013). Characteristic inner retinal changes are increased reflectivity of the inner neurosensory layer, areas of hyporeflectivity, again termed by some as cavities, and, at later stages, lamellar or full thickness macular holes and pigment migration from outer into inner retina (Wong et al 2009; Sallo et al 2012; Charbel Issa et al 2013; Coscas et al 2013). Macular fundus autofluorescence (FAF) abnormalities have also been described in MacTel (Charbel Issa et al 2013; Balaskas et al 2014).…”

Section: Introductionmentioning

confidence: 99%

Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure

Ledolter

Ristl

Palmowski-Wolfe

et al. 2021

Acta Ophthalmologica

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To assess the impact of neurodegenerative morphologic alterations due to macular telangiectasia type 2 (MacTel) on microperimetry (MP) and multifocal electroretinography (mfERG). Methods: Thirty-five eyes of 18 patients with MacTel were examined using spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), mfERG and MP. Software was used to match SD-OCT B-scans with the corresponding retinal sensitivity map and multifocal electroretinograms (mfERGs), thus enabling direct structure/function correlation. Results: Loss of the ellipsoid zone (EZ) had the strongest negative association with retinal sensitivity (16.77 dB versus 4.58 dB, adj. p < 0.001) of all parameters examined, and a limited negative effect on mfERGs (0.32 SD versus À1.97 SD adj. p = 0.121). Ellipsoid zone (EZ) irregularity was associated with reduced MP values but preserved mfERGs. There was a significant association between areas of inner retinal hyporeflectivity and loss of MP sensitivity (adj. p < 0.001) but the reduction in sensitivity was less than in locations with EZ loss. Areas of mfERG abnormality showed similar sensitivity loss with either inner retinal hyporeflectivity or EZ loss (adj. p = 0.063). In areas with EZ loss alone, preservation of the external limiting membrane (ELM) was associated with higher MP values than in areas with additional ELM loss; the integrity of the ELM alone was not associated with changes either in MP or mfERG. Increased FAF was observed in 51% of eyes, mixed/reduced FAF in 40%, and no abnormality was detected in 9% of eyes. Conclusion:The data suggest both MP and mfERG to be useful non-invasive modalities for detecting localised macular dysfunction in MacTel. The findings suggest a different sensitivity of the two modalities to inner and outer retinal changes in macular function and are therefore complementary.

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“…The introduction of spectral domain optical coherence tomography (SD-OCT) has allowed detailed observation of the structural characteristics, such as cavities in the inner and outer retina, disruption of the ellipsoid zone (EZ), and retinal atrophy, of macular telangiectasia (MacTel) type 2 1 , 2 . However, the pathogenesis of MacTel type 2 has not been well described thus far.…”

Section: Introductionmentioning

confidence: 99%

Characterizing right-angled vessel in macular telangiectasia type 2 with structural optical coherence tomography

Chung

Kim

et al. 2021

Sci Rep

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We investigated the structural findings on spectral-domain optical coherence tomography (SD-OCT) related to the presence of right-angled vessels (RAV) in patients with macular telangiectasia (MacTel) type 2 with severity 3 in Korea. A retrospective multicenter cross-sectional study was conducted in six tertiary hospitals in Korea; the study included 116 MacTel type 2 eyes with severity 3. The SD-OCT findings were compared between eyes with RAV on fundus photography or fluorescein angiography and those without RAV. Logistic regression was performed to determine factors associated with the presence of RAV. Fifty eyes presented with RAV and 61 eyes without RAV. More eyes presented with only inner retinal (IR) cavities on SD-OCT among eyes without RAV than among those with RAV (P < 0.001). However, eyes with RAV presented with IR disorganization, outer retinal (OR) cavity, and ellipsoid zone (EZ) disruption more frequently than eyes without RAV did (all P < 0.001). These SD-OCT findings were significantly associated with the presence of RAV. The presence of RAV was closely related to IR disorganization, OR cavities, and EZ disruption on SD-OCT. These findings suggest an advanced phase of MacTel type 2.

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SD-OCT Stages of Progression of Type 2 Macular Telangiectasia in a Patient followed for 3 Years

Abstract: We describe the 6 steps of progression from earlier SD-OCT findings that led to a complete disorganization and fusion of the inner layers (probably due to changes in the Müller cells) to the typical intraretinal cysts.

Cited by 7 publications

References 7 publications

Idiopathic macular telangiectasia type 2: A six-year study with multimodal imaging of a presumed unilateral case

Idiopathic macular telangiectasia type 2: A six-year study with multimodal imaging of a presumed unilateral case

Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure

Characterizing right-angled vessel in macular telangiectasia type 2 with structural optical coherence tomography

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