Characteristics of fundus image in macular telangiectasia type 2_Chinese Journal of Ocular Fundus Diseases

Authors：

JiYuying , ZhangXiongze , LiMiaoling , PengYuting , YeBaikang , MiLan , LiuBing ,  WenFeng

State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou 510060, China;

Corresponding author：

WenFeng, Email: wenfeng208@foxmail.com

Keywords：

Retinal Diseases/diagnosis; Macula lutea/injuries; Disease attributes

DOI：

10.3760/cma.j.issn.1005-1015.2016.04.009

Video：

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Objective To observe the fundus image characteristics of macular telangiectasia type 2 (MacTel type 2) patients. Methods A total of 8 patients (16 eyes) diagnosed of MacTel type 2 were included in this study. There were 4 males and 4 females, age ranged from 44 to 69 years old with a median age of (59.88±7.85) years. All patients received examination of best-corrected visual acuity (BCVA), slit lamp microscope, indirect ophthalmoscopy, fundus color photography, fundus autofluorescence (AF), fundus fluorescein angiography (FFA), spectral domain optical coherence tomography (OCT) and macular pigment optical density (MPOD). Four eyes of 2 patients received OCT angiography examination at the same time. Classification was made according to the Gass and Blodi's criteria. The follow-up time was from 1 to 19 months with the average time of (11.00±8.91) months. The clinical characteristics were observed and analyzed. Results The BCVA was 0.07-0.8. There were 1 eye in stage 1, 1 eye in stage 2, 6 eyes in stage 3, 8 eyes in stage 4. The disease showed a bilateral appearance with a low progression. Fundus features included loss of retinal transparency (14 eyes, 87.5%), blunted retinal venule (15 eyes, 93.75%), pseudo-lamellar hole (5 eyes, 31.25%), pigment proliferation (9 eyes, 56.25%). FFA findings were telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. Spectral domain OCT features included depletion of the retinal inner, outer structures, cavity (7 eyes, 43.75%), and atrophy of the neurosensory retina (9 eyes,56.25%). On AF, reduced foveal masking due to loss of macular pigment can be observed. The loss of macular pigment could also be seen on MPOD. OCTA showed the increased intervascular spaces, broken regular network of foveal avascular zone (FAZ), right-angled vessel dipping, dilatations, traction of superficial and deep capillary layers in both the superficial and deep layers, especially in the deep layer. Only one eye of one patient proceeded from stage 4 to stage 5. Conclusions Characteristic features of MacTel type 2 included cavities and outer retina atrophy on OCT; loss of MP on MPOD; broken regular network of FAZ, morphological and structural abnormalities of superficial and deep layers of perifoveal capillary network on OCTA.

Citation： JiYuying, ZhangXiongze, LiMiaoling, PengYuting, YeBaikang, MiLan, LiuBing, WenFeng. Characteristics of fundus image in macular telangiectasia type 2. Chinese Journal of Ocular Fundus Diseases, 2016, 32(4): 382-386. doi: 10.3760/cma.j.issn.1005-1015.2016.04.009 Copy

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2.	Charbel IP, Kupitz EH, Heeren TF, et al. Treatment for macular telangiectasia type 2[J]. Dev Ophthalmol,2016,55:189-195.DOI:10.1159/000431263.
3.	Sallo FB, Leung I, Clemons TE, et al. Multimodal imaging in type 2 idiopathic macular telangiectasia[J]. Retina,2015,35(4):742-749.DOI:10.1097/IAE.0000000000000365.
4.	Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis:update of classification and follow-up study[J]. Ophthalmology,1993,100(10):1536-1546.
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6.	Barthelmes D, Gillies MC, Sutter FK. Quantitative OCT analysis of idiopathic perifoveal telangiectasia[J]. Invest Ophthalmol Vis Sci,2008,49(5):2156-2162.DOI:10.1167/iovs.07-0478.
7.	Spaide RF, Klancnik JJ, Cooney MJ. Retinal vascular layers in macular telangiectasia type 2 imaged by optical coherence tomographic angiography[J]. JAMA Ophthalmol,2015,133(1):66-73.DOI:10.1001/jamaophthalmol.2014.3950.
8.	Nowilaty SR, Al-Shamsi HN, Al-Khars W. Idiopathic juxtafoveolar retinal telangiectasis:a current review[J]. Middle East Afr J Ophthalmol,2010,17(3):224-241.DOI:10.4103/0974-9233.65501.
9.	Wu L, Evans T, Arevalo JF. Idiopathic macular telangiectasia type 2(idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2)[J]. Surv Ophthalmol,2013,58(6):536-559. DOI:10.1016/j.survophthal.2012.11.007.
10.	Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis[J]. Arch Ophthalmol,1982,100(5):769-780.
11.	Chidambara L, Gadde SG, Yadav NK, et al. Characteristics and quantification of vascular changes in macular telangiectasia type 2 on optical coherence tomography angiography[J/OL]. Br J Ophthalmol,2016, 2016:E1[2016-01-28]. http://bjo.bmj.com/cgi/pmidlookup?view=long&pmid=26823394. DOI:10.1136/bjophthalmol-2015-307941.[published online ahead of print].
12.	Bone RA, Landrum JT, Hime GW, et al. Stereochemistry of the human macular carotenoids[J]. Invest Ophthalmol Vis Sci,1993,34(6):2033-2040.
13.	Junghans A, Sies H, Stahl W. Macular pigments lutein and zeaxanthin as blue light filters studied in liposomes[J]. Arch Biochem Biophys,2001,391(2):160-164.DOI:10.1006/abbi.2001.2411.
14.	Ahmed SS, Lott MN, Marcus DM. The macular xanthophylls[J]. Surv Ophthalmol,2005,50(2):183-193.DOI:10.1016/j.survophthal.2004.12.009.
15.	Helb HM, Charbel IP, Van Der Veen RL, et al. Abnormal macular pigment distribution in type 2 idiopathic macular telangiectasia[J]. Retina,2008,28(6):808-816.DOI:10.1097/IAE.0b013e31816d81aa.

1. Charbel IP, Gillies MC, Chew EY, et al. Macular telangiectasia type 2[J]. Prog Retin Eye Res, 2013, 34:49-77. DOI:10.1016/j.preteyeres.2012.11.002.
2. Charbel IP, Kupitz EH, Heeren TF, et al. Treatment for macular telangiectasia type 2[J]. Dev Ophthalmol,2016,55:189-195.DOI:10.1159/000431263.
3. Sallo FB, Leung I, Clemons TE, et al. Multimodal imaging in type 2 idiopathic macular telangiectasia[J]. Retina,2015,35(4):742-749.DOI:10.1097/IAE.0000000000000365.
4. Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis:update of classification and follow-up study[J]. Ophthalmology,1993,100(10):1536-1546.
5. Clemons TE, Gillies MC, Chew EY, et al. Baseline characteristics of participants in the natural history study of macular telangiectasia (MacTel) MacTel Project Report No. 2[J]. Ophthalmic Epidemiol,2010,17(1):66-73.DOI:10.3109/09286580903450361.
6. Barthelmes D, Gillies MC, Sutter FK. Quantitative OCT analysis of idiopathic perifoveal telangiectasia[J]. Invest Ophthalmol Vis Sci,2008,49(5):2156-2162.DOI:10.1167/iovs.07-0478.
7. Spaide RF, Klancnik JJ, Cooney MJ. Retinal vascular layers in macular telangiectasia type 2 imaged by optical coherence tomographic angiography[J]. JAMA Ophthalmol,2015,133(1):66-73.DOI:10.1001/jamaophthalmol.2014.3950.
8. Nowilaty SR, Al-Shamsi HN, Al-Khars W. Idiopathic juxtafoveolar retinal telangiectasis:a current review[J]. Middle East Afr J Ophthalmol,2010,17(3):224-241.DOI:10.4103/0974-9233.65501.
9. Wu L, Evans T, Arevalo JF. Idiopathic macular telangiectasia type 2(idiopathic juxtafoveolar retinal telangiectasis type 2A, Mac Tel 2)[J]. Surv Ophthalmol,2013,58(6):536-559. DOI:10.1016/j.survophthal.2012.11.007.
10. Gass JD, Oyakawa RT. Idiopathic juxtafoveolar retinal telangiectasis[J]. Arch Ophthalmol,1982,100(5):769-780.
11. Chidambara L, Gadde SG, Yadav NK, et al. Characteristics and quantification of vascular changes in macular telangiectasia type 2 on optical coherence tomography angiography[J/OL]. Br J Ophthalmol,2016, 2016:E1[2016-01-28]. http://bjo.bmj.com/cgi/pmidlookup?view=long&pmid=26823394. DOI:10.1136/bjophthalmol-2015-307941.[published online ahead of print].
12. Bone RA, Landrum JT, Hime GW, et al. Stereochemistry of the human macular carotenoids[J]. Invest Ophthalmol Vis Sci,1993,34(6):2033-2040.
13. Junghans A, Sies H, Stahl W. Macular pigments lutein and zeaxanthin as blue light filters studied in liposomes[J]. Arch Biochem Biophys,2001,391(2):160-164.DOI:10.1006/abbi.2001.2411.
14. Ahmed SS, Lott MN, Marcus DM. The macular xanthophylls[J]. Surv Ophthalmol,2005,50(2):183-193.DOI:10.1016/j.survophthal.2004.12.009.
15. Helb HM, Charbel IP, Van Der Veen RL, et al. Abnormal macular pigment distribution in type 2 idiopathic macular telangiectasia[J]. Retina,2008,28(6):808-816.DOI:10.1097/IAE.0b013e31816d81aa.

Chinese Journal of Ocular Fundus Diseases

Characteristics of fundus image in macular telangiectasia type 2

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