Oman Journal of Ophthalmology

CLINICAL QUIZ
Year
: 2014  |  Volume : 7  |  Issue : 2  |  Page : 102-

Progressive Painless Loss of Vision - What is the diagnosis?


Radha Shenoy, Badar Al Barwani, Rashid Al Saidi 
 Department of Ophthalmology, Armed Forces Hospital, Oman

Correspondence Address:
Radha Shenoy
Department of Ophthalmology, Armed Forces Hospital
Oman




How to cite this article:
Shenoy R, Al Barwani B, Al Saidi R. Progressive Painless Loss of Vision - What is the diagnosis?.Oman J Ophthalmol 2014;7:102-102


How to cite this URL:
Shenoy R, Al Barwani B, Al Saidi R. Progressive Painless Loss of Vision - What is the diagnosis?. Oman J Ophthalmol [serial online] 2014 [cited 2019 Aug 22 ];7:102-102
Available from: http://www.ojoonline.org/text.asp?2014/7/2/102/137176


Full Text

A 55-year-old hypertensive lady presented to the clinic with progressive Painless loss of vision in both eyes. On examination the best corrected visual acuity in both eyes was 1/60. Slit lamp examination showed normal anterior segment. Fundus examination showed CDR of 0.3, blood vessels was normal with dull or distorted foveal reflex surrounded by a grey zone in both eyes [Figure 1]. An initial diagnosis of Age-Related Macular Degeneration (dry type) was made. However fundus fluorescein angiography and optical coherence tomography ( OCT ) findings were not consistent with the diagnosis of Age related macular degeneration and optical coherence tomography (OCT) findings [Figure 2], [Figure 3], [Figure 4].{Figure 1}{Figure 2}{Figure 3}{Figure 4}

 Questions



What is the possible diagnosis?What are the different groups? To which group do you think that our patient belongs?What do you see on the Fundus Fluorescein angiogram?What are the salient and distinguishable features (Clinical/Fluorescein angiographic and or Optical coherence tomographic) particular to our patient 's group?What do you see in the OCT of the macula?What salient features on OCT aid in the diagnosis of the particular group of our patient?Name the most specific, and sensitive test that would have aided in early diagnosis in our patientName two other new tests that can aid in early diagnosis other than Fluorescein angiogram and OCT.

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 Answers



Idiopathic juxtafoveolar telangiectasis (IJFT).Clinically 3 Groups-Group I, II, and III. Our patient belongs to Group II.Fundus Fluorescein angiogram both eyes demonstrating early hyperfluorescence followed by diffuse retinal staining in the late phase corresponding topographically to the retinal graying with sparing of the central fovea.Three key and distinguishing features of Group II IJFT Absence of prominent retinal aneurysms or hemorrhage on clinical evaluationAbsence of cystic macular edema or lipid exudation (unless SRNV has developed)The presence of foveolar atrophy, best seen with OCT simulating a lamellar macular hole.Optical Coherence Tomography of macula in our patient shows thinning and atrophy of retinal layers at the fovea with cyst like spaces.OCT features of macula are the following: Blunting of foveal pitThinning and disruption of the photo receptor layerFoveaolar atrophy-simulating a lamellar macular holeCyst like structures in the foveola and inner retinal layers (cystoids)Intraretinal hyper reflective lesions or hyperpigmented retinal pigment epithelial plaques causing nonspecific posterior shadowing.Fundus Auto Fluorescence Increase fundus auto fluorescence signal from fovea due to lack of luteal pigment occurs earlier than the fluorescein angiographic findings and is the most sensitive and specific finding in IJFT II.(a) Confocal blue reflectance imaging (at 488 nm) and (b) Macular Pigment Optical Density.

IJFT, also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups are identified. Group I is unilateral, with easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Photocoagulation is generally effective in controlling the macular edema. Group II, the most common, is bilateral occurring in both middle aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is progressive and primarily due to retinal atrophy, not exudation. More rapid visual loss can occur with sub retinal neo vascularization. Treatment options for this group are still very limited, and have shown effectiveness only for the subretinal neovascular component. This is primarily because the pathogenesis of this telangiectasis remains an enigma and is possibly secondary to a retinal neuronal dysfunction. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. It is poorly understood because of the scarcity of cases.