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Parafoveal telangiectasia-- AJAY DUDANI
- 1. Parafoveal Telangiectasia Prof. Ajay Dudani - Mumbai Retina Centre - K.J Somaiya College
- 2. Terminologies and description 2 Idiopathic juxtafoveolar telangiectasis idiopathic parafoveal telangiectasis Macular telangiectasia Perifoveal telangiectasis Incompetence, ectasia, and/or irregular dilations of the capillary network affecting only the juxtafoveolar region of one or both eyes Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 3. Classification: Gass-Blodi Model to Now. 3 NOW Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 4. 4 • (A) Color fundus photograph of an eye with IJFT I. • Corresponding early (B) and late (C) fluorescein angiogram showing easily visible telangiectatic vessels and aneurysmal capillary dilations straddling the horizontal raphe causing late leakage. • (D) Optical coherence tomography scan of an eye with IJFT I showing increased central retinal thickness, intraretinal fluid-filled spaces, and subretinal fluid Congenital or developmental form of IJFT: Group 1: Aneursymal Telangiectasia Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 5. Classification: Gass-Blodi Model to Now. 5 NOW Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 6. Group II A: Perifoveal Telangiectasia • The most common type • Different from Group I. • Acquired, not congenital. • Bilateral, but may be asymmetric appearing as unilateral in its early stages. 6 Category Description 0 Normal results on all imaging methods (fellow eyes) 1 Mild increased foveal autofluorescence on FAF; no other abnormalities 2 Mild-to-moderate increased foveal autofluorescence + funduscopic and angiographic features of IJFT IIA. No atrophic or cystic abnormalities on OCT imaging. No MP deficits 3 Moderate to marked increased foveal autofluorescence + funduscopic and angiographic features of IJFT IIA + foveal atrophy and cysts on OCT + centrally decreased retinal sensitivity on MP 4 Mixed patterns of increased and decreased FAF signal + clinically evident pigment clumping + central outer retinal atrophy on OCT + scotomas on MP correlating with decreased FAF signal or retinal atrophy on OCT Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 7. Group II: Stage I 7 A) A : Color fundus photograph of an eye with JFT IIA Stage 1 showing a loss of transparency of the temporal parafoveolar retina. B) (B and C) Corresponding fluorescein angiogram showing early discrete staining of the temporal parafoveolar capillaries (B), followed by late retinal staining (C). C) Note the absence of clearly visible telangiectasis Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 8. 8 Group II: Stage 2 • A) Color fundus photograph of an eye with JFT IIA Stage 2 showing a mild parafoveolar retinal graying in a ring configuration that spares the foveal center. The latter appears darker and thinner. Note the absence of visible telangiectasis. • (B and C) Fluorescein angiogram of the same eye demonstrating early hyperfluorescence corresponding topographically to the retinal graying (B), followed by diffuse retinal staining in the late phase (C). • Note the central foveal sparing Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 9. 9 Group II: Stage 3 A) A: Color fundus photograph of an eye with IJFT IIA Stage 3 showing a grayish ring around the foveal center with numerous superficial retinal crystals and a slightly dilated right-angled venule temporally. B) (B and C) Corresponding fluorescein angiogram showing in the early phase clearly visible dilation and telangiectasis of the perifoveolar capillary network beneath the right-angled venule (B). These capillaries cause late intraretinal staining (C) Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 10. Group II: Stage 4 10 Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 11. Group II: Stage 5 11 (A) A: Color fundus photograph of an eye with IJFT IIA Stage 5 (proliferative stage) showing temporal parafoveal retinal elevation with subretinal fluid, mild subretinal lipid exudation, and subretinal blood characteristic of the onset of subretinal neovascularization. Note the superficial refractile crystals nasally. (B) B and C: Corresponding fluorescein angiogram featuring subretinal neovascualirization, temporal to the foveal center, that is rapidly hyperfluorescent in the early stage (B) increasing in fluorescence and leaking intensely in the late phase (C). Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 12. 12 Group IIA: OCT scan Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 13. Treatment Modalities • 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. 13 PDT IVTA PDT+ANTI- VEGF ANTI-VEGF ? Middle East Afr J Ophthalmol. 2010 Jul-Sep; 17(3): 224–241
- 14. Case 1: PDT vs Ranibizumab • A 60-year-old diabetic man presented with a history of decrease in vision in both eyes since 2 weeks. At presentation, best corrected visual acuity (BCVA) in the right eye (RE) was 20/30 and that in the left eye (LE) was 20/80. • The right fundus revealed a grayish reflex, yellowish crystalline deposits and retinal pigment epithelial hyperplasia at the macula. The left fundus showed subretinal fluid and temporal subretinal hemorrhage near a grayish reflex at the macula. • A diagnosis in both eyes of idiopathic macular telangiectasia (IMT) type 2A with RE stage 4 and LE stage 5, choroidal neovascularization (CNVM) was made. The patient was treated with photodynamic therapy (PDT) in LE. The visual acuity improved to 20/40 over the next 6 months. • At a 4-year follow-up, he developed decreased vision in RE diagnosed as IMT with CNV and was treated with intravitreal ranibizumab. At 6-month follow-up post injection, the vision was 20/40p 14 Indian J Ophthalmol. 2013 Jul; 61(7): 353–355.
- 15. 15 Left eye fundus photograph showing stage 5 idiopathic macular telangiectasia (IMT) confirmed on optical coherence tomogram (OCT) and fundus fluorescein angiography (FFA) Indian J Ophthalmol. 2013 Jul; 61(7): 353–355.
- 16. 16 Resolution of the choroidal neovascular membrane (CNVM) post photodynamic therapy (PDT) therapy Indian J Ophthalmol. 2013 Jul; 61(7): 353–355.
- 17. 17 Right eye fundus photograph showing stage 5 idiopathic macular telangiectasia (IMT) confirmed on optical coherence tomogram (OCT) and fundus fluorescein angiography (FFA) Indian J Ophthalmol. 2013 Jul; 61(7): 353–355.
- 18. 18 Resolution of the choroidal neovascular membrane (CNVM) post intravitreal ranibizumab Indian J Ophthalmol. 2013 Jul; 61(7): 353–355.
- 19. Case 2: Bilateral IPT type 1 and unilateral type 1 treated with Ranibizumab • Four eyes of three patients were included in this interventional case series. • One patient (two eyes) had bilateral IPT (type 2) and two patients (two eyes) had unilateral (type 1) IPT. • Retreatment was scheduled in case of leakage persistence in combination with visual acuity (VA) deterioration. • Fluorescein angiography and optical coherence tomography were performed together with a full ophthalmic examination at baseline, 1, 3, 6, 9, and 12 months after injection. 19 Clinical Ophthalmology 2013:7 1357–1362
- 20. Demographics 20 Clinical Ophthalmology 2013:7 1357–1362
- 21. 21 Clinical Ophthalmology 2013:7 1357–1362
- 22. 22 Clinical Ophthalmology 2013:7 1357–1362
- 23. 23 Clinical Ophthalmology 2013:7 1357–1362
- 24. Summary • Use of ranibizumab might be efficient in eliminating leakage activity in the macular region in patients with IPT. • Nevertheless, improvement in VA was infrequent. • Preexisting early photoreceptor alteration in IPT might render such patients unable to improve VA 24 Clinical Ophthalmology 2013:7 1357–1362
- 25. Case 3: • A 65-year-old lady presented with decreased vision in her left eye (LE). • Best corrected visual acuity (BCVA) was 1/20. • Complete examination showed idiopathic juxtafoveal retinal telangiectasis associated with subretinal neovascularization and she was treated with intravitreal ranibizumab every month for three months in the LE. After four months, her BCVA increased to 3/10. • Fluorescein angiography (FA) showed minimal leakage and optical coherence tomography (OCT) confirmed absence of intra- or subretinal fluid in the macula. • Examinations were repeated monthly for another 12 months and showed no recurrence. Intravitreal ranibizumab showed promising results for subretinal neovascularization due to idiopathic juxtafoveal retinal telangiectasis. • A prospective study with large series of patients and controls may be necessary in order to determine the effectiveness of this treatment. 25 Clinical Interventions in Aging 2009:4 63–65
- 26. 26
- 27. 27 A) Oblique section of OCT of the LE revealing the characteristic appearance of outer and inner retina having similar reflectivity and an area temporal to the fovea with high reflectivity corresponding to the temporal area of choroidal neovascularization observed in the angiogram. B) OCT of the same section of LE revealing signifi cant reduction in retinal thickness. The RPE remains thickened.
- 28. 28 A) Left eye: Color fundus picture following ranibizumab treatment revealing signifi cant improvement with no subretinal hemorrhage. B) late venous phase of FA of the LE showing minimal leakage due to telangiectasis but no signs of active neovascular membrane.
- 29. Key Highlights • Currently, there is no established treatment for type 1 IMT, although retinal photocoagulation has been successful in some cases, reducing the lipid exudates but not always improving vision. • VEGF stimulates angiogenesis, increases vascular permeability, and is implicated in the formation of abnormal blood vessels in type 2 IMT, which may be the plausible reason for Anti-VEGF treatment • As for group III, it is featured primarily as a perifoveolar capillary occlusive condition, and is poorly understood because of the scarcity of cases reported. 29
- 30. Thank you
Editor's Notes
- Idiopathic juxtafoveolar telangiectasis (also known as idiopathic parafoveal, perifoveal or macular telangiectasia or telangiectasis) is a descriptive term for various disease entities presenting with incompetence, ectasia, and/or irregular dilations of the capillary network affecting only the juxtafoveolar region of one or both eyes. These entities are distinguished from more generalized retinal telangiectasis (such as in Coats’ disease) or secondary juxtafoveal retinal telangiectasis due to retinal vein occlusion, diabetes, irradiation, or carotid artery obstruction
- The term idiopathic juxtafoveolar retinal telangiectasis (IJFT) was coined by Gass and Oyakawa1 in 1982, who proposed the first classification of these entities into four groups based largely on their clinical and fluorescein angiographic (FA) features. In 1993, Gass and Blodi2 further updated this classification, by subdividing IJFT into three distinct groups I, II, and III (also known as groups 1, 2, and 3), with two subgroups in each (A and B), based on demographic difference or clinical severity. More recently, based on newly recognized clinical, angiographic, and optical coherence tomography (OCT) imaging observations, Yannuzzi et al.3 proposed a simplified classification of IJFT, essentially a revision and simplification of the Gass–Blodi model. They proposed the term “idiopathic macular telangiectasia” with two distinct types: Type 1 or “aneurysmal telangiectasia” equivalent to IJFT group I (A and B combined), which is the second most common form of IJFT; and type 2 or "perifoveal telangiectasia" equivalent to IJFT group IIA, the most common type of IJFT. The remaining types described by Gass and Blodi (group IIB and groups IIIA and B) were omitted from Yannuzzi’s classification because of their rarity.
- The term idiopathic juxtafoveolar retinal telangiectasis (IJFT) was coined by Gass and Oyakawa1 in 1982, who proposed the first classification of these entities into four groups based largely on their clinical and fluorescein angiographic (FA) features. In 1993, Gass and Blodi2 further updated this classification, by subdividing IJFT into three distinct groups I, II, and III (also known as groups 1, 2, and 3), with two subgroups in each (A and B), based on demographic difference or clinical severity. More recently, based on newly recognized clinical, angiographic, and optical coherence tomography (OCT) imaging observations, Yannuzzi et al.3 proposed a simplified classification of IJFT, essentially a revision and simplification of the Gass–Blodi model. They proposed the term “idiopathic macular telangiectasia” with two distinct types: Type 1 or “aneurysmal telangiectasia” equivalent to IJFT group I (A and B combined), which is the second most common form of IJFT; and type 2 or "perifoveal telangiectasia" equivalent to IJFT group IIA, the most common type of IJFT. The remaining types described by Gass and Blodi (group IIB and groups IIIA and B) were omitted from Yannuzzi’s classification because of their rarity.
- Affected patients are middAcquired, not congenital. This disorder is bilateral, but may be asymmetric appearing as unilateral in its early stages.2 Similarly, patients may have visual loss in only one eye. le-aged or older (mean 55 years). Males and females are affected equally.
- Serial color fundus pictures of the right eye (A-C) and left eye (D-F) of a patient with JFT IIA. (A) Early Stage 4 depicting intraretinal pigment epithelial deposition in the vicinity of the temporal right-angled venule. (B and C) Over a period of 5 years, a progressive increase in the number and size of the intraretinal pigment clumps was observed. The clumps are located preferentially close to the nearby parafoveal vessels. Note the increasing superficial refractile crystals over time, and the central foveal atrophy which is more clearly seen in (C). In the left eye, which was at an earlier stage (Stage 2 in D) similarly showed increasing intraretinal pigment epithlelial migration over time (Stage 4 in E and F). This example illustrates that IJFT II can be asymmetric. Note how the foveal depression simulates a macular hole in this eye (D)
- Note that the capillary telangiectasis is also easily visible at this stage of the disease (nasally), demonstrating early capillary wall staining and late intraretinal staining that differs in character form the more intense late hyperfluorescent leakage of the subretinal neovascularization
- (A) Optical coherence tomogrpahy scan at the fovea demonstrating a small inner lamellar retinal cyst “cystoid” (arrow) typical of IJFT IIA. Note the absence of retinal thickening or fluid-filled spaces. (B) Optical coherence tomogrpahy scan demonstrating an intraretinal hyper-reflective area (arrow) that corresponds to an intraretinal pigment epithelial plaque which causes posterior shadowing
- Right eye of Patient 1. Fluorescein angiograms and optical coherence tomography images of each visit are presented. (A, D, G, J) show early phase fluorescein angiograms of baseline and at the 1st, 6th and 12th month. The telangiectatic capillaries in the perifoveal area (A) subside significantly postintervention. (B, E, H, K) Late phase fluorescein angiograms of the same eye. Leakage/staining in the perifoveal area (B) corresponds to the telangiectatic capillaries of image A. (C, F, I, L) Optical coherence tomography images of each visit detecting severe alterations in photoreceptor layer at inner/outer segments.
- Left eye of Patient 1. As in the right eye (Figure 1), early phase fluorescein angiograms (A, D, G, J) again detect the telangiectatic capillaries of the perifoveal area during follow-up. Leakage/staining in the perifoveal area in late phase (B, baseline) has ceased after treatment (E, H, K). Optical coherence tomography images again detect the defects of inner/outer segments. Additionally, a hyporeflective space in the fovea center (C) represents a loss of retinal tissue and remains almost stable throughout the repeated exams (F, I, L).
- Left eye of Patient 2. Fluorescein angiograms of the early phase (A, D, G, J) show similar findings to those in Figures 1 and 2. In late phase images, cessation of leakage was documented, as focal hot spots have faded within the entire staining area (B, E, H, K). However, this staining was evident throughout follow-up with slight leakage relapsing by the last visit (K). In optical coherence tomography images, structural changes of the photoreceptors inner/outer segments are a consistent finding (C, F, I, L).
- Right eye: Color fundus picture showing slightly dilated right-angle veins with retinal pigment epithelium hyperplasia beneath them. B) Left eye: Color fundus picture showing subretinal hemorrhage inferior and temporal to the fovea. C) Venous phase of FA of the LE depicts early hyperfl uorescence corresponding to the neovascular membrane and hypofl uorescence inferior to the membrane corresponding to the subretinal hemorrhage. D) Late phase of FA showing leakage from the neovascular membrane.
- Optical coherence tomography and fluorescein angiography images obtained from patient #3 at presentation and at 1 month after intravitreal bevacizumab injection. Average age of the patients (4 female and 1 male) was 62±11.8 years. Average follow-up period was 26±11 months. Patients received an average of 2.3 (range 1-4) injections during follow-up. Average Snellen BCVA of the patients was 0.48±0.29. BCVA increased at final examination compared to baseline in all of the patients. The difference between baseline and final visual acuities was significant (p<0.05). The patients’ average CMT was 328±139 μm at baseline and decreased by a mean of 85±153 μm at 1 month after the first injection and 65±142 μm at final examination, but the changes were not significant. CMT decreased at final examination compared to baseline in four patients and increased in both eyes of one patient