|Year : 2013 | Volume
| Issue : 3 | Page : 183-187
Role of focal laser ablation in the treatment of type 1 idiopathic juxtafoveolar retinal telangiectasis
Department of Ophthalmology, Faculty of Medicine, Menoufia University, Menoufia, Egypt
|Date of Submission||21-Jan-2013|
|Date of Acceptance||24-Mar-2013|
|Date of Web Publication||28-Feb-2014|
Menoufia University, 13-32111, Menoufia
Source of Support: None, Conflict of Interest: None
Idiopathic juxtafoveolar retinal telangiectasis (IJRT) is a retinal vascular anomaly characterized by irregular dilation and incompetence of retinal vessels. Although the etiology of the condition is unknown, its various presentations have been characterized into three groups. Treatment is based mainly on disease classification and includes laser photocoagulation and observation besides other modalities.
The aim of the work was to study the role of focal laser treatment for type 1 IJRT.
Patients and methods
A retrospective noncomparative study was carried out at the Ophthalmology Department, Menoufia University, Egypt, during the period from January 2007 to December 2011, wherein four patients (three male and one female) diagnosed with type 1 (exudative) IJRT on the basis of fluorescein angiography and after ruling out other causes were included in the study. Four eyes with macular edema and exudation were subjected to focal laser treatment (double-frequency YAG) with green laser (wavelength 532 nm). The follow-up period after laser treatment extended from 6 to 24 months.
Four patients diagnosed with type 1 IJRT were included in the study (three male and one female). Their age ranged from 40 to 42 years, with a mean age of 40.75 years. Focal double-frequency YAG green laser (wavelength 532 nm) treatment resulted in the reduction of both macular edema and exudation with improvement in visual acuity from one to two lines on follow-up.
Focal laser treatment of type 1 IJRT lesions proved to be effective. The treatment is a safe method for managing leakage and stabilizing vision.
Keywords: Focal laser photocoagulation; idiopathic juxtafoveolar retinal telangiectasis; retinal vascular disorders
|How to cite this article:|
Elmazar H. Role of focal laser ablation in the treatment of type 1 idiopathic juxtafoveolar retinal telangiectasis. J Egypt Ophthalmol Soc 2013;106:183-7
|How to cite this URL:|
Elmazar H. Role of focal laser ablation in the treatment of type 1 idiopathic juxtafoveolar retinal telangiectasis. J Egypt Ophthalmol Soc [serial online] 2013 [cited 2020 Feb 21];106:183-7. Available from: http://www.jeos.eg.net/text.asp?2013/106/3/183/127390
| Introduction|| |
Idiopathic juxtafoveolar retinal telangiectasis (IJRT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia (IMT), 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 .
The term IJRT was coined by Gass and Oyakawa 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 Blodi  further updated this classification, by subdividing IJRT 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 differences or the clinical severity. Each main group had a presumed independent etiology. Despite its complexity, the Gass and Blodi classification is the most commonly used to date ,.
More recently, on the basis of newly recognized clinical, angiographic, and optical coherence tomography (OCT) imaging observations, Yannuzzi et al.  proposed a simplified classification of IJRT, essentially a revision and simplification of the Gass and Blodi model. They proposed the term IMT with two distinct types: type 1 or aneurysmal telangiectasia equivalent to IJRT group I (A and B combined), which is the second most common form of IJRT, and type 2 or 'perifoveal telangiectasia' equivalent to IJRT group IIA, the most common type of IJRT. The remaining types described by Gass and Blodi (group IIB and groups IIIA and IIIB) were omitted from the Yannuzzi classification because of their rarity. They furthermore simplified the five stages of group IIA proposed by Gass and Blodi into two distinct stages that have clinical, therapeutic, and prognostic relevance: the nonproliferative and the proliferative stages .
Type 1 (exudative) IJRT is a congenital or developmental form of IJRT that occurs predominantly in men and is typically unilateral (97% of cases). Although the onset of symptoms can occur at any age, the mean age at presentation is 40 years. On biomicroscopy, prominent easily visible telangiectatic retinal capillaries, with variable-sized aneurysmal dilations, are a consistent hallmark of this type of IJRT. The telangiectasis usually involves an area covering two disc diameters or greater in the temporal to the foveal region. Macular edema and lipid deposition of variable amounts is a characteristic feature. Of note, no blunted right-angled venules, superficial vitreoretinal interface crystalline deposits, plaques of pigment epithelial hyperplasia, intraretinal pigment migration or subretinal neovascularization (SRNV) are seen in this type of IJRT. The median visual acuity (VA) at presentation is 20/40 in Gass series. Some patients retain excellent VA for years without treatment. The vascular malformations may function normally for years and then progress to a pathological state later in life. In some cases, spontaneous resolution may occur ,. If progressive visual loss occurs, however, treatment with laser photocoagulation may be effective in reducing the exudation and improving or stabilizing vision .
Type 2 (nonexudative) is the most common type of IJRT, and it differs completely from IJRT I. It is acquired not congenital. Affected patients are middle-aged or older (mean age 55 years). Men and women are affected equally. This disorder is bilateral, but may be asymmetric appearing as unilateral in its early stages. Similarly, patients may have visual loss in only one eye. The natural course of IJRT IIA has been subdivided by Gass and Blodi into five stages. Although this staging has been simplified recently, the main observations remain identical. In stage 1, patients are generally asymptomatic. A slight loss of the retinal transparency, typically grayish and usually in the temporal juxtafoveolar area, where this condition most often starts, may be the only biomicroscopic clue to the presence of the telangiectasis. In stage 2, patients may be asymptomatic or have minimal disturbances in central vision such as blurred vision, metamorphopsia, or paracentral positive scotoma. A slight graying of the parafoveolar retina approximately one disc diameter in size, confined temporally or forming a partial or complete horizontal oval around the foveal center, exists. In stage 3, patients may experience decreased vision, which is slow in onset and progression. Paracentral vertically oriented slightly dilated right-angled venules draining the telangiectatic area are evident biomicroscopically. In stage 4, as a result of retinal pigment epithelium (RPE) migration into the retina along the course of the right-angled vessels, one or more loci of black retinal pigmented epithelial hyperplasia or clumps may be seen around the parafoveolar right-angled vessels. Finally, stage 5 is marked by the onset of SRNV, which occurs as a result of retinal capillary remodeling, proliferation, and invasion of the outer retina, which has progressively atrophied ,.
The five stages were simplified into two distinct stages that have clinical, prognostic, and therapeutic implications: the nonproliferative stage (stages 1-4), characterized by telangiectasis and foveal atrophy without SRNV, and the proliferative stage (stage 5 of Gass and Blodi) defined by the advent of SRNV and fibrosis .
The key distinguishing features of IJRT IIA are as follows:
- The absence of prominent aneurysms or hemorrhage.
- The absence of cystic macular edema or lipid exudation (unless SRNV has developed). The loss of retinal transparency and fluorescein staining are primarily caused by intracellular edema (in contrast to the extracellular fluid causing cystoid macular edema (CME) and lipid exudation in IJRT I).
- The presence of foveolar atrophy, best seen with OCT, which can simulate a lamellar macular hole. Foveal atrophy is the primary cause of the slow progressive visual loss occurring over years in these patients (to 20/200 or worse), distinguishable from the rapid and severe visual loss that may occur with the advent of SRNV and fibrosis .
Gass and Blodi noted that the long-term prognosis for patients with IJRT IIA is poor, and laser treatment resulted in either a worsening or no change in VA. Hence, they did not recommend argon laser photocoagulation in nonproliferative IJRT . Park et al.  also found no improvement or stabilization of vision with grid argon laser photocoagulation. In addition, treatment was associated with retinal pigment epithelial changes: post-treatment retinal hemorrhages, vascularized retinal scars, and increased retinal vascular distortion .
Different treatment options have been investigated by many authors, including intravitreal injection of steroids or anti-vasogenic substances [anti-vascular endothelial growth factor (anti-VEGF)], the application of photodynamic therapy, transpupillary thermotherapy, or surgical removal of neovascular membranes. Current preliminary results suggest that intravitreal delivery of anti-VEGF therapy combined with or without photodynamic therapy appears efficacious and should be considered as a treatment option for proliferative IJRT IIA. However, long-term follow-up and larger series are needed to address the long-term outcomes, the needed frequency of anti-VEGF drug delivery, and specific side effects or complications of anti-VEGF therapy in this condition. It is still unclear whether anti-VEGF injections would be of value in the treatment of nonproliferative IJRT IIA. Given the current lack of convincing evidence of efficacy, the concern about the potential deleterious effects of repeated injections, including endophthalmitis, and the cost of treatment, continued therapy of nonproliferative IJRT IIA with VEGF antagonists appears, at this time, questionable .
Type 3 (occlusive) is a rare form of IJRT described by Gass characterized by progressive bilateral perifoveal capillary obliteration, capillary telangiectasis, and minimal exudation clinically and on FA leading to visual loss in association with systemic or cerebral familial disease ,.
| Aim of the work|| |
The aim of the work was to study the role of focal laser treatment for type 1 IJRT.
| Patients and methods|| |
After obtaining the necessary approval from the ethics committee, a retrospective noncomparative study was carried out at the Ophthalmology Department, Menoufia University, Egypt, during the period from January 2007 to December 2011, wherein four eyes from four patients (three male and one female) diagnosed with type 1 (exudative) IJRT based on FA and after ruling out other causes were included in the study.
Eyes were found to have a significant visual dysfunction due to macular edema and exudation. Focal laser treatment with (double-frequency YAG) green laser (wavelength 532 nm) was carried out as follows:
- After obtaining the patient history and performing medical and ophthalmological examinations, fundus fluorescein angiography (FFA) was conducted and a discussion was carried out with the patient about the condition and the possible treatment options.
- Patients were informed about the benefits and risks of laser therapy and a written consent was obtained from all patients.
- The affected eye was prepared by applying both a local anesthetic and pupillary dilatation eye drops; a Goldman three mirror lens was fitted, and patients were instructed to look forward.
- Double-frequency YAG laser (wavelength 532 nm) was applied to induce photocoagulation in the leaking areas on the basis of the FA findings with a spot size of 100 mm, a power of 100-200 mW, and a duration of 0.1 s aiming to induce minimal reaction (one or two shots were applied first in the peripheral retina to insure minimal reaction before applying the laser to the macular area).
- The leaking areas were then subjected to focal laser shots to induce photocoagulation to the abnormal vessels on the basis of FA results.
- Patients were advised to return for follow-up on the next day, and after 1 week, 1 month, and every 6 months thereafter, when visual assessment and clinical examinations were carried out.
- Colored fundus photography and FA were performed after 1 month, 6 months, and when necessary.
| Results|| |
Four patients were included in the study (three male and one female); their age ranged from 40 to 42 years, with a mean age of 40.75 years. The diagnosis was based on clinical examination, colored fundus photography, and FFA as shown for patient number 1 [Figure 1]a-d. The follow-up period after laser treatment extended from 6 to 24 months, with a mean of 17 months.
Focal double-frequency YAG green laser (wavelength 532 nm) was applied to the affected eyes, aiming to induce photocoagulation to the abnormal vessels, and visual improvement was noticed at the 6-month follow-up in all treated eyes [Table 1]. Reduction in both macular edema and exudation was recorded with colored fundus photography and FFA on follow-up as shown for patient number 1 before and after laser treatment [Figure 2]a-d.
|Table 1: Laser parameters and visual acuity of the treated eyes before and after (6 months) treatment|
Click here to view
| Discussion|| |
Four cases diagnosed with type 1 IJRT were included in the study (three male and one female; male to female ratio 3 : 1); all were adult patients with a mean age of 40.75 years with no evidence of systemic or vascular diseases, and this agreed with Abujamra et al. , who studied a series of eight cases from group I IJRT and 11 cases from group II and concluded that men were affected two times more often than women in group I and women were affected four times more often than men in group II and added that all patients in group I presented with unilateral diseases .
The diagnosis was based on clinical examination and colored and fluorescein fundus photography, and the presence of easily seen telangiectasis, lipid deposits, and cystic macular edema were the key for diagnosis, which agreed with Nowilaty et al.  who mentioned that macular edema and lipid deposition of variable amount is a characteristic feature in type 1 IJRT .
FA findings in IJRT were also reported by many authors as mentioned by Abujamra et al. , who reported that on the basis of FA in group IA IJRT, the main affected macular quadrant was the inferior temporal region, followed by the inferior nasal region. In group IIA IJRT, all eyes had inferior and superior temporal macular quadrant involvement . Also, Mansour and Schachat  reported that IJRT is characterized by temporal telangiectasias and small foveal avascular zone (FAZ).
The site of telangiectatic vessels in relation to FAZ was variable, and this agreed with Narayanan et al.  who studied the distance between FAZ and telangiectasias in 21 patients and concluded that telangiectasia may be seen farther from the parafoveal area, as the mean distance from the center of the FAZ up to which telangiectasia could be observed was 1340 ± 400 μm and the maximum distance from the FAZ at which telangiectasia was identified was 2530 μm . Nowilaty et al.  mentioned that in type 1 IJRT, focal vascular changes may also develop in the mid-peripheral fundus and even in the more anterior fundus.
In contrast, many investigators described the role of OCT in the diagnosis of IJRT and concluded that OCT findings provide additional criteria for the diagnosis of IJRT ,.
The beneficial effect of laser therapy in IJRT remains questionable and many authors investigated the effect of laser photocoagulation on type 1 IJRT and some of them reported a beneficial effect on this group of patients ,,,.
Kiraly and Nasemann  in their study of long-term follow-up of six cases of IJRT treated with argon laser coagulation concluded that the patients did not show a better visual outcome after laser coagulation and that because of the relatively benign spontaneous course of IJRT, laser coagulation cannot be recommended in general, and they added that laser coagulation can be of benefit only in exceptional cases (e.g. development of SRNV) .
However, a study conducted by Stoffelns et al.  to evaluate the effectiveness of laser photocoagulation for IMT concluded that laser photocoagulation was able to achieve a visual improvement in IMT type 1, but a laser photocoagulation indication should be considered very carefully in IMT type 2, because in this group no visual improvement was reached and a secondary induction of subretinal neovascular membranes seems likely .
In this study, laser treatment applied to treat four eyes with type 1 IJRT was found to be effective in improving visual acuity and reducing macular edema and exudation, and this may be related to the exudative nature of the disease in this group of patients as mentioned by Gass and Blodi who reported that the telangiectasis appears to be caused primarily by retinal capillary leakage in group I, capillary diffusion abnormalities in group II, and capillary occlusion in group III, and added that photocoagulation is probably beneficial for patients in group I and not for patients in group II, at least before their development of SRNV .
The effect of laser treatment on patients with IJRT was also investigated by Watzke et al. , who studied the long-term effect of laser treatment on six eyes and concluded that IJRT prognosis depends on its type and clinical features.
In this study, minimal laser reaction was achieved in all treated eyes and no postlaser treatment complications were encountered during follow-up periods.
However, Freidman et al.  reported a case with subretinal hemorrhage after grid laser photocoagulation for IJRT.
Despite the fact that different treatment options have been investigated by many authors, laser photocoagulation remains mostly effective in type 1 IJRT ,,,,,,,,.
| Conclusion|| |
Focal laser ablation of type 1 IJRT lesions proved to be effective. The treatment is a safe method for managing the leakage from vessels, resulting in stabilization of vision.
| Acknowledgements|| |
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]