Management
The most crucial aspect of managing patients with IJRT is recognition of the clinical signs. This condition is relatively uncommon and has only been described in the literature during the past 25 years; hence, many practitioners may not be familiar with or experienced in diagnosing the disorder. IJRT must be part of the differential in any case of idiopathic paramacular hemorrhage, vasculopathy, macular edema or focal pigment hyper-trophy, especially in those patients with-out a history of retinopathy or contributory systemic disease.Diagnosis of IJRT may be aided by the use of ancillary testing. OCT can help to identify the abnormal vessels, pigment plaques, retinal crystals, fovealatrophy and intra-retinal cysts associated with this disorder.
Likewise, fluorescein angiography is beneficial in identifying the anomalous vasculature, particularly in the early stages of Type 2 disease.While some have argued that angiography is essential in making a definitive diagnosis, others suggest that such testing may be unnecessary when a diagnosis is apparent via less invasive means.
The natural history of IJRT suggests a slowly progressive disorder. A retrospective series of 20 patients over 10 to21 years showed deterioration of vision in more than 84% of eyes, either due to intra-retinal edema and serous retinal detachment (Type 1) or pigmented RPE scar formation or CNV (Type 2).
Historically, laser photocoagulation has been the recommended treatment early in the course of Type I IJRT to help suspend the exudative process and diminish macular edema.
In contrast, laser therapy is not considered a viable treatment option for Type 2 IJRT, unless frank neovascularization is evident on fluorescein angiography.
In fact, laser therapy may actually enhance vessel ectasia and promote intra-retinal fibrosis in these individuals.
Today, additional therapies include surgical removal of CNV, photodynamic therapy with verteporfin and treatment with anti-VEGF drugs such as bevacizumab.
However, these treatment modalities should be considered only in cases of marked and rapid vision loss secondary to macular edema or CNV. Otherwise, a conservative approach is recommended, since many of these patients will stabilize without intervention.
Read more about this topic: Macular Telangiectasia
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