Three cases are presented spanning the spectrum of primary retinal telangiectasia. [ncbi.nlm.nih.gov]

Presentation and Clinical Findings The most common presenting signs of Coats' disease are decreased visual acuity, strabismus and leukocoria. [reviewofophthalmology.com]

• Dry Eyes

  Another common condition also deserving of consideration is dry eye. [lowvision.preventblindness.org]

• Skin Patch

  Neurofibromatosis (or von Recklinghausen disease) is associated with typical discoloured café au lait skin patches and with plexiform neurofibromas in the area of eyelids and central nervous system (CNS) meningiomas [ 11 – 13 ]. [bmcophthalmol.biomedcentral.com]

• Sparse Hair

  Some individuals have sparse hair, abnormal pigmentation of the skin, and dysplastic nails as well. Some extraretinal features are also found in patients with dyskeratosis congenita ( 127550 ), and in Labrune syndrome ( 614561 ). [disorders.eyes.arizona.edu]

• Arthritis

  Gass and Blodi (1993) postulate this category of disease to be related to systemic conditions, such as gouty arthritis or hypoglycemia (low blood sugar). [lowvision.preventblindness.org]

• Rabies

  Retinal telangiectasia Anomalía congénita de los vasos retinianos … Diccionario de oftalmología List of diseases (R) — A list of diseases in the English wikipedia.DiseasesTOC RaRab Rai* Rabies * Rabson Mendenhall syndrome * Radial defect Robin sequence [translate.academic.ru]

• Neglect

  Amblyopia therapy, strabismus surgery, and other types of ancillary rehabilitation may be useful and should not be neglected as part of the total treatment of these patients. REFERENCES 1. Coats G. Forms of retinal dysplasia with massive exudation. [medtextfree.wordpress.com]

While no treatment has been proven to prevent progression, treatment may be helpful in some cases. [rarediseases.info.nih.gov]

Therefore, no treatment was advised, and the patient remains with acuities of 20/100- in the right eye and 20/80- in the left. Initially, retinal signs can be subtle in type 2A IJRT, with no treatment being indicated. [ncbi.nlm.nih.gov]

In these patients, treatment should be considered in case of documented progression. Argon laser photocoagulation is the treatment of choice in the early stages. [institut-vision.org]

Treatment There are several treatment modalities for Coats’ disease, depending on its stage; they are briefly summarized in table 2. [karger.com]

IJRT prognosis depends on type and clinical features. Long-term prognosis for central vision is poor. [journals.lww.com]

The presenting visual acuity also correlates with the long-term prognosis. [institut-vision.org]

Prognosis The primary factors determining final visual outcome in patients with Coats' disease are the presenting visual acuity, extent of retinal detachment and the extent of macular exudation. [reviewofophthalmology.com]

The ultimate prognosis for eyes with Coats’ diseases can be measured in terms of two end points: visual acuity and anatomical stability. [medtextfree.wordpress.com]

Whilst Macular Telangiectasia type 2 can lead to significant visual loss in some cases, overall the prognosis is favourable, with average visual acuities of 6/12 being reported. [maculaspecialists.com.au]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

Etiology and pathogenesis The etiology remains poorly understood. Recent data indicate the causative role of somatic mutation in the NDP gene. [institut-vision.org]

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

The etiology of both types of MacTel is still unknown and no treatment has been found to be effective to prevent further progression. [en.wikipedia.org]

Macular telangiectasia type 2 is a progressive bilateral disease of the macula of unknown etiology, associated with changes in the macular capillary network and retinal atrophy [ 2 ]. [bmcophthalmol.biomedcentral.com]

Epidemiology Coats disease is a very rare eye disorder. The exact prevalence is unknown. There is a clear male predominance (more than 75% of patients) and no ethnic predisposition. [institut-vision.org]

Table : Characteristics of the three types of idiopathic juxtafoveolar telangiectasia Types of IJFT* Epidemiology Signs and symptoms Treatment Prognosis IJFT type I Predominantly male. Mean age 40yo. [eyerounds.org]

In 1915 Leber wrote two more articles and concluded that the disease he had described in 1912 was a variant of Coats’ disease.[2] EPIDEMIOLOGY Coats’ disease is characterized by discrete zones of alteration in the retinal vascular structure with aneurysmal [medtextfree.wordpress.com]

Coats' disease, Leber's miliary aneurysms, and IMT may be part of a singular clinical spectrum sharing pathophysiologic and histopathologic features and similarities in clinical presentation. [ncbi.nlm.nih.gov]

Pathophysiology IMT pathophysiology is incompletely understood. 1-11 IMT is categorized into types (IMT-1 and IMT-2), with each type subcategorized by various stages connoting characteristics. 1-11 Each subtype and stage takes into account the entity’ [reviewdiseasehandbook.com]

Pathophysiology Chronic neurodegenerative processes, vascular inflammation, occlusion and ectatic capillary changes lead to loss of outer retinal layers and formation of pseudolamellar macular holes with cysts or cavitations that can also be in inner [eyewiki.aao.org]

Thus, it is possible that the pathophysiology, including the involvement of VEGF, in the 2 types of IMT may be considerably different. Intravitreal bevacizumab treatment may rapidly improve retinal oedema in other diseases. [nature.com]

• To treat those cases with macular thickening that prevents a good laser spot. • To be able to repeat treatments. • In cases with glaucoma or repeated intravitreal triamcinolone injections, intravitreal Bevacizumab is a good option to prevent increase [evrs.eu]

While no treatment has been proven to prevent progression, treatment may be helpful in some cases. [rarediseases.info.nih.gov]

Watzke, MD, Casey Eye Institute, Oregon Health & Science University, 3375 SW Terwilliger Boulevard, Portland, OR 97239-4197; e-mail: [email protected] Supported in part by an unrestricted grant to Casey Eye Institute from Research to Prevent Blindness, [journals.lww.com]

Treatment in stage 3 and 4 disease is primarily aimed at anatomic correction of the retinal detachment, lowering of intraocular pressure and prevention of phthisis. [reviewofophthalmology.com]