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Retinal Telangiectasia

Coats' Disease


Presentation

  • Three cases are presented spanning the spectrum of primary retinal telangiectasia.[ncbi.nlm.nih.gov]
  • The most common appearance, type 2A, typically presents as juxtafoveolar telangiectasias with minimal exudation, superficial retinal crystalline deposits, and right-angle venules.[ncbi.nlm.nih.gov]
  • Right-angle venules OU, refractile deposits OS, and no macular edema OU were present. Fluorescein angiography demonstrated areas of parafoveal leakage temporal and nasal OD and temporal OS.[ncbi.nlm.nih.gov]
  • The clinical histories of four patients in whom APRT developed after retinal surgery are presented. For each patient studied, no retinal vascular abnormality was observed at the time of initial surgery.[ncbi.nlm.nih.gov]
  • Presents a more streamlined format to the printed text to help you focus on the clinically actionable information you need everyday.[books.google.de]
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]
Photopsia
  • Photopsia and floaters are common. Persistent color patterns may also be perceived. Often, the unaffected eye compensates for the loss of vision in the other eye; however, this may result in some loss of depth perception and parallax.[institut-vision.org]
  • The patient has had intermittent photopsias in both eyes over the past two years. He denies floaters.[eyerounds.org]
Dry Eyes
Rubeosis Iridis
  • Spontaneous resolution is rare and the disease often progresses to massive sub-retinal exudation, exudative retinal detachment, rubeosis iridis (neovascularization of the colored part of the eye), secondary cataract, and secondary glaucoma.[lowvision.preventblindness.org]
Corneal Edema
  • edema, anterior chamber shallowing, suspension of lipid and protein in the aqueous humor, or neovascular glaucoma.[reviewofophthalmology.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]
Ataxia
  • Systemic Features: As a result of intracranial calcifications, leukodystrophy and brain cysts, patients have a variety of neurologic signs including spasticity, ataxia, dystonia, cognitive decline, and seizures.[disorders.eyes.arizona.edu]
  • […] microscopy reveal that photoreceptor cells lose their outer segments, but may not be completely destroyed, suggesting strategies promoting cell restoration could be as viable as cell replacement. 25 Some studies have implicated the gene responsible for ataxia[retinalphysician.com]
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]
Dystonia
  • Systemic Features: As a result of intracranial calcifications, leukodystrophy and brain cysts, patients have a variety of neurologic signs including spasticity, ataxia, dystonia, cognitive decline, and seizures.[disorders.eyes.arizona.edu]

Workup

Gliosis
  • Small foveal cavitations that have the appearance of lamellar macular holes can also be seen, but there is usually no accompanying epiretinal gliosis or traction and there is typically an ILM drape.[retinalphysician.com]

Treatment

  • 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]
  • CLOSE Medical Disclaimer The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes.[diseaseinfosearch.org]
  • Macular laser treatment to the right eye was planned, and an improvement in her vision was anticipated with resolution of macular oedema following focal laser treatment.[gponline.com]

Prognosis

  • 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]
  • 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]
  • Copyright American Society of Retina Specialists 2016 Treatment and prognosis JFT usually results in vision loss when the abnormal blood vessels leak fluid or bleed into or under the retina.[asrs.org]

Etiology

  • 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]
  • Associations VATER syndrome The exact etiology is unknown and the disease is a non-hereditary disorder.[radiopaedia.org]
  • Etiology The Mac Tel Project found a prevalence of diabetes mellitus (DM) of 28% and hypertension (HTN) of 52% in Mac Tel type 2.[eyewiki.aao.org]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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 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]

Prevention

  • . • 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: watzker@ohsu.edu 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]

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