Presentation
Adult onset coats disease in a 56-year-old male: An atypical presentation. Med J DY Patil Univ 2015;8:350-3 How to cite this URL: Dhawan B, Goel A, Singh R, Vig V. Adult onset coats disease in a 56-year-old male: An atypical presentation. [mjdrdypu.org]
Retinal exudation was present in 1, 2, 3 and 4 quadrants in 5 eyes (22.7%), 7 (31.8%), 3 (13.6%), and 7 (31.8%) respectively. Foveal exudation was present in 18 eyes (81.8%). [iovs.arvojournals.org]
Cases presenting with leucocoria or strabismus presented early whereas subjective visual loss presented much later. A large proportion of eyes (44%) were blind at diagnosis. [nature.com]
We present a rare case of Coats' disease in an adult with leukocoria. A 37-year-old male presented with dimness of vision with constricted visual field in his left eye for the past 3 months. [jmedscindmc.com]
All eyes presented with profound visual loss with uniocular presentation and exudative retinal detachments at the late stages, with poor visual prognosis. [journalajmah.com]
Entire Body System
- Pathologist
General pathologists usually do not have the opportunity to receive and study specimens from patients with Coats' disease. Even ophthalmic pathologists rarely receive an enucleated eye because of Coats' disease. [diagnosticpathology.biomedcentral.com]
Coats' disease - disease of the eye rarely encountered by pathologists. Indian J Pathol Microbiol 2018;61:98-100 How to cite this URL: Fernandes H, Umashankar T, Richie AJ, Hegde S. [ijpmonline.org]
Cardiovascular
- Vascular Disease
Pollock, The Houston Chronicle, 27 Jan. 2011 Coats' disease is a developmental vascular disease of the retina. … It is more common in males, is usually detected early in childhood, and cannot be recognized by any noninvasive test. — David S. [merriam-webster.com]
You are here Home › Education › Digital Reference of Ophthalmology › Vitreous & Retina › Retinal Vascular Diseases › Severe form of retinal telangiectasia Typically presents in the first decade; average age 5 Clinical features: Signs: Telangiectatic vessels [columbiaeye.org]
[…] with retinal vascular abnormalities and exudation in Coats' disease. 38 Zhang J...Huang X 30414256 2019 26 Longitudinal Examination of Fellow-Eye Vascular Anomalies in Coats' Disease With Widefield Fluorescein Angiography: A Multicenter Study. 38 Jeng-Miller [malacards.org]
Expression of vascular endothelial growth factor in eyes with Coats’ disease. Invest Ophthalmol Vis Sci 2013;54:57–62. 18 Zhao Q, Peng XY, Chen FH, Zhang YP, Wang L, You QS, Jonas JB. Vascular endothelial growth factor in Coats’ disease. [ghrnet.org]
Sohan Singh Eye Hospital, Amritsar, Punjab India Source of Support: None, Conflict of Interest: None DOI: 10.4103/0975-2870.157084 Coats disease is a congenital retinal vascular disease, which is seen commonly in childhood and remits and exacerbates throughout [mjdrdypu.org]
Skin
- Macula
Intraretinal and subretinal exudates often affect areas of the retina remote from the telangiectasias and tend to migrate toward the macula, although there is no clear explanation as to why the exudation preferentially accumulates in the macula, she said [eurotimes.org]
In adults, it typically has a more insidious clinical course. [1] In this article, we report a case of adult-onset Coats' disease that presented with macula involvement and was treated with laser photocoagulation with good outcome. [ojoonline.org]
Normal macula Coats’ disease with severe accumulation of yellow lipid under the macula. How is Coats’ Disease diagnosed? Coats’disease is a clinical diagnosis found during a dilated retinal examination. [eyecenters.com]
When the macula is already involved, treatment can, in some instances, improve central vision if the fluid resolves. [gosh.nhs.uk]
The visual acuity is usually not much affected unless exudation involves the macula, which requires treatment. [tnoajosr.com]
Eyes
- Blurred Vision
In addition, blurred vision, especially when one eye is closed (due to unilateral nature of the disease). [gotzaridis.gr]
The consequences of Coats' disease for the patient's vision may include deterioration in either central or peripheral vision. Symptoms typically become apparent in the form of blurred vision that seems to be worse when one eye is closed. [ivyroses.com]
The typical presenting signs include blurred vision, unilateral decreased vision (visual acuity of 20/200 or worse), strabismus and/or leucocoria. Deterioration in either central or peripheral vision is the main symptom. [institut-vision.org]
Symptoms Symptoms begin as blurred vision, usually pronounced when one eye is closed (due to the unilateral nature of the disease). [wikidoc.org]
In its early stages, Coats’ disease tends to affect peripheral vision. The affected person will begin to experience blurred vision. The appearance of the affected eye in flash photography can also be an early warning sign suggesting Coats’ disease. [2020detroit.com]
- Macula Edema
Resolution of severe macula edema in adult Coats' disease with intravitreal Triamcinolone and Bevacizumab injection. Korean J Ophthalmol 2008;22:190-3. 5. Adam RS, Kertes PJ, Lam WC. Observations on the management of Coats' disease: Less is more. [ojoonline.org]
Workup
Other Pathologies
- Gliosis
[…] telangiectasia •Remain visible throughout course •MC in inferotemporally, temporally & superotemporally •Tortuous but regular dilatation towards a mass •Disappear into tumor •Occur in any quadrant of tumor Retinal exudation + - Retinal mass - + Retinal gliosis [slideshare.net]
The detached retina was juxtaposed in the center and was thickened with gliosis along with telangiectatic blood vessels. A diagnosis of Coats' disease was offered [Figure 2]. [ijpmonline.org]
[…] subretinal exudate consists of cholesterol crystals, macrophages laden with cholesterol and pigment, erythrocytes, and hemosiderin. [11] A granulomatous reaction, induced by the exudate, may be seen with the retina. [12] Portions of the retina may develop gliosis [en.wikipedia.org]
Green WR (1967) Bilateral Coats‘ disease—Massive gliosis of the retina. Arch Ophthalmol 77: 378–383 PubMed 30. Grizzard WF, Deutman AF, Pinckers AJ (1978) Retinal dystrophies associated with peripheral retinal vasculopathies. [springermedizin.de]
Treatment
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]
Historically, treatment was based on clinical findings. However, with the advent of wide-field imaging, the first step which guides treatment is now wide-angle fluorescein angiography to identify areas of leakage and capillary dropout. [morancore.utah.edu]
Treatment: The treatment of choice is laser photocoagulation or cryotherapyto destroy anomalous vasculature. Prognosis: Left untreated, the disease will eventually cause blindness due tototal retinal detachment. [brainkart.com]
Parlak and Saatci reviewed the use of bevacizumab, ranibizumab, and pegaptanib as an adjuvant treatment with subsequent necessary treatments as mentioned above [10]. There were no reports on the use of aflibercept in children with Coats disease. [cureus.com]
The pre-treatment VEGF concentration in aqueous humour was 908 pg/ml and reduced to 167 pg/mL after the treatment. [ghrnet.org]
Prognosis
The presenting visual acuity also correlates with the long-term prognosis. [institut-vision.org]
The prognosis varies in each case. Factors that effect the prognosis include the stage of disease at the time of diagnosis, the age at diagnosis, the rate of disease progression, and the effectiveness of treatment. [rarediseases.info.nih.gov]
Stage 1 : (Telangiectesia with minimal/no exudation) • Observation • Laser photocoagulation Prognosis Eye can be saved Visual prognosis is favorable 46. [slideshare.net]
The key to better prognosis is earlier detection, which yields earlier treatment intervention. [jmedscindmc.com]
Prognosis The prognosis depends on the extent of the disease with milder, older presenting cases, having a better prognosis, of even spontaneous regression, whereas children under the age of three have a poorer prognosis. [eyewiki.aao.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]
Etiology An abnormal permeability of capillary endothelial cells in the retina, along with abnormal pericytes, causes the retinal vascular leakage that is the hallmark of CD. [orpha.net]
Associations VATER syndrome The exact etiology is unknown and the disease is a non-hereditary disorder. [radiopaedia.org]
Coats disease is a telangiectatic neovascular disease of the retina of unknown etiology that frequently affects unilateral eyes of young males. [eyewiki.aao.org]
Subsequent investigations and the failure of anti-inflammatory adrenocorticotropic hormone and steroid therapies have refuted early theories of a primary infectious or inflammatory etiology. 13, 14 Pathologic samples of eyes with Coats disease were once [clinicalgate.com]
Epidemiology
Epidemiology: This rare disorder manifests itself in young children andteenagers. Boys are usually affected (in about 90% of all cases). Coats’ disease usually occurs in young and teenage boys. It is nearly always unilateral. [brainkart.com]
A population-based study of Coats disease in the United Kingdom I: epidemiology and clinical features at diagnosis. [benthamopen.com]
Summary Epidemiology Annual incidence is Clinical description CD onset predominantly occurs in male children between 6 and 8 years old. Early stages of the disease are generally asymptomatic and evolution is variable. [orpha.net]
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]
Pathophysiology
Our understanding of the underlying pathophysiology continues to improve, as do our diagnostic and treatment modalities. Intravitreal therapy represents an exciting addition to traditional ablative techniques. [reviewofophthalmology.com]
Stable cases may be observed. 3, 7 However, Coats’ disease has been reported to recur even 10 years after initial diagnosis so life-long observation is recommended. 8 Pathophysiology: The exact mechanism remains elusive, but there is some evidence that [morancore.utah.edu]
Prevention
This prevents the normal flow of blood, and allows fluid to leak out of the blood vessels causing a buildup of fatty (lipid) material into the retina. [aapos.org]
Treatment is effective in preventing blindness in about 50% of all patients. Study Material, Lecturing Notes, Assignment, Reference, Wiki description explanation, brief detail [brainkart.com]
In very occasional cases, treatment can improve vision, but in most cases the aim of treatment is to prevent further worsening of vision. [rnib.org.uk]
Luckily, if it's detected early enough, it can be prevented. Doctors say the main problem with Coats' is that its symptoms are hard to spot, even for the individual suffering from the disease. [businessinsider.com]