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PubMed2006

Idiopathic macular telangiectasia.

OBJECTIVES: To review the frequency and nature of idiopathic macular telangiectasia and to classify the disorders based on new clinical and imaging observations. METHODS: A combined retrospective and prospective analysis of newly diagnosed patients seen over a period of 3 years. Patients were identified based on the Gass-Blodi classification and were studied with biomicroscopy, fluorescein angiography, and optical coherence tomography. RESULTS: Ten patients associated with aneurysmal telangiectasia (Gass-Blodi group 1) and 26 patients with perifoveal telangiectasia (Gass-Blodi group 2) were recruited. None with occlusive telangiectasia (Gass-Blodi group 3) were identified. New observations based on clinical, fluorescein angiographic, and optical coherence tomographic findings were made. CONCLUSIONS: Our series was similar to that in the Gass-Blodi study in terms of frequency. New observations in groups 1 and 2 have expanded our knowledge of the clinical spectrum of these disorders. A simplified classification termed idiopathic macular telangiectasia with 2 distinct types (type I, or aneurysmal telangiectasia, and type II, or perifoveal telangiectasia) was proposed to produce a better understanding of the entities and to enhance teaching and research. The third type, occlusive telangiectasia, has been omitted from our classification based on its rarity and presence of capillary nonperfusion rather than macular telangiectasia as the primary abnormality.

Adult↗
PubMed1976

Perforating diathermy coagulation for retinal angiomas.

Perforating diathermy coagulation can be used efficiently to destroy large angiomas of the retina. Five patients who had developed large angiomas (greater than 5 disc diameters) in association with various retinal diseases were treated with this technique. The follow-up period ranged from 1 1/2 to 7 years, except for one case in which the patient died five days after the surgical procedure. Several techniques of perforating diathermy are described, and the indications, surgical precautions, and complications of each method are discussed.

Adolescent↗
PubMed1983

Retinal telangiectasia secondary to bilateral carotid artery occlusion.

A man complaining of blurred vision had diffuse retinal capillary telangiectasia as a primary retinal finding. Evaluation of the carotid arteries by a noninvasive angiographic method revealed bilateral, silent occlusions of both common carotid arteries. Thus, some cases of "idiopathic retinal capillary telangiectasia" may in fact be harbingers of underlying carotid artery disease.

Angiography↗
PubMed1975

Coat's disease. An epidemiologic and Fluorescein angiographic study.

Coats' disease represents a disorder of the retinal vascular walls. In a series of 112 consecutive eyes with Coats' disease the involvement was unilateral in 90.2%. The majority (72%) of patients were male. 57.8% were under 20 years of age. There was, however, a second peak of the disease (30.4%) between the age of 41 and 60. Severe cases were found mainly among juvenile patients, whereas in the senile group milder cases prevailed. Fluorescein angiographic findings comprised vascular aneurysms in almost all cases, rarefaction and coarsening of the capillary bed in 71%, capillary occlusions in 70%, occlusions of arterioles or arteries in 27%, shunt formations in 27%, extensive widening of arteries or veins in 27% and leakage of dye mainly in advanced cases. The participation of the central retina in the disease process was variable. Evidence is presented that Leber's military aneurysm retinitis is not a separate entity but a special form of Coats' disease.

Adolescent↗
PubMed1979

Rhegmatogenous retinal detachment with retinal telangiectasia.

A 34-year-old man with unilateral retinal telangiectasia developed a bullous retinal detachment. A horseshoe retinal tear was found at 12 o'clock. The detachment resolved with placement of an encircling scleral buckle. The prompt and permanent resolution of subretinal fluid supported our belief that this was a rhegmatogenous retinal detachment.

Adult↗
PubMed2006

The effects of external subretinal fluid drainage on secondary glaucoma in Coats' disease.

PURPOSE: We sought to investigate the effect of external subretinal fluid drainage (SRFD) on secondary or impending secondary glaucoma caused by bullous exudative retinal detachment for saving eyes with Coats' disease. METHODS: By retrospective chart review, we collected the treatment results of 56 patients younger than 15 years of age with Coats' disease. External SRFD was performed when exudative retinal detachment became bullous enough to cause anterior displacement of the lens-iris diaphragm or when secondary angle-closure glaucoma occurred. Treatment results were regarded as successful when secondary angle-closure glaucoma was prevented or responded to treatment. RESULTS: The mean age of those who underwent external SRFD was 3.1+/-1.8 years. Exudative retinal detachment was found in 48 eyes (86%), and external SRFD was needed initially in 19 (28%). External SRFD initially was performed in 19 eyes (28%) and in 2 (3%) after initial cryotherapy. Of these 21 eyes, no definite neovascular glaucoma was detected, and it took on average 1.2 SRFDs to treat or prevent secondary angle-closure glaucoma. All treatments were successful, and no eye was enucleated. CONCLUSIONS: External SRFD should be considered early as a treatment for secondary angle-closure glaucoma associated with bullous exudative retinal detachment in Coats' disease.

Adolescent↗
PubMed2001

Clinical variations and complications of Coats disease in 150 cases: the 2000 Sanford Gifford Memorial Lecture.

PURPOSE: The purpose of this report is to review the clinical variations and natural course of Coats disease, using strict diagnostic guidelines. METHODS: In a retrospective, consecutive series, Coats disease was defined as idiopathic retinal telangiectasia with intraretinal or subretinal exudation without appreciable signs of retinal or vitreal traction. We reviewed our experience with the clinical features, complications, and diagnostic approaches to Coats disease. RESULTS: In 150 consecutive patients (158 eyes), Coats disease was diagnosed at a median age of 5 years (range, 1 month to 63 years), occurred in 114 males (76%), and was unilateral in 142 patients (95%). There was no predilection for race or laterality. The most common referral diagnoses were Coats disease in 64 cases (41%) and retinoblastoma in 43 (27%). The first symptom or sign was decreased visual acuity in 68 cases (34%), strabismus in 37 (23%), leukocoria in 31 (20%), and 13 patients (8%) were asymptomatic. Visual acuity at presentation was 20/200 to no light perception in 121 eyes (76%). The anterior segment was normal in 142 eyes (90%). The retinal telangiectasia involved the midperipheral or peripheral fundus in 156 of the 158 eyes (99%) and was restricted to the macular area in two eyes (1%); involved mainly the temporal fundus in 66 eyes (42%), inferior fundus in 41 eyes (26%), and more than one sector in 34 eyes (22%). Retinal exudation was present in all 12 clock hours in 86 eyes (55%) and six or more clock hours in 115 eyes (73%). There was a total retinal detachment in 74 eyes (47%) and neovascular glaucoma in 12 (8%). Retinal macrocysts were present in 18 eyes (11%), a vasoproliferative tumor in nine eyes (6%) and retinal neovascularization in four eyes (3%). Fluorescein angiography in 49 of the 158 eyes (37%) disclosed early hyperfluorescence of the telangiectasias and macular edema in 18 of eyes (37%). Ultrasonography typically showed a retinal detachment but no solid mass. CONCLUSIONS: Coats disease is a distinct clinical entity characterized by idiopathic retinal telangiectasia and retinal exudation. It is usually unilateral, occurs mostly in young males, and can cause severe visual loss resulting from exudative retinal detachment. The clinician should follow strict criteria in making the diagnosis, to avoid confusing Coats disease with other forms of exudative retinopathy.

Adolescent↗
PubMed1982

Coats' disease. Evaluation of management.

Forty-one patients representing 43 eyes with retinal telangiectasia and subretinal exudates (Coats' Disease) were managed between 1966 and 1980. Thirty-three eyes in 31 patients between 4 and 45 years of age had an average of two quadrants involved with telangiectasia. Of four untreated eyes, three deteriorated and one stabilized. In contrast, of 29 eyes treated with cryotherapy or photocoagulation, eight deteriorated, 15 stabilized, and six improved. Ten patients in the series were under 4 years of age and had more severe disease than older patients. On the average three quadrants were involved with telangiectasia and subretinal exudate. Three untreated eyes rapidly progressed to total retinal detachment with glaucoma. The seven treated children required frequent assessment and repeated treatment. Of these, one stabilized but had no light perception, and the remaining six improved. The authors stress that the disease can be controlled with aggressive treatment. Their treatment methods are described.

Adolescent↗
PubMed2005

Long-term juxtafoveal retinal telangiectasia.

PURPOSE: To determine the long-term natural history of idiopathic juxtafoveal telangiectasia (IJRT). METHODS: Record review in 2 university-based and 2 private vitreoretinal practices sought patients with IJRT documented by color photographs and fluorescein angiograms (FAs) during the period January 1, 1980, to December 31, 1993. Patients then had repeated examinations and FAs. RESULTS: Twenty patients with IJRT in 32 eyes had follow-up examinations. Fifteen patients had color photographs and FAs, and one had color photographs alone. Follow-up varied from 10 years to 21 years (average, 15 years). Six eyes were treated by laser photocoagulation at onset. Twenty-four of the 26 untreated eyes lost vision as measured by Snellen testing. Visual loss and morphologic progression depended on IJRT type. Six of 8 untreated eyes with type IA IJRT lost vision by >/ or = 3 lines (Snellen), 4 to 20/70 or worse. Vision loss was caused by progressive telangiectatic changes and intraretinal edema. Fifteen of 20 initially untreated eyes with type IIA IJRT developed either central retinal pigment epithelium membranes or subretinal neovascularization with loss of vision to 20/80 or less. CONCLUSION: IJRT prognosis depends on type and clinical features. Long-term prognosis for central vision is poor.

Adult↗
PubMed2006

Coats disease: a lifetime disease.

PURPOSE: To document the recurrent nature of Coats Disease. METHODS: This study is a retrospective chart review approved by our Institutional Review Board. Thirteen patients (13 eyes) were diagnosed with Coats Disease starting in 1966. Age, visual acuity, time of onset, method of treatment, and the number and intervals of recurrences were documented. Recurrences were recorded once initial treatment proved successful and most exudates had absorbed. The length of follow-up for each patient and the most recent visual acuity was recorded. RESULTS: The average follow-up period for the 13 patients was 12.4 years with a range from 4.0 to 37.5 years. Eleven patients (85%) were male, two (15%) were female and all had unilateral involvement. The average age at diagnosis was 7.0 years with a range from 9 months to 27 years. Four out of the twelve treated patients (33%) had recurrences, and three of the four had multiple recurrences. The average elapsed time from successful treatment to the first recurrence was 4.3 years with a range from 3.3 to 5.4 years. The average number of recurrences was 3.3. CONCLUSION: Coats Disease can recur so it is important that parents educate any affected children regarding the necessity of follow-up, including throughout their adult years.

Adolescent↗
PubMed2006

Observations on the management of Coats' disease: less is more.

BACKGROUND: In this article we share our experience of treating various severities of Coats' disease and focus on optimal therapy for advanced disease. METHODS: Retrospective chart review of 10 patients treated with varied techniques including intraocular surgery, cryopexy and/or laser photocoagulation. RESULTS: Nine patients were male. At presentation the average age was 4.6 years (range 21 months-7 years), the average number of retinal quadrants involved with telangiectasia was 2.7 (range 1-4, median 3), eight of the 10 patients had retinal detachment, six of these being total, and all patients had macular involvement with either exudate or fibrosis. Average follow-up was 2.3 years (range 1-4.5 years). The best visual outcomes were observed in patients who presented with less severe disease. For example, the only four patients to maintain ambulatory vision all presented without total retinal detachment, two or fewer quadrants of retinal telangiectasia and a visual acuity better than light perception. No patient developed secondary angle closure glaucoma, and all patients have kept a cosmetically acceptable eye. CONCLUSION: In this limited series, visual outcomes in the setting of advanced Coats' disease are largely dependent on disease severity and visual status at the time of presentation. Minimally invasive surgery with vitreous infusion through the pars plana, combined with external drainage of subretinal fluid together with cryotherapy and/or laser photocoagulation is sufficient to effect retinal re-attachment and prevent loss of the eye.

Child↗
PubMed1998

Management of moderate to advanced Coats' disease.

Sixteen patients (16 eyes) with Coats' disease who had either total bullous exudative retinal detachment or macular involvement and/or at least two quadrants of exudative retinal detachment were followed for a mean period of 60.6 months. Three patients with no light perception received no treatment. Ten eyes received cryotherapy on one or more occasions and two had laser photocoagulation. One eye underwent subretinal fluid drainage, intraocular infusion and cryotherapy. In those patients who could be tested, all the treated eyes retained their initial visual acuities. All 16 eyes had an acceptable cosmesis and none progressed to painful neovascular glaucoma or phthisis bulbi. Patients with late onset disease had a more benign course.

Adolescent↗
PubMedDate not supplied

[Immunopathogenetic, clinical features and treatment of external exudative Coats' retinitis].

The findings of comprehensive clinical and immunopathogenetic examinations of 42 patients with external exudative Coats' retinitis showed the significance of infection in the etiopathogenesis of disease and helped identify the etiology, mainly infectious, in 47.6% patients, which correlated with case histories indicating foci of chronic infection and type I immunological insufficiency. Toxoplasma infection was the predominant etiological factor (38 cases). The predominant clinical symptoms were solid exudation, vascular changes, hemorrhages, vitreous involvement, and exudative detachment of the retina. Changes in the immune status were as follows: shifts in T- and B-immunity, impaired complex formation, in some cases selected deficit of IgA. Association of Coats' retinitis with erythrocytic phenotype B(III) was detected in 44% patients (p < 0.02); carriers of this blood group are at a high risk (RR 11.16) of the disease. The treatment was carried out with consideration for etiology, immunopathogenesis, and clinical picture and was supplemented by argon laser coagulation, if indicated.

Adolescent↗
PubMed2003

[Analysis of misdiagnostic causes of Coats disease].

OBJECTIVE: To analyze the clinical features and misdiagnostic causes of Coats disease. METHODS: The clinical data were retrospectively analyzed in 69 eyes of 68 patients with Coats disease. RESULTS: Misdiagnostic histories to a varying degree occurred in 11 of the 68 cases with a misdiagnosis rate of 16.18% and misdiagnostic mean time of 7.5 months. CONCLUSION: Lack of knowledge on pathogenic characteristics of Coats disease, lack of typical manifestations, no timely accessory examinations, and advanced cases are the main causes of misdiagnosis.

Adolescent↗