Carol L. Shields
BASICS
DESCRIPTION
• Nonhereditary condition
• Unilateral
• Retinal telangiectasia
• Retinal exudation
• Exudative retinal detachment
• Classification proposed by Shields et al:
– Stage 1. Retinal telangiectasia (T)
– Stage 2. T + and exudation (E)
– Stage 3. T + E + subretinal fluid (F)
– Stage 4. T + E + F + neovascular glaucoma (G)
– Stage 5. T + E + F + G + phthisis bulbi
ALERT
Any child with an abnormal pupillary reflex should have a fundus examination to rule out Coats’ disease.
Geriatric Considerations
Coats’ disease occurs almost exclusively in young patients under the age of 10 years. It is rare to diagnose this condition in the elderly.
Pediatric Considerations
Coats’ disease occurs primarily in children at approximately of 2–11 years of age and rarely is related to systemic conditions.
EPIDEMIOLOGY
Unknown
RISK FACTORS
None.
Genetics
No proven genetic defect.
PATHOPHYSIOLOGY
• Idiopathic congenital retinal telangiectasia.
• Affects temporal retina most often but can be 360 degrees in the fundus.
• Progressive retinal and subretinal exudation.
• Exudative retinal detachment
COMMONLY ASSOCIATED CONDITIONS
• None in true unilateral Coats’ disease
• In cases of bilateral exudative retinopathy that resemble Coats’ disease (but not true Coats’ disease), one must consider a list of systemic conditions including IRVAN syndrome, familial exudative vitreoretinopathy, dyskeratosis congenital, retinopathy of prematurity, facioscapulohumeral dystrophy, turner syndrome, incontinentia pigmenti, neurofibromatosis, kabuki makeup syndrome, and others.
DIAGNOSIS
HISTORY
• Blurred vision
• Yellow or white pupillary reflex
• Strabismus
PHYSICAL EXAM
• The following data is based on an analysis in 150 consecutive cases by Shields et al.
• Symptoms include
– Decreased visual acuity in 43%
– Strabismus in 23%
– Leukocoria in 20%
– Pain in 3%
– Heterochromia in 1%
– Nystagmus in 1%
– No symptoms in 8%
• Findings include
– Telangiectasia in 100%
– Retinal exudation in 99%
– Exudative retinal detachment in 81%
– Retinal hemorrhage in 13%
– Retinal macrocyst in 11%
– Vasoproliferative tumor in 6%
– Neovascularization of disc in 2%
– Neovascularization of retina in 1%
– Neovascularization of iris in 8%
– Corneal edema in 3%
– Anterior chamber cholesterol in 3%
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Initial lab tests
No lab abnormalities except in older patients, check blood cholesterol, and lipids.
Follow-up & special considerations
• If bilateral involvement, evaluate patient for related syndromes as previously mentioned.
• Treat all leaking telangiectasia
Imaging
Initial approach
• Fundus photography to document the retinal findings.
• Ocular ultrasonography to confirm retinal detachment.
• Fluorescein angiography to identify leaking telangiectasia.
• Optical coherence tomography to study the macular region for edema or fluid.
Follow-up & special considerations
• Repeat fundus photography at each visit.
• Repeat fluorescein angiography if subretinal fluid or exudation detected.
• Repeat optical coherence tomography for macular findings.
Diagnostic Procedures/Other
Pathological Findings
• Peripheral retinal telangiectasia
• Retinal detachment
• Retinal and subretinal exudation
• Cholesterol clefts in subretinal space
• Advanced cases show neovascular glaucoma
DIFFERENTIAL DIAGNOSIS
• Retinoblastoma
• Retinal hemangioblastoma
• Retinal vasoproliferative tumor
• Persistent hyperplastic primary vitreous
• Rhegmatogenous retinal detachment
• Radiation retinopathy
• Hypertensive crisis
TREATMENT
MEDICATION
First Line
Management depends on stage of disease.
• No medication necessary
• Laser photocoagulation
• Cryotherapy
Second Line
• Intravitreal bevacizumab
• Intravitreal triamcinolone
• Subtenons triamcinolone
• Antiglaucoma medications if necessary
ADDITIONAL TREATMENT
General Measures
• Laser photocoagulation
• Cryotherapy
Issues for Referral
Any child with suspected Coats’ disease should be referred for management by a qualified specialist.
Additional Therapies
• Medical treatment for secondary glaucoma
• Cataract extraction if necessary
• Vitrectomy with repair retinal detachment
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• The patient should be reassessed every 2–4 months following treatment.
• At each reassessment, a judgment on the status of the retina will be made and the need for further treatment. In general, this condition requires at least 3 extensive treatments to quiet the leakage.
• All examinations are best performed under anesthesia, especially if the child is under 4 years of age.
• When 4 years old or older, office exams can be considered.
Patient Monitoring
• Check retina status long-term twice yearly for life.
• Return for examination if visual acuity decreases.
PATIENT EDUCATION
PROGNOSIS
• The systemic prognosis is unaffected by this ophthalmic condition.
• The visual prognosis with stage of disease, based on an analysis of 150 consecutive eyes. Below is the risk for poor visual outcome of 20/200 or worse:
– Stage 1 – 0%
– Stage 2 – 50%
– Stage 3 – 70%
– Stage 4 – 100%
– Stage 5 – 100%
COMPLICATIONS
• Retinal detachment
• Cataract
• Chronic pain
• Phthisis bulbi
ADDITIONAL READING
• Shields JA, Shields CL, Honavar S, et al. Coats’ disease. Clinical variations and complications of Coats’ disease in 150 cases. The 2000 Sanford Gifford Memorial Lecture. Am J. Ophthalmol 2001;131:561–71.
• Shields JA, Shields CL, Honavar SG, et al. Classification and management of Coats’ disease. The 2000 Proctor lecture. Am J Ophthalmol 2001;131:572–83.
• Shields JA, Shields CL. Lesions that can simulate retinoblastoma. In: Shields JA, Shields CL (eds). Intraocular tumors, an atlas and textbook, 2nd ed. Philadelphia: Lippincott, Williams and Wilkins, 2008:364–365.
CODES
ICD9
• 361.2 Serous retinal detachment
• 362.12 Exudative retinopathy
• 362.15 Retinal telangiectasia
CLINICAL PEARLS
• Coats’ disease can simulate retinoblastoma
• Typical features can assist in diagnosis
• Visual prognosis good if discovered early before macular exudation.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
