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SYMPTOMS
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Decrease in vision
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SIGNS
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Acute phase
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Stage 1: Presence of a thin, white, and flat demarcation line between the vascularized posterior retina and non-vascularized anterior retina
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Stage 2: The demarcation line becomes an elevated ridge and is present above the plane of the retina. Small tufts of new blood vessels are noted
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Stage 3: The demarcation ridge has associated neovascularization with fibrovascular tissue that grows into the vitreous
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Stage 4: 4a (Presence of a partial retinal detachment that does not involve the fovea) 4b (Presence of a partial retinal detachment that does involve the fovea)
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Stage 5: 5a (Presence of a total funnel-shaped retinal detachment with a visible optic disc) 5b (Presence of a total funnel-shaped retinal detachment with an optic disc that is not visible) 5c (Presence of a total funnel-shaped retinal detachment with an optic disc that is not visible and abnormalities of the anterior segment)
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Retinal detachments associated with retinopathy of prematurity can be tractional or exudative
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Cicatricial/Regression phase
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Temporal retinal dragging, Macular ectopia, Glaucoma, Retinal detachments, RPE proliferation, Cataracts
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Plus disease: Presence of posterior pole retinal vessels that are dilated and tortuous (indicates a more severe form of retinopathy of prematurity and subsequent poor prognosis). Also associated with vitreous haze and pupil rigidity
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Pre-Plus disease: Presence of dilated and tortuous posterior pole retinal vessels that does not meet the criteria for Plus disease
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Rush disease/Aggressive retinopathy of prematurity: Plus disease with vascularization only in Zone I and rapidly developing retinal neovascularization (indicates rapid progression and very poor prognosis)
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Threshold disease: Level of retinopathy of prematurity where there is 50% chance of developing a retinal detachment if left untreated
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WORK-UP
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Pupils, EOMs, Cover test, Visual field, Full eye exam with dilated retinal exam, OCT analysis of the optic nerve, OCT analysis of the macula, Fundus Autofluorescence, Fluorescein Angiography, Fundus photos, Infrared photos, B-scan ultrasound
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TREATMENT
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If the patient is in the acute phase of retinopathy of prematurity, refer patient to a retinal specialist or pediatric ophthalmologist ASAP for further evaluation and treatment
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If the patient is in the cicatricial/regression phase of retinopathy of prematurity and there is no evidence of glaucoma, retinal detachments, or glaucoma, the patient should be monitored closely
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If the patient is in the cicatricial/regression phase of retinopathy of prematurity and there is a retinal detachment, the patient should be referred to a retinal specialist or pediatric ophthalmologist ASAP
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If the patient is in the cicatricial/regression phase of retinopathy of prematurity and there is evidence of glaucoma, the patient should be referred to a glaucoma specialist or pediatric ophthalmologist
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If the patient is in the cicatricial/regression phase of retinopathy of prematurity and there is visually significant cataracts, the patient should be referred to a cataract surgeon or pediatric ophthalmologist
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FOLLOW-UP
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If the patient is in the cicatricial/regression phase of retinopathy of prematurity and there is no evidence of glaucoma, retinal detachments, or glaucoma, the patient should be monitored and seen back every 3-4 months (more frequently if patient is an infant)
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The patient most likely will continue to be followed up by a retinal specialist/pediatric ophthalmologist/glaucoma specialist on a regular basis if the patient has acute retinopathy of prematurity or develops complications secondary to cicatricial retinopathy of prematurity
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ADDITIONAL LAB | TESTS
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None
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ETIOLOGY
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Retinal vasculature in premature infants not being able to fully develop and grow to the anterior retina leads to damage of the developing blood vessels and pre-existing blood vessels which leads to retinal ischemia (occurs due to high levels of supplemental oxygen). This causes growth of retinal neovascularization and fibrovascular tissue
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Risk factors for development of retinopathy of prematurity include: Infants born before 36 weeks gestation, Infant birth weight that is 2000g or less, High levels of supplemental oxygen
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DIFFERENTIAL DX
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Familial exudative vitreoretinopathy, Ocular toxocariasis, Coats disease, Retinoblastoma
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NOTES
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Patients with retinopathy of prematurity typically have high myopic and astigmatic refractive errors
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85% of infants with retinopathy of prematurity will have spontaneous regression
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