Richard Kaiser

BASICS

DESCRIPTION

Retinopathy of prematurity (ROP) is a potentially blinding proliferative vascular retinopathy which is associated with prematurity and low birth weight and can cause lifelong ocular morbidity.

EPIDEMIOLOGY

Incidence

1,300 cases of visual loss from ROP per year in the USA

RISK FACTORS

• Low birth weight (<1,500 g)

• Prematurity (<30 weeks EGA)

• Complicated clinical course

• Prolonged supplemental oxygen

Genetics

Sporadic

GENERAL PREVENTION

Avoid excessive oxygen therapy using blood gas monitoring

PATHOPHYSIOLOGY

Normal retinal vascular development is arrested by premature birth. Peripheral retinal ischemia leads to release of vascular growth factors. Retinal and extraretinal neovascular proliferation occurs. Advanced proliferative disease can bleed, cause traction, and can lead to a retinal detachment and blindness.

COMMONLY ASSOCIATED CONDITIONS

With premature birth:

• Myopia

• Strabismus

• Cataract

• Retinal detachment

• Glaucoma

DIAGNOSIS

HISTORY

• Birth weight and EGA

• Neonatal clinical course

PHYSICAL EXAM

• Dilated stereoscopic fundus exam:

– Dilated fundus exams for neonates 1,500 g or less, 28 weeks EGA or less, or any neonate 2,000 g or less felt to be at high risk.

– First exam should be at 4–6 weeks of life, or at 31–33 weeks EGA, whichever is later.

• Staging of ROP

• Location:

– Zone I: A circle of the retina centered at the optic disc with a radius twice the distance from the disc to the fovea

– Zone II: The area of the retina beyond zone I within a circle centered at the optic disc with a radius equal to the distance from the disc to the nasal ora serrata

– Zone III: A crescent of the retina outside of zone II in the far periphery which is widest at the temporal retina

• Severity:

– Stage 1: Demarcation line between vascularized and nonvascularized retina

– Stage 2: Demarcation ridge

– Stage 3: Ridge with extraretinal fibrovascular proliferation

– Stage 4: Subtotal retinal detachment (4A: extrafoveal, 4B: fovea involved)

– Stage 5: Total retinal detachment

• Plus: Plus disease is defined as dilation and tortuosity of retinal vessels, vitreous haze and iris vessel engorgement

• Type 1 ROP (treatment recommended within 72 h):

– Zone I, stage 3, or any stage with plus

– Zone II, stage 2 or 3 with plus

• Type 2 ROP (no treatment recommended, close follow-up)

– Zone I, stage 1 or 2 without plus

– Zone II, stage 3 without plus

DIAGNOSTIC TESTS & INTERPRETATION

DIFFERENTIAL DIAGNOSIS

• Familial exudative vitreoretinopathy

• Retinoblastoma

• Incontinentia pigmenti

• Persistent fetal vasculature

• Norrie’s disease

TREATMENT

MEDICATION

First Line

Laser photocoagulation of the avascular retina

Second Line

Cryotherapy ablation of the avascular retina

SURGERY/OTHER PROCEDURES

Retina surgery for retinal detachment

Nursing

Bradycardia and apnea can occur during the eye exam and laser treatment of neonates. Close nursing and respiratory supervision is essential for safety during examinations and treatments.

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Examination twice a week for type 2 ROP

• Examination once a week for borderline type 2 ROP

• Examination every 2 weeks for low-risk patients

PATIENT EDUCATION

Educate parents about lifelong morbidity of ROP and the need for close follow-up with ophthalmology.

ADDITIONAL READING

• Cryotherapy for Retinopathy of Prematurity Cooperative Group. Multi-center trial of cryotherapy for retinopathy of prematurity: Ophthalmological outcomes at 10 years. Arch Ophthalmol 2001;119:1110–1118.

• Early Treatment for Retinopathy of Prematurity Cooperative Group. Revised indications for treatment of retinopathy of prematurity: Results of the early treatment of retinopathy of prematurity randomized trial. Arch Ophthalmol 2003;121:1684–1696.

• Tasman W, Patz A, McNamara JA, et al. Retinopathy of prematurity: The life of a lifetime disease. Am J Ophthalmol 2006;141:167–174.

CODES

ICD9

• 362.20 Retinopathy of prematurity, unspecified

• 362.84 Retinal ischemia

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