Retinopathy of Prematurity Workup

Updated: Sep 20, 2021
  • Author: KN Siva Subramanian, MD, FAAP; Chief Editor: Santina A Zanelli, MD  more...
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Workup

Approach Considerations

The evaluation of premature infants at risk for retinopathy of prematurity (ROP) should be limited to practitioners who are facile with binocular indirect ophthalmoscopy and scleral depression, and who have significant experience with ROP.

Ocular ultrasonography is an important tool when there is a media opacity (eg, cataract, vitreous hemorrhage) that precludes a view of the retina.

Some center utilize fundus photography with remote image reading. [20] There have been encouraging results with this approach.

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Other Tests

Ophthalmologic evaluation in retinopathy of prematurity (ROP)

Record the vascular maturity (how far out the vessels have grown), as indicated by zone, stage of disease, and the presence or absence of plus disease or preplus disease.

Quantify the extent of ROP on the basis of number and contiguity of clock hours (meridians) in which the disease is present in the retina.

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Staging

The International Classification of Retinopathy of Prematurity (ICROP) describes five stages of ROP, as follows [16] :

  • Stage I is characterized by a line of demarcation between the vascular and avascular retina. Branching or arborization can be seen growing at the leading edge of the retinal vasculature. (See the image below.)

  • Retinopathy of Prematurity. Stage I retinopathy of Retinopathy of Prematurity. Stage I retinopathy of prematurity.
  • Stage II is characterized by an elevated ridge, rather than a flat demarcation line. Flat neovascularization may be present but is posterior to the ridge. (See the following image.)

  • Retinopathy of Prematurity. Stage II retinopathy o Retinopathy of Prematurity. Stage II retinopathy of prematurity.
  • Stage 3 is extraretinal neovascularization, or vessels that grow off the ridge into the vitreous toward the examiner. (See the image below.)

  • Retinopathy of Prematurity. Stage III retinopathy Retinopathy of Prematurity. Stage III retinopathy of prematurity.
  • Stage IV refers to partial retinal detachment. Stage IV-A is outside the macula; stage IV-B involves the macula.

  • Stage IV is total retinal detachment. (See the following image.)

  • Retinopathy of Prematurity. A comparison between a Retinopathy of Prematurity. A comparison between attached and detached retina.

 

Some ophthalmologists describe an immature or avascular retina as stage 0 ROP.

Plus disease is an important marker of disease activity/severity; plus refers to severe tortuosity of vessels in the posterior pole. Rapidly progressing plus disease is sometimes referred to as Rush disease. Preplus disease is defined as vascular abnormalities of the posterior pole characterized by more arterial tortuosity and more venous dilatation than normal but not severe enough to be classified as plus disease. 

Zones of disease

Zone I is the innermost area of the retina surrounding the macula (see the image below).

Retinopathy of Prematurity. Zone I retinopathy of Retinopathy of Prematurity. Zone I retinopathy of prematurity.

 

Zone II is the middle third of the retina, nasally extending to the edge of the retina (see the following image).

Retinopathy of Prematurity. Zone II retinopathy of Retinopathy of Prematurity. Zone II retinopathy of prematurity.

 

Zone III is the most peripheral area of the retina on the temporal side (see the image below).

Retinopathy of Prematurity. Zone III retinopathy o Retinopathy of Prematurity. Zone III retinopathy of prematurity.

Types

As of the publication of the Early Treatment for Retinopathy of Prematurity (ETROP) study, more ophthalmologists are describing eyes with ROP into two types (see the image below):

Retinopathy of Prematurity. Treatment guidelines, Retinopathy of Prematurity. Treatment guidelines, according to the Early Treatment for Retinopathy of Prematurity (ET-ROP) study.

Type 1 ROP requires treatment and includes the following:

  • Eyes with zone I, stage III ROP without plus disease, or

  • Eyes with zone II, stage II or stage III ROP with plus disease

Type 2 ROP requires observation and includes the following:

  • Eyes with zone I, stage I or stage III ROP without plus disease, or

  • Eyes with zone II, stage III ROP without plus disease

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Procedures

A proper examination of the fundus requires adequate pupillary dilation. Typically, the infant's nurse will instill a combination of 0.2% cyclopentolate hydrochloride (HCl)/1% phenylephrine HCl) (Cyclogyl) ophthalmic solution 2-3 times in the hour before the scheduled examination. Mydriatic agents can cause blanching of the skin of the lids and increased blood pressure,

Infants examined in the neonatal intensive care unit should be relatively medically stable. They should be swaddled, and the attending nurse should assist the examination by stabilizing the head and upper body of the baby.

Wire lid speculums are used to hold the lids open. Most examiners will instill a topical anesthetic to reduce the discomfort of the speculum and the scleral depression (gentle pressing on the sclera to bring the peripheral retina into better view).

The examination can cause bradycardia, apnea, and hypertension (in response to pain) and, rarely, seizures and arrhythmia. Examiners should be able to examine each eye in a few minutes to reduce the risk of these complications. Medications and equipment for cardiovascular support should be at hand.

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