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Retinopathy of Prematurity

According to the National Eye Institute, about 15,000 infants develop retinopathy of prematurity (ROP) annually in the United States. Of these infants, about 520 become legally blind every year. ROP has increased in prevalence as advances in medical care have increased survival of extremely premature infants. Infants less than 32 weeks or below 1500 grams should be screened for ROP, which was previously known as retrolental fibroplasia. [Section: 2006]
ROP, characterized by abnormal retinal vascularization in preterm infants, occurs in 2 phases:
  1. Inhibited vascularization leading to retinal hypoxia (~30 - 32 weeks postmenstrual age)
  2. Hypoxia-driven neovascularization (begins ~32 - 34 weeks postmenstrual age) [Chen: 2011]
Of the 50 identified risk factors for ROP, prematurity is primary. *Others include:
  • Low-birth weight
  • Greater than a week of assisted ventilation
  • Surfactant administration
  • Large blood transfusion volume
  • Illness acuity
  • Hyperglycemia
  • Insulin administration
  • Sepsis
  • Blood gas fluctuation
  • Intraventricular hemorrhage
  • Bronchopulmonary dysplasia
  • Fungal infection
  • Early erythropoietin administration
  • High arterial oxygen tension
  • Slow longitudinal growth
  • Low insulin-like growth factor binding protein 1 and 3
*[Bharwani: 2008] [Carlo: 2010] [Hagadorn: 2007] [Hellström: 2009] [Kaempf: 2011] [Lad: 2009] [Löfqvist: 2006] [Löfqvist: 2009] [Noyola: 2002] [PATZ: 1952] [Seiberth: 2000]

Diagnosis

Evaluation is completed by an ophthalmologist specialized in neonatal disorders. The pupil is dilated in order to visualize the retina and vitreous by indirect ophthalmoscope examination.

Recommended Follow-Up

Initial ROP screening should be completed at 32-weeks postmenstrual age or 5-weeks postnatal age, whichever comes second. Follow-up assessments are based on initial findings. See Table 1: Schedule for ROP Screening Frequency or Treatment Based on Examination Findings in Best Evidence Statement (BESt) Screening for Retinopathy of Prematurity (ROP) (PDF Document 100 KB).

Treatment Considerations

Preterm infants who are not treated for stage 3+ ROP (especially when in zone 1) have poor visual outcomes. [Cryotherapy: 2002] Early treatment (prethreshold vs. threshold) is recommended for the best outcomes. [Early: 2003] [Good: 2005] Prethreshold is defined as follows: [Good: 2005]
  • Any stage (less than threshold) ROP in zone 1
  • Stage 2 with plus disease in zone 2
  • Stage 3 without plus disease in zone 2
  • Stage 3 with plus disease in zone 2, but fewer clock hours than threshold
Treatment of ROP in the United States typically involves diode laser photocoagulation. Cryotherapy was the initial form of treatment and is still performed in developing countries. Laser photocoagulation-treated eyes had 5.2 times the visual improvement of cryotherapy-treated eyes. However, laser therapy also can cause complications or adverse outcomes; in one study, 2 of 23 laser-treated eyes (8.6%) advanced to stage 5 ROP. [Ng: 2002] The follow-up evaluation excluded infants who had had retinal detachment.
New treatments for ROP that focus on decreasing VEGF, erythropoietin levels, or IGF-1 targeting supplements are in the exploratory stages. A promising new treatment is bevacizumab injection, but results have only included small sample sizes.

Resources

Information & Support

For Professionals

Best Evidence Statement (BESt) Screening for Retinopathy of Prematurity (ROP) (PDF Document 100 KB)
From Cincinnati Children's Hospital Medical Center, this document provides detailed information on screening and follow-up for ROP.

For Parents and Patients

Support

Facts About Retinopathy of Prematurity (NEI)
Information to help patients and their families search for general information about retinopathy of prematurity (ROP);National Eye Institute.

Tools

The International Classification of Retinopathy of Prematurity Revisited (JAMA)
A revised consensus statement of an international group of retinopathy of prematurity experts.

Services

Pediatric Ophthalmology

See all Pediatric Ophthalmology services providers (8) in our database.

For other services related to this condition, browse our Services categories or search our database.

Authors

Lead Author: Jeniel L. Jacobs, DNP, APRN, NNP-BC - 9/2014
Content Last Updated: 7/2015

Page Bibliography

Bharwani SK, Dhanireddy R.
Systemic fungal infection is associated with the development of retinopathy of prematurity in very low birth weight infants: a meta-review.
J Perinatol. 2008;28(1):61-6. PubMed abstract / Full Text

Carlo WA, Finer NN, Walsh MC, Rich W, Gantz MG, Laptook AR, Yoder BA, Faix RG, Das A, Poole WK, Schibler K, Newman NS, Ambalavanan N, Frantz ID 3rd, Piazza AJ, Sánchez PJ, Morris BH, Laroia N, Phelps DL, Poindexter BB, Cotten CM, Van Meurs KP, Duara S, Narendran V, Sood BG, O'Shea TM, Bell EF, Ehrenkranz RA, Watterberg KL, Higgins RD.
Target ranges of oxygen saturation in extremely preterm infants.
N Engl J Med. 2010;362(21):1959-69. PubMed abstract / Full Text

Chen J, Stahl A, Hellstrom A, Smith LE.
Current update on retinopathy of prematurity: screening and treatment.
Curr Opin Pediatr. 2011;23(2):173-8. PubMed abstract / Full Text

Cryotherapy for Retinopathy of Prematurity Cooperative Group.
Multicenter trial of cryotherapy for retinopathy of prematurity: natural history ROP: ocular outcome at 5(1/2) years in premature infants with birth weights less than 1251 g.
Arch Ophthalmol. 2002;120(5):595-9. PubMed abstract / Full Text

Early Treatment For Retinopathy Of Prematurity Cooperative Group.
Revised indications for the treatment of retinopathy of prematurity: results of the early treatment for retinopathy of prematurity randomized trial.
Arch Ophthalmol. 2003;121(12):1684-94. PubMed abstract / Full Text

Good WV, Hardy RJ, Dobson V, Palmer EA, Phelps DL, Quintos M, Tung B.
The incidence and course of retinopathy of prematurity: findings from the early treatment for retinopathy of prematurity study.
Pediatrics. 2005;116(1):15-23. PubMed abstract

Hagadorn JI, Richardson DK, Schmid CH, Cole CH.
Cumulative illness severity and progression from moderate to severe retinopathy of prematurity.
J Perinatol. 2007;27(8):502-9. PubMed abstract / Full Text

Hellström A, Hård AL, Engström E, Niklasson A, Andersson E, Smith L, Löfqvist C.
Early weight gain predicts retinopathy in preterm infants: new, simple, efficient approach to screening.
Pediatrics. 2009;123(4):e638-45. PubMed abstract

International Committee for the Classification of Retinopathy of Prematurity.
The International Classification of Retinopathy of Prematurity revisited.
Arch Ophthalmol. 2005;123(7):991-9. PubMed abstract / Full Text

Kaempf JW, Kaempf AJ, Wu Y, Stawarz M, Niemeyer J, Grunkemeier G.
Hyperglycemia, insulin and slower growth velocity may increase the risk of retinopathy of prematurity.
J Perinatol. 2011;31(4):251-7. PubMed abstract

Lad EM, Hernandez-Boussard T, Morton JM, Moshfeghi DM.
Incidence of retinopathy of prematurity in the United States: 1997 through 2005.
Am J Ophthalmol. 2009;148(3):451-8. PubMed abstract

Löfqvist C, Andersson E, Sigurdsson J, Engström E, Hård AL, Niklasson A, Smith LE, Hellström A.
Longitudinal postnatal weight and insulin-like growth factor I measurements in the prediction of retinopathy of prematurity.
Arch Ophthalmol. 2006;124(12):1711-8. PubMed abstract / Full Text

Löfqvist C, Hansen-Pupp I, Andersson E, Holm K, Smith LE, Ley D, Hellström A.
Validation of a new retinopathy of prematurity screening method monitoring longitudinal postnatal weight and insulinlike growth factor I.
Arch Ophthalmol. 2009;127(5):622-7. PubMed abstract / Full Text

Ng EY, Connolly BP, McNamara JA, Regillo CD, Vander JF, Tasman W.
A comparison of laser photocoagulation with cryotherapy for threshold retinopathy of prematurity at 10 years: part 1. Visual function and structural outcome.
Ophthalmology. 2002;109(5):928-34; discussion 935. PubMed abstract

Noyola DE, Bohra L, Paysse EA, Fernandez M, Coats DK.
Association of candidemia and retinopathy of prematurity in very low birthweight infants.
Ophthalmology. 2002;109(1):80-4. PubMed abstract

PATZ A, HOECK LE, DE LA CRUZ E.
Studies on the effect of high oxygen administration in retrolental fibroplasia. I. Nursery observations.
Am J Ophthalmol. 1952;35(9):1248-53. PubMed abstract

Section on Ophthalmology.
Screening examination of premature infants for retinopathy of prematurity.
Pediatrics. 2006;117(2):572-6. PubMed abstract / Full Text
From the Section on Ophthalmology American Academy of Pediatrics, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus.

Seiberth V, Linderkamp O.
Risk factors in retinopathy of prematurity. a multivariate statistical analysis.
Ophthalmologica. 2000;214(2):131-5. PubMed abstract