Diagnostic Considerations

The presence of congenital retinal abnormalities in low birth weight (LBW) infants is rare, but it can pose significant risks. Familial exudative vitreoretinopathy (FEVR) is an idiopathic retinal vascular abnormality that mimics late-stage retinopathy of prematurity (ROP). FEVR is caused by abnormal retinal vascular development leading to exudation, traction through the macula, and retinal detachment. Vitreous hemorrhage can result from birth trauma, primary and secondary coagulopathies, and Terson syndrome.

Infectious causes that may mimic ROP include Toxoplasma, Toxocara, and Herpesviridae, especially cytomegalovirus.

Late ROP, especially stage 5 (total retinal detachment), can lead to cataract formation. Other causes of lens opacity in the neonate include congenital cataract, FEVR, rubella, and other infections.

Retinoblastoma (RB) is a rare genetically-driven tumor that in a young infant would most likely have arisen from a germinal mutation (chromosome 13). Germinal mutations give rise to multifocal disease; thus, a young infant with RB should be evaluated and treated promptly, and the treating physicians must be alert to the possibility of new tumor development before the age of 1 year.

Media opacities (vitreous hemorrhage, cataract, corneal opacity) can be followed up using ocular ultrasonography performed by an experienced examiner. An injection of intravitreal bevacizumab can resolve vitreous hemorrhage and allow fundoscopic evaluation.

Differential Diagnoses

Congenital Cataract
Familial exudative vitreoretinopathy
Pediatric Rubella
Retinoblastoma

Workup

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Media Gallery

Retinopathy of Prematurity. Stage I retinopathy of prematurity.
Retinopathy of Prematurity. Scheme of retina of right eye (RE) and left eye (LE) showing zone borders and clock hours used to describe location and extent of retinopathy of prematurity.
Retinopathy of Prematurity. Zone I retinopathy of prematurity.
Retinopathy of Prematurity. Zone II retinopathy of prematurity.
Retinopathy of Prematurity. Zone III retinopathy of prematurity.
Retinopathy of Prematurity. Stage II retinopathy of prematurity.
Retinopathy of Prematurity. Stage III retinopathy of prematurity.
Retinopathy of Prematurity. Mild extraretinal fibrovascular proliferation.
Retinopathy of Prematurity. Moderate extraretinal fibrovascular proliferation.
Retinopathy of Prematurity. Severe extraretinal fibrovascular proliferation.
Retinopathy of Prematurity. A comparison between attached and detached retina.
Retinopathy of Prematurity. Retinopathy of prematurity (ROP) threshold, according to the Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) Cooperative Group.
Retinopathy of Prematurity. Treatment guidelines, according to the Early Treatment for Retinopathy of Prematurity (ET-ROP) study.
Retinopathy of Prematurity. Laser photocoagulation. RPE = retinal pigment epithelium.
Retinopathy of Prematurity. Cryotherapy probe application.

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Author

Siva Subramanian, MD, FAAP Emeritus Chief of Neonatal-Perinatal Medicine, Medstar Georgetown University Hospital; Professor Emeritus of Pediatrics and Obstetrics, Senior Ethicist, Pellegrino Center for Clinical Bioethics, Georgetown University Medical Center

Siva Subramanian, MD, FAAP is a member of the following medical societies: American Pediatric Society, American Association for the Advancement of Science, American College of Nutrition, American Pediatric Society, American Society for Parenteral and Enteral Nutrition, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Melissa D Kern, MD Owner and Ophthalmologist in Pediatric and Adolescent Ophthalmology and Adult Strabismus, Arlington-Loudon Pediatric Ophthalmology; Chief of Surgery (Ophthalmology), Virginia Hospital Center

Melissa D Kern, MD is a member of the following medical societies: Northern Virginia Academy of Ophthalmology

Disclosure: Nothing to disclose.

William F Deegan, III, MD Associate Clinical Professor of Ophthalmology, Georgetown University School of Medicine; Attending Surgeon, Spectrum Retina and Ocular Oncology, Virginia Hospital Center Health System

William F Deegan, III, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, Massachusetts Medical Society, Washington Academy of Eye Physicians and Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Arun K Pramanik, MD, MBBS Professor of Pediatrics, Louisiana State University Health Sciences Center

Arun K Pramanik, MD, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, National Perinatal Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Santina A Zanelli, MD Associate Professor, Department of Pediatrics, Division of Neonatology, University of Virginia Health System

Santina A Zanelli, MD is a member of the following medical societies: American Academy of Pediatrics, American Epilepsy Society, Society for Neuroscience, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Oussama Itani, MD, FAAP, FACN Clinical Associate Professor of Pediatrics and Human Development, Michigan State University; Medical Director, Department of Neonatology, Borgess Medical Center

Oussama Itani, MD, FAAP, FACN is a member of the following medical societies: American Academy of Pediatrics, American Association for Physician Leadership, American Heart Association, American College of Nutrition

Disclosure: Nothing to disclose.

Monisha Bahri, MBBS, MD Attending Physician, Department of Neonatalogy, Washington Hospital Center

Monisha Bahri, MBBS, MD is a member of the following medical societies: American Academy of Pediatrics, Medical Council of India, Indian Academy of Pediatrics

Disclosure: Nothing to disclose.

Gonzalo (Vike) Vicente, MD, FAAP Consulting Ophthalmologist, Eye Doctors of Washington

Gonzalo (Vike) Vicente, MD, FAAP is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association

Disclosure: Nothing to disclose.

Gestational Age at Birth (wk)	Chronologic Age (wk)	Postmenstrual Age (wk)
22^*	9	31
23^*	8	31
24	7	31
25	6	31
26	5	31
27	4	31
28	4	32
29	4	33
30	4	34
31 (if necessary)	4	35
32 (if necessary)	4	36

Retinopathy of Prematurity Differential Diagnoses

Diagnostic Considerations

Differential Diagnoses

References

Tables

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