eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Neonatology

Retinopathy of Prematurity: Differential Diagnoses & Workup

Author: KN Siva Subramanian, MD, Professor of Pediatrics and Obstetrics/Gynecology, Chief of Neonatal Perinatal Medicine, Director of Nurseries, Georgetown University Hospital
Coauthor(s): Monisha Bahri, MBBS, MD, Fellow in Neonatal/Perinatal Medicine, Department of Neonatology, Georgetown University Hospital; Gonzalo (Vike) Vicente, MD, FAAP, Consulting Ophthalmologist, Eye Doctors of Washington
Contributor Information and Disclosures

Updated: Jun 29, 2009

Differential Diagnoses

Familial exudative vitreoretinopathy

Workup

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 retinopathy of prematurity on the basis of number and contiguity of clock hours in which the disease is present in the retina.
    • Some centers are also experimenting by taking retinal fundus photographs and having an off-site ophthalmologist evaluate the photos.

Staging

  • The international classification of retinopathy of prematurity (ICROP) describes 5 stages of retinopathy of prematurity, as follows:12
    • Stage I is characterized by a line of demarcation. Extra vessels can be seen growing at the leading edge of the retinal vasculature. The line of demarcation separates the vascularized portion of the retina from the anteriorly positioned avascular retina.
    • Stage II is characterized by an elevated ridge, rather than a flat demarcation line. Neovascularization may be present but is posterior to the ridge.
    • Stage III refers to extraretinal neovascularization or vessels that grow onto the ridge and then into the vitreous toward the examiner. 
    • Stage IV refers to partial retinal detachment.
    • Stage V is total retinal detachment.
    • Some ophthalmologists describe an immature or avascular retina as Stage 0 retinopathy of prematurity.
  • Plus disease refers to severe tortuosity of vessels. 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. Rapidly progressing plus disease is sometimes referred to as Rush disease.
  • In addition, the zone of disease is designated as follows (see Multimedia):
    • Zone I is the innermost area of the retina surrounding the macula.

      Zone I retinopathy of prematurity.

      Zone I retinopathy of prematurity.

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      Zone I 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.

      Zone II retinopathy of prematurity.

      Zone II retinopathy of prematurity.

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      Zone II retinopathy of prematurity.

      Zone II retinopathy of prematurity.

    • Zone III is the most peripheral area of the retina on the temporal side.

      Zone III retinopathy of prematurity.

      Zone III retinopathy of prematurity.

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      Zone III retinopathy of prematurity.

      Zone III retinopathy of prematurity.

  • As of the publication of the Early Treatment for Retinopathy of Prematurity (ETROP) study, more ophthalmologist are describing eyes with retinopathy of prematurity into 2 types: 

    Treatment guidelines, according to the Early Trea...

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

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    Treatment guidelines, according to the Early Trea...

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

    • Type 1 (requires treatment)
      • Eyes with zone I, stage III retinopathy of prematurity without plus disease or
      • Eyes with zone II, stage II or stage III retinopathy of prematurity with plus disease
    • Type 2 (requires observation)
      • Eyes with zone I, stage I or stage III retinopathy of prematurity without plus disease or
      • Eyes with zone II, stage III retinopathy of prematurity without plus disease

More on Retinopathy of Prematurity

Overview: Retinopathy of Prematurity
Differential Diagnoses & Workup: Retinopathy of Prematurity
Treatment & Medication: Retinopathy of Prematurity
Follow-up: Retinopathy of Prematurity
Multimedia: Retinopathy of Prematurity
References
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References

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  50. Vanderveen DK, Mansfield TA, Eichenwald EC. Lower oxygen saturation alarm limits decrease the severity of retinopathy of prematurity. J AAPOS. Oct 2006;10(5):445-8. [Medline].

  51. Wallace DK. Oxygen saturation levels and retinopathy of prematurity--are we on target?. J AAPOS. Oct 2006;10(5):382-3. [Medline].

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Further Reading

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Keywords

retinopathy of prematurity, ROP, retrolental fibroplasia, retinal neovascularization, extremely low birth weight infants, ELBW, respiratory distress syndrome, RSD, bronchopulmonary dysplasia, BPD, sepsis, retinal hypoxia, tortuosity of vessels, maternal preeclampsia, pulmonary hemorrhage, visual impairment, blindness, myopia, amblyopia, strabismus, patent ductus arteriosus, intraventricular hemorrhage, visual impairment, blindness, supplemental oxygen, treatment, diagnosis

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Contributor Information and Disclosures

Author

KN Siva Subramanian, MD, Professor of Pediatrics and Obstetrics/Gynecology, Chief of Neonatal Perinatal Medicine, Director of Nurseries, Georgetown University Hospital
KN Siva Subramanian, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Nutrition, American Society for Parenteral and Enteral Nutrition, American Society of Law Medicine and Ethics, New York Academy of Sciences, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Monisha Bahri, MBBS, MD, Fellow in Neonatal/Perinatal Medicine, Department of Neonatology, Georgetown University Hospital
Monisha Bahri, MBBS, MD is a member of the following medical societies: American Academy of Pediatrics, Indian Academy of Pediatrics, and Medical Council of India
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, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

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 College of Nutrition, American College of Physician Executives, and American Heart Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Arun K Pramanik, MD, MBBS, Professor of Pediatrics, Director of Neonatal Fellowship, 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, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Carol L Wagner, MD, Professor of Pediatrics, Medical University of South Carolina
Carol L Wagner, MD is a member of the following medical societies: American Academy of Pediatrics, American Chemical Society, American Medical Women's Association, American Public Health Association, American Society for Bone and Mineral Research, American Society for Clinical Nutrition, Massachusetts Medical Society, National Perinatal Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Ted Rosenkrantz, MD, Professor, Departments of Pediatrics and Obstetrics/Gynecology, Division of Neonatal-Perinatal Medicine, University of Connecticut School of Medicine
Ted Rosenkrantz, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Pediatric Society, Connecticut State Medical Society, Eastern Society for Pediatric Research, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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