eMedicine Specialties > Ophthalmology > Retina

Retinopathy, Hemoglobinopathies: Differential Diagnoses & Workup

Author: Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Coauthor(s): Hon-Vu Q Duong, MD, Ophthalmologist, Department of Ophthalmology, Westfield Eye Center; Michael J Shapiro, MD, Associate Professor, Vitreoretinal Service, Director, Eye Trauma Services, Department of Ophthalmology, University of Illinois at Chicago; Vitreoretinal Consultant, Pediatric Genetics and Birth Defects Clinic; Oswaldo Castro, MD, Director of Center for Sickle Cell Disease, Professor, Department of Internal Medicine, Howard University; Richard G Fiscella, RPh, MPH, Clinical Associate Professor, Departments of Pharmacy and Ophthalmology, University of Illinois at Chicago
Contributor Information and Disclosures

Updated: Sep 8, 2009

Differential Diagnoses

Branch Retinal Vein Occlusion
Retinopathy of Prematurity
Central Retinal Vein Occlusion
Retinopathy, Diabetic, Proliferative
Chronic Renal Failure
Sarcoidosis
Eales Disease
Systemic Lupus Erythematosus
Hypertension

Workup

Laboratory Studies

  • Per internal medicine/hematology consultation
  • Hemoglobin electrophoresis
  • Oxygen saturation
  • Systemic hydration/acidosis

Imaging Studies

  • Per internal medicine/hematology consultation

Other Tests

  • Perform fluorescein angiography when the examination does not explain the degree of vision loss.
  • Perform ocular B-scan ultrasonography when unable to adequately visualize the retina.
  • Consider cerebral vascular accident (CVA).
  • Consider retinal vascular emboli.

Histologic Findings

  • RPE atrophy
  • RPE hyperplasia
  • RPE migration into partially degenerated retina and perivascular location
  • Retinal thinning with loss of outer retinal layers
  • Retinal detachment with cystic degeneration
  • Retinal layers may be disorganized.
  • Pigment-laden macrophages are seen within and beneath the retina.
  • Pupillary space may be filled by a thin fibrovascular membrane.
  • Cataractous changes may be noted.
  • Thick-walled blood vessels often are filled with sickled erythrocytes.
  • Proliferation of blood vessels may be seen in the retina and the vitreous, leading to intravitreal neovascularization.

More on Retinopathy, Hemoglobinopathies

Overview: Retinopathy, Hemoglobinopathies
Differential Diagnoses & Workup: Retinopathy, Hemoglobinopathies
Treatment & Medication: Retinopathy, Hemoglobinopathies
Follow-up: Retinopathy, Hemoglobinopathies
References
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Further Reading

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Keywords

hemoglobin retinopathy, proliferative retinopathy, sickle cell disease, sickle cell hemoglobinopathy, sickle cell retinopathy, homozygous sickle cell disease, sickle cell C disease, sickle cell-thalassemia disease, SS disease, SC disease, S-Thal disease

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Contributor Information and Disclosures

Author

Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Hon-Vu Q Duong, MD, Ophthalmologist, Department of Ophthalmology, Westfield Eye Center
Hon-Vu Q Duong, MD is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Michael J Shapiro, MD, Associate Professor, Vitreoretinal Service, Director, Eye Trauma Services, Department of Ophthalmology, University of Illinois at Chicago; Vitreoretinal Consultant, Pediatric Genetics and Birth Defects Clinic
Michael J Shapiro, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Oswaldo Castro, MD, Director of Center for Sickle Cell Disease, Professor, Department of Internal Medicine, Howard University
Disclosure: Nothing to disclose.

Richard G Fiscella, RPh, MPH, Clinical Associate Professor, Departments of Pharmacy and Ophthalmology, University of Illinois at Chicago
Disclosure: Nothing to disclose.

Medical Editor

Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas
Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians & Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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