Which optic disc findings are characteristic of hemoglobinopathy retinopathy?

Answer

Vascular changes in the optic disc can be seen secondary to intravascular occlusions. These intravascular occlusions primarily affect the small vessels on the surface of the optic disc.

These lesions appear as dark red spots or clumps on the nerve head, often called the disc sign of sickling. The proposed mechanism for this sign is the transient plugging of sickled (deoxygenated) erythrocytes.

Fluorescein angiography (FA) reveals segments of linear or Y-configuration of hypofluorescence that correspond to the dark red spots. Blood flow through these vessels (as seen on angiography) is not impaired.^[2]Like conjunctival vascular changes, these lesions are transient and do not produce any appreciable visual symptoms.

Optic nerve neovascularization is a rare complication of hemoglobinopathies.

Optic disc changes appear to be more common in patients with sickle cell anemia than in those with sickle cell C disease or sickle cell-thalassemia disease.

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Author

Brian A Phillpotts, MD

Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, National Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Hon-Vu Quang Duong, MD Clinical Instructor of Ophthalmology and Ophthalmic Pathology, Westfield Eye Center; Senior Lecturer of Neurosciences (Anatomy and Physiology), Nevada State College

Hon-Vu Quang 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, Association for Research in Vision and Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Oswaldo Castro, MD Senior Advisor, Center for Sickle Cell Disease, Professor Emeritus of Medicine and Pediatrics, College of Medicine

Disclosure: Nothing to disclose.

Richard G Fiscella, PharmD, MPH ClinicalProfessor, Departments of Pharmacy and Ophthalmology, University of Illinois at Chicago

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steve Charles, MD Founder and CEO of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Russell P Jayne, MD 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, American Society of Retina Specialists

Disclosure: Nothing to disclose.

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