Terminology for Retinal Findings in Sickle Cell Disease Research: A Scoping Review

Area of Science:

• Ophthalmology
• Hematology
• Medical Research Methodology

Background:

• Sickle cell disease (SCD) is a genetic blood disorder with significant ocular manifestations.
• Ocular complications, particularly retinopathy, are a major cause of vision loss in SCD patients.
• Current research on SCD's ophthalmic findings exhibits variability in methodology and reporting.

Purpose of the Study:

• To systematically review the SCD literature and assess definitions of retinopathy.
• To identify and analyze ocular outcomes measured in SCD studies.
• To highlight inconsistencies in the documentation of ophthalmic findings and diagnostic methods.

Main Methods:

• A systematic scoping review of English-language literature from 2000-2021.
• Searched major databases including PubMed, Google Scholar, Embase, and Cochrane Library.
• Extracted data on study characteristics, patient demographics, vision outcomes, and retinopathy definitions/diagnoses.

Main Results:

• 111 studies were analyzed from 4006 unique citations.
• Retinopathy definitions were inconsistent, with many studies lacking clarity or not defining it.
• Diagnostic methods varied, including fundus examination, OCT, and angiography, with inconsistent reporting.
• Ophthalmologists were senior authors in over half the studies, with a focus on North American and European research.

Conclusions:

• Significant inconsistencies exist in the documentation of methods and outcomes in SCD ophthalmic studies.
• Lack of standardized definitions for sickle cell retinopathy and reporting of examination methods is a concern.
• Clear, consistent reporting of ophthalmic outcomes is essential for advancing SCD research and patient care, especially with new therapies.