[Maculopathy in sickle cell disease]

Area of Science:

• Ophthalmology
• Hematology
• Genetics

Background:

• Sickle cell disease (SCD) is an inherited blood disorder causing vaso-occlusive crises and organ damage.
• Sickle cell retinopathy (SCR) is a known ocular complication, but sickle cell maculopathy (SCM) can also occur.
• SCM may present early in SCD, independent of peripheral retinopathy.

Purpose of the Study:

• To review ocular manifestations of SCD, focusing on SCR and SCM.
• To discuss current systemic therapies for SCD.
• To highlight the increasing recognition of SCM in clinical practice.

Main Methods:

• Literature review of international and German studies on ocular involvement in SCD.
• Focus on SCR and SCM, including diagnostic advancements.
• Overview of systemic treatment strategies for SCD.

Main Results:

• SCM, characterized by temporal inner retinal thinning, has gained recognition recently with advanced imaging (SD-OCT, OCTA).
• Approximately 50% of SCD patients may develop SCM early, irrespective of SCR.
• Increased prevalence of SCM is expected in Germany due to improved therapies and migration.

Conclusions:

• SCM is an important, often early, ocular complication of SCD that can occur without peripheral SCR.
• Awareness of SCM is crucial for early diagnosis and avoiding unnecessary investigations.
• Advances in imaging and systemic treatments necessitate increased clinical vigilance for SCM in SCD patients.