PARACENTRAL ACUTE MIDDLE MACULOPATHY WITH SICKLE CELL TRAIT.

Area of Science:

• Ophthalmology
• Vascular Biology
• Genetics

Background:

• Sickle cell trait is a common hemoglobinopathy.
• Retinal detachment surgery can involve risks.
• Pediatric patients may have unique physiological responses to surgery.

Observation:

• A 16-year-old female with sickle cell trait experienced sudden vision loss post-scleral buckling surgery.
• Ophthalmic imaging confirmed paracentral acute middle maculopathy, an inner retinal ischemic event.

Findings:

• The condition was linked to potential sickling triggered by dehydration and intraocular pressure spikes during surgery.
• Visual acuity improved significantly over 3 months, with imaging showing resolution of the acute lesion but residual retinal thinning.
• A paracentral scotoma persisted, corresponding to the atrophic area.

Implications:

• This case highlights the risk of sickling events in patients with sickle cell trait undergoing ophthalmic surgery.
• Recommendations include optimizing hydration, managing intraocular pressure, and considering presurgical hemoglobin electrophoresis.
• Preventive strategies are crucial to avoid vision loss in at-risk populations during retinal procedures.