Introduction

Dome-shaped macula (DSM), first described in 2008, has evolved from an imaging curiosity to a recognized clinical entity with significant implications for retinal practice. Once thought to occur exclusively in high myopia with posterior staphyloma, DSM is now identified across the refractive spectrum—from hyperopia to emmetropia. This post synthesizes recent evidence to provide vitreoretinal surgeons with updated insights into DSM epidemiology, pathophysiology, complications, and management.

Epidemiology and Prevalence

DSM prevalence varies widely based on population and diagnostic criteria:

• General population: ~0.2–1.9%
• High myopia: 10–20%
• UK Biobank study: 4.8% overall, up to 22% in high hyperopia

Notably, DSM is increasingly detected in non-myopic eyes, challenging the traditional myopia-centric view.

Pathophysiology: Two Key Mechanisms

1. High Myopia: DSM may result from differential scleral expansion—localized macular resistance within a posterior staphyloma. Polarization-sensitive OCT reveals aggregated inner scleral fibers, suggesting a biomechanical rather than purely adaptive process.
2. Emmetropization: In children and non-myopic eyes, DSM may represent an adaptive response during ocular development, involving choroidal thickening and retinal forward shift.

Morphological Classification

DSM is morphologically heterogeneous:

• Horizontal oval (most common)
• Vertical oval (higher complication risk)
• Round
• Ridge-shaped macula (younger patients)

Vertical configurations are associated with increased serous retinal detachment (SRD) risk.

Key Complications

1. Serous Retinal Detachment (SRD)

• Prevalence: 8–50% of DSM cases
• Pathophysiology: Altered RPE function, choroidal hemodynamic changes
• Natural history: Often stable; spontaneous resolution in ~47% of cases
• Treatment: Variable efficacy with carbonic anhydrase inhibitors, subthreshold laser, PDT, anti-VEGF (high recurrence)

2. RPE Atrophy

• Correlates with dome height (>400 μm)
• Progressive, irreversible, impacts long-term visual prognosis

3. Choroidal Neovascularization (CNV)

• DSM is an independent risk factor (OR 4.95)
• Often extrafoveal, with similar anti-VEGF response as non-DSM eyes

4. Myopic Retinoschisis

• DSM shows a protective effect against foveal schisis
• Retinoschisis is predominantly extrafoveal (76.7%) and located at the dome base

The Dual Role of DSM: Protective vs. Pathological

DSM exhibits a unique duality:

• Protective: Limits foveal schisis, may improve post-cataract surgery visual outcomes in high myopia
• Pathological: Predisposes to SRD and RPE atrophy when dome height exceeds thresholds (~350–400 μm)

This threshold effect suggests moderate dome heights may be beneficial, while extreme bulges become pathological.

Diagnostic and Monitoring Recommendations

• Imaging: Multimodal approach with OCT (radial scans), OCT-A, and polarization-sensitive OCT
• Follow-up: Annual exams for stable cases; 3–4 monthly monitoring if dome height >350 μm, SRD present, or progressive choroidal thinning

Management Considerations

• SRD: Consider observation given benign natural history; treat if symptomatic with carbonic anhydrase inhibitors or subthreshold laser.
• CNV: Standard anti-VEGF protocols apply; DSM not a negative prognostic factor.
• Surgery: DSM may protect against macular hole retinal detachment.

Future Directions

• Threshold determination for protective vs. pathological effects
• Standardized imaging protocols
• Ethnic variability studies
• Longitudinal studies to optimize management

Conclusion

DSM is a multifactorial manifestation of ocular adaptation with significant clinical implications. Understanding its dual role—protective against some complications while predisposing to others—is crucial for individualized patient management. Continued research will refine thresholds, imaging techniques, and therapeutic strategies.