Pellucid Marginal Degeneration: An Overview
Pellucid marginal degeneration (PMD) is a rare, bilateral, non-inflammatory corneal ectasia characterised by a peripheral band of inferior thinning with relative central sparing. Patients usually present in early to mid-adulthood with progressive visual distortion from high against-the-rule and irregular astigmatism. Clinical examination typically reveals a clear, quiet eye with a crescentic band of thinning located 1–2 mm from the inferior limbus. Corneal topography and tomography are essential for diagnosis and commonly show a “crab-claw” pattern of inferior steepening with corresponding thinning on pachymetry. PMD may be misdiagnosed as keratoconus, particularly when only anterior curvature maps are reviewed. Non-surgical management focuses on optical rehabilitation with spectacles in early disease and rigid, corneoscleral or scleral contact lenses in more advanced cases. Corneal collagen cross-linking (CXL) has emerged as a strategy to stabilise progression, although evidence is largely limited to small series and requires modification of protocols for thin peripheral corneas. Intracorneal ring segments (ICRS) can regularise corneal shape in selected patients, while lamellar or penetrating keratoplasty is reserved for advanced disease, scarring or contact lens intolerance. Overall prognosis is favourable with early recognition, judicious use of contact lenses and targeted surgical intervention, although keratoplasty in PMD can be more challenging than in keratoconus with a higher risk of residual astigmatism.
