Pellucid Marginal Degeneration: A Comprehensive Review of Pathophysiology, Diagnosis, and Management Strategies

Abstract

Purpose: Pellucid Marginal Degeneration (PMD) is a rare ectatic corneal disorder characterized by inferior peripheral thinning and significant irregular astigmatism. Despite its clinical similarities to keratoconus, PMD presents unique diagnostic and therapeutic challenges. This review aims to provide a comprehensive update on the pathophysiology, clinical features, diagnostic approaches, and management strategies for PMD, emphasizing the latest advancements in treatment options. Methods: A systematic literature search was performed in MEDLINE (via PubMed), Google Scholar, and Scopus up to February 2025 using the terms: "pellucid marginal degeneration," "PMD," "ectatic corneal disorders," "keratoplasty in PMD," "corneal cross-linking in PMD," "ICRS in PMD," "toric IOL PMD" and their Boolean combinations (AND/OR). The search was restricted to English-language studies involving human subjects, including case reports, case series, retrospective studies, clinical trials, and systematic reviews. A total of 76 studies met the inclusion criteria addressing treatment outcomes in PMD. Results: PMD is characterized by a crescent-shaped band of inferior corneal thinning, leading to high irregular astigmatism and reduced visual acuity. Diagnosis relies on advanced imaging techniques such as Scheimpflug-based corneal tomography, which reveals the characteristic "crab-claw" pattern. Conservative management includes rigid gas-permeable (RGP) lenses and scleral lenses, which provide effective visual rehabilitation in mild to moderate cases. Surgical options, such as CXL, ICRS, and toric IOLs, are reserved for advanced cases, with varying degrees of success. Newer techniques such as CAIRS, employing donor tissue instead of synthetic rings, show promising outcomes in corneal remodeling with potentially improved biocompatibility. Penetrating keratoplasty (PK) and deep anterior lamellar keratoplasty (DALK) remain definitive treatments for severe PMD, though they are associated with significant risks, including graft rejection and postoperative astigmatism. Conclusions: PMD is a complex and progressive corneal disorder that requires a tailored approach to management. Early diagnosis and intervention are critical to optimizing visual outcomes. While conservative measures are effective in mild cases, surgical interventions offer promising results for advanced disease. Further research is needed to refine treatment protocols and improve long-term outcomes for patients with PMD.

Keywords: CAIRS; corneal ectasia; corneal graft; corneal wedge excision; pellucid marginal degeneration.

Figure 1

Slit-lamp image collage of PMD. Picture (A) shows inferior thinning with a beer-belly appearance while picture (B) shows the ectatic zone extending in a crescent-shaped pattern from the 4 to 8 o’clock positions. Arrow pointing the area of maximal thinning typically located 1–2 mm from the limbus.

Figure 2

Tomographic image of Axial Sagittal Curvature map showing the crab claw appearance of PMD on (a) sagittal curvature map; (b) corneal thickness, showing greatest thinning inferiorly near the limbus; and (c) anterior and (d) posterior elevation perpendicular to the thinning.