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Pellucid corneal marginal degeneration: A review
Amit Jinabhai1, Hema Radhakrishnan, Clare O'Donnell
1The University of Manchester, England, UK.
Abstract:
Pellucid marginal corneal degeneration (PMD) is a rare ectatic disorder which typically affects the inferior peripheral cornea in a crescentic fashion. The condition is most commonly found in males and usually appears between the 2nd and 5th decades of life affecting all ethnicities. The prevalence and aetiology of this disorder remain unknown. Ocular signs and symptoms of patients with PMD differ depending on the severity of the condition. Unless corneal topography is evaluated, early forms of PMD may often be undetected however, in the later stages PMD can often be misdiagnosed as keratoconus. Visual signs and symptoms include longstanding reduced visual acuity or increasing against-the-rule irregular astigmatism leading to a slow reduction in visual acuity. In rare cases, patients may present with a sudden loss of vision and excruciating ocular pain due to corneal hydrops or spontaneous perforation. The vast majority of PMD patients are managed using spectacles and contact lenses. Several surgical procedures have been used in an attempt to improve visual acuity when spectacles and contact lenses do not provide adequate vision correction. Since patients with PMD make poor candidates for laser vision correction, an awareness of the topographical and slit-lamp features of PMD will be useful to clinicians screening for signs of corneal abnormality before corneal refractive surgery. This review describes the clinical features of PMD, its differential diagnosis and various management strategies presently available.
Insights
Pellucid marginal corneal degeneration (PMD) is a rare corneal disorder affecting the inferior periphery. Early diagnosis via corneal topography is crucial, as PMD can mimic keratoconus and impact vision.
Area of Science:
- Ophthalmology
- Corneal Diseases
- Ocular Surface Disorders
Background:
- Pellucid marginal corneal degeneration (PMD) is a rare, progressive ectatic disorder.
- It characteristically affects the inferior peripheral cornea in a crescentic pattern.
- The exact prevalence and etiology of PMD remain largely unknown.
Purpose of the Study:
- To review the clinical features, differential diagnosis, and management strategies for PMD.
- To highlight the importance of corneal topography in early detection.
- To inform clinicians about PMD's distinct characteristics to prevent misdiagnosis, especially with keratoconus.
Main Methods:
- Literature review of clinical features, diagnostic methods, and treatment options for PMD.
- Analysis of diagnostic challenges, including differentiation from keratoconus.
- Synthesis of current management approaches, from conservative to surgical.
Main Results:
- PMD typically affects males between the 2nd and 5th decades, regardless of ethnicity.
- Symptoms range from reduced visual acuity and irregular astigmatism to rare cases of sudden vision loss.
- Early PMD may be missed without corneal topography, while advanced stages can be misdiagnosed as keratoconus.
Conclusions:
- Accurate diagnosis of PMD relies on recognizing its unique topographical and slit-lamp features.
- Spectacles and contact lenses are primary management tools, with surgery for visual rehabilitation.
- Awareness of PMD is vital for clinicians, particularly before considering refractive surgery.

