Macular corneal dystrophy: in vivo confocal and structural data

Area of Science:

• Ophthalmology
• Corneal Diseases
• Microscopy

Background:

• Macular corneal dystrophy (MCD) is a rare inherited condition affecting corneal clarity.
• Understanding the in vivo and ex vivo morphologic changes is crucial for diagnosis and management.
• Previous studies have utilized various microscopy techniques, but direct correlation in MCD patients is limited.

Purpose of the Study:

• To correlate in vivo confocal microscopy (CM) findings with light and electron microscopy of corneal specimens from MCD patients.
• To detail the specific corneal morphologic alterations in MCD using multiple high-resolution imaging techniques.
• To enhance the understanding of MCD pathogenesis through integrated microscopic analysis.

Main Methods:

• A case series design involving five consecutive patients with MCD undergoing penetrating keratoplasty (PK).
• Pre-operative examinations included slit-lamp, optical pachymetry, and in vivo CM.
• Corneal buttons from PK were processed for light, transmission electron microscopy (TEM), and scanning electron microscopy (SEM).

Main Results:

• In vivo CM revealed hyperreflective basal epithelial cells and rectilinear hyperreflective areas in the anterior stroma.
• Stromal changes included granular appearance, dark striae, and accumulation of electron-lucent material and undulated lamellae.
• Endothelial cells exhibited polymegethism and intracytoplasmic granules; histopathology confirmed thickened Bowman's layer and intracellular vesicles in keratocytes.

Conclusions:

• Histopathologic findings provide a clear explanation for the in vivo CM observations in MCD.
• The study enhances the understanding of MCD's structural pathology at a cellular level.
• Integrated microscopic analysis facilitates better noninvasive study of disease progression and evolution in vivo.