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Can true monocular keratoconus occur?

Anthony J Phillips¹

¹Contact Lens Unit, Deprtment of Ophthalmology, Flinders Medical Centre, Flinders University, Bedford Park, South Australia, Australia.

Clinical & Experimental Optometry

|November 25, 2003

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Keratoconus is often genetic, typically affecting both eyes. This case study presents a rare instance of true monocular keratoconus, highlighting extreme variable gene expression.

Area of Science:

Ophthalmology
Genetics

Background:

Keratoconus is increasingly recognized as a genetic disorder.
Variable gene expression often leads to differing severity in both eyes, resulting in pseudo-monocular cases.

Observation:

A rare case of true monocular keratoconus, present for over 23 years, is documented.
The unaffected eye showed normal corneal thickness and no signs of forme fruste keratoconus via videokeratoscopy.
No history of monocular eye rubbing was reported.

Findings:

The presented case demonstrates a unilateral manifestation of keratoconus, with advanced disease in one eye requiring corneal grafting.
Videokeratoscopy confirmed the absence of subclinical disease in the contralateral eye, ruling out forme fruste.
The subject's age suggests no future onset is likely.

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Implications:

This case represents an extreme example of variable gene expression in inherited keratoconus.
It underscores the importance of considering true monocular presentations, however rare, in the genetic understanding of keratoconus.