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Related Experiment Videos

Keratoconus associated with cone-rod dystrophy: a case report.

Rajesh Fogla1, Geetha K Iyer

  • 1Sankara Nethralaya Medical and Vision Research Foundations, Chennai, Tamil Nadu, India. mrf@sankaranethralaya.org

Cornea
|March 28, 2002
PubMed
Summary

Penetrating keratoplasty for keratoconus did not restore vision in one patient, who was later diagnosed with cone-rod dystrophy. Preoperative electrophysiologic testing may help predict outcomes in similar cases.

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Area of Science:

  • Ophthalmology
  • Genetics
  • Neuroscience

Background:

  • Keratoconus is a progressive thinning of the cornea, often associated with apical scarring.
  • Penetrating keratoplasty (corneal transplant) is a surgical option for advanced keratoconus.
  • Visual outcomes after corneal transplantation can vary, necessitating further investigation into prognostic factors.

Observation:

  • A 31-year-old male with bilateral keratoconus and apical scarring underwent successful penetrating keratoplasty in the left eye.
  • Postoperative visual acuity remained limited to 20/120, prompting further ocular examination.
  • Fundus examination revealed bull's eye maculopathy, and Ishihara testing indicated color blindness.

Findings:

  • Electoretinogram (ERG) studies demonstrated markedly delayed implicit times and reduced rod response amplitudes.

Related Experiment Videos

  • Cone waveforms were extinguished, confirming a diagnosis of cone-rod dystrophy.
  • Genetic counseling revealed this condition to be an isolated defect with no family history.

    • Implications:

    • This case highlights the potential for co-existing retinal dystrophies in patients with keratoconus.
    • Preoperative electrophysiologic assessments, including ERG, may offer prognostic value for visual outcomes after corneal surgery.
    • Identifying underlying retinal conditions is crucial for managing patient expectations and planning comprehensive eye care.