Asymmetric phenotype of Axenfeld-Rieger anomaly and aniridia associated with a novel PITX2 mutation
2011

Asymmetric Phenotype in Axenfeld-Rieger Syndrome

Sample size: 8 publication Evidence: moderate

Author Information

Author(s): Simon K. Law, Maha Piri Natik, Anne L. Coleman, Joseph Caprioli

Primary Institution: Jules Stein Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, CA

Hypothesis

Does the anterior segment phenotype of Axenfeld-Rieger syndrome show asymmetry between the two eyes?

Conclusion

The study found that variability in phenotype may occur between the two eyes of an individual affected by Axenfeld-Rieger syndrome.

Supporting Evidence

  • Eight patients with Axenfeld-Rieger syndrome were identified from over 5,000 screened.
  • One patient had an asymmetric phenotype with Axenfeld-Rieger anomaly in one eye and aniridia in the other.
  • A novel mutation in the PITX2 gene was identified in the patient with the asymmetric phenotype.

Takeaway

Some people with Axenfeld-Rieger syndrome can have different eye problems in each eye, which is unusual.

Methodology

The study reviewed medical records of patients with Axenfeld-Rieger syndrome and compared the anterior segment characteristics of both eyes.

Limitations

The study is based on a small sample size and retrospective data.

Participant Demographics

The study included eight patients with Axenfeld-Rieger syndrome from a tertiary glaucoma practice.

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