Causes of congenital corneal opacities and their management in a tertiary care center

Arq Bras Oftalmol. 2020 Mar-Apr;83(2):98-102. doi: 10.5935/0004-2749.20200023.


Purpose: To evaluate causes and management of congenital corneal opacities (CCO) diagnosed in a tertiary care eye center and to compare the data with a previous study at the same institution.

Methods: Computerized medical records in all patients with congenital corneal opacities diagnosed in the Cornea Service at Wills Eye Hospital (Philadelphia, PA) between January 1, 2007, and December 31, 2015, were retrospectively reviewed. Children aged 12 years and younger at the first visit were included in the study. Patients' demographics, ocular diagnosis, laterality, associated ocular abnormalities, other ocular surgery performed prior or subsequent to the first visit, and their treatment were extracted from the medical records.

Results: A total of 77 eyes in 56 patients were examined. The mean age at presentation was 32.8 ± 44.2 months, with the mean follow-up period of 26.7 ± 30.1 months. The most frequent diagnosis was Peters anomaly (53.2%), followed by limbal dermoid (13.0%), aniridia with glaucoma and microphthalmos (6.5%), sclerocornea and congenital glaucoma (5.2%), idiopathic (3.9%), Axenfeld-Rieger anomaly and Hurler syndrome (2.6%), and microcornea (1.3%). Primary keratoplasty was performed in 26 eyes, with the outcome rate in the clear cornea of 76.0% during the follow-up.

Conclusion: Peters anomaly is the most common cause of congenital corneal opacities encountered at our institution. Penetrating keratoplasty is the most frequent choice of corneal surgery to treat congenital corneal opacities. Additional interventions during penetrating keratoplasty were moderately positively correlated with graft failure. This study also shows the rates of some etiologies of that changed over the recent decades in our tertiary care Cornea Service. Although Peters anomaly remains the most common presenting reason for congenital corneal opacities, its rate appears to be increasing over the recent decade. Congenital corneal opacities due to birth trauma, which is one of the preventable causes, were observed in a previous study in our clinic; however, no new cases were noted in this study.

MeSH terms

  • Anterior Eye Segment / abnormalities
  • Child
  • Child, Preschool
  • Cornea / abnormalities
  • Cornea / pathology
  • Corneal Diseases / complications
  • Corneal Opacity / complications
  • Corneal Opacity / congenital*
  • Corneal Opacity / surgery*
  • Eye Abnormalities / complications
  • Eye Diseases, Hereditary / complications
  • Female
  • Glaucoma / complications
  • Humans
  • Infant
  • Infant, Newborn
  • Keratoplasty, Penetrating / methods
  • Male
  • Medical Records
  • Retrospective Studies
  • Risk Factors
  • Statistics, Nonparametric
  • Tertiary Care Centers*
  • Treatment Outcome

Supplementary concepts

  • Axenfeld-Rieger syndrome
  • Peters anomaly
  • Sclerocornea