2020–2021 BCSC Basic and Clinical Science Course™
8 External Disease and Cornea
Chapter 5: Congenital Anomalies of the Cornea and Sclera
Secondary Abnormalities Affecting the Fetal Cornea
Congenital Corneal Keloid
Corneal keloids are relatively rare lesions, most commonly occurring following corneal perforation, trauma, or surgery. Congenital corneal keloids, which are often bilateral, have been described in Lowe (oculocerebrorenal) syndrome (an X-linked recessive disorder characterized by cataracts, renal failure, intellectual disability, and seizures), Rubinstein-Taybi syndrome, and the ACL syndrome (acromegaly, cutis verticis gyrata, corneal leukoma syndrome). Autosomal dominant inheritance has been observed in the ACL syndrome. Corneal keloids can occur in association with cataracts, aniridia, and glaucoma and may represent a developmental anomaly with failure of normal differentiation of corneal tissue. Histologic examination reveals thick collagen bundles haphazardly arranged, with focal areas of myofibroblastic proliferation.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.