A 2-year-old male presents with a bilateral corneal problem. Examination shows glaucoma and bilateral axial corneal haze. The lenses are not cataractous and are not adherent to the retrocorneal surface.
Figure 1-peripheral cornea showing an intact Descemet’s and endothelial population.
Figure 2-PAS stain of figure 1
Figure 3-axial cornea
Figure 4-PAS stain of axial cornea.
1. What does Figure 3 show?
2. What does Figure 4 show?
3. What is the clinical differential diagnosis?
4. What is the likeliest diagnosis in this case?
5. What is the cause of the glaucoma in this patient?
1. Adherent retrocorneal iris type pigment.
2. Descemet’s tapering and then being absent where the iris type tissue is adherent.
3. Posterior keratoconus, sclerocornea, trauma, intrauterine keratitis, mucopolysaccharidoses, congenital hereditary endothelial dystrophy, corneal dermoids, posterior polymorphous dystrophy, Peters anomaly.
4. Peters anomaly. This is type 1 because the lens was not adherent to the axial cornea.
5. Angle dysgenesis.