title: Pathological and Immunohistochemical Alterations of the Cornea in Congenital Corneal Opacification Secondary to Primary Congenital Glaucoma and Peters Anomaly.
abstract: OBJECTIVE
  To examine the immunohistochemical alterations in the corneal stroma in Peters anomaly (PA) and congenital glaucoma (CG) compared with age-matched normals and acquired adult corneal scarring (AACS).
  
  
  METHODS
  The clinical features of PA and CG patients who underwent penetrating keratoplasty were recorded. Immunohistochemistry of cornea and control tissue (normal and acquired corneal scars) was performed with antibodies against collagen types I, III, keratan sulfate, lumican, decorin, and smooth muscle actin followed by semiquantitative analysis of immunolabeling.
  
  
  RESULTS
  Clinical features in 2 groups were consistent with PA and CG. Microscopy showed thickened stromal collagen bundles in PA (n = 15), CG (n = 11), and AACS (n = 20) compared with normals (n = 18). PA and CG had distinct immunophenotypes compared with controls. Type I collagen labeling was more intense in CG compared with PA (intensity grading (IG) 2.73 vs. 2.07; P < 0.001). Decorin, lumican, and keratan sulfate labeling was significantly less intense in PA versus AACS (IG; 1.91, 0.38, 1.75 in PA and 2.7, 1.11, 2.61 in AACS. respectively; P = 0.002, P = 0.001 and P = 0.004) and normals (IG 1.92, 1.06, 2.59 respectively; P < 0.001, P < 0.001 and P = 0.005). Collagen I labeling was less intense in CG versus AACS (IG 2.73 vs. 3.09) (P = 0.007). Collagen III labeling was more intense in PA/CG than in normals (IG 0.9, 0.64, 0.62 retrospectively) (P < 0.001 in both).
  
  
  CONCLUSIONS
  The immunophenotype of the corneal scar in PA and CG differs from normal corneas and so does PA from AACS. The similarities between CG and AACS suggest that CG scarring has an acquired component.
authors.affiliation: *King Khaled Eye Specialist Hospital, Riyadh, Kingdom of Saudi Arabia; and †Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD.
authors.forename: Mohammed
authors.initials: M
authors.lastname: Al Shamrani
authors.forename: Khitam
authors.initials: K
authors.lastname: Al Hati
authors.forename: Hind
authors.initials: H
authors.lastname: Alkatan
authors.forename: Mosa
authors.initials: M
authors.lastname: Alharby
authors.forename: Sabah
authors.initials: S
authors.lastname: Jastaneiah
authors.forename: Jonathan
authors.initials: J
authors.lastname: Song
authors.forename: Deepak P
authors.initials: DP
authors.lastname: Edward
mesh_terms: D000293:Adolescent; D000869:Anterior Eye Segment / Q000002:abnormalities* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D011508:Chondroitin Sulfate Proteoglycans / Q000378:metabolism; D024042:Collagen Type I / Q000378:metabolism*; D024061:Collagen Type III / Q000378:metabolism*; D003315:Cornea / Q000378:metabolism* / Q000473:pathology; D003318:Corneal Opacity / Q000150:complications / Q000209:etiology / Q000378:metabolism* / Q000601:surgery; D058575:Decorin / Q000378:metabolism; D005124:Eye Abnormalities / Q000150:complications* / Q000601:surgery; D005260:Female; D006801:Humans; D006871:Hydrophthalmos / Q000150:complications* / Q000601:surgery; D007150:Immunohistochemistry; D007223:Infant; D007632:Keratan Sulfate / Q000378:metabolism; D015948:Keratoplasty, Penetrating; D000071234:Lumican; D008297:Male; D011509:Proteoglycans / Q000378:metabolism*; D012189:Retrospective Studies
chemical_list: D011508:Chondroitin Sulfate Proteoglycans; D024042:Collagen Type I; D024061:Collagen Type III; D058575:Decorin; C000604306:LUM protein, human; D000071234:Lumican; D011509:Proteoglycans; D007632:Keratan Sulfate