By W. Lisch, B. Seitz, F. Bandello
Corneal dystrophies (CD) are bilateral hereditary problems of the cornea during which one or a number of elements of the cornea lose their transparency. because the dystrophy can begin in several layers of the cornea, they're labeled therefore as epithelial dystrophies, stromal dystrophies and endothelial dystrophies. This quantity contains a description of the hot foreign IC3D type of CD reflecting what we at present understand of the medical, pathological, and genetic elements of those issues. additional contributions supply an perception into differential diagnostics and histology, that may make sure the prognosis as for example in granular CD kind 2, in addition to DNA research of CD offering additional info concerning the pathogenesis. additionally, methods comparable to the phototherapeutic keratectomy with the excimer laser, the hot modalities of lamellar keratoplasty, and penetrating keratoplasty are defined. This book holds a wealth of latest and topical info on CD for ophthalmologists and geneticists alike.
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Additional info for Corneal Dystrophies (Developments in Ophthalmology)
3 Hidayat AA, Risco JM: Amyloidosis of corneal stroma in patients with trachoma. A clinicopathologic study of 62 cases. Ophthalmology 1989;96: 1203–1211. 4 Aldave AJ, Principe AH, Lin DY, et al: Lattice dystrophy-like localized amyloidosis of the cornea secondary to trichiasis. Cornea 2005;24:112–115. 5 Robin AL, Green WR, Lapsa TP, et al: Recurrence of macular corneal dystrophy after lamellar keratoplasty. Am J Ophthalmol 1977;84:457–461. Histological Landmarks in Corneal Dystrophy 6 Weiss JS, Orlando F, Albert DM: The usefulness of routine alcian blue staining of corneal specimens.
The mild form is characterized by subepithelial amyloid nodules; the moderate form shows amyloid deposits in a sheet-like pattern in the subepithelial region and superficial stroma (fig. 1a). These deposits correspond to the gelatinous masses seen clinically and stain brick red with Congo red stain (fig. 1b). It is interesting to note that despite significant amounts of amyloid being deposited in the anterior stroma and in the subepithelial region, there is no overall increase in the thickness of the cornea.
Curly fibers are immunopositive for transforming growth factor β-induced protein (keratoepithelin) in TBCD (5q31). Other Imaging Modalities. Confocal microscopy shows distinct deposits in the epithelial basal layer, which exhibits homogenous reflectivity with round edges accompanying a dark shadow. This is less reflective as compared to RBCD. Grayson-Wilbrandt Corneal Dystrophy Light Microscopy. PAS-positive material is noted between Bowman’s layer and the epithelium, which stains negative with Alcian blue and Masson’s trichrome stain [50, 51].