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Dystrophies of the cornea in children
Last reviewed: 19.11.2021
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Dystrophies of the cornea, usually represent bilateral and symmetrical disorders of a hereditary nature. Distinctions of the cornea and degenerations that do not have a genetic basis and develop against the background of aging or previous inflammation of the cornea.
Changes in the cornea, in appearance resembling a geographical map and fingerprints; specks; microcysts Kogan, degeneration of the basal membrane. Memorable fingerprints of opacity, localized beneath the epithelial layer. Visual acuity is reduced rarely. The disorder is inherited by an autosomal dominant type and is not associated with general pathology. The only significant complication of the disease is recurrent erosion.
Juvenile epithelial dystrophy; Dystrophy of Mesman-Wilke. Autosomal dominant type of inheritance with incomplete penetrance. The main clinical manifestation is the formation of tiny bubbles in the epithelial layer. The irritation of the eyeball and photophobia are caused by recurrent erosions. Vision is reduced rarely. With a curative purpose, contact lenses can be prescribed.
Dystrophy of Rais-Buckler (Reis-Bucklers). Cases that make their debut in early childhood are characterized by an autosomal dominant type of inheritance. Clinical manifestations include intermittent photophobia, pain and redness of the eyeball. Microscopic subepithelial protrusions are associated with the deposition of pathological substances at the level of the Bowman membrane. The sensitivity of the cornea usually suffers. Reduced vision occurs in the third or fourth decade of life. You may need keratoplasty.
Nodular dystrophy; Grenouva (Groenow) type I. Type of inheritance is autosomal-dominant. Characteristic of constant expressiveness in all generations. The disease is associated with the pathology of the chromosome 5q. There is a formation of small opacities in the form of nodules, originally located above the Bowman shell, and subsequently engaging the stroma of the cornea. The visual acuity remains normal for a long time. Through the fifth to sixth decade of life, a keratoplasty may be required.
Lattic degeneration; Dystrophy of the Haab-Biber-Dimmer (Haab-Biber-Dimmer) . The disease is inherited by autosomal dominant type and is associated with the pathology of the 5q chromosome. Often has an asymmetric character. It is associated with the deposition of amyloid substances in the cornea. A typical latticular form of the disease sometimes appears only in adulthood. End-to-end keratoplasty may be required. In some cases, this pathology of the cornea occurs against a background of general amyloidosis or a progressive paresis of the intracranial and peripheral nerves.
Spotted dystrophy; Grenouve type II. Autosomal recessive nature of inheritance. Rarely manifested in childhood. As the disease progresses, there is a thickening of the cornea and a gentle turbidity of its stroma. The need for end-to-end keratoplasty can occur only at distant stages of the development of pathology.
Central crystalline dystrophy of Schnyder. Autosomal dominant type of inheritance with different expressiveness. Characterized by the formation of discoid opacification in the center of the cornea with or without inclusion of cholesterol crystals. Pathology accompanies the lipoidal arch of the cornea. In adulthood, diffuse opacities are formed in the stromal layers, and as a result, a through keratoplasty is shown.
Dermochondrial dystrophy of the cornea. A rare disease characterized by dystrophic changes in the anterior cornea in the center. It is combined with cataracts, deformation of the limbus and nodular lesion of the skin.
Rear polymorphic dystrophy Schlichting (Schlichting). The disease is inherited by an autosomal dominant type. Characterized by the appearance of asymmetric, slowly progressing ring-shaped opacities, localized in the deep layers of the cornea at the descemet membrane level. Pathology manifests itself in childhood. It can be accompanied by a change in the depth of the anterior chamber and the development of glaucoma.
Momeni's Dystrophy (Maumenee). Inherited by autosomal dominant or autosomal recessive type. Debuts at birth. Diffuse avascular haze of a bluish-white color resembling a frosted glass. Gradually develops a progressive thickening of the cornea. Over time, turbidity is capable of spontaneous resorption. The need for through keratoplasty usually does not arise.
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