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Corneal dystrophies in children
Last reviewed: 07.07.2025
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Corneal dystrophies are usually bilateral and symmetrical disorders of a hereditary nature. There are corneal dystrophies and degenerations that do not have a genetic basis and develop against the background of aging or previous inflammation of the cornea.
Corneal changes resembling a geographical map and fingerprints; spots; Kogan microcysts, basal membrane dystrophy. Opacities resembling fingerprints localized under the epithelial layer. Visual acuity is rarely reduced. The disorder is inherited in an autosomal dominant manner and is not associated with general pathology. The only significant complication of the disease is recurrent erosions.
Juvenile epithelial dystrophy; Meesman-Wilke dystrophy. Autosomal dominant inheritance with incomplete penetrance. The main clinical manifestation is the formation of tiny bubbles in the epithelial layer. Irritation of the eyeball and photophobia are due to recurrent erosions. Vision is rarely impaired. Contact lenses may be prescribed for therapeutic purposes.
Reis-Bucklers dystrophy. Cases with onset in early childhood are inherited in an autosomal dominant pattern. Clinical manifestations include intermittent photophobia, pain, and redness of the eyeball. Microscopic subepithelial protrusions are associated with deposition of pathological substances at the level of Bowman's membrane. Corneal sensitivity is usually impaired. Visual impairment occurs in the third or fourth decade of life. Keratoplasty may be required.
Nodular dystrophy; Groenow type I. The inheritance pattern is autosomal dominant. Constant expressivity is characteristic in all generations. The disease is associated with chromosome 5q pathology. The formation of small opacities in the form of nodules is observed, initially located above the Bowman's membrane, and later capturing the corneal stroma. Visual acuity remains normal for a long time. In the fifth or sixth decade of life, penetrating keratoplasty may be required.
Lattice dystrophy; Haab-Biber-Dimmer dystrophy. The disease is inherited in an autosomal dominant manner and is associated with abnormality of chromosome 5q. It is often asymmetrical. It is associated with deposition of amyloid substances in the cornea. The typical lattice form of the disease sometimes appears only in adulthood. Penetrating keratoplasty may be required. In some cases, this corneal pathology occurs against the background of general amyloidosis or progressive paresis of the intracranial and peripheral nerves.
Spotted dystrophy; Grenowa type II. Autosomal recessive inheritance. Rarely manifests itself in childhood. As the disease progresses, thickening of the cornea and gentle opacity of its stroma occur. The need for penetrating keratoplasty may arise only at late stages of the pathology development.
Schnyder's central crystalline dystrophy. Autosomal dominant inheritance with variable expressivity. Characterized by the formation of a discoid opacity in the center of the cornea with or without cholesterol crystals. The pathology is accompanied by a lipoid arc of the cornea. In adulthood, diffuse opacity is formed in the stromal layers, which is why penetrating keratoplasty is indicated.
Dermochondrial corneal dystrophy. A rare disease characterized by dystrophic changes in the anterior layers of the cornea in the center. Combined with cataracts, limbus deformation and nodular lesions of the skin.
Posterior polymorphic dystrophy of Schlichting. The disease is inherited in an autosomal dominant manner. It is characterized by the appearance of asymmetric, slowly progressing ring-shaped opacities localized in the deep layers of the cornea at the level of Descemet's membrane. The pathology manifests itself already in childhood. It can be accompanied by a change in the depth of the anterior chamber and the development of glaucoma.
Maumenee dystrophy. Inherited in an autosomal dominant or autosomal recessive manner. Debuts at birth. Diffuse avascular opacity of a bluish-white color, resembling ground glass. Progressive thickening of the cornea gradually develops. Over time, the opacity is capable of spontaneous resorption. Penetrating keratoplasty is usually not necessary.
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