Genetics
Pediatric corneal opacities may be congenital or acquired. Congenital corneal opacities
Congenital reasons for this condition include:

Congenital hereditary endothelial dystrophy (CHED):  There are 2 forms of congenital hereditary endothelial dystrophy (CHED). Commonest is an autosomal recessive form, which is present at birth, but nonprogressive. Nystagmus is seen in association with this form. Another is an autosomal dominant form that occurs within the first few years of life. This form is progressive, but nystagmus is not seen. Deafness and CHED are seen in Harboyan syndrome. The histologic findings are very similar to those seen in pseudophakic/ aphakic bullous keratopathy. The appearance of the cornea is similar to that in congenital glaucoma but without increased corneal diameter and elevated intraocular pressure. Posterior polymorphous corneal dystrophy (PPMD, PPCD): PPCD, also known as Schlichting dystrophy, is an autosomal dominant disorder of the corneal endothelium and Descemet’s membrane. It is usually present in the second or third decade of life. It has the same entity as the first form of CHED. Most cases of PPMD are asymptomatic, and these cases generally do not require treatment. PPMD patients with bilateral, corneal opacities that can affect vision, descemet’s membrane endothelial keratoplasty or penetrating keratoplasty are the treatments of choice to improve vision and to avoid amblyopia. Congenital hereditary stromal dystrophy (CHSD): CHSD is also known as Congenital stromal corneal dystrophy or Congenital stromal dystrophy of the cornea. It is a rare autosomal dominant disease caused by mutations in the DCN gene. In this bilateral Snowflake, whitish opacities appear throughout the cornea.
In severe cases, a combination of systemic glucocorticoids and broad-spectrum antibiotics has been recommended. Epidemiology
The disorder typically appears among young girls and adolescents but cases in children as young as 17 months have been reported. History
The disease was first described in October 1912 by Galician-born Austrian dermatologist and microbiologist Benjamin Lipschütz, who published a series of four cases in girls aged 14 to 17. He initially ascribed the ulcer to infection with "Bacillus crassus" (Lactobacillus acidophilus). See also
Vulvovaginal health

References


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