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Sclero-cornea and defective mesodermal migration.

A H Friedman, S Weingeist, A Brackup

    The British Journal of Ophthalmology
    |November 1, 1975
    PubMed
    Summary
    This summary is machine-generated.

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    This case study details a rare bilateral sclero-cornea with systemic abnormalities in an infant. Histopathology revealed severe corneal and anterior segment developmental defects, suggesting an early embryogenesis error.

    Area of Science:

    • Ophthalmology
    • Developmental Biology
    • Genetics

    Background:

    • Bilateral sclero-cornea is a rare congenital condition.
    • Associated systemic abnormalities can occur.
    • Normal karyotype in affected infants is possible.

    Purpose of the Study:

    • To describe the histopathological findings of a unique case of bilateral sclero-cornea.
    • To investigate the potential underlying cause of the severe ocular malformations.

    Main Methods:

    • Histopathological examination of an enucleated eye.
    • Clinical assessment of systemic abnormalities.
    • Karyotyping.

    Main Results:

    • The eye showed an irregular corneal epithelium, absent Bowman's and Descemet's membranes, and vascularized stroma.

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  • Absence of angle structures, iris/ciliary body hypoplasia, and kerato-iridic adhesions were noted.
  • The infant had multiple systemic abnormalities but a normal karyotype.

    • Conclusions:

    • A profound defect in early embryogenesis, specifically defective mesodermal migration, is postulated.
    • This defect likely underlies the severe ocular and systemic malformations observed.
    • Further research into early mesodermal development is warranted.