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Impressum
Hereditary pterygoid corneal dystrophy
Wolter-Roessler E., Seitz B., Naumann G. O. H.
Augenklinik mit Poliklinik der Universität Erlangen-Nürnberg, Schwabachanlange 6, 91054 Erlangen
Background: Pterygia are characterized by progressive growth of
fibrovascular tissue over the nasal limbus towards the corneal center
leaving the temporal cornea unaffected. Typically, they occur spontaneously
without well known etiology. Despite various primarily curative microsurgical
techniques recurrences are frequent.
Patients: Herein we report on three patients with a special type
of pterygium in one middle European family. They include an 81-year-old
female, her 61-year-old son and her 37-year-old grandson with bilateral
symmetrical affection and involvment of the nasal and temporal limbus.
An atypical "subepithelial wall" resembling Salzmann´sche
nodular degeneration was present at the central margin of the pterygia.
Both male patients had multiple excisiones (including lamellar keratoplasty)
but suffered from persistent recurrences, which led to sustainable reduction
of the visual acuity. By histologic examination the typical elastoid degeneration
of collagen fibres was found.
Conclusion: We propose an autosomal dominant heredity with varying
expression for this pterygoid corneal dystrophy. It does not seem to differ
histologically from normal pterygia, but recurrences appear to be more
frequent and more aggressive.