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Ausgeprägte intrakorneale Keloidbildung bei Peters-Plus-Syndrom und bei einer Maximalvariante der Peters-Anomalie

Intensive intracorneal keloid formation in a case of Peters plus syndrome and in Peters anomaly with maximum manifestation

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Zusammenfassung

Wir stellen 2 Fälle der Peters-Anomalie (ein Peters-Plus-Syndrom und eine Maximalvariante) mit abnormal dicker Hornhaut und kornealem Staphylom vor. Die bei der perforierenden Keratoplastik entnommenen Hornhautscheibchen zeigten histologisch eine deutliche Dickenzunahme, die sich als vermehrte Bindegewebseinlagerung im Stroma im Sinne eines Keloids darstellte. Beide Augen zeigten die charakteristischen Veränderungen einer Peters-Anomalie mit Hornhauttrübung, Anhaftung der Linsen- und Irisvorderfläche an der Hornhautrückfläche, fehlendem Endothel, Descemet und Bowman-Schicht. Eine Peters-Anomalie mit einer intrakornealen Keloidbildung und einer kongenitalen Staphylombildung der Hornhaut stellt eine extrem seltene Fehlbildung dar.

Abstract

We present two cases of Peters anomaly (Peters plus syndrome and a maximum manifestation variant) with abnormally thickened cornea and corneal staphyloma. Both patients presented to our hospital shortly after birth and were treated with perforating keratoplasty and lensectomy. Histological analysis showed marked thickening of the corneal stroma due to abnormal stromal connective tissue deposition. Additionally, both eyes showed the characteristic changes of Peters anomaly with corneal opacity, adherence of the iris stroma and anterior lens surface to the posterior corneal surface, absence of the corneal endothelium, Descemet and Bowmans layers. Peters anomaly with abnormally thick intracorneal fibrosis with or without congenital corneal staphyloma is a very rare manifestation.

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Authors and Affiliations

  1. Universitäts-Augenklinik Freiburg, Killianstraße 5, 79106, Freiburg, Deutschland

    P. Eberwein, T. Reinhard, H. Agostini, C.M. Poloschek & C. Auw-Haedrich

  2. Universitäts-Augenklinik Rostock, Rostock, Deutschland

    R. Guthoff

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  1. P. Eberwein
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  2. T. Reinhard
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  3. H. Agostini
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  4. C.M. Poloschek
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  5. R. Guthoff
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  6. C. Auw-Haedrich
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Correspondence to P. Eberwein.

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Eberwein, P., Reinhard, T., Agostini, H. et al. Ausgeprägte intrakorneale Keloidbildung bei Peters-Plus-Syndrom und bei einer Maximalvariante der Peters-Anomalie. Ophthalmologe 107, 178–181 (2010). https://doi.org/10.1007/s00347-009-2014-1

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  • DOI: https://doi.org/10.1007/s00347-009-2014-1

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