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Peters anomaly - External and Internal Eye
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Peters anomaly - External and Internal Eye

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Contributors: Harold E. Cross MD, PhD, Lowell A. Goldsmith MD, MPH, Brandon D. Ayres MD, Christopher Rapuano MD, Harvey A. Brown MD, Sunir J. Garg MD, Lauren Patty Daskivich MD, MSHS
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Synopsis

Peters anomaly is a congenital ocular condition with variable features of anterior segment dysgenesis. The essential clinical signs are a central or paracentral corneal scar (leukoma) with iris adhesions to the posterior surface (Type I). Occasionally the lens is adherent to the same area and contains some opacification (cataract) as well (Type II). These malformations are nonprogressive and are present unilaterally as an isolated finding, although they can be bilateral. The Peters-plus syndrome has both bilateral findings of Peters anomaly as well as systemic manifestations. The condition in most cases seems to be inherited in an autosomal recessive pattern as the result of mutations in a number of regulatory homeobox genes. Since these are primarily active during embryogenesis of ocular structures, it is not uncommon to find some features of Peters anomaly in other ocular conditions.

Peters anomaly is of clinical importance not only for its potential impact on vision, but because it is frequently associated with congenital glaucoma, for which early diagnosis and treatment is essential to the preservation of vision.

For more information on Peters anomaly, see OMIM.

For more information on Peters-plus syndrome, see OMIM.

Codes

ICD10CM:
Q13.4 – Other congenital corneal malformations

SNOMEDCT:
204153003 –  Irido-corneo-trabecular dysgenesis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The discrete unilateral corneal scar is diagnostic, although it must be distinguished from the more diffuse corneal opacification of corneal dystrophies or congenital glaucoma.
  • An infected corneal ulcer (fungal corneal ulcer, bacterial corneal ulcer) can also lead to a localized opacity, but the eye is usually inflamed with considerable conjunctival injection.
  • In rare cases, perforation with an amniocentesis needle or trauma during forceps delivery can cause a corneal scar.
  • An ocular dermoid (see epibulbar dermoid cyst) has some similarities to the corneal scar in Peters anomaly but is usually elevated and is typically found at the limbus with extension posteriorly into the retrolimbal region.

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 03/29/2017
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Peters anomaly - External and Internal Eye
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Peters anomaly : Corneal opacities, Visual impairment, Iris abnormality
Clinical image of Peters anomaly
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