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Kamiar Mireskandari, Asim Ali, Uri Elbaz, Hermina Strungaru; Characterizing the phenotypic spectrum of Peters anomaly: from mild to severe disease . Invest. Ophthalmol. Vis. Sci. 2015;56(7 ):1553.
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© ARVO (1962-2015); The Authors (2016-present)
To characterize the wide phenotypic spectrum of Peters anomaly-affected eyes and to suggest a simple treatment scheme adjusted to this phenotypic variation.
The charts of all children diagnosed with Peters anomaly between January 2000 to December 2013 were reviewed retrospectively. Anterior segment photographs, optical coherence tomography and ultrasound biomicroscopy images were reviewed to assess structural morbidity. Patients were stratified according to disease severity based on localization of corneal opacity and lens involvement. The main outcome measures included laterality, location of corneal opacity, grade of corneal opacity density, presence of iris adhesions, lens involvement, presence of central corneal vessels, anterior conjunctival insertion, microphthalmos and posterior stromal thinning at first visit as well as associated systemic findings.
Eighty eyes of 54 patients (27 males and 27 females) participated in the study. Twenty-eight patients had unilateral disease and 26 patients had bilateral disease. Sixty-one eyes (76.3%) had a centrally located opacity and 56 eyes (70.0%) had a dense opacity obscuring iris details. Sixteen eyes (20.0%), 32 eyes (40.0%), 69 eyes (86.3%) and 29 eyes (36.3%) presented with central corneal vascularization, anterior conjunctival insertion, iridocorneal adhesions and posterior corneal thinning, respectively. Microphthalmos was present in 22 eyes (27.5%). Seventeen eyes (21.3%), 18 eyes (22.5%), 1 eye (1.3%) and 1 eye (1.3%) had keratolenticular adhesions or apposition, cataract, aphakia and lens remnant, respectively. Peters type II disease, defined as having at least one of these latter signs, was present in 23 eyes (28.8%) and Peters Type I disease was present in 57 eyes (71.3%). Associated systemic abnormalities were common occurrence (20 patients, 37.0%) in peters anomaly patients.
Peters anomaly presents with a variable phenotype ranging from minimal peripheral corneal opacity not affecting vision to extensive iris and lens adhesions with dense central corneal opacity detrimental to vision. This should be recognized by the paediatric ophthalmologist as management is tailored to the patient according to disease severity. In addition, a high index of suspicion for systemic abnormalities should be raised when Peters anomaly is diagnosed.
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