Primary aphakia : clinical recognition is the key to diagnosis
Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved..
PURPOSE: To describe the presentation and treatment outcomes of a cohort of children with primary aphakia (PA).
METHODS: Clinical photographs and ultrasound biomicroscopy (UBM) images of children presenting with sclerocornea and undetermined anterior segment dysgenesis between July 2017 and December 2020 were reviewed retrospectively. Children who had no crystalline lens visible on UBM were included.
RESULTS: A total of 124 UBM images were captured for 124 children with cloudy corneas. Twelve children were identified with congenital primary aphakia: 5 had bilateral buphthalmos, 2 had buphthalmos in one eye and microphthalmos in the other, and 5 had bilateral sclerocornea-microphthalmia complex. All patients had a peculiar silvery-blue corneal appearance, with fine vascularization on the corneal surface. The overall corneal thickness was 409.1 ± 8.7 μm. The intraocular pressure (IOP) in eyes with glaucoma was 24.5 ± 7.3 mm Hg; in microphthalmic eyes, 11.4 ± 3.4 mm Hg (P <0.001). The raised IOP was treated with limited trans-scleral cyclophotocoagulation under transillumination and topical antiglaucoma medications. Children with glaucoma gained ambulatory vision with spectacles.
CONCLUSIONS: Congenital primary aphakia has a characteristic clinical appearance and may present as buphthalmos or microphthalmos, depending on the extent of dysgenesis. Incisional surgery may result in phthisis because of ciliary body dysgenesis and unpredictable aqueous production.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:26 |
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| Enthalten in: |
Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus - 26(2022), 6 vom: 30. Dez., Seite 298.e1-298.e5 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Kaushik, Sushmita [VerfasserIn] |
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| Anmerkungen: |
Date Completed 15.12.2022 Date Revised 21.12.2022 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.jaapos.2022.07.012 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM346994969 |
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| 500 | |a Date Revised 21.12.2022 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2022 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved. | ||
| 520 | |a PURPOSE: To describe the presentation and treatment outcomes of a cohort of children with primary aphakia (PA) | ||
| 520 | |a METHODS: Clinical photographs and ultrasound biomicroscopy (UBM) images of children presenting with sclerocornea and undetermined anterior segment dysgenesis between July 2017 and December 2020 were reviewed retrospectively. Children who had no crystalline lens visible on UBM were included | ||
| 520 | |a RESULTS: A total of 124 UBM images were captured for 124 children with cloudy corneas. Twelve children were identified with congenital primary aphakia: 5 had bilateral buphthalmos, 2 had buphthalmos in one eye and microphthalmos in the other, and 5 had bilateral sclerocornea-microphthalmia complex. All patients had a peculiar silvery-blue corneal appearance, with fine vascularization on the corneal surface. The overall corneal thickness was 409.1 ± 8.7 μm. The intraocular pressure (IOP) in eyes with glaucoma was 24.5 ± 7.3 mm Hg; in microphthalmic eyes, 11.4 ± 3.4 mm Hg (P <0.001). The raised IOP was treated with limited trans-scleral cyclophotocoagulation under transillumination and topical antiglaucoma medications. Children with glaucoma gained ambulatory vision with spectacles | ||
| 520 | |a CONCLUSIONS: Congenital primary aphakia has a characteristic clinical appearance and may present as buphthalmos or microphthalmos, depending on the extent of dysgenesis. Incisional surgery may result in phthisis because of ciliary body dysgenesis and unpredictable aqueous production | ||
| 650 | 4 | |a Journal Article | |
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| 700 | 1 | |a Kaur, Savleen |e verfasserin |4 aut | |
| 700 | 1 | |a Kaur, Anupriya |e verfasserin |4 aut | |
| 700 | 1 | |a Choudhary, Sandeeep |e verfasserin |4 aut | |
| 700 | 1 | |a Thattaruthody, Faisal |e verfasserin |4 aut | |
| 700 | 1 | |a Pandav, Surinder Singh |e verfasserin |4 aut | |
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