Leber’s Congenital Amaurosis and its Complications – A Very Rare Presentation
Leber’s congenital amaurosis is one of the most severe forms of retinal dystrophies responsible for congenital blindness. It accounts for 10%–18% of congenital blindness cases. It is characterized by at least three findings: severe and early visual impairment, sluggish or near-absent pupillary respo...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2024-01-01
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| Series: | Delhi Journal of Ophthalmology |
| Subjects: | |
| Online Access: | https://journals.lww.com/10.4103/DLJO.DLJO_99_23 |
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| Summary: | Leber’s congenital amaurosis is one of the most severe forms of retinal dystrophies responsible for congenital blindness. It accounts for 10%–18% of congenital blindness cases. It is characterized by at least three findings: severe and early visual impairment, sluggish or near-absent pupillary responses, and severely subnormal or nondetectable electroretinogram. Some patients exhibit solely retinal blindness and show evidence of multisystemic involvement. Here, in this article, we are presenting a case of complicated Leber’s congenital amaurosis with cataract and primary hyperplastic primary vitreous along with neurodevelopmental delay. |
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| ISSN: | 0972-0200 2454-2784 |