title: Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction.
abstract: A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8-10 years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a conical protrusion in the pupillary axis of the anterior lens capsule with a clear underlying lens. During surgery, central protruded fragile lens capsule was meticulously handled to create well-centered continuous curvilinear capsulorhexis by using paediatric rhexis forceps. Histopathology of excised capsule showed markedly thinned lens capsule with normal epithelium. Electron microscopy of the anterior lens capsule showed multiple linear and irregular zones of dehiscence. Few of these had fibrillar, irregular electron-dense material and vacuoles; and adjacent cells were irregular suggestive of Alport syndrome. Postoperative course was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction is an effective means of rehabilitating visually symptomatic anterior lenticonus of Alport syndrome.
authors: Department of Pediatric Ophthalmology, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.
authors: Shilpa
authors: S
authors: Sonarkhan
authors: Department of Cornea and Anterior Segment Services, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.
authors: Muralidhar
authors: M
authors: Ramappa
authors: Department of Cornea and Anterior Segment Services, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.
authors: Sunita
authors: S
authors: Chaurasia
authors: Department of Ocular Pathology Services, L V Prasad Eye Institute, Hyderabad, Andhra Pradesh, India.
authors: Kaustubh
authors: K
authors: Mulay
mesh_terms: D000328:Adult; D002386:Cataract / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D002387:Cataract Extraction; D005260:Female; D006801:Humans; D007903:Lens Capsule, Crystalline / Q000473:pathology* / Q000601:surgery; D007905:Lens Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery; D019654:Lens Implantation, Intraocular; D009394:Nephritis, Hereditary / Q000473:pathology*; D018918:Phacoemulsification; D014792:Visual Acuity