Clear lens extraction in Alport syndrome with combined anterior and posterior lenticonus or ruptured anterior lens capsule

Gupta, Arvind; Babu, K Ramesh; Srinivasan, Renuka; Mohanty, Debasish

doi:10.1016/j.jcrs.2011.09.005

Cited by 25 publications

(28 citation statements)

References 15 publications

(32 reference statements)

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“…Mutation in the gene that codes for these constituents will result in capsule fragility leading to conical protrusion of the capsule at its weakest point and rarely can it get ruptured. Anterior lenticonus is more commonly seen than posterior, which leads to progressive lenticular myopia developing by the second decade as seen in our patient 4 5. In our case, axial length and corneal curvature were normal, which further substantiate that the myopia was lenticular in origin.…”

Section: Discussionsupporting

confidence: 74%

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Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction

Sonarkhan

Ramappa²,

Chaurasia

et al. 2014

BMJ Case Reports

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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.

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Section: Discussionsupporting

confidence: 74%

“…All the intracapsular manoeuvers should be performed with utmost caution to avoid posterior capsular rupture. Acrylic intra-ocular lens with square edged designs were used to minimise capsular opacification 5 6…”

Section: Discussionmentioning

confidence: 99%

Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction

Sonarkhan

Ramappa²,

Chaurasia

et al. 2014

BMJ Case Reports

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show abstract

“…[456] Fragile lens capsule makes capsulorrhexis difficult, and it tends to go to periphery. [346] Hence, we performed it under DisCoVisc in two stages, initially, a small capsulorrhexis of about 3 mm and then enlargement to 5.5 mm.…”

Section: Discussionmentioning

confidence: 99%

“…However, lenticonus is known to develop and progress in the second decade, and hence amblyopia is usually not seen; as has also been reported in previous studies. [5] The cause of nonimprovement even with glasses correction is due to the inability of the eye to focus rays at the fovea due to irregular refraction by the conus. This was illustrated by inability of the fundus camera to focus macula preoperatively.…”

Section: Discussionmentioning

confidence: 99%

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Toric multifocal intraocular lens implantation in a case of bilateral anterior and posterior lenticonus in Alport syndrome

Ladi¹,

Shah²

2016

Indian J Ophthalmol

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We report the first case of toric multifocal intraocular lens (IOL) implantation in both the eyes of a young patient of Alport syndrome with anterior and posterior lenticonus with a successful outcome. An 18-year-old female patient presented with progressively blurred vision in both eyes since 4–5 years not improving with glasses. Refraction showed high myopia with astigmatism; however, the vision did not improve beyond 6/60 with glasses correction. Clinical examination on slit lamp showed anterior and posterior lenticonus bilaterally with a classical oil droplet appearance. We performed clear lens extraction by phacoemulsification with toric multifocal IOL implantation in both eyes. Postoperatively, the patient achieved an excellent refractive outcome with the unaided vision of 6/9, N6 in both eyes.

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Ocular Manifestations of Pediatric Renal Diseases

Kurup

Keswani

Enzenauer

2017

The Eye in Pediatric Systemic Disease

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Clear lens extraction in Alport syndrome with combined anterior and posterior lenticonus or ruptured anterior lens capsule

Abstract: No author has a financial or proprietary interest in any material or method mentioned.

Cited by 25 publications

References 15 publications

Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction

Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction

Toric multifocal intraocular lens implantation in a case of bilateral anterior and posterior lenticonus in Alport syndrome

Ocular Manifestations of Pediatric Renal Diseases

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