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Alport syndrome’s lenticonus, surgical management and optical considerations: a case report

Poster Details


First Author: K.Bradly BELGIUM

Co Author(s): K. Bradly   P. Zeboulon   D. Gatinel              

Abstract Details

Purpose:

We report a case of a male patient who suffered from progressive vision loss due to both anterior lenticonus and Posterior Polymorph Corneal Dystrophy (PPCD), and who underwent clear lens extraction surgery. We then discuss optical considerations of Alport Syndrome’s lenticonus and its consequences on the ocular wavefront.

Setting:

Alport syndrome is a hereditary basement membrane disease that causes nephritis, hearing loss and ocular impairments. The most frequent ocular manifestations are anterior lenticonus and fleck retinopathy. Anterior lenticonus impairs the quality of vision due to important higher order aberrations.

Methods:

We performed clear lens extraction surgery and analysed optical and anatomical changes, using aberrometry (OPD scan III, Nidek, Japan) and high resolution OCT (Anterion, Heidelberg, Germany) among other tests.

Results:

At one-month follow-up, the patient had regained a BCVA of 20/32, compared to 20/50 pre-operatively, with refractive measures of +1.75 (-1.75 x105). OPD scan III (NIDEK, Japan) showed the reduction of the negative spherical aberration and it also showed that the anterior chamber depth (ACD) widened postoperatively.

Conclusions:

Anterior lenticonus is a common ocular manifestation of Alport syndrome that induces an important myopic shift and important negative spherical aberration impairing the patient’s visual acuity. Clear lens extraction is a challenging but feasible treatment as long as the anatomic specificities of the patients are considered, especially the fragile lens capsule and the potential difficulties encountered for IOL power calculation.

Financial Disclosure:

None

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