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Alport syndrome revealed by a decrease in visual acuity

Poster Details


First Author: M.Essakhi MOROCCO

Co Author(s): I. Adnane   I. Elboukhani   A. Mchachi   L. Benhmidoune   R. Rachid   M. Elbelhadji     

Abstract Details

Purpose:

Alport syndrome is a rare inherited disease characterized by the association of progressive hematuric nephropathy, bilateral deafness and ocular manifestations. Alport syndrome accounts for 2% of end stage nephropathies in Europe and the USA. We report a case of Alport syndrome revealed by a decrease in visual acuity

Setting:

Service d'ophtalmologie adulte. hopital 20 Aout 1953, CHU Ibn Rochd, Université Hassan II. Casablanca

Methods:

A 29-year-old woman consulted for a rapidly progressive bilateral decrease in visual acuity predominating in her left eye two years prior to her consultation. The patient is followed for chronic kidney disease since childhood with secondary hypertension. In the past 2 years she had severe hematuria attacks. Her brother had died of chronic renal disease at the age of fifteen. There was no other similar case in the family. In the ophthalmic examination, the visual acuity of the right eye was 3/10 and in the left eye 1/10, it did not improve after optical correction

Results:

Slit-lamp examination revealed bilateral anterior lenticonus in both eyes and posterior subcapsular cataract  on the left eye.  Multiple white flecks were detected in the macula sparing the foveolar and parafoveal regions in both eyes. The peripheral fundus was apparently normal. Alport syndrome was suspected and was confirmed by a renal biopsy. An audiogram was done showing bilateral deafness. The patient was programmed for phacoemulsification of the left eye with a foldable posterior chamber intraocular lens. After three months of follow-up, the visual acuity of the left eye increased to 6/10.

Conclusions:

Ophthalmologist plays an important role in early detection of Alport Syndrome. This disease must be suspected in young patients followed by chronic kidney disease associated with ocular and auditory functional signs.

Financial Disclosure:

None

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