A Case Report: Bilateral Lenticonus In Alport Syndrome

Objective: To report a case of bilateral lenticonus with Alport Syndrome.Methods : We described and analyzed a case with Alport Syndrome,who has anterior and posterior lenticonus in the right eye,and anterior lenticonus in the left eye complicated with anterior polar cataract.Results: A 39-year-old male patient came to the Ophthalmic Department in the First Affiliated Hospital of Soochow University in July of 2016.He complained painless and progressive deterioration of vision in both eyes for several years and worsening symptom in left eye for two months.On examination,uncorrected visual acuity was 20/166 in the right eye and 20/100 in the left eye.Best corrected visual acuity in the right eye was 20/40 and uncorrectable in the left eye.A typical "oil-droplet" sign and conical protrusion of the clear lens in the right eye anteriorly and posteriorly as well as front bulge and opacity of the left lens were found by slit-lamp examination.Scheimpflug imaging showed evident bulge of bilateral lens into anterior chamber.Retinal topography suggested temporal retinal thinning in both eyes.The patient had a history of re nal failure and sensorineural hearing loss and also had family history of nephritis,leading to clinical diagnosis of Alport Syndrome.With appropriate operative indications,phacoemulsification and intraocular lens implantation performed in left eye,with visual acuity improved to 20/25 and corrected to 20/20 one month after surgery.To assess the visual quality of the right eye,iTrace wavefront analyzer examination was taken and showed severe aberration from inside the eye.6 months later,best corrected visual acuity of right eye fell to 20/133,thus clear lens extraction with intraocular lens implantation was performed.O ne month after the second surgery,visual acuity in right eye was 20/33 and reached 20/20 by correcting.Fundus photography showed peri-macular dot and fleck retinopathy,while visual field and pattern visual evoked potential examination were nearly normal.The capsule of lens from surgery underwent a5(?)immunohistochemical staining and gave an abnormal result,defining the dignosis of Alport Syndrome.Conclusion: Ocular features in Alport Syndrome are specific and useful diagnostically.As soon as the formation of lenticonus affects visual function obviously,even if lens is clear,surgery is recommended.Early treatment of lenticonus may improve life quality and reduce surgery complications in a great deal.Immunohistochemical staining for a5(?)in lens capsule may be considered as another method for defining Alport Syndrome.