Abstract
IntroductionAlport Syndrome has a prevalence of 1 case per 5,000 people and 85% of patients have the X-linked form, where affected males develop renal failure and usually have high-tone sensorineural deafness by age 20. The main abnormality is deficient synthesis of type IV collagen, the main component of basement membranes. Common ocular abnormalities of this syndrome consist of dot-and-fleck retinopathy, posterior polymorphous corneal dystrophy, and anterior lenticonus, but other ocular defects such as cataracts, posterior lenticonus, and retinal detachments have also been reported.Case presentationWe report two cases of anterior lenticonus due to Alport Syndrome and describe clear lens phacoemulsification and foldable intraocular lens implantation as an effective and safe refractive procedure in the four eyes of these two patients.ConclusionAll four eyes of the two patients were in good condition after surgery and achieved satisfactory optical and visual results and had no remarkable complications at six-months follow-up. Clear lens phacoemulsification with foldable intraocular lens implantation can be used as an efficient and safe procedure for vision disorders in these patients.
Highlights
Alport Syndrome has a prevalence of 1 case per 5,000 people and 85% of patients have the X-linked form, where affected males develop renal failure and usually have high-tone sensorineural deafness by age 20
All four eyes of the two patients were in good condition after surgery and achieved satisfactory optical and visual results and had no remarkable complications at six-months followup
The main abnormality of this syndrome is deficient synthesis of type IV collagen, which is the main component of basement membranes in the human body
Summary
AL is a rare condition in which severe myopia and lenticular irregular astigmatism may decrease the patient's vision. Correction of this type of visual disturbance is extremely difficult, but refractive lens exchange seems to be a valuable solution. The results of this study are in agreement with the previous reports [69]. We recommend clear lens phacoemulsification with foldable IOL implantation as a safe and efficient procedure in AL patients. We preferred to use general anesthesia for the procedure in our cases because of the hearing problems and other mentioned difficulties in our patients
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