RESUMO
Purpose: to report three cases of Alport syndrome in one family with anterior lenticonus and retinal flecks
Patients and findings: three members of one family with consanguineous parents are presented who had renal and ocular involvement. They had anterior lenticonus and a beaten bronze appearance in the macula. Reduced visual acuity and photophobia were the chief complaints of these patients. Despite correction of her refractive error, one of them underwent clear lens extraction with intraocular lens implantation due to severe impairment of visual acuity. Two patients had severe renal failure with subsequent renal transplantation. Biopsy of one of them confirmed the diagnosis of Alport syndrome
Conclusion: despite the rarity of ocular involvement in Alport syndrome, especially in females, all three patients had anterior lenticonus and retinal flecks. Regardless of macular involvement, the main cause of decreased visual acuity was lenticular anomaly. Clear lens extraction with intraocular lens implantation was an effective treatment modality in these patients