RESUMEN
Alport's syndrome with anterior lenticonus poses significant challenges during capsulorhexis with high chances of peripheral extension. We report a case of 23-year-old lady with Alport's syndrome with anterior lenticonus in the left eye that underwent successful femtosecond laser-assisted cataract surgery (FLACS). The anterior lenticonus was eccentric nasally; hence, the FLACS capsulotomy was positioned slightly nasally so that the capsulotomy margin was outside the cone base. The distance vision improved from 6/24 to 6/6 at one-month follow-up. In summary, FLACS improved the safety of cataract surgery in an eye with anterior lenticonus and yielded excellent visual outcomes.
RESUMEN
Objective@#To present a case of Alport syndrome, its pathogenesis, etiology, clinical manifestation, diagnosis, and management.@*Methods@#This is a case report.@*Results@#A 22-year-old male presented with blurring of vision associated with bilateral anterior and posterior lenticonus. The patient had a history of blurring of vision on both eyes, occasional right lower-quadrant pain on urination, frothy urine, and bilateral hearing loss. Family medical history showed one brother who died at 15 years of heart disease, and another brother at 17 from chronic kidney disease. Slitlamp examination showed a conspicuous oil droplet reflex seen through retroillumination. Anterior and posterior bulging of the lens was noted, highly suggestive of anterior and posterior lenticonus. On indirect ophthalmoscopy, no perimacular dot-and-fleck retinopathy was seen. Further systemic workup revealed elevated serum levels of blood urea nitrogen (BUN) and creatinine, and marked proteinuria and hematuria. Ultrasound of the kidneys revealed bilateral renal parenchymal disease. Pure tone audiometry confirmed bilateral moderate sensorineural hearing loss. @*Conclusions@#There should be a high index of suspicion for Alport syndrome in any patient presenting with anterior and posterior lenticonus. A thorough history-taking and physical examination, including slitlamp examination through a dilated pupil, are necessary to fully support its diagnosis. There is no specifically defined treatment for Alport’s syndrome; management should be individualized and approached in a multidisciplinary fashion. Lenticonus can be treated by phacoemulsification with careful capsulorrhexis.
Asunto(s)
Nefritis HereditariaRESUMEN
·AIM: To analyze the clinical manifestation of Alport syndrome, especially the ocular features.·METHODS: The physical, ophthalmologic and audiologic examination results of thirty two patients with Alport syndrome were analyzed retrospectively.·RESULTS: Thirty (93.7%) patients had some family history. All patients had renal disease: eighteen(56.3%) patients with chronic renal failure, four(12.5%) patients with renal insufficiency, and the other ten(31.3%) patients with hematuria. Twenty (62.5%) patients had sensorineural deafness. Thirteen (40.6%) patients had ocular deformity, five(15.6%) patients had typical ocular changes: three patients with anterior lenticonus, and two patients with macular flecks.·CONCLUSION: Ocular anomalies are not requisite for the diagnosis of Alport syndrome. But its typical ocular features should be recognized by the ophthalmologists which supports the diagnosis.
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· AIM: We describe visual improvement after phacoemulsification with intraocular lens (IOL) implantation in 7 eyes with anterior lenticonus secondary to Alport syndrome.· METHODS: 7 eyes from 4 patients with Alport syndrome underwent phacoemulsification with IOL implantation due to anterior lenticonus.· RESULTS: All of the patients resumed excellent visual acuity after surgery.· CONCLUSION: We recommend the phacoemulsification with IOL implantation as a safe procedure in anterior lenticonus due to Alport syndrome.
RESUMEN
Alport's syndrome, first described by Alport in 1927, is a clinical entity which consists of lens abnormalities, hereditary hemorrhagic nephritis and nerve deafness. Anterior lenticonus is a rare structual anomaly of lens in which there is a conicalor spherical protrusion of the anterior lens surface into the anterior chamber as a characteristic finding of Alport's syndrome. The authers experienced a case of Alport's syndrome in a 22-year-old man associated with bilateral anterior and posterior lenticonus, hematuria, proteinuria, renal failur earld nerve deafness(complete form).