Alport syndrome is a hereditary, progressive disease characterized by progressive nephritis, sensorineural deafness, and ocular abnormalities, including anterior lenticonus. The ultrastructure of the lens capsule abnormalities in Alport syndrome is reported. Four anterior lens capsules from 31-year-old patient and 26-year-old patient with lenticonus who were affected by the Alport syndrome were obtained at capsulectomy. And all four anterior lens capsules were examined by transmission electron microscopy. The histopathologic findings showed that the thickness of the anterior lens capsules was decreased (4~13 micrometer) and that there were many vascular dehiscences localized at the inner part of the lens capsule. There were large numbers of capsular dehiscences containing fibrillar materials and vacuoles. The anterior capsules were clearly fragile in this disease, forming the basis for the progressive lenticonus and anterior polar cataract.
Adult ; Epithelial Cells/ultrastructure ; Humans ; Lens Capsule, Crystalline/*ultrastructure ; Lens Diseases/genetics/*pathology ; Lens Implantation, Intraocular ; Male ; Nephritis, Hereditary/genetics/*pathology ; Phacoemulsification

A 45-year-old man with retinitis pigmentosa (RP), who had undergone uneventful extracapsular cataract extraction (ECCE) in his right eye eight years previously, and phacoemulsification in his left eye six years previously, had spontaneously dislocated intraocular lenses (IOL) within the capsular bag in both eyes one month apart. We removed the dislocated IOLs, and performed anterior vitrectomy and scleral fixation of the new IOLs. Mild contraction of the capsular bags and uneven distribution of the zonular remnants' clumps along the equator of the capsules were found by scanning electron microscopic (SEM) examination. In this study, we propose the correlation between RP and zonular weakness. To our knowledge, this is the first case report of bilateral spontaneous dislocation of IOLs within the capsular bag of an RP patient.
Adult ; Foreign-Body Migration/*etiology ; Humans ; Lens Capsule, Crystalline/*pathology ; Lens Implantation, Intraocular ; *Lenses, Intraocular ; Ligaments/ultrastructure ; Male ; Microscopy, Electron, Scanning ; Phacoemulsification ; Reoperation ; Retinitis Pigmentosa/*complications ; Vitrectomy