RARE CASE OF ISOLATED ANTERIOR LENTICONUS

Abstract

Lenticonus is a bulging of the lens capsule and the underlying cortex. The diagnosis of lenticonus is essentially a clinical diagnosis which is made by biomicroscopic examination. According to the localization of the conus a distinction is made between lenticonus anterior and lenticonus posterior. Sometimes isolated, it may be an ocular manifestation of Alport syndrome, a hereditary nephritis accompanied by deafness and other ocular signs such as ecked retinopathy or posterior polymorphous corneal dystrophy. All these manifestations are a result of a genetic defect in type IV collagen, a major component of basal membranes in the human body. The clinical aspects and therapy of anterior lenticonus and Alport syndrome are described, as well as the exploration that must begin when anterior lenticonus is discovered. Preconical lenses are less commonly reported and are ［ ］ more common in Alport syndrome (AS) If a patient with a conical 2 lens in front of eye is observed clinically, the possibility of being an AS patient should be ruled out rst. AS,typically manifested as renal injury (hematuria, proteinuria and progressive renal insufciency), neurosensorineural deafness and eye injury triad, more common in women, but male patients are more seriously ill, and most of them have renal failure before the age of 40. 11%~92% of AS patients have eye injury, of which anterior conus lens and spotted retinopathy are characteristic changes, mostly involving both eyes, usually only when renal failure occurs. Reference to the Flinter standard or the new Gregory standard ,For the family history and eye, ear, and kidneyrelated examinations of patients with suspected AS, clinical diagnosis can usually be established, and combined with pathological examination and collagen molecular immunopathological examination, clinicopathological diagnosis can be established in most cases .