Anterior conical protrusion of the central lens surface caused by the thinning of affected anterior capsule.
Deficiency of the subjacent epithelium resulting in irregular astigmatism.
The lens defect may be an isolated finding or more commonly associated with Alport's syndrome.
Alport's syndrome is an autosomal recessive disorder with chronic progressive hemorrhagic nephropathy, bilateral high-tone perceptive deafness and ocular findings.
Other ocular findings that have been described in Alport's syndrome are spherophakia, anterior polar and posterior cortical cataract, fundus lesions such as drusen, retinal flecks and retinal neovascularization.
Spontaneous rupture of the capsule has also been reported.