- 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.