Ultrastructural fragility and type IV collagen abnormality of the anterior lens capsules in a patient with Alport syndrome.

PURPOSE: To investigate ultrastructural and immunohistological abnormalities of the lens capsules in a patient with Alport syndrome.

METHODS

Two anterior lens capsules were obtained at phacoemulsification from a 43-year-old female patient with bilateral lenticonus who was affected by Alport syndrome. The right capsule was examined by transmission electron microscopy and the left capsule was stained with two monoclonal antibodies against the triple-helical domains of type IV collagen alpha2 and alpha5 chains.

RESULTS

Numerous vertical dehiscences with many disrupted interdigitations were observed in the right anterior lens capsule. Decreased reactivity of monoclonal antibodies against type IV collagen alpha5 chain and normal reactivity against the alpha2 chain were shown in the left anterior lens capsule.

CONCLUSION

The ultrastructural fragility of the anterior lens capsules in this patient with Alport syndrome appears to be associated with the abnormality of the type IV collagen molecules including the alpha5 chain.