[The various stages of vitelliform macular degeneration]

Hereditary autosomal dominant vitelliform macular degeneration and Best's macular degeneration are synonyma. The different namens for this disease rise from its varying clinical manifestation. The age at which it becomes manifest also varies. Our findings in two families with this hereditary foveal disease confirm that the macular changes are polymorphous. Nine different characteristic states of vitelliform macular degeneration can be distinguished. The vitelliform stage may represent a previtelliform or a resorptive stage. The differential diagnosis between vitelliform disc and vitelliform cyst can only be made unequivocally by fluorescein angiography. If the vitelliform cyst is scarred directly the scar is sharply delineated. A vitelliform stage or a pseudohypopyon stage may develop, especially form larger vitelliform cysts. In such cases the final stage may be a poorly delineated foveal scar. Occassionally, a mixed picture of two stages is observed, e.g., of cystic and scarred states. Exact knowledge of all stages of the disease is of great interest with resepct to differential diagnosis.