Acquired myelinated nerve fibers in association with optic disk drusen.

[kabuki syndrome]

Myelinated retinal nerve fibers are a well-recognized anomaly of the ocular fundus associated with many ocular and systemic conditions . Myelination is almost always congenital and stable , but progression has been documented in rare cases . Optic disk drusen are the result of a degenerative process at the optic nerve head and are often found incidentally on ophthalmologic examination . To our knowledge , optic disk drusen have only been reported once in association with acquired and progressive myelinated retinal nerve fibers . We present 2 such cases and consider the implications for the pathogenesis of myelinated nerve fibers .