Congenital fibrosis of the extraocular muscles

The syndrome of congenital fibrosis of the extraocular muscles is characterized by replacement of normal contractile muscle by fibrous tissue, in varying degrees, which causes severe limitation in the action of the muscles. Depending on the muscles affected, five different clinical pictures have been described. Two patients are reported, in one of whom there was a problem of differential diagnosis, because the clinical findings included unilateral ptosis, paresis of supraversion and pseudopapilledema, in addition to a pattern of exotropia. The other patients had pronounced esotropia with limitation of eye movement in the horizontal plane.