Anomalous extraocular muscles in Crouzon syndrome with V-pattern exotropia

Abstract

Strabismus associated with Crouzon syndrome is common and often complex. V-pattern strabismus is most commonly reported in this condition and is mainly thought to be due to an excyclorotation of the orbits and rectus muscle pulleys. We report two cases of children with Crouzon syndrome and V-pattern exotropia who had rectus muscle heterotopy on orbital imaging and were also found intraoperatively to have anomalous extraocular muscles. At the time of surgery, bifid insertion, misdirection, and fibrosis of extraocular muscles were noted. This highlights the various causes of V-pattern strabismus associated with Crouzon syndrome, including dysmorphic orbits and extraocular muscle anomalies.