Ciancia’s syndrome is a large-angle congenital esotropia with

cross-fixation, and both eyes appear to be “stuck” in toward the

nose. It consists of the following characteristics:

(1) large-angle esotropia

(2) bilateral limited abduction with intact abduction saccades

(3) fixing eye in adduction

(4) nystagmus on attempted abduction with no nystagmus in adduction

(5) face turn to the side of the fixing eye

In Ciancia’s syndrome, the abduction deficit is most likely secondary to tight medial rectus muscles. Clinical examination shows good lateral rectus function, evidenced by normal brisk abduction saccades

Forced duction at the time of surgery shows moderately tight medial rectus muscles. The abduction nystagmus is a jerk nystagmus with the fast phase in the direction of the fixing eye and only occurs when the fixing eye abducts. This nystagmus probably represents an exaggerated endpoint nystagmus, as the lateral rectus muscle pulls against the tight medial rectus muscles. Ciancia found that approximately one-third of his patients with congenital esotropia had this syndrome. It is likely that many of the patients described by Ciancia would have been classified in the American literature as large-angle congenital esotropia with cross-fixation. The reason for the face turn in these patients with a large-angle esotropia, and the fixing eye in adduction is probably not to damp the nystagmus, as the nystagmus is usually minimal if present at all; the face turn is adopted because the medial rectus is tight and holds the fixing eye in adduction

Surgically correcting the esotropia associated with Ciancia’s syndrome is difficult, as undercorrections are frequent. One of the problems is measuring the full deviation, as both eyes are stuck in adduction and it is difficult to get the fixing eye into primary position for a true measurement.

The surgery of choice is large medial rectus recessions, approximately 7mm posterior to the insertion site.