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Answers: 2004 series -  July 27, 2004 Lecture 23 of 50  NEXT»

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This child is one year old and has been noted by her parents to have eyes crossed since shortly after birth. 


 1. 

The alignment in primary position and right and left gaze are typical for: 

a
-- This child appears to have BOTH eyes crossed and there is limited abduction.  This is typical of the congenital esotropia pattern called Ciancia's syndrome.  This syndrome described by Ciancia in the 1960's includes the following characteristics:

  1.

early onset esotropia 

  2.

large angle deviation 

  3.

fixation in adduction 

  4.

torticollis

  5.

head turn

  6.

dissociated development of vision 

  7.

delayed development of vision 

  8.

no significant hypertropia 

  9.

low family incidence of strabismus 

 10.

characteristic electro-oculogram findings, with nystagmus increasing as the eye moves toward abduction, the fast phase toward and damping with the eye in adduction.  

 11.

asymmetry of visually evoked potential and optokinetic nystagmus

 

 

 

 

 

 

 

 

 

 2.

Additional testing should include:

-- As in all cases of strabismus, the examiner should do all the tests needed and appropriate for a given patient in order to make a proper diagnosis.  In this child's case these tests would include a cycloplegic refraction to determine primarily if hyperopia could be a factor in the esodeviation.  Although this is unlikely in the case shown, the test should be done as well as a careful examination of the ocular media and the retina through the dilated pupil.  Vision should also be assessed.  In a one year old it can be determined that the child can fix intently on an interesting object and does NOT object to occlusion of either eye.  The crucial test to differentiate this patient from a bilateral sixth nerve palsy such as could occur with Moebius syndrome is the doll's head rotation which will demonstrate ABDUCTION due to vestibular response.  Even when a child prefers fixation in adduction as they do with Ciancia's syndrome, intact abduction and therefore sixth nerve function can be demonstrated by noting abduction of an eye while the head is rotated in the direction opposite abduction of each eye.

 

 3. 

Alignment of the eyes in this case would be accomplished most likely by:

a -- Since the most likely diagnosis in this case is congenital esotropia (Ciancia's syndrome), surgery would be the choice for alignment of the eyes.  The exact type and timing of surgery depends on the surgeon.  A "main stream" approach would be a large bimedial rectus recession done now, at age one year.  Some surgeons will do surgery on cases like this at four months of age provided there are not health or other issues indicating the need for delay.     


Lecture 23 of 50 «Previous Lecture   1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50    Next»