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Case of the week 74  ( September 2012 )
Congenital Third Nerve Palsy


 
The mother of this 8 months old infant noted that her baby has a dropped Rt eye lid and outward deviation of the eye. The condition started at birth. There is no history of birth trauma, the baby was full term with appropriate weight. Fundus examination shows no abnormalities.


When the oculomotor nerve is completely paralyzed, the position of the affected eye is determined by the function of the only two remainin intact muscles, the lateral rectus and the superior oblique muscles. Thus the paralyzed eye will be in a position of abduction, slight depression, and intorsion. Concurrent paralysis of the levator palpebrae will cause ptosis of the upper lid, and general relaxation of tonus of four of the six extraocular muscles may produce a small degree of proptosis. The motility of the affected eye will be limited to abduction, to small degrees of depression in abduction and to an adduction movement of the eye that does not go beyond the primary position. With a complete cranial nerve III paralysis, the intrinsic muscles of the eye also are involved, causing the pupil to be dilated and nonreactive and a paralysis of accommodation.

Congenital oculomotor nerve palsies are rare and are often associated with neurological abnormalities. Aberrant regeneration is common,which, if present, argues against a nuclear lesion. Many of these can be associated with perinatal trauma. A neurologic evaluation is therefore advisable and an evaluation by neuroimaging to search for associated structural anomalies of the brain has been recommended

The surgical management of a complete cranial nerve III paralysis presents a formidable challenge and the therapeutic possibilities are limited. As may be expected, the sensorimotor outcome of treatment in children is poor. At the very best, the surgeon will succeed in moving the paretic eye into the primary position without restoring adduction, elevation, or depression to a significant degree.

A maximal recession-resection of the horizontal rectus muscles will, at best, create only temporary improvement of the eye position. Eventually, the eye will drift back into an abducted position and a more radical approach will be called for. After trying several procedures, including maximal horizontal surgery with upward transposition of the muscle tendons or transposition of the superior oblique tendon to the insertion of the medial rectus muscle, the following operation has given the best results:
tenotomy of the lateral rectus and superior oblique muscles combined with a transposition of the vertical rectus muscles to the insertion of the medial rectus muscle.
Even though the treated eye will continue to be immobile, it will at least be nearly centered.
If this happened, a guarded frontalis sling is performed to just a position where the pupillary axis will be exposed.

Before After


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The Egyptian Site of Strabismus & Oculomotor Disorders