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Case of the week 68  ( August 2012 )
Congenital 6th nerve palsy



The mother of this 10 months old infant noted that he had this abnormal looking behaviour since birth , he had a history of normal labour, he is a fine infant with no obvious neurological or any other systemic diseases.
Because he is very cooperative, fundus examination and cyclorefraction could be easily done in the office with no detected abnormalities  .
 


 See video of this patient
 

 

The most striking features in this infant are the left face turn and the limitation of abduction . The clinician should be aware of other causes of limitation of abduction in this age group , these are Duane Syndrome type I and III and Mobius syndrome (Moebius syndrome is a bilateral congenital paralysis of the abducens and facial nerves resulting in esotropia and an expressionless facial appearance. There is also atrophy of the distal third of the tongue with a history of poor feeding) , he should also be aware about pseudo abducens palsy occuring in many infants with congenital esotropia.  Infantile esotropia and nystagmus blockage by convergence are often accompanied by apparent limitation of abduction. The doll's head maneuver or patching of one eye distinguishes pseudoabducens paralysis from a true paralysis in these cases.

Congenital 6th nerve palsy as an isolated phenomenon is exceedingly rare but may occur from injury to the abducens nerve shortly before or during birth.

Congenital 6th nerve palsy usually occurs with other neurologic or systemic anomalies. Patients with cerebral palsy seem to have an increased prevalence of congenital 6th nerve palsy .

Surgical intervention for congenital sixth nerve palsy is indicated when the deviation has been stable for a minimum of 10-12 months. The choice of surgical procedure for sixth nerve palsies depends upon the recovery of function of the lateral rectus muscle, which can be assessed by determining the saccadic velocity. Patients who have good return of function usually do quite well with an ipsilateral recess-resect procedure. Patients who have little or no lateral rectus function need muscle transposition surgery.



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