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Case of the week 176  ( June 2014 )
Double elevator palsy



12 years old girl complaining of dropped Rt eye lid. The condition started since early childhood. Ocular examination reveals no abnormalities except motility disorders seen here.



 See video of this patient
 


Double elevator palsy
( Monocular elevation deficiency )

Palsy of both elevator muscles ( SR & IO ) is a rare anomaly of ocular motility. On Fixation with the sound eye, the paretic eye will take a hypotropic position and the upper lid will be slightly ptotic. On Fixation with the paretic eye, the ptosis may disappear, here the name pseudoptosis, where usually, no levator surgery is needed . There is limitation of elevation of the paretic eye from any position of gaze, in Brown syndrome the limitation of elevation is seen only during adduction. Bell’s phenomenon is usually preserved. The ductions of the paretic eye are normal in all other positions of gaze. The chin is usually elevated. This anomaly is often congenital.

The differential diagnosis of a double elevator palsy are :

 1. blow-out fracture of the orbital floor.

2. Congenital fibrosis of the EOM.

3. Endocrine myopathy.

4. Anomalous insertion of the inferior rectus muscle.

5. Brown Syndrome

A positive forced duction test and increased intraocular pressure in upward gaze may distinguish these conditions from double elevator palsy.

Surgery is indicated only when there is hypotropia in primary position or chin elevation or both. The aim of surgery is to restore single binocular vision in primary position.

Knapp introduced vertical transposition of the horizontal rectus muscles to the medial and lateral edge of the superior rectus muscle with or without recession of the IR.




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