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Monocular Elevation Deficiency/ Double Elevator Palsy

Fig. 1 Patient with left-sided monocular elevation deficiency/double elevator palsy attempting to look upward.

What is monocular elevation deficiency (double elevato palsy)?

Monocular Elevation Deficiency, also known by the older term Double Elevator Palsy, is an inability to elevate one eye, usually resulting in one eye that is pointed downward relative to the other eye (hypotropia) [See figure 1].

Is Monocular Elevation Deficiency hereditary?

This entity is not known to be hereditary (inherited). It is a rare condition, and can be congenital (present at birth) or acquired after birth.

Is Monocular Elevation Deficiency associated with ptosis (droopy eyelid)?

Yes. The eyelid on the involved side is droopy (ptosis) 25% of the time 75% of cases have pseudoptosis (only the appearance caused by the eye being hypotropic (downward deviation).

What is the cause of Monocular Elevation Deficiency?

The inability to elevate the eye most commonly results from restriction of the inferior rectus muscle on that side (70%). The cause is not precisely known for those cases without restriction of the inferior rectus. A hypothesized cause is an abnormal cranial nerve development with secondary muscle fibrosis (scarring).

Is Monocular Elevation Deficiency associated with jaw winking?

25% of those with Monocular Elevation Deficiency and Congenital Ptosis have a phenomenon called Marcus Gunn jaw-winking. This a condition in which the cranial nerve that usually controls eyelid movement is mis-wired with the cranial nerve that controls chewing or sucking thus creating a "wink" when chewing or sucking.

Is Monocular Elevation Deficiency associated with other diseases or developmental problems?

There is no known association between Monocular Elevation Deficiency and systemic or neurological diseases. Other disorders can occur in conjunction with Monocular Elevation Deficiency, but they are not directly related.

What are the treatment options for Monocular Elevation Deficiency?

The treatment of Monocular Elevation Deficiency is surgery, which is indicated if there is a significant hypotropia , amblyopia and/or a chin-up head posture. The type of surgery depends on the cause of the elevation deficit. Surgery can involve weakening the tightened inferior rectus muscle (if restricted) or transposing the medial and lateral rectus muscles to the location of the superior rectus (if palsied). Rarely, surgery on a muscle in the opposite eye is necessary to fully correct the deficit.

Do exercises or Vision Therapy help to fix Monocular Elevation Deficiency?

No.

Should eye patching be performed for Monocular Elevation Deficiency?

Patching is sometimes necessary to treat amblyopia (weak vision) that can result from misalignment of the eyes. Patching will not realign the eyes, but helps to strengthen the vision on the unpatched side.