6.9 Monocular Elevation Deficiency
(“Double Elevator Palsy”)

Definition

  • Hallmark: Limitation of elevation above midline, in adduction and abduction

Aetiology

  • Congenital Cranial Dysinnervation Disorder (CCDD)
  1. Paresis of superior rectus (SR) & inferior oblique (IO) (“Double elevator palsy”) or
  2. Inferior rectus (IR) restriction

IR restriction

Elevator weakness

Combination

Forced Duction Test

IR restriction

Positive

Elevator weakness

Negative

Combination

Positive

Forced Generation Test

IR restriction

Positive

Elevator weakness

Negative

Combination

Positive

Saccades

IR restriction

Normal

Elevator weakness

Decreased elevation velocity

Combination

Decreased elevation velocity

Treatment

IR restriction

IR recession ± ptosis repair

Elevator weakness

Knapp procedure ± ptosis repair

Combination

Knapp + IR recession ± ptosis repair (staged for anterior segment ischaemia)

Examination Structure

1. Visual Acuity (Best Corrected)

  • Amblyopia?

2. Spectacles

3. Inspection

  1. Abnormal head position - chin up
  2. Hirschberg (Pupil margin = 15°, Iris margin = 30°) - hypotropia
  3. Lids- can get ptosis or pseudoptosis (secondary to hypotropia, disappears when fixing with the affected eye)
  4. (Pupils normal)
Figure 6.9.1 Left Monocular Elevation Deficit

Figure 6.9.1
Left Monocular Elevation Deficit

4. Cover-Testing

  • Incomitant hypotropia (increases on attempted upgaze)

5. Ocular Rotations

  • Limitation of elevation that is the same in adduction and abduction
  • No difference between versions and ductions

6. Marcus-Gunn Jaw-Wink?

  • Monocular elevation palsies may be associated with a Marcus-Gunn jaw-wink

7. Forced Duction Testing

  • Normal: If paresis of SR and SO
  • Abnormal: If IR restriction

Differential Diagnosis

  1. SR or IO palsy (isolated)
  2. Brown Syndrome
  3. Restriction (TED, Orbital wall fracture, Myositis)

Management

          

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