6.7 Duane Syndrome

Definition

  • Hallmark: Retraction of the globe on attempted adduction (co-contraction of lateral and medial rectus), resulting in narrowing of palpebral fissure.

Aetiology

Congenital cranial dysinnervation disorder.

  1. CNVI nucleus absent
  2. CNIII nucleus innervates LR (inappropriately) and MR (appropriately)

95% are sporadic (rarely autosomal dominant).

(Huber) Classification

Type I (>50%)

Limited AbDuction ± Esotropia in primary (mostly straight)

Type II

Limited ADDuction ± Exotropia in primary

Type III

Limited AbDuction and ADDuction ± Esotropia in primary

NB: Count the number of D’s to determine the type!
Can also be classified: esotropic, exotropic, or orthotropic Duane Syndrome

Examination Structure

1. Visual Acuity (Best Corrected)

  • Amblyopia uncommon

2. Spectacles

3. Inspection

  1. Abnormal head position- head turn / tilt to the same side (in Type 1)
  2. Hirschberg (Pupil margin = 15°, Iris margin = 30°) - ± small esotropia (in Type 1)
  3. (Lids often normal in primary, close on attempted adduction)
  4. (Pupils normal)
  5. Inspect for systemic associations (ears, face, neck etc. - see below)
Figure 6.7.1a Left Duane Syndrome (Type III)

Figure 6.7.1
Left Duane Syndrome (Type III)
A: Limited adduction of the left eye. Not narrowing of the left palpebral fissure.

Figure 6.7.1b Left Duane Syndrome (Type III)

Figure 6.7.1
Left Duane Syndrome (Type III)
B: Eyes straight in primary.

Figure 6.7.1c Left Duane Syndrome (Type III)

Figure 6.7.1
Left Duane Syndrome (Type III)
C: Limited abduction of the left eye. No narrowing of the palpebral fissure.

4. Cover-Testing

  • Often straight in primary despite marked abduction deficit (in Type 1)

5. Ocular Rotations

  • ~ 85% Unilateral
  • Hallmark: Retraction of the globe on attempted adduction (co-contraction of lateral and medial recti), resulting in narrowing of palpebral fissure
  • ± Upshoot / Downshoot in adduction

6. Systemic Associations? (~30% of Patients)

Duane syndrome can be associated with:

Goldenhar Syndrome
“LUMPED”

  • L ipodermoid
  • U pper lid colobomas
  • M icrophthalmia, Microsomia (hemifacial)
  • P reauricular skin tags
  • E pibulbar dermoid / Ear anomalies
  • D uane syndrome

          

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