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Symptoms ⬇

Difference in size of pupils, blurred near vision, and photophobia. May be asymptomatic.

Signs ⬆ ⬇

Critical

An irregularly dilated pupil that has minimal or no reactivity to light. Slow, tonic constriction with convergence, and slow redilation. May have vermiform iris movement and/or sectoral iris sphincter paresis.

NOTE:

Typically presents unilaterally and more commonly in young women.

Other

May have an acute onset and become bilateral. The involved pupil may become smaller than the normal pupil over time.

Differential Diagnosis ⬆ ⬇

See 10.1, ANISOCORIA.

  • Parinaud syndrome/dorsal midbrain lesion: Bilateral mid-dilated pupils that react poorly to light but constrict normally with convergence (not tonic). Associated with eyelid retraction (Collier sign), supranuclear upgaze paralysis, and convergence retraction nystagmus. An MRI should be performed to rule out pinealoma and other midbrain pathology.
  • Holmes–Adie syndrome: Tonic pupil and tendon areflexia. May be associated with autonomic and peripheral neuropathy.
  • Argyll Robertson pupils: See 10.3, ARGYLL ROBERTSON PUPILS.

Etiology ⬆ ⬇

Idiopathic most commonly. Orbital trauma, surgery, and varicella zoster virus infection are seen frequently. Early syphilis, parvovirus B19, herpes simplex virus, botulism, paraneoplastic syndrome, giant cell arteritis (GCA), panretinal photocoagulation, and neurologic Lyme disease less commonly. Rare associations reported with endometriosis, seminomas, and Sjögren syndrome.

Work Up ⬆ ⬇

Workup

See 10.1, ANISOCORIA, for a general workup when the diagnosis is uncertain.

  1. Evaluate pupils and iris at slit lamp or with a muscle light for irregular slow constriction or abnormal movement.
  2. Test for cholinergic hypersensitivity. Instill 0.125% pilocarpine in both eyes and recheck pupils in 10 to 15 minutes. An Adie pupil constricts while the normal pupil does not.
  3. If bilateral simultaneous Adie pupils, consider further laboratory investigations including testing for the aforementioned etiologies. For unilateral involvement, no further laboratory investigations are necessary.
  4. If Adie pupil is present in a patient younger than 1 year, consult a pediatric neurologist to rule out familial dysautonomia (Riley–Day syndrome).
NOTE:

The dilute pilocarpine test may occasionally be positive in familial dysautonomia. Hypersensitivity may not be present with an acute Adie pupil and may need to be retested a few weeks later.

Treatment ⬆ ⬇

Pilocarpine 0.125% b.i.d. to q.i.d. may be considered for cosmesis and to aid in accommodation.

Follow Up ⬆

If the diagnosis is certain, follow up is routine.