From the Department of Ophthalmology, Sunderland Eye Infirmary, Sunderland, United Kingdom.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Lawrence Gnanaraj, FRCS (Ophth), FRCSEd, Department of Ophthalmology, Sunderland Eye Infirmary, Sunderland, SR2 9HP, U K. E-mail: Lawrenceg@doctors.org.uk
Tonic pupil results from damage to the ciliary ganglion, short ciliary nerves, or both.1 This post-ganglionic parasympathetic denervation manifests clinically as poor pupillary reaction to light with sectoral palsy of iris sphincter, tonic response to near stimuli followed by slow redilatation, accommodation paresis, and cholinergic supersensitivity of the iris sphincter to weak diluted pilocarpine.
The average age of onset is 32 years with a female preponderance. Most cases are unilateral, but they can also become bilateral.2 Bilateral tonic pupil in children is uncommon.3 We report a case of a 10-year-old girl with a history of headaches who was noted to have abnormal pupils by her optometrist and subsequently diagnosed as having idiopathic bilateral tonic pupils and migraine.
A 10-year-old girl attended her optician complaining of headaches. She was otherwise fit and well. Her corrected visual acuity was 20/25 in both eyes. Her refractive errors were +1.25/−2.25@180 in the right eye and +0.75/−1.75@180 in the left eye. She was found to have anisocoria and was thus referred to the pediatric eye clinic.
Examination revealed bilateral asymmetrical enlarged pupils (Fig. 1A) measuring 6 mm in the right eye and 4 mm in the left eye. Her pupils reacted poorly to light and showed tonic response to near stimuli in both eyes. Slit-lamp examination showed vermiform sphincter movements in both eyes. Her anterior and posterior segment examinations were unremarkable. Her corneal sensation was intact.
Figure 1. External Clinical Photograph Showing Bilateral Adie’s Pupils. The Photograph’s Flash Power Was Standardized in Both Occasions. (A) Asymmetrical Dilated Pupils While Looking for Distance. (B) Bilateral Constricted Pupils Due to Supersensitivity to the Weak Pilocarpine Drops.
The diagnosis of bilateral Adie’s pupil was confirmed by demonstrating constriction of both dilated pupils to one drop of weak guttae pilocarpine 0.125% (Chauvin Pharmaceuticals, Ltd., Essex, UK) (Fig. 1B). She was referred to a pediatrician and results of detailed neurological examination and investigations were all normal so the diagnosis of migraine was made.
Adie’s pupil is characterized by an enlarged pupil with poor response to light and tonic response with slow redilatation to near stimulation. The pathophysiologies described are local orbital causes involving the ciliary ganglion, including inflammation, diffuse peripheral or autonomic neuropathy, and idiopathic tonic pupil.1 Tonic pupil begins unilaterally in 69% of cases and later becomes bilateral in at least 20% of the patients.2 They may also show regional corneal hypesthesia due to interruption of fibers of the ophthalmic division of the trigeminal nerve as they traverse the ciliary ganglion.2 In addition to ocular signs, the deep tendon reflexes, especially the knee and ankle reflexes, may be diminished or absent.1
Unilateral or bilateral Adie’s pupil is rare in childhood. There are two case reports of bilateral idiopathic tonic pupil in children in the literature.3,4 The first involves a 4-year-old boy who developed bilateral consecutive idiopathic Adie’s syndrome. He was first diagnosed at age 4 years with a right tonic pupil3 and amblyopia because the accommodative difficulty unmasked his latent hypermetropia. Six years later, there was tonic involvement of his left pupil and absent deep tendon reflexes.5 The second case reported is an 8-year-old girl who presented with complaints of blurred vision. She was found to have latent hypermetropia and prescribed glasses that did not alleviate her symptoms. On subsequent examination she was diagnosed as having bilateral Adie’s syndrome4 and her Adie’s pupils were found to be the cause of her visual problem.
Cases of unilateral Aide’s pupil associated with migraine have previously been described.6–8 The youngest case was an 18 year old who presented with transient unilateral tonic pupil and migraine.6 This association is thought to result from vasoconstriction related to migraine causing transient ischemia to the ciliary ganglion and hence post-ganglionic parasympathetic dysfunction.7 To our knowledge, this is the first case to be reported with bilateral Adie’s pupil secondary to migraine in a child.
Tonic pupils can also be part of diffuse peripheral or autonomic neuropathy; these include syphilis, diabetes mellitus, Guillian–Barre syndrome, and other hereditary neuropathy. It has also been described in a child with neuroblastoma and attributed to a paraneoplastic process.9
We hereby present a unique case of a girl who was found to have anisocoria and migraine. Although uncommon, it is important to recognize this clinical condition in relation to migraine because the clinical diagnosis can be easily confirmed by assessing the response to diluted pilocarpine drops that reflects the parasympathetic supersensitivity.
- Loewenfield IE, Thompson HS. The tonic pupil: a re-evaluation. Am J Ophthalmol. 1967;63:46–87.
- Thompson HS. Adie’s syndrome: some new observations. Trans Am Ophthalmol Soc. 1977;75:587–626.
- Dutton GN, Paul R. Adie syndrome in a child: case report. J Pediatr Ophthalmol Strabismus. 1992;29:126.
- Gotz-Wieckowska A, Kociecki J. Bilateral Adie’s syndrome in child [article in Polish]. Klin Oczna. 2006;108:450–451.
- Agbeja AM, Dutton GN. Adie’s syndrome as a cause of amblyopia. J Pediatr Ophthalmol Strabismus. 1987;24:176–177.
- Drouet A, De Carvalho A, Mage F, Guilloton L, Felten D. Adie’s tonic pupil and migraine: a chance association? [article in French]. J Fr Ophtalmol. 2008;31:e5.
- Massey EW. Pupillary dysautonomia and migraine: is Adie’s pupil caused by migraine?Headache. 1981;21:143–146. doi:10.1111/j.1526-4610.1981.hed2104143.x [CrossRef]
- Purvin VA. Adies tonic pupil secondary to migraine. J Neuro Ophthalmol. 1995;15:43–44.
- West CE, Repka MX. Tonic pupils associated with neuroblastoma. J Pediatr Ophthalmol Strabismus. 1992;29:382–383.