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Clinicopathologic Reports, Case Reports, and Small Case Series
October 2004

Bilateral Optic Neuropathy Associated With Voluntary Globe Luxationand Floppy Eyelid Syndrome

Author Affiliations
 

W. RICHARDGREENMD

Arch Ophthalmol. 2004;122(10):1555-1556. doi:10.1001/archopht.122.10.1555
Report of a Case

An obese 35-year-old man was examined because of loss of vision in hisright eye and gradually decreasing vision in his left eye. He also complainedof redness, irritation, and a foreign-body sensation bilaterally. His medicalhistory and his vision had previously been excellent, according to his medicalrecords at the company at which he was employed as a computer engineer. However,during the past 4 years he had had an obsessive-compulsive disorder, whichwas treated with risperidone. His parents reported that he had a peculiarhabit of luxating his eyes several times a day. When he was asked to demonstratethis, he easily everted his upper eyelids (Figure 1) and luxated his globes with his finger (Figure 2). He then pushed the globes back to their normal position.

Figure 1.
Voluntary eversion of the floppyupper eyelid.

Voluntary eversion of the floppyupper eyelid.

Figure 2.
A, Luxation of the globe withthe patient's finger. B, Lateral view of the luxated globe. C, Luxated globeas seen from above the patient.

A, Luxation of the globe withthe patient's finger. B, Lateral view of the luxated globe. C, Luxated globeas seen from above the patient.

On examination, the patient had blepharitis and corneal haze in botheyes (Figure 3). Ophthalmic examinationshowed no light perception in the right eye and a visual acuity of 20/200in the left eye. Slitlamp examination showed palpebral conjunctival hyperemiawith papillary hypertrophy1 and tears inthe Descemet membrane2,3 inboth eyes (Figure 4). Funduscopicexamination showed bilateral optic atrophy, more marked in the right eye.The optic disc was uniformly white without cupping in both eyes. The remainderof the results of fundus examination and the intraocular pressure were normal.The patient's poor vision did not permit visual fields to be obtained. Ultrasoundscan, Hertel measurements, and thyroid function test results showed no abnormalities.Electrophysiologic testing showed substantial reduction of the latent timeof the visual evoked responses. Results of further neurologic examination,including magnetic resonance imaging, for demyelinating disease, drug toxiceffects, and thyroid eye disease were negative.

Figure 3.
Blepharitis and corneal haze inboth eyes.

Blepharitis and corneal haze inboth eyes.

Figure 4.
Tears in the Descemet membraneand palpebral conjunctival hyperemia with papillary hypertrophy.

Tears in the Descemet membraneand palpebral conjunctival hyperemia with papillary hypertrophy.

The patient was instructed to avoid luxating his globes, as he refusedsurgical treatment of the floppy eyelids.4 Stabilizationof his psychiatric disease allowed the patient to be cooperative. Unfortunately,4 months later he was totally blind. His parents reported that he had neverstopped luxating his globes.

Comment

We suspect that the blindness of the patient described in this casewas due to a unique traumatic optic neuropathy. The easily everted floppyeyelids of this obese man enabled the voluntary luxation of the globes byinserting the finger deeply into the orbit. The patient's psychiatric disorderaggravated the condition. Concomitant tears in the Descemet membrane supportthe traumatic origin of the neuropathy.

In the literature we have found reported cases of mental retardationand floppy eyelid syndrome5 and one casereport of spontaneous globe luxation associated with floppy eyelid syndrome.6 There are also reported cases of self-inflictedocular injuries related to psychiatric problems.7,8 Toour knowledge, this is the first reported case of voluntary globe luxationand bilateral optic neuropathy associated with floppy eyelid syndrome.

Correspondence: Dr Apostolopoulos, 25 Vasilissis Sofias Ave, 74 Athens,Greece (damanakis@ath.forthnet.gr).

The authors have no relevant financial interest in this article.

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