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Bilateral Optic Neuropathy Associated With Voluntary Globe Luxation and Floppy Eyelid Syndrome
Arch Ophthalmol. 2004;122:1555-1556.
Report of a Case
An obese 35-year-old man was examined because of loss of vision in his right eye and gradually decreasing vision in his left eye. He also complained of redness, irritation, and a foreign-body sensation bilaterally. His medical history and his vision had previously been excellent, according to his medical records 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, which was treated with risperidone. His parents reported that he had a peculiar habit of luxating his eyes several times a day. When he was asked to demonstrate this, 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.
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Figure 1. Voluntary eversion of the floppy upper eyelid.
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Figure 2. A, Luxation of the globe with the patient's finger. B, Lateral view of the luxated globe. C, Luxated globe as seen from above the patient.
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On examination, the patient had blepharitis and corneal haze in both eyes (Figure 3). Ophthalmic examination showed no light perception in the right eye and a visual acuity of 20/200 in the left eye. Slitlamp examination showed palpebral conjunctival hyperemia with papillary hypertrophy1 and tears in the Descemet membrane2-3 in both eyes (Figure 4). Funduscopic examination showed bilateral optic atrophy, more marked in the right eye. The optic disc was uniformly white without cupping in both eyes. The remainder of the results of fundus examination and the intraocular pressure were normal. The patient's poor vision did not permit visual fields to be obtained. Ultrasound scan, Hertel measurements, and thyroid function test results showed no abnormalities. Electrophysiologic testing showed substantial reduction of the latent time of the visual evoked responses. Results of further neurologic examination, including magnetic resonance imaging, for demyelinating disease, drug toxic effects, and thyroid eye disease were negative.
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Figure 3. Blepharitis and corneal haze in both eyes.
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Figure 4. Tears in the Descemet membrane and palpebral conjunctival hyperemia with papillary hypertrophy.
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The patient was instructed to avoid luxating his globes, as he refused surgical treatment of the floppy eyelids.4 Stabilization of his psychiatric disease allowed the patient to be cooperative. Unfortunately, 4 months later he was totally blind. His parents reported that he had never stopped luxating his globes.
Comment
We suspect that the blindness of the patient described in this case was due to a unique traumatic optic neuropathy. The easily everted floppy eyelids of this obese man enabled the voluntary luxation of the globes by inserting the finger deeply into the orbit. The patient's psychiatric disorder aggravated the condition. Concomitant tears in the Descemet membrane support the traumatic origin of the neuropathy.
In the literature we have found reported cases of mental retardation and floppy eyelid syndrome5 and one case report of spontaneous globe luxation associated with floppy eyelid syndrome.6 There are also reported cases of self-inflicted ocular injuries related to psychiatric problems.7-8 To our knowledge, this is the first reported case of voluntary globe luxation and bilateral optic neuropathy associated with floppy eyelid syndrome.
The authors have no relevant financial interest in this article.
AUTHOR INFORMATION
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Michael Apostolopoulos, MD;
Alexis Papaspirou, MD;
Alexandros Damanakis, MD;
George Theodossiadis, MD;
Michael Moschos, MD
Correspondence: Dr Apostolopoulos, 25 Vasilissis Sofias Ave, 74 Athens, Greece (damanakis{at}ath.forthnet.gr).
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5. Boulton JE, Sullivan TJ. Floppy eyelid syndrome and mental retardation. Ophthalmology. 2000;107:1989-1991.
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6. Alexandrakis G, Tse DT, Chang WJ. Spontaneous globe luxation associated with floppy eyelid syndrome and shallow orbits. Arch Ophthalmol. 1999;117:138-139.
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7. Koh KG, Lyes BK. Self-enucleation in a young schizophrenic patienta case report. Singapore Med J. 2002;43:159-160.
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8. Detry-Morel M, Philippart R, Boschi A, Luts A. Self-inflicted repetitive optic nerve injury: a case report. Eur J Ophthalmol. 2002;12:440-442.
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ABSTRACT
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