Kunesh, John C. M.D.; Katz, Steven E. M.D.
Uncommonly, a patient will present to the physician’s office or emergency room with acute involuntary spontaneous luxation of the globe, which is often a first episode. Eyelid manipulation or trauma may be the inciting event. Corneal exposure leads to blepharospasm, setting up a cycle in which globe reduction may be difficult.
Luxation occurs when the equator of the globe is allowed to protrude anterior to the eyelid aperture. The orbicularis muscle contracts, voluntarily or involuntarily, causing further anterior displacement, and the globe is “caught” outside the eyelid aperture. Relaxation of the orbicularis muscle is necessary to return the globe to its natural position in the orbit. Some predisposed individuals learn to voluntarily induce globe luxation.
Globe luxation has generally been categorized as spontaneous, voluntary, or traumatic. The literature, however, has not been consistent in defining these categories. For our review, we define spontaneous luxation as occurring without conscious effort, with or without a precipitating factor. 1–20 Voluntary luxation is the ability to luxate one’s globe at will, without any precipitating trigger. Traumatic luxation occurs without conscious effort after trauma.
We report a patient who developed spontaneous globe luxation while attempting to place a contact lens in his left eye. The literature regarding spontaneous globe luxation is reviewed with respect to the epidemiology, clinical characteristics, risk factors, complications, and treatments. A step-wise approach to management is presented.
A 42-year-old black man was inserting a soft contact lens in his left eye when the globe “popped out of the socket.” In demonstration of his technique for lens insertion, he spread the eyelids with the ipsilateral thumb and index finger, and applied posterior pressure on the lid tissues against the superior and inferior orbital rims. He came to the emergency room with left globe luxation one-half hour later (Fig. 1). He complained of moderate discomfort and blurred vision. Visual acuity was not assessed initially; however, the pupils were brisk, and there was no relative afferent pupillary defect. Neither the patient nor the emergency room physician was able to reduce the eye manually. Short-acting sedation, consisting of midazolam (Versed) 4 mg and fentanyl 150 μg, was given. This allowed manual reduction of the globe, but it was one and one-half hours after the onset of luxation.
Initially, the patient was sedated and minimally responsive. There was no history of thyroid disease, hospitalization, or surgery, and no known drug allergies. No relative afferent pupillary defect was detected. The anterior segment was grossly quiet, and on direct ophthalmoscopy, the posterior poles were grossly within normal limits. Three hours after presentation to the emergency room, he was evaluated further in the ophthalmology clinic. He achieved 20/25 OD with -6.50 sphere and 20/30 OS with -6.00 sphere. Interpalpebral fissures were 9.5 mm with no evidence of lid retraction or upper eyelid lag. Ocular ductions were full with no manifest deviation, and sensory divisions of cranial nerve V were intact. Exophthalmometry measurements were 24.5 OD and 25.0 OS at a base of 95 mm. There was diffuse superficial punctate keratitis OS with a corneal abrasion and subconjunctival hemorrhage superiorly. On dilated funduscopic examination, the cup-to-disc ratios were 0.6 bilaterally with no pallor or edema. The macula, vessels, and periphery were within normal limits.
He was treated with ciprofloxacin drops and instructed to discontinue wearing contacts for one week. The cornea healed completely in 2 days. He was found to be euthyroid with a normal thyroid-stimulating hormone (TSH) and no evidence of autoimmune thyroid disease on lab testing. In 4 years of follow up, he continues to wear contact lenses successfully and has not had another episode of globe luxation.
Our review found 26 reported cases of spontaneous globe luxation presented in the English literature over the last century. 1–20 Including the present case, patients ranged in age from 11 months to 73 years with a mean of 38 years. There were 11 males, 14 females and one unknown. The racial distribution consisted of 13 white, 8 black, 1 Indian, 1 Native American, and 3 unreported patients. Luxation occurred bilaterally in 11 cases, unilaterally in 13 cases, and was not specified in 2 cases. The unilateral cases involved the left eye in 12 cases and the right eye in one case.
Although “spontaneous,” spontaneous luxation occurred in most individuals (73%) after some precipitating trigger. The most common trigger (79%) was lid manipulation, most often by an ophthalmologist during an examination. Luxation has been reported with exophthalmometry, applanation tonometry, indirect funduscopic examination, and foreign body removal. To our knowledge, we report the first case of spontaneous globe luxation associated with lid manipulation for contact lens placement. Other triggers included extremes of gaze and Valsalva maneuvers, including crying, coughing, sneezing, nose-blowing, bending over, and straining. Spontaneous luxation without any reported precipitating trigger was recorded in 27% of the patients. From that group, six out of seven patients had orbital disease contributing to exophthalmos, and the other patient was obese, with presumed increased orbital fat.
The most commonly reported risk factor associated with spontaneous globe luxation was exophthalmos (81%), which can be related to space-occupying orbital pathophysiologic processes and conditions with shallow orbits. Space-occupying orbital processes that have been reported include Grave disease, orbital tumors, histiocytosis X, Englemann disease, excessive orbital fat, and cerebral gumma. Conditions associated with shallow orbits include craniostenosis (i.e., oxycephaly and brachycephaly) and craniofacial dysostoses (i.e., Crouzon disease). Only 11 cases reported exophthalmometry readings that ranged from 22 to 29 mm (mean = 24.7 mm).
Exophthalmos cannot be the only factor because many patients present with exophthalmometry readings that are far more significant, without any prior history of globe luxation. Lid retraction appears to be a minor risk factor, as it was specifically noted in 27% of cases. Ambiguous laxity of the extraocular muscles and supporting fascia may also play a role. One patient with floppy-eyelid syndrome was reported with spontaneous luxation. Seven of the cases reported refractive errors that ranged from −8.25 to plano to +5.00; no pattern of refractive error emerged as an independent risk factor.
Our patient had mild exophthalmos and moderate myopia. The manual spreading of the eyelids, posterior pressure against the orbital rims, and the possible relaxation of the retractor bulbi muscles allowed the equator of the globe to advance forward beyond the lid aperture. When the corneal surface became dry, a blink reflex was initiated. Contraction of the orbicularis muscle in this scenario allowed the lids to become imbricated behind the equator of the globe. Further drying of the corneal surface allowed further lid contraction, setting up a cycle that made manual reduction difficult.
Immediate complications associated with spontaneous globe luxation may include exposure keratitis, corneal abrasion, blurred vision, pain, blepharospasm, and high anxiety. Long-term sequelae such as permanent visual loss appear to be uncommon, presumably due to laxity of the optic nerve. Kimball (1914) reported a 30-year-old male inpatient at a hospital for the insane who regularly luxated his left globe with lid manipulation and developed “complete atrophy of the optic nerve.”3 Chandran and Thomas (1981) reported a 29-year-old woman with Crouzon disease, with severe exposure keratoconjunctivitis and optic atrophy, who progressed to no light perception vision bilaterally after refusing surgical intervention. 15 These two cases with profound visual loss had unique social and anatomic circumstances.
In 1978, Meyer and colleagues reported a 73-year-old man who presented 2 days after an episode of spontaneous luxation of the left eye. No visual complaints were reported; however, on examination, the vision was reduced to 6/60 compared with 6/8 on the right. On funduscopic evaluation, a superotemporal branch retinal vein occlusion was noted. This elderly patient had a history of hypertension and significant evidence of arteriolar attenuation and arteriovenous crossing changes as well. Globe luxation may have impaired venous drainage from the central retinal vein and caused the vein occlusion in this patient who had predisposing factors. No visual sequelae were reported in the 23 other cases reviewed.
Acute treatment of spontaneous globe luxation is the immediate reduction of the globe, and long-term treatment is the prevention of further luxation episodes. Relaxation of the patient and especially the orbicularis muscle are the key elements in globe reduction in the acute setting. Exposure keratoconjunctivitis often leads to further blepharospasm, making manual reduction a challenge. Topical anesthetic to the exposed cornea may allow sufficient relaxation of the orbicularis to allow reduction. Manual reduction generally involves gentle posterior pressure on the globe combined with pulling the eyelids anteriorly. A lid speculum, Desmarres retractor, or tissue forceps may be helpful. These methods are easily augmented with a facial nerve block, which works quickly to paralyze the orbicularis muscle. Lateral canthotomy has not been necessary in our experience. Systemic sedation and even general anesthesia 17 may be necessary in some cases.
Prophylaxis from further episodes begins with educating the patient about potential triggers, especially lid manipulation. Surprisingly, our patient continues to successfully wear contact lenses and has learned to place them with less lid manipulation. Many patients, especially those with thyroid orbitopathy, learn to reduce the globe themselves. If there is an identifiable disease process contributing to the globe luxation, definitive treatment of the underlying disease may be beneficial. Luxation in a patient with histiocytosis X resolved after treatment with radiation and steroid therapy. 17 Orbital decompression in patients with thyroid orbitopathy and proptosis has been successful in eradicating spontaneous globe luxation (authors’ personal experience in 7 patients). Removal of an orbital tumor may also be definitive (personal experience in a patient with a 3-cm intraconal cavernous hemangioma). Two obese patients had measurable reduction in exophthalmometry readings and decreased spontaneous luxation with weight loss alone. 9,16
The most commonly mentioned treatment in older reports was temporal tarsorrhaphy; however, in some patients, this failed to prevent further episodes of luxation. 13,15,17 Wood (1988) reported that a lateral tarsorrhaphy may actually increase the risk of luxation by producing a tighter orbit and increased intraorbital pressure. 17 Furthermore, once luxation had occurred, the smaller remaining palpebral aperture hindered reduction of the globe. Lid lengthening procedures (i.e., mullerectomy, levator recession, lower lid elevation, etc.) may be helpful in cases where lid retraction is thought to play a role in causing the luxation.
We report a patient who developed spontaneous globe luxation while attempting to place a contact lens. Eye care specialists who fit contact lenses need to be aware of risk factors associated with globe luxation and aware of potential interventions to avoid sequelae.
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© 2002 The Contact Lens Association of Ophthalmologists, Inc.