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Man presents with sudden onset ocular congestion
The left eye had ptosis, loss of periorbital muscle tone, complete ophthalmoplegia and no light perception.
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A 46-year-old white man was referred to the New England Eye Center because of blindness in the left eye associated with periorbital and orbital congestion.
The patient had taken an excessive amount of cyclobenzaprine (Flexeril) 1 week before presentation. He was found unconscious 12 hours later in his home. He was taken to a local hospital where he remained semiconscious and in agitated delirium for 5 days. Left periorbital swelling, bruising and ptosis were noted in the intensive care unit. Seven days after the incident, before his discharge from the hospital, he was seen by a local ophthalmologist who urgently referred the patient for further evaluation.
 Shazia Ahmed |  My Hanh T. Nguyen |
His general health was good except for occasional back pain, for which he was taking cyclobenzaprine. He drank alcohol occasionally and smoked half a pack of cigarettes a day. There was no history of ocular trauma or surgery. Review of systems was insignificant except for profound depression leading up to the suicide attempt.
Examination
In the emergency room, the patient was comfortable but despondent. General physical examination was normal. A complete blood count with differential, erythrocyte sedimentation rate and chemistry panel was within normal limits.
Ophthalmic examination showed 20/20 visual acuity in the right eye and no light perception in the left. The right pupil was reactive from 5 mm to 3 mm with direct stimulation, but it dilated when the left eye was stimulated. The left pupil was dilated and nonreactive at 6 mm. IOP was 17 mm Hg in both eyes. Exophthalmometry measurements were 17 mm in the right eye and 20 mm in the left.
There was complete left ptosis with loss of periorbital muscle tone. Mild upper and lower lid swelling with blue discoloration was noted (Figure 1). Extraocular movement was normal in the right eye, and there was complete ophthalmoplegia in the left (Figure 2). Slit lamp examination showed 360° of moderate chemosis with diffuse conjunctival and episcleral injection. There was stromal corneal edema with folds in Descemet’s membrane. There was mild nuclear sclerotic cataract in both eyes. The left anterior chamber had a few cells. Fundus examination showed no abnormalities in the right eye. The view into the left eye was hazy, but there were a few macular hemorrhages, diffuse retinal whitening, and attenuated arteries and veins. The optic disc was normal. A fluorescein angiogram performed 3 days after presentation showed delayed choroidal filling and no perfusion to the retina (Figure 3).
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Images: Yamamoto I, Hedges TR |
What is your diagnosis?
Ocular congestion
The differential diagnosis of blindness with complete ophthalmoplegia and orbital congestion includes infiltrative disorders of the orbit, cavernous sinus syndromes and orbital ischemia. Infiltrative orbital disorders, which can affect ocular motility and optic nerve function, include orbital cellulitis, idiopathic inflammatory orbital pseudotumor, sarcoidosis, Wegener’s granulomatosis, thyroid orbitopathy and retrobulbar tumors (particularly carcinoma and lymphoma). Retrobulbar hemorrhage can rarely do this. Cavernous sinus syndromes, which can present with blindness and ophthalmoplegia, include cavernous sinus thrombosis, carotid-cavernous fistula from trauma, intracavernous carotid or ophthalmic artery aneurysms, tumors (particularly malignancies arising from the nasopharynx) or idiopathic granulomatous inflammation (Tolosa-Hunt syndrome). Orbital ischemia can occur from vasculitis, particularly giant cell arteritis. Fungal infections such as mucormycosis and aspergillus can lead to ischemia of the retina, optic nerve and extraocular muscles. Finally, prolonged compression of the orbit, which has been described during spinal surgery, may lead to orbital ischemia.
In our patient, there was no history of facial cellulites, sinusitis, gingivitis or recent upper respiratory infection to indicate cavernous sinus thrombosis. There may have been head trauma, raising the possibility of carotid-cavernous fistula, but IOP was normal. Retrobulbar hemorrhage may have occurred from trauma while unconscious. The lack of pain made inflammatory disorders less likely. We felt that external compression must have been applied to the orbit for a prolonged period of time after the patient lost consciousness and before he was found lying on the floor in a coma. The pressure was present long enough to compromise blood flow through the ophthalmic artery, leading to complete ischemia of the orbital tissues, including the extraocular muscles and the retina.
Urgent computed tomography with angiography showed enlarged extraocular muscles and slight thickening of the sclera on the affected side. The cavernous sinus appeared normal, and the superior ophthalmic vein was normal in shape and size (Figure 4). Based on the CT results, the patient was diagnosed with ischemic orbital compartment syndrome from prolonged external compression directly to the orbit during unconsciousness. Systemic hypotension may also have compounded the problem.
Discussion
Ischemic orbital compartment syndrome occurs when external pressure is applied directly over the periorbital area for a prolonged period. This causes pressure to build up within a muscle cone, especially at the orbital apex, resulting in compression of the ophthalmic artery and vein. Branches of the ophthalmic artery, including the central retinal artery, arteries to the extraocular muscles and ciliary arteries, become compromised to the point where there is retinal nonperfusion and complete internal and external ophthalmoplegia.
This syndrome has been reported after prolonged spinal surgeries while patients were placed in the prone position over silicone head rests under general anesthesia. On extubation, facial and periorbital swelling has been noted, and the affected patients complain of periorbital pain and decreased vision. Attempts to reproduce this phenomenon have indicated that systemic hypotension exacerbates or precipitates retinal artery nonperfusion.
Magnetic resonance imaging or computed tomography with angiography needs to be performed urgently in cases of blindness and ophthalmoplegia to rule out orbital infiltrative disorders, cavernous sinus syndromes and retrobulbar hemorrhage. If IOP is elevated in association with a “tight orbit,” a lateral canthotomy and cantholysis should be considered. Otherwise, there is no treatment for this syndrome. Oral prednisone has been tried previously in an attempt to decrease inflammation and edema due to ischemia. However, the visual prognosis is often poor. Periorbital tissue swelling and proptosis often gradually resolve along with improvement in extraocular movement and ptosis.
Our patient was given oral prednisone initially with a quick taper. One week later the ptosis had resolved to some extent, but there still was no light perception in the eye.
For more information:
- Izumi Yamamoto, MD, and Thomas R. Hedges III, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Edited by Shazia Ahmed, MD, and My Hanh T. Nguyen, MD. Drs. Ahmed and Nguyen can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Ahmed and Nguyen have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
- Fuller JR, Vote BJ. Upside-down orbitopathy: Unilateral orbital dependent-tissue oedema causing total visual loss. Clin Experiment Ophthalmol. 2001;29(4):265-267.
- Leibovitch I, Casson R, Laforest C, Selva D. Ischemic orbital compartment syndrome as a complication of spinal surgery in the prone position. Ophthalmology. 2006;113(1):105-108.
- Linberg JV. Orbital compartment syndromes following trauma. Adv Ophthalmic Plast Reconstr Surg. 1987;6:51-62.