Eye Pain for the Ophthalmologist
Andrew G. Lee, MD Â· Paul W. Brazis, MD
Eye pain is a common and often frustrating complaint of patients. This tutorial reviews the common causes of eye pain and discusses patients who have an abnormal eye examination (eg, optic neuritis, uveitis, orbital inflammatory pseudotumor, angle closure or secondary glaucoma, dry eyes, or corneal abrasion), a history of transient symptoms or intermittent signs but a normal ocular examination at the time of the encounter, or a normal ocular examination but specific eye and headache syndromes.1-96
Patients Presenting With an Abnormal Eye Examination
A complete ocular history and examination should be performed in every patient with eye pain. This examination should include an assessment of best-corrected visual acuity, pupil size, reactivity, testing for a relative afferent pupillary defect (RAPD), slit lamp biomicroscopy, extraocular motility, exophthalmometry, intraocular pressure (IOP) measurement, external and adnexal examinations, and a dilated fundus examination. The slit lamp examination may confirm conjunctivitis, episcleritis, scleritis, corneal abrasions or erosions, uveitis, or dry eye. Additional testing such as a Schirmer test or rose bengal staining of the corneal epithelium may reveal an underlying dry eye syndrome. Patients should be evaluated for signs of optic neuropathy or orbital involvement, which may prompt orbital imaging in patients with eye pain. In addition to the complete eye examination, a formal exophthalmometry (eg, Hertel exophthalmometer) and a trigeminal nerve assessment (ie, cutaneous pinprick sensation in the three trigeminal divisions and corneal sensation) should be performed in all patients with eye pain.1-5 Although most cases of eye pain are benign (eg, dry eye, foreign body, corneal abrasion or erosion), some cases are potentially vision threatening or associated with underlying systemic disease.
Dry eye is a common cause of recurrent, intermittent, or constant unilateral or bilateral eye pain. The pain may be mild, moderate, or severe. Patients with eye pain should be assessed for tear film abnormalities, corneal epithelial erosions, (eg, rose bengal, fluorescein) and possibly Schirmer tear testing. A history of refractive surgery, anticholinergic medications, or systemic disorders affecting the lacrimal gland or tearing (eg, SjÃ¶gren syndrome, sarcoidosis) may also cause dry eye. A topical anesthetic trial in the clinic (one drop in the eye lane) may differentiate corneal epithelial and dry eye conditions from more serious intraocular or intracranial causes of eye pain. An empiric trial of artificial tears, even without definite dry eye findings upon ocular examination, may be diagnostic.1 Chronic eye pain, dry eyes, light sensitivity, and reflex blepharospasm (ie, "photo-oculodynia syndrome") may be an "eye pain equivalent" of sympathetic dystrophy and may respond to cervical sympathetic block.20
Scleritis produces pain that is often severe, deep, penetrating, and intense. The pain may be intermittent or constant, centered on the eye or orbital, and radiate to the face, forehead, brow, jaw, or sinuses. The pain may be so severe that it awakens a patient at night. The eye is often tender to touch. Past medical history of inflammatory disorders (eg, systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, psoriatic arthritis, gout, inflammatory bowel disease, relapsing polychondritis, polymyositis, SjÃ¶gren syndrome, mixed connective tissue disease, progressive systemic sclerosis, polyarteritis nodosa, Wegenerâ€™s granulomatosis) may be helpful. Examination in natural light may disclose subtle findings of scleritis. Intraocular inflammation may also be present (eg, uveitis, glaucoma), and scleral thinning in severe or chronic cases with a blue hue to the sclera may occur. Although anterior scleritis usually shows marked injection of the sclera, deep vessel engorgement, focal tenderness, scleral nodule, or thinning, these findings are often absent in posterior scleritis. Posterior segment findings of posterior scleritis may include choroidal folds, a posterior segment mass, effusion or detachment, exudative retinal detachment, macular edema, or optic disc edema. Orbital and ocular ultrasound may be helpful in these cases.
Primary open-angle glaucoma is painless. However, some secondary glaucomas (eg, Posner-Schlossman, intermittent-angle closure, pigmentary, and uveitic glaucoma) can produce intermittent pain. Patients with secondary glaucoma may have a normal eye examination and IOP between attacks. Patients experiencing glaucomatocyclitic crises (Posner-Schlossman syndrome) may present with eye pain due to transient attacks of acute IOP elevation.22 The typical patient is a young adult (,50 years) and experiences photophobia, blurred vision, or colored halos during eye pain episodes. Secondary glaucoma is usually unilateral, but may be bilateral. An ocular examination performed during an attack may show a mild uveitis and fine keratic precipitates, whereas a gonioscopy typically shows an open angle without synechiae. Acute angle-closure glaucoma can produce intermittent eye pain, and the diagnosis can be clinically confirmed during an attack (eg, corneal edema, markedly elevated IOP, closed angle on gonioscopy). Subacute and intermittent episodes of angle-closure glaucoma may produce eye pain, and a narrow angle can be documented on gonioscopy. Pigmentary glaucoma, such as Posner-Schlossman syndrome, may produce acute intermittent spikes in IOP and eye pain, which may occur during exercise. Pigmentary cells may be seen in the anterior chamber, and gonioscopy may show pigmentation in the trabecular meshwork. Radial mid-peripheral "spoke-like" iris transillumination defects or a pigmented vertical band on the corneal endothelium (ie, Krukenberg spindle) may be present. Patients with intermittent uveitis and secondary uveitic glaucoma may also have intermittent eye pain. Signs of active or prior uveitis (eg keratitic precipitates, posterior synechiae) should be sought. Prior cataract extraction with secondary inflammation caused by the IOL can produce intermittent pain, uveitis, glaucoma, or hyphema (UGH syndrome).23
Eye pain may occur in the setting of an optic neuropathy (ie, decreased visual acuity, color vision, or visual field, an ipsilateral RAPD, and a pale, swollen, or, in acute retrobulbar cases, a normal optic nerve). Eye pain, especially on eye movement with clinical evidence for an acute optic neuropathy in young patients, is typically demyelinating or inflammatory optic neuritis. Neuroimaging (preferably fat suppressed post-contrast orbital and brain magnetic resonance imaging [MRI]) is recommended for eye pain with an optic neuropathy. Cranial MRI may show enhancement of the optic nerve and demyelinating white matter lesions.6 Likewise, patients with optic perineuritis may have pain in the setting of an optic neuropathy. MRI typically shows enhancement of the optic nerve sheath rather than the optic nerve as in optic neuritis. Pain is often a predominant component in optic perineuritis, and in general the disorder is exquisitely steroid sensitive.7 Ophthalmologists should also be aware that pain in both optic neuritis and optic perineuritis may precede visual loss.
Orbital disease (eg, infectious, neoplastic, inflammatory, traumatic, vascular) typically produces orbital signs (eg, proptosis, chemosis, injection, or ophthalmoplegia). Intermittent eye pain may occur with orbital vascular lesions (eg, venous or arteriovenous malformations or lymphangioma) that may be precipitated or worsened intermittently. The pain may worsen with straining (eg, Valsalva maneuver), coughing, crying, bending, or hyperextending the neck.24 The eye examination may be normal between episodes.
Orbital inflammatory pseudotumor
Idiopathic orbital inflammation may accompany eye pain. The inflammation may be centered on the extraocular muscles (ie, myositis), the lacrimal gland (ie, dacryoadenitis), the sclera (ie, scleritis), or the trochlea (ie, trochleitis). The diagnosis is obvious when patients present with severe pain, conjunctival chemosis and injection, ptosis, ophthalmoplegia, or proptosis. However, orbital involvement (eg, isolated myositis, dacryoadenitis, or trochleitis) may not have external eye findings. The diagnosis should be suspected in patients who have pain with eye movement, ophthalmoplegia, or proptosis. Dacryoadenitis may show erythema and edema in the lateral superior lid, proptosis, or globe displacement. Trochleitis may produce focal tenderness in the superonasal orbit. Orbital ultrasound may reveal characteristic inflammatory findings in orbital inflammatory pseudotumor, but orbital imaging (eg, computed tomography or MRI) may be necessary for a definitive diagnosis.10-14 Nonsteroidal anti-inflammatory agents (NSAIDS) or steroid treatment may resolve the symptoms.
A carotid-cavernous fistula (CCF) typically presents with inflammatory orbital and lid findings (eg, dilated periorbital vessels, conjunctival chemosis and arterialization, orbital congestion, lid swelling, proptosis, subjective and objective bruits, increased IOP, and ophthalmoplegia) secondary to venous congestion and arterialization of flow (typically through the superior ophthalmic vein). Posterior drainage of the fistula (ie, drainage into the inferior petrosal sinus) may lack orbital findings of the "red-eyed" anterior draining fistula. The posterior draining CCF (ie, "white-eye shunt")15-16 may require orbital ultrasound (demonstrating arterialized anterior orbital flow) or orbital imaging to make the diagnosis. The pain in the CCF may precede the other more classic signs of CCF, especially in the posterior draining CCF.
Phantom Eye Pain
"Phantom eye pain" (similar to phantom limb pain) may occur following enucleation. The symptoms may develop days to months after surgery and may be more frequent in patients with preexisting headache history.30 A post-enucleation amputation neuroma may produce eye pain as well.
Giant cell arteritis
Giant cell arteritis (GCA) should be considered in patients with new onset headache, scalp tenderness, or severe eye pain with or without visual loss (in elderly patients). A serum erythrocyte sedimentation rate, C-reactive protein, and temporal artery biopsy may be necessary. Although isolated eye pain is an unusual symptom of GCA (presumably related to orbital ischemia), in one series eye pain was seen in seven of 83 patients (8.6%), and in another series one patient out of 18 (5.6%) did not have systemic symptoms of GCA ("occult GCA").91-92
Herpes zoster virus
Herpes zoster ophthalmicus (HZO) involves the ophthalmic division of the trigeminal nerve and can produce eye pain. Although an active or past vesicular is typically present during the dermatomal distribution cutaneous eruption phase in HZO, the pain may rarely precede the rash. HZO can produce intraocular involvement (eg, scleritis, uveitis, keratitis). Post-herpetic neuralgia in the ipsilateral trigeminal distribution may develop and is more common in older patients than in younger patients. The pain may be intermittent or constant, burning or gnawing, and may involve the ophthalmic distribution (ie, eye pain).1
The trigeminal (fifth cranial) nerve innervates the ipsilateral face and eye. Every patient with eye pain should have an assessment of the three divisions of the cutaneous trigeminal nerve (ie, ophthalmic, maxillary, and mandibular), including corneal sensation. Patients with ophthalmic division involvement may have eye pain as a presenting or predominant complaint. Unexplained paresthesias, numbness, or sensory loss in the trigeminal distribution should prompt neuroimaging. Perineural spread of an ipsilateral cutaneous malignancy should also be considered in patients with trigeminal pain or paresthesias. Patients may not volunteer the history of prior removal of the skin malignancy without prompting (eg, basal cell or squamous cell carcinoma).17-19
Trigeminal neuralgia (tic douloureux) produces acute, severe, excruciating, lancinating, paroxysmal, and unilateral pain in the distribution of one or more of the divisions (often the maxillary or mandibular) of the trigeminal nerve. Although ophthalmic division involvement is uncommon, it can produce stabbing eye pain. Trigeminal neuralgia is more common in older women than in older men. Bilateral cases are rare but can occur in demyelinating disease. The paroxysms are brief (ie, seconds) and may occur multiple times per day. Attacks may awaken patients at night and are often triggered by non-nociceptive facial stimulation (eg, touch, jaw movement, drinking hot or cold liquids). Neuroimaging is recommended for unexplained trigeminal neuralgia to exclude intracranial lesions affecting the fifth cranial nerve (eg, multiple sclerosis, brainstem infarction, cerebellopontine angle tumor, aberrant posterior circulation vessels).68-77
Transient ischemic attack
Transient ischemic monocular visual loss (ie, amaurosis fugax) may be associated with eye pain. Intracranial disorders can produce pain by radiating to the ophthalmic division of the trigeminal nerve. Up to 25% of patients with ischemic internal carotid artery or middle cerebral artery stroke have ipsilateral frontal or orbit or eye pain.25,26 Thus, ophthalmologists should not rely solely upon the presence of headaches or eye pain in patients with transient visual loss as proof of the diagnosis of "migraine with aura." The diagnosis of "ocular" migraine in older or vasculopathic patients should be one of exclusion, particularly on the first episode. Ipsilateral carotid dissection should also be considered in the differential diagnosis of pain (neck, head, face, eye) and amaurosis fugax.
Transient increased intracranial pressure
Eye pain, usually in the setting of a headache, can occur due to increased intracranial pressure. Transient increased intracranial pressure may occur after coughing, sneezing, straining, laughing, bending, stooping, or weight lifting and may occur as a result of intracranial disease (eg, Chiari malformation, basilar invagination from Pagetâ€™s disease, posterior fossa tumor).
Exertional or cough-related headache or eye pain
ough-, post-coital-, or exertion-induced headaches or eye pain are usually benign. The pain is short lasting (seconds to minutes) but is often severe, explosive, or throbbing. Unexplained cough, Valsalva, or exertion-induced headaches or eye pain may occur in cerebral aneurysm, vertebrobasilar disease, or carotid stenosis and may be an indication for neuroimaging.27-29
Headache syndromes that may produce eye pain
Specific neurologic pain syndromes may present to ophthalmologists and include primary short duration eye pain syndromes with and without autonomic features, primary long-duration headache syndromes, and secondary eye pain syndromes.
Primary Short-lasting Eye Pain Syndromes with Autonomic Features
Cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform pain with conjunctival injection and tearing (SUNCT) may present with eye pain to the ophthalmologist. Cluster headache is typically periodic, often severe, unilateral, and paroxysmal, and occurs in clusters. Cluster headache is more common in young men than in other patients. The pain may be localized to the eye, temple, forehead, or cheek region, lasts minutes at a time (20 to 45 minutes), occurs in clusters (several per day) or may be periodic, and may be accompanied by ipsilateral tearing, conjunctival congestion, rhinorrhea, forehead/facial sweating, and lid edema. Cluster-tic syndrome is a cluster variant with stabbing, icepick-like neuralgic pain components affecting the eye, face, and jaw.31 Horner syndrome may occur transiently or become permanent after multiple attacks.1 Neuroimaging is recommended for new onset cluster headache, especially if Horner syndrome is present.
Paroxysmal hemicrania (PH) is a unilateral, severe but short-duration headache associated with ipsilateral autonomic manifestations. Unlike cluster headache, PH is more common in women than in men. PH typically is short lasting (10 to 15 minutes) with a variable frequency (few to multiple episodes per day). The clinical features of PH are similar to those of cluster headache, but the duration of PH tends to be more chronic than the duration of cluster headache. Activity can provoke PH attacks, and patients prefer to lie down in a quiet room rather than pace like patients with cluster headache. PH may be chronic (ie, chronic paroxysmal hemicrania or CPH) or episodic (episodic paroxysmal hemicrania).40-55
Short-lasting unilateral neuralgiform pain with conjunctival injection and tearing
SUNCT is characterized by short attacks lasting seconds to minutes. SUNCT occurs more frequently in men than in women (4:1), the frequency of attacks is high (multiple attacks per hour), and the pain is associated with autonomic features (eg, conjunctival injection and tearing, forehead sweating, or rhinorrhea). Neuroimaging may be required in atypical cases because secondary SUNCT syndrome has been described in intracranial disorders (eg, cerebellopontine angle and brainstem arteriovenous or cavernous malformations).55-67
Sphenopalatine and vidian neuralagia
Sphenopalatine neuralgia (Sluderâ€™s neuralgia) is characterized by paroxysms of severe, unilateral pain (similar to trigeminal neuralgia) but is localized to the middle third of the face, behind the eye, upper jaw, teeth, nose, and soft palate rather than confined to one or more trigeminal distributions. As in SUNCT and cluster headache, associated vasomotor activity (eg, nasal drainage, conjunctival injection or irritation, and lacrimation) may be present. Sphenopalatine neuralgia is more common in women than in men. The pain may involve the temple, occiput, and neck. Vidian neuralgia is similar to sphenopalatine neuralgia, but the pain is localized to the face, neck, and shoulder.78 Greater occipital neuralgia (greater occipital nerve) arises from the occiput and may radiate anterior to the ipsilateral eye. Local anesthetic injection of the greater occipital nerve may relieve the pain.79-81 Idiopathic stabbing headache (ie,"jabs and jolts" syndrome, icepick-like headache,"" needle-in-the-eye" syndrome) is characterized by acute, short-duration (seconds) paroxysms of pain confined to the head, face, or eye.
Ice cream headache
Having an ice cream ("brain freeze") or cold drink can produce a paroxysm of severe, short-lasting (seconds) pain that may be referred to the eye. It is common in patients with migraines and is benign.82-83
Primary Long-lasting Headache Syndromes
Migraine is a common headache syndrome that affects women more than men. Migraine is long lasting (typically hours to days), usually hemicranial (but can be bilateral or diffuse), has a "pulsating or throbbing" quality, and is typically moderate to severe in intensity (disrupts activity). Migraine is worsened by activity and is often improved by lying down in a dark room. Associated nausea or vomiting is present, as well as photophobia or phonophobia. A visual aura or other prodrome may or not precede the headache phase. Migraine headache may be centered on or be associated with eye pain.
Tension headache, like migraine, is a common headache syndrome. As opposed to migraine, however, tension headache is typically described as a "pressure" or "tightening" band-like sensation rather than a throbbing sensation. Although tension may be severe, it is usually of mild or moderate severity. Unlike patients with migraines, patients with tension headaches often do not disrupt their activities or lie down in a dark room for relief. In contrast to migraine, tension headache is often bilateral and frontal. Also, the autonomic features of migraine (eg, nausea and vomiting) are not present in tension headache.
Hemicrania continua (HC) is a long-lasting and sometimes continuous unilateral headache. HC is moderate to severe, is often associated with superimposed ipsilateral stabbing, jabbing, or icepick-like pains, and can be provoked by physical exertion. HC affects more women than men, and patients are usually young (mean 35 years).
Secondary pain referred to the eye from nonophthalmic or noncranial pathology
The secondary pain syndromes that can produce eye pain include referred pain from adjacent structures (eg, paranasal sinus disease, oromaxillofacial disease, carotid disease), and rarely nonmetastatic lung cancer.93-94
Ophthalmologists should be aware of the major causes of eye pain including pain affecting patients with abnormal ocular examinations, transient symptoms or intermittent examination findings, and normal eye examinations but defined neurologic or neuro-ophthalmic eye pain syndromes. The recommended approach to eye pain is summarized in Table 1. Most patients with isolated eye pain and a normal eye examination do not require neuroimaging, and experience has shown that the diagnostic yield is low in this setting for imaging. Nevertheless, specific eye findings (eg, optic neuropathy, proptosis, ophthalmoplegia, Horner syndrome) or specific pain syndromes (eg, trigeminal neuralgia) should undergo imaging to exclude intracranial or intraorbital etiologies for the eye pain.
A complete ocular examination including assessment for optic neuropathy, ophthalmoplegia, proptosis (ie, Hertel exophthalmetry), and trigeminal nerve function should be performed for every patient with eye pain to exclude an intraocular or intraorbital etiology.
Patients with intermittent symptoms may benefit from an ocular examination during an attack.
A careful history should be taken to diagnose the major primary and secondary headache syndromes associated with eye pain.
Giant cell arteritis should be considered in elderly patients with eye or face pain.
Orbital ultrasound or orbital imaging might be useful for diagnosing posterior scleritis or orbital myositis even in the absence of ocular examination findings.
Neuroimaging studies are generally low yield in patients with isolated eye pain but should be considered in patients with localizing signs (eg, Horner syndrome, proptosis, ophthalmoplegia, optic neuropathy) or in patients with defined headache or eye pain syndromes associated with underlying structural pathology (eg, atypical facial pain, trigeminal dysfunction, or persistent unremitting and unexplained symptoms).
Dry eye and orbital myositis are common causes of isolated eye pain with a normal eye examination, and an empiric trial of artificial tears and/or over-the-counter NSAIDS may be useful.
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