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Ophthalmic Emergencies
Paul Riordan-Eva, FRCOphth
INTRODUCTION
Prompt recognition and treatment of ophthalmic emergencies are crucial to prevention of unnecessary visual impairment. Using simple guidelines, patients requiring emergency or urgent ophthalmologic evaluation can be identified. Intensity and duration of pain, rapidity of onset and severity of visual loss (primarily assessed by visual acuity, which should be measured for each eye in all patients presenting with ophthalmic emergencies), gross appearance of the globe, and abnormalities on ophthalmoscopy are particularly important parameters.
Excluding ocular and orbital trauma, which is covered in Chapter 19, this chapter reviews the common ophthalmic emergencies, for the most part grouped according to the predominant symptom. For each group, the section on triage highlights the features that are crucial during initial assessment such as on presentation to an emergency department. The section on clinical assessment emphasizes what is important during ophthalmologic evaluation. The management of the more common or important entities is then briefly discussed, principally to provide reference to discussion in other chapters.
ACUTE RED EYE
The majority of patients with acute red eye have a relatively benign condition, such as bacterial, viral, or allergic conjunctivitis, subconjunctival hemorrhage, or blepharitis, which poses little or no threat to vision. Conversely a few are at risk
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of rapid progression within a few hours or days to severe visual impairment, even blindness, such as from acute angle-closure glaucoma, intraocular infection (endophthalmitis), bacterial, viral, amebic, or fungal corneal infection, acute uveitis, or scleritis.
Triage
(See Differential Diagnosis of Common Causes of the Inflamed Eye on Inside Front Cover)
Emergency or urgent ophthalmologic evaluation should be arranged for any patient with acute red eye and a history within the past few weeks of intraocular surgery, which predisposes to endophthalmitis; contact lens wear, which predisposes to corneal infection; recent or distant history of corneal transplantation because of the possibility of graft rejection; previous episodes of acute uveitis or scleritis; or systemic diseases predisposing to uveitis or scleritis, such as ankylosing spondylitis and rheumatoid arthritis. In acutely ill patients, particularly those with sepsis or requiring prolonged intravenous cannulation such as in intensive therapy units or for parenteral nutrition, an acute red eye may be due to bacterial or fungal endophthalmitis. Ocular involvement in toxic epidermal necrolysis, Stevens-Johnson syndrome, or erythema multiforme requires urgent ophthalmologic assessment.
Pain, rather than discomfort, should be regarded as inconsistent with conjunctivitis, episcleritis, or blepharitis. It is suggestive of keratitis, intraocular or scleral inflammation, or raised intraocular pressure, with the likelihood of a serious cause increasing with increasing severity. Associated nausea and vomiting are particularly suggestive of markedly raised intraocular pressure. Deep, boring pain, typically waking the patient at night, is characteristic of scleritis. Photophobia characteristically occurs in keratitis and anterior uveitis.
Reduced vision, whether reported by the patient or identified by measurement of visual acuity, in the absence of a pre-existing explanation, should also be regarded as inconsistent with conjunctivitis, episcleritis, or blepharitis, and as with pain, the greater the severity, the greater is the likelihood of a serious cause.
Severity of redness is not necessarily a guide to the seriousness of the underlying condition for example, despite its bright red appearance, subconjunctival hemorrhage is a benign entity. Distribution of redness can be helpful; predominance around the limbus (circumcorneal) is indicative of intraocular disease, diffuse redness involving the tarsal and bulbar conjunctiva is
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indicative of conjunctivitis, focal or diffuse redness of the globe is consistent with episcleritis, and redness of the eyelid margins is indicative of blepharitis. Bluish redness (violaceous discoloration) of the globe, best identified in natural rather than artificial light, is characteristic of scleritis. Vesicles or ulcerations of the lids or periocular skin are typical of ophthalmic zoster (shingles) and less commonly varicella or primary herpes simplex virus infection.
Conjunctivitis usually causes purulent, mucoid, or watery discharge, and allergic conjunctivitis typically causes itching. Profuse purulent discharge is characteristic of gonococcal conjunctivitis, which requires emergency treatment (see later in the chapter).
Any abnormality of the cornea apparent on gross examination, such as ulceration (Figure 3–1) or focal opacity (Figure 3–2), which may be due to infection, or diffuse cloudiness, which may be due to markedly raised intraocular pressure when it is usually associated with a semi-dilated unreactive pupil, warrants emergency ophthalmologic assessment unless it is known to be longstanding (eg, pterygium). Instillation of fluorescein facilitates identification of an epithelial defect (Figure 3–3), including dendritic ulceration due to herpes simplex virus keratitis. A constricted pupil is suggestive of intraocular inflammation, typically due to anterior uveitis. Hypopyon (pus within the anterior chamber), a feature of corneal infection, intraocular infection, or acute anterior uveitis (iritis) (Figure 3–4), necessitates emergency ophthalmologic assessment.
Figure 3–1. Acute red eye with peripheral corneal ulceration.
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Figure 3–2. Acute red eye with focal corneal opacity.
Figure 3–3. Large corneal epithelial defect. A: Before instillation of fluorescein. B: After instillation of fluorescein.
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Figure 3–4. Hypopyon in acute anterior uveitis (iritis).
Clinical Assessment
Slitlamp examination facilitates assessment of distribution of redness; identification of conjunctival abnormalities, including examination of the superior tarsal conjunctiva following eversion of the upper eyelid; diagnosis of episcleritis and scleritis; characterization of corneal lesions; and detection of corneal keratic precipitates, anterior chamber flare and cells, and possibly hypopyon indicative of anterior chamber inflammation due to anterior uveitis, intraocular infection, or secondary to corneal inflammation, including infection. In cases of intraocular inflammation, dilated fundal examination is essential to determine whether there is involvement of the vitreous, retina, or choroid, which is important to diagnosis as well as assessment of severity (see Chapters 7 and 15).
Management
There are many causes of acute conjunctivitis, which in most cases is a benign, often self-limiting, condition (see Chapter 5). However, care needs to be exercised in neonates (ophthalmia neonatorum) (see Chapter 17) because of the possibility of infection with chlamydia or gonococcus, both of which are associated with nonocular disease that requires systemic therapy, or herpes simplex virus, which may be associated with encephalitis and requires hospitalization and parenteral antiviral therapy. Gonococcal conjunctivitis at all ages, characteristically causing profuse purulent discharge as well as severe conjunctival inflammation, necessitates emergency investigation by microscopy and culture of the discharge and parenteral antibiotic therapy with ceftriaxone to avoid progression to severe corneal damage. Treatment with parenteral antiviral therapy within 72 hours of the appearance of the rash reduces the likelihood of ocular complications in ophthalmic zoster (shingles) (see Chapters 5 and 6). Skin lesions on the tip of the nose (Hutchinson’s sign) or the eyelid margins are predictive of ocular complications.
Management of acute keratitis primarily involves identification and treatment of infection, for which contact lens wear, pre-existing ocular surface disease, and corneal anesthesia or exposure are the common predisposing factors (see Chapter 6). Occasionally it is apparent straightaway that there is a noninfectious inflammatory process that requires other therapy, possibly topical
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or systemic steroids, but steroid therapy should not be started without ophthalmologic assessment.
Management of acute intraocular inflammation (uveitis) also primarily involves identification and treatment of infection, particularly if there is posterior segment involvement (vitritis, retinitis, or choroiditis) or recent history of intraocular surgery, but a noninfectious inflammatory process is more common than in acute keratitis (see Chapter 7). Topical or systemic steroid therapy should not be started without ophthalmologic assessment. Scleritis is infrequently caused by infection, with autoimmune disease being more commonly responsible, and can often be managed in the first instance by oral nonsteroidal anti-inflammatory drugs (NSAIDs), but ophthalmologic assessment is necessary to make the diagnosis and exclude other entities.
In acute angle-closure glaucoma, prompt recognition and treatment are required if severe visual loss is to be avoided (see Chapter 11). The mainstay of initial treatment is intravenous and oral acetazolamide, as well as topical agents, to reduce intraocular pressure, supplemented by topical steroids to reduce inflammation and topical pilocarpine to constrict the pupil. Definitive treatment is usually laser peripheral iridotomy with prophylactic treatment to the fellow eye. Emergency ophthalmologic assessment is essential to establish the diagnosis, including exclusion of other causes of markedly raised intraocular pressure that may require distinctly different treatment.
ACUTE ORBITAL DISEASE
Acute orbital disease is uncommon, but a few entities need to be recognized promptly to avoid the severe visual loss, or even nonocular morbidity and possibly mortality, that may result from delay in treatment (see Chapter 13).
Triage
Orbital cellulitis is usually a disease of childhood and due to spread of infection from the ethmoid sinuses. It is characterized by fever, pain, eyelid swelling and erythema, proptosis, limitation of extraocular movements, and systemic upset with leukocytosis. Pre-septal cellulitis, in which there is no proptosis or limitation of eye movement, may be due to a localized infection in the anterior (pre-septal) portion of the eyelid or may be the early manifestation of orbital
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cellulitis. In adolescents and young adults, orbital signs may be indicative of extension of infection from the fronto-ethmoidal sinus complex. In diabetics and the immunocompromised, acute orbital disease may be due to fungal infection (mucormycosis), with a high risk of death even with early treatment.
Usually occurring in patients with autoimmune hyperthyroidism (Graves’ disease), which may or may not have been diagnosed previously, acute Graves’ ophthalmopathy may lead to marked proptosis, with the possibility of corneal exposure or optic nerve compression, and limitation of eye movements. Pseudotumor, another inflammatory orbital disease, and carotid artery– cavernous sinus fistula, due to dural shunts that typically occur in patients with diabetes and/or systemic hypertension or due to spontaneous rupture of an intracavernous internal carotid artery aneurysm, may present in a similar manner.
Clinical Assessment
Reduced vision unexplained by corneal exposure, especially if associated with impaired color vision and/or a relative afferent pupillary defect, indicates optic nerve dysfunction. In orbital cellulitis, nonaxial proptosis implies abscess formation. Other complications include cavernous sinus thrombosis and intracranial infection, the latter being more likely if there is infection in the frontal sinus.
Management
Orbital cellulitis is a clinical diagnosis and requires immediate institution of antibiotic therapy, usually intravenously, together with early ophthalmologic and otolaryngologic assessments. Orbital imaging may be undertaken in all cases or reserved for those in whom orbital abscess or another complication is suspected.
Orbital imaging, usually computed tomography (CT) but possibly magnetic resonance imaging (MRI), is generally sufficient to differentiate between Graves’ ophthalmopathy, orbital pseudotumor, and carotid artery–cavernous sinus fistula, but orbital ultrasound blood flow studies are particularly helpful in diagnosing the third.
ACUTE PAINLESS VISUAL LOSS
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Sudden-onset painless visual loss is a very important symptom, because it may be due to ophthalmic disease that requires emergency or urgent treatment, ocular vascular disease with immediate or early threat to the patient’s life or remaining vision, or acute intracranial disease.
Triage
It is essential to determine from the outset whether the reported visual loss involves one or both eyes, including clearly distinguishing monocular visual loss from loss of vision to one side in both eyes (ie, homonymous hemianopia). Patients often will not have checked, by closing one eye and then the other, and if necessary, they should be asked to carry out this simple test. Monocular visual loss indicates disease of the globe or optic nerve, whereas bilateral visual loss, including homonymous hemianopia, indicates a lesion at or posterior to the optic chiasm.
Also it is essential to determine whether the visual loss that has been noticed is definitely of recent onset or whether it may have been longstanding and only recently identified. This requires establishing when the patient was last aware that vision in the affected eye(s) was unaffected, such as when last tested by an optometrist.
History of recent onset of black spots or shapes (“floaters”) with flashing lights (photopsia) followed by a field defect progressing upward from below in one eye is characteristic of retinal detachment (see Chapter 9). Preservation of good central vision, implying that the central retina (macula) has not yet detached, warrants emergency ophthalmologic referral. Sudden onset of floaters may also be caused by vitreous hemorrhage, of which the main causes are retinal tear and proliferative retinopathy due to diabetes or retinal vein occlusion. Any patient with sudden-onset floaters and/or flashes, even with otherwise normal vision, requires urgent ophthalmologic assessment. Unless another cause is apparent, patients age 55 or older with acute or subacute unilateral central visual loss, particularly if associated with distortion of images, should be assumed to have wet (neovascular) age-related macular degeneration, and urgent ophthalmologic referral should be arranged.
A reliable account of the rapidity of progression of visual loss can be a very helpful clue to diagnosis, with an abrupt onset being very suggestive of an arterial vascular event. Whether there has been any recovery of vision is important; full recovery after a short period of impairment suggests an embolic
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arterial event. All patients with possible ocular vascular disease should be asked about vascular risk factors, such as diabetes mellitus, systemic hypertension, and hyperlipidemia. Patients age 55 or older with suspected arterial disease must be questioned about symptoms of giant cell arteritis and have their erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) checked.
Ophthalmoscopy (see Chapter 2) often provides the diagnosis in acute painless visual loss. Lack of a red reflex with abnormal or absent view of the retina is suggestive of vitreous hemorrhage or retinal detachment, for which urgent or emergency ophthalmologic referral is required (see Chapter 10). Widespread or sectoral retinal hemorrhages indicate central or branch retinal vein occlusion for which urgent ophthalmologic assessment is indicated. Widespread retinal whitening with a cherry-red spot indicates central retinal artery occlusion for which emergency ophthalmologic assessment must be arranged, and giant cell arteritis and embolic disease need to be excluded. Sectoral retinal whitening indicates branch retinal artery occlusion, for which urgent ophthalmologic assessment is important to confirm the diagnosis, but prompt investigations for embolic disease need to be undertaken. Optic disk swelling in an eye with recent acute or subacute visual loss is commonly due to anterior ischemic optic neuropathy (see Chapter 14), for which giant cell arteritis must be excluded in patients age 55 or older.
Clinical Assessment
The ophthalmologist must clarify whether the visual loss is monocular or binocular, not only by reviewing the history but also by assessment of visual acuity and visual field in each eye, the latter initially by confrontation testing but, if necessary, by perimetry. Detection of bilateral visual field loss, including abnormality in a subjectively unaffected fellow eye, may establish that the disease process involves the optic chiasm, when there is a bitemporal hemianopia or a temporal hemianopia in the subjectively unaffected fellow eye, or the retrochiasmal visual pathways, when there is homonymous hemianopia. Assessment of color vision and pupillary reactions to light, particularly looking for a relative afferent pupillary defect, is important in detection of optic nerve disease (see Chapter 14).
Fundal examination following pupillary dilation provides the best means of diagnosing retinal tears with or without retinal detachment; vitreous hemorrhage and its cause if the hemorrhage is not too dense (otherwise ultrasound examination is necessary); age-related macular degeneration, including whether
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it has features of the wet type; retinal vein or artery occlusion; and anterior ischemic optic neuropathy. In giant cell arteritis, fundal examination may be normal when visual loss is due to choroidal ischemia or posterior ischemic optic neuropathy.
Management
Retinal detachment is usually treated surgically, with urgency primarily being determined by whether the macula is detached but also the underlying cause (see Chapter 10). Management of vitreous hemorrhage is determined by the underlying cause (see Chapters 9 and 10). Repeated intravitreal injection of inhibitors of vascular endothelial growth factor (VEGF) has become the standard treatment for wet age-related macular degeneration (see Chapter 10).
All patients with transient monocular visual loss (“amaurosis fugax”) likely to be due to retinal emboli should undergo investigations for carotid and cardiac sources (see Chapters 14 and 15) with consideration of urgent carotid revascularization if there is 70% or greater ipsilateral internal carotid artery stenosis. Transient visual loss can also be due to giant cell arteritis or optic disk swelling due to raised intracranial pressure (see later in the chapter).
No treatment in the acute stage is established to alter visual outcome in central or branch retinal vein occlusion, but various treatments, including intravitreal injections of vascular endothelial growth factor (VEGF) inhibitors or triamcinolone, retinal laser photocoagulation, and various surgical techniques, are effective for the long-term complications (see Chapter 10). Various treatments, including intra-arterial thrombolytic therapy, are advocated in the acute stage of central retinal artery occlusion, but evidence for their usefulness is lacking, particularly in view of the risk of adverse events in the case of intraarterial thrombolysis (see Chapters 10 and 14). No treatment in the acute stage is established to alter visual outcome in nonarteritic anterior ischemic optic neuropathy (see Chapters 14 and 15), but failure to promptly treat giant cell arteritis causing anterior ischemic optic neuropathy or central retinal artery occlusion is likely to lead rapidly to complete bilateral blindness (see Chapters 14 and 15).
Sudden visual loss due to optic chiasmal or retrochiasmal disease necessitates emergency imaging and appropriate management thereafter, which may involve neurology or neurosurgery referral.
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ACUTE PAINFUL VISUAL LOSS WITHOUT A RED EYE
A number of relatively uncommon but important conditions present with painful visual loss without a red eye because, for the most part, they are retrobulbar in location.
Triage
There are many causes of optic nerve inflammation (“optic neuritis”) (see Table 14–1). The most common is the acute demyelinative optic neuropathy associated with multiple sclerosis, occurring as the initial manifestation or part of a subsequent relapse. It characteristically presents as subacute monocular visual loss with perior retro-ocular discomfort exacerbated by eye movements (see Chapter 14).
Pituitary apoplexy, usually due to hemorrhagic infarction of a pituitary tumor, is rare but requires prompt recognition and treatment to reduce the risk of severe morbidity, possibly death, as well as severe visual loss, possibly complete blindness. Characteristically, it presents with sudden-onset headache, unilateral or bilateral visual loss, sometimes impaired eye movements, and metabolic and circulatory derangement due to pituitary failure, particularly resulting in adrenal insufficiency. Sphenoid sinusitis also presents with headache, typically localized to the vertex, and acute unilateral or bilateral visual loss. Diagnosis of posterior scleritis is often delayed because of the frequent lack of specific diagnostic features, including the absence of apparent inflammation of the globe to suggest an inflammatory condition (see Chapter 7).
Clinical Assessment
In acute demyelinative optic neuropathy, there are features of optic nerve dysfunction (impaired color vision, visual field loss, and a relative afferent pupillary defect), with progression of visual loss over a few days, and usually no ophthalmoscopic abnormality, but mild optic disk swelling is present in onethird of cases.
The ophthalmic manifestations of pituitary apoplexy are unilateral or bilateral often severe visual loss with impaired pupillary light reactions, sometimes
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impaired eye movements (external ophthalmoplegia) due to ocular motor cranial nerve palsies, and normal or pale optic disks depending on whether the pituitary tumor previously has caused anterior visual pathway compression.
In sphenoid sinusitis, the visual loss also has features of optic nerve dysfunction, with normal or pale optic disks depending on whether there has been chronic optic nerve compression from a pre-existing sphenoid sinus mucocele (see Chapter 13). The clinical signs in posterior scleritis include proptosis, limitation of eye movements, induced refractive error, choroidal folds, fundal mass, exudative retinal detachment, and optic disk swelling (see Chapter 7). Ultrasound is the best diagnostic test.
Management
In most cases of acute demyelinative optic neuropathy, the vision recovers spontaneously, and management centers on investigation of the likelihood of multiple sclerosis and the need for disease-modifying therapy, but the crucial issue in the acute management is excluding other entities that require urgent treatment. Pituitary apoplexy is an endocrine and neurosurgical emergency. The patient may require emergency resuscitation. Intravenous hydrocortisone should be given to all patients prior to investigation with MRI, or CT if the patient’s condition is unstable. Urgent neurosurgery is frequently required, particularly in patients with visual loss. Sphenoid sinusitis causing visual loss requires surgical drainage as well as antibiotic therapy. Posterior scleritis may respond to oral NSAIDs, but oral steroid therapy may be required.
DOUBLE VISION AND EYE MOVEMENT ABNORMALITIES
Double vision has many causes, ranging from the benign entity of an incorrect spectacle prescription to the life-threatening expansion of a posterior communicating artery aneurysm. Ocular, orbital, intracranial, generalized neurologic, and systemic diseases can all present with double vision.
Triage
Assessment of double vision is complex and can cause great difficulty. In
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oculomotor (third cranial) nerve palsy, there may be clues from associated ptosis or pupillary abnormality. Otherwise, unless the pattern of double vision reported by the patient or the examination of the range of eye movements quickly leads to identification of a specific entity such as an abducens (sixth cranial) nerve (lateral rectus) palsy, more useful guidance to the clinical urgency is derived from other features, such as whether there is also impairment of vision, orbital signs such as lid swelling or proptosis, periocular pain or headache, nonocular neurologic abnormalities, or systemic illness. In general, patients with multiple cranial nerve palsies or other neurologic features, severe headache, associated systemic illness, or age under 50 with single or multiple cranial nerve palsy are most likely to have a serious underlying condition.
Clinical Assessment
Double vision is usually due to ocular misalignment, but the first step in its evaluation is to determine whether it is monocular or binocular. If double vision, or even more than two images, is present when the patient is viewing with only one eye (monocular), whether it is just with one eye or with each eye alone, the visual disturbance is not due to ocular misalignment. Instead, it is likely to be due to refractive error, lens opacity, or possibly macular disease. Unless there are other features to clearly implicate cerebral disease, multiple images on monocular viewing can be assumed not to be due to intracranial disease.
Effectively every episode of double vision has an acute onset because double vision is either present or not. What needs to be established is for how long double vision has been noticed and whether, during the one or many episodes that have occurred, there has been change in the pattern, as judged by the direction of separation of images and the directions of gaze in which double vision has been present, or severity, as judged by the distance separating the two images. It is also helpful to establish whether the double vision can be overcome with voluntary effort, because this implies a longstanding abnormality that has become more difficult to overcome (decompensated).
Whenever an ocular motor cranial nerve palsy is diagnosed, it is essential to determine whether it is isolated or part of multiple cranial nerve dysfunction, including assessment of trigeminal nerve as well optic nerve function, not only to provide anatomical localization of the disease process but also as a guide to the likelihood of a serious underlying condition.
In oculomotor nerve palsy, the presence of pupillary dysfunction, either
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anisocoria or particularly an impaired response to light, provides an important clue to the possibility of a compressive lesion such as a posterior communicating artery aneurysm (see Chapter 14). Severe pain is another important clue to the presence of an aneurysm, but it also may occur in pituitary apoplexy (see earlier in the chapter).
In all cases of double vision, careful attention needs to be paid to identification of any orbital signs, not least to avoid unnecessary investigation for a possible intracranial lesion. Specific eye movement abnormalities provide precise anatomical localization. Internuclear ophthalmoplegia, in which there is impairment of adduction of one or both eyes, localizes to the medial longitudinal fasciculus within the brainstem (see Chapter 14). Horizontal gaze palsy, in which there is loss of conjugate horizontal gaze to one or both sides, localizes to the pons, whereas vertical gaze palsy localizes to the midbrain (see Chapter 14). Variability of double vision, during or between episodes, typically with increasing severity with fatigue that may also manifest as increasing ptosis, is suggestive of myasthenia gravis (see Chapter 14).
Management
Investigation of patients with binocular double vision depends on the clinical assessment. Many cases of isolated ocular motor cranial nerve palsy in patients over 50 are due to ischemic (microvascular) disease, which requires little investigation apart from exclusion of giant cell arteritis and review of vascular risk factors, and in which spontaneous recovery is the rule. In contrast, in isolated oculomotor nerve palsy, suspicion of posterior communicating artery aneurysm due to pupillary involvement, severity of pain, or age under 50 necessitates emergency imaging, with the outcome being much better if treatment can be undertaken prior to subarachnoid hemorrhage due to aneurysm rupture. Similarly, multiple cranial nerve dysfunction requires urgent investigation, usually guided primarily by a neurologist, who will also guide investigations when the disease process localizes to the brainstem. Management of orbital disease usually depends on the outcome of imaging with CT or MRI. When clinical evaluation suggests decompensation of a longstanding abnormality, such as a congenital superior oblique (trochlear) palsy, further investigation may not be required, and initial treatment will be with prisms (see Chapter 14).
In any patient with suspected myasthenia gravis, it is important to establish whether there is nonocular weakness suggesting generalized disease, especially
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impairment of breathing or swallowing, for which emergency neurologic assessment is essential.
PUPIL ABNORMALITIES
Abnormalities of pupil size and/or reactions result from a wide variety of causes, including structural abnormalities of the iris, which also usually cause pupillary distortion; miosis in intraocular inflammation; mydriasis in markedly raised intraocular pressure; tonic pupil; oculomotor nerve palsy; Horner’s syndrome; and midbrain dysfunction.
Triage
Acute isolated, dilated, unreactive pupil in an otherwise well individual is rarely due to a serious underlying condition, with the likely possibilities being the benign entity of tonic pupil (see Chapter 14) or pharmacologic mydriasis, such as from accidental ocular inoculation with an anticholinergic agent in travel sickness medication. In contrast, isolated, dilated, unreactive pupil in a patient with depressed conscious level due to head injury or other acute intracranial disease is an ominous sign, being suggestive of tentorial herniation. As discussed earlier, pupil involvement in oculomotor nerve palsy is an important clue to the possibility of a compressive lesion, including posterior communicating artery aneurysm. Miosis with ptosis is characteristic of Horner’s syndrome (see Chapter 14). Acute painful Horner’s syndrome, possibly following neck trauma, requires urgent exclusion of carotid dissection. Pupillary light-near dissociation (impaired pupillary constriction to light with better constriction to near) is traditionally associated with central nervous system syphilis (Argyll Robertson pupils), but can be due to midbrain dysfunction, typically compression from a pineal tumor or dilated third ventricle in hydrocephalus, when usually there is also impairment of vertical eye movements.
Clinical Assessment
Besides confirming a suspected diagnosis of oculomotor nerve palsy or Horner’s syndrome, the ophthalmologist’s particular role in assessment of acute pupil abnormalities is the identification of benign entities, such as tonic pupil and
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pharmacologic mydriasis, to avoid unnecessary investigation, and ophthalmic entities, such as acute angle-closure glaucoma, to direct management. In all three instances, there will be no related ptosis or impairment of eye movements. Tonic pupil may be identified by the delayed dilation following a near response from which it derives its name; abnormal spiraling (“vermiform”) movements of the iris when constricting to a light stimulus, best seen on slitlamp examination; or constriction to dilute (0.125%) pilocarpine eye drops. Pharmacologic mydriasis is characterized by lack of pupil constriction to bright light and standard-strength (2%) pilocarpine eye drops.
BILATERAL OPTIC DISK SWELLING
There are many causes of optic disk swelling, including inflammatory or ischemic optic neuropathy, central retinal vein occlusion, uveitis, posterior scleritis, and intra-orbital optic nerve compression, all of which are usually unilateral. Bilateral optic disk swelling is a characteristic feature of raised intracranial pressure and malignant (accelerated) systemic hypertension, both of which require emergency or urgent investigation and treatment (see Chapters 14 and 15).
Triage
Papilledema (optic disk swelling due to raised intracranial pressure) is usually identified as part of the examination of a patient with neurologic symptoms, particularly headache. It may be identified incidentally, such as during routine optometric examination, but even then, it still requires urgent head imaging to exclude an intracranial mass lesion. Blood pressure should be checked in every patient with bilateral optic disk swelling, even a child.
Clinical Assessment
When the abnormalities are florid, recognition of optic disk swelling is straightforward. When the abnormalities are less marked (see Figure 14–9), ophthalmologic assessment may be crucial, particularly to identify other entities such as myelinated nerve fibers, optic nerve head drusen, or congenitally small and crowded optic disks (pseudopapilledema) (Figure 3–5) that mimic optic disk
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swelling, so that unnecessary investigations and anxiety can be avoided. In individuals with papilledema, particularly when it is acute with retinal exudates or atrophic, assessment of vision by an ophthalmologist, including visual fields, is a crucial guide to urgency of treatment. In malignant hypertension, optic disk swelling is usually accompanied by retinal and choroidal abnormalities and is an indication for urgent reduction in blood pressure, although precipitous reduction should be avoided to reduce the risk of optic nerve infarction.
Figure 3–5. Congenitally small and crowded optic disks (pseudopapilledema). A: Right eye. B: Left eye.
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