Ординатура / Офтальмология / Английские материалы / Primary Care Ophthalmology_Palay, Krachmer_2005
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294 CHAPTER 15 • Systemic Disease and Therapies
Treatment
•Patients should be referred to an ophthalmologist.
•Cycloplegia and topical corticosteroids are used for anterior uveitis.
•The ocular prognosis generally is very good.
Behçet’s Disease
Behçet’s disease is a systemic vasculitis of small blood vessels with major mucocutaneous and ophthalmic manifestations. The diagnosis is based on presence of oral ulcers, which are the most consistent clinical sign, genital ulcers, uveitis or retinitis, and characteristic skin lesions (erythema nodosum). Arthritis and neurologic involvement also are features of this disease.
Symptoms
•Pain, redness, and light sensitivity accompany anterior uveitis if present.
•Vision is decreased if the retina or optic nerve is involved.
Signs
•In cases of anterior uveitis, conjunctival injection, anterior chamber cells and flare, corneal cellular precipitate, and an accumulation of white cells in the anterior chamber (hypopyon) are seen on examination.
•Retinal vasculitis occurs.
•Optic nerve atrophy can occur (Fig. 15–1).
Treatment
•Patients should be referred to an ophthalmologist.
•Cycloplegia and topical corticosteroids are administered.
•Systemic immunosuppression may be necessary for retinal or systemic vasculitis.
FIGURE 15–1 End-stage retinal disease in Behçet’s disease, resulting from recurrent retinal vascular occlusive episodes. Note markedly attenuated retinal vessels, fibrosis, and optic atrophy.
Dermatomyositis 295
•Systemic corticosteroids, cyclosporine, azathioprine, and a combination of azathioprine and cyclophosphamide have been used for treatment.
•The prognosis is poor even with aggressive treatment. Severe vasculitis may lead to a loss of vision.
Dermatomyositis
Dermatomyositis is a collagen-vascular disease that primarily involves the skin and muscle. It is an immune complex–mediated vasculopathic disorder and may occur with other collagen diseases such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and polyarteritis nodosa. Muscle weakness, especially of the proximal limb muscles, may develop over weeks or may be of acute onset. Muscle pain may be present.
Symptoms
•Usually no ocular symptoms are noted.
•Pain, redness, and light sensitivity accompany anterior uveitis if present.
Signs
•A heliotrope discoloration of upper eyelids often is present in patients with telangiectasia (Fig. 15–2).
•Periorbital and conjunctival edema are reported.
•Anterior uveitis is rarely found.
•Retinopathy may manifest as cotton-wool spots.
•Hemorrhages, variable pigmentation of the fundus, and optic atrophy caused by occlusive vasculitis are rare findings.
Treatment
•No specific ocular therapy is necessary unless anterior uveitis is present.
•The ocular prognosis generally is good.
FIGURE 15–2 The violaceous discoloration on the upper lid is a heliotrope rash, a manifestation of telangiectasia, in this case associated with dermatomyositis.
296 CHAPTER 15 • Systemic Disease and Therapies
Diabetes
Diabetes is a metabolic vasculopathy in which permanent visual loss is possible and typically results from retinal vascular disease. (For a more detailed description of diabetic retinopathy, see Chapter 10.)
Symptoms
•Patients have no symptoms until retinopathy is advanced.
•A sudden change in visual acuity occurs; most often, the patient becomes nearsighted.
•A sudden, painless visual loss is associated with vitreous hemorrhage from retinal neovascularization.
•A gradual visual loss often is due to cataracts or macular edema.
•A sudden, painful visual loss is associated with neovascular glaucoma.
Signs
•A change in the refractive error occurs (i.e., glasses need to be stronger or weaker).
•Small, poorly reactive pupils (pseudo–Argyll Robertson pupils) are observed.
•Cortical spokes (Fig. 15–3) or snowflake-type cataracts are evident.
•Retinal hemorrhages, exudates, microaneurysms, neovascularization, and vitreous hemorrhage may be other findings (see Chapter 10).
Ehlers-Danlos Syndrome
Symptoms
•No ocular symptoms are present.
•Vision loss may result as a complication of angioid streaks.
FIGURE 15–3 Spokelike clefts in the lens cortex (1). Cataracts may occur at an earlier age in patients with diabetes than in the general population.
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Ehlers-Danlos Syndrome |
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Signs
•Angioid streaks are found in the retina adjacent to the optic disc (Fig. 15–4). Retinal bleeding may occur as a result of these streaks. Other causes of angioid streaks include sickle cell disease, Paget’s disease of the bone, lead poisoning, and other collagen or connective tissue disorders such as Weill-Marchesani syndrome and pseudoxanthoma elasticum.
•Extreme thinning of the cornea (keratoglobus) can occur with Ehlers-Danlos syndrome type VI (Fig. 15–5).
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FIGURE 15–4 Ehlers-Danlos syndrome: Angioid streaks (1). These irregular, hypopigmented lines are a result of degeneration and breaks in Bruch’s membrane. Angioid streaks also are seen in pseudoxanthoma elasticum, sickle cell disease, and Paget’s disease (osteitis deformans).
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FIGURE 15–5 Ehlers-Danlos syndrome: Blue sclera. This syndrome is characterized by poor cross-linking of collagen, resulting in joint hypermobility, skin hyperextensibility, easy bruising, and a propensity toward tissue rupture. The apparent blue coloration of the sclera results from thinning of the sclera.
298 CHAPTER 15 • Systemic Disease and Therapies
Treatment
•No specific ocular treatment exists. With sudden visual loss resulting from subretinal neovascular membrane associated with angioid streaks, laser photocoagulation may be indicated.
•Patients with extremely thin corneas (keratoglobus) must wear safety glasses at all times, because minor trauma can result in globe rupture and blindness.
Human Immunodeficiency Virus Infection
The human immunodeficiency virus (HIV) is a retrovirus that destroys the cells of the body’s immune system, resulting in a host of systemic and ophthalmic problems.
Molluscum Contagiosum
Molluscum contagiosum is characterized by the presence of nodular, umbilicated lesions often up to 2 to 3 mm in diameter (see Fig. 4–18). This condition occurs more often in HIV-infected patients, and lesions are seen on the eyelids in many cases. They can cause a chronic conjunctivitis. Treatment usually focuses on evacuating the contents of the crater, but simple excision probably is a more definitive treatment.
Herpes Zoster Ophthalmicus
Herpes zoster ophthalmicus is seen with increased frequency in young adults with HIV infection, and it may be the initial sign of HIV infection. It typically begins with a rash or vesicles in the distribution of the fifth cranial nerve (see Fig. 6–9). Signs of ocular involvement include corneal inflammation, glaucoma, and uveitis. The eye abnormalities may progress as the skin lesions resolve. (For treatment, see Chapter 6.)
Kaposi’s Sarcoma
Kaposi’s sarcoma is a bluish-purple vascular tumor frequently seen on the eyelid or conjunctiva in HIV-infected patients. These lesions usually are painless and discrete and may appear as flat patches, elevated papules, or raised nodules. When the lesions occur on the conjunctiva, the disorder initially may manifest as a hemorrhagic conjunctivitis, most commonly occurring inferiorly (Fig. 15–6). Treatment options include surgical excision, cryotherapy, radiotherapy, and chemotherapy, or no treatment may be implemented.
Cotton-Wool Spots
Cotton-wool spots are the most common ocular finding in early HIV-related retinopathy. The lesion is a nerve fiber layer infarct caused by a microvasculopathy. HIVrelated cotton-wool spots appear identical to such spots seen in patients with diabetes or hypertension (see Fig. 10–10). Distinguishing between cotton-wool spots and very early cytomegalovirus retinitis may be difficult; observation leads to the correct diagnosis. No specific treatment of cotton-wool spots is indicated.
Hyperlipidemia 299
FIGURE 15–6 Kaposi’s sarcoma mimics subconjunctival hemorrhage or hemangioma, but the nodularity and thickness of the vessels are distinctive.
FIGURE 15–7 Arcus senilis. A heavy deposition of lipid in the peripheral cornea at a young age (i.e., before the age of 50 years) may be associated with hyperlipidemia. Arcus usually occurs as a normal aging change.
Cytomegalovirus Retinitis
See Chapter 10 for a discussion of ophthalmic disease associated with cytomegalovirus infection.
Hyperlipidemia
Symptoms
• Usually no symptoms are present.
Signs
The following signs may be associated with hyperlipidemia, but not invariably so:
•Heavy arcus of the cornea (especially in patients younger than 50 years of age) (Fig. 15–7)
•Xanthelasma (especially in those younger than 50), although in most cases the xanthelasma is not associated with increased lipids (see Fig. 4–11)
300 CHAPTER 15 • Systemic Disease and Therapies
•Lipid deposition in the retina (lipemia retinalis)—a rare finding that may be seen when serum triglyceride levels exceed 2500 mg/mL
Treatment
•Systemic treatment of hyperlipidemia is given if indicated.
•Areas of xanthelasma may be excised for cosmetic purposes.
•Lipid deposition in the retina usually resolves when the triglyceride levels are normalized.
Hypertension
Symptoms
•Usually no symptoms are present.
•With malignant hypertension, blurred vision, blind spots, and visual loss are possible.
Signs
Ocular Manifestations
•Externally, no signs are noted.
•Retinal findings include focal narrowing of retinal arterioles or general vessel narrowing, light reflex changes, nerve fiber layer hemorrhages, and cotton-wool spots (see Chapter 10).
Additional Signs with Malignant Hypertension
•Papilledema is evident.
•Lipid exudates are found in a star configuration (Fig. 15–8).
•Macular edema occurs.
FIGURE 15–8 Advanced hypertensive retinopathy. Lipid deposits in the macula (1), cotton-wool spots (2), small flame hemorrhages (3), vascular light reflex changes, venous tortuosity, and early disc edema are present.
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Treatment
• The elevated blood pressure is treated.
Inflammatory Bowel Disease
Inflammatory bowel disease, most notably Crohn’s disease, and ulcerative colitis may be associated with ocular abnormalities in the anterior segment of the eye.
Symptoms
•Pain and redness are noted if episcleritis or scleritis is present.
•Pain, light sensitivity, and redness are noted if anterior uveitis is present.
Signs
•In cases of episcleritis and scleritis, local or diffuse scleral injection is observed.
•In cases of anterior uveitis, perilimbal injection, anterior chamber cell and flare, and corneal cellular precipitates are findings.
•Limbal corneal infiltrates may not stain with fluorescein and usually occur adjacent to an area of scleritis (Fig. 15–9).
•Cataracts are possible if the patient has been taking systemic corticosteroids for long periods.
•Dry eye and night blindness are possible findings in severe cases and are secondary to vitamin A deficiency with malabsorptive states.
Treatment
•Patients should be referred to an ophthalmologist.
•Topical corticosteroids and cycloplegics help control inflammation of the cornea or in the anterior chamber.
•Scleritis and associated limbal corneal infiltrates may respond to topical corticosteroids, but in some cases, systemic immunosuppression (e.g., with oral corticosteroids) is needed.
FIGURE 15–9 Sclerokeratitis (1) in a patient with Crohn’s disease.
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302 CHAPTER 15 • Systemic Disease and Therapies
Marfan Syndrome
Marfan syndrome is a connective tissue disorder of autosomal dominant inheritance with ocular, skeletal, and cardiovascular manifestations. The basic defect is in collagen (fibrillin deficiency).
Symptoms
•Monocular diplopia occurs.
•A painless decrease in vision is noted.
•Fluctuating vision occurs.
Signs
•A subluxation or luxation of the lens, usually upward, occurs and is best seen on dilated examination (to allow visualization of the edge of the lens with a slit lamp) or occasionally on gross examination (Fig. 15–10). A tremulousness of the iris known as iridodonesis may indicate a lack of normal lens support.
•Glaucoma may be an associated finding.
•The axial length of the eye increases, resulting in myopia.
Treatment
•Contact lenses are prescribed if the luxated lens is out of the visual axis.
•Lens extraction is performed if the luxated lens obstructs the visual axis.
Mucopolysaccharidoses
The mucopolysaccharidoses are a group of lysosomal storage diseases caused by a deficiency of the enzymes that degrade glycosaminoglycans. Subtypes are categorized by enzyme deficit, inheritance pattern, and clinical features.
FIGURE 15–10 Luxation of crystalline lens (1) in a patient with |
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FIGURE 15–11 Diffuse corneal clouding in Maroteaux-Lamy syndrome, a disorder of mucopolysaccharide metabolism with an autosomal recessive pattern of inheritance.
Symptoms
•Central vision is poor.
•Night vision is poor.
Signs
•Corneal clouding is due to the presence of diffuse, fine punctate opacities in the stroma that are homogeneous and bilateral (Fig. 15–11).
•Depending on the type of storage disease, a pigmentary retinopathy may be manifested as a salt-and-pepper appearance of the retina.
•Papilledema and optic atrophy are possible findings.
•Glaucoma often is an associated disease.
Treatment
•No specific treatment for the eyes is prescribed.
•With extreme corneal clouding, corneal transplantation may be performed.
•Prognosis varies with the subtype of mucopolysaccharidosis.
Myotonic Dystrophy
Myotonic dystrophy is a disorder of autosomal dominant inheritance that involves body musculature with significant associated ophthalmic abnormalities.
Symptoms
•Foreign body sensation, tearing, and a burning sensation are noted with corneal drying and exposure due to the disease.