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Ординатура / Офтальмология / Английские материалы / Moorfields Manual of Ophthalmology_Jackson_2007

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Uveitis in Pregnancy

Patients with uveitis who wish to get pregnant need to consider the risks and benefits of any systemic medication. Acetazolamide is commonly avoided. Prednisolone 15 mg daily is probably safe in the first trimester and throughout pregnancy. There are fewer data on other immunosuppressive agents so avoid if possible, but as with transplant patients azathioprine, ciclosporin, and mycophenolate can be used in pregnancy at the lowest effective dose. Many of the drugs can be found in breast milk, and this information must be given to mothers wishing to breast feed. For drug use in pregnancy see page 702.

Immune-mediated uveitis may get better during pregnancy, stay the same, or get worse. The same applies to postpartum uveitis. Periocular steroids may be used during pregnancy and breastfeeding, and can be given bilaterally if necessary. Although some drug is absorbed systemically by this route, the amount is extremely small and is not thought to harm the fetus.

Patients with reactivation of toxoplasma retinochoroiditis present no infective risk to their babies, but consider which drugs can be used safely. Patients developing primary toxoplasma infection, even if apparently in the eye alone, must be made aware that this is a systemic infection which carries a serious risk of fetal involvement, the extent of which depends on the stage of the pregnancy.

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HIV

HIV

Background The ocular manifestations of HIV vary with the level of immunocompromise, reflected in the presence of HIV virus in the blood (viral load) and CD4 + count. A CD4 + count >1000 cells/mL is normal; < 50 cells/μL is extremely low with a high risk of serious ocular infection, particularly cytomegalovirus (CMV) retinitis. Highly active antiretroviral therapy (HAART) markedly improves CD4 + counts and reduces opportunistic infections, but lymphoma has become more common.

History and examination Ask about systemic illnesses, infections, interventions, medications, and duration of HIV infection. A minimum routine examination includes VA, eyelids, conjunctival fornices, anterior segment, and dilated fundoscopy including peripheral retina. Examination specifically to exclude uveitis and CMV retinitis.

Clinical features The following are associated with HIV infection:

Blepharitis : often severe.

Molluscum contagiosum : usually extensive.

Premature presbyopia.

Conjunctival Kaposi’s sarcoma : signifies AIDS.

Conjunctival squamous cell carcinoma : more common in Africa.

Herpes zoster ophthalmicus : a common presenting feature of AIDS in developing nations.

HIV retinopathy : cotton-wool spots and dot haemorrhages are common and inconsequential (Fig. 8.18).

Uveitis : can be of infective, inflammatory, or malignant aetiology. Improved immunity following antiretroviral therapy

may cause an immune recovery uveitis that is a leading cause of new visual loss. As the CD4 + count drops, infections become increasingly important, particularly syphilis, toxoplasmosis, herpes simplex, and herpes zoster. HIV itself can cause a panuveitis. Lymphoma may cause a masquerade-

type of uveitis that may have CNS or systemic involvement. Syphilitic uveitis can occur at any CD4 + count and in the presence of HIV may indicate tertiary syphillis.

CMV and varicella-zoster retinitis (previously called PORN or

374

progressive outer retinal necrosis): both CMV retinitis and

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Fig. 8.18: HIV retinopathy.

Fig. 8.19: Varicella-zoster retinitis (progressive outer retinal necrosis).

PORN can occur with little or no intraocular inflammation (a useful diagnostic clue). PORN (Fig. 8.19) tends to be more aggressive than acute retinal necrosis (ARN) that occurs in immunocompetent patients.

Toxoplasmosis : retinal lesions may be bilateral and multifocal;

 

56% have CNS involvement.

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HIV

Metastatic bacterial or fungal endophthalmitis : particularly

following sepsis, surgery, or intravenous drugs use.

 

Tuberculosis : increasing incidence in the HIV population.

 

Ocular manifestations include uveitis and optic nerve

 

involvement. Choroidal granulomas are an indication of miliary

 

TB.

 

Pneumocystis choroiditis : implies systemic infection and

 

requires systemic treatment. No additional ocular treatment is

 

required. Pneumocystis very rarely causes conjunctivitis,

 

orbital masses, and optic neuropathy.

 

Retrobulbar neuritis : due to Cryptococcus, syphilis,

 

histoplasmosis, or herpes zoster.

 

Toxic optic neuropathy : from drug therapy, especially

 

dideoxyinosine and ethambutol.

 

Papillitis : secondary to CMV, syphilis, or toxoplasmosis.

 

Cortical visual loss : causes include non-Hodgkin’s lymphoma,

 

toxoplasmosis, and progressive multifocal

 

leucoencephalopathy.

 

Cryptococcal meningitis : may cause visual loss through raised

 

intracranial pressure. Optic nerve sheath fenestration may

 

help.

 

Reiter’s disease : comprises a triad of urethritis, arthritis, and

 

conjunctivitis. Treat conjunctivitis with topical steroids. Add

 

antibiotics if it looks infective.

 

Follow up In patients with CD4 + counts < 50 cells/μL,

 

perform dilated fundoscopy every 6 weeks or earlier if they

 

develop floaters, field loss, or blurred vision. The need for regular

 

screening decreases with CD4 + counts >200 cells/μL.

 

 

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Episcleritis

Background A self-limiting condition of unknown aetiology.

Clinical features Episcleritis is often mistaken for scleritis. In episcleritis, congestion is limited to the radially orientated superficial episcleral vessels within Tenon’s capsule (Fig. 8.20). In anterior scleritis, the irregular-crossing deep episcleral vessels on the scleral surface are also involved and there is accompanying scleral thickening. G. phenylephrine 10% only produces blanching of the superficial episcleral venous plexus. The onset of episcleritis is typically more acute, the sensation is not of pain but grittiness, there is no corneal or intraocular involvement, and vision is unaffected.

Management Offer reassurance. Treatment is not routinely required as symptoms usually resolve. If frequently recurrent, oral NSAIDs such as Froben 50 mg p.o. t.d.s. may help but have side effects (gastric/duodenal ulceration, exacerbation of asthma). Topical steroids are sometimes useful, e.g. fluorometholone 0.1% q.d.s. 2 weeks.

Follow up Not required, but explain that recurrences are common.

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Fig. 8.20: Mild episcleritis.

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Scleritis

Scleritis

Background Less common than episcleritis. Scleritis has frequent (50%) systemic associations and is vision threatening. Systemic therapy is essential. Broadly classified into anterior and posterior scleritis.

Anterior scleritis Has a subacute onset of severe ocular and periocular pain, often worse at night, with redness, photophobia, and globe tenderness (Fig. 8.21). Deep episcleral vessels on the surface of the sclera are dilated with vascular closure in necrotizing disease. The sclera may have a bluish tint that is best appreciated with the naked eye and bright room light. Avascular areas are best seen with red-free (green) slit lamp light. May be diffuse, nodular, or necrotizing.

A

B

Fig. 8.21: Scleritis. Mild anterior diffuse scleritis (A) and necrotising anterior scleritis (B) with uveal tissue

378 visible beneath thinned sclera.

Diffuse

a.May involve small or large areas of sclera.

b.The most common type of scleritis.

c.Pain may be severe.

d. Not usually sight threatening and usually resolves.

Nodular

a.Part of the inflamed sclera is raised in one or more nodules.

b.May progress.

c.Commonly recurs.

Necrotising

a.Least frequent but most severe type of anterior scleritis.

b.Female preponderance.

c.Associated with vascular closure and pale regions of scleral necrosis within areas of active scleritis.

d. Scleromalacia perforans is a very rare form of necrotizing anterior scleritis which occurs without inflammation, is asymptomatic, bilateral, and seen only in advanced rheumatoid arthritis (RA), usually in females. Perforation of the thinned and translucent sclera is unusual without trauma. There is no ocular treatment.

Associations

a.Local : ocular surgery, infection (toxoplasmosis, acanthamoeba, herpes simplex virus, varicella-zoster virus, bacterial), and masquerade syndrome (ocular surface carcinoma, intraocular tumour).

b.Systemic (50%): most commonly RA but also other collagen/vascular disorders including Wegener’s granulomatosis, polyarteritis nodosa (PAN), systemic lupus erythematosus (SLE).

Posterior scleritis May be nodular or diffuse. Presentation is with pain and reduced vision. Rarely, there is conjunctival chemosis with some limitation of movement and proptosis. Posterior segment signs are variable and may include serous retinal detachment, swollen optic disc, subretinal mass lesion, choroidal folds, and choroidal detachment. Approximately one-third of patients have associated anterior scleritis. Local associations are as for anterior scleritis. Systemic associations (30%) include

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Scleritis

RA, Wegener’s granulomatosis, PAN, SLE, relapsing polychondritis, and neoplasia.

Differential diagnosis Episcleritis may overlay scleritis, so it is important not to miss the scleritis. For distinguishing features, see page 377.

Investigations There is a high incidence of systemic associations. If there is a suggestive history and general examination arrange urine dipstick for blood and protein, FBC, ESR, CRP, RhF, ANA, U&E, LFTs, and ANCA (if Wegener’s granulomatosis suspected). Discuss with physicians. In many cases the diagnosis is already established. In patients with suspected posterior scleritis, B-scan ultrasonography is very useful in demonstrating either diffuse or nodular scleral thickening (>2 mm) with an adjacent echolucent area from oedema in Tenon’s space. Fluorescein angiography in patients with serous retinal detachment typically shows multiple small foci of leakage at the level of the RPE which may look similar to Vogt-Koyanagi- Harada syndrome.

Management

Casualty : Treat anterior non-necrotizing scleritis, and mild posterior scleritis with no visible posterior segment abnormalities with Froben 100 mg p.o. t.d.s. (combined with omeprazole 20 mg p.o. o.d. if there is a history/risk of gastric/duodenal ulceration). Review in 1–2 weeks. Reduced pain indicates a good response, and treatment can then be tailed off, but warn of the risk of recurrence. If pain and inflammation persist, refer to clinic and consider oral prednisolone 0.5–1 mg/kg o.d., then tapered off. Necrotizing scleritis, and posterior scleritis associated with retinal or choroidal detachment, disc swelling, or subretinal mass lesion, require same-day specialist review. If there is hypertension, protein or blood on urine dipstick, or associated systemic disease, then consult urgently with physicians; systemic disease is potentially life threatening.

Clinic : High-dose oral steroids (prednisolone 1 mg/kg) are recommended for necrotizing anterior scleritis and posterior scleritis with visual loss or threat. Consider admission and pulsed i.v. methylprednisolone 0.5–1.0 g for 1–3 days followed by oral steroids in severe cases. Adjunct immunosuppressive therapy is often required for those with systemic disease or when scleritis is resistant to steroids alone. Cyclophosphamide may be necessary in very severe necrotizing disease, as may occur in Wegener’s granulomatosis. Surgical treatment may be required either for diagnostic (infection, masquerade syndrome)

380 or therapeutic (cataract, corneal/scleral grafts) indications.

Ideally, surgery is undertaken when scleritis has been controlled medically and under corticosteroid cover (prednisolone 40 mg p.o. o.d. 2 weeks preoperatively). Clear corneal incisions are less likely to require high-dose steroid cover than scleral tunnels.

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Endophthalmitis

Endophthalmitis

Background Endophthalmitis is vitreous and/or anterior chamber inflammation of presumed infective origin. Classified as exogenous (after surgery, keratitis, or trauma) or endogenous (also called metastatic).

Exogenous Postoperative

Endophthalmitis

Clinical features

Acute onset (0–17 days): ocular pain, reduced vision and headache with lid oedema, conjunctival hyperaemia, chemosis, purulent discharge, corneal oedema, anterior chamber reaction, hypopyon, and poor red reflex.

Delayed acute (18–60 days): due to less virulent organisms or from a suture track, wound dehiscence, or vitreous wick.

Chronic (>60 days): usually a capsular bag infection with a low-virulence organism (often Propionibacterium acnes or Staphylococcus epidermidis ). Presents with reduced vision and minimal pain. May have signs of steroid-responsive iritis, capsular plaque, granulomatous uveitis, or localized vitritis. Hypopyon uveitis following YAG capsulotomy is a classic presentation. Endophthalmitis may occur years after trabeculectomy (p. 323).

History and examination Assess severity, which relates to the speed of onset, VA at presentation, RAPD, and fundal view. Measure the hypopyon height.

Differential diagnosis Assume all severe postoperative inflammation is infective endophthalmitis unless there is another obvious cause, especially in patients with no history of uveitis. If in doubt and VA is 6/12 or worse, admit for 6–12 hours of halfhourly, topical, preservative-free dexamethasone 0.1% and reassess 2 hourly.

Investigations Lid and conjunctival swabs are of no value. Take a corneal scrape if keratitis is present. Arrange aqueous and vitreous samples in all patients (including those with perception of light vision at presentation) (Box 8.1). If there is no fundal view, arrange ultrasound prior to sampling. If not immediately available, proceed with biopsy but arrange ultrasound at the first opportunity. Request baseline FBC, U&E, weight, blood sugar, and blood

382 pressure measurements before starting on systemic steroids.