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332UVEITIS

51.Name major categories of alternate immunosuppressives.

&Antimetabolites (e.g., methotrexate): Often used for their steroid-sparing effect

&T-cell inhibitors (e.g., cyclosporine)

&Alkylating agents (e.g., cyclophosphamide): Typically reserved for severe, sight-threatening uveitis not adequately responsive to aforementioned agents

&Biologicals (e.g., infliximab): Tumor necrosis factor (TNF) inhibitors are one example of this expanding arsenal of immunosuppressant and anti-inflammatory agents

Jabs DA, Rosenbaum JT, Foster CS, et al. Guidelines for the use of immunosuppressive drugs in patients with ocular inflammatory disorders: recommendations of an expert panel. Am J Ophthalmol 130:492–513, 2000.

MASQUERADE SYNDROMES

52.Define masquerade syndrome.

The term masquerade syndrome refers to ophthalmic disorders that are not primarily inflammatory in nature but may present clinically as either anterior or posterior uveitis

(Table 39-5). These entities may be mistaken for, or masquerade as, primary uveitis. Extensive evaluation is often initiated because patients manifest with atypical features, recurrent episodes of uveitis, or uveitis that is unresponsive to standard therapy.

KEY POINTS: COMMON MASQUERADE SYNDROMES

1.Retinoblastoma in children

2.Leukemia in children

3.Primary intraocular lymphoma in the elderly

4.Ocular ischemic syndrome in the elderly

5.Peripheral retinal detachment in any age group

53.In what age groups should one have the highest suspicion for masquerade syndromes?

In the very young and in the elderly.

54.Describe the clinical features of retinoblastoma.

Retinoblastoma is the most common primary intraocular malignancy in

children, usually presenting before age

 

2. The most common signs are

 

leukocoria (white pupillary reflex) and

 

strabismus. Occasionally, tumor

 

necrosis may produce significant

 

inflammation. Tumor cells layered in the

 

anterior chamber may produce a

 

 

 

 

pseudohypopyon (Fig. 39-2).

Figure 39-2. Pseudohypopyon caused by seeding

Retinoblastoma cells may enter the

of retinoblastoma cells in the anterior chamber.

vitreous, as vitreous seeds, and

(From Shields JA, Shields CL: Intraocular Tumors:

simulate vitritis. Calcification on

A Text and Atlas. Philadelphia, W.B. Saunders,

ultrasonography and CT scan may help

1992.)

 

TABLE 39-5. M O S T C O M M O N M A S Q U E R A D E S Y N D R O M E S T H A T M A Y M I M I C U V E I T I S

Disease

Location

Age (yr)

Signs of Inflammation

Diagnostic Tests

 

 

 

 

 

Retinoblastoma

Anterior

<15

Flare, cells, pseudohypopyon

Aqueous tap for LDH levels and cytology

Leukemia

Anterior

<15

Flare, cells, heterochromia

Bone marrow, peripheral blood smear, aqueous cytology

Intraocular foreign body

Anterior

Any age

Flare, cells

X-ray, ultrasound, CT scan

Malignant melanoma

Anterior

Any age

Flare, cells

Angiography (fluorescein, ICG), ultrasound, MRI

Ocular ischemic

Anterior

50þ

Cell, flare, redness

IVFA, carotid Doppler

syndrome

 

 

 

 

Peripheral retinal

Anterior

Any age

Flare, cells

Ophthalmoscopy, ultrasound

detachment

 

 

 

 

Retinitis pigmentosa

Posterior

Any age

Cells in vitreous

ERG, EOG, visual fields

Primary intraocular

Posterior

15þ

Vitreous cells, retinal

Cytology of aqueous/vitreous fluid

lymphoma

 

 

hemorrhage or exudates,

 

 

 

 

RPE infiltrates

 

Lymphoma

Posterior

15þ

Retinal hemorrhage, exudates,

Biopsy of lymph node/bone marrow, physical examination

 

 

 

vitreous cells

 

Retinoblastoma

Posterior

<15

Vitreous cells, retinal exudate

Ultrasound, aqueous tap

Malignant melanoma

Posterior

15þ

Vitreous cells

Fluorescein ultrasound

CT ¼ computed tomography, ERG ¼ electroretinogram, EOG ¼ electro-oculogram, ICG ¼ indocyanine green, IVFA ¼ fluorescein angiography, LDH ¼ lactate dehydrogenase, MRI ¼ magnetic resonance imaging, RPE ¼ retinal pigment epithelium.

Adapted from American Academy of Ophthalmology: Ophthalmology Basic and Clinical Science Course, Section 6. San Francisco, American Academy of Ophthalmology, 1997.

UVEITIS

333

Figure 39-4. Leukemic infiltration of the optic nerve head, retina, and choroid in an 8-year-old child. (From Shields JA, Shields CL: Intraocular Tumors: A Text and Atlas. Philadelphia, W.B. Saunders, 1992.)
Figure 39-3. Yellow-white chorioretinal infiltrates in intraocular lymphoma. (From Shields JA, Shields CL: Intraocular Tumors: A Text and Atlas. Philadelphia, W.B. Saunders, 1992.)

334 UVEITIS

to differentiate retinoblastoma from various forms of childhood uveitis, including toxoplasmosis, toxocariasis, cysticercosis, and pars planitis.

Shields JA, Augsburger JJ: Current approaches to the diagnosis and management of retinoblastoma. Surv Ophthalmol 25:347–372, 1981.

55.What may present with chronic steroid-resistant panuveitis in a patient older than age 50?

Primary intraocular lymphoma, formerly known as reticulum cell sarcoma (Fig. 39-3). Elderly individuals may present with bilateral vitreous cells, anterior chamber reaction, and retinal and choroidal infiltrates. The retinal infiltrates may be patchy and

associated with hemorrhage and exudate. Dense vitritis may be the only presenting sign. Most patients eventually develop some form of CNS involvement. CT or magnetic resonance imaging (MRI) may demonstrate CNS

tumors. Vitreous aspirate or lumbar puncture may establish the diagnosis. Therapy may include ocular and CNS irradiation combined with intrathecal chemotherapy.

Char DH, Ljung BM, Miller T, Phillips T: Primary intraocular lymphoma (ocular reticulum cell sarcoma): Diagnosis and management. Ophthalmology 95:625–630, 1988.

56.Describe the ocular findings associated with leukemia.

Clinically, the retina is the most commonly affected (Fig. 39-4). Retinal vascular dilation and tortuosity, hemorrhages, cotton-wool spots, and peripheral neovascularization occur. Roth spots are hemorrhages with white centers composed of leukemic cells or platelet-fibrin aggregates. Histopathologically, the choroid is the most commonly involved and may cause exudative retinal detachment. IVFA demonstrates multiple areas of hyperfluorescence similar to that found

in VKH syndrome. Anterior segment findings include conjunctival mass, iris heterochromia, anterior chamber cell and flare, pseudohypopyon, spontaneous hyphema, and elevated

intraocular pressure. Optic nerve infiltration and orbital involvement are common.

Kincaid MC, Green WR: Ocular and orbital involvement in leukemia. Surv Ophthalmol 27:211–232, 1983.

57.How can a malignant melanoma produce inflammatory signs?

Necrotic tumors may elicit an intense inflammatory response associated with seeding of tumor cells into the vitreous cavity and anterior segment. Occasionally, melanophages or tumor cells that contain melanin produce a brown pseudohypopyon. Blockage of the