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116

4 Clinical Considerations of Episcleritis and Scleritis

 

 

Fig. 4.23 Posterior scleritis with annular retinochoroidal and serous retinal detachment

Fig. 4.24 Same eye as shown in Fig. 4.23. Fluorescein angiogram, conÞrming the Þndings described in Fig. 4.23

head down; despite a cloudy subretinal ßuid, a pale gray subretinal mass with a surrounding dark gray line and an overlying normal choroidal vascular pattern may sometimes be visible through the poorly mobile bullous serous retinal detachment. The bullous serous retinal detachments do not have retinal holes or Þxed retinal folds. The ciliochoroidal or retinal detachments usually resolve completely with prompt and aggressive treatment of the scleritis. Although they may disappear within hours, they are often absorbed slowly over a period of several weeks or months, leaving only a diffuse pigmentation in the affected area; however, if the macular area has been

involved, loss of vision will remain as a permanent sequela [5].

Associated Diseases

Disease association in posterior scleritis is less common than in anterior scleritis [2, 7]. In our series of 31 patients with posterior scleritis, 6 (19%) were found to have an associated systemic disease (Table 4.4). This is in contrast to the 36% disease association found in our patients with anterior scleritis. Diagnoses included psoriatic arthritis in two patients, HLA-B27+ (without spondyloarthropathy)-associated scleritis in one patient, arthritis and inßammatory bowel disease in one patient, systemic lupus erythematosus in one patient, and tubulointerstitial nephritis and uveitis syndrome in one patient (Table 4.5).

Complications

Posterior uveitis is universally present in posterior scleral inßammation because the choroid is always involved by the adjacent posterior scleral inßammation; [2, 6] anterior uveitis may also appear, but it is often associated with concomitant anterior scleritis. Glaucoma, particularly in combination with uveitis, is considered to be an ominous sign in the course of scleritis because its presence indicates a more diffuse and severe process [3]. Whether caused by anterior uveitis, ciliochoroidal detachment, angle neovascularization, or chronic use of steroids, increased intraocular pressure in posterior scleritis may result in the development of irreversible optic nerve damage [9, 100].

Ancillary Tests

Ultrasonography

Ultrasonography is the most useful test in the diagnosis of posterior scleritis because it shows the ßattening and thickening of the posterior coats of the eye (choroid and sclera) associated with retrobulbar edema [84, 86, 89, 101] (Fig. 4.25). Occasionally, retinal and choroidal detachments may also be detected. B-scan ultrasonography shows multiple internal echoes in the area of the scleral thickening and lack of echoes in the area of retrobulbar edema; the multiple echoes remain after sound beam attenuation, indicating high

4.2 Scleritis

117

 

 

Fig. 4.25 B scan ultrasonogram with superimposed A scan proÞle. Note the thickening of the retinal choroid layer and the edema in TenonÕs space

Fig. 4.26 Ultrasonogram of a patient with posterior scleritis. Note particularly the A scan tracing showing the multiple retrobulbar echoes. The multiple echoes in the area of the sclera indicate high internal reßectivity of the sclera

Fig. 4.27 Ultrasonogram of choroidal thickening in a patient with chronic uveitis without scleritis. Note particularly the A scan tracing showing a lack of high internal reßectivity in the area of the sclera with sound beam attenuation

Fig. 4.28 Ultrasonogram ÒTÓ sign formed by the sonagraphically, empty space occupied by the optic nerve and the edematous TenonÕs space adjacent to the optic nerve

internal reßectivity of the scleral mass (Fig. 4.26). On the other hand, in choroidal thickening without scleral thickening, the echoes do not remain after sound beam attenuation (low internal reßectivity) (Fig. 4.27). When retrobulbar edema surrounds the optic nerve, the ÒTÓ sign may appear, which consists of a lack of echoes in the edematous TenonÕs space and adjacent optic nerve (Fig. 4.28). A-scan ultrasonography shows highamplitude internal spikes in the area of the scleral thickening and low-amplitude internal spikes in the area of retrobulbar edema. The combination of both A scan and B scan techniques gives the most useful results in distinguishing posterior scleritis

from orbital, choroidal, and retinal entities that clinically may mimic it (Tables 4.7Ð4.9) [86].

Computerized Tomography (CT) Scanning

Computerized tomography also shows scleral thickening, the image of which can be enhanced by radiopaque medium injection [86, 102]. Retrobulbar edema may also be seen. The ability of computerized tomography to delineate extraocular muscles, lacrimal glands, optic nerves, scleral coats, orbital walls, and paranasal tissues helps to detect extraocular muscle or lacrimal gland enlargement, optic nerve or scleral inßammation, bone erosion, and sinus involvement,

Table 4.7 Differential diagnosis of posterior scleritis: proptosis, chemosis, lid swelling, and limitation of ocular movements

 

 

 

Acute diffuse idiopathic orbital

 

Parameter

Posterior scleritis

Orbital tumor

inßammation

Thyroid ophthalmopathy

Sex predilection

Female

Ð

Ð

Female

 

 

 

 

 

Age predilection

Middle aged and elderly

Ð

Ð

Middle aged and elderly

 

 

 

 

 

Laterality

Unilateral

Unilateral

Unilateral

Bilateral

Onset

Gradual

Gradual

Acute

Gradual

 

 

 

 

 

Pain

Variable

±

Variable

 

 

 

 

 

Tenderness

+

+

 

 

 

 

 

Anterior scleritis

+

Visual loss

+

±

±

Variable

 

 

 

 

 

Fundus mass

Variable

±

±

 

 

 

 

 

Color of the mass

Orange

Orange

Orange

Proptosis

±

+

+

+

Motility disturbance

±

+

+

+

 

 

 

 

 

Conjunctival chemosis

±

+

+

 

 

 

 

 

Lid edema

±

+

+

Pigment epithelium

Yellowish nodules

Normal

Normal

Normal

 

 

 

 

 

Disk edema

+

±

±

±

 

 

 

 

 

Uveitis

+

±

 

 

 

 

 

Choroidal folds

+

±

±

±

Serous retinal detachment

+

±

 

 

 

 

 

Fluorescein angiography

Multiple small leaks

Normal

Normal

Normal

(other than choroidal folds)

 

 

 

 

 

 

 

 

 

Ultrasound

Scleral and choroid thickening

Orbital mass

Orbital mass (low reßectivity)

EOM enlargement

 

retrobulbar edema (high reßectivity)

 

and/or EOM enlargement

 

 

 

 

 

 

CT scan

Scleral and choroid thickening

Orbital mass with sinus

Orbital mass without sinus involvement

EOM enlargement

 

 

involvement/bone erosion

or bone erosion; EOM enlargement

 

 

 

 

 

 

Biopsy indication

No biopsy

Biopsy

Biopsy

No biopsy

 

 

 

 

 

Response to steroids

Good

Absent

Very good

Variable

EOM extraocular muscle

 

 

 

 

118

Scleritis and Episcleritis of Considerations Clinical 4

Table 4.8 Differential diagnosis of posterior scleritis: subretinal mass

Parameter

Posterior scleritis

Choroidal melanoma

Metastatic uveal carcinoma

Choroidal hemangioma

Sex predilection

Female

Ð

Ð

Ð

 

 

 

 

 

Age predilection

Middle aged and elderly

Elderly

Middle aged and elderly

Middle aged and elderly

 

 

 

 

 

Laterality

Unilateral

Unilateral

Unilateral

Unilateral

Onset

Gradual

Gradual

Gradual

Gradual

 

 

 

 

 

Pain

Variable

Ð

Ð

Ð

 

 

 

 

 

Tenderness

+

Anterior scleritis

+

Visual loss

+

Variable

Variable

Variable

 

 

 

 

 

Color of the mass

Orange

Hyperor hypo-pigmented

Hypopigmented

Pinkish orange

 

 

 

 

 

Overlying retina

Yellow deposits

Orange pigment

Dark mottling

Cystoid edema

Proptosis

±

 

 

 

 

 

Motility disturbance

±

 

 

 

 

 

Conjunctival chemosis

±

 

 

 

 

 

Lid edema

±

Disk edema

+

 

 

 

 

 

Uveitis

+

 

 

 

 

 

Choroidal folds

+

±

±

Serous retinal detachment/

+/cloudy

+/clear

+/clear

+/clear

subretinal ßuid

 

 

 

 

 

 

 

 

 

Fluorescein angiography

Small leaks; intrinsic vasculature

Small leaks

Small leaks

Small leaks; early ßuorescence

(other than choroidal folds)

 

 

 

prior to Þlling retinal vessels

 

 

 

 

 

Ultrasound

Scleral and choroid thickening

Choroidal mass

Choroidal mass

Choroidal mass (high reßectivity);

 

(high reßectivity) retrobulbar edema

(low reßectivity);

(moderate reßectivity);

no retrobulbar edema

 

 

no retrobulbar edema

no retrobulbar edema

 

 

 

 

 

 

Response to steroids

Good

Absent

Absent

Absent

 

 

 

 

 

Scleritis 2.4

119

Table 4.9 Differential diagnosis of posterior scleritis: serous detachment of choroid, ciliary body, and retina

 

 

 

 

Idiopathic centralserous

Parameter

Posterior scleritis

Uveal effusion syndrome

VogtÐKoyanagiÐHarada syndrome

chorio retinopathy

 

 

 

 

 

Sex predilection

Female

Male

Ð

Male

Age predilection

Middle aged and elderly

Middle aged

Young and middle aged

Middle aged

Race predilection

Ð

Ð

Oriental and pigmented

Caucasian

 

 

 

 

 

Laterality

Unilateral

Bilateral

Bilateral

Unilateral

 

 

 

 

 

Pain

Variable

− (photophobia)

Anterior scleritis

+

 

 

 

 

 

Uveitis

+

+

 

 

 

 

 

Disk edema

+

+

+

 

 

 

 

 

Pigment epithelium

Yellowish nodules

ÒLeopard spotsÓ

Depigmented or

Serous detachments

 

 

 

hyperpigmented lines

pigment epithelium

 

 

 

 

 

Serous retinal detachment

+

+

+

+

Serous ciliochoroidal

+

+

±

detachment

 

 

 

 

Subretinal ßuid

Cloudy

Clear

Cloudy

Clear

 

 

 

 

 

Fluorescein angiography

Multiple small leaks

Slow choroidal perfusion;

Multiple small leaks; late-staining

Serous detachments

 

 

occasional leaks

subretinal ßuid

pigment epithelium

Ultrasound

Scleral and choroidal thickening (high

Choroid thickened; serous

Choroid thickened (low internal

Serous retinal

 

reßectivity); retrobulbar edema; serous

cilio-choroid and retinal

reßectivity) Serous retinal

detachment

 

ciliochoroid and retinal detachment

detachment

detachment

 

 

 

 

 

 

Miscellaneous

Collagen vascular disease association

High protein level in CSF

Headaches, fever dysacousis,

Anxiety

 

 

(50% of cases)

vitiligo, meningism (50% of cases)

 

 

 

 

 

 

120

Scleritis and Episcleritis of Considerations Clinical 4

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