Sclera and Episclera Flashcards
Describe the anatomy to the episcelra
The very outer layer of the sclera
Dense, vascular CT.
Provides nutrition to sclera
Mscular fusion (syonival membrane) IE connected to Tenon’s capsule
Describe the vasculature of the episclera
Superficial episcleral plexus and deep episcleral plexus
What is episcleritis?
Benign, transient, sudden onset inflammation of episclera
What layers of episclera are involved?
Conjunctival vessels, deep and superficial scleral plexus (Tenon’s vessels)
What are symptoms of episcleritis?
Acute onset of redness
Mild pain but generally NOT painful
Can be recurrent
No discharge
What are signs of episcleritis?
Sectorial (less commonly diffuse) redness of one or both eyes
Mild tenderness over area of episcleral injection
Nodule that is somewhat mobile
Anterior uveitis/corneal involvement is rare
VA normal.
What sort of history should you check for in episcleritis?
30% associated systemic disease, CT disease, atopy, rosacea, gout, herpes, syphillis, rheumatoid arthritis
Tend to be women more than men
History of rash, arthritis, venereal disease, recent viral ilness or medical problems
What are some lab tests to do to check for Episcleritis?
ANA, rhemuatoid factor, ESR, serum uric acid level, RPR, FTA-ABS
What’s the Dx for Scleritis?
A deep pain that’s severe and radiates to ipsilateral face
What’s the Dx for Iritis?
Cells and flare in the anterior chamber
What’s the Dx for Conjunctivitis?
Discharge and inferior tarsal conjunctival follicles/papillae
Describe some noninfectious etiologies of episcleritis
Idiopathic CT disease (RA, polyarteritis nodosa, systemic lupus erthematosus, Wegener granulomatosis) Gout (Increased serum uric acid) Inflammatory bowel disease Rosacea/Atopy Thyroid disease
Describe some infectious etiologies of episceleritis
Herpes zoster ivrus Herpes simplex virus Lyme disease Suphilis (FTA-ABS +) Hepatitis B
Describe some of the work up for episcleritis
External evaluation - Look for a bluish hue of scleritis
SLE (Lupus) - Conj/sclera, cornea, AC, check IOP. Anesthetisze and move conj to determine depth of injection)
Lab tests if history suggests it (ANA - antinuclear antibody, Rheumatoid factor, ESR (Erythrocyte sedimentation rate), Serum uric acid level, FTA-ABS (Syphilis))
What effect does 2.5% phenylephrine have on episcleritis?
Blanches the episcleral vessels (makes them clear/white) after 15 minutes and clears up some redness
Name the classifications of episcleritis
Simple - Sectorial/Diffuse
Nodular
Of the classifications, which is the most common kind of episcleritis?
Simple (78-83%)
Describe Simple Episcleritis
Generalized, moderate episcleral swelling and injection
See greyish infiltrates and will resolve in first 3 weeks half the time
Describe Nodular episcleritis
See a nodule of localized edema within area of injection (Red bump in the red patch)
Single or multiple nodules
MOVABLE nodule over deep episcleral plexus
Takes longer to resolve than simple episcleritis
How do you treat episcleritis?
SELF LIMITING, No treatment required. Treatment is for patient peace of mind
Mild - Chilled artificial tears, decongestants and cold compresses topically
Moderate - Mild topical steroid (Loteprednol or flurometholone) with tapering
Oral NSAID/ASA ok too
Describe how a follow up for episcleritis would go
If on steroids, check weekly and do IOP
If on artificial tears/vasoconstrictors, check in 2-3 weeks
Describe Chronic/Stubborn Episcleritis
Rare, nodule formation,scleral thinning possible (not necrosis), trasparency and ‘bluish’ color
How do you treat chronic episcleritis?
NSAIDs, oral steroids (better option)
Can a chronic episcleritis become scleritis?
No
Describe the anatomy of the sclera
Collage and elastic bundles, a firm and flexible protective layer.
Rich nerve sypply via long ciliary nerves
AVASCULAR
Low metabolism supplied by choroid and episclera
Fully hydrated
Continuous with corneal stroma
Highlighting anatomy - What supplies the metabolic needs of sclera?
Choroid and episclera
Is the sclera vascular?
No
What is the sclera continuous with?
Corneal stroma
What is scleritis?
Uncommon CHRONIC granulomatous inflammatory disease of sclera, both anterior or posterior.
Inflammation affects deep episcleral plexus, (choroidal vasculature) gives it a dark red with blue tint
Can blind patients and half tend to be associated with underlying disease, CT disease or trauma/infection
Describe anterior scleritis
Non-nectorizing (85%). VA ok unless uveitis occurs. Patient presents with redness of eye and SEVERE eye pain
Describe posterior scleritis
Scleritis without pain or redness. Can have EOM restriction, proptosis and permenant decreased VA.
May see an amelanotic choroidal mass (no color)
Usually unrelated to systemic disease
What age and population tend to be most affected by scleritis?
40-60 years and females>males 8 to 5
What kind of complications can arise from scleritis affecting the choroidal vasculature?
Intraocular complications - uveitis, retinitis/retinal detachment, glaucoma, CAT, cornea (peripheral keratitis, limbal guttering)
Describe some symptoms for scleritis
SEVERE OCULAR PAIN (Deep boring radiating pain that can wake from sleep, goes from temple, brow, jaw or sinus)
Gradual onset red eye with decreasing VA
Recurrent episodes
Scleromalacia perforans (holes in sclera) but may have minimal symptoms
Describe some signs of scleritis
Inflammed sclera, episcleral or conjunctival vessels
Injection of vessels, giving sclera/conj a red look
Sclera has bluish hue (Best seen in natural light) - Sclera may be thing or edematous
Photophobia or tearing of sclera
What is the Dx for episcleritis
Sclera not involved, blood vessels blanch with topical phenylephrine
More acute onset than scleritis
Patient will be younger generally speaking
Mild to no symptoms
What are some systemic causes for scleritis?
50% of cases associated with systemic diseases: Collagen diseases Metabolic diseases Granulomatous disease Infectious diseases Ocular diseases
What are and describe the two kinds of anterior scleritis
Diffuse - Widespread inflammation of anterior sclera that is most common kind and most benign (no progression)
Nodular - 1+ erythematous (red patch) with immovable, tender inflamed nodules on anterior sclera. 20% become necrotizing and takes up to 8 weeks to clear
How do you treat diffuse/nodular anterior scleritis?
Oral NSAIDs
Oral prednisone with slow tapering
Immunosuppressive therapy –> systemic steroids used with NSAIDs
When selecting oral NSAIDs to treat anterior scleritis, how would you know the treatment was not working?
Prescribing three different NSAIDs and all must fail before calling treatment failed
Describe anterior necrotizing scleritis
Most severe form of scleritis and has vision threatening complications (permanent)
Severe pain, damage to sclera is significant and becomes transparent
See a necrotic/avascular patch and can have conjunctival perforation
What class of systemic conditions can cause anterior necrotizing scleritis with inflammation? (Give example)
Systemic collagen vascular disorders like Rheumatoid arthritis
Describe the ophthalmic emergency that can occur with scleritis
Anterior necrotizing scleritis with inflammation
Sclera thins and is blue
Gradual extreme painful red eye
Can have associated corneal inflammation
What is corneal inflammation caused by scleritis called?
Sclerokeratitis
What are some secondary complications with anterior necrotizing scleritis with inflammation?
Sclerosing keratitis Cataract Hyphema Retina involvement (Staphyloma, ectasia) Secondary glaucoma
What is Ectasia?
Bulging of sclera without a uveal lining
What is Staphyloma?
Localized thinning of sclera with bulging of uvea into thinned/stretched area of sclera
Are named on location
Scleritis, myopia, RD, or CT disease can be involved
Check with a BIO exam and A/B scan
What is another name for scleromalacia perforans?
Anterior necrotizing scleritis WITHOUT inflammation
Describe scleromalacia perforans
Frequently in patients with long standing RA –> Formation of a rheumatoid nodule in sclera
NO PAIN
Visible avascular patch
Thinning scleral tissue that necrotizes
Steady progression
Perforation rate is high unless the IOP is high
How do you treat necrotizing scleritis?
Oral Prednisone
Immunosuppressive agents (cyclophosphamide, methotrexate, azathiprine, cyclosporin)
Abundant lubrication for scleromalacia perforans
Severe cases require scleral patch graft surgery to repair damaged corneal tissue
What is the Dx for posterior scleritis?
Retrobulbar optic neuritis
Retinal detachment
Tumor
Orbital disease
What are some signs of posterior scleritis?
Optic disc swelling Macular edema Retinal hemorrhage Retinal detachment Vitritis Choroidal folds/detachment Intraretinal white deposits
How do you treat a posterior scleritis?
Controversial, not a lot of consensus ASA NSAIDs Steroids Immunosuppressive treatment
In general, what is the treatment mentality when treating scleritis: Infectious etiologies
Use of topical/systemic antibodies
In general, what is the treatment mentality when treating scleritis: Foreign body
Remove it
In general, what is the treatment mentality when treating scleritis: What else to consider?
Glasses/eye shielding at all times if there is significant thinning/risk of perforation
Topical steroids are NOT effective
What is contraindicated in treated scleritis?
Subconjunctival steroids, especially in necrotizing scleritis –> increased scleral thinning/perforation
Describe the work up for scleritis
History - previous episodes? Systemic diseases?
Examine sclera in all directions via gross inspection in natural/adequate room light
Slit lamp exam with red-free filter to check if there are avascular zones
DFE to rule out posterior involvement
COMPLETE physical exam (CBC, ESR, uric acid, syphilis, rheumatoid factor, ANA, FBS (fasting blood sugar), PPD, radiograph of sacroiliac joints)
How would you differentiate episcleritis and scleritis?
Conjunctival manipulation (vessels move with episcleritis) OTC decongestants (should clear up with episcleritis) 10% phenylephrine (blanching vessels with episcleritis not deep scleral vessels)
What are the congenital anomalies?
Pigment cuffs Osteogenesis Imperfecta Melanosis Oculi Nevus of Ota Tumors of the sclera Senile hyaline plaque
What’s a pigment cuff?
Common anatomical varient
Involves short anterior ciliary nerves
Bluish cuff
Describe “Blue Sclera”
Tissue coloration due to scleral thinning, exposing uvea beneath
Normal in infants and asymptomatic
List some causes for Blue Sclera
Osteogenesis imperfecta Marfan's syndrome Pseudoxanthoma elasticum Ehlers-Danlos syndrome Pseudohyperparathyroidism Van der Hoeve syndrome Keratoconus and keratoglobus Buphthalmos High myopia Corticossteroid overuse Melanosis Nevus of Ota
What is Osteogenesis Imperfecta?
Inherited permenant blue sclera
Involves ekelton, ear, joints, teeth, skin and eyes
Four clinical presentations with variable inheritance and deafness
What are the three mai nsigns of Osteogenesis Imperfecta?
Blue sclera
Deafness
Bone fractures
What is Melanosis oculi?
Congenital bilateral hyperpigmentation of conjunctiva, episclera, sclera, uvea and choroid
What is the Nevus of Ota?
"Oculodermal melanocytosis" Pigmentation of periorbital skin Can be on lid, lid margin or entire face Distribution of pigment along opthalmic and maxillary divisions of CN V Congenital and unilateral Malignant degeneration
What is a Senile Hyaline Plaque?
Localized non-inflammatory thinning
Tends to be anterior to insertion of MR and LR muscles
Seen in older population, common, asymptomatic and benign
Can be multiple and diffuse
To treat, reassure patient and monitor
What is Icterus/Jaundice?
Yellowing of sclera or skin due to high concentration of bilirubin in blood
Need medical exam
Describe scleral perforation
High veolocity projectiles striking the sclera
Varying degrees of pain
Sight may not be detectable
Associated wit hconjunctivitis, keratitis, uveitis, intraocular heme, lowered IOP or hemosiderosis (rusting in the eye)
How do you treat and examine for scleral perforations?
Perform gonio, DFE, B-scan, CT scan, DO NOT USE MRI
Treat with immediate hospitalization and Fox shield to protect eyes
