001_ca0113_Corneal Atlas_JM.indd 1 12/28/12 3:41 PM AD

001_ca0113_Corneal Atlas_JM.indd 2 12/28/12 3:41 PM Ernest Bowling, OD, FAAO, is in private prac- tice in Gadsden, AL, and is associate clinical Table of Contents editor of Review of Cornea & Contact Lenses. E-mail him at [email protected]. Bacterial ...... 4

Viral ...... 5

Jeffrey Sonsino, OD, FAAO, is an assistant Acanthamoeba Keratitis ...... 7 professor at Vanderbilt Eye Institute in Nashville, TN. He is a diplomate in Cornea, Contact Lens, Fungal ...... 8 and Refractive Technology of the American Academy of Optometry, a council member of the Epithelial ...... 9 Cornea and Contact Lens Section of the American Optometric Association, and board member of the Optometrists at Academic Medical Centers. Bowman’s ...... 11

Joseph P. Shovlin, OD, FAAO, is in private Stroma ...... 11 practice in Scranton, PA, associate clinical edi- tor of Review of Optometry and clinical editor of Review of Cornea & Contact Lenses. Endothelium ...... 14 E-mail him at [email protected]. Degenerations ...... 16

Mechanical ...... 16 Christine W. Sindt, OD, FAAO, is Director of Contact Lens Service and Associate Clinical Chemical ...... 19 Professor at the University of Iowa Hospitals and Clinics, Iowa City, IA, immediate past Chair of the AOA Contact Lens and Cornea Council, Inflammatory ...... 20 a monthly columnist for Review of Cornea & Contact Lenses, co-clinical editor for Review of Optometry, and photo editor for the Corneal Atlas. E-mail her at christine-sindt@ The opinions expressed in this supplement to Review of uiowa.edu. Optometry do not necessarily reflect the views, or imply endorsement, of the editor or publisher. Copyright 2013, Review of Optometry®. All rights reserved.

This supplement has been underwritten through the generosity of Alcon.

6 11 14 17 22

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001_ca0113_Corneal Atlas_JM.indd 3 12/28/12 3:41 PM Bacterial

Etiology Approximately 25,000 Americans develop bacterial keratitis annually.1 Of patients with bacterial keratitis, 19-42% are contact lens wearers.1 Bacterial keratitis is one of the most important potential complications associated with contact lens wear. While even daily wear of contact lenses carries an increased risk of infection, extended or overnight contact lens wear is the greatest risk factor for infectious keratitis in patients choosing to wear contact lenses.2 Large-scale epidemiological studies Corneal ulcer Corneal ulcer have confirmed that the absolute and relative risk of microbial keratitis is unchanged with overnight use of silicone hydrogel materials. with fluorescein.5 Additional findings include Avoid steroids initially. Corticosteroid The key findings included the following: (1) diffuse epithelial edema, stromal infiltration treatment resulted in a statistically significant The risk of infection with 30 nights of sili- surrounding the ulceration, and mucopuru- delay in corneal re-epithelialization and cone hydrogel use is equivalent to six nights lent exudation. An anterior chamber reaction steroid use did not translate to a significant of hydrogel extended wear; (2) Occasional and hypopyon may be present. It is impor- difference in visual acuity or infiltrate/scar overnight lens use is associated with a greater tant to document the depth and location of size.12 Infectious keratitis may worsen with risk than daily lens use.3 Microbial contami- the epithelial defect and stromal infiltration. topical steroid use, especially when caused by nation of CL storage cases is a great risk for The anterior chamber should be evaluated fungus, atypical Mycobacteria or Pseudomonas. gram-negative bacterial infection among soft for cells and flare and examined for a hypo- Once the cornea has re-epithelialized and CL wearers.4 Many bacteria have been identi- pyon, and the intraocular pressure should be the causative organism has demonstrated fied in contact lens related microbial keratitis, checked. sensitivity to the antibiotic (usually after 72 with the most common organisms cultured Much has been made regarding culture hours of treatment), a steroid may be added from bacterial ulcers being Staphylococcus, and sensitivity testing in cases of microbial to the therapeutic regimen to control persis- Streptococcus, Pseudomonas and Moraxella.5 The keratitis. In general, consider cultures in ulcers tent inflammation and reduce tissue dam- gram-negative rod Pseudomonas aeruginosa is greater than 1mm to 2mm, defects in the age. However, the results of the Steroids in commonly associated with microbial kera- visual axis, ulcers unresponsive to initial ther- Corneal Ulcers Trial (SCUT) study showed titis in soft contact lens wear but a higher apy, or if an unusual organism is suspected. that adjunctive topical corticosteroid did not incidence of gram-positive organisms than Remember that just approximately 40% of improve three-month vision in patients with gram-negative organisms are recovered by cul- corneal cultures identify causative pathogens. bacterial corneal ulcers.13 The patient with ture from infections associated with contact For this reason, culturing the contact lens and an infectious keratitis needs to be followed lens wear.6,7 It is important to remember that contact lens storage case in addition to scrap- daily, with careful monitoring of the findings. the organisms Neisseria gonorrhoeae, Listeria, ing the corneal ulcer can aid in identifying The antibiotic regimen should be reduced Corynebacterium and Haemophilus aegyptius do the causative organism.9 depending on the response, but should never not require damage to the cornea and may be tapered below the minimum dose (usually invade directly through intact corneal epithe- Treatment q.i.d. to t.i.d.) to prevent the possibility of lium.8 Up to 20% of cases of fungal keratitis Ulcers need to be considered infectious bacterial resistance. (particularly candidiasis) are complicated by until proven otherwise. Therapy begins with Collagen cross-linking has been shown to bacterial coinfection.1 immediate, intensive, aggressive treatment improve healing of infectious corneal ulcers with fourth-generation fluoroquinolones in treatment-resistant cases or as an adjunct to Presentation while awaiting lab results. In vitro studies and antibiotic treatment.10 Patients with microbial keratitis present prospective clinical trials have shown that with symptoms including decreased vision, fourth-generation fluoroquinolones are better photophobia, moderate to severe ocular pain, than the older generation fluoroquinolones ICD-9 Codes redness, swelling and discharge. On slit lamp and are as potent as combined fortified • 370.00 Corneal ulcer, unspecified examination, the critical finding is a focal antibiotics against common pathogens that • 370.01 Marginal corneal ulcer 10 white opacity in the corneal stroma with an cause bacterial keratitis. Besifloxacin 0.6% • 370.02 Ring corneal ulcer overlying corneal epithelial defect that stains is a topical fluoroquinolone that has similar • 370.03 Central corneal ulcer potency against ocular pathogenic bacteria 11 • 370.04 Hypopyon ulcer as the fourth-generation agents. Dosage is • 370.05 Mycotic corneal ulcer every 30 minutes for the first six hours, fol- • 370.06 Perforated corneal ulcer lowed by hourly administration around the • 371.00 Corneal opacity/scar, clock until improvement is noted. The appli- cation of fourth-generation fluoroquinolones unspecified (upon resolution) in the treatment of corneal ulcers is an off- label use of these medications, but routinely References performed. Cycloplegic drops are valuable 1. Murillo-Lopez FH. Bacterial Keratitis. Medscape. Available for patient comfort and to prevent synechiae at : http://emedicine.medscape.com/article/1194028- Pseudomonas corneal ulcer formation in accompanying iritis. overview#a0199. Accessed 11/26/2012.

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001_ca0113_Corneal Atlas_JM.indd 4 12/28/12 3:42 PM 2. Keay L, Stapleton F, Schein O. Epidemiology of contact- 8. Reed WP, Williams RC. Bacterial adherence: First step in lens related inflammation and microbial keratitis: A 20-year pathogenesis of certain infections. J Chronic Dis 1978; 31:67. perspective. Eye Cont Lens 2007; 33: 346-353. 9. Das S, Sheorey H, Taylor HR, et al. Association between cul- 3. Stapleton F, Keay L, Edwards K, et al. The Epidemiology of tures of contact lens and corneal scraping in contact lens relat- Microbial Keratitis With Silicone Hydrogel Contact Lenses. Eye ed microbial keratitis. Arch Ophthalmol 2007; 125: 1182-1185. Contact Lens. 2012 Nov 20. [Epub ahead of print]. Available 10. Wong RL, Gangwani RA, Yu LW, et al. New treatments at: http://www.ncbi.nlm.nih.gov/pubmed/23172318. Accessed for bacterial keratitis. J Ophthalmol 2012;2012:831502. Epub 11/26/2012. 2012 Sep 6. Available at: http://www.ncbi.nlm.nih.gov/pmc/ 4. Bharathi MJ, Ramakrishnan R, et al. Ulcerative keratitis articles/PMC3444050/. Accessed 11/26/2012. associated with contact lens wear. Indian J Ophthalmol 2007; 11. Morris TW, Haas W, Brunner LS, et al. Clinical efficacy of 55: 64-67. besifloxacin ophthalmic suspension: integrated microbiological 5. Ehlers JP, Shah CP, eds. The Wills Eye Manual: Office and results of 3 trials for topical treatment of bacterial conjuncti- Staph marginal ulcer Emergency Room Diagnosis and Treatment of Eye 5th ed. vitis. Paper presented at: Association for Research in Vision Philadelphia: Lippincott Williams & Wilkins, 2008: 62-66. and Ophthalmology (ARVO) Annual Meeting: May 3-7, 2009; 6. Green M, Apel A, Stapleton F. Risk factors and causative Ft. Lauderdale, FL. 13. Srinivasan M, Mascarenhas J, Rajaraman R, et al. organisms in microbial keratitis. Cornea 2008; 27: 22-27. 12. Srinivasan M, Lalitha P, Mahalakshmi R,et al. Corticosteroids for Bacterial Keratitis: The Steroids for Corneal 7. Jeng BH, McLeod SD. Microbial keratitis. Br J Ophthalmol Corticosteroids for Bacterial Corneal Ulcers. Br J Ophthalmol Ulcers Trial (SCUT). Arch Ophthalmol 2011 Oct 10. [Epub 2003; 87: 805–806. 2009; 93(2):198-202. ahead of print].

Viral Infections

ADENOVIRAL KERATOCONJUNCTIVITIS Etiology Adenoviruses produce the most common viral conjunctival infections.1 The condition is quite contagious and is transmitted readily in respiratory and ocular secretions, eye drop- pers and mascara bottles, and contaminated swimming pools. Eye care professionals are often guilty of spreading adenovirus because it is highly contagious and has 53 serotypes Petechial hemorrhages in adenoviral conjunctivitis with variable morphology.2 The incubation Bulbar conjunctival follicles in epidemic keratoconjunctivitis period is usually five to 12 days, and the clinical illness is present from five to 15 days. decongestants/antihistamines. Betadine 5% is Most cases of viral conjunctivitis resolve a few days later. Signs include conjunctival now available as an FDA-approved product spontaneously, without sequelae, within days injection and swelling. The lids may be swol- that can be used “off-label” to treat EKC. to weeks. Adenovirus is often difficult to len. Inferior palpebral conjunctival follicles are This agent has minimal toxicity when used diagnose based on clinical appearance and, seen. Pinpoint subconjunctival hemorrhages properly and followed by saline irrigation. in the early stages, is associated with a red eye and membrane formation over the palpebral In vitro, povidone iodine is effective against or superficial keratitis common to herpes and conjunctiva are occasionally seen. In some adenovirus as well as many other infectious other infections.2 cases multiple, focal infiltrates in the cornea agents. Topical steroids are indicated when There are four forms of adenoviral anterior to mid-stroma may be seen. A pre- the visual axis is involved or membrane conjunctivitis: Follicular conjunctivitis, auricular lymphadenopathy is present. A rapid, or pseudo-membrane formation is noted. pharyngoconjunctival fever, epidemic kerato- in-office immunodiagnostic test using antigen Patients should discontinue contact lens wear. conjunctivitis (EKC) and acute hemorrhagic detection is available for adenovirus conjunc- Avoid the use of topical and oral antibiotic or conjunctivitis.3 Follicular conjunctivitis is tivitis. In a study of 186 patients with acute antiviral agents as these will not help resolu- the mildest form of adenoviral conjunctivi- conjunctivitis, this test had a sensitivity of 88% tion and may promote antibiotic resistance. tis. Pharyngoconjunctival fever is the most to 89% and a specificity of 91% to 94%.4 Patients with adenoviral conjunctivitis need common ocular adenoviral infection and is to understand that the condition is highly characterized by a combination of pharyngi- Treatment contagious and should be informed of appro- tis, fever, and conjunctivitis. EKC is a more Palliative therapy is often sufficient for priate measures to reduce the risk of spread- severe form of conjunctivitis and typically most cases of adenoviral conjunctivitis: ing the infection to their other eye or to other lasts for seven to 21 days. EKC can affect the cold compresses, artificial tears and topical people. Recent data also indicate ganciclovir cornea with coarse keratitis and sub-epithelial 0.15% ophthalmic gel may hold promise as a infiltrates (SEIs). SEIs may last for months, treatment for adenoviral keratoconjunctivitis.5 affecting visual acuity. Acute hemorrhagic conjunctivitis produces a severe, painful fol- HERPES SIMPLEX KERATITIS licular conjunctivitis with the development of Etiology tiny subconjunctival hemorrhages. The herpes simplex virus (HSV) is the leading cause of vision loss in the United Presentation States. Keratitis caused by HSV is also the In general, viral infections present with red- most common cause of cornea-derived blind- ness, irritation, itching, foreign body sensation, ness in developed nations.6 The HSV is a tearing and photophobia. The condition starts Subepithelial infiltrates in epidemic kerato- DNA virus that resides latent in the trigemi- in one eye and then progresses to the other conjunctivitis nal ganglion, only to resurrect during periods

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001_ca0113_Corneal Atlas_JM.indd 5 12/28/12 3:42 PM Herpes zoster keratitis manifests in five basic clinical forms: • Epithelial keratitis (acute or chronic). Multiple, fine, raised intraepithelial lesions located paracentrally or at the limbus, which stain mildly with fluorescein but intensely with rose bengal. • Nummular stromal keratitis. Multiple, fine, granular infiltrates in the anterior corneal HSV keratitis Atypical HSV ulcer stroma. • Disciform keratitis. A central, well-defined, of intense stress, illness, irritation and photo- oral dosage. Alternatively, oral valacyclovir disc-shaped area of diffuse stromal edema toxic exposure. The disease can be present as (Valtrex), a prodrug of acyclovir, can be given without vascularization. Corneal edema with superficial lesions, neurotrophic disease, or 500mg three times a day. Since these drugs anterior chamber inflammation. with deep stromal involvement. are cleared through the kidneys, it is critically • Limbal vascular keratitis. Limbal-vessel important for the patient with renal disease ingrowth and stromal edema. May be associ- Presentation that the patient’s nephrologist or primary care ated with adjacent episcleral or scleral inflam- Patients with HSV infection present with physician and/or pharmacist be consulted mation. rapid onset unilateral pain and redness, regarding dosing. For a patient on long-term • Neurotrophic keratitis. An inferior, oval watering and light sensitivity. Diagnosis of oral antiviral therapy for recurrent disease, epithelial defect with rolled edges. Can lead HSV infection is primarily based on clinical check creatinine levels to ensure there is no to corneal perforation. findings. The disease starts as a punctate kidney damage. Normal levels of serum cre- HZO can cause either a nongranuloma- epithelial keratitis, coalescing into the clas- atinine are approximately 0.8mg to 1.4mg per tous or granulomatous anterior uveitis with sic branching epithelial ulceration with deciliter in adults. Oral antiviral prophylaxis keratic precipitates and posterior synechiae. terminal end bulbs within 24 to 48 hours.7 has been associated with a decreased risk The diagnosis of herpes zoster disease is gen- The dendrites stain with rose bengal or lis- of recurrence of epithelial keratitis, stromal erally based on clinical findings. samine green. Corneal sensitivity may be keratitis, conjunctivitis, and blepharitis due decreased. The neurotrophic form of HSV to HSV.10 Treatment disease is characterized by areas of intense All patients with ophthalmic zoster, punctate change or epithelial denudement, HERPES ZOSTER OPHTHALMICUS irrespective of age or severity of symptoms, and can result in corneal scarring. Deep Etiology should be prescribed oral antiviral drugs at stromal lesions appear as a round, fluid Herpes zoster ophthalmicus (HZO) is the first sign of disease. Patients with HZO filled circle. Scarring can develop in later a recurrent infection of the varicella zoster are treated with oral acyclovir (800mg, five stages with loss of stromal thickness and (chickenpox) virus in the ophthalmic division times daily) for seven to 10 days. Early treat- corneal thinning. of the trigeminal dermatome, most often ment with acyclovir (within 72 hours after affecting the nasociliary branch. HZO can rash onset) reduces the percentage of eye Treatment affect any of the ocular and adnexal tissues. disorders in ophthalmic zoster patients from Treatment of active HSV keratitis consists One in four people will contract herpes zoster 50% down to 20% to 30% and also lessens of topical trifluridine 1% solution every one in their lifetime, with this risk rising markedly acute pain.14 Famciclovir 500mg three times to two hours until no sign of active infection after 50 years of age, especially in very elderly daily for seven days or valacyclovir 1,000mg (lack of dendrite patterns), then five times individuals.11 Ophthalmic herpes zoster repre- three times daily are alternatives to acyclovir. a day for an additional seven to 10 days.8 sents 10% to 20% of all zoster cases.12 In general, the dosages for HZO are twice Zirgan (ganciclovir) ophthalmic gel 0.15% is that for HSV infections. Palliative therapy indicated for the treatment of acute herpetic Presentation including cool compresses, mechanical keratitis. Four international, randomized, HZO usually begins as an influenza-like cleansing of the involved skin and topical multicenter clinical trials have demonstrated illness characterized by fatigue, malaise, nau- antibiotic ointment without steroid are help- that ganciclovir gel is at least as effective sea and mild fever accompanied by progres- ful in treating skin lesions. Epithelial defects as acyclovir ointment for the treatment of sive pain and skin hyperesthesia. A diffuse associated with HZO keratitis may be treated HSV keratitis. Ganciclovir gel was better erythematous or maculopapular rash appears with nonpreserved artificial tears, eye oint- tolerated, with lower rates of blurred vision, over a single dermatome three to five days ments, punctal occlusion, pressure patching, eye irritation, and punctate keratitis.5 The later. The skin of the forehead and upper or therapeutic soft contact lenses. Topical recommended dosing regimen for Zirgan is eyelid is commonly affected and strictly steroids are useful in the management of kera- one drop in the affected eye five times per obeys the midline with involvement of one touveitis, interstitial keratitis, anterior stromal day (approximately every three hours while or more branches of the ophthalmic divi- infiltrates, and disciform keratitis. Topical awake) until the corneal ulcer heals, and then sion of the trigeminal nerve. Involvement of cycloplegics prevent ciliary spasm associated one drop three times per day for seven days.9 the tip of the nose (Hutchinson’s sign) has Topical acyclovir 3% ointment (no longer been thought to be a clinical predictor of commercially available, but can be obtained ocular involvement. Although patients with from specialized compounding pharmacies) a positive Hutchinson’s sign have twice the can be used five times a day as an alternative incidence of ocular involvement, one third in patients with a known sensitivity to the of patients without the sign develop ocular above medications. manifestations.13 HZO conjunctivitis is a Oral antivirals are gaining use in the common ocular finding and the conjunctiva treatment of epithelial disease. Acyclovir appears swollen and injected, with occa- 400mg five times a day is the most common sional vesicles and petechial hemorrhages. Subepithelial infiltrates in viral conjunctivitis

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001_ca0113_Corneal Atlas_JM.indd 6 12/28/12 3:42 PM of herpes simplex virus keratitis: a systematic review. Ocul ICD-9 Codes Surf 2007; 5(3): 240-50. 7. Kahn BF, Pavan-Langston D. Clinical manifestations and • 372.00 Unspecified conjunctivitis treatment modalities in herpes simplex virus of the ocular • 372.02 Acute follicular conjunctivitis anterior segment. Int Ophthalmol Clin 2004;44:103-33. • 372.03 Other mucopurulent 8. Ehlers JP, Shah CP, eds. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease, conjunctivitis 5th Ed. Philadelphia, PA: Lippincott, Willliams and Wilkins, 2008. • 372.04 Pseudomembranous 9. Zirgan package insert. Bausch + Lomb Pharmaceuticals. Interstitial keratitis conjunctivitis 10. Young RC, Hodge DO, Liesegang TJ, et al. Incidence, • 372.11 Simple chronic conjunctivitis recurrence, and outcomes of herpes simplex virus eye disease in Olmsted County, Minnesota, 1976-2007: the effect of oral with herpes zoster inflammatory disease. • 077.1 Epidemic keratoconjunctivitis antiviral prophylaxis. Arch Ophthalmol 2010;128(9):1178-83. Aqueous suppressants and topical corticoste- • 077.3 Adenoviral (acute follicular) 11. Johnson RW, Bouhassira D, Kassianos G ,et al. The roids should be used to treat glaucoma asso- impact of herpes zoster and post-herpetic neuralgia on quality-of-life. BMC Med 2010;8:37. ciated with HZ disease. Consider Zostavax 877–94. 12. Ragozzino MW, Melton LJ 3rd, Kurland LT, et al. vaccine for your patients over 60 years of age. 2.Kaufman HE. Adenovirus advances: new diagnostic and Population-based study of herpes zoster and its sequelae. In the landmark Shingles Prevention Study, therapeutic options. Curr Opin Ophthalmol 2011; 22 (4): 290-3. Medicine (Baltimore) 1982;61:310-16. the live attenuated varicella zoster virus vac- 3. Yanoff M, Duker JS, eds. Ophthalmology, 3rd ed. Maryland 13. Harding SP, Lipton JR, Wells JC. Natural history of herpes Heights, MO, Elsevier, 2009. zoster ophthalmicus: predictors of postherpetic neuralgia and cine reduced the risk of developing shingles 4. Sambursky R, Tauber S, Schirra F, et al. The RPS ocular involvement. Br J Ophthalmol 1987;71:353–58. 15 by 51%. adeno detector for diagnosing adenoviral conjunctivitis. 14. Cobo LM, Foulks GN, Liesegang T, et al. Oral acyclo- Ophthalmology 2006;113:1758-64. vir in the treatment of acute herpes zoster ophthalmicus. References 5.Kaufman HE, Haw WH. Ganciclovir ophthalmic gel 0.15%: Ophthalmology 1986; 93:763-70. 1.Gordon, JS, Aoki, K, Kinchington, PR. Adenovirus kerato- safety and efficacy of a new treatment for herpes simplex 15. Oxman MN, Levin MJ, Johnson GR, et al. A vaccine to conjunctivitis. In: Pepose JS, Holland GN, Wilhelmus KR, eds. keratitis. Curr Eye Res 2012; 37 (7): 654-60. prevent herpes zoster and postherpetic neuralgia in older Ocular Infection and Immunity. St. Louis: Mosby; 1996. pp. 6. Guess S, Stone DU, Chodosh J. Evidence-based treatment adults. N Engl J Med 2005; 352:2271-84.

Acanthamoeba Keratitis

Background secrete a variety of proteases, which facilitate often resemble those of herpes simplex kerati- Acanthamoeba keratitis can be severe and the dissolution of the corneal stroma.5 When tis; however, the Acanthamoeba lesions appear vision-threatening. It was first recognized in environmental conditions become unfavor- edematous and necrotic rather than frank contact lens wearers in the early 1970s, and able, the organism converts to a dormant ulcerations. contact lens wear is thought to be associ- cystic form, which is able to survive many A ring infiltrate is classically thought of as ated with 80% of the cases.1,2 Symptom years. These double-walled cysts are highly the defining sign of Acanthamoeba keratitis; onset is greatest during the summer months. resistant to killing by desiccation, freeze/ however, it tends to form four to eight weeks Acanthamoeba species are found in virtu- thaw cycles, irradiation, chlorination levels after onset of symptoms and is rarely the ally every environment. These protozoa are and antimicrobial agents. Co-infection with presenting sign. Radial perineuritis (perhaps ubiquitous in the soil, dust, lakes, rivers, hot bacteria or fungi is common, providing food explaining the intense pain) may be seen on tubs and salt water. They have been isolated for amoeba. slit lamp examination or confocal micros- from heating, venting and air conditioner copy. Unchecked, there may be progressive units, humidifiers, dialysis units and contact Presentation corneal thinning and risk of perforation. Up lens paraphernalia. Acanthamoeba have been Acanthamoeba keratitis presents with pain to 40% of patients may have mild to severe found in the nose and throat of healthy (ranging from mild foreign body sensation to anterior uveitis. Scleritis has been reported in people as well as those with compromised severe pain), photophobia, decreased vision, patients with Acanthamoeba keratitis; however, immune systems. Contact lens wear and poor injection, irritation, tearing and a protracted the scleral inflammation was attributed to an lens hygiene are often singled out as the big- clinical course. The patient often presents immune-mediated response to necrotic organ- gest risk factors for Acanthamoeba keratitis. with a unilateral red eye, where the pain is isms and was not believed to be the result of The true incidence is not known; however, it disproportionately worse than one would active infection.6-8 is thought to be rare, affecting approximately surmise from the clinical appearance. Early Severe glaucoma has been associated 1.65-2.01 per million contact lens wearers per corneal findings include irregular epithelium, with Acanthamoeba keratitis secondary to year in the United States.3 However, it has punctate epithelial erosions, microcystic an inflammatory angle-closure mechanism, been reported as high as 1/30,000 contact edema, perilimbal injection and dendritiform apparently without direct infiltration of the lens wearers per year outside the U.S.4 epithelial lesions. The dendritiform lesions organism.9 Etiology There are more than 20 different species, several of which are known to cause infec- tions in humans, including A. culbertsoni, A. polyphaga, A. castellanii, A. healyi, A. astronyxis, A. hatchetti and A. rhysodes. A. castellanii is the most common amoeba associated with cor- neal infection. The life cycle of these organ- isms is comprised of two stages: trophozoite and cystic forms. Trophozoites bind to and desquamate the corneal epithelium. They Early Acanthamoeba keratitis Late stage Acanthamoeba keratitis

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001_ca0113_Corneal Atlas_JM.indd 7 12/28/12 3:42 PM Treatment 2. Illingworth CD, Cook SD, Karabatsas CH, Easty DL. Diagnosis largely depends on the ability to Acanthamoeba keratitis: risk factors and outcome. Br J Ophthalmol. 1995 Dec;79(12):1078-82. visualize the organism. Definitive diagnosis is 3. Schaumberg DA, Snow KK, Dana MR. The epidemic of made by corneal scrapings. Confocal micros- Acanthamoeba keratitis: where do we stand? Cornea. 1998 copy is clinically useful to quickly identify Jan;17(1):3-10. Review. the organism in vivo. Attempts to culture the 4. Seal DV. Acanthamoeba keratitis update-incidence, molec- ular epidemiology and new drugs for treatment. Eye. 2003 organism is time- consuming and expensive, Nov;17(8):893-905. Review. 5. Investigative Ophthalmology & often with poor yields. Polymerase chain reac- Visual Science, July 2005, Vol. 46, No. 7. tion may be beneficial for identifying amoeba 6. Lee GA, Gray TB, Dart JK, et al. Acanthamoeba sclero- in the future.10 keratitis: treatment with systemic immunosuppression. Ophthalmology. 2002;109:1178-1182. Differential diagnosis includes herpetic 7. Garner A. Pathogenesis of Acanthamoeba keratitis: keratitis, bacterial keratitis, toxic keratopathy hypothesis based on a histological analysis of 30 cases. Br J (solution related), stem cell failure, fungal Central scarring from Acanthamoeba keratitis Ophthalmol. 1993;77:366-370. keratitis, severe dry eye and contact lens relat- 8. Lindquist TD, Fritsche TR, Grutzmacher RD. Scleral ectasia secondary to Acanthamoeba keratitis. Cornea. 1990;9:74-76. ed corneal oxygen deficiency. Debridement, cornea, even in a cornea that appears quiet, 9. Kelley PS, Dossey AP, Patel D, Whitson JT, Hogan particularly early in the disease, will reduce which may incite infection after graft. After RN, Cavanagh HD. Secondary glaucoma associated with the number of organisms and deprive the corneal transplant, protective, maintenance advanced Acanthamoeba keratitis. Eye Contact Lens. 2006 Acanthamoeba of its food supply.11 Cationic doses of medication should be used to help Jul;32(4):178-82. 10. Laummaunwai P, Ruangjirachuporn W, Boonmars T. antiseptic agents, such as chlorhexidine prevent the recurrence of the Acanthamoeba A simple PCR condition for detection of a single cyst of 17 0.02% and polyhexamethylene biguanide infection. Acanthamoeba species. Parasitol Res. 2011 Oct 12. [Epub 0.02%, are generally considered primary ahead of print] medical treatments. Biguanides disrupt the Recommendations 11. Lindquist TD. Treatment of Acanthamoeba keratitis. Cornea. 1998 Jan;17(1):11-6. phospholipid structure of cell membranes. • Avoid swimming with contact lenses on. 12. Seal D. Treatment of Acanthamoeba keratitis. Expert Rev Aromatic diamides (Brolene) directly affect • Showering in contact lenses may increase Anti Infect Ther. 2003 Aug;1(2):205-8. Review. the amoebas’ nucleic acids and are thought to risk of infection.15 13. Hammersmith KM. Diagnosis and management of have a synergistic effect with the biguanides. • Careful following of recommended lens Acanthamoeba keratitis. Curr Opin Ophthalmol. 2006 Aug;17(4):327-31. There is a risk of significant corneal epithelial care systems. 14. McClellan K, Howard K, Niederkorn JY, Alizadeh H. Effect 12,13 toxicity with q1-2h dosing. Corticosteroids of steroids on Acanthamoeba cysts and trophozoites. Invest are used to treat associated uvetitis or scleritis; ICD-9 Codes Ophthalmol Vis Sci. 2001 Nov;42(12):2885-93. however, this should be done with extreme 15. Oldenburg, CE, Acharya NR, Tu EY, et al. Practice • 370.02 Ring corneal ulcer Patterns and Opinions in the Treatment of Acanthamoeba caution because trophozoite proliferation has Keratitis. Cornea October 2011 (Epub ahead of print). 14 • 370.40-006.8 Keratoconjunctivitis been observed when exposed to steroids. 16. Visvesvara GS, Stehr-Green JK. Epidemiology of free- liv- Penetrating keratoplasty (PKP) may be neces- ing ameba infections. The Journal of protozoology 1990, sary for tectonic or optical corneal rehabilita- References: 37(4):25S-33S. 1. Jones DB, Visvesvara GS, Robinson NM. Acanthamoeba 17. Joslin, CE, Tu, EY, Stayner L, Davis F. The association tion. In most situations, PKP is postponed polyphaga keratitis and Acanthamoeba uveitis associated with between Acanthamoeba keratitis and showering with con- 15,16 until resolution of infection. There may fatal meningoencephalitis. Transactions of the ophthalmologi- tact lenses. Paper presented at the American Academy of be residual dormant cysts in the peripheral cal societies of the United Kingdom 1975, 95(2):221-232. Optometry October 22, 2008.

Fungal Keratitis

Fungal keratitis is relatively rare in the injection, tearing and possible discharge; how- United States (approximately 5% to 10% of ever, the degree of symptoms may vary. In reported cases), although it accounts for up some cases, the progression of symptoms may to 50% of ulcerative keratitis elsewhere in the be slow, while in others it may move very world. Fungal keratitis is usually associated quickly. Corneal infiltrates tend to have feath- with a history of ocular trauma, ocular surface ery borders, are generally grayish-white and disease or topical steroid use.1-3 There has may have satellite lesions. Larger infiltrates been a lot of attention focused on the recent are associated with poor visual prognosis. epidemic of fungal keratitis in soft contact The epithelium is usually raised, and at times lens wearers in 2005 and 2006; however, a Fungal corneal ulcer may be intact over the infiltrate. An epithelial recent review indicates the number of fungal defect, anterior chamber reaction or hypo- keratitis cases associated with contact lens bacterial co-infection. Risk factors for the pyon may be present. wear has been steadily increasing the past 20 development of fungal keratitis include ocu- Diagnosis may be difficult based on clini- 1,4,5 years. lar trauma, topical corticosteroids, systemic cal examination alone. Confocal microscopy immunosuppression, penetrating or refractive may reveal hyphae in filamentary fungal dis- Etiology surgery, chronic keratitis (vernal/atopic kerati- ease, such as Aspergillus or Fusarium, or bud- Fungi require an epithelial defect for cor- tis and neurotrophic ulcers) and contact lens ding yeast forms such as Candida.6,7 Fungal neal penetration. Once the epithelium has wear with certain lens solutions. cultures are the gold standard for diagnosis.8 been violated, the present fungi can multiply Corneal scrapings and cultures may be posi- and cause severe tissue damage. Up to 30% of Presentation tive in up to 90% of initial scrapings. Most fungal keratitis cases may be associated with Patients present with pain, photophobia, fungi grow well in blood agar or Sabouraud

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001_ca0113_Corneal Atlas_JM.indd 8 12/28/12 3:42 PM dextrose agar as culture media. Growth usu- fungal keratitis reported from a University of ally occurs within three or four days but Florida study showed poor contact lens care.14 can take as long as four to six weeks.9 Gram and giemsa stains or potassium hydroxide (KOH) wet mounts are useful for identifying ICD-9 Codes fungal elements. Polymerase chain reaction • 370.05 Mycotic corneal ulcer (PCR) is another diagnostic tool. Results • 370.04 Hypopyon corneal ulcer from clinical studies suggest that PCR is more sensitive than culture as a diagnostic References 1. Chang DC, Grant GB, O’Donnell K, et al. Multistate out- aid in ocular fungal infections and is also Fungal corneal ulcer much faster. Results are known within 24 break of Fusarium keratitis associated with use of a contact lens solution. JAMA 2006; 296(8): 953-63. hours. However, PCR is associated with a 2. Rosa RH Jr, Miller D, Alfonso EC. The changing spectrum 8 high false-positive rate. If there is strong sus- evaluation. Therapeutic penetrating kera- of fungal keratitis in south Florida. Ophthalmology 1994; 101: picion of fungus and other tests are negative, toplasty is often required to restore vision 1005-1113. biopsy may be required. impairment due to corneal scarring. Despite 3. Wong TY, AuEong KG, Chan WK, et al. Fusarium keratitis following the use of topical antibiotic-corticosteroid therapy in maximum pharmacologic therapy, early trans- traumatized eyes. Ann Acad Med Singapore 1996; 25: 862- Treatment plant during active disease may be required 865. Topical natamycin 5% or topical ampho- early in cases of perforation or near perfora- 4. Khor WB, Aung T, Saw SM, et al. An outbreak of Fusarium tericin B 0.15% is first-line therapy for symp- tion.11 As many as 27% of patients with keratitis associated with contact lens wear in Singapore. 8 JAMA. 2006;295(24):2867-73. toms of suspected superficial fungal keratitis. ocular fungal infections can require corneal 5. Tuli SS, Iyer SA, Driebe WT. Fungal keratitis and contact 2 Natamycin is currently the only topical transplants. lenses: an old enemy unrecognized or a new enemy on the ophthalmic antifungal compound approved The use of topical steroids is detrimental block? Eye Cont Lens 2007; 33 (6): 415-417. by the FDA.10 It penetrates the cornea well in the treatment of fungal keratitis.12 Extreme 6. Kaufman SC, Musch DC, Belin MW, et al. Confocal micros- copy: a report by the American Academy of Ophthalmology. after topical administration and is the drug caution should be used with steroids until a Ophthalmology 2004; 111(2): 396-406. of choice for fungal keratitis. Amphotericin sufficient amount of time for clinical stabi- 7. Klont RR, Eggink CA, Rijs AJ, et al. Successful treatment of B, because of its numerous toxicities, is lization has been achieved because steroids Fusarium keratitis with cornea transplantation and topical and administered as a second-line treatment to reduce the patient’s immune ability to elimi- systemic voriconazole. Clin Infect Dis 2005; 40(12): 110-112. 13 8. Hu WS, Fan VC, Koonapareddy C, et al. Contact lens- natamycin. Recommended dosage is 1mg/ nate infection. related fusarium infection: case series experience in New York kg/day intravenously or topically in 0.15% to City and review of fungal keratitis. Eye Cont Lens 2007; 33 0.3% solution every 30 to 60 minutes. Side Recommendations (6): 322-328. effects can include renal toxicity, headaches, Early on, the patient may present with no 9. Thomas PA. Current perspectives on ophthalmic myco- 10 ses. Clin Microbiol Rev 2003; 16: 730-797. fevers, chills and anorexia. As is the case for more than a “gritty” foreign body sensation 10. O’Brien TP. Therapy of ocular fungal infections. most anterior segment injuries and infections, with only a small, indistinct infiltrate. Fungal Ophthalmol Clin North Am 1999; 12: 33-50. cycloplegics should be dispensed to improve keratitis is commonly confused with bacterial 11. Winchester K, Mathers WD, Sutphin JE. Diagnosis of patient comfort. In addition to standard ther- keratitis. There should be a high level of sus- Aspergillus keratitis in vivo with confocal microscopy. Cornea 1997; 16(1): 27-31. apy for fungal keratitis, voriconazole (topical picion of fungal agents if the lesions do not 12. O’Day DM, Ray WA, Robinson RD. Efficacy of anti-fungal and oral) has also been successfully used to resolve/improve despite antibiotic therapy. agents in the cornea. II. Influence of corticosteroids. Invest impart a clinical cure. Steroids will worsen/exacerbate the disease Ophthalmol Vis Sci 1984; 25: 331-335. Mechanical debridement of the corneal and should not be used in suspected fungal 13. Krachmer JH, Mannis MJ, Holland EJ. Cornea, 2nd Edition, Vol. 1. Philadelphia: Elsevier Mosby, 2005. pp.1101-1113. epithelium may aid in penetration of topical infections. 14. Iyer SA, Tuli SS, Wagoner RC. Fungal keratitis: Emerging medication into the stroma while providing Optimal contact lens care is essential. trends and treatment outcomes. Eye Cont Lens 2006; 32: a specimen for histopathological stains and Nearly all of the cases of contact lens related 267-271.

Epithelial

The corneal epithelium serves a variety adherences. The typical epithelial layer flow more anteriorly towards the surface, of roles, including the primary mode of is approximately 50µm thick. During cell ultimately losing their nuclei and becom- protection for the corneal surface and as division, the epithelial cells migrate and ing “wafer” thin. the interface tissue between the corneal stroma and the tear film. Derived from surface ectoderm, it can be best described as a nonkeratinized, stratified squamous layer possessing many of the same prop- erties of regeneration after injury and, fortunately enough, recovers in rapid fash- ion. For purposes of brevity, the corneal epithelium consists of deep columnar cells attached to the anterior limiting lamina (or Bowman’s membrane), midlayer polygonal cells, and surface wing cells all bound together through a variety of tight Epithelial basement membrane dystrophy Epithelial basement membrane dystrophy

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001_ca0113_Corneal Atlas_JM.indd 9 12/28/12 3:42 PM Corneal abrasions are one of the most and photophobia.3 Tests to Help Delineate Structure and common forms of ocular trauma present- Lisch epithelial corneal dystrophy Function ing to an optometric clinic. Damage to (LECD) is a relatively new disorder that • All tests should have an interpretation of the corneal epithelium in corneal abrasion was first described in 1992.4 It is unusual findings. and its subsequent repair is influenced by because it is linked to the X chromosome. • Corneal topography—useful for cases of a variety of comorbidities, including the It is a gelatinous, whorl-like corneal dys- epithelial irregularity involving visual axis. presence of conditions such as diabetes, trophy associated with surface deposition • Anterior segment photography—useful Sjögren’s or other tear chemistry altering ill- and appears as epithelial microcysts on for documenting presence and extent of sur- ness, corneal denervation and neuropathy, retroillumination. It typically begins in face changes. lagophthalmos, and basement membrane childhood, sparing the center of the cornea. • Tear film performance tests—Shirmer’s I degenerations or dystrophies. Most epithe- As the opacities progress toward the center or II, tear break-up time, and phenol red test. lial trauma will recover within three to five they can decrease acuity but are not associ- days by a coordinated migration of cells as ated with recurrent erosions.5 Gelatinous, Pearls an epithelial sheet. Large or total abrasions drop-like corneal dystrophy is a rare corneal • Never use a bandage contact lens with can take significantly longer to heal. Careful presentation association with surface deposi- corneal trauma that might be at risk for fun- attention needs to be paid to the size, posi- tion thought to be caused by a mutation of gal infection. For instance, caution should be tion, and depth of the abrasion and whether the M1S1 gene.6 Typically associated with used with a patient presenting to the office or not any signs of infiltrate are present. Japanese heritage, the condition has been with a tree branch injury to the cornea. Fluorescein dye should be instilled to mea- noted in other parts of the world and char- • Consider using a stiffer modulus soft sure the abrasion and monitor its improve- acterized by severe visual impairment. contact lens for corneal irregularities in the ment; for a deeper abrasion it is imperative corneal visual axis. Stiffer designs can drape to assess for risk of perforation and check Treatment—Abrasion over irregular zones and dampen the effects for Seidel’s sign. Should either of these be The treatment will depend upon the of irregular astigmatism. detected, prompt medical comanagement extent and presentation of the injury. In • For patients considering cataract surgery should be initiated. Though any abrasion cases where there are irregular, ragged edges AND possessing significant EBMD in the can predispose to future erosions, those of epithelial tissue it is essential to debride visual axis, consider a targeted epithelial caused by paper, fingernails or tree branches that tissue. Antibiotic drops can be dosed debridement, particularly if located near the are more commonly associated with recur- from q.i.d. to q.h. depending on the size patients’ visual axis. rent erosions and the patient should be and depth of the injury, along with a cyclo- • For patients with EBMD, Meesmann’s treated and educated accordingly. Sodium plegic agent b.i.d.-q.i.d. for photophobia. or Reis-Bucklers (Bowman’s layer), consider chloride eyedrops and ointments, such as A bandage contact lens can be extremely the use of bandage soft lenses for episodes of Muro 128 5%, are advised. beneficial to promote reepithelialization and recurrent erosion. Furthermore, encourage the There are a variety of corneal epithelial improve patient comfort. use of nightly hyperosmostic ointments to dystrophies. Almost all are considered reces- limit intermittent nightly erosions. sive in nature and can cause the patient Treatment—Dystrophies and symptomatic visual distortion in the form Degenerations of irregular astigmatism or intermittent pain EBMD treatment depends on the level ICD-9 Codes secondary to recurrent erosions. of visual performance and patient discom- • 918.00 Corneal abrasion Epithelial basement membrane dys- fort. For mild presentations, the patient • 367.22 Irregular astigmatism trophy (EBMD), also known as map-dot- can use artificial tears and hyperosmotic • 371.42 Recurrent erosion of cornea fingerprint dystrophy or Cogan’s dystrophy, drops and/or ointments. Ointment at bed- • 371.51 Meesmann’s is described as hereditary in nature, presents time is especially helpful for those patients • 371.52 EBMD bilaterally and is progressive.1 Typically, having difficulty with overnight erosions. EBMD can be visualized in white light at Moisture chamber goggles for nighttime New CPT Code: 92071 Fitting of a the biomicroscope, by using sodium fluores- use is part of the palliative treatment. For contact lens for treatment of ocular cein dye and a wratten filter and looking for patients with blurred vision due to irregu- surface disease areas of discrete negative staining, as well by lar astigmatism, a soft, rigid or hybrid irregularity on corneal topography. Typically contact lens can be utilized. If these treat- these patients have reduced acuity that can ments are unsuccessful, the faulty epitheli- References be improved with a gas permeable trial lens. um can be debrided by diamond burr ker- 1. http://emedicine.medscape.com/article/1193945-overview Subepithelial mucinous corneal dys- atectomy, phototherapeutic keratectomy 2. Feder RS, Jay M, Yue By, Stock EL, O’Grady RB, Roth SI. trophy is a very rare autosomal dominant (PTK) or anterior stromal micropuncture Subepithelial mucinous corneal dystrophy. Clinical and path- ological correlations. Arch Ophthalmol 1993; 111: 1106-14. condition characterized by frequent recur- (ASM). Treatment of the other epithelial 3. Jalbert I, Stapleton F. Management of symptomatic rent erosions in the first decade of life fol- dystrophies need to be considered on a Meesmann dystrophy. Optom Vis Sci. 2009; 86: E1202- 06. lowed by progressive vision loss. The lesions case-by-case basis where treatment ranges 4. Lisch W, Steuhl KP, Lisch C, Weidle EG, Emming CT, involve the entire cornea, but are typically from symptomatic relief with lubrication, Cohen KL, Perry HD. A new, band-shaped and whorled 2 microcystic dystrophy of the corneal epithelium. Am J found centrally. Muro 128, and bandage contact lenses Ophthalmol. 1992; 114: 35-44. Meesmann’s corneal dystrophy is a to more advanced treatments including 5. Aldave A, Sonmez B. Elucidating the molecular bilateral autosomal dominant disorder that debridement, PTK, and penetrating and genetic basis of the corneal dystrophies. Arch Ophthalmol generally appears early in life as epithe- lamellar keratoplasty. However, there is a 2007;12592):172-186. 6. Ha NT, Fujiki K, Hotta Y, Nakayasu K, Kanai A. Q118X lial microcysts in the first decade of life. risk of the dystrophy recurring within the mutation of M1S1 gene caused gelatinous drop-like corneal Patients may remain asymptomatic for graft. All tests should be medically neces- dystrophy: the P501T of BIGH3 gene found in a family with years, until epithelial cysts produce symp- sary and be important for the management gelatinous drop-like corneal dystrophy. Am J Ophthalmol toms, such as impaired visual acuity, pain of the case. 2000; 130: 119-20.

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001_ca0113_Corneal Atlas_JM.indd 10 12/28/12 3:42 PM Bowman’s

Reis-Bucklers (Corneal Dystrophy of dystrophy of Grayson and Wilbrandt and the Bowman Layer Type 1 and Granular honeycomb dystrophy of Thiel and Behnke Dystrophy Type III) may represent clinical variants of Reis- Gray, hazy, reticular deposits (crystalliza- Bucklers dystrophy.2 tion) typify this Bowman’s layer dystrophy.1-3 Management of the epithelial erosion The deposits will generally form symmetrical poses the major problem earlier in life. patterns in each eye and can easily be detect- Lubrication and appropriate supportive ed at the slit lamp. The opacities cause an therapy for erosions is helpful. When opaci- elevation of the surface epithelium and often fication of the cornea impairs vision signifi- create a visual disturbance by affecting surface Reis-Bucklers corneal dystrophy cantly, superficial keratectomy may improve epiuthelium as well as underlying stromal vision. Advanced cases require lamellar or areas of the cornea.1 It is believed to be an pattern of the central and mid-peripheral full thickness grafting but recurrence is com- autosomal dominant inherited corneal dystro- cornea with prominent corneal nerves. An mon. Over the past decade, researchers using phy associated with mutations in the TGFBI increased corneal thickness, irregular astig- molecular genetics have redefined dystrophies gene and in some cases with R124L and matism and decreased corneal sensation are of Bowman’s layer to include two distinct G623D mutations.1,3 Onset generally occurs hallmarks of the corneal malady.1,2,4 entities (Reis-Bucklers and Thiel-Behnke).1 by the first decade of life, typically four to Treatment and management includes topi- five years of age. This dystrophy is quite rare cal palliative therapy to reduce the frequency ICD-9 Codes and its incidence is unknown.1 of recurrent corneal erosion that includes Patient history and symptoms will invari- artificial tears, epithelial debridement (manual • 371.52 Other anterior corneal dystrophy ably include recurrent corneal erosion or laser) for the recurrent erosions, and kera- • 371.42 Recurrent corneal erosion (traumatic or spontaneous), pain, light sensi- tectomy or keratoplasty (lamellar or penetrat- • 371.0 Corneal scar and opacity code tivity and visual disturbance/distortion. The ing) for visual restoration.2 Recurrence of this range episodes of recurrent erosion tend to occur anterior corneal dystrophy is not uncommon 2 multiples times each year. A severe disrup- after corneal transplantation. Over-the- References tion of the basal epithelial cells and their counter pain relievers, lubricants, NSAIDs 1. Bushley DM, Afshari NA:Reis-Bucklers Corneal Dystrophy. stromal attachments accounts for the frequent and cycloplegics can be used for pain man- IN:Roy FH, Fraunfelder FW and Fraunfelder FT: Current Ocular erosions. The epithelium is loosely attached agement when necessary.4 There are no sys- Therapy. Saunders/Elsevier, New York,2008, pp. 391-392. 2 2. http://dro.hs.columbia.edu/reisbucklers.htm (Digital Reference since a basement membrane is focally absent. temic treatments for this dystrophy. of Ophthalmology Corneal & External Diseases, 2003). After 30, the erosions tend to become infre- 3. Laibson PR: Anterior Corneal Dystrophies and Corneal quent but acuity continues to decline due to Recommendations Erosions. IN: Leibowitz HM: Corneal Disorders: Clinical 3 Diagnosis and Management. WB Saunders Co, New York, increased superficial corneal opacification. This condition must be differentiated from 1984, pp 222-224. Early reticular superficial opacity will advance among several anterior stromal conditions. 4. http://legacy.revoptom.com/index.asp?page=2_13021.htm to a honeycomb, gray-white opacity fishnet To add to the confusion, anterior membrane (Review of Optometry Online).

Stroma

The stroma accounts for 90% of the Symptoms include decreased vision and corneal thickness and is composed mainly recurrent corneal erosions. of water, collagen and keratocytes. It is Granular corneal dystrophy (GCD) the specific arrangement of the tightly is a bilateral, autosomal dominant dis- bound collagen fibers in the stroma that ease associated with a mutation in the allows for the transparency and mechani- BIGH3 gene that leads to the deposi- cal strength of the cornea. Descemet’s tion of a hyaline material in the corneal membrane is a thick basement membrane stroma. It typically presents within the that is located at the posterior portion first decade of life with focal granular of the stroma. Four corneal dystrophies deposits between the anterior to mid including granular, lattice, Avellino and stromal regions. These opacities are dis- Reis-Bucklers have been linked to a muta- crete deposits located centrally, with clear Lattice corneal dystrophy tion in the TGF-1 gene, also known as cornea located in the periphery and clear the BIGH3 gene. cornea between deposits. The disease is tern that affects the central cornea and typically asymptomatic early on, but with Types of Stromal Dystrophies can increase over time. LCD is associated time the opacities can coalesce and lead Lattice corneal dystrophy (LCD) is the with a genetic mutation in the BIGH3 to decreased vision. Recurrent corneal most common of the stromal dystrophies. gene, resulting in deposits of amyloid erosions can occur in GCD but at a lower It has an autosomal dominant pattern within the anterior corneal stroma. Five incidence than in LCD. Three types of of inheritance and appears after the first subtypes of LCD have been identified. GCD have been described. GCD Type decade of life as a linear branching pat- LCD Type I is the classic form of LCD. I is the classic form of GCD.1 Avellino

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001_ca0113_Corneal Atlas_JM.indd 13 12/28/12 3:42 PM corneal stroma, keratocytes, sera or carti- dystrophies is observation and lubrica- lage, and is the most common variant of tion for corneal erosions if they occur. MCD worldwide. It is an inherited auto- Phototherapeutic keratectomy (PTK) and somal recessive condition. It is typically corneal transplants are options as vision noted during the first decade of life, char- becomes impaired, but the dystrophy can acterized by gray-white anterior stromal recur in the graft. lesions similar to GCD. There is severe stromal haze throughout the entire stro- Tests to Help Delineate Structure and ma and limbus to limbus, with patients Function typically developing severe visual loss by • Corneal topography—useful for cases the second to third decade of life. of stromal thinning causing irregularity Schnyder corneal crystalline dystrophy involving the corneal surface. (SCCD) is a slowly progressive autosomal • Anterior segment photography—use- Recurrent lattice dystrophy after corneal transplant dominant dystrophy that appears early in ful for documenting presence and extent life, but may not cause vision loss until of stromal changes. corneal dystrophy is GCD Type II. It is the fifth decade of life. It is linked to a • Pachymetry—useful for documenting linked to a mutation in the BIGH3 gene metabolic defect of corneal keratocytes thickness changes or variations. that leads to a deposition of both hya- that leads to crystalline lipid deposition. line and amyloid in the corneal stroma. Clinically the disease presents with a Pearls Typically, patients present in their second ring-shaped accumulation of fine needle • Monitor visual acuity with contrast decade with granular opacities like in shaped polychromatic crystal deposits changes. While Snellen acuity might be GCD, but later in the disease process within Bowman’s layer and the anterior satisfactory, loss of contrast will affect develop lattice lines as well. The disease stroma, and is often associated with a pre- visual quality more and result in patient was thought to have originated from a senile peripheral lipid arcus.4 complaints of performance. family in Avellino, Italy. However, GCD Congenital stromal dystrophy is an type II has now been reported in patients autosomal dominantly inherited con- from many other countries as well.2,3 dition that is caused by mutations in ICD-9 Codes Reis-Bucklers is GCD Type III the DCN (decorin) gene, leading to • 371.53 Granular corneal dystrophy (Bowman’s layer). It is characterized corneal haze and reduced visual acuity. • 371.54 Lattice corneal dystrophy by faint, gray-white superficial scarring Strabismus is common, and corneal thick- • 371.55 Macular corneal dystrophy patterns that are often greatest in the ness is increased. • 371.56 Crystalline corneal dystrophy central cornea. The patient may experi- Corneal fleck dystrophy (CFD) is a ence corneal erosions, photophobia and rare autosomal dominant dystrophy with irritation that can occur in early child- that is often asymptomatic. Photophobia, References hood. Analysis of the area with confocal reduced vision and recurrent erosions 1. Keefe KS, Milman T, Rodrigues MM, Hidayat AA. microscopy will often reveal the absence may occur. It is characterized by bilateral Conjunctival and Corneal Pathology. Albert and Jakobiec’s of Bowman’s layer. Vision can vary and is irregular shaped “flecks” of grayish mat- Principles and Practice of Ophthalmology, 3rd edition. 5 Saunders. 2008: 3592-3595. diminished by the severity of superficial ter in the posterior stroma. Posterior 2. Poulaki V, Colby K. Genetics of anterior and stromal corneal scarring and irregular astigmatism. amorphous corneal dystrophy is a rare dystrophies. Seminars in Ophthalmology, 2008; 23: 1,9-17. Macular corneal dystrophy (MCD) is condition characterized by bilateral sheet- 3. Bron AJ. Genetics of the Corneal Dystrophies: What we have learned in the past 25 years. Cornea 2000; 19(5): 699-711. the least common, but the most severe, like opacification of the posterior stroma 4. www.webeye.ophth.uiowa.edu. Birkholz ES, Syed NA, of the stromal corneal dystrophies. Three in association with corneal flattening and Wagoner MD. Corneal stromal dystrophies: A clinico- patho- subtypes of MCD have been described thinning It appears to be non-progressive logic review. based on the presence or absence of and patients are asymptomatic.6 5. http://www.ncbi.nlm.nih.gov/bookshelf www.dro. hs.columbia.edu/fleck. immunoreactive keratan sulfate within 6. Moshegov CN, Hoe WK, Wiffen SJ, Daya SM. Posterior various tissues. Type I does not have Treatment amorphous corneal dystrophy. A new pedigree with pheno- immunoreactive keratan sulfate in the The treatment for the stromal corneal typic variation. Ophthalmology. 1996 Mar;103(3):474-8.

Endothelium

The endothelium is the posterior layer mm2 in the periphery. With age, disease or Fuchs’ dystrophy is an autosomal domi- of the cornea, consisting of a single layer trauma, the cell density decreases. But with nant inherited disease that affects women of cells, about 5µm thick, bound together disease or trauma, this reduction may affect greater than men. It often presents in the and predominantly hexagonal in shape. corneal transparency, as some fluid then fifth to sixth decade of life as multiple Anteriorly, it is in contact with Descemet’s leaks into the cornea.1,2 The endothelial cor- central corneal guttata (excrescences of membrane and posteriorly with the aque- neal dystrophies, which result from primary Descemet’s membrane) associated with ous humor. It is the structure responsible endothelial dysfunction, include Fuchs’ pigment dusting on the endothelium. The for the relative dehydration of the corneal endothelial corneal dystrophy (FECD), condition spreads from the center toward stroma. In the normal adult eye the cell posterior polymorphous corneal dystrophy the periphery. As the endothelial cells fall, density varies from around 3,000 cells/mm2 (PPCD or PPMD) and congenital hereditary the remaining cells enlarge to cover the in the central cornea to about 2,000 cells/ endothelial dystrophy (CHED). gap. With the reduced number of endo-

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001_ca0113_Corneal Atlas_JM.indd 14 12/28/12 3:42 PM thelial cells, the pump function suffers.3 of stromal thickening or other disease This leads to stromal and epithelial edema, changes. and loss of visual acuity. Vision is typi- • Endothelial cell photography—allows cally worse upon awakening because of the tracking of endothelial cell density as well swelling induced by nighttime lid closure.4 as imaging for pleomorphism and poly- In more advanced stages, the epithelial megathism. microcysts later coalesce and form bullae, • Pachymetry—useful for documenting which can rupture, causing foreign body thickness changes or variations in cornea. sensation and pain, as well as exposing the cornea to the danger of infectious keratitis. Pearls Posterior polymorphous corneal dys- • Caution should be given for use of trophy (PPCD) is an autosomal dominant mitomycin C for procedures in Fuchs’ disorder with extremely variable expres- Endothelial cell imaging of guttata with no corneal patients as studies have raised the poten- sion. Three genes have been implicated in edema (left) and guttata with corneal edema in tial for further damage. PPCD (VSX1, COL8A2, TCF8), but the Fuchs’ endothelial dystrophy (right) • Monitor corneal grafts with pachym- evidence implicating VSX1 and COL8A2 etry to look for signs of rejection. is questionable.5 PPCD presents earlier gold standard for treatment of complica- Typically, the graft will thicken over time. than Fuchs’ and is typically more benign. tions relating to Fuchs’ dystrophy and • Fuchs’ dystrophy can be transmitted It is characterized by the early appear- other endothelial disorders. Long term, to the recipient of a corneal transplant. ance of vesicle-like lesions, bands or dif- the results have been impressive with Do not rule out this possibility if gut- fuse opacities. These opacities represent graft survival rates.9 Because of the com- tae, stromal, and epithelial edema are more diffuse thickenings in Descemet’s plications associated with the surgery, observed in a graft. membrane. PPCD can result in peripheral including healing time, graft failure and • The “A” for “automated” in DSAEK anterior synechiae so these patients must visual variability, the surgery has largely refers to the recent practice of the eye- be monitored for increased intraocular been relegated to second choice behind bank preparing the tissue in advance of pressure. Corneal edema is also a feature Descemet’s stripping automated endothe- the procedure instead of the surgeon of PPCD.6 lial keratoplasty (DSAEK). preparing the tissue. This practice reduces Congenital hereditary endothelial DSAEK is a surgery designed to handling problems with eyebank tissue in dystrophy (CHED) presents at or shortly replace the endothelium alone without the operating room. after birth with bilateral corneal edema. violating any of the overlying structures, The pathology of CHED is attributed including stroma and epithelium. The to endothelial cell degeneration during surgery has an even higher rate of success ICD-9 Codes gestation.7 There are two types: Type I compared to PK as long as graft adhesion • 371.57 Corneal guttata/Fuchs’ (CHED 1) is inherited as an autosomal- to the recipient cornea is successful.10 dystrophy dominant trait that presents with clear Newer techniques of reducing graft • 371.58 Posterior polymorphous corneas at birth. Type II (CHED II) is dislocation have made the surgery even dystrophy more common but more severe. It is safer. Graft rejection can be difficult to inherited as an autosomal-recessive trait, spot, so it is prudent to look for keratic 11 associated with nystagmus and corneal precipitates or diffuse corneal edema. References edema from birth. 1. http://medical-dictionary.thefreedictionary.com/ X-linked endothelial corneal dystrophy Tests to Help Delineate Structure and corneal+endothelium. (XECD) was first described in 2006.7 Function 2. http://emedicine.medscape.com/ article/1193591-overview 3. DeNaeyer G. Diagnosing and Treating Corneal Dystrophy, The course in XECD is slowly progres- • Anterior segment photography—use- Contact Lens Spectrum, June 2008. sive with intermittent corneal clouding ful for documenting presence and extent 4. Bass S. The genetics of corneal dystrophies. Rev of in the form of ground glass and moon Optom;143(11);2006. crater-like changes of the corneal endo- 5. Mehta JS, Vithana EN, Tan DT, Yong VH, Yam GH, Law RW, Chong WG, Pang CP, Aung T. Analysis of the posterior thelium. The corneal opacification may polymorphous corneal dystrophy 3 gene, TCF8, in late-onset be severe and associated with nystagmus. Fuchs endothelial corneal dystrophy. Invest Ophthalmol Vis In advanced cases, a subepithelial band Sci. 2008 Jan;49(1):184-8. keratopathy develops.8 6 Maumenee AE. Congenital hereditary corneal dystrophy. Am J Ophthalmol 1960; 50:1114-24. 7. Schmid E, Lisch W, Philipp W, Lechner S, Göttinger W, Treatment Schlötzer-Schrehardt U, Müller T, Utermann G, Janecke Early treatment includes hypertonic AR. A new, X-linked endothelial corneal dystrophy. Am J solutions during the day and nighttime Ophthalmol. 2006 Mar;141(3):478-487. 8. Thompson RW, Price MO, Bowers PJ, Price FW. Long-term hypertonic ointment. With extreme epi- graft survival after penetrating keratoplasty. Ophthalmology. thelial edema, bullae may form and cause 2003; 110: 1396-1402. pain and photophobia. Bandage soft con- 9. Terry MA, Hoar KL, Wall J, Ousley P. Histology of disloca- tact lenses may provide temporary relief. tions in endothelial keratoplasty (DSEK and DLEK): a labora- tory-based, surgical solution to dislocation in 100 consecutive However, these patients require care- DSEK cases. Cornea. 2006; 25: 926-32. ful follow-up care to reduce the risk of 10. Jordan CS, Price MO, Trespalacios R, Price FW Jr. Graft stromal neovascularization or infectious rejection episodes after Descemet stripping with endothelial keratitis. If at some point vision degrades keratoplasty: part one: clinical signs and symptoms. Br J Ophthalmol. 2009; 93: 387-90. Epub 2008 Nov 19. to a disabling level, surgery should be 11. Roh DS, Funderburgh JL.Impact on the corneal endothe- considered. No countable endothelial cells in severe Fuchs’ lium of mitomycin C during photorefractive keratectomy. J Penetrating keratoplasty had been the dystrophy Refract Surg. 2009; 25: 894-7.

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001_ca0113_Corneal Atlas_JM.indd 15 12/28/12 3:42 PM Degenerations

Keratoconus and pellucid marginal degen- eration are the two most common noninflam- matory corneal ectasias. Though they have very distinct features, early cases of either can be difficult to distinguish from the other.1 Keratoconus is seen in adolescents and young adults. Its onset is usually at puberty. There may be a family history in 10% of patients. It may be associated with atopic disease, Down syndrome, retinitis pigmen- Keratoconus with severe hydrops Slit lamp view showing apical thinning tosa, Leber’s congenital amaurosis, Marfan syndrome, Ehlers-Danlos, osteogenesis imper- Topography reveals vertical flattening with be corneal lipid deposition. fecta, or other noninflammatory connective horizontal steepening centrally, while inferior- tissue diseases.1 Patients present with blurred ly there is vertical steepening with horizontal Treatment vision and exhibit progressive myopia with flattening. This results in the classic bowed or Treatment options for keratoconus include irregular astigmatism. Slit lamp examination “bent bowtie” appearance on topography. collagen crosslinking, INTACS, full thick- reveals central or paracentral corneal thinning Acute hydrops is caused by a break in ness penetrating keratoplasty or deep anterior with protrusion of the cornea at the area of Descemet’s membrane with subsequent influx lamellar keratoplasty, and contact lenses. thinning. Usually, the apex of protrusion is of aqueous into the stroma. The break is just below the center of the cornea. The base self-repairing with resolution over weeks to Pearls of the cone is often outlined by a Fleisher months. Hyperosmotics may hasten recovery. • Keratoconus should be considered any ring, or an epithelial iron line. Vogt’s striae Rarely does the cornea thin enough to time visual acuity does not meet expected are stress lines in Descemet’s membrane at perforate. A penetrating keratoplasty (PKP) norms and other pathology is not obvious. the apex of the cone that disappear with digi- may be indicated if central scarring is severe, Not all inferior steepening seen on corneal tal pressure. Breaks in Bowman’s membrane although frequently CL fitting is easier after a topography is keratoconus and diagnosis can lead to scarring and subepithelial fibrosis. hydrops episode. PKP is contraindicated dur- should not be made solely on an axial topo- Acute ruptures in Descemet’s membrane can ing the acute phase of the disease. graphical map. Comprehensive diagnosis, lead to swelling or hydrops. Corneal topogra- In contrast to keratoconus and pellucid treatment, topography interpretation and fit- phy reveals inferior steepening with paracen- marginal degeneration, Terrien’s marginal ting videos are available at GPLI.org. tral thinning and elevation of the posterior degeneration usually occurs in middle age and anterior corneal surfaces.2 to elderly males. The thinning usually starts The onset of pellucid marginal degenera- superiorly and may occasionally be accom- ICD-9 Codes tion is usually similar to keratoconus, in the panied by inflammation. Neovascular vessels • 371.60 Keratoconus unspecified second through fourth decades of life.3 It is may cross the area of thinning and there may • 371.61 Keratoconus stable also bilateral, but in contrast to keratoconus, • 371.62 Keratoconus acute hydrops it is not inherited and does not have other • 371.00 Corneal scar unspecified typical ocular or systemic associations. The • 367.22 Irregular astigmatism corneal ectasia is located peripherally, usually • 371.48 Terrien’s marginal degeneration in the inferior cornea extending from 4 to 8 • 371.10 Iron deposits cornea o’clock positions. The thinning is typically 1 to 2mm wide, and located 1 to 2mm from the limbus. Maximal corneal protrusion References occurs just superior to the area of thinning, in 1. Cornea, second edition, volume 1, Krachmer, Mannis and contrast to keratoconus. The name “pellucid” Holland editors, Elsevier 2005 pages 955-974. means “clear.” The cornea is clear without 2. Holladay JT. Keratoconus detection using corneal topogra- phy. J Refract Surg. 2009 Oct;25(10 Suppl):S958-62. any iron rings or striae. Typically there is no 3. Joseph A, Fernandez ST, Ittyarath TP, Williams J. scarring; however, acute hydrops can occur as Keratectomy in terriens marginal degeneration. Indian J in keratoconus, which can then leave scarring. Keratoconus post hydrops Ophthalmol 1984;32:113-4.

Mechanical

ABRASION are always a possibility, especially in the and provide proper medical management to Corneal abrasions are a common form contact-lens wearing patient. Contact lenses hasten visual recovery. of injury most often resulting from ocular can compromise the corneal epithelium and trauma or inherently poor adhesion of the may facilitate the development of infectious Etiology epithelium to the underlying basement or non-infectious keratitis. It is important to Damage to the corneal epithelium results membrane. When the cornea is injured or recognize the signs and symptoms of corneal in loss of the connections of the surrounding damaged, sight-threatening consequences abrasion in order to ease the patient’s pain epithelial cells, but Bowman’s layer generally

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001_ca0113_Corneal Atlas_JM.indd 16 12/28/12 3:42 PM remains intact. Deeper involvement affecting paralyze the ciliary body and thus decrease stroma is rare and generally due to trauma ocular pain. Mild abrasion can be managed from a sharp or abrasive object. Often cor- with cyclopentolate 1%, while more severe neal flaps of various sizes and thickness can presentations may require homatropine 2% be seen.1 The speed by which the abrasion or 5%.4-6 resolves can be affected by a variety of factors Fortunately, the cornea heals very rapidly, including whether the patient has diabetes, but keep in mind that recurrent corneal ero- corneal denervation, dry eye, lagophthalmos, sion is possible even months to years later.1,6 previous recurrent corneal erosions or base- Lesions that are purely epithelial often heal ment membrane changes. In general, an epi- quickly and completely without scarring. The thelial insult will recover within 24-48 hours presence of subepithelial infiltration may and is accomplished by having the neighbor- be a sign of infection. Lesions such as these ing epithelial cells slide over and begin cover- Full thickness corneal laceration should be considered vision-threatening and ing the wound. Common causes of mechani- may warrant culturing and the use of fortified cal abrasions are fingernail, paper, foreign at q2h for 24 hours or more conservative at antibiotic therapy.2 body, curling iron, mascara brush, plant or q.i.d. Antibiotic ointments can be liberally To prevent recurrent erosion and reduce other vegetative source, and contact lens.1 applied during the day or augment drops by corneal edema, a hypertonic solution or oint- using them at bed-time.2 Ointments tend ment may be prescribed as long as the cornea Presentation to provide better barrier and lubricating has re-epithelialized.2 Examine both eyes care- Slit-lamp biomicroscopy of the injured function, but will temporarily blur vision. fully for any evidence of corneal dystrophy. cornea reveals epithelial disruption and dif- Debridement of loose or hanging epithelium Prevention includes wearing safety glasses fuse corneal edema. In severe cases, when is necessary to enhance healing.1,2 Therapeutic for athletes and industrial workers.7 Eyelids edema is excessive, folds and inflammation in soft contact lenses can be used in abrasion should be adequately taped for any patient Descemet’s membrane may be visible. A drop management. Large abrasions have been undergoing general anesthesia. of topical anesthetic may aid in the examina- found to heal more quickly under the protec- tion of the ocular structures. The corneal tion of a bandage contact lens.3-5 The use of Recommendations abrasion should be documented for location, bandage contact lenses, however, does not • If the patient’s blepharospasm is intense size, shape and depth with either a drawing come without risk, including additional ocu- and visual acuity cannot be obtained, instill or photograph, making any notation of an lar irritation, epithelial and stromal edema, one drop of anesthetic onto the bulbar infiltrative process. This visual documenta- sterile infiltrates and hypopyon, infection and conjunctiva. This should allow you to imme- tion will aid in follow-up to chart the healing ulceration.4 Bandage contact lenses should be diately record visual acuity. A new (sterile) progress. Fluorescein dye can be instilled to avoided in all situations where plant injury bottle of anesthetic should be used if a pen- identify the corneal defect. The newly created or false fingernails is suspected because of the etrating injury is suspected. wound appears bright green compared to the risk of fungal keratitis.5 • Always remember to evert the upper rest of the cornea because the dye accumu- Bed rest, inactivity and over-the-counter lid to check for residual foreign substance. lates in the defect where cell loss and disrup- analgesics can be used to manage pain.6 Cylcoplegics will make the patient more tion have occurred.1,2 The anterior chamber Topical non-steroidal anti-inflammatory comfortable and decrease the likelihood of should be observed and any anterior chamber drops can be a useful adjunctive therapy traumatic iritis developing. Steroids are NOT reaction should be noted. for the management of pain from corneal always necessary and should be avoided ini- Patient symptoms include eye pain, for- abrasion and often obviate the need for oral tially.2 eign-body sensation, photophobia, blepharo- pain medications. These medications provide • If using a bandage lens, make sure that spasm and tearing. The time, place and activ- patient comfort and do not adversely affect you are using a lens of appropriate Dk/t to ity surrounding the injury should be noted. corneal healing time.6 reduce the likelihood of corneal swelling. For medical and legal purposes, the visual Oral analgesics may be necessary for • Avoid bandage lenses if injury involves acuity should be taken before performing any pain not controlled by topical medications. plant material or false fingernails.1,2 procedures. Differentials include recurrent These include aspirin, ibuprofen, toradol • Patching is rarely necessary. Never patch corneal erosion, herpes simplex keratitis, and and tramadol. These medications provide contact lens related abrasions or injuries confluent superficial punctate keratopathy.2 both analgesic as well as anti-inflammatory involving vegetative material or false finger- effects. Analgesics such as acetaminophen nails. Patients should be re-evaluated within Treatment lack the anti-inflammatory component. The 24 hours.2 There are several treatment options for cor- above anti-inflammatory medications can • Encourage the use of nighttime hyperos- neal abrasions, including bandage soft contact be prescribed in combination with narcotic motic ointments once the cornea has re-epi- lenses, topical antibiotic ointment and drops, analgesics such as codeine or hydrocodone. thelialized up to three-six months post insult topical non-steroidal and steroidal anti- Cycloplegic agents should be prescribed to in cases involving a significant abrasion. inflammatory drops, cycloplegic preparations, and hypertonic drops and ointments. Topical ICD-9 Codes antibiotics are the mainstay of corneal abra- • 371.42 recurrent corneal erosion sion therapy. There is a risk of infection with • 37.82 corneal disorder due to contact any open wound or defect involving the lens wear basal lamina. Topical antibiotics—such as • 918.1 significant injury to the cornea polymyxin B/trimethoprim, aminoglycosides or fluoroquinolones—are all reasonable for 3 providing prophylactic antibiotic coverage. References Depending on the extent and severity of 1. Arunagiri G, Trikha R. Corneal Abrasions, Contusions, presentation, dosing might be more aggressive Graphite deposits from gunshot injury Lacerations, and Perforations. IN:Roy FH, Fraunfelder FW and

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001_ca0113_Corneal Atlas_JM.indd 17 12/28/12 3:42 PM Fraunfelder FT: Current Ocular Therapy. Saunders/ Elsevier, and eyelid gold weights for seventh nerve Treatment New York,2008, pp. 359-62. 2 2. Cullom RD, Chang B. Trauma: Corneal Abrasion. In: palsies can be helpful. A careful history will determine the Cullom RD, Chang B eds. The Wills Eye Manual: Office and mechanism of injury. A lid speculum may Emergency Room Diagnosis and Treatment of Eye Disease. Recommendations aid in the evaluation. Some attempt should Philadelphia, PA: JB Lippincott, 1994: 22-23. 3. Gurwood AG. Managing corneal abrasions. www.optom- • Close observation is necessary when an be made to determine the size, shape, weight, etry.co.uk. infection is present or a high probability of velocity, force and composition of the 4. Webster RG. Corneal Injuries. In: Smollen G, Thoft RA. The infection exists. object.1 Visual acuity assessment should be Cornea. Boston: Little, Brown & Co. 1987: 517-527. 5. Fujikawa LS, Nusenblatt RB. Recurrent and chronic corneal • Floppy eyelid syndrome and Parkinson’s performed before any procedure is attempted. epithelial defects. In: Abbott RL, ed. Surgical Intervention disease can also cause a poor blink and result Topical anesthetic agents can control pain in Corneal and External Diseases. New York, NY: Grune & in exposure staining.2 and blepharospasm to aid in examination.1 Stratton. 1987: 59-67. 6. Sowka J, Gurwood A, Kabat A. Handbook of Ocular • Vaseline petroleum jelly preparations Slit lamp examination will determine the Disease Management. Rev Optom. applied to the periocular skin can be thera- location, depth and whether there are any 7. Bloom B. Corneal Abrasion. IN: Maguire JI, Murchison AP peutic in certain cases of ectropion and may self-sealing lacerations. Evert the eyelids and and Jaeger, EA. Wills Eye Institute 5-Minute Ophthalmology Consult. Lippincott, Wilkins and Williams, 2012, pp. 218-19. obviate the need for surgery. carefully inspect the fornices. With conjuncti- val laceration be certain Tenon’s membrane is intact to rule out scleral laceration or perfora- ICD-9 Codes EXPOSURE KERATOPATHY tion of the globe.1 • 370.34 Exposure keratitis Exposure staining of the ocular surface Corneal foreign body can be removed by appears to be associated with incomplete • 374.2 Lagophthalmos use of a fine forceps or foreign body spud at blinking with improper lid closure and move- • 374.5 Floppy eyelid syndrome the slit lamp after topical anesthetic is applied ment and can have several different causes.1,2 • 374.0 Entropion to the eye. Multiple superficial foreign bod- Whenever possible, correcting any underlying • 374.1 Ectropion ies may be irrigated.1 Broad spectrum topi- problem will often provide immediate relief cal antibiotics should be used to treat the and prevention is critical in many cases to resultant defect after assessing the size of the maintain normal corneal clarity.2 References defect (see Corneal Abrasion section, p. 16-17, 1. McMonnies C: Incomplete blinking: exposure keratopathy, lid 1 wiper epitheliopathy, dry eye, refractive surgery and dry contact for a more complete discussion). Similar treat- Etiology lenses. Contact Lens and Anterior Eye, 30(1), 37-51. ment employed for any epithelial defect or Major causes of exposure keratopathy 2. Ehlers JP and Shah CP. Exposure Keratopathy. In: Ehlers JP abrasion can be used for pain management. and Shah CP eds. The Wills Eye Manual: Office and Emergency relate to lid malposition or deformity (eye- Room Diagnosis and Treatment of Eye Disease. Philadelphia, Conjunctival (and sometimes corneal) foreign lid scarring from trauma or herpes zoster), PA: Wolters/Kluwer/JB Lippincott, 2008: 55-56. body can usually be removed successfully ectropion, or chemical burns.1,2 Other causes 3. Feldman B. Exposure Keratopathy. IN: Maguire JI, Murchison using fine forceps or a cotton-tipped applica- stem from lid surgery or acquired defects/ AP and Jaeger, EA. Wills Eye Institute 5-Minute Ophthalmology tor soaked in topical anesthetic.1 Residual deformities (especially ptosis repair and Consult. Lippincott, Wilkins and Williams, 2012, pp. 292-3. and not easily accessible conjunctival foreign blepharoplasty), nocturnal lagophthalmos, bodies may sometimes be left without undue sedation and altered mental status, proptosis FOREIGN BODY harm unless they are infectious or pro-inflam- or cranial nerve palsies that affect the lid or A variety of materials in the environment, matory.1 Deep injury to the posterior cornea eye.2 Exposure keratopathy is commonly seen often metallic or organic, resulting in foreign may perforate the cornea and require sutures, in Parkinson’s disease, Bell’s palsy, neuro- body of the eye can be found on the surface glue and or a bandage contact lens. trophism and ectropion.3 of the cornea or conjunctiva, or even be intraocular/intraorbital.1,2 Fortunately, the Recommendations Treatment majority of foreign bodies encountered in • Carefully examine the anterior chamber A careful medical history may uncover clinical practice lodge on either the cornea or and iris for any signs of intraocular foreign previous Bell’s palsy, lid surgery, anterior conjunctiva and do not enter the eye or orbit. body. A low intraocular pressure or anterior segment trauma or thyroid disease.2 Careful A dilated eye examination must be performed chamber shallowing effects may indicate cor- evaluation should include assessment of to rule out any posterior segment involve- neal penetration.1 eyelid closure/laxity and corneal exposure. ment and appropriate testing such as B scan • Any infiltrate suggesting an infectious Always check to be certain there is no evi- ultrasonography, computed tomography (CT process must be managed with appropriate dence of reduced corneal sensation increas- scan) of the orbit or ultrasonographic biomi- antibiotic therapy.1 ing the risk of corneal complications.2 A slit croscopy (UBM) to rule out an intraocular or • Remove any rust as completely as pos- lamp examination will often uncover any intraorbital foreign body should also be done sible. This might require allowing time for the tear film abnormalities, corneal integrity when necessary.1 rust to migrate to the surface of the cornea issues or other external or anterior chamber with removal of the rust after several days in reaction.2 Lubrication is essential for any Presentation order to minimize scarring.1 Routine dilated sedated or obtunded patient.2 Artificial tears, Patients will generally provide a history of examination, gonioscopy and intraocular lubricating ointments and gels are the main- trauma and foreign body sensation with any pressure checks may be necessary depending stay of treatment. Punctal plugs can also foreign body of the cornea and/or conjunc- on the extent of the injury. be used when necessary for neurotrophic tiva. Additional symptoms include photopho- states or other dry eye related complica- bia and lacrimation. A slit lamp examination ICD-9 Codes tions. Eyelid taping and patching can be will reveal a single or multiple foreign bodies. • 930.0 Corneal foreign body tried when the condition is believed to be Metallic corneal foreign body will produce • 930.1 Foreign body in the conjuncti- temporary. In severe, recalcitrant cases, with rust. The eye is injected with eyelid edema val sac progressive corneal deterioration, eyelid and punctate keratopathy is seen with a for- • 360.60 foreign body-intraocular reconstruction, partial tarsorrhaphy and eign body under the lid. An anterior chamber • 930.8 Foreign body in other and com- amniotic membrane transplants can be ben- reaction is possible with any significant invov- bined sites on external eye eficial.2 Orbital decompression for proptosis lement.1

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001_ca0113_Corneal Atlas_JM.indd 18 12/28/12 3:42 PM References Office and Emergency Room Diagnosis and Treatment of Eye 2. Onofrey B: Superficial ocular foreign body. In: Onofrey 1. Ehlers JP and Shah CP. Corneal and Conjunctival Foreign Disease. Philadelphia: Wolters/Kluwer/ JB Lippincott, 2008: BE, Skorin L and Holdeman NR eds. Ocular Therapeutics Bodies. In: Ehlers JP and Shah CP eds. The Wills Eye Manual: 16-18. Handbook. Lippincott-Raven, Philadelphia 1998: 303-4.

Chemical

TOXIC/SOLUTION KERATITIS are symptomatic with lens wear. Chemical toxicity can result from any • Obtain a careful history in contact lens contact lens solution, and care products are wearers including solution used, lens type and an under-recognized cause of intolerance to replacement frequency. contact lens wear. A wide range of signs and symptoms are possible, ranging from mild sensitivity from a micro-punctate keratopathy ICD-9 Codes to significant discomfort as a result of diffuse • 370.21 Punctate keratitis corneal staining. The patient may experience • 370.3 Certain types of keratoconjunc- a foreign body sensation and photophobia. tivitis Alkali burn status post stem cell transplant with clear visual axis The condition is generally bilateral and disap- • 370.4 Other and unspecified kerato- pears after removing the offending agent. conjunctivitis results from contact with hot liquids, gases Etiology References or molten metal. Cell death from thermal Virtually any contact lens care product has 1. Carnt N, Jalberty J, Stretton S et al: Solution toxicity in soft burns is generally limited to the superficial the potential to create a toxic effect to the contact lens daily wear associated with corneal inflammation. epithelium, but thermal necrosis and deeper ocular surface, especially inadvertent applica- Optometry and Vision Science: April 2007 84 (4): 309-315. penetration can occur.4 tion of non-neutralized hydrogen peroxide 2. Szczotka-Flynn L, et al. Arch Ophthalmol 2007:125(4):488. • Ultraviolet burns: Punctate keratitis results directly in the eye. A less severe adverse ocu- from an epithelial injury. Delayed pain is sec- lar response of diffuse punctate staining and CHEMICAL BURNS ondary to actinic keratosis.3 conjunctival redness has been associated with Chemical injuries following exposure • Alkali burns: Since alkali substances are wearing contact lenses, especially silicone to solid, liquid or gas forms of corrosives more lipophilic, they penetrate more rapidly hydrogel lenses and using certain multipur- have the potential to permanently damage than acids.1,3 The damaged tissues stimulate pose disinfecting solutions.1 Solution-induced the ocular surface (unilateral or bilateral). an inflammatory response that damages staining should not be classified as a toxic Pathophysiological cascades that may influ- the tissue further by release of proteolytic response as this finding (what the fluorescein ence the final visual outcome include: 1) enzymes (liquefactive necrosis) and alkali staining actually represents) is being debated. ocular surface injury and repair, 2) stromal substances can pass into the anterior chamber matrix repair and or ulceration and 3) cor- rapidly, exposing the crystalline lens, ciliary Presentation neal inflammation.1,2 Ocular burn severity body and trabecular meshwork.3,4 When the Patients experiencing a toxic response correlates to exposure duration and noxious pH value is above 11.5, irreversible damage often present with symptoms of stinging, tear- agent.4 Specifically, chemical burn severity occurs.4 ing, burning, dryness and a decreased wearing relates to pH, duration, solution quantity and • Acid burns: Acid burns cause protein time. Signs include redness, conjunctival permeability.3 Ocular burns caused by acids coagulation of the epithelium, thereby limit- chemosis, follicular response, variable corneal are generally less severe compared to alkali ing in most cases further penetration and pro- staining patterns and infiltrates. burns due to the natural buffering capacity of gression beyond the superficial cornea.4 the corneal stroma and the barrier to penetra- Treatment tion formed by coagulated epithelial cells.1 Presentation Palliative therapy should include the use Immediate therapy should include prompt Patients will often give a history of a liquid of artificial tears (non-preserved) or gels/ irrigation and removal of any remaining or gas being splashed or sprayed into the eye ointments and antibiotic prophylaxis when reservoir of chemical contact.2,3 Ocular burns or of particles falling into the eye.4 The local indicated for more severe forms of coalesced represent 7-18% of ocular trauma. The vast Poison Control Center may be an invalu- corneal staining. Topical corticosteroids majority are chemical burns that occur in the able resource in determining the nature of or antibiotic/steroid combinations may be industrial/occupational setting.4 the chemical when unknown.3 Common warranted especially when there is an infiltra- tive response, assuming the risk of corneal Etiology infection has been carefully considered and Any noxious agent or radiant energy (ther- ruled out. A change in solution is advised mal or ultraviolet) has potential to irritate/ to remove the offending agent, and some injure the ocular surface and produce other clinicians will switch from a multipurpose more severe forms of anterior segment abnor- solution to an oxidative hydrogen peroxide mality. This includes alkali (i.e., lye, cements, system. plasters, airbag powder), acids, solvent, deter- gents and irritants (i.e., mace).3 Burns damage Recommendations tissues primarily by denaturing and coagulat- • Some mild forms of toxicity/hypersensi- ing cellular proteins and through vascular tivity are barely detectable and the use of vital ischemic changes.1-4 stain is essential in evaluating patients who • Thermal burns: Injury from radiant energy Acute alkali burn

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001_ca0113_Corneal Atlas_JM.indd 19 12/28/12 3:42 PM complaints include pain, foreign body sensa- tival or limbal stem cell transplants from the tion, blurred vision, excessive tearing and fellow eye may be necessary for injuries that photophobia.2-4 A wide range of findings will fail to heal within several weeks to months.3,4 be influenced by the severity of the injury but Prevention includes ocular protection in may include particles in the fornix, conjuncti- industrial settings. Appropriate masks and val inflammation, perilimbal/limbal ischemia, breathing apparatus should be used when corneal epithelial defects, stromal haze, ante- working with noxious fumes or gases.5 rior chamber reaction, adnexal damage/scar- ring, increased intraocular pressure, periocular Anhydrous ammonia burn with conjunctival flap to Recommendations skin burns and corneal perforation/melt.3,4 treat a healing epithelial defect • If an epithelial defect is not appreci- Severe burns may also cause a pronounced ated initially, re-instill fluorescein; the defect chemosis, conjunctival blanching and local Therapeutic agents depending on severity may be slow to take up dye. If the entire necrotic retinopathy resulting from direct include antibiotic for significant epithelial epithelium has sloughed, Bowman’s layer penetration of alkali through the sclera.2,3 defects, cycloplegia, topical steroids, pain remains and takes up fluorescein poorly.3 The clinical appearance can be deceiving and medication and anti-glaucoma agents when Closely monitor IOP. An immediate rise in rapidly change. The initial status of the cor- IOP is elevated.2-4 Intraocular pressure checks pressure can result from collagen deforma- nea can appear clear or stable, yet thin/melt can be difficult; digital assessment may be tion and shortening that directly affects the within hours.5 necessary.5 Using steroids beyond seven anterior chamber. Prolonged increases in IOP days after severe burns is controversial and are directly related to the degree of anterior Treatment will occasionally result in perforation and chamber inflammation.4 The initial physical examination should ulceration of the cornea due to the effects assess potential life-threatening injuries. Initial on wound repair.1 Conformer rings may examination of the eye may be limited to prevent symblepharon formation.5 Topical ICD-9 Codes pH values and acuity determination.2,3 After artificial tears and lubricating ointments play • 370.24 Photo keratitis copious irrigation for 20 to 30 minutes with a significant role in managing these cases. • 940.1 Chemical burn of the eyelid saline or water, a full ocular examination is Other cases may require lysis of conjunctival and periocular area essential.3 Fluorescein and pH evaluation is adhesions, bandage contact lenses, aprotinin • 940.2 Alkaline chemical burn of cor- necessary after a short equilibration period. (inhibits proteinase activity), topical hepa- nea and conjunctival sac Ocular irrigation with lactated Ringer’s solu- rin (re-opening of occluded blood vessels), • 940.3 Acid chemical burn of cornea tion (1-2L) or other available intravenous ascorbate (10%) and citrate for alkali burns.1 and conjunctival sac solutions should be continued in the emer- With any melting processes of the cornea, • 940.4 Other burn of cornea and con- gency facility. Attention should be given to collagenase inhibitors such as tetracycline and junctival sac the clarity of the cornea, degree of limbal tissue adhesives may be used.3 Glaucoma fol- • 940.5 Burn with resulting rupture and ischemia and intraocular pressure. Lid ever- lowing chemical burns will generally respond destruction of eyeball sion is important to detect and eliminate any to conventional agents (oral and topical). particles or foreign bodies. Immediately fol- Additionally, emergent patch grafts may be lowing the injury, it is important to estimate necessary. Added measures such as conjunc- References and grade the severity of limbal stem cell 1. Wagoner MD: Chemical injuries of the eye: current con- injury and debride any necrotic epithelium to cepts in pathophysiology and therapy. Surv Ophthalmol. 1997; 1 41(4): 275-279. allow normal tissue to re-epithelialize. The 2. Roy FH: Alkaline Injury. In: Roy FH, Fraunfelder FW and grading can be accomplished by assessing Fraunfelder FT: Current Ocular Therapy. Saunders/Elsevier, the degree of limbal, conjunctival and scleral New York, 2008, pp. 277-78. 3. Ehlers JP and Shah CP. Chemical Burn. In: Ehlers JP and ischemia and necrosis, and by evaluating the Shah CP eds. The Wills Eye Manual: Office and Emergency penetration of the noxious agent by assessing Room Diagnosis and Treatment of Eye Disease. Philadelphia, the clarity of the stroma and any additional PA: Wolters/Kluwer/JB Lippincott, 2008: 12-15. anterior segment abnormalities.1,2 If the lim- 4. http://emedicine.medscape.com/article/798696-overview. 5. Dua HS, Said DG: Chemical Burns. IN: Maguire JI, bus is affected significantly, the cornea may Murchison AP and Jaeger, EA. Wills Eye Institute 5-Minute develop epithelial defects and conjunctival Chemical burn to cornea from calcium hydroxide Ophthalmology Consult. Lippincott, Wilkins and Williams, invasion onto the cornea.3 based white wash paint 2012, pp. 148-9.

Inflammatory Corneal Conditions

STAPH MARGINAL KERATITIS/CLPU diagnosis. Differentiating a CLPU from early CLPUs are different from peripheral Etiology stage MK is primarily based on clinical judg- marginal ulcers caused by S. aureus exotoxins A contact lens-induced peripheral ulcer ment rather than on microbiologic or histo- that are often found in the corneal periphery, (CLPU) typically presents as a single well-cir- pathologic investigations.2 However, there is which are more often oval and may be associ- cumscribed, circular, dense focal corneal infil- often an overlap in the signs and symptoms, ated with corneal vascularization. However, trate involving the anterior corneal layers.1 It which can complicate the diagnosis. A critical a recent study showed that bacterial carriage is sometimes difficult to distinguish between sign, however, is the response of the presenta- on contact lenses during extended wear a CLPU and microbial keratitis (MK), so MK tion immediately after lens wear is discontin- predisposes the wearer to the development is always considered part of the differential ued.3 of corneal inflammatory events including

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001_ca0113_Corneal Atlas_JM.indd 20 12/28/12 3:42 PM contact lens-associated red eye (CLARE), con- tion of the lacrimal gland and aid in deter- tact lens peripheral ulcers, and asymptomatic mining if the condition is aqueous deficient infiltrates.4 Any diagnosis of CLPU must be dry eye or evaporative dry eye. The system monitored carefully to ensure it is not MK.3 also tests IgE levels that can mask as dry eye. Presentation Treatment Patients may complain of redness, discom- There is no single method for determin- fort, light sensitivity, tearing and foreign body ing if a patient is a candidate for dry eye sensation. These symptoms are milder than therapy. Patient symptoms and clinical in microbial keratitis; however, suspicion signs should be considered when deciding must always be high in contact lens-wearing on therapeutic intervention. The manage- patients of a possible underlying microbial Lissamine green staining dry eye ment of dry eye disease encompasses both cause. pharmacologic and non-pharmacologic Slit-lamp examination will demonstrate depending on its symptoms or complica- approaches, including avoidance of exacer- mild to moderate conjunctival injection tions.6 There are two etiopathogenic distinc- bating factors, eyelid hygiene, tear supple- near the corneal infiltrate and a round, focal tions of dry eye: aqueous deficient dry eye mentation, tear retention, tear stimulation, peripheral infiltrate approximately 2mm or and evaporative dry eye. There are numerous and anti-inflammatory agents.9 Artificial tears less. The overlying small epithelial defect causes for each. Dry eye is the single most are the mainstay of dry eye disease therapy stains with fluorescein. Anterior chamber common complaint among contact lens but, although they improve symptoms and reaction, if present, is very mild. A significant wearers. It is the number one reason patients objective findings, they do not resolve the anterior chamber reaction raises the suspicion discontinue contact wear. Approximately underlying inflammation. Topical corticoste- of microbial keratitis. 34% of patients discontinue contact lens wear roids are effective anti-inflammatory agents, at least once, most frequently because of dry but are only used short-term because of their Treatment eye symptoms. adverse-effect profiles. Topical cyclosporine— The initial management of CLPU involves currently the only approved pharmacologic discontinuing contact lens wear. Patients are Presentation treatment for DED—is safe for long-term use often started on topical antibiotics such as a Physical examination includes visual and is a disease-modifying therapy. fourth-generation fluoroquinolone and fol- acuity measurement, external examination, Treatment selection is guided primarily by lowed with serial examinations for evidence and slit-lamp biomicroscopy.7 Additional DED severity.10 Replacement of tear volume of either improvement or worsening of the diagnostic tests may be performed to assess with nonpreserved wetting agents and stan- condition to rule out microbial keratitis. tear film instability, ocular surface damage, dard typical anti-inflammatory corticosteroid Topical steroids should generally be with- and aqueous tear flow. Tear film instability and/or cyclosporine A continues to be held until the epithelial defect is healed and is commonly evaluated by performing a tear central current conventional therapy for dry there is no evidence of fungal, protozoan, or breakup time (TBUT) test. Ocular surface eye.11 Autologous serum eye drops have been herpetic infection. In staph-induced marginal damage is commonly assessed by staining reported to be effective for the treatment of keratitis where severe inflammation exists, with rose bengal, lissamine green or fluores- severe dry eye-related ocular surface disorders antibiotic and corticosteroid combination cein dye. Abnormal corneal and/or conjunc- (Sjögren’s syndrome).12 With appropriate drops or ointments can be rubbed into the tival staining patterns, observed on slit-lamp management of the ocular surface condi- lid margins following lid scrubs. examination, are a sign of damage. Aqueous tions that produce dry eye, careful selection Oral therapy with tetracycline 250mg four tear flow is commonly assessed by perform- of contact lenses and solutions, and vigilant times a day or doxycycline 100mg twice a ing a Schirmer test. While helpful in making follow-up, successful CL wear should be day or minocycline 50mg twice a day may be the diagnosis, diagnostic test results generally achievable for the dry eye patient.13 needed for more severe infections. The lesion correlate poorly with symptoms.8 There are generally heals with a small, mid-stromal scar two new diagnostic tests available com- ICD-9 Codes and recurrences are common. mercially: The TearLab Osmolarity System • 370.33 Keratoconjunctivitis sicca, not is intended to measure the osmolarity of Sjogren’s ICD-9 Codes human tears to aid in the diagnosis of dry • 370.23 Filamentary keratitis • 370.00 Corneal ulcer, unspecified eye disease in patients suspected of having • 370.01 Marginal corneal ulcer dry eye disease. Advanced Tear Diagnostics has just launched a Tear Assay system that UVEITIS measures the quantity of lactoferrin in dry Etiology DRY EYE eye patient populations. Lactoferrin is a Anterior uveitis is the most common form Etiology marker that will indicate the secretory func- of uveitis encountered in general ophthalmic From the 2007 DEWS Report: “Dry eye is a multifactorial disease of the tears and ocular surface that results in symptoms of discomfort, visual disturbance and tear film instability with potential damage to the ocu- lar surface. It is accompanied by increased osmolarity of the tear film and inflammation of the ocular surface.”5 Dry eye disease is a common disorder that especially affects adults and women and can cause quality of life impairment comparable to migraine, short- ness of breath and chronic renal insufficiency, Contact lens-associated red eye (CLARE) Dry eye

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001_ca0113_Corneal Atlas_JM.indd 21 12/28/12 3:42 PM practice with acute anterior uveitis (AAU) forward. Discontinuing contact lens wear accounting for 90% of all cases of uveitis.14 and treatment with frequent preservative-free Half of all cases of AAU are HLA-B27 posi- ocular lubricants is the initial therapy. In tive. It is often self-limiting, but can, in some most cases, this palliative treatment will suf- cases, lead to complications such as posterior fice. In more severe presentations where the synechiae, cataract, glaucoma and chronic patient is experiencing significant discomfort, uveitis.15 it might be necessary to concurrently treat the patient with steroid drops during the day Presentation and steroid ointment in the evening. Dosing Symptoms of unilateral pain, photo- schedule will vary depending on the case phobia, redness and watering develop over presentation. The treatment for SLK is gener- one to two days, with little or no effect on ally more involved and can require thermo- vision.16 Pain and photophobia may pre- Keratic precipitates in acute anterior uveitis cautery, conjunctival resection or chemical cede slit lamp signs, due to unseen ciliary cauterization with silver nitrate solution. body inflammation. Signs depend on the corneal staining combined with tarsal and severity of the inflammation but typically superior limbal hypertrophy. There may be include circumlimbal flush, miotic pupil, an association with atopy, contact lens-related ICD-9 Codes anterior chamber cells and flare, and small, trauma, hypoxia, hypersensitivity responses • 370.21 Punctate keratitis fine keratic precipitates (KP). The pupil to proteins deposited on contact lens and • 371.82 Corneal disorder due to may appear sluggish and may be small and chemical preservatives, especially thimerosal contact lens irregular. In more severe attacks, posterior in lens care systems. SLK on the other hand • 372.10 Chronic conjunctivitis, synechiae, peripheral anterior synechiae, is seen in older populations of women and unspecified corneal edema, spill-over vitritis and macular might be related to thyroid eye conditions or edema may be seen. The intraocular pressure other autoimmune diseases. (IOP) generally falls slightly with attacks but References 17 1. Sweeney DF, Sankaridurg PR, Holden B, et al. A typical occasionally it may increase. Presentation presentation of contact lens induced peripheral ulcers–mul- Both conditions will manifest with tiple focal corneal infiltrates. ARVO poster 2002. Treatment punctate staining of the superior corneal 2. Aasuri MK, Wenkata N, Kumar VM. Differential diagnosis In the majority of cases of AAU, espe- epithelium, although CLSLK is often a more of microbial keratitis and contact lens-induced peripheral ulcer. Eye Cont Lens 2003; 29: S60-62. cially those of mild to moderate intensity, aggressive presentation. SLK is typically a 3. Diec J, Carnt N, Tilia D, et al. Prompt diagnosis and topically applied steroids are the mainstay of bilateral condition and is clearly evident treatment of microbial keratitis in a daily wear lens. Optom treatment.18 Dosage regimens for topically upon visualization. CLSLK is usually mon- Vis Sci 2009; 86: E904-907. applied steroids depend on the severity of ocular with tremendous variability of presen- 4. Willcox M, Sharma S, Naduvilath TJ, et al. External ocu- lar surface and lens microbiota in contact lens wearers with AAU but all strategies must include aggressive tation. Patients with CLSLK may complain corneal infiltrates during extended wear of hydrogel lenses. initial treatment with the goal of bringing the of contact lens intolerance, light sensitivity, Eye Contact Lens 2011; 37: 90-95. inflammation under control quickly. Hourly burning, redness, watering and pain. Upon 5. 2007 Report of the International Dry Eye Workshop instillation for the first few days is typical. lifting the upper lid, an intense area of local- (DEWS). The Ocular Surface 2007; 5: 75-88. 6. Fonseca EC, Arruda GV, Rocha EM. Dry eye: etio- A loading dose to achieve a therapeutic ized injection at 12 o’clock with associated pathogenesis and treatment. Arq Bras Oftalmol 2010; 73: concentration at an early stage is often recom- loose and boggy bulbar conjunctiva can be 197-203. mended.19 observed. An irregular epithelial surface, 7. American Academy of Ophthalmology. Preferred practice Mydriatic/cyclopegic agents may also be punctate staining with fluorescein, and pattern: dry eye syndrome. www.aao.org/education/ guide- lines/ppp/upload/Dry_Eye_Syndrome-2.pdf. added to reduce pain and prevent the devel- subepithelial infiltrates may be found on 8. Perry HD. Dry eye disease: Pathophysiology, classifica- opment of posterior synechiae. Topical corti- the superior aspect of the cornea in associa- tion and diagnosis. Am J Manag Care 2008; 14: S79- S87. costeroids and cycloplegic agents remain the tion with hyperemia of the superior bulbar 9. Behrens et al. Dysfunctional Tear Syndrome: A Delphi cornerstones of treatment for AAU. conjunctivae. There may be significant Approach to Treatment Recommendations. Cornea 2006; 25: 900–907. neovascularization and fibro-vascular pannus 10. Lemp MA. Management of dry eye. Am J Manag Care ICD-9 Codes extending into the superior cornea. Evert the 2008; 14: S88-S10. • 364.00 Acute and subacute iridocycli- eyelids on all contact lens patients, paying 11. Gumus K, Cavanagh DH. The role of inflammation and antiinflammation therapies in keratoconjunctivitis sicca. Clin tis, unspecified careful attention to the bulbar and tarsal conjunctivas at the superior limbus. Ophthalmol 2009; 3: 57-67. • 364.01 Primary iridocyclitis 12. Quinto GG, Campos M, Behrens A. Autologous serum • 364.02 Recurrent iridocyclitis for ocular surface diseases. Arq Bras Oftalmol 2008; 71(6 Treatment Suppl): 47-54. • 364.05 Hypopyon Case management for CLSLK is straight- 13. Sindt CW, Longmuir RA. Contact lens strategies for the patient with dry eye. Ocul Surf 2007; 5: 294-307. 14. Wakefield D, Chang JH. What is new HLA-B27 acute CONTACT LENS SUPERIOR LIMBIC anterior uveitis? Ocul Immunol Inflamm 2011; 19: 139-144. KERATOCONJUNCTIVITIS 15. Islam N, Pavesio C. Uveitis (acute anterior). Clin Evid Etiology (Online) 2010 Apr 8; pii: 0705. Accessed November 16, 2011. 16. Hogan MJ, Kimura SJ, Thygeson P. Signs and symp- Contact lens superior limbic keratocon- toms of uveitis. Am J Ophthalmol 1959; 47: 155-170. junctivitis (CLSLK) and superior limbic 17. Gutteridge IF, Hall AJ. Acute anterior uveitis in primary keratoconjunctivitis (SLK) are inflammatory care. Clin Exp Optom 2007; 90: 70-82. conditions that affect the superior bulbar con- 18. Yanoff M, Duker JS, eds. Ophthalmology, 3rd ed. Maryland Heights, MO, Elsevier, 2009. junctiva and adjacent corneal surface. CLSLK 19. Ehlers JP, Shah CP, eds. The Wills Eye Manual: Office is recognized as a completely separate event and Emergency Room Diagnosis and Treatment of Eye from Theodore’s SLK. CLSLK is identified Disease, 5th Ed. Philadelphia, PA: Lippincott, Williams and in patients wearing soft lenses by superior Fibrin in anterior chamber in acute anterior uveitis Wilkins, 2008.

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