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January 2009 ICO International Clinical Guidelines This document contains 24 International Clinical Guidelines defined by the International Council of Ophthalmology (ICO). The Guidelines are designed to be translated and adapted by ophthalmologic societies to help ophthalmologists assess how they are treating patients. They are intended to serve a supportive and educational role and ultimately to improve the quality of eye care for patients. Below is a list of the Guidelines available with links to each Guideline in this document, followed by the Preface to the Guidelines. Also see the Introduction to the ICO International Clinical Guidelines www.icoph.org/enhancing_eyecare/international_clinical_guidelines.html. For the latest information on the ICO Clinical Guidelines and to download individual Guidelines as separate PDF files, see the www.icoph.org resources listings. List of Guidelines Available Age-Related Macular Degeneration (Initial and Follow-up Evaluation) Age-Related Macular Degeneration (Management Recommendations) Amblyopia (Initial and Follow-up Evaluation) Bacterial Keratitis (Initial Evaluation) Bacterial Keratitis (Management Recommendations) Blepharitis (Initial and Follow-up Evaluation) Cataract (Initial and Follow-up Evaluation) Conjunctivitis (Initial Evaluation and Therapy) Diabetic Retinopathy (Initial and Follow-up Evaluation) Diabetic Retinopathy (Management Recommendations) Dry Eye Syndrome (Initial Evaluation) Dry Eye Syndrome (Management Recommendations) Esotropia (Initial and Follow-up Evaluation) Exotropia (Initial and Follow-up Evaluation) Eye Disease in Leprosy (Initial Evaluation and Management) Idiopathic Macular Hole (Initial Evaluation and Therapy) Keratorefractive Surgery (Initial and Follow-up Evaluation) Ocular HIV/AIDS Related Diseases (Initial and Follow-up Evaluation) Posterior Vitreous Detachment, Retinal Breaks and Lattice Degeneration (Initial and Follow-up Evaluation) Primary Open-Angle Glaucoma (Initial Evaluation) Primary Open-Angle Glaucoma (Follow-up Evaluation) Primary Open-Angle Glaucoma Suspect (Initial and Follow-up Evaluation) Primary Angle Closure (Initial Evaluation and Therapy) Trachoma Preface to the Guidelines International Clinical Guidelines are prepared and distributed by the International Council of Ophthalmology. These Guidelines are to serve a supportive and educational role for ophthalmologists worldwide. These guidelines are intended to improve the quality of eye care for patients. They have been adapted in many cases from similar documents (Benchmarks of Care) created by the American Academy of Ophthalmology based on their Preferred Practice Patterns. While it is tempting to equate these to Standards, it is impossible and inappropriate to do so. The multiple circumstances of geography, equipment availability, patient variation and practice settings preclude a single standard. Guidelines on the other hand are a clear statement of expectations. These include comments of the preferred level of performance assuming conditions that allow the use of optimum equipment, pharmaceuticals and/or surgical circumstances. Thus, a basic expectation is created and if the situation is optimum, the optimum facets of diagnosis, treatment and follow up may be employed. Excellent, appropriate and successful care can also be provided where optimum conditions do not exist. Simply following the Guidelines does not guarantee a successful outcome. It is understood that, given the uniqueness of a patient and his or her particular circumstance, physician judgment must be employed. This can result in a modification in application of a guideline in individual situations. Medical experience has been relied upon in the preparation of these guidelines, and they are whenever possible, evidence-based. This means these Guidelines are based on the latest available scientific information. The ICO is committed to provide updates of these guidelines on a regular basis (approximately every two to three years). (Also see the Introduction to the ICO International Clinical Guidelines at www.icoph.org/guide/guideintro.html and the list of other Guidelines at www.icoph.org/guide/guidelist.html.) ICO International Clinical Guidelines Page 2 Age-Related Macular Degeneration (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Symptoms (metamorphopsia, decreased vision) (A:II) Medications and nutritional supplements (B:III) Ocular history (B:II) Systemic history (any hypersensitivity reactions) (B:II) Family history, especially family history of AMD (B:II) Social history, especially smoking (B:II) Initial Physical Exam (Key elements) Visual acuity (A:III) Stereo biomicroscopic examination of the macula (A:III) Ancillary Tests Intravenous fundus fluorescein angiography in the clinical setting of AMD is indicated: (A:I) o when patient complains of new metamorphopsia o when patient has unexplained blurred vision o when clinical exam reveals elevation of the RPE or retina, subretinal blood, hard exudates or subretinal fibrosis o to detect the presence of and determine the extent, type, size, and location of CVN and to calculate the percentage of the lesion composed of or consisting of classic CNV o to guide treatment (laser photocoagulation surgery or verteporfin PDT) o to detect persistent or recurrent CNV following treatment o to assist in determining the cause of visual loss that is not explained by clinical exam Each angiographic facility must have a care plan or an emergency plan and a protocol to minimize the risk and manage any complications. (A:III) ICO International Clinical Guidelines Page 3 Follow-up Exam History Visual symptoms, including decreased vision and metamorphopsia (A:II) Changes in medications and nutritional supplements (B:III) Interval ocular history (B:III) Interval systemic history (B:III) Changes in social history, especially smoking (B:II) Follow-up Physical Exam Visual acuity (A:III) Stereo biomicroscopic examination of the fundus (A:III) Follow-up Treatment after Neovascular AMD Discuss risks, benefits and complications with the patient and obtain informed consent (A:III) Examine patients treated with ranibizumab intravitreal injections approximately 4 weeks after treatment (A:III) Examine patients treated with bevacizumab intravitreal injections approximately 4 to 8 weeks after treatment (A:III) Examine patients treated with pegaptanib sodium injection approximately 6 weeks following the treatment (A:III) Examine and perform fluorescein angiography at least every 3 months for up to 2 years after verteporfin PDT (A:I) Examine patients treated with thermal laser photocoagulation approximately 2 to 4 weeks after treatment and then at 4 to 6 weeks (A:III) Optical coherence tomography, (A:III) fluorescein angiography, (A:I) and fundus photography (A:III) may be helpful to detect signs of exudation and should be used when clinically indicated Subsequent examinations should be performed as indicated depending on the clinical findings and the judgment of the treating ophthalmologist (A:III) Patient Education Educate patients about the prognosis and potential value of treatment as appropriate for their ocular and functional status (A:III) Encourage patients with early AMD to have regular dilated eye exams for early detection of intermediate AMD (A:III) Educate patients with intermediate AMD about methods of detecting new symptoms of CVN and about the need for prompt notification to an ophthalmologist (A:III) Instruct patients with unilateral disease to monitor their vision in their fellow eye and to return periodically even in absence of symptoms, but promptly after onset of new or significant visual symptoms (A:III) Instruct patients to report symptoms suggestive of endophthalmitis, including eye pain or increased discomfort, worsening eye redness, blurred or decreased vision, increased sensitivity to light, or increased number of ICO International Clinical Guidelines Page 4 floaters promptly (A:III) Encourage patients who are currently smoking to stop (A:I) because there are observational data that support a causal relationship between smoking and AMD (A:II) and other considerable health benefits of smoking cessation Refer patients with reduced visual function for vision rehabilitation (see www.aao.org/smartsight) and social services (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks for Preferred Practice Patterns™ (PPPs) (www.aao.org) ICO International Clinical Guidelines Page 5 Age-Related Macular Degeneration (Management Recommendations) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Treatment Recommendations and Follow-up Plans for Age-Related Macular Degeneration Recommended Treatment Observation with no medical or surgical therapies (A:I) Diagnoses Eligible for Treatment No clinical signs of AMD (AREDS category 1) As recommended in the Comprehensive Adult Medical Eye Evaluation PPP (A:III) Early AMD (AREDS category 2) Return exam at 6 to 24 months if asymptomatic or prompt exam for new symptoms suggestive of CVN (A:III) Advanced AMD with bilateral subfoveal geographic atrophy or disciform scars Antioxidant vitamin and mineral supplements as recommended in the AREDS reports (A:I) Follow-up Recommendations No fundus photos or fluorescein angiography unless symptomatic (A:I) Intermediate AMD (AREDS category 3) Monitoring of monocular near vision (reading/Amsler grid) (A:III) Advanced AMD in one eye (AREDS category 4) Return exam at 6 to 24 months if asymptomatic or prompt exam for new symptoms suggestive of CVN (A:III) Fundus photography as appropriate Fluorescein angiography if there is evidence of edema or other signs and symptoms of CVN Ranibizumab intravitreal injection 0.5 mg as recommended in ranibizumab literature (A:I) Subfoveal CNV Patients should be instructed to report any symptoms suggestive of endophthalmitis promptly, including eye pain or increased discomfort, worsening eye redness, blurred or decreased vision, increased sensitivity to light, or increased number of floaters (A:III) ICO International Clinical Guidelines Page 6 Return exam approximately 4 weeks after treatment; subsequent follow-up depends on the clinical findings and judgement of the treating ophthalmologist (A:III) Monitoring of monocular near vision (reading/Amsler grid) (A:III) Bevacizumab intravitreal injection as described in published reports (A:III) Subfoveal CNV The ophthalmologist should provide appropriate informed consent with respect to the off-label status (A:III) Patients should be instructed to report any symptoms suggestive of endophthalmitis promptly, including eye pain or increased discomfort, worsening eye redness, blurred or decreased vision, increased sensitivity to light, or increased number of floaters (A:III) Return exam approximately 4 to 8 weeks after treatment; subsequent follow-up depends on the clinical findings and judgement of the treating ophthalmologist (A:III) Monitoring of monocular near vision (reading/Amsler grid) (A:III) Pegaptanib sodium intravitreal injection as recommended in pegaptanib sodium literature (A:I) Subfoveal CNV, new or recurrent, for predominantly classic lesions <12 MPS disc area in size Minimally classic, or occult with no classic lesions where the entire lesion is <12 disc areas in size, subretinal hemorrhage associated with CVN comprises <50% of lesion, and/or there is lipid present, and/or the patient has lost 15 or more letters of visual acuity during the previous 12 weeks PDT with verteporfin Subfoveal CNV, new Patients should be instructed to report any symptoms suggestive of endophthalmitis promptly, including eye pain or increased discomfort, worsening eye redness, blurred or decreased vision, increased sensitivity to light, or increased number of floaters (A:III) Return exam with retreatments every 6 weeks as indicated (A:III) Monitoring of monocular near vision (reading/Amsler grid) (A:III) Return exam approximately every 3 ICO International Clinical Guidelines Page 7 as recommended in the TAP and VIP reports (A:I) or recurrent, where the classic component is >50% of the lesion and the entire lesion is <5400 microns in greatest linear diameter months until stable, with retreatments as indicated (A:III) Monitoring of monocular near vision (reading/Amsler grid) (A:III) Occult CNV may be considered for PDT with vision <20/50 or if the CVN is <4 MPS disc areas in size when the vision is >20/50 Thermal laser photocoagulation surgery as recommended in the MPS reports (A:I) Extrafoveal classic CNV, new or recurrent May be considered for juxtapapillary CVN Return exam with fluorescein angiography approximately 2 to 4 weeks after treatment, and then at 4 to 6 weeks and thereafter depending on the clinical and angiographic findings (A:III) Retreatments as indicated Monitoring of monocular near vision (reading/Amsler grid) (A:III) AMD = Age-related Macular Degeneration; AREDS = Age-related Eye Disease Study; CNV = choroidal neovascularization; MPS = Macular Photocoagulation Study; PDT = photodynamic therapy; TAP = Treatment of Age-related Macular Degeneration with Photodynamic Therapy; VIP = Verteporfin in Photodynamic Therapy * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 8 Amblyopia (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Ocular symptoms and signs (A:III) Ocular history (A:III) Systemic history, including review of prenatal, perinatal, and postnatal medical factors (A:III) Family history, including eye conditions and relevant systemic diseases (A:III) Initial Physical Exam (Key elements) Assessment of visual acuity and fixation pattern (A:III) Ocular alignment and motility (A:III) Red reflex or binocular red reflex (Brückner) test (A:III) Pupil examination (A:III) External examination (A:III) Anterior segment examination (A:III) Cycloplegic retinoscopy/refraction (A:III) Funduscopic examination (A:III) Binocularity/stereoacuity testing (A:III) Care Management Choose treatment based on patient's age; visual acuity; compliance with previous treatment; and physical, social, and psychological status. (A:III) Treatment goal is to achieve equalization/normalization of fixation patterns or visual acuity. (A:III) Once maximal visual acuity has been obtained, treatment should be tapered and eventually stopped. (A:III) Follow-up Evaluation Follow-up visits should include: o Interval history (A:III) o Tolerance to therapy (A:III) o Examinations and testing as indicated (A:III) ICO International Clinical Guidelines Page 9 Amblyopia Follow-up Evaluation Intervals During Active Treatment Period (A:III) Age (years) 0-1 1-2 2-3 3-4 4-5 5-7 7-9 High- Percentage Occlusion (70% or more of waking hours/≥ 6 hours per day) 1-4 weeks 2-8 weeks 3-12 weeks 4-16 weeks 4-16 weeks 6-16 weeks 8-16 weeks Low-Percentage Occlusion (<70% of waking hours/<6 hours per day) or Penalization 2-8 weeks 2-4 months 2-4 months 2-6 months 2-6 months 2-6 months 3-6 months Maintenance Treatment or Observation 1-4 months 2-4 months 2-4 months 2-6 months 2-6 months 2-6 months 3-12 months Patient Education Discuss diagnosis, severity of disease, prognosis and treatment plan with patient, parents and /or caregivers. (A:III) Explain the disorder and recruit the family in a collaborative approach to therapy. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 10 Bacterial Keratitis (Initial Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Ocular symptoms (A:III) Contact lens history (A:II) Review of other ocular history (A:III) Review of other medical problems and systemic medications (A:III) Current and recently used ocular medications (A:III) Medication allergies (A:III) Initial Physical Exam Visual acuity (A:III) General appearance of patient (B:III) Facial examination (B:III) Eyelids and eyelid closure (A:III) Conjunctiva (A:III) Nasolacrimal apparatus (B:III) Corneal sensation (A:III) Slit-lamp biomicroscopy o Eyelid margins (A:III) o Conjunctiva (A:III) o Sclera (A:III) o Cornea (A:III) o Anterior chamber (A:III) o Anterior vitreous (A:III) Contralateral eye (A:III) Diagnostic Tests Manage majority of community-acquired cases with empiric therapy and without smears or cultures. (A:III) Indications for smears and cultures: o Sight-threatening or severe keratitis of suspected microbial origin prior to initiating therapy (A:III) o A large corneal infiltrate that extends to the middle to deep stroma (A:III) o Chronic in nature (A:III) o Unresponsive to broad spectrum antibiotic therapy (A:III) o Clinical features suggestive of fungal, amoebic, or mycobacterial keratitis (A:III) The hypopyon that occurs in eyes with bacterial keratitis is usually sterile, ICO International Clinical Guidelines Page 11 and aqueous or vitreous taps should not be performed unless there is a high suspicion of microbial endophthalmitis. (A:III) Corneal scrapings for culture should be inoculated directly onto appropriate culture media to maximize culture yield. (A:III). If this is not feasible, place specimens in transport media. (A:III). In either case, immediately incubate cultures or take promptly to the laboratory. (A:III) Care Management Topical antibiotic eye drops are preferred method in most cases. (A:III) Use topical broad-spectrum antibiotics initially in the empiric treatment of presumed bacterial keratitis. (A:III) For central or severe keratitis (e.g., deep stromal involvement or an infiltrate larger than 2 mm with extensive suppuration), use a loading dose (e.g., every 5 to 15 minutes for the first 1 to 3 hours), followed by frequent applications (e.g., every 30 minutes to 1 hour around the clock). (A:III) For less severe keratitis, a regimen with less frequent dosing is appropriate. (A:III) Use systemic therapy for gonococcal keratitis. (A:II) In general, modify initial therapy when there is a lack of improvement or stabilization within 48 hours. (A:III) For patients treated with ocular topical corticosteroids at time of presentation of suspected bacterial keratitis, reduce or eliminate corticosteroids until infection has been controlled. (A:III) When the corneal infiltrate compromises the visual axis, may add topical corticosteroid therapy following at least 2 to 3 days of progressive improvement with topical antibiotics. (A:III) Continue topical antibiotics at high levels with gradual tapering. (A:III) Examine patients within 1 to 2 days after initiation of topical corticosteroid therapy. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 12 Bacterial Keratitis (Management Recommendations) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Follow-up Evaluation Frequency depends on extent of disease, but follow severe cases initially at least daily until clinical improvement or stabilization is documented. (A:III) Patient Education Inform patients with risk factors predisposing them to bacterial keratitis of their relative risk, the signs and symptoms of infection, and to consult an ophthalmologist promptly if they experience such warning signs or symptoms (A:III) Educate about the destructive nature of bacterial keratitis and need for strict compliance with therapy. (A:III) Discuss possibility of permanent visual loss and need for future visual rehabilitation. (A:III) Educate patients with contact lenses about increased risk of infection associated with contact lens, overnight wear, and importance of adherence to techniques to promote contact lens hygiene. (A:III) Refer patients with significant visual impairment or blindness for vision rehabilitation if they are not surgical candidates (see www.aao.org/smartsight). (A:III) Antibiotic Therapy of Bacterial Keratitis [A:III] Organism Antibiotic Cefazolin with Tobramycin or gentamicin or Fluoroquinolones Topical Concentration 50 mg/ml 9-14 mg/ml 3 or 5 mg/ml Various** Subconjunctival Dose 100 mg in 0.5 ml 20 mg in 0.5 ml No organism identified or multiple types of organisms Gram-positive Cocci Cefazolin Vancomycin*** Bacitracin*** Fluoroquinolones* 50 mg/ml 15-50 mg/ml 10,000 IU Various** 100 mg in 0.5 ml 25 mg in 0.5 ml ICO International Clinical Guidelines Page 13 Gram-negative rods Tobramycin or gentamicin Ceftazidime Fluoroquinolones 9-14 mg/ml 50 mg/ml Various** 20 mg in 0.5 ml 100 mg in 0.5 ml Gram-negative Cocci**** Ceftriaxone Ceftazidime Fluoroquinolones 50 mg/ml 50 mg/ml Various** 100 mg in 0.5 ml 100 mg in 0.5 ml Nontuberculous Mycobacteria Amikacin Clarithromycin Azithromycin***** Fluoroquinolones Sulfacetamide Amikacin Trimethoprim/Sulfa methoxazole: Trimethoprim Sulfamethoxazole 20-40 mg/ml 10 mg/ml 10 mg/ml Various** 100 mg/ml 20-40 mg/ml 20 mg in 0.5 ml Nocardia 20 mg in 0.5 ml 16 mg/ml 80 mg/ml *Fewer gram-positive cocci are resistant to gatifloxacin and moxifloxacin than other fluoroquinolones. **Ciprofloxacin 3 mg/ml; gatifloxacin 3 mg/ml; levofloxacin 15 mg/ml; moxifloxacin 5mg/ml; ofloxacin 3 mg/ml, all commercially available at these concentrations. ***For resistant Enterococcus and Staphylococcus species and penicillin allergy. Vancomycin and Bacitracin have no gram-negative activity and should not be used as a single agent empirically in treating bacterial keratitis. **** Systemic therapy is necessary for suspected gonococcal infection. ***** Data from Chandra NS, Torres MF, Winthrop KL. Cluster of Mycobacterium chelonae keratitis cases following laser in-situ keratomileusis. Am J Ophthalmol 2001; 132:819-30. * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 14 Blepharitis (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Ocular symptoms and signs (A:III) Time of day when symptoms are worse (A:III) Duration of symptoms (A:III) Unilateral or bilateral presentation (A:III) Exacerbating conditions (e.g., smoke, allergens, wind, contact lens, low humidity, retinoids, diet, alcohol consumption, eye makeup) (A:III) Symptoms related to systemic diseases (e.g., rosacea, allergy) (A:III) Current and previous systemic and topical medications (A:III) Recent exposure to an infected individual (e.g., pediculosis) (C:III) Ocular history (e.g., previous intraocular and eyelid surgery, local trauma, including mechanical, thermal, chemical, and radiation injury) (A:III) Systemic history (e.g., dermatological diseases, such as rosacea, atopic disease, and herpes zoster ophthalmicus) (A:III) Initial Physical Exam Visual acuity (A:III) External examination o Skin (A:III) o Eyelids (A:III) Slit-lamp biomicroscopy o Tear film (A:III) o Anterior eyelid margin (A:III) o Eyelashes (A:III) o Posterior eyelid margin (A:III) o Tarsal conjunctiva (A:III) o Bulbar conjunctiva (A:III) o Cornea (A:III) Measurement of IOP (A:III) Diagnostic Tests Cultures may be indicated for patients with recurrent anterior blepharitis with severe inflammation as well as for patients who are not responding to therapy. (A:III) Biopsy of the eyelid to exclude the possibility of carcinoma may be indicated in cases of marked asymmetry, resistance to therapy or unifocal recurrent chalazia that do not respond well to therapy. (A:II) Consult with the pathologist prior to obtaining the biopsy if sebaceous cell carcinoma is suspected.(A:II) ICO International Clinical Guidelines Page 15 Care Management Treat patients with blepharitis initially with a regimen of warm compress and eyelid hygiene. (A:III) For patients with staphylococcal blepharitis, a topical antibiotic such as erythromycin can be prescribed to be applied one or more times daily or at bedtime on the eyelids for one or more weeks. (A:III) For patients with meibomian gland dysfunction, whose chronic symptoms and signs are not adequately controlled with eyelid hygiene, oral tetracyclines can be prescribed. (A:III) A brief course of topical corticosteroids may be helpful for eyelid or ocular surface inflammation. The minimal effective dose of corticosteroids should be utilized and long-term corticosteroid therapy should be avoided if possible. (A:III) Follow-up Evaluation Follow-up visits should include: o Interval history (A:III) o Visual acuity (A:III) o External exam (A:III) o Slit-lamp biomicroscopy (A:III) If corticosteroid therapy is prescribed, re-evaluate patient within a few weeks to determine the response to therapy, measure intraocular pressure, and assess treatment compliance (A:III) Patient Education Counsel patients about the chronicity and recurrence of the disease process. (A:III) Inform patients that symptoms can frequently be improved but are rarely eliminated. (A:III) Advise patient that if warm compress and eyelid hygiene treatment is effective, symptoms often recur if treatment is stopped so may be necessary long term (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 16 Cataract (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Symptoms (A:II) Ocular history (A:III) Systemic history (A:III) Assessment of visual functional status (A:II) Initial Physical Exam Visual acuity, with current correction (A:III) Measurement of BCVA (with refraction when indicated) (A:III) Ocular alignment and motility(A:III) Pupil reactivity and function (A:III) Measurement of IOP (A:III) External examination (A:III) Slit-lamp biomicroscopy (A:III) Evaluation of the fundus (through a dilated pupil) (A:III) Assessment of relevant aspects of general and mental health (B:III) Care Management Treatment is indicated when visual function no longer meets the patient's needs and cataract surgery provides a reasonable likelihood of improvement. (A:II) Cataract removal is also indicated when there is evidence of lens-induced diseases or when it is necessary to visualize the fundus in an eye that has the potential for sight. (A:III) Surgery should not be performed under the following circumstances: (A:III) glasses or visual aids provide vision that meets the patient's needs’, surgery will not improve visual function; the patient cannot safely undergo surgery because of coexisting medical or ocular conditions; appropriate postoperative care cannot be obtained. Indications for second eye surgery are the same as for the first eye. (A:II) (with consideration given to the needs for binocular function) Preoperative Care Ophthalmologist who is to perform the surgery has the following responsibilities: Examine the patient preoperatively (A:III) Ensure that the evaluation accurately documents symptoms, findings and indications for treatment (A:III) ICO International Clinical Guidelines Page 17 Inform the patient about the risks, benefits and expected outcomes of surgery (A:III) Formulate surgical plan, including selection of an IOL (A:III) Review results of presurgical and diagnostic evaluations with the patient (A:III) Formulate postoperative plans and inform patient of arrangements (A:III) Follow-up Evaluation High-risk patients should be seen within 24 hours of surgery. (A:III) Routine patients should be seen within 48 hours of surgery. (A:III) Frequency and timing of subsequent visits depend on refraction, visual function, and medical condition of the eye. More frequent follow-up usually necessary for high risk patients. Components of each postoperative exam should include: o Interval history, including new symptoms and use of postoperative medications (A:III) o Patient's assessment of visual functional status (A:III) o Assessment of visual function (visual acuity, pinhole testing) (A:III) o Measurement of IOP (A:III) o Slit-lamp biomicroscopy (A:III) Nd:YAG Laser Capsulotomy Treatment is indicated when vision impaired by posterior capsular opacification does not meet the patient's functional needs or when it critically interferes with visualization of the fundus. (A:III) Educate about the symptoms of posterior vitreous detachment, retinal tears and detachment and need for immediate examination if these symptoms are noticed. (A:III) Patient Education For patients who are functionally monocular, discuss special benefits and risks of surgery, including the risk of blindness. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 18 Conjunctivitis (Initial Evaluation and Therapy) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Ocular symptoms and signs (e.g., itching, discharge, irritation, pain, photophobia, blurred vision) (A:III) Duration of symptoms (A:III) Exacerbating factors (A:III) Unilateral or bilateral presentation (A:III) Character of discharge (A:III) Recent exposure to an infected individual (A:III) Trauma (mechanical, chemical, ultraviolet) (A:III) Contact lens wear (e.g., lens type, hygiene and use regimen) (A:III) Symptoms and signs potentially related to systemic diseases (e.g., genitourinary discharge, dysuria, upper respiratory infection, skin and mucosal lesions) (A:III) Allergy, asthma, eczema (A:III) Use of topical and systemic medications (A:III) Use of personal care products (A:III) Ocular history (e.g., previous episodes of conjunctivitis (A:III) and previous ophthalmic surgery) (B:III) Systemic history (e.g., compromised immune status, current and prior systemic diseases) (B:III) Social history (e.g., smoking, occupation and hobbies, travel and sexual activity) (C:III) Initial Physical Exam Visual acuity (A:III) External examination o Regional lymphadenopathy (particularly preauricular) (A:III) o Skin (A:III) o Abnormalities of the eyelids and adnexae (A:III) o Conjunctiva (A:III) Slit-lamp biomicroscopy o Eyelid margins (A:III) o Eyelashes (A:III) o Lacrimal puncta and canaliculi (B:III) o Tarsal and forniceal conjunctiva (A:II) o Bulbar conjunctiva/limbus (A:II) o Cornea (A:I) o Anterior chamber/iris (A:III) o Dye-staining pattern (conjunctiva and cornea) (A:III) ICO International Clinical Guidelines Page 19 Diagnostic Tests Cultures, smears for cytology and special stains are indicated in cases of suspected infectious neonatal conjunctivitis. (A: I) Smears for cytology and special stains are recommended in cases of suspected gonococcal conjunctivitis. (A:II) Confirm diagnosis of adult and neonate chlamydial conjunctivitis with immunodiagnostic test and/or culture. (A:III) Biopsy the bulbar conjunctiva and take a sample from an uninvolved area adjacent to the limbus in an eye with active inflammation when ocular mucous membrane pemphigoid is suspected. (A:III) A full-thickness lid biopsy is indicated in cases of suspected sebaceous carcinoma. (A:II) Care Management Avoid indiscriminate use of topical antibiotics or corticosteroids because antibiotics can induce toxicity and corticosteroids can prolong adenoviral infections and worsen herpes simplex virus infections (A:III) Treat mild allergic conjunctivitis with an over-the-counter antihistamine/vasoconstrictor agent or second-generation topical histamine H1-receptor antagonists. (A:III) If the condition is frequently recurrent or persistent, use mast-cell stabilizers (A:I) For contact lens-related keratoconjunctivitis, discontinue contact lens wear for 2 or more weeks (A:III) If corticosteroids are indicated, prescribe the minimal amount based on patient response and tolerance (A:III) If corticosteroids are used, perform baseline measurement of intraocular pressure (A:III) Use systemic antibiotic treatment for conjunctivitis due to Neisseria gonorrhoeae (A:I) or Chlamydia trachomatis. (A:II) Treat sexual partners to minimize recurrence and spread of disease when conjunctivitis is associated with sexually transmitted diseases and refer patients and their sexual partners to an appropriate medical specialist. (A:III) Refer patients with manifestation of a systemic disease to an appropriate medical specialist. (A:III) Follow-up Evaluation Follow-up visits should include: o Interval history (A:III) o Visual acuity (A:III) o Slit-lamp biomicroscopy (A:III) If corticosteroids are used, perform periodic measurement of intraocular pressure and pupillary dilation to evaluate for cataract and glaucoma (A:III) ICO International Clinical Guidelines Page 20 Patient Education Counsel patients with contagious varieties to minimize or prevent spread of diseases in the community (A:III) Inform patients who may require repeat short-term therapy with topical corticosteroid of potential complications of corticosteroid use (A:III) Advise patients with allergic conjunctivitis that frequent clothes washing and bathing/showering before bedtime may be helpful (B:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 21 Diabetic Retinopathy (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Duration of diabetes (A:I) Past glycemic control (hemoglobin A1c) (A:I) Medications (A:III) Systemic history (e.g., obesity (A:III), renal disease (A:II), systemic hypertension (A:I), serum lipid levels (A:II), pregnancy (A:I)) Ocular history (A:III) Initial Physical Exam (Key elements) Visual acuity (A:I) Measurement of IOP (A:III) Gonioscopy when indicated (for neovascularization of the iris or increased IOP) (A:III) Slit-lamp biomicroscopy (A:III) Dilated funduscopy including stereoscopic examination of the posterior pole (A:I) Examination of the peripheral retina and vitreous, best performed with indirect ophthalmoscopy or with slit-lamp biomicroscopy, combined with a contact lens (A:III) Diagnosis Classify both eyes as to category and severity of diabetic retinopathy, with presence/absence of CSME.(A:III) Each category has an inherent risk for progression. Follow-up History Visual symptoms (A:III) Systemic status (e.g., pregnancy, blood pressure, serum cholesterol, renal status) (A:III) Glycemic status (hemoglobin A1c) (A:I) Follow-up Physical Exam Visual acuity (A:I) Measurement of IOP (A:III) Slit-lamp biomicroscopy with iris examination (A:II) Gonioscopy (if neovascularization is suspected or present or if intraocular ICO International Clinical Guidelines Page 22 pressure is increased) (A:II) Stereo examination of the posterior pole after dilation of the pupils (A:I) Examination of the peripheral retina and vitreous when indicated (A:II) Ancillary Tests Fundus photography is seldom of value in cases of minimal diabetic retinopathy or when diabetic retinopathy is unchanged from the previous photographic appearance. (A:III) Fundus photography may be useful for documenting significant progression of disease and response to treatment. (B:III) Fluorescein angiography is used as a guide for treating CSME (A:I) and as a means of evaluating the cause(s) of unexplained decreased visual acuity. (A:III) Angiography can identify macular capillary nonperfusion (A:II) or sources of capillary leakage resulting in macular edema as possible explanations for visual loss. Fluorescein angiography is not routinely indicated as part of the examination of patients with diabetes. (A:III) Fluorescein angiography is not needed to diagnose CSME or PDR, both of which are diagnosed by means of the clinical exam. Patient Education Discuss results or exam and implications. (A:II) Encourage patients with diabetes but without diabetic retinopathy to have annual dilated eye exams. (A:II) Inform patients that effective treatment for diabetic retinopathy depends on timely intervention, despite good vision and no ocular symptoms. (A:II) Educate patients about the importance of maintaining near-normal glucose levels and near-normal blood pressure and lowering serum lipid levels. (A:III) Communicate with the attending physician, e.g., family physician, internist, or endocrinologist, regarding eye findings. (A:III) Provide patients whose conditions fail to respond to surgery and for whom treatment is unavailable with proper professional support and offer referral for counseling, rehabilitative, or social services as appropriate. (A:III) Refer patients with reduced visual function for vision rehabilitation (see www,aao.org/smartsight) and social services (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 23 Diabetic Retinopathy (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Duration of diabetes (A:I) Past glycemic control (hemoglobin A1c) (A:I) Medications (A:III) Systemic history (e.g., obesity (A:III), renal disease (A:II), systemic hypertension (A:I), serum lipid levels (A:II), pregnancy (A:I)) Ocular history (A:III) Initial Physical Exam (Key elements) Visual acuity (A:I) Measurement of IOP (A:III) Gonioscopy when indicated (for neovascularization of the iris or increased IOP) (A:III) Slit-lamp biomicroscopy (A:III) Dilated funduscopy including stereoscopic examination of the posterior pole (A:I) Examination of the peripheral retina and vitreous, best performed with indirect ophthalmoscopy or with slit-lamp biomicroscopy, combined with a contact lens (A:III) Diagnosis Classify both eyes as to category and severity of diabetic retinopathy, with presence/absence of CSME.(A:III) Each category has an inherent risk for progression. Follow-up History Visual symptoms (A:III) Systemic status (e.g., pregnancy, blood pressure, serum cholesterol, renal status) (A:III) Glycemic status (hemoglobin A1c) (A:I) Follow-up Physical Exam Visual acuity (A:I) Measurement of IOP (A:III) Slit-lamp biomicroscopy with iris examination (A:II) Gonioscopy (if neovascularization is suspected or present or if intraocular ICO International Clinical Guidelines Page 24 pressure is increased) (A:II) Stereo examination of the posterior pole after dilation of the pupils (A:I) Examination of the peripheral retina and vitreous when indicated (A:II) Ancillary Tests Fundus photography is seldom of value in cases of minimal diabetic retinopathy or when diabetic retinopathy is unchanged from the previous photographic appearance. (A:III) Fundus photography may be useful for documenting significant progression of disease and response to treatment. (B:III) Fluorescein angiography is used as a guide for treating CSME (A:I) and as a means of evaluating the cause(s) of unexplained decreased visual acuity. (A:III) Angiography can identify macular capillary nonperfusion (A:II) or sources of capillary leakage resulting in macular edema as possible explanations for visual loss. Fluorescein angiography is not routinely indicated as part of the examination of patients with diabetes. (A:III) Fluorescein angiography is not needed to diagnose CSME or PDR, both of which are diagnosed by means of the clinical exam. Patient Education Discuss results or exam and implications. (A:II) Encourage patients with diabetes but without diabetic retinopathy to have annual dilated eye exams. (A:II) Inform patients that effective treatment for diabetic retinopathy depends on timely intervention, despite good vision and no ocular symptoms. (A:II) Educate patients about the importance of maintaining near-normal glucose levels and near-normal blood pressure and lowering serum lipid levels. (A:III) Communicate with the attending physician, e.g., family physician, internist, or endocrinologist, regarding eye findings. (A:III) Provide patients whose conditions fail to respond to surgery and for whom treatment is unavailable with proper professional support and offer referral for counseling, rehabilitative, or social services as appropriate. (A:III) Refer patients with reduced visual function for vision rehabilitation (see www,aao.org/smartsight) and social services (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 25 Diabetic Retinopathy (Management Recommendations) Management Recommendations for Patients with Diabetes Severity of Retinopathy 1. Normal or minimal NPDR 2. Mild to moderate NPDR Presence FollowPanretinal Focal Fluorescein of up Photocoagulation and/or Angiography CSME* (Months) (Scatter) Laser Laser† No 12 No No No No Yes 6-12 2-4 No No No Usually No Usually*^ No Yes 4. Non-high-risk No PDR Yes 5. High-risk PDR No Yes 6. No Inactive/involuted Yes PDR 2-4 2-4 2-4 2-4 2-4 2-4 6-12 2-4 Sometimes‡ Sometimes‡ Sometimes‡ Sometimes‡ Usually Usually No No Rarely Usually Rarely Usually Rarely Usually No Usually No Usually** No Usually^ No Usually** Usually Usually 3. Severe NPDR CSME = clinically significant macular edema; NPDR = nonproliferative diabetic retinopathy; PDR = proliferative diabetic retinopathy * Exceptions include: hypertension or fluid retention associated with heart failure, renal failure, pregnancy, or any other causes that may aggravate macular edema. Deferral of photocoagulation for a brief period of medical treatment may be considered in these cases. Also, deferral of CSME treatment is an option when the center of the macula is not involved, visual acuity is excellent, and the patient understands the risks. † Adjunctive treatment that may be considered include intravitreal corticosteroids or anti-vascular endothelial growth factor agents (off-label use). ^ Deferring focal photocoagulation for CSME is an option when the center of the macula is not involved, visual acuity is excellent, close follow-up is possible, and the patient understands the risks. However, initiation of treatment with focal photocoagulation should also be considered because although treatment with focal photocoagulation is less likely to improve the vision, it is more likely to stabilize the current visual acuity. Treatment of lesions close to the foveal avascular zone may result in damage to central vision and with time, such laser scars may expand and cause further vision deterioration. Closer follow-up may be necessary for macular edema that is not clinically significant. ICO International Clinical Guidelines Page 26 ‡ Panretinal photocoagulation surgery may be considered as patients approach high-risk PDR. The benefit of early panretinal photocoagulation at the severe nonproliferative or worse stage of retinopathy is greater in patients with type 2 diabetes than in those with type 1. Treatment should be considered for patients with severe NPDR and type 2 diabetes. Other factors, such as poor compliance with follow-up, impending cataract extraction or pregnancy, and status of fellow eye will help in determining the timing of the panretinal photocoagulation. ** It is preferable to perform the focal photocoagulation first, prior to panretinal photocoagulation laser-induced exacerbation of the macular edema. * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 27 Dry Eye Syndrome (Initial Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Ocular symptoms and signs (A:III) Exacerbating conditions (B:III) Duration of symptoms (A:III) Topical medications used and their effect on symptoms (A:III) Ocular history, including o Contact lens wear, schedule and care (A:III) o Allergic conjunctivitis (B:III) o Ocular surgical history (A:III) (prior keratoplasty, cataract surgery, keratorefractive surgery) o Punctal surgery (A:III) o Ocular surface disease (A:III) (e.g., herpes simplex virus, varicella zoster virus, ocular mucous membrane pemphigoid, StevensJohnson syndrome, aniridia, graft-versus-host disease) o Punctal surgery (A:III) o Eyelid surgery (A:III) (e.g. prior ptosis repair, blepharoplasty, entropion/ectropion repair) o Bell palsy (A:III) Systemic history, including o Smoking or exposure to second-hand smoke (A:III) o Dermatological diseases (A:III) (e.g., rosacea) o Technique and frequency of facial washing including eyelid and eyelash hygiene (A:III) o Atopy (A:III) o Menopause (A:III) o Systemic inflammatory diseases (A:III) (e.g., Sjogren’s syndrome, graft-versus- host disease, rheumatoid arthritis, systemic lupus erythematosus, scleroderma) o Other systemic conditions (A:III) (e.g., lymphoma, sarcoidosis) o Systemic medications (A:III) (e.g., antihistamines, diuretics, hormones and hormonal antagonists, antidepressants, cardiac antiarrhythmic drugs, isotretinoin, diphenoxylate/atropine, betaadrenergic antagonists, chemotherapy agents, any other drug with anticholinergic effects) o Trauma (A:III) (e.g., chemical) o Chronic viral infections (B:III) (e.g., hepatitis C, human immunodeficiency virus) o Nonocular surgery (B:III) (e.g., bone marrow transplant, head and neck surgery, trigeminal neuralgia surgery) o Radiation of orbit (B:III) ICO International Clinical Guidelines Page 28 o Neurological conditions (B:III) (e.g., Parkinson’s disease, Bell palsy, Riley-Day syndrome, trigeminal neuralgia) o Dry mouth, dental cavities, oral ulcers (B:III) Initial Physical Exam Visual acuity (A:III) External examination o Skin (A:III) o Eyelids (A:I) o Adnexae (A:III) o Proptosis (B:III) o Cranial nerve function (A:III) o Hands (B:III) Slit-lamp biomicroscopy o Tear film (A:III) o Eyelashes (A:III) o Anterior and posterior eyelid margins (A:III) o Puncta (A:III) o Inferior fornix and tarsal conjunctiva (A:III) o Bulbar conjunctiva (A:III) o Cornea (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 29 Dry Eye Syndrome (Management Recommendations) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Care Management Treat any causative factors that are amenable to treatment as patients with dry eye symptoms often have many contributory factors (A:III) Sequence and combination of therapies is determined based on the patient’s needs and preferences and the treating ophthalmologist’s medical judgment (A:III) For mild dry eye, the following measures are appropriate: o Education and environmental modifications (A:III) o Elimination of offending topical or systemic medications (A:III) o Aqueous enhancement using artificial tear substitutes, gels/ointments (A:III) o Eyelid therapy (warm compresses and eyelid hygiene) (A:III) o Treatment of contributing ocular factors such as blepharitis or meibomianitis (A:III) For moderate dry eye, in addition to above treatments, the following measures are appropriate: o Anti-inflammatory agents (topical cyclosporine (A:I) and corticosteroids, (A:II) systemic omega-3 fatty acids supplements (A:II)) o Punctal plugs (A:III) o Spectacle side shields and moisture chambers (A:III) For severe dry eye, in addition to above treatments, the following measures are appropriate: o Systemic cholinergic agonists (A:I) o Systemic anti-inflammatory agents (A:III) o Mucolytic agents (A:III) o Autologous serum tears (A:III) o Contact lenses (A:III) o Correction of eyelid abnormalities (A:III) o Permanent punctal occlusion (A:III) Monitor patients prescribed corticosteroids for adverse effects such as increased intraocular pressure, corneal melting, and cataract formation (A:III) Patient Education Counsel patients about the chronic nature of dry eye and its natural history. (A:III) ICO International Clinical Guidelines Page 30 Provide specific instructions for therapeutic regimens. (A:III) Reassess periodically the patient's compliance and understanding of the disease, risks for associated structural changes and realistic expectations for effective management, and reinforce education. (A:III) Refer patients with manifestation of a systemic disease to an appropriate medical specialist. (A:III) Caution patients with pre-existing dry eye that keratorefractive surgery may worsen their dry eye condition. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 31 Esotropia (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Ocular symptoms and signs (A:III) Ocular history (date of onset and frequency of the deviation, presence or absence of diplopia) (A:III) Systemic history (review of prenatal, perinatal and postnatal medical factors) (A:III) Family history, including presence of strabismus, amblyopia, extraocular muscle surgery, genetic diseases. (A:III) Initial Physical Exam (Key elements) Visual acuity (A:III) Ocular alignment (at distance and near) and motility (A:III) Extraocular muscle function (A:III) Detection of nystagmus (A:III) Sensory testing (A:III) Cycloplegic retinoscopy/refraction (A:III) Fundoscopic examination (A:III) Care Management Consider all forms of esotropia for treatment and re-establish ocular alignment promptly (A:III) Prescribe corrective lenses for any clinically significant refractive error (A:I) If optical correction does not align the eyes, then surgical correction is indicated (A:III) Start amblyopia treatment before surgery to reduce angle of strabismus or increase likelihood of binocularity (A:III) Follow-up Evaluation Periodic evaluations necessary until visual maturity reached (A:II) Hyperopia should be assessed every 1 to 2 years (A:III) More frequent cycloplegic examinations are indicated in cases with changes in acuity, amblyopia, or unstable alignment (A:III) If the examination has been stable, follow-up evaluations are appropriate every 1 to 2 years during teenage years (A:I) ICO International Clinical Guidelines Page 32 Esotropia Follow-up Evaluation Intervals (A:III) Age (years) 0-1 1-5 5 Interval (months) 3-6 6-12 12-24 Note: More frequent visits may be necessary if amblyopia is present or if there is a recent deterioration of alignment. Patient Education Discuss findings with the patient when appropriate and/or parents/caregivers to enhance understanding of disorder and to recruit them in a collaborative approach to therapy. (A:III) Formulate treatment plans in consultation with the patient and/or family/caregivers. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 33 Exotropia (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Ocular symptoms and signs (A:III) Ocular history (date of onset and frequency of the deviation, presence or absence of diplopia) (A:III) Systemic history (review of prenatal, perinatal and postnatal medical factors) (A:III) Family history, including presence of strabismus, amblyopia, extraocular muscle surgery, genetic diseases (A:III) Initial Physical Exam (Key elements) Visual acuity (A:III) Ocular alignment (at distance and near) and motility (A:III) Extraocular muscle function (A:III) Detection of nystagmus (A:III) Sensory testing (A:III) Cycloplegic retinoscopy/refraction (A:III) Fundoscopic examination (A:III) Care Management Consider all forms of exotropia for treatment and re-establish ocular alignment as soon as possible if deviation is manifest a large percentage of the time. (A:III) Prescribe corrective lenses for any clinically significant refractive error. (A:III) Optimal modes of therapy are not well established. Follow-up Evaluation Periodic evaluations necessary until visual maturity reached (A:III) Intervals are reduced if strabismus is stable (A:III) Includes interval history, tolerance to treatment (if any), and routine examination and testing of ocular motility (A:III) ICO International Clinical Guidelines Page 34 Exotropia Follow-up Evaluation Intervals (A:III) Age (years) 0-1 1-5 5 Interval (months) 3-6 6-12 12-24 Note: More frequent visits may be necessary if patching therapy is being administered, or if there is a recent deterioration of alignment. Patient Education Discuss findings with the patient when appropriate and/or parents/caregivers to enhance understanding of disorder and recruit them in a collaborative approach to therapy. (A:III) Formulate treatment plans in consultation with the patient and/or family/caregivers. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 35 Eye Disease in Leprosy (Initial Evaluation and Management) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Ocular symptoms (decreased vision, epiphora, symptoms of irritation) (A:III) Duration of lagophthalmos (<or>6 months) (A:III) Duration of leprosy (usually from date of diagnosis) (B:III) Type of leprosy (A:III) MDT treatment; what drugs and for how long (A:III) History of leprosy reactions (B:III) Initial Physical Exam Visual acuity (A:III) Eyelids and lid closure (A:III) Corneal sensation (A:III) Conjunctiva (A:III) Sclera (A:III) Pupil (A:III) Nasolacrimal apparatus (A:III) Slit lamp biomicroscopy o Corneal epithelial integrity (A:III) o Corneal nerve beading, stromal opacity (B:III) o Anterior chamber (A:III) o Iris atrophy (A:III) o Iris "pearls" (B:III) o Posterior synechiae (A:III) o Cataract (A:III) Care Management The main important conditions (cataract, lagophthalmos, anterior uveitis) are managed as for any patient, and people with leprosy should be integrated into the normal eye care service, specifically: Cataract should be removed when it adversely affects patient's visual function (A:III) IOL is not contraindicated as long as quality of surgery is good and eye is quiet (A:III) Chronic lagophthalmos should be treated surgically if cornea is compromised or cosmesis is a problem, regardless of severity of lagophthalmos, by whatever procedure the surgeon does best (A:III) Special considerations in a person afflicted with leprosy include: ICO International Clinical Guidelines Page 36 o New onset lagophthalmos (duration <6 months) should be treated with oral prednisolone 25-30 mg per day tapered over 6 months. (A:III) o Acute uveitis should be treated with intensive topical steroid; associated systemic leprosy reaction must be ruled out or treated if present with systemic steroid give dose) (A:III) Patient Education At the end of MDT all patients should be warned that lagophthalmos could develop and understand the risks associated with this. (A:III) Patients with residual lagophthalmos must be told about the risk form exposure and specifically warned about development of red eye and decreased vision. (A:III) Patients should understand risks to eye during reaction and given explicit instructions on where to report if reaction develops. (A:III) All patients should be informed of significance of decreased vision and told to report this to case worker for referral to higher level. (A:III) ICO International Clinical Guidelines Page 37 Idiopathic Macular Hole (Initial Evaluation and Therapy) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Duration of symptoms (A:III) Ocular history: glaucoma or other prior eye diseases, injuries, surgery, or other treatments; prolonged gazing at the sun (A:III) Medications that may be related to macular cysts (A:III) Initial Physical Exam (Key elements) Visual acuity (A:III) Slit-lamp biomicroscopic examination of the macula and the vitreoretinal interface (A:III) Management Recommendations for Macular Hole Stage 1-A Management Observation Follow-up [A:III] Prompt return if new symptoms [A:II] Every 4 to 6 months in the absence of symptoms 1-B Observation Prompt return if new symptoms [A:II] Every 4 to 6 months in the absence of symptoms 2 Surgery [A:II] * 1 to 2 days postoperatively, then 1 to 2 weeks Frequency and timing of subsequent visits varies depending on the outcome of surgery and the patient’s symptoms If no surgery, every 4 to 8 months 3 Surgery [A:I] 1 to 2 days postoperatively, then 1 to 2 weeks Frequency and timing of subsequent visits varies depending on the outcome of surgery and the patient’s symptoms 4 Surgery [A:I] 1 to 2 days postoperatively, then 1 to 2 weeks Frequency and timing of subsequent visits varies depending on the outcome of surgery and the patient’s symptoms * Although surgery is usually performed, observation is also appropriate. ICO International Clinical Guidelines Page 38 Surgical and Postoperative Care if Patient Receives Treatment Inform the patient about relative risks, benefits, and alternatives to surgery, and the need for use of expansile intraocular gas or special patient positioning postoperatively (A:III) Formulate a postoperative care plan and inform the patient of these arrangements (A:III) Inform patients with glaucoma of possible perioperative increase in IOP (A:III) Examine postoperatively within 1 or 2 days and again 1 to 2 weeks after surgery (A:III) Patient Education Inform patients to notify their ophthalmologist promptly if they have symptoms such as increase in floaters, a loss of visual field, or a decrease in visual acuity (A:II) Inform patients that air travel, high altitudes, or general anesthesia with nitrous oxide should be avoided until the gas tamponade is nearly completely gone (A:III) Inform patients who have had a macular hole in one eye that they have a 10% to 20% chance of macular hole formation in the fellow eye, especially if the hyaloid remains attached (A:III) Refer patients with functionally limiting postoperative visual impairment for vision rehabilitation (see www.aao.org/smartsight) and social services (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 39 Keratorefractive Surgery (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Present status of visual function (A:III) Ocular history (A:III) Systemic history (A:III) Medications (A:III) Initial Physical Exam Visual acuity without correction (A:III) Manifest, and where appropriate, cycloplegic refraction (A:III) Computerized corneal topography (A:III) Central corneal thickness measurement (A:III) Evaluation of tear film (A:III) Evaluation of ocular motility and alignment (A:III) Care Management Discontinue contact lenses before preoperative exam and procedure (A:III) Inform patient of the potential risks, benefits, and alternatives to and among the different refractive procedures (A:III) Document informed consent process; patient should be given an opportunity to have all questions answered before surgery (A:III) For LASIK, residual stromal bed thickness should not be less than 250 um (A:III) Check and calibrate instrumentation before the procedure (A:III) Surgeon confirms the identity of the patient, the operative eye, and that the parameters are correctly entered into the excimer laser’s computer (A:III) Postoperative Care Operating surgeon is responsible for postoperative management (A:III) For surface ablation techniques, examine on the day following surgery and every 2 to 3 days thereafter until the epithelium is healed (A:III) For uncomplicated LASIK, examine within 48 hours following surgery, a second visit 1 to 4 weeks postoperatively, and further visits thereafter as appropriate (A:III) ICO International Clinical Guidelines Page 40 Patient Education Discuss the risks and benefits of the planned procedure with the patient. (A:III) Elements of the discussion include the following: Range of expected refractive outcomes Residual refractive error Reading and/or distance correction postoperatively Loss of best-corrected visual acuity Side effects and complications (e.g., microbial keratitis, sterile keratitis, keratectasia Changes in visual function not necessarily measured by Snellen acuity, including glare and function under low-light conditions Night vision symptoms (e.g., glare, haloes) developing or worsening; careful consideration should be given to this issue for patients with high degrees of ametropia or for individuals who require a high level of visual function in low-light conditions Effect on ocular alignment Dry eye symptoms developing or worsening Monovision advantages and disadvantages (for patients of presbyopic age) Conventional and wavefront-guided ablations advantages and disadvantages Advantages and disadvantages of same-day bilateral keratorefractive surgery versus sequential surgery. Because vision might be poor for some time after bilateral same-day photorefractive keratectomy, the patient should be informed that activities such as driving might not be possible for weeks Postoperative care plans (setting of care, providers of care) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 41 Ocular HIV/AIDS Related Diseases (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) General - Initial Exam History Age (B:III) Ocular symptoms including laterality (A:III) Systemic symptoms (A:III) Complete review of systems (A:III) Prior ocular history (A:III) Prior medical history (A:III) Prior surgical history (B:III) History of other sexually transmitted diseases (A:III) History of AIDS-defining illnesses or complications (A:III) Method of HIV acquisition (B:III) Duration of HIV infection (A:III) Past and current risk factors – sexual behavior, intravenous drug abuse, transfusion history (A:III) Current anti-HIV regimen – duration and compliance (A:III) Current medications (A:II) Current CD4 count (A:II) Current viral load (A:II) Medication allergies (B:III) General - Initial Physical Exam General appearance (A:III) External examination – face, ocular adnexa (A:III) Lymphatics – preauricular and submandibular nodes (A:III) Visual acuity (A:III) Extraocular motility (A:III) Confrontation visual fields (A:III) Eyelids – lid closure, interpalpebral fissure height (B:III) Lacrimal gland (B:III) Evaluation of tear film – Schirmer, rose bengal and fluorescein staining (A:III) Nasolacrimal function (B:III) Slit-lamp examination o Eyelid margins (A:III) o Conjunctiva (A:III) o Sclera (A:III) o Cornea (A:III) ICO International Clinical Guidelines Page 42 o Anterior chamber (A:III) o Iris (A:III) o Lens (A:III) o Anterior vitreous (A:III) Dilated ophthalmoscopic examination o Vitreous – cell/flare, blood, condensations (A:III) o Optic disc (A:III) o Retinal vasculature (A:III) o Macula/fovea (A:III) o Peripheral retina with scleral depression (A:III) o Choroid (A:III) General - Diagnostic Tests HIV infection – for increased risk populations and/or suspected infection o Anti-HIV ELISA to screen for infection, followed by confirmation with Western blot (A:II) AIDS o Presence of AIDS-defining illness(es) (A:III) o CD4 (< 200 cells/µl, per CDC criteria) (A:II) Known HIV/AIDS patient o CD4 count (A:II) o Viral load (A:II) General - Care Management Management of HIV/AIDS should involve a multidisciplinary team, including an infectious disease specialist and an ophthalmologist (A:III) Anti-Retroviral Therapy (ART) or Highly Active Anti-Retroviral Therapy (HAART), where available (A:II) Emphasis on prevention of disease transmission (A:III) Identification and treatment of HIV/AIDS associated illnesses/infections (particularly tuberculosis and syphilis) (A:II) HIV Retinopathy – Initial Exam History CD4 count (A:II) Ocular symptoms – usually asymptomatic (B:III) HIV Retinopathy – Initial Physical Exam Visual acuity (A:III) Slit lamp examination (B:III) Dilated ophthalmoscopic examination (A:II) Screen for other HIV/AIDS related illnesses/infections (B:III) HIV Retinopathy - Care Management Treat immune compromise with HAART (A:II) ICO International Clinical Guidelines Page 43 Consider corticosteroids (B:III) or focal laser (A:II) for macular edema HIV Retinopathy – Follow-up Evaluation Lesions usually resolve over weeks to months (A:II) Dilated ophthalmoscopic examination every 3 months for CD4 counts persistently below 50 cells/µl (A:II) Cytomegalovirus (CMV) Retinitis – Initial Exam History Interval since AIDS diagnosis (A:II) History of CMV related systemic complications (A:II) Ocular symptoms –blurred vision, floaters, photopsias, scotomata (A:II) Cytomegalovirus (CMV) Retinitis – Initial Physical Exam Visual acuity (A:II) Cornea for small endothelial deposits (B:III) Anterior chamber for signs of inflammation (A:II) Dilated ophthalmoscopic examination of both eyes – including optic disc, macula, and retinal periphery. The choroid should be examined to rule out co-infection with other agents (A:II) Cytomegalovirus Retinitis – Diagnostic Tests CD4 count – typically less than 50 cells/µl (A:II) Cytomegalovirus Retinitis – Ancillary Testing Fundus photography may be useful to document disease progression or response to treatment and fluorescein angiography as indicated to evaluate for the presence of macular edema or ischemia (A:III) Test for syphilis and vitreous biopsy for other causes of necrotizing retinitis (varicella zoster virus, herpes simplex virus, toxoplasmosis) when diagnosis uncertain (A:II) Cytomegalovirus – Care Management Main objectives include direct treatment of CMV retinitis with anti-CMV medications, and improvement of immune status with initiation/optimization of HAART if not already taking anti-retroviral therapy (A:II) To reduce the possibility of immune recovery uveitis, patients with newly diagnosed CMV retinitis who are not on HAART should be treated with antiCMV medications until the retinitis is inactive, or at least less active. HAART should then be initiated (A:II) Also, in cases with expected persistent immune suppression, e.g. poor response to or unavailability of ART, immediate treatment is indicated (A:II) Local anti-CMV therapy, as might be achieved using intravitreal injection of ganciclovir or foscarnet, may be used immediately when active CMV retinitis either involves or threatens the optic disc or macula (A:II) Induction followed by indefinite maintenance therapy in cases of persistent immune suppression (A:II) ICO International Clinical Guidelines Page 44 Ganciclovir o Intravenous – 5 mg/kg every 12 hours for 2 to 3 weeks, then 5 mg/kg/day 5 to 7 times per week indefinitely. (A:I) Monitor for leukopenia, the risk of which may be lessened by administering leukocyte-stimulating factors such as granulocyte colony-stimulating factor (A:II) o Intraocular – 2 to 2.5 mg/0.1 ml intravitreal injection twice per week until inactive, then weekly (A:I) o Intravitreal sustained-release implant (Vitrasert) – 4.5 mg implant that releases 1 µg/hr for eight months. This should be combined with oral valganciclovir therapy for systemic coverage (A:I) Foscarnet o Intravenous – 60 mg/kg every 8 hours or 90 mg/kg every 12 hours for 14 days, then 90 to 120 mg/kg/day. Monitor for renal toxicity (A:I) o Intraocular – 1.2 mg/0.05 ml (or 2.4 mg/0.1 ml) (A:I) Valganciclovir o Oral – 900 mg twice daily for 2 weeks, (A:I) then 900 mg daily indefinitely. (A:II) Monitor for leukopenia (A:II) Cytomegalovirus – Follow-up Evaluation Recurrence is very common, and patients being treated with anti-CMV medications should be evaluated monthly (A:II) Intervals may be extended when CD4 counts are elevated, anti-CMV medications are discontinued, and the disease remains inactive in the setting of immune recovery (A:II) Visual symptoms (A:II) CD4 count and HIV viral load (A:II) Review of systems for CMV related systemic complications or drug-induced side effects (A:II) Cytomegalovirus – Follow-up Examination Visual acuity (A:II) Slit lamp examination (B:II) Ophthalmoscopic examination – including the macula and peripheral retina (A:II) Serial fundus photography (B:II) Cytomegalovirus – Follow-up Management No treatment can eliminate CMV from the eye (A:II) Patient education about the symptoms of CMV retinitis is crucial (A:III) For recurrences, first line is re-induction with the same therapy in the absence of side effects or evidence of drug resistance (A:II) Persistent or progressive retinitis after 6 weeks of induction-level therapy implies resistance or incorrect diagnosis (A:II) UL97 and UL54 mutations in CMV DNA are associated with relative ganciclovir resistance (A:II) ICO International Clinical Guidelines Page 45 Anti-CMV drugs may be discontinued in patients on HAART with no signs of active CMV retinitis in whom CD4 counts are above 100 to 150 cells/µl for at least three to six months (A:II) Tuberculosis – Initial Exam History CD4 count (typically < 200 cells/µl) (A:II) Visual and ocular symptoms (A:II) History M. Tuberculosis infection, systemic complications, or exposure (A:II) Tuberculosis – Initial Physical Exam Visual acuity (A:III) External examination – including eyelids and adnexa (B:III) Slit lamp examination (B:III) Intraocular pressure (B:III) Dilated ophthalmoscopic examination - optic disc, macula, retinal periphery, and choroid (A:II) Tuberculosis – Diagnostic Tests Presumptive diagnosis by clinical examination combined with PPD skin testing and chest x-ray (A:II) Requires a high index of clinical suspicion (B:III) Consider leukocyte stimulation based assays where available, particularly ® when PPD skin testing is unreliable (QuantiFERON -TB Gold Test; ® T.SPOT-TB test) (A:II) Definitive diagnosis requires biopsy with histopathologic examination (A:III) Tuberculosis – Ancillary Testing Fluorescein angiography to evaluate suspected retinal vasculitis (A:III) Indocyanine green angiography may be helpful to detect subclinical choroidal involvement (A:III) Optical coherence tomography to diagnose and monitor for cystoid macular edema (A:III) Tuberculosis – Care Management Systemic treatment is indicated with rifampin (500 mg/day for weight > 50 kg and 600 mg/day for weight < 50 kg), isoniazid (5 mg/kg/day), pyrimethamine (25 to 30 mg/kg/day, and ethambutol (15 mg/kg/day) for 2 months then rifampin and isoniazid for another 4 to 7 months (A:II) Oral prednisone (1 mg/kg/day), taper as directed by clinical response (A:II) Initiate/optimize HAART if not already taking anti-retroviral therapy (A:II) Coordinate care with an infectious disease specialist (A:III) Tuberculosis – Follow-up Evaluation Monitor all patients for medication toxicity (A:II) Examine patients monthly until there is significant clinical improvement ICO International Clinical Guidelines Page 46 (A:III) Toxoplasmosis (T. gondii) – Initial Exam History CD4 count (typically < 200 cells/µl) (A:II) Visual and ocular symptoms (A:III) History of T. gondii infection, systemic complications, or exposure (A:III) Toxoplasmosis – Initial Physical Exam Visual acuity (A:II) Intraocular pressure (B:II) Slit lamp examination (C:II) Dilated ophthalmoscopic examination (A:II) Toxoplasmosis – Diagnostic Tests Primarily a clinical diagnosis (A:III) Serologic testing for anti-T. gondii IgM/IgG antibodies (A:II) In unclear cases, can perform PCR on aqueous or vitreous for T. gondii DNA (B:II) Toxoplasmosis – Care Management Initial treatment involves oral antimicrobials for 4 to 6 weeks. Options include: o Trimethoprim/sulfamethoxazole (800/160) 500 mg PO twice daily (A:II) o Pyrimethamine (100 mg loading dose given over 24 hours, followed by 25 to 50 mg daily) and sulfadiazine (1 g given four times daily) for 4 to 6 weeks. Should be given concurrently with folinic acid (3 to 5 mg twice weekly) to prevent leukopenia and thrombocytopenia (B:II) o Clindamycin (300 mg orally every 6 hours) for 3 or more weeks (B:II) o Atovaquone (750 mg orally four times daily) for 3 months (B:II) o Consider use of Azithromycin in patients with sulfa-related allergy (B:III) Maintenance therapy with at least one of the above medications is recommended for patients with ocular toxoplasmosis who remain severely immunodeficient (A:III) Oral corticosteroids may be considered when inflammation contributes to vision loss (vitritis, vasculitis, serous retinal detachment, lesion involving or threatening the optic disc or macula) - 0.5 mg/kg/day with taper, initiated and ended concurrent with antimicrobial therapy (A:III) Topical corticosteroids may be considered for significant anterior chamber inflammation (A:III) Toxoplasmosis – Follow-up Evaluation Initial follow-up should be one week after initiation of treatment, then as indicated by examination and treatment response (A:III) Lesions typically take several months to resolve (A:III) ICO International Clinical Guidelines Page 47 Syphilis – Initial Exam History CD4 count (often less than 200 cells/µl). However, ocular syphilis in the setting of HIV/AIDS may occur at any CD4 count. (A:II) Visual symptoms and rapidity of onset (A:III) Previous syphilis infection, related complications, or exposure (A:III) History of other sexually-transmitted diseases (B:III) Syphilis – Initial Physical Exam Visual acuity (A:II) Intraocular pressure (B:II) Slit lamp examination (B:III) Dilated ophthalmoscopic examination (A:II) Syphilis – Diagnostic Tests Both non-treponemal (RPR or VDRL) and treponemal (MHA-TP or FTAABS) testing should be obtained (up to one-third of patients with syphilitic uveitis have a negative non-treponemal test) (A:II) Patients with profound immune suppression may present with seronegative syphilis (A:II) CSF examination (RPR or VDRL) in all HIV/AIDS patients with ocular syphilis (A:II) Syphilis – Care Management Treat as neurosyphilis (A:II) Involve an infectious disease specialist in coordinating systemic management (A:III) First-line treatment is with IV penicillin G, 18 to 24 million units for 14 days (A:II) Worsening ocular inflammation following the initiation of penicillin may be indicative of a Jarish-Herxheimer reaction (A:II) Syphilis – Follow-up Evaluation Serial serum and CSF antibody levels every month for 3 months, then every 6 months until CSF cell count normalizes and CSF VDRL/RPR becomes non-reactive (A:III) Serum quantitative nontreponemal testing every 3 months for one year, then yearly (A:III) Maintenance therapy is not necessary or recommended (B:II) ICO International Clinical Guidelines Page 48 Table 1. Adnexal Manifestations of HIV/AIDS (A:III unless otherwise indicated) CD4 Entity History Examination count Key Findings Diagnostic workup Management Follow-up IV acyclovir 10 mg/kg every 8 hours for 7 days (A:II) Alternatives: valacyclovir (1 gram PO 3 times daily) or oral acyclovir (800 mg PO 5 times daily); close follow-up for signs of disseminated infection including cerebritis (A:II) Patient receiving high doses of valacyclovir should be monitored for TTP/HUS (A:II) Maintain on oral acyclovir 800 mg, 3 to 5 times daily indefinitely (A:II) Alternatively, can maintain on oral famciclovir or valacyclovir Topical corticosteroids for iridocyclitis and/or stromal keratitis (A:II) Immune reconstitution (A:II) Indications for treatment: 1) loss of normal lid function, 2) discomfort, 3) cosmesis Treatment depends on the size and location of lesions (A:II) Treatment options include intralesional vinblastine or interferon-alpha, local radiation therapy, excision, and cryotherapy (A:II) Systemic chemotherapy if disseminated disease (A:II) Reduce size of large lesions prior to excision Observe for post-herpetic neuralgia Serial DOE Eyelid lesions commonly recur within 6 to 8 weeks (A:II) Routine preand postoperative examinations Herpes Zoster Ophthalmicus < 200 cells/µ l (A:II) Prior zoster infection (A:II) Age Periorbita Eyelids SLE Sclera AC DOE Vesiculobullous dermatitis in CN V1 distribution (A:II) Complications include keratitis, uveitis, scleritis, retinitis, and optic neuritis (A:II) Hemorrhagic hypopyon (B:II) Clinical examination Can confirm diagnosis with viral culture, Tzanck smear, PCR (A:II) Kaposi’s Sarcoma < 200 cells/µ l (A:II) Manner of HIV acquisition (sexual more common) (B:II) Dry eye symptoms (B:II) Pain (rare) (B:II) Reduced vision (rare) (C:II) Highly vascularized tumor of the skin or mucous membranes (A:II) May involve eyelids and/or conjunctiva (A:II) Eyelid lesions may appear as a purplish nodule (A:II) Conjunctival lesions can mimic SCH (B:II) Clinical examination Biopsy with histopatholog y of suspicious lesions (A:II) Molluscum Contagiosum Any (A:II) History of molluscum exposure (A:II) Immune reconstitution (A:II) Topical agents: liquid nitrogen, trichloracetic acid, cantharadin (A:II) Incision with curettage, excision, or cryotherapy (A:II) Any (A:II) Geographic location – higher risk in Africa (A:II) History of HPV infection (B:II) Biopsy with histopatholog ic examination (A:II) Wide excision with cryotherapy for non-invasive lesions (A:II) Frozen section pathologic examination (A:II) Alternatives include MMC, 5-FU, and interferon (A:II) Cutaneous or Conjunctival Lymphoma Any (A:II) Presence/history of systemic lymphoma (A:II) Ocular symptoms VA External examination SLE DOE (C:III) Papulonodular dermatitis of the skin and mucous membranes (A:II) Multiple small umbilicated lesions (A:II) Papilliform, gelatinous, or leukoplakic lesion at the interpalpebral limbus (A:II) SCC: lesion is more extensive, feeder vessels more common (A:II) Erythematous lesion of the eyelid or conjunctiva (A:II) Clinical examination Squamous Cell Carcinoma (SCC) and Conjunctival Intraepithelial Neoplasia (CIN) External examination Lymphatics Oral cavity SLE Eyelids Lacrimal gland Skin of face and upper body Periorbita SLE Trunk and genitalia (B:III) VA External examination SLE Gonioscopy Biopsy (A:II) Systemic evaluation (A:II) Radiation (A:II) Chemotherapy (A:II) Involve an oncologist Conjunctival Microvasculopath y Any (A:II) Typically asymptomatic (B:II) SLE DOE (B:III) Inferior perilimbus (A:II) Segmental vascular dilation and narrowing (A:II) Comma-shaped vascular fragments (A:II) Clinical examination Not indicated Recurrences are common (A:II) As directed by treatment in coordination with oncologist Unnecessary Table 1. Adnexal Manifestations of HIV/AIDS (A:III unless otherwise indicated) CD4 Entity History Examination count Conjunctival Microvasculopath y Any (A:II) Typically asymptomatic (B:II) SLE DOE (B:III) Conjunctivitis Any (A:II) Symptoms of irritation, discharge (A:II) VA SLE Atopic dermatitis Any (A:II) History of atopic triad: dermatitis, rhinitis, asthma Typical symptomatology (B:II) Triggers (A:II) Use of Indonavir (B:II) Itching, FBS, redness External examination Eyelids Key Findings Inferior perilimbus (A:II) Segmental vascular dilation and narrowing (A:II) Comma-shaped vascular fragments (A:II) Microaneurysms (A:II) Blood column granularity (A:II) Conjunctival erythema (A:II) Watery, mucoid, or purulent discharge (A:II) Pruritis and excematous changes of the periorbital skin (A:II) Diagnostic workup Management Follow-up Clinical examination Not indicated Unnecessary Clinical examination Culture and gram stain of discharge (A:II) Clinical examination Guided by results of gram stain and culture Clinical examination should be used to initiate empiric treatment Every 5 to 7 days until resolution Topical corticosteroids (i.e. hydrocortisone 0.5% cream) (A:II) Topical calcineurin inhibitors, Elidel (pimecrolimus) and Protopic (tacrolimus) are contraindicated in immunosuppressed patients (B:II) Serial examinations until resolution External Crusting and erythema of Clinical Lid hygiene Every 4 examination eyelid margins (A:II) examination weeks Consider culture in high-risk patients Eyelids SLE – including tear film and cornea SLE = slit lamp examination, AC = anterior chamber, DOE = dilated ophthalmoscopic examination, PCR = polymerase chain reaction, PO = per os (by mouth), IV = intravenous, TTP/HUS = thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, HIV = human immunodeficiency virus, SCH = subconjunctival hemorrhage, HPV = human papilloma virus, VA = visual acuity, MMC = mitomycin C, 5-FU = 5-fluorouracil, FBS = foreign body sensation = visual acuity, MMC = mitomycin C, 5-FU = 5-fluorouracil, FBS = foreign body sensation Blepharitis Any (A:II) ICO International Clinical Guidelines Page 50 Table 2. Corneal and Anterior Segment Manifestations of HIV/AIDS (A:III unless otherwise indicated) Entity CD4 count Examination Key Findings Lagophthalmos and reduced blink rate (B:II) Diminished tear meniscus (B:III) Rapid TBUT (A:II) Interpalpebral staining with rose bengal or fluorescein (A:II) Dendritic epithelial keratitis (A:II) Decreased corneal sensation (A:II) Elevated IOP (B:II) Iris atrophy (B:II) May present with a mild conjunctivitis or anterior uveitis (B:II) 1/3 develop stromal involvement (B:II) Dendritic epithelial keratitis, which may be larger in HIV+ patients (A:II) Limbal involvement (B:II) Clinical examination Schirmer testing (A:II) TBUT (B:II) Rose bengal or fluorescein staining (A:II) Artificial tears Long-acting lubricants Consider punctual occlusion in resistant cases As dictated by examination Clinical examination Corneal sensation (A:II) May confirm with viral culture, DFA, PCR (B:II) Acyclovir 800 mg PO 5 times daily or 10 mg/kg IV tid (A:II) Foscarnet IV for resistant cases (A:II) Consider maintenance dose of acyclovir (600 mg PO tid) (A:II) Infectious dendrites can be treated with oral (as described above) or topical antiviral medications (trifluridine 1% 9 times daily) (A:II) Every 1 to 7 days until resolution, then every 6 months Observe for stromal and/or neurotrophic keratitis and postherpetic neuralgia (B:III) Every 1 to 7 days until resolution HSV appears to recur more frequently in HIV/AIDS patients (A:II) Epithelial defect with stromal infiltrate (A:II) Tend to be more severe and bilateral in HIV+ patients (A:II) Punctate epithelial keratopathy (A:II) Mild papillary conjunctivitis (A:II) Mild AC inflammation (A:II) Clinical examination Culture and gram stain (A:II) Topical trifluridine 1% 9 times daily or Acyclovir ointment 5 times daily (A:II) May treat with oral acyclovir (400-800 mg PO 5 times daily) alone (A:II) Consider lesion debridement (B:III) Long term suppression with acyclovir 400 mg PO bid for 1 year (A:I) Guided by culture results (B:II) Aggressive treatment with topical fortified antibiotics and/or antifungal agents (A:II) Immune reconstitution (A:II) Directed treatment options include: topical propamidine isethionate, topical fumagillin, oral albendazole, oral itraconazole (A:II) ConsiderICO debulking (B:III) International Serial examinations until resolution Keratoconjunctivitis sicca Any (A:II) Typical history History of HIV encephalopathy (B:III) Duration of infection with HIV (B:III) VA Periorbita (B:III) Lacrimal gland (C:III) Eyelids (A:II) SLE with fluorescein Viral keratiti s Any (B:II) Reduced vision Ocular symptoms Presence or recent history of zoster dermatitis (A:II) Prior history of zoster or herpes infection (A:II) VZV VA IOP Periorbita Eyelids/lash es Corneal sensation SLE with fluorescein DOE with scleral depression HSV Gonorrhea Syphilis Tuberculosis Cryptococcu s Any (B:II) Reduced vision Discharge Timing of symptom onset (B:III) VA SLE with fluorescein DOE (C:III) Microsporidial keratitis < 100 cells/ µl (A:II) Reduced vision Ocular symptoms – FBS, irritation, photophobia VA SLE with fluorescein Bacteri al or fungal keratiti s Vortex keratopathy (Phospholipidosis) Any (B:II) Diagnostic workup History FBS Medication history (eg. amiodarone, chloroquine, VA SLE Characteristic whorl-like pattern of gray-white Scraping or biopsy of suspicious corneal and conjunctival lesions (A:II) Giemsa stain (A:II) History and clinical examination Management Follow-up Daily follow-up until substantial improvement High risk for corneal perforation (A:II) Clinical Guidelines Page 51 Reduce or discontinue offending medication, if possible (A:II) Lesions resolve slowly Table 2. Corneal and Anterior Segment Manifestations of HIV/AIDS (A:III unless otherwise indicated) Entity CD4 count History Examination Key Findings Diagnostic workup Management Follow-up FBS VA Characteristic History and Reduce or discontinue offending Lesions resolve whorl-like pattern clinical medication, if possible (A:II) slowly Medication history (eg. SLE of gray-white examination amiodarone, chloroquine, subepithelial chlorpromazine, corneal deposits ganciclovir, acyclovir) (A:II) (A:II) Drug-associated uveitis Any Reduced vision VA AC inflammation History and Topical corticosteroids with or Serial every 1 to (A:II) Cidofovir (A:II) clinical without dose reduction of 2 weeks Medication history, SLE examination offending medication (A:II) examinations Rifabutin including daily doses Rifabutin IOP (B:III) until resolution (A:II) associated Usually unnecessary to Terbinafine DOE (B:III) Immune status (B:III) hypopyon (A:II) discontinue offending medication (B:III) Duration on HAART Mydriatic agent (B:III) History of CMV retinitis (A:II) HIV = human immunodeficiency virus, VA = visual acuity, SLE = slit lamp examination, TBUT = tear film breakup time, VZV = varicella zoster virus, HSV = herpes zoster virus, IOP = intraocular pressure, DOE = dilated ophthalmoscopic examination, DFA = direct fluorescent antibody, PCR = polymerase chain reaction, PO = per os (by mouth), IV = intravenous, AIDS = acquired immunodeficiency syndrome, FBS = foreign body sensation, AC = anterior chamber, HAART = highly active antiretroviral therapy, CMV = cytomegalovirus Vortex keratopathy (Phospholipidosis) Any (B:II) ICO International Clinical Guidelines Page 52 Table 3. Posterior Manifestations of HIV/AIDS (A:III unless otherwise indicated) Entity CD4 count History Examination HIV retinopathy < 50 cells/µl (A:II) Visual and ocular symptoms (typically asymptomatic) (B:III) VA SLE (B:III) DOE (A:II) CMV retinitis < 50 cells/µl (A:II) Duration of AIDS (A:II) History of systemic CMV infection (A:II) Ocular symptoms including blurred vision, gradual visual field loss, photopsia, and floaters (A:II) VA (A:II) SLE (B:II) DOE (A:II) Toxoplasmosis < 200 cells/µl (A:II) Visual symptoms (A:II) Exposure to undercooked meat or cats (A:II) Tuberculosis < 200 cells/µl (A:II) Visual symptoms (A:II) History of M. Tuberculosis infection, systemic complications, VA External examination (B:III) SLE (B:III) IOP (B:III) DOE (A:II) VA (A:II) IOP (B:II) SLE (C:II) DOE (A:II) Key Findings Diagnostic workup Management Follow-up Conjunctival microvascular changes (B:II) CWS (A:II) IRH (A:II) MAs (A:II) Retinal ischemia (A:II) CME (A:II) Geographic thickening and opacification of the retina (A:II) Mild anterior chamber and vitreous inflammation (B:II) Characteristic linear or stellate KP (B:II) 3 main types: granular retinitis with satellite lesions, hemorrhagic retinitis with prominent edema, or perivascular retinitis (A:II) Clinical diagnosis Improve immune status with HAART (A:II) Screen for other infections/illnesses Consider corticosteroids (B:III) or focal laser (A:II) for macular edema Primarily a clinical diagnosis CD4 count (A:II) Rule out syphilis and other causes of retinitis (A:II) Consider vitreous biopsy in challenging cases Moderate-to-severe AC and vitreous inflammation (B:II) Retinochoroiditis with a relative lack of retinal hemorrhage (A:II) Smooth leading edge without satellite lesions (B:II) A rare cause of isolated anterior uveitis(C:II) Vitritis (A:II) Choroidal tubercles and tuberculomas (A:II) Overlying exudative retinal detachment (B:II) Retinal periphlebitis (A:II) Clinical diagnosis Anti-Toxoplasma IgM/IgG (A:II) PCR of aqueous in unclear cases (B:II) Improve immune status, although CMV cannot be eliminated from consider delay of HAART in the eye (A:II); patient education for HAART-naïve patients until recurrences is crucial retinitis is improved to reduce the Reevaluate patients monthly while risk of IRU (A:II) treating with anti-CMV Immediate treatment if persistent medications (A:II) immune suppression is expected Extend visit intervals when CD4 (A:II) counts are elevated, anti-CMV Induction followed by maintenance medications are discontinued, and (A:II) the disease remains inactive in the Ganciclovir: IV (5 mg/kg every 12 setting of immune recovery (A:II) hours for 3 weeks, then 5 Consider serial fundus mg/kg/day) (A:I); IO (2photography (B:II) 2.5mg/0.1ml twice weekly until Treat recurrences with reinactive) (A:I); intraocular implant induction of same therapy, unless (A:I), combine with oral anti-CMV contraindicated due to side effects medications for systemic coverage or resistance (A:II) (A:II) May discontinue maintenance Foscarnet: IV (60 mg/kg every 8 therapy in patients without active hours or 90 mg/kg every 12 hours CMV retinitis and at least 6 months for 14 days, then 90 to 120 of CD4 cell counts above 150 mg/kg/day) (A:I); IO (1.2 mg/0.05 cells/µl (A:II) ml) (A:I) Valganciclovir: PO (900 mg bid for 2 weeks, then 900 mg daily). Monitor for leukopenia (A:II) Trimethoprim/sulfamethoxazole Initially every 3 to 5 days, then as (800/160) 500 mg PO bid for 4 to 6 indicated by examination weeks (A:II) Maintenance therapy with at least Pyrimethamine and one medication is recommended sulfamethoxazole for 4 to 6 weeks for all patients with persistent (option of combination with severe immune deficiency azithromycin) (B:II) Clindamycin (300 mg PO every 6 hours) for 3 or more weeks (B:II) Atovaquone (750 mg PO qid) for 3 months (B:II) Systemic treatment with rifampin Monitor all patients for drug (500 mg/day for weight > 50 kg toxicity (A:II) ICO International and 600 mg/day for weight < 50 Clinical ExamineGuidelines patients monthly until a 53 kg), isoniazid (5 mg/kg/day), significant Page improvement pyrimethamine (25 to 30 mg/kg/day, and ethambutol (15 mg/kg/day) for 2 months then Presumptive diagnosis combined with PPD skin testing and CXR (A:II) Consider IGRAs (eg. QuantiFERON®-TB Gold; T.SPOT-TB®) Lesions spontaneously resolve over weeks to months (A:II) DOE every 3 months for CD4 counts persistently < 50 cells/µl (A:II) Table 3. Posterior Manifestations of HIV/AIDS (A:III unless otherwise indicated) Entity CD4 count History Examination Key Findings Diagnostic workup Management Systemic treatment with rifampin (500 mg/day for weight > 50 kg and 600 mg/day for weight < 50 kg), isoniazid (5 mg/kg/day), pyrimethamine (25 to 30 mg/kg/day, and ethambutol (15 mg/kg/day) for 2 months then rifampin and isoniazid for another 4 to 7 months (A:II) PO prednisone (1 mg/kg/day), taper as directed by clinical response (A:II) Immune reconstitution (A:II) Involve an infectious disease specialist Treat as neurosyphilis (A:II) Involve an infectious disease specialist IV penicillin G, 18 to 24 million units for 14 days (A:II) Tuberculosis < 200 cells/µl (A:II) Visual symptoms (A:II) History of M. Tuberculosis infection, systemic complications, or exposure (A:II) VA External examination (B:III) SLE (B:III) IOP (B:III) DOE (A:II) Vitritis (A:II) Choroidal tubercles and tuberculomas (A:II) Overlying exudative retinal detachment (B:II) Retinal periphlebitis (A:II) Presumptive diagnosis combined with PPD skin testing and CXR (A:II) Consider IGRAs (eg. QuantiFERON®-TB Gold; T.SPOT-TB®) (B:II) FA, ICG, and OCT when indicated (see text) (B:III) Syphilis Often < 200 cells/µl, but can vary (A:II) Visual symptoms (A:II) Sexual history (B:II) VA (A:II) IOP (B:II) SLE (B:II) DOE (A:II) Iridocyclitis or diffuse inflammation (A:II) Necrotizing retinitis (A:II) Subretinal plaque (B:II) Papillitis, optic neuritis, or neuroretinitis (A:II) RPR or VDRL (A:II) FTA-ABS or MHATP (A:II) Consider seronegative syphilis (B:II) CSF examination (A:II) Non-CMV necrotizing herpetic retinitis PORN: < 50 cells/µl (A:II) ARN: > 50cells/µl (A:II) History of HZO or dermatitis (A:II) History of herpes encephalitis (B:II) Visual symptoms (pain, vision loss, new floaters or scotomata) (A:II) VA (A:II) IOP (B:III) SLE (B:III) DOE (A:II) Retinal whitening with occasional hemorrhages (A:II) Multiple large confluent areas of retinitis (A:II) Rapid progression (A:II) Prominent (ARN) or minimal (PORN) vitreal inflammation (B:II) Clinical diagnosis Aqueous or vitreous biopsy for PCR-based analysis can aid in diagnosis (B:II) Note location and extent of involved retina Immune recovery uveitis >100 cells/µl or 50 cell/µl increase (A:II) VA (A:II) IOP (B:II) SLE (A:II) DOE (A:II) Panuveitis with vitreous predominance (A:II) May be complicated by TRD, RNV, ERM formation, or CME (A:II) Diagnosis based on history and clinical examination Consider FA to rule out CME (B:III) Pneumocystis choroiditis < 200 cells/µl (A:II) History/extent of CMV retinitis (A:II) History of cidofovir use (B:II) History of aerosolized pentamidine use (A:II) Multiple welldemarcated yellowish choroidal lesions in the posterior pole (A:II) Lack of iritis, vitritis, or vasculitis (A:II) Clinical diagnosis Consider workup for systemic disease, including CXR, ABG analysis, abdominal CT, and liver function testing VA (A:II) SLE (C:III) DOE OU (A:II) Induction with high-dose intravenous acyclovir (15 mg/kg q 8 hours) (A:II) Intraocular ganciclovir (2 to 2.5mg/0.1ml twice weekly) or foscarnet (1.2 mg/0.05ml) as indicated (A:II) Maintenance with long term oral valacyclovir or valganciclovir may be considered (B:II) Patients receiving high doses of valacyclovir should be monitored for TTP/HUS (A:II) Patients receiving valganciclovir should be monitored for leukopenia (A:II) Topical, periocular, or intraocular corticosteroids (A:II) PPV for VMTS, ERM, cataract, PVR (A:II) TMP-SMX or pentamidine (4 mg/kg/day) (A:II) ICO International Follow-up Monitor all patients for drug toxicity (A:II) Examine patients monthly until a significant improvement Serial serum and CSF antibody levels – every month for 3 months, then every 6 months until CSF cell count normalizes and CSF VDRL becomes non-reactive (A:III) Maintenance therapy not recommended (B:II) Monitor patients for a JarishHerxheimer reaction (A:II) Can progress rapidly (A:II) Daily until significant improvement, then weekly Weekly until resolution Monthly until resolution – usually 1 to 3 months Following a 3 week IV induction regimen, maintain on oral Clinical Guidelines prophylactic treatment until Page recovers 54 immune system (CD4 count above 200 cells/µl) (A:II) Entity CD4 count History Examination Key Findings Diagnostic workup Management Follow-up Cryptococcus < 50 cells/µl (A:II) Visual symptoms including vision loss, diplopia, and new scotomata (A:II) Headache/ meningismus (A:II) VA SLE (B:II) EOM (A:II) DOE (A:II) Signs and symptoms of central nervous system infection (A:II) Papilledema (A:II) Retrobulbar optic neuritis (B:II) Multifocal choroiditis (A:II) Other findings may include iritis, iris mass, vitritis, necrotizing retinitis, and eyelid or conjunctival mass (B:II) Clinical diagnosis CNS symptoms – think of cryptococcal meningitis (A:II) Skin lesions – biopsy (B:II) Isolated choroiditis: IV fluconazole, 400 mg/day and IV flucytosine, 100 to 150 mg/kg/day for 10 weeks (A:II) Associated with meningitis: IV amphotericin B, 0.7 to 1 mg/kg/day and IV flucytosine 100 mg/kg/day for 2 weeks followed by IV fluconazole for at least 10 weeks (A:II) Weekly until resolution HIVassociated retinitis > 120 cells/µl (A:II) Visual symptoms (A:II) VA IOP (C:II) SLE (C:III) DOE (A:II) Clinical diagnosis Rule out other entities, particularly syphilis (A:II) Antiretroviral therapy should lead to regression (A:II) Weekly until resolution Intraocular lymphoma < 500 cells/µl (A:II) Floaters (A:II) Vision loss (A:II) VA (A:II) DOE (A:II) Peripheral multifocal retinitis (A:II) Retinal vasculitis (A:II) Mild vitreous inflammation (B:II) Lack of retinal hemorrhage (B:II) Slow progression (B:II) Necrotizing retinitis (A:II) Retinal vasculitis (B:II) Subretinal mass (A:II) Vitritis (A:II) Multifocal choroiditis (A:II) Poor response to treatment (A:II) CNS symptoms (A:II) Radiation and chemotherapy (A:II) Involve Oncology Immune reconstitution (B:II) Monthly DOE Poor prognosis (A:II) History/extent of necrotizing retinitis (A:II) History of trauma (B:II) History of myopia (B:II) VA (A:II) SLE (B:II) DOE (A:II) Workup and treatment for infectious processes (A:II) AC tap for IL-10 (B:II) Vitreous biopsy with cytologic examination (A:II) Consider retinal biopsy MRI for CNS lymphoma (A:II) Clinical diagnosis B-scan ultrasound if visualization is poor Rhegmatogenous retinal PPV with long-term silicone oil Routine post-operative follow-up detachment (A:II) tamponade and scleral buckling As directed by other disorders (A:II) Holes or microholes in areas of areas of atrophic retina or chronic retinitis (A:II) Note extent of detachment, number, size, and location of retinal holes, and involvement of the macula (A:II) HIV = human immunodeficiency virus, VA = visual acuity, SLE = slit lamp examination, DOE = dilated ophthalmoscopic examination, CWS = cotton wool spots, IRH = intraretinal hemorrhages, MA = microaneurysms, CME = cystoid macular edema, HAART = highly active antiretroviral therapy, CMV = cytomegalovirus, AIDS = acquired immunodeficiency syndrome, KP = keratic precipitates, IRU = Immune recovery uveitis, IV = intravenous, IO = intraocular, PO = per os (by mouth), IOP = intraocular pressure, AC = anterior chamber, PCR = polymerase chain reaction, IOP = intraocular pressure, PPD = purified protein derivative, CXR = chest X-ray, IGRA = Interferon-gamma release assay, FA = fluorescein angiography, ICG = indocyanine green angiography, OCT = optical coherence tomography, RPR = rapid plasma reagin, VDRL = venereal disease research laboratory, FTA-ABS = fluorescent treponemal antibody absorption, MHA-TP = microhemagluttination-Treponema pallidum, CSFICO = cerebrospinal fluid,Clinical PORN =Guidelines progressive outer retinal International necrosis, ARN = acute retinal necrosis, HZO = herpes zoster ophthalmicus, TTP/HUS = thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, TRD = tractional retinal detachment, RNV55 = retinal Page neovascularization, ERM = epiretinal membrane, CME = cystoid macular edema, FA = fluoroscein angiography, PPV = pars plana vitrectomy, VMTS = vitreomacular traction syndrome, PVR = proliferative vitreoretinopathy, OU = oculus uterque (both eyes), CXR = chest X-ray, ABG = arterial blood gas, CT = computed tomography, TMP-SMX = trimethoprim sulfamethoxazole, EOM = extraocular motility, CNS = central nervous system, MRI = magnetic resonance imaging, N/A = not applicable Retinal detachment N/A Posterior Vitreous Detachment, Retinal Breaks and Lattice Degeneration (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Symptoms of PVD (A:I) Family history (A:II) Prior eye trauma (A:III) Myopia (A:II) History of ocular surgery including refractive lens exchange and cataract surgery (A:II) Initial Physical Exam (Key elements) Examination of the vitreous for hemorrhage detachment and pigmented cells (A:III) Examination of the peripheral fundus with scleral depression (A:III) The preferred method of evaluating peripheral vitreoretinal pathology is with indirect ophthalmoscopy combined with scleral depression (A:III) Ancillary Tests Perform B-scan ultrasonography if peripheral retina cannot be evaluated. (A:II) If no abnormalities are found, frequent follow-up examinations are recommended. (A:III) Surgical and Postoperative Care if Patient Receives Treatment: Inform patient about the relative risks, benefits and alternatives to surgery (A:III) Formulate a postoperative care plan and inform patient of these arrangements (A:III) Advise patient to contact ophthalmologist promptly if they have a substantial change in symptoms such as new floaters or visual field loss (A:II) ICO International Clinical Guidelines Page 56 Care Management Management Options Type of Lesion Acute symptomatic horseshoe tears Acute symptomatic operculated tears Traumatic retinal breaks Asymptomatic horseshoe tears Asymptomatic operculated tears Asymptomatic atrophic round holes Asymptomatic lattice degeneration without holes Asymptomatic lattice degeneration with holes Asymptomatic dialyses Fellow eyes atrophic holes, lattice degeneration, or asymptomatic horseshoe tears Treatment Treat promptly (A:II) Treatment may not be necessary (A:III) Usually treated (A:III) Usually can be followed without treatment (A:III) Treatment is rarely recommended (A:III) Treatment is rarely recommended (A:III) Not treated unless PVD causes a horseshoe tear (A:III) Usually does not require treatment (A:III) No consensus on treatment and insufficient evidence to guide management No consensus on treatment and insufficient evidence to guide management PVD = Posterior vitreous detachment Follow-up History Visual symptoms (A:I) Interval history of eye trauma or intraocular surgery (A:II) Follow-up Physical Exam Visual acuity (A:III) Evaluation of the status of the vitreous, with attention to the presence of pigment, hemorrhage, or syneresis (A:II) Examination of the peripheral fundus with scleral depression (A:II) B-scan ultrasonography if the media are opaque (A:II) Patients who present with vitreous hemorrhage sufficient to obscure retinal details and a negative B-scan should be followed periodically. For eyes in which a retinal tear is suspected, a repeat B-scan should be performed within approximately 4 weeks of the initial examination (A:III) ICO International Clinical Guidelines Page 57 Patient Education Educate patients at high risk of developing retinal detachment about the symptoms of PVD and retinal detachment and the value of periodic followup exams. (A:II) Instruct all patients at increased risk of retinal detachment to notify their ophthalmologist promptly if they have a substantial change in symptoms such as an increase in floaters, loss of visual field, or decrease in visual acuity. (A:III) *Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2008 (www.aao.org) ICO International Clinical Guidelines Page 58 Primary Open-Angle Glaucoma (Initial Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Ocular history (A:III) Systemic history (A:III) Family history (A:II) Assessment of impact of visual function on daily living and activities (A:III) Review of pertinent records (A:III) Initial Physical Exam (Key elements) Visual acuity (A:III) Pupils (B:II) Slit-lamp biomicroscopy of anterior segment (A:III) Measurement of IOP (A:I) o Time of day recorded because of diurnal variation (B:III) Central corneal thickness (A:II) Gonioscopy (A:III) Evaluation of optic nerve head and retinal nerve fiber layer with magnified stereoscopic visualization (A:III) Documentation of the optic disc morphology, best performed by color stereophotography or computer-based image analysis (A:II) Evaluation of the fundus (through a dilated pupil whenever feasible) (A:III) Visual field evaluation, preferably by automated static threshold perimetry (A:III) Management Plan for Patients in Whom Therapy is Indicated Set an initial target pressure of at least 20% lower than pretreatment IOP, assuming that the measured pretreatment pressure range contributed to optic nerve damage. (A:I) The more advanced the damage, the lower the initial target pressure should be. (A:III) In many instances, topical medications constitute effective initial therapy. (A:III) Laser trabeculoplasty is an appropriate initial therapeutic alternative. (A:I) Filtering surgery may sometimes be an appropriate initial therapeutic alternative. (A:I) Choose a regimen of maximal effectiveness and tolerance to achieve desired therapeutic response. (A:III) Surgery and Postoperative Care for Laser Trabeculoplasty Patients Ensure the patient receives adequate postoperative care. (A:III) Plan prior to and after surgery includes: ICO International Clinical Guidelines Page 59 o Informed consent. (A:III) o At least one preoperative evaluation and IOP measurement by the surgeon. (A:III) o At least one IOP check within 30 to 120 minutes following surgery. (A:I) o Examine within 6 weeks of surgery or sooner if concerned about IOP-related optic nerve damage. (A:III) Surgery and Postoperative Care for Filtering Surgery Patients Ensure the patient receives adequate postoperative care. (A:III) Plan prior to and after surgery includes: o Informed consent. (A:III) o At least one preoperative evaluation by the surgeon. (A:III) o Follow-up on first day (12 to 36 hours after surgery) and at least once from the second to tenth postoperative day. (A:II) o In absence of complications, additional routine postoperative visits during a 6-week period. (A:III) o Use topical corticosteroids in the postoperative period, unless contraindicated. (A:II) o Add more frequent visits, if needed, for patients with postoperative complications. (A:III) o Additional treatments as necessary to maximize chances for longterm success. (A:III) Patient Education for Patients with Medical Therapy Discuss diagnosis, severity of the disease, prognosis and management plan, and likelihood that therapy will be lifelong. (A:III) Educate about eyelid closure or nasolacrimal occlusion when applying topical medications to reduce systemic absorption. (B:II) Encourage patients to alert their ophthalmologist to physical or emotional changes that occur when taking glaucoma medications. (A:III) Educate about the disease process, rationale and goals of intervention, status of their condition, and relative benefits and risks of alternative interventions so that patients can participate meaningfully in developing an appropriate plan of action. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2006 (www.aao.org) ICO International Clinical Guidelines Page 60 Primary Open-Angle Glaucoma (Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Exam History Interval ocular history (A:III) Interval systemic medical history (B:III) Side effects of ocular medication (A:III) Frequency and time of last IOP-lowering medications, and review of use of medications (B:III) Physical Exam Visual acuity (A:III) Slit-lamp biomicroscopy (A:III) Measurement of IOP and time of day of measurement (A:III) Evaluation of optic nerve and visual fields (see table below) (A:III) Pachymetry should be repeated after any event that may alter central corneal thickness (A:II) Management Plan for Patients on Medical Therapy: Reconsider current IOP and its relationship to the target IOP at each visit. (A:III) At each exam, record dosage and frequency of use, discuss adherence to the therapeutic regimen and patient's response to recommendations for therapeutic alternatives or diagnostic procedures. (A:III) Perform gonioscopy if there is a suspicion of angle closure, anteriorchamber shallowing or anterior-chamber angle abnormalities or if there is an unexplained change in IOP. (A:III) Perform gonioscopy periodically (e.g., 1-5 years). (A:III) Assess treatment regimen if target IOP is not achieved and maintained in light of potential risks and benefits of additional or alternative treatment. (A:III) If a drug fails to reduce IOP, replace with an alternate agent until effective medical treatment is established. (A:III) Adjust target pressure downward if disc or visual field change is progressive. (A:III) Within each of the recommended intervals, factors that determine frequency of evaluation include the severity of damage, the stage of disease, the rate of progression, the extent to which the IOP exceeds the target pressure and the number and significance of other risk factors for damage to the optic nerve. (A:III) Deleting or adding medication justifies a follow-up visit at an interval ICO International Clinical Guidelines Page 61 appropriate for washout or maximal effect of medication withdrawn or added. (A:III) Follow-Up: Recommended Guidelines for Follow‐up: Target IOP Achieved Progression of Damage Duration of Control (months) Followup Interval [B:III] Optic Nerve Head Evaluation [A:III] Visual Field Evaluation [A:III] Yes No <6 Within 6 months 3-12 months 3-12 months Yes No >6 Within 12 3-12 months months 3-12 months Yes Yes (n/a) Within 4 months 1-12 months 12 months No Yes or No (n/a) Within 4 months 1-12 months 12 months Patient Education for Patients with Medical Therapy: Encourage patients to alert their ophthalmologist to physical or emotional changes that occur when taking glaucoma medications. (A:III) Refer for or encourage patients with significant visual impairment or blindness to use appropriate vision rehabilitation and social services. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2006 (www.aao.org) ICO International Clinical Guidelines Page 62 Primary Open-Angle Glaucoma (Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Exam History Interval ocular history (A:III) Interval systemic medical history (B:III) Side effects of ocular medication (A:III) Frequency and time of last IOP-lowering medications, and review of use of medications (B:III) Physical Exam Visual acuity (A:III) Slit-lamp biomicroscopy (A:III) Measurement of IOP and time of day of measurement (A:III) Evaluation of optic nerve and visual fields (see table below) (A:III) Pachymetry should be repeated after any event that may alter central corneal thickness (A:II) Management Plan for Patients on Medical Therapy: Reconsider current IOP and its relationship to the target IOP at each visit. (A:III) At each exam, record dosage and frequency of use, discuss adherence to the therapeutic regimen and patient's response to recommendations for therapeutic alternatives or diagnostic procedures. (A:III) Perform gonioscopy if there is a suspicion of angle closure, anteriorchamber shallowing or anterior-chamber angle abnormalities or if there is an unexplained change in IOP. (A:III) Perform gonioscopy periodically (e.g., 1-5 years). (A:III) Assess treatment regimen if target IOP is not achieved and maintained in light of potential risks and benefits of additional or alternative treatment. (A:III) If a drug fails to reduce IOP, replace with an alternate agent until effective medical treatment is established. (A:III) Adjust target pressure downward if disc or visual field change is progressive. (A:III) Within each of the recommended intervals, factors that determine frequency of evaluation include the severity of damage, the stage of disease, the rate of progression, the extent to which the IOP exceeds the target pressure and the number and significance of other risk factors for damage to the optic nerve. (A:III) Deleting or adding medication justifies a follow-up visit at an interval ICO International Clinical Guidelines Page 63 appropriate for washout or maximal effect of medication withdrawn or added. (A:III) Follow-Up: Recommended Guidelines for Follow‐up: Target IOP Achieved Progression of Damage Duration of Control (months) Followup Interval [B:III] Optic Nerve Head Evaluation [A:III] Visual Field Evaluation [A:III] Yes No <6 Within 6 months 3-12 months 3-12 months Yes No >6 Within 12 3-12 months months 3-12 months Yes Yes (n/a) Within 4 months 1-12 months 12 months No Yes or No (n/a) Within 4 months 1-12 months 12 months Patient Education for Patients with Medical Therapy: Encourage patients to alert their ophthalmologist to physical or emotional changes that occur when taking glaucoma medications. (A:III) Refer for or encourage patients with significant visual impairment or blindness to use appropriate vision rehabilitation and social services. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2006 (www.aao.org) ICO International Clinical Guidelines Page 64 Primary Open-Angle Glaucoma Suspect (Initial and Follow-up Evaluation) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Ocular history (A:III) Systemic history (A:III) Family history (A:III) Review of pertinent records (A:III) Assessment of impact of visual function on daily living and activities (A:III) Initial Physical Exam (Key elements) Visual acuity (A:III) Pupils (B:II) Slit-lamp biomicroscopy of anterior segment (A:III) Measurement of IOP (A:I) Central corneal thickness (A:II) Gonioscopy (A:III) Evaluation of optic nerve head and retinal nerve fiber layer, with magnified stereoscopic visualization (A:III) Documentation of the optic disc morphology, best performed by color stereophotography or computer-based image analysis(A:II) Evaluation of the fundus (through a dilated pupil whenever feasible) (A:III) Visual field evaluation, preferably by automated static threshold perimetry (A:III) Management Plan for Patients in Whom Therapy is Indicated: An appropriate initial goal is to set target pressure 20% less than mean of several IOP measurements and <24 mm Hg. (A:I) Choose regimen of maximal effectiveness and tolerance to achieve desired therapeutic response. (A:III) Follow-Up Exam History Interval ocular history (A:III) Interval systemic medical history and any change of systemic medications (B:III) Side effects of ocular medications if patient is being treated (A:III) Frequency and time of last glaucoma medications, and review of use, if patient is being treated (B:III) ICO International Clinical Guidelines Page 65 Follow-Up Physical Exam Visual acuity (A:III) Slit-lamp biomicroscopy (A:III) IOP and time of day measurement (A:III) Gonioscopy is indicated when there is a suspicion of an angle-closure component, anterior chamber shallowing or unexplained change in IOP (A:III) Recommended Guidelines for Follow-up [A:III] Treatment Target IOP Achieved High Risk of Damage Follow-up Interval 6-24 months 3-12 months No N/A No No N/A Yes Yes Yes Yes Yes No Yes Frequency of Optic Nerve Head and Visual Field Evaluation 6-24 months 6-18 months 3-12 6-18 months months < 4 months 3-12 months Patient Education for Patients with Medical Therapy: Discuss number and severity of risk factors, prognosis, management plan and likelihood that therapy, once started, will be long term. (A:III) Educate about disease process, rationale and goals of intervention, status of their condition, and relative benefits and risks of alternative interventions. (A:III) Educate about eyelid closure and nasolacrimal occlusion when applying topical medications to reduce systemic absorption. (B:II) Encourage patients to alert their ophthalmologist to physical or emotional changes that occur when taking glaucoma medications. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2006 (www.aao.org) ICO International Clinical Guidelines Page 66 Primary Angle Closure (Initial Evaluation and Therapy) (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History (Key elements) Systemic history (e.g., use of topical or systemic medications) (A:III) Ocular history (symptoms suggestive of intermittent angle-closure attacks) (A:III) Family history of acute angle-closure glaucoma (B:II) Initial Physical Exam (Key elements) Visual acuity (A:III) Refractive status (A:III) Pupils (A:III) Slit-lamp biomicroscopy (A:III) o Anterior chamber inflammation suggestive of a recent or current attack o Corneal edema o Central and peripheral anterior-chamber depth o Iris atrophy, particularly sector types, posterior synechiae or middilated pupil o Signs of previous angle closure attacks Measurement of IOP (A:III) Gonioscopy of both eyes (A:III) Evaluation of fundus and optic nerve head using direct ophthalmoscope or biomicroscope (A:III) Diagnosis Establish a diagnosis of primary angle closure, excluding secondary forms (A:III) Management Plan for Patients in Whom Iridotomy is Indicated Treat acute PAC by laser iridotomy or incisional iridectomy if a laser iridotomy cannot be successfully performed. (A:III) In acute angle-closure attacks, usually use medical therapy first to lower the IOP, to reduce pain and clear corneal edema in preparation for iridotomy. (A:III) Perform prophylactic iridotomy in fellow eye if chamber angle is anatomically narrow. (A:II) Perform surgery on one eye at a time for patients requiring bilateral incisional iridectomy (several days apart) whenever feasible to avoid ICO International Clinical Guidelines Page 67 simultaneous bilateral complications. (A:III) Surgery and Postoperative Care for Iridotomy Patients Ensure the patient receives adequate postoperative care. (A:III) Plan prior to and after surgery includes: o Informed consent (A:III) o At least one preoperative evaluation by the surgeon (A:III) o At least one IOP check within 30 to 120 minutes following laser surgery (A:II) o Use of topical anti-inflammatory agents in the postoperative period, unless contraindicated (A:III) Follow-up evaluations include: o Evaluation of patency of iridotomy (A:III) o Measurement of IOP (A:III) o Gonioscopy, if not performed immediately after iridotomy (A:III) o Pupil dilation to reduce risk of posterior synechiae formation (A:III) o Fundus examination as clinically indicated (A:III) Use medications perioperatively to avert sudden IOP elevation, particularly in patients with severe disease. (A:III) Refer for and encourage patients with significant visual impairment or blindness to use vision rehabilitation and social services. (A:III) Evaluation and Follow-up of Patients with Iridotomy: After iridotomy, follow patients with glaucomatous optic neuropathy as specified in the Primary Open-Angle Glaucoma PPP. (A:III) Follow all other patients as specified in the Primary Open-Angle Glaucoma Suspect PPP. (A:III) Education for Patients if Iridotomy is not Performed: Inform patients at risk for acute angle closure about symptoms of acute angle-closure attacks and instruct them to notify immediately if symptoms occur. (A:III) Warn patients of danger of taking medicines that could cause pupil dilation and induce an angle-closure attack. (A:III) * Adapted from the American Academy of Ophthalmology Summary Benchmarks, November 2006 (www.aao.org) ICO International Clinical Guidelines Page 68 Trachoma (Ratings: A: Most important, B: Moderately important, C: Relevant but not critical Strength of Evidence: I: Strong, II: Substantial but lacks some of I, III: consensus of expert opinion in absence of evidence for I & II) Initial Exam History Living in a trachoma-endemic region (A:I) Duration of red eye (an acute follicular conjunctivitis may be due to other organisms) (C:III) Any previous similar episodes (active trachoma is often recurrent) (C:III) Household contacts with history of trachoma or chronic conjunctivitis (B:I) Purulent discharge (although active trachoma is often sub-clinical or asymptomatic) (C:III) Duration of trichiasis (C:III) History of previous lid surgery (A:III) Initial Physical Exam Using 2.5x magnification loupes and adequate lighting (daylight or torchlight) or using a slit lamp, assess signs of trachoma using the WHO simplified grading scale: www.who.int/ncd/vision2020_actionplan/documents/Simplifiedgradingoftrac homa.PDF (A:III) Briefly, note any trichiasis or corneal opacity. Evert the upper palpebral conjunctivae and note follicles over the tarsal plate (5 follicles greater than 0.5 mm in the central tarsus constitutes the WHO grade of TF), intense inflammatory thickening obscuring 50% of the normal, underlying conjunctival vasculature (TI), and easily visible scarring (TS). Diagnostic (Laboratory) Tests PCR testing for chlamydial DNA—this is the gold standard for identifying infection but not for diagnosing trachoma (B:I) Direct Chlamydial Immunofluorescence test +/- chlamydial culture of conjunctival epithelial cells (C:II) Chlamydial culture (difficult to perform) (C:II) Giemsa stain of conjunctival scrape to look for: o Basophilic intracytoplasmic inclusion bodies in epithelial cells (C:III) o Polymorphonuclear leucocytes (C:III) Management Management of trachoma should be community based. The WHO recommends the integrated SAFE Strategy, surgery for trichiasis, community wide antibiotic treatment, facial cleanliness education and environmental improvements (B:III) Surgical: Trichiasis surgery (bilamellar tarsal rotation or the related Trabut ICO International Clinical Guidelines Page 69 procedure) should be considered if any of the following are present: o one or more in-turned eyelashes are abrading the cornea when the patient is looking straight ahead (A:II) o pre-existing evidence of corneal damage from trichiasis (B:II) o severe discomfort from trichiasis (C:III) o Contra-indications to trichiasis surgery include defective lid closure, children with trichiasis (may need general anesthetic), and poor general health. (C:III) o Epilation is considered an alternative for refusal to have surgery (B:III) Community-wide antibiotic treatment is recommended if there is >10% active trachoma in children aged 1-9 years of age in the community. Targeted treatment of clinically active cases is recommended for a lower prevalence. Household contacts, and in particular, siblings, may also be treated, even if they have no active signs of infection (B:II) The following antibiotic treatment is recommended by the WHO: o Single dose azithromycin: in children aged <16 years dosage is 20mg/kg (maximum dose 1g); in adults dosage is 1g (A:I) o Or, use topical 1% tetracycline eye ointment in pregnant women, children aged below 6 months and those allergic to macrolides, used twice daily in both eyes for 6 weeks (A:I) o It is acceptable to treat follicular conjunctivitis in a trachoma-endemic area with antibiotics even without laboratory documentation of active chlamydial infection (A:I) Facial Cleanliness: promote regular face-washing with clean water. Clean faces have been associated with clinically active trachoma, but it should be noted that face-washing interventions have not been shown to reduce ocular chlamydial infection (B:II) Environmental Improvements: (improving water supply, latrine provision and fly control). The face fly Musca sorbens has been implicated as a possible vector for trachoma and breeds preferentially on human feces. These flies cannot breed in latrines, so latrine construction is thought to reduce fly populations and trachoma transmission (B:II) Follow-up Evaluation WHO recommends annual, community based treatment with reassessment at three years. (B:II) Note that follicles can take months to clear even after infection has been eliminated, and that re-treatment may not be warranted if follicles are slowly improving depending on the time that has elapsed since the last treatment was given. (B:II) For treatment of individual more frequent examinations can be undertaken. Follow-up 1 month after treatment, with retreatment as necessary is reasonable. Re-infection frequently occurs in endemic areas, so patient education regarding methods that may reduce transmission is useful. (C:III) After trichiasis surgery, patients should be seen within 2 weeks for suture ICO International Clinical Guidelines Page 70 removal, and annually to ensure that trichiasis has not returned. (A:III) ICO International Clinical Guidelines Page 71