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COURSE 6 Diagnosing and Managing Ocular Emergencies and Urgencies COPE Course 43805-SD COURSE 7 Pre- and Post-Operative OCT Evaluation of Retinal Disease COPE Course 43986-PO COURSE 8 Optometric Management of Children with Special Needs COPE Course 43881-FV COURSE 9 Pill Problems: Ocular Complications from Systemic Medications COPE Course 35069-OP 4/6/15 Diagnosing and Managing Ocular Emergencies and Urgencies Blair Lonsberry, MS, OD, MEd., FAAO Diplomate, American Board of Optometry Clinic Director and Professor of Optometry Pacific University College of Optometry [email protected] 1 What Classifies an Emergency? • Any condition in which the patient has the potential for: – vision loss, – currently experiencing vision loss, – permanent structural damage, – pain or discomfort, – or is an “emergency” for the patient. • It is important to be able to triage a walk-in patient and, more importantly, a call-in patient. What ques*ons to ask? Onset suddenly noticed or sudden onset? Visual Loss any loss of vision? loss vs. blurry vision one eye or both part of visual field or all transient vs. permanent Pain is there pain? constant? scale (1-10) Redness is there any redness? location? Associated Factors contact lens wear? trauma? discharge? photophobia? medical history (eg. DM) 1 4/6/15 Common Types of Ocular Emergencies • Vision Loss: – Gradual vs. sudden onset – Vision loss with or without pain • Trauma • Red eyes Visual Loss • Visual loss varies greatly in meaning from patient to patient – ranging from blur to complete blindness and may affect one or both eyes • Components include: – acuity, – visual field, – color and brightness may be affected jointly or separately • Detailed history and extent of vision loss crucial Profound Loss of Vision • Referring to a complete or greatly diminished vision affecting the whole field • Common causes of severe vision loss: Vascular central retinal vein occlusion, central retinal artery occlusion, vitreous heme Inflammatory optic neuritis Infiltrative Mechanical optic neuropathy retinal detachment 2 4/6/15 Monocular vs. Binocular • Ocular or optic nerve pathology causes monocular vision loss • lesion at or posterior to chiasm causes binocular vision loss – VF defects become more congruous the further back in the visual pathway – Homonymous VF defects noted posterior to chiasm • Difference between mono vs. bino usually straightforward, keeping the following in mind: – Patients occasionally mistake homonymous hemianopsia (similar loss of visual field in both eyes) for a monocular loss Visual Defects Monocular • Differentiate between eyes that have lost all useful vision and those that have blurred vision • Blurring of vision is not localized and may be caused by pathology anywhere from cornea to optic nerve • Need to get anatomical diagnosis first before considering the cause 3 4/6/15 General Appearance • Level of consciousness – When introducing yourself be aware of the patient’s gross level of consciousness? • Is the patient awake, alert and responsive? • Personal Hygiene and Dress – Is it appropriate for the environment, temperature, age and social status of the patient? – Is the patient malodorous or disheveled? General Appearance • Posture and Motor control – What posture does patient assume while sitting in the exam chair – Are there any signs of involuntary motor activity such as tremors • E.g. damage to the cerebellum may produce a tremor that usually worsens with movement of the affected limb Case Example • 48 yr old white female presented for diabetic eye exam on referral from her PCP – She was scheduled 2 weeks previously but had fallen and was unable to make that appointment – She reports that her vision in her right eye seems to be getting worse over the past several weeks. – Was diagnosed with diabetes 1.5 years ago • BS control has been erratic with range between 120-240 • Last A1C: 9.1 4 4/6/15 Blood Sugar • Throughout a 24 hour period blood sugar typically maintained between 70-145 – Diabetes is diagnosed with a fasting BS of > 126 or an A1c value of > 6.5 • Hypoglycemia is typically defined as plasma glucose 70 or less – patients typically become symptomatic of hypoglycemia at 50 or less Entrance Skills/Health Assessment VA: OD: finger count OS: 20/40 CVF: OD: unable to assess OS: temporal hemianopsia Pupils: sluggish reactivity with a 2+ RAPD OD SLE: corneal arcus noted, no other significant findings IOP: 16, 16 mmHG OD, OS DFE: see photos Note: not patient photos http://content.lib.utah.edu/cdm4/ item_viewer.php?CISOROOT=/ EHSL-WFH&CISOPTR=159 Physical Presentation • Upon entering the room I noted that her right hand was twitching – I asked her how long that had been going on and she said about 2-3 weeks – I asked her if she experienced headaches, to which she said she had bad headaches that even woke her up at night 5 4/6/15 Referral • Contacted her PCP who reported that she had examined the patient 3 weeks prior and had not noted any of these findings • Referred the patient for an immediate MRI – wasn’t able to be scheduled until the next day Imaging/Surgery Referral • MRI revealed large mass in her brain – Patient was diagnosed with a Craniopharyngioma – She was referred for immediate surgery – Neurosurgeon reported that she removed a tangerine sized Craniopharyngioma – was the largest tumor she has ever removed Note: not patient MRI http://neurosurgery.ucla.edu/images/ Pituitary%20Program/ Craniopharyngioma/ Cranio_Sag_Preop_fullylabeled.jpg Craniopharyngioma • Craniopharyngioma: – slow-growing, – epithelial-squamous origin, – calcified cystic tumor – arises from remnants of the craniopharyngeal duct • Craniopharyngiomas have a benign histology but malignant behavior – they have a tendency to invade surrounding structures and recur after what was thought to be total resection 6 4/6/15 Craniopharyngioma • Visual field examination may reveal various patterns of visual loss – most frequently bitemporal hemianopsia • suggestive of compression of the optic chiasma and/ or tracts Our Patient • Patient had a complete resection of the tumor in addition to radiation therapy • She developed several significant perioperative complications: – Leakage of CSF which resulted in her having to have a shunt • She subsequently developed an infection post surgically – She is NLP in her right eye, but did regain 20/40 vision in her left eye • Retains a temporal hemianopsia OS – Diabetes control became erratic and was put on several hormone replacement medications Neurological Screening: Cerebrum • Frontal lobe – Emotions, drive, affect, self-awareness, and responses related to emotional states – Motor cortex associated with voluntary skeletal movement and speech formation (Broca) 7 4/6/15 Right vs Left Brain Injury • So what happens if one side of the brain is injured? – People who have an injury to the right side of the brain "don't put things together" and fail to process important information. • As a result, they often develop a "denial syndrome" and say "there's nothing wrong with me.“ Right vs Left Brain Injury • The left side of the brain deals more with language and helps to analyze information given to the brain. – If you injure the left side of the brain, you're aware that things aren't working (the right hemisphere is doing its job) but are unable to solve complex problems or do a complex activity. – People with left hemisphere injuries tend to be more depressed, have more organizational problems, and have problems using language. Case • 20 year old male presents with a red painful eye – complains about red/painful right eye – Started that morning when he woke up – reports a watery discharge, no itching, and is not a contact lens wearer • SLE: – See attached image with NaFl stain 8 4/6/15 Question What would you begin treatment with? 1. oral acyclovir 400 mg 5 times a day 2. topical trifluridine (Viroptic) 1 drop every 2 hours 3. Topical ganciclovir (Zirgan) 5 times per day 4. Topical trifluridine (Viroptic) every two hours plus FML BID 5. both oral and a topical Herpes Simplex Keratitis: Clinical Features • Characterized by primary outbreak and subsequent reactivation • Primary outbreak is typically mild or subclinical • After primary infection, the virus becomes latent in the trigeminal ganglion or cornea • Stress, UV radiation, and hormonal changes can reactivate the virus • Lesions are common in the immunocompromised (i.e. recent organ transplant or HIV patients) Dendri*c Ulcers 2 7 9 4/6/15 Herpes Simplex Keratitis • Topical: – Viroptic (trifluridine) q 2h until epi healed then taper down for 10-14 days. • Viroptic is toxic to the cornea. – Zirgan (ganciclovir) available, use 5 times a day until epi healed then 3 times for a week (US only) • Oral acyclovir (2 g/day) has been reported to be as effective as topical antivirals without the toxicity – Valtrex (valcyclovir)) 500 mg TID for 7-10 days – Famvir (famciclovir) 250 mg TID for 7-10 days • If stomal keratitis present, after epi defect has healed, add Pred Forte QID until inflammation reduced and then slowly taper 10 4/6/15 Prophylaxis?? • Prophylaxis of 400 mg acyclovir BID vs placebo for 1 year resulted in a lower recurrence in the treatment arm (19% vs 32%) – Valtrex 500 mg qd was found to be equivalent to acyclovir BID • Pitfalls to Prophylaxis: – Reduction of recurrence does not persist once drug stopped – Resistance???? • van Velzen, et. al., (2013) demonstrated that long-‐ term ACV prophylaxis predisposes to ACV-‐refractory disease due to the emergence of corneal ACVR HSV-‐1. Dendritic ulceration before treatment with Zirgan Cornea after treatment with Zirgan HERPES ZOSTER OPHTHALMICUS 11 4/6/15 Herpes Zoster Herpes Zoster Ophthalmicus 35 Herpes Zoster • Presents with: – pain and tingling in region of skin supplied by V few days before lesions, – malaise and fever, – papulomacular then pustular rash, – mucopurulent conjunctivitis, – uveitis, glaucoma, episcleritis, keratitis, and retinitis can all occur. – neurological complications include cranial nerve palsies and optic neuritis. 12 4/6/15 Herpes Zoster • Associated factors include increasing age, immune deficiency and stress. • Only people who had natural infec*on with wild-‐type VZV or had varicella vaccina*on can develop herpes zoster. • Children who get the varicella vaccine appear to have a lower risk of herpes zoster compared with people who were infected with wild-‐type VZV. • A person's risk for herpes zoster increases sharply aRer 50 years of age. • Almost 1 out of 3 people in the United States will develop herpes zoster during their life*me. • A person’s risk of developing post-‐herpe*c neuralgia also increases sharply with age. Herpes Zoster • Management includes: – oral an*virals: • 800mg acyclovir 5x/day • valacyclovir (Valtrex) 1g TID, • famciclovir (Famvir) 500 mg TID – effec*veness of therapy is best started within 72 hours – oral steroids, and – management of pain (tricyclic an*depressants, gabapen*n). – If ocular complica*ons, consider topical steroids (Pred Forte QID). Vaccine (Zostavax®) • The Advisory Commidee on Immuniza*on Prac*ces (ACIP) recommends zoster vaccine (Zostavax®) for people aged 60 years and older. • The vaccine reduced the overall incidence of shingles by 51% and the incidence of PHN by 67% • Even people who have had herpes zoster should receive the vaccine to help prevent future occurrences of the disease. • In adults vaccinated at age 60 years or older, vaccine efficacy wanes within the first 5 years aRer vaccina*on, and protec*on beyond 5 years is uncertain 13 4/6/15 Corneal Ulcers • • Infec*ve bacterial and fungal corneal lesions cause severe pain and loss of vision Signs and Symptoms: – Pain, photophobia, tearing – Mucopurulent discharge with generalized conjunc*val injec*on – Decreased VA (esp if on visual axis) – Possible AC reac*on and hypopyon – Dense infiltrate – Satellite lesions around main lesion may indicate fungal infec*on 14 4/6/15 Sterile vs Infec*ous Infiltrates Peripheral (Sterile) Corneal Ulcer Infectious Corneal Ulcer 15 4/6/15 Corneal Ulcers • The Steroids for Corneal Ulcers Trial (SCUT) • Conclusions: – no overall difference in 3-month BSCVA and no safety concerns with adjunctive corticosteroid therapy for bacterial corneal ulcers – researchers did find significant vision improvement for one specific subgroup of the study by using steroid therapy on pa*ents with severe ulcers • Application to Clinical Practice: – Adjunctive topical corticosteroid use does not improve 3month vision in patients with bacterial corneal ulcers unless in the severe category Corneal Ulcers • • Infective bacterial and fungal corneal lesions cause severe pain and loss of vision S and S: – Pain, photophobia, tearing – Mucopurulent discharge with generalized conjunctival injection – Decreased VA (esp if on visual axis) – Possible AC reaction and hypopyon – Dense infiltrate – Satellite lesions around main lesion may indicate fungal infection Associated Factors • Contact lens wear, especially soft and extended wear lens • Recent history of corneal trauma • Topical steroid use • History of exposure to vegetative matter (fungal etiology) 16 4/6/15 Protein Synthesis Inhibitors • These antibiotics work by targeting the bacterial ribosome. – they are structurally different from mammalian ribosomes, – in higher concentrations many of these antibiotics can cause toxic effects. • This group includes: – (a) tetracyclines, (b) aminoglycosides, (c) macrolides, – (d) chloramphenicol, (e) clindamycin, (f) quinupristin/dalfopristin and (g) linezolid Tetracyclines • This group includes: – Tetracycline (250mg - 500 mg cap BID-QID) needs to be taken 1 hour before or 2 hours after a meal. – Minocycline (100 mg cap BID) – Doxycycline (20mg - 100 mg cap or tab BID) Anti-inflammatory effects • Degrade extracellular proteins • Tetracyclines inhibit MMPs • Anti-inflammatory 17 4/6/15 Pseudomonas case report “Doxycycline as an adjunctive therapy…may help to stabilize corneal breakdown and prevent subsequent perforation.” AM. McElvanney 750 Preseptal Cellulitis • infection and inflammation anterior to the orbital septum and limited to the superficial periorbital tissues and eyelids. – Signs and Symptoms include: • eyelid swelling, • redness, • ptosis, • pain and • low grade fever. Preseptal Cellulitis Treatment Treatment: • Mild: – Keflex or Ceclor 250-500mg QID for 5-7 days – Augmentin 500 mg TID – or 875 mg BID for 5-7 days • Moderate to severe: – IM Rocephin (ceftriaxone) 1-2 grams/day or – IV Fortaz (ceftazidime) 1-2 g q8h. 18 4/6/15 Case • 65 year old Caucasian patient presents with sudden onset loss/blurring of vision in the right eye • PMHx: HTN for 15 years, takes “water pill” • VA’s: 20/60 OD, 20/25 OS • Pupils: PERRL –APD • CVF: Inferior defect right eye, no defects noted in the left eye Vision Loss Without Pain: Diabetes/Diabe*c Re*nopathy Microvascular complications resulting in capillary closure & abnormal permeability S&S include; ◦ blurring of vision (maculopathy and refractive error shifts), ◦ sudden drop in vision (vitreous heme), ◦ dot and blot hemes, ◦ exudate, ◦ cotton wool spots, ◦ neovascularization (iris, retina and disc) VEGF and DME 19 4/6/15 Aug. 10, 2012: FDA approves Lucentis to treat diabetic macular edema • The drug’s safety and effectiveness to treat DME were established in two clinical studies involving 759 patients who were treated and followed for three years. – patients were randomly assigned to receive monthly injections of Lucentis at 0.3 milligrams (mg) or 0.5 mg, or no injections during the first 24 months of the studies – after 24 months, all patients received monthly Lucentis either at 0.3 mg or 0.5 mg • Results: – 34-45% of those treated with monthly Lucentis 0.3 mg gained at least three lines of vision compared with 12-18% of those who did not receive an injection. What’s New? • Sept. 16, 2014 -‐-‐ Regeneron Pharmaceu*cals, Inc. announced that the FDA has granted EYLEA® (aflibercept) Injec*on Breakthrough Therapy designa*on for the treatment of diabe*c re*nopathy in pa*ents with diabe*c macular edema (DME). • Sept 29, 2014: The FDA approved Ozurdex (dexamethasone intravitreal implant) for the general pa*ent popula*on being treated for DME • Sept 29, 2014: The FDA approved Iluvien (fluocinolone acetonide implant) for the treatment of DME in pa*ents previously treated with cor*costeroids who did not have a significant increase in IOP Vision Loss Without Pain: Vein Occlusion • Associated with: – hypertension, – coronary artery disease, – DM and – peripheral vascular disease. • Usually seen in elderly patients (60-70), slight male and hyperopic predilection. • Second most common vascular disease after diabetic retinopathy. 20 4/6/15 Branch Re*nal Vein Occlusion: Signs/Symptoms BRVO: sudden, painless, visual field defect. ◦ patients may have normal vision. ◦ quadrantic VF defect, ◦ dilated tortuous retinal veins with superficial hemes and CWS ◦ typically occurs at A/V crossing (sup/temp) BRVO BRVO more common than CRVO and has more favorable prognosis ◦ Overall 50-60% of BRVO patients will maintain VA of 20/40 or better Visual loss results from: ◦ Macular edema ◦ Foveal hemorrhage ◦ Vitreous heme ◦ Epiretinal membrane ◦ RD ◦ Macular ischemia ◦ Neovascularization complications Study Design (n=397) BRVO BRAnch retinal Vein Occlusion study safety/efficacy Macular Edema Secondary to BRVO 1:1:1 RandomizaBon Ranibizumab Ranibizumab 0.5 mg 0.3 mg (n=131) (n=134) Monthly InjecBons (last at 5M) Rescue Laser (if eligible beginning at Month 3) Sham (n=132) 12M PRN ranibizumab for all paBents Rescue Lase aser (if eligible beginning at Month 9) Ranibizumab Ranibizumab 0.5 mg 0.3 mg Month 6 Primary Endpoint Ranibizumab 0.5 mg 21 4/6/15 Mean Change from Baseline BCVA Mean Change from Baseline BCVA (ETDRS Letters) Sham/0.5 mg (n=132) BRVO 0.3 mg Ranibizumab (n=134) 0.5 mg Ranibizumab (n=131) +18.3* 20 +18.3 18 +16.4 16 14 +16.6* +11.6 12 10 +12.1 +10.2 8 6 +7.3 +3.1 4 2 0 07 2 4 Day 0–Month 5 Monthly Treatment 6 Month 8 10 12 Months 6–11 PRN Treatment The gain of additional 3 lines occurred at a rate of 61% of 0.5 AVT grp, 55% for 0.3 AVT & 29% placebo Central Re*nal Vein Occlusion: Signs/Symptoms CRVO: thrombus occurring at lamina is classical theory but new evidence indicates that the occlusion is typically in the optic nerve posterior to the lamina cribrosa ◦ decreased VA ranging from near normal to hand motion with majority 20/200 range ◦ dilated tortuous vessels, with numerous retinal hemes and CWS Central Re*nal Vein Occlusion • Visual morbidity and blindness are primarily from: – persistent macular edema, – macular ischemia and – neovascular glaucoma • CRVO’s can be ischemic or non. – Classical definition of ischemic is 10-disc area of nonperfusion found on angiography – RAPD and ERG maybe better predictor – VA’s typically worse in ischemic – Increased number of cotton wool spots with decreased VA maybe predictive 22 4/6/15 Central Re*nal Vein Occlusion Ischemic CRVO may lead to iris neovascularization and neovascular glaucoma ◦ Estimated apprx 20% of CRVO’s are ischemic with 45% of those developing neo Regular examinations (1-2 wks) to monitor for ischemia or neo development ◦ should include gonio as angle neo can precede iris rubeosis Study Design CRUISE (n=392) CRVO Central Retinal vein occlUsIon Study: Efficacy & safety Macular Edema Secondary to CRVO Sham (n=130) 1:1:1 RandomizaBon Ranibizumab 0.5 mg (n= 130) Ranibizumab 0.3 mg (n=132) Monthly InjecBons (last at 5M): 6M tx period 12M trial PRN LucenBs available for for all paBents: 6M tx period 0.5 mg Ranibizumab 0.3 mg Month 6 Primary Endpoint Ranibizumab 0.5 mg Mean Change from Baseline BCVA Mean Change from Baseline BCVA (ETDRS Le\ers) Sham/0.5 mg (n=130) CRVO 0.3 mg Ranibizumab (n=132) 18 16 0.5 mg Ranibizumab (n=130) +14.9* +13.9 +13.9 14 12 +12.7* 10 8 +7.3 6 4 +0.8 2 0 -‐2 0 7 2 4 Day 0–Month 5 Monthly Treatment 6 8 10 12 Months 6–11 Month PRN Treatment Pts with >/= 3 line improvement was noted in 48% of .5 AVT, 26 of .3 AVT & 17% of sham 23 4/6/15 Hot Off The Presses! • October 8, 2014: The FDA has expanded the indica*on of aflibercept (Eylea, Regeneron) injec*on to include all forms of macular edema aRer re*nal vein occlusion (RVO), including branch RVO (BRVO) and CRVO (previously approved). Vision Loss Without Pain: Artery Occlusion • Primarily embolic in nature from cholesterol, calcifications, plaques. • Usually occurs in elderly associated with: – hypertension (67%), – carotid occlusive disease (25%), – DM (33%) and – cardiac valvular disease. • Sudden loss of unilateral, painless vision – defect dependent upon location of occlusion Vision Loss Without Pain: Artery Occlusion • BRAO typically located in temporal retinal bifurcations. 24 4/6/15 CRAO • CRAO has profound vision loss with history of amaurosis fugax. – Vision is usually CF (count fingers) to LP (light perception) with positive APD. – Diffuse retinal whitening with arteriole constriction, cherry red macula. Ophthalmic Emergency Treatment is controversial due to poor prognosis and questionable benefit. Treat immediately before workup, if patient presents within 24 hours of visual loss: ◦ Digital ocular massage, ◦ systemic acetozolamide (500 mg IV or po), ◦ topical ocular hypertensive drops (Iopidine, B-blocker), ◦ anterior chamber paracentesis, ◦ consider admission to hospital for carbogen Tx (high carbon dioxide) Flashes and Floaters • Pa*ents oRen present complaining of “spots” or “cobwebs” in front of their eyes • Causes of floaters include: posterior vitreous detachment (PVD), re*nal tear, vitreous heme, uvei*s. • Since PVD and re*nal tears present the same way, a RT has to be eliminated • Ask the pa*ent whether spots move with eye and con*nue to move aRer the eye has stopped • Large spots could be blood clots 25 4/6/15 Posterior Vitreous Detachment (PVD) Vitreous Heme Retinal Tear 26 4/6/15 PDS Clinical Features • Posterior segment – Latce degenera*on occurs in 8-‐11% of the general popula*on • The incidence of atrophic holes in latce degenera*on ranges from 18-‐42% – Lattice retinal degeneration has been reported to be evident in 20–33% of cases of PDS and PDG • greater than would be expected for the associated myopia PDS Clinical Features • Posterior segment – retinal breaks occur more frequently than in normal eyes, affecting 12% of eyes with PDS and PDG – risk of re*nal detachment is only 0.1-‐0.7% in the “normal” phakic eye • retinal detachments have been reported to occur in 5.5–6.6% of PDS cases • higher than expected for the degree of myopia and is independent of miotic use Flashes and Floaters • Sudden onset typically means a PVD, retinal tear or heme • If the spots appear after flashing light, then retinal tear must be eliminated • Myopes tend to have floaters and will notice them for a long time • Key is to rule out potentially sight threatening condition for the floaters, ie retinal tear. • Patients with retinal condition such as lattice degeneration and myopes need to be educated about S&S of RD (flashes and floaters) – 8-11% population has lattice – Risk of RD with lattice is <1% – 30-50% of patients with a RD have lattice 27 4/6/15 Flashes and Floaters: Management • A pa*ent who presents with a sudden onset PVD without re*nal breaks or hemorrhage requires repeat peripheral examina*on in six weeks, as the risk of re*nal complica*ons is highest within the six weeks following vitreous detachment. • If no re*nal breaks are seen at that point, rou*ne yearly examina*on is all that is needed Ques*on 75 white female complains of sudden decreased vision left eye. From picture above what is most likely cause? 1. BRVO 2. Ischemic optic neuropathy 3. Papilledema 4. Low tension glaucoma Epidemiology • Nonarteritic: usually seen in younger patients – Fellow eye involved in 25-40% of cases – Associated with hypertension and diabetes 28 4/6/15 Epidemiology Arteritic: usually seen in >55 yrs old (mostly over 70) ◦ fellow eye involved in 75% of cases within 2 weeks without treatment Symptoms • Acute visual loss (arteritic>non) • dyschromatopsia • Arteritic may also have associated: – Headache, fever, malaise, – weight loss, scalp tenderness, jaw claudication, – amaurosis fugax, diplopia, and eye pain. Ocular Signs • Sudden, unilateral, painless decreased vision and color vision • Positive RAPD • Altitudinal visual field defect (usually inferior and large) • Swollen optic disc • Fellow nerve often crowded with small or absent cup (“disc at risk”) 29 4/6/15 Additional Testing • Lab tests: – STAT ESR (rule out arteritic form) – CBC (low hematocrit, high platelets) – Fasting blood sugar – C reactive protein, – VDRL/FTA-ABS – ANA • Check blood pressure Management • Arteritic: – Systemic steroids to prevent fellow eye involvement • methylprednisolone 1 g IV qd in divided doses for 3 days then, • prednisone 60-100 mg po qd with a slow taper – Check PPD, blood glc and chest radiographs before starting systemic steroids • Non-arteritic: – Consider daily aspirin Vision Loss Without Pain: TIA/TMB/Amaurosis Fugax Refers to temporary visual impairment of variable duration (seconds to hours) ◦ TIA: transient ischemic attack-can be cerebral or retinal ◦ TMB: transient monocular blindness secondary to a retinal TIA ◦ Amaurosis Fugax: same as TMB Abrupt onset, progression to involve all or part of visual field, sight usually returns Within affected area, visual acuity maybe dimmed or completely lost 30 4/6/15 TIA’s • Stroke is 3rd leading cause of mortality in developed countries and most common cause of neurological disability • 15-20% of patients with stroke have a preceding TIA, though guidelines for referral and evaluation are debated – Traditional guidelines suggested that assessment should be complete within 1 week of TIA TIA’s • Risk of stroke after TIA has traditionally been considered relatively low, but – new studies indicate that the risk is much higher than previously thought and the time window for prevention is short. • Effective secondary prevention depends on reliable identification of those at high risk and targeting treatment. TIA’s: High Risk Factors Five (5) risk factors are associated with a high risk (30%) of recurrent stroke at 3 months: ◦ Age over 60 ◦ Symptom duration greater than 10 minutes ◦ Motor weakness ◦ Speech impairment ◦ Diabetes Isolated sensory of visual symptoms were associated with low risk of stroke! 31 4/6/15 TIA: Early Treatment Several treatments are likely to be effective in preventing stroke in the acute phase after a TIA: ◦ Aspirin ◦ Anticoagulants ◦ Statins ◦ Endarterectomy (for >50% carotid stenosis) ◦ Further research needed for: Lowering blood pressure acutely after TIA Prophylactic use of neuroprotective drugs Amaurosis Fugax:TMB • Most common cause is: – thromboembolic disease (eg carotid artery disease throwing emboli) or – vasospasm • Described as “curtain falling over vision” • Risk of stroke or death is about 3-5%, – which is significantly lower than for a cerebral TIA (15-20%) • Px still require work-up to determine cause: – e.g. carotid doppler Alkali Chemical Burns • Alkali exposure results in: – – – – Loss of corneal and conjunctival epi, stromal keratocytes and endothelium Loss of clarity is secondary to stromal hydration Damage to the vascular endothelium of conjunctival and episcleral vessels Intraocular structures such as iris, lens and ciliary body are rapidly damaged if alkali penetrates cornea. 32 4/6/15 Acidic Chemical Burns • • Epithelium provides effective barrier to weak acids. Stronger acids cause protein precipitation in epithelium and stroma which creates a barrier to further penetration. Very strong acids penetrate as quickly as alkalis Chemical Burn Treatment • Immediate irrigation is of paramount importance • Most patients are disabled by severe blepharospasm and disorientation so require assistance away from harm and to initiate irrigation. • Make sure to remove any solid particulate matter prior to beginning irrigation • Minimum of 15 minutes constant irrigation (some recommend 30 minutes) Chemical Burn Treatment • Water is commonly recommended however it is hypotonic to corneal tissue and can result in increased water intake into the corneal and subsequent diffusion of corrosive materials deeper into cornea. • Recommend fluids of higher osmolarity such as sterile lactated Ringers and balanced saline solution. 33 4/6/15 Chemical Burn Treatment • Effectiveness of irrigation can be assessed using pH paper and continued as long as pH outside of the normal range. • For grade I and II burns will typically heal without permanent damage. – Topical steroid/antibiotic drops/ung recommended and daily follow up. – Cycloplegia for pain and further reduction of inflammation. Chemical Burn Treatment • Severe ocular burns are difficult to treat and may require months of healing • Basic treatment of these eyes is to reduce inflammatory response caused by necrotic tissue – Corticosteroid use – Prophylactic antibiotics (consider doxycycline as it inhibits proteinase activity) – May require surgical intervention with debridement of necrotic tissue and possibly reconstructive surgery. 34 1/16/2015 S/P RD Repair Pre- and Post-operative OCT evaluation of retinal disease. Jorge Calzada, MD S/P RD Repair Inferior Perimacular RD Inferior Juxtafoveal RD Mirror Image Artifact 1 1/16/2015 Sup RD and AMD Postop Sup RD + AMDm CNV Mac Off RD in 7 year old Fovea OFF RD Photoreceptor loss post RD And a month later…EMM 2 1/16/2015 Diabetic TRD Diabetic TRD TRD postop Subfoveal fluid post RD VA: 20/100 -> Redetachment, silicone oil glaucoma -> NLP Bullous Sup RD Mac OFF 3 1/16/2015 Dense EMM post RD Outer retinal cords post RD repair Recurrent RD under oil with foveal atrophy Persistent subfoveal fluid post RD 1 month later Fovea OFF RD 4 1/16/2015 Shallow fovea off RD Chronic Inf RD with foveal atrophy Mac ON RD Macular Hole MH postop 1 month EMM Rip 5 1/16/2015 Macular Hole MH postop 1 month- Foveal Atrophy Chronic RD with Subretinal PVR Chronic RD + Subretinal PVR Subretinal PVR 6 1/16/2015 7 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV OPTOMETRIC MANAGEMENT OF CHILDREN WITH SPECIAL NEEDS Karen A. Kehbein, OD, FCOVD Assistant Professor Southern College of Optometry Copyright K. Kehbein 2015-COPE# 43881FV Financial Disclosures I have no financial interests to disclose. Copyright K. Kehbein 2015-COPE# 43881FV Course Outline • Down Syndrome • Definition, General Physical Characteristics, Common Ocular Findings • Autism • Definition, General Physical Characteristics, Common Ocular Findings • Cerebral Palsy • Definition, General Physical Characteristics, Common Ocular Findings • Examination Techniques • Binocular Vision Abnormalities • Prescribing Glasses • Vision Therapy 1 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV DOWN SYNDROME Copyright K. Kehbein 2015-COPE# 43881FV Down Syndrome • One of the first developmental disabilities to be recognized as a syndrome • Langdon Down in 1866 • Historically diagnosed based on physical findings • 1. Upward slanting of temporal palpebral fissures • 2. Significant presence of epicanthal folds Copyright K. Kehbein 2015-COPE# 43881FV Inherited Genetic Anomaly • Trisomy 21 • Inherited 3 copies of chromosome 21 • About 94% of all cases • Translocation • Portion of chromosome 21 breaks off and attaches to another chromosome • Attaching to chromosome 14 is most common • About 5% of all cases • Mosaicism • Some cells have 46 chromosomes and some have 47 chromosomes- 3 copies of chromosome 21 • About 1% of all cases 2 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Prevalence • 1 in 691 babies born with in the United States with Down Syndrome • Affects all races and economic levels • Most common genetic anomaly • Risk Factors • Increasing maternal age • Greater risk of having a baby with Down Syndrome • More babies with Down Syndrome born to women under 35yo • Younger women are more fertile Copyright K. Kehbein 2015-COPE# 43881FV Physical Characteristics http://www.doctortipster.com/3349-down-syndrome-mongolism-or-trisomy-21.html Copyright K. Kehbein 2015-COPE# 43881FV Speech and Language • Poor hearing • Slow processing • Small instruction sets • Wait for comprehension • Poor expressive speech • Unable to say what they want/mean • Able to understand what you are telling/asking them 3 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings • Refractive Error • Born with roughly normal amounts • Deficient emmetropization? • Moderate to High Amounts • Hyperopia • Myopia • Astigmatism • Increases with age, oblique axis • Reduced visual acuity with optimal correction • Consider modifications for home, school, work Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Accommodative Insufficiency • Poor ability to stimulate accommodation to age-normal levels • Significantly high lag • Likely due to sensory pathway deficit • Higher amounts of hyperopia show higher lag • Lower amounts of hyperopia show lower lag • Use of bifocals • Help improve near acuity • Help improve accommodative functioning • May no longer need bifocal after improvement Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Strabismus • Esotropia more common than exotropia • Commonly alternating vs. unilateral • If possible amblyopia • Follow PEDIG patching recommendations • Moderate amblyopia: 2 hours of patching/day with 1 hour of near work • Severe amblyopia: 6 hours of patching/day with 1 hour of near work 4 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Ocular Health • Blepharitis • Cataracts • Keratoconus • Brushfield Spots • More common in lighter pigmented iris • Vessel changes in fundus Copyright K. Kehbein 2015-COPE# 43881FV AUTISM Copyright K. Kehbein 2015-COPE# 43881FV Autism • Group of developmental brain disorders • Starts before age 3yo Autismems.net 5 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Autism Spectrum Disorder Autismc.com Copyright K. Kehbein 2015-COPE# 43881FV Prevalence • Depends on study • 2008: 1 in 88 • Centers for Disease Control and Prevention (CDC) • 1 in 54 boys • 1 in 252 girls • Causes/Risk Factors: • 4 times more common in males • Combination between environment, genetics, and biology • Increased risk if sibling has autism • Parents over 35yo • Mother with autoimmune condition • Low birth weight/ prematurity/ breech birth Copyright K. Kehbein 2015-COPE# 43881FV Physical Characteristics • Gastro-intestinal problems • At least 24% have one chronic problem • Sleep disturbances • Trouble falling and staying asleep • Mood disorders • Phobias, ADD/ADHD, obsessive compulsive disorder, anxiety, depression • Intellectual Disability • Estimated to be over 60% • Medical costs are 7x higher for people with ASD • Approximately 75% need lifelong support 6 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings • Decreased eye contact • Poor oculomotor control • Deficient saccades and/ore pursuits • Optic neuropathy • Secondary to dietary deficiencies Black K, McCarus C, Collins ML, Jensen A. Ocular manifestations of autism in ophthalmology. Strabismus. 2013;21(2):98-102. Copyright K. Kehbein 2015-COPE# 43881FV Early Eye Tracking • At-risk infants (family member with autism spectrum disorder) vs. control infants • Ages 6-10 months • Is there a change in brain potential when a face shifts gaze toward and away infant? • Used potential components which are precursors for adult facial sensitivity • At-risk infants: no change in potential • Control infants: change in potential • Higher proportion of at-risk infants developed autism at 36 months Copyright K. Kehbein 2015-COPE# 43881FV Treatment • Variety of therapies • Applied Behavior Analysis • Analysis of behavior to help make changes to more socially acceptable behavior • Positive reinforcement for socially acceptable behavior • No reinforcement for less than ideal behaviors • Those that could pose harm • Those that prevent learning 7 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV CEREBRAL PALSY Copyright K. Kehbein 2015-COPE# 43881FV Cerebral Palsy • Group of disorders of the development of posture and movement • First described by Dr. William James Little in 1862 • Includes: • Limitations in activity • Non-progressive disturbances • Other disturbances • Sensation • Cognition • Communication • Perception • Behavior • Seizures Copyright K. Kehbein 2015-COPE# 43881FV Prevalence • 1 to 2 per 1,000 in developed countries • Higher prevalence in low birth weight children • Higher in lower socioeconomic class, when normal birth weight 8 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Causes/Risk Factors • Prenatal • Intrauterine infection, death of co-twin, cerebral ischemia • Perinatal • Neonatal convulsions, jaundice, infection, multiple gestations • Postnatal • Brain damage in first few months of life • Congenital or Acquired • Congenital: development, malformations, syndromes • Acquired: trauma, infection, ischemia, hypoxia Copyright K. Kehbein 2015-COPE# 43881FV Physical Characteristics • Physiological Subtypes • 1. Spastic: 70-80% of cases • Stiffness of muscles • Damage to periventricular white matter • 2. Dyskinetic: 10-15% of cases • Uncontrolled, slow, writhing movements • Damage to basal ganglia • 3. Ataxic: <5% of cases • Difficulty with balance and coordination • Cerebellar damage Copyright K. Kehbein 2015-COPE# 43881FV Physical Characteristics cont. • Anatomical Subtypes • 1. Hemiplegia: 20-30% of cases • Dysfunction of one side of the body • 2. Diplegia: 30-40% of cases • Reduced ability to use upper or lower limbs • 3. Quadriplegia: 10-15% of cases • Reduced ability in all 4 limbs and trunk • Most severe form 9 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings • Refractive Error: • Low to moderate hyperopia • High myopia in more severe forms • Visual Field Defects: • More peripheral field defects than central field defects Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Strabismus • Roughly 50% have strabismus • Equal amounts of exotropia and esotropia • Worsening binocularity with more severe CP • 70% lack binocularity • Potential for amblyopia with constant strabismus • Alternating strabismus common Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Oculomotor Dysfunction • Gross assessment of saccades/pursuits • NSUCO • 25% show deficit • Equal amounts of saccadic and pursuit deficiency • Assessment of reading eye movements • Developmental Eye Movement Test (DEM) • 20% have normal findings • 80% have oculomotor dysfunction, automaticity problem or combination of the two 10 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Accommodative Insufficiency • 60% have higher than normal lag • Consider plus reading glasses at near • Visual Perceptual Deficits • Poor visual imagery • Poor visual-spatial relations Copyright K. Kehbein 2015-COPE# 43881FV Common Ocular Findings cont. • Ocular Disease • Nystagmus • Optic Atrophy • Retinopathy of Prematurity Fazzi E, Signorini SG, La Piana R, et.al. Neuroophthalmological disorders in cerebral palsy: ophthalmological, oculomotor, and visual aspects. Developmental Medicine & Child Neurology. 2012;54:730-6. Copyright K. Kehbein 2015-COPE# 43881FV EXAMINATION TECHNIQUES 11 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Fix and Follow • Not able to quantify visual acuity • Only able to tell if patient can see object • Able to tell if fixation preference • Resistance to occlusion • Can be performed as part of ocular motility testing Copyright K. Kehbein 2015-COPE# 43881FV Preferential Looking • Easy to administer to young children • Provides a way to assess what the child is seeing • Large range of spatial frequencies available • 20/2400 to 20/10 • Extended period of time to administer • OD, OS, OU • Show card and make decision as to where the child is looking • 50% chance of getting it correct Copyright K. Kehbein 2015-COPE# 43881FV Lea Symbols • 4 pictures • Circle, square, house, apple • Well calibrated • Distance and near 12 3/16/15 Snellen • Recognition acuity • Standard for comparison of Copyright K. Kehbein 2015-COPE# 43881FV other techniques • Historical “Gold Standard” Copyright K. Kehbein 2015-COPE# 43881FV Binocularity/ Accommodation • Hirschberg • Assessment for strabismus vs. pseudo-strabismus • Bruckner • Assessment for difference in refractive error, strabismus, ocular health complications • Cover Test • Assessment for binocular posture • MEM Retinoscopy • Assessment of accommodative response • Push-up/ Pull-away amplitude • Assessment of accommodative amplitude Copyright K. Kehbein 2015-COPE# 43881FV NSUCO • Northeastern State University College of Optometry oculomotor test • Direct observation of saccades and pursuits • Type of free space testing • Rate patient based on • Ability • Accuracy • Head and body movement • Graded on a 1-5 scale (5 is the best) • Norms • Ages 5-14yo • Girls show better scores earlier • Boys “catch up” around 9yo 13 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV King Devick Test • Visual verbal format to test saccades • Type of psychometric test • One demonstration card and 3 test cards • Patient calls out numbers as quickly as possible • Evaluate time it takes to complete card based on age norms • Norms • Ages 6-14yo • Compare time taken to average range Copyright K. Kehbein 2015-COPE# 43881FV Developmental Eye Movement Test • Visual verbal format to test saccades • Type of psychometric test • Patient asked to call off a series of numbers as quickly as possible • 2 vertical tests • 1 horizontal test • Not allowed to use finger or ruler as a guide • Time to complete each test is recorded as well as the errors • Norms • Ages 6-34yo • Calculate total times and ratio • Calculate z-score for vertical and ratio Refractive Error • Retinoscopy • Dry • Static= accommodation relaxed, distance • Mohindra • Near retinoscopy • Monocular in a darkened room (only light from retinoscope) • 50cm working distance • Subtract 1.25D from neutralizing lens Copyright K. Kehbein 2015-COPE# 43881FV viewing • Dynamic= near accommodative abilities • Cycloplegic • Decreases fluctuations from accommodation 14 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Ocular Health • Intraocular Pressure • Tonopen • iCare • Anterior Segment • Slit lamp • 20D and penlight • Posterior Segment • BIO Copyright K. Kehbein 2015-COPE# 43881FV Developmental Testing Copyright K. Kehbein 2015-COPE# 43881FV Visual Information Processing Testing • Visual Analysis testing • Investigates visual discrimination, visual memory, visual closure, form constancy, spatial relationships, figure-ground • Types of tests: • Test of Visual Perceptual Skills (TVPS) • Motor Free Visual Perceptual Test (MVPT) • Visual Motor Integration • Investigates eye-hand coordination • Types of tests: • Beery VMI • Developmental Test of Visual Perception (DTVP) 15 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV BINOCULAR VISION ABNORMALITIES Copyright K. Kehbein 2015-COPE# 43881FV Accommodative Abnormalities • Accommodative Insufficiency • Poor ability to stimulate accommodation • Finding of high lag on MEM testing • Reduced amplitude of accommodation • Recommended treatment: • Near work glasses • Bifocal correction • How much to prescribe? • Does it actually work? Copyright K. Kehbein 2015-COPE# 43881FV Bifocals • Used to help improve near acuity and poor accommodative skills • Found to also help improve visual perceptual skills in patients with Down Syndrome: • Improvement in reading scores • Improvement in Visual Closure • Improvement in Visual Form Constancy • No improvement in handwriting 16 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Eye Tracking Abnormalities • Oculomotor dysfunction • Poor fixation, saccades, and/or pursuits • Testing with NSUCO • Autism: poor fixation skills • Cerebral Palsy: poor motor control • Testing with DEM • Cerebral Palsy • 20% normal • 20% oculomotor problem only • 32% automaticity problem only • 27% oculomotor and automaticity problems Copyright K. Kehbein 2015-COPE# 43881FV Eye Tracking Abnormalities cont. • Recommended Treatment: • Vision Therapy • Accommodations in the classroom • Use finger or ruler to follow along • Large print worksheets/textbooks Copyright K. Kehbein 2015-COPE# 43881FV Binocular Abnormalities • Strabismus • Cerebral Palsy: • Almost 50% have strabismus • Most likely to be alternating vs. unilateral • Equal between esotropia and exotropia • Down Syndrome: • Slightly less than 50% have strabismus • Esotropia more common than exotropia 17 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Binocular Abnormalities cont. • Non strabismic binocular vision abnormalities • Autism: • Poor convergence skills • Likely poor stereopsis • Recommended Treatment: • Vision Therapy • Strabismus Surgery • Studies have found that strabismus surgery on developmentally delayed children may result in overcorrection when using the standard measurements • Occlusion • Prism Glasses Copyright K. Kehbein 2015-COPE# 43881FV PRESCRIBING GLASSES Copyright K. Kehbein 2015-COPE# 43881FV Prescribing Full Correction • Hyperopia: • Consider patient’s accommodative ability • If poor, may struggle with seeing well in distance/ near • Myopia: • Consider patient’s accommodative ability • May need bifocal at near to help supplement accommodation • Astigmatism: • Improvement of visual functioning/ acuity 18 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Cutting the Prescription • Hyperopia: • Consider patient’s binocular vision status • If poor, too much plus may cause negative change in posture • Myopia: • Consider patient’s visual needs • If mostly at near, may not need full minus correction Copyright K. Kehbein 2015-COPE# 43881FV Contact Lenses • Patients with keratoconus • Patients with dry eye Copyright K. Kehbein 2015-COPE# 43881FV VISION THERAPY 19 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Goals and Expectations • Improvement in visual skills • Decrease in strabismus frequency or magnitude • Improved fixation and eye tracking skills • Flexibility of accommodation • Setting appropriate expectations • May not be “normal” • Did the patient show improvements in behavior? • Did the patient show improvements in visual skills? Copyright K. Kehbein 2015-COPE# 43881FV Improvement of Accommodation • Doctor Goals for Vision Therapy • Greater flexibility • More accurate response • Increased amplitude • Patient/Parent Goals for Vision Therapy • Less visual fatigue • Less blur • Improved time with near tasks Copyright K. Kehbein 2015-COPE# 43881FV Improvement of Eye Tracking • Doctor Goals for Vision Therapy • Increased fixation time • More accurate saccades/pursuits • Decreased head/body movement • Patient/Parent Goals for Vision Therapy • Eye contact • Potential for improvement in reading skills 20 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Improvement of Binocularity • Doctor Goals for Vision Therapy • Decrease in angle of strabismus • Improvement in fusion capabilities • Patient/Parent Goals for Vision Therapy • Straight eyes • Appreciation of depth perception Copyright K. Kehbein 2015-COPE# 43881FV CASE EXAMPLE Copyright K. Kehbein 2015-COPE# 43881FV Patient Demographics • 10 year old Caucasian female • Chief complaint: alternating exotropia, equal at distance and near • Ophthalmologist wants to do surgery, Mom wants a second opinion • Medical History: Cerebral Palsy • Ocular History: eye turn, glasses wear 21 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Preliminary Data • Entering aided acuities (distance and near) • OD: 20/20• OS: 20/20• Habitual Rx • +6.00DS OU • Pupils: Equal, round, reactive to light (-) APD • EOMs: Full, no restrictions, poor pursuits • Confrontation fields: full to finger count OU • Cover Test through Habitual Rx: • Distance 25∆ Alternating Exotropia • Near 25∆ Alternating Exotropia Copyright K. Kehbein 2015-COPE# 43881FV Refractive Correction • Took off glasses for lensometry… • Patient now has esotropia • Distance retinoscopy: • +6.00DS OU • Trial amount to get good acuity and binocularity • +2.00DS OU------ still had esotropia, reduced acuity • +4.00DS OU------ exophoria, good acuity Copyright K. Kehbein 2015-COPE# 43881FV Assessment and Plan • Assessment: • Hyperopia OU • Exotropia/Esotropia • Oculomotor dysfunction • Plan: • New glasses prescription: +4.00DS OU • Begin vision therapy to work on eye tracking and fusion activities 22 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV Thank You • Karen Kehbein, OD, FCOVD • [email protected] Copyright K. Kehbein 2015-COPE# 43881FV References- Down Syndrome • National Down Syndrome Society • http://www.ndss.org/ • Taub M, Bartuccio M, Maino D. Visual Diagnosis and Care of the Patient with Special Needs. 2012: Lippincott Williams & Wilkins. Chapter 4 • Kranjc B. Ocular abnormalities and systemic disease in down syndrome. Strabismus. 2012;20(2):74-7. • Anderson HA, Manny RE, Glasser A, Stuebing KK. Static and Dynamic Measurements of Accommodation in Individuals with Down Syndrome. Investigative Ophthalmology & Visual Science. 2011;52(1):310-7. • Nandakumar K, Evans, MA, Briand K, Leat SJ. Bifocals in Down Syndrome study (BiDS): analysis of video recorded sessions of literacy and visual perceptual skills. Clinical and Experimental Optometry. 2011;94(6):575-85. • Yahalom C, Mechoulam H,Cohen E, Anteby I. Strabismus surgery outcome among children and young adults with Down syndrome. JAAPOS. 2010;14(2):117-9. Copyright K. Kehbein 2015-COPE# 43881FV References-Autism • Autism Speaks • www.autismspeaks.org • Autism Society • www.autism-society.org • Taub M, Bartuccio M, Maino D. Visual Diagnosis and Care of the Patient with Special Needs. 2012: Lippincott Williams & Wilkins. Chapter 8 • Elsabbagh M, Mercure E, Hudry K, et.al. Infant Neural Sensitivity to Dynamic Eye Gaze is Associated with Later Emerging Autism. Current Biology. 2012;22(4):338-42. • Black K, McCarus C, Collins ML, Jensen A. Ocular manifestations of autism in ophthalmology. Strabismus. 2013;21(2):98-102. • Pendergrass S, Girirajan S, Selleck S. Uncovering the etiology of autism spectrum disorders: genomics, bioinformatics, environment, data collection and exploration, and future possibilities. Biocomputing. 2014; 422-26. 23 3/16/15 Copyright K. Kehbein 2015-COPE# 43881FV References-Cerebral Palsy • United Cerebral Palsy • www.ucp.org • Taub M, Bartuccio M, Maino D. Visual Diagnosis and Care of the Patient with Special Needs. 2012: Lippincott Williams & Wilkins. Chapter 3 • Fazzi E, Signorini SG, La Piana R, et.al. Neuroophthalmological disorders in cerebral palsy: ophthalmological, oculomotor, and visual aspects. Developmental Medicine & Child Neurology. 2012;54:730-6. 24 Pill Problems PILL PROBLEMS: OCULAR COMPLICATIONS FROM SYSTEMIC MEDICATIONS Alan G. Kabat, OD, FAAO Memphis, Tennessee Common Drugs with Ocular Complications Alendronate Amiodarone Benztropine Diphenhydramine Hydroxychloroquine Sildenafil Tamsulosin Tetracycline Topiramate Warfarin Trade: Benadryl, numerous generic Drug class: non-selective histamine blocker Primary: nasal & non-nasal signs and symptoms of seasonal allergy, especially allergic rhinitis Secondary: insomnia, vertigo, motion sickness Ingredient in numerous cold medications and sleep aids (e.g. Nytol, Tylenol PM) Alan G. Kabat, OD, FAAO Indication(s): Typical dosage: 25-50 mg, q4h or PRN 1 Pill Problems Ocular Complications Dry Eye Dry Eye Due to anticholinergic effects of the medication1 Diminishes aqueous production via autonomic innervation to the primary lacrimal gland Opposite action of Salagen (pilocarpine) Can also cause dry mouth, urinary retention and constipation Dose-dependent effect Reversible 1. Simons FE. Advances in H1-antihistamines. N Engl J Med 2004; 351(21):2203-17. Mah FS, O'Brien T, Kim T, Torkildsen G. Evaluation of the effects of olopatadine ophthalmic solution, 0.2% on the ocular surface of patients with allergic conjunctivitis and dry eye. Curr Med Res Opin. 2008 Feb;24(2):441-7. Other Manifestations … to evaluate the safety of olopatadine 0.2% in a population of patients with both allergic conjunctivitis and dry eye. 52 patients with ocular allergy and mild-to-moderate dry eye were evaluated. Randomized to either olopatadine hydrochloride 0.2% or a tear saline once-daily for 1 week. Evaluated TBUT, corneal and conjunctival staining, fluorophotometry, Schirmer's test, injection, and symptom evaluations. No significant differences between the treatment groups were observed ( p > 0.05). Drowsiness & fatigue Anticholinergic effects including dry mouth, urinary retention, and constipation Potential for cardiac complications, particularly arrhythmias and tachycardia Potential for recreational use/abuse Conclusion: As there were no significant changes in the signs & symptoms of dry eye, olopatadine 0.2% is safe to use in ocular allergy patients with mild-to-moderate dry eye. Simons FE. Advances in H1-antihistamines. N Engl J Med 2004; 351(21):2203-17. OTC vs. Rx Drugs Similar Medications with Similar Effects Chlorpheniramine (Chlor-Trimeton) Brompheniramine (Dimetane) Dimenhydrinate (Dramamine) Meclizine (Bonine) Loratadine (Claritin, Alavert) Cetirizine (Zyrtec) Alan G. Kabat, OD, FAAO Patients do not always equate items that they buy on store shelves with the terms “drugs” or “medications”. Practitioners and technicians must be SPECIFIC when screening. Checklists on intake forms work well. 2 Pill Problems and derivatives Trade: Sumycin®, Tetracyn®, numerous generics Drug class: Tetracycline antibiotic and Indication(s): Includes doxycycline and minocycline, among others Primary: infection by susceptible bacterial strains Ocular Complications Scleral discoloration (minocycline) derivatives Respiratory, skin/soft tissue, UTIs most commonly Rarely a “first-line” antibiotic therapy Secondary: immunomodulatory agent for sebaceous disorders, including rosacea and MGD Typical dosage: 250 mg QID or 500 mg BID Ocular Complications Pseudotumor cerebri or Idiopathic intracranial hypertension Miraldi V, Singh AD, Jeng BH. The whites of my eyes have turned blue! EyeNet, March 2007 Pseudotumor cerebri 0.9 per 100,000 people in general population, including children Increased risk in women aged 20-44 who are 20% or more above their ideal body weight Diagnosis - based on modified Dandy criteria Awake and alert patient Signs and symptoms of increased ICP Absence of localized neuro exam findings, except for CN VI paresis Normal CSF fluid findings except for increased pressure Absence of deformity, displacement, and obstruction of ventricular system No other identifiable cause of intracranial hypertension Other compounds associated with PTC Oral contraceptives Vitamin A Amiodarone Alan G. Kabat, OD, FAAO Glucocorticoids (withdrawal) Mineralocorticoids (withdrawal) e.g. e.g. cortisol aldosterone 3 Pill Problems Other Manifestations Tooth Discoloration Photosensitivity Trade: Coumadin, numerous generics Drug class: anticoagulant (“blood thinner”) Ocular Complications Indication(s): Subconjunctival hemorrhage Prophylaxis and/or treatment of venous thrombosis and pulmonary embolism Thromboembolic complications associated with atrial fibrillation and/or cardiac valve replacement To reduce the risk of death, recurrent myocardial infarction, and thromboembolic events such as stroke or systemic embolization after myocardial infarction Hypercoagulable states Typical dosage: 5-10 mg daily Bodack MI. A warfarin-induced subconjunctival hemorrhage. Optometry. 2007;78(3):113-8. Ocular Complications Hyphema 76-year-old female Subconjunctival hemorrhage & heaadache Case review showed concurrent therapy with warfarin, levothyroxine, atorvastatin, metoprolol, and paroxetine. INR = 9.9 Alan G. Kabat, OD, FAAO 4 Pill Problems Ocular Complications Retinal hemorrhage Other Manifestations Bleeding and bruising - can be potentiated by a variety of drugs & other substances: Antibiotics (e.g. aminoglycosides, macrolides, fluoroquinolones and tetracyclines) Beta-blockers Levothyroxine Atorvastatin Fish oil / Ω-3 / vitamin E Alcoholic beverages Cranberry products Ginseng Garlic Ginko biloba St. John’s wort Management Tips Patients on warfarin therapy need to be cognizant of everything they put in their mouths. Medications, food, beverages… EVERYTHING!! Trade: Cordarone, Pacerone, numerous generics Drug class: anti-arrhythmic agent (Class III) Indication: for life-threatening cardiac arrhythmias INR (International Normalized Ratio) should be performed by PCP routinely. Measures the extrinsic pathway of coagulation Normal: 0.8 – 1.2 Target range on therapy: 2.0 – 3.0 Dangerous: >4.0 Ocular Complications Corneal Verticillata hemodynamically unstable ventricular tachycardia shock-resistant, recurrent ventricular fibrillation Typical dosage: 200-400 mg/day Corneal Verticillata i.e. “vortex keratopathy”, “hurricane keratopathy” Generally asymptomatic Alan G. Kabat, OD, FAAO Rarely may cause haloes or slight decrease in VA Seen in ~90% of patients on amiodarone >6 mos, especially those taking >400 mg/day. No management required; Self-limiting & reversible 5 Pill Problems WARNING: Vortex keratopathy can also be associated with… ? FABRY’S DISEASE Hereditary enzyme deficiency Ocular Complications Pseudotumor cerebri or Idiopathic intracranial hypertension α-Galactosidase A located on the X-chromosome Leads to intracellular accumulation of neutral glycosphingolipids in various organs, e.g. skin, eyes, nervous tissue, kidney and heart Findings: angiokeratomas, pain in the hands & feet, lesions of the mouth and multiple ocular signs Other Manifestations “Blue skin”, “blue man syndrome” Long-term use; more commonly seen with lighter skin tones Ocular Complications Trade: Topamax Drug class: anticonvulsant Indication(s): Acute myopic shift Acute angle-closure glaucoma Primary: treatment of epilepsy and other seizure disorders Secondary: prevention of migraine headaches in adults Off-label: treatment of bipolar disorder, obsessivecompulsive disorder, alcoholism, smoking cessation, cocaine dependence, eating disorders, and neuropathic pain. Typical dosage: (adults) 100 – 400 mg daily Alan G. Kabat, OD, FAAO 6 Pill Problems Levy J, Yagev R, Petrova A, Lifshitz T. Topiramateinduced bilateral angle-closure glaucoma. Can J Ophthalmol. 2006;41(2):221-5. Pathological Mechanism Appears to be a sulfa-allergic response 35-year-old woman presenting to E.D. c/o severe eye pain & blurry vision OU Hx: Oral topiramate 50 mg BID X 1 week IOP: 57 mm Hg OD, 56 mm Hg OS B-scan revealed 360° ciliochoroidal detachment OU Cyclocongestive glaucoma Normal open angle Cyclocongestive angle closure Trade: Flomax Drug class: alpha-adrenergic antagonist Indication(s): NO pupil block; NO iris bombé! Dysgeusia (taste perversion) Parasthesias (numbness & tingling) Fatigue Difficulty with concentration, attention and memory Weight loss Results in lens thickening; this, in addition to the forward rotation of the lens-iris diaphragm induces a myopic shift Lens thickening generally does not contribute to angle closure Other Manifestations Congestion of ciliary body allows lens zonules to go slack Swelling/congestion and forward rotation of the ciliary body Ciliochoroidal effusion with forward shifting of lens-iris diaphragm Induces extreme anterior chamber shallowing and angle-closure Mechanism: works by relaxing smooth muscle at the distal portion of the urethra Primary: signs and symptoms of benign prostatic hyperplasia (BPH) Off label: urinary retention in women and those with multiple sclerosis; facilitated passage of kidney stones Typical dosage: 0.4 mg once daily Alan G. Kabat, OD, FAAO 7 Pill Problems Ocular Complications IFIS - Intra-operative Floppy Iris Syndrome IFIS Clinical manifestations: Management: Other Manifestations Poor preoperative dilation Iris billowing and prolapse Progressive intraoperative miosis Identify patients at risk and discontinue medication if possible Use of stronger dilating agents, e.g. epinephrine and/or atropine Use of Malyugin or Morcher ring Sulfa Allergy Pustular, erythematous skin eruptions with urticaria Can affect any part of the body May progress to Stevens-Johnson syndrome in severe cases Other Manifestations Fever, chills, body aches, or flu symptoms Light headedness, dizziness, weakness, drowsiness Headache Nausea, diarrhea Runny nose Trade: Viagra Similar medications: tadalafil (Cialis), vardenafil (Levitra, Staxyn) Drug class: phosphodiesterase enzyme inhibitor (PDEI) Indication(s): Originally studied as an anti-angina medication! Primary: treatment of erectile dysfunction Secondary: symptoms of benign prostatic hyperplasia Off-label: pulmonary hypertension, Raynaud's phenomenon (Revatio) Diminished ejaculate Decreased sex drive, which leads us to… Alan G. Kabat, OD, FAAO Typical dosage: 50 mg (not to exceed 100 mg) 8 Pill Problems Ocular Manifestations Mechanism of action (warning: GRAPHIC) Cyanopsia (“blue vision”) By affecting PDE6 in the retina, sildenafil can lead to altered color vision perception (usually a blue or green “tinge” to vision). 4 out of 5 men without vascular risk factors reported this problem after taking sildenafil. Ocular Manifestations Tarantini A, Faraoni A, Menchini F, Lanzetta P. Bilateral simultaneous nonarteritic anterior ischemic optic neuropathy after ingestion of sildenafil for erectile dysfunction. Case Report Med. 2012. Nonarteritic anterior ischemic optic neuropathy Other Manifestations Headache Stuffy nose Facial flushing 60-year-old diabetic man c/o sudden decrease of vision OU, 16 hours after his 3rd consecutive 50 mg daily sildenafil ingestion. “In patients with a predisposing diabetic condition, sildenafil intake can cause changes in NO balance altering the normal vascular autoregulation so that the ocular circulation may not be able to compensate for a drop in systemic blood pressure. ” Alan G. Kabat, OD, FAAO And of course… 9 Pill Problems Ocular Manifestations Trade: Plaquenil, numerous generic Drug class: aminoquinoline anti-malarial drug DMARD treatment of malaria treatment of discoid and systemic lupus erythematosus, and rheumatoid arthritis Typical dosage: 400-800 mg/day (malaria) 200-400 mg/day (lupus & RA) Ocular Manifestations Corneal deposits Indication(s): “Bulls-eye” maculopathy Dosso A, Rungger-Brändle E. In vivo confocal microscopy in hydroxychloroquine-induced keratopathy. Graefes Arch Clin Exp Ophthalmol. 2007;245(2):318-20. Ocular Manifestations “Bulls-eye” maculopathy Anderson C, Blaha GR, Marx JL. Humphrey visual field findings in hydroxychloroquine toxicity. Eye (Lond) 2011 December; 25(12): 1535-45. Alan G. Kabat, OD, FAAO 66 visual fields from patients with HCQ retinal toxicity. HVF changes preceded fundus changes in 60% of patients. Abnormalities were more obvious on pattern deviation than the gray scale. Authors recommend white stimulus 10-2 fields (vs. redstimulus), as per AAO guidelines. 10 Pill Problems OCT: The New Standard Rodriguez-Padilla JA, Hedges TR 3rd, Monson B, et al. High-speed ultra-high-resolution optical coherence tomography findings in hydroxychloroquine retinopathy. Arch Ophthalmol. 2007 Jun;125(6):775-80. Chen E, Brown DM, Benz MS, et al. Spectral domain optical coherence tomography as an effective screening test for hydroxychloroquine retinopathy (the "flying saucer" sign).Clin Ophthalmol. 2010 Oct 21;4:1151-8. ERG: The Emerging Standard OCT: The New Standard Chen JJ, Tarantola, RM, Kay CN, Mahajan VB. Hydroxychloroquine (Plaquenil) Toxicity and Recommendations for Screening. EyeRounds.org. August 30, 2011. Available from: http://EyeRounds.org/cases/139IplaquenilItoxicity.htm. Focal thinning and loss of parafoveal PIL (photoreceptor integrity line) Risk factors for maculopathy Maintenance dose greater than 6.5 mg/kg/d Normal mfERG mfERG in HCQ toxicity 120 lb. woman: >400 mg/d 200 lb. man: >600 mg/d Duration of treatment: >10 years Evidence of renal insufficiency or hepatic disease Obesity Advanced age Presence of macular degeneration or dystrophy Other Manifestations Vertigo, tinnitus, headache Skin rashes and dermatitis GI disturbances Muscle weakness Trade: Fosamax, numerous generic Drug class: aminobiphosphonate anti-resorptive agent (strengthens bones) similar drugs include Actonel, Boniva Indication(s): Primary: treatment or prevention of osteoporosis, treatment of Paget’s disease Off label: Metastatic bone cancer, hypercalcemia, vitamin D overdose Alan G. Kabat, OD, FAAO Typical dosage: 5-10 mg/day (osteoporosis) 40 mg/day (Paget’s disease) X 6 months 11 Pill Problems Ocular Manifestations Non-specific conjunctivitis and/or keratitis Ocular Manifestations Episcleritis, scleritis, anterior uveitis McKague M, Jorgenson D, Buxton KA. Ocular side effects of bisphosphonates: A case report and literature review. Can Fam Physician. 2010 Oct;56(10):1015-7 Other Manifestations Nausea, dyspepsia, acid regurgitation Abdominal pain, constipation, diarrhea Musculoskeletal pain Hypocalcemia Osteonecrosis of the jaw Trade: Cogentin (discontinued in US); numerous generics Drug class: anti-parkinsonian medication Indication(s): Possesses both anticholinergic and antihistaminic effects As an adjunct in the therapy of all forms of parkinsonism For control of medication-induced movement disorders due to antipsychotic agents, e.g. Parkinsonian Tremor Typical dosage: 1-2 mg/day Ocular Manifestations Anticholinergic effects (think atropine!): Mydriasis Cycloplegia Alan G. Kabat, OD, FAAO Chlorpromazine (Thorazine), haloperidol (Haldol), risperidone (Risperdal), olanzapine (Zyprexa), quetiapine (Seroquel) Impaired accommodation Transient refractive shift Dry eyes 12 Pill Problems Ocular Manifestations Esotropia / diplopia Proposed mechanism: The ratio of convergence to accommodation may increase with anticholinergics due to partial block of accommodation. To see a near target in the setting of blocked accommodation, children would increase accommodative effort, resulting in increased convergence. Too much convergence may cause esotropia. Other Manifestations MORE anticholinergic effects PERIPHERAL Dry mouth Hot, dry skin Tachycardia Constipation Urinary retention CENTRAL Sedation Confusion Delirium Slowed cognitive function Oh SY, Shin BS, Lee YH, Lee AY, Kim JS. Benztropine-induced Esotropia and Mydriasis. J Neuroophthalmol. 2007 Dec;27(4):312-3. Risk of falls CONCLUSIONS: Optometric PHYSICIANS must realize that the eye is impacted by numerous systemic diseases and drugs. A working knowledge of pharmacology and common drugs is essential (especially when dealing with an adult or geriatric population). Even if you don’t (or can’t) prescribe them, you have the responsibility to recognize the potential ocular impact of commonly prescribed medications. Questions? Email me at: [email protected] Alan G. Kabat, OD, FAAO 13