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This resource highlights rare but critical eye conditions that can be diagnosed during eye exams, potentially saving lives. Learn to recognize signs and symptoms, differentiate emergencies, and understand management approaches. Be prepared to identify conditions like CNIII Palsy, CNIV Palsy, CNVI Palsy, CNVII Palsy, Muscle Entrapment, Visual Field Tumors, and Eyelid Abnormalities such as Sebaceous Cell Carcinoma and Capillary Hemangioma. Early detection and appropriate follow-up are crucial in these cases.
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An Eye Exam can Save a Life Potentially fatal conditions found on eye exam
Disclaimers • No financial interests • Any reference to branded pharmaceuticals is unintentional • This is not an exhaustive list • This type of lecture will drive you crazy: skipping right past what’s common, what’s likely, and highlighting what is fatal. This can be unsettling. • You may never see these diagnoses in practice, but it’s best to be prepared
CNIII Palsy http://www.mrcophth.com/ptosis/thirdnerveplasy.html https://www.aafp.org/afp/2010/0715/p187.html
Parasympathetic pupillomotor fibers: Peripheral/compression Vaso Nervorum: Microvascular Disease/diabetes/hypertension https://twitter.com/alaamri14121/status/752168425217200128
Posterior Communicating Artery Aneurysm https://www.researchgate.net/figure/MRI-showing-a-posterior-communicating-artery-aneurysm_fig4_5411575
CNIII Palsy Management • Who to treat as emergencies: • Pupil-involving CNIII palsy ER • Evolving CNIII ER • “Over the last few weeks this eyelid keeps drooping more and my double vision is getting worse and worse” • Who can be monitored • Some clinicians image everyone for liability reasons • A 70-year-old diabetic hypertensive with an acute onset, pupil-sparing CNIII palsy can be watched. Follow-up is needed.
CNIV Palsy • Usually: trauma, congenital, vasculopathy • But, rarely… tumor (often other signs/symptoms) • Watch for: • no improvement over weeks to a couple months • Progression over time
CNVI Palsy • Usually due to intracranial hypertension, microvascular disease, trauma or idiopathic • But, rarely… tumor • Watch for: • no improvement over weeks to a couple months • Progression over time
CNVII Palsy: Bell vs Stroke https://www.slideshare.net/CpArya/facial-nerve-palsy-by-dr-chandra-prakash-arya-bsc-bds-mds-pms-rntcp
Stroke: forehead sparing https://www.jems.com/articles/print/volume-39/issue-5/features/differentiating-facial-weakness-caused-b.html
Bell palsy: forehead involving https://www.jems.com/articles/print/volume-39/issue-5/features/differentiating-facial-weakness-caused-b.html
CNVII Palsy • Also consider sarcoidosis, lyme disease • With hearing loss consider Ramsay Hunt Syndrome/Zoster • If frontalis function is preserved with facial droop ER • If entire hemiface affected, highly likely Bell Palsy treat K exposure
Muscle Entrapment: Oculocardiac reflex (OCR) https://entokey.com/15-3/
EOM Entrapment in kids • Suspected entrapment of a muscle in a child ER • The OCR: • V1 relays through the ciliary ganglion and the vagus nerve is activated, decreasing cardiac output • Worsened with hypercarbia (CO2), hypoxia (O2), opiate ingestion, younger age (increased vagal tone), and light anesthesia • Norco/Percocet/Oxycodone not recommended for pain en route to ER • Frequently encountered with strabismus surgery, and occasionally with any operation near branches of the trigeminal nerve (ENT, Plastics etc)
Visual Fields: tumors https://www.neec.com/neuro-ophthalmology-boston/pituitary-tumor/
Melanoma http://morancore.utah.edu/basic-ophthalmology-review/eyelid-masses/ https://www.aao.org/diagnose-this/diagnose-this-malignant-melanoma-of-eyelid-2
Eyelids • Easy to look past the eyelids straight to the eyes • Discipline affords opportunities to detect rare, life altering and life threatening conditions • Cicatricial changes • Madarosis • Lack of cysts (nevi have cysts—melanoma doesn’t) • Lymph node enlargement
Sebaceous cell carcinoma • Mortality between 5-25% • Pagetoid spread—meanders between cells • Tumors <12mm not associated with distant metastases • Early detection if possible • Recurrent pterygia in same spot despite surgery: red flag • 3rd most common eyelid malignancy (1. basal 2. squamous) • Masquerades, can be difficult to diagnose
http://www.ijo.in/viewimage.asp?img=IndianJOphthalmol_2018_66_9_1235_239331_f2.jpghttp://www.ijo.in/viewimage.asp?img=IndianJOphthalmol_2018_66_9_1235_239331_f2.jpg https://eyecancer.com/eye-cancer/conditions/eyelid-tumors/sebaceous-carcinoma-eyelid/ https://www.aad.org/public/diseases/skin-cancer/sebaceous-carcinoma http://www.ijo.in/viewimage.asp?img=IndianJOphthalmol_2018_66_9_1235_239331_f2.jpg
Vascular tumors Capillary hemangioma: Consider Kasabach-Merritt PHACE Syndrome http://webeye.ophth.uiowa.edu/eyeforum/cases/57-Capillary-Hemangioma-Eyelid.htm https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/phaces-syndrome/
Fatality is rare with capillary hemangiomas on the eyelids—the hemangioma would have to be large enough to cause a consumptive coagulopathy/thrombocytopenia • Kasaback-Merrit: 35% mortality rate • PHACE Syndrome: usually non-fatal • But watch out for airway hemangiomas, arterial anomalies • Deserves imaging of head, neck, chest
Juvenile Cystinosis https://www.aao.org/image/cystinosis https://disorders.eyes.arizona.edu/disorders/cystinosis
Juvenile Cystinosis • Retinal crystals nephropathic • Kidney transplantation in 1st decade of life • likely to exhibit dwarfism • Not to be confused with Meesman, Lisch keratopathies
Multiple Myeloma https://www.thelancet.com/article/S0140-6736(12)60162-7/abstract
Multiple Myeloma • Life expectancy between 2-7 years depending on stage • Also consider: Monoclonal gammopathy of undetermined significance (MGUS)
Interstitial keratitis Leprosy Tuberculosis Syphilis https://emedicine.medscape.com/article/1213853-overview http://pubs.sciepub.com/ajmcr/4/5/4/index.html https://eyerounds.org/atlas/pages/syphilis-congenital-interstitial-keratitis.html
Interstitial Keratitis: Cogan Syndrome https://www.nejm.org/doi/full/10.1056/NEJMicm1709103 http://www.djo.harvard.edu/site.php?url=/physicians/cr/2197
Cogan syndrome • Bilateral hearing loss, vertigo, interstitial keratitis • Often with medium/large vessel vasculitis, myalgias, arthralgias • Interstitial keratitis: often concentric in mid-periphery with central sparing
Conjunctival tumors Benign melanosis (racial melanosis) Primary acquired melanosis https://www.willseye.org/disease_condition/conjunctival-primary-acquired-melanosis/ https://eyecancer.com/eye-cancer/conditions/conjunctival-tumors/pigmented-conjunctival-cancers-primary-acquired-melanosis/
PAM • 47% of PAM with atypia go on to become melanoma • More common in individuals with lighter pigmentation • May arise on palpebral conjunctiva—this is a more troublesome finding and is more likely to be missed • Small lesions (1-2 clock hours) without nodularity, vascularization or thickening may be observed annually. Photographs recommended. • >2 clock hours biopsy, cryotherapy of the margins
Melanoma Melanoma Melanoma Melanoma https://www.reviewofoptometry.com/article/a-guide-to-conjunctival-tumors
Conjunctival Melanoma • Risk factors for poorer outcome • Male • De novo lesion (didn’t arise from nevus or PAM) • Non-limbal location (examine fornices, caruncle) • Older age • 50% recurrence rate at 7 years • 5-17% mortality at 5 years • 9-35% mortality at 10 years
Ocular surface squamous neoplasia http://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/CIN-Conjunctival-intraepithelial-neoplasia.htm
OSSN vs Pterygium • Location: pterygium 3 & 9 o’clock • Pterygium: wing-shaped • Pattern of evolution (old photographs, previous examinations)
Ophthalmia Neonatorum https://emedicine.medscape.com/article/1192190-overview https://en.wikipedia.org/wiki/Neonatal_conjunctivitis
Ophthalmia Neonatorum • Causes: • Chemical (silver nitrate): day 1 • Gonococcal: days 3-5 • Chlamydial: days 7-14 • HSV: variable, usually latest • HSV, chlamydia pose highest mortality risk • Concomitant chlamydial pneumonia • Systemic HSV, neurological sequelae
62 yo ♂ w/ eye pain https://www.merckmanuals.com/professional/eye-disorders/conjunctival-and-scleral-disorders/scleritis
Scleritis • Potentially fatal conditions include: • Granulomatosis with polyangiitis • Polyarteritis nodosa • Inflammatory bowel disease • Tuberculosis • Lyme disease • Necrotizing anterior scleritis: likely to have underlying systemic inflammatory disease https://www.merckmanuals.com/professional/eye-disorders/conjunctival-and-scleral-disorders/scleritis
Scleritis • History: • Bloody noses/blood in urine? (GPA/Wegner) • Blood in stool/abdominal pain (Inflammatory bowel disease) • Tick bites? (Lyme) • Recent travel to developing countries? Visited a prison? (TB)
Lens • Cataracts: mortality by all causes has a slight decrease after cataract surgery • No surgery: mortality risk 2.78 per 100 person years • Surgery: mortality risk 2.98 per 100 person years
Refractive Error • The mortality benefit of the correction of refractive errors is so obvious that it will never be studied rigorously • No specialty contributes to the safety of society as much as optometry
Neuroblastoma http://www.ijdvl.com/viewimage.asp?img=ijdvl_2012_78_6_740_102370_f1.jpg
Neuroblastoma • Metastatic disease, usually from adrenal glands • ~20% of advanced neuroblastoma cases develop orbital metastases • Periorbital eccymoses are likely from obstructed palpebral blood vessels
Orbital cellulitis http://eyerounds.org/cases/103-Pediatric-Orbital-Cellulitis.htm
Orbital cellulitis • Look for sinusitis, subperiosteal abscess, diabetes, nasal black eschar http://eyerounds.org/cases/103-Pediatric-Orbital-Cellulitis.htm
Orbital cellulitis • Look for: rAPD, decreased vision, EOM changes, diplopia, proptosis, fever • Extension of sinus disease • In non-diabetics usually a single pathogen • In diabetics, often polymicrobial • Subperiostial abscesses often require surgical drainage
Retinoblastoma Image in public domain
Retinoblastoma • Inherited cases • 90% penetrance AD inheritance of RB1 • 45% chance of siblings being affected • Higher risk of bilateral disease, pinealoblastoma (trilateral RB), primitive neuroectodermal tumors (poor prognosis), osteosarcoma. • Sporadic cases • 85-90% of cases • Less likely bilateral • Less risk of other cancers
Choroidal melanoma https://www.sciencedirect.com/topics/medicine-and-dentistry/choroid-melanoma