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Melanie Smith; 32 6/13/1978 Author: Colin M. O’Dea, MD Reviewer: Sharon Griswold, MD Case Title: Painful Monocular Visual Loss in a Young Adult Female Target Audience: Medical Students and Residents Primary Learning Objectives: 1. Verbalize the differential diagnosis for a patient who presents with painful visual loss 2. Perform components of a proper emergency department eye examination including visual acuity and IOP. 3. Demonstrate understanding of necessary examination findings sufficient to rule out acute angle-closure glaucoma. 4. Demonstrate an understanding of the imaging and treatment options for a patient that presents with optic neuritis. 5. Obtains appropriate consultation and/or referral to ophthalmology. Secondary Learning Objectives: detailed technical goals, behavioral goals, didactic points 1. Avoids delay in thorough examination of eye. 2. Demonstrates understanding that normal IOP with reactive pupils rules out acute angleclosure glaucoma. 3. Formulate plan for either inpatient care with urgent MRI and IV methylprednisolone therapy or outpatient workup in consultation with ophthalmology. Critical Actions Checklist 1. Completes thorough eye examination including visual acuity, IOP and attempted fundoscopic examination. 2. Consults ophthalmology. 3. Demonstrates ability to effectively communicate diagnosis of optic neuritis with the patient. Environment (if using as a simulation case) 1. Room Set Up – Emergency Department Room a. Manikin Set Up – Sim Man with Pupillary Response Capability b. Props i. Snellen Eye Chart ii. Tonopen iii. Slit lamp 2. Distractors – None Actors (optional) 1. Roles – Patient, nurse, ophthalmologist 2. Who May Play Them – Other residents, other students, actors 1 Melanie Smith; 32 6/13/1978 For Examiner Only Author: Colin M. O’Dea, MD Reviewer: Sharon Griswold, MD Case Title: Painful Monocular Visual Loss in a Young Adult Female CASE SUMMARY CORE CONTENT AREA Ophthalmology Neurology SYNOPSIS OF HISTORY/ Scenario Background This case is of a 34 year-old female with no known medical problems who presents to the ED with chief complaint of “I can’t see out of my right eye.” Symptoms occurred suddenly with rapid progression to current severity and occurred 30 minutes prior to arrival. The candidate should recognize the potential for permanent visual loss in this scenario, immediately perform appropriate physical examination of the eye including visual acuity with confrontation visual fields, Pupillary response, extra-ocular motion, measurement of intraocular pressure and fundoscopic examination, and consult ophthalmology to discuss the need for emergent MRI and IV steroid administration. SYNOPSIS OF PHYSICAL Initial scenario conditions: 1. Vitals: BP 146/78, HR 96, RR18, Temp 36.9, SpO2 100% 2. Physical Examination: Normal outside of ocular examination. Patient will show normal exam of the left eye with 20/25 vision, but an abnormal exam of the right eye with light perception only, a normal external examination, an afferent papillary defect, pain with extra-ocular motion and papilledema on fundoscopy. 2 Melanie Smith; 32 6/13/1978 For Examiner Only CRITICAL ACTIONS SCENARIO BRANCH POINTS/ PLAY OF CASE GUIDELINES Key teaching points or branch points that result in changes in patient’s condition 1. Critical Action – Check Visual Acuity This should be done very early in the patient interaction. When the patient states that she cannot see the Snellen chart, ability to see a waving hand and, ultimately, ability to perceive light should be tested. Cueing Guideline: Triage nurse can ask, “are they other methods of showing visual acuity if a patient cannot read an eye chart?”. 2. Critical Action – Complete Thorough Eye Examination The candidate should complete a thorough eye examination. The candidate should inspect the external eye, check visual fields, check pupillary response and evaluate for afferent pupillary defect by first checking each pupil individually and then swing a light between the two eyes, check extraocular motion, perform fundoscopy, check intraocular pressure using the tonopen, examine the eye using fluorescein and examination the eye with a slit lamp. Cueing Guideline: The nurse confederate may bring in the props of the slit lamp/ eye tray/ tonopen or other resources to prompt the learner. 3. Critical Action – Consult Ophthalmology The candidate should consult ophthalmology to discuss the need for MRI and IV steroids. While data is inconclusive regarding the necessity of either, the candidate should demonstrate the knowledge of appropriate outpatient imaging workup and the appropriate medical therapy. Cueing Guideline: Have the patient ask “what will be the next steps?” “Do I need any more tests today?” Consultant can provide scripted help for lower level learners. SCORING GUIDELINES/ CASE BRANCHPOINTS (Critical Action No.) 1. Completes thorough eye examination including visual acuity, visual fields, relative afferent papillary defect (RAPD), IOP and attempted fundoscopic examination. 3 Melanie Smith; 32 6/13/1978 a. A successful candidate must obtain the essential parts of the history and physical examination to rule out diagnoses such as acute angle closure that would require more immediate definitive therapy. A candidate should not pass the case without consideration of alternative diagnoses. 2. Consults ophthalmology. a. It is likely that ophthalmology consultation will occur via telephone. A successful candidate will discuss the case over the phone with the ophthalmologist and arrange for further care if discharged. 3. Demonstrates ability to effectively communicate diagnosis of optic neuritis with the patient. a. The patient may become more emotional during the ED visit and the provider should remain compassionate and answer the patient’s questions with empathy. 4 Melanie Smith; 32 6/13/1978 For Examiner Only HISTORY Onset of Symptoms: Symptoms occurred suddenly with rapid progression to current severity and occurred 30 minutes prior to arrival. Background Info: Patient was at home watching television when she began noticing pain in her right eye and that she was having trouble seeing out of her right eye. The symptoms progressed over only a few minutes to the degree to which they are currently. Her eye is more painful with movement. Chief Complaint: “I can’t see out of my right eye.” Past Medical Hx: None Past Surgical Hx: None Habits: Smoking: Denies ETOH: Socially Drugs: Denies Family Medical Hx: Mother with osteoporosis. Father with Hyperlipidemia. Social Hx: Marital Status: Married Children: Two Education: Bachelors Degree Employment: Teaches second grade ROS: Pertinent Positives: Right sided visual loss, pain in right eye, and pain in right eye worsening with extraocular motion ROS otherwise negative 5 Melanie Smith; 32 6/13/1978 For Examiner Only PHYSICAL EXAM Patient Name: Melanie Smith Age & Sex: 32 year old Female General Appearance: Well-developed, well-nourished female in moderate distress Vital Signs: BP 146/78, HR 96, RR18, Temp 36.9, SpO2 100% Head: Normal Eyes: Visual Acuity: OD: Able only to perceive light OS: 20/25 Visual Fields: OD: Unable to test OS: Normal External Eye Exam: Normal Pupils: Pupils equal, round, reactive to light, however, afferent pupillary defect is present. Extraocular Motion: Extraocular motion intact but considerable pain in right eye with extraocular motion. Fundoscopy: OD: Optic nerve photo, Note grade 2 papilledema, characterized by 360 degree nerve elevation without obscuration of the vessels OS: Normal IOP: 6 OD: 16, 18, 18 Melanie Smith; 32 6/13/1978 OS: 17, 18, 16 Fluorescein Exam: Normal Slit Lamp Exam: Normal Ears: Normal Mouth: Normal Neck: Normal Skin: Normal Chest: Normal Lungs: Normal Heart: Normal Back: Normal Abdomen: Normal Extremities: Normal Rectal: Not Performed Pelvic: Not Performed Neurological: Normal except for right eye Mental Status: Normal 7 Melanie Smith; 32 6/13/1978 For Examiner Only STIMULUS INVENTORY Suggested items as relevant to the case #1 Emergency Admitting Form #2 Results of Eye Examination #3 CBC #4 BMP #5 MRI Brain/Orbits 8 Melanie Smith; 32 6/13/1978 For Examiner Only LAB DATA & IMAGING RESULTS Learner Stimulus #2 Results of Eye Exam (Must Demonstrate Desire to Perform 6 of 9 Portions of Exam to Receive Learner Stimulus Sheet #2) See reference for photo citation http://webeye.ophth.uiowa.edu/eyeforum/cases/30-AcuteDemyelinatingEncephalomyelitis.htm. Retrieved February 1, 2011. Learner Stimulus #3 Intraocular Pressure: OD: 16, 18, 18 OS: 17, 18, 16 http://www.optivision2020.com/image-files/tono-pen-reichert-xl.jpg. Retrieved February 8, 2011. For Examiner Only 9 Melanie Smith; 32 6/13/1978 LAB DATA & IMAGING RESULTS Learner Stimulus #4 Complete Blood Count (CBC) WBC 6.8 /mm3 Hgb 12.6 g/dL Hct 37.4 % Platelets 212 /mm3 Differential Segs 75% Bands 0% Lymphs 20% Monos 4% Eos 1% Learner Stimulus #5 Basic Metabolic Profile (BMP) Na+ 140 mEq/L + K 4.0 mEq/L Cl109 mEq/L CO2 22 mEq/L Glucose 100 mg/dL BUN 15 mg/dL Creatinine 0.8 mg/dL 10 Melanie Smith; 32 6/13/1978 For Examiner Only LAB DATA & IMAGING RESULTS Learner Stimulus #6 MRI Brain/Orbits: MRI brain, axial T1 post-gadolinium. Enhancement of the right optic nerve is evident 11 Melanie Smith; 32 6/13/1978 Learner Stimulus #1 ABEM General Hospital Emergency Admitting Form Name: Melanie Smith Age: 32 years Sex: Female Method of Transportation: Private car Person giving information: Patient Presenting complaint: “I cannot see out of my right eye” Background: Patient was at home watching television when she began noticing pain in her right eye accompanied by an acute loss of vision. The symptoms started approximately 30 minutes ago and have progressed rapidly to the current severity. Triage or Initial Vital Signs BP: 146/78 HP: 96 RR: 18 Temp: 36.9 orally SpO2: 100% 12 Melanie Smith; 32 6/13/1978 Learner Stimulus #2 Fundoscopy: OD: OS: Normal 13 Melanie Smith; 32 6/13/1978 Learner Stimulus #3 Intraocular Pressure: OD: 16, 18, 18 OS: 17, 18, 16 http://www.optivision2020.com/image-files/tono-pen-reichert-xl.jpg. Retrieved February 8, 2011. 14 Melanie Smith; 32 6/13/1978 Learner Stimulus #4 Complete Blood Count (CBC) WBC 6.8 /mm3 Hgb 12.6 g/dL Hct 37.4 % Platelets 212 /mm3 Differential Segs 75% Bands 0% Lymphs 20% Monos 4% Eos 1% 15 Melanie Smith; 32 6/13/1978 Learner Stimulus #5 Basic Metabolic Profile (BMP) Na+ 140 mEq/L + K 4.0 mEq/L CO2 22 mEq/L Cl 109 mEq/L Glucose 100 mg/dL BUN 15 mg/dL Creatinine 0.8 mg/dL 16 Melanie Smith; 32 6/13/1978 Learner Stimulus #6 MRI Results: 17 Melanie Smith; 32 6/13/1978 Optional Addendum #1 Feedback/ Assessment Forms (may choose form dependent on use of case) Painful Monocular Visual Loss in a Young Adult Female Candidate ________________________ Examiner _________________________ Critical Actions: Critical Action #1 – Check Visual Acuity Critical Action #2 – Complete Thorough Eye Examination Critical Action #3 – Consult Ophthalmology Dangerous Actions: (Performance of one dangerous action results in failure of the case) Dangerous Action #1 – Failure to Check Visual Acuity Dangerous Action #2 – Failure to Check IOP Overall Score: Pass Fail 18 Melanie Smith; 32 6/13/1978 Optional Addendum 2: Core Competency Assessment Painful Monocular Visual Loss in a Young Adult Female Candidate ________________________ Examiner _________________________ Does Not Meet Expectations Patient Care Medical Knowledge Interpersonal Skills and Communication Professionalism Practice-based Learning and Improvement Systems-based Practice 19 Meets Expectations Exceeds Expectations Melanie Smith; 32 6/13/1978 For Examiner Date: _________ Examiner: _____________________ Examinee(s): ______________________ Scoring: In accordance with the Standardized Direct Observational Tool (SDOT) The learner should be scored (based on level of training) for each item above with one of the following: NI = Needs Improvement ME = Meets Expectations AE = Above Expectations NA= Not Assessed Critical Actions Obtained Visual Acuity Completed Eye Exam including IOP to Rule Out Acute Angle-Closure Glaucoma Obtained necessary consultation from Ophthalmology to develop ED and outpatient plan of care. Demonstrate / utilize effective communication techniques such as specifying order details and closed loop communication NI ME AE NA Category PC, MK, PBL PC, MK, PBL PC, MK, PBL P, ICS Category: One or more of the ACGME Core Competencies as defined in the SDOT PC= MK= PBL= ICS= P= 20 Patient Care Compassionate, appropriate, and effective for the treatment of health problems and the promotion of health Medical Knowledge Residents are expected to formulate an appropriate differential diagnosis with special attention to life-threatening conditions, demonstrate the ability to utilize available medical resources effectively, and apply this knowledge to clinical decision making Practice Based Learning & Improvement Involves investigation and evaluation of their own patient care, appraisal and assimilation of scientific evidence, and improvements in patient care Interpersonal Communication Skills Results in effective information exchange and teaming with patients, their families, and other health professionals Professionalism Melanie Smith; 32 6/13/1978 Manifested through a commitment to carrying out professional responsibilities, adherence to ethical principles, and sensitivity to a diverse patient population SBP= Systems Based Practice Manifested by actions that demonstrate an awareness of and responsiveness to the larger context and system of health care and the ability to effectively call on system resources to provide care that is of optimal value 21 Melanie Smith; 32 6/13/1978 Debriefing Materials: Please attach any powerpoint lectures, diagrams, images or other pertinent material the instructor may use for debriefing material. Debriefing Discussions: Emergency Department Eye Examination Afferent Pupillary Defect Painful Monocular Visual Loss Optic Neuritis Add 4-6 keywords for future searching functions Optic Neuritis Acute Angle-Closure Glaucoma Eye Examination Has this work been previously published? No 22 Melanie Smith; 32 6/13/1978 Debriefing Discussion: Emergency Department Eye Examination As this case illustrates, proper history regarding ocular complaints and proper examination of the eye are both essential in the management of patients who present to the emergency department with eye complaints. A proper history relating to ocular emergencies should, as always, begin with a history of present illness. Important aspects of HPI as related to ocular emergencies should always begin with questions of acuity, including general acuity, ability to differentiate color, loss of specific visual fields and, if pertinent, the pattern of acuity loss including a “curtain or veil”, tunneling of vision or central visual loss. Following questions of acuity should be questions regarding pain, either at rest or with extraocular movement, rate of progression of symptoms, any associated symptoms and circumstances surrounding onset of symptoms including trauma and environmental or chemical exposure. After the HPI past medical, surgical, family and social histories should be taken. It is important to remember that these histories should all be taken both generally and as specifically relates to the eye. While a history of diabetes or atrial fibrillation is very important to elucidate in a case such as this, past history of type of visual correction used, contact lens hygiene including leaving contacts in while sleeping, and past ocular surgeries is just as important. Following an appropriate history examination of the eye should begin. However, in the case of a potentially emergent eye complaint as in all potentially emergent complaints, it is important to consider taking only a very brief history before immediately continuing to examination of the eye so as not to delay necessary diagnosis and intervention. The components of an emergency department eye exam are as follows. While listed in a particular order, certain components are more or less important in the evaluation of different eye complaints and should be performed earlier or later in the evaluation of the eye depending on the complaint. 1. Visual Acuity: This should be done using a Snellen chart if available. If a patient is unable to read a Snellen chart, have the patient count fingers at the furthest possible distance. If the patient is unable to count fingers, check basic light perception. 2. External Eye: The external eye, lids and periorbital skin should be examined both grossly and, if appropriate, with fluorescein to evaluate for trauma, infection, deformity, crepitus or proptosis. If appropriate, the orbital rims should be palpated for step-offs. 3. Confrontation Visual Fields: Visual field testing should be performed in both eyes. Visual field defects can provide considerable information and can be particularly difficult to elicit from history as many patient describe visual field defects simply as “blurry.” 4. Pupils: Evaluate both pupils for reaction to both light and accommodation, both directly and consensually. 5. Ocular Motility: Extraocular motility should be checked bilaterally. 6. Anterior Segment: A slit-lamp should be used to evaluate the anterior segment of the eye to evaluate the conjunctiva, cornea, anterior chamber, iris, lens and ciliary body. The slit lamp can be used to visualize hemorrhage, deformity, discharge, inflammation, 23 Melanie Smith; 32 6/13/1978 foreign body, abrasions, dendrites, perforations, aqueous humor leakage, hyphema, hypopion, cell and flare. 7. Fundoscopic Examination: Fundoscopic examination is used to evaluate the optic nerve, macula and retina. Chemical dilation of the pupil aids in fundoscopic examination but is not often practical in the emergency department. Performing fundoscopic examination in a dark room often allows sufficient pupillary dilation to complete the exam. 8. Intraocular Pressure: Intraocular pressure should be checked using whichever tool is available. While several different tools may be available, most emergency departments now have tono-pens available and most tono-pen kits include instruction manuals for calibration and use. 24 Melanie Smith; 32 6/13/1978 Debriefing Discussion: Afferent Pupillary Defect An afferent pupillary defect exists when pupillary response to light in one eye is not as great as pupillary response to light in the other eye, or when pupillary response to light in one eye is lost entirely. This is tested using the “Swinging Flashlight Test” which is depicted here. These illustrations demonstrate an afferent pupillary defect of the left eye. A. Evaluate both pupils at baseline B. Light shined into right pupil causes bilateral pupillary constriction C. Swinging the light to the left pupil causes dilation of both pupils as the light reflex on the left is either diminished compared with the right or absent D. Swinging the light into right pupil causes bilateral constriction once again 25 Melanie Smith; 32 6/13/1978 Debriefing Discussion: Painful Monocular Visual Loss This case illustrates a presentation of undifferentiated painful monocular visual loss in the absence of trauma to the Emergency department. The differential diagnosis for visual loss is quite long, including globe rupture, retinal detachment, retrobulbar hematoma, acute angleclosure glaucoma, optic neuritis, central retinal vain occlusion, central retinal artery occlusion and temporal arteritis. Excluding very obscure etiologies and very late findings in infectious diseases such as orbital cellulitis, however, the differential diagnosis for painful monocular visual loss in the absence of trauma is only two things: acute angle-closure glaucoma vs. optic neuritis. Recognition of this differential is essential because of the need for emergent treatment for patient presenting with acute angle-closure glaucoma. The patient in this case presented with painful monocular visual loss due to optic neuritis. As you will learn, the treatment for optic neuritis is controversial with observation and outpatient follow up alone remaining a viable option. As such, the two most important aspect of the management of this case are the performance of a proper eye exam and the use of findings from the history and physical exam to rule out acute angle-closure glaucoma as the etiology of this patient’s complaint. Acute angle closure glaucoma occurs when dilation of the pupil blocks flow of the aqueous humor from the posterior chamber where it is produced by the ciliary body through the pupil to the anterior chamber where it drains through the Canal of Schlemm. The blockage of aqueous humor flow causes a build up of pressure in the eye. Excessive build-up of pressure has the potential to irreversibly damage the eye, causing permanent blindness. Diagnosis of angle-closure glaucoma is based on an appropriate history along with a mid-dilated nonreactive pupil and increased intraocular pressure on exam. Treatment for acute angle-closure glaucoma is aimed at decreasing IOP through the use of topical beta-blockers, topical alphaagonists, topical steroids, IV diamox and IV mannitol. During an acute attack, pressure-induced ischemia causes paralysis of the iris. Once the pressure is decreased, topical pilocarpine may be used to promote miosis and increase aqueous humor flow into the anterior chamber and out of the eye. In this case, the patient had reactive pupils, with an afferent pupillary defect, and normal IOP bilaterally. This information is sufficient to rule out acute angle-closure glaucoma as the etiology of the patient’s symptoms and to allow the clinician to focus on optic neuritis as the likely etiologic agent. 26 Melanie Smith; 32 6/13/1978 Debriefing Discussion: Optic Neuritis Optic neuritis is caused by demyelinating inflammation of the optic nerve and may occur alone or as a symptom of, and sometimes an initial presentation of, multiple sclerosis. It may also occur in the setting of adjacent infection involving the orbits or paranasal sinuses or in the setting of systemic viral illness. It occurs more commonly in Caucasians, affects women twice as often as men and typically presents initially between the ages of 20-45. Symptoms of optic neuritis include rapidly progressing painful visual loss in one or both eyes that is typically worse with extraocular movement. The visual loss may range from slightly blurry vision to complete visual loss, may include changes in color perception, may be exacerbated by heat and may include perception that objects moving in a straight line have a curved trajectory. On physical examination, patients’ with optic neuritis are found to have a decrease either in visual acuity or color perception, pain exacerbated by extraocular movement while extraocular movement remaining intact, an afferent pupillary defect and, in 1/3 of cases, papilledema present on fundoscopy. Aside from possible injection, the external eye exam should be normal, as should the anterior segment, the IOP and visual fields relative to one another. A red desaturation test, performed by having the patient stare at a dark red object with one eye and then testing the other eye to see if the object appears the same color, is often abnormal in optic neuritis. Laboratory studies are not particularly useful in the diagnosis of optic neuritis as most laboratory studies are typically found to be normal. Optic neuritis can be detected on MRI but other imaging modalities, like laboratory studies, are typically not of use. Treatment of optic neuritis was studied extensively in The Optic Neuritis Treatment Trial, a large, randomized study on the treatment of optic neuritis. While very informative, the study showed no statistically significant difference between high dose IV steroids and observation alone in terms of visual acuity at the five-year mark. The study did suggest that IV steroids may be slightly beneficial in the shorter term and may be slightly beneficial in keeping optic neuritis from progressing to multiple sclerosis, but these differences were small and remain somewhat controversial. The study did show, however, that both observation and high dose steroids lead to better outcomes than high dose oral steroids, which should be avoided. Being that observation without treatment is a reasonable course of action, the need for emergent MRI from the ER for definitive diagnosis is controversial. As such, the best approach is to consult an ophthalmologist and develop with the ophthalmologist a plan either for observation with outpatient follow up and outpatient MRI or MRI for definitive diagnosis in the ED and IV steroids. 27 Melanie Smith; 32 6/13/1978 References: Andreoli, T.E., et al (Eds.). Cecil Essentials of Medicine (6th ed). Philadelphia, PA: Saunders. Beck RW. The Optic Neuritis Treatment Trial. Arch Ophthalmol. 1988;106(8):10511053. Ergene, E, Machens, NA. (2009, July 30). Adult Optic Neuritis. Retrieved from eMedicine website: http://emedicine.medscape.com/article/1217083-overview Kumar, V., et al (Eds.). Robbins and Cotran Pathologic Basis of Disease (7th ed). Philadelphia, PA: Elsevier Saunders. Tantri, AP, Lee, AG. (2005, Feb 21). Acute Demyelinating Encephalomyelitis (ADEM) with associated optic neuritis: 9 year-old girl presents to an outside hospital with fatigue, poor appetite, and decreased activity for 3 weeks. Retrieved from EyeRounds.org website: http://webeye.ophth.uiowa.edu/eyeforum/cases/30AcuteDemyelinatingEncephalomyelitis.htm Tintinalli, J., et al (Eds.). Emergency Medicine A Comprehensive Study Guide (6th ed). New York, NY: McGraw Hill. 28