Acute Progressive Vision Loss in an Adolescent

1. Acute Progressive Vision Loss in an Adolescent Adam Clarke, MD February 8, 2019

2. Patient Presentation CC Worsening blurry vision OU x 2 weeks HPI 12 yo F who presents with 2 weeks of worsening blurry vision OU, intermittent headaches and dizziness. Pt cannot recall exact onset of symptoms but became problematic enough to mention it to her parents approx. 1 week prior to presentation. Describes vision as “blurry” and denies photophobia, ocular pain, diplopia, VF loss. Denies history of recent illness, rash, fevers, trauma, parasthesias or focal weakness

3. History Past Ocular Hx – none Past Medical Hx – no significant hx, vaccinations up to date, term birth without complications Past Surgical Hx– tonsillectomy and adenoidectomy at 6 yrs of age Fam Hx- Noncontributory Meds – none Allergies - NKDA Social Hx– lives at home with 3 siblings and both parents

4. History 6 months prior - pt seen by optometrist in Bowling Green, KY - VA 20/20, 20/25 4 days prior - pt seen by optometrist - VA noted to be 20/70 OU - no abnormalities noted on exam, referred to ophtho Day of presentation - pt seen by ophthalmologist in Bowling Green - VA noted to be 20/100, 20/150 - HVF 30-2 performed, unreliable with high FP - no abnormalities noted on exam - sent to Norton Children’s Hospital for further workup

5. External Exam

6. Anterior Segment Exam

7. Posterior Segment Exam

8. Hospital Course Pt admitted by ED to neurology service for further workup MRI Brain and Orbits w/wo contrast - paranasal sinus disease, otherwise normal pre- and post-contrast MRI Stereopsis testing - stereo fly, 5/9 dots correct Discharged to KLEC for further imaging

9. Fundus Photo

10. Fundus Photo

11. Assessment • 12 yo F with 2 weeks of blurry vision and 4 day history of progressive bilateral vision loss • Differential Diagnosis • Functional Vision Loss • Acute Macular Neuroretinopathy • Multiple Evanescent White Dot Syndrome • Acute Retinal Pigment Epitheliitis (Krill’s) • Acute Posterior Multifocal Placoid (APMPPE)

12. SD-OCT Macula OD

13. SD-OCT Macula OD

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23. SD-OCT Macula OD

24. SD-OCT Macula OS

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37. SD-OCT Macula OS

38. OCT-A OD

39. OCT-A OS

40. Diagnosis • 12 yo F with 2 weeks of acute progressively worsening blurry vision with ONL hyperreflectivity and choriocapillaris flow void on OCT-A • Differential Diagnosis • Functional Vision Loss • Acute Macular Neuroretinopathy • Multiple Evanescent White Dot Syndrome • Acute Retinal Pigment Epitheliitis (Krill’s) • Acute Posterior Multifocal Placoid (APMPPE)

41. Diagnosis • 12 yo F with 2 weeks of acute progressively worsening blurry vision with ONL hyperreflectivity and choriocapillaris flow void on OCT-A • Differential Diagnosis • Functional Vision Loss • Acute Macular Neuroretinopathy • Multiple Evanescent White Dot Syndrome • Acute Retinal Pigment Epitheliitis (Krill’s) • Acute Posterior Multifocal Placoid (APMPPE)

42. Acute Macular Neuroretinopathy • First described in 1975 by Deutman and Bos • 4 cases of young women (25-33) on OCPs • As of today, ~140 publications exist on AMN

43. Acute Macular Neuroretinopathy

44. Acute Macular Neuroretinopathy • Epidemiology – limited data • 84% female • Mean age 29.5 (12-65)* • 80% Caucasian • Symptoms • Acute, often bilateral (54%) • Central scotoma (72%) • Decreased vision (16%) • Blurry vision (12%) • Metamorphopsia (3%)

45. Acute Macular Neuroretinopathy • Clinical Features • Red-brown petaloid/tear drop or wedge-shaped perifoveal lesions • Often delayed-onset in appearance (3-60 days) • VA 20/40 or better in 80% of eyes, 20/200 or worse in 6% • Pathogenesis • Possibly due to ischemia of deep retinal capillary plexus or choriocapillaris • Classification • Type 1 – also known as Paracentral Acute Middle Maculopathy (PAMM) • INL involvement • Now considered by many to be distinct disease • Type 2 – typical AMN also known as Acute Macular Outer Retinopathy (AMOR) • ONL involvement

46. Acute Macular Neuroretinopathy • Associated with: • Nonspecific flu-like or viral illness (47%) • Oral contraceptive pills (36%) • Vasopressors (8%) • Also: • Dengue fever • Checkpoint inhibitor therapy • Anemia, thrombocytopenia • Ulcerative colitis • Stimulant use

47. Acute Macular Neuroretinopathy • Diagnosis • IR imaging – hyporeflective, well-demarcated macular lesions • SD-OCT – ellipsoid zone disruption, hyper-reflectivity in ONL, thinning of ONL • OCT-A – flow voids in choriocapillaris, sometimes in deep capillary plexus • HVF 10-2 – sometimes identifies central or paracentral scotoma • FAF – usually unremarkable • FA, ICG – usually unremarkable • Full field and mfERG – usually unremarkable

48. Acute Macular Neuroretinopathy • IR Imaging • hyporeflective, well-demarcated macular lesions

49. Acute Macular Neuroretinopathy • SD-OCT • ellipsoid zone disruption, hyper-reflectivity in ONL, thinning of ONL

50. Acute Macular Neuroretinopathy • OCT-A • Choriocapillaris flow void often seen to correlate to shape of lesion on IR imaging