Challenging Cases Panel Naval Aerospace Medical Institute (NAMI) Challenging Cases Panel

Disclosure Information Naval Aerospace Medical Institute Eye Department Terry Waggoner, OD LCDR Kyle Dohm, OD CAPT Matt Rings, MD CAPT Kevin McGowan, OD I have a financial interest in the Blind Spot Amsler Grid and the Waggoner Computerized Color Vision Test. Both helped support the diagnosis in this presentation. I will not discuss off-label use and/or investigational use in my presentation. The views expressed in this presentation are those of the presenter and do not necessarily reflect the official position of the Department of the Navy, Department of Defense, nor the U.S. government. May 2017NAMI Challenging Cases Panel 88th Annual Scientific Meeting of the Aerospace Medical Association

THIS PRESENTATION COVERS THE VISUAL IMPLICATIONS OF CENTRAL SEROUS RETINOPATHY (CSR) AND INCLUDES AN “ATYPICAL” CSR CASE REPORT Naval Aerospace Medical Institute Eye Department Terry Waggoner, OD LCDR Kyle Dohm, OD CAPT Matt Rings, MD CAPT Kevin McGowan, OD

Learning Objectives The audience will recognize the signs and symptoms of CSR. Become aware of CSR’s effect on visual acuity, it’s healing pattern, expected course duration, and likely prognosis. Learn of an “atypical” CSR case seen at NAMI and become familiar with the Navy’s waiver process relating to the case.

“Typical” Central Serous Retinopathy CSR is Leakage of fluid under the retina. It has a propensity to accumulate under the central macula resulting in the appearance of a “blister” which is seen with an ophthalmoscope and typically confirmed using optical coherence tomography (OCT). The result is a subject will normally start to experience blurred or distorted vision*. *The Upper right image shows an example of fluid under the retina seen with OCT. Lower left is the “Blister” seen with an ophthalmoscope and lower right the “Blister” demonstrated with a black and white photo.

CSR mostly affects white males in the age group 20 to 50 CSR mostly affects white males in the age group 20 to 50. Typically considered idiopathic, but the condition has been associated with increased stress levels and with corticosteroid, alcohol, and antibiotic use. Symptoms include: Decreased visual acuity Metamorphopsia ( which is visual distortion of straight lines, magnification and minification ) Skewed color vision perception Typically a central relative scotoma Vision loss can vary from mild to acutely reduced to 20/200 or worse. Severe vision loss is reported in 5% and recurrence up to 50% of the time in cases (usually within one year). Typically CSR is self-limiting after several weeks to months. Most patient’s have good visual recovery once the fluid starts to resolve and the eye heals. However, some visual abnormalities can remain even if visual acuity is measured at 20/20. Lasting problems include decreased night vision, reduced color discrimination, and localized distortion caused by scarring of the sub-retinal layers.

Now that you know what a typical case of CSR is, lets discuss the “Atypical” case seen at NAMI?

“Atypical” CSR Case 1. The subject is a Naval Aviation Water Survival Instructor. He works in a high risk training environment to include underwater egress training. 2. In 2007 he reported blurriness in his right eye, at which time he was diagnosed with CSR in his right eye. He had a second occurrence in 2011. Naval Aviation Water Survival Instructors must correct to 20/20 in one eye and no less than 20/40 in their worse eye, so he was not actually DQ'd for his VA. He was DQ’d due to retinal edema and a VF defect associated with the CSR. Both have remained since 2007. *As a past Vietnam Combat Pilot, I can confirm aviation can be a stressful environment. 4. He has a “congenital” moderate deutan (green) color vision defect and a suspected “acquired” mild tritan (blue) color vision defect. An “acquired” tritan defect is a symptom of CSR. It is important to note CSR is a relatively common clinical entity that affects military aviation and other operational personnel, possibly due to the high-stress nature of their jobs*.

Recurrence 2011 1. 2007 - A blister of subretinal fluid (SRF) formed causing a neurosensory retina detachment (NSD) in the macula of the right eye, just inferior of fovea. It resolved over a 3 months period. BCVA improved from 20/30 to 20/20. Frist CSR 2007 2. In 2011 CSR reoccurred. This time there was no blister and the neurosensory retinal detachment was temporal to the optic nerve head which is “atypical”. BCVA 20/20 Fundus drawings by Capt. O’ Connell Retinal Specialist 3. The 2014 Photo is similar to the 2011 drawing. BCVA 20/20 4. The 2016 Photo indicates overall improved state of healing/course/location. BCVA 20/20* 2016 2014 *Bottom right hand 2016 photo shows a decrease in the total area of subretinal fluid. Dr. Waggoner/ Dr. Dohm Dr. Waggoner

2011 In 2011, 2014, & 2016 the optical coherence tomography (OCT) images remained constant over a 5 year period. All three displayed a very thin area of subretinal fluid (indicated by the three red arrows) adjacent to the optic nerve head. The fovea was “not” a bullous blister.* Captain O’Connell 2014 2016 * You can see the fovea was flat in 2011 and 2014 (green arrows). It was recorded as flat in 2016 but is not included in the lower right 2016 OCT image. Dr. Waggoner Dr. Waggoner

Metamorphopsia was measured using the Blind Spot Amsler Grid in 2014 and 2016. No change was noted*. 2014 2016 *The patient subjectively drew the distorted areas he observed.

2014 Visual field testing using the Humphrey Visual Field Analyzer in 2011, 2014, 2016 indicated there was no change in the relative scotoma*. 2011 2014 2016 *In aviation, VF defects are considered disqualifying and important in any waiver process. Captain O’Connell Dr. Waggoner Dr. Dohm

The Waggoner Computerized Color Vision Test recorded the congenital red/green CVD remained unchanged in 2014 and 2016. The acquired blue CVD recorded in 2014 had resolved itself in 2016.* 2014 2016 *** *** Moderate Deutan & Mild Tritan Moderate Deutan Waggoner Waggoner

In this “Atypical" case , annual exams were required with a waiver approved (continued for Naval Aviation as a Water Survival Instructor dive/at the pool). The reason being: 1. The subject demonstrated he functioned normally as an NAWSI. 2. Visual acuity met standards for non-aircrew 3. He had stable OCT readings from 2011 to 2016. 4. Visual fields and Blind Spot Amsler Grid distortion were stable plus there was binocular over lap insuring safe performance of his duties*. 6. The “acquired” tritan color vision deficiency, which is a symptom of CSR, resolved itself. 7. Fundus photographs indicated an overall improved state of healing course/location. 8. Most importantly, there was no active/recurrent disease over a period of 5 years. *The scotoma surrounding the optic nerve in his right eye was covered by the normal field of view of his left eye.

Take Home Message: 1. Typical CSR is due to leakage of fluid under the retina. It has a propensity to occur under the macula resulting in blurred or distorted vision. Atypical cases “exist” where the subretinal fluid is not limited to the macula. 2. CSR effects mostly males in the age group of 20-50 and is believed to be exacerbated by stress, corticosteroid, alcohol, and/or antibiotic use. 3. Prognosis is normally good once the fluid starts to resolve, but even if visual acuity improves, there is often residual decreased night vision, reduced color discrimination, and localized distortion caused by scarring of the sub-retinal layers. 4. CSR is CD for aviation duty. Waivers may be “considered”, but not necessarily granted, once the vision returns to standards and there is no active/recurrent disease*. *My review of aero indicated the majority of CSR cases did receive a waive.

References Review of Optometry: The Handbook of Ocular Disease Management, 14 ed, 2012. https://www.reviewofoptometry.com/CMSDocuments/2012/6/ro0612_hndbk_em.pdf http://www.aao.org/eyenet/article/diagnosing-managing-central-serous-chorioretinopat https://www.reviewofophthalmology.com/article/cscr-diagnosis-and-treatment Recurrent CSR and Treatment with Avastin and Photo Dynamic Therapy. Poster. Jimenez, McGowan. American Acadamy of Optometry 2011 annual meeting. Dietrich KC. Fighter pilot with recurrent central serous chorioretinopathy. Aerosp Med Hum Perform. 2016; 87(10):901–905. http://emedicine.medscape.com/article/1227025-clinical Naval Aerospace Medical Institute. (2016). U.S. Navy aeromedical reference and waiver guide. Washington, DC: U.S. Navy.

Associations may include: Idiopathic “Type A” personalities / increased stress levels Corticosteroid use Hypertension Phosphodiesterase inhibitor use Obstructive sleep apnea Pregnancy SLE Systemic Lupus Erythematosus Alcohol use Allergic respiratory disorders Antibiotic use

Treatment Options for CSR No therapies are FDA-approved Certain retinal treatments may be employed for chronic/recalcitrant cases Anti-VEGF intravitreal injections (i.e. Avastin) Photodynamic Therapy (PDT) with verteporfin Focal laser photocoagulation (thermal) Transpupillary thermotherapy Oral therapy: Corticosteroid antagonists Adrenergic receptor antagonists Carbonic anhydrase inhibitors Rifampin