W. Reid Cluff, OD AAO 2017 Case Report Abstract and Outline UAB School of

Title Clinical Diagnosis of in the Setting of Traumatic Brain Injury

Abstract This case highlights the importance of comprehensive examination of patients with visual complaints, regardless of previous diagnosis. A patient being treated for traumatic brain injury was found to have retinitis pigmentosa with cystoid macular edema.

I. Case History • Patient demographics o 31 year old, Hispanic, male • Chief complaint o Referred by brain injury rehabilitation clinic for vision examination secondary to complaints of chronic, constant blurry vision in both since concussive brain injury in 2012. Nothing seems to help the vision get better. He reported worse vision at night and difficulty reading for long periods of time due to headaches and strain. • Ocular and Medical History o No significant personal or family ocular history. He does not wear refractive correction of any kind. o Medical history is positive for traumatic brain injury (TBI) in 2012 and post- traumatic stress disorder (PTSD) in 2012 o Pertinent history: patient is a soldier in the US Army. • Medications o Trazodone, Naproxen, Lidocaine Patch, Venlafaxine XR, Prazosin, Cyclopenzaprine II. Pertinent Findings • Clinical Exam (initial visit) o Unaided distance visual acuity (DVA): OD 20/50+2, OS 20/60+2, PHNI OU o Unaided near visual acuity (NVA): OD 20/70, OS 20/40 o Preliminary testing unremarkable except confrontation visual fields (CVF), the patient notes blur in the periphery, especially temporally OU o Manifest refraction: ▪ OD: -0.50-1.25x175 20/20-2, NVA: 20/30+2 ▪ OS: -0.50-0.75x167 20/20-2, NVA: 20/30+2 o NRA/PRA: +2.00 / -1.00 o FCC: +1.25 o Accommodative amplitudes: 4.00 D OD/OS o Slit lamp biomicroscopy: Normal anterior segment OU o Dilated eye exam deferred to follow up visit. • Follow up visit (2 weeks later) o Interval history: the patient received new glasses and noted mild improvement in vision. He feels his vision is worse in the periphery and finds himself bumping into things, especially at night. He needs a lot of light to see where he is going. o Corrected DVA: OD 20/30+2, OS 20/30, PHNI OU o Corrected NVA: 20/25 OD/OS o CVF: Constricted temporal visual fields OD/OS o Slit lamp biomicroscopy: Normal anterior segment OU o Dilated exam: (+) PVD OD/OS, epiretinal membrane causing macular pucker OD/OS, mild arteriolar attenuation OD/OS, (+) “bone-spicule” pigment clumping seen in periphery 360 degrees OD/OS o OCT (Photo available) ▪ Cystoid macular edema (CME)seen in both eyes but worse in the right eye o Visual field (Photo available) ▪ Reliable visual fields taken OD and OS showing a generally constricted ring scotoma 360 degrees in both eyes, worse temporally. o Fundus Photos (Photo Available) ▪ Attenuated arterioles with significant pigment clumping in the periphery in both eyes. III. Differential Diagnosis • Retinitis Pigmentosa, epiretinal membrane, cystoid macular edema OU • Accommodative dysfunction, likely a sequela of TBI IV. Diagnosis and Discussion Retinitis pigmentosa (RP) with associated CME was diagnosed. RP is conventionally diagnosed based on the clinical triad of waxy pallor of the optic nerve, pigment clumping (or bone spicules) in the periphery, and attenuated retinal arterioles. Other common symptoms include difficulty seeing at night (nyctalopia), reduced peripheral vision, and blurry central vision. It can also present with or without cystoid macular edema (10-50% of cases) and some patients will develop posterior subcapsular (50%) leading to central vision loss. This patient had a history of TBI with presenting visual symptoms common to both conditions. V. Treatment and Management Currently, there is no effective treatment for retinitis pigmentosa. Patients who develop this disease typically continue to progress until they have a very small central island of vision with some experiencing complete vision loss. This patient was referred to where his diagnosis was confirmed with decreased full field electroretinogram (ERG) and decreased dark adaptometry. He was put on a combination steroid and NSAID treatment for the CME and continues to be followed in ophthalmology. VI. Conclusion Despite coming in with only symptoms of chronic blurry vision, it was significant that this patient was dilated and that a comprehensive examination was performed. Although his best corrected visual acuity was only slightly reduced, the reason for his chronic condition was discovered with a closer look at the fundus during dilated exam. This case highlights the importance of a comprehensive assessment of ocular health and persistence despite acceptable visual acuities. References Hartong, D., Berson, E., Dryja, T. Retinitis Pigmentosa. The Lancet. Nov 2006. 368: 1795-1809. Accessed online 17 Aug 2017. https://doi.org/10.1016/S0140-6736(06)69740-7. Strong, S., Liew, G., Michaelides, M. Retinitis Pigmentosa-associated Cystoid Macular Oedema: Pathogenesis and Avenues of Intervention. Br J Ophthalmol. Jan 2017. 101(1):31-37. Accessed online 17 Aug 2017. doi: 10.1136/bjophthalmol-2016-309376