Grand Rounds Conference Eric Downing MD University of Louisville Department of Ophthalmology and Visual Sciences 11/7/2014

Subjective CC/HPI: 51M presents with general complaint of ↓VA over last few years. He would like new glasses.

History POH: none PMH: HTN, HLD, Arthritis Eye Meds: none Meds: Carvedilol, Pravastatin, Lisinopril, Alendronate, Glucosamine, Chondroiton Allergies: NKA

Objective OD OS OD OS VA: 20/20 ( x110 20/20- ( x080) Pupils: 4→3 4→3, +rAPD OD IOP:20 18 EOM: full OU

Objective OD OS OD OS VA: 20/20 ( x110 20/20- ( x080) Pupils: 4→3 4→3, +rAPD OD IOP:20 18 EOM: full OU

Objective PLE: ODOS E/L/Lmild MGDmild MGD C/SWhite, quiet OU K Clear OU AC Deep & quiet OU I/L Trace NS OU Vit WNL OU DFE:

Clinical photos

B Scan

OCT

HVF 30-2

Assessment 51M presents for regular exam and optic disc drusen found as ancillary finding causing asymmetric VF defects and an rAPD OD. 51M presents for regular exam and optic disc drusen found as ancillary finding causing asymmetric VF defects and an rAPD OD.

Plan Observe Observe

Optic Disc Drusen Common, usually bilateral, benign congenital anomaly of the optic nerve Common, usually bilateral, benign congenital anomaly of the optic nerve Calcified, laminated hyaline deposits characteristically found in the prelaminar region of the optic nerve head Calcified, laminated hyaline deposits characteristically found in the prelaminar region of the optic nerve head

Pathophysiology Unclear, but thought to be from axoplasmic transport alteration and/or axonal degeneration in the presence of small scleral canal Unclear, but thought to be from axoplasmic transport alteration and/or axonal degeneration in the presence of small scleral canal May be visible or buried May be visible or buried Higher incidence of VF loss associated with coexisting OHTN Higher incidence of VF loss associated with coexisting OHTN

Epidemiology Prevalence ranges from % of population Prevalence ranges from % of population Males/females equally affected, but rarely affects non-whites Males/females equally affected, but rarely affects non-whites 75-86% bilateral 75-86% bilateral Thought to have a dominant inheritance pattern with incomplete penetrance Thought to have a dominant inheritance pattern with incomplete penetrance

History & Exam Most patients do not experience symptoms, but roughly 8.6% may experience transient visual obscurations Most patients do not experience symptoms, but roughly 8.6% may experience transient visual obscurations Most common cause of pseudopapilledema (i.e. no disc hyperemia or vessel obscuration) Most common cause of pseudopapilledema (i.e. no disc hyperemia or vessel obscuration) Can cause flame hemorrhages, AION, or peripapillary subretinal neovascularization Can cause flame hemorrhages, AION, or peripapillary subretinal neovascularization NFB defects are found in 75-87% of patients NFB defects are found in 75-87% of patients rAPD may be present if asymmetric visual field loss rAPD may be present if asymmetric visual field loss

Testing B-scan B-scan Highly reflective with high echogenicity Highly reflective with high echogenicity OCT: decreased RNFL thickness in nasal quadrant OCT: decreased RNFL thickness in nasal quadrant Fluorescein Angiography/AF Fluorescein Angiography/AF ONHD display autofluorescence and do not show leakage ONHD display autofluorescence and do not show leakage CT scan/MRI CT scan/MRI

Autofluorescence FA OCT Optic Disc DrusenDisc edema

Treatment No proven treatment exists, but regular follow-up every 6-12 months with IOP monitoring and VF testing No proven treatment exists, but regular follow-up every 6-12 months with IOP monitoring and VF testing Radial optic neurotomy Radial optic neurotomy Glaucoma medications for elevated IOPs Glaucoma medications for elevated IOPs

Prospective, comparative, cross-sectional study Prospective, comparative, cross-sectional study 34 patients with visible or suspected ODD 34 patients with visible or suspected ODD 3 groups: definite, suspected, and normal-appearing optic nerves 3 groups: definite, suspected, and normal-appearing optic nerves Performed SD-OCT, EDI-OCT, B-scan, HVF on all eyes Performed SD-OCT, EDI-OCT, B-scan, HVF on all eyes ModeAll eyesSuspectedSuspected or normal EDI-OCT52/6817/2520/36 B-scan40/68(p=<0.001)7/25(p=0.002)8/36(p=<0.001)

EDI-OCT SD-OCT With increased detection rates and better ability to assess shape and structure, EDI- OCT may be superior to conventional tests With increased detection rates and better ability to assess shape and structure, EDI- OCT may be superior to conventional tests

References 1. BCSC. Neuro-Ophthalmology. Pp Tso MO. Pathology and pathogenesis of drusen of the optic nerve head. Ophthalmology. 88(1991). Pp Grippo TM, Shihadeh WA, Schargus M, et al. Optic nerve head drusen and visual field loss in normotensive and hypertensive eyes. J Glaucoma 2008;17: Haritoglou C, Priglinger SG, Grueterich M, Kampik A, Kriegelstein GK. Radial Optic neurotomy for the treatment of acute functional impairment associated with optic nerve drusen. Br J Ophthalmol. 2005;89: Merchant KY, Su D, Park SC, Qayum S, Banik R, Liebmann JM, et al. Enhanced Depth Imaging Optical Coherence Tomography of Optic Nerve Head Drusen. Ophthalmology. Mar