Learn Connect Succeed JCAHPO Regional Meetings 2015
OPTIC NEUROPATHY AS EASY AS 1,2,3,4 OPTIC NERVE ANATOMY M. Tariq Bhatti, MD Departments of Ophthalmology and Neurology Duke Eye Center and Duke University Medical Center OPTIC NEUROPATHY As Easy As 1, 2, 3, 4 OPTIC NERVE ANATOMY I. Optic Nerve a. Anatomy b. Physiology c. Vascular supply d. Clinical examination II. Case Studies III. Summary 1.2 million axons/nerve (1% subserve pupil) White matter tract covered by: Pia mater Arachnoid mater Dura mater Myelinated by oligodendrocytes at lamina cribosa Astrocytes (supporting cells) OPTIC NERVE ANATOMY OPTIC NERVE ANATOMY Photoreceptor Ganglion cell Axon 4 segments: intraocular (2 mm) intraorbital (20 30 mm) intracanalicular (6 10 mm) intracranial (3 16 mm) 1
OPTIC NERVE ANATOMY OPTIC NERVE PROJECTIONS 4 segments: intraocular (2 mm) intraorbital (20 30 mm) intracanalicular (6 10 mm) intracranial (3 16 mm) VISION PUPIL SACCADES/FIXATION CIRCADIAN RHYTHM VISUAL PATHWAY BLOOD SUPPLY OPTIC CHIASM ANATOMY OPTIC NERVE HEAD BLOOD SUPPLY OPTIC CHIASM Pre fixed chiasm Post fixed chiasm 2
Onset and Duration Progression (tempo) Description of visual loss Associated symptoms Medications Surgery Review of systems Visual acuity range: 20/15 NLP Levitan s Swinging Flash Light Test (Relative Afferent Pupillary Defect) 3
Visual Evoked Potentials (VEP) Electroretinogram (ERG) AMSLER GRID OPTIC NERVE VERSUS MACULA 4
Normal Optic Nerve Optic neuritis Traumatic optic neuropathy Compressive optic neuropathy Posterior ischemic optic neuropathy Radiation optic neuropathy Optic Nerve Pallor (on presentation) Compressive optic neuropathy Toxic/nutritional optic neuropathy Infectious (syphilitic) optic neuropathy Hereditary optic neuropathy Optic Nerve Edema Optic neuritis Anterior ischemic optic neuropathy Optic nerve glioma/optic nerve sheath meningioma Leber hereditary optic neuropathy (pseudoedema) Papilledema (often bilateral) 4 Responses: As Easy as 1, 2, 3, 4 1. Nothing 2. Cupping 3. Swelling 4. Palloring Associated Optic Nerve Findings Peripapillary hemorrhages: superficial (acquired) vs subretinal (congenital) Crowded fellow optic nerve (NA-AION) Anomalous blood vessel branching pattern (congenital) Optociliary shunt vessel (optic nerve tumor) Associated Retinal Findings Macular star (neuroretinitis) Choroidal folds (hypotony, orbital tumor) Retinal hemorrhages (CRVO) Retinitis, white dots, retinal scars (infection, inflammation) Associated Retinovascular Findings Cotton wool spots (GCA, hypertension, vasculitis) Embolus (carotid or cardiac disease) Peripapillary telangiectatic vessels (LHON) BOW TIE ATROPHY 5
OPTIC NEUROPATHY Elevated ICP Neoplastic (compressive) Inflammatory Infectious Vascular Metabolic/Toxic/Medication Hereditary Ocular Congenital Traumatic Ophthalmoscopic Features: PSEUDOPAPILLEDEMA COURTESY OF MAYS A. EL DAIRI, MD elevated disc: margins obscured absence of central cup anomalous branching pattern of blood vessels no obscuration of blood vessels SVP s present no exudates no cotton wool spots rare hemorrhages 6
OPTIC NERVE DRUSEN MALIGNANT HYPERTENSION (178/106) OPTIC NERVE DRUSEN TILTED OPTIC NERVE MYELINATED NERVE FIBER LAYER VITREORETINAL TRACTION 7
MORNING GLORY ANOMALY CASE STUDIES MENINGOENCEPHALOCELE OPTIC NERVE HYPOPLASIA Case 1: A 33 year old black female woke up with a blind spot in the vision in the right eye associated with pain with eye movements. Past medical history notable for aneurysm repair 4.5 years previously. On examination: Visual acuity: 20/25 both eyes Color vision: decreased right eye Pupils: right relative afferent pupillary defect Fundus exam: normal SEPTO OPTIC DYSPLASIA (DE MORSIER SYNDROME) OPTIC NERVE PIT Unsuccessful clipping of right supraclinoid internal carotid artery aneurysm, distal to the origin of the ophthalmic artery. Wrapped with muslin,coated with cyanoacrylate glue and subsequently embolized. COLOBOMA 8
Recalls visiting parents several weeks ago and playing with their new kittens. Mother diagnosed with cat scratch disease. Serology positive for Bartonella henselae. Eight days later: DIAGNOSIS: MUSLINOMA DIAGNOSIS: CAT SCRATCH DISEASE Case3: 61 year old Caucasian woman with 2 months of painless decreased vision OD. Vision 20/30. 20/70 VA < 20/50 PH 20/40 Pupils: RAPD OD 14 IOP < 14 no control Color Plates < 7.5/ 10 EOM: Full SLE: 1+NS OU Case 2: 31 year old woman suddenly noticed black spot in vision of left eye. No pain with eye movements. Brief viral illness 1 week previously. No past medical history. On examination: Visual acuity: right eye 20/20 left eye CF Color vision: right eye 11/11 left eye 0/11 Pupils: left RAPD 9
DIAGNOSIS: MALIGNANT OPTIC NERVE GLIOMA 10
Case 4: 41 yo monocular female developed headaches and 1 week later noticed blurred vision right eye with a pull like sensation. On Examination: visual acuity: right eye 4/200 color: right eye 1/11 slit lamp exam: normal motility: normal CSF, CxR, ACE, ANA, C ANCA, P ANCA, RPR, ESR, Bartonella and Lyme titers: all negative or normal. Admitted to hospital and received IV Solu Medrol. 1 week later: increase pain and decrease vision DIAGNOSIS: HZV ACUTE RETINAL NECROSIS (ARN) Case 5: 20 year old Caucasian woman with one day history of decreased vision in the left eye associated with ocular pain with eye movements. Past ocular, medical, surgical and neurological histories unremarkable. Medications: birth control pills. Review of systems: denies cat scratch or tick bite. Visual acuity: right eye 20/20 left eye 20/400 Color vision: right eye 11/11 left eye 0/11 Left relative afferent pupillary defect (Marcus Gunn pupil) Eye Movements: full Neurological exam: normal 11
Case 1: FOLLOW UP VISUAL FIELD DIAGNOSIS: ACUTE DEMYELINATING OPTIC NEURITIS Case 6: 39 yo black female noticed blurred vision right eye after grease splattered in eye 3 days previously. Vision continued to worsen for the next 3 weeks. No ocular pain. On examination: visual acuity: LP right eye color: 0 right eye Right RAPD 20/20 left eye 11/11 left eye slit lamp, motility: normal neuro exam: normal Visual acuity: 20/20 right eye 20/25 left eye Color vision: 11/11 right eye 8/11 left eye Subtle left afferent pupillary defect SIX WEEKS LATER MRI, syphilis serology, ANA, C ANCA, Bartonella and Lyme: all normal or negative ESR: elevated DIAGNOSIS: SARCOIDOSIS 12
SUMMARY OPTIC NEUROPATHY Case 7: Healthy 11 year old boy with one history of decrease vision right eye and pressure over right eye followed within several weeks with decrease vision left eye. Medications: none Family History: maternal uncle with visual loss in 20s Differential diagnosis extensive Requires detailed history, physical examination and paraclinical studies Elevated ICP (papilledema) Neoplastic (compressive) Inflammatory Infectious Vascular Metabolic/Toxic/Nutritional/Medication Hereditary Ocular Congenital Traumatic OPTIC NEUROPATHY As Easy as 1, 2, 3, 4, 5,6,7,8,9,10. Work up: MRI enhancement right optic nerve. Blood tests: positive toxoplasmosis titers Lumbar puncture: normal 3 days IV solumedrol with continued loss of vision DIAGNOSIS: LEBER HEREDITARY OPTIC NEUROPATHY THANK YOU 13