KU EYE OptomCon CE Optometry Conference Friday, September 30, 2016 1:00 pm - 5:00 pm SPONSORED BY THE Department of Ophthalmology University of Kansas Medical Center PROGRAM AGENDA 1:00 – 2:00 p.m. Update on Pseudotumor Cerebri Syndrome – Thomas J. Whittaker, JD, MD, Professor, Department of Ophthalmology, University of Kansas Medical Center 2:00 – 4:00 p.m. Anterior Uveitis: Common Infectious and Non- Infections Etiologies – Dominick Opitz, O.D., F.A.A.O., Illinois College of Optometry, Chicago, IL 4:00 – 5:00 p.m. Detecting Functional Change in Progressing Glaucoma – Paul Munden, MD, Associate Professor, Department of Ophthalmology, University of Kansas Medical Center Financial Disclaimer • I have no financial interest in any matter Update on Pseudotumor Cerebri discussed in this presentation. Syndrome Thomas J. Whittaker MS JD MD Update on Pseudotumor Cerebri Revised Classification and Diagnostic Agenda Criteria for PTCS • Revised classification scheme • New Classification‐‐Umbrella Term “Pseudotumor Cerebri Syndrome”(PTCS) includes both • Revised diagnostic criteria – Primary Pseudotumor Cerebri – With/without presence of papilledema – Secondary Pseudotumor Cerebri – Opening pressure on LP—adults vs children • New criteria for diagnosis – “Same old/same old” modified Dandy criteria for cases with • Initial results of IIHTT—Idiopathic Intracranial papilledema Hypertension Treatment Trial – New criteria for cases without papilledema • – Diet and Diamox vs Diet and Placebo in treatment New terminology: no longer benign idiopathic intracranial hypertension of IIH Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children, D. Friedman, G. Liu, K. Digre, Neurology 81:1159‐1165 (2013). Classification: Secondary PTC Classification: Primary PTC • Cerebral venous abnormalities • Medications – Cerebral venous sinus thrombosis – Antibiotics: Tetracycline, – Bilateral jugular vein thrombosis or minocycline, doxycycline, nalidixic surgical ligation acid, sulfa drugs – Middle ear or mastoid infection – Vitamin A and retinoids: – Increased right heart Primary pseudotumor cerebri—same old/same old pressure/Superior vena cava Hypervitaminosis A, isotretinoin, all‐ syndrome trans retinoic acid for promyelocytic – “Idiopathic intracranial hypertension (IIH)” – Arteriovenous fistulas leukemia, excessive liver ingestion – Decreased CSF absorption from – Hormones: Human growth hormone, previous intracranial infection or thyroxine (in children), leuprorelin • Includes patients with obesity, recent weight subarachnoid hemorrhage acetate, levonorgestrel,(Norplant – Hypercoagulable states gain, polycystic ovarian syndrome, and thin system), anabolic steroids children • Medical Conditions – Withdrawal from chronic – Endocrine disorders corticosteroids – Meets modified Dandy criteria of papilledema, – Addison disease – Lithium – Hypoparathyroidism – Chlordecone normal neuroimaging, elevated intracranial – Hypercapnia – Sleep apnea pressure on LP, and normal CSF – Pickwickian syndrome – Anemia – Renal failure – Turner syndrome – Down syndrome Revised Diagnosis Criteria Without Papilledema Revised Criteria for Diagnosis A. Papilledema In the absence of papilledema, a diagnosis of pseudotumor cerebri syndrome B. Normal neurologic examination except for cranial nerve abnormalities can be made if: C. Neuroimaging: Normal brain parenchyma without evidence of 1. B–E from above are satisfied, and hydrocephalus, mass, or structural lesion and no abnormal meningeal 2. In addition the patient has a unilateral or bilateral abducens nerve enhancement on MRI, with and without gadolinium, for typical patients palsy (female and obese), and MRI, with and without gadolinium, and In the absence of papilledema or sixth nerve palsy, a diagnosis of magnetic resonance venography for others; if MRI is unavailable or pseudotumor cerebri syndrome can be suggested but not made if: contraindicated, contrast‐enhanced CT may be used 1. B–E from above are satisfied, and D. Normal CSF composition 2. In addition at least 3 of the following neuroimaging criteria are E. Elevated lumbar puncture opening pressure (>250 mm CSF in adults satisfied: and >280 mm CSF in children [250 mm CSF if the child is not sedated and – i. Empty sella not obese]) in a properly performed lumbar puncture – ii. Flattening of the posterior aspect of the globe – iii. Distention of the perioptic subarachnoid space with or without a A diagnosis of pseudotumor cerebri syndrome is definite if the patient fulfills criteria A–E. The diagnosis is considered probable if criteria A–D are met but the tortuous optic nerve measured CSF pressure is lower than specified for a definite diagnosis. – iv. Transverse venous sinus stenosis Initial results of the IIHTT IIHTT Initial Results Idiopathic Intracranial Hypertension Treatment Trial: a multicenter, double‐blind, randomized, placebo‐controlled • 38 sites in North America enrolled 161 women and 4 study of acetazolamide in subjects with mild visual loss. men from March 2010 to November 2012 with follow up ending June 2013. Subjects had to meet the modified Dandy criteria for IIH, be aged 18‐60, and have: • Randomized to supervised diet either with 1. reproducible mild visual loss (−2 to −7 dB perimetric mean acetazolamide or matching placebo deviation [PMD]), 2. bilateral papilledema, • Study drug‐‐acetazolamide 250 mg, two tabs twice a 3. elevated CSF opening pressure, day, with dosage increase of one tab/week up to 4 4. be untreated with regard to IIH, and grams daily 5. no secondary cause of increased intracranial pressure present. • Subjects evaluated at screening, baseline, and 1,2,3,4,5,and 6 months after baseline Study method details may be found in JAMA 311(16):1641‐51 (2014). Baseline Symptoms in IIHTT Baseline Visual Field Defects in the IIHTT Results: papilledema IIHTT improvement IIHTT results: PMD improvement WHAT HAVE WE LEARNED FROM THE IIHTT: 1. Acetazolamide in IIH patients with mild visual loss produces a modest improvement in PMD over six months, much greater with moderate to high grade papilledema. 2. Acetazolamide has its greatest effect on visual field function and papilledema in the first month of escalating dosage. 3. Acetazolamide‐plus‐diet patients lost twice as much weight as placebo‐plus‐diet patients. 4. Risk factors for treatment failure: presence of high grade papilledema, lower ETDRS visual acuity measures at baseline, being Caucasian male. Treatment with the maximally tolerated dosage of acetazolamide appears to substantially reduce the risk of reaching IIHTT criteria of treatment failure. 5. IIH patients on acetazolamide as the only diuretic do not need potassium supplementation. 6. Perimetry performance failures were common—Dr. Keltner’s “bad hair days”‐‐so repeat HVFs if it doesn’t make sense. 7. Perimetric mean deviation is an excellent measure for follow‐up. Summary • Classification scheme has changed: – Umbrella term is Pseudotumor Cerebri Syndrome Resources: – Good ol’ PTC is now IIH or Primary PTC Wall, M, McDermott, MP, Kieburtz, KD, et al. Effect of acetazolamide – Secondary PTC associated with various medications, on visual function in patients with idiopathic intracranial medical conditions and venous abnormalities hypertension and mild visual loss: the idiopathic intracranial • Diagnostic criteria changed a little hypertension treatment trial. JAMA. 2014;1641‐1651. – Good ol’ PTC‐‐same modified Dandy criteria Friedman, DI, McDermott, MP, Kieburtz, K, et al. The Idiopathic • Note opening pressure‐‐25 cm adults, 28 in children Intracranial Hypertension Treatment Trial: Design Considerations and Methods. J Neuroophthalmol. 2014. – PTCS without papilledema: different criteria require 6th nerve palsy or MRI abnormalities Wall, M, Kupersmith, MJ, Kieburtz, KD, et al. The Idiopathic Intracranial Hypertension Treatment Trial: Clinical Profile at Baseline. • IIHTT initial results JAMA Neurology. 2014. – Diamox works in larger doses, is safe, and well tolerated Keltner, JL, Johnson, CA, Cello, KE, et al. Baseline visual field findings – Risk for treatment failures in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). – Role for surgery/cerebral sinus stenosis stenting not yet Invest Ophthalmol Vis Sci. 2014; 55:3200‐3207. evaluated 9/26/2016 Disclosures Anterior Uveitis: Common Infectious and Non- •Shire • B+L (Valeant) Infections Etiologies • Allergan Dominick L. Opitz, O.D., F.A.A.O. • Glaukos Associate Professor of Optometry Senior Director, Ophthalmology Services and Practice Development Illinois College of Optometry 1 Outline • Classification – Anatomy – Clinical course – Histopathology – Etiology • Specific Conditions – Non-infectious CASES – Infectious – Other Clinical Approach to Uveitis History (symptoms) Physical exam (signs) Anatomical classification Associated factors Lab studies CLASSIFICATION OF UVEITIS Etiology Treatment 14 D. Opitz, O.D., F.A.A.O. 1 9/26/2016 Summary Anatomical Classification Based on… Classification • Anatomy • anterior uveitis (iritis, iridocyclitis, and • what part of the uveal tract is affected • Clinical Course anterior cyclitis) •Acute • intermediate uveitis (para planitis, • Chronic • Recurrent posterior cyclitis, and hyalitis) • Histopathology • posterior uveitis (focal, multifocal, or • Granulomatous diffuse choroiditis, chorioretinitis, retinitis, • Nongranulomatous • Etiology and neuroretinitis) • Infectious • panuveitis (anterior chamber, vitreous, • Noninfectious • Masquerades retina, and choroid) 2 Sub-classes of Anterior Uveitis: Why Localize