FUCHS' UVEITIS SYNDROME: NO LONGER a SYNDROME? Elke
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Academiejaar 2010 - 2011 FUCHS’ UVEITIS SYNDROME: NO LONGER A SYNDROME? Elke KREPS Promotor: Prof. Dr. Ph. Kestelyn Scriptie voorgedragen in de 2de Master in het kader van de opleiding tot MASTER IN DE GENEESKUNDE Acknowledgements I am very grateful to my supervisor, Professor Kestelyn, for his guidance and constructive criticism that enabled me to develop insight into the subject. I would also like to thank Dirk Desmet for his much appreciated linguistic assistance. Many thanks also go to Thierry Dervaux for his helpful comments on the first draft of this work and to Professor Van Maele for his statistical advice. Table of content ABSTRACT (ENGLISH) .................................................................................................................................................. 1 ABSTRACT (NEDERLANDS) .......................................................................................................................................... 2 1. INTRODUCTION ...................................................................................................................................................... 3 1.1 INTRODUCTION ............................................................................................................................................................. 3 1.2 MATERIALS AND METHODS .............................................................................................................................................. 4 2. CLINICAL FEATURES ................................................................................................................................................ 5 2.1 EPIDEMIOLOGY ............................................................................................................................................................. 5 2.2 TERMINOLOGY .............................................................................................................................................................. 5 2.3 CLINICAL SYMPTOMS AT PRESENTATION ............................................................................................................................. 6 2.4 CLINICAL SIGNS ............................................................................................................................................................. 6 2.4.1 Cornea ............................................................................................................................................................... 7 2.4.2 Anterior chamber .............................................................................................................................................. 7 2.4.3 Iris ...................................................................................................................................................................... 8 2.4.3.1 The normal iris ..............................................................................................................................................................8 2.4.3.2 Inflammation .................................................................................................................................................................8 2.4.3.3 Iris atrophy ....................................................................................................................................................................9 2.4.3.4 Heterochromia ..............................................................................................................................................................9 2.4.3.5 Bilateral cases..............................................................................................................................................................10 2.4.3.6 Iris nodules ..................................................................................................................................................................11 2.4.4 Anterior chamber angle .................................................................................................................................. 12 2.4.5 Lens ................................................................................................................................................................. 13 2.4.6 Vitreous cavity ................................................................................................................................................. 13 2.4.7 Fundus ............................................................................................................................................................. 14 2.5 DIAGNOSTIC CRITERIA ................................................................................................................................................... 14 3. COMPLICATIONS ....................................................................................................................................................16 3.1 CATARACT .................................................................................................................................................................. 16 3.1.1 Surgical procedure .......................................................................................................................................... 16 3.1.2 Postoperative complications ........................................................................................................................... 17 3.2 GLAUCOMA ................................................................................................................................................................ 19 3.2.1 Etiology of secondary glaucoma ..................................................................................................................... 19 3.2.2 Management................................................................................................................................................... 20 3.3 VITREOUS OPACITIES AND VITRECTOMY ............................................................................................................................ 21 4. TREATMENT AND PROGNOSIS ...............................................................................................................................21 5. IMMUNOLOGY AND PATHOGENESIS .....................................................................................................................22 5.1 ULTRASTRUCTURE OF THE IRIS ........................................................................................................................................ 22 5.2 CELLULAR COMPONENTS OF THE AQUEOUS ....................................................................................................................... 23 5.3 HUMORAL COMPONENTS OF THE AQUEOUS ...................................................................................................................... 23 5.4 CHARACTERISTICS OF INFLAMMATION .............................................................................................................................. 24 5.5 AUTO-IMMUNITY ......................................................................................................................................................... 24 6. ETIOLOGY ...............................................................................................................................................................25 6.1 FUCHS’ THEORY ........................................................................................................................................................... 25 6.2 THE ORTHOSYMPATHETIC THEORIES ................................................................................................................................. 25 6.2.1 Lesions of trophic fibers .................................................................................................................................. 25 6.2.2 Lesions of the orthosympathicus ..................................................................................................................... 26 6.2.3 Status dysraphicus ........................................................................................................................................... 27 6.2.4 Association with the syndrome of Parry-Romberg .......................................................................................... 27 6.3 INHERITANCE .............................................................................................................................................................. 28 6.4 VASCULAR THEORY ....................................................................................................................................................... 29 6.5 OCULAR TRAUMA ........................................................................................................................................................ 30 6.6 INFECTIOUS AGENTS ..................................................................................................................................................... 30 6.6.1 Toxoplasma gondii ........................................................................................................................................... 30 6.6.2 Herpes Simplex Virus (HSV) ............................................................................................................................. 33 6.6.3 Cytomegalovirus (CMV)..................................................................................................................................