Gottfried O.H. Naumann, Leonard M. Holbach, Friedrich E. Kruse (Eds.)Applied Pathology for Ophthalmic Microsurgeons

G.O.H. Naumann, L.M. Holbach, F.E. Kruse (Eds.)Additional main authors: Claus Cursiefen, Ludwig M. Heindl,Antonia M. Joussen, Anselm Jünemann, Christian Y. Mardin,and Ursula Schlötzer-Schrehardt

Applied Pathology forOphthalmic Microsurgeons

With 317 figures in 1063 parts mostly in color and 108 tables

Gottfried O.H. Naumann, Prof. (emer.) Dr. med. Dr. hc. (mult.)Department of Ophthalmology, University of Erlangen-NürnbergSchwabachanlage 6, D-91054 Erlangen, Germany

Leonard M. Holbach, Prof. Dr. med.Department of Ophthalmology, University of Erlangen-NürnbergSchwabachanlage 6, D-91054 Erlangen, Germany

Friedrich E. Kruse, Prof. Dr. med.Department of Ophthalmology, University of Erlangen-NürnbergSchwabachanlage 6, D-91054 Erlangen, Germany

ISBN 978-3-540-24189-8 Springer-Verlag Berlin Heidelberg New York

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Preface

Interaction of Pathology and Ophthalmic Microsurgery

Ophthalmic pathology means the study of the symptoms and morphologic signs ofdiseases of the eye and ocular adnexae both biomicroscopically and in the laboratory.Precise definition of the phenotype is a prerequisite for correlation to the genotype,helping to design clinical studies. Spectacular progress is considered a hallmark ofboth conservative medicine and surgery today. This is driven by the application ofboth the methods of molecular biology and the new approaches in biotechnology.

Microsurgical magnification and miniaturization of instruments and non-me-chanical tools, e.g., lasers, have made possible procedures beyond our imagination afew years ago. They are characterized by small-access wounds into the eye and onlyminimal threshold trauma beyond the targeted intraocular tissue. In view of all theglittering advances of biotechnology, the details of critical anatomy morphology aretoo often neglected or forgotten although they are essential for the success of our mi-crosurgical manipulations.

This book is intended for ophthalmologists in training and also for mature eyephysicians. Hopefully, this should help them to avoid pre-, intra- and postoperativecomplications and make them more alert to explaining the risks to their patientsbefore surgery. It attempts to remind the ophthalmic microsurgeon that knowledgeof the minutiae of structures is a prerequisite to achieving the intended outcome. Mi-crosurgical landmarks need to be defined more precisely than in conventional oph-thalmic surgery without microscopic magnification. Awareness of the specific pa-thology is crucial for the appropriate indications and suitability of instrumentationto overcome the specific tissue resistance.

Finally, while biotechnology is always changing, the morphologic elements of theeye and ocular adnexae in health and disease remain essentially the same. However,wound healing, scars and complications are also modified by new microsurgical ap-proaches.

Our intention is not to compile a complete list of references but instead to give thereader a selection of textbooks and review articles that may be helpful for furtherstudy.

We have not concentrated on the details of instrumentation and surgical tech-niques. Both are developing continuously. As an exception the reader is referred to sec-tions on non-mechanical trephination penetrating keratoplasty with the excimer laseralong metal masks and direct surgery of the ciliary body (see Chapters 5.1 and 5.4).

Clinical findings including imaging by biomicroscopy, “biocytology”, ultra-sound, OCT, CT, MRI etc. of the phenotype – supported by knowledge of the “thirddimension” supplied by ophthalmic pathology – therefore are essential for patientcare. They present in vivo and in the laboratory the foundation for solving diagnosticand therapeutic challenges for the clinician and microsurgeon: Ophthalmic patholo-gy is also a clinical subspecialty.

We want to emphasize that demanding and complex “ophthalmic microsurgery isapplied ophthalmic pathology.” This is particularly true if intra- or postoperativecomplications need to be corrected or minimized.

Therefore we have tried to do the following:

1. Alert the ophthalmic microsurgeon to the critical surgical anatomy and patholo-gy, landmarks and potential complications before he or she plans and starts to dis-cuss the indications with the patient and then the procedure itself.

2. Emphasize respect for the structure of the eye and ocular adnexae and point outthe most vulnerable cell populations in the various microsurgical interventions.

3. Point out the peculiar and unique features of intraocular microsurgery in the“closed system” and “open eye,” focusing on acute ocular hypotony-paracentesiseffects and the influence on blood-ocular barriers.

4. Illustrate the consequences of the movement of the iris-lens diaphragm (“vis a ter-go”) in relation to the systemic arterial pressure in the open eye; also the signifi-cance of maintaining or restoring the anterior chamber at the end of all intraocularprocedures involving the anterior segment.

5. Show how knowledge of the specific pathology modifies microsurgical interven-tions. We mention here only a few examples and refer the reader to the relevantchapters.) Loss of Bowman’s layer – focal or diffuse – recognized before corneal incisions

or grafting makes a surplus of interrupted sutures advisable: because sutureloosening is more likely. Running sutures can then be particularly annoying,because if one loop becomes loose the entire suture and wound will be unsta-ble. Single loose interrupted sutures can be easily removed at the slit lamp – thearchitecture of the wound beyond one loose suture is usually not endangered.) Calcifications within Bowman’s layer in band degeneration of the cornea can

usually be chemically dissolved and do not justify sacrifice of this structure,e.g., by excimer laser.) Limbal stem cell insufficiency alerts one to serious ocular surface problems.) Avascular corneal lesions after herpes corneae may more likely harbor herpes

simplex-virus in the stroma, which may lead to recurrence after keratoplasty. Incontrast vascularized herpetic scars indicate an increased risk for postoperativeimmune reactions following corneal transplantation.) Granulomatous reaction against Descemet’s membrane in herpes corneae leads

to defects and signals imminent corneal perforation. This may be an argumentfor urgent curative perforating keratoplasty.) Tumors of the “iris root” de facto involve the anterior face of the ciliary body

and need to be treated by block excision of iris and ciliary body.) Cystic and diffuse epithelial ingrowth into the anterior chamber usually reach

the angle and cover the anterior surface of the ciliary body. Therefore “blockexcisions” are necessary including removal of the adjacent pars plicata of theciliary body, iris, cornea and sclera – acting as a “shell.” Only small epithelialimplantations in the central iris region can be cured by iridectomy if the angleis not involved.) Fibrous pseudo-metaplasia of the anterior subcapsular lens epithelium results

in mechanical properties such as sclera; it may divert the standard capsulorr-hexis around the anterior subcapsular cataract.) Pseudoexfoliation syndrome (PEX) is a generalized disease of the extracellular

matrix. It affects all tissues of the anterior segment of the eye. Although com-mon, it may very often be overlooked and may manifest asymmetrically but infact is always present bilaterally. It is not a harmless anomaly but a disease lead-ing in its advanced stages to potentially catastrophic complications.We need to consider not only the risk of secondary open angle and angle closureglaucomas, but also the zonular instability, blood-aqueous barrier breakdown,anterior segment hypoxia, poor mydriasis, and reduced mobility of the iris. Thevulnerability of the directly involved corneal endothelium contributes to the in-creased risk of corneal decompensation. To miss the diagnosis of PEX or disre-gard it may imply many unpleasant surprises or “recklessly running into a trap.”

VIII Preface

) Intraocular neovascularization commonly develops in the end stages of manyocular and systemic diseases. Until recently medical and laser therapy attempt-ed to eliminate angiogenic factors originating from hypoxic retina. The newcapillaries did not disappear but were less recognizable because of decreasedblood flow in the persisting vascular scaffold. A spectacular advance currentlyis evolving by new local application of specific inhibitors of vascular endotheli-al growth factors (VEGFs) in the therapy of these entities. The endothelial cap-illary sprouts can actually regress as long as a capillary basement membranehas not yet been formed.) Defining structures (“Leitstrukturen”) of the retina help to better interpret

optical coherence tomography (OCT), retinal thickness analysis (RTA) andfluorescence angiography to outline indications for microsurgery.) Uveal malignant melanomas almost always invade the sclera, although their

degree often is not detectable clinically – particularly if they are unpigmented.6. Not to forget: All cataract surgeons exert some tension on the lens capsule and

thus exert traction on the vitreous base via Wieger’s ligaments – but many are notreally aware of this in their daily routine. The peripheral retina also should beinspected before and after cataract surgery.

7. Features of normal and pathologic wound healing (“scar wars”) deserve attentionin the various tissues following mechanical or non-mechanical (laser, cryo-, dia-thermia coagulation) interventions in the closed and open eye constellations.

Beyond the practical implications these pages might also encourage our colleaguesto study the living eye and ocular adnexae – with a conscious knowledge of the thirddimension – and with enlightened curiosity and magnified attention.

We sincerely hope that our book might be helpful in the clinical care of patients.

Erlangen, Summer 2007 Gottfried O.H. NaumannLeonard Holbach

Friedrich E. Kruse

Interaction of Pathology and Ophthalmic Microsurgery IX

Acknowledgements

We would like to thank our numerous coworkers at the Departments of Ophthalmol-ogy of the Universities of Hamburg (1961–74), Tübingen (1975–80) and Erlangen(since 1980), who have made such a large contribution to this book. It is impossibleto mention them all.

Our colleagues from the European Ophthalmic Pathology Society (EOPS), theVerhoeff-Zimmerman Society and the German Speaking Ophthalmic Pathologists(DOP) and many ophthalmologists from around the world were a great source of in-spiration in the preparation of the book.

Frau Carmen Rummelt of the Ophthalmic Pathology Laboratory in Erlangenmade expert technical contributions to the cutting and staining of specimens for thefigures and helped with the digital microphotographs and SEM. The excellent workof our photographers, particularly H. Strahwald and M. Vogler in Erlangen, is evi-dent from their clinical pictures, and Jörg Kühn, Heidelberg, created some finegraphical work by modifying sketches mainly from previous publications by the au-thors, unless mentioned otherwise.

Most of the schematic drawings and tables are based on drafts by the First Editor/Author, who also supplied numerous microphotographs.

We are very grateful to Frau Marion Philipp and Frau Martina Himberger, Spring-er-Verlag, Heidelberg, for their encouragement and tolerance with unforeseeable de-lays; Mr. W. Shufflebotham, who with great patience transferred our teutonic versionof the manuscript into correct English; and Herr Joachim W. Schmidt, Munich, whocombined the text, figures, tables and references very efficiently into the book in itspresent form.

Frau Iris Schmitt not only typed many of the chapters but was essential in main-taining orderly communications during the long process of preparing the text andarranging tables, figures and literature of the manuscript. Her experience in prepar-ing books and publications since 1975 proved invaluable. Without her untiring sup-port this book would not yet be finished.

Finally, we are very grateful for the support of our partners and families for theirunderstanding and patience during the completion of this project.

X Acknowledgements

Condensed Table of Contents

1 IntroductionG.O.H. Naumann, Friedrich E. Kruse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

2 General Ophthalmic Pathology: Principal Indications and Complications, ComparingIntra- and Extraocular Surgery

G.O.H. Naumann, F.E. Kruse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7

3 Special Anatomy and Pathology in Surgery of the Eyelids, Lacrimal System,Orbit and Conjunctiva

L.M. Holbach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29

3.1 EyelidsL.M. Heindl, L.M. Holbach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30

3.2 Lacrimal Drainage SystemL.M. Heindl, A. Jünemann, L.M. Holbach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45

3.3 OrbitL.M. Holbach, L.M. Heindl, R.F. Guthoff . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

3.4 Conjunctiva and Limbus CorneaeC. Cursiefen, F.E. Kruse, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67

4 General Pathology for Intraocular Microsurgery: Direct Woundsand Indirect Distant Effects

G.O.H. Naumann, F.E. Kruse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76

5 Special Anatomy and Pathology in Intraocular Microsurgery

5.1 Cornea and LimbusC. Cursiefen, F.E. Kruse, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97

5.2 Glaucoma SurgeryA. Jünemann, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131

5.3 IrisG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152

5.4 Ciliary BodyG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176

5.5 Lens and Zonular FibersU. Schlötzer-Schrehardt, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . 217

5.6 Retina and VitreousA.M. Joussen and G.O.H. Naummanwith Contributions by S.E. Coupland, E.R. Tamm, B. Kirchhof, N. Bornfeld 255

5.7 Optic Nerve and Elschnig’s Scleral RingC.Y. Mardin, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335

6 Influence of Common Generalized Diseases on Intraocular MicrosurgeryG.O.H. Naumann, U. Schlötzer-Schrehardt . . . . . . . . . . . . . . . . . . . . . . . . . . 350

6.1 Diabetes MellitusG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351

6.2 Arterial Hypertension and “Vis A Tergo”G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353

6.3 Pseudoexfoliation Syndrome: Pathological Manifestations of Relevance toIntraocular Surgery

U. Schlötzer-Schrehardt, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . 354

6.4 Other Generalized DiseasesG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378

6.4.1 Infectious Disorders (AIDS, Sepsis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378

6.4.2 Hematologic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378

6.5.3 Neurologic and Muscular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378

General References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379

List of Figures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 381

List of Tables . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387

Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391

XII Condensed Table of Contents

Contents

1 IntroductionG.O.H. Naumann, F.E. Kruse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11.1 Ophthalmic Pathology in Clinical Practice, Teaching and Research . . . . . . 11.1.1 Confirmation and Quality Control of Clinical Diagnoses . . . . . . . . . . . . . . . 11.1.2 Modern Ophthalmomicrosurgery is Applied Ophthalmopathology . . . . . . 11.1.3 Ophthalmic Pathology is the Science of the Phenotype . . . . . . . . . . . . . . . . 11.1.4 Ophthalmic Pathology also Connects Experimental and Clinical

Ophthalmology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

1.2 Historical Sketch of Ophthalmic Surgery from Antiquity to ModernTimes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

1.3 Overview of Advances in Ophthalmic Pathology in the Nineteenthand Twentieth Centuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

2 General Ophthalmic Pathology: Principal Indications and Complications,Comparing Intra- and Extraocular Surgery

G.O.H. Naumann, F.E. Kruse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72.1 Principal Indications: Clinico-pathologic Correlation . . . . . . . . . . . . . . . . . 72.1.1 Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72.1.2 Excess of Tissue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82.1.3 Altered Tissue In Situ . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82.1.4 Displaced Tissue . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82.1.5 Neovascularization and Scars . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

2.2 Intraocular Compared with Extraocular Surgery: DistinguishingFeatures and Potential Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

2.2.1 Anterior Movement of the Iris-Lens Diaphragm (“Vis a Tergo”) . . . . . . . . 132.2.2 Paracentesis Effect . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 142.2.3 Expulsive Choroidal Hemorrhage and Uveal Effusion . . . . . . . . . . . . . . . . 172.2.4 Pupillary and Ciliary Block . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 172.2.5 Purulent Endophthalmitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182.2.6 Sympathetic Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192.2.7 Diffuse and Cystic Epithelial Ingrowth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192.2.8 Hemorrhage from Vasoproliferative Processes . . . . . . . . . . . . . . . . . . . . . . 212.2.9 “Toxic Anterior Segment Syndrome” (TASS) . . . . . . . . . . . . . . . . . . . . . . . . 222.2.10 “Intraoperative Floppy Iris Syndrome” (IFIS) . . . . . . . . . . . . . . . . . . . . . . . 22

2.3 Choice of Anesthesia and Knowledge of Ophthalmic Pathology . . . . . . . . 24

2.4 Instrumentation and Physical Principles 26References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

3 Special Anatomy and Pathology in Surgery of the Eyelids, Lacrimal System,Orbit and Conjunctiva

L.M. Holbach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29

3.1 EyelidsL.M. Heindl, L.M. Holbach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303.1.1 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303.1.1.1 Arterial Supply . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323.1.1.2 Venous Drainage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323.1.1.3 Lymphatic Drainage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323.1.1.4 Motor Nerve Supply . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323.1.1.5 Sensory Nerve Supply . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32

3.1.2 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 333.1.2.1 Disorders of Eyelid: Position and Movement . . . . . . . . . . . . . . . . . . . . . . 333.1.2.1.1 Surgical Pathology and Anatomic Principles of Ectropion Repair . . . . . 343.1.2.1.2 Surgical Pathology and Anatomic Principles of Entropion and

Distichiasis Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 373.1.2.1.3 Surgical Pathology and Anatomic Principles of Blepharoptosis Repair 393.1.2.2 Eyelid Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44

3.2 Lacrimal Drainage SystemL.M. Heindl, A. Jünemann, L.M. Holbach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453.2.1 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45

3.2.2 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46

3.2.3 Principles of Lacrimal Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473.2.3.1 External Dacryocystorhinostomy (with or without Silicone Tube

Intubation) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473.2.3.2 External Canaliculo-Dacryocystorhinostomy . . . . . . . . . . . . . . . . . . . . . . 473.2.3.3 External Dacryocystorhinostomy with Bypass Tube Insertion . . . . . . . 48References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48

3.3 OrbitL.M. Holbach, L.M. Heindl, R.F. Guthoff . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493.3.1 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

3.3.2 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 503.3.2.1 Orbital Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 503.3.2.2 Thyroid-Associated Orbitopathy – Endocrine Orbitopathy/

Ophthalmopathy – Graves’ Ophthalmopathy . . . . . . . . . . . . . . . . . . . . . . 62

3.3.3 Principles of Orbital Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 643.3.3.1 Surgical Approaches to the Orbit in Orbital Tumor Surgery . . . . . . . . . 643.3.3.1.1 Anterior Orbitotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 643.3.3.1.2 Lateral Orbitotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 653.3.3.2 Surgical Management in Thyroid Eye Disease . . . . . . . . . . . . . . . . . . . . . 653.3.3.2.1 Orbital Decompression . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 653.3.3.2.2 Extraocular Muscle Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 653.3.3.2.3 Eyelid Surgery to Prevent Lagophthalmus . . . . . . . . . . . . . . . . . . . . . . . . 653.3.3.3 Surgical Approaches in Anophthalmic Socket Surgery . . . . . . . . . . . . . . 653.3.3.3.1 Enucleation with Orbital Implants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 653.3.3.3.2 Evisceration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 663.3.3.3.3 Exenteration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66

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3.4 Conjunctiva and Limbus CorneaeC. Cursiefen, F.E. Kruse, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 673.4.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67

3.4.2 Surgical Anatomy, Landmarks, Nerve Supply, and Vascular Supplywith Blood Vessels and Lymphatics, Including Regional Lymph Nodesand Aqueous Episcleral Veins . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67

3.4.2.1 Limbal Stem Cells, Vogt’s Palisades, Cornea Verticillata, Tear Film . . . . 693.4.2.2 Langerhans Cells in the Limbal and Corneal Epithelium . . . . . . . . . . . . 693.4.2.3 Bowman’s Layer as Mechanical Barrier . . . . . . . . . . . . . . . . . . . . . . . . . . . 693.4.2.4 Keratocyte Distribution, Reaction to Trauma . . . . . . . . . . . . . . . . . . . . . . 693.4.2.5 Nerve Supply of Superficial Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 693.4.2.6 Anatomic Landmarks for Surgical Limbus: Edge of Bowman’s Layer,

Schlemm’s Canal, Scleral Spur . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69

3.4.3 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 693.4.3.1 Hereditary Anomalies of the Conjunctiva: Conjunctival

Lymphangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703.4.3.2 Conjunctival Inflammations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703.4.3.3 Sarcoidosis and Conjunctival Involvement in Systemic Disease . . . . . . 713.4.3.4 Pterygium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 723.4.3.5 Conjunctival Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 723.4.3.6 Conjunctival Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 723.4.3.6.1 Melanocytic Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 723.4.3.6.2 Acquired Epithelial Melanosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 723.4.3.7 Amniotic Membrane Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . 733.4.3.8 Limbal Stem Cell Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 733.4.3.9 Dry Eye . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 733.4.3.10 Superior Limbal Keratitis (Theodore) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 743.4.3.11 Ligneous Conjunctivitis (Conjunctivitis Lignosa) . . . . . . . . . . . . . . . . . . 74

3.4.4 Indications for Smear Cytology, Incisional or Excisional Biopsies,Autologous or Homologous Transplantation, Radiation, and Localand Systemic Chemotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74

3.4.4.1 Conjunctival Oncology: Melanocytic Processes . . . . . . . . . . . . . . . . . . . . 753.4.4.2 Limbus Stem Cell Insufficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 753.4.4.3 Amnion Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75

3.4.5 Wound Healing: Influence of Basic Disease and Adjunct Therapy(Radiation, Chemotherapy) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75

4 General Pathology for Intraocular Microsurgery: Direct Woundsand Indirect Distant Effects

G.O.H. Naumann, F.E. Kruse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 764.1. Access into the Eye: Principal Options and Anterior Segment Trauma 764.1.1 Direct Incisions and Wounds . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 764.1.1.1 Transcorneal Access . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 764.1.1.2 Access via Corneal “Limbus,” Fornix and Limbus Based Conjunctival

Flaps . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 864.1.1.3 Pars Plana Transscleral Approaches . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 904.1.2 Indirect Distant Effects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90

4.2 Obvious and Potential Compartments of the Intraocular Space . . . . . . 90

4.3 Variants of Intraocular Microsurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . 904.3.1 Wide Open Sky Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 904.3.2 Minimally Invasive Intraocular Microsurgery . . . . . . . . . . . . . . . . . . . . . 914.3.3 Intraocular Surgery Without Opening of the Eye Wall . . . . . . . . . . . . . 91

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4.4 Microsurgical Manipulations in the Anterior Chamber: CriticalStructures and Vulnerable Cell Populations . . . . . . . . . . . . . . . . . . . . . . 92

4.4.1 Free Access to Trabecular Meshwork in Open Angle and Free Flowin the Pupillary Zone and Between the Pars Plicata and Lens Equator . . 92

4.4.2 Corneal Endothelium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 924.4.3 Iris Microanatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 934.4.4 Lens Capsule and Anterior Zonular Insertion . . . . . . . . . . . . . . . . . . . . 93

4.5 Surgical Manipulation in the Vitreous Cavity: Critical Structuresand Vulnerable Cell Populations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93

4.5.1 Vitreous Attachments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 934.5.2 Sensory Retina, Retinal Pigment Epithelium and Optic Nerve Head 934.5.3 Posterior Lens Capsule and Posterior Zonula Insertion . . . . . . . . . . . . 944.5.4 Choroidal Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94

4.6 Role of the Size of the Eye . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94

4.7 Wound Healing After Intraocular Microsurgery and Trauma . . . . . . . 944.7.1 “Surgically Induced Necrotizing Scleritis” (SINS) . . . . . . . . . . . . . . . . . 964.7.2 Concept of a “Minimal Eye” . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 96

5 Special Anatomy and Pathology in Intraocular Microsurgery

5.1 Cornea and LimbusC. Cursiefen, F.E. Kruse, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . 975.1.1 Surgical Anatomy of the Cornea and Limbus . . . . . . . . . . . . . . . . . . . . 975.1.1.1 Corneal Epithelium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 975.1.1.2 Bowman’s Layer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 985.1.1.3 Corneal Stroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1005.1.1.4 Descemet’s Membrane . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1005.1.1.5 Endothelial Cells . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1015.1.1.6 Limbal Vascular Arcade . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1015.1.1.7 Tear Film . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1025.1.1.8 Anterior Chamber Associated Immune Deviation (ACAID),

Corneal Immune Privilege, Corneal Antiangiogenic Privilege . . . . . . . 1055.1.1.9 Limbal Epithelial and Corneal Stromal Stem Cells

U. Schlötzer-Schrehardt . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1075.1.1.10 Corneal Innervation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1085.1.1.11 Antigen Presenting Cells in the Cornea (Dendritic Cells and

Macrophages) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1085.1.1.12 Corneal Landmarks (Definition of Limbus) . . . . . . . . . . . . . . . . . . . . . . 1095.1.1.13 Corneal Dimensions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110

5.1.2 Surgical Pathology of the Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1105.1.2.1 Hereditary Diseases of the Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1105.1.2.1.1 Corneal Dystrophies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1105.1.2.1.2 Ectatic Disorders (Keratoconus, Keratoglobus, Keratotorus) . . . . . . . . 1135.1.2.2 Acquired Corneal Pathologies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1145.1.2.2.1 Degenerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1145.1.2.2.2 Corneal Neovascularization (Angiogenesis and Lymphangiogenesis) . . 1145.1.2.2.3 Neurotrophic Keratopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1165.1.2.2.4 Keratitis/Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1165.1.2.2.5 Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1185.1.2.2.6 Immune Reactions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119

5.1.3 Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1215.1.3.1 Penetrating Keratoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1215.1.3.1.1 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121

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5.1.3.1.2 Suturing Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1235.1.3.1.3 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1235.1.3.2 Lamellar Keratoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1235.1.3.3 Refractive Surgery and Phototherapeutic Keratectomy . . . . . . . . . . . . . 1255.1.3.4 Corneal Abrasion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1255.1.3.5 Amniotic Membrane Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . 1255.1.3.6 Stem Cell Transplantation and Donor Limbal Stem Cell Procurement . . 126

5.1.4 Wound Healing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1265.1.4.1 Epithelial-Stromal Interactions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1265.1.4.2 Epithelial Invasion (LASIK, Keratoplasty) . . . . . . . . . . . . . . . . . . . . . . . . 1275.1.4.3 Reinnervation After Penetrating Keratoplasty and Refractive

Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1275.1.4.4 Hem- and Lymphangiogenesis After Keratoplasty . . . . . . . . . . . . . . . . . 1275.1.4.5 Recurrence of Corneal Dystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1285.1.4.6 Replacement of Donor by Host Tissue After Keratoplasty . . . . . . . . . . 128References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128

5.2 Glaucoma SurgeryA. Jünemann, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1315.2.1 Principal Aspects of Glaucomas and Their Terminology . . . . . . . . . . . 131

5.2.2 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1315.2.2.1 Landmarks for Gonioscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1315.2.2.2 Landmarks for Surgical Corneal Limbus . . . . . . . . . . . . . . . . . . . . . . . . 135

5.2.3 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1365.2.3.1 Angle Closure Glaucomas (ACG) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1365.2.3.2 Open Angle Glaucomas (OAG) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1385.2.3.3 Secondary Open Angle Glaucomas (SOAG) . . . . . . . . . . . . . . . . . . . . . . 1385.2.3.4 Congenital Open Angle Glaucomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140

5.2.4 Indications and Contraindications for Microsurgery of Glaucomas . . 1435.2.4.1 YAG-Laser Iridotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1435.2.4.2 Mechanical Peripheral Iridotomy/Iridectomy . . . . . . . . . . . . . . . . . . . . 1435.2.4.3 Laser Trabeculoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1445.2.4.4 Transscleral Thermic Diode Laser . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1445.2.4.5 Mechanical Goniotomy and Trabeculotomy . . . . . . . . . . . . . . . . . . . . . . 1445.2.4.6 Procedures for Acute Secondary Open Angle Glaucomas . . . . . . . . . . . 1455.2.4.7 Filtrating Glaucoma Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1465.2.4.8 Concept of a Transtrabecular Shunt Between the Anterior Chamber

and Schlemm’s Canal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1485.2.4.9 Transscleral Coagulation of the Ciliary Body . . . . . . . . . . . . . . . . . . . . . 1485.2.4.10 Contraindication to Filtering Procedures . . . . . . . . . . . . . . . . . . . . . . . . 149

5.2.5 Complications with Excessive and Deficient Wound Healing . . . . . . . 1495.2.5.1 Acute Postoperative Decompensation of Intraocular Pressure . . . . . . . 1495.2.5.2 Late Conjunctival Bleb Wound Dehiscence . . . . . . . . . . . . . . . . . . . . . . . 1505.2.5.3 Failure of Goniotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1505.2.5.4 Failure of Filtering Surgery for Chronic Open Angle Glaucoma . . . . . 1505.2.5.5 Postoperative Peripheral Anterior Synechiae . . . . . . . . . . . . . . . . . . . . . 1505.2.5.5.1 Thermic Laser Trabeculoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1505.2.5.5.2 Subsequent Filtering Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1505.2.5.6 Consequences of Acute and Persistent Ocular Hypotony Following

Filtrating Glaucoma Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1515.2.5.7 Corneal Endothelial Proliferation and Migration After Filtrating

Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151

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5.3 IrisG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1525.3.1 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1535.3.1.1 Blood-Aqueous Barrier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1545.3.1.2 “Biocytology” of Normal Pigmented Cells of the Iris . . . . . . . . . . . . . . 155

5.3.2 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1565.3.2.1 “Biocytology” of Minimal Microsurgical Trauma . . . . . . . . . . . . . . . . . 1565.3.2.2 Melanin Dispersion from the Iris Pigment Epithelium . . . . . . . . . . . . . 1565.3.2.3 Rubeosis Iridis Followed by Secondary Open and Angle Closure

Glaucomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1565.3.2.4 Angle Closure Glaucomas Via Pupillary and Ciliary Block . . . . . . . . . . 1585.3.2.5 Iridodialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1595.3.2.6 Tumors of the Iris . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1615.3.2.7 Epithelial Ingrowth, Diffuse and Cystic . . . . . . . . . . . . . . . . . . . . . . . . . . 1645.3.2.8 Non-invasive In Vivo Diagnostic Procedures for Processes of the Iris . . 166

5.3.3 Indications for Surgical Procedures Involving the Iris . . . . . . . . . . . . . 1695.3.3.1 YAG-Laser Iridotomy and Iridoplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1695.3.3.2 Mechanical Iridotomy and Iridectomy . . . . . . . . . . . . . . . . . . . . . . . . . . 1705.3.3.3 Mechanical Mydriasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1715.3.3.4 Sector Iridectomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1715.3.3.5 Closure of Iridodialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1715.3.3.6 Localized Excision and Block Excisions of Iris Tumors and Epithelial

Implantation Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171

5.3.4 Wound Healing and Complications of Procedures Involving the Iris . . 1725.3.4.1 Iris Sutures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1725.3.4.2 Laser-Idirotomy and Hemorrhage from Iris . . . . . . . . . . . . . . . . . . . . . . 173References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175

5.4 Ciliary BodyG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1765.4.1 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178

5.4.2 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1825.4.2.1 Presbyopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1825.4.2.2 Cataract Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1825.4.2.3 Contusion Deformity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1835.4.2.4 Traumatic Cyclodialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1835.4.2.5 Pseudoadenomatous Hyperplasia (“Fuchs’ Adenoma”) . . . . . . . . . . . . 1865.4.2.6 Tumors of the Ciliary Body . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1865.4.2.7 Epithelial Ingrowth Involving the Anterior Chamber . . . . . . . . . . . . . . 1935.4.2.8 Zonular Apparatus in Pseudoexfoliation Syndrome and

Homocystinuria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1935.4.2.9 Non-invasive In Vivo Diagnostic Procedures . . . . . . . . . . . . . . . . . . . . . 193

5.4.3 Indications for Procedures Involving the Ciliary Body . . . . . . . . . . . . . 1935.4.3.1 Posterior Sclerotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1935.4.3.2 Pars Plana Vitrectomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1935.4.3.3 Direct Cyclopexy for Treating Persisting Ocular Hypotony Resulting

from Traumatic or Iatrogenic Cyclodialysis . . . . . . . . . . . . . . . . . . . . . . 1935.4.3.4 “Block Excision” of Tumors and Epithelial Ingrowth of Anterior Uvea . . 1955.4.3.4.1 Block Excision of Localized Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2045.4.3.4.2 Block Excision of Epithelial Ingrowth . . . . . . . . . . . . . . . . . . . . . . . . . . . 2045.4.3.5 Cyclodestruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213

5.4.4 Wound Healing and Complications After Procedures Involvingthe Ciliary Body . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215

XVIII Contents

5.4.4.1 Intraoperative Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2155.4.4.2 Local Recurrence and Metastasis of Tumors . . . . . . . . . . . . . . . . . . . . . . 2155.4.4.3 Prevention of Retinal Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2155.4.4.4 Corneal Endothelium Proliferation and Migration in Traumatic

Postcontusional Cyclodialysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2155.4.4.5 Ocular Hypotony After Block Excision for Tumors or Epithelial

Ingrowth . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2155.4.4.6 Relative Ocular Hypertension After Block Excision . . . . . . . . . . . . . . . 2155.4.4.7 Lens Decentration/Subluxation and Cataract Formation After Block

Excision . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216

5.5 Lens and Zonular FibersU. Schlötzer-Schrehardt, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . 2175.5.1 Key Features of the Lens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217

5.5.2 Basic Aspects of Intraocular Anatomy for Microsurgery of the Lens 2195.5.2.1 Position of the Lens and the Lens-Iris Diaphragm . . . . . . . . . . . . . . . . 2195.5.2.2 Characteristics of the Lens Capsule . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2195.5.2.3 Hyalocapsular Ligament (Wieger’s Ligament) . . . . . . . . . . . . . . . . . . . . 2195.5.2.4 Features of Lens Epithelial and Fiber Cells . . . . . . . . . . . . . . . . . . . . . . . 2205.5.2.5 Iatrogenic Mydriasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2205.5.2.6 Pseudophakic Lens Implants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220

5.5.3 Surgical Anatomy of the Lens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2205.5.3.1 Gross Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2205.5.3.2 Lens Capsule . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2215.5.3.3 Lens Epithelium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2225.5.3.4 Lens Fibers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2245.5.3.5 Suspensory Apparatus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2255.5.3.5.1 Zonular Fibers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2255.5.3.5.2 Wieger’s Ligament . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 226

5.5.4 Aging Changes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2265.5.4.1 Gross Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2265.5.4.2 Lens Capsule, Epithelium, Fibers, and Zonules . . . . . . . . . . . . . . . . . . . 2275.5.4.3 Biochemical Changes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 228

5.5.5 Surgical Pathology of the Lens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2295.5.5.1 Anomalies of Size and Shape . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2295.5.5.1.1 Aphakia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2295.5.5.1.2 Duplication of the Lens (Biphakia) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2295.5.5.1.3 Microspherophakia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2295.5.5.1.4 Lens Coloboma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2295.5.5.1.5 Lenticonus and Lentiglobus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2305.5.5.1.6 Persistent Hyperplastic Primary Vitreous . . . . . . . . . . . . . . . . . . . . . . . . 2305.5.5.2 Lens Dislocations (Ectopia Lentis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2325.5.5.2.1 Isolated Dislocation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2325.5.5.2.2 Systemic Diseases and Lens Dislocation . . . . . . . . . . . . . . . . . . . . . . . . . 2325.5.5.2.3 Traumatic Luxation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2345.5.5.3 Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2345.5.5.3.1 Basic Mechanisms of Cataract Formation . . . . . . . . . . . . . . . . . . . . . . . . 236

5.5.6 Basic Aspects of Cataract Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2445.5.6.1 Preoperative Examinations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2445.5.6.2 Indications for Removal of the Lens and Artificial Lens

Implantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2445.5.6.2.1 Extracapsular Cataract Extraction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2455.5.6.2.2 Intracapsular Cataract Extraction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 246

Contents XIX

5.5.7 Complications After Cataract Surgery and Wound Healing . . . . . . . . 2465.5.7.1 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2475.5.7.1.1 Corneal Endothelial Cell Loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2475.5.7.1.2 Iris Damage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2475.5.7.1.3 Retinal Damage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2475.5.7.1.4 Endophthalmitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2475.5.7.2 Secondary Cataract After Extracapsular Cataract Surgery . . . . . . . . . . 2475.5.7.2.1 Fibrosis-Type of Posterior Capsular Opacification (PCO) . . . . . . . . . . 2485.5.7.2.2 Pearl-Type PCO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2495.5.7.2.3 Soemmerring’s Ring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2495.5.7.2.4 Other Causes of PCO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2515.5.7.2.5 Prevention and Treatment of PCO . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2515.5.7.3 Pseudophakia and Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2515.5.7.3.1 True Pseudophakic Accommodation and Pseudoaccommodation . . . 2525.5.7.4 Complications After Intracapsular Cataract Extraction . . . . . . . . . . . . 252References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 252

5.6 Retina and VitreousA.M. Joussen and G.O.H. Naumannwith Contributions by S.E. Coupland, E.R. Tamm, B. Kirchhof, N. Bornfeld 2555.6.1 Surgical Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2555.6.1.1 Vitreous Attachments at the Base of the Ora Serrata

and Martegiani’s Ring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2555.6.1.2 Bursa Macularis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2565.6.1.3 Bergmeister Disc . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2565.6.1.4 Potential Subretinal Spaces: Foveolar and Ora Clefts . . . . . . . . . . . . . . 2565.6.1.4.1 Potential “Ora Cleft” . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2565.6.1.4.2 Potential “Foveola Cleft” . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2565.6.1.5 Horizontal Barriers (“Leitstrukturen”) . . . . . . . . . . . . . . . . . . . . . . . . . . 2575.6.1.6 Vertical Barriers (“Leitstrukturen”) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2575.6.1.7 Subretinal Immune Privilege . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 258

5.6.2 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2605.6.2.1 Peripheral Retinal Degenerations and Vitreous Traction . . . . . . . . . . . 2605.6.2.1.1 Lattice Degeneration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2605.6.2.1.2 Cystic Retinal Tuft . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2615.6.2.1.3 Peripheral Microcystoid Degeneration . . . . . . . . . . . . . . . . . . . . . . . . . . 2625.6.2.2 Peripheral Retinal Holes and Tears . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2635.6.2.3 Retinal Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2665.6.2.3.1 Effects of Detachment of the Ocular Tissues . . . . . . . . . . . . . . . . . . . . . . 2675.6.2.3.2 Histopathology of Rhegmatogenous Detachment . . . . . . . . . . . . . . . . . 2675.6.2.3.3 Exudative Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2685.6.2.3.4 Central Serous Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2685.6.2.3.5 Traction Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2695.6.2.3.6 Special Forms of Retinal Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . 2695.6.2.4 Retinoschisis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2705.6.2.4.1 X-Linked Retinoschisis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2725.6.2.5 Macular Hole . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2735.6.2.6 Vitreoschisis and Both Macular and Peripheral Pucker . . . . . . . . . . . . 2795.6.2.7 Retinal Vascular Abnormalities and Neovascularization . . . . . . . . . . . 2815.6.2.7.1 Central Retinal Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2875.6.2.7.2 Retinopathy of Prematurity (ROP) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2875.6.2.7.3 Hemoglobinopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2875.6.2.7.4 Diabetic Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2875.6.2.7.5 Radiation Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2895.6.2.7.6 Eales’ Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2895.6.2.7.7 Norrie’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289

XX Contents

5.6.2.7.8 Coats’ Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2905.6.2.7.9 Familial Exudative Vitreoretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . 2905.6.2.8 Choroidal Neovascularization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2915.6.2.9 Tumors of Choroid and Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2945.6.2.10 Dyes, Vitreous Substitutes and Infusion Fluids . . . . . . . . . . . . . . . . . . 2995.6.2.10.1 Dyes for Intravitreal Use . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2995.6.2.10.2 Vitreous Substitutes and Tamponades . . . . . . . . . . . . . . . . . . . . . . . . . 3005.6.2.10.3 Infusion Fluids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302

5.6.3 Principal Indications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3025.6.3.1 Closure of Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3025.6.3.1.1 Buckling or Scleral Indentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3035.6.3.1.2 Vitrectomy Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3035.6.3.2 Release of Vitreous Traction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3055.6.3.3 Removal of Internal Limiting Membrane . . . . . . . . . . . . . . . . . . . . . . . 3065.6.3.4 Removal of Vitreous Opacities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3075.6.3.5 Retinal Hypoxia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3105.6.3.6 Choroidal Neovascularization and Atrophy . . . . . . . . . . . . . . . . . . . . . 3115.6.3.7 Chorioretinal Biopsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3115.6.3.8 General Concepts in the Treatment of Tumors of Choroid

and Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3125.6.3.8.1 Controversies in the Management of Malignant Melanomas

of the Posterior Uvea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312

5.6.4 Wound Healing and Complications of Therapy . . . . . . . . . . . . . . . . . . 3145.6.4.1 Retinal Pigment Epithelium Scars . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3145.6.4.2 Proliferative Vitreoretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3145.6.4.3 Active Cell Populations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3165.6.4.3.1 Retinal Pigment Epithelial Cells . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3165.6.4.3.2 Hyalocytes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3175.6.4.3.3 Müller Cells and Their Functions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3175.6.4.3.4 Endothelial Cells, Astrocytes and Vascular Development . . . . . . . . . 3185.6.4.4 Distant Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3185.6.4.4.1 Nuclear Cataracts After Vitrectomy . . . . . . . . . . . . . . . . . . . . . . . . . . . 3185.6.4.4.2 Angle Closure Glaucoma After Vitreoretinal Surgery . . . . . . . . . . . . . 319References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320

5.7 Optic Nerve and Elschnig’s Scleral RingC.Y. Mardin, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3355.7.1 Anatomy, Landmarks, Harmless and Other Anomalies, Age Related

Changes, Juxtapapillary Coni . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3355.7.1.1 Anomalies of the Optic Disc Are Associated with the Disc’s Size . . . 337

5.7.2 Surgical Pathology and Indications for Microsurgery . . . . . . . . . . . . 3395.7.2.1 The Glaucomatous Optic Disc . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3395.7.2.1.1 Glaucomatous Disc Damage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3405.7.2.1.2 Juxtapapillary Glaucoma Damage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3405.7.2.2 Giant Cell Arteritis and Biopsy of the Temporal Artery . . . . . . . . . . . 3425.7.2.2.1 Biopsy and Histology of the Temporal Artery . . . . . . . . . . . . . . . . . . . 3425.7.2.3 Optic Disc in Non-/Ischemic Central Retinal Vein Occlusion

and Opticotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3435.7.2.3.1 Non-ischemic Central Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . 3435.7.2.3.2 Ischemic Central Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3435.7.2.4 Congenital Pits of the Optic Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3445.7.2.4.1 Vitreoretinal Microsurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3455.7.2.5 Tumors of the Optic Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3455.7.2.6 Intracranial Hypertension: Acute and Chronic Variants Must Be

Distinguished . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 346

Contents XXI

5.7.2.6.1 Acute Intracranial Pressure Rise and Terson’s Syndrome . . . . . . . . . . . 3465.7.2.6.2 Benign Chronic Intracranial Hypertension and Optic Disc Swelling

(Pseudotumor Cerebri) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 346

5.7.3 Wound Healing and Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3475.7.3.1 Pseudocavernous Optic Neuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3475.7.3.2 Radiation Opticoneuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 347References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348

6 Influence of Common Generalized Diseases on Intraocular MicrosurgeryG.O.H. Naumann, U. Schlötzer-Schrehardt . . . . . . . . . . . . . . . . . . . . . . . . . . 350

6.1 Diabetes MellitusG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3516.1.1 Diabetic Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3516.1.2 Diabetic Iridopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3516.1.3 Recurrent Corneal Erosions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3516.1.4 Cataracts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3516.1.5 Risk of Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 352

6.2 Arterial Hypertension and “Vis A Tergo”Gottfried O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353

6.3 Pseudoexfoliation Syndrome: Pathological Manifestations of Relevanceto Intraocular Surgery

U. Schlötzer-Schrehardt, G.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . 3546.3.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354

6.3.2 Pathobiology of PEX Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355

6.3.3 Clinical Diagnosis and Early Recognition . . . . . . . . . . . . . . . . . . . . . . . . 3576.3.3.1 Manifest PEX Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3576.3.3.2 Masked PEX Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3596.3.3.3 Early Stages of PEX Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3596.3.3.4 Asymmetry of Involvement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359

6.3.4 Surgical Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3606.3.4.1 Lens, Ciliary Body, and Zonular Apparatus . . . . . . . . . . . . . . . . . . . . . . 3606.3.4.2 Iris . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3646.3.4.3 Trabecular Meshwork . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3686.3.4.4 Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371

6.3.5 Microsurgical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3746.3.5.1 Intraoperative Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3746.3.5.2 Postoperative Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375

6.3.6 Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375

6.4 Other Generalized DiseasesG.O.H. Naumann . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3786.4.1 Infectious Disorders (AIDS, Sepsis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3786.4.2 Hematologic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3786.4.3 Neurologic and Muscular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378

General References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379

List of Figures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 381

List of Tables . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387

Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391

XXII Contents

List of Contributors

Norbert Bornfeld, Prof. Dr. med.Department of Ophthalmology, University of EssenHufelandstrasse 55, D-45122, Essen, Germany

Sarah E. Coupland, MBBS, PhDSchool of Cancer Studies, University of Liverpool5 049, Duncan Building, Daulby Street, LiverpoolL69 3GA, UK

Claus Cursiefen, Priv.-Doz. Dr. med.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Rudolf F. Guthoff, Prof. Dr. med.Department of Ophthalmology, Rostock UniversityDoberaner Strasse 140, D-18057 Rostock, Germany

Ludwig M. Heindl, Dr. med.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Leonard M. Holbach, Prof. Dr. med.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Antonia M. Joussen, Prof. Dr. med.Department of Ophthalmology, Heinrich-HeineUniversity, Moorenstrasse 5, 40225 DüsseldorfGermany

Anselm Jünemann, Prof. Dr. med.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Bernd Kirchhof, Prof. Dr. med.Department of Vitreoretinal Surgery, Center forOphthalmology, University of Cologne, Joseph-Stelzmann-Strasse 9, D-50931 Cologne, Germany

Friedrich E. Kruse, Prof. Dr. med.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Christian Y. Mardin, Prof. Dr. med.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Gottfried O.H. Naumann, Prof. (emer.) Dr. med. Dr. h.c. (mult.)Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Ursula Schlötzer-Schrehardt, Prof. Dr. rer. nat.Department of Ophthalmology, University ofErlangen-Nürnberg, Schwabachanlage 6D-91054 Erlangen, Germany

Ernst R. Tamm, Prof. Dr. med.Department of Anatomy, University of RegensburgUniversitätsstrasse 31, D-93053 Regensburg, Germany

List of Abbreviations

AMD Age related macula degenerationARMD Age related macula degenerationBAB Blood-aqueous barrierBM Bruch’s membraneBRB Blood-retina barrierCM Ciliary muscleCNTF Ciliary neurotrophic factorCNV Choroidal neovascularizationCPC Clinicopathologic correlationCRVO Central retinal vein occlusionEMZL Extranodal marginal zone B-cell lymphomaFEVR Familial exudative vitreoretinopathyFR Fuchs’ rollGFAP Glial fibrillary acid proteinHE Haematoxylin and EosinICG Indocyanine greenILM Inner limiting membraneIFIS Intraoperative floppy iris syndromeLASIK Laser in situ keratomileusisMLM Intermediate (middle) limiting membraneNHL Non-Hodgkin lymphomaNVD Neovascularization of the diskNVE Neovascularization elsewhereOCT Optical coherence tomographyOLM Outer limiting membrane

PAS Periodic Acid Schiff StainPCO Posterior Capsular OpacificationPDGF Platelet-derived growth factorPEDF Pigment epithelium-derived factorPKP Perforating keratoplastyPOAG Primary open angle glaucomaPVR Proliferative vitreoretinopathyRAM Relative anterior microphthalmusROP Retinopathy of prematurityROP Retinopathia of prematurityRPE Retinal pigment epitheliumRTA Retinal thickness analysisSC Schlemm’s canalSEM Scanning ElectromikroscopySINS Surgically induced necrotizing scleritisSL Schwalbe lineSOAG Secondary open angle glaucomaSS Sclera spurTASS Toxic anterior segment syndromeTM Trabecular meshworkTTT Transpupillary thermotherapyUSB Ultrasound biomicroscopyVEGF Vascular endothelial growth factorVIP Vasoactive intestinal polypeptide