Ординатура / Офтальмология / Английские материалы / Applied Pathology for Ophthalmic Microsurgeons_Naumann, Holbach, Kruse_2008

5.1.4.5

Recurrence of Corneal Dystrophy

Since all surgical treatments available so far have not cured the diseased limbal epithelial stem cells themselves, recurrence of corneal dystrophies – especially the epithelial (and historically termed “stromal”) ones caused by mutations in the keratoepithelin gene – is not surprising. Recurrences are most common after surgery for granular, then lattice and least common after surgery for macular corneal dystrophy. Nevertheless, we recently observed recurrence of a macular corneal dystrophy 49 years after initial penetrating keratoplasty. The former can often be treated with laser ablation of early superficial recurrence.

5.1.4.6

Replacement of Donor by Host Tissue After Keratoplasty

In contrast to other solid tissue transplantations and more like hematological transplants, most components of a donor cornea are replaced by recipient cells after keratoplasty. Whereas both clinical and experimental studies agree that corneal epithelium is quickly replaced by host epithelium, the rate and extent by which corneal stroma and endothelium is replaced by host tissue is very variable and depends on the primary cause of keratoplasty. Nonetheless both experimental and human studies demonstrate cases of a complete replacement of donor tissue by host cells. In contrast, late immune reactions occurring more than 10 years after keratoplasty indicate that depending on the primary cause, only incomplete replacement of donor tissue occurs.

References

Bock F, Dietrich T, Zimmermann P, Onderka J, Baier M, Cursiefen C. Antiangiogene Therapie am vorderen Augenabschnitt. Ophthalmologe 2007; 104:336 – 44

Bock F, Onderka J, Zahn G, Dietrich T, Bachmann B, Kruse FE, Cursiefen C. Bevacizumab is a potent inhibitor of inflammatory corneal angiogenesis and lymphangiogenesis. Invest Ophthalmol Vis Sci. 2007; 48:2545 – 52

Braun M, Holbach L, Naumann GOH. Die korneale Lipofuszinose – klinisch-pathologische Untersuchung von zehn Patienten. Klin Monatsbl Augenheilkd 1997; 210:121 – 123

Chen JJ, Tseng SC, 1991. Abnormal corneal epithelial wound healing in partial-thickness removal of limbal epithelium. Invest. Ophthalmol. Vis. Sci. 32, 2219 – 2233

Chen L, Hamrah P, Cursiefen C, Jackson D, Streilein JW, Dana MR. Vascular Endothelial Growth Factor Receptor-3 (VEGFR-3) mediates dendritic cell migration to lymph nodes and induction of immunity to corneal transplants. Nature Medicine 2004; 10:813 – 5

Chen Z, de Paiva CS, Luo L, Kretzer FL, Pflugfelder SC, Li D-Q, 2004. Characterization of putative stem cell phenotype in human limbal epithelia. Stem Cells 22, 355 – 366

Cursiefen C, Küchle M, Naumann GOH. Angiogenesis in cor-

neal diseases: Histopathology of 254 human corneal buttons with neovascularization. Cornea 1998; 17: 611 – 613

Cursiefen C, Küchle M, Naumann GOH. Changing indications for penetrating keratoplasty: Histopathology of 1250 corneal buttons. Cornea 1998; 17: 468 – 470

Cursiefen C, Hofmann-Rummelt C, Schlötzer-Schrehardt U, Fischer DC, Küchle M. Immunphänotypische Klassifizierung der makulären Hornhautdystrophie: Erstbeschreibung des Immunphänotyps I A außerhalb Saudi-Arabiens. Klin Monatsbl Augenheilkd 2000; 217: 118 – 126

Cursiefen C, Rummelt C, Küchle M. Immunohistochemical localization of VEGF, TGF [ and TGF q 1 in human corneas with neovascularization. Cornea 2000; 19: 526 – 533

Cursiefen C, Schlötzer-Schrehardt U, Holbach M, Vieth M, Kuchelmeister K, Stolte M. Ocular findings in Fryns syndrome. Acta Ophthalmol Scand 2000; 78: 710 – 713

Cursiefen C, Hofmann-Rummelt C, Schlötzer-Schrehardt U, Fischer D-C, Haubeck H-D, Küchle M, Naumann GOH. Immunohistochemical classification of primary and recurrent macular corneal dystrophy in Germany. Subclassification of immunophenotype I A using a novel keratan sulfate antibody. Exp Eye Res 2001; 73: 593 – 600

Cursiefen C, Wenkel H, Martus P, Langenbucher A, Seitz B, Nguyen N, Küchle M, Naumann GOH. Peripheral corneal neovascularization after non-high risk-keratoplasty: influence of shortversus longtime topical steroids. Graefe’s Arch Clin Exp Ophthalmol 2001; 239: 514 – 521

Cursiefen C, Martus P, Nguyen NX, Langenbucher A, Seitz B, Küchle M. Corneal neovascularization after nonmechanical versus mechanical corneal trephination for non-high-risk keratoplasty. Cornea 2002; 21: 648 – 652

Cursiefen C, Schlötzer-Schrehardt U, Küchle M, Sorokin L, Breitender-Geleff S, Alitalo K, Jackson D. Lymphatic vessels in vascularized human corneas: immunohistochemical investigation using LYVE-1 and Podoplanin. Invest Ophthalmol Vis Sci 2002; 43: 2127 – 2135

Cursiefen C, Chen L, Dana MR, Streilein JW. Corneal lymphangiogenesis: Evidence, mechanisms and implications for transplant immunology. Cornea 2003; 22: 273 – 81

Cursiefen C, Seitz B, Dana MR, Streilein JW. Angiogenese und Lymphangiogenese in der Hornhaut: Pathogenese, Klinik und Therapieoptionen. Ophthalmologe 2003; 100:292 – 9

Cursiefen C, Rummelt C, Küchle M, Schlötzer-Schrehardt U. Pericyte recruitment in human corneal angiogenesis. Br J Ophthalmol 2003; 87: 101 – 106

Cursiefen C, Chen L, Borges L, Jackson D, D’Amore PA, Dana MR, Wiegand SJ, Streilein JW. Via bone marrow-derived macrophages, VEGF A mediates lymphand hemangiogenesis in inflammatory neovascularization. J Clin Investigation 2004; 113:1040 – 50

Cursiefen C, Maruyama K, Liu Y, Chen L, Jackson D, Wiegand S, Dana MR, Streilein JW. Inhibition of hemangiogenesis and lymphangiogenesis after normal-risk corneal transplantation by neutralizing VEGF promotes graft survival. Invest Ophthalmol Vis Sci 2004;45:2666 – 73.

Cursiefen C, Masli S, Ng TF, Dana MR, Bornstein P, Lawler J, Streilein JW. Roles of thrombospondin 1 and 2 in regulating spontaneous and induced angiogenesis in the cornea and iris. Invest Ophthalmol Vis Sci 2004; 45:1117 – 24

Cursiefen C, Ikeda S, Nishina PM, Smith RS, Ikeda A, Jackson D, Mo JS, Chen L, Dana RM, Pytowski B, Kruse FE, Streilein JW. Spontaneous corneal hemand lymphangiogenesis in mice with destrin-mutation depend on VEGFR3-signaling. Am J Pathol 2005; 166: 1367 – 1377

Cursiefen C, Seitz B, Kruse FE. Neurotrophe Keratopathie: Pathogenese, Klinik und Therapie. Ophthalmologe 2005;102: 7 – 14

Cursiefen C, Seitz B, Kruse FE. 100 Jahre Hornhauttrabsplanta-

tion. Eine Erfolgsgeschichte mit Zukunft. Deutsches Ärzteblatt 2005:45: Seite A–3078

Cursiefen C, Chen L, Saint-Geniez M, Hamrah P, Jin Y, Rashid S, Pytowski B, Streilein JW, Dana MR. Nonvascular VEGF receptor 3 expression by corneal epithelium maintains avascularity and vision. Proc Natl Acad Sci U S A. 2006; 103: 11405 – 10

Cursiefen C, Maruyama M, Jackson DG, Streilein W, Kruse FE. Time-course of angiogenesis and lymphangiogenesis after brief corneal inflammation. Cornea 2006;25:443 – 7

Cursiefen C, Rummelt C, Neuhuber W, Kruse FE, Schroedl F. Absence of blood and lymphatic vessels in the developing human cornea. Cornea 2006; 25:722 – 6

Cursiefen C, Rummelt C, Kruse FE. Amniotic membrane covered bi-onlay. Br J Ophthalmol 2007; 91:841 – 2

Daniels JT, Dart JKG, Tuft SJ, Khaw PT, 2001. Corneal stem cells in review. Wound Rep. Reg. 9, 483 – 494

Davanger M, Evensen A, 1971. Role of the pericorneal papillary structure in renewal of corneal epithelium. Nature 229, 560 – 561

Dua HS, Azuara-Blanco A, 2000. Limbal stem cells of the corneal epithelium. Surv. Ophthalmol. 44, 415 – 425

Dua HS, Joseph A, Shanmuganathan VA, Jones RE, 2003. Stem cell differentiation and the effects of deficiency. Eye 17, 877 – 885

Goldberg MF, Bron AJ, 1982. Limbal palisades of Vogt. Trans. Am. Ophthalmol. Soc. 80, 155 – 171

Gottschalk K, Rummelt C, Cursiefen C. Therapierefraktäre Bindehautchemosis. Klin Monatsbl Augenheilkd 2006; 223: 696 – 8

Grueterich M, Espana EM, Touhami A, Ti SE, Tseng SC, 2002. Phenotypic study of a case with successful transplantation of ex vivo expanded human limbal epithelium for unilateral total limbal stem cell deficiency. Ophthalmology 109, 1547 – 1552

Grueterich M, Espana EM, Tseng SCG, 2003. Ex vivo expansion of limbal epithelial stem cells: amniotic membrane serving as a stem cell niche. Surv. Ophthalmol. 48, 631 – 646

Hamrah P, Liu Y, Zhang Q, Dana MR. The corneal stroma is endowed with a significant number of resident dendritic cells. Invest Ophthalmol Vis Sci. 2003;44:581 – 9.

Holbach LM, Font RL, Baehr W, Pittler SJ. HSV antigen and HSV DNA in a vascular and vascularized lesion of Hornhautstroma keratitis. Curr Eye Res 1991; 10 Suppl. 63 – 68

Holbach LM, Seitz B, Rummelt C, Naumann GOH. An increasing optical density of stromal lesions is associated with a decrease of detectable HSV antigen in HS-keratitis. Invest Ophthalmol Vis Sci 1992; 33: 482

Holland EJ, Schwartz GS, 1996. The evolution of epithelial transplantation for severe ocular surface disease and a proposed classification system. Cornea 15, 549 – 556

Huang AJ, Tseng SC, 1991. Corneal epithelial wound healing in the absence of limbal epithelium. Invest. Ophthalmol. Vis. Sci. 32, 96 – 105

Inatomi T, Nakamura T, Koizumi N, Sotozono C, Kinoshita S, 2005. Current concepts and challenges in ocular surface reconstruction using cultivated mucosal epithelial transplantation. Cornea. 24, S32–S38

Kenyon KR, Tseng SC, 1989. Limbal autograft transplantation for ocular surface disorders. Ophthalmology 96, 709 – 722 Koizumi N, Inatomi T, Suzuki T, Sotozono C, Kinoshita S, 2001.

Cultivated corneal epithelial stem cell transplantation in ocular surface disorders. Ophthalmology 108, 1569 – 1574

Kruse FE, Reinhard T, 2001. Limbal transplantation for ocular surface reconstruction. Ophthalmologe 98, 818 – 831

Kruse FE, Cursiefen C, Seitz B, Voelcker HE, Naumann GOH, Holbach L. Klassifikation von Erkrankungen der Augenoeberflaeche. Teil I. Ophthalmologe 2003; 100:899 – 915

Küchle M, Cursiefen C, Fischer D-C, Schlötzer-Schrehardt U,

Naumann GOH. Recurrent macular corneal dystrophy type II 49 years after penetrating keratoplasty. Arch Ophthalmol 1999; 117: 528 – 531

Küchle M, Cursiefen C, Nguyen NX, Langenbucher A, Seitz B, Wenkel H, Martus P, Naumann GOH. Risk factors for corneal allograft rejection: intermediate results of a prospective normal-risk keratoplasty study. Graefe’s Arch Clin Exp Ophthalmol 2002; 240: 580 – 584

Lavker RM, Dong G, Cheng SZ, Kudoh K, Cotsarelis G, Sun TT, 1991. Relative proliferative rates of limbal and corneal epithelia. Implications of corneal epithelial migration, circadian rhythm, and suprabasally located DNA-synthesizing keratinocytes. Invest. Ophthalmol. Vis. Sci. 32, 1864 – 1875

Lavker RM, Sun T-T, 2000. Epidermal stem cells: properties, markers, and location. Proc. Natl. Acad. Sci. USA. 97, 10960 – 10965

Lavker RM, Tseng SCG, Sun T-T, 2004. Corneal epithelial stem cells at the limbus: looking at some old problems from a new angle. Exp. Eye Res. 78, 433 – 446

Lehrer MS, Sun T-T, Lavker RM, 1998. Strategies of epithelial repair: modulation of stem cell and transit amplifying cell proliferation. J. Cell Sci. 111, 2867 – 2875

Lindberg K, Brown ME, Chaves HV, Kenyon KR, Rheinwald JG, 1993. In vitro propagation of human ocular surface epithelial cells for transplantation. Invest. Ophthalmol. Vis. Sci. 34, 2672 – 2679

Maruyama K, Li M, Cursiefen C, Keino H, Tomita M, Takenaka H, Jackson DG, Losordo DW, Streilein JW. Inflammatory lymphangiogenesis arises from CD11b+ macrophages. J Clin Invest 2005; 115: 2363 – 2372

Muller LF, Marfurt CF, Kruse FE, Tervo T. Corneal nerves: structure, contents and function. Exp Eye Res. 2003; 76: 521 – 42 Naumann GOH et al. Pathologie des Auges. Berlin: Springer

1980; 2. edition, 1997

Naumann GOH, Sautter H, (Mitwirkung von Bigar F) Surgical procedures of the Cornea, chapter 7 in: “Surgical Ophthalmology 1”, Blodi FC, Mackensen G, Neubauer H. (eds.), Hei- delberg-New York, Springer Verlag, 433 – 508, 1991

Naumann GOH. The Bowman Lecture Nr. 56, Part II: Corneal Transplantation in Anterior Segment Diseases. Eye 1995; 9:398 – 421

Nguyen NX, Langenbucher A, Seitz B, Graupner M, Cursiefen C, Kuchle M, Naumann GOH. Blood-aqueous barrier breakdown after penetrating keratoplasty with simultaneous extracapsular cataract extraction and posterior chamber lens implantation. Graefe’s Arch Clin Exp Ophthalmol 2001; 239: 114 – 117

Nguyen NX, Seitz B, Langenbucher A, Wenkel H, Cursiefen C. [Clinical aspects and treatment of immunological endothelial graft rejection following penetrating normal-risk keratoplasty]. Klin Monatsbl Augenheilkd. 2004;221:467 – 72.

Pellegrini G, Traverso CE, Franzi AT, Zingirian M, Cancedda R, de Luca M, 1997. Long-term restoration of damaged corneal surface with autologous cultivated corneal epithelium. Lancet 349, 990 – 993

Puangsricharern V, Tseng SCG, 1995. Cytologic evidence of corneal diseases with limbal stem cell deficiency. Ophthalmology 102, 1476 – 1485

Sangwan VS, 2002. Limbal stem cells in health and disease. Biosci. Rep. 21, 385 – 405

Schermer A, Galvin S, Sun T-T, 1986. Differentiation-related expression of a major 64K corneal keratin in vivo and in culture. J. Cell Biol. 103, 49 – 62

Schlötzer-Schrehardt U, Kruse FE. Identification and characterization of limbal stem cells. Exp Eye Res. 2005;81:247 – 64 Schwab IR, Reyes M, Isseroff RR, 2000. Successful transplantation of bioengineered tissue replacements in patients with

ocular surface disease. Cornea 19, 421 – 426

Seitz B, Grüterich M, Cursiefen C, Kruse FE. Konservative und chirurgische Therapie der neurotrophen Keratopathie. Ophthalmologe 2005;102:15 – 26

Streilein JW. Ocular immune privilege: therapeutic opportunities from an experiment of nature. Nat Rev Immunol. 2003; 3:879 – 89

Sun T-T, Lavker RM, 2004. Corneal epithelial stem cells: past, presence, and future. J. Invest. Dermatol. Symp. Proc., 1 – 6.

Tseng SCG, 1989. Concept and application of limbal stem cells. Eye 3, 141 – 157

Tseng SCG, 1996. Regulation and clinical implications of corneal epithelial stem cells. Mol. Biol. Rep. 23, 47 – 58

Tseng SC, Meller D, Anderson DF, Touhami A, Pires RT, Grueterich M, Solomon A, Espana E, Sandoval H, Ti SE, Goto E, 2002. Ex vivo preservation and expansion of human limbal epithelial stem cells on amniotic membrane for treating corneal diseases with total limbal stem cell deficiency. Adv. Exp. Med. Biol. 506, 1323 – 1334

Vinh L, Nguyen N, Martus P, Seitz B, Kruse FE, Cursiefen C. Surgery-related factors influencing corneal neovascularization after low-risk keratoplasty. Am J Ophthalmol 2006; 141:260 – 266

A. Jünemann, G.O.H. Naumann

5.2.1

Principal Aspects of Glaucomas and Their Terminology

Glaucomas are a group of diseases that share as their main, but not only, risk factor an increased intraocular pressure leading to progressive and irreversible damage to the optic nerve head (Table 5.2.1). Acute glaucomas may develop sectorial iris necrosis and if the intraocular pressure is not relieved progressive ischemic infarcts of the prelaminar optic nerve disc are invaded by hyaluronic acid flowing from the vitreous cavity through the defective internal limiting membrane, socalled cavernous optic atrophy. In the acute phase the optic disc is prominent with indistinct borders – often invisible because of opaque optic media.

Chronic glaucomas develop a characteristic excavation, which can be documented by imaging techniques and quantified. Damage to the optic nerve head is the most important consequence of all glaucomas but the other intraocular tissues are also affected.

Primary glaucomas occur without obvious other intraocular disease, while secondary glaucomas follow other intraocular or extraocular diseases. The rise in intraocular pressure is almost always the consequence of an obstruction of the aqueous outflow either via the iris root covering the trabecular meshwork up to Schwalbe’s line or due to obstacles in the trabecular meshwork in a gonioscopically open angle. Hypersecretion of aqueous is rare and not of practical significance.

Therapy for the acute glaucomas as a rule requires immediate microsurgical intervention to improve out-

Table 5.2.1. Principal terminology of glaucomas

flow. Therapy for the chronic glaucomas today usually starts with medications, most often applied locally but occasionally systemically. We shall discuss below both laserand mechanical microsurgery for the improvement of aqueous outflow. If this is unsuccessful, cyclodestruction by various means has the aim of reducing aqueous inflow in the pars plicata of the ciliary body in order to balance the reduced outflow in acute and chronic glaucomas.

5.2.2

Surgical Anatomy

The intraocular pressure depends on a free flow of aqueous from the pars plicata of the ciliary body through the free space between the lens equator and ciliary body, the pupillary zone to the anterior chamber and the chamber angle. 85 ° continue through the trabecular meshwork to Schlemm’s canal and 15 % via the uveoscleral outflow following the supraciliary space. Flow from the secreting non-pigmenting ciliary epithelium through the narrow space between the lens equator and ciliary body is only rarely inhibited. Outflow through the normal pupillary zone has to overcome a physiologic pupillary resistance, which increases with age due to enlargement of the lens and rising rigidity of the iris sphincter. It is not continuous but intermitted and pulsatile. In comparison to other tissues the normal intraocular pressure is elevated. This is maintained by a physiologic resistance of outflow through the trabecular meshwork to Schlemm’s canal and the collector channels leading to the aqueous veins visible at the limbus corneae (Fig. 5.2.1).

5.2.2.1

Landmarks for Gonioscopy

Landmarks for gonioscopy can only be recognized if the histopathology is kept in mind. Schwalbe’s line or rim, corneoscleral and uveoscleral portions of the trabecular meshwork, scleral spur, and ciliary muscle insertion (Fig. 5.2.2) are gonioscopically the distinct parts of the anterior chamber angle (see also Chapter 5.3).

CO

FR

c

d

Fig. 5.2.1. Structures of anterior segment for aqueous circulation. a Secretion of pars plicata of ciliary body, physiological pupillary resistance and physiologic transtrabecular resistance to Schlemm’s canal, collector channels and episcleral aqueous veins. b Normal chamber angle iris root as thinnest part of the iris, peripheral to Fuchs’ roll (FR), collarette of iris (CO). c Section through Schlemm’s canal (SC), trabecular meshwork (TM), most peripheral anterior chamber (AC), anterior portion of ciliary body with ciliary processes (PP). Ciliary muscle (CM) d Trabecular meshwork showing uveocorneal and scleral corneal portion of trabecular meshwork and Schlemm’s canal with septae (Masson stain)

As persisting pathologic increased intraocular pressure originates from changes in the chamber angle, this region deserves special attention. The normal chamber angle really forms not an angle but rather a rounded bay and shows considerable variation in the degree of the angle between iris and cornea and also in the amount of uveal tissue connecting the iris stroma and scleral spur with strands up to Schwalbe’s line (Fig. 5.2.1f).

Orientation is facilitated with aging as phagocytized melanin granules in the trabecular endothelium by

their contrast allow easier recognition of the landmarks. Schlemm’s canal is not directly visible, but retrograde blood filling from the aqueous veins to the collector channels into Schlemm’s canal may help to locate this critical structure particularly in relatively young individuals – melanin dispersion and phagocytosis in the trabecular meshwork are usually not yet obvious. The relationship between Fuchs’ roll and Schwalbe’s ring must be kept in mind. Again, the position of the lens equator in relation to structures of the trabecular

c3

c5

Fig. 5.2.2 (Cont.)

meshwork and pars plicata of the ciliary body individually vary widely. Asian eyes often are characterized by their relatively narrow anterior segment, which plays a role in the more common variants of angle closure glaucoma – also with a chronic course.

5.2.2.2

Landmarks for Surgical Corneal Limbus

Obviously this is the second most important region in glaucoma surgery: Current microsurgical procedures focus on the region of the limbus corneae, a relatively vague term. The landmarks for the microsurgeon working under a conjunctival flap are the peripheral edge of Bowman’s layer and after creating a thick limbus based scleral flap the scleral spur, Schlemm’s canal and Schwalbe’s line or rim.

The anatomical limbus is situated where the peripheral cornea meets the sclera externally. The conjunctiva and Tenon’s fascia insert separately within about 0.5 mm; the conjunctival epithelium covers the peripheral insertion of Tenon’s fascia.

c4

The limbus is a broad area of transition about 1 mm in width. The microscopic structure of the central border of the limbus towards the cornea is the end of Bowman’s lamella and the end of Descemet’s membrane, respectively. The peripheral border of the limbus is the scleral spur. Macroscopically a bluish-gray nacre-like appearance, due to the extension of the deeper corneal lamellae beyond the external margin of the peripheral cornea, constitutes the surgical limbus (see Fig. 5.2.14).

Posterior to the deep extending corneal lamellae in the scleral bed the sclera shows a more whitish and nacre-like appearance within a small area. This is the external landmark of the scleral spur. Anterior to this landmark, Schlemm’s canal and trabecular meshwork appear as a grayish band. Retrograde blood filling of Schlemm’s canal, i.e., following paracentesis, marks it as a reddish gray band. Additional landmarks can be aqueous veins, lymphatic channels (see Chapter 5.1) and surrounding capillaries along the canal of Schlemm. The scleral spur extends slightly posterior to this junction. It is important to recognize these landmarks, particularly when performing trabeculotomy of “non-penetrating” filtration surgery. The ciliary body is attached to the uveocorneal junction of the trabecular band and the sclera at the scleral spur.

Dissection through the sclera posterior to this junction will expose the ciliary muscle behind the pars plicata. Dissection of the scleral spur in trabeculotomy due to the false position of the trabeculotomy probe may result in cyclodialysis with persistent ocular hypotony (Chapter 5.4).

5.2.3

Surgical Pathology

5.2.3.1

Angle Closure Glaucomas (ACG)

Gonioscopy or low magnification light microscopy shows that the access to the trabecular meshwork is occluded by the iris root in contact with the trabecular meshwork up to Schwalbe’s line (Figs. 5.2.3, 5.2.4a–d). Angle closures following pupillary or ciliary block (see Chapter 2) result in a drastic rise of intraocular pressure and the signs of an acute glaucoma: corneal endothelium decompensation, sectorial ischemic iris-necrosis with

immobile distorted pupil and massive conjunctival and ciliary hyperemia of the episcleral vessels and multiple focal necrosis of the lens epithelium (“Glaukomflecken”) (Fig. 5.2.5a–d). The cavernous atrophy of the optic disc usually is not visible because of the opaque media.

Patients from Asia also develop a chronic type of glaucoma due to progressive closure of the angle caused by iris plateau syndrome in the narrow anterior chamber with ciliary processes crowded behind the iris root.

Secondary ACGs most often develop rubeosis iridis following central retinal vein occlusion (CRVO), diabetic retinopathy and persisting retinal detachment (see Chapter 5.6). Insufficient restoration of the anteri-

a

c

Table 5.2.2. Classification of primary and secondary angle closure glaucoma

Pupillary block

Primary “classic acute angle closure glaucoma” Secondary: often with iris bomb´e

By ciliary block

Primary (very rare)

Secondary: ciliolenticular block: “malignant glaucoma”: ciliovitreal block: seen in aphakia (rare)

Without pupillary or ciliary block Primary: plateau iris (rare in Caucasians)

Secondary: due to peripheral synechiae without rubeosis iridis (e.g., associated with uveitis or after perforating injury)

With rubeosis iridis (associated with proliferative retinopathy) (e.g., diabetes mellitus, central vein occlusion and corneal endothelial overgrowth)

b

d

Fig. 5.2.5. Angle closure glaucomas. a–d Following pupillary block: a Extensive iris necrosis in brown iris after long-stand- ing pupillary block. b In comparison to the uninvolved eye. c, d Anterior pupillary block by lens luxation in anterior chamber. Intumescent cataract. Trabecular meshwork (TM). Cornea (C), Sclera (S)

Table 5.2.3. Differential diagnosis of angle closure glaucomas